Science.gov

Sample records for acute clinical manifestations

  1. Acute dacryocystitis: another clinical manifestation of sporotrichosis.

    PubMed

    Freitas, Dayvison Francis Saraiva; Lima, Iluska Augusta Rocha; Curi, Carolina Lemos; Jordão, Livia; Zancopé-Oliveira, Rosely Maria; Valle, Antonio Carlos Francesconi do; Galhardo, Maria Clara Gutierrez; Curi, Andre Luiz Land

    2014-04-01

    Sporotrichosis associated with exposure to domestic cats is hyperendemic in Rio de Janeiro, Brazil. A review of the clinical records at our institute revealed four patients with clinical signs of dacryocystitis and a positive conjunctival culture for Sporothrix who were diagnosed with Sporothrix dacryocystitis. Three patients were children (< 13 years of age) and one patient was an adult. Two patients reported contact with a cat that had sporotrichosis. Dacryocystitis was associated with nodular, ulcerated lesions on the face of one patient and with granulomatous conjunctivitis in two patients; however, this condition manifested as an isolated disease in another patient. All of the patients were cured of the fungal infections, but three patients had chronic dacryocystitis and one patient developed a cutaneous fistula. Sporotrichosis is usually a benign disease, but may cause severe complications when the eye and the adnexa are affected. Physicians, especially ophthalmologists in endemic areas, should be aware of the ophthalmological manifestations and complications of sporotrichosis.

  2. Acute dacryocystitis: another clinical manifestation of sporotrichosis

    PubMed Central

    Freitas, Dayvison Francis Saraiva; Lima, Iluska Augusta Rocha; Curi, Carolina Lemos; Jordão, Livia; Zancopé-Oliveira, Rosely Maria; do Valle, Antonio Carlos Francesconi; Galhardo, Maria Clara Gutierrez; Curi, Andre Luiz Land

    2013-01-01

    Sporotrichosis associated with exposure to domestic cats is hyperendemic in Rio de Janeiro, Brazil. A review of the clinical records at our institute revealed four patients with clinical signs of dacryocystitis and a positive conjunctival culture for Sporothrix who were diagnosed with Sporothrix dacryocystitis. Three patients were children (< 13 years of age) and one patient was an adult. Two patients reported contact with a cat that had sporotrichosis. Dacryocystitis was associated with nodular, ulcerated lesions on the face of one patient and with granulomatous conjunctivitis in two patients; however, this condition manifested as an isolated disease in another patient. All of the patients were cured of the fungal infections, but three patients had chronic dacryocystitis and one patient developed a cutaneous fistula. Sporotrichosis is usually a benign disease, but may cause severe complications when the eye and the adnexa are affected. Physicians, especially ophthalmologists in endemic areas, should be aware of the ophthalmological manifestations and complications of sporotrichosis. PMID:24810176

  3. Clinical Manifestation of Self-Limiting Acute Retinal Necrosis

    PubMed Central

    Brydak-Godowska, Joanna; Borkowski, Piotr; Szczepanik, Szymon; Moneta-Wielgoś, Joanna; Kęcik, Dariusz

    2014-01-01

    Background The purpose of this paper was to present a case series of self-limiting, peripheral acute retinal necrosis and to demonstrate efficacy of treatment with valacyclovir in patients resistant to acyclovir. The diagnosis was made on ophthalmoscopic examination and positive serum tests for herpes viruses. Material/Methods Ten patients (6F and 4M) aged 19–55 years were diagnosed and treated for self-limiting acute retinal necrosis (ARN). The following endpoints were reported: visual outcomes, clinical features, disease progression, treatment, and complications. Patients received only symptomatic treatment because they did not consent to vitreous puncture. Results Peripheral, mild retinitis was diagnosed in all eyes at baseline. Initially, all patients were treated with systemic acyclovir (800 mg, 5 times a day), prednisone (typically 40–60 mg/day), and aspirin in an outpatient setting. In 6 patients, treatment was discontinued at 6 months due to complete resolution of the inflammatory process. Four patients with immune deficiency showed signs and symptoms of chronic inflammation. Two patients did not respond to acyclovir (2 non-responders); however, those patients were successfully treated with valacyclovir. Complete resolution of inflammatory lesions was observed in 8 patients. In 2 patients, the disease progressed despite treatment – 1 female patient after kidney transplant who stopped the prescribed medications, and 1 male patient with SLE and antiphospholipid syndrome who experienced breakthrough symptoms on-treatment. He died due to cerebral venous sinus thrombosis. Neurological complications (encephalitis and meningitis) were observed in 2 female patients. Prophylactic laser photocoagulation was performed in 1 subject. Conclusions A series of cases of self-limiting acute retinal necrosis (ARN) is presented. This clinical form of ARN can resemble toxoplasmic retinitis in some cases. Oral antiviral medications provide an effective alternative to

  4. Clinical and pathological manifestations of cardiovascular disease in rat models: the influence of acute ozone exposure.

    PubMed

    Ramot, Yuval; Kodavanti, Urmila P; Kissling, Grace E; Ledbetter, Allen D; Nyska, Abraham

    2015-01-01

    Rodent models of cardiovascular diseases (CVD) and metabolic disorders are used for examining susceptibility variations to environmental exposures. However, cross-model organ pathologies and clinical manifestations are often not compared. We hypothesized that genetic CVD rat models will exhibit baseline pathologies and will thus express varied lung response to acute ozone exposure. Male 12-14-week-old healthy Wistar Kyoto (WKY), Wistar (WIS), and Sprague-Dawley (SD) rats and CVD-compromised spontaneously hypertensive (SH), fawn-hooded hypertensive (FHH), stroke-prone SH (SHSP), obese SH heart-failure (SHHF), obese diabetic JCR (JCR) rats were exposed to 0.0, 0.25, 0.5, or 1.0 ppm ozone for 4 h and clinical biomarkers, and lung, heart and kidney pathologies were compared immediately following (0-h) or 20-h later. Strain differences were observed between air-exposed CVD-prone and WKY rats in clinical biomarkers and in kidney and heart pathology. Serum cholesterol was higher in air-exposed obese SHHF and JCR compared to other air-exposed strains. Ozone did not produce lesions in the heart or kidney. CVD-prone and SD rats demonstrated glomerulopathy and kidney inflammation (WKY = WIS = SH < SD = SHSP < SHHF < JCR = FHH) regardless of ozone. Cardiac myofiber degeneration was evident in SH, SHHF, and JCR, while only JCR tends to have inflammation in coronaries. Lung pathology in air-exposed rats was minimal in all strains except JCR. Ozone induced variable alveolar histiocytosis and bronchiolar inflammation; JCR and SHHF were less affected. This study provides a comparative account of the clinical manifestations of disease and early-life organ pathologies in several rat models of CVD and their differential susceptibility to lung injury from air pollutant exposure.

  5. [Acute hemolytic crisis followed by fulminant hepatic failure with fatal outcome, as a first clinical manifestation of Wilson's disease].

    PubMed

    de Andrade Júnior, D R; Fujita Neto, F G; Vieira, G S; Tibério, I F; Warth, M P; Calich, I

    1994-01-01

    We describe in this work a clinical case of a female patient aged 21 years, bearer of Wilson's disease, a first clinical manifestation of the disease occurred as an acute hemolytic crisis followed by fulminant hepatic failure evolving to death after 26 days' internment. The definitive diagnosis was obtained only as a quantitative measurement of hepatic copper from the necropsy material. The search for Kayser-Fleischer ring was negative and the serum ceruloplasmin level was 9 mg/dl (15 to 60). No involvement of the central nervous system was noted from the pathologic analysis. The patient presented two Coombs negative hemolytic crises during the internment; the first on being admitted to hospital and the second after a transjugular hepatic biopsy carried out on the 16th day after internment. The last hemolytic crisis was accompanied by an increase of serum and urinary copper levels. On this occasion the patient evolved to a progressive hepatic failure with severe jaundice and hepatic encephalopathy. We are presenting the clinical-biochemical evolution of the patient and we shall discuss the existent hypotheses to the pathophysiology of this rare form for manifestation of the Wilson's disease as well the diagnostic difficulties.

  6. Clinical manifestations of synovial cysts.

    PubMed

    Burt, T B; MacCarter, D K; Gelman, M I; Samuelson, C O

    1980-08-01

    Although synovial cysts are most commonly associated with rheumatoid arthritis and osteoarthritis, they may occur in many other conditions. The clinical manifestations of these cysts are numerous and may result from pressure, dissection or acute rupture. Vascular phenomena occur when popliteal cysts compress vessels, and result in venous stasis with subsequent lower extremity edema or thrombophlebitis. Rarely, popliteal cysts may cause arterial compromise with intermittent claudication. Neurological sequelae include pain, paresthesia, sensory loss, and muscle weakness or atrophy. When synovial cysts occur as mass lesions they may mimic popliteal aneurysms or hematomas, adenopathy, tumors or even inguinal hernias. Cutaneous joint fistulas, septic arthritis or osteomyelitis, and spinal cord and bladder compression are examples of other infrequent complications. Awareness of the heterogeneous manifestations of synovial cysts may enable clinicians to avoid unnecessary diagnostic studies and delay in appropriate management. Arthrography remains the definitive diagnostic procedure of choice, although ultrasound testing may be useful.

  7. Microbiologic Characteristics, Serologic Responses, and Clinical Manifestations in Severe Acute Respiratory Syndrome, Taiwan1

    PubMed Central

    Hsueh, Po-Ren; Hsiao, Cheng-Hsiang; Yeh, Shiou-Hwei; Wang, Wei-Kung; Chen, Pei-Jer; Wang, Jin-Town; Chang, Shan-Chwen

    2003-01-01

    The genome of one Taiwanese severe acute respiratory syndrome-associated coronavirus (SARS-CoV) strain (TW1) was 29,729 nt in length. Viral RNA may persist for some time in patients who seroconvert, and some patients may lack an antibody response (immunoglobulin G) to SARS-CoV >21 days after illness onset. An upsurge of antibody response was associated with the aggravation of respiratory failure. PMID:14519257

  8. Differences in Clinical Manifestations of Acute and Early HIV-1 Infection between HIV-1 Subtypes in African Women

    PubMed Central

    Watkins, Richard R.; Morrison, Charles S.; Kwok, Cynthia; Chipato, Tsungai; Musoke, Robert; Arts, Eric J.; Nankya, Immaculate; Salata, Robert A.

    2015-01-01

    Little is known about the differences in clinical manifestations between women with various HIV-1 subtypes during acute (AI) and early (EI) HIV infection. In a longitudinal cohort study, clinical signs and symptoms among Uganda and Zimbabwe women with AI and EI were compared with HIV-negative controls; symptoms were assessed quarterly for 15 to 24 months. Early HIV infection was defined as the first visit during which a woman tested HIV antibody positive. Women who were HIV negative serologically but DNA polymerase chain reaction positive were considered AI. In all, 26 women were classified AI and 192 EI, with 654 HIV-negative controls. Primary HIV infection (AI and EI) was associated with unexplained fever (P <.01), weight loss (P <.01), fatigue (P <.01), inguinal adenopathy (P <.01), and cervical friability (P =.01). More women with subtype C infection had unexplained fever, fatigue, and abnormal vaginal discharge compared to subtype A or D infection. Inguinal adenopathy occurred less often in women with subtype A infection than those with subtype C or D infection. PMID:24106054

  9. Differences in Clinical Manifestations of Acute and Early HIV-1 Infection between HIV-1 Subtypes in African Women.

    PubMed

    Lemonovich, Tracy L; Watkins, Richard R; Morrison, Charles S; Kwok, Cynthia; Chipato, Tsungai; Musoke, Robert; Arts, Eric J; Nankya, Immaculate; Salata, Robert A

    2015-01-01

    Little is known about the differences in clinical manifestations between women with various HIV-1 subtypes during acute (AI) and early (EI) HIV infection. In a longitudinal cohort study, clinical signs and symptoms among Uganda and Zimbabwe women with AI and EI were compared with HIV-negative controls; symptoms were assessed quarterly for 15 to 24 months. Early HIV infection was defined as the first visit during which a woman tested HIV antibody positive. Women who were HIV negative serologically but DNA polymerase chain reaction positive were considered AI. In all, 26 women were classified AI and 192 EI, with 654 HIV-negative controls. Primary HIV infection (AI and EI) was associated with unexplained fever (P <.01), weight loss (P <.01), fatigue (P <.01), inguinal adenopathy (P <.01), and cervical friability (P =.01). More women with subtype C infection had unexplained fever, fatigue, and abnormal vaginal discharge compared to subtype A or D infection. Inguinal adenopathy occurred less often in women with subtype A infection than those with subtype C or D infection.

  10. Clinical manifestation of mitochondrial diseases.

    PubMed

    Magner, Martin; Kolářová, Hana; Honzik, Tomáš; Švandová, Ivana; Zeman, Jiří

    2015-01-01

    Mitochondrial disorders (MD) represent a clinically, biochemically and genetically heterogeneous group of diseases associated with dysfunction of the oxidative phosphorylation system and pyruvate dehydrogenase complex. Our aim was to illustrate the most common clinical presentation of MD on the example of selected diseases and syndromes. The minimal prevalence of MD is estimated as 1 to 5,000. MD may manifest at any age since birth until late-adulthood with acute manifestation or as a chronic progressive disease. Virtually any organ may be impaired, but the organs with the highest energetic demands are most frequently involved, including brain, muscle, heart and liver. Some MD may manifest as a characteristic cluster of clinical features (e.g. MELAS syndrome, Kearns-Sayre syndrome). Diagnostics includes detailed history, the comprehensive clinical examination, results of specialized examinations (especially cardiology, visual fundus examination, brain imaging, EMG), laboratory testing of body fluids (lactate, aminoacids, organic acids), and analysis of bioptic samples of muscle, skin, and liver, eventually. Normal lactate level in blood does not exclude the possibility of MD. Although the aimed molecular genetic analyses may be indicated in some of mitochondrial diseases, the methods of next generation sequencing come into focus. Examples of treatment are arginine supplementation in MELAS syndrome, ketogenic diet in pyruvate oxidation disorders or quinone analogs in patients with LHON. Conclusion: The clinical suspicion of a mitochondrial disorder is often delayed, or the disease remains undiagnosed. The correct diagnosis and adequate treatment can improve prognosis of the patient. Access to genetic counseling is also of great importance.

  11. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    PubMed Central

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF. PMID:27625576

  12. Acute pancreatitis: Manifestation of acute HIV infection in an adolescent

    PubMed Central

    Bitar, Anas; Altaf, Muhammad; Sferra, Thomas J.

    2012-01-01

    Summary Background: Pancreatitis in the pediatric age group is not as common as in adults. Etiologies are various and differ from those in adults. Although infectious etiology accounts for a significant number of cases of pancreatitis, acute infection with Human Immunodeficiency Virus (HIV) was rarely reported as a possible etiology for acute pancreatitis in adults. Acute pancreatitis has never been reported as a presenting manifestation of acute HIV infection in children. Case Report: We describe a pediatric patient who presented with acute pancreatitis that revealed acute HIV infection. Conclusions: Acute pancreatitis as a primary manifestation of HIV infection is very rare. It may represent an uncommon aspect of primary HIV infection. We suggest that acute HIV infection should be considered in the differential diagnosis of acute pancreatitis at all ages. PMID:23569476

  13. ACUTE KIDNEY INJURY CAUSED BY Crotalus AND Bothrops SNAKE VENOM: A REVIEW OF EPIDEMIOLOGY, CLINICAL MANIFESTATIONS AND TREATMENT

    PubMed Central

    Albuquerque, Polianna L.M.M.; Jacinto, Camilla N.; Silva, Geraldo B.; Lima, Juliana B.; Veras, Maria do Socorro B.; Daher, Elizabeth F.

    2013-01-01

    SUMMARY Ophidic accidents are an important public health problem due to their incidence, morbidity and mortality. An increasing number of cases have been registered in Brazil in the last few years. Several studies point to the importance of knowing the clinical complications and adequate approach in these accidents. However, knowledge about the risk factors is not enough and there are an increasing number of deaths due to these accidents in Brazil. In this context, acute kidney injury (AKI) appears as one of the main causes of death and consequences for these victims, which are mainly young males working in rural areas. Snakes of the Bothrops and Crotalus genera are the main responsible for renal involvement in ophidic accidents in South America. The present study is a literature review of AKI caused by Bothrops and Crotalus snake venom regarding diverse characteristics, emphasizing the most appropriate therapeutic approach for these cases. Recent studies have been carried out searching for complementary therapies for the treatment of ophidic accidents, including the use of lipoic acid, simvastatin and allopurinol. Some plants, such as Apocynaceae, Lamiaceae and Rubiaceae seem to have a beneficial role in the treatment of this type of envenomation. Future studies will certainly find new therapeutic measures for ophidic accidents. PMID:24037282

  14. Acute kidney injury caused by Crotalus and Bothrops snake venom: a review of epidemiology, clinical manifestations and treatment.

    PubMed

    Albuquerque, Polianna L M M; Jacinto, Camilla N; Silva Junior, Geraldo B; Lima, Juliana B; Veras, Maria do Socorro B; Daher, Elizabeth F

    2013-01-01

    Ophidic accidents are an important public health problem due to their incidence, morbidity and mortality. An increasing number of cases have been registered in Brazil in the last few years. Several studies point to the importance of knowing the clinical complications and adequate approach in these accidents. However, knowledge about the risk factors is not enough and there are an increasing number of deaths due to these accidents in Brazil. In this context, acute kidney injury (AKI) appears as one of the main causes of death and consequences for these victims, which are mainly young males working in rural areas. Snakes of the Bothrops and Crotalus genera are the main responsible for renal involvement in ophidic accidents in South America. The present study is a literature review of AKI caused by Bothrops and Crotalus snake venom regarding diverse characteristics, emphasizing the most appropriate therapeutic approach for these cases. Recent studies have been carried out searching for complementary therapies for the treatment of ophidic accidents, including the use of lipoic acid, simvastatin and allopurinol. Some plants, such as Apocynaceae, Lamiaceae and Rubiaceae seem to have a beneficial role in the treatment of this type of envenomation. Future studies will certainly find new therapeutic measures for ophidic accidents.

  15. Hereditary angioedema: imaging manifestations and clinical management.

    PubMed

    Gakhal, Mandip S; Marcotte, Gregory V

    2015-02-01

    Hereditary angioedema is a genetic disorder typically related to insufficient or dysfunctional C1-esterase inhibitor. Patients present with episodic swelling of various body parts, such as the face, neck, bowel, genitals, and extremities. Acute or severe symptoms can lead to patients presenting to the emergency room, particularly when the neck and abdominopelvic regions are affected, which is often accompanied by radiologic imaging evaluation. Patients with hereditary angioedema can pose a diagnostic challenge for emergency department physicians and radiologists at initial presentation, and the correct diagnosis may be missed or delayed, due to lack of clinical awareness of the disease or lack of its consideration in the radiologic differential diagnosis. Timely diagnosis of hereditary angioedema and rapid initiation of appropriate therapy can avoid potentially life-threatening complications. This article focuses on the spectrum of common and characteristic acute imaging manifestations of hereditary angioedema and provides an update on important recent developments in its clinical management and treatment.

  16. Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis

    PubMed Central

    Gopal, Mangala; Patel, Harsh

    2017-01-01

    Central pontine myelinolysis (CPM) is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Common clinical manifestations of CPM include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees; however, coma, “locked-in” syndrome, or death can occur in most severe cases. Rarely, CPM presents with neuropsychiatric manifestations, such as personality changes, acute psychosis, paranoia, hallucinations, or catatonia, typically associated with additional injury to the brain, described as extrapontine myelinolysis (EPM). We present a patient with primarily neuropsychiatric manifestations of CPM, in the absence of focal neurologic deficits or radiographic extrapontine involvement. A 51-year-old female without significant medical history presented with dizziness, frequent falls, diarrhea, generalized weakness, and weight loss. Physical examination showed no focal neurological deficits. Laboratory data showed severe hyponatremia, which was corrected rather rapidly. Subsequently, the patient developed symptoms of an acute psychotic illness. Initial brain magnetic resonance imaging (MRI) was unremarkable, although a repeat MRI two weeks later revealed changes compatible with CPM. This case demonstrates that acute psychosis might represent the main manifestation of CPM, especially in early stages of the disease, which should be taken into consideration when assessing patients with acute abnormalities of sodium metabolism. PMID:28392953

  17. The neurologic manifestations of the acute porphyrias.

    PubMed

    Simon, Neil G; Herkes, Geoffrey K

    2011-09-01

    The porphyrias are diseases characterised by accumulation of porphyrins and porphyrin precursors owing to enzymatic deficiencies of the haem synthetic pathway. In the acute hepatic porphyrias accumulation of porphyrin precursors, in particular delta-aminolaevulinic acid (ALA), cause dysfunction of the central, peripheral and autonomic nervous systems. This leads to the characteristic clinical findings of abdominal pain, neuropsychiatric symptoms and neuropathy. The exact pathogenic mechanism is not clear but evidence to date suggests both direct toxic effects of ALA and intracellular metabolic derangement contribute to the neurologic disorders. This review explores the mechanisms of neural dysfunction in the acute porphyrias and the resultant clinical features of an acute attack.

  18. [Cellulitis: clinical manifestations and management].

    PubMed

    Blum, C-L; Menzinger, S; Genné, D

    2013-10-09

    Cellulitis is an acute bacterial non-necrotizing dermal-hypodermal infection predominantly affecting the lower limbs. It is characterised by a circumscribed erythema with a raised border and fever. The predisposing factors are skin wounds, edema from any cause and systemic factors (diabetes, immunosuppression). The diagnosis is clinical and the most common complication is recurrence. Other complications include local abscess, fasciitis and bacteremia. The germ is rarely identified. The majority of infections (85%) is due to group A beta-hemolytic streptococcus. The treatment of cellulitis consists of an association of an antibiotic with rest of the concerned area.

  19. Unusual manifestations of acute Q fever: autoimmune hemolytic anemia and tubulointerstitial nephritis.

    PubMed

    Korkmaz, Serdal; Elaldi, Nazif; Kayatas, Mansur; Sencan, Mehmet; Yildiz, Esin

    2012-05-18

    Q fever is a worldwide zoonotic infection that caused by Coxiella burnetii, a strict intracellular bacterium. It may be manifested by some of the autoimmune events and is classified into acute and chronic forms. The most frequent clinical manifestation of acute form is a self-limited febrile illness which is associated with severe headache, muscle ache, arthralgia and cough. Meningoencephalitis, thyroiditis, pericarditis, myocarditis, mesenteric lymphadenopathy, hemolytic anemia, and nephritis are rare manifestations. Here we present a case of acute Q fever together with Coombs' positive autoimmune hemolytic anemia (AIHA) and tubulointerstitial nephritis treated with chlarithromycin, steroids and hemodialysis. Clinicians should be aware of such rare manifestations of the disease.

  20. Clinical manifestations of sleep apnea

    PubMed Central

    2015-01-01

    Obstructive sleep apnea (OSA) may manifest in a number of ways from subtle intrusion into daily life to profound sleepiness, snoring, witnessed apneas and other classic symptoms. Although there is increasing evidence suggesting OSA can adversely affect health in a variety of ways, this disorder remains underdiagnosed. The most well-escribed health consequences of OSA relate to the cardiovascular system. Hypertension and arrhythmias have a strong association with OSA, and evidence suggests that treatment of OSA in patients with refractory hypertension and in patients planning cardioversion for atrial fibrillation may be of particularly importance. Significant associations between heart failure and OSA as well as complex sleep apnea have also been well-described. Cerebrovascular insult, impaired neurocognition, and poorly controlled mood disorder are also associated with in OSA. Therapy for OSA may ameliorate atherosclerotic progression and improve outcomes post-cerebrovascular accident (CVA). OSA should be considered in patients complaining of poor concentration at work, actual or near-miss motor vehicle accidents, and patients with severe sleepiness as a component of their co-morbid mood disorders. The metabolic impact of OSA has also been studied, particularly in relation to glucose homeostasis. Also of interest is the potential impact OSA has on lipid metabolism. The adverse effect untreated OSA has on glucose tolerance and lipid levels has led to the suggestion that OSA is yet another constituent of the metabolic syndrome. Some of these metabolic derangements may be related to the adverse effects untreated OSA has on hepatic health. The cardiovascular, neurocognitive, and metabolic manifestations of OSA can have a significant impact on patient health and quality of life. In many instances, evidence exists that therapy not only improves outcomes in general, but also modifies the severity of co-morbid disease. To mitigate the long-term sequela of this disease

  1. Clinical manifestations of sleep apnea.

    PubMed

    Stansbury, Robert C; Strollo, Patrick J

    2015-09-01

    Obstructive sleep apnea (OSA) may manifest in a number of ways from subtle intrusion into daily life to profound sleepiness, snoring, witnessed apneas and other classic symptoms. Although there is increasing evidence suggesting OSA can adversely affect health in a variety of ways, this disorder remains underdiagnosed. The most well-escribed health consequences of OSA relate to the cardiovascular system. Hypertension and arrhythmias have a strong association with OSA, and evidence suggests that treatment of OSA in patients with refractory hypertension and in patients planning cardioversion for atrial fibrillation may be of particularly importance. Significant associations between heart failure and OSA as well as complex sleep apnea have also been well-described. Cerebrovascular insult, impaired neurocognition, and poorly controlled mood disorder are also associated with in OSA. Therapy for OSA may ameliorate atherosclerotic progression and improve outcomes post-cerebrovascular accident (CVA). OSA should be considered in patients complaining of poor concentration at work, actual or near-miss motor vehicle accidents, and patients with severe sleepiness as a component of their co-morbid mood disorders. The metabolic impact of OSA has also been studied, particularly in relation to glucose homeostasis. Also of interest is the potential impact OSA has on lipid metabolism. The adverse effect untreated OSA has on glucose tolerance and lipid levels has led to the suggestion that OSA is yet another constituent of the metabolic syndrome. Some of these metabolic derangements may be related to the adverse effects untreated OSA has on hepatic health. The cardiovascular, neurocognitive, and metabolic manifestations of OSA can have a significant impact on patient health and quality of life. In many instances, evidence exists that therapy not only improves outcomes in general, but also modifies the severity of co-morbid disease. To mitigate the long-term sequela of this disease

  2. Radiation nephritis. Clinical manifestations and pathophysiologic mechanisms

    SciTech Connect

    Krochak, R.J.; Baker, D.G.

    1986-05-01

    Radiation nephritis is both volume and dose related. Clinical experience would indicate that a minimum of one third of the renal volume needs to be excluded from nephrotoxic doses which appears to have a threshold of 2,000 cGy. The site of damage leading to renal failure appears to be the microvasculature ultimately expressed as glomerulosclerosis. How much direct damage to the tubular system contributes to this process is unclear, but undoubtedly the resultant systemic physiologic effects potentiate the expression of damage in the irradiated kidney. The acute syndrome, with all the potential manifestations of renal failure, rarely presents sooner than six months and appears to have no clear prodrome, although it would seem reasonable that a subclinical syndrome consisting of abnormalities detectable by urinalysis may occur. Treatment of radiation-induced nephritis or hypertension is no different from treatment for nephritis from any other cause and should be aggressive with lifelong follow-up. Carcinogenesis is a rare late expression of radiation-induced kidney damage. 25 references.

  3. Chloracne. Clinical manifestations and etiology

    SciTech Connect

    Zugerman, C. )

    1990-01-01

    Chloracne is a rare but important acneiform eruption often associated with the ingestion of chlorinated phenolic agents such as dioxins with subsequent toxicity from these chemicals. Clinically, chloracne can be distinguished from acne vulgaris by the distribution and appearance of the lesions and by taking a detailed history. In some instances, it may be associated with particularly xerotic skin, pigmentation, follicular hyperkeratosis, conjunctivitis, and actinic elastosis. Histologically, the primary lesion is a follicular plug containing keratinous material. Chloracne is difficult if not impossible to treat adequately and once present, may persist for years. Consequently, good hygiene, safe manufacturing processes so that no inhalation or skin contact is possible, and the elimination of atmospheric contamination are all necessary in the prevention of this potentially debilitating disease.34 references.

  4. ECG manifestations in acute organophosphorus poisoning.

    PubMed

    Paul, Uttam Kumar; Bhattacharyya, Anup Kumar

    2012-02-01

    A cross-sectional study was conducted to evaluate the electrocardiographic changes in 107 patients of acute organophosphorus poisoning admitted at casuality ward of MGM Medical College, Kisanganj from June 2007 to June 2010. Electrocardiographic changes were recorded before the administration of atropine. Prolonged Q-Tc interval was the commonest ECG abnormality, found in 67 patients (62.6%), followed by sinus tachycardia in 36 patients (33.6%). Sinus bradycardia was found in 33 patients (30.8%). Elevation of ST segment was seen in 27 patients (25.2%). T wave inversion was seen in 21 patients (19.6%). First-degree heart block (P-R interval >0.20 seconds) occurred in 9 cases (8.4%). Atrial fibrillation was seen in 5 patients (4.6%). Ventricular tachycardia was seen in 6 cases (5.6%) and ventricular premature complexes in 3 patients (2.8%). Of these 6 cases of ventricular tachycardia 1 responded to intravenous lignocaine, and the other 5 developed ventricular fibrillation leading to death despite other resuscitative measures. All the electrocardiographical abnormalities returned to normal before the patients were discharged. Seventeen patients died. The cause of death was ventricular fibrillation in 5 patients and non-cardiogenic pulmonary oedema in others. In conclusion it can be said that ECG should be carefully recorded and analysed in all patients of acute organophosphorus poisoning, and depending upon these changes and other clinical and biochemical parameters, the patients should immediately be shifted to well equipped ICU for better care which will reduce the mortality rate caused by these highly lethal poisons.

  5. Clinical manifestations of essential tremor

    PubMed Central

    Critchley, Edmund

    1972-01-01

    A clinical study of 42 patients with essential tremor is presented. In the case of 12 patients the family history strongly suggested an autosomal dominant mode of transmission, in four the mode of inheritance was indeterminate, and the remaining 26 patients were sporadic cases without an established genetic basis. The tremor involved the upper extremities in 41 patients, the head in 25, lower limbs in 15, and trunk in two. Seven patients showed involvement of speech. Variations were found in the speed and regularity of the tremor. Leg involvement took a variety of forms: (1) direct involvement by tremor; (2) a painful limp associated with forearm tremor; (3) associated dyskinetic movements; (4) ataxia; (5) foot clubbing; and (6) evidence of peroneal muscular atrophy. Several minor symptoms—hyperhidrosis, cramps, dyskinetic movements, and ataxia—were associated with essential tremor. Other features were linked phenotypically to the ataxias and system degenerations. Apart from minor alterations in tone, expression, and arm swing, features of Parkinsonism were notably absent. Images PMID:5035310

  6. Gender and its clinical manifestations.

    PubMed

    Olesker, Wendy

    2003-01-01

    Based on a study of five analytic cases (three reported here), and using a modern classical psychoanalytic perspective in which both biology and social forces are integrated, our study group on gender investigated why for some gender assumes a distorted and exaggerated role in the subjective sense of self. Clinical material was used because we wanted data allowing us to study patterns emerging from an exploration of patients' unconscious fantasies as they shed light on the psychological significance and function of gender. We found that in each case gender disturbances were never primary but secondary to difficulties in integration, cohesiveness, separateness, stability of solid sense of self and of the object, to depression and especially to problems with aggression and rivalry which had to be analyzed first. Conflicted gender gave rise to solutions that were literal and concrete. Not surprisingly, in each case mothers wished for a child of the opposite gender and treated gender expression in highly ambivalent ways. In terms of technique we learned that ways of coping with rejection, evolving paternal transferences which allowed for metabolisis of the maternal relationship, and ego building techniques had to take place before conflicts around gender could be analyzed productively.

  7. [Lyme disease--clinical manifestations and treatment].

    PubMed

    Stock, Ingo

    2016-05-01

    Lyme disease (Lyme borreliosis) is a systemic infectious disease that can present in a variety of clinical manifestations. The disease is caused by a group of spirochaetes--Borrelia burgdorferi sensu lato or Lyme borrelia--that are transmitted to humans by the bite of Ixodes ticks. Lyme disease is the most common arthropode-borne infectious disease in many European countries including Germany. Early localized infection is typically manifested by an erythema migrans skin lesion, in rarer cases as a borrelial lymphocytoma. The most common early disseminated manifestation is (early) neuroborreliosis. In adults, neuroborreliosis appears typically as meningoradiculoneuritis. Neuroborreliosis in children, however, is typically manifested by meningitis. In addition, multiple erythema migrans lesions and Lyme carditis occur relatively frequently. The most common manifestation oflate Lyme disease is Lyme arthritis. Early manifestations (and usually also late manifestations) of Lyme disease can be treated successfully by application of suitable antibacterial agents. For the treatment of Lyme disease, doxycycline, certain penicillins such as amoxicillin and some cephalosporins (ceftriaxone, cefotaxime, cefuroxime axetil) are recommended in current guidelines. A major challenge is the treatment of chronic, non-specific disorders, i. e., posttreatment Lyme disease syndrome and "chronic Lyme disease". Prevention of Lyme disease is mainly accomplished by protecting against tick bites. Prophylactic administration of doxycycline after tick bites is generally not recommended in Germany. There is no vaccine available for human beings.

  8. Sickle cell disease: acute clinical manifestations in early childhood and molecular characteristics in a group of children in Rio de Janeiro

    PubMed Central

    da Silva Filho, Isaac Lima; Ribeiro, Georgina Severo; Moura, Patrícia Gomes; Vechi, Monica Longo; Cavalcante, Andréa Cony; de Andrada-Serpa, Maria José

    2012-01-01

    Objectives To describe clinical events of sickle cell disease and the correlation with β-globin haplotypes and α-thalassemia in under 6-year-old children. Methods A retrospective study was conducted of under 6-year-old children from the neonatal screening program in Rio de Janeiro. Forty-eight male and 48 female children were enrolled in this study, 79 with sickle cell anemia and 17 with hemoglobin SC. The mean age was 29.9 (standard deviation = 20.9) months, 62 (16.2 ± 8.6) were aged between 0-3 years old and 34 (54.9 ± 11.3) were from 3-6 years old. Painful events, acute splenic sequestration, hemolytic crises, hand-foot and acute chest syndromes and infections were evaluated. Results The events were more frequent in under 3-year-old children, 94% of children had at least one episode. Infection was the most common event affecting 88.5% of children. Acute splenic sequestration took place earlier, while painful crises and acute chest syndromes in under 6-year-old children. Thal-α 3.7 was observed in 20.9% of cases. Bantu was the most frequent haplotype found, followed by Benin. No correlation was observed between clinical events and β-globin haplotypes. Children with sickle cell anemia and α-thalassemia have less infectious events. No correlation was found among these polymorphisms and clinical events, however, the majority of children with Bantu/Bantu and without α-thalassemia had more clinical events. PMID:23049419

  9. Clinical and pathological manifestations of human henipavirus infection.

    PubMed

    Wong, K T; Tan, C T

    2012-01-01

    The clinicopathological features of human Nipah virus and Hendra virus infections appear to be similar. The clinical manifestations may be mild, but if severe, includes acute encephalitic and pulmonary syndromes with a high mortality. The pathological features in human acute henipavirus infections comprise vasculopathy (vasculitis, endothelial multinucleated syncytia, thrombosis), microinfarcts and parenchymal cell infection in the central nervous system, lung, kidney and other major organs. Viral inclusions, antigens, nucleocapsids and RNA are readily demonstrated in blood vessel wall and numerous types of parenchymal cells. Relapsing henipavirus encephalitis is a rare complication reported in less than 10% of survivors of the acute infection and appears to be distinct from the acute encephalitic syndrome. Pathological evidence suggests viral recrudescence confined to the central nervous system as the cause.

  10. Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

    PubMed Central

    Alkhotani, Ashjan; Shirah, Bader

    2016-01-01

    Background and Purpose Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before. Methods A retrospective database review of all patients with a diagnosis of APMPPE was performed. Clinical, ophthalmological, and neurological data were analyzed, and only cases of APMPPE with neurological complications were included. A literature review of several databases was also performed, and previous case reports were reviewed and analyzed in detail. Results In total, 56 cases of APMPPE-associated neurological complications were included in the analyses: 54 from the literature and 2 from our own practice. The most common complication was cerebral vasculitis, which affected 28 patients (50%), followed by headaches in 15 patients (26.8%). The other complications include sixth-cranial-nerve palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, and viral meningitis. Conclusions This report adds to the literature of a novel association of APMPPE with peripheral neuropathy, and comprehensively reviews the neurological manifestations of this disease. A high level of suspicion should be applied when dealing with a case of APMPPE. We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. Early treatment with steroids and long-term treatment with immunosuppressive azathioprine with interval neurological evaluations will contribute positively to the outcomes and avoid fatal complications, namely strokes. PMID:27819416

  11. [Cerebrotendinous xanthomatosis: physiopathology, clinical manifestations and genetics].

    PubMed

    Preiss, Yudith; Santos, José L; Smalley, Susan V; Maiz, Alberto

    2014-05-01

    Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disease, caused by genetic deficiency of the 27-hydroxylase enzyme (encoded by CYP27A1). It plays a key role in cholesterol metabolism, especially in bile acid synthesis and in the 25-hydroxylation of vitamin D3 in the liver. Its deficiency causes reduced bile acid synthesis and tissue accumulation of cholestanol. Clinical manifestations are related to the presence of cholestanol deposits and include tendon xanthomas, premature cataracts, chronic diarrhea, progressive neurologic impairment and less frequently coronary heart disease, early onset osteoporosis and abnormalities in the optic disk and retina. An early diagnosis and treatment with quenodeoxycholic acid may prevent further complications, mainly neurological manifestations. This review summarizes cholesterol metabolism related to bile acid synthesis, physiopathology, biochemistry and treatment of cerebrotendinous xanthomatosis.

  12. Patent foramen ovale: clinical manifestations and treatment.

    PubMed

    Kedia, Gautam; Tobis, Jonathan; Lee, Michael S

    2008-01-01

    A persistent patent foramen ovale produces an intermittent intra-atrial right-to-left shunt and occurs in approximately 25% of the general population. Although the vast majority of people with patent foramen ovale are asymptomatic, a patent foramen ovale is believed to act as a pathway for chemicals or thrombus that can result in a variety of clinical manifestations, including stroke, migraine headache, decompression sickness, high-altitude pulmonary edema, and platypnea-orthodeoxia syndrome. The optimal management of patients with patent foramen ovale who experience cryptogenic stroke is unclear. Percutaneous closure appears to have a low risk profile and has been considered in high-risk patients who are not candidates for randomized clinical trials. Randomized clinical trials that are underway should help define the best management of patent foramen ovale, as well as the true safety and efficacy of percutaneous closure devices.

  13. Clinical manifestations of choanal atresia in infancy.

    PubMed

    Cozzi, F; Steiner, M; Rosati, D; Madonna, L; Colarossi, G

    1988-03-01

    The clinical features of 41 infants with various types of nasal obstruction were reviewed to study the correlation between the degree of nasal obstruction and clinical manifestations. Twenty-one subjects had bilateral choanal atresia/stenosis; 12 had unilateral choanal atresia/stenosis, and eight who were referred with suspected choanal atresia had a simple inflammatory nasal obstruction. Patients with bilateral choanal obstruction and patients with unilateral choanal obstruction or rhinitis showed no differences in clinical picture or in referral age. Many infants with either unilateral or bilateral choanal obstruction had one or more symptoms not fully relieved after surgical repair, although the choanae were widely patent. At long-term follow-up the surviving patients showed spontaneous recovery and good nasal respiration. Overall, five patients died. Since the common syndrome appeared to be related to a dysfunction of the autonomic nervous system, we conclude that any type of nasal obstruction may exacerbate or precipitate an underlying maturational autonomic disturbance.

  14. [Clinical manifestations of epilepsy in hereditary schizophrenia].

    PubMed

    Dvirskiĭ, A E; Shevtsov, A G

    1991-01-01

    A study was made of the effect of hereditary loading with schizophrenia on the clinical characteristics of epilepsy. In the population sample of epileptic patients (n = 3489), hereditary loading with schizophrenia was revealed in 4.7% of the patients, among whom loading with both schizophrenia and epilepsy was recorded in 0.6% of the cases. The clinical manifestations of epilepsy were compared in 143 patients loaded with schizophrenia and in 158 patients without hereditary loading with mental diseases. It has been established that in epileptic patients, the factor of hereditary loading with schizophrenia exerts a modifying effect on the premorbid characteristics, increasing the rate of schizoepileptoid, schizoid, and astheno-neurotic++ personality traits, and contributes to a favorable course of the disease. The data obtained standpoint of the previously advanced ++clinico-genetic concept of combined psychoses.

  15. [Uncommon clinical manifestations of cutaneous leishmaniasis].

    PubMed

    Hayani, K; Dandashli, A; Weisshaar, E

    2014-10-01

    Cutaneous leishmaniasis is one of the most common dermatoses of the tropics. A major focus of this disease is the Syrian city of Aleppo, after which it was named in many textbooks ("Aleppo boil"). The first cases of cutaneous leishmaniasis were reported from Aleppo particularly more than 100 years ago. Syria is one of the most affected countries worldwide. This disease used to be well documented until the onset of the war in Syria in 2012, which is also supported by the numbers of the World Health Organisation (WHO), and Aleppo used to be the most affected Syrian city. Since 2012, the documentation of cutaneous leishmaniasis in Syria is no longer possible. An outbreak of cutaneous leishmaniasis has been detected especially in the besieged regions due to missing prevention measures against the sandflies and a lack of medical care. A short summary of the epidemiologic situation in Syria as well as outstanding and uncommon clinical manifestations of cutaneous leishmaniasis in Aleppo are presented.

  16. [Features of the course of schizophrenia manifesting as an acute Kandinsky-Clérambault syndrome].

    PubMed

    Dikaia, V I

    1986-01-01

    A clinical follow-up study of 57 schizophrenic patients revealed heterogeneity of the clinical role of acute Kandinsky-Clerambault syndrome in the picture of the disease. The author describes the syndrome of psychic automatism in the framework of "schizophrenic reactions" in the time-course of latent schizophrenia, in the picture of the attack in shift-like and recurrent course and in the structure of the shift resembling clinically the exacerbation of the continuously progressive process. The author also shows the correlation between the premanifest period, clinical mechanisms of the development of manifest psychosis, the structure of acute Kandinsky-Clerambault syndrome and the subsequent characteristics of the schizophrenia course. The question of prognostic significance of the differential approach to the assessment of acute Kandinsky-Clerambault syndrome and of its different clinical significance in the picture of clinical diseases is discussed.

  17. Hypertonicity: Clinical entities, manifestations and treatment

    PubMed Central

    Rondon-Berrios, Helbert; Argyropoulos, Christos; Ing, Todd S; Raj, Dominic S; Malhotra, Deepak; Agaba, Emmanuel I; Rohrscheib, Mark; Khitan, Zeid J; Murata, Glen H; Shapiro, Joseph I; Tzamaloukas, Antonios H

    2017-01-01

    Hypertonicity causes severe clinical manifestations and is associated with mortality and severe short-term and long-term neurological sequelae. The main clinical syndromes of hypertonicity are hypernatremia and hyperglycemia. Hypernatremia results from relative excess of body sodium over body water. Loss of water in excess of intake, gain of sodium salts in excess of losses or a combination of the two are the main mechanisms of hypernatremia. Hypernatremia can be hypervolemic, euvolemic or hypovolemic. The management of hypernatremia addresses both a quantitative replacement of water and, if present, sodium deficit, and correction of the underlying pathophysiologic process that led to hypernatremia. Hypertonicity in hyperglycemia has two components, solute gain secondary to glucose accumulation in the extracellular compartment and water loss through hyperglycemic osmotic diuresis in excess of the losses of sodium and potassium. Differentiating between these two components of hypertonicity has major therapeutic implications because the first component will be reversed simply by normalization of serum glucose concentration while the second component will require hypotonic fluid replacement. An estimate of the magnitude of the relative water deficit secondary to osmotic diuresis is obtained by the corrected sodium concentration, which represents a calculated value of the serum sodium concentration that would result from reduction of the serum glucose concentration to a normal level. PMID:28101446

  18. Monogenic autoinflammatory diseases: concept and clinical manifestations.

    PubMed

    Almeida de Jesus, Adriana; Goldbach-Mansky, Raphaela

    2013-06-01

    The objective of this review is to describe the clinical manifestations of the growing spectrum of monogenic autoinflammatory diseases including recently described syndromes. The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary "periodic fever syndromes", familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) and 6. very rare conditions presenting with autoinflammation and immunodeficiency.

  19. Clinical manifestations of parainfluenza infection in children.

    PubMed

    Yang, Tsung-Yen; Lu, Chun-Yi; Kao, Chuan-Liang; Chen, Rong-Tsung; Ho, Yu-Huai; Yang, Shun-Cheng; Lee, Ping-Ing; Chen, Jong-Min; Lee, Chin-Yun; Huang, Li-Min

    2003-12-01

    Parainfluenza viruses are major pathogens causing respiratory illness, manifesting from mild upper respiratory tract infection to bronchiolitis and pneumonia. This retrospective study aimed at providing clinical and epidemiologic data addressing the parainfluenza virus infection in Taiwan. A total of 39 patients were enrolled in this study from March 1999 to December 2000. Infants and young children were the major susceptible population, with 87.2% of them younger than 3 years. No seasonal trend was noted for parainfluenza type 1 and type 2 infections. One clustering of parainfluenza virus type 3 infections occurred in late spring of 2000 based on collected results. Parainfluenza type 1 viral isolates accounted for all of the cases of croup. Most isolates of parainfluenza virus type 3 were associated with upper and/or lower respiratory tract infections. A substantial proportion of the patients had skin involvement; the identification of one case of possible parainfluenza virus-related erythema multiforme is particularly interesting, especially because the chances of a causal relation between viral infection and skin symptoms are formerly thought to be slight. The identification of parainfluenza virus in illnesses classically considered to be due to other viruses is intriguing and may have important implications in the management of childhood illness clinically.

  20. Clinical manifestations and management of Gaucher disease

    PubMed Central

    Linari, Silvia; Castaman, Giancarlo

    2015-01-01

    Summary Gaucher disease is a rare multi-systemic metabolic disorder caused by the inherited deficiency of the lysosomal enzyme β-glucocerebrosidase, which leads to the accumulation of its normal substrate, glucocerebroside, in tissue macrophages with damage to haematological, visceral and bone systems. Anaemia, thrombocytopenia, enlargement of liver and/or spleen, skeletal abnormalities (osteopenia, lytic lesions, pathological fractures, chronic bone pain, bone crisis, bone infarcts, osteonecrosis and skeletal deformities) are typical manifestations of the most prevalent form of the disease, the so-called non-neuronopathic type 1. However, severity and coexistence of different symptoms are highly variable. The determination of deficient β-glucocerebrosidase activity in leukocytes or fibroblasts by enzymatic assay is the gold standard for the diagnosis of Gaucher disease. Comprehensive and reproducible evaluation and monitoring of all clinically relevant aspects are fundamental for the effective management of Gaucher disease patients. Enzyme replacement therapy has been shown to be effective in reducing glucocerebroside storage burden and diminishing the deleterious effects caused by its accumulation. Tailored treatment plan for each patient should be directed to symptom relief, general improvement of quality of life, and prevention of irreversible damage. PMID:26604942

  1. Clinic manifestations in granulomatosis with polyangiitis.

    PubMed

    Greco, A; Marinelli, C; Fusconi, M; Macri, G F; Gallo, A; De Virgilio, A; Zambetti, G; de Vincentiis, M

    2016-06-01

    Granulomatosis with polyangiitis (GPA), formerly Wegener's granulomatosis (WG), is an uncommon immunologically mediated systemic small-vessel vasculitis that is pathologically characterised by an inflammatory reaction pattern (necrosis, granulomatous inflammation and vasculitis) that occurs in the upper and lower respiratory tracts and kidneys. Although the aetiology of GPA remains largely unknown, it is believed to be autoimmune in origin and triggered by environmental events on a background of genetic susceptibility.In Europe, the prevalence of GPA is five cases per 100,000 population, with greater incidence in Northern Europe. GPA can occur in all racial groups but predominantly affects Caucasians. Both sexes are affected equally. GPA affects a wide age range (age range, 8-99 years).Granulomatosis with polyangiitis is characterised by necrotising granulomatous lesions of the respiratory tract, vasculitis and glomerulonephritis. Classically, the acronym ELK is used to describe the clinical involvement of the ear, nose and throat (ENT); lungs; and kidneys. Because the upper respiratory tract is involved in 70-100% of cases of GPA, classic otorhinolaryngologic symptoms may be the first clinical manifestation of disease. The nasal cavity and the paranasal sinuses are the most common sites of involvement in the head and neck area (85-100%), whereas otological disease is found in approximately 35% (range, 19-61%) of cases.Diagnosis of GPA is achieved through clinical assessment, serological tests for anti-neutrophil cytoplasmic antibodies (ANCA) and histological analysis. The 10-year survival rate is estimated to be 40% when the kidneys are involved and 60-70% when there is no kidney involvement.The standard therapy for GPA is a combination of glucocorticoids and cyclophosphamide. In young patients, cyclophosphamide should be switched to azathioprine in the maintenance phase.A multidisciplinary approach, involving otorhinolaryngologists, oral and maxillofacial

  2. [Acute encephalitis. Neuropsychiatric manifestations as expression of influenza virus infection].

    PubMed

    Moreno-Flagge, Noris; Bayard, Vicente; Quirós, Evelia; Alonso, Tomás

    2009-01-01

    The aim is to review the encephalitis in infants and adolescents as well as its etiology, clinical manifestation, epidemiology, physiopathology, diagnostic methods and treatment, and the neuropsyquiatric signs appearing an influenza epidemy. Encephalitis is an inflammation of the central nervous system (CNS) which involves the brain. The clinical manifestations usually are: headache, fever and confusional stage. It could also be manifested as seizures, personality changes, or psiqyiatric symptoms. The clinical manifestations are related to the virus and the cell type affected in the brain. A meningitis or encephalopathy need to be ruled out. It could be present as an epidemic or isolated form, beeing this the most frequent form. It could be produced by a great variety of infections agents including virus, bacterias, fungal and parasitic. Viral causes are herpesvirus, arbovirus, rabies and enterovirus. Bacterias such as Borrelia burgdorferi, Rickettsia and Mycoplasma neumoniae. Some fungal causes are: Coccidioides immitis and Histoplasma capsulatum. More than 100 agents are related to encephalitis. The diagnosis of encephalitis is a challenge for the clinician and its infectious etiology is clear in only 40 to 70% of all cases. The diagnosis of encephalitis can be established with absolute certainty only by the microscopic examination of brain tissue. Epidemiology is related to age of the patients, geographic area, season, weather or the host immune system. Early intervention can reduce the mortality rate and sequels. We describe four patients with encephalitis and neuropsychiatric symptoms during an influenza epidemic.

  3. Electrocardiographic findings and cardiac manifestations in acute aluminum phosphide poisoning.

    PubMed

    Soltaninejad, Kambiz; Beyranvand, Mohammad-Reza; Momenzadeh, Seyed-Akbar; Shadnia, Shahin

    2012-07-01

    Aluminium phosphide (AlP) poisoning has a high mortality due to cardiovascular involvement. In this study, we evaluated the frequency of cardiac manifestations and electrocardiographic (ECG) findings in 20 patients with acute AlP poisoning, who were admitted to the intensive care unit (ICU) in Tehran, Iran, over a period of 6 months (between October 2008 and April 2009). The sex, age, cause and manner of ingestion, number of ingested AlP tablets, cardiac and ECG manifestations, creatine phosphokinase (CPK), CPK-myocardial band (CPK-mb) and troponin-T (TnT) were extracted from the patients' files. All data were analysed with Statistical Package for the Social Sciences (SPSS) software. The majority (60%) of patients were male. The mean age was 27 ± 8.7 years. The mortality rate was 40%. In all of the patients, the cause of poisoning was intentional suicide and ingestion was the route of exposure. The mean number of ingested AlP tablets per patient was 2.2 ± 1.1. The average time interval between admission and cardiovascular manifestations or ECG findings was 168.8 ± 116.2 min. The range of systolic (SBP) and diastolic blood pressure was 60-130 mmHg and 40-70 mmHg, respectively. Dysrhythmia was observed in nine (45%) cases. Elevation of the ST segment was seen in nine cases (45%). Seven patients (35%) had prolonged QTc intervals. Bundle branch block (BBB) was observed in four (20%) patients. In nine (45%) patients, the serum cardiac TnT qualitative assay was positive. There were no significant differences between normal and abnormal ECG groups according to sex, age, number and manner of ingested AlP tablets and SBP. There was a significant correlation between cardiac manifestations and ECG findings and TnT-positive results with mortality in acute AlP poisoning.

  4. Periodic eye opening and upward eye deviation accompanied by burst-suppression, as an isolated clinical manifestation of acute post-hypoxic myoclonus.

    PubMed

    Dericioglu, Nese; Arsava, Ethem Murat; Topcuoglu, Mehmet Akif

    2015-03-01

    Various movement disorders have been described following hypoxic-ischaemic brain injury. Here, we present a 72-year-old female patient who developed periodic opening and upward deviation of the eyes as an isolated clinical finding, within 24 hours after cardio-pulmonary arrest. These movements were accompanied by burst-suppression on EEG, and both clinical and electrophysiological findings were suppressed 18 hours after intravenous levetiracetam infusion. The strictly periodic nature of both EEG discharges and eye opening with vertical deviation suggest a cause due to either activation of a subcortical/brainstem pacemaker reciprocally stimulating the cortex, or, alternatively, post-anoxic burst activity of viable cortical neural networks, somehow stimulating the relevant oculomotor nuclei. Together with previous similar cases, our case expands the spectrum of post-resuscitation myoclonus syndromes with the addition of this rare isolated oculopalpebral subtype. [Published with video sequence].

  5. Esophageal Candidiasis as the Initial Manifestation of Acute Myeloid Leukemia.

    PubMed

    Komeno, Yukiko; Uryu, Hideki; Iwata, Yuko; Hatada, Yasumasa; Sakamoto, Jumpei; Iihara, Kuniko; Ryu, Tomiko

    2015-01-01

    A 47-year-old woman presented with persistent dysphagia. A gastroendoscopy revealed massive esophageal candidiasis, and oral miconazole was prescribed. Three weeks later, she returned to our hospital without symptomatic improvement. She was febrile, and blood tests showed leukocytosis (137,150 /μL, blast 85%), anemia and thrombocytopenia. She was diagnosed with acute myeloid leukemia (AML). She received chemotherapy and antimicrobial agents. During the recovery from the nadir, bilateral ocular candidiasis was detected, suggesting the presence of preceding candidemia. Thus, esophageal candidiasis can be an initial manifestation of AML. Thorough examination to detect systemic candidiasis is strongly recommended when neutropenic patients exhibit local candidiasis prior to chemotherapy.

  6. Stereotypies as a manifestation of acute hyperglycemia without ketosis.

    PubMed

    Baizabal-Carvallo, José Fidel; Ondo, William G

    2012-04-15

    Acute hyperglycemia without ketosis is recognized to induce movement disorders characterized by hemichorea, hemiballismus, or hemidystonia. A video-case of hyperkinetic movement disorder resembling stereotypies in the context of uncompensated hyperglycemia without ketosis is presented, expanding the clinical phenotype of this disorder.

  7. Clinical manifestations of hysteria: an epistemological perspective or how historical dynamics illuminate current practice.

    PubMed

    Medeiros De Bustos, Elisabeth; Galli, Sylvio; Haffen, Emmanuel; Moulin, Thierry

    2014-01-01

    Hysteria has generated the most heated debates among physicians, from antiquity to the present day. It has been long confused with neuroses and neurological pathologies such as Parkinson's disease and epilepsy, principally associated with women and sexual disorders. The clinical manifestations must first be seen in their historical context, as interpretation varies according to the time period. Recently, the Diagnostic and Statistical Manual of Mental Disorders by the American Psychiatric Association marked a break in the consensus that previously seemed to apply to the concept of hysteria and approach to the clinical manifestations. The clinical manifestations of hysteria are numerous and multifaceted, comprising 3 main classifications: paroxysms, attacks, and acute manifestations; long-lasting functional syndromes, and visceral events. Each main classification can be subdivided into several subgroups. The first main group of paroxysms, attacks, and acute manifestations includes major hysterical attacks, such as prodrome, trance and epileptic states, minor hysterical attacks such as syncope and tetany, twilight states, paroxysmal amnesia, and cataleptic attacks. The second group includes focal hysterical symptoms, paralyses, contractures and spasms, anesthesia, and sensory disorders. Visceral manifestations can be subdivided into spasms, pain, and general and trophic disorders. The diversity of the symptoms of hysteria and its changing clinical presentation calls into question the same hysterical attacks and the same symptoms, which have had only a few differences for over 2,000 years. A new definition of hysteria should be proposed, in that it is a phenomenon that is not pathological, but physiological and expressional.

  8. Acute onset anarthria without hepatic manifestation: a rare presentation of Wilson disease.

    PubMed

    Verma, Rajesh; Bhandari, Aveg; Tiwari, Navin; Chaudhari, Tejendra S

    2013-08-20

    Wilson disease (WD) is one of the few inherited but treatable disorder mainly affecting the liver and brain resulting in severe disability or death if left untreated. Hence, it is important to keep a high index of suspicion for diagnosing this clinical entity in appropriate clinical settings. The clinical presentation can be quite variable and they may present solely with neurological features sans hepatic symptoms. Such neurological manifestations usually follow subacute to chronic course. Acute onset anarthria as the heralding and predominant presenting feature has been rarely reported in the literature. We reported a case of a 12-year-old girl who presented with acute onset anarthria and dystonia of 1-month duration. On further evaluation, a diagnosis of WD was made. The patient showed partial improvement after she was started on copper chelating agents and anticholinergics.

  9. Acute onset anarthria without hepatic manifestation: a rare presentation of Wilson disease

    PubMed Central

    Verma, Rajesh; Bhandari, Aveg; Tiwari, Navin; Chaudhari, Tejendra S

    2013-01-01

    Wilson disease (WD) is one of the few inherited but treatable disorder mainly affecting the liver and brain resulting in severe disability or death if left untreated. Hence, it is important to keep a high index of suspicion for diagnosing this clinical entity in appropriate clinical settings. The clinical presentation can be quite variable and they may present solely with neurological features sans hepatic symptoms. Such neurological manifestations usually follow subacute to chronic course. Acute onset anarthria as the heralding and predominant presenting feature has been rarely reported in the literature. We reported a case of a 12-year-old girl who presented with acute onset anarthria and dystonia of 1-month duration. On further evaluation, a diagnosis of WD was made. The patient showed partial improvement after she was started on copper chelating agents and anticholinergics. PMID:23966348

  10. [Allergic vascularities with clinical skin manifestations].

    PubMed

    Puissant, A

    1981-01-01

    After describing the cutaneous and histo-pathological symptoms of allergic vasculazities, the author describes: Gougerot's tri-symptomatic disease, its differential diagnoses, and its various aetiologies. The pathology of the latter leads us to consider immune complexes, but our study does not deal with it in detail. Purpura rheumatica produces gammaglobulins A. Urticarial vasculazities may or may not be accompanied by anomalies of the complementary system; they may be transposed auto-immune disorders such as lupus erythematosus; their development is fairly unpredictable., Acute hemorrhagic oedema of the skin of nurslings also has allergic vasculazities at its basis. The author also mentions the transitory state between these different pictures, notably polyarteritis nodosa, granulomatotic pulmonary angitis of Churg and Strauss, and Wegener's granulomatosis.

  11. Clinical Manifestations Associated with Neurocysticercosis: A Systematic Review

    PubMed Central

    Carabin, Hélène; Ndimubanzi, Patrick Cyaga; Budke, Christine M.; Nguyen, Hai; Qian, Yingjun; Cowan, Linda Demetry; Stoner, Julie Ann; Rainwater, Elizabeth; Dickey, Mary

    2011-01-01

    Background The clinical manifestations of neurocysticercosis (NCC) are poorly understood. This systematic review aims to estimate the frequencies of different manifestations, complications and disabilities associated with NCC. Methods A systematic search of the literature published from January 1, 1990, to June 1, 2008, in 24 different electronic databases and 8 languages was conducted. Meta-analyses were conducted when appropriate. Results A total of 1569 documents were identified, and 21 included in the analysis. Among patients seen in neurology clinics, seizures/epilepsy were the most common manifestations (78.8%, 95%CI: 65.1%–89.7%) followed by headaches (37.9%, 95%CI: 23.3%–53.7%), focal deficits (16.0%, 95%CI: 9.7%–23.6%) and signs of increased intracranial pressure (11.7%, 95%CI: 6.0%–18.9%). All other manifestations occurred in less than 10% of symptomatic NCC patients. Only four studies reported on the mortality rate of NCC. Conclusions NCC is a pleomorphic disease linked to a range of manifestations. Although definitions of manifestations were very rarely provided, and varied from study to study, the proportion of NCC cases with seizures/epilepsy and the proportion of headaches were consistent across studies. These estimates are only applicable to patients who are ill enough to seek care in neurology clinics and likely over estimate the frequency of manifestations among all NCC cases. PMID:21629722

  12. Clinical manifestations and stages of Rett syndrome.

    PubMed

    Hagberg, Bengt

    2002-01-01

    The presentation and clinical diagnosis of Rett syndrome at various ages and stages are reviewed. In addition to the classical form, variability in phenotype between different atypical Rett forms is given. Obligatory, supportive, and differential diagnostic criteria are summarized. Long-term follow-up findings in ageing Rett women are addressed.

  13. Orofacial tuberculosis: Clinical manifestations, diagnosis and management

    PubMed Central

    Bansal, Ramta; Jain, Aditya; Mittal, Sunandan

    2015-01-01

    Orofacial tuberculosis (TB) is an uncommon form of extrapulmonary TB and is nonspecific in its clinical presentation. It can be misdiagnosed especially when oral lesions are present before systemic symptoms become apparent. Doctors especially attending dentist who generally is the first among clinicians to come across such pathological entity should be aware of the orofacial lesions of TB and consider them in the differential diagnosis of suspicious oral lesions to ensure early diagnosis of TB and its treatment. In this review, we have discussed in detail the clinical presentation of various forms of orofacial TB, diagnosis, and management of patients. Also, an update is provided about recent anti-TB drug development. PMID:26288770

  14. Immune reconstitution inflammatory syndrome Kaposi sarcoma in the liver manifesting as acute obstructive hepatitis: another potential role for montelukast?

    PubMed

    Read, P J; Lucas, S; Morris, S; Kulasegaram, R

    2013-02-01

    Immune reconstitution inflammatory syndrome has been described in Kaposi sarcoma, but does not usually manifest as acute hepatitis. We describe a case of rapid obstructive jaundice after initiation of antiretroviral therapy, in which the liver biopsy confirmed hepatic Kaposi sarcoma, and the clinical course was altered by the addition of montelukast.

  15. Diabetic neuropathy, A review of clinical manifestations.

    PubMed

    Lawrence, A M; Abraira, C

    1976-01-01

    Diabetic neuropathy in some form or other afflicts a majority of patients with diabetes mellitus. Neuropathic disturbance of sensory, motor or autonomic nerves may occur singly or in combination. Cranial nerve and other mononeuropathies generally resolve spontaneously. Autonomic neuropathy which can result in orthostatic hypotension, gastroparesis diabeticorum, nocturnal diarrhea, atonic bladder and impotence, although chronic, may wax and wane in clinical severity. Neuritis, disesthesias and painful sensory neuritis may resolve with good diabetic control; on occasion, diphenylhydantoin has been of therapeutic benefit.

  16. Equine phacoclastic uveitis: the clinical manifestations, light microscopic findings, and therapy of 7 cases.

    PubMed Central

    Grahn, B H; Cullen, C L

    2000-01-01

    This retrospective clinical study describes the clinical manifestations, light microscopic findings, and diagnosis and treatment of acute and chronic lens rupture in the horse. Rupture of the lens capsule in the horse usually results in a chronic, blinding inflammation (phacoclastic uveitis) unless prompt surgical and medical therapies are implemented. The clinical manifestations of acute lens capsule rupture included: cataract; intralenticular displacement of iridal pigment; lens cortical fragments attached to the perforated lens capsule, iris, and corneal endothelium; miosis; aqueous flare; and usually a corneal or scleral perforation with ulceration or focal full thickness corneal edema and scarring. The clinical signs of chronic phacoclastic uveitis include blindness, phthisis bulbi, and generalized corneal opacification related to scarring, vascularization, pigmentation, and edema. In one horse, acute phacoclastic uveitis was successfully treated with phacoemulsification to remove the ruptured lens and medical therapy to control the accompanying inflammation. The affected eyes of the horses with chronic phacoclastic uveitis were enucleated because of persistent clinical signs of nonulcerative keratitis and uveitis, despite long-term medical management. The clinical manifestations and lack of improvement with medical therapy are similar in the horse, dog, cat, and rabbit. However, the histologic findings in equine phacoclastic uveitis differ significantly from those in the dog, and rabbit. Images Figure 1. Figure 2. Figure 3. Figure 4. Figure 5. Figure 6. Figure 7. Figure 8. PMID:10816830

  17. Benign prostatic hyperplasia: clinical manifestations and evaluation.

    PubMed

    Santos Dias, José

    2012-12-01

    Benign prostatic hyperplasia (BPH) is a very common condition, related to aging and causing symptoms, called lower urinary tract symptoms. On account of its huge prevalence, it is important for clinicians who are involved in the management of patients with BPH to be aware of the very strict recommendations for BPH evaluation. In this article, we describe the different steps and procedures doctors should follow to evaluate these patients; symptoms and signs of BPH are reviewed, as well as the clinical evaluation steps and examinations available. The basic evaluation of the patients with BPH should include, according to the recommendations of the most relevant international guidelines, lower urinary tract symptoms evaluation with appropriate symptom scores, digital rectal examination, voiding charts, prostate-specific antigen and creatinine measurement, urinalysis, and imaging of the urinary tract.

  18. Symptomatic pineal cysts: clinical manifestations and management.

    PubMed

    Michielsen, G; Benoit, Y; Baert, E; Meire, F; Caemaert, J

    2002-03-01

    Between 1991 and 2000, seven patients presented with symptomatic pineal cysts at our hospital (6 females, 1 male). Average age was 22 years (range 4-33 years). Headache was present in 6 patients, who were subsequently operated on. A scotoma and a transient inferior visual field deficit were minor signs in two patients respectively. A Parinaud syndrome with vertical gaze paralysis was found in none. In one child, paroxysmal pupillary dilatations and contractions ('springing pupils') constituted the only signs and a conservative policy was adopted. Four patients presented with hydrocephalus and were treated by an endoscopic resection of their pineal cysts (one stereotactically, three free-hand). Two other patients presented with a prolonged history of symptoms and signs: headache alone in one, headache with discrete neurological deficits in the other. Ventricles in these two patients were not dilated and therefore an open cyst resection by infratentorial supracerebellar approach was performed. Average follow-up in the six "operated" patients was 29 months (range 12-108 months). All four patients treated by endoscopy, are symptom-free at follow-up, whereas the two who were approached by open surgery, are not. Clinical presentation, radiological evaluation and treatment modalities of pineal cysts are discussed and compared with experiences reported in the literature. It is concluded that pineal cysts in the presence of obstructive hydrocephalus are a clear indication for endoscopy with a rigid endoscope.

  19. [Clinical Manifestations of Spinal Epidural Hematoma-Stroke Mimic and Pitfalls in Diagnosis].

    PubMed

    Kuriyama, Masaru

    2017-02-01

    Clinical manifestations of spinal epidural hematoma are presented, and the cases mimicking acute ischemic stroke have been reviewed from the literature. Many reports described the cases of spinal epidural hematoma with acute hemiparesis mimicking ischemic stroke in which intravenous thrombolytic treatment with recombinant tissue plasminogen activator was considered. A correct diagnosis of acute ischemic stroke must be made within 4.5 hours from the onset of symptoms, a relatively short window period. A spinal epidural hematoma is a potentially important stroke mimic in a wide variety of conditions that mimic a stroke. The literature review and discussion will emphasize allowing the distinction between these hemiparetic presentation of spinal epidural hematoma and acute ischemic stroke. A spinal epidural hematoma should be considered in the differential diagnosis of patients with acute onset of hemiparesis when associated with neck pain and signs of Horner's syndrome and Brown-Sēquard syndrome.

  20. Fatal cardiac tamponade as the first manifestation of acute myeloid leukemia.

    PubMed

    Leptidis, John; Aloizos, Stavros; Chlorokostas, Panagiotis; Gourgiotis, Stavros

    2014-10-01

    Acute myeloid leukemia is a hemopoietic myeloid stem cell neoplasm. It is the most common acute leukemia affecting adults,and its incidence increases with age. Acute myeloid leukemia is characterized by the rapid growth of abnormal white blood cells that accumulate in the bone marrow and interfere with the production of normal blood cells. As the leukemic cells keep filling the bone marrow, symptoms of the disease started to appear: fatigue, bleeding, increased frequency of infections, and shortness of breath. Cardiac tamponade or pericardial tamponade is an acute medical condition in which the accumulation of pericardial fluid prevents the function of the heart. Signs and symptoms include Beck triad (hypotension, distended neck veins, and muffled heart sounds), paradoxus pulses, tachycardia, tachypnea, and breathlessness. Pericardial effusion and cardiac tamponade are rare and severe complications of leukemia; they often develop during the radiation therapy, chemotherapy, or infections in the course of leukemia. This study sought to assess the fatal cardiac tamponade as the first manifestation of acute myeloid leukemia (AML). We found no reports in the literature linking these 2 clinical entities. Although the patient had no signs or diagnosis of AML previously, this case was remarkable for the rapidly progressive symptoms and the fatal outcome. The pericardial effusion reaccumulated rapidly after its initial drainage; it is a possible explanation that the leukemic cells interfered with cardiac activity or that they decreased their contractility myocytes secreting a toxic essence.

  1. Characteristic manifestations of acute paint thinner-intoxicated children.

    PubMed

    Agin, Khosrow; Hassanian-Moghaddam, Hossein; Shadnia, Shahin; Rahimi, Hamid Reza

    2016-07-01

    In this study, we evaluated the acute clinical and para-clinical abnormalities arising from paint thinner (PT) poisoning in children. Data were collected from 2008 to 2013, and sourced from the medical records of PT-intoxicated children and through questionnaires. A total of 42 children were enrolled in the study. The mean age was 37.2±2.4 months. The participants ranged from10 to 96 months of age; with a median age of 3 months; and a modal age of 24 months. The sex ratio in the study was 22 males (64.9%) to 20 females (35.1%). Clinical biochemistry results of participants revealed the significant presence of alkaline phosphatase (ALP, 569.25±151.58U/L), and lactate dehydrogenase (LDH, 576.14±164.97 IU/L). Arterial blood gas (ABG) analysis was also carried out. Chest X-ray predominantly revealed right side alveolar air space consolidation. These results confirmed hepatotoxicity and pneumonia in PT-intoxicated children. The study also revealed that positive outcomes were achieved in patients with early treatment and management. In addition, the current finding confirmed the timely transfer of the victim to the poisoning center.

  2. Staphylococcus aureus Infections: Epidemiology, Pathophysiology, Clinical Manifestations, and Management

    PubMed Central

    Davis, Joshua S.; Eichenberger, Emily; Holland, Thomas L.

    2015-01-01

    SUMMARY Staphylococcus aureus is a major human pathogen that causes a wide range of clinical infections. It is a leading cause of bacteremia and infective endocarditis as well as osteoarticular, skin and soft tissue, pleuropulmonary, and device-related infections. This review comprehensively covers the epidemiology, pathophysiology, clinical manifestations, and management of each of these clinical entities. The past 2 decades have witnessed two clear shifts in the epidemiology of S. aureus infections: first, a growing number of health care-associated infections, particularly seen in infective endocarditis and prosthetic device infections, and second, an epidemic of community-associated skin and soft tissue infections driven by strains with certain virulence factors and resistance to β-lactam antibiotics. In reviewing the literature to support management strategies for these clinical manifestations, we also highlight the paucity of high-quality evidence for many key clinical questions. PMID:26016486

  3. Animal Models That Best Reproduce the Clinical Manifestations of Human Intoxication with Organophosphorus Compounds

    PubMed Central

    Pereira, Edna F. R.; Aracava, Yasco; DeTolla, Louis J.; Beecham, E. Jeffrey; Basinger, G. William; Wakayama, Edgar J.

    2014-01-01

    The translational capacity of data generated in preclinical toxicological studies is contingent upon several factors, including the appropriateness of the animal model. The primary objectives of this article are: 1) to analyze the natural history of acute and delayed signs and symptoms that develop following an acute exposure of humans to organophosphorus (OP) compounds, with an emphasis on nerve agents; 2) to identify animal models of the clinical manifestations of human exposure to OPs; and 3) to review the mechanisms that contribute to the immediate and delayed OP neurotoxicity. As discussed in this study, clinical manifestations of an acute exposure of humans to OP compounds can be faithfully reproduced in rodents and nonhuman primates. These manifestations include an acute cholinergic crisis in addition to signs of neurotoxicity that develop long after the OP exposure, particularly chronic neurologic deficits consisting of anxiety-related behavior and cognitive deficits, structural brain damage, and increased slow electroencephalographic frequencies. Because guinea pigs and nonhuman primates, like humans, have low levels of circulating carboxylesterases—the enzymes that metabolize and inactivate OP compounds—they stand out as appropriate animal models for studies of OP intoxication. These are critical points for the development of safe and effective therapeutic interventions against OP poisoning because approval of such therapies by the Food and Drug Administration is likely to rely on the Animal Efficacy Rule, which allows exclusive use of animal data as evidence of the effectiveness of a drug against pathologic conditions that cannot be ethically or feasibly tested in humans. PMID:24907067

  4. Rheumatic manifestations preceding adult acute leukemia: characteristics and implication in course and prognosis.

    PubMed

    Gur, H; Koren, V; Ehrenfeld, M; Ben-Bassat, I; Sidi, Y

    1999-03-01

    The manifestations and outcome of adult patients with acute leukemia (AL) were examined to study the characteristics of the rheumatological prodrome of AL and to find whether it is a marker of a distinct clinical and laboratory course and whether it has any prognostic implication. During a 10-year period, 8/139 (5.8%) of AL patients presented with rheumatic manifestations. The average duration of the arthritis syndrome preceding the diagnosis of AL was 3.25 months. The most common pattern of presentation was a reactive arthritis-like syndrome involving the large joints asymmetrically and associated with low back pain. Distinctive features suggesting a paraneoplastic arthritis were severe pain disproportionate to physical findings, a poor response to conventional antirheumatic treatment, and early significant osteopenia or lytic bone lesions. The epidemiological, clinical and laboratory characteristics of patients with or without rheumatic manifestations were comparable, except for fever on presentation, the presence of transient metabolic derangement following chemotherapy, and the initial average hemoglobin, hematocrit and serum uric acid values. Moreover, the initial outcome of the two groups was similar, as the early mortality rates were comparable (42.8% vs. 45% for patients with or without arthritis, respectively). In conclusion, rheumatic syndrome presentation of adult AL is uncommon, and apparently has no deleterious effects on initial prognosis. A timely diagnosis requires an increased awareness to distinctive features.

  5. Acute anterior uveitis and other extra-articular manifestations of spondyloarthritis

    PubMed Central

    Mitulescu, TC; Popescu, C; Naie, A; Predeţeanu, D; Popescu, V; Alexandrescu, C; Voinea, LM

    2015-01-01

    Background: Spondyloarthritis (SpA) is associated with an array of peripheral manifestations. Our study aims to evaluate extra-articular manifestations of SpA in a Romanian academic clinical setting and to observe their associations with different disease measures. Methods: The study was designed to note the extra-articular manifestations of SpA patients in a cross-sectional and retrospective manner. Records included demographics, inflammation markers, SpA clinical characteristics, treatment regimes, associated osteoporosis and cardiovascular morbidity. Data were assessed by using appropriate non-parametric tests. Results: A total of 126 SpA patients were included. The most common extra-articular manifestations were skin involvement in the form of psoriasis (34.1%), eye involvement in the form of acute anterior uveitis (8.7%) and dactylitis (7.2%). Compared to patients with no record of uveitis, uveitis-affected cases were more frequently males, more frequently diagnosed with ankylosing spondylitis, but less frequently dyslipidemic and diagnosed with psoriasis. Psoriasis-affected patients were older and had a higher prevalence of peripheral SpA diagnosis, but a lower prevalence of radiographic sacroiliitis. Conclusions:Acute anterior uveitis in SpA predominantly affects males with AS. This is relevant both to clinical and fundamental science, since its management requires both ophthalmology and rheumatology clinical settings. Psoriasis was associated more frequently with peripheral SpA. Abbreviations: AHT = arterial hypertension, AS = ankylosing spondylitis, ASAS = Assessment in SpondyloArthritis international Society, aSpA = axial spondyloarthritis, BASFI = Bath Ankylosing Spondylitis Functional Index, BASDAI = Bath Ankylosing Spondylitis Disease Activity Index, CRP = C-reactive protein, ESR = erythrocyte sedimentation rate, DM2 = type 2 diabetes mellitus, HLA = human leukocyte antigen, IBD = inflammatory bowel disease, MRI = magnetic resonance imaging, m

  6. The clinical experience of acute cyanide poisoning.

    PubMed

    Yen, D; Tsai, J; Wang, L M; Kao, W F; Hu, S C; Lee, C H; Deng, J F

    1995-09-01

    The authors reviewed the clinical manifestations, complications, and the prognosis affected by Lilly Cyanide Antidote in 21 victims of acute cyanide poisoning over a 10-year period. The clinical signs and symptoms in cyanide poisoning are variable. Among 21 cases, loss of consciousness (15), metabolic acidosis (14), and cardiopulmonary failure (9) were the three leading manifestations of cyanide intoxication. Anoxic encephalopathy (6) was not uncommon in the severely intoxicated victims. Diabetes insipidus (1) or clinical signs and symptoms mimicking diabetes insipidus (3) may be an ominous sign to encephalopathy victims. The major cause of fatal cyanide poisoning is the intentional ingestion of cyanide compounds as part of a suicide attempt. Decrease of arteriovenous difference of O2 partial pressure may be a clue for the suspicion of cyanide intoxication. Although the authors cannot show a statistically significant difference (P = .47) for the Lilly cyanide antidote kit in terms of improving the survival rate for victims of cyanide poisoning, the antidote kit was always mandatory in our study in the cases of severely intoxicated victims who survived. Early diagnosis, prompt, intensive therapy with antidote, and supportive care are still the golden rules for the treatment of acute cyanide poisoning, whether in the ED or on the scene.

  7. Neurological counterparts of hyponatremia: pathological mechanisms and clinical manifestations.

    PubMed

    Podestà, Manuel Alfredo; Faravelli, Irene; Cucchiari, David; Reggiani, Francesco; Oldani, Silvia; Fedeli, Carlo; Graziani, Giorgio

    2015-04-01

    Hyponatremia, defined as a serum sodium concentration <135 mEq/L, represents the most frequent electrolyte disorder in older hospitalized patients. Early recognition of hyponatremia is mandatory, since it represents an independent risk factor that increases hospital mortality by 40 %. Delayed correction of hyponatremia may worsen brain edema, resulting in different degrees of neural damage. However, an overly rapid correction of serum sodium levels can lead to osmotic demyelination syndrome (ODS), a dreadful neurological picture. In recent years, hyponatremia and ODS have received growing attention both in terms of clinical management and pathophysiology, leading to the discovery of new drugs and treatment algorithms. In this review, we recapitulate the pathogenetic background, clinical manifestations, and treatment guidelines of hyponatremia, focusing on the neurological alterations. Neurological symptoms may be neglected when they manifest as early signs of mild hyponatremia, while brain damage can irremediably affect patients' conditions in the context of ODS.

  8. Oral manifestations of psoriasis. Clinical presentation and management.

    PubMed

    Dreyer, Lois N; Brown, Gwen Cohen

    2012-04-01

    Psoriasis is a chronic immune-mediated disease of unknown etiology that affects the skin and mucous membranes. According to the National Institutes of Health (NIH), approximately five million Americans, 3% of the population, have been diagnosed with psoriasis. Oral manifestations of psoriasis are less well recognized than skin lesions, and treatment for oral lesions is not standardized. This article will review the clinical presentation of skin and mucous membrane psoriasis, along with the therapeutic modalities available to oral health-care providers.

  9. Rapidly progressing, fatal and acute promyelocytic leukaemia that initially manifested as a painful third molar: a case report

    PubMed Central

    2009-01-01

    Introduction Acute promyelocytic leukaemia, an uncommon and devastating subtype of leukaemia, is highly prevalent in Latin American populations. The disease may be detected by a dentist since oral signs are often the initial manifestation. However, despite several cases describing oral manifestations of acute promyelocytic leukaemia and genetic analysis, reports of acute promyelocytic leukaemia in Hispanic populations are scarce. The identification of third molar pain as an initial clinical manifestation is also uncommon. This is the first known case involving these particular features. Case presentation A 24-year-old Latin American man without relevant antecedents consulted a dentist for pain in his third molar. After two dental extractions, the patient experienced increased pain, poor healing, jaw enlargement and bleeding. A physical examination later revealed that the patient had pallor, jaw enlargement, ecchymoses and gingival haemorrhage. Laboratory findings showed pancytopaenia, delayed coagulation times, hypoalbuminaemia and elevated lactate dehydrogenase. Splenomegaly was detected on ultrasonography. Peripheral blood and bone marrow analyses revealed a hypercellular infiltrate of atypical promyelocytic cells. Cytogenetic analysis showing genetic translocation t(15;17) further confirmed acute promyelocytic leukaemia. Despite early chemotherapy, the patient died within one week due to intracranial bleeding secondary to disseminated intravascular coagulation. Conclusion The description of this unusual presentation of acute promyelocytic leukaemia, the diagnostic difficulties and the fatal outcome are particularly directed toward dental surgery practitioners to emphasise the importance of clinical assessment and preoperative evaluation as a minimal clinically-oriented routine. This case may also be of particular interest to haematologists, since the patient's cytogenetic analysis, clinical course and therapeutic response are well documented. PMID:19946580

  10. Acute myoedema: an unusual presenting manifestation of hypothyroid myopathy

    PubMed Central

    Bhansali, A; Chandran, V; Ramesh, J; Kashyap, A; Dash, R

    2000-01-01

    We describe a patient with primary hypothyroidism due to autoimmune thyroiditis, presenting with acute myoedema and spontaneous rhabdomyolysis. During his hospital stay, he developed altered sensorium due to hypo-osmolal hyponatraemia and later developed bilateral foot drop that responded to appropriate treatment.


Keywords: hypothyroidism; myoedema; rhabdomyolysis PMID:10644388

  11. Acute water intoxication as another unusual manifestation of child abuse.

    PubMed Central

    Mortimer, J G

    1980-01-01

    A 4 1/2-year-old boy developed hyponatraemia with coma and convulsions as the result of acute water intoxication. Information subsequently obtained strongly suggested that the excessive water ingestion was enforced by a punitive foster father. PMID:7436477

  12. [Clinical manifestations of hematological non-neoplastic diseases in Dentistry].

    PubMed

    Bascones-Martínez, Antonio; Muñoz-Corcuera, Marta; Bascones-Ilundain, Cristina

    2012-06-02

    Systemic disease can cause clinical manifestations in the oral and maxillofacial area, which is important to recognize because it could be the first symptom of an undiagnosed illness. There are different oral signs that could suggest the clinician a blood disorder, such as pallor, petechiae, ecchymosis, ulcerations, gingival hypertrophy or spontaneous gingival bleeding. In addition, blood disorders will determine the dental management of these patients and the protocol for limiting possible complications that may arise due to the treatment itself. This paper reviews the oral manifestations and dental management of non-neoplastic alterations of red cells, white cells and hemostasis, with emphasis on two-way relationship that must exist between the dentist and the patient's hematologist for making a treatment plan.

  13. Clinical manifestations and outcomes of pulmonary aspergillosis: experience from Pakistan

    PubMed Central

    Iqbal, Nousheen; Irfan, Muhammad; Zubairi, Ali Bin Sarwar; Jabeen, Kauser; Awan, Safia; Khan, Javaid A

    2016-01-01

    Introduction Pulmonary aspergillosis has variable course of illness, severity and outcomes depending on underlying conditions. There is limited data available on the clinical manifestations and outcome of pulmonary aspergillosis from Pakistan. Methods To determine the clinical manifestations and outcome of pulmonary aspergillosis in a tertiary care hospital a retrospective study was conducted from 2004 to 2014 in patients admitted with pulmonary aspergillosis at the Aga Khan University Hospital Karachi, Pakistan. Results Of the 280 cases with provisional diagnosis of aspergillosis 69 met the inclusion criteria. The mean age was 45±15.7 years, 48 (69.6%) were men and 21 (30.4%) had diabetes mellitus (DM). The average length of hospital stay (LOS) was 10.61±9.08 days. Aspergillus fumigatus was the most common (42.0%), followed by Aspergillus flavus (28.9%). More than one-third of patients previously had tuberculosis (TB) (39.13%). The commonest pulmonary manifestation was chronic pulmonary aspergillosis (CPA) 47 (68.1%) followed by invasive pulmonary aspergillosis (IPA) 12 (17.4%) and subacute invasive aspergillosis (SAIA) 8 (11.6%). Surgical excision was performed in 28 patients (40.57%). Intensive care unit admission was required for 18 patients (26.08%). Case fatality rate was 14/69 (20.3%). DM, mean LOS and hypoxic respiratory failure were identified as independent risk factors of mortality on multivariate analysis. Conclusion A. fumigatus was the most frequent species found especially in patients with prior TB. CPA was the commonest pulmonary manifestation seen as post TB sequel. Diabetes, hypoxic respiratory failure and increased LOS were independent predictors of poor outcomes. Overall patients had good outcome with CPA compared with SAIA and IPA. PMID:28074136

  14. Dentomaxillofacial manifestations of Gaucher's disease: preliminary clinical and radiographic findings

    PubMed Central

    Nobre, RM; Ribeiro, ALR; Alves-Junior, SM; Tuji, FM; Rodrigues Pinheiro, M das G; Pinheiro, LR; Pinheiro, JJV

    2012-01-01

    Objectives A wide variety of manifestations is presented in patients with Gaucher's disease (GD), including bone, haematology and visceral disturbances. This study was conducted to ascertain the main maxillofacial abnormalities by means of clinical survey, panoramic and cone beam CT (CBCT); to compare the patient's group with an age–sex matched control group; and to correlate clinical and radiological data. Methods Ten patients previously diagnosed with GD were submitted to clinical and radiological surveys (CBCT and panoramic radiographs). The examination consisted of anamnesis, extra- and intraoral examinations and analyses of each patient's records. Imaging data were collected from the point of view of 3 observers, and the results compared with a healthy group (20 individuals) by means of statistical analysis (Fisher's exact test). Results Gaucher patients had significantly more manifestations than otherwise healthy carriers. The most prevalent findings were enlarged marrow spaces, generalized osteopenia and effacement of jaw structures (mandibular canal, lamina dura and mental foramen). Here we describe a case in which thickening of the maxillary sinus mucosa was observed on CBCT rather than opacification of the sinus as seen on panoramic radiographs. Pathological fractures, root resorption and delay on tooth eruption were not observed. Conclusions A poor relationship could be observed between clinical and radiological data. Patients showed important bone manifestations, which require careful diagnostic and surgical planning whenever necessary. Although panoramic radiographs have shown significant differences, CBCT is more effective in pointing out differences between patients and a control group, thus showing it as an important tool for evaluation of Gaucher patients. PMID:22988312

  15. Acute acalculous cholecystitis in systemic lupus erythematosus: a rare initial manifestation.

    PubMed

    Manuel, Valdano; Pedro, Gertrudes Maria; Cordeiro, Lemuel Bornelli; de Miranda, Sandra Maria da Rocha Neto

    2016-01-01

    Acute acalculous cholecystitis is a very rare gastrointestinal manifestation in systemic lupus erythematosus and becomes rarer as an initial manifestation. There are only two cases reported. The authors report a 20-year-old black woman that presented acute acalculous cholecystitis revealed by abdominal computed tomography. During hospitalization, she was diagnosed systemic lupus erythematosus. Conservative treatment with antibiotics was performed with complete remission of the symptoms. Corticosteroid was started in ambulatory. Cholecystectomy has been the treatment of choice in acute acalculous cholecystitis as a complication of systemic lupus erythematosus. The patient responded well to conservative treatment, and surgery was not required. This case is unique in the way that corticosteroid was started in ambulatory care. We should not forget that the acute acalculous cholecystitis can be the initial presentation of systemic lupus erythematosus although its occurrence is very rare. Conservative treatment should be considered. Abdominal computed tomography was a determinant exam for better assessment of acute acalculous cholecystitis.

  16. Acute meningitis as an initial manifestation of Erysipelothrix rhusiopathiae endocarditis.

    PubMed

    Joo, Eun-Jeong; Kang, Cheol-In; Kim, Wook Sung; Lee, Nam Yong; Chung, Doo Ryeon; Peck, Kyong Ran; Song, Jae-Hoon

    2011-10-01

    Erysipelothrix rhusiopathiae is a gram-positive bacillus which is found worldwide. Although bloodstream infections caused by E. rhusiopathiae are not common, there is a strong association between bacteremia and the development of infective endocarditis. The risk of human infection with Erysipelothrix is closely related to the opportunity for exposure to the organisms. We report a case of community-acquired meningitis as an initial manifestation of E. rhusiopathiae endocarditis in a 56-year-old woman, who had no history of exposure to animals.

  17. [Clinical manifestations, complications and treatment of brucellosis: 45-patient study].

    PubMed

    Zribi, M; Ammari, L; Masmoudi, A; Tiouiri, H; Fendri, C

    2009-07-01

    The aim of this study was to evaluate the clinical, laboratory findings and therapeutic features of patients with brucellosis. The diagnosis was made by clinical findings, automated blood culture, serology (Rose Bengal plate agglutination test, standard tube agglutination (Wright) and immunofluorerescence). The susceptibility of 13 strains was tested in vitro. The base sequence was determined for four strains. Forty-five cases were collected (31 acute and 14 sub-acute). Contamination was digestive in 62%. Symptoms of patients were fever (93%), sweating (82%), arthralgia (78%) and splenomegaly (51%). Elevated erythrocyte sedimentation rate was determined in 80%, leukopenia in 49% and anaemia in 37% of cases. Blood cultures were positives in 39% of cases. The four sequenced strains were identified as Brucella melitensis biovar abortus. Six strains were resistant to sufomethoxazol-trimetoprim (54%). In 93% of cases, the treatment was associated rifampicin and doxycyclin. One patient died. No relapse was reported.

  18. [Petit mal status epilepticus with unusual clinical manifestations].

    PubMed

    Savitskaia, O N; Karlov, V A; Gleĭzer, M A; Pmotskaia, E E

    1980-01-01

    A unique observation of an epileptic female examined in detail in a neurological clinic is presented. The patient was later hospitalized 3 times for disturbances of consciousness and extrapyramid disorders characterized by acute onset. An encephalographic examination carried out during such a state showed an epileptic status of atypic absence. The development of the Parkinsonian syndrome is regarded as a proof of the role of the caudate nucleus in the genesis of the petit mal epileptic status.

  19. Autoimmune bullous skin diseases. Part 1: Clinical manifestations.

    PubMed

    Kneisel, Andrea; Hertl, Michael

    2011-10-01

    Autoimmune bullous skin diseases are characterized by autoantibodies against adhesion molecules of the skin. Pemphigus is a disorder with an intraepidermal loss of adhesion and is characterized by fragile blisters and erosions. Pemphigus vulgaris often shows extensive lesions of the oral mucosa, while pemphigus foliaceus is commonly restricted to cutaneous involvement with puff pastry-like scale formation. Paraneoplastic pemphigus is obligatorily associated with malignancies and often presents as hemorrhagic stomatitis with multiforme-like exanthems. IgA pemphigus typically presents with pustules and annular plaques but not with mucosal involvement. The clinical spectrum of the pemphigoids includes tense blisters, urticarial plaques, and prurigo- like eczematous lesions. Pemphigoid gestationis mostly occurs during the last trimester of pregnancy and mucous membrane pemphigoid primarily involves the oral mucosa and conjunctivae and leads to scarring. Linear IgA bullous dermatosis manifests with tense blisters in a "cluster of jewels"-like pattern in childhood and is more heterogeneous in adulthood. Classical epidermolysis bullosa acquisita shows extensive skin fragility. Dermatitis herpetiformis is associated with gluten-sensitive enteropathy and manifests clinically with severe itching and papulovesicles on the extensor surfaces of the extremities and the lumbosacral area. The intention of the review is to demonstrate the heterogeneous clinical spectrum of autoimmune bullous disorders.

  20. Myelitis and Lupus: Clinical Manifestations, Diagnosis and Treatment. Review.

    PubMed

    Chiganer, Edson Hernán; Hryb, Javier Pablo; Carnero Contentti, Edgar

    2016-08-23

    Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple systems. Myelopathy is one of 19 neuropsychiatric syndromes related to SLE defined by the American College of Rheumatology. Although infrequent, it is a severe manifestation, leading to motor and sensory deficits, and sphincter dysfunction. The pathogenesis is not clearly known, but may be related to arterial thrombosis and vasculitis. Diagnosis is based on clinical findings, laboratory tests and the use of gadolinium-enhanced magnetic resonance imaging. The standard therapy is the combination of intravenous cyclophosphamide and glucocorticoids. In refractory disease, other treatments such as plasmapheresis or rituximab have been used.

  1. [Congenital deficiency of protein C with late clinical manifestation].

    PubMed

    Ortega Calvo, M; Méndez Mora, J L; Macías Pérez, V; Mellado Fuentes, F

    1994-07-01

    The congenital deficit of protein C has a great diversity of clinical manifestations regarding age. In this paper, we describe the case of a man whose initial symptomatology appeared at the age of 68. Protein C is a vitamin-K dependent plasmatic glycoprotein which has anticoagulant activity through the inactivation of factors Va and VIIIa. The hereditary deficit of protein C is usually presented in a dominant autosomic mode with partial penetration and its prevalence is estimated in each of 200-300 healthy blood donors.

  2. Nasopharyngeal Tuberculosis: Epidemiology, Mechanism of Infection, Clinical Manifestations, and Management

    PubMed Central

    Sittitrai, Pichit

    2016-01-01

    Nasopharyngeal tuberculosis (NPTB) is a noteworthy disease especially in its worldwide spread of the Mycobacterium infection. Although NPTB has been identified in less than one percent of TB cases, recent multiple case reports indicate an either increased awareness or incidence of this disease. The most helpful diagnostic tool is an uncomplicated nasopharyngeal biopsy. However, NPTB is usually ignored because it has varied clinical manifestations and similar presentations with other more common head and neck diseases. Furthermore, the most common presenting symptom is cervical lymphadenopathy mimicking nasopharyngeal carcinoma, a more common and serious disease. Treatment outcomes of NPTB are good in both HIV-positive or HIV-negative patients. In addition, pulmonary tuberculosis association was reported in wide range between 8.3% and 82% which should be considered in a treatment program. In conclusion, early diagnosis and management in NPTB can be achieved by (1) increased awareness of this disease, (2) improvement in knowledge regarding clinical manifestations, and (3) improvement of diagnostic techniques. PMID:27034677

  3. Nasopharyngeal Tuberculosis: Epidemiology, Mechanism of Infection, Clinical Manifestations, and Management.

    PubMed

    Srivanitchapoom, Chonticha; Sittitrai, Pichit

    2016-01-01

    Nasopharyngeal tuberculosis (NPTB) is a noteworthy disease especially in its worldwide spread of the Mycobacterium infection. Although NPTB has been identified in less than one percent of TB cases, recent multiple case reports indicate an either increased awareness or incidence of this disease. The most helpful diagnostic tool is an uncomplicated nasopharyngeal biopsy. However, NPTB is usually ignored because it has varied clinical manifestations and similar presentations with other more common head and neck diseases. Furthermore, the most common presenting symptom is cervical lymphadenopathy mimicking nasopharyngeal carcinoma, a more common and serious disease. Treatment outcomes of NPTB are good in both HIV-positive or HIV-negative patients. In addition, pulmonary tuberculosis association was reported in wide range between 8.3% and 82% which should be considered in a treatment program. In conclusion, early diagnosis and management in NPTB can be achieved by (1) increased awareness of this disease, (2) improvement in knowledge regarding clinical manifestations, and (3) improvement of diagnostic techniques.

  4. [The sacroiliac joint dysfunction: clinical manifestations, diagnostics and manual therapy].

    PubMed

    Grgić, Vjekoslav

    2005-01-01

    Sacroiliac joint dysfunction is one of the proved causes of sacroiliac joint syndrome. We are talking about the restricted mobility of sacrum opposite to ilium the type of "reversible blockage of movement". Main characteristics of dysfunction are as follows: restricted "joint play", referred pain, normal radiological finding, normal lab results and disappearance of clinical symptoms after deblocking of articular bodies. Pain from a blocked joint can be referred to lower back, buttocks, hip, groin, thigh, calf and lower part of abdomen. Dispersion of painful regions is a consequence of a complex and variable innervation of articular capsule. Blocked position of articular bodies and protracted tension of articular capsule causes a stimulus of nociceptors by which a capsule is protected. Nociceptive activity is manifested with referred pains in innervational region of stimulated sensitive nerves. In the article, besides the clinical manifestations, there is described a diagnostics and manual therapy of dysfunction. Springing tests by means of which a passive mobility ("joint play") is being tested, are most valuable in dysfunction diagnostics. Manual therapy (mobilization/manipulation) is indicated and efficacious with the patients suffering from dysfunction.

  5. Moebius syndrome: clinical manifestations in a pediatric patient.

    PubMed

    Lima, Luciana Monti; Diniz, Michele Baffi; dos Santos-Pinto, Lourdes

    2009-01-01

    Moebius syndrome is a congenital, nonprogressive disorder clinically characterized by loss of facial expression, impaired stomatognathic system functions, incapacity to close the eyelids, and several oral impairments. The purpose of this paper was to present the clinical manifestations and the dental treatment in a 5-year, 2-month-old male Moebius syndrome patient. The child presented with facial asymmetry, difficulty performing facial mimic movements and pronouncing some letters, and compromised suction, mastication, breathing, and deglutition. An intraoral examination revealed hypofunction of the perioral muscles, cheeks and tongue, ankyloglossia, anterior open bite, and absence of carious lesions and dental anomalies. The dental treatment consisted of frenectomy and further placement of a removable orthodontic appliance with a palatal crib for correction of the anterior open bite. After 12 months of follow-up, anterior open bite decreased and speech, deglutition, and mastication improved.

  6. Incidence, pathophysiology, and clinical manifestations of antiphospholipid syndrome.

    PubMed

    Brock, Clifton O'neill; Brohl, Andrew Scott; Običan, Sarah Gloria

    2015-09-01

    Antiphospholipid syndrome (APLS) is a complex systemic disease with a wide variety of clinical manifestations. In the obstetric population, recurrent early pregnancy loss, fetal loss, and thrombosis are hallmarks of the disease. Patients with APLS have developed one or more pathogenic auto-antibodies directed against plasma and cell surface proteins. These antibodies are characterized by their affinity for anionic phospholipids. Interactions between APLS antibodies and their protein targets influence a wide variety of biological systems and signaling pathways, including monocytes, platelets, the complement system, and endothelial cells. While much research is currently directed at understanding the mechanisms involved in this autoimmune disease, the key clinical presentation is the hypercoagulable state resulting in thrombosis occurring in essentially any arterial or venous location, as well as numerous obstetrical complications. Treatment of APLS is generally directed at preventing thrombosis and poor pregnancy outcomes by ameliorating the hypercoagulable state.

  7. Clinical Features and Extraintestinal Manifestations of Crohn Disease in Children

    PubMed Central

    Lee, Young Ah; Chun, Peter; Hwang, Eun Ha; Mun, Sang Wook; Lee, Yeoun Joo

    2016-01-01

    Purpose The aim of this study was to investigate the clinical features and extraintestinal manifestations (EIMs) of Crohn disease (CD) in Korean pediatric patients. Methods The medical records of 73 children diagnosed with CD were retrospectively reviewed. Data regarding baseline demographic and clinical characteristics, including CD phenotype at diagnosis based on the Montreal classification, and clinical features and course of EIMs were investigated. Results Fifty-two (71.2%) of the patients were males. The mean age of the patients was 12.5 years. The mean follow-up period was 3.4 years. The disease location was ileal in 3 (4.1%) of the patients, colonic in 13 (17.8%), ileocolonic in 56 (76.7%). The clinical behavior was inflammatory in 62 (84.9%) of the patients, stricturing in 8 (11.0%), and penetrating in 3 (4.1%). Perianal abscesses or fistulas were found in 37 (50.7%) of the patients. EIMs observed during the study period were anal skin tag in 25 patients (34.2%), hypertransaminasemia in 20 (27.4%), peripheral arthritis in 2 (2.7%), erythema nodosum in 2 (2.7%), vulvitis in 1 (1.4%), uveitis in 1 (1.4%), and pulmonary thromboembolism in 1 (1.4%). Conclusion Perianal diseases and manifestations were present in more than half of Korean pediatric CD patients at diagnosis. Inspection of the anus should be mandatory in Korean children with suspicious CD, as perianal fistulas, abscesses, and anal skin tags may be the first clue to the diagnosis of CD. PMID:28090468

  8. Acute pancreatitis in juvenile systemic lupus erythematosus: a manifestation of macrophage activation syndrome?

    PubMed

    Campos, L M A; Omori, C H; Lotito, A P N; Jesus, A A; Porta, G; Silva, C A A

    2010-12-01

    Acute pancreatitis (AP) is a rare and life-threatening manifestation of juvenile systemic lupus erythematosus (JSLE). The objective of this study was to evaluate the prevalence and clinical features of AP in our JSLE population. AP was defined according to the presence of abdominal pain or vomiting associated to an increase of pancreatic enzymes and/or pancreatic radiological abnormalities. Of note, in the last 26 years, 5367 patients were followed up at our Pediatric Rheumatology Unit and 263 (4.9%) of them had JSLE diagnosis (ACR criteria). AP was observed in 4.2% (11/263) of JSLE patients. The median of age of the JSLE patients at AP diagnosis was 12.4 years (8.8-17.9). All of them had lupus disease activity at AP onset. Three patients were receiving corticosteroids before AP diagnosis. Interestingly, 10/11 JSLE patients fulfilled preliminary guidelines for macrophage activation syndrome, three of them with macrophage hemophagocytosis in bone marrow aspirate and hyperferritinemia. The hallmark of this syndrome is excessive activation and proliferation of T lymphocytes and macrophages with massive hypersecretion of proinflammatory cytokines and clinically it is characterized by the occurrence of unexplained fever, cytopenia and hyperferritinemia. AP treatment was mainly based on intravenous methylprednisolone. Four JSLE patients with AP died and two developed diabetes mellitus. In conclusion, AP was a rare and severe manifestation in active pediatric lupus. The association between AP and macrophage activation syndrome suggests that the pancreas could be a target organ of this syndrome and that pancreatic enzyme evaluation should also be carried out in all patients.

  9. Case report: cardiac tamponade resembling an acute myocardial infarction as the initial manifestation of metastatic pericardial adenocarcinoma.

    PubMed

    Scheinin, Scott A; Sosa-Herrera, Jose

    2014-01-01

    Pericardial malignancies are uncommon, usually metastatic, linked to terminal oncology patients, and rarely diagnosed premortem. A very small number of patients will develop signs and symptoms of malignant pericardial effusion as initial clinical manifestation of neoplastic disease. Among these patients, a minority will progress to a life-threatening cardiac tamponade. It is exceedingly rare for a cardiac tamponade to be the unveiling clinical manifestation of an unknown malignancy, either primary or metastatic to pericardium. We present the case of a 50-year-old male who was admitted to the emergency department with an acute myocardial infarction diagnosis that turned out to be a cardiac tamponade of unknown etiology. Further studies revealed a metastatic pericardial adenocarcinoma with secondary cardiac tamponade. We encourage considering malignancies metastatic to pericardium as probable etiology for large pericardial effusions and cardiac tamponade of unknown etiology.

  10. Epidemiology and clinical manifestation of fungal infection related to Mucormycosis in hematologic malignancies

    PubMed Central

    Noorifard, M; Sekhavati, E; Jalaei Khoo, H; Hazraty, I; Tabrizi, R

    2015-01-01

    Introduction: Mucormycosis is an opportunist fungus infection with acute and rapidly progressive nature in the hematologic malignancy patients. This study was done to investigate the prevalence and clinical manifestations of this infection among hematologic malignancies. Methodology:This cross-sectional study (descriptive-analytical) was performed while investigating medical records of 30 patients with hematologic malignancy affected by Mucormycosis in Imam Reza Hospital between 2001 and 2013. After collecting the data, it was entered in SPSS 19 Software with a provided checklist that included demographic characteristics, clinical manifestations, and it was analyzed by using descriptive (mean, frequency) and inferential (chi- square and independent -t-test) statistical methods (p-value < 0.05 was considered as statistically significant). Findings:Overall, the prevalence of Mucormycosis was 4.29 per 100 patient hematologic malignancies. The infection proportion among men and women was 72. 2, 27.6%, respectively. The maximum cases of Mucormycosis were observed among AML patients (62.1%). The most common place of involvement was lung (89.4%) and fever was the most popular sign of the infection (100%). The most considerable and effective factor in the prognosis of infection was using combined therapy of Amphotericin Band surgery (debridement) that has statistically significant correlation (p<0.05). Conclusion:Considerable prevalence and death related to Mucormycosis infection among patients of hematologic malignancy showed the importance of having strategies for its prevention and early diagnosis especially among acute leukemia patients.

  11. Rectal prolapse as initial clinical manifestation of colon cancer.

    PubMed

    Chen, C-W; Hsiao, C-W; Wu, C-C; Jao, S-W

    2008-04-01

    Rectal prolapse as the initial clinical manifestation of colorectal cancer is uncommon. We describe the case of a 75-year-old woman who was diagnosed as having adenocarcinoma of the sigmoid colon after presenting with complete rectal prolapse. The tumor caused rectosigmoid intussusception and then it prolapsed out through the anus. She underwent rectosigmoidectomy and rectopexy. The postoperative course was uneventful. The relationship between colorectal cancer and rectal prolapse has not been clearly established. This case report describes an unusual presentation of colorectal cancer. It suggests that rectal prolapse can present as the initial symptom of colorectal cancer and may also be a presenting feature of the occult intra-abdominal pathology. The importance of adequate investigation such as colonoscopy should be emphasized in patients who develop a new onset of rectal prolapse.

  12. An oral lesion as the primary clinical manifestation of sarcoidosis.

    PubMed

    Gill, I; Siddiqi, J

    2017-03-16

    An oral lesion as the first clinical presentation of sarcoidosis is extremely rare. We present the case of a 39-year-old woman who was referred to the oral and maxillofacial surgery department with a persistent asymptomatic nodular lesion in the hard palate. This was located adjacent to a grossly carious upper first molar and a provisional diagnosis of chronic periapical granuloma was made. An incisional biopsy of the lesion was carried out, which unexpectedly revealed the presence of a non-caseating granulomatous inflammatory reaction. A referral was sent to the respiratory medicine team and a diagnosis was later confirmed of stage II sarcoidosis. This case report highlights the need for clinicians to be aware of all possible causes of oral lesions, including rare manifestations of underlying systemic disease.

  13. Pediatric carbamazepine suspension overdose-clinical manifestations and toxicokinetics.

    PubMed

    Perez, Alberto; Wiley, James F

    2005-04-01

    Two toddlers ingested unknown quantities of their older sibling's carbamazepine suspension and rapidly manifested central nervous depression requiring intubation in 1 patient. Coma was the primary clinical finding throughout their care with no anticholinergic syndrome, seizures, or dysrhythmia. Both patients recovered without sequelae within 24 hours. Initial carbamazepine concentrations were 36.6 and 22.7 mg/L. The elimination rates (zero-order kinetic) were approximately 1.4 and 0.75 mg/L per hour. We provide the first toxicokinetic data for carbamazepine suspension overdose in children. We confirm that the oral absorption of suspension carbamazepine is rapid necessitating prompt referral to a health care facility for this exposure.

  14. ["Flash" pulmonary edema as a clinical manifestation of renovascular hypertension].

    PubMed

    Kalimonovska-Ostrić, Dimitra; Ivanović, Branislava; Ostrić, Vladimir; Knezivić, Vesna; Stojanov, Vesna; Simić, Dragan

    2003-01-01

    One of the clinical manifestations of renovascular hypertension (RVH) may be a recurrent pulmonary oedema both in the absence or in the presence of systolic left ventricular dysfunction. This type of pulmonary oedema characterized as "flash" pulmonary oedema is ascribed to elevated angiotensin II concentrations with consequent hypertension as well as to volume overload resulting from decreased pressor natriuresis when there are significant stenoses of both or one renal arteries. The investigation included 30 patients with RVH treated by percutaneous transluminal angioplasty of the stenosed renal artery (PTRA) and/or stent implantation (PTR-ST) and 30 patients with surgical resection of the abdominal aortic aneurysm (AAA). The first group was divided in two subgroups according to the etiology of renal artery stenosis (RAS). In the subgroup with fibromuscular dysplasia (FMD) the mean age was 37.5 years, in the subgroup with atherosclerotic renal artery stenosis (ARAS) 54.8 years and in the group with operated AAA 68.6 years. There were more females than males only in the FMD subgroup (10:3). Two patients of the first group experienced pulmonary oedema, both in the subgroup with atherosclerotic renal artery stenosis associated with atherosclerosis of other arteries. Normalization of the blood pressure following PTRA in both and an uncomplicated course after a surgical myocardial revascularization in one of them illustrates the importance of renal revascularization. Pulmonary oedema occurred preoperatively in four out of 30 patients with abdominal aortic aneurysm in whom significant renal artery stenoses coexisted. Two patients died despite surgery, one patient is clinically stable and the medicament treatment of heart failure is inevitable in the fourth with a left ventricular aneurysm following myocardial infarction. The occurrence or recurrence of pulmonary oedema in the absence of other explanation should suggest the possibility of bilateral or unilateral renal

  15. Severe neurologic manifestations in acute intermittent porphyria developed after spine surgery under general anesthesia: a case report

    PubMed Central

    Park, Eun Young; Kim, Yi Seul; Lim, Kyung-Jee; Lee, Hye Kyoung; Lee, Soo Kyung; Choi, Hyun

    2014-01-01

    Porphyrias are inherited metabolic disorders resulting from a specific enzyme defect in the heme biosynthetic pathway. Porphyrias are induced by various precipitants. Clinical features include abdominal pain, neurologic manifestations, autonomic neuropathy, and mental disturbance. Diagnosis may be delayed because of variable symptoms that mimic other diseases and because of the rarity of of porphyrias. Although most patients with known porphyria can complete anesthesia and surgery safely, undiagnosed porphyric patients are in danger of porphyric crisis due to inadvertent exposure to precipitating drugs and environment. We report a case of a patient who experienced delayed emergence with neurological disturbance after general anesthesia, ultimately diagnosed as acute intermittent porphyria. PMID:25302100

  16. Epidemiology and Clinical Manifestations of Enteroaggregative Escherichia coli

    PubMed Central

    Hebbelstrup Jensen, Betina; Olsen, Katharina E. P.; Struve, Carsten; Petersen, Andreas Munk

    2014-01-01

    SUMMARY Enteroaggregative Escherichia coli (EAEC) represents a heterogeneous group of E. coli strains. The pathogenicity and clinical relevance of these bacteria are still controversial. In this review, we describe the clinical significance of EAEC regarding patterns of infection in humans, transmission, reservoirs, and symptoms. Manifestations associated with EAEC infection include watery diarrhea, mucoid diarrhea, low-grade fever, nausea, tenesmus, and borborygmi. In early studies, EAEC was considered to be an opportunistic pathogen associated with diarrhea in HIV patients and in malnourished children in developing countries. In recent studies, associations with traveler's diarrhea, the occurrence of diarrhea cases in industrialized countries, and outbreaks of diarrhea in Europe and Asia have been reported. In the spring of 2011, a large outbreak of hemolytic-uremic syndrome (HUS) and hemorrhagic colitis occurred in Germany due to an EAEC O104:H4 strain, causing 54 deaths and 855 cases of HUS. This strain produces the potent Shiga toxin along with the aggregative fimbriae. An outbreak of urinary tract infection associated with EAEC in Copenhagen, Denmark, occurred in 1991; this involved extensive production of biofilm, an important characteristic of the pathogenicity of EAEC. However, the heterogeneity of EAEC continues to complicate diagnostics and also our understanding of pathogenicity. PMID:24982324

  17. Superwarfarin intoxication: hematuria is a major clinical manifestation.

    PubMed

    Wu, Yi-Feng; Chang, Cheng-Shyong; Chung, Chih-Yuan; Lin, Hsuan-Yu; Wang, Chuan-Cheng; Shen, Ming-Ching

    2009-09-01

    Since superwarfarin is popular and readily available in stores, it may cause intoxication or overexposure, which can result in coagulopathy or abnormal bleeding in humans and, thus, is an important public health problem. We report our clinical experience with superwarfarin intoxication. Nine patients, including eight patients who had histories of ingesting superwarfarin, were studied. Of the patients, hematuria occurred in eight. Laboratory tests among the nine patients showed extremely prolonged prothrombin times and activated partial thromboplastin times, which could be corrected to normal by mixing 1:1 with normal pooled plasma; they also had very low functional levels of factor II, VII, IX, X, and proteins C and S, but normal functional levels of factors V, VIII, fibrinogen, and anti-thrombin III. Large doses of vitamin K1 were needed for 3 months or more to treat and correct the coagulopathy among the patients. The majority of the patients presented with gross hematuria, suggesting that hematuria is probably a major clinical manifestation of superwarfarin intoxication. Prolonged use of large doses of vitamin K1 is needed for the treatment of superwarfarin intoxication.

  18. IgG4-related disease manifesting as an acute gastric-pericardial fistula.

    PubMed

    Frydman, James; Grunner, Shahar; Kluger, Yoram

    2014-11-28

    IgG4-related disease is a recently recognized entity linked initially to autoimmune pancreatitis and has been subsequently described in nearly every organ system. Men over the age of 50 represent the most affected demographic group and a comprehensive set of diagnostic criteria has been developed to aid treating clinicians. Though elevated levels of IgG4 in the serum are suggestive of the disease, definitive diagnosis is made on histopathology. Treatment is tailored to the clinical presentation with corticosteroid therapy known to have proven efficacy. Gastric manifestations of the IgG4-related disease primarily come in two varieties, notably chronic ulceration or pseudotumor formation. Autoimmune pancreatitis conveys increased risk for IgG4-related disease of the stomach, which is independent of Helicobacter pylori status. In this case report, we present an acute gastric-pericardial fistula secondary to IgG4-related disease that required urgent operative management. To our knowledge, this is the first report in the medical literature describing this complication of IgG4-related disease.

  19. Clinical Manifestations in 82 Neurobrucellosis Patients from Kosovo

    PubMed Central

    Dreshaj, Shemsedin; Shala, Nexhmedin; Dreshaj, Gresa; Ramadani, Naser; Ponosheci, Albina

    2016-01-01

    Background: Central nervous system involvement is a serious complication of brucellosis with various incidence and various clinical presentations. Patients and Methods: Hospitalized patients in University Clinical Centre, Clinic for Infectious diseases in Prishtina, with laboratory-confirmed brucellosis, were analyzed, a brucellosis-endemic region. Among the 648 confirmed cases with brucellosis during the period 1991- 2013, 82 patients (12.65%) were diagnosed with neurobrucellosis. The clinical manifestations in patients with neurobrucellosis were evaluated and compared with brucellosis patients. Results: The major presentations among the brucellosis patients were headache, fever, sweating, nausea, weight loss and arthralgia, while from CNS predominant complains were: headache, vomiting, tremor, low back pain, hearing loss and visual disturbance. The mean age of 82 neurobrucellosis patients was 31.46 years with age distribution 12-71 years, from them 5 (6.1%), younger than 16 years, with a non-significant predominance of women (53.65%). The most common neurological findings were radiculopathies of legs (41.46%) neck rigidity (46.34%), agitation (25,6%), behavioral disorders (18.3%), disorientation (19.5%) and stroke (1.22%). Cranial nerves were involved in 20 of 82 patients (24.4%). Neurological consequences were evidenced in 5 (6.1%) patients. Three patients leave hospital with consequences of peripheral facial paresis, two with sensorineural hearing loss and one with left hemiparesis. Headache, nausea and vomiting and weight loss are significantly (p<0.001) more frequent complains in neurobrucellosis patients compare to patients with brucellosis. On the other hand, as regard to the physical findings and complications, meningeal signs and splenomegaly are significantly more frequent in neurobrucellosis (p<0.01) whereas the hepatomegaly and lymphadenopathy were more frequent (p<0.01) in brucellosis patients. Different significant correlations were observed among

  20. Severe congenital thrombocytopaenia--first clinical manifestation of Noonan syndrome.

    PubMed

    Nunes, Paula; Aguilar, Sara; Prado, Sara Noéme; Palaré, Maria João; Ferrão, Anabela; Morais, Anabela

    2012-03-20

    This report focuses on a male infant, the first born of non-consanguineous parents diagnosed with polyhydramnios at 26 weeks of gestation. The newborn was admitted during the neonatal period with bleeding diathesis associated with a low platelet count at birth (5×10(9)/l).The authors registered a persistent low platelet count (9000-129 000/l) during the infants 1st year of life. Physical examination revealed a petechial rash, a dysmorphic face and bilateral cryptorchidism, in the absence of organomegaly. Additionally, cardiologic evaluation revealed an aortic valve dysplasia and an atrial septal defect, while bone marrow biopsy and aspiration were found normal. Throughout the investigation, the authors excluded congenital infection, alloimmune and familiar thrombocytopaenia, Fanconi anaemia and thrombocytopaenia absent radius syndrome. The cytogenetic analysis revealed a mutation in the PTPN11 gene associated with Noonan syndrome. Here the author highlights that severe neonatal thrombocytopaenia is a manifestation that should be considered in the diagnosis and clinical management of Noonan's syndrome.

  1. Severe congenital thrombocytopaenia – first clinical manifestation of Noonan syndrome

    PubMed Central

    Nunes, Paula; Aguilar, Sara; Prado, Sara Noéme; Palaré, Maria João; Ferrão, Anabela; Morais, Anabela

    2012-01-01

    This report focuses on a male infant, the first born of non-consanguineous parents diagnosed with polyhydramnios at 26 weeks of gestation. The newborn was admitted during the neonatal period with bleeding diathesis associated with a low platelet count at birth (5×109/l).The authors registered a persistent low platelet count (9000–129 000/l) during the infants 1st year of life. Physical examination revealed a petechial rash, a dysmorphic face and bilateral cryptorchidism, in the absence of organomegaly. Additionally, cardiologic evaluation revealed an aortic valve dysplasia and an atrial septal defect, while bone marrow biopsy and aspiration were found normal. Throughout the investigation, the authors excluded congenital infection, alloimmune and familiar thrombocytopaenia, Fanconi anaemia and thrombocytopaenia absent radius syndrome. The cytogenetic analysis revealed a mutation in the PTPN11 gene associated with Noonan syndrome. Here the author highlights that severe neonatal thrombocytopaenia is a manifestation that should be considered in the diagnosis and clinical management of Noonan’s syndrome. PMID:22605701

  2. Arrhythmogenic right ventricular cardiomyopathy, clinical manifestations, and diagnosis.

    PubMed

    Haugaa, Kristina H; Haland, Trine F; Leren, Ida S; Saberniak, Jørg; Edvardsen, Thor

    2016-07-01

    This review aims to give an update on the pathogenesis, clinical manifestations, and diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC). Arrhythmogenic right ventricular cardiomyopathy is mainly an autosomal dominant inherited disease linked to mutations in genes encoding desmosomes or desmosome-related proteins. Classic symptoms include palpitations, cardiac syncope, and aborted cardiac arrest due to ventricular arrhythmias. Heart failure may develop in later stages. Diagnosis is based on the presence of major and minor criteria from the Task Force Criteria revised in 2010 (TFC 2010), which includes evaluation of findings from six different diagnostic categories. Based on this, patients are classified as having possible, borderline, or definite ARVC. Imaging is important in ARVC diagnosis, including both echocardiography and cardiac magnetic resonance imaging for detecting structural and functional abnormalities, but importantly these findings may occur after electrical alterations and ventricular arrhythmias. Electrocardiograms (ECGs) and signal-averaged ECGs are analysed for depolarization and repolarization abnormalities, including T-wave inversions as the most common ECG alteration. Ventricular arrhythmias are common in ARVC and are considered a major diagnostic criterion if originating from the RV inferior wall or apex. Family history of ARVC and detection of an ARVC-related mutation are included in the TFC 2010 and emphasize the importance of family screening. Electrophysiological studies are not included in the diagnostic criteria, but may be important for differential diagnosis including RV outflow tract tachycardia. Further differential diagnoses include sarcoidosis, congenital abnormalities, myocarditis, pulmonary hypertension, dilated cardiomyopathy, and athletic cardiac adaptation, which may mimic ARVC.

  3. Clinical manifestations and oral findings in Fraser syndrome.

    PubMed

    Diniz, Michele Baffi; Lima, Luciana Monti; Sacono, Nancy Tomoko; de Paula, Andréia Bolzan; dos Santos-Pinto, Lourdes

    2007-01-01

    This article is the first known case report of Fraser syndrome in the dental literature. Its purpose was to present the clinical manifestations, oral findings, and dental treatment of a 14-year, 10-month-old female patient. Fraser syndrome is a rare recessive autosomal genetic disorder characterized by multisystemic malformation, usually comprising cryptophthalmos, syndactyly, and renal defects. The child presented with: (1) hydrocephaly; (2) face asymmetry; (3) low-inserted ears; (4) flat nose bridge; (5) cryptophthalmos; (6) bilateral absence of eyeballs; (7) hypertelorism; (8) syndactyly on the left fingers and toes; (9) skeletal defects; and (10) lower limb asymmetry. The intraoral examination revealed: (1) complete primary denture; (2) malocclusion; (3) tooth crowding; (4) ogival palate; (5) normal labial frena; (6) absence of lingual frenum (not compromising the tongue movements); (7) parched lips; (8) supragingival calculus adhered to all tooth surfaces; and (9) moderate gingivitis. The dental treatment consisted of periodic monitoring of the patient's oral health status and supragingival scaling associated with topical applications of 0.12% chlorhexidine digluconate gel at 2-week intervals to reduce gingivitis.

  4. Serotype-specific differences in clinical manifestations of dengue.

    PubMed

    Balmaseda, Angel; Hammond, Samantha N; Pérez, Leonel; Tellez, Yolanda; Saborío, Saira Indira; Mercado, Juan Carlos; Cuadra, Ricardo; Rocha, Julio; Pérez, Maria Angeles; Silva, Sheyla; Rocha, Crisanta; Harris, Eva

    2006-03-01

    Dengue, the most prevalent arthropod-borne viral disease of humans, is caused by four serotypes of dengue virus (DENV 1-4). Although all four DENV serotypes cause a range of illness, defining precisely which clinical characteristics are associated with the distinct serotypes has been elusive. A cross-sectional study was conducted on 984 and 313 hospitalized children with confirmed DENV infections during two time periods, respectively, in the same hospitals in Nicaragua: a 3-year period (1999-2001) when DENV-2 accounted for 96% of the viruses identified, and the 2003 dengue season when DENV-1 predominated (87% of identified serotypes). When the two periods were compared, more shock (OR 1.91, 95% CI 1.35-2.71) and internal hemorrhage (OR 2.05, CI 1.16-3.78) were observed in the period when DENV-2 predominated, whereas increased vascular permeability was associated to a greater degree with the DENV-1 period (OR 2.36, CI 1.80-3.09). Compared with the DENV-2 period, the DENV-1 season was associated with more hospitalized primary dengue cases (OR 3.86, CI 2.72-5.48) and more primary DENV infections with severe manifestations (OR 2.93, CI 2.00-4.28). These findings provide new data to characterize the pathogenic potential of distinct DENV serotypes in human populations.

  5. Acute seronegative hepatitis C manifesting itself as adult giant cell hepatitis--a case report and review of literature.

    PubMed

    Kryczka, Wiesław; Walewska-Zielecka, Bozena; Dutkiewicz, Ewa

    2003-08-01

    Adult giant cell hepatitis (AGCH) is a rare event and only about 100 cases have been reported within the last 20 years. The AGCH has been observed in association with viral infection, drug reactions or autoimmune disorders but in many cases its etiology remains unclear. AGCH manifests clinically as severe form of hepatitis histologically characterized by diffuse giant cell transformation of hepatocytes. We report the case of a 39-yr-old man with acute community-acquired hepatitis without previous pathology of the liver. Laboratory data revealed slight hypergammaglobulinemia and high titer of anti-smooth-muscle antibody with negative serology of hepatotropic viruses and absence of other known causes of hepatitis. Preliminary diagnosis of autoimmune hepatitis was established, additionally confirmed by excellent clinical and biochemical improvement during corticosteroid treatment. A liver biopsy showed the typical findings of panlobular syncytial giant cell hepatitis and positive HCV-RNA both in serum and liver. The above verified the diagnosis of acute type C hepatitis manifested histologically as adult giant cell hepatitis. After three months of treatment we withdrew corticosteroids as spontaneous clearance of HCV occurred and the lack of autoantibodies in serum as well as significant improvement of liver histology was ascertained. Within 30 months of the follow-up we have not observed biochemical and immunological abnormalities and control liver biopsy has shown no signs of hepatitis.

  6. Oral manifestations of coeliac disease.: A clinical-statistic study.

    PubMed

    Costacurta, M; Maturo, P; Bartolino, M; Docimo, R

    2010-01-01

    AIM.: The aim of the clinical-statistic study was to evaluate the prevalence of the different oral manifestations in a sample of coeliac patients, in comparison with a control group of healthy subjects. Moreover, a second objective was to determine if the clinical oral examination is useful as a diagnostic tool of screening for atypical forms of coeliac disease (CD). METHODS.: The enrolment of 300 coeliac patients, aged between 4 and 13 years (mean age 8.16), was carried out at the Pediatric Dentistry Unit in patients sent from the Pediatric Gastroenterology Unit of the PTV Hospital, University of Rome Tor Vergata. The control group was composed of 300 healthy subjects, age-matched (mean age 8.29), enrolled from the Pediatric Dentistry Unit. The patients were examined for hard tissues (enamel hypoplasia, dental caries), soft tissues (recurrent aphthous stomatitis RAS, atrophic glossitis, geographic tongue) and delay dental eruption. Enamel defects were classified according to Aine's criteria, while dental caries was recorded as dmft/DMFT indices. Statistical analysis was carried out by using SPSS/PC+ Software. Differences between case and control groups were tested using Paired samples T-test, and Chi-Square Test, depending on the variable considered. The minimal level of significance of the differences was fixed at p≤0.05 for all the procedures. RESULTS.: Statistical differences between groups were observed for the prevalence of enamel defects (p=0.0001), RAS (p=0.005), delay in dental eruption (p=0.0001), but not for the prevalence of atrophic glossitis (p=0.664). Differences in symmetrical distribution and a chronologic coherence of enamel defects were statistically significant between CD and control groups (p=0.0001). Regarding dental caries, the coeliac patients had higher indexes of caries than healthy subjects, both in deciduous teeth (dmft 2.31±1.84 vs 1.42±1.13; p= 0.021) and permanent teeth (DMFT 2.97±1.74 vs 1.74±1.64; p=0.0001). CONCLUSIONS.: The

  7. Craniofacial muscle pain: review of mechanisms and clinical manifestations.

    PubMed

    Svensson, P; Graven-Nielsen, T

    2001-01-01

    Epidemiologic surveys of temporomandibular disorders (TMD) have demonstrated that a considerable proportion of the population--up to 5% or 6%--will experience persistent pain severe enough to seek treatment. Unfortunately, the current diagnostic classification of craniofacial muscle pain is based on descriptions of signs and symptoms rather than on knowledge of pain mechanisms. Furthermore, the pathophysiology and etiology of craniofacial muscle pain are not known in sufficient detail to allow causal treatment. Many hypotheses have been proposed to explain cause-effect relationships; however, it is still uncertain what may be the cause of muscle pain and what is the effect of muscle pain. This article reviews the literature in which craniofacial muscle pain has been induced by experimental techniques in animals and human volunteers and in which the effects on somatosensory and motor function have been assessed under standardized conditions. This information is compared to the clinical correlates, which can be derived from the numerous cross-sectional studies in patients with craniofacial muscle pain. The experimental literature clearly indicates that muscle pain has significant effects on both somatosensory and craniofacial motor function. Typical somatosensory manifestations of experimental muscle pain are referred pain and increased sensitivity of homotopic areas. The craniofacial motor function is inhibited mainly during experimental muscle pain, but phase-dependent excitation is also found during mastication to reduce the amplitude and velocity of jaw movements. The underlying neurobiologic mechanisms probably involve varying combinations of sensitization of peripheral afferents, hyperexcitability of central neurons, and imbalance in descending pain modulatory systems. Reflex circuits in the brain stem seem important for the adjustment of sensorimotor function in the presence of craniofacial pain. Changes in somatosensory and motor function may therefore be

  8. History, Epidemiology, and Clinical Manifestations of Zika: A Systematic Review

    PubMed Central

    Barreto, Florisneide; da Glória Teixeira, Maria; da Conceição N. Costa, Maria; Rodrigues, Laura C.

    2016-01-01

    Objectives. To describe salient epidemiological characteristics of Zika virus outbreaks across the world and to examine the clinical presentations, complications, and atypical manifestations related to their occurrence in recent history. Methods. We conducted a systematic review of the literature by searching through MEDLINE, Embase, and Global Health Library, as well as the epidemiological bulletins and alerts from the World Health Organization, the Pan American Health Organization, and the European Centre for Disease Prevention and Control over the period 1954 to 2016. Results. The search yielded 547 records. We retained 333 for further analysis, to which we added 11 epidemiological bulletins from various sources. Of these, we systematically reviewed 52 articles and reports, revealing some epidemiological features and patterns of spread of the Zika virus worldwide, as well as pathological outcomes suspected to be linked to Zika outbreaks. Neurologic disorders among zika patients were similar in Brazil and French Polynesia but a causal link is not established. Incidence of zika infection in pregnant women is not known. In Brazil, during the zika outbreak the incidence of microcephaly increased more than 20 times. Among 35 infants with microcephaly, born from women suspected to have Zika infection during pregnancy in northeast Brazil, 74% of the mothers reported rash during the first and second trimester. Conclusions. On February 1, 2016, The World Health Organization declared the ongoing Zika crisis an emergency and that, although not yet scientifically proven, the link between the virus and growing numbers of microcephaly cases was “strongly suspected.” However, the causal relationship between zika and microcephaly is not universally accepted. Public Health Implications. The current situation with regard to Zika is not encouraging, because there is no vaccine, no treatment, and no good serological test, and vector control remains a challenge. PMID:26959260

  9. Intrauterine Tuberculosis Manifesting as Acute Chorioamnionitis: A Case Report and Review of the Literature.

    PubMed

    Taweevisit, Mana; Nisagornsen, Charuwan; Thorner, Paul Scott

    2015-01-01

    Congenital tuberculosis involving the placenta is an infrequent diagnosis, and the typical features of tuberculous placentitis involve a granulomatous reaction, reflecting a delayed hypersensitivity immune response in the host. However, the first reaction of the placenta to organisms hematogenously transmitted from the maternal circulation typically involves the innate immune response, manifesting as an acute neutrophilic villitis or intervillositis or both. This acute pattern of response to mycobacteria has only been documented rarely. We present a case of acute mycobacterial infection occurring in a preterm female with 28-weeks gestation, who was delivered by cesarean section because of fetal distress and who was subsequently confirmed to have congenital tuberculosis. The placenta showed an acute chorioamnionitis associated with acid-fast bacilli consistent with Mycobacteria tuberculosis. The mother was found to have a necrotizing granulomatous deciduitis, and that was postulated to have resulted in the direct spread of mycobacteria to the amniotic cavity. Thus, our case extends the acute placental response to mycobacteria to include chorioamnionitis. Although extremely rare, mycobacteria should be considered in the differential diagnosis of infectious agents causing acute chorioamnionitis, especially in geographic areas where tuberculosis is more prevalent.

  10. Purpura fulminans as clinical manifestation of atypical SLE with antiphospholipid antibodies: a case report.

    PubMed

    Gamba, G; Montani, N; Montecucco, C M; Caporali, R; Ascari, E

    1991-01-01

    Purpura Fulminans and DIC were the main clinical manifestations of the antiphospholipid syndrome observed in a 62-year-old man. The patient was well until 44 years of age when he began to suffer from recurrent thrombophlebitis, without other symptoms suggestive of immune disease. At the time of hospital admission the pt. appeared acutely ill, showing high fever, severe anemia, massive urinary blood loss, multiple purpuric patches evolving to hemorrhagic bullae and gangrene rapidly spreading over about 30% of the total body area. No signs of neurological involvement or of visceral thrombotic occlusions were present. Clotting tests were consistent with a diagnosis of DIC, further confirmed by skin biopsy showing the presence of thrombi in dermal arterioles. The autoantibody research was positive as follows: Waaler-Rose 1:40, Anti-DNA 1:80; ANF 1:640, aCA IgG 100 GPL. LA was diagnosed according to standard criteria: prolonged KCT and RVVT not corrected by a mixture of normal plasma and abnormal TTI. Plasma exchange in association with heparin and prednisone was effective in arresting the progression of the skin lesion; nevertheless the patient died ten days after hospital admission for sepsi and acute renal failure.

  11. Cutaneous myeloid sarcoma: natural history and biology of an uncommon manifestation of acute myeloid leukemia.

    PubMed

    Hurley, M Yadira; Ghahramani, Grant K; Frisch, Stephanie; Armbrecht, Eric S; Lind, Anne C; Nguyen, Tudung T; Hassan, Anjum; Kreisel, Friederike H; Frater, John L

    2013-05-01

    We conducted a retrospective study of patients with cutaneous myeloid sarcoma, from 2 tertiary care institutions. Eighty-three patients presented, with a mean age of 52 years. Diagnosis of myeloid sarcoma in the skin was difficult due to the low frequency of myeloperoxidase and/or CD34+ cases (56% and 19% of tested cases, respectively). Seventy-one of the 83 patients (86%) had ≥ 1 bone marrow biopsy. Twenty-eight (39%) had acute myeloid leukemia with monocytic differentiation. Twenty-three had other de novo acute myeloid leukemia subtypes. Thirteen patients had other myeloid neoplasms, of which 4 ultimately progressed to an acute myeloid leukemia. Seven had no bone marrow malignancy. Ninety-eight percent of the patients received chemotherapy, and approximately 89% died of causes related to their disease. Cutaneous myeloid sarcoma in most cases represents an aggressive manifestation of acute myeloid leukemia. Diagnosis can be challenging due to lack of myeloblast-associated antigen expression in many cases, and difficulty in distinguishing monocyte-lineage blasts from neoplastic and non-neoplastic mature monocytes.

  12. Various Manifestations of Hyperthyroidism in an Ambulatory Clinic: Case Studies

    PubMed Central

    Tripp, Warren; Rao, Vijaya; Creary, Ludlow B.

    1987-01-01

    This study reviews five cases of women with hyperthyroidism, three black women and two Hispanic women. Initially, two patients presented with voice changes, weight loss, and increased appetite. Only two patients presented with classical symptoms of hyperthyroidism. Examination showed all patients had diffusely enlarged thyroids and exaggerated reflexes. Two patients showed Graves' opthalmopathy. These cases document the variety of presentations of hyperthyroidism. Hence, a high index of suspicion must exist for this disease, even in the absence of a number of the classical manifestations of hyperthyroidism. When patients present to primary care centers with a constellation of symptoms, an examination of the thyroid gland is essential. PMID:3694696

  13. Spontaneous rupture of pyometra manifesting as an acute abdomen: a case report.

    PubMed

    Singh, Alpana; Mundhra, Rajlaxmi; Agarwal, Tannavi; Radhakrishnan, Gita

    2015-07-01

    Spontaneous perforation of pyometra is a rare entity with a reported incidence in the range of 0.01-0.05%. The clinical picture is similar to peritonitis arising from intestinal perforation and commonly the correct diagnosis is only made perioperatively. We report a case in an elderly postmenopausal woman presenting with an acute abdomen.

  14. Manifestation of Latent Left Ventricular Outflow Tract Obstruction in the Acute Phase of Takotsubo Cardiomyopathy

    PubMed Central

    Ozaki, Kazuyuki; Okubo, Takeshi; Tanaka, Komei; Hosaka, Yukio; Tsuchida, Keiichi; Takahashi, Kazuyoshi; Oda, Hirotaka; Minamino, Tohru

    2016-01-01

    Objective Left ventricular outflow tract (LVOT) obstruction is a complication in 15-25% of patients with Takotsubo cardiomyopathy and sometimes leads to catastrophic outcomes, such as cardiogenic shock or cardiac rupture. However, the underlying mechanisms have not been clarified. Methods and Results We experienced 22 cases of Takotsubo cardiomyopathy during 3 years, and 4 of these 22 cases were complicated with LVOT obstruction in the acute phase (mean age 79±5 years, 1 man, 21 women). The LVOT pressure gradient in the acute phase was 100±17 mmHg. Transthoracic echocardiogram (TTE) revealed left ventricular hypertrophy (LVH) in one case and sigmoid-shaped septum without LVH in three cases. The complete resolution of the LVOT obstruction was achieved in a few days with normalization of the left ventricular wall motion following administration of beta-blockers. A dobutamine provocation test after normalization of the left ventricular wall motion reproduced the LVOT obstruction in all cases and revealed the presence of latent LVOT obstruction. Conclusion The manifestation of latent LVOT obstruction in the acute phase of Takotsubo cardiomyopathy is one potential reason for the complication of LVOT obstruction with Takotsubo cardiomyopathy. PMID:27904102

  15. Post-stroke Movement Disorders: Clinical Manifestations and Pharmacological Management

    PubMed Central

    Siniscalchi, Antonio; Gallelli, Luca; Labate, Angelo; Malferrari, Giovanni; Palleria, Caterina; Sarro, Giovambattista De

    2012-01-01

    Involuntary abnormal movements have been reported after ischaemic and haemorrhagic stroke. Post stroke movement disorders can appear as acute or delayed sequel. At the moment, for many of these disorders the knowledge of pharmacological treatment is still inadequate. Dopaminergic and GABAergic systems may be mainly involved in post-stroke movement disorders. This article provides a review on drugs commonly used in post-stroke movement disorders, given that some post-stroke movement disorders have shown a partial benefit with pharmacological approach. PMID:23449883

  16. Hyperthyroidism in four guinea pigs: clinical manifestations, diagnosis, and treatment.

    PubMed

    Künzel, F; Hierlmeier, B; Christian, M; Reifinger, M

    2013-12-01

    Hyperthyroidism was diagnosed in four guinea pigs by demonstration of an increased serum total thyroxine concentration. The main clinical signs were comparable with those observed in feline hyperthyroidism and included weight loss despite maintenance of appetite and a palpable mass in the ventral cervical region. Three animals were treated successfully with methimazole for between 13 and 28 months. Clinical signs and regular measurement of circulating total thyroxine concentrations appear to be convenient parameters for monitoring response to medical therapy.

  17. Temporomandibular Joint Hypermobility Manifestation Based on Clinical Observations

    PubMed Central

    Nosouhian, Saeid; Haghighat, Abbas; Mohammadi, Iman; Shadmehr, Elham; Davoudi, Amin; Badrian, Hamid

    2015-01-01

    Background: Joint range of motion might affected by some factors like laxity and increase joint mobility. Generalized joint hypermobility and temporomandibular joint hypermobility (TMJH) are reported as risk factors for temporomandibular disorders. The aim of this study was to survey the etiological factors of TMJH and its relations to habitual status. Materials and Methods: In this cross-sectional descriptive study, 69 patients with TMJH were involved. After profiling personal information and medical history, the patients were divided into three groups based on their maximum mouth opening (MMO) as follow: (Light) MMO of 50-55 mm, (moderate): MMO between 55 and 65 mm, (severe) MMO >65 mm. For subjective observations, patients were asked to fill the prepared questionnaire. The objective evaluations conducted by a specialist. Finally, all the data subjected Chi-Square test by using SPSS software version 22 at a significant level of 0.05. Results: TMJH was more common in women (74.2%). The light group had significant differences with other groups in the discomfort of TMJ and TMJ sound (P < 0.05). Furthermore, sever group manifested highest percentage of masticatory pains, significantly (P < 0.05). Conclusion: It can be concluded that pain in TMJ would have a correlation with MMO. PMID:26464530

  18. The clinical manifestations, diagnosis, and treatment of adrenal emergencies.

    PubMed

    Tucci, Veronica; Sokari, Telematé

    2014-05-01

    Emergency medicine physicians should be able to identify and treat patients whose clinical presentations, including key historical, physical examination, and laboratory findings are consistent with diagnoses of primary, secondary, and tertiary adrenal insufficiency, adrenal crisis, and pheochromocytoma. Failure to make a timely diagnosis leads to increased morbidity and mortality. As great mimickers, adrenal emergencies often present with a constellation of nonspecific signs and symptoms that can lead even the most diligent emergency physician astray. The emergency physician must include adrenal emergencies in the differential diagnosis when encountering such clinical pictures.

  19. MELAS syndrome: Clinical manifestations, pathogenesis, and treatment options.

    PubMed

    El-Hattab, Ayman W; Adesina, Adekunle M; Jones, Jeremy; Scaglia, Fernando

    2015-01-01

    Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is one of the most frequent maternally inherited mitochondrial disorders. MELAS syndrome is a multi-organ disease with broad manifestations including stroke-like episodes, dementia, epilepsy, lactic acidemia, myopathy, recurrent headaches, hearing impairment, diabetes, and short stature. The most common mutation associated with MELAS syndrome is the m.3243A>G mutation in the MT-TL1 gene encoding the mitochondrial tRNA(Leu(UUR)). The m.3243A>G mutation results in impaired mitochondrial translation and protein synthesis including the mitochondrial electron transport chain complex subunits leading to impaired mitochondrial energy production. The inability of dysfunctional mitochondria to generate sufficient energy to meet the needs of various organs results in the multi-organ dysfunction observed in MELAS syndrome. Energy deficiency can also stimulate mitochondrial proliferation in the smooth muscle and endothelial cells of small blood vessels leading to angiopathy and impaired blood perfusion in the microvasculature of several organs. These events will contribute to the complications observed in MELAS syndrome particularly the stroke-like episodes. In addition, nitric oxide deficiency occurs in MELAS syndrome and can contribute to its complications. There is no specific consensus approach for treating MELAS syndrome. Management is largely symptomatic and should involve a multidisciplinary team. Unblinded studies showed that l-arginine therapy improves stroke-like episode symptoms and decreases the frequency and severity of these episodes. Additionally, carnitine and coenzyme Q10 are commonly used in MELAS syndrome without proven efficacy.

  20. Chromoblastomycosis: an overview of clinical manifestations, diagnosis and treatment.

    PubMed

    Queiroz-Telles, Flavio; Esterre, Phillippe; Perez-Blanco, Maigualida; Vitale, Roxana G; Salgado, Claudio Guedes; Bonifaz, Alexandro

    2009-02-01

    Chromoblastomycosis is one of the most frequent infections caused by melanized fungi. It is a subcutaneous fungal infection, usually an occupational related disease, mainly affecting individuals in tropical and temperate regions. Although several species are etiologic agents, Fonsecaea pedrosoi and Cladophialophora carrionii are prevalent in the endemic areas. Chromoblastomycosis lesions are polymorphic and must be differentiated from those associated with many clinical conditions. Diagnosis is confirmed by the observation of muriform cells in tissue and the isolation and the identification of the causal agent in culture. Chromoblastomycosis still is a therapeutic challenge for clinicians due to the recalcitrant nature of the disease, especially in the severe clinical forms. There are three treatment modalities, i.e., physical treatment, chemotherapy and combination therapy but their success is related to the causative agent, the clinical form and severity of the chromoblastomycosis lesions. There is no treatment of choice for this neglected mycosis, but rather several treatment options. Most of the patients can be treated with itraconazole, terbinafine or a combination of both. It is also important to evaluate the patient's individual tolerance of the drugs and whether the antifungal will be provided for free or purchased, since antifungal therapy must be maintained in long-term regimens. In general, treatment should be guided according to clinical, mycological and histopathological criteria.

  1. An update of clinical management of acute intermittent porphyria

    PubMed Central

    Pischik, Elena; Kauppinen, Raili

    2015-01-01

    Acute intermittent porphyria (AIP) is due to a deficiency of the third enzyme, the hydroxymethylbilane synthase, in heme biosynthesis. It manifests with occasional neuropsychiatric crises associated with overproduction of porphyrin precursors, aminolevulinic acid and porphobilinogen. The clinical criteria of an acute attack include the paroxysmal nature and various combinations of symptoms, such as abdominal pain, autonomic dysfunction, hyponatremia, muscle weakness, or mental symptoms, in the absence of other obvious causes. Intensive abdominal pain without peritoneal signs, acute peripheral neuropathy, and encephalopathy usually with seizures or psychosis are the key symptoms indicating possible acute porphyria. More than fivefold elevation of urinary porphobilinogen excretion together with typical symptoms of an acute attack is sufficient to start a treatment. Currently, the prognosis of the patients with AIP is good, but physicians should be aware of a potentially fatal outcome of the disease. Mutation screening and identification of type of acute porphyria can be done at the quiescent phase of the disease. The management of patients with AIP include following strategies: A, during an acute attack: 1) treatment with heme preparations, if an acute attack is severe or moderate; 2) symptomatic treatment of autonomic dysfunctions, polyneuropathy and encephalopathy; 3) exclusion of precipitating factors; and 4) adequate nutrition and fluid therapy. B, during remission: 1) exclusion of precipitating factors (education of patients and family doctors), 2) information about on-line drug lists, and 3) mutation screening for family members and education about precipitating factors in mutation-positive family members. C, management of patients with recurrent attacks: 1) evaluation of the lifestyle, 2) evaluation of hormonal therapy in women, 3) prophylactic heme therapy, and 4) liver transplantation in patients with severe recurrent attacks. D, follow-up of the AIP

  2. An update of clinical management of acute intermittent porphyria.

    PubMed

    Pischik, Elena; Kauppinen, Raili

    2015-01-01

    Acute intermittent porphyria (AIP) is due to a deficiency of the third enzyme, the hydroxymethylbilane synthase, in heme biosynthesis. It manifests with occasional neuropsychiatric crises associated with overproduction of porphyrin precursors, aminolevulinic acid and porphobilinogen. The clinical criteria of an acute attack include the paroxysmal nature and various combinations of symptoms, such as abdominal pain, autonomic dysfunction, hyponatremia, muscle weakness, or mental symptoms, in the absence of other obvious causes. Intensive abdominal pain without peritoneal signs, acute peripheral neuropathy, and encephalopathy usually with seizures or psychosis are the key symptoms indicating possible acute porphyria. More than fivefold elevation of urinary porphobilinogen excretion together with typical symptoms of an acute attack is sufficient to start a treatment. Currently, the prognosis of the patients with AIP is good, but physicians should be aware of a potentially fatal outcome of the disease. Mutation screening and identification of type of acute porphyria can be done at the quiescent phase of the disease. The management of patients with AIP include following strategies: A, during an acute attack: 1) treatment with heme preparations, if an acute attack is severe or moderate; 2) symptomatic treatment of autonomic dysfunctions, polyneuropathy and encephalopathy; 3) exclusion of precipitating factors; and 4) adequate nutrition and fluid therapy. B, during remission: 1) exclusion of precipitating factors (education of patients and family doctors), 2) information about on-line drug lists, and 3) mutation screening for family members and education about precipitating factors in mutation-positive family members. C, management of patients with recurrent attacks: 1) evaluation of the lifestyle, 2) evaluation of hormonal therapy in women, 3) prophylactic heme therapy, and 4) liver transplantation in patients with severe recurrent attacks. D, follow-up of the AIP

  3. Pleural effusion as the initial extramedullary manifestation of Acute Myeloid Leukemia

    PubMed Central

    Nieves-Nieves, José

    2012-01-01

    Leukemias rarely debut by pleural involvement as the first manifestation of the hematologic malignancy. This complication is most commonly seen in solid tumors such as carcinomas of the breast, lung, gastrointestinal tract and lymphomas. We present a case of a 66 year old male who presented with a pleural leukemic infiltration of his undiagnosed Acute Myeloid Leukemia that was not a complication of the disease extension, but the acute presentation of the illness. Progressive shortness of breath for two weeks, cough, clear sputum and weight loss were the initial complaints. Serum dyscrasia suggested a hematologic abnormality. A chest x-ray performed demonstrated a buildup of fluid with layering in the left pleural cavity. Diagnostic thoracentesis suggested an exudative etiology with cytology remarkable for 62% leukemic myeloblast. The diagnosis was confirmed by bone marrow biopsy with expression of the antigens CD 34+ and CD13+, with unfavorable cytogenetic prognosis and a trisomy 21 chromosomal defect. Chemotherapy was initiated, though no remission achieved with induction chemotherapy. Complications and disease progression precludes in the patient’s death. Although rare, due to the unusual presentation of the disease, this case clearly demonstrates the importance of biochemical analysis and cytopathology specimens obtained in pleural fluid. PMID:24358836

  4. Salvage of Theileria infected calves with clinical manifestation of exophthalmia.

    PubMed

    Singh, Shanker K; Sudan, Vikrant; Sachan, Pratibha; Srivastava, Ashish

    2015-09-01

    Two crossbred female calves aged between 30 and 35 days were presented with bilateral exophthalmia, inappetence, pyrexia and cachexia since last 15 days. Clinical examination revealed mainly bilateral exophthalmia with dry and pulpy cornea, generalized enlargement of superficial lymph nodes, pallor mucous, petechiae, high rectal temperature and sternal recumbency. The calves were severely infested with Hyalomma anatolicum anatolicum ticks and thin layer blood smears revealed presence of piroplasm in the RBCs, while lymph nodes aspirate smear examination revealed presence schizonts in the mononuclear cells. The calves were treated with buparvaquone; meloxicam, nandrolone decanoate and vitamins A, D3, E and H. From day second post-therapy a remarkable improvement in the clinical condition was noticed and substantial reduction in the both protruded eyeballs was noticed by 7 days post-therapy in the both calves. Further at day 47 post-therapy the one calf was free from the parasite on blood smear examination and right eye was retracted in its orbits with full of sight. Moreover the left eye was also retracted in its orbit but there was loss of sight and opacity developed in this eye. While, the other calf also revealed remarkable improvement in the clinical condition and both eye balls retracted completely into the orbit at day 30 post-therapy. But, at day 86 the calf developed microphthalmia and complete loss of sight in both eyes. It can be concluded that adjunction of antioxidants and hematopoietic agents may salvage the calves suffering from fatal theileriosis.

  5. Clinical manifestations of Kingella kingae infections: case report and review.

    PubMed

    Morrison, V A; Wagner, K F

    1989-01-01

    A patient with antecedent coccidioidal pulmonary cavitary disease who developed an empyema due to Kingella kingae prompted our analysis of the literature regarding this unusual bacterial pathogen. Formerly classified among other genera and considered a nonpathogen, K. kingae has been increasingly recognized as a cause of human infection. While the most commonly diagnosed infections due to this organism are endocarditis and septic arthritis, there have also been isolated reports of bacteremia, diskitis, abscesses, meningitis, and oropharyngeal infections. The treatment of choice is penicillin, to which K. kingae strains are uniformly susceptible. Recognition of the potential pathogenicity of this microorganism in appropriate clinical settings will probably result in more prompt and specific therapy.

  6. Feline Oral Squamous Cell Carcinoma: Clinical Manifestations and Literature Review.

    PubMed

    Bilgic, Ozgur; Duda, Lili; Sánchez, Melissa D; Lewis, John R

    2015-01-01

    Squamous cell carcinoma (SCC) is the most commonly encountered malignant oral tumor in cats. The etiology of this locally invasive tumor is likely multifactorial. Several risk factors have been identified, including the use of flea collars, and a history of feeding canned food and canned tuna. Clinical signs vary depending on tumor location. The tumor commonly arises from the gingiva and mucosa of the maxilla, mandible, tongue, sublingual area, or tonsillar region. Maxillary SCC commonly presents clinically as an ulcerative lesion, whereas mandibular SCC is commonly proliferative, expansile, and firm. Lingual/sublingual SCC may be ulcerative, necrotic, infiltrative, or proliferative. In general, feline oral SCC is an invasive and malignant neoplasm regardless of its location. Surgery, radiation therapy, chemotherapy and combinations thereof have been attempted with rarely a satisfactory response. Currently, cures are obtained only in a small subset of cats whose tumors are amenable to complete resection, or where resection with microscopic residual disease is followed by definitive radiation therapy. A multimodal treatment approach likely offers the best chance of success. For cats with advanced disease, palliative care may improve patients' quality of life, albeit transiently. Sequelae associated with tumor progression and local tissue destruction often result in euthanasia of feline patients with oral SCC.

  7. Clinical Manifestations of Fibrosing Mediastinitis in Chinese Patients

    PubMed Central

    Hu, Yan; Qiu, Jian-Xing; Liao, Ji-Ping; Zhang, Hong; Jin, Zhe; Wang, Guang-Fa

    2016-01-01

    Background: Fibrosing mediastinitis (FM) is a rare disease. FM is thought to be related to prior granulomatous mediastinal infection, such as histoplasmosis or tuberculosis. The majority of cases have been reported in endemic regions for histoplasmosis. The characteristics of cases of FM in China, where the prevalence of tuberculosis is high, have not been reported. We analyzed the clinical, imaging, and bronchoscopic features of Chinese patients with FM to promote awareness of this disease. Methods: Between January 2005 and June 2015, twenty patients were diagnosed with FM in our hospital. Medical records and follow-up data were collected. Imaging and biopsy findings were reviewed by radiologists and pathologists. Results: A total of 20 patients were analyzed (8 males and 12 females). The age ranged from 43 to 88 years with a mean age of 69.5 years. Previous or latent tuberculosis was found in 12 cases. Clinical symptoms included dyspnea (18/20), cough (17/20), expectoration (7/20), and recurrent pneumonia (3/20). Chest computed tomography scans showed a diffuse, homogeneous, soft tissue process throughout the mediastinum and hila with compression of bronchial and pulmonary vessels. Calcification was common (15/20). Pulmonary hypertension was present in 9 of 20 cases. Diffuse black pigmentation in the bronchial mucosa was frequently seen on bronchoscopy (12/13). The patients’ response to antituberculosis treatment was inconsistent. Conclusions: FM in Chinese patients is most likely associated with tuberculosis. Some characteristics of FM are different from cases caused by histoplasmosis. PMID:27824002

  8. Pancreas Adenocarcinoma: Ascites, Clinical Manifestations, and Management Implications

    PubMed Central

    Hicks, Angel Mier; Chou, Joanne; Capanu, Marinela; Lowery, Maeve A.; Yu, Kenneth H.; O’Reilly, Eileen M.

    2016-01-01

    spontaneous bacterial peritonitis in 7 (11%) managed with paracenteses versus 26 (23%) who had a catheter placed, catheter malfunction in 8 (7%), and acute renal failure in 6 (3%). After ascites development, 79 (44%) patients received active anti-cancer therapy, and 101 (56%) patients were managed with supportive care alone. Conclusions In patients with PAC who presented with or developed ascites, serial paracenteses and indwelling catheters are common methods used for providing symptomatic relief. The complication rate was higher with indwelling catheters, primarily related to infection (eg, bacterial peritonitis). Overall, ascites has a significantly negative prognostic import with a short median survival. PMID:27262896

  9. Non-stiff anti-amphiphysin syndrome: clinical manifestations and outcome after immunotherapy.

    PubMed

    Moon, Jangsup; Lee, Soon-Tae; Shin, Jung-Won; Byun, Jung-Ick; Lim, Jung-Ah; Shin, Yong-Won; Kim, Tae-Joon; Lee, Keon-Joo; Park, Kyung-Il; Jung, Keun-Hwa; Jung, Ki-Young; Lee, Sang Kun; Chu, Kon

    2014-09-15

    Amphiphysin antibody causes paraneoplastic stiff-person syndrome and can also result in a variety of neurological manifestations. Here, we investigated the clinical spectrum of 20 patients with non-stiff anti-amphiphysin syndrome and their responses to immunotherapy. The most common neurological manifestation was limbic encephalitis (n=10), followed by dysautonomia (n=9), and cerebellar dysfunction (n=6). Cancer was detected in only seven patients. Intravenous immunoglobulin or steroid treatment was effective in most patients, but three improved only after rituximab treatment. Our study suggests that anti-amphiphysin syndrome can manifest as non-stiff encephalomyelitis and is only partially associated with cancer. Active immunotherapy, including rituximab, would be beneficial.

  10. [A single metastasis in the carpal bones as the first clinical manifestation of a hepatocellular carcinoma].

    PubMed

    Corrales Pinzón, R; Alonso Sánchez, J M; de la Mano González, S; El Karzazi Tarazona, K

    2014-01-01

    Hepatocellular carcinoma is the most common primary tumor of the liver. Spreading outside the liver usually takes place in advanced stages of the disease, and bone is the third most common site of metastases. We present a case of hepatocellular carcinoma in which the first clinical manifestation was a single metastasis to the carpal bones. The interest of this case lies in the way this hepatocellular carcinoma manifested as well as in the unusual site of the metastasis.

  11. Clinical Manifestations of Senecavirus A Infection in Neonatal Pigs, Brazil, 2015

    PubMed Central

    Leme, Raquel A.; Oliveira, Thalita E.S.; Alcântara, Brígida K.; Headley, Selwyn A.; Alfieri, Alice F.; Yang, Ming

    2016-01-01

    We identified new clinical manifestations associated with Senecavirus A infection in neonatal piglets in Brazil in 2015. Immunohistochemical and molecular findings confirmed the association of Senecavirus A with these unusual clinical signs and more deaths. Other possible disease agents investigated were not associated with these illnesses. PMID:27315157

  12. Atrial Septal Defects – Clinical Manifestations, Echo Assessment, and Intervention

    PubMed Central

    Martin, Seth S; Shapiro, Edward P; Mukherjee, Monica

    2014-01-01

    Atrial septal defect (ASD) is a common congenital abnormality that occurs in the form of ostium secundum, ostium primum, sinus venosus, and rarely, coronary sinus defects. Pathophysiologic consequences of ASDs typically begin in adulthood, and include arrhythmia, paradoxical embolism, cerebral abscess, pulmonary hypertension, and right ventricular failure. Two-dimensional (2D) transthoracic echocardiography with Doppler is a central aspect of the evaluation. This noninvasive imaging modality often establishes the diagnosis and provides critical information guiding intervention. A comprehensive echocardiogram includes evaluation of anatomical ASD characteristics, flow direction, associated abnormalities (eg, anomalous pulmonary veins), right ventricular anatomy and function, pulmonary pressures, and the pulmonary/systemic flow ratio. The primary indication for ASD closure is right heart volume overload, whether symptoms are present or not. ASD closure may also be reasonable in other contexts, such as paradoxical embolism. ASD type and local clinical expertise guide choice of a percutaneous versus surgical approach to ASD closure. PMID:25861226

  13. Temporal complexity in clinical manifestations of lung disease.

    PubMed

    Frey, Urs; Maksym, Geoffrey; Suki, Béla

    2011-06-01

    In this review, we summarize results of recent research on the temporal variability of lung function, symptoms, and inflammatory biomarkers. Specifically, we demonstrate how fluctuation analysis borrowed from statistical physics can be used to gain insight into neurorespiratory control and complex chronic dynamic diseases such as asthma viewed as a system of interacting components (e.g., inflammatory, immunological, and mechanical). Fluctuation analysis tools are based on quantifying the distribution and the short- and long-term temporal history of tidal breathing and lung function parameters to assess neurorespiratory control and monitor chronic disease. The latter includes the assessment of severity and disease control, the impact of treatment and environmental triggers, the temporal characterization of disease phenotypes, and the individual risk of exacerbation. While in many cases specific mechanistic insight into the fluctuations still awaits further research, appropriate analyses of the fluctuations already impact on clinical science and practice.

  14. [Bouveret's syndrome: biliary ileus manifested by acute upper gastrointestinal hemorrhage and impaired gastric emptying].

    PubMed

    Simonek, J; Lischke, R; Drábek, J; Pafko, P

    2002-05-01

    The authors present a very rare case of impaired gastric evacuation, known as Bouveret's syndrome, caused by a large biliary concrement wedged in the duodenum as a result of the development of a cholecystoduodenal fistula in a 77-year-old man. The condition was manifested clinically by developed high ileus and subsequent haemorrhage into the upper GIT. The diagnosis was established on the background of the clinical picture, passage through the upper GIT and endoscopy. As the attempt to remove the concrement endoscopically failed, laparotomy had to be used. In the conclusion of this case-record the authors discuss the method of assessment of the correct diagnosis endoscopically and possibilities of therapeutic strategy.

  15. Transplant renal artery stenosis: clinical manifestations, diagnosis and therapy.

    PubMed

    Chen, Wei; Kayler, Liise K; Zand, Martin S; Muttana, Renu; Chernyak, Victoria; DeBoccardo, Graciela O

    2015-02-01

    Transplant renal artery stenosis (TRAS) is a well-recognized vascular complication after kidney transplant. It occurs most frequently in the first 6 months after kidney transplant, and is one of the major causes of graft loss and premature death in transplant recipients. Renal hypoperfusion occurring in TRAS results in activation of the renin-angiotensin-aldosterone system; patients usually present with worsening or refractory hypertension, fluid retention and often allograft dysfunction. Flash pulmonary edema can develop in patients with critical bilateral renal artery stenosis or renal artery stenosis in a solitary kidney, and this unique clinical entity has been named Pickering Syndrome. Prompt diagnosis and treatment of TRAS can prevent allograft damage and systemic sequelae. Duplex sonography is the most commonly used screening tool, whereas angiography provides the definitive diagnosis. Percutaneous transluminal angioplasty with stent placement can be performed during angiography if a lesion is identified, and it is generally the first-line therapy for TRAS. However, there is no randomized controlled trial examining the efficacy and safety of percutaneous transluminal angioplasty compared with medical therapy alone or surgical intervention.

  16. Prevalence and clinical manifestations of malaria in Aligarh, India.

    PubMed

    Asma, Umm-e; Taufiq, Farha; Khan, Wajihullah

    2014-12-01

    Malaria is one of the most widespread infectious diseases of tropical countries with an estimated 207 million cases globally. In India, there are endemic pockets of this disease, including Aligarh. Hundreds of Plasmodium falciparum and P. vivax cases with severe pathological conditions are recorded every year in this district. The aim of this study is to find out changes in liver enzymes and kidney markers. Specific diagnosis for P. falciparum and P. vivax was made by microscopic examination of Giemsa stained slides. Clinical symptoms were observed in both of these infections. Liver enzymes, such as AST, ALT, and ALP, and kidney function markers, such as creatinine and urea, were estimated by standard biochemical techniques. In Aligarh district, P. vivax, P. falciparum, and mixed infections were 64%, 34%, and 2%, respectively. In case of P. falciparum infection, the incidences of anemia, splenomegaly, renal failure, jaundice, and neurological sequelae were higher compared to those in P. vivax infection. Recrudescence and relapse rates were 18% and 20% in P. falciparum and P. vivax infections, respectively. Liver dysfunctions and renal failures were more common in P. falciparum patients, particularly in elderly patients. Artesunate derivatives must, therefore, be introduced for the treatment of P. falciparum as they resist to chloroquine as well as sulfadoxine-pyrimethamine combinations.

  17. Correlation of Serotype-Specific Dengue Virus Infection with Clinical Manifestations

    PubMed Central

    Halsey, Eric S.; Marks, Morgan A.; Gotuzzo, Eduardo; Fiestas, Victor; Suarez, Luis; Vargas, Jorge; Aguayo, Nicolas; Madrid, Cesar; Vimos, Carlos; Kochel, Tadeusz J.; Laguna-Torres, V. Alberto

    2012-01-01

    Background Disease caused by the dengue virus (DENV) is a significant cause of morbidity throughout the world. Although prior research has focused on the association of specific DENV serotypes (DENV-1, DENV-2, DENV-3, and DENV-4) with the development of severe outcomes such as dengue hemorrhagic fever and dengue shock syndrome, relatively little work has correlated other clinical manifestations with a particular DENV serotype. The goal of this study was to estimate and compare the prevalence of non-hemorrhagic clinical manifestations of DENV infection by serotype. Methodology and Principal Findings Between the years 2005–2010, individuals with febrile disease from Peru, Bolivia, Ecuador, and Paraguay were enrolled in an outpatient passive surveillance study. Detailed information regarding clinical signs and symptoms, as well as demographic information, was collected. DENV infection was confirmed in patient sera with polyclonal antibodies in a culture-based immunofluorescence assay, and the infecting serotype was determined by serotype-specific monoclonal antibodies. Differences in the prevalence of individual and organ-system manifestations were compared across DENV serotypes. One thousand seven hundred and sixteen individuals were identified as being infected with DENV-1 (39.8%), DENV-2 (4.3%), DENV-3 (41.5%), or DENV-4 (14.4%). When all four DENV serotypes were compared with each other, individuals infected with DENV-3 had a higher prevalence of musculoskeletal and gastrointestinal manifestations, and individuals infected with DENV-4 had a higher prevalence of respiratory and cutaneous manifestations. Conclusions/Significance Specific clinical manifestations, as well as groups of clinical manifestations, are often overrepresented by an individual DENV serotype. PMID:22563516

  18. [Acute lithiasic cholecystitis, situs viscerum inversus, vena porta preduodenale (considerations on a clinical case)].

    PubMed

    Amadio, M; Galimberti, G B; Perovani Vicari, P

    1982-10-01

    Authors report a case of preduodenal portal vein in a woman 68 years old with acute cholecystitis in situs viscerum inversus adbominis and levocardia. Surgery was difficult. They shortly analyze also etiology and clinical manifestations of situs inversus and PPV, with related malformations. They conclude that it is important to recognize the presence of situs inversus preoperatively and everytime suspect other anatomical anomalies.

  19. Pemphigus in Korea: clinical manifestations and treatment protocol.

    PubMed

    Seo, Pan Gyo; Choi, Won Woo; Chung, Jin Ho

    2003-11-01

    Pemphigus, a rare, chronic blistering disease of the skin and mucous membranes with severe morbidity and occasional mortality, is the most common autoimmune bullous disease in Korea. The purpose of this study was to evaluate the clinical features and propose a treatment strategy for patients with pemphigus. A retrospective analysis was conducted of 51 pemphigus patients seen between 1993 and 2001. Pemphigus vulgaris (PV) was the most common type with 32 cases, followed by 19 cases of pemphigus foliaceus (PF). The male to female ratio was 1:1.3, with females predominating, particularly among PV patients (PV, 1:1.5; PF, 1:1.1). The average ages at onset of PV and PF were 44.3 and 51.0 years old, respectively. Mucosal involvement was noted in 27 cases (84.4%) of PV but in only 3 cases (15.8%) of PF. Most patients initially received relatively low to intermediate doses (0.3-1.0 mg/kg/day) of prednisolone, and 23 (71.9%) PV patients and 10 (52.6%) PF patients also received immunosuppressive agents. Oral prednisolone and azathioprine (100 mg/day) formed the mainstay of treatment for our patients (47.1%). At the time of writing, 25.5% (13/51) of patients are in complete remission, and 72.5% (37/51) are undergoing maintenance therapy. One patient died due to sepsis during the treatment. For the treatment of pemphigus, a course of the lowest possible corticosteroid dosage in combination with immunosuppressive agents appears to be effective and less toxic than a high corticosteroid dosage.

  20. Clinical manifestations of Eosinophilic meningitis due to infection with Angiostrongylus cantonensis in children.

    PubMed

    Sawanyawisuth, Kittisak; Chindaprasirt, Jarin; Senthong, Vichai; Limpawattana, Panita; Auvichayapat, Narong; Tassniyom, Sompon; Chotmongkol, Verajit; Maleewong, Wanchai; Intapan, Pewpan M

    2013-12-01

    Eosinophilic meningitis, caused by the nematode Angiostrongylus cantonensis, is prevalent in northeastern Thailand, most commonly in adults. Data regarding clinical manifestations of this condition in children is limited and may be different those in adults. A chart review was done on 19 eosinophilic meningitis patients aged less than 15 years in Srinagarind Hospital, Faculty of Medicine, Khon Kaen University, Thailand. Clinical manifestations and outcomes were reported using descriptive statistics. All patients had presented with severe headache. Most patients were males, had fever, nausea or vomiting, stiffness of the neck, and a history of snail ingestion. Six patients had papilledema or cranial nerve palsies. It was shown that the clinical manifestations of eosinophilic meningitis due to A. cantonensis in children are different from those in adult patients. Fever, nausea, vomiting, hepatomegaly, neck stiffness, and cranial nerve palsies were all more common in children than in adults.

  1. Acute Psychosis as Major Clinical Presentation of Legionnaires' Disease

    PubMed Central

    Silva-dos-Santos, Amílcar; Talina, Miguel Cotrim

    2016-01-01

    We report a case of a 61-year-old woman who presented with acute psychosis as a major manifestation of Legionnaires' disease in the absence of other neuropsychiatric symptoms. Clinical history revealed dry cough and nausea. Observation showed fever and auscultation crackles in the lower lobe of the right lung. Laboratory testing demonstrated elevated C-reactive protein and lung chest radiograph showed patchy peribronchial and right lower lobe consolidation. Soon after admission, she started producing purulent sputum. Epidemiological data suggested Legionella pneumophila as possible cause of the clinical picture that was confirmed by urinary antigen detection and polymerase chain reaction of the sputum. She was treated with levofloxacin 750 mg/day for 10 days with complete remission of pulmonary and psychiatric symptoms. She has not had further psychotic symptoms. PMID:27547478

  2. Clinical features of acute corpus callosum infarction patients

    PubMed Central

    Yang, Li-Li; Huang, Yi-Ning; Cui, Zhi-Tang

    2014-01-01

    The clinical manifestation of acute corpus callosum (CC) infarction is lack of specificity and complex, so it is easily missed diagnosis and misdiagnosis in the early stage. The present study aims to describe the clinical features of the acute CC infarction. In this study, 25 patients with corpus callosum infarction confirmed by the brain MRI/DWI and the risk factors were summarized. Patients were classified into genu infarction (3 cases), body infarction (4cases), body and genu infarction (4 cases), body and splenium infarction (1 case), splenium infarction (13 cases) according to lesion location. Clinical manifestation and prognosis were analyzed among groups. The results indicated that CC infarction in patients with high-risk group accounted for 72%, moderate-risk group accounted for 20%, low-risk group (8%). The main risk factors are carotid intimal thickening or plaque formation, hypertension, hyperlipidemia, cerebral artery stenosis, and so on. The CC infarction often merged with other parts infarction, and splenium infarction had the highest incidence, the clinical symptoms in the body infarction which can appear typical signs and symptoms, but in other parts infarction which always merged many nerve defect symptoms. The body infarction prognosis is poor; the rest parts of infarction are more favorable prognosis. In conclusion, CC infarction has the highest incidence in the stroke of high-risk group; neck color Doppler and TCD examination can be found as early as possible to explore the pathogenic factors. Prognosis is usually much better by treatment according to the location and risk factors. PMID:25197390

  3. Epidemiological features and clinical manifestations of Lyme borreliosis in Korea during the period 2005-2012.

    PubMed

    Moon, Shinje; Hong, Yeongseon; Hwang, Kyu-Jam; Kim, Suyeon; Eom, Jihye; Kwon, Donghyok; Park, Ji-Hyuk; Youn, Seung-Ki; Sohn, Aeree

    2015-01-01

    Lyme borreliosis is one of the most common tick-borne infections in the northern hemisphere. However, the epidemiological features and clinical manifestations of this disease in Korea are unknown. The present study is the first to investigate the characteristics of Lyme borreliosis in Korea. We traced suspected cases of Lyme borreliosis during the period 2005-2012. Of the 16 identified patients with the disease, 11 had acquired autochthonous infection within Korea, while 5 patients were infected outside Korea. The history of past exposure was investigated in 8 of the 11 patients with autochthonous infection; 5 of these 8 patients (62.5%) were suspected to have acquired the infection in the northeastern alpine region. Clinically, of 11 patients with autochthonous infection, 6 (54.5%) showed early skin manifestations, 4 (36.4%) showed neurological manifestations, and 1 (9.1%) showed acrodermatitis chronica atrophicans. In conclusion, Lyme borreliosis could be endemic in the northeastern alpine region of Korea, and neurological and early skin manifestations are likely to be the major clinical characteristics of autochthonous Lyme borreliosis in Korea.

  4. Latrodectism: variations in clinical manifestations provoked by Latrodectus species of spiders.

    PubMed

    Maretić, Z

    1983-01-01

    The clinical picture caused by the bite of Latrodectus spp. is described, including the patient's general condition with agonizing pains, cramps, spasms, motor unrest, boardlike abdomen, "facies latrodectismica", sweating, oliguria, hypertension, anxiety, mental excitation and an extended convalescence. The notorious biters are mainly L. mactans, L. variolus, L. hesperus, L. tredecimguttatus, L. hasselti, L. indistinctus and L. menavodi, all of which appear to provoke somewhat similar clinical pictures. For some species, however, there is limited data and for the toxicity of some spiders, such as L. rhodesiensis, no data are available, However, it would appear that the clinical manifestations of the venom are similar in all species.

  5. Minamata disease revisited: an update on the acute and chronic manifestations of methyl mercury poisoning.

    PubMed

    Ekino, Shigeo; Susa, Mari; Ninomiya, Tadashi; Imamura, Keiko; Kitamura, Toshinori

    2007-11-15

    The first well-documented outbreak of acute methyl mercury (MeHg) poisoning by consumption of contaminated fish occurred in Minamata, Japan, in 1953. The clinical picture was officially recognized and called Minamata disease (MD) in 1956. However, 50 years later there are still arguments about the definition of MD in terms of clinical symptoms and extent of lesions. We provide a historical review of this epidemic and an update of the problem of MeHg toxicity. Since MeHg dispersed from Minamata to the Shiranui Sea, residents living around the sea were exposed to low-dose MeHg through fish consumption for about 20 years (at least from 1950 to 1968). These patients with chronic MeHg poisoning continue to complain of distal paresthesias of the extremities and the lips even 30 years after cessation of exposure to MeHg. Based on findings in these patients the symptoms and lesions in MeHg poisoning are reappraised. The persisting somatosensory disorders after discontinuation of exposure to MeHg were induced by diffuse damage to the somatosensory cortex, but not by damage to the peripheral nervous system, as previously believed.

  6. Clinical manifestations in female carriers of mucopolysaccharidosis type II: a spanish cross-sectional study

    PubMed Central

    2013-01-01

    Background Mucopolysaccharidosis type II (MPS II) is an inherited X-linked disease associated with a deficiency in the enzyme iduronate 2-sulfatase due to iduronate 2-sulfatase gene (IDS) mutations. Recent studies in MPS II carriers did not find clinical involvement, but these were mainly performed by anamnesis and patients’ self-reported description of signs and symptoms. So although it is rare in heterozygous carriers, investigations in other types of inherited X-linked disorders suggest that some clinical manifestations may be a possibility. The aim of this study was to evaluate the clinical pattern in female carriers of MPS II and to determine whether clinical symptoms were associated with the X-chromosome inactivation (XCI) pattern and age. Methods Female carriers of MPS II were genetically identified by molecular analysis of IDS. The clinical evaluation protocol included pedigree analysis, a comprehensive anamnesis, complete physical examination, ophthalmological evaluation, brain-evoked auditory response, electrocardiogram, echocardiogram, pulmonary function tests, abdominal sonogram, skeletal survey, neurophysiological studies, blood cell counts and biochemistry, urine glycosaminoglycan (GAGs) quantification, karyotype and pattern of XCI. Results Ten women were included in the study. The mean age of the participants was 40.2 ± 13.1 years. Six carriers presented a skewed XCI pattern, 3 of whom (aged 38, 42 and 52 years) had increased levels of GAGs in the urine and showed typical MPS II clinical manifestations, such as skeletal anomalies, liver abnormalities, carpal tunnel syndrome, recurrent ear infection, hypoacusia and more frequent severe odontological problems without coarse facial features. Conclusions This is the first study performing a comprehensive evaluation of heterozygous MPS II carriers. Our results provide evidence of possible progressive, age-dependent, mild clinical manifestations in MPS II female carriers with a skewed XCI pattern

  7. Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment.

    PubMed

    Ortega-Hernandez, Oscar-Danilo; Shoenfeld, Yehuda

    2012-02-01

    The most common clinical manifestations of mixed connective disease are Raynaud's phenomenon, arthralgias, swollen joints, esophageal dysfunction, muscle weakness and fingers sausage-like appearance together with the presence of anti-ribonucleoprotein (RNP) antibodies. However, organ involvement is more extensive than first descriptions reported. The disease can be serious with development of pulmonary, kidney, cardiovascular, gastrointestinal and central nervous system manifestations. The worst prognosis and high mortality are associated with the presence of pulmonary disease. Although a different set of clinical criteria have been proposed, there is no consensus about the most accurate. There is no full agreement about treatment and the initial impression of a satisfactory response to low doses of steroids is not always the rule. Herein, we review available evidence to a better approach to all previous topics.

  8. Dengue Infection in Children in Ratchaburi, Thailand: A Cohort Study. II. Clinical Manifestations

    PubMed Central

    Sirivichayakul, Chukiat; Limkittikul, Kriengsak; Chanthavanich, Pornthep; Jiwariyavej, Vithaya; Chokejindachai, Watcharee; Pengsaa, Krisana; Suvannadabba, Saravudh; Dulyachai, Wut; Letson, G. William; Sabchareon, Arunee

    2012-01-01

    Background Dengue infection is one of the most important mosquito-borne diseases. More data regarding the disease burden and the prevalence of each clinical spectrum among symptomatic infections and the clinical manifestations are needed. This study aims to describe the incidence and clinical manifestations of symptomatic dengue infection in Thai children during 2006 through 2008. Study Design This study is a school-based prospective open cohort study with a 9,448 person-year follow-up in children aged 3–14 years. Active surveillance for febrile illnesses was done in the studied subjects. Subjects who had febrile illness were asked to visit the study hospital for clinical and laboratory evaluation, treatment, and serological tests for dengue infection. The clinical data from medical records, diary cards, and data collection forms were collected and analyzed. Results Dengue infections were the causes of 12.1% of febrile illnesses attending the hospital, including undifferentiated fever (UF) (49.8%), dengue fever (DF) (39.3%) and dengue hemorrhagic fever (DHF) (10.9%). Headache, anorexia, nausea/vomiting and myalgia were common symptoms occurring in more than half of the patients. The more severe dengue spectrum (i.e., DHF) had higher temperature, higher prevalence of nausea/vomiting, abdominal pain, rash, diarrhea, petechiae, hepatomegaly and lower platelet count. DHF cases also had significantly higher prevalence of anorexia, nausea/vomiting and abdominal pain during day 3–6 and diarrhea during day 4–6 of illness. The absence of nausea/vomiting, abdominal pain, diarrhea, petechiae, hepatomegaly and positive tourniquet test may predict non-DHF. Conclusion Among symptomatic dengue infection, UF is most common followed by DF and DHF. Some clinical manifestations may be useful to predict the more severe disease (i.e., DHF). This study presents additional information in the clinical spectra of symptomatic dengue infection. PMID:22389735

  9. Molecular analysis and association with clinical and laboratory manifestations in children with sickle cell anemia

    PubMed Central

    Camilo-Araújo, Roberta Faria; Amancio, Olga Maria Silverio; Figueiredo, Maria Stella; Cabanãs-Pedro, Ana Carolina; Braga, Josefina Aparecida Pellegrini

    2014-01-01

    Objectives To analyze the frequency of βS-globin haplotypes and alpha-thalassemia, and their influence on clinical manifestations and the hematological profile of children with sickle cell anemia. Method The frequency of βS-globin haplotypes and alpha-thalassemia and any association with clinical and laboratorial manifestations were determined in 117 sickle cell anemia children aged 3–71 months. The confirmation of hemoglobin SS and determination of the haplotypes were achieved by polymerase chain reaction-restriction fragment length polymorphism, and alpha-thalassemia genotyping was by multiplex polymerase chain reaction (single-tube multiplex-polymerase chain reaction). Results The genotype distribution of haplotypes was 43 (36.7%) Central African Republic/Benin, 41 (35.0%) Central African Republic/Central African Republic, 20 (17.0%) Rare/atypical, and 13 (11.1%) Benin/Benin. The frequency of the α3.7 deletion was 1.71% as homozygous (−α3.7/−α3.7) and 11.9% as heterozygous (−α3.7/αα). The only significant association in respect to haplotypes was related to the mean corpuscular volume. The presence of alpha-thalassemia was significantly associated to decreases in mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte count and to an increase in the red blood cell count. There were no significant associations of βS-globin haplotypes and alpha-thalassemia with clinical manifestations. Conclusions In the study population, the frequency of alpha-thalassemia was similar to published data in Brazil with the Central African Republic haplotype being the most common, followed by the Benin haplotype. βS-globin haplotypes and interaction between alpha-thalassemia and sickle cell anemia did not influence fetal hemoglobin concentrations or the number of clinical manifestations. PMID:25305165

  10. The clinics of acute coronary syndrome

    PubMed Central

    Rastelli, Gianni

    2016-01-01

    Risk stratification and management of patients with chest pain continues to be challenging despite considerable efforts made in the last decades by many clinicians and researchers. The throutful evaluation necessitates that the physicians have a high index of suspicion for acute coronary syndrome (ACS) and always keep in mind the myriad of often subtle and atypical presentations of ischemic heart disease, especially in certain patient populations such as the elderly ones. In this article we aim to review and discuss the available evidence on the value of clinical presentation in patients with a suspected ACS, with special emphasis on history, characteristics of chest pain, associated symptoms, atypical presentations, precipitating and relieving factors, drugs, clinical rules and significance of clinical Gestalt. PMID:27294087

  11. Clinical practice guideline: management of acute pancreatitis

    PubMed Central

    Greenberg, Joshua A.; Hsu, Jonathan; Bawazeer, Mohammad; Marshall, John; Friedrich, Jan O.; Nathens, Avery; Coburn, Natalie; May, Gary R.; Pearsall, Emily; McLeod, Robin S.

    2016-01-01

    There has been an increase in the incidence of acute pancreatitis reported worldwide. Despite improvements in access to care, imaging and interventional techniques, acute pancreatitis continues to be associated with significant morbidity and mortality. Despite the availability of clinical practice guidelines for the management of acute pancreatitis, recent studies auditing the clinical management of the condition have shown important areas of noncompliance with evidence-based recommendations. This underscores the importance of creating understandable and implementable recommendations for the diagnosis and management of acute pancreatitis. The purpose of the present guideline is to provide evidence-based recommendations for the management of both mild and severe acute pancreatitis as well as the management of complications of acute pancreatitis and of gall stone–induced pancreatitis. Une hausse de l’incidence de pancréatite aiguë a été constatée à l’échelle mondiale. Malgré l’amélioration de l’accès aux soins et aux techniques d’imagerie et d’intervention, la pancréatite aiguë est toujours associée à une morbidité et une mortalité importantes. Bien qu’il existe des guides de pratique clinique pour la prise en charge de la pancréatite aiguë, des études récentes sur la vérification de la prise en charge clinique de cette affection révèlent des lacunes importantes dans la conformité aux recommandations fondées sur des données probantes. Ces résultats mettent en relief l’importance de formuler des recommandations compréhensibles et applicables pour le diagnostic et la prise en charge de la pancréatite aiguë. La présente ligne directrice vise à fournir des recommandations fondées sur des données probantes pour la prise en charge de la pancréatite aiguë, qu’elle soit bénigne ou grave, ainsi que de ses complications et de celles de la pancréatite causée par un calcul biliaire. PMID:27007094

  12. Correlating clinical manifestations with factor levels in rare bleeding disorders: a report from Southern India.

    PubMed

    Viswabandya, A; Baidya, S; Nair, S C; Abraham, A; George, B; Mathews, V; Chandy, M; Srivastava, A

    2012-05-01

    Data on the clinical manifestations of patients with clotting factor defects other than Haemophilia A, B and von Willebrand disease are limited because of their rarity. Due to their autosomal recessive nature of inheritance, these diseases are more common in areas where there is higher prevalence of consanguinity. There is no previous large series reported from southern India where consanguinity is common. Our aim was to analyze clinical manifestations of patients with rare bleeding disorders and correlate their bleeding symptoms with corresponding factor level. Data were collected in a standardized format from our centre over three decades on 281 patients who were diagnosed with rare bleeding disorders (fibrinogen, prothrombin, factor V (FV), FVII, FX, FXI, FXIII and combined FV or FVIII deficiency). Patients with liver dysfunction or those on medications which can affect factor level were excluded. All patients with <50% factor levels were included in this analysis. Patients were analysed for their salient clinical manifestations and it was correlated with their factor levels. The data shows that FXIII deficiency is the commonest and FXI deficiency is the rarest in Southern India. There was no significant difference in bleeding symptoms among those who were < or >1% factor coagulant activities among all disorders, except for few symptoms in FVII and FX deficiency. An international collaborative study is essential to find out the best way of classifying severity in patients with rare bleeding disorders.

  13. Clinical manifestation, imaging, and genotype analysis of two pedigrees with spinocerebellar ataxia.

    PubMed

    Peipei, Liu; Yang, Liu; Weihong, Gu; Xiaonan, Song

    2011-12-01

    The objective of this study was to analyze the clinical manifestation, imaging characteristics, genotype, and the relationship between the three aforementioned parameters in two pedigrees suffering from spinocerebellar ataxia. To evaluate the clinical manifestation of the two pedigrees and to compare the characteristics, we performed the MRI analysis of some patients from both pedigrees, while 2 ml of the peripheral blood sample was collected for gene analysis. The gene analysis data showed that pedigree 1 was certified spinocerebellar ataxia type-2 (SCA2); the CAG repeats in the proband, proband's mother, and proband's brother were 44, 36, and 38, respectively. The MRI revealed brainstem cerebellar atrophy and "cross sign" and "ordinate sign" of pons. Pedigree 2 was certified SCA1; the CAG repeats of the proband, proband's aunt, and proband's asymptomatic cousin were 60, 51, and 52, respectively. The MRI revealed cerebellar atrophy in these individuals. We, therefore, concluded that it was difficult to diagnose the SCA subset solely through the clinical manifestation. The imaging characteristics analysis and final diagnosis depended basically on gene analysis data.

  14. Thin-Section Computed Tomography Manifestations During Convalescence and Long-Term Follow-Up of Patients with Severe Acute Respiratory Syndrome (SARS).

    PubMed

    Wu, Xiaohua; Dong, Dawei; Ma, Daqing

    2016-08-08

    BACKGROUND SARS is not only an acute disease, but also leads to long-term impaired lung diffusing capacity in some survivors. However, there is a paucity of data regarding long-term CT findings in survivors after SARS. The aim of this study was to assess the changes in lung function and lung thin-section computed tomography (CT) features in patients recovering from severe acute respiratory syndrome (SARS), especially the dynamic changes in ground-glass opacity (GGO). MATERIAL AND METHODS Clinical and radiological data from 11 patients with SARS were collected. The serial follow-up thin-section CTs were evaluated at 3, 6, and 84 months after SARS presentation. The distribution and predominant thin-section CT findings of lesions were evaluated. RESULTS The extent of the lesions on the CT scans of the 11 patients decreased at 6 and 84 months compared to 3 months. The number of segments involved on 84-month follow-up CTs was less than those at 6 months (P<0.05). The predominant thin-section CT manifestation at 84 months (intralobular and interlobular septal thickening) was different than that at 6 months, at which GGO was predominant. CONCLUSIONS During convalescence after SARS, GGO and intralobular and interlobular septal thickening were the main thin-section CT manifestation. Intralobular and interlobular septal thickening predominated over GGO at 84 months.

  15. Long-Term Clinical Outcomes According to Previous Manifestations of Atherosclerotic Disease (from the FAST-MI 2010 Registry).

    PubMed

    Puymirat, Etienne; Aissaoui, Nadia; Lemesle, Gilles; Cottin, Yves; Coste, Pierre; Schiele, François; Ferrières, Jean; Simon, Tabassome; Danchin, Nicolas

    2017-03-01

    The prognosis of patients with acute myocardial infarction (AMI) has notably improved in the past 20 years. Using the French Registry of ST-Elevation and Non-ST-elevation Myocardial Infarction (FAST-MI) 2010 registry, we investigated whether previous manifestations of atherosclerotic disease (i.e., previous MI, or a history of any form of atherosclerotic disease) are at truly increased risk compared with those in whom AMI is the first manifestation of the disease. FAST-MI 2010 is a nationwide French registry including 3,079 patients with AMI, among whom 1,062 patients had a history of cardiovascular atherosclerotic disease and 498 patients had a history of MI. Overall, patients with a history of atherosclerotic disease (or MI) were older compared with patients without known cardiovascular disease (71 ± 13 vs 63 ± 14 years) and had higher cardiovascular risk profiles and co-morbidities. Using fully adjusted Cox multivariate analysis, previous manifestations of atherosclerotic disease were associated with higher 3-year mortality (hazard ratio 1.80, 95% confidence interval 1.40 to 2.31; p <0.001) as history of previous MI alone (hazard ratio 1.32, 95% confidence interval 1.00 to 1.73; p = 0.048). Similar results were found in patients discharged alive. In conclusion, previous cardiovascular atherosclerotic disease represents 1/3 of patients with AMI and are strongly associated with worse long-term clinical outcomes. Intensive follow-up and therapy should be encouraged in this high-risk population.

  16. Acute clinical onset chronic inflammatory demyelinating polyneuropathy in a dog.

    PubMed

    Molín, Jéssica; Márquez, Mercedes; Raurell, Xavier; Matiasek, Kaspar; Ferrer, Isidre; Pumarola, Martí

    2011-09-01

    We report a case of acute-onset ambulatory paraparesis with electrophysiological abnormalities compatible with axonal and demyelinating lesions in a Rottweiler dog. Although the clinical findings were compatible with acute canine idiopathic polyneuropathy, postmortem investigations revealed a chronic demyelinating polyneuropathy affecting the nerve roots. Due to the combination of acute clinical presentation and chronic pathologic features, this case is consistent with the acute-onset form of chronic inflammatory demyelinating polyneuropathy (A-CIDP).

  17. [Acute cerebral ischemia: an unusual clinical presentation of isolated left ventricular noncompaction in an adult patient].

    PubMed

    Fiorencis, Andrea; Quadretti, Laura; Bacich, Daniela; Chiodi, Elisabetta; Mele, Donato; Fiorencis, Roberto

    2013-01-01

    Isolated left ventricular noncompaction in adults is uncommon. The most frequent clinical manifestations are heart failure due to left ventricular systolic dysfunction and supraventricular and ventricular arrhythmias, which may be sustained and associated with sudden death. Thromboembolic complications are also possible. We report the case of an adult patient with isolated left ventricular noncompaction who came to our observation because of acute cerebral ischemia, an initial presentation of the disease only rarely described.

  18. Acute bithalamic infarct manifesting as sleep-like coma: A diagnostic challenge.

    PubMed

    Honig, Asaf; Eliahou, Ruth; Eichel, Roni; Shemesh, Ari Aharon; Ben-Hur, Tamir; Auriel, Eitan

    2016-12-01

    Bilateral thalamic infarction (BTI) typically presents as a sleep-like coma (SLC) without localizing signs, posing a diagnostic challenge that may lead the treating physician to search for toxic or metabolic causes and delay treatment. We review our experience with BTI of different etiologies, and emphasize the critical role of timely imaging, diagnosis, and management in a series of 12 patients with a presentation of SLC and acute BTI who were managed in our Medical Centers from 2006-2015. In 11/12, urgent head CT scans showed normal brain tissue, while diffusion-weighted (DWI) MRI revealed symmetric bilateral thalamic hyperintense lesions with variable degrees of brainstem involvement. In 1/12, CT scans revealed a contralateral subacute stroke from a thalamic infarct 1month earlier with a unilateral hyperintense lesion on DWI-MRI. From clinical and imaging findings (DWI-MRI, CT angiography and venography), etiology was attributed to embolic causes (cardio-embolism, artery-to-artery mechanism), small vessel disease, or deep sinus vein thrombosis secondary to dural arteriovenous (AV) fistula. Three patients had good outcomes after prompt diagnosis and optimal treatment in <3hours (intravenous tissue plasminogen activator in two patients cardio-embolic etiology and neuro-endovascular repair in one patient with venous infarction due to a dural AV fistula). The diagnosis was made beyond the therapeutic window in seven patients, who were left with significant neurological sequelae. Higher awareness of BTI presenting as SLC is warranted. Optimal patient management includes urgent DWI-MRI. In cases of BTI, further imaging workup is indicated to provide a comprehensive assessment for etiology. Early diagnosis and prompt, targeted intervention are crucial.

  19. Pattern of initial clinical manifestations of systemic lupus erythematosus in a tertiary care hospital

    PubMed Central

    Batool, Shabnam; Ahmad, Nighat Mir; Saeed, Muhammad Ahmed; Farman, Sumaira

    2016-01-01

    Objective: To determine the pattern of initial clinical manifestations of Systemic Lupus Erythematosus (SLE) and to compare these features with those recorded elsewhere in Pakistan Methods: This cross-sectional, descriptive study was performed in the Department of Rheumatology, Fatima Memorial Hospital, Lahore, Pakistan, from November 2015 to January 2016. Sixty one patients of SLE diagnosed as per ACR (American College of Rheumatology) 1982 revised criteria, were enrolled. The patients were evaluated for the initial clinical manifestations of SLE. The information was collected on a specially designed proforma and analyzed by using SPSS version 17. Results: Out of 61 patients, 49 (80.3%) were females and 12 (19.7%) males, showing a female to male ratio of 4:1. The mean age of patients was 26.2 ± 7.9 years. Fatigue was the most common presenting feature in 56 (91.8%) patients, followed by joint pains in 55 (90.2%) and fever in 54 (88.5%). Renal involvement was found in 46 (75.4%). Comparison of these presenting features was made with other studies carried out in Northern Pakistan (Islamabad) and in central Punjab (Pakistan). There were statistically significant differences in fever, fatigue and arthritis between our patients and the other two above mentioned study groups. However, comparison of renal manifestations showed significant difference only with Islamabad study, and not with previous study from central Punjab. Conclusion: In this study, majority of patients presented with combination of fatigue, fever, rash and arthritis. Almost three-fourth of patients had renal manifestations at initial presentation. Therefore, it is important for clinicians to have high index of suspicion for SLE, when patients present with above symptoms. PMID:27881995

  20. Clinical and immunological manifestations in 151 SLE patients living in Dubai.

    PubMed

    AlSaleh, J; Jassim, V; ElSayed, M; Saleh, N; Harb, D

    2008-01-01

    To gain better understanding of systemic lupus erythematosus (SLE) in Dubai we studied the clinical and immunological manifestations in a cohort of 151 patients attended Rheumatology Clinic in Dubai Hospital between January 2002 and January 2007. We found that the female to male ratio was 20.5:1, with a mean age of 35.5 years (0.9). The mean age at disease onset was 28.9 years (0.8) and mean disease duration 6.7 years (0.4). Five-year survival rate in our cohort was 94%. The commonest clinical manifestations in this cohort were arthritis (88%), haematological abnormalities (61.6%), and malar rash (60.3%). Leucopenia, fever, hair loss and proteinuria were observed in approximately half of the patients. Anaemia was found in 44.3% but only 9.9% had haemolytic anaemia. Photosensitive rash was seen in 43% of patients. Approximately one-third of the patients had serositis and mouth ulcers, 30.5 and 27.2% respectively. Vasculitis was observed in 19.2% of patients. Neuropsychiatric manifestations (15.9%), discoid lupus lesions (12.6%), and brain infarcts (13.2%) were infrequent. Subacute cutaneous lupus (6%) was also uncommon. Anti-nuclear antibodies were detected in 98%, anti-double stranded DNA antibodies in 88.7%, anti-Sm antibodies in 19.7%, anti-RNP in 40.4%, anti-Ro antibodies in 52.3% and anti-La antibodies in 19.8%. Anti-cardiolipin IgM and IgG were detected in 25.3 and 22.4%, respectively. This study suggests that Arabs with SLE residing in Dubai have comparable clinical features to their counterparts in other Arab countries and Western countries. The high prevalence of positive anti-Ro antibodies among our Arab patients probably reflects a character, that is, commonly seen in SLE patients of Middle East origin.

  1. The clinical manifestation of MCAD deficiency: challenges towards adulthood in the screened population.

    PubMed

    Schatz, Ulrich A; Ensenauer, Regina

    2010-10-01

    Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is the most common fatty acid oxidation disorder. Typically, undiagnosed individuals are asymptomatic until an episode of increased energy demand and fasting occurs, resulting in metabolic derangement. Phenotypic heterogeneity has been increasingly realized, with reports of both neonates and adults manifesting with life-threatening symptoms including encephalopathy, rhabdomyolysis, and cardiac failure. If diagnosed presymptomatically, outcome is favorable basically by avoidance of fasting. Early detection by newborn screening (NBS) has significantly reduced the incidence of severe adverse events including deaths. In this manuscript we focus on the natural course of the disease in both children and adults. Although NBS for MCADD has been successfully established, continuing efforts need to be made to avoid acute crises and deterioration of outcome in screened patients entering adolescence and adulthood.

  2. Epidemiology, clinical manifestations, and diagnosis of Chikungunya fever: lessons learned from the re-emerging epidemic.

    PubMed

    Mohan, Alladi; Kiran, D H N; Manohar, I Chiranjeevi; Kumar, D Prabath

    2010-01-01

    Chikungunya fever, caused by "Chikungunya virus," is an arbovirus disease transmitted by the bite of infected mosquitoes belonging to the genus Aedes. Chikungunya fever epidemics have been reported from several countries around the world. The disease that was silent for nearly 32 years re-emerged in the October 2005 outbreak in India that is still ongoing. The incubation period ranges from 3 to 12 days. The onset is usually abrupt and the acute stage is characterized by sudden onset with high-grade fever, severe arthralgias, myalgias, and skin rash. Swollen tender joints and crippling arthritis are usually evident. In the chronic stage, relapses that include sensation of fever, asthenia, exacerbation of arthralgias, inflammatory polyarthritis, and stiffness may be evident. Neurological, ocular, and mucocutaneous manifestations have also been described. Chronic arthritis may develop in about 15% of the patients. Viral culture is the gold standard for the diagnosis of Chikungunya fever. Reverse transcription polymerase chain reaction and real-time loop-mediated isothermal amplification have also been found to be useful. Serodiagnostic methods for the detection of immunoglobulin M and immunoglobulin G antibodies against Chikungunya virus are more frequently used. Chikungunya is a self-limiting disease; however, severe manifestations such as meningoencephalitis, fulminant hepatitis, and bleeding manifestations may sometimes be life-threatening. Treatment is symptomatic and supportive. Prevention by educating the community and public health officials, vector control measures appear to be the best approach at controlling Chikungunya fever as no commercially available vaccine is available for public use in India for this condition presently.

  3. Minireview: Multiomic candidate biomarkers for clinical manifestations of sickle cell severity: Early steps to precision medicine

    PubMed Central

    Pace, Betty S; Hansen, Kirk C; D’alessandro, Angelo; Xia, Yang; Daescu, Ovidiu; Glatt, Stephen J

    2016-01-01

    In this review, we provide a description of those candidate biomarkers which have been demonstrated by multiple-omics approaches to vary in correlation with specific clinical manifestations of sickle cell severity. We believe that future clinical analyses of severity phenotype will require a multiomic analysis, or an omics stack approach, which includes integrated interactomics. It will also require the analysis of big data sets. These candidate biomarkers, whether they are individual or panels of functionally linked markers, will require future validation in large prospective and retrospective clinical studies. Once validated, the hope is that informative biomarkers will be used for the identification of individuals most likely to experience severe complications, and thereby be applied for the design of patient-specific therapeutic approaches and response to treatment. This would be the beginning of precision medicine for sickle cell disease. PMID:27022133

  4. Maxillofacial manifestations of Langerhans cell histiocytosis: a clinical and therapeutic analysis of 10 patients.

    PubMed

    Eckardt, A; Schultze, A

    2003-10-01

    The definition of Langerhans cell histiocytosis (formerly known as histiocytosis X) includes the clinical syndromes Hand-Schueller-Christian syndrome, Abt-Letterer-Siwe syndrome, and eosinophilic granuloma. The paper gives an overview of current diagnostic and treatment strategies of LCH. Furthermore, records and clinical data of 10 patients with LCH were evaluated retrospectively. Patients' age ranged from 13 years to 42 years. The mandible was more frequently involved than the maxilla. Three patients (30%) had systemic manifestations of LCH in addition to their oral lesions. The longest follow-up period was 12 years. During follow-up six patients (60%) developed recurrent LCH and received adjuvant chemo- or radiation therapy. For solitary bone lesions, surgical curettage is the recommended treatment. Those patients with multi-organ involvement or recurrent LCH should be included into clinical trials initiated by the Histiocyte Society.

  5. Orofacial manifestations in HIV positive children attending Mildmay Clinic in Uganda.

    PubMed

    Nabbanja, Juliet; Gitta, Sheba; Peterson, Stefan; Rwenyonyi, Charles Mugisha

    2013-01-01

    The aim of this study was to assess the orofacial manifestations and their influence on oral function in human immunodeficiency virus (HIV) positive children attending Mildmay Clinic in Uganda. This was a cross-sectional study based on clinical examination, medical records and a structured questionnaire of 368 children aged between 1.5 and 17 years. The clinical examination for dental caries was based on decayed, extracted and filled teeth (deft) and decayed, missing and filled teeth (DMFT) indices as defined by World Health Organisation. The soft tissue orofacial lesions were assessed using the classification and diagnostic criteria as described by the Collaborative Workgroup on the Oral Manifestations of Pediatric HIV Infections. Approximately 67.4% of the children were on highly active antiretroviral therapy (HAART). The majority (77.4%) of the children had at least one orofacial lesion associated with HIV, pseudomembranous candidiasis being the most prevalent. Overall, 61.7% of the children with orofacial lesions reported at least one form of discomfort in the mouth. Discomfort was particularly associated with swallowing. The prevalence of orofacial lesions was significantly higher in children with poor oral hygiene and lower in those on HAART than in their respective counterparts. The CD4+ cell count, age and gender of the children did not significantly influence the distribution of orofacial lesions. The mean deft and DMFT scores were 11.8 and 2.7, respectively. The d- and D-components contributed 54.7 and 42.1%, respectively. Consumption of sugary snacks was directly associated with dental caries. Despite these children attending an HIV care centre of excellence, they have a high prevalence of orofacial manifestations associated with HIV. The majority of the children experienced discomfort in the oral cavity, particularly during swallowing.

  6. [The clinical manifestations of Sjögren's syndrome in children].

    PubMed

    Tomiita, M; Kohno, Y; Honma, K; Aoyagi, M; Katsuki, T; Hoshioka, A; Saito, K; Shimojo, N; Kuroda, K; Tanabe, E

    1994-10-01

    Sjögren's syndrome (SS) is thought to be uncommon in children. We studied the clinical manifestations and laboratory findings of 12 pediatric patients with SS, all of children did not have sicca symptoms but have lymphocytic infiltration of salivary glands, abnormal sialograms or abnormal results of scintigraphy compatible with typical SS. Seven cases had primary SS and five were secondary SS and had other autoimmune disorders (three cases with systemic lupus erythematosus, one case with dermatomyositis, and the other with mixed connective tissue disease). All patients were female. The mean age at onset of symptoms, including other autoimmune manifestations, was 12.2 years (range 9-15 years). The initial symptoms were some systemic manifestations (fever, exanthema, arthralgia, etc.) and various autoimmune phenomena (butterfly rash, Raynaud's phenomenon, proteinuria, weakness of muscles, etc.). On the other hand, no patients complained sicca symptoms. Laboratory studies in our patients revealed elevated levels of IgG (92%), antinuclear antibody (92%), rheumatoid factor (58%), anti-SS-A antibody (75%). These findings were similar to those found in adult patients with sicca symptoms previously reported in literature. From these studies, we suggest that lip biopsy, sialography and/or salivary gland's scintigraphy should be carried out in patients who had abnormal laboratory findings as mentioned above, irrespective of absence of sicca symptoms, in order to diagnose SS at early period.

  7. Clinical spectrum of cutaneous manifestations in renal and renopancreatic recipients in two centers in Uruguay.

    PubMed

    Dufrechou, L; Nin, M; Curi, L; Larre Borges, P; Martínez Asuaga, M; Noboa, O; Orihuela, S; González-Martínez, F; Larre Borges, A

    2014-11-01

    Uruguay, with a total population of 3,345,000 inhabitants, is the Latin American country with the second highest number of renal replacement therapies. Long-term immunosuppressant therapy is essential for graft survival but results in reduced immunosurveillance, leading to an increased risk of complications. A variety of dermatological manifestations and a large increase in nonmelanoma skin cancers have been reported in this population. The purpose of this study was to evaluate the frequency and clinical spectrum of cutaneous manifestations in renal and renopancreatic recipients in 2 reference centers in Uruguay. Two hundred and six renal or renopancreatic recipients between 21 and 77 years old were evaluated between September 2009 and September 2011. A total of 206 dermatoses were observed; 60% of the patients had at least 1 cutaneous manifestation. The most frequent dermatoses were cutaneous side effects due to immunosuppressive treatment (40.6%), followed by infections (26.1%), miscellaneous causes (18.9%), and malignant and premalignant lesions (14.4%). Transplant recipients represent a high-risk dermatological population. Physicians in transplant units should be aware of the importance of dermatological screening in order to promote early detection of skin cancer.

  8. Unusual late clinical manifestation of Bland-White-Garland syndrome as sudden cardiac death survival.

    PubMed

    Plank, Fabian; Feuchtner, Gudrun; Chevtchik, Orest; Mair, Johannes

    2015-03-01

    In this report, we present an extremely late clinical manifestation of Bland-White-Garland syndrome in a 53-year old woman who was ttransferred to our hospital after successful cardiopulmonary resuscitation. Coronary angiography revealed a dilated right coronary artery giving rise to multiple dominant septal collaterals to the left coronary artery (LCA) which arose from the pulmonary trunk (left to right shunt 0.15). Cardiac computed tomography identified a retropulmonary course of the LCA. The patient underwent cardiac surgery with LCA occlusion and triple coronary bypass grafting.

  9. A case of Bloom syndrome with uncommon clinical manifestations confirmed on genetic testing.

    PubMed

    Jian-Bing, Wu; Cheng-Rang, Li; Yi-Ping, Ma; Nan, Sheng; Hui, Li; Lin, Lin

    2016-02-01

    Bloom syndrome, a rare autosomal-recessive disorder, characteristically presents with photosensitivity, telangiectatic facial erythema, and growth deficiency. We present a case of Bloom syndrome with uncommon clinical manifestations including alopecia areata, eyebrow hair loss, flat nose, reticular pigmentation, and short sharpened distal phalanges with fingernails that were wider than they were long. We detected the Bloom syndrome gene, BLM, which is one of the members of the RecQ family of DNA helicases, and found changes in 2 heterozygous nucleotide sites in the patient as well as her father and mother.

  10. Venomous and poisonous arthropods: identification, clinical manifestations of envenomation, and treatments used in human injuries.

    PubMed

    Haddad Junior, Vidal; Amorim, Paulo Cezar Haddad de; Haddad Junior, William Teixeira; Cardoso, João Luiz Costa

    2015-01-01

    This review presents the main species of venomous and poisonous arthropods, with commentary on the clinical manifestations provoked by the toxins and therapeutic measures used to treat human envenomations. The groups of arthopods discussed include the class Arachnida (spiders and scorpions, which are responsible for many injuries reported worldwide, including Brazil); the subphylum Myriapoda, with the classes Chilopoda and Diplopoda (centipedes and millipedes); and the subphylum Hexapoda, with the class Insecta and the orders Coleoptera (beetles), Hemiptera (stink bugs, giant water bugs, and cicadas), Hymenoptera (ants, wasps, and bees), and Lepidoptera (butterflies and moths).

  11. Pulmonary edema following scorpion envenomation: mechanisms, clinical manifestations, diagnosis and treatment.

    PubMed

    Bahloul, Mabrouk; Chaari, Anis; Dammak, Hassen; Samet, Mohamed; Chtara, Kamilia; Chelly, Hedi; Ben Hamida, Chokri; Kallel, Hatem; Bouaziz, Mounir

    2013-01-10

    Scorpion envenomation is common in tropical and subtropical regions. Cardio-respiratory manifestations, mainly cardiogenic shock and pulmonary edema, are the leading causes of death after scorpion envenomation. The mechanism of pulmonary edema remains unclear and contradictory conclusions were published. However, most publications confirm that pulmonary edema has been attributed to acute left ventricular failure. Cardiac failure can result from massive release of catecholamines, myocardial damage induced by the venom or myocardial ischemia. Factors usually associated with the diagnosis of pulmonary edema were young age, tachypnea, agitation, sweating, or the presence of high plasma protein concentrations. Treatment of scorpion envenomation has two components: antivenom administration and supportive care. The latter mainly targets hemodynamic impairment and cardiogenic pulmonary edema. In Latin America, and India, the use of Prazosin is recommended for treatment of pulmonary edema because pulmonary edema is associated with arterial hypertension. However, in North Africa, scorpion leads to cardiac failure with systolic dysfunction with normal vascular resistance and dobutamine was recommended. Dobutamine infusion should be used as soon as we have enough evidence suggesting the presence of pulmonary edema, since it has been demonstrated that scorpion envenomation can result in pulmonary edema secondary to acute left ventricular failure. In severe cases, mechanical ventilation can be required.

  12. Inflammation and Rupture of a Congenital Pericardial Cyst Manifesting Itself as an Acute Chest Pain Syndrome

    PubMed Central

    Cheong, Benjamin Y.C.; Lufschanowski, Roberto

    2016-01-01

    We present the case of a 63-year-old woman with a remote history of supraventricular tachycardia and hyperlipidemia, who presented with recurrent episodes of acute-onset chest pain. An electrocardiogram showed no evidence of acute coronary syndrome. A chest radiograph revealed a prominent right-sided heart border. A suspected congenital pericardial cyst was identified on a computed tomographic chest scan, and stranding was noted around the cyst. The patient was treated with nonsteroidal anti-inflammatory drugs, and the pain initially abated. Another flare-up was treated similarly. Cardiac magnetic resonance imaging was then performed after symptoms had resolved, and no evidence of the cyst was seen. The suspected cause of the patient's chest pain was acute inflammation of a congenital pericardial cyst with subsequent rupture and resolution of symptoms. PMID:28100978

  13. Hepatitis E in Qatar imported by expatriate workers from Nepal: epidemiological characteristics and clinical manifestations.

    PubMed

    Ibrahim, Abdulsalam Saif; Alkhal, Abdulatif; Jacob, Joyal; Ghadban, Wisam; Almarri, Ajayeb

    2009-06-01

    Prompted by cases of acute hepatitis in expatriate workers presenting at Alkhor Hospital, Qatar, a limited prospective observational study was conducted from July 2005 to June 2006 to determine the epidemiological and clinical features of patients (predominantly Nepalese) presenting with acute hepatitis. Countrywide during that period samples from 86 Nepalese presenting at different centers were found to be anti-HEV IgG positive and 50 of these were also positive for anti-HEV IgM. Fifty-eight of those Nepalese were seen and treated at Alkhor Hospital and of them 43 were confirmed as cases of acute HEV, being positive for both anti-HEV IgM and IgG. The remaining 15 were diagnosed as probable cases of acute HEV on the basis of clinical and epidemiological similarity. It seems likely that transit in Kathmandu in reportedly unsanitary conditions was the focus of infection. In some of those examined at Alkhor, ultrasound detected a thickened gallbladder wall in 30 of 39 (76.9%) with two cases having clinical acalcular cholecystitis. Higher levels of alanine aminotransferase and aspartate aminotransferase were associated with severe disease and derangement in coagulation. On the available evidence hepatitis E was imported by expatriate workers and it is clear that medical screening of these workers pre- and post-arrival must be improved to prevent further outbreaks. It is also essential that health care workers in Qatar are made aware of this ongoing problem of imported HEV and understand the variable presentation of the condition.

  14. [Clinical manifestations of "Hunger Disease" among children in the ghettos during the Holocaust].

    PubMed

    Hercshlag-Elkayam, Orit; Even, Lea; Shasha, Shaul M

    2003-05-01

    The harsh life in the ghettos were characterized by overcrowding, shortage of supplies (e.g. money, sanitation, medications), poor personal hygiene, inclement weather and exhaustion. Under these conditions, morbidity was mainly due to infectious diseases, both endemic and epidemic outbreaks with a high mortality rate. The dominant feature was hunger. Daily caloric allowance was 300-800, and in extreme cases (i.e. Warsaw ghetto) it was only 200 calories. The food was lacking important nutrients (e.g. vitamins, trace elements) leading to protean clinical expression, starvation and death. The clinical manifestations of starvation were referred to as "the Hunger Disease", which became the subject of research by the medical doctors in the ghettos, mainly in the Warsaw ghetto in which a thorough documentation and research were performed. The first victims of hunger were children. First they failed to thrive physically and later mentally. Like their elders, they lost weight, but later growth stopped and their developmental milestones were lost with the loss of curiosity and motivation to play. The mortality rate among babies and infants was 100%, as was described by the ghetto doctors: "when the elder children got sick, the small ones were already dead...". In the last weeks of the ghettos there were no children seen in the streets. In this article the environmental conditions and daily life of children in the ghettos are reviewed, and the manifestations of "Hunger Disease" among them is scrutinized.

  15. Oral manifestations of HIV/AIDS in clients attending TASO clinics in Uganda.

    PubMed

    Tirwomwe, J F; Rwenyonyi, C M; Muwazi, L M; Besigye, B; Mboli, F

    2007-09-01

    The objective of the study is to establish the prevalence of oral manifestations and their influence on oral functions. A total of 514 subjects aged 18 to 58 years (mean 42 years) were randomly recruited from five The AIDS Support Organization (TASO) clinics in Uganda. They were clinically examined for oral lesions under field conditions by four trained dentists based on World Health Organization criteria. Women constituted 74.5% of the study population. Oral manifestations were recorded in 72% of the subjects, out of which 70% had candidiasis of pseudomembranous, erythematous, and angular cheilitis variants. Non-Hodgkin's lymphoma, atypical ulcers, necrotizing periodontitis, and hairy leucoplakia were least frequently observed in the subjects. Of those who had oral lesions (n = 370), 68.4% had some form of discomfort in the mouth. Tooth brushing, chewing, and swallowing were frequently associated with discomfort. Reported forms of discomfort were dry mouth, increased salivation, and burning sensation especially on taking salty and spicy foods or acidic drinks. Only 8.5% (n = 44) of the subjects were taking medications specifically for oral lesions, which included antifungal, antiviral, and antibacterial agents. None of the subjects were on antiretroviral therapy. Oral lesions associated with human immunodeficiency virus/acquired immunodeficiency syndrome in TASO clients is a major public health problem requiring education in recognition and appropriate management.

  16. Clinical manifestations and etiology of renal stones in children less than 14 years age.

    PubMed

    Sepahi, Mohsen Akhavan; Heidari, Akram; Shajari, Ahmad

    2010-01-01

    Urolithiasis is one of the commonest problems in pediatric nephrology. Prevalence of urolithiasis in pediatric patients is increasing. The purpose was to properly diagnose and treat with the special attention to the risk factors. This study is case-series and was performed on 100 pediatric patients for evaluation of clinical manifestation and etiology of renal stone in Qom. Hundred Children, fewer than 14 years old with mean age of 3.32 years, were included (54% male). Etiology of urolithiasis in 5% was unclear. Metabolic disorders found in patients were mainly: Hypocitraturia in 54, hyperoxaluria in 14, hyperuricosuria in 25, cystinuria in 6, hypercalciuria in 28 and phosphaturia in 8 patients. The main clinical presentation was fever, pain, irritability, dysuria and hematuria. Family history of urolithiasis was found in 23% of patients and 54% presented with urinary tract infection (UTI). We conclude that majority of patients were symptomatic and hypocitraturia was the commenest risk factor among others.

  17. Mycoses of implantation in Latin America: an overview of epidemiology, clinical manifestations, diagnosis and treatment.

    PubMed

    Queiroz-Telles, Flavio; Nucci, Marcio; Colombo, Arnaldo Lopes; Tobón, Angela; Restrepo, Angela

    2011-04-01

    Implantation or subcutaneous mycoses are a frequent health problem in Latin American countries and other tropical and subtropical areas. Although such infections rarely cause disseminated or invasive disease, they have an important impact on public health, and timely diagnosis and appropriate treatment remain important. Although some implantation mycoses are found in immunocompromised persons, the immunocompetent population is the principal target in Latin America. Most etiologic agents are found in soil, vegetation, and decaying matter in tropical, subtropical, and humid environments and infection is commonly the result of penetrating injury. Infections primarily occur (1) among low socioeconomic groups, (2) among those living in rural areas or involved in farming, hunting, or other outdoor activities, and (3) particularly among adult men. This review focuses on the epidemiology of the most clinically significant implantation mycoses in Latin America, i.e., sporotrichosis, eumycetoma, chromoblastomycosis, subcutaneous phaeohyphomycosis, subcutaneous zygomycosis, and lacaziosis. Main epidemiologic findings, clinical manifestations, diagnosis, and treatment options are also discussed.

  18. Clinical review of pulmonary manifestations of IgG4-related disease.

    PubMed

    Campbell, Sabrina N; Rubio, Edmundo; Loschner, A Lukas

    2014-11-01

    IgG4-related disease (IgG4-RD) is a recently recognized systemic disease characterized by tumefactive lesions in various organ systems. The list of organs that can be involved continues to expand, and recently computed tomography (CT) descriptions of the pulmonary lesions found in the disease have been described. The clinical symptoms are nonspecific and may include cough, dyspnea, chest pain, and fever. The appropriate clinical presentation along with elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and storiform fibrosis is consistent with the disease. Steroids are used to treat this disease in addition to immunosupressives such as cyclosporine or rituxumab for steroid refractory disease. The pulmonary manifestations and imaging features can often mimic malignancy, and as such knowledge of the diagnostic, clinicopathologic, and radiographic features of the disease is required in order to provide appropriate diagnostic workup and treatment.

  19. HUMAN GIARDIASIS IN MALAYSIA: CORRELATION BETWEEN THE PRESENCE OF CLINICAL MANIFESTATION AND GIARDIA INTESTINALIS ASSEMBLAGE.

    PubMed

    Anuar, Tengku Shahrul; Moktar, Norhayati; Salleh, Fatmah Md; Al-Mekhlafi, Hesham M

    2015-09-01

    Clinical manifestations of giardiasis vary from asymptomatic infection to chronic diarrhea. A total of 611 stool samples from Aboriginal participants residing in Jelebu, Gerik and Temerloh States, Malaysia, ages 2 to 74 years were screened for Giardia intestinalis using microscopic examination and sequence analysis of a fragment of nested-PCR amplified triosephosphate isomerase (tpi) gene. Demographic data was collected through a structured questionnaire. tpi was successfully amplified from 98/110 samples microscopically positive for G. intestinalis, with 62 and 36 belonging to assemblage A and B, respectively. There is a significant correlation between assemblage A and symptomatic infection only in participants of < 15 years of age. In the other age group, host factors may have more effects on the presence of clinical signs and symptoms than G. intestinalis assemblage types.

  20. Pathogenesis and clinical presentation of acute heart failure.

    PubMed

    Ponikowski, Piotr; Jankowska, Ewa A

    2015-04-01

    Acute heart failure constitutes a heterogeneous clinical syndrome, whose pathophysiology is complex and not completely understood. Given the diversity of clinical presentations, several different pathophysiological mechanisms along with factors triggering circulatory decompensation are involved. This article discusses the available evidence on the pathophysiological phenomena attributed or/and associated with episodes of acute heart failure and describes different clinical profiles, which, from a clinical perspective, constitute a key element for therapeutic decision-making.

  1. Spontaneous Dissection of Right Coronary Artery Manifested with Acute Myocardial Infarction

    PubMed Central

    Paraskevaidis, Stelios; Theofilogiannakos, Efstratios K; Chatzizisis, Yiannis S; Mantziari, Lilian; Economou, Fotis; Ziakas, Antonios; Hadjimiltiades, Stavros; Styliadis, Ioannis H

    2010-01-01

    Spontaneous coronary artery dissection is a rare cause of acute ischemic coronary events and sudden cardiac death. It usually occurs in young women without traditional risk factors for coronary artery disease during pregnancy or postpartum period. However, it has also been reported in patients with atherosclerotic coronary disease. We present a case of spontaneous right coronary artery dissection in a 48-year male with recent myocardial infarction and previous percutaneous coronary intervention. PMID:21127744

  2. Clinical and immunological relevance of anti-neuronal antibodies in celiac disease with neurological manifestations

    PubMed Central

    Caio, Giacomo; Giorgio, Roberto De; Venturi, Alessandro; Giancola, Fiorella; Latorre, Rocco; Boschetti, Elisa; Serra, Mauro; Ruggeri, Eugenio; Volta, Umberto

    2015-01-01

    Aim: To assess anti-neuronal antibodies (NA) prevalence and their correlation with neurological disorders and bowel habits in celiac disease (CD) patients. Background: Neurological manifestations are estimated to occur in about 10% of celiac disease patients and NA to central nervous system (CNS) and enteric nervous system (ENS) are found in a significant proportion of them. Little is known about the clinical and immunological features in CD patients with neurological manifestations. Patients and methods: NA to CNS and ENS were investigated in 106 CD patients and in 60 controls with autoimmune disorders by indirect immunofluorescence on rat / primate cerebellar cortex and intestinal (small and large bowel) sections. Results: IgG NA to CNS (titer 1:50 - 1:400) were positive in 23 celiacs (21%), being more frequently detected in those with neurological disorders that in those without neurological dysfunction (49% vs. 8%, P< 0.0001). Of the 26 celiacs (24%) with IgG NA to ENS, 11 out of 12 with an antibody titer > 1:200 had severe constipation. Only one patient with cerebellar ataxia and intestinal sub-occlusion was positive for NA to CNS and ENS. NA to CNS and ENS were found in 7% and 5% of controls, respectively. Conclusion: In CD the positivity of NA to CNS can be regarded as a marker of neurological manifestations. High titer NA to ENS are associated with severe constipation. The demonstration of NA to CNS and ENS suggests an immune-mediated pathogenesis leading to central neural impairment as well as gut dysfunction (hence constipation), respectively. PMID:25926940

  3. Skin Manifestations of Insulin Resistance: From a Biochemical Stance to a Clinical Diagnosis and Management.

    PubMed

    González-Saldivar, Gloria; Rodríguez-Gutiérrez, René; Ocampo-Candiani, Jorge; González-González, José Gerardo; Gómez-Flores, Minerva

    2017-03-01

    Worldwide, more than 1.9 billion adults are overweight, and around 600 million people suffer from obesity. Similarly, ~382 million individuals live with diabetes, and 40-50% of the global population is labeled at "high risk" (i.e., prediabetes). The impact of these two chronic conditions relies not only on the burden of illnesses per se (i.e., associated increased morbidity and mortality), but also on their increased cost, burden of treatment, and decreased health-related quality of life. For this review a comprehensive search in several databases including PubMed (MEDLINE), Ovid EMBASE, Web of Science, and Scopus was conducted. In both diabetes and obesity, genetic, epigenetic, and environmental factors overlap and are inclusive rather than exclusive. De facto, 70-80% of the patients with obesity and virtually every patient with type 2 diabetes have insulin resistance. Insulin resistance is a well-known pathophysiologic factor in the development of type 2 diabetes, characteristically appearing years before its diagnosis. The gold standard for insulin resistance diagnosis (the euglycemic insulin clamp) is a complex, invasive, costly, and hence unfeasible test to implement in clinical practice. Likewise, laboratory measures and derived indexes [e.g., homeostasis model assessment of insulin resistance (HOMA-IR-)] are indirect, imprecise, and not highly accurate and reproducible tests. However, skin manifestations of insulin resistance (e.g., acrochordons, acanthosis nigricans, androgenetic alopecia, acne, hirsutism) offer a reliable, straightforward, and real-time way to detect insulin resistance. The objective of this review is to aid clinicians in recognizing skin manifestations of insulin resistance. Diagnosing these skin manifestations accurately may cascade positively in the patient's health by triggering an adequate metabolic evaluation, a timely treatment or referral with the ultimate objective of decreasing diabetes and obesity burden, and improving the

  4. Membrane lipidomics in schizophrenia patients: a correlational study with clinical and cognitive manifestations

    PubMed Central

    Tessier, C; Sweers, K; Frajerman, A; Bergaoui, H; Ferreri, F; Delva, C; Lapidus, N; Lamaziere, A; Roiser, J P; De Hert, M; Nuss, P

    2016-01-01

    Schizophrenia is a severe mental condition in which several lipid abnormalities—either structural or metabolic—have been described. We tested the hypothesis that an abnormality in membrane lipid composition may contribute to aberrant dopamine signaling, and thereby symptoms and cognitive impairment, in schizophrenia (SCZ) patients. Antipsychotic-medicated and clinically stable SCZ outpatients (n=74) were compared with matched healthy subjects (HC, n=40). A lipidomic analysis was performed in red blood cell (RBC) membranes examining the major phospholipid (PL) classes and their associated fatty acids (FAs). Clinical manifestations were examined using the positive and negative syndrome scale (PANSS). Cognitive function was assessed using the Continuous Performance Test, Salience Attribution Test and Wisconsin Card Sorting Test. Sphingomyelin (SM) percentage was the lipid abnormality most robustly associated with a schizophrenia diagnosis. Two groups of patients were defined. The first group (SCZ c/SM−) is characterized by a low SM membrane content. In this group, all other PL classes, plasmalogen and key polyunsaturated FAs known to be involved in brain function, were significantly modified, identifying a very specific membrane lipid cluster. The second patient group (SCZ c/SM+) was similar to HCs in terms of RBC membrane SM composition. Compared with SCZ c/SM+, SCZ c/SM− patients were characterized by significantly more severe PANSS total, positive, disorganized/cognitive and excited psychopathology. Cognitive performance was also significantly poorer in this subgroup. These data show that a specific RBC membrane lipid cluster is associated with clinical and cognitive manifestations of dopamine dysfunction in schizophrenia patients. We speculate that this membrane lipid abnormality influences presynaptic dopamine signaling. PMID:27701405

  5. Advances in clinical determinants and neurological manifestations of B vitamin deficiency in adults.

    PubMed

    Sechi, GianPietro; Sechi, Elia; Fois, Chiara; Kumar, Neeraj

    2016-05-01

    B vitamin deficiency is a leading cause of neurological impairment and disability throughout the world. Multiple B vitamin deficiencies often coexist, and thus an understanding of the complex relationships between the different biochemical pathways regulated in the brain by these vitamins may facilitate prompter diagnosis and improved treatment. Particular populations at risk for multiple B vitamin deficiencies include the elderly, people with alcoholism, patients with heart failure, patients with recent obesity surgery, and vegetarians/vegans. Recently, new clinical settings that predispose individuals to B vitamin deficiency have been highlighted. Moreover, other data indicate a possible pathogenetic role of subclinical chronic B vitamin deficiency in neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis. In light of these findings, this review examines the clinical manifestations of B vitamin deficiency and the effect of B vitamin deficiency on the adult nervous system. The interrelationships of multiple B vitamin deficiencies are emphasized, along with the clinical phenotypes related to B vitamin deficiencies. Recent advances in the clinical determinants and diagnostic clues of B vitamin deficiency, as well as the suggested therapies for B vitamin disorders, are described.

  6. Prediction of manifest Huntington disease with clinical and imaging measures: A 12-year prospective observational study

    PubMed Central

    Paulsen, Jane S.; Long, Jeffrey D.; Ross, Christopher A.; Harrington, Deborah L.; Erwin, Cheryl J.; Williams, Janet K.; Westervelt, Holly James; Johnson, Hans J.; Aylward, Elizabeth H.; Zhang, Ying; Bockholt, H. Jeremy; Barker, Roger A.

    2015-01-01

    BACKGROUND Although correlation between cytosine-adenine-guanine (CAG) repeat length and age of Huntington disease (HD) onset is well known, improved prediction of onset would be advantageous for clinical trial design and prognostic counseling. We compared genetic, demographic, motor, cognitive, psychiatric, functional and imaging measures for tracking progression and predicting conversion to manifest HD. METHODS N=1078 research participants with the gene mutation for HD, but without a rating of 4 on the Diagnostic Confidence Level (DCL) following administration of the 15-item motor assessment of the Unified Huntington’s Disease Rating Scale. Participants were from 33 world wide sites and followed for up to 12 years (mean=5, SD=3·3) over the period 2001–2013. A subset of 225 participants prospectively converted to manifest HD according to the DCL (“meets the operational definition of the unequivocal presence of an otherwise unexplained extrapyramidal movement disorder in a subject at risk for HD” with ≥99% confidence). Joint modeling of longitudinal and survival data was used to examine the extent to which baseline and change of 40 variables analyzed separately was predictive of CAG-adjusted age at motor diagnosis. FINDINGS Cross-sectional and longitudinal clinical and imaging measures were significant predictors of motor diagnosis beyond CAG repeat length and age. The strongest predictors in the top three phenotypic domains were total motor score (motor), putamen volume (imaging), and Stroop word test (cognitive). A one standard deviation (SD) difference in total motor score increased the risk of a motor diagnosis by 3·1 times (95% CI=[2·3,4·2]), one SD loss in putamen volume increased risk by 3·3 times ([2·4,4·7]) and one SD cognitive decline increased risk by 2·3 ([1·9,2·9]). INTERPRETATION Prediction of HD diagnosis can be considerably improved beyond that obtained by CAG repeat length and age alone. Such knowledge about potential predictors

  7. Clinical Manifestations Associated with Overweight/Obesity in Puerto Ricans with Fibromyalgia Syndrome

    PubMed Central

    Fred-Jiménez, Ruth M.; Arroyo-Ávila, Mariangelí; Mayor, Ángel M.; Ríos, Grissel; Vilá, Luis M.

    2016-01-01

    Objective. To determine the clinical manifestations associated with overweight/obesity in Hispanics from Puerto Rico with fibromyalgia syndrome (FMS). Methods. A cross-sectional study was performed in 144 patients with FMS (per American College of Rheumatology (ACR) classification criteria). Sociodemographic features, FMS-related symptoms, tender points (per ACR criteria), comorbidities, and FMS treatment were examined. BMI was calculated and patients were grouped into two categories: BMI ≤ 24.9 kg/m2 (nonoverweight/obese) and BMI ≥ 25 kg/m2 (overweight/obese). Bivariate and multivariate analyses were used to evaluate differences between the study groups. Results. The mean (standard deviation (SD)) age of patients was 50.2 (9.9) years; 95.1% were females and 75.7% were overweight/obese. In the bivariate analysis, overweight/obese patients were more likely to have self-reported memory impairment, anxiety, shortness of breath, and urinary frequency than nonoverweight/obese patients. In addition, the tender point count was higher in the overweight/obese group. In the logistic regression analyses, self-reported memory impairment and urinary frequency differences remained significant after adjusting for confounding variables. Conclusion. In this population of Puerto Ricans with FMS, overweight/obese patients experienced more FMS-related manifestations than nonoverweight/obese individuals. However, prospective studies are needed to confirm these associations and to elucidate if weight reduction interventions could favorably impact the severity of FMS. PMID:26885384

  8. The epigenomics of polycystic ovarian syndrome: from pathogenesis to clinical manifestations.

    PubMed

    Li, Shuxia; Zhu, Dongyi; Duan, Hongmei; Tan, Qihua

    2016-12-01

    Polycystic ovarian syndrome (PCOS) is a complex condition of ovarian dysfunction and metabolic abnormalities with widely varying clinical manifestations resulting from interference of the genome and the environment through integrative biological mechanisms with the emerging field of epigenetics offering an appealing tool for studying the nature and nurture of the disease. We review the current literature of epigenetic studies on PCOS from disease development to the association analysis of the DNA methylome and to exploratory studies on the molecular mechanisms of disease heterogeneity and comorbidity. Recent data based on profiling of the DNA methylome of PCOS in different tissues provided consistent molecular evidence in support of epidemiological findings on disease comorbidity suggesting a possible autoimmune basis in the pathogenesis of the disease. We show that the field of epigenetics and epigenomics could serve to link molecular regulatory mechanisms with disease development and disease manifestation which could contribute to PCOS prevention and treatment and eventually promote reproductive health in fertile age women. We summarize the up-to-date findings and discuss the implications of various studies and point to new avenues of research on PCOS in the rapidly developing field of epigenetics and epigenomics.

  9. Season of birth, clinical manifestations and Dexamethasone Suppression Test in unipolar major depression

    PubMed Central

    Fountoulakis, Konstantinos N; Iacovides, Apostolos; Karamouzis, Michael; Kaprinis, George S; Ierodiakonou, Charalambos

    2007-01-01

    Background Reports in the literature suggest that the season of birth might constitute a risk factor for the development of a major psychiatric disorder, possibly because of the effect environmental factors have during the second trimester of gestation. The aim of the current paper was to study the possible relationship of the season of birth and current clinical symptoms in unipolar major depression. Methods The study sample included 45 DSM-IV major depressive patients and 90 matched controls. The SCAN v. 2.0, Hamilton Depression Rating Scale (HDRS) and Hamilton Anxiety Scale (HAS) were used to assess symptomatology, and the 1 mg Dexamethasone Suppression Test (DST) was used to subcategorize patients. Results Depressed patients as a whole did not show differences in birth season from controls. However, those patients born during the spring manifested higher HDRS while those born during the summer manifested the lowest HAS scores. DST non-suppressors were almost exclusively (90%) likely to be born during autumn and winter. No effect from the season of birth was found concerning the current severity of suicidal ideation or attempts. Discussion The current study is the first in this area of research using modern and rigid diagnostic methodology and a biological marker (DST) to categorize patients. Its disadvantages are the lack of data concerning DST in controls and a relatively small size of patient sample. The results confirm the effect of seasonality of birth on patients suffering from specific types of depression. PMID:17683542

  10. Obesity as the initial manifestation of central nervous system relapse of acute lymphoblastic leukemia: case report and literature review.

    PubMed

    Zhang, Li-Dan; Li, Yan-Hong; Ke, Zhi-Yong; Huang, Li-Bin; Luo, Xue-Qun

    2012-01-01

    A 6-year-old boy with acute lymphoblastic leukemia in remission experienced hyperphagia, obesity, and emotional disorders. Cytomorphologic examination of cerebral spinal fluid (CSF) and cranial MRI did not help in differentiating between central nervous system leukemia (CNSL) and other CNS diseases including tuberculosis in this boy. Flow cytometric CSF analysis on repeated lumber puncture detected lymphoblasts, while microscopic CSF examination did not definitively show relapse disease. The diagnosis of CNSL was thus made and confirmed by the response to leukemia treatment. Obesity can be the first manifestation of CNSL and the diagnosis can be challenging. A combination of CSF cytomorphology, CSF flow cytometry, and cranial MRI can be useful in the diagnosis of the disease. Two mechanisms of CNSL-related obesity are discussed based on the literature review.

  11. Scorpion envenomation among children: clinical manifestations and outcome (analysis of 685 cases).

    PubMed

    Bahloul, Mabrouk; Chabchoub, Imen; Chaari, Anis; Chtara, Kamilia; Kallel, Hatem; Dammak, Hassen; Ksibi, Hichem; Chelly, Hedi; Rekik, Noureddine; Ben Hamida, Chokri; Bouaziz, Mounir

    2010-11-01

    Our objective was to characterize both epidemiologically and clinically manifestations after severe scorpion envenomation and to define simple factors indicative of poor prognosis in children. We performed a retrospective study over 13 years (1990-2002) in the medical intensive care unit (ICU) of a university hospital (Sfax-Tunisia). The diagnosis of scorpion envenomation was based on a history of scorpion sting. The medical records of 685 children aged less than 16 years who were admitted for a scorpion sting were analyzed. There were 558 patients (81.5%) in the grade III group (with cardiogenic shock and/or pulmonary edema or severe neurological manifestation [coma and/or convulsion]) and 127 patients (18.5%) in the grade II group (with systemic manifestations). In this study, 434 patients (63.4%) had a pulmonary edema, and 80 patients had a cardiogenic shock; neurological manifestations were observed in 580 patients (84.7%), 555 patients (81%) developed systemic inflammatory response syndrome (SIRS), and 552 patients (80.6%) developed multi-organ failure. By the end of the stay in the ICU, evolution was marked by the death in 61 patients (8.9%). A multivariate analysis found the following factors to be correlated with a poor outcome: coma with Glasgow coma score ≤ 8/15 (odds ratio [OR] = 1.3), pulmonary edema (OR = 2.3), and cardiogenic shock (OR = 1.7). In addition, a significant association was found between the development of SIRS and heart failure. Moreover, a temperature > 39°C was associated with the presence of pulmonary edema, with a sensitivity at 20.6%, a specificity at 94.4%, and a positive predictive value at 91.7%. Finally, blood sugar levels above 15 mmol/L were significantly associated with a heart failure. In children admitted for severe scorpion envenomation, coma with Glasgow coma score ≤ 8/15, pulmonary edema, and cardiogenic shock were associated with a poor outcome. The presence of SIRS, a temperature > 39°C, and blood sugar levels

  12. The Clinical Manifestations, Diagnosis and Management of Williams-Campbell Syndrome

    PubMed Central

    Noriega Aldave, Adrian Pedro; William Saliski, DO

    2014-01-01

    Williams-Campbell syndrome is a rare congenital syndrome characterized by the absence of cartilage in subsegmental bronchi leading to formation of bronchiectasis distal to the affected bronchi. The differential diagnosis of bronchiectasis is broad and the rarity of the disease poses a diagnostic and management challenge for clinicians. This present review aims to help the understanding of the clinical manifestations, pathophysiological features, diagnostic modalities, management and differential diagnosis of Williams-Campbell syndrome. A MedLine/PubMed search was performed identifying all relevant articles. No restrictions were used for publication dates. The author used the keywords “Williams-Campbell syndrome,” “non-cystic fibrosis bronchiectasis” and “congenital bronchiectasis” finding 503, 195 and 489 articles, respectively. PMID:25317385

  13. Differential Diagnoses of Overgrowth Syndromes: The Most Important Clinical and Radiological Disease Manifestations

    PubMed Central

    Lacerda, Letícia da Silva; Alves, Úrsula David; Zanier, José Fernando Cardona; Machado, Dequitier Carvalho; Camilo, Gustavo Bittencourt; Lopes, Agnaldo José

    2014-01-01

    Overgrowth syndromes comprise a heterogeneous group of diseases that are characterized by excessive tissue development. Some of these syndromes may be associated with dysfunction in the receptor tyrosine kinase (RTK)/PI3K/AKT pathway, which results in an increased expression of the insulin receptor. In the current review, four overgrowth syndromes were characterized (Proteus syndrome, Klippel-Trenaunay-Weber syndrome, Madelung's disease, and neurofibromatosis type I) and illustrated using cases from our institution. Because these syndromes have overlapping clinical manifestations and have no established genetic tests for their diagnosis, radiological methods are important contributors to the diagnosis of many of these syndromes. The correlation of genetic discoveries and molecular pathways that may contribute to the phenotypic expression is also of interest, as this may lead to potential therapeutic interventions. PMID:25009745

  14. Paraneoplastic syndromes in patients with laryngeal neuroendocrine carcinomas: clinical manifestations and prognostic significance.

    PubMed

    Ferlito, Alfio; Rinaldo, Alessandra; Bishop, Justin A; Hunt, Jennifer L; Vander Poorten, Vincent; Williams, Michelle D; Triantafyllou, Asterios; Devaney, Kenneth O; Gnepp, Douglas R; Kusafuka, Kimihide; Halmos, Gyorgy B; Westra, William H; Takes, Robert P; Thompson, Lester D R

    2016-03-01

    Paraneoplastic syndromes are associated with a variety of malignant neoplasms and are systemic and non-metastatic manifestations that develop in a minority of cancer patients. This review examines all published cases of paraneoplastic syndromes associated with neuroendocrine carcinomas of the larynx. There are a total of ten patients reported with paraneoplastic syndromes associated with laryngeal neuroendocrine carcinomas in the literature. Of these, nine died and the tenth is alive with liver metastases. There were five cases of small-cell neuroendocrine carcinoma, four cases of moderately differentiated neuroendocrine carcinoma, and one case of well-differentiated neuroendocrine carcinoma associated with paraneoplastic syndromes. As these syndromes have significant clinical relevance, physicians should be aware of the possible presence of paraneoplastic syndromes in the diagnostic process of patients with neuroendocrine carcinoma of the larynx.

  15. Testicular hemorrhage, necrosis, and vasculopathy: likely manifestations of intermittent torsion that clinically mimic a neoplasm.

    PubMed

    Kao, Chia-Sui; Zhang, Chen; Ulbright, Thomas M

    2014-01-01

    parenchyma in all 30 cases. Clinical follow-up in 20 patients (4 to 131 mo, mean 38 mo) showed no evidence of recurrence in the contralateral testis or later development of systemic vasculitis. The histologic findings were compared with those in 11 orchiectomies resected for clinical acute torsion. All clinical acute torsion cases showed both parenchymal and fibrinoid vascular necrosis, and 10 had hemorrhage/hematoma; they lacked vasculitis, interstitial inflammation, and chronic vascular changes. Testicular vasculopathy, characterized by acute and chronic vascular injury, commonly occurs in testes with parenchymal hemorrhage and necrosis that clinically mimic a tumor. It shares the acute features of recent torsion but also has findings indicative of chronic injury. Testicular vasculopathy is most likely a result of chronic intermittent torsion that leads to localized hemorrhage/necrosis and should be distinguished from cases of systemic vasculitis given the significantly different clinical implications.

  16. A Retrospective Study of Chinese Patients With Giant Cell Arteritis (GCA): Clinical Features and Factors Associated With Severe Ischemic Manifestations.

    PubMed

    Sun, Fei; Ma, Sha; Zheng, Wenjie; Tian, Xinping; Zeng, Xiaofeng

    2016-03-01

    A retrospective study was performed on 70 giant cell arteritis (GCA) patients in Peking Union Medical College Hospital (PUMCH). The aim of this study was to describe the clinical features of these Chinese GCA patients and explore the possible associated factors for severe ischemic manifestations. Medical charts of all patients were reviewed, and the demographic, clinical, and laboratory data were analyzed. The mean age at disease onset was 65.2 years old, and the ratio of male to female was 1:1. Fever and headache were most prominent symptoms at onset, which occurred in 51.4% and 30.0% of patients, respectively. Common manifestations at diagnosis were constitutional symptoms (85.7%), headache (68.8%), visual impairment (38.6%), jaw claudication (30%), scalp tenderness (30%), and concurrent polymyalgia rheumatic (27.1%). No significant difference in clinical manifestations between genders was observed. Comparisons between patients with and without severe ischemic manifestations including jaw claudication, permanent visual loss, or cerebrovascular accident had shown that fever and asthenia were significantly less frequent in patients with severe ischemic manifestations (P = 0.006 and 0.023, respectively), and the mean value of erythrocyte sedimentation rate (ESR) was significantly lower in patients with severe ischemic manifestations than patients without (P = 0.001). History of smoking was more frequent in patients with severe ischemic manifestations (P = 0.038). This is the largest group of GCA patients from China so far. When compared our data with patients reported in the literature, this series of GCA patients were younger and without female predominance. The clinical manifestations of patients in this report were similar to other studies except for a higher prevalence of constitutional symptoms. The results of this study indicated that lower systemic inflammatory response and the history of smoking might be associated with severe ischemic damages.

  17. Multiple clinical and biological autoimmune manifestations in 50 workers after occupational exposure to silica.

    PubMed Central

    Sanchez-Roman, J; Wichmann, I; Salaberri, J; Varela, J M; Nuñez-Roldan, A

    1993-01-01

    OBJECTIVES--A self referred group of four workers from a factory producing scouring powder with a high silica content showed a surprisingly high number of features compatible with a connective tissue disease. Further subjects working at the same factory were subsequently studied to evaluate the relation between this exposure and the development of autoimmune processes. METHODS--A total of 50 subjects (44 women, six men; mean (SD) age 43.7 (5.5) years; mean duration of employment 6.1 years) underwent a prospective study including clinical history and physical examination, an immunobiological study, HLA typing, radiological and functional oesophageal and respiratory examination, ophthalmological examination, and isotopic testing of salivary glands. RESULTS--Symptoms of a systemic illness were present in 32 (64%) subjects: six with Sjögren's syndrome; five with the criteria for systemic sclerosis; three with systemic lupus erythematosus (SLE); five with an 'overlap syndrome'; and 13 with undifferentiated findings not meeting the criteria for a defined disease. Antinuclear antibodies were present in 36 (72%) subjects; four had antibodies to native DNA, including two subjects with SLE, one with systemic sclerosis associated with secondary Sjögren's syndrome, and one with overlap syndrome. Anticentromere antibodies were not detected. The frequency of HLA-DR3 was increased in the clinically affected subjects, but did not reach statistical significance. CONCLUSIONS--This descriptive study emphasises the high probability of workers occupationally exposed to silica developing a multiple spectrum of clinical and serological autoimmune manifestations. PMID:8394065

  18. [Cough and hypoxemia as clinical manifestation of pulmonary alveolar proteinosis. Clinical case report].

    PubMed

    Nieto, Mary; Dicembrino, Manuela; Ferraz, Rubén; Romagnoli, Fernando; Giugno, Hilda; Ernst, Glenda; Siminovich, Monica; Botto, Hugo

    2016-06-01

    Alveolar proteinosis is a rare chronic lung disease, especially in children, characterized by abnormal accumulation of lipoproteins and derived surfactant in the intra-alveolar space that generates a severe reduction of gas exchange. Idiopathic presentation form constitutes over 90% of cases, a phenomenon associated with production of autoimmune antibodies directed at the receptor for granulocyte-macrophage colony-stimulating factor. A case of a girl of 5 years of age treated because of atypical pneumonia with unfavorable evolution due to persistent hypoxemia is presented. The diagnosis is obtained through pathologic examination of lung biopsy by thoracotomy, as treatment is carried out by 17bronchopulmonary bronchoscopy lavages and the patient evidences marked clinical improvement.

  19. Influenza C virus infection in military recruits--symptoms and clinical manifestation.

    PubMed

    Kauppila, Jaana; Rönkkö, Esa; Juvonen, Raija; Saukkoriipi, Annika; Saikku, Pekka; Bloigu, Aini; Vainio, Olli; Ziegler, Thedi

    2014-05-01

    Due to the lack of rapid diagnostic tests, clinical features of Influenza C virus infections are poorly characterized. Respiratory infections in military recruits in eastern Finland were monitored between July 2004 and December 2005 in order to study the epidemiology and clinical picture of infections caused by this virus. Blood samples were obtained at entry and at the end of the military service, and during each episode of respiratory infection to measure antibody responses against 10 viral and 2 bacterial pathogens. If possible, sputum samples were collected during the acute phase of respiratory infection episodes. Symptoms of the episodes were recorded for comparison of the clinical picture caused by various infectious agents. Infection with influenza C virus was detected in 38 of 892 young men during their service. The virus usually caused a mild upper respiratory tract infection. Most typical clinical features of influenza C virus infection were cough, rhinitis, and hoarseness. A striking difference to infections caused by influenza A virus was the lack of fever. Influenza C virus is an important cause of a respiratory tract infection in army conscripts. Infections with this virus are usually mild but can be complicated in some cases.

  20. Skin and Mucocutaneous Manifestations: Useful Clinical Predictors of HIV/AIDS

    PubMed Central

    Chopra, Shashi; Arora, Usha

    2012-01-01

    Background and Aims The HIV infection is associated with several dermatological conditions which may be the first pointer towards the existence of HIV. These may present with unusual and atypical manifestations in the course of the HIV infection. Keeping this in mind, the seroprevalence of HIV in these persons and the spectrum of the skin and the mucocutaneous lesions in the HIV positive patients was studied. Methods The current prospective study was conducted over a period of 3 years (2006-2008). A total of 604 persons who had any kind of skin and mucocutaneous infections were screened for the HIV infection as per the NACO guidelines after recording their clinical and epidemiological profiles. Results Out of the 604 patients who were screened, 90(14.90%) were seropositive for the HIV-I antibodies and none was positive for the HIV-2 antibodies. Seventy three point thirty three percent 73.33 of the seropositive patients were in the age group of 15-40 years, with a male-female ratio of 1:1.05. The heterosexual route was the most common mode of transmission (86.6%).A wide range of infectious and noninfectious lesions were observed. In the HIV seropositive patients, oral candidiasis (32.22%) was the most common infectious disease which was observed, followed by herpes zoster (13.33%), genital warts (7.77%) and genital herpes (6.66%). The most common noninfectious manifestation was seborrhoic dermatitis (8.88%), followed by pruritic papular eruptions (7.77%). Conclusion As there is a high prevalence of the HIV infection in patients who have skin and mucocutaneous disorders, the doctors, during the investigation of these patients, must have a high level of suspicion for the HIV infection in their mind. An early detection of HIV optimizes the chemoprophylaxis for many opportunistic mucocutaneous disorders. PMID:23373031

  1. Cutaneous manifestations of antiphospholipid syndrome: a review of the clinical features, diagnosis and management.

    PubMed

    Pinto-Almeida, Teresa; Caetano, Mónica; Sanches, Madalena; Selores, Manuela

    2013-01-01

    Antiphospholipid syndrome is a relatively recent systemic autoimmune disorder defined by thrombotic events and/or obstetric complications in the presence of persistent elevated antiphospholipid antibodies. It\\'s characterized by a wide spectrum of clinical presentations and virtually any organ system or tissue may be affected by the consequences of vascular occlusion. Diagnosis is sometimes difficult and although classification criteria have been published and revised there remain ongoing issues regarding nomenclature, expanding clinical features, laboratory tests and management and much still has to be done. Cutaneous manifestations are common and frequently the first sign of the disease. Although extremely diverse it\\'s important to know which dermatological findings should prompt consideration of antiphospholipid syndrome and the appropriate management for those patients. Much has been debated about when to consider antiphospholipid syndrome and consensus still does not exist, however in spite of being a diagnostic challenge clinicians should know when to look for antiphospholipid antibodies since an early diagnosis is important to prevent further and serious complications. In this article we focus on the cutaneous features that should raise suspicion on the presence of antiphospholipid syndrome and on the complex management of such patients. Many other dermatological signs related to this syndrome have been described in the literature but only occasionally and without consistency or statistic impact and therefore will not be considered here.

  2. Vancomycin-resistant enterococcal infections: epidemiology, clinical manifestations, and optimal management

    PubMed Central

    O’Driscoll, Tristan; Crank, Christopher W

    2015-01-01

    Since its discovery in England and France in 1986, vancomycin-resistant Enterococcus has increasingly become a major nosocomial pathogen worldwide. Enterococci are prolific colonizers, with tremendous genome plasticity and a propensity for persistence in hospital environments, allowing for increased transmission and the dissemination of resistance elements. Infections typically present in immunosuppressed patients who have received multiple courses of antibiotics in the past. Virulence is variable, and typical clinical manifestations include bacteremia, endocarditis, intra-abdominal and pelvic infections, urinary tract infections, skin and skin structure infections, and, rarely, central nervous system infections. As enterococci are common colonizers, careful consideration is needed before initiating targeted therapy, and source control is first priority. Current treatment options including linezolid, daptomycin, quinupristin/dalfopristin, and tigecycline have shown favorable activity against various vancomycin-resistant Enterococcus infections, but there is a lack of randomized controlled trials assessing their efficacy. Clearer distinctions in preferred therapies can be made based on adverse effects, drug interactions, and pharmacokinetic profiles. Although combination therapies and newer agents such as tedizolid, telavancin, dalbavancin, and oritavancin hold promise for the future treatment of vancomycin-resistant Enterococcus infections, further studies are needed to assess their possible clinical impact, especially in the treatment of serious infections. PMID:26244026

  3. Clinical and histopathological features of cutaneous manifestations of adult-onset Still disease.

    PubMed

    Santa, Erin; McFalls, Jeanne M; Sahu, Joya; Lee, Jason B

    2017-03-25

    Adult-onset Still disease (AOSD) is a rare autoinflammatory syndrome characterized by recurring fevers, arthralgia, and consistent laboratory abnormalities that include leukocytosis and hyperferritinemia. Skin findings accompany the disease in nearly 90% of the cases. Early reports described evanescent, pruritic, salmon-pink or urticarial lesions, referred to as the typical eruption of AOSD. Histopathologic findings consist of superficial perivascular dermatitis with varying number of interstitial neutrophils. Later reports described a more persistent rash that tended to be photodistributed, hyperpigmented, often in a linear configuration, sometimes in a rippled pattern, referred to as the atypical eruption of AOSD. The presence of individual necrotic keratinocytes in the upper spinous layer has been the consistent histopathologic finding. The persistent rash may not represent an atypical presentation of AOSD as recent reports indicate a high prevalence of the rash. Emerging data also suggest that patients with persistent eruption have a worse prognosis. The recognition of the clinical and histopathological findings of skin eruptions of AOSD may facilitate an earlier diagnosis, potentially improving disease outcome. Herein, clinical and histopathological features of cutaneous manifestation of AOSD in two Asian women are highlighted accompanied by relevant review of the disease.

  4. From Pathogenesis, Clinical Manifestation, and Diagnosis to Treatment: An Overview on Autoimmune Pancreatitis

    PubMed Central

    Cai, Ou

    2017-01-01

    Autoimmune pancreatitis (AIP) is a special type of chronic pancreatitis which is autoimmune mediated. The international consensus diagnostic criteria (ICDC) 2011 proposed two types of AIP: type I is associated with histological pattern of lymphoplasmacytic sclerosing pancreatitis (LPSP), characterized by serum IgG4 elevation, whereas type 2 is named idiopathic duct-centric pancreatitis (IDCP), with granulocytic epithelial lesion (GEL) and immunoglobulin G4 (IgG4) negative. The pathogenic mechanism is unclear now; based on genetic factors, disease specific or related antigens, innate and adaptive immunity may be involved. The most common clinical manifestations of AIP are obstructive jaundice and upper abdominal pain. The diagnosis can be made by a combination of parenchymal and ductal imaging, serum IgG4 concentrations, pancreatic histology, extrapancreatic disease, and glucocorticoid responsiveness according to ICDC 2011. Because of the clinical and imaging similarities with pancreatic cancer, general work-up should be done carefully to exclude pancreatic malignant tumor before empirical trial of glucocorticoid treatment. Glucocorticoid is the most common drug for AIP to induce remission, while there still exists controversy on steroid maintenance and treatment for relapse. Further studies should be done to identify more specific serum biomarkers for AIP, the pathogenic mechanisms, and the treatment for relapse. PMID:28197205

  5. The Clinical Manifestation and Genetic Evaluation in Patients with 45,X/46,XY Mosaicism.

    PubMed

    Wu, Qinghua; Wang, Cong; Shi, Huirong; Kong, Xiangdong; Ren, Shumin; Jiang, Miao

    2017-02-18

    45,X/46,XY mosaicism is a rare chromosomal abnormality and probably underdiagnosed. Although clinical and genetic analyses have been performed in some disorders of sexual development, there have been few studies focusing on the phenotype and genetic details of 45,X/46,XY mosaicism, especially in the Chinese population. The aim of this study was to describe the experience of our service in relation to 16 cases with 45,X/46,XY mosaicism. The age at the first evaluation of the patients ranged from 43 days to 30 years. Eight patients were reared as female and 8 as male. The main reasons for examination were primary amenorrhea, sterility, or ambiguous genitalia. Short stature was more common in female than in male patients. Two patients accepted gonadectomy due to tumor risk and none presented gonadal malignancy. The SRY gene was amplified positively in all of the patients. AZF gene microdeletions were present in 6 of 8 male patients, and all adult male patients had no sperm. No correlation has been found between clinical manifestations and the proportion of mosaic cells in peripheral blood. Our observations may permit a better management of people with 45,X/46,XY mosaicism.

  6. [Acute ischemia of an arm as an unusual manifestation of ergotism].

    PubMed

    Heinz, M; Theiss, W; Golder, W; Schömig, A

    1994-11-04

    A 27-year-old woman developed acute pain, pallor and feeling of cold in her left arm. She had been a smoker of 15-20 cigarettes daily since the age of 15 years, but had not previously had any serious illness. In addition to contraceptives she had had been taking one to several suppositories containing caffeine and ergotamine tartrate (2 mg) daily against migraine. Angiological examination 5 days after onset of symptoms discovered a weak brachial pulse low in the left upper arm, while ulnar and radial pulses were absent. All other pulses were normally palpable. Colour duplex sonography demonstrated occlusion of the brachial artery which angiographically was due to a 5 cm severe narrowing without thrombus, blood flowing distally via collaterals. No improvement was achieved by local injection of 100,000 IU urokinase, 0.5 mg nitroglycerin, 20 mg tolazoline and a 3-hour infusion of alprostadil. On infusion of 560 ml hydroxyethylstarch over 8 hours, 400 mg naftidrofuryl, therapeutic doses of heparin and abstinence from ergotamine (since admission) the vessel diameter increased by 50% within 23 hours and after a further 24 hours to almost 100% of the comparable arterial segment of the right arm while merely on heparin infusion.

  7. Three clinical cases of the DiGeorge syndrome manifested with the biliary system disease.

    PubMed

    Tabutsadze, T; Pachkoria, Kh; Atuashvili, G

    2007-11-01

    DiGeorge syndrome is a rare congenital disease that affects the baby's immune system. Its symptoms vary greatly between individuals but commonly include a history of recurrent infection, heart defects, and characteristic facial features. Few cases of DiGeorge syndrome have been reported in adults. The article describes rare (three cases of DiGeorge syndrome) in adults (18, 32 and 34 years old patients) in Georgia (Caucasus). In clinical practice DiGeorge syndrome may proceed under the course of gastroenterologic, endocrine, nervous and surgical symptoms. 3 cases of DiGeorge syndrome are reported in the article. The authors describe DiGeroge syndrome as a multidisciplinary disorder; it is masqueraded by acute surgical diseases; with sharp immunodeficiency and endocrine, cardiologic and neurologic semiotics.

  8. Nipple eczema, an indicative manifestation of atopic dermatitis? A clinical, histological, and immunohistochemical study.

    PubMed

    Song, Hyo Sang; Jung, Soo-Eun; Kim, You Chan; Lee, Eun-So

    2015-04-01

    Nipple eczema exhibits as a minor manifestation of atopic dermatitis (AD) or occurs as a single skin symptom on the nipple. To characterize the relationship between nipple eczema and AD, a clinical evaluation and an immunohistochemical study were performed. All cases of nipple eczema were confirmed histopathologically. We divided the patients with nipple eczema into 2 groups, namely, those with AD and those without AD, and compared several clinical features. Upon histological examination, the degree of inflammation was subjectively graded as mild, moderate, or severe by 2 separate investigators. Immunohistochemical stainings were performed by using antiinterleukin (IL)-4, anti-IL-13, anti-CD4, and anti-CD8 antibodies, and the results were scored semiquantitatively. In 43 cases evaluated, 12 were nipple eczema with AD. The clinical analysis and histological examination showed no significant differences between the groups. There were consistent findings of IL-4 expressions throughout the epidermis and IL-13 expression mainly in the perivascular area of the dermis. Although CD4 and CD8 were expressed in the cells in the dermis, CD8 expression was detected in the serocrusts of the epidermis. Expression levels of IL-4, IL-13, CD4, and CD8 exhibited no significant differences between the nipple eczema group with AD and the nipple eczema group without AD. Although nipple eczema may accompany AD, we found no definite differences in the degree or pattern of inflammation and cytokine expression level regardless of whether AD was present or not. Serocrust formation seemed to be mainly a collection of CD8-positive cells.

  9. Clinical manifestations, treatment outcomes, and risk factors for sternoclavicular septic arthritis.

    PubMed

    Rodchuae, Muchima; Ruangpin, Chonlada; Katchamart, Wanruchada

    2017-02-26

    Septic arthritis of the sternoclavicular joint (SCJ) is an atypical and rarely seen clinical condition. The prognosis for patients with SCJ septic arthritis is often poor. The objective of this study was to compare clinical characteristics between SCJ and non-sternoclavicular joint (NSCJ) septic arthritis and to identify independent risk factors for SCJ septic arthritis. A total of 450 adult patients diagnosed with septic arthritis during the January 2002 to December 2013 study period were included in this retrospective cohort study. Patient characteristics, clinical manifestations, and treatment outcomes were examined. Univariate and multivariate analysis was performed to identify potential risk factors for SCJ septic arthritis. Thirty-three (7.3%) of 450 patients had SCJ septic arthritis and the remaining 417 patients had NSCJ. Oligoarthritis or polyarthritis were seen more often in SCJ patients than in NSCJ patients (55 vs. 19%; p < 0.05). Abscess formation and bacteremia were more commonly found at presentation in the SCJ group than in the NSCJ group (18 vs. 8%; p < 0.05 for abscess formation; and, 53.1 vs. 26.6%; p < 0.05 for bacteremia). In multivariate analysis, extra-articular infection (OR 2.7, 95% CI 1.2-6.4; p = 0.02), cirrhosis (OR 1.9, 95% CI 1.1-3.3; p = 0.02), and malignancy (OR 3, 95% CI 1.1-7.8; p = 0.03) were independent risk factors for SCJ septic arthritis. SCJ septic arthritis is an uncommon septic arthritis that frequently presents with local and systemic complications. Factors found to be significantly associated with SCJ septic arthritis were extra-articular infection and immunocompromised host. A high index of suspicion in high-risk patients is the key to achieving improved outcomes.

  10. Aspergillus arthritis: analysis of clinical manifestations, diagnosis, and treatment of 31 reported cases.

    PubMed

    Gamaletsou, Maria N; Rammaert, Blandine; Bueno, Marimelle A; Sipsas, Nikolaos V; Moriyama, Brad; Kontoyiannis, Dimitrios P; Roilides, Emmanuel; Zeller, Valerie; Taj-Aldeen, Saad J; Henry, Michael; Petraitis, Vidmantas; Denning, David W; Lortholary, Olivier; Walsh, Thomas J

    2016-09-08

    Aspergillus arthritis is a debilitating form of invasive aspergillosis. Little is known about its epidemiology, clinical manifestations, laboratory features, treatment, and prognosis. Cases of Aspergillus arthritis were reviewed in the English literature from 1967 through 2015 for variables of arthritis with Aspergillus spp. recovered from joint and/or adjacent bone, underlying conditions, symptoms, signs, inflammatory biomarkers, diagnostic imaging, management, and outcome. Among 31 evaluable cases, 87% were males and 13% pediatric. Median age was 50 y (range 1-83 y). Seventeen (55%) patients were immunosuppressed with such conditions as hematological malignancies (26%), corticosteroids (39%), and/or transplantation (26%). Approximately one-half (52%) of patients had hematogenous seeding of the joint, and more than 80% had de novo infection with no prior antifungal therapy. Oligoarticular infection (2-3 joints) occurred in 45% and contiguous osteomyelitis was present in 61%. Clinical manifestations included pain (87%), edema (26%), and limited function (23%), with knees (35%), intervertebral discs (26%), and hips (16%) being most commonly infected. Aspergillus fumigatus constituted 77% of cases followed by Aspergillus flavus in 13%, Aspergillus niger in 3%, and not specified in 7%. Median ESR was 90 mm/hr and median CRP was 3.6 mg/dl. Median synovial fluid WBC was 17,200/μL (7,300-128,000) with 72% PMNs (range 61-92). Osteolysis occurred in 35%, and soft-tissue extension 47%. Nineteen patients (61%) were managed with combined medical and surgical therapy, 10 (32%) with medical therapy only, and 2 (6%) surgery only. Amphotericin B and itraconazole were the most frequently used agents with median duration of therapy of 219 days (range 30-545). Surgical interventions included debridement in 61%, drainage 19%, and amputation 6%. Complete or partial response was achieved in 71% and relapse occurred in 16%. Medical therapy was reinstituted with successful outcome in

  11. Alternating hemiparesis and orolingual apraxia as manifestations of methotrexate neurotoxicity in a paediatric case of acute lymphoblastic leukaemia.

    PubMed

    Yap, Siew Mei; MacEneaney, Peter; Ryan, Clodagh; O'Toole, Orna

    2016-04-25

    A 15-year-old girl with a recent diagnosis of acute lymphoblastic leukaemia was admitted to hospital with pancytopaenia after having received high-dose intrathecal methotrexate 1 day prior. During the next week she had intermittent episodes of alternating hemiparesis associated with speech arrest lasting minutes to hours at a time. The episodes were not associated with altered level of consciousness or headache. MRI of the brain showed features consistent with methotrexate encephalopathy. This report discusses the typical clinical and radiological features of methotrexate neurotoxicity in addition to differential diagnoses and the proposed pathophysiological mechanisms.

  12. Clinical manifestations, disability and use of folk medicine in dracunculus infection in Nigeria.

    PubMed

    Ilegbodu, V A; Ilegbodu, A E; Wise, R A; Christensen, B L; Kale, O O

    1991-02-01

    A cross-sectional survey of households was carried out in a dracunculiasis endemic village in Oyo state Nigeria. Data were collected on history of dracunculiasis, occupational and domestic sources of drinking water, clinical manifestations, disability, use of folk medicine, and incorporation of previous dracunculiasis control programmes. The findings indicated that dracunculiasis patients were usually unaware of their infection 3-5 days before the appearance of a bleb; that religious affiliation appeared to be positively related to increasing morbidity; and that ulcers were predominantly in the ankles and feet, particularly among young children. Severe disability was related to age, site and number of ulcers, and the form of selected treatment. Indigenous remedy was the treatment of choice, although traditional healers in the community indicated no knowledge of any efficacious remedy. Mortality from secondary tetanus infection was associated with outbreak of dracunculiasis. The impact of dracunculiasis on agricultural, economic and recreational activities was considerable, with the infected farmers being unable to attend to their farms at the critical farming period. Sixty-one per cent of the residents were opposed on religious and aesthetic grounds to the treatment of the local surface water which contained cyclops species. Sixty-three per cent regarded the boiling and filtration of portions of their domestic water as an additional burden, cumbersome and impracticable.(ABSTRACT TRUNCATED AT 250 WORDS)

  13. Clinical and Associated Immunological Manifestations of HFMD Caused by Different Viral Infections in Children

    PubMed Central

    Wang, Jingjing; Pu, Jing; Liu, Longding; Che, Yanchun; Liao, Yun; Wang, Lichun; Guo, Lei; Feng, Min; Liang, Yan; Fan, Shengtao; Cai, Lukui; Zhang, Ying; Li, Qihan

    2016-01-01

    Hand, foot, and mouth disease (HFMD), with vesiculae on the hands, feet and mouth, is an infectious disease caused by many viral pathogens. However, the differences of immune response induced by these pathogens are unclear. We compared the clinical manifestations and the levels of immunologic indicators from 60 HFMD patients caused by different viral pathogens to analyze the differences in the immune response. It was shown that Th2 cytokines (IL-4 and IL-10) increased significantly in EV71-infected children; Th1 cytokines (IL-2 and IFN-γ) rose in CA16-infected children; both Th1 and Th2 cytokines elevated in non-EVG-infected children; only individual cytokines (such as IL-10) went up in EVG-infected children. Meanwhile, the antibodies induced by viral infection could not cross-interfere between the different pathogens. These differences might be due to variations in the immune response induced by the individual pathogens or to the pathogenesis of the infections by the individual pathogens. PMID:27336013

  14. Specific clinical manifestations of Nocardia: A case report and literature review

    PubMed Central

    Yu, Songsong; Wang, Jing; Fang, Qiuhong; Zhang, Jixin; Yan, Fengcai

    2016-01-01

    Nocardiosis is a rare bacterial infection of either the lungs (pulmonary) or body (systemic) that usually affects immunocompromised individuals. It is caused by Gram-positive, aerobic actinomycetes of the Nocardia genus. Multiple high-density sheet shadows in both lungs along with nodules or cavities are the most common presentations of nocardiosis, whereas a large pulmonary mass is considered to be rare. However, there is no specificity in the clinical manifestation of the disease. Therefore, isolation and identification of Nocardia strains is the only reliable diagnostic method. The present study describes the cases of two male patients of Asian descent with nocardiosis. Chest computed tomography scans showed a suspected tumor mass in both patients. Microscopic analysis and culturing of tissue samples obtained using a bronchoscope detected the presence of Nocardia wallacei. Neither patient showed signs of immunosuppression. The present study aimed to improve the understanding of lung nocardiosis and demonstrated that pulmonary nocardiosis should be suspected in the case of non-immunocompromised patients with a large mass in the lung. Furthermore, a review of the literature on infection with Nocardia was conducted. PMID:27698688

  15. Hepatocellular Carcinoma associated with Extra-hepatic Primary Malignancy: its Secular change, Clinical Manifestations and Survival

    PubMed Central

    Kee, Kwong Ming; Wang, Jing-Houng; Wang, Chih-Chi; Cheng, Yu-Fan; Lu, Sheng-Nan

    2016-01-01

    Clinical manifestations between hepatocellular carcinoma (HCC) and extra-hepatic primary malignancy (EHPM) are lack of large-scale study. We enrolled 14555 HCC patients between 1986 and 2013 retrospectively. The EHPM was classified as prior, synchronous and metachronous group based on before, within and after 6 months of HCC diagnosis, respectively. The incidence rate of EHPM is 3.91% (95% confidence interval [CI]: 3.60–4.23%). Urogenital cancers, kidney and bladder, were at unexpected higher ranks. Older in age, Child-Pugh A cirrhosis, negativity of HBsAg and anti-HCV, and earlier BCLC staging are independent factors associated with EHPM. The survival rates of EHPM improve over time and also better than HCC-alone. Cox proportional-hazards regression shows independent poor prognostic factors are age >60, male, AFP levels ≥400 ng/ml, positivity of HBsAg, Child-Pugh B vs. A, Non-metachronous group, respectively, treated with local ablation, transcatheter arterial embolization, radiotherapy and supportive care vs. surgery, respectively, TNM stage IIIA vs. I, and BCLC stages A, B, C and D vs. 0, respectively. Survival of EHPM improve could be explained by early diagnosis and improve treatment of cancers. PMID:27444261

  16. Pseudoxanthoma elasticum and skin: Clinical manifestations, histopathology, pathomechanism, perspectives of treatment

    PubMed Central

    Marconi, Barbara; Bobyr, Ivan; Campanati, Anna; Molinelli, Elisa; Consales, Veronica; Brisigotti, Valerio; Scarpelli, Marina; Racchini, Stefano; Offidani, Annamaria

    2015-01-01

    Summary Pseudoxantoma elasticum (PXE), also known as Groenblad-Strandberg syndrome, is a rare heritable disease with an estimated prevalence of 1:50,000 in the general population. PXE is considered a prototype of multisystem ectopic mineralization disorders and it is characterized by aberrant mineralization of soft connective tissue with degeneration of the elastic fibers, involving primarily the eyes, the cardiovascular system, and the skin. Cutaneous lesions consist of small, asymptomatic, yellowish papules or larger coalescent plaques, typically located on the neck and the flexural areas. PXE is caused by mutations in the ABCC6 (ATP-binding cassette subfamily C member 6) gene that encodes a transmembrane ATP binding efflux transporter, normally expressed in the liver and the kidney; however, the exact mechanism of ectopic mineralization remains largely unknown. The histological examination of cutaneous lesions, revealing accumulation of pleomorphic elastic structures in middermis, is essential for the definitive diagnosis of PXE, excluding PXE-like conditions. PXE is currently an intractable disease; although the cutaneous findings primarily present a cosmetic problem, they signify the risk for development of ocular and cardiovascular complications associated with considerable morbidity and mortality. The purpose of this review is to present a comprehensive overview of this rare form of hereditary connective tissue disorders, focus on the pathogenesis, the clinical manifestation, and the differential diagnosis of PXE. Emphasis is also placed on the management of cutaneous lesions and treatment perspectives of PXE. PMID:26361562

  17. Portal hypertension: an uncommon clinical manifestation of Takayasu arteritis in a 9-year-old child

    PubMed Central

    Herrera, Cristina N; Tomala-Haz, Javier E

    2016-01-01

    Takayasu arteritis (TA) is the third most common childhood vasculitis and its clinical manifestations depend on the arteries involved. We report a case of a 9-year-old boy with multiple aneurysms in carotid and iliac arteries, subclavian and coronary arteries, and abdominal aorta. At the age of 7 years, he presented with recurrent fever and hepatosplenomegaly. An angio-computed tomography scan showed aneurysms in the left subclavian artery, abdominal aorta, and both proximal iliac arteries. He was diagnosed with TA and was treated with corticosteroids, aspirin, and enalapril. One year later, he was admitted to Dr Roberto Gilbert Children’s Hospital because of intracranial hemorrhage. Angiography revealed enlargement of aneurysms enlargement and new aneurysms. He also developed portal hypertension. Treatment with intravenous corticosteroids, azathioprine, and monthly intravenous cyclophosphamide was begun. After 6 months of no improvement, infliximab was begun. The aim of this article was to report the concurrence of coronary involvement and portal vein hypertension in pediatric TA because there were scarce reports on this matter. PMID:27895519

  18. A case of symptomatic cervical perineural (Tarlov) cyst: clinical manifestation and management.

    PubMed

    Kim, Keewon; Chun, Se Woong; Chung, Sun G

    2012-01-01

    Perineural (Tarlov) cysts are most often found in the sacral region and are rare in the cervical spine. Although they are usually asymptomatic, a small number of those at the lumbosacral level have been known to produce localized or radicular pain. Few reports are available on symptomatic perineural cysts in the cervical spine and it has not been discussed how they should be managed. We present here a case of cervical perineural cysts with persistent radicular pain where the pain was adequately managed with repetitive transforaminal epidural steroid injection (TFESI). The patient had experienced intractable pain in the posterior neck and left upper extremity for more than 7 years. The nature of the pain was cramping and a tingling sensation, which was aggravated in the supine position. Magnetic resonance imaging revealed a perineural cyst in the neural foramen of left C7 root. The patient underwent three repetitive TFESIs targeted at the root. Each injection provided incremental relief, which lasted more than 6 months. Follow-up image revealed shrinkage of the cyst. This case illustrates in detail the clinical manifestation of a rare symptomatic perineural cyst in the cervical region and to our knowledge is the first to report the beneficial effect of repetitive TFESI.

  19. Clinical heterogeneity in autoimmune acute liver failure

    PubMed Central

    Chavez-Tapia, Norberto C; Martinez-Salgado, Julio; Granados, Julio; Uribe, Misael; Tellez-Avila, Felix I

    2007-01-01

    AIM: To describe the outcome and prognosis in a cohort of patients with acute liver failure due to autoimmune hepatitis without liver transplantation. METHODS: A retrospective trial was conducted in 11 patients with acute liver failure due to autoimmune hepatitis who attended the Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubiran. Demographic, biochemical and severity indexes, and treatment and outcome were assessed. RESULTS: Among the 11 patients, with a median age of 31 years, 72% had inflammatory response syndrome, and six patients received corticosteroids. The mortality rate within four weeks was 56%, and the one-year survival was 27%. In the survivors, severity indexes were lower and 83% received corticosteroids. CONCLUSION: We observed a relatively high survival rate in patients with acute liver failure due to autoimmune hepatitis. This survival rate could be influenced by severity of the disease and/or use of corticosteroids. PMID:17465474

  20. Minimal Brain Dysfunction Child: Some Clinical Manifestations, Definitions, Descriptions and Remediation Approaches.

    ERIC Educational Resources Information Center

    Stock, Claudette, Comp.

    Remediation of learning disabilities is discussed and a table of teaching materials related to psychological and motor functions is provided. Guides on 11 behavioral manifestations and three specific learning disabilities furnish definitions and description as well as techniques for training and management. Behavioral manifestations considered…

  1. The Relation between Diverse Phenotypes of PCOS with Clinical Manifestations, Anthropometric Indices and Metabolic Characteristics.

    PubMed

    Shahrami, Seyedeh Hajar; Abbasi Ranjbar, Zahra; Milani, Forozan; Kezem-Nejad, Ehsan; Hassanzadeh Rad, Afagh; Dalil Heirat, Seyedeh Fatemeh

    2016-02-01

    Critical issue regarding to variation of findings based on different phenotypes led investigators to define whether they are distinct features or overlapping ones. Therefore, we aimed to investigate the association between diverse phenotypes of PCOS (Poly Cystic Ovary Syndrome) with clinical manifestations, anthropometric indices, and metabolic characteristics. This was a descriptive cross-sectional study conducted in 15-39 years old women with PCOS referred to infertility clinics in the north part of Iran, Rasht during 2010-2011. Data were gathered through an interview by a form consisted of demographic characteristics, laboratory findings, ovarian volume and anthropometric indices. A total of 214 patients consisted of 161 PCOS (cases) and 53 normal women (controls) participated in this study. The most prevalent phenotype in PCOS population was IM/PCO/HA (54%), followed by IM/HA (28%) and IM/PCO (13%). PCO/HA was present only in 6 PCOS patients (5%). PCOS patients were significantly younger than controls (P=0.07). Results showed that increased ovarian volume were higher in PCOS group in comparison with controls and IM/PCO/HA, and IM/PCO had respectively the largest ovarian volumes. Also, a significant relation was observed based on Cholesterol, 17OHP, LH, TG, 2hpp, and LH/FSH between patients with PCOS and control groups. There were significant differences in demographic, anthropometric, hormonal and ultrasound findings between PCOS and controls. Therefore, it seems that classification of the characteristics of each phenotype could offer an appropriate guide for screening risks of PCOS and may facilitate performing most favorable treatment for these complications.

  2. The plethora, clinical manifestations and treatment options of autoimmunity in patients with primary immunodeficiency

    PubMed Central

    Barış, Hatice Ezgi; Kıykım, Ayça; Nain, Ercan; Özen, Ahmet Oğuzhan; Karakoç-Aydıner, Elif; Barış, Safa

    2016-01-01

    Aim Although the association between primary immunodeficiency and autoimmunity is already well-known, it has once again become a topic of debate with the discovery of newly-defined immunodeficiencies. Thus, investigation of the mechanisms of development of autoimmunity in primary immunodefficiency and new target-specific therapeutic options has come to the fore. In this study, we aimed to examine the clinical findings of autoimmunity, autoimmunity varieties, and treatment responses in patients who were genetically diagnosed as having primary immunodeficiency. Material and Methods The files of patients with primary immunodeficiency who had clinical findings of autoimmunity, who were diagnosed genetically, and followed up in our clinic were investigated. The demographic and clinical features of the patients and their medical treatments were evaluated. Results Findings of autoimmunity were found in 30 patients whose genetic mutations were identified. The mean age at the time of the first symptoms was 8.96±14.64 months, and the mean age of receiving a genetic diagnosis was 82.55±84.71 months. The most common diseases showing findings of autoimmunity included immune dysregulation, polyendocrinopathy, enteropathy X-linked syndrome (16.7%); autoimmune lymphoproliferative syndrome (10%); lipopolysaccharide-responsive beige-like anchor protein deficiency (10%); and DiGeorge syndrome (10%). Twelve (40%) patients showed findings of autoimmunity at the time of first presentation. The most common findings of autoimmunity included inflammatory bowel disease, inflammatory bowel disease-like findings (n=14, 46.7%), immune thrombocytopenic purpura (n=11, 36.7%), and autoimmune hemolytic anemia (n=9, 30.0%). A response to immunosupressive agents was observed in 15 (50%) patients. Ten patients underwent hematopoietic stem cell transplantation. Six patients were lost to follow-up due to a variety of complications. Conclusion Autoimmunity is frequently observed in patients with

  3. Effects of Dimensional and Categorical Classification on the Clinical Manifestation of Major Depressive Disorder

    PubMed Central

    GÜLEÇ, Hüseyin; Karabekİroğlu, Aytül; YENEL, Aynil; BAYKARAN, Mustafa Burak; KELEŞ ÜNAL, Elif

    2014-01-01

    Introduction The aim of this study was to combine the dimensional concept with the categorical system in major depressive disorder (MDD) to reduce the complexity of the diagnosis. Furthermore, it was aimed to match categorical and dimensional approaches in a clear and simple manner. Method The study included a patient group of 131 consecutive outpatients diagnosed with MDD according to the DSM-IV diagnosis criteria, and a control group of 99 people that is matched with the patient group by gender, age and education level. All subjects completed the Beck Depression Inventory, the Beck Anxiety Inventory and the Toronto Alexithymia Scale (TAS). Results Cronbach’s alpha values for the analysis of the internal consistency of the scale for the patients group, control group and the total participants were determined as .94–.97, .87–.92 and .93–.96, respectively. Nine factors were obtained from the results of exploratory factor analysis. According to the Scree-plot, it was decided that the two-factor structure represents best. Although depression and anxiety are two distinct dimensions, the relationship between them was found to be significantly significant. This was valid for both patient and control groups. When the relationship between the DSM-IV diagnosis criteria and all variables (depression and its sub-dimensions, anxiety and its sub-dimensions and the number of symptoms) was evaluated, the number of symptoms was found to be significantly related with all of the criteria. Conclusion The number of symptoms and the severity of illness are found to be important in the clinical manifestation of MDD. The relationship of the severity of the illness with sleep and appetite seems weaker. While loss of interest was mainly predicting the disorder, weight changes, psychomotor changes, difficulty in concentration, fatigue, and worthlessness were determined not to be predictors of the manifestations. According to dimensional approach, somatic anxiety and deterioration in

  4. Amyopathic Dermatomyositis: A Concise Review of Clinical Manifestations and Associated Malignancies.

    PubMed

    Udkoff, Jeremy; Cohen, Philip R

    2016-10-01

    Amyopathic dermatomyositis is a rare, idiopathic, connective tissue disease that presents with dermatologic lesions of classic dermatomyositis but lacks the myopathy of this disease. Cutaneous manifestations may include Gottron's sign, heliotrope rash, and characteristic patterns of poikiloderma. There is a substantial risk for developing interstitial lung disease or malignancy in patients with amyopathic dermatomyositis. A literature review of amyopathic dermatomyositis was performed using the PubMed medical database. The key features of amyopathic dermatomyositis, including autoantibodies, clinical presentation and dermatologic manifestations, epidemiology, history, associated malignancies, management, and pathogenesis, are summarized in this review. Cancer (solid tumor) (73/79, 89 %) and hematologic malignancies (9/79, 11 %) were reported in 79 patients, with three patients having more than one malignancy. In addition, there were six patients with amyopathic dermatomyositis who had tumor of unknown primary, and eight patients with cancer-associated amyopathic dermatomyositis for whom no additional details were provided. From the group of 73 tumors for whom primary origin and sex were available, malignancy of the genitourinary organs (24/73, 33 %), aerorespiratory organs (15/73, 21 %), and breast (14/73, 19 %) were the most commonly observed solid organ tumors. Tumors of the genitourinary organs (15/48, 31 %) and breast (14/48, 29 %) were the most frequent neoplasms in women, accounting for 29 of 48 (60 %) cancers, with the most common sites being breast (14/48, 29 %), ovary (8/48, 17 %), and cervix or uterus (5/48, 10 %). In men, tumors of the aerorespiratory (9/25, 36 %) and genitourinary (9/25, 36 %) tracts were the most common neoplasms, accounting for 72 % (18/25) of cancers; the most common sites of primary malignancy were nasopharyngeal (6/25, 24 %), bladder (4/25, 16 %), and either colorectal, lung or prostate cancer (three cancers each

  5. The role of mast cells in the genesis of acute manifestations following the intravenous injection of meperidine in dogs.

    PubMed

    Akcasu, A; Yillar, D O; Akkan, A G; Küçkhüseyin, C

    2009-01-01

    The effects of intravenous (iv) administration of the synthetic opioid analgesic meperidine in conscious dogs and their relation to histamine stored in mast cells were studied in comparison with those induced by compound 48/80, potent mast cell degranulator. When 48/80 (0.5 mg/ kg) and meperidine (10 mg/kg) were injected iv into conscious dogs, an acute brief period of yelling, flare reaction, scratching, hypersalivation, urination, defecation, and tachypnea occurred after a latency of 30-35 sec. In addition, meperidine-treated dogs showed marked sedation. Dogs whose histamine stores were depleted by 48/80 manifested none of those effects induced by meperidine except sedation. Likewise, pretreatment with meperidine prevented the effects of a subsequent injection of 48/80. Sedation appeared to be independent of the histamine-releasing effect of meperidine, whereas other effects elicited by its intravenous injection of the drug were suppressed by 48/80 and thus were probably mediated via released histamine. We concluded that the peripheral effects of meperidine show histamine dependency and mast cells are a potential important site for the peripheral actions of meperidine as well.

  6. A SONOGRAPHIC SHORT CERVIX AS THE ONLY CLINICAL MANIFESTATION OF INTRA-AMNIOTIC INFECTION

    PubMed Central

    HASSAN, SONIA; ROMERO, ROBERTO; HENDLER, ISRAEL; GOMEZ, RICARDO; KHALEK, NAHLA; ESPINOZA, JIMMY; NIEN, JYH KAE; BERRY, STANLEY M.; BUJOLD, EMMANUEL; CAMACHO, NATALIA; SOROKIN, YORAM

    2006-01-01

    OBJECTIVE A sonographically short cervix is a powerful predictor of spontaneous preterm delivery. However, the etiology and optimal management of a patient with a short cervix in the mid-trimester of pregnancy remain uncertain. Microbial invasion of the amniotic cavity (MIAC) and intra-amniotic inflammation are frequently present in patients with spontaneous preterm labor or acute cervical insufficiency. This study was conducted to determine the rate of MIAC and intra-amniotic inflammation in patients with a cervical length <25 mm in the mid-trimester. STUDY DESIGN A retrospective cohort study was conducted of patients referred to our high risk clinic because of a sonographic short cervix or a history of a previous preterm birth. Amniocenteses were performed for the evaluation of MIAC and for karyotype analysis in patients with a short cervix. Fluid was cultured for aerobic and anaerobic bacteria, as well as genital mycoplasmas. Patients with MIAC were treated with antibiotics selected by their physician. RESULTS Of 152 patients with a short cervix at 14–24 weeks, 57 had amniotic fluid analysis. The prevalence of MIAC was 9% (5/57). Among these patients, the rate of preterm delivery (<32 weeks) was 40% (2/5). Microorganisms isolated from amniotic fluid included Ureaplasma urealyticum (n=4) and Fusobacterium nucleatum (n=1). Patients with a positive culture for Ureaplasma urealyticum received intravenous Azithromycin. Three patients with Ureaplasma urealyticum had a sterile amniotic fluid culture after treatment, and subsequently delivered at term. The patient with Fusobacterium nucleatum developed clinical chorioamnionitis and was induced. CONCLUSION 1) Sub-clinical MIAC was detected in 9% of patients with a sonographically short cervix (<25 mm); and 2) maternal parenteral treatment with antibiotics can eradicate MIAC caused by Ureaplasma urealyticum. This was associated with delivery at term in the three patients whose successful treatment was documented by

  7. A Review of Chikungunya Virus-induced Arthralgia: Clinical Manifestations, Therapeutics, and Pathogenesis

    PubMed Central

    Goupil, Brad A.; Mores, Christopher N.

    2016-01-01

    Background: Chikungunya virus (CHIKV) is a mosquito-borne alphavirus that circulates predominantly in tropical and subtropical regions, potentially affecting over 1 billion people. Recently, an outbreak began in the western hemisphere and has resulted in over 1.8 million reported suspected cases. Infection often results in severe fever, rash and debilitating polyarthralgia lasting weeks to months. Additionally, the current literature reports that CHIKV can result in a severe chronic arthralgia and/or arthritis that can last months to years following the initial infection. Objective: The purpose of this review is to evaluate the literature and summarize the current state of knowledge regarding CHIKV-associated disease, including clinical presentation, diagnosis, risk factors for development of severe disease, treatment, and pathogenesis in human patients. Additionally, recommendations are presented regarding avenues for clinical research to help further elucidate the pathogenesis of joint disease associated with CHIKV infection. Conclusion: While there is an association between initial CHIKV infection and acute disease, a causal relationship with development of chronic arthralgia has not been established at this time. Potential causes of chronic CHIKV-induced arthritis have been postulated, including viral persistence, induction of autoimmune disease, and exacerbation of pre-existing joint disease. While there are numerous reports of chronic CHIKV-associated arthralgia and/or arthritis, there is currently no evidence of a definitive link between initial infection and development of chronic disease. Additional, prospective clinical research on CHIKV-associated disease is necessary to further determine the potential role of virus and development of chronic joint disease. PMID:28077980

  8. Clinical manifestations of hemochromatosis in HFE C282Y homozygotes identified by screening

    PubMed Central

    McLaren, Gordon D; McLaren, Christine E; Adams, Paul C; Barton, James C; Reboussin, David M; Gordeuk, Victor R; Acton, Ronald T; Harris, Emily L; Speechley, Mark R; Sholinsky, Phyliss; Dawkins, Fitzroy W; Snively, Beverly M; Vogt, Thomas M; Eckfeldt, John H

    2008-01-01

    BACKGROUND: Patients with hemochromatosis may suffer organ damage from iron overload, often with serious clinical consequences. OBJECTIVE: To assess prevalences of self-reported symptoms and clinical signs and conditions in persons homozygous for the hemochromatosis gene (HFE) mutation (C282Y) identified by screening. METHODS: Participants were adults 25 years of age or older enrolled in the Hemochromatosis and Iron Overload Screening (HEIRS) Study. C282Y homozygotes (n=282) were compared with control participants without the HFE C282Y or H63D alleles (ie, wild type/wild type; n=364). RESULTS: Previously diagnosed C282Y homozygotes and newly diagnosed homozygotes with elevated serum ferritin levels had higher prevalences of certain symptoms such as chronic fatigue (OR 2.8; 95% CI 1.34 to 5.95, and OR 2.0; 95% CI 1.07 to 3.75, respectively), and had more hyperpigmentation on physical examination (OR 4.7; 95% CI 1.50 to 15.06, and OR 3.7; 95% CI 1.10 to 12.16, respectively) and swelling or tenderness of the second and third metacarpophalangeal joints (OR 4.2; 95% CI 1.37 to 13.03, and OR 3.3; 95% CI 1.17 to 9.49, respectively) than control subjects. Joint stiffness was also more common among newly diagnosed C282Y homozygotes with elevated serum ferritin than among control subjects (OR 2.7; 95% CI 1.38 to 5.30). However, the sex- and age-adjusted prevalences of self-reported symptoms and signs of liver disease, heart disease, diabetes and most other major clinical manifestations of hemochromatosis were similar in C282Y homozygotes and control subjects. CONCLUSIONS: Some symptoms and conditions associated with hemochromatosis were more prevalent among C282Y homozygotes identified by screening than among control subjects, but prevalences of most outcomes were similar in C282Y homozygotes and controls in this primary care-based study. PMID:19018338

  9. Acute high-altitude illness: a clinically orientated review

    PubMed Central

    Smedley, Tom

    2013-01-01

    Acute high-altitude illness is an encompassing term for the range of pathology that the unacclimatised individual can develop at increased altitude. This includes acute mountain sickness, high-altitude cerebral oedema and high-altitude pulmonary oedema. These conditions represent an increasing clinical problem as more individuals are exposed to the hypobaric hypoxic environment of high altitude for both work and leisure. In this review of acute high-altitude illness, the epidemiology, risk factors and pathophysiology are explored, before their prevention and treatment are discussed. Appropriate ascent rate remains the most effective acute high-altitude illness prevention, with pharmacological prophylaxis indicated in selected individuals. Descent is the definitive treatment for acute high-altitude illness, with the adjuncts of oxygen and specific drug therapies. PMID:26516505

  10. Impact of sex disparities on the clinical manifestations in patients with systemic lupus erythematosus

    PubMed Central

    Boodhoo, Kamini Devi; Liu, Sijia; Zuo, Xiaoxia

    2016-01-01

    Abstract Background: Systemic lupus erythematosus (SLE) is a chronic autoimmune multiorgan disorder of unknown etiology. It affects both men and women, but with different disease manifestations of differing disease severity and in varying proportion, with a female predominance of approximately 90%. There have been numerous studies addressing this issue, especially its implications in relation to optimal sex-tailored treatment and improvement of survival rate; however, further research is warranted. A meta-analysis of studies was performed to compare the impact of sex on the clinical outcomes of SLE in different populations. Methods: A literature search of the MEDLINE/PubMed and EMBASE databases (until January 2016) was conducted to identify relevant articles. Clinical manifestations reported in these patients were considered as endpoints for this meta-analysis. Two independent reviewers determined eligibility criteria. A fixed-effect model has been used where a small heterogeneity was observed, or else, a random-effect model has been used among the studies. Odd ratio (OR) with 95% confidence interval (CI) was used to express the pooled effect on dichotomous variables, and the pooled analyses were performed with RevMan 5.3. Results: Sixteen studies consisting of a total of 11,934 SLE patients (10,331 females and 1603 males) have been included in this meta-analysis. The average female-to-male ratio of all the included studies is around 9.3:1. Several statistically significant differences were found: alopecia, photosensitivity, and oral ulcers were significantly higher in female patients (OR 0.36, 95% CI 0.29–0.46, P < 0.00001; OR 0.72, 95% CI 0.63–0.83, P < 0.00001; and OR 0.70, 95% CI 0.60–0.82, P < 0.00001, respectively). Malar rash was significantly higher in female patients (OR 0.68, 95% CI 0.53–0.88, P = 0.003), and arthritis was significantly lower in male patients (OR 0.72, 95% CI 1.25–1.84, P < 0.00001). However, serositis and

  11. Clinical Manifestations in Individuals with Recent Diagnosis of HTLV Type I Infection

    PubMed Central

    Poetker, Shelene K.W.; Porto, Aurelia F.; Giozza, Silvana P.; Muniz, Andre L.; Caskey, Marina F.; Carvalho, Edgar M.; Glesby, Marshall J.

    2011-01-01

    Background Human T-lymphotropic virus type 1 (HTLV-1) is known to cause HTLV-associated myelopathy (HAM)/tropical spastic paraparesis and adult T cell leukemia. A growing body of evidence links HTLV-1 infection with an increasing spectrum of disease, including uveitis, periodontal disease, arthropathy, sicca syndrome, and neurologic deficits. Objectives Despite recent findings, the natural history of HTLV-1 infection remains poorly defined. This study was designed to better characterize initial clinical and neurological findings in individuals diagnosed with HTLV-1 infection. Study Design We conducted a cross-sectional study of 71 individuals recently diagnosed with HTLV-1 and 71 uninfected age- and sex-matched blood donors in Salvador, Brazil. Subjects were administered a standardized questionnaire and underwent physical exam. Results HTLV-1 infected subjects were significantly more likely than controls to report complaints of hand and foot numbness (OR=5.3; 95% CI: 1.8-15.3; p=0.002 and OR=4.0; 95% CI: 1.3-12; p=0.013 respectively), difficulty running (OR=4.0; 95% CI: 1.1-14.2, p=0.032), nocturia (OR=5.0, 95% CI: 1.1-22.8, p=0.038), arthralgia (OR 3.3, 95% CI: 1.4-7.7, p=0.006), and photophobia (OR 3.3, 95% CI: 1.4-7.7, p=0.006). Conclusions Neurologic, ocular and rheumatologic complaints may be the first manifestations of HTLV-1 infection. Therefore, all patients presenting with initial diagnosis should be rigorously screened for these symptoms. PMID:21388871

  12. Association of PAX2 and Other Gene Mutations with the Clinical Manifestations of Renal Coloboma Syndrome

    PubMed Central

    Higashide, Tomomi; Sakurai, Mayumi; Hashimoto, Shin-ichi; Shinozaki, Yasuyuki; Hara, Akinori; Iwata, Yasunori; Sakai, Norihiko; Sugiyama, Kazuhisa; Kaneko, Shuichi; Wada, Takashi

    2015-01-01

    Background Renal coloboma syndrome (RCS) is characterized by renal anomalies and optic nerve colobomas. PAX2 mutations contribute to RCS. However, approximately half of the patients with RCS have no mutation in PAX2 gene. Methods To investigate the incidence and effects of mutations of PAX2 and 25 candidate genes, patient genes were screened using next-generation sequence analysis, and candidate mutations were confirmed using Sanger sequencing. The correlation between mutations and clinical manifestation was evaluated. Result Thirty patients, including 26 patients (two families of five and two, 19 sporadic cases) with RCS, and 4 optic nerve coloboma only control cases were evaluated in the present study. Six PAX2 mutations in 21 probands [28%; two in family cohorts (n = 5 and n = 2) and in 4 out of 19 patients with sporadic disease] including four novel mutations were confirmed using Sanger sequencing. Moreover, four other sequence variants (CHD7, SALL4, KIF26B, and SIX4) were also confirmed, including a potentially pathogenic novel KIF26B mutation. Kidney function and proteinuria were more severe in patients with PAX2 mutations than in those without the mutation. Moreover, the coloboma score was significantly higher in patients with PAX2 gene mutations. Three out of five patients with PAX2 mutations had focal segmental glomerulosclerosis (FSGS) diagnosed from kidney biopsies. Conclusion The results of this study identify several new mutations of PAX2, and sequence variants in four additional genes, including a novel potentially pathogenic mutation in KIF26B, which may play a role in the pathogenesis of RCS. PMID:26571382

  13. Plasma globotriaosylsphingosine: diagnostic value and relation to clinical manifestations of Fabry disease.

    PubMed

    Rombach, S M; Dekker, N; Bouwman, M G; Linthorst, G E; Zwinderman, A H; Wijburg, F A; Kuiper, S; Vd Bergh Weerman, M A; Groener, J E M; Poorthuis, B J; Hollak, C E M; Aerts, J M F G

    2010-09-01

    Fabry disease is an X-linked lysosomal storage disorder due to deficiency of alpha-Galactosidase A, causing accumulation of globotriaosylceramide and elevated plasma globotriaosylsphingosine (lysoGb3). The diagnostic value and clinical relevance of plasma lysoGb3 concentration was investigated. All male and adult female patients with classical Fabry disease could be discerned by an elevated plasma lysoGb3. In young pre-symptomatic Fabry heterozygotes, lysoGb3 levels can be normal. Individuals carrying the R112H and P60L mutations, without classical Fabry symptoms, showed no elevated plasma lysoGb3. Multiple regression analysis showed that there is no correlation of plasma lysoGb3 concentration with total disease severity score in Fabry males. However, plasma lysoGb3 concentration did correlate with white matter lesions (odds ratio: 6.1 per 100 nM lysoGb3 increase (95% CI: 1.4-25.9, p=0.015). In females, plasma lysoGb3 concentration correlated with overall disease severity. Furthermore, plasma lysoGb3 level was related to left ventricular mass (19.5+/-5.5 g increase per 10 nM lysoGb3 increase; p=0.001). In addition, it was assessed whether lifetime exposure to lysoGb3 correlates with disease manifestations. Male Fabry patients with a high lysoGb3 exposure (>10,000 U), were moderately or severely affected, only one mildly. Female patients with a low exposure (<1000 U) were asymptomatic or mildly affected. A large proportion of the females with an exposure >1000 U showed disease complications. Plasma lysoGb3 is useful for the diagnosis of Fabry disease. LysoGb3 is an independent risk factor for development of cerebrovascular white matter lesions in male patients and left ventricular hypertrophy in females. Disease severity correlates with exposure to plasma lysoGb3.

  14. Acute Q fever in Portugal. Epidemiological and clinical features of 32 hospitalized patients

    PubMed Central

    Palmela, Carolina; Badura, Robert; Valadas, Emília

    2012-01-01

    Introduction Q fever is a worldwide zoonosis caused by Coxiella burnetii. The main characteristic of acute Q fever is its clinical polymorphism, usually presenting as a febrile illness with varying degrees of hepatitis and/or pneumonia. Q fever is endemic in Portugal, and it is an obligatory notifiable disease since 1999. However, its epidemiological and clinical characteristics are still incompletely described. Methods We performed a retrospective study of 32 cases admitted in the Infectious Diseases Department, Santa Maria’s University Hospital, from January 2001 to December 2010, in whom acute Q fever was diagnosed by the presence of antibodies to phase II Coxiella burnetii antigens associated with a compatible clinical syndrome. Results Out of the 32 cases recorded, 29 (91%) were male, with a male:female ratio of 9.7:1. Individuals at productive age were mainly affected (88%, n=28, with ages between 25 and 64 years). Clinically, the most common manifestation of acute Q fever was hepatic involvement (84%, n=27), which occurred isolated in 53% (n=17) of the cases. Hepatitis was more severe, presenting with higher values of liver function tests, in patients presenting both pulmonary and hepatic involvement. Additionally, we report one case of myocarditis and another one with neurological involvement. Empiric but appropriate antibiotic therapy was given in 66% (n=21) of the cases. There was a complete recovery in 94% (n=30) of the patients, and one death. We confirmed the sub-notification of this disease in Portugal, with only 47% (n=15) of the cases notified. Conclusion In Portugal further studies are needed to confirm our results. From the 32 cases studied, acute Q fever presented more frequently as a febrile disease with hepatic involvement affecting mainly young male individuals. Furthermore, acute Q fever is clearly underdiagnosed and underreported in Portugal, which suggests that an increased awareness of the disease is needed, together with a broader use

  15. Cutaneous Manifestations of Polycystic Ovary Syndrome: A Cross-Sectional Clinical Study

    PubMed Central

    Keen, Mohammad Abid; Shah, Iffat Hassan; Sheikh, Gousia

    2017-01-01

    Background: Polycystic ovary syndrome (PCOS) is one of the most common endocrine disorders in women, affecting 5–10% of reproductive-aged women. The dermatologic manifestations of hyperandrogenism, chiefly hirsutism, acne vulgaris, androgenic alopecia, and acanthosis nigricans, are among the cardinal manifestations of PCOS. Aim: To study the incidence and prevalence of various cutaneous manifestations in patients with PCOS and to correlate these skin manifestations with hormonal changes. Settings and Design: This study was conducted at a dermatology centre over a period of 1 year from November 2012 to 2013. Materials and Methods: The present study included 100 women diagnosed to have PCOS. Hormonal analysis as well as radiological assessment was done in all the cases. Cutaneous manifestations were ascertained and inferences were drawn. Statistical Analysis: Statistical analysis was carried out by the Chi-square test and independent samples t-test. Statistical significance was determined at a level of P < 0.05. Results: In our study, the prevalence of hirsutism, acne, female pattern hair loss, acanthosis nigricans, seborrhea, striae and acrochordons was 78%, 48%, 31%, 30%, 29%, 13%, and 9%, respectively. Conclusion: Dermatologic manifestations of PCOS play a significant role in making the diagnosis and constitute a substantial portion of the symptoms experienced by women with this syndrome.

  16. Cohort study on maternal cytomegalovirus seroprevalence and prevalence and clinical manifestations of congenital infection in China

    PubMed Central

    Wang, Shiwen; Wang, Tongzhan; Zhang, Wenqiang; Liu, Xiaolin; Wang, Xiaofang; Wang, Haiyan; He, Xiaozhou; Zhang, Shunxian; Xu, Shuhui; Yu, Yang; Jia, Xingbing; Wang, Maolin; Xu, Aiqiang; Ma, Wei; Amin, Minal M.; Bialek, Stephanie R.; Dollard, Sheila C.; Wang, Chengbin

    2017-01-01

    Abstract Congenital cytomegalovirus (CMV) infection is the leading viral cause of birth defects and developmental disabilities in developed countries. However, CMV seroprevalence and burden of congenital CMV infection are not well defined in China. Cohort of newborns from 5 birthing hospitals in 2 counties of Shandong Province, China, were enrolled from March 2011 to August 2013. Dried blood spots (DBS) and saliva were collected within 4 days after birth for IgG testing for maternal seroprevalence and real-time PCR testing for congenital CMV infection, respectively. Among 5020 newborns tested for CMV IgG, 4827 were seropositive, resulting in CMV maternal seroprevalence of 96.2% (95% confidence interval [CI]:95.6%–96.7%). Of the 10,933 newborns screened for congenital CMV infection, 75 had CMV detected, resulting in an overall prevalence of 0.7% (95% CI: 0.5%–0.9%), with prevalences of 0.4% (14/3995), 0.6% (66/10,857), and 0.7% (52/7761) for DBS, wet saliva, and dried saliva specimens screened, respectively. Prevalence of congenital CMV infection decreased with increasing maternal age (0.9%, 0.6%, and 0.3% among newborns delivered from mothers aged 16–25, 26–35, and >35 years, respectively; P = 0.03), and was higher among preterm infants than full term infants (1.3% vs 0.6%, P = 0.04), infants with intrauterine growth restriction (IUGR) than those without (1.8% vs 0.7%, P = 0.03), and twins or triplets than singleton pregnancies (2.8% vs 0.7%, P = 0.04). None of the 75 newborns exhibited symptomatic congenital CMV infection, and there was no difference in clinical characteristics and newborn hearing screening results between infants with and without congenital CMV infection at birth. Congenital CMV infection prevalence was lower and the clinical manifestations were milder in this relatively developed region of China compared to populations from other countries with similarly high maternal seroprevalence. Follow-up on children with congenital

  17. Fever and increased serum IL-1 activity as a systemic manifestation of acute phototoxicity in New Zealand White rabbits.

    PubMed

    Ansel, J C; Luger, T A; Green, I

    1987-07-01

    Ultraviolet (UV) radiation is a significant environmental hazard for humans and animals. Although the clinical effect of an acute UV exposure such as cutaneous inflammation, malaise, somnolence, chills, and fever have been appreciated for many years, the underlying mechanisms mediating these effects are poorly understood. Since chills and fever are the most dramatic systemic sequelae after a prolonged exposure to UV, we specifically examined the effect of whole-body UV irradiation on core body temperature and serum endogenous pyrogen activity of New Zealand White rabbits, correlating this with serum interleukin 1 (IL-1) activity and alterations of serum divalent cation levels. We found that an acute dose of UV irradiation (Westinghouse FS-40 lamps, 0.2 mJ/cm2/s X 8 h) resulted in a significant increase in the core body temperature 2 h post UV (0.8 degree C), peaking 5 h post UV (1.8 degree C), and returning to normal 24 h post UV. Likewise, the sera from the UV-irradiated rabbits had significant endogenous pyrogen activity when transferred into naive recipient animals, causing an increase in core body temperature within 45 min (0.65 +/- 0.12 degree C), decreasing over the next 2 h, and returning to normal 6 h post injection. No endotoxin contamination was detected in any serum samples. This post-UV febrile response was accompanied by a prolonged increase in serum IL-1 activity (5-10 X) and a significant alteration in serum divalent cation levels, with the rabbits becoming euthermic even as the serum IL-1 levels remained elevated. These findings provide new information concerning the pathogenesis and kinetics of these systemic effects after an acute dose of UV irradiation.

  18. Clinical acute cholecystitis and the Curtis-Fitz-Hugh syndrome.

    PubMed Central

    Shanahan, D.; Lord, P. H.; Grogono, J.; Wastell, C.

    1988-01-01

    When patients are admitted with clinically diagnosed acute cholecystitis, no cause will be found for their pain in 9-13% (4.5). Our retrospective study shows that women between 15-35 years are most likely to be in this group. Our prospective study of all patients in the 15-35 year age group admitted with clinical 'acute cholecystitis', showed that in 6 out of 7 patients with 'undiagnosed' pain, the Curtis-Fitz-Hugh syndrome was the cause. We suggest that screening for the Curtis-Fitz-Hugh syndrome is performed in all patients with right upper quadrant pain who have a normal ultrasound scan. PMID:3408139

  19. Host Immune Transcriptional Profiles Reflect the Variability in Clinical Disease Manifestations in Patients with Staphylococcus aureus Infections

    PubMed Central

    Banchereau, Romain; Jordan-Villegas, Alejandro; Ardura, Monica; Mejias, Asuncion; Baldwin, Nicole; Xu, Hui; Saye, Elizabeth; Rossello-Urgell, Jose; Nguyen, Phuong; Blankenship, Derek; Creech, Clarence B.; Pascual, Virginia; Banchereau, Jacques; Chaussabel, Damien; Ramilo, Octavio

    2012-01-01

    Staphylococcus aureus infections are associated with diverse clinical manifestations leading to significant morbidity and mortality. To define the role of the host response in the clinical manifestations of the disease, we characterized whole blood transcriptional profiles of children hospitalized with community-acquired S. aureus infection and phenotyped the bacterial strains isolated. The overall transcriptional response to S. aureus infection was characterized by over-expression of innate immunity and hematopoiesis related genes and under-expression of genes related to adaptive immunity. We assessed individual profiles using modular fingerprints combined with the molecular distance to health (MDTH), a numerical score of transcriptional perturbation as compared to healthy controls. We observed significant heterogeneity in the host signatures and MDTH, as they were influenced by the type of clinical presentation, the extent of bacterial dissemination, and time of blood sampling in the course of the infection, but not by the bacterial isolate. System analysis approaches provide a new understanding of disease pathogenesis and the relation/interaction between host response and clinical disease manifestations. PMID:22496797

  20. Acute Clinical Worsening after Steroid Administration in Cervical Myelitis May Reveal a Subdural Arteriovenous Fistula

    PubMed Central

    Rain, Silvia; Udding, Jan; Broere, Daniel

    2016-01-01

    Subdural arteriovenous fistula (SDAVF) is a rare condition characterized by clinical manifestations ranging from mild bilateral sensory deficits to quadriplegia. The diagnosis is often delayed due to unspecific neurological symptoms, initially diagnosed as polyneuropathy or myelopathy. The diagnosis can be delayed for as long as 1–15 years. The following report describes a cervical SDAVF case initially misdiagnosed as myelitis transversa and treated with intravenous steroids. A 56-year-old male presented with sensory deficits and mild leg and right arm weakness. Cervical MRI showed a central medullary hyperintense lesion with contrast enhancement. After metabolic, infectious, and malignant causes were excluded, myelitis transversa was presumed and the patient was treated intravenously with methylprednisolone. Shortly after that, he developed quadriplegia. Cervical MRI imaging showed engorged cervical perimedullary vessels, which were not visible on the initial MRI. The diagnosis was revised and a SDAVF identified. Prompt surgical treatment led to a complete recovery. The effect of intravenous steroids in SDAVF is controversial. Acute clinical worsening after steroid administration is previously reported in several publications; however, due to the paucity of clinical studies on SDAVF, this effect remains mostly overlooked or unknown. The findings in this patient support the causative relation between SDAVF clinical worsening and steroid administration. We propose that acute clinical worsening under steroids in patients initially diagnosed with myelitis should raise suspicion of an SDAVF. PMID:27920716

  1. A Multicentre Study of Shigella Diarrhoea in Six Asian Countries: Disease Burden, Clinical Manifestations, and Microbiology

    PubMed Central

    von Seidlein, Lorenz; Kim, Deok Ryun; Ali, Mohammad; Lee, Hyejon; Wang, XuanYi; Thiem, Vu Dinh; Canh, Do Gia; Chaicumpa, Wanpen; Agtini, Magdarina D; Hossain, Anowar; Bhutta, Zulfiqar A; Mason, Carl; Sethabutr, Ornthipa; Talukder, Kaisar; Nair, G. B; Deen, Jacqueline L; Kotloff, Karen; Clemens, John

    2006-01-01

    Background The burden of shigellosis is greatest in resource-poor countries. Although this diarrheal disease has been thought to cause considerable morbidity and mortality in excess of 1,000,000 deaths globally per year, little recent data are available to guide intervention strategies in Asia. We conducted a prospective, population-based study in six Asian countries to gain a better understanding of the current disease burden, clinical manifestations, and microbiology of shigellosis in Asia. Methods and Findings Over 600,000 persons of all ages residing in Bangladesh, China, Pakistan, Indonesia, Vietnam, and Thailand were included in the surveillance. Shigella was isolated from 2,927 (5%) of 56,958 diarrhoea episodes detected between 2000 and 2004. The overall incidence of treated shigellosis was 2.1 episodes per 1,000 residents per year in all ages and 13.2/1,000/y in children under 60 months old. Shigellosis incidence increased after age 40 years. S. flexneri was the most frequently isolated Shigella species (1,976/2,927 [68%]) in all sites except in Thailand, where S. sonnei was most frequently detected (124/146 [85%]). S. flexneri serotypes were highly heterogeneous in their distribution from site to site, and even from year to year. PCR detected ipaH, the gene encoding invasion plasmid antigen H in 33% of a sample of culture-negative stool specimens. The majority of S. flexneri isolates in each site were resistant to amoxicillin and cotrimoxazole. Ciprofloxacin-resistant S. flexneri isolates were identified in China (18/305 [6%]), Pakistan (8/242 [3%]), and Vietnam (5/282 [2%]). Conclusions Shigella appears to be more ubiquitous in Asian impoverished populations than previously thought, and antibiotic-resistant strains of different species and serotypes have emerged. Focusing on prevention of shigellosis could exert an immediate benefit first by substantially reducing the overall diarrhoea burden in the region and second by preventing the spread of

  2. Clinical manifestations of colorectal cancer patients from a large multicenter study in Colombia

    PubMed Central

    Bohorquez, Mabel; Sahasrabudhe, Ruta; Criollo, Angel; Sanabria-Salas, María Carolina; Vélez, Alejandro; Castro, Jorge Mario; Marquez, Juan Ricardo; Mateus, Gilbert; Bolaños, Fernando; Panqueva, Cesar; Restrepo, Jose Ignacio; Puerta, Juan Dario; Murillo, Raul; Bravo, María Mercedes; Hernández, Gustavo; Rios, Angela; Prieto, Rodrigo; Tomlinson, Ian; Echeverry, Magdalena; Carvajal-Carmona, Luis G.

    2016-01-01

    Abstract Colorectal cancer (CRC) is a major public health problem, and its incidence is rising in developing countries. However, studies characterizing CRC clinicopathological features in cases from developing countries are still lacking. The goal of this study was to evaluate clinicopathological and demographic features in one of the largest CRC studies in Latin America. The study involved over 1525 CRC cases recruited in a multicenter study in Colombia between 2005 and 2014 as part of ongoing genetic and epidemiological studies. We gathered clinicopathological data such as age at diagnosis, sex, body mass index, tobacco and alcohol consumption, family history of cancer, and tumor features including location, histological type, and stage. Statistical analyses were performed to test the association between age of onset, sex, and clinical manifestations. The average age at CRC diagnosis was 57.4 years, with 26.5% of cases having early-onset CRC (diagnosed by age 50 years). Most cases were women (53.2%; P = 0.009), 49.2% were overweight or obese, 49.1% were regular alcohol drinkers, 52% were smokers/former smokers, and 12.2% reported relatives with cancer. Most tumors in the study were located in the rectum (42.7%), were adenocarcinomas (91.5%), and had advanced stage (T3–T4, 79.8%). Comparisons by sex found that male cases were more likely to be obese (36.5% vs 31.1%; P = 0.001), less likely to have a family history of cancer (9.7% vs 15.3%; P = 0.016), and more likely to have advanced-stage tumors (83.9% vs 76.1%; P = 0.036). Comparisons by age of onset found that early-onset cases were more likely to be women (59.3% vs 51.0%; P = 0.005) and report a family history of cancer (17.4% vs 10.2%; P = 0.001). To our knowledge, our study is the largest report of clinicopathological characterization of Hispanic CRC cases, and we suggest that further studies are needed to understand CRC etiology in diverse Hispanic populations. PMID:27749544

  3. Clinical manifestations of colorectal cancer patients from a large multicenter study in Colombia.

    PubMed

    Bohorquez, Mabel; Sahasrabudhe, Ruta; Criollo, Angel; Sanabria-Salas, María Carolina; Vélez, Alejandro; Castro, Jorge Mario; Marquez, Juan Ricardo; Mateus, Gilbert; Bolaños, Fernando; Panqueva, Cesar; Restrepo, Jose Ignacio; Puerta, Juan Dario; Murillo, Raul; Bravo, María Mercedes; Hernández, Gustavo; Rios, Angela; Prieto, Rodrigo; Tomlinson, Ian; Echeverry, Magdalena; Carvajal-Carmona, Luis G

    2016-10-01

    Colorectal cancer (CRC) is a major public health problem, and its incidence is rising in developing countries. However, studies characterizing CRC clinicopathological features in cases from developing countries are still lacking. The goal of this study was to evaluate clinicopathological and demographic features in one of the largest CRC studies in Latin America.The study involved over 1525 CRC cases recruited in a multicenter study in Colombia between 2005 and 2014 as part of ongoing genetic and epidemiological studies. We gathered clinicopathological data such as age at diagnosis, sex, body mass index, tobacco and alcohol consumption, family history of cancer, and tumor features including location, histological type, and stage. Statistical analyses were performed to test the association between age of onset, sex, and clinical manifestations.The average age at CRC diagnosis was 57.4 years, with 26.5% of cases having early-onset CRC (diagnosed by age 50 years). Most cases were women (53.2%; P = 0.009), 49.2% were overweight or obese, 49.1% were regular alcohol drinkers, 52% were smokers/former smokers, and 12.2% reported relatives with cancer. Most tumors in the study were located in the rectum (42.7%), were adenocarcinomas (91.5%), and had advanced stage (T3-T4, 79.8%). Comparisons by sex found that male cases were more likely to be obese (36.5% vs 31.1%; P = 0.001), less likely to have a family history of cancer (9.7% vs 15.3%; P = 0.016), and more likely to have advanced-stage tumors (83.9% vs 76.1%; P = 0.036). Comparisons by age of onset found that early-onset cases were more likely to be women (59.3% vs 51.0%; P = 0.005) and report a family history of cancer (17.4% vs 10.2%; P = 0.001).To our knowledge, our study is the largest report of clinicopathological characterization of Hispanic CRC cases, and we suggest that further studies are needed to understand CRC etiology in diverse Hispanic populations.

  4. Massive Bleeding as the First Clinical Manifestation of Metastatic Prostate Cancer due to Disseminated Intravascular Coagulation with Enhanced Fibrinolysis

    PubMed Central

    Lopes, João Madeira; Victorino, Rui M. M.; Meneses Santos, João

    2016-01-01

    Disseminated intravascular coagulation (DIC) is the most frequent coagulation disorder associated with metastatic prostate adenocarcinoma. However, DIC with enhanced fibrinolysis as an initial presentation of prostate cancer is extremely rare. The appropriate treatment to control bleeding in these situations is challenging, controversial, and based on isolated case reports in the literature. A 66-year-old male presented at the emergency department with acute severe spontaneous ecchymoses localized to the limbs, laterocervical hematoma, and hemothorax. Prostate specific antigen level was 385 μg/L, bone scintigraphy revealed multiple bone metastases, and prostate biopsy confirmed adenocarcinoma (Gleason 9; 4 + 5). Laboratory investigation showed a pattern of enhanced fibrinolysis rather than the more common intravascular coagulation mechanism. Epsilon aminocaproic acid in monotherapy was initiated with a clear and rapid control of bleeding manifestations. This rare case of massive bleeding due to DIC with enhanced fibrinolysis as the first manifestation of prostate cancer suggests that in selected cases where the acute bleeding dyscrasia is clearly associated with a dominant fibrinolysis mechanism it is possible to use an approach of monotherapy with antifibrinolytics. PMID:27803823

  5. Porphyria and its neurologic manifestations.

    PubMed

    Tracy, Jennifer A; Dyck, P James B

    2014-01-01

    Porphyrias are rare disorders resulting from a defect in the heme biosynthetic pathway. They can produce significant disease of both the peripheral and central nervous systems, in addition to other organ systems, with acute intermittent porphyria, hereditary coproporphyria, and variegate porphyria as the subtypes associated with neurologic manifestations. The presence of a motor-predominant peripheral neuropathy (axonal predominant), accompanied by gastrointestinal distress and neuropsychiatric manifestations, should be a strong clue to the diagnosis of porphyria. Clinical confirmation can be made through evaluation of urine porphyrins during an exacerbation of disease. While hematin is helpful for acute treatment, long-term effective management requires avoidance of overstimulation of the cytochrome P450 pathway, as well as other risk factor control.

  6. Normobaric oxygen treatment in acute ischemic stroke: a clinical perspective

    PubMed Central

    Shi, Shu-hai; Qi, Zhi-feng; Luo, Yu-min; Ji, Xun-ming; Liu, Ke Jian

    2016-01-01

    Acute ischemic stroke is a common and serious neurological disease. Oxygen therapy has been shown to increase oxygen supply to ischemic tissues and improve outcomes after cerebral ischemia/reperfusion. Normobaric hyperoxia (NBO), an easily applicable and non-invasive method, shows protective effects on acute ischemic stroke animals and patients in pilot studies. However, many critical scientific questions are still unclear, such as the therapeutic time window of NBO, the long-term effects and the benefits of NBO in large clinic trials. In this article, we review the current literatures on NBO treatment of acute ischemic stroke in preclinical and clinical studies and try to analyze and identify the key gaps or unknowns in our understanding about NBO. Based on these analyses, we provide suggestions for future studies. PMID:27867482

  7. Widespread headache as the first clinical manifestation of giant cell arteritis in patients affected by polymyalgia rheumatica

    PubMed Central

    2016-01-01

    Introduction In giant cell arteritis (GCA) headache of new onset due to inflammatory involvement of the temporal artery (TA) represents a diagnostic criterion. A widespread headache (WH) with scalp tenderness due to cranial arteritis can represent another manifestation of GCA. Material and methods In 225 elderly patients with polymyalgia rheumatica (PMR) followed in our rheumatologic outpatient clinic from 2004 until June 2016, the frequency of WH as the first clinical manifestation of GCA was evaluated. Results Among 26 patients with GCA+PMR (11.6% of total), 5 (23.07%) had WH as first clinical manifestation of GCA without TA. In all these patients TA colour duplex sonography (CDS) and 18-fluorodeoxyglucose positron emission tomography (FDG-PET) with total body contrast-enhanced CT was consistent with the diagnosis of arteritis. TA biopsy was not performed. High doses of prednisone (1 mg/kg/day) led to the immediate and total disappearance of the headache. Conclusions The widespread headache should be considered as the first symptom GCA and in cases of suspicion of vasculitis patients should have a full diagnostics examination. Colour duplex sonography and FDG-PET with total body contrast-enhanced CT are useful tools for non-invasive diagnosis of GCA. PMID:27994267

  8. Enigmatic human tails: A review of their history, embryology, classification, and clinical manifestations.

    PubMed

    Tubbs, R Shane; Malefant, Jason; Loukas, Marios; Jerry Oakes, W; Oskouian, Rod J; Fries, Fabian N

    2016-05-01

    The presence of a human tail is a rare and intriguing phenomenon. While cases have been reported in the literature, confusion remains with respect to the proper classification, definition, and treatment methods. We review the literature concerning this anatomical derailment. We also consider the importance of excluding underlying congenital anomalies in these patients to prevent neurological deficits and other abnormal manifestations.

  9. Cutaneous Manifestations in Dermatomyositis: Key Clinical and Serological Features-a Comprehensive Review.

    PubMed

    Muro, Yoshinao; Sugiura, Kazumitsu; Akiyama, Masashi

    2016-12-01

    Dermatomyositis (DM) is a common idiopathic inflammatory myopathy. The pathogenesis is considered to be microangiopathy affecting skin and muscle. The cutaneous manifestations of DM are the most important aspect of this disease, and their correct evaluation is important for early diagnosis. The skin signs are various: Some are pathognomonic or highly characteristic, and others are compatible with DM. Recently, DM has been categorized into several disease subsets based on the various autoantibodies present in patients. Sometimes, characteristic cutaneous manifestations are strongly associated with the presence of specific autoantibodies. For example, anti-Mi-2 antibody is associated with the classic features of DM, including heliotrope rash, Gottron's papules, the V-neck sign, the shawl sign, cuticular overgrowth, and photosensitivity. Frequent cutaneous features in anti-transcriptional intermediary factor 1 gamma (TIF1γ)-positive patients are diffuse photoerythema, including "dusky red face," while skin ulcerations, palmar papules (inverse Gottron), diffuse hair loss, panniculitis, and oral pain and/or ulcers are sometimes associated with anti-melanoma differentiation-associated gene 5 product (MDA5) antibody. Here, we review important cutaneous manifestations seen in patients with DM, and we examine the relationship between the skin changes and myositis-associated autoantibodies. Correct evaluation of cutaneous manifestations and myositis-associated autoantibodies should help the clinician in the early diagnosis of DM, for a quick recognition of cutaneous signs that may be the symptom of onset before muscle inflammation.

  10. Paracoccidioidomycosis: acute-subacute clinical form, juvenile type*

    PubMed Central

    Marques, Silvio Alencar; Lastória, Joel Carlos; de Camargo, Rosangela Maria Pires; Marques, Mariangela Esther Alencar

    2016-01-01

    The authors report aspects of paracoccidioidomycosis, acute-subacute clinical form, juvenile type, in a 19-year-old female patient. Paracoccidioidomycosis, juvenile type, classically occurs in young patients, both sexes, with lymphoma-like aspects as initial presentation. However, following the natural history of the disease the lymph nodes assume patterns of infectious disease, as an abscess and fistulae. Systemic dissemination of the disease can occur and lethality and morbidity are significant in this clinical presentation. PMID:27438214

  11. Novel clinical manifestations in Pallister-Killian syndrome: comprehensive evaluation of 59 affected individuals and review of previously reported cases.

    PubMed

    Wilkens, Alisha; Liu, Hongbin; Park, Kristen; Campbell, Lindsey B; Jackson, Marie; Kostanecka, Anna; Pipan, Mary; Izumi, Kosuke; Pallister, Phillip; Krantz, Ian D

    2012-12-01

    Pallister-Killian syndrome is a rare, multi-system developmental diagnosis typically caused by tetrasomy of chromosome 12p that exhibits tissue-limited mosaicism. The spectrum of clinical manifestations in Pallister-Killian syndrome is wide and includes craniofacial anomalies, clefts, ophthalmologic, audiologic, cardiac, musculoskeletal, diaphragmatic, gastrointestinal, genitourinary, and cutaneous anomalies in association with intellectual disability and seizures. Growth parameters are often normal to elevated at birth with deceleration of growth postnatally. No formal estimate of the prevalence of Pallister-Killian syndrome has been made. Here, we report the clinical findings in 59 individuals with Pallister-Killian syndrome who were ascertained at Pallister-Killian syndrome Foundation family meetings held in the summers of 2006, 2008, 2009, and 2010. In addition, the clinical findings of 152 cases reported in the medical literature were reviewed and compared to the cohort examined here. Several novel clinical characteristics were identified through detailed dysmorphology examinations of this cohort and reassertion of a mild developmental variant is described. This report expands the clinical manifestations of Pallister-Killian syndrome and highlights the variable expressivity of this diagnosis with important implications for diagnosis and counseling.

  12. Outcome of B-Cell Acute Lymphoblastic Leukemia in Brazilian Children: Immunophenotypical, Hematological, and Clinical Evaluation.

    PubMed

    Cézar, Rodrigo S; Cerqueira, Bruno A V; da Paz, Silvana de Souza; Barbosa, Cynara G; de Moura Neto, José P; Barreto, José H de S; Goncalves, Marilda de S

    2015-08-01

    The aim of this study is to investigate the clinical, hematological, and immunophenotypic characteristics of Brazilian children with B-cell acute lymphoblastic leukemia (B-ALL) to identify prognostic biomarkers of the disease. Thirty-three children newly diagnosed with B-ALL were followed between March 2004 and December 2009. Information about the demographic profile, diagnosis, immunophenotype, clinical manifestations, and disease outcome were gathered from the patients' medical records. Of the 33 patients with B-ALL, 18 were male and 15 female. Eighteen patients were classified as high risk; 13 as low risk, and 2 as true low risk. The frequencies of cluster of differentiation (CD)10, CD19, and CD20 antigens were 69.7%, 81.8%, and 18.2%, respectively. Six patients (18.2%) had aberrant expression of myeloid antigens. At diagnosis, patients immunopositive for CD20 had elevated white blood cell counts (P = 0.018) and lower platelet counts (P = 0.017). The 6-year overall survival was 67.5%± 3.47%. Our results demonstrate the distinct immunophenotypic and prognostic characteristics of patients with B-ALL, which can be related to the Brazilian racial admixture. Consequently, these results will most likely aid in the selection of additional prognostic markers and their use in monitoring the clinical manifestations and treatment response among B-ALL patients.

  13. Cutaneous manifestations in neuro-oncology: clinically relevant tumor and treatment associated dermatologic findings.

    PubMed

    Strowd, Roy E; Strowd, Lindsay C; Blakeley, Jaishri O

    2016-06-01

    Skin findings are a rare but important aspect of the evaluation and management of patients with tumors of the nervous system. Skin findings have the highest prevalence in genetic tumor syndromes termed neuro-genodermatoses, which include neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and tuberous sclerosis. Skin changes are observed in patients with non-syndromic nervous system malignancy, often as a result of pharmacotherapy. The skin may also manifest findings in paraneoplastic conditions that affect the nervous system, providing an early indication of underlying neoplasm, including dermatomyosistis, neuropathic itch, and brachioradial pruritus. In this article, we review the major cutaneous findings in patients with tumors of the brain, spine, and peripheral nervous system focusing on (1) cutaneous manifestations of genetic and sporadic primary nervous system tumor syndromes, and (2) paraneoplastic neurological syndromes with prominent cutaneous features.

  14. Clinical and Neurologic Manifestation of Minimal Hepatic Encephalopathy and Overt Hepatic Encephalopathy.

    PubMed

    Basu, P Patrick; Shah, Niraj James

    2015-08-01

    Hepatic encephalopathy (HE) shows a wide spectrum of neuropsychiatric manifestations. A combined effort with neuropsychological and psychometric evaluation has to be performed to recognize the syndrome, whereas minimal HE (MHE) is largely under-recognized. Subtle symptoms of MHE can only be diagnosed through specialized neuropsychiatric testing. Early diagnosis and treatment may drastically improve the quality of life for many cirrhotic patients. Further research to gain better insight into the pathophysiology and diagnostic accuracy of HE will help determine future management strategies.

  15. Abdominal Tuberculosis with an Acute Abdomen: Our Clinical Experience

    PubMed Central

    Dey, Ramprasad; Bhattacharya, Ujjwal

    2014-01-01

    Introduction: Tuberculosis is an important cause of morbidity in India. Abdominal Tuberculosis is a great mimicker and is difficult to diagnose. This prospective observational study is based on those patients who were diagnosed to be suffering from Abdominal Tuberculosis only after they presented with an acute abdomen. This study aims to document the nature of different types of acute presentation in Abdominal Tuberculosis according to involved sites and surgical pathology. The study also discusses the indications and extent of surgical intervention. Materials and Methods: Seventy new cases of Abdominal Tuberculosis (out of 718 cases of acute abdomen) were diagnosed and treated over a period of three years in the surgical ward of Calcutta National Medical College. Macroscopic appearance of abdominal tissues during surgery suggested the diagnosis of tuberculosis. The diagnosis was confirmed by histopathology and tissue culture. All patients were subsequently treated with a full course of antitubercular drugs (ATD). Results: The clinical presentations of acute abdomen included acute intestinal obstruction, perforative peritonitis and acute appendicitis etc. Terminal ileum and ileocaecal region were predominantly involved. The most common pathology was intestinal stricture with or without perforation. Most of the patients (approx 78.5%) required emergency surgery as a therapeutic intervention. A two-stage procedure was preferred in peritonitis and sepsis. Most of the remaining patients (12.8%) required surgery after initial conservative treatment for the first few days. Undiagnosed Abdominal Tuberculosis represents a notable percentage (10%) of patients who present with an acute abdomen as a surgical emergency. Conclusion: Abdominal Tuberculosis is very difficult to diagnose and diagnosis is often delayed till an acute abdomen is presented with. Almost all patients needed surgical intervention. Irrespective of surgery, all patients of abdominal tuberculosis require a

  16. An insight into the gastrointestinal component of fibromyalgia: clinical manifestations and potential underlying mechanisms.

    PubMed

    Slim, Mahmoud; Calandre, Elena Pita; Rico-Villademoros, Fernando

    2015-03-01

    Fibromyalgia syndrome is characterized by chronic generalized pain accompanied by a broad symptomatologic spectrum. Besides chronic fatigue, sleep disturbances, headaches and cognitive dysfunction that are extensively described in the literature, a considerable proportion of patients with fibromyalgia experience gastrointestinal symptoms that are commonly overlooked in the studies that are not specifically dedicated to evaluate these manifestations. Nevertheless, various attempts were undertaken to explore the gastrointestinal dimension of fibromyalgia. Several studies have demonstrated an elevated comorbidity of irritable bowel syndrome (IBS) among patients with fibromyalgia. Other studies have investigated the frequency of presentation of gastrointestinal symptoms in fibromyalgia in a nonspecific approach describing several gastrointestinal complaints frequently reported by these patients such as abdominal pain, dyspepsia and bowel changes, among others. Several underlying mechanisms that require further investigation could serve as potential explanatory hypotheses for the appearance of such manifestations. These include sensitivity to dietary constituents such as gluten, lactose or FODMAPs or alterations in the brain-gut axis as a result of small intestinal bacterial overgrowth or subclinical enteric infections such as giardiasis. The gastrointestinal component of fibromyalgia constitutes a relevant element of the multidisciplinary pathophysiologic mechanisms underlying fibromyalgia that need to be unveiled, as this would contribute to the adequate designation of relevant treatment alternatives corresponding to these manifestations.

  17. Limb apraxia in acute ischemic stroke: a neglected clinical challenge?

    PubMed

    Schell, Caroline; Suchan, Julia; Himmelbach, Marc; Haarmeier, Thomas; Borchers, Svenja

    2014-04-01

    Symptoms of limb apraxia and executive dysfunctions are currently not explicitly considered by the National Institutes of Health Stroke Scale and, thus, not routinely tested by clinicians in the acute care of patients with suspected stroke. Neuropsychological testing, clinical examination, MRI, and functional magnetic resonance imaging (fMRI) were performed in a right-handed patient with acute onset of left-sided sensorimotor hemiparesis due to a right hemisphere ischemic stroke. Deficits in the execution of meaningless and meaningful gestures were not detected properly on initial clinical examination but were revealed later on through neuropsychological testing. Instead, the patient's inability to respond to specific instructions in the acute care setting was mistaken to reflect severe deficits in auditory comprehension. fMRI revealed right-hemispheric localization of language in the right-handed patient. We suggest including a bedside test for limb apraxia symptoms in acute clinical care of stroke patients. The distinction between deficits in limb praxis and impairments of language can be complicated owing to the common hemispheric co-localization of the two functions.

  18. [Blood-stasis and toxin causing catastrophe hypothesis and acute cardiovascular events: proposal of the hypothesis and its clinical significance].

    PubMed

    Xu, Hao; Shi, Da-Zhu; Yin, Hui-Jun

    2008-10-01

    A hypothesis of " blood-stasis and toxin causing catastrophe engender acute cardiovascular event (ACE)" was put forward according to TCM cognition on blood-stasis and toxin, in combining with the up to date concept of atherosclerosis and coronary heart disease, and together with our clinical practical experiences. The etiology, pathogenesis, evolving law, initial characteristics, clinical manifestation, therapeutic methods, prescriptions and their compatibility, as well as the well-suited time for applying TCM intervention were discussed. The authors stressed that it is of great significance for further reducing the morbidity of ACE and improving the effect of integrative medicine for preventing and treating cardiovascular thrombotic disease.

  19. Influence of previous aspirin treatment and smoking on the electrocardiographic manifestations of injury in acute myocardial infarction

    PubMed Central

    Kennon, S; Barakat, K; Suliman, A; MacCallum, P; Ranjadayalan, K; Wilkinson, P; Timmis, A

    2000-01-01

    OBJECTIVE—To examine demographic and clinical characteristics of patients with acute myocardial infarction in order to identify factors affecting the electrocardiographic evolution of injury.
METHODS—Prospective cohort study of 1399 consecutive patients with a first myocardial infarction. Baseline clinical data associated with ST elevation and Q wave development were identified and 12 month survival was estimated.
RESULTS—Smoking had complex effects on the evolution of injury, increasing the odds of ST elevation (odds ratio (OR) 1.61; 95% confidence interval (CI) 1.08 to 2.36), but reducing the odds of Q wave development (OR 0.69, 95% CI 0.49 to 0.96). The effects of previous aspirin treatment were more consistent with reductions in the odds of ST elevation (OR 0.57, 95% CI 0.35 to 0.94) and Q wave development (OR 0.53, 95% CI 0.34 to 0.84). ST elevation and Q wave development were both associated with an adverse prognosis, with estimated 12 month survival rates of 80.6% (95% CI 78.2% to 83.1%) and 80.0% (95% CI 77.5% to 82.5%), respectively, compared with 86.5% (95% CI 81.2% to 91.9%) and 89.9% (95% CI 86.2% to 93.7%) for patients without these ECG changes.
CONCLUSIONS—The thrombogenicity of the blood may be a major determinant of infarct severity. Smoking increases thrombogenicity and the likelihood of ST elevation, but because coronary occlusion is relatively more thrombotic in smokers, responses to both endogenous and exogenous thrombolysis are better, reducing the risk of Q wave development. Previous aspirin treatment reduces thrombogenicity, protecting against ST elevation and Q wave development.


Keywords: myocardial infarction; smoking; aspirin PMID:10862586

  20. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

    PubMed Central

    Walker, U A; Tyndall, A; Czirják, L; Denton, C; Farge‐Bancel, D; Kowal‐Bielecka, O; Müller‐Ladner, U; Bocelli‐Tyndall, C; Matucci‐Cerinic, M

    2007-01-01

    Background Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with lcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs lcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion dcSSc and lcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody‐based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally. PMID:17234652

  1. [Characteristics of immunologic changes and clinical manifestations of allergy in children with impaired thyroid function].

    PubMed

    Illek, Ia Iu; Ganiev, A G; Makhmudzhanova, K A; Alimdzhanova, I I

    1990-01-01

    The majority of children with allergic diathesis, living in the Andizhan region of the Uzbek SSR manifest the signs of stable abnormalities of thyroid function, that aggravate immunological alterations and the course of the process. This prompts the use in these children of therapeutic measures aimed at correction of the activity of the thyroid apparatus. With this purpose in view, the children with allergic diathesis associated with increased thyroid function may be administered aqueous solution of potassium iodide whereas those with functional thyroid deficiency may be given thyroidin in proper age-associated doses.

  2. [Abnormality of blood coagulation indexes in patients with de novo acute leukemia and its clinical significance].

    PubMed

    Xiao, Fang-Fang; Hu, Kai-Xun; Guo, Mei; Qiao, Jian-Hui; Sun, Qi-Yun; Ai, Hui-Sheng; Yu, Chang-Lin

    2013-04-01

    To explore hemorrhage risk and the clinical significance of abnormal change of prothrombin time (PT), activated partial thromboplastin time (APTT), plasma fibrinogen (FIB), plasma thrombin time (TT) and d-dimer (D-D) in de novo acute leukemia (except for APL), the different bleeding manifestations of 114 cases of de novo acute leukemia with different coagulation indexes were analyzed retrospectively. The correlation between these blood coagulation indexes and the possible correlative clinical characteristics were analysed, including age, sex, type of acute leukemia, initial white blood cell(WBC) and platelet(Plt) count, the proportion of blast cells in bone marrow and cytogenetic abnormality of patients at diagnosis. The results indicated that the incidence of abnormal blood coagulation was as high as 78.1% for de novo AL patients. These patients with 5 normal blood coagulation indexes may have mild bleeding manifestation, but the more abnormal indexes, the more severe bleeding. Both PT and D-D were sensitive indexes for diagnosis of level II bleeding. Incidence of abnormal blood coagulation significantly correlates with the proportion of blast cells in bone marrow (χ(2) = 4.184, OR = 1.021, P < 0.05) and more with D-D (P < 0.01), while age, sex, type of AL, WBC count, Plt count and abnormality of cytogenetics did not correlate with abnormal blood coagulation. It is concluded that the coagulation and fibrinolysis are abnormal in most patients with de novo acute leukemia. More abnormal indexes indicate more severe bleeding, and both PT and D-D are sensitive indexes for diagnosis of level II bleeding. Higher proportion of blast cells in bone marrow predicts higher incidence of abnormal blood clotting. Acute leukemia with elderly age, high white blood cell count and adverse cytogenetics do not predict severer abnormal blood clotting. Detection of PT, APTT, TT, FIB, and D-D may help to judge whether the patients are in a state of hypercoagulability or disseminated

  3. MUCOCUTANEOUS MANIFESTATIONS OF CHIKUNGUNYA FEVER

    PubMed Central

    Bandyopadhyay, Debabrata; Ghosh, Sudip Kumar

    2010-01-01

    Chikungunya fever (CF) is an arboviral acute febrile illness transmitted by the bite of infected Aedes mosquitoes. After a quiescence of more than three decades, CF has recently re-emerged as a major public health problem of global scale. CF is characterized by an acute onset of high fever associated with a severe disabling arthritis often accompanied by prominent mucocutaneous manifestations. The disease is usually self-limiting, but the joint symptoms and some of the cutaneous features may persist after the defervescence. A wide range of mucocutaneous changes has been described to occur in association with CF during the current epidemic. Besides a morbilliform erythema, hyperpigmentation, xerosis, excoriated papules, aphthous-like ulcers, vesiculobullous and lichenoid eruptions, and exacerbation of pre-existing or quiescent dermatoses had been observed frequently. These unusual features may help in the clinical differential diagnosis of acute viral exanthems mimicking CF. PMID:20418982

  4. Comparative analysis of a novel disease phenotype network based on clinical manifestations

    PubMed Central

    Chen, Yang; Zhang, Xiang; Zhang, Guo-qiang; Xu, Rong

    2015-01-01

    Systems approaches to analyzing disease phenotype networks in combination with protein functional interaction networks have great potential in illuminating disease pathophysiological mechanisms. While many genetic networks are readily available, disease phenotype networks remain largely incomplete. In this study, we built a large-scale Disease Manifestation Network (DMN) from 50,543 highly accurate disease-manifestation semantic relationships in the United Medical Language System (UMLS). Our new phenotype network contains 2305 nodes and 373,527 weighted edges to represent the disease phenotypic similarities. We first compared DMN with the networks representing genetic relationships among diseases, and demonstrated that the phenotype clustering in DMN reflects common disease genetics. Then we compared DMN with a widely-used disease phenotype network in previous gene discovery studies, called mimMiner, which was extracted from the textual descriptions in Online Mendelian Inheritance in Man (OMIM). We demonstrated that DMN contains different knowledge from the existing phenotype data source. Finally, a case study on Marfan syndrome further proved that DMN contains useful information and can provide leads to discover unknown disease causes. Integrating DMN in systems approaches with mimMiner and other data offers the opportunities to predict novel disease genetics. We made DMN publicly available at nlp/case.edu/public/data/DMN. PMID:25277758

  5. Imbalanced presence of Borrelia burgdorferi s.l. multilocus sequence types in clinical manifestations of Lyme borreliosis.

    PubMed

    Coipan, E Claudia; Jahfari, Setareh; Fonville, Manoj; Oei, G Anneke; Spanjaard, Lodewijk; Takumi, Katsuhisa; Hovius, Joppe W R; Sprong, Hein

    2016-08-01

    In this study we used typing based on the eight multilocus sequence typing scheme housekeeping genes (MLST) and 5S-23S rDNA intergenic spacer (IGS) to explore the population structure of Borrelia burgdorferi sensu lato isolates from patients with Lyme borreliosis (LB) and to test the association between the B. burgdorferi s.l. sequence types (ST) and the clinical manifestations they cause in humans. Isolates of B. burgdorferi from 183 LB cases across Europe, with distinct clinical manifestations, and 257 Ixodes ricinus lysates from The Netherlands, were analyzed for this study alone. For completeness, we incorporated in our analysis also 335 European B. burgdorferi s.l. MLST profiles retrieved from literature. Borrelia afzelii and Borrelia bavariensis were associated with human cases of LB while Borrelia garinii, Borrelia lusitaniae and Borrelia valaisiana were associated with questing I. ricinus ticks. B. afzelii was associated with acrodermatitis chronica atrophicans, while B. garinii and B. bavariensis were associated with neuroborreliosis. The samples in our study belonged to 251 different STs, of which 94 are newly described, adding to the overall picture of the genetic diversity of Borrelia genospecies. The fraction of STs that were isolated from human samples was significantly higher for the genospecies that are known to be maintained in enzootic cycles by mammals (B. afzelii, B. bavariensis, and Borrelia spielmanii) than for genospecies that are maintained by birds (B. garinii and B. valaisiana) or lizards (B. lusitaniae). We found six multilocus sequence types that were significantly associated to clinical manifestations in humans and five IGS haplotypes that were associated with the human LB cases. While IGS could perform just as well as the housekeeping genes in the MLST scheme for predicting the infectivity of B. burgdorferi s.l., the advantage of MLST is that it can also capture the differential invasiveness of the various STs.

  6. Carney complex and McCune Albright syndrome: an overview of clinical manifestations and human molecular genetics.

    PubMed

    Salpea, Paraskevi; Stratakis, Constantine A

    2014-04-05

    Endocrine neoplasia syndromes feature a wide spectrum of benign and malignant tumors of endocrine and non-endocrine organs associated with other clinical manifestations. This study outlines the main clinical features, genetic basis, and molecular mechanisms behind two multiple endocrine neoplasia syndromes that share quite a bit of similarities, but one can be inherited whereas the other is always sporadic, Carney complex (CNC) and McCune-Albright (MAS), respectively. Spotty skin pigmentation, cardiac and other myxomas, and different types of endocrine tumors and other characterize Carney complex, which is caused largely by inactivating Protein kinase A, regulatory subunit, type I, Alpha (PRKAR1A) gene mutations. The main features of McCune-Albright are fibrous dysplasia of bone (FD), café-au-lait macules and precocious puberty; the disease is caused by activating mutations in the Guanine Nucleotide-binding protein, Alpha-stimulating activity polypeptide (GNAS) gene which are always somatic. We review the clinical manifestations of the two syndromes and provide an update on their molecular genetics.

  7. [Specific clinical and functional features in the patients presenting with neurologic manifestations of deforming dorsopathies and concomitant osteoarthrosis].

    PubMed

    Miriutova, N F; Zaĭtsev, A A; Popova, A V

    2012-01-01

    The present study has revealed some specific clinical and functional features in the patients presenting with neurologic manifestations of deforming dorsopathies and concomitant osteoarthrosis. The negative influence of the latter condition on the pain intensity, microcirculation, and duration of the exacerbation of neurologic syndrome has been demonstrated based on the analysis of correlational relationships between the severity of referred projectional nerve root pain and articular pain syndrome. Special emphasis is laid on the interrelation between the duration of pain syndrome and characteristics of microcirculation.

  8. Acute febrile torticollis in youth: clinical investigation and current management

    PubMed Central

    Ouattassi, Naouar; Chmiel, Mohammed; Kerouiti, Zakaria El; Ridal, Mohammed; Alami, Mohammed Nouredine

    2015-01-01

    Acute febrile torticollis in children is a rare and a special clinical picture of variable causes. It may indicate an inflammatory or an infectious pathology affecting any of the anatomical structures of the neck. Treatment is quite clearly defined, and it may be a therapeutic emergency. It is a condition that all ENT specialists must be familiar with since they are most likely to be the first physician to whom such a child is brought PMID:26328000

  9. Simultaneous presentation of acute disseminated encephalomyelitis (ADEM) and systemic lupus erythematosus (SLE) after enteroviral infection: can ADEM present as the first manifestation of SLE?

    PubMed

    Kim, J-M; Son, C-N; Chang, H W; Kim, S-H

    2015-05-01

    Central Nervous System (CNS) involvement of Systemic Lupus Erythematosus (SLE) includes a broad range of neuropsychiatric syndromes. Acute Disseminated Encephalomyelitis (ADEM) is a demyelinating CNS disorder characterized by encephalopathy and multifocal lesions predominantly involving the white matter on brain magnetic resonance imaging. ADEM associated with SLE has been only rarely reported. We report an unusual case of a 17-year-old girl who developed ADEM after enteroviral infection as the first manifestation of SLE. The authors emphasize that the patient's illness was preceded by enteroviral infection and that ADEM occurred before any other symptoms of SLE, which makes this case unique.

  10. Anderson-Fabry disease: clinical manifestations and impact of disease in a cohort of 98 hemizygous males

    PubMed Central

    MacDermot, K; Holmes, A; Miners, A

    2001-01-01

    OBJECTIVES—To determine the natural history of Anderson-Fabry disease (AFD) as a baseline for efficacy assessment of potentially therapeutic drugs.
DESIGN—The first large cross sectional study of a patient cohort from the AFD clinical and genetic register (UK), maintained for the last 15 years.
MEASURES—Prevalence, mortality, frequency of AFD manifestations, and impact of disease on patient lives, assessed from the AFD register and the disease specific questionnaire.
RESULTS—The median cumulative survival was 50 years (n=51), which represents an approximately 20 year reduction of life span. Neuropathic pain was present in 77% (n=93) with mean pain score of 5 (scale 0-10) despite treatment with anticonvulsants and opiates. Pain stopped in only 11%. Cerebrovascular complications developed in 24.2% and renal failure in 30%. The onset and progression of serious AFD manifestations was highly variable. The relationship of gastrointestinal manifestations on weight, using body mass index (BMI), was significant (p=0.01). High frequency sensorineural deafness was confirmed in 78% of audiograms. Neuropathic pain and angiokeratoma were absent in five adult males (~5%). Median age at diagnosis of AFD was 21.9 years (n=64).
IMPACT OF DISEASE—Attendance at school, sports, and social activity were significantly affected by AFD. Only 56.6% (n=46) of patients were employed. Psychosexual effects of genital angiokeratoma, genital pain, and impotence were not previously recognised.
CONCLUSION—The majority of males experience multiple disease manifestations and the duration of neuropathic pain was lifelong. The AFD register proved useful for the determination of baseline disease parameters in this cohort.


Keywords: Anderson-Fabry disease; natural history; mortality; prevalence PMID:11694547

  11. [Complete atrioventricular block as the first clinical manifestation of a tick bite (Lyme disease)].

    PubMed

    Bacino, Luca; Gazzarata, Massimo; Siri, Giambattista; Cordone, Stefano; Bellotti, Paolo

    2011-03-01

    A 52-year-old male patient presented to the emergency department because of malaise and frequent dizziness. The ECG revealed high-grade atrioventricular block that required placement of a temporary pacemaker. There were no other abnormalities in physical and echocardiographic examination, and coronary angiography excluded the presence of coronary artery disease. IgM and IgG antibodies against Borrelia were positive, and antibiotic therapy with ceftriaxone at the dose of 2 g/die for 15 days resulted in rapid regression of atrioventricular block. Seven-day ECG recording immediately after discharge and 24h ECG monitoring at 40 days confirmed the total disappearance of atrioventricular block. This represents a case of atrioventricular block as the first manifestation of Borrelia infection (Lyme disease). A prompt diagnosis and antibiotic therapy usually result in complete resolution of atrioventricular block without the need for a permanent pacemaker.

  12. Clinical and Biochemical Manifestations of Depression: Relation to the Neurobiology of Stress

    PubMed Central

    Gold, Phillip W.; Machado-Vieira, Rodrigo; Pavlatou, Maria G.

    2015-01-01

    Major depressive disorder (MDD) is a chronic, recurrent, and severe psychiatric disorder with high mortality and medical comorbidities. Stress-related pathways have been directly involved in the pathophysiology and treatment of MDD. The present paper provides an overview on the stress system as a model to understand key pathophysiological paradigms in MDD. These mechanisms involve behavioral, cognitive, and systemic manifestations and are also associated with the mechanisms of action of effective antidepressants. Aspects such as depression subtypes, inflammation, insulin resistance, oxidative stress, and prothrombotic states in critical brain circuits and periphery are critically appraised. Finally, new strategies for approaching treatment-resistant major depression and potential adverse effects associated with this complex and intricate network are highlighted. The authors used PubMed as the database for this review. Each author extracted relevant data and assessed the methodological quality of each study. PMID:25878903

  13. Clinical and pathological manifestations of cardiovascular disease in rat models: the influence of acute ozone exposure

    EPA Science Inventory

    This paper shows that rat models of cardiovascular diseases have differential degrees of underlying pathologies at a young age. Rodent models of cardiovascular diseases (CVD) and metabolic disorders are used for examining susceptibility variations to environmental exposures. How...

  14. Mucocutaneous Manifestations of HIV and the Correlation with WHO Clinical Staging in a Tertiary Hospital in Nigeria

    PubMed Central

    Oninla, Olumayowa Abimbola

    2014-01-01

    Skin diseases are indicators of HIV/AIDS which correlates with WHO clinical stages. In resource limited environment where CD4 count is not readily available, they can be used in assessing HIV patients. The study aims to determine the mucocutaneous manifestations in HIV positive patients and their correlation with WHO clinical stages. A prospective cross-sectional study of mucocutaneous conditions was done among 215 newly diagnosed HIV patients from June 2008 to May 2012 at adult ART clinic, Wesley Guild Hospital Unit, OAU Teaching Hospitals Complex, Ilesha, Osun State, Nigeria. There were 156 dermatoses with oral/oesophageal/vaginal candidiasis (41.1%), PPE (24.4%), dermatophytic infections (8.9%), and herpes zoster (3.8%) as the most common dermatoses. The proportions of dermatoses were 4.5%, 21.8%, 53.2%, and 20.5% in stages 1–4, respectively. A significant relationship (using Pearson's Chi square with P value <0.05) was obtained between dermatoses and WHO clinical stages. Pearson's correlation coefficient showed a positive correlation between the number of dermatoses and the WHO clinical stages. Dermatoses can therefore serve as diagnostic and prognostic markers in resource limited settings to initiate HAART in clinical stages 3 and 4. PMID:25587439

  15. Serum tonicity, extracellular volume and clinical manifestations in symptomatic dialysis-associated hyperglycemia treated only with insulin.

    PubMed

    Tzamaloukas, A H; Rohrscheib, M; Ing, T S; Siamopoulos, K C; Elisaf, M F; Spalding, C T

    2004-09-01

    The absence of osmotic diuresis modifies the effects of hyperglycemia on body fluids in patients with advanced renal failure. To determine the relationship between clinical manifestations and abnormalities in tonicity and extracellular volume in such patients, we analyzed 43 episodes of severe dialysis-associated hyperglycemia (serum glucose exceeding 600 mg/dL) treated only with insulin. The main manifestations were dyspnea in 22 cases (pulmonary edema in 19), nausea and vomiting in 15, coma in 13 and seizures in 3, while 5 patients had no symptoms. Treatment with insulin resulted in a decrease in serum glucose value from 913 +/- 197 mg/dL to 170 +/- 78 mg/dL, an increase in serum sodium level from 125 +/- 5 to 136 +/- 5 mmol/L, and a fall in calculated serum tonicity value from 300 +/- 13 to 282 +/- 11 mmol/kg (all at p < 0.001). The ratio of the change in serum sodium level over change in serum glucose concentration was -1.50 +/- 0.22 mmol/L per 100 mg/dL. The percent increase in extracellular volume secondary to hyperglycemia developing from the prior euglycemic state and calculated from changes in serum sodium and chloride concentrations, was 10.9% +/- 4.6% (1.5% +/- 0.6% per 100 mg/dL increase in serum glucose level). All clinical manifestations dissipated after correction of hyperglycemia in 42 patients. One woman developed during treatment a fatal myocardial infarction. Dialysis patients with severe hyperglycemia may develop symptoms as a result of hypertonicity and extracellular expansion. Insulin alone may be sufficient treatment for these symptoms. The changes in serum tonicity and electrolytes during treatment are consistent with theoretical predictions.

  16. Acute fatty liver of pregnancy: a clinical study of 12 episodes in 11 patients.

    PubMed Central

    Reyes, H; Sandoval, L; Wainstein, A; Ribalta, J; Donoso, S; Smok, G; Rosenberg, H; Meneses, M

    1994-01-01

    Twelve episodes of acute fatty liver of pregnancy (AFLP) were diagnosed in 11 patients during the past 18 years in a general hospital in Santiago, Chile, with a prevalence of 1 per 15,900 deliveries. Acute fatty liver of pregnancy started between the 31st and 38th weeks of pregnancy, with malaise, vomiting, jaundice, and lethargy as the main clinical manifestations. Polydipsia (in nine episodes) and skin pruritus (in seven episodes) were unusual clinical findings. In two patients, pruritus started two and four weeks before AFLP, suggesting that an intrahepatic cholestasis of pregnancy preceded AFLP in those patients. Considering the current prevalence of both diseases in Chile, their association should be considered fortuitous. In another patient, two consecutive pregnancies were affected by AFLP, raising to three the number of reported patients with recurrent AFLP. In 11 episodes, liver biopsies supported the diagnosis of AFLP by showing small and midsized vacuolar cytoplasmic transformation as the most prominent histopathological feature. Positive intracellular fat staining was found in the four samples analysed. Studies by electron microscopy showed megamitochondria with paracrystalline inclusions in four samples. All the mothers survived, but fetal mortality was 58.3%. Several extrahepatic complications delayed maternal recovery for up to four weeks after delivery. This study confirms an improvement in maternal prognosis in AFLP, discusses the possibility of an epidemiological association with intrahepatic cholestasis of pregnancy, and increases the number of patients reported with recurrent AFLP. Images Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 PMID:8307428

  17. Neurologic manifestations of von Hippel-Lindau disease. Grand Rounds at the Clinical Center of the National Institutes of Health

    PubMed Central

    Butman, John A.; Linehan, W. Marston; Lonser, Russell R.

    2012-01-01

    von Hippel-Lindau disease (VHL) is an autosomal dominant neoplasia syndrome that is the result of a germline mutation of the VHL tumor suppressor gene on the short arm of chromosome 3. VHL patients are predisposed to develop lesions of the central nervous system (CNS) and viscera. CNS lesions include hemangioblastomas (the most common tumor in VHL) and endolymphatic sac tumors (ELSTs). Visceral manifestations include renal carcinomas and cysts, pancreatic neuroendocrine tumors and cysts, pheochromocytomas and cystadenomas of the reproductive adnexal organs. Despite their benign pathology, hemangioblastomas and ELSTs are a frequent cause of morbidity and mortality in VHL patients. Recent molecular biologic investigations into these VHL-associated CNS lesions provide new insight into their origin and development. Emerging data from serial imaging and clinical surveillance protocols provide insight into the natural history of these lesions. Because of the dissimilar pathobiology and clinical course between hemangioblastomas and ELSTs, the optimal management strategies for these neurologic manifestations of VHL are very different. PMID:18799446

  18. Peroxisome biogenesis disorders in the Zellweger spectrum: An overview of current diagnosis, clinical manifestations, and treatment guidelines

    PubMed Central

    Braverman, Nancy E.; Raymond, Gerald V.; Rizzo, William B.; Moser, Ann B.; Wilkinson, Mark E.; Stone, Edwin M.; Steinberg, Steven J.; Wangler, Michael F.; Rush, Eric T.; Hacia, Joseph G.

    2016-01-01

    Peroxisome biogenesis disorders in the Zellweger spectrum (PBD-ZSD) are a heterogeneous group of genetic disorders caused by mutations in PEX genes responsible for normal peroxisome assembly and functions. As a result of impaired peroxisomal activities, individuals with PBD-ZSD can manifest a complex spectrum of clinical phenotypes that typically result in shortened life spans. The extreme variability in disease manifestation ranging from onset of profound neurologic symptoms in newborns to progressive degenerative disease in adults presents practical challenges in disease diagnosis and medical management. Recent advances in biochemical methods for newborn screening and genetic testing have provided unprecedented opportunities for identifying patients at the earliest possible time and defining the molecular bases for their diseases. Here, we provide an overview of current clinical approaches for the diagnosis of PBD-ZSD and provide broad guidelines for the treatment of disease in its wide variety of forms. Although we anticipate future progress in the development of more effective targeted interventions, the current guidelines are meant to provide a starting point for the management of these complex conditions in the context of personalized health care. PMID:26750748

  19. CLINICAL AND THERAPEUTIC CORRELATIONS IN PATIENTS WITH SLIGHT ACUTE PANCREATITIS

    PubMed Central

    MUNHOZ-FILHO, Clewis Henri; BATIGÁLIA, Fernando; FUNES, Hamilton Luiz Xavier

    2015-01-01

    Background Acute pancreatitis is an inflammatory disease of the pancreas due to enzymatic autodigestion which can cause necrosis or multiple organ failure; its pathophysiology is not fully known yet. Aim To evaluate the correlation between clinical and therapeutic data in patients with mild acute pancreatitis. Methods A retrospective study in 55 medical records of patients admitted with acute mild pancreatitis was realized to analyze the association between age, leukocytosis, serum glutamic-oxaloacetic transaminase and lactate dehydrogenase, glucose, antibiotics, time admission and Ranson´s scores. Results There was a positive association between less intensive care (strict hydration, analgesia and monitoring of vital signs), early antibiotic therapy (monotherapy), early return to diet after 48 hours and laboratory control of the serum amylase and lipase (high in the first week and decreasing after 10 days, without any prognostic value). Conclusions Changes in the management of patients with mild acute pancreatitis, such as enteral nutrition, rational use of lower spectrum antibiotics and intensive care, have contributed significantly to the reduction of hospitalization time and mortality. PMID:25861064

  20. Influence of the bacterial phenotypes on the clinical manifestations in Klebsiella pneumoniae bacteremia patients: A retrospective cohort study.

    PubMed

    Togawa, Atsushi; Toh, Hiromi; Onozawa, Kyoko; Yoshimura, Michinobu; Tokushige, Chiemi; Shimono, Nobuyuki; Takata, Tohru; Tamura, Kazuo

    2015-07-01

    Ninety-four episodes of Klebsiella pneumoniae bloodstream infection were identified at a university hospital in Japan. After excluding extended-spectrum beta lactamase-producing strains, 83 blood isolates from these patients were assayed in terms of their bacterial phenotypes such as the mucoid and hypermucoviscosity phenotypes. Bacterial phenotypes were correlated with the patients' clinical manifestations. The hypermucoviscosity phenotype was significantly associated with septic shock at the onset of infections (odds ratio, 15.92; 95% confidence interval, 1.27-468.12), but was not associated with liver abscess formation. Mortality was determined by the presence of septic shock. RmpA gene was associated with the induction of the hypermucoviscosity phenotype. These results reveal unique roles of bacterial phenotypes on the patient's clinical condition in K. pneumoniae bacteremia.

  1. Epidemiology, clinical manifestations, and molecular typing of salmonella typhi isolated from patients with typhoid fever in Lebanon.

    PubMed

    Kanj, Souha S; Kanafani, Zeina A; Shehab, Marwa; Sidani, Nisreen; Baban, Tania; Baltajian, Kedak; Dakdouki, Ghenwa K; Zaatari, Mohamad; Araj, George F; Wakim, Rima Hanna; Dbaibo, Ghassan; Matar, Ghassan M

    2015-06-01

    The objective of this study was to examine the epidemiology and the clinical manifestations of typhoid fever as well as the susceptibility and strain relatedness of Salmonella typhi isolates in Lebanon from 2006 to 2007. A total of 120 patients with typhoid fever were initially identified from various areas of the country based on positive culture results for S. typhi from blood, urine, stools, bone marrow and/or positive serology. Clinical, microbiological and molecular analysis was performed on cases with complete data available. These results indicated that drinking water was an unlikely mode of transmission of the infection. Despite increasing reports of antimicrobial resistance among S. typhi isolates, the vast majority of these isolates were susceptible to various antibiotic agents, including ampicillin, cephalosporins, quinolones, and trimethoprim/sulfamethoxazole. Molecular analysis of the isolates revealed a predominance of one single genotype with no variation in distribution across the geographical regions.

  2. Clinical Manifestations, Epidemiology, and Laboratory Diagnosis of Human Monocytotropic Ehrlichiosis in a Commercial Laboratory Setting

    PubMed Central

    Olano, Juan P.; Hogrefe, Wayne; Seaton, Brent; Walker, David H.

    2003-01-01

    Clinical, epidemiological, and laboratory diagnostic issues of human monocytotropic ehrlichiosis (HME) were investigated in a retrospective case study conducted at a national reference laboratory (Focus Technologies, formerly MRL Reference Laboratory), and at the University of Texas Medical Branch at Galveston, Texas, during 1997 and 1998. Standard questionnaires were sent to physicians for each laboratory-diagnosed patient 2 days to 2 weeks after immunofluorescent antibody assay results were available. Among the 41 cases for which data were obtained, 32 (78%) were definite cases of HME, and 9 (22%) were probable cases of HME. Tick bite or exposure to ticks was recorded in more than 97% of cases. The most prominent clinical findings were fever, abdominal tenderness, and regional lymphadenopathy. There was an association between age and severity of illness. The main laboratory findings included leukopenia, thrombocytopenia, and elevated aspartate aminotransferase and alanine aminotransferase. Clinical and laboratory findings were nonspecific and were not good predictors of the severity of illness. The 90% of patients who received doxycycline treatment underwent rapid clinical improvement with a favorable outcome. The usual duration of effective treatment with doxycycline was 7 to 10 days. This retrospective study is unique because it was based in a commercial reference laboratory setting that receives specimens from different geographic locations. The clinical and laboratory information from 41 patients provides insight into the epidemiological, clinical, and laboratory characteristics of HME. PMID:12965923

  3. Clinical epidemiology of acute hepatitis C in South America.

    PubMed

    Dirchwolf, Melisa; Marciano, Sebastián; Mauro, Ezequiel; Ruf, Andrés Eduardo; Rezzonico, Lucrecia; Anders, Margarita; Chiodi, Daniela; Petta, Néstor Gill; Borzi, Silvia; Tanno, Federico; Ridruejo, Ezequiel; Barreyro, Fernando; Shulman, Carolina; Plaza, Pablo; Carbonetti, Rodolfo; Tadey, Luciana; Schroder, Teresa; Fainboim, Hugo

    2017-02-01

    There is scarce data pertaining to acute hepatitis C (aHC) infection in South America. We aimed to describe clinical characteristics and evolution of aHC in a South American cohort. A retrospective survey was conducted at 13 hepatology units. All patients ≥16 years old with aHC diagnosis were included. Demographic, clinical and outcome information were registered in a standardized ad hoc questionnaire. Sixty-four patients were included. The majority were middle-aged (median age: 46 years) and female (65.6%); most of them were symptomatic at diagnosis (79.6%). HCV-1 was the most prevalent genotype (69.2%). Five patients had liver failure: three cases of severe acute hepatitis, one case of fulminant hepatitis and one case of acute-on-chronic liver failure. Nosocomial exposure was the most prevalent risk factor. Evolution was assessed in 46 patients. In the untreated cohort, spontaneous resolution occurred in 45.8% and was associated with higher values of AST/ALT and with the absence of intermittent HCV RNA viremia (P = 0.01, 0.05, and 0.01, respectively). In the treated cohort, sustained virological response was associated with nosocomial transmission and early treatment initiation (P = 0.04 each). The prevalence of nosocomial transmission in this South-American cohort of aHC stresses the importance of following universal precautions to prevent HCV infection. J. Med. Virol. 89:276-283, 2017. © 2016 Wiley Periodicals, Inc.

  4. Clinical signs of dysphagia in infants with acute viral bronchiolitis☆

    PubMed Central

    Barbosa, Lisiane De Rosa; Gomes, Erissandra; Fischer, Gilberto Bueno

    2014-01-01

    Objective: To determine the occurrence of clinical signs of dysphagia in infants with acute viral bronchiolitis, to compare the respiratory parameters during deglutition, and to ensure the intra- and inter- examiners agreement, as well as to accomplish intra and interexaminators concordance of the clinical evaluation of the deglutition. Methods: This was a cross-sectional study of 42 infants aged 0-12 months. The clinical evaluation was accompanied by measurements of respiratory rate and pulse oximetry. A score of swallowing disorders was designed to establish associations with other studied variables and to ensure the intra- and interrater agreement of clinical feeding assessments. Caregivers also completed a questionnaire about feeding difficulties. Significance was set at p<0.05. Results: Changes in the oral phase (prolonged pauses) and pharyngeal phase (wheezing, coughing and gagging) of swallowing were found. A significant increase in respiratory rate between pre- and post-feeding times was found, and it was determined that almost half of the infants had tachypnea. An association was observed between the swallowing disorder scores and a decrease in oxygen saturation. Infants whose caregivers reported feeding difficulties during hospitalization stated a significantly greater number of changes in the swallowing evaluation. The intra-rater agreement was considered to be very good. Conclusions: Infants with acute viral bronchiolitis displayed swallowing disorders in addition to changes in respiratory rate and measures of oxygen saturation. It is suggested, therefore, that infants displaying these risk factors have a higher probability of dysphagia. PMID:25479843

  5. Autoimmune Syndromes Presenting as a Paraneoplastic Manifestation of Myelodysplastic Syndromes: Clinical Features, Course, Treatment and Outcome

    PubMed Central

    Williamson, Bradley T.; Foltz, Lynda; Leitch, Heather A.

    2016-01-01

    Autoimmune manifestations (AIM) are reported in up to 10-30% of myelodysplastic syndromes (MDS) patients; this association is not well defined. We present herein a retrospective chart review of single center MDS patients for AIM, a case discussion and a literature review. Of 252 MDS patients examined, 11 (4.4%) had AIM around MDS diagnosis. International Prognostic Scoring System scores were: low or intermediate (int)-1 (n=7); int-2 or high (n=4). AIM were: culture negative sepsis (n=7); inflammatory arthritis (n=3); vasculitis (n=4); sweats; pericarditis; polymyalgia rheumatica (n=2 each); mouth ulcers; pulmonary infiltrates; suspicion for Behcet’s; polychondritis and undifferentiated (n=1 each). AIM treatment and outcome were: prednisone +/- steroid sparing agents, n=8, ongoing symptoms in 5; azacitidine (n=3), 2 resolved; and observation, n=1, ongoing symptoms. At a median follow up of 13 months, seven patients are alive. In summary, 4.4% of MDS patients presented with concomitant AIM. MDS should remain on the differential diagnosis of patients with inflammatory symptoms. PMID:27499837

  6. Systemic manifestations of traumatic brain injury.

    PubMed

    Gaddam, Samson Sujit Kumar; Buell, Thomas; Robertson, Claudia S

    2015-01-01

    Traumatic brain injury (TBI) affects functioning of various organ systems in the absence of concomitant non-neurologic organ injury or systemic infection. The systemic manifestations of TBI can be mild or severe and can present in the acute phase or during the recovery phase. Non-neurologic organ dysfunction can manifest following mild TBI or severe TBI. The pathophysiology of systemic manifestations following TBI is multifactorial and involves an effect on the autonomic nervous system, involvement of the hypothalamic-pituitary axis, release of inflammatory mediators, and treatment modalities used for TBI. Endocrine dysfunction, electrolyte imbalance, and respiratory manifestations are common following TBI. The influence of TBI on systemic immune response, coagulation cascade, cardiovascular system, gastrointestinal system, and other systems is becoming more evident through animal studies and clinical trials. Systemic manifestations can independently act as risk factors for mortality and morbidity following TBI. Some conditions like neurogenic pulmonary edema and disseminated intravascular coagulation can adversely affect the outcome. Early recognition and treatment of systemic manifestations may improve the clinical outcome following TBI. Further studies are required especially in the field of neuroimmunology to establish the role of various biochemical cascades, not only in the pathophysiology of TBI but also in its systemic manifestations and outcome.

  7. Correlation of serum IgE levels and clinical manifestations in patients with actinic prurigo*

    PubMed Central

    Cuevas-Gonzalez, Juan Carlos; Lievanos-Estrada, Zahide; Vega-Memije, Maria Elisa; Hojyo-Tomoka, Maria Teresa; Dominguez-Soto, Luciano

    2016-01-01

    BACKGROUND: Actinic prurigo is an idiopathic photodermatosis, the pathophysiology of which has been hypothesized to involve subtype IV type b (Th2) hypersensitive response, whereby IL4, IL5, and IL13 are secreted and mediate the production of B cells, IgE, and IgG4. OBJECTIVES: To examine the association of serum IgE levels and the clinical severity of injuries. METHODS: This case-control study comprised patients with a clinical and histopathological diagnosis of actinic prurigo, as well as clinically healthy subjects, from whom 3cc of peripheral blood was taken for immunoassay. Cases were classified by lesion severity as mild, moderate, and severe. Descriptive statistics were analyzed, and chi-square test was performed. RESULTS: We included 21 actinic prurigo patients and 21 subjects without disease; 11 patients with actinic prurigo had elevated serum IgE levels, and 10 had low serum levels. Six actinic prurigo (AP) patients with elevated serum levels of IgE had moderate injuries, 4 had severe injuries, and 1 had minor injuries. Eight out of 10 patients with normal IgE levels presented with minor injuries in the clinical evaluation. The 21 controls did not have increased serum IgE levels. CONCLUSIONS: Elevated IgE levels are associated with moderate to severe clinical lesions, suggesting that actinic prurigo entails a type IV subtype b hypersensitivity response in which Th2 cells predominate. PMID:26982774

  8. Herpes Simplex Virus Infection in a University Health Population: Clinical Manifestations, Epidemiology, and Implications

    ERIC Educational Resources Information Center

    Horowitz, Robert; Aierstuck, Sara; Williams, Elizabeth A.; Melby, Bernette

    2010-01-01

    Objective: The authors described clinical presentations of oral and genital herpes simplex virus (HSV) infections in a university health population and implications of these findings. Participants and Methods: Using a standardized data collection tool, 215 records of patients with symptomatic culture-positive HSV infections were reviewed. Results:…

  9. Clinical Manifestations in Paroxysmal Kinesigenic Dyskinesia Patients with Proline-Rich Transmembrane Protein 2 Gene Mutation

    PubMed Central

    Youn, Jinyoung; Kim, Ji Sun; Lee, Munhyang; Lee, Jeehun; Roh, Hakjae

    2014-01-01

    Background and Purpose Given the diverse phenotypes including combined non-dyskinetic symptoms in patients harboring mutations of the gene encoding proline-rich transmembrane protein 2 (PRRT2), the clinical significance of these mutations in paroxysmal kinesigenic dyskinesia (PKD) is questionable. In this study, we investigated the clinical characteristics of PKD patients with PRRT2 mutations. Methods Familial and sporadic PKD patients were enrolled and PRRT2 gene sequencing was performed. Demographic and clinical data were compared between PKD patients with and without a PRRT2 mutation. Results Among the enrolled PKD patients (8 patients from 5 PKD families and 19 sporadic patients), PRRT2 mutations were detected in 3 PKD families (60%) and 2 sporadic cases (10.5%). All familial patients with a PRRT2 gene mutation had the c.649dupC mutation, which is the most commonly reported mutation. Two uncommon mutations (c.649delC and c.629dupC) were detected only in the sporadic cases. PKD patients with PRRT2 mutation were younger at symptom onset and had more non-dyskinetic symptoms than those without PRRT2 mutation. However, the characteristics of dyskinetic movement did not differ between the two groups. Conclusions This is the first study of PRRT2 mutations in Korea. The presence of a PRRT2 mutation was more strongly related to familial PKD, and was clinically related with earlier age of onset and common non-dyskinetic symptoms in PKD patients. PMID:24465263

  10. Thrombocytopenia in Systemic Lupus Erythematosus: Clinical Manifestations, Treatment, and Prognosis in 230 Patients.

    PubMed

    Jung, Jin-Hee; Soh, Moon-Seung; Ahn, Young-Hwan; Um, Yoo-Jin; Jung, Ju-Yang; Suh, Chang-Hee; Kim, Hyoun-Ah

    2016-02-01

    The aim of the study was to examine the clinical characteristics and prognosis according to severity of thrombocytopenia and response to treatment for thrombocytopenia in patients with systemic lupus erythematosus (SLE).We retrospectively evaluated 230 SLE patients with thrombocytopenia, and reviewed their clinical data and laboratory findings. Thrombocytopenia was defined as platelet counts under 100,000/mm, and patients were divided into 3 thrombocytopenia groups according to severity: mild (platelet counts >50,000/mm), moderate (>20,000/mm, ≤50,000/mm), and severe (≤20,000/mm). Clinical characteristics, treatments, and prognoses were compared among the groups. Furthermore, complete remission of thrombocytopenia was defined as platelet counts >100,000/mm after treatment.There was no significant difference in clinical or laboratory findings among the groups according to severity of thrombocytopenia. However, hemorrhagic complications were more frequent in severe thrombocytopenia (P < 0.001) and mortality was also higher (P = 0.001). Complete remission was achieved in 85.2% of patients. The clinical characteristics and modality of treatment did not differ between the patients with and without complete remission. Mortality in patients with complete remission (1.5%) was significantly lower than in those without complete remission (29.4%, P < 0.001). Survival was significantly higher in patients with complete remission from thrombocytopenia (odds ratio = 0.049, 95% confidence interval: 0.013-0.191, P < 0.001).The severity of thrombocytopenia in SLE patients can be a useful independent prognostic factor to predict survival. Moreover, complete remission of thrombocytopenia after treatment is an important prognostic factor. The severity of thrombocytopenia and response to treatment should be closely monitored to predict prognosis in SLE patients.

  11. Spontaneous murine lupus-like syndromes. Clinical and immunopathological manifestations in several strains.

    PubMed

    Andrews, B S; Eisenberg, R A; Theofilopoulos, A N; Izui, S; Wilson, C B; McConahey, P J; Murphy, E D; Roths, J B; Dixon, F J

    1978-11-01

    MRL/1 and BXSB male mice have a systemic lupus erythematosus (SLE)-like disease similar to but more acute than that occurring in NZB X W mice. The common elements of lymphoid hyperplasia, B-cell hyperactivity, autoantibodies, circulating immune complex (IC), complement consumption, IC glomerulonephritis with gp70 deposition, and thymic atrophy were found in all three kinds of SLE mice. On the basis of these common elements, SLE seen in these mice can be considered a single disease in the same sense that human SLE is one disease. The differences in the SLE expressed in the different mice are no greater than those found in an unselected series of humans with SLE. However, the significant quantitative and qualitative variations in abnormal immunologic expression suggest that different constellations of factors, genetic and/or pathophysiologic, may operate in the three murine strains and that each constellation is capable of leading, via its particular abnormal immunologic consequences, to the activation of common immunopathologic effector mechanisms that cause quite similar SLE-like syndromes. From an experimental point of view, the availability of several inbred murine strains of commonplace histocompatibility types that express an SLE-like syndrome makes possible innumerable manipulations which should help to elucidate the nature and cause(s) of this disorder.

  12. Comparison of neurological clinical manifestation in patients with hemorrhagic and ischemic stroke

    PubMed Central

    Ojaghihaghighi, Seyedhossein; Vahdati, Samad Shams; Mikaeilpour, Akram; Ramouz, Ali

    2017-01-01

    BACKGROUND: Cerebrovascular accident (CVA) is the third leading cause of death and disability in developed countries. Anyone suspected of having a stroke should be taken immediately to a medical facility for diagnosis and treatment. The symptoms that follow a stroke aren’t significant and depend on the area of the brain that has been affected and the amount of tissue damaged. Parameters for predicting long-term outcome in such patients have not been clearly delineated, therefore the aim of this study was to investigate this possibility and to test a system that might practicably be used routinely to aid management and predict outcomes of individual stroke patients. METHODS: A descriptive hospital-based study of the neurological symptoms and signs of 503 patients with ischemic stroke, including severe headache, seizure, eye movement disorder, pupil size, Glasgow Coma Scale (GCS), agitation were analyzed in this study. RESULTS: In the current study, dilated pupils, agitation, acute onset headache, lower GCS score, seizure, and eye gaze impairment had significantly higher prevalence in hemorrhagic stroke patients (P<0.001). However, the rate of gradual progressive headache is significantly higher in ischemic stroke patients (P<0.001). CONCLUSION: Although this result provides reliable indicators for discrimination of stroke types, imaging studies are still the gold standard modality for diagnosis. PMID:28123618

  13. Pulmonary thromboembolic disease. Clinical management of acute and chronic disease.

    PubMed

    Torbicki, Adam

    2010-07-01

    Pulmonary thromboembolism falls between the areas of pulmonology and cardiology, internal medicine and intensive care, radiology and nuclear medicine, and hematology and cardiothoracic surgery. Depending on their clinical background, physicians faced with a patient with a pulmonary thromboembolism may speak different languages and adopt different treatment approaches. Now, however, there is an opportunity to end the Tower of Babel surrounding pulmonary thromboembolism. There is a growing acknowledgement that the key clinical problems in both acute pulmonary embolism and chronic thromboembolic pulmonary hypertension are linked to right ventricular pressure overload and right ventricular failure. As a result, cardiologists and cardiac intensive care specialists are taking an increasing interest in understanding and combating these conditions. The European Society of Cardiology was the first to elaborate comprehensive clinical practice guidelines for pulmonary thromboembolism and chronic thromboembolic pulmonary hypertension. The task forces involved in producing these guidelines included radiologists, pulmonologists, hematologists, intensive care physicians and surgeons, which ensured that the final document was universally acceptable. The aim of this article was to provide an overview of the epidemiology, risk factors, diagnosis, treatment, prognosis and prevention of acute pulmonary thromboembolism and chronic thromboembolic pulmonary hypertension, while taking into account European Society of Cardiology guidelines and incorporating new evidence where necessary.

  14. Clinical and allergological analysis of ocular manifestations of sick building syndrome

    PubMed Central

    Saeki, Yusuke; Kadonosono, Kazuaki; Uchio, Eiichi

    2017-01-01

    Purpose The disease concept of sick building syndrome (SBS) is still unclear. Ocular mucous membrane irritation is one of the major symptoms of SBS. However, the immunological aspects of the ocular complications of SBS are not yet clarified. The clinical and allergological aspects of SBS cases with ocular disorders with special reference to allergic conjunctival diseases (ACD) were analyzed, especially with respect to local immunological features. Methods Twelve cases of SBS with ocular findings and 49 cases of ACD (allergic conjunctivitis [AC], atopic keratoconjunctivitis [AKC], and vernal keratoconjunctivitis [VKC]) for comparison were evaluated. The clinical findings in SBS and ACD were scored, and tear film breakup time (BUT) was measured. Cytokine (interferon-γ [IFN-γ], interleukin [IL]-2, IL-4, IL-5, IL-6, IL-8, and IL-13) concentrations in tears were analyzed by cytometric bead arrays. Eosinophil count in peripheral blood, total IgE in serum, and multiple allergen simultaneous test (MAST) for antigen-specific IgE were also measured. Results In SBS, conjunctival lesions were observed in all cases, and corneal abnormalities were found in two-thirds of the cases. Limbal lesions were observed in 2 pediatric cases. Mean serum total IgE level in SBS was significantly higher than that in AC; however, it was significantly lower than that in AKC and VKC. Eosinophil count in peripheral blood and number of positive allergens in MAST were significantly lower in SBS than in AKC and VKC. Significant elevation of tear IL-4 was observed in SBS and ACD. However, in contrast to ACD, elevation of other cytokines in tears was not observed in SBS. Mean tear BUT in SBS was in the normal range. Conclusion From these results, SBS is thought to be partially induced by an allergic response. However, clinical dissociation of the ocular clinical findings and local immunological features in tear cytokines may suggest that SBS belongs to a different entity from ACD. PMID:28352150

  15. Desmoid tumors: clinical features and outcome of an unpredictable and challenging manifestation of familial adenomatous polyposis.

    PubMed

    Campos, Fábio Guilherme; Martinez, Carlos Augusto Real; Novaes, Marleny; Nahas, Sérgio Carlos; Cecconello, Ivan

    2015-06-01

    Background/Aims Desmoid tumors (DTs) are rare, locally invasive neoplasms that may affect 10-25% of familial adenomatous polyposis (FAP) patients. Our aim was to evaluate incidence and clinical presentation among our patients, the potential impact on FAP outcome and to discuss treatment. Materials and methods Charts from 133 FAP (1977-2013) were reviewed. Patients with DTs were separated to retrieve demographic, clinical and management data. Follow-up was focused on disease evolution causing complications or death. Results 19 (14.3%) DTs were diagnosed, either after previous trauma (16) or during FAP surgery (3). This group comprised 8 men (42.1%) and 11 women (57.9%) with an average age of 32.9 years. Intervals from surgical trauma to DTs ranged from 7 to 60 months. ECMs were detected in 12 (63.1%) patients. DTs were located in the abdominal wall (8), abdominal cavity (8), abdominal wall and cavity (2) and left arm (1). Five patients (26.3%) referred family history of DTs. Patients presented severe complications such as small bowel obstruction (4) and hydronephrosis (2), being directly responsible for death in three patients. Conclusions (1) DTs developed in 14.3% of FAP, mostly after surgical trauma; (2) 30% caused severe morbidity; (3) identification of clinical risk factors may help surgeons to develop screening and therapeutic decisions.

  16. Diabetes and cardiac autonomic neuropathy: Clinical manifestations, cardiovascular consequences, diagnosis and treatment

    PubMed Central

    Balcıoğlu, Akif Serhat; Müderrisoğlu, Haldun

    2015-01-01

    Cardiac autonomic neuropathy (CAN) is a frequent chronic complication of diabetes mellitus with potentially life-threatening outcomes. CAN is caused by the impairment of the autonomic nerve fibers regulating heart rate, cardiac output, myocardial contractility, cardiac electrophysiology and blood vessel constriction and dilatation. It causes a wide range of cardiac disorders, including resting tachycardia, arrhythmias, intraoperative cardiovascular instability, asymptomatic myocardial ischemia and infarction and increased rate of mortality after myocardial infarction. Etiological factors associated with autonomic neuropathy include insufficient glycemic control, a longer period since the onset of diabetes, increased age, female sex and greater body mass index. The most commonly used methods for the diagnosis of CAN are based upon the assessment of heart rate variability (the physiological variation in the time interval between heartbeats), as it is one of the first findings in both clinically asymptomatic and symptomatic patients. Clinical symptoms associated with CAN generally occur late in the disease process and include early fatigue and exhaustion during exercise, orthostatic hypotension, dizziness, presyncope and syncope. Treatment is based on early diagnosis, life style changes, optimization of glycemic control and management of cardiovascular risk factors. Medical therapies, including aldose reductase inhibitors, angiotensin-converting enzyme inhibitors, prostoglandin analogs and alpha-lipoic acid, have been found to be effective in randomized controlled trials. The following article includes the epidemiology, clinical findings and cardiovascular consequences, diagnosis, and approaches to prevention and treatment of CAN. PMID:25685280

  17. Clinical Evaluation of Specific Oral Manifestations in Pediatric Patients with Ascertained versus Potential Coeliac Disease: A Cross-Sectional Study

    PubMed Central

    Matacena, Giada; Costa, Stefano; Magazzù, Giuseppe

    2014-01-01

    Patients involved on coeliac disease (CD) have atypical symptoms and often remain undiagnosed. Specific oral manifestations are effective risk indicators of CD and for this reason an early diagnosis with a consequent better prognosis can be performed by the dentist. There are not researches analysing the frequency of these oral manifestations in potential coeliac patients. The aim of this study is to investigate the oral hard and soft tissue lesions in potential and ascertained coeliac children in comparison with healthy controls. 50 ascertained children, 21 potential coeliac patients, and 54 controls were recruited and the oral examination was performed. The overall oral lesions were more frequently present in CD patients than in controls. The prevalence of oral soft tissue lesions was 62% in ascertained coeliac, 76.2% in potential coeliac patients, and 12.96% in controls (P < 0.05). Clinical dental delayed eruption was observed in 38% of the ascertained coeliac and 42.5% of the potential coeliac versus 11.11% of the controls (P < 0.05). The prevalence of specific enamel defects (SED) was 48% in ascertained coeliac and 19% in potential coeliac versus 0% in controls (P < 0.05; OR = 3.923). The SED seem to be genetically related to the histological damage and villous atrophy. PMID:25197270

  18. [Piriformis muscle syndrome: etiology, pathogenesis, clinical manifestations, diagnosis, differential diagnosis and therapy].

    PubMed

    Grgić, Vjekoslav

    2013-01-01

    The term 'piriformis syndrome' (PS), introduced by Robinson in 1947, implies a group of signs and symptoms caused by piriformis muscle (PM) disorders. Since PM disorders lead to irritation/compression of the anatomic structures passing under its belly, the main clinical PS signs and symptoms are actually the clinical signs and symptoms of irritation/ compression of neural and vascular structures passing through the infrapiriform foramen: sciatic nerve/SN, inferior gluteal nerve, posterior femoral cutaneous nerve, pudendal nerve, inferior gluteal artery and vein and inferior pudendal artery and vein. The clinical picture is usually dominated by signs and symptoms of irritation/compression of SN (SN irritation --> low back and buttock pain, sciatica,paresthesias in distribution of SN; SN compression --> low back and buttock pain,sciatica, paresthesias and neurologic deficit in distribution of SN). Irritation/compression of other structures can result in the following signs and symptoms: inferior gluteal nerve --> atrophy of gluteal muscles; posterior femoral cutaneous nerve --> pain, paresthesias and sensory disturbances in the posterior thigh; pudendal nerve --> pudendal neuralgia, painful sexual intercourse (dyspareunia), sexual dysfunction, urination and defecation problems; inferior gluteal artery --> ischemic buttock pain; inferior pudendal artery --> ischemic pain in the area of external sex organs, perineum and rectum, sexual dysfunction, urination and defecation problems; inferior gluteal vein --> venous stasis in gluteal area; inferior pudendal vein --> venous stasis in external sex organs and rectum. Functional/non-organic and organic PM disorders can cause PS: spasm, shortening, hypertrophy, anatomic variations, edema, fibrosis, adhesions, hematoma, atrophy, cyst, bursitis, abscess, myositis ossificans, endometriosis, tumors (functional disorders: PM spasm and shortening). The most common causes for PS are PM spasm, shortening and hypertrophy and anatomic

  19. Network analysis reveals distinct clinical syndromes underlying acute mountain sickness.

    PubMed

    Hall, David P; MacCormick, Ian J C; Phythian-Adams, Alex T; Rzechorzek, Nina M; Hope-Jones, David; Cosens, Sorrel; Jackson, Stewart; Bates, Matthew G D; Collier, David J; Hume, David A; Freeman, Thomas; Thompson, A A Roger; Baillie, John Kenneth

    2014-01-01

    Acute mountain sickness (AMS) is a common problem among visitors at high altitude, and may progress to life-threatening pulmonary and cerebral oedema in a minority of cases. International consensus defines AMS as a constellation of subjective, non-specific symptoms. Specifically, headache, sleep disturbance, fatigue and dizziness are given equal diagnostic weighting. Different pathophysiological mechanisms are now thought to underlie headache and sleep disturbance during acute exposure to high altitude. Hence, these symptoms may not belong together as a single syndrome. Using a novel visual analogue scale (VAS), we sought to undertake a systematic exploration of the symptomatology of AMS using an unbiased, data-driven approach originally designed for analysis of gene expression. Symptom scores were collected from 292 subjects during 1110 subject-days at altitudes between 3650 m and 5200 m on Apex expeditions to Bolivia and Kilimanjaro. Three distinct patterns of symptoms were consistently identified. Although fatigue is a ubiquitous finding, sleep disturbance and headache are each commonly reported without the other. The commonest pattern of symptoms was sleep disturbance and fatigue, with little or no headache. In subjects reporting severe headache, 40% did not report sleep disturbance. Sleep disturbance correlates poorly with other symptoms of AMS (Mean Spearman correlation 0.25). These results challenge the accepted paradigm that AMS is a single disease process and describe at least two distinct syndromes following acute ascent to high altitude. This approach to analysing symptom patterns has potential utility in other clinical syndromes.

  20. Network Analysis Reveals Distinct Clinical Syndromes Underlying Acute Mountain Sickness

    PubMed Central

    Hall, David P.; MacCormick, Ian J. C.; Phythian-Adams, Alex T.; Rzechorzek, Nina M.; Hope-Jones, David; Cosens, Sorrel; Jackson, Stewart; Bates, Matthew G. D.; Collier, David J.; Hume, David A.; Freeman, Thomas; Thompson, A. A. Roger; Baillie, John Kenneth

    2014-01-01

    Acute mountain sickness (AMS) is a common problem among visitors at high altitude, and may progress to life-threatening pulmonary and cerebral oedema in a minority of cases. International consensus defines AMS as a constellation of subjective, non-specific symptoms. Specifically, headache, sleep disturbance, fatigue and dizziness are given equal diagnostic weighting. Different pathophysiological mechanisms are now thought to underlie headache and sleep disturbance during acute exposure to high altitude. Hence, these symptoms may not belong together as a single syndrome. Using a novel visual analogue scale (VAS), we sought to undertake a systematic exploration of the symptomatology of AMS using an unbiased, data-driven approach originally designed for analysis of gene expression. Symptom scores were collected from 292 subjects during 1110 subject-days at altitudes between 3650 m and 5200 m on Apex expeditions to Bolivia and Kilimanjaro. Three distinct patterns of symptoms were consistently identified. Although fatigue is a ubiquitous finding, sleep disturbance and headache are each commonly reported without the other. The commonest pattern of symptoms was sleep disturbance and fatigue, with little or no headache. In subjects reporting severe headache, 40% did not report sleep disturbance. Sleep disturbance correlates poorly with other symptoms of AMS (Mean Spearman correlation 0.25). These results challenge the accepted paradigm that AMS is a single disease process and describe at least two distinct syndromes following acute ascent to high altitude. This approach to analysing symptom patterns has potential utility in other clinical syndromes. PMID:24465370

  1. Diffuse alveolar hemorrhage in patients with systemic lupus erythematosus. Clinical manifestations, treatment, and prognosis.

    PubMed

    Martínez-Martínez, Marco Ulises; Abud-Mendoza, Carlos

    2014-01-01

    Diffuse alveolar hemorrhage (DAH) in patients with systemic lupus erythematosus is a rare but potentially fatal condition. Although the pathogenesis of this condition is unknown, high disease activity is the main characteristic; moreover, histopathology in some studies showed alveolar immune complex deposits and capillaritis. Clinical features of DAH include dyspnea, a drop in hemoglobin, and diffuse radiographic alveolar images, with or without hemoptysis. Factors associated with mortality include mechanical ventilation, renal failure, and infections. Bacterial infections have been reported frequently in patients with DAH, but also invasive fungal infections including aspergillosis. DAH treatment is based on high dose methylprednisolone; other accepted therapies include cyclophosphamide (controversial), plasmapheresis, immunoglobulin and rituximab.

  2. [Andersen-Tawil syndrome: a review of its clinical and genetic diagnosis with emphasis on cardiac manifestations].

    PubMed

    Márquez, Manlio F; Totomoch-Serra, Armando; Vargas-Alarcón, Gilberto; Cruz-Robles, David; Pellizzon, Oscar A; Cárdenas, Manuel

    2014-01-01

    The Andersen-Tawil syndrome is a cardiac ion channel disease that is inherited in an autosomal dominant way and is classified as type 7 of the congenital long QT syndromes. Affected gene is KCNJ2, which forms the inward rectifier potassium channel designated Kir2.1. This protein is involved in stabilizing the resting membrane potential and controls the duration of the action potential in skeletal muscle and heart. It also participates in the terminal repolarization phase of the action potential in ventricular myocytes and is a major component responsible for the correction in the potassium current during phase 3 of the action potential repolarization. Kir 2.1 channel has a predominant role in skeletal muscle, heart and brain. Alterations in this channel produce flaccid paralysis, arrhythmias, impaired skeletal development primarily in extremities and facial area. In this review we address the disease from the point of view of clinical and molecular diagnosis with emphasis on cardiac manifestations.

  3. [The clinical manifestations and diagnostics of otitis media caused by tuberculosis].

    PubMed

    Kriukov, A I; Garov, E V; Ivoĭlov, A Y U; Shadrin, G B; Sidorina, N G; Lavrova, A S

    2015-01-01

    The objective of the present study was to clarify the characteristic pathognomonic features of middle ear lesions associated with tuberculosis and the approaches to their diagnostics under the present-day conditions. The study included 11 cases (18 ears) of tuberculosis otitis media and the related lesions of the mastoid process diagnosed with the use of clinical, roentgenological, cytological, bacteriological, pathomorphological, and molecular-genetic methods (including PCR diagnostics). The primary localization of tuberculosis in the middle ear was documented in 6 patients; in 5 patients, it was associated with pulmonary involvement. Five patients presented with smoldering exudative otitis media and the remaining six ones with suppurative perforating otitis media. The tuberculous process was diagnosed with the use of various methods including clinical examination, bacteriological (9%), cytological (27.3%), pathomorphological (18%) studies, and PCR diagnostics (55%). Diagnosis was made within a period from 1 month to 1.5 years after the application of the patients for medical assistance which suggests the difficulty of verification of tuberculous etiology of the disease of the middle ear. It is concluded that the high index of suspicion in the case of smoldering middle ear pathology facilitates its early diagnostics and successful treatment.

  4. The autoantibody profile and its association with clinical manifestations in Malay SLE patients.

    PubMed

    Maraina, C H Che; Kamaliah, M D; Ishak, M

    2004-03-01

    A cross sectional study was conducted to determine the auto-antibody profile of Malay SLE patients in Kelantan, North East Malaysia and to correlate them with clinical presentations. Eighty-two Malay SLE patients who fulfilled the ARA criteria underwent the following tests: ANA, anti-dsDNA antibody, anti-ENA antibody and RF. The results revealed that ANA was positive in 91.5% of the patients, anti-dsDNA antibody in 53.7%, however, anti-ENA antibodies were positive in only 9.8% of the cases at the time of the study and none had a positive RF. The profile of autoantibodies was similar to other studies except for a lower incidence of anti-ENA antibodies. Sixty three percent of patients had lupus nephritis. The pattern of clinical presentations were noted to be more similar to those found among Chinese and Indian SLE populations than compared to the Caucasians. There was a significant association between anti-dsDNA antibody and lupus nephritis and between anti-ENA antibody and thrombocytopenia.

  5. An enterovirus 71 epidemic in Guangdong Province of China, 2008: epidemiological, clinical, and virogenic manifestations.

    PubMed

    Sun, Li-mei; Zheng, Huan-ying; Zheng, Hui-zhen; Guo, Xue; He, Jian-feng; Guan, Da-wei; Kang, Min; Liu, Zheng; Ke, Chang-wen; Li, Jian-sen; Liu, Leng; Guo, Ru-ning; Yoshida, Hiromu; Lin, Jin-yan

    2011-01-01

    Enterovirus 71 (EV71) is shown to be a major causative agent in outbreaks of hand, foot, and mouth disease (HFMD) reported in Guangdong (GD) Province of China in 2008. A total of 48,876 HFMD cases (131 severe and 21 fatal) were reported to the GD HFMD web-based surveillance system, which covers 871 clinics. The main causes of death included central nervous system damage, heart failure, and pulmonary edema. The incidence rate was 52 per 100,000, and the epidemic peak appeared in May and June. EV71 was found in 59% and coxsackievirus A16 in 26% of 936 laboratory-confirmed cases. Other viruses are likely to be responsible for the remaining 15% of cases. Of the 185 EV71 cases collected, 62% were mild, 27% were severe, and the remaining 11% were fatal. A total of 17 EV71 isolates were subjected to nucleotide sequencing of the entire VP1 gene. Phylogenetic analysis showed that the GD EV71 strains belonged to the C4 subgenotype and that EV71 circulates at a national rather than a regional level. A Comparison with the VP1 gene from a different clinical case showed that there was no obvious virulence determinant in this locus. Furthermore, this study found that most deaths occurred in rural areas, thereby indicating that delayed diagnosis and incorrect treatment may play an important role.

  6. Advanced primary pulmonary lymphoepithelioma-like carcinoma: clinical manifestations, treatment, and outcome

    PubMed Central

    Lin, Chun-Yu; Chen, Ying-Jen; Hsieh, Meng-Heng; Wang, Chih-Wei

    2017-01-01

    Background Primary pulmonary lymphoepithelioma-like carcinoma (LELC) is rare, with better clinical outcomes than other lung cancers. However, reports on advanced LELC characteristics and prognosis are lacking. Methods This retrospective study included adults diagnosed with advanced LELC (at least stage IIIA) between January 2003 and December 2015. Clinical characteristics, treatment modalities, and outcomes were recorded. Results Study population comprised 23 patients with a mean age of 63.7±10.6 years. The Eastern Cooperative Oncology Group status on diagnosis was 0 in five patients and 1 in the others. Most patients received multimodality treatment and all received cisplatin-based chemotherapy. Median follow-up duration was 28.8 months. The median progression free survival (PFS) was 14.6 months in patients received palliative chemotherapy. There were nine (39.1%) deaths. The median overall survival (OS) was not achieved. Until July 31, 2016, median OS was 54.1 months for stage IIIB and 27.6 months for stage IV. There was no significant difference in OS among all stages. No prognostic factors were found. Conclusions Advanced LELC responded well to cisplatin-based chemotherapy and/or radiotherapy. Main tumor resection is probably beneficial for advanced LELC. Long-term survival is possible for advanced LELC after multimodality treatment. PMID:28203414

  7. Osteogenesis imperfecta type V: clinical and radiographic manifestations in mutation confirmed patients.

    PubMed

    Kim, Ok-Hwa; Jin, Dong-Kyu; Kosaki, Keisuke; Kim, Jung-Wook; Cho, Sung Yoon; Yoo, Won Joon; Choi, In Ho; Nishimura, Gen; Ikegawa, Shiro; Cho, Tae-Joon

    2013-08-01

    Osteogenesis imperfecta (OI) type V is a specific OI phenotype with interosseous membrane calcification of the forearm and hyperplastic callus formation as typical features. The causative gene mutation for OI type V has been recently discovered. The purpose of this report is to review the clinical and radiographic characteristics of mutation confirmed OI type V in detail. Sixteen (nine familial and seven sporadic) patients were enrolled in the study. Blue sclera and dentinogenesis imperfecta were not evident in any patient. However, hypodontia in the permanent teeth, ectopic eruption, and short roots in molars were additionally observed in 11 patients. Of the radiographic abnormalities, cortical thickening and bony excrescence of interosseous margin of the ulna was the most common finding, followed by overgrowth of the olecranon and/or coronoid process of the ulna. Slender ribs and sloping of the posterior ribs with or without fractures were also a consistent finding. Hyperplastic callus was detected in 75% of patients and was commonly encountered at the femur. Heterotopic ossification in the muscles and tendon insertion sites were noted in four patients, which resulted in bony ankylosis or contracture of joints. The current study confirms common clinical and radiographic findings of OI type V and reports additional phenotypic information. These observations provide clues to recognize OI type V more promptly and guide to direct targeted molecular study. © 2013 Wiley Periodicals, Inc.

  8. Altered E-Cadherin Levels and Distribution in Melanocytes Precede Clinical Manifestations of Vitiligo.

    PubMed

    Wagner, Roselyne Y; Luciani, Flavie; Cario-André, Muriel; Rubod, Alain; Petit, Valérie; Benzekri, Laila; Ezzedine, Khaled; Lepreux, Sébastien; Steingrimsson, Eirikur; Taieb, A; Gauthier, Yvon; Larue, Lionel; Delmas, Véronique

    2015-07-01

    Vitiligo is the most common depigmenting disorder resulting from the loss of melanocytes from the basal epidermal layer. The pathogenesis of the disease is likely multifactorial and involves autoimmune causes, as well as oxidative and mechanical stress. It is important to identify early events in vitiligo to clarify pathogenesis, improve diagnosis, and inform therapy. Here, we show that E-cadherin (Ecad), which mediates the adhesion between melanocytes and keratinocytes in the epidermis, is absent from or discontinuously distributed across melanocyte membranes of vitiligo patients long before clinical lesions appear. This abnormality is associated with the detachment of the melanocytes from the basal to the suprabasal layers in the epidermis. Using human epidermal reconstructed skin and mouse models with normal or defective Ecad expression in melanocytes, we demonstrated that Ecad is required for melanocyte adhesiveness to the basal layer under oxidative and mechanical stress, establishing a link between silent/preclinical, cell-autonomous defects in vitiligo melanocytes and known environmental stressors accelerating disease expression. Our results implicate a primary predisposing skin defect affecting melanocyte adhesiveness that, under stress conditions, leads to disappearance of melanocytes and clinical vitiligo. Melanocyte adhesiveness is thus a potential target for therapy aiming at disease stabilization.

  9. Ringo: discordance between the molecular and clinical manifestation in a golden retriever muscular dystrophy dog.

    PubMed

    Zucconi, Eder; Valadares, Marcos Costa; Vieira, Natássia M; Bueno, Carlos R; Secco, Mariane; Jazedje, Tatiana; da Silva, Helga Cristina Almeida; Vainzof, Mariz; Zatz, Mayana

    2010-01-01

    Of the various genetic homologues to Duchenne Muscular Dystrophy (DMD), the Golden Retriever Muscular Dystrophy (GRMD) dog, which presents a variable but usually severe and progressive muscle weakness, has the closest relevance to DMD in both clinical severity and histopathological change. Among 77 GRMD dogs born in our colony in Brazil, we have identified a very mildly affected dog, Ringo, born July 2003. Among his descendants, at least one male, Suflair, is also showing a mild course. In an attempt to better characterize these two dogs, we studied the pattern of muscle proteins expression in Ringo and Suflair, as compared to severely affected and normal control dogs. Dystrophin was absent in both and utrophin was overexpressed in a pattern similar to the observed in severely affected dogs. Understanding the mechanism that is protecting Ringo and Suflair from the deleterious effect of the dystrophin gene mutation is of utmost interest. In addition it points out that the clinical impact of therapeutic trials should be interpreted with caution.

  10. The relevance of "non-criteria" clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features.

    PubMed

    Abreu, Mirhelen M; Danowski, Adriana; Wahl, Denis G; Amigo, Mary-Carmen; Tektonidou, Maria; Pacheco, Marcelo S; Fleming, Norma; Domingues, Vinicius; Sciascia, Savino; Lyra, Julia O; Petri, Michelle; Khamashta, Munther; Levy, Roger A

    2015-05-01

    The purpose of this task force was to critically analyze nine non-criteria manifestations of APS to support their inclusion as APS classification criteria. The Task Force Members selected the non-criteria clinical manifestations according to their clinical relevance, that is, the patient-important outcome from clinician perspective. They included superficial vein thrombosis, thrombocytopenia, renal microangiopathy, heart valve disease, livedo reticularis, migraine, chorea, seizures and myelitis, which were reviewed by this International Task Force collaboration, in addition to the seronegative APS (SN-APS). GRADE system was used to evaluate the quality of evidence of medical literature of each selected item. This critical appraisal exercise aimed to support the debate regarding the clinical picture of APS. We found that the overall GRADE analysis was very low for migraine and seizures, low for superficial venous thrombosis, thrombocytopenia, chorea, longitudinal myelitis and the so-called seronegative APS and moderate for APS nephropathy, heart valve lesions and livedo reticularis. The next step can be a critical redefinition of an APS gold standard, for instance derived from the APS ACTION registry that will include not only current APS patients but also those with antiphospholipid antibodies not meeting current classification criteria.

  11. Hypofibrinolytic State in Subjects with Type 2 Diabetes Mellitus Aggravated by the Metabolic Syndrome before Clinical Manifestations of Atherothrombotic Disease.

    PubMed

    Aburto-Mejía, Elsa; Santiago-Germán, David; Martínez-Marino, Manuel; María Eugenia Galván-Plata; Almeida-Gutiérrez, Eduardo; López-Alarcón, Mardia; Hernández-Juárez, Jesús; Alvarado-Moreno, Antonio; Leaños-Miranda, Alfredo; Majluf-Cruz, Abraham; Isordia-Salas, Irma

    2017-01-01

    Background. Metabolic and genetic factors induce plasminogen activator inhibitor type-1 (PAI-1) overexpression; higher PAI-1 levels decrease fibrinolysis and promote atherothrombosis. Aim. To assess PAI-1 antigen levels among subjects with type 2 diabetes mellitus (T2DM) plus Metabolic Syndrome (MetS) before clinical manifestations of atherothrombosis and the contribution of metabolic factors and 4G/5G polymorphism of PAI-1 gene on the variability of PAI-1. Methods. We conducted an observational, cross-sectional assay in a hospital in Mexico City from May 2010 to September 2011. MetS was defined by the International Diabetes Federation criteria. PAI-1 levels and 4G/5G polymorphism were determined by ELISA and PCR-RFLP analysis. Results. We enrolled 215 subjects with T2DM plus MetS and 307 controls. Subjects with T2DM plus MetS had higher PAI-1 levels than the reference group (58.4 ± 21 versus 49.9 ± 16 ng/mL, p = 0.026). A model with components of MetS explained only 12% of variability on PAI-1 levels (R(2) = 0.12; p = 0.001), with β = 0.18 (p = 0.03) for hypertension, β = -0.16 (p = 0.05) for NL HDL-c, and β = 0.15 (p = 0.05) for NL triglycerides. Conclusion. Subjects with T2DM plus MetS have elevated PAI-1 levels before clinical manifestations of atherothrombotic disease. Metabolic factors have a more important contribution than 4G/5G polymorphism on PAI-1 plasma variability.

  12. Hypofibrinolytic State in Subjects with Type 2 Diabetes Mellitus Aggravated by the Metabolic Syndrome before Clinical Manifestations of Atherothrombotic Disease

    PubMed Central

    Aburto-Mejía, Elsa; Santiago-Germán, David; Martínez-Marino, Manuel; María Eugenia Galván-Plata; Almeida-Gutiérrez, Eduardo; Hernández-Juárez, Jesús; Alvarado-Moreno, Antonio; Leaños-Miranda, Alfredo

    2017-01-01

    Background. Metabolic and genetic factors induce plasminogen activator inhibitor type-1 (PAI-1) overexpression; higher PAI-1 levels decrease fibrinolysis and promote atherothrombosis. Aim. To assess PAI-1 antigen levels among subjects with type 2 diabetes mellitus (T2DM) plus Metabolic Syndrome (MetS) before clinical manifestations of atherothrombosis and the contribution of metabolic factors and 4G/5G polymorphism of PAI-1 gene on the variability of PAI-1. Methods. We conducted an observational, cross-sectional assay in a hospital in Mexico City from May 2010 to September 2011. MetS was defined by the International Diabetes Federation criteria. PAI-1 levels and 4G/5G polymorphism were determined by ELISA and PCR-RFLP analysis. Results. We enrolled 215 subjects with T2DM plus MetS and 307 controls. Subjects with T2DM plus MetS had higher PAI-1 levels than the reference group (58.4 ± 21 versus 49.9 ± 16 ng/mL, p = 0.026). A model with components of MetS explained only 12% of variability on PAI-1 levels (R2 = 0.12; p = 0.001), with β = 0.18 (p = 0.03) for hypertension, β = −0.16 (p = 0.05) for NL HDL-c, and β = 0.15 (p = 0.05) for NL triglycerides. Conclusion. Subjects with T2DM plus MetS have elevated PAI-1 levels before clinical manifestations of atherothrombotic disease. Metabolic factors have a more important contribution than 4G/5G polymorphism on PAI-1 plasma variability. PMID:28271069

  13. Acute transverse myelitis as a rare manifestation of Campylobacter diarrhoea with concomitant disruption of the blood brain barrier.

    PubMed

    Gozzard, Paul; Orr, David; Sanderson, Frances; Sandberg, Michael; Kennedy, Angus

    2012-02-01

    We describe a case of acute transverse myelitis following Campylobacter diarrhoea in an adult. The patient presented with diplegia due to a longitudinal spinal cord lesion. The CSF demonstrated an aseptic meningitis. Oligoclonal bands and C. jejuni-specific IgG were detected in serum and cerebrospinal fluid at the beginning of the neurological illness. The patient was treated with antimicrobial therapy and steroids. A near full recovery was made and there were no relapses. C. jejuni is strongly implicated in the aetiology of acute motor axonal neuropathy and Miller Fisher syndrome through molecular mimicry of neuronal gangliosides. These gangliosides are expressed throughout the nervous system yet C. jejuni related central nervous system disease is exceedingly rare. We conclude that disruption of the blood-brain barrier was the key event in the pathogenesis of immune mediated post-infectious myelitis in our patient.

  14. Noninvasive imaging in acute coronary disease. A clinical perspective.

    PubMed

    Gersh, B J

    1991-09-01

    Numerous highly complex and sensitive noninvasive imaging techniques have enhanced the care of patients with acute myocardial infarction. Optimum use requires specific objectives to be defined in advance, including a review of the potential impact of the test on subsequent decisions. An additional issue that is subject to scrutiny in the current climate of cost containment relates to the incremental value of a specific examination. The imaging modality to be used will partially depend on other issues, including accessibility, cost, and interindividual or institutional expertise with a particular technique. Major applications in noninvasive imaging in the acute coronary syndromes include the following: 1) diagnosis, including identification of associated diseases and contraindications for acute reperfusion; 2) evaluation and management of complications (mechanical and nonmechanical); 3) determination of prognosis (both early and late); 4) estimation of myocardial viability; 5) assessment of therapeutic efficacy; 6) investigational approaches, including 99mTc-sestamibi tomographic imaging, ultrafast cine computed tomographic scanning, and nuclear magnetic resonance imaging. Previous studies in the prethrombolytic era have documented the powerful impact of radionuclide stress testing on prognosis, but this needs to be reevaluated in the light of the changing current population undergoing stress testing. Preliminary data imply that the prognostic accuracy of stress testing after thrombolytic therapy is diminished. Moreover, the role of the open infarct-related artery in traditional estimates of prognosis (e.g., ejection fraction) requires further study. Noninvasive imaging has multiple applications in the diagnosis and management of patients with acute coronary disease, but the decision to use a specific technology in a particular circumstance mandates good clinical judgment and selectivity.

  15. High blood pressure in acute ischemic stroke and clinical outcome.

    PubMed

    Manabe, Yasuhiro; Kono, Syoichiro; Tanaka, Tomotaka; Narai, Hisashi; Omori, Nobuhiko

    2009-11-16

    This study aimed to evaluate the prognostic value of acute phase blood pressure in patients with acute ischemic stroke by determining whether or not it contributes to clinical outcome. We studied 515 consecutive patients admitted within the first 48 hours after the onset of ischemic strokes, employing systolic and diastolic blood pressure measurements recorded within 36 hours after admission. High blood pressure was defined when the mean of at least 2 blood pressure measurements was ≥200 mmHg systolic and/or ≥110 mmHg diastolic at 6 to 24 hours after admission or ≥180 mmHg systolic and/or ≥105 mmHg diastolic at 24 to 36 hours after admission. The high blood pressure group was found to include 16% of the patients. Age, sex, diabetes mellitus, hypercholesterolemia, atrial fibrillation, ischemic heart disease, stroke history, carotid artery stenosis, leukoaraiosis, NIH Stroke Scale (NIHSS) on admission and mortality were not significantly correlated with either the high blood pressure or non-high blood pressure group. High blood pressure on admission was significantly associated with a past history of hypertension, kidney disease, the modified Rankin Scale (mRS) on discharge and the length of stay. On logistic regression analysis, with no previous history of hypertension, diabetes mellitus, atrial fibrillation, and kidney disease were independent risk factors associated with the presence of high blood pressure [odds ratio (OR), 1.85 (95% confidence interval (CI): 1.06-3.22), 1.89 (95% CI: 1.11-3.22), and 3.31 (95% CI: 1.36-8.04), respectively]. Multi-organ injury may be presented in acute stroke patients with high blood pressure. Patients with high blood pressure had a poor functional outcome after acute ischemic stroke.

  16. Parkin disease in a Brazilian kindred: Manifesting heterozygotes and clinical follow-up over 10 years.

    PubMed

    Khan, Naheed L; Horta, Wagner; Eunson, Louise; Graham, Elizabeth; Johnson, Janel O; Chang, Shannon; Davis, Mary; Singleton, Andrew; Wood, Nicholas W; Lees, Andrew J

    2005-04-01

    We report on a large Brazilian kindred with young-onset parkinsonism due to either a homozygous or heterozygous mutation in parkin. A total of 6 members were affected: 5 were homozygous and 1 heterozygous for a deletion in exon 4. Two other heterozygotes also had extrapyramidal signs. All affected subjects showed characteristic features of parkin disease with foot dystonia and an excellent response to levodopa complicated by motor fluctuations and dyskinesia within 3 years of therapy. Careful clinical follow-up over 10 years showed the phenotype was similar in all the homozygotes with asymmetrical limb bradykinesia and early walking difficulties. Some acceleration of disability was observed in some of the cases as they entered the third decade of illness, but dementia was absent.

  17. Cryptococcus gattii infections: contemporary aspects of epidemiology, clinical manifestations and management of infection.

    PubMed

    McMullan, Brendan Joseph; Sorrell, Tania Christine; Chen, Sharon Chih-Ann

    2013-12-01

    Cryptococcus gattii is an important primary and opportunistic pathogen, predominantly causing meningoencephalitis and pulmonary disease with substantial mortality. Initially considered geographically restricted to immune-competent, highly exposed individuals in the tropics, an apparent epidemic in North America has led to new perspectives on its ecology, epidemiology and clinical associations, which are distinct from its sibling species Cryptococcus neoformans. The role of C. gattii molecular genotypes/subtypes in different settings is under investigation. Diagnostic and treatment strategies are similar to those for C. neoformans in immunocompetent hosts, although data indicate that more prolonged induction, as well as total duration of therapy, is required. Exclusion of CNS involvement is mandatory. Brain cryptococcomas are characteristic of C. gattii infection, and raised intracranial pressure is common, for which surgery is often required. Immune reconstitution syndrome may occur. Ongoing C. gattii research and greater awareness and availability of specific diagnostic tests are required to improve patient outcomes.

  18. Severe Acute Respiratory Syndrome: Clinical Outcome and Prognostic Correlates1

    PubMed Central

    Kwok, Man Leung; Yuen, Hon; Lai, Sik To

    2003-01-01

    Severe acute respiratory syndrome (SARS) poses a major threat to the health of people worldwide. We performed a retrospective case series analysis to assess clinical outcome and identify pretreatment prognostic correlates of SARS, managed under a standardized treatment protocol. We studied 127 male and 196 female patients with a mean age of 41±14 (range 18–83). All patients, except two, received ribavirin and steroid combination therapy. In 115 (36%) patients, the course of disease was limited. Pneumonitis progressed rapidly in the remaining patients. Sixty-seven (21%) patients required intensive care, and 42 (13%) required ventilator support. Advanced age, high admission neutrophil count, and high initial lactate dehydrogenase level were independent correlates of an adverse clinical outcome. SARS-associated coronavirus caused severe illnesses in most patients, despite early treatment with ribavirin and steroid. This study has identified three independent pretreatment prognostic correlates. PMID:14519241

  19. Blastocystis and urticaria: Examination of subtypes and morphotypes in an unusual clinical manifestation.

    PubMed

    Casero, Rodolfo Daniel; Mongi, Florencia; Sánchez, Angie; Ramírez, Juan David

    2015-08-01

    Blastocystis is a human common enteric protist that may colonize a large variety of non-human hosts linked to symptoms and diseases such as abdominal pain, constipation, diarrhea, urticaria, flatulence and irritable bowel syndrome (IBS). Blastocystis exhibits remarkable genetic diversity and multiple subtypes (STs) within the genus with no absolute associations with clinical symptomatology. Here we analyzed fecal samples from Argentinean patients (n=270) belonging to symptomatic (urticaria and non-specific gastrointestinal symptoms, n=39) and asymptomatic control (n=28). Those patients infected with Blastocystis (n=67) were submitted for morphological analysis, DNA extraction, 18S PCR, sequencing and STs identification according to DNA barcoding. Blastocystis vacuolar forms were the predominant morphotype (75%), ameboid-like forms were evidenced in 1.5% of samples. Blastocystis ST3 was detected in 71.6% (n=48), of which 71.4%, (n=35) and 28.6% (n=14) belonged to symptomatic and asymptomatic respectively. Other subtypes identified were ST1 (14.9%), ST6 (7.5%) and ST2 (5.9%). Blastocystis 18S barcoding evidenced in non-urticaria symptomatic patients and asymptomatic control group the presence of allele 134 (ST3) (p<0.0001), while allele 34 (ST3) was detected in 85.7% (18/21) of symptomatic uricaria as compared with control group (1/21) (p<0.0001). The presence of a particular allele (a34) significantly associated with urticaria patients was detected and the clinical implications of these findings are herein discussed.

  20. Clinical manifestations of elderly patients with digitalis intoxication in the emergency department.

    PubMed

    Pita-Fernández, Salvador; Lombardía-Cortiña, Mónica; Orozco-Veltran, Domingo; Gil-Guillén, Vicente

    2011-01-01

    This study aimed to determine the clinical characteristics of elderly patients diagnosed with digitalis intoxication, on the Emergency Department, University Hospital Complex, A Coruña, Spain. During the study period (January-September 2008) cases were included in which digitalis intoxication was confirmed by plasma digoxin levels. We collected data on age, gender, base-line diseases, therapeutic indications for digoxin, functional classification, ejection fraction, plasma digoxin levels, creatinine clearance, ions, gasometry, electrocardiogram, concomitant medication, symptomatology and treatment. The results were: mean age 82.0 ± 6.6 years, predominantly female subjects (83.7%). The most prevalent pathologies were cardiac valvulopathy (81.0%), hypertension (68.3%) and ischemic cardiopathy (46.3%), 95.1% had a background of cardiac insufficiency, and 52.6% were in functional grade III. The mean digoxin level was 2.7 ± 0.69 ng/ml, 23.1% of the patients had a creatinine clearance of less than 60 ml/min/1.73 m(2) and 2.6% had a severely reduced glomerular filtration rate (GFR) (clearance<30 ml/min/1.73 m(2)). A negative correlation was found between digoxin levels and clearance (r = -0.22; p = 0.18) and between the levels and cardiac frequency (r = -0.35; p = 0.026). Of the patients, 47.5% presented bradycardia and 87.8% arrhythmias, most frequently auricular fibrillation. The most frequent symptoms were nausea (54.8%), fatigue (42.9%), vomiting (33.3%) and anorexia (28.6%). We conclude that clinical digestive symptoms in elderly women who are taking digitalis, with bradycardia and impaired renal functioning, should lead us to suspect digitalis intoxication.

  1. MANAGEMENT OF ACUTE SEVERE ULCERATIVE COLITIS: A CLINICAL UPDATE

    PubMed Central

    SOBRADO, Carlos Walter; SOBRADO, Lucas Faraco

    2016-01-01

    ABSTRACT Introduction: Acute severe colitis is a potentially lethal medical emergency and, even today, its treatment remains a challenge for clinicians and surgeons. Intravenous corticoid therapy, which was introduced into the therapeutic arsenal in the 1950s, continues to be the first-line treatment and, for patients who are refractory to this, the rescue therapy may consist of clinical measures or emergency colectomy. Objective: To evaluate the indications for and results from drug rescue therapy (cyclosporine, infliximab and tacrolimus), and to suggest a practical guide for clinical approaches. Methods: The literature was reviewed using the Medline/PubMed, Cochrane library and SciELO databases, and additional information from institutional websites of interest, by cross-correlating the following keywords: acute severe colitis, fulminating colitis and treatment. Results: Treatments for acute severe colitis have avoided colectomy in 60-70% of the cases, provided that they have been started early on, with multidisciplinary follow-up. Despite the adverse effects of intravenous cyclosporine, this drug has been indicated in cases of greater severity with an imminent risk of colectomy, because of its fast action, short half-life and absence of increased risk of surgical complications. Therapy using infliximab has been reserved for less severe cases and those in which immunosuppressants are being or have been used (AZA/6-MP). Indication of biological agents has recently been favored because of their ease of therapeutic use, their good short and medium-term results, the possibility of maintenance therapy and also their action as a "bridge" for immunosuppressant action (AZA/6-MP). Colectomy has been reserved for cases in which there is still no response five to seven days after rescue therapy and in cases of complications (toxic megacolon, profuse hemorrhage and perforation). Conclusion: Patients with a good response to rescue therapy who do not undergo emergency

  2. Molecular Epidemiology and Clinical Manifestations of Adenovirus Respiratory Infections in Taiwanese Children

    PubMed Central

    Wang, Ya-Fang; Shen, Fan-Ching; Wang, Shan-Li; Kuo, Pin-Hwa; Tsai, Huey-Pin; Liu, Ching-Chuan; Wang, Jen-Ren; Chi, Chia-Yu

    2016-01-01

    Abstract Human adenoviruses (HAdVs) are important causes of respiratory infections in children. They usually cause mild upper respiratory symptoms, but they can also produce severe pneumonia and other complications. The aims of this retrospective study were to better define the molecular epidemiology of respiratory adenoviruses circulating in Taiwanese children during 2002 and 2013, detect reinfections and co-infections, and characterize the clinical features and laboratory findings according to the causative genotypes. We collected a representative sample of 182 isolates of adenoviruses from 175 children during the 12-year study period. The most prevalent species was HAdV-B genotype 3 (HAdV-3) (92/182, 50.5%) followed by HAdV-C (HAdV-2) (38/182, 20.9%). A single outbreak of HAdV-E (6/182, 3.3%) was noted in 2007. The mean age of children with adenovirus infections was 3.7 ± 2.0 years, with a slight predominance of males (53.1%). Children with HAdV-B tended to be older, had more lower respiratory tract infections, gastrointestinal symptoms, and a higher rate of hospitalization than those with HAdV-C (P < 0.05). Adenovirus co-infections were noted in 25/175 (14.3%) of the children. The most frequent co-infections were with species B (HAdV-3) and C (HAdV-2) (14/25, 56.0%). Additional infections were noted in 23/175 (13.1%) of the children. Of these repeated infections, the initial isolates were always genotypes of HAdV-C. The second isolates were genotypes of HAdV-B or HAdV-E. The clinical features of the first HAdV-B infection and the reinfection of HAdV-B followed the HAdV-C were similar. In conclusion, HAdV-B, C, and E were the only adenovirus species that were isolated from children who were sufficiently ill with respiratory infections to require a visit to the hospital. Human adenovirus B (HAdV-3) accounted for half of these species. HAdV-B was more likely than other species to produce severe disease. The high incidence of adenovirus co-infection and

  3. The epidemiology and clinical manifestations of dysexecutive syndrome in Parkinson's disease.

    PubMed

    Ceravolo, Roberto; Pagni, Cristina; Tognoni, Gloria; Bonuccelli, Ubaldo

    2012-01-01

    This mini-review summarizes the evidence of the cognitive and behavioral features of dysexecutive syndrome in Parkinson's disease (PD). Deficits in response inhibition, set-shifting, mental flexibility, and strategy have been frequently described from the earliest stages of PD, although there are inconsistencies in study findings due to the complexity of the executive function (EF) construct and methodological limitations. Behavioral disorders of PD, e.g., apathy, distractibility, perseverative behavior, and impulse-control disorders, may be viewed as the other side of dysexecutive syndrome. Despite the interrelationship between the cognitive and behavioral domains, some reports reveal that the two syndromes may be dissociated, suggesting that both aspects must be clinically assessed. EFs are widely associated with the prefrontal areas, although dysexecutive syndrome may be observed in patients with damage to other brain regions. EFs drive numerous abilities essential to daily life, such as prospective remembering and language comprehension, which may be impaired in PD subjects. Considering the impact of dysexecutive syndrome on independence and quality of life, early detection of executive impairment is crucial in the management of PD.

  4. Clinical and radiological manifestations of the rheumatoid wrist after the Sauvé-Kapandji procedure.

    PubMed

    Momohara, Shigeki; Mamizuka, Kyoko; Yonemoto, Kouichi; Tomatsu, Taisuke; Inoue, Kazuhiko

    2004-01-01

    A retrospective study was performed to investigate the clinical and radiological results of the Sauvé-Kapandji (S-K) procedure for the rheumatoid wrist. One hundred and eight rheumatoid wrists in 98 patients were operated on in our institute from 1992 to 2000, and in 82 wrists we used the S-K procedure. In other cases, synovectomy alone was performed on 16 wrists, and partial and total arthrodeses were performed concurrently on 5 wrists each. Carpal bones and/or radiocarpal joints in which the union could not be assessed radiologically were found in 49 wrists (59.8%) after the S-K procedure, and among them there was definite non-fusion of the carpal bone and radiocarpal joints in 29 wrists (35.4%). However, definite fusion of carpal bones and/or radiocarpal joints was found in 33 wrists (40.2%). The formation of carpal bones and partial radiocarpal fusion with some mobility was detected in some cases. Therefore, the S-K procedure may stabilize the carpus in the rheumatoid wrist to some extent while maintaining a functionally important range of motion and relieving pain. However, it does not stop the disease process and cannot reestablish or maintain carpal height. We concluded that the S-K procedure is the treatment of choice for the rheumatoid wrist, and if the wrist is unstable, as seen with arthritis mutilans, we then perform either radio-lunate partial arthrodesis or total wrist arthrodesis.

  5. Clinical manifestations of diarrhea in calves infected with rotavirus and enterotoxigenic Escherichia coli.

    PubMed Central

    Tzipori, S R; Makin, T J; Smith, M L; Krautil, F L

    1981-01-01

    The susceptibility of gnotobiotic, colostrum-derived, or suckling calves to four bovine rotavirus isolates was found to be age dependent. Calves older than 7 days remained clinically normal, although they excreted virus in their feces and subsequently developed antibody against the virus, Enterotoxigenic Escherichia coli, fed to gnotobiotic, colostrum-deprived, or suckling calves ranging in age from a few hours to 26 days old, only caused diarrhea in animals younger than 24 h old. In contrast, diarrhea was consistently induced in 1- and 2-week-old calves infected with both enterotoxigenic E. coli and rotavirus. In general, diarrhea appeared after a rotavirus incubation period of approximately 3 days and was independent of the order in which the two microbial agents were given, the age of the calf, or the level of circulating rotavirus antibodies. The disease episode coincided with the excretion of rotavirus, rather than enterotoxigenic E. coli, in the feces. Infection with enterotoxigenic E. coli became established within 24 h of inoculation, and in older calves enterotoxigenic E. coli was often excreted in very small numbers and for a longer period than rotavirus. PMID:6265493

  6. [Immunophenotyping and clinical manifestations of NHL in Jilin Province and Akita county].

    PubMed

    Zhang, X; Miura, R; Nakahachi, A

    1995-05-01

    This was a comparative study of NHL in Jilin Province, China (JPC) and Akita county, Japan (ACJ). A total with 219 cases of their clinical features analyzed. The results indicated that in both areas, B-NHL predominated with a B to T cell ratio of 5.1 : 1 (JPC) and 2.4 : 1 (ACJ) respectively. Histologically B-NHL of defuse large-cell type was most commonly seen, the frequency of which was higher in JPC (61.5%) than in ACJ (37.2%). However, immunoblastic B-NHL was of common occurence in ACJ (26 out of 91 cases). In contrast, immunoblastic lympaoma of T cell lineage was very common in both areas, accounting for a half of all T-NHL. Most of the patients were treated with CHOP or COPP protocol, but the responses differred between the two phenotypes of NHL. Patients with B-NHL responded favorably to chemotherapy, with complete response (CR) in 54 of 70 treated cases (77.0%) in ACJ and 47 of 82 treated cases (57.3%) in JPC. The 3-year disease-free survial rate was 44% and 37%, respectively. The response to treatment in T-NHL patients was inferior with a CR rate of 59% in JPC cases. Only 2 in each series of patients kept the remission over 1 year.

  7. Coexistence of chronic lymphocytic thyroiditis with papillary thyroid carcinoma: clinical manifestation and prognostic outcome.

    PubMed

    Jeong, Jun Soo; Kim, Hyun Ki; Lee, Cho-Rok; Park, Seulkee; Park, Jae Hyun; Kang, Sang-Wook; Jeong, Jong Ju; Nam, Kee-Hyun; Chung, Woong Youn; Park, Cheong Soo

    2012-08-01

    The study aimed to identify the clinical characteristics of coexisting chronic lymphocytic thyroiditis (CLT) in papillary thyroid carcinoma (PTC) and to evaluate the influence on prognosis. A total of 1,357 patients who underwent thyroid surgery for PTC were included. The clinicopathological characteristics were identified. Patients who underwent total thyroidectomy (n = 597) were studied to evaluate the influence of coexistent CLT on prognosis. Among the total 1,357 patients, 359 (26.5%) had coexistent CLT. In the CLT group, the prevalence of females was higher than in the control group without CLT (P < 0.001). Mean tumor size and mean age in the patients with CLT were smaller than without CLT (P = 0.040, P = 0.047, respectively). Extrathyroidal extension in the patients with CLT was significantly lower than without CLT (P = 0.016). Among the subset of 597 patients, disease-free survival rate in the patients with CLT was significantly higher than without CLT (P = 0.042). However, the multivariate analysis did not reveal a negative association between CLT coexistence and recurrence. Patients with CLT display a greater female preponderance, smaller size, younger and lower extrathyroidal extension. CLT is not a significant independent negative predictive factor for recurrence, although presence of CLT indicates a reduced risk of recurrence.

  8. Facial manifestations of Epstein-Barr virus-related lymphoproliferative disease in childhood acute lymphoblastic leukemia in remission: Two atypical presentations.

    PubMed

    Lu, Benjamin Y; Kojima, Lisa; Huang, Mary S; Friedmann, Alison M; Ferry, Judith A; Weinstein, Howard J

    2016-11-01

    Epstein-Barr virus-related lymphoproliferative disease (EBV-LPD) rarely occurs in patients with acute lymphoblastic leukemia (ALL), who have not received hematopoietic transplantation. We describe EBV-LPD manifesting as facial lesions in two children with ALL in remission. One patient was a 16-year-old male with T-cell ALL with an EBV-positive angiocentric polymorphous lip lesion presenting as right-sided facial swelling. The other patient was a 12-year-old male with B-cell ALL with an EBV-positive polymorphous lymphoplasmacytic infiltrate presenting as bilateral dacryoadenitis. Neither patient had known primary immunodeficiencies. Both cases improved with immunosuppressant de-escalation. These cases suggest that immunosuppression induced by maintenance chemotherapy is sufficient to promote EBV-LPD.

  9. Post vaccine acute disseminated encephalomyelitis as the first manifestation of chromosome 22q11.2 deletion syndrome in a 15-month old baby: a case report.

    PubMed

    Valenzise, Mariella; Cascio, Antonio; Wasniewska, Malgorzata; Zirilli, Giuseppina; Catena, Maria Ausilia; Arasi, Stefania

    2014-09-29

    We describe a case of a 15-month-old female child admitted to our hospital because of fever, rash, neurological signs (oscillation between states of irritability and drowsiness), palpebral edema and drooping eyelid, appeared 10 days after the vaccination for measles, mumps and rubella. Brain MRI images showed multiple bilateral hyperintense lesions in the white matter typical of acute disseminated encephalomyelitis (ADEM), an autoimmune demyelinating disorder with inflammatory lesions of the central nervous system, due to viral antigens or vaccines. In the mean time, because of patient's vague phenotypic manifestations, suggestive of a genetic defect, array comparative genomic hybridization was carried out which showed the presence of a microdeletion 22q11.21, linked to the DiGeorge syndrome. Our case suggests that pediatric cases of post-vaccination ADEM, in which neurological signs persist, should be investigated for genetic phenotypical features, in order to exclude the presence of a genetic syndrome or disease.

  10. Baseline plasma fibrinolysis and its correlation with clinical manifestations in patients with Raynaud's phenomenon.

    PubMed Central

    Lau, C S; McLaren, M; Mackay, I; Belch, J J

    1993-01-01

    OBJECTIVES--(1) To assess if patients with various forms of Raynaud's phenomenon (RP) have abnormal plasma fibrinolysis that may contribute to diminished digital blood flow; (2) to assess whether patients with RP with evidence of endothelial damage have abnormal plasma fibrinolysis; (3) to determine the clinical relevance of abnormalities, if any, in plasma fibrinolysis in patients with RP. METHODS--One hundred and sixty eight patients with significant RP were studied--46 had primary Raynaud's disease (RD), 32 had suspected Raynaud's syndrome secondary to an undifferentiated connective tissue disorder (undifferentiated CTD), 25 had Raynaud's syndrome associated with atherosclerosis (athero RS), and 65 had an underlying connective tissue disease (CTD RS). All attended in the morning after a low fat light breakfast. After a clinical history was obtained, venous blood samples were collected without stasis for assays of plasma fibrinolysis and factor VIII von Willebrand factor antigen (fVIII vWF Ag). Results were compared with those obtained from normal subjects matched for sex and age. As patients with athero RS were significantly older than the other patients with Raynaud's phenomenon, two groups of control subjects were recruited--namely, 'old' and 'young' control subjects. RESULTS--Patients with CTD RS and athero RS had higher concentrations of fVIII vWF Ag (CTD RS median 174.5 range (45-370)% v 100 (38-202)%, p < 0.001; athero RS 182-5 (100-240)% v 100 (50-158)%, p < 0.001). Both had raised fibrinogen (CTD RS 3.25 (1.9-6.8) g/l v 2.4 (1.2-4.2) g/l, p < 0.001; athero RS 3.4 (2.2-6.2) g/l v 2.5 (1.8-3.9) g/l, p < 0.001) and both had diminished fibrinolysis with reduced plasminogen activator activity (CTD RS 79.5 (31-72) mm2 v 92 (37-197) mm2, p < 0.04; athero RS 73 (45-125) mm2 v 98 (41-197) mm2, p < 0.03). Patients with CTD RS also had raised plasminogen activity (3.3 (2.3-5.8) cU/ml v 2.9 (1.5-5.4) cU/ml, p < 0.001). On the contrary, patients with primary RD and

  11. Tuberculosis in neonates and infants: epidemiology, pathogenesis, clinical manifestations, diagnosis, and management issues.

    PubMed

    Skevaki, Chrysanthi L; Kafetzis, Dimitrios A

    2005-01-01

    Tuberculosis is one of the leading infectious causes of death and as such represents a major global health problem. Infants may develop congenital tuberculosis from an infectious mother or, most commonly, they may acquire postnatal disease by contact with an infectious adult source. Important epidemiologic, pathogenetic, and clinical data regarding the management of infantile disease are reviewed. Diagnostic evaluation includes tuberculin skin tests, chest radiography and other imaging studies, smears and cultures, examination of the cerebrospinal fluid, and polymerase chain reaction, as well as the more recent interferon-gamma assay. Pregnant women with a positive Mantoux skin test but normal chest x-ray should either start chemoprophylaxis during gestation or after delivery depending on the likelihood of being recently infected, their risk of progression to disease, as well as their clinical evidence of disease. Pregnant women with a positive Mantoux skin test and chest x-ray or symptoms indicative of active disease should be treated with non-teratogenic agents during gestation; all household contacts should also be screened. When tuberculosis is suspected around delivery, the mother should be assessed by chest x-ray and sputum smear; separation of mother and offspring is indicated only if the mother is non-adherent to medical treatment, needs to be hospitalized, or when drug-resistant tuberculosis is involved. According to the American Academy of Pediatrics, treatment of latent infection is highly effective with isoniazid administration for 9 months. This regimen may be extended to 12 months for immunocompromised patients. When drug resistance is suspected, combination therapies, which usually consist of isoniazid with rifampin (rifampicin), are administered until the results of susceptibility tests become available. Organisms resistant to isoniazid only may be treated with rifampin alone for a total of 6-9 months. All infants with tuberculosis disease should be

  12. Characterization of endocrine-disruption and clinical manifestations in large-mouth bass from Florida lakes

    SciTech Connect

    Gross, D.A.; Gross, T.S.; Johnson, B.; Folmar, L.

    1995-12-31

    Previous efforts from this laboratory have documented altered endocrine function and sexual differentiation for alligators and turtles from Lake Apopka in Central Florida. This lake has been exposed to a variety of contaminants which are potentially endocrine-disrupting. Therefore, a survey of large mouth bass populations was conducted on several lakes in North Central Florida to examine reproductive and clinical health. Large-mouth bass were collected from lakes Apopka, Griffin, Jessup and Woodruff. Approximately 24 fish (12 males and 12 females) were collected from each lake during the spawning (March--April) and non-reproductive (July--August) seasons. Plasma samples were collected for analysis of estrogen, testosterone and 11-keto-testosterone concentrations. Gonadal and liver tissues were collected for histological analysis. General blood chemistry analyses and parasite surveys were also conducted to estimate general health. Additionally, fillet samples were collected and analyzed for pesticide levels. Fish from Lake Apopka had unusual concentrations of estrogen and 11-keto-testosterone in plasma when compared to bass from Lakes Woodruff, Jessup and Griffin. Parasites loads were significantly higher for bass from lake Apopka than from the other lakes. Male bass on Apopka had depressed concentrations of 11-keto-testosterone, skewing the E/T ratios upward while female bass had higher concentrations of estrogens than females from the other lakes, again resulting in skewed E/T ratios. These skewed E/T ratios are similar to those observed for alligators on the same lake and raise the possibility that they are caused by contaminants. However, contaminant levels in fillets did not differ significantly between lakes. These studies indicate potentially altered reproductive and immunological function for large-mouth bass living in a contaminated lake.

  13. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome

    SciTech Connect

    Kimonis, V.E.; Yang, M.L.; Bale, S.J.

    1997-03-31

    Nevoid basal cell carcinoma syndrome (NBCC; Gorlin syndrome), an autosomal dominant disorder linked to 9q22.3-q31, and caused by mutations in PTC, the human homologue of the Drosophila patched gene, comprises multiple basal cell carcinomas, keratocysts of the jaw, palmar/plantar pits, spine and rib anomalies and calcification of the falx cerebri. We reviewed the findings on 105 affected individuals examined at the NIH since 1985. The data included 48 males and 57 females ranging in age from 4 months to 87 years. Eighty percent of whites (71/90) and 38% (5/13) of African-Americans had at least one basal cell carcinoma (BCC), with the first tumor occurring at a mean age of 23 (median 20) years and 21 (median 20) years, respectively. Excluding individuals exposed to radiation therapy, the number of BCCs ranged from 1 to >1,000 (median 8) and 1 to 3 (median 2), respectively, in the 2 groups. Jaw cysts occurred in 78/105 (74%) with the first tumor occurring in 80% by the age of 20 years. The number of total jaw cysts ranged from 1 to 28 (median 3). Palmar pits and plantar pits were seen in 87%. Ovarian fibromas were diagnosed by ultrasound in 9/52 (17%) at a mean age of 30 years. Medulloblastoma occurred in 4 patients at a mean age of 2.3 years. Three patients had cleft lip or palate. Physical findings include {open_quotes}coarse face{close_quotes} in 54%, relative macrocephaly in 50%, hypertelorism in 42%, frontal bossing in 27%, pectus deformity in 13%, and Sprengel deformity in 11%. This study delineates the frequency of the clinical and radiological anomalies in NBCC in a large population of US patients and discusses guidelines for diagnosis and management. 48 refs., 3 figs., 5 tabs.

  14. Alternating hemiplegia of childhood in Denmark: clinical manifestations and ATP1A3 mutation status.

    PubMed

    Hoei-Hansen, Christina E; Dali, Christine Í; Lyngbye, Troels J B; Duno, Morten; Uldall, Peter

    2014-01-01

    Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder characterized by early-onset recurrent distinctive hemiplegic episodes commonly accompanied by other paroxysmal features and developmental impairment. De novo mutations in ATP1A3 were recently identified as a genetic cause of AHC. To describe the entire Danish cohort of paediatric AHC patients we approached neuropaediatricians nationwide. All currently acknowledged Danish patients ≤16 years with AHC were genetically tested and seen by the same child neurologist (PU). Ten patients; seven girls and three boys were identified. Mean present age was 10.0 years (range 1-16). Mean age at presentation was 7.4 months (range 1-18 months). Sequencing of ATP1A3 in all ten patients revealed a pathogenic mutation in seven. Two females with moderate psychomotor impairment were heterozygous for the known p.G947R mutation, whereas one severely retarded boy was heterozygous for the common p.E815K mutation. The prevalent p.D801N mutation was identified in two moderate to severely retarded children. Interestingly, in a set of monochorionic male twins a novel p.D801E mutation was identified, underscoring that the asparagine at position 801 is a mutation hotspot. Three girls aged 5-13 years did not reveal any ATP1A3 mutations. They were rather mildly clinically affected and displayed a normal or near-normal psychomotor development. This is the first study of AHC in the Danish paediatric population. The patients harboured a wide range of psychomotor difficulties. Patients with no mutation detected tended to be less severely affected. Prevalence was approximately 1 per 100,000 children.

  15. Rheumatic manifestations of scurvy.

    PubMed

    Ferrari, Claudia; Possemato, Niccolò; Pipitone, Nicolò; Manger, Bernhard; Salvarani, Carlo

    2015-04-01

    This paper reviews the rheumatological manifestations of scurvy, based on articles published in English from 1965 until October 2014, with a particular focus on rheumatological manifestations. Scurvy is a rare, uncommon disease in developed countries. Due to its clinical heterogeneity, the disease can easily mimic rheumatologic conditions leading to a delay in diagnosis and treatment.

  16. Acute Kidney Injury: Definition, Pathophysiology and Clinical Phenotypes

    PubMed Central

    Makris, Konstantinos; Spanou, Loukia

    2016-01-01

    Acute kidney injury (AKI) is a clinical syndrome that complicates the course and worsens the outcome in a significant number of hospitalised patients. Recent advances in clinical and basic research will help with a more accurate definition of this syndrome and in the elucidation of its pathogenesis. With this knowledge we will be able to conduct more accurate epidemiologic studies in an effort to gain a better understanding of the impact of this syndrome. AKI is a syndrome that rarely has a sole and distinct pathophysiology. Recent evidence, in both basic science and clinical research, is beginning to change our view for AKI from a single organ failure syndrome to a syndrome where the kidney plays an active role in the progress of multi-organ dysfunction. Accurate and prompt recognition of AKI and better understanding of the pathophysiologic mechanisms underlying the various clinical phenotypes are of great importance to research for effective therapeutic interventions. In this review we provide the most recent updates in the definition, epidemiology and pathophysiology of AKI. PMID:28303073

  17. Acute Chorioamnionitis and Funisitis: Definition, Pathologic Features, and Clinical Significance

    PubMed Central

    Kim, Chong Jai; Romero, Roberto; Chaemsaithong, Piya; Chaiyasit, Noppadol; Yoon, Bo Hyun; Kim, Yeon Mee

    2015-01-01

    Acute inflammatory lesions of the placenta consist of diffuse infiltration of neutrophils at different sites in the organ. These lesions include acute chorioamnionitis, funisitis, and chorionic vasculitis, and represent a host response (maternal or fetal) to a chemotactic gradient in the amniotic cavity. While acute chorioamnionitis is evidence of a maternal host response, funisitis and chorionic vasculitis represent fetal inflammatory responses. Intra-amniotic infection has been generally considered to be the cause of acute histologic chorioamnionitis and funisitis; however, recent evidence indicates that “sterile” intra-amniotic inflammation, which occurs in the absence of demonstrable microorganisms but can be induced by “danger signals”, is frequently associated with these lesions. In the context of intra-amniotic infection, chemokines (such as interleukin-8 and granulocyte chemotactic protein) establish a gradient favoring the migration of neutrophils from maternal or fetal circulation into the chorioamniotic membranes or umbilical cord, respectively. Danger signals released during the course of cellular stress or cell death can also induce the release of neutrophil chemokines. The prevalence of chorioamnionitis is a function of gestational age at birth, and is present in 3-5% of placentas delivered at term, but in 94% of placentas delivered between 21-24 weeks of gestation. The frequency is higher in patients with spontaneous labor, preterm labor, clinical chorioamnionitis (preterm or term), or ruptured membranes. Funisitis and chorionic vasculitis are the hallmarks for the fetal inflammatory response syndrome, a condition characterized by an elevation in fetal plasma concentrations of interleukin-6, associated with the impending onset of preterm labor, a higher rate of neonatal morbidity (after adjustment for gestational age), and multi-organ fetal involvement. This syndrome is the counterpart of the systemic inflammatory response syndrome in adults

  18. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome.

    PubMed

    Kimonis, V E; Goldstein, A M; Pastakia, B; Yang, M L; Kase, R; DiGiovanna, J J; Bale, A E; Bale, S J

    1997-03-31

    affected individuals. This study delineates the frequency of the clinical and radiological anomalies in NBCC in a large population of US patients and discusses guidelines for diagnosis and management.

  19. Effect of Tobacco Smoking on The Clinical, Histopathological, and Serological Manifestations of Sjögren’s Syndrome

    PubMed Central

    Stone, Donald U.; Fife, Dustin; Brown, Michael; Earley, Keith E.; Radfar, Lida; Kaufman, C. Erick; Lewis, David M.; Rhodus, Nelson L.; Segal, Barbara M.; Wallace, Daniel J.; Weisman, Michael H.; Venuturupalli, Swamy; Brennan, Michael T.; Lessard, Christopher J.; Montgomery, Courtney G.; Scofield, R. Hal; Sivils, Kathy L.

    2017-01-01

    Objectives To assess the association of smoking habits with the clinical, serological, and histopathological manifestations of Sjögren’s syndrome (SS) and non-Sjögren’s sicca (non-SS sicca). Methods Cross-sectional case-control study of 1288 patients with sicca symptoms (587 SS and 701 non-SS sicca) evaluated in a multi-disciplinary research clinic. Smoking patterns were obtained from questionnaire data and disease-related clinical and laboratory data were compared between current, past, ever, and never smokers. Results Current smoking rates were 4.6% for SS patients compared to 14.1% in non-SS sicca (p = 5.17x10E-09), 18% in a local lupus cohort (p = 1.13x10E-14) and 16.8% in the community (p = 4.12x10E-15). Current smoking was protective against SS classification (OR 0.35, 95%CI 0.22–0.56, FDR q = 1.9E10-05), focal lymphocytic sialadenitis (OR 0.26, 95%CI 0.15–0.44, FDR q = 1.52x10E-06), focus score ≥1 (OR 0.22, 95%CI 0.13–0.39, FDR q = 1.43x10E-07), and anti-Ro/SSA(+) (OR 0.36, 95%CI 0.2–0.64, FDR q = 0.0009); ever smoking was protective against the same features and against anti-La/SSB(+) (OR 0.52, 95%CI 0.39–0.70, FDR q = 5.82x10E-05). Duration of smoking was inversely correlated with SS even after controlling for socioeconomic status, BMI, alcohol and caffeine consumption. Conclusions Current tobacco smoking is negatively and independently associated with SS, protecting against disease-associated humoral and cellular autoimmunity. The overall smoking rate amongst SS patients is significantly lower than in matched populations and the effects of smoking are proportional to exposure duration. In spite of the protective effects of tobacco on SS manifestations, it is associated with other serious comorbidities such as lung disease, cardiovascular risk and malignancy, and should thus be strongly discouraged in patients with sicca. PMID:28166540

  20. Quinine allergy causing acute severe systemic illness: report of 4 patients manifesting multiple hematologic, renal, and hepatic abnormalities

    PubMed Central

    2003-01-01

    Quinine is widely used for the common symptom of leg cramps. Quinine tablets require a prescription, but quinine and the product from which it is derived, cinchona, are also available without prescription. They are components of over-the-counter remedies for many common symptoms, of nutrition products, and of beverages such as tonic water and bitter lemon. Although quinine has been used for centuries, initially as an extract from the bark of the cinchona tree, allergic reactions to quinine can be severe and can affect multiple organs. These allergic reactions can cause thrombocytopenia, neutropenia, anemia, disseminated intravascular coagulation, acute renal failure, liver toxicity, and neurological abnormalities. Because quinine use is often intermittent, defining quinine as a cause of an acute disorder may be difficult. Moreover, since quinine use is often self-regulated, patients may not mention it in response to direct questions about medication use, adding to diagnostic difficulty. The diversity and severity of quinine-associated disorders and the difficulties of diagnosis are illustrated by the presentation of 4 case histories. Awareness of the variety of potential quinine-associated reactions is important for accurate diagnosis and critical for prevention of recurrent illness. PMID:16278718

  1. N-acetylcysteine in Acute Organophosphorus Pesticide Poisoning: A Randomized, Clinical Trial.

    PubMed

    El-Ebiary, Ahmad A; Elsharkawy, Rasha E; Soliman, Nema A; Soliman, Mohammed A; Hashem, Ahmed A

    2016-08-01

    Organophosphorus poisoning is a major global health problem with hundreds of thousands of deaths each year. Research interest in N-acetylcysteine has grown among increasing evidence of the role of oxidative stress in organophosphorus poisoning. We aimed to assess the safety and efficacy of N-acetylcysteine as an adjuvant treatment in patients with acute organophosphorus poisoning. This was a randomized, controlled, parallel-group trial on 30 patients suffering from acute organophosphorus poisoning, who were admitted to the Poison Control Center of Tanta University Emergency Hospital, Tanta, Egypt, between April and September 2014. Interventions included oral N-acetylcysteine (600 mg three times daily for 3 days) as an added treatment to the conventional measures versus only the conventional treatment. Outcome measures included mortality, total dose of atropine administered, duration of hospitalization and the need for ICU admission and/or mechanical ventilation. A total of 46 patients were screened and 30 were randomized. No significant difference was found between both groups regarding demographic characteristics and the nature or severity of baseline clinical manifestations. No major adverse effects to N-acetylcysteine therapy were reported. Malondialdehyde significantly decreased and reduced glutathione significantly increased only in the NAC-treated patients. The patients on NAC therapy required less atropine doses than those who received only the conventional treatment; however, the length of hospital stay showed no significant difference between both groups. The study concluded that the use of N-acetylcysteine as an added treatment was apparently safe, and it reduced atropine requirements in patients with acute organophosphorus pesticide poisoning.

  2. A recently isolated Lassa virus from Mali demonstrates atypical clinical disease manifestations and decreased virulence in cynomolgus macaques.

    PubMed

    Safronetz, David; Strong, James E; Feldmann, Friederike; Haddock, Elaine; Sogoba, Nafomon; Brining, Douglas; Geisbert, Thomas W; Scott, Dana P; Feldmann, Heinz

    2013-04-15

    The virulence of Soromba-R, a Lassa virus strain recently isolated from southern Mali, was assessed in 2 animal models of Lassa fever: inbred strain 13 guinea pigs and cynomolgus macaques. In both models, the Malian isolate demonstrated tissue tropism and viral titers similar to those of historical Lassa virus isolates from Sierra Leone (Josiah) and Liberia (Z-132); however, the Soromba-R isolate was found to be less pathogenic, as determined by decreased mortality and prolonged time to euthanasia in macaques. Interestingly, in addition to the classic indicators of Lassa fever, Soromba-R infection presented with moderate to severe pulmonary manifestations in the macaque model. Analysis of host responses demonstrated increased immune activation in Soromba-R-infected macaques, particularly in neutrophil-activating or -potentiating proinflammatory cytokines or growth factors, including tumor necrosis factor α, macrophage inflammatory protein 1α, interleukin 1β, and granulocyte colony-stimulating factor, as well as interleukin 5, which may be responsible for the decreased lethality and uncharacteristic clinical presentation. These results suggest that the strain of Lassa virus circulating in Mali might be less pathogenic than strains circulating in the historical region of endemicity and may result in an atypical presentation for Lassa fever, which could complicate clinical diagnosis.

  3. Clinical and electrophysiological parameters distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy.

    PubMed

    Dionne, Annie; Nicolle, Michael W; Hahn, Angelika F

    2010-02-01

    Up to 16% of chronic inflammatory demyelinating polyneuropathy (CIDP) patients may present acutely. We performed a retrospective chart review on 30 acute inflammatory demyelinating polyneuropathy (AIDP) and 15 acute-onset CIDP (A-CIDP) patients looking for any clinical or electrophysiological parameters that might differentiate AIDP from acutely presenting CIDP. A-CIDP patients were significantly more likely to have prominent sensory signs. They were significantly less likely to have autonomic nervous system involvement, facial weakness, a preceding infectious illness, or need for mechanical ventilation. With regard to electrophysiological features, neither sural-sparing pattern, sensory ratio >1, nor the presence of A-waves was different between the two groups. This study suggests that patients presenting acutely with a demyelinating polyneuropathy and the aforementioned clinical features should be closely monitored as they may be more likely to have CIDP at follow-up.

  4. Ocular Granulocytic Sarcoma as an Initial Clinical Presentation of Acute Myeloid Leukemia Identified on Flurodeoxyglucose Positron Emission Tomography/Computed Tomography

    PubMed Central

    Chandra, Piyush; Purandare, Nilendu; Shah, Sneha; Agrawal, Archi; Rangarajan, Venkatesh

    2017-01-01

    Granulocytic sarcoma (GS) or chloroma, rare extramedullary manifestation of acute myeloid leukemia and not infrequently, can be presenting clinical feature. Multiple studies have demonstrated the clinical utility of fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) in early detection and follow-up assessment of GS after chemotherapy. Commonly involved areas include bones, lymph nodes, breasts, and skin and not uncommonly, the disease can be multifocal. We present a rare case of ocular GS, where FDG-PET/CT in addition to the identifying the ocular mass, revealed multiple clinically occult extramedullary lesions. PMID:28242990

  5. Hashimoto's Encephalopathy Presenting with Acute Cognitive Dysfunction and Convulsion.

    PubMed

    Kang, Woo-Hyuk; Na, Ju-Young; Kim, Meyung-Kug; Yoo, Bong-Goo

    2013-12-01

    Hashimoto's encephalopathy is an immune-mediated disorder characterized by acute or subacute encephalopathy related to increased anti-thyroid antibodies. Clinical manifestations of Hashimoto's encephalopathy may include stroke-like episodes, altered consciousness, psychosis, myoclonus, abnormal movements, seizures, and cognitive dysfunction. Acute cognitive dysfunction with convulsion as initial clinical manifestations of Hashimoto's encephalopathy is very rare. We report a 65-year-old man who developed acute onset of cognitive decline and convulsion due to Hashimoto's encephalopathy.

  6. Severe hyponatremia caused by syndrome of inappropriate secretion of antidiuretic hormone developed as initial manifestation of human herpesvirus-6-associated acute limbic encephalitis after unrelated bone marrow transplantation.

    PubMed

    Kawaguchi, T; Takeuchi, M; Kawajiri, C; Abe, D; Nagao, Y; Yamazaki, A; Sugita, Y; Tsukamoto, S; Sakai, S; Takeda, Y; Ohwada, C; Sakaida, E; Shimizu, N; Yokote, K; Iseki, T; Nakaseko, C

    2013-04-01

    Severe hyponatremia is a critical electrolyte abnormality in allogeneic stem cell transplantation (allo-SCT) recipients and >50% of cases of severe hyponatremia are caused by the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Here, we present a patient with rapidly progressive severe hyponatremia as an initial sign and symptom of human herpesvirus-6-associated post-transplantation acute limbic encephalitis (HHV-6 PALE) after allo-SCT. A 45-year-old woman with acute lymphoblastic leukemia received unrelated bone marrow transplantation from a one locus-mismatched donor at the DR locus. On day 21, she developed a generalized seizure and loss of consciousness with severe hyponatremia, elevated serum antidiuretic hormone (ADH), and decreased serum osmolality. A high titer of HHV-6 DNA was detected in cerebrospinal fluid. Treatment with foscarnet sodium and hypertonic saline was started with improvement of neurological condition within several days. Although an elevated serum ADH, low serum osmolality, and high urinary osmolality persisted for 2 months, she had no other recurrent symptoms of encephalitis. Our experience suggests that hyponatremia accompanied by SIADH should be recognized as a prodromal or concomitant manifestation of HHV-6 PALE, and close monitoring of serum sodium levels in high-risk patients for HHV-6 PALE is necessary for immediate diagnosis and treatment initiation.

  7. Suspected acute exacerbation of idiopathic pulmonary fibrosis as an outcome measure in clinical trials

    PubMed Central

    2013-01-01

    Background Acute exacerbation of idiopathic pulmonary fibrosis has become an important outcome measure in clinical trials. This study aimed to explore the concept of suspected acute exacerbation as an outcome measure. Methods Three investigators retrospectively reviewed subjects enrolled in the Sildenafil Trial of Exercise Performance in IPF who experienced a respiratory serious adverse event during the course of the study. Events were classified as definite acute exacerbation, suspected acute exacerbation, or other, according to established criteria. Results Thirty-five events were identified. Four were classified as definite acute exacerbation, fourteen as suspected acute exacerbation, and seventeen as other. Definite and suspected acute exacerbations were clinically indistinguishable. Both were most common in the winter and spring months and were associated with a high risk of disease progression and short-term mortality. Conclusions In this study one half of respiratory serious adverse events were attributed to definite or suspected acute exacerbations. Suspected acute exacerbations are clinically indistinguishable from definite acute exacerbations and represent clinically meaningful events. Clinical trialists should consider capturing both definite and suspected acute exacerbations as outcome measures. PMID:23848435

  8. Clinical practice guidelines for the management of constipation in adults. Part 1: Definition, aetiology and clinical manifestations.

    PubMed

    Serra, Jordi; Mascort-Roca, Juanjo; Marzo-Castillejo, Mercè; Delgado Aros, Silvia; Ferrándiz Santos, Juan; Rey Diaz Rubio, Enrique; Mearin Manrique, Fermín

    2017-03-01

    Clinical practice guidelines for the management of constipation in adults aim to generate recommendations on the optimal approach to chronic constipation in the primary care and specialized outpatient setting. Their main objective is to provide healthcare professionals who care for patients with chronic constipation with a tool that allows them to make the best decisions about the prevention, diagnosis and treatment of this condition. They are intended for family physicians, primary care and specialist nurses, gastroenterologists and other health professionals involved in the treatment of these patients, as well as patients themselves. The guidelines have been developed in response to the high prevalence of chronic constipation, its impact on patient quality of life and recent advances in pharmacological management. The Grading of Recommendations Assessment, Development and Evaluation Working Group (GRADE) system has been used to classify the scientific evidence and strengthen the recommendations.

  9. Evaluation of an enzyme-linked immunosorbent assay using purified, deglycosylated histoplasmin for different clinical manifestations of histoplasmosis

    PubMed Central

    Guimarães, Allan Jefferson; Pizzini, Claudia Vera; De Abreu Almeida, Marcos; Peralta, José Mauro; Nosanchuk, Joshua Daniel; Zancopé-Oliveira, Rosely Maria

    2010-01-01

    Diagnosis of invasive fungal diseases remains problematic, especially in undeveloped countries. We have developed an enzyme-linked immunosorbent assay (ELISA) for the detection of antibodies to Histoplasma capsulatum using metaperiodate treated purified histoplasmin (ptHMIN). Our ELISA was validated comparing sera from patients with histoplasmosis, related mycoses, and healthy individuals. The overall test specificity was 96%, with sensitivities of 100% (8/8) in acute disease, 90% (9/10) in chronic disease, 89% (8/9) in disseminated infection in individuals without HIV infection, 86% (12/14) in disseminated disease in the setting of HIV infection and 100% (3/3) in mediastinal histoplasmosis. These parameters are superior to the use of untreated histoplasmin in diagnostic ELISAs. The high specificities, sensitivities, and simplicity of our ELISA support further development of a deglycosylated HMIN ELISA for clinical use and for monitoring the humoral immune response during therapy in patients with chronic and disseminated histoplasmosis. PMID:21691458

  10. Genital chlamydial infection among women in Nicaragua: validity of direct fluorescent antibody testing, prevalence, risk factors and clinical manifestations.

    PubMed Central

    Herrmann, B; Espinoza, F; Villegas, R R; Smith, G D; Ramos, A; Egger, M

    1996-01-01

    OBJECTIVE: To validate the performance of a direct fluorescence antibody (DFA) test and to determine the prevalence, risk factors and clinical manifestations of cervical chlamydia infection in different groups of women in Nicaragua. STUDY POPULATION: 926 women, 863 routine clinic attenders (mean age 27 years) and 63 sex workers (mean age 25 years) attending health centres in León, Corinto, Matagalpa and Bluefields. METHODS: Cervical specimens were examined using the Syva MicroTrak test system with a cut-off of 10 or more elementary bodies (EBs). The DFA results were validated by a one-step polymerase chain reaction (PCR) assay. Discordant results were further examined in nested PCR assays directed at two different target genes. An interviewer-administered questionnaire and a standard gynaecological examination were completed. RESULTS: Sensitivity of DFA was 80.1%, specificity 98.3%, and positive and negative predictive values 62.5% and 99.3%, respectively. Values were lower in locations where samples thawed because of electricity breaks and higher among sex workers. The majority of discordant results was confirmed as positive in nested PCR assays. Prevalence of cervical chlamydia infection based on positivity in DFA and/or PCR ranged from 2% among routine clinic attenders aged 35 years or older, to 8% among adolescent clinic attenders, and to 14% among sex workers. Among routine clinic attenders, young age (odds ratio [OR] 3.6, 95% confidence intervals [95% CI] 1.4-8.9 for women aged 15-19 years as compared with 1 in women 25 years of age or older) and use of oral contraceptives (OR 4.0, 95% CI 1.7-9.6) were the only statistically significant risk factors identified in multivariate logistic regression analysis. Presence of mucopurulent cervical discharge (OR 5.9, 95% CI 3.0-11.5) and presence of ectropion (OR 2.6, 95% CI 1.1-6.5) were the clinical signs independently associated with infection. CONCLUSIONS: Our results indicate that the DFA test was sensitive and

  11. Linking Doses with Clinical Scores of Hematopoietic Acute Radiation Syndrome.

    PubMed

    Hu, Shaowen

    2016-10-01

    In radiation accidents, determining the radiation dose the victim received is a key step for medical decision making and patient prognosis. To reconstruct and evaluate the absorbed dose, researchers have developed many physical devices and biological techniques during the last decades. However, using the physical parameter "absorbed dose" alone is not sufficient to predict the clinical development of the various organs injured in an individual patient. In operational situations for radiation accidents, medical responders need more urgently to classify the severity of the radiation injury based on the signs and symptoms of the patient. In this work, the author uses a unified hematopoietic model to describe dose-dependent dynamics of granulocytes, lymphocytes, and platelets, and the corresponding clinical grading of hematopoietic acute radiation syndrome. This approach not only visualizes the time course of the patient's probable outcome in the form of graphs but also indirectly gives information of the remaining stem and progenitor cells, which are responsible for the autologous recovery of the hematopoietic system. Because critical information on the patient's clinical evolution can be provided within a short time after exposure and only peripheral cell counts are required for the simulation, these modeling tools will be useful to assess radiation exposure and injury in human-involved radiation accident/incident scenarios.

  12. Nuclear medicine in the acute clinical setting: indications, imaging findings, and potential pitfalls.

    PubMed

    Uliel, Livnat; Mellnick, Vincent M; Menias, Christine O; Holz, Andrew L; McConathy, Jonathan

    2013-01-01

    Nuclear medicine imaging provides valuable functional information that complements information obtained with anatomic imaging techniques in the evaluation of patients with specific acute clinical manifestations. Nuclear medicine studies are most often used in conjunction with other imaging modalities and as a problem-solving tool. Under certain circumstances a nuclear medicine study may be indicated as the first-line imaging modality, as in the case of renal scintigraphy for transplant dysfunction in the early postoperative period. Nuclear imaging may be preferred when a conventional first-line study is contraindicated or when it is important to minimize radiation exposure. The portability of nuclear imaging offers particular advantages for the evaluation of critically ill patients whose clinical condition is unstable and who cannot be safely transported out of the intensive care unit. The ability to visualize physiologic and pathophysiologic processes over relatively long time periods without adding to the patient's radiation exposure contributes to the high diagnostic sensitivity of several types of nuclear medicine studies. Viewing the acquired images in the cine mode adds to the value of these studies for diagnosing and characterizing dynamic abnormalities such as intermittent internal bleeding and bile or urine leakage. In this pictorial review, the spectrum of nuclear medicine studies commonly performed in the acute care setting is reviewed according to body systems and organs, with detailed descriptions of the indications, technical considerations, findings, and potential pitfalls of each type of study. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.332125098/-/DC1.

  13. Pulmonary manifestations of sickle cell disease

    PubMed Central

    Siddiqui, A; Ahmed, S

    2003-01-01

    Pulmonary complications account for significant morbidity and mortality in patients with sickle cell disease. Clinical lung involvement manifests in two major forms: the acute chest syndrome and sickle cell chronic lung disease. Acute chest syndrome is characterised by fever, chest pain, and appearance of a new infiltrate on chest radiograph. Sickle cell chronic lung disease, on the other hand, manifests as radiographic interstitial abnormalities, impaired pulmonary function, and, in its most severe form, by the evidence of pulmonary hypertension. Progress has been made in understanding the pathophysiology and management of these complications. In this review the current knowledge of the mechanism, diagnosis, and treatment of pulmonary complications of sickle cell disease are discussed. PMID:12897216

  14. Clinical manifestations of autoimmune disease-related non-Hodgkin lymphoma: a Korean single-center, retrospective clinical study

    PubMed Central

    Jeon, Young-Woo; Yoon, Jae-Ho; Lee, Sung-Eun; Eom, Ki-Seong; Kim, Yoo-Jin; Kim, Hee-Je; Lee, Seok; Min, Chang-Ki; Lee, Jong Wook; Min, Woo-Sung; Cho, Seok-Goo

    2016-01-01

    Background/Aims: Recently, large cohort studies regarding associations between autoimmune disease and lymphomas have been reported in a few Western countries. However, Asian data concerning autoimmune-related lymphomas are limited. Therefore, we evaluated the clinical characteristics and prognostic factors of patients with autoimmune disease-related non-Hodgkin lymphoma (NHL) in a single center in Korea. Methods: We analyzed the data from 11 patients with autoimmune-related NHL. Patients were categorized into two groups, those with rheumatoid arthritis (RA) and those with non-RA-related NHL. Then patients were re-categorized into a group with methotrexate (MTX) usage and a MTX non-usage group. Histological subtype, MTX duration, autoimmune disease duration, treatment modalities, and other data were collected and analyzed. Results: Our study revealed that older RA patients have a greater likelihood of occurrence of NHL (p = 0.042). We confirmed that MTX duration and cumulative dose of MTX have no significant correlation with autoimmune disease and NHL (p = 0.073). In the management of autoimmune disease-related NHL, all patients were directly treated with systemic chemotherapy instead of employing a wait and watch approach. Overall survival (OS) and progression-free survival (PFS) in all autoimmune disease-related NHL were 100% and 87.5%, with no treatment-related mortality during the 2-year follow-up period of our study. Conclusions: Our study suggests that patients with RA-NHL are characterized by older age at onset compared to those with non-RA-NHL. Also considering of OS and PFS, intensive treatment strategy instead of delayed watchful managements may be required for autoimmune disease-related NHL including of old age group. PMID:27384438

  15. Does chronic hepatitis B infection affect the clinical course of acute hepatitis A?

    PubMed

    Shin, Su Rin; Moh, In Ho; Jung, Sung Won; Kim, Jin Bae; Park, Sang Hoon; Kim, Hyoung Su; Jang, Myung Kuk; Lee, Myung Seok

    2013-01-01

    The impact of chronic hepatitis B on the clinical outcome of acute hepatitis A remains controversial. The aim of present study was to evaluate the clinical characteristics of acute hepatitis A in cases with underlying chronic hepatitis B compared to cases of acute hepatitis A alone. Data on 758 patients with acute hepatitis A admitted at two university-affiliated hospitals were reviewed. Patients were classified into three groups: group A, patients with both acute hepatitis A and underlying chronic hepatitis B (n = 27); group B, patients infected by acute hepatitis A alone whose sexes and ages were matched with patients in group A (n  = 54); and group C, patients with acute hepatitis A alone (n = 731). None of the demographic features of group A were significantly different from those of group B or C, except for the proportion of males and body weight, which differed from group C. When comparing to group B, clinical symptoms were more frequent, and higher total bilirubin and lower albumin levels were observed in group A. When comparing to group C, the albumin levels were lower in group A. There were no differences in the duration of hospital stay, occurrence of acute kidney injury, acute liver failure, prolonged cholestasis, or relapsing hepatitis. This study revealed that clinical symptoms and laboratory findings were less favorable for patients with acute hepatitis A and chronic hepatitis B compared to those with acute hepatitis A alone. However, there were no differences in fatal outcomes or serious complications.

  16. Current status of hepatic glycogen storage disease in Japan: clinical manifestations, treatments and long-term outcomes.

    PubMed

    Kido, Jun; Nakamura, Kimitoshi; Matsumoto, Shirou; Mitsubuchi, Hiroshi; Ohura, Toshihiro; Shigematsu, Yosuke; Yorifuji, Tohru; Kasahara, Mureo; Horikawa, Reiko; Endo, Fumio

    2013-05-01

    Many reports have been published on the long-term outcome and treatment of hepatic glycogen storage diseases (GSDs) overseas; however, none have been published from Japan. We investigated the clinical manifestations, treatment, and prognosis of 127 hepatic GSD patients who were evaluated and treated between January 1999 and December 2009. A characteristic genetic pattern was noted in the Japanese GSD patients: most GSD Ia patients had the g727t mutation, and many GSD Ib patients had the W118R mutation. Forty-one percent (14/34) of GSD Ia patients and 18% (2/11) of GSD Ib patients of ages 13 years 4 months had liver adenoma. Among subjects aged 10 years, 19% (7/36) of the GSD Ia patients and none of the GSD Ib patients had renal dysfunction. The mean height of male GSD Ia patients aged 18 years was 160.8±10.6 cm (n=14), and that of their female counterparts was 147.8±3.80 cm (n=9). Patients with hepatic GSDs develop a variety of symptoms but can survive in the long term by diet therapy, corn starch treatment and supportive care. Liver transplantation for hepatic GSDs is an important treatment strategy and can help improve the patients'quality of life.

  17. The Spectrum of Infectious Diseases in Kidney Transplantation: A Review of the Classification, Pathogens and Clinical Manifestations.

    PubMed

    Anastasopoulos, Nikolaos-Andreas; Duni, Anila; Peschos, Dimitrios; Agnantis, Niki; Dounousi, Evangelia

    2015-01-01

    Kidney transplantation is the treatment-of-choice for a significant number of patients with end-stage renal disease. Renal transplant recipients (RTRs) benefit from a longer life expectancy, with a better quality of life. Despite, recent accomplishments in the field of kidney transplantation, both short- and long-term, surgical and medical complications still exist. Among these complications, cardiovascular disease, carcinogenesis and infections are the most important. Infectious diseases constitute the most common complications after renal transplantation and the second most common cause of death among RTRs with a functioning graft. Theoretically, all infectious pathogens could cause disease in immunocompromised RTRs, yet among these, one could identify more important ones, such as the Enterobacteriaceae, causing urinary tract infections; pneumonia due to Pneumocystis jirovecii; Candida species which cause invasive fungal infections; herpes viruses; hepatitis viruses and parasites. Early diagnosis and effective treatment are key elements in salvaging both the allograft and the patient. However, clinical manifestations and diagnosis of such infectious diseases are not easily identified due to the altered state of immune response of the RTR. Thus, apart from possessing a deep knowledge of the etiology and the treatment options in each case, transplant physicians should also always remain alert when dealing with RTRs.

  18. Is a hypothetical melanoma-like neuromelanin the underlying factor essential for the aetiopathogenesis and clinical manifestations of multiple sclerosis?

    PubMed Central

    2013-01-01

    Background Multiple sclerosis (MS) has undergone a significant increase in incidence in the industrialised nations over the last 130 years. Changing environmental factors, possibly infections or a lack of or altered timing of them, determine the prevalence of the disease. Although a plethora of aetiological factors, clearly evident in a group of children with MS, appear relevant, there may nevertheless be a single factor essential for the aetiopathogenesis and clinical manifestation of MS. Description and discussion This hitherto unknown factor is postulated to be a ‘melanoma-like neuromelanin’ (MLN) dependent on the activation of a gene for syncytin-1. An involvement of MLN could explain the diverse findings in the epidemiology, immunology and pathology of MS, requiring a consideration of a complex infectious background, the human leucocyte antigens, as well as cosmic radiation causing geomagnetic disturbances, vitamin D deficiency, smoking, and lower levels of uric acid. Summary In principle, the MLN-based concept is a unifying one, capable of explaining a number of characteristics of the disease. To date, MLN has not been addressed in studies on MS and future work will need to be done on human patients, as there is little or no neuromelanin (the precursor of MLN) in the animals used as experimental models in the study of MS. PMID:23865526

  19. Clinical review: the implications of experimental and clinical studies of recruitment maneuvers in acute lung injury.

    PubMed

    Piacentini, Enrique; Villagrá, Ana; López-Aguilar, Josefina; Blanch, Lluis

    2004-04-01

    Mechanical ventilation can cause and perpetuate lung injury if alveolar overdistension, cyclic collapse, and reopening of alveolar units occur. The use of low tidal volume and limited airway pressure has improved survival in patients with acute lung injury or acute respiratory distress syndrome. The use of recruitment maneuvers has been proposed as an adjunct to mechanical ventilation to re-expand collapsed lung tissue. Many investigators have studied the benefits of recruitment maneuvers in healthy anesthetized patients and in patients ventilated with low positive end-expiratory pressure. However, it is unclear whether recruitment maneuvers are useful when patients with acute lung injury or acute respiratory distress syndrome are ventilated with high positive end-expiratory pressure, and in the presence of lung fibrosis or a stiff chest wall. Moreover, it is unclear whether the use of high airway pressures during recruitment maneuvers can cause bacterial translocation. This article reviews the intrinsic mechanisms of mechanical stress, the controversy regarding clinical use of recruitment maneuvers, and the interactions between lung infection and application of high intrathoracic pressures.

  20. Biomarkers in acute kidney injury - pathophysiological basis and clinical performance.

    PubMed

    Schrezenmeier, E V; Barasch, J; Budde, K; Westhoff, T; Schmidt-Ott, K M

    2017-03-01

    Various biomarkers of acute kidney injury (AKI) have been discovered and characterized in the recent past. These molecules can be detected in urine or blood and signify structural damage to the kidney. Clinically, they are proposed as adjunct diagnostics to serum creatinine and urinary output to improve the early detection, differential diagnosis and prognostic assessment of AKI. The most obvious requirements for a biomarker include its reflection of the underlying pathophysiology of the disease. Hence, a biomarker of AKI should derive from the injured kidney and reflect a molecular process intimately connected with tissue injury. Here, we provide an overview of the basic pathophysiology, the cellular sources and the clinical performance of the most important currently proposed biomarkers of AKI: neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), liver-type fatty acid-binding protein (L-FABP), interleukin-18 (IL-18), insulin-like growth factor-binding protein 7 (IGFBP7), tissue inhibitor of metalloproteinase 2 (TIMP-2) and calprotectin (S100A8/9). We also acknowledge each biomarker's advantages and disadvantages as well as important knowledge gaps and perspectives for future studies.

  1. UNUSUAL CLINICAL CASES THAT MIMIC ACUTE DISSEMINATED ENCEPHALOMYELITIS.

    PubMed

    Duman, Özgür; Yürekli, Vedat Ali; Gencpinar, Pinar; Karaali, Kamil; Gümüş, Hakan; Okuyaz, Çetin; Hazar, Volkan; Haspolat, Şenay

    2015-09-01

    Acute disseminated encephalomyelitis (ADEM) is an immune-mediated monophasic inflammatory demyelinating disorder of the central nervous system which poses a diagnostic challenge. We report on six cases of different etiologies that mimicked the clinical and radiologic findings of ADEM. The cases were collected from four different reference hospitals in Turkey. The same radiologist from the Akdeniz University Faculty of Medicine examined the magnetic resonance images of all patients. Three (50%) patients had antecedent infections. Initial symptoms of the patients were as follows: fever in 50%, altered consciousness in 33.3% and convulsions in 16.7% of patients. Neurologic examination showed long tract signs in 83.3%, ataxia in 50% and altered consciousness in 50% of patients. Cerebrospinal fluid examination revealed lymphocytic pleocytosis only in case 6. Four patients received steroid pulse therapy and one of these initially underwent intravenous immunoglobulin therapy. The patients' definitive diagnoses were as follows: paraspinal neuroblastoma-associated paraneoplastic syndrome; histiocytic sarcoma; mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes; and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy in one patient each, while two patients had hemophagocytic syndrome. The present case series demonstrated difficulties in diagnosing ADEM while revealing extremely rare disorders that mimic ADEM radiologically and clinically.

  2. FLT3 inhibitors: clinical potential in acute myeloid leukemia

    PubMed Central

    Hospital, Marie-Anne; Green, Alexa S; Maciel, Thiago T; Moura, Ivan C; Leung, Anskar Y; Bouscary, Didier; Tamburini, Jerome

    2017-01-01

    Acute myeloid leukemia (AML) is an aggressive hematopoietic malignancy that is cured in as few as 15%–40% of cases. Tremendous improvements in AML prognostication arose from a comprehensive analysis of leukemia cell genomes. Among normal karyotype AML cases, mutations in the FLT3 gene are the ones most commonly detected as having a deleterious prognostic impact. FLT3 is a transmembrane tyrosine kinase receptor, and alterations of the FLT3 gene such as internal tandem duplications (FLT3-ITD) deregulate FLT3 downstream signaling pathways in favor of increased cell proliferation and survival. FLT3 tyrosine kinase inhibitors (TKI) emerged as a new therapeutic option in FLT3-ITD AML, and clinical trials are ongoing with a variety of TKI either alone, combined with chemotherapy, or even as maintenance after allogenic stem cell transplantation. However, a wide range of molecular resistance mechanisms are activated upon TKI therapy, thus limiting their clinical impact. Massive research efforts are now ongoing to develop more efficient FLT3 TKI and/or new therapies targeting these resistance mechanisms to improve the prognosis of FLT3-ITD AML patients in the future. PMID:28223820

  3. [Prediction of infiltrative pulmonary tuberculosis outcomes depending on the procedure of its detection and on the manifestation of clinical and x-ray symptoms].

    PubMed

    Volchegorskiĭ, I A; Novoselov, P N; Bolotov, A A

    2009-01-01

    The possibility of predicting the outcomes of infiltrative pulmonary tuberculosis (IPT) depending on the procedure of its detection and on the manifestations of clinical and X-ray symptoms has been assessed. The severity of clinical symptoms of IPT is shown to be much higher in patients who actively seek medical aid than that in patients identified at planned fluorographic study. Actively seeking medical aid and the high baseline degree of clinical and X-ray manifestations have been found to be significant predictors of a good response to combined drug therapy for IPT with the clinical recovery being achieved in patients, the minor residual (posttuberculosis) changes developed in the lung, and the decreased need for surgical treatment for IPT.

  4. Acute Cardioembolic Cerebral Infarction: Answers to Clinical Questions*

    PubMed Central

    Arboix, Adrià; Alió, Josefina

    2012-01-01

    Cardioembolic cerebral infarction (CI) is the most severe subtype of ischaemic stroke but some clinical aspects of this condition are still unclear. This article provides the reader with an overview and up-date of relevant aspects related to clinical features, specific cardiac disorders and prognosis of CI. CI accounts for 14−30% of ischemic strokes; patients with CI are prone to early and long-term stroke recurrence, although recurrences may be preventable by appropriate treatment during the acute phase and strict control at follow-up. Certain clinical features are suggestive of CI, including sudden onset to maximal deficit, decreased level of consciousness at onset, Wernicke’s aphasia or global aphasia without hemiparesis, a Valsalva manoeuvre at the time of stroke onset, and co-occurrence of cerebral and systemic emboli. Lacunar clinical presentations, a lacunar infarct and especially multiple lacunar infarcts, make cardioembolic origin unlikely. The most common disorders associated with a high risk of cardioembolism include atrial fibrillation, recent myocardial infarction, mechanical prosthetic valve, dilated myocardiopathy and mitral rheumatic stenosis. Patent foramen ovale and complex atheromatosis of the aortic arch are potentially emerging sources of cardioembolic infarction. Mitral annular calcification can be a marker of complex aortic atheroma in stroke patients of unkown etiology. Transthoracic and transesophageal echocardiogram can disclose structural heart diseases. Paroxysmal atrial dysrhyhtmia can be detected by Holter monitoring. Magnetic resonance imaging, transcranial Doppler, and electrophysiological studies are useful to document the source of cardioembolism. In-hospital mortality in cardioembolic stroke (27.3%, in our series) is the highest as compared with other subtypes of cerebral infarction. Secondary prevention with anticoagulants should be started immediately if possible in patients at high risk for recurrent cardioembolic stroke in

  5. Myanmarese Neuropathy: Clinical Description of Acute Peripheral Neuropathy Detected among Myanmarese Refugees in Malaysia.

    PubMed

    Fu Liong, Hiew; Santhi, Datuk Puvanarajah; Shanthi, Viswanathan; Mohd Hanip, Rafia

    2014-01-01

    Background. Since 2008, we have observed an increasing number of Myanmarese refugees in Malaysia being admitted for acute/subacute onset peripheral neuropathy. Most of them had a preceding history of starvation. Methods. We retrospectively studied the clinical features of all Myanmarese patients admitted with peripheral neuropathy from September 2008 to January 2014. Results. A total of 24 patients from the Chin, Rohingya, and Rakhine ethnicities (mean age, 23.8 years; male, 96%) had symmetrical, ascending areflexic weakness with at least one additional presenting symptom of fever, lower limb swelling, vomiting, abdominal pain, or difficulty in breathing. Twenty (83.3%) had sensory symptoms. Ten (41.6%) had cranial nerve involvement. Nineteen patients had cerebrospinal fluid examinations but none with evidence of albuminocytological dissociation. Neurophysiological assessment revealed axonal polyneuropathy, predominantly a motor-sensory subtype. Folate and vitamin B12 deficiencies were detected in 31.5% of them. These findings suggested the presence of a polyneuropathy related to nutrition against a backdrop of other possible environmental factors such as infections, metabolic disorders, or exposure to unknown toxin. Supportive treatment with appropriate vitamins supplementation improved functional outcome in most patients. Conclusion. We report a spectrum of acquired reversible neurological manifestations among Myanmarese refugees likely to be multifactorial with micronutrient deficiencies playing an important role in the pathogenesis.

  6. Clinical and pathological features of fat embolism with acute respiratory distress syndrome.

    PubMed

    Kao, Shang Jyh; Yeh, Diana Yu-Wung; Chen, Hsing I

    2007-09-01

    FES (fat embolism syndrome) is a clinical problem, and, although ARDS (acute respiratory distress syndrome) has been considered as a serious complication of FES, the pathogenesis of ARDS associated with FES remains unclear. In the present study, we investigated the clinical manifestations, and biochemical and pathophysiological changes, in subjects associated with FES and ARDS, to elucidate the possible mechanisms involved in this disorder. A total of eight patients with FES were studied, and arterial blood pH, PaO(2) (arterial partial pressure of O(2)), PaCO(2) (arterial partial pressure of CO(2)), biochemical and pathophysiological data were obtained. These subjects suffered from crash injuries and developed FES associated with ARDS, and each died within 2 h after admission. In the subjects, chest radiography revealed that the lungs were clear on admission, and pulmonary infiltration was observed within 2 h of admission. Arterial blood pH and PaO(2) declined, whereas PaCO(2) increased. Plasma PLA(2) (phospholipase A(2)), nitrate/nitrite, methylguanidine, TNF-alpha (tumour necrosis factor-alpha), IL-1beta (interleukin-1beta) and IL-10 (interleukin-10) were significantly elevated. Pathological examinations revealed alveolar oedema and haemorrhage with multiple fat droplet depositions and fibrin thrombi. Fat droplets were also found in the arterioles and/or capillaries in the lung, kidney and brain. Immunohistochemical staining identified iNOS (inducible nitric oxide synthase) in alveolar macrophages. In conclusion, our clinical analysis suggests that PLA(2), NO, free radicals and pro-inflammatory cytokines are involved in the pathogenesis of ARDS associated with FES. The major source of NO is the alveolar macrophages.

  7. Non-human Immunodeficiency Virus-related Ocular Syphilis in a Korean Population: Clinical Manifestations and Treatment Outcomes

    PubMed Central

    Kim, Yonguk; Kwak, Hyung Woo

    2016-01-01

    Purpose To describe the clinical manifestations and treatment outcomes of ocular syphilis in patients without human immunodeficiency virus (HIV) infection. Methods A total of 45 eyes from 39 patients with ocular syphilis confirmed by serologic tests were reviewed retrospectively. The included cases were all non-HIV-infected patients presenting with intraocular inflammation from 2002 to 2014 at Kyung Hee University Hospital. Medical records of 45 eyes were analyzed and included best-corrected visual acuity and ophthalmologic examination findings of the anterior and posterior segments to determine the focus of inflammation. Optical coherence tomography and fluorescein angiography findings as well as both medical and surgical management were also analyzed. Results The mean patient age was 61.0 years (range, 37 to 89 years). Bilateral ocular involvement occurred in 6 patients (15.4%), and diagnoses at presentation were most frequently related to posterior uveitis (38%), followed by panuveitis (29%) and optic neuritis (11%). Isolated interstitial keratitis and intermediate uveitis were uncommon (4%, both). Twenty-eight eyes (62.2%) were treated with penicillin, and 11 eyes (24.4%) underwent surgical treatment. The mean baseline best corrected visual acuity was 0.79 ± 0.59 (mean ± standard deviation, logarithm of the minimum angle of resolution) and significantly improved to 0.60 ± 0.63 at the final follow-up after treatment (p = 0.019). Mean visual improvement was significantly greater in the penicillin-treated group (p = 0.001). Visual impairment at the final visit occurred in 11 eyes (24.4%). Among the visual impairment group, 10 eyes (90.1%) had posterior segment-involving uveitis. Conclusions Visual outcomes of treated, non-HIV-related ocular syphilis were favorable regardless of time to presentation. Posterior segment-involving uveitis at presentation was associated with poor visual outcome. PMID:27729756

  8. The influence of clinical manifestations and treatment on satisfaction with life together with positive and negative emotions in systemic lupus erythematosus patients.

    PubMed

    Kulczycka, Lilianna; Sysa-Jędrzejowska, Anna; Robak, Ewa

    2011-01-01

    The aim was to determine the satisfaction with life together with positive and negative emotions in systemic lupus erythematosus (SLE) patients, and correlate them with clinical manifestations of the disease and method of treatment. The study included 83 SLE patients. Satisfaction with life was measured using the Satisfaction with Life Scale. Positive and negative aspects were assessed using the Positive and Negative Affects Schedule. Other data were collected from patients at the time of measurement. Satisfaction with life as well as positive and negative emotions are connected with both the patient clinical condition and mode of therapy. There are correlations of these parameters with the number of medicines used and clinical manifestations of the disease. Systemic lupus erythematosus as a long-lasting and incurable disease has an impact not only on the patient quality of life but also on satisfaction with life and patient emotions. That is why it is very important to measure all these parameters to improve patient compliance.

  9. DQ2, DQ7 and DQ8 Distribution and Clinical Manifestations in Celiac Cases and Their First-Degree Relatives

    PubMed Central

    Araya, Magdalena; Oyarzun, Amaya; Lucero, Yalda; Espinosa, Nelly; Pérez-Bravo, Francisco

    2015-01-01

    HLA-linked genes are relevant to celiac disease (CD); the potential genetic differences present worldwide are not fully understood. Previous results suggest that the distribution of HLA-DQ2/DQ7/DQ8 in Chile may differ from that in Europe and North America. In celiac patients and their first-degree relatives (FDRS), we assessed their clinical, serological and histological characteristics, determined HLA-DQ2, HLA-DQ7 and HLA-DQ8 alleles and genotypes, and evaluated the relations between them. A total of 222 individuals were assessed (56 cases, 166 FDRs). 16.9% of FDRs were tTG positive; 53.6% of them showed overweight/obesity and 3% undernourishment; they spontaneously declared being asymptomatic, but detailed questioning revealed that 60.7% experienced symptoms, which had not been investigated. DQ2 was present in 53.9% and 43.9.0% of cases and FDRs (p < 0.05). The most frequent genotype distribution was DQ2/DQ7 (fr 0.392 (cases) and 0.248 (FDRs), respectively, p < 0.02). The next most common genotypes were HLA-DQ2/DQ8 (fr 0.236 in FDRs and 0.176 in cases, p < 0.05). 3.92% cases were not HLA-DQ2/DQ8 carriers. Among tTG positive FDRs, 57.4%, 22.3% and 20.2% carried DQ2, DQ7 and DQ8, respectively. In cases, 72.7% of the biopsies classified Marsh ≥3 carried at least one DQ2; 91.7% of DQ2/DQ2 and 88.3% of DQ2/DQ7 were Marsh ≥3. Thus, DQ2 frequency is lower than reported; the higher frequency found for DQ8 and DQ7 concur with recent publications from Argentine and Brazil. These results suggest that although CD may manifest clinically in ways similar to those described in other populations, some genetic peculiarities in this region deserve further study. PMID:26096569

  10. Discriminant validity of constructs derived from the self-regulative model for evaluation anxiety for predicting clinical manifestations of test anxiety.

    PubMed

    Herzer, Frank; Wendt, Julia; Hamm, Alfons O

    2015-10-01

    Test anxiety is a highly prevalent and impairing syndrome. However, research on clinically relevant manifestations of test anxiety and especially on effective treatment components is still very sparse. In the present study we examined the predictive validity of constructs derived from the self-regulative model for evaluation anxiety proposed by Zeidner and Matthews (2007) for discriminating clinical and non-clinical levels of test anxiety. We compared self-report data from 47 clinically test anxious patients with those from 41 healthy university students. Results showed that learning goals, self-concept of abilities, self-incrimination, elaboration and perfectionism were the constructs that independently separated clinical from non-clinical levels of test anxiety, thus providing the strongest discriminant validity even when controlling for an effect of the global severity of mental health problems. These constructs spread across all three domains proposed in the model, thus providing important implications for possible targets of interventions to reduce clinical levels of test anxiety.

  11. Correlation between clinical presentation, peroperative finding and histopathological report in acute appendicitis.

    PubMed

    Khan, S A; Gafur, M A; Islam, A; Rahman, M S

    2011-10-01

    Acute appendicitis is usually encountered clinically as acute abdomen. Typical cases are easy to diagnose, but sometimes it is very difficult to make a diagnosis in atypical cases. The objective of the study was to determine the diagnostic accuracy in patient of clinically diagnosed acute appendicitis. This prospective study conducted in Mymensingh medical college hospital on 1136 patients presented with acute abdomen and clinically diagnosed as acute appendicitis from July 2004 to June 2010. Emergency appendicectomy was done in all consecutive patients after relevant investigation. Intraoperative findings along with histopathological reports were compared with clinical diagnosis. On the basis of histopathological report, 85.65% were found to have acute appendicitis with misdiagnosis in rest of the subjects requiring unnecessary explorations. Negative exploration was more in emergency than office hour. This may be due to diagnostic inaccuracy and decision-making in the management of the acute appendicitis. Management errors can be significantly reduced by accurate preoperative diagnosis of acute appendicitis by improving clinical skill and appropriate investigations.

  12. Canadian clinical practice guidelines for acute and chronic rhinosinusitis

    PubMed Central

    2011-01-01

    This document provides healthcare practitioners with information regarding the management of acute rhinosinusitis (ARS) and chronic rhinosinusitis (CRS) to enable them to better meet the needs of this patient population. These guidelines describe controversies in the management of acute bacterial rhinosinusitis (ABRS) and include recommendations that take into account changes in the bacteriologic landscape. Recent guidelines in ABRS have been released by American and European groups as recently as 2007, but these are either limited in their coverage of the subject of CRS, do not follow an evidence-based strategy, or omit relevant stakeholders in guidelines development, and do not address the particulars of the Canadian healthcare environment. Advances in understanding the pathophysiology of CRS, along with the development of appropriate therapeutic strategies, have improved outcomes for patients with CRS. CRS now affects large numbers of patients globally and primary care practitioners are confronted by this disease on a daily basis. Although initially considered a chronic bacterial infection, CRS is now recognized as having multiple distinct components (eg, infection, inflammation), which have led to changes in therapeutic approaches (eg, increased use of corticosteroids). The role of bacteria in the persistence of chronic infections, and the roles of surgical and medical management are evolving. Although evidence is limited, guidance for managing patients with CRS would help practitioners less experienced in this area offer rational care. It is no longer reasonable to manage CRS as a prolonged version of ARS, but rather, specific therapeutic strategies adapted to pathogenesis must be developed and diffused. Guidelines must take into account all available evidence and incorporate these in an unbiased fashion into management recommendations based on the quality of evidence, therapeutic benefit, and risks incurred. This document is focused on readability rather than

  13. Pulmonary drug toxicity: radiologic and pathologic manifestations.

    PubMed

    Rossi, S E; Erasmus, J J; McAdams, H P; Sporn, T A; Goodman, P C

    2000-01-01

    Pulmonary drug toxicity is increasingly being diagnosed as a cause of acute and chronic lung disease. Numerous agents including cytotoxic and noncytotoxic drugs have the potential to cause pulmonary toxicity. The clinical and radiologic manifestations of these drugs generally reflect the underlying histopathologic processes and include diffuse alveolar damage (DAD), nonspecific interstitial pneumonia (NSIP), bronchiolitis obliterans organizing pneumonia (BOOP), eosinophilic pneumonia, obliterative bronchiolitis, pulmonary hemorrhage, edema, hypertension, or veno-occlusive disease. DAD is a common manifestation of pulmonary drug toxicity and is frequently caused by cytotoxic drugs, especially cyclophosphamide, bleomycin, and carmustine. It manifests radiographically as bilateral hetero- or homogeneous opacities usually in the mid and lower lungs and on high-resolution computed tomographic (CT) scans as scattered or diffuse areas of ground-glass opacity. NSIP occurs most commonly as a manifestation of carmustine toxicity or of toxicity from noncytotoxic drugs such as amidarone. At radiography, it appears as diffuse areas of heterogeneous opacity, whereas early CT scans show diffuse ground-glass opacity and late CT scans show fibrosis in a basal distribution. BOOP, which is commonly caused by bleomycin and cyclophosphamide (as well as gold salts and methotrexate), appears on radiographs as hetero- and homogeneous peripheral opacities in both upper and lower lobes and on CT scans as poorly defined nodular consolidation, centrilobular nodules, and bronchial dilatation. Knowledge of these manifestations and of the drugs most frequently involved can facilitate diagnosis and institution of appropriate treatment.

  14. Prevalence, severity, and clinical features of acute and chronic pancreatitis in patients with systemic lupus erythematosus.

    PubMed

    Wang, Qiang; Shen, Min; Leng, Xiaomei; Zeng, Xiaofeng; Zhang, Fengchun; Qian, Jiaming

    2016-10-01

    Pancreatitis is a rare, life-threatening complication of systemic lupus erythematosus (SLE). This study aimed to describe the clinical features of acute pancreatitis (AP) and chronic pancreatitis (CP) in patients with SLE. Data of patients who fulfilled the revised criteria of the American Rheumatism Association for diagnosis of SLE were retrospectively analyzed. SLE activity was graded according to the SLE Disease Activity Index. Logistic regression analysis was conducted to find out independent associations. Survival rates were estimated by using Kaplan-Meier plots. This study included 5665 SLE patients admitted between January 1983 and January 2014, of whom 52 patients were diagnosed with pancreatitis. Pancreatitis prevalence in SLE patients was 0.92 % (52/5665). AP (0.8 %, 46/5665) was more prevalent than CP (0.1 %, 6/5665), presented mostly during active SLE, and affected more organs. Hypertriglyceridemia occurred in 76.9 % of AP patients and in none of the CP patients. AP patients were divided into severe (n = 10) or mild (n = 20) cases. The average triglyceride level in severe AP cases was higher than that in mild AP cases (P = 0.006), and the mortality rate of lupus-associated AP was 32.6 % (15/46). Concomitant infections and thrombocytopenia were independently associated with poor prognosis (P < 0.001, P = 0.028, respectively). There were significant differences in the clinical manifestations of AP and CP. Patients with severe AP were found to have a higher incidence of concomitant infection and serum triglyceride levels. Concomitant infections and thrombocytopenia were independent risk factors for poor prognosis.

  15. [Clinical and microbiological study of acute pelvic inflammatory disease].

    PubMed

    Ovalle, A; Martínez, M A; Casals, A; Yuhaniak, R; Giglio, M S

    1993-01-01

    Upper genital tract infection was investigated in 46 women admitted to hospital with clinic diagnosis of acute pelvic inflammatory disease (PID) and 62 control women accepted to hospital for laparoscopy Fallopian tubes sterilization. Diagnosis was ratified by laparoscopy in mild and moderate salpingitis; culdocentesis and ultrasonography were performed in severe salpingitis and endometrial sample was made in endometritis. Microbiological specimens were taken from the cervix and abdomen. Antecedents and complete clinical studies were obtained. Patients were treated with antibiotic association sodic G penicillin, chloramphenicol and gentamicin. Risk factors to development PID were: single female (p < 0.05), multiple sexual partner (p < 0.01), previous PID (p < 0.05), infertility (p < 0.05), mean year of IUD use in severe salpingitis (p = 0.05) and mean years of age from women with sexually transmitted bacterias (STB) vs endogenous bacterias (EB) (p < 0.05). In the control group no abdomen bacterias were isolated. In patients with PID, C. trachomatis was detected by serology in 28.3%. N. gonorrhoeae was isolated from the cervix in 23.9% and from the abdomen 17.4%. Besides it was isolated from the abdomen: M. hominis 17.3% and E. coli 15.2%. STB were isolated in 54.3% and EB in 47.8% of the patients. Bacterial association was present on the 37%. Cervix isolation of G. vaginalis and Mycoplasma were not correlated with development of PID. Cervix microbiological samples were useful to know abdomen microbic etiology. They coincide with those in the 90.9%. EB were more frequently isolated from severe salpingitis (p = 0.05) and STB from mild and moderate salpingitis (p = 0.05). Antibiotic association cured all the mild and moderate salpingitis with independence of bacterial etiology. Failure occurred in 2 diffuse peritonitis and 13/14 tubo-ovarian abscesses. Surgery used in severe salpingitis and diffuse peritonitis, principally consisted in anexectomy, peritoneal toilet and

  16. Acute kidney injury in pregnancy: a clinical challenge.

    PubMed

    Machado, Susana; Figueiredo, Nuno; Borges, Andreia; São José Pais, Maria; Freitas, Luís; Moura, Paulo; Campos, Mário

    2012-01-01

    The incidence of acute kidney injury in pregnancy declined significantly over the second half of the 20th century; however, it is still associated with major maternal and perinatal morbidity and mortality. A set of systemic and renal physiological adaptive mechanisms occur during a normal gestation that will constrain several changes in laboratory parameters of renal function, electrolytes, fluid and acid-base balances. The diagnosis of acute kidney injury in pregnancy is based on the serum creatinine increase. The usual formulas for estimating glomerular filtration rate are not validated in this population. During the first trimester of gestation, acute kidney injury develops most often due to hyperemesis gravidarum or septic abortion. In the third trimester, the differential diagnosis is more challenging for the obstetrician and the nephrologist and comprises some pathologies that are reviewed in this article: preeclampsia/HELLP syndrome, acute fatty liver of pregnancy and thrombotic microangiopathies.

  17. The Clinical Course of a Drug-induced Acute Dystonic Reaction in the Emergency Room

    PubMed Central

    Marano, Massimo; di Biase, Lazzaro; Salomone, Gaetano; Di Santo, Alessandro; Montiroli, Annalisa; Di Lazzaro, Vincenzo

    2016-01-01

    Background Acute dystonic reactions following the administration of safe, reliable drugs can occur and must be promptly recognized and treated in the emergency room. Phenomenology Shown The entire clinical course of an acute dystonic reaction due to metoclopramide, from early motor signs to full-blown clinical symptoms and resolution. Educational Value Providing elements for early recognition of a drug-induced movement disorder phenomenology. PMID:28105387

  18. Acute Flaccid Myelitis: A Clinical Review of US Cases 2012–2015

    PubMed Central

    Messacar, Kevin; Schreiner, Teri L.; Van Haren, Keith; Yang, Michele; Glaser, Carol A.; Tyler, Kenneth L.; Dominguez, Samuel R.

    2016-01-01

    This review highlights clinical features of the increasing cases of acute flaccid paralysis associated with anterior myelitis noted in the United States from 2012 to 2015. Acute flaccid myelitis refers to acute flaccid limb weakness with spinal cord gray matter lesions on imaging or evidence of spinal cord motor neuron injury on electrodiagnostic testing. Although some individuals demonstrated improvement in motor weakness and functional deficits, most have residual weakness a year or more after onset. Epidemiological evidence and biological plausibility support an association between enterovirus D68 and the recent increase in acute flaccid myelitis cases in the United States. PMID:27422805

  19. Acute severe mitral regurgitation. Pathophysiology, clinical recognition, and management.

    PubMed

    DePace, N L; Nestico, P F; Morganroth, J

    1985-02-01

    Acute severe mitral regurgitation often goes unrecognized as an emergency requiring prompt, lifesaving treatment. Its causes, physical signs, natural history, echocardiographic features, and findings on chest roentgenography, electrocardiography, and nuclear scintigraphic scanning are reviewed. Acute severe mitral insufficiency can be differentiated from chronic severe mitral insufficiency by noninvasive two-dimensional echocardiography. M-mode echocardiography is a valuable tool in evaluating mitral prosthetic paravalvular regurgitation.

  20. Hyaline fibromatosis syndrome: cutaneous manifestations*

    PubMed Central

    Marques, Silvio Alencar; Stolf, Hamilton Ometto; Polizel, Juliana Ocanha; Munhoz, Tânia; Brandão, Marcela Calixto; Marques, Mariangela Esther Alencar

    2016-01-01

    Hyaline fibromatosis syndrome is the current name for clinical manifestations of diseases previously known as “infantile systemic hyalinosis” and “juvenile hyaline fibromatosis”. The authors report representative clinical cases of each one of the above subtypes with emphasis on cutaneous manifestations and difficulties for early diagnosis in this syndrome, essentially of multidisciplinary approach. PMID:27192526

  1. Gastrointestinal system manifestations in juvenile systemic lupus erythematosus.

    PubMed

    Sönmez, Hafize Emine; Karhan, Asuman Nur; Batu, Ezgi Deniz; Bilginer, Yelda; Gümüş, Ersin; Demir, Hülya; Yüce, Aysel; Özen, Seza

    2017-02-16

    Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68.4%). The GIS manifestations due to SLE were autoimmune hepatitis (AIH) (n = 8) and lupus enteritis (n = 1). Manifestations associated with SLE were hepatomegaly and hypertransaminasemia due to macrophage activation syndrome (MAS) (n = 3) and hepatic steatosis (n = 1). GIS manifestations as a result of the adverse events of drugs were as follows: toxic hepatitis (n = 3; associated with methotrexate and nonsteroidal anti-inflammatory drugs in one, methotrexate in another, and azathioprine in another patient), azathioprine-induced cholestatic hepatitis (n = 1), and gastritis associated with corticosteroid (n = 1). In one patient, acute appendicitis occurred as a coincidence. In this study, one of every five pediatric SLE patients had GIS-related manifestations. GIS involvement may occur as an initial manifestation of the disease.

  2. Clinical and Mechanistic Drivers of Acute Traumatic Coagulopathy

    PubMed Central

    Cohen, Mitchell Jay; Kutcher, Matt; Redick, Britt; Nelson, Mary; Call, Mariah; Knudson, M Margaret; Schreiber, Martin A; Bulger, Eileen M; Muskat, Peter; Alarcon, Louis H; Myers, John G; Rahbar, Mohammad H; Brasel, Karen J; Phelan, Herb A; del Junco, Deborah J; Fox, Erin E; Wade, Charles E; Holcomb, John B; Cotton, Bryan A; Matijevic, Nena

    2013-01-01

    Background Acute Traumatic Coagulopathy (ATC) occurs after severe injury and shock and is associated with increased bleeding, morbidity and mortality. The effects of ATC and hemostatic resuscitation on outcome are not well-explored. The PRospective Observational Multicenter Major Trauma Transfusion (PROMMTT) study provided a unique opportunity to characterize coagulation and the effects of resuscitation on ATC after severe trauma. Methods Blood samples were collected upon arrival on a subset of PROMMTT patients. Plasma clotting factor levels were prospectively assayed for coagulation factors. These data were analyzed with comprehensive PROMMTT clinical data. Results There were 1198 patients with laboratory results of whom 41.6% were coagulopathic. Using International Normalized Ratio (INR)≥1.3, 41.6% (448) of patients were coagulopathic while 20.5% (214) were coagulopathic using partial thromboplastin time (PTT)≥35. Coagulopathy was primarily associated with a combination of an ISS>15 and a BD<−6 (P<.05). Regression modeling for INR-based coagulopathy shows that pre-hospital crystalloid (odds ratio (OR)=1.05), Injury Severity Score (ISS, OR=1.03), Glasgow Coma Scale (OR=0.93), heart rate (OR=1.08), systolic blood pressure (OR=0.96), base deficit (BD, OR=0.92) and temperature (OR=0.84) were significant predictors of coagulopathy (all P<.03). A subset of 165 patients had blood samples collected and coagulation factor analysis performed. Elevated ISS and BD were associated with elevation of aPC and depletion of factors (all P<.05). Reductions in factors I, II, V, VIII and an increase in aPC drive ATC (all p<.04). Similar results were found for PTT-defined coagulopathy. Conclusions ATC is associated with depletion of factors I, II, V, VII, VIII, IX and X and is driven by the activation of the protein C system. These data provide additional mechanistic understanding of the drivers of coagulation abnormalities after injury. Further understanding of the drivers of

  3. Manifestations of Lyme carditis.

    PubMed

    Kostić, Tomislav; Momčilović, Stefan; Perišić, Zoran D; Apostolović, Svetlana R; Cvetković, Jovana; Jovanović, Andriana; Barać, Aleksandra; Šalinger-Martinović, Sonja; Tasić-Otašević, Suzana

    2017-04-01

    The first data of Lyme carditis, a relatively rare manifestation of Lyme disease, were published in eighties of the last century. Clinical manifestations include syncope, light-headedness, fainting, shortness of breath, palpitations, and/or chest pain. Atrioventricular (AV) electrical block of varying severity presents the most common conduction disorder in Lyme carditis. Although is usually mild, AV block can fluctuates rapidly and progress from a prolonged P-R interval to a His-Purkinje block within minutes to hours and days. Rarely, Lyme disease may be the cause of endocarditis, while some studies and reports, based on serological and/or molecular investigations, have suggested possible influence of Borrelia burgdorferi on degenerative cardiac valvular disease. Myocarditis, pericarditis, pancarditis, dilated cardiomyopathy, and heart failure have also been described as possible manifestations of Lyme carditis. The clinical course of Lyme carditis is generally mild, short term, and in most cases, completely reversible after adequate antibiotic treatment.

  4. Infantile Cortical Hyperostosis: Report of a Case with Observations on Clinical Manifestations, Radiology, and Pathology with a Late Follow-Up of Eight Years

    PubMed Central

    Aymore, Ierecê Lins; Amoedo, Armando Rocha; Hemais, Paulo Miguel

    2016-01-01

    Purpose. The purpose of our study was to investigate clinical manifestations, roentgen images, histopathological studies, and evolution of the disease in patient displaying infantile cortical hyperostosis. Methods. Roentgenograms were made to evaluate a neonatal patient presenting multiple soft-tissue swellings. The initial radiographs insinuated that the disease had been present for some time in utero. Bone puncture biopsy of the tibia for histopathological observation and diagnosis conclusions was performed. Results. The disease was demonstrated radiographically by massive cortical diaphyseal thickening and also extensive periosteal new bone formation surrounding several bones. Results in blood count were as follows: discrete anemia, moderate leukocytosis, and elevated sedimentation rate. Histological pattern of tissue removed from tibia showed lamellar cortical bones and hyperplasia. Biopsy studies disclosed no evidence of neoplasia as well as of bacterial infection. Comments. Clinical manifestations in a neonatal patient displaying infantile cortical hyperostosis have gradually decreased. Radiograph findings have demonstrated complete recovery of bones manifested by the disease. The pathologic findings are in accordance with previous microscopic examination summarized by the literature. Total patient cure, without sequels, could be demonstrated. PMID:28050300

  5. PATTERN OF CUTANEOUS MANIFESTATIONS IN DIABETES MELLITUS

    PubMed Central

    Goyal, Abhishek; Raina, Sujeet; Kaushal, Satinder S; Mahajan, Vikram; Sharma, Nand Lal

    2010-01-01

    Background: Diabetes mellitus affects individuals of all ages and socioeconomic status. Skin is affected by the acute metabolic derangements as well as by chronic degenerative complications of diabetes. Aims: To evaluate the prevalence of skin manifestations in patients with diabetes mellitus. To analyze the prevalence and pattern of skin disorders among diabetic patients from this region of Western Himalayas. Materials and Methods: One hundred consecutive patients with the diagnosis of diabetes mellitus and having skin lesions, either attending the diabetic clinic or admitted in medical wards were included in this study. Results: The common skin disorders were: Xerosis (44%), diabetic dermopathy (36%), skin tags (32%), cutaneous infections (31%), and seborrheic keratosis (30%). Conclusion: Skin is involved in diabetes quite often and the manifestations are numerous. High prevalence of xerosis in our diabetic population is perhaps due to cold and dry climatic conditions in the region for most of the time in the year. PMID:20418975

  6. A Clinical Skills Instruction Program: The Acute Abdomen.

    ERIC Educational Resources Information Center

    Laube, Douglas W.; And Others

    1982-01-01

    An effective evaluation of the acutely ill female implies a thorough examination that integrates skills representing three learning domains. This process should include: a thorough medical history, a physical examination, good patient-physician rapport, and development of an efficacious management plan. A University of Iowa simulation approach is…

  7. Acute pulmonary oedema.

    PubMed

    Powell, Jessica; Graham, David; O'Reilly, Sarah; Punton, Gillian

    2016-02-03

    Acute pulmonary oedema is a distressing and life-threatening illness that is associated with a sudden onset of symptoms. For the best possible patient outcomes, it is essential that nurses in all clinical areas are equipped to accurately recognise, assess and manage patients with acute pulmonary oedema. This article outlines the pathophysiology of acute cardiogenic and non-cardiogenic pulmonary oedema, and suggests a systematic approach to the recognition and management of its most serious manifestations. Long-term care and symptom recognition are discussed and suggestions for ongoing patient self-management are provided.

  8. Integrated Clinical Geriatric Pharmacy Clerkship in Long Term, Acute and Ambulatory Care.

    ERIC Educational Resources Information Center

    Polo, Isabel; And Others

    1994-01-01

    A clinical geriatric pharmacy clerkship containing three separate practice areas (long-term, acute, and ambulatory care) is described. The program follows the medical education clerkship protocol, with a clinical pharmacy specialist, pharmacy practice resident, and student. Participation in medical rounds, interdisciplinary conferences, and…

  9. Acute care for alcohol intoxication. Be prepared to consider clinical dilemmas.

    PubMed

    Yost, David A

    2002-12-01

    The clinical assessment of an acutely intoxicated patient should be performed with meticulous care and include repetitive examinations to properly determine the patient's condition. Multiple factors, such as trauma and concomitant use of other drugs, can confuse the diagnostic picture and affect the choice of therapy. In this article, Dr Yost reviews the diagnostic considerations, appropriate treatment, and clinic discharge for the intoxicated patient.

  10. Cutaneous Manifestations of ESRD

    PubMed Central

    Cronin, Antonia J.; Leslie, Kieron S.

    2014-01-01

    Summary A broad range of skin diseases occurs in patients with ESRD: from the benign and asymptomatic to the physically disabling and life-threatening. Many of them negatively impact on quality of life. Their early recognition and treatment are essential in reducing morbidity and mortality. The cutaneous manifestations can be divided into two main categories: nonspecific and specific. The nonspecific manifestations are commonly seen and include skin color changes, xerosis, half-and-half nails, and pruritus. The specific disorders include acquired perforating dermatosis, bullous dermatoses, metastatic calcification, and nephrogenic systemic fibrosis. This review article describes these conditions and considers the underlying pathophysiology, clinical presentations, diagnosis, and treatment options. PMID:24115194

  11. Clinical course of acute atrial fibrillation treated with rapid digitalization.

    PubMed

    Weiner, P; Bassan, M M; Jarchovsky, J; Iusim, S; Plavnick, L

    1983-02-01

    Forty-seven episodes of acute atrial fibrillation (AF) in 45 patients were examined prospectively to determine the course of the disorder treated with rapid digitalization. Patients received 1.5 mg of digoxin intravenously over 12 hours. In 40 of the 47 attacks, reversion to sinus rhythm occurred with no additional therapy at 1 to 96 hours (median 4 hours) after beginning digoxin. In thirty-two patients, conversion occurred within 8 hours; only one patient showed important ventricular slowing before conversion. Thus, if digoxin facilitates conversion, it does not do so by slowing the ventricular response. Of the 11 patients still in AF at 16 hours, conversion subsequently occurred in only four who were receiving digoxin alone. We conclude that the prognosis for quick reversion to sinus rhythm in patients with acute AF treated with rapid digitalization alone is excellent. If reversion does not occur by 16 to 24 hours, additional measures to restore sinus rhythm are indicated.

  12. Antimicrobial therapy of acute diarrhoea: a clinical review.

    PubMed

    Lübbert, Christoph

    2016-01-01

    Diarrhoea is one of the most commonly occurring diseases. This article presents a review of the current state of the treatment of acute infectious diarrhoea, as well as of the most important pathogens. The general principles of the therapy of diarrhoea are exemplified, followed by a description of the targeted antimicrobial therapy of the most important bacterial gastrointestinal infections, including salmonellosis, shigellosis and Campylobacter infections, as well as infections with pathogenic Escherichia coli strains, yersiniosis and cholera. Diarrhoea caused by toxigenic Clostridium difficile strains has increased in incidence and in severity. These infections will therefore be described in detail, including important new aspects of treatment. Symptomatic therapy is still the most important component of the treatment of infectious diarrhoea. However, empirical antibiotic therapy should be considered for severely ill patients with a high frequency of stools, fever, bloody diarrhoea, underlying immune deficiency, advanced age or significant comorbidities. Increasing resistance, in particular against fluoroquinolones, must be taken into consideration. Therapy with motility inhibitors is not recommended for Shiga toxin-producing Escherichia coli (STEC) infections, Clostridium difficile infections (CDI), and severe colitis. The macrocyclic antibiotic fidaxomicin can reduce the rate of recurrent disease in CDI. Furthermore, evidence for the benefits of faecal microbiota transplantation as a treatment option for multiple recurrences of CDI is increasing. In conclusion, the treatment of acute diarrhoea is still primarily supportive. General empirical antibiotic therapy for acute diarrhoea is not evidence-based.

  13. Clinical Efficacy of Electroneurography in Acute Facial Paralysis.

    PubMed

    Lee, Dong-Hee

    2016-04-01

    The estimated incidence of acute facial paralysis is approximately 30 patients per 100000 populations annually. Facial paralysis is an extremely frightening situation and gives extreme stress to patients because obvious disfiguring face may cause significant functional, aesthetic, and psychological disturbances. For stressful patients with acute facial paralysis, it is very important for clinicians to answer the questions like whether or not their facial function will return to normal, how much of their facial function will be recovered, and how long this is going to take. It is also important for clinicians to treat the psychological aspects by adequately explaining the prognosis, in addition to providing the appropriate medical treatment. For decades, clinicians have used various electrophysiologic tests, including the nerve excitability test, the maximal stimulation test, electroneurography, and electromyography. In particular, electroneurography is the only objective measure that is useful in early stage of acute facial paralysis. In this review article, we first discuss the pathophysiology of injured peripheral nerve. And then, we describe about various electrophysiologic tests and discuss the electroneurography extensively.

  14. Clinical Efficacy of Electroneurography in Acute Facial Paralysis

    PubMed Central

    2016-01-01

    The estimated incidence of acute facial paralysis is approximately 30 patients per 100000 populations annually. Facial paralysis is an extremely frightening situation and gives extreme stress to patients because obvious disfiguring face may cause significant functional, aesthetic, and psychological disturbances. For stressful patients with acute facial paralysis, it is very important for clinicians to answer the questions like whether or not their facial function will return to normal, how much of their facial function will be recovered, and how long this is going to take. It is also important for clinicians to treat the psychological aspects by adequately explaining the prognosis, in addition to providing the appropriate medical treatment. For decades, clinicians have used various electrophysiologic tests, including the nerve excitability test, the maximal stimulation test, electroneurography, and electromyography. In particular, electroneurography is the only objective measure that is useful in early stage of acute facial paralysis. In this review article, we first discuss the pathophysiology of injured peripheral nerve. And then, we describe about various electrophysiologic tests and discuss the electroneurography extensively. PMID:27144227

  15. Epidemiology, clinical manifestations, and long-term outcomes of a major outbreak of chikungunya in a hamlet in sri lanka, in 2007: a longitudinal cohort study.

    PubMed

    Kularatne, Senanayake A M; Weerasinghe, Sajitha C; Gihan, Champika; Wickramasinghe, Sujantha; Dharmarathne, Samath; Abeyrathna, Asanka; Jayalath, Thilak

    2012-01-01

    Chikungunya outbreaks occurred in the central province, Sri Lanka in 2006. This community-based study reports the epidemiology and the natural history of the infection from an affected village. Of the 199 families and 1001 individuals in the village, 159 (80%) and 513 (51%) were affected, respectively, comprising 237 (46%) males with peak incidence at 40-50 years. The acute illness caused polyarthritis in 233 (46%), and of them 230 (98%) progressed to chronic arthritic disability (CAD). Of the CAD patients, 102 (44%) had recovered in 141 days (range 30-210 days) from the disability state whilst 128 (56%) had persisting disability which lasted 12, 24, and 36 months in 41 (17.8%), 22 (9.5%), and 14 (6.1%) individuals, respectively. Carpal tunnel syndrome (CTS) manifested in 110 (21%). Females showed preponderance for complications over males: acute arthritis 147 versus 86, P = 0.001; CAD 136 versus 84, P = 0.029; CTS 88 versus 22, P = 0.001; relapses 105 versus 68, P = 0.001. Chikungunya was highly communicable and caused lasting crippling complications.

  16. Cyclosporin A acute encephalopathy and seizure syndrome in childhood: clinical features and risk of seizure recurrence.

    PubMed

    Gleeson, J G; duPlessis, A J; Barnes, P D; Riviello, J J

    1998-07-01

    Cyclosporin A is associated with an acute encephalopathy including seizures and alterations in mental status, herein referred to as cyclosporin A acute encephalopathy and seizure syndrome. The clinical history, electroencephalogram (EEG), and neuroimaging findings in 19 children with cyclosporin A acute encephalopathy and seizure syndrome over a 10-year period were reviewed in order to delineate clinical characteristics, imaging features, and to determine the risk of seizure recurrence in this population. All 19 had motor seizures associated with other features of cortical and subcortical dysfunction. The acute mean cyclosporin A level was 342 microg/L, but was within the "therapeutic" range in five cases. Brain imaging by computed tomography (CT) or magnetic resonance imaging (MRI) in the acute or subacute phase revealed lesions characteristic of cyclosporin A toxicity in 14 cases. Acute EEG abnormalities were present in all and included epileptiform discharges or focal slowing. Patients were followed for a median of 49 months (1-9 years). Follow-up imaging (n = 10) showed lesion resolution or improvement in the majority while EEG (n = 10) had normalized in only three. Seizures recurred in six patients and only in those with persistent EEG or imaging abnormalities. No patient had a second episode of cyclosporin A associated neurotoxicity or seizure. It appears that a significant risk of seizure recurrence exists following cyclosporin A acute encephalopathy and seizure syndrome and primarily in those children with persistent EEG or imaging abnormalities.

  17. Markedly Elevated Cardiac Bio-Markers at Presentation With Normal Ventricular Function: A Novel Clinical Subset of Myocarditis Manifestation

    PubMed Central

    Palla, Amruth R; Sontineni, Siva; Mani, Susan

    2011-01-01

    We present a case of a 19-year-old woman with myocarditis who had significantly elevated cardiac markers at presentation even before any myocardial damage ensued. The patient had complicated clinical course with ventricular arrhythmia and cardiac arrest requiring resuscitation but eventually recovered completely. Though there is limited information available regarding such cases, the significantly elevated initial cardiac markers in the absence of left ventricular decompensation may probably represent a clinical subset of myocarditis and may portend an impending complicated clinical course. Further systematic research is required to define the clinical phenotype and elucidate underlying mechanisms.

  18. Acute left-sided colonic diverticulitis: clinical expressions, therapeutic insights, and role of computed tomography

    PubMed Central

    Ambrosetti, Patrick

    2016-01-01

    The diagnostic approach of patients with suspected acute diverticulitis remains debated. On the one hand, a scoring system with the best predictive value in diagnosing acute diverticulitis has been developed in order to reduce the use of computed tomography (CT) scan, while, on the other hand, patients with a high probability of acute diverticulitis should benefit from CT scan from a clinical viewpoint, ensuring that they will receive the most appropriate treatment. The place and classification of CT scan for acute diverticulitis need to be reassessed. If the management of uncomplicated acute diverticulitis, abscess, and fecal peritonitis is now well codified, urgent surgical or medical treatment of hemodynamically stable patients presenting with intraperitoneal air or fluid without uncontrolled sepsis is still under discussion. Furthermore, the indications for laparoscopic lavage are not yet well established. It is known for years that episode(s) of acute uncomplicated diverticulitis may induce painful recurrent bowel symptoms, known as symptomatic uncomplicated diverticular disease and irritable bowel syndrome-like diverticular disease. These two clinical expressions of diverticular disease, that may darken quality of life, are treated medically aimed at symptom relief. The possible place of surgery should be discussed. Clinical and CT scan classifications should be separated entities. PMID:27574459

  19. [Clinico-diagnostic evaluation of acute delirious syndromes in schizophrenia].

    PubMed

    Panteleeva, G P

    1989-01-01

    Psychopathologic and nosologic issues of acute paranoid and Kandinsky-Clerambault syndromes are discussed on the background of clinical studies of 225 schizophrenic patients with these syndromes being initial manifestations. The data on the syndromes typology, clinical value and prognosis of acute delirious disorders are presented. These are shown to be not confined to progredient schizophrenia, including its paranoid form. Rather, they can manifest a course of the disease unspecific for schizophrenia, the so-called schizophrenic reactions and phasic states thus reflecting the course of latent schizophrenia. A differentiated approach to clinical and psychopathological analysis of acute delirious syndromes in schizophrenia is essential for adequate choice of medicosocial measures and epidemiologic investigations.

  20. Research design considerations for single-dose analgesic clinical trials in acute pain: IMMPACT recommendations.

    PubMed

    Cooper, Stephen A; Desjardins, Paul J; Turk, Dennis C; Dworkin, Robert H; Katz, Nathaniel P; Kehlet, Henrik; Ballantyne, Jane C; Burke, Laurie B; Carragee, Eugene; Cowan, Penney; Croll, Scott; Dionne, Raymond A; Farrar, John T; Gilron, Ian; Gordon, Debra B; Iyengar, Smriti; Jay, Gary W; Kalso, Eija A; Kerns, Robert D; McDermott, Michael P; Raja, Srinivasa N; Rappaport, Bob A; Rauschkolb, Christine; Royal, Mike A; Segerdahl, Märta; Stauffer, Joseph W; Todd, Knox H; Vanhove, Geertrui F; Wallace, Mark S; West, Christine; White, Richard E; Wu, Christopher

    2016-02-01

    This article summarizes the results of a meeting convened by the Initiative on Methods, Measurement, and Pain Assessment in Clinical Trials (IMMPACT) on key considerations and best practices governing the design of acute pain clinical trials. We discuss the role of early phase clinical trials, including pharmacokinetic-pharmacodynamic (PK-PD) trials, and the value of including both placebo and active standards of comparison in acute pain trials. This article focuses on single-dose and short-duration trials with emphasis on the perioperative and study design factors that influence assay sensitivity. Recommendations are presented on assessment measures, study designs, and operational factors. Although most of the methodological advances have come from studies of postoperative pain after dental impaction, bunionectomy, and other surgeries, the design considerations discussed are applicable to many other acute pain studies conducted in different settings.

  1. Differential diagnosis between Crohn’s disease and intestinal tuberculosis using integrated parameters including clinical manifestations, T-SPOT, endoscopy and CT enterography

    PubMed Central

    Zhang, Tianyu; Fan, Rong; Wang, Zhengting; Hu, Shurong; Zhang, Maochen; Lin, Yun; Tang, Yonghua; Zhong, Jie

    2015-01-01

    Background: The aim of the study was to evaluate clinical manifestations, T-SPOT, endoscopy and CT enterography to differentiate Crohn’s disease (CD) from intestinal tuberculosis (ITB). Methods: 128 in patients with suspected CD and ITB were prospectively enrolled in the study. Demographic, clinical, laboratory, endoscopic and CT enterographic data were collected. After treatment for 6 months, when a definite diagnosis was reached, the differential diagnostic value of each parameter was analyzed. Multivariable logistic regression was used to analyze further, parameters of statistical significance to establish a mathematical regression equation. Receiver operating characteristic curves were plotted. Results: Clinical parameters helpful in differentiating CD from ITB included diarrhea, night sweat and perianal disease. Endoscopic parameters were useful in differentiating CD from ITB including transverse ulcers, longitudinal ulcers, rodent-like ulcers and patulous ileocecal valve. CT enterographic parameters aided the identification of the two conditions. The sensitivity, specificity, accuracy, positive predictive value and negative predictive value of a mathematical regression model established for 6 parameters of clinical endoscopy and CT enterography were 97.8%, 96.8%, 97.6%, 98.9% and 93.7% respectively, whereas those for T-SPOT were 96.8%, 91.3%, 92.7%, 78.9% and 98.8% respectively. Conclusions: T-SPOT is useful to exclude a diagnosis of ITB. Differentiating CD from ITB is a difficult clinical problem that requires a consideration of clinical, T-SPOT, endoscopic and CT enterographic parameters for accurate diagnosis. PMID:26770348

  2. [Hypokalemia-induced paraplegia secondary to acute diarrhea].

    PubMed

    Ortuño Andériz, F; Cabello Clotet, N; de Diego Gamarra, R; Salaverría Garzón, I; Vázquez Rizaldos, S

    2002-02-01

    Hypokalemia can give a variety of syntomatology but more often courses without it or with inespecific clinical manifestations. In our enviroment the etiology of hypokalemia is wide but one of the most common causes in third world countries are diarrheas. We describe a case of severe hypokalemia due to acute diarrhea which was manifested with severe neurologic symtoms but improves with conventional treatment.

  3. Health Impact of Supplying Safe Drinking Water on Patients Having Various Clinical Manifestations of Fluorosis in an Endemic Village of West Bengal

    PubMed Central

    Majumdar, Kunal K.; Sundarraj, Shunmuga N.

    2013-01-01

    Background: Excessive fluoride in drinking water causes dental, skeletal and non-skeletal fluorosis which is encountered in endemic proportions in several parts of the world. The World Health Organization (WHO) guideline value and the permissible limit of fluoride as per the Bureau of Indian Standards (BIS) is 1.5 mg/L. Studies showed that withdrawal of sources identified for fluoride, often leads to reduction of fluoride in the body fluids (re-testing urine and serum after a week or ten days) and results in the disappearance of non-skeletal fluorosis within a short duration of 10-15 days. Objective: To determine the prevalence of signs and symptoms of suspected dental, skeletal and non-skeletal fluorosis along with food habits, addictions and use of fluoride-containing toothpaste among participants taking water with fluoride concentration above permissible limit and to assess the changes in clinical manifestations of the above participants after consumption of safe drinking water with fluoride concentration below permissible limit. Materials and Methods: A longitudinal intervention study was conducted from October 2010 to December 2011 in a village selected randomly in Purulia District of West Bengal which is endemic for fluorosis. Thirty-six families with 104 family members in the above village having history of taking unsafe water containing high level of fluoride were selected for the study. The occurrence of various dental, skeletal and non-skeletal manifestations of fluorosis along with food habits, addictions and use of fluoride-containing toothpaste among the study population was assessed; the impact of taking safe water with fluoride concentration below permissible limit from a supplied community filter on these clinical manifestations was studied by follow-up examination of the above participants for six months. The data obtained is compared with the collected data from the baseline survey. Results: The prevalence of signs and symptoms of dental, skeletal

  4. A Clinical Study of Acute Kidney Injury in Tropical Acute Febrile Illness

    PubMed Central

    Bhat, Ajay; Prabhu, Mangalore Venkatraya

    2016-01-01

    Introduction Tropical Acute Febrile Illness (TAFI) is one of the most common causes of morbidity within the community. Acute Kidney Injury (AKI) due to infective and non infective causes is a major complication. Presence of AKI is a major cause of mortality among patients with TAFI. Aim To study the spectrum of tropical acute febrile illness; the proportion, spectrum and staging of acute kidney injury; Renal Replacement Therapy (RRT) initiation and in-hospital mortality. Materials and Methods A total of 600 TAFI patients were prospectively studied at a tertiary care centre in coastal Karnataka between September 2012 and September 2014 for the aetiology of TAFI; the development and staging of AKI based on Kidney disease: Improving global outcomes (KDIGO) guidelines; the initiation of RRT and in-hospital mortality. Statistical Analysis: Data analysis was done using SPSS version 17.0 with statistical significance calculated using chi-square and Fisher’s exact t-test for which p-value <0.05 was considered significant. Results The spectrum of TAFI, in decreasing order, was vivax malaria, leptospirosis, dengue fever, falciparum malaria, mixed malaria, enteric fever, scrub typhus and the most common aetiology was malaria. The proportion of AKI was 54%. The most common cause of AKI, its stages 2 and 3, RRT initiation and in-hospital mortality was leptospirosis; and AKI stage 1 was dengue fever. KDIGO AKI stage 1, 2 and 3 was seen in 46.9%, 31.2% and 21.9% of AKI patients, respectively. RRT initiation was required in 10.2% of AKI patients and in-hospital mortality was 3% among all patients. AKI, RRT initiationand in-hospital mortality were significantly associated with older age, fever duration and other presenting complaints, examination findings, renal function and other parameters, leptospirosis, dengue fever, falciparum malaria. Conclusion The aetiology in about half of TAFI patients in coastal Karnataka was malaria. More than 50% develop AKI with greater than one

  5. Acute cyanide poisoning: clinical spectrum, diagnosis, and treatment.

    PubMed

    Borron, S W; Baud, F J

    1996-09-01

    Cyanide poisoning presents in many forms. Industrial intoxications occur due to extensive use of cyanide compounds as reaction products. Smoke inhalation, a polyintoxication, is most often responsible for domestic cyanide poisonings. Suicidal poisonings are rare. Cyanogenic compounds may produce acute or subacute toxicity. Signs of cyanide poisoning include headache, vertigo, agitation, confusion, coma, convulsions and death. Definitive laboratory confirmation is generally delayed. Elevated plasma lactate, associated with cardiovascular collapse, should suggest cyanide intoxication. Immediate treatment includes 100% oxygen, assisted ventilation, decontamination, correction of acidosis and blood pressure support. Antidotes include oxygen, hydroxocobalamin, di-cobalt EDTA and methaemoglobin-inducers. Hydroxocobalamin is an attractive antidote due to its rapid cyanide binding and its lack of serious side-effects, even in the absence of cyanide intoxication. Sodium thiosulphate acts more slowly than other antidotes and is indicated in subacute cyanogen poisoning and as an adjunct to acute cyanide poisoning. Initial evaluation of antidotal efficacy is based on correction of hypotension and lactic acidosis; the final analysis rests on the degree of permanent central nervous system injury.

  6. Clinical manifestations and case management of Ebola haemorrhagic fever caused by a newly identified virus strain, Bundibugyo, Uganda, 2007-2008.

    PubMed

    Roddy, Paul; Howard, Natasha; Van Kerkhove, Maria D; Lutwama, Julius; Wamala, Joseph; Yoti, Zabulon; Colebunders, Robert; Palma, Pedro Pablo; Sterk, Esther; Jeffs, Benjamin; Van Herp, Michel; Borchert, Matthias

    2012-01-01

    A confirmed Ebola haemorrhagic fever (EHF) outbreak in Bundibugyo, Uganda, November 2007-February 2008, was caused by a putative new species (Bundibugyo ebolavirus). It included 93 putative cases, 56 laboratory-confirmed cases, and 37 deaths (CFR = 25%). Study objectives are to describe clinical manifestations and case management for 26 hospitalised laboratory-confirmed EHF patients. Clinical findings are congruous with previously reported EHF infections. The most frequently experienced symptoms were non-bloody diarrhoea (81%), severe headache (81%), and asthenia (77%). Seven patients reported or were observed with haemorrhagic symptoms, six of whom died. Ebola care remains difficult due to the resource-poor setting of outbreaks and the infection-control procedures required. However, quality data collection is essential to evaluate case definitions and therapeutic interventions, and needs improvement in future epidemics. Organizations usually involved in EHF case management have a particular responsibility in this respect.

  7. Serologically proven acute rubella infection in patients with clinical diagnosis of dengue.

    PubMed Central

    Bustos, J.; Hamdan, A.; Loroño, M. A.; Montero, M. T.; Gómez, B.

    1990-01-01

    Patients with a clinical diagnosis of dengue but negative by serological testing were studied for rubella infection. Paired sera were obtained from 69 patients during an outbreak in Yucatán, México. The presence of specific anti-viral IgM in the acute sera was considered as diagnostic for rubella infection. The immunoglobulin was determined by measuring the difference in the inhibition of hemagglutination between the non-reduced and the reduced fractionated sera. Immunoglobulins were separated by sucrose density centrifugation. Acute rubella infection was found in 7 (10.1%) of the patients. These results demonstrate active rubella infection in patients clinically diagnosed as dengue. PMID:2323361

  8. Drugs under preclinical and clinical study for treatment of acute and chronic lymphoblastic leukemia

    PubMed Central

    Jacob, Joe Antony; Salmani, Jumah Masoud Mohammad; Chen, Baoan

    2016-01-01

    Targeted therapy has modernized the treatment of both chronic and acute lymphoblastic leukemia. The introduction of monoclonal antibodies and combinational drugs has increased the survival rate of patients. Preclinical studies with various agents have resulted in positive outputs with Phase III trial drugs and monoclonal antibodies entering clinical trials. Most of the monoclonal antibodies target the CD20 and CD22 receptors. This has led to the approval of a few of these drugs by the US Food and Drug Administration. This review focuses on the drugs under preclinical and clinical study in the ongoing efforts for treatment of acute and chronic lymphoblastic leukemia. PMID:27382259

  9. Clinical and echocardiographic findings of patients with suspected acute pulmonary thromboembolism who underwent computed tomography pulmonary angiography

    PubMed Central

    Adibi, Atoosa; Nouri, Shadi; Moradi, Maryam; Shahabi, Javad

    2016-01-01

    Background: The aim of the study was to determine the correlation between clinical and echocardiographic findings and risk factors of patients with suspected acute pulmonary thromboembolism (PTE) who underwent computed tomography pulmonary angiography (CTPA). Materials and Methods: In this cross-sectional study, 310 hospitalized patients aged >18 years with high clinical suspicion of PTE referred to imaging center of our hospital from different wards for CTPA were enrolled. The frequency of different clinical presentations, risk factors, items of Wells’ criteria, and echocardiographic findings was compared in patients with and without PTE, which have been diagnosed according to the CTPA results. Results: PTE was diagnosed in 53 (17.1%) of patients with suspected PTE. From clinical manifestations, tachypnea, pleuritic chest pain, and edema of lower extremities were significantly more frequent among patients with PTE (P < 0.05). Major surgery was the risk factor which was significantly more prevalent among patients with PTE (P < 0.05). Frequency of all criteria of Wells’ criteria, except hemoptysis, was significantly higher in patients with PTE (P < 0.05). The frequency of all studied echocardiographic variables was significantly higher in patients with PTE (P < 0.05). Conclusion: It is suggested that we could use the results of this study for utilizing the diagnostic process of PTE in patients with highly clinical suspicion of PTE and providing more validated decision. Using the results of this study, we could identify high-risk patients and made appropriate risk assessment for better management of patients with suspected PTE as well as reduce the rate of unnecessary CTPA and its related adverse consequences.

  10. Biliary tract and pancreatic surgery complicated by acute pancreatitis: a clinical analysis

    PubMed Central

    Sun, Chenggang; Li, Xin; Sun, Jintang; Zou, Peng; Gao, Shubo; Zhang, Peixun

    2015-01-01

    Objective: To study the clinical treatment features of biliary tract and pancreatic surgery complicated by acute pancreatitis. Methods: A retrospective analysis of 21 cases of biliary tract and pancreatic surgery complicated by acute pancreatitis in the Department of General Surgery in our hospital during May 2005 to July 2011 was performed; the clinical treatment features were analyzed in terms of surgical option, onset interval of acute pancreatitis after last surgery, length of stay in hospital and Ranson score. Results: There was no statistic difference between the two groups (A: The onset interval of acute pancreatitis after last surgery < 0.5 year. B: The onset interval of acute pancreatitis after last surgery > 0.5 year) in pathogenetic condition and length of stay in hospital. All patients were discharged after treatment, a follow-up of 6-18 months found no recurrence of pancreatitis. Conclusion: There is no relevance between the treatment feature and onset interval of biliary and pancreatic surgery complicated by acute pancreatitis. The disease is still treated meanly with symptomatic and supportive treatment, while the etiological treatment is also particularly important. PMID:26131243

  11. Future prospects of therapeutic clinical trials in acute myeloid leukemia.

    PubMed

    Khan, Maliha; Mansoor, Armaghan-E-Rehman; Kadia, Tapan M

    2017-03-01

    Acute myeloid leukemia (AML) is a markedly heterogeneous hematological malignancy that is most commonly seen in elderly adults. The response to current therapies to AML is quite variable, and very few new drugs have been recently approved for use in AML. This review aims to discuss the issues with current trial design for AML therapies, including trial end points, patient enrollment, cost of drug discovery and patient heterogeneity. We also discuss the future directions in AML therapeutics, including intensification of conventional therapy and new drug delivery mechanisms; targeted agents, including epigenetic therapies, cell cycle regulators, hypomethylating agents and chimeric antigen receptor T-cell therapy; and detail of the possible agents that may be incorporated into the treatment of AML in the future.

  12. [Acute lymphoblastic leukemia of T progenitors: from biology to clinics].

    PubMed

    Genescà, Eulàlia; Ribera, Jordi; Ribera, Josep-Maria

    2015-03-09

    Acute lymphoblastic leukemia (ALL) is the most common cancer in children and the main cause of morbidity among childhood blood disorders. There are 2 subtypes according to the affected lymphoid progenitor: B-ALL and T-ALL. The T-ALL is the less common and, although historically was associated with poor prognosis in both adults and children, at present, treatment outcomes do not differ significantly between the 2 types of ALL. The T-ALL subtype is the most complex and heterogeneous at the genetic level and currently the one with less new therapeutic alternatives available. This trend is changing thanks to the remarkable progress upon understanding its biology. This review summarizes the most recent and important biological findings in T-ALL and their possible therapeutic implications.

  13. [Professor WU Xu's clinical experiences on acupuncture for acute upper abdominal pain].

    PubMed

    Wu, Xiao-Liang; Lu, Bin; Sun, Jian-Hua; Ai, Bing-Wei; Bao, Chao; Wu, Wen-Zhong; Li, Jian-Bing; Liu, Lan-Ying; Wu, Wen-Yun; Pei, Li-Xia; Zhou, Jun-Ling; Li, Yan-Cai; Qin, Shan

    2014-03-01

    The clinical experiences and proven cases of distinguished doctor of TCM, professor WU Xu, on acupuncture for acute upper abdominal pain is introduced. Professor WU's manipulation characteristics of acupuncture for acute upper abdominal pain, including acute cholecystitis, kidney stone, acute stomach pain, are one-hand shape but both hands in nature, moving like Tai Chi, force on the tip of needle, movement of qi mainly. The main technique posture is one-hand holding needle with middle finger for pressing, the needle is hold by thumb and index finger, and is assisted by middle finger. The special acupuncture experience of emergency is treatment according to syndrome differentiation, combination of acupuncture and moxibustion, selecting acupoint based on experience, blood-letting acupuncture therapy and so on.

  14. Acute pain management in morbid obesity - an evidence based clinical update.

    PubMed

    Budiansky, Adele Sandra; Margarson, Michael P; Eipe, Naveen

    2017-03-01

    Increasing numbers of patients with morbid obesity are presenting for surgery and their acute pain management requires an evidence-based clinical update. The objective of this study was to complete a literature review for acute pain management in morbid obesity and provide an evidence-based clinical update with recommendations. Using standardized search terms, in March 2015, we completed a literature search to determine evidence for different acute pain pharmacological modalities in morbid obesity. For each modality the highest level of evidence was ascertained and recommendations for each pharmacological modality are presented. Though overall evidence is limited to few well conducted clinical trials, mostly related to weight loss surgery, multimodal analgesia with step-wise, severity-based, opioid-sparing approach appears to improve acute pain management in morbid obesity. The perioperative use of non-opioid adjuvants appears to offer further improvements in patient safety and outcomes. Further research into standardization of pain assessments and implementation of acute pain management protocols is required.

  15. Diverse manifestations of trichloroethylene.

    PubMed Central

    McCunney, R J

    1988-01-01

    Trichloroethylene, a solvent used in a variety of industrial settings for more than 60 years, has caused adverse health effects on the central and peripheral nervous system, the skin, liver, kidney, and heart. Three men have shown relatively unusual manifestations secondary to exposure to trichloroethylene in degreasing operations in the jewelry industry. Toxic encephalopathy, hepatitis, and carpal spasm occurred among young, healthy workers. Clinical and laboratory data, including measurement of urinary trichloroacetic acid concentrations, are presented. PMID:3342194

  16. [Task analysis of clinical laboratory physician in acute hospital].

    PubMed

    Murakami, Junko

    2013-06-01

    Appropriate communications between clinical divisions and clinical laboratories are required to improve the quality of health care in hospitals. In this paper, the routine work of a clinical laboratory physician is presented. 1. In order to support attentive medical practice, we have established a consultation service system for handling questions from medical staff. The main clients are doctors and clinical laboratory technologists. 2. In order to improve the quality of infectious disease analysis, we have recommended obtaining two or more blood culture sets to achieve good sensitivity. The order rate of multiple blood culture sets increased 90% or more in 2011. 3. In order to provide appropriate blood transfusion, we intervene in inappropriate transfusion plans. 4. In order to support prompt decision making, we send E-mails to physicians regarding critical values. 5. We send reports on the morphology of cells(peripheral blood and bone marrow), IEP, flow cytometry, irregular antibodies, and so on. It has been realized that doctors want to know better solutions immediately rather than the best solution tomorrow morning. We would like to contribute to improving the quality of health care in Saitama Cooperative Hospital as clinical laboratory physicians.

  17. [Effect of 3-oxypyridine and succinic acid derivatives on clinical manifestations of inflammatory diseases of the uterus and its appendages].

    PubMed

    Volchegorskiĭ, I A; Pravdin, E V; Uzlova, T V

    2013-01-01

    Two-week-long, prospective, placebo-controlled, single-blind randomized study of the effects of intravenous emoxipin (150 mg daily), reamberin (400 ml daily) and mexidol (300 mg daily) infusion on the dynamics of clinical symptoms of non-complicated recrudescence of inflammatory diseases of the uterus and its appendages (IDUA) in comparison to the standard therapy was performed. It was established that emoxipin, reamberin and mexidol administration appreciably improved the clinical state dynamics of women with recrudescence of IDUA. The effect was associated with more pronounced decrease in severity of genital and abdominal symptoms versus standard therapy results.

  18. Acute hepatitis E presenting with clinical feature of autoimmune hepatitis

    PubMed Central

    Patel, Ishan; Companioni, Rafael Ching; Bansal, Raghav; Vyas, Neil; Catalano, Carmine; Aron, Joshua; Walfish, Aaron

    2016-01-01

    A 32-year-old immigrant man presented with new onset jaundice. His past medical history was significant for type 2 diabetes mellitus, hypertension, and hyperlipidemia. His initial laboratory finding and liver biopsy were suggestive of autoimmune hepatitis (AIH). The plan was to start steroids pending negative results for viral serology, but it came back positive for hepatitis E virus. The patient's liver function test and clinical condition improved significantly on conservative management over a period of 1 month. Therefore, we suggest testing for hepatitis E especially in immigrants or recent travelers to endemic areas who presents with clinical features suggestive of AIH. PMID:27987286

  19. Ocular manifestations of feline herpesvirus.

    PubMed

    Andrew, S E

    2001-03-01

    Feline herpesvirus-1 (FHV-1) infection is ubiquitous in the domestic cat population worldwide. The most common clinical ocular manifestations of infection with FHV-1 are conjunctivitis and keratitis. This paper reviews the pathogenesis of feline herpesvirus-1 and discusses the various clinical ocular manifestations, diagnostic techniques and treatment of FHV-1-induced diseases. Ocular manifestations include: conjunctivitis, keratitis, stromal keratitis, keratoconjunctivitis sicca, ophthalmia neonatorium, symblepharon, corneal sequestrum, eosinophilic keratitis and anterior uveitis. Diagnostic techniques discussed include: virus isolation, fluorescent antibody testing, serum neutralising titers, ELISA and polymerase chain reaction. Various therapies are also discussed.

  20. Sickle cell anemia in Guadeloupean children: pattern and prevalence of acute clinical events.

    PubMed

    Tarer, Vanessa; Etienne-Julan, Maryse; Diara, Jean-Pierre; Belloy, Marie Sylvaine; Mukizi-Mukaza, Martin; Elion, Jacques; Romana, Marc

    2006-03-01

    We analyzed the records of 153 Guadeloupean children with sickle cell anemia (SCA), for whom clinical and laboratory data were prospectively collected (mean follow-up duration 8.4 +/- 4.6 yr). Prevalence and age-specific frequencies of acute clinical events were determined and correlations between complications, hematological parameters and potential modulating factors investigated. Painful crisis and acute chest syndrome (ACS) were the two most common complications, affecting 65.4% and 58.8% of the patients, respectively. The frequency of acute anemia was 49.7% (acute splenic sequestration 24.8%; acute aplastic anemia 15.0%). Prevalences of septicemia-meningitis and osteomyelitis were 15.7% and 16.3%, respectively. A higher incidence of infections, painful crises and acute anemia was detected in patients who developed ACS. The well-documented protective effect of HbF level on the overall disease expression was observed with higher HbF level in asymptomatic than in symptomatic patients (17.5% +/- 8% vs. 9.9% +/- 6.4%, P = 0.01) with similar ages and sex ratio. It was also confirmed on ACS and, for the first time, further extended to acute anemic events and septicemia. Besides its effect on hematological parameters, alpha-thalassemia seems to have little impact on the prevalence of complications, as do beta(S)-globin haplotypes. Comparison with other series suggests that the natural history of SCA in Guadeloupe is more similar to that in Jamaica with regard to those reported in Europe and the United States, suggesting a potential impact of environmental factors on the clinical course of the disease.

  1. Clinical Course, Radiological Manifestations, and Outcome of Pneumocystis jirovecii Pneumonia in HIV Patients and Renal Transplant Recipients

    PubMed Central

    Rauch, Andri; Furrer, Hansjakob; Cusini, Alexia; Meyer, Andreas M. J.; Weiler, Stefan; Huynh-Do, Uyen; Heverhagen, Johannes; Arampatzis, Spyridon; Christe, Andreas

    2016-01-01

    Background Pneumocystis jirovecii pneumonia (PCP) is a frequent opportunistic infection in immunocompromised patients. In literature, presentation and outcome of PCP differs between patients with human immunodeficiency virus (HIV) infection and renal transplant recipients (RTRs). Methods We conducted a cross-sectional study of patients with PCP based on the HIV and renal transplant registries at our institution. Radiological and clinical data from all confirmed PCP cases between 2005 and 2012 were compared. Results Forty patients were included: 16 with HIV and 24 RTRs. Radiologically, HIV patients had significantly more areas of diffuse lung affection (81% HIV vs. 25% RTR; p = 0.02), more ground glass nodules 5–10 mm (69% vs. 4%; p = <0.001) and enlarged hilar lymph nodes were found only in HIV patients (44%). Cough and dyspnea were the most common clinical signs (>80%) in both groups. Duration from illness onset to hospital presentation was longer in the HIV patients (median of 18 vs. 10 days (p = 0.02)), implying a less fulminant clinical course. Sixty percent of PCP cases in RTRs occurred >12 months after transplantation. Lengths of hospitalization, admission rates to the intensive care unit, and requirements for mechanical ventilation were similar. Outcome in both groups was favourable. Conclusions While important differences in radiological presentation of PCP between HIV patients and RTRs were found, clinical presentation was similar. PCP only rarely presented with fulminant respiratory symptoms requiring ICU admission, with similar results and outcomes for HIV patients and RTRs. Early diagnosis and treatment is mandatory for clinical success. PMID:27824870

  2. Recurrent acute obstructive hydrocephalus as clinical onset of cerebral Whipple's disease.

    PubMed

    Seneca, Vincenzo; Imperato, Alessia; Colella, Giuseppe; Cioffi, Valentina; Mariniello, Giuseppe; Gangemi, Michelangelo

    2010-10-01

    Whipple's disease is a rare multisystemic infection caused by the intracellular bacteria Thropheryma whippelii. Central nervous system (CNS) involvement is not rare. The most frequent CNS manifestations are cognitive and behavioural changes, sopranuclear ophtalmoplegia, myoclonus, epilepsy, ataxia, meningitis and focal cerebral palsy. We report one case of cerebral localization of Whipple's disease with a clinical presentation of recurrent endocranic hypertension and hydrocephalus, and uncommon neurological symptoms, successfully treated by endoscopic third ventriculostomy and antibiotic therapy with ceftriaxone and Trimethoprim-Sulfamethoxazole.

  3. Efficacy of egg yolk and nitroglycerin ointment as treatments for acute anal fissures: A randomized clinical trial study

    PubMed Central

    Salari, Masoumeh; Salari, Roshanak; Dadgarmoghadam, Maliheh; Khadem-Rezaiyan, Majid; Hosseini, Mousalreza

    2016-01-01

    Background Acute anal fissure as a common disease in society has several etiologies and manifestations such as severe anal pain and bleeding. Nitroglycerin ointment 0.2% is the most common topical treatment used. The most common side effect of nitroglycerin is headache, which is annoying for patients and often leads to discontinuation of the drug. Objective Comparison of egg yolk as a natural substance with analgesic and anti-inflammatory properties and minimal side effects with nitroglycerin ointment in the treatment of acute anal fissure. Methods This randomized clinical trial was carried out during a 10-day period in the Gastroenterology clinic of Ghaem Hospital, Mashhad, Iran (year 2015). 126 patients who filled the inclusion criteria were enrolled. The patients were randomly divided into two groups. Nitroglycerin ointment (0.2%) was applied by patients in the first group, twice daily for 10 days. For the second group, one egg yolk once a day was administered rectally up to 10 days. The pain and bleeding severity were recorded every two days up to 10 days after finishing the treatment course, based on visual scale Results The results showed that egg yolk caused a significant reduction in pain and bleeding compared with nitroglycerin (p<0.05). At the beginning of the study, the difference in pain intensity between the two groups was not statistically significant (p-value = 0.25). However, it became significant in the following days. Changes in the frequency of rectorrhagia were also significant in both groups, showing a major decrease in the number of rectorrhagia cases (p<0.001). Conclusion Egg yolk is more efficient than nitroglycerin in the treatment of acute anal fissure. In addition, lack of any side effects results in the completion of the treatment course by the patients. Trial Registration The trial was registered at the Iranian Registry of Clinical Trials (http://www.irct.ir) with the Irct ID: IRCT2015050718915N3. Funding This work was supported by a

  4. Intramammary application of ozone therapy to acute clinical mastitis in dairy cows.

    PubMed

    Ogata, A; Nagahata, H

    2000-07-01

    The infusion of ozone into the inflamed quarter of cows with clinical mastitis was performed and the efficacy of ozone therapy was evaluated. Ozone was infused into the inflamed quarter via a teat canal using ozone gas generating equipment. Nineteen Holstein cows with acute clinical mastitis were divided into two groups: 15 cows treated with ozone therapy, and 4 cows treated with antibiotic therapy. Systemic and local clinical signs, California Mastitis Test scores, the mastitis causing pathogens, electronic conductivity of milk, and somatic cell counts in milk from ozone- and antibiotic-treated quarters, were compared between the groups. Sixty percent (9/15) of cows with acute clinical mastitis treated with ozone therapy, did not require any antibiotics for recovery. This newly developed ozone therapy method was proven to be effective, safe, and cost effective, and carries no risk of drug residues in milk.

  5. Imaging Manifestations of Mediastinal Fat Necrosis

    PubMed Central

    Bhatt, Malay Y.; Martínez-Jiménez, Santiago; Rosado-de-Christenson, Melissa L.; Watson, Kenneth R.; Walker, Christopher M.; Kunin, Jeffrey R.

    2013-01-01

    Mediastinal fat necrosis (MFN) or epipericardial fat necrosis, as it is commonly referred to in the literature, is a rare self-limiting cause of chest pain of unclear etiology. MFN affects previously healthy individuals who present with acute pleuritic chest pain. Characteristic computed tomography (CT) findings include a fat attenuation lesion with intrinsic and surrounding increased attenuation stranding. There is often associated thickening of the adjacent pericardium and/or pleural effusions. We present two cases of MFN manifesting as ovoid fat attenuation lesions demarcated by a soft tissue attenuation rim with intrinsic and surrounding soft tissue attenuation stranding and review the clinical and pathologic features of these lesions. Knowledge of the clinical presentation of patients with MFN and familiarity with the characteristic imaging findings of these lesions should allow radiologists to prospectively establish the correct diagnosis and suggest conservative management and follow-up. PMID:24369521

  6. [Acute life threatening catatonia--clinical significance and therapeutic possibilities].

    PubMed

    Bridler, R; Hell, D

    1997-09-13

    Malignant catatonia, associated with different somatic and psychiatric disorders, is a rare, life-threatening syndrome. Immediate recognition and adequate treatment are essential and may be life-saving. We describe a case of malignant catatonia and discuss the clinical implications. Additionally, we review the recent literature regarding epidemiology, nosology, current pathophysiological concepts, differential diagnosis, and treatment recommendations.

  7. Clinical manifestations and molecular epidemiology of five cases of diarrhoea in children associated with Vibrio metschnikovii in Arequipa, Peru.

    PubMed

    Dalsgaard, A; Alarcon, A; Lanata, C F; Jensen, T; Hansen, H J; Delgado, F; Gil, A I; Penny, M E; Taylor, D

    1996-12-01

    In April 1994, Vibrio metschnikovii was isolated from five infants with watery diarrhoea in Arequipa, Peru, as part of a passive cholera surveillance system. The children ranged in age from 11 to 20 months and had acute diarrhoea, with two cases showing moderate dehydration. Two children also had traces of blood in liquid stool. The children were seen at two different hospitals, and no evidence of a common source of infection was found. No additional V. metschnikovii isolates were identified in the remaining surveillance period that covered the rest of 1994 and 1995. However, stool samples were not screened for enteric pathogens other than vibrios. V. metschnikovii strains isolated from stool samples produced opaque and translucent colonies on agar plates, suggesting capsular material. All isolates were resistant to ampicillin, erythromycin and streptomycin. Plasmid analysis revealed a common 200-kb plasmid in isolates from all cases and an additional 2.7-kb plasmid in three of the isolates. Ribotyping of each isolate after restriction with BglI and HindIII endonucleases demonstrated identical ribotyping patterns. The cases reported suggest that V. metschnikovii may be associated with diarrhoea in man by mechanisms so far unknown.

  8. Early Clinical Implications of Microalbuminuria in Patients with Acute Ischaemic Stroke

    PubMed Central

    Pais, Christopher C.

    2016-01-01

    Introduction Cardiovascular and cerebrovascular diseases are leading causes of morbidity and mortality worldwide. Stroke accounts for the second leading cause of death, about 11.13% of total deaths worldwide. Microalbuminuria is known to be associated with increased risk of mortality in ischaemic stroke patients. But there have been no studies to assess whether microalbuminuria affects the early clinical outcome of patients with acute ischaemic stroke. Aim This study aims to investigate whether microalbuminuria affects the early clinical outcome of patients with acute ischaemic stroke. Materials and Methods This is a prospective study of patients with ischaemic stroke (who presented within 24 hours of symptom onset) who were consecutively admitted in three tertiary care centres during the time period from November 2013 to June 2015. Early clinical outcomes in patients were assessed by investigating the presence of Early Neurological Deterioration (END) using the National Institute of Health Stroke Scale. Urine albumin creatinine ratio was divided into two categories – Normal (less than 30mg/g of creatinine) or Urine Microalbuminuria (30-300 mg/g of creatinine). Results Total 42 out of 70 patients (60%) were found to have microalbuminuria. In multivariate logistic regression analysis, microalbuminuria was found to be independently associated with END in patients with acute ischaemic stroke (p=0.044). Conclusion In the early periods following acute ischaemic stroke, patients with microalbuminuria have worse clinical outcome. PMID:27790489

  9. [Pathophysiology of hormonal, immune, metabolic changes in acute and chronic pancreatitis. Experimental and clinical studies].

    PubMed

    Trubitsyna, I E; Chikunova, B Z; Tkachenko, E V; Tsaregorodtseva, T M; Vinokurova, L V; Varvanina, G G

    2008-01-01

    There is literature review of the acute and chronic pancreatitis experimental models. Patogenetic necrosis mechanisms with fibrosis progress in pancreas were revealed. The stimulation of the proteolytic enzymes synthesis and secretion, that was examined in experiments were compared with clinical examinations. The patients with chronic pancreatitis were investigated in the Central Research Institute of Gastroenterology.

  10. Abdominal pain and syndrome of inappropriate antidiuretic hormone secretion as clinical presentation of acute intermittent porphyria.

    PubMed

    Valle Feijóo, M L; Bermúdez Sanjurjo, J R; González Vázquez, L; Rey Martínez, M; de la Fuente Aguado, J

    2015-01-01

    Acute intermittent porphyria (AIP) is a rare condition characterized by abdominal pain and a wide range of nonspecific symptoms. We report the case of a woman with abdominal pain and syndrome of inappropriate antidiuretic hormone secretion (SIADH) as clinical presentation of AIP. The diagnosis was achieved through the etiologic study of the SIADH.

  11. Changes in the Neuropsychological Correlates of Clinical Dimensions between the Acute and Stable Phase of Schizophrenia

    ERIC Educational Resources Information Center

    Guillem, F.; Ganeva, E.; Pampoulova, T.; Stip, E.; Lalonde, P.; Sasseville, M.

    2005-01-01

    This study was designed to investigate whether the neuropsychological correlates of the symptom dimensions of schizophrenia vary with the clinical state in patients followed from the acute to stable the phase of the illness. Fifteen patients were assessed for symptoms (SAPS-SANS) and undergone a complete neuropsychological assessment at two…

  12. [Clinical practice guidelines on the diagnosis and treatment of infectious acute diarrhea in children Peru - 2011].

    PubMed

    Gonzales S, Carlos; Bada M, Carlos; Rojas G, Raúl; Bernaola A, Guillermo; Chávez B, Carlos

    2011-01-01

    The Clinical Practice Guidelines cover the Diagnosis and Treatment of Acute Diarrhea in Pediatric Infectious is a consice information about definition, inclusion and exclusion criteria; epidemiology and etiology of infectious diarrhea. The guidelines cover aspects of diagnosis and treatment (dehydration, antibiotics, supportive therapy), nutritional support and other aspects of transferences and prevention.

  13. 21 CFR 320.28 - Correlation of bioavailability with an acute pharmacological effect or clinical evidence.

    Code of Federal Regulations, 2013 CFR

    2013-04-01

    ... 21 Food and Drugs 5 2013-04-01 2013-04-01 false Correlation of bioavailability with an acute pharmacological effect or clinical evidence. 320.28 Section 320.28 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) DRUGS FOR HUMAN USE BIOAVAILABILITY AND...

  14. Clinical Practice Guideline of Acute Respiratory Distress Syndrome

    PubMed Central

    Cho, Young-Jae; Moon, Jae Young; Shin, Ein-Soon; Kim, Je Hyeong; Jung, Hoon; Park, So Young; Kim, Ho Cheol; Sim, Yun Su; Rhee, Chin Kook; Lim, Jaemin; Lee, Seok Jeong; Lee, Won-Yeon; Lee, Hyun Jeong; Kwak, Sang Hyun; Kang, Eun Kyeong; Chung, Kyung Soo

    2016-01-01

    There is no well-stated practical guideline for mechanically ventilated patients with or without acute respiratory distress syndrome (ARDS). We generate strong (1) and weak (2) grade of recommendations based on high (A), moderate (B) and low (C) grade in the quality of evidence. In patients with ARDS, we recommend low tidal volume ventilation (1A) and prone position if it is not contraindicated (1B) to reduce their mortality. However, we did not support high-frequency oscillatory ventilation (1B) and inhaled nitric oxide (1A) as a standard treatment. We also suggest high positive end-expiratory pressure (2B), extracorporeal membrane oxygenation as a rescue therapy (2C), and neuromuscular blockage for 48 hours after starting mechanical ventilation (2B). The application of recruitment maneuver may reduce mortality (2B), however, the use of systemic steroids cannot reduce mortality (2B). In mechanically ventilated patients, we recommend light sedation (1B) and low tidal volume even without ARDS (1B) and suggest lung protective ventilation strategy during the operation to lower the incidence of lung complications including ARDS (2B). Early tracheostomy in mechanically ventilated patients can be performed only in limited patients (2A). In conclusion, of 12 recommendations, nine were in the management of ARDS, and three for mechanically ventilated patients. PMID:27790273

  15. Cost and Utilization of Retail Clinics vs. Other Providers for Treatment of Pediatric Acute Otitis Media.

    PubMed

    Duncan, Ian; Clark, Kara; Wang, Stacy

    2016-10-01

    A common acute condition seen by providers in retails clinics is the evaluation and treatment of acute otitis media (AOM) in children younger than age 20. Annual direct treatment costs for AOM were US $5.3 billion in 1998 dollars. Based on the experience of a large retail pharmacy employer, the authors compared AOM episodes in covered dependents younger than age 20 in retail clinic states to those in states without retail clinic access. Relative costs as well as frequency of visits and antibiotic prescriptions were analyzed for both retail clinic-based, and non-retail clinic-based episodes. Rates of AOM episodes were lower in retail clinic than in non-retail clinic states (62.5 vs. 76.9 per 1000 members per year; P < .0001). The average number of visits per episode was similar between retail clinic and non-retail clinic states (1.417 vs. 1.430, respectively; P = 0.657), suggesting that retail clinics do not result in an increase in overall utilization. On a risk-adjusted basis, retail clinic episodes cost approximately $30-$130 less than community episodes, depending on year. In retail clinic states, the antibiotic prescription fill rate was 95.4% for retail clinic episodes and 82.8% for community episodes, consistent with rates in the literature. This study confirms results of earlier studies that retail clinics are a less costly setting than the community for the treatment of episodes of otitis media There also is little evidence that retail clinics lead to duplication of services (patients receiving follow-up care in other settings).

  16. Bayesian Machine Learning Techniques for revealing complex interactions among genetic and clinical factors in association with extra-intestinal Manifestations in IBD patients

    PubMed Central

    Menti, E; Lanera, C; Lorenzoni, G; Giachino, Daniela F.; Marchi, Mario De; Gregori, Dario; Berchialla, Paola

    2016-01-01

    The objective of the study is to assess the predictive performance of three different techniques as classifiers for extra-intestinal manifestations in 152 patients with Crohn’s disease. Naïve Bayes, Bayesian Additive Regression Trees and Bayesian Networks implemented using a Greedy Thick Thinning algorithm for learning dependencies among variables and EM algorithm for learning conditional probabilities associated to each variable are taken into account. Three sets of variables were considered: (i) disease characteristics: presentation, behavior and location (ii) risk factors: age, gender, smoke and familiarity and (iii) genetic polymorphisms of the NOD2, CD14, TNFA, IL12B, and IL1RN genes, whose involvement in Crohn’s disease is known or suspected. Extra-intestinal manifestations occurred in 75 patients. Bayesian Networks achieved accuracy of 82% when considering only clinical factors and 89% when considering also genetic information, outperforming the other techniques. CD14 has a small predicting capability. Adding TNFA, IL12B to the 3020insC NOD2 variant improved the accuracy. PMID:28269885

  17. Bayesian Machine Learning Techniques for revealing complex interactions among genetic and clinical factors in association with extra-intestinal Manifestations in IBD patients.

    PubMed

    Menti, E; Lanera, C; Lorenzoni, G; Giachino, Daniela F; Marchi, Mario De; Gregori, Dario; Berchialla, Paola

    2016-01-01

    The objective of the study is to assess the predictive performance of three different techniques as classifiers for extra-intestinal manifestations in 152 patients with Crohn's disease. Naïve Bayes, Bayesian Additive Regression Trees and Bayesian Networks implemented using a Greedy Thick Thinning algorithm for learning dependencies among variables and EM algorithm for learning conditional probabilities associated to each variable are taken into account. Three sets of variables were considered: (i) disease characteristics: presentation, behavior and location (ii) risk factors: age, gender, smoke and familiarity and (iii) genetic polymorphisms of the NOD2, CD14, TNFA, IL12B, and IL1RN genes, whose involvement in Crohn's disease is known or suspected. Extra-intestinal manifestations occurred in 75 patients. Bayesian Networks achieved accuracy of 82% when considering only clinical factors and 89% when considering also genetic information, outperforming the other techniques. CD14 has a small predicting capability. Adding TNFA, IL12B to the 3020insC NOD2 variant improved the accuracy.

  18. Clinical study of childhood acute disseminated encephalomyelitis, multiple sclerosis, and acute transverse myelitis in Fukuoka Prefecture, Japan.

    PubMed

    Torisu, Hiroyuki; Kira, Ryutaro; Ishizaki, Yoshito; Sanefuji, Masafumi; Yamaguchi, Yui; Yasumoto, Sawa; Murakami, Yoshihiko; Shimono, Masayuki; Nagamitsu, Shinichiro; Masuzaki, Mayumi; Amamoto, Masano; Kondo, Rikako; Uozumi, Tomohiko; Aibe, Miyuki; Gondo, Kenjiro; Hanai, Toshio; Hirose, Sinichi; Matsuishi, Toyojiro; Shirahata, Akira; Mitsudome, Akihisa; Hara, Toshiro

    2010-06-01

    Acute disseminated encephalomyelitis (ADEM) has recently been studied in several countries owing to the development and wide spread use of imaging technology, but few epidemiological studies of childhood ADEM have been undertaken in Asian countries. To perform a comprehensive survey of ADEM and related diseases in Japanese children, we conducted a multicenter, population-based study on childhood ADEM, multiple sclerosis, and acute isolated transverse myelitis in Fukuoka Prefecture, Japan. We identified 26 children with ADEM, 8 with multiple sclerosis, and 4 with acute transverse myelitis during 5 years between September 1998 and August 2003. The incidence of childhood ADEM under the age of 15 years was 0.64 per 100,000 person-years, mean age at onset was 5.7 years, and male-female ratio was 2.3:1. The prevalence of childhood multiple sclerosis was 1.3 per 100,000 persons. The mean age at onset of multiple sclerosis, 9.3 years, was significantly higher than that of ADEM. Nineteen (73%) and four (15%) patients with ADEM experienced antecedent infectious illnesses and vaccinations, respectively, within 1 month before the onset. Clinical and radiological findings of ADEM revealed that the frequency of seizures, mean white blood cell counts in cerebrospinal fluid, and the frequency of subcortical lesions in Fukuoka study, seemed to be higher than those in previous non-Asian studies. These findings suggest that there are ethnic or geographical differences in the incidence and clinical features of ADEM, and that there might be potent genetic or environmental risk factors for ADEM distinct from those for multiple sclerosis.

  19. Ophthalmic manifestations in IgG4-related disease: Clinical presentation and response to treatment in a French case-series.

    PubMed

    Ebbo, Mikael; Patient, Matthieu; Grados, Aurelie; Groh, Matthieu; Desblaches, Julien; Hachulla, Eric; Saadoun, David; Audia, Sylvain; Rigolet, Aude; Terrier, Benjamin; Perlat, Antoinette; Guillaud, Constance; Renou, Frederic; Bernit, Emmanuelle; Costedoat-Chalumeau, Nathalie; Harlé, Jean-Robert; Schleinitz, Nicolas

    2017-03-01

    IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series.Patients with IgG4-RD and orbital or orbital adnexa involvement included in the French multicentric IgG4-RD case-registry were identified. Only patients fulfilling "modified" comprehensive diagnostic criteria with pathological documentation were retained for the study. Clinical, biological, pathological, radiological findings and data regarding the response to treatment were retrospectively analyzed.According to our data registry, the frequency of IgG4-related ophthalmic disease (IgG4-ROD) was 17%. Mean age at diagnosis was 55.1 ± 7.1 years with a male/female ratio of 2.2. The 19 cases of IgG4-ROD consisted of lacrimal gland (68.4%), soft tissue (57.9%), extra-ocular muscles (36.8%), palpebral (21.1%), optical nerve (10.5%), orbital bone (10.5%), and mononeuritis (V1 and/or V2, 10.5%) involvements. IgG4-ROD was bilateral in 57.9% of cases. Extra-ophthalmic manifestations were reported in 78.9% of cases. All patients responded to prednisone but two-thirds of patients relapsed within a mean (SD) of 9.8 (3.5) months and 72.2% required long-term glucocorticoids and/or immunosuppressive agents. Eight patients were treated by rituximab with a favorable response in 87.5% of cases.Lacrimal involvement is the most frequent ophthalmic manifestation of IgG4-RD and is frequently associated with extra-orbital manifestations. Despite initial favorable response to steroids, the long-term management of relapsing patients needs to be improved.

  20. Chronic polyarthritis as isolated manifestation of toxocariasis.

    PubMed

    Viola, Gabriela R; Giacomin, Maria Fernanda A; França, Camila M P; Sallum, Adriana M E; Jacob, Cristina M A; Silva, Clovis A

    2016-01-01

    Human toxocariasis is a parasitic zoonosis mainly caused by Toxocara canis or Toxocara cati and is acquired by ingestion of the parasite's embryonated eggs. Arthralgia and/or arthritis were reported in up to 17% of the cases, generally with acute duration (less than 6 weeks). However, to our knowledge, chronic polyarthritis, as the isolated presentation of Toxocara infection, was not reported. One of the 5809 patients that was followed up at our service (0.017%) had chronic polyarthritis as the single manifestation of toxocariasis and was described herein. A 3-year-old girl was referred to our service with severe painful chronic polyarthritis for a period longer than 10 weeks and morning stiffness of 30min. Dog contact exposure history in the recreational areas of neighborhood was reported. Her exams showed high levels of eosinophils in peripheral blood (29%), bone marrow aspirate revealed marked eosinophilia (32%) and Toxocara enzyme-linked immunosorbent assay (Elisa) was positive (1:1280). She was treated with paracetamol (40mg/kg/day) and thiabendazole (25mg/kg/day) for 10 days, and all manifestations reduced. After eight months of follow-up, she was on clinical and laboratorial remission. In conclusion, we described a case of chronic polyarthritis, as isolated manifestation of toxocariasis, mimicking juvenile idiopathic arthritis and leukemia. Importantly, this zoonosis should be considered in patients with arthritis and eosinophilia.

  1. The evolution of clinical trials for infant acute lymphoblastic leukemia

    PubMed Central

    Kotecha, R S; Gottardo, N G; Kees, U R; Cole, C H

    2014-01-01

    Acute lymphoblastic leukemia (ALL) in infants has a significantly inferior outcome in comparison with older children. Despite initial improvements in survival of infants with ALL since establishment of the first pediatric cooperative group ALL trials, the poor outcome has plateaued in recent years. Historically, infants were treated on risk-adapted childhood ALL protocols. These studies were pivotal in identifying the need for infant-specific protocols, delineating prognostic categories and the requirement for a more unified approach between study groups to overcome limitations in accrual because of low incidence. This subsequently led to the development of collaborative infant-specific studies. Landmark outcomes have included the elimination of cranial radiotherapy following the discovery of intrathecal and high-dose systemic therapy as a superior and effective treatment strategy for central nervous system disease prophylaxis, with improved neurodevelopmental outcome. Universal prospective identification of independent adverse prognostic factors, including presence of a mixed lineage leukemia rearrangement and young age, has established the basis for risk stratification within current trials. The infant-specific trials have defined limits to which conventional chemotherapeutic agents can be intensified to optimize the balance between treatment efficacy and toxicity. Despite variations in therapeutic intensity, there has been no recent improvement in survival due to the equilibrium between relapse and toxicity. Ultimately, to improve the outcome for infants with ALL, key areas still to be addressed include identification and adaptation of novel prognostic markers and innovative therapies, establishing the role of hematopoietic stem cell transplantation in first complete remission, treatment strategies for relapsed/refractory disease and monitoring and timely intervention of late effects in survivors. This would be best achieved through a single unified

  2. Clinical Effects of Hypertension on the Mortality of Patients with Acute Myocardial Infarction

    PubMed Central

    Kang, Dong Goo; Ahn, Yongkeun; Chae, Shung Chull; Hur, Seung Ho; Hong, Taek Jong; Kim, Young Jo; Seong, In Whan; Chae, Jei Keon; Rhew, Jay Young; Chae, In Ho; Cho, Myeong Chan; Bae, Jang Ho; Rha, Seung Woon; Kim, Chong Jin; Jang, Yang Soo; Yoon, Junghan; Seung, Ki Bae; Park, Seung Jung

    2009-01-01

    The incidence of ischemic heart disease has been increased rapidly in Korea. However, the clinical effects of antecedent hypertension on acute myocardial infarction have not been identified. We assessed the relationship between antecedent hypertension and clinical outcomes in 7,784 patients with acute myocardial infarction in the Korea Acute Myocardial Infarction Registry during one-year follow-up. Diabetes mellitus, hyperlipidemia, cerebrovascular disease, heart failure, and peripheral artery disease were more prevalent in hypertensives (n=3,775) than nonhypertensives (n=4,009). During hospitalization, hypertensive patients suffered from acute renal failure, shock, and cerebrovascular event more frequently than in nonhypertensives. During follow-up of one-year, the incidence of major adverse cardiac events was higher in hypertensives. In multi-variate adjustment, old age, Killip class ≥III, left ventricular ejection fraction <45%, systolic blood pressure <90 mmHg on admission, post procedural TIMI flow grade ≤2, female sex, and history of hypertension were independent predictors for in-hospital mortality. However antecedent hypertension was not significantly associated with one-year mortality. Hypertension at the time of acute myocardial infarction is associated with an increased rate of in-hospital mortality. PMID:19794974

  3. Evaluation of Clinical Alvarado Scoring System and CT Criteria in the Diagnosis of Acute Appendicitis.

    PubMed

    Gunes Tatar, Idil; Yilmaz, Kerim Bora; Sahin, Alpaslan; Aydin, Hasan; Akinci, Melih; Hekimoglu, Baki

    2016-01-01

    Aim. The aim was to evaluate the clinical Alvarado scoring system and computed tomography (CT) criteria for the diagnosis of acute appendicitis. Material and Methods. 117 patients with acute abdominal pain who underwent abdominal CT were enrolled in this retrospective study. Patient demographics, clinical Alvarado scoring, CT images, and pathologic results of the patients were evaluated. Results. 39 of the 53 patients who were operated on had pathologically proven acute appendicitis. CT criteria of appendiceal diameter, presence of periappendiceal inflammation, fluid, appendicolith, and white blood cell (WBC) count were significantly correlated with the inflammation of the appendix. The best cut-off value for appendiceal diameter was 6.5 mm. The correlation between appendiceal diameter and WBC count was 80% (P = 0.01 < 0.05). The correlation between appendiceal diameter and Alvarado score was 78.7% (P = 0.01 < 0.05). Conclusion. Presence of CT criteria of appendiceal diameter above 6.5 mm, periappendiceal inflammation, fluid, and appendicolith should prompt the diagnosis of acute appendicitis. Since patients with acute appendicitis may not always show the typical signs and symptoms, CT is a helpful imaging modality for patients with relatively low Alvarado score and leukocytosis and when physical examination is confusing.

  4. Folate and vitamin B12 levels in levodopa-treated Parkinson's disease patients: their relationship to clinical manifestations, mood and cognition.

    PubMed

    Triantafyllou, Nikolaos I; Nikolaou, Chrysoula; Boufidou, Fotini; Angelopoulos, Elias; Rentzos, Michael; Kararizou, Evangelia; Evangelopoulos, Maria-Eleftheria; Vassilopoulos, Dimitrios

    2008-01-01

    We tested the hypothesis that mood, clinical manifestations and cognitive impairment of levodopa-treated Parkinson's disease (PD) patients are associated with vitamin B12 and folate deficiency. To this end, we performed this cross-sectional study by measuring serum folate and vitamin B12 blood levels in 111 consecutive PD patients. Levodopa-treated PD patients showed significantly lower serum levels of folate and vitamin B12 than neurological controls, while depressed patients had significantly lower serum folate levels as compared to non-depressed. Cognitively impaired PD patients exhibited significantly lower serum vitamin B12 levels as compared to cognitively non-impaired. In conclusion, lower folate levels were associated with depression, while lower vitamin B12 levels were associated with cognitive impairment. The effects of vitamin supplementation merit further attention and investigation.

  5. Atypical Manifestations of Cutaneous Leishmaniasis in a Region Endemic for Leishmania braziliensis: Clinical, Immunological and Parasitological Aspects

    PubMed Central

    Guimarães, Luiz Henrique; Queiroz, Adriano; Silva, Juliana A.; Silva, Silvana C.; Magalhães, Viviane; Lago, Ednaldo L.; Machado, Paulo Roberto L.; Bacellar, Olívia; Wilson, Mary E.; Beverley, Stephen M.; Carvalho, Edgar M.; Schriefer, Albert

    2016-01-01

    Background Atypical cutaneous leishmaniasis (ACL) has become progressively more frequent in Corte de Pedra, Northeast Brazil. Herein we characterize clinical presentation, antimony response, cytokine production and parasite strains prevailing in ACL. Methodology/Principal Findings Between 2005 and 2012, 51 ACL (cases) and 51 temporally matched cutaneous leishmaniasis (CL) subjects (controls) were enrolled and followed over time in Corte de Pedra. Clinical and therapeutic data were recorded for all subjects. Cytokine secretion by patients’ peripheral blood mononuclear cells (PBMC) stimulated with soluble parasite antigen in vitro, and genotypes in a 600 base-pair locus in chromosome 28 (CHR28/425451) of the infecting L. (V.) braziliensis were compared between the two groups. ACL presented significantly more lesions in head and neck, and higher rate of antimony failure than CL. Cytosine–Adenine substitutions at CHR28/425451 positions 254 and 321 were highly associated with ACL (p<0.0001). In vitro stimulated ACL PBMCs produced lower levels of IFN-γ (p = 0.0002) and TNF (p <0.0001), and higher levels of IL-10 (p = 0.0006) and IL-17 (p = 0.0008) than CL PBMCs. Conclusions/Significance ACL found in Northeast Brazil is caused by distinct genotypes of L. (V.) braziliensis and presents a cytokine profile that departs from that in classical CL patients. We think that differences in antigenic contents among parasites may be in part responsible for the variation in cytokine responses and possibly immunopathology between CL and ACL. PMID:27906988

  6. Characterization and identification of Erysipelothrix rhusiopathiae isolated from an unnatural host, a cat, with a clinical manifestation of depression.

    PubMed

    Lee, Jin Ju; Kim, Dong Hyeok; Lim, Jeong Ju; Kim, Dae Geun; Chang, Hong Hee; Lee, Hu Jang; Kim, Sang Hun; Rhee, Man Hee; Endale, Mehari; Imada, Yumiko; Kim, Ok Jin; Kim, Suk

    2011-02-01

    Erysipelothrix rhusiopathiae is pathogenic for humans, many domestic animals and wild birds, but infectious cases with clinical symptoms in cats have not been reported. E. rhusiopathiae was recovered from a 4-month Russian blue breed cat with a very poor body condition score of 1 (BCS: 1/5). The isolate was typed as serotype 2b. Mice experimentally infected with the clinical isolate of E. rhusiopathiae through subcutaneous or intraperitoneal routes survived, and the organism was recovered from the spleen and synovial and pericardial fluids. Cats experimentally inoculated with the isolate either orally or subcutaneously survived but commonly exhibited depression and emaciation together with localized erythemal lesion of the skin accompanied by purulent ocular discharge. On hematological analysis, the number of total white blood cells was high compared with that in normal cats. Histological examination revealed congestion and moderate inflammation with focal necrosis. This observation may provide insight on E. rhusiopathiae infection in cats with the possible epidemiological significance and implications as a potential source of infection to other animals and humans.

  7. Outbreak investigation identifies a single Listeria monocytogenes strain in sheep with different clinical manifestations, soil and water.

    PubMed

    Dreyer, M; Thomann, A; Böttcher, S; Frey, J; Oevermann, A

    2015-08-31

    Listeria (L.) monocytogenes causes orally acquired infections and is of major importance in ruminants. Little is known about L. monocytogenes transmission between farm environment and ruminants. In order to determine potential sources of infection, we investigated the distribution of L. monocytogenes genetic subtypes in a sheep farm during a listeriosis outbreak by applying four subtyping methods (MALDI-TOF-MS, MLST, MLVA and PFGE). L. monocytogenes was isolated from a lamb with septicemia and from the brainstem of three sheep with encephalitis. Samples from the farm environment were screened for the presence of L. monocytogenes during the listeriosis outbreak, four weeks and eight months after. L. monocytogenes was found only in soil and water tank swabs during the outbreak. Four weeks later, following thorough cleaning of the barn, as well as eight months later, L. monocytogenes was absent in environmental samples. All environmental and clinical L. monocytogenes isolates were found to be the same strain. Our results show that the outbreak involving two different clinical syndromes was caused by a single L. monocytogenes strain and that soil and water tanks were potential infection sources during this outbreak. However, silage cannot be completely ruled out as the bales fed prior to the outbreak were not available for analysis. Faeces samples were negative, suggesting that sheep did not act as amplification hosts contributing to environmental contamination. In conclusion, farm management appears to be a crucial factor for the limitation of a listeriosis outbreak.

  8. Clinical aspects of accidents resulting in acute total body irradiation

    SciTech Connect

    Cronkite, E.P.

    1988-01-01

    That the management of whole body radiation injury involves: (1) watchful waiting, (2) observation of the hematologic parameters, (3) use of antibiotics, platelet red cell and possibly granulocyte transfusions, (4) administration of hemopoietic molecular regulators of granulopoiesis, and (5) bone marrow transplantation as the last line of defense. The clinical indication for the preceding will not be discussed, since this will be a subject of later speakers in this conference. Certainly, if a radiation casualty is fortunate enough to have an identical twin, a marrow transplant may be lifesaving and certainly can do no harm to the patient, and there is little risk to the donor.

  9. Blood pressure lowering in acute phase of stroke: latest evidence and clinical implications

    PubMed Central

    Patarroyo, Sully Xiomara Fuentes

    2012-01-01

    Persistent controversy exists as to whether there are worthwhile beneficial effects of early, rapid lowering of elevated blood pressure (BP) in acute stroke. Elevated BP or ‘hypertension’ (i.e. systolic >140 mmHg) is common in stroke, especially in patients with pre-existing hypertension and large strokes, due to variable ‘autonomic stress’ and raised intracranial pressure. While positive associations between BP levels and poor outcomes are evident across a range of studies, very low BP levels and large reductions in BP have also been shown to predict death and dependence, more so for ischaemic stroke (IS) than intracerebral haemorrhage (ICH). Accumulating evidence indicates that early BP lowering can reduce haematoma expansion in ICH, but there is uncertainty over whether this translates into improved clinical outcomes, particularly since such an effect was not evident from haemostatic therapy in clinical trials. Guidelines generally recommend control of high systolic BP (>180 mmHg), but recent evidence indicates that even more modest elevation (>140 mmHg) increases risks of cerebral oedema and haemorrhagic transformation following thrombolysis in IS. Thus, any potential benefits of rapid BP lowering in acute stroke, particularly in IS, must be balanced against the potential risks of worsening cerebral ischaemia from altered autoregulation/perfusion. This paper explores current knowledge regarding the management of hypertension in acute stroke and introduces ongoing clinical trials aimed at resolving such a critical issue in the care of patients with acute stroke. PMID:23342232

  10. A Palpable Painless Axillary Mass as the Clinical Manifestation of Castleman's Disease in a Patient with Hepatitis C Disease

    PubMed Central

    Papazafiropoulou, Athanasia K.; Angelidi, Angeliki M.; Kousoulis, Antonis A.; Christofilidis, Georgios; Sagia, Chariklia; Kaftanidou, Liountmila; Manoloudaki, Kassiani; Tsavari, Aikaterini; Kranidiotis, Georgios; Kamaratos, Alexandros; Melidonis, Andreas

    2016-01-01

    Introduction. Castleman's disease (CD) is a rare lymphoproliferative disorder. CD is divided into two clinical subtypes: the most common unicentric and the less usual multicentric subtype. The majority of unicentric CD affects the mediastinum, while neck, abdomen, and axilla are less common locations. Case Presentation. Herein, we describe a rare case of unicentric CD in the right axilla in a 36-year-old white male with a medical history of hepatitis C virus infection admitted to our hospital due to palpation of a painless mass in the right axilla. Complete excision of the lesion was performed and, one year after the diagnosis, patient was free of the disease. Conclusions. Although infrequent, it is important to include CD in the differential diagnosis when evaluating axillary lymphadenopathy particularly in young patients with a low-grade inflammation process and chronic disease even in the absence of an abnormal blood picture or organomegaly. PMID:27313621

  11. A four-point clinical criteria distinguishes immune thrombocytopenia from acute lymphoblastic leukaemia.

    PubMed

    Lum, S H; How, S J; Ariffin, H; Krishnan, S

    2016-02-01

    Immune thrombocytopenia is the most common diagnosis of isolated thrombocytopenia. The dilemma encountered by paediatricians is missing diagnosis of acute leukaemia in children with isolated thrombocytopenia. We demonstrated childhood ITP could be diagnosed using a four point clinical criteria without missing a diagnosis of acute leukaemia. Hence, bone marrow examination is not necessary in children with typical features compatible with ITP prior to steroid therapy. This can encourage paediatricians to choose steroid therapy, which is cheaper and non-blood product, as first line platelet elevating therapy in children with significant haemorrhage.

  12. Demographics, Clinical Characteristics, Management, and Outcomes of Acute Heart Failure Patients: Observations from the Oman Acute Heart Failure Registry

    PubMed Central

    Panduranga, Prashanth; Sulaiman, Kadhim; Al-Zakwani, Ibrahim; Alazzawi, Aouf AbdlRahman; Abraham, Abraham; Singh, Prit Pal; Narayan, Narayan Anantha; Rajarao, Mamatha Punjee; Khdir, Mohammed Ahmed; Abdlraheem, Mohamad; Siddiqui, Aftab Ahmed; Soliman, Hisham; Elkadi, Osama Abdellatif; Bichu, Ruchir Kumar; Al Lawati, Kumayl Hasan

    2016-01-01

    Objectives We sought to describe the demographics, clinical characteristics, management and outcomes of patients in Oman with acute heart failure (AHF) as part of the Gulf aCute heArt failuRe rEgistry (CARE) project. Methods Data were analyzed from 988 consecutive patients admitted with AHF to 12 hospitals in Oman between 14 February and 14 November 2012. Results The mean age of our patients was 63±12 years. Over half (57%) were male and 95% were Omani citizens. Fifty-seven percent of patients presented with acute decompensated chronic heart failure (ADCHF) while 43% had new-onset AHF. The primary comorbid conditions were hypertension (72%), coronary artery disease (55%), and diabetes mellitus (53%). Ischemic heart disease (IHD), hypertensive heart disease, and idiopathic cardiomyopathy were the most common etiologies of AHF in Oman. The median left ventricular ejection fraction of the cohort was 36% (27–45%) with 56% of the patients having heart failure with reduced ejection fraction (< 40%). Atrial fibrillation was seen in 15% of patients. Acute coronary syndrome (ACS) and non-compliance with medications were the most common precipitating factors. At discharge, angiotensin converting enzyme inhibitors and beta-blockers were prescribed adequately, but aldosterone antagonists were under prescribed. Within 12-months follow-up, one in two patients were rehospitalized for AHF. In-hospital mortality was 7.1%, which doubled to 15.7% at three months and reached 26.4% at one-year post discharge. Conclusions Oman CARE was the first prospective multicenter registry of AHF in Oman and showed that heart failure (HF) patients present at a younger age with recurrent ADCHF and HF with reduced ejection fraction. IHD was the most common etiology of HF with a low prevalence of AHF, but a high prevalence of acute coronary syndrome and non-compliance with medications precipitating HF. A quarter of patients died at one-year follow-up even though at discharge medical therapy was

  13. The effect of vitamin D on clinical manifestations and activity of Behçet’s disease

    PubMed Central

    Demirci, Kamile; Güler, Tuba; Dörtbaş, Fulya; Kale, Ebru

    2017-01-01

    Introduction Immune mechanisms have been implemented to have a role in the pathogenesis of Behçet’s disease (BD) and vitamin D has been shown to have a regulatory role in the immune system function. Aim To evaluate the vitamin D levels of BD patients and its relationship between clinical findings and disease activity of BD. Material and methods Sixty-eight patients with BD and 70 age- and sex-matched controls were examined retrospectively. Demographic features, vitamin D levels for both groups and clinical findings, disease activity, drug usage for BD patients were examined from their medical reports. Disease activity was calculated for each patient according to Krause’s BD activity assessment. Results Mean vitamin D levels of patients and controls were 15.35 ±7.18 ng/ml and 18.44 ±5.79 ng/ml, respectively. Vitamin D levels were significantly lower in BD patients than in controls (p = 0.006). Mean vitamin D levels of active and inactive BD cases were 15.68 ±7.31 ng/ml and 15.08 ±7.17 ng/ml, respectively (p = 0.73). Disease activity of patients using and not using vitamin D was similar (p = 0.51). Conclusions Significantly lower levels of vitamin D were observed in BD patients. Our results indicate that there is no correlation between BD activity and a vitamin D level. Together with these, vitamin D replacement treatment was found to have no effect on disease activity. PMID:28261027

  14. Crohn’s disease in the elderly: Clinical presentation and manifestations from a tertiary referral center in Turkey

    PubMed Central

    Saygili, Fatih; Saygili, Saba Mukaddes; Tenlik, Ilyas; Yuksel, Mahmut; Kilic, Zeki Mesut Yalin; Ozin, Yasemin Ozderin; Kayacetin, Ertugrul

    2016-01-01

    OBJECTIVE: There is no precise consensus at present on age to define elderly patients with inflammatory bowel disease (IBD), but recently, age of more than 60 years has been widely accepted. Characteristics of IBD in the elderly are somewhat different from what is seen in younger patients. The elderly have milder disease activity, and therapeutic options are fewer because of their age and features such as comorbidities, drug interactions, and loss of organ function. There are few reports on Crohn’s disease in the elderly. Herein, first report on this topic with respect to population of this country is presented. METHODS: Characteristics of 95 patients with Crohn’s disease, who were over age 60 from 3125 patients with IBD treated in our clinic between 1996 and 2015 were analyzed. Research was performed using patient files, and outpatient clinic visits, when possible. RESULTS: Median age of the group was 66 years, and male:female ratio was 1.6. Of the total, 48.4% of the patients had colonic disease, 37.9% had ileocolonic disease, and 13.7% had small bowel disease. Data indicated that 23.1% of patients had undergone surgical procedures, which were primarily right hemicolectomy and ileotransversostomy. Disease was most often managed with mesalazine or azathioprine. It was also determined that 12.6% patients had 2 or more comorbidities, and findings indicated coronary heart disease and hypertension were most prevalent. CONCLUSION: Analysis revealed similar features in characteristics of disease compared with recent knowledge reported in the literature. This is the first report from our country to describe Crohn’s disease in the elderly population, and the number of patients is sufficient to provide general information about this group. PMID:28275749

  15. Inflammatory Manifestations of Lymphedema

    PubMed Central

    Ly, Catherine L.; Kataru, Raghu P.; Mehrara, Babak J.

    2017-01-01

    Lymphedema results from lymphatic insufficiency leading to a progressive inflammatory process that ultimately manifests as discomfort, recurrent infections, and, at times, secondary malignancy. Collectively, these morbidities contribute to an overall poor quality of life. Although there have been recent advances in microsurgical interventions, a conservative palliative approach remains the mainstay of treatment for this disabling disease. The absence of a cure is due to an incomplete understanding of the pathophysiological changes that result in lymphedema. A histological hallmark of lymphedema is inflammatory cell infiltration and recent studies with animal models and clinical biopsy specimens have suggested that this response plays a key role in the pathology of the disease. The purpose of this report is to provide an overview of the ongoing research in and the current understanding of the inflammatory manifestations of lymphedema. PMID:28106728

  16. Preconditioning with Triiodothyronine Improves the Clinical Signs and Acute Tubular Necrosis Induced by Ischemia/Reperfusion in Rats

    PubMed Central

    Ferreyra, Carla; Vargas, Félix; Rodríguez-Gómez, Isabel; Pérez-Abud, Rocío; O'Valle, Francisco; Osuna, Antonio

    2013-01-01

    Background Renal ischemia/reperfusion (I/R) injury is manifested by acute renal failure (ARF) and acute tubular necrosis (ATN). The aim of this study was to evaluate the effectiveness of preconditioning with 3, 3, 5 triiodothyronine (T3) to prevent I/R renal injury. Methodology/Principal Findings The rats were divided into four groups: sham-operated, placebo-treated (SO-P), sham-operated T3- treated (SO- T3), I/R-injured placebo-treated (IR-P), and I/R-injured T3-treated (IR- T3) groups. At 24 h before ischemia, the animals received a single dose of T3 (100 μg/kg). Renal function and plasma, urinary, and tissue variables were studied at 4, 24, and 48 h of reperfusion, including biochemical, oxidative stress, and inflammation variables, PARP-1 immunohistochemical expression, and ATN morphology. In comparison to the SO groups, the IR-P groups had higher plasma urea and creatinine levels and greater proteinuria (at all reperfusion times) and also showed: increased oxidative stress-related plasma, urinary, and tissue variables; higher plasma levels of IL6 (proinflammatory cytokine); increased glomerular and tubular nuclear PARP-1 expression; and a greater degree of ATN. The IR-T3 group showed a marked reduction in all of these variables, especially at 48 h of reperfusion. No significant differences were observed between SO-P and SO-T3 groups. Conclusions This study demonstrates that preconditioning rats with a single dose of T3 improves the clinical signs and ATN of renal I/R injury. These beneficial effects are accompanied by reductions in oxidative stress, inflammation, and renal PARP-1 expression, indicating that this sequence of factors plays an important role in the ATN induced by I/R injury. PMID:24086411

  17. Clinical and Laboratory Methods in Diagnosis of Acute Appendicitis in Children

    PubMed Central

    Groselj-Grenc, Mojca; Repše, Stane; Vidmar, Dubravka; Derganc, Metka

    2007-01-01

    Aim To compare the diagnostic accuracy of clinical examination, white blood cell and differential count, and C-reactive protein as routine tests for acute appendicitis with that of interleukin-6 (IL-6) and ultrasonography. Methods Eighty-two children were admitted to the Department of Pediatric Surgery and Intensive Care, Ljubljana, Slovenia because of suspected acute appendicitis. Among them, 49 children underwent surgery for acute appendicitis and 33 had abdominal pain but were not treated surgically and were diagnosed with non-specific abdominal pain or mesenteric lymphadenitis on sonography. Clinical signs of acute appendicitis were determined by surgeons on admission. White blood cell count and differential and serum concentrations of C-reactive protein and IL-6 were measured and abdominal ultrasonography was performed. Results Ultrasonography showed the highest diagnostic accuracy (92.9%; 95% confidence interval [CI], 84.5%-98.0%, Bayes’ theorem), followed by serum IL-6 concentration (77.6% [67.1-86.1%] receiver-operating characteristic [ROC] curve analysis), clinical signs (69.5% [59.5-79.0%] Bayes’ theorem), white blood cell count (68.4% [57.2-78.3%] ROC curve analysis), and serum C-reactive protein concentration (63.7% [52.1-74.3%] ROC curve analysis). Ultrasonography achieved also the highest specificity (95.2%) and positive (93.8%) and negative (93.3%) predictive values, whereas clinical signs showed the highest sensitivity (93.9%). Conclusion Ultrasonography was a more accurate diagnostic method than IL-6 serum concentration, laboratory marker with the highest diagnostic accuracy in our study, and hence it should be a part of the diagnostic procedure for acute appendicitis in children. PMID:17589979

  18. Plasma C-Reactive Protein and Clinical Outcomes after Acute Ischemic Stroke: A Prospective Observational Study

    PubMed Central

    Matsuo, Ryu; Ago, Tetsuro; Hata, Jun; Wakisaka, Yoshinobu; Kuroda, Junya; Kuwashiro, Takahiro; Kitazono, Takanari; Kamouchi, Masahiro

    2016-01-01

    Background and Purpose Although plasma C-reactive protein (CRP) is elevated in response to inflammation caused by brain infarction, the association of CRP with clinical outcomes after acute ischemic stroke remains uncertain. This study examined whether plasma high-sensitivity CRP (hsCRP) levels at onset were associated with clinical outcomes after acute ischemic stroke independent of conventional risk factors and acute infections after stroke. Methods We prospectively included 3653 patients with first-ever ischemic stroke who had been functionally independent and were hospitalized within 24 h of onset. Plasma hsCRP levels were measured on admission and categorized into quartiles. The association between hsCRP levels and clinical outcomes, including neurological improvement, neurological deterioration, and poor functional outcome (modified Rankin scale ≥3 at 3 months), were investigated using a logistic regression analysis. Results Higher hsCRP levels were significantly associated with unfavorable outcomes after adjusting for age, sex, baseline National Institutes of Health Stroke Scale score, stroke subtype, conventional risk factors, intravenous thrombolysis and endovascular therapy, and acute infections during hospitalization (multivariate-adjusted odds ratios [95% confidence interval] in the highest quartile versus the lowest quartile as a reference: 0.80 [0.65–0.97] for neurological improvement, 1.72 [1.26–2.34] for neurological deterioration, and 2.03 [1.55–2.67] for a poor functional outcome). These associations were unchanged after excluding patients with infectious diseases occurring during hospitalization, or those with stroke recurrence or death. These trends were similar irrespective of stroke subtypes or baseline stroke severity, but more marked in patients aged <70 years (Pheterogeneity = 0.001). Conclusions High plasma hsCRP is independently associated with unfavorable clinical outcomes after acute ischemic stroke. PMID:27258004

  19. Retrospective Study of the Epidemiology and Clinical Manifestations of Cryptococcus gattii Infections in Colombia from 1997–2011

    PubMed Central

    Lizarazo, Jairo; Escandón, Patricia; Agudelo, Clara Inés; Firacative, Carolina; Meyer, Wieland; Castañeda, Elizabeth

    2014-01-01

    Background Cryptococcosis due to Cryptococcus gattii is endemic in various parts of the world, affecting mostly immunocompetent patients. A national surveillance study of cryptococcosis, including demographical, clinical and microbiological data, has been ongoing since 1997 in Colombia, to provide insights into the epidemiology of this mycosis. Methodology/Principal Findings From 1,209 surveys analyzed between 1997–2011, 45 cases caused by C. gattii were reported (prevalence 3.7%; annual incidence 0.07 cases/million inhabitants/year). Norte de Santander had the highest incidence (0.81 cases/million/year), representing 33.3% of all cases. The male: female ratio was 3.3∶1. Mean age at diagnosis was 41±16 years. No specific risk factors were identified in 91.1% of patients. HIV infection was reported in 6.7% of patients, autoimmune disease and steroids use in 2.2%. Clinical features included headache (80.5%), nausea/vomiting (56.1%) and neurological derangements (48.8%). Chest radiographs were taken in 21 (46.7%) cases, with abnormal findings in 7 (33.3%). Cranial CT scans were obtained in 15 (33.3%) cases, with abnormalities detected in 10 (66.7%). Treatment was well documented in 30 cases, with most receiving amphotericin B. Direct sample examination was positive in 97.7% cases. Antigen detection was positive for all CSF specimens and for 75% of serum samples. C. gattii was recovered from CSF (93.3%) and respiratory specimens (6.6%). Serotype was determined in 42 isolates; 36 isolates were serotype B (85.7%), while 6 were C (14.3%). The breakdowns of molecular types were VGII (55.6%), VGIII (31.1%) and VGI (13.3%). Among 44 strains, 16 MLST sequence types (ST) were identified, 11 of them newly reported. Conclusions/Significance The results of this passive surveillance study demonstrate that cryptococcosis caused by C. gattii has a low prevalence in Colombia, with the exception of Norte de Santander. The predominance of molecular type VGII is of concern

  20. Repair of oxidative DNA damage, cell-cycle regulation and neuronal death may influence the clinical manifestation of Alzheimer's disease.

    PubMed

    Silva, Aderbal R T; Santos, Ana Cecília Feio; Farfel, Jose M; Grinberg, Lea T; Ferretti, Renata E L; Campos, Antonio Hugo Jose Froes Marques; Cunha, Isabela Werneck; Begnami, Maria Dirlei; Rocha, Rafael M; Carraro, Dirce M; de Bragança Pereira, Carlos Alberto; Jacob-Filho, Wilson; Brentani, Helena

    2014-01-01

    Alzheimer's disease (AD) is characterized by progressive cognitive decline associated with a featured neuropathology (neuritic plaques and neurofibrillary tangles). Several studies have implicated oxidative damage to DNA, DNA repair, and altered cell-cycle regulation in addition to cell death in AD post-mitotic neurons. However, there is a lack of studies that systematically assess those biological processes in patients with AD neuropathology but with no evidence of cognitive impairment. We evaluated markers of oxidative DNA damage (8-OHdG, H2AX), DNA repair (p53, BRCA1, PTEN), and cell-cycle (Cdk1, Cdk4, Cdk5, Cyclin B1, Cyclin D1, p27Kip1, phospho-Rb and E2F1) through immunohistochemistry and cell death through TUNEL in autopsy hippocampal tissue samples arrayed in a tissue microarray (TMA) composed of three groups: I) "clinical-pathological AD" (CP-AD)--subjects with neuropathological AD (Braak ≥ IV and CERAD = B or C) and clinical dementia (CDR ≥ 2, IQCODE>3.8); II) "pathological AD" (P-AD)--subjects with neuropathological AD (Braak ≥ IV and CERAD = B or C) and without cognitive impairment (CDR 0, IQCODE<3.2); and III) "normal aging" (N)--subjects without neuropathological AD (Braak ≤ II and CERAD 0 or A) and with normal cognitive function (CDR 0, IQCODE<3.2). Our results show that high levels of oxidative DNA damage are present in all groups. However, significant reductions in DNA repair and cell-cycle inhibition markers and increases in cell-cycle progression and cell death markers in subjects with CP-AD were detected when compared to both P-AD and N groups, whereas there were no significant differences in the studied markers between P-AD individuals and N subjects. This study indicates that, even in the setting of pathological AD, healthy cognition may be associated with a preserved repair to DNA damage, cell-cycle regulation, and cell death in post-mitotic neurons.

  1. Acute Undifferentiated Febrile Illness in Patients Presenting to a Tertiary Care Hospital in South India: Clinical Spectrum and Outcome

    PubMed Central

    Abhilash, Kundavaram Paul Prabhakar; Jeevan, Jonathan Arul; Mitra, Shubhanker; Paul, Nirvin; Murugan, Thimiri Palani; Rangaraj, Ajay; David, Sandeep; Hansdak, Samuel George; Prakash, John Antony Jude; Abraham, Asha Mary; Ramasami, Prakash; Sathyendra, Sowmya; Sudarsanam, Thambu David; Varghese, George M

    2016-01-01

    Background: Acute undifferentiated febrile illness (AUFI) may have similar clinical presentation, and the etiology is varied and region specific. Materials and Methods: This prospective observational study was conducted in a tertiary hospital in South India. All adult patients presenting with AUFI of 3–14 days duration were evaluated for etiology, and the differences in presentation and outcome were analyzed. Results: The study cohort included 1258 patients. A microbiological cause was identified in 82.5% of our patients. Scrub typhus was the most common cause of AUFI (35.9%) followed by dengue (30.6%), malaria (10.4%), enteric fever (3.7%), and leptospirosis (0.6%). Both scrub typhus and dengue fever peaked during the monsoon season and the cooler months, whereas no seasonality was observed with enteric fever and malaria. The mean time to presentation was longer in enteric fever (9.9 [4.7] days) and scrub typhus (8.2 [3.2] days). Bleeding manifestations were seen in 7.7% of patients, mostly associated with dengue (14%), scrub typhus (4.2%), and malaria (4.6%). The requirement of supplemental oxygen, invasive ventilation, and inotropes was higher in scrub typhus, leptospirosis, and malaria. The overall mortality rate was 3.3% and was highest with scrub typhus (4.6%) followed by dengue fever (2.3%). Significant clinical predictors of scrub typhus were breathlessness (odds ratio [OR]: 4.96; 95% confidence interval [CI]: 3.38–7.3), total whole blood cell count >10,000 cells/mm3 (OR: 2.31; 95% CI: 1.64–3.24), serum albumin <3.5 g % (OR: 2.32; 95% CI: 1.68–3.2). Overt bleeding manifestations (OR: 2.98; 95% CI: 1.84–4.84), and a platelet count of <150,000 cells/mm3 (OR: 2.09; 95% CI: 1.47–2.98) were independent predictors of dengue fever. Conclusion: The similarity in clinical presentation and diversity of etiological agents demonstrates the complexity of diagnosis and treatment of AUFI in South India. The etiological profile will be of use in the development

  2. [Main manifestations of the phenomenon of remote post-conditioning of the heart. Clinical application of post-conditioning].

    PubMed

    Maslov, L N

    It was determined that remote post-conditioning (RP) exerts infarction-limiting and antiapoptotic effects. The infarction-limiting effect of RP is associated with enhancement of autophagia of cardiomyocytes. It was determined that RP may be an effective method of preventing reperfusion contractile dysfunction of the heart. The problem of whether RP exerts an antiarrhythmic effect in cardiac ischaemia-reperfusion remains unsolved as yet. Limitation of leukocytic invasion may have direct relation to the cardioprotector effect of RP. Decreasing the level of anti-inflammatory cytokines and MCP-1 chemokine may contribute to limitation of leukocytic infiltration into the reperfusion zone. It was determined that RP provides a decrease in intensity of lipid peroxidation. Remote postconditioning prevents reperfusion damage to cardiomyocytes both in children and adults, but RP does not improve the clinical course of the postoperative period in patients with coronary bypass grafting. In children, RP improves the course of the postoperative period. What is the reason of such difference between children and adults remains unknown. It was shown that RP exerts an infarction-limiting effect in transcutaneous coronary interventions in patients with ST elevation myocardial infarction.

  3. Expression of the galectin-9-Tim-3 pathway in glioma tissues is associated with the clinical manifestations of glioma.

    PubMed

    Liu, Zengjin; Han, Huamin; He, Xin; Li, Shouwei; Wu, Chenxing; Yu, Chunjiang; Wang, Shengdian

    2016-03-01

    Glioma is known to induce local and systemic immunosuppression, which inhibits antitumor T cell responses. The galectin-9-Tim-3-pathway negatively regulates T cell pathways in the tumor immunosuppressive environment. The present study assessed the expression of Tim-3 and galectin-9 in glioma patients, and evaluated the association between the expression of Tim-3 and galectin-9 with clinical characteristics. The present study identified that Tim-3 expression was significantly increased in peripheral blood T cells of glioma patients compared with those of healthy controls, and was additionally increased on tumor-infiltrating T cells. The expression of Tim-3 on tumor-infiltrating T cells was associated with the World Health Organization (WHO) grade of glioma, but negatively correlated with the Karnofsky Performance Status score of the glioma patients. Immunohistochemical analysis revealed that the expression of galectin-9 in tumor tissues was associated with Tim-3 expression on tumor-infiltrating T cells and the WHO grade of glioma. These findings suggest that the galectin-9-Tim-3 pathway may be critical in the immunoevasion of glioma and may be a potent target for immunotherapy in glioma patients.

  4. Malfunction of Nuclease ERCC1-XPF Results in Diverse Clinical Manifestations and Causes Cockayne Syndrome, Xeroderma Pigmentosum, and Fanconi Anemia

    PubMed Central

    Kashiyama, Kazuya; Nakazawa, Yuka; Pilz, Daniela T.; Guo, Chaowan; Shimada, Mayuko; Sasaki, Kensaku; Fawcett, Heather; Wing, Jonathan F.; Lewin, Susan O.; Carr, Lucinda; Li, Tao-Sheng; Yoshiura, Koh-ichiro; Utani, Atsushi; Hirano, Akiyoshi; Yamashita, Shunichi; Greenblatt, Danielle; Nardo, Tiziana; Stefanini, Miria; McGibbon, David; Sarkany, Robert; Fassihi, Hiva; Takahashi, Yoshito; Nagayama, Yuji; Mitsutake, Norisato; Lehmann, Alan R.; Ogi, Tomoo

    2013-01-01

    Cockayne syndrome (CS) is a genetic disorder characterized by developmental abnormalities and photodermatosis resulting from the lack of transcription-coupled nucleotide excision repair, which is responsible for the removal of photodamage from actively transcribed genes. To date, all identified causative mutations for CS have been in the two known CS-associated genes, ERCC8 (CSA) and ERCC6 (CSB). For the rare combined xeroderma pigmentosum (XP) and CS phenotype, all identified mutations are in three of the XP-associated genes, ERCC3 (XPB), ERCC2 (XPD), and ERCC5 (XPG). In a previous report, we identified several CS cases who did not have mutations in any of these genes. In this paper, we describe three CS individuals deficient in ERCC1 or ERCC4 (XPF). Remarkably, one of these individuals with XP complementation group F (XP-F) had clinical features of three different DNA-repair disorders—CS, XP, and Fanconi anemia (FA). Our results, together with those from Bogliolo et al., who describe XPF alterations resulting in FA alone, indicate a multifunctional role for XPF. PMID:23623389

  5. Malfunction of nuclease ERCC1-XPF results in diverse clinical manifestations and causes Cockayne syndrome, xeroderma pigmentosum, and Fanconi anemia.

    PubMed

    Kashiyama, Kazuya; Nakazawa, Yuka; Pilz, Daniela T; Guo, Chaowan; Shimada, Mayuko; Sasaki, Kensaku; Fawcett, Heather; Wing, Jonathan F; Lewin, Susan O; Carr, Lucinda; Li, Tao-Sheng; Yoshiura, Koh-ichiro; Utani, Atsushi; Hirano, Akiyoshi; Yamashita, Shunichi; Greenblatt, Danielle; Nardo, Tiziana; Stefanini, Miria; McGibbon, David; Sarkany, Robert; Fassihi, Hiva; Takahashi, Yoshito; Nagayama, Yuji; Mitsutake, Norisato; Lehmann, Alan R; Ogi, Tomoo

    2013-05-02

    Cockayne syndrome (CS) is a genetic disorder characterized by developmental abnormalities and photodermatosis resulting from the lack of transcription-coupled nucleotide excision repair, which is responsible for the removal of photodamage from actively transcribed genes. To date, all identified causative mutations for CS have been in the two known CS-associated genes, ERCC8 (CSA) and ERCC6 (CSB). For the rare combined xeroderma pigmentosum (XP) and CS phenotype, all identified mutations are in three of the XP-associated genes, ERCC3 (XPB), ERCC2 (XPD), and ERCC5 (XPG). In a previous report, we identified several CS cases who did not have mutations in any of these genes. In this paper, we describe three CS individuals deficient in ERCC1 or ERCC4 (XPF). Remarkably, one of these individuals with XP complementation group F (XP-F) had clinical features of three different DNA-repair disorders--CS, XP, and Fanconi anemia (FA). Our results, together with those from Bogliolo et al., who describe XPF alterations resulting in FA alone, indicate a multifunctional role for XPF.

  6. Urogenital Chlamydia trachomatis Serovars in Men and Women with a Symptomatic or Asymptomatic Infection: an Association with Clinical Manifestations?

    PubMed Central

    Morré, S. A.; Rozendaal, L.; van Valkengoed, I. G. M.; Boeke, A. J. P.; van Voorst Vader, P. C.; Schirm, J.; de Blok, S.; van den Hoek, J. A. R.; van Doornum, G. J. J.; Meijer, C. J. L. M.; van den Brule, A. J. C.

    2000-01-01

    To determine whether certain Chlamydia trachomatis serovars are preferentially associated with a symptomatic or an asymptomatic course of infection, C. trachomatis serovar distributions were analyzed in symptomatically and asymptomatically infected persons. Furthermore, a possible association between C. trachomatis serovars and specific clinical symptoms was investigated. C. trachomatis-positive urine specimens from 219 asymptomatically infected men and women were obtained from population-based screening programs in Amsterdam. Two hundred twenty-one C. trachomatis-positive cervical and urethral swabs from symptomatically and asymptomatically infected men and women were obtained from several hospital-based departments. Serovars were determined using PCR-based genotyping, i.e., restriction fragment length polymorphism analysis of the nested-PCR-amplified omp1 gene. The most prevalent C. trachomatis serovars, D, E, and F, showed no association with either a symptomatic or asymptomatic course of infection. The most prominent differences found were (i) the association of serovar Ga with symptoms in men (P = 0.0027), specifically, dysuria (P < 0.0001), and (ii) detection of serovar Ia more often in asymptomatically infected people (men and women) (P = 0.035). Furthermore, in women, serovar K was associated with vaginal discharge (P = 0.002) and serovar variants were found only in women (P = 0.045). PMID:10834991

  7. Cardiovascular risk across the histological spectrum and the clinical manifestations of non-alcoholic fatty liver disease: An update.

    PubMed

    Athyros, Vasilios G; Tziomalos, Konstantinos; Katsiki, Niki; Doumas, Michael; Karagiannis, Asterios; Mikhailidis, Dimitri P

    2015-06-14

    Non-alcoholic fatty liver disease (NAFLD) is considered to be an independent cardiovascular disease (CVD) risk factor. However, simple steatosis has a benign clinical course without excess mortality. In contrast, the advanced form of NAFLD, non-alcoholic steatohepatitis (NASH) with liver fibrosis increases mortality by approximately 70%, due to an increase in CVD mortality by approximately 300%. Chronic kidney disease (CKD) may be caused by NAFLD/NASH and it substantially increases CVD risk, especially in the presence of type 2 diabetes mellitus. Moreover, CKD may trigger NAFLD/NASH deterioration in a vicious cycle. NAFLD/NASH is also related to increased arterial stiffness (AS), an independent CVD risk factor that further raises CVD risk. Diagnosis of advanced liver fibrosis (mainly by simple non-invasive tests), CKD, and increased AS should be made early in the course of NAFLD and treated appropriately. Lifestyle measures and statin treatment may help resolve NAFLD/NASH and beneficially affect the CVD risk factors mentioned above.

  8. Molecular Pathogenesis, Epidemiology, and Clinical Manifestations of Respiratory Infections Due to Bordetella pertussis and Other Bordetella Subspecies

    PubMed Central

    Mattoo, Seema; Cherry, James D.

    2005-01-01

    Bordetella respiratory infections are common in people (B. pertussis) and in animals (B. bronchiseptica). During the last two decades, much has been learned about the virulence determinants, pathogenesis, and immunity of Bordetella. Clinically, the full spectrum of disease due to B. pertussis infection is now understood, and infections in adolescents and adults are recognized as the reservoir for cyclic outbreaks of disease. DTaP vaccines, which are less reactogenic than DTP vaccines, are now in general use in many developed countries, and it is expected that the expansion of their use to adolescents and adults will have a significant impact on reducing pertussis and perhaps decrease the circulation of B. pertussis. Future studies should seek to determine the cause of the unique cough which is associated with Bordetella respiratory infections. It is also hoped that data gathered from molecular Bordetella research will lead to a new generation of DTaP vaccines which provide greater efficacy than is provided by today's vaccines. PMID:15831828

  9. Prevalence of antibodies to Yersinia enterocolitica O:3 among Norwegian military recruits: association with risk factors and clinical manifestations.

    PubMed

    Saebø, A; Kapperud, G; Lassen, J; Waage, J

    1994-12-01

    IgG antibody activity to Yersinia enterocolitica serogroup O:3 was detected in sera from 56 (7.4%) of 755 Norwegian military recruits, using an enzyme-linked immunosorbent assay. The highest prevalence was found among recruits from Oslo city (12/56, 21.4%). The recruits answered a questionnaire which covered demographic data, specific exposures, and clinical information. The following risk factors were found to be independently associated with IgG activity in logistic regression analysis: receiving drinking water from a private well (odds ratio (OR) = 3.40; p = 0.004), being a resident of Oslo city (OR = 2.99; p = 0.006), and living in eastern Norway (OR = 2.25; p = 0.015). By univariate analysis, living in an urban area was associated with IgG activity, but this factor did not independently affect risk. Present or previous contact with animals, including pigs, and travels abroad were not associated with an increased risk. Yersinia enterocolitica O:3 seropositive recruits were more likely to report previous surgery for suspected appendicitis than seronegative individuals (OR = 4.26; p = 0.0024). Among recruits with previous appendectomy, mesenteric lymphadenitis as the sole peroperative finding was more common in patients with IgG activity to Y. enterocolitica O:3 (4/7) than in seronegative patients (1/19) (p = 0.01). Recurrent diarrhea, steatorrhea or joint complaints were not associated with antibody activity.

  10. Design of clinical trials in acute kidney injury: report from an NIDDK workshop on trial methodology.

    PubMed

    Palevsky, Paul M; Molitoris, Bruce A; Okusa, Mark D; Levin, Adeera; Waikar, Sushrut S; Wald, Ron; Chertow, Glenn M; Murray, Patrick T; Parikh, Chirag R; Shaw, Andrew D; Go, Alan S; Faubel, Sarah G; Kellum, John A; Chinchilli, Vernon M; Liu, Kathleen D; Cheung, Alfred K; Weisbord, Steven D; Chawla, Lakhmir S; Kaufman, James S; Devarajan, Prasad; Toto, Robert M; Hsu, Chi-yuan; Greene, Tom; Mehta, Ravindra L; Stokes, John B; Thompson, Aliza M; Thompson, B Taylor; Westenfelder, Christof S; Tumlin, James A; Warnock, David G; Shah, Sudhir V; Xie, Yining; Duggan, Emily G; Kimmel, Paul L; Star, Robert A

    2012-05-01

    Acute kidney injury (AKI) remains a complex clinical problem associated with significant short-term morbidity and mortality and lacking effective pharmacologic interventions. Patients with AKI experience longer-term risks for progressive chronic ESRD, which diminish patients' health-related quality of life and create a larger burden on the healthcare system. Although experimental models have yielded numerous promising agents, translation into clinical practice has been unsuccessful, possibly because of issues in clinical trial design, such as delayed drug administration, masking of therapeutic benefit by adverse events, and inadequate sample size. To address issues of clinical trial design, the National Institute of Diabetes and Digestive and Kidney Diseases sponsored a workshop titled "Clinical Trials in Acute Kidney Injury: Current Opportunities and Barriers" in December 2010. Workshop participants included representatives from academia, industry, and government agencies whose areas of expertise spanned basic science, clinical nephrology, critical care medicine, biostatistics, pharmacology, and drug development. This document summarizes the discussions of collaborative workgroups that addressed issues related to patient selection, study endpoints, the role of novel biomarkers, sample size and power calculations, and adverse events and pilot/feasibility studies in prevention and treatment of AKI. Companion articles outline the discussions of workgroups for model trials related to prevention or treatment of established AKI in different clinical settings, such as in patients with sepsis.

  11. [Clinical case--voluminous diaphragmatic hernia--surgically acute abdomen: diagnostic and therapeutical challenges].

    PubMed

    Dumitrescu, D; Savlovschi, C; Borcan, R; Pantu, H; Serban, D; Gradinaru, S; Smarandache, G; Trotea, T; Branescu, C; Musat, L; Comandasu, M; Priboi, M; Baldir, M; Sandolache, B; Oprescu, S

    2011-01-01

    We present the case of a 58-year old male patient admitted in the surgery section of the University Emergency Hospital of Bucharest and diagnosed with acute abdomen. The minimal clinical-paraclinical investigation (i.e., thorax-pulmonary Xray, biological probes) raises questions as to the differentiated diagnosis and other associated diseases, also suggesting the existence of voluminous diaphragmatic hernia. The CT thorax-abdomen examination confirms the diaphragmatic hernia suspicion, with intra-thorax ascent of the colon up to the anterior C4 level, but does not explain the abdominal suffering; thus we suspected a biliary ileus or acute appendicitis. Medial laparotomy was imperative. Intrasurgically peritonitis was noticed located by gangrenous acute apendicitis, perforated, with coprolite, for which apendictomy and lavage-drainage pf the peritoneal cavity was performed. Post-surgical status: favourable to recovery.

  12. [Fragile X syndrome with Dandy-Walker variant: a clinical study of oral and written communicative manifestations].

    PubMed

    Lamônica, Dionísia Aparecida Cusin; Ferraz, Plínio Marcos Duarte Pinto; Ferreira, Amanda Tragueta; Prado, Lívia Maria do; Abramides, Dagma Venturini Marquez; Gejão, Mariana Germano

    2011-01-01

    The Fragile X syndrome is the most frequent cause of inherited intellectual disability. The Dandy-Walker variant is a specific constellation of neuroradiological findings. The present study reports oral and written communication findings in a 15-year-old boy with clinical and molecular diagnosis of Fragile X syndrome and neuroimaging findings consistent with Dandy-Walker variant. The speech-language pathology and audiology evaluation was carried out using the Communicative Behavior Observation, the Phonology assessment of the ABFW - Child Language Test, the Phonological Abilities Profile, the Test of School Performance, and the Illinois Test of Psycholinguistic Abilities. Stomatognathic system and hearing assessments were also performed. It was observed: phonological, semantic, pragmatic and morphosyntactic deficits in oral language; deficits in psycholinguistic abilities (auditory reception, verbal expression, combination of sounds, auditory and visual sequential memory, auditory closure, auditory and visual association); and morphological and functional alterations in the stomatognathic system. Difficulties in decoding the graphical symbols were observed in reading. In writing, the subject presented omissions, agglutinations and multiple representations with the predominant use of vowels, besides difficulties in visuo-spatial organization. In mathematics, in spite of the numeric recognition, the participant didn't accomplish arithmetic operations. No alterations were observed in the peripheral hearing evaluation. The constellation of behavioral, cognitive, linguistic and perceptual symptoms described for Fragile X syndrome, in addition to the structural central nervous alterations observed in the Dandy-Walker variant, caused outstanding interferences in the development of communicative abilities, in reading and writing learning, and in the individual's social integration.

  13. Presence of an acute phase response in sheep with clinical classical scrapie

    PubMed Central

    2012-01-01

    Background Work with experimental scrapie in sheep has been performed on-site for many years including studies on PrPSc dissemination and histopathology of organs and tissues both at preclinical and clinical stages. In this work serum was sampled at regular intervals from lambs which were infected immediately after birth and from parallel healthy controls, and examined for acute phase proteins. In contrast to earlier experiments, which extensively studied PrPSc dissemination and histopathology in peripheral tissues and brain, this experiment is focusing on examination of serum for non-PrPSc markers that discriminates the two groups, and give insight into other on-going processes detectable in serum samples. Results There was clear evidence of an acute phase response in sheep with