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Sample records for stevens-johnson syndrome complicating

  1. Stevens Johnson Syndrome associated with Lamotrigine

    PubMed Central

    Parveen, Shama; Javed, M. Afzal

    2013-01-01

    Stevens-Johnsons Syndrome (SJS) is an immune-complex-mediated hypersensitivity reaction and has been linked as an adverse side effects to many drugs. Lamotrigine, an anticonvulsive medication and also a commonly used mood stabiliser, can be associated with this adverse reaction. Although this has not been reported very commonly , SJS has high mortality and morbidity and requires careful attention as the use of Lamotrigine is increasing in clinical practice. We present a case where the patient developed Stevens - Johnson Syndrome three weeks after being started on Lamotrigine. The case is discussed for its relevance to the use of Lamotrigine which is currently prescribed very commonly in psychiatric practices. PMID:24550973

  2. Stevens Johnson Syndrome associated with Lamotrigine.

    PubMed

    Parveen, Shama; Javed, M Afzal

    2013-11-01

    Stevens-Johnsons Syndrome (SJS) is an immune-complex-mediated hypersensitivity reaction and has been linked as an adverse side effects to many drugs. Lamotrigine, an anticonvulsive medication and also a commonly used mood stabiliser, can be associated with this adverse reaction. Although this has not been reported very commonly , SJS has high mortality and morbidity and requires careful attention as the use of Lamotrigine is increasing in clinical practice. We present a case where the patient developed Stevens - Johnson Syndrome three weeks after being started on Lamotrigine. The case is discussed for its relevance to the use of Lamotrigine which is currently prescribed very commonly in psychiatric practices. PMID:24550973

  3. Imatinib-induced Stevens-Johnsons syndrome.

    PubMed

    Jha, Praveen; Himanshu, D; Jain, Nirdesh; Singh, Ajay Kumar

    2013-01-01

    Imatinib mesylate is a tyrosine kinase inhibitor used widely as the first-line treatment for chronic myeloid leukaemia (CML). The side-effect profile of this drug includes fluid retention, muscle cramps, diarrhoea, myelosuppression and skin rashes. Of these, rashes of the type maculo-papular eruptions and oedema developed most commonly. The cutaneous adverse reactions other than maculo-papular eruptions are rare with imatinib. Severe and life-threatening cutaneous reactions can occur in 5% cases. Here, the author reports a case of newly diagnosed CML that developed Steven-Johnsons syndrome due to imatinib therapy. Patient responded and discharged successfully on withdrawal of the culminating drug. PMID:23349042

  4. Imatinib-induced Stevens-Johnsons syndrome

    PubMed Central

    Jha, Praveen; D, Himanshu; Jain, Nirdesh; Singh, Ajay Kumar

    2013-01-01

    Imatinib mesylate is a tyrosine kinase inhibitor used widely as the first-line treatment for chronic myeloid leukaemia (CML). The side-effect profile of this drug includes fluid retention, muscle cramps, diarrhoea, myelosuppression and skin rashes. Of these, rashes of the type maculo-papular eruptions and oedema developed most commonly. The cutaneous adverse reactions other than maculo-papular eruptions are rare with imatinib. Severe and life-threatening cutaneous reactions can occur in 5% cases. Here, the author reports a case of newly diagnosed CML that developed Steven-Johnsons syndrome due to imatinib therapy. Patient responded and discharged successfully on withdrawal of the culminating drug. PMID:23349042

  5. Phenytoin induced Stevens-Johnson syndrome exacerbated by cefepime

    PubMed Central

    Prabhu, Varsha A.; Doddapaneni, Sahiti; Thunga, Girish; Thiyagu, Rajakannan; Prabhu, M. Mukyaprana; Naha, Kushal

    2013-01-01

    Steven Johnson syndrome (SJS) is a rare drug induced mucocutaneous reaction. Here, we present an elaborate report of a 28-year-old female patient who developed Phenytoin induced SJS, which was exacerbated by cefepime. PMID:24250210

  6. Late onset imatinib-induced Stevens-Johnson syndrome.

    PubMed

    Bois, Evan; Holle, Lisa M; Farooq, Umar

    2014-12-01

    Imatinib, a BCR-ABL tyrosine kinase inhibitor, is known to cause mild to moderate cutaneous reactions in up to approximately 20% of patients. It rarely causes severe reactions, such as Stevens-Johnson syndrome, which can be life threatening. Typically, these rashes occur within two months of initiating therapy. We report a case of Stevens-Johnson syndrome induced by imatinib after several years of therapy, but later successfully was treated with nilotinib therapy. PMID:24399835

  7. Stevens-Johnson syndrome associated with Mycoplasma pneumoniae infections.

    PubMed

    Sontheimer, R D; Garibaldi, R A; Krueger, G G

    1978-02-01

    The Stevens-Johnson syndrome is a multisystem inflammatory disorder associated with a widespread erythematous eruption that can result in death. Although usually considered a pediatric disease, this syndrome frequently affects adults. There are many etiologic associations including drugs and infections; however, the pathophysiology of the syndrome remains obscure. Treatment at present is symptomatic and supportive. Although frequently used, the beneficial role of corticosteroids in this syndrome remains to be proved. The case report describes a young woman who after treatment with several drugs developed the Stevens-Johnson syndrome in association with a Mycoplasma pneumoniae infection. We include a brief review of the literature with emphasis on the Stevens-Johnsons syndrome's association with M pneumoniae infections. Those caring for patients with skin disease should be aware of the association between such treatable infections and this syndrome. PMID:629550

  8. Co-amoxiclav-induced Stevens Johnson Syndrome in a child

    PubMed Central

    Fathallah, Neila; Hanen, Zayani; Slim, Raoudha; Boussofara, Lobna; Najet, Ghariani; Bouraoui, Kamel; Salem, Chaker Ben

    2013-01-01

    Stevens-Johnson Syndrome is an uncommon life threatening disease generally induced by drugs. Antibiotics, mainly sulphonamides, are the most involved drugs in Stevens-Johnson Syndrome in children. Co-amoxiclav is a well tolerated antibiotic. It has never been reported to cause, lonely this syndrome in children. Herein, we report a co-amoxiclav-induced Stevens-Johnson Syndrome occurring in an 18-month-old child. The diagnosis of SJS is often challenging in children and other possible diseases should be ruled out. The etiology of this syndrome is not yet fully understood. It is thought to be mediated by an immunologic mechanism. Management involves early identification, withdrawal of the culprit drug and rapid initiation of supportive therapies. PMID:23560121

  9. Genetics Home Reference: Stevens-Johnson syndrome/toxic epidermal necrolysis

    MedlinePlus

    ... Conditions Stevens-Johnson syndrome/toxic epidermal necrolysis Stevens-Johnson syndrome/toxic epidermal necrolysis Enable Javascript to view ... Download PDF Open All Close All Description Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a ...

  10. Spontaneous complete regression of hypothalamic pilocytic astrocytoma after partial resection in a child, complicated with Stevens-Johnson syndrome: a case report and literature review.

    PubMed

    Samadian, Mohammad; Bakhtevari, Mehrdad Hosseinzadeh; Haddadian, Karim; Alavi, Hossein Afshin; Rezaei, Omidvar

    2016-04-01

    Pilocytic astrocytoma (PA) is the most common pediatric central nervous system glial neoplasm and the most common pediatric cerebellar tumor. The spontaneous regression that occurs after partial/subtotal resection is multifactorial, depending on multiple factors, as for the case of humoral and cell-mediated immune responses of the host to the implanted tumor. A 7-year-old boy was referred to a neurosurgery clinic with headache. Further imaging workup revealed hypothalamic PA. Partial resection of the lesions was performed with right-side pterional approach. The patient developed a severe panmucositis [Stevens-Johnson syndrome (SJS)] and respiratory failure plus conjunctivitis, due to phenytoin allergy. During the patient's 6-month follow-up, postoperative magnetic resonance imaging (MRI) revealed a residual tumor, and about 9 months later (at 15 months postoperatively), the MRI showed total regression of the tumor. Clinically, symptomatic PA may undergo spontaneous regression after partial resection. We report a well-documented case of spontaneous regression hypothalamic PA after partial resection that complicated with SJS. Immune system reaction in SJS may have a role in tumor behavior and spontaneous regression. Multiple studies confirmed spontaneous regression in PA after partial/subtotal resection. This phenomenon occurs due to humoral and cell-mediated host immune responses to the implanted tumor. The immune system reaction in SJS may have a role in tumor behavior and spontaneous regression. PMID:26662551

  11. Stevens-Johnson syndrome: The role of an ophthalmologist.

    PubMed

    Jain, Rajat; Sharma, Namrata; Basu, Sayan; Iyer, Geetha; Ueta, Mayumi; Sotozono, Chie; Kannabiran, Chitra; Rathi, Varsha M; Gupta, Nidhi; Kinoshita, Shigeru; Gomes, José A P; Chodosh, James; Sangwan, Virender S

    2016-01-01

    Stevens-Johnson syndrome (SJS) is an acute blistering disease of the skin and mucous membranes. Acute SJS leads to the acute inflammation of the ocular surface and chronic conjunctivitis. If not properly treated, it causes chronic cicatricial conjunctivitis and cicatricial lid margin abnormalities. Persistent inflammation and ulceration of the ocular surface with cicatricial complications of the lids leads to chronic ocular sequelae, ocular surface damage, and corneal scarring. The destruction of the glands that secrete the tear film leads to a severe form of dry eye that makes the management of chronic SJS difficult. The option that is routinely used for corneal visual rehabilitation, keratoplasty, is best avoided in such cases. We describe the management strategies that are most effective during the acute and chronic stages of SJS. Although treatments for acute SJS involve immunosuppressive and immunomodulatory therapies, amniotic membrane transplantation is also useful. The options for visual rehabilitation in patients with chronic SJS are undergoing radical change. We describe the existing literature regarding the management of SJS and highlight recent advances in the management of this disorder. PMID:26829569

  12. Paracetamol-induced Stevens Johnson syndrome and cholestatic hepatitis.

    PubMed

    Slim, Raoudha; Fathallah, Neila; Aounallah, Amina; Ksiaa, Mehdi; Sriha, Badreddine; Nouira, Rafiaa; Ben Salem, Chaker

    2015-01-01

    Stevens-Johnson syndrome (SJS) is an uncommon life-threatening skin disease, generally induced by drugs. Extracutaneous manifestations of the syndrome can occur, and may involve the conjunctiva, buccal mucosa, gastrointestinal and genitourinary tracts. Cholestatic hepatitis has been rarely described in SJS. A 29-year-old woman was admitted with generalized cutaneous eruption. A self-medication with paracetamol had been started three days earlier. Clinical signs and skin biopsy were consistent with SJS. Five days later, the patient developed jaundice. Serial liver function tests showed rising transaminases, bilirubin, alkaline phosphatase and γ-glutamyl transferase. Liver biopsy was performed and was consistent with the diagnosis of drug-induced cholestatic hepatitis. Adequate supportive care was provided to the patient. Skin lesions disappeared within two weeks. Jaundice disappeared progressively, and liver tests returned to normal. Herein, we report the first case of SJS associated with cholestatic hepatitis after ingestion of therapeutic doses of paracetamol. PMID:25158788

  13. Metolazone Associated Stevens Johnson Syndrome-Toxic Epidermal Necrolysis Overlap

    PubMed Central

    Chauhan, Ajay; Charaniya, Riyaz; Ghosh, Anindya; Tandon, Vaibhav

    2016-01-01

    Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe mucocutaneous disease with high mortality rate. It is characterised by severe necrosis and detachment of the epidermis. Drugs are the most common triggering agent for SJS/TEN. These are commonly reported with the use of aromatic antiepileptics, antiretrovirals, allopurinol, NSAID’S and sulfonamide antibiotics. Non antibiotic sulfonamides rarely cause SJS/TEN. Metolazone is a well known diuretic and is extensively used by clinicians. Although this drug is in market for last several decades, no case of SJS/TEN has been reported till date. We report a rare case of metolazone induced SJS/TEN overlap in a 55-year-old lady. PMID:27134890

  14. Paracetamol induced Steven-Johnson syndrome: A rare case report

    PubMed Central

    Rajput, Rajan; Sagari, Shitalkumar; Durgavanshi, Astha; Kanwar, Alpana

    2015-01-01

    In the contemporary era, use of drugs is the dominant paradigm of health care. The most quotidian drug used for fever and pain is paracetamol. Although adverse reactions to paracetamol in India are rare, at times they can cause life-threatening situations. Stevens-Johnson syndrome (SJS) is one such potentially lethal adverse drug reaction. The most reported cases of analgesic-induced SJS were due to oxicams or propionic acid derivatives. There are very few detailed reports of SJS due to the use of paracetamol. We report a case of SJS, which occurred due to the use of paracetamol. The clinical features of this condition and multidisciplinary management of the patient are described in brief. PMID:26604588

  15. [Diagnostic problems of Stevens-Johnson syndrome. A case report].

    PubMed

    Djordjević, N; Sarović, N; Pasić, S; Dujić, A

    1994-01-01

    Stivens-Johnson Syndrome is a rare, severe, bullose form of erythema multiforme of unknown etiology. The role of immunological factors in its pathogenesis elucidates. A patients (Sh.V.), nine years of age, was admitted for reccurent streptococcal infections with skin and mucose membrane lesions. In June 1990 streptococcal pharyngitis, fever (38.8-39,9 degrees C) were registered. Penicillin was given. Next day bullous lesions on lips, left ear, trunk and lower extremities and vesiculose lesions with a wide, erythematose base ("iris") and then conjuctivitis were registered. Laboratory tests: SR70.; Leu - 11,0; anti-herpes Ab IgG 1/64, IgM 1/8. Stevens-Johnson was diagnosed. There was a recidivation two years after - oral lesions followed by necrosis and bleeding, after half a year a second recidivation with spreading of bullous and vesiculous lesions to penis gland with prepuce of the penis. Last recidivation in February 1993. Anamnesis: Viral meningitis in 1988. mother suffers from herpes labialis. Peripheral blood immunophenotiping lymphocite extremly indicated decreasing values of B Ly, NK and IL-2R+ cells. Bacteriological tests showed an increase of anti-Chlamidia Ab titer (IgG 1/128, IgA and IgM +). In virological testing there was no increase of titer of Abs against viral antigens (Herpes simplex virus, Varicella-Zoster virus, Citomegalovirus, Adenovirus). We conclude that Stevens-Johnson Sy to be diagnosed by characteristic clinical features, aspecialy by frequent reccurences. Immunological testing during the last recidivation showed that parameters of humoral immune reactivity were within normal ranges while revealed defects of cellular immune reactivity cannot elucidate the ethiopathogenesis of this disease. PMID:18173215

  16. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: An Update.

    PubMed

    Dodiuk-Gad, Roni P; Chung, Wen-Hung; Valeyrie-Allanore, Laurence; Shear, Neil H

    2015-12-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening mucocutaneous reactions, predominantly drug induced. The mortality rates for SJS and TEN are as high as 30 %, and short- and long-term morbidities are very common. SJS/TEN is one of the few dermatological diseases that constitute a true medical emergency. Early recognition and prompt and appropriate management can be lifesaving. In recent years, our understanding of the pathogenesis, clinical presentation, and management of SJS/TEN has improved. Nevertheless, in 2015, there are still no internationally accepted management guidelines. This review summarizes up-to-date insights on SJS/TEN and describes a protocol for assessment and treatment. We hope these suggested guidelines serve as a practical clinical tool in the management of SJS/TEN. The classic manifestation of SJS/TEN consists of initial "flu-like" symptoms (malaise, fever, anorexia) in the prodromal phase, followed by cutaneous and mucous membrane (ocular, oral, and genital) inflammation and pain, and other systemic involvement. Symptoms usually begin 4-28 days after the onset of drug intake. Treatment is multidisciplinary and includes identification and withdrawal of the culprit drug, transfer to a specialist unit, supportive care, medical treatment, communication, and provision of appropriate information and emotional support. PMID:26481651

  17. Outbreak of Mycoplasma pneumoniae–Associated Stevens-Johnson Syndrome

    PubMed Central

    Watkins, Louise K. Francois; Demirjian, Alicia; Lin, Xia; Robinson, Christine C.; Pretty, Kristin; Benitez, Alvaro J.; Winchell, Jonas M.; Diaz, Maureen H.; Miller, Lisa A.; Foo, Teresa A.; Mason, Melanie D.; Lauper, Ursula L.; Kupfer, Oren; Kennedy, Jeffrey; Glodé, Mary P.; Kutty, Preeta K.; Dominguez, Samuel R.

    2015-01-01

    BACKGROUND: Stevens-Johnson syndrome (SJS) is an uncommon, sporadic disease and outbreaks are rare. In November 2013, an outbreak of SJS was identified at Children’s Hospital Colorado. METHODS: Outbreak cases were children aged 5–21 with a discharge diagnosis of SJS admitted from September 1 to November 30, 2013. Medical charts were reviewed using standardized data collection forms. Respiratory specimens were tested for viruses and Mycoplasma pneumoniae (Mp) by polymerase chain reaction (PCR). We conducted a separate 4-year retrospective case-control study comparing hospitalized SJS cases with and without evidence of Mp infection. RESULTS: During the outbreak, 8 children met SJS criteria. Median age was 11.5 years (range 8–16 years); 5 (63%) were boys and 5 (63%) were Mp-PCR–positive. Of the 5 PCR-positive children, none had preceding medication exposure, and all had radiographic pneumonia. All outbreak Mp isolates were macrolide susceptible. The retrospective case-control analysis showed that Mp-associated SJS episodes (n = 17) were more likely to have pneumonia (odds ratio [OR] 10.0, confidence interval [CI] 1.3–5.1), preceding respiratory symptoms (OR 30.0, CI 1.6–72.6), an erythrocyte sedimentation rate ≥35 mg/dL (OR 22.8, CI 2.1–244.9), and ≤3 affected skin sites (OR 4.5, CI 1.2–17.4) than non–Mp-associated SJS episodes (n = 23). CONCLUSIONS: We report the largest outbreak of SJS in children, which was also predominately associated with Mp infection. Mp-associated SJS was associated with a distinct clinical presentation that included less extensive skin disease, an elevated erythrocyte sedimentation rate, and evidence of a preceding respiratory infection. PMID:26216320

  18. [Stevens-Johnson syndrome in the literature and authors' own studies].

    PubMed

    Chłystowska, Marta; Pietruszka-Chmarra, Anna; Szafrański, Tomasz; Michalak, Jerzy

    2008-01-01

    Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis (Lyell Syndrome) are severe, multisystem disease caused mainly by reaction to drugs. The clinical features include: changes on the skin and mucosa as well as lesions in the internal organs. There are no standards of treatment in this diesease group. There are ongoing trials in various groups of drugs. Apart from providing the appriopriate conditions and symptomatic treatment, immunoglobulins, cytostatics and plasmapheresis are used. The authors present the clinical course of a patient with Stevens-Johnsons Syndrome, probably caused by klaritromycin. The 13 y.o. boy was admitted to a Department of Paediatric Surgery specializing in treatment of burns. Immunoglobulin, cytostatics, antibacterial and antiviral drugs were used as well as topical medicines routinely applied in burns. The method proved to give good results. The treatment of Stevens-Johnsons Syndrome is difficult and expensive. Due to lack of standards, the treatment depends on one's own experience and up to date literature. PMID:19305034

  19. Stevens-Johnson Syndrome triggered by chemical hair relaxer: a case report.

    PubMed

    Booker, Matthew J

    2009-01-01

    This case report describes a 41-year-old Afro-Caribbean lady presenting with a constellation of pyrexia, conjunctivitis, arthralgia, sterile dysuria, apthous ulceration, labial crusting and widespread erythema multiforme. A diagnosis of Stevens-Johnson Syndrome was made. She had taken no medications recently (the most common precipitant of Stevens-Johnson Syndrome) and a full screen for the common and atypical bacterial and viral triggers was negative. The identified trigger was the use of a chemical hair relaxant treatment a few days previously. With supportive measures and a course of oral prednisolone, the patient quickly improved and made a full recovery. This case highlights the importance of considering occupational and recreational precipitants of Stevens-Johnson Syndrome. PMID:19918477

  20. Stevens-Johnson Syndrome triggered by chemical hair relaxer: a case report

    PubMed Central

    Booker, Matthew J

    2009-01-01

    This case report describes a 41-year-old Afro-Caribbean lady presenting with a constellation of pyrexia, conjunctivitis, arthralgia, sterile dysuria, apthous ulceration, labial crusting and widespread erythema multiforme. A diagnosis of Stevens-Johnson Syndrome was made. She had taken no medications recently (the most common precipitant of Stevens-Johnson Syndrome) and a full screen for the common and atypical bacterial and viral triggers was negative. The identified trigger was the use of a chemical hair relaxant treatment a few days previously. With supportive measures and a course of oral prednisolone, the patient quickly improved and made a full recovery. This case highlights the importance of considering occupational and recreational precipitants of Stevens-Johnson Syndrome. PMID:19918477

  1. Burn unit care of Stevens Johnson syndrome/toxic epidermal necrolysis: A survey.

    PubMed

    Le, Hong-Gam; Saeed, Hajirah; Mantagos, Iason S; Mitchell, Caroline M; Goverman, Jeremy; Chodosh, James

    2016-06-01

    Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a systemic disease that can be associated with debilitating acute and chronic complications across multiple organ systems. As patients with acute SJS/TEN are often treated in a burn intensive care unit (BICU), we surveyed burn centers across the United States to determine their approach to the care of these patients. The goal of our study was to identify best practices and possible variations in the care of patients with acute SJS/TEN. We demonstrate that the method of diagnosis, use of systemic therapies, and involvement of subspecialists varied significantly between burn centers. Beyond supportive care provided to every patient, our data highlights a lack of standardization in the acute care of patients with SJS/TEN. A comprehensive guideline for the care of patients with acute SJS/TEN is indicated. PMID:26810444

  2. Prevention of Vulvovaginal Sequelae in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

    PubMed Central

    Kaser, Daniel J; Reichman, David E; Laufer, Marc R

    2011-01-01

    Vulvovaginal sequelae of Stevens-Johnson syndrome and toxic epidermal necrolysis are well documented in the literature, although little consensus exists about effective prevention strategies. This review summarizes the available literature and offers expert opinion about how to minimize long-term vaginal impairment from these rare but often devastating illnesses. PMID:22102931

  3. Concurrence of Stevens-Johnson Syndrome and Bilateral Parotitis after Minocycline Therapy

    PubMed Central

    Yoon, Jimi; Lee, Seung Hun; Kim, Tae-Heung; Choi, Deok-Jin; Kim, Jin-Pyeong; Yoon, Tae-Jin

    2010-01-01

    Minocycline is an antibiotic of tetracycline derivatives that is commonly used in the treatment of moderate to severe acne vulgaris. It has been reported to cause rare adverse events from mild cutaneous eruption to severe forms including drug-induced lupus, serum sickness-like reaction, and hypersensitivity reactions, etc. The risks of adverse events attributed to minocycline have not been ascertained reliably and there are concerns about the safety of minocycline which could possibly result in life-threatening events such as the Stevens-Johnson syndrome. Here we demonstrate an unusual case of Stevens-Johnson syndrome in conjunction with bilateral parotitis after the intake of minocycline in a Korean boy suggesting discreet use of the drug. PMID:21103193

  4. Emergent retrograde tracheal intubation in a 3-year-old with stevens-johnsons syndrome.

    PubMed

    He, Michael

    2014-01-01

    A 3-year-old girl suffering from Stevens-Johnsons Syndrome with severe sloughing of the oropharyngeal mucosa was brought to the operating room for an emergent tracheostomy after multiple failed attempts to intubate the trachea in the pediatric intensive care unit. However, a retrograde tracheal intubation was successfully performed in the operating room to secure her airway, after which a tracheostomy was performed. Retrograde intubation can be a quick and effective method for securing the difficult airway. PMID:25612259

  5. Evaluation of conjunctival bacterial flora in patients with Stevens-Johnson Syndrome

    PubMed Central

    Frizon, Luciana; Araújo, Marília Cavalcante; Andrade, Larissa; Yu, Maria Cecília Zorat; Wakamatsu, Tais Hitomi; Höfling-Lima, Ana Luisa; Gomes, José Álvaro Pereira

    2014-01-01

    OBJECTIVE: To determine the conjunctival bacterial flora present in patients with Stevens-Johnson syndrome. METHODS: A prospective study of the conjunctival bacterial flora was performed in 41 eyes of 22 patients with Stevens-Johnson syndrome. The information gathered included the patient's sex and age, the duration of disease, the cause of Stevens-Johnson syndrome, and treatments. Scrapings of the inferior conjunctival fornix were performed in both eyes. Fourteen days before scraping, the patients were asked to interrupt all topical medication and start using 0.5% nonpreserved methylcellulose. The microbiological evaluation included microorganism identification and determination of antibiotic sensitivity. RESULTS: Of 22 patients (41 eyes), 14 (64%) were females, and eight (36%) were males. The mean age was 33.2 years, and the mean duration of disease was 15.6 years. Visual acuity ranged from light perception to 20/25 (1.57 logMar). The treatment received by most patients consisted of tear substitutes, topical antibiotics, and contact lenses. Bacterial identification was positive in 39 eyes (95%) and negative in two eyes (5%). Gram-positive cocci accounted for 55.5% of the microorganisms, whereas gram-positive bacilli and gram-negative bacilli accounted for 19% and 25.5%, respectively. Half of the patients (54%) had multiple bacterial species in their flora, and only one bacterial species was identified in the other half. Resistant bacteria were isolated from four eyes. The antibiotic sensitivity results for the Streptococcus group showed the lowest sensitivity and the highest microbial resistance identified. CONCLUSION: Patients with Stevens-Johnson syndrome have a diverse conjunctival flora that includes many pathogenic species. PMID:24626941

  6. Steven-Johnson syndrome due to ayurvedic drugs

    PubMed Central

    Shivamurthy, Raghu Prasada M.; Kallappa, Ravindra; Reddy, Shashikala G. H.; Rangappa, Druva Kumar B.

    2012-01-01

    Steven–Johnson syndrome may be considered as a cytotoxic immune reaction to drugs, infections etc. This is a case report of Steven–Johnson syndrome due to an ayurvedic preparation which was used in the treatment of mental retardation in a young girl. PMID:22345890

  7. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis in the Pediatric Population: A Review.

    PubMed

    Alerhand, Stephen; Cassella, Courtney; Koyfman, Alex

    2016-07-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe dermatologic reactions with mucocutaneous involvement that carry elevated mortality rates. They differ along a spectrum of severity based upon body surface area affected. These conditions, usually caused by a drug or infection, are believed to result from cell-mediated and often drug-specific cytotoxic reactions against keratinocytes, leading to widespread dermal-epidermal detachment. Studies attempting to identify potential curative therapies such as intravenous immune globulin (IVIG) and corticosteroids remain inconclusive. However, improved outcomes have been demonstrated by early withdrawal of offending medications, early transfer to an intensive care unit or burn unit, and aggressive supportive care. Due to the rare incidence of SJS and TEN, its recurrence among survivors hints at future vulnerability for these patients, and notorious offending medications should thus be avoided. This clinical review will highlight the diagnostic and therapeutic challenges posed by SJS and TEN, while emphasizing the need to maintain them high on the emergency medicine physician's differential. The review will also detail the supportive measures to take for preventing the rapid progression of mucocutaneous complications and subsequent sepsis-related mortality. PMID:27380605

  8. Phenytoin induced Steven-Johnson syndrome and bronchiolitis obliterans - case report and review of literature.

    PubMed

    Pannu, Bibek S; Egan, Ashley M; Iyer, Vivek N

    2016-01-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are both rare but serious idiosyncratic drug reactions characterized by diffuse muco-epidermoid injury and high mortality. Keratinocytes in both skin and mucous membranes (including eyes, mouth and genitalia) are injured resulting in a diffuse maculopapular rash, blistering lesions and epithelial detachment with minimal force (Nikolsky's sign). SJS is typically diagnosed when less than 10% of the skin surface is involved and the term TEN is used in cases with more than 30% involvement. Respiratory involvement in SJS-TEN is common with 30-50% of cases demonstrating respiratory epithelial sloughing with severe short and long term complications. Patients who survive SJS-TEN are often left with impaired respiratory function and bronchiolitis obliterans. Cases of bronchiolitis obliterans with SJS/TEN have been very rarely reported. We report a case of phenytoin induced SJS/TEN followed by severe bronchiolitis obliterans in an adult patient. The presentation, pathophysiology and management of SJS/TEN related bronchiolitis obliterans is also reviewed. PMID:27222786

  9. Rapid onset of Stevens-Johnson syndrome and toxic epidermal necrolysis after ingestion of acetaminophen.

    PubMed

    Kim, Eun-Jin; Lim, Hyun; Park, So Young; Kim, Sujeong; Yoon, Sun-Young; Bae, Yun-Jeong; Kwon, Hyouk-Soo; Cho, You Sook; Moon, Hee-Bom; Kim, Tae-Bum

    2014-01-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but life-threatening, severe cutaneous adverse reactions most frequently caused by exposure to drugs. Several reports have associated the use of acetaminophen with the risk of SJS or TEN. A typical interval from the beginning of drug therapy to the onset of an adverse reaction is 1-3 weeks. A 43-year-old woman and a 60-year-old man developed skin lesions within 3 days after administration of acetaminophen for a 3-day period. Rapid identification of the symptoms of SJS and TEN caused by ingestion of acetaminophen enabled prompt withdrawal of the culprit drug. After administration of intravenous immunoglobulin G, both patients recovered fully and were discharged. These two cases of rapidly developed SJS/TEN after ingestion of acetaminophen highlight the possibility that these complications can develop within only a few days following ingestion of over-the-counter medications such as acetaminophen. PMID:24527413

  10. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Associated with Acetaminophen Use during Viral Infections.

    PubMed

    Ban, Ga-Young; Ahn, Seun-Joo; Yoo, Hye-Soo; Park, Hae-Sim; Ye, Young-Min

    2016-08-01

    An association between drug treatment for viral infections and severe cutaneous adverse reactions has been noted. We investigated six patients diagnosed with Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) after being prescribed acetaminophen for suspected viral illnesses. Multiplex analysis was performed to measure cytokine levels in sera before and after treatment. IL-2Rα levels significantly decreased during the convalescence phase. Although acetaminophen is relatively safe, the drug can trigger SJS/TEN in patients with suspected viral infections. T-cells and monocytes may be key components of the link between viral infection and acetaminophen-induced SJS/TEN. PMID:27574505

  11. Stevens-Johnson syndrome / toxic epidermal necrolysis: an Asia-Pacific perspective

    PubMed Central

    2013-01-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) to drugs which are associated with significant morbidity and mortality. High risk drugs in Asia are similar to those reported worldwide. Human leukocyte antigen (HLA)-related risk alleles for carbamazepine and allopurinol SCAR are unique to Asians. Although prognostic scoring systems like the SCORTEN have been used for more than a decade, pitfalls and caveats need to be recognized, in particular in patients with multiple medical co-morbidities and systemic features in SJS/TEN. In centres without a tertiary Burns Centre, SJS/TEN patients can still be managed successfully in general and dermatology wards with well-executed supportive/nursing care. Controversy remains regarding the effectiveness of immunomodulation in reducing SJS/TEN morbidity, mortality and hastening re-epithelialization. Despite paucity of robust evidence, intravenous immunoglobulins and ciclosporin remain the most commonly used modalities worldwide. Acute and long-term ocular effects are an important source of morbidity for which emerging ophthalmic therapies appear promising. Quality of life issues have now become an important outcome in patients with SJS/TEN as they often impact survivors' future attitudes towards pharmacotherapy. Even though pharmacogenetic testing for high-risk drugs appears to be the panacea for preventing carbamazepine- and allopurinol-induced SJS/TEN in ethnic Asians, many issues remain before health regulators in our region can conclusively determine whether testing should be made mandatory or highly recommended as standard of care. PMID:24260726

  12. Data Mining FAERS to Analyze Molecular Targets of Drugs Highly Associated with Stevens-Johnson Syndrome.

    PubMed

    Burkhart, Keith K; Abernethy, Darrell; Jackson, David

    2015-06-01

    Drug features that are associated with Stevens-Johnson syndrome (SJS) have not been fully characterized. A molecular target analysis of the drugs associated with SJS in the FDA Adverse Event Reporting System (FAERS) may contribute to mechanistic insights into SJS pathophysiology. The publicly available version of FAERS was analyzed to identify disproportionality among the molecular targets, metabolizing enzymes, and transporters for drugs associated with SJS. The FAERS in-house version was also analyzed for an internal comparison of the drugs most highly associated with SJS. Cyclooxygenases 1 and 2, carbonic anhydrase 2, and sodium channel 2 alpha were identified as disproportionately associated with SJS. Cytochrome P450 (CYPs) 3A4 and 2C9 are disproportionately represented as metabolizing enzymes of the drugs associated with SJS adverse event reports. Multidrug resistance protein 1 (MRP-1), organic anion transporter 1 (OAT1), and PEPT2 were also identified and are highly associated with the transport of these drugs. A detailed review of the molecular targets identifies important roles for these targets in immune response. The association with CYP metabolizing enzymes suggests that reactive metabolites and oxidative stress may have a contributory role. Drug transporters may enhance intracellular tissue concentrations and also have vital physiologic roles that impact keratinocyte proliferation and survival. Data mining FAERS may be used to hypothesize mechanisms for adverse drug events by identifying molecular targets that are highly associated with drug-induced adverse events. The information gained may contribute to systems biology disease models. PMID:25876064

  13. Modified tectonic keratoplasty with minimal corneal graft for corneal perforation in severe Stevens - Johnson syndrome: a case series study

    PubMed Central

    2014-01-01

    Background Corneal perforation in severe Stevens-Johnson syndrome (SJS) presenting great therapeutic difficulties, the imperative corneal transplantation always result in graft failure and repeated recurrence of perforation. The aim of this study was to evaluate the effectiveness of a modified small tectonic keratoplasty (MSTK) with minimal corneal graft in the management of refractory corneal perforation in severe SJS. Methods Refractory corneal perforations in ten patients (10 eyes) with severe SJS were mended with a minimal corneal patch graft, under the guidance of anterior chamber optical coherence tomography, combined with conjunctival flap covering. The outcome measures included healing of the corneal perforation, survival of the corneal graft and conjunctival flap, relevant complications, and improvement in visual acuity. Results Corneal perforation healed, and global integrity was achieved in all eyes. No immune rejection or graft melting was detected. Retraction of conjunctival flap occurred in one eye, which was treated with additional procedure. Visual acuity improved in six eyes (60%), unchanged in three eyes (30%) and declined in one eye (10%). Conclusions The MSTK combined with conjunctival flap covering seems to be effective for refractory corneal perforation in severe SJS. PMID:25102918

  14. Clinical characteristics and treatment outcome of Stevens-Johnson syndrome and toxic epidermal necrolysis

    PubMed Central

    CHANTAPHAKUL, HIROSHI; SANON, THANOMSAK; KLAEWSONGKRAM, JETTANONG

    2015-01-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are erythematous skin lesions with blister formation accompanied by mucosal involvement. These conditions are considered to be life-threatening illnesses. Understanding the clinical presentation, risk factors, treatment options and results will be advantageous for physicians in the management of patients in the future. The aim of the present study was to review and analyze the clinical manifestations, drug implications, treatment and outcome of patients with SJS and/or TEN who had been hospitalized in a tertiary care center. All hospitalized patients with SJS and/or TEN during a 5-year period were retrospectively reviewed. The clinical severity was graded according to the score of toxic epidermal necrolysis (SCORTEN) scale. Clinical symptoms, diagnosis, possible precipitating factors, management and outcome data were collected for analysis. A total of 43 patients (mean age, 49.5 years) were hospitalized and classified into the SJS group (55.8%), SJS/TEN overlap group (20.9%) and TEN group (23.3%). The majority of the patients (90.7%) had mucocutaneous eruptions associated with oral drug administration. Allopurinol, anticonvulsants and antibiotics were the most common causative agents for the mucocutaneous eruption. Twenty-eight patients (65.1%) were treated with corticosteroids. The mortality rate was 6.9%. Comparison between the survival group and the non-survival group revealed that patient age >70 years (P=0.014) and body surface area involvement >20% (P<0.01) were the significant factors associated with mortality. The use of systemic steroids was higher in the survival group in comparison with the non-survival group (65.1 vs. 0%, respectively; P=0.014). The mucocutaneous eruptions in SJS and TEN are mostly caused by medication. With early recognition and treatment, the mortality rate in this study was lower than that in previous reports. Patient age and the area of mucocutaneous involvement

  15. Healthcare utilization and cost of Stevens-Johnson syndrome and toxic epidermal necrolysis management in Thailand

    PubMed Central

    Dilokthornsakul, P; Sawangjit, R; Inprasong, C; Chunhasewee, S; Rattanapan, P; Thoopputra, T; Chaiyakunapruk, N

    2016-01-01

    Background: Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are life-threatening dermatologic conditions. Although, the incidence of SJS/TEN in Thailand is high, information on cost of care for SJS/TEN is limited. This study aims to estimate healthcare resource utilization and cost of SJS/TEN in Thailand, using hospital perspective. Methods: A retrospective study using an electronic health database from a university-affiliated hospital in Thailand was undertaken. Patients admitted with SJS/TEN from 2002 to 2007 were included. Direct medical cost was estimated by the cost-to-charge ratio. Cost was converted to 2013 value by consumer price index, and converted to $US using 31 Baht/1 $US. The healthcare resource utilization was also estimated. Results: A total of 157 patients were included with average age of 45.3±23.0 years. About 146 patients (93.0%) were diagnosed as SJS and the remaining (7.0%) were diagnosed as TEN. Most of the patients (83.4%) were treated with systemic corticosteroids. Overall, mortality rate was 8.3%, while the average length of stay (LOS) was 10.1±13.2 days. The average cost of managing SJS/TEN for all patients was $1,064±$2,558. The average cost for SJS patients was $1,019±$2,601 while that for TEN patients was $1,660±$1,887. Conclusions: Healthcare resource utilization and cost of care for SJS/TEN in Thailand were tremendous. The findings are important for policy makers to allocate healthcare resources and develop strategies to prevent SJS/TEN which could decrease length of stay and cost of care. PMID:27089110

  16. Stevens-Johnson Syndrome Associated with Drugs and Vaccines in Children: A Case-Control Study

    PubMed Central

    Raucci, Umberto; Rossi, Rossella; Da Cas, Roberto; Rafaniello, Concita; Mores, Nadia; Bersani, Giulia; Reale, Antonino; Pirozzi, Nicola; Menniti-Ippolito, Francesca; Traversa, Giuseppe; in Drug and Children, Italian Multicenter Study Group for Vaccine Safety

    2013-01-01

    Objective Stevens-Johnson Syndrome (SJS) is one of the most severe muco-cutaneous diseases and its occurrence is often attributed to drug use. The aim of the present study is to quantify the risk of SJS in association with drug and vaccine use in children. Methods A multicenter surveillance of children hospitalized through the emergency departments for acute conditions of interest is currently ongoing in Italy. Cases with a diagnosis of SJS were retrieved from all admissions. Parents were interviewed on child’s use of drugs and vaccines preceding the onset of symptoms that led to the hospitalization. We compared the use of drugs and vaccines in cases with the corresponding use in a control group of children hospitalized for acute neurological conditions. Results Twenty-nine children with a diagnosis of SJS and 1,362 with neurological disorders were hospitalized between 1st November 1999 and 31st October 2012. Cases were more frequently exposed to drugs (79% vs 58% in the control group; adjusted OR 2.4; 95% CI 1.0–6.1). Anticonvulsants presented the highest adjusted OR: 26.8 (95% CI 8.4–86.0). Significantly elevated risks were also estimated for antibiotics use (adjusted OR 3.3; 95% CI 1.5–7.2), corticosteroids (adjusted OR 4.2; 95% CI 1.8–9.9) and paracetamol (adjusted OR 3.2; 95% CI 1.5–6.9). No increased risk was estimated for vaccines (adjusted OR: 0.9; 95% CI 0.3–2.8). Discussion Our study provides additional evidence on the etiologic role of drugs and vaccines in the occurrence of SJS in children. PMID:23874553

  17. Oesophageal stricturing secondary to adult Stevens-Johnson syndrome: similarities in presentation and management to corrosive injury.

    PubMed

    Agrawal, A; Bramble, M G; Shehade, S; Dean, J

    2003-05-01

    Clinical decisions often have to be made in the absence of evidence. In some cases, it is appropriate to use evidence from similar but more common conditions for which studies have resulted in evidence-based practice. This report describes a case of oesophageal stricture following Stevens-Johnson syndrome illustrating this concept, although it is likely that there are many other conditions in which the same principle will stand the clinician in good stead. Dilatation led to long-standing relief of dysphagia in our case. PMID:12701021

  18. Amniotic Membrane Transplantation as a New Therapy for the Acute Ocular Manifestations of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

    PubMed Central

    Shay, Elizabeth; Kheirkhah, Ahmad; Liang, Lingyi; Sheha, Hossam; Gregory, Darren G.; Tseng, Scheffer C.G.

    2010-01-01

    Stevens-Johnson syndrome and its more severe variant, toxic epidermal necrolysis, have relatively low overall incidence; however, this disease presents with high morbidity and mortality. The majority of patients develop ocular inflammation and ulceration at the acute stage. Due to the hidden nature of these ocular lesions and the concentration of effort toward life-threatening issues, current acute management has not devised a strategy to preclude blinding cicatricial complications. This review summarizes recent literature data, showing how sight-threatening corneal complications can progressively develop from cicatricial pathologies of lid margin, tarsus, and fornix at the chronic stage. It illustrates how such pathologies can be prevented with the early intervention of cryopreserved amniotic membrane transplantation to suppress inflammation and promote epithelial healing at the acute stage. Significant dry eye problems and photophobia can also be avoided with this intervention. This new therapeutic strategy can avert the catastrophic ophthalmic sequelae of this rare but devastating disease. PMID:19699503

  19. The Amnion Doughnut: A Novel Method for Sutureless Fixation of Amniotic Membrane to the Bulbar and Palpebral Conjunctiva in Acute Ocular-Involving Stevens-Johnson Syndrome

    PubMed Central

    Pruet, Christopher M.; Queen, Joanna H.; Kim, Gene

    2014-01-01

    Purpose To describe a novel surgical method for sutureless placement of amniotic membrane on the bulbar and palpebral conjunctiva in the setting of ocular-involving acute Stevens-Johnson syndrome. Methods Six days into an acute Stevens-Johnson episode, a 27-year-old male developed early symblepharon, despite aggressive lubrication and topical steroid therapy. He underwent symblepharon lysis and placement of an amniotic membrane wrapped around a symblepharon ring. Results The patient maintained 20/20 vision in each eye with no recurrent symblepharon formation except for the temporal canthus (which was not covered with amniotic membrane). Conclusion Amniotic-membrane-wrapped symblepharon rings provide a sutureless way to fixate amniotic membrane to the bulbar and palpebral conjunctiva with very good anatomic and functional outcomes in an acute Stevens-Johnson patient. Future research could be directed towards development of a symblepharon ring able to better protect the far temporal conjunctiva. PMID:25222004

  20. Stevens-Johnson syndrome-like exanthema secondary to methotrexate histologically simulating acute graft-versus-host disease.

    PubMed

    Hani, N; Casper, C; Groth, W; Krieg, T; Hunzelmann, N

    2000-01-01

    A 61 year old male patient suffering from psoriasis vulgaris developed a severe skin reaction with toxic myelosuppression three days after administration of 20 mg methotrexate (MTX) p.o. per week and concomitant 100 mg acetylic salicylic acid (ASA) per day. The skin lesions simulated Stevens-Johnson syndrome with ulcerations of the oral mucosa and erythema multiforme-like target lesions. The histology of the epidermis resembled an acute graft-versus-host reaction. The increased toxic effect of MTX on keratinocytes in our patient was most likely caused by a lowered plasma binding capacity and reduced renal excretion of MTX due to concomitant administration of ASA. Thus in the treatment of severe forms of psoriasis with MTX, the combined administration of drugs aggravating MTX toxicity, particularly of ASA, should be carefully considered, due to the increased toxicity and risk of severe skin reactions. PMID:11056429

  1. Stevens Johnson Syndrome and Toxic Epidermal Necrolysis: Maternal and Foetal Outcomes in Twenty-Two Consecutive Pregnant HIV Infected Women

    PubMed Central

    Knight, Lauren; Todd, Gail; Muloiwa, Rudzani; Matjila, Mushi; Lehloenya, Rannakoe J.

    2015-01-01

    Introduction Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) form a spectrum of a rare and life-threatening cutaneous drug reaction. SJS/TEN in pregnancy poses largely unknown risk factors and outcomes for both the mother and foetus compared to the general population. Methods We conducted a study of consecutive pregnant women admitted to single tertiary referral centre in South Africa with SJS/TEN over a 3 year period. They were all managed by the same medical team using the same protocols. We evaluated their underlying illnesses, offending drugs and the course of pregnancy and outcomes to determine factors influencing maternal and foetal outcomes. Results We identified twenty-two women who developed SJS/TEN while pregnant, all of them HIV-infected. Their median age was 29 years. The majority 16/22 (73%) had SJS, the milder variant of the disease affecting < 10% body surface area. Nevirapine was the offending drug in 21/22 (95%) cases. All 22 of the mothers survived with 3/22 (14%) developing postpartum sepsis. Pregnancy outcomes were known in 18/22 women and 9/18 (50%) babies were delivered by caesarean section. There were 2 foetal deaths at 21 and 31 weeks respectively and both were associated with post-partum sepsis. Postnatal complications occurred in 5 cases, 3 involving the respiratory system and the other two being low birth weight deliveries. Eight placentae and one foetus were sent for histology and none showed macroscopic or microscopic features of SJS/TEN. On follow-up, only 12/20 children were tested for HIV at 6 weeks post-delivery and none of them were HIV-infected. All had received prophylactic ARVs including nevirapine. Conclusions TEN, the severe form of the disease, was associated with poorer foetal outcomes. SJS/TEN-associated mortality is not increased in HIV-infected pregnant women. Maternal SJS/TEN does not seem to commonly manifest in the foetus. PMID:26267659

  2. Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis (SJS/TEN): Could Retinoids Play a Causative Role?

    PubMed Central

    Mawson, Anthony R.; Eriator, Ike; Karre, Sridhar

    2015-01-01

    Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are overlapping manifestations on a spectrum of acute drug-induced conditions associated with severe blistering, skin peeling, and multi-organ damage. TEN is an eruption resembling severe scalding, with ≥30% skin detachment. SJS is a mild form of TEN, characterized histologically by epidermal keratinocyte apoptosis with dermo-epidermal separation and extensive small blisters with <10% body surface skin detachment. The syndrome can be induced by numerous medications and typically occurs 1–4 weeks after the initiation of therapy. Granulysin is found in the lesions of patients with SJS/TEN and plays a significant pathogenic role in the condition, but the overall mechanisms linking medications, granulysin, and disease manifestations remain obscure. This paper reviews evidence suggesting that the different medications implicated in SJS/TEN have the common property of interacting and synergizing with endogenous retinoids (vitamin A and its congeners), in many instances causing the latter to accumulate in and damage the liver, the main storage organ for vitamin A. It is hypothesized that liver damage leads to the spillage of toxic retinoid compounds into the circulation, resulting in an endogenous form of hypervitaminosis A and cytotoxicity with widespread apoptosis, mediated by granulysin and recognized as SJS/TEN. Subject to testing, the model suggests that symptom worsening could be arrested at onset by lowering the concentration of circulating retinoids and/or granulysin via phlebotomy or plasmapheresis or by pharmacological measures to limit their expression. PMID:25579087

  3. Stevens Johnson Syndrome-Toxic Epidermal Necrolysis Overlap Secondary to Interaction Between Methotrexate and Etoricoxib: A Case Report

    PubMed Central

    Anuradha, HV; Mounika, Reddy

    2015-01-01

    Rheumatoid arthritis (RA) is an autoimmune disease affecting about 1% of people, with the highest incidence between 40 and 70 years. Methotrexate is an anti-folate analogue that has good efficacy and safety in the treatment of RA. Methotrexate (MTX) and non-steroidal anti inflammatory drugs are often concomitantly administered in clinical practice for the treatment of RA. In this case report, a 57-year-old female was treated with oral methotrexate 7.5 mg per week for a diagnosed case of RA. Since her pain persisted after completing six weeks of treatment with methotrexate, oral etoricoxib 60 mg once daily was added to the treatment regimen. Six weeks later, the patient complained of oral ulcerations and blisters on all fours limbs and trunk. The patient was re-evaluated and was diagnosed with Stevens-Johnson syndrome-Toxic epidermal necrolysis (SJS-TEN) overlap. This case highlights the possible pharmacokinetic interaction between methotrexate and etoricoxib that has a significant clinical implication. PMID:26417551

  4. Stevens-Johnson syndrome and toxic epidermal necrolysis in Dr. Hasan Sadikin General Hospital Bandung, Indonesia from 2009-2013

    PubMed Central

    Suwarsa, Oki; Dharmadji, Hartati Purbo; Sutedja, Endang

    2016-01-01

    Background Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) with high mortality and have a significant public health impact because of high mortality and morbidity. Objective To describe data the epidemiological features, etiology, and treatment of retrospectively reviewed data of all patients with SJS and TEN. Methods Retrospective study was conducted in patients with SJS and TEN treated from January 1, 2009 to December 31, 2013 in Dr. Hasan Sadikin General Hospital Bandung, Indonesia. Results A total of 57 patients were enrolled in the study. Thirty-nine cases of SJS (21 males and 18 females), 7 cases of SJS overlapping TEN (4 males and 3 females), and 11 cases of TEN (5 males and 6 females) were reported. All cases of SJS and TEN were caused by drugs, such as paracetamol (16.56%), carbamazepine (7%), amoxicillin (5.73%), ibuprofen (4.46%), rifampicin (3.18%), and trihexyphenidyl (3.18%). All cases were treated systemically with corticosteroid alone (100%). Seven from 57 patients (12,28%) died; 5 cases developed sepsis and 2 cases developed respiratory failure. The mortality rate was 7.69% in SJS, 0% in SJS/TEN overlap, and 36.36% in TEN. Conclusion The role of systemic corticosteroids in SJS and TEN are still controversial, but with a prompt and earlier treatment reduces mortality and improves outcomes of SJS and TEN patients. PMID:26844219

  5. In Silico Risk Assessment of HLA-A*02:06-Associated Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Caused by Cold Medicine Ingredients

    PubMed Central

    Isogai, Hideto; Miyadera, Hiroko; Ueta, Mayumi; Sotozono, Chie; Kinoshita, Shigeru; Tokunaga, Katsushi

    2013-01-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe drug hypersensitivities with high mortality. Typical over-the-counter drugs of cold medicines are suggested to be causative. As multiple ingredients are generally contained in cold medicines, it is of particular interest to investigate which ingredients are responsible for SJS/TEN. However, experimental examination of causal relationships between SJS/TEN and a particular drug molecule is not straightforward. Significant association between HLA-A*02:06 and SJS/TEN with severe ocular surface complications has been observed in the Japanese. In the present study, we have undertaken in silico docking simulations between various ingredients contained in cold medicines available in Japan and the HLA-A*02:06 molecule. We use the composite risk index (CRI) that is the absolute value of the binding affinity multiplied by the daily dose to assess the potential risk of the adverse reactions. The drugs which have been recognized as causative drugs of SJS/TEN in Japan have revealed relatively high CRI, and the association between SJS/TEN and HLA-A*02:06 has been qualitatively verified. The results have also shown that some drugs whose links to SJS/TEN have not been clinically recognized in Japan show the high CRI and suggested that attention should be paid to their adverse drug reactions. PMID:24285954

  6. Spontaneous bilateral corneal perforation in stevens- johnsons syndrome-a challenge in management.

    PubMed

    Md Noh, Umi Kalthum; Then, Kong Yong

    2013-01-01

    A 42-year-old man from Ghana presented with bilateral painful corneal perforations following ingestion of a sulphur-based antibiotic. Emergency bilateral penetrating keratoplasty was performed, with restoration of globe integrity. However, surgical complications arose such as non-healing epithelial defect, secondary infection, graft dehiscence, and mounting intraocular pressure. This case illustrates the challenges faced in managing corneal grafts in patients with already compromised ocular surfaces. PMID:23785259

  7. Spontaneous reports of drug-induced erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis in Denmark 1968-1991.

    PubMed

    Gaist, D; Andersen, M; Schou, J S

    1996-03-01

    Spontaneous reporting systems (SRS) have been established to monitor drug safety problems after marketing, especially rare, but serious adverse drug reactions (ADRs). Among these are the skin disorders erythema multiforme (EM), Stevens- Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). The purpose of this study has been to evaluate the data on these serious skin disorders available in a SRS. All reports concerning these diseases submitted to the Danish Committee on ADRs during the period 1968 to 1991 were reviewed according to predefined criteria. Information was often scarce,and the diagnosis of the reporter had to be accepted at face value in 28% of cases. Two hundred cases of EM, 74 of SJS and 29 of TEN were identified. More than 60% of cases were hospitalized. The diseases had fatal outcome in six patients with TEN, three with SJS and a single patient suffering from EM. One hundred and twenty-eight different drugs were reported as causal agents. Major drug groups involved were antibiotics (sulphonamides and penicillins), non-steroidal anti-inflammatory drugs, anti-epileptics and analgesics. Incidence estimates based on spontaneous reports were compared to the incidence according to the literature and data from a nationwide hospital discharge diagnosis register. The reporting fraction for EM and SJS is estimated to 10-30%, and for TEN to 25-50%, but the validity of reports is in some cases difficult to assess owing to lack of detail. PMID:15073835

  8. Independent strong association of HLA-A*02:06 and HLA-B*44:03 with cold medicine-related Stevens-Johnson syndrome with severe mucosal involvement.

    PubMed

    Ueta, Mayumi; Kaniwa, Nahoko; Sotozono, Chie; Tokunaga, Katsushi; Saito, Yoshiro; Sawai, Hiromi; Miyadera, Hiroko; Sugiyama, Emiko; Maekawa, Keiko; Nakamura, Ryosuke; Nagato, Masaki; Aihara, Michiko; Matsunaga, Kayoko; Takahashi, Yukitoshi; Furuya, Hirokazu; Muramatsu, Masaaki; Ikezawa, Zenrou; Kinoshita, Shigeru

    2014-01-01

    Stevens-Johnson syndrome (SJS) and its severe variant, toxic epidermal necrolysis (TEN), are acute inflammatory vesiculobullous reactions of the skin and mucous membranes. Cold medicines including non-steroidal anti-inflammatory drugs (NSAIDs) and multi-ingredient cold medications are reported to be important inciting drugs. We used two sample sets of Japanese patients to investigate the association between HLA genotypes and cold medicine-related SJS/TEN (CM-SJS/TEN), including acetaminophen-related SJS/TEN (AR-SJS/TEN) with severe mucosal involvement such as severe ocular surface complications (SOC). HLA-A*02:06 was strongly associated with CM-SJS/TEN with SOC and AR-SJS/TEN with SOC. HLA-B*44:03 was also detected as an independent risk allele for CM-, including AR-SJS/TEN with SOC. Analyses using data obtained from CM-SJS/TEN patients without SOC and patients with CM-unrelated SJS/TEN with SOC suggested that these two susceptibility alleles are involved in the development of only CM-SJS/TEN with SOC patients. PMID:24781922

  9. Severe delayed skin reactions related to drugs in the paediatric age group: A review of the subject by way of three cases (Stevens-Johnson syndrome, toxic epidermal necrolysis and DRESS).

    PubMed

    Belver, M T; Michavila, A; Bobolea, I; Feito, M; Bellón, T; Quirce, S

    2016-01-01

    Severe delayed drug-induced skin reactions in children are not common but potentially serious. This article describes aspects concerning the etiology, pathogenesis and clinical manifestations of these processes; it presents three paediatric cases, namely STS (Steven Johnson Syndrome), TEN (toxic epidermal necrolysis), probably related to amoxicillin/clavulanate and ibuprofen and DRESS (a drug reaction with eosinophilia and systemic symptoms) secondary to phenytoin; and in relation to them, the diagnosis and the treatment of these processes are discussed and reviewed. The AGEP (acute generalised exanthematous pustulosis) is also reviewed. The aetiological diagnosis of severe non-immediate reactions is difficult, and the value of current allergological testing is not well defined in these cases. Diagnosis is based on clinical history, the empirical risk of drugs to trigger SJS/TEN or DRESS, and the in vivo and in vitro testing of the suspect drug. Skin biopsy confirms that the clinical diagnosis and delayed hypersensitivity tests, especially the patch test and the lymphoblastic transformation test (LTT), may be important to confirm the aetiological diagnosis, in our cases emphasising the latter. These diseases can be life threatening (especially DRESS and TEN) and/or have a high rate of major complications or sequelae (SJS/TEN). The three cases described progressed well without sequelae. All were treated with corticosteroids, which is the most currently accepted treatment although the effect has not been clearly demonstrated. PMID:26089185

  10. A decade of burn unit experience with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: Clinical pathological diagnosis and risk factor awareness.

    PubMed

    Lim, Victoria M; Do, Annie; Berger, Timothy G; Nguyen, Austin H; DeWeese, Jeffrey; Malone, J David; Jordan, Kathleen; Hom, Fred; Tuffanelli, Lucia; Fillari, Paula; Siu, Shirley; Grossman, Richard

    2016-06-01

    Stevens-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN) is a rare and often fatal spectrum of mucocutaneous diseases usually attributable to severe adverse drug reactions. Burn units are referral centers for patients at the most extreme end of the disease continuum. Our burn center admits a much higher percentage of TEN (>30% BSA) cases than reported in most prior reviews. The purpose of this study was to analyze the diagnostic and prognostic value of variables collected on referred SJS/TEN patients. We retrospectively analyzed 94 patients admitted to our unit with a presumptive SJS/TEN diagnosis made in most cases by the referring center. Most of the diagnoses were clinical. Fifty of the 94 patients underwent biopsy when the clinical diagnosis was questionable. Of the 50 patients who underwent biopsy, 18 (36%) received an alternative diagnosis. Analysis was therefore limited to 76 patients, i.e. 44 patients felt to have firm clinical diagnoses plus 32 patients with diagnoses confirmed by biopsy. Mean age was 54.3 years (17-93) and overall gender ratio was 43 F vs. 33 M (56.6% vs. 43.4%). Mean LOS was 15.2 days (1-48) and overall mortality was 23.7% (18/76). Univariate analysis revealed percent body surface area (%BSA) did not show statistically significant association with mortality. Histopathological correlation for diagnosis is not standardized across institutions worldwide. Due to challenges in the diagnosis of SJS/TEN and the high incidence of error in clinical diagnosis, it is recommended that all patients with presumed SJS/TEN receive skin biopsies with H&E and direct immunofluorescence. We propose a diagnostic approach in order to address this need. Lack of association between %BSA and mortality suggests that all biopsy-proven SJS/TEN cases belong in specialty centers due to the unstable nature of the disease and risk for rapid progression. PMID:26847613

  11. HLA-B*59:01: a marker for Stevens-Johnson syndrome/toxic epidermal necrolysis caused by methazolamide in Han Chinese.

    PubMed

    Yang, F; Xuan, J; Chen, J; Zhong, H; Luo, H; Zhou, P; Sun, X; He, L; Chen, S; Cao, Z; Luo, X; Xing, Q

    2016-02-01

    Methazolamide is an intraocular pressure-lowering drug that is used in the treatment of glaucoma and other ophthalmologic abnormalities. The use of methazolamide has been shown to cause Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in patients of Asian ancestry. Methazolamide-induced SJS/TEN is associated with the presence of HLA-B59 serotype/HLA-B*59:01 in Korean and Japanese populations. To better understand the genetic risk factors for these adverse reactions in the Han Chinese population, we characterized the HLA class I genotypes of eight Chinese patients with methazolamide-induced SJS/TEN from 2008 to 2014. The frequency of HLA-B*59:01 was 87.5% (7/8) in the case patients, which was significantly different from 0% (0/30) in the methazolamide-tolerant patients (odds ratio (OR)=305.0; P=6.3 × 10(-7)) and 0.35% (1/283) in healthy subjects from the human major histocompatibility complex database (OR=1974.0; P=2.0 × 10(-12)). HLA-C*01:02, which is closely linked to HLA-B*59:01, had a weaker but notable association with methazolamide-induced SJS/TEN compared with the tolerant controls (OR=12.1; P=0.016) and general population (OR=15.5; P=2.0 × 10(-3)). The distribution of the HLA-B*59:01-C*01:02 haplotype was also significantly different in cases and controls. This study demonstrated a strong association between HLA-B*59:01 and methazolamide-induced SJS/TEN in the Han Chinese population for the first time. Pretherapy screening for HLA-B*59:01 would be useful to reduce the risk of methazolamide-induced SJS/TEN. PMID:25918017

  12. A study of HLA class I and class II 4-digit allele level in Stevens-Johnson syndrome and toxic epidermal necrolysis.

    PubMed

    Cristallo, A F; Schroeder, J; Citterio, A; Santori, G; Ferrioli, G M; Rossi, U; Bertani, G; Cassano, S; Gottardi, P; Ceschini, N; Barocci, F; Ribizzi, G; Cutrupi, V; Cairoli, R; Rapisarda, V; Pastorello, E A; Barocci, S

    2011-08-01

    Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are represented by rare but life-threatening cutaneous adverse reactions to different drugs. Previous studies have found that in a Han Chinese population from Taiwan and other Asian Countries, a strong genetic association between HLA-class I alleles (B*15:02, B*58:01) and SJS and TEN was induced by carbamazepine and allopurinol, respectively. To identify genetic markers that covered the MHC region, we carried out a case-control association enrolling 20 Caucasian patients with SJS/TEN. Our patient series included 10 cases related to paracetamol, 7 to allopurinol and 3 to different drugs (plaquenil, itraconazol, nabumetone). Healthy controls were represented by 115 Caucasian bone marrow or stem cell donors. The HLA-A*, B*, C*, DRB1*, DQB1*, DQA1* and DPB1* genotyping were determined. The frequencies of HLA-A*33:03 as well as C*03:02 and C*08:01 were significantly higher in SJS/TEN patient subgroup showing allopurinol drug-induced severe cutaneous adverse reactions (SCAR) as compared to controls (28.6% vs 0%, P=0.00002, Pc=0.0011; 28.6% vs 0%, P=0.00002, Pc=0.001; 28.6% vs 0%, P=0.00002, Pc=0.001, respectively). In the same subgroup the frequencies of B*58:01, DRB1*15:02 and DRB1*13:02 alleles, although considerably higher than in control group (42.8% vs 5.2%, P=0.003; 28.6% vs 1.7%, P=0.005; 28.6% vs 3.5%, P=0.037, respectively), appeared no more statistically different after P correction (Pc=0.248; Pc=0.29; Pc=1.00, respectively). In addition, in 10 of the 20 SJS/TEN patient subgroup with paracetamol-induced SCAR no statistically significant association with HLA alleles could be found. However, in the same SJS/TEN patient subgroup showing allopurinol drug-induced SCAR, haplotype analysis indicated that B*58:01, DRB1*13:02 and DRB1*15:02 alleles, that in a single allele analysis lost statistical significance after P correction, may still confer susceptibility, because the B*58:01-DRB1*13:02 and DRB1

  13. Case Report: Stevens-Johnson syndrome following a single double dosing of nevirapine-containing regimen once in an HIV-infected woman on long-term antiretroviral therapy.

    PubMed Central

    Kakande, Betty; Isaacs, Thuraya; Muloiwa, Rudzani; Dlamini, Sipho; Lehloenya, Rannakoe

    2015-01-01

    A 31-year old HIV-infected African woman on nevirapine, tenofovir and lamivudine for more than 4 years presented with an 8-day history of symptoms and signs of Stevens-Johnson syndrome. She was on no other medication. Her viral load was undetectable and she had maintained a CD4 count of between 356 and 387cells/mm 3 in the preceding 2½ years. She missed her antiretrovirals 10 days before the onset of her symptoms and subsequently doubled her daily dose the following day. She had been on no other medication in the preceding 8 weeks. Her ARVs were stopped and she fully re-epithelialized with the exception of the lips, over the following 10 days. She was started on a daily single tablet of Odimune® (a fixed drug combination antiretroviral containing tenofovir, emtricitabine and efavirenz). Nevirapine is the most common offender in cases of antiretroviral-associated SJS in published literature. Lamivudine is very rarely implicated while there are no similar reports with tenofovir.  We concluded that nevirapine was by far the most likely offender in this case. Nevirapine toxicity is associated with high CD4 counts, undetectable viral load and high drug plasma level. We postulate that the sudden increase of the plasma levels of nevirapine in a patient with a high CD4 count and undetectable viral load created a perfect storm for the development of SJS in our patient, who had been on the NVP-containing regimen for many years. Clinicians should be aware that severe adverse drug reactions are dynamic and can occur even when the drug has been in use for a long time. PMID:26629333

  14. Structural modeling of HLA-B*1502/peptide/carbamazepine/T-cell receptor complex architecture: implication for the molecular mechanism of carbamazepine-induced Stevens-Johnson syndrome/toxic epidermal necrolysis.

    PubMed

    Zhou, Peng; Zhang, Shilei; Wang, Yewang; Yang, Chao; Huang, Jian

    2016-08-01

    Drug-induced adverse reactions are a significant problem in healthcare worldwide and are estimated to cost billions of dollars annually in the United States. A portion of such reactions is observed to strongly associate with certain human leukocyte antigen (HLA) alleles; one of the strongest associations is the HLA-B*1502 protein with carbamazepine (CBZ)-induced Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) - the odds ratio value can even be higher than one thousand. The particularly strong association in CBZ-induced SJS/TEN suggests that the HLA-B*1502 is not only a genetic marker but also a participant in the pathogenesis of the disease. In the current study, we attempt to computationally model the atomic-level structure of the complete HLA-B*1502/peptide/CBZ/T-cell receptor (TCR) complex architecture based on prior knowledge obtained from epidemiological investigations as well as in vitro and in vivo assays. The model tells a different story about the molecular mechanism of CBZ-induced SJS/TEN from that previously reported for abacavir (ABC)-induced hypersensitivity (HSR); the CBZ molecule is located at the interface between HLA-B*1502/peptide and TCR, directly contacts the P3-P6 residues of antigen peptide, and bound within a pocket region encompassed by two TCR CDR3 fingers. Molecular dynamics simulation and binding energy analysis further reveal that the CBZ shows considerably high affinity to TCR over HLA-B*1502/peptide, which can tightly interact with the former rather than the latter. From the model, two hypotheses are proposed that can well explain most previous observations and are expected to guide next wet-lab experiments. This study could help to promote our understanding of the molecular mechanism and pathological implication underlying CBZ-induced SJS/TEN. PMID:26488421

  15. Antecedent Drug Exposure Aetiology and Management Protocols in Steven-Johnson Syndrome and Toxic Epidermal Necrolysis, A Hospital Based Prospective Study

    PubMed Central

    Farhat, Samina; Hassan, Iffat

    2016-01-01

    Aim The study sought to identify the magnitude and characteristic of severe cutaneous adverse reactions (SCAR’s) like Steven–Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN). Materials and Methods A prospective study was conducted by the Department of Pharmacology in association with Department of Dermatology in SMHS hospital. The study was carried out from June 2013-June 2015 on hospitalized cases of cutaneous adverse drug reaction reporting in hospital. The SCAR’s were reported in a structured questionnaire based on adverse drug reaction (ADR) reporting form provided by the Central Drug Standard Control Organization (CDSCO) Ministry of Health and Family welfare, Government of India. The SCAR’s were analysed for their characteristics, causality, severity and prognosis. Causality assessment was done by using a validated ADR probability scale of Naranjo as well as WHO Uppsala Monitoring Center (WHO-UMC) system for standardized case causality assessment. The management protocol were analysed for their clinical outcome through a proper follow up period. Results A total of 52 hospitalized cases of cutaneous adverse drug reactions were reported during the study period. We identified a total of 15 cases (28%) of SCAR’s involving 9(17%) of SJS and 6 (12%) of TEN. SJS was seen in 2(22%) males and 7(78%) females. TEN was seen in all females (100%) and in no male. Drugs implicated in causing these life threatening reactions were identified as anticonvulsant agents like carbamazepine (CBZ), phenytoin (PHT) and Lamotrigine (LTG), oxicam NSAID, Sulfasalazine and levofloxacin. Despite higher reported mortality rates in SJS and TEN all patients survived with 2 patients surviving TEN suffered from long term opthalmological sequelae of the disease. Conclusion Present study suggest that drug induced cutaneous eruptions are common ranging from common nuisance rashes to rare life threatening diseases like SJS and TEN, SJS/TEN typically occur 1-3 weeks after

  16. Innovation Squared: Comparison of Models by Tony Wagner and Steven Johnson

    ERIC Educational Resources Information Center

    Fluellen, Jerry E., Jr.

    2012-01-01

    On the surface, Tony Wagner's model of innovation differs from Steven Johnson's. One explores the following: how might we develop a nation of innovators? The other offers seven patterns that mark environments for innovation. Drawing from triangulated data, both authors create regularities (not laws) that have new paradigm, scientific credibility.…

  17. Role of Oxidative and Nitrosative Stress in Pathophysiology of Toxic Epidermal Necrolysis and Stevens Johnson Syndrome—A Pilot Study

    PubMed Central

    Peter, Dincy; Amirtharaj, G Jayakumar; Mathew, Teena; Pulimood, Susanne; Ramachandran, Anup

    2015-01-01

    Background: Oxidative and nitrosative stress caused by drug metabolism may be a trigger for keratinocyte apoptosis in the epidermis seen in toxic epidermal necrolysis (TEN) and Stevens Johnson syndrome (SJS). Aims: To estimate oxidative damage in the serum and to examine the role of nitric oxide in mediating epidermal damage in patients with TEN and SJS. Materials and Methods: A prospective study was conducted among TEN and SJS patients and controls in a tertiary care center between January 2006 and February 2010. Patients with a maculopapular drug rash without detachment of skin constituted the control group 1 (drug exposed). Patients without a drug rash constituted the control group 2 (drug unexposed). The serum values of protein carbonyls, malondialdehyde, conjugated diene and nitrates were measured. Two-group comparison with the non-parametric Mann–Whitney U test was used. Significance of differences if any was established using Pearson's Chi-square test. Results: Ten patients in the SJS-TEN group (study group), 8 patients in control group 1 and 7 patients in control group 2 were included. More than one drug was implicated in 4/10 patients in group 1 and 3/8 patients in group 2. SCORTEN of 0, 1 and 3 at admission were seen in 2, 6 and 2 patients, respectively. The serum values of protein carbonyls, malondialdehyde, conjugated diene and nitrates were not significantly increased in the study group when compared to the controls. Conclusions: There was no elevation of oxidative stress markers in patients with TEN and SJS as compared to the control population. PMID:26538686

  18. Rare extrapulmonary complications of Mycoplasma pneumoniae infection.

    PubMed

    Dhaliwal, Kiran; Enright, Kevin

    2016-01-01

    Stevens-Johnsons syndrome (SJS) is a rare extra-pulmonary complication of Mycoplasma pneumoniae infection. We present the case of a 26-year-old man with fever, cough, extensive oral mucosal ulceration and a widespread truncal rash. He was diagnosed with M. pneumoniae-induced SJS. He responded well to antibiotics and steroids initially, but went on to develop pseudomembranous conjunctivitis requiring bilateral amniotic membrane grafting. SJS is most commonly drug-induced, however, M. pneumoniae is the commonest infectious cause and should be considered in the differential diagnosis. It is also important to get specialist care involved early to minimise the long-term effects of any complications. PMID:26837942

  19. Complications of nephrotic syndrome.

    PubMed

    Park, Se Jin; Shin, Jae Il

    2011-08-01

    Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox), thromboembolism (e.g., venous thromboembolism and pulmonary embolism), hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension), cardiovascular problems (e.g., hyperlipidemia), acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception). The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS. PMID:22087198

  20. Spontaneous Bilateral Corneal Perforation in Stevens- Johnsons Syndrome–A Challenge in Management

    PubMed Central

    Md Noh, Umi Kalthum; Then, Kong Yong

    2013-01-01

    A 42-year-old man from Ghana presented with bilateral painful corneal perforations following ingestion of a sulphur-based antibiotic. Emergency bilateral penetrating keratoplasty was performed, with restoration of globe integrity. However, surgical complications arose such as non-healing epithelial defect, secondary infection, graft dehiscence, and mounting intraocular pressure. This case illustrates the challenges faced in managing corneal grafts in patients with already compromised ocular surfaces. PMID:23785259

  1. Severe idiosyncratic drug reaction (Lyells syndrome) after ingesting dihydroartemisinin.

    PubMed

    Ugburo, A O; Ilombu, C A; Temiye, E O; Fadeyibi, I O; Akinolai, O I

    2009-06-01

    Lyells syndrome also called Toxic epidermal necrolysis is the extreme form of idiosyncratic drug reaction that is called Steven Johnsons Syndrome: The condition results in an extensive loss of the skin with mucous membrane involvement. Lyells syndrome has been induced by many agents. The commonest agent in the literature being sulphonamides. However, in our search of the medical literature there was no report of dihydroarthemisinin as a cause of Lyells syndrome. We report three patients seen at two tertiary health institutions with Lyells syndrome after treatment for malaria with dihydroarthemisinin. This resulted from administration of dihydroarthemisinin with chloroquine in two patients and dihydroarthemisinin with Amodiaquine in one patient. The first patient was a seven year old child who developed 90% cutaneous involvement and died from hemorrhagic shock. The second was a 28 old female that developed a 76% body surface involvement and died from septicemia. The third patient was a pregnant 37 year old woman that developed 52% body involvement and died from septic shock. In these patients the earliest symptoms were not recognized and there was considerable delay before referral. In view of the recent WHO recommendation ofArthemisinin Combination Treatment (ACT) for malaria, we expect more cases of Steven Johnson Syndrome and Lyells syndrome from ACT treatment. The aim of this report is to raise the awareness of clinicians to this potentially fatal complication. PMID:19764682

  2. Complications of Guillain-Barré syndrome.

    PubMed

    Wang, Ying; Zhang, Hong-Liang; Wu, Xiujuan; Zhu, Jie

    2016-04-01

    Guillain-Barré syndrome (GBS) is an immune-mediated disorder in the peripheral nervous system with a wide spectrum of complications. A good understanding of the complications of GBS assists clinicians to recognize and manage the complications properly thereby reducing the mortality and morbidity of GBS patients. Herein, we systemically review the literature on complications of GBS, including short-term complications and long-term complications. We summarize the frequency, severity, clinical manifestations, managements and possible mechanisms of different kinds of complications, and point out the flaws of current studies as well as demonstrate the further investigations needed. PMID:26642351

  3. Use of Cross-linked Donor Corneas as Carriers for the Boston Keratoprosthesis

    ClinicalTrials.gov

    2013-03-20

    Chemical Injuries; Unspecified Complication of Corneal Transplant; Autoimmune Diseases; Ocular Cicatricial Pemphigoid; Stevens Johnson Syndrome; Lupus Erythematosus, Systemic; Rheumatoid Arthritis; Other Autoimmune Diseases

  4. Invasive aspergillosis complicating Cushing's syndrome.

    PubMed

    Walsh, T J; Mendelsohn, G

    1981-08-01

    Patients with Cushing's syndrome are susceptible to opportunistic infections. Invasive fungal infections in patients with Cushing's syndrome caused by endogenous glucocorticoid excess rarely are reported, and aspergillosis occurring in this setting, to our knowledge, has not been described. Two patients with Cushing's syndrome and notably elevated levels of circulating cortisol had invasive aspergillosis develop. A patient with endogenous hypercortisolism caused by adrenal cortical carcinoma suffered a subarachnoid hemorrhage from rupture of an Aspergillus mycotic aneurysm. The other patient, who had an adrenocorticotrophic hormone-producing prostatic carcinoma, had pulmonary and disseminated aspergillosis develop. Exogenous corticosteroids are known clinically and experimentally to facilitate disseminated aspergillosis. Endogenous hypercortisolism also may bae an important factor predisposing to invasive aspergillosis. PMID:7259386

  5. [Tapia's syndrome : Rare complication of securing airways].

    PubMed

    Brandt, L

    2015-02-01

    Nerve injuries are a rare complication of airway management. Two cases of Tapia's syndrome following orotracheal intubation are reported. Case 1: a 23-year-old male patient underwent an otorhinolaryngology (ENT) surgical procedure with orotracheal intubation. A left-sided Tapia's syndrome was verified 3 days later. Case 2: a 67-year-old patient developed a right-sided Tapia's syndrome following an arthroscopic intervention of the left shoulder in the beach-chair position. In both cases there was permanent damage of both nerves. On the basis of a comprehensive literature survey the reasons for an intubation-induced Tapia's syndrome are discussed. In order to avoid a glottis or immediate subglottic position it is recommended to check and to document the position of the cuff (depth of intubation) and the measured cuff pressure immediately after intubation. It also seems to be advisable to document an overstretched head position if required for the operation. PMID:25523320

  6. Klippel-Trenaunay syndrome complicating pregnancy

    PubMed Central

    González-Mesa, Ernesto; Blasco, Marta; Andérica, José; Herrera, José

    2012-01-01

    The Klippel-Trenaunay syndrome is a rare congenital disorder that affects one or more limbs. It is characterised by cutaneous vascular nevi, venous malformations and hypertrophy of soft tissues and bone. There are very few cases reported in pregnant women, so the level of uncertainty is high when it appears during gestation. It is a disease that increases obstetric risk and can exacerbate complications, mainly thromboembolic and haemorrhagic. We report below the case of a pregnant woman diagnosed with this syndrome and the multidisciplinary management held in our centre. PMID:22854239

  7. Idiopathic pulmonary hemosiderosis complicated by Down syndrome.

    PubMed

    Watanabe, Hirofumi; Ayusawa, Mamoru; Kato, Masataka; Chou, Ami; Komori, Akiko; Abe, Yuriko; Matsumura, Masaharu; Kamiyama, Hiroshi; Izumi, Hiroyuki; Takahashi, Shori

    2015-10-01

    We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH. PMID:26508184

  8. Neurosurgical management for complicated catastrophic antiphospholipid syndrome.

    PubMed

    Drazin, Doniel; Westley Phillips, H; Shirzadi, Ali; Drazin, Noam; Schievink, Wouter

    2014-04-01

    Antiphospholipid syndrome (APS) is an autoimmune condition involving arterial and venous thrombosis. An unusual APS variant, catastrophic antiphospholipid syndrome (CAPS), includes rapid multi-organ failure from widespread small vessel thrombosis. Central nervous system complications arise in one-third of CAPS patients. In rare cases, CAPS co-manifests with cerebellar hemorrhage presenting a neurosurgical emergency. We present a 65-year-old woman with CAPS-related cerebellar hematoma, co-morbid idiopathic thrombocytopenic purpura, deep vein thrombosis and altered mental status, with treatment complicated by thrombocytopenia. The patient suddenly deteriorated, secondary to a cerebellar subdural hematoma, and underwent decompression and excision of the hematoma. After recovery in the intensive care unit, she developed a new spontaneous epidural hematoma requiring additional surgery. Management of these patients is hematologically complex and often requires a multi-disciplinary team of physicians. This patient provides an important learning point for clinicians - consider CAPS when hemorrhage and thrombosis are present. PMID:24269552

  9. Teeth syndrome: diagnosis, complications and management

    PubMed Central

    Sbai, Mohamed Ali; Benzarti, Sofien; Boussen, Monia; Maalla, Riadh

    2015-01-01

    Teeth syndrome or fight bite is a specific entity in hand surgery that is little known. It includes infectious complications of the hand following a fist against the teeth. Neglected or misdiagnosed this injury frequently leads to serious complications that could compromise the function of the hand. A retrospective study was performed on 20 patients treated for teeth syndrome at our department, during a period of 12 years (January 2003 to April 2015). All young adults with a mean age of 28 years and a significant male predominance. The dominant side was involved in 15 patients. Lesions were divided into 4 cases of simple dorsal wounds facing the MP joint, 8 cases of dorsal hand cellulitis, and 8 cases of arthritis and osteoarthritis of the metacarpophalangeal (MP) joint of the long fingers. The index was the most affected finger. Treatment consisted in debridement of necrotic tissues, stabilization with external fixation for arthritis, skin reconstruction was performed secondarily. Result was assessed as good in 60% of cases. Clenched fist injuries to the mouth (teeth syndrome or fight bite) are known as being the worst human bites. Usually treated as minor injuries, without realizing a breach of the joint capsule, a lesion of the extensor tendon, or a contamination by oral flora. Any patient with a wound near the joint of the hand and was involved in a fight, need an appropriate evaluation and a specialized treatment to avoid serious complications. PMID:26834924

  10. Limy bile syndrome complicated by obstructive jaundice.

    PubMed

    Sasaki, Takamitsu; Kato, Daisuke; Matsuoka, Nobuhide; Yamashita, Yuichi

    2010-01-01

    Limy bile syndrome is a rare condition in which the gallbladder is filled with a paste-like radiopaque material. The presence of limy bile in the common bile duct is rare. A 72-year-old woman was admitted to our hospital with epigastric pain and jaundice. Plain abdominal radiography on admission showed a radiopaque material in the gallbladder. Computed tomography also showed that the gallbladder and the common bile duct were filled with a radiopaque material. The patient had never received any cholecystographic contrast agents. As a result, a diagnosis of obstructive jaundice due to choledocholithiasis, which includes limy bile, was made. We herein report the process by which limy bile syndrome, complicated by obstructive jaundice, was successfully treated through combined treatment via endoscopic sphincterotomy and laparoscopic cholecystectomy. PMID:20480840

  11. Parkinsonian syndrome complicating systemic lupus erythematosus.

    PubMed

    Shahar, E; Goshen, E; Tauber, Z; Lahat, E

    1998-05-01

    Two girls with florid extrapyramidal parkinsonism complicating systemic lupus erythematosus (SLE) are reported. One patient (15 years old) presented with extreme rigidity, irritability, and mutism initially diagnosed as acute psychosis. Examination revealed severe extrapyramidal akinetic mutism, along with marked restlessness. CT and MRI imaging of the brain were unremarkable. EEG revealed moderate generalized disturbance of background activity. 99mTc-HmPAO SPECT cerebral scanning detected decreased regional cerebral blood flow at the basal ganglia. Dopamine-agonist drugs led to complete recovery after 3 months, along with normalization of EEG and SPECT alterations. The second patient (16 years old) was assessed for progressive bradykinesia and apathy impeding her active daily activities, and she was suspected to have developed depression. Neurologic assessment revealed a parkinsonian syndrome that was less severe than that of the first patient. The EEG showed mild disturbance of background activity, and 99mTc-HmPAO SPECT demonstrated impaired regional cerebral blood flow over the basal ganglia. A parkinsonian extrapyramidal syndrome complicating SLE should therefore be taken into account in any patient with SLE presenting with marked behavioral alterations, rigidity, or akinetic mutism. PMID:9650692

  12. Posterior Reversible Encephalopathy Syndrome Complicating Traumatic Pancreatitis

    PubMed Central

    Sigurtà, Anna; Terzi, Valeria; Regna-Gladin, Caroline; Fumagalli, Roberto

    2016-01-01

    Abstract We are reporting a case of posterior reversible encephalopathy syndrome (PRES) developed in an unusual clinical scenario without the presence of the most described symptoms. PRES is a neurological and radiological syndrome described in many different clinical conditions. In children it has been mostly reported in association with hematological and renal disorders. Our patient was a 15 years old boy, admitted to our intensive care unit for pancreatitis after blunt abdominal trauma. During the stay in the intensive care unit, he underwent multiple abdominal surgical interventions for pancreatitis complications. He had a difficult management of analgesia and sedation, being often agitated with high arterial pressure, and he developed a bacterial peritonitis. After 29 days his neurological conditions abruptly worsened with neuroimaging findings consistent with PRES. His clinical conditions progressively improved after sedation and arterial pressure control. He was discharged at home with complete resolution of the neurological and imaging signs 2 months later. The pathophysiology of PRES is controversial and involves disordered autoregulation ascribable to hypertension and endothelial dysfunction. In this case both hypertension and endothelial activation, triggered by sepsis and pancreatitis, could represent the culprits of PRES onset. Even if there is no specific treatment for this condition, a diagnosis is mandatory to start antihypertensive and supportive treatment. We are therefore suggesting to consider PRES in the differential diagnosis of a neurological deterioration preceded by hypertension and/or septic state, even without other “typical” clinical features. PMID:27258506

  13. Buried bumper syndrome: a rare complication of percutaneous endoscopic gastrostomy.

    PubMed

    Kurek, Krzysztof; Baniukiewicz, Andrzej; Świdnicka-Siergiejko, Agnieszka

    2015-09-01

    Feeding via percutaneous endoscopic gastrostomy (PEG) is the preferred form of alimentation when oral feeding is impossible. Although it is a relatively safe method, some complications may occur. One uncommon PEG complication is buried bumper syndrome. In this paper we report a case of buried bumper syndrome, successfully managed with PEG tube repositioning. PMID:26649105

  14. Pregnancy complications in polycystic ovary syndrome patients.

    PubMed

    Katulski, Krzysztof; Czyzyk, Adam; Podfigurna-Stopa, Agnieszka; Genazzani, Andrea R; Meczekalski, Blazej

    2015-02-01

    Infertility is a widely disputed problem affecting patients suffering from polycystic ovary syndrome (PCOS). As a serious dysfunction, it frequently occurs in PCOS patients. It is, therefore, important to devote more attention to pregnancy in PCOS sufferers. According to various data, the risk of miscarriage in PCOS women is three times higher than the risk of miscarriage in healthy women. Unfortunately, the risk of most frequent pregnancy pathologies is also higher for PCOS patients, as gestational diabetes (GD), pregnancy-induced hypertension and pre-eclampsia, and small for gestational age (SGA) children. Impaired glucose tolerance and GD in pregnant PCOS patients occur more frequently than in healthy women. A quadruple increase in the risk of pregnancy-induced hypertension linked to arterial wall stiffness has also been observed in PCOS patients. The risk of pre-eclampsia, the most severe of all complications, is also four times higher in those suffering from PCOS. Pre-eclampsia is also more frequent in patients presenting additional risk factors accompanying PCOS, such as obesity or GD. At that point, it should be mentioned that PCOS patients are under 2.5 higher risk of giving birth to SGA children than healthy women. It appears that SGA can be linked to insulin resistance and insulin-dependent growth dysfunction. Therefore, PCOS pregnant women are patients of special obstetrical care. PMID:25356655

  15. Grave Complication of Pharyngitis: Lemierre syndrome

    PubMed Central

    Patel, Ankit Kumar B.; Patel, Kimmyben; Doshi, Raj Kumar P.; Patel, Narottam A.

    2015-01-01

    Fusobacterium necrophorum is the causative agent in the pharyngitis. After invasion of the oropharyngeal mucosa by the organism leads to the development of the grave complications like superficial thrombophlebitis of internal jugular vein, septic emboli in the lung and coagulopathy. A 23-year-old male presented with chief complaints of sore throat, headache and high grade fever with past history of upper respiratory tract infection one month ago. On examination, he had high grade fever, tachycardia, hypotension and tender cervical lymph nodes. Oropharyngeal mucosa appeared ooedematous and congested. Leucocytosis with raised ESR was present. Blood culture positive for Fusobacterium necrophorum. Dilated right IJV with the hypoechoic thrombosis seen on Ultrasonography & Doppler. Patient was diagnosed as a case of Lemierre syndrome secondary to oropharyngeal infection was made. He was treated with intravenous imipenem and metronidazole, and then shifted to oral amoxicillin. After completion of full course of the therapy of 6 weeks, patient was improved clinically and all routine blood investigations were normalized. Blood culture was negative for Fusobacterium necrophorum. No evidence of thrombosis within the right internal jugular vein on Doppler. PMID:26266187

  16. Tapia's syndrome — a rare complication following cardiac surgery

    PubMed Central

    Nalladaru, Zubin; Wessels, Andre; DuPreez, Leon

    2012-01-01

    Tapia's syndrome is a rare complication following cardiac surgery. It includes the extracranial involvement of the recurrent laryngeal nerve and the hypoglossal nerve and results in ipsilateral paralysis of the vocal cord and the tongue. It is usually a complication related to anaesthesia and positioning of the head of the patient during surgery. We describe this rare complication which occurred at our institute. A 49-year old man developed Tapia's syndrome after an uneventful coronary artery bypass surgery. He complained of dysphonia, hoarseness of voice and an inability to swallow soon after extubation. The syndrome resolved completely over the following weeks with no neurological deficit. PMID:22108947

  17. Purple glove syndrome: a dreadful complication of intravenous phenytoin administration

    PubMed Central

    Lalla, Rakesh; Malhotra, Hardeep Singh; Garg, Ravindra Kumar; Sahu, Ritesh

    2012-01-01

    Purple glove syndrome is an uncommon but dreaded complication of intravenous phenytoin administration characterised by pain, oedema and purple-blue discolouration of the limb distal to the site of injection. We describe a 37-year-old gentleman having the characteristic purple glove appearance after phenytoin loading, and discuss the salient features of this syndrome highlighting the pathophysiological and preventive aspects. PMID:22922927

  18. Acute Lower Leg Compartment Syndrome: A Rare Complication following CABG

    PubMed Central

    Galvin, Sean

    2016-01-01

    Compartment syndrome of lower legs following coronary artery bypass grafting is a rare complication which results from a combination of local and systemic factors. Early recognition is vital for good outcome. The case discussed describes this rare complication of CABG resulting in long term disability. PMID:27525152

  19. Bouveret Syndrome - An Exceptional Complication of a Very Frequent Disease.

    PubMed

    Gavrila, Daniel; Galusca, Cosmin; Berbecel, Madalina; Boros, Mirela; Dumitrascu, Traian

    2016-01-01

    The Bouveret syndrome is an exceptional complication of the gallbladder lithiasis. Hereby it is described the case of a patient with a history of gallstones complicated on the long-term outcome with gastric outlet obstruction, due to a large gallstone of the duodenum, migrated via a cholecysto-duodenal fistula. The clinical, radiological features and the patient management are described. PMID:27452944

  20. Haemophagocytic syndrome complicating acute lymphoblastic leukaemia.

    PubMed Central

    Stark, R.; Manoharan, A.

    1989-01-01

    A 41 year old female developed reactive haemophagocytic histiocytosis secondary to herpes simplex infection, during remission induction for acute lymphoblastic leukaemia. She recovered fully with acyclovir and supportive treatment. Previous publications on the association between acute lymphoblastic leukaemia and haemophagocytic syndrome are reviewed, and the nature of the haemophagocytic disorder is discussed. Images Figure 1 PMID:2687829

  1. Limy Bile Syndrome Complicated with Primary Hyperparathyroidism

    PubMed Central

    Koca, Yavuz Savas; Koca, Tugba; Barut, Ibrahim

    2015-01-01

    Limy bile is a relatively rare condition, in which a radiopaque material is visible in the gallbladder on plain radiography or computerized tomography. Cases of complicated hyperparathyroidism are extremely rare. We report a patient with right upper quadrant and epigastric pain and extremity weakness in whom abdominal tomography showed limy bile in the gallbladder and laboratory values showed high levels of serum calcium and parathormone. PMID:25821626

  2. Neurologic Complications of the Acquired Immune Deficiency Syndrome

    PubMed Central

    Slade, Walter R.

    1987-01-01

    The acquired immune deficiency syndrome (AIDS) is a syndrome requiring unique knowledge of its versatile manifestations for accurate diagnosis and skillfull management of its numerous complications for successful treatment. The human T-cell lymphotropic virus type III (HTLV-III), a replication-complete virus, is now reported as the etiologic agent. The neurologic complications of AIDS cover the spectrum of neurologic diseases and usually have multiple causative factors, all of which should be appropriately managed. These complications can be successfully treated, although constant monitoring is required because recurrence is frequent. The neurologic complications are the second most frequent cause of death in AIDS patients. Tests that are usually reliable in diagnosing neurologic diseases may not be reliable in patients with AIDS. The management of AIDS is a multidisciplinary effort, and the neurologist should fulfill a role in the management team. ImagesFigure 1Figure 2Figure 3Figure 4 PMID:3334059

  3. Complications of Cushing's syndrome: state of the art.

    PubMed

    Pivonello, Rosario; Isidori, Andrea M; De Martino, Maria Cristina; Newell-Price, John; Biller, Beverly M K; Colao, Annamaria

    2016-07-01

    Cushing's syndrome is a serious endocrine disease caused by chronic, autonomous, and excessive secretion of cortisol. The syndrome is associated with increased mortality and impaired quality of life because of the occurrence of comorbidities. These clinical complications include metabolic syndrome, consisting of systemic arterial hypertension, visceral obesity, impairment of glucose metabolism, and dyslipidaemia; musculoskeletal disorders, such as myopathy, osteoporosis, and skeletal fractures; neuropsychiatric disorders, such as impairment of cognitive function, depression, or mania; impairment of reproductive and sexual function; and dermatological manifestations, mainly represented by acne, hirsutism, and alopecia. Hypertension in patients with Cushing's syndrome has a multifactorial pathogenesis and contributes to the increased risk for myocardial infarction, cardiac failure, or stroke, which are the most common causes of death; risks of these outcomes are exacerbated by a prothrombotic diathesis and hypokalaemia. Neuropsychiatric disorders can be responsible for suicide. Immune disorders are common; immunosuppression during active disease causes susceptibility to infections, possibly complicated by sepsis, an important cause of death, whereas immune rebound after disease remission can exacerbate underlying autoimmune diseases. Prompt treatment of cortisol excess and specific treatments of comorbidities are crucial to prevent serious clinical complications and reduce the mortality associated with Cushing's syndrome. PMID:27177728

  4. Possible complications of subclavian crush syndrome

    PubMed Central

    Said, S.A.M.; Ticheler, C.H.J.M.; Stassen, C.M.; Derks, A.; Droste, H.T.

    2005-01-01

    A 70-year-old woman with symptomatic Mobitz type II atrioventricular block underwent implantation of a dual-chamber pacemaker 11 years ago. The leads were inserted through a percutaneous puncture of the right subclavian vein, using standard techniques. Both leads were passive fixation leads. Due to battery failure and end of life criteria, the pulse generator (PG) had been routinely replaced six years previously. Predischarge pacemaker control revealed normal pacing, sensing thresholds and impedance for both leads. Because of a syncopal attack subsequent to lead fractures, most likely secondary to right subclavian crush syndrome (SCS) of both leads, she underwent a double lead re-implantation one year after PG replacement by access via left subclavian vein puncture. After a symptom-free period of few years she was re-analysed because of palpitations, dizziness, angina pectoris and tiredness. Pulmonary embolisation and myocardial perfusion defects were detected utilising scintigraphic techniques. Chest X-ray revealed the crushed atrial lead dislocated from the right subclavian region and lodged into the right ventricle towards the inferior septum. Because she was symptomatic, a retrieval technique was applied and the crushed atrial lead was pulled back from the right ventricle and securely fixed to its former position. On maintenance medical treatment, she remains well. ImagesFigure 1BFigure 2AFigure 2BFigure 2CFigure 3Figure 4 PMID:25696461

  5. Descending necrotizing mediastinitis complicating hyperimmunoglobulin E syndrome

    PubMed Central

    Hayakawa, Takamitsu; Neyatani, Hiroshi; Funai, Kazuhito

    2016-01-01

    Abstract Descending necrotizing mediastinitis (DNM) is a potentially lethal disease that originates from a deep neck infection (DNI); it is often associated with an immunocompromised state. Hyperimmunoglobulin E syndrome (HIES) is an extremely rare complex immune deficiency characterized by recurrent abscesses of staphylococcal etiology. A rare case of a 34‐year‐old woman wherein HIES putatively promoted the development of DNI into DNM is described. She presented with cervical purulent lymphadenitis and retropharyngeal abscess. Despite immediate cervical drainage and use of broad‐spectrum antibiotics, she developed DNI and subsequently DNM. Mediastinal drainage with thoracotomy and subsequent deep neck drainage were performed. Bacterial culture of the abscess isolated methicillin‐resistant Staphylococcus aureus (MRSA). Although a postoperative recurrent deep neck abscess required a second surgery, we succeeded in conservative remission of recurrent mediastinal abscess with long‐term use of anti‐MRSA drugs. Sufficient drainage under thoracotomy and robust administration of postoperative antibiotics resulted in successful management of HIES‐associated DNM. PMID:27512566

  6. Myhre syndrome: Clinical features and restrictive cardiopulmonary complications.

    PubMed

    Starr, Lois J; Grange, Dorothy K; Delaney, Jeffrey W; Yetman, Anji T; Hammel, James M; Sanmann, Jennifer N; Perry, Deborah A; Schaefer, G Bradley; Olney, Ann Haskins

    2015-12-01

    Myhre syndrome, a connective tissue disorder characterized by deafness, restricted joint movement, compact body habitus, and distinctive craniofacial and skeletal features, is caused by heterozygous mutations in SMAD4. Cardiac manifestations reported to date have included patent ductus arteriosus, septal defects, aortic coarctation and pericarditis. We present five previously unreported patients with Myhre syndrome. Despite varied clinical phenotypes all had significant cardiac and/or pulmonary pathology and abnormal wound healing. Included herein is the first report of cardiac transplantation in patients with Myhre syndrome. A progressive and markedly abnormal fibroproliferative response to surgical intervention is a newly delineated complication that occurred in all patients and contributes to our understanding of the natural history of this disorder. We recommend routine cardiopulmonary surveillance for patients with Myhre syndrome. Surgical intervention should be approached with extreme caution and with as little invasion as possible as the propensity to develop fibrosis/scar tissue is dramatic and can cause significant morbidity and mortality. PMID:26420300

  7. Gradenigo's syndrome--a rare complication of otitis media.

    PubMed

    Jagadeesan, Padmaja; Madeswaran, K; Thiruppathy, S P; Kalairajan, D; Inbasekaran, V

    2002-11-01

    Petrous apicitis is a rare intracranial complication of otitis media. A 4-year-old female child was presented with persistent ear discharge, retro-orbital pain and lateral rectus palsy (triad of Gradenigo's syndrome). A right temporal burr-hole was placed and tapping was done under antibiotic coverage. Turbid cerebrospinal fluid could be drained. Follow-up could not be done as the patient refused treatment and was discharged against medical advice. PMID:12797641

  8. Megacystis microcolon intestinal hypoperistalsis syndrome complicated by perforation.

    PubMed

    Jain, Vishal K; Garge, Saurabh; Singh, Sangram; Lahoti, Brijesh

    2011-01-01

    Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a quite rare congenital and fatal disease. The disease is three to four times more prevalent in females than males. Most of the cases die within the early months of their lives. We present the case of a newborn male with antenatal ultrasound suggesting the diagnosis of MMIHS. The case was admitted for perforation complicated with prematurity after birth. This case is th e only one where MMIHS presented as perforation. PMID:21478590

  9. Case Report: Cervical chondrocalcinosis as a complication of Gitelman syndrome

    PubMed Central

    Iqbal, Zahra; Mead, Paul; Sayer, John A.

    2016-01-01

    Gitelman syndrome is an inherited tubulopathy leading to a hypokalaemic metabolic alkalosis with hypomagnesaemia and hypocalciuria. Most cases are due to mutations in SLC12A3, encoding the apical thiazide sensitive co-transporter in the distal convoluted tubule. Musculoskeletal effects of Gitelman syndrome are common, including muscle weakness, tetany and cramps. Chronic hypomagnesaemia can lead to chondrocalcinosis, which often affects knees but can affect other joints. Here we present a case of Gitelman syndrome complicated by cervical chondrocalcinosis leading to neck pain and numbness of the fingers. Treatments directed at correcting both hypokalaemia and hypomagnesaemia were initiated and allowed conservative non-surgical management of the neck pain. Recognition of chondrocalcinosis is important and treatments must be individualised to correct the underlying hypomagnesaemia. PMID:27303630

  10. Case Report: Cervical chondrocalcinosis as a complication of Gitelman syndrome.

    PubMed

    Iqbal, Zahra; Mead, Paul; Sayer, John A

    2016-01-01

    Gitelman syndrome is an inherited tubulopathy leading to a hypokalaemic metabolic alkalosis with hypomagnesaemia and hypocalciuria. Most cases are due to mutations in SLC12A3, encoding the apical thiazide sensitive co-transporter in the distal convoluted tubule. Musculoskeletal effects of Gitelman syndrome are common, including muscle weakness, tetany and cramps. Chronic hypomagnesaemia can lead to chondrocalcinosis, which often affects knees but can affect other joints. Here we present a case of Gitelman syndrome complicated by cervical chondrocalcinosis leading to neck pain and numbness of the fingers. Treatments directed at correcting both hypokalaemia and hypomagnesaemia were initiated and allowed conservative non-surgical management of the neck pain. Recognition of chondrocalcinosis is important and treatments must be individualised to correct the underlying hypomagnesaemia. PMID:27303630

  11. Grisel's syndrome: a rare complication following traditional uvulectomy

    PubMed Central

    Elyajouri, Abdelhakim; Assermouh, Abdellah; Abilkacem, Rachid; Agadr, Aomar; Mahraoui, Chafiq

    2015-01-01

    A case is reported of an eight-month-old female patient who had traditional uvulectomy for sore throat complicated by Grisel's syndrome. She was admitted into the hospital one week after uvulectomy with Torticolis. Grisel's syndrome is a nontraumatic atlantoaxial subluxation, usually secondary of an infection or an inflammation at the head and neck area, or after surgery in the same area. Patients typically suffer from painful torticollis. Diagnosis of Grisel's syndrome is largely based on suspicion of the patient who has recently undergone surgery or has a history of an infection in head and neck area. Physical examination and imaging techniques assist in diagnosis. Thus, clinicians should be aware of acute nontraumatic torticollis if patient had a recent surgery in the head or neck area or undergone an upper respiratory tract infection. In this paper, a case of an eight-month-old female patient who had Grisel's syndrome after uvulectomy is discussed. This case is reported to highlight this neurogical threatening complication following traditional uvulectomy as well as highlighting the unnecessary morbidity and mortality associated with this persisting mode of treatment in Africa. PMID:26090020

  12. Sjögren's syndrome complicated with Fanconi syndrome and Hashimoto's thyroiditis: Case report and literature review.

    PubMed

    Shi, Mingmin; Chen, Lei

    2016-06-01

    We report a unique case of Sjögren's syndrome complicated with Fanconi syndrome and Hashimoto's thyroiditis in a 53-year-old Chinese woman, initially found to have proteinuria, fatigue and multiple old costal fractures. Distal tubular dysfunction is the most common renal damage in Sjögren's syndrome, while Fanconi syndrome (which is caused by proximal tubular dysfunction) and Hypothyroidism are rare complications of Sjögren's syndrome. PMID:26966155

  13. Neurologic complications of cerebral angiography in childhood moyamoya syndrome.

    PubMed

    Robertson, R L; Chavali, R V; Robson, C D; Barnes, P D; Eldredge, E A; Burrows, P E; Scott, R M

    1998-11-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher's exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. PMID:9799310

  14. A case of metastatic testicular cancer complicated by tumour lysis syndrome and choriocarcinoma syndrome.

    PubMed

    Kawai, Koji; Takaoka, Ei-Ichiro; Naoi, Makito; Mori, Kensaku; Minami, Manabu; Shimazui, Toru; Akaza, Hideyuki

    2006-10-01

    A 26-year-old man was referred to our hospital for treatment of metastatic testicular cancer. The pathological diagnosis was choriocarcinoma with seminoma. Sequential computerized tomography examinations revealed rapidly progressing bulky liver metastases and a lung metastasis. Chemotherapy with bleomycin, etoposide and cisplatin (BEP) was started on the day of admission. Subsequently, the patient suffered from tumour lysis syndrome (TLS) and massive haemorrhage at metastatic sites. The latter complication is also called choriocarcinoma syndrome. To our knowledge, this is the first case report of testicular cancer complicated with both critical conditions. Intensive care and radiological intervention barely prevented a fatal outcome. The urological oncologist should be aware of the potential complications TLS and choriocarcinoma syndrome in cases of rapidly progressive and high-volume choriocarcinoma. PMID:16935862

  15. Osteomyelitis: A rare complication of pancreatitis and PPP-syndrome.

    PubMed

    Langenhan, Ronny; Reimers, Niklas; Probst, Axel

    2016-03-01

    Pancreatic diseases can be accompanied by periarthritis with bone necrosis and panniculitis (PPP-syndrome). It is postulated that this is caused by systemic activity of pancreatic enzymes leading to microcirculatory disturbances and fat necrosis. The morbidity and mortality of the PPP-syndrome is high. Successful treatment of pancreatitis can lead to resolution of accompanying panniculitis and periarthritis without adverse sequelae, but weeks or months after pancreatitis, asymptomatic necrosis of the bone may become symptomatic by fracturing spontaneously. In this report, we also describe osteomyelitis as a severe septic complication of bone necrosis caused by pancreatitis, in one case as acute tissue necrosis and in another case months after pancreatitis spread haematogenously. PMID:26471414

  16. Down syndrome: perinatal complications and counseling experiences in 216 patients.

    PubMed

    Spahis, J K; Wilson, G N

    1999-06-25

    Two hundred and sixteen infant evaluations were selected for analysis from those of 669 outpatients (930 total visits) at a weekly Down syndrome clinic. Each record contained perinatal history and physical examination results, and 191 of the 216 included a systematic interview regarding parental experiences with diagnosis and counseling. Gastrointestinal problems (77% of neonates), cardiac anomalies (38%), and hematologic problems (11%) were the most common complications; cited problems included anal stenosis (11%), which is described as a newly recognized cause of constipation in early infancy. Counseling experiences were positive in 66 (34%) of the 191 parent interviews, with counselor knowledge, timing, setting, and attitudes being cited as key factors. Appropriate counseling and surveillance for gastrointestinal problems can greatly facilitate parental adjustment to the neonate with Down syndrome. PMID:10559764

  17. Locus coeruleus syndrome as a complication of tectal surgery.

    PubMed

    Kronenburg, Annick; Spliet, Wim G; Broekman, Marike; Robe, Pierre

    2015-01-01

    We describe a case of a 48-year-old woman who underwent a resection of a tectal pilocytic astrocytoma complicated by a sequence of fluctuating consciousness, psychosis with complex hallucinations and lasting sleeping disturbances in which she vividly acts out her dreams. Based on the clinical and anatomical evidence of this case, we propose the term locus coeruleus syndrome to describe this association of iatrogenic symptoms. Along with those of the locus coeruleus, lesions of the dorsal raphe nucleus, ventral tegmentum, substantia nigra pars compacta, the superior colliculus and other peduncular lesions (such as peduncular hallucinosis) are involved in the regulation of sleep-wake/arousal, behaviour, sleeping disorders and rapid eye movement atonia. However, iatrogenic lesion of the locus coeruleus could explain the complications on all levels in our patient. PMID:25903199

  18. Buried bumper syndrome: A complication of percutaneous endoscopic gastrostomy

    PubMed Central

    Cyrany, Jiri; Rejchrt, Stanislav; Kopacova, Marcela; Bures, Jan

    2016-01-01

    Percutaneous endoscopic gastrostomy (PEG) is a widely used method of nutrition delivery for patients with long-term insufficiency of oral intake. The PEG complication rate varies from 0.4% to 22.5% of cases, with minor complications being three times more frequent. Buried bumper syndrome (BBS) is a severe complication of this method, in which the internal fixation device migrates alongside the tract of the stoma outside the stomach. Excessive compression of tissue between the external and internal fixation device of the gastrostomy tube is considered the main etiological factor leading to BBS. Incidence of BBS is estimated at around 1% (0.3%-2.4%). Inability to insert, loss of patency and leakage around the PEG tube are considered to be a typical symptomatic triad. Gastroscopy is indicated in all cases in which BBS is suspected. The depth of disc migration in relation to the lamina muscularis propria of the stomach is critical for further therapy and can be estimated by endoscopic or transabdominal ultrasound. BBS can be complicated by gastrointestinal bleeding, perforation, peritonitis, intra-abdominal and abdominal wall abscesses, or phlegmon, and these complications can lead to fatal outcomes. The most important preventive measure is adequate positioning of the external bolster. A conservative approach should be applied only in patients with high operative risk and dismal prognosis. Choice of the method of release is based on the type of the PEG set and depth of disc migration. A disc retained inside the stomach and completely covered by the overgrowing tissue can be released using some type of endoscopic dissection technique (needle knife, argon plasma coagulation, or papillotome through the cannula). Proper patient selection and dissection of the overgrowing tissue are the major determinants for successful endoscopic therapy. A disc localized out of the stomach (lamina muscularis propria) should be treated by a surgeon. PMID:26811611

  19. Complications from carcinoid syndrome: review of the current evidence

    PubMed Central

    Mota, José Mauricio; Sousa, Luana Guimarães; Riechelmann, Rachel P

    2016-01-01

    Patients with well-differentiated neuroendocrine tumours may develop carcinoid syndrome (CS), which is characterised by flushing, abdominal cramps, diarrhoea, and bronchospasms. In this scenario, long-term secretion of vasoactive substances—serotonin, tachynins, and others, may induce fibrogenic responses in local or distant tissues, leading to complications such as carcinoid heart disease (CHD), mesenteric and/or retroperitoneal fibrosis. Rare cases of lung/pleural fibrosis and scleroderma have also been described. Despite it not being well described yet, current evidence suggests the pathogenesis of such fibrogenic complications relies on signalling through 5-HT2B and TGF-β1. Medical management is still very limited and lacks prospective and randomised studies for definitive recommendations. Surgical procedures remain the best definitive treatment option for CHD and abdominal fibrosis. Recently, cognitive impairment has also been described as a potential consequence of CS. This review critically discusses the literature concerning the epidemiology, pathogenesis, clinical features, diagnosis, and treatment options for CS-related long-term complications. PMID:27594907

  20. Complications from carcinoid syndrome: review of the current evidence.

    PubMed

    Mota, José Mauricio; Sousa, Luana Guimarães; Riechelmann, Rachel P

    2016-01-01

    Patients with well-differentiated neuroendocrine tumours may develop carcinoid syndrome (CS), which is characterised by flushing, abdominal cramps, diarrhoea, and bronchospasms. In this scenario, long-term secretion of vasoactive substances-serotonin, tachynins, and others, may induce fibrogenic responses in local or distant tissues, leading to complications such as carcinoid heart disease (CHD), mesenteric and/or retroperitoneal fibrosis. Rare cases of lung/pleural fibrosis and scleroderma have also been described. Despite it not being well described yet, current evidence suggests the pathogenesis of such fibrogenic complications relies on signalling through 5-HT2B and TGF-β1. Medical management is still very limited and lacks prospective and randomised studies for definitive recommendations. Surgical procedures remain the best definitive treatment option for CHD and abdominal fibrosis. Recently, cognitive impairment has also been described as a potential consequence of CS. This review critically discusses the literature concerning the epidemiology, pathogenesis, clinical features, diagnosis, and treatment options for CS-related long-term complications. PMID:27594907

  1. Large Gastric Perforation in Carmi Syndrome: A Morbid Complication in a Rare Association

    PubMed Central

    Krishnan, L; Kuruvila, S

    2012-01-01

    The association between epidermolysis bullosa (EB) and congenital pyloric atresia (CPA) named Carmi Syndrome is rare. We report unusual and morbid complication of gastric perforation resulting in peritonitis in a preterm neonate born with Carmi Syndrome. PMID:26023416

  2. Management and Complications of Short Bowel Syndrome: an Updated Review.

    PubMed

    Carroll, Robert E; Benedetti, Enrico; Schowalter, Joseph P; Buchman, Alan L

    2016-07-01

    Short bowel syndrome (SBS) is defined as loss of bowel mass from surgical resection, congenital defects, or disease. Intestinal failure (IF) includes the subset of SBS unable to meet nutrition needs with enteral supplements and requires parenteral nutrition (PN). The parenteral treatment of SBS is now a half-century old. Recent pharmacologic treatment (GLP-2 analogues) has begun to make a significant impact in the care and ultimate management of these patients such that the possibility of reducing PN requirements in formerly PN-dependent patients is a now a real possibility. Finally, newer understanding and possible treatment for some of the complications related to IF have more recently evolved and will be an emphasis of this report. This review will focus on developments over the last 10 years with the goal of updating the reader to new advances in our understanding of the care and feeding of the SBS patient. PMID:27324885

  3. Gastrointestinal variant of Lemierre's syndrome complicating ruptured appendicitis.

    PubMed

    Akhrass, Fadi Al; Abdallah, Lina; Berger, Steven; Sartawi, Rami

    2015-01-01

    Fusobacterium necrophorum is a non-spore-forming, obligate anaerobic, filamentous, gramnegative bacillus that frequently colonizes the human oral cavity, respiratory tract, and gastrointestinal tract. Fusobacterium species have rarely been implicated in cases of gastrointestinal variant of Lemierre's syndrome. We describe a case of F. necrophorum bacteremia associated with suppurative porto-mesenteric vein thrombosis (PVT) following acute ruptured appendicitis. In addition, we list the documented twelve cases of Fusobacterium pylephlebitis. Recanalization of the porto-mesenteric veins and relief of the extrahepatic portal hypertension were achieved with early empiric antibiotic and local thrombolytic therapy. Our patient's case underscores the importance of recognizing Fusobacterium bacteremia as a possible cause of suppurative PVT after disruption of the gastrointestinal mucosa following an acute intraabdominal infectious process. Early treatment of this condition using anticoagulation and endovascular thrombolysis as adjunctive therapies may prevent PVT complications. PMID:26793462

  4. HELLP syndrome: a diagnostic conundrum with severe complications.

    PubMed

    Rao, Devika; Chaudhari, Nikulkumar Kumar; Moore, Robert Michael; Jim, Belinda

    2016-01-01

    The HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome is believed to be part of the spectrum of pre-eclampsia, which falls within the category of hypertensive disorders of pregnancy. Maternal and fetal complications are more severe in HELLP as opposed to pre-eclampsia alone. We describe a 26-year-old primigravida woman with no medical history who presents with signs of HELLP with marked transaminitis and mild disseminated intravascular coagulation at 35 weeks of gestation who required emergent delivery of the fetus; the patient also sustained acute kidney injury requiring continuous veno-venous hemodiafiltration and a prolonged intensive care unit admission. Remarkably, with supportive care, all laboratory derangements, including renal function, normalised after 4 weeks. We discuss the diagnostic conundrum when faced with the possible diagnosis of HELLP in discriminating from its many imitators in order to assume proper treatment. PMID:27535735

  5. Endovascular treatment of multiple aneurysms complicating Cogan syndrome.

    PubMed

    Angiletta, Domenico; Wiesel, Paola; Pulli, Raffaele; Marinazzo, Davide; Bortone, Alessandro Santo; Regina, Guido

    2015-02-01

    To report the use of endografts to manage multiple aneurysms due to Cogan syndrome (CS). A 38-year-old woman with descending thoracic aorta and right common carotid artery aneurysms due to CS was treated with endovascular grafts. After 4 years, angio computed tomography scan demonstrated complete exclusion of the aneurysms with no signs of endoleak, whereas echo color Doppler showed patency of the carotid graft, no signs of restenosis, no progression of the disease in the landing zones, and complete aneurysm exclusion. Endovascular repair seems to have favorable long-term outcomes and should be considered a viable alternative to surgery in unfit for open surgery patients, even if they are young, and when the aneurysm size and location would pose a higher risk of perioperative and postoperative complications after an open surgical procedure. PMID:25462550

  6. Gastrointestinal variant of Lemierre's syndrome complicating ruptured appendicitis

    PubMed Central

    Akhrass, Fadi Al; Abdallah, Lina; Berger, Steven; Sartawi, Rami

    2015-01-01

    Fusobacterium necrophorum is a non-spore-forming, obligate anaerobic, filamentous, gramnegative bacillus that frequently colonizes the human oral cavity, respiratory tract, and gastrointestinal tract. Fusobacterium species have rarely been implicated in cases of gastrointestinal variant of Lemierre's syndrome. We describe a case of F. necrophorum bacteremia associated with suppurative porto-mesenteric vein thrombosis (PVT) following acute ruptured appendicitis. In addition, we list the documented twelve cases of Fusobacterium pylephlebitis. Recanalization of the porto-mesenteric veins and relief of the extrahepatic portal hypertension were achieved with early empiric antibiotic and local thrombolytic therapy. Our patient's case underscores the importance of recognizing Fusobacterium bacteremia as a possible cause of suppurative PVT after disruption of the gastrointestinal mucosa following an acute intraabdominal infectious process. Early treatment of this condition using anticoagulation and endovascular thrombolysis as adjunctive therapies may prevent PVT complications. PMID:26793462

  7. Acquired hemophilia complicated by cardiorenal syndrome type 3

    PubMed Central

    Sharma, Rakesh; Dash, Sananta Kumar; Chawla, Rajesh; Kansal, Sudha; Agrawal, Devender Kumar; Dua, Harsh

    2013-01-01

    Development of autoantibodies against coagulation factor VIII (FVIII) leads to a rare condition defined as acquired hemophilia (AH). If not diagnosed and treated early, AH may be associated with high mortality and morbidity. A 65-year-old woman presented with history of macrohematuria, acute renal failure, cardiogenic shock, and acute respiratory failure. Blood investigation revealed azotemia, prolonged activated partial thromboplastin time (aPTT), coagulation FVIII level of <1%, and presence of FVIII inhibitor. Echocardiography showed global hypokinesia and ultrasonography and computed tomography (CT) revealed bilateral hydroureteronephrosis. The final diagnosis was acquired hemophilia A, complicated by acute obstructive renal failure and cardiorenal syndrome (CRS) type 3. Patient was managed with mechanical ventilation, heparin-free hemodialysis, negative fluid balance, recombinant activated factor VII, and prednisolone. Hematuria was relieved, renal function improved, and cardiac function showed improvement on repeat echocardiography. Patient was discharged on prednisolone with subsequent follow ups. PMID:24501492

  8. A new mouse model of metabolic syndrome and associated complications

    PubMed Central

    Wang, Yun; Zheng, Yue; Nishina, Patsy M; Naggert, Jürgen K.

    2010-01-01

    Metabolic Syndrome (MS) encompasses a clustering of risk factors for cardiovascular disease, including obesity, insulin resistance, and dyslipidemia. We characterized a new mouse model carrying a dominant mutation, C57BL/6J-Nmf15/+ (B6-Nmf15/+), which develops additional complications of MS such as adipose tissue inflammation and cardiomyopathy. A backcross was used to genetically map the Nmf15 locus. Mice were examined in the CLAMS™ animal monitoring system, and dual energy X-ray absorptiometry and blood chemistry analyses were performed. Hypothalamic LepR, SOCS1 and STAT3 phosphorylation were examined. Cardiac function was assessed by Echo- and Electro Cardiography. Adipose tissue inflammation was characterized by in situ hybridization and measurement of Jun kinase activity. The Nmf15 locus mapped to distal mouse chromosome 5 with a LOD score of 13.8. Nmf15 mice developed obesity by 12 weeks of age. Plasma leptin levels were significantly elevated in pre-obese Nmf15 mice at 8 weeks of age and an attenuated STAT3 phosphorylation in the hypothalamus suggests a primary leptin resistance. Adipose tissue from Nmf15 mice showed a remarkable degree of inflammation and macrophage infiltration as indicated by expression of the F4/80 marker and increased phosphorylation of JNK1/2. Lipidosis was observed in tubular epithelial cells and glomeruli of the kidney. Nmf15 mice demonstrate both histological and pathophysiological evidence of cardiomyopathy. The Nmf15 mouse model provides a new entry point into pathways mediating leptin resistance and obesity. It is one of few models that combine many aspects of metabolic syndrome and can be useful for testing new therapeutic approaches for combating obesity complications, particularly cardiomyopathy. PMID:19398498

  9. Abdominal compartment syndrome: an underrated complication in pediatric kidney transplantation.

    PubMed

    Fontana, I; Bertocchi, M; Centanaro, M; Varotti, G; Santori, G; Mondello, R; Tagliamacco, A; Cupo, P; Barabani, C; Palombo, D

    2014-09-01

    The transplantation of a large kidney in small children can lead to many complications, including an underrated complication known as abdominal compartment syndrome (ACS), which is defined as intra-abdominal pressure (IAP)≥20 mm Hg with dysfunction of at least one thoracoabdominal organ. Presenting signs of ACS include firm tense abdomen, increased peak inspiratory pressures, oliguria, and hypotension. Between June 1, 1985, and September 30, 2013, our center performed 420 kidney transplants (deceased/living related donors: 381/39) in 314 pediatric recipients (female/male: 147/167). ACS occurred in 9 pediatric patients (weight<15 kg) who received a large kidney from adult donors. In 1 case, the patient underwent abdominal decompression with re-exploration and closure with mesh in the immediate postoperative period. In a second case, the patient developed a significant respiratory compromise with hemodynamic instability necessitating catecholamines, sedation, and assisted ventilation. For small children transplanted with a large kidney, an early diagnosis of ACS represents a critical step. From 2005 we have measured IAP during transplantation via urinary bladder pressure, and immediately after wound closure we use intraoperative and postoperative duplex sonography to value flow dynamics changes. We recommend that bladder pressure should be routinely checked in small pediatric kidney recipients who are transplanted with a large graft. PMID:25242763

  10. A complication of xanthogranulomatous cholecystitis with Mirizzi syndrome.

    PubMed

    Zhang, H-Y; Cao, X-D; Chen, J-J; Luo, Y-Q; Wang, X-C

    2015-05-01

    A patient had right upper quadrant pain with sclera was transferred from emergency room to the hospital, she was proposed to have acute cholecystitis, gallstones, obstructive jaundice, and a four-year history of gallbladder stones. The NMR results showed that the gallbladder was significantly enlarged and the gallbladder wall was thickening irregularly. The liver morphology was not abnormal except with extensive intrahepatic bile duct dilatation. The MRCP results demonstrated that the intrahepatic bile ducts were significant expanded. The ERCP results showed that duodenal stenosis and extra-hepatic bile duct stenosis. We placed a plastic stent of 8.5Fr and 12 cm in length in the hepatic duct, and after biliary plastic stent placement, jaundice was rapidly reduced and liver function was improved significantly. A surgery was performed and the final pathologic diagnosis is a complication of Xanthogranulomatous cholecystitis with Mirizzi syndrome. After the surgery of cholecystectomy and a bile duct repair were performed, the patient was recovered well. Conclusively, if a patient was diagnosed as biliary stricture, a biliary metal stent should not be placed until pathological diagnosis of malignancy. PMID:26044215

  11. Complications after spinal anesthesia in adult tethered cord syndrome.

    PubMed

    Liu, Jing-Jie; Guan, Zheng; Gao, Zhen; Xiang, Li; Zhao, Feng; Huang, Sheng-Li

    2016-07-01

    Since little has been reported about complications of spinal anesthesia in adult tethered cord syndrome (TCS), we sought to delineate the characteristics of the condition.A total of 4 cases of adult TCS after spinal anesthesia were reviewed. The medical charts of the patients were obtained. Anesthesia, which was combined spinal and epidural anesthesia or spinal anesthesia was performed, and follow-up were carried out in all patients.The most common neurological symptom of adult TCS before surgery was occasional severe pain in back, perineal region, or legs. Frequent micturition, diminished knee and ankle reflexes, and difficulty in bending were exhibited in partial patients. Paraesthesia of perineal region or/and lower extremities existed 2 to 3 days after spinal anesthesia in all the cases. Weakness of lower extremities existed in 1 case. Lumbar magnetic resonance imaging showed the low location of conus medullaris. At follow-up, 3 cases recovered completely within 3 weeks, and 1 case underwent permanent disability.These cases suggest anesthesiologists and surgeons alert to the association of adult TCS and spinal anesthesia. Spinal anesthesia should be prohibited in patients with adult TCS to prevent neurological damages. PMID:27442670

  12. Complications and challenges associated with polycystic ovary syndrome: current perspectives

    PubMed Central

    Palomba, Stefano; Santagni, Susanna; Falbo, Angela; La Sala, Giovanni Battista

    2015-01-01

    Polycystic ovary syndrome (PCOS) represents the most common endocrine dysfunction in fertile women and it is considered a heterogeneous and multifaceted disorder, with multiple reproductive and metabolic phenotypes which differently affect the early- and long-term syndrome’s risks. Women with PCOS present an adverse reproductive profile, including a high risk of pregnancy-induced hypertension, preeclampsia, and gestational diabetes mellitus. Patients with PCOS present not only a higher prevalence of classic cardiovascular risk factors, such as hypertension, dyslipidemia, and type-2 diabetes mellitus, but also of nonclassic cardiovascular risk factors, including mood disorders, such as depression and anxiety. Moreover, at the moment, clinical data on cardiovascular morbidity and mortality in women with PCOS are controversial. Finally, women with PCOS show an increased risk of endometrial cancer compared to non-PCOS healthy women, particularly during premenopausal period. Currently, we are unable to clarify if the increased PCOS early- and long-term risks are totally due to PCOS per se or mostly due to obesity, in particular visceral obesity, that characterized the majority of PCOS patients. In any case, the main endocrine and gynecological scientific societies agree to consider women with PCOS at increased risk of obstetric, cardiometabolic, oncology, and psychological complications throughout life, and it is recommended that these women be accurately assessed with periodic follow-up. PMID:26261426

  13. Dialysis disequilibrium syndrome: A preventable fatal acute complication.

    PubMed

    Mah, D Y; Yia, H J; Cheong, W S

    2016-04-01

    Dialysis disequilibrium syndrome (DDS) is a neurological disorder with varying severity that is postulated to be associated with cerebral oedema. We described a case of DDS resulting in irreversible brain injury and death following acute haemodialysis. A 13-year-old male with no past medical history and weighing 30kg, presented to hospital with severe urosepsis complicated by acute kidney injury (Creatinine 1422mmol/L; Urea 74.2mmol/L, Potassium 6.3mmol/L, Sodium 137mmol/L) and severe metabolic acidosis (pH 6.99, HC03 1.7mmol/L). Chest radiograph was normal. Elective intubation was done for respiratory distress. Acute haemodialysis performed due to refractory metabolic acidosis. Following haemodialysis, he became hypotensive which required inotropes. His Riker's score was low with absence of brainstem reflexes after withholding sedation. CT Brain showed generalised cerebral oedema consistent with global hypoxic changes involving the brainstem. The symptoms of DDS are caused by water movement into the brain causing cerebral oedema. Two theories have been proposed: reverse osmotic shift induced by urea removal and a fall in cerebral intracellular pH. Prevention is the key to the management of DDS. It is important to identify high risk patients and haemodialysis with reduced dialysis efficacy and gradual urea reduction is recommended. Patients who are vulnerable to DDS should be monitored closely. Low efficiency haemodialysis is recommended. Acute peritoneal dialysis might be an alternative option, but further studies are needed. PMID:27326954

  14. Effect of plasma exchange on refractory hemophagocytic syndrome complicated with myelodysplastic syndrome.

    PubMed

    Satomi, A; Nagai, S; Nagai, T; Niikura, K; Ideura, T; Ogata, H; Akizawa, T

    1999-11-01

    A case of hemophagocytic syndrome (HPS) refractory to corticosteroid therapy was successfully treated by plasma exchange. The patient was a 56-year-old woman who had undergone regular hemodialysis for 10 years for complicated myelodysplastic syndrome (MDS) and then had had lung tuberculosis. After the onset of tuberculosis, she suffered from HPS and was treated by antituberculosis agents and high dose corticosteroid administration without any effect on the HPS. After adding a series of plasma exchanges, the HPS improved gradually, and her MDS began to respond to corticosteroid therapy. Plasma hypercytokinemia due to HPS was corrected by plasma exchange, and the correction of a high level of plasma inflammatory cytokine was considered to be one of the contributing factors for the improvement of HPS. These results suggest that therapeutic plasma exchange should be considered as a therapeutic tool for HPS refractory to conventional therapy. PMID:10608726

  15. Holmium laser use in the treatment of selected dry eye syndrome complications

    NASA Astrophysics Data System (ADS)

    Kecik, Dariusz; Kecik, Tadeusz; Kasprzak, Jan; Kecik, Mariusz

    1996-03-01

    The authors present initial results of treatment selected complications of dry eye syndrome with holmium laser. The lacrimal puncta obliteration and coagulation of the corneal ulcer surface were done.

  16. Erythema multiforme

    MedlinePlus

    Lyell's syndrome; Stevens-Johnson syndrome; Erythema multiforme minor; Erythema multiforme major ... more severe. It is also known as Stevens-Johnson syndrome. This form is usually caused by reactions ...

  17. Long-term complications after surgical correction of Laubry-Pezzi syndrome

    PubMed Central

    Sbizzera, Marc; Cosset, Benoit; Koffel, Catherine; Obadia, Jean François; Robin, Jacques

    2016-01-01

    Laubry-Pezzi syndrome is a rare congenital heart disease (CHD) combining a ventricular septal defect (VSD) and an aortic regurgitation (AR) due to a prolapse of the right coronary or, less frequently, non-coronary cusp. In the literature there are very few reports of long-term complications after surgical correction of this syndrome. We present the late occurrence of an aortic root pseudoaneurysm and a residual VSD in a patient with Laubry-Pezzi syndrome operated during childhood. PMID:27076977

  18. Hemoptysis Complicating Scimitar Syndrome: From Diagnosis to Treatment

    SciTech Connect

    Nedelcu, Cosmina; Carette, Marie-France; Parrot, Antoine; Hammoudi, Nadjib; Marsault, Claude; Khalil, Antoine

    2008-07-15

    We report the case of a 24-year-old patient with known scimitar syndrome presenting with hemoptysis. Multidetector CT angiography showed the scimitar vein draining the right lung to the inferior vena cava associated with right lower lobe bronchopulmonary sequestration. The presence of hemoptysis associated with scimitar syndrome is likely secondary to an anomalous systemic arterial blood supply. We treated this patient successfully with transcatheter occlusion of the anomalous feeding arterial vessels.

  19. [Psychiatric complications of alcoholism: alcohol withdrawal syndrome and other psychiatric disorders].

    PubMed

    Maciel, Cláudia; Kerr-Corrêa, Florence

    2004-05-01

    Alcohol withdrawal syndrome is an acute condition secondary to total or partial reduction of alcohol consumption, characterized by self limited signs and symptoms and different degrees of severity. It can be complicated by several clinical and/or other psychiatric related problems. The objective of this article is to review the most important psychiatric complications to alcohol withdrawal syndrome as well as other psychiatric disorders associated with alcohol dependence as Wernicke Korsakoff and Marchiava Bignami syndromes. We aim to promote early diagnosis and treatment of these conditions, minimizing morbidity and mortality associated with them. PMID:15729445

  20. Lemierre's syndrome: a serious complication of a routine dental procedure.

    PubMed

    Ghaly, B; Bertram, A; Naim, A

    2013-06-01

    Lemierre's syndrome is the clinical presentation characterized by oropharyngeal or tonsillar infection, anaerobic septicaemia often with distant septic emboli, and clinical or radiological evidence of thrombosis of the internal jugular vein. We report a case of Lemierre's syndrome in a 44-year-old type II diabetic male. This case is unique for two reasons. Firstly, we believe it is the first reported iatrogenic case following a routine dental procedure. Secondly, the internal jugular vein thrombosis was found to occur on the contralateral side to the primary infection. PMID:23713647

  1. Tumor Lysis Syndrome: An Unreported Complication of Intrathecal Ara-C.

    PubMed

    Simangan, Lenore R; Kline, Ronald M

    2015-04-01

    Intrathecal (IT) chemotherapy is an established method of preventing and treating CNS leukemia. Although this intervention is beneficial and necessary, understanding the potential adverse effects of IT chemotherapy is important so that these potential effects can be anticipated and prevented. Tumor lysis syndrome is a known complication of systemic chemotherapy and has also been reported as a rare complication after IT chemotherapy in patients with CNS disease. We report the first case of tumor lysis syndrome occurring in a patient with T-cell acute lymphoblastic leukemia without CNS disease. The systemic effects of the IT chemotherapy were confirmed by the decreased size of the presenting mediastinal mass. PMID:24942026

  2. Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.

    PubMed

    Evans, D G; Ladusans, E J; Rimmer, S; Burnell, L D; Thakker, N; Farndon, P A

    1993-06-01

    There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. We have been able to show the relative frequencies of these problems in a population based study of 84 cases in the north west of England. The major complications of basal cell carcinomas and jaw cysts occur in over 90% of patients by 40 years of age, but may both occur before 10 years of age. Less well described complications are ovarian calcification or fibroma (24%), medulloblastoma (5%), cardiac fibroma (3%), cleft palate (5%), and ophthalmic abnormalities such as squint or cataract (26%). This study more clearly defines the possible complications of the syndrome and gives clearer guidelines for counselling and screening affected and at risk persons. PMID:8326488

  3. [Two unusual complications of the Klippel-Trénaunay syndrome: carpal tunnel syndrome and cerebral venous thrombosis].

    PubMed

    Awada, A; Al Jumah, M; Al Ayafi, H

    2003-07-01

    Most neurological complications of the Klippel-Trénaunay syndrome are due to the presence of cerebral or spinal arteriovenous malformations and their consequences. We report 2 cases of unusual complications of this syndrome. In the first case, where the anomalies were restricted to the left upper extremity, the occurrence of hand numbness led to the discovery of a carpal tunnel syndrome, probably due to the lymphatic abnormal proliferation within the carpal tunnel. In the second case the disease was much more widespread and affecting, among other localizations, the cephalic extremity. The patient presented with intractable left motor seizures that appeared to be due to an occlusion of the right transverse and sigmoid sinuses. Treatment with heparin and anticonvulsants led to rapid recovery. PMID:12910081

  4. Respiratory complications of Ehlers-Danlos syndrome type IV.

    PubMed

    Hatake, Katsuhiko; Morimura, Yoshifumi; Kudo, Risa; Kawashima, Wataru; Kasuda, Shogo; Kuniyasu, Hiroki

    2013-01-01

    We describe a case of Ehlers-Danlos syndrome (EDS) type IV in a male in early half in his twenties, who experienced recurrent and eventually fatal pulmonary hemorrhage. EDS type IV is a rare disorder of type III collagen synthesis that is characterized by unusual facies, thin translucent skin with a venous vascular pattern, easy bruising, and hypermobility of the small joints. Autopsy findings showed hypermobility of the joints and distensibility of the skin. Microscopically, the abdominal skin showed substantially decreased dermal thickness. Moreover, the reticular dermis showed fine collagen bundles and large interstitial spaces compared with the skin from a normal control that showed large collagen bundles. Individual elastic fibers were also thicker than those observed in the skin of a normal control. The thoracic aorta showed thin adventitia and a relative increase in elastic fibers. The parenchyma of both the lungs showed markedly diffuse hemorrhage with hemosiderin-laden alveolar macrophages or old thrombi and organized thrombi in the small bronchi. Furthermore, both sections of the lung showed multiple fibrous nodules containing benign metaplastic bone. Vascular wall disruption and tearing of the vessel walls in the lung parenchyma were also observed. We concluded that EDS type IV led to the patient's death because of pulmonary hemorrhage. Because this syndrome resulted in the patient's death from arterial and bowel rupture, it is important to consider EDS as a potential cause of sudden death. PMID:22940417

  5. Late Intrahepatic Hematoma Complicating Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome

    SciTech Connect

    Terreni, Natalia; Vangeli, Marcello; Raimondo, Maria Luisa; Tibballs, Jonathan M.; Patch, David; Burroughs, Andrew K.

    2007-09-15

    Late intrahepatic hematoma is a rare complication of the transjugular intrahepatic portosystemic shunt (TIPS) procedure. We describe a patient with Budd-Chiari syndrome (BCS), who presented with a large inrahepatic hematoma 13 days after TIPS. Review of the literature reveals only two previous cases, both occurring in patients with BCS and presenting after a similar time interval. This potentially serious complication appears to be specific for TIPS in BCS.

  6. Antiphospholipids Syndrome Complicated by a Systemic Capillary Leak-Like Syndrome Treated With Steroids and Intravenous Immunoglobulins

    PubMed Central

    Prete, Marcella; Urso, Livio; Fatone, Maria Celeste; Pinto, Vincenzo; Perosa, Federico

    2016-01-01

    Abstract This report describes the onset of systemic capillary leak (SCL)-like syndrome in a 30-year-old woman with antiphospholipids syndrome (APS) during puerperium. Twelve hours after a cesarean section, she presented a sudden fever and abdominal pains followed by dyspnea, severe edema of the limbs and pelvis. Computer tomography shows congestion of interstitial pulmonary parenchyma, pericardial and pleural effusion, edema of intestinal wall and of perivisceral adipose tissue, and periportal lymphedema. Laboratory tests showed neutrophilic leukocytosis, hypoalbuminemia, and an increase of erythrocyte sedimentation rate and C-reactive protein. Because fever and raised inflammation parameters are not observed in idiopathic capillary leak syndrome (SCLS; Clarkson disease), a diagnosis of SCL-like syndrome was made. Albumin solution, high-dose methylprednisolone and intravenous immunoglobulins (IVIG) infusion were administered with a rapid improvement of her clinical condition. The prompt treatment with steroids and IVIG likely prevented the life-threatening shock syndrome that can occur in SCLS, with acute hypotensive attacks, and severe limbs edema requiring fasciotomy. All clinical and laboratory findings supported autoinflammation as the underlying pathogenic mechanism of the syndrome. The data indicate that SCL-like syndrome can be considered a novel clinical syndrome, which can complicate APS. PMID:26844485

  7. [Pulmonary complications of liver cirrhosis: hepatopulmonary syndrome, portopulmonary hypertension and hepatic hydrothorax].

    PubMed

    Halank, M; Strassburg, C P; Hoeper, M M

    2010-03-01

    Hepatopulmonary syndrome, portopulmonary hypertension and hepatic hydrothorax are typical pulmonary complications in patients with liver cirrhosis. Whereas hepatopulmonary syndrome and portopulmonary hypertension represent pulmonary vascular diseases, the development of hepatic hydrothorax is associated with the presence of ascites and phrenic lesions. For severe hepatopulmonary syndrome and refractory hepatic hydrothorax, liver transplantation is the treatment of choice. In severe portopulmonary hypertension specific medical treatment is indicated. In selected patients, beside intravenous prostanoids, oral endothelin receptor antagonists and phosphodiesterase type-5 inhibitors are possible treatment options. PMID:20098977

  8. Osteomalacia complicating renal tubular acidosis in association with Sjogren's syndrome.

    PubMed

    El Ati, Zohra; Fatma, Lilia Ben; Boulahya, Ghada; Rais, Lamia; Krid, Madiha; Smaoui, Wided; Maiz, Hedi Ben; Beji, Soumaya; Zouaghi, Karim; Moussa, Fatma Ben

    2014-09-01

    Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA), which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L), hypophosphatemia (0.4 mmol/L), hypocalcemia (2.14 mmol/L) and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L). The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7), glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer's test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®), calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L. PMID:25193912

  9. Budd-Chiari Syndrome Complicating Hydatid Cyst of the Liver Managed by Venoplasty and Stenting

    SciTech Connect

    Sarawagi, Radha Keshava, Shyamkumar N. Surendrababu, Narayanam R. S.; Zachariah, Uday G. Eapen, Eapen C.

    2011-02-15

    Budd-Chiari syndrome (BCS) and portal hypertension is an uncommon complication of hydatid cyst of the liver. Previous reports describe cyst excision or portosystemic shunt surgery for such patients. Here we present a case of hydatid cyst of the liver with BCS that was treated successfully with hepatic venoplasty and transjugular stent placement.

  10. Fatal Complications After Self-Expandable Metallic Stent Placement for Inferior Vena Cava Syndrome

    SciTech Connect

    Yamagami, Takuji Nakamura, Toshiyuki; Kin, Yoko; Takimoto, Yukiko; Nishimura, Tsunehiko

    2003-08-15

    We present the case of a 71-year-old man with inferior vena cava syndrome due to metastatic lymph nodes from hepatocellular carcinoma with serious complications that were strongly suspected to result from rapid changes in hemodynamics after self-expandable metallic stent placement.

  11. Novel pharmacological strategies to prevent aortic complications in Marfan syndrome.

    PubMed

    Matt, Peter; Eckstein, Friedrich

    2011-12-01

    The Marfan syndrome (MFS) is a systemic connective tissue disorder caused by mutations in the FBN1 gene. Recent molecular studies, most performed in mouse models, revealed that the MFS is more a developmental abnormality with broad and complex effects on the morphogenesis and function of multiple organ systems. FBN1 haploinsufficiency and dysregulated transforming growth factor-beta (TGF-β) signaling seem to be critical for clinical manifestations in MFS including aortic root dilatation. Aortic root aneurysm and aortic dissection represent the main causes of morbidity and mortality in MFS. Most importantly, TGF-β antagonism through angiotensin II type 1 receptor blockers (ARBs), for example losartan, has been shown to prevent and possibly reverse aortic root dilatation in a mouse model of MFS. A first human study on a small pediatric cohort confirmed those promising results in reducing the aortic root growth over a follow-up period of 12 to 47 months. So, a large multicenter trial has been set up and results should be available soon. Other therapeutic strategies which might be combined with losartan include traditional β-blockade, doxycyclin and statins. Such management could offer the first potential for primary prevention of clinical manifestations in MFS. PMID:22783312

  12. Systemic lupus erythematosus complicated by acquired von Willebrand's syndrome.

    PubMed

    Hong, Sc; Lee, Jh; Chi, Hs; Lee, Ck; Nah, Ss; Kim, Yg; Oh, Js; Moon, Hb; Yoo, B

    2008-09-01

    Haematological abnormalities are common in systemic lupus erythematosus (SLE). In some cases of acquired von Willebrand syndrome (AvWS), von Willebrand disease (vWD) is associated with autoimmune or lymphoproliferative disorders. In this study, we describe a 36-year-old woman with SLE and AvWS. The patient was referred to our hospital because of easy bruisability and recurrent vaginal bleeding. She had no history of bleeding tendency and no family history of bleeding diathesis, but she had a history of recurrent arthralgia, photosensitivity and sicca symptoms. Tests for antinuclear, anti-double stranded DNA, anticardiolipin and anti-beta2-glycoprotein I antibodies were all positive. Analysis of haemostatic parameters showed complete absence of von Willebrand factor ristocetin cofactor (vWF:Rco), von Willebrand antigen (vWF:Ag) and ristocetin-induced platelet aggregation (RIPA). Electrophoretic analysis of plasma showed a complete absence of high-molecular weight vWF multimer. The presence of antibody to vWF was detected by enzyme linked immunosorbent assay (ELISA). Treatment with corticosteroids improved SLE symptoms and corrected bleeding diasthesis. Also, the multimeric patterns of vWF became normalised and anti-vWF antibody disappeared. These findings indicated that this patient had SLE associated with AvWS, which was ameliorated by corticosteroid treatment. PMID:18755868

  13. Ischemic spinal cord syndrome after transthoracic esophagectomy: two cases of a rare neurologic complication.

    PubMed

    Zantl, N; Stein, H J; Brücher, B L; Bartels, H; Siewert, J R

    2000-01-01

    Anterior spinal artery syndrome (ASAS) is a rare complication after surgery of the thoracic or abdominal aorta. The sulco commissuralis syndrome represents a partial or incomplete ASAS. We report two cases of ischemic spinal cord syndromes after transthoracic esophagectomy. This represents a prevalence of this syndrome of 0.2% in more than 1000 consecutive esophagectomies performed at our institution. Patient 1 developed an ASAS on the first day after esophagectomy. Patient 2 showed the pathognomonic clinical signs associated with sulco commissuralis syndrome after an asymptomatic window. In both patients, the extent of the neurologic symptoms initially improved but then remained unchanged for the rest of the follow-up of 9 and 12 months. Although the prognosis of neurologic syndromes resulting from spinal cord infarction is poor, preoperative tests to identify patients at risk appear not to be justified because of the very low incidence of these syndromes after esophagectomy and the poor sensitivity and specificity of currently available diagnostic modalities. However, the possibility of ischemic spinal cord syndrome should be kept in mind when patients present with neurologic symptoms after esophagectomy. PMID:11284985

  14. A case of Lemierre's syndrome with septic shock and complicated parapneumonic effusions requiring intrapleural fibrinolysis

    PubMed Central

    Croft, Daniel P.; Philippo, Sean M.; Prasad, Paritosh

    2015-01-01

    Lemierre's syndrome is a septic thrombophlebitis of the internal jugular vein, which can lead to severe systemic illness. We report a case of an otherwise healthy 26-year-old man who suffered from pharyngitis followed by septic shock requiring intubation and vasopressor support from Fusobacterium necrophorum bacteremia. The septic emboli to his lungs caused complicated bilateral parapneumonic effusions, which recurred after initial drainage. He required bilateral chest tubes and intrapleural tPA to successfully drain his effusions. His fever curve and overall condition improved with the resolution of his effusions and after a 33-day hospitalization, he recovered without significant disability. The severity of his illness and difficult to manage complicated parapneumonic effusions were the unique facets of this case. Using an evidence-based approach of tPA and DNase for complicated parapneumonic effusions in Lemierre's syndrome can be safe and effective. PMID:26744664

  15. Buried Bumper Syndrome Revisited: A Rare but Potentially Fatal Complication of PEG Tube Placement

    PubMed Central

    Biswas, Saptarshi; Dontukurthy, Sujana; Rosenzweig, Mathew G.; Kothuru, Ravi

    2014-01-01

    Percutaneous endoscopic gastrostomy (PEG) has been used for providing enteral access to patients who require long-term enteral nutrition for years. Although generally considered safe, PEG tube placement can be associated with many immediate and delayed complications. Buried bumper syndrome (BBS) is one of the uncommon and late complications of percutaneous endoscopic gastrostomy (PEG) placement. It occurs when the internal bumper of the PEG tube erodes into the gastric wall and lodges itself between the gastric wall and skin. This can lead to a variety of additional complications such as wound infection, peritonitis, and necrotizing fasciitis. We present here a case of buried bumper syndrome which caused extensive necrosis of the anterior abdominal wall. PMID:24829838

  16. A case of Lemierre's syndrome with septic shock and complicated parapneumonic effusions requiring intrapleural fibrinolysis.

    PubMed

    Croft, Daniel P; Philippo, Sean M; Prasad, Paritosh

    2015-01-01

    Lemierre's syndrome is a septic thrombophlebitis of the internal jugular vein, which can lead to severe systemic illness. We report a case of an otherwise healthy 26-year-old man who suffered from pharyngitis followed by septic shock requiring intubation and vasopressor support from Fusobacterium necrophorum bacteremia. The septic emboli to his lungs caused complicated bilateral parapneumonic effusions, which recurred after initial drainage. He required bilateral chest tubes and intrapleural tPA to successfully drain his effusions. His fever curve and overall condition improved with the resolution of his effusions and after a 33-day hospitalization, he recovered without significant disability. The severity of his illness and difficult to manage complicated parapneumonic effusions were the unique facets of this case. Using an evidence-based approach of tPA and DNase for complicated parapneumonic effusions in Lemierre's syndrome can be safe and effective. PMID:26744664

  17. Deltoid compartment syndrome as a complication of lateral decubitus positioning for contralateral elbow surgery in an anabolic steroid abuser

    PubMed Central

    Wijesuriya, Julian D; Izod, Christopher; Burton, David JC

    2014-01-01

    Compartment syndrome as a result of patient positioning for surgery is a rare but serious complication. Compartment syndrome as a result of anabolic steroid use is more rare. We present a unique case of compartment syndrome related to anabolic steroid use and patient positioning for complex distal humerus fracture fixation and also provide a review of the literature related to this topic.

  18. Nodding syndrome in Ugandan children—clinical features, brain imaging and complications: a case series

    PubMed Central

    Idro, Richard; Opoka, Robert Opika; Aanyu, Hellen T; Kakooza-Mwesige, Angelina; Piloya-Were, Theresa; Namusoke, Hanifa; Musoke, Sarah Bonita; Nalugya, Joyce; Bangirana, Paul; Mwaka, Amos Deogratius; White, Steven; Chong, Kling; Atai-Omoruto, Anne D; Mworozi, Edison; Nankunda, Jolly; Kiguli, Sarah; Aceng, Jane Ruth; Tumwine, James K

    2013-01-01

    Objectives Nodding syndrome is a devastating neurological disorder of uncertain aetiology affecting children in Africa. There is no diagnostic test, and risk factors and symptoms that would allow early diagnosis are poorly documented. This study aimed to describe the clinical, electrophysiological and brain imaging (MRI) features and complications of nodding syndrome in Ugandan children. Design Case series. Participants 22 children with nodding syndrome brought to Mulago National Referral Hospital for assessment. Outcome measures Clinical features, physical and functional disabilities, EEG and brain MRI findings and a staging system with a progressive development of symptoms and complications. Results The median age of symptom onset was 6 (range 4–10) years and median duration of symptoms was 8.5 (range 2–11) years. 16 of 22 families reported multiple affected children. Physical manifestations and complications included stunting, wasting, lip changes and gross physical deformities. The bone age was delayed by 2 (range 1–6) years. There was peripheral muscle wasting and progressive generalised wasting. Four children had nodding as the only seizure type; 18 in addition had myoclonic, absence and/or generalised tonic–clonic seizures developing 1–3 years after the onset of illness. Psychiatric manifestations included wandering, aggression, depression and disordered perception. Cognitive assessment in three children demonstrated profound impairment. The EEG was abnormal in all, suggesting symptomatic generalised epilepsy in the majority. There were different degrees of cortical and cerebellar atrophy on brain MRI, but no hippocampal changes. Five stages with worsening physical, EEG and brain imaging features were identified: a prodrome, the development of head nodding and cognitive decline, other seizure types, multiple complications and severe disability. Conclusions Nodding syndrome is a neurological disorder that may be characterised as probably

  19. Two cases of hepatic adenomas in patients with Wolf-Hirschhorn syndrome: a new rare complication?

    PubMed

    Prunotto, Giulia; Cianci, Paola; Cereda, Anna; Scatigno, Agnese; Fossati, Chiara; Maitz, Silvia; Biondi, Andrea; Selicorni, Angelo

    2013-07-01

    Wolf-Hirschhorn syndrome (WHS) is a rare microdeletion syndrome associated with a characteristic facial appearance, failure to thrive, psychomotor delays, and various major malformations of internal organs; many medical complications have been described (feeding difficulties, epilepsy, hearing problems). Benign or malignant oncologic problems are not a typical feature of the natural history of these patients. We report on two patients with WHS patients in whom hepatic adenoma (HA) were diagnosed during adolescence. The clinical evolution of liver involvement was different between the two. We discuss the possibility of considering HA as a rare medical problem in the follow-up of WHS patients. © 2013 Wiley Periodicals, Inc. PMID:23696331

  20. HELLP Syndrome Complicated with Postpartum Subcapsular Ruptured Liver Hematoma and Purtscher-Like Retinopathy

    PubMed Central

    Cernea, Daniela; Dragoescu, Alice; Novac, Marius

    2012-01-01

    Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity. PMID:22852104

  1. Use of Eculizumab in Atypical Hemolytic Uremic Syndrome, Complicating Systemic Lupus Erythematosus.

    PubMed

    Bermea, Rene S; Sharma, Niharika; Cohen, Kenneth; Liarski, Vladimir M

    2016-09-01

    Atypical hemolytic uremic syndrome is characterized by the presence of thrombocytopenia, microangiopathic hemolytic anemia, and end-organ injury. In this report, we describe two patients with systemic lupus erythematosus who presented with findings compatible with atypical hemolytic uremic syndrome, complicated by acute kidney injury that was refractory to conventional therapies. Both patients exhibited a response to eculizumab, a monoclonal antibody to complement protein C5, with stabilization of their platelet count. On 1-year follow-up from their initial presentation, their hematologic disease remained in remission without recurrence. PMID:27556240

  2. Hypothalamic-Pituitary-Adrenal Suppression and Iatrogenic Cushing's Syndrome as a Complication of Epidural Steroid Injections

    PubMed Central

    2013-01-01

    Epidural steroid injections are well accepted as a treatment for radicular back pain in appropriate candidates. While overall incidence of systemic side effects has not been well established, at least five biochemically proven cases of iatrogenic Cushing's Syndrome have been reported as complications of epidural steroid treatment. We present an additional case of iatrogenic Cushing's Syndrome and adrenal suppression in a middle-aged woman who received three epidural steroid injections over a four-month period. We review this case in the context of previous cases and discuss diagnostic and management issues. PMID:23991341

  3. Liver biopsy complicated by hemorrhage in a patient with ARC syndrome.

    PubMed

    Hayes, Jason A; Kahr, Walter H A; Lo, Bryan; Macpherson, Bruce A

    2004-11-01

    ARC syndrome is a rare disorder consisting of arthrogryposis, renal tubular acidosis and cholestatic liver disease. We report the case of a 5-week-old patient who underwent a percutaneous liver biopsy complicated by hemorrhage, and was subsequently diagnosed with ARC syndrome. A review of the literature demonstrates that these patients are at increased risk of bleeding caused by platelet dysfunction. The evaluation and management of unexpected hemorrhage in pediatric patients as a result of undiagnosed congenital bleeding problems is discussed. PMID:15500499

  4. HELLP Syndrome Complicated by Subcapsular Hematoma of Liver: A Case Report and Review of the Literature

    PubMed Central

    Karateke, Atilla; Silfeler, Dilek; Karateke, Faruk; Kurt, Raziye; Guler, Ayse; Kartal, Ismail

    2014-01-01

    Subcapsular liver hematoma (SLH) is a rare complication of severe preeclampsia and HELLP syndrome. These patients must be followed up in intensive care unit for advanced medical support with infused fluid, replacement of blood products, and treatment of underlying disorders. There are a lot of therapeutic options varying from conservative management to surgical treatment including hepatic resection, hepatic artery ligation, and liver transplantation. In this report we aimed to present a 26-year-old woman with SLH secondary to HELLP syndrome. PMID:24804129

  5. Compartment syndrome of the thigh complicating surgical treatment of ipsilateral femur and ankle fractures

    NASA Technical Reports Server (NTRS)

    Moore, M. R.; Garfin, S. R.; Hargens, A. R.

    1987-01-01

    A 26-year-old man presented with ipsilateral femur and ankle fractures. The patient was treated with interlocking nail of his femur fracture, followed by open reduction and internal fixation of his ankle fracture under tourniquet control. Postoperatively, the patient developed compartment syndrome of his thigh with elevated pressures, requiring decompressive fasciotomies. This case illustrates the possible complication of treating a femur fracture with intramedullary nailing and then immediately applying a tourniquet to treat an ipsilateral extremity fracture. Because of the complication with this patient, we feel the procedure should be staged, or a tourniquet should be avoided if possible.

  6. Relationship between Helicobacter pylori Infections in Diabetic Patients and Inflammations, Metabolic Syndrome, and Complications

    PubMed Central

    Kayar, Yusuf; Pamukçu, Özgül; Eroğlu, Hatice; Kalkan Erol, Kübra; Ilhan, Aysegul; Kocaman, Orhan

    2015-01-01

    Helicobacter pylori infection and diabetes mellitus are two independent common diseases. It is showed that the worsening glycemic and metabolic control increases the rates of Helicobacter pylori infections and Helicobacter pylori is shown as one of the common problems in diabetic patients with complaints of gastrointestinal diseases. In this study, we aimed to investigate the prevalence and eradication rates of Helicobacter pylori in diabetic patients and the relationship of Helicobacter pylori with the risk factors and diabetic complications. In our study, in which we have included 133 patients, we have shown a significant relationship between Helicobacter pylori infections and metabolic syndrome, insulin resistance, inflammations, and diabetic complications. PMID:26464868

  7. Juvenile idiopathic arthritis complicated by amyloidosis with secondary nephrotic syndrome – effective treatment with tocilizumab

    PubMed Central

    Kwiatkowska, Małgorzata; Jednacz, Ewa

    2015-01-01

    A case report of a boy with juvenile idiopathic arthritis since the age of 2 years, generalized onset, complicated by nephrotic syndrome due to secondary type A amyloidosis is presented. In the patient the disease had an especially severe course, complicated by frequent infections, making routine treatment difficult. Amyloidosis was diagnosed in the 5th year of the disease based on a rectal biopsy. Since the disease onset the boy has been taking prednisolone and sequentially cyclosporine A, methotrexate, chlorambucil, etanercept, and cyclophosphamide. Clinical and laboratory remission was observed after treatment with tocilizumab. After 42 months of treatment with tocilizumab the boy's condition is good. There is no pain or joint edema, and no signs of nephrotic syndrome.

  8. Neuromyelitis Optica in Pregnancy Complicated by Posterior Reversible Encephalopathy Syndrome, Eclampsia and Fetal Death

    PubMed Central

    Igel, Catherine; Garretto, Diana; Robbins, Matthew S; Swerdlow, Michael; Judge, Nancy; Dayal, Ashlesha

    2015-01-01

    Neuromyelitis optica (NMO) is a demyelinating syndrome characterized by optic neuritis and acute myelitis with poor recovery and a progressive course. We report a poor outcome complicated by posterior reversible encephalopathy syndrome (PRES) and eclampsia and review available literature and current evidence for anticipation of adverse fetal and maternal effects. After a pregnancy complicated by multiple admissions for painful NMO exacerbations, a primiparous patient with seropositive NMO presented at 31 + 3/7 weeks with eclampsia, HELLP and subsequent fetal death. MRI confirmed PRES. NMO may be associated with eclampsia and leads to adverse maternal and fetal outcomes. Posited mechanisms include antibody-mediated placental damage and a heightened risk of eclampsia-associated PRES. Further characterization of the course of NMO and its relationship with pregnancy outcomes in larger series would be invaluable. PMID:25584107

  9. Iatrogenic Baastrup's Syndrome: A Potential Complication Following Anterior Interbody Lumbar Spinal Surgery

    PubMed Central

    Russo, Glenn S.; Castro, Carlos A.

    2015-01-01

    Background Baastrup's Syndrome is a condition that occurs when there is abnormal contact between two adjacent spinous processes resulting in back pain. An alteration in lumbar spinal alignment and/or adjacent segment compensatory motion is thought to be potential causative factors. The objective of this study was to present a case series of what appears to be iatrogenic Baastrup's Syndrome as a mid-to-late term complication following anterior lumbar interbody surgery. Methods A retrospective chart review was performed of all patients undergoing anterior lumbar surgery for either fusion or disc replacement to determine the prevalence of Baastrup's Syndrome. Results Over a 12-year period, 855 patients who had undergone an anterior approach for lumbar spine surgery were identified. Of them 8 patients with evidence of Baastrup's Syndrome were found; this demonstrated a prevalence of 0.9%. Diagnostic injection was a helpful clinical tool in confirming the diagnosis of iatrogenic Baastrup's Syndrome. The partial removal of the impinging spinous processes resulted in excellent clinical relief. Conclusions Iatrogenic Baastrup's Syndrome may be an iatrogenic result of anterior lumbar surgery in small group of patients. Spinous process excision is a suggested treatment option. Further studies are necessary to explore the above phenomenon. This study is a Level 3 retrospective case series. PMID:26767158

  10. Allergic bronchopulmonary aspergillosis complicating Swyer-James-Macleod's syndrome: case report and review of literature.

    PubMed

    Sehgal, I S; Dhooria, S; Behera, D; Agarwal, R

    2016-05-01

    Allergic bronchopulmonary aspergillosis (ABPA) is a pulmonary disorder that results from immune responses mounted against antigens of Aspergillus fumigatus, resulting in non-specific respiratory symptoms and structural lung damage. Classically defined in individuals suffering from bronchial asthma and cystic fibrosis, ABPA has recently been described in other lung diseases including COPD, pulmonary tuberculosis, idiopathic bronchiectasis and others. Herein, we report the first case of ABPA complicating Swyer-James-Macleod's syndrome that was successfully treated with oral antifungal therapy. PMID:27152607

  11. A Rare Case of Neonatal Complicated Appendicitis in a Child with Patau's Syndrome

    PubMed Central

    Pastore, Valentina; Bartoli, Fabio

    2014-01-01

    Neonatal appendicitis is a rare condition with high mortality rate. Signs and symptoms are often nonspecific, imaging modalities are not always diagnostic, and preoperative diagnosis is difficult with subsequent delay and complications. Its pathophysiology may be different from appendicitis in older children and comorbidities can be found. We report a case of a female neonate with Patau's syndrome, intestinal malrotation, and Fallot tetralogy in whom perforated appendix, probably occurring during fetal period due to vascular insufficiency, was found at laparotomy. PMID:25276460

  12. Postural orthostatic tachycardia syndrome complicating pregnancy: a case report with review of literature

    PubMed Central

    Pramya, N; Puliyathinkal, Shyjus; Sagili, Haritha; Jayalaksmi, D; Reddi Rani, P

    2012-01-01

    Postural orthostatic tachycardia syndrome (POTS) affects women of child-bearing age. There are little reported data on the outcomes of pregnancy in women with POTS. The most common mode of delivery reported in the literature is the caesarean section. Here we describe a woman with POTS who delivered vaginally without any complications and present a comprehensive review of the literature on pregnancy in POTS.

  13. Generalized tetanus could be complicated with Guillain-Barré syndrome.

    PubMed

    Lee, Jae Hoon; Hwang, Yun Su; Cho, Ji Hyun

    2016-07-01

    A retrospective analysis of patients diagnosed with tetanus was conducted to evaluate the occurrence of Guillain-Barré syndrome (GBS). Two of 13 tetanus cases were complicated with GBS. Their symptoms and signs related to GBS improved markedly after a 5-day infusion of intravenous immunoglobulin. Physicians should keep in mind that GBS can be an important cause of muscle weakness in patients with tetanus. PMID:27109107

  14. [Septic shock Fusobacterium necrophorum from origin gynecological at complicated an acute respiratory distress syndrome: a variant of Lemierre's syndrome].

    PubMed

    Huynh-Moynot, Sophie; Commandeur, Diane; Danguy des Déserts, Marc; Drouillard, Isabelle; Leguen, Patrick; Ould-Ahmed, Mehdi

    2011-01-01

    We report a case of a female patient of 47 years old who presents in a state of septic shock with acute insufficient respiratory complicated with syndrome of acute respiratory distress, together with a list of abdominal pain and polyarthralgia too. In her case of medical history, it is retained that she has had a intra-uterine device since 6 years without medical follow up. The initial thoraco-abdomino-pelvic scan shows a left ovarian vein thrombosis, as well as the opaqueness alveolus diffused interstitiel bilaterally and an aspect of ileitis. The IUD is taken off because of sudden occuring of purulent leucorrhoea. This results in a clinical and paraclinical improvement, whereas aminopenicillin was administered to the patient since 1 week. The microbiological blood test allows to put in evidence Fusobacterium necrophorum found in a blood culture and is sensitive to the amoxicilline-acide clavulanique and metronidazole. Isolation of this bacteria, classically found in Lemierre's syndrome, allowed to explain the multilfocalization of the symtoms and the list of pain. The whole concerns about a variant of Lemierre's syndrom: a state of septic shock secondary then caused by the anaerobic Gram negative bacilli, which is a commensal bacteria of the female genital tractus, complicated of septic emboli typical. PMID:21464014

  15. Cavernous sinus thrombosis syndrome and brainstem involvement in patient with leptospirosis: Two rare complications of leptospirosis.

    PubMed

    Alian, Shahriyar; Taghipour, Mehrdad; Sharifian, Rayka; Fereydouni, Mohammad Amin

    2014-09-01

    Leptospirosis is a bacterial disease that is caused by pathogenic spirochetes of the genus Leptospira. It can affect humans and animals. In humans, it can lead to a wide spectrum of symptoms. It is known as the most common zoonosis in the world. The typical presentation of the disease is an acute biphasic febrile illness with or without jaundice. Less common clinical manifestations may result from involvement of different human body systems. In many places, this disease may be under-diagnosed, especially when associated with neurological complications. Moreover, without treatment, leptospirosis can lead to organ damages, and even death. Neurological complications are uncommon and are reported in a few cases. Cavernous sinus thrombosis syndrome and brainstem involvement are rare complications of leptospirosis and are associated with a high mortality risk. To our knowledge, no such cases have been reported in the literature. PMID:25535508

  16. Acute Respiratory Distress Syndrome: A Rare Complication in Pediatric Diabetic Ketoacidosis.

    PubMed

    Sudhanshu, Siddhnath; Jevalikar, Ganesh; Das, Pravin K; Singh, Pramod K; Bhatia, Eesh; Bhatia, Vijayalakshmi

    2016-05-01

    Cerebral edema (CE) and non cardiogenic pulmonary edema (acute respiratory distress syndrome, ARDS) are life-threatening complications of diabetic ketoacidosis (DKA). In contrast to CE complicating DKA, which is primarily reported in pediatric patients, ARDS is rarely described in this age group. Here, the authors present a child with DKA who developed both cerebral edema and ARDS during the course of her management. It is feasible that severe acidosis, hypotension, azotemia, hypoalbuminemia and the superimposed aggressive intravenous fluid administration were important risk factors for the development of cerebral edema and ARDS in the index patient. The report highlights the importance of early diagnosis and aggressive therapy in the management of ARDS, and summarizes the published literature on this rarely reported complication of pediatric DKA. PMID:26666907

  17. Cavernous sinus thrombosis syndrome and brainstem involvement in patient with leptospirosis: Two rare complications of leptospirosis

    PubMed Central

    Alian, Shahriyar; Taghipour, Mehrdad; Sharifian, Rayka; Fereydouni, Mohammad Amin

    2014-01-01

    Leptospirosis is a bacterial disease that is caused by pathogenic spirochetes of the genus Leptospira. It can affect humans and animals. In humans, it can lead to a wide spectrum of symptoms. It is known as the most common zoonosis in the world. The typical presentation of the disease is an acute biphasic febrile illness with or without jaundice. Less common clinical manifestations may result from involvement of different human body systems. In many places, this disease may be under-diagnosed, especially when associated with neurological complications. Moreover, without treatment, leptospirosis can lead to organ damages, and even death. Neurological complications are uncommon and are reported in a few cases. Cavernous sinus thrombosis syndrome and brainstem involvement are rare complications of leptospirosis and are associated with a high mortality risk. To our knowledge, no such cases have been reported in the literature. PMID:25535508

  18. Down syndrome and postoperative complications after paediatric cardiac surgery: a propensity-matched analysis

    PubMed Central

    Tóth, Roland; Szántó, Péter; Prodán, Zsolt; Lex, Daniel J; Sápi, Erzsébet; Szatmári, András; Gál, János; Szántó, Tamás; Székely, Andrea

    2013-01-01

    OBJECTIVES The incidence of congenital heart disease is ∼50%, mostly related to endocardial cushion defects. The aim of our study was to investigate the postoperative complications that occur after paediatric cardiac surgery. METHODS Our perioperative data were analysed in paediatric patients with Down syndrome undergoing cardiac surgery. We retrospectively analysed the data from 2063 consecutive paediatric patients between January 2003 and December 2008. After excluding the patients who died or had missing data, the analysed database (before propensity matching) contained 129 Down patients and 1667 non-Down patients. After propensity matching, the study population comprised 222 patients and 111 patients had Down syndrome. RESULTS Before propensity matching, the occurrences of low output syndrome (21.2 vs 32.6%, P = 0.003), pulmonary complication (14 vs 28.7%, P < 0.001) and severe infection (11.9 vs 22.5%, P = 0.001) were higher in the Down group. Down patients were more likely to have prolonged mechanical ventilation [median (interquartile range) 22 (9–72) h vs 49 (24–117) h, P = 0.007]. The total intensive care unit length of stay [6.9 (4.2–12.4) days vs 8.3 (5.3–13.2) days, P = 0.04] and the total hospital length of stay [17.3 (13.3–23.2) days vs 18.3 (15.1–23.6) days, P = 0.05] of the Down patients were also longer. Mortality was similar in the two groups before (3.58 vs 3.88%, P = 0.86) and after (5.4 vs 4.5%, P = 1.00) propensity matching. After propensity matching, there was no difference in the occurrence of adverse events. CONCLUSIONS After propensity matching Down syndrome was not associated with increased mortality or complication rate following congenital cardiac surgery. PMID:23832837

  19. Prevalence of ocular pseudoexfoliation syndrome and associated complications in Riyadh, Saudi Arabia

    PubMed Central

    Al-Saleh, Sulaiman A.; Al-Dabbagh, Najwa M.; Al-Shamrani, Saad M.; Khan, Nusrat M.; Arfin, Misbahul; Tariq, Mohammad; Al-Faleh, Hafez M.

    2015-01-01

    Objectives: To assess the prevalence of pseudoexfoliation syndrome (PEX), and associated ophthalmic complications among Saudi patients. Methods: The prevalence of PEX and associated ocular co-morbidities were determined among the Saudi patients visiting the Primary Care Clinic of Prince Sultan Military Medical City, Riyadh, Kingdom of Saudi Arabia, between January 2009 and January 2010. A total of 1967 patients were examined biomicoscopically by ophthalmologists to determine the presence of PEX and associated ocular complications. Results: Sixty-nine of the 1967 examined patients (3.5%) showed the presence of PEX with no significant gender difference. There was an age dependent increase in the prevalence of PEX after the age of 50 years. Pseudoexfoliation syndrome was associated with higher intraocular pressure, cataract, and poor vision. There was no significant difference in the prevalence of PEX in male and female Saudi patients. Conclusion: Pseudoexfoliation syndrome is an age-related disorder, and its prevalence increases with age. Further larger population based studies are warranted to assess the prevalence of PEX and associated risk factors. PMID:25630014

  20. Hemophagocytic Syndrome, an Uncommon Complication of Microscopic Polyangitis: A Case Report From Senegal

    PubMed Central

    Cisse, Mouhamadou Moustapha; Abdoul Karim Omar, Daher; Nzambaza, Jean De Dieu; Ba, Sidy; Ndao, Awa Cheikh; Sall, Abibatou; Dial, Cherif Mouhamed; Faye, Maria; Ka, El Hadji Fary; Faye, Moustapha; Lemrabott, Ahmed Tall; Niang, Abdou; Diouf, Boucar

    2015-01-01

    Introduction: We reported a case of hemophagocytic syndrome complicating microscopic polyangitis presented by crescentic glomerulonephritis. Case Presentation: A 22-year-old female patient originated from Dakar, Senegal presented with nephrotic syndrome and rapidly progressive glomerulonephritis. On physical examination, we noticed hyperchromic diffuse punctilious purpura skin lesions predominant on the trunk, the neck and the upper thigh. Immunology investigations revealed strongly positive anti SSA/Ro and anti-SSB. Anti-neutrophil cytoplasmic antibodies had positive results with a peri-nuclear type fluorescence, specific to myeloperoxidase. In optic microscopy, renal biopsy showed a crescentic glomerulonephritis with circumferential cellular and fibrous proliferation affecting 85% of glomeruli. The diagnosis of microscopic polyangitis with renal and skin involvement was retained. The patient received methylprednisolone and cyclophosphamide 700 mg/m2 every 15 days for the first 3 pulses and every 21 days thereafter. After the 5th month, she developed obnubilation, fever and central pancytopenia. Bone marrow aspiration was performed, which showed medullary invasion by macrophages with signs of hemophagocytosis. Diagnosis of hemophagocytic syndrome complicating a microscopic polyangitis was retained and methylprednisolone pulses started. The patient was under hemodialysis after follow-up of about 9 months with stable clinical state. Conclusions: The occurrence of SAM in pauci-autoimmune vasculitis is rarely described, particularly in Africa. Our case is an illustration of the reality of this association. PMID:26866006

  1. Lumboatrial shunt in a patient with Crouzon syndrome complicated by pseudotumor cerebri.

    PubMed

    Sankey, Eric W; Khattab, Mohamed H; Elder, Benjamin D; Goodwin, C Rory; Rekate, Harold L; Rigamonti, Daniele

    2015-09-01

    A 25-year-old man with Crouzon syndrome complicated by pseudotumor cerebri and multiple shunt failures presented with progressive back and neck pain, intermittent headaches, and associated vomiting secondary to shunt infection. Due to his previous history of repeated failure of both ventriculoperitoneal and lumboperitoneal (LP) shunting procedures, the decision was made to place a lumboatrial (LA) shunt via an approach through the internal jugular vein. The procedure was uncomplicated and the man's symptoms were relieved. Despite significant improvement, the LA shunt limited his exercise tolerance, and as an avid runner and weight lifter, he requested reconversion back to an LP shunt. At a follow-up of 20months, he continued to do well both clinically and radiographically. This case report summarizes the successful placement and use of an LA shunt for the treatment of intracranial hypertension in the setting of Crouzon syndrome. PMID:26021731

  2. Organophosphate intermediate syndrome with neurological complications of extrapyramidal symptoms in clinical practice

    PubMed Central

    Detweiler, Mark B.

    2014-01-01

    Organophosphates (OPs) are ubiquitous in the world as domestic and industrial agricultural insecticides. Intentional poisoning as suicides attempts are clinical phenomena seen in emergency departments and clinics in agricultural areas. Intermediate syndrome with the neurological complication of extra pyramidal symptoms following acute OP ingestion may occur in pediatric and adult cases. While death is the most serious consequence of toxic OP doses, low levels of exposure and nonfatal doses may disrupt the neurobehavioral development of fetuses and children in addition to bring linked to testicular cancer and male and female infertility. These are disturbing. Chronic and acute toxicity from OPs are barriers to the health of our present and future generations. Symptoms and treatment of acute and chronic OP exposure are briefly referenced with inclusion of the intermediate syndrome. Suggestions for local and systemic reduction of the acute and long term consequences of OP ingestion are opined. PMID:25002781

  3. Organophosphate intermediate syndrome with neurological complications of extrapyramidal symptoms in clinical practice.

    PubMed

    Detweiler, Mark B

    2014-07-01

    Organophosphates (OPs) are ubiquitous in the world as domestic and industrial agricultural insecticides. Intentional poisoning as suicides attempts are clinical phenomena seen in emergency departments and clinics in agricultural areas. Intermediate syndrome with the neurological complication of extra pyramidal symptoms following acute OP ingestion may occur in pediatric and adult cases. While death is the most serious consequence of toxic OP doses, low levels of exposure and nonfatal doses may disrupt the neurobehavioral development of fetuses and children in addition to bring linked to testicular cancer and male and female infertility. These are disturbing. Chronic and acute toxicity from OPs are barriers to the health of our present and future generations. Symptoms and treatment of acute and chronic OP exposure are briefly referenced with inclusion of the intermediate syndrome. Suggestions for local and systemic reduction of the acute and long term consequences of OP ingestion are opined. PMID:25002781

  4. Cutaneous metastatic adenocarcinoma complicated by spontaneous tumor lysis syndrome: A case report

    PubMed Central

    WANG, YU; YUAN, CAIJUN; LIU, XIAOMEI

    2014-01-01

    The present study reports the case of a 71-year-old female with metastatic adenocarcinoma of the skin who developed tumor lysis syndrome (TLS) upon admittance to the First Affiliated Hospital of Liaoning Medical University (Jinzhou, China). The patient presented to the hospital due to multiple subcutaneous nodules, lethargy and weakness, but succumbed without any cancer therapy. Metastases to the skin from solid carcinomas are uncommon, and several studies have reported patients with minimal primary symptoms despite extensive metastatic skin disease. However, few cases were accompanied with spontaneous TLS at the time of presentation. TLS may be a severe complication during the therapy for hematological and oncological diseases. Although spontaneous TLS in internal tumors has been reported, it is extremely rare. The present study highlights the fact that multiple subcutaneous metastases may occur with the symptoms of spontaneous TLS, and may be key for the early recognition of this syndrome. PMID:25013514

  5. Bilateral Giant Coronary Artery Aneurysms Complicated by Acute Coronary Syndrome and Cardiogenic Shock.

    PubMed

    Chiu, Peter; Lynch, Donald; Jahanayar, Jama; Rogers, Ian S; Tremmel, Jennifer; Boyd, Jack

    2016-04-01

    Giant coronary aneurysms are rare. We present a 25-year-old woman with a known history of non-Kawasaki/nonatherosclerotic bilateral coronary aneurysms. She was transferred to our facility with acute coronary syndrome complicated by cardiogenic shock. Angiography demonstrated giant bilateral coronary aneurysms and complete occlusion of the left anterior descending (LAD) artery. Emergent coronary artery bypass grafting was performed. Coronary artery bypass grafting is the preferred approach for addressing giant coronary aneurysms. Intervention on the aneurysm varies in the literature. Aggressive revascularization is recommended in the non-Kawasaki/nonatherosclerotic aneurysm patient, and ligation should be performed in patients with thromboembolic phenomena. PMID:27000621

  6. [Loco-regional complications of pharyngitis: the example of Lemierre's syndrome].

    PubMed

    Tschopp, J; Chuard, C

    2015-10-01

    Pharyngitis is a common cause of consultation in ambulatory medicine. Although it is benign in most cases, serious complications may happen and must be recognized quickly. Lemierre's syndrome is one of them. It consists in the association of thrombosis of the internal jugular vein and septic emboli that generally involve the lungs and is classically associated with Fusobacterium necrophorum. It is usually found in young and healthy adults and has an estimated mortality of 5%. Diagnosis relies essentially on the characteristic presentation of the disease. Therapy consists of surgical drainage of purulent collections and necrotic tissues associated with a prolonged course of antibiotics. Some authors also recommend a anticoagulation. PMID:26638515

  7. Posterior reversible encephalopathy syndrome (PRES) as a complication of Guillain-Barre' syndrome (GBS).

    PubMed

    Nabi, Sumaira; Rajput, Haris Majid; Badshah, Mazhar; Ahmed, Shahzad

    2016-01-01

    A 17-year-old Pakistani female patient presented with acute onset flaccid quadriparesis with nerve conduction studies showing demyelinating polyneuropathy consistent with Guillain-Barre' syndrome. She was treated with 4 plasmapheresis sessions. She developed raised blood pressure, headache, visual loss and generalised seizures on the 13th day of admission. MRI of the brain on contrast showed findings of altered signals low on T1-weighted image, high on T2-weighted image and fluid-attenuated inversion recovery in the white matter of bilateral occipital, parietal and right frontal lobe consistent with posterior reversible encephalopathy syndrome. The patient was administered antiepileptic and antihypertensive drugs to control seizures and blood pressure. She was discharged in a stable state. On follow-up her visual loss had recovered completely and she had regained full motor strength in all four extremities after 6 weeks. Fresh MRI of the brain revealed complete resolution of lesions. Antihypertensive and antiepileptic medication was discontinued. She is independent in all her daily activities. PMID:27489061

  8. [ROLE OF ULTRASONIC CAVITATION IN COMPLEX TREATMENT OF SUPPURATIVE-NECROTIC COMPLICATIONS OF DIABETIC FOOT SYNDROME].

    PubMed

    Petrenko, O M

    2015-07-01

    The microbial landscape of wounds with suppurative-necrotic complications in diabetic foot syndrome (DFS) was investigated. Capabilities by inhibiting the formation of bacterial biofilms using ultrasonic cavitation (USC) were determined. In 32 patients over the purulent necrotic complications DFS USC applied using the apparatus "Sonoca-180" company Soring (Germany), 20 patients performed a basic therapy. Qualitative and quantitative composition of microflora were determined by the results of microbiological investigations using parallel transmission electron microscopy dynamics with USC. In patients with DFS noted the formation of bacterial biofilms that contained clusters of microorganisms in a polysaccharide matrix and were fixed to the wound surface. In the microbial landscape of wounds dominated gram-negative microorganisms. Application of USC contributed to the rapid cleanse wounds. PMID:26591218

  9. Blood rheology and platelet function in untreated early-stage essential hypertensives complicated with metabolic syndrome.

    PubMed

    Sugimori, Hiroko; Tomoda, Fumihiro; Koike, Tsutomu; Kinuno, Hiroyuki; Kurosaki, Hiroko; Masutani, Toshitaka; Inoue, Hiroshi

    2012-01-01

    We examined whether hemorheology and platelet function are affected in essential hypertensives (EHTs) of the World Health Organization stage I when complicated with metabolic syndrome (Mets). In 156 untreated EHTs, blood viscosity and platelet surface markers were determined. Blood viscosity was significantly elevated in 54 subjects with Mets compared with 102 subjects without Mets. Hematocrit and plasma viscosity increased in the group with Mets, although red blood cell rigidity index "k" did not differ between groups. As a whole group, blood viscosity correlated positively with hematocrit and plasma viscosity. Additionally, plasma viscosity correlated positively with plasma leptin, triglyceride, homeostasis model assessment index, C-reactive protein, and plasma fibrinogen, but negatively with high-density lipoprotein cholesterol. In contrast, no differences were seen in platelet surface markers between groups. In conclusion, EHTs of the early stage complicated with Mets are characterized by increased blood viscosity due to hemoconcentration and increased plasma viscosity. PMID:22570768

  10. Life-Threatening Complication During Percutaneous Ablation of Adrenal Gland Metastasis: Takotsubo Syndrome

    SciTech Connect

    Tsoumakidou, Georgia Buy, Xavier; Zickler, Pierre; Zupan, Michel Douchet, Marie-Pierre; Gangi, Afshin

    2010-06-15

    A rare life-threatening complication during percutaneous cryoablation of an adrenal gland metastasis from a lung carcinoma is reported. The patient presented hypertensive crisis at the beginning of the thawing cycle, followed by electrocardiographic change which necessitated interruption of the procedure and his transfer to the intensive care unit with suspicion of heart infarct. There was a slight increase in cardiac enzyme levels, and ventricular angiography demonstrated transient hypokinesis-dyskinesis of the mid left ventricular segments without apical involvement, while the coronary arteries showed no significant stenosis on coronarography. These findings led to the diagnosis of Takotsubo cardiomyopathy left ventricular dysfunction syndrome. This is the first case of Takotsubo cardiomyopathy occurring as a complication during percutaneous ablation of an adrenal gland tumor.

  11. [Varicella zoster virus-induced meningoencephalitis complicated with orbital apex syndrome: a case report].

    PubMed

    Wakida, Kenji; Sakurai, Takeo; Nishida, Hiroshi

    2014-09-01

    A 69-year-old male was admitted to hospital with clouded consciousness and abnormal behavior. His body temperature was 38.2 degree Celsius upon admission and he was somnolent. Herpes zoster was observed along the first division of the trigeminal nerve on the right side of the face. The right palpebra was severely swollen, and the right eye showed a dilated pupil, loss of light reflex, and total ophthalmoplegia. A spinal tap revealed pleocytosis and increased proteins, and a DNA-PCR test for varicella-zoster virus (VZV) was positive. Optic neuritis was diagnosed based on fundoscopy. Following acyclovir administration, the patient regained full consciousness and the rash was alleviated; however, visual acuity did not recover. VZV-induced meningoencephalitis complicated with orbital apex syndrome is rarely observed. We suspect that VZV initially infected the nasociliary nerve at the distal end of the first division of trigeminal nerve and spread to the adjacent optic, oculomotor, trochlear, and abducens nerves, resulting in VZV-induced meningoencephalitis complicated with orbital apex syndrome. PMID:25200582

  12. The Effectiveness of GECB Pastille in Reducing Complications of Dry Socket Syndrome

    PubMed Central

    Haghighat, Abbas; Bahri Najafi, Rahim; Bazvand, Mostafa; Badrian, Hamid; Khalighinejad, Navid; Goroohi, Hossein

    2012-01-01

    Background and Purpose. Dry socket syndrome is one of the most irritating complications after tooth extraction. This study aims to investigate the efficacy of pastille GECB compared to ZOE. Materials and Methods. 30 patients with dry socket syndrome were selected and divided into two groups. GECB pastille was produced with 3% Guaiacol, 3% Eugenol 1.6% Chlorobutanol, sized 3 × 7 × 10 mm. GECB was applied in one group, and ZOE was used for the other group. Duration of pain after treatment and painkiller intake values were recorded within 20 days. The data were analyzed with independent samples t-test, Mann-Whitney, and Chi-Square tests. Results. Pain persisted for 45.53 ± 33.34 minutes in patients treated with ZOE and 19.87 ± 21.80 minutes in those treated with GECB (P = 0.19). Patients in the ZOE group reported more painkiller intake within 20 days (P = 0.031). Conclusion. GECB showed more significant efficacy in reducing complications after tooth extraction. PMID:22577382

  13. The hypertension of Cushing's syndrome: controversies in the pathophysiology and focus on cardiovascular complications

    PubMed Central

    Isidori, Andrea M.; Graziadio, Chiara; Paragliola, Rosa Maria; Cozzolino, Alessia; Ambrogio, Alberto G.; Colao, Annamaria; Corsello, Salvatore M.; Pivonello, Rosario

    2015-01-01

    Cushing's syndrome is associated with increased mortality, mainly due to cardiovascular complications, which are sustained by the common development of systemic arterial hypertension and metabolic syndrome, which partially persist after the disease remission. Cardiovascular diseases and hypertension associated with endogenous hypercortisolism reveal underexplored peculiarities. The use of exogenous corticosteroids also impacts on hypertension and cardiovascular system, especially after prolonged treatment. The mechanisms involved in the development of hypertension differ, whether glucocorticoid excess is acute or chronic, and the source endogenous or exogenous, introducing inconsistencies among published studies. The pleiotropic effects of glucocorticoids and the overlap of the several regulatory mechanisms controlling blood pressure suggest that a rigorous comparison of in-vivo and in-vitro studies is necessary to draw reliable conclusions. This review, developed during the first ‘Altogether to Beat Cushing's syndrome’ workshop held in Capri in 2012, evaluates the most important peculiarities of hypertension associated with CS, with a particular focus on its pathophysiology. A critical appraisal of most significant animal and human studies is compared with a systematic review of the few available clinical trials. A special attention is dedicated to the description of the clinical features and cardiovascular damage secondary to glucocorticoid excess. On the basis of the consensus reached during the workshop, a pathophysiology-oriented therapeutic algorithm has been developed and it could serve as a first attempt to rationalize the treatment of hypertension in Cushing's syndrome. PMID:25415766

  14. Abdominal compartment syndrome – the prevention and treatment of possible lethal complications following hip arthroscopy: a case report

    PubMed Central

    2014-01-01

    Introduction Intra-abdominal hypertension and abdominal compartment syndrome have been increasingly recognized as a hip arthroscopy complication over the past decade. In the absence of consensus definitions and treatment guidelines, the diagnosis and management of intra-abdominal hypertension and abdominal compartment syndrome remains variable from institution to institution. Case presentation We report the occurrence of the extravasation of fluid into the abdomen during arthroscopic treatment of femoroacetabular impingement combined with resection of trochanteric bursa and our management of the condition in a 55-year old Caucasian woman. Conclusions We present an algorithm of treatment of abdominal compartment syndrome, as a hip arthroscopy complication, according to the consensus definitions and recommendations of the World Society of the Abdominal Compartment Syndrome. In the algorithm options, we have included paracentesis and percutaneous catheter decompression as the main point of treatment. Our algorithm will have a broader clinical impact on orthopedic surgery, anesthesiology and emergency medicine. PMID:25394557

  15. Complication of chronic eosinophilic pneumonia in an elderly patient with Sjögren syndrome.

    PubMed

    Waseda, Koichi; Hagiya, Hideharu; Hanayama, Yoshihisa; Terasaka, Tomohiro; Kimura, Kosuke; Tsuzuki, Takao; Hasegawa, Kou; Nada, Takahiro; Nakamura, Eri; Murakami, Kazutoshi; Kondo, Eisei; Otsuka, Fumio

    2015-01-01

    An 81-year-old Japanese male with primary Sjögren syndrome (pSS) developed a low-grade fever and productive cough which were refractory to antibiotic therapy. Based on the high level of eosinophils observed in his bronchial alveolar lavage, he was diagnosed with chronic eosinophilic pneumonia (CEP) and successfully treated by oral prednisolone. Interstitial lung diseases associated with pSS (pSS-ILDs) usually present as nonspecific interstitial pneumonia or usual interstitial pneumonia; therefore, the present case is extremely unique in that the patient's condition was complicated with CEP. A diagnosis of advanced gallbladder cancer was made in the patient's clinical course, suggesting the advisability of a whole-body workup in cases of pSS, especially in elderly patients. PMID:25899635

  16. Herlyn-Werner-Wunderlich Syndrome Complicated with Pyocolpos: An Unusual Cause of Postabortal Sepsis

    PubMed Central

    Sharma, Deepti; Janu, MK; Gaikwad, Ramesh; Usha, MG

    2011-01-01

    Obstructive mullerian anomalies give rise to a spectrum of clinical presentations and are uncommon in routine gynecologic practice. The patient usually becomes symptomatic in early reproductive years. Recurrent pelvic pain, dysmenorrhea, enlarging abdominopelvic mass, and abnormal vaginal discharge are the common presenting symptoms. We describe a rare case of a mullerian anomaly getting diagnosed 13 years after attaining menarche during the evaluation of postabortal sepsis. Patient presented 2 weeks following evacuation carried out for missed abortion, with acute abdominal pain, fever and foul smelling discharge per vaginum. The anomaly was identified as uterus didelphys with obstructed left hemivagina and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome) complicated by pyocolpos. She was successfully managed by single-stage transvaginal septum resection under laparoscopic control. PMID:26085755

  17. Juvenile rheumatoid arthritis in velo-cardio-facial syndrome: Coincidence of unusual complication?

    SciTech Connect

    Rasmussen, S.A.; Williams, C.A.; Gray, B.A.

    1996-09-06

    We report on two patients with velo-cardio-facial syndrome (VCFS) and juvenile rheumatoid arthritis (JRA). The first, a 9-year-old girl, presented with microcephaly, characteristic face, congenital heart disease, and velopharyngeal insufficiency. Fluorescence in situ hybridization (FISH) study showed deletion of D22S75 (N25), confirming the diagnosis of VCFS. At age 7, she developed joint pain, and polyarticular JRA was diagnosed. Awareness of this case led to the subsequent diagnosis of VCFS (also confirmed by FISH) in another, unrelated 12-year-old girl with characteristic face, hypernasal speech, and obesity. JRA was first diagnosed in this case at age 5 years, and she subsequently developed severe polyarticular disease. Neither patient had clinical or laboratory evidence of immunodeficiency. This observation represents the first report of the association of JRA with VCFS and raises the question of whether this is a coincidental association or a rare complication of this condition. 33 refs., 4 figs., 1 tab.

  18. [A Case of Corticobasal Syndrome Complicated with Hypopituitarism and Hashimoto's Disease].

    PubMed

    Morimoto, Satoru; Kinbara, Yoshiyuki; Terada, Makoto; Komiya, Tadashi; Ishii, Kenji; Takao, Masaki; Kanemaru, Kazutomi; Murayama, Shigeo

    2015-06-01

    We report the case of an individual with corticobasal syndrome (CBS), hypopituitarism due to a post-traumatic leptomeningeal cyst, and Hashimoto's disease. A 71-year-old woman was admitted to our hospital because of cognitive dysfunction and bradykinesia. Following a primary diagnosis of hypopituitarism and hypothyroidism, she was given hormone replacement therapy, and her clinical symptoms appeared to improve. However, some cognitive impairment and extrapyramidal symptoms remained. The results of careful neurological examinations, as well as magnetic resonance, single-photon emission computed tomography, and positron emission tomography images, suggested a diagnosis of CBS-CBD (corticobasal degeneration). Because parkinsonism and cognitive impairment can be caused by endocrinopathy, it was initially difficult to reach the complete diagnosis that included CBS. Thus, it is important to understand that complicated neurological presentations can be caused by several different disorders. PMID:26062591

  19. Prevalence of polycystic ovary syndrome and its associated complications in Iranian women: A meta-analysis

    PubMed Central

    Jalilian, Anahita; Kiani, Faezeh; Sayehmiri, Fatemeh; Sayehmiri, Kourosh; Khodaee, Zahra; Akbari, Malihe

    2015-01-01

    Background: Polycystic ovary syndrome (PCOS) is the most common endocrine disorder in women of reproductive age and is the most common cause of infertility due to anovulation. There is no single criterion for the diagnosis of this syndrome. Objective: The purpose of this study was to investigate the prevalence of PCOS and its associated complications in Iranian women using meta-analysis method. Materials and Methods: Prevalence of PCOS was investigated from the SID, Goggle scholar, PubMed, Magiran, Irandoc, and Iranmedex, and weighting of each study was calculated according to sample size and prevalence of the binomial distribution. Data were analyzed using a random-effects model meta-analysis (Random effects model) and the software R and Stata Version 11.2. Results: 30 studies conducted between the years 2006 to 2011 were entered into meta-analysis. The total sample size was 19, 226 women aged between 10-45 years. The prevalence of PCOS based on National institute of child health and human disease of the U.S was, 6.8% (95 % CI: 4.11–8.5), based on Rotterdam was 19.5% (95 % CI: 2.24-8.14), and based on ultrasound was 4.41% (95% CI: 5.68-4.14). Also, the prevalence of hirsutism was estimated to be 13%, acne 26%, androgenic alopecia 9%, menstrual disorders 28%, overweight 21%, obesity 19%, and infertility 8%. Conclusion: The prevalence of PCOS in Iran is not high. However, given the risk of complications such as heart disease - cardiovascular and infertility, prevention of PCOS is important; we suggest that health officials must submit plans for the community in this respect. PMID:26644787

  20. Posterior Reversible Encephalopathy Syndrome Complicating Traumatic Pancreatitis: A Pediatric Case Report.

    PubMed

    Sigurtà, Anna; Terzi, Valeria; Regna-Gladin, Caroline; Fumagalli, Roberto

    2016-05-01

    We are reporting a case of posterior reversible encephalopathy syndrome (PRES) developed in an unusual clinical scenario without the presence of the most described symptoms. PRES is a neurological and radiological syndrome described in many different clinical conditions. In children it has been mostly reported in association with hematological and renal disorders.Our patient was a 15 years old boy, admitted to our intensive care unit for pancreatitis after blunt abdominal trauma.During the stay in the intensive care unit, he underwent multiple abdominal surgical interventions for pancreatitis complications. He had a difficult management of analgesia and sedation, being often agitated with high arterial pressure, and he developed a bacterial peritonitis. After 29 days his neurological conditions abruptly worsened with neuroimaging findings consistent with PRES. His clinical conditions progressively improved after sedation and arterial pressure control.He was discharged at home with complete resolution of the neurological and imaging signs 2 months later.The pathophysiology of PRES is controversial and involves disordered autoregulation ascribable to hypertension and endothelial dysfunction. In this case both hypertension and endothelial activation, triggered by sepsis and pancreatitis, could represent the culprits of PRES onset. Even if there is no specific treatment for this condition, a diagnosis is mandatory to start antihypertensive and supportive treatment. We are therefore suggesting to consider PRES in the differential diagnosis of a neurological deterioration preceded by hypertension and/or septic state, even without other "typical" clinical features. PMID:27258506

  1. From arterial hypertension complications to von Hippel-Lindau syndrome diagnosis.

    PubMed

    Kozaczuk, Sylwia; Ben-Skowronek, Iwona

    2015-01-01

    Von Hippel-Lindau syndrome is a rare, genetically based, autosomal dominant disorder. Its course is accompanied by the development of multiple neoplasms with the following tumours diagnosed most commonly in the central nervous system haemangioblastoma, clear cell renal cell carcinoma, phaeochromocytomas, pancreatic islet tumours, and endolymphatic sac tumours. Additionally, renal and pancreatic cystadenomas and epididymal cystadenomas have been diagnosed in males and cystadenomas of the broad ligament of the uterus have been diagnosed in females.The following paper presents the diagnostic way in a boy with vision disorders as the first symptom. Hypertension retinopathy and extremely elevated blood pressure were observed during ophthalmologic consultation. Complications of arterial hypertension were confirmed by echocardiography, which diagnosed hypertension cardiomyopathy. Hypertension retinopathy was confirmed by optical coherence tomography. Examinations performed in the neurology, cardiology, and finally endocrinology indicated a bilateral phaeochromocytoma as the cause of arterial hypertension. Moreover, some genetic investigations showed a mutation in the VHL ex.1 p.Y112 C gene responsible for the hereditary form of phaeochromocytoma which confirmed von Hippel-Lindau syndrome. After surgical treatment of phaeochromocytoma the patient needed careful management according to the surveillance protocol for von Hippel-Lindau disease. PMID:26268347

  2. [Successful treatment with recombinant thrombomodulin for disseminated intravascular coagulation complicated with hemophagocytic syndrome].

    PubMed

    Yamazaki, Hiroyuki; Kondo, Tadakazu; Tatsumi, Goichi; Kotani, Shin-Ichi; Arai, Yasuyuki; Shirakawa, Kotaro; Kitano, Toshiyuki; Hishizawa, Masakatsu; Kadowaki, Norimitsu; Takaori-Kondo, Akifumi

    2015-03-01

    Recombinant human thrombomodulin (rTM) improves the blood coagulation disorder characteristic of disseminated intravascular coagulation (DIC) as well as, or even better than, other anti-DIC drugs. On post-marketing surveillance, its effectiveness has been recognized for hematologic disorders, sepsis and solid tumor subgroups. However, the effect on hemophagocytic syndrome (HPS) complicated by DIC remains unclear. We treated three HPS patients with rTM in addition to chemotherapy for the underlying diseases including nasal NK/T cell lymphoma, angioimmunoblastic T-cell lymphoma and refractory acute myeloid leukemia post cord blood transplantation. Although being refractory to medical management was suspected in our cases, clinical status rapidly came under control including not only amelioration of the blood coagulation disorder but also inflammatory reactions, such as serum ferritin and lactic acid dehydrogenase abnormalities, which represent HPS activity. These observations suggest that rTM might exert marked synergistic effects on HPS with DIC. Given the results obtained in these three cases, administration of rTM appears to offer a promising method of treating HPS complicated by DIC. PMID:25876785

  3. FKBP14-related Ehlers-Danlos syndrome: expansion of the phenotype to include vascular complications.

    PubMed

    Murray, Mitzi L; Yang, Margaret; Fauth, Christine; Byers, Peter H

    2014-07-01

    Biallelic mutations in FKBP14 cause a recessive form of Ehlers-Danlos syndrome (EDS) characterized by progressive kyphoscoliosis, myopathy, and hearing loss. To date, four children and one adult with this condition have been reported. We recently identified a 42-year-old man with severe kyphoscoliosis, restrictive/obstructive lung disease, short stature, mild hearing loss, decreased muscle mass, and a dissection of the celiac artery at age 41. He also had complete occlusion of the superior mesenteric artery with compensatory flow through an enlarged and tortuous inferior mesenteric artery. He was homozygous for a previously identified FKBP14 mutation, c.362dupC, p.(Glu122Argfs*7). He had no mutations in COL3A1, ACTA2, TGFBR1, TGFBR2, or SMAD3. The FKBP14 mutations in our patient occurred on the same haplotype as others with this same mutation. Although one family member in a previous report was thought to have early vascular complications, it could not be confirmed that she had biallelic mutations in FKBP14. This report expands the phenotype of FKBP14-related EDS to include risk for vascular complications and also raises the question of whether the shared haplotype represents a risk allele or founder mutation. PMID:24677762

  4. [Prevention and treatment of the complications of polycystic ovarian syndrome--the significance of evidence-based, interdisciplinary management].

    PubMed

    Gődény, Sándor; Csenteri, Orsolya Karola

    2015-12-13

    Polycystic ovary syndrome is the most common hormonal and metabolic disorder likely to affect women. The syndrome is often associated with obesity, hyperinsulinemia and adversely affects endocrine, metabolic, and cardiovascular health. The complex feature of the syndrome requires an interdisciplinary approach to treatment, where cooperation of paediatrician, internist, gynaecologist, endocrinologist, dermatologist, psychologist and oncologist is essential. The prevention and the treatment should be based on the best available evidence. This should include physical examination, laboratory tests for hormones, serum insulin, glucose, lipids, in addition patient's preferences should be considered, too. To maximise health gain of polycystic ovarian syndrome, adequate, effective, efficient and safe treatment is necessary. This article summarises the highest available evidence provided by meta-analyses and systematic reviews of the prevention of metabolic and cardiovascular complications of the syndrome, and discusses the relevant evidence published in the literature. PMID:26639643

  5. Prevalence of Metabolic Syndrome: Association with Risk Factors and Cardiovascular Complications in an Urban Population

    PubMed Central

    Moreira, Gisela Cipullo; Cipullo, José Paulo; Ciorlia, Luiz Alberto Souza; Cesarino, Cláudia Bernardi; Vilela-Martin, José Fernando

    2014-01-01

    Introduction Metabolic syndrome (MS) is a set of cardiovascular risk factors and type 2 diabetes, responsible for a 2.5-fold increased cardiovascular mortality and a 5-fold higher risk of developing diabetes. Objectives 1-to evaluate the prevalence of MS in individuals over 18 years associated with age, gender, socioeconomic status, educational levels, body mass index (BMI), HOMA index and physical activity; moreover, to compare it to other studies; 2-to compare the prevalence of elevated blood pressure (BP), high triglycerides and plasma glucose levels, low HDL cholesterol and high waist circumference among individuals with MS also according to gender; 3-to determine the number of risk factors in subjects with MS and prevalence of complications in individuals with and without MS aged over 40 years. Methods A cross-sectional study of 1369 Individuals, 667 males (48.7%) and 702 females (51.3%) was considered to evaluate the prevalence of MS and associated factors in the population. Results The study showed that 22.7% (95% CI: 19.4% to 26.0%) of the population has MS, which increases with age, higher BMI and sedentary lifestyle. There was no significant difference between genders until age ≥70 years and social classes. Higher prevalence of MS was observed in lower educational levels and higher prevalence of HOMA positive among individuals with MS. The most prevalent risk factors were elevated blood pressure (85%), low HDL cholesterol (83.1%) and increased waist circumference (82.5%). The prevalence of elevated BP, low HDL cholesterol and plasma glucose levels did not show significant difference between genders. Individuals with MS had higher risk of cardiovascular complications over 40 years. Conclusion The prevalence of MS found is similar to that in developed countries, being influenced by age, body mass index, educational levels, physical activity, and leading to a higher prevalence of cardiovascular complications after the 4th decade of life. PMID:25180496

  6. Neurological Complications of Middle East Respiratory Syndrome Coronavirus: A Report of Two Cases and Review of the Literature

    PubMed Central

    Subahi, Ahmad; Shirah, Bader

    2016-01-01

    Middle East Respiratory Syndrome Coronavirus (MERS-CoV) was first discovered in September 2012 in Saudi Arabia. Since then, it caused more than 1600 laboratory-confirmed cases and more than 580 deaths among them. The clinical course of the disease ranges from asymptomatic infection to severe lower respiratory tract illness with multiorgan involvement and death. The disease can cause pulmonary, renal, hematological, and gastrointestinal complications. In this paper, we report neurological complications of MERS-CoV in two adult patients, and we hypothesize the pathophysiology. The first patient had an intracerebral hemorrhage as a result of thrombocytopenia, disseminated intravascular coagulation, and platelet dysfunction. The second case was a case of critical illness polyneuropathy complicating a long ICU stay. In these cases, the neurological complications were secondary to systemic complications and long ICU stay. Autopsy studies are needed to further understand the pathological mechanism. PMID:27239356

  7. Identification of a Monoclonal Antibody That Attenuates Antiphospholipid Syndrome-Related Pregnancy Complications and Thrombosis

    PubMed Central

    Mineo, Chieko; Lanier, Lane; Jung, Eunjeong; Sengupta, Samarpita; Ulrich, Victoria; Sacharidou, Anastasia; Tarango, Cristina; Osunbunmi, Olutoye; Shen, Yu-Min; Salmon, Jane E.; Brekken, Rolf A.; Huang, Xianming; Shaul, Philip W.

    2016-01-01

    In the antiphospholipid syndrome (APS), patients produce antiphospholipid antibodies (aPL) that promote thrombosis and adverse pregnancy outcomes. Current therapy with anticoagulation is only partially effective and associated with multiple complications. We previously discovered that aPL recognition of cell surface β2-glycoprotein I (β2-GPI) initiates apolipoprotein E receptor 2 (apoER2)-dependent signaling in endothelial cells and in placental trophoblasts that ultimately promotes thrombosis and fetal loss, respectively. Here we sought to identify a monoclonal antibody (mAb) to β2-GPI that negates aPL-induced processes in cell culture and APS disease endpoints in mice. In a screen measuring endothelial NO synthase (eNOS) activity in cultured endothelial cells, we found that whereas aPL inhibit eNOS, the mAb 1N11 does not, and instead 1N11 prevents aPL action. Coimmunoprecipitation studies revealed that 1N11 decreases pathogenic antibody binding to β2-GPI, and it blocks aPL-induced complex formation between β2-GPI and apoER2. 1N11 also prevents aPL antagonism of endothelial cell migration, and in mice it reverses the impairment in reendothelialization caused by aPL, which underlies the non-thrombotic vascular occlusion provoked by disease-causing antibodies. In addition, aPL inhibition of trophoblast proliferation and migration is negated by 1N11, and the more than 6-fold increase in fetal resorption caused by aPL in pregnant mice is prevented by 1N11. Furthermore, the promotion of thrombosis by aPL is negated by 1N11. Thus, 1N11 has been identified as an mAb that attenuates APS-related pregnancy complications and thrombosis in mice. 1N11 may provide an efficacious, mechanism-based therapy to combat the often devastating conditions suffered by APS patients. PMID:27463336

  8. Hyperacute abdominal compartment syndrome: an unrecognized complication of massive intraoperative resuscitation for extra-abdominal injuries.

    PubMed

    Rodas, Edgar B; Malhotra, Ajai K; Chhitwal, Reena; Aboutanos, Michel B; Duane, Therese M; Ivatury, Rao R

    2005-11-01

    Primary and secondary abdominal compartment syndrome (ACS) are well-recognized entities after trauma. The current study describes a "hyperacute" form of secondary ACS (HACS) that develops intraoperatively while repair of extra-abdominal injuries is being carried out simultaneous with massive resuscitation for shock caused by those injuries. The charts of patients requiring abdominal decompression (AD) for HACS at time of extra-abdominal surgery at our level I trauma center were reviewed. The following data was gathered: age, Injury Severity Score (ISS), mechanism, resuscitation details, time to AD, time to abdominal closure, and outcome. All continuous data are presented as mean +/- standard error of mean. Hemodynamic and ventilatory data pre- and post-AD was compared using paired t test with significance set at P < 0.05. Five (0.13%) of 3,750 trauma admissions developed HACS during the 15-month study period ending February 2004. Mean age was 32 +/- 7 years, and mean ISS was 19 +/- 2. Four of five patients arrived in hemorrhagic shock (blunt subclavian artery injury, 1; chest gunshot, 1; gunshot to brachial artery, 1; stab transection of femoral vessels, 1) and were immediately operated upon. One of five patients (70% burn) developed HACS during burn wound excision on day 2. HACS developed after massive crystalloid (15 +/- 1.7 L) and blood (11 +/- 0.4 units) resuscitation during prolonged surgery (4.8 +/- 0.8 hours). Pre- versus post-AD comparisons revealed significant (P < 0.05) improvements in mean arterial pressure (55 +/- 6 vs 88 +/- 3 mm Hg), peak airway pressure (44 +/- 5 vs 31 +/- 2 mm Hg), tidal volume (432 +/- 96 vs 758 +/- 93 mL), arterial pH (7.16 +/- 0.0 vs 7.26 +/- 0.04), and PaCO2 (52 +/- 6 vs 45 +/- 6 mm Hg). There was no mortality among the group, and all patients underwent abdominal closure by fascial reapproximation in 2-5 days. Two (40%) of the five patients required extremity fasciotomy for compartment syndrome. HACS is a rare complication of

  9. Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome

    PubMed Central

    Zhang, Lili; Wu, Xiuhua; Wang, Laifang; Li, Jing; Chen, Hua; Zhao, Yan; Zheng, Wenjie

    2016-01-01

    Abstract The aim of the study was to investigate the clinical features of systemic lupus erythematous (SLE) complicated with Evans syndrome (ES). We conducted a retrospective case–control study to compare the clinical and laboratory features of age- and gender-matched lupus patients with and without ES in 1:3 ratios. In 5724 hospitalized SLE patients, we identified 27 (0.47%, 22 women and 5 men, average age 34.2 years) SLE patients complicated with ES. Fifteen patients (55.6%) presented with hematologic abnormalities initially, including 6 (22.2%) cases of isolated ITP, 4 (14.8%) cases of isolated AIHA, and 5 (18.5%) cases of classical ES. The median intervals between hematological presentations the diagnosis of SLE was 36 months (range 0–252). ES developed after the SLE diagnosis in 4 patients (14.8%), and concomitantly with SLE diagnosis in 8 patients (29.6%). Systemic involvements are frequently observed in SLE patients with ES, including fever (55.6%), serositis (51.9%), hair loss (40.7%), lupus nephritis (37%), Raynaud phenomenon (33.3%), neuropsychiatric (33.3%) and pulmonary involvement (25.9%), and photosensitivity (25.9%). The incidence of photosensitivity, hypocomplementemia, elevated serum IgG level, and lupus nephritis in patients with ES or without ES was 25.9% vs 6.2% (P = 0.007), 88.9% vs 67.1% (P = 0.029), 48.1% vs 24.4% (P = 0.021), and 37% vs 64.2% (P = 0.013), respectively. Twenty-five (92.6%) patients achieved improvement following treatment of glucocorticoids and immunosuppressants as well as splenectomy, whereas 6 patients experienced the relapse and 1 patient died from renal failure during the follow-up. ES is a relatively rare complication of SLE. Photosensitivity, hypocomplementemia, and elevated serum IgG level were frequently observed in ES patients, but lupus nephritis was less observed. More than half of patients presented with hematological manifestation at onset, and progress to typical lupus over months to years

  10. Compartment syndrome as a complication of ileofemoral deep venous thrombosis:a case presentation.

    PubMed

    Lamborn, David R; Schranz, Craig

    2014-02-01

    A 22-year-old morbidly obese, nonpregnant woman presented with left ileofemoral deep vein thrombosis (DVT) presenting as low back pain and bilateral, left greater than right, leg swelling and pain for 2 days. While on heparin, she developed compartment syndrome in her left leg and had evidence of dead muscle tissue at the time of fasciotomy. Three options exist for treatment of ileofemoral DVT: catheter-directed thrombolysis (CDT), CDT plus pharmacomechanical thrombolysis or percutaneous mechanical thrombectomy, and surgical thrombectomy. Catheter-directed thrombolysis alone or in conjunction with pharmacomechanical thrombolysis in patients with low risk of bleeding has shown significant lysis of occlusion in 79% of patients with ileofemoral DVT with relatively low complication rates. Surgical thrombectomy and fasciotomy have not proven to be as effective but are appropriate alternatives if CDT is not available. Standard anticoagulation alone is likely not a sufficient treatment for ileofemoral DVT. Other therapies including CDT, CDT plus pharmacomechanical thrombolysis or percutaneous mechanical thrombectomy, or surgical thrombectomy to address lysis of the clot should be attempted first or in conjunction with anticoagulation for appropriate patients. Catheter-directed thrombolysis with or without pharmacomechanical thrombolysis is the preferred initial treatment. PMID:24091199

  11. A Rare Case of Subclinical Primary Aldosteronism and Subclinical Cushing's Syndrome without Cardiovascular Complications.

    PubMed

    Kitajima, Natsumi; Seki, Toshiro; Yasuda, Atsushi; Oki, Masayuki; Takagi, Atsushi; Hanai, Kazuya; Terachi, Toshiro; Fukagawa, Masafumi

    2016-04-01

    We report a rare case of subclinical primary aldosteronism (PA) and subclinical Cushing's syndrome (CS). A 49-year-old woman was referred to our hospital for the evaluation of an adrenal incidentaloma. The patient had no previous medical history and no family history of notable illness. Her blood pressure was 103/60 mmHg. She had no Cushingoid features. Routine laboratory examinations were within the normal ranges including normokalemia. Based on the endocrinological results and imaging findings, we finally made a diagnosis of subclinical PA caused by both adrenal glands and subclinical CS caused by bilateral adrenal tumors. Interestingly, this patient had no risk factors for cardiovascular disease. In addition, the optimal management of patients with subclinical CS and subclinical PA has not been established. Therefore, we are observing her without medical therapy. Four years after diagnosis, no cardiovascular complications have been detected, including cerebral infarction, chronic kidney disease, cardiomegaly on echocardiography, and atherosclerosis on carotid ultrasonography. One important question is why the excessive hormone secretion did not affect the cardiovascular status of this patient. In this regard, we discuss several possible mechanisms including mineralocorticoid resistance and glucocorticoid sensitivity. PMID:27050894

  12. Warfarin-induced venous limb ischemia/gangrene complicating cancer: a novel and clinically distinct syndrome.

    PubMed

    Warkentin, Theodore E; Cook, Richard J; Sarode, Ravi; Sloane, Debi A; Crowther, Mark A

    2015-07-23

    Venous limb gangrene (VLG) can occur in cancer patients, but the clinical picture and pathogenesis remain uncertain. We identified 10 patients with metastatic cancer (7 pathologically proven) who developed severe venous limb ischemia (phlegmasia/VLG) after initiating treatment of deep-vein thrombosis (DVT); in 8 patients, cancer was not known or suspected at presentation. The patients exhibited a novel, clinically distinct syndrome: warfarin-associated supratherapeutic international normalized ratio (INR; median, 6.5) at onset of limb ischemia, rising platelet count during heparin anticoagulation, and platelet fall after stopping heparin. Despite supratherapeutic INRs, patient plasma contained markedly elevated thrombin-antithrombin (TAT) complex levels (indicating uncontrolled thrombin generation) and protein C (PC) depletion; this profile resembles the greatly elevated TAT/PC activity ratios reported in patients with warfarin-associated VLG complicating heparin-induced thrombocytopenia. Analyses of vitamin K-dependent factors in 6 cancer patients with available serial plasma samples showed that variations in the INR corresponded most closely with changes in factor VII, with a highly collinear relationship between VII and PC. We conclude that venous limb ischemia/gangrene is explained in some cancer patients by profoundly disturbed procoagulant-anticoagulant balance, whereby warfarin fails to block cancer-associated hypercoagulability while nonetheless contributing to severe PC depletion, manifest as a characteristic supratherapeutic INR caused by parallel severe factor VII depletion. PMID:25979950

  13. Drug Hypersensitivity: Pharmacogenetics and Clinical Syndromes

    PubMed Central

    Phillips, Elizabeth J.; Chung, Wen-Hung; Mockenhaupt, Maja; Roujeau, Jean-Claude; Mallal, Simon A.

    2011-01-01

    Severe cutaneous adverse reactions (SCARs) include syndromes such as drug reaction, eosinophilia and systemic symptoms (DRESS) or drug-induced hypersensitivity syndrome (DIHS) and Stevens-Johnson Syndrome/Toxic epidermal necrolysis (SJS/TEN). An important advance has been the discovery of associations between HLA alleles and many of these syndromes including abacavir hypersensitivity reaction, allopurinol DRESS/DIHS and SJS/TEN and SJS/TEN associated with aromatic amine anticonvulsants. These HLA associations have created the promise for prevention through screening and have additionally shed further light on the immunopathogenesis of SCARs. The roll-out of HLA-B*5701 into routine clinical practice as a genetic screening test to prevent abacavir hypersensitivity provides a translational roadmap for other drugs. Numerous hurdles exist in the widespread translation of several other drugs such as carbamazepine where the positive predictive value of HLA-B*1502 is low and the negative predictive value of HLA-B*1502 for SJS/TEN may not be 100% in all ethnic groups. International collaborative consortia have been formed with the goal of developing phenotype standardization and undertaking HLA and genome-wide analyses in diverse populations with these syndromes. PMID:21354501

  14. Factors associated with major complications in the short-term postoperative period in dogs undergoing surgery for brachycephalic airway syndrome.

    PubMed

    Ree, Jennifer J; Milovancev, Milan; MacIntyre, Laura A; Townsend, Katy L

    2016-09-01

    Surgical correction of brachycephalic airway syndrome (BAS) in dogs has been reported to result in low complication rates and good long-term outcomes. Previous reports have not identified risk factors for the development of complications following BAS surgery. This retrospective study evaluated a wide variety of patient- and procedure-related, pre-operative, intra-operative, and post-operative factors for an association with the development of major postoperative complications in the short-term period following BAS surgery. The overall major complication rate, including death or euthanasia, was 4/55 (7%) dogs. Temporary tracheostomy was the only major surgical complication identified (n = 3). Multiple logistic regression identified postoperative radiographic evidence of pneumonia as associated with the development of any major complication overall, requirement of a temporary tracheostomy postoperatively, and death or euthanasia, within the short-term postoperative period. Future prospective studies should evaluate specific risk factors for an association with major complications following BAS surgery in dogs to improve patient outcomes. PMID:27587891

  15. Prenatal management and perinatal outcome in giant placental chorioangioma complicated with hydrops fetalis, fetal anemia and maternal mirror syndrome

    PubMed Central

    2012-01-01

    Background Giant placental chorioangiomas have been associated with a number of severe fetal complications and high perinatal mortality. Case presentation We report a case of giant chorioangioma with fetal hydrops, additionally complicated by severe anemia, mild cardiomegaly with hyperdinamic heart circulation and maternal mirror syndrome. Intrauterine blood transfusion and amniodrainage was performed at 29 weeks. Worsening of the fetal and maternal condition prompted us to proceed with delivery at 29 + 5 weeks. The newborn died 3 hours later due to pulmonary hypoplasia and hemodynamic failure. Maternal course was favourable, mirror syndrome resolved in the second day and the patient was discharged four days following delivery. Conclusions In the case described here, fetal condition got worse despite of the anemia correction and amniodrainage. Our outcome raises the issue whether additional intrauterine clinical intervention, as intersticial laser, should have been performed to stop further deterioration of the fetal condition when progressive severe hydrops develops. PMID:22840187

  16. Clinical correlates of complicated grief among individuals with acute coronary syndromes

    PubMed Central

    Pini, Stefano; Gesi, Camilla; Abelli, Marianna; Cardini, Alessandra; Lari, Lisa; Felice, Francesca; Di Stefano, Rossella; Mazzotta, Gianfranco; Bovenzi, Francesco; Bertoli, Daniele; Borelli, Lucia; Michi, Paola; Oligeri, Claudia; Balbarini, Alberto; Manicavasagar, Vijaya

    2015-01-01

    Objective The study aimed at exploring bereavement and complicated grief (CG) symptoms among subjects without a history of coronary heart disease (CHD) at the time of a first acute coronary syndrome (ACS) and to evaluate the relationship of CG symptoms and ACS. Method Overall, 149 subjects with ACS (namely, acute myocardial infarct with or without ST-segment elevation or unstable angina), with no previous history of CHD, admitted to three cardiac intensive care units were included and evaluated by the Structured Clinical Interview for Complicated Grief (SCI-CG), Hamilton Depression Rating Scale, Hamilton Anxiety Rating Scale, and the 36-item Short-Form Health Survey (MOS-SF-36). Results Of the total sample of 149 subjects with ACS, 118 (79.2%) met criteria for DSM-5 persistent complex bereavement disorder. Among these, subjects who lost a partner, child, or sibling were older (P=0.008), less likely to be working (P=0.032), and more likely to be suffering from hypertension (P=0.021), returned higher scores on the SCI-CG (P=0.001) and developed the index ACS more frequently between 12 and 48 months after the death than those who lost a parent or another relative (P≤0.0001). The occurrence of ACS 12–48 months (P=0.019) after the loss was positively correlated with SCI-CG scores. An inverse relationship with SCI-CG scores was observed for patients who experienced ACS more than 48 months after the loss (P=0.005). The SCI-CG scores significantly predicted lower scores on the “general health” domain of MOS-SF-36 (P=0.030), as well as lower scores on “emotional well-being” domain (P=0.010). Conclusion A great proportion of subjects with ACS report the loss of a loved one. Among these, the loss of a close relative and the severity of CG symptoms are associated with poorer health status. Our data corroborate previous data indicating a strong relationship between CG symptoms and severe cardiac problems. PMID:26504390

  17. [The SAPHO syndrome or psoriatic arthritis: a complicated differential diagnosis. Apropos 3 cases].

    PubMed

    Sanjuán Portugal, F; Manero Ruiz, F J; Figueras Villalba, P; Martínez Alvarez, R

    1995-04-01

    SAPHO syndrome is characterized by osteoarticular involvement of ventral chest wall in the form of sternocostoclavicular osteoarthritis and hyperostosis and skin changes such as palmoplantar pustulosis and acne. Occasionally, psoriatic lesions and sacroiliitis are observed. However, despite the higher frequency of psoriasis in this syndrome, its inclusion in psoriatic arthropathy spectrum is not clearly established. The authors report three cases of SAPHO syndrome in psoriatic patients commenting on the difficulty in differentiating this entity from psoriatic arthritis as well as its relationship with seronegative spondyloarthropathies. This disease has been described mainly in Japan and only a few cases of this disease have been reported in the European or American literature. PMID:7784657

  18. Analysis of Families with Lynch Syndrome Complicated by Advanced Serrated Neoplasia: The Importance of Pathology Review and Pedigree Analysis

    PubMed Central

    Walsh, Michael D; Buchanan, Daniel D; Walters, Rhiannon; Roberts, Aedan; Arnold, Sven; McKeone, Diane; Clendenning, Mark; Ruszkiewicz, Andrew R; Jenkins, Mark A; Hopper, John L; Goldblatt, Jack; George, Jillian; Suthers, Graeme K; Phillips, Kerry; Young, Graeme P; Macrae, Finlay; Drini, Musa; Woods, Michael O; Parry, Susan; Jass, Jeremy R; Young, Joanne P

    2009-01-01

    The identification of Lynch syndrome has been greatly assisted by the advent of tumour immunohistochemistry (IHC) for mismatch repair (MMR) proteins, and by the recognition of the role of acquired somatic BRAF mutation in sporadic MMR-deficient colorectal cancer (CRC). However, somatic BRAF mutation may also be present in the tumours in families with a predisposition to develop serrated polyps in the colorectum. In a subgroup of affected members in these families, CRCs emerge which demonstrate clear evidence of MMR deficiency with absent MLH1 staining and high-level microsatellite instability (MSI). This may result in these families being erroneously classified as Lynch syndrome or, conversely, an individual is considered “sporadic” due to the presence of a somatic BRAF mutation in a tumour. In this report, we describe two Lynch syndrome families who demonstrated several such inconsistencies. In one family, IHC deficiency of both MSH2 and MLH1 was demonstrated in tumours from different affected family members, presenting a confusing diagnostic picture. In the second family, MLH1 loss was observed in the lesions of both MLH1 mutation carriers and those who showed normal MLH1 germline sequence. Both families had Lynch syndrome complicated by an independently segregating serrated neoplasia phenotype, suggesting that in families such as these, tumour and germline studies of several key members, rather than of a single proband, are indicated to clarify the spectrum of risk. PMID:19241144

  19. Abdominal compartment syndrome as a rare complication following component separation repair: case report and review of the literature.

    PubMed

    Oliver-Allen, H S; Hunter, C; Lee, G K

    2015-04-01

    One of the most feared complications following a massive ventral hernia repair is abdominal compartment syndrome (ACS). ACS is caused by an acute increase in intra-abdominal pressure (IAP), which can lead to multi-organ dysfunction and ultimately result in death. Component separation repair (CST) has been successful for most large hernia repairs in reducing the risk of ACS by increasing abdominal volume and reducing abdominal wall tension during a tight closure. However, reduction of a large hernia can lead to elevated IAP and possible progression to ACS. Here, we describe the detailed intra-operative and post-operative course of a patient who developed abdominal compartment syndrome following CST repair. PMID:25739715

  20. Acute abdominal compartment syndrome as a complication of Holmium laser enucleation of the prostate: a case report

    PubMed Central

    2014-01-01

    Background In 1996, Holmium laser enucleation of the prostate was introduced and has been shown to be safe and highly effective. Case presentation We report a case of a rare complication that resulted in intra-abdominal compartment syndrome with prolonged intubation and intensive care, involving an 74-year-old male after holmium laser enucleation of prostate, with a massive irrigant fluid leakage into the retroperitoneal space. The elevated abdominal pressure was reduced by forced diuresis. The tracheal tube was removed 18 hours after the patient’s transfer to the ICU. The patient was discharged to home one week after the operation. Conclusion In rare cases when no obvious ruptures of the prostate capsule or the bladder occur during laser enucleation of prostate, knowledge regarding possible emersion of massive amounts of irrigant fluid into the retroperitoneal space leading to intra-abdominal compartment syndrome aids in the diagnosis and subsequent successful therapy of intra-abdominal hypertension. PMID:24910537

  1. Tetrabenazine-induced oculogyric crisis - a rare complication in the treatment of Gilles de la Tourette syndrome.

    PubMed

    Janik, Piotr; Figura, Monika

    2016-01-01

    Tetrabenazine is used in the treatment of chorea, tardive dyskinesia, tics, and dystonia. It rarely causes acute eyeball dystonia and the description of this complication in Gilles de la Tourette syndrome is limited. We provide a description of an acute oculogyric crisis caused by tetrabenazine in a patient with severe tics. The patient had never developed acute dystonic reactions, although he was previously exposed to numerous dopamine receptor-blocking agents. After 8 days of therapy with tetrabenazine at a dose of 62.5 mg daily, the patient developed involuntary movement of the eyeballs. Withdrawal of tetrabenazine caused resolution of all symptoms after a week. The purpose of this description is to draw attention to the potential of tetrabenazine to induce acute oculogyric crisis as well as the difficulty of differentiating drug-induced dystonia from dystonic tics in patients with Gilles de la Tourette syndrome. PMID:26955276

  2. Tetrabenazine-induced oculogyric crisis – a rare complication in the treatment of Gilles de la Tourette syndrome

    PubMed Central

    Janik, Piotr; Figura, Monika

    2016-01-01

    Tetrabenazine is used in the treatment of chorea, tardive dyskinesia, tics, and dystonia. It rarely causes acute eyeball dystonia and the description of this complication in Gilles de la Tourette syndrome is limited. We provide a description of an acute oculogyric crisis caused by tetrabenazine in a patient with severe tics. The patient had never developed acute dystonic reactions, although he was previously exposed to numerous dopamine receptor-blocking agents. After 8 days of therapy with tetrabenazine at a dose of 62.5 mg daily, the patient developed involuntary movement of the eyeballs. Withdrawal of tetrabenazine caused resolution of all symptoms after a week. The purpose of this description is to draw attention to the potential of tetrabenazine to induce acute oculogyric crisis as well as the difficulty of differentiating drug-induced dystonia from dystonic tics in patients with Gilles de la Tourette syndrome. PMID:26955276

  3. Sjögren's syndrome: diagnosis and management of oral complications.

    PubMed

    Lilly, J P; Fotos, P G

    1996-01-01

    Sjogren's syndrome, a common autoimmune disease process, is characterized by destruction of exocrine (including the lacrimal and salivary) glands. Patients with this disorder may initially complain of a dry mouth or have rapid onset of new carious lesions. Here, a case of Sjogren's syndrome is presented that was initially diagnosed because of dental complaints, and long-term treatment of Sjogren's patients is discussed. PMID:9171038

  4. Complicated endoscopic management of Bouveret's syndrome. A case report and review.

    PubMed

    Moschos, John; Pilpilidis, Ioannis; Antonopoulos, Zissis; Paikos, Dimitrios; Tzilves, Dimitrios; Kadis, Savvas; Katsos, Ioannis; Tarpagos, Anestis

    2005-03-01

    A rare case of gastric outlet obstruction due to a large gallstone (Bouveret's syndrome) presenting with abdominal pain and vomiting is reported. The endoscopic management of the gallstone was unsuccessful because of the size of the stone that got stuck in oesophagus. The final management of the patient was surgical and the gallstone was retrieved via a gastrotomy. The case report is followed by a brief review of Bouveret's syndrome. PMID:15800698

  5. Syndrome of iron pill inhalation in four patients with accidental tablet aspiration: Severe airway complications are described

    PubMed Central

    Caterino, U.; Battistoni, P.; Batzella, S.; Iacono, R. Dello; Lucantoni, G.; Galluccio, G.

    2015-01-01

    Iron pill inhalation represents a uncommon cause of syntomatic endobronchial foreign bodies. Unlike foreign body, the direct contact of iron tablet onto the bronchial mucosa results in severe bronchial damage in addition to obstruction and local irritation. Four patients with Iron Pill Inhalation Syndrome are described. All but one patient developed irreversible bronchial stenosis as late post inflammatory complication. Bronchoscopic features and clinical evolution are described in order to reduce the risk of severe side-effects in patients highly suspected for iron pill aspiration. PMID:26236596

  6. Syndrome of iron pill inhalation in four patients with accidental tablet aspiration: Severe airway complications are described.

    PubMed

    Caterino, U; Battistoni, P; Batzella, S; Iacono, R Dello; Lucantoni, G; Galluccio, G

    2015-01-01

    Iron pill inhalation represents a uncommon cause of syntomatic endobronchial foreign bodies. Unlike foreign body, the direct contact of iron tablet onto the bronchial mucosa results in severe bronchial damage in addition to obstruction and local irritation. Four patients with Iron Pill Inhalation Syndrome are described. All but one patient developed irreversible bronchial stenosis as late post inflammatory complication. Bronchoscopic features and clinical evolution are described in order to reduce the risk of severe side-effects in patients highly suspected for iron pill aspiration. PMID:26236596

  7. Multiple organ dysfunction syndrome, an unusual complication of heroin intoxication: a case report and review of literature

    PubMed Central

    Feng, Gang; Luo, Qiancheng; Guo, Enwei; Yao, Yulan; Yang, Feng; Zhang, Bingyu; Li, Longxuan

    2015-01-01

    Multiple organ dysfunction syndrome (MODS) has rarely been described in patients with heroin intoxication. Here, we report a rare case of MODS involving six organs, due to heroin intoxication. The patient was a 32-year-old Chinese man with severe heroin intoxication complicated by acute pulmonary edema and respiratory insufficiency, shock, myocardial damage and cardiac insufficiency, rhabdomyolysis and acute renal insufficiency, acute liver injury and hepatic insufficiency, toxic leukoencephalopathy, and hypoglycemia. He managed to survive and was discharged after 10 weeks of intensive care. The possible pathogenesis and therapeutic measures of MODS induced by heroin intoxication and some suggestions for preventing and treating severe complications of heroin intoxication, based on clinical evidence and the pertinent literature, are discussed in this report. PMID:26617935

  8. Specific complications of monochorionic twin pregnancies: twin-twin transfusion syndrome and twin reversed arterial perfusion sequence.

    PubMed

    Chalouhi, G E; Stirnemann, J J; Salomon, L J; Essaoui, M; Quibel, T; Ville, Y

    2010-12-01

    Monochorionic twins are subjected to specific complications which originate in either imbalance or abnormality of the single placenta serving two twins. This unequal placental sharing can cause complications including twin-twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence (TAPS), selective intrauterine growth restriction or twin reversed arterial perfusion sequence (TRAP). Monochorionicity also makes the management of these specific complications as well as that of a severe malformation in one twin hazardous since the spontaneous death of one twin exposes the co-twin to a risk of exsanguination into the dead twin and its placenta. The latter is responsible for the death of the co-twin in up to 20% of the cases and in ischemic sequelae in about the same proportions in the survivors. Although the symptoms of all these complications are very different, the keystone of their management comes down to either surgical destruction of the inter-twin anastomoses on the chorionic plate when aiming at dual survival or selective and permanent occlusion of the cord of a severely affected twin aiming at protecting the normal co-twin. This can be best achieved by fetoscopic selective laser coagulation and bipolar forceps cord coagulation respectively. PMID:20855238

  9. Vasculitis complicating granulocyte colony stimulating factor treatment of leukopenia and infection in Felty's syndrome.

    PubMed

    Farhey, Y D; Herman, J H

    1995-06-01

    Recombinant myeloid growth factors have been increasingly used in recent years to combat induced and disease associated neutropenia. Their application in the management of Felty's syndrome with intercurrent infection has raised concern that resultant neutrophilia and activation of a diverse array of polymorphonuclear cell functions may have an adverse effect on the rheumatoid disease process. We describe a patient with Felty's syndrome receiving short term treatment with recombinant human granulocyte colony stimulating factor (GCSF), who then developed acute renal failure in conjunction with leukocytoclastic vasculitis and presumptive gout. We address the issue of "adding fuel to the fire" and review reported implications of GCSF in induction of vasculitis. PMID:7545756

  10. A case of Fournier gangrene complicating idiopathic nephrotic syndrome of childhood.

    PubMed

    Wright, A J; Lall, A; Gransden, W R; Joyce, M R; Rowsell, A; Clark, G

    1999-11-01

    A 10-year-old boy presenting with steroid resistant nephrotic syndrome developed Fournier gangrene of the scrotum. Antimicrobial drug therapy, intravenous albumin, excision of necrotic scrotum and left orchidectomy followed by skin grafting 3 weeks later led to an excellent cosmetic and medical result. Six months later he remains nephrotic on diuretic and angiotensin converting enzyme inhibitor medication. PMID:10603132

  11. Occam Paradox? A Variation of Tapia Syndrome and an Unreported Complication of Guidewire-Assisted Pedicle Screw Insertion

    PubMed Central

    Emohare, Osa; Peterson, Erik; Slinkard, Nathaniel; Janus, Seth; Morgan, Robert

    2013-01-01

    Study Design Case report. Clinical Question The clinical aim is to report on a previously unknown association between guidewire-assisted pedicle screw insertion and neuropraxia of the recurrent laryngeal nerve (RLN), and how this may overlap with the signs of Tapia syndrome; we also report our approach to the clinical management of this patient. Methods A 17-year-old male patient with idiopathic scoliosis experienced Tapia syndrome after posterior instrumentation and arthrodesis at the level of T1–L1. After extubation, the patient had a hoarse voice and difficulty in swallowing. Imaging showed a breach in the cortex of the anterior body of T1 corresponding to the RLN on the right. Results Otolaryngological examination noted right vocal fold immobility, decreased sensation of the endolarynx, and pooling of secretions on flexible laryngoscopy that indicated right-sided cranial nerve X injury and left-sided tongue deviation. Aspiration during a modified barium swallow prompted insertion of a percutaneous endoscopic gastrostomy tube before the patient was sent home. On postoperative day 20, a barium swallow demonstrated reduced aspiration, and the patient reported complete resolution of symptoms. The feeding tube was removed, and the patient resumed a normal diet 1 month later. Tapia syndrome, or persistent unilateral laryngeal and hypoglossal paralysis, is an uncommon neuropraxia, which has previously not been observed in association with a breached vertebral body at T1 along the course of the RLN. Conclusion Tapia syndrome should be a differential diagnostic consideration whenever these symptoms persist postoperatively and spine surgeons should be aware of this as a potential complication of guidewires in spinal instrumentation. PMID:24436711

  12. Beneficial role of bitter melon supplementation in obesity and related complications in metabolic syndrome.

    PubMed

    Alam, Md Ashraful; Uddin, Riaz; Subhan, Nusrat; Rahman, Md Mahbubur; Jain, Preeti; Reza, Hasan Mahmud

    2015-01-01

    Diabetes, obesity, and metabolic syndrome are becoming epidemic both in developed and developing countries in recent years. Complementary and alternative medicines have been used since ancient era for the treatment of diabetes and cardiovascular diseases. Bitter melon is widely used as vegetables in daily food in Bangladesh and several other countries in Asia. The fruits extract of bitter melon showed strong antioxidant and hypoglycemic activities in experimental condition both in vivo and in vitro. Recent scientific evaluation of this plant extracts also showed potential therapeutic benefit in diabetes and obesity related metabolic dysfunction in experimental animals and clinical studies. These beneficial effects are mediated probably by inducing lipid and fat metabolizing gene expression and increasing the function of AMPK and PPARs, and so forth. This review will thus focus on the recent findings on beneficial effect of Momordica charantia extracts on metabolic syndrome and discuss its potential mechanism of actions. PMID:25650336

  13. Beneficial Role of Bitter Melon Supplementation in Obesity and Related Complications in Metabolic Syndrome

    PubMed Central

    Subhan, Nusrat; Rahman, Md Mahbubur; Jain, Preeti; Reza, Hasan Mahmud

    2015-01-01

    Diabetes, obesity, and metabolic syndrome are becoming epidemic both in developed and developing countries in recent years. Complementary and alternative medicines have been used since ancient era for the treatment of diabetes and cardiovascular diseases. Bitter melon is widely used as vegetables in daily food in Bangladesh and several other countries in Asia. The fruits extract of bitter melon showed strong antioxidant and hypoglycemic activities in experimental condition both in vivo and in vitro. Recent scientific evaluation of this plant extracts also showed potential therapeutic benefit in diabetes and obesity related metabolic dysfunction in experimental animals and clinical studies. These beneficial effects are mediated probably by inducing lipid and fat metabolizing gene expression and increasing the function of AMPK and PPARs, and so forth. This review will thus focus on the recent findings on beneficial effect of Momordica charantia extracts on metabolic syndrome and discuss its potential mechanism of actions. PMID:25650336

  14. Orbital Lipomatosis: A Complication of Steroid Therapy in the Sweet Syndrome.

    PubMed

    Da Costa, Gwendoline; Pare, Arnaud; Sury, Florent; Goga, Dominque; Laure, Boris

    2016-01-01

    The description of a Sweet syndrome steroid dependant-induced orbital lipomatosis is reported. A 76-year-old-man with history of Sweet syndrome presented with severe bilateral proptosis (Hertel value, 25 mm) with decreased visual acuity and evoked potentials lengthened. A bilateral transpalpebral orbital decompression was performed by resection of intraorbital fat without bone removal. The surgery was uneventful. The volume of resected orbital fat was 15 ml for both sides. Proptosis reduction was 6 mm. Postoperative Hertel values were 19 mm, and evoked potentials were improved. The proptosis was managed successfully. Orbital lipectomy led to minimal sequelae and may be repeated if necessary in this case. PMID:25120096

  15. Association between primary Sjögren's syndrome and pregnancy complications: a systematic review and meta-analysis.

    PubMed

    Upala, Sikarin; Yong, Wai Chung; Sanguankeo, Anawin

    2016-08-01

    Systemic autoimmune disorders may interfere with normal reproductive function resulting in negative outcome of pregnancy. Primary Sjögren's syndrome (pSS) is a common rheumatic disease that mostly affects females. There are many reports that this condition may increase risk of pregnancy complications and fetal loss. However, data regarding these adverse outcomes are scarce and inconclusive. We performed a systematic review and meta-analysis of available articles that assess the association between pSS and adverse pregnancy outcome. We comprehensively searched the databases of MEDLINE and EMBASE from their dates of inception to March 2016 and reviewed papers with validity criteria. A random-effects model was used to evaluate pregnancy complications in patients with pSS and healthy controls. From 20 full-text articles, 7 studies involving 544 patients and 1586 pregnancies were included in the meta-analysis. Fetal complications included spontaneous abortion, stillbirth, neonatal deaths, and intrauterine growth retardation. Compared with healthy pregnancy, patients with pSS had significantly higher chance of neonatal deaths (pooled odds ratio (OR) = 1.77, 95 % confidence interval (CI) 1.28 to 1.46, p = 0.01). However, there were no significant associations between pSS and premature birth (OR = 2.10, 95 % CI 0.59-7.46, p = 0.25), spontaneous abortion (OR = 1.46, 95 % CI 0.72-2.93, p = 0.29), artificial abortion (OR = 1.12, 95 % CI 0.52-2.61, p = 0.71), or stillbirth (OR = 1.05, 95 % CI 0.38-2.97, p = 0.92). There is an increased risk of fetal loss in pregnant patients with pSS. The presented evidence further supports multidisciplinary care for these patients to prevent complications during pregnancy. PMID:27271701

  16. Acute Abdominal Compartment Syndrome as a Complication of Percutaneous Nephrolithotomy: Two Cases Reports and Literature Review.

    PubMed

    Tao, Jing; Sheng, Lu; Zhang, Hao-Jie; Chen, Ran; Sun, Zhong-Quan; Qian, Wei-Qing

    2016-09-01

    Percutaneous nephrolithotomy (PCNL) is a technique commonly used to remove large or multiple kidney stones and stones in the inferior calyx, with the advantages of lower morbidity rates, decrease in post-operative pain with faster recovery. Intra-abdominal irrigation fluid extravasation which leads to abdominal hypertension is a rare complication of PCNL with little reports. Early detection of intra-abdominal extravagation is very important to prevent morbidity and mortality. We present two cases and review the literature. PMID:27313986

  17. Fatal diffuse pulmonary arterial thrombosis as a complication of nephrotic syndrome.

    PubMed

    Matsuda, Akiko; Tsuchiya, Ken; Yabuki, Yasuko; Naito, Masayo; Koike, Minako; Yumura, Wako; Nitta, Kosaku

    2007-12-01

    A 21-year-old man was admitted to our hospital because of leg edema. Because laboratory findings revealed massive proteinuria and hypoproteinemia, he was diagnosed as having nephritic syndrome caused by minimal change disease. He was given a continuous heparin infusion and intravenous steroid therapy, at a prednisolone dose of 1 mg/kg per day, and his condition gradually improved. Five months after discharge, the patient's proteinuria relapsed. He was readmitted to our hospital and we restarted anticoagulant treatment with intravenous heparin and 60 mg prednisolone. On the third hospital day, he complained of chest pain with sudden onset and dyspnea. He quickly developed shock and died. The findings of an autopsy confirmed the presence of diffuse fibrin thrombi in bilateral pulmonary arteries, and we diagnosed the cause of death as diffuse pulmonary artery thrombosis. A coagulation test for activated partial thromboplastin time (aPTT) had already shown that aPTT was prolonged before the initiation of treatment. There may have been a deficit of antithrombin III (ATIII) - a cofactor of heparin - because of the proteinuria; thus, the continuous heparin treatment might not have been effective for the prevention of thrombosis. Alternatives to heparin treatment that do not suppress AT III, such as nafamostat mesilate or argatroban, which do not require the presence of AT III for their anticoagulant action, should be considered in cases similar to the that in the patient reported here. In patients with nephrotic syndrome who exhibit altered coagulation test results, the choice of anticoagulation therapy for treatment of the hypercoagulabilty status associated with nephrotic syndrome should be carefully considered. PMID:18085394

  18. [A patient with Parkinson's disease complicated by hypothyroidism who developed malignant syndrome after discontinuation of etizolam].

    PubMed

    Kawajiri, Masakazu; Ohyagi, Yasumasa; Furuya, Hirokazu; Araki, Takehisa; Inoue, Naohide; Esaki, Shigemitsu; Yamada, Takeshi; Kira, Junichi

    2002-02-01

    A 59-year-old man, who was diagnosed as having Parkinson's disease and depression seven years ago and was on oral antiparkinsonian agents, antianxiety agents, and antidepressants, developed a high fever, disturbed consciousness, and marked muscle rigidity after discontinuation of etizolam and amitriptyline. He was admitted to a nearby hospital. Hypothyroidism had been noted two months before admission. Marked muscle rigidity and increased serum CK were observed. Since discontinuation of benzodiazepine has been known to rarely trigger a neuroleptic malignant syndrome (NMS), he was diagnosed as having NMS. After receiving dantrolene and bromocriptine, these symptoms temporarily improved but he again developed consciousness disturbance, and convulsive seizures associated with an elevated serum CK. He was transferred to our hospital. On admission, the CK level was normal at 168 IU/l, while free T4 was 0.6 ng/dl (normal range, 0.9-2.3) and TSH was 108.7 mU/ml (normal range, 0.2-4.2) in serum, indicating the presence of primary hypothyroidism. As an increase in thyroid hormone dosage improved the thyroid function to normal level, his disturbed consciousness and muscle rigidity gradually improved. Convulsive seizure and recurrence of NMS in a short interval are unusual in neuroleptic malignant syndrome. In this patient, hypothyroidism may have contributed to the development of malignant syndrome through metabolic changes of the central dopaminergic system, and discontinuation of etizolam, a kind of benzodiazepine, may have triggered NMS, since there has not been reported that discontinuation of antidepressants including amitriptyline triggers NMS. PMID:12424963

  19. Le syndrome de Budd-Chiari: une complication rare de la sarcoïdose hépatique (à propos d'un cas)

    PubMed Central

    Sghier, Ismael Ait; Billah, Nabil Moatassim

    2016-01-01

    L'atteinte hépatique au cours de la sarcoïdose est une localisation fréquente, habituellement asymptomatique. La cholestase anictérique et l'hypertension portale représentent ses principales complications. Le syndrome de Budd-Chiari est une complication peu connue qui demeure exceptionnelle. Nous rapportons un nouveau cas de syndrome de Budd-Chiari compliquant une sarcoïdose hépatique chez une jeune femme de 45 ans. PMID:27200114

  20. Neuromyelitis optica spectrum disorder complicated with Sjogren syndrome successfully treated with tocilizumab: A case report.

    PubMed

    Komai, Toshihiko; Shoda, Hirofumi; Yamaguchi, Kenichi; Sakurai, Keiichi; Shibuya, Mihoko; Kubo, Kanae; Takahashi, Toshiyuki; Fujio, Keishi; Yamamoto, Kazuhiko

    2016-01-01

    A 38-year-old woman with relapsing longitudinal extensive transverse myelitis and Sjogren's syndrome (SS) was admitted with lower extremity muscle weakness. Studies showed high serum titer of anti-aquaporin4 antibody and gadolinium-enhanced-MRI T1-weighted lesions within thoracic cord. Clinical findings suggested neuromyelitis optica-spectrum disorder (NMO-SD). High-dose corticosteroids, plasma exchange and cyclophosphamide were not effective. After starting tocilizumab, her neurological findings gradually improved. This report describes the first evidence to show tocilizumab could be effective for NMO-SD with SS. PMID:24313919

  1. Management of severe asymmetric pectus excavatum complicating aortic repair in a patient with Marfan's syndrome.

    PubMed

    Yeung, Jonathan C; Marcuzzi, Danny; Peterson, Mark D; Ko, Michael A

    2016-05-01

    We describe the case of a 28-year old man with Marfan's syndrome and severe pectus excavatum who required an aortic root replacement for an ascending aortic aneurysm. There was a near-vertical angulation of the sternum that presented challenges with opening and exposure of the heart during aortic surgery. Furthermore, removal of the sternal retractor after aortic repair resulted in sudden loss of cardiac output. A Ravitch procedure was then performed to successfully close the chest without further cardiovascular compromise. We propose that patients with a severe pectus excavatum and mediastinal displacement seen on preoperative CT scanning should be considered for simultaneous, elective repair. PMID:26874148

  2. Late postpartum eclampsia complicated with posterior reversible encephalopathy syndrome: a case report and a literature review

    PubMed Central

    Zhang, Lihong; Wang, Yacong; Shi, Liang; Cao, Jianhui

    2015-01-01

    Posterior reversible encephalopathy syndrome (PRES) is a rare but serious clinical-neuroradiological entity characterized by headache, vomiting, visual disturbances, altered mental status, seizures, and unconsciousness associated with the characteristic imaging findings including sub-cortical vasogenic edema at the bilateral parietal and occipital lobes. We describe a case of 28-year-old PRES patient secondary to delayed maternal postpartum eclampsia. This patient was not initially diagnosed with pre-eclampsia and PRES. The diagnosis was established after magnetic resonance imaging. After treatment this patient’s PRES resolved. Early diagnosis and treatment are the keys to reverse PRES. A literature review for PRES is provided in this report. PMID:26807372

  3. Excessive naked megakaryocyte nuclei in myelodysplastic syndrome mimicking idiopathic thrombocytopenic purpura: a complicated pre- and post-transplantation course.

    PubMed

    Olcay, Lale; Tuncer, A Murat; Okur, Hamza; Erdemli, Esra; Uysal, Zumrut; Cetin, Mualla; Duru, Feride; Cetinkaya, Duygu Uckan

    2009-09-01

    A boy 3 years 7 months old with thrombocytopenia and history of intracranial hemorrhage who underwent bone marrow transplantation is presented. He was refractory to steroids, immunoglobulin G, vincristine, azathioprine, cyclosporine A, interleukin-11, chemotherapy, and splenectomy. Idiopathic thrombocytopenic purpura was excluded by light /electron microscopic and flow cytometric findings; the diagnosis of refractory cytopenia, a subgroup of pediatric myelodysplastic syndrome, was made. Naked megakaryocyte nuclei were 55.38 +/- 28.2% vs. 31.67 +/- 23.22% of all megakaryocytes in the patient and the control group of 9 patients with idiopathic thrombocytopenic purpura, respectively (p = .016). The posttransplatation course was complicated by delayed platelet engraftment, bronchiolitis obliterans associated with pneumocystis carinii pneumonia, which resolved completely. PMID:19657988

  4. Major donor area complication after a mandibular reconstruction with an osseous fibular free flap: pseudo-compartment syndrome.

    PubMed

    Kuvat, Samet Vasfi; Keklik, Barş; Özden, Burcu Celet; Uçar, Adem; Cizmeci, Orhan

    2012-03-01

    The popularity of the fibular free flap in mandibular reconstructions is persisting, and major donor area complications rarely occur after fibular free flap operations. Still, we have observed a pseudo-compartment syndrome in a 52-year-old patient on the 12th postoperative day after a mandibular reconstruction with a fibular free flap. When an obstruction in the deep venous system (deep vein thrombosis) was observed in the Doppler ultrasound-guided imaging, the patient has been taken to the operating room for an emergency surgery and the donor area has been completely reopened (in the manner of a fasciotomy). After this procedure, the circulation in the foot appeared to return to normal. The exposed muscles of the patient, who was started on a low-molecular-weight heparin treatment for the deep vein thrombosis, have been closed with skin grafts on the 10th day. No functional loss was observed during the 2-month follow-up period. PMID:22421858

  5. Primary stomal lymphoma. An unusual complication of ileostomy in a patient with transfusion-related acquired immune deficiency syndrome.

    PubMed

    Levecq, H; Hautefeuille, M; Hoang, C; Galian, A; Hautefeuille, P; Rambaud, J C

    1990-02-15

    A 73-year-old heterosexual man developed a high-grade non-Hodgkin's lymphoma at the site of an ileostomy only 2 years after proctectomy for undetermined colitis not cured by previous colectomy. In fact, the early occurrence of this usually very late and rare complication of ileostomy was probably favored by the simultaneous presence of acquired immune deficiency syndrome (AIDS) due to repeated blood transfusions for refractory anemia with excess blasts. The intestinal location of the tumor, its high-grade malignancy and B-cell origin are all features of AIDS-related non-Hodgkin's lymphoma. This case report seems to be one of the rarely identified examples of the cooperation between general predisposing factors and local irritating agents at the origin of a malignant tumor. PMID:2297651

  6. Spinal cord infarction as a rare complication of fat embolism syndrome following bilateral intramedullary nailing of femur fractures.

    PubMed

    Kearsley, RoseMarie; Galbraith, John; Dalton, David; Motherway, Catherine

    2016-01-01

    Fat embolism syndrome (FES) is a rare and potentially fatal complication occurring most often after long bone or pelvic fractures and orthopaedic procedures. It can consist of pulmonary, central nervous system and cutaneous manifestations. The exact pathophysiology of emboli reaching the arterial circulation is poorly understood.1 It is suggested that this may occur by either 'paradoxical' embolism or microembolism.2 3 Its true incidence is unknown but increases in the presence of multiple closed fractures. It can be a diagnostic dilemma for clinicians and if suspected diffusion-weighted MRI is the modality of choice for the investigation of the central nervous system.4 We present the case of a 22-year-old man who developed multifocal cerebral infarcts, a right-sided cerebellar infarct and an infarct in the anterior cord bilaterally at the level of C5-C6 as a result of FES. PMID:27624445

  7. Pyoderma gangrenosum complicated with myelodysplastic syndrome followed by rapidly progressing pyothorax-associated lymphoma: a case report.

    PubMed

    Goto, Akiko; Yamamoto, Satoshi; Notoya, Atsushi; Takada, Akio; Mukai, Masaya

    2006-07-01

    This report describes a patient with pyoderma gangrenosum (PG) complicated with myelodysplastic syndrome (MDS) followed by rapidly progressing pyothorax-associated lymphoma (PAL). A 74-year-old man was admitted with cutaneous gangrene associated with MDS. We diagnosed him as having PG, and high-dose oral prednisolone was started. Two months after admission he developed lymphoma rapidly. The patient died in spite of radiation therapy. On autopsy, the pathological diagnosis was diffuse large cell lymphoma. Epstein-Barr virus (EBV)-encoded RNA, and EBV-encoded nuclear antigen (EBNA) were detected in lymphoma cells. This case suggested that immunosuppressive therapy might favour the clonal proliferation of EBV-infected cells. PMID:16892654

  8. Endoscopic treatment for esophageal varices complicated by Isaacs' syndrome involving difficulty with conventional sedation.

    PubMed

    Suzuki, Yuhei; Yamazaki, Yuichi; Hashizume, Hiroaki; Kobayashi, Takeshi; Ohyama, Tatsuya; Horiguchi, Norio; Sato, Ken; Kakizaki, Satoru; Kusano, Motoyasu; Yamada, Masanobu

    2016-02-01

    A 54-year-old male consulted a local doctor with a chief complaint of systemic convulsions and muscle stiffness and was diagnosed with Isaacs' syndrome based on positive findings for antibodies against voltage-gated potassium channels in 2009. He subsequently experienced repeated hematemesis in 2013, at which time he was taken to our hospital by ambulance. Emergent endoscopy revealed esophageal varices with spurting bleeding. The bleeding was stopped with urgent endoscopic variceal ligation. Three days later, the patient developed sudden dyspnea with stridor during inspiration under sedation with an intravenous injection of low-dose flunitrazepam prior to receiving additional treatment and was aroused with intravenous flumazenil, after which his dyspnea immediately improved. Dyspnea may be induced by muscle cramps associated with Isaacs' syndrome exacerbated by sedation. Endoscopic variceal ligation was performed safely using multiple ligation devices in an awake state following pre-medication with hydroxyzine, without sudden dyspnea. Endoscopists should be cautious of the use of sedatives in patients with diseases associated with muscle twitching or stiffness, as in the current case. In addition, it is necessary to administer endoscopic treatment in an awake state or under conscious sedation in patients with a high risk of dyspnea. PMID:26862027

  9. Orbital apex syndrome and meningoencephalitis: a rare complication of herpes zoster

    PubMed Central

    Xiao, Zheman; Lu, Zuneng; Pan, Songqing; Liang, Jingjing; Liu, Zhongchun

    2015-01-01

    Orbital apex syndrome is a rare manifestation of Herpes Zoster (HZ). HZ Mmeningoencephalitis are also rare in immunocompetent persons. We report a rare case that was considered to be HZ meningoencephalitis with orbital apex syndrome in an immunocompetent female. The patient initially manifested with HZ skin lesions and lose of her left sight, diplopia, ptosis, followed by headache, neck pain, and fever, dizziness. Cerebrospinal fluid analysis showed elevation of lymphocytes and protein. A MRI abnormality was remarkable for the presence of a left cerebellum, occipital lobe as well as dura lesion. Head computed tomography and Magnetic resonance venography was normal. Corticosteroid therapy and antiviral therapy was effective to decrease the headache and skin pain. Symptoms were markedly improved after corticosteroid therapy. Three months later, we called the patient to follow up. Her meningoencephalitis symptom recovered. Her follow up brain MRI was normal. But left blindness and external ophthalmoplegia was persistent. This case suggested HZ could affect central nervous system and peripheral nervous system at the same time. PMID:26550408

  10. Late-onset cytomegalovirus infection complicated by Guillain-Barre syndrome in a kidney transplant recipient: case report and review of the literature.

    PubMed

    Shaban, E; Gohh, R; Knoll, B M

    2016-04-01

    Cytomegalovirus (CMV) infection remains a common infection after solid-organ transplantation. In the general population CMV disease is associated with Guillain-Barre syndrome (GBS), an autoimmune disease leading to an acute peripheral neuropathy, in 1 of 1000 cases. Interestingly, GBS is a rarely observed complication in solid-organ transplant recipients, possibly related to maintenance immunosuppression. We describe a case of CMV infection complicated by GBS in a kidney transplant recipient and review the literature. PMID:26141820

  11. [An autopsied case of chronic active Epstein-Barr virus infection complicated in systemic lupus erythematosus and antiphospholipid antibody syndrome].

    PubMed

    Ogawa, Jun; Koike, Ryuji; Sugihara, Takahiko; Hagiyama, Hiroyuki; Nishio, Junko; Kohsaka, Hitoshi; Kubota, Tetsuo; Kawachi, Hiroshi; Kasahara, Ichiro; Miyasaka, Nobuyuki

    2002-12-01

    We have experienced a case of chronic active Epstein-Barr virus infection (CAEBV) complicated in systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS). A 35-year-old woman was admitted to our hospital with complaints of fever and dyspnea on exertion. She was diagnosed as having SLE on the basis of arthritis, oropharyngeal ulcer, lymphopenia, and positive autoantibodies against DNA, RNP and SSA. The diagnosis of APS was also made because of positive anti-cardiolipin IgG antibodies and the existence of multiple pulmonary infarction with pulmonary hypertension. The administration of 30 mg/day of prednisolone and anti-coagulation significantly improved clinical symptoms. However, she was again admitted to the hospital four months later because of progressive liver damage and pancytopenia. Increment of prednisolone did not improve the clinical situation and she expired because of pulmonary hemorrhage. At autopsy, there were a significant increase of histiocytes with hemophagocytosis and a dense infiltration of atypical lymphocytes in the liver, spleen, lymph nodes and bone marrow. Infiltrated lymphocytes were positive for CD 3 and EBER 1 in immunohistochemical staining and EBVmRNA was detected by in situ hybridization. Final pathological diagnosis was CAEBV with hemophagocytic syndrome in association with lupus nephritis, pulmonary hemorrhage and pulmonary infarction. PMID:12599514

  12. Primary intracranial neuroendocrine tumor with ectopic adrenocorticotropic hormone syndrome: A rare and complicated case report and literature review

    PubMed Central

    LIU, HAILONG; ZHANG, MINGSHAN; WANG, XUAN; QU, YANMING; ZHANG, HONGWEI; YU, CHUNJIANG

    2016-01-01

    Neuroendocrine tumors (NETs) and ectopic adrenocorticotropic hormone (ACTH) syndrome are frequent in adult patients. However, primary intracranial NETs, exhibiting immunonegativity for ACTH, high serum ACTH level and treated with anterior skull base reconstruction, are rare and complicated. We herein present a case of a primary intracranial NET immunonegative for ACTH, resulting in ectopic ACTH syndrome. A 40-year-old woman presented with intermittent rhinorrhea, rapid weight gain, polydipsia, polyuria, hypertension, dimness, bilateral exophthalmus, diminution of vision in the left eye and pigmentation of the skin of the face and trunk. Computed tomography (CT) and magnetic resonance imaging scans revealed a sizeable enhancing tumor in the anterior cranial fossa, which infiltrated the sphenoid and ethmoid sinuses bilaterally, the left maxillary sinus and the nasal cavity. Abdominal CT scans revealed bilateral adrenal hyperplasia. The biochemical findings included hypokalemia and high glucose, cortisol, plasma ACTH, 24-h urinary free cortisol and testosterone levels. The neoplasm was exposed through a right frontal craniotomy, while anterior skull base reconstruction was performed during surgery. The intracranial surgery achieved gross removal of the tumor; however, part of the tumor remained in the nasal cavity. Histopathological examination of the surgical specimen confirmed the diagnosis of a low-grade small-cell NET, exhibiting immunonegativity for ACTH. A postoperative abdominal CT scan demonstrated bilateral regression of the adrenal gland hyperplasia and the serum ACTH level returned to normal after 16 days. To the best of our knowledge, there are no previous reports of primary intracranial NETs, immunohistochemically negative for ACTH, resulting in ectopic ACTH syndrome. PMID:27330775

  13. Nephrotic syndrome as a complication of chronic graft-versus-host disease after allogeneic haemopoietic stem cell transplantation.

    PubMed

    Wong, E; Lasica, M; He, S Z; Bajel, A; Roberts, A W; Mason, K D; Ritchie, D S; Szer, J

    2016-06-01

    Nephrotic syndrome (NS) is a rare complication following allogeneic haemopoietic stem cell transplantation (allo-HSCT), with limited current understanding of its pathogenesis. Here, we describe four cases of NS following allo-HSCT diagnosed at our institutions to identify key clinical and pathological features. In addition, a PubMed search was performed to identify existing reports that were pooled together with our cases for analysis. NS occurred as a late complication following allo-HSCT, with median onset 19.5 months after transplant (range: 3.9-84 months). The most common histopathology observed was membranous nephropathy; however, cases of minimal change disease have also been reported. There is a high incidence of prior extra-renal graft-versus-host disease (GvHD), with all four of our cases and 82% of published cases having prior GvHD. Glucocorticosteroids are the most common treatment, with variable degrees of response. Responses to immunosuppression with calcineurin inhibitors and rituximab have been described in steroid-refractory cases. PMID:27257151

  14. Nephrotic syndrome: an under-recognised immune-mediated complication of non-myeloablative allogeneic haematopoietic cell transplantation.

    PubMed

    Srinivasan, R; Balow, J E; Sabnis, S; Lundqvist, A; Igarashi, T; Takahashi, Y; Austin, H; Tisdale, J; Barrett, J; Geller, N; Childs, R

    2005-10-01

    Nephrotic syndrome (NS) is an extremely rare complication of myeloablative allogeneic haematopoietic cell transplantation (HCT) that usually occurs in association with chronic graft-versus-host disease (C-GVHD). We observed an unexpectedly high incidence of NS in a cohort of 163 consecutive patients undergoing non-myeloablative HCT from a related human leucocyte antigen-compatible donor. Seven patients developed NS at a median 318 d post-transplant (range 119-1203 d; cumulative incidence 6.1%). The median age at onset of NS was 46 years (range 33-59 years); three of the seven patients had no evidence of C-GVHD while four had accompanying limited C-GVHD. At diagnosis, median proteinuria was 16.5 g/24 h (range 3-24 g/24 h). Renal biopsy was performed in four cases and revealed membranous nephropathy. NS was not always associated with other symptoms of C-GVHD, and in contrast to previous reports, usually did not improve with the re-initiation of aggressive immunosuppression, resulting in progressive renal failure necessitating dialysis in three of seven cases. Membranous nephropathy resulting in NS is a previously unrecognised and clinically significant complication of non-myeloablative HCT. PMID:16173966

  15. Clival osteomyelitis and hypoglossal nerve palsy--rare complications of Lemierre's syndrome.

    PubMed

    He, Jingzhou; Lam, Jonathan Chun Leuk; Adlan, Tarig

    2015-01-01

    An increasingly reported entity, Lemierre's syndrome classically presents with a recent oropharyngeal infection, internal jugular vein thrombosis and the presence of anaerobic organisms such as Fusobacterium necrophorum. The authors report a normally fit and well 17-year-old boy who presented with severe sepsis following a 5-day history of a sore throat, myalgia and neck stiffness requiring intensive care admission. Blood cultures grew F. necrophorum and radiological investigations demonstrated left internal jugular vein, cavernous sinus and sigmoid sinus thrombus, left vertebral artery dissection and thrombus within the left internal carotid artery. Imaging also revealed two areas of acute ischaemia in the brain, consistent with septic emboli, skull base (clival) osteomyelitis and an extensive epidural abscess. The patient improved on meropenem and metronidazole and was warfarinised for his cavernous sinus thrombosis. He has an on-going left-sided hypoglossal (XIIth) nerve palsy. PMID:26323975

  16. Middle lobe syndrome: a singularly rare presentation of complicated pulmonary hydatid disease.

    PubMed

    Kunal, Shekhar; Pilaniya, Vikas; Shah, Ashok

    2016-01-01

    Imaging is crucial to the diagnosis of pulmonary hydatid disease, as it is often the first modality that raises suspicion of the disease. Middle lobe syndrome (MLS) as a presentation of this disease is a distinct rarity. A 45-year-old woman, a never-smoker, presented with cough and streaky haemoptysis. Imaging demonstrated a trapezoidal opacity with its base towards the hilum and contiguous with the right cardiac border. The reformatted sagittal view confirmed the diagnosis of MLS. Fibreoptic bronchoscopy (FOB) revealed an avascular white membranous structure wholly occluding the medial segment of the middle lobe. This was completely removed through gentle suction. Bronchial aspirate revealed hooklets of hydatid and echinococcal serology was positive. Subsequently, three cycles of albendazole were administered with remarkable clinical and radiological improvement. To the best of our knowledge, this is the first detailed description of MLS caused by pulmonary hydatidosis that was completely removed through FOB. PMID:27045051

  17.  An autoimmune polyglandular syndrome complicated with celiac disease and autoimmune hepatitis.

    PubMed

    Dieli-Crimi, Romina; Núñez, Concepción; Estrada, Lourdes; López-Palacios, Natalia

    2016-01-01

     Autoimmune polyglandular syndrome (APS) is a combination of different autoimmune diseases. The close relationship between immune-mediated disorders makes it mandatory to perform serological screening periodically in order to avoid delayed diagnosis of additional autoimmune diseases. We studied a patient with type 1 diabetes (T1D) who later developed an autoimmune thyroid disease (ATD) and was referred to our hospital with a serious condition of his clinical status. The patient was suffering from an advance stage of celiac disease (CD), the delay in its diagnosis and in the establishment of a gluten-free dietled the patient to a severe proteincalorie malnutrition. Later, the patient developed an autoimmune hepatitis (AIH). We consider that clinical deterioration in patients with APS should alert physicians about the possible presence of other immune-mediated diseases. Periodic screening for autoantibodies would help to prevent delayed diagnosis and would improve patient's quality of life. PMID:27236159

  18. Hidden in plain sight: macrophage activation syndrome complicating Adult Onset Still's Disease.

    PubMed

    Benitez, Lourdes; Vila, Salvador; Mellado, Robert Hunter

    2010-01-01

    Hemophagocytic Lymphystiocytosis is a rare and fatal complication of rheumatic diseases, particularly Adult Onset Still's Disease (AOSD). It may be precipitated with immunosuppressive drugs and with viral and bacterial infections. A diagnosis depends on a high index of suspicion associated to certain clinical manifestations (fever, rash, Splemomegaly, any cytology blood dyscrasia, hipertrigliceridemia, hiperfibrinogenemia, and others), as well as pathologic evidence of hemophagocitosis from bone marrow biopsy or tissue samples of affected organs. Therapy consists of high dose corticosteroids and immunosuppressive drugs. We present a 42 year old woman with AOSD in remission who developed HLH in spite of receiving therapy with high dose steroids and immunosuppressive drugs. She had 2 negative bone marrow aspirates. Evidence of Hemophagocytosis was detected in both bone marrow biopsies. Timely evaluation and recognition of the signs and symptoms of HLH is crucial for the prompt management and a decrease in the mortality associated with this disease. PMID:23875527

  19. Complications of 5-azacytidine: Three cases of severe ischemic colitis in elderly patients with myelodysplastic syndrome.

    PubMed

    Melchardt, Thomas; Weiss, Lukas; Pleyer, Lisa; Steinkirchner, Susanne; Auberger, Jutta; Hopfinger, Georg; Greil, Richard; Egle, Alexander

    2013-12-01

    5-Azacytidine (5-AZA) was the first drug to be approved for the treatment of high-risk myelodysplastic syndrome (MDS). The adverse event profile of this drug appears favorable compared with the conventional intensive chemotherapy that is used for MDS or acute myeloid leukemia. However, uncommon adverse events may have remained undetected in the limited number of patients that have been treated to date. The present study describes three cases/66.8 person-years (4,491 cases/100,000 person-years) of severe ischemic colitis in a single center cohort of 95 patients who were consecutively treated using subcutaneous 5-AZA. The results demonstrated a much higher incidence of colitis compared with the rates in the general population or in patients of greater ages and co-morbidities. The present study investigated whether the combination of anemia and constipation due to the co-medication of 5-HT3 receptor antagonists may explain the three cases of ischemic colitis. PMID:24260071

  20. Complications of 5-azacytidine: Three cases of severe ischemic colitis in elderly patients with myelodysplastic syndrome

    PubMed Central

    MELCHARDT, THOMAS; WEISS, LUKAS; PLEYER, LISA; STEINKIRCHNER, SUSANNE; AUBERGER, JUTTA; HOPFINGER, GEORG; GREIL, RICHARD; EGLE, ALEXANDER

    2013-01-01

    5-Azacytidine (5-AZA) was the first drug to be approved for the treatment of high-risk myelodysplastic syndrome (MDS). The adverse event profile of this drug appears favorable compared with the conventional intensive chemotherapy that is used for MDS or acute myeloid leukemia. However, uncommon adverse events may have remained undetected in the limited number of patients that have been treated to date. The present study describes three cases/66.8 person-years (4,491 cases/100,000 person-years) of severe ischemic colitis in a single center cohort of 95 patients who were consecutively treated using subcutaneous 5-AZA. The results demonstrated a much higher incidence of colitis compared with the rates in the general population or in patients of greater ages and co-morbidities. The present study investigated whether the combination of anemia and constipation due to the co-medication of 5-HT3 receptor antagonists may explain the three cases of ischemic colitis. PMID:24260071

  1. Epoxyeicosatrienoic acids and heme oxygenase-1 interaction attenuates diabetes and metabolic syndrome complications

    PubMed Central

    Burgess, Angela; Vanella, Luca; Bellner, Lars; Schwartzman, Michal L.; Abraham, Nader G.

    2011-01-01

    MSCs are considered to be the natural precursors to adipocyte development through the process of adipogenesis. A link has been established between decreased protective effects of EETs or HO-1 and their interaction in metabolic syndrome. Decreases in HO-1 or EET were associated with an increase in adipocyte stem cell differentiation and increased levels of inflammatory cytokines. EET agonist (AKR-I-27-28) inhibited MSC-derived adipocytes and decreased the levels of inflammatory cytokines. We further describe the role of CYP-epoxygenase expression, HO expression, and circulating cytokine levels in an obese mouse, ob/ob−/− mouse model. Ex vivo measurements of EET expression within MSCs derived from ob/ob−/− showed decreased levels of EETs that were increased by HO induction. This review demonstrates that suppression of HO and EET systems exist in MSCs prior to the development of adipocyte dysfunction. Further, adipocyte dysfunction can be ameliorated by induction of HO-1 and CYP-epoxygenase, i.e. EET. PMID:22100745

  2. Life-Threatening Postpneumonectomy Syndrome Complicated with Right Aortic Arch after Left Pneumonectomy.

    PubMed

    Karasaki, Takahiro; Tanaka, Makoto

    2015-01-01

    A 54-year-old man with right aortic arch underwent left lower lobectomy and lingular segmentectomy, followed by complete pneumonectomy, for refractory nontuberculous mycobacterial infection. Three months after the pneumonectomy, he developed acute respiratory distress. Computed tomography showed an excessive mediastinal shift with an extremely narrowed bronchus intermedius and right lower bronchus compressed between the right pulmonary artery and the right descending aorta. Soon after the nearly obstructed bronchus intermedius was observed by bronchoscopy, he began to exhibit frequent hypoxic attacks, perhaps due to mucosal edema. Emergent surgical repositioning of the mediastinum and decompression of the bronchus was indicated. After complete adhesiolysis of the left thoracic cavity was performed, to maintain the proper mediastinal position, considering the emergent setting, an open wound thoracostomy was created and piles of gauze were inserted, mildly compressing the heart and the mediastinum to the right side. Thoracoplasty was performed three months later, and he was eventually discharged without any dressings needed. Mediastinal repositioning under thoracostomy should be avoided in elective cases because of its extremely high invasiveness. However, in the case of life-threatening postpneumonectomy syndrome in an emergent setting, mediastinal repositioning under thoracostomy may be an option to save life, which every thoracic surgeon could attempt. PMID:26106501

  3. Life-Threatening Postpneumonectomy Syndrome Complicated with Right Aortic Arch after Left Pneumonectomy

    PubMed Central

    Karasaki, Takahiro; Tanaka, Makoto

    2015-01-01

    A 54-year-old man with right aortic arch underwent left lower lobectomy and lingular segmentectomy, followed by complete pneumonectomy, for refractory nontuberculous mycobacterial infection. Three months after the pneumonectomy, he developed acute respiratory distress. Computed tomography showed an excessive mediastinal shift with an extremely narrowed bronchus intermedius and right lower bronchus compressed between the right pulmonary artery and the right descending aorta. Soon after the nearly obstructed bronchus intermedius was observed by bronchoscopy, he began to exhibit frequent hypoxic attacks, perhaps due to mucosal edema. Emergent surgical repositioning of the mediastinum and decompression of the bronchus was indicated. After complete adhesiolysis of the left thoracic cavity was performed, to maintain the proper mediastinal position, considering the emergent setting, an open wound thoracostomy was created and piles of gauze were inserted, mildly compressing the heart and the mediastinum to the right side. Thoracoplasty was performed three months later, and he was eventually discharged without any dressings needed. Mediastinal repositioning under thoracostomy should be avoided in elective cases because of its extremely high invasiveness. However, in the case of life-threatening postpneumonectomy syndrome in an emergent setting, mediastinal repositioning under thoracostomy may be an option to save life, which every thoracic surgeon could attempt. PMID:26106501

  4. The Ochoa urofacial syndrome: recognize the peculiar smile and avoid severe urological and renal complications.

    PubMed

    Rondon, Atila Victal; Leslie, Bruno; Netto, José Murillo Bastos; Freitas, Ricardo Garcia de; Ortiz, Valdemar; Macedo Junior, Antonio

    2015-01-01

    Ochoa syndrome is rare and its major clinical problems frequently unrecognized. We describe facial characteristics of six patients to help health professional recognize the inverted smile that these patients present and refer them to proper treatment. Patients' medical records were reviewed and patients' urological status clinically reassessed. At last evaluation patients' mean age was 15.5 years, and age ranged from 12 to 32 years. Mean follow-up was 35 months (12 to 60). Initial symptoms were urinary tract infections in four patients (67%) associated with enuresis and incontinence in three of them (50%). One patient had only urinary tract infection and two lower urinary tract symptoms without infections. Initial treatment consisted of clean intermittent catheterization with anticholinergics for all patients. Four patients (67%) were submitted to bladder augmentation. Two patients had end-stage renal disease during follow-up, one received kidney transplantation and one patient remained on the waiting list for a renal transplantation. Familial consanguinity was present in only one case. This significant condition is rare, but it must be recognized by pediatricians, nephrologists and urologists in order to institute early aggressive urological treatment. PMID:25946049

  5. Infectious Mimicry Complicates Diagnosis in Hemophagocytic Syndrome Caused by Anaplastic Large-Cell Lymphoma

    PubMed Central

    Peluso, Michael J.; Chia, David; Sheen, Whitney; Hutchinson, Christoph; Barakat, Lydia

    2012-01-01

    Hemophagocytic syndrome (HPS) arises secondary to genetic, rheumatologic, neoplastic, and infectious causes. We discuss a patient whose presentation was consistent with systemic infection but was discovered to have HPS of unknown etiology. The presenting symptoms, as well as unremarkable malignancy and rheumatologic workups, led to the pursuit of an infectious cause, but the patient was ultimately discovered to have an occult anaplastic large-cell lymphoma (ALCL). This case demonstrates the diagnostic challenges that result from infectious mimicry in the context of HPS—first, in distinguishing noninfectious HPS from the systemic inflammation that can result from a widespread infectious process, second, in the identification of the precipitating cause of HPS. While evidence of these challenges has been suggested by the limited literature on HPS and ALCL, our case illustrates the diagnostic dilemma that arises when tissue biopsy does not quickly reveal an etiology. It is important that all physicians be aware that HPS can mimic infection and be prepared to redirect the workup when an infectious etiology for HPS cannot be identified. PMID:22761627

  6. D-Lactic Acidosis: An Underrecognized Complication of Short Bowel Syndrome

    PubMed Central

    Kowlgi, N. Gurukripa; Chhabra, Lovely

    2015-01-01

    D-lactic acidosis or D-lactate encephalopathy is a rare condition that occurs primarily in individuals who have a history of short bowel syndrome. The unabsorbed carbohydrates act as a substrate for colonic bacteria to form D-lactic acid among other organic acids. The acidic pH generated as a result of D-lactate production further propagates production of D-lactic acid, hence giving rise to a vicious cycle. D-lactic acid accumulation in the blood can cause neurologic symptoms such as delirium, ataxia, and slurred speech. Diagnosis is made by a combination of clinical and laboratory data including special assays for D-lactate. Treatment includes correcting the acidosis and decreasing substrate for D-lactate such as carbohydrates in meals. In addition, antibiotics can be used to clear colonic flora. Although newer techniques for diagnosis and treatment are being developed, clinical diagnosis still holds paramount importance, as there can be many confounders in the diagnosis as will be discussed subsequently. PMID:25977687

  7. [Predictive indicators for progression to severe complications(hemolytic-uremic syndrome and encephalopathy) and their prevention in enterohemorrhagic Escherichia coli infection].

    PubMed

    Joh, K

    1997-03-01

    The treatment of infection with enterohemorrhagic Escherichia coli(EHEC) aims for early prediction and prevention of severe complications such as hemolytic-uremic syndrome, encephalopathy and/or thrombotic thrombocytopenic purpura. Factors related to the complications are divided into three categories; risk factors or predisposition, predictors, and indicators of severity and outcome. Risk factors for complications include two extreme ages, infection with verotoxin 2 producing E. coli, positive stool culture for EHEC, use of antimotility drug, use of trimethoprim-sulfamethoxazole. Predictors for complications include severe abdominal pain and bloody diarrhea development of high fever, change of consciousness, urinal protein and/or occult blood, abrupt increase of white blood cell count, urinal NAG, alpha 1 microglobulin, beta 2 microglobulin, low osmolar urine, high thrombomodulin level, marked thickening of intestinal wall, increased brightness of kidney in ultrasound sonography. No preventive treatment for these complications is proven except SYNSORB-pk which is expected to effectively aborb verotoxin in the intestine. PMID:9086784

  8. Bacterial sinusitis and its frightening complications: subdural empyema and Lemierre syndrome

    PubMed Central

    Salgado, German Alcoba; Ferreira, Cristiane Rúbia; Felipe-Silva, Aloísio; Gilio, Alfredo Elias

    2015-01-01

    The symptoms of a previously healthy 14-year-old female with an initial history of tooth pain and swelling of the left maxillary evolved to a progressive headache and altered neurological findings characterized by auditory hallucinations, sleep disturbances, and aggressiveness. She was brought to the emergency department after 21 days of the initial symptoms. An initial computed tomography (CT) scan showed frontal subdural empyema with bone erosion. The symptoms continued to evolve to brain herniation 24 hours after admission. A second CT scan showed a left internal jugular vein thrombosis. The outcome was unfavorable and the patient died on the second day after admission. The autopsy findings depicted rarefaction of the cranial bone at the left side of the frontal sinus, and overt meningitis. The severe infection was further complicated by thrombophlebitis of the left internal jugular vein up to the superior vena cava with septic embolization to the lungs, pneumonia, and sepsis. This case report highlights the degree of severity that a trivial infection can reach. The unusual presentation of the sinusitis may have wrongly guided the approach of this unfortunate case. PMID:26894042

  9. Bacterial sinusitis and its frightening complications: subdural empyema and Lemierre syndrome.

    PubMed

    Benevides, Gabriel Núncio; Salgado, German Alcoba; Ferreira, Cristiane Rúbia; Felipe-Silva, Aloísio; Gilio, Alfredo Elias

    2015-01-01

    The symptoms of a previously healthy 14-year-old female with an initial history of tooth pain and swelling of the left maxillary evolved to a progressive headache and altered neurological findings characterized by auditory hallucinations, sleep disturbances, and aggressiveness. She was brought to the emergency department after 21 days of the initial symptoms. An initial computed tomography (CT) scan showed frontal subdural empyema with bone erosion. The symptoms continued to evolve to brain herniation 24 hours after admission. A second CT scan showed a left internal jugular vein thrombosis. The outcome was unfavorable and the patient died on the second day after admission. The autopsy findings depicted rarefaction of the cranial bone at the left side of the frontal sinus, and overt meningitis. The severe infection was further complicated by thrombophlebitis of the left internal jugular vein up to the superior vena cava with septic embolization to the lungs, pneumonia, and sepsis. This case report highlights the degree of severity that a trivial infection can reach. The unusual presentation of the sinusitis may have wrongly guided the approach of this unfortunate case. PMID:26894042

  10. [Perioperative management of an obese patient complicated with sleep apnea syndrome (SAS) undergoing awake craniotomy].

    PubMed

    Komayama, Noriaki; Kamata, Kotoe; Maruyama, Takashi; Nitta, Masayuki; Muragaki, Yoshihiro; Ozaki, Makoto

    2014-10-01

    Both obesity (BMI over 30) and SAS are risks for Supper airway maintenance. We report an obese patient (BMI 33.5) with SAS who underwent awake craniotomy. Weight reduction was instructed 1 month before the operation, and the patient lost enough weight to use intraoperative MRI. Under general anesthesia, surgical pads containing 2% lidocaine with adrenaline were inserted into the nasal cavities. The patient's airway S was secured by i-gel® until dura was opened. A nasal airway was then inserted to confirm the upper airway patency and anesthetics were terminated The patient regained consciousness and started respiration. The i-gel® was removed. The nasal airway was changed to an RAE tracheal tube ; the tube was fixed above the vocal cords under bronchofiberscopic observation. Continuous positive airway pressure (CPAP) via RAE tube was started. Neither coughing nor epistaxis was observed.The RAE tube prevented glossoptosis and did not disturb speech mapping. Emergent endotracheal intubation was easily managed because the tube was close to the glottis. The RAE tube was removed and nasal CP AP was applied overnight Carefully prepared CP AP support via nasal RAE tube was practical in keeping upper airway patency for an obese patient complicated with SAS undergoing awake craniotomy. PMID:25693340