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1

Whitmore's disease: an uncommon urological presentation.  

PubMed

The incidence of prostatic abscesses has much decreased in the antibiotic era. We present an uncommon cause of prostatic abscess secondary to melioidosis, also known as Whitmore's disease or pseudoglanders. The disease is endemic in South East Asia and Australia. Although India is considered endemic for Burkholderia pseudomallei, the causative organism of melioidosis, not many cases have been reported. Most of the reported cases from India are from the South-West coastal regions of Kerala and Karnataka, Vellore, West Bengal and Bihar. Our index patient was successfully treated with parenteral antibiotics and endoscopic deroofing of the abscess. PMID:24518392

Naganathan, Karthickeyan; Pillai, Sunil Bhaskara; Kumar, Praveen; Hegde, Padmaraj

2014-01-01

2

Uncommon neurological manifestations of a common tropical vector borne disease  

PubMed Central

Malaria poses a major public health problem in India, where it is endemic, especially severe malaria caused by Plasmodium falciparum infestation. There have been great changes in the clinical manifestation of severe falciparum malaria over the past couple of decades, with a shift from cerebral malaria to fever with jaundice, renal failure, bleeding diathesis, and multi-organ dysfunction syndrome. Here, we discuss two cases of severe falciparum malaria which presented with extremely uncommon neurological manifestations. PMID:25709956

Ghosh, Saikat; Das, Somak Kumar; Sharma, Anand

2015-01-01

3

Syphilitic Hepatitis: An Uncommon Manifestation of a Common Disease  

PubMed Central

Hepatitis being first manifestation of secondary syphilis is rare. Here in we report a case of 39 years old male who was being treated for hepatitis and presented to us subsequently with itchy maculopapular rash. Venereal disease research laboratory (VDRL) titre was 1:16. Treponema pallidum hemagglutination assay (TPHA) was positive. He was treated with intramuscular Benzathine Penicillin. His hepatitis improved rapidly. PMID:24700957

Baveja, Sukriti; Garg, Shilpa; Rajdeo, Amol

2014-01-01

4

Recovery of uncommon bacteria from blood: association with neoplastic disease.  

PubMed Central

Table 6 is a summary of the organisms discussed with a listing of the environmental source, the endogenous source, the predisposing factors including neoplasms, and the postulated mechanisms by which the organism can gain access to the circulation. The evidence considered indicates that the entrance of one of these microorganisms into the bloodstream of a human being depends on the presence of multiplicity of predisposing factors. In the majority of cases of bacteremia due to one of these unusual organisms, two or more predisposing factors are present. Certain predisposing factors, such as cancer chemotherapy or intravenous catheterization, often provide a barrier break, while others, such as liver disease, may render the host immune system less capable of clearing organisms from the circulation. For organisms such as Campy-lobacter, Listeria, and Salmonella spp., attributes that allow the invasion of a healthy host are present and seem to be enhanced by the simultaneous presence of a predisposing condition, such as liver disease, in the host. Although somewhat fragmentary, a number of individual case reports describe bacteremia due to one of these organisms occurring weeks to years after surgery and after other therapeutic measures had effected a supposed cure of a cancer. It may be speculated that cancer patients, even after a cure, are still susceptible to bloodstream invasion by one of the aforementioned organisms by virtue of the presence of one or more predisposing metabolic, physiologic, or immunologic factors, even though these factors may be cryptic. The predominance of hematologic malignancies among cases of bacteremia due to these unusual organisms is also apparent. Although, as pointed out by Keusch (169), the reduction in the performance of immune function in hematologic malignancies compared with solid tumors is likely to be responsible, other associations of certain organisms with specific neoplasms warrant further examination. The frequency of bloodstream infections of Salmonella typhimurium and Capno-cytophaga canimorsus in Hodgkin's disease patients seems likely due to a particular mechanism which infection by these species is favored. The specific nature of these mechanisms remains to be determined. The recovery of any unusual bacterium from blood should warrant a careful consideration of the possibility of underlying disease, especially cancer. Microbiologists should advise clinicians of the unusual nature of the identified organism and provide the counsel that certain neoplastic processes, often accompanied by neutropenia, render the human host susceptible to invasion by almost any bacterium. The recovery of such organisms as C. septicum or S. bovis should prompt the clinician to aggressively seek to identify an occult neoplasm if one has not yet been diagnosed. PMID:7553569

Beebe, J L; Koneman, E W

1995-01-01

5

Salmonella hepatitis: an uncommon complication of a common disease.  

PubMed

Typhoid fever is a very common infectious disease of tropics, associated with high morbidity and mortality. Typhoid fever is often associated with hepatomegaly and mildly deranged liver functions; a clinical picture of acute hepatitis is a rare complication. We report a young patient who presented with fever and jaundice and was found to have acute hepatitis secondary to typhoid fever. Recognition of Salmonella hepatitis is of clinical importance as it can mimic acute viral hepatitis. Early institution of specific therapy can improve the prognosis in these patients. Typhoid fever is a very common infectious disease of tropics, associated with high morbidity and mortality. Typhoid fever is often associated with hepatomegaly and mildly deranged liver functions; a clinical picture of acute hepatitis is a rare complication. We report a young patient who presented with fever and jaundice and was found to have acute hepatitis secondary to typhoid fever. Recognition of Salmonella hepatitis is of clinical importance as it can mimic acute viral hepatitis. Early institution of specific therapy can improve the prognosis in these patients. PMID:24479030

Karoli, Ritu; Fatima, Jalees; Chandra, Ashok; Singh, Gagandeep

2012-07-01

6

Is immunoproliferative small intestinal disease uncommon in India?  

PubMed

Till date only three series of immunoproliferative small intestinal disease (IPSID) describing 22 patients have been reported from India. Seven patients with IPSID in two tertiary referral centers in India are included in the study. Diagnosis was based on typical clinical features [diarrhoea (7/7), weight loss (7/7), clubbing (6/7), fever (3/7), abdominal pain and lump (3/7)], biochemical evidence of malabsorption and duodenal biopsy findings. All patients were young males (mean age 29.8 +/- 11.8 years, range 17-53). Atypical features included gastric involvement (1/7), colonic involvement (1/7) and appearance of pigmented nails following anti-cancer chemotherapy (1/7) which disappeared six months after omitting doxorubin from chemotherapy regimen. Parasitic infestation was common. Ascaris lumbricoides (1/7), Giardia lamblia and hookworm (1/7), Strongyloides stercoralis and Trichuris trichura (1/7). In the latter patient S. stercoralis became disseminated after anti-malignant chemotherapy. One patient had gastric H. pylori infection. Four of the seven patients who were misdiagnosed as tropical sprue were treated with tetracycline. This raises doubt on efficacy of tetracycline alone in treatment of IPSID. One other patient was misdiagnosed and treated as intestinal tuberculosis. Early diagnosis and administration of chemotherapy may improve survival in this disease. PMID:11398237

Ghoshal, U C; Chetri, K; Banerjee, P K; Choudhuri, G; Pal, B B; Dabadghao, S; Dhar, K; Naik, S; Naik, S R

2001-01-01

7

Bartonella henselae Infection: An Uncommon Mimicker of Autoimmune Disease  

PubMed Central

We present a case of a seven-year-old immunocompetent female patient who developed systemic symptoms mimicking an autoimmune rather than an infectious disease. The patient presented with rash, biquotidian fever, night sweats, and arthralgias. There was no antecedent history of cat contact. Investigations showed increased inflammatory markers, leukocytosis, thrombocytosis, hypercalcemia, and raised angiotensin-converting enzyme. Interferon-gamma releasing assay for tuberculosis infection was negative. Abdominal imaging demonstrated multifocal lesions of the liver and spleen (later proved to be granulomata), chest X-ray showed enlarged hilar lymph nodes, and ophthalmology review revealed uveitis. Clinical, laboratory, and imaging features pointed towards sarcoidosis. Subsequently, raised titers (IgM 1 : 32, IgG 1 : 256) against Bartonella confirmed the diagnosis of B. henselae infection. She was treated with gentamycin followed by ciprofloxacin; repeat investigations showed complete resolution of findings. The presence of hepatic and splenic lesions in children with bartonellosis is well documented. Our case, however, exhibited certain unusual findings such as the coexistence of acute ocular and systemic involvement in an immunocompetent host. Serological testing is an inexpensive and effective way to diagnose bartonellosis in immunocompetent patients; we suggest that bartonella serology is included in the baseline tests performed on children with prolonged fever even in the absence of contact with cats in countries where bartonellosis is prevalent. PMID:23424700

Maritsi, Despoina N.; Zarganis, Diagoras; Metaxa, Zoi; Papaioannou, Georgia; Vartzelis, George

2013-01-01

8

Multimodality imaging of common and uncommon peritoneal diseases: a review for radiologists.  

PubMed

Peritoneal disease can be caused by a wide spectrum of pathologies. While peritoneal disease is usually caused by primary or secondary malignancies, benign diseases can occur and mimic malignancies. This article begins with an overview of peritoneal embryology and anatomy followed by a detailed description of the multimodality imaging appearance of peritoneal diseases. Common diseases include peritoneal carcinomatosis, pseudomyxoma peritonei, lymphomatosis, sarcomatosis, and tuberculous peritonitis. The uncommon diseases which cause peritoneal disease include desmoid fibromatosis, desmoplastic small round cell tumor, malignant mesothelioma, well-differentiated mesothelioma, multicystic mesothelioma, papillary serous carcinoma, leiomyomatosis, extramedullary hematopoiesis, inflammatory pseudotumor and amyloidosis. This manuscript will help the radiologist become familiar with the different peritoneal spaces, pathways of spread, multimodality imaging appearance and differential diagnoses of peritoneal diseases in order to report the essential information for surgeons and oncologists to plan treatment. PMID:25139643

Vicens, Rafael A; Patnana, Madhavi; Le, Ott; Bhosale, Priya R; Sagebiel, Tara L; Menias, Christine O; Balachandran, Aparna

2015-02-01

9

Nodular fasciitis: an uncommon disease with common medical management challenges at a remote Naval Hospital.  

PubMed

Nodular fasciitis is a rare benign soft tissue lesion that is often confused with malignant sarcoma, which can make management of this pathology challenging. We present here a case of head and neck nodular fasciitis that was managed at a remote U.S. Naval Hospital with limited diagnostic and therapeutic resources. The aim of this article is to bring to light this uncommon pathology so that it can be given its due consideration in the differential diagnosis from both a clinical and pathologic perspective. However, the more important purpose of this article is to highlight the complex decision-making process that sometimes occurs when evaluating patients within a medical infrastructure that is significantly less than that of a typical military treatment facility or U.S. hospital. Young surgeons and other providers may find this discussion useful before deploying, whether it be to a remote land-based facility or to an aircraft carrier. PMID:24005559

Spinelli, Nicholas; Khorassani, Nima

2013-09-01

10

Uncommon Knowledge  

NSDL National Science Digital Library

Hosted by Peter M. Robinson of the Hoover Institution, Uncommon Knowledge has been taking a critical look at public policy issues since 1996. With support from the John M. Olin Foundation, Uncommon Knowledge is distributed by American Public Television and can also be heard on NPR. In keeping with the traditions of the Hoover Institution, the programs feature lively debate on any number of topics, ranging from gun control to international foreign diplomacy. Visitors to the website can browse through a list of recent shows, or look through their archives which date from 1997. Visitors may also wish to view the entire television program on their computer, listen to the audio presentation, or read a transcript.

Robinson, Peter.

2005-01-01

11

Systemic Lupus Erythematosus Pancreatitis: An Uncommon Presentation of a Common Disease  

PubMed Central

Patient: Female, 21 Final Diagnosis: Systemic lupus erythematosus pancreatitis Symptoms: Abdominal pain Medication: — Clinical Procedure: — Specialty: Gastroenterology and Hepatology Objective: Challenging differential diagnosis Background: Acute pancreatitis is uncommon in systemic lupus erythematosus (SLE). When recognized early and properly treated with IV steroids and hydration, the course may be benign, as exemplified in the following report. Case Report: A 21-year-old woman with history of SLE and stage IV lupus nephritis, was admitted to the Sergio Bernales Hospital ICU (Lima, Peru), complaining of worsening epigastric pain radiating to the back, and nausea and vomiting for 1 week. She denied prior cholelithiasis, alcohol use, or recent medication changes. On examination, she was tachycardic and normotensive, with a slightly distended abdomen and epigastric tenderness on deep palpation, without signs of peritoneal irritation. Laboratory results demonstrated leukocytosis without left shift, creatinine of 2.26 mg/dL, amylase of 750 U/L, and lipase of 1038 U/L. Liver chemistries, calcium, lactic acid, triglycerides, and IgG4 were normal and alcohol level was undetectable. Ultrasound did not show cholelithiasis, biliary sludge, or common bile duct dilation. CT of the abdomen showed pancreas head (parenchyma) stranding with uniform enhancement consistent with interstitial pancreatitis. Despite receiving IV fluids, opiates, anti-emetics, and nothing by mouth, her clinical condition deteriorated, prompting the use of IV methylprednisolone. After completing 1 week of IV steroids, she was transferred to the medical floor clinically improved. The patient was discharged with an oral steroid taper and complete resolution of symptoms. Conclusions: After ruling out common causes, such as hepatobiliary pathology or toxin-related insults like alcohol, hypercalcemia, hypertriglyceridemia or medications, steroids may be used in SLE pancreatitis because they might improve the overall prognosis. PMID:25399483

Rodriguez, Eduardo A.; Sussman, Daniel A.; Rodriguez, Vanessa R.

2014-01-01

12

Uncommon Lives  

NSDL National Science Digital Library

The Uncommon Lives series on the National Archives of Australia website takes an approach to Australian history that not only encompasses the well-known history-makers, but also lesser known people's role in shaping Australian history. One of the stated goals of the Uncommon Lives series is to show how amateur historians and researchers alike can use the archives to find biographical resources. There are five stories the visitor can discover by simply clicking on the image of the person or people next to the brief description of their story including, "Muslim Journeys", "Charles and Ruth Lane Poole", "Jessie Street", "Wolf Klaphake", and "Dhakiyarr Wirrpanda". By clicking on any choice, visitors will find each story divided into subsections. Explanatory text accompanies the thumbnails of each image and these can be expanded into a high quality image by clicking on them. Each of these stories provides a unique and compelling look into Australian history. For instance in Wolf Klaphake's story you can listen to or read the transcript of segments of the ABC radio play "A Doubtful Character" which is about Klaphake's life and in Dhakiyarr Wirrpanda's story, you can view the actual court records of his case, which was the first for an Aboriginal Australian in the High Court.

2007-01-01

13

Isolated Nasopharyngeal Castleman Disease: An Uncommon Diagnosis in an Unusual Location  

PubMed Central

Localised nasopharyngeal Castleman disease has rarely been reported. We present a case involving a 23-year-old female, describe the clinical, imaging, and histopathologic features of this challenging diagnosis, and review the literature. PMID:25126439

McDonnell, O.; Morris, Melinda; Khaleel, Z.

2014-01-01

14

DNA analysis of an uncommon missense mutation in a Gaucher disease patient of Jewish-Polish-Russian descent  

SciTech Connect

Gaucher disease is the most frequent lysosomal lipid storage disease. It results from deficient glucocerebrosidase activity and is transmitted as an autosomal recessive trait. Three clinical forms of Gaucher disease have been described: type 1, non-neuronopathic; type 2, acute neuronopathic; and type 3, subacute neuronopathic. We have sequenced the full length cDNA of the glucocerebrosidase gene and identified an uncommon mutation in nucleotide position 1604 (genoma DNA nucleotide position 6683) from a Gaucher disease patient of Jewish-Polish-Russian descent with type 1 Gaucher disease. It is a G{yields}A transition in exon 11 that results in {sup 496}Arg{yields}{sup 496}His of glucocerebrosidase. This missense mutation is present in the heterozygous form and creates a new cleavage site for the endonuclease HphI. We have developed a simple method to detect the presence of this mutation by using HphI restriction fragment length polymorphism analysis of glucocerebrosidase genomic DNA or cDNA. The mutation in the other Gaucher allele of this patient is an A{yields}G transition at cDNA nucleotide position 1226 which creates an XhoI cleavage site after PCR mismatch amplification. The presence of this mutation was also confirmed by sequence analysis. Based on previous reports that mutation 1226 is present only in type 1 Gaucher disease and the observation that there is no neurological involvement in this patient, we conclude that our patient with the 1226/1604 genotype is diagnosed as having type 1 Gaucher disease. Since it was also postulated that mutation 1226 in the homozygous form will usually result in a good prognosis, we speculate that the orthopedic complications and the unusual presence of glomerulosclerosis in this patient may be attributable to the mutation at nucleotide 1604. This speculation will require a description of more patients with this mutation for confirmation. 32 refs., 5 figs.

Choy, F.Y.M.; Wei, C.; Applegarth, D.A.; McGillivray, B.C. [Univ. of British Columbia, Vancouver (Canada)

1994-06-01

15

Celiac disease and gastric hyperplastic polyps: a case series of an uncommon association.  

PubMed

Gastric polypoid lesions are found in ?1-4% of patients who undergo esophagogastroduodenoscopy. The hyperplastic lesions are considered non-neoplastic polyps that are distributed randomly in the stomach and they are usually related to chronic gastritis as a result of the exaggerated mucosal healing response. Although several conditions have been associated with celiac disease (CD), such as thyroiditis, Addison's disease, type 1 diabetes mellitus, and autoimmune hepatitis, the association with gastric polyps is rare. In this case series, we present seven newly diagnosed patients (six women) with CD and hyperplastic gastric polyps. Helicobacter pylori infection was ruled out through histology in all the patients and two out of seven had a history of occasional proton pump inhibitor use. An unusual association was found between CD and hyperplastic polyps in this case series. PMID:24901825

Galvez-Ríos, Shareni; Arano-Acua, Miguel; Meixueiro-Daza, Arturo; Grube-Pagola, Peter; Remes-Troche, José M

2014-07-01

16

Palpable purpura with foot drop: Common presentations in an uncommon disease.  

PubMed

Churg-Strauss syndrome is a rare disease manifested by hypereosinophilia, vasculitis and tissue infiltration. This report describes the case of a 45-year-old man who presented with a history of fever, difficulty in breathing, reddish lesions over the extremities and inability to walk since two weeks. The cutaneous features prompted us to conduct serial lab investigations which led to an early, potentially life saving diagnosis. PMID:25821734

Mathanda, Teena Ramesh; Bhat, Ramesh; Alva, Jayaprakash

2015-01-01

17

Palpable purpura with foot drop: Common presentations in an uncommon disease  

PubMed Central

Churg–Strauss syndrome is a rare disease manifested by hypereosinophilia, vasculitis and tissue infiltration. This report describes the case of a 45-year-old man who presented with a history of fever, difficulty in breathing, reddish lesions over the extremities and inability to walk since two weeks. The cutaneous features prompted us to conduct serial lab investigations which led to an early, potentially life saving diagnosis.

Mathanda, Teena Ramesh; Bhat, Ramesh; Alva, Jayaprakash

2015-01-01

18

Orbital Pseudotumor: Uncommon Initial Presentation of IgG4-Related Disease  

PubMed Central

IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.

Carbone, Teresa; Azędo Montes, Ricardo; Andrade, Beatriz; Lanzieri, Pedro; Mocarzel, Luis

2015-01-01

19

Diurnal and twenty-four hour patterning of human diseases: acute and chronic common and uncommon medical conditions.  

PubMed

The symptom intensity and mortality of human diseases, conditions, and syndromes exhibit diurnal or 24 h patterning, e.g., skin: atopic dermatitis, urticaria, psoriasis, and palmar hyperhidrosis; gastrointestinal: esophageal reflux, peptic ulcer (including perforation and hemorrhage), cyclic vomiting syndrome, biliary colic, hepatic variceal hemorrhage, and proctalgia fugax; infection: susceptibility, fever, and mortality; neural: frontal, parietal, temporal, and occipital lobe seizures, Parkinson's and Alzheimer's disease, hereditary progressive dystonia, and pain (cancer, post-surgical, diabetic neuropathic and foot ulcer, tooth caries, burning mouth and temporomandibular syndromes, fibromyalgia, sciatica, intervertebral vacuum phenomenon, multiple sclerosis muscle spasm, and migraine, tension, cluster, hypnic, and paroxysmal hemicranial headache); renal: colic and nocturnal enuresis and polyuria; ocular: bulbar conjunctival redness, keratoconjunctivitis sicca, intraocular pressure and anterior ischemic optic neuropathy, and recurrent corneal erosion syndrome; psychiatric/behavioral: major and seasonal affective depressive disorders, bipolar disorder, parasuicide and suicide, dementia-associated agitation, and addictive alcohol, tobacco, and heroin cravings and withdrawal phenomena; plus autoimmune and musculoskeletal: rheumatoid arthritis, osteoarthritis, axial spondylarthritis, gout, Sjögren's syndrome, and systemic lupus erythematosus. Knowledge of these and other 24 h patterns of human pathophysiology informs research of their underlying circadian and other endogenous mechanisms, external temporal triggers, and more effective patient care entailing clinical chronopreventive and chronotherapeutic strategies. PMID:25129839

Smolensky, Michael H; Portaluppi, Francesco; Manfredini, Roberto; Hermida, Ramon C; Tiseo, Ruana; Sackett-Lundeen, Linda L; L Haus, Erhard

2014-07-18

20

Bacterial infections: uncommon presentations  

Microsoft Academic Search

The essence of dermatology is morphology. The most important instrument in the practice of dermatology has always been, and still is, the naked eye; however, “We see only what we are ready to see, what we have been taught to see” (Jean Martin Charcot). Although most practitioners will easily correctly diagnose common bacterial skin diseases (such as cellulitis, erysipelas, impetigo,

Hagit Matz; Edith Orion; Ronni Wolf

2005-01-01

21

Imaging of uncommon esophageal malignancies.  

PubMed

Malignant esophageal neoplasms other than squamous cell carcinoma and adenocarcinoma are uncommon and include endocrine tumors, lymphoid malignancies, melanoma, malignant stromal tumors, and secondary tumors (metastases). Imaging, though not diagnostic in many cases, helps in selecting the appropriate treatment strategy by determining the anatomic extent of the tumor and locoregional and distant spread. In this article, we provide a comprehensive review of the imaging features of these uncommon esophageal malignancies. PMID:24635682

Tirumani, H; Rosenthal, M H; Tirumani, S H; Shinagare, A B; Krajewski, K M; Ramaiya, N H

2014-03-17

22

Uncommon and/or bizarre features of dementia.  

PubMed

This study aimed at describing uncommon or bizarre symptoms observed in patients suffering from dementia. Medline and Google scholar searches were conducted for relevant articles, chapters, and books published since 1967. Search terms used included uncommon presentation, behavioural and psychological symptoms, dementia, Alzheimer's disease, and fronto-temporal dementia. Publications found through this indexed search were reviewed for further relevant references. The uncommon symptoms are described as case-reports and there are no systematic investigations. Bizarre behaviours arising late in life should be thoroughly investigated as symptoms of dementia. PMID:24854147

Cipriani, Gabriele; Lucetti, Claudio; Danti, Sabrina; Ulivi, Martina; Nuti, Angelo

2015-03-01

23

Uncommon pancreatic tumors and pseudotumors.  

PubMed

A heterogeneous group of uncommon neoplastic and non-neoplastic pancreatic pathologies exists that can mimic pancreatic adenocarcinoma. These "imitators" are unique and may demonstrate characteristic clinical and imaging features. Imaging characteristics of some of these diverse lesions are not well described in the literature, and erroneous diagnoses of these entities as pancreatic carcinoma may be responsible for unnecessary surgeries. Knowledge of these selected pancreatic pathologies is essential to facilitate optimal patient management. PMID:25063236

Lalwani, Neeraj; Mannelli, Lorenzo; Ganeshan, Dhakshina Moorthy; Shanbhogue, Alampady K; Dighe, Manjiri K; Tiwari, Hina Arif; Maximin, Suresh; Monti, Serena; Ragucci, Monica; Prasad, Srinivasa R

2015-01-01

24

Uncommon surgical emergencies in neonatology.  

PubMed

Objective. Over the past decade, multiple factors have changed the pattern of neonatal surgical emergencies. An increase in prenatal screenings and the development of neonatal tertiary care centres have changed the clinical approach to these kids. Materials and methods. Between 1995 to 2011 were retrospectively reviewed 34 patients with diagnosis of uncommon rare neonatal surgical emergencies at our institute. We analyzed: sex, gestational age, weight at birth, primary pathology, prenatal diagnosis, associated anomalies, age and weight at surgery, clinical presentation, start of oral feeding and hospitalization. The follow-up was performed at 6,12, 24 and 36 months. Results. There were 21 male and 13 female. The gestational age ranged between 28 and 36 weeks. The weight at birth ranged between 700 and 1400 grams. Oral feeding was started between 4th and 10th postoperative day. The average hospitalization was about 70.47 days. To date, all patients have finished the followup. They are healthy. Conclusion. The outcome of the patients with uncommon surgical emergencies is different based on the etiology. Overall survival is generally good but is influenced by the associated anomalies. PMID:25669890

Angotti, R; Bulotta, A L; Ferrara, F; Molinaro, F; Cerchia, E; Meucci, D; Messina, M

2014-01-01

25

Secondary Syphilis: Uncommon Manifestations a Common Disease  

PubMed Central

Reports of cases of primary and secondary syphilis are increasing in the United States, particularly in urban areas and among homosexual men. While primary syphilis poses little diagnostic difficulty, many physicians are unfamiliar with the multisystem nature of secondary lues. Patients who have secondary syphilis commonly present with systemic signs, skin rash, mucous membrane lesions and generalized adenopathy. Less commonly, secondary syphilis may occur as acute meningitis, sensorineural hearing loss, iritis, anterior uveitis, optic neuritis, Bell's palsy, gastropathy, proctitis, hepatitis, pulmonary infiltration, nephrotic syndrome, glomerulonephritis, periostitis, tenosynovitis and polyarthritis. The diagnosis of secondary syphilis is easily confirmed. Its various manifestations are readily treated with penicillin and, if treated early, are entirely reversible. Two recent cases of secondary syphilis, one presenting as nephrotic syndrome and one as chorioretinitis and ptosis, illustrate the usual and unusual features of this common infection. Images PMID:6702190

McPhee, Stephen J.

1984-01-01

26

An uncommon case of hepatopulmonary amoebiasis.  

PubMed

Amoebiasis is an uncommon infection in developed countries caused by the protozoan Entamoeba histolytica. Amoebic liver abscess is the most frequent extraintestinal presentation of the disease; pleuropulmonary involvement is rare, occurring mostly by rupture of the abscess into the pleural space or lung parenchyma. We describe a case of a 48-year-old migrant from Săo Tomé e Príncipe, with fever, wasting, dry cough and right upper abdominal pain for the past 2?months. The CT scan revealed a voluminous liver abscess with thrombosis of the right suprahepatic and inferior vena cava, right pulmonary lobar abscess and multiple diffuse condensations in both lungs. Aspirated pus resembled anchovy sauce; blood and aspirated material cultures for infectious agents were negative. Serology for E. histolytica was positive, and the diagnosis of hepatopulmonary amoebiasis with infectious phlebitis was confirmed by positive PCR in the liver pus. Treatment with metronidazole+paramomycin led to clinical and radiological resolution. PMID:25155487

Patrício, Catarina; Amaral, Patrícia; Lourenço, Joăo

2014-01-01

27

Oral ulcer: an uncommon site in primary tuberculosis.  

PubMed

Tuberculosis is a chronic infectious disease and a major cause of morbidity and mortality worldwide. It can affect any part of the body, including the oral cavity. Oral lesions of tuberculosis, though uncommon, have been observed in both primary and secondary stages of the disease. This article presents a case of primary tuberculosis manifested as a non-healing, tender ulcer on the lingual mucosa of the edentulous right mandibular arch molar zone, an uncommon site. The diagnosis was confirmed after histopathology examination, polymerase chain reaction and purified protein derivative tests and chest radiograph. A recommended treatment plan of six months with four anti-tuberculotic antibiotics was commenced. Clinically, the oral ulcer disappeared three months after the commencement of treatment. The resurgence of tuberculosis should compel clinicians to include the disease in the differential diagnosis of various types of non-healing oral ulcers. PMID:25721285

Aoun, N; El-Hajj, G; El Toum, S

2015-03-01

28

Uncommon Locations and Presentations of Hydatid Cyst  

PubMed Central

Background: Hydatid disease (HD) is an ancient disease and even was known to Hippocrates. This disease involves all human parts and most common affected organs are liver and lungs. Incidence of unusual site is about 8-10%. The clinical picture depends upon the involved organs, its effects on adjacent structures, complications due to secondary infection, rupture, and anaphylaxis caused by hydatid cysts. Aim: The aim of this study was to find out incidence of unusual location of hydatid cyst in the human body. Materials and Methods: A retrospective study of HD was carried in a medical college between July 2007 and June 2012. A total 79 cases of HD were treated during this period. Information on clinical presentation and management were reviewed, and results presented as summary statistics. Results: Sixty one cases were of liver HD, and 11 were with hydatid lung disease. Fifty cases were with right lobe involvement, and rest 11 were with both lobe involvement. Out of 11 lung hydatid only one case was with bilateral lung involvement. Only eight cases of HD of uncommon locations and presentations were encountered during this period. First case presented with left hypochondriac mass as splenic HD, second with pelvic HD along with obstructive uropathy, third with non-functioning right kidney with bilateral psoas muscles HD, fourth with HD involving mesentery, fifth with pelvic pain due to right ovary HD, sixth with simultaneous involvement of the liver and right subdiaphragmatic region, seventh with HD of right inguinal region, and eighth with hydatid cyst of the left kidney. Even though, there was no mortality found in these patients, there was high morbidity. Conclusion: We conclude that Echinococcus granulosus can affect any organ in the body from head to toe, and a high suspicion of this disease is justified in endemic regions. Moreover, medical treatment should be given in the pre-operative period as well as in the post-operative period for 4-6 weeks. PMID:24971224

Sachar, S; Goyal, S; Goyal, S; Sangwan, S

2014-01-01

29

Uncommon Success: A Conversation with Brett Peiser  

ERIC Educational Resources Information Center

This article introduces a conversation with Brett Peiser, named chief executive officer of "Uncommon Schools" in July of 2012, along with the principal of North Star Academy Vailsburg Middle School, a charter school in Newark, New Jersey's West Ward. There is no doubt that "Uncommon Schools" has given thousands of…

Meyer, Peter

2014-01-01

30

Uncommon hepatic tumors: iconographic essay - Part 1*  

PubMed Central

Most malignant liver tumors are represented by hepatocellular carcinoma and cholangiocarcinoma; however a variety of other uncommon hepatic lesions might also be found. Common lesions such as hemangioma, focal nodular hyperplasia and metastases are well known and have already been extensively documented in the literature. The diagnosis of typical hepatic lesions may be done with some reliability by means of several imaging methods; on the other hand, uncommon lesions normally represent a diagnostic challenge for the radiologist. In this first part of the study, the authors will approach five uncommon liver tumors - angiosarcoma, angiomyolipoma, cystadenoma/biliary carcinoma, epithelioid hemangioendothelioma, and fibrolamellar hepatocellular carcinoma -, describing their main characteristics and image findings with focus on computed tomography and magnetic resonance imaging. PMID:25741106

Pedrassa, Bruno Cheregati; da Rocha, Eduardo Lima; Kierszenbaum, Marcelo Longo; Bormann, Renata Lilian; Torres, Lucas Rios; D'Ippolito, Giuseppe

2014-01-01

31

Uncommon hepatic tumors: iconographic essay - Part 1.  

PubMed

Most malignant liver tumors are represented by hepatocellular carcinoma and cholangiocarcinoma; however a variety of other uncommon hepatic lesions might also be found. Common lesions such as hemangioma, focal nodular hyperplasia and metastases are well known and have already been extensively documented in the literature. The diagnosis of typical hepatic lesions may be done with some reliability by means of several imaging methods; on the other hand, uncommon lesions normally represent a diagnostic challenge for the radiologist. In this first part of the study, the authors will approach five uncommon liver tumors - angiosarcoma, angiomyolipoma, cystadenoma/biliary carcinoma, epithelioid hemangioendothelioma, and fibrolamellar hepatocellular carcinoma -, describing their main characteristics and image findings with focus on computed tomography and magnetic resonance imaging. PMID:25741106

Pedrassa, Bruno Cheregati; da Rocha, Eduardo Lima; Kierszenbaum, Marcelo Longo; Bormann, Renata Lilian; Torres, Lucas Rios; D'Ippolito, Giuseppe

2014-01-01

32

Gleason Grade Progression Is Uncommon  

PubMed Central

Gleason grade is universally used for pathologic scoring the differentiation of prostate cancer. However, it is unknown whether prostate tumors arise well-differentiated and then progress to less differentiated forms or if Gleason grade is an early and largely unchanging feature. Prostate Specific Antigen (PSA) screening has reduced the proportion of tumors diagnosed at advanced stage, which allows assessment of this question on a population level. If Gleason grade progresses as stage does, one would expect a similar reduction in high grade tumors. We studied 1,207 Physicians’ Health Study and Health Professionals Follow-up Study participants diagnosed with prostate cancer 1982–2004 and treated with prostatectomy. We compared the distribution of grade and clinical stage across the pre-PSA and PSA screening eras. We re-reviewed grade using the ISUP 2005 revised criteria. The proportion of advanced stage tumors dropped more than six-fold, from the earliest period (12/1982–1/1993), 19.9% stage ?T3, to the latest (5/2000–12/2004), 3% stage T3, none T4. The proportion of Gleason score ?8 decreased substantially less, from 25.3% to 17.6%. A significant interaction between stage and diagnosis date predicting grade (p=0.04) suggests the relationship between grade and stage varies by time period. As the dramatic shift in stage since the introduction of PSA screening was accompanied by a more modest shift in Gleason grade, these findings suggest grade may be established early in tumor pathogenesis. This has implications for the understanding of tumor progression and prognosis, and may help patients diagnosed with lower grade disease feel more comfortable choosing active surveillance. PMID:23946472

Penney, Kathryn L.; Stampfer, Meir J.; Jahn, Jaquelyn L; Sinnott, Jennifer A.; Flavin, Richard; Rider, Jennifer R.; Finn, Stephen; Giovannucci, Edward; Sesso, Howard D.; Loda, Massimo; Mucci, Lorelei A.; Fiorentino, Michelangelo

2013-01-01

33

Uncommon features of polyarteritis nodosa: psychosis and angio-oedema.  

PubMed

Psychosis and swelling of the face and hands are rarely observed in adult polyarteritis nodosa (PAN). We describe a 21-year-old woman who presented with fever, livedo reticularis, tender subcutaneous nodules and arthritis. These manifestations did not respond to prednisone, but remitted when the drug was tapered. She had had psychosis since the age of 16 years. During the flares of the disease she presented with facial, periorbital and hand swelling. This finding is rarely observed in adult PAN. Arteriography showed multiple small aneurysms, of the mesenteric vessels consistent with a diagnosis of PAN. Our report discusses the diagnosis of PAN and emphasises the uncommon presentation of this case. PMID:9776125

Fernandes, S R; Coimbra, I B; Costallat, L T; Ribeiro Filho, J E

1998-01-01

34

Threatened and uncommon plants of New Zealand  

Microsoft Academic Search

A reappraisal of the conservation status of New Zealand's threatened and uncommon vascular plants is presented. The list comprises 511 taxa (22% of New Zealand's indigenous vascular flora) in the following categories: Presumed Extinct 5 taxa, Threatened 107 taxa (comprising 24 taxa Critically Endangered, 33 taxa Endangered, 50 taxa Vulnerable), Declining 60 taxa, Recovering 17 taxa (comprising 14 taxa Conservation

P. J. de Lange; P. B. Heenan; D. R. Given; D. A. Norton; C. C. Ogle; P. N. Johnson; E. K. Cameron

1999-01-01

35

Culture, Relevance, and Schooling: Exploring Uncommon Ground  

ERIC Educational Resources Information Center

In "Culture, Relevance, and Schooling: Exploring Uncommon Ground," Lisa Scherff, Karen Spector, and the contributing authors conceive of culturally relevant and critically minded pedagogies in terms of opening up new spatial, discursive, and/or embodied learning terrains. Readers will traverse multiple landscapes and look into a variety of spaces…

Scherff, Lisa, Ed.; Spector, Karen, Ed.

2011-01-01

36

Uncommon opportunistic yeast bloodstream infections from Qatar.  

PubMed

Eleven uncommon yeast species that are associated with high mortality rates irrespective of antifungal therapy were isolated from 17/187 (201 episodes) pediatric and elderly patients with fungemia from Qatar. The samples were taken over a 6-year period (January 2004-December 2010). Isolated species included Kluyveromyces marxianus, Lodderomyces elongisporus, Lindnera fabianii, Candida dubliniensis, Meyerozyma guilliermondii, Candida intermedia, Pichia kudriavzevii, Yarrowia lipolytica, Clavispora lusitaniae, Candida pararugosa, and Wickerhamomyces anomalus. Matrix-assisted laser desorption/ionization time-of-flight mass spectrometry provided correct identifications compared with molecular analysis testing of the same isolates. Low minimal inhibitory concentrations were found when isavuconazole and voriconazole were used for all uncommon yeast species evaluated in this study. Resistance to antifungal drugs was low and remained restricted to a few species. PMID:24934803

Taj-Aldeen, Saad J; AbdulWahab, Atqah; Kolecka, Anna; Deshmukh, Anand; Meis, Jacques F; Boekhout, Teun

2014-07-01

37

Early syphilitic hepatitis in an immunocompetent patient: really so uncommon?  

PubMed

The incidence of syphilis has increased substantially over the past years, particularly in men who have sex with men. The clinical manifestations of syphilis are variable and liver involvement is uncommon, but may occur at any stage of the disease. We report a case of early syphilitic hepatitis (ESH) in an immunocompetent patient referring multiple bisexual exposures, who presented at admission with jaundice, tiredness, an ulcerated genital lesion and an increase of liver aminotransferases. During his hospital stay, he developed a skin rash, and serology for syphilis was found positive. Our case report strengthens the need to take into consideration the diagnosis of ESH in all patients with unexplained liver enzyme increase and epidemiological data of unsafe sexual exposures. Indeed, an early recognition of the clinical manifestations of syphilis can lead to a prompt treatment, and allows the prevention of the transmission of this disease to other individuals. PMID:18275654

Noto, P; Del Nonno, F; Licci, S; Chinello, P; Petrosillo, N

2008-01-01

38

Uncommon mold infections in hematological patients: epidemiology, diagnosis and treatment.  

PubMed

Invasive fungal diseases continue to be an important cause of morbidity and mortality in immunosuppressed patients. This is of particular interest, since the progress we made in the treatment of underlying malignancies has led to an increase of the number of persons 'at high risk'. During the last few years, several changes in clinical practice in hematology (new immunosuppressants, hematopoietic stem cell transplants) have influenced the epidemiology of invasive fungal diseases; in particular, cases due to some uncommon etiologic agents are being increasingly reported, making it even more urgent to reconsider differential diagnoses in high-risk patients. A better understanding of epidemiology, risk factors and prognosis appears to be crucial to analyze prevention and diagnostic strategies, as well as to guarantee an early and adequate treatment. PMID:21810058

Caira, Morena; Trecarichi, Enrico Maria; Mancinelli, Mario; Leone, Giuseppe; Pagano, Livio

2011-07-01

39

Uncommon hepatic tumors: iconographic essay – Part 2  

PubMed Central

In cases where typical aspects are shown, the diagnosis of most frequent hepatic lesions can be made with some safety by means of several imaging methods; on the other hand, uncommon lesions generally represent a diagnostic challenge for the radiologist. In the present second part of the study, the authors describe four rare hepatic lesions, as follows: primary hepatic lymphoma, myofibroblastic tumor, primary hepatic neuroendocrine tumor and desmoplastic small round cell tumor, approaching their main characteristics and imaging findings with emphasis on computed tomography and magnetic resonance imaging. PMID:25741121

Pedrassa, Bruno Cheregati; da Rocha, Eduardo Lima; Kierzenbaum, Marcelo Longo; Bormann, Renata Lilian; Francisc, Viviane Vieira; D’Ippolito, Giuseppe

2014-01-01

40

Papulonecrotic Tuberculid with Scrofuloderma: An Uncommon Association  

PubMed Central

Cutaneous tuberculosis can be classified as true cutaneous tuberculosis and tuberculids which is regarded as a hypersensitivity reaction to M. tuberculosis in patients with a high degree of tuberculin sensitivity. Papulonecrotic tuberculid (PNT) is a form of tuberculid. It is an uncommon manifestation even in areas with high prevalence of tuberculosis. We report a case of 35-year-old man who presented with necrotizing papules in symmetrical fashion over the trunk and extremities for last one year along with a discharging sinus in right axilla for last two months. Papulonecrotic tuberculid (PNT) with scrofuloderma were suspected on clinical examination. Mantoux test was strongly positive with 20x20mm. He was treated successfully with ATT without any further appearance of new lesions.

2015-01-01

41

Uncommon Sense - The Heretical Nature of Science  

NASA Astrophysics Data System (ADS)

Most people believe that science arose as a natural end-product of our innate intelligence and curiosity, as an inevitable stage in human intellectual development. But physicist and educator Alan Cromer disputes this belief. Cromer argues that science is not the natural unfolding of human potential, but the invention of a particular culture, Greece, in a particular historical period. Indeed, far from being natural, scientific thinking goes so far against the grain of conventional human thought that if it hadn't been discovered in Greece, it might not have been discovered at all.In Uncommon Sense , Alan Cromer develops the argument that science represents a radically new and different way of thinking. Using Piaget's stages of intellectual development, he shows that conventional thinking remains mired in subjective, "egocentric" ways of looking at the world--most people even today still believe in astrology, ESP, UFOs, ghosts and other paranormal phenomena--a mode of thought that science has outgrown. He provides a fascinating explanation of why science began in Greece, contrasting the Greek practice of debate to the Judaic reliance on prophets for acquiring knowledge. Other factors, such as a maritime economy and wandering scholars (both of which prevented parochialism) and an essentially literary religion not dominated by priests, also promoted in Greece an objective, analytical way of thinking not found elsewhere in the ancient world. He examines India and China and explains why science could not develop in either country. In China, for instance, astronomy served only the state, and the private study of astronomy was forbidden. Cromer also provides a perceptive account of science in Renaissance Europe and of figures such as Copernicus, Galileo, and Newton. Along the way, Cromer touches on many intriguing topics, arguing, for instance, that much of science is essential complete; there are no new elements yet to be discovered. He debunks the vaunted SETI (Search for Extraterrestrial Intelligence) project, which costs taxpayers millions each year, showing that physical limits--such as the melting point of metal--put an absolute limit on the speed of space travel, making trips to even the nearest star all but impossible. Finally, Cromer discusses the deplorable state of science education in America and suggests several provocative innovations to improve high school education, including a radical proposal to give all students an intensive eighth and ninth year program, eliminating the last two years of high school.Uncommon Sense is an illuminating look at science, filled with provocative observations. Whether challenging Thomas Kuhn's theory of scientific revolutions, or extolling the virtues of Euclid's Elements , Alan Cromer is always insightful, outspoken, and refreshingly original.

Cromer, Alan

1995-08-01

42

Uncommon epiloptogenic lesions affecting the temporal lobe.  

PubMed

There are several processes implicated as uncommon causes of temporal lobe epilepsy. Trauma is the leading cause of epilepsy in young adults, intracerebral blood collection being the most consistent risk factor of seizures, especially subdural hematomas and brain contusions. Infarction is the entity most commonly related to epilepsy in the elderly population. Seizures usually present as complex seizures with high recurrence between 6 months and 2 years after stroke. There are some radiological characteristics of the affectation associated with high risk of early and late seizures. Noninfectious limbic encephalitis is a syndrome characterized by seizures, memory loss, and confusion. It includes paraneoplasic and non-paraneoplasic limbic encephalitis, both presenting as hyperintense lesion affecting temporobasal regions more evident with fluid-attenuated inversion recovery sequences. Paraneoplasic limbic encephalitis is associated with several types of tumor-induced autoimmunity against the nervous system. The tumors most frequently implicated are the lungs, testis, and breast, including Hodgkin's lymphoma, teratoma, and thymoma in young patients. Once a tumor is excluded, non-paraneoplasic limbic encephalitis has to be considered by investigating the presence of antibodies against voltage-gated potassium channels. It is associated with hyponatremia and responds to regimens of steroids, plasma exchange, and intravenous immunoglobulins. Finally, herpetic limbic encephalitis is also associated with seizures, accompanied by fever and neurologic symptoms. It presents characteristic findings and distribution on magnetic resonance imaging, which shows abnormalities in more than 90% of patients with proven Herpes simplex virus type 1. PMID:18383907

Ramos, Ana; Ballenilla, Federico; Martin, Patricia

2008-02-01

43

Uncommon corrosion phenomena of archaeological bronze alloys  

NASA Astrophysics Data System (ADS)

In the framework of the EFESTUS project (funded by the European Commission, contract No. ICA3-CT-2002-10030) the corrosion products of a large number of archaeological bronze artefacts are investigated by means of the combined use of scanning electron microscopy (SEM), energy dispersive spectrometry (EDS), X-ray diffraction (XRD) and optical microscopy (OM) and tentative correlation of their nature with the chemical composition of the artefacts and the burial context is proposed. The results provide good insight into the corrosion layers and evidence in some bronze Roman coins and artefacts; the occurrence of uncommon corrosion phenomena that give rise to the formation of a yellowish-green complex chlorine-phosphate of lead (pyromorphite, (PbCl)Pb4(PO4)3) and of a gold-like thick layer of an iron and copper sulphide (chalcopyrite, CuFeS2). The micro-chemical and micro-structural results show that the coins were buried in a soil enriched in phosphorus for the accidental presence of a large amount of decomposing fragments of bones or in an anaerobic and humus rich soil where the chalcopyrite layer has been produced via the interaction between the iron of the soil, the copper of the coin and the sulphur produced by the decomposition of organic matter in an almost oxygen free environment. Finally, some unusual periodic corrosion phenomena occurring in high tin bronze mirrors found at Zama (Tunisia) are described.

Ingo, G. M.; de Caro, T.; Riccucci, C.; Khosroff, S.

2006-06-01

44

Uncommon complication after revision hip surgery.  

PubMed

Cauda equina syndrome is an uncommon complication of ankylosing spondylitis characterized by the slow and insidious development of severe neurologic impairment related to dural ectasia. This report describes a unique case of cauda equina syndrome in a patient with ankylosing spondylitis after hip revision surgery. A 70-year-old man with long-standing ankylosing spondylitis underwent standard hip revision surgery; combined spinal and general anesthesia was administered. Pain was controlled with intravenous opioids postoperatively (patient-controlled analgesia). As per routine protocol, on the first postoperative day, the patient remained supine on a hip abduction pillow; mobilization was initiated on the second postoperative day. On postoperative day 1, the patient had severe low back pain that was controlled with patient-controlled analgesia. On postoperative day 2, the Foley catheter was removed and the patient sat and dangled. Back pain persisted while supine; in addition, the patient noticed involuntary loss of urine. On postoperative day 3, the patient had below-the-knee numbness that progressed to saddle anesthesia and foot flexor and extensor weakness. An epidural hematoma was suspected and urgent magnetic resonance imaging was performed, which showed severe degenerative stenosis at the L4-L5 level (mainly by dense ligamentum flavum). An L4-L5 decompression and instrumented fusion was performed; intraoperatively, L4-L5 was found to be the sole mobile segment. The extension of the spine in the supine position that completely obliterated the spinal canal was considered the mechanism of cauda equina syndrome. The intensity of back pain is a good indicator of a severe spinal lesion; however, pain can be dampened by intravenous opioids. High suspicion is required in patients with preexisting spinal pathology, such as ankylosing spondylitis. PMID:24972447

Sakellariou, Vasileios I; Papadopoulos, Elias C; Babis, George C

2014-06-01

45

Pyramid heart: uncommon sequel of a common disease.  

PubMed

A 40-year-old woman presented 19 years after ring-annuloplastyfor rheumatic mitral regurgitation. Long-standing rheumatic heartdisease resulted in an unusual finding on her chest radiograph. PMID:22964650

Patil, Nikhil Prakash; Katti, Karuna; Yadav, Niraj; Satsangi, Deepak Kumar

2015-01-01

46

Unilateral nevoid acanthosis nigricans: Uncommon variant of a common disease  

PubMed Central

Acanthosis nigricans (AN) is a fairly common dermatosis characterized by hyperpigmented velvety plaques, having a predilection for the intertriginous areas. We herein present a case of unilateral nevoid acanthosis nigricans over the left lateral chest, in an adult male. The rarity of documentation of this entity in the world literature prompted us to report the case. PMID:25506563

Das, Anupam; Bhattacharya, Sabari; Kumar, Piyush; Gayen, Tirthankar; Roy, Kunal; Das, Nilay K.; Gharami, Ramesh C.

2014-01-01

47

Erythema nodosum - a review of an uncommon panniculitis.  

PubMed

Panniculitis, inflammation of the subcutaneous fat, is a relatively uncommon condition that usually presents with inflammatory nodules or plaques. Erythema nodosum (EN) is clinically the most frequent form of panniculitis and is considered a reactive process that may be triggered by a wide variety of stimuli. Whilst up to 55% of EN is considered idiopathic, the most common causes include infections, drugs, systemic illnesses such as sarcoidosis and inflammatory bowel disease, pregnancy, and malignancy. EN typically presents in the teens and 20s, and is seen more commonly in females. It is often preceded by a non-specific prodrome of one to three weeks, which may include fever, malaise, and symptoms of an upper respiratory tract infection. Cutaneous lesions then follow, typically localized on the extensor aspect of the limbs. The lesions are painful rounded or oval, slightly raised, non-ulcerative red nodules. The exact pathogenesis of EN is not understood, although is thought to result from deposition of immune complexes in the venules of the septae in subcutaneous fat, causing a neutrophilic panniculitis. The classical histopathological picture is of a septal panniculitis without vasculitis. However, the pathological features vary with the chronology of the lesions. Even without specific therapy for a causative condition, EN typically resolves without treatment. Therefore, symptomatic support is adequate for the majority of patients. PMID:24746312

Blake, Tristan; Manahan, Melissa; Rodins, Karl

2014-01-01

48

An uncommon cause of Ortner syndrome.  

PubMed

Ortner syndrome is a rare cause of hoarseness, resulting from compression of the left recurrent laryngeal nerve by a cardiovascular etiology. It was initially described secondary to left atrial enlargement and mitral valve disease. Other causes have now been recently described. We present a case of Ortner syndrome caused by penetrating atherosclerotic ulcers of the thoracic aorta. PMID:20463611

Mickus, Timothy J; Mueller, Jeffrey; Williams, Robert

2010-08-01

49

Uncommon opportunistic fungal infections of oral cavity: A review  

PubMed Central

The majority of opportunistic oral mucosal fungal infections are due to Candida albicans and Aspergillus fumigatus species. Mucor and Cryptococcus also have a major role in causing oral infections, whereas Geotrichum, Fusarium, Rhodotorula, Saccharomyces and Penicillium marneffei are uncommon pathogens in the oral cavity. The broad spectrum of clinical presentation includes pseudo-membranes, abscesses, ulcers, pustules and extensive tissue necrosis involving bone. This review discusses various uncommon opportunistic fungal infections affecting the oral cavity including their morphology, clinical features and diagnostic methods. PMID:25328305

Deepa, AG; Nair, Bindu J; Sivakumar, TT; Joseph, Anna P

2014-01-01

50

Uncommon gastrointestinal complications of enteric fever in a non-endemic country  

PubMed Central

Enteric fever is a systemic illness with varying presentation. It is an important infectious disease in developing countries and also in industrialized countries where many migrants reside. Enteric fever can result in complications in different organ systems and delay in identification and prompt treatment can be fatal. The important gastrointestinal complications of enteric fever include hepatitis, intestinal ulcers, bleeding and bowel perforation. We report three relatively uncommon complications of enteric fever in Qatar, a non-endemic country, ileal ulcer presenting with hematochezia; duodenal ulcer with polyserositis, cholestatic hepatitis and bone marrow suppression; enteric fever related peritonitis. PMID:25320692

Thandassery, Ragesh Babu; Sharma, Manik; Abdelmola, Abdellatif; Derbala, Moutaz F.M.; Al Kaabi, Saad Rashid

2014-01-01

51

Clinical and Radiologic Review of Uncommon Cause of Profound Iron Deficiency Anemia: Median Arcuate Ligament Syndrome  

PubMed Central

Median arcuate ligament syndrome is an anatomic and clinical entity characterized by dynamic compression of the proximal celiac artery by the median arcuate ligament, which leads to postprandial epigastric pain, vomiting, and weight loss. These symptoms are usually nonspecific and are easily misdiagnosed as functional dyspepsia, peptic ulcer disease, or gastropathy. In this report, we presented a 72-year-old male patient with celiac artery compression syndrome causing recurrent abdominal pain associated with gastric ulcer and iron deficiency anemia. This association is relatively uncommon and therefore not well determined. In addition, we reported the CT angiography findings and three-dimensional reconstructions of this rare case. PMID:25053902

Asil, K?yasettin; Aksoy, Yakup Ersel; Tatl? Ayhan, Laçin

2014-01-01

52

Uncommon Craniosynostosis Syndromes: A Review of Thirteen Conditions  

Microsoft Academic Search

Uncommon craniosynostosis syndromes, while individually less well characterized than more common conditions such as Apert, Crouzon, Pfeiffer, Muenke, and Saethre-Chotzen syndromes, comprise a significant proportion of craniosynostosis cases when considered in sum. Thirteen of these rare syndromes are covered here with respect to nosology, associated clinical characteristics, and molecular genetics. They were selected for discussion in this chapter due to

M. S. Raam; M. Muenke

2011-01-01

53

Alström syndrome – an uncommon cause of early childhood retinal dystrophy  

PubMed Central

Alström syndrome (AS) is a ciliopathy and an uncommon cause of syndromic retinal dystrophy. This case reports findings in a 5-year-old boy with severe early onset retinal dystrophy, and how the recognition of extraocular features with genetic analysis led to the correct diagnosis of AS after 4 years of investigation. PMID:22688943

Sheck, Leo; Al-Taie, Rasha; Sharp, Dianne; Vincent, Andrea

2011-01-01

54

Thoracoscopic surgery for pulmonary oncocytoma, an uncommon neoplasm.  

PubMed

Oncocytoma is an uncommon neoplasm with a characteristic histologic feature of abundant eosinophilic granular cytoplasm. It almost always occurs in kidney, thyroid, or salivary gland. Pulmonary oncocytoma is a rare pulmonary neoplasm, with fewer than 10 cases reported so far in the available English literature. We encountered one such case that was successfully managed by thoracoscopic lobectomy. PMID:24523615

Zhang, B; Jin, J; Ye, Z; Zheng, H

2014-02-01

55

Catalog of Uncommon Facilities in Western Colleges and Universities.  

ERIC Educational Resources Information Center

A list of rare, and often unique, facilities in the western part of the United States is presented that is designed to serve as a resource for researchers. The list of facilities is a guide to what uncommon equipment, centers or institutes, and collections are available at western higher education facilities and what provisions exist for sharing…

Viehland, Dennis, Comp.

56

5 CFR 630.210 - Uncommon tours of duty.  

Code of Federal Regulations, 2010 CFR

...establish an uncommon tour of duty for each firefighter compensated under part 550, subpart...duty shall correspond directly to the firefighter's regular tour of duty, as defined...1302 of this chapter, so that each firefighter accrues and uses leave on the...

2010-01-01

57

Advocacy: Emphasizing the Uncommon about the Common Core State Standards  

ERIC Educational Resources Information Center

The author describes key issues and uncommon concerns about the Common Core State Standards that fit within two categories: philosophical and pedagogical. Philosophically, Common Core State K-12 Standards should not be expected to be mastered at a specific grade level but based on developmental readiness. Pedagogically, Common Core State Standards…

Kaplan, Sandra N.

2014-01-01

58

Thoracoscopic surgery for pulmonary oncocytoma, an uncommon neoplasm  

PubMed Central

Oncocytoma is an uncommon neoplasm with a characteristic histologic feature of abundant eosinophilic granular cytoplasm. It almost always occurs in kidney, thyroid, or salivary gland. Pulmonary oncocytoma is a rare pulmonary neoplasm, with fewer than 10 cases reported so far in the available English literature. We encountered one such case that was successfully managed by thoracoscopic lobectomy. PMID:24523615

Zhang, B.; Jin, J.; Ye, Z.; Zheng, H.

2014-01-01

59

Uncommon aetiological agents of catheter-related bloodstream infections.  

PubMed

SUMMARY The clinical and microbiological characteristics of catheter-related bloodstream infection (CR-BSI) due to uncommon microorganisms was assessed in a retrospective case-control study over a 9-year period in a tertiary teaching hospital. Uncommon microorganisms were defined as those representing <0·5% of all CR-BSI. Diagnosis of CR-BSI required that the same microorganism was grown from at least one peripheral venous blood culture and a catheter tip culture. Thirty-one episodes of CR-BSI were identified due to 13 different genera and these accounted for 2·3% of all CR-BSI in the hospital. Although these infections were not associated with increased mortality, they occurred in patients with more severe underlying conditions who were receiving prolonged antibiotic therapy. PMID:24887020

Reigadas, E; Rodríguez-Créixems, M; Sánchez-Carrillo, C; Martín-Rabadán, P; Bouza, E

2015-03-01

60

Uncommon variants of the scimitar syndrome in two siblings  

PubMed Central

The Scimitar syndrome is a complex association of cardiovascular and bronchopulmonary abnormalities, with the main feature a partial or total anomalous right pulmonary venous drainage to the inferior vena cava. A number of cases that lack of all the features of the typical syndrome have been described as Scimitar variant, but the incidence is rare. Familial occurrence is exceptional and limited to few cases in literature. We report two sibling diagnosed with an uncommon variant of the Scimitar syndrome. PMID:25684890

Bo, Ilaria; Daubeney, Piers E F; Rigby, Michael L

2015-01-01

61

Capsular retraction: an uncommon imaging finding in hepatic inflammatory pseudotumour  

PubMed Central

Capsular retraction is an infrequent but characteristic feature of malignant liver lesions such as hepatic metastases and intrahepatic cholangiocarcinoma. Rarely, this finding may be observed in association with benign lesions, such as atypical haemangiomas. Capsular retraction has not previously been reported in association with hepatic inflammatory pseudotumour (IPT). Hepatic IPT is an uncommon benign hepatic lesion with a good clinical prognosis. In this report, we discuss the case of a 48-year-old woman with capsular retraction secondary to multifocal hepatic inflammatory IPTs. PMID:19934068

Ganesan, K; Viamonte, B; Peterson, M; Kono, Y; Santillan, C; Middleton, M; Sirlin, C

2009-01-01

62

Acute Esophageal Necrosis: An Uncommon Cause of Hematemesis  

PubMed Central

Acute esophageal necrosis or black esophagus is an uncommon clinical entity, diagnosed at the upper gastrointestinal endoscopy with the presence of strikingly black necrotic esophagus. Very often no definite etiology will be identified even though a large list of potential associations has been postulated. Upper gastrointestinal bleeding is the most common clinical presentation, others being epigastric pain, retrosternal chest discomfort and dysphagia. Only about a hundred cases of acute esophageal necrosis have been described in medical literature till this date. We report a case of acute esophageal necrosis in an elderly female who had presented with hematemesis. PMID:25170416

Zacharia, George Sarin; Sandesh, K; Ramachandran, TM

2014-01-01

63

Common variable immunodeficiency disorder - An uncommon cause for bronchiectasis.  

PubMed

Bronchiectasis continues to be a common respiratory problem of varied etiology. Common variable immunodeficiency disorder (CVID) is an uncommon cause for bronchiectasis. However, the prevalence of bronchiectasis remains very high in patients with CVID. This remains largely an underdiagnosed entity as primary immunodeficiency is not suspected in adults as a cause of bronchiectasis and hence, serum immunoglobulin (Ig) levels are not measured routinely. In addition to bronchiectasis, patients with CVID usually present with various extrapulmonary symptoms. I report here a case of young man who presented with bronchiectasis and multisystem complains who was diagnosed as CVID. PMID:25378851

Panigrahi, Manoj Kumar

2014-10-01

64

Lipofibromatosis: an institutional and literature review of an uncommon entity.  

PubMed

We report six new cases of lipofibromatosis, an uncommon pediatric soft tissue neoplasm. This is the only series of patients to be described since the initial case series of 45 patients that characterized this entity in 2000. The purpose of this study was to characterize the presentation of lipofibromatosis to further define the clinical phenotype of this rare entity. Six patients were diagnosed with lipofibromatosis at our institution from 2000 to 2012. Patient age, sex, and ethnicity were recorded, along with tumor site and size, management, and recurrence data. Half of our patients were younger than 2 years old at presentation and the other half were school age. Boys and girls were affected with equal frequency. In five of six patients, lipofibromatosis presented in its "classic" form as a mass on the distal extremities. These tumors typically measured 1 to 2 cm in diameter, in contrast to case reports in the medical literature highlighting the occurrence of lipofibromatosis of greater size and at varied anatomic sites. The tumors in our series were managed using excision, with recurrence noted in 33%. Lipofibromatosis is an uncommon tumor typically found on the distal extremities of infants, although it can appear in various sizes and locations. It should be considered in the differential diagnosis of pediatric soft tissue neoplasms. PMID:24758203

Boos, Markus D; Chikwava, Kudakwashe R; Dormans, John P; Chauvin, Nancy A; Jen, Melinda

2014-01-01

65

European and German food legislation facing uncommon foodstuffs.  

PubMed

In Europe, uncommon foodstuff (UFS, i.e., traditional foods from specific European regions and uncommon ethnic foods from non-EU countries) have been contributing to a diversification of the food supply. E-commerce and specialized retail shops are the main sources for UFS. This article discusses the legal bases for UFS introduction and evaluation. By means of 35 representative UFS, this article analyses the possibilities of trade and veterinary inspection of these products in Germany, comparing European Union and national food legislation with the many idiosyncrasies the UFS presents. Conservatory legislation bans the trade with endangered species (primates, cetaceans, songbirds), but for many other species, this is a complex matter that may ban only subpopulations from trade. Although introduction of legal UFS is regulated (yet complicated), the lack of appropriate definitions, intra-European trade harmonization, and of sufficient scientific knowledge hampers a satisfactory evaluation of many UFSs, for example, reptile meat or terrestrial insects. In these cases, official inspection would only be very basic. PMID:23768143

Grabowski, Nils Th; Klein, Günter; López, Antonio Martínez

2013-01-01

66

Inferior olivary hypertrophy is uncommon in progressive supranuclear palsy.  

PubMed

Inferior olivary hypertrophy (IOH) is an uncommon disorder attributed to transsynaptic degeneration and characterized clinically by palatal myoclonus and histopathologically by swollen, fenestrated neurons and bizarre astrocytic gliosis. IOH is usually associated with the interruption of the dentato-olivary tract, most often from cerebrovascular lesions in the dentate nucleus or red nucleus. In progressive supranuclear palsy (PSP), the dentato-olivary tract is consistently affected, and there are reports suggesting that IOH may be relatively common in PSP. To address this issue, the frequency of IOH was investigated in 264 PSP brains. To determine if there was a relationship between IOH and neurofibrillary degeneration in the dentato-olivary pathway, the severity of neurofibrillary degeneration was assessed in the inferior olivary nucleus, cerebellar dentate nucleus and red nucleus in PSP cases with and without IOH. IOH was uncommon in PSP, being found in only 4 of 264 (1.5%) brains, which was not significantly different from the frequency in non-PSP controls (8 of 862; 0.9%). In all non-PSP cases IOH was associated with cerebrovascular lesions, but not in any of the PSP cases with IOH. The severity of neurofibrillary degeneration in the inferior olivary nucleus was significantly greater in PSP cases with IOH than in PSP cases without IOH, whereas there were no significant differences in the cerebellar dentate nucleus and red nucleus. These findings suggest that direct neurofibrillary degeneration in the inferior olivary nucleus may be related to IOH in PSP rather than transsynaptic degeneration. PMID:15235807

Katsuse, Omi; Dickson, Dennis W

2004-08-01

67

Multi-institute study finds uncommon BRAF melanoma mutation  

Cancer.gov

An uncommon mutation of the BRAF gene in melanoma patients has been found to respond to MEK inhibitor drugs, providing a rationale for routine screening and therapy in melanoma patients who harbor the BRAF L597 mutation. Mutations in BRAF V600E or KIT are common in about 40 percent to 50 percent of melanomas, and drugs that block or inhibit BRAF V600E were recently approved for treatment of melanoma patients with these mutations. However, there has been no effective treatment for patients with wildtype (WT) melanoma that is negative for these driver mutations. This study included researchers from the Vanderbilt-Ingram Cancer Center, UCLA Jonsson Comprehensive Cancer Center, and Memorial Sloan-Kettering Cancer Center.

68

Recognizing Uncommon Presentations of Psychogenic (Functional) Movement Disorders  

PubMed Central

Background Psychogenic or functional movement disorders (PMDs) pose a challenge in clinical diagnosis. There are several clues, including sudden onset, incongruous symptoms, distractibility, suggestibility, entrainment of symptoms, and lack of response to otherwise effective pharmacological therapies, that help identify the most common psychogenic movements such as tremor, dystonia, and myoclonus. Methods In this manuscript, we review the frequency, distinct clinical features, functional imaging, and neurophysiological tests that can help in the diagnosis of uncommon presentations of PMDs, such as psychogenic parkinsonism, tics, and chorea; facial, palatal, and ocular movements are also reviewed. In addition, we discuss PMDs at the extremes of age and mass psychogenic illness. Results Psychogenic parkinsonism (PP) is observed in less than 10% of the case series about PMDs, with a female–male ratio of roughly 1:1. Lack of amplitude decrement in repetitive movements and of cogwheel rigidity help to differentiate PP from true parkinsonism. Dopamine transporter imaging with photon emission tomography can also help in the diagnostic process. Psychogenic movements resembling tics are reported in about 5% of PMD patients. Lack of transient suppressibility of abnormal movements helps to differentiate them from organic tics. Psychogenic facial movements can present with hemifacial spasm, blepharospasm, and other movements. Some patients with essential palatal tremor have been shown to be psychogenic. Convergence ocular spasm has demonstrated a high specificity for psychogenic movements. PMDs can also present in the context of mass psychogenic illness or at the extremes of age. Discussion Clinical features and ancillary studies are helpful in the diagnosis of patients with uncommon presentations of psychogenic movement disorders. PMID:25667816

Baizabal-Carvallo, José Fidel; Fekete, Robert

2015-01-01

69

Typical uses of certain common and uncommon plants.  

PubMed

The beginning of medicinal uses of plants dates back to the scribing period of Vedas in India. In ancient days, such uses came into vogue due to accidental experimentation or observation which subsequently gave rise to practice either by a qualified physician or by an astrologer or by lay men, called nostrum or folk-lore. All these have their own distinct manner of use though they are very often intermingled. Apart from classical uses as mentioned in Ayurvedic, Unani or Sidha therapeutic treatises, material medica, texts on / pharmacy etc.; the new dimension of collecting additional information started in early part of 20(th) century where on several botanists contributed a lot for exploration of the same.Since Orissa is a treasure of folk-core claims and besides qualified practitioners, certain lay men especially in rural area and tribal area, saints and priests at different places also possess knowledge on certain typical uses of several plants, there is a larger scope to highlight the same for future study from difference angles. In this paper an attempt has been made to highlight certain newer information's on certain common and uncommon plants like Lygodium flexuosum, Vitex peduncularis, Barleria lupulina, Leptadenia reticulate, Selaginella indica, etc., collected from different parts of Orissa. PMID:22557106

Hota, N P; Padhi, M M

2003-07-01

70

TYPICAL USES OF CERTAIN COMMON AND UNCOMMON PLANTS  

PubMed Central

The beginning of medicinal uses of plants dates back to the scribing period of Vedas in India. In ancient days, such uses came into vogue due to accidental experimentation or observation which subsequently gave rise to practice either by a qualified physician or by an astrologer or by lay men, called nostrum or folk-lore. All these have their own distinct manner of use though they are very often intermingled. Apart from classical uses as mentioned in Ayurvedic, Unani or Sidha therapeutic treatises, material medica, texts on / pharmacy etc.; the new dimension of collecting additional information started in early part of 20th century where on several botanists contributed a lot for exploration of the same. Since Orissa is a treasure of folk-core claims and besides qualified practitioners, certain lay men especially in rural area and tribal area, saints and priests at different places also possess knowledge on certain typical uses of several plants, there is a larger scope to highlight the same for future study from difference angles. In this paper an attempt has been made to highlight certain newer information's on certain common and uncommon plants like Lygodium flexuosum, Vitex peduncularis, Barleria lupulina, Leptadenia reticulate, Selaginella indica, etc., collected from different parts of Orissa. PMID:22557106

Hota, N. P.; Padhi, M.M.

2003-01-01

71

Ewing’s Sarcoma: An Uncommon Breast Tumor  

PubMed Central

Ewing’s sarcoma/primitive neuroectodermal tumors (EWS/PNET) are rare malignant and aggressive tumors, usually seen in the trunk and lower limbs of children and young adults. They are uncommon in the breast. We report a case of a 43-year-old woman who developed a painless breast mass. An initial core needle biopsy concluded to a fibrocystic dystrophy contrasting with a rapidly growing mass; thus a large lumpectomy was done. Diagnosis of primary PNET of the breast was established, based on both histopathological examination and immunohistochemical findings. Surgical margins were positive, therefore, left modified radical mastectomy with axillary lymph nodes dissection was performed. The patient was given 6 cycles of adjuvant chemotherapy containing cyclophosphamide, adriamycin and vincristine. Twenty months later, she is in life without recurrence or metastasis. EWS/PNET may impose a diagnostic challenge. Indeed, mammography and ultrasonography features are non specific. The histopathological pattern is variable depending on the degree of neuroectodermal differentiation. Immuno-phenotyping is necessary and genetic study is the only confirmatory tool of diagnosis showing a characteristic cytogenetic anomaly; t (11; 22) translocation. PMID:25332765

Meddeb, Sawsen; Rhim, Mohamed Salah; Kouira, Mouna; Mestiri, Sarra; Bibi, Mohamed; Yacoubi, Mohamed Tahar

2014-01-01

72

An uncommon case of Marine-Lenhart syndrome.  

PubMed

The term Marine-Lenhart syndrome describes the association between Graves' disease and autonomously functioning thyroid nodules (AFTN), such as toxic adenoma or toxic multinodular goiter. The two diseases may coexist or may be present at different moments in the same patient. In the literature, there are many reports on the development of Graves' disease after radioiodine treatment for AFTN, but very little information may be found on the occurrence of AFTN after radioiodine therapy for Graves' disease. We describe here the case of a female patient with Graves' disease who was successfully treated with radioiodine for Graves' disease, returning to normal thyroid function. Three years later, biochemical analysis and ultrasound examination identified a thyroid nodule that progressively increased in size. The 99mTc-pertechnetate scintigraphy showed avid uptake in the right lobule, which corresponded to a nodular lesion consistent with AFTN. PMID:24936736

Giuffrida, Giuseppe; Giovinazzo, Salvatore; Certo, Rosaria; Vicchio, Teresa Manuela; Baldari, Sergio; Campenně, Alfredo; Ruggeri, Rosaria Maddalena

2014-06-01

73

Lymphoepithelioma-like Hepatocellular Carcinoma: An Uncommon Variant of Hepatocellular Carcinoma With Favorable Outcome.  

PubMed

Lymphoepithelioma-like hepatocellular carcinoma (LEL-HCC) is an uncommon variant of HCC with only 22 cases reported in the literature. To better determine the incidence, clinicopathologic features, prognostic significance, and molecular pathogenesis of LEL-HCC, we presented the largest series of LEL-HCC from a 9-year retrospective cohort of patients with HCC undergoing surgical resection. LEL-HCC was identified in 20 patients (4.9%). Compared with patients having HCC without significant tumor-infiltrating lymphocyte (TIL), patients with LEL-HCC had a relatively lower frequency of male sex (P=0.022), tended to present at early-stage disease (80.0% vs. 56.3% as AJCC stage I, P=0.037; 100% vs. 77.3% as BCLC stage 0/A, P=0.010), and all harbored a solitary tumor only (P=0.006). There was no significant difference in the age at presentation, underlying chronic liver disease, cirrhotic background, serum ?-fetoprotein level, tumor size, histologic grade, and frequencies of vascular invasion. Most of the TILs in LEL-HCC were cytotoxic T lymphocytes. None of the LEL-HCCs was associated with Epstein-Barr virus. LEL-HCC was associated with better overall (5-y survival: 94.1% vs. 63.9%; P=0.007) and progression-free (5-y survival: 87.8% vs. 46.6%; P=0.002) survivals compared with HCC without significant TIL. The multivariate analysis revealed that LEL-HCC was an independent prognostic factor for overall and progression-free survivals. The adjusted hazard ratio of cancer death and tumor progression for LEL-HCC was 0.12 (P=0.037) and 0.14 (P=0.002), respectively. LEL-HCC did not differ in frequencies of microsatellite instability, BRAF mutation, and DNA hypermethylation. In brief, LEL-HCC is a distinct uncommon variant of HCC characterized by dense cytotoxic T-cell infiltration and favorable prognosis. PMID:25675010

Chan, Anthony W H; Tong, Joanna H M; Pan, Yi; Chan, Stephen L; Wong, Grace L H; Wong, Vincent W S; Lai, Paul B S; To, Ka-Fai

2015-03-01

74

The Uncommon Case of Jahi McMath.  

PubMed

A 13-year-old patient named Jahi McMath was determined to be dead by neurologic criteria following cardiopulmonary arrest and resuscitation at a hospital in Oakland, California. Her family did not agree that she was dead and refused to allow her ventilator to be removed. The family's attorney stated in the media that families, rather than physicians, should decide whether patients are dead and argued in the courts that the families' constitutional rights of religion and privacy would be violated otherwise. Ultimately, a judge agreed that the patient was dead in keeping with California law, but the constitutional issue was undecided. The patient was then transferred to a hospital in New Jersey, a state whose laws allow families to require on religious grounds that death be determined by cardiopulmonary criteria. Although cases such as this are uncommon, they demonstrate public confusion about the concept of neurologic death and the rejection of this concept by some families. The confusion may be caused in part by a lack of uniformity in state laws regarding the legal basis of death, as reflected in the differences between New Jersey and California statutes. Families who reject the determination of death by neurologic criteria on religious grounds should be given reasonable accommodation in all states, but society should not pay for costly treatments for patients who meet these criteria unless the state requires it, as only New Jersey does. Laws that give physicians the right to determine death by neurologic criteria in other states probably can survive a constitutional challenge. Physicians and hospitals faced with similar cases in the future should follow state laws and work through the courts if necessary. PMID:25846530

Luce, John M

2015-04-01

75

Predicting mortality for paediatric inpatients where malaria is uncommon  

PubMed Central

Objective As the proportion of children living low malaria transmission areas in sub-Saharan Africa increases, approaches for identifying non-malarial severe illness need to be evaluated to improve child outcomes. Design As a prospective cohort study, we identified febrile paediatric inpatients, recorded data using Integrated Management of Childhood Illness (IMCI) criteria, and collected diagnostic specimens. Setting Tertiary referral centre, northern Tanzania. Results Of 466 participants with known outcome, median age was 1.4 years (range 2 months–13.0 years), 200 (42.9%) were female, 11 (2.4%) had malaria and 34 (7.3%) died. Inpatient death was associated with: Capillary refill >3 s (OR 9.0, 95% CI 3.0 to 26.7), inability to breastfeed or drink (OR 8.9, 95% CI 4.0 to 19.6), stiff neck (OR 7.0, 95% CI 2.8 to 17.6), lethargy (OR 5.2, 95% CI 2.5 to 10.6), skin pinch >2 s (OR 4.8, 95% CI 1.9 to 12.3), respiratory difficulty (OR 4.0, 95% CI 1.9 to 8.2), generalised lymphadenopathy (OR 3.6, 95% CI 1.6 to 8.3) and oral candidiasis (OR 3.4, 95% CI 1.4 to 8.3). BCS <5 (OR 27.2, p<0.001) and severe wasting (OR 6.9, p<0.001) were independently associated with inpatient death. Conclusions In a low malaria transmission setting, IMCI criteria performed well for predicting inpatient death from non-malarial illness. Laboratory results were not as useful in predicting death, underscoring the importance of clinical examination in assessing prognosis. Healthcare workers should consider local malaria epidemiology as malaria over-diagnosis in children may delay potentially life-saving interventions in areas where malaria is uncommon. PMID:22872067

Clifton, Dana C; Ramadhani, Habib O; Msuya, Levina J; Njau, Boniface N; Kinabo, Grace D; Buchanan, Ann M; Crump, John A

2012-01-01

76

Cutaneous manifestations of gastrointestinal diseases  

Microsoft Academic Search

There are a myriad of dermatologic disorders asso- ciated with gastrointestinal (GI) diseases. This article covers the common dermatologic conditions that may be associated with underlying GI diseases and several uncommon conditions that the dermatologist should recognize as being associated with GI disor- ders. Table 1 presents an outline of the diseases that are covered. Inflammatory bowel diseases Inflammatory disorders

Erin E. Boh; Raed Mahmoud

77

Cytodiagnosis of Epidermoid Cyst of the Upper Lip: A Common Lesion in an Uncommon Site  

PubMed Central

Epidermoid cyst and dermoid cysts are developmental pathologies thought to derive from aberrant ectodermal tissue. They are uncommon in the head and neck region. Rarely, they can be found in the oral cavity and buccal mucosa. However, epidermoid cyst is extremely uncommon in the upper lip and is rarely reported. In this study, we report an uncommon case of epidermoid cyst occurring in the upper lip diagnosed by fine-needle aspiration cytology (FNAC). We present this case because of its extremely rare site of presentation and also to highlight the role of FNAC to in the pre-operative diagnosis of this benign lesion. PMID:24696565

Phukan, Jyoti Prakash; Sinha, Anuradha; Pal, Subrata; Jalan, Shilpa

2014-01-01

78

A clinical and radiologic review of uncommon types and causes of pancreatitis.  

PubMed

Acute pancreatitis is one of the most common conditions for which emergent imaging is indicated. Alcohol consumption and cholelithiasis are the most common causes of acute pancreatitis in adults, whereas the majority of cases in children are idiopathic or secondary to trauma. A wide variety of structural and biochemical abnormalities may also cause pancreatitis. Although in some cases it is difficult to identify the specific cause of the disease radiologically, certain uncommon types of acute or chronic pancreatitis may have unique imaging features that can help the radiologist make an accurate diagnosis. These unusual types include autoimmune pancreatitis, groove pancreatitis, tropical pancreatitis, hereditary pancreatitis, and pancreatitis in ectopic or heterotopic pancreatic tissue. Pancreatitis may occasionally be seen in association with cystic fibrosis or pancreas divisum, or secondary to worm infestation of the pancreaticobiliary tree (eg, by Ascaris lumbricoides). In addition, primary pancreatic and duodenal masses may occasionally manifest as acute or recurrent acute pancreatitis. Knowledge of the classic imaging findings of these entities allows prompt recognition of the relevant pathologic condition, thereby preventing misdiagnosis and subsequent inappropriate or delayed management. PMID:19605653

Shanbhogue, Alampady Krishna Prasad; Fasih, Najla; Surabhi, Venkateswar R; Doherty, Geoffrey P; Shanbhogue, Divya Krishna Prasad; Sethi, Sumer K

2009-01-01

79

Orchestration of an Uncommon Maturation Cascade of the House Dust Mite Protease Allergen Quartet  

PubMed Central

In more than 20% of the world population, sensitization to house dust mite allergens triggers typical allergic diseases such as allergic rhinitis and asthma. Amongst the 23 mite allergen groups hitherto identified, group 1 is cysteine proteases belonging to the papain-like family whereas groups 3, 6, and 9 are serine proteases displaying trypsin, chymotrypsin, and collagenolytic activities, respectively. While these proteases are more likely to be involved in the mite digestive system, they also play critical roles in the initiation and in the chronicity of the allergic response notably through the activation of innate immune pathways. All these allergenic proteases are expressed in mite as inactive precursor form. Until recently, the exact mechanisms of their maturation into active proteases remained to be fully elucidated. Recent breakthroughs in the understanding of the activation mechanisms of mite allergenic protease precursors have highlighted an uncommon and unique maturation pathway orchestrated by group 1 proteases that tightly regulates the proteolytic activities of groups 1, 3, 6, and 9 through complex intra- or inter-molecular mechanisms. This review presents and discusses the currently available knowledge of the activation mechanisms of group 1, 3, 6, and 9 allergens of Dermatophagoides pteronyssinus laying special emphasis on their localization, regulation, and interconnection. PMID:24744761

Dumez, Marie-Eve; Herman, Julie; Campizi, Vincenzo; Galleni, Moreno; Jacquet, Alain; Chevigné, Andy

2014-01-01

80

Acromegaly presenting as hirsuitism: Uncommon sinister aetiology of a common clinical sign  

PubMed Central

Hirsuitism though not uncommon (24%), is not considered to be a prominent feature of acromegaly because of its lack of specificity and occurrence. Hirsuitism is very common in women of reproductive age (5-7%) and has been classically associated with polycystic ovarian syndrome (PCOS). Twenty-eight year lady with 3 year duration of hirsuitism (Modified Ferriman Gallwey score-24/36), features of insulin resistance (acanthosis), subtle features of acromegaloidism (woody nose and bulbous lips) was diagnosed to have acromegaly in view of elevated IGF-1 (1344 ng/ml; normal: 116-358 ng/ml), basal (45.1 ng/ml) and post glucose growth hormone (39.94 ng/ml) and MRI brain showing pituitary macroadenoma. Very high serum androstenedione (>10 ng/ml; normal 0.5-3.5 ng/ml), elevated testosterone (0.91 ng/ml, normal <0.8) and normal dehydroepiandrosterone sulphate (DHEAS) (284 mcg/dl, normal 35-430 mcg/dl) along with polycystic ovaries on ultrasonography lead to diagnosis of associated PCOS. She was also diagnosed to have diabetes. This case presentation intends to highlight that hirsuitism may rarely be the only prominent feature of acromegaly. A lookout for subtle features of acromegaly in all patients with hirsuitism and going for biochemical evaluation (even at the risk of investigating many patients of insulin resistance and acromegloidism) may help us pick up more patients of acromegaly at an earlier stage thus help in reducing disease morbidity. PMID:23565405

Jain, Rajesh; Dutta, Deep; Shivaprasad, KS; Maisnam, Indira; Ghosh, Sujoy; Mukhopadhyay, Satinath; Chowdhury, Subhankar

2012-01-01

81

Bartonella spp.: throwing light on uncommon human infections.  

PubMed

After 2 decades of Bartonella research, knowledge on transmission and pathology of these bacteria is still limited. Bartonella spp. have emerged to be important pathogens in human and veterinary medicine. For humans, B. henselae is considered to represent the most relevant zoonotic Bartonella species and is responsible for cat scratch disease, bacillary angiomatosis, and other disorders. Over the years, many Bartonella species have been isolated from humans, cats, dogs, and other mammals, and infections range from an asymptomatic state (e.g., animal-specific species) to even life-threatening diseases (e.g., Oroya fever). It is obvious that the analysis of pathogenicity mechanisms underlying Bartonella infections is needed to increase our understanding of how these pathogens adapt to their mammalian hosts resulting in acute or chronic diseases. PMID:20833105

Kaiser, Patrick O; Riess, Tanja; O'Rourke, Fiona; Linke, Dirk; Kempf, Volkhard A J

2011-01-01

82

Genomic Duplication of PTPN11 is an Uncommon Cause of Noonan Syndrome  

PubMed Central

Noonan syndrome (NS) is a genetically heterogeneous disorder caused most commonly by activating mutations in PTPN11. We report a patient with hypotonia, developmental delay and clinical features suggestive of NS. High-resolution chromosome analysis was normal, and sequence analyses of PTPN11, SOS1, KRAS, BRAF, RAF1, MEK, and MEK2 were also normal. Array CGH revealed a single copy gain of 9 BAC clones at 12q24.11q24.21 (8.98 Mb in size), which encompassed the PTPN11 locus at 12q24.13 and was confirmed by FISH analysis. Shchelochkov et al., [2008] reported a similar case and speculated that such duplications might account for 15–30% of NS cases with no detectable mutation in NS genes. We screened more than 250 NS cases without mutation in known NS disease-causing genes by quantitative PCR, and none of these studies produced results in the duplicated range. We also explored the possibility that de novo changes affecting the untranslated region (UTR) of the PTPN11 transcript might represent an alternative event involved in SHP2 enhanced expression. DHPLC analysis and direct sequencing of the entire 3' UTR in 36 NS patients without mutation in known genes did not show any disease-associated variant. These findings indicate that duplications of PTPN11 represent an uncommon cause of NS, and functionally relevant variations within the 3'UTR of the gene do not appear to play a major role in NS. However, recurrent observations of NS in individuals with duplications involving the PTPN11 locus suggest that increased dosage of SHP2 may have dysregulating effects on intracellular signaling. PMID:19760651

Graham, John M.; Kramer, Nancy; Bejjani, Bassem A.; Thiel, Christian T.; Carta, Claudio; Neri, Giovanni; Tartaglia, Marco; Zenker, Martin

2009-01-01

83

Hiccups, eructation, and other uncommon prodromal manifestations of herpes zoster  

Microsoft Academic Search

Although the most frequent presentation of herpes zoster involves sensory neurons, motor and autonomic symptomatology is also known to occur in this disease. An unusual symptom of hiccups is described here. Other infrequent manifestations of this common illness, including the Ramsay Hunt syndrome, herpes zoster ophthalmicus, urinary and fecal retention, sexual dysfunction, and zoster sine herpete, are reviewed. Greater awareness

Alexander L. Berlin; Channy Y. Muhn; Robin C. Billick

2003-01-01

84

Tubercular liver abscess: an uncommon presentation of disseminated tuberculosis.  

PubMed

We present a case of tubercular liver abscess with disseminated tuberculosis, associated with underlying HIV infection. The patient responded well to percutaneous drainage of the abscess and first-line quadruple antitubercular therapy. We report this case to highlight a rare manifestation of a common disease and to create greater awareness which may ensure timely diagnosis and avoid unnecessary surgical intervention. PMID:25429791

Carrara, E; Brunetti, E; Di Matteo, A; Ravetta, V; Minoli, L; Youkee, D

2015-04-01

85

Hiccups, eructation, and other uncommon prodromal manifestations of herpes zoster.  

PubMed

Although the most frequent presentation of herpes zoster involves sensory neurons, motor and autonomic symptomatology is also known to occur in this disease. An unusual symptom of hiccups is described here. Other infrequent manifestations of this common illness, including the Ramsay Hunt syndrome, herpes zoster ophthalmicus, urinary and fecal retention, sexual dysfunction, and zoster sine herpete, are reviewed. Greater awareness of unusual presentations of herpes zoster is necessary for proper diagnosis and timely management of complications that may otherwise lead to disability and serious long-term sequelae. PMID:14639397

Berlin, Alexander L; Muhn, Channy Y; Billick, Robin C

2003-12-01

86

Coeliac disease in children of West Indian origin.  

PubMed Central

Coeliac disease is uncommon in populations of non-European origin. Two English born West Indian children with coeliac disease are presented. The diagnosis should be considered in children of West Indian origin with chronic diarrhoea. PMID:7574866

Hung, J C; Phillips, A D; Walker-Smith, J A

1995-01-01

87

Uncommon and rarely reported adverse events of endoscopic retrograde cholangiopancreatography.  

PubMed

Endoscopic retrograde cholangiopancreatography (ERCP) has become a primary tool for the treatment of biliary and pancreatic ductal diseases. It is essential for the endoscopist carrying out the ERCP to have a thorough understanding of the potential adverse events. Typically, endoscopists are well familiar with common adverse events such as post-ERCP pancreatitis, cholangitis, post-sphincterotomy bleeding, post-sphincterotomy perforation, and sedation-related cardiopulmonary compromises. However, there are other less common adverse events that arecritical to promptly recognize in order to provide appropriate therapy and prevent disastrous outcomes. This review focuses on the presentation and management of the less common and rare adverse events of an ERCP from the perspective of the practicing endoscopist. PMID:24118211

Chavalitdhamrong, Disaya; Donepudi, Suman; Pu, Liping; Draganov, Peter V

2014-01-01

88

Intramedullary craniovertebral junction tuberculoma: An uncommon location of a common disease.  

PubMed

Central nervous system involvement is observed in no more than 10% of patients with systemic tuberculosis. Although CNS tuberculosis is not rare in endemic countries, such as India, intramedullary tuberculosis is not commonly reported. In this study, we report a case of a 40-year-old female who presented with a six-year history of insidious onset, gradually progressive, asymmetric quadriparesis. She was diagnosed with intramedullary tuberculoma at the craniovertebral junction and showed significant clinico-radiological improvement with medical management alone. To the best of our knowledge, this report describes the first case of intramedullary tuberculoma at the craniovertebral junction to be reported. With the increased availability of MRI in developing countries, it is now possible for clinicians to diagnose this condition without performing a biopsy. It is important for the clinicians in developed countries to be highly suspicious of intramedullary tuberculoma, as there has been a resurgence of CNS tuberculosis due to the emergence of the HIV pandemic. PMID:24930634

Jain, Rajendra S; Handa, Rahul; Srivastava, Trilochan; Prakash, Swayam; Nagpal, Kadam; Bhana, Indu; Gupta, Pankaj K

2014-01-01

89

Uncommon types of polyglucosan bodies in the human brain: distribution and relation to disease  

Microsoft Academic Search

The significance of the development of polyglucosan bodies (PBs) in the CNS is incompletely understood. We present the clinicopathological features of three autopsy cases with numerous PBs other than the common corpora amylacea or Lafora bodies. The first patient had pleomorphic PBs in the neuronal processes of pallidum and substantia nigra which, thus, are consistent with Bielschowsky bodies. Bielschowsky bodies

H. Sugiyama; J. A. Hainfellner; H. Lassmann; S. Indravasu; H. Budka

1993-01-01

90

Torsion of the Retroperitoneal Kidney: Uncommon or Underreported?  

PubMed Central

Vascular torsion in a renal allograft after placement in the retroperitoneum is rare and has only been reported twice in the literature. It is an extrinsically mediated process that occurs at the vascular pedicle resulting in graft compromise and potential loss. Rapid diagnosis and immediate surgical intervention may salvage allograft function. Herein, we present a unique case of a 42-year-old male that developed renal allograft torsion following a second kidney transplant placed in the retroperitoneum. Immediate detorsion did not resolve allograft dysfunction, and a biopsy revealed acute cellular mediated rejection. After antithymocyte globulin treatment, allograft function was salvaged. A review of the current literature shows that the incidence, morbidity, and long term allograft function of intraperitoneal and extraperitoneal torsion are different. As such, torsion of the retroperitoneal kidney demonstrates encouraging allograft salvage rates. Only the third case reported to date, this serves as a contribution to the growing body of literature in retroperitoneal renal torsion and reviews the risks, medication considerations, diagnostic tests, and treatment modalities in a unique disease process. PMID:24551473

Sosin, Michael; Lumeh, Wuya; Cooper, Matthew

2014-01-01

91

Uncommon Applications of Deep Brain Stimulation in Hyperkinetic Movement Disorders  

PubMed Central

Background In addition to the established indications of tremor and dystonia, deep brain stimulation (DBS) has been utilized less commonly for several hyperkinetic movement disorders, including medication-refractory myoclonus, ballism, chorea, and Gilles de la Tourette (GTS) and tardive syndromes. Given the lack of adequate controlled trials, it is difficult to translate published reports into clinical use. We summarize the literature, draw conclusions regarding efficacy when possible, and highlight concerns and areas for future study. Methods A Pubmed search was performed for English-language articles between January 1980 and June 2014. Studies were selected if they focused primarily on DBS to treat the conditions of focus. Results We identified 49 cases of DBS for myoclonus-dystonia, 21 for Huntington's disease, 15 for choreacanthocytosis, 129 for GTS, and 73 for tardive syndromes. Bilateral globus pallidus interna (GPi) DBS was the most frequently utilized procedure for all conditions except GTS, in which medial thalamic DBS was more common. While the majority of cases demonstrate some improvement, there are also reports of no improvement or even worsening of symptoms in each condition. The few studies including functional or quality of life outcomes suggest benefit. A limited number of studies included blinded on/off testing. There have been two double-blind controlled trials performed in GTS and a single prospective double-blind, uncontrolled trial in tardive syndromes. Patient characteristics, surgical target, stimulation parameters, and duration of follow-up varied among studies. Discussion Despite these extensive limitations, the literature overall supports the efficacy of DBS in these conditions, in particular GTS and tardive syndromes. For other conditions, the preliminary evidence from small studies is promising and encourages further study. PMID:25713746

Smith, Kara M.; Spindler, Meredith A.

2015-01-01

92

Small bowel obstruction in the emergency department: MDCT features of common and uncommon causes.  

PubMed

Small bowel obstruction is a common condition encountered by the radiologist in the emergency department. Though intestinal adhesions and obstructed hernias are the most common causes of acute small bowel obstruction, a variety of uncommon and rare conditions can cause acute and subacute bowel obstruction. Imaging plays a key role in the workup of bowel obstruction by identifying the cause, level, and degree of bowel obstruction. In this article, we present a comprehensive review of the multi-detector computed tomography features of common and uncommon causes of acute and subacute small bowel obstruction. PMID:24861419

Tirumani, Harika; Vassa, Ravi; Fasih, Najla; Ojili, Vijayanadh

2014-01-01

93

Autism is uncommon in 22q: the how and why of wrong diagnoses  

E-print Network

Autism is uncommon in 22q: the how and why of wrong diagnoses Kathleen Angkustsiri, Khyati Disclosures · Co-investigator on clinical trials in autism and fragile X syndrome ­Novartis ­Roche ­Seaside Pharmaceuticals ­Forest Laboratories #12;22q and Autism Spectrum Disorders · Autism Spectrum Disorders

Nguyen, Danh

94

Labio-Patalization in Twi: Contrastive, Quantal, and Organizational Factors Producing an Uncommon Sound.  

ERIC Educational Resources Information Center

Proposes that the typologically uncommon combination of labial and palatal constriction in Twi has arisen from a convergence created by general patterns of coarticulation of consonants and vowels. This convergence has been systematized in a consonantal acoustic dimension partially independent from the original vocalic dimensions of contrast for…

de Jong, Kenneth; Obeng, Samuel Gyasi

2000-01-01

95

Anasarca as the initial presentation of juvenile polymyositis: an uncommon occurrence  

Microsoft Academic Search

Polymyositis is uncommon in childhood. Unlike dermatomyositis, which is common in pediatric age group, skin involvement is\\u000a lacking in polymyositis. We report an 8-year-old boy who presented with anasarca as the initial complaint. This presentation\\u000a has been rarely reported before in the pediatric literature.

Dhrubajyoti Sharma; Surjit Singh; Deepti Suri; Amit Rawat; Upender Shava; Kushaljit S. Sodhi

96

Mesenteric Inflammatory Venoocclusive Disease in a Patient with Sjögren's Syndrome  

PubMed Central

Mesenteric inflammatory venoocclusive disease is an uncommon cause of intestinal ischemia. Certain diseases, such as hypercoagulation disorders, autoimmune diseases, or drugs have been associated with the pathogenesis of mesenteric inflammatory venoocclusive disease. Here, we report a patient with Sjögren's syndrome who underwent surgery for suspected acute appendicitis with a subsequent pathological diagnosis of mesenteric inflammatory venoocclusive disease. PMID:25477970

Rios-Fernández, Raquel; Callejas-Rubio, José-Luis; Caba-Molina, Mercedes; Ríos-Peregrina, Rosa; Ortego-Centeno, Norberto

2014-01-01

97

A Rare Case of Unrecognized and Uncommon Bladder Perforation after Transobturator Tape Procedure  

PubMed Central

The transobturator tape (TOT) procedure has become practically widespread worldwide. Complications seem to be rare, but recognizing them intraoperatively is the most significant step because some of the complications which may appear in postoperative period can be challenging for both physicians and patients. The purpose of this case, with this patient who was operated on with open surgery, is to evaluate this rarely seen unrecognized and uncommon bladder perforation after TOT procedure and thus make some contribution to the literature. Here, we present a case report about the treatment of a 48-year-old woman patient with unrecognized and uncommon bladder perforation after TOT procedure, 5 months postoperatively. Cystoscopic evaluation is not recommended routinely, but it must be performed if the patient is complicated enough to create doubt and also the surgeon's skill and ability are not sufficient enough to operate decently. PMID:25685154

K?l?nç, Ercüment; Akpak, Ya?am Kemal

2015-01-01

98

Distribution Patterns of Ohio Stoneflies, with an Emphasis on Rare and Uncommon Species  

PubMed Central

Presently, 102 stonefly species (Plecoptera) have been reported from Ohio. All 9 Nearctic families are represented. Over 90% of the fauna exhibit a combination of broad Nearctic-widespread, eastern Nearctic-widespread, Appalachian, and eastern Nearctic-unglaciated distributions. In contrast, only 2 species display a central Nearctic-Prairie distribution. Seven species of Perlidae are likely no longer present (Acroneuria evoluta Klapálek, A. perplexa Frison, Attaneuria ruralis (Hagen), and Neoperla mainensis Banks) or have experienced marked range reductions (Acroneuria abnormis (Newman), A. frisoni Stark and Brown, and A. filicis Frison). Another nearly 31% of the fauna (32 species) are rare, uncommon, or have highly-limited distributions within the state. Twelve of these species have Appalachian distributions, and an additional 8 have eastern Nearctic-unglaciated distributions. The distributional status for each of the 32 rare/uncommon species is discussed. PMID:24219390

Grubbs, Scott A.; Pessimo, Massimo; DeWalt, R. Edward

2013-01-01

99

Meta-analysis of Dense Genecentric Association Studies Reveals Common and Uncommon Variants Associated with Height  

PubMed Central

Height is a classic complex trait with common variants in a growing list of genes known to contribute to the phenotype. Using a genecentric genotyping array targeted toward cardiovascular-related loci, comprising 49,320 SNPs across approximately 2000 loci, we evaluated the association of common and uncommon SNPs with adult height in 114,223 individuals from 47 studies and six ethnicities. A total of 64 loci contained a SNP associated with height at array-wide significance (p < 2.4 × 10?6), with 42 loci surpassing the conventional genome-wide significance threshold (p < 5 × 10?8). Common variants with minor allele frequencies greater than 5% were observed to be associated with height in 37 previously reported loci. In individuals of European ancestry, uncommon SNPs in IL11 and SMAD3, which would not be genotyped with the use of standard genome-wide genotyping arrays, were strongly associated with height (p < 3 × 10?11). Conditional analysis within associated regions revealed five additional variants associated with height independent of lead SNPs within the locus, suggesting allelic heterogeneity. Although underpowered to replicate findings from individuals of European ancestry, the direction of effect of associated variants was largely consistent in African American, South Asian, and Hispanic populations. Overall, we show that dense coverage of genes for uncommon SNPs, coupled with large-scale meta-analysis, can successfully identify additional variants associated with a common complex trait. PMID:21194676

Lanktree, Matthew B.; Guo, Yiran; Murtaza, Muhammed; Glessner, Joseph T.; Bailey, Swneke D.; Onland-Moret, N. Charlotte; Lettre, Guillaume; Ongen, Halit; Rajagopalan, Ramakrishnan; Johnson, Toby; Shen, Haiqing; Nelson, Christopher P.; Klopp, Norman; Baumert, Jens; Padmanabhan, Sandosh; Pankratz, Nathan; Pankow, James S.; Shah, Sonia; Taylor, Kira; Barnard, John; Peters, Bas J.; M. Maloney, Cliona; Lobmeyer, Maximilian T.; Stanton, Alice; Zafarmand, M. Hadi; Romaine, Simon P.R.; Mehta, Amar; van Iperen, Erik P.A.; Gong, Yan; Price, Tom S.; Smith, Erin N.; Kim, Cecilia E.; Li, Yun R.; Asselbergs, Folkert W.; Atwood, Larry D.; Bailey, Kristian M.; Bhatt, Deepak; Bauer, Florianne; Behr, Elijah R.; Bhangale, Tushar; Boer, Jolanda M.A.; Boehm, Bernhard O.; Bradfield, Jonathan P.; Brown, Morris; Braund, Peter S.; Burton, Paul R.; Carty, Cara; Chandrupatla, Hareesh R.; Chen, Wei; Connell, John; Dalgeorgou, Chrysoula; Boer, Anthonius de; Drenos, Fotios; Elbers, Clara C.; Fang, James C.; Fox, Caroline S.; Frackelton, Edward C.; Fuchs, Barry; Furlong, Clement E.; Gibson, Quince; Gieger, Christian; Goel, Anuj; Grobbee, Diederik E.; Hastie, Claire; Howard, Philip J.; Huang, Guan-Hua; Johnson, W. Craig; Li, Qing; Kleber, Marcus E.; Klein, Barbara E.K.; Klein, Ronald; Kooperberg, Charles; Ky, Bonnie; LaCroix, Andrea; Lanken, Paul; Lathrop, Mark; Li, Mingyao; Marshall, Vanessa; Melander, Olle; Mentch, Frank D.; J. Meyer, Nuala; Monda, Keri L.; Montpetit, Alexandre; Murugesan, Gurunathan; Nakayama, Karen; Nondahl, Dave; Onipinla, Abiodun; Rafelt, Suzanne; Newhouse, Stephen J.; Otieno, F. George; Patel, Sanjey R.; Putt, Mary E.; Rodriguez, Santiago; Safa, Radwan N.; Sawyer, Douglas B.; Schreiner, Pamela J.; Simpson, Claire; Sivapalaratnam, Suthesh; Srinivasan, Sathanur R.; Suver, Christine; Swergold, Gary; Sweitzer, Nancy K.; Thomas, Kelly A.; Thorand, Barbara; Timpson, Nicholas J.; Tischfield, Sam; Tobin, Martin; Tomaszweski, Maciej; Verschuren, W.M. Monique; Wallace, Chris; Winkelmann, Bernhard; Zhang, Haitao; Zheng, Dongling; Zhang, Li; Zmuda, Joseph M.; Clarke, Robert; Balmforth, Anthony J.; Danesh, John; Day, Ian N.; Schork, Nicholas J.; de Bakker, Paul I.W.; Delles, Christian; Duggan, David; Hingorani, Aroon D.; Hirschhorn, Joel N.; Hofker, Marten H.; Humphries, Steve E.; Kivimaki, Mika; Lawlor, Debbie A.; Kottke-Marchant, Kandice; Mega, Jessica L.; Mitchell, Braxton D.; Morrow, David A.; Palmen, Jutta; Redline, Susan; Shields, Denis C.; Shuldiner, Alan R.; Sleiman, Patrick M.; Smith, George Davey; Farrall, Martin; Jamshidi, Yalda; Christiani, David C.; Casas, Juan P.; Hall, Alistair S.; Doevendans, Pieter A.; D. Christie, Jason; Berenson, Gerald S.; Murray, Sarah S.; Illig, Thomas; Dorn, Gerald W.; Cappola, Thomas P.; Boerwinkle, Eric; Sever, Peter; Rader, Daniel J.; Reilly, Muredach P.; Caulfield, Mark; Talmud, Philippa J.; Topol, Eric; Engert, James C.; Wang, Kai; Dominiczak, Anna; Hamsten, Anders; Curtis, Sean P.; Silverstein, Roy L.; Lange, Leslie A.; Sabatine, Marc S.; Trip, Mieke; Saleheen, Danish; Peden, John F.; Cruickshanks, Karen J.; März, Winfried; O'Connell, Jeffrey R.; Klungel, Olaf H.; Wijmenga, Cisca; Maitland-van der Zee, Anke Hilse; Schadt, Eric E.; Johnson, Julie A.; Jarvik, Gail P.; Papanicolaou, George J.; Grant, Struan F.A.; Munroe, Patricia B.; North, Kari E.; Samani, Nilesh J.; Koenig, Wolfgang; Gaunt, Tom R.; Anand, Sonia S.; van der Schouw, Yvonne T.; Soranzo, Nicole; FitzGerald, Garret A.; Reiner, Alex; Hegele, Robert A.; Hakonarson, Hakon; Keating, Brendan J.

2011-01-01

100

Moral identity and the experience of moral elevation in response to acts of uncommon goodness.  

PubMed

Four studies using survey and experimental designs examined whether people whose moral identity is highly self-defining are more susceptible to experiencing a state of moral elevation after being exposed to acts of uncommon moral goodness. Moral elevation consists of a suite of responses that motivate prosocial action tendencies. Study 1 showed that people higher (vs. lower) in moral identity centrality reported experiencing more intense elevating emotions, had more positive views of humanity, and were more desirous of becoming a better person after reading about an act of uncommon goodness than about a merely positive situation or an act of common benevolence. Study 2 showed that those high in moral identity centrality were more likely to recall acts of moral goodness and experience moral elevation in response to such events more strongly. These experiences were positively related to self-reported prosocial behavior. Study 3 showed a direct effect on behavior using manipulated, rather than measured, moral identity centrality. Study 4 replicated the effect of moral identity on the states of elevation as well as on self-reported physical sensations and showed that the elevation mediates the relationship between moral identity, witnessing uncommon goodness, and prosocial behavior. PMID:21443375

Aquino, Karl; McFerran, Brent; Laven, Marjorie

2011-04-01

101

A severe foodborne outbreak of diarrhoea linked to a canteen in Italy caused by enteroinvasive Escherichia coli, an uncommon agent.  

PubMed

We describe a foodborne outbreak in Italy caused by enteroinvasive Escherichia coli (EIEC), an enteric pathogen uncommon in industrialized countries. On 14 April 2012 a number of employees of the city of Milan Fire Brigade (FB) were admitted to hospital with severe diarrhoea after attending their canteen. Thirty-two patients were hospitalized and a total of 109 cases were identified. A case-control study conducted on 83 cases and 32 controls attending the canteen without having symptoms identified cooked vegetables to be significantly associated with the disease. Stool samples collected from 62 subjects were screened for enteric pathogens using PCR-based commercial kits: 17 cases and two asymptomatic kitchen-workers were positive for the Shigella marker gene ipaH; an ipaH-positive EIEC strain O96:H19 was isolated from six cases. EIEC may cause serious dysentery-like outbreaks even in Western European countries. Microbiologists should be aware of microbiological procedures to detect EIEC, to be applied especially when no common enteric pathogens are identified. PMID:24534429

Escher, M; Scavia, G; Morabito, S; Tozzoli, R; Maugliani, A; Cantoni, S; Fracchia, S; Bettati, A; Casa, R; Gesu, G P; Torresani, E; Caprioli, A

2014-12-01

102

Gorham's disease of femur  

PubMed Central

Gorham's disease is a rare condition of bones characterized by spontaneous massive and progressive osteolysis. Less than 200 cases have been reported so far. Femur is an uncommon site of Gorham disease. We report a young female, presenting as pathological fracture of the femur, wherein rapid osteolysis of femur occurred. The clinical, pathological, and radiological picture suggested a diagnosis of Gorham's disease. The patient was subjected to radiotherapy (40 Gys) and put on bisphosphonates. At a follow-up of 2 years, the disease process had stopped and partial recalcification of the bone had occurred. PMID:22144753

Baba, Asif Nazir; Bhat, Yasmeen J; Paljor, Simon D; Nazir, Abid; Khan, Nazir A

2011-01-01

103

Sodium channel gene mutations in hypokalemic periodic paralysis: an uncommon cause in the UK.  

PubMed

Eleven of 36 families with hypokalemic periodic paralysis (hypoPP) harbored mutations in the skeletal muscle calcium channel gene (CACNA1S). The authors screened the skeletal muscle sodium channel gene (SCN4A) in the remainder. One family harbored a new heterozygous point mutation C2014A in exon 12 (R672S) of SCN4A. The authors identified the genetic defect underlying hypoPP in 33% of individuals tested. The authors conclude that SCN4A mutations are an uncommon cause of hypoPP in this UK population. PMID:11591859

Davies, N P; Eunson, L H; Samuel, M; Hanna, M G

2001-10-01

104

An unusual case of cardiogenic shock: common cause from uncommon etiology.  

PubMed

Mechanical complications of an acute coronary syndrome can lead to hemodynamic instability out of proportion to the degree of left ventricular dysfunction. We present the case of a patient with cardiogenic shock secondary to severe mitral regurgitation in the setting of an acutely occluded obtuse marginal artery. Echocardiography and pathologic findings revealed an uncommon cause of anterolateral papillary muscle rupture. Using the unique features of this case, we present a clinical self-assessment exercise highlighting the challenges involved in the management of this type of patient. PMID:24882542

Giles, Katie M; Rao, Rajeev V; Ascah, Kathryn J; Veinot, John P; Lam, Buu-Khanh; Froeschl, Michael

2014-06-01

105

Pneumonia by Listeria monocytogenes: A Common Infection by an Uncommon Pathogen  

PubMed Central

Infections by Listeria monocytogenes typically occur in infants, the elderly, pregnant women, and immunosuppressed subjects. Pulmonary infections in adults are extremely uncommon and only few reports can be found in the literature. We here report a case of Listeria pneumonia in an 85-year-old female patient and we discuss our diagnostic and therapeutic approach. Despite being rare and in most cases difficult to be identified, Listeria pneumonia should always be considered in immunosuppressed patients, presenting with fever and symptoms from the lower respiratory system. PMID:25802774

Koufakis, Theocharis; Chatzopoulou, Marianneta; Margaritis, Anastasios; Tsiakalou, Maria; Gabranis, Ioannis

2015-01-01

106

[Placenta percreta with bladder invasion: an uncommon cause of hematuria during pregnancy].  

PubMed

Placenta percreta with bladder invasion is a very uncommon condition that can lead to very severe complications in pregnant women. Although it is often diagnosed during delivery, imaging techniques are very useful for early diagnosis, which is fundamental for planning surgery and avoiding potentially lethal complications. We present the case of a woman with a history of cesarean section who presented with hematuria and low back pain. The diagnosis of placenta percreta with bladder invasion was suggested after ultrasonography and magnetic resonance imaging and was confirmed at surgery. We provide a brief review of the literature, emphasizing the role of imaging techniques. PMID:23523415

Sierra-Solís, A; Leo-Barahona, M; Romero-López, A I; Gómez-Guerrero, J M

2014-01-01

107

Uncommon Breeding Birds in North Dakota: Population Estimates and Frequencies of Occurrence  

NSDL National Science Digital Library

The Northern Prairie Wildlife Research Center (NPWRC) has posted five newly online resources at the Center's homepage. These scientific articles were originally published in print journals, but are fully (and freely) available here, complete with figures. The final paper is by authors Lawrence D. Igl, Douglas H. Johnson, and Harold A. Kantrud, and was originally published in 1999 in Canadian field-Naturalist [113(4):646-651]. The paper presents statewide population estimates and frequencies of occurrence of uncommon species, based on surveys conducted in 1967, 1992, and 1993. All papers may be downloaded as .zip files.

108

An uncommon treatment of totally extruded and lost talus: a case report  

PubMed Central

Introduction Total extrusion and loss of the talus is a rare injury with a wide choice of appropriate treatment, but rarely resulting in a fully functional recovery. We report on an uncommon case, both for the severity of the injury and for the uncommon treatment due to the patient’s rejection of secondary surgery. Case presentation We treated a 16-year-old Caucasian man with the most extreme variant of a totally extruded and lost talus, accompanied with complex injury of the soft tissues of the ankle and foot. The treatment included urgent microvascular foot reimplantation, microvascular muscle free flap transfer, and temporary fixation. This kind of injury should typically be treated by tibiocalcaneal arthrodesis. However, this was not performed, as after the successful early stages of the treatment he strongly objected to another surgery due to his fully functional status and the successful therapeutic results of our early treatment. Conclusions The injury described in this case study would ordinarily be treated by amputation, but due to the well-executed treatment in the early stages after the injury, the outcome was satisfying. Surprisingly and against our expectations, the late results of the treatment were successful even without arthrodesis. He is now 37 years old and has a functional foot 21 years after the injury. PMID:25266945

2014-01-01

109

The Cyborg Astrobiologist: scouting red beds for uncommon features with geological significance  

NASA Astrophysics Data System (ADS)

The `Cyborg Astrobiologist' has undergone a second geological field trial, at a site in northern Guadalajara, Spain, near Riba de Santiuste. The site at Riba de Santiuste is dominated by layered deposits of red sandstones. The Cyborg Astrobiologist is a wearable computer and video camera system that has demonstrated a capability to find uncommon interest points in geological imagery in real time in the field. In this second field trial, the computer vision system of the Cyborg Astrobiologist was tested at seven different tripod positions, on three different geological structures. The first geological structure was an outcrop of nearly homogeneous sandstone, which exhibits oxidized-iron impurities in red areas and an absence of these iron impurities in white areas. The white areas in these `red beds' have turned white because the iron has been removed. The iron removal from the sandstone can proceed once the iron has been chemically reduced, perhaps by a biological agent. In one instance the computer vision system found several (iron-free) white spots to be uncommon and therefore interesting, as well as several small and dark nodules. The second geological structure was another outcrop some 600 m to the east, with white, textured mineral deposits on the surface of the sandstone, at the bottom of the outcrop. The computer vision system found these white, textured mineral deposits to be interesting. We acquired samples of the mineral deposits for geochemical analysis in the laboratory. This laboratory analysis of the crust identifies a double layer, consisting of an internal millimetre-size layering of calcite and an external centimetre-size efflorescence of gypsum. The third geological structure was a 50 cm thick palaeosol layer, with fossilized root structures of some plants. The computer vision system also found certain areas of these root structures to be interesting. A quasi-blind comparison of the Cyborg Astrobiologist's interest points for these images with the interest points determined afterwards by a human geologist shows that the Cyborg Astrobiologist concurred with the human geologist 68% of the time (true-positive rate), with a 32% false-positive rate and a 32% false-negative rate. The performance of the Cyborg Astrobiologist's computer vision system was by no means perfect, so there is plenty of room for improvement. However, these tests validate the image-segmentation and uncommon-mapping technique that we first employed at a different geological site (Rivas Vaciamadrid) with somewhat different properties for the imagery.

McGuire, Patrick Charles; Díaz-Martínez, Enrique; Ormö, Jens; Gómez-Elvira, Javier; Rodríguez-Manfredi, José Antonio; Sebastián-Martínez, Eduardo; Ritter, Helge; Haschke, Robert; Oesker, Markus; Ontrup, Jörg

2005-04-01

110

Apomictic parthenogenesis in a parasitoid wasp Meteorus pulchricornis, uncommon in the haplodiploid order Hymenoptera.  

PubMed

Although apomixis is the most common form of parthenogenesis in diplodiploid arthropods, it is uncommon in the haplodiploid insect order Hymenoptera. We found a new type of spontaneous apomixis in the Hymenoptera, completely lacking meiosis and the expulsion of polar bodies in egg maturation division, on the thelytokous strain of a parasitoid wasp Meteorus pulchricornis (Wesmael) (Braconidae, Euphorinae) on pest lepidopteran larvae Spodoptera litura (Fabricius) (Noctuidae). The absence of the meiotic process was consistent with a non-segregation pattern in the offspring of heterozygous females, and no positive evidence was obtained for the induction of thelytoky by any bacterial symbionts. We discuss the conditions that enable the occurrence of such rare cases of apomictic thelytoky in the Hymenoptera, suggesting the significance of fixed heterosis caused by hybridization or polyploidization, symbiosis with bacterial agents, and occasional sex. Our finding will encourage further genetic studies on parasitoid wasps to use asexual lines more wisely for biological control. PMID:24521569

Tsutsui, Y; Maeto, K; Hamaguchi, K; Isaki, Y; Takami, Y; Naito, T; Miura, K

2014-06-01

111

Loss of PTEN expression is not uncommon, but lacks prognostic value in stage I NSCLC.  

PubMed

The PTEN gene is a tumor-suppressor gene that is inactivated in several types of human tumors. The loss of PTEN expression has been supported as a prognostic marker. Using immunohistochemical analysis, we retrospectively analyzed PTEN expression in specimens from 53 patients with completely resected stage I non-small cell lung cancer (NSCLC) for whom clinical follow-up data were available. Seven of the 53 specimens showed a total lack of staining for PTEN. No statistically significant relationship could be found between PTEN expression and clinicopathological parameters. Although genetic alterations of the PTEN gene are rare in NSCLC, loss of PTEN protein is not an uncommon event in early-stage NSCLC (13.2%). Here, we also report that the level of PTEN protein expression is not an independent prognostic marker in early-stage NSCLC. PMID:14981940

Olaussen, Ken Andre; Soria, Jean-Charles; Morat, Luc; Martin, Antoine; Sabatier, Laure; Morere, Jean-Francois; Khayat, David; Spano, Jean-Philippe

2003-01-01

112

An uncommon disorder with multiple skeletal anomalies: Gorlin-Goltz syndrome.  

PubMed

Gorlin-Goltz syndrome is an uncommon disorder transmitted through autosomal dominant inheritance. This syndrome is characterized by multiple odontogenic keratocysts, along with congenital skeletal anomalies and basal cell carcinomas. A 16-year-old girl was admitted with a complaint of swelling on the lower jaw. She had multiple basal cell nevi on both hands. Multiple lytic bone lesions on radiographs were defined as odontogenic keratocysts following the biopsy. The patient was referred to the Department of Physical Medicine and Rehabilitation for detailed musculoskeletal evaluation. Adam's forward bend test revealed a hump on the right side representing right thoracic scoliosis. Cervical kyphosis, thoracic lordosis and scoliosis, bifid rib and sacral and lumbar spina bifida on plain radiographs led to the diagnosis of Gorlin-Goltz syndrome. Since early diagnosis may provide optimum surveillance for related neoplasms, it is of paramount importance for pediatricians as well as physicians dealing with the musculoskeletal system to be aware of this rare condition. PMID:25818966

Keçeli, Onur; Coskun-Benliday?, ?lke; Benliday?, M Emre; Erdo?an, Özgür

2014-01-01

113

Treatment of uncommon sites of focal primary hyperhidrosis: experience with pharmacological therapy using oxybutynin  

PubMed Central

OBJECTIVES: Primary hyperhidrosis usually affects the hands, armpits, feet and cranio-facial region. Sweating in other areas is common in secondary hyperhidrosis (after surgery or in specific clinical conditions). Oxybutynin has provided good results and is an alternative for treating hyperhidrosis at common sites. Our aim was to evaluate the efficacy of oxybutynin as a treatment for primary sweating at uncommon sites (e.g., the back and groin). METHODS: This retrospective study analyzed 20 patients (10 females) who received oxybutynin for primary focal hyperhidrosis at uncommon sites. The subjects were evaluated to determine quality of life before beginning oxybutynin and six weeks afterward and they were assigned grades (on a scale from 0 to 10) to measure their improvement at each site of excessive sweating after six weeks and at the last consult. RESULTS: The median follow-up time with oxybutynin was 385 days (133-1526 days). The most common sites were the back (n?=?7) and groin (n?=?5). After six weeks, the quality of life improved in 85% of the subjects. Dry mouth was very common and was reported by 16 patients, 12 of whom reported moderate/severe dry mouth. Five patients stopped treatment (two: unbearable dry mouth, two: excessive somnolence and one: palpitations). At the last visit, 80% of patients presented with moderate/great improvement at the main sites of sweating. CONCLUSION: After six weeks, more than 80% of the patients presented with improvements in their overall quality of life and at the most important site of sweating. Side effects were common (80% reported at least one side effect) and caused 25% of the patients to discontinue treatment. Oxybutynin is effective for treating bothersome hyperhidrosis, even at atypical locations and most patients cope well with the side effects. PMID:25318092

Teivelis, Marcelo Passos; Wolosker, Nelson; Krutman, Mariana; Kauffman, Paulo; de Campos, José Ribas Milanez; Puech-Leăo, Pedro

2014-01-01

114

Uncommon Pathways of Immune Escape Attenuate HIV-1 Integrase Replication Capacity  

PubMed Central

An attenuation of the HIV-1 replication capacity (RC) has been observed for immune-mediated escape mutations in Gag restricted by protective HLA alleles. However, the extent to which escape mutations affect other viral proteins during natural infection is not well understood. We generated recombinant viruses encoding plasma HIV-1 RNA integrase sequences from antiretroviral-naďve individuals with early (n = 88) and chronic (n = 304) infections and measured the in vitro RC of each. In contrast to data from previous studies of Gag, we observed little evidence that host HLA allele expression was associated with integrase RC. A modest negative correlation was observed between the number of HLA-B-associated integrase polymorphisms and RC in chronic infection (R = ?0.2; P = 0.003); however, this effect was not driven by mutations restricted by protective HLA alleles. Notably, the integrase variants S119R, G163E, and I220L, which represent uncommon polymorphisms associated with HLA-C*05, -A*33, and -B*52, respectively, correlated with lower RC (all q < 0.2). We identified a novel C*05-restricted epitope (HTDNGSNF114–121) that likely contributes to the selection of the S119R variant, the polymorphism most significantly associated with lower RC in patient sequences. An NL4-3 mutant encoding the S119R polymorphism displayed a ?35%-reduced function that was rescued by a single compensatory mutation of A91E. Together, these data indicate that substantial HLA-driven attenuation of integrase is not a general phenomenon during HIV-1 adaptation to host immunity. However, uncommon polymorphisms selected by HLA alleles that are not conventionally regarded to be protective may be associated with impaired protein function. Vulnerable epitopes in integrase might therefore be considered for future vaccine strategies. PMID:22496233

Chopera, Denis R.; Olvera, Alex; Brumme, Chanson J.; Sela, Jennifer; Markle, Tristan J.; Martin, Eric; Carlson, Jonathan M.; Le, Anh Q.; McGovern, Rachel; Cheung, Peter K.; Kelleher, Anthony D.; Jessen, Heiko; Markowitz, Martin; Rosenberg, Eric; Frahm, Nicole; Sanchez, Jorge; Mallal, Simon; John, Mina; Harrigan, P. Richard; Heckerman, David; Brander, Christian; Walker, Bruce D.; Brumme, Zabrina L.

2012-01-01

115

Mammary and extramammary Paget's disease  

PubMed Central

Mammary and extramammary Paget's disease are uncommon intraepithelial adenocarcinomas. Both conditions have similar clinical features, which mimic inflammatory and infective diseases. Histological diagnostic confusion can arise between Paget's disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant melanoma and atypical squamous disease. The glandular differentiation of both mammary Paget's disease and extramammary Paget's disease is indicated by morphological appearances, the presence of intracellular mucin in many cases, and positive immunohistochemical staining for glandular cytokeratins, epithelial membrane antigen, and carcinoembryonic antigen. This article provides an overview of mammary and extramammary Paget's disease and discusses recent evidence regarding the cell of origin. The concepts of primary and secondary Paget's disease are presented and the differential diagnosis is discussed with reference to immunohistochemical markers that might be of diagnostic value. Key Words: mammary Paget's disease • extramammary Paget's disease PMID:11064666

Lloyd, J; Flanagan, A

2000-01-01

116

An Uncommon VisionBY TED BOSCIA The Human Ecology Commons--the expansive interior space linking  

E-print Network

> > > T An Uncommon VisionBY TED BOSCIA The Human Ecology Commons--the expansive interior space linking historic Martha Van Rensselaer Hall and the new Human Ecology Building--is fast becoming the heart at Gruzen Samton to plan a space that reflects such core values of Human Ecology as sustainability

Chen, Tsuhan

117

Languages of Eastern Europe and the Soviet Union. A Survey of Materials for the Study of the Uncommonly Taught Languages.  

ERIC Educational Resources Information Center

This is an annotated bibliography of basic tools of access for the study of the uncommonly taught languages of Eastern Europe and the Soviet Union. It is one of eight fascicles which constitute a revision of "A Provisional Survey of Materials for the Study of the Neglected Languages" (CAL 1969). Although the focus is on materials for the…

Johnson, Dora E.; And Others

118

Four uncommon nanocage-based Ln-MOFs: highly selective luminescent sensing for Cu˛? ions and selective CO? capture.  

PubMed

Four uncommon isostructural nanocage-based 3D Ln-MOFs, 1-Ln (Ln = Eu, Tb, Gd and Dy), were constructed using a new diisophthalate ligand with active pyridyl sites. 1-Eu exhibits highly efficient luminescent sensing for Cu(2+) ions and selective CO2 capture. PMID:24963971

Liu, Bo; Wu, Wei-Ping; Hou, Lei; Wang, Yao-Yu

2014-08-14

119

Lichen Planus Is an Uncommon Cause of Nonspecific Proximal Esophageal Inflammation  

PubMed Central

Background/Aims Esophageal lichen planus (LP) has been described as a cause of nonspecific esophagitis that may cause dysphagia, but its incidence is unknown. We aimed to estimate the incidence of esophageal LP in a defined geographic region and describe the clinical characteristics of affected patients. Methods A histopathology database for a population of 1 million people was searched for all esophageal mucosal biopsy results over an 8-year period. Cases showing inflammation or abnormalities without a diagnosis after three or more biopsies were reviewed for findings of LP. Results Of 13,589 esophageal biopsies, only one received a diagnosis of LP. Seven patients (four male; mean age, 59 years; range, 39 to 76 years) were identified as having chronic dysphagia and nonspecific proximal esophagitis for which no diagnosis could be made. All patients had proximal inflammation, and six of seven had full-thickness lymphocytic infiltration. Elongation of the lamina propria papillae was noted in all patients, whereas six patients had parakeratosis and ballooning. Only one patient had findings potentially consistent with, but not sufficient for, a diagnosis of esophageal LP. Conclusions Esophageal LP appears to be extremely uncommon in this North American population, and esophageal biopsy alone is likely not sufficient to establish a diagnosis of LP. PMID:23898378

Linton, Matthew S.; Zhao, Liena; Gui, Xianyong; Storr, Martin

2013-01-01

120

Pseudoperforation: an uncommon histologic phenomenon in prurigo misleading for the diagnosis of reactive perforating collagenosis.  

PubMed

Prurigo is a common skin condition characterized by vigorous scratching. Although ulceration is not uncommon in prurigo, a perforating-like lesion was not previously reported. In this study we described series of cases of prurigo with perforating-like lesions and discussed its relation to acquired perforating dermatoses. The study included 32 cases, during the period from 2008 to 2013. Clinical data and histological features were recorded and analyzed. The study included 78.1% males and 21.9% females with a mean age of 39.3 ± 5.61 years. History of insect bite was evident in 28.1%, hepatitis C virus infection in 46.9%, and diabetes mellitus in 9.4% of patients. Histologically, well developed lesions showed full thickness epidermal degeneration overlay by a cup-shaped crater. The contents of the crater included collagen and elastic fibers, bacterial colonies, inflammatory cells and necrotic keratin. The dermis showed non-altered collagen, increased vascularity and mixed inflammatory infiltrate. We believe that this pseudoperforation process is a secondary response to vigorous scratching in prurigo patients and not a primary mechanism as occurred in perforating dermatoses. The absence of altered collagen, the presence of full thickness epidermal necrosis and concomitant elimination of elastic fibers are significant histologic clues for differentiation between both conditions. PMID:25042386

El-Khalawany, Mohamed; Saudi, Wael; Kamel, Abeer; Fawzy, Sameh; Hassan, Hatem

2014-12-01

121

A Reappraisal of the Diagnostic and Therapeutic Management of Uncommon Histologies of Primary Ocular Adnexal Lymphoma  

PubMed Central

Lymphoma is the most common malignancy arising in the ocular adnexa, which includes conjunctiva, lachrymal gland, lachrymal sac, eyelids, orbit soft tissue, and extraocular muscles. Ocular adnexal lymphoma (OAL) accounts for 1%–2% of non-Hodgkin lymphoma and 5%–15% of extranodal lymphoma. Histology, stage, and primary localizations are the most important variables influencing the natural history and therapeutic outcome of these malignancies. Among the various lymphoma variants that could arise in the ocular adnexa, marginal zone B-cell lymphoma (OA-MZL) is the most common one. Other types of lymphoma arise much more rarely in these anatomical sites; follicular lymphoma is the second most frequent histology, followed by diffuse large B-cell lymphoma and mantle cell lymphoma. Additional lymphoma entities, like T-cell/natural killer cell lymphomas and Burkitt lymphoma, only occasionally involve orbital structures. Because they are so rare, related literature mostly consists of anecdotal cases included within series focused on OA-MZL and sporadic case reports. This bias hampers a global approach to clinical and molecular properties of these types of lymphoma, with a low level of evidence supporting therapeutic options. This review covers the prevalence, clinical presentation, behavior, and histological and molecular features of uncommon forms of primary OAL and provides practical recommendations for therapeutic management. PMID:23814042

Govi, Silvia; Licata, Giada; Mappa, Silvia; Giordano Resti, Antonio; Politi, Letterio S.; Spagnuolo, Lorenzo; Di Cairano, Eliana; Doglioni, Claudio; Ferreri, Andrés J. M.

2013-01-01

122

Uncommon formation of two antiparallel sperm bundles per cyst in tenebrionid beetles (Coleoptera)  

NASA Astrophysics Data System (ADS)

Several species of Tenebrionidae are stored-grain pests. Since they belong to a specious family, the systematics of these beetles is still in doubt. In insects, spermatogenesis and the spermatozoa exhibit great diversity, and are therefore commonly used in phylogenetic and taxonomic analyses. During the spermatogenetic process in these organisms, the cells originating from a single spermatogonium develop synchronically in groups referred to as cysts. At the end of this process, there is usually only one sperm bundle per cyst, with all the cells in the same orientation. This paper details the spermiogenesis of the tenebrionid beetles Tenebrio molitor, Zophobas confusa, Tribolium castaneum and Palembus dermestoides using whole mount and histological sections of the cysts. In these species, spermatogenesis is similar to that which occurs in most insects. However, during spermiogenesis, the nuclei of the spermatids migrate to two opposite regions at the periphery of the cyst, leading to the uncommon formation of two bundles of spermatozoa per cyst. This feature is possibly an apomorphy for Tenebrionidae.

Dias, Glenda; Yotoko, Karla S. C.; Gomes, Luiz F.; Lino-Neto, José

2012-09-01

123

Unusual Presentation of Uncommon Disease: Anorexia Nervosa Presenting as Wernicke-Korsakoff Syndrome—A Case Report from Southeast Asia  

PubMed Central

Anorexia nervosa presenting as Wernicke-Korsakoff syndrome is rare. The causes of Wernicke-Korsakoff syndrome are multiple like alcohol abuse, thyrotoxicosis, haemodialysis, severe malnutrition because of gastric carcinoma and pyloric obstruction, hyperemesis gravidarum, and prolonged parenteral feeding. We report a case of anorexia nervosa, who presented with Wernicke's encephalopathy and progressed to Korsakoff's syndrome. Knowledge, awareness, and early intervention of anorexia nervosa by mental health professionals can prevent development of Wernicke-Korsakoff syndrome. PMID:24963430

Mushtaq, Raheel; Shah, Tabindah; Bhat, Mudasir; Mushtaq, Sahil

2014-01-01

124

Drug-Induced Nephrotoxicity in Inflammatory Bowel Disease  

Microsoft Academic Search

Conservative management of inflammatory bowel disease (IBD) is based on a combination of drugs, including aminosalicylates (ASAs), steroids, antibiotics, immunosuppressives and biologic agents. Although various side effects have been related to treatment regimens, drug-induced nephrotoxicity is rather uncommon. Furthermore, it is often underestimated since renal function deterioration may be attributed to the underlying disease. The nephrotoxicity of ASAs and cyclosporine

Konstantinos A. Oikonomou; Andreas N. Kapsoritakis; Ioannis Stefanidis; Spyros P. Potamianos

2011-01-01

125

Positioning patients for spine surgery: Avoiding uncommon position-related complications  

PubMed Central

Positioning patients for spine surgery is pivotal for optimal operating conditions and operative-site exposure. During spine surgery, patients are placed in positions that are not physiologic and may lead to complications. Perioperative peripheral nerve injury (PPNI) and postoperative visual loss (POVL) are rare complications related to patient positioning during spine surgery that result in significant patient disability and functional loss. PPNI is usually due to stretch or compression of the peripheral nerve. PPNI may present as a brachial plexus injury or as an isolated injury of single nerve, most commonly the ulnar nerve. Understanding the etiology, mechanism and pattern of injury with each type of nerve injury is important for the prevention of PPNI. Intraoperative neuromonitoring has been used to detect peripheral nerve conduction abnormalities indicating peripheral nerve stress under general anesthesia and to guide modification of the upper extremity position to prevent PPNI. POVL usually results in permanent visual loss. Most cases are associated with prolonged spine procedures in the prone position under general anesthesia. The most common causes of POVL after spine surgery are ischemic optic neuropathy and central retinal artery occlusion. Posterior ischemic optic neuropathy is the most common cause of POVL after spine surgery. It is important for spine surgeons to be aware of POVL and to participate in safe, collaborative perioperative care of spine patients. Proper education of perioperative staff, combined with clear communication and collaboration while positioning patients in the operating room is the best and safest approach. The prevention of uncommon complications of spine surgery depends primarily on identifying high-risk patients, proper positioning and optimal intraoperative management of physiological parameters. Modification of risk factors extrinsic to the patient may help reduce the incidence of PPNI and POVL. PMID:25232519

Kamel, Ihab; Barnette, Rodger

2014-01-01

126

Ten uncommon and unusual variants of renal angiomyolipoma (AML): radiologic-pathologic correlation.  

PubMed

Classic (triphasic) renal angiomyolipoma (AML) is currently classified as a neoplasm of perivascular epithelioid cells. For diagnosis of AML, the use of thin-section non-contrast enhanced CT (NECT) improves diagnostic accuracy; however, identifying gross fat within a very small AML is challenging and often better performed with chemical-shift MRI. Although the presence of gross intra-tumoural fat is essentially diagnostic of AML; co-existing intra-tumoural fat and calcification may represent renal cell carcinoma (RCC). Differentiating AML from retroperitoneal sarcoma can be difficult when AML is large; the feeding vessel and claw signs are suggestive imaging findings. AML can haemorrhage, with intra-tumoural aneurysm size >5 mm a more specific predictor of future haemorrhage than tumor size >4 cm. Diagnosis of AML in the setting of acute haemorrhage is complex; comparison studies or follow-up imaging may be required. Not all AML contain gross fat and imaging features of AML without visible fat overlap with RCC; however, homogeneity, hyperdensity at NECT, low T2-weighted signal intensity and, microscopic fat are suggestive features. Patients with tuberous sclerosis often demonstrate a combination of classic and minimal fat AML, but are also at a slightly increased risk for RCC and should be imaged cautiously. Several rare pathological variants of AML exist including AML with epithelial cysts and epithelioid AML, which have distinct imaging characteristics. Classic AML, although benign, can be locally invasive and the rare epithelioid AML can be frankly malignant. The purpose of this review is to highlight the imaging manifestations of 10 uncommon and unusual variants of AML using pathological correlation. PMID:25468637

Schieda, N; Kielar, A Z; Al Dandan, O; McInnes, M D F; Flood, T A

2015-02-01

127

Diseases of the nasal cavity.  

PubMed

Conditions of this portion of the respiratory tract are relatively uncommon. This article reviews these conditions and discusses the different modes of therapy. The conditions covered are redundant alar folds, diseases of the nasal septum, fungal infections, neoplasms, nasal polyps, and ethmoid hematomas. The different surgical approaches to the nasal cavity are reviewed, and surgery of removal of the nasal septum and the alar fold are described. PMID:8472195

Nickels, F A

1993-04-01

128

Enteropathy Associated T Cell Lymphoma – A Case Report of An Uncommon Extranodal T Cell Lymphoma  

PubMed Central

Enteropathy associated T cell lymphoma is a rare primary intestinal lymphoma. It is often, but not always associated with celiac disease. Intraepithelial T cells are postulated as the cell of origin. It is a rare disease accounting for fewer than 5% of all gastrointestinal tract lymphomas. Recent studies indicate that EATL consists of two diseases that are morphologically and genetically distinct and differ with respect to their frequency of association with celiac disease. Current WHO classification recognises two subtypes of EATL – type 1 (classic) and type 2, based on morphology and immunophenotype. EATL type 1 is a large cell lymphoma which is more common and is more commonly associated with celiac disease compared to type 2. Most common site of involvement is the small intestine. We report a case of EATL type 1, in a 62-year-old female patient who presented with features of intestinal obstruction. However, she did not have spruce like featutes. PMID:25478355

V, Geetha; Kudva, Ranjini

2014-01-01

129

Orbital solitary fibrous tumor with multinucleate giant cells: case report of an unusual finding in an uncommon tumor.  

PubMed

Solitary fibrous tumor (SFT) is a rare soft-tissue neoplasm which may occur at any site although it is more frequent in the pleura, mediastinum and lung. Orbital involvement by SFT is uncommon. Giant cells are extremely rare to be seen in a SFT and have been described to be immunoreactive for CD34. We present a case of orbital SFT with multinucleate giant cells expressing CD68 and lacking immunoreactivity for CD34. The differential diagnosis is discussed. PMID:24152511

Mulay, Kaustubh; Honavar, Santosh G

2013-01-01

130

Uncommon primary tumors of the orbit diagnosed by computed tomography-guided core needle biopsy: report of two cases.  

PubMed

Computed tomography-guided percutaneous biopsy is a safe and effective alternative method for evaluating selected intra-orbital lesions where the preoperative diagnosis is important for the therapeutic planning. The authors describe two cases of patients with uncommon primary orbital tumors whose diagnosis was obtained by means of computed tomography-guided core needle biopsy, with emphasis on the technical aspects of the procedure. PMID:25741122

Jeng Tyng, Chiang; Matushita, Joăo Paulo Kawaoka; Bitencourt, Almir Galvăo Vieira; Neves, Flávia Branco Cerqueira Serra; Amoedo, Maurício Kauark; Barbosa, Paula Nicole Vieira; Chojniak, Rubens

2014-01-01

131

Uncommon primary tumors of the orbit diagnosed by computed tomography-guided core needle biopsy: report of two cases  

PubMed Central

Computed tomography-guided percutaneous biopsy is a safe and effective alternative method for evaluating selected intra-orbital lesions where the preoperative diagnosis is important for the therapeutic planning. The authors describe two cases of patients with uncommon primary orbital tumors whose diagnosis was obtained by means of computed tomography-guided core needle biopsy, with emphasis on the technical aspects of the procedure. PMID:25741122

Jeng Tyng, Chiang; Matushita Jr., Joăo Paulo Kawaoka; Bitencourt, Almir Galvăo Vieira; Neves, Flávia Branco Cerqueira Serra; Amoedo, Maurício Kauark; Barbosa, Paula Nicole Vieira; Chojniak, Rubens

2014-01-01

132

Bilateral retiform variant of sertoli leydig cell tumour of ovary: An uncommon tumor with review of literature  

PubMed Central

Sertoli-leydig cell tumors are the uncommon sex-cord stromal tumors of the ovary. We report a case of 42-year-old female with retiform variant of sertoli-leydig cell tumour. She presented with the complaint of mass in abdomen for 7 years. Ultrasound revealed bilateral ovarian mass suggestive of malignancy. Bilateral salpingo-oopherectomy with surgical staging was done. The tumor was diagnosed as stage I retiform variant of sertoli-leydig cell tumor on histopathology and immunohistochemistry.

Rathi, Monika; Budania, Satish Kumar; Khalid, Mohammad; Mittal, Ankur

2015-01-01

133

Fasciola hepatica (Trematoda: Digenea): its effect on the life history traits of Pseudosuccinea columella (Gasteropoda: Lymnaeidae), an uncommon interaction  

Microsoft Academic Search

An uncommon snail-trematode interaction pattern was found for the Pseudosuccinea columella - Fasciola hepatica model under laboratory conditions. Week-old juveniles from two isolates showed very high rates of infection (90.0% and 93.3%) after exposure to five miracidia. They also showed differences in their life history traits when compared to a control group. However, they did not exhibit the increase in

Alfredo Gutiérrez; Mary Yong; Gloria Perera; Jorge Sánchez; André Théron

2002-01-01

134

The spectrum of orofacial manifestations in renal osteodystrophy: diagnostic challenges of an uncommon presentation.  

PubMed

Renal osteodystrophy refers to a spectrum of bone diseases caused by pathologic alterations in the metabolism of calcium, phosphate, and bone in the context of end-stage renal disease and secondary hyperparathyroidism. Radiographic alterations affecting the jaw and facial skeleton are common and among the earliest signs of renal bone disease. Renal osteodystrophy also shares clinical, histologic, and radiologic similarities with several benign fibro-osseous conditions affecting the craniofacial region, and its recognition is critical to prevention, choice of therapy, and overall prognosis. The aim of this article is to review the craniofacial manifestations of renal osteodystrophy, describe the work-up of a patient with macrognathia and facial disfigurement caused by renal bone disease, discuss the challenges in arriving at a definitive diagnosis, and highlight an interdisciplinary approach to evaluation and timely diagnosis in overall management. PMID:21716979

Fatahzadeh, Mahnaz

2011-01-01

135

Green Herring Syndrome: Bacterial Infection in Patients With Mucormycosis Cavitary Lung Disease  

PubMed Central

Mucormycosis is a life-threatening fungal disease in patients with hematological malignancies. The diagnosis of pulmonary mucormycosis is particularly challenging. We describe 3 mucormycosis cases with an uncommon presentation in patients whose cavitary lung disease was attributed to well documented bacterial infection, although evolution and reassessment established mucormycosis as the underlying disease. PMID:25734087

Peixoto, Driele; Hammond, Sarah P.; Issa, Nicolas C.; Madan, Rachna; Gill, Ritu R.; Milner, Danny A.; Colson, Yolonda L.; Koo, Sophia; Baden, Lindsey R.; Marty, Francisco M.

2014-01-01

136

[Uncommon cutaneous ulcerative and systemic sarcoidosis. Successful treatment with hydroxychloroquine and compression therapy].  

PubMed

Sarcoidosis is a granulomatous multisystemic disease of unclear etiology, which can affect any organ. The cutaneous manifestations are variable, but ulcerative cutaneous sarcoidosis is very rare. One must rule out other granulomatous skin diseases, especially necrobiosis lipoidica. There is no standarized therapy; usually an interdisciplinary approach over years taking multiple side effects into consideration is needed. A 58-year-old woman with a long history of cutaneous, nodal and pulmonary sarcoidosis suddenly developed ulcerations within the disseminated skin lesions on her legs. The combination of systemic hydroxychloroquine and modern wound management lead to complete healing of the ulcers and a significant improvement in the remaining skin lesions. PMID:21656110

Meyersburg, D; Schön, M P; Bertsch, H P; Seitz, C S

2011-09-01

137

Castleman's Disease of the Porta Hepatis.  

PubMed

Castleman's disease is an uncommon cause of lymph node enlargement that mostly involves the mediastinum and lung hilum. It is divided into 2 types: localized, which is usually asymptomatic and presents with a mass lesion; and multicentric, which is characterized by chills, anemia, generalized lymphadenopathy and hepatosplenomegaly. An extrathoracic site of involvement is very uncommon, and may be located in the mesentery of the intestines, axilla, and pelvis. Hepatic localization of this disease is a rare event. Herein, we report our experience with a symptomatic case of Castleman's disease in the porta hepatis who has been treated successfully by excision of the hilar lymph nodes, but recurred after 2 years with the same clinical picture plus abnormal liver function tests. However, at the time of recurrence ,no lymphadenopathy was detected and liver biopsy showed giant lymphoid follicles with germinal centers. She was treated with steroids and showed a dramatic response. PMID:24829655

Geramizadeh, Bita; Fattahi, Mohammad Reza; Bagheri Lankarani, Kamran

2012-07-01

138

Pleomorphic Adenoma of Breast: A Radiological and Pathological Study of a Common Tumor in an Uncommon Location  

PubMed Central

Pleomorphic adenoma occurs commonly in the major salivary glands but is uncommonly encountered in the breast. In both of these locations, the tumor is typically grossly circumscribed and has a “mixed” histological appearance, being composed of myoepithelial and epithelial components amid a myxochondroid matrix. Herein, we report a case of pleomorphic adenoma of the breast which was preoperatively thought to represent a fibroadenoma on clinical and radiological grounds. It is the rarity of the tumor in the breast, rather than its histological appearance, that causes diagnostic difficulty.

Ginter, Paula S.; Scognamiglio, Theresa; Tauchi-Nishi, Pamela; Antonio, Lilian B.; Hoda, Syed A.

2015-01-01

139

Bilateral retiform variant of sertoli leydig cell tumour of ovary: An uncommon tumor with review of literature.  

PubMed

Sertoli-leydig cell tumors are the uncommon sex-cord stromal tumors of the ovary. We report a case of 42-year-old female with retiform variant of sertoli-leydig cell tumour. She presented with the complaint of mass in abdomen for 7 years. Ultrasound revealed bilateral ovarian mass suggestive of malignancy. Bilateral salpingo-oopherectomy with surgical staging was done. The tumor was diagnosed as stage I retiform variant of sertoli-leydig cell tumor on histopathology and immunohistochemistry. PMID:25861207

Rathi, Monika; Budania, Satish Kumar; Khalid, Mohammad; Mittal, Ankur

2015-01-01

140

Grave's Disease and Primary Biliary Cirrhosis—An Unusual and Challenging Association  

PubMed Central

Jaundice in Grave's diseases is uncommon, but when it does occur, complication of thyrotoxicosis (heart failure/infection) or intrinsic liver disease should be considered. Grave's disease can cause asymptomatic elevation of liver enzymes, jaundice and rarely acute liver failure. It is associated with other autoimmune diseases like autoimmune hepatitis, or primary biliary cirrhosis. The cause of jaundice in Grave's disease is multifactorial. PMID:25755537

Shetty, Shiran; Rajasekaran, Senthilkumar; Venkatakrishnan, Leela

2013-01-01

141

An experimental and theoretical study to relate uncommon rock/fluid properties to oil recovery. Final report  

SciTech Connect

Waterflooding is the most commonly used secondary oil recovery technique. One of the requirements for understanding waterflood performance is a good knowledge of the basic properties of the reservoir rocks. This study is aimed at correlating rock-pore characteristics to oil recovery from various reservoir rock types and incorporating these properties into empirical models for Predicting oil recovery. For that reason, this report deals with the analyses and interpretation of experimental data collected from core floods and correlated against measurements of absolute permeability, porosity. wettability index, mercury porosimetry properties and irreducible water saturation. The results of the radial-core the radial-core and linear-core flow investigations and the other associated experimental analyses are presented and incorporated into empirical models to improve the predictions of oil recovery resulting from waterflooding, for sandstone and limestone reservoirs. For the radial-core case, the standardized regression model selected, based on a subset of the variables, predicted oil recovery by waterflooding with a standard deviation of 7%. For the linear-core case, separate models are developed using common, uncommon and combination of both types of rock properties. It was observed that residual oil saturation and oil recovery are better predicted with the inclusion of both common and uncommon rock/fluid properties into the predictive models.

Watson, R.

1995-07-01

142

Xanthogranulomatous orchiepididymitis: a very uncommon cause of scrotal mass in childhood.  

PubMed

Xanthogranulomatous orchitis (XGO) is an extremely rare inflammatory destructive lesion of testis. We report a case of a 14-year-old boy who presented with painful right testicular mass and discharging scrotal fistulas. Serologic tumor markers were normal. Tissue destruction was profound in the initial biopsy. The antibiotic treatment did not change the course of the disease. The definitive cure was achieved by orchiectomy and excision of the fistulous extension to the scrotum. Histopathological investigation was consistent with xanthogranulomatous process. Although XGO is very rare, the basic interest in the present report is considering this entity in the broad differential diagnosis spectrum of scrotal masses in childhood. PMID:23523294

Ezer, Semire Serin; Oguzkurt, Pelin; Temiz, Abdulkerim; Bal, Nebil; Hicsonmez, Akgun

2013-07-01

143

[An uncommon cause of dyspnea in children. Plexiform neurofibroma of the larynx].  

PubMed

Plexiform neurofibroma is a rare benign tumor developed from cells of the sheath of Schwann peripheral nervous system often associated with neurofibromatosis type 1 or Von Recklinghausen disease. Laryngeal involvement in neurofibromatosis is rare and usually presents with obstructive respiratory symptoms. Imaging including CT and MRI provides the diagnosis as well as the staging of this lesion, whose treatment is primarily surgical. We report the case of a plexiform neurofibroma of the larynx found in a 4-year-old child revealing a mass completely obstructing the laryngeal vestibule. PMID:23628124

Moussali, N; Belmoukari, S; Elmahfoudi, H; Elbenna, N; Abdelouafi, A; Gharbi, A

2013-06-01

144

Molecular and clinical analysis in a series of patients with Pyknodysostosis reveals some uncommon phenotypic findings  

PubMed Central

Pyknodysostosis is a rare autosomal recessive skeletal dysplasia characterized by short stature, deformity of the skull, osteosclerosis, hypoplasia of the clavicle, and bone fragility. Radiographs show increased bone density, osteosclerosis, and acroosteolysis of the terminal phalanges. The pycnodysostosis gene is located on chromosome 1q21 and encodes an enzyme called Cathepsin K. Cathepsin K is a cysteine protease lysosomal protein associated with the degradation of bone and cartilage. In the current study, the authors described the clinical, radiological and molecular features of a group of six Mexican patients, including two familial and two sporadic cases, with Pyknodysostosis. One of the patients presented hypoacusia, an unusual finding in this disease. PMID:25550899

Valdes-Flores, Margarita; Hidalgo-Bravo, Alberto; Casas-Avila, L; Chima-Galan, Carmen; Hazan-Lasri, Eric J; Pineda-Gomez, Ernesto; Lopez-Estrada, Druso; Zenteno, Juan C

2014-01-01

145

Inherited renal diseases.  

PubMed

Genetic disorders of the kidney include cystic diseases, metabolic diseases and immune glomerulonephritis. Cystic diseases include autosomal dominant and recessive polycystic kidney disease (ADPKD, ARPKD, respectively). Neonates with enlarged, cystic kidneys should be evaluated for PKD. Patients with ADPKD have cysts and renal enlargement. Most patients present with hypertension, hematuria or flank pain; the most common extrarenal manifestation is polycystic liver disease. Oligohydramnios, bilaterally enlarged kidneys and decreased urine are featured in utero in ARPKD. Medullary sponge kidney is uncommon and features nephrocalcinosis, recurrent calcium stones and a history of polyuria/nocturia and/or urinary tract infections. Alport syndrome (AS) is an inherited disease of the glomerular basement membrane that is usually inherited as an X-linked dominant trait. Most patients with AS present in the first two decades of life with persistent microscopic or gross hematuria. Later, proteinuria is seen and its presence portends disease progression. Other findings may include sensorineural hearing loss and ocular abnormalities. There are various inherited tubulopathies, including Bartter syndrome, a group of renal tubular disorders that consist of two phenotypes with four genotypes. Patients usually present early in life with salt wasting, hypokalemia and metabolic alkalosis. Other features, depending on genotype, may include polyhydramnios and premature birth. Gitelman syndrome is also a salt-losing tubulopathy characterized by hypokalemic alkalosis. The majority of patients with Gitelman syndrome present during adolescence or early adulthood. PMID:25088262

Leung, Jocelyn C

2014-01-01

146

The inguinal canal: anatomy and imaging features of common and uncommon masses.  

PubMed

A variety of benign and malignant masses can be found in the inguinal canal (IC). Benign causes of masses in the IC include spermatic cord lipoma, hematoma, abscess, neurofibroma, varicocele, desmoid tumor, air, bowel contrast material, hydrocele, and prostheses. Primary neoplasms of the IC include liposarcoma, Burkitt lymphoma, testicular carcinoma, and sarcoma. Metastases to the IC can occur from alveolar rhabdomyosarcoma, monophasic sarcoma, prostate cancer, Wilms tumor, carcinoid tumor, melanoma, or pancreatic cancer. In patients with a known malignancy and peritoneal carcinomatosis, the diagnosis of metastases can be suggested when a mass is detected in the IC. When peritoneal disease is not evident, a mass in the IC is indicative of stage IV disease and may significantly alter clinical and surgical treatment of the patient. A combination of the clinical history, symptoms, laboratory values, and radiologic features aids the radiologist in accurately diagnosing mass lesions of the IC. Supplemental material available at radiographics.rsnajnls.org/cgi/content/full/28/3/819/DC1. PMID:18480486

Bhosale, Priya R; Patnana, Madhavi; Viswanathan, Chitra; Szklaruk, Janio

2008-01-01

147

Intrahepatic Cholestasis in Sickle Cell Disease: A Case Report  

E-print Network

an uncommon but potentially fatal complication of sickle cell disease (SCD), with a high death rate, observed mainly in patients with homozygous sickle cell anemia. Herein, we describe a case of severe SCIC treated successfully with aggressive manual exchange transfusion (ET). The patient was

Article Id; Denise Menezes Brunetta; Ana Cristina Silva-pinto; Maria Carmo; Favarin Macedo; Sarah Cristina Bassi; Joao Victor; Piccolo Feliciano; A Borges Ribeiro; Benedito Pina; Almeida Prado; Gil Cunha; De Santis; Ivan De Lucena Angulo; Dimas Tadeu Covas

148

Liddle's syndrome, an uncommon form of hyporeninemic hypoaldosteronism: functional and histopathological studies.  

PubMed

Liddle's syndrome was diagnosed in a 72-year-old man who presented clinically with hypertension and muscle weakness. This disorder has been characterized by hyporeninemic hypoaldosteronism, hypertension, hypokalemia and enhanced erythrocyte sodium influx. Administration of spironolactone failed to correct the hypertension and electrolyte abnormality, which subsequently improved with triamterene therapy and a low salt diet. However, suppression of the renin-angiotensin-aldosterone system remained unchanged after this treatment. In addition, an atrophic juxtaglomerular apparatus and hypertensive lesions in the arterioles were confirmed by kidney biopsy after triamterene therapy. Therefore, a process of intrinsic hyperactive distal sodium reabsorption, probably affected by aldosterone-independent sodium transport into erythrocytes, appears to be important in the pathogenesis of this syndrome. Triamterene therapy, which usually is performed in patients with this disease, might not be the ultimate therapy in the future even if electrolyte abnormalities were to be improved temporarily. PMID:3550146

Nakada, T; Koike, H; Akiya, T; Katayama, T; Kawamata, S; Takaya, K; Shigematsu, H

1987-04-01

149

Phaeochromocytoma presenting with polyuria: an uncommon presentation of a rare tumour  

PubMed Central

Summary Children rarely present with phaeochromocytoma. Their presentation differs from that of adults. The classic triad of sweating, headache and palpitation may not always present in children with phaeochromocytoma. In this study, we present a 6-year-old girl who came to us with polyuria and polydipsia for evaluation of suspected diabetes insipidus. She gave a clear history of increased sweating in the recent past. On clinical examination, she was noted to have high blood pressure. Subsequent investigations revealed a phaeochromocytoma. Her polyuria and hypertension resolved immediately after the surgery. We did not have the facilities to arrange for genetic tests; however, the patient and the family members are under follow-up for other associated conditions. Learning points Polyuria and polydipsia are rare symptoms of phaeochromocytoma.Complete physical examination prevented unnecessary investigations for polyuria and led to a correct diagnosis.Classic features are not always necessary for diagnostic evaluation of rare diseases. PMID:25332770

Imalke, K A C P; Madarasinghe, M; Lamahewage, A; de Silva, K S H

2014-01-01

150

Rate of port-site metastasis is uncommon in patients undergoing robotic surgery for gynecologic malignancies  

PubMed Central

Objective To describe the rate of port-site metastasis in patients who underwent robotic surgery for suspected gynecologic malignancy. Methods Using a prospective database, we identified all patients who underwent robotic surgery performed by the Gynecologic Oncology service at 1 institution between December 2006 and March 2010. Records of patients with confirmed malignancy were reviewed for clinicopathologic data and information about port-site metastasis. Results One hundred eighty-one patients met the inclusion criteria. The median age was 55.4 years (range, 19–82), and the median body mass index was 29.6 kg/m2 (range, 17.9–70.7). Port-site metastases were detected in two patients (1.1%) at 3 weeks (patient 1) and 11 months (patient 2) after surgery. Patient 1 underwent surgery for an adnexal mass and pathologic examination revealed gallbladder adenocarcinoma metastatic to the ovary. She had a recurrence in the right lateral-abdominal-wall robotic-trocar site with concurrent metastases in the gallbladder fossa and liver. Patient 2 was diagnosed with adenocarcinoma of unclear (cervical vs. endometrial) origin. Imaging showed metastases in pelvic and paraaortic lymph nodes. She underwent laparoscopy and was found intraoperatively to have gross disease on the right ovary. The patient underwent right salpingo-oophorectomy and chemoradiation. She had residual disease in the cervix and subsequently underwent robotic hysterectomy and left salpingo-oophorectomy. Pathologic examination revealed endometrial cancer. She had a recurrence at the transumbilical trocar site concurrent with retroperitoneal lymphadenopathy and carcinomatosis. There were no cases of isolated port-site metastasis. Conclusions The rate of port-site metastasis after robotic surgery in women with gynecologic cancer is low and similar to the rate for laparoscopic procedures. PMID:21633306

Ndofor, Bih T.; Soliman, Pamela T.; Schmeler, Kathleen M.; Nick, Alpa M.; Frumovitz, Michael; Ramirez, Pedro T.

2015-01-01

151

Rectal ulcer with an elusive diagnosis: all that ulcers is not Crohn disease  

Technology Transfer Automated Retrieval System (TEKTRAN)

A single rectal ulcer is an uncommon finding in children with gastrointestinal disease. Although inflammatory bowel disease (IBD) is foremost among the differential diagnoses, a primary immunological defect should not be forgotten. Because of the paucity of literature on the association of rectal ul...

152

Melanized Fungi in Human Disease  

PubMed Central

Summary: Melanized or dematiaceous fungi are associated with a wide variety of infectious syndromes. Many are soil organisms and are generally distributed worldwide, though certain species appear to have restricted geographic ranges. Though they are uncommon causes of disease, melanized fungi have been increasingly recognized as important pathogens, with most reports occurring in the past 20 years. The spectrum of diseases with which they are associated has also broadened and includes allergic disease, superficial and deep local infections, pneumonia, brain abscess, and disseminated infection. For some infections in immunocompetent individuals, such as allergic fungal sinusitis and brain abscess, they are among the most common etiologic fungi. Melanin is a likely virulence factor for these fungi. Diagnosis relies on careful microscopic and pathological examination, as well as clinical assessment of the patient, as these fungi are often considered contaminants. Therapy varies depending upon the clinical syndrome. Local infection may be cured with excision alone, while systemic disease is often refractory to therapy. Triazoles such as voriconazole, posaconazole, and itraconazole have the most consistent in vitro activity. Further studies are needed to better understand the pathogenesis and optimal treatment of these uncommon infections. PMID:20930077

Revankar, Sanjay G.; Sutton, Deanna A.

2010-01-01

153

An uncommon presentation of eosinophilic granulomatosis with polyangiitis: a case report  

PubMed Central

Introduction Eosinophilic granulomatosis with polyangiitis is a rare and potentially fatal disease if not readily diagnosed. Cerebral involvement is extremely rare and clinical presentation as hemorrhagic stroke is even rarer. Case presentation A 58-year-old Caucasian man was admitted to our medical unit because of a computed tomography-diagnosed hemorrhagic stroke with right-sided hemiparesis and fever. A chest computed tomography scan also revealed multiple bilateral pulmonary infiltrates; coronary artery, and carotid and left vertebral artery calcifications were also observed. Empiric antimicrobial therapy with cephalosporins was promptly undertaken; low-molecular-weight heparin was introduced as prophylaxis for venous thromboembolism. Over the following days, magnetic resonance imaging scans showed a regression of the hemorrhagic framework, also revealing hypoxic areas consistent with acute ischemic lesions. With a computed tomography scan showing a worsening of his pulmonary framework, antimicrobial therapy was modified and corticosteroids were introduced. A new blood cell count revealed further increased leukocytosis (17.49×103?L), characterized by a surprising rise of eosinophilic cells (32.8%). Angiography of the coronary arteries found diffuse dilatations with severe signs of endothelial damage. Such an unexpected framework induced a strong suspicion that the stroke was the expression of a systemic vasculitis, which had triggered his cerebral, coronary, and pulmonary frameworks. The search for antineutrophil cytoplasmic antibody was positive for perinuclear antineutrophil cytoplasmic antibody, and eosinophilic granulomatosis with polyangiitis was diagnosed. Explaining to the patient the rarity of his disease, and what the most typical presentations of eosinophilic granulomatosis with polyangiitis were, he revealed that before admission he had had scalp injuries, in the nuchal region, and had taken corticosteroids as self-medication, with subsequent disappearance of the lesions. Therefore, high-dose corticosteroid treatment was started, and at discharge he was in good clinical condition with a slight right-sided hyposthenia. Conclusions A diagnosis of eosinophilic granulomatosis with polyangiitis is often difficult, but we are convinced that intake of corticosteroids on a self-prescribed basis may have obscured the clinical presentation. Therefore, this case also suggests how the growing phenomenon of self-medication can be harmful, and that a careful investigation of clinical history is still an act of paramount importance. PMID:24928069

2014-01-01

154

Methylphenidate intoxication: somnolence as an uncommon clinical symptom and proof of overdosing by increased serum levels of ritalinic acid.  

PubMed

There is considerable evidence for an increase of methylphenidate (MPH) abuse; thus, physicians might be confronted more frequently with MPH intoxications. Possible symptoms of intoxications with MPH are orofacial, stereotypic movements and tics as well as tachycardia, cardiac arrhythmias, arterial hypertension, hyperthermia, hallucinations and epileptic seizures. Here we report a patient who demonstrated somnolence as an uncommon clinical feature of MPH intoxication. The patient exhibited subnormal MPH serum levels (3 ?g/l), however markedly increased serum levels of ritalinic acid (821 ?g/l; inactive metabolite of MPH), that finally confirmed the initially suspected MPH intoxication. Due to the short half-life of orally administered MPH (t1/2~3 h) the sole measurement of MPH serum levels might be misleading concerning the proof of MPH overdosing in some cases. Parallel measurement of MPH and ritalinic acid is recommended in cases with suspected MPH intoxication and insufficient anamnestic data. PMID:25121992

Gahr, M; Kölle, M A

2014-09-01

155

3-Methyl-2-butene-1-thiol: identification, analysis, occurrence and sensory role of an uncommon thiol in wine.  

PubMed

A highly uncommon odorant, 3-methyl-2-butene-1-thiol was detected by using Gas Chromatography-Olfactometry (GC-O) and unequivocally identified for the first time in wine. A purge and trap sampling technique which provides highly representative extracts for olfactometric analysis was used for the extraction of the volatile fraction of a Spanish red wine made from Prieto Picudo grapes. The identification of the odorant was achieved by multidimensional gas chromatography analysis of the same purge and trap extract. Mass spectrum and retention indices in both polar and non-polar columns allowed knowing unequivocally the identity. To obtain quantitative data a method was validated for the analysis of the compound at ng L(-1) level with acceptable precision. This powerful odorant presented an odor threshold in wine of 0.5-1 ng L(-1) and it has been detected in several Prieto Picudo wines at concentrations slightly above the odor threshold. PMID:22967545

San-Juan, Felipe; Cacho, Juan; Ferreira, Vicente; Escudero, Ana

2012-09-15

156

New and uncommon indole- and imidazole-alkylamines in skins of amphibians from Australia and Papua New Guinea.  

PubMed

Extracts of the skin of some amphibians from Australia and Papua New Guinea contained, in addition to the usual 5-hydroxyindolealkylamines and histamine: a. two new, hitherto unknown indolealkylamines, i.e. O-sulphate of bufotenidine and 2-(3-indolyl) ethyltrimethylammonium, a quaternary ammonium base of tryptamine. The rare O-sulphate of bufotenine was also present, the occurrence of which had previously been demonstrated only in the skin of some South American toads; b. a series of uncommon imidazolealkylamines, such as N'-acetylhistamine, N'-methylhistamine, N',N'-dimethylhistamine, spinaceamine and 6-methylspinaceamine. It appears evident that amphibian skin continues to be an exceptionally rich source of aromatic amines. PMID:134551

Roseghini, M; Endean, R; Temperilli, A

1976-01-01

157

Myocardial infarction as an uncommon clinical manifestation of intravascular large cell lymphoma.  

PubMed

Intravascular large cell lymphoma (IVL) is a very rare variant of non-Hodgkin's lymphoma presenting with puzzling clinical manifestations. There is a predilection for the central nervous system, but the tumour often affects also skin, lung, and kidneys while lymphadenopathy and hepatosplenomegaly are usually absent. Myocardial infarction due to IVL has not been reported so far. We here report on a 56-year-old patient who was admitted to our hospital with fever and clinical signs of erysipelas. He had a 6-month history of "collagen vasculitic disease" treated with prednisolone and azathioprine. He received antibiotic treatment, but after transient improvement fever recurred with generalized seizures and myocardial infarction, which required transfer to the intensive care unit where the patient died with signs of an acute cardiogenic shock. Autopsy revealed a generalized high-grade B cell lymphoma of IVL type affecting and obstructing small vessels of a variety of tissues including heart, brain and lungs. The tumorous obliteration of small intramyocardial vessels had led to an acute ischaemia with infarction and subsequent signs of myocardial insufficiency. To the best of the authors' knowledge myocardial infarction as a leading symptom of IVL has not been described. PMID:16261789

Bauer, Alexander; Perras, Boris; Sufke, Sven; Horny, Hans-Peter; Kreft, Burkhard

2005-10-01

158

A Pseudo MS3 Approach for Identification of Disulfide-Bonded Proteins: Uncommon Product Ions and Database Search  

NASA Astrophysics Data System (ADS)

It has previously been reported that disulfide and backbone bonds of native intact proteins can be concurrently cleaved using electrospray ionization (ESI) and collision-induced dissociation (CID) tandem mass spectrometry (MS/MS). However, the cleavages of disulfide bonds result in different cysteine modifications in product ions, making it difficult to identify the disulfide-bonded proteins via database search. To solve this identification problem, we have developed a pseudo MS3 approach by combining nozzle-skimmer dissociation (NSD) and CID on a quadrupole time-of-flight (Q-TOF) mass spectrometer using chicken lysozyme as a model. Although many of the product ions were similar to those typically seen in MS/MS spectra of enzymatically derived peptides, additional uncommon product ions were detected including ci-1 ions (the ith residue being aspartic acid, arginine, lysine and dehydroalanine) as well as those from a scrambled sequence. The formation of these uncommon types of product ions, likely caused by the lack of mobile protons, were proposed to involve bond rearrangements via a six-membered ring transition state and/or salt bridge(s). A search of 20 pseudo MS3 spectra against the Gallus gallus (chicken) database using Batch-Tag, a program originally designed for bottom up MS/MS analysis, identified chicken lysozyme as the only hit with the expectation values less than 0.02 for 12 of the spectra. The pseudo MS3 approach may help to identify disulfide-bonded proteins and determine the associated post-translational modifications (PTMs); the confidence in the identification may be improved by incorporating the fragmentation characteristics into currently available search programs.

Chen, Jianzhong; Shiyanov, Pavel; Schlager, John J.; Green, Kari B.

2012-02-01

159

Rapidly progressive congenital rhabdomyosarcoma presenting with multiple cutaneous lesions: an uncommon diagnosis and a therapeutic challenge.  

PubMed

Congenital rhabdomyosarcomas (RMSs) are rare tumors with variable clinical presentations. A 2 month-old, term male neonate (37 weeks, 4 days), weighing 3.2kg, born to a 24 year-old primigravida, by simple vaginal delivery presented with multiple erythematous papulonodular lesions over his trunk that progressed to his whole body, on the first day of delivery. Prior to conception, his mother was treated for polycystic ovarian disease. On the tenth day, his chest computed tomogram scans revealed multiple, heterogeneously enhancing, bilateral pleural-based soft tissue density nodular lesions, along with multiple soft tissue density lesions, involving skeletal muscles of all his body parts. Microsections from two biopsies (on 10th day and after 2 months) revealed a malignant round cell tumor with cells arranged in a diffuse, solid pattern, comprising embryonal and solid alveolar components. Immunohistochemically, the tumor cells were diffusely positive for desmin, myoD1 and myogenin. Diagnosis of embryonal and alveolar (mixed type) RMS was offered. Further molecular cytogenetic analysis was negative for PAX3-FKHR and PAX7-FKHR. The patient was induced on chemotherapy as per intergroup rhabdomyosarcoma study IV protocol. There was treatment response with near total remission after 8 weeks of treatment. Thereafter, new lesions started appearing that also disappeared after modification of the chemotherapy drugs. However, after 16 months, the baby died of brain metastasis. The present case forms the fourth case report of an aggressive form of a congenital RMS with extensive cutaneous involvement and brain metastasis. A review of previously diagnosed cases of congenital RMSs is discussed herewith. PMID:24635971

Rekhi, Bharat; Qureshi, Sajid Shafique; Narula, Gaurav; Gujral, Sumeet; Kurkure, Purna

2014-05-01

160

Combined hepatocellular-cholangiocarcinoma: a population-level analysis of an uncommon primary liver tumor.  

PubMed

Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare primary liver cancer. Our aims were to analyze the demographic, clinical, and pathological characteristics of cHCC-CC at a population level and to investigate the effects of these features as well as different management strategies on the prognosis. The Surveillance, Epidemiology, and End Results (SEER) database was analyzed for 1988-2009. Data analyses were performed with chi-square tests, analyses of variance, Kaplan-Meier curves, and Cox proportional hazards regression. Four hundred sixty-five patients with cHCC-CC, 52,825 patients with hepatocellular carcinoma (HCC), and 7181 patients with cholangiocarcinoma (CC) were identified. cHCC-CC was more common in patients who were white, male, and older than 65 years. Treatment was more frequently nonsurgical/interventional. Patients with cHCC-CC, HCC, and CC had 5-year overall survival (OS) and disease-specific survival rates of 10.5%, 11.7%, and 5.7% (P < 0.001) and 17.8%, 21.0%, and 11.9% (P < 0.001), respectively. For cHCC-CC patients, an increasing invasiveness of the therapeutic approach was significantly associated with prolonged survival (P < 0.001). In a multivariate model, black race, a distant SEER stage, and a tumor size of 5.0 to 10.0 cm were independently associated with lower survival for cHCC-CC patients; a year of diagnosis after 1995 and surgical treatment with minor hepatectomy, major hepatectomy (MJH), or liver transplantation (LT) were independently associated with better survival for cHCC-CC patients. Patients diagnosed with cHCC-CC, HCC, and CC and treated with LT had 5-year OS rates of 41.1%, 67.0%, and 29.0%, respectively (P < 0.001). In conclusion, cHCC-CC patients appear to have intermediate demographic, clinical, and survival characteristics in comparison with HCC and CC patients. cHCC-CC patients undergoing LT showed inferior survival in comparison with HCC patients, and the role and indications for LT in cHCC-CC have yet to be defined. At this time, MJH may be considered the best therapeutic approach for such patients. PMID:24777610

Garancini, Mattia; Goffredo, Paolo; Pagni, Fabio; Romano, Fabrizio; Roman, Sanziana; Sosa, Julie Ann; Giardini, Vittorio

2014-08-01

161

Three Cases of Lichen Nitidus Associated with Various Cutaneous Diseases  

PubMed Central

Lichen nitidus (LN) is an uncommon, usually asymptomatic cutaneous eruption characterized by the presence of multiple, small, flesh-colored papules. The epidemiologic and pathophysiologic characteristics of LN have not yet been defined. Furthermore, LN has rarely been described in association with other cutaneous diseases. We herein report 3 cases of LN associated with various cutaneous diseases, including lichen striatus, oral lichen planus, and psoriasis vulgaris. PMID:25143682

Cho, Eun Byul; Kim, Heung Yeol; Park, Eun Joo; Kwon, In Ho; Kim, Kwang Joong

2014-01-01

162

Scheuermann's disease: an update.  

PubMed

Scheuermann's disease is a juvenile osteochondrosis of the spine. It is a disease of the growth cartilage endplate, probably due to repetitive strain on the growth cartilage weakened by a genetic background. The radiographic aspects are related to the vertebral endplate lesions and include vertebral wedging, irregularity of the vertebral endplate, and Schmorl's node (intraossous disk herniation). Disc alterations are frequent and may be secondary to dysfunction of the disc-vertebra complex. The definitions of Scheuermann's disease are varied; it can refer to the classical form of juvenile kyphosis, described by Scheuermann as well as asymptomatic radiographic abnormalities. Lumbar involvement is probably as frequent as the thoracic form and might be more painful. The first-line treatment is medical and includes rehabilitation and bracing. The earlier the start of treatment, the better the outcome, which highlights the importance of early diagnosis. Surgery is uncommon and must be limited to severe involvement after failure of conservative treatment. The natural history of Scheuermann's disease is unknown, but it might be associated with increased risk of back pain. The evolution of thoracolumbar and lumbar disease is unknown. PMID:24468666

Palazzo, Clémence; Sailhan, Frédéric; Revel, Michel

2014-05-01

163

Cultural-Linguistic Aspects in Asian Language Teaching. Proceedings of the First Annual Meeting of the American Council of Teachers of Uncommonly-Taught Asian Languages.  

ERIC Educational Resources Information Center

Problems encountered by teachers of uncommonly-taught Asian languages attempting to teach the culture of the native speakers of the target language are discussed in these articles: (1) "Cultural Context, Linguistic Categories, and Foreign Language Teaching: A Case from Marathi" by Vasant S. Khokle, (2) "The Ethnology of Communication and the…

Dardjowidjojo, Soenjono, Comp.

164

Reconstructors: Uncommon Scents - Episode 2. Students learn the health effects of solvent exposure on memory and selective attention, inhalant abuse, and refusal strategies  

NSDL National Science Digital Library

In Uncommon Scents Episode 2, students investigate exposure to toxic chemicals. They will discover that many common household products contain toxic chemicals, and also learn how inhaling these product can severely damage the nervous system and other parts of the body.

Center for Technology in Teaching and Learning

2011-09-28

165

Reconstructors: Uncommon Scents - Episode 3. Students learn the health effects of solvent exposure on memory and selective attention, inhalant abuse, and refusal strategies  

NSDL National Science Digital Library

In Uncommon Scents Episode 3, students investigate exposure to toxic chemicals. They will discover that many common household products contain toxic chemicals, and also learn how inhaling these product can severely damage the nervous system and other parts of the body.

Center for Technology in Teaching and Learning

2011-09-28

166

Reconstructors: Uncommon Scents - Episode 1. Students learn the health effects of inhalant abuse and exposure to hazardous household products such as organic solvents  

NSDL National Science Digital Library

In Uncommon Scents Episode 1, students investigate a chemical accident and learn about the health effects of exposure to toxic chemicals. They will discover that many common household products contain toxic chemicals, learn how inhaling these product can severely damage the nervous system and other parts of the body, and find ways to protect themselves from exposure to toxic vapors.

Center for Technology in Teaching and Learning

2011-09-28

167

Cutaneous Manifestations in Inflammatory Bowel Diseases: Eight Cases of Psoriasis Induced by Anti-Tumor-Necrosis-Factor Antibody Therapy  

Microsoft Academic Search

Background: Ulcerous rectocolitis and Crohn’s disease are the best known forms of inflammatory bowel disease (IBD). Skin manifestations are not uncommon in IBD and may be divided into specific cutaneous signs, aspecific cutaneous signs, and cutaneous signs caused by drugs used for IBD therapy. The specific signs (fistulas, rhagades and ulcers) are the result of the diffusion of the intestinal

Beatrice Passarini; Salvatore Domenico Infusino; Elena Barbieri; Elisa Varotti; Paolo Gionchetti; Fernando Rizzello; Claudia Morselli; Rosy Tambasco; Massimo Campieri

2007-01-01

168

Mutation update and uncommon phenotypes in a French cohort of 96 patients with WFS1-related disorders.  

PubMed

WFS1 mutations are responsible for Wolfram syndrome (WS) characterized by juvenile-onset diabetes mellitus and optic atrophy, and for low-frequency sensorineural hearing loss (LFSNHL). Our aim was to analyze the French cohort of 96 patients with WFS1-related disorders in order (i) to update clinical and molecular data with 37 novel affected individuals, (ii) to describe uncommon phenotypes and, (iii) to precise the frequency of large-scale rearrangements in WFS1. We performed quantitative polymerase chain reaction (PCR) in 13 patients, carrying only one heterozygous variant, to identify large-scale rearrangements in WFS1. Among the 37 novel patients, 15 carried 15 novel deleterious putative mutations, including one large deletion of 17,444 base pairs. The analysis of the cohort revealed unexpected phenotypes including (i) late-onset symptoms in 13.8% of patients with a probable autosomal recessive transmission; (ii) two siblings with recessive optic atrophy without diabetes mellitus and, (iii) six patients from four families with dominantly-inherited deafness and optic atrophy. We highlight the expanding spectrum of WFS1-related disorders and we show that, even if large deletions are rare events, they have to be searched in patients with classical WS carrying only one WFS1 mutation after sequencing. PMID:24890733

Chaussenot, A; Rouzier, C; Quere, M; Plutino, M; Ait-El-Mkadem, S; Bannwarth, S; Barth, M; Dollfus, H; Charles, P; Nicolino, M; Chabrol, B; Vialettes, B; Paquis-Flucklinger, V

2015-05-01

169

Uncommon or cryptic? Challenges in estimating leopard seal abundance by conventional but state-of-the-art methods  

NASA Astrophysics Data System (ADS)

The method traditionally used to estimate pack-ice seal abundance employs sighting surveys from ships or aircraft to estimate the number of seals hauled out on the ice, combined with studies of haul-out behaviour to estimate the proportion of time spent on the ice. Application of this approach has been improved in recent times by developments in survey methodology and satellite technology that theoretically allow biases in the estimation of hauled-out abundance and haul-out behaviour to be accounted for that previously could not be addressed. A survey using these conventional but state-of-the-art methods was undertaken in the summer of 1999/2000 off east Antarctica between longitudes 64°E and 150°E to estimate the abundance of leopard ( Hydrurga leptonyx) and other pack-ice seal species. Because they are either uncommon or very cryptic, very few leopard seals were encountered despite a large survey effort. This presented challenges in both application of the methods and analysis of the data. Abundance estimates were derived using a number of plausible predictive models. The model considered as the most reliable returned best estimates of 7300 and 12,100 for definite and definite plus probable leopard seal sightings, respectively, with 95% confidence intervals of 3700-14,500 and 7100-23,400. These estimates are likely to be negatively biased and should be treated as minimum estimates only.

Southwell, Colin; Paxton, Charles G. M.; Borchers, David; Boveng, Peter; Rogers, Tracey; de la Mare, William K.

2008-04-01

170

Fasciola hepatica (Trematoda: Digenea): its effect on the life history traits of Pseudosuccinea columella (Gasteropoda: Lymnaeidae), an uncommon interaction.  

PubMed

An uncommon snail-trematode interaction pattern was found for the Pseudosuccinea columella - Fasciola hepaticamodel under laboratory conditions. Week-old juveniles from two isolates showed very high rates of infection (90.0% and 93.3%) after exposure to five miracidia. They also showed differences in their life history traits when compared to a control group. However, they did not exhibit the increase in size and reduction/cessation in host fecundity which is usually observed in most of the other related snail-trematode systems. In contrast, infected juvenile P. columella showed increased egg laying after the onset of cercarial emission and there was no effect on growth. A third isolate of P. columella was found to be refractory to miracidial infection. This constitutes the first report of non-susceptibility to F. hepatica in a lymnaeid species which is usually susceptible. These non-susceptible snails exhibited lower fecundity and survival compared to non-exposed susceptible snails under identical laboratory conditions. The differences observed in terms of life span and reproduction could result from the cost of resistance for individuals from this isolate. PMID:12107475

Gutiérrez, Alfredo; Yong, Mary; Perera, Gloria; Sánchez, Jorge; Théron, André

2002-06-01

171

The University's Uncommon Community  

ERIC Educational Resources Information Center

In the UK, as elsewhere in the world, the global financial crisis has focused attention on the cost of public services and the need to reduce expenditure, not least in respect of higher education. This, however, raises a set of prior questions: What kind of society do we want? What is important to democratic society? What kind of higher education…

Harris, Suzy

2012-01-01

172

An uncommon pulmonary embolism  

PubMed Central

Objectives: To report an unusual case of suicide attempt secondary complicated of pulmonary and systemic embolisms. Data Source: A 49-year-old-woman, with a factor V Leiden mutation and a notion of chronic depression, admitted to our intensive care unit for a suicide attempt by ingestion ofmepronizine and lormetazepam. Data Extraction: We report the rare evolution of this patient with a persistent alteration of consciousness associating a respiratory degradation. Despite the drug intoxication and possibility of aspiration, we performed a computed tomography (CT) angiography which confirmed the presence of a bilateral, proximal, pulmonary embolism suspected on transthoracic echocardiography. A cerebral CT showed left sylvian and cerebellar infarctions complicated of perilesional edema. Association of stroke and pulmonary embolism led us to suspect a patent foramen ovale (PFO). There was also a context of genetical perturbation of hemostasis. Transesophageal echocardiography confirmed the presence of a PFO undiagnosed by transthoracic echography. The PFO was complicated by an entrapped thrombus. The thrombotic complications were treated by unfractionated heparin. Data Synthesis: Neurological and respiratory degradation following voluntary drug intoxication led to the discovery of both a pulmonary and cerebral embolism secondary to a PFO entrapped thrombus. Conclusions: An entrapped thrombus in a PFO is a rare and dangerous situation, associated with many complications. Association of systemic and pulmonary embolisms should lead to PFO detection to guide therapeutic interventions.

Prevel, Renaud; Garcon, Philippe; Philippart, François

2015-01-01

173

Hidden places, uncommon persons.  

PubMed

Specialized hospital units recently created to house and maintain ventilator or other technology-dependent persons in the United States are new cultural forms that enable beings who are neither fully alive, biologically dead, nor "naturally" self-regulating, yet who are sustained by modern medical practices, to exist. These institutions both fabricate and complicate the persons who are patients there through surveillance and maintenance of their conditions. This article concerns the relationship of person to place when the consciousness of an individual, considered to be the essence of personhood in the modern Western philosophical tradition, is problematic because the person resides in a technologically produced border zone between life and death. The article explores the ways in which place and person become implicated one another: first, how consciousness and thus personhood is assessed and negotiated through the inter-subjective knowledge of hospital staff; second, how that knowledge is tied to the particular situate-dness of patients; and third, how embodiment itself-the reflexive knowledge of the-self-in-the-body-is perceived as emplaced in social and spatial relations. PMID:12719179

Kaufman, Sharon R

2003-06-01

174

An Uncommon Cottage  

ERIC Educational Resources Information Center

This article features the Cliffs Cottage, a "showcase home" at Furman University which demonstrates the use of green technology in residential building and teaches about sustainability. Custom-built for the shelter-magazine dreams of "Southern Living," a sponsor of the home, the house seems better suited for a tony subdivision. The Cliffs Cottage…

Carlson, Scott

2008-01-01

175

Uncommon cause of pneumoperitoneum.  

PubMed

Free intraperitoneal air is thought to be pathognomonic for perforation of a hollow viscus. Here, we present a patient with pain in the upper left quadrant, a mild fever and leukocytosis. Free air was suggested under the left diaphragm but during the explorative laparotomy no signs of gastric or diverticular perforation were seen. Further exploration and revision of the computed tomography revealed a perforated splenic abscess. Splenic abscesses are a rare clinical entity. Presenting symptoms are often non-specific and include upper abdominal pain, recurrent or persistent fever, nausea and vomiting, splenomegaly, leukocytosis and left lower chest abnormalities. Predisposing conditions can be very divergent and include depressed immunosuppressed state, metastatic or contiguous infection, splenic infarction and trauma. Splenic abscess should therefore be considered in a patient with fever, left upper abdominal pain and leukocytosis. Moreover, our case shows that splenic abscess can present in an exceptional way without clear underlying aetiology and should even be considered in the presence of free abdominal air. PMID:24392184

van Nunspeet, Laura; Eddes, Eric Hans; de Noo, Mirre E

2013-12-27

176

[Differential diagnosis of elevated liver transaminases in rheumatic diseases].  

PubMed

During laboratory monitoring of patients with rheumatic diseases it is not uncommon to notice elevated liver transaminase levels. From a rheumatological perspective there are multiple causes for this. Liver dysfunction can be the result of certain rheumatological diseases, such as systemic lupus erythematosus. Primary biliary cirrhosis and primary sclerosing cholangitis are associated with rheumatic diseases. On the other hand, hepatological diseases, such as hepatitis C and autoimmune hepatitis show rheumatological symptoms. The most common cause of elevation of liver transaminase levels in rheumatic patients is without doubt the anti-rheumatic therapy. PMID:25691107

Mayet, W-J

2015-03-01

177

Salivary gland diseases in children  

PubMed Central

Salivary gland diseases in children are rare, apart from viral-induced diseases. Nevertheless, it is essential for the otolaryngologist to recognize these uncommon findings in children and adolescents and to diagnose and initiate the proper treatment. The present work provides an overview of the entire spectrum of congenital and acquired diseases of the salivary glands in childhood and adolescence. The current literature was reviewed and the results discussed and summarized. Besides congenital diseases of the salivary glands in children, the main etiologies of viral and bacterial infections, autoimmune diseases and tumors of the salivary glands were considered. In addition to the known facts, new developments in diagnostics, imaging and therapy, including sialendoscopy in obstructive diseases and chronic recurrent juvenile sialadenitis were taken into account. In addition, systemic causes of salivary gland swelling and the treatment of sialorrhoea were discussed. Although salivary gland diseases in children are usually included in the pathology of the adult, they differ in their incidence and some­times in their symptoms. Clinical diagnostics and especially the surgical treatment are influenced by a stringent indications and a less invasive strategy. Due to the rarity of tumors of the salivary glands in children, it is recommended to treat them in a specialized center with greater surgical experience. Altogether the knowledge of the differential diagnoses in salivary gland diseases in children is important for otolaryngologists, to indicate the proper therapeutic approach. PMID:25587366

Iro, Heinrich; Zenk, Johannes

2014-01-01

178

A free terminal ileal perforation from active crohn disease in pregnancy: a diagnostic challenge.  

PubMed

The surgical management of the complications of Crohn disease is often challenging. These difficulties are compounded in pregnancy by competing interests of the mother and the baby. In this report, we describe the presentation and surgical management of a patient in her second trimester with active Crohn disease who required emergent surgical intervention. She had presented with the uncommon complication of a free perforation in the presence of active untreated disease. PMID:25785326

Philip, Sunu; Kamyab, Armin; Orfanou, Paraskevi

2015-03-01

179

Parallel manifestation of Crohn's disease and acute pericarditis: a report of two cases.  

PubMed

Pericarditis is an uncommon extraintestinal manifestation of inflammatory bowel disease (IBD), which may occur at any time during its natural course. Moreover, it may be associated with the medications used to treat IBD, especially mesalamine. We report on two patients with acute pericarditis who were subsequently diagnosed with Crohn's disease. It is likely that mild, longstanding, virtually asymptomatic intestinal disease preceded the onset of pericarditis in both cases. PMID:17541784

Stasinopoulou, Panagiota; Kaziani, Aikaterini; Mantzaris, Gerassimos; Roussos, Anastasios; Skoutelis, Athanasios

2007-09-01

180

Mitogenomes from Two Uncommon Haplogroups Mark Late Glacial/Postglacial Expansions from the Near East and Neolithic Dispersals within Europe  

PubMed Central

The current human mitochondrial (mtDNA) phylogeny does not equally represent all human populations but is biased in favour of representatives originally from north and central Europe. This especially affects the phylogeny of some uncommon West Eurasian haplogroups, including I and W, whose southern European and Near Eastern components are very poorly represented, suggesting that extensive hidden phylogenetic substructure remains to be uncovered. This study expanded and re-analysed the available datasets of I and W complete mtDNA genomes, reaching a comprehensive 419 mitogenomes, and searched for precise correlations between the ages and geographical distributions of their numerous newly identified subclades with events of human dispersal which contributed to the genetic formation of modern Europeans. Our results showed that haplogroups I (within N1a1b) and W originated in the Near East during the Last Glacial Maximum or pre-warming period (the period of gradual warming between the end of the LGM, ?19 ky ago, and the beginning of the first main warming phase, ?15 ky ago) and, like the much more common haplogroups J and T, may have been involved in Late Glacial expansions starting from the Near East. Thus our data contribute to a better definition of the Late and postglacial re-peopling of Europe, providing further evidence for the scenario that major population expansions started after the Last Glacial Maximum but before Neolithic times, but also evidencing traces of diffusion events in several I and W subclades dating to the European Neolithic and restricted to Europe. PMID:23936216

Olivieri, Anna; Pala, Maria; Gandini, Francesca; Kashani, Baharak Hooshiar; Perego, Ugo A.; Woodward, Scott R.; Grugni, Viola; Battaglia, Vincenza; Semino, Ornella; Achilli, Alessandro; Richards, Martin B.; Torroni, Antonio

2013-01-01

181

Campylobacter jejuni, an uncommon cause of splenic abscess diagnosed by 16S rRNA gene sequencing.  

PubMed

Splenic abscess is a rare disease that primarily occurs in patients with splenic trauma, endocarditis, sickle cell anemia, or other diseases that compromise the immune system. This report describes a culture-negative splenic abscess in an immunocompetent patient caused by Campylobacter jejuni, as determined by 16S rRNA gene sequencing. PMID:25447730

Seng, Piseth; Quenard, Fanny; Menard, Amélie; Heyries, Laurent; Stein, Andreas

2014-12-01

182

Zygomycetes in Human Disease  

PubMed Central

The Zygomycetes represent relatively uncommon isolates in the clinical laboratory, reflecting either environmental contaminants or, less commonly, a clinical disease called zygomycosis. There are two orders of Zygomycetes containing organisms that cause human disease, the Mucorales and the Entomophthorales. The majority of human illness is caused by the Mucorales. While disease is most commonly linked to Rhizopus spp., other organisms are also associated with human infection, including Mucor, Rhizomucor, Absidia, Apophysomyces, Saksenaea, Cunninghamella, Cokeromyces, and Syncephalastrum spp. Although Mortierella spp. do cause disease in animals, there is no longer sufficient evidence to suggest that they are true human pathogens. The spores from these molds are transmitted by inhalation, via a variety of percutaneous routes, or by ingestion of spores. Human zygomycosis caused by the Mucorales generally occurs in immunocompromised hosts as opportunistic infections. Host risk factors include diabetes mellitus, neutropenia, sustained immunosuppressive therapy, chronic prednisone use, iron chelation therapy, broad-spectrum antibiotic use, severe malnutrition, and primary breakdown in the integrity of the cutaneous barrier such as trauma, surgical wounds, needle sticks, or burns. Zygomycosis occurs only rarely in immunocompetent hosts. The disease manifestations reflect the mode of transmission, with rhinocerebral and pulmonary diseases being the most common manifestations. Cutaneous, gastrointestinal, and allergic diseases are also seen. The Mucorales are associated with angioinvasive disease, often leading to thrombosis, infarction of involved tissues, and tissue destruction mediated by a number of fungal proteases, lipases, and mycotoxins. If the diagnosis is not made early, dissemination often occurs. Therapy, if it is to be effective, must be started early and requires combinations of antifungal drugs, surgical intervention, and reversal of the underlying risk factors. The Entomophthorales are closely related to the Mucorales on the basis of sexual growth by production of zygospores and by the production of coenocytic hyphae. Despite these similarities, the Entomophthorales and Mucorales have dramatically different gross morphologies, asexual reproductive characteristics, and disease manifestations. In comparison to the floccose aerial mycelium of the Mucorales, the Entomophthorales produce a compact, glabrous mycelium. The asexually produced spores of the Entomophthorales may be passively released or actively expelled into the environment. Human disease with these organisms occurs predominantly in tropical regions, with transmission occurring by implantation of spores via minor trauma such as insect bites or by inhalation of spores into the sinuses. Conidiobolus typically infects mucocutaneous sites to produce sinusitis disease, while Basidiobolus infections occur as subcutaneous mycosis of the trunk and extremities. The Entomophthorales are true pathogens, infecting primarily immunocompetent hosts. They generally do not invade blood vessels and rarely disseminate. Occasional cases of disseminated and angioinvasive disease have recently been described, primarily in immunocompromised patients, suggesting a possible emerging role for this organism as an opportunist. PMID:10756000

Ribes, Julie A.; Vanover-Sams, Carolyn L.; Baker, Doris J.

2000-01-01

183

Report of an uncommon cluster of septic arthritis of the hip in premature newborns: three cases in a period of 3 months.  

PubMed

We report an uncommon cluster of this rare condition which occurred within a very short period of time in our unit. We reviewed the current literature and observed that the diagnosis is often delayed, which can have very serious consequences for the outcome. Our 3 patients have had an uncomplicated course so far and follow-up examination at almost one year was normal. A high index of suspicion for the diagnosis and early and aggressive treatment is necessary. PMID:15999320

Kaczala, G; McDougall, J; Slongo, T; Nelle, M

2005-06-01

184

Unifying concept for Alzheimer's disease, vascular dementia and normal pressure hydrocephalus – a hypothesis  

Microsoft Academic Search

The three common forms of dementias in the elderly include Alzheimer's disease (AD), vascular dementia (VD) and normal pressure hydrocephalus (NPH). These disorders are distinguished by their specific pathological features. However, overlapping clinical and imaging features in a given case are not too uncommon. Based on alterations in CSF dynamics study, a unifying concept in the pathogenesis of AD and

Ambar Chakravarty

2004-01-01

185

Pathology of sickle cell disease.  

PubMed

Sickle cell disease (SCD) is a hereditary chronic hemolytic anemia with numerous clinical consequences. Intravascular sickling of red blood cells leads to multiorgan dysfunction. Although the pathophysiology of SCD has been well studied, there remains a lack of effective treatment. Refinements in overall care have improved quality of life; however, premature death is still not uncommon. SCD usually presents in childhood and is common in areas where malaria is (or was) common. The association with malaria is apparently of benefit to the individual because these individuals tend to contract a milder form of the disease. This review highlights the spectrum of pathology seen in people with SCD, with an emphasis on the pathogenesis of sudden death. PMID:22372205

Malowany, Janet I; Butany, Jagdish

2012-02-01

186

Bacteroides fragilis endocarditis in a patient with Crohn's disease.  

PubMed

Bacteroides fragilis is an uncommon cause of endocarditis and its occurrence in Crohn's disease has never been reported. We present a case of a B fragilis bacteraemia and endocarditis caused by seeding of left ventricular thrombus formed secondary to severe left ventricular dysfunction. A 44-years-old man with a history of persistent bloody diarrhoea for many years presented with 1-month duration of generalised weakness, malaise, fever and chills. The patient also developed right foot pain associated with cyanotic discolouration. On examination, he was cachectic and his right foot was pulseless, cold and blue in colour. Echocardiogram showed three intraventricular echo densities. Colonoscopy revealed multiple fistulous openings and blood cultures grew B fragilis. He was treated with intravenous metronidazole and underwent a proctocolectomy with ileostomy. Biopsy of the specimen confirmed Crohn's disease. This case emphasises the importance of identifying anaerobic bacteria as an uncommon but important cause of endocarditis and recognise likely source. PMID:23605837

Singh, Shailendra; Goyal, Vishal; Padhi, Parikshit; Aoun, Elie

2013-01-01

187

Localized Castleman disease of plasma cell type in the abdomen.  

PubMed

Castleman disease is a relatively rare entity, with the hyaline-vascular type the predominant form. Although the plasma cell type is uncommon, it still comprises approximately 10% of cases of localized diseases. In addition, the abdomen is a rare site for involvement and localized Castleman disease of the plasma cell type in the abdomen is rare. The radiologic features of localized plasma cell type in the abdomen are mostly limited to case reports. In addition to the conventional imaging findings, we present some new imaging findings of localized plasma cell type in the abdomen. PMID:22040445

Lu, Zhi-hua; Wu, Mei

2011-09-01

188

Cutaneous Rosai-Dorfman Disease presenting as a flank swelling  

PubMed Central

Rosai-Dorfman disease (RDD) is primarily a disease of lymph nodes caused by accumulation of excessive histiocytes. The extra-nodal sites are simultaneously involved in almost half the cases. However, presentation with isolated extra-nodal sites is uncommon and disease confined to skin and soft tissues is a rare entity. We report RDD in a 33 year old man who presented as a flank mass and was a diagnostic challenge for the surgical team. This case report strengthens the evidence that RDD should be added to the list of rare causes of skin and soft tissue swellings and informs how to manage such patients.

AlSalamah, Saleh M.; Abdullah, Muhammad; AlSalamah, Reem Abdullah; Al Hadlaq, Abdul Rehman Abdullah; Al Mogbel, EbtehalSulaiman; Al Yahya, Ghadah Mohammed

2014-01-01

189

Systemic vasculitis with prolonged pyrexia, recurrent facial urticaria, skin nodules, pleural effusions and venous thrombosis: an unusual presentation of an uncommon disease  

PubMed Central

Classically presenting with multiple or single peripheral cytopenias of variable severity, the myelodysplastic syndromes may occasionally present with bizarre manifestations that confuse the clinical picture and result in significant delays in making the correct diagnosis. We describe the case of an elderly male patient whose presentation with prolonged unexplained fever coupled with cutaneous, pulmonary and other systemic features of inflammation was finally diagnosed as having a primary myelodysplastic syndrome with associated vasculitis after a delay of 4 years. PMID:22031795

Hassan, Imad Salah; Dar, Javeed

2011-01-01

190

Some problems with Cochrane reviews of diet and chronic disease  

Microsoft Academic Search

The sum of evidence-based nutrition has to be more than a Cochrane-type meta-analysis of randomised controlled trials (RCTs). Most of the evidence base in nutrition is observational, especially cohort studies. RCTs of diet change through to disease outcome are uncommon and the change has usually been addition or removal of only a single food component. Trials with whole diets through

A S Truswell

2005-01-01

191

[Basedow disease in a young boy].  

PubMed

Hyperthyroidism in children is an uncommon disease. It occurs as congenital hyperthyroidism, caused by transplacental passage of thyroid-stimulating antibodies, and as Graves' disease (morbus basedowii), caused by thyroid-stimulating antibodies produced by lymphocytes. The latter has onset in pubertal children and affects girls five times as often as boys. Hyperthyroidism is very rarely seen outside these two age-groups. A case of Graves' disease in a 3 1/2-year-old boy exhibiting characteristic symptoms of hyperthyroidism is reported. In addition he had hypersalivation, a symptom not previously noticed in Graves' disease. The symptoms disappeared during antithyroid medication. At the age of nine a relapse necessitated a subtotal thyroidectomy. PMID:7694411

Andersen, U M

1993-10-01

192

A report of three cases of untreated Graves’ disease associated with pancytopenia in Malaysia  

PubMed Central

Generally, clinical presentations of Graves’ disease range from asymptomatic disease to overt symptomatic hyperthyroidism with heat intolerance, tremor, palpitation, weight loss, and increased appetite. However, atypical presentation of Graves’ disease with hematological system involvement, notably pancytopenia, is distinctly uncommon. Hereby, we present and discuss a series of three untreated cases of Graves’ disease clinically presented with pancytopenia and the hematological abnormalities that responded well to anti-thyroid treatment. With resolution of the thyrotoxic state, the hematological parameters improved simultaneously. Thus, it is crucial that anti-thyroid treatment be considered in patients with Graves’ disease and pancytopenia after a thorough hematological evaluation.

Rafhati, Abdullah Noor; See, Chee Keong; Hoo, Fan Kee; Badrulnizam, Long Bidin Mohamed

2014-01-01

193

Malignant degeneration of multilevel monostotic Paget's disease involving the thoracic spine: an unusual presentation.  

PubMed

Paget's disease is the second most common metabolic bone disease after osteoporosis and is characterized by abnormal bone turnover and remodeling that can lead to pain, pathological fracture, bony deformity and nerve compression syndromes. The lumbar region is the most commonly affected site within the spine followed by the thoracic and cervical spine. Even though the spine is affected very commonly in Paget's disease, malignant degeneration is exceptionally rare. Multilevel monostotic spine involvement due to Paget's disease is very uncommon. An unusual clinico-radiological manifestation of multilevel thoracic Paget's disease with sarcomatous degeneration presenting as a neurosurgical emergency is reported with a pertinent review of the literature. PMID:24411323

Tan, Lee A; Kasliwal, Manish K; Harbhajanka, Aparna; Miller, Ira J; Deutsch, Harel

2014-07-01

194

Extranodal Castleman disease of the extremities: a case report and review of the literature.  

PubMed

Castleman disease is a rare lymphoproliferative disorder of unknown etiology that most commonly presents as a mediastinal nodal mass or, in the extranodal form of the disease, a mass located in the mediastinum or retroperitoneum. It is exceptionally uncommon for Castleman disease to present in the extremities. We report a rare case of extranodal Castleman disease presenting as a muscular forearm mass. We compare our case with the seven other reported cases in which Castleman disease presented as an isolated soft tissue mass in the extremities. PMID:24970669

Eward, William C; DeWitt, Suzanne B; Brigman, Brian E; Kontogeorgakos, Vasilios; Lagoo, Anand S

2014-11-01

195

Dystonia in Parkinson’s disease  

Microsoft Academic Search

\\u000a Abstract\\u000a   Dystonia can occasionally be found in idiopathic Parkinson’s disease. It is very uncommon in untreated patients and is more\\u000a frequently seen as a complication of its treatment. In this review, the various types of dystonia occurring in PD, the differential\\u000a diagnosis with other parkinsonian syndromes associated with dystonia and treatments available are revised.\\u000a \\u000a Dystonia unrelated to treatment can be

Eduardo Tolosa; Yaroslau Compta

2006-01-01

196

Cutaneous metastases presenting as genital ulcer disease.  

PubMed

Cutaneous metastasis from an internal organ malignancy is rare and as, the presenting sign of malignancy is an uncommon phenomenon. Their presence, signals a poor prognosis. We report a case of 50-year-old female who was referred to sexually transmitted diseases - out patient department, with complaints of multiple genital ulcers to rule out sexually transmitted infections. After thorough evaluation, she was found to be a case of carcinoma cervix with metastatic squamous cell carcinomatous deposits on external genitalia. This case was unique because of relatively asymptomatic nature of internal malignancy and atypical presentation of carcinoma cervix as cutaneous metastasis. PMID:24958986

Vasuki, S; Durgalakshmi, J; Latha, J

2014-01-01

197

Minimal change disease associated with MALT lymphoma  

Microsoft Academic Search

Low-grade Extranodal Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, a subtype of non-Hodgkin’s Lymphoma,\\u000a involving the kidney is a rare clinical entity. Association of Minimal change disease nephrotic range proteinuria with Hodgkin’s\\u000a lymphoma is well described, however is extremely uncommon with non-Hodgkin’s lymphoma. We describe a patient who presented\\u000a with nephrotic syndrome and a kidney biopsy revealed marginal zone

Rahil Kasmani; Vamsee Priya Marina; Syed Abidi; Bikram Johar; Deepak Malhotra

198

Giardia lamblia infestation reveals underlying Whipple's disease in a patient with longstanding constipation.  

PubMed

Whipple's disease is an uncommon disorder, generally associated with gastrointestinal symptoms; of these, diarrhea is a common feature. We report a case of Whipple's disease associated with chronic constipation which was not diagnosed until after Giardia lamblia infestation had caused diarrhea. To the best of our knowledge this association has not previously been reported. The clinical, laboratory, endoscopic, and manometric aspects are described and discussed. PMID:1705390

Bassotti, G; Pelli, M A; Ribacchi, R; Miglietti, M; Cavalletti, M L; Rossodivita, M E; Giovenali, P; Morelli, A

1991-03-01

199

Vegetarian Dietary Patterns as a Means to Achieve Reduction in Cardiovascular Disease and Diabetes Risk Factors  

Microsoft Academic Search

Cardiovascular disease and type 2 diabetes are uncommon in people consuming vegetarian and vegan diets. Vegetarian and vegan\\u000a dietary patterns tend to result in lower body weight and better nutritional profiles than conventional healthy eating patterns\\u000a and have been shown to be an effective tool for management of cardiovascular disease and diabetes risk. The consistency of\\u000a observed beneficial outcomes with

Amy Joy Lanou; Barbara Svenson

2010-01-01

200

Identification of Uncommon Recurrent Potocki-Lupski Syndrome-Associated Duplications and the Distribution of Rearrangement Types and Mechanisms in PTLS  

PubMed Central

Nonallelic homologous recombination (NAHR) can mediate recurrent rearrangements in the human genome and cause genomic disorders. Smith-Magenis syndrome (SMS) and Potocki-Lupski syndrome (PTLS) are genomic disorders associated with a 3.7 Mb deletion and its reciprocal duplication in 17p11.2, respectively. In addition to these common recurrent rearrangements, an uncommon recurrent 5 Mb SMS-associated deletion has been identified. However, its reciprocal duplication predicted by the NAHR mechanism had not been identified. Here we report the molecular assays on 74 subjects with PTLS-associated duplications, 35 of whom are newly investigated. By both oligonucleotide-based comparative genomic hybridization and recombination hot spot analyses, we identified two cases of the predicted 5 Mb uncommon recurrent PTLS-associated duplication. Interestingly, the crossovers occur in proximity to a recently delineated allelic homologous recombination (AHR) hot spot-associated sequence motif, further documenting the common hot spot features shared between NAHR and AHR. An additional eight subjects with nonrecurrent PTLS duplications were identified. The smallest region of overlap (SRO) for all of the 74 PTLS duplications examined is narrowed to a 125 kb interval containing only RAI1, a gene recently further implicated in autism. Sequence complexities consistent with DNA replication-based mechanisms were identified in four of eight (50%) newly identified nonrecurrent PTLS duplications. Our findings of the uncommon recurrent PTLS-associated duplication at a relative prevalence reflecting the de novo mutation rate and the distribution of 17p11.2 duplication types in PTLS reveal insights into both the contributions of new mutations and the different underlying mechanisms that generate genomic rearrangements causing genomic disorders. PMID:20188345

Zhang, Feng; Potocki, Lorraine; Sampson, Jacinda B.; Liu, Pengfei; Sanchez-Valle, Amarilis; Robbins-Furman, Patricia; Navarro, Alicia Delicado; Wheeler, Patricia G.; Spence, J. Edward; Brasington, Campbell K.; Withers, Marjorie A.; Lupski, James R.

2010-01-01

201

An outbreak of salmonella chester infection in Canada: rare serotype, uncommon exposure, and unusual population demographic facilitate rapid identification of food vehicle.  

PubMed

Salmonella Chester infection has rarely been reported in the literature. In 2010, 33 case patients were reported in 2 months in four Canadian provinces. We conducted an outbreak investigation in collaboration with public health agencies, food safety specialists, regulatory agencies, grocery store chains, and the product distributor. We used case patient interviews, customer loyalty cards, and microbiological testing of clinical and food samples to identify nationally distributed head cheese as the food vehicle responsible for the outbreak. The rare serotype, a limited affected demographic group, and an uncommon exposure led to the rapid identification of the source. Control measures were implemented within 9 days of notification of the outbreak. PMID:22488063

Taylor, John; Galanis, Eleni; Wilcott, Lynn; Hoang, Linda; Stone, Jason; Ekkert, Judi; Quibell, Doug; Huddleston, Mark; McCormick, Rachel; Whitfield, Yvonne; Adhikari, Bijay; Grant, Christopher C R; Sharma, Davendra

2012-04-01

202

Increased frequency of the uncommon allele of a tumour necrosis factor alpha gene polymorphism in rheumatoid arthritis and systemic lupus erythematosus.  

PubMed

The frequency of the uncommon allele (TNF2) of a polymorphism in the promoter region of the tumour necrosis factor alpha (TNF alpha) gene in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) was found to be 3 times that of the normal anglo-saxon population. In SLE patients, this allele was strongly associated with HLA-DR3 expression and was also more frequent in patients who did not have malar rash. Functional studies of normal monocyte cytokine production in vitro showed that this genotype was associated with increased IL-1 alpha protein production but there were no differences in the production of TNF alpha protein. PMID:7614782

Danis, V A; Millington, M; Hyland, V; Lawford, R; Huang, Q; Grennan, D

1995-03-01

203

Posterior cortical atrophy in Alzheimer's disease: analysis of a new case and re-evaluation of a historical report  

Microsoft Academic Search

Disturbances of visual function are not uncommon in Alzheimer's disease and several cases with complex impairment of visuospatial abilities have been described. For instance, posterior cortical atrophy has been demonstrated in cases displaying Balint's syn-drome as the first symptom of the dementing illness. Such cases showed very high lesion counts in the occipital cortex, as well as in visual association

P. R. Hof; N. Archin; A. P. Osmand; J. H. Dougherty; C. Wells; C. Bouras; J. H. Morrison

1993-01-01

204

Imatinib Added to Chemotherapy Keeps Disease in Check for Longer in Children with Rare Form of Acute Lymphoblastic Leukemia  

Cancer.gov

The addition of the targeted drug imatinib to chemotherapy for children with an uncommon form of acute lymphoblastic leukemia (ALL) dramatically improved the length of time the disease remained in remission, according to the November 1, 2009, Journal of Clinical Oncology.

205

Myeloproliferative disease in a cat  

SciTech Connect

Myeloproliferative disorders, a complex of cytologic abnormalities arising in the bone marrow, are among domestic animals most frequently recognized in cats but are relatively uncommon. A 4-year-old female Siamese, with splenomegaly and weight loss, was listless, anorectic, pale and dehydrated. A hemogram showed severe, macrocytic normochromic anemia, leukocytosis and reticulocytosis, with abnormally high numbers of nucleated RBC and undifferentiated blast cells. Bone marrow smears contained predominantly undifferentiated blast cells, RBC precursors and myeloblasts. The fluorescent antibody test for FeLV was positive. The cat died 66 days later despite a blood transfusion and chemotherapy. Necropsy confirmed a diagnosis of myeloproliferative disease, with hepatic and splenic invasion. 15 references, 5 figures, 1 table.

Yates, R.W.; Weller, R.E.; Feldman, B.F.

1984-10-01

206

Diarrhoea due to pancreatic diseases.  

PubMed

The exocrine pancreas provides essential digestive enzymes necessary for the proper breakdown and absorption of ingested food in humans. Any disruption of this process can lead to malabsorption and resultant diarrhoea. Typically, disruption of over 90 percent of the pancreatic parenchyma is needed to result in diarrhoea. This disruption can result from widespread pancreatic necrosis in acute pancreatitis, fibrotic replacement of the parenchyma as seen in chronic pancreatitis and in patients with pancreatic cancer where normal tissue is replaced by tumour and/or the pancreatic duct becomes obstructed. Several uncommon tumours of the pancreas can also cause diarrhoea through the secretion of hormones. This article will explore each of these diseases, including the pathogenesis and treatment. PMID:23384807

Brelian, Daniel; Tenner, Scott

2012-10-01

207

[Goodpasture's disease: a case series].  

PubMed

Goodpasture's disease (GD) is an uncommon and severe autoimmune disorder caused by circulating autoantibodies directed against the glomerular basement membrane cross-reacting with the alveolar basement membrane. GD is clinically characterized by rapidly progressive glomerulonephritis, often associated with pulmonary hemorrhage representing a nephrological emergency. We present the clinical features of 9 cases, diagnosed in 1997-2012, in our Renal Unit. Contrary to previous reports, we found a predominance of GD in females and we observed unusual clinical patterns, such as the association with renal vein thrombosis in a pregnant patient, thrombosis of the pulmonary arteries and a late isolated recurrence of alveolitis. In dialysis-dependent patients, renal transplantation can represent an available treatment option. PMID:24671842

Dugo, Mauro; Pasi, Alessandra; Gatti, Pier Luigi; Mastrosimone, Stefania; Laurino, Licia; Barberio, Giuseppina; Farneti, Fabrizio; Facchini, Cesarina; Maresca, Maria Cristina

2014-01-01

208

Multicentric Castleman's Disease in a Child Revealed by Chronic Diarrhea  

PubMed Central

Multicentric Castleman's disease is a rare benign and unexplained lymphoproliferative disorder that is extremely uncommon in children. It presents with fever, systemic symptoms, generalized lymphadenopathy, and laboratory markers of inflammation. Its treatment is not standardized and its prognosis is poor. We report a novel case of multicentric Castleman's disease in a 13-year-old girl who had presented with chronic diarrhea as the only initial presenting symptom. The diagnosis of celiac or inflammatory bowel diseases was suspected, but two and a half years later, the diagnosis of multicentric Castleman's disease was brought following the appearance of abdominal mass whose biopsy revealed Castleman's disease in the plasma cell form. The outcome was favorable after treatment by corticosteroid, chemotherapy, and surgery. The occurrence of diarrhea as the initial symptom of multicentric Castleman's disease without lymph node involvement is very rare. This case report underlines the diagnostic difficulties and the long interval between onset and diagnosis when diarrhea occurs first. PMID:25737793

Benmiloud, Sarra; Chaouki, Sana; Atmani, Samir; Hida, Moustapha

2015-01-01

209

The role of glia in retinal vascular disease.  

PubMed

Retinal vascular diseases collectively represent a leading cause of blindness. Unsurprisingly, pathological characterisation and treatment of retinal 'vascular' diseases have primarily focused on the aetiology and consequences of vascular dysfunction. Far less research has addressed the contribution of neuronal and glial dysfunction to the disease process of retinal vascular disorders. Ample evidence now suggests that retinal vasculopathy only uncommonly occurs in isolation, usually existing in concert with neuropathy and gliopathy. Retinal glia (Müller cells, astrocytes and microglia) have been reported to exhibit morphological and functional changes in both early and advanced phases of almost every retinal vascular disease. It is anticipated that identifying the causes of glial activation and dysfunction, and their contribution to loss of vision in retinal vascular disease, will lead to a better understanding of retinal vascular diseases, which might ultimately be translated into novel clinical therapies. PMID:22519424

Coorey, Nathan J; Shen, Weiyong; Chung, Sook H; Zhu, Ling; Gillies, Mark C

2012-05-01

210

Parkinson's Disease  

MedlinePLUS

... Brain Stimulation Consortium Meeting Summary Parkinson's Disease Cell Biology Meeting Summary Parkinson's Disease Cell Biology Meeting Summary Udall Centers Meeting—Expediting Parkinson’s Disease ...

211

Pick disease  

MedlinePLUS

Semantic dementia; Dementia - semantic; Frontotemporal dementia; Arnold Pick disease ... can help doctors tell Pick disease apart from Alzheimer disease. (Memory loss is often the main, and earliest, ...

212

Cardiovascular adaptation and cardiac disease in the elite athlete.  

PubMed

Cardiovascular diseases are uncommon among trained athletes. Their occurrences mostly depend on the individual's age and fitness levels. Adequate understanding of the cardiovascular adaptations undergone by the competitive athletes' heart is of paramount importance in order to differentiate them from serious cardiovascular conditions. Diagnosing these abnormalities early may prevent rare but devastating potential complications associated with athletic activities and defines appropriate activity restrictions to minimize the risk of sudden cardiac death. This article will review concerns related to competitive athlete's cardiovascular adaptations and diseases, in light of specific recommendations presented in the 36th Bethesda Conference guidelines. PMID:23882976

Del Río-santiago, Valentín; Santiago Trinidad, Ricardo; Vicenty Rivera, Sonia

2012-01-01

213

Tumour Calcification and Calciphylaxis in End-Stage Renal Disease  

PubMed Central

Although soft tissue and vascular calcifications are common in CKD and progress as an independent risk factor of all-cause mortality, tumour calcification and calciphylaxis are uncommon in patients with end-stage renal disease (ESRD). Here, we discuss a rare case of a patient with tumour calcification complicated with calciphylaxis developed septic shock from infection. Our patient is a 57-year-old man in his late stage of renal disease who presented with a huge mass at the right hip and necrotic cutaneous ulcers on the lower legs followed by local and systemic infection and death due to septic shock. PMID:25477966

Di, Jia; Jiang, Zhenxing; Yang, Min

2014-01-01

214

Primary Testicular Carcinoid Tumor presenting as Carcinoid Heart Disease  

PubMed Central

Primary carcinoid tumors of the testis are very rare, and they seldom present with carcinoid syndrome. We report a hereto unreported instance, where a patient with a long-standing testicular mass presented with carcinoid heart disease, an uncommon form of carcinoid syndrome. He presented with symptoms of right heart failure, episodic facial flushing and was found to have severe right-sided valvular heart disease. His urinary 5-hydroxy indole acetic acid level was elevated. He underwent orchidectomy and the histopathology confirmed a testicular carcinoid tumor. PMID:25624579

Chikkaraddi, Manjunath L.; Krishna, Sudeep; Shetty, Manjunath

2015-01-01

215

Zinc absorption in inflammatory bowel disease  

SciTech Connect

Zinc absorption was measured in 29 patients with inflammatory bowel disease and a wide spectrum of disease activity to determine its relationship to disease activity, general nutritional state, and zinc status. Patients with severe disease requiring either supplementary oral or parenteral nutrition were excluded. The mean 65ZnCl2 absorption, in the patients, determined using a 65Zn and 51Cr stool-counting test, 45 +/- 17% (SD), was significantly lower than the values, 54 +/- 16%, in 30 healthy controls, P less than 0.05. Low 65ZnCl2 absorption was related to undernutrition, but not to disease activity in the absence of undernutrition or to zinc status estimated by leukocyte zinc measurements. Mean plasma zinc or leukocyte zinc concentrations in patients did not differ significantly from controls, and only two patients with moderate disease had leukocyte zinc values below the 5th percentile of normal. In another group of nine patients with inflammatory bowel disease of mild-to-moderate severity and minimal nutritional impairment, 65Zn absorption from an extrinsically labeled turkey test meal was 31 +/- 10% compared to 33 +/- 7% in 17 healthy controls, P greater than 0.1. Thus, impairment in 65ZnCl2 absorption in the patients selected for this study was only evident in undernourished persons with moderate or severe disease activity, but biochemical evidence of zinc deficiency was uncommon, and clinical features of zinc depletion were not encountered.

Valberg, L.S.; Flanagan, P.R.; Kertesz, A.; Bondy, D.C.

1986-07-01

216

Lentil Diseases  

Technology Transfer Automated Retrieval System (TEKTRAN)

Major lentil diseases around the world have been described and reviewed. The major diseases include Ascochyta blight, Fusarium wilt, Botrytis Gray Mold, Lentil rust, Stemphylium blight, Anthracnose, and virus diseases. The management practices for these diseases are also presented....

217

Meningococcal Disease  

MedlinePLUS

... Disease Mumps Pertussis (Whooping Cough) Pneumococcal Disease Rubella (German Measles) Shingles (Herpes Zoster) Tetanus (Lockjaw) Professional Resources ... Disease Mumps Pertussis (Whooping Cough) Pneumococcal Disease Rubella (German Measles) Shingles (Herpes Zoster) Tetanus (Lockjaw) You May ...

218

Lyme Disease  

MedlinePLUS

... Is Lyme Disease? People get Lyme disease through tick bites. The disease is caused by a bacterium called ... a sesame seed. It's easy to overlook a tick bite. Many people who get Lyme disease don't ...

219

Isolated lacrimal gland involvement in Rosai-Dorfman-Destombes disease  

PubMed Central

Rosai-Dorfman-Destombes (sinus histiocytosis with massive lymphadenopathy) disease is an uncommon disease characterized by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal disease. Orbital involvement occurs in 9-11% of cases. However, isolated Rosai- Dorfman-Destombes disease of the lacrimal gland without any systemic involvement is very rare with only three case reports. We describe here one such young male patient with unilateral lacrimal gland swelling. Excision biopsy revealed almost complete replacement of the lacrimal gland by lymphocytes, plasma cells and large pale histiocytes. The latter exhibited emperipolesis and stained positive for S-100 and CD68 on immunohistochemistry. Patient is well and has no other manifestation or recurrence of the disease during a follow-up of 24 months. PMID:18974525

Bhalla, Sunita; Srivastava, Amit

2008-01-01

220

Controlling distant metastasis and surgical treatment are crucial for improving clinical outcome in uncommon head and neck malignancies, such as non-squamous cell carcinoma  

PubMed Central

The objective of this study was to elucidate the clinical characteristics of uncommon head and neck malignancies, such as non-squamous cell carcinoma (SCC), in order to improve patient outcomes. A total of 463 head and neck malignancies were retrospectively analyzed, with 43 cases (9.3%) diagnosed as non-SCC. The overall survival rate of patients with adenoid cystic carcinoma was significantly worse compared to that of patients with SCC. The 5-year survival rates were <50% for patients with malignant melanoma, adenocarcinoma, small-cell carcinoma and sarcomas. Distant metastasis to the lung was frequently observed in cases with a poor outcome. Non-SCC malignancies treated without surgery were associated with a worse outcome. Some non-SCC patients had a poor prognosis and distant metastasis was associated with an unsatisfactory outcome. Timely treatment and control of distant metastasis are essential and surgical treatment should be prioritized in non-SCC cases to improve patient outcomes. PMID:24940505

SHIIBA, MASASHI; UNOZAWA, MOTOHARU; HIGO, MORIHIRO; KOUZU, YUKINAO; KASAMATSU, ATSUSHI; SAKAMOTO, YOSUKE; OGAWARA, KATSUNORI; UZAWA, KATSUHIRO; TAKIGUCHI, YUICHI; TANZAWA, HIDEKI

2014-01-01

221

Long-term disease-free survival after surgical resection for multiple bone metastases from rectal cancer.  

PubMed

Bone metastasis of primary colorectal cancer is uncommon. When it occurs, it is usually a late manifestation of disease and is indicative of poor prognosis. We describe a patient with multiple metachronous bone metastases from lower rectal cancer who was successfully treated with multimodal treatment including surgical resections and has shown 32 mo disease-free survival. Surgical resection of metastatic bone lesion(s) from colorectal cancer may be a good treatment option in selected patients. PMID:21876853

Choi, Seok Jin; Kim, Jong Hun; Lee, Min Ro; Lee, Chang Ho; Kuh, Ja Hong; Kim, Jung Ryul

2011-08-10

222

Transitional Cell Carcinoma of the Kidney Graft: An Extremely Uncommon Presentation of Tumor in Renal Transplant Recipients  

PubMed Central

Purpose. Transitional cell carcinoma (TCC) affecting the graft after renal transplantation is a very infrequent way of presentation of this tumor. Our aim is to present our single institution experience with 2 cases, as well as to perform a review of the literature about this tumor after the transplant. Materials and Methods. TCC of the graft developed in 2 of 1365 patients from 1977 to 2010, both cases in women. Data were analyzed for incidence, clinical presentation, treatment, and outcomes. Results. Both cases occurred in 2 mid-age women and resulted to be high grade and locally advanced TCCs, representing an incidence of 0,14% (2/1365). Clinical presentation was urinary obstruction for the first case and incidental ultrasound finding for the second. Preoperative staging was made with CT, cytology, pyelography, ureterorenoscopy, and biopsy. Treatment performed was nephroureterectomy of the graft with bladder cuff and regional lymphadenectomy. Pathological examination showed in both cases a locally advanced and high grade urothelial carcinoma of the pelvis allograft. After 24 and 14 months of followup, both patients are disease free. Conclusions. TCC of the kidney graft is an infrequent tumor that has only been reported in a few cases in the literature. It usually appears at a lower age, more often locally advanced, and with poor differentiation. A multidisciplinary approach to treatment should be required in these cases. PMID:23781380

Gómez, Victoria; Álvarez, Sara; Díez Nicolás, Víctor; Gómez del Cańizo, Carmen; Orosa, Andrea; Galeano Álvarez, Cristina; Burgos Revilla, F. J.

2013-01-01

223

Imaging of diffuse liver disease.  

PubMed

Advances in imaging technology and development of liver-specific contrast agents have significantly increased the role of radiology in the detection and characterization of processes diffusely involving the liver. Tailored magnetic resonance imaging (MRI) sequences allow an accurate detection of many storage and metabolic diseases, such as iron overload disorders and steatosis (fatty liver). Faster scanning techniques available with both computed tomography (CT) and MRI provide, by assessing contrast dynamics, sufficient information for the characterization of diffuse neoplastic and vascular disorders. Characteristic changes in attenuation on CT, signal intensity on MRI, and enhancing features can be used to diagnose specific diffuse diseases such as candidiasis, diffuse/multifocal hepatocellular carcinoma, and schistosomiasis. Although an overlap in imaging findings still exists, familiarity with the imaging features of uncommon disorders such as Wilson's disease, amyloidosis, and sarcoidosis may be diagnostic in the proper clinical setting. This review focuses on the current role of imaging in the detection and characterization of diffuse liver disorders. Recent developments that have amplified the role of noninvasive diagnostic evaluation of these conditions are especially highlighted. PMID:11436572

Mortele, K J; Ros, P R

2001-05-01

224

Identification of novel biomarker candidates by proteomic analysis of cerebrospinal fluid from patients with moyamoya disease using SELDI-TOF-MS  

Microsoft Academic Search

BACKGROUND: Moyamoya disease (MMD) is an uncommon cerebrovascular condition with unknown etiology characterized by slowly progressive stenosis or occlusion of the bilateral internal carotid arteries associated with an abnormal vascular network. MMD is a major cause of stroke, specifically in the younger population. Diagnosis is based on only radiological features as no other clinical data are available. The purpose of

Yoshio Araki; Kazuhiro Yoshikawa; Sho Okamoto; Masaki Sumitomo; Mikio Maruwaka; Toshihiko Wakabayashi

2010-01-01

225

Commentary: is Alzheimer's disease uniquely human?  

PubMed

That Alzheimer's disease (AD) might be a human-specific disease was hypothesized by Rapoport in 1989. Apes and humans share an identical amyloid beta (A?) peptide amino acid sequence and accumulate considerable A? deposits after age 40 years, an age when amyloid plaques are uncommon in humans. Despite their early A? buildup, ape brains have not shown evidence dystrophic neurites near plaques. Aging great ape brains also have few neurofibrillary tangles, with one exception of 1 obese chimpanzee euthanized after a stroke who displayed abundant neurofibrillary tangles, but without the typical AD distribution. We discuss the need for more exacting evaluation of neuron density with age, and note husbandry issues that may allow great apes to live to greater ages. We remain reserved about expectations for fully developed AD-like pathology in the great apes of advanced ages and cautiously support Rapoport's hypothesis. PMID:25533426

Finch, Caleb E; Austad, Steven N

2015-02-01

226

Commentary: is Alzheimer's disease uniquely human?  

PubMed Central

That Alzheimer's disease (AD) might be a human-specific disease was hypothesized by Rapoport in 1989. Apes and humans share an identical amyloid beta (A?) peptide amino acid sequence and accumulate considerable A? deposits after age 40 years, an age when amyloid plaques are uncommon in humans. Despite their early A? buildup, ape brains have not shown evidence dystrophic neurites near plaques. Aging great ape brains also have few neurofibrillary tangles, with one exception of 1 obese chimpanzee euthanized after a stroke who displayed abundant neurofibrillary tangles, but without the typical AD distribution. We discuss the need for more exacting evaluation of neuron density with age, and note husbandry issues that may allow great apes to live to greater ages. We remain reserved about expectations for fully developed AD-like pathology in the great apes of advanced ages and cautiously support Rapoport's hypothesis. PMID:25533426

Finch, Caleb E.; Austad, Steven N.

2015-01-01

227

Pleural effusion in hepatic vena cava disease.  

PubMed

Pleural effusion is not uncommon in developing countries. It is usually considered to be due to tuberculosis and treated with anti-tubercular chemotherapy without much diagnostic workup. Hepatic vena cava disease (HVD), a disease caused by obliterative lesion of the hepatic portion of inferior vena cava induced by bacterial infection is common in developing countries. We report here the occurrence of pleural effusion in 10% of the cases of HVD. Four patients, one with acute and three with chronic HVD that presented with pleural effusion are described. Pleural effusion in HVD responded to treatment with antibiotic and diuretic. In developing countries HVD should be considered in the differential diagnosis of pleural effusion. It is postulated that bacterial infection and sodium retention resulting from acute caval obstruction are important in the pathogenesis of pleural effusion in HVD. PMID:18604023

Shrestha, S M

2007-01-01

228

Chagas Disease  

MedlinePLUS

... Health Division of Parasitic Diseases and Malaria CS226359 Chagas Disease Fact Sheet What is Chagas disease? ? A disease that can cause serious heart ... benchuca,” “vinchuca,” “chinche,” or “barbeiro” Who can get Chagas disease? Anyone. However, people have a greater chance ...

229

VESICULAR DISEASES  

Technology Transfer Automated Retrieval System (TEKTRAN)

The principal vesicular diseases that affect swine are foot-and-mouth disease (FMD), vesicular stomatitis (VS), and swine vesicular diseases (SVD). There are other infectious diseases and conditions that can produce signs and pathologies in pigs similar to those seen in these three viral diseases, ...

230

Alzheimer's Disease  

MedlinePLUS

... version of this page please turn Javascript on. Alzheimer's Disease What Is Alzheimer's Disease? Alzheimer’s disease is a brain disease that slowly ... it has no cure. A Common Cause of Dementia Alzheimer’s disease is the most common cause of ...

231

Foodborne Diseases  

MedlinePLUS

... JavaScript on. Read more information on enabling JavaScript. Foodborne Diseases Top Banner Content Area Skip Content Marketing Share ... on a clean kitchen countertop. Credit: CDC. Understanding Foodborne Diseases Infectious diseases spread through food or beverages are ...

232

Crohn's Disease  

MedlinePLUS

Crohn's disease causes inflammation of the digestive system. It is one of a group of diseases called inflammatory ... small intestine called the ileum. The cause of Crohn's disease is unknown. It may be due to an ...

233

Alzheimer's Disease  

MedlinePLUS

Alzheimer's disease (AD) is the most common form of dementia among older people. Dementia is a brain disorder that ... higher if a family member has had the disease. No treatment can stop the disease. However, some ...

234

Infectious Diseases  

MedlinePLUS

Infectious diseases kill more people worldwide than any other single cause. Infectious diseases are caused by germs. Germs are tiny living ... live NIH: National Institute of Allergy and Infectious Diseases

235

Bone Diseases  

MedlinePLUS

... break Osteogenesis imperfecta makes your bones brittle Paget's disease of bone makes them weak Bone disease can make bones easy to break Bones can also develop cancer and infections Other bone diseases are caused by poor nutrition, genetic factors or ...

236

Behcet's Disease  

MedlinePLUS

... Old Silk Route,” which spans the region from Japan and China in the Far East to the ... the disease’s epidemiology is not well understood. In Japan, Behcet’s disease ranks as a leading cause of ...

237

Parkinson's Disease  

MedlinePLUS

... symptoms of something called Parkinson's disease. What Is Parkinson's Disease? You may have seen the actor Michael J. ... it needs to move normally. Continue What Causes Parkinson's Disease? Experts agree that low dopamine levels in the ...

238

Reportable diseases  

MedlinePLUS

... be reported to the U.S. Centers for Disease Control and Prevention (CDC). Reportable diseases are divided into several groups: Mandatory written reporting: A report of the disease must be made ...

239

Pompe Disease  

MedlinePLUS

... Manifestations of Pompe Disease, Acid Maltase Deficiency, Glycogen Storage Disease Table of Contents (click to jump to ... Clinical Trials Organizations Column1 Column2 Association for Glycogen Storage Disease P.O. Box 896 Durant, IA 52747 ...

240

Lyme Disease  

MedlinePLUS

... Lyme Disease Top Banner Content Area Skip Content Marketing Share this: Main Content Area Understanding Lyme Disease ... about NIAID Lyme disease research . Research at NIAID Diagnostic Research Co-Infection Antibiotic Therapy Vaccines Research in ...

241

Farber's Disease  

MedlinePLUS

... a group of inherited metabolic disorders called lipid storage diseases, in which excess amounts of lipids (oils, ... Institutes of Health (NIH), conducts research about lipid storage diseases such as Farber’s disease in laboratories at ...

242

Huntington's Disease  

MedlinePLUS

Huntington's disease (HD) is an inherited disease that causes certain nerve cells in the brain to waste away. ... express emotions. If one of your parents has Huntington's disease, you have a 50 percent chance of getting ...

243

Krabbe Disease  

MedlinePLUS

... Krabbe Disease? Krabbe disease is a rare, inherited degenerative disorder of the central and peripheral nervous systems. ... receive umbilical cord blood stem cells prior to disease onset or early bone marrow transplantation. Persons with juvenile- or adult-onset ...

244

Recurrent glomerular disease in the kidney allograft.  

PubMed

Glomerulonephritis is responsible for nearly 15% of prevalent end-stage renal disease, and many of these patients will receive kidney transplants with the potential for a long duration of allograft survival. Recurrent glomerular disease, however, is not uncommon and can lead to both substantial morbidity and/or loss of the kidney allograft. The timing of recurrence after transplantation as well as the prevalence of recurrent disease vary by study, especially accounting for differences in protocol versus clinically-indicated biopsies, the use of immunofluorescence or electron microscopy in histopathological evaluation, and length of follow-up. Transplant immunosuppression alone may be sufficient to keep some recurrent disease in a subclinical form, whereas other recurrent glomerular diseases may be clinically evident and progress to threaten the allograft. This review highlights the epidemiology, diagnosis, and treatment of five common glomerular diseases that may recur in the transplant: focal and segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN), immunoglobulin A nephropathy (IgAN), and lupus nephritis (LN). PMID:25553369

Menn-Josephy, Hanni; Beck, Laurence H

2015-01-01

245

Lyme disease in the United Kingdom.  

PubMed

Lyme disease, while still an uncommon disease in the UK, is on the increase. Case numbers have increased by 3.6-fold since 2001, with over 950 cases reported by the Health Protection Agency (HPA) in 2011, compared with less than 500 cases annually pre-2004. HPA indications of the true incidence are suggested to be closer to 3000 cases/year, of which around 82% of cases are indigenously acquired. Three genospecies, Borrelia burgdorferi sensu stricto, Borrelia afzelli and Borrelia garinii, represent the predominant pathogenic variants in the UK. Erythema migrans is the commonest manifestation, occurring in 60%-91% of cases. In the UK, neuroborelliosis is the most common complication, while myocarditis is unusual, and death from either conduction disease or carditis is extremely rare. The role of Borrelia infection in chronic dilated cardiomyopathy in the UK remains unproven. Controversy over the existence of either 'chronic Lyme disease' and/or 'post-Lyme disease syndrome' continues unabated. National medical societies, patient advocacy groups, insurance companies, lawyers, doctors, the private health medical sector and scientific journals have all become embroiled in this bitter controversy. New developments include diagnostic tests able to detect Lyme disease at an earlier stage, shorter durations of antibiotic therapy and potential advances in vaccines against Borrelia. PMID:24198341

Dubrey, Simon W; Bhatia, Ajay; Woodham, Sarah; Rakowicz, Wojtek

2014-01-01

246

Large-scale gene-centric analysis identifies novel variants for coronary artery disease  

Microsoft Academic Search

Coronary artery disease (CAD) has a significant genetic contribution that is incompletely characterized. To complement genome-wide association (GWA) studies, we conducted a large and systematic candidate gene study of CAD susceptibility, including analysis of many uncommon and functional variants. We examined 49,094 genetic variants in approximately 2,100 genes of cardiovascular relevance, using a customised gene array in 15,596 CAD cases

A. S. Butterworth; P. S. Braund; R. J. Hardwick; D. Saleheen; J. F. Peden; N. Soranzo; J. C. Chambers; M. E. Kleber; B. Keating; A. Qasim; N. Klopp; J. Erdmann; H. Basart; J. H. Baumert; C. R. Bezzina; B. O. Boehm; J. Brocheton; P. Bugert; F. Cambien; R. Collins; D. Couper; J. S. de Jong; P. Diemert; K. Ejebe; C. C. Elbers; P. Elliott; M. Fornage; P. Frossard; S. Garner; S. E. Hunt; J. J. Kastelein; O. H. Klungel; H. Kluter; K. Koch; I. R. Konig; A. S. Kooner; K. Liu; R. McPherson; M. D. Musameh; S. Musani; G. Papanicolaou; A. Peters; B. J. Peters; S. Potter; B. M. Psaty; A. Rasheed; J. Scott; U. Seedorf; J. S. Sehmi; N. Sotoodehnia; K. Stark; J. Stephens; C. E. van der Schoot; Y. T. van der Schouw; P. van der Harst; R. S. Vasan; A. A. Wilde; C. Willenborg; B. R. Winkelmann; M. Zaidi; W. Zhang; A. Ziegler; W. Koenig; W. Matz; M. D. Trip; M. P. Reilly; S. Kathiresan; H. Schunkert; A. Hamsten; A. S. Hall; J. S. Kooner; S. G. Thompson; J. R. Thompson; H. Watkins; J. Danesh; T. Barnes; S. Rafelt; V. Codd; N. Bruinsma; L. R. Dekker; J. P. Henriques; R. J. de Winter; M. Alings; C. F. Allaart; A. P. Gorgels; F. W. A. Verheugt; M. Mueller; C. Meisinger; S. DerOhannessian; N. N. Mehta; J. Ferguson; H. Hakonarson; W. Matthai; R. Wilensky; J. C. Hopewell; S. Parish; P. Linksted; J. Notman; H. Gonzalez; A. Young; T. Ostley; A. Munday; N. Goodwin; V. Verdon; S. Shah; C. Edwards; C. Mathews; R. Gunter; J. Benham; C. Davies; M. Cobb; L. Cobb; J. Crowther; A. Richards; M. Silver; S. Tochlin; S. Mozley; S. Clark; M. Radley; K. Kourellias; P. Olsson; S. Barlera; G. Tognoni; S. Rust; G. Assmann; S. Heath; D. Zelenika; I. Gut; F. Green; M. Farrall; A. Goel; H. Ongen; M. G. Franzosi; M. Lathrop; R. Clarke; A. Aly; K. Anner; K. Bjorklund; G. Blomgren; B. Cederschiold; K. Danell-Toverud; P. Eriksson; U. Grundstedt; M. Heinonen; M. L. Hellenius; F. van't Hooft; K. Husman; J. Lagercrantz; A. Larsson; M. Larsson; M. Mossfeldt; A. Malarstig; G. Olsson; M. Sabater-Lleal; B. Sennblad; A. Silveira; R. Strawbridge; B. Soderholm; J. Ohrvik; K. S. Zaman; N. H. Mallick; M. Azhar; A. Samad; M. Ishaq; N. Shah; M. Samuel; T. L. Assimes; H. Holm; M. Preuss; A. F. Stewart; M. Barbalic; C. Gieger; D. Absher; Z. Aherrahrou; H. Allayee; D. Altshuler; S. Anand; K. Andersen; J. L. Anderson; D. Ardissino; S. G. Ball; A. J. Balmforth; L. C. Becker; D. M. Becker; K. Berger; J. C. Bis; S. M. Boekholdt; E. Boerwinkle; M. J. Brown; M. S. Burnett; I. Buysschaert; J. F. Carlquist; L. Chen; R. W. Davies; G. Dedoussis; A. Dehghan; S. Demissie; J. Devaney; A. Doering; N. E. El Mokhtari; S. G. Ellis; R. Elosua; J. C. Engert; S. Epstein; U. de Faire; M. Fischer; A. R. Folsom; J. Freyer; B. Gigante; D. Girelli; S. Gretarsdottir; V. Gudnason; J. R. Gulcher; S. Tennstedt; E. Halperin; N. Hammond; S. L. Hazen; A. Hofman; B. D. Horne; T. Illig; C. Iribarren; G. T. Jones; J. W. Jukema; M. A. Kaiser; L. M. Kaplan; K. T. Khaw; J. W. Knowles; G. Kolovou; A. Kong; R. Laaksonen; D. Lambrechts; K. Leander; M. Li; W. Lieb; G. Lettre; C. Loley; A. J. Lotery; P. M. Mannucci; N. Martinelli; P. P. McKeown; T. Meitinger; O. Melander; P. A. Merlini; V. Mooser; T. Morgan; Muhleisen T. W; J. B. Muhlestein; K. Musunuru; J. Nahrstaedt; M. M. Nothen; O. Olivieri; F. Peyvandi; R. S. Patel; C. C. Patterson; L. Qu; A. A. Quyyumi; D. J. Rader; L. S. Rallidis; C. Rice; F. R. Roosendaal; D. Rubin; V. Salomaa; M. L. Sampietro; M. S. Sandhu; E. Schadt; A. Schafer; A. Schillert; S. Schreiber; J. Schrezenmeir; S. M. Schwartz; D. S. Siscovick; M. Sivananthan; S. Sivapalaratnam; A. V. Smith; T. B. Smith; J. D. Snoep; J. A. Spertus; K. Stefansson; K. Stirrups; M. Stoll; W. H. Tang; G. Thorgeirsson; G. Thorleifsson; M. Tomaszewski; A. G. Uitterlinden; A. M. van Rij; B. F. Voight; N. J. Wareham; G. AWells; H. E. Wichmann; J. C. Witteman; B. J. Wright; S. Ye; L. A. Cupples; T. Quertermous; W. Marz; S. Blankenberg; U. Thorsteinsdottir; R. Roberts; C. J. O'Donnell; N. C. Onland-Moret; J. van Setten; P. I. de Bakker; W. M. Verschuren; J. M. Boer; C. Wijmenga; M. H. Hofker; A. H. Maitland-van der Zee; A. de Boer; D. E. Grobbee; T. Attwood; S. Belz; J. Cooper; A. Crisp-Hihn; P. Deloukas; N. Foad; A. H. Goodall; J. Gracey; E. Gray; R. Gwilliams; S. Heimerl; C. Hengstenberg; J. Jolley; U. Krishnan; H. Lloyd-Jones; I. Lugauer; P. Lundmark; S. Maouche; J. S. Moore; D. Muir; E. Murray; C. P. Nelson; J. Neudert; D. Niblett; K. O'Leary; W. H. Ouwehand; H. Pollard; A. Rankin; H. Sager; N. J. Samani; J. Sambrook; G. Schmitz; M. Scholz; L. Schroeder; A. C. Syvannen; C. Wallace

2011-01-01

247

Radiological features of superomedial iliac insufficiency fractures: a possible mimicker of metastatic disease  

Microsoft Academic Search

Objective  Pelvic insufficiency fractures are common in elderly patients. Because both osteoporosis and metastatic disease occur in similar\\u000a patient populations, insufficiency fractures may be mistaken for metastatic foci. Although the ilium is not an uncommon location\\u000a for metastases, insufficiency fractures rarely involve the ilium. The radiological features of insufficiency fractures adjacent\\u000a to the sacroiliac joint (superomedial ilium) have not been well

Andrea Donovan; Mark E. Schweitzer; Mahvash Rafii; Allison Lax

2009-01-01

248

Pigmented Bowen's disease of the penis and scrotum in a patient with AIDS.  

PubMed

Patients with HIV have higher risk of developing squamous cell carcinoma of the skin given the increased risk of HPV infection, which alters cell proliferation and apoptosis [1]. Pigmented Bowen's disease is an uncommon form of squamous cell carcinoma in-situ characterized by pigmented lesions that can clinically mimic superficial spreading melanoma, pigmented basal cell carcinoma, melanocytic nevus, Bowenoid papulosis, and seborrheic keratosis. PMID:24746300

Al-Dawsari, Najla A; Raslan, Wasim; Dawamneh, M Fawaz

2014-01-01

249

Unique Presentation of Urachal Cyst Disease: Incidental Finding to Complicated Infection  

PubMed Central

Urachal remnant disease is uncommon in adults and can present with symptoms ranging from drainage near the umbilicus to a severe abdominal infection. Most cases are referred for treatment once diagnosed either clinically or radiographically with ultrasound or computerized tomography. We present a unique case of an infected urachal cyst visualized on a series of CT scans in an adult patient with abdominal pain over a period of years. PMID:23710411

Lee, Seong K.; Kiffin, Chauniqua; Sanchez, Rafael; Carrillo, Eddy; Rosenthal, Andrew

2013-01-01

250

Geographical and temporal distribution of Ockelbo disease in Sweden.  

PubMed

The incidence of Ockelbo disease and the prevalence of Ockelbo virus neutralizing antibodies were investigated in a sample of the Swedish population. The disease occurs throughout most of Sweden but with higher incidence and antibody prevalence rates in the central part of the country. It generally affects middle-aged men and women, with equal incidence between sexes, and is uncommon in people younger than 20 years of age. The disease occurs during a short period each year between the third week of July and the first week of October, with a peak during the second half of August. During the 8 years studied (1981-8), an average of 31 Ockelbo patients/year were diagnosed. The antibody prevalence rates in the oldest age groups were 20-40 times higher than the accumulated life-risk of being diagnosed and reported as an Ockelbo disease patient, which suggests that many cases are asymptomatic and/or unreported. PMID:1646735

Lundström, J O; Vene, S; Espmark, A; Engvall, M; Niklasson, B

1991-06-01

251

Myxomatous mitral valve disease in dogs: Does size matter?  

PubMed Central

Myxomatous mitral valve disease (MMVD) is the most commonly diagnosed cardiovascular disease in the dog accounting for more than 70% of all cardiovascular disease in dogs. As are most canine diseases with genetic underpinnings, risk of MMVD is greatly increased in a subset of breeds. What is uncommon is that the vast majority of the breeds at elevated risk for MMVD are small or toy breeds with average adult weights under 9 kg. These breeds appear to have little in common other than their diminutive size. In the following review we propose a number of mechanisms by which relatively unrelated small breeds may have developed a predisposition for chronic valvular disorders. Although factors such as age are key in the expression of MMVD, taking a comprehensive look at the commonalities, as well as the differences, between the susceptible breeds may assist in finding the causal variants responsible for MMVD and translating them to improved treatments for both dogs and humans. PMID:22356836

Parker, Heidi G.; Kilroy-Glynn, Paul

2012-01-01

252

Bladder Diseases  

MedlinePLUS

... frequent, urgent urination Bladder cancer Doctors diagnose bladder diseases using different tests. These include urine tests, x- ... National Institute of Diabetes and Digestive and Kidney Diseases

253

Newcastle disease  

Technology Transfer Automated Retrieval System (TEKTRAN)

Newcastle disease (ND), referred to as Exotic Newcastle disease (END) in the U. S., is an acute viral disease of domestic poultry and many other bird species and a recognized worldwide problem. Occurrence of END is due to an infection with virulent strains of Newcastle disease virus (NDV) and is a ...

254

Heart Diseases  

MedlinePLUS

... you're like most people, you think that heart disease is a problem for others. But heart disease is the number one killer in the ... of disability. There are many different forms of heart disease. The most common cause of heart disease ...

255

Revisiting Mednick’s Model on Creativity-Related Differences in Associative Hierarchies. Evidence for a Common Path to Uncommon Thought  

PubMed Central

Fifty years ago, Mednick [Psychological Review, 69 (1962) 220] proposed an elaborate model that aimed to explain how creative ideas are generated and why creative people are more likely to have creative ideas. The model assumes that creative people have flatter associative hierarchies and as a consequence can more fluently retrieve remote associative elements, which can be combined to form creative ideas. This study aimed at revisiting Mednick’s model and providing an extensive test of its hypotheses. A continuous free association task was employed and association performance was compared between groups high and low in creativity, as defined by divergent thinking ability and self-report measures. We found that associative hierarchies do not differ between low and high creative people, but creative people showed higher associative fluency and more uncommon responses. This suggests that creativity may not be related to a special organization of associative memory, but rather to a more effective way of accessing its contents. The findings add to the evidence associating creativity with highly adaptive executive functioning. PMID:24532853

Benedek, Mathias; Neubauer, Aljoscha C

2013-01-01

256

Evaluation of Epidemiological Cut-Off Values Indicates that Biocide Resistant Subpopulations Are Uncommon in Natural Isolates of Clinically-Relevant Microorganisms  

PubMed Central

To date there are no clear criteria to determine whether a microbe is susceptible to biocides or not. As a starting point for distinguishing between wild-type and resistant organisms, we set out to determine the minimal inhibitory concentration (MIC) and minimal bactericidal concentration (MBC) distributions for four common biocides; triclosan, benzalkonium chloride, chlorhexidine and sodium hypochlorite for 3319 clinical isolates, with a particular focus on Staphylococcus aureus (N?=?1635) and Salmonella spp. (N?=?901) but also including Escherichia coli (N?=?368), Candida albicans (N?=?200), Klebsiella pneumoniae (N?=?60), Enterobacter spp. (N?=?54), Enterococcus faecium (N?=?53), and Enterococcus faecalis (N?=?56). From these data epidemiological cut-off values (ECOFFs) are proposed. As would be expected, MBCs were higher than MICs for all biocides. In most cases both values followed a normal distribution. Bimodal distributions, indicating the existence of biocide resistant subpopulations were observed for Enterobacter chlorhexidine susceptibility (both MICs and MBCs) and the susceptibility to triclosan of Enterobacter (MBC), E. coli (MBC and MIC) and S. aureus (MBC and MIC). There is a concern on the potential selection of antibiotic resistance by biocides. Our results indicate however that resistance to biocides and, hence any potential association with antibiotic resistance, is uncommon in natural populations of clinically relevant microorganisms. PMID:24466194

Morrissey, Ian; Oggioni, Marco Rinaldo; Knight, Daniel; Curiao, Tania; Coque, Teresa; Kalkanci, Ayse; Martinez, Jose Luis

2014-01-01

257

Threatening auditory hallucinations and Cotard syndrome in Parkinson disease.  

PubMed

Psychotic symptoms are commonly reported in patients with Parkinson disease (PD). In particular, patients experience nonthreatening visual hallucinations that can occur with insight (so called hallucinosis) or without. Auditory hallucinations are uncommon, and schizophrenialike symptoms such as pejorative and threatening auditory hallucinations and delusions that are persecutory, referential, somatic, religious, or grandiose have rarely been reported. The authors present 2 PD patients who experienced threatening auditory hallucinations, without visual hallucinations, and schizophrenialike delusions with detailed description of the clinical phenomenology including 1 patient with Cotard syndrome. PMID:15602098

Factor, Stewart A; Molho, Eric S

2004-01-01

258

Prion Diseases  

Microsoft Academic Search

The modern history of the prion diseases is one of novel microbes, anthropological intrigue, and food safety mishaps. The\\u000a prion diseases, also called the transmissible spongiform encephalopathies, are fatal neurodegenerative diseases that can be\\u000a sporadic, inherited, or acquired. These multiple origins are unique among human disease. The basis of all prion diseases is\\u000a the misfolding of the host prion protein

Qingzhong Kong; Richard A. Bessen

259

Epidemiology of Gallbladder Disease: Cholelithiasis and Cancer  

PubMed Central

Diseases of the gallbladder are common and costly. The best epidemiological screening method to accurately determine point prevalence of gallstone disease is ultrasonography. Many risk factors for cholesterol gallstone formation are not modifiable such as ethnic background, increasing age, female gender and family history or genetics. Conversely, the modifiable risks for cholesterol gallstones are obesity, rapid weight loss and a sedentary lifestyle. The rising epidemic of obesity and the metabolic syndrome predicts an escalation of cholesterol gallstone frequency. Risk factors for biliary sludge include pregnancy, drugs like ceftiaxone, octreotide and thiazide diuretics, and total parenteral nutrition or fasting. Diseases like cirrhosis, chronic hemolysis and ileal Crohn's disease are risk factors for black pigment stones. Gallstone disease in childhood, once considered rare, has become increasingly recognized with similar risk factors as those in adults, particularly obesity. Gallbladder cancer is uncommon in developed countries. In the U.S., it accounts for only ~ 5,000 cases per year. Elsewhere, high incidence rates occur in North and South American Indians. Other than ethnicity and female gender, additional risk factors for gallbladder cancer include cholelithiasis, advancing age, chronic inflammatory conditions affecting the gallbladder, congenital biliary abnormalities, and diagnostic confusion over gallbladder polyps. PMID:22570746

Stinton, Laura M.

2012-01-01

260

Five Uncommon but Useful Knots.  

ERIC Educational Resources Information Center

Describes five useful, little-known knots: mooring hitch for securing a line to a stump or post; highwayman's cutaway for securing canoe lines or horses' reins; taut-line hitch or midshipman's hitch for securing tent guys; and Hedden knot and C&F belay hitch, used by rock climbers and mountaineers, which combine in a simple rescue haul system. (SV)

Chisnall, Rob

1997-01-01

261

Rare species occupy uncommon niches.  

PubMed

The fact that temperate grasslands often contain upwards of 30 vascular plant species per m(2) yet these species seem to have relatively similar life histories and resource requirements has made explaining species coexistence in these communities a major focus of research. While the reduction of competition by disturbance has been a popular explanation for species coexistence, in tallgrass prairies any level of disturbance either has no effect, or decreases diversity, since it favors the dominant plants. Although there has long been speculation that grassland species could coexist by niche partitioning the concept received renewed interest when it was shown that soil hydrology could explain species coexistence. One aspect of community structure that has not been explained by niche partitioning is the rareness and commonness of species within communities. There are three classes of explanations for rareness: narrow habitat requirements, low competitive ability combined with frequency dependent fitness and, dispersal ability. However, evidence for these explanations tend to be anecdotal, focusing on particular species. Here I show that in tallgrass prairies common and rare species consistently occupy different parts of niche space, with rare species being restricted by the cover of common species and occupying the rare available niches. PMID:25110113

Markham, John

2014-01-01

262

Uncommon commentaries [on energy subjects  

SciTech Connect

Comments from several individuals are given on cogeneration expansion (Lindsay Audin); electric power system reliability in a deregulated market (Marija D. Ilic, MIT and Leonard S. Hyman, Salomon Smith Barney); demand size management (Robert J. Bradley, Jr., Cato Institute); and new questions about the competitive electric power market (Mike Sherman, Peregrine Energy Group).

NONE

1998-04-01

263

Network Externality: An Uncommon Tragedy  

Microsoft Academic Search

Economists have defined 'network externality' and have examined putative inframarginal market failures associated with it. This paper distinguishes between network effects and network externalities, where the latter are market failures. The authors argue that while network effects are important, network externalities are theoretically fragile and empirically undocumented. Some network externalities are merely pecuniary. Network ownership or transactions among network participants

S. J. Liebowitz; Stephen E Margolis

1994-01-01

264

Rare species occupy uncommon niches  

NASA Astrophysics Data System (ADS)

The fact that temperate grasslands often contain upwards of 30 vascular plant species per m2 yet these species seem to have relatively similar life histories and resource requirements has made explaining species coexistence in these communities a major focus of research. While the reduction of competition by disturbance has been a popular explanation for species coexistence, in tallgrass prairies any level of disturbance either has no effect, or decreases diversity, since it favors the dominant plants. Although there has long been speculation that grassland species could coexist by niche partitioning the concept received renewed interest when it was shown that soil hydrology could explain species coexistence. One aspect of community structure that has not been explained by niche partitioning is the rareness and commonness of species within communities. There are three classes of explanations for rareness: narrow habitat requirements, low competitive ability combined with frequency dependent fitness and, dispersal ability. However, evidence for these explanations tend to be anecdotal, focusing on particular species. Here I show that in tallgrass prairies common and rare species consistently occupy different parts of niche space, with rare species being restricted by the cover of common species and occupying the rare available niches.

Markham, John

2014-08-01

265

Uncommon Connections with Common Numerators  

ERIC Educational Resources Information Center

Undergraduate students who are pre-service teachers need to make connections between the college mathematics they are learning and the pre-college mathematics they will be teaching. Spanning a broad range of undergraduate curricula, this article describes useful lesser-known connections, explorations, and original proofs involving fractions. In…

Lesser, Lawrence M.; Guthrie, Joe A.

2012-01-01

266

The Y111 H (T415C) polymorphism in exon 3 of the gene encoding adiponectin is uncommon in Polish obese patients.  

PubMed

The genetic background of obesity is under research. Obesity-related phenotype candidate genes include the gene encoding adiponectin (AdipoQ). In this study, exon 3 of the adiponectin gene was screened for the Y111 H (Tyr111His, or T415C, rs17366743) polymorphism, and adiponectin serum concentrations were measured in 206 obese subjects (110 women and 96 men, aged 50.5+/-16.9 years). Their BMI, % of body fat, plasma glucose, insulin, and glycosylated hemoglobin were measured. Adiponectin was determined by enzyme-linked immunosorbent assay. Genomic DNA was extracted from peripheral blood leukocytes. A fragment of exon 3 of the adiponectin gene was amplified in PCR and screened for the Y111 H polymorphism in SSCP analysis. Genetic screening revealed a different SSCP pattern in 2 subjects. Subsequent genotyping disclosed the TC genotype in both subjects, resulting in Y111 H heterozygote variant frequency of 0.01 in the whole cohort. Other results for SNP (single nucleotide polymorphism) positive and negative subjects were as follows, respectively: BMI (kg/m (2)) 39.95+/-9.83 vs. 38.12+/-8.56; waist circumference (cm) 122+/-18.4 vs.115+/-16; glucose (mmol/l) 7.51+/-1.86 vs. 5.56+/-0.74; HbA1c (%) 7.55+/-1.86 vs. 6.58+/-1.36; body fat (%) 51+/-2 vs. 44+/-10; plasma insulin (mU/l) 28.92+/-16.50 vs. 37.59+/-47.34; adiponectin (ng/ml) 1301+/-15.8 vs. 5682+/-4156. Due to a proportion of 2 vs. 204, statistical calculations were not possible. The Y111 H adiponectin gene variant is uncommon in Polish obese subjects. Although we observed low adiponectin concentrations in Y111 H SNP heterozygote carriers, this finding was not confirmed by statistics. PMID:17992633

Owecki, M; Miczke, A; Kaczmarek, M; Hoppe-Go?ebiewska, J; Pupek-Musialik, D; S?omski, R; Bryll, W; Cymerys, M; Nikisch, E; Sowi?ski, J

2007-11-01

267

Isavuconazole and nine comparator antifungal susceptibility profiles for common and uncommon Candida species collected in 2012: application of new CLSI clinical breakpoints and epidemiological cutoff values.  

PubMed

The in vitro activity of isavuconazole and nine antifungal comparator agents was assessed using reference broth microdilution methods against 1,421 common and uncommon species of Candida from a 2012 global survey. Isolates were identified using CHROMagar, biochemical methods and sequencing of ITS and/or 28S regions. Candida spp. were classified as either susceptible or resistant and as wild type (WT) or non-WT using CLSI clinical breakpoints or epidemiological cutoff values, respectively, for the antifungal agents. Isolates included 1,421 organisms from 21 different species of Candida. Among Candida spp., resistance to all 10 tested antifungal agents was low (0.0-7.9 %). The vast majority of each species of Candida, with the exception of Candida glabrata, Candida krusei, and Candida guilliermondii (modal MICs of 0.5 µg/ml), were inhibited by ?0.12 µg/ml of isavuconazole (99.0 %; range 94.3 % [Candida tropicalis] to 100.0 % [Candida lusitaniae and Candida dubliniensis]). C. glabrata, C. krusei, and C. guilliermondii were largely inhibited by ?1 µg/ml of isavuconazole (89.7, 96.9 and 92.8 %, respectively). Decreased susceptibility to isavuconazole was most prominent with C. glabrata where the modal MIC for isavuconazole was 0.5 µg/ml for those strains that were SDD to fluconazole or WT to voriconazole, and was 4 µg/ml for those that were either resistant or non-WT to fluconazole or voriconazole, respectively. In conclusion, these data document the activity of isavuconazole and generally the low resistance levels to the available antifungal agents in a large, contemporary (2012), global collection of molecularly characterized species of Candida. PMID:24952015

Castanheira, Mariana; Messer, Shawn A; Rhomberg, Paul R; Dietrich, Rachel R; Jones, Ronald N; Pfaller, Michael A

2014-08-01

268

Binswanger's Disease  

MedlinePLUS

... Trials Organizations What is Binswanger's Disease? Binswanger's disease (BD), also called subcortical vascular dementia , is a type ... and brain tissue dies. A characteristic pattern of BD-damaged brain tissue can be seen with modern ...

269

Raynaud's Disease  

MedlinePLUS

Raynaud's disease is a rare disorder of the blood vessels, usually in the fingers and toes. It causes the ... secondary Raynaud's, which is caused by injuries, other diseases, or certain medicines. People in colder climates are ...

270

Gaucher's Disease  

MedlinePLUS

Gaucher's disease is a rare, inherited disorder in which you do not have enough of an enzyme called glucocerebrosidase. ... It usually starts in childhood or adolescence. Gaucher's disease has no cure. Treatment options for types 1 ...

271

Addison Disease  

MedlinePLUS

... blood pressure and water and salt balance. Addison disease happens if the adrenal glands don't make ... problem with your immune system usually causes Addison disease. The immune system mistakenly attacks your own tissues, ...

272

Fifth Disease  

MedlinePLUS

Fifth disease is a viral infection caused by parvovirus B19. The virus only infects humans; it's not the same parvovirus that dogs and cats can get. Fifth disease mostly affects children. Symptoms can include a low ...

273

Legionnaires' Disease  

MedlinePLUS

Legionnaires' disease is a type of pneumonia caused by bacteria. You usually get it by breathing in mist from ... spread from person to person. Symptoms of Legionnaires' disease include high fever, chills, a cough, and sometimes ...

274

Parasitic Diseases  

MedlinePLUS

... a bug bite, or sexual contact. Some parasitic diseases are easily treated and some are not. Parasites ... be seen with the naked eye. Some parasitic diseases occur in the United States. Contaminated water supplies ...

275

Wilson Disease  

MedlinePLUS

Wilson disease is a rare inherited disorder that prevents your body from getting rid of extra copper. You need ... copper into bile, a digestive fluid. With Wilson disease, the copper builds up in your liver, and ...

276

Eye Diseases  

MedlinePLUS

... the back of the eye Macular degeneration - a disease that destroys sharp, central vision Diabetic eye problems ... defense is to have regular checkups, because eye diseases do not always have symptoms. Early detection and ...

277

Chagas Disease  

MedlinePLUS

Chagas disease is caused by a parasite. It is common in Latin America but not in the United States. ... nose, the bite wound or a cut. The disease can also spread through contaminated food, a blood ...

278

Parkinson's Disease  

MedlinePLUS

... and Parkinson’s disease illustrates value of exposome studies (Mar. 2014) Exploring the haunting legacy – benomyl and Parkinson’s ( ... 2011) Deciphering a Core Process in Parkinson's Disease (Mar. 2011) Workshop Examines the Role of Air Pollution ...

279

Lyme Disease  

MedlinePLUS

... wondered whether the European rash, called erythema migrans (EM), and Lyme disease might have the same cause. ... that a spirochete caused both Lyme disease and EM. The spirochete was later named Borrelia burgdorferi in ...

280

Heart Disease  

MedlinePLUS

... this? Submit What's this? Submit Button Related CDC Web Sites Division for Heart Disease and Stroke Prevention ... this? Submit What's this? Submit Button Related CDC Web Sites Division for Heart Disease and Stroke Prevention ...

281

Batten Disease  

MedlinePLUS

... The NINDS supports two national human brain specimen banks. These banks supply investigators around the world with tissue from patients with neurological and psychiatric diseases. Both banks need brain tissue from Batten disease patients to ...

282

Mycobacterial Diseases  

MedlinePLUS

... Differences Between Lab and Live Results . Related Links Tuberculosis Leprosy (Hansen's Disease) National Library of Medicine, MedlinePlus ... coats that can be found throughout the world. Tuberculosis and leprosy (Hansen’s disease) are the best known ...

283

Wildlife Diseases  

E-print Network

chronic diarrhea, abdominal cramps, bloating and fatigue. Giardiasis is not usually a life threatening disease, and once diag- nosed can be effectively treated with medication. To prevent the disease, avoid drinking or acci- dentally ingesting untreated...

Texas Wildlife Services

2007-03-13

284

Hirschsprung Disease  

MedlinePLUS

... For Kids For Parents MORE ON THIS TOPIC Digestive System Irritable Bowel Syndrome (IBS) Inflammatory Bowel Disease X- ... GI) Irritable Bowel Syndrome Inflammatory Bowel Disease Your Digestive System Constipation Upper GI (Video) Digestive System Inflammatory Bowel ...

285

Celiac Disease  

MedlinePLUS

... with celiac disease, a lifelong disorder of the digestive system, these foods aren't always the treats that ... commonly consumed ingredient. What Is Celiac Disease? The digestive system is the set of organs that digest food ...

286

Celiac Disease  

MedlinePLUS

... immune disease in which people can't eat gluten because it will damage their small intestine. If you have celiac disease and eat foods with gluten, your immune system responds by damaging the small ...

287

Huntington disease  

MedlinePLUS

Huntington disease is a disorder in which nerve cells in certain parts of the brain waste away, or ... Huntington disease is caused by a genetic defect on chromosome 4. The defect causes a part of DNA, ...

288

Fifth Disease  

MedlinePLUS

... Search The CDC Cancel Submit Search The CDC Parvovirus B19 and Fifth Disease Note: Javascript is disabled ... message, please visit this page: About CDC.gov . Parvovirus Home About Parvovirus B19 Fifth Disease Pregnancy and ...

289

Fifth disease  

MedlinePLUS

Parvovirus B19; Erythema infectiosum; Slapped cheek rash ... Fifth disease is caused by human parvovirus B19. It often affects preschoolers or school-age children during the spring. The disease spreads through the fluids in the nose and ...

290

Leigh's Disease  

MedlinePLUS

... Leigh's disease can be caused by mutations in mitochondrial DNA or by deficiencies of an enzyme called pyruvate ... kidney function. In Leigh’s disease, genetic mutations in mitochondrial DNA interfere with the energy sources that run cells ...

291

Alexander Disease  

MedlinePLUS

... may be other genetic or perhaps even non-genetic causes of Alexander disease. Current research is aimed at understanding the mechanisms by which the mutations cause disease, developing better animal models for the disorder, and exploring potential strategies ...

292

Lyme Disease  

MedlinePLUS

Lyme disease is a bacterial infection you get from the bite of an infected tick. The first symptom ... Muscle and joint aches A stiff neck Fatigue Lyme disease can be hard to diagnose because you may ...

293

Behcet's Disease  

MedlinePLUS

... other diseases of the digestive tract, such as ulcerative colitis and Crohn’s disease, careful evaluation is essential to ... is put directly on the affected body part. Ulcerative colitis. Inflammation of the colon. Symptoms include stomach pain ...

294

Autoimmune Diseases  

MedlinePLUS

... of the digestive tract. Crohn's (krohnz) disease and ulcerative colitis (UHL-sur-uh-tiv koh-LEYE-tuhss) are ... Crohn's disease) Painful or difficult bowel movements (in ulcerative colitis) Inflammatory myopathies (meye-OP-uh-theez) A group ...

295

Neurodegenerative Diseases  

Microsoft Academic Search

The neurodegenerative disorders, which include Amyotrophic Lateral Sclerosis (ALS), Alzheimer Disease (AD), Parkinson Disease\\u000a (PD), and Huntington Disease (HD), are clinically heterogeneous. Medications can ameliorate some symptoms, but none reverse\\u000a the relentless progression of the illnesses. The past several decades have seen a dramatic increase in the understanding of\\u000a the complex pathophysiology underlying the diseases. Overlapping features at the cellular

Jinsy A. Andrews; Paul H. Gordon

296

Prion diseases  

Microsoft Academic Search

Human prion diseases are a rare and diverse group of neurodegenerative diseases that have long provoked interest from physicians and scientists. Initially, this related to the enigma of a group of diseases with inherited, sporadic and acquired forms, and then subsequently to the proposition that the infectious agent comprised an abnormally folded but widely expressed cell-surface protein. More recently, the

Diego Kaski; Simon Mead

2009-01-01

297

Prion diseases  

Microsoft Academic Search

Human prion diseases are a rare and diverse group of neurodegenerative diseases that have long provoked interest from physicians and scientists. Initially, this related to the enigma of a disease with inherited, sporadic and acquired forms, and the subsequent demonstration that the infectious agent comprised an abnormally folded but widely expressed cell-surface protein. More recently, the epidemic of bovine spongiform

Simon Mead

2005-01-01

298

Celiac Disease  

MedlinePLUS

... absorbing enough iron. Continue Why Do Kids Get Celiac Disease? No one is sure why celiac disease happens, but it appears to run in families. ... have a 5% to 10% chance of getting celiac disease if someone in your family has it. It's ...

299

Kawasaki disease  

PubMed Central

Short history of Kawasaki disease, clinical features (principal symptoms and other significant symptoms or findings), diagnosis, cardiovascular involvement, epidemiology. Pathological features (lesion of vessels and lesion of organs exclusive of vessels), comparison between infantile periarteritis nodosa (IPN)/Kawasaki disease and classic periarteritis nodosa (CPN), etiology, treatment and management of Kawasaki disease are described.

Kawasaki, Tomisaku

2006-01-01

300

Sandhoff Disease  

MedlinePLUS

... body. Sandhoff disease is a severe form of Tay-Sachs disease--which is prevalent primarily in people of Eastern ... Worldwide NINDS Clinical Trials Organizations Column1 Column2 National Tay-Sachs and Allied Diseases Association 2001 Beacon Street Suite 204 Boston, MA ...

301

Crinkle Disease  

Technology Transfer Automated Retrieval System (TEKTRAN)

Crinkle disease of hop was first described in Europe in 1930, and subsequent reports of the disease appear in literature published in the 1960s and 1970s. The disease appears to be of little importance in most regions of hop production. A fastidious rickettsia-like organism (RLO) is thought to cau...

302

NEWCASTLE DISEASE  

Technology Transfer Automated Retrieval System (TEKTRAN)

Concise information about Newcastle disease (ND) is provided for a book that serves as a quick reference guide to the infectious, parasitic, metabolic, nutritional, and toxic diseases of domesticated animals and birds as well some exotic species that a veterinarian might encounter. Newcastle disease...

303

Meniere's Disease.  

ERIC Educational Resources Information Center

Meniere's disease is characterized by unpredictable spells of severe vertigo and fluctuations in hearing and tinnitus. This article discusses the incidence of Meniere's disease, the present status of our understanding of this disease, controversies in its diagnosis, and the multiple therapeutic modalities recruited in its treatment. (Contains…

Schessel, David A.

1997-01-01

304

Lyme Disease  

MedlinePLUS

... Most cases of Lyme disease occur in the spring and summer months. Lyme disease, in most cases, can be eliminated with antibiotics, especially if treatment is started when symptoms are first noted. Lyme disease is divided into 3 phases: Early localized : Symptoms start a few days to a ...

305

Prostate Diseases  

MedlinePLUS

Aging & Health A to Z Prostate Diseases Basic Facts & Information What are Prostate Diseases? The prostate—one of the components of a man's sex organs—is a ... out anything serious. The Most Common Types of Prostate Diseases Benign prostatic hyperplasia (BPH) Prostatitis Prostate cancer ...

306

Lyme Disease.  

ERIC Educational Resources Information Center

This overview of the public health significance of Lyme disease includes the microbiological specifics of the infectious spirochete, the entomology and ecology of the ticks which are the primary disease carrier, the clinical aspects and treatment stages, the known epidemiological patterns, and strategies for disease control and for expanded public…

Taylor, George C.

1991-01-01

307

Crohn's Disease  

MedlinePLUS

... Crohn’s disease are very similar to those of ulcerative colitis , except that Crohn’s disease can happen anywhere along ... tract, from the mouth to the anus , while ulcerative colitis is restricted to the colon . Crohn’s disease may ...

308

HIV-associated Multicentric Castleman Disease  

PubMed Central

Purpose of review HIV-associated multicentric Castleman disease (HIV MCD) is a rare lymphoproliferative disorder, the incidence of which appears to be increasing in the highly active antiretroviral therapy (HAART) era. Current knowledge of the disease is limited and this review will discuss what is known about the pathophysiology, diagnosis, management, and prognosis of HIV MCD. Recent findings HIV MCD has been shown to be associated with infection with human herpervirus-8 (HHV8). Vascular endothelial growth factor and the cytokine interleukin-6 (IL-6) are also thought to play a role in the pathogenesis of MCD. Currently, rituximab is often used alone or in combination with chemotherapy for treatment of MCD. Novel monoclonal antibodies targeting IL-6 and the IL-6 receptor are also being studied for the management of this disease. Summary Because HIV MCD is an uncommon diagnosis, comprehensive clinical studies have not been done, and understanding of the disease is incomplete. Further studies are needed to make definitive conclusions regarding optimal treatment of HIV MCD. PMID:21760504

Reddy, Deepa; Mitsuyasu, Ronald

2015-01-01

309

The challenge of treating orphan disease.  

PubMed

Uncommon or orphan diseases are less frequently addressed in mainstream medical journals and, as a consequence, their understanding and clinical recognition may rely on case series or anecdotal data with limited guidelines and management directions. The study of selected underrepresented autoimmune and allergy conditions is the subject of the present issue of Clinical Reviews in Allergy and Immunology to provide peculiar perspectives on common and rare themes. First, allergy remains a major concern for physicians worldwide despite the limited developments over the past years, particularly for antigens such as mite or Alternaria alternata, and due to the increasing incidence of drug hypersensitivity. Second, the female predominance of autoimmune diseases such as systemic sclerosis is well recognized but enigmatic, and a unifying hypothesis remains elusive. Third, the management of conditions triggered by infectious agents as in Guillain-Barre syndrome or mixed cryoglobulinemia is challenging, and clinical guidelines are needed in the setting of infections and autoimmunity. Fourth, gamma-delta T cells represent major players in innate immunity and are the subject of extensive studies in autoimmune diseases to provide new therapeutic targets for disease prevention or modulation in the near future. Ultimately, we acknowledge the major developments in the broad fields of rheumatology and immunology and expect that microbiota definition, epigenetics studies, and microRNA analysis will provide new exciting avenues toward the understanding and treatment of chronic and acute inflammation. PMID:25395247

Dias, Carlos; Selmi, Carlo

2014-12-01

310

Kawasaki disease.  

PubMed

Kawasaki disease (KD) is the archetypal pediatric vasculitis, exemplifying the unique aspects and challenges of vascular inflammation in children. The condition is almost unheard of in adults, is closely associated with infections, and is self-limited, with fever resolving after an average of 12 days even without treatment. Yet KD is also a potentially fatal disease and the most common cause of acquired heart disease in the developed world. Unraveling of the developmental, immunologic, and genetic secrets of Kawasaki disease promises to improve our understanding of vasculitis in particular, and perhaps also to provide a window on the fundamental mysteries of inflammatory diseases in general. PMID:25399940

Sundel, Robert P

2015-01-01

311

Coeliac disease among children in Kuwait: difficulties in diagnosis and management.  

PubMed

Twenty children with coeliac disease were diagnosed over a five year period in an area with 10,000-12,000 births per year. The average annual incidence was 1:3000 births. All children presented with severe symptoms and rickets was not uncommon (25%). Mean age at onset of symptoms was 38 months (range 6-120) and 72 months at the time of diagnosis (range 13-192), with a mean delay of 34 months. No cases were diagnosed during infancy. The difficulties in the differentiation of coeliac disease from the more common causes of chronic diarrhoea, and problems with diagnosis and management are discussed. PMID:3428686

Khuffash, F A; Barakat, M H; Shaltout, A A; Farwana, S S; Adnani, M S; Tungekar, M F

1987-12-01

312

FDG PET images in a patient with Erdheim-Chester Disease  

PubMed Central

Erdheim-Chester disease (ECD) is an uncommon non-Langerhans-cell histiocytosis, due to excessive production of histiocytes deposited in various organs and tissues in the human body. FDG PET was performed in a 68-year-old man with documented active ECD to evaluate the extent of the disease. The patient was previously treated with high dose subcutaneous Interferon alpha 2b, 1,000,000 units 3 times a week, but treatment was interrupted approximately 5 weeks prior to evaluation at NIH due to side effects of the medication. FDG PET/CT showed lesions were imaged in brain, heart, mediastinum and abdomen. PMID:23640213

Sioka, Chrissa; Estrada-Veras, Juvianee; Maric, Irinia; Gahl, William A.; Chen, Clara C.

2013-01-01

313

[Positron emission tomography in a patient with Von Recklinghausen disease and left dorsal neurofibrosarcoma].  

PubMed

The development of neurofibrosarcomas in patients with Von Recklinghausen disease is a rare complication that can appear in the daily clinical practice. We report a clinical case with Von Recklinghausen disease and recurrence of a left dorsal neurofibrosarcoma diagnosed by Positron Emission Tomography with fluorine-18-fluoro-deoxyD-glucose (FDG-PET). We believe this work is important due to the relatively uncommon clinical presentation and the discussion on the utility of 18-FDG PET and other conventional imaging methods in the diagnosis of this entity. PMID:14588235

Ruíz-Hernández, G; Hornedo-Muguiro, J; Salinas-Hernández, P; Pérez-Castejón, M J; Lapeńa-Gutiérrez, L; Montz-Andrée, R; Carreras-Delgado, J L

2003-11-01

314

Serous microcystic adenoma of the pancreas associated with malrotation and Hirschsprung's disease in an infant.  

PubMed

Serous microcystic adenoma of the pancreas is an uncommon tumor in adults, and reported in pediatric patients only a handful of times. The authors present a case of a 6 month patient with incidental finding of a large cystic mass on the tail of the pancreas during an operation for Hirschsprung's disease. Final pathology was consistent with serous microcystic adenoma. This is the second case report describing this lesion in an infant, and the first time it has been associated with Hirschsprung's disease. PMID:25623596

Larson, Kelsey; Seifarth, Federico G; Plesec, Thomas; Magnuson, David K

2015-04-01

315

[Wilson's disease].  

PubMed

Wilson's disease is an autosomal recessive disorder of copper metabolism. The Wilson disease protein is a copper-transporting P-type ATPase, ATP7B, the malfunction of which results in the toxic accumulation of copper in the liver and brain, causing the hepatic and/or neurological symptoms accompanying this disease. Patients present, generally between the ages of 10 and 40 years, with liver disease, neurological disease of a movement disorder type, or behavioral abnormalities, and often with a combination of these. Because Wilson's disease is effectively treated, it is extremely important for physicians to learn to recognize and diagnose the disease. The laboratory diagnosis of Wilson's disease is confirmed by decreased serum ceruloplasmin, increased urinary copper content, and elevated hepatic copper concentration. Molecular genetic analysis is complex as more than 200 unique mutations have been identified and most individuals are compound heterozygotes. The treatment of Wilson's disease must be life long. Copper chelation with penicillamine is an effective therapy in most patients. Another chelating agent which has been used successfully as the initial therapy is trientine. The search for new anticopper drugs for Wilson's disease is culminating in two excellent new drugs: zinc for maintenance therapy and ammonium tetrathiomolybdate (which is to date still an experimental drug) for initial therapy. Liver transplantation is indicated for the fulminant form and in those patients with severe disease not responding to optimal medical management. This paper reviews the pathogenesis, pathology, clinical presentation and diagnosis of the Wilson's disease as well as the most recent views on the molecular genetics and the treatment of this disease. PMID:14582469

Kovacevi?, Igor; Zekan, Mirta

2003-01-01

316

Lyme Disease  

PubMed Central

Synopsis Lyme disease, caused by spirochete Borrelia burgdorferi, is the most common vector-borne disease in the United States. The clinical presentation varies depending on the stage of the illness: early disease includes erthyma migrans, early disseminated disease includes multiple erythema migrans, meningitis, cranial nerve palsies and carditis; late disease is primarily arthritis. The symptoms and signs of infection resolve in the vast majority of patients after appropriate treatment with antimicrobials for from 2-4 weeks. Serologic testing should be used judiciously as it often results in misdiagnosis when performed on blood from patients with a low prior probability of disease and those with non-specific symptoms such as fatigue or arthralgia without signs of infection. PMID:20513553

Murray, Thomas S.; Shapiro, Eugene D.

2013-01-01

317

Prion Diseases  

NSDL National Science Digital Library

Prion Diseases is one of a set of lecture notes for Virology 335 by Shaun Heaphy of Leicester University (UK). It contains detailed information on its topic, along with selected links. Although prion research has been going on for over 25 years, the scientific and medical communities have only recently acknowledged the existence of prions and there remains serious debate over their role in a variety of neurological diseases. The name "prion" is derived from "proteinaceous infectious particles," and was coined by Dr. Stanley Prusiner, who discovered the agents and who recently received the Nobel Prize for Medicine for his work. Prions are thought to be the first transmissible and heritable disease-causing agents that lack DNA and RNA. They are composed solely of protein and appear to be the cause of such diseases as kuru and Creutzfeldt-Jakob disease in humans, and bovine spongiform encephalopathies, mad cow disease, and scrapie in sheep and goats.

Heaphy, Shaun.

1997-01-01

318

Infectious disease  

NASA Technical Reports Server (NTRS)

This is a collection of viewgraphs on the Johnson Space Center's work on infectious disease. It addresses their major concern over outbreaks of infectious disease that could jeopardize the health, safety and/or performance of crew members engaged in long duration space missions. The Antarctic environment is seen as an analogous location on Earth and a good place to carry out such infectious disease studies and methods for proposed studies as suggested.

Pierson, Duane L.

1990-01-01

319

Gaucher disease.  

PubMed

Gaucher disease is the commonest lysosomal storage disease seen in India and worldwide. It should be considered in any child or adult with an unexplained splenohepatomegaly and cytopenia which are seen in the three types of Gaucher disease. Type 1 is the non-neuronopathic form and type 2 and 3 are the neuronopathic forms. Type 2 is a more severe neuronopathic form leading to mortality by 2 years of age. Definitive diagnosis is made by a blood test-the glucocerebrosidase assay. There is no role for histological examination of the bone marrow, liver or spleen for diagnosis of the disease. Molecular studies for mutations are useful for confirming diagnosis, screening family members and prognosticating the disease. A splenectomy should not be performed except for palliation or when there is no response to enzyme replacement treatment or no possibility of getting any definitive treatment. Splenectomy may worsen skeletal and lung manifestations in Gaucher disease. Enzyme replacement therapy (ERT) has completely revolutionized the prognosis and is now the standard of care for patients with this disease. Best results are seen in type 1 disease with good resolution of splenohepatomegaly, cytopenia and bone symptoms. Neurological symptoms in type 3 disease need supportive care. ERT is of no benefit in type 2 disease. Monitoring of patients on ERT involves evaluation of growth, blood counts, liver and spleen size and biomarkers such as chitotriosidase which reflect the disease burden. Therapy with ERT is very expensive and though patients in India have so far got the drug through a charitable access programme, there is a need for the government to facilitate access to treatment for this potentially curable disease. Bone marrow transplantation is an inferior option but may be considered when access to expensive ERT is not possible. PMID:25755533

Nagral, Aabha

2014-03-01

320

Genetic Diseases  

Microsoft Academic Search

\\u000a Many dermatologic diseases have a genetic component, and so this group of diseases may be expanded as more evidence is unearthed.\\u000a Atopic dermatitis, for instance, is currently included in the dermatitis\\/eczema group. Filaggrin gene defects have been found\\u000a in approximately 50% of the patients with atopic dermatitis, but the relationship of this defect to the pathogenesis of this\\u000a disease has

Herbert B. Allen

321

Gaucher Disease  

PubMed Central

Gaucher disease is the commonest lysosomal storage disease seen in India and worldwide. It should be considered in any child or adult with an unexplained splenohepatomegaly and cytopenia which are seen in the three types of Gaucher disease. Type 1 is the non-neuronopathic form and type 2 and 3 are the neuronopathic forms. Type 2 is a more severe neuronopathic form leading to mortality by 2 years of age. Definitive diagnosis is made by a blood test–the glucocerebrosidase assay. There is no role for histological examination of the bone marrow, liver or spleen for diagnosis of the disease. Molecular studies for mutations are useful for confirming diagnosis, screening family members and prognosticating the disease. A splenectomy should not be performed except for palliation or when there is no response to enzyme replacement treatment or no possibility of getting any definitive treatment. Splenectomy may worsen skeletal and lung manifestations in Gaucher disease. Enzyme replacement therapy (ERT) has completely revolutionized the prognosis and is now the standard of care for patients with this disease. Best results are seen in type 1 disease with good resolution of splenohepatomegaly, cytopenia and bone symptoms. Neurological symptoms in type 3 disease need supportive care. ERT is of no benefit in type 2 disease. Monitoring of patients on ERT involves evaluation of growth, blood counts, liver and spleen size and biomarkers such as chitotriosidase which reflect the disease burden. Therapy with ERT is very expensive and though patients in India have so far got the drug through a charitable access programme, there is a need for the government to facilitate access to treatment for this potentially curable disease. Bone marrow transplantation is an inferior option but may be considered when access to expensive ERT is not possible. PMID:25755533

Nagral, Aabha

2014-01-01

322

Comprehensive carrier screening and molecular diagnostic testing for recessive childhood diseases.  

PubMed

Of 7,028 disorders with suspected Mendelian inheritance, 1,139 are recessive and have an established molecular basis. Although individually uncommon, Mendelian diseases collectively account for ~20% of infant mortality and ~18% of pediatric hospitalizations. Molecular diagnostic testing is currently available for only ~300 recessive disorders. Preconception screening, together with genetic counseling of carriers, has resulted in remarkable declines in the incidence of several severe recessive diseases including Tay-Sachs disease and cystic fibrosis. However, extension of preconception screening and molecular diagnostic testing to most recessive disease genes has hitherto been impractical. Recently, we reported a preconception carrier screen / molecular diagnostic test for 448 recessive childhood diseases. The current status of this test is reviewed here. Currently, this reports analytical validity of the comprehensive carrier test. As the clinical validity and clinical utility in the contexts described is ascertained, this article will be updated. PMID:22872815

Kingsmore, Stephen

2012-01-01

323

Comprehensive Carrier Screening and Molecular Diagnostic Testing for Recessive Childhood Diseases  

PubMed Central

Of 7,028 disorders with suspected Mendelian inheritance, 1,139 are recessive and have an established molecular basis. Although individually uncommon, Mendelian diseases collectively account for ~20% of infant mortality and ~18% of pediatric hospitalizations. Molecular diagnostic testing is currently available for only ~300 recessive disorders. Preconception screening, together with genetic counseling of carriers, has resulted in remarkable declines in the incidence of several severe recessive diseases including Tay-Sachs disease and cystic fibrosis. However, extension of preconception screening and molecular diagnostic testing to most recessive disease genes has hitherto been impractical. Recently, we reported a preconception carrier screen / molecular diagnostic test for 448 recessive childhood diseases. The current status of this test is reviewed here. Currently, this reports analytical validity of the comprehensive carrier test. As the clinical validity and clinical utility in the contexts described is ascertained, this article will be updated. PMID:22872815

Kingsmore, Stephen

2012-01-01

324

Hodgkin's disease  

SciTech Connect

The outlook for patients with Hodgkin's disease has improved dramatically over the past 20 years. The question is no longer whether cure is possible, but rather, how can cure be best achieved. With better understanding of the biology of Hodgkin's disease and with continued evolution of treatment approaches, the goal of curing all patients with Hodgkin's disease is clearly within reach. This article provides a summary of current concepts in the biology and management of Hodgkin's disease. Staging, treatment options, and complications of therapy are discussed.

Portlock, C.S.

1984-05-01

325

Disease Detective  

NSDL National Science Digital Library

This activity (on pages 35-43) lets learners analyze a "herd of elk" to detect the spread of a bacterial disease called brucellosis. The activity simulates how wildilfe veterinarians study elk in the wild by sampling only a subset of the animals. Based on a brucellosis problem with elk in Yellowstone National Park, learners cut out representations for two herds and then pick some at random to "test" for disease (denoted as a plus sign on a diseased animal). The results indicate that elk fed in Wyoming over the winter have more disease than the wild elk that go north to Montana

University of Nebraska State Museum

2002-01-01

326

Inflammatory Bowel Disease in Children of Middle Eastern Descent  

PubMed Central

Increasing rates of inflammatory bowel disease (IBD) are now seen in populations where it was once uncommon. The pattern of IBD in children of Middle Eastern descent in Australia has never been reported. This study aimed to investigate the burden of IBD in children of Middle Eastern descent at the Sydney Children's Hospital, Randwick (SCHR). The SCHR IBD database was used to identify patients of self-reported Middle Eastern ethnicity diagnosed between 1987 and 2011. Demographic, diagnosis, and management data was collected for all Middle Eastern children and an age and gender matched non-Middle Eastern IBD control group. Twenty-four patients of Middle Eastern descent were identified. Middle Eastern Crohn's disease patients had higher disease activity at diagnosis, higher use of thiopurines, and less restricted colonic disease than controls. Although there were limitations with this dataset, we estimated a higher prevalence of IBD in Middle Eastern children and they had a different disease phenotype and behavior compared to the control group, with less disease restricted to the colon and likely a more active disease course. PMID:24987422

Naidoo, Christina Mai Ying; Leach, Steven T.; Day, Andrew S.; Lemberg, Daniel A.

2014-01-01

327

Secondary causes of nonalcoholic fatty liver disease  

PubMed Central

Nonalcoholic fatty liver disease (NAFLD) is becoming the most common cause of chronic liver disease in the developing world, found in 17-30% of the population in Western countries and 2-4% worldwide. Defined as the accumulation of fatty acid content greater than 5% of liver weight, NAFLD is a spectrum of disease ranging from simple steatosis to nonalcoholic steatohepatitis. The pathophysiology of NAFLD involves increased de novo synthesis of fatty acids in hepatocytes, the retention of lipids due to impaired hepatocyte apolipoprotein secretion or beta-oxidation. The well-known primary causes of NAFLD are obesity, type II diabetes, dyslipidemia, and insulin resistance. However, other less common conditions can cause a similar clinical and histologic picture, and should be considered in patients who present with NAFLD but do not have traditional risk factors. In this review, we discuss uncommon but important causes of NAFLD, including inborn errors of metabolism, iatrogenic causes, viral hepatitis, and nutritional disorders to provide practicing clinicians with an understanding of the less well recognized causes of NAFLD. PMID:22570680

Kneeman, Jacob M.; Misdraji, Joseph

2012-01-01

328

Thoracic irradiation in Hodgkin's disease: Disease control and long-term complications  

SciTech Connect

A total of 590 patients with Stage IA-IIIB Hodgkin's disease received mantle irradiation at the Joint Center for Radiation Therapy between April 1969 and December 1984 as part of their initial treatment. Recurrence patterns as well as pulmonary, cardiac and thyroid complications were analyzed. Pulmonary recurrence was more frequently seen in patients with large mediastinal adenopathy (LMA); 11% of patients with LMA recurred in the lung in contrast to 3.1% with small or no mediastinal disease, p = 0.003. Hilar involvement, when corrected for size of mediastinal involvement, was not predictive of lung relapse. Patients with LMA also had a high rate of nodal relapse above the diaphragm (40%) following radiation therapy (RT) alone as compared to similarly treated patients with small or no mediastinal adenopathy (6.5%), p less than 0.0001. This risk of nodal recurrence was greatly reduced (4.7%) for LMA patients receiving combined radiation therapy and chemotherapy (CMT), p less than 0.0001. Sixty-seven patients (11%) with hilar or large mediastinal involvement received prophylactic, low dose, whole lung irradiation. No decrease in the frequency of lung recurrence was seen with the use of whole lung irradiation. Radiation pneumonitis was seen in 3% of patients receiving radiation therapy alone. In contrast, the use of whole lung irradiation was associated with a 15% risk of pneumonitis, p = 0.006. The risk of pneumonitis was also significantly increased with the use of chemotherapy (11%), p = 0.0001. Cardiac complications were uncommon with pericarditis being the most common complication (2.2%). Thyroid dysfunction was seen in 25% of patients and appeared to be age-related. These data suggest that the long-term complications of mantle irradiation are uncommon with the use of modern radiotherapeutic techniques.

Tarbell, N.J.; Thompson, L.; Mauch, P. (Harvard Medical School, Boston, MA (USA))

1990-02-01

329

Alzheimer's Disease  

Microsoft Academic Search

The proliferation of information regarding Alzheimer's disease in current years has had a tremendous impact on the literature being generated on this topic. Until fairly recently, Alzheimer's disease was believed to be incurable, and its progress inexorable. In recent years research has come up with encouraging results which give hope that new treatments and even a cure is possible in

Carol Gillen

1993-01-01

330

Cardiovascular Disease  

Technology Transfer Automated Retrieval System (TEKTRAN)

Cardiovascular disease (CVD), particularly CHD (coronary heart disease) and stroke, remain the leading causes of death of women in America and most developed countries. In recent years the rate of CVD has declined in men but not in women. This is contributed to by an under-recognition of women’s C...

331

Addison's Disease  

MedlinePLUS

... is Addison’s disease? Addison’s disease affects your body’s adrenal glands. The adrenal glands are part of the endocrine system. The endocrine ... your moods, growth, metabolism, and tissue function. The adrenal glands are located just above your kidneys. They produce ...

332

Mitochondrial Diseases  

PubMed Central

Mitochondria contain the respiratory chain enzyme complexes that carry out oxidative phosphorylation and produce the main part of cellular energy in the form of ATP. Although several proteins related with signalling, assembling, transporting, and enzymatic function can be impaired in mitochondrial diseases, most frequently the activity of the respiratory chain protein complexes is primarily or secondarily affected, leading to impaired oxygen utilization and reduced energy production. Mitochondrial diseases usually show a chronic, slowly progressive course and present with multiorgan involvement with varying onset between birth and late adulthood. Neuromuscular system is frequently affected in mitochondrial diseases. Although there is actually no specific therapy and cure for mitochondrial diseases, the understanding of the pathophysiology may further facilitate the diagnostic approach and open perspectives to future in mitochondrial diseases. PMID:24649452

Lee, Young-Mock

2012-01-01

333

[Graves' disease].  

PubMed

Genetic and environmental determinants inducing Graves' disease are still poorly defined, especially those leading to the appearance of TSH receptor antibodies, which are both the hallmark of the disease and the keystone of its diagnosis. The treatment of Graves' disease is based on the use of antithyroid drugs and no therapeutic protocol has proven superior to the other. Surgical or radiometabolic treatments, largely restricted to relapses and to patients with comorbidities could be considered earlier provided we had reliable predictive markers for relapse. The treatment of Graves' orbitopathy relies first on a rigorous analysis of severity and activity of the disease. Intravenous steroids appear as a reference treatment in active forms. Evaluation of new therapeutics is in process. Graves' disease during pregnancy requires a multidisciplinary approach and an expert ultrasound evaluation of the fetus. Because of a suspected teratogenicity of carbimazole and methimazole, propylthiouracyl is the preferred drug used during early pregnancy. PMID:25090772

Illouz, Frédéric; Rodien, Patrice

2014-06-01

334

Beryllium disease.  

PubMed Central

The increasing use of beryllium in a variety of industries continues to be a hazard. New cases are still being reported to the UK Beryllium Case Registry, now numbering 60 in the period 1945-1988. The majority of cases follow inhalation which results in acute beryllium disease (chemical pneumonitis) or more commonly chronic beryllium disease--a granulomatous pneumonitis. Granulomatous skin nodules also occur following local implantation. The clinical and radiological features are briefly described with the emphasis on pathology and immunology. Laser microprobe mass spectrometry analysis of tissue sections is a major advance in diagnosis. Detection of beryllium distinguishes the granulomas of chronic beryllium disease from other diseases, in particular sarcoidosis. The role of beryllium lymphocyte transformation tests is discussed. Chronic beryllium disease is steroid dependent and local excision of skin lesions appears to be curative. There is no evidence that beryllium is carcinogenic. Images Figure 1 PMID:3074283

Jones Williams, W.

1988-01-01

335

Choledochal cyst- Presentation of the disease with a case report  

PubMed Central

Choledochal cyst is a rare disease of the biliary tract. There are five main types of choledochal cysts with a few recognized sub-types. The etiology of choledochal cysts still is unclear. The incidence of biliary tract cancer in patients with choledochal cysts increases with age. In the past, choledochal cysts were often treated using drainage procedures; however, the optimal treatment used today is likely to involve the complete excision of the extrahepatic duct, cholecystectomy, and Roux-en-Y hepaticojejunostomy. Endoscopic treatment of type III choledochocele should be limited to the management of smaller lesions. We report a case of 75 years old patient with distal choledochal diverticuly, Todani’s type III- choledochocele. Delay in the diagnosis increases the frequency of associated biliary pathology, malignant alternation and suboptimal surgical therapy. Often, intraoperative finding of choledochal cyst is the first contact with this rear entity, so awareness of possible presence of this uncommon disease is very important for surgeon. PMID:21875424

Cvetkovic, Aleksandar; Markovic, Rade; Milosevic, Bojan

2011-01-01

336

Paradoxical brain embolism associated with kimura disease mimics watershed infarction.  

PubMed

Kimura disease (KD) is an uncommon chronic inflammatory disease presenting as subcutaneous lymphadenopathy with eosinophilia. To date, only a single case of brain embolism caused by fibroblastic endocarditis associated with KD has been reported. Watershed infarction was seen in patients with episodes of severe hypotension or cardiac surgery. We here report a young case of KD who developed ischemic stroke and showed multiple small infarcts in the border zones between the territories of major cerebral arteries, mimicking watershed infarction. Transesophageal echocardiography revealed patent foramen ovale and atrial septal aneurysm. Concurrently, deep venous thrombus in the femoral vein was found on duplex ultrasonography. Our case supports the notion that paradoxical brain embolism associated with KD can cause multiple small embolisms and mimic watershed infarction. PMID:25447210

Tanaka, Yasutaka; Ueno, Yuji; Shimada, Yoshiaki; Yamashiro, Kazuo; Tanaka, Ryota; Urabe, Takao; Hattori, Nobutaka

2015-02-01

337

Fanconi anemia associated with moyamoya disease in Saudi Arabia  

PubMed Central

We report a 10-year-old Saudi girl who has Fanconi anemia (FA) and was admitted due to acute hemiplegia, of the right side. She had a previous attack of left side hemiplegia that resolved spontaneously. The brain magnetic resonance angiography showed a cerebrovascular pattern of moyamoya disease. She underwent partially matched related donor stem cell transplantation (SCT), but unfortunately died 3 months later with post SCT complications. The association of moyamoya disease with FA is uncommon, and is rarely reported in the literature. Although this condition may be acquired, it is considered a truly congenital defect in FA, and to identify the etiology of this association furthermore genetic mutation analysis is needed. PMID:25719591

Al-Hawsawi, Zakaria M.; Al-Zaid, Mohamed A.; Barnawi, Ashwaq I.; Yassine, Saadeddine M.

2015-01-01

338

Chronobiology in aortic diseases - "is this really a random phenomenon?".  

PubMed

Although acute aortic rupture or dissection is relatively uncommon, it ranks in third position among necropsy-confirmed causes of out-of-hospital sudden death in the general population. Similar to other acute cardiovascular events (e.g., acute myocardial infarction, sudden death, stroke, and pulmonary embolism) there is a growing body of evidence regarding temporal patterns in onset, characterized by circadian, seasonal and weekly variations for aortic aneurysms. On one hand, it is possible that these cardiovascular diseases share common underlying pathophysiologic mechanisms, e.g., increase in blood pressure, heart rate, sympathetic activity, basal vascular tone, vasoconstrictive hormones, and prothrombotic tendency. On the other hand, the possibility exists that the connecting link is an internal disruption (dyssynchrony) of some molecular mechanisms intrinsic to the peripheral biological clock (that of cardiomyocyte is the most widely investigated). Such disruption may contribute to cardiovascular disease and biological rhythms - an intriguing hypothesis for future research. PMID:23993245

Manfredini, Roberto; Fabbian, Fabio; Manfredini, Fabio; Salmi, Raffaella; Gallerani, Massimo; Bossone, Eduardo

2013-01-01

339

[Peyronie's disease].  

PubMed

Peyronie's disease is caused by collagen deposits in the tunica albuginea of the corpus cavernosum following microtrauma. Symptoms may include a combination of penile curvature, a palpable plaque, painful erections and erectile dysfunction. Peyronie's disease can have a major impact on the quality of life. In the course of the disease two phases can be discerned. In the first, active phase there is penile curvature with painful erections. The second, stable phase is characterised by painless curvature of the penis. Treatment in the active phase is conservative and supportive. Surgical treatment is useful only in the stable phase and may consist of penile plication surgery or penile graft surgery. PMID:25004781

Ruiter, Annebeth E C; Meuleman, Eric J H

2014-01-01

340

Gaucher's disease  

PubMed Central

Gaucher's disease (GD) is the most common amongst the various disorders classified under the lysosomal storage disorders. GD is a model for applications of molecular medicine to clinical delineation, diagnosis, and treatment. The multiorgan and varied presentation of the disease makes it a challenge to diagnose GD early. The advent of enzyme replacement therapy in the early 1990s changed the management, and survival, of patients with GD. In addition to this, development of substrate reduction, pharmacological chaperone, and gene therapies has broadened the horizon for this rare disease. However, in resource-poor countries like ours, optimal management is still a distant dream. PMID:21897894

Bohra, Vijay; Nair, Velu

2011-01-01

341

Leishmaniasis Disease  

MedlinePLUS

... message, please visit this page: About CDC.gov . Parasites - Leishmaniasis Parasites Home Share Compartir Disease Ulcerative skin lesion, with ... with some of the species (types) of the parasite that cause cutaneous leishmaniasis in parts of Latin ...

342

Celiac Disease  

Microsoft Academic Search

Celiac disease is induced by the ingestion of gluten, which is derived from wheat, barley, and rye. The gluten protein is enriched in glutamine and proline and is poor- ly digested in the human upper gastrointestinal tract. The term \\

Peter H. R. Green; Christophe Cellier

2007-01-01

343

Kennedy's Disease  

MedlinePLUS

... of a group of disorders called lower motor neuron disorders (which involve disruptions in the transmission of nerve cell signals in the brain to nerve cells in the brain stem and spinal cord). Onset of the disease is ...

344

Meniere's Disease  

MedlinePLUS

... pressure in some cases of Méničre?s disease. The auditory brain stem response (ABR), a computerized test of ... has advantages and drawbacks. In many people, careful control of salt in the diet and the use ...

345

Menkes Disease  

MedlinePLUS

... defective gene named ATPTA 1 that regulates the metabolism of copper in the body. The disease primarily affects male infants. Copper accumulates at abnormally low levels in the liver and brain, but at higher than normal levels ...

346

Alzheimer's Disease  

MedlinePLUS

... progressive brain disease that slowly destroys memory and thinking skills, and eventually even the ability to carry ... people. Dementia is the loss of cognitive functioning—thinking, remembering, and reasoning—and behavioral abilities, to such ...

347

Canavan Disease  

MedlinePLUS

... brain responsible for making myelin sheaths, known as oligodendrocytes, cannot properly complete this critical developmental task. Myelin ... support for nerve cells. In Canavan disease, many oligodendrocytes do not mature and instead die, leaving nerve ...

348

Liver Diseases  

MedlinePLUS

Your liver is the largest organ inside your body. It helps your body digest food, store energy, and remove poisons. There are many kinds of liver diseases. Viruses cause some of them, like hepatitis ...

349

Behcet's Disease  

MedlinePLUS

... of Behcet's disease include recurrent ulcers in the mouth (resembling canker sores) and on the genitals, and eye inflammation. The disorder may also cause various types of skin lesions, arthritis, bowel inflammation, meningitis (inflammation of the membranes ...

350

Meniere's disease.  

PubMed

Meniere's disease is a chronic illness that affects a substantial number of patients every year worldwide. The disease is characterised by intermittent episodes of vertigo lasting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus, and aural pressure. Although there is currently no cure, more than 85% of patients with Meniere's disease are helped by either changes in lifestyle and medical treatment, or minimally invasive surgical procedures such as intratympanic steroid therapy, intratympanic gentamicin therapy, and endolymphatic sac surgery. Vestibular neurectomy has a very high rate of vertigo control and is available for patients with good hearing who have failed all other treatments. Labyrinthectomy is undertaken as a last resort and is best reserved for patients with unilateral disease and deafness. PMID:18675691

Sajjadi, Hamed; Paparella, Michael M

2008-08-01

351

Diverticular Disease  

MedlinePLUS

... your large intestine. It is caused by small pouches (called diverticula) that can form anywhere in your ... disease are: Diverticulosis. People who have diverticulosis have pouches in the large intestine. Most people who have ...

352

Nail Diseases  

MedlinePLUS

... growth rate can be signs of lung, heart, kidney, and liver diseases, as well as diabetes and anemia. White spots and vertical ridges are harmless. Nail problems that sometimes require treatment include Bacterial and fungal infections Ingrown nails Tumors ...

353

Chagas disease  

MedlinePLUS

... help control the spread of the disease. Blood banks in Central and South America screen donors for ... discarded if the donor tests positive. Most blood banks in the United States began screening for Chagas ...

354

Fungal Diseases  

MedlinePLUS

... Chest & Lungs Chronic Conditions Developmental Disabilities Ear, Nose & Throat Emotional Problems Fever Genitals & Urinary Tract Head, Neck & Nervous System Obesity Skin Treatments View all Injuries & Emergencies Sports Injuries Vaccine Preventable Diseases Diphtheria Haemophilus ...

355

Graves' Disease  

MedlinePLUS

... Graves' disease can cause the following symptoms: Nervousness Insomnia Emotional swings Sweating Hand tremor Palpitations Unexplained weight ... medication such as propanolol (Inderal). For anxiety and insomnia, your doctor may prescribe diazepam (Valium), lorazepam (Ativan) ...

356

Alpers' Disease  

MedlinePLUS

... disease, failure to thrive, infection-associated encephalopathy, spasticity, myoclonus (involuntary jerking of a muscle or group of ... be difficult to control and unrelenting seizures can cause developmental regression as well. "Alpers-like" disorders without ...

357

Lung Disease  

MedlinePLUS

... Infections, such as influenza and pneumonia Lung cancer Sarcoidosis (sar-KOY-doh-sis) and pulmonary fibrosis Lung ... and can reduce oxygen flow into the blood. Sarcoidosis and pulmonary fibrosis. These inflammatory diseases cause stiffening ...

358

Alzheimer disease  

MedlinePLUS

Senile dementia - Alzheimer type (SDAT); SDAT ... The exact cause of Alzheimer disease (AD) is not known. Research shows that certain changes in the brain lead to AD developing. You are more likely ...

359

Lyme disease  

MedlinePLUS

Lyme disease is caused by bacteria called Borrelia burgdorferi ( B. burgdorferi) . Blacklegged ticks and other species of ticks can carry these bacteria. The ticks pick up the bacteria when they bite mice ...

360

Gaucher disease  

MedlinePLUS

... harmful substances to build up in the liver, spleen, bones, and bone marrow. The substances prevent cells ... common. It involves bone disease, anemia, an enlarged spleen and thrombocytopenia. Type I affects both children and ...

361

Méničre's Disease  

MedlinePLUS

... membranous labyrinth) filled with endolymph. Credit: NIH Medical Arts The symptoms of Méničre’s disease are caused by ... dizziness. Location of endolymphatic sac Credit: NIH Medical Arts Surgery. Surgery may be recommended when all other ...

362

Graves disease  

MedlinePLUS

... levels of TSH, T3, and free T4 Radioactive iodine uptake and scan This disease may also affect ... or more of the following: Antithyroid medications Radioactive iodine Surgery If you have had radioactive iodine treatment ...

363

Gaucher Disease  

MedlinePLUS

... problems and blood disorders. Is there any treatment? Enzyme replacement therapy is available for most people with types 1 ... and 3 Gaucher disease. What is the prognosis? Enzyme replacement therapy is very beneficial for type 1 and most ...

364

Fabry's Disease  

MedlinePLUS

... fever, and gastrointestinal difficulties. Is there any treatment? Enzyme replacement therapy has been approved by the U.S. Food and Drug Administration for the treatment of Fabry disease. Enzyme replacement therapy can reduce lipid storage, ease pain, and improve ...

365

Lung disease  

MedlinePLUS

... the lungs to take in oxygen and release carbon dioxide. People with this type of lung disorder often ... the lungs to take up oxygen and release carbon dioxide. These diseases may also affect heart function. An ...

366

Disease Resources  

Cancer.gov

Key Programs Disease Resources The ASCUS/LSIL Triage Study for Cervical Cancer (ALTS) Human Papillomavirus Cervical Cancer Screening NCI Bethesda System 2001 The Bethesda System Web Atlas National Cervical Cancer Coalition American Social

367

Crohn disease  

PubMed Central

Crohn disease (CD) is a chronic and debilitating inflammatory condition of the gastrointestinal tract.1 Prevalence in western populations is 100–150/100,000 and somewhat higher in Ashkenazi Jews. Peak incidence is in early adult life, although any age can be affected and a majority of affected individuals progress to relapsing and chronic disease. Medical treatments rely significantly on empirical corticosteroid therapy and immunosuppression, and intestinal resectional surgery is frequently required. Thus, 80% of patients with CD come to surgery for refractory disease or complications. It is hoped that an improved understanding of pathogenic mechanisms, for example by studying the genetic basis of CD and other forms of inflammatory bowel diseases (IBD), will lead to improved therapies and possibly preventative strategies in individuals identified as being at risk. PMID:20729636

Stappenbeck, Thaddeus S.; Rioux, John D.; Mizoguchi, Atsushi; Saitoh, Tatsuya; Huett, Alan; Darfeuille-Michaud, Arlette; Wileman, Tom; Mizushima, Noboru; Carding, Simon; Akira, Shizuo; Parkes, Miles; Xavier, Ramnik J.

2011-01-01

368

Parkinson disease  

MedlinePLUS

... with Parkinson disease: Stay healthy by eating nutritious foods and not smoking. Make changes in what you ... Jankovic J. Movement disorders. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley's Neurology in ...

369

Crohn's Disease  

MedlinePLUS

... KB) Spanish Version Additional Links Colonoscopy Microscopic Colitis Ulcerative Colitis Upper GI Endoscopy Upper GI Series Contact Us ... the GI tract, called inflammatory bowel disease (IBD). Ulcerative colitis and microscopic colitis are the other common IBDs. ...

370

Parkinson's Disease  

MedlinePLUS

... worse, people with the disease may have trouble walking, talking, or doing simple tasks. They may also have problems such as depression, sleep problems, or trouble chewing, swallowing, or speaking. There ...

371

Parkinson's Disease  

MedlinePLUS

... severe, people with the disorder may have difficulty walking, talking, or completing other simple tasks. They also experience non-motor, or movement, symptoms including mental and behavioral changes, sleep problems, depression, memory difficulties, and fatigue. Parkinson's disease ...

372

Celiac Disease  

MedlinePLUS

... Phone: 703–622–3331 Email: info@americanceliac.org Internet: www.americanceliac.org American Dietetic Association 120 South ... Chicago, IL 60606–6995 Email: hotline@eatright.org Internet: www.eatright.org Celiac Disease Foundation 13251 Ventura ...

373

Lyme Disease  

MedlinePLUS

... occur in the spring and summer months. The tick bite is rarely painful, so after any exposure to ... tick still attached to the skin. Not every tick bite will result in Lyme disease, but it is ...

374

Chronic Diseases  

Microsoft Academic Search

Although diabetes mellitus, cardiovascular disease, and human immunodeficiency virus infection are three separate entities,\\u000a each has causal and non-causal risk factors that are common in the stage 5 chronic kidney disease population. The medical\\u000a nutrition therapies are similar, which emphasize adequate protein and energy intakes, fluid control, and possibly carbohydrate\\u000a and fat modifications. Each patient requires an individualized evaluation, taking

Sharon R. Schatz

375

Meničre's disease.  

PubMed

Meničre's disease is the most common cause of true vertigo and it affects about 2.4 million Americans. Episodic tinnitus, hearing loss, vertigo, and aural fullness are the classic findings. The symptom which is most debilitating and which causes most people to seek treatment is vertigo. Most patients respond extremely well to medical management and only rarely is surgery necessary. This paper discusses the symptoms, diagnosis, and clinical course of Meničre's disease. Medical and surgical therapeutic options are discussed. PMID:7844461

Mixon, T; Letta, C; Amedee, R

1994-12-01

376

Rice Diseases.  

E-print Network

constraints (particularly fuel prices) have all but eliminated this practice that served to reduce initial inoculum of many rice diseases by deep burial of sclerotia and infected crop residue. *Extension plant pathologist , The Texas A&M University System... slower to emerge may suffer reductions in stand and potential yield not encountered with varieties that emerge rapidly. Chemical seed treatment is a very cost effective way of minimizing potential damage from seedling disease. See the seed treatment...

Jones, Roger K.

1987-01-01

377

Lentil Diseases  

Microsoft Academic Search

Fungal diseases of lentils are the most important biological constraint to productivity. Ascochyta lentis (ascochyta blight) and Fusarium oxysporum f. sp. lentis (fusarium wilt) are the major fungal pathogens that can cause severe losses in most lentil growing regions of the world.\\u000a Fungal diseases such as botrytis grey mould (Botrytis fabae and B. cinerea), rust (Uromyces viciae-fabae), stemphylium blight (Stemphylium

Paul Taylor; Kurt Lindbeck; Weidong Chen; Rebecca Ford

378

Parkinson's Disease  

Microsoft Academic Search

In this chapter the topic of inflammation in Parkinson’s disease (PD) and in models of human disease will be reviewed. To\\u000a set the stage, the biological, clinical and pathological hallmarks of PD and related degenerative conditions will be presented.\\u000a A more comprehensive review of PD can be found in the following two references (Fahn and Przedborski, 2005; Dauer and Przedborski,

Serge Przedborski

379

Méničre Disease  

Microsoft Academic Search

A computerized PubMed search of MEDLINE 1966-May 2005 was performed. The terms “Meniere disease” and “gentamicin” were exploded,\\u000a and the resulting articles were combined. The terms “intratympanic” and “transtympanic” were entered as text words as the\\u000a search term “intratympanic OR transtympanic,” and the results were combined with the Méničre disease\\/gentamicin articles.\\u000a The resulting 136 articles were limited to the English

Iee-Ching Wu Anderson; John P. Carey; Walter Kutz; William H. Slattery

380

Caroli's disease.  

PubMed

Caroli's disease is characterized by multifocal segmental dilatation of the intrahepatic bile ducts. It is a rare congenital condition, which appears to be autosomal recessively inherited in most cases. There are two forms of disease, one associated with congenital hepatic fibrosis and a simple form occurring alone. Recent reports suggest that the simple form may be as common as that with congenital hepatic fibrosis. Other conditions, including choledochal cyst and renal cystic disease, are frequently associated. The major clinical feature is recurrent cholangitis, which may be complicated by intrahepatic calculi and hepatic abscess formation. There is good evidence that malignancy complicates Caroli's disease in approximately 7% of cases. The diagnosis rests on demonstrating that the cystic liver lesions are in continuity with the biliary tree. Modern imaging techniques allow the diagnosis to be made more easily and without invasive imaging of the biliary tree. The treatment depends on the clinical features and the location of the biliary abnormality. When the disease is localized to one hepatic lobe, hepatectomy relieves symptoms and appears to remove the risk of malignancy. In diffuse Caroli's disease, treatment options include conservative or endoscopic therapy, internal biliary bypass procedures and liver transplantation in carefully selected cases. PMID:9581983

Taylor, A C; Palmer, K R

1998-02-01

381

Diffuse Parenchymal Diseases Associated With Aluminum Use and Primary Aluminum Production  

PubMed Central

Aluminum use and primary aluminum production results in the generation of various particles, fumes, gases, and airborne materials with the potential for inducing a wide range of lung pathology. Nevertheless, the presence of diffuse parenchymal or interstitial lung disease related to these processes remains controversial. The relatively uncommon occurrence of interstitial lung diseases in aluminum-exposed workers—despite the extensive industrial use of aluminum—the potential for concurrent exposure to other fibrogenic fibers, and the previous use of inhaled aluminum powder for the prevention of silicosis without apparent adverse respiratory effects are some of the reasons for this continuing controversy. Specific aluminum-induced parenchymal diseases described in the literature, including existing evidence of interstitial lung diseases, associated with primary aluminum production are reviewed. PMID:24806728

2014-01-01

382

Potential treatment of inflammatory bowel disease: a review of helminths therapy  

PubMed Central

An inflammatory bowel disease (IBD) is most common in highly industrialized Western countries but uncommon in less developed areas of the world where helminths are frequent. The hygiene hypothesis proposes that the recent increase in allergic and autoimmune diseases is due to modern highly hygienic life styles and medical conditions. Loss of routine exposure to parasitic helminths, as a result of increasing lifestyle-associated factors, may be one factor leading to the increased disease prevalence. In animal models and clinical trials of IBD, gastrointestinal nematodes colonization suppresses intestinal inflammation through multiple mechanisms including induction of innate and adaptive regulatory circuits. Studies using helminths like Trichuris suis or Necator americanus showed that these helminths are safe and may be effective therapeutic approaches for the control of IBD and other immune diseases. The aim of present review was to exploring the therapeutic use of helminths for the control of IBD. PMID:25436093

Taghipour, Niloofar; Aghdaei, Hamid Asadzadeh; Haghighi, Ali; Mossafa, Nariman; Tabaei, Seyyed Javad Seyyed

2014-01-01

383

Adopting orphans: comprehensive genetic testing of Mendelian diseases of childhood by next-generation sequencing  

PubMed Central

Orphan diseases are individually uncommon but collectively contribute significantly to pediatric morbidity, mortality and healthcare costs. Current molecular testing for rare genetic disorders is often a lengthy and costly endeavor, and in many cases a molecular diagnosis is never achieved despite extensive testing. Diseases with locus heterogeneity or overlapping signs and symptoms are especially challenging owing to the number of potential targets. Consequently, there is immense need for scalable, economical, rapid, multiplexed diagnostic testing for rare Mendelian diseases. Recent advances in next-generation sequencing and bioinformatic technologies have the potential to change the standard of care for the diagnosis of rare genetic disorders. These advances will be reviewed in the setting of a recently developed test for 592 autosomal recessive and X-linked diseases. PMID:22022947

Kingsmore, Stephen F; Dinwiddie, Darrell L; Miller, Neil A; Soden, Sarah E; Saunders, Carol J

2011-01-01

384

Infection and Cardiovascular Disease  

ClinicalTrials.gov

Cardiovascular Diseases; Coronary Disease; Cerebrovascular Accident; Heart Diseases; Myocardial Infarction; Infection; Chlamydia Infections; Cytomegalovirus Infections; Helicobacter Infections; Atherosclerosis

2005-06-23

385

[Allergic diseases as environmental diseases].  

PubMed

Respiratory allergic diseases belong to atopic diseases. Their prevalence has steadily increased over the past decades. Recently, a plateau effect seems to occur. This increased prevalence should be related to environmental changes. However, "classical" aerocontaminants, such as aero-allergens and air pollutants cannot account for such a drastic increase in prevalence. Change in diet can account for biological changes but the relation to allergic diseases seems questionable. Use of contraceptive pills and caesarean section had been put forward as hypotheses but their implication seems unlikely. Among the classical risk factors, 2 only should remain in the primary prevention of atopic diseases, namely, prolonged breast feeding and avoiding passive smoking. The major input of epidemiological research over the past decades lies in the "hygienic hypothesis" and the protective effect of early exposure to farm animals and raw cow milk. Progress to come in this field should lie in a better knowledge of in utero exposure on the foetal immune system. PMID:17717941

Charpin, Denis

2007-06-30

386

Neurodegenerative diseases ranging from Alzheimer disease and polyglutamine diseases to transmissible spongiform enceph-  

E-print Network

, we focus on three neuro- degenerative diseases, Alzheimer disease, Huntington disease and prion with Neurodegenerative Diseases The protein deposits found in Alzheimer disease, Huntington disease and prion diseaseNeurodegenerative diseases ranging from Alzheimer disease and polyglutamine diseases

Lindquist, Susan

387

The gastric cardia in gastro-oesophageal disease  

PubMed Central

Background—There have been conflicting reports concerning the use of cardia biopsies in screening patients for gastro-oesophageal disease. Aim—To define the histopathological changes in the gastric cardia of patients with and without gastro-oesophageal disease. Methods—Topographically mapped gastric biopsy specimens were obtained from patients with gastro-oesophageal disease and from controls. Biopsies were scored on a visual analogue scale of 0 to 5 for Helicobacter pylori, intestinal metaplasia, pancreatic metaplasia, foveolar hyperplasia, and active inflammation. The presence or absence of cardiac glands was recorded. Results—Sixty five patients with gastro-oesophageal disease and 71 controls were examined. Intestinal metaplasia was present in cardia biopsies of 10 patients with gastro-oesophageal disease and 11 controls. Only two patients with gastro-oesophageal disease and intestinal metaplasia in the cardia had no evidence of exposure to H pylori. Intestinal metaplasia was not found in the cardia of those with long segment Barrett's oesophagus. Carditis was strongly associated with active H pylori infection (p = 0.000) and resolved after treatment of the infection. A negative association was present between gastro-oesophageal disease and the presence of cardiac glands in cardiac biopsies (p = 0.003). Pancreatic metaplasia was found in 15 of 65 and foveolar hyperplasia in 19 of 65 cases but neither was related to gastro-oesophageal disease. Conclusion—Intestinal metaplasia in the cardia is uncommon in gastro-oesophageal disease in the absence of H pylori infection. With chronic H pylori infection the junction between the cardia and corpus expands in a cardia–corpal direction. Key Words: Barrett's oesophagus • gastro-oesophageal disease • cardia • Helicobacter pylori PMID:11002767

El-Zimaity, H.; Verghese, V.; Ramchatesingh, J.; Graham, D.

2000-01-01

388

Meničre's disease  

PubMed Central

Introduction Meničre's disease causes recurrent vertigo, hearing loss, tinnitus, and fullness or pressure in the ear, which mainly affects adults aged 40-60 years. Meničre's disease is at first progressive but fluctuating, and episodes can occur in clusters. Vertigo usually resolves but hearing deteriorates, and symptoms other than hearing loss and tinnitus usually improve regardless of treatment. Methods and outcomes We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of treatments for acute attacks of Meničre's disease; and of interventions to prevent attacks and delay disease progression of Meničre's disease? We searched: Medline, Embase, The Cochrane Library and other important databases up to January 2006 (BMJ Clinical Evidence reviews are updated periodically, please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found 17 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review we present information relating to the effectiveness and safety of the following interventions: anticholinergics, benzodiazepines, betahistine, cinnarizine, dietary modification, diuretics, phenothiazines, psychological support, trimetazidine, vestibular rehabilitation. PMID:19454061

2007-01-01

389

Wilson's disease.  

PubMed

(Full text is available at http://www.manu.edu.mk/prilozi). Wilson's disease (WD) is a disorder of copper transport resulting from the defective function of a copper transporting P-type ATPase, ATP7B. The WD incidence is approximately 1/50-10,000 live births worldwide. Clinical manifestations of WD may be of any kind, but usually the symptoms of presentation are hepatic or neuropsychiatric, with a vast range of disturbances for both groups of symptoms. In children, however, clinical symptoms may be absent, making the diagnosis of the disease more difficult than in adults. Hepatic manifestations may range from asymptomatic minor biochemical disturbances, to acute, but mostly chronic, hepatitis, cirrhosis or severe fulminant hepatic failure. The spectrum of neurological manifestations is wide, including tremor, hypersalivation, Dysarthria, coordination defects, dystonia, ataxia. The spectrum of psychiatric manifestations is considerable and may include different disturbances such as altered working performance, anxiety, depression and antisocial behaviour. Kayser-Fleischer rings (KF) are present in 95% of patients with neurological symptoms and somewhat over half of those without neurological symptoms. In children presenting with liver disease, KF rings are usually absent. To obtain a more reliable diagnosis of WD, the Leipzig scoring system was proposed by an international consensus of experts. Wilson's disease copper overload is treated with chelating agents such as penicillamine, trientine and tetrathiomolybdate. Zinc is used mostly for mantainance therapy or the treatment of asymptomatic WD patients. Key words: Wilson diseases, copper, cirrhosis, children. PMID:24798599

Loudianos, G; Lepori, M B; Mameli, E; Dessě, V; Zappu, A

2014-01-01

390

Fabry's disease.  

PubMed

Fabry's disease is an X-linked lysosomal storage disorder caused by abnormalities in the GLA gene, which leads to a deficiency in ?-galactosidase A. The abnormal accumulation of glycosphingolipids, primarily globotriaosylceramide, manifests as serious and progressive impairment of renal and cardiac functions. In addition, patients experience pain, gastrointestinal disturbance, transient ischemic attacks and strokes. Disease presentation in female heterozygotes may be as severe as in males although women may also remain asymptomatic. This review covers all basic aspects of the disease such as epidemiology, pathophysiology, clinical presentation by systems, diagnosis, management, prevention, and repercussions on quality of life. With the development of enzyme replacement therapy in the past few years, early initiation of treatment was found to be key for reduction of disease burden in major affected organs with improvement in neuropathic pain, decreased cardiac mass and stabilization of renal function, gastrointestinal symptoms, and hearing. This review aims to raise the awareness of the signs and symptoms of Fabry's disease as well as to provide guidelines for the diagnosis and treatment. PMID:25106696

El-Abassi, Rima; Singhal, Divya; England, John D

2014-09-15

391

Skeletal muscle disease: patterns of MRI appearances  

PubMed Central

Although the presumptive diagnosis of skeletal muscle disease (myopathy) may be made on the basis of clinical–radiological correlation in many cases, muscle biopsy remains the cornerstone of diagnosis. Myopathy is suspected when patients complain that the involved muscle is painful and tender, when they experience difficulty performing tasks that require muscle strength or when they develop various systemic manifestations. Because the cause of musculoskeletal pain may be difficult to determine clinically in many cases, MRI is increasingly utilised to assess the anatomical location, extent and severity of several pathological conditions affecting muscle. Infectious, inflammatory, traumatic, neurological, neoplastic and iatrogenic conditions can cause abnormal signal intensity on MRI. Although diverse, some diseases have similar MRI appearances, whereas others present distinct patterns of signal intensity abnormality. In general, alterations in muscle signal intensity fall into one of three cardinal patterns: muscle oedema, fatty infiltration and mass lesion. Because some of the muscular disorders may require medical or surgical treatment, correct diagnosis is essential. In this regard, MRI features, when correlated with clinical and laboratory findings as well as findings from other methods such as electromyography, may facilitate correct diagnosis. This article will review and illustrate the spectrum of MRI appearances in several primary and systemic disorders affecting muscle, both common and uncommon. The aim of this article is to provide radiologists and clinicians with a collective, yet succinct and useful, guide to a wide array of myopathies. PMID:22960244

Theodorou, D J; Theodorou, S J; Kakitsubata, Y

2012-01-01

392

Leptospirosis and Weil's disease in the UK.  

PubMed

The recent high-profile death of a British Olympic rower from leptospirosis has raised awareness to this uncommon but potentially fatal disease. The re-emergence of the disease abroad is well documented in the literature, but less is known about cases in the UK. The increase in participation in water sports, foreign travel and often a combination of the two, has increased the exposure of tourists subsequently returning to the UK from areas of high prevalence. Leptospirosis is a zoonotic infection. The bacteria are shed in the urine of animals to the environment from where humans are infected by incidental hosts. There is a wide spectrum of severity of symptoms, from a self-limiting febrile illness to fatal pulmonary haemorrhage, renal or liver failure. It is thought that cases remain unrecognized every year in the UK, largely due to the mild nature of symptoms and the wide differential for febrile illness and partly due to lack of awareness among clinicians. This review examines the epidemiology of leptospirosis in the UK, over the period 2006-10, the clinical features, diagnostic techniques and treatment. PMID:22843698

Forbes, A E; Zochowski, W J; Dubrey, S W; Sivaprakasam, V

2012-12-01

393

Gallstone disease in children  

Microsoft Academic Search

Context  Little is known about the epidemiology of cholelithiasis in children. Cholelithiasis and choledocholithiasis were considered\\u000a to be uncommon in infants and children but have been increasingly diagnosed in recent years due to widespread use of ultrasonography.\\u000a However, there is not much of information from India and no consensus among Indian pediatricians and pediatric surgeons regarding\\u000a management of gallstones in children.

Ujjal Poddar; Sanjay Gandhi

2010-01-01

394

Single Gene Disease Risk  

Microsoft Academic Search

\\u000a The diagnosis of a child with a single gene disorder can take on different meanings for different families. It is not uncommon\\u000a for some families to arrive at a pediatric genetics clinic after months or years of searching for an underlying reason for\\u000a their child’s symptoms. The fact that, through genetic testing, clinicians can put a name to the collection

Tricia See; Cynthia J. Tifft

395

Crohn's Disease  

NSDL National Science Digital Library

This patient education program explains Crohn's Disease, one of the most common inflammatory bowel diseases, including the symptoms, diagnosis, and treatment options. It also reviews the anatomy of the gastrointestinal system, causes of the disease, dietary triggers, and pregnancy in the Crohn's patient. This resource is a MedlinePlus Interactive Health Tutorial from the National Library of Medicine, designed and developed by the Patient Education Institute. NOTE: This tutorial requires a special Flash plug-in, version 4 or above. If you do not have Flash, you will be prompted to obtain a free download of the software before you start the tutorial. You will also need an Acrobat Reader, available as a free download, in order to view the Reference Summary.

Patient Education Institute

396

Celiac disease.  

PubMed

On the basis of strong evidence, gastrointestinal symptoms and failure to thrive are classic presentations of celiac disease, but atypical, nongastrointestinal symptoms are also extremely common, particularly in the older child and adolescent. (3)(4)(8). On the basis of some research evidence and consensus, guidelines recommend celiac testing in symptomatic children with typical and atypical symptoms and consideration of testing in those with associated conditions and first-degree relatives of those with celiac disease. (3)(9). On the basis of strong research evidence, measurement of tTG IgA and total serum IgA level has been reported to be the most cost-effective and accurate means of serologic testing for celiac disease and is the test of choice unless the child is younger than 2 years or IgA deficient. (9). On the basis of strong research evidence, children with elevated titers of celiac antibodies or strong clinical suspicion for celiac disease should be referred to a gastroenterologist for upper endoscopy and biopsy. Until this procedure is performed, the child should continue on a diet with ingestion of gluten. (3)(9). On the basis of strong research evidence, all those with a confirmed diagnosis of celiac disease should follow a strict gluten-free diet for life, with avoidance of all foods that contain wheat, barley, and rye ingredients. (3)(4). Referral to a health care professional with specialized knowledge of celiac disease and the gluten-free diet is critical because of the numerous ways, often hidden, in which gluten may be present in the diet and environment. PMID:25274968

Ediger, Tracy R; Hill, Ivor D

2014-10-01

397

Pilonidal Disease  

PubMed Central

Pilonidal disease presents many therapeutic challenges to surgeons throughout the world. Its varied clinical presentations necessitate a wide range of treatments, thus underscoring the need to tailor the treatment to the patient and the severity of disease. Recent studies confirm the efficacy of smaller, more conservative operations for appropriate indications. When flap closures are performed, every attempt should be directed to placing sutures off (lateral) to the midline gluteal cleft. Meticulous attention to the details of immediate and long-term postoperative care is paramount. PMID:22379405

Khanna, Amit; Rombeau, John L.

2011-01-01

398

Castleman's Disease with Cutaneous Involvement Manifestating as Multiple Violaceous Plaques on Entire Body  

PubMed Central

Castleman's disease (CD) is an uncommon B-cell lymphoproliferative disorder characterized by lymph node hyperplasia with vascular proliferation. Cutaneous involvement in CD is rare. A 65-year-old man presented with a 7-year history of gradually developing multiple reddish to violaceous indurated plaques on the scalp, trunk, and legs. On physical examination, there were palpable enlarged cervical, axillary, and inguinal lymph nodes. Laboratory examination revealed anemia, thrombocytosis, hyperproteinemia, hypoalbuminemia, and polyclonal hypergammaglobulinemia. An inguinal lymph node biopsy and a skin biopsy were performed and the patient was diagnosed with the plasma cell type of CD. Chemotherapy was started and the lesions have responded to treatment. PMID:22148042

Park, Hyeon-Young; Lee, Je-Jung; Lee, Jee-Bum; Kim, Seong-Jin; Lee, Seung-Chul; Won, Young Ho

2011-01-01

399

Development of non-Hodgkin's lymphoma following therapy for Hodgkin's disease  

SciTech Connect

Three patients developed non-Hodgkin's lymphoma (NHL) 3 to 6 years after treatment for Hodgkin's disease (HD). In no instance was there evidence of recurrence of HD following the initial chemotherapy or radiotherapy. None of these patients had received both radiation therapy and chemotherapy. All patients responded well to conventional chemotherapy for NHL and are alive at 23 +, 37 +, and 65+ months after that secondary diagnosis. This report, when coupled with at least ten other such reported patients, suggests that NHL may be a relatively uncommon but significant complication of therapy for HD and must be distinguished for recurrence of HD.

Kim, H.D.; Bedetti, C.D.; Boggs, D.R.

1980-12-15

400

Histiocytic necrotising lymphadenitis (Kikuchi-Fujimoto disease) of axillary lymph nodes.  

PubMed

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotising lymphadenitis is a rare entity, occurring most commonly in young Asian adults. KFD is characterised by fever with tender lymph node enlargement. The cervical group of lymph nodes is most commonly involved, and the diagnosis is conclusively made by lymph node biopsy and histopathology. KFD is a self-limiting condition, which usually resolves over 1-4?months. Symptomatic treatment with antipyretics and/or non-steroidal anti-inflammatory drugs is recommended. Here we describe an uncommon presentation of KFD in a young woman in which only the axillary lymph nodes were enlarged. PMID:25564582

Nagaraju, Santosh; Vaishnav, Sakshi; Burke, Leandra H; Norman, Earl M

2015-01-01

401

Anterior ischemic optic neuropathy associated with Vogt–Koyanagi–Harada disease  

Microsoft Academic Search

Background  Optic disc swelling is a common finding associated with Vogt–Koyanagi–Harada disease (VKH); however, visual field loss from\\u000a optic disc involvement is uncommon. This reports report presents recent findings regarding unusual patients with visual field\\u000a defects from optic disc involvement, thus suggesting the presence of anterior ischemic optic neuropathy (AION) in the acute\\u000a phase of VKH.\\u000a \\u000a \\u000a \\u000a Methods  Observational case series. A consecutive

Kumiko Nakao; Yuka Mizushima; Noriko Abematsu; Nanako Goh; Taiji Sakamoto

2009-01-01

402

Porocarcinoma coexisting at a site of Bowen disease in a 63-year-old woman.  

PubMed

Porocarcinoma is an unusual, locally aggressive and potentially fatal neoplasm. Several cutaneous malignancies have been described in association with porocarcinoma, including squamous cell carcinoma, basal cell carcinoma and tricholemmal carcinoma. Previous reports have indicated that the occurrence of malignant tumours in combination with porocarcinoma is extremely rare, in particular with regard to Bowen disease (BD). We report an uncommon case of porocarcinoma occurring synchronously in a single BD lesion in a 63-year-old woman with multiple BD lesions. The clinical and histological findings confirmed this diagnosis. PMID:25559897

Zheng, L-Q; Han, X-C; Huang, Y; Li, H-W; Niu, X-D; Li, J

2015-04-01

403

When should a nephrologist suspect a mitochondrial disease?  

PubMed

Mitochondrial diseases, taking into account those that affect the processes of the respiratory chain (RC) and mitochondrial oxidative phosphorylation system (OXPHOS), make up a relatively frequent group within rare diseases that usually have multisystem involvement, a very variable phenotypic expression and a complex genetic base. Renal involvement is uncommon, with the tubule being the most affected, specifically its proximal portion, developing into full Toni-Debré-Fanconi syndrome in the most serious cases. However, in some cases the glomerulus is involved, fundamentally in focal segmental glomerulosclerosis form (FSGS), expressed by proteinuria and renal failure. It is important that the Nephrologist keeps in mind the possibility of a mitochondrial disease in patients with this type of renal involvement that present clinical data with these characteristics, especially diabetes mellitus and deafness. In cases with FSGS, a correct diagnosis will avoid the inappropriate use of immunosuppressive medication. Specific treatments do not exist for the majority of mitochondrial diseases, but it is likely that the intense research that currently exists for these diseases will eventually produce effective treatment possibilities. PMID:25611829

Cavero, Teresa; Rabasco, Cristina; Molero, Aida; Blázquez, Alberto; Hernández, Eduardo; Martín, Miguel A; Praga, Manuel

2015-01-01

404

Lung Diseases  

MedlinePLUS

When you breathe, your lungs take in oxygen from the air and deliver it to the bloodstream. The cells in your body need oxygen to ... you breathe nearly 25,000 times. People with lung disease have difficulty breathing. Millions of people in ...

405

Graves' Disease  

MedlinePLUS

... disease that affects the adrenal glands, which make hormones that help your body respond to stress and regulate your blood pressure and water and ... find the gene or genes involved. Gender. Sex hormones might play a role, ... than men. Stress. Severe emotional stress or trauma might trigger the ...

406

Peyronie's Disease  

MedlinePLUS

... Behcet’s syndrome––inflammation of the blood vessels Family History of Peyronie’s Disease Medical experts believe that Peyronie’s ... a physical exam imaging tests Medical and Family History Taking a medical and family history is one ...

407

SMUT DISEASES  

Technology Transfer Automated Retrieval System (TEKTRAN)

A COMPREHENSIVE REVIEW OF MOST ASPECTS OF COMMON BUNT AND DWARF BUNT DISEASES OF WHEAT IS PRESENTED. INCLUDED ARE SECTIONS ON HISTORY, DISTRIBUTION AND ECONOMIC IMPORTANCE, TAXONOMY, MORPHOLOGY, SPORE GERMINATION, CULTURE, AND PHYSIOLOGY. EXTENSIVE SECTIONS DEAL WITH RESEARCH METHODOLOGY AND DISEA...

408

Moyamoya disease.  

PubMed

A 4 years old boy presented with acute left hemiplegia. Preliminary neuroimaging suggested an arterial ischemic process. Clinical and laboratory evaluation excluded haematologic, metabolic and vasculitic causes. Cerebral angiography confirmed the diagnosis of Moyamoya disease. Treatment included physiotherapy and close follow-up for recurrence. PMID:21798142

Rafiq, Asim; Vaqar, Abeer; Javaid, Khalid Hussain; Parveen, Rashida; Sadaf, Rabia

2011-08-01

409

Smelling Diseases  

NSDL National Science Digital Library

We all use our noses to make quick judgments from time to time -- whether it's checking to see if the milk's still good, or if a shirt needs to go in the wash. Now, doctors are developing a kind of sniff test to screen for diseases. Find out more in this Science Update.

Science Update

2004-06-14

410

Prion Diseases  

MedlinePLUS

... nasal test for the human prion disease CJD Javascript Error Your browser JavaScript is turned off causing certain features of the ... incorrectly. Please visit your browser settings and turn JavaScript on. Read more information on enabling JavaScript. Prion ...

411

Infectious Diseases  

NSDL National Science Digital Library

With the threat of a warmer, wetter world and a larger global population, scientists are researching how climate change may impact the spread of infectious diseases,ťsuch as cholera and dengue fever, and how outbreaks may be prevented.ť "Changing Planet" is produced in partnership with the National Science Foundation.

NBC Learn

2010-10-07

412

Avocado diseases  

Microsoft Academic Search

Several fungi can cause diseases of avocado (Persea americana (Mill.)) of which Phytophthora cinnamomi Rands is the most serious. Phytophthora root rot causes extensive losses of avocado trees in nearly every country where avocados are grown. The fungus can be isolated from soil and roots by using selective agar media containing antibiotic chemicals and by using various types of baits

G. A. Zentmyer

1984-01-01

413

Dent's disease  

Microsoft Academic Search

Dent's disease is a renal tubular disorder characterized by manifestations of proximal tubule dysfunction, including low-molecular-weight proteinuria, hypercalciuria, nephrolithiasis, nephrocalcinosis, and progressive renal failure. These features are generally found in males only, and may be present in early childhood, whereas female carriers may show a milder phenotype. Prevalence is unknown; the disorder has been reported in around 250 families to

Olivier Devuyst; Rajesh V. Thakker

2010-01-01

414

Coronary heart disease  

MedlinePLUS

Heart disease, Coronary heart disease, Coronary artery disease; Arteriosclerotic heart disease; CHD; CAD ... Coronary heart disease (CHD) is the leading cause of death in the United States for men and women. Coronary ...

415

Diabetic Heart Disease  

MedlinePLUS

... from the NHLBI on Twitter. What Is Diabetic Heart Disease? The term "diabetic heart disease" (DHD) refers ... Kidney Diseases' Introduction to Diabetes Web page. What Heart Diseases Are Involved in Diabetic Heart Disease? DHD ...

416

Acid Lipase Disease  

MedlinePLUS

NINDS Acid Lipase Disease Information Page Synonym(s): Cholesterol Ester Storage Disease, Wolman’s Disease Table of Contents (click to jump ... research is being done? Clinical Trials What is Acid Lipase Disease ? Acid lipase disease occurs when the ...

417

American Behcet's Disease Association  

MedlinePLUS

... through a grant from Celgene Corporation. Rare Disease Day Rare Disease Day Behcet's Disease Awareness Share your story and educate ... Behcet's Disease Month May 20th is Behcet's Awareness Day American Behcet's Disease Association Contact Us | Website Policy | ...

418

Coronary Heart Disease  

MedlinePLUS

... from the NHLBI on Twitter. What Is Coronary Heart Disease? Espańol Coronary heart disease (CHD) is a disease ... Coronary Heart Disease, visit www.clinicaltrials.gov . Coronary Heart Disease in the News November 18, 2014 NHLBI Media ...

419

HIV and Rheumatic Disease  

MedlinePLUS

... Patient Resources > Diseases & Conditions Back to Diseases & Conditions HIV and Rheumatic Disease PRINT Download PDF HIV infection ... treatment and HIV infection all overlap. What are HIV-associated rheumatic diseases? Some diseases of the joints ...

420

Fabry disease  

PubMed Central

Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal ?-galactosidase A activity. FD is pan-ethnic and the reported annual incidence of 1 in 100,000 may underestimate the true prevalence of the disease. Classically affected hemizygous males, with no residual ?-galactosidase A activity may display all the characteristic neurological (pain), cutaneous (angiokeratoma), renal (proteinuria, kidney failure), cardiovascular (cardiomyopathy, arrhythmia), cochleo-vestibular and cerebrovascular (transient ischemic attacks, strokes) signs of the disease while heterozygous females have symptoms ranging from very mild to severe. Deficient activity of lysosomal ?-galactosidase A results in progressive accumulation of globotriaosylceramide within lysosomes, believed to trigger a cascade of cellular events. Demonstration of marked ?-galactosidase A deficiency is the definitive method for the diagnosis of hemizygous males. Enzyme analysis may occasionnally help to detect heterozygotes but is often inconclusive due to random X-chromosomal inactivation so that molecular testing (genotyping) of females is mandatory. In childhood, other possible causes of pain such as rheumatoid arthritis and 'growing pains' must be ruled out. In adulthood, multiple sclerosis is sometimes considered. Prenatal diagnosis, available by determination of enzyme activity or DNA testing in chorionic villi or cultured amniotic cells is, for ethical reasons, only considered in male fetuses. Pre-implantation diagnosis is possible. The existence of atypical variants and the availability of a specific therapy singularly complicate genetic counseling. A disease-specific therapeutic option - enzyme replacement therapy using recombinant human ?-galactosidase A - has been recently introduced and its long term outcome is currently still being investigated. Conventional management consists of pain relief with analgesic drugs, nephroprotection (angiotensin converting enzyme inhibitors and angiotensin receptors blockers) and antiarrhythmic agents, whereas dialysis or renal transplantation are available for patients experiencing end-stage renal failure. With age, progressive damage to vital organ systems develops and at some point, organs may start to fail in functioning. End-stage renal disease and life-threatening cardiovascular or cerebrovascular complications limit life-expectancy of untreated males and females with reductions of 20 and 10 years, respectively, as compared to the general population. While there is increasing evidence that long-term enzyme therapy can halt disease progression, the importance of adjunctive therapies should be emphasized and the possibility of developing an oral therapy drives research forward into active site specific chaperones. PMID:21092187

2010-01-01

421

Morgellons disease?  

PubMed

Morgellons disease, a pattern of dermatologic symptoms very similar, if not identical, to those of delusions of parasitosis, was first described many centuries ago, but has recently been given much attention on the internet and in the mass media. The present authors present a history of Morgellons disease, in addition to which they discuss the potential benefit of using this diagnostic term as a means of building trust and rapport with patients to maximize treatment benefit. The present authors also suggest "meeting the patient halfway" and creating a therapeutic alliance when providing dermatologic treatment by taking their cutaneous symptoms seriously enough to provide both topical ointments as well as antipsychotic medications, which can be therapeutic in these patients. PMID:18318880

Accordino, Robert E; Engler, Danielle; Ginsburg, Iona H; Koo, John

2008-01-01

422

Thyroid disease  

SciTech Connect

Presenting a multidisciplinary approach to the diagnosis and treatment of thyroid disease, this volume provides a comprehensive picture of current thyroid medicine and surgery. The book integrates the perspectives of the many disciplines that deal with the clinical manifestations of thyroid disorders. Adding to the clinical usefulness of the book is the state-of-the-art coverage of many recent developments in thyroidology, including the use of highly sensitive two-site TSH immunoradionetric measurements to diagnose thyroid activity; thyroglobulin assays in thyroid cancer and other diseases; new diagnostic applications of MRI and CT; treatment with radionuclides and chemotherapy; new developments in thyroid immunology, pathology, and management of hyperthyroidism; suppressive treatment with thyroid hormone; and management of Graves' ophthalmopathy. The book also covers all aspects of thyroid surgery, including surgical treatment of hyperthyroidism; papillary, follicular, and other carcinomas; thyroidectomy; and prevention and management of complications.

Falk, S.

1990-01-01

423

Angiolymphoid hyperplasia with eosinophilia of the eyelid and orbit: the Western cousin of Kimura's disease?  

PubMed

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon disease most frequently occurring in the head and neck region. It occurs in all races. In spite of a clear distinction between ALHE and Kimura's disease on histological grounds for almost 20 years now, confusion and misdiagnosis still exist to date. Three Spanish patients presented to our institution over a 6 year period with mass lesions in the superior orbit, medial orbito-nasal wall and eyelid, respectively. All patients were of Caucasian extraction and were diagnosed with epithelioid haemangioma on histological tissue analysis. We report on the history and management of these patients which resulted in good functional and cosmetic outcome with no subsequent evidence of disease recurrence. PMID:23460400

Cunniffe, G; Alonso, T; Dinarčs, C; Medina, F J; Medel, R

2014-02-01

424

Castleman disease mimicked pancreatic carcinoma: report of two cases.  

PubMed

Castleman disease (CD) is an uncommon benign lymphoproliferative disorder, which usually presents as solitary or multiple masses in the mediastinum. Peripancreatic CD was rarely reported. Herein, we report two cases of unicentric peripancreatic CD from our center. A 43-year-old man and a 58-year-old woman were detected to have a pancreatic mass in the routine medical examinations. Both of them were asymptomatic. The computed tomography and ultrasonographic examination revealed a mild enhancing solitary mass at the pancreatic head/neck. No definite preoperative diagnosis was established and Whipple operations were originally planned. The intraoperative frozen section diagnosis of both patients revealed lymphoproliferation. Then the local excisions of mass were performed. Histological examination revealed features of CD of hyaline-vascular type. No recurrence was found during the follow-up period. CD should be included in the differential diagnosis of pancreatic tumors. Local excision is a suitable surgical choice. PMID:22824621

Guo, Hua; Shen, Yan; Wang, Wei-Lin; Zhang, Min; Li, Hong; Wu, Ying-Sheng; Yan, Sheng; Xu, Xiao; Wu, Jian; Zheng, Shu-Sen

2012-01-01

425

Postpartum Acute Pulmonary Oedema with Sub clinical Rheumatic Heart Disease  

PubMed Central

Acute dyspnea with pulmonary oedema in postpartum is uncommon but life-threatening event. Contributing factors for pulmonary oedema include, administration of tocolytics, underlying cardiac disease, iatrogenic fluid overload and preeclampsia acounting 0.08% of pregnancies. Pulmonary embolism, amniotic fluid embolism, pneumonia, aspiration and pulmonary oedema are some of the potentially devastating conditions that should be considered by the attending physician. Here, we report a case of postpartum acute pulmonary oedema referred to causality after an emergency caesarean section in a private hospital. No matter what the underlying pathology, prompt administration and appropriate resuscitation is always the first priority. Only after the patient has been stabilized attention must be turned to diagnosis and specific treatment. A diagnosis of severe Mitral Stenosis, probably of rheumatic origin was made after stabilizing the patient.

Gande, Sri Krishna Padma Challa Rao.

2015-01-01

426

Kidney Disease  

Microsoft Academic Search

Crohn’s disease (CD) potentially affects many organs, including, albeit rarely, the kidney. The most frequent renal manifestations\\u000a are calcium oxalate stones and their complications; secondary amyloidosis; acute tubular necrosis related to volume depletion\\u000a caused by diarrheal fluid losses or inadequate fluid intake, or both. Tubulo-interstitial nephritis is another common finding\\u000a and, among patients with CD, the most common cause is

Margherita Rosa; Salvatore Giulio

427

Wilson's disease  

Microsoft Academic Search

Wilson's disease is an autosomal recessive disorder of hepatic copper disposition caused by mutations in the gene ATP7B, located on chromosome 13. This gene encodes a P-type adenosine triphosphatase (ATPase), known as the Wilson ATPase, which functions within hepatocytes to move copper across intracellular membranes. The copper-transporting action directly supports production of the ferroxidase caeruloplasmin, in which copper is incorporated,

Eve A. Roberts

2011-01-01

428

[Wilsons disease].  

PubMed

Wilsons disease is an autosomal recessive genetic disorder in which copper accumulates in tissues, especially in the liver and the brain. The genetic defect affects the P type ATPase gene (ATP7B). More than 500 mutations causing Wilsons disease have been described. The most common mutation in Central Europe concerns H1069Q. The symptoms of Wilsons disease include hepatic or neurological conditions. The hepatic condition is manifested as steatosis, acute or chronic hepatitis or cirrhosis. The neurological conditions are most often manifested after the age of 20 as motor disorders (tremor, speech and writing disorders), which may result in severe extrapyramidal syndrome with rigidity, dysarthria and muscle contractions. The dia-gnosis is based on clinical and laboratory assessments (neurological signs, liver lesions, low ceruloplasmin, increased free serum copper, high Cu volumes in urine, KayserFleischer ring). The dia-gnosis is confirmed by a high Cu level in liver tissue or genetic proof. Untreated Wilsons disease causes death of the patient. If treated properly the survival rate approximates to the survival rate of the common population. The treatment concerns either removal of copper from the body using chelating agents excreted into the urine (Penicillamine, Trientine) or limitation of copper absorption from the intestine and reducing the toxicity of copper (zinc, ammonium tetrathiomolybdate). In the Czech Republic, Penicillamine or zinc is used. A liver transplant is indicated in patients with fulminant hepatic failure or decompensated liver cirrhosis. In the family all siblings of the affected individual need to be screened in order to treat any asymptomatic subjects. PMID:23909262

Mare?ek, Z; Br?ha, R

2013-07-01

429

Desmoid Disease  

Microsoft Academic Search

\\u000a Desmoid disease is an overgrowth of fibrous tissue that presents in patients with Familial Adenomatous Polyposis (FAP) as\\u000a a spectrum of lesions from flat, twodimensional sheets to large, rapidly growing, three-dimensional tumors. FAPrelated desmoid\\u000a tumors can be confused with sporadic desmoids that occur in the general population with an incidence of two to three per million\\u000a [1]. These sporadic desmoids

James M. Church

430

Kawasaki Disease  

Microsoft Academic Search

\\u000a Kawasaki disease (KD) is more common in Japanese population and those of Japanese descent. Incidence is higher in males than\\u000a females. Etiology of KD continues to be unclear, there is evidence supporting both genetic and environmental factors. Diagnosis\\u000a of KD is made when there is fever of at least 5 days duration, plus bilateral, nonpurulent conjunctivitis, polymorphous skin\\u000a rash, mucous

Rami Kharouf; Daniel E. Felten

431

Stargardt Disease  

Microsoft Academic Search

When the adenosine triphosphate (ATP)-binding cassette (ABC) transporter gene, ABCA4 (originally named ABCR), was cloned and characterized in 1997 as the causal gene for autosomal recessive Stargardt disease (arSTGD or STGD1) (1) it seemed as if just another missing link was added to the extensive table of genetic determinants of rare monogenic retinal\\u000a dystrophies. Now, 9 yr later, the ABCA4

Rando Allikmets

432

Sorghum Diseases.  

E-print Network

made for maximum use of available land. practices. 20 A 1 SELECTED REFERENCES DICKSON, JAMES G., 1956, Diseases of Field Crops, McGraw-Hill Book Company, Znc., New York, p. 517. EDMUNDS, L. K., 1963, Combined relation of plant maturity... of commercially grown grain sorghum and forage sorghum hybrids and varieties to downy mildew (Sclerospora sorghi). Texas Agricultural Experiment Station Progress Report-PR2629. FREDERIKSEN, R. A., D. T. ROSENOW, L. REYES, and L. K. EDMUNDS, 1969, A new race...

Amador, Jose; Berry, Robert W.; Frederiksen, Richard A.; Horne, C. Wendell; Thames, Walter H.; Toler, Robert W.

1969-01-01

433

[Liver diseases].  

PubMed

In Digestive Disease Week 2008, interesting advances in the treatment of hepatitis C were presented, especially related to the promising increase in the therapeutic efficacy of the combination of conventional treatment with the protease inhibitor, telaprevir. Also revealed was the influence of viral replication of hepatitis B virus in the development of complications, including hepatocarcinoma. In this sense, drugs such as entecavir and tenofovir seem to provide sustained inhibition of viral replication without the development of resistance. Among the complications of cirrhosis, the growing prognostic implications of bacterial infections, associated with an increased risk of death, have been demonstrated. Likewise, diverse studies have provided greater understanding of the role of elastography in the evaluation of liver fibrosis, including the role of this technique in the evaluation of treatment response and in liver transplantation. The importance of fatty acid liver disease was clearly shown in several studies confirming the influence of diabetes in the presence of inflammation and fibrosis and the increase in the indication of liver transplantation for this disease, which may recur in the graft, especially in patients with a high body mass index. A meta-analysis of hepatocarcinoma and a decision model were unable to detect differences in survival in small tumors treated with radiofrequency or surgery. PMID:19434874

Calleja, José Luis; Albillos, Agustín; Bańares, Rafael

2008-10-01

434

Celiac Disease National Digestive Diseases Information Clearinghouse  

E-print Network

Celiac Disease National Digestive Diseases Information Clearinghouse National Institute. This list is not complete, so people with celiac disease should discuss gluten-free food choices with a dietitian or physician who specializes in celiac disease. People with celiac disease should always read food

Rau, Don C.

435

Plant Disease Lesson: Rhizoctonia Diseases of Turfgrass  

NSDL National Science Digital Library

This plant disease lesson on Rhizoctonia diseases of turfgrass (caused by the fungi Rhizoctonia species) includes information on symptoms and signs, pathogen biology, disease cycle and epidemiology, disease management, and the significance of the disease. Selected references are listed and a glossary is also available for use with this resource.

Lane P. Tredway (University of Georgia, Athens; )

2001-11-09

436

Plant Disease Lesson: Lesion nematode disease  

NSDL National Science Digital Library

This plant disease lesson on Lesion nematode disease (caused by Pratylenchus) includes information on symptoms and signs, pathogen biology, disease cycle and epidemiology, disease management, and the significance of the disease. Selected references are listed and a glossary is also available for use with this resource.

Eric L. Davis (North Carolina State University; )

2000-10-30

437

Plant Disease Lesson: Soybean cyst nematode disease  

NSDL National Science Digital Library

This plant disease lesson on Soybean cyst nematode disease (caused by Heterodera glycines) includes information on symptoms and signs, pathogen biology, disease cycle and epidemiology, disease management, and the significance of the disease. Selected references are listed and a glossary is also available for use with this resource.

Eric L. Davis (North Carolina State University; )

2000-07-25

438

Plant Disease Lesson: Dutch elm disease  

NSDL National Science Digital Library

This plant disease lesson on Dutch elm disease (caused by the fungus Ophiostoma ulmi) includes information on symptoms and signs, pathogen biology, disease cycle and epidemiology, disease management, and the significance of the disease. Selected references are listed and a glossary is also available for use with this resource.

Cleora J. D'Arcy (University of Illinois; )

2000-07-21

439

A de novo monoclonal immunoglobulin deposition disease in a kidney transplant recipient: a case report  

PubMed Central

Introduction Myeloma following kidney transplantation is a rare entity. It can be divided into two groups: relapse of a previous myeloma and de novo myeloma. Some of these myelomas can be complicated by a monoclonal immunoglobulin deposition disease, which is even less common. Less than ten cases of monoclonal immunoglobulin deposition disease after renal graft have been reported in the literature. The treatment of these patients is not well codified. Case presentation We report the case of a 43-year-old white European man who received a renal transplant for a nephropathy of unknown etiology and developed a nephrotic syndrome with kidney failure at 2-years follow-up. We diagnosed a de novo monoclonal immunoglobulin deposition disease associated with a kappa light chain multiple myeloma, which is a very uncommon presentation for this disease. Three risk factors were identified in this patient: Epstein–Barr virus reactivation with cytomegalovirus co-infection; intensified immunosuppressive therapy during two previous rejection episodes; and human leukocyte antigen-B mismatches. Chemotherapy treatment and decrease in the immunosuppressive therapy were followed by remission and slight improvement of renal function. A relapse occurred 8 months later and his renal function worsened rapidly requiring hemodialysis. He died from septic shock 4 years after the diagnosis of monoclonal immunoglobulin deposition disease. Conclusions This rare case of post-transplant lymphoproliferative disorder with an uncommon presentation illustrates the fact that treatment in such a situation is very difficult to manage because of a small number of patients reported and a lack of information on this disease. There are no guidelines, especially concerning the immunosuppressive therapy management. PMID:24942882

2014-01-01

440

Peripheral neuropathy incidence in inflammatory bowel disease  

PubMed Central

Objective: Our aim was to determine the incidence of peripheral neuropathy in a population-based inflammatory bowel disease (IBD) cohort from Olmsted County, Minnesota. Methods: We retrospectively ascertained neuropathy incidence in a population-based cohort of adult persons newly diagnosed with IBD between 1940 and 2004 in Olmsted County, Minnesota, using the medical records linkage system of the Rochester Epidemiology Project. The Kaplan-Meier method was used to estimate the cumulative incidence of neuropathy. Results: A total of 772 Olmsted County residents aged 18 to 91 years were diagnosed with IBD. After 12,476 person-years, 9 patients developed neuropathy, providing an overall incidence rate of 72 (95% confidence interval [CI] 33–137) cases per 100,000 IBD person-years. The cumulative incidence rates after 10, 20, and 30 years were 0.7% (95% CI 0.0%–1.3%), 0.7% (95% CI 0.0%–1.5%), and 2.4% (95% CI 0.6%–4.6%), respectively. Neuropathy was diagnosed after 1 to 44 years from IBD onset. Only 2 patients had active bowel disease at the time of neuropathy onset. The clinical spectrum consisted of 1) monophasic immune radiculoplexus neuropathy (comorbid diabetes in 2 of 4 patients) and 2) chronic distal sensorimotor polyneuropathy (comorbid diabetes in 2 of 5 patients). Conclusions: Our population-based study suggests that neuropathy is uncommon in the patient population of IBD. Radiculoplexus neuropathy and sensorimotor polyneuropathy were both observed, commonly during periods of bowel disease inactivity. Clinicians should consider other etiologies of neuropathy in patients with IBD. PMID:23576624

Loftus, Edward V.; Harmsen, William S.; Dyck, P. James B.; Klein, Christopher J.

2013-01-01

441

Activated protein C resistance, thrombophilia, and inflammatory bowel disease.  

PubMed

Thromboembolic events frequently complicate the clinical course of patients with inflammatory bowel disease (IBD). Hereditary thrombophilia may contribute to this tendency. Resistance to activated protein C is the most recently described thrombophilic state and may account for up to 40% of patients with thrombophilia. Thirty-seven patients with IBD were studied (mean age 44 years, range 18-82 years). Three patients had a history of thrombotic episodes. The 37 controls included 23 men and 17 women (mean age 48 years, range 16-89 years). Disease activity was assessed using the Harvey Bradshaw index for patients with Crohn's disease and the Truelove and Witts grading system for patients with ulcerative colitis. Levels of fibrinogen, antithrombin III (ATIII), protein C, protein S, activated protein C resistance (APCR), and the presence of a lupus anticoagulant (LA) were determined. Median ATIII levels in patients with IBD were significantly lower than controls (98% vs 106%, P = 0.007), while fibrinogen was elevated (4.2 vs 3.3 g/liter, P = 0.026) despite quiescent disease activity. LA was detected in 7/37 patients in the IBD group compared to 0/37 controls. (chi2 = 5.68, P = 0.017). No significant difference was observed in levels of inherited thrombophilic factors and in particular APCR between IBD patients and controls. In conclusion, the presence of inherited thrombophilic defects, in particular APCR, is uncommon in patients with IBD and does not merit routine screening. PMID:9635631

Heneghan, M A; Cleary, B; Murray, M; O'Gorman, T A; McCarthy, C F

1998-06-01

442

Systemic Granulomatous Diseases Associated with Multiple Palpable Masses That May Involve the Breast: Case Presentation and an Approach to the Differential Diagnosis  

PubMed Central

Palpable mass is a common complaint presented to the breast surgeon. It is very uncommon for patients to report breast mass associated with palpable masses in other superficial structures. When these masses are related to systemic granulomatous diseases, the diagnosis and initiation of specific therapy can be challenging. The purpose of this paper is to report a case initially assessed by the breast surgeon and ultimately diagnosed as granulomatous variant of T-cell lymphoma, and discuss the main systemic granulomatous diseases associated with palpable masses involving the breast. PMID:25342951

Jales, Rodrigo Menezes; Pereira, Patrick Nunes; Stelini, Rafael Fantelli; Moro, Luciano

2014-01-01

443

Circular Abscess Formation of the Inner Preputial Leaf as a Complication of a Penile Mondor's Disease: The First Case Report  

PubMed Central

Introduction. Mondor's disease of the penis is an uncommon condition characterized by thrombosis or thrombophlebitis involving the superficial dorsal veins. An accompanied lymphangitis is discussed. There is typical self-limiting clinical course. Case Presentation. This paper firstly reports a secondary abscess formation of the preputial leaf two weeks after penile Mondor's disease and subcutaneous lymphangitis as complication of excessive sexual intercourse of a 44-year-old man. Sexual transmitted diseases could be excluded. Lesions healed up completely under abscess drainage, antibiotic, and anti-inflammatory medication. Conclusion. Previous reports in the literature include several entities of the penile Mondor's disease. Our patient is very unusual in that he presented with a secondary preputial abscess formation due to superficial thrombophlebitis, subcutaneous lymphangitis, and local bacterial colonisation. Abscess drainage plus antiphlogistic and antibiotic medication is the treatment of choice. PMID:24868480

Wendler, Johann Jakob; Schindele, Daniel; Liehr, Uwe-Bernd; Porsch, Markus; Schostak, Martin

2014-01-01

444

Maintenance with rituximab is safe and not associated with severe or uncommon infections in patients with follicular lymphoma: results from the phase IIIb MAXIMA study.  

PubMed

Previous randomized trials have demonstrated that rituximab maintenance (R-maintenance) can prolong time to progressive disease in patients with follicular lymphoma (FL). The phase IIIb MAXIMA study (NCT00430352) was a large prospective evaluation of R-maintenance in a daily care setting. The primary objective was safety. Secondary objectives included progression-free survival, overall survival, time to next lymphoma treatment, and partial response (PR) to complete response/unconfirmed (CR/CRu) conversion rate. Patients (n = 545) with first-line or relapsed FL who responded to 8 cycles of rituximab-based induction received R-maintenance every 2 months for 2 years. At study entry, 380 patients had CR or CRu, and 165 had PR. The median age was 57.0 years. The most common non-hematologic adverse events (AEs, excluding infusion-related reactions) were cough (9.9 % of patients), fatigue (7.5 %), nasopharyngitis (7.1 %), back pain (6.5 %), diarrhea (6.9 %), arthralgia (6.0 %), headache and hypertension (5.2 % each), and pyrexia (5.1 %). The majority of AEs were grade 1 or 2. Grade 3, 4, and 5 infections occurred in 21 (3.9 %), 2 (0.4 %), and 1 (0.2 %) patient, respectively. Fifty-one hematologic AEs occurred in 6.6 % (n = 35) of patients. Grade 3/4 prolonged neutropenia and hypogammaglobulinemia occurred in 13 (2.4 %) and 5 (0.9 %) patients, respectively. All cases of prolonged neutropenia or hypogammaglobulinemia were manageable and resolved. Fast infusion did not alter the safety profile. Efficacy was comparable with results from previous trials. R-maintenance is safe in a daily care setting for patients with first-line or relapsed FL. PMID:24824768

Witzens-Harig, Mathias; Foá, Robin; Di Rocco, Alice; van Hazel, Guy; Chamone, Dalton F A; Rowe, Jacob M; Arcaini, Luca; Poddubnaya, Irina; Ho, Anthony D; Ivanova, Valentina; Vranovsky, Andrej; Thurley, Dan; Oertel, Stephan

2014-10-01

445

Parkinson's Disease  

NSDL National Science Digital Library

This patient education program discusses the symptoms, and treatment options for Parkinson's Disease, including their benefits and side effects. It also reviews the anatomy of the central nervous system. This resource is a MedlinePlus Interactive Health Tutorial from the National Library of Medicine, designed and developed by the Patient Education Institute. NOTE: This tutorial requires a special Flash plug-in, version 4 or above. If you do not have Flash, you will be prompted to obtain a free download of the software before you start the tutorial. You will also need an Acrobat Reader, available as a free download, in order to view the Reference Summary.

Patient Education Institute

446

Infectious disease.  

PubMed

Athletes are susceptible to the same infections as the general population. However, special considerations often need to be taken into account when dealing with an athlete who has contracted an infectious disease. Health care providers need to consider how even common illnesses can affect an athlete's performance, the communicability of the illness to team members, and precautions/contraindications related to athletic participation. Recent advances in the prevention, diagnosis, and/or management of frequently encountered illnesses, as well as certain conditions that warrant special attention in the athletic setting, are discussed in detail. PMID:21658549

Jaworski, Carrie A; Donohue, Brian; Kluetz, Joshua

2011-07-01

447

Celiac Disease  

PubMed Central

Purpose of review To summarize recent advances in celiac disease (CD) published between August 2008 and July 2009. Recent findings CD affects ~1% of most populations but remains largely unrecognized. In the last year, work has shown that the prevalence of CD has increased dramatically, not simply due to increased detection. Also, undiagnosed CD may be associated with increased mortality. Significant progress has been made in understanding how gliadin peptides can cross the intestinal border and access the immune system. New genetic loci and candidate genes that may contribute to the risk of CD and its overlap with type 1 diabetes mellitus have been identified. New deamidated gliadin peptides antibodies have better diagnostic accuracy over native gliadin-based tests. The inclusion of duodenal bulb biopsy specimens may increase the rate of CD detection. The spectrum of CD likely includes a minority of patients with mild enteropathy. A practical 7-item instrument may facilitate standardized evaluation of gluten-free diet adherence. Finally, refractory CD, whilst rare, is associated with a poor prognosis. Summary Celiac disease is a global health problem that requires a multidisciplinary and increasingly cooperative multinational research effort. PMID:20040864

Rubio-Tapia, Alberto; Murray, Joseph A

2010-01-01

448

[Morton's disease].  

PubMed

Morton's disease refers to neuralgia at the web space of the toes with a pseudo-neuroma. It commonly occurs in the third web space of the foot in middle-aged and older women. The pseudo-neuroma is thought to be a secondary change after entrapment or repeated microtrauma. Patients complain of forefoot pain while walking. Typically, symptoms are caused by tight high-heeled shoes. The physical examination includes palpation of the web spaces and Mulder's test. Weight bearing foot radiographs are used to evaluate the deformity of the foot, especially at metatarsophalangeal (MTP) joints. MRI is useful for differential diagnosis of pseudo-neuroma, MTP joint arthritis, and interdigital bursitis. Conservative treatments are shoe modification, use of orthotic insoles, and injection of corticosteroids and local anesthesia. The injections are useful not only for the treatment but also for diagnosis of Morton's disease. If the local injection is not temporally effective, surgical treatment is not indicated. If the conservative treatment fails, surgical treatment is indicated. The most common surgery is excision of the pseudo-neuroma. The surgery is usually performed using a dorsal approach. PMID:25475032

Isomoto, Shinji; Tanaka, Yasuhito

2014-12-01

449

Adenotonsillar disease.  

PubMed

Adenotonsillar disease (adenoiditis and recurrent tonsillitis) is a prevalent otolaryngologic disorder aetiologically based on chronic inflammation triggered by a persistent bacterial infection. These bacteria, mostly Staphylococcus aureus, Haemophilus sp., and Streptococcus sp., persist predominantly intracellular and within mucosal biofilms. The recurrent or chronic inflammation of the adenoids and faucial tonsils leads to chronic activation of the cell-mediated and humoral immune response, resulting in hypertrophy of the lymphoid tonsillar tissue. This hypertrophic tissue is the cause for the prominent clinical symptoms: obstruction of the upper airways, snoring, and sleep apnea for adenoiditis or sore throat, dysphagia and halitosis for recurrent tonsillitis. Treatment strategies should target the persisting bacteria within their biofilm or intracellular shelter. Macrolide antibiotics like clarithromycin are able to modulate the immune system and to interfere in bacterial signaling within biofilms. Clindamycin, quinupristin-dalfopristin, and oritavancin are intracellular high active compounds. Surgical removal of the hypertrophic tissue by modern procedures like laser tonsil ablation, eliminates not only a mechanical obstacle of the airways, it removes also the basis for the aetiologic cause, the "biofilm carrier". This review summarizes the role of bacterial persistence in mucosal biofilms for the aetiology, diagnosis and treatment of adenotonsillar disease and relevant patents. PMID:22452646

Zautner, Andreas E

2012-05-01

450

Chagas' disease.  

PubMed Central

Chagas' disease, caused by Trypanosoma cruzi, is an important cause of morbidity in many countries in Latin America. The important modes of transmission are by the bite of the reduviid bug and blood transfusion. The organism exists in three morphological forms: trypomastigotes, amastigotes, and epimastigotes. The mechanism of transformation and differentiation is currently being explored, and signal transduction pathways of the parasites may be involved in this process. Parasite adherence to and invasion of host cells is a complex process involving complement, phospholipase, penetrin, neuraminidase, and hemolysin. Two clinical forms of the disease are recognized, acute and chronic. During the acute stage pathological damage is related to the presence of the parasite, whereas in the chronic stage few parasites are found. In recent years the roles of tumor necrosis factor, gamma interferon, and the interleukins in the pathogenesis of this infection have been reported. The common manifestations of chronic cardiomyopathy are arrhythmias and thromboembolic events. Autoimmune, neurogenic, and microvascular factors may be important in the pathogenesis of the cardiomyopathy. The gastrointestinal tract is another important target, and "mega syndromes" are common manifestations. The diagnosis and treatment of this infection are active areas of investigation. New serological and molecular biological techniques have improved the diagnosis of chronic infection. Exacerbations of T. cruzi infection have been reported for patients receiving immuno-suppressive therapy and for those with AIDS. Images PMID:1423218

Tanowitz, H B; Kirchhoff, L V; Simon, D; Morris, S A; Weiss, L M; Wittner, M

1992-01-01

451

Riga-Fede disease and neonatal teeth  

PubMed Central

SUMMARY Aim The aim of this study is to present a case of Riga-Fede disease (RFD). RFD is a benign and uncommon mucosal disorder, characterized by an ulceration of the tongue, often caused by repetitive traumatic injuries due to backward and forward movements of the tongue over the mandibular anterior incisors. RFD is most commonly associated with the eruption of primary lower incisor in older infants or natal-neonatal teeth in newborns. Methods A 2-month-old female infant was referred to our Paediatric Dentistry Unit for ulceration (13 mm diameter) on the ventral surface of the tongue and neonatal teeth. The extraction of neonatal teeth was selected as treatment of choice, over more conservative treatments, for the rapid resolution of the lesion and for the limited risk of inadequate nutrients intake. The extracted teeth underwent a macroscopic/microscopic examination. The complete healing of the lesion took 4 weeks; subsequently, the infant, revised at the 1-year follow-up visit. Conclusion Early detection of RFD is recommended because such lesions properly may induce deformity or mutilation of tongue, dehydration, inadequate nutrients intake by the infant and growth retardation. PMID:23285403

COSTACURTA, M.; MATURO, P.; DOCIMO, R.

2012-01-01

452

Ultrasound in patients affected with Peyronie's disease.  

PubMed

Peyronie's disease (PD, induratio penis plastica) is uncommon. Its etiology is unknown and the incidence is stated to be 1%, although more recent data suggest that it is higher. The symptoms are penile deviation and painful erection in association with penile plaques. The diagnosis is performed by palpation of the penis. X-ray diagnostics are only used to visualize the calcified plaques in soft tissue imaging, and the survey of plaque size and consistence is done by sonography with high reproduction. This enables the exact evaluation of treatment effects. By application of high frequency scanners (7-12 MHz) the extent and depth of the plaques can be estimated non-invasively in high resolution images. Furthermore, color coded duplex sonography and recording of Doppler spectra are used to assess hemo-perfusion in the penile vessels and the analysis of erectile dysfunction, often seen in combination with PD. Sonographic diagnosis appears as a valuable supplement and requirement for a successful therapy of PD. PMID:15672280

Fornara, P; Gerbershagen, H-P

2004-11-01

453

Kidney Disease Basics  

MedlinePLUS

... Disease Children and Kidney Disease Additional Kidney Information Kidney Disease Basics Your kidneys filter extra water and ... blood pressure are the most common causes of kidney disease. These conditions can slowly damage the kidneys ...

454

Depression and Heart Disease  

MedlinePLUS

... see the NIMH booklet on Depression . What is heart disease? Heart disease refers to a number of ... and save your life. How are depression and heart disease linked? People with heart disease are more ...

455

Motor Neuron Diseases  

MedlinePLUS

NINDS Motor Neuron Diseases Information Page Condensed from Motor Neuron Diseases Fact Sheet Table of Contents (click to jump to ... is being done? Clinical Trials Organizations What are Motor Neuron Diseases? The motor neuron diseases (MNDs) are a ...

456

Parkinson's Disease Dementia  

MedlinePLUS

Parkinson's Disease Dementia Tweet Parkinson's disease dementia is an impairment in thinking and reasoning that eventually affects many people with Parkinson's ... Symptoms Diagnosis Causes & risks Treatments About Parkinson's disease dementia The brain changes caused by Parkinson's disease begin ...

457

About Alzheimer's Disease: Symptoms  

MedlinePLUS

... more about other early signs of Alzheimer's » Mild Alzheimer's disease As the disease progresses, memory loss worsens, and ... disease is often diagnosed at this stage. Moderate Alzheimer's disease In this stage, damage occurs in areas of ...

458

Dejerine-Sottas Disease  

MedlinePLUS

... Progress Search form Search Charcot-Marie-Tooth Disease (CMT) Dejerine-Sottas disease What is Dejerine-Sottas disease? ... is a subtype of Charcot-Marie-Tooth disease (CMT), a genetic, neurological disorder that causes damage to ...

459

Testing for Kidney Disease  

MedlinePLUS

... and Kidney Disease Additional Kidney Information Testing for Kidney Disease Early kidney disease usually does not have signs ( ... how often you should be tested. Testing for Kidney Disease Overview Understanding GFR Understanding Urine Albumin Videos Testing ...

460

Machado-Joseph Disease  

MedlinePLUS

NINDS Machado-Joseph Disease Information Page Condensed from Machado-Joseph Disease Fact Sheet Table of Contents (click to jump ... is being done? Clinical Trials Organizations What is Machado-Joseph Disease? Machado-Joseph disease (MJD), which is ...

461

Poorly Responsive Celiac Disease  

MedlinePLUS

... Sources of Gluten . Microscopic Colitis, Crohn’s Disease and Ulcerative Colitis People with celiac disease have an increased incidence ... colitis and inflammatory bowel disease (Crohn’s disease and ulcerative colitis). Microscopic colitis is an inflammation of the colon, ...

462

Learning about Crohn's Disease  

MedlinePLUS

... Dietary Treatment of Crohn's Disease Immune Regulation in Ulcerative Colitis or Crohn's Disease Current NHGRI Clinical Studies Search ... to finding the cure for Crohn's disease and ulcerative colitis. Crohn's Disease and Ulcerative Colitis: A Parent's Guide [ ...

463

Mitochondrial disease  

Microsoft Academic Search

Opinion statement  \\u000a \\u000a \\u000a \\u000a \\u000a – \\u000a \\u000a •Mitochondrial diseases are disorders of energy metabolism that include defects of pyruvate metabolism, Krebs cycle, respiratory\\u000a chain (RC), and fatty acid oxidation (FAO).\\u000a \\u000a \\u000a \\u000a \\u000a – \\u000a \\u000a •Treatment of pyruvate metabolism, Krebs cycle, and RC disorders is, in general, disappointing. Therapeutic approaches consist\\u000a of electron acceptors, enzyme activators, vitamins, coenzymes, free-radical scavengers, dietary measures, and supportive therapy.\\u000a These treatment assumptions

Roser Pons; Darryl C. De Vivo

2001-01-01

464

Alzheimer disease  

PubMed Central

Alzheimer disease (AD) has traditionally been thought to involve the misfolding and aggregation of two different factors that contribute in parallel to pathogenesis: amyloid-? (A?) peptides, which represent proteolytic fragments of the transmembrane amyloid precursor protein, and tau, which normally functions as a neuronally enriched, microtubule-associated protein that predominantly accumulates in axons. Recent evidence has challenged this model, however, by revealing numerous functional interactions between A? and tau in the context of pathogenic mechanisms for AD. Moreover, the propagation of toxic, misfolded A? and tau bears a striking resemblance to the propagation of toxic, misfolded forms of the canonical prion protein, PrP, and misfolded A? has been shown to induce tau misfolding in vitro through direct, intermolecular interaction. In this review we discuss evidence for the prion-like properties of both A? and tau individually, as well as the intriguing possibility that misfolded A? acts as a template for tau misfolding in vivo. PMID:22965142

Nussbaum, Justin M.; Seward, Matthew E.; Bloom, George S.

2013-01-01

465

Vibroacoustic disease.  

PubMed

Vibroacoustic disease (VAD) is a whole-body, systemic pathology, characterized by the abnormal proliferation of extra-cellular matrices, and caused by excessive exposure to low frequency noise (LFN). VAD has been observed in LFN-exposed professionals, such as, aircraft technicians, commercial and military pilots and cabin crewmembers, ship machinists, restaurant workers, and disk-jockeys. VAD has also been observed in several populations exposed to environmental LFN. This report summarizes what is known to date on VAD, LFN-induced pathology, and related issues. In 1987, the first autopsy of a deceased VAD patient was performed. The extent of LFN induced damage was overwhelming, and the information obtained is, still today, guiding many of the associated and ongoing research projects. In 1992, LFN-exposed animal models began to be studied in order to gain a deeper knowledge of how tissues respond to this acoustic stressor. In both human and animal models, LFN exposure causes thickening of cardiovascular structures. Indeed, pericardial thickening with no inflammatory process, and in the absence of diastolic dysfunction, is the hallmark of VAD. Depressions, increased irritability and aggressiveness, a tendency for isolation, and decreased cognitive skills are all part of the clinical picture of VAD. LFN is a demonstrated genotoxic agent, inducing an increased frequency of sister chromatid exchanges in both human and animal models. The occurrence of malignancies among LFN-exposed humans, and of metaplastic and displastic appearances in LFN-exposed animals, clearly corroborates the mutagenic outcome of LFN exposure. The inadequacy of currently established legislation regarding noise assessments is a powerful hindrance to scientific advancement. VAD can never be fully recognized as an occupational and environmental pathology unless the agent of disease--LFN--is acknowledged and properly evaluated. The worldwide suffering of LFN-exposed individuals is staggering and it is unethical to maintain this status quo. PMID:15273020

Branco, N A A Castelo; Alves-Pereira, M

2004-01-01

466

Alzheimer's disease: strategies for disease modification  

Microsoft Academic Search

Alzheimer's disease is the largest unmet medical need in neurology. Current drugs improve symptoms, but do not have profound disease-modifying effects. However, in recent years, several approaches aimed at inhibiting disease progression have advanced to clinical trials. Among these, strategies targeting the production and clearance of the amyloid-? peptide — a cardinal feature of Alzheimer's disease that is thought to

Martin Citron

2010-01-01

467

Myeloproliferative disease in a cat  

Microsoft Academic Search

Myeloproliferative disorders, a complex of cytologic abnormalities arising in the bone marrow, are among domestic animals most frequently recognized in cats but are relatively uncommon. A 4-year-old female Siamese, with splenomegaly and weight loss, was listless, anorectic, pale and dehydrated. A hemogram showed severe, macrocytic normochromic anemia, leukocytosis and reticulocytosis, with abnormally high numbers of nucleated RBC and undifferentiated blast

R. W. Yates; R. E. Weller; B. F. Feldman

1984-01-01

468

[Immunoglobulin G4 (IgG4)-related disease. A review of head and neck manifestations].  

PubMed

Immunoglobulin G4 (IgG4)-related disease (also known as hyper-IgG4 disease) is a recently defined emerging condition with highly heterogeneous clinicopathological features and variable disease manifestations. This disorder is characterized by unifocal or multifocal (multiorgan) involvement by tumefactive plasma cell-rich inflammatory infiltrates associated with prominent fibrosclerosis. This not uncommonly interferes with organ function resulting in diverse clinical symptoms. The autoimmune pancreatitis represents the prototype of this disease; however, to date almost all organs have been reported to be involved in this disorder. In the head and neck area several presentations of this disease may be encountered in salivary glands, lacrimal glands, thyroid gland, lymph nodes, soft tissue of the neck, ear and sinonasal tract. However, IgG4 positive plasma cells are occasionally prominent in non-specific chronic inflammatory conditions of the head and neck and the oral cavity unrelated to autoimmune diseases or systemic disorders, thus representing diagnostic pitfalls. The diagnosis of IgG4-related disease should be based on a combination of typical histological, clinical and serological findings. PMID:24619525

Agaimy, A; Ihrler, S

2014-03-01

469

Fox-Fordyce-like disease following laser hair removal appearing on all treated areas.  

PubMed

Fox-Fordyce disease is an uncommon inflammatory disease of the apocrine sweat glands. Two recent reports indicated laser hair removal as a novel cause of axillary Fox-Fordyce disease. We report the first case of Fox-Fordyce disease developing in women after completing treatment with a depilatory hair laser appearing in the axillae, umbilicus, and pubis. We describe a case of Fox-Fordyce disease that developed in a 27-year-old woman 3 months after she had completed two LightSheer Diode laser treatments of her axilla, periumbilical region, and bikini area. Clinical and histopathological changes are as well detailed. Laser therapy induces damage to follicular infundibulum, resulting in altered maturation of keratinocytes which led to keratin plugging causing the common pathologic features in Fox-Fordyce disease. Differences in the physiologic features of the anatomic sites, in the susceptibility to laser-induced injury among these areas, or additional factors may contribute to Fox-Fordyce disease. PMID:23318918

Helou, Josiane; Maatouk, Ismaël; Moutran, Roy; Obeid, Grace

2013-07-01

470

Acute tubulointerstitial nephritis complicating Legionnaires' disease: a case report  

PubMed Central

Introduction Legionnaires' disease is recognized as a multi-systemic illness. Afflicted patients may have pulmonary, renal, gastrointestinal tract and central nervous system complications. However, renal insufficiency is uncommon. The spectrum of renal involvement may range from a mild and transient elevation of serum creatinine levels to anuric renal failure requiring dialysis and may be linked to several causes. In our present case report, we would like to draw attention to the importance of the pathological documentation of acute renal failure by reporting a case of a patient with acute tubulointerstitial nephritis complicating Legionnaires' disease. Case presentation A 55-year-old Caucasian man was admitted to our hospital for community-acquired pneumonia complicated by acute renal failure. Legionella pneumophila serogroup type 1 was diagnosed. Although the patient's respiratory illness responded to intravenous erythromycin and ofloxacin therapy, his renal failure worsened, he became anuric, and hemodialysis was started. A renal biopsy was performed, which revealed severe tubulointerstitial nephritis. After initiation of steroid therapy, his renal function improved dramatically. Conclusions This case highlights the importance of kidney biopsies in cases where acute renal failure is a complicating factor in Legionnaires' disease. If the presence of acute tubulointerstitial nephritis can be confirmed, it will likely respond favorably to steroidal treatment and thus irreversible renal damage and chronic renal failure will be avoided. PMID:22475340

2012-01-01

471

Genetic testing for Alzheimer disease. Practical and ethical issues.  

PubMed

The dissection of the heterogeneous genetics of the Alzheimer diseases (ADs) is currently more advanced than in any other common disease. Not only are 3 uncommon autosomal dominant mutation loci identified, but universally inherited susceptibility polymorphisms associated with risk and age of onset distributions for familial and "sporadic" AD are also confirmed. The utility of testing for mutations of the amyloid precursor protein and the presenilin 1 and presenilin 2 genes conforms to strategies in common use for rare mutations. The selection of patients with very early-onset AD, especially those with family histories of the disease, will increase the possibility of diagnosis. All testing should be performed using recommended counseling procedures. Apolipoprotein E (ApoE) susceptibility polymorphisms are genetic risk factors but do not allow prediction of the age of onset of AD for cognitively normal individuals. Recent large, collaborative studies have found that when ApoE genotyping is used sequentially in diagnosis following criteria-based evaluations, the specificity of early diagnosis is significantly increased. For patients clinically diagnosed with AD who carried an ApoE epsilon 4 allele, the positive predictive value was 94% and 97% in 2 multicenter collaborative series. The utility of ApoE genotyping is reviewed and recommendations for early use in diagnosis are explained. There are ethical, social, actuarial, and legal problems currently associated with genetic testing and these concerns are also discussed. PMID:9341568

Roses, A D

1997-10-01

472

Imaging of the porta hepatis: spectrum of disease.  

PubMed

A wide array of pathologic conditions can arise within the porta hepatis, which encompasses the portal triad (the main portal vein, common hepatic artery, and common bile ducts), lymphatics, nerves, and connective tissue. Major vascular diseases of the portal triad include thrombosis, stenosis, and aneurysm. Portal vein thrombosis can complicate liver cirrhosis and hepatocellular carcinoma and has important therapeutic implications. Hepatic artery thrombosis and stenosis require immediate attention to reduce graft loss in liver transplant recipients. Congenital (eg, choledochal cyst) and acquired (benign and malignant) diseases of the biliary system can manifest as mass lesions in the porta hepatis. Lymphadenopathy can arise from neoplastic and nonneoplastic entities. Uncommon causes of mass lesions arise from nerves (eg, neurofibroma, neurofibrosarcoma) and connective tissue (sarcomas) and are rare. The hepatoduodenal ligament is a peritoneal reflection at the porta hepatis and is an important route for the spread of pancreatic and gastrointestinal cancers. Imaging plays a major role in diagnosis and enables appropriate management. Ultrasonography accurately demonstrates anatomic variations and pathologic conditions and is the initial modality of choice for detection of vascular and biliary lesions. Multidetector computed tomography and magnetic resonance imaging allow characterization and differentiation of various masses in the porta hepatis. Imaging-guided interventions, including embolization and stent placement, also play a key role in disease management. PMID:24428283

Tirumani, Sree Harsha; Shanbhogue, Alampady Krishna Prasad; Vikram, Raghunandan; Prasad, Srinivasa R; Menias, Christine O

2014-01-01

473

Diet and skin disease in dogs and cats.  

PubMed

Dietary factors have a major role in the maintenance of healthy coat and skin, and are significant in the etiology and therapy of certain skin diseases. Nutritional deficiencies are now uncommon as a result of the widespread feeding of complete and balanced pet foods. Deficiencies of (n-6) polyunsaturated fatty acids, zinc and vitamins, however, do arise in certain animal- or product-related instances. Supraphysiologic doses of vitamin A have been used in the management of vitamin A-responsive dermatosis in Cocker spaniels; other keratinization defects and seborrheic conditions may respond to retinoid therapy. Much interest has been paid to the therapeutic value of polyunsaturated fatty acid supplements in the management of dermatologic conditions associated with hypersensitivity reactions or keratinization defects. These studies have generally yielded disappointing results, which may reflect shortcomings in the design of some trials. Nevertheless, a placebo-controlled, double-blind, cross-over study has demonstrated a clear benefit of high dose (n-3) fatty acids in the management of pruritic skin disease. There is also preliminary experimental evidence that specific dietary (n-6):(n-3) fatty ratios are useful in the dietary management of inflammatory diseases. Although results of controlled clinical trials are awaited, the argument exists that it is the absolute amount of (n-3) fatty acid intake rather than ratio that is responsible for potential health benefits. PMID:9868266

Watson, T D

1998-12-01

474

Biological therapy for dermatological manifestations of inflammatory bowel disease  

PubMed Central

Ulcerative colitis and Crohn’s disease are the two forms of inflammatory bowel disease (IBD). The advent of biological drugs has significantly changed the management of these conditions. Skin manifestations are not uncommon in IBD. Among the reactive lesions (immune-mediated extraintestinal manifestations), erythema nodosum (EN) and pyoderma gangrenosum (PG) are the two major cutaneous ills associated with IBD, while psoriasis is the dermatological comorbidity disease observed more often. In particular, in the last few years, anti-tumor necrosis factor (TNF)-? agents have been successfully used to treat psoriasis, especially these kinds of lesions that may occur during the treatment with biological therapies. The entity of the paradoxical manifestations has been relatively under reported as most lesions are limited and a causal relationship with the treatment is often poorly understood. The reason for this apparent side-effect of the therapy still remains unclear. Although side effects may occur, their clinical benefits are undoubted. This article reviews the therapeutic effects of the two most widely used anti-TNF-? molecules, infliximab (a fusion protein dimer of the human TNF-? receptor) and adalimumab (a fully human monoclonal antibody to TNF-?), for the treatment of the major cutaneous manifestations associated with IBD (EN, PG and psoriasis). PMID:24303470

Zippi, Maddalena; Pica, Roberta; De Nitto, Daniela; Paoluzi, Paolo

2013-01-01

475

Cushing's disease.  

PubMed

Cushing's disease (CD) is caused by a pituitary tumor secreting corticotropin (ACTH), leading to cortisol excess. Patients with CD comprise approximately 70% of patients with endogenous Cushing's syndrome (CS). If the diagnosis and treatment are delayed, patients with CD may suffer the deleterious consequences of hypercortisolism, leading to significant morbidity and mortality. The diagnosis of CD is complex and includes two separate steps: establishing the presence of pathologic hypercortisolism and identifying the underlying cause. The management of CD usually includes transsphenoidal pituitary surgery (TSS) as the primary form of therapy. Pituitary surgery by the most experienced surgeons leads to remission of CD in 70-90% of patients. Recurrence of CD may occur in up to 25% of patients on long-term follow-up. Patients with recurrent CD may be treated with repeat TSS, radiation therapy to the pituitary with interim medical therapy (including steroidogenesis inhibitors, centrally acting agents or a glucocorticoid receptor antagonist) or bilateral adrenalectomy. Improvements in diagnosis and management of CD have led to higher patient survival. However, quality of life is impaired on long-term follow-up in some patients, even those in remission. Better understanding of the pathogenesis of CD may lead to the development of more effective therapies. PMID:25248590

Tritos, Nicholas A; Biller, Beverly M K

2014-01-01

476

Kimura's Disease  

PubMed Central

Kimuras disease is a chronic inflammatory disorder of unknown etiology. It is rare in the West, but endemic in Asia. It typically presents as solitary or multiple subcutaneous nodules, that slowly increase in size. The lesions are variably painful and pruritic. It often accompanied by regional lymphadenopathy, raised serum eosinophil counts, and markedly elevated serum immunoglobulin E levels. Histologically, the lesions are characterized by reactive lymphoid follicles with eosinophilic infiltration and an increased amount of postcapillary venules. The optimal treatment for KD remains controversial. Although the condition seldom resolves spontaneously, malignant transformation has not been reported to date, and the prognosis is good. We describe a male patient with a 4-year pruritic progressive “bump” in front of his left ear. On physical examination, the patient had 2 discrete lesions on the left side of his face near his ear. Postauricularly, there was a 3 × 5 cm erythematous to violaceous, indurated nodule. Preauricularly, there was a similar, but smaller cyst-like nodule. Punch biopsy showed a superficial and deep nodular and interstitial infiltrate, reactive lymphoid follicles with a dense infiltration of eosinophils and areas of eosinophilic follicle lysis. The patient received intralesional triamcinolone acetonide injections 10 mg/cc behind left ear with a good improvement. PMID:19718429

Guimaraes, Cláudia Savassi; Moulton-Levy, Natalie; Sapadin, Allen; Vidal, Claudia

2009-01-01

477

Alzheimer disease: An interactome of many diseases  

PubMed Central

Alzheimer Disease (AD) is an outcome as well as source of many diseases. Alzheimer is linked with many other diseases like Diabetes type 2, cholesterolemia, hypertension and many more. But how each of these diseases affecting other is still unknown to scientific community. Signaling Pathways of one disease is interlinked with other disease. But to what extent healthy brain is affected when any signaling in human body is disturbed is the question that matters. There is a need of Pathway analysis, Protein-Protein interaction (PPI) and the conserved interactome study in AD and linked diseases. It will be helpful in finding the potent drug or vaccine target in conscious manner. In the present research the Protein-Protein interaction of all the proteins involved in Alzheimer Disease is analyzed using ViSANT and osprey tools and pathway analysis further reveals the significant genes/proteins linking AD with other diseases. PMID:24753659

Rao, Balaji S.; Gupta, Krishna Kant; Karanam, Pujitha; Peruri, Anusha

2014-01-01

478

Coeliac disease among children in Kuwait: difficulties in diagnosis and management.  

PubMed Central

Twenty children with coeliac disease were diagnosed over a five year period in an area with 10,000-12,000 births per year. The average annual incidence was 1:3000 births. All children presented with severe symptoms and rickets was not uncommon (25%). Mean age at onset of symptoms was 38 months (range 6-120) and 72 months at the time of diagnosis (range 13-192), with a mean delay of 34 months. No cases were diagnosed during infancy. The difficulties in the differentiation of coeliac disease from the more common causes of chronic diarrhoea, and problems with diagnosis and management are discussed. Images Fig. 1(a) Fig. 1(b) Fig. 1(c) PMID:3428686

Khuffash, F A; Barakat, M H; Shaltout, A A; Farwana, S S; Adnani, M S; Tungekar, M F

1987-01-01

479

Wilson's disease: A Clinical autopsy case report with review of literature  

PubMed Central

Wilson's disease is an autosomal recessive disease resulting in defective copper metabolism, which is usually seen in young adults, predominantly affecting liver and brain. Although it is not uncommon in India, variation in epidemiology, clinical presentation and course are reported. However, community-based incidence and prevalence rates are not available in India and incidences are limited to hospital based reports. Most often, the diagnosis is delayed. We present a clinical autopsy case in a 39 year-old female who had presented with clinical symptoms at 18 years of age. The duration of illness was 21 years. Patient's parent had consanguineous marriage and the younger sibling had died at 5 years of age with similar complaints.

Raju, Kalyani; Bangalore, Gayathri Nagaraj; Thuruvekere, Suresh Nagaraj; Pathavanalli, Venkatarathnamma Narayanappa

2015-01-01

480

Management of a pregnant patient with Graves' disease complicated by propylthiouracil induced agranulocytosis.  

PubMed

Relapse and exacerbation of Graves' disease during pregnancy is rare, and thionamide induced agranulocytosis is an uncommon side effect. We report a case of a pregnant woman in her 24th week of gestation that experienced a relapse of Graves' disease that was complicated by propylthiouracil induced agranulocytosis. Following the discontinuation of propylthiouracil and administration of a broad-spectrum of antibiotics, agranulocytosis subsided within 10 days. A total thyroidectomy to avoid any future relapse was planned and a short course of a beta-adrenergic blocker and Lugol solution were prescribed before the operation. At the 28th week of gestation, a total thyroidectomy was performed without complications and thyroxine replacement therapy was commenced. At the 40th week of gestation, labor was induced and a 3,370 g healthy male infant was born without clinical features of thyrotoxicosis. We report herein on the patient and the treatment options for this rare and complicated case. PMID:16491833

Cho, Yoon-Young; Shon, Ho Sang; Yoon, Hyun Dae

2005-12-01

481

Rosai-Dorfman Disease in Thoracic Spine: A Rare Case of Compression Fracture  

PubMed Central

Sinus histiocytosis with massive lymphadenopathy known as Rosai-Dorfman disease is characterized by painless bilateral cervical lymphadenopathy. Extranodal manifestations are uncommon and spinal involvement is rare. A 15-year-old man presented with intermittent midthoracic back pain only. He had no specific findings on neurologic examinations, hematologic and biochemical laboratory tests. Radiological examination of thoracic spine revealed collapse of T6 vertebrae with thoracic kyphosis and osteolytic lesion of T12 vertebra body. He underwent a removal of bone tumor, anterior reconstruction with mesh and pedicle screw fixation via posterior approach for pathologic confirmation and stabilization. Histopathologic study of the lesion revealed focal infiltration of large histiocytes showing emperipolesis. Immunochemistry stain of histiocytes was positive for CD68 and S-100 but negative for CD1a. This report presents a rare case and literature review of extranodal Rosai-dorfman disease in thoracic spine. PMID:25346769

Kim, Do Young; Park, Ji Hye; Shin, Dong Ah; Yi, Seung; Ha, Yoon; Yoon, Do Heum; Kim, Keung Nyun

2014-01-01

482

Spatial connectedness of plant species: potential links for apparent competition via plant diseases  

E-print Network

. Bouteloua curtipendula was uncommon but occurred in all environments, while Buchloe dactyloides and Bouteloua gracilis were uncommon and only occurred in upland sites. Co-occurrence of plant species

Garrett, Karen A.

483

RARE DISEASES LIST  

EPA Science Inventory

The rare disease list includes rare diseases and conditions for which information requests have been made to the Office of Rare Diseases. A rare disease is defined as a disease or condition for which there are fewer than 200,000 affected persons alive in the United States. The Of...

484

Cardiac Disease in Chronic Obstructive Pulmonary Disease  

Microsoft Academic Search

The cardiac manifestations of chronic obstructive pulmonary disease (COPD) are numerous. Impairments of right ventricular dysfunction and pulmonary vascular disease are well known to complicate the clinical course of COPD and correlate inversely with survival. The pathogenesis of pulmonary vascular disease in COPD is likely multi- factorial and related to alterations in gas exchange and vascular biology, as well as

Jeremy A. Falk; Steven Kadiev; Gerard J. Criner; Steven M. Scharf; Omar A. Minai; Philip Diaz

2008-01-01

485

Alzheimer's Disease: The Death of the Disease.  

ERIC Educational Resources Information Center

Alzheimer's disease, a form of dementia in middle-age and older adults is becoming more evident because of growing numbers of older people and better diagnosis and detection methods. Describes the behavioral and physical symptoms of the disease as well as specific suggestions for care of patients with Alzheimer's disease, including dealing with…

McBroom, Lynn W.

1987-01-01

486

Vanishing White Matter Disease  

MedlinePLUS

What is Vanishing White Matter Disease? Vanishing White Matter Disease (VWM) is inherited in an autosomal recessive manner, meaning that it is a ... information about this). Other Clinical Names for Vanishing White Matter Disease Other clinical names of Vanishing White ...

487

Chronic obstructive pulmonary disease  

MedlinePLUS

COPD; Chronic obstructive airways disease; Chronic obstructive lung disease; Chronic bronchitis; Emphysema; Bronchitis - chronic ... heart swelling and heart failure due to chronic lung disease) Pneumonia Pneumothorax Severe weight loss and malnutrition Thinning ...

488

Heart disease - resources  

MedlinePLUS

Resources - heart disease ... The following organizations are good resources for information on heart disease: American Heart Association - www.americanheart.org Centers for Disease Control and Prevention - www.cdc.gov/heartdisease/

489

Kidney disease - resources  

MedlinePLUS

Resources - kidney disease ... The following organizations are good resources for information on kidney disease: National Kidney Disease Education Program - www.nkdep.nih.gov National Kidney Foundation - www.kidney.org National ...

490

Sickle Cell Disease Quiz  

MedlinePLUS

... False: People with sickle cell disease cannot get malaria . a) True b) False 4. True or False: ... False: People with sickle cell disease cannot get malaria. FALSE People with sickle cell disease can get ...

491

Cardiovascular Disease and Diabetes  

MedlinePLUS

Cardiovascular Disease & Diabetes Updated:Jan 31,2013 The following statistics speak loud and clear that there is a strong correlation between cardiovascular disease (CVD) and diabetes. Heart diseases and stroke are ...

492

Parkinson disease - discharge  

MedlinePLUS

Your doctor has told you that you have Parkinson disease . This disease affects the brain and leads to ... have you take different medicines to treat your Parkinson disease and many of the problems that may come ...

493

Amyloidosis and Kidney Disease  

MedlinePLUS

... Info Statistics Research Resources About Us Espańol National Kidney and Urologic Diseases Information Clearinghouse Publications Tools and ... Failure Series : Amyloidosis and Kidney Disease Amyloidosis and Kidney Disease On this page: What is amyloidosis? What ...

494

Acquired Cystic Kidney Disease  

MedlinePLUS

... Info Statistics Research Resources About Us Espańol National Kidney and Urologic Diseases Information Clearinghouse Publications Tools and ... Failure Series : Acquired Cystic Kidney Disease Acquired Cystic Kidney Disease On this page: What is acquired cystic ...

495

Tay-Sachs disease  

MedlinePLUS

Tay-Sachs disease is a life-threatening disease of the nervous system passed down through families. ... Tay-Sachs disease occurs when the body lacks hexosaminidase A, a protein that helps break down a chemical found ...

496

Autoimmune liver disease panel  

MedlinePLUS

Liver disease test panel - autoimmune ... Autoimmune disorders are a possible cause of liver disease. The most common of these diseases are autoimmune hepatitis and primary biliary cirrhosis. This group of tests helps your health care provider ...

497

Carotid Artery Disease  

MedlinePLUS

... cerebrovascular disease, stroke, transient ischemic attacks (TIA) Carotid artery disease is a form of disease that affects ... to the brain by the 2 large carotid arteries in the front of your neck and by ...

498

Heart Disease in Women  

MedlinePLUS

... page from the NHLBI on Twitter. How Does Heart Disease Affect Women? Espańol In the United States, ... about coronary MVD and broken heart syndrome. Coronary Heart Disease CHD is a disease in which plaque ( ...

499

Hypothyroidism and Heart Disease  

MedlinePLUS

... and Heart Disease Share: Fact Sheet Hypothyroidism and Heart Disease January 2014 Download PDFs English Espanol Editors ... hormone. Why does hypothyroidism increase your risk for heart disease? Both thyroid hormones (T4 and T3) are ...

500

Types of Periodontal Disease  

MedlinePLUS

Types of Periodontal Disease Gingivitis Chronic Periodontitis Aggressive Periodontitis Periodontitis Caused by Conditions of the Body Necrotizing Periodontal Diseases Periodontal disease can refer to any condition that affects the gums and ...