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Sample records for suspected idiopathic sclerosing

  1. Idiopathic sclerosing encapsulating peritonitis: an uncommon cause of intestinal obstruction

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    José de Arimatéia Batista Araújo Filho

    2012-12-01

    Full Text Available Sclerosing encapsulating peritonitis (SEP, also called encapsulating peritonitis, is a rare and benign cause of intestinal obstruction of unknown etiology. Its onset may be acute or subacute although there are some reports with a two-month history. More commonly, this entity is secondary to chronic peritoneal dialysis, ventriculoperitoneal and peritoneovenous shunting, the use of β-blockers and systemic lupus erythematous. Recurrent episodes of bacterial peritonitis, intestinal tuberculosis, sarcoidosis, familial Mediterranean fever, gastrointestinal cancer, liver transplantation, intra-abdominal fibrogenic foreign bodies, and luteinized ovarian thecomas are also related to SEP. The idiopathic presentation is more rare. Abdominal pain, nausea, vomiting, weight loss, malnutrition, and clinically palpable mass characterize the clinical features. Diagnosis is frequently made with gross findings during surgery, imaging workup and histopathology. The authors report the case of a 36-yearold male patient with a 10-day history of abdominal pain that was operated on because of intestinal obstruction. Diagnosis was made preoperatively and confirmed by the intraoperative findings and histopathology.

  2. IDIOPATHIC SCLEROSING ENCAPSULATING PERITONITIS CAUSING ACUTE INTESTINAL OBSTRUCTION AND GANGRENE: A CASE REPORT

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    Nava

    2016-04-01

    Full Text Available INTRODUCTION Sclerosing encapsulating peritonitis (SEP is a relatively rare cause of intestinal obstruction resulting from encasement of variable lengths of bowel by dense fibro-collagenous membrane. It is more common in young females, and shows tropical and sub-tropical distribution. The idiopathic cases of SEP, which lack any identifiable cause from clinical, radiological and histopathological findings, are also reported under the descriptive term “abdominal cocoon syndrome”. SEP presents with acute or sub-acute intestinal obstruction with or without a mass. In the era of laparoscopic surgery, inadvertent damage to the small bowel at insertion of the trocar and cannula can occur by being unaware of this condition resulting in unnecessary bowel resection. Persistent untreated SEP may advance to bowel gangrene or intestinal perforation, representing life threatening conditions. We report the clinical presentation of a 75-year-old female presenting with signs of intestinal obstruction whose imaging findings revealed abdominal cocoon with bowel gangrene leading to perforation and the same confirmed at surgery. Surgical excision of the fibrotic sac encasing the bowel, resection of gangrenous bowel segment and end ileostomy was performed. Histopathology of the excised membrane confirmed sclerosing encapsulating peritonitis. To our knowledge, only a few cases of abdominal cocoon with perforation have been reported in literature so far. Radiologists should be aware of this relatively rare cause of intestinal obstruction, its imaging findings and complications, as preoperative diagnosis will prevent delay and aid in treatment planning to the surgeon. Identification of soft tissue density membrane encasing congregated small bowel loops into a single area on computed-tomography gives diagnostic clue. Surgical excision of sac, release of bowel loops and adhesions with partial intestinal resection when necessary is the treatment.

  3. Idiopathic sclerosing encapsulating peritonitis (abdominal cocoon in adult male. A case report

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    Amer Hashim Al Ani

    2014-01-01

    CONCLUSION: Primary sclerosing encapsulating peritonitis (cocoon abdomen diagnosis needs a high index of suspicion, as signs and symptoms are nonspecific and imaging findings are not always conclusive. Careful excision of the accessory peritoneal sac and lysis of adhesions between bowels is the best treatment. Prognosis is generally good.

  4. The roles of magnetic resonance and endoscopic retrograde cholangiopancreatography (MRCP and ERCP) in the diagnosis of patients with suspected sclerosing cholangitis: a cost-effectiveness analysis.

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    Meagher, S; Yusoff, I; Kennedy, W; Martel, M; Adam, V; Barkun, A

    2007-03-01

    The optimal approach for diagnosing sclerosing cholangitis remains unclear in the face of competing imaging technologies. We aimed to determine the most cost-effective strategy. A decision model compared three approaches in the work-up of patients with suspected sclerosing cholangitis; all included an initial test, with, if unsuccessful, performance of a second cholangiographic method. They were magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP), termed "MRCP_ERCP", ERCP and MRCP ("ERCP_MRCP"), or ERCP and a repeat ERCP ("ERCP_ERCP"). The implications of true and false positive and negative results with regard to costs and procedural complications were considered, including that of a liver biopsy, if indicated as a result of a negative work-up in the face of persistent clinical suspicion. The unit of effectiveness adopted was that of a correct diagnosis. Probability assumptions were derived from published literature, while cost estimates were derived from time-motion microanalyses or a national database, and expressed in Canadian dollars at 2004 values. Sensitivity analyses, including clinically relevant threshold analyses, were carried out. The average cost-effectiveness ratios were $414 for MRCP_ERCP, $1101 for ERCP_MRCP and $1123 for ERCP_ERCP, per correct diagnosis. The ERCP_MRCP strategy was dominated (more expensive and less effective) by MRCP_ERCP, while ERCP_ERCP was more effective and more costly than MRCP_ERCP, at $289,292 per additional correct diagnosis. Sensitivity and threshold analyses confirmed the robustness of these findings. Based on the model assumptions, a strategy of initial MRCP, followed, if negative, by ERCP is currently the most cost-effective approach to the work-up of patients with suspected sclerosing cholangitis.

  5. Radiotherapy With or Without Surgery for Patients With Idiopathic Sclerosing Orbital Inflammation Refractory or Intolerant to Steroid Therapy

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    Lee, Jong Hoon [Department of Radiation Oncology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Kim, Yeon-Sil, E-mail: yeonkim7@catholic.ac.kr [Department of Radiation Oncology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Yang, Suk Woo; Cho, Won-Kyung [Department of Ophthalmology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Lee, Sang Nam [Department of Radiation Oncology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Lee, Kyung Ji [Department of Hospital Pathology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of); Ryu, Mi-Ryeong; Jang, Hong Seok [Department of Radiation Oncology, Seoul St. Mary' s Hospital, College of Medicine, Catholic University of Korea, Seoul (Korea, Republic of)

    2012-09-01

    Purpose: To evaluate the outcomes of patients with idiopathic sclerosing orbital inflammation (ISOI) treated with radiotherapy with or without surgery. Methods and Materials: We retrospectively reviewed 22 patients with histopathologically confirmed ISOI who had been refractory or intolerant to steroid therapy and treated with radiation with or without surgery. The radiation dose ranged from 20 to 40 Gy (median, 20 Gy) at 2 Gy per fraction. Presenting signs and treatment outcomes were assessed. Results: Proptosis was the most common sign at presentation, seen in 19 (86.3%) patients, followed by restriction of extraocular movements in 10 (45.4%) patients. Response to radiotherapy was complete in 15 (68.1%) patients, partial in 3 (13.6%) patients, and none in 4 (18.2%) patients. At the median follow-up of 34 months, 14 (63.6%) patients had progression-free state of symptoms and signs, with the progression-free duration ranging from 3 to 75 months (median, 41.5 months), whereas 8 (36.4%) patients had recurrent or persistent disease although they had received radiotherapy. Of the 14 progression-free patients, 6 underwent a bimodality treatment of debulking surgery of ocular disease and radiotherapy. They had had no recurrent disease. Cataract was the most common late complications, and 2 patients experienced a Grade 3 cataract. Conclusion: Our study suggests that for patients with ISOI who are refractory or intolerant to steroid therapy, 20 Gy of radiotherapy appears to be effective for the control of disease with acceptable complications, especially when it is combined with surgery.

  6. Bedside Optic Nerve Sheath Diameter Assessment in the Identification of Increased Intracranial Pressure in Suspected Idiopathic Intracranial Hypertension.

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    Irazuzta, Jose E; Brown, Martha E; Akhtar, Javed

    2016-01-01

    We determined whether the bedside assessment of the optic nerve sheath diameter could identify elevated intracranial pressure in individuals with suspected idiopathic intracranial hypertension. This was a single-center, prospective, rater-blinded study performed in a freestanding pediatric teaching hospital. Patients aged 12 to 18 years scheduled for an elective lumbar puncture with the suspicion of idiopathic intracranial hypertension were eligible to participate. Optic nerve sheath diameter was measured via ultrasonography before performing a sedated lumbar puncture for measuring cerebrospinal fluid opening pressure. Abnormal measurements were predefined as optic nerve sheath diameter ≥4.5 mm and a cerebrospinal fluid opening pressure greater than 20 cmH2O. Thirteen patients participated in the study, 10 of whom had elevated intracranial pressure. Optic nerve sheath diameter was able to predict or rule out elevated intracranial pressure in all patients. Noninvasive assessment of the optic nerve sheath diameter could help to identify patients with elevated intracranial pressure when idiopathic intracranial hypertension is suspected. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. International Veterinary Epilepsy Task Force's current understanding of idiopathic epilepsy of genetic or suspected genetic origin in purebred dogs.

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    Hülsmeyer, Velia-Isabel; Fischer, Andrea; Mandigers, Paul J J; DeRisio, Luisa; Berendt, Mette; Rusbridge, Clare; Bhatti, Sofie F M; Pakozdy, Akos; Patterson, Edward E; Platt, Simon; Packer, Rowena M A; Volk, Holger A

    2015-08-28

    Canine idiopathic epilepsy is a common neurological disease affecting both purebred and crossbred dogs. Various breed-specific cohort, epidemiological and genetic studies have been conducted to date, which all improved our knowledge and general understanding of canine idiopathic epilepsy, and in particular our knowledge of those breeds studied. However, these studies also frequently revealed differences between the investigated breeds with respect to clinical features, inheritance and prevalence rates. Awareness and observation of breed-specific differences is important for successful management of the dog with epilepsy in everyday clinical practice and furthermore may promote canine epilepsy research. The following manuscript reviews the evidence available for breeds which have been identified as being predisposed to idiopathic epilepsy with a proven or suspected genetic background, and highlights different breed specific clinical features (e.g. age at onset, sex, seizure type), treatment response, prevalence rates and proposed inheritance reported in the literature. In addition, certain breed-specific diseases that may act as potential differentials for idiopathic epilepsy are highlighted.

  8. International Veterinary Epilepsy Task Force's current understanding of idiopathic epilepsy of genetic or suspected genetic origin in purebred dogs

    NARCIS (Netherlands)

    Hülsmeyer, Velia-Isabel; Fischer, Andrea; Mandigers, Paul J J; DeRisio, Luisa; Berendt, Mette; Rusbridge, Clare; Bhatti, Sofie F M; Pakozdy, Akos; Patterson, Edward E; Platt, Simon; Packer, Rowena M A; Volk, Holger A

    2015-01-01

    Canine idiopathic epilepsy is a common neurological disease affecting both purebred and crossbred dogs. Various breed-specific cohort, epidemiological and genetic studies have been conducted to date, which all improved our knowledge and general understanding of canine idiopathic epilepsy, and in

  9. International Veterinary Epilepsy Task Force's current understanding of idiopathic epilepsy of genetic or suspected genetic origin in purebred dogs

    OpenAIRE

    Hülsmeyer, V I; A. Fischer; Mandigers, P.J.J.; De Risio, L.; Berendt, M.; Rusbridge, C; Bhatti, S F M; Pakozdy, A; Patterson, E E; Platt, S.; Packer, R M A; Volk, H. A.

    2015-01-01

    Canine idiopathic epilepsy is a common neurological disease affecting both purebred and crossbred dogs. Various breed-specific cohort, epidemiological and genetic studies have been conducted to date, which all improved our knowledge and general understanding of canine idiopathic epilepsy, and in particular our knowledge of those breeds studied. However, these studies also frequently revealed differences between the investigated breeds with respect to clinical features, inheritance and prevale...

  10. Pregabalin as an adjunct to phenobarbital, potassium bromide, or a combination of phenobarbital and potassium bromide for treatment of dogs with suspected idiopathic epilepsy.

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    Dewey, Curtis W; Cerda-Gonzalez, Sofia; Levine, Jonathan M; Badgley, Britton L; Ducoté, Julie M; Silver, Gena M; Cooper, Jocelyn J; Packer, Rebecca A; Lavely, James A

    2009-12-15

    To assess tolerability and short-term efficacy of oral administration of pregabalin as an adjunct to phenobarbital, potassium bromide, or a combination of phenobarbital and potassium bromide for treatment of dogs with poorly controlled suspected idiopathic epilepsy. Open-label, noncomparative clinical trial. 11 client-owned dogs suspected of having idiopathic epilepsy that was inadequately controlled with phenobarbital, potassium bromide, or a combination of these 2 drugs. Dogs were treated with pregabalin (3 to 4 mg/kg [1.4 to 1.8 mg/lb], PO, q 8 h) for 3 months. Number of generalized seizures in the 3 months before and after initiation of pregabalin treatment was recorded. Number of responders (>or= 50% reduction in seizure frequency) was recorded, and seizure frequency before and after initiation of pregabalin treatment was compared by use of a nonparametric Wilcoxon signed rank test. Seizures were significantly reduced (mean, 57%; median, 50%) after pregabalin administration in the 9 dogs that completed the study; 7 were considered responders with mean and median seizure reductions of 64% and 58%, respectively. Adverse effects for pregabalin were reported in 10 dogs. Mean and median plasma pregabalin concentrations for all dogs were 6.4 and 7.3 microg/mL, respectively. Pregabalin may hold promise as a safe and effective adjunct anticonvulsant drug for epileptic dogs poorly controlled with the standard drugs phenobarbital or potassium bromide. Adverse effects of pregabalin appeared to be mild. Additional studies with larger numbers of dogs and longer follow-up intervals are warranted.

  11. Sclerosing Encapsulating Peritonitis; Review

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    Norman O. Machado

    2016-05-01

    Full Text Available Sclerosing encapsulating peritonitis (SEP is a rare chronic inflammatory condition of the peritoneum with an unknown aetiology. Also known as abdominal cocoon, the condition occurs when loops of the bowel are encased within the peritoneal cavity by a membrane, leading to intestinal obstruction. Due to its rarity and nonspecific clinical features, it is often misdiagnosed. The condition presents with recurrent episodes of small bowel obstruction and can be idiopathic or secondary; the latter is associated with predisposing factors such as peritoneal dialysis or abdominal tuberculosis. In the early stages, patients can be managed conservatively; however, surgical intervention is necessary for those with advanced stage intestinal obstruction. A literature review revealed 118 cases of SEP; the mean age of these patients was 39 years and 68.0% were male. The predominant presentation was abdominal pain (72.0%, distension (44.9% or a mass (30.5%. Almost all of the patients underwent surgical excision (99.2% without postoperative complications (88.1%.

  12. Sclerosing Stromal Tumor of Ovary: A Case Report

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    Menka Khanna

    2012-01-01

    Full Text Available Sclerosing stromal tumor (SST is an extremely rare and distinctive sex cord stromal tumor which occurs predominantly in the second and third decades of life. We report a case of a 32-year-old woman who developed a sclerosing stromal tumor of ovary and presented with irregular menstruation and pelvic pain. Her hormonal status was normal but CA-125 was raised. She was suspected to have a malignant tumor on computed tomography and underwent bilateral salpingo-oopherectomy. It is therefore necessary to keep in mind the possibility of sclerosing stromal tumor in a young woman.

  13. Suspected accelerated disease progression after discontinuation of nintedanib in patients with idiopathic pulmonary fibrosis: Two case reports.

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    Okamori, Satoshi; Asakura, Takanori; Masuzawa, Keita; Yasuda, Hiroyuki; Kamata, Hirofumi; Ishii, Makoto; Betsuyaku, Tomoko

    2017-12-01

    The efficacy of nintedanib, a multitarget receptor tyrosine kinase inhibitor, has been demonstrated in recent randomized controlled trials involving patients with idiopathic pulmonary fibrosis (IPF). However, accelerated disease progression after nintedanib discontinuation has never been reported. We report 2 cases involving patients with a history of IPF who presented with respiratory deterioration at 3 weeks after the discontinuation of nintedanib therapy for IPF. Neither patient fulfilled the definition of "acute exacerbation of IPF" on unilateral computed tomography. Accelerated disease progression after the discontinuation of nintedanib therapy for IPF. One patient received steroid therapy. The other patient refused to undergo steroid therapy. The first patient showed that the affected lobe exhibited volume loss with traction bronchiectasis after receiving steroid therapy, and succumbed to pneumothorax after 3 months. The other patient was transferred to another hospital because of a decline in his general condition. To our knowledge, this report is the first to document accelerated disease progression after the discontinuation of nintedanib therapy for IPF. Although the accurate mechanism remains unclear, the effects of nintedanib against vascular endothelial growth factor and platelet-derived growth factor receptor may play a role. Our findings suggest that physicians should carefully monitor patients with IPF after nintedanib discontinuation.

  14. Primary Sclerosing Cholangitis: Multiple Phenotypes, Multiple Approaches.

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    Sarkar, Souvik; Bowlus, Christopher L

    2016-02-01

    Primary sclerosing cholangitis (PSC) is a heterogeneous, idiopathic, inflammatory disorder frequently associated with inflammatory bowel diseases. PSC patients may be classified into several subphenotypes. Investigations of pediatric, nonwhite, and female PSC patients have revealed distinguishing features. The natural history of PSC is variable in progression with numerous possible clinical outcomes. PSC patients may suffer bacterial cholangitis, cholangiocarcinoma, or colorectal adenocarcinoma. Treatments focusing on bile acid therapy and immunosuppression have not proven beneficial. Interest in PSC and international collaboration has led to improved understanding of the heterogeneity and the genetic structure and introduced possible effective therapeutics. Copyright © 2016 Elsevier Inc. All rights reserved.

  15. Lymphoplasmacytic Sclerosing Pancreatitis and Retroperitoneal Fibrosis

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    Nigel K. F. Koo Ng

    2008-01-01

    Full Text Available Although cases of lymphoplasmacytic sclerosing pancreatitis (LSP associated with idiopathic retroperitoneal fibrosis have been reported, the association is rare. We describe a 74-year-old man who presented with obstructive jaundice and weight loss. Nineteen months earlier, he had been diagnosed with idiopathic retroperitoneal fibrosis and treated with bilateral ureteric stents. Initial investigations were suggestive of a diagnosis of LSP, however, a malignant cause could not be ruled out. He underwent an exploratory laparotomy and frozen sections confirmed the diagnosis of LSP. An internal biliary bypass was performed using a Roux loop of jejunum, and the patient made an uneventful recovery. This case illustrates the difficulty in distinguishing LSP from pancreatic carcinoma preoperatively.

  16. Sclerosing thymoma-like thymic amyloidoma with nephrotic syndrome: a case report.

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    Kato, Yuto; Okuda, Miyuki; Fukuda, Koji; Tanaka, Nobuya; Yoshizawa, Akihiko; Saika, Yoshinori; Haruna, Yoshisumi; Kitaguchi, Shouji; Nohara, Ryuji

    2017-09-07

    Primary localized amyloidosis presenting as an isolated mediastinal mass is extremely rare, especially in the thymus. Sclerosing thymoma is also an extremely rare anterior mediastinal tumor, pathologically characterized by extensive sclerotic lesions with hyalinization and calcification. Only 14 cases of sclerosing thymoma and five cases of thymic amyloidosis have been reported to date. A 78-year-old Japanese woman was diagnosed as having sclerosing thymoma (Masaoka stage IVa pericardial dissemination)-like thymic amyloidoma. She was diagnosed as having either lung cancer or mediastinal tumor with pericardial dissemination, and received palliative treatment. Three years later, she was readmitted with a complaint of general malaise. Since minimal change nephrotic syndrome was suspected based on the disease onset and selectivity index of urinary protein, steroid pulse therapy was started. Subsequently, because a marked reduction in tumor size was observed during maintenance treatment with prednisolone, a thoracoscopic needle biopsy was performed for a definitive diagnosis. According to the pathological findings and clinical investigations, a final diagnosis of sclerosing thymoma (Masaoka stage IVa pericardial dissemination)-like thymic amyloidoma was made. This is a case report of sclerosing thymoma-like thymic amyloidoma. Both sclerosing thymoma and thymic amyloidoma are extremely rare diseases: only 14 cases of sclerosing thymoma and five cases of thymic amyloidosis have been reported to date. In either diagnosis, our case is the first case in which marked reduction in tumor size was observed with steroid therapy. All reported cases of sclerosing thymomas underwent surgical resection, but steroid therapy to sclerosing thymoma has not been reported. It is still unknown whether steroid therapy is effective or not. The hyalinized components of sclerosing thymoma possibly contain amyloid deposits. The marked reduction in tumor size with steroid therapy may result in

  17. [The colecistectomy in primary sclerosing cholangitis: case report and review of literature].

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    Izzo, L; Costi, U; Adduci, M; Gazzanelli, S; Casullo, A; Kharrub, Z; Sassayanis, Ph G; Marini, M; Stasolla, A; Pulcinelli, V; Meucci, M; Caputo, M

    2005-01-01

    Primary sclerosing cholangitis (PSC) is an idiopathic chronic inflammatory condition affecting intra- and extra-hepatic biliary system. The aim of this study is to stress the importance of cholecystectomy in patients with PSC. Consideration should be given to performing a cholecystectomy in PSC patients with gallbladder adenomyomas or calculi, even asymptomatic; this happens in approximately 15% of cases.

  18. Emerging treatments for primary sclerosing cholangitis.

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    Rodriguez, Eduardo A; Carey, Elizabeth J; Lindor, Keith D

    2017-05-01

    Primary sclerosing cholangitis (PSC) is a chronic, cholestatic, idiopathic liver disease that can progress to end-stage liver disease, cirrhosis and cholangiocarcinoma. PSC is an uncommon and highly heterogeneous disease, associated with inflammatory bowel disease and a complex pathophysiology. To date, no medical therapies have proved effective. The only available treatment for end-stage PSC is liver transplant, but recurrence is a significant complication. Areas covered: This review will explore previously tested treatments, discuss current treatment strategies and present viewpoints about future emerging therapies in PSC. We searched PubMed using the noted keywords. We included data from full-text articles published in English. Further relevant articles were identified from the reference lists of review articles. Expert commentary: The development of new therapies in PSC has been challenging. However, with greater awareness of the disease nowadays, new insights into the disease may help in the design of future therapeutic agents in PSC and ultimately in effective therapies.

  19. Glucocorticosteroids for primary sclerosing cholangitis

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    Giljaca, Vanja; Poropat, Goran; Stimac, Davor

    2010-01-01

    Primary sclerosing cholangitis is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterised by chronic periductal inflammation and sclerosis of the ducts, which results in segmental stenoses of bile ducts, cholestasis, fibrosis, and ultimately, liver cirrhosis....... Patients with primary sclerosing cholangitis are at higher risk of cholangiocarcinoma as well as of colonic neoplasia, since primary sclerosing cholangitis is associated with inflammatory bowel disease in more than 80% of the patients. Several therapeutic modalities have been proposed for primary...... sclerosing cholangitis, like ursodeoxycholic acid, glucocorticosteroids, and immunomodulatory agents, but none has been successful in reversing the process of the disease. To date, liver transplantation is the only definite therapeutic solution for patients with advanced primary sclerosing cholangitis...

  20. Primary Sclerosing Cholangitis: Current and Future Management Strategies

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    Eaton, John E.; Talwalkar, Jayant A.

    2013-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease. The etiology of this disorder is unknown and there are no effective medical therapies. PSC is associated with inflammatory bowel disease and an increased risk for hepatobiliary and colorectal malignancies. The aim of this review is to highlight the clinical features and diagnostic approach to patients with suspected PSC, characterize associated comorbidities, review screening strategies for PSC associated malignancies and review contemporary and future therapies. PMID:23682242

  1. Subacute sclerosing panencephalitis presenting as schizophrenia with an alpha coma pattern in a child.

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    Kartal, Ayşe; Kurt, Ayşegül Neşe Çitak; Gürkaş, Esra; Aydin, Kurşad; Serdaroğlu, Ayşe

    2014-10-01

    Subacute sclerosing panencephalitis, a progressive neurodegenerative disorder of the central nervous system, can present atypically with uncharacteristic electroencephalographic (EEG) features at its onset albeit typically with progressive mental deterioration, behavioral changes, and myoclonic jerks. An atypical presentation of subacute sclerosing panencephalitis can lead to a delay in diagnosis, thus hindering early treatment. Herein, we describe a 14-year-old girl who presented with insomnia, amnesia, auditory and visual hallucinations. The patient's electroencephalography on admission showed an alpha coma pattern. In spite of antipsychiatric treatment (olanzapine 20 mg/d) for 3 months, a progressive deterioration in neurologic function was observed. Subacute sclerosing panencephalitis was suspected and diagnosis was confirmed by increased titers of measles antibodies in the cerebrospinal fluid. The attention of pediatricians should be drawn to psychiatric symptoms as possible initial presentations of subacute sclerosing panencephalitis in order to avoid needless diagnostic and treatment procedures. © The Author(s) 2013.

  2. Primary sclerosing cholangitis

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    Chapman Roger

    2006-10-01

    Full Text Available Abstract Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9–1.31/100,000 and point prevalence of 8.5–13.6/100,000. The onset of PSC is usually insidious and many patients are asymptomatic at diagnosis or have mild symptoms only such as fatigue, abdominal discomfort and pruritus In late stages, splenomegaly and jaundice may be a feature. In most, the disease progresses to cirrhosis and liver failure. Cholangiocarcinoma develops in 8–30% of patients. PSC is thought to be immune mediated and is often associated with inflammatory bowel disease, especially ulcerative colitis. The disease is diagnosed on typical cholangiographic and histological findings and after exclusion of secondary sclerosing cholangitis. Median survival has been estimated to be 12 years from diagnosis in symptomatic patients. Patients who are asymptomatic at diagnosis, the majority of whom will develop progressive disease, have a survival rate greater than 70% at 16 years after diagnosis. Liver transplantation remains the only effective therapeutic option for patients with end-stage liver disease from PSC, although high dose ursodeoxycholic acid may have a beneficial effect.

  3. Role of the Microbiota and Antibiotics in Primary Sclerosing Cholangitis

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    James H. Tabibian

    2013-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is an idiopathic, progressive, cholestatic liver disease with considerable morbidity and mortality and no established pharmacotherapy. In addition to the long-recognized association between PSC and inflammatory bowel disease, several lines of preclinical and clinical evidence implicate the microbiota in the etiopathogenesis of PSC. Here we provide a concise review of these data which, taken together, support further investigation of the role of the microbiota and antibiotics in PSC as potential avenues toward elucidating safe and effective pharmacotherapy for patients afflicted by this illness.

  4. Role of the Microbiota and Antibiotics in Primary Sclerosing Cholangitis

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    Tabibian, James H.; Talwalkar, Jayant A.; Lindor, Keith D.

    2013-01-01

    Primary sclerosing cholangitis (PSC) is an idiopathic, progressive, cholestatic liver disease with considerable morbidity and mortality and no established pharmacotherapy. In addition to the long-recognized association between PSC and inflammatory bowel disease, several lines of preclinical and clinical evidence implicate the microbiota in the etiopathogenesis of PSC. Here we provide a concise review of these data which, taken together, support further investigation of the role of the microbiota and antibiotics in PSC as potential avenues toward elucidating safe and effective pharmacotherapy for patients afflicted by this illness. PMID:24232746

  5. Secondary Sclerosing Cholangitis During Pregnancy

    National Research Council Canada - National Science Library

    Abdulqader, Yasir; Chuang, Keng-Yu; Ravi, Jyotsna; Nadir, Abdul

    2016-01-01

    ... of secondary sclerosing cholangitis. Maternal and fetal outcomes of primary sclerosis cholangitis have been reported, and this case highlights the importance of considering other diseases besides the benign intrahepatic cholestasis...

  6. Surge of subacute sclerosing panencephalitis

    National Research Council Canada - National Science Library

    Lisel V Richter-Joubert; Donovan Jacobs; Tracy Kilborn

    2017-01-01

    Subacute sclerosing panencephalitis (SSPE) is a poorly understood complication of measles infection, presenting in up to 1 in 2500 infected children, resulting in devastating neurocognitive outcomes and a high mortality rate...

  7. Primary sclerosing cholangitis and pregnancy

    Directory of Open Access Journals (Sweden)

    Casper Q. Kammeijer

    2011-08-01

    Full Text Available Primary sclerosing cholangitis is a progressive disease, and coincidentally in pregnancy it is rare. It is characterized by progressive inflammation and destruction of bile ducts finally resulting in liver failure. A rare case of primary sclerosing cholangitis in pregnancy is presented. The course of the pregnancy was marked by threatened preterm delivery and exacerbation of cholestasis. She was successfully treated with ursodeoxycholic acid (UDCA. Although, primary sclerosing cholangitis has both maternal and fetal effects on pregnancy, the overall outcome is favorable. Only few cases have been reported using high dose ursodeoxycholic acid for primary sclerosing cholangitis in pregnancy, it often improves pruritus but has no protection against stillbirth. Data on the safety to the fetus or neonate and long-term outcome are scarce.

  8. Secondary Sclerosing Cholangitis During Pregnancy

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    Abdulqader, Yasir; Chuang, Keng-Yu; Ravi, Jyotsna; Nadir, Abdul

    2016-01-01

    We report a case of secondary sclerosing cholangitis that manifested itself during pregnancy. A tentative diagnosis of intrahepatic cholestasis of pregnancy was considered, but after her third delivery, a liver biopsy and imaging, as well as review of past records, confirmed the diagnosis of secondary sclerosing cholangitis. Maternal and fetal outcomes of primary sclerosis cholangitis have been reported, and this case highlights the importance of considering other diseases besides the benign ...

  9. Secondary Sclerosing Cholangitis During Pregnancy.

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    Abdulqader, Yasir; Chuang, Keng-Yu; Ravi, Jyotsna; Nadir, Abdul

    2016-07-01

    We report a case of secondary sclerosing cholangitis that manifested itself during pregnancy. A tentative diagnosis of intrahepatic cholestasis of pregnancy was considered, but after her third delivery, a liver biopsy and imaging, as well as review of past records, confirmed the diagnosis of secondary sclerosing cholangitis. Maternal and fetal outcomes of primary sclerosis cholangitis have been reported, and this case highlights the importance of considering other diseases besides the benign intrahepatic cholestasis of pregnancy as a cause of cholestasis in pregnancy.

  10. Subacute sclerosing panencephalitis and chronic viral encephalitis.

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    Anlar, Banu

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a chronic infection of the central nervous system associated with the presence of mutant measles virus in the brain. It presents as a progressive, usually fatal disease. The diagnosis is based on clinical criteria and an elevated titer of measles antibodies in the cerebrospinal fluid (CSF). Electroencephalography and imaging studies provide supportive laboratory data. A brain biopsy is indicated only when CSF serology is negative or equivocal in a suspected case to assess the presence of inclusion bodies, measles virus antigens, or viral RNA. Among many drugs and methods tried in the treatment, the highest rate of stabilization or improvement was obtained with intraventricular human lymphoblastoid interferon-α and oral inosiplex. Further research for more available and efficient therapeutic regimens is warranted. Measles and SSPE are preventable by maintenance of high rates of immunization in the population. Copyright © 2013 Elsevier B.V. All rights reserved.

  11. Sclerosing segmental neurofibromatosis.

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    Lee, Joong Sun; Kim, You Chan

    2005-04-01

    Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait macules and/or neurofibromas limited to a single body segment. The neurofibromas in segmental neurofibromatosis are usually soft, non-tender nodules as in other types of neurofibromatosis. Histopathologically, they are usually non-encapsulated, loosely textured dermal tumors. We report a case of sclerosing segmental neurofibromatosis, in which the patient presented with several grouped, erythematous to brownish, firm tender nodules on the left side of the posterior neck. Histopathologically, the stroma was mostly very fibrotic, especially around hair follicles, in addition to the usual features of neurofibroma. The atypical clinical feature, hardness, and tenderness of the lesions may be associated with the fibrosis.

  12. Sclerosing epithelioid fibrosarcoma.

    Science.gov (United States)

    Patterson, James W; Tchernev, Georgi; Chokoeva, Anastasiya Atanasova; Wick, Mark R

    2017-04-01

    We report the case of a 55-year-old woman who developed a mass in the soft tissue of the anterior right proximal thigh. Microscopic findings in the biopsy specimen supported the diagnosis of sclerosing epithelioid fibrosarcoma (SEF). This entity is part of a spectrum of lesions that includes low-grade fibromyxoid sarcoma (LGFMS) and hyalinizing spindle cell tumor with giant rosettes (HSCTGR). It shares with LGFMS occasional overlapping histopathologic features and immunopositivity for MUC4. Although FUS and CREB3L2 gene rearrangements, characteristics of LGFMS, have been found in hybrid tumors (those with features of both SEF and LGFMS) and in examples of LGFMS with relapses showing SEF morphology, it appears that EWSR1 and CREB3L1 rearrangements predominate over FUS and CREB3L2 rearrangements among "pure" SEF tumors. Recent information about SEF is briefly reviewed.

  13. Inflammatory bowel disease of primary sclerosing cholangitis: a distinct entity?

    Science.gov (United States)

    Nakazawa, Takahiro; Naitoh, Itaru; Hayashi, Kazuki; Sano, Hitoshi; Miyabe, Katsuyuki; Shimizu, Shuya; Joh, Takashi

    2014-03-28

    This is a review of the characteristic findings of inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC) and their usefulness in the diagnosis of sclerosing cholangitis. PSC is a chronic inflammatory disease characterized by idiopathic fibrous obstruction and is frequently associated with IBD. IBD-associated with PSC (PSC-IBD) shows an increased incidence of pancolitis, mild symptoms, and colorectal malignancy. Although an increased incidence of pancolitis is a characteristic finding, some cases are endoscopically diagnosed as right-sided ulcerative colitis. Pathological studies have revealed that inflammation occurs more frequently in the right colon than the left colon. The frequency of rectal sparing and backwash ileitis should be investigated in a future study based on the same definition. The cholangiographic findings of immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are similar to those of PSC. The rare association between IBD and IgG4-SC and the unique characteristics of PSC-IBD are useful findings for distinguishing PSC from IgG4-SC.

  14. Bile acids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Chen, Weikeng; Gluud, C

    2003-01-01

    Bile acids have been used for treating primary sclerosing cholangitis, but their beneficial and harmful effects remain unclear.......Bile acids have been used for treating primary sclerosing cholangitis, but their beneficial and harmful effects remain unclear....

  15. Measles-vaccinated Israeli boy with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Har-Even, Ronly; Aichenbaum, Sergio; Rabey, Jose M; Livne, Amir; Bistritzer, Tzvy

    2011-06-01

    Subacute sclerosing panencephalitis is a rare neurologic disorder of childhood and adolescence. We describe a 16-year-old boy who manifested the disease despite proper vaccinations. He was hospitalized because of bedwetting, involuntary limb movements, abnormal speech, and balance disturbances. Immunoglobulin G antibodies against measles were strongly positive, with a high relative cerebrospinal fluid/serum ratio. Polymerase chain reaction for measles produced negative results. Electroencephalography registered slow activity with high voltage discharges every few seconds, and with triphasic complex morphology. Magnetic resonance imaging revealed diffuse white matter changes, mostly around the posterior regions and lateral ventricles. Treatment with valproic acid, levetiracetam, carbamazepine, and intravenous immunoglobulin G was ineffective. Inosiplex and interferon-β-1a were also administrated. The patient became comatose, with generalized myoclonic jerks, and died 1 year later. An autopsy was not performed. This patient illustrates that subacute sclerosing panencephalitis should be suspected among young vaccinated subjects. Copyright © 2011 Elsevier Inc. All rights reserved.

  16. Subacute sclerosing panencephalitis in pregnancy.

    Science.gov (United States)

    Chiu, Michael H; Meatherall, Bonnie; Nikolic, Ana; Cannon, Kristine; Fonseca, Kevin; Joseph, Jeffrey T; MacDonald, Judy; Pabbaraju, Kanti; Tellier, Raymond; Wong, Sallene; Koch, Marcus W

    2016-03-01

    We present a case of subacute sclerosing panencephalitis that developed in a previously healthy 29-year-old pregnant woman who had returned from a trip to rural India shortly before the onset of symptoms. She was admitted to hospital at 27 weeks' gestation with a history of cognitive decline and difficulty completing simple tasks. She had no clinical signs of infection. The working diagnosis was autoimmune encephalitis, although extensive investigations did not lead to a final classifying diagnosis. The patient became comatose and developed hypertension, and an emergency caesarean section was done at 31 weeks to deliver the child, who seemed healthy. The patient died about 6 weeks after the onset of symptoms. The patient was found to have had subacute sclerosing panencephalitis at autopsy. In this Grand Round, we review the clinical features and treatment of subacute sclerosing panencephalitis, and the epidemiological and public health aspects of the case. Copyright © 2016 Elsevier Ltd. All rights reserved.

  17. Primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    Marina G Silveira

    2008-01-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy. PSC primarily affects young and middle-aged men, often in association with underlying inflammatory bowel disease. The etiology of PSC includes immune-mediated components and elements of undefined nature. A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase, nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies, and diffuse multifocal biliary strictures, resulting in a ‘beaded’ appearance on radiographic studies, are the hallmarks of the disease. No effective medical therapy is currently available, although clinical studies are in progress. Ursodeoxycholic acid at high doses (28 mg/kg/day to 30 mg/kg/day is the most promising agent but is unproven so far. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease, although recurrent disease can be observed in the transplanted liver. The multiple complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, peristomal varices, bacterial cholangitis, dominant biliary strictures, gallbladder stones and polyps, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of PSC.

  18. Subacute sclerosing panencephalitis (SSPE) presenting as acute disseminated encephalomyelitis in a child.

    Science.gov (United States)

    Goraya, Jatinder; Marks, Harold; Khurana, Divya; Legido, Agustin; Melvin, Joseph

    2009-07-01

    Subacute sclerosing panencephalitis (SSPE) typically presents with progressive mental deterioration, behavioral changes, and myoclonic jerks. Atypical presentations are not unknown and may result in diagnostic delays. A 9-year-old girl presented with poor balance and ataxia following an episode of upper respiratory tract infection. Neurological examination revealed mild hemiparesis and ataxia. Brain magnetic resonance imaging revealed scattered areas of T2 and fluid-attenuated inversion recovery hyperintensities in the white matter consistent with acute disseminated encephalomyelitis. Despite treatment with intravenous methylprednisolone, intravenous immunoglobulins, and plasmapheresis, progressive neurological worsening occurred. Later during the course of her illness, subacute sclerosing panencephalitis was suspected from the appearance of burst-suppression pattern on electroencephalogram, and the diagnosis confirmed by elevated titers of measles antibodies in cerebrospinal fluid. Physicians taking care of children need to be aware of atypical presentations of subacute sclerosing panencephalitis and must have a high index of suspicion to prevent diagnostic delays and avoid unnecessary diagnostic and therapeutic interventions.

  19. Radiologic Findings of Immunoglobulin G4 Related Sclerosing Esophagitis: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Mi Sun; Kim, Su Young; Lee, Byung Hoon; Hwang, Yoon Joon; Han, Yoon Hee [Dept. of Radiology, Ilsan Paik Hospital, Inje University College of Medicine, Koyang (Korea, Republic of)

    2012-02-15

    We describe a case of immunoglobulin G4 (IgG4)-related sclerosing esophagitis occurring in a 63-year-old man with progressive dysphagia and 10-kg weight loss over 9 months. An esophagoscopy revealed significant stricture with diffuse mucosal friability and ulceration at mid esophagus level. Barium esophagogram showed diffuse stenosis at the mid and lower esophagus levels with ulcerations and irregularity of the mucosa. Multidetector computed tomography revealed diffuse edematous and circumferential thickening of the submucosa and muscle layer of this esophageal segment. Fluorine 18 fluorodeoxyglucose positron emission tomography (FDG PET) revealed diffuse mild FDG uptake in mid to lower esophagus. Although benign inflammatory lesion was suspected based on the imaging findings, the patient underwent surgery for worsening esophageal stricture and the esophageal lesion was pathologically confirmed as IgG4-related sclerosing esophagitis. Radiologic benignancy and high clinical suspicion for IgG4-related sclerosing disease may help making a proper decision and avoiding unnecessary operation.

  20. Glucocorticosteroids for primary sclerosing cholangitis.

    Science.gov (United States)

    Giljaca, Vanja; Poropat, Goran; Stimac, Davor; Gluud, Christian

    2010-01-20

    Primary sclerosing cholangitis is a chronic cholestatic disease of intrahepatic and extrahepatic biliary ducts, characterised by chronic periductal inflammation and sclerosis of the ducts, which results in segmental stenoses of bile ducts, cholestasis, fibrosis, and ultimately, liver cirrhosis. Patients with primary sclerosing cholangitis are at higher risk of cholangiocarcinoma as well as of colonic neoplasia, since primary sclerosing cholangitis is associated with inflammatory bowel disease in more than 80% of the patients. Several therapeutic modalities have been proposed for primary sclerosing cholangitis, like ursodeoxycholic acid, glucocorticosteroids, and immunomodulatory agents, but none has been successful in reversing the process of the disease. To date, liver transplantation is the only definite therapeutic solution for patients with advanced primary sclerosing cholangitis with liver cirrhosis. To assess the beneficial and harmful effects of glucocorticosteroids for patients with primary sclerosing cholangitis. We searched The Cochrane Hepato-Biliary Group Controlled Trials Register, The Cochrane Central Register of Controlled Trials in The Cochrane Library, MEDLINE, EMBASE, and LILACS from their inception until September 2009, as well as reference lists. Randomised clinical trials comparing any dose or duration of glucocorticosteroids versus placebo, no intervention, or other immunosuppressive agents. We included trials irrespective of language, blinding, or publication status. Authors extracted data independently and assessed the methodological quality by the generation of the allocation sequence, allocation concealment, double blinding, follow-up, incomplete outcome data reporting, selective reporting, baseline imbalance, and early stopping. The results of the meta-analyses were presented as relative risks (RR) or mean difference (MD), both with 95% confidence intervals (CI). The primary outcome measures were mortality and liver-related morbidity. Two

  1. Primary sclerosing cholangitis associated with systemic sclerosis.

    Science.gov (United States)

    Fraile, G; Rodríguez-García, J L; Moreno, A

    1991-02-01

    Primary sclerosing cholangitis is a chronic inflammatory fibrotic disorder strongly associated with inflammatory bowel disease. Although an association between some inflammatory fibrotic conditions, such as Riedel's thyroiditis and retroperitoneal fibrosis and primary sclerosing cholangitis has been described, to our knowledge there are no reports of primary sclerosing cholangitis in patients with systemic sclerosis. A patient with this combination of conditions is presented and the possible significance of the association discussed.

  2. Primary sclerosing cholangitis associated with systemic sclerosis.

    OpenAIRE

    Fraile, G.; Rodríguez-García, J. L.; Moreno, A.

    1991-01-01

    Primary sclerosing cholangitis is a chronic inflammatory fibrotic disorder strongly associated with inflammatory bowel disease. Although an association between some inflammatory fibrotic conditions, such as Riedel's thyroiditis and retroperitoneal fibrosis and primary sclerosing cholangitis has been described, to our knowledge there are no reports of primary sclerosing cholangitis in patients with systemic sclerosis. A patient with this combination of conditions is presented and the possible ...

  3. Subacute sclerosing panencephalitis: A clinical appraisal

    National Research Council Canada - National Science Library

    Jagtap, Sujit Abajirao; Nair, M D; Kambale, Harsha J

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus...

  4. Idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne M; Jensen, Rigmor H

    2015-01-01

    AIMS: The aims of this article are to characterize the headache in idiopathic intracranial hypertension (IIH) and to field-test the ICHD diagnostic criteria for headache attributed to IIH. MATERIALS AND METHODS: We included 44 patients with new-onset IIH. Thirty-four patients with suspected...... tinnitus may suggest intracranial hypertension. Based on data from a well-defined IIH cohort, we propose a revision of the ICDH-3 beta diagnostic criteria with improved clinical applicability and increased sensitivity and specificity....

  5. Pathogenesis of primary sclerosing cholangitis

    OpenAIRE

    Pollheimer, Marion J.; Halilbasic, Emina; Fickert, Peter; Trauner, Michael

    2011-01-01

    Primary sclerosing cholangitis (PSC) represents a chronic cholestatic liver disease with fibroobliterative sclerosis of intra- and/or extrahepatic bile ducts, eventually leading to biliary cirrhosis. The association with human leukocyte antigen (HLA) and non-HLA haplotypes and the presence of autoantibodies in sera of PSC patients support a crucial role for immune-mediated mechanisms in the initiation and progression of PSC. The strong clinical association between PSC and inflammatory bowel d...

  6. Pathogenesis of primary sclerosing cholangitis.

    Science.gov (United States)

    Pollheimer, Marion J; Halilbasic, Emina; Fickert, Peter; Trauner, Michael

    2011-12-01

    Primary sclerosing cholangitis (PSC) represents a chronic cholestatic liver disease with fibroobliterative sclerosis of intra- and/or extrahepatic bile ducts, eventually leading to biliary cirrhosis. The association with human leukocyte antigen (HLA) and non-HLA haplotypes and the presence of autoantibodies in sera of PSC patients support a crucial role for immune-mediated mechanisms in the initiation and progression of PSC. The strong clinical association between PSC and inflammatory bowel diseases led to intriguing pathogenetic concepts, in which the inflamed gut with translocation of bacterial products and homing of gut-primed memory T lymphocytes via aberrantly expressed adhesion molecules plays a fundamental role. Genetically or chemically modified bile composition was shown to induce sclerosing cholangitis and liver fibrosis in a number of animal models ("toxic bile concept"). The potential role of vascular injury with ischemia of bile duct epithelium cells in the development of sclerosing cholangitis is supported by animal models of endothelial cell injury showing close morphological similarities with human PSC. 2011 Elsevier Ltd. All rights reserved.

  7. Pathogenesis of primary sclerosing cholangitis

    Science.gov (United States)

    Pollheimer, Marion J.; Halilbasic, Emina; Fickert, Peter; Trauner, Michael

    2011-01-01

    Primary sclerosing cholangitis (PSC) represents a chronic cholestatic liver disease with fibroobliterative sclerosis of intra- and/or extrahepatic bile ducts, eventually leading to biliary cirrhosis. The association with human leukocyte antigen (HLA) and non-HLA haplotypes and the presence of autoantibodies in sera of PSC patients support a crucial role for immune-mediated mechanisms in the initiation and progression of PSC. The strong clinical association between PSC and inflammatory bowel diseases led to intriguing pathogenetic concepts, in which the inflamed gut with translocation of bacterial products and homing of gut-primed memory T lymphocytes via aberrantly expressed adhesion molecules plays a fundamental role. Genetically or chemically modified bile composition was shown to induce sclerosing cholangitis and liver fibrosis in a number of animal models ("toxic bile concept"). The potential role of vascular injury with ischemia of bile duct epithelium cells in the development of sclerosing cholangitis is supported by animal models of endothelial cell injury showing close morphological similarities with human PSC. PMID:22117638

  8. D-penicillamine for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Klingenberg, S L; Chen, Weikeng

    2006-01-01

    Primary sclerosing cholangitis is a cholestatic disease. D-penicillamine is suggested as a treatment option due to its copper reducing and immunomodulatory potential.......Primary sclerosing cholangitis is a cholestatic disease. D-penicillamine is suggested as a treatment option due to its copper reducing and immunomodulatory potential....

  9. Bile acids for primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Poropat, Goran; Giljaca, Vanja; Stimac, Davor

    2011-01-01

    Primary sclerosing cholangitis is a progressive chronic cholestatic liver disease that usually leads to the development of cirrhosis. Studies evaluating bile acids in the treatment of primary sclerosing cholangitis have shown a potential benefit of their use. However, no influence on patients...

  10. Sclerosing PEComa: A Histologic Surprise | Valiathan | African ...

    African Journals Online (AJOL)

    Sclerosing PEComa: A Histologic Surprise. M Valiathan, BP Sunil, L Rao, V Geetha, P Hedge. Abstract. PEComa represents a group of mesenchymal tumors consisting of perivascular epithelioid cells. We present a 50-year old female patient with a rare distinctive variant, sclerosing PEComa, characterized by extensive ...

  11. Measles virus–specific plasma cells are prominent in subacute sclerosing panencephalitis CSF

    Science.gov (United States)

    Owens, G.P.; Ritchie, A.M.; Gilden, D.H.; Burgoon, M.P.; Becker, D.; Bennett, J.L.

    2012-01-01

    Objective To demonstrate the specificity of expanded CD138+ plasma cell clones recovered from the CSF of a patient with subacute sclerosing panencephalitis (SSPE) for measles virus (MV). Methods IgG variable region sequences of single-antibody-secreting CD138+ cells sorted from SSPE CSF were amplified by single-cell PCR and analyzed. Human IgG1 recombinant antibodies (rAbs) were produced from four expanded CD138+ clones and assayed for immunoreactivity against MV proteins. Results Clonal expansion was a prominent feature of the SSPE plasma cell repertoire, and each of the four rAbs assayed was specific for either the MV fusion or the MV nucleocapsid protein. Conclusions Expanded plasma cell clones in the CSF of patients with subacute sclerosing panencephalitis produce disease-relevant antibodies. Recombinant antibodies derived from CSF B cells could provide a tool to identify target antigens in idiopathic inflammatory disorders. PMID:17515543

  12. Spooky Suspects

    Science.gov (United States)

    Pacifici, Lara

    2011-01-01

    This activity presents an option for covering biology content while engaging students in an investigation that highlights the spirit of Halloween. Students are engaged in the story line and have fun trying to solve the mystery kidnapping by using science skills to examine the evidence and eliminate some ghoulish suspects. (Contains 1 figure.)

  13. Idiopathic Anaphylaxis

    OpenAIRE

    Nwaru, Bright; Dhami, Sangeeta; Sheikh, Aziz

    2017-01-01

    Opinion statement Idiopathic anaphylaxis is a rare life-threatening disorder with symptoms similar to other forms of anaphylaxis. There is lack of a robust evidence base underpinning the treatment of anaphylaxis and even less so for idiopathic anaphylaxis. Much of the evidence therefore comes from relatively small case series and expert opinion. Idiopathic anaphylaxis is a diagnosis of exclusion, requiring a thorough history and careful diagnostic work-up investigating possible triggers and u...

  14. Familial subacute sclerosing panencephalitis associated with short latency.

    Science.gov (United States)

    Sharma, Vinod; Gupta, Vineet B; Eisenhut, Michael

    2008-03-01

    The familial recurrence of subacute sclerosing panencephalitis is rare. The study of such cases and a comparison of intrafamilial with sporadic cases of subacute sclerosing panencephalitis may shed light on important pathogenetic factors. We report on the occurrence of subacute sclerosing panencephalitis in two brothers from rural India, who contracted measles infection simultaneously at ages 3 and 11 years. They developed subacute sclerosing panencephalitis 21 and 37 months later, respectively. A diagnosis of subacute sclerosing panencephalitis was based on history, electroencephalographic changes, and significantly raised levels of cerebrospinal-fluid anti-measles virus immunoglobulin G. A comparison of intrafamilial with sporadic cases of subacute sclerosing panencephalitis revealed that latency in familial subacute sclerosing panencephalitis involved a median of 6.4 years (range, 1.0-10.9), significantly (P panencephalitis from the Indian subcontinent. We confirmed a more rapid manifestation of subacute sclerosing panencephalitis after measles virus infection in intrafamilial compared with sporadic subacute sclerosing panencephalitis.

  15. Survey of subacute sclerosing panencephalitis in Japan.

    Science.gov (United States)

    Abe, Yusaku; Hashimoto, Koichi; Iinuma, Kazuie; Ohtsuka, Yoko; Ichiyama, Takashi; Kusuhara, Koichi; Nomura, Keiko; Mizuguchi, Masashi; Aiba, Hideo; Suzuki, Yasuhiro; Mizusawa, Hidehiro; Hosoya, Mitsuaki

    2012-12-01

    Investigators conducted a retrospective epidemiological study of subacute sclerosing panencephalitis, a fatal disease caused by measles infection, over the past few years in Japan. Data on 118 cases obtained from a questionnaire sent to attending physicians were analyzed. The annual incidence of subacute sclerosing panencephalitis was approximately 0.03 cases per million from 2001 to 2005. Children infected with measles at a young age (panencephalitis, and those infected before 6 months of age showed earlier onset. Because a positive correlation was found between the prevalence of measles and the onset of subacute sclerosing panencephalitis, particularly among children infected at an early age, it is vital to eradicate measles infection by vaccination.

  16. Role of Carbamazepine in the Symptomatic Treatment of Subacute Sclerosing Panencephalitis: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Sandhya Ravikumar

    2013-01-01

    Full Text Available We describe the clinical presentation and clinical course of subacute sclerosing panencephalitis in a 13-year-old previously healthy boy who recently immigrated to the United States from Iraq. He presented with macular retinopathy, followed by progressive myoclonus and encephalopathy. After extensive workup, a diagnosis of subacute sclerosing panencephalitis was suspected by the presence of period epileptiform discharges on electroencephalogram and confirmed by elevated measles titers in the cerebrospinal fluid. Combination immunomodulatory therapy with isoprinosine, ribavirin, and intra-Ommaya interferon alpha did not result in clinical improvement. Within days following the administration of carbamazepine, there was remarkable improvement in the myoclonus and he was able to ambulate independently for a period of 4 months at which time he unfortunately progressed to a vegetative state. This case highlights the importance of carbamazepine as a potential first line symptomatic treatment of subacute sclerosing panencephalitis and provides a review of the literature on the subject.

  17. Role of carbamazepine in the symptomatic treatment of subacute sclerosing panencephalitis: a case report and review of the literature.

    Science.gov (United States)

    Ravikumar, Sandhya; Crawford, John Ross

    2013-01-01

    We describe the clinical presentation and clinical course of subacute sclerosing panencephalitis in a 13-year-old previously healthy boy who recently immigrated to the United States from Iraq. He presented with macular retinopathy, followed by progressive myoclonus and encephalopathy. After extensive workup, a diagnosis of subacute sclerosing panencephalitis was suspected by the presence of period epileptiform discharges on electroencephalogram and confirmed by elevated measles titers in the cerebrospinal fluid. Combination immunomodulatory therapy with isoprinosine, ribavirin, and intra-Ommaya interferon alpha did not result in clinical improvement. Within days following the administration of carbamazepine, there was remarkable improvement in the myoclonus and he was able to ambulate independently for a period of 4 months at which time he unfortunately progressed to a vegetative state. This case highlights the importance of carbamazepine as a potential first line symptomatic treatment of subacute sclerosing panencephalitis and provides a review of the literature on the subject.

  18. Update on primary sclerosing cholangitis.

    Science.gov (United States)

    Karlsen, Tom H; Boberg, Kirsten Muri

    2013-09-01

    Primary sclerosing cholangitis (PSC) remains one of the most challenging conditions of clinical hepatology. There has been a steady growth in research to overcome this fact and the present review aims at summarizing the most recently published literature. The main emphasis will be put on the link of recent pathogenetic insights to clinical characteristics and patient management. With regard to pathogenesis, there is no consensus yet as to whether immune mediated injury or factors related to bile acid physiology are the most important. It also remains to be clarified whether PSC is a mixed bag of various secondary etiologies yet to be defined, or a disease entity predominantly represented by sclerosing cholangitis in the context of inflammatory bowel disease. Most important, there is no available medical therapy with proven influence on clinical end points, and timing of liver transplantation and patient follow-up are challenging due to the unpredictable and high risk of cholangiocarcinoma. Copyright © 2013 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

  19. [A child with primary sclerosing cholangitis].

    Science.gov (United States)

    Rademaker, Anouk A E M; Benninga, Marc A; Beuers, Ulrich H W; Plötz, Frans B

    2015-01-01

    Primary sclerosing cholangitis is a rare liver disease which is mainly diagnosed in adults. This chronic progressive disease, characterised by inflammation, fibrosis and strictures of the intra- and extrahepatic bile ducts, leads to cirrhosis. There is a strong association between primary sclerosing cholangitis and inflammatory bowel disease (IBD). A 10-year-old boy presented at the accident and emergency department with fever, episodes of abdominal pain, nausea, vomiting, fatigue and hepatomegaly. Blood tests, pathology investigations, liver biopsy and magnetic resonance cholangiopancreatography (MRCP) led to the diagnosis of primary sclerosing cholangitis. The patient was treated with ursodeoxycholic acid and later, because of unbearable itching, sequentially with lidocaine 3% ointment, rifampicin, an endoprosthesis in the common bile duct and glucocorticoids. One year later he returned to the paediatrician with abdominal pain and bloody diarrhoea. Endoscopy revealed features of indeterminate colitis. Remission of the disease was achieved quickly after treatment with mesalazine. Primary sclerosing cholangitis can develop in childhood and is often associated with IBD.

  20. Primary sclerosing cholangitis and liver transplantation

    NARCIS (Netherlands)

    Klompmaker, IJ; Haagsma, EB; Jansen, PLM; Slooff, MJH

    1996-01-01

    Primary sclerosing cholangitis is a chronic disease, strongly associated with ulcerative colitis and cholangiocarcinoma. Ulcerative colitis itself does not influence the liver transplant results. However; intensified screening after liver transplantation for carcinoma of the colon may be necessary.

  1. Sclerosing haemangioma arising within extralobar pulmonary sequestration

    Energy Technology Data Exchange (ETDEWEB)

    Ahmetoglu, Ali; Kosucu, Polat; Guemele, Halit Resit [Department of Radiology, Farabi Hospital, Karadeniz Technical University, 61080 Trabzon (Turkey); Imamoglu, Mustafa; Cay, Ali [Department of Paediatric Surgery, Farabi Hospital, Karadeniz Technical University, Trabzon (Turkey); Reis, Abdulkadir [Department of Pathology, Farabi Hospital, Karadeniz Technical University, Trabzon (Turkey)

    2003-09-01

    Extralobar pulmonary sequestration is a rare anomaly of abnormal pulmonary tissue without any communication to the bronchial tree. Sclerosing haemangioma is a rare lung tumour, generally seen in middle-aged women. The combination of these two rare pathologies has not been previously reported. We describe the CT and CT angiographic findings of sclerosing haemangioma arising within an extralobar pulmonary sequestration in a 2-year-old girl. (orig.)

  2. Peritumoral Hyperplasia in Hepatic Sclerosed Hemangioma

    OpenAIRE

    Shimada, Seitarou; Tajiri, Kazuto; Baba, Hayato; Minemura, Masami; Tsuneyama, Koichi; Nakano, Masayuki; Sugiyama, Toshiro

    2017-01-01

    Peritumoral hyperplasia (PTH) is a hyperplastic lesion located around hypervascular tumors. Hepatic sclerosed hemangioma is a very rare form of hemangioma with sclerotic changes and is distinct from sclerosing hemangioma. We present a patient with non-alcoholic steatohepatitis-induced cirrhosis who presented with a hypervascular tumor. The tumor showed atypical findings of hemangioma and was treated with surgical resection because hepatic malignancy could not be ruled out. Histopathologic exa...

  3. Sclerosing encapsulating peritonitis: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Candido, Paula de Castro Menezes; Werner, Andrea de Freitas; Pereira, Izabela Machado Flores; Matos, Breno Assuncao; Pfeilsticker, Rudolf Moreira; Silva Filho, Raul, E-mail: paulacmcandido@yahoo.com.br [Hospital Felicio Rocho, Belo Horizonte, MG (Brazil)

    2015-01-15

    Sclerosing encapsulating peritonitis, a rare cause of bowel obstruction, was described as a complication associated with peritoneal dialysis which is much feared because of its severity. The authors report a case where radiological findings in association with clinical symptoms have allowed for a noninvasive diagnosis of sclerosing encapsulating peritonitis, emphasizing the high sensitivity and specificity of computed tomography to demonstrate the characteristic findings of such a condition. (author)

  4. Pseudotumor cerebri secondary to subacute sclerosing panencephalitis.

    Science.gov (United States)

    Ayçiçek, Ali; Işcan, Akin; Ceçe, Hasan

    2009-05-01

    Unusual presentations are not rare in subacute sclerosing panencephalitis. Five patients initially diagnosed with pseudotumor cerebri were ultimately determined to have pseudotumor cerebri secondary to subacute sclerosing panencephalitis. The present study retrospectively reviewed 56 cases history, neurologic symptoms, and clinical and laboratory data, as well as the outcomes. On admission, five patients (group 1) presenting with pseudotumor cerebri exhibited bilateral papilledema, and in each of them cranial magnetic resonance imaging revealed small lateral ventricles, effacement of the subarachnoid space, and no mass lesion. On admission, 51 patients (group 2) had no pseudotumor cerebri findings. The year of original measles infection, the interval between measles and onset of subacute sclerosing panencephalitis, and initial neurologic symptoms were similar, but length of symptoms before diagnosis of subacute sclerosing panencephalitis was shorter in group 1, and the clinical stage of subacute sclerosing panencephalitis on admission was more advanced in group 2. Cerebrospinal fluid mean open pressure was 378 +/- 22 H(2)O in group 1 and 146 +/- 28 H(2)O in group 2; cerebrospinal fluid antibody was 2038 +/- 768 U/L in group 1 and was 664 +/- 214 U/L in group 2. Only three of the five patients with pseudotumor cerebri had typical periodic discharges on electroencephalographic examination. These findings suggest that subacute sclerosing panencephalitis can cause pseudotumor cerebri.

  5. Garre′s sclerosing osteomyelitis

    Directory of Open Access Journals (Sweden)

    Suma R

    2007-05-01

    Full Text Available Garre′s sclerosing osteomyelitis is a specific type of chronic osteomyelitis that mainly affects children and young adults. This disease entity is well-described in dental literature and is commonly associated with an odontogenic infection resulting from dental caries. This paper describes a case of Garre′s osteomyelitis in a 10-year-old boy, in whom the condition arose following pulpoperiapical infection in relation to permanent mandibular right first molar. Clinically the patient presented with bony hard, non-tender swelling and the occlusal radiograph revealed pathognomic feature of "onion skin" appearance. The elimination of periapical infection was achieved by endodontic therapy and the complete bone remodeling was seen radiographically after three months follow-up.

  6. MRI in subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Tuncay, R. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Akman-Demir, G. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Goekyigit, A. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Eraksoy, M. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Barlas, M. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Tolun, R. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey); Guersoy, G. [Department of Neurology, Istanbul Medical Faculty, University of Istanbul (Turkey)

    1996-10-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive, slow virus infection of the brain, caused by the measles virus, attacking children and young adults. We investigated 15 patients with SSPE by MRI, with 5 normal and 10 pathological results. In the early period, lesions were in the grey matter and subcortical white matter. They were asymmetrical and had a predilection for the posterior parts of the hemispheres. Later, high-signal changes in deep white matter and severe cerebral atrophy were observed. Parenchymal lesions significantly correlated with the duration of disease. A significant relationship between MRI findings and clinical stage was observed in the 1st year of the disease. (orig.). With 4 figs., 1 tab.

  7. [Eosinophilic cholangitis from almost normal appearance to the bile duct sclerosis similar to primary sclerosing cholangitis].

    Science.gov (United States)

    Kimura, Yoshito; Yamashita, Yukimasa; Mikami, Sakae; Ono, Hiroshi; Itai, Ryosuke; Matsumoto, Yoshihide; Yamada, Satoshi; Takada, Mariko; Sumitomo, Yasuhiko

    2013-02-01

    A 48-year-old man was admitted to our hospital because of eosinophilia and liver dysfunction. Initial abdominal CT and MRI (MRCP) finding showed almost normal liver and bile duct. Liver biopsy demonstrated mild portal infiltration of lymphocytes and eosinophils. Definitive diagnosis was difficult, but we suspected autoimmune disease. Oral steroid administration was started, which led to a rapid improvement of eosinophilia and liver dysfunction. Dose reduction of steroid administration resulted in exacerbation of eosinophilia and liver dysfunction. Follow-up MRCP and ERCP study revealed biliary strictures similar to primary sclerosing cholangitis (PSC). A second liver biopsy revealed dense infiltration composed of lymphocytes and eosinophils in the portal area. Therefore we diagnosed eosinophilic cholangitis. This is the first case of eosinophilic cholangitis, observed after changes of the bile duct from an almost normal appearance to diffuse sclerosing and narrowing similar to PSC by imaging and pathological studies.

  8. Primary sclerosing cholangitis and Biliary cirrhosis associated with Ulcerative colitis

    Directory of Open Access Journals (Sweden)

    Aleem Ahmed Khan

    2011-07-01

    Full Text Available Primary sclerosing cholangitis and Ulcerative colitis are caused by progressive inflammation of the bile duct and large intestine respectively. The existence of any plausible association between Primary sclerosing cholangitis and Ulcerative colitis remains highly elusive. Little is known about the incidence and prevalence of primary sclerosing cholangitis with concomitant Ulcerative colitis in the Indian subcontinent. We report a case of Primary sclerosing cholangitis with long standing Ulcerative colitis which later also developed Primary biliary cirrhosis.

  9. Idiopathic granulomatous mastitis.

    Science.gov (United States)

    Ozturk, Erkan; Akin, Murat; Can, Mehmet F; Ozerhan, Ismail; Kurt, Bulent; Yagci, Gokhan; Tufan, Turgut

    2009-01-01

    To discuss the clinical and radiological features and treatment approaches in 14 patients diagnosed with idiopathic granulomatous mastitis (GM). We retrospectively evaluated the clinical features, radiological findings, and treatment approaches in 14 patients with idiopathic GM in the General Surgery Department, Gulhane School of Medicine, Ankara, Turkey between April 2000 and June 2006. The mean age of the patients was 34.5 years (range 27-41 years). The complaints at admission were a mass in the breast in 7 (50%) patients, an abscess and a mass in 6 (42.8%), and a skin fistula in one (7.2%). Granulomatous mastitis was unilateral in all subjects (on the right in 5 patients and on the left in 9). All of the patients underwent ultrasonographic evaluation. Mammography was performed in 8 and magnetic resonance imaging in 5 patients. Seven patients (50%) were suspected to have breast carcinoma according to radiological findings. We performed large excision in 11, incisional biopsy plus abscess drainage in one, and incisional biopsy plus abscess drainage plus medical treatment (prednisolone, methotrexate) in 2 patients. Due to the development of abscess after 9 months, drainage and large excision were also performed in one patient who received medical treatment. Idiopathic GM is a disease that generally affects young women of reproductive age and may be mistaken for breast carcinoma in clinical and radiological evaluations. The gold standard for the diagnosis is histopathologic evaluation.

  10. Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis.

    Science.gov (United States)

    Siu, K K; Li, Rever; Lam, S Y

    2015-04-01

    This report demonstrates pulmonary haemorrhage as a differential cause of anaemia. Idiopathic pulmonary hemosiderosis is a rare disease in children; it is classically described as a triad of haemoptysis, pulmonary infiltrates on chest radiograph, and iron-deficiency anaemia. However, anaemia may be the only presenting feature of idiopathic pulmonary hemosiderosis in children due to occult pulmonary haemorrhage. In addition, the serum ferritin is falsely high in idiopathic pulmonary hemosiderosis which increases the diagnostic difficulty. We recommend that pulmonary haemorrhage be suspected in any child presenting with iron-deficiency anaemia and persistent bilateral pulmonary infiltrates.

  11. Subacute sclerosing panencephalitis: an update.

    Science.gov (United States)

    Gutierrez, Jose; Issacson, Richard S; Koppel, Barbara S

    2010-10-01

    Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis occurring after infection with measles virus. The prevalence of the disease varies depending on uptake of measles vaccination, with the virus disproportionally affecting regions with low vaccination rates. The physiopathology of the disease is not fully understood; however, there is evidence that it involves factors that favour humoral over cellular immune response against the virus. As a result, the virus is able to infect the neurons and to survive in a latent form for years. The clinical manifestations occur, on average, 6 years after measles virus infection. The onset of SSPE is insidious, and psychiatric manifestations are prominent. Subsequently, myoclonic seizures usually lead to a final stage of akinetic mutism. The diagnosis is clinical, supported by periodic complexes on electroencephalography, brain imaging suggestive of demyelination, and immunological evidence of measles infection. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Trials of treatment with interferon, ribavirin, and isoprinosine using different methodologies have reported beneficial results. However, the disease shows relentless progression; only 5% of individuals with SSPE undergo spontaneous remission, with the remaining 95% dying within 5 years of diagnosis.

  12. The evolution of natural history of primary sclerosing cholangitis.

    Science.gov (United States)

    Takakura, Will R; Tabibian, James H; Bowlus, Christopher L

    2017-03-01

    Primary sclerosing cholangitis (PSC) is a rare, idiopathic biliary disease often with an insidious onset, variable disease course, and premature death related to benign and malignant PSC-related sequelae. This review aims to discuss the epidemiology, clinical variants, and natural history of PSC, incorporating data from recent population-based studies. PSC naturally leads to cirrhosis, cholangiocarcinoma, other hepatobiliary malignancies, dominant strictures, hepatic osteodystrophy, and bacterial cholangitis. The incidence of PSC appears to be increasing, the reasons for which are unclear. The time from diagnosis to liver transplant appears to be longer in more recent studies compared with earlier studies, suggesting a better overall prognosis than previously believed. In addition, with an increasing number of patients undergoing liver transplantation for PSC, the frequency of death because of liver failure has decreased, whereas cancer-related deaths have increased among patients with PSC. PSC is a heterogeneous disease with a variety of clinical outcomes, both fatal and nonfatal. The progression of liver fibrosis in an individual patient is difficult to predict and may vary from a relatively benign, nonprogressive form to a rapidly progressive form with the need for liver transplantation.

  13. A new lethal sclerosing bone dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kingston, H.M. (Saint Mary' s Hospital, Manchester (UK). Regional Genetics Centre); Freeman, J.S. (Tameside General Hospital, Ashton-under-Lyne (UK). Dept. of Paediatrics); Hall, C.M. (Hospital for Sick Children, London (UK). Dept. of Paediatric Radiology)

    1991-02-01

    A neonate is described with a lethal sclerosing bone dysplasia associated with prenatal fractures and craniofacial abnormalities including microcephaly, exophthalmos, hypoplastic nose and mid-face, small jaw and nodular hyperplasia of the gums. Parental consanguinity suggests that an autosomal recessive mutation is the likely aetiology. (orig.).

  14. Primary sclerosing cholangitis: a clinical review

    NARCIS (Netherlands)

    Ponsioen, C. I.; Tytgat, G. N.

    1998-01-01

    Primary sclerosing cholangitis (PSC) is a cholestatic liver disease characterized by fibro-obliterative inflammation of the entire biliary tree. It is a slowly progressive disease with an undulating course, resulting in terminal biliary cirrhosis after a median period of about 12 years after

  15. Inflammatory bowel disease with primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    Sørensen, Jakob Ørskov; Nielsen, Ole Haagen; Andersson, Mikael

    2017-01-01

    BACKGROUND AND AIMS: Inflammatory bowel disease (IBD) may be complicated by primary sclerosing cholangitis (PSC). We aimed to assess the characteristics of Danish PSC-IBD patients and to compare their prognosis with IBD patients without PSC. METHODS: A retrospective nationwide population...

  16. Risk factors for primary sclerosing cholangitis

    NARCIS (Netherlands)

    Boonstra, Kirsten; de Vries, Elisabeth M G; van Geloven, Nan; van Erpecum, Karel J.; Spanier, Marcel; Poen, Alexander C; van Nieuwkerk, Carin M; Witteman, Ben J; Tuynman, Hans A; Naber, Anton H; Kingma, Paul J; Beuers, Ulrich; Ponsioen, Cyriel Y

    BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease of unknown cause, but strongly associated with inflammatory bowel disease (IBD). Potential risk factors triggering PSC have never been studied on a population level. The aim of this study was to

  17. Sclerosing polycystic adenosis: a case report

    NARCIS (Netherlands)

    Kloppenborg, R. P.; Sepmeijer, J. W.; Sie-Go, D. M. D. S.; De Ru, J. A.; Slootweg, P. J.

    2006-01-01

    About 70 to 80 percent of all salivary gland neoplasms, the majority of which are benign, arise in the parotid gland. Sclerosing Polycystic Adenosis (SPA) is a relatively unknown and newly described entity that is considered to be benign in nature. A 55-year-old patient was treated for SPA in our

  18. Subacute Sclerosing Panencephalitis: The Foothold in Undervaccination.

    Science.gov (United States)

    Holt, Rebecca L; Kann, Dylan; Rassbach, Caroline E; Schwenk, Hayden T; Ritter, Jana M; Rota, Paul A; Elbers, Jorina

    2016-12-01

    Subacute sclerosing panencephalitis (SSPE) is a fatal complication of measles infection. We present a case of a fully vaccinated 3-year-old boy who was diagnosed with and treated for autoimmune encephalitis before arriving at a diagnosis of SSPE. We discuss the challenges of diagnosing SSPE in developed countries. Copyright © 2016 Elsevier Inc. All rights reserved.

  19. Sclerosing peritonitis with gross calcification: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Cheung Sook; Kim, Young Jae; Min, Seon Jeong; Cho, Seong Whi; Lee, Gyung Kyu; Lee, Eil Seong; Kang, Ik Won [Hallym University College of Medicine, Seoul (Korea, Republic of)

    2003-09-01

    Sclerosing peritonitis is an uncommon complication of continuous ambulatory peritoneal dialysis (CAPD) and can lead to small bowel dysfunction involving abdominal pain, progressive loss of ultrafiltration, and small intestinal obstruction. Peritoneal thickening, in which calcification can develop, often starts as al small plaque which gradually becomes larger. We report a case of CAPD-related calcifying peritonitis.

  20. Sclerosing nevus with pseudomelanomatous features.

    Science.gov (United States)

    Fabrizi, Giuseppe; Pennacchia, Ilaria; Pagliarello, Calogero; Massi, Guido

    2008-11-01

    Among the pigmented lesions with a central area of scar, we found a group of cases histologically characterized by striking architectural alteration of the melanocytic component, but with no cytological atypia and mitotically quiescent. The aim of the current study was to assess the biological nature of such lesions. We selected 19 of these melanocytic neoplasms that had the following characteristics: (a) a clinically evident whitish central area suggestive of regression (with no history of a previous surgical procedure or trauma), (b) histological features of fibrous scar-like tissue at the center of the lesion, (c) the presence of large, confluent and unusually shaped melanocytic nests at the dermoepidermal junction and in the dermis, (d) a pagetoid spread of melanocytes above the epidermal basal layer and (e) remnants of nevus tissue at the border of the scar. The lesions showed no evidence of cytological atypia, expansive nodules of melanocytes, significant numbers of mitoses or cellular necrosis. All the cases have been followed up and none have recurred or metastasized. Histologically, these neoplasms have important similarities with the so-called recurrent nevus, nevi on lichen sclerosus and nevi developed during or following cutaneous inflammatory and sclerosing processes. The origin of the scar in each case was obscure but was probably related to minor unnoticed trauma or to chronic friction on a nevus. In few cases, the fibrosis was probably the result of partial regression of the nevus or a sequel to folliculitis. The pseudomelanomatous features appear to be related to the presence of the scar, as already reported for nevi that are involved in fibrotic or scarring processes. In our study, the nevi involved in the fibrotic process were congenital nevi and common or dysplastic nevi. One case was a Spitz nevus. From our data we concluded that, despite their worrisome clinical and histological aspect, the lesions described in this case series were most

  1. Subacute Sclerosing Panencephalitis in a Child with Recurrent Febrile Seizures

    OpenAIRE

    Ayşe Kartal; Ayşegül Neşe Çıtak Kurt; Tuğba Hirfanoğlu; Kürşad Aydın; Ayşe Serdaroğlu

    2015-01-01

    Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recu...

  2. Subacute sclerosing panencephalitis in a child with recurrent febrile seizures.

    Science.gov (United States)

    Kartal, Ayşe; Çıtak Kurt, Ayşegül Neşe; Hirfanoğlu, Tuğba; Aydın, Kürşad; Serdaroğlu, Ayşe

    2015-01-01

    Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis.

  3. Subacute Sclerosing Panencephalitis in a Child with Recurrent Febrile Seizures

    Directory of Open Access Journals (Sweden)

    Ayşe Kartal

    2015-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a devastating disease of the central nervous system (CNS caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis.

  4. Idiopathic Anaphylaxis.

    Science.gov (United States)

    Nwaru, Bright I; Dhami, Sangeeta; Sheikh, Aziz

    2017-01-01

    Idiopathic anaphylaxis is a rare life-threatening disorder with symptoms similar to other forms of anaphylaxis. There is lack of a robust evidence base underpinning the treatment of anaphylaxis and even less so for idiopathic anaphylaxis. Much of the evidence therefore comes from relatively small case series and expert opinion. Idiopathic anaphylaxis is a diagnosis of exclusion, requiring a thorough history and careful diagnostic work-up investigating possible triggers and underlying predisposing factors. Key diagnostic tests include skin-prick testing, tests for specific-IgE, component-resolved diagnostics, and in some cases for allergen challenge tests. Other recognized causes of anaphylaxis, such as foods, medications, insect stings, latex, and exercise, should all be considered, as should differential diagnoses such as asthma. While the cause of idiopathic anaphylaxis remains unknown, prompt treatment with intramuscular epinephrine (adrenaline) administered into the anterolateral aspect of the thigh is associated with good prognosis. There may also be a role for H1-antihistamines and corticosteroids as second-line agents. Patients need to be carefully monitored for signs of deterioration and/or a possible protracted or biphasic reaction. Patients with frequent episodes of anaphylaxis (e.g., six or more episodes/year) should be considered for preventive therapy, which may include corticosteroids, H1- and H2-antihistamines, and, in some cases, mast cell stabilizers such as ketotifen. Alternative immune-suppressants (e.g., methotrexate) and anti-IgE may rarely also need to be considered. In many cases, the frequency of anaphylaxis declines such that regular use of corticosteroids can be discontinued after 9-12 months. Pediatric patients should be treated with similar regimens as adults, but with appropriate dose adjustments. Patients should carry their self-injectable epinephrine and other emergency medications at all times in order to deal with emergency

  5. Idiopathic Scoliosis

    OpenAIRE

    Jens Ivar Brox

    2014-01-01

    Introduction. Idiopathic scoliosis is a structural and lateral curvature of the spine for which a currently recognizable cause has not been found and there is no basic evidence for physical and radiographic pathology. Complications. Scoliosis could be a cause of the back pain, deformities, respiratory and cardiology problems. There is a higher risk for decreasing of bone mineral density. Diagnosis and Management. Physical examination, radiography and stereophotogrametry are used in diag...

  6. Sclerosing Wegener's granulomatosis in the orbit

    DEFF Research Database (Denmark)

    Ostri, Christoffer; Heegaard, Steffen; Prause, Jan Ulrik

    2008-01-01

    PURPOSE: To report three cases of sclerosing Wegener's granulomatosis in the orbit and to compare the histopathological morphology with those of other types of sclerosing orbital inflammation. METHODS: We analysed the clinical data along with histopathological specimens from orbital biopsies......, tissue necrosis, vasculitis and widespread dense fibrosis. In certain areas of all specimens the fibrous tissue was arranged in characteristic concentric, onionskin-like whorls of collagen around obliterated small blood vessels. CONCLUSIONS: Wegener's granulomatosis may lead to orbital fibrosis...... and sclerosis. The stromal changes and cellular infiltrate resemble the non-specific orbital inflammation previously termed orbital pseudotumour. We present the first 'onionskin' lesions in association with Wegener's granulomatosis in the orbit. This latter morphology may represent an abnormal fibrotic reaction...

  7. Brainstem involvement in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Sharma, Pawan; Singh, Dileep; Singh, Maneesh Kumar; Garg, Ravindra Kumar; Kohli, Neera

    2011-01-01

    The parieto-occipital region of the brain is most frequently and severely affected in subacute sclerosing panencephalitis (SSPE). The basal ganglia, cerebellum and corpus callosum are less commonly involved. Brainstem involvement is rarely described in SSPE, and usually there is involvement of other regions of the brain. We describe a patient with subacute sclerosing panencephalitis with brain magnetic resonance imaging showing extensive brainstem involvement without significant involvement of other cortical structures. Though rarely described in SSPE, one should be aware of such brainstem and cerebellum involvement, and SSPE should be kept in mind when brainstem signal changes are seen in brain MRI with or without involvement of other regions of brain to avoid erroneous reporting.

  8. Subacute sclerosing panencephalitis in immunized Thai children.

    Science.gov (United States)

    Khusiwilai, Khanittha; Viravan, Sorawit

    2011-12-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive neurodegenerative disease with high mortality and poor prognosis. This is caused by persistent defective measles virus infection. Clinical presentations are variable including behavioral-cognitive change, myoclonic seizure, visual problem, spasticity or abnormal movement. The authors report a case of 10 year-old boy, previously healthy with complete immunization, presenting with frequent myoclonic jerks, abnormal movements, spasticity and altered mental status. Electroencephalographic (EEG), magnetic resonance imaging (MRI), and laboratory findings are typical for SSPE.

  9. Subacute sclerosing panencephalitis presenting as mania

    Directory of Open Access Journals (Sweden)

    Aggarwal Ashish

    2011-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized.

  10. Late Onset Subacute Sclerosing Panencephalitis: Presenting Psychosis

    Directory of Open Access Journals (Sweden)

    Yavuz Altunkaynak

    2013-04-01

    Full Text Available Subacute Sclerosing Panencephalitis (SSPE is the late complication of measles and is characterized by seizures, myoclonus, ataxia, behavioral and personality changes, extrapyramidal dysfunctions and vision problems. A 19 year old female patient with SSPE who was followed up at psychiatry clinic with the diagnosis of atypical psychotic disorder was presented. While psychiatric signs and symptoms were dominant, she was diagnosed as SSPE.

  11. Subacute sclerosing panencephalitis: serial electroencephalographic studies.

    Science.gov (United States)

    Wulff, C H

    1982-01-01

    A total of 42 EEGs from five patients with subacute sclerosing panencephalitis were studied. Periodic complexes were noticed in 35 (83%) of these. The interval between the complexes shortened in all patients with progression of the illness. The gradual EEG changes may reflect the increasing number of infected cells as well as an on-going accumulation of immature virus structures. The records without complexes were either from the early onset (one record) or terminal stage (six records). PMID:7086454

  12. Subacute sclerosing panencephalitis presenting as mania

    Science.gov (United States)

    Aggarwal, Ashish; Khandelwal, Ashish; Jain, Manish; Jiloha, R. C.

    2011-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare, invariably fatal degenerative disease of the central nervous system developing after measles infection. Besides neurological symptoms as initial presenting symptoms, rare reports of its presentation with pure psychiatric symptoms have been reported. We here report a case of 14 year old male who initially presented with manic symptoms and then subsequently diagnosed to be suffering from SSPE. Improtance of ruling our organic conditions is emphasized. PMID:21808475

  13. Cytogenetic study of a pulmonary sclerosing hemangioma.

    Science.gov (United States)

    Pareja, María J; Vargas, María T; Sánchez, Ana; Ibáñez, José; González-Cámpora, Ricardo

    2009-11-01

    Pulmonary sclerosing hemangioma (PSH) is an uncommon benign tumor that presents as a solitary asymptomatic and slow-growing nodule. It occurs in both young and old persons; peak incidence is in the fifth decade. Both sexes are affected by this tumor, but women more frequently than men. On histological examination, PSH shows prominent sclerotization and vascularization of the tissue. Recent studies conclude that PSH derives from type II pneumocytes, but the potential for progression and histogenesis remains controversial. We report a case of pulmonary sclerosing hemangioma in a 61-year-old woman with a neoplastic node 1 cm in diameter. The karyotype was 46,XX,t(8;18),der(14;15),+14 in all the cells analyzed. PTEN (10q23) and IgH (14q32) probes were analyzed in interphase nuclei and paraffin-embedded tissues of tumor cells. These chromosome abnormalities could provide information about the relationship of genetic changes to the biological properties of sclerosing hemangioma tumors.

  14. Ultrasonographic findings of sclerosing encapsulating peritonitis

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jong Kyu; Lee, Hae Kyung; Moon, Chul; Hong, Hyun Sook; Kwon, Kwi Hyang; Choi, Deuk Lin [Soonchunhyangi University College of Medicine, Seoul (Korea, Republic of)

    2001-03-15

    To evaluate the ultrasonographic findings of the patients with sclerosing encapsulating peritonitis (SEP). Thirteen patients with surgically confirmed sclerosing encapsulating peritonitis were involved in this study. Because of intestinal obstruction, all patients had received operations. Among 13 patients, 12 cases had continuous ambulatory peritoneal dialysis (CAPD) for 2 months-12 years and 4 months from (mean; 6 years and 10 months), owing to chronic renal failure and one patient had an operation due to variceal bleeding caused by liver cirrhosis. On ultrasonographic examination, all patients showed loculated ascites which were large (n=7) or small (n=6) in amount with multiple separations. The small bowel loops were tethered posteriorly perisaltic movement and covered with the thick membrane. The ultrasonographic of findings of sclerosing encapsulating peritonitis were posteriorly tethered small bowels covered with a thick membrane and loculated ascites with multiple septa. Ultrasonographic examination can detect the thin membrane covering the small bowel loops in the early phase of the disease, therefore ultrasonography would be a helpful modality to diagnose SEP early.

  15. Diagnosis, Differential Diagnosis, and Epidemiology of Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Ponsioen, Cyriel Y.

    2015-01-01

    According to recent guidelines, primary sclerosing cholangitis (PSC) is diagnosed when a patient has a cholestatic liver enzyme profile, characteristic bile duct changes on imaging, and when secondary causes of sclerosing cholangitis are excluded. In patients with a clinical suspicion but normal

  16. Glucocorticosteroids for primary sclerosing cholangitis. Protocol for a Cochrane Review

    DEFF Research Database (Denmark)

    Chen, W; Gluud, C

    2003-01-01

    Glucocorticosteroids have been suggested for primary sclerosing cholangitis, which is characterised by chronic inflammation and fibrosis in the intrahepatic and extrahepatic biliary tree.......Glucocorticosteroids have been suggested for primary sclerosing cholangitis, which is characterised by chronic inflammation and fibrosis in the intrahepatic and extrahepatic biliary tree....

  17. Sclerosing cholangitis with ulcerative colitis in a Nigerian woman ...

    African Journals Online (AJOL)

    Primary Sclerosing Cholangitis (PSC) is a relatively rare cause of chronic liver disease worldwide. It presents as chronic cholestasis associated with jaundice and pruritus. We report a middle aged Nigerian woman ... in the diagnosis of chronic liver diseases. Keywords: Sclerosing cholangitis, Nigeria, cholestasis, diarrhea ...

  18. PRIMARY SCLEROSING CHOLANGITIS IN CHILDREN AND ADOLESCENTS.

    Science.gov (United States)

    Fagundes, Eleonora Druve Tavares; Ferreira, Alexandre Rodrigues; Hosken, Caroline Caldeira; Queiroz, Thaís Costa Nascentes

    2017-12-01

    Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18. Diagnosis was established through clinical, laboratory, radiographic and/or histopathologic criteria. Other chronic liver diseases were excluded, as well as secondary causes of cholangitis. Data analysis used statistic resources in SPSS software. Variables were expressed as averages, standard deviation, absolute frequency, and percentage. Twenty-one patients fulfilled criteria to be included in the research sample. Male patients predominated (3.2:1) and average age at diagnosis was 6.7±3.9 years. Five (23.8%) patients had associated inflammatory bowel disease, four had ulcerative colitis and one indeterminate colitis. Signs and symptoms vary and are usually discrete at presentation. The most frequent symptom was abdominal pain (47.6%) followed less frequently by jaundice (28.6%) and itching (14.3%). The reason for medical investigation was asymptomatic or oligosymptomatic enzyme alterations in 33.3% of patients. All patients presented increased hepatic enzymes: aminotransferases, gamma glutamyl transferase, and alkaline phosphatase. Twenty patients had alterations compatible to primary sclerosing cholangitis in their cholangiography exam; one patient had no alterations at magnetic resonance cholangiography, but presented histopathologic alterations that were compatible to small duct cholangitis. Hepatic

  19. Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Jens Ivar Brox

    2014-07-01

    Full Text Available Idiopathic scoliosis (IS is a lifetime condition and is defined as a structural, lateral rotated curvature of the spine of >10° on standing coronal plane radiographs. It should be distinguished from other causes of scoliosis. It can be classified as infantile, juvenile, and adolescent according to age. As a rule of thumb, about 80% of all curves are idiopathic, right convex thoracic, and present in otherwise healthy girls at the beginning of puberty. A family member most commonly detects scoliosis. The structural asymmetry of the spine is best observed by asking the patient to bend forward. IS is often seen in more than one member of a family, but the aetiology remains unknown. Multiple genes are likely to be involved with incomplete penetrance and variable expressivity. Early detection by screening allows for monitoring curve progression and timely initiation of bracing, but school screening is controversial and practises vary worldwide. Most patients have minor scoliosis and treatment is generally not recommended for patients with curves 45°. Scoliosis surgery was not successful until the introduction of Harrington’s instrumentation in the 1960s. Modern instrumentation has evolved from the Cotrel-Dubousset system in the 1980s, and a variety of methods are available today. Although scoliosis may be a burden, long-term studies suggest that a good quality of life is maintained in most patients.

  20. Primary sclerosing cholangitis: a clinical update.

    Science.gov (United States)

    Williamson, Kate D; Chapman, Roger W

    2015-06-01

    Primary sclerosing cholangitis (PSC) is a progressive cholestatic disorder that ultimately can lead to cirrhosis, liver failure, malignancy and death. It is strongly associated with inflammatory bowel disease (IBD), and though a rare disease, its incidence is increasing. There are no proven medical therapies for PSC. Ovid Medline was utilised to search for articles with keywords 'sclerosing cholangitis' and 'cholangiocarcinoma' and containing titles 'primary sclerosing cholangitis', and references of these papers were cross-referenced for further relevant manuscripts. PSC is a rare disease, and there is a strong association with risk loci within the major histocompatibility complex and other genes common to other autoimmune diseases. PSC is a premalignant condition, associated with higher rates of hepatobiliary and colorectal cancer in patients with ulcerative colitis (UC). The pathogenesis is unclear, and competing theories exist surrounding toxic bile acids, enhanced homing of particular T cells from the gut to the liver and increased passage of toxins to the liver through a permeable bowel wall. It is unclear whether the higher rate of colonic cancer in PSC/UC occurs in PSC/Crohn's disease. Ursodeoxycholic acid therapy reduces liver enzymes but has not been shown to improve survival. It may reduce the prevalence of bowel cancer. Recent genetic studies have revealed new risk loci, pointing to the importance of the immune system and its interaction with the biome. On the basis of the genetic studies discussed earlier, novel agents are being developed and trialled in the treatment of PSC. © The Author 2015. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  1. Endoscopic retrograde cholangiopancreatography for primary sclerosing cholangitis.

    Science.gov (United States)

    Thosani, Nirav; Banerjee, Subhas

    2014-11-01

    Although there are no randomized, controlled trials evaluating the efficacy of endoscopic retrograde cholangiography (ERC) in primary sclerosing cholangitis (PSC) patients, substantial indirect evidence supports the effectiveness of ERC in symptomatic PSC patients with a dominant stricture. Currently, cumulative evidence supports the role of ERC with endoscopic dilation with or without additional short-term stent placement for symptomatic PSC patients with a dominant stricture. Differentiating benign dominant strictures from cholangiocarcinoma (CCA) remains difficult; however, newer endoscopic techniques and advanced cytologic techniques are likely to improve sensitivity for the diagnosis of CCA over that achieved by traditional cytology brushing alone. Copyright © 2014 Elsevier Inc. All rights reserved.

  2. Pathophysiological Mechanisms in Sclerosing Skin Diseases

    Directory of Open Access Journals (Sweden)

    Beate Eckes

    2017-08-01

    Full Text Available Sclerosing skin diseases represent a large number of distinct disease entities, which include systemic sclerosis, localized scleroderma, and scleredema adultorum. These pathologies have a common clinical appearance and share histological features. However, the specific interplay between cytokines and growth factors, which activate different mesenchymal cell populations and production of different extracellular matrix components, determines the biomechanical properties of the skin and the clinical features of each disease. A better understanding of the mechanisms underlying these events is prerequisite for developing novel targeted therapeutic approaches.

  3. Subacute sclerosing panencephalitis: a case report.

    Science.gov (United States)

    Praticò, A D; Saporito, M; Iacono, O; Castellano-Chiodo, D; Pavone, P

    2011-02-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence caused by persistent defective measles virus. Clinical manifestations appear many years after the acute measles infection. The incidence of SSPE has substantially declined after the introduction of an effective vaccine. We report a case of a child with SSPE that began with atonia, dysarthria, and intellectual deterioration without the presence of any particular EEG anomalies. We have reported this girl who was affected by this severe affliction in the hope that, because of the rarity of SSPE, it would not go undiagnosed.

  4. Computed tomography appearances of sclerosing encapsulating peritonitis

    Energy Technology Data Exchange (ETDEWEB)

    George, C. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom)]. E-mail: cheriangeorge@hotmail.com; Al-Zwae, K. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom); Nair, S. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom); Cast, J.E.I. [Department of Radiology, Hull Royal Infirmary, Hull (United Kingdom)

    2007-08-15

    Sclerosing encapsulating peritonitis (SEP) is a serious complication of peritoneal dialysis (PD) characterized by thickened peritoneal membranes, which lead to decreased ultra-filtration and intestinal obstruction. Its early clinical features are nonspecific, and it is often diagnosed late following laparotomy and peritoneal biopsy, when the patient develops small bowel obstruction, which can be a life-threatening complication. However, this is changing with increasing awareness of computed tomography (CT) findings in SEP. CT can yield an early, non-invasive diagnosis that may improve patient outcome. We present a review of the CT appearances of SEP.

  5. Lymphadenopathy of IgG4-related sclerosing disease.

    Science.gov (United States)

    Cheuk, Wah; Yuen, Hunter K L; Chu, Stephenie Y Y; Chiu, Edmond K W; Lam, L K; Chan, John K C

    2008-05-01

    IgG4-related sclerosing disease is a recently recognized syndrome characterized by mass-forming lesions in exocrine glands or extranodal tissues due to lymphoplasmacytic infiltrates and sclerosis, a raised serum IgG4 level and increased IgG4+ plasma cells in the involved tissues. We report the morphologic features of the enlarged regional (n=3) and nonregional lymph nodes (n=3) in patients with this syndrome. The patients presented with autoimmune pancreatitis, lymphoplasmacytic sclerosing cholangitis, chronic sclerosing dacryoadenitis, or chronic sclerosing sialadenitis. The histologic features of the lymph nodes could be categorized into 3 patterns: Castleman diseaselike, follicular hyperplasia, and interfollicular expansion by immunoblasts and plasma cells. The percentage of IgG4+/IgG+ plasma cells was markedly elevated (mean 62% vs. 9.9% in 54 control lymph nodes comprising a wide variety of reactive conditions). We also report 6 cases of primary lymphadenopathy characterized by increased IgG4+/IgG+plasma cells (mean 58%). These cases share many clinical and pathologic similarities with IgG4-related sclerosing disease. In fact, 2 of these patients developed lymphoplasmacytic sclerosing cholangitis or lacrimal and submandibular gland involvement during the clinical course. These cases therefore probably represent primary lymph node manifestation of the disease. The importance of recognition of the lymphadenopathic form of IgG4-related sclerosing disease lies in the remarkable response to steroid therapy, and the potential of mistaking the disease for lymphoma either clinically or histologically.

  6. Definition and pathology of primary sclerosing cholangitis.

    Science.gov (United States)

    Nakanuma, Y; Harada, K; Katayanagi, K; Tsuneyama, K; Sasaki, M

    1999-01-01

    Although primary sclerosing cholangitis (PSC) is not a common disease, it is important in the differential diagnosis of hepatobiliary tract diseases in clinical practice. A diagnosis of PSC should be made only after the exclusion of similar diseases with well known etiologies or pathogeneses. In this review, the pathology of classical PSC and its variants or related diseases is highlighted. PSC is histologically characterized by progressive periductal fibrosis with luminal stenosis or obliteration, along with the formation of a fibrous core, as well as dilatation (cholangiectasis). Its etiology is unknown. Bacterial ascending cholangitis is superimposed on its long clinical course. Such a heterogeneous distribution of biliary lesions with biliary obliteration and cholangiectasis is responsible for the radiological demonstration of biliary abnormalities, particularly the beaded appearance. Sampling variability is common in needle or wedge biopsied specimens. As a result of biliary damage, the liver shows progressive cholestatic change followed by biliary fibrosis and cirrhosis, and this hepatic progression is divisible into four stages. There are several variants of PSC or related diseases, such as localized biliary sclerosis and stenosis, sclerosing cholangitis associated with inflammatory pseudotumor, and PSC-autoimmune hepatitis overlapping syndrome. Cholelithiasis, including secondary hepatolithiasis and, to a lesser degree, biliary carcinoma and dysplasia, are also known to develop at the perihilar bile ducts as a late complication of PSC.

  7. Primary sclerosing cholangitis: diagnostic and management challenges

    Directory of Open Access Journals (Sweden)

    Sirpal S

    2017-11-01

    Full Text Available Sanjeev Sirpal,1 Natasha Chandok2 1Department of Medicine, Centre Hospitalier de l’Université de Montréal (CHUM, University of Montreal, Montreal, QC, 2Department of Medicine, University of Western Ontario, London, ON, Canada Abstract: Primary sclerosing cholangitis (PSC is a chronic immune-mediated disease affecting intra- and extrahepatic bile ducts, primarily the large biliary ducts. Clinical manifestations are broad, and the spectrum encompasses asymptomatic cholestasis, icteric cholangitis with pruritis, cirrhosis, and cholangiocarcinoma. Though rare, PSC has a propensity to affect young to middle-aged males and is strongly associated with inflammatory bowel disease. There is an unmet need for effective medical treatments for PSC, and to date, the only curative therapy is liver transplantation reserved for those with end-stage liver disease. This article addresses the diagnostic and management challenges of PSC, with a succinct analysis of existing therapies, their limitations, and a glimpse into the future of the management of this multifaceted pathologic entity. Keywords: primary sclerosing cholangitis, management, PSC

  8. Clinical profile of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Akram, Muhammad; Naz, Farrah; Malik, Akbar; Hamid, Haroon

    2008-08-01

    To describe the clinical manifestations of subacute sclerosing panencephalitis in children. Case series. This study was conducted in the Department of Neurology at The Children's Hospital and the Institute of Child Health, Lahore, from April 2005 to April 2007. Fifty patients were diagnosed as subacute sclerosing panencephalitis during the study period. Their diagnosis was based on a detailed history, clinical examination, presence of antimeasles antibodies in Cerebrospinal Fluid (CSF) and typical electroencephalogram (EEG). The findings were described as average, mean and percentages. Fifty patients were included in this study. The average age of the patients was 8 years. Thirty-eight (76%) were males and 12 (24%) were females. The average duration of symptoms before presentation was 66.72 days. History of measles infection was present in 31 patients (62%) and measles vaccination in 43 patients (86%). Motor regression was present in all (100%) patients and cognition decline in 43 patients (86%). Seizures were focal (10%), generalized tonicclonic (16%) and myoclonic (74%). Burst-suppression pattern Electroencephalogram (EEG) and the antimeasles antibody in CSF were positive in 100% of patients. SSPE is an indicator of high incidence of measles infection among the paediatric population even among vaccinated children. Males are more common sufferers. SSPE can present with different types of seizures, cognition decline and motor regression being supported by suggestive EEG and presence of anti-measles antibodies in CSF.

  9. Primary Hepatic Adenosquamous Carcinoma Associated with Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Yamao, Kentaro; Takenaka, Mamoru; Imai, Hajime; Nakai, Atsushi; Omoto, Shunske; Kamata, Ken; Minaga, Kosuke; Miyata, Takeshi; Sakurai, Toshiharu; Watanabe, Tomohiro; Nishida, Naoshi; Matsumoto, Ippei; Takeyama, Yosihumi; Chikugo, Takaaki; Kudo, Masatoshi

    2017-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disorder characterized by multiple fibrotic strictures of the bile duct. More than 40% of deaths in PSC patients are related to malignant tumors, including cholangiocarcinoma. Primary hepatic adenosquamous carcinoma (ASC) is a rare subtype of cholangiocarcinoma containing adenocarcinoma (AC) and squamous cell carcinoma (SCC) components, with a poorer prognosis than other cholangiocarcinomas. We report the first case of a hepatic ASC in a patient with PSC. A 28-year-old man was referred for diagnosis and treatment of a liver abscess suspected by contrast-enhanced computed tomography (CE-CT). He had a history of ulcerative colitis and PSC. Abdominal CE-CT revealed a 60-mm-diameter ring-shaped mass with central necrosis in the left lobe. Magnetic resonance imaging demonstrated a poorly circumscribed low-signal-intensity mass in T1-weighted imaging and a high-signal-intensity mass with a scattered low-signal-intensity area in T2-weighted imaging. Abdominal ultrasonography showed a hypoechoic component with a diffuse hyperechoic area in the tumor. Ultrasound-guided biopsy and histological examination showed tumor cells with both squamous and glandular differentiation. Left lobectomy was performed. Microscopic examination revealed 2 components, including moderately differentiated AC and well-differentiated SCC. The final diagnosis was hepatic ASC. This is the first reported case of hepatic ASC in a patient with PSC. Patients with PSC should be recognized as being at a risk of not only general cholangiocarcinoma, hepatocellular carcinoma, and metastatic liver tumor, but also ASC. © 2017 S. Karger AG, Basel.

  10. Clinical features and management of primary sclerosing cholangitis

    Science.gov (United States)

    Silveira, Marina G; Lindor, Keith D

    2008-01-01

    Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in cirrhosis and need for liver transplantation and reduced life expectancy. The majority of cases occur in young and middle-aged men, often in association with inflammatory bowel disease. The etiology of primary sclerosing cholangitis includes immune-mediated components and elements of undefined nature. No effective medical therapy has been identified. The multiple complications of primary sclerosing cholangitis include metabolic bone disease, dominant strictures, bacterial cholangitis, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of primary sclerosing cholangitis. Liver transplantation is currently the only life-extending therapeutic alternative for patients with end-stage disease, although recurrence in the allografted liver has been described. A PSC-like variant attracting attention is cholangitis marked by raised levels of the immunoglobulin G4 subclass, prominence of plasma cells within the lesions, and steroid responsiveness. PMID:18528931

  11. Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity

    National Research Council Canada - National Science Library

    Pavan S. Upadhyayula; Jason Yang; John K. Yue; Joseph D. Ciacci

    2017-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine...

  12. Pathogenesis and medical therapy of primary sclerosing cholangitis. Any news?

    NARCIS (Netherlands)

    van den Berg, A; Jansen, PLM

    Primary sclerosing cholangitis is characterized by inflammation and fibrosis of the intra- and extrahepatic bile ducts. Medical therapy has focused on anticholestatic, antiinflammatory and immunosuppressive drugs. Although inflammation, fibrosis and cholestasis may all occur at the same time,

  13. Genetics Home Reference: polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy

    Science.gov (United States)

    ... Lipomembranous Osteodysplasia with Sclerosing Leukoencephalopathy (PLOSL) MedlinePlus Encyclopedia: Dementia General Information from MedlinePlus (5 links) Diagnostic Tests Drug Therapy Genetic Counseling Palliative Care Surgery and Rehabilitation Related Information How are genetic ...

  14. Idiopathic oedema and diuretics.

    Science.gov (United States)

    Dunnigan, M. G.; Denning, D. W.; Henry, J. A.; de Wolff, F. A.

    1987-01-01

    Diuretic abuse has been invoked as the cause of idiopathic oedema. In this study, eight patients with idiopathic oedema were studied. Symptoms and weight variation continued despite the proven absence of diuretics in seven of them as determined by urinary chromatograms. Idiopathic oedema cannot therefore be attributed to diuretic use alone. PMID:3671223

  15. Persistent idiopathic facial pain.

    Science.gov (United States)

    Benoliel, Rafael; Gaul, Charly

    2017-06-01

    Background Persistent idiopathic facial pain (PIFP) is a chronic disorder recurring daily for more than two hours per day over more than three months, in the absence of clinical neurological deficit. PIFP is the current terminology for Atypical Facial Pain and is characterized by daily or near daily pain that is initially confined but may subsequently spread. Pain cannot be attributed to any pathological process, although traumatic neuropathic mechanisms are suspected. When present intraorally, PIFP has been termed 'Atypical Odontalgia', and this entity is discussed in a separate article in this special issue. PIFP is often a difficult but important differential diagnosis among chronic facial pain syndromes. Aim To summarize current knowledge on diagnostic criteria, differential diagnosis, pathophysiology and management of PIFP. Methods We present a narrative review reporting current literature and personal experience. Additionally, we discuss and differentiate the common differential diagnoses associated with PIFP including traumatic trigeminal neuropathies, regional myofascial pain, atypical neurovascular pains and atypical trigeminal neuropathic pains. Results and conclusion The underlying pathophysiology in PIFP is still enigmatic, however neuropathic mechanisms may be relevant. PIFP needs interdisciplinary collaboration to rule out and manage secondary causes, psychiatric comorbidities and other facial pain syndromes, particularly trigeminal neuralgia. Burden of disease and psychiatric comorbidity screening is recommended at an early stage of disease, and should be addressed in the management plan. Future research is needed to establish clear diagnostic criteria and treatment strategies based on clinical findings and individual pathophysiology.

  16. Subacute sclerosing panencephalitis: brain stem involvement in a peculiar pattern

    Energy Technology Data Exchange (ETDEWEB)

    Senol, U. [Akdeniz University, Antalya (Turkey). Faculty of Medicine; Haspolat, S. [Akdeniz University, Antalya (Turkey). Dept. of Child Neurology; Cevikol, C. [Akdeniz University, Antalya (Turkey). Dept. of Radiodiagnostics; Saatci, I. [Hacettepe University (Turkey). Medical Faculty

    2000-12-01

    The most common pattern in subacute sclerosing panencephalitis, is in the cerebral hemisphere white matter on T2-weighted images with or without atrophy. Brain-stem lesions are rare. We report brain-stem involvement in two children with subacute sclerosing panencephalitis. A peculiar pattern, with involvement of the pons with extension to both middle cerebellar peduncles and substantia nigra but sparing the pontine tegmentum, is suggested. (orig.)

  17. A case of cutaneous scleroderma with primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    H P Nandeesh

    2014-01-01

    Full Text Available Sclerosing cholangitis comprises of a spectrum of cholestatic conditions that are characterized by patchy fibrosis, inflammation and destruction of intra hepatic and extrahepatic ducts. We report a case of a 42 year old woman who presented with darkening of skin with yellowish discolouration of the eyes. Clinical examination revealed icterus, taut skin with hepatosplenomegaly. Liver function tests showed a cholestatic picture. Skin biopsy showed features of cutaneous scleroderma. MRCP and Liver biopsy was suggestive of sclerosing cholangitis.

  18. Mineral Oil-induced Sclerosing Lipogranuloma of the Penis

    OpenAIRE

    Bjurlin, Marc A.; Carlsen, Jens; Grevious, Mark; Jordan, Michael D.; Taylor, Aisha; Divakaruni, Naveen; Hollowell, Courtney M. P.

    2010-01-01

    Sclerosing lipogranuloma of the penis results from injection of high viscosity fluid for the purpose of penile augmentation and may have devastating cosmetic and sexual function consequences. Although rare, sclerosing lipogranuloma should be considered in the differential diagnosis of subcutaneous induration or nodules of the male genitalia as it may mimic carcinoma and poses a diagnostic challenge in patients reluctant to admit to injection therapy. Surgical excision with penile reconstructi...

  19. Serial diffusion-weighted imaging in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Kanemura, Hideaki; Aihara, Masao

    2008-06-01

    Subacute sclerosing panencephalitis may be associated with clinical features of frontal lobe dysfunction. We previously reported that frontal lobe volume falls significantly as clinical stage progresses, using three-dimensional magnetic resonance imaging-based brain volumetry. The hypothesis that frontal volume increases correlate with clinical improvement, however, was not tested in our previous study. Therefore, we reevaluated our patient with subacute sclerosing panencephalitis, to determine whether apparent diffusion coefficient maps can characterize the clinical course of subacute sclerosing panencephalitis. We studied an 8-year-old boy with subacute sclerosing panencephalitis, using serial diffusion-weighted imaging magnetic resonance imaging, and measured the regional apparent diffusion coefficient. The regional apparent diffusion coefficient of the frontal lobe decreased significantly with clinical progression, whereas it increased to within normal range during clinical improvements. The apparent diffusion coefficient of the other regions did not change. These results suggest that the clinical signs of patients with subacute sclerosing panencephalitis are attributable to frontal lobe dysfunction, and that apparent diffusion coefficient measurements may be useful in predicting the clinical course of subacute sclerosing panencephalitis.

  20. Primary sclerosing cholangitis: diagnostic and management challenges

    Science.gov (United States)

    Sirpal, Sanjeev; Chandok, Natasha

    2017-01-01

    Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease affecting intra- and extrahepatic bile ducts, primarily the large biliary ducts. Clinical manifestations are broad, and the spectrum encompasses asymptomatic cholestasis, icteric cholangitis with pruritis, cirrhosis, and cholangiocarcinoma. Though rare, PSC has a propensity to affect young to middle-aged males and is strongly associated with inflammatory bowel disease. There is an unmet need for effective medical treatments for PSC, and to date, the only curative therapy is liver transplantation reserved for those with end-stage liver disease. This article addresses the diagnostic and management challenges of PSC, with a succinct analysis of existing therapies, their limitations, and a glimpse into the future of the management of this multifaceted pathologic entity. PMID:29138587

  1. Subacute sclerosing panencephalitis presenting as neuromyelitis optica.

    Science.gov (United States)

    Raut, Tushar Premraj; Singh, Maneesh Kumar; Garg, Ravindra Kumar; Naphade, Pravin Umakant

    2012-12-14

    Subacute sclerosing panencephalitis (SSPE) is a slowly progressing inflammatory and degenerative disorder of the brain caused by a mutant measles virus. The diagnosis of SSPE is based on characteristic clinical and EEG findings (periodic complexes) and demonstration of elevated antibody titres against measles in cerebrospinal fluid. SSPE can have atypical clinical features at the onset. The authors here report a case of a 3-year-old child who presented with vision loss followed 15 months later by quadriparesis with bladder involvement. These clinical features resembled that of neuromyelitis optica. However, as the disease progressed, appearance of myoclonic jerks, periodic discharges on EEG and positive cerebrospinal fluid serology for measles led to the final diagnosis of SSPE.

  2. Basal ganglia lesions in subacute sclerosing panencephalitis

    Directory of Open Access Journals (Sweden)

    Kelson James Almeida

    Full Text Available ABSTRACT The parieto-occipital region of the brain is the most frequently and severely affected in subacute sclerosing panencephalitis (SSPE. The basal ganglia, cerebellum and corpus callosum are less commonly involved. We describe a patient with SSPE confirmed by neuropathology based on brain magnetic resonance imaging showing extensive basal ganglia involvement and no significant involvement of other cortical structures. Though rarely described in SSPE, clinicians should be aware of this involvement. SSPE should be kept in mind when changes in basal ganglia signal are seen on brain magnetic resonance imaging with or without involvement of other regions of the human brain to avoid erroneous etiological diagnosis of other pathologies causing rapidly progressive dementia.

  3. Subacute Sclerosing Panencephalitis Presenting with Hemidystonia

    Directory of Open Access Journals (Sweden)

    Hepsen Mine Serin

    2014-06-01

    Full Text Available In this paper, we present a case of subacute sclerosing panencephalitis (SSPE in an 11-year-old boy who presented with hemidystonia. Electroencephalogram (EEG revealed periodic epileptiform discharges which did not disappear with diazepam induction. His cranial magnetic resonance imaging was normal. SSPE diagnosis was considered and it was confirmed with the identification of measles antibodies in cerebrospinal fluid. SSPE is a progressive disease. Hemidystonia is not an expected presentation of SSPE. We aimed to emphasize that SSPE may present with different clinical findings such as hemidystonia. (The Me­di­cal Bul­le­tin of Ha­se­ki 2014; 52: 137-9

  4. NEUTROPHIL CYTOPLASMIC AUTOANTIBODIES AFTER LIVER-TRANSPLANTATION IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS

    NARCIS (Netherlands)

    HAAGSMA, EB; MULDER, AHL; GOUW, ASH; HORST, G; MEERMAN, L; SLOOFF, MJH; KALLENBERG, CGM

    The immunopathogenic importance of neutrophil cytoplasmic autoantibodies in ulcerative colitis and primary sclerosing cholangitis is unknown. These autoantibodies were investigated before and after liver transplantation in 9 patients with primary sclerosing cholangitis. Sera from 10 patients

  5. NEUTROPHIL CYTOPLASMIC AUTOANTIBODIES AFTER LIVER-TRANSPLANTATION IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS

    NARCIS (Netherlands)

    Haagsma, E.B.; MULDER, A.H.L.; Gouw, A.S.H.; MEERMAN, L.; Slooff, M.JH; Kallenberg, Cees; Horst, G.

    1993-01-01

    The immunopathogenic importance of neutrophil cytoplasmic autoantibodies in ulcerative colitis and primary sclerosing cholangitis is unknown. These autoantibodies were investigated before and after liver transplantation in 9 patients with primary sclerosing cholangitis. Sera from 10 patients

  6. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial

    NARCIS (Netherlands)

    Beuers, U.; Spengler, U.; Kruis, W.; AYDEMIR, U.; WIEBECKE, B.; HELDWEIN, W.; WEINZIERL, M.; Pape, G. R.; Sauerbruch, T.; Paumgartner, G.

    1992-01-01

    The efficacy and safety of ursodeoxycholic acid for the treatment of primary sclerosing cholangitis were evaluated in a prospective, randomized, double-blind, placebo-controlled trial. Fourteen patients with primary sclerosing cholangitis documented by cholestatic serum enzyme pattern, liver

  7. Idiopathic lymphocytopenia.

    Science.gov (United States)

    Gholamin, Mehran; Bazi, Ali; Abbaszadegan, Mohammad Reza

    2015-01-01

    Idiopathic CD4⁺ lymphocytopenia (ICL) is defined by the reduction of the main lymphocyte subtype in peripheral blood and CD4⁺ T cells below 300/μl in the absence of any secondary known causes of lymphopenia, including viral causes. The present review aims to state the latest available data on clinical, pathological and therapeutic aspects related to ICL, published from 1990 to 2014. The last observed clinical presentation and complications of ICL patients are described. The latest findings and possible mechanisms involved in the development of ICL features are included in the present review; however, pathogenesis of ICL has remained mainly obscured. Finally, recent therapeutic efforts considered in ICL patients are discussed. In spite of the serious complications ICL has on the patients' quality of life, data on clinical, etiopathological and therapeutic behavior for ICL are very limited. On one side, an abnormal blood cell count may be the sole presentation; however, occurrence of disseminated malignant tumors is not uncommon in patients. Recent findings highlight the role of cytokines, especially interleukin-2, on features such as phenotype severity and responsiveness of the condition to therapy. In addition, some studies have suggested that a defect in hematopoietic stem cells may be involved in disease progression, an idea that is supported by the success of bone marrow transplantation in acquiring persistent remissions in ICL patients. ICL is a hematologic condition of increasing importance due to its diverse clinical and pathological spectrum. Molecular studies have shown the presence of mutations involved in lymphocyte development as potential factors that may contribute to ICL occurrence. ICL patients could present either with common infections or really serious malignant conditions. The role of cytokines, especially interleukin-2, has emerged as one of the main possible mechanisms involved in clinical and pathological behavior of ICL. Today, the main

  8. Subacute Sclerosing Panencephalitis in a Child with Human Immunodeficiency Virus Co-Infection

    OpenAIRE

    Maurya, Pradeep Kumar; Thakkar, Mayur Deepak; Kulshreshtha, Dinkar; Singh, Ajai Kumar; Thacker, Anup Kumar

    2016-01-01

    Subacute sclerosing panencephalitis is a fatal infectious disease of childhood caused by persistence of the measles virus in the brain. The effect of human immunodeficiency virus (HIV) co-infection on subacute sclerosing panencephalitis remains elusive and rare. We report a child who developed subacute sclerosing panencephalitis following a short latency period and a rapidly progressive course with HIV co-infection.

  9. FUS rearrangements are rare in 'pure' sclerosing epithelioid fibrosarcoma.

    Science.gov (United States)

    Wang, Wei-Lien; Evans, Harry L; Meis, Jeanne M; Liegl-Atzwanger, Bernadette; Bovee, Judith V M G; Goldblum, John R; Billings, Steven D; Rubin, Brian P; López-Terrada, Dolores; Lazar, Alexander J

    2012-06-01

    Several recent reports have described low-grade fibromyxoid sarcoma with sclerosing epithelioid fibrosarcoma-like areas. We evaluated cases of pure sclerosing epithelioid fibrosarcoma lacking areas of low-grade fibromyxoid sarcoma for FUS rearrangement to determine whether this entity could be related to low-grade fibromyxoid sarcoma. Available formalin-fixed paraffin-embedded tissue of 27 sclerosing epithelioid fibrosarcoma from 25 patients was retrieved and tabulated with clinical information. Unstained slides from formalin-fixed paraffin-embedded blocks were prepared and fluorescence in-situ hybridization was performed using a commercial FUS break-apart probe. The median patient age at presentation was 50 (range, 14-78) years, with 14 males and 10 females. Sclerosing epithelioid fibrosarcoma most commonly involved the extremities (n=8) or chest (n=6). Sixteen patients had a median follow-up of 17 (range, 1-99) months; seven were alive and well at 12 (range, 5-30) months; three alive with disease at 28 (range, 9-99) months; five dead of disease at a median of 22 (range, 1-36) months and one was dead of unknown causes. Twelve patients were known to have metastases; the most common site was lung (n=7), followed by bone (n=3), lymph nodes (n=2) and peritoneum (n=1). Only 2 of 22 (9%) analyzable cases of sclerosing epithelioid fibrosarcoma showed rearrangement in the FUS locus by fluorescence in-situ hybridization. Although cytogenetically confirmed low-grade fibromyxoid sarcoma can have sclerosing epithelioid fibrosarcoma-like areas, FUS rearrangement, which is characteristic of low-grade fibromyxoid sarcoma, appears to be relatively rare in pure sclerosing epithelioid fibrosarcoma.

  10. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

    Science.gov (United States)

    ... Asked Questions Español Condiciones Chinese Conditions Idiopathic Intracranial Hypertension (Pseudotumor Cerebri) En Español Read in Chinese What is idiopathic intracranial hypertension? Idiopathic intracranial hypertension (IIH) is a disorder that ...

  11. Idiopathic arterial calcification in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Patel, Maya [Department of Paediatric Radiology, Red Cross Children' s Hospital, Cape Town (South Africa); Red Cross Children' s Hospital, School of Child and Adolescent Health, University of Cape Town, Klipfontein Road, Rondebosch, Cape Town (South Africa); Andronikou, Savvas; Solomon, Rustum; Sinclair, Paul; McCulloch, Mignon [Department of Paediatric Radiology, Red Cross Children' s Hospital, Cape Town (South Africa)

    2004-08-01

    Idiopathic arterial calcification in infancy is usually fatal with death in early life and diagnosis at post mortem. This report describes a unique, late presentation with hypertension and cardiac failure in a child aged 33 months, found to have widespread arterial calcification at radiological imaging. The calcium-phosphate axis was normal and there was no other demonstrable cause for calcification. Additionally, the histological features of arterial calcification at renal biopsy paralleled the findings in infants with this disorder. The late presentation in this case is unusual and has not been previously reported. Ultrasound and CT are sensitive for calcification, and the disease should be suspected in children presenting with cardiac or respiratory manifestations and features of arterial calcification, where no metabolic cause is established. (orig.)

  12. Posterior asymmetry and idiopathic scoliosis

    CERN Document Server

    Rousie, D L; Berthoz, A

    2009-01-01

    Study design Are there neuro-anatomical abnormalities associated with idiopathic scoliosis (IS)? Posterior Basicranium (PBA) reflects cerebellum growth and contains vestibular organs, two structures suspected to be involved in scoliosis. Objective The aim of this study was to compare posterior basicranium asymmetry (PBA) in Idiopathic scoliosis (IS) and normal subjects. Method: To measure the shape of PBA in 3D, we defined an intra-cranial frame of reference based on CNS and guided by embryology of the neural tube. Measurements concerned three directions of space referred to a specific intra cranial referential. Data acquisition was performed with T2 MRI (G.E. Excite 1.5T, mode Fiesta). We explored a scoliosis group of 76 women and 20 men with a mean age of 17, 2 and a control group of 26 women and 16 men, with a mean age of 27, 7. Results: IS revealed a significant asymmetry of PBA (Pr>|t|<.0001) in 3 directions of space compared to the control group. This asymmetry was more pronounced in antero-posterior...

  13. Idiopathic Opticochiasmatic Arachnoiditis

    National Research Council Canada - National Science Library

    Lessell, Simmons; E. Grzybowski, Andrzej

    A critical review of the literature indicates that idiopathic opticochiasmatic arachnoiditis, once considered an important consideration in patients with otherwise unexplained optic atrophy, is not a...

  14. Sclerosing cholangitis: Clinicopathologic features, imaging spectrum, and systemic approach to differential diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Seo, Ni Eun [Dept. of Radiology, Research Institute of Radiological Science, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, So Yeon; Lee, Seung Soo; Byun, Jae Ho; Kim, Hyoung Jung; Kim, Jin Hee; Lee, Moon Gyu [Dept. of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul (Korea, Republic of)

    2016-02-15

    Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.

  15. Endoscopic Management of Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Barkin, Jodie A; Levy, Cynthia; Souto, Enrico O

    Primary sclerosing cholangitis (PSC) remains a rare but potentially devastating chronic, cholestatic liver disease. PSC causes obstruction of intra- and/or extra-hepatic bile ducts by inflammation and fibrosis, leading to biliary obstruction, cirrhosis and portal hypertension with all associated sequelae. The most dreaded consequence of PSC is cholangiocarcinoma, occurring in 10-20% of patients with PSC, and with population-based estimates of a 398-fold increased risk of cholangiocarcinoma in patients with PSC compared to the general population. We use the 4-D approach to endoscopic evaluation and management of PSC based on currently available evidence. After laboratory testing with liver chemistries and high-quality cross-sectional imaging with MRCP, the first D is Dominant stricture diagnosis and evaluation. Second, Dilation of strictures found during ERCP is performed using balloon dilation to as many segments as possible. Third, Dysplasia and cholangiocarcinoma diagnosis is performed by separated brushings for conventional cytology and fluorescence in situ hybridization (FISH), and consideration for direct cholangioscopy with SpyGlass™. Fourt and finally, Dosing of antibiotics is critical to prevent peri-procedural cholangitis. The aim of this review article is to explore endoscopic tools and techniques for the diagnosis and management of PSC and provide a practical approach for clinicians.

  16. Diagnosis and classification of primary sclerosing cholangitis.

    Science.gov (United States)

    Yimam, Kidist K; Bowlus, Christopher L

    2014-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of the liver and that is characterized by progressive inflammation, fibrosis, and stricturing of the intrahepatic and extrahepatic bile ducts. It is progressive in most patients and leads to cirrhosis. It is a rare disease, mostly affecting people of northern European descent, males greater than females. The diagnosis is best established by contrast cholangiography, which reveals a characteristic picture of diffuse, multifocal strictures and focal dilation of the bile ducts, leading to a beaded appearance. Inflammatory bowel disease (IBD) is present in ~75% of the patients with PSC, mostly ulcerative colitis (~85% of the cases). In addition to biliary cirrhosis, complications of PSC include dominant strictures of the bile ducts, cholangitis, cholangiocarcinoma, colon dysplasia and cancer in patients with IBD, gallbladder polyps and cancer, and hepatic osteodystrophy. The etiology of PSC is not clear, but studies are ongoing. The median survival without liver transplantation is 12 to 15 years after diagnosis. Currently there are no effective treatments except liver transplantation. Immunosuppressive medications have not been shown to be effective but antibiotics and anti-fibrotic agents seem promising. Copyright © 2014 Elsevier B.V. All rights reserved.

  17. Primary sclerosing cholangitis: diagnosis, prognosis, and management.

    Science.gov (United States)

    Singh, Siddharth; Talwalkar, Jayant A

    2013-08-01

    Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease of the liver of unclear etiology, characterized by chronic inflammation and fibrosis of bile ducts. It primarily affects middle-aged men and is associated with 4-fold increased mortality as compared with an age- and sex-matched population. Progressive biliary and hepatic damage results in portal hypertension and hepatic failure in a significant majority of patients over a 10- to 15-year period from the initial diagnosis. In addition, PSC confers a markedly increased risk of hepatobiliary cancer, including cholangiocarcinoma and gallbladder cancer, as compared with the general population, and cancer is the leading cause of mortality in patients with PSC. It is associated with inflammatory bowel disease in 70% of patients and increases the risk of colorectal cancer almost 10-fold. Despite significant research efforts in this field, the pathogenic mechanisms of PSC are still incompletely understood, although growing evidence supports the role of genetic and immunologic factors. There are no proven medical therapies that alter the natural course of the disease. Thus, liver transplantation is the only available treatment for patients with advanced PSC, with excellent outcomes in this population. Copyright © 2013 AGA Institute. Published by Elsevier Inc. All rights reserved.

  18. New Therapeutic Strategies for Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Williamson, Kate D; Chapman, Roger W

    2016-02-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, which in the majority of patients progresses to liver transplantation or death. To date, no medical treatment has been proven to be of benefit, although ursodeoxycholic acid is widely used. The etiopathogenesis of PSC is unclear, although it is associated with inflammatory bowel disease. Various hypotheses have been suggested, which have led to different therapeutic strategies. Recent studies have suggested that the microbiome may play a role in PSC, raising the possibility of efficacy of antibiotics and fecal microbiota transplantation. Gut-homing T cells may be important in the pathogenesis of PSC, and several agents are in development, targeting various receptors, integrins, and ligands on this pathway, including VAP-1, MAdCAM-1, α4β7, and CCR9. Nuclear receptor agonists such as obeticholic acid and fibrates hold promise, as do other therapies that alter bile acid composition such as norUDCA. Antifibrotic agents such as Loxl2 inhibitors are also being assessed. In conclusion, it is likely that an effective drug therapy for PSC will become available over the next decade. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  19. Primary sclerosing cholangitis - the Norwegian experience.

    Science.gov (United States)

    Schrumpf, Erik; Boberg, Kirsten Muri; Karlsen, Tom H

    2015-06-01

    Research related to primary sclerosing cholangitis (PSC) has since 1980 been a major activity at the Oslo University Hospital Rikshospitalet. The purpose of this publication is to describe the development of this research, the impact of this research on the clinical handling of the patients, and finally to describe what we believe are the most urgent, remaining problems to be solved. During the early years, our research dealt primarily with clinical aspects of the disease. The concomitant inflammatory bowel disease (IBD) seen in most patients with PSC was a major interest and we also started looking into genetic associations of PSC. Prognosis, malignancy development and treatment with special emphasis on transplantation have later been dealt with. These activities has had impact on several aspects of PSC management; when and how to diagnose PSC and variant forms of PSC, how to handle IBD in PSC and how to deal with the increased rate of malignancy? The problems remaining to be solved are many. What is the role of the gut and the gut microbiota in the development of PSC? Do the PSC patients have an underlying disturbance in the bile homeostasis? And how does the characteristic type of fibrosis in PSC develop? The genetic studies have supported a role for the adaptive immune system in the disease development, but how should this be dealt with? Importantly, the development of malignancy in PSC is still not understood, and we lack appropriate medical treatment for our patients.

  20. Subacute sclerosing panencephalitis: clinical and demographic characteristics.

    Science.gov (United States)

    Rafique, Arshad; Amjad, Nida; Chand, Prem; Zaidi, Syed Sohail Zahoor; Rana, Muhammad Suleman; Ahmed, Khalid; Ibrahim, Shahnaz

    2014-08-01

    To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE). Case series. The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. RESULTS were expressed as percentages. Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients (39.5%). All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 (51%) patients. CSF for antimeasles antibodies was positive in approximately 86% of the 40 (93%) children. EEG showed burst suppression pattern in 36 (83.7%) cases. Forty-two patients (97.6%) were discharged home in a vegetative state. SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions.

  1. Idiopathic head tremor in English bulldogs.

    Science.gov (United States)

    Guevar, Julien; De Decker, Steven; Van Ham, Luc M L; Fischer, Andrea; Volk, Holger A

    2014-02-01

    Idiopathic head tremor (IHT) syndrome is a recognized but poorly characterized movement disorder in English bulldogs (EBs). The data analyzed were collected via a detailed online questionnaire and video recordings. Thirty-eight percent of the population demonstrated IHT. The first presentation was early in life. There was no sex or neutered status predisposition. The condition disappeared with time in 50% of the cases. The direction of the head movement was vertical or horizontal. The number of episodes per day and the duration of the episodes were greatly variable. The majority of episodes occurred at rest. Most of the episodes were unpredictable. And there was no alteration of the mental status for most dogs during the episodes. Stress has been reported as a suspected trigger factor. IHT in EBs can be considered an idiopathic paroxysmal movement disorder. © 2013 Movement Disorder Society.

  2. A case of idiopathic omental hemorrhage

    Directory of Open Access Journals (Sweden)

    Toshimitsu Hosotani

    2016-05-01

    Full Text Available With the exception of trauma, intraperitoneal hemorrhage in young women is caused by the high frequency of ectopic pregnancy and ovarian bleeding. Here, we describe a case of idiopathic omental hemorrhage, which is a rare cause of intraperitoneal hemorrhage. Intraperitoneal hemorrhage was suspected in a 38-year-old Japanese woman based on contrast-enhanced computed tomography. Her last menstrual period was 23 days prior, and ovarian bleeding was considered based on bloody ascites revealed by culdocentesis. She underwent emergency surgery for hypovolemic shock. Although both ovaries were of normal size and no abnormal findings were observed, we performed a partial omentectomy because multiple clots were attached only to the greater omentum. Postoperatively, no rebleeding occurred, and she was discharged 11 days after the surgery. Because she did not have a clear history of trauma and underlying disease, idiopathic omental hemorrhage was diagnosed.

  3. Current therapies and future perspectives in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Tatli, Burak; Ekici, Bariş; Ozmen, Meral

    2012-04-01

    Subacute sclerosing panencephalitis is a progressive neurological disorder of children and young adults caused by a measles virus that became defective by persisting in the host. According to the results of clinical trials, antiviral and/or immunomodulatory therapy can slow the progression of the disease and improve life expectancy in patients. However, its long-term effects and eventual outcome remain debatable due to conflicting results and its lack of effect on the rapidly progressive form of the disease. Possible future therapies for subacute sclerosing panencephalitis are RNAi and antiapoptotic agents, which are currently in the hypothetical and experimental stages of research.

  4. Sclerosing lymphangitis of the penis: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ki Soon; Lee, Yul; Chung, Soo Young; Kim, Ki Kyung; Lee, Young Goo; Park, Heung Won; Ahn, Hae Kyung [College of Medicine, Hallym University, Chuncheon (Korea, Republic of)

    1993-03-15

    Sclerosing lymphangitis of the penis occurs as a skin-colored or dusty red, cord-like thickening of tissue at the corona or within the coronal sulcus. The thickened, elongated lesion is firm and relatively nontender. The cause is unknown: because it occurs primarily in those who are sexually very active, however, it is likely that chronic trauma plays an important etiologic role. We experienced a case of pathologically proven sclerosing lymphangitis in a 31 year-old man. Ultrasonographic finding showed circumferential cord like hypoechoic band with irregular, but well demarcated margin.

  5. Characteristic Findings of Primary Sclerosing Cholangitis on Endoscopic Retrograde Cholangiography: Which is the Most Common Finding?

    Directory of Open Access Journals (Sweden)

    Amir Houshang Mohammad Alizadeh

    2012-01-01

    Full Text Available Background Primary sclerosing cholangitis (PSC is a chronic cholestatic liver disease and one of the most common indications for liver transplantation in adults. There are conflicting data regarding characteristic findings of PSC disease on endoscopic retrograde cholangiography (ERCP. We undertook this study to clarify whether there is a specific pattern of involvement of the biliary tract in patients with PSC and to evaluate features of PSC disease on ERCP in order to be able to manage this disease better. Methods This retrospective study included 45 patients admitted to Taleghani Hospital in 2004-2010 and diagnosed to have PSC on the basis of typical cholangiographic findings in combination with clinical and laboratory data. Patients suspected to have secondary sclerosing cholangitis were excluded. Demographic and clinical data were recorded, along with cholangiographic findings and the frequency of large duct and small duct PSC. Results Forty-five patients of mean age 34.8 (range 15-66 years were included. Twenty-nine patients (64.4% had inflammatory bowel disease, and the frequency of large duct PSC and small duct PSC was 93.4% and 6.6%, respectively. The intrahepatic ducts alone were involved in 11 (24.4% patients and the extrahepatic ducts were involved in 14 (31.1%, with 17 (37.7% patients having both intrahepatic and extrahepatic PSC. Three (6.6% patients did not have bile duct involvement on ERCP, and their disease was diagnosed by liver biopsy as small duct PSC. The most common type of cholangiographic feature of intrahepatic duct involvement was type 2, found in 15 (33.3% patients, with type 3 being the most common type of extrahepatic duct involvement and detected in 16 (35.5% patients. Conclusion Our study demonstrates that the most common PSC finding on ERCP is involvement of both the extrahepatic and intrahepatic bile ducts, with small duct PSC being less common than large duct PSC.

  6. Idiopathic pulmonary hemosiderosis

    OpenAIRE

    Sunilkumar, B M; Sathishkumar, K M; Somashekhar, A R; Maiya, P P

    2010-01-01

    Recurrent or chronic pulmonary hemorrhage is rare in children. Idiopathic pulmonary hemosiderosis (IPH) manifests as hemoptysis, diffuse parenchymal infiltrates on chest radiographs and microcytic hypochromic anemia. The hemoptysis present may be mistaken for more common diseases, delaying the diagnosis and further management. Idiopathic pulmonary hemosiderosis is a disorder of unknown etiology. Treatment of IPH includes immunosuppressive drugs along with supportive measures.

  7. Animal models for primary sclerosing cholangitis.

    Science.gov (United States)

    Vierling, J M

    2001-08-01

    Since the aetiopathogenesis of primary sclerosing cholangitis (PSC) in humans remains undefined, investigators have studied a variety of animal models to gain insights into immunopathogenetic mechanisms associated with obliterative fibrous cholangitis of intra- and extra-hepatic bile ducts. To date, no animal model has been developed that exhibits all of the attributes of PSC. Rodent models instigated by bacterial cell components or colitis are promising because they may help to explain the strong association between PSC and inflammatory bowel disease (IBD). Other models of direct injury to biliary epithelia, peribiliary vascular endothelia or portal venous endothelia indicate that inflammation, chemokines and cytokines can produce diffuse sclerosis of bile ducts. Models of toxic, infectious or intra-luminal injury of the biliary tract also exhibit focal biliary sclerosis mediated by inflammation and cytokines. The histopathology of several models suggests a sequence of events beginning with secretion of proinflammatory cytokines by activated hepatic macrophages followed by peribiliary infiltration with CD4 and CD8 T cells with a T helper 1 phenotype. These results strongly suggest co-ordinated, pathogenetic roles for both the innate and adaptive immune responses. However, the stimuli that initiate and perpetuate peribiliary fibrosis remain unknown. Interestingly, several models are also associated with the development of anti-neutrophil cytoplasmic antibodies that react in a perinuclear and cytoplasmic pattern similar to that observed in patients with ulcerative colitis and/or PSC. Finally, models of extra-hepatic biliary obstruction continue to provide important information about the pathogenesis of portal fibrosis and secondary biliary cirrhosis that occurs in PSC and other diseases with obstruction of bile flow. Future studies in either existing or new animal models should advance our understanding of the pathogenesis of PSC, the major prerequisite for the

  8. Epilepsy in children with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Jović, Nebojša J

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, fatal neurodegenerative disease of childhood and early adolescence caused by defective measles virus. The initial symptoms of SSPE usually involve regression in cognitive functioning and behavior or recurrent myoclonic jerks. Seizures revealing SSPE and epilepsy during the clinical course can occur. The aim of the study was to analyze clinical and EEG characteristics of both initially occurred seizures and epilepsy which developed in the course of the disease. Retrospective study was carried out on 19 children (14 boys, 5 girls) with SSPE diagnosed and treated at our Clinic from 1995 to 2010. Seizures revealed SSPE in our patients aged from 6.5 to 11.5 years (mean 8.6 years). SSPE onset ranged from 4.5 to 16.5 years (mean 10.05). Complete vaccination was performed in nine patients. Cognitive and behavioral decline was preceeded by 6-18 months in two children with intractable focal motor seizures with secondary generalization, one child with complex partial seizures and one with atypical absences. During the clinical course of the disease epilepsy developed in 10 (52.6%) cases, including four patients with seizures as the initial SSPE sign. It occurred mainly in the first year, while in three cases seizures appeared between 1 and 5 years of the disease evolution. Myoclonus was present independently from seizures. No significant inter-group differences were found relating to the type of SSPE progression and history of epilepsy. The only child with fulminant SSPE presented with initial seizures. Favorable seizure control was achieved in 60.0% patients. Intractable epilepsy developed in four patients. Atypical SSPE presentation can include mainly focal intractable seizures. Epilepsy developed during clinical course in 52.6% cases. No significant influence was found of the history of epilepsy on the type of SSPE progression.

  9. Subacute sclerosing panencephalitis: A clinical appraisal.

    Science.gov (United States)

    Jagtap, Sujit Abajirao; Nair, M D; Kambale, Harsha J

    2013-10-01

    Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken's criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Study included 34 patients, 26 (76.5%) males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5%) followed by seizures (23.5%). Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG) showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years). Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.

  10. Epilepsy in children with subacute sclerosing panencephalitis

    Directory of Open Access Journals (Sweden)

    Jović Nebojša J.

    2013-01-01

    Full Text Available Introduction. Subacute sclerosing panencephalitis (SSPE is a rare, progressive, fatal neurodegenerative disease of childhood and early adolescence caused by defective measles virus. The initial symptoms of SSPE usually involve regression in cognitive functioning and behavior or recurrent myoclonic jerks. Seizures revealing SSPE and epilepsy during the clinical course can occur. Objective. The aim of the study was to analyze clinical and EEG characteristics of both initially occurred seizures and epilepsy which developed in the course of the disease. Methods. Retrospective study was carried out on 19 children (14 boys, 5 girls with SSPE diagnosed and treated at our Clinic from 1995 to 2010. Seizures revealed SSPE in our patients aged from 6.5 to 11.5 years (mean 8.6 years. Results. SSPE onset ranged from 4.5 to 16.5 years (mean 10.05. Complete vaccination was performed in nine patients. Cognitive and behavioral decline was preceeded by 6-18 months in two children with intractable focal motor seizures with secondary generalization, one child with complex partial seizures and one with atypical absences. During the clinical course of the disease epilepsy developed in 10 (52.6% cases, including four patients with seizures as the initial SSPE sign. It occurred mainly in the first year, while in three cases seizures appeared between 1 and 5 years of the disease evolution. Myoclonus was present independently from seizures. No significant inter-group differences were found relating to the type of SSPE progression and history of epilepsy. The only child with fulminant SSPE presented with initial seizures. Favorable seizure control was achieved in 60.0% patients. Intractable epilepsy developed in four patients. Conclusion. Atypical SSPE presentation can include mainly focal intractable seizures. Epilepsy developed during clinical course in 52.6% cases. No significant influence was found of the history of epilepsy on the type of SSPE progression.

  11. Subacute sclerosing panencephalitis: A clinical appraisal

    Directory of Open Access Journals (Sweden)

    Sujit Abajirao Abajirao

    2013-01-01

    Full Text Available Introduction: Subacute sclerosing panencephalitis (SSPE is a rare chronic, progressive encephalitis affecting primarily children and young adults, caused by a persistent infection of immune resistant measles virus. The aim of the present study is to describe the clinical profile and natural history of patients with SSPE. Methods: We collected data of patients with SSPE during 2004-2010 who fulfilled Dyken′s criteria. We analyzed demographical, clinical, electrophysiological, and imaging features. Results: Study included 34 patients, 26 (76.5% males with age of onset from 3 to 31 years. Twenty one patients were below 15 years of age formed childhood SSPE and 13 above 15 years of age constituted adult onset group. 85.3% had low-socioeconomic status. Eleven received measles vaccination and seven were unvaccinated. 59.9% patients had measles history. Most common presenting symptom was scholastic backwardness (52.5% followed by seizures (23.5%. Three patients each had cortical blindness, macular degeneration, decreased visual acuity, and optic atrophy. Electroencephalographic (EEG showed long interval periodic complexes and cerebrospinal fluid anti-measles antibody was positive in all. Magnetic resonance imaging was done in 70.5% with was abnormal in 52.5%. Mean incubation period of SSPE after measles was 9.6 years. The follow-up duration was 1-10 years, (average of 2 years. Only one patient died from available data of follow-up, 9 were stable and 10 deteriorated in the form of progression of staging. Conclusion: SSPE is common in low-socioeconomic status. The profile of adult onset did not differ from childhood onset SSPE, except for a longer interval between measles infection and presence of the ophthalmic symptom as presenting feature in adult onset group.

  12. Idiopathic pulmonary hemosiderosis complicated by Down syndrome.

    Science.gov (United States)

    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. © 2015 Japan Pediatric Society.

  13. Genome-Wide Association Analysis in Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Karlsen, Tom H.; Franke, Andre; Melum, Espen; Kaser, Arthur; Hov, Johannes Roksund; Balschun, Tobias; Lie, Benedicte A.; Bergquist, Annika; Schramm, Christoph; Weismueller, Tobias J.; Gotthardt, Daniel; Rust, Christian; Philipp, Eva E. R.; Fritz, Teresa; Henckaerts, Liesbet; Weersma, Rinse K.; Stokkers, Pieter; Ponsioen, Cyriel Y.; Wijmenga, Cisca; Sterneck, Martina; Nothnagel, Michael; Hampe, Jochen; Teufel, Andreas; Runz, Heiko; Rosenstiel, Philip; Stiehl, Adolf; Vermeire, Severine; Beuers, Ulrich; Manns, Michael P.; Schrumpf, Erik; Boberg, Kirsten Muri; Schreiber, Stefan

    BACKGROUND & AIMS: We aimed to characterize the genetic susceptibility to primary sclerosing cholangitis (PSC) by means of a genome-wide association analysis of single nucleotide polymorphism (SNP) markers. METHODS: A total of 443,816 SNPs on the Affymetrix SNP Array 5.0 (Affymetrix, Santa Clara,

  14. Sclerosing Cholangitis and Primary Biliary Cirrhosis—a Disease Spectrum?

    Science.gov (United States)

    Fee, Henry J.; Gewirtz, Harold; Schiller, Juan; Longmire, William P.

    1977-01-01

    Sclerosing diseases of the biliary system encompass a spectrum ranging from primary sclerosing cholangitis (usually of the extrahepatic biliary tree) to primary biliary cirrhosis of the intrahepatic bile canaliculi. In a study of 35 patients with primary intra- and extrahepatic biliary sclerosis, age of onset, sex distribution, symptomatology, associated diseases, radiographic abnormalities and chemical profile were considered. The difficulty of differentiating sclerosing cholangitis and biliary cirrhosis from other causes of obstructive jaundice preoperatively was stressed, in addition to points of differential clinical and laboratory findings. The etiology of these entities as well as the possibility that they represent variant clinical manifestations of the same disease process were also considered. Mechanical and pharmacological treatment alternatives that were attempted included drainage procedures, the easiest and most widely used of which was the T-tube. However, this could prove to be a source of infection and should therefore be removed early, inasmuch as cholangitis represents a major cause of morbidity. Steroids have been used with varying effectiveness; subjective improvement was generally attained, although objective improvement has been difficult to document. When choleuretics and cholestyramine were administered, we noted significant palliation. Antibiotics were reserved for treatment of cholangitis. Until the underlying etiology of this rare malignant sclerosing process is found, only symptomatic treatment can be offered. PMID:921353

  15. Biomarkers for disease progression of primary sclerosing cholangitis

    NARCIS (Netherlands)

    de Vries, Elisabeth M. G.; Beuers, Ulrich; Ponsioen, Cyriel Y.

    2015-01-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin. There is no medical treatment of proven benefit on survival; once patients have progressed to end-stage liver disease, the only treatment option is liver transplantation. Over the last years, some progress

  16. Resolving MRI abnormalities with progression of subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Winer, J.B. (Saint Mary' s Hospital, Birmingham (UK)); Pires, M.; Kermode, A.; Ginsberg, L. (National Hospital for Nervous Diseases, Birmingham (UK)); Rossor, M. (Saint Mary' s Hospital, Birmingham (UK) National Hospital for Nervous Diseases, Birmingham (UK))

    1991-04-01

    Serial MRI was performed on a 15 year old girl with Subacute Sclerosing Pan-encephalitis (SSPE). After a period of remission she entered a phase of progressive deterioration. A repeat MRI showed significant resolution of the previous abnormalities. Her pathology and MRI scans are discussed.

  17. Epilepsia partialis continua in children with fulminant subacute sclerosing panencephalitis.

    Science.gov (United States)

    Kravljanac, Ruzica; Jovic, Nebojsa; Djuric, Milena; Nikolic, Ljubica

    2011-12-01

    Various inflammatory diseases of central nervous system, including subacute sclerosing panencephalitis, could cause epilepsia partialis continua. Two boys with epilepsia partialis continua with onset in terminal phase of atypical subacute sclerosing panencephalitis have been reported. Children were not vaccinated against measles, and the second case had history of measles at an early age. In both cases, the onset of subacute sclerosing panencephalitis was characterized by altered behavior and cognitive decline with very fast mental and neurological deterioration. One boy was suffering from complex partial seizures and myoclonic jerks synchronous with periodic electroencephalographic pattern. Diagnosis was proved by increased titers of antimeasles antibodies in both serum and cerebrospinal fluid. In terminal phase of the disease, epilepsia partialis continua of localized group of the muscles was diagnosed, with good response to intravenous infusion of midazolam. Surface electroencephalographic recordings during epilepsia partialis continua did not show the epileptic discharges. During the terminal phase of the disease, no other type of seizures and movement disorders were recognized, except epilepsia partialis continua. In spite of the treatment, period from the onset of disease to death lasted less than 3 months, suggesting very fulminant course of subacute sclerosing panencephalitis.

  18. High-Dose Intravenous Ribavirin Therapy for Subacute Sclerosing Panencephalitis

    Science.gov (United States)

    Hosoya, Mitsuaki; Shigeta, Shiro; Mori, Shuichi; Tomoda, Akemi; Shiraishi, Seiji; Miike, Teruhisa; Suzuki, Hitoshi

    2001-01-01

    Two patients with subacute sclerosing panencephalitis (SSPE) were treated safely and effectively with high doses of intravenous ribavirin combined with intraventricular alpha interferon. The ribavirin concentrations maintained in the serum and cerebrospinal fluid were higher than those which inhibit SSPE virus replication in vitro and in vivo. PMID:11181386

  19. A close look at EEG in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Demir, Nurhak; Cokar, Ozlem; Bolukbasi, Feray; Demirbilek, Veysi; Yapici, Zuhal; Yalcinkaya, Cengiz; Direskeneli, Guher Saruhan; Yentur, Sibel; Onal, Emel; Yilmaz, Gulden; Dervent, Aysin

    2013-08-01

    To define atypical clinical and EEG features of patients with subacute sclerosing panencephalitis that may require an overview of differential diagnosis. A total of 66 EEGs belonging to 53 (17 females and 36 males) consecutive patients with serologically confirmed subacute sclerosing panencephalitis were included in this study. Patient files and EEG data were evaluated retrospectively. EEGs included in the study were sleep-waking EEGs and/or sleep-waking video-EEG records with at least 2 hours duration. Cranial MRIs of the patients taken 2 months before or after the EEG records were included. Age range at the onset of the disease was 15 to 192 months (mean age: 80.02 months). Epilepsy was diagnosed in 21 (43%) patients. Among epileptic seizures excluding myoclonic jerks, generalized tonic-clonic type constituted the majority (58%). Tonic seizures were documented during the video-EEG recordings in four patients. Epileptogenic activities were found in 56 (83%) EEG recordings. They were localized mainly in frontal (58%), posterior temporal, parietal, occipital (26%), and centrotemporal (8%) regions. Multiple foci were detected in 26 recordings (39%). Epileptiform activities in the 39 (59%) EEGs appeared as unilateral or bilateral diffuse paroxysmal discharges. Recognition of uncommon clinical and EEG findings of subacute sclerosing panencephalitis, especially in countries where subacute sclerosing panencephalitis has not been eliminated yet, could be helpful in prevention of misdiagnosis and delay in the management of improvable conditions.

  20. Primary sclerosing cholangitis and disease distribution in inflammatory bowel disease.

    LENUS (Irish Health Repository)

    O'Toole, Aoibhlinn

    2012-04-01

    The relationship between site of intestinal inflammation and primary sclerosing cholangitis (PSC) development in inflammatory bowel disease (IBD) has not been studied extensively, but may be important in understanding the pathogenesis of PSC. We aimed to determine patterns of disease distribution in IBD patients with and without PSC.

  1. Pediatric glaucoma suspects

    Directory of Open Access Journals (Sweden)

    Kooner K

    2014-06-01

    Full Text Available Karanjit Kooner,1 Matthew Harrison,1 Zohra Prasla,1 Mohannad Albdour,1 Beverley Adams-Huet21Department of Ophthalmology, 2Department of Clinical Sciences, Division of Biostatistics, University of Texas Southwestern Medical Center, Dallas, TX, USAPurpose: To report demographic and ocular features of pediatric glaucoma suspects in an ethnically diverse population of North Central Texas.Design: Retrospective cross-sectional chart review.Participants: Subjects included 75 (136 eyes pediatric glaucoma suspects. Patients with one or more of the following risk factors were included: cup-to disc (C/D ratio of ≥0.6; intraocular pressure (IOP ≥21 mmHg; family history of glaucoma; congenital glaucoma in the opposite eye; history of blunt trauma to either eye; and presence of either Sturge–Weber or Axenfeld–Rieger syndrome, or oculodermal melanocytosis.Methods: Data were extracted from electronic patient medical records. Patient records with incomplete data were excluded. The main outcome measures were race, sex, age, IOP, C/D, family history of glaucoma; and glaucoma treatment.Results: Subjects included 28 (37.3% Hispanics, 20 (26.6% African Americans, 20 (26.6% Caucasians, and seven (9.3% Asians. Forty (53.3% of the patients were male. Suspicious optic disc was seen in 57 (76%; elevated IOP in 25 (33.3%; presence of family history in 13 (17.3%, and Sturge–Weber syndrome in nine (12% patients. The average C/D ratio was 0.58±0.2. The C/D ratios of African American (0.65±0.2, Hispanic (0.63±0.2, and Asian (0.62±0.15 patients were significantly greater than those of Caucasians (0.43±0.18; P=0.0004, 0.0003, and 0.0139, respectively. Caucasian patients were the youngest (7.9±4.8 years. Eleven cases (14.7% required medication.Conclusion: Thirty-three point seven percent of patients seen in the glaucoma clinic were glaucoma suspects. The most common risk factors for suspected glaucoma were suspicious optic discs, elevated IOP, and family history

  2. Idiopathic opticochiasmatic arachnoiditis.

    Science.gov (United States)

    Lessell, Simmons; E Grzybowski, Andrzej

    2014-09-01

    : A critical review of the literature indicates that idiopathic opticochiasmatic arachnoiditis, once considered an important consideration in patients with otherwise unexplained optic atrophy, is not a valid disease entity.

  3. Juvenile Idiopathic Arthritis

    Science.gov (United States)

    ... Teens With Diabetes Protecting Your Online Identity and Reputation ADHD Medicines Juvenile Idiopathic Arthritis (JIA) KidsHealth > For ... you find foods that have a higher nutritional value to make up for having a poor appetite. ...

  4. Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Safak Ekinci

    2014-06-01

    Full Text Available Scoliosis is called idiopathic when no other underlying disease can be identified. The etiology of adolescent idiopathic scoliosis (AIS is still unknown despite many years of research effort. Theories on AIS's etiology have included mechanical, hormonal, metabolic, neuromuscular, growth, and genetic abnormalities. Skeletally immature patients with adolescent idiopathic scoliosis are at risk of curve progression. The adolescent onset of severe idiopathic scoliosis has traditionally been evaluated using standing posteroanterior radiographs of the full spine to assess lateral curvature with the Cobb method. Scoliosis in children of school age and above primarily occurs in girls. The therapeutic goal in children is to prevent progression. In children, scoliosis of 20 and deg; or more should be treated with a brace, and scoliosis of 45 and deg; or more with surgery. [Arch Clin Exp Surg 2014; 3(3.000: 174-182

  5. Increased risk of colorectal cancer and dysplasia in patients with Crohn's colitis and primary sclerosing cholangitis.

    Science.gov (United States)

    Lindström, Lina; Lapidus, Annika; Ost, Ake; Bergquist, Annika

    2011-11-01

    Almost 10% of all patients with primary sclerosing cholangitis receive a diagnosis of Crohn's disease. Clinical characteristics and the risk of colon cancer or dysplasia in Crohn's disease and primary sclerosing cholangitis are less well examined than in ulcerative colitis. This study aimed to describe the clinical characteristics and risk of colorectal dysplasia and cancer in Crohn's disease in patients with primary sclerosing cholangitis. This is a cohort study of all patients diagnosed with primary sclerosing cholangitis and colorectal Crohn's disease at Karolinska University Hospital, Huddinge, 1978 to 2006. Each patient was matched for age and the onset of Crohn's disease to 2 controls with colorectal Crohn's disease without liver disease. This study was conducted at a tertiary referral center. Twenty-eight patients (61% male) with primary sclerosing cholangitis and Crohn's disease and 46 patients (50% male) with Crohn's disease alone were studied. Clinical and endoscopic data were retrieved from medical records. Colonic biopsies from patients with primary sclerosing cholangitis were re-reviewed. The primary outcome measured was the proportion of patients developing colorectal cancer. Colorectal cancer or dysplasia developed in 9 patients with primary sclerosing cholangitis and in 3 controls. Patients with primary sclerosing cholangitis were more likely to develop colorectal dysplasia or cancer than controls (OR 6.78; 95% CI (1.65-27.9); P = .016). In patients with primary sclerosing cholangitis compared with controls, perianal fistulas occurred in 3% vs 33% (P = .003), bowel strictures occurred in 7% vs 30% (P = .03), and bowel surgery was performed in 18% vs 46% (P = .01). Histological granulomas were seen in 29% of the patients with primary sclerosing cholangitis compared with 43% in controls (P = not significant). This study was limited by its retrospective nature and the limited cohort. Primary sclerosing cholangitis is a risk factor for the development

  6. Subacute Sclerosing Panencephalitis in a Child with Human Immunodeficiency Virus Co-Infection.

    Science.gov (United States)

    Maurya, Pradeep Kumar; Thakkar, Mayur Deepak; Kulshreshtha, Dinkar; Singh, Ajai Kumar; Thacker, Anup Kumar

    2016-12-01

    Subacute sclerosing panencephalitis is a fatal infectious disease of childhood caused by persistence of the measles virus in the brain. The effect of human immunodeficiency virus (HIV) co-infection on subacute sclerosing panencephalitis remains elusive and rare. We report a child who developed subacute sclerosing panencephalitis following a short latency period and a rapidly progressive course with HIV co-infection. © 2016 Marshfield Clinic.

  7. MRCP in primary sclerosing cholangitis; MRCP bei primaerer sklerosierender Cholangitis

    Energy Technology Data Exchange (ETDEWEB)

    Weber, C.; Krupski, G.; Lorenzen, J.; Adam, G. [Universitaetsklinikum Hamburg-Eppendorf (Germany). Klinik und Poliklinik fuer Diagnostische und Interventionelle Radiologie; Grotelueschen, R.; Rogiers, X. [Universitaetsklinikum Hamburg-Eppendorf (Germany). Abt. fuer Hepatobilaere Chirurgie; Seitz, U. [Universitaetsklinikum Hamburg-Eppendorf (Germany). Klinik fuer Interdisziplinaere Endoskopie

    2003-02-01

    Purpose: Evaluation of MR-cholangiopancreaticography (MRCP) for the diagnosis of primary sclerosing cholangitis (PSC) in correlation with endoscopic retrograde cholangiopancreaticography (ERCP) and in comparison to the diagnostic accuracy of various T2-weighted sequences. Methods and Materials: Fifty-five patients (34 males, 21 females: mean age 40 years, range 16 to 65 years) with suspected PSC were examined in a 1.5 T MR unit (Magnetom Vision, Siemens, Erlangen), using breath-hold transverse and coronal HASTE, paracoronal RARE and thin-sliced HASTE (TS-HASTE) sequences. Applying a five-point-scale, two blinded investigators assessed the image quality for ROC analysis. Morphologic criteria of PSC were documented and correlated with ERCP, which served as the gold standard, and sensitivity, specificity and diagnostic accuracy were calculated. Results: PSC was confirmed in 40 of 55 patients (ERCP 55 of 55, liver biopsy 37 of 55), with concomitant chronic ulcerative colitis in 27 and Crohn's disease in 6 of the 40 patients. Qualitative analysis of the image quality showed no significant difference between RARE, HASTE and thin-sliced HASTE sequences (3.4/3.5/3.2). The RARE sequence had the highest sensitivity (97%), specificity (64%) and accuracy (84%) for the detection of PSC. The difference between HASTE and thin-sliced HASTE was statistically significant (p<0.01). Of the 40 patients with confirmed PSC, 29 were followed by MRI and 3 underwent a liver transplantation within the follow-up period. A Klatskin tumor, which was misdiagnosed by MRCP, was diagnosed by brush biopsy in 1 of the 40 patients. Interobserver variability was adequate to good (kappa 0.4 to 0.7), depending on the chosen sequence. (orig.) [German] Ziel: Wertigkeit der MRCP bei der Diagnostik der primaeren sklerosierenden Cholangitis (PSC) in Korrelation zur endoskopischen retrograden Cholangiopankreatikographie (ERCP) und Vergleich der diagnostischen Treffsicherheit verschiedener T{sub 2

  8. Controversies in the management of primary sclerosing cholangitis

    Science.gov (United States)

    Nayagam, Jeremy S; Pereira, Stephen P; Devlin, John; Harrison, Phillip M; Joshi, Deepak

    2016-01-01

    Primary sclerosing cholangitis (PSC) remains a rare but significant disease, which affects mainly young males in association with inflammatory bowel disease. There have been few advances in the understanding of the pathogenesis of the condition and no therapeutics with proven mortality benefit aside from liver transplantation. There remain areas of controversy in the management of PSC which include the differentiation from other cholangiopathies, in particular immunoglobulin G4 related sclerosing cholangitis, the management of dominant biliary strictures, and the role of ursodeoxycholic acid. In addition, the timing of liver transplantation in PSC remains difficult to predict with standard liver severity scores. In this review, we address these controversies and highlight the latest evidence base in the management of PSC. PMID:26925200

  9. Characterization of animal models for primary sclerosing cholangitis (PSC).

    Science.gov (United States)

    Fickert, Peter; Pollheimer, Marion J; Beuers, Ulrich; Lackner, Carolin; Hirschfield, Gideon; Housset, Chantal; Keitel, Verena; Schramm, Christoph; Marschall, Hanns-Ulrich; Karlsen, Tom H; Melum, Espen; Kaser, Arthur; Eksteen, Bertus; Strazzabosco, Mario; Manns, Michael; Trauner, Michael

    2014-06-01

    Primary sclerosing cholangitis (PSC) is a chronic cholangiopathy characterized by biliary fibrosis, development of cholestasis and end stage liver disease, high risk of malignancy, and frequent need for liver transplantation. The poor understanding of its pathogenesis is also reflected in the lack of effective medical treatment. Well-characterized animal models are utterly needed to develop novel pathogenetic concepts and study new treatment strategies. Currently there is no consensus on how to evaluate and characterize potential PSC models, which makes direct comparison of experimental results and effective exchange of study material between research groups difficult. The International Primary Sclerosing Cholangitis Study Group (IPSCSG) has therefore summarized these key issues in a position paper proposing standard requirements for the study of animal models of PSC. Copyright © 2014 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

  10. Sclerosing stromal tumor of the ovary: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Hyun Koo; Koh, Byung Hee; Rhim, Hyun Chul; Cho, On Koo; Kim, Yong Soo; Hahm, Chang Kok [School of Medicine, Hanyang Univ., Seoul (Korea, Republic of)

    2002-07-01

    Sclerosing stromal tumor of the ovary is a rare benign neoplasm, with distinctive clinical and pathologic features. It occurs predominantly in females during the second and third decades of life. Histologically, it is composed of cellular and acellular collagenized areas, and edematous stromal areas, and at ultrasonography and computed tomography is seen as a distinctive mixed solid and cystic mass lesion. We report a case of sclerosing stromal tumor of the ovary in a 15-year-old girl with a history of menorrhagia since menarche. Ultrasonography revealed the tumor as a well-defined, lobulated, heterogenous echogenic pelvic mass, while at CT, a huge pelvic mass 9 x 9 x 10 cm in size, was seen. This comprised a well-enhanced internal solid portion, a capsule, septa, and a non-enhanced cystic portion.

  11. Diagnosis, Differential Diagnosis, and Epidemiology of Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Ponsioen, Cyriel Y

    2015-01-01

    According to recent guidelines, primary sclerosing cholangitis (PSC) is diagnosed when a patient has a cholestatic liver enzyme profile, characteristic bile duct changes on imaging, and when secondary causes of sclerosing cholangitis are excluded. In patients with a clinical suspicion but normal cholangiography, a liver biopsy is indicated to establish a diagnosis of small duct PSC. Several other disease entities such as IgG4-associated cholangitis (IAC), cholangiocarcinoma (CCA), and secondary causes of sclerosing cholangitis such as choledocholithiasis, AIDS-cholangiopathy, ischemia, surgical bile duct trauma, or mast cell cholangiopathy can mimic PSC. IAC can be differentiated from PSC by applying the HISORt criteria including the serum IgG4 level. In cases where serum IgG4 is less than 2 × ULN, the ratio of IgG4/IgG1 >0.24 is indicative for IAC. Choledocholithiasis with recurrent cholangitis as a cause of sclerosing cholangitis can pose a conundrum, since PSC itself is associated with an increased prevalence of gallstones. The epidemiology of PSC worldwide has been poorly described. Incidence and prevalence rates vary from 0-1.3 and 0-16.2 per 100,000 inhabitants respectively. However, these figures are not based on population-based cohorts. A recent large population-based cohort from the Netherlands reported an incidence of 0.5 and a prevalence of 6/100,000. Approximately 10% fulfil the criteria for small duct PSC. At diagnosis of PSC, concurrent inflammatory bowel disease (IBD), primarily ulcerative colitis or Crohn's colitis is present in 50%, but increasing to 80%, 10 years or more after diagnosis. Conversely, 3% of IBD patients will develop PSC. PSC predisposes to malignancy. The estimated cumulative risk of developing CCA after 30 years is 20%. For colorectal carcinoma in PSC/colitis patients, the estimated cumulative risk at 30 years is 13%. © 2015 S. Karger AG, Basel.

  12. Visual deterioration as presentation of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Johnston, H M; Wise, G A; Henry, J G

    1980-01-01

    A 7-year-old boy presented with deteriorating vision and macular degenerative changes. A month later he had developed unusual behaviour and increasing forgetfulness. An electroencephalogram showing periodic complexes, and high measles complement-fixation titres in the cerebrospinal fluid and blood, confirmed the diagnosis of subacute sclerosing panencephalitis. Four months after the onset of visual symptoms he started having myoclonic jerks. Images Fig. 1 Fig. 2 PMID:7436466

  13. Subacute sclerosing panencephalitis. Changes on CT scan during acute relapse

    Energy Technology Data Exchange (ETDEWEB)

    Modi, G.; Bill, P. (Wentworth Hospital, Durban (South Africa). Dept. of Medicine); Campbell, H. (Wentworth Hospital, Durban (South Africa). Dept. of Radiology)

    1989-11-01

    A 19-year-old female patient presented in an acute state of akinetic mutism. Serological analysis of serum and cerebrospinal fluid demonstrated the presence of antibodies to measles virus. CT scan carried out during this acute phase of relapse demonstrated white matter enhancement affecting the cortical white matter of the frontal lobes and corpus callosum. These features indicate that active demyelination occurs during acute relapse in subacute sclerosing panencephalitis (SSPE) and suggest that immunotherapy should be considered during this acute phase. (orig.).

  14. Antibodies to borna disease virus in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Güngör, Serdal; Anlar, Banu; Turan, Nuri; Yilmaz, Hüseyin; Helps, Chris R; Harbour, Dave A

    2005-09-01

    Mechanisms causing persistence and reactivation of measles virus in subacute sclerosing panencephalitis (SSPE) are unknown. Borna disease virus (BDV) frequently causes latent or persistent infection in the nervous system. We investigated a possible association of these viruses in SSPE. Although BDV seropositivity was similar in SSPE and control groups, SSPE patients with high antibodies to BDV had earlier and more rapid disease. The findings suggest that BDV might be involved in the course, but not in the etiopathogenesis, of SSPE.

  15. Trigger mechanisms of secondary sclerosing cholangitis in critically ill patients

    OpenAIRE

    Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Hetzer, Roland; Schaffartzik, Walter; Tryba, Michael; Neuhaus, Peter; Seehofer, Daniel

    2015-01-01

    Introduction In recent years the development of secondary sclerosing cholangitis in critically ill patients (SSC-CIP) has increasingly been perceived as a separate disease entity. About possible trigger mechanisms of SSC-CIP has been speculated, systematic investigations on this issue are still lacking. The purpose of this study was to evaluate the prevalence and influence of promoting factors. Methods Temporality, consistency and biological plausibility are essential prerequisites for causal...

  16. Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: an Update on MR Imaging Findings with Recent Developments.

    Science.gov (United States)

    Kovač, Jelena Djokić; Weber, Marc-André

    2016-12-01

    Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are the most common immune-mediated chronic cholestatic liver diseases leading to cirrhosis and liver failure. Although magnetic resonance imaging (MRI) is not a necessary procedure for the diagnosis of PBC, MRI is recommended for monitoring disease progression and early detection of complications. Even though liver cirrhosis subtypes have similar MR imaging features, there are some findings which could indicate PBC, such as the periportal halo sign. Additionally, MRI using diffusion-weighted imaging with apparent diffusion coefficient measurements provides non-invasive assessment of the stage of liver fibrosis. The role of cholangiography is crucial for the diagnosis of PSC. Since endoscopic retrograde cholangiography is an invasive procedure with occasional post-procedural complications, the latest guidelines suggest magnetic resonance cholangiography as a reference procedure for evaluation of patients suspected with PSC. Characteristic magnetic resonance cholangiography findings include multiple segmental strictures with slightly dilated ducts among them, usually on both intrahepatic and extrahepatic bile ducts. Furthermore, magnetic resonance cholangiography is useful in the follow-up of these patients, allowing for timely diagnosis of complications such as cholangiocellular carcinoma. With the exception of ursodeoxycholic acid, which slows the progression of PBC, the only curative treatment for both PSC and PBC is still liver transplantation. However, recurrent disease occurs in some patients indicating the need for development of new more effective therapies.

  17. Cerebrospinal fluid nitric oxide levels in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Yilmaz, Deniz; Yüksel, Deniz; Senbil, Nesrin; Eminzade, Sude; Kilinç, Kamer; Anlar, Banu; Gürer, Yavuz

    2009-09-01

    Oxidative damage plays a role in neurodegenerative diseases. Levels of cerebrospinal fluid nitrite and nitrate levels (oxidation products that provide an indirect estimation of nitric oxide) were investigated in relation to clinical and laboratory features in subacute sclerosing panencephalitis (n = 47) and age-matched control (n = 43) groups. Significantly decreased levels of nitrite (median, 4.91 micromol/L) and nitrate (median, 6.14 micromol/L) were found in the patients. Nitrite and nitrate levels did not correlate with clinical or laboratory findings, except for presence of myoclonus. Cerebrospinal fluid nitrite levels of subacute sclerosing panencephalitis patients without myoclonic jerks were significantly higher than in those with myoclonus (median, 15.63 vs 4.34 micromol/L, respectively). The higher levels of nitrite in these patients can be explained by short disease duration and early stages of disease. Nitrate levels in subacute sclerosing panencephalitis patients with myoclonus (median, 9.26 micromol/L) were higher than in those without myoclonus (median, 4.25 micromol/L). Microbleeding resulting in conversion of nitrite to nitrate and increased production of superoxide can be suggested as possible mechanisms underlying these findings.

  18. The hundredth case of sclerosing epithelioid fibrosarcoma (SEF).

    Science.gov (United States)

    Monarca, Cristiano; Fino, Pasquale; Rizzo, Maria Ida; Palmieri, Annapina; Tarallo, Mauro; Scuderi, Nicolò

    2013-01-01

    Sclerosing Epithelioid Fibrosarcoma (SEF) is a rare and distinct variant of low grade fibrosarcoma, found mainly in deep soft tissue of adult extremities. We report a case of Sclerosing Epithelioid Fibrosarcoma of soft tissue, which developed in a 69-year-old woman who presented a tumor involving the fourth finger of her right foot and which has not progressed much in size during the three months prior the surgical excision. Our patient is the hundredth case of Sclerosing Epithelioid Fibrosarcoma reported in literature since 1995, thus confirming the rarity of the tumor. Our experience showed that it is important to make an early diagnosis, in consideration of the clinical aggressiveness of this cancer. Another important aspect concerns the postoperative follow-up. The monitoring of PET-CT technique , although not standardized, could become part of proceedings of therapy and follow-up of tumor, thus allowing oncological radicality and avoid large amputations. To date, 24 months after diagnosis of SEF, our patient feels well, attends our outpatient clinic regularly and shows no evidence of relapse and/or metastasis.

  19. Biliary phosphatidylcholine and lysophosphatidylcholine profiles in sclerosing cholangitis.

    Science.gov (United States)

    Gauss, Annika; Ehehalt, Robert; Lehmann, Wolf-Dieter; Erben, Gerhard; Weiss, Karl-Heinz; Schaefer, Yvonne; Kloeters-Plachky, Petra; Stiehl, Adolf; Stremmel, Wolfgang; Sauer, Peter; Gotthardt, Daniel Nils

    2013-09-07

    To analyze phospholipid profiles in intrahepatic bile from patients with primary sclerosing cholangitis (PSC) and secondary sclerosing cholangitis (SSC). Intrahepatic bile specimens collected via endoscopic retrograde cholangiography from 41 patients were analyzed. Fourteen of these patients were diagnosed with PSC, 10 with SSC, 11 with choledocholithiasis or no identifiable biliary disease, and 6 with cholangiocellular carcinoma (CCC). Bile acid, cholesterol, protein, and bilirubin contents as well as pancreas lipase activity in bile were determined by biochemical methods. Phosphatidylcholine (PC) and lysophosphatidylcholine (LPC) species were quantified using nano-electrospray ionization tandem mass spectrometry. Bile from all the examined patient groups showed a remarkably similar PC and LPC species composition, with only minor statistical differences. Total biliary PC concentrations were highest in controls (8030 ± 1843 μmol/L) and lowest in patients with CCC (1969 ± 981 μmol/L) (P = 0.005, controls vs SSC and CCC, respectively, P < 0.05). LPC contents in bile were overall low (4.2% ± 1.8%). Biliary LPC/PC ratios and ratios of biliary PC to bilirubin, PC to cholesterol, PC to protein, and PC to bile acids showed no intergroup differences. PC and LPC profiles being similar in patients with or without sclerosing cholangitis, these phospholipids are likely not of major pathogenetic importance in this disease group.

  20. [Subacute sclerosing panencephalitis cases diagnosed by increased CSF/serum measles antibody indices].

    Science.gov (United States)

    Samlıoğlu, Pınar; Unalp, Aycan; Gökçay, Ahmet; Altuğlu, Imre; Oztürk, Aysel; Zeytinoğlu, Ayşın

    2012-10-01

    Subacute sclerosing panencephalitis (SSPE) caused by persistent defective measles virus strains, is a progressive neurological disorder of children and adolescents. The aim of this letter was to share the data from SSPE-suspected cases who were definitely diagnosed by the detection of increased antibody index in serum and cerebrospinal fluid (CSF) samples. A total of 11 patients (mean age: 14.3 years) with suspected SSPE between February 2006 to August 2008, were included in the study. Simultaneously obtained serum and CSF samples from patients were analyzed in terms of albumin, total IgG and measles-specific IgG levels (Measles Virus IgG ELISA for CSF Diagnostics, Euroimmun, Germany). The value of CSQrel (relative CSF/serum quotient) ≥ 1.5 was accepted indicative for intrathecal measles antibody synthesis. Seven (63.6%) of the 11 patients' diagnosis were confirmed with the demonstration of elevated CSF/serum indices (CSQrel range: 2.3-36.9; mean: 12.9). Mean age of those seven cases was 12.3 years (age range: 7-21) and four of them were male. The history of patients with high antibody indices indicated that three of four patients who had measles infection had not been vaccinated against measles. These three unvaccinated patients had measles infection at 3rd, 8th and 30th months of age, respectively, and the period of SSPE development were 15, 6 and 4.5 years, respectively. With this letter we would like to emphasize once more that effective measles vaccination is the only way for the prevention of measles and SSPE and the demonstration of increased measles antibody index in simultaneously obtained serum and CSF samples is crucial for the diagnosis of SSPE.

  1. Technology and the Glaucoma Suspect

    National Research Council Canada - National Science Library

    Blumberg, Dana M; De Moraes, Carlos Gustavo; Liebmann, Jeffrey M; Garg, Reena; Chen, Cynthia; Theventhiran, Alex; Hood, Donald C

    2016-01-01

    ...), stereoscopic disc photographs, and automated perimetry as assessed by a group of glaucoma specialists in differentiating individuals with early glaucoma from suspects. Forty-six eyes (46 patients...

  2. Understanding idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Markey, Keira A; Mollan, Susan P; Jensen, Rigmor H

    2016-01-01

    Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus...... of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT) reported beneficial effects of acetazolamide in patients with mild visual loss. Studies have also established weight loss as an effective disease-modifying treatment, and further clinical trials to investigate new treatments are underway....... The incidence of idiopathic intracranial hypertension is expected to increase as rates of obesity increase; efforts to reduce diagnostic delays and identify new, effective approaches to treatment will be key to meeting the needs of a growing number of patients....

  3. Sclerosing polycystic adenosis of the salivary gland: a report of 16 cases.

    NARCIS (Netherlands)

    Gnepp, D.R.; Wang, L.J.; Brandwein-Gensler, M.; Slootweg, P.J.; Gill, M.; Hille, J.

    2006-01-01

    Sclerosing polycystic adenosis is a recently described, extremely rare, reactive, sclerosing, inflammatory process somewhat similar to fibrocystic changes and adenosis tumor of the breast. To date, there have been 22 cases described in the literature. Because of the infrequency of this lesion, we

  4. Sclerotic cemental masses of the jaws (so-called chronic sclerosing osteomyelitis, sclerosing osteitis, multiple enostosis, and gigantiform cementoma.

    Science.gov (United States)

    Waldron, C A; Giansanti, J S; Browand, B C

    1975-04-01

    A series of thirty-eight cases of densely sclerotic lesions of the jaws was studied. The lesions were all at least 1.5 cm, in diameter. Pain, drainage, or localized expansion of the jaw was present in two thirds of the patients. Thirty-four of the thirty-eight patients were women, and twenty-five were negros. In fourteen instances, a radiographic survey of the entire jaws was available. Thirteen of these fourteen patients had multiple lesions which were usually symmetrically distributed throughout the jaws. In twenty-four patients radiographs of the lesional area only were available for review. In thirty-four of the thirty-eight cases (89 per cent), the sclerotic masses were interpreted as cementum. Although this condition has previously been described as chronic sclerosing osteomyelitis, sclerosing osteitis, multiple enostosis, or gigantiform cementoma, it appears more appropriate to consider these lesions as part of the spectrum of the benign fibro-osseous lesions of periodontal ligament origin. Four of the sclerotic lesions, which were radiographically indistinguishable from the cemental lesions, consisted only of bone. Two of these appeared to be examples of true chronic sclerosing osteomyelitis, and two may be appropriately designated as enostosis or osteomas.

  5. [Idiopathic Addison's disease].

    Science.gov (United States)

    Khodasevich, L S; Sukhanov, S G

    1988-01-01

    Two cases of idiopathic Addison's disease affecting heterozygous twins who died from addisonian crisis at the ages of 7 and 10 years, are described, including descriptions of histologic findings in the adrenals, thymus, hypophysis, kidneys, esophagus, upper respiratory tract, and other viscera. The authors believe that autoimmunization in children with idiopathic Addison's disease develops in the presence of congenitally defective immunity and propose that activation of the juxtaglomerular complex and hypergranulation of interstitial cells in the inner renal medulla may be used as morphologic criteria in the diagnosis of addisonian crisis.

  6. Idiopathic 20-nail dystrophy

    DEFF Research Database (Denmark)

    Larsen, Camilla; Bygum, Anette

    2016-01-01

    Twenty-nail dystrophy is a rare disease, typically with all 20 nails affected, which normally occurs as an idiopathic condition in childhood but can be linked to other diseases. We report a case of a 7-year-old girl with a 3-year history of 20-nail dystrophy and no associated diseases or family...... history of skin or nail diseases. She was followed and treated conservatively and, after 6 years of follow-up, we found a marked improvement and almost full resolution of her nail dystrophy. The aim of this report is to show that idiopathic 20-nail dystrophy in children is a self-limiting condition...

  7. Midazolam or diazepam administration during electroencephalography helps to diagnose subacute sclerosing panencephalitis (SSPE).

    Science.gov (United States)

    Yilmaz, Kutluhan; Sahin, Derya Aydin

    2010-08-01

    Although diagnostic contribution of intravenous diazepam administration during electroencephalography (EEG) recording in subacute sclerosing panencephalitis has been known, no another drug with less potential side effects has been studied in this procedure. In this study, diazepam is compared with midazolam in 25 subacute sclerosing panencephalitis-diagnosed children and 10 children with subacute sclerosing panencephalitis-suggesting symptoms, normal EEG findings and no certain diagnosis. Neither midazolam nor diazepam affected typical periodic slow-wave complexes. However, in the patients with atypical EEG abnormalities, midazolam, like diazepam, attenuated sharp or sharp-and-slow waves, and therefore made the identification of periodic slow-wave paroxysms easier. In the patients with normal EEGs, both midazolam and diazepam revealed typical periodic complexes on EEG recording in the same 3 patients. Cerebrospinal fluid examination verified the diagnosis of subacute sclerosing panencephalitis. The findings suggest that midazolam or diazepam administration increases the contribution of EEG recording in atypical cases with subacute sclerosing panencephalitis.

  8. Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

    Science.gov (United States)

    Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

    2010-09-01

    To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

  9. Idiopathic benign breast calcification.

    Science.gov (United States)

    Connors, Alissa M; Svensson, William E; Shousha, Sami

    2004-01-01

    A 56-year-old woman who came in for screening mammography was found to have extensive unilateral calcification of her left breast which had developed since her previous screening mammogram. The calcification had a ductal and lobular appearance. Possible known etiologies are discussed, but these do not explain the appearance in this case, implying that the cause is idiopathic.

  10. Idiopathic granulomatous orbital inflammation

    NARCIS (Netherlands)

    Mombaerts, I.; Schlingemann, R. O.; Goldschmeding, R.; Koornneef, L.

    1996-01-01

    PURPOSE: Granulomatous orbital inflammation may occur as an isolated condition of unknown origin. These idiopathic granulomatous lesions are believed to belong to the orbital pseudotumor group by some authors, whereas others consider them sarcoidosis limited to the orbit. The aim of this study is to

  11. Idiopathic Interstitial Pneumonias

    Science.gov (United States)

    ... Most Often Affected Percentage of Affected People Who Smoke Cigarettes Treatment Outlook Idiopathic pulmonary fibrosis More frequently, men ... HealthDay Vitamin D Linked to Lower Risk of Respiratory Infections News HealthDay Remede System Approved for Sleep Apnea News HealthDay Nasal Swab ...

  12. Idiopathic Retroperitoneal Hematoma

    African Journals Online (AJOL)

    In the early stages it typically presents as generalized abdominal pain, nausea, anorexia. The symptoms and signs of hypovolemic shock normally present late. As management of idiopathic retroperitoneal hematoma is usually conservative, early diagnosis can save the patient an unnecessary exploration. This will improve.

  13. Idiopathic neuralgic amyotrophy in children. Case report, 4 year follow up and review of the literature

    DEFF Research Database (Denmark)

    Høst, Christian; Skov, Liselotte

    2010-01-01

    Idiopathic neuralgic amyotrophy (INA) is a neurological disorder with a suspected autoimmune cause, and is characterized by a sudden onset of pain and subsequent atrophies of the arm and shoulder muscles. It has rarely been documented in children, but it has been suggested that the prognostic...

  14. Idiopathic neuralgic amyotrophy in children. Case report, 4 year follow up and review of the literature

    DEFF Research Database (Denmark)

    Høst, Christian; Skov, Liselotte

    2010-01-01

    Idiopathic neuralgic amyotrophy (INA) is a neurological disorder with a suspected autoimmune cause, and is characterized by a sudden onset of pain and subsequent atrophies of the arm and shoulder muscles. It has rarely been documented in children, but it has been suggested that the prognostic out...

  15. Distinctive inflammatory bowel disease phenotype in primary sclerosing cholangitis.

    Science.gov (United States)

    de Vries, A Boudewijn; Janse, Marcel; Blokzijl, Hans; Weersma, Rinse K

    2015-02-14

    To review the current literature for the specific clinical characteristics of inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC). A systematical review for clinical characteristics of IBD in PSC was performed by conducting a broad search for "primary sclerosing cholangitis" in Pubmed. "Clinical characteristics" were specified into five predefined subthemes: epidemiology of IBD in PSC, characteristics of IBD in PSC (i.e., location, disease behavior), risk of colorectal cancer development, IBD recurrence and de novo disease after liver transplantation for PSC, and safety and complications after proctocolectomy with ileal pouch-anal anastomosis. Papers were selected for inclusion based on their relevance to the subthemes, and were reviewed by two independent reviewers. Only full papers relevant to PSC-IBD were included. Additionally the references of recent reviews for PSC ( 75%). The highest IBD rates in PSC are found in papers reviewing both endoscopic and histological data for IBD diagnosis. Although IBD in PSC is found to be a quiescent disease, pancolitis occurs often, with rates varying from 35% to 95%. Both backwash ileitis and rectal sparing are observed infrequently. The development of dysplasia or colorectal carcinoma is increased in PSC-IBD; the cumulative 10 years risk varying between 0% and 11%. Exacerbation of IBD is common after liver transplantation for PSC and de novo disease is seen in 1.3% to 31.3% of PSC-IBD patients. The risk for development of pouchitis in PSC-IBD is found to be significant, affecting 13.8% to 90% of the patients after proctocolectomy with ileo anal-pouch anastomosis. IBD in primary sclerosing cholangitis represents a distinct phenotype that differs from UC and Crohn's disease and therefore requires specialized management.

  16. Lymphoplasmacytic sclerosing cholangitis: assessment of clinical, CT, and pathological findings

    Energy Technology Data Exchange (ETDEWEB)

    Itoh, S., E-mail: shigekimiyo@luck.ocn.ne.j [Department of Technical Radiology, Nagoya University School of Health Sciences, Nagoya (Japan); Nagasaka, T. [Department of Pathology, Nagoya University Graduate School of Medicine, Nagoya (Japan); Suzuki, K.; Satake, H.; Ota, T.; Naganawa, S. [Department of Radiology, Nagoya University Graduate School of Medicine, Nagoya (Japan)

    2009-11-15

    Aim: To assess the clinical, computed tomography (CT), and pathological findings in patients with lymphoplasmacytic sclerosing cholangitis. Materials and methods: Fifteen consecutive patients (four women and 11 men, mean age 71 years) with lymphoplasmacytic sclerosing cholangitis and without the characteristic features of underlying disorders causing benign biliary strictures were retrospectively recruited. Two radiologists evaluated multiphase contrast-enhanced CT images acquired with 0.5 or 1-mm collimation. One pathologist performed all histological examinations, including IgG4 immunostaining. Results: The intrahepatic biliary ducts showed dilatation in all 15 patients, but only seven presented with jaundice. Although laboratory data were not available in all patients, serum gammaglobulin and IgG levels were elevated in five of six patients and six of eight patients, respectively. Anti-nuclear antibody was detected in three of six patients. The involved biliary ducts showed the following CT findings: involvement of the hilar biliary duct (14/15), a mean wall thickness of 4.9 mm, a smooth margin (10/15), a narrow but visible lumen (6/15), hyper-attenuation during the late arterial phase (9/15), homogeneous hyper-attenuation during the delayed phase (11/11), and no vascular invasion (14/15). Abnormal findings in the pancreas and urinary tract were detected in eight of 15 patients. In 13 patients with adequate specimens, moderate to severe lymphoplasmacytic infiltration associated with dense fibrosis was observed. Infiltration of IgG4-positive plasma cells was moderate or severe in nine patients and minimal or absent in four patients. Conclusion: Lymphoplasmacytic sclerosing cholangitis exhibits relatively characteristic clinical and CT findings, although they are not sufficiently specific for differentiation from other biliary diseases.

  17. Primary sclerosing cholangitis and the management of uncertainty and complexity.

    Science.gov (United States)

    Arndtz, Katherine; Hirschfield, Gideon M

    2017-10-01

    Primary sclerosing cholangitis (PSC) is a rare chronic autoimmune disease with no effective therapy and a large unmet need for new treatments. Patients require significant healthcare resources over their lifetime with high rates of hospital admission, malignancy, liver transplantation and death. As a rare disease, expertise in management can be limited to large referral liver transplant programmes, and even then there is frequently variation in practice. In this case-based review, we aim to discuss common clinical dilemmas encountered by clinicians managing patients with PSC and address related competencies in the 2010 Gastroenterology Curriculum.

  18. An 111In-Pentetreotide Positive Sclerosing Pneumocytoma.

    Science.gov (United States)

    Savelli, Giordano; Bnà, Claudio; Zambelli, Claudia; Illuminati, Sonia; Bonello, Luke

    2017-04-01

    A 43-year-old woman had an incidental lung mass identified on shoulder x-ray performed for pain. Contrast-enhanced CT showed a 38-mm mass in the medial segment of the right middle lobe, with features suggestive of carcinoid tumor. A In-pentetreotide scan showed intense uptake; furthermore, fine needle aspiration biopsy yielded neuroendocrine cells confirming the carcinoid hypothesis. However, definitive surgical histology showed a sclerosing pneumocytoma. This could potentially suggest that such rare tumors, with metastatic potential, could respond to somatostatin analogue treatment.

  19. Primary Sclerosing Cholangitis: Therapeutic Options and Surveillance Management

    Directory of Open Access Journals (Sweden)

    Aditi Kumar

    2016-09-01

    Full Text Available Primary sclerosing cholangitis is a chronic immune-mediated liver disease. Though rare, it poses several clinical concerns for the managing physician. There are currently limited therapeutic options in the management of the condition and weak evidence base behind them. Endoscopic intervention is limited to those patients with obstructing stricture-related disease, and even liver transplantation has a risk of disease recurrence. Surveillance for inflammatory bowel disorders, metabolic bone disease, and malignancy is paramount when managing such patients. This article provides an overview of the condition with further focus on current therapeutic options and guidance on surveillance management.

  20. Management of primary sclerosing cholangitis: Conventions and controversies

    Science.gov (United States)

    Chandok, Natasha; Hirschfield, Gideon M

    2012-01-01

    Primary sclerosing cholangitis (PSC) is a chronic inflammatory cholangiopathy that results in fibrotic strictures and dilations of the intra- and extrahepatic bile ducts. PSC is uncommon, occurs predominantly in males and has a strong association with inflammatory bowel disease. While the pathogenesis of PSC has not been fully elucidated, emerging evidence supports roles for the innate and adaptive immune systems, and genome-wide analyses have identified several genetic associations. Using the best available evidence, the present review summarizes the current understanding of the diagnosis, pathogenesis and management of PSC. Despite its rarity, there is an urgent need for collaborative research efforts to advance therapeutic options for PSC beyond liver transplantation. PMID:22590699

  1. Current Concepts in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

    Science.gov (United States)

    Sclair, Seth N; Little, Ester; Levy, Cynthia

    2015-01-01

    Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic, cholestatic diseases of the liver with common clinical manifestations. Early diagnosis and treatment of PBC slows progression and decreases the need for transplant. However, one-third of patients will progress regardless of treatment. Bilirubin cholangitis must be excluded when considering the diagnosis of PSC, to allow for proper treatment, and monitoring of disease progression. In addition to the lack of therapy, PSC is a pre-malignant condition and close surveillance is indicated. PMID:26312413

  2. The Gut-Liver Axis in Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Eksteen, Bertus

    2016-02-01

    Dysregulation of the key genetic, immunologic, and microbiome compounds of the gut-liver axis is the basis for inflammatory bowel disease (IBD) and primary sclerosing cholangitis (PSC). This creates opportunities to accelerate therapies that have been traditionally developed for IBD to be used in PSC to the benefit of both diseases. Shared genetic susceptibility loci has yielded important clues into the pathogenesis of PSC-IBD. Understanding of the critical links between PSC and IBD are essential in designing clinical care pathways for these complex patients. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Atypical clinical course subacute sclerosing panencephalitis presenting as acute Encephalitis

    Science.gov (United States)

    Komur, Mustafa; Arslankoylu, Ali E; Okuyaz, Cetin; Kuyucu, Necdet

    2012-01-01

    We report a 14-year-old boy who presented with loss of consciousness and gait instability. The electroencephalogram (EEG) showed generalized slowing with irregular activity and cerebral magnetic imaging revealed asymmetrical nonspecific signals on basal ganglia. His second electroencephalogram revealed periodical generalized high-voltage slow wave complexes which did not disappear with diazepam induction. Subacute sclerosing panencephalitis (SSPE) was considered and the diagnosis was confirmed with the identification of measles antibodies in cerebrospinal fluid. Our findings show that SSPE should be in mind in the differential diagnosis of meningoencephalitis and acute disseminated encephalomyelitis and highlight the significance of EEG in the diagnosis of unidentified cases. PMID:23248691

  4. Catatonia as presenting clinical feature of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Dayal, Prabhoo; Balhara, Yatan Pal Singh

    2014-01-01

    Catatonia is not a usual clinical presentation of subacute sclerosing panencephalitis (SSPE), especially in the initial stages of illness. However, there is only one reported case of SSPE presenting as catatonia among children. In this report, however, there were SSPE-specific changes on EEG and the catatonia failed to respond to lorazepam. We describe a case of SSPE in a child presenting as catatonia that presented with clinical features of catatonia and did not have typical EEG findings when assessed at first contact. He responded to lorazepam and EEG changes emerged during the course of follow-up.

  5. Computed tomographic findings of early subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Pedersen, H.; Wulff, C.H.

    1982-03-01

    Computed tomography of the brain (CT) was carried out at the early stages of subacute sclerosing panencephalitis (SSPE) in three children. The lateral ventricles were very small and the hemispheric sulci and interhemispheric fissures were not visible in all three patients in contrast to severe atrophy found at a later stage in one patient. The early CT abnormalities were revealed at the same time as the titres of measles antibodies in blood and cerebrospinal fluid were elevated, and the characteristic periodic complexes in the electroencephalogram established the diagnosis of SSPE. The CT changes indicating brain swelling reflect the reactive changes of this slow virus infection.

  6. Oxidant and antioxidant status in children with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Caksen, Hüseyin; Ozkan, Mustafa; Cemek, Mustafa; Cemek, Fatma

    2014-11-01

    We analyzed serum alpha-tocopherol, beta-carotene, retinol, and ascorbic acid levels and malondialdehyde and reduced glutathione concentrations on erythrocyte and cerebrospinal fluid in 30 patients with subacute sclerosing panencephalitis to evaluate oxidant and antioxidant status. Serum alpha-tocopherol, beta-carotene, retinol, ascorbic acid levels, and erythrocyte and cerebrospinal fluid reduced glutathione concentrations were decreased; however, erythrocyte and cerebrospinal fluid malondialdehyde levels were increased in the patients. Cerebrospinal fluid malondialdehyde levels were different between clinical stages of the disease (P panencephalitis and that antioxidants were increased as defense mechanisms of the organism against oxidative damage. © The Author(s) 2013.

  7. Silicone-induced Penile Sclerosing Lipogranuloma: Magnetic Resonance Imaging Findings

    Directory of Open Access Journals (Sweden)

    Athina C Tsili

    2016-01-01

    Full Text Available Sclerosing lipogranuloma is a rare benign disease, representing a peculiar granulomatous reaction of fatty tissue. The majority of cases are secondary to injection of exogenous foreign bodies, such as silicone, paraffin, mineral, or vegetable oils. To the best of our knowledge, we present the first case of a silicone-induced penile lipogranuloma in a 52-year-old man evaluated with a multiparametric magnetic resonance imaging (MRI protocol, including diffusion-weighted imaging, magnetization transfer imaging, and dynamic contrast-enhanced MRI. MRI of the penis by combining both conventional and functional information represents an important imaging tool in the preoperative workup of silicone-induced penile lipogranuloma.

  8. IgG4(+) plasma cells in sclerosing variant of mucoepidermoid carcinoma.

    Science.gov (United States)

    Tian, Wei; Yakirevich, Evgeny; Matoso, Andres; Gnepp, Douglas R

    2012-07-01

    IgG4-related sclerosing disease is a recently described syndrome with unique histologic features characterized by intense lymphoplasmacytic infiltrates with increased IgG4 plasma cells and dense stromal sclerosis. The disease spectrum frequently includes benign inflammatory diseases, such as autoimmune pancreatitis, cholangitis, and chronic sclerosing sialadenitis (CSS). Mucoepidermoid carcinoma (MEC) is the most common primary malignancy in the salivary gland. The rare sclerosing variant of MEC is characterized by dense stromal sclerosis and lymphoplasmacytic infiltrates. Our goal was to further characterize lymphoplasmacytic infiltrates with respect to IgG4 expression. Six sclerosing MECs from our pathology service over the past 20 years were selected. In addition, 11 regular MECs with lymphoplasmacytic infiltrates, 4 CSS cases, and 12 nonsclerosing chronic sialadenitis cases were evaluated. None of the sclerosing MEC patients had IgG4-related sclerosing disease. The absolute number of IgG4 plasma cells was significantly increased in sclerosing MEC as compared with the regular type (75 vs. 20 per image field; Psclerosis in cancer.

  9. Expression patterns of micro-RNAs 146a, 181a, and 155 in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Yiş, Uluç; Tüfekçi, Uğur Kemal; Genç, Şermin; Çarman, Kürşat Bora; Bayram, Erhan; Topçu, Yasemin; Kurul, Semra Hız

    2015-01-01

    Subacute sclerosing panencephalitis is caused by persistent brain infection of mutated virus, showing inflammation, neurodegeneration, and demyelination. Although many factors are emphasized in the pathogenesis of subacute sclerosing panencephalitis, the exact mechanism of neurodegeneration remains unknown. Micro-RNAs are small, noncoding RNAs that regulate gene expression at the posttranscriptional levels. Micro-RNAs are essential for normal immune system development; besides they are also implicated in the pathogenesis of many chronic inflammatory disorders. The aim of this study is to investigate the expression patterns of micro-RNAs 146a, 181a, and 155 in peripheral blood mononuclear cells of patients with subacute sclerosing panencephalitis. We enrolled 39 patients with subacute sclerosing panencephalitis and 41 healthy controls. Quantitative analysis of micro-RNAs 146a, 181a, and 155 were performed using specific stem-loop primers followed by real-time polymerase chain reaction. All of 3 micro-RNAs were upregulated in subacute sclerosing panencephalitis patients. In addition, the level of micro-RNA 155 expression was higher in stage 3 patients. But, micro-RNA 146a and 181a expression levels showed no association or correlation with clinically relevant data. Alteration of peripheral blood mononuclear cell micro-RNAs in subacute sclerosing panencephalitis may shed new light on the pathogenesis of disease and may contribute to the aberrant systemic rise in mRNA levels in subacute sclerosing panencephalitis. © The Author(s) 2014.

  10. Genetics Home Reference: idiopathic pulmonary fibrosis

    Science.gov (United States)

    ... Twitter Home Health Conditions Idiopathic pulmonary fibrosis Idiopathic pulmonary fibrosis Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Idiopathic pulmonary fibrosis is a chronic, progressive lung disease. This condition ...

  11. Genetics Home Reference: adolescent idiopathic scoliosis

    Science.gov (United States)

    ... Home Health Conditions Adolescent idiopathic scoliosis Adolescent idiopathic scoliosis Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Adolescent idiopathic scoliosis is an abnormal curvature of the spine that ...

  12. Extracorporeal life support for a 5-week-old infant with idiopathic pulmonary hemosiderosis.

    Science.gov (United States)

    Gutierrez, Sherrill; Shaw, Susanna; Huseni, Shehlanoor; Sachdeva, Shagun; Costello, John P; Basu, Sonali; Nath, Dilip S; Klugman, Darren

    2014-12-01

    Idiopathic pulmonary hemosiderosis is a rare disease defined by the triad of iron deficiency anemia, hemoptysis, and diffuse pulmonary infiltrates on chest radiograph. Idiopathic pulmonary hemosiderosis is known to cause dyspnea and, in some cases, acute onset of massive pulmonary hemorrhage which is traditionally treated with conventional mechanical ventilation or high-frequency oscillation in conjunction with immunosuppressive therapy. In this case report, we describe a 5-week-old infant presenting with hemoptysis, massive pulmonary hemorrhage, and significant hypercapnic respiratory failure. The patient failed conventional ventilation but responded well to extracorporeal life support that was initiated early in his course. Idiopathic pulmonary hemosiderosis was suspected in light of his response to high-dose steroids and was confirmed by subsequent lung biopsies. Patients with severe pulmonary hemorrhage secondary to idiopathic pulmonary hemosiderosis can be safely supported with extracorporeal life support when conventional therapies have been exhausted.

  13. Long-term follow-up of children and adolescents with primary sclerosing cholangitis and autoimmune sclerosing cholangitis.

    Science.gov (United States)

    Smolka, Vratislav; Karaskova, Eva; Tkachyk, Oksana; Aiglova, Kvetoslava; Ehrmann, Jiri; Michalkova, Kamila; Konecny, Michal; Volejnikova, Jana

    2016-08-01

    Sclerosing cholangitis (SC) is a chronic cholestatic hepatobiliary disease with uncertain long-term prognosis in pediatric patients. This study aimed to evaluate long-term results in children with SC according to the types of SC. We retrospectively followed up 25 children with SC over a period of 4-17 years (median 12). The diagnosis of SC was based on biochemical, histological and cholangiographic findings. Patients fulfilling diagnostic criteria for probable or definite autoimmune hepatitis at the time of diagnosis were defined as having autoimmune sclerosing cholangitis (ASC); other patients were included in a group of primary sclerosing cholangitis (PSC). The incidence of the following complications was studied: obstructive cholangitis, portal hypertension, advanced liver disease and death associated with the primary disease. Fourteen (56%) patients had PSC and 11 (44%) had ASC. Patients with ASC were significantly younger at the time of diagnosis (12.3 vs 15.4 years, P=0.032) and had higher IgG levels (22.7 vs 17.2 g/L, P=0.003). The mentioned complications occurred in 4 (16%) patients with SC, exclusively in the PSC group: one patient died from colorectal cancer, one patient underwent liver transplantation and two patients, in whom severe bile duct stenosis was present at diagnosis, were endoscopically treated for acute cholangitis. Furthermore, two other children with ASC and 2 children with PSC had elevated aminotransferase levels. The 10-year overall survival was 95.8% in all patients, 100% in patients without complicated liver disease, and 75.0% in patients with complications. In children, ASC is a frequent type of SC, whose prognosis may be better than that in patients with PSC.

  14. An evaluation of neuropeptide Y status in subacute sclerosing panencephalitis patients.

    Science.gov (United States)

    Abuhandan, M; Calik, M; Almaz, V; Taskın, A; Cakmak, A; Selek, S

    2013-01-01

    This study aimed to evaluate the neuropeptide Y values of patients with subacute sclerosing panencephalitis. The study comprised 38 patients diagnosed with subacute sclerosing panencephalitis at our clinic, who were being routinely followed-up, together with a control group of 36. Using the Risk and Haddad classification for clinical staging, 16 patients were determined as Stage II and 22 patients as Stage III. Neuropeptide Y values were measured by Enzyme Immunoassay methods. The neuropeptide Y values of the all patients with subacute sclerosing panencephalitis were found to be significantly high compared to the control group (ppanencephalitis.

  15. Acute Idiopathic Scrotal Edema

    Directory of Open Access Journals (Sweden)

    Micheál Breen

    2013-01-01

    Full Text Available We report a case of acute idiopathic scrotal edema (AISE in a 4-year-old boy who presented with acute scrotal pain and erythema. The clinical features, ultrasound appearance, and natural history of this rare diagnosis are reviewed. In this report, we highlight the importance of good ultrasound technique in differentiating the etiology of the acute scrotum and demonstrate the color Doppler “Fountain Sign” that is highly suggestive of AISE.

  16. Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity.

    Science.gov (United States)

    Upadhyayula, Pavan S; Yang, Jason; Yue, John K; Ciacci, Joseph D

    2017-11-07

    Subacute sclerosing panencephalitis (SSPE) is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine. SSPE is a devastating disease with a characteristic clinical course in subcortical white matter; however, atypical presentations of brainstem involvement may be seen in rare cases. This review summarizes reports to date on brainstem involvement in SSPE, including the clinical course of disease, neuroimaging presentations, and guidelines for treatment. A comprehensive literature search was performed for English-language publications with keywords "subacute sclerosing panencephalitis" and "brainstem" using the National Library of Medicine PubMed database (March 1981-September 2017). Eleven articles focusing on SSPE of the brainstem were included. Predominant brainstem involvement remains uncharacteristic of SSPE, which may lead to misdiagnosis and poor outcome. A number of case reports have demonstrated brainstem involvement associated with other intracranial lesions commonly presenting in later SSPE stages (III and IV). However, brainstem lesions can appear in all stages, independent of higher cortical structures. The varied clinical presentations complicate diagnosis from a neuroimaging perspective. SSPE of the brainstem is a rare but important clinical entity. It may present like canonical SSPE or with unique clinical features such as absence seizures and pronounced ataxia. While SSPE generally progresses to the brainstem, it can also begin with a primary focus of infection in the brainstem. Awareness of varied SSPE presentations can aid in early diagnosis as well as guide management and treatment.

  17. Subacute Sclerosing Panencephalitis of the Brainstem as a Clinical Entity

    Directory of Open Access Journals (Sweden)

    Pavan S. Upadhyayula

    2017-11-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a rare progressive neurological disorder of early adolescence caused by persistent infection of the measles virus, which remains prevalent worldwide despite an effective vaccine. SSPE is a devastating disease with a characteristic clinical course in subcortical white matter; however, atypical presentations of brainstem involvement may be seen in rare cases. This review summarizes reports to date on brainstem involvement in SSPE, including the clinical course of disease, neuroimaging presentations, and guidelines for treatment. A comprehensive literature search was performed for English-language publications with keywords “subacute sclerosing panencephalitis” and “brainstem” using the National Library of Medicine PubMed database (March 1981–September 2017. Eleven articles focusing on SSPE of the brainstem were included. Predominant brainstem involvement remains uncharacteristic of SSPE, which may lead to misdiagnosis and poor outcome. A number of case reports have demonstrated brainstem involvement associated with other intracranial lesions commonly presenting in later SSPE stages (III and IV. However, brainstem lesions can appear in all stages, independent of higher cortical structures. The varied clinical presentations complicate diagnosis from a neuroimaging perspective. SSPE of the brainstem is a rare but important clinical entity. It may present like canonical SSPE or with unique clinical features such as absence seizures and pronounced ataxia. While SSPE generally progresses to the brainstem, it can also begin with a primary focus of infection in the brainstem. Awareness of varied SSPE presentations can aid in early diagnosis as well as guide management and treatment.

  18. Cholangiocarcinoma and its mimickers in primary sclerosing cholangitis.

    Science.gov (United States)

    Lee, Jonghun John; Schindera, Sebastian T; Jang, Hyun-Jung; Fung, Scott; Kim, Tae Kyoung

    2017-12-01

    Cholangiocarcinoma (CCA) is the most common malignancy in primary sclerosing cholangitis (PSC). Approximately half of CCA are diagnosed within two years of initial diagnosis and often have a poor prognosis because of advanced tumor stage at the time of diagnosis. Thus, rigorous initial imaging evaluation for detecting CCA is important. CCA in PSC usually manifests as intrahepatic mass-forming or perihilar periductal-infiltrating type. Imaging diagnosis is often challenging due to pre-existing biliary strictures and heterogeneous liver. Multimodality imaging approach and careful comparison with prior images are often helpful in detecting small CCA. Ultrasound is widely used as an initial test, but has a limited ability to detect small tumors in the heterogeneous liver with PSC. MRI combined with MRCP is excellent to demonstrate focal biliary abnormalities as well as subtle liver masses. Contrast-enhanced ultrasound is useful to demonstrate CCA by demonstrating rapid and marked washout. In addition, there are other disease entities that mimic CCA including hepatocellular carcinoma, confluent hepatic fibrosis, IgG4-related sclerosing cholangitis, inflammatory mass, and focal fat deposition. In this pictorial essay, imaging findings of CCA in PSC is described and discuss the challenges in imaging surveillance for CCA in the patients with PSC. Imaging findings of the mimickers of CCA in PSC and their differentiating features are also discussed.

  19. Steroid-refractory ulcerative colitis and associated primary sclerosing cholangitis treated with infliximab.

    Science.gov (United States)

    Duca, Ileana; Ramírez de la Piscina, Patricia; Estrada, Silvia; Calderón, Rosario; Spicakova, Katerina; Urtasun, Leire; Marra-López, Carlos; Zabaleta, Salvador; Bengoa, Raquel; Marcaide, María Asunción; García-Campos, Francisco

    2013-01-28

    Primary sclerosing cholangitis is an infrequent extraintestinal manifestation of ulcerative colitis. Damage to bile ducts is irreversible and medical therapies to prevent progression of the disease are usually ineffective. We describe a patient with long-standing ulcerative colitis, which was refractory to corticosteroid therapy who developed primary sclerosing cholangitis (biochemical stage II/IV) in the course of his pancolitis. Treatment with infliximab (5 mg/kg as an induction dose followed by maintenance doses every two months) was indicated because of steroid-dependent disease associated to primary sclerosing cholangitis as well as sacroiliitis and uveitis and previous episode of severe azathioprine-related hepatic toxicity. At present, after two years of follow-up, the patient is asymptomatic with normal liver tests and complete resumption of daily life activities. This case draws attention to the usefulness of anti-tumor necrosis factor-alpha therapy for the management of primary sclerosing cholangitis as extraintestinal manifestation of inflammatory bowel disease.

  20. BILATERAL SUBRETINAL FLUID AND RETINAL VASCULOPATHY ASSOCIATED WITH SUBACUTE SCLEROSING PANENCEPHALITIS.

    Science.gov (United States)

    Agarwal, Aniruddha; Singh, Ramandeep; Kumar, Abiraj; Dogra, Mangat R; Gupta, Amod

    2017-01-01

    To report a case of bilateral retinopathy associated with subacute sclerosing panencephalitis. History and clinical examination, fluorescein angiography, and optical coherence tomography. We report a rare case of unilateral, followed by bilateral retinopathy, subretinal fluid, and vasculopathy in a young boy. History of missed measles vaccination, behavioral and neurologic symptoms, and electroencephalogram suggested a diagnosis of subacute sclerosing panencephalitis. Retinal imaging using optical coherence tomography was performed to document changes in the retinal microstructure through the natural course of the disease. Within 8 weeks, the changes progressed to retinal atrophy in both eyes. The progressive course of retinitis associated with subacute sclerosing panencephalitis can be monitored on optical coherence tomography. Retinitis is subacute sclerosing panencephalitis rapidly progressive from the acute stage to the stage of atrophy, involving full thickness of the retina.

  1. Unique optical coherence tomography findings in a case of macular retinitis caused by subacute sclerosing panencephalitis.

    Science.gov (United States)

    Hata, Masayuki; Oishi, Akio; Kurimoto, Yasuo

    2012-01-01

    Subacute sclerosing panencephalitis is a rare neurodegenerative disease. The ophthalmologic findings, typically necrotizing retinitis, often precede other neurologic signs and symptoms. Ocular findings are helpful for early diagnosis, but the findings of optical coherence tomography (OCT) images in an active phase of retinitis have never been reported. We report unique findings of OCT images in a case of subacute sclerosing panencephalitis. A 13-year-old boy was referred to our hospital complaining of visual loss and other neurologic symptoms. The fundus examination showed necrotizing retinitis. The OCT images showed empty spaces involving all layers of retina except the inner limiting membrane and hyperreflective lesion in inner retina. The diagnosis of subacute sclerosing panencephalitis was made from the blood and the spinal cerebral fluid test. This is the first report on OCT images of necrotizing retinitis in an active stage of subacute sclerosing panencephalitis case. The unique findings of OCT images as in this case help to diagnose the rare devastating disease.

  2. Sclerosing epithelioid fibrosarcoma of the thigh: report of two cases with synchronous bone metastases.

    Science.gov (United States)

    Righi, A; Gambarotti, M; Manfrini, M; Benini, S; Gamberi, G; Cocchi, S; Casadei, R; Picci, P; Vanel, D; Dei Tos, A P

    2015-09-01

    We report two cases of sclerosing epithelioid fibrosarcoma occurring in the deep soft tissue of the thigh, confirmed by molecular analysis and associated with bone metastases in the lumbar vertebrae and the iliac wing at the time of diagnosis. Synchronous bone metastases of sclerosing epithelioid fibrosarcoma are extremely difficult to diagnose because clinical and radiological features are not specific. In addition, the range of differential diagnoses is very wide, including metastatic carcinoma and osteosarcoma. At present, all but three published cases of sclerosing epithelioid fibrosarcoma with bone metastases showed bone metastases during follow-up. We confirm in our two cases that the distinct pattern of immunohistochemical staining for MUC4, associated with the absence of staining for both SATB2, a marker of osteoblastic differentiation, and pan-cytokeratin, allows differentiating between sclerosing epithelioid fibrosarcoma and metastatic carcinoma or osteosarcoma.

  3. Idiopathic Fascicular Left Ventricular Tachycardia.

    Science.gov (United States)

    Alahmad, Yaser; Asaad, Nidal Ahmad; Arafa, Salaheddin Omran; Ahmad Khan, Shahul Hameed; Mahmoud, Alsayed

    2017-01-01

    Idiopathic left fascicular ventricular tachycardia (ILFVT) is characterized by right bundle branch block morphology and left axis deviation. We report a case of idiopathic left ventricular fascicular tachycardia in a young 31-year-old male patient presenting with a narrow complex tachycardia.

  4. Idiopathic Fascicular Left Ventricular Tachycardia

    OpenAIRE

    Alahmad, Yaser; Asaad, Nidal Ahmad; Arafa, Salaheddin Omran; Ahmad Khan, Shahul Hameed; Mahmoud, Alsayed

    2017-01-01

    Idiopathic left fascicular ventricular tachycardia (ILFVT) is characterized by right bundle branch block morphology and left axis deviation. We report a case of idiopathic left ventricular fascicular tachycardia in a young 31-year-old male patient presenting with a narrow complex tachycardia.

  5. Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature

    OpenAIRE

    Chow, L T C; Lui, Y H; Kumta, S M; Allen, P W

    2004-01-01

    Sclerosing epithelioid fibrosarcoma is a rare tumour characterised histologically by a predominant population of epithelioid cells arranged in strands and nests, embedded in a fibrotic and hyalinised stroma. It is a low grade tumour with an indolent course. A 48 year old woman presented with a painful swelling over her back for six months. Investigation and biopsy revealed features of sclerosing epithelioid fibrosarcoma involving the left half of the sacrum, left sacro-iliac joint, and poster...

  6. Bilateral optic neuritis--the only ocular finding in a case of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Ozer, Pinar Altiaylik; Ozkan, Mehpare; Sekeroglu, Hande Taylan; Kadayifcilar, Sibel; Yuksel, Deniz; Aksoy, Ayse

    2014-02-01

    Subacute sclerosing panencephalitis is a rare disease of central nervous system caused by defective measles virus. Chorioretinitis with macular involvement is the mostly observed ocular finding in the disease. Other reported ocular findings in the disease are cortical blindness, hemianopsia, nystagmus, extraocular muscle paresis and optic atrophy. We present a rare case of subacute sclerosing panencephalitis with isolated bilateral optic neuritis as the only ocular finding without macular involvement.

  7. Nonconvulsive Status Epilepticus on Electroencephalography: An Atypical Presentation of Subacute Sclerosing Panencephalitis in Two Children

    Science.gov (United States)

    Singhi, Pratibha; Saini, Arushi Gahlot; Sahu, Jitendra Kumar

    2012-01-01

    Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography. We report two pediatric cases who presented with periodic myoclonic jerks and cognitive decline. In both cases, the electroencephalogram showed continuous nonconvulsive status epilepticus activity. Both had elevated measles antibodies in cerebrospinal fluid and blood. Pediatricians need to be aware of this atypical presentation of subacute sclerosing panencephalitis. PMID:23094174

  8. Nonconvulsive Status Epilepticus on Electroencephalography: An Atypical Presentation of Subacute Sclerosing Panencephalitis in Two Children

    Directory of Open Access Journals (Sweden)

    Pratibha Singhi

    2012-01-01

    Full Text Available Subacute sclerosing panencephalitis is a neurodegenerative disease secondary to measles infection that usually has a typical presentation with progressive myoclonia, cognitive decline, and periodic slow-wave complexes on electroencephalography. We report two pediatric cases who presented with periodic myoclonic jerks and cognitive decline. In both cases, the electroencephalogram showed continuous nonconvulsive status epilepticus activity. Both had elevated measles antibodies in cerebrospinal fluid and blood. Pediatricians need to be aware of this atypical presentation of subacute sclerosing panencephalitis.

  9. Idiopathic short stature

    Directory of Open Access Journals (Sweden)

    Vlaški Jovan

    2013-01-01

    Full Text Available Growth is a complex process and the basic characteristic of child- hood growth monitoring provides insight into the physiological and pathological events in the body. Statistically, the short stature means departure from the values of height for age and sex (in a particular environment, which is below -2 standard deviation score, or less than -2 standard deviation, i.e. below the third percentile. Advances in molecular genetics have contributed to the improvement of diagnostics in endocrinology. Analysis of patients’ genotypes should not be performed before taking a classical history, detailed clinical examination and appropriate tests. In patients with idiopathic short stature specific causes are excluded, such as growth hormone deficiency, Turner syndrome, short stature due to low birth weight, intrauterine growth retardation, small for gestational age, dysmorphology syndromes and chronic childhood diseases. The exclusion of abovementioned conditions leaves a large number of children with short stature whose etiology includes patients with genetic short stature or familial short stature and those who are low in relation to genetic potential, and who could also have some unrecognized endocrine defect. Idiopathic short stature represents a short stature of unknown cause of heterogeneous etiology, and is characterized by a normal response of growth hormone during stimulation tests (>10 ng/ml or 20 mJ/l, without other disorders, of normal body mass and length at birth. In idiopathic short stature standard deviation score rates <-2.25 (-2 to -3 or <1.2 percentile. These are also criteria for the initiation of growth hormone therapy. In children with short stature there is also the presence of psychological and social suffering. Goals of treatment with growth hormone involve achieving normal height and normal growth rate during childhood.

  10. IDIOPATHIC PULMONARY HEMOSIDEROSIS

    Science.gov (United States)

    Yettra, Maurice; Goldenberg, Erwin; Weiner, Herman

    1960-01-01

    Idiopathic pulmonary hemosiderosis is a rare condition manifested by recurrent pulmonary hemorrhage of unknown cause, diffuse radiologic abnormalities, cough, hemoptysis and moderate to severe hypochromic anemia. Diagnosis can be confirmed by iron stains of the sputum or lung aspiration or by biopsy. Prolonged spontaneous remission may occur without the use of corticosteroid therapy. Studies here reported indicated that the anemia is hypochromic and microcytic anemia of blood loss and iron deficiency, in spite of the presence of large amounts of iron in the pulmonary tissue. Correction of the anemia by intensive iron therapy and transfusion is considered an important part of therapy. ImagesFigure 1.Figure 2.Figure 3. PMID:13787318

  11. Early juvenile idiopathic arthritis.

    Science.gov (United States)

    Marzan, Katherine Anne B; Shaham, Bracha

    2012-05-01

    Early juvenile idiopathic arthritis (JIA) is important to recognize as timely diagnosis and treatment improves prognosis. It is a misconception that complications of JIA arise only from long-standing disease and that children will outgrow it. Early aggressive treatment is the paradigm as early disease activity has long-term consequences. There are predictors of persistent disease and joint erosions that may identify patients at higher risk. Control of disease activity within the first 6 months of onset confers improved clinical course and outcomes. The treatment perspective is thus one of early aggressive treatment for induction of disease control and ultimately remission. Copyright © 2012 Elsevier Inc. All rights reserved.

  12. Hypercalcemia in idiopathic myelofibrosis

    DEFF Research Database (Denmark)

    Voss, A; Schmidt, K; Hasselbalch, H

    1992-01-01

    A case of idiopathic myelofibrosis (IMF) presenting with hypercalcemia and hypercalcitriolemia is reported. It is proposed that ectopic production of the active vitamin D metabolite related to ongoing clonal expansion in the bone marrow accounts for the hypercalcemic state. Consistently low levels...... of circulating type I procollagen propeptide (PICP) and lack of progression of the bone marrow fibrosis during almost 6 months of follow-up point to an in vivo inhibition of type I collagen synthesis by 1,25-dihydroxyvitamin D3....

  13. Molecular and radiological diagnosis of sclerosing bone dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Hul, Wim van E-mail: vhul@uia.ac.be; Vanhoenacker, Filip; Balemans, Wendy; Janssens, Katrien; Schepper, A.M. de

    2001-12-01

    Bone mineral density (BMD) is a quantitative trait for which the heritability of the variance is estimated to be up to 80%, based on epidemiological and twin studies. Further illustration of the involvement of genetic factors in bone homeostasis, is the existence of an extended group of genetic conditions associated with an abnormal bone density. The group of conditions with increased bone density has long been poorly studied and understood at the molecular genetic level but recently, thanks to recent developments in molecular genetics and genomics, for some of them major breakthroughs have been made. These findings will make the molecular analysis of such patients an additional tool in diagnostics and in genetic counseling. However, the initial identification of affected patients is still largely dependent upon recognition of clinical and radiological stigmata of the disease. Therefore, in this overview of sclerosing bone dysplasias, the classical clinical and radiological signs of this group of disorders will be discussed along with the new molecular insights.

  14. Recurrence and rejection in liver transplantation for primary sclerosing cholangitis

    Science.gov (United States)

    Fosby, Bjarte; Karlsen, Tom H; Melum, Espen

    2012-01-01

    Primary sclerosing cholangitis (PSC) is a chronic progressive inflammatory disease affecting the bile ducts, leading to fibrosis and eventually cirrhosis in most patients. Its etiology is unknown and so far no effective medical therapy is available. Liver transplantation (LTX) is the only curative treatment and at present PSC is the main indication for LTX in the Scandinavian countries. Close to half of the PSC patients experience one or more episodes of acute cellular rejection (ACR) following transplantation and approximately 1/5 of the transplanted patients develop recurrent disease in the graft. In addition, some reports indicate that ACR early after LTX for PSC can influence the risk for recurrent disease. For these important post-transplantation entities affecting PSC patients, we have reviewed the current literature on epidemiology, pathogenesis, treatment and the possible influence of rejection on the risk of recurrent disease in the allograft. PMID:22228965

  15. Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

    Directory of Open Access Journals (Sweden)

    Roosy Aulakh

    2013-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a devastating “slow virus” brain disease resulting from persistent measles virus infection of neurons. The age at presentation is usually 8 to 11 years with onset usually occurring 2–10 years after measles infection. We report a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline. He was diagnosed as a case of SSPE based on clinical features, typical electroencephalographic finding, and elevated cerebrospinal fluid/serum measles antibody titers. He had measles 4 months prior to onset of symptoms. This case along with review of recently published reports suggests progressively decreasing latency period between measles infection and onset of symptoms observed in cases with SSPE. Clinical implication would mean investigating for SSPE even in infants or toddlers with compatible clinical features and recent history of measles infection.

  16. Subacute sclerosing panencephalitis resembling Rasmussen's encephalitis on magnetic resonance imaging.

    Science.gov (United States)

    Jakkani, Ravi Kanth; Sureka, Jyoti; Panwar, Sanuj

    2015-09-01

    Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients. Magnetic resonance (MR) imaging is the modality of choice for determining such changes in white matter. SSPE typically demonstrates bilateral but asymmetric periventricular and subcortical white matter involvement. We herein report a rare case of unilateral white matter involvement in a 13-year-old boy with SSPE that closely simulated Rasmussen's encephalitis. To the best of our knowledge, this is the first report of an atypical presentation on MR imaging in which SSPE was a rare cause of unilateral brain parenchymal involvement in a patient with intractable seizures.

  17. Notes from the Field: Subacute Sclerosing Panencephalitis Death - Oregon, 2015.

    Science.gov (United States)

    Liko, Juventila; Guzman-Cottrill, Judith A; Cieslak, Paul R

    2016-01-15

    In 2015, the Oregon Health Authority was notified of the death of a boy with subacute sclerosing panencephalitis (SSPE), a rare and fatal complication of measles. The patient, aged 14 years, had reportedly been vaccinated against measles in the Philippines at age 8 months. However, the patient contracted measles at age 1 year while still in the Philippines. He had been well until 2012, when his neurodegenerative symptoms began. After the diagnosis of SSPE was made, the patient remained in home hospice care until his death. Investigators from the Oregon Health Authority and the Oregon Health and Science University reviewed the patient's medical records and interviewed the parents. Vaccination against measles can prevent not only acute measles and its complications, but also SSPE.

  18. Cerebral glucose metabolism in the course of subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Huber, M.; Herholz, K.; Pawlik, G.; Szelies, B.; Juergens, R.H.; Heiss, W.D.

    1989-01-01

    Regional cerebral glucose metabolism was studied in a 15-year-old boy with subacute sclerosing panencephalitis before and after therapy with human interferon beta, using positron emission tomography of fluorine 18-2-fluoro-2-deoxyglucose. At first examination, metabolism was symmetrically decreased in the thalamus, cerebellum, and all cortical areas except prerolandic motor cortex, but increased in lentiform nucleus. A computed tomographic scan was normal. Six months later, bilateral focal necrosis centered in the previously hypermetabolic putamen was demonstrated by computed tomography and magnetic resonance imaging. The caudate nucleus and the superoposterior part of the putamen were spared, still showing increased metabolism. Corresponding with some clinical improvement, cortical glucose consumption rates had returned to a normal level.

  19. Subacute sclerosing panencephalitis (SSPE) the story of a vanishing disease.

    Science.gov (United States)

    Gadoth, Natan

    2012-10-01

    Subacute sclerosing panencephalitis (SSPE), is a devastating "slow virus" brain disease which affects young children who had measles some 6-7 years earlier. Although, the pandemic of SSPE during 1960-1980's was almost eradicated due to mass immunization, the disease is still taking the life of young children in countries where measles immunization is incomplete and in world regions where genetic polymorphism to this particular infection is present. The present review was written for the fortunate young generation of pediatricians and pediatric neurologists who probably have not seen a case of SSPE during their career, and for those who work in counties where the disease has not been eradicated. It is also a reminder that with full coverage of measles immunization this devastating disease can be fully eradicated. Copyright © 2012 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  20. Subacute Sclerosing Panencephalitis with Atypical Clinical and MRI Findings

    Directory of Open Access Journals (Sweden)

    Buse Rahime Hasırcı

    2016-04-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a progressive inflammatory disorder of the central nervous system and a slow virus infection caused by aberrant measles virüs. Typical neurological manifestations include psychomotor impairment, progressive intellectual deterioration, myoclonic jerks and behavioral changes, with or without pyramidal symptoms. It usually affecting people aged 10 to 14 year. We report the case of an 17-year-old girl presenting with initial symptom of visual loss, seizures, a lack of SSPE specific EEG pattern, late onset and atypical fast progression of disease. The case highlights the importance of atypical clinical findings of SSPE at onset and also firstly disappearing, then appearing MRI findings at sequential images which can complicate the accurate diagnosis. High suspicion is needed because of its rareness.

  1. Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis

    Directory of Open Access Journals (Sweden)

    Biplab Das

    2016-01-01

    Full Text Available Objectives: Subacute sclerosing panencephalitis (SSPE is rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection with measles virus. No cure for SSPE exists, but the condition can be managed by medication if treatment is started at an early stage. Methods and Results: Heterogeneity of imaging findings in SSPE is not very uncommon. But pial and gyral enhancements are very rarely noticed. Significant asymmetric onset as well as pial-gyral enhancements is not reported. Herein we present a case of 16 years adolescent of SSPE having remarkable asymmetric pial-gyral enhancements, which were misinterpreted as tubercular infection. Conclusion: Early diagnosis and treatment is encouraging in SSPE, although it is not curable with current therapy. Clinico-radiological and electrophysiological correlation is very important in diagnosis of SSPE, more gravely in patients having atypical image findings as in our index case.

  2. Fulminant adult-onset subacute sclerosing panencephalitis: a case report

    Science.gov (United States)

    Faivre, Anthony; Souraud, Jean-Baptiste; McGonigal, Aileen; Alla, Philippe; Grapperon, Jacques; Valance, Jacques

    2009-01-01

    We present the case of a young adult who developed acute encephalopathy with severe status epilepticus and rapid deterioration to vegetative state and death within 6 weeks. Although the clinical picture, MRI and EEG findings were atypical, the hypothesis of subacute sclerosing panencephalitis (SSPE) was suggested by markedly increased intrathecal IgG synthesis in the cerebrospinal fluid, and diagnosis was confirmed by the presence of high antimeasles antibodies in cerebrospinal fluid and brain biopsy findings. Acute SSPE is an exceptionally rare and little-known form of SSPE with protean symptomatology, and this case is to our knowledge the first observation of SSPE presenting with status epilepticus in adults. Our case reinforces the need to include, even in developed countries, SSPE as a diagnostic possibility in unexplained acute encephalopathies. PMID:21686559

  3. Mesenchymal stem cell application in children with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Kuşkonmaz, Bariş; Uçkan, Duygu; Yalnizoğlu, Dilek; Günel, Mintaze; Karli Oğuz, Kader; Konuşkan, Bahadir; Anlar, Banu

    2015-09-01

    Subacute sclerosing panencephalitis (SSPE) is a serious, often fatal disease that responds poorly to current treatment modalities. Recently, the ability of mesenchymal stem cells (MSCs) to produce neurotrophic factors and inflammatory molecules has placed them among potential treatment agents for neurological conditions. We report the results of four patients treated with MSC for SSPE. The patients were followed up clinically, and by periodical laboratory evaluations, magnetic resonance imaging (MRI), and electroencephalography. One patient deteriorated to stage III of the disease, two patients remained in the same stage, and one died from disease progression and respiratory problems. Neurological findings and electroencephalography scores were consistent with the clinical course of the patient whereas MRI showed new inflammatory lesions in two patients. This is the first report of the application of MSC in SSPE. No benefit is demonstrated. © 2015 Mac Keith Press.

  4. Atypical magnetic resonance imaging features in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Das, Biplab; Goyal, Manoj Kumar; Modi, Manish; Mehta, Sahil; Chakravarthi, Sudheer; Lal, Vivek; Vyas, Sameer

    2016-01-01

    Subacute sclerosing panencephalitis (SSPE) is rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection with measles virus. No cure for SSPE exists, but the condition can be managed by medication if treatment is started at an early stage. Heterogeneity of imaging findings in SSPE is not very uncommon. But pial and gyral enhancements are very rarely noticed. Significant asymmetric onset as well as pial-gyral enhancements is not reported. Herein we present a case of 16 years adolescent of SSPE having remarkable asymmetric pial-gyral enhancements, which were misinterpreted as tubercular infection. Early diagnosis and treatment is encouraging in SSPE, although it is not curable with current therapy. Clinico-radiological and electrophysiological correlation is very important in diagnosis of SSPE, more gravely in patients having atypical image findings as in our index case.

  5. Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

    Science.gov (United States)

    Aulakh, Roosy; Tiwari, Abhimanyu

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a devastating “slow virus” brain disease resulting from persistent measles virus infection of neurons. The age at presentation is usually 8 to 11 years with onset usually occurring 2–10 years after measles infection. We report a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline. He was diagnosed as a case of SSPE based on clinical features, typical electroencephalographic finding, and elevated cerebrospinal fluid/serum measles antibody titers. He had measles 4 months prior to onset of symptoms. This case along with review of recently published reports suggests progressively decreasing latency period between measles infection and onset of symptoms observed in cases with SSPE. Clinical implication would mean investigating for SSPE even in infants or toddlers with compatible clinical features and recent history of measles infection. PMID:24416610

  6. Occult Celiac Disease Associated with Lymphocytic Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    Hugh J Freeman

    1994-01-01

    Full Text Available A 60-year-old male with dermatitis herpetiformis and a previously treated lymphoma involving an inguinal lymph node developed abnormal liver chemistry tests. Because of intermittent diarrhea, additional studies revealed lymphocytic colitis and occult celiac disease that responded to a gluten-free diet. A liver biopsy done to explore persistently abnormal liver chemistry tests showed a portal tract-centred inflammatory process characterized by biliary ductal proliferation, epithelial lymphocytosis and concentric lamellar fibrosis. Quantitation of immunoglobulins was normal and antimitochondrial antibodies were negative. Retrograde cholangiograms showed radiological features typical of primary sclerosing cholangius. The epithelial lymphocycosis reported in gastric, small and large intestinal mucosa of some patients with celiac disease may also be present in the biliary ductal columnar epithelium. This report provides additional evidence that celiac disease may be a far more extensive pathological process.

  7. Primary sclerosing cholangitis – The arteriosclerosis of the bile duct?

    Science.gov (United States)

    Fickert, Peter; Moustafa, Tarek; Trauner, Michael

    2007-01-01

    Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of unknown aetiology affecting the large bile ducts and characterized by periductal fibrosis and stricture formation, which ultimately result in biliary cirrhosis and liver failure. Arteriosclerosis involves the accumulation of altered lipids and lipoproteins in large arteries; this drives inflammation and fibrosis and ultimately leads to narrowing of the arteries and hypoperfusion of dependent organs and tissues. Knowledge of the causative factors is crucial to the understanding of disease mechanisms and the development of specific treatment. Based on pathogenetic similarities between PSC and arteriosclerosis, we hypothesize that PSC represents "arteriosclerosis of the bile duct" initiated by toxic biliary lipids. This hypothesis is based on common molecular, cellular, and morphological features providing the conceptual framework for a deeper understanding of their pathogenesis. This hypothesis should stimulate translational research to facilitate the search for novel treatment strategies for both diseases. PMID:17254334

  8. Primary sclerosing cholangitis associated with inflammatory bowel disease: an update.

    Science.gov (United States)

    Rossi, Roberta E; Conte, Dario; Massironi, Sara

    2016-02-01

    Primary sclerosing cholangitis (PSC) is a chronic progressive disease, usually associated with underlying inflammatory bowel diseases (IBDs), with a prevalence of 60-80% in western countries. Herein, we review the current knowledge about the association between PSC and IBD in terms of clinical approach and long-term patient management. A PubMed search was conducted for English-language publications from 2000 through 2015 using the following keywords: primary sclerosing cholangitis, inflammatory bowel disease, ulcerative colitis, Crohn's disease, diagnosis, therapy, follow-up, and epidemiology. In terms of diagnosis, liver function tests and histology are currently used. The medical treatment options for PSC associated with IBD do not differ from the cases of PSC alone, and include ursodeoxycholic acid and immunosuppressive agents. These treatments do not seem to improve survival, even if ursodeoxycholic acid given at low doses may be chemopreventive against colorectal cancer (CRC). Liver transplantation is the only potential curative therapy for PSC with reported survival rates of 85 and 70% at 5 and 10 years after transplant; however, there is a risk for PSC recurrence, worsening of IBD activity, and de-novo IBD occurrence after liver transplantation. PSC-IBD represents an important public health concern, especially in view of the increased risk for malignancy, including CRC. Long-life annual surveillance colonoscopy is usually recommended, although the exact timescale is still unclear. Further studies are required both to clarify whether annual colonoscopy is cost-effective, especially in younger patients, and to identify potential pharmaceutical agents and genetic targets that may retard disease progression and protect against CRC.

  9. Trigger mechanisms of secondary sclerosing cholangitis in critically ill patients.

    Science.gov (United States)

    Leonhardt, Silke; Veltzke-Schlieker, Wilfried; Adler, Andreas; Schott, Eckart; Hetzer, Roland; Schaffartzik, Walter; Tryba, Michael; Neuhaus, Peter; Seehofer, Daniel

    2015-03-31

    In recent years the development of secondary sclerosing cholangitis in critically ill patients (SSC-CIP) has increasingly been perceived as a separate disease entity. About possible trigger mechanisms of SSC-CIP has been speculated, systematic investigations on this issue are still lacking. The purpose of this study was to evaluate the prevalence and influence of promoting factors. Temporality, consistency and biological plausibility are essential prerequisites for causality. In this study, we investigated the temporality and consistency of possible triggers of SSC-CIP in a large case series. Biological plausibility of the individual triggers is discussed in a scientific context. SSC-CIP cases were recruited retrospectively from 2633 patients who underwent or were scheduled for liver transplantation at the University Hospital Charité, Berlin. All patients who developed secondary sclerosing cholangitis in association with intensive care treatment were included. Possible trigger factors during the course of the initial intensive care treatment were recorded. Sixteen patients (68% males, mean age 45.87 ± 14.64 years) with a confirmed diagnosis of SSC-CIP were identified. Of the 19 risk factors investigated, particularly severe hypotension with a prolonged decrease in mean arterial blood pressure (MAP) to <65 mmHg and systemic inflammatory response syndrome (SIRS) were established as possible triggers of SSC-CIP. The occurrence of severe hypotension appears to be the first and most significant step in the pathogenesis. It seems that severe hypotension has a critical effect on the blood supply of bile ducts when it occurs together with additional microcirculatory disturbances. In critically ill patients with newly acquired cholestasis the differential diagnosis of SSC-CIP should be considered when they have had an episode of haemodynamic instability with a prolonged decrease in MAP, initial need for large amounts of blood transfusions or colloids, and early

  10. Idiopathic Calcinosis Cutis over Back

    Directory of Open Access Journals (Sweden)

    Tanveer Shaikh

    2017-04-01

    Full Text Available Calcinosis Cutis is characterized by the deposition of calcium salts in the skin and subcutaneous tissue. Idiopathic Calcinosis Cutis is a rare condition and hence is usually a diagnosis of exclusion. Idiopathic Calcinosis Cutis occurs in the absence of known trauma, inciting agent or metabolic defect. This is a case report of an adult female presenting with Idiopathic Calcinosis Cutis over the back. The exact mechanism of occurrence of this condition is not known. Patients are usually managed with pharmacotherapy, surgery being reserved for those with pain, recurrent infection and impaired function. The lesions are known to recur and a periodic follow-up of these patients is essential.

  11. Perinatal outcomes of idiopathic polyhydramnios

    Science.gov (United States)

    Taskin, Salih; Kanmaz, Ahkam Göksel; Kahraman, Korhan; Kurtay, Gülay

    2013-01-01

    Objective To investigate the perinatal outcomes of cases with idiopathic polyhydramnios. Study Design Retrospective analysis of 160 singleton pregnancies that were under routine surveillance at the department of obstetrics from 2008 to 2010 was performed to assess perinatal outcomes. Finally, 59 cases were included as idiopathic polyhydramnios, and 101 cases were included as controls. Preterm delivery (4000 g), 1- and 5-min APGAR scores polyhydramnios group compared with the control group. Conclusion Although perinatal outcomes are conflicting in literature, idiopathic polyhydramnios warrants close surveillance especially near term. PMID:24265884

  12. Comparison of intraductal ultrasonography findings between primary sclerosing cholangitis and IgG4-related sclerosing cholangitis.

    Science.gov (United States)

    Naitoh, Itaru; Nakazawa, Takahiro; Hayashi, Kazuki; Miyabe, Katsuyuki; Shimizu, Shuya; Kondo, Hiromu; Nishi, Yuji; Yoshida, Michihiro; Umemura, Shuichiro; Hori, Yasuki; Kato, Akihisa; Okumura, Fumihiro; Sano, Hitoshi; Ohara, Hirotaka; Joh, Takashi

    2015-06-01

    Comparisons of intraductal ultrasonography (IDUS) findings between primary sclerosing cholangitis (PSC) and IgG4-related sclerosing cholangitis (IgG4-SC) have not been elucidated. We aimed to clarify the differences in transpapillary IDUS findings between PSC and IgG4-SC. We retrospectively compared transpapillary IDUS findings between 15 patients with PSC and 35 patients with IgG4-SC between 2004 and 2014. IDUS findings of circular-asymmetric wall thickness, irregular inner margin, diverticulum-like outpouching, unclear outer margin, heterogeneous internal echo, and disappearance of three layers were significantly higher in PSC than in IgG4-SC (P < 0.001). Irregular inner margin, diverticulum-like outpouching, and disappearance of three layers were specific IDUS findings for PSC compared to IgG4-SC. Diverticulum-like outpouching on IDUS and endoscopic retrograde cholangiogram (ERC) was observed in 10 (67%) and five (33%) of 15 patients with PSC, respectively. However, based on IDUS and ERC, diverticulum-like outpouching was not observed in any patient with IgG4-SC. All five patients with diverticulum-like outpouching on ERC had diverticulum-like outpouching on IDUS, and five (50%) of 10 patients without diverticulum-like outpouching on ERC had diverticulum-like outpouching on IDUS. The IDUS findings differed between PSC and IgG4-SC. Irregular inner margin, diverticulum-like outpouching, and disappearance of three layers are specific IDUS findings for PSC compared to IgG4-SC. IDUS is a more useful procedure than ERC for the early detection of diverticulum-like outpouching. © 2015 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd.

  13. Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI.

    Science.gov (United States)

    Kim, Jin Hee; Byun, Jae Ho; Kim, So Yeon; Lee, Seung Soo; Kim, Hyoung Jung; Kim, Myung-Hwan; Lee, Moon-Gyu

    2013-07-01

    It is essential to differentiate sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) from primary sclerosing cholangitis (PSC) as the treatment and prognosis of the two diseases are totally different. To compare image findings of SC-AIP and PSC on endoscopic retrograde cholangiography (ERC), magnetic resonance cholangiography (MRC), computed tomography (CT), and magnetic resonance imaging (MRI). Two radiologists retrospectively reviewed ERC, MRC, CT, and MRI in 28 SC-AIP and 23 PSC patients in consensus. Factors evaluated included the length, location, and multiplicity of bile duct stricture, the presence of characteristic cholangiographic features of PSC on ERC and MRC, and the presence, location, thickness, and pattern of bile duct wall thickening on CT and MRI. On ERC, focal stricture, multifocal and intrahepatic bile duct stricture, and beaded, pruned-tree, and diverticulum-like appearance were more frequent in PSC than in SC-AIP patients (P ≤ 0.006). On MRC, multifocal and intrahepatic bile duct stricture and pruned-tree appearance were more frequent in PSC than in SC-AIP patients (P ≤ 0.044). On CT and MRI, the bile duct wall was thicker (5.1 mm vs. 3.1 mm; P = 0.033 and 4.3 mm vs. 3.0 mm; P = 0.01, respectively) in SC-AIP than in PSC patients. PSC was more frequently associated with intrahepatic bile duct wall thickening on both CT (93% vs. 50%; P = 0.024) and MRI (100% vs. 50%; P = 0.023) than SC-AIP. The combination of ERC or MRC with cross-sectional images, including CT and MRI, may be helpful in differentiating between SC-AIP and PSC.

  14. Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis.

    Science.gov (United States)

    Moon, Sung-Hoon; Kim, Myung-Hwan; Lee, Jong Kyun; Baek, Seunghee; Woo, Young Sik; Cho, Dong Hui; Oh, Dongwook; Song, Tae Jun; Park, Do Hyun; Lee, Sang Soo; Seo, Dong Wan; Lee, Sung Koo

    2017-04-01

    Recent research has shown that a substantial number of patients with primary sclerosing cholangitis (PSC) can also have elevated serum/tissue IgG4. The aim of our study was to develop a simple scoring system for the discrimination of IgG4-related sclerosing cholangits (IgG4-SC) from PSC. Patients with IgG4-SC (n = 39) and PSC (n = 76) who had intrahepatic/hilar strictures were included. Candidate-differentiating variables included patient age, other organ involvement (OOI), inflammatory bowel disease, serum IgG4, and cholangiographic features. A scoring system was developed on the basis of these variables, and its performance was internally validated using a bootstrapping-based method. The scoring system in the final model included age (<30 years, 0 points; 30-39 years, 1 point; 40-49 years, 2 points; 50-59 years, 3 points; ≥60 years, 4 points), OOI (no, 0 points; yes, 3 points), and beaded appearance (yes, 0 points; no, 2 points). The patients were classified according to their total score into three categories: 0-4 points, probable PSC; 5-6 points, indicating diagnostic steroid trial; 7-9 points, probable IgG4-SC. The discrimination between IgG4-SC and PSC using the scoring system was excellent (area under the receiver operating characteristic curve, 0.986). A reliable differentiation of IgG4-SC from PSC can be made using the scoring system presented here. We suggest the diagnosis of IgG4-SC at a cutoff of 7 points or higher and the indication of diagnostic steroid trial at 5 or 6 points. External validation of our scoring system is warranted.

  15. Sclerosing cholangitis with autoimmune pancreatitis versus primary sclerosing cholangitis: comparison on endoscopic retrograde cholangiography, MR cholangiography, CT, and MRI

    Energy Technology Data Exchange (ETDEWEB)

    Kim; Jin Hee; Byun, Jae Ho; Kim, So Yeon; Lee, Seung Soo; Kim, Hyoung Jung; Lee, Moon-Gyu [Dept. of Radiology and Research Inst. of Radiology, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)], e-mail: jhbyun@amc.seoul.kr; Kim, Myung-Hwan [Dept. of Internal Medicine, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)

    2013-07-15

    Background: It is essential to differentiate sclerosing cholangitis with autoimmune pancreatitis (SC-AIP) from primary sclerosing cholangitis (PSC) as the treatment and prognosis of the two diseases are totally different. Purpose: To compare image findings of SC-AIP and PSC on endoscopic retrograde cholangiography (ERC), magnetic resonance cholangiography (MRC), computed tomography (CT), and magnetic resonance imaging (MRI). Material and Methods: Two radiologists retrospectively reviewed ERC, MRC, CT, and MRI in 28 SC-AIP and 23 PSC patients in consensus. Factors evaluated included the length, location, and multiplicity of bile duct stricture, the presence of characteristic cholangiographic features of PSC on ERC and MRC, and the presence, location, thickness, and pattern of bile duct wall thickening on CT and MRI. Results: On ERC, focal stricture, multifocal and intrahepatic bile duct stricture, and beaded, pruned-tree, and diverticulum-like appearance were more frequent in PSC than in SC-AIP patients (P = 0.006). On MRC, multifocal and intrahepatic bile duct stricture and pruned-tree appearance were more frequent in PSC than in SC-AIP patients (P = 0.044). On CT and MRI, the bile duct wall was thicker (5.1 mm vs. 3.1 mm; P = 0.033 and 4.3 mm vs. 3.0 mm; P = 0.01, respectively) in SC-AIP than in PSC patients. PSC was more frequently associated with intrahepatic bile duct wall thickening on both CT (93% vs. 50%; P = 0.024) and MRI (100% vs. 50%; P = 0.023) than SC-AIP. Conclusion: The combination of ERC or MRC with cross-sectional images, including CT and MRI, may be helpful in differentiating between SC-AIP and PSC.

  16. Diffuse sclerosing variant of papillary carcinoma of the thyroid. Clinical importance, surgical treatment, and follow-up study

    Energy Technology Data Exchange (ETDEWEB)

    Fujimoto, Y.; Obara, T.; Ito, Y.; Kodama, T.; Aiba, M.; Yamaguchi, K. (Tokyo Women' s Medical College (Japan))

    1990-12-01

    A diffuse sclerosing variant is not very rare among papillary carcinomas of the thyroid when the patients are female and younger than 30 years of age. The variant is characterized by diffuse involvement of one or both thyroid lobes, with dense sclerosis, patchy lymphocytic infiltration, and abundant psammoma bodies. Controversy still exists concerning its prognosis. We reviewed our experience with 14 patients treated between 1958 and 1988. All patients were young females, their age being from 10 to 28 years with a mean of 19.6. Hashimoto's thyroiditis had been suspected in nine patients before they came to our clinic. Nowadays the diagnosis of this cancer is possible when we have this entity in mind and detect abundant psammoma bodies either by ultrasonography or by soft-tissue roentgenography of the neck. Total thyroidectomy with modified neck dissection was carried out in eight patients, subtotal thyroidectomy with neck dissection in five, and lobectomy with neck dissection in one. All of them are alive and well without distant metastasis at a mean follow-up of 16 years. Because most of the patients with this variant of papillary carcinoma are young women and the prognosis is favorable, a complete resection without causing later recurrence, but also cosmetic and complication-free surgery, should be considered.

  17. Sclerosing variant of well-differentiated liposarcoma: relative prevalence and spectrum of CT and MRI features.

    Science.gov (United States)

    Bestic, Joseph M; Kransdorf, Mark J; White, Lawrence M; Bridges, Mellena D; Murphey, Mark D; Peterson, Jeffrey J; Garner, Hillary W

    2013-07-01

    The purpose of this study was to determine the relative prevalence of the sclerosing variant of well-differentiated liposarcoma at one institution and to elucidate the CT and MRI characteristics of this subtype of well-differentiated liposarcoma. A retrospective computerized search was conducted to calculate the relative prevalence of the sclerosing variant of well-differentiated liposarcoma among all well-differentiated liposarcoma subtypes at one institution. The MRI and CT features of a total of 19 cases of pathologically proven sclerosing variant of well-differentiated liposarcoma were evaluated (seven identified from the study institution database and 12 cases contributed by other institutions). The cases of a total of 36 patients with well-differentiated liposarcoma were identified in the pathology database; six (17%) cases had evidence of dedifferentiation. Seven (19%) cases of sclerosing variant of well-differentiated liposarcoma were identified. Of these, three (43%) had evidence of dedifferentiation. On images, the sclerosing variant of well-differentiated liposarcoma typically presented as a large (average, 16.6 cm) well-circumscribed heterogeneous mass most commonly situated in the retroperitoneum (58%). Sixteen of the 19 tumors evaluated (84%) had predominantly well-circumscribed margins. Tumor composition ranged from predominantly fatty to entirely devoid of macroscopic fat; only three (16%) were composed of more than 75% fat. Variable amounts of nonlipomatous elements were identified in all cases. Enhancement of these elements was evident at CT or MRI in all 14 cases in which enhancement could be reliably assessed. The sclerosing variant of well-differentiated liposarcoma should be included in the differential diagnosis of any well-circumscribed lipomatous mass containing variable amounts of nonlipomatous elements, particularly when located in the retroperitoneum. Unlike other subtypes of well-differentiated liposarcoma, the sclerosing variant is

  18. Idiopathic thrombocytopenic purpura

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    L Kayal

    2014-01-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP is defined as a hematologic disorder, characterized by isolated thrombocytopenia without a clinically apparent cause. The major causes of accelerated platelet consumption include immune thrombocytopenia, decreased bone marrow production, and increased splenic sequestration. The clinical presentation may be acute with severe bleeding, or insidious with slow development with mild or no symptoms. The initial laboratory tests useful at the first visit to predict future diagnosis were erythrocyte count, leukocyte count, anti-glycoprotein IIb/IIIa antibodies, reticulated platelets, plasma thrombopoietin level. Treatment should be restricted to those patients with moderate or severe thrombocytopenia who are bleeding or at risk of bleeding. We present a case report on ITP with clinical presentation, diagnosis and management.

  19. Juvenile idiopathic arthritis

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    Krupa H Bhatt

    2014-01-01

    Full Text Available Juvenile Idiopathic Arthritis (JIA is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential.

  20. Idiopathic Bilateral Bloody Tearing

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    Emrullah Beyazyıldız

    2015-01-01

    Full Text Available Bloody tear is a rare and distinct clinic phenomenon. We report a case presenting with the complaint of recurrent episodes of bilateral bloody tearing. A 16-year-old girl presented to our clinic with complaint of bloody tearing in both eyes for 3 months. Bloody tearing was not associated with her menses. A blood-stained discharge from the punctum was not observed during the compression of both nasolacrimal ducts. Nasolacrimal passage was not obstructed. Imaging studies such as dacryocystography and gradient-echo magnetic resonance imaging (MRI of nasolacrimal canal were normal. Intranasal endoscopic evaluation was normal. We collected samples from bloody tears two times and pathological examination was performed. Pathological analysis showed lots of squamous cells and no endometrial cells; dysplastic cells were found. Further evaluations for underlying causes were unremarkable. No abnormalities were found in ophthalmologic, radiologic, and pathologic investigations. This condition is likely a rare abnormality and the least recognized aetiology for the idiopathic phenomenon.

  1. ADHD in idiopathic epilepsy

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    Marcos H. C. Duran

    2014-01-01

    Full Text Available Our aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD with epilepsy and behavior problems. This was a cross-sectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners’ Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%, seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.

  2. Idiopathic Normal Pressure Hydrocephalus

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    Basant R. Nassar BS

    2016-04-01

    Full Text Available Idiopathic normal pressure hydrocephalus (iNPH is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” “gait disturbances,” “cognitive function,” “neuropsychology,” “imaging,” and “pathogenesis,” articles were obtained for this review. The majority of the articles were retrieved from the past 10 years. The purpose of this review article is to aid general practitioners in further understanding current findings on the pathogenesis, diagnosis, and treatment of iNPH.

  3. Idiopathic pulmonary fibrosis: treatment update.

    LENUS (Irish Health Repository)

    O'Connell, Oisin J

    2011-11-01

    Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Despite multiple recent clinical trials, there is no strong evidence supporting a survival advantage for any agent in the management of patients with IPF. The limited effectiveness of current treatment regimes has led to a search for novel therapies including antifibrotic strategies. This article reviews the evidence supporting the treatments currently used in the management of IPF.

  4. Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature.

    Science.gov (United States)

    Chow, L T C; Lui, Y H; Kumta, S M; Allen, P W

    2004-01-01

    Sclerosing epithelioid fibrosarcoma is a rare tumour characterised histologically by a predominant population of epithelioid cells arranged in strands and nests, embedded in a fibrotic and hyalinised stroma. It is a low grade tumour with an indolent course. A 48 year old woman presented with a painful swelling over her back for six months. Investigation and biopsy revealed features of sclerosing epithelioid fibrosarcoma involving the left half of the sacrum, left sacro-iliac joint, and posterior part of the left ilium. Preoperative radiotherapy and wide location excision of the tumour were followed by metastatic recurrence of the tumour in the lung and scalp six years after initial presentation. The tumour showed typical histology of sclerosing epithelioid fibrosarcoma. The radiological features confirmed its primary location in the sacrum. The patient declined chemotherapy and died of disseminated disease eight years after initial presentation. Review of the literature confirms the fact that sclerosing epithelioid fibrosarcoma, despite its low grade, is a clinicopathologically distinct tumour with full malignant potential, the recurrence, metastasis, and mortality rate being 48%, 60%, and 35%, respectively. Sclerosing epithelioid fibrosarcoma can occur as a primary bone tumour, the clinical behaviour of which is probably similar to its soft tissue counterpart.

  5. Telomere dysfunction in peripheral blood lymphocytes from patients with primary sclerosing cholangitis and inflammatory bowel disease.

    Science.gov (United States)

    Laish, Ido; Katz, Hila; Stein, Assaf; Liberman, Meytal; Naftali, Timna; Kitay-Cohen, Yona; Biron-Shental, Tal; Konikoff, Fred M; Amiel, Aliza

    2015-09-01

    Primary sclerosing cholangitis and inflammatory bowel disease are two associated, chronic inflammatory, pre-malignant conditions. We hypothesized that patients with these disorders may harbour telomere dysfunction as a marker of chromosomal instability. The aim of our study was to compare parameters of the telomere-telomerase system in these cohorts. In this prospective study, peripheral blood was withdrawn from patients with primary sclerosing cholangitis (N=20), inflammatory bowel disease (N=20) and healthy controls (N=20), and lymphocytes were isolated. Telomere length was quantified as a function of the signal intensity and telomere number. Random aneuploidy and telomere capture were determined by fluorescence in situ hybridization technique with specific probes. Patients with inflammatory bowel disease had higher measures of intestinal disease activity than patients with primary sclerosing cholangitis. Despite this, shorter telomere length and telomere aggregates, especially the fusion of 2-5 telomeres, were observed at significantly higher rate in patients with primary sclerosing cholangitis relative to inflammatory bowel disease or healthy controls. Rates of aneuploidy and telomere capture were higher in the two probes in both diseases compared to controls (pprimary sclerosing cholangitis patients more than inflammatory bowel disease and healthy controls patients, which attests to genetic instability and immunosenescence. NCT02247622. Copyright © 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  6. Morphological changes in vascular and circulating blood cells following exposure to detergent sclerosants.

    Science.gov (United States)

    Cooley-Andrade, O; Connor, D E; Ma, D D F; Weisel, J W; Parsi, K

    2016-04-01

    To investigate morphological changes in vascular and circulating blood cells following exposure to detergent sclerosants sodium tetradecyl sulfate and polidocanol. Samples of whole blood, isolated leukocytes, platelets, endothelial cells, and fibroblasts were incubated with varying concentrations of sclerosants. Whole blood smears were stained with Giemsa and examined by light and bright field microscopy. Phalloidin and Hoechst stains were used to analyze cytoplasmic and nuclear morphology by fluorescence microscopy. Endothelial cell and fibroblasts were analyzed by live cell imaging. Higher concentrations of sclerosants induced cell lysis. Morphological changes in intact cells were observed at sublytic concentrations of detergents. Low concentration sodium tetradecyl sulfate induced erythrocyte acanthocytosis and macrocytosis, while polidocanol induced Rouleaux formation and increased the population of target cells and stomatocytes. Leukocytes showed swelling, blebbing, vacuolation, and nuclear degradation following exposure to sodium tetradecyl sulfate, while polidocanol induced pseudopodia formation, chromatin condensation, and fragmentation. Platelets exhibited pseudopodia with sodium tetradecyl sulfate and a "fried egg" appearance with polidocanol. Exposure to sodium tetradecyl sulfate resulted in size shrinkage in both endothelial cell and fibroblasts, while endothelial cell developed distinct spindle morphology. Polidocanol induced cytoplasmic microfilament bundles in both endothelial cell and fibroblasts. Patchy chromatin condensation was observed following exposure of fibroblasts to either agent. Detergent sclerosants are biologically active at sublytic concentrations. The observed morphological changes are consistent with cell activation, apoptosis, and oncosis. The cellular response is concentration dependent, cell-specific, and sclerosant specific. © The Author(s) 2015.

  7. Idiopathic intracranial hypertension: a possible complication in the natural history of advanced prostate cancer.

    Science.gov (United States)

    Valcamonico, Francesca; Arcangeli, Giuseppina; Consoli, Francesca; Nonnis, Daniela; Grisanti, Salvatore; Gatti, Enza; Berruti, Alfredo; Ferrari, Vittorio

    2014-03-01

    Idiopathic intracranial hypertension is a variety of intracranial hypertension that is extremely rare in men. Obesity and hypogonadism are the most important predictive factors. Etiological hypotheses include increased central venous pressure, and various hormonal and metabolic changes commonly found in obese patients. We described the case of an obese man with prostate cancer who showed a consistent bodyweight increase during treatment with taxanes and prednisone. He was hospitalized because of a severe loss of vision as a consequence of idiopathic intracranial hypertension. A complete symptom remission was obtained after 3 weeks of anti-edema therapies (steroids, acetazolamide). Castration-resistant prostate cancer is a risk factor for idiopathic intracranial hypertension. Long-term androgen deprivation therapy, bodyweight increase, and fluid retention during chronic steroid administration and taxane chemotherapy might favor the disease onset. This severe complication has a good outcome, and should be suspected in the presence of symptoms and signs of intracranial hypertension. © 2013 The Japanese Urological Association.

  8. Unusual Sclerosing Orbital Pseudotumor Infiltrating Orbits and Maxillofacial Regions

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    Huseyin Toprak

    2014-01-01

    Full Text Available Idiopathic orbital pseudotumor (IOP is a benign inflammatory condition of the orbit without identifiable local or systemic causes. Bilateral massive orbital involvement and extraorbital extension of the IOP is very rare. We present an unusual case of IOP with bilateral massive orbital infiltration extending into maxillofacial regions and discuss its distinctive magnetic resonance imaging (MRI features that help to exclude other entities during differential diagnoses.

  9. Recurrence of primary sclerosing cholangitis in pediatric liver transplant recipients.

    Science.gov (United States)

    Venkat, Veena L; Ranganathan, Sarangarajan; Mazariegos, George V; Sun, Qing; Sindhi, Rakesh

    2014-06-01

    There is little detailed clinical information on recurrent primary sclerosing cholangitis (rPSC) after liver transplantation in children. Our purpose was to describe the characteristics of children who had experienced rPSC after liver transplantation so that we could identify potential risk factors for recurrence. Clinical information for pediatric patients undergoing transplantation for primary sclerosing cholangitis (PSC) was retrospectively reviewed, and variables related to the pretransplant diagnosis of PSC and posttransplant variables were abstracted. The studied variables included the following: cytomegalovirus/Epstein-Barr virus status, early/late rejection, induction regimen, immunosuppression in the first year, steroid-resistant rejection, diagnosis of inflammatory bowel disease, and human leukocyte antigen markers commonly associated with PSC. A diagnosis of rPSC was made on the basis of radiographic features, histology, or both. Twelve patients underwent liver transplantation for PSC between 1993 and 2012. Patients received tacrolimus for maintenance immunosuppression after induction with steroids (n = 6) or thymoglobulin (n = 6). Three patients were diagnosed with rPSC 44, 60, and 62 months after transplantation. A fourth patient underwent retransplantation for graft failure with features of both hepatic artery stenosis and rPSC. This patient had distinct histological features of rPSC in the second graft. Three of the 4 patients were 7 years old or younger at the diagnosis of PSC. The patient and graft survival rates were similar for the steroid and thymoglobulin groups. All 4 children with rPSC received steroid-free thymoglobulin induction. In conclusion, our observation of an association between thymoglobulin, and age less than 10 years at the diagnosis of PSC, and rPSC adds to the existing suggestion of a link between the immune environment and the pathogenesis of rPSC. Defining the natural history of rPSC and searching for the etiology and

  10. Echocardiographic evaluation of patients with subacute sclerosing panencephalitis

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    Derya Çimen

    2014-03-01

    Full Text Available Objective: Subacute sclerosing panencephalitis is a slowly progressive, inflammatory and neurodegenerative disease caused by virus infection in the central nervous system. Since there are a limited number of studies in the literature evaluating the cardiovascular functions of patients with SSPE, the present study evaluates the patients with SSPE using tissue Doppler echocardiography and compares them between the control group in order to shed some light on the subject. Methods: The study is a prospective observational study. 49 patients (17 female, 32 male with SSPE were included in the study. Patients were divided into two groups: Stage 2 (n=29 and Stage 3 (n=20. Echocardiographic data were compared with a control group of 26 which is the same average age. All children underwent a detailed echocardiography, which contained an M-mode, pulse Doppler and tissue Doppler imaging. Results: Sinus tachycardia ( >100 beats/min in children was detected in nineteen (38.7% patients. There were not significant differences between parameters of systolic and diastolic function of the heart. Stage 2 group, EF: 69.9±6.4; SF: 39.2±5.58; and MPI (mitral: 0.38±0.03 and MPI (tricuspid: 0.39±0.10. And in the Stage 3 group, EF: 68.5±7.0, SF: 37.8±5.34, MPI (mitral: 0.37±0.09 and MPI (tricuspid: 0.38±0.12. In the control group EF:70.96±5.54; SF:39.96±5.05 and MPI(mitral: 0.35±0.06 MPI (tricuspid:0.36±0.04 and statistically meaningful differences were not found between patients and control groups (p >0.05. Conclusion: Cardiac functions may be preserved and cardiac functions constitute no significant risks of mortality in the advanced stages of patients with Subacute sclerosing panencephalitis, which is a group of chronic and bedridden patients.

  11. Update on idiopathic intracranial hypertension.

    Science.gov (United States)

    Bruce, Beau B; Biousse, Valérie; Newman, Nancy J

    2011-08-01

    To provide an update on various features of idiopathic intracranial hypertension. Perspective. Selected articles on the epidemiology, clinical and imaging features, natural history, pathophysiology, and treatment of idiopathic intracranial hypertension were reviewed and interpreted in the context of the authors' clinical and research experience. Idiopathic intracranial hypertension primarily is a disease of obese women of childbearing age, but it can affect patients of any weight, sex, and age. Although a relatively rare disorder, idiopathic intracranial hypertension's associated costs in the United States entail hundreds of millions of dollars. Even after treatment, headaches frequently are persistent and may require the continued involvement of a neurologist. Quality-of-life reductions and depression are common among idiopathic intracranial hypertension patients. However, visual dysfunction, especially visual field abnormalities, represents the major morbidity of this disorder, and serial automated perimetry remains the primary method of patient monitoring. Patients who are men, black, very obese, or anemic are at higher risk of visual loss. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding the pathophysiology of idiopathic intracranial hypertension. Treatment studies show that lumbar puncture is a valuable treatment (in addition to its crucial diagnostic role), and that weight management is critical. However, open questions remain regarding the efficacy of acetazolamide, cerebrospinal fluid diversion procedures, and cerebral venous stenting. Many questions remain unanswered about idiopathic intracranial hypertension. Ongoing studies, especially an ongoing National Institutes of Health-funded clinical trial of acetazolamide, should provide more insight into this important, yet poorly understood, syndrome of isolated intracranial hypertension. Copyright © 2011

  12. Idiopathic pulmonary fibrosis

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    Noble Paul W

    2008-03-01

    Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

  13. Successful treatment of encephalopathy and myoclonus with levetiracetam in a case of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Becker, Daniel; Patel, Anup; Abou-Khalil, Bassel W; Pina-Garza, Jesus E

    2009-06-01

    Subacute sclerosing panencephalitis is a devastating progressive degenerative disease of the nervous system presumably caused by a persistent measles virus. Patients commonly present with myoclonia or encephalopathy. There are currently no known curative therapeutic options or effective symptomatic therapy. We treated a 12-year-old boy with subacute sclerosing panencephalitis who presented with acute encephalopathy and myoclonus. Electroencephalogram showed characteristic generalized periodic discharges. Levetiracetam produced dramatic improvement in both myoclonus and encephalopathy. The improvement was clear within 4 days. The electroencephalogram pattern showed improvement as well. Levetiracetam is a promising symptomatic therapy in subacute sclerosing panencephalitis for both the myoclonus and the encephalopathy. In this patient, it also appeared to improve the electroencephalographic pattern. We suggest that the generalized periodic discharges associated with the myoclonus contributed to the patient's encephalopathy.

  14. Large facial lymphatic malformation treatment using sclerosing agent followed by surgical resection: clinical and pathology report.

    Science.gov (United States)

    Laviv, Amir; Maly, Alexander; Abu-Tair, Jawad

    2017-07-22

    Lymphatic malformations are rare slow-flow vascular malformations, with high tendency to appear in the head and neck region. The treatment of these lesions ranges from follow-up to sclerosing agent injection to surgical excision. The authors present a case of a new born with large extensive lingual and submandibular lymphatic malformation, for which the patient underwent tracheostomy and gastrostomy insertion. He was then treated successfully with sclerosing agent injections followed by surgical excision, with 7 years follow-up. The second case presented is a two and a half baby with large lingual lymphatic malformation, treated successfully with doxycycline injections followed by intraoral excision of the lesion. Pathology of the excised lesion is then demonstrated, which shows for the first time the different layers affected by the sclerosing agent.

  15. Sclerosing encapsulating peritonitis in chronic ambulatory peritoneal dialysis;preoperative catheter drainage : a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Tae Hoon [Dankook Univ. Hospital, Seoul (Korea, Republic of)

    1996-10-01

    Sclerosing encapsulating peritonitis is a well recognized, but uncommon, complication of chronic ambulatory peritoneal dialysis. I report a case of sclerosing encapsulating peritonitis in which percutaneous catheter drainage was performed preoperatively. Ultrasonography(US) and computed tomography(CT) showed a large multi-septated cystic mass which occupied nearly all the peritoneal cavity. Percutaneous drainage with two 8.5 French catheters was preoperatively performed under fluoroscopy and about 2100 ml of bloody fluid was drained for 20 days. On follow-up CT, the size of the cyst had significantly decreased and anoperation was performed. It is considered that percutaneous catheter drainage is useful in the preoperative decompression of sclerosing encapsulating peritonitis.

  16. Idiopathic scoliosis and the vestibular system

    National Research Council Canada - National Science Library

    Hawasli, Ammar H; Hullar, Timothy E; Dorward, Ian G

    2015-01-01

    ... in the etiology of scoliosis. In this article, we discuss putative mechanisms for adolescent idiopathic scoliosis and review the current evidence supporting a role for the vestibular system in adolescent idiopathic...

  17. Idiopathic Thoracic Spinal Deformaties and Compensatory Mechanisms

    NARCIS (Netherlands)

    Hosman, A.J.F.

    2003-01-01

    Idiopathic scoliotic and kyphotic deformities have been described since Hippocrates. During the early twentieth-century fusion techniques were introduced, but only during the last decades innovations in the operative techniques have improved the surgical outcome of idiopathic spinal deformities.

  18. Sclerosing cholangitis by cytomegalovirus in highly active antiretroviral therapy era

    Directory of Open Access Journals (Sweden)

    Carmen Hidalgo-Tenorio

    2013-10-01

    Full Text Available Sclerosing colangitis (SC due to cytomegalovirus (CMV is very rare. It has been described mainly in immunocompromised patients. Currently, in HIV infected patients it is exceptional. The most of cases belong to pre-highly active antiretroviral therapy (pre-HAART and those cases were in stage AIDS with less than 100 CD4/μl. The most frequently involved pathogen in pre-HAART period was Cryptosporidium parvum (30-57% and CMV (10-30%; in late HAART period this information are unaware. CMV has been implicated as a possible etiological agent in primary SC partly because of the ability to cause liver damage and its relationship with smooth muscle antibodies. The most effective treatment for SC was the combination of antiretroviral therapy and endoscopic retrograde cholangiopancreatography with sphincterotomy and stent placement. Following, we present the first case of late HAART period which describes a SC extrahepatic without papillary stenosis with CMV as the only cause and clinical presentation of HIV infection in a woman with 177 CD4/μl.

  19. Primary Sclerosing Cholangitis – Diagnosis, Prognosis and Management

    Science.gov (United States)

    Singh, Siddharth; Talwalkar, Jayant A.

    2013-01-01

    Primary sclerosing cholangitis (PSC) is a chronic immune-mediated disease of the liver of unclear etiology, characterized by chronic inflammation and fibrosis of bile ducts. It primarily affects middle aged men, and is associated with 4-fold increased mortality as compared to ageand gender-matched population. Progressive biliary and hepatic damage results in portal hypertension and hepatic failure in a significant majority of patients over a 10–15 year period from initial diagnosis. In addition, PSC confers a markedly increased risk of hepatobiliary cancer, including cholangiocarcinoma and gallbladder cancer as compared to the general population, and cancer is the leading cause of mortality in patients with PSC. It is associated with inflammatory bowel disease in 70% patients, and increases the risk of colorectal cancer almost 10-fold. Despite significant research efforts in this field, the pathogenic mechanisms of PSC are still incompletely understood, although growing evidence supports the role of genetic and immunologic factors. There are no proven medical therapies that alter the natural course of the disease. Thus, liver transplantation is the only available treatment for patients with advanced PSC, with excellent outcomes in this population. PMID:23454027

  20. Geoepidemiology of primary sclerosing cholangitis: a critical review.

    Science.gov (United States)

    Tanaka, Atsushi; Takikawa, Hajime

    2013-10-01

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin, characterized by progressive destruction of bile ducts caused by diffuse inflammation and fibrosis. Previous epidemiological studies in Northern Europe and North America demonstrated that incidence and prevalence rates are ranging from 0.5 to 1.3 and from 3.85 to 16.2 per 100,000 inhabitants per year, respectively. It is of note that the incidence of PSC appears to be gradually increasing. We have extensively reviewed the geoepidemiology of PSC and attempted to place it in context with the incidence in Japan. In 2012, the clinical diagnostic criteria of IgG4-SC were established and published by the Japan Biliary Association, rendering it possible for physicians to clinically differentiate PSC from IgG4-SC. We conducted a new nationwide survey for PSC as well as IgG4-SC, and have identified 197 patients with PSC and 43 patients with IgG4-SC without pancreatic involvement. In this survey we estimated prevalence rate of PSC in Japan as 0.95, lower than those in North America and European countries. Also we identified other unique features of Japanese PSC patients, including 2 peaks in age distribution at diagnosis and fewer presences of comorbid inflammatory bowel diseases, occurring in only 34% of PSC. This data is placed in the perspective of the international experience on PSC. Copyright © 2013 Elsevier Ltd. All rights reserved.

  1. Cancer Surveillance in Patients with Primary Sclerosing Cholangitis

    Science.gov (United States)

    Razumilava, Nataliya; Gores, Gregory J.; Lindor, Keith D.

    2011-01-01

    Primary sclerosing cholangitis (PSC) is a chronic fibroinflammatory syndrome involving the biliary tract, often accompanied by inflammatory bowel disease. This syndrome is a prototype disease linking chronic inflammation to carcinogenesis. Indeed, PSC is associated with an increased risk of cholangiocarcinoma, gallbladder cancer, hepatocellular carcinoma, and colorectal cancer. Herein, we review the risk for these malignancies in PSC and discuss rational cancer surveillance strategies for these patients. Where evidence is limited, we suggest a pragmatic approach. In this regard we recommend interval screening for cholangiocarcinoma with non-invasive imaging modalities and serum carbohydrate antigen 19-9 determinations annually. These imaging studies also serve to screen for gallbladder cancer and hepatocellular carcinoma. Screening for colorectal cancer is more firmly established in PSC patients with inflammatory bowel disease and includes colonoscopy at the time of PSC diagnosis and, thereafter, at 1-2 year intervals. We also highlight areas where more information is required such as management of biliary tract dysplasia and cancer chemoprevention in PSC. PMID:21793028

  2. Bile duct basement membrane thickening in primary sclerosing cholangitis.

    Science.gov (United States)

    Colling, Richard; Verrill, Clare; Fryer, Eve; Kartsonaki, Christiana; Wang, Lai M; Chapman, Roger; Rajabally, Naayil; Fleming, Kenneth

    2016-05-01

    Primary sclerosing cholangitis (PSC) is characterized histologically by portal inflammation, bile duct injury and regeneration and concentric periductal fibrosis. Although seen commonly in our experience, the significance of histological thickening of the bile duct basement membrane on periodic acid Schiff (PAS)-positive, diastase-resistant (DPAS) staining has never been analysed formally. In this paper we provide an evidence-based assessment of basement membrane thickening (BMT) reproducibility and diagnostic accuracy. A total of 128 archived medical liver core biopsies were retrieved and blinded for review by two independent histopathologists. BMT was assessed and designated as absent or present with a grade (G) of G1-G3. The sensitivity of any BMT for PSC was good at 77%, with moderate specificity at 61%. When only G3 BMT was considered positive, the specificity was high at 95% but the sensitivity was poor at 16%. The interobserver agreement (0.69) and consistency (0.72) were good. Basement membrane thickening is a reproducible predictor for PSC with good sensitivity and specificity. The presence of G2 and especially G3 BMT showed high specificity and could be regarded as highly predictive of PSC. The presence of more than G1 BMT should be reported and the possibility of PSC should be raised in the differential diagnosis. © 2015 John Wiley & Sons Ltd.

  3. Epigenetics in the Primary Biliary Cholangitis and Primary Sclerosing Cholangitis

    Science.gov (United States)

    Cheung, Angela C.; LaRusso, Nicholas F.; Gores, Gregory J.; Lazaridis, Konstantinos N.

    2017-01-01

    Epigenomics, the study of modifications to genetic material that do not alter the underlying DNA sequence, is generating increasing interest as a means to help clarify disease pathogenesis and outcomes. Although genome-wide association studies have identified several potential candidate genes that may be implicated in primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), it is estimated that these genes explain less than 20% of the heritability of these diseases. Thus, to date, the origins of “missing heritability” for PBC and PSC remain elusive. The epigenome may provide a potentially elegant solution to this phenomenon, as it can be modified by both internal and external exposures (coined the “exposome”). This may explain differences in disease presentation, treatment response, and rates of progression between individuals. Epigenetic changes may also provide a framework for discovering potential biomarkers for diagnosis and screening of PBC and PSC. Importantly, because the epigenome is modifiable, it may also highlight novel pathways for therapeutic discovery and interventions of these diseases. PMID:28564724

  4. Clinical efficacy of cyclophosphamide in treatment of primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    CHENG Peng

    2013-12-01

    Full Text Available ObjectiveTo investigate the clinical efficacy of cyclophosphamide in the treatment of primary sclerosing cholangitis (PSC. MethodsTwenty-four patients with PSC who received treatment in the department of gastroenterology in our hospital from January 2004 to December 2012 were selected as subjects and divided into observation group (n=13 and control group (n=11. The control group was treated with ursodesoxycholic acid, while the observation group with ursodesoxycholic acid plus cyclophosphamide. After four weeks of treatment, clinical outcome, changes in serum bilirubin levels, and adverse reactions were compared between the two groups. Categorical data were analyzed by Fisher exact probability test, and continuous data by independent-samples t test and paired-samples t test. ResultsCompared with the control group, the observation group had a significantly higher response rate (84.62% vs 45.45%, P=0.0489, significantly more improvements in serum total and direct bilirubin levels (t=12.45, P<0.001; t=5.60, P<0.001, and a nonsignificantly higher incidence of adverse reactions (23.08% vs 18.18%, P=0.741. ConclusionCyclophosphamide has significant clinical efficacy and tolerable incidence of adverse events in the treatment of PSC, so it is worthy of application in clinical treatment.

  5. Pathogenesis and clinical spectrum of primary sclerosing cholangitis

    Science.gov (United States)

    Gidwaney, Neelam G; Pawa, Swati; Das, Kiron M

    2017-01-01

    Primary sclerosing cholangitis (PSC) is a disease of the biliary tract, which has been documented in the literature since 1867. This disease has a strong predilection for affecting men and can be seen in individuals as young as 2 years of age. PSC has a strong associated with inflammatory bowel disease, more commonly with ulcerative colitis, and is also part of the clinical spectrum of IgG4-related diseases. Small-duct PSC, a variant of PSC, also has an association with inflammatory bowel disease. The exact pathogenesis of PSC is not well understood at present, however, is likely a combination of a genetic predisposition with alteration of the molecular structure of the gut. Abnormal serum liver chemistry and presence of certain autoimmune markers are usually the first indicators leading to a diagnosis of PCS, however, these may often be normal in early stages of this disease. The diagnosis is made by cholangiography, which is now considered the gold standard. PSC is a known pre-malignant condition. Such patients have an increased risk of developing cholangiocarcinoma, gallbladder neoplasia, and colon cancer. Many new treatment modalities have emerged in the recent past, including anti-tumor necrosis factor- α and anti-integrins; however, liver transplantation is the only known cure for PSC. Despite past and present research, PSC remains an enigmatic biliary disease with few viable treatment options. PMID:28465630

  6. Sclerosing thymoma: case report Timoma esclerosante: relato de caso

    Directory of Open Access Journals (Sweden)

    Gesine Gregorio Siqueira

    2012-08-01

    Full Text Available We present a rare case of thymoma in a 36-year old woman, who was initially diagnosed with severe myasthenia gravis and subsequently undergone surgical resection. During surgery tumor was found at the anterior mediastinum, tightly attached to the phrenic nerve, pleura and pericardium. Histological assessment showed large areas of sclerosis and fibrous collagenous tissue as well as islands of epithelial and lymphoid cells. Sclerosing thymoma, which is a rare subtype of thymoma (Relatamos um caso raro de timoma em uma mulher de 36 anos de idade, com clínica e diagnóstico de miastenia gravis de difícil controle clínico, submetida à ressecção cirúrgica. No intraoperatório, observou-se tumor no mediastino anterior, firmemente aderido ao nervo frênico, à pleura e ao pericárdio. Ao exame histológico, foram evidenciadas extensas áreas de tecido fibrocolagenoso e esclerose, assim como ilhas de células epiteliais e células linfoides. Diagnosticado timoma esclerosante, subtipo raro de timoma (< 1%, sendo este o primeiro caso relatado no Brasil. A paciente apresentou melhora parcial dos sintomas associados à miastenia gravis.

  7. PD-1 gene polymorphism in children with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Piskin, Ibrahim Etem; Calık, Mustafa; Abuhandan, Mahmut; Kolsal, Ebru; Celik, Sevim Karakas; Iscan, Akın

    2013-08-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive inflammatory and degenerative disorder of the central nervous system. Several factors influence the risk of chronic brain infection with the mutant measles virus. However, to date, no pathogenic mechanism that may predispose to SSPE has been determined. Studies have indicated that specific polymorphisms in certain host genes are probably involved in impairing the ability of host immune cells to eradicate the measles virus in SSPE patients. Programmed cell death protein 1 (PD-1), a member of the CD28 family, is a negative regulator of the immune system. The purpose of our study was to investigate whether PD-1 gene polymorphisms affect susceptibility to the development of SSPE in Turkish children. In total, 109 subjects (54 SSPE patients and 55 healthy controls) were genotyped for the PD-1.9 C/T (rs2227982) single-nucleotide polymorphism (SNP). The distributions of T alleles in the PD-1.9 polymorphism in SSPE patients and healthy controls were 2.8 and 10.9%, respectively. There was a statistically significant difference between the groups; the 95% confidence interval (CI) was 0.06 to 0.85 and the odds ratio (OR) was 0.23 (χ(2) test). Thus, we identified an association between SSPE and the PD-1 rs2227982 gene polymorphism; the frequency of T alleles was higher in controls than in SSPE patients. Georg Thieme Verlag KG Stuttgart · New York.

  8. Cerebrospinal fluid apolipoprotein E levels in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Yüksel, Deniz; Ichiyama, Takashi; Yilmaz, Deniz; Anlar, Banu

    2012-04-01

    Neurofibrillary tangles (NFTs) have been shown in 20% of subacute sclerosing panencephalitis (SSPE) cases. NFTs contain paired helical filaments formed by hyperphosphorylated tau. The intraneuronal tau metabolism and the rate of formation of paired helical filaments can be regulated by interactions between tau and isoforms of Apolipoprotein E (Apo E). Tau binds in vitro to Apo E3, interferes with the hyperphosphorylation of tau and may reduce the formation of NFTs. We investigated cerebrospinal fluid (CSF) Apo E levels in SSPE (n=37) and age-matched control (n=38) groups. The median level of total Apo E and Apo E4 were lower in the SSPE than the control group (p<0.001 and p=0.002). On the other hand, median Apo E3 level (0.28±0.23 μg/ml) was higher in the SSPE group (p<0.001). Such elevated levels of ApoE3 might play a role in controlling the formation of NFTs in SSPE. Because NFT-associated neurodegeneration is a slow process, comparison of the long-term clinical course of SSPE cases with high and low Apo E3 levels might provide further understanding or the role of these molecules in this disease, and help the planning of neuroprotective treatment. Copyright © 2011 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  9. Neuronal loss is an early component of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Yüksel, Deniz; Diren, Barış; Ulubay, Hakan; Altunbaşak, Sakir; Anlar, Banu

    2014-09-02

    We performed diffusion tensor imaging (DTI) and magnetic resonance spectroscopy (MRS) studies in a group of patients with subacute sclerosing panencephalitis (SSPE) in order to estimate the pathologic process underlying the phenotypic variability. Patients with SSPE who had MRI including DTI and MRS examinations were evaluated according to their clinical status as determined by the SSPE Scoring System and their mental age as determined by tests appropriate for age and developmental level. Comparisons of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values and metabolite ratios of frontal periventricular white matter, parieto-occipital periventricular white matter, and globus pallidus in both hemispheres were made between control and SSPE groups, and between SSPE subgroups. Control (n = 18) and SSPE (n = 39) groups differed in all DTI and MRS parameters except FA, choline (Cho), and Cho/creatine (Cr). SSPE cases had higher ADC and lower N-acetylaspartate (NAA), NAA/Cho, and NAA/Cr in all regions of interest, suggesting cell loss. Disease progression rate and neurologic deficit appeared to be associated with the degree of ADC elevation and NAA reduction: the group with severe global deterioration had the lowest NAA (230.75 ± 197.97 in forceps minor), and rapid progression was associated with acute reduction in NAA. The combination of MRS and diffusion MRI findings suggests neuronal loss can be a primary target in rapidly or subacutely progressing SSPE, and preservation or regeneration of axonal structure may be beneficial in chronic cases. © 2014 American Academy of Neurology.

  10. Granzyme B gene polymorphism associated with subacute sclerosing panencephalitis.

    Science.gov (United States)

    Yentur, Sibel P; Aydin, Hatice Nur; Gurses, Candan; Demirbilek, Veysi; Kuru, Umit; Uysal, Serap; Yapici, Zuhal; Baris, Safa; Yilmaz, Gülden; Cokar, Ozlem; Onal, Emel; Gokyigit, Ayşen; Saruhan-Direskeneli, Güher

    2014-10-01

     Subacute sclerosing panencephalitis (SSPE) is a late complication of measles infection. Immune dysfunction related to genetic susceptibility has been considered in disease pathogenesis. A functional single nucleotide polymorphism (SNP) of granzyme B gene (GZMB) reported in several pathologies may also be involved in susceptibility to SSPE.  An SNP (rs8192917, G → A, R→Q) was screened in 118 SSPE patients and 221 healthy controls (HC) by polymerase chain reaction-restriction fragment length polymorphism. Frequencies were compared between groups. In vitro production of GZMB was measured in controls with different genotypes.  The SNP had a minor allele (G) frequency of 0.22 in patients and 0.31 in controls. GG genotype was significantly less frequent in patients (odds ratio, 0.23). G allele carriers produced relatively higher levels of GZMB, when stimulated in vitro.  These findings implicate possible effect of this genetic polymorphism in susceptibility to SSPE which needs to be confirmed in bigger populations. Georg Thieme Verlag KG Stuttgart · New York.

  11. Idiopathic Retroperitoneal Hematoma

    Directory of Open Access Journals (Sweden)

    Tomoyuki Abe

    2010-09-01

    Full Text Available A 34-year-old female presented with sudden onset of severe abdominal pain in a flank distribution. A large mass was palpable in the right upper quadrant on physical examination. Abdominal contrast-enhanced computed tomography showed a well-defined, right-sided, retroperitoneal cystic lesion located between the abdominal aorta and the inferior vena cava (IVC. The tumor size was 55 × 58 mm, and it compressed the gallbladder and the duodenum. Upper gastrointestinal radiography revealed a stricture of the second portion of the duodenum by the tumor. T2-weighted magnetic resonance imaging showed that the whole part was hyperintense with hypointense rims, but the inner was partially hypointense. Based on the radiological findings, the preoperative differential diagnosis included retroperitoneal teratoma, Schwannoma, abscess, and primary retroperitoneal tumor. On laparotomy, the tumor was located in the right retroperitoneal cavity. Kocher maneuver and medial visceral rotation, which consists of medial reflection of the upper part of right colon and duodenum by incising their lateral peritoneal attachments, were performed. Although a slight adhesion to the IVC was detected, the tumor was removed safely. Thin-section histopathology examination detected neither tumor tissues nor any tissues such as adrenal gland, ovarian tissue, or endometrial implants. The final pathological diagnosis was idiopathic retroperitoneal hematoma; the origin of the bleeding was unclear. The patient was discharged without any complication 5 days after the operation.

  12. Idiopathic Fascicular Ventricular Tachycardia

    Directory of Open Access Journals (Sweden)

    Johnson Francis

    2004-07-01

    Full Text Available Idiopathic fascicular ventricular tachycardia is an important cardiac arrhythmia with specific electrocardiographic features and therapeutic options. It is characterized by relatively narrow QRS complex and right bundle branch block pattern. The QRS axis depends on which fascicle is involved in the re-entry. Left axis deviation is noted with left posterior fascicular tachycardia and right axis deviation with left anterior fascicular tachycardia. A left septal fascicular tachycardia with normal axis has also been described. Fascicular tachycardia is usually seen in individuals without structural heart disease. Response to verapamil is an important feature of fascicular tachycardia. Rare instances of termination with intravenous adenosine have also been noted. A presystolic or diastolic potential preceding the QRS, presumed to originate from the Purkinje fibers can be recorded during sinus rhythm and ventricular tachycardia in many patients with fascicular tachycardia. This potential (P potential has been used as a guide to catheter ablation. Prompt recognition of fascicular tachycardia especially in the emergency department is very important. It is one of the eminently ablatable ventricular tachycardias. Primary ablation has been reported to have a higher success, lesser procedure time and fluoroscopy time

  13. Idiopathic Acute Eosinophilic Pneumonia

    Directory of Open Access Journals (Sweden)

    Kuan-Ting Liu

    2006-07-01

    Full Text Available Idiopathic acute eosinophilic pneumonia (IAEP is a rare disease but of clinical importance because of its good prognosis if treated promptly and appropriately. The etiology remains unknown and the temporal relationship between IAEP and a history of resent onset of cigarette smoking has been described. We report a typical case of a 21-year-old male with recent onset of smoking, who presented with acute febrile hypoxemic respiratory failure. High-resolution chest computed tomography scan revealed patchy ground glass opacity and ill-defined nodules, diffuse interlobar and interlobular septal thickening, and bilateral small amount of pleural effusion, which mimicked congestive heart failure except that the heart size was within normal limits. Bronchoalveolar lavage (BAL was performed soon after the patient was admitted and remarkable eosinophilia was noted in BAL fluid. Clinical condition and chest radiographs improved dramatically after cor-ticosteroid treatment. Because effective treatment and prompt institution of therapy can obviate unnecessary morbidity and mortality, IAEP should be kept in mind when treating patients presenting with diffuse parenchymal lung disease and acute respiratory failure. In that case, BAL is valuable and should be performed as soon as possible.

  14. A Localized Sclerosing Osteomyelitis at the Periapex of a Vital Tooth: Report of a Misdiagnosis

    Science.gov (United States)

    Owlia, Fatemeh; Akhavan Karbassi, Mohammad-Hassan; Mirjalili, Narges; Taghipour Zahir, Shokouh; Gholami, Neda; Karimi, Sharare

    2011-01-01

    Osteomyelitis is an inflammatory infectious condition of bones, occurringeither acutely or chronically. The clinical course of the disease leads to destructive or sclerosing patterns of the involved bone. This report presents a case of chronic focal sclerosing osteomyelitis in a 19-year-old male with a history of an uncontrolled convulsive condition. The lesion was first diagnosed as an osteoblastoma. Chronic trauma or traumatic occlusion has the potential to induce osteomyelitis and should be considered a possible diagnosis in differentiating periapical radiopacities, even in relation with vital teeth. PMID:22991615

  15. A Localized Sclerosing Osteomyelitis at the Periapex of a Vital Tooth: Report of a Misdiagnosis

    Directory of Open Access Journals (Sweden)

    Fatemeh Owlia

    2011-09-01

    Full Text Available Osteomyelitis is an inflammatory infectious condition of bones, occurring either acutely or chronically. The clinical course of the disease leads to destructive or sclerosing patterns of the involved bone. This report presents a case of chronic focal sclerosing osteomyelitis in a 19-year-old male with a history of an uncontrolled convulsive condition. The lesion was first diagnosed as an osteoblastoma. Chronic trauma or traumatic occlusion has the potential to induce osteomyelitis and should be considered a possible diagnosis in differentiating periapical radiopacities, even in relation with vital teeth.

  16. Rapidly progressive subacute sclerosing panencephalitis presenting with acute loss of vision.

    Science.gov (United States)

    Ekici, Bariş; Calişkan, Mine; Tatli, Burak; Aydinli, Nur; Ozmen, Meral

    2011-12-01

    A 10-year-old male presented with vision loss and behavioral changes. He had midpoint pupils with no reaction to light and normal funduscopic examination. Cranial magnetic resonance imaging revealed bilateral cortical lesions at parieto-occipital lobes. Elevated measles antibody titers in the cerebrospinal fluid confirmed the diagnosis of subacute sclerosing panencephalitis. Despite oral inosiplex and supportive care, patient developed generalized seizures with frequent myoclonic jerks and rapidly progressed into coma. Cortical blindness in subacute sclerosing panencephalitis can be an early indicator for fulminant course.

  17. Evidence of nerve sheath differentiation and high grade morphology in sclerosing epithelioid fibrosarcoma

    Science.gov (United States)

    Hanson, I; Pearson, J; Eyden, B; Slawik, S; Harris, M

    2001-01-01

    Sclerosing epithelioid fibrosarcoma is a recently described sarcoma in which ultrastructural evidence of fibroblastic differentiation forms part of the diagnostic criteria. This report describes a further case of this tumour, which showed evidence of both fibroblastic and perineurial differentiation by immunohistochemistry and electron microscopy, and which had areas of high grade morphology. The tumour metastasised and the patient died of disease 12 months after presentation. The relevance of these findings to diagnosis and differentiation in these tumours is discussed. Key Words: sclerosing epithelioid fibrosarcoma • nerve sheath differentiation • high grade morphology PMID:11533083

  18. Idiopathic chondrolysl;s, of the hip

    African Journals Online (AJOL)

    Idiopathic chondrolysl;s, of the hip. ,~. A case report. E. J. SMITH,. D. T. NININ, A. C. KEAYS. Fig. 1. The initial radiograph of the right hip showing the features of idiopathic chondrolysis ... Case report. A report of a case of idiopathic chondrolysis of the ... the right hip, peri-articular osteoporosis, blurring of the femoral head and ...

  19. Multiple idiopathic apical root resorption.

    Science.gov (United States)

    Kanungo, Manish; Khandelwal, Vishal; Nayak, Ullal Anand; Nayak, Prathibha Anand

    2013-04-23

    Idiopathic external root resorption is a rarely reported condition which has been observed in single or multiple teeth. This is a rare case of multiple idiopathic apical root resorption (MIARR) in a 16-year-old boy. External root resorption of the permanent teeth is a multifactorial process. Well-recognised causes of apical root resorption in permanent teeth include orthodontic therapy, trauma, periapical or periodontal inflammation, tumours, cysts, occlusal stresses, impacted teeth, systemic conditions, endocrine imbalances and dietary habits. When none of these causes are present, it is termed idiopathic root resorption which may be either cervical or apical. MIARR is a rare condition which is usually detected as an incidental radiographic finding. However, it may cause pain and mobility in severe cases.

  20. Idiopathic polyhydramnios and fetal gender.

    Science.gov (United States)

    Stanescu, A D; Banica, R; Olaru, G; Ghinda, E; Birdir, Cahit

    2015-05-01

    To determine the relationship between idiopathic polyhydramnios and fetal gender in the absence of fetal or maternal abnormalities. This was a retrospective population-based register study. 295 women with singleton pregnancies complicated by idiopathic polyhydramnios (amniotic fluid index (AFI) higher than 24 cm) who were delivered at our institution from January 2002 till December 2012 were included. Only pregnancies with an uncomplicated outcome were accepted in this study. The incidence of the male to female fetuses was compared with the one in the general population. Among pregnancies complicated by idiopathic polyhydramnios, the following gender distribution was found: 72.9% male and 37.1% female. The distribution in the general population was 51.5% female and 48.5% male. The mean AFI was significantly increased in male fetuses (p polyhydramnios is more frequent in male normal fetuses than in female ones.

  1. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype...... or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant...

  2. Adolescent idiopathic scoliosis: current perspectives

    Directory of Open Access Journals (Sweden)

    Miyanji F

    2014-01-01

    Full Text Available Firoz MiyanjiBritish Columbia Children's Hospital, Vancouver, BC, CanadaAbstract: Adolescent idiopathic scoliosis (AIS remains a common and potentially severe musculoskeletal disorder. Although its etiology is largely unknown, ongoing research endeavors continue to improve our understanding of its potential origin and its natural history. Advances in understanding its true three-dimensional nature have sought to improve classification and management strategies. This review will attempt to highlight and give an overview of the current concepts in adolescent idiopathic scoliosis.Keywords: AIS, current, natural history, management strategies

  3. Papillary Thyroid Carcinoma of a Diffuse Sclerosing Variant: Ultrasonographic Monitoring from a Normal Thyroid Gland to Mass Formation

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hye Seong; Han, Boo-Kyung; Shin, Jung Hee; Ko, Eun Young; Sung, Chang Ohk; Oh, Young Lyun; Song, Sang Yong [Sungkyunkwan University School of Medicine, Samsung Medical Center, Seoul (Korea, Republic of)

    2010-10-15

    A diffuse sclerosing variant of papillary thyroid carcinoma is uncommon and has a tendency for rapid growth and a higher incidence of cervical lymph node metastases. We experienced a case of a diffuse sclerosing variant of papillary thyroid carcinoma in a 48-year-old man. This case showed benign features on nitial ultrasonography and positron emission tomography (PET) scan. A new nodule was detected on follow-up ultrasonography that showed rapid enlargement. This case was confirmed by surgical excision. We herein describe the initial and follow-up ultrasonographic findings of a diffuse sclerosing variant of papillary thyroid carcinoma

  4. Performance of magnetic resonance elastography in primary sclerosing cholangitis.

    Science.gov (United States)

    Eaton, John E; Dzyubak, Bogdan; Venkatesh, Sudhakar K; Smyrk, Thomas C; Gores, Gregory J; Ehman, Richard L; LaRusso, Nicholas F; Gossard, Andrea A; Lazaridis, Konstantinos N

    2016-06-01

    Liver stiffness (LS) measured by magnetic resonance elastography (MRE) is emerging as an important biomarker in chronic liver diseases. We examined the diagnostic performance of MRE, factors associated with an increased LS and the prognostic value of LS as measured by MRE among patients with primary sclerosing cholangitis (PSC). We performed a retrospective review of 266 patients with PSC to examine whether LS was associated with the primary endpoint of hepatic decompensation (ascites, variceal hemorrhage and hepatic encephalopathy). The ability of MRE to differentiate stages of fibrosis was examined in a subset of patients who underwent a liver biopsy (n = 20). An LS of 4.93 kPa was the optimal point to detected F4 fibrosis (sensitivity, 1.00; 95% confidence interval (CI), 0.40-1.00; specificity, 0.94; 95%CI, 0.68-1.00). While a serum alkaline phosphatase primary endpoint (hazard ratio, 0.26; 95%CI, 0.01-1.33). However, LS was associated with the development of decompensated liver disease (hazard ratio, 1.55; 95%CI, 1.41-1.70). The optimal LS thresholds that stratified patients at a low, medium and high risk for hepatic decompensation were 6.0 kPa (respectively). Magnetic resonance elastography is able to detect cirrhosis with high specificity and an alkaline phosphatase <1.5 times the upper limit of normal makes the presence of advanced LS unlikely. Moreover, LS obtained by MRE is predictive of hepatic decompensation in PSC. © 2015 Journal of Gastroenterology and Hepatology Foundation and John Wiley & Sons Australia, Ltd.

  5. Serum and cerebrospinal fluid cytokine concentrations in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Aydin, Omer Faruk; Ichiyama, Takashi; Anlar, Banu

    2010-06-01

    Subacute sclerosing panencephalitis (SSPE) is a neurodegenerative disease due to persistent measles virus infection. Its immunopathogenesis is unknown. Tumor necrosis factor (TNF)-alpha, interleukin (IL)-2, IL-6, IL-10 and IL-4 concentrations were measured in cerebrospinal fluid (CSF) and serum samples from 30 SSPE patients and 19 control subjects by cytometric bead array. CSF and serum IFN-gamma, IL-12 and IL-18 levels were measured in 18 SSPE patients by ELISA. Serum IL-4 and IL-10 (p<0.001), CSF IL-4 (p<0.001) and IL-6 (p=0.049) concentrations were lower, and serum IL-2 concentrations, higher (p=0.001) in SSPE patients. Serum TNF-alpha and IL-6, CSF TNF-alpha, IL-10, and IL-2 concentrations were not different between SSPE and control groups. Serum IFN-gamma levels were higher in stage I and II than stage III patients (p<0.05), whereas there was no difference between stages in terms of other cytokines. The levels of Th2-type cytokines: IL-4, IL-6 and IL-10 were suppressed in our SSPE cases. This finding, along with relatively elevated IFN-gamma and IL-2 levels, may suggest more active effector T cells compared to regulatory T cells (Treg), especially induced Treg, in early disease. High serum IL-2 concentrations might indicate peripheral Th1 activation. Discrepancies between various reports in the literature should be examined in view of the ages, stage and treatments of the patients studied. The interplay of various cytokines or cellular systems which may vary over time and between patients. Studies of treatment measures favoring the preservation of the early inflammatory response may be of interest in SSPE. Copyright (c) 2009 Elsevier B.V. All rights reserved.

  6. Primary sclerosing epithelioid fibrosarcoma of bone: analysis of a series.

    Science.gov (United States)

    Wojcik, John B; Bellizzi, Andrew M; Dal Cin, Paola; Bredella, Miriam A; Fletcher, Christopher D M; Hornicek, Francis J; Deshpande, Vikram; Hornick, Jason L; Nielsen, G Petur

    2014-11-01

    Sclerosing epithelioid fibrosarcoma (SEF) is a rare, aggressive malignant neoplasm characterized by small nests and linear arrays of epithelioid cells embedded in a dense collagenous matrix. Very few primary SEFs of bone have been reported. Recognition is critical, as the dense extracellular collagenous matrix can be interpreted as osteoid, leading to misdiagnosis as-osteosarcoma. MUC4 and SATB2 are 2 recently characterized immunohistochemical markers for SEF and osteosarcoma, respectively. In reports to date, osteosarcomas are positive for SATB2 and negative for MUC4, whereas soft tissue SEFs have shown the opposite immunohistochemical profile (SATB2-/MUC4+). The purpose of this study was to characterize the clinicopathologic and immunohistochemical features of 8 primary SEFs of bone. The patients presented at a wide range of ages (25 to 73 y; median 52 y). Tumors mostly involved long bones of the extremities, with 3 cases involving the femur, 2 involving the ulna, and 1 involving the humerus. Other sites of involvement included the second rib (1) and the C6 vertebra (1). Follow-up information was available for 7 patients, 3 of whom developed metastases within 2 years of diagnosis. The other 4 patients were free of local recurrence or metastases at 1, 5, 12, and >84 months of follow-up, respectively. Radiographically, the tumors were predominantly lytic and poorly marginated. Histologically, 6 tumors showed pure SEF morphology, and 2 showed hybrid SEF/low-grade fibromyxoid sarcoma morphology. Focal dystrophic mineralization was seen in 1 case but was limited to areas of necrosis. None of the tumors showed the lace-like pattern of mineralization typical of osteosarcoma. The majority (6/8) of the tumors strongly expressed MUC4. SATB2 was negative in all but 1 case, which showed variable weak to moderate staining in ∼50% of nuclei. In general, the combination of morphology, MUC4 expression, and the absence of SATB2 expression was highly useful in arriving at the

  7. Natural history of primary sclerosing cholangitis and prognostic value of cholangiography in a Dutch population

    NARCIS (Netherlands)

    Ponsioen, C. Y.; Vrouenraets, S. M. E.; Prawirodirdjo, W.; Rajaram, R.; Rauws, E. A. J.; Mulder, C. J. J.; Reitsma, J. B.; Heisterkamp, S. H.; Tytgat, G. N. J.

    2002-01-01

    Background: Median survival of patients with primary sclerosing cholangitis (PSC) has been estimated to be 12 years. Cholangiography is the gold standard for diagnosis but is rarely used in estimating prognosis. Aims: To assess the natural history of Dutch PSC patients and to evaluate the prognostic

  8. Characterization of breast precancerous lesions and myoepithelial hyperplasia in sclerosing adenosis with apocrine metaplasia

    DEFF Research Database (Denmark)

    Celis, J.E.; Gromova, I.; Cabezón, T.

    2007-01-01

    that includes 15-prostaglandin dehydrogenase (15-PGDH), a protein that is expressed by all benign apocrine lesions, and markers that are highly overexpressed by pure invasive apocrine carcinomas such as MRP14 (S100A9), psoriasin (S100A7), and p53 to identify precancerous lesions in sclerosing adenosis (SA...

  9. Case report 834: Chronic sclerosing osteomyelitis of the mandible with long bone periostitis.

    Science.gov (United States)

    Stewart, A; Carneiro, R; Pollock, L; Shaw, D

    1994-04-01

    We present the case of a patient with primary chronic osteomyelitis of the mandible, the radiological appearance of which is compatible with a diagnosis of chronic sclerosing osteomyelitis. The accompanying femoral and tibial periosteal reactive new bone formation and the benign clinical course suggest that this presentation may represent a form of chronic multifocal osteomyelitis.

  10. A patient with hereditary hemochromatosis, ulcerative colitis, and primary sclerosing cholangitis: genetic aspects

    NARCIS (Netherlands)

    Ponsioen, C. Y.; Stokkers, P. C.; van der Horst, A. R.; Tytgat, G. N.; van Deventer, S. J.

    2001-01-01

    This report describes a family in which the rare combination of hereditary hemochromatosis, ulcerative colitis and primary sclerosing cholangitis was found. Subsequent to the index patient, who had all three diseases, a screening was done in his parents and siblings that included HLA-DR, HLA-DQ and

  11. Colorectal neoplasia in patients with primary sclerosing cholangitis undergoing liver transplantation

    DEFF Research Database (Denmark)

    Jørgensen, Kristin Kaasen; Lindström, Lina; Cvancarova, Milada

    2012-01-01

    OBJECTIVE: Several studies have implicated primary sclerosing cholangitis (PSC) as an additional risk factor for colorectal neoplasia in inflammatory bowel disease (IBD). Some reports have indicated that the risk is even higher in PSC-IBD patients after liver transplantation (Ltx), but this issue...

  12. Risk factors and prognosis for recurrent primary sclerosing cholangitis after liver transplantation

    DEFF Research Database (Denmark)

    Lindström, Lina; Jørgensen, Kristin K; Boberg, Kirsten M

    2018-01-01

    OBJECTIVES: The risk for recurrent primary sclerosing cholangitis (rPSC) after liver transplantation is associated with inflammatory bowel disease (IBD). We assessed the frequency of rPSC and studied risk factors for recurrent disease with special focus on IBD. We also evaluated the importance of r...

  13. Survival without biliary complications after liver transplant for primary sclerosing cholangitis.

    Science.gov (United States)

    Mogl, Martina T; Albert, Kathrin; Pascher, Andreas; Sauer, Igor; Puhl, Gero; Gül, Safak; Schönemann, Constanze; Neuhaus, Peter; Guckelberger, Olaf

    2013-12-01

    Patients who have a liver transplant for primary sclerosing cholangitis may develop recurrent disease and biliary complications, organ loss necessitating revision liver transplant, or death. We evaluated long-term outcomes in patients who had liver transplant for primary sclerosing cholangitis. In 71 patients who had a liver transplant for end-stage liver disease because of primary sclerosing cholangitis, a retrospective review was done to evaluate biliary complication-free survival, transplanted organ survival, and death. Human leukocyte antigen typing and matching were reviewed. There were 39 patients (55%) who had biliary complications, loss of the liver transplant, or death at a mean 12.1 years after transplant. The 5- and 10-year event-free survival reached 74.6% and 45% (53 patients after 5 years, and 32 patients after 10 years). Male sex of transplant recipients was a significant risk factor for biliary complications, revision liver transplant, or death. Most patients had inflammatory bowel disease, primarily ulcerative colitis. The human leukocyte antigen profile or number of mismatches had no effect on complication-free survival. Biliary complications, revision liver transplant, and death are a useful combined primary endpoint for recurrent primary sclerosing cholangitis after liver transplant.

  14. Immunosuppression after liver transplantation for primary sclerosing cholangitis influences activity of inflammatory bowel disease

    DEFF Research Database (Denmark)

    Jørgensen, Kristin Kaasen; Lindström, Lina; Cvancarova, Milada

    2013-01-01

    Previous studies have shown conflicting results regarding the course of inflammatory bowel disease (IBD) after liver transplantation in patients with primary sclerosing cholangitis (PSC). We studied the progression of IBD in patients with PSC who have undergone liver transplantation. We also...

  15. Deterioration of cholestasis after endoscopic retrograde cholangiography in advanced primary sclerosing cholangitis

    NARCIS (Netherlands)

    Beuers, U.; Spengler, U.; Sackmann, M.; Paumgartner, G.; Sauerbruch, T.

    1992-01-01

    Complications of endoscopic retrograde cholangiography specific to patients with primary sclerosing cholangitis have not yet been reported. We observed transient rises of serum bilirubin after diagnostic endoscopic retrograde cholangiography in five of 15 patients and persistent rises in three of 15

  16. Validation of the prognostic value of histologic scoring systems in primary sclerosing cholangitis

    DEFF Research Database (Denmark)

    de Vries, Elisabeth M G; de Krijger, Manon; Färkkilä, Martti

    2017-01-01

    Histologic scoring systems specific for primary sclerosing cholangitis (PSC) are not validated. We recently determined the applicability and prognostic value of three histological scoring systems in a single PSC cohort. The aim of this study was to validate their prognostic use and reproducibility...

  17. Alkaline phosphatase at diagnosis of primary sclerosing cholangitis and one year later: Evaluation of prognostic value

    NARCIS (Netherlands)

    de Vries, Elisabeth M. G.; Wang, Junfeng; Leeflang, Mariska M. G.; Boonstra, Kirsten; Weersma, Rinse K.; Beuers, Ulrich H.; Geskus, Ronald B.; Ponsioen, Cyriel Y.

    2016-01-01

    BACKGROUND: Primary sclerosing cholangitis (PSC) is a slowly progressive liver disease. Reliable biomarkers to predict outcome are urgently needed to serve as surrogate endpoints and/or stratifiers in clinical trials. Reduction of serum alkaline phosphatase (ALP) has been proposed as prognostic

  18. Alkaline phosphatase normalization is associated with better prognosis in primary sclerosing cholangitis.

    Science.gov (United States)

    Stanich, Peter P; Björnsson, Einar; Gossard, Andrea A; Enders, Felicity; Jorgensen, Roberta; Lindor, Keith D

    2011-04-01

    Primary sclerosing cholangitis results in elevated but fluctuating serum alkaline phosphatase levels that occasionally return to normal. To investigate the frequency of normalization of alkaline phosphatase in newly diagnosed primary sclerosing cholangitis patients and the subsequent clinical outcomes. Records of newly diagnosed primary sclerosing cholangitis patients were examined retrospectively for laboratory values and clinical end points (cholangiocarcinoma, liver transplantation and death) within 10 years of diagnosis. Data from a recent prospective ursodeoxycholic acid treatment trial were also studied. Eighty-seven patients met the inclusion criteria. Normalization of alkaline phosphatase was seen in 35 (40%) patients. Five (14%) patients with normalization reached an end point whereas 17 (33%) of the patients with persistent elevation reached an end point (P = 0.02). Ursodeoxycholic acid was used similarly by both groups. When the investigative criteria were applied to a prospective trial, there was again a significant relationship between normalization of alkaline phosphatase and survival in patients receiving ursodeoxycholic acid (P alkaline phosphatase was found to normalize in a high proportion of newly diagnosed primary sclerosing cholangitis patients. This was significantly associated with a better prognosis in a retrospective cohort and when data from a prospective treatment trial was evaluated. Copyright © 2010 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  19. Case Report and Molecular Analysis of Subacute Sclerosing Panencephalitis in a South African Child

    Science.gov (United States)

    Vardas, Eftyhia; Leary, P. M.; Yeats, Jane; Badrodien, Waseila; Kreis, Stephanie

    1999-01-01

    This is the first case of subacute sclerosing panencephalitis from South Africa in which the molecular characteristics of the causative measles virus were examined. The virus found is classified as genotype D3, which has not previously been found in Africa and was last circulating in the United States before 1992. PMID:9986851

  20. Subacute sclerosing panencephalitis (SSPE): an insight into the diagnostic errors from a tertiary care university hospital.

    Science.gov (United States)

    Prashanth, L K; Taly, A B; Sinha, S; Ravi, V

    2007-06-01

    Subacute sclerosing panencephalitis (SSPE) is a progressive disease caused by wild-type measles virus leading to premature death. Early diagnosis may help in medical interventions and counseling. The aim of this study was to ascertain diagnostic errors and their possible causes. Retrospective case record analysis of patients with subacute sclerosing panencephalitis, evaluated over a 10-year period, was performed. The following data were analyzed: initial symptoms and diagnosis, interval between onset of symptoms to diagnosis, and implications of delayed diagnosis. Among the 307 patients evaluated, initial diagnosis by various health care professionals was other than subacute sclerosing panencephalitis in 242 patients (78.8%). These included seizures, absence seizures, metachromatic leukodystrophy, Schilder's disease, cerebral palsy, hemiparkinsonism, Wilson's disease, vasculitis, spinocerebellar ataxia, motor neuron disease, nutritional amblyopia, tapetoretinal degeneration, catatonic schizophrenia, and malingering, among others. The interval between precise diagnosis and first reported symptom was 6.2 +/- 11.3 months (range, 0.2-96 months; median, 3 months). Forty-four patients (14.3%) who had symptoms for more than 1 year before the precise diagnosis had a protracted course as compared to the rest of the cohort ( P = .0001). Early and accurate diagnosis of subacute sclerosing panencephalitis needs a high index of suspicion.

  1. An unusual case of acute encephalitic syndrome: Is it acute measles encephalitis or subacute sclerosing panencephalitis?

    Science.gov (United States)

    Garg, Ravindra K; Malhotra, Hardeep S; Rizvi, Imran; Kumar, Neeraj; Jain, Amita

    2017-01-01

    Subacute sclerosing panencephalitis is a late complication of measles infection and develops usually 6 to 15 years after the primary measles infection. Fulminant subacute sclerosing panencephalitis is an infrequently encountered form wherein the disease rapidly progresses to death. A six-year old male child presented with fever, abnormal movements of the left side of body followed by weakness of the left side of the body, and involuntary abnormal movements of right upper and lower limbs. On examination, he was drowsy and was unable to communicate. He had right-sided hemiballismus. He also had left-sided hemiparesis and the left plantar reflex was extensor. Cerebrospinal fluid examination revealed elevated protein and cells. In the serum and cerebrospinal fluid, anti-measles IgG antibodies were found to be positive. No other viral marker was noted in the cerebrospinal fluid. Magnetic resonance imaging of the brain showed extensive damage to the right temporal, parietal, and to a lesser extent, the frontal region as well as subcortical structures of these regions. Electroencephalography revealed generalized slowing of waves. Over a period of the next 3 days, the intensity and frequency of choreiform movements markedly reduced and the patient developed periodic generalized myoclonus, which was predominantly present on the right side. The patient succumbed to his illness and died after one month. Fulminant subacute sclerosing panencephalitis may have unusual clinical manifestations such as hemiballismus. In fulminant subacute sclerosing panencephalitis, neuroimaging may show extensive cortical damage.

  2. Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Weismueller, Tobias J.; Trivedi, Palak J; Bergquist, Annika; Imam, Mohamad; Lenzen, Henrike; Ponsioen, Cyriel Y.; Holm, Kristian; Gotthardt, Daniel; Faerkkilae, Martti A.; Marschall, Hanns-Ulrich; Thorburn, Douglas; Weersma, Rinse K.; Fevery, Johan; Mueller, Tobias; Chazouilleres, Olivier; Schulze, Kornelius; Lazaridis, Konstantinos N.; Almer, Sven; Pereira, Stephen P.; Levy, Cynthia; Mason, Andrew L.; Naess, Sigrid; Bowlus, Christopher L.; Floreani, Annarosa; Halilbasic, Emina; Yimam, Kidist K.; Milkiewicz, Piotr; Beuers, Ulrich; Huynh, Dep K.; Pares, Albert; Manser, Christine N.; Dalekos, George N.; Eksteen, Bertus; Invernizzi, Pietro; Berg, Christoph P.; Kirchner, Gabi I.; Sarrazin, Christoph; Zimmer, Vincent; Fabris, Luca; Braun, Felix; Marzioni, Marco; Juran, Brian D.; Said, Karouk; Rupp, Christian; Jokelainen, Kalle; de Valle, Maria Benito; Saffioti, Francesca; Cheung, Angela; Trauner, Michael; Schramm, Christoph; Chapman, Roger W.; Karlsen, Tom H.; Schrumpf, Erik; Strassburg, Christian P.; Manns, Michael P.; Lindor, Keith D; Hirschfield, Gideon M.; Hansen, Bettina E.; Boberg, Kirsten M.

    BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. METHODS: We

  3. Mutational Characterization of the Bile Acid Receptor TGR5 in Primary Sclerosing Cholangitis

    NARCIS (Netherlands)

    Hov, Johannes R.; Keitel, Verena; Laerdahl, Jon K.; Spomer, Lina; Ellinghaus, Eva; ElSharawy, Abdou; Melum, Espen; Boberg, Kirsten M.; Manke, Thomas; Balschun, Tobias; Schramm, Christoph; Bergquist, Annika; Weismueller, Tobias; Gotthardt, Daniel; Rust, Christian; Henckaerts, Liesbet; Onnie, Clive M.; Weersma, Rinse K.; Sterneck, Martina; Teufel, Andreas; Runz, Heiko; Stiehl, Adolf; Ponsioen, Cyriel Y.; Wijmenga, Cisca; Vatn, Morten H.; Stokkers, Pieter C. F.; Vermeire, Severine; Mathew, Christopher G.; Lie, Benedicte A.; Beuers, Ulrich; Manns, Michael P.; Schreiber, Stefan; Schrumpf, Erik; Haeussinger, Dieter; Franke, Andre; Karlsen, Tom H.

    2010-01-01

    Background: TGR5, the G protein-coupled bile acid receptor 1 (GPBAR1), has been linked to inflammatory pathways as well as bile homeostasis, and could therefore be involved in primary sclerosing cholangitis (PSC) a chronic inflammatory bile duct disease. We aimed to extensively investigate TGR5

  4. [A hybrid lesion: Low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF)].

    Science.gov (United States)

    Kesrouani, Carole; Zemoura, Leila; Trassard, Martine; Laé, Marick

    2016-10-01

    We report the case of a 34-year-old woman presenting a 10cm axillary mass diagnosed as hybrid tumor low-grade fibromyxoid sarcoma/sclerosing epithelioid fibrosarcoma. Microbiopsy for morphological and immunohistochemical analyses was associated with molecular analysis (FISH and PCR) to confirm the diagnosis. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

  5. Generation of sclerosant foams by mechanical methods increases the foam temperature.

    Science.gov (United States)

    Tan, Lulu; Wong, Kaichung; Connor, David; Fakhim, Babak; Behnia, Masud; Parsi, Kurosh

    2017-08-01

    Objective To investigate the effect of agitation on foam temperature. Methods Sodium tetradecyl sulphate and polidocanol were used. Prior to foam generation, the sclerosant and all constituent equipment were cooled to 4-25℃ and compared with cooling the sclerosant only. Foam was generated using a modified Tessari method. During foam agitation, the temperature change was measured using a thermocouple for 120 s. Results Pre-cooling all the constituent equipment resulted in a cooler foam in comparison with only cooling the sclerosant. A starting temperature of 4℃ produced average foam temperatures of 12.5 and 13.2℃ for sodium tetradecyl sulphate and polidocanol, respectively. It was also found that only cooling the liquid sclerosant provided minimal cooling to the final foam temperature, with the temperature 20 and 20.5℃ for sodium tetradecyl sulphate and polidocanol, respectively. Conclusion The foam generation process has a noticeable impact on final foam temperature and needs to be taken into consideration when creating foam.

  6. Genetic and Epigenetic Abnormalities in Primary Sclerosing Cholangitis-associated Cholangiocarcinoma

    NARCIS (Netherlands)

    Timmer, Margriet R.; Beuers, Ulrich; Fockens, Paul; Ponsioen, Cyriel Y.; Rauws, Erik A.; Wang, Kenneth K.; Krishnadath, Kausilia K.

    2013-01-01

    Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiology, characterized by chronic inflammation of the biliary tree with subsequent fibrosis and cirrhosis of the liver. Patients with PSC are at increased risk for the development of cholangiocarcinoma (CCA), a highly

  7. Value of brush cytology for dominant strictures in primary sclerosing cholangitis

    NARCIS (Netherlands)

    Ponsioen, C. Y.; Vrouenraets, S. M.; van Milligen de Wit, A. W.; Sturm, P.; Tascilar, M.; Offerhaus, G. J.; Prins, M.; Huibregtse, K.; Tytgat, G. N.

    1999-01-01

    Around 10% of patients with primary sclerosing cholangitis (PSC) develop cholangiocarcinoma, which is cholangiographically often indistinguishable from a benign dominant stricture. The aim of the present study was to assess the value of brush cytology in discriminating between benign and malignant

  8. Antioxidant therapy in idiopathic oligoasthenoteratozoospermia

    Directory of Open Access Journals (Sweden)

    Ahmad Majzoub

    2017-01-01

    Conclusion: Additional randomized controlled studies are required to confirm the efficacy and safety of antioxidant supplementation in the medical treatment of idiopathic male infertility as well as the dosage required to improve semen parameters, fertilization rates, and pregnancy outcomes in iOAT.

  9. Perinatal Outcome in Idiopathic Polyhydramnios.

    Science.gov (United States)

    Lallar, Meenakshi; Anam Ul Haq; Nandal, Rajesh

    2015-10-01

    To study perinatal outcome in idiopathic polyhydramnios. Case-control study was conducted in 500 pregnant women with idiopathic polyhydramnios (study group) and 500 normal pregnant women (control group) attending the outpatient department of SHKM Medical College, Haryana. Perinatal outcomes were recorded in both the groups. Out of 500 cases with idiopathic polyhydramnios, maximum cases were diagnosed between 28 and 36 weeks of pregnancy (84.6 %), and maximum presented with mild polyhydramnios (82 %). In the study and control groups, there were no statistically significant differences in preeclampsia and gestational hypertension (p = 0.445 and p = 0.230). In the study and control groups, 74.6 and 79.6 % women, respectively, had normal vaginal delivery (p = 0.250). The study group recorded much higher number of preterm deliveries than the control group (54 %) (p = 0.000). In the study group, 51.8 % women had maternal complications, while in the control group, 13.6 % women had obstetrical complications. The study group recorded higher perinatal mortality (10.4 %) than the control group. Idiopathic polyhydramnios is associated with higher perinatal morbidity and mortality than normal pregnancy.

  10. Cough in idiopathic pulmonary fibrosis

    NARCIS (Netherlands)

    M.J.G. Van Manen (Mirjam J.G.); S.S. Birring (Surinder S.); C. Vancheri (Carlo); V. Cottin (Vincent); Renzoni, E.A. (Elisabetta A.); Russell, A.-M. (Anne-Marie); M.S. Wijsenbeek (Marlies)

    2016-01-01

    textabstractMany patients with idiopathic pulmonary fibrosis (IPF) complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF,

  11. CASE REPORT Idiopathic granulomatous mastitis

    African Journals Online (AJOL)

    Abstract. The rare condition of idiopathic granulomatous mastitis (IGM) is presented here, unusually, in a 54-year-old woman. IGM mimics breast carcinoma and further differentials include tuberculosis and fungal infections of the breast together with other chronic granulomatous conditions. Of note is its characteristic ...

  12. Familial idiopathic hypertrophic subaortic stenosis

    Directory of Open Access Journals (Sweden)

    Shah Lilam

    1979-01-01

    Full Text Available Echocardiographic features of idiopathic hypertrophic subaortic stenosis (IHSS are described. Systolic anterior motion (SAM of anterior mitral leaflet and asymetric septal hypertrophy (ASH are considered as the diagnostic criteria of IHSS. Effects of amyl nitrite and propranolol-a beta blocker are studied. Echocardio-graphic screening of family members revealed this as a case of fami-lial IHSS.

  13. Suspected levamisole intoxication in calves.

    Science.gov (United States)

    Müller, K R; Dwyer, C

    2016-07-01

    A group of 32 Friesian and four Hereford calves, 3-4 months old with body weights between 100-120 kg, were purchased from a weaner sale. On arrival at the property the Hereford calves were treated with a combination anthelmintic containing 2 g/L abamectin and 80 g/L levamisole hydrochloride. Shortly afterwards they developed tremors and frothing from the mouth, and two died overnight. The Friesian calves were treated with the same anthelmintic on the following day, when some showed hypersalivation and frothing from the mouth. Examination of the three most severely affected Friesian calves revealed severe nicotinic-type symptoms including hypersalivation, frothing from the mouth, muscle tremors, recumbency, rapid respiration, hyperaesthesia, and central nervous system depression. Other calves showed mild to moderate signs of intoxication including restlessness, tail switching, salivation, tremors, frequent defaecation, mild colic and jaw chomping. Two calves died shortly afterwards. An adverse drug event investigation revealed that the formulation and quality of the anthelmintic was within the correct specification, and that the drench gun was functioning correctly. Suspected levamisole intoxication due to a combination of possible overdosing, dehydration, and stress caused by transportation and prolonged yarding. Susceptibility to levamisole toxicity in New Zealand calves can be increased if factors like dehydration or stress are present. Levamisole has a narrow margin of safety, and overdosing in calves can easily occur if the dose rate is not based on their actual weight or health status.

  14. A novel biomimetic analysis system for quantitative characterisation of sclerosing foams used for the treatment of varicose veins.

    Science.gov (United States)

    Carugo, Dario; Ankrett, Dyan N; O'Byrne, Vincent; Willis, Sean; Wright, David D I; Lewis, Andrew L; Hill, Martyn; Zhang, Xunli

    2013-06-01

    A novel analysis system for the quantification of sclerosing foam properties under clinically relevant conditions was developed with the purpose of establishing a robust methodology for comparative characterisation of different foam formulations and production strategies. The developed biomimetic-inspired model comprised of 4 or 10 mm inner diameter polytetrafluoroethylene tubing, filled with a blood substitute and fixed to a platform with an adjustable inclination angle. Sclerosing foams were produced by mixing polidocanol with either atmospheric air or 100 % CO₂, using a double-syringe system method. Individual foams were injected into the tube, while videos were captured simultaneously. Videos were then transferred to an in-house computational foam analysis system (CFAS) which performed a sequence of semi-automated operations, allowing quantitative characterisation of sclerosing foam dynamic behaviour. Using CFAS, degradation rates of different foams were measured and the effect of gas composition, liquid sclerosant concentration and time delay between foam production and injection were evaluated.

  15. Multiple hepatic sclerosing hemangioma mimicking metastatic liver tumor successfully treated by laparoscopic surgery: Report of a case

    Directory of Open Access Journals (Sweden)

    Masaki Wakasugi

    2015-01-01

    Conclusion: Sclerosing hemangioma should be included among the differential diagnoses of multiple liver tumors in patients with colorectal cancer. Laparoscopic hepatectomy is useful for diagnostic therapy for undiagnosed multiple liver tumors.

  16. Alcohol consumption in patients with primary sclerosing cholangitis.

    Science.gov (United States)

    Hagström, Hannes; Stål, Per; Stokkeland, Knut; Bergquist, Annika

    2012-06-28

    To assess the alcohol drinking patterns in a cohort of primary sclerosing cholangitis (PSC) patients and the possible influence on the development of fibrosis. Ninety-six patients with PSC were evaluated with a validated questionnaire about a patient's lifetime drinking habits: the lifetime drinking history (LDH) questionnaire. In addition, clinical status, transient elastography and biochemistry values were analysed and registered. Patients were defined as having either significant or non-significant fibrosis. Significant fibrosis was defined as either an elastography value of ≥ 17.3 kPa or the presence of clinical signs of cirrhosis. Patients were divided into two groups depending on their alcohol consumption patterns; no/low alcohol consumption (one drink or unit/d) and moderate/high alcohol consumption (≥ 1 drink or unit/d). LDH data were calculated to estimate lifetime alcohol intake (LAI), current alcohol intake, drinks per year before and after diagnosis of PSC. We also calculated the number of episodes of binge-drinking (defined as consuming ≥ 5 drinks per occasion) in total, before and after the diagnosis of PSC. The mean LAI was 3882 units of alcohol, giving a mean intake after onset of alcohol consumption of 2.6 units per week. Only 9% of patients consumed alcohol equal to or more than one unit per day. Current alcohol intake in patients with significant fibrosis (n = 26) was less than in patients without significant fibrosis (n = 70), as shown by lower values of phosphatidylethanol (B-PEth) (0.1 μmol/L vs 0.33 μmol/L, respectively, P = 0.002) and carbohydrate-deficient transferrin (CDT) (0.88% vs 1.06%, respectively, P = 0.02). Self-reported LAI was similar between the two groups. Patients with significant fibrosis reduced their alcohol intake after diagnosis from 103 to 88 units per year whereas patients without fibrosis increased their alcohol intake after PSC diagnosis from 111 to 151 units/year. There were no correlations between

  17. Newly Diagnosed Colonic Adenocarcinoma: The Presenting Sign in a Young Woman with Undiagnosed Crohn’s Disease in the Absence of Primary Sclerosing Cholangitis and a Normal Microsatellite Instability Profile

    Directory of Open Access Journals (Sweden)

    Brett Matthew Lowenthal

    2017-01-01

    Full Text Available Ulcerative colitis has long been linked with an increased risk for colonic adenocarcinoma, whereas Crohn’s disease (CD has recently been reported to pose a similar increased risk. We report a 33-year-old healthy female with no family history who presented with abdominal pain and a colon mass. Histopathology revealed a moderately differentiated adenocarcinoma extending through the muscularis propria with metastatic lymph nodes and intact mismatch repair proteins by immunohistochemical expression and gene sequencing. The nonneoplastic grossly uninvolved background mucosa showed marked crypt distortion, crypt abscesses, CD-like lymphoid hyperplasia, transmural inflammation, and reactive epithelial atypia. Additional patient questioning revealed frequent loose stools since she was a teenager leading to diagnosis of a previously undiagnosed CD without primary sclerosing cholangitis (PSC. The adenocarcinoma is suspected to be related to the underlying CD. Newly diagnosed adenocarcinoma in a young female as the presenting sign for CD in the absence of PSC is extremely rare.

  18. Frequent overlap of active hepatitis in recurrent primary sclerosing cholangitis after living-donor liver transplantation relates to its rapidly progressive course.

    Science.gov (United States)

    Miyagawa-Hayashino, Aya; Egawa, Hiroto; Yoshizawa, Atsusi; Ueda, Yoshihide; Ichida, Takafumi; Ueno, Yoshiyuki; Uemoto, Shinji; Harada, Kenichi; Nakanuma, Yasuni

    2011-09-01

    Recurrence of primary sclerosing cholangitis after liver transplantation is a challenging issue. Liver pathologies of recurrent primary sclerosing cholangitis after living-donor liver transplantation have not been reported. Here, liver pathologies of explanted grafts and biopsies of 9 patients who underwent retransplantation for recurrent primary sclerosing cholangitis were compared with those of native livers. Recurrence was diagnosed in 13 of 36 patients for primary sclerosing cholangitis post-living-donor liver transplantation, and 9 of them underwent retransplantation. All explanted grafts revealed biliary cirrhosis with sclerosing cholangitis, and 6 patients had additional features of active hepatitis. Liver biopsies showed that 3 had active hepatitis in addition to fibrous cholangitis at recurrence of primary sclerosing cholangitis. Two developed active hepatitis later after the diagnosis of recurrence. In explanted grafts, in addition to extensive hilar lymphoplasmacytic cholangitis, 4 cases showed hilar xanthogranulomatous cholangitis. The latter was not evident in 7 native livers. Ductopenia was extensive in all native livers, although such changes were relatively mild in explanted grafts at retransplantation. Patients with recurrent primary sclerosing cholangitis developed progressive graft failure, and the interval between diagnosis of recurrence and retransplantation (mean, 3.2 years) was shorter than that between diagnosis of primary sclerosing cholangitis and first transplantation (mean, 7.7 years). The rather rapid deterioration of recurrent primary sclerosing cholangitis after liver transplantation may be related to the frequent overlap of active hepatitis. Copyright © 2011 Elsevier Inc. All rights reserved.

  19. A Review of Pinealectomy-Induced Melatonin-Deficient Animal Models for the Study of Etiopathogenesis of Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Man Gene Chi Wai

    2014-09-01

    Full Text Available Adolescent idiopathic scoliosis (AIS is a common orthopedic disorder of unknown etiology and pathogenesis. Melatonin and melatonin pathway dysfunction has been widely suspected to play an important role in the pathogenesis. Many different types of animal models have been developed to induce experimental scoliosis mimicking the pathoanatomical features of idiopathic scoliosis in human. The scoliosis deformity was believed to be induced by pinealectomy and mediated through the resulting melatonin-deficiency. However, the lack of upright mechanical spinal loading and inherent rotational instability of the curvature render the similarity of these models to the human counterparts questionable. Different concerns have been raised challenging the scientific validity and limitations of each model. The objectives of this review follow the logical need to re-examine and compare the relevance and appropriateness of each of the animal models that have been used for studying the etiopathogenesis of adolescent idiopathic scoliosis in human in the past 15 to 20 years.

  20. A Review of Pinealectomy-Induced Melatonin-Deficient Animal Models for the Study of Etiopathogenesis of Adolescent Idiopathic Scoliosis

    Science.gov (United States)

    Wai, Man Gene Chi; Jun, Wang William Wei; Yee, Yim Annie Po; Ho, Wong Jack; Bun, Ng Tzi; Ping, Lam Tsz; Man, Lee Simon Kwong; Wah, Ng Bobby Kin; Chiu, Wang Chi; Yong, Qiu; Yiu, Cheng Jack Chun

    2014-01-01

    Adolescent idiopathic scoliosis (AIS) is a common orthopedic disorder of unknown etiology and pathogenesis. Melatonin and melatonin pathway dysfunction has been widely suspected to play an important role in the pathogenesis. Many different types of animal models have been developed to induce experimental scoliosis mimicking the pathoanatomical features of idiopathic scoliosis in human. The scoliosis deformity was believed to be induced by pinealectomy and mediated through the resulting melatonin-deficiency. However, the lack of upright mechanical spinal loading and inherent rotational instability of the curvature render the similarity of these models to the human counterparts questionable. Different concerns have been raised challenging the scientific validity and limitations of each model. The objectives of this review follow the logical need to re-examine and compare the relevance and appropriateness of each of the animal models that have been used for studying the etiopathogenesis of adolescent idiopathic scoliosis in human in the past 15 to 20 years. PMID:25238413

  1. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo

    2013-01-01

    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can...... and magnetic resonance imaging data and obtained follow-up (FU) information on 77 of these patients over a mean duration of 4 years. The AIIDLs presented as a single lesion in 72 (80 %) patients and exhibited an infiltrative (n = 35), megacystic (n = 16), Baló (n = 10) or ring-like (n = 16) lesion appearance...... in 77 (86 %) patients. Additional multiple sclerosis (MS)-typical lesions existed in 48 (53 %) patients. During FU, a further clinical attack occurred rarely (23-35 % of patients) except for patients with ring-like AIIDLs (62 %). Further attacks were also significantly more often in patients...

  2. Epidemiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ley B

    2013-11-01

    Full Text Available Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence and public health impact (ie, health care costs and resource utilization. Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. Keywords: idiopathic pulmonary fibrosis, epidemiology, incidence, prevalence, mortality, risk factors

  3. Idiopathic chondrolysis of the hip

    Directory of Open Access Journals (Sweden)

    Shefali Gupta

    2015-01-01

    Full Text Available Idiopathic chondrolysis is a rare condition characterized by the ultimate loss of femoroacetabular articular cartilage seen in a child with no history of trauma, slipped capital femoral epiphysis, infection, prolonged immobilization, or any other previously described disorder. The diagnosis is often delayed secondary to the insidious onset of symptoms, progressive radiographic findings, and the absence of diagnostic laboratory test. Typical radiographic features include localized osteoporosis, subchondral erosions, femoral head changes, and reduction of the joint space. Later changes include complete loss of the joint space, subchondral cysts, trochanteric and epiphyseal physeal closure, osteophytes, and in severe cases, protrusio acetabuli, ankylosis, and osteoarthritis. Magnetic resonance imaging of the hip demonstrates cartilage loss, joint effusion, marrow edema, femoral and acetabular remodeling, significant regional muscle atrophy, and synovial enhancement. We report a case of Idiopathic chondrolysis of the hip in an 11-year-old Indian girl.

  4. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype or a...... antibiotic treatment, signs and symptoms of elevated intracranial pressure resolved completely. Syphilis is a rare, but very important, differential diagnosis that in this case was clinically indistinguishable from IIH....

  5. Epigenomics of idiopathic pulmonary fibrosis

    OpenAIRE

    Yang, Ivana V

    2012-01-01

    Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology. Development of IPF is influenced by both genetic and environmental factors. Gene-expression profiling studies have taught us quite a bit about the biology of this fatal disease, but epigenetic marks may be the missing link that connects the environmental exposure in genetically predisposed individuals to transcriptome changes associated with the development of IPF. This review will begin with an introduction to...

  6. Epigenetics of Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Yang, Ivana V.; Schwartz, David A.

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology. Development of IPF is influenced by both genetic and environmental factors. Recent work by our and other groups has identified strong genetic predisposition factors for the development of pulmonary fibrosis while cigarette smoke remains the most strongly associated environmental exposure risk factor. Gene expression profiling studies of IPF lung have taught us quite a bit about the biology of this fatal disease...

  7. Papilledema and idiopathic intracranial hypertension.

    Science.gov (United States)

    Friedman, Deborah I

    2014-08-01

    Papilledema is one of the most concerning physical examination findings in neurology: it has a broad differential diagnosis of intracranial (and occasionally spinal) pathology associated with increased intracranial pressure. Papilledema impairs axoplasmic flow within the optic nerves and compresses the optic nerves externally; it may lead to profound visual loss. Thus, detection of papilledema and assessment of visual function are essential to patient management. This article reviews the treatment of papilledema-related visual loss in pseudotumor cerebri syndrome, one of the most common causes of papilledema encountered by neurologists. Results from the Idiopathic Intracranial Hypertension Trial (IIHTT), the first randomized, double-masked, placebo-controlled trial for the treatment of patients with mild visual loss from idiopathic intracranial hypertension, were published in April 2014. The IIHTT provides the first evidence-based treatment recommendations, showing the benefit of acetazolamide and weight loss for improving visual status in patients with mild visual field loss from idiopathic intracranial hypertension. A detailed ophthalmic examination, including perimetry, is critical to the evaluation, treatment, and assessment of treatment response in patients with papilledema.

  8. Canine pododermatitis and idiopathic disease.

    Science.gov (United States)

    Breathnach, Rory M; Fanning, Shay; Mulcahy, Grace; Bassett, Hugh F; Jones, Boyd R

    2008-05-01

    Pododermatitis is a common inflammatory skin disease of dogs. As pedal lesions are reported in many canine dermatoses, a methodical series of diagnostic tests is required to establish the underlying aetiology. However, laboratory/ancillary investigations may prove unrewarding, prompting a diagnosis of idiopathic disease. Various hypotheses have been proposed to explain the pathogenesis of idiopathic pododermatitis including pedal conformation, trauma, immunosuppression, bacterial infection, furunculosis and dermal granuloma formation. Idiopathic pododermatitis accounts for 0.5% of all dermatology referrals to the authors' clinic. A sub-group within this population is characterised histopathologically by epidermal hyperplasia, hyperkeratosis, spongiosis, dermal oedema and perivascular aggregates of lymphocytes and plasma cells. The term lymphocytic-plasmacytic pododermatitis (LPP) has previously been proposed to reflect the histological appearance of such lesions. Affected dogs, although systemically well, characteristically have pruritus, erythema, swelling, pain and alopecia of the feet. Although non-responsive to antimicrobial therapy, antiparasitic agents and elimination diets, these dogs typically respond well to immunomodulatory therapy.

  9. [Physical therapy for idiopathic scoliosis].

    Science.gov (United States)

    Steffan, K

    2015-11-01

    The objective is the description and summary of the current state of idiopathic scoliosis treatment with physical therapy based on new scientific knowledge and concluded from more than 15 years of experience as a leading physician in two well-known clinics specializing in the conservative treatment of scoliosis. Based on current scientific publications on physical therapy in scoliosis treatment and resulting from the considerable personal experience gained working with conservative treatment and consulting scoliosis patients (as inpatients and outpatients), the current methods of physical therapy have been compared and evaluated. Physical therapy according to Schroth and Vojta therapy are at present the most common and effective methods in the physical treatment of idiopathic scoliosis. These methods can be applied during inpatient or outpatient treatment or intensified in the practice of specialized therapists. As there are only a few scientific studies on this subject, the author's findings are based mainly on his own experiences of the conservative treatment of idiopathic scoliosis. Athough these experiences are the results of over 15 years of working in the field of therapy, and the Schroth method in combination with corrective bracing presents highly promising results, it would nevertheless be desirable to conduct detailed scientific studies to verify the effectiveness of conservative treatment.

  10. Idiopathic polyhydramnios and postnatal findings.

    Science.gov (United States)

    Dorleijn, Desirée M J; Cohen-Overbeek, Titia E; Groenendaal, Floris; Bruinse, Hein W; Stoutenbeek, Philip

    2009-04-01

    Our objective was to investigate the outcome of neonates with idiopathic polyhydramnios in the first year after birth. All patients diagnosed in the Erasmus Medical Centre Rotterdam and the University Medical Centre Utrecht between January 2000 and April 2005 with idiopathic polyhydramnios were studied. The outcome variables included mode of delivery, pre-term delivery, birth weight, macrosomia, large-for-gestational-age (weight > p90), neonatal or infant mortality and infant morbidity at 1 year after delivery. These were related to antenatal findings, including the onset of polyhydramnios and ultrasonographic evidence of macrosomia (estimated fetal weight > p90). Polyhydramnios was diagnosed at a mean gestational age (+/- s.d.) of 31.0 +/- 4.9 weeks. The mean gestational age at birth (+/- s.d.) was 37.9 +/- 3.7 weeks. Macrosomia at birth was present in 25.3% (22/88). Sixty-three of 88 infants were in good health 1 year after birth; 20 infants had an abnormality and 5 children had died. Macrosomia and detection of polyhydramnios in the third trimester was associated significantly with a good outcome. In neonates with idiopathic polyhydramnios, abnormalities were detected during the first year of life in 28.4%. Detection of polyhydramnios in the second trimester and low or normal birth weight are risk factors for associated abnormalities.

  11. The challenges of liver transplantation in children with primary sclerosing cholangitis.

    Science.gov (United States)

    Venkat, Veena L; Ranganathan, Sarangarajan; Sindhi, Rakesh

    2015-03-01

    Primary sclerosing cholangitis (PSC) in children can progress to end-stage liver disease requiring liver transplantation. PSC poses many challenges beginning with evaluation and classification of ductal involvement and overlap syndromes, few options for medical management and unique risks in the post-transplant period. The construct that PSC may be an autoimmune disease is based on positive autoantibodies, association with inflammatory bowel disease, linkage to HLA type and overlap/autoimmune sclerosing cholangitis; however, PSC is not responsive to standard immunosuppression. Study of PSC and post-transplant outcomes in children may provide a unique background in which to study this challenging disease. This is particularly intriguing in the subset of patients diagnosed in the first decade of life, suggesting a strong link to predisposing genetic susceptibility and immune dysregulation. Long-term, multicenter effort is likely to be the only mechanism to study this rare disease in children and to improve outcomes in the future.

  12. [Association between Crohn's disease and primary sclerosing cholangitis in a 10 year old girl].

    Science.gov (United States)

    Muñoz Lozón, Ana; Iglesias Blázquez, Cristina; Menéndez Arias, Cristina; Domínguez Sánchez, Patricia

    2016-06-01

    A 10 year old girl with diarrhea, abdominal pain, weight loss and fever of one month and a half of evolution. Analytical and sonographic findings raised the possibility of inflammatory bowel disease. Endoscopy and histology showed findings consistent with Crohn's disease. Treatment was initiated with mesalazine and exclusive enteral nutrition. Later corticosteroid treatment, immunosuppressive drugs and ursodeoxycholic acid were added due to cholestasis and persistent hypergammaglobulinemia. Magnetic resonance cholangiography and liver biopsy confirmed the diagnosis of concomitant primary sclerosing cholangitis. The association between Crohn's disease and primary sclerosing cholangitis is rare, predominantly in males between 20 and 40 years old and it presents a great clinical variability. The confirmation of the diagnosis requires magnetic resonance cholangiography or endoscopic retrograde cholangiopancreatography. The prognosis is poor and there is no treatment to slow the progression of the disease. Sociedad Argentina de Pediatría.

  13. Current research on the treatment and diagnosis of primary sclerosing cholangitis

    Directory of Open Access Journals (Sweden)

    Huan-yan PENG

    2016-12-01

    Full Text Available Primary sclerosing cholangitis (PSC is a chronic autoimmune cholestatic liver disease, with intrahepatic and/or extrahepatic bile duct diffuse inflammation, fibrosis, focal or segmental stenosis as the main features. Eventually this disease leads to bile duct obstruction, biliary cirrhosis and liver failure. The detection and diagnosis rate of PSC are increasing year by year, thus PSC becomes the hot spot for the study of hepatic inflammatory diseases. In this paper, we especially put our focus on the recent progress about diagnosis and treatment of PSC, and some special cases, including primary sclerosing cholangitis-autoimmune hepatitis (PSCAIH, autoimmune pancreatitis (AIP, ductile of PCS. These should help clinicians make a appropriate diagnosis and give correct treatment for these diseases. DOI: 10.11855/j.issn.0577-7402.2016.11.15

  14. Brain CT and MRI findings of a long-term case of subacute sclerosing panencephalitis

    Energy Technology Data Exchange (ETDEWEB)

    Aoshiba, Kazunori; Ota, Kohei; Komatsuzaki, Satoshi; Kobayashi, Itsuro; Maruyama, Shoichi

    1987-11-01

    Our study involved a long-term case (ten years) of subacute sclerosing panencephalitis. The case began with a 23 year-old experiencing visual deterioration. During the course of his illness, amnesia, autism and abnormal behavior were observed without any myoclonus. On the electroencephalogram, periodic synclonous discharge was shown in the early stage of his illness and subsequently disappeared. The brain CT and the MRI disclosed diffuse lesions in both cortical and subcortical areas of the cerebral hemispheres. The location and spread of lesions were more clearly revealed by the MRI than the brain CT. These findings suggest that the MRI is more useful than the brain CT in the diagnosis of subacute sclerosing panencephalitis.

  15. Adult fulminant subacute sclerosing panencephalitis: pathological and molecular studies--a case report.

    Science.gov (United States)

    Souraud, J B; Faivre, A; Waku-Kouomou, D; Gaillard, T; Aouad, N; Meaudre, E; Wild, F T; Fouet, B; Soulard, R

    2009-01-01

    Subacute sclerosing panencephalitis is an uncommon progressive neurological disorder caused by a persistent defective measles virus, typically affecting children. We describe a case of fulminant subacute sclerosing panencephalitis in a 25-year-old male. Brain tissue biopsy showed histologic evidence of encephalitis with eosinophilic intranuclear inclusion bodies (Cowdry Type A and B), intracytoplasmic inclusion bodies, perivascular lymphoplasmacytic infiltration and gliosis. Immunohistochemical studies were positive using an anti-measles antibody. Reverse transcriptase-PCR detected measles virus RNA and phylogenetic analysis indicated a C2 genotype. The rare adult-onset form is often atypical and difficult to diagnose and should be included in the differential diagnosis of subacute "unexplained" neurological diseases and uncommon infectious disorders.

  16. Lymphocyte subsets at different stages of subacute sclerosing panencephalitis: a study with monoclonal antibodies.

    Science.gov (United States)

    Marrosu, M G; Cianchetti, C; Ennas, M G

    1986-01-01

    Lymphocyte subsets in cerebrospinal fluid (CSF) and peripheral blood were studied using monoclonal antibodies, in patients with subacute sclerosing panencephalitis, eight of whom were at stage 2 and seven at stage 4. Eighteen subjects affected with non immunological diseases constituted the controls. Regardless of the stage, patients with subacute sclerosing panencephalitis had lower percentages of OKT3+ (pan-T) cells in both CSF and peripheral blood, with an increase of OKIa+ cells (B cells, macrophages and active T cells) in peripheral blood. A difference was found in the proportion of OKT4+ (helper-inducer) and OKT8+ (suppressor/cytotoxic) cells in relation to the stage, the most striking finding being a significant decrease of OKT8+ with an increase of T4/T8 ratio in peripheral blood at an early stage. PMID:2942644

  17. Sclerosing lymphangitis of the penis associated with marked penile oedema and skin erosions.

    Science.gov (United States)

    Karray, Mehdi; Litaiem, Noureddine; Jones, Mariem; Zeglaoui, Faten

    2017-07-27

    Sclerosing lymphangitis of the penis is a benign, under-reported condition consisting of a asymptomatic firm cord-like swelling around the coronal sulcus of the penis usually affecting men in the second or third decade of life. Penile oedema and erosions are rarely reported. Clinical signs may be remarkable contrasting with the self-limited character of the disease. We report a new case of sclerosing lymphangitis of the penis occurring in a 59-year-old patient marked by penile swelling and several overlying skin erosions, and discuss the clinical features and the pathogenesis aspects of the disease. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  18. A Radial Sclerosing Lesion Mimicking Breast Cancer on Mammography in a Young Woman

    Directory of Open Access Journals (Sweden)

    Masashi Furukawa

    2012-02-01

    Full Text Available A spiculated mass on a mammogram is highly suggestive of malignancy. We report the case of a 32-year-old woman with a radial sclerosing lesion that mimicked breast cancer on mammography. She visited her physician after palpating a lump in her left breast. Mammography showed architectural distortion in the upper inner quadrant of the left breast. Ultrasonography showed a low echoic area with an ambiguous boundary. Core needle biopsy was performed because of the suspicion of malignancy. Histological examination did not reveal any malignant cells. After 6 months, the breast lump became larger and the patient was referred to our hospital. Mammography performed in our hospital showed a spiculated mass, and therefore mammotome biopsy was performed. Histological examination revealed dense fibroelastic stroma with a wide variety of mastopathic changes, leading to a diagnosis of a radial sclerosing lesion. One year after the biopsy, the lump on her left breast had disappeared and mammography showed no spiculated mass.

  19.  Alkaline phosphatase normalization is a biomarker of improved survival in primary sclerosing cholangitis.

    Science.gov (United States)

    Hilscher, Moira; Enders, Felicity B; Carey, Elizabeth J; Lindor, Keith D; Tabibian, James H

    2016-01-01

     Introduction. Recent studies suggest that serum alkaline phosphatase may represent a prognostic biomarker in patients with primary sclerosing cholangitis. However, this association remains poorly understood. Therefore, the aim of this study was to investigate the prognostic significance and clinical correlates of alkaline phosphatase normalization in primary sclerosing cholangitis. This was a retrospective cohort study of patients with a new diagnosis of primary sclerosing cholangitis made at an academic medical center. The primary endpoint was time to hepatobiliaryneoplasia, liver transplantation, or liver-related death. Secondary endpoints included occurrence of and time to alkaline phosphatase normalization. Patients who did and did not achieve normalization were compared with respect to clinical characteristics and endpoint-free survival, and the association between normalization and the primary endpoint was assessed with univariate and multivariate Cox proportional-hazards analyses. Eighty six patients were included in the study, with a total of 755 patient-years of follow-up. Thirty-eight patients (44%) experienced alkaline phosphatase normalization within 12 months of diagnosis. Alkaline phosphatase normalization was associated with longer primary endpoint-free survival (p = 0.0032) and decreased risk of requiring liver transplantation (p = 0.033). Persistent normalization was associated with even fewer adverse endpoints as well as longer survival. In multivariate analyses, alkaline phosphatase normalization (adjusted hazard ratio 0.21, p = 0.012) and baseline bilirubin (adjusted hazard ratio 4.87, p = 0.029) were the only significant predictors of primary endpoint-free survival. Alkaline phosphatase normalization, particularly if persistent, represents a robust biomarker of improved long-term survival and decreased risk of requiring liver transplantation in patients with primary sclerosing cholangitis.

  20. MRI and MR spectroscopy findings of a case of subacute sclerosing panencephalitis affecting the corpus callosum.

    Science.gov (United States)

    Öztürk, Mehmet; Sığırcı, Ahmet; Yakıncı, Cengiz

    2015-07-10

    Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressive, fatal, inflammatory and neurodegenerative disease that is seen mostly in children and young adolescents, and primarily affects the parieto-occipital lobes. The corpus callosum, cerebellum and basal ganglia are less frequently involved. MR spectroscopy (MRS) may illustrate the pathophysiological features of SSPE. To the best of our knowledge, this is the second report of MRS findings of corpus callosum involvement in a stage 3 SSPE case. 2015 BMJ Publishing Group Ltd.

  1. Atypical clinical and electroencephalographic pattern in a patient with subacute sclerosing panencephalitis

    Directory of Open Access Journals (Sweden)

    Délrio F. Silva

    1995-06-01

    Full Text Available We describe an atypical clinical and electroencephalographic (EEG pattern observed during the course of subacute sclerosing panencephalitis in a 14 year-old boy. In this patient with a two weeks history of partial complex seizures, the atypical EEG pattern was characterized by an initial left temporal focus which evolved to periodic lateralized epileptiform discharges (PLEDs and, only during the 3rd and 4th weeks the typical bilateral and generalized periodic complexes appeared.

  2. Wild-type Measles Virus in Brain Tissue of Children with Subacute Sclerosing Panencephalitis, Argentina

    Science.gov (United States)

    Barrero, Paola Roxana; Grippo, Jorge; Viegas, Mariana

    2003-01-01

    We studied eight children who had measles at 6 to 10 months of age during the 1998 Argentine measles outbreak and in whom subacute sclerosing panencephalitis developed 4 years later. We report the genetic characterization of brain tissue–associated measles virus samples from three patients. Phylogenetic relationships clustered these viruses with the wild-type D6 genotype isolated during the 1998 outbreak. The children received measles vaccine; however, vaccinal strains were not found. PMID:14609476

  3. Role of CSF serology in follow-up of subacute Sclerosing Panencephalitis patients on treatment

    Directory of Open Access Journals (Sweden)

    Gupta E

    2006-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is a progressive inflammatory disease of the central nervous system with poor prognosis and high mortality. No effective treatment has a proven role; oral isoprinosine and intrathecal administration of a-interferon may prolong survival. We report an unusual case of adult onset SSPE patient on treatment with significant clinical improvement, even in the absence of conversion to seronegativity in either CSF or serum, on follow-up serological examination.

  4. Bilateral sclerosing stromal tumor of the ovary in a premenarchal girl

    Energy Technology Data Exchange (ETDEWEB)

    Chang, Yun-Woo; Hong, Seong Sook [Soonchunhyang University Hospital, Department of Radiology, Yongsan-ku, Seoul (Korea); Jeen, Yoon-Mi [Soonchunhyang University Hospital, Department of Pathology, Seoul (Korea); Kim, Mi Kyung [Soonchunhyang University Hospital, Department of Gynecology, Seoul (Korea); Suh, Eun Sook [Soonchunhyang University Hospital, Department of Pediatrics, Seoul (Korea)

    2009-07-15

    Sclerosing stromal tumor (SST) is a rare benign ovarian neoplasm classified as a type of sex cord stromal tumor that occurs predominantly in young patients. Several reports have described the US, CT and MR features of SST, but there have been no reports of a bilateral calcified SST in a child. We present a case of a bilateral SST of the ovary with calcification in a 12-year-old premenarchal girl and describe the US, CT, MR and pathological findings. (orig.)

  5. A case of suspect “cyanosis”

    OpenAIRE

    Elisabetta Antonucci; Matteo Conte; Michele Di Pumpo; Giuseppe Antonucci

    2013-01-01

    CLINICAL CASE A 70-year old woman was admitted to our hospital because of fever, asthenia and a suspected stroke. Her medical history showed a congenital cardiopathy (Patent Foramen Ovale, PFO). Skin and oral mucosa pigmentation, orthostatic hypotension, hypoglycemia and hyponatriemia arose the suspect of Addison’s disease. The diagnosis was confirmed by the evaluation of basal levels of plasma ACTH and serum cortisol, and serum cortisol levels after ACTH stimulation. Abdominal CT scan showed...

  6. The impact of elevated serum IgG4 levels in patients with primary sclerosing cholangitis.

    Science.gov (United States)

    Benito de Valle, Maria; Müller, Tobias; Björnsson, Einar; Otten, Morgane; Volkmann, Martin; Guckelberger, Olaf; Wiedenmann, Bertram; Sadik, Riadh; Schott, Eckart; Andersson, Mats; Berg, Thomas; Lindkvist, Björn

    2014-10-01

    Elevated IgG4 levels have been reported among patients with primary sclerosing cholangitis. Epidemiological data has only been provided from tertiary centres. To investigate the prevalence of elevated IgG4 levels and to compare prognosis between patients with and without elevated IgG4 levels in serum in two European cohorts of patients with primary sclerosing cholangitis. Serum IgG4-levels were measured in a consecutive series of patients from Berlin, and retrospectively collected in a population-based cohort from Sweden (total N=345). Cox's proportional hazard analysis was used to calculate relative risks for liver-related death or liver transplantation and cholangiocarcinoma. Elevated IgG4 values were demonstrated in 10% of patients. A previous history of pancreatitis, combined intra- and extrahepatic biliary involvement and jaundice were independently associated with elevated IgG4 in multivariate analysis. IgG4 status was not associated with an increased risk for the combined endpoint liver-related death or liver transplantation or cholangiocarcinoma. The prevalence of elevated IgG4 values among European patients with primary sclerosing cholangitis is similar to what previously has been reported from the United States. Elevated IgG4 was not associated with an increased risk of liver transplantation or liver-related death or cholangiocarcinoma. Copyright © 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  7. The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis.

    Science.gov (United States)

    Floreani, Annarosa; Motta, Raffaella; Cazzagon, Nora; Franceschet, Irene; Roncalli, Massimo; Del Ross, Teresa; Rosina, Floriano; Lleo, Ana; Mescoli, Claudia; Colloredo, Guido; Invernizzi, Pietro

    2015-05-01

    The overlap syndrome between primary biliary cirrhosis and primary sclerosing cholangitis is an extremely rare condition that has been reported in only six published cases so far. Here we report two cases showing the clinical manifestations of both primary biliary cirrhosis and primary sclerosing cholangitis. In one case the overlap condition was associated with psoriatric arthritis, and the patient successfully underwent dual treatment with ursodeoxycholic acid and the anti-tumour necrosis factor-alpha agent adalimumab. In the second case, the predominant condition was, initially, an antimitochondrial antibody-negative primary biliary cirrhosis with progressive course towards end-stage liver disease; the patient then developed either antimitochondrial antibody positivity or changes in the biliary tree compatible with primary sclerosing cholangitis. These two cases add information on a controversial issue in the literature, and indicate the importance of recognizing a possible overlap syndrome to optimize treatment. Copyright © 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  8. The features of mucosa-associated microbiota in primary sclerosing cholangitis.

    Science.gov (United States)

    Torres, J; Bao, X; Goel, A; Colombel, J-F; Pekow, J; Jabri, B; Williams, K M; Castillo, A; Odin, J A; Meckel, K; Fasihuddin, F; Peter, I; Itzkowitz, S; Hu, J

    2016-04-01

    Little is known about the role of the microbiome in primary sclerosing cholangitis. To explore the mucosa-associated microbiota in primary sclerosing cholangitis (PSC) patients across different locations in the gut, and to compare it with inflammatory bowel disease (IBD)-only patients and healthy controls. Biopsies from the terminal ileum, right colon, and left colon were collected from patients and healthy controls undergoing colonoscopy. Microbiota profiling using bacterial 16S rRNA sequencing was performed on all biopsies. Forty-four patients were recruited: 20 with PSC (19 with PSC-IBD and one with PSC-only), 15 with IBD-only and nine healthy controls. The overall microbiome profile was similar throughout different locations in the gut. No differences in the global microbiome profile were found. However, we observed significant PSC-associated enrichment in Barnesiellaceae at the family level, and in Blautia and an unidentified Barnesiellaceae at the genus level. At the operational taxa unit level, most shifts in PSC were observed in Clostridiales and Bacteroidales orders, with approximately 86% of shifts occurring within the former order. The overall microbiota profile was similar across multiple locations in the gut from the same individual regardless of disease status. In this study, the mucosa associated-microbiota of patients with primary sclerosing cholangitis was characterised by enrichment of Blautia and Barnesiellaceae and by major shifts in operational taxa units within Clostridiales order. © 2016 John Wiley & Sons Ltd.

  9. An investigation on the influence of glycerin on sclerosant foam stability.

    Science.gov (United States)

    Peterson, J D; Goldman, M P

    2011-09-01

    Foam sclerotherapy is an increasingly popular modality in the treatment of varicose veins. Foam stability varies according to foam composition, volume and injection technique. A disposable plastic connector was used to create foam from 0.50% sodium tetradecyl sulphate (STS) mixed with varying volumes of glycerin. As a measure of foam stability, the half liquid time was defined as the time required for half of the original volume of sclerosing solution to settle. Three recordings were determined for each of the three mixtures of sclerosant foam. The time for sclerosing solution to settle to half of its initial volume was found to be 89 seconds for 0.50% STS alone, 117.7 seconds with the addition of 0.1 mL of 72% glycerin, and 114.7 seconds with the addition of 0.2 mL of 72% glycerin. The small volumes of glycerin added to STS prolonged the half liquid time of STS foam up to 35%. As glycerin alone is unable to be foamed with the double-syringe system technique there may be a point at which further addition of glycerin has a negative effect on the half-life of foam.

  10. Subacute sclerosing panencephalitis presenting as acute cerebellar ataxia and brain stem hyperintensities.

    Science.gov (United States)

    Saini, Arushi Gahlot; Sankhyan, Naveen; Padmanabh, Hansashree; Sahu, Jitendra Kumar; Vyas, Sameer; Singhi, Pratibha

    2016-05-01

    Subacute sclerosing panencephalitis is a devastating neurodegenerative disease with a characteristic clinical course. Atypical presentations may be seen in 10% of the cases. To describe the atypical clinical and radiological features of SSPE in a child form endemic country. A 5-year-old boy presented with acute-onset cerebellar ataxia without associated encephalopathy, focal motor deficits, seizures or cognitive decline. He had varicella-like illness with vesicular, itchy truncal rash erupting one month prior to the onset of these symptoms. He underwent detailed neurological assessment, relevant laboratory and radiological investigations. Neuroimaging revealed peculiar brain stem lesions involving the pons and cerebellum suggestive of demyelination. With a presumptive diagnosis of clinically isolated syndrome of demyelination, he was administered pulse methylprednisolone (30 mg/kg/day for 5 days). Four weeks later he developed myoclonic jerks. Electroencephalogram showed characteristic periodic complexes time-locked with myoclonus. CSF and serum anti-measles antibody titres were elevated (1:625). Our report highlights that subacute sclerosing panencephalitis can present atypically as isolated acute cerebellar ataxia and peculiar involvement of longitudinal and sparing of transverse pontine fibres. The predominant brainstem abnormalities in the clinical setting may mimick acute demyelinating syndrome. Hence, it is important to recognize these features of subacute sclerosing panencephalitis in children, especially in the endemic countries. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  11. An evaluation of subacute sclerosing panencephalitis patients with diffusion-weighted magnetic resonance imaging.

    Science.gov (United States)

    Abuhandan, M; Cece, H; Calik, M; Karakas, E; Dogan, F; Karakas, O

    2013-03-01

    This study aimed to evaluate the contribution of diffusion weighted magnetic resonance imaging to the diagnosis and staging of subacute sclerosing panencephalitis. The study comprised 26 patients diagnosed with subacute sclerosing panencephalitis at our clinic who were undergoing regular follow-up, and a control group of 18 subjects. Clinical staging was determined by Risk and Haddad classification; 12 at Stage II and 14 at Stage III. Diffusion weighted magnetic resonance images were taken of six areas (frontal, parieto-occipital, cerebellar, deep white matter, thalamus and basal ganglia) and by calculating the apparent diffusion coefficient (ADC) values, and a comparison was made between the stages and with the control group. The ADC values of all the areas of the subacute sclerosing panencephalitis patients were found to be significantly higher compared to the control group (p 0.05). The ADC values of all the areas of the Stage III patients were found to be significantly high compared to the Stage II values (p panencephalitis and to reveal differences between the stages.

  12. MDM2 Amplification and PI3KCA Mutation in a Case of Sclerosing Rhabdomyosarcoma

    Directory of Open Access Journals (Sweden)

    Ken Kikuchi

    2013-01-01

    Full Text Available A rare sclerosing variant of rhabdomyosarcoma characterized by prominent hyalinization and pseudovascular pattern has recently been described as a subtype biologically distinct from embryonal, alveolar, and pleomorphic forms. We present cytogenetic and molecular findings as well as experimental studies of an unusual case of sclerosing rhabdomyosarcoma. The primary lesion arose within the plantar subcutaneous tissue of the left foot of an otherwise healthy 23-year-old male who eventually developed pulmonary nodules despite systemic chemotherapy. Two genetic abnormalities identified in surgical and/or autopsy samples of the tumor were introduced into 10T1/2 murine fibroblasts to determine whether these genetic changes cooperatively facilitated transformation and growth. Cytogenetic analysis revealed a complex abnormal hyperdiploid clone, and MDM2 gene amplification was confirmed by fluorescence in situ hybridization. Cancer gene mutation screening using a combination of multiplexed PCR and mass spectroscopy revealed a PIK3CA exon 20 H1047R mutation in the primary tumor, lung metastasis, and liver metastasis. However, this mutation was not cooperative with MDM2 overexpression in experimental assays for transformation or growth. Nevertheless, MDM2 and PIK3CA are genes worthy of further investigation in patients with sclerosing rhabdomyosarcoma and might be considered in the enrollment of these patients into clinical trials of targeted therapeutics.

  13. The B-mode Sonography and Sonoelastographic Features of Sclerosing Adenosis of the Breast

    Energy Technology Data Exchange (ETDEWEB)

    Myong, Joo Hwa; Kim, Sung Hun; Kang, Bong Joo; Ahn, Young I; Yoon, Soo Kyoung; Lee, A Won; Yim, Kwang Il; Kim, Tae Eun; Song, Byung Joo [Seoul St. Mary' s Hospital, The Catholic University, Seoul (Korea, Republic of)

    2011-06-15

    The purpose of this study was to evaluate the B-mode sonographic and sonoelastographic features of high risk lesions of the breast. From April 2009 to February 2010, 1390 patients with breast lesions underwent US-guided core-biopsy. Among them, 13 lesions were confirmed to be pure sclerosing adenosis by subsequent surgical excision or on imaging follow-up of more than 1 year. Two radiologists retrospectively analyzed the B-mode sonography according to the Breast Imaging Reporting and Data System classification. The sonoelastographic images were classified into 5 elasticity scores according to the Itoh classification and the strain ratio between the mass and the surrounding fat tissue was reviewed. We considered the sonoelastographic patterns to be suspicious for the case with a score of 4 and 5 and a strain ratio of more than a 2.24. The common B-mode sonographic features of sclerosing adenosis were an irregular shape (69.2%, 9 of 13), an indistinct margin (92.3%, 12 of 13), hypoechogenicity (76.9%, 10 of 13) and category 4A, a low suspicion of malignancy (61.5%, 8 of 13). The common sonoelastographic features were a score of 2 (42%, 6 of 13) and a strain ratio < 2.24 (69.2%, 9 of 13). Sclerosing adenosis showed suspicious B-mode sonographic findings, but it had benign sonolastographic features

  14. Effective nonoperative treatment in juvenile idiopathic scoliosis

    NARCIS (Netherlands)

    van Hessem, L.; Schimmel, J.J.P.; Graat, H.C.A.; de Kleuver, M.

    2014-01-01

    Nonoperative management of juvenile idiopathic scoliosis (JIS) has been reported to be less effective than that of infantile idiopathic scoliosis. The goal of this study was to analyse the results of casting and/or bracing in JIS. Clinical data from seven patients with JIS, treated with casting

  15. Endoscopic third ventriculostomy in idiopathic normal pressure ...

    African Journals Online (AJOL)

    Objective: To determine the efficacy of endoscopic fenestration of the third ventricle in the treatment of idiopathic normal pressure hydrocephalus. Methods: 16 patients with idiopathic normal pressure hydrocephalus were treated by endoscopic third ventriculostomy. This study lasted three years. All patients were over 50 ...

  16. Endoscopic third ventriculostomy in idiopathic normal pressure ...

    African Journals Online (AJOL)

    Mohammed Ahmed Eshra

    2013-12-22

    Dec 22, 2013 ... Abstract Objective: To determine the efficacy of endoscopic fenestration of the third ventricle in the treatment of idiopathic normal pressure hydrocephalus. Methods: 16 patients with idiopathic normal pressure hydrocephalus were treated by endoscopic third ventriculostomy. This study lasted three years.

  17. Cerebrospinal Fluid Biomarkers in Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Ville Leinonen

    2011-01-01

    Full Text Available The diagnosis of idiopathic normal pressure hydrocephalus (iNPH is still challenging. Alzheimer's disease (AD, along with vascular dementia, the most important differential diagnosis for iNPH, has several potential cerebrospinal fluid (CSF biomarkers which might help in the selection of patients for shunt treatment. The aim of this study was to compare a battery of CSF biomarkers including well-known AD-related proteins with CSF from patients with suspected iNPH collected from the external lumbar drainage test (ELD. A total of 35 patients with suspected iNPH patients were evaluated with ELD. CSF was collected in the beginning of the test, and the concentrations of total tau, ptau181, Aβ42, NFL, TNF-α, TGFβ1, and VEGF were analysed by ELISA. Twenty-six patients had a positive ELD result—that is, their gait symptoms improved; 9 patients had negative ELD. The levels of all analyzed CSF biomarkers were similar between the groups and none of them predicted the ELD result in these patients. Contrary to expectations lumbar CSF TNF-α concentration was low in iNPH patients.

  18. Successful low-dose azathioprine for myasthenia gravis despite hepatopathy from primary sclerosing cholangitis: a case report

    Directory of Open Access Journals (Sweden)

    Höflich Sonja

    2010-11-01

    Full Text Available Abstract Introduction Although myasthenia gravis is frequently associated with other disorders, it has not been reported together with primary sclerosing cholangitis, complicating the administration of liver-toxic immunosuppressive therapy. Case presentation A 73-year-old Caucasian woman with a history of arterial hypertension, thyroid dysfunction, glaucoma, right-sided ptosis and later generalized weakness, was diagnosed with myasthenia gravis. Additionally, primary sclerosing cholangitis was detected, initially prohibiting the administration of immunosuppressants. Despite treatment with steroids and pyridostigmine she repeatedly experienced myasthenic crises. After the fifth crisis and after antibody titers had reached levels > 100 nmol/L during two years of follow-up, it was decided to restart azathioprine. Interestingly, low-dose azathioprine (1.5 mg/kg/day was well tolerated, had a positive clinical and immunological effect and did not worsen primary sclerosing cholangitis. Conclusion Myasthenia gravis may occur together with primary sclerosing cholangitis in the same patient. Mild immunosuppression with azathioprine is feasible and effective in such a patient, without worsening myasthenia gravis or primary sclerosing cholangitis.

  19. The phenomenology of specialization of criminal suspects.

    Directory of Open Access Journals (Sweden)

    Michele Tumminello

    Full Text Available A criminal career can be either general, with the criminal committing different types of crimes, or specialized, with the criminal committing a specific type of crime. A central problem in the study of crime specialization is to determine, from the perspective of the criminal, which crimes should be considered similar and which crimes should be considered distinct. We study a large set of Swedish suspects to empirically investigate generalist and specialist behavior in crime. We show that there is a large group of suspects who can be described as generalists. At the same time, we observe a non-trivial pattern of specialization across age and gender of suspects. Women are less prone to commit crimes of certain types, and, for instance, are more prone to specialize in crimes related to fraud. We also find evidence of temporal specialization of suspects. Older persons are more specialized than younger ones, and some crime types are preferentially committed by suspects of different ages.

  20. A fatal case of tuberculous meningitis in a child with juvenile idiopathic arthritis: a diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Zumrut Sahbudak Bal

    Full Text Available Abstract The prognosis of tuberculous meningitis, a rare form of extrapulmonary tuberculosis, depends on the stage of treatment initiation. We report a fatal case of tuberculous meningitis. The patient had received successive tumor necrosis factor (TNF antagonists and abatacept to treat juvenile idiopathic arthritis, with negative results for polymerase chain reaction and acid-fast bacilli on smear, had normal cerebrospinal fluid (CSF adenosine deaminase and glucose levels. Six weeks post-admission, the CSF culture demonstrated Mycobacterium tuberculosis. The altered immunological responses caused by anti-TNF treatment made the diagnosis challenging. Clinicians should bear this in mind and, if suspected, treatment should be initiated immediately.

  1. Nintedanib for Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Tepede, Abisola; Yogaratnam, Dinesh

    2017-01-01

    To review the pharmacology, safety, and efficacy of nintedanib for the treatment of idiopathic pulmonary fibrosis (IPF). A literature search was conducted via PubMed using the MeSH term "idiopathic pulmonary fibrosis" combined with the key word "nintedanib." Additional online searches using Google Scholar, Micromedex, and PubMed were performed to obtain prescribing and cost information. One phase II and 2 replicate phase III clinical trials that examined the safety and efficacy of nintedanib for IPF were identified. In patients with IPF, nintedanib was more effective than placebo in slowing the annual rate of decline in forced vital capacity (FVC). Improvements in mortality, quality of life, and risk of acute exacerbations have not been consistently demonstrated in clinical trials. Diarrhea was the most common adverse effect associated with nintedanib use. Outside of these clinical trials, there are limited data evaluating nintedanib for the treatment of IPF. Nintedanib is a safe and effective treatment option for patients with IPF. Nintedanib slows IPF disease progression by reducing the rate of decline in FVC. Reductions in mortality and acute exacerbations may be present in certain subgroups of patients, but these outcomes require further research. Future studies on nintedanib are needed to explore its use in more advanced stages of IPF, its long-term safety and efficacy, its value in combination with pirfenidone or other therapies for IPF, and its cost-effectiveness in clinical practice.

  2. Qualitative Dermatoglyphics In Idiopathic Epilepsy

    Directory of Open Access Journals (Sweden)

    Ranganath Priya

    2004-01-01

    Full Text Available Genetic aetiology has been proposed for both idiopathic epilepsy and dermatoglyphics. Hence, the present study has been undertaken to find out the existence of any correlation between dermatoglyphics and idiopathic epilepsy. Material consisted of 100 patients (58 males and 42 females and 100 controls (52 males and 48 females. Patient′s age ranged from 5 to 40 years and controls were between 18 and 25 years. Dermatoglyphics were obtained by painting method. Qualitative parameters observed were percentage frequency of fingerprint patterns (loops, whorls and arches,, patterns in hypothenar area/ interdigital are and flexion creases (Simian crease, sydney line. On comparison with controls, in males, with hands combined, loops (52.24% and arches (7.93% were increased and whorls (39.83% were decreased (p<0.05. In females, with hands combined, arches (13.1% and whorls (36.43% were increased and loops (50.48% were decreased (p< 0.03. Significant differences have not been observed for the patterns in hypothenar area /interdigital area and flexion creases. These dermatoglyphics features could be used as additional markers to evaluate patients of epilepsy.

  3. Vitamin D deficiency in chronic idiopathic urticaria.

    Science.gov (United States)

    Movahedi, Masoud; Tavakol, Marzieh; Hirbod-Mobarakeh, Armin; Gharagozlou, Mohammad; Aghamohammadi, Asghar; Tavakol, Zahra; Momenzadeh, Kaveh; Nabavi, Mohammad; Dabbaghzade, Abbas; Mosallanejad, Asieh; Rezaei, Nima

    2015-04-01

    Chronic urticaria is the most common skin diseases, characterized by chronic cutaneous lesions which severely debilitates patients in several aspects of their everyday life. Vitamin D is known to exert several actions in the immune system and to influence function and differentiation of mast cells, central role players in the pathogenesis of chronic idiopathic urticaria. This study was performed to evaluate the relationship between vitamin D levels and susceptibility to chronic idiopathic urticaria. One hundred and fourteen patients with chronic idiopathic urticaria were recruited in this study along with one hundred and eighty seven sex-matched and age-matched healthy volunteers as the control group. For each patient, urticaria activity score was calculated and autologous serum skin test was done. Vitamin D metabolic statue was measured in serum as 25 hydroxyvitamin D using enzyme immunoassay method. Patients with chronic idiopathic urticaria significantly showed lower levels of vitamin D. Vitamin D deficiency was significantly associated with increased susceptibility to chronic idiopathic urticaria. There was a significant positive correlation between vitamin D levels and urticaria activity score. This study showed that patients with chronic idiopathic urticaria had reduced levels of vitamin D, while vitamin D deficiency could increase susceptibility to chronic idiopathic urticaria.

  4. A case of suspect “cyanosis”

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    Elisabetta Antonucci

    2013-05-01

    Full Text Available CLINICAL CASE A 70-year old woman was admitted to our hospital because of fever, asthenia and a suspected stroke. Her medical history showed a congenital cardiopathy (Patent Foramen Ovale, PFO. Skin and oral mucosa pigmentation, orthostatic hypotension, hypoglycemia and hyponatriemia arose the suspect of Addison’s disease. The diagnosis was confirmed by the evaluation of basal levels of plasma ACTH and serum cortisol, and serum cortisol levels after ACTH stimulation. Abdominal CT scan showed atrophy and calcification of adrenal glands. CONCLUSIONS In most cases, Addison’s disease is provoked by autoimmune destruction of the adrenal cortex; however, in our reported patient, tuberculosis could be a possible cause.

  5. Primary (Poorly Differentiated Sclerosing Liposarcoma of Temporal Region. An Uncommon Tumor in a Rare Site: A Case Report

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    Anuradha CK Rao

    2015-02-01

    Full Text Available Liposarcoma (LS in the head and neck region is a rare tumor. The sclerosing variant of LS is a subtype of well-differentiated LS characterized by areas of conventional LS admixed with hypocellular areas of stromal sclerosis that show atypical lipomatous cells. The (poorly differentiated sclerosing LS, on the other hand, is more cellular with atypical, pleomorphic and often bizarre giant tumor cells admixed with atypical lipoblasts. We report a case of poorly differentiated sclerosing LS of temporal region in a 49-year-old man. Radiologically, the tumor was dumbbell shaped with intra and extra cranial extension. In this case, we discuss the clinico-radiological and pathological findings of an unusual tumor in a rare location. [J Interdiscipl Histopathol 2015; 3(1.000: 33-35

  6. Sclerosing Mesenteritis as a Cause of Abdominal Mass and Discomfort in an Elderly Patient: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Farzana Nawaz Ali

    2010-01-01

    Full Text Available Sclerosing mesenteritis is a rare benign process that involves inflammation, fat necrosis, and fibrosis of the mesentery. The disease poses great diagnostic challenge due to its nonspecific clinical and diagnostic findings. We report the case of a 75-year-old man who presented with vague abdominal discomfort associated with an intra-abdominal mass. With suspicion of a bowel carcinoid tumor on computed tomography scans, the patient underwent diagnostic laparoscopy. A diagnosis of sclerosing mesenteritis was made on histological examination. The patient's symptoms responded to a combination of immunosuppressive drugs, with no interval change in the size of the mass on radiological examination after fifteen months.

  7. Multiple pulmonary sclerosing hemangiomas (pneumocytoma) mimicking lung metastasis detected in fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography.

    Science.gov (United States)

    Kamaleshwaran, Koramadai Karuppusamy; Rajan, Firoz; Mehta, Sangita; Mohanan, Vyshakh; Shinto, Ajit Sugunan

    2014-07-01

    Pulmonary sclerosing hemangioma (PSH), or the alternative name of "sclerosing pneumocytoma," is a rare benign neoplasm. PSH is often asymptomatic and presents as a solitary or multiple pulmonary nodules on radiologic imaging studies. Few articles have been reported to describe the fluorodeoxyglucose positron emission tomography (FDG PET) findings about PSH. The authors describe an interesting but uncommonly encountered cause of false positive FDG PET scan in the thorax in a 25-year-old woman, a known case of arteriovenous malformation of oral cavity who underwent embolization and presented with incidental detection of bilateral lung nodules. She is asymptomatic and is on follow-up.

  8. [Asynchronous Bilateral Testicular Infarction with Suspected Polyarteritis Nodosa : A Case Report].

    Science.gov (United States)

    Kurokawa, Masayuki; Naito, Sei; Ichiyanagi, Osamu; Suto, Aya; Kurota, Yuta; Sakurai, Toshihiko; Nishida, Hayato; Kawazoe, Hisashi; Kato, Tomoyuki; Nagaoka, Akira; Ito, Hiromi; Yamakawa, Mitsunori; Tsuchiya, Norihiko

    2016-12-01

    This report documents a case of asynchronous bilateral testicular infarction. The patient was a 42- year-old man who presented with left testicular pain and swelling. He had a past history of right idiopathic testicular infarction and underwent a right orchiectomy 6 years ago. He also had received treatment for 5 years for suspected polyarteritis nodosa (PAN). The left scrotal pain persisted for a week and left orchiectomy was performed. Pathological evaluations demonstrated a benign testis with testicular hemorrhage and chronic vasculopathy. There was no fibrinoid necrosis of medium-size vessel walls which characterizes PAN. In this report, we review the pathogenesis, risk of contralateral testicular infarction, and management of testicular infarction.

  9. Congenital Malaria Among Newborns Admitted for Suspected ...

    African Journals Online (AJOL)

    Background: Signs and symptoms of congenital malaria do not differ much from those of neonatal sepsis: both can co-exist, and most times very difficult to differentiate clinically. Objective: To document the prevalence, risk factors for congeni tal malar ia among neonates admitted for suspected neonatal sepsis, and ...

  10. Congenital Malaria Among Newborns Admitted for Suspected ...

    African Journals Online (AJOL)

    None of the clinical feature had good sensitivity, specificity or predictive value for congenital malaria, and only 1.6% death was recorded in a baby with high parasite density. Conclusion: Congenital malaria is common in newborns with suspected neonatal sepsis. History of peripartum pyrexia, prematurity and intrauterine ...

  11. MRI for clinically suspected appendicitis during pregnancy.

    NARCIS (Netherlands)

    Cobben, L.P.; Groot, I.; Haans, L.; Blickman, J.G.; Puylaert, J.

    2004-01-01

    OBJECTIVE: The purpose of this study was to evaluate whether MRI can be used to accurately diagnose or exclude appendicitis in pregnant patients with clinically suspected appendicitis. CONCLUSION: Our results suggest that MRI is helpful in the examination and diagnosis of acute appendicitis in

  12. Biomechanical properties of keratoconus suspect eyes.

    Science.gov (United States)

    Saad, Alain; Lteif, Yara; Azan, Elodie; Gatinel, Damien

    2010-06-01

    Measuring corneal biomechanical properties may help detect keratoconus suspect corneas and eliminate the risk of ectasia after LASIK. Data of 504 eyes separated into three groups were retrospectively reviewed: normal (n = 252), keratoconus suspect (n = 80), and keratoconus (n = 172). Corneal hysteresis (CH) and corneal resistance factor (CRF) were measured with an ocular biomechanics analyzer. Mean corneal hysteresis was 10.6 +/- 1.4 (SD) mm Hg in the normal group, compared with 10.0 +/- 1.6 mm Hg in the keratoconus suspect group and 8.1 +/- 1.4 mm Hg in the keratoconus group. The mean CRF was 10.6 +/- 1.6 mm Hg in the normal group compared with 9.7 +/- 1.7 in the keratoconus suspect group and 7.1 +/- 1.6 mm Hg in the keratoconus group. Mean CH and CRF were significantly different between the three groups (P corneas. Analyzing signal curves obtained with the biomechanics analyzer may provide additional valuable information for selecting qualified patients for refractive surgery.

  13. Characterization of suspected illegal skin whitening cosmetics.

    Science.gov (United States)

    Desmedt, B; Van Hoeck, E; Rogiers, V; Courselle, P; De Beer, J O; De Paepe, K; Deconinck, E

    2014-03-01

    An important group of suspected illegal cosmetics consists of skin bleaching products, which are usually applied to the skin of the face, hands and décolleté for local depigmentation of hyper pigmented regions or more importantly, for a generalized reduction of the skin tone. These cosmetic products are suspected to contain illegal active substances that may provoke as well local as systemic toxic effects, being the reason for their banning from the EU market. In that respect, illegal and restricted substances in cosmetics, known to have bleaching properties, are in particular hydroquinone, tretinoin and corticosteroids. From a legislative point of view, all cosmetic products containing a prohibited whitening agent are illegal and must be taken off the EU market. A newly developed screening method using ultra high performance liquid chromatography-time off flight-mass spectrometry allows routine analysis of suspected products. 163 suspected skin whitening cosmetics, collected by Belgian inspectors at high risk sites such as airports and so-called ethnic cosmetic shops, were analyzed and 59% were classified as illegal. The whitening agents mostly detected were clobetasol propionate and hydroquinone, which represent a serious health risk when repeatedly and abundantly applied to the skin. Copyright © 2013 Elsevier B.V. All rights reserved.

  14. Suspecting Neurological Dysfunction From E Mail Messages ...

    African Journals Online (AJOL)

    A non medical person suspected and confirmed neurological dysfunction in an individual, based only on e mail messages sent by the individual. With email communication becoming rampant “peculiar” email messages may raise the suspicion of neurological dysfunction. Organic pathology explaining the abnormal email ...

  15. [Idiopathic chylothorax in an infant: management and progression].

    Science.gov (United States)

    Chemaou, A; Ayachi, M; Ailal, F; Najib, J

    2012-07-01

    Chylothorax is a rare disease (1-2 % of pleural effusions), with a prevalence between 1/8600 and 1/15,000 births. It is characterized by the presence of chyle in the pleural cavity. Three categories of chylothorax are known: congenital chylothorax, which can be either idiopathic or the result of a malformation, and traumatic chylothorax (mostly postoperative). We report the observation of a 9-month-old infant with idiopathic chylothorax revealed by respiratory symptoms, with pleural effusion and collapse of the ipsilateral lung on chest X-ray and ultrasound examination. Cytology and chemical analysis of the pleural fluid showed an exudative liquid with a chylous aspect, a high concentration of albumin (52 g/dL), triglycerides (11.42 g/L), and a high number of cells (6600 cells/mL), with lymphocyte predominance (96 %). The culture was sterile. Chylothorax is usually revealed by dyspnea, but also by nausea, vomiting, anorexia and/or malnutrition. The diagnosis is suspected when milky white fluid is obtained from thoracocentesis and is confirmed by the presence of a triglyceride level greater than 1.2 mmol/L and more than 1000 cells/mL, with lymphocyte predominance. The treatment of chylothorax can be either conservative or surgical. Conservative treatment (medical) has four goals: ensure pleural emptiness, decrease production of chyle, restore and/or maintain proper nutritional status, and treatment of the cause when identified. Surgical intervention is indicated when conservative management fails and aims to stop a radical and permanent leakage of chyle. Copyright © 2012. Published by Elsevier SAS.

  16. Idiopathic Inflammatory Myopathies: An update

    Directory of Open Access Journals (Sweden)

    Bulent KURT

    2016-06-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are a heterogeneous group of disease with complex clinical features. It has been sub-classified as: (1 Dermatomyositis, (2 Polymyositis, and (3 Inclusion body myositis (IBM. Nowadays, there are some studies in literature suggest necrotizing autoimmune myopathy and immune-mediated necrotizing myopathy should also be added to this group of disease. There is a debate in the diagnosis of IIMs and up until now, about 12 criteria systems have been proposed. Some of the criteria systems have been used widely such as Griggs et al.'s proposal for IBM. Clinical findings, autoantibodies, enzymes, electrophysiological, and muscle biopsy findings are diagnostic tools. Because of diseases' complexity, none of the findings are diagnostic alone. In this study, we discussed the diagnostic criteria of IMMs and described detailed morphological features. [J Interdiscipl Histopathol 2016; 4(2.000: 41-45

  17. Epigenomics of idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Yang, Ivana V

    2012-04-01

    Idiopathic pulmonary fibrosis (IPF) is a complex lung disease of unknown etiology. Development of IPF is influenced by both genetic and environmental factors. Gene-expression profiling studies have taught us quite a bit about the biology of this fatal disease, but epigenetic marks may be the missing link that connects the environmental exposure in genetically predisposed individuals to transcriptome changes associated with the development of IPF. This review will begin with an introduction to the disease, followed by brief summaries of studies of gene expression in IPF and epigenetic marks associated with exposures relevant to IPF. The majority of the discussion will focus on epigenetic studies conducted so far in IPF, the limitations, challenges nd future directions in this field.

  18. Pathogenesis of Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    Wolters, Paul J.; Collard, Harold R.; Jones, Kirk D.

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with aging that is characterized by the histopathological pattern of usual interstitial pneumonia. Although an understanding of the pathogenesis of IPF is incomplete, recent advances delineating specific clinical and pathologic features of IPF have led to better definition of the molecular pathways that are pathologically activated in the disease. In this review we highlight several of these advances, with a focus on genetic predisposition to IPF and how genetic changes, which occur primarily in epithelial cells, lead to activation of profibrotic pathways in epithelial cells. We then discuss the pathologic changes within IPF fibroblasts and the extracellular matrix, and we conclude with a summary of how these profibrotic pathways may be interrelated. PMID:24050627

  19. [Physiotherapy for juvenile idiopathic arthritis].

    Science.gov (United States)

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  20. Genetics Home Reference: idiopathic inflammatory myopathy

    Science.gov (United States)

    ... foreign invaders (such as viruses and bacteria). Each HLA gene has many different normal variations, allowing each person's ... range of foreign proteins. Specific variations of several HLA genes seem to affect the risk of developing idiopathic ...

  1. Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

    African Journals Online (AJOL)

    Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic Arthritis in a Nigerian boy. ... This may create considerable diagnostic difficulty and ... of two months duration, had an elevated Rheumatoid factor and X-ray findings suggestive ...

  2. Family history of idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

    Dauvilliers, Yves; Postuma, Ronald B; Ferini-Strambi, Luigi

    2013-01-01

    To compare the frequency of proxy-reported REM sleep behavior disorder (RBD) among relatives of patients with polysomnogram-diagnosed idiopathic RBD (iRBD) in comparison to controls using a large multicenter clinic-based cohort....

  3. Idiopathic polyhydramnios and postnatal abnormalities.

    Science.gov (United States)

    Abele, Harald; Starz, Sandra; Hoopmann, Markus; Yazdi, Britta; Rall, Katharina; Kagan, Karl Oliver

    2012-01-01

    To investigate the proportion and type of fetal anomalies that are associated with polyhydramnios and to examine whether in cases with idiopathic polyhydramnios during the course of pregnancy and fetal anomalies only diagnosed after birth, antenatal characteristics differ. This was a retrospective study involving all pregnancies with polyhydramnios defined by a deepest pool of amniotic fluid ≥8 cm and a detailed ultrasound examination, a 75 g glucose tolerance test and a TORCH serology. Between 2004 and 2010, 272 pregnancies fulfilled the inclusion criteria. In 89 (32.7%) and 65 (23.9%) cases, there was a fetal anomaly or diabetes. In 118 (43.4%) pregnancies, polyhydramnios was classified as idiopathic. In 11 (9.3%) of the 118 fetuses, an anomaly was found after birth, mainly gastrointestinal atresia. In these cases, median deepest pool of amniotic fluid was 9.6 cm, and median estimated fetal weight was at the 69th centile, whereas in cases without anomalies diagnosed after birth, median deepest pool was 9.0 cm and median estimated fetal weight at the 90th centile (Mann-Whitney U test: deepest pool p = 0.116, and estimated fetal weight centile p = 0.377). There was also no difference in the maternal and gestational age distribution of these cases (Mann-Whitney U test: maternal age p = 0.293, and gestational age p = 0.499). In about 40% of pregnancies, polyhydramnios remains unexplained during the course of pregnancy. In 10% of these cases, an anomaly will only be found after birth. In this group, antenatal characteristics such as amniotic fluid volume, estimated fetal weight or gestational and maternal age at the time of diagnosis do not help to detect these anomalies before birth. Copyright © 2012 S. Karger AG, Basel.

  4. Gain of Function Mutations of PIK3CD as a Cause of Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Hartman, Heather N; Niemela, Julie; Hintermeyer, Mary K; Garofalo, Mary; Stoddard, Jennifer; Verbsky, James W; Rosenzweig, Sergio D; Routes, John M

    2015-01-01

    Gain of function (GOF) mutation in the p110δ catalytic subunit of the phosphatidylinositol-3-OH kinase (PIK3CD) is the cause of a primary immunodeficiency (PID) characterized by recurrent sinopulmonary infections and lymphoproliferation. We describe a family of two adults and three children with GOF mutation in PIK3CD, all with recurrent sinopulmonary infections and varied infectious and non-infectious complications. The two adults have Primary Sclerosing Cholangitis (PSC) without evidence of Cryptosporidium parvum infection and have required liver transplantation. PSC is a novel phenotype of GOF mutation in PIK3CD.

  5. HIV-associated sub-acute sclerosing panencephalitis - an emerging threat?

    Science.gov (United States)

    Manesh, Abi; Moorthy, Mahesh; Bandopadhyay, Rini; Rupali, Priscilla

    2017-08-01

    Earlier age of measles virus infection predisposes to development of sub-acute sclerosing panencephalitis (SSPE) and this risk is heightened in HIV-infected children. We describe a HIV-infected young adult on antiretroviral therapy, presenting with a non-classical, fulminant form of SSPE to highlight the unpredictable nature of measles presentation. The recent spate of measles outbreaks due to virus introduction in populations with sub-optimal vaccine coverage or waning immunity and co-existing paediatric HIV cohorts is a cause for concern.

  6. Subacute sclerosing panencephalitis presenting as rapidly progressive young-onset dementia.

    Science.gov (United States)

    Chakor, Rahul Tryambak; Santosh, Nandanavana Subbareddy

    2013-07-01

    Onset of dementia before 65 years of age is termed as young-onset dementia (YOD). Very little literature exists regarding the clinical features and diagnoses of dementia in younger individuals. We present a case series of four patients of age 10 to 23 years with severe dementia within 18 months of clinical onset (rapidly progressive dementia). Three patients had generalised periodic complexes typical of subacute sclerosing panencephalitis (SSPE) on electroencephalogram (EEG). All patients had elevated cerebrospinal fluid (CSF) IgG measles antibodies. Our case series highlights that SSPE is an important cause of rapidly progressive YOD in developing countries like India.

  7. A 22-year-old Australian woman with atypical subacute sclerosing panencephalitis diagnosed at postmortem.

    Science.gov (United States)

    Fabian, Victoria A; Lee, Hwei-Yee; Keith-Rokosh, Julia Lee; de Souza, Jean-Louis; Stewart-Wynne, Edward

    2010-09-01

    Measles remains a significant global health problem. Despite the decline in measles prevalence in Australia following the implementation of a national vaccination program, challenges surrounding this disease remain. This report describes a 22-year-old woman who presented with coordination loss, tremor, choreiform movements and marked visual blurring, and her condition rapidly deteriorated to coma and death. Antemortem investigations did not yield a unifying diagnosis. Postmortem examination provided a diagnosis of subacute sclerosing panencephalitis. This patient had a rare neurological complication of measles infection, and her condition is remarkable for the atypical clinical presentation. Copyright 2010. Published by Elsevier Ltd.

  8. Subacute Sclerosing Panencephalitis Presenting with Isolated Positive Psychotic and Catatonic Symptoms.

    Science.gov (United States)

    Parmar, Arpit; Ranjan, Rajeev; Sagar, Rajesh

    2017-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare slowly progressing neurological illness. Although patients with SSPE initially present with symptoms such as myoclonic jerks, cognitive decline, and personality/behavioral changes usually, rarely pure psychiatric symptoms (e.g., mania, psychosis, and catatonia) have also been reported during the initial course of the illness. We report an unusual case of an adolescent with SSPE presenting with prominent positive psychotic and catatonic symptoms with the absence of classical symptoms of SSPE in initial course of illness and further discussed the relevant literature.

  9. Sclerosing liposarcoma of epididymis: Role of chemical shift magnetic resonance imaging

    Directory of Open Access Journals (Sweden)

    Subramaniyan Ramanathan

    2016-01-01

    Full Text Available Sclerosing liposarcoma of epididymis is a rare extratesticular scrotal tumor with variable prognosis. Ultrasonography is the initial imaging modality of choice for the evaluation of scrotal mass and helps to differentiate testicular and extratesticular masses, thereby narrowing down the differential diagnosis. Magnetic resonance imaging with its excellent soft tissue resolution can help in the further characterization of the nature of the tumor. In this case report, we highlight the role of chemical shift imaging in making a confident preoperative diagnosis of liposarcoma thereby guiding optimal and timely management.

  10. Sclerosing adenosis: mammographic and ultrasonographic findings with clinical and histopathological correlation

    Energy Technology Data Exchange (ETDEWEB)

    Guenhan-Bilgen, Isil E-mail: isilbilgen@hotmail.com; Memis, Aysenur; Uestuen, Esin Emin; Oezdemir, Necmettin; Erhan, Yildiz

    2002-12-01

    Objective: To evaluate the mammographic and ultrasonographic findings of sclerosing adenosis, a relatively uncommon entity which may sometimes mimic carcinoma. Materials and methods: A retrospective review of the records of 33700 women, who have undergone mammographic examination at our institution between January 1985 and July 2001 revealed 43 histopathologically proven sclerosing adenosis. The history, physical examination, mammographic and ultrasonographic findings were analyzed in all patients. In 30 patients, the nonpalpable lesions were preoperatively localized by the needle-hookwire system under the guidance of mammography (n=22) or ultrasonography (US) (n=8). Radiological features were correlated with histopathological findings. Results: The age of the patients varied between 32 and 55 years (mean, 43.7 years). Only two patients had a family history of breast cancer. In six patients, the presenting complaint was mastalgia. A palpable mass was present in 13 cases. The mammographic findings were; microcalcifications in 24 (55.8%) (clustered in 22, diffuse in two), mass in five (11.6%), asymmetric focal density in three (6.9%), and focal architectural distortion in three (6.9%) patients. Four of the masses were irregularly contoured, while one was well-circumscribed. On US, focal acoustic shadowing without a mass configuration was noted in the three patients who showed asymmetrical focal density on mammography. In eight patients, who showed normal mammograms, a solid mass was detected on US. Two masses had discrete well-circumscribed oval or lobulated contours, while six showed microlobulation and irregularity. In one case, the irregularly contoured mass had marked posterior acoustic shadowing. Two of the three patients, who had focal architectural distortion on mammograms, had an irregularly contoured solid mass, while the third presented as focal acoustic shadowing without a mass configuration. Conclusion: Sclerosing adenosis mostly presents as a nonpalpable

  11. Sclerosing mucoepidermoid carcinoma with eosinophilia of the thyroid: A cytological dilemma

    Directory of Open Access Journals (Sweden)

    Chayanika Pantola

    2016-01-01

    Full Text Available Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE of the thyroid is a rare primary thyroid tumor arising in a background of Hashimoto′s/lymphocytic thyroiditis and has been recently introduced in the World Health Organization (WHO classification of thyroid tumors. It is characterized by extensive sclerosis, squamous and glandular differentiation, and inflammatory infiltrate rich in eosinophil. Here, we are discussing the cytological features of this rare case in a 35-year-old female presented with thyroid swelling and lymph-node enlargement.

  12. Garre's chronic diffuse sclerosing osteomyelitis of the sacrum: a rare condition mimicking malignancy.

    LENUS (Irish Health Repository)

    Nasir, N

    2012-02-03

    Garre\\'s chronic diffuse sclerosing osteomyelitis (DSOM) is a rare disease that occurs most commonly in the mandible. We present a case of sacral DSOM that simulated an expanding destructive sacral tumour. Treatment was conducted on the basis of the available experience with the mandibular form of the disease, with partial symptomatic relief, but progressive sclerosis of the sacral lesion. To the best of our knowledge, this is the first case initially presenting in the sacrum. As an osteolytic expanding lesion simulating malignancy, it is important to recognize this entity in the sacrum.

  13. Natural history of idiopathic diabetes insipidus.

    Science.gov (United States)

    Richards, Gail E; Thomsett, Michael J; Boston, Bruce A; DiMeglio, Linda A; Shulman, Dorothy I; Draznin, Martin

    2011-10-01

    To determine what percentage of diabetes insipidus (DI) in childhood is idiopathic and to assess the natural history of idiopathic DI. We conducted a retrospective chart review of 105 patients with DI who were born or had DI diagnosed between 1980-1989 at 3 medical centers. A second cohort of 30 patients from 6 medical centers in whom idiopathic DI was diagnosed after 1990 was evaluated retrospectively for subsequent etiologic diagnoses and additional hypothalamic/pituitary deficiencies and prospectively for quality of life. In the first cohort, 11% of patients had idiopathic DI. In the second cohort, additional hypothalamic/pituitary hormone deficiencies developed in 33%, and 37% received an etiologic diagnosis for DI. Health-related quality of life for all the patients with idiopathic DI was comparable with the healthy reference population. Only a small percentage of patients with DI will remain idiopathic after first examination. Other hormone deficiencies will develop later in one-third of those patients, and slightly more than one-third of those patients will have an etiology for the DI diagnosed. Long-term surveillance is important because tumors have been diagnosed as long as 21 years after the onset of DI. Quality of life for these patients is as good as the reference population. Copyright © 2011 Mosby, Inc. All rights reserved.

  14. Suspected poisoning of domestic animals by pesticides.

    Science.gov (United States)

    Caloni, Francesca; Cortinovis, Cristina; Rivolta, Marina; Davanzo, Franca

    2016-01-01

    A retrospective study was carried out by reviewing all suspected cases of domestic animal poisoning attributed to pesticides, reported to the Milan Poison Control Centre (MPCC) between January 2011 and December 2013. During this period, pesticides were found to be responsible for 37.3% of all suspected poisoning enquiries received (815). The most commonly species involved was the dog (71.1% of calls) followed by the cat (15.8%), while a limited number of cases involved horses, goats and sheep. Most cases of exposure (47.1%) resulted in mild to moderate clinical signs. The outcome was reported in 59.9% of these cases, with death occurring in 10.4% of them. Insecticides (40.8%) proved to be the most common group of pesticides involved and exposure to pyrethrins-pyrethroids accounted for the majority of calls. According to the MPCC data, there has been a decrease in the number of suspected poisonings cases attributed to pesticides that have been banned by the EU, including aldicarb, carbofuran, endosulfan and paraquat. In contrast, there has been an increase of suspected poisoning cases attributed to the neonicotinoids, imidacloprid and acetamiprid, probably due to their widespread use in recent years. Cases of suspected poisoning that involved exposure to rodenticides accounted for 27.6% of calls received by the MPCC and anticoagulant rodenticides were the primary cause of calls, with many cases involving brodifacoum and bromadiolone. Herbicides were involved in 14.2% of calls related to pesticides and glyphosate was the main culprit in cases involving dogs, cats, horses, goats and sheep. As far as exposure to molluscicides (11.5%) and fungicides (5.9%), most of the cases involved dogs and the suspected poisoning agents were metaldehyde and copper compounds respectively. The data collected are useful in determining trends in poisoning episodes and identifying newly emerging toxicants, thus demonstrating the prevalence of pesticides as causative agents in animal

  15. Idiopathic Ophthalmodynia and Idiopathic Rhinalgia: A Prospective Series of 16 New Cases.

    Science.gov (United States)

    Pareja, Juan A; Montojo, Teresa; Guerrero, Ángel L; Álvarez, Mónica; Porta-Etessam, Jesús; Cuadrado, María L

    2015-01-01

    Idiopathic ophthalmodynia and idiopathic rhinalgia were described a few years ago. These conditions seem specific pain syndromes with a distinctive location in the eye or in the nose. We aimed to present a new prospective series in order to verify the consistency of these syndromes. We performed a descriptive study of all patients referred to our regional neurologic clinics from 2010 to 2014 because of facial pain exclusively felt in the eye or in the nose fulfilling the proposed diagnostic criteria for idiopathic ophthalmodynia and idiopathic rhinalgia. There were 9 patients with idiopathic ophthalmodynia and 7 patients with idiopathic rhinalgia, with a clear female preponderance, and a mean age at onset in the fifth decade. The pain was usually moderate and the temporal pattern was generally chronic. Only one patient reported accompaniments (hypersensitivity to the light and to the flow of air in the symptomatic eye). Preventive treatment with amitriptyline, pregabalin, or gabapentin was partially or totally effective. The clinical features of this new series parallels those of the original description, thus indicating that both idiopathic ophthalmodynia and idiopathic rhinalgia have clear-cut clinical pictures with excellent consistency both inter- and intra-individually. © 2015 American Headache Society.

  16. The evaluation of suspected child physical abuse.

    Science.gov (United States)

    Christian, Cindy W

    2015-05-01

    Child physical abuse is an important cause of pediatric morbidity and mortality and is associated with major physical and mental health problems that can extend into adulthood. Pediatricians are in a unique position to identify and prevent child abuse, and this clinical report provides guidance to the practitioner regarding indicators and evaluation of suspected physical abuse of children. The role of the physician may include identifying abused children with suspicious injuries who present for care, reporting suspected abuse to the child protection agency for investigation, supporting families who are affected by child abuse, coordinating with other professionals and community agencies to provide immediate and long-term treatment to victimized children, providing court testimony when necessary, providing preventive care and anticipatory guidance in the office, and advocating for policies and programs that support families and protect vulnerable children. Copyright © 2015 by the American Academy of Pediatrics.

  17. Improved survival with primary sclerosing cholangitis. A review of clinicopathologic features and comparison of symptomatic and asymptomatic patients.

    Science.gov (United States)

    Helzberg, J H; Petersen, J M; Boyer, J L

    1987-06-01

    The clinicopathologic features and natural history of primary sclerosing cholangitis were reviewed in 53 patients followed at the Yale Liver Center during the past 30 yr. At presentation, the mean age of patients was 46 yr, and the male to female ratio was 1.4:1. Biliary sclerosis was limited to the intrahepatic ductal system in 21% of the patients. Fifty-three percent of the patients had mild disease without portal hypertension at presentation, and 25% had no symptoms attributable to their liver disease. Long-term follow-up was available for 42 patients and averaged 56 mo. Over this period, 16 patients remained mildly symptomatic, and 11 were asymptomatic. Survival was calculated by a Kaplan-Meier life-table analysis and demonstrated that 75% of the patients were alive 9 yr after the diagnosis of primary sclerosing cholangitis. A multivariate analysis of clinical features revealed that hepatomegaly and a serum bilirubin level greater than 1.5 mg/dl at the onset of disease were independent discriminators of a poor prognosis. Patients referred to this university medical center displayed different clinical characteristics than previously reported in primary sclerosing cholangitis. A higher percentage were older, female, and asymptomatic, and more had disease limited to the intrahepatic ductal system. Survival was also considerably improved in this group of patients and suggests that the long-term prognosis for patients with primary sclerosing cholangitis may be considerably better than previously believed.

  18. Sclerosing cholangitis following severe trauma: description of a remarkable disease entity with emphasis on possible pathophysiologic mechanisms.

    Science.gov (United States)

    Benninger, Johannes; Grobholz, Rainer; Oeztuerk, Yurdaguel; Antoni, Christoph H; Hahn, Eckhart G; Singer, Manfred V; Strauss, Richard

    2005-07-21

    Persistent cholestasis is a rare complication of severe trauma or infections. Little is known about the possible pathomechanisms and the clinical course. Secondary sclerosing cholangitis was diagnosed in five patients with persistent jaundice after severe trauma (one burn injury, three accidents, one power current injury). Medical charts were retrospectively reviewed with regard to possible trigger mechanisms for cholestasis, and the clinical course was recorded. Diagnosis of secondary sclerosing cholangitis was based in all patients on the primary sclerosing cholangitis (PSC)-like destruction of the intrahepatic bile ducts at cholangiography after exclusion of PSC. In four patients, arterial hypotension with subsequent ischemia may have caused the bile duct damage, whereas in the case of power current injury direct thermal damage was assumed to be the trigger mechanism. The course of secondary liver fibrosis was rapidly progressive and proceeded to liver cirrhosis in all four patients with a follow-up >2 years. Therapeutic possibilities were limited. Posttraumatic sclerosing cholangitis is a rare but rapidly progressive disease, probably caused by ischemia of the intrahepatic bile ducts via the peribiliary capillary plexus due to arterial hypotension. Gastroenterologists should be aware of this disease in patients with persistent cholestasis after severe trauma.

  19. Case report: Cervical spinal cord signal changes in a case of adult-onset subacute sclerosing panenchephalitis

    Directory of Open Access Journals (Sweden)

    Sharma Sandeep

    2010-01-01

    Full Text Available In this article, we report a case of subacute sclerosing panencephalitis (SSPE in which there were central cervical cord signal changes on MRI. The spinal cord is uncommonly involved in SSPE. However, demonstration of spinal cord signal change in a patient of SSPE has significant implications for the differential diagnosis and management

  20. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2

    DEFF Research Database (Denmark)

    Knobler, R.; Moinzadeh, P.; Hunzelmann, N.

    2017-01-01

    The term sclerosing diseases of the skin' comprises specific dermatological entities which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish...

  1. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1

    DEFF Research Database (Denmark)

    Knobler, R.; Moinzadeh, P.; Hunzelmann, N.

    2017-01-01

    The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distingui...

  2. Suspects in criminal investigations of rape

    Directory of Open Access Journals (Sweden)

    Marinković Darko

    2014-01-01

    Full Text Available Investigations of sexual assaults mostly focus on victims and their credibility, which may cause lack of firm evidence in relation to suspects. Given the fact that the criminal offence of rape is characterised by a high incidence of false reports and accusations, frequently indicating specific persons as the perpetrators, certain caution is necessary in the investigation in order to avoid false accusations and/or convictions. As regards the personality of the rapist and motives for committing a forcible sexual act, certain types or rather certain categories of perpetrators can be distinguished, although it should be noted that a large number of rapists do not belong to one category only, but rather combine characteristics of several different types. During a criminal investigation it is of vital importance to differentiate between a rape as a surprise attack and a rape as abuse of trust, as they are compatible with the nature of the suspect's defence. The suspect shall be subjected to a forensic examination in the course of the investigation in order to find traces which prove vaginal, anal or oral penetration, coerced sexual intercourse and identity of the rapist. While conducting an interrogation of a suspected rapist, a crime investigating officer shall use either factual or emotional approach to his interviewee, depending on his psychological and motivational characteristics. In this regard, the factual approach is believed to be more efficient with anger rapists and sadistic rapists, whereas the compassionate approach gives good results with the gentlemen-rapists and partly with the power asserting rapists.

  3. Glaucoma suspect & Humphrey Field Analyzer a correlation

    Directory of Open Access Journals (Sweden)

    P Dahal

    2012-09-01

    Full Text Available Glaucoma originally meant "clouded", in Greek.The term glaucoma refers to a group of diseases that have in common characteristic optic neuropathy with associated visual field loss for which elevated intraocular pressure is one of the primary risk factor. The purpose of the study is to correlate the clinically diagnosed cases of glaucoma suspect with the Humphrey Field Analyzer (HFA. Fifty cases of glaucoma suspect who attended the glaucoma clinic of Nepal Eye Hospital Tripureswor, Kathmandu, Nepal and who meets at least two criteria, among the four types of glaucoma suspects were advised for the HFA for the study. In this study out of 50 patient, 36 (72% patients had normal visual field. 14 (28% patients had thinning of the neural retinal rim (NRR in both eyes. The significant relation with thinning of neural retina rim and glaucomatous hemifield test was found in the study. Journal of College of Medical Sciences-Nepal,2012,Vol-8,No-1, 23-28 DOI: http://dx.doi.org/10.3126/jcmsn.v8i1.6822

  4. Tocolytics for suspected intrapartum fetal distress.

    Science.gov (United States)

    Kulier, R; Hofmeyr, G J

    2000-01-01

    Prophylactic tocolysis with betamimetics and other agents has become widespread as a treatment for fetal distress. Uterine relaxation may improve placental blood flow and therefore fetal oxygenation. However there may also be adverse maternal cardiovascular effects. The objective of this review was to assess the effects of tocolytic therapy for suspected fetal distress on fetal, maternal and perinatal outcomes. We searched the Cochrane Pregnancy and Childbirth Group trials register and the Cochrane Controlled Trials Register. Date of last search: February 1999. Randomised trials comparing tocolytic therapy with no treatment or treatment with another tocolytic agent for suspected fetal distress. Two reviewers assessed trial quality and extracted data. Three studies were included. Compared with no treatment, there were fewer failed improvements in fetal heart rate abnormalities with tocolytic therapy (relative risk 0.26, 95% 0.13 to 0.53). Betamimetic therapy compared with magnesium sulphate showed a non-significant trend towards reduced uterine activity (relative risk 0.07, 95% confidence interval 0.00 to 1.10). Betamimetic therapy appears to be able to reduce the number of fetal heart rate abnormalities and perhaps reduce uterine activity. However there is not enough evidence based on clinically important outcomes to evaluate the use of betamimetics for suspected fetal distress.

  5. Efficacy and safety of erythromycin as sclerosing agent in patients with recurrent malignant pleural effusion.

    Science.gov (United States)

    Balassoulis, George; Sichletidis, Lazaros; Spyratos, Dionisios; Chloros, Diamantis; Zarogoulidis, Kostas; Kontakiotis, Theodoros; Bagalas, Vassilios; Porpodis, Kostas; Manika, Katerina; Patakas, Dimitrios

    2008-08-01

    The aim of pleurodesis in malignant pleural effusions is to prevent reaccumulation of the fluid, symptoms, and avoid the need for repeated hospitalization for thoracentesis. The purpose of this study was to evaluate the efficacy and safety of erythromycin as a pleural sclerosing agent. Over a 2-year period, 34 patients with a symptomatic, recurrent, malignant pleural effusion who referred for chest tube drainage and pleurodesis were included. They had not received prior intrapleural therapy and had predicted survival of at least 1 month. All underwent pleural drainage and chemical pleurodesis with erythromycin. Complications and response to pleurodesis, according to clinical and radiographic criteria after 90 days, were recorded. The overall response was 88.2%. Complete response (no reaccumulation of pleural fluid after 90 days) was observed in 27 patients (79.4%). Partial response (reaccumulation of fluid but without symptoms, not requiring drainage) was observed in 3 (8.8%). No response (symptomatic reaccumulation of fluid that required drainage) was observed in 4 (11.8%). All patients experienced pleurodynia that was treated with administration of paracetamol and/or dextropropoxyphene. Sinus tachycardia and concurrent mild systemic hypertension were observed 2 and 4 hours after pleurodesis. Both of them were attributed to pleurodynia as there was remission with analgesics. This study suggests that erythromycin is effective and safe as a sclerosing agent for pleurodesis in patients with recurrent malignant pleural effusions.

  6. Prognostic role of natural killer cells in pediatric mixed cellularity and nodular sclerosing Hodgkin's disease.

    Science.gov (United States)

    Ortaç, Ragip; Aktaş, Safiye; Diniz, Gülden; Erbay, Ayşe; Vergin, Canan

    2002-10-01

    To study natural killer cells' spontaneous cytotoxic capacity against tumor cells and their prognostic significance in classical Hodgkin's disease. Thirty-eight pediatric mixed cellularity and nodular sclerosing Hodgkin's disease patients were included in the study. Immunohistochemical staining was performed for natural killer cells in the background using the monoclonal antibody CD57 in serial sections of B5-formalin-fixed, paraffin-embedded tissue blocks. CD57-positive cells were counted with an immersion objective among 5,000 cells on representative areas of the tumors. The degree of natural killer cells was classified as low ( or = 150 cells). Multivariate regression analysis was performed to determine the differences between patients with and without relapse. The mean of CD57-positive cell numberfor all the cases was 173.42 +/- 117.34 (range, 20-500). CD57-positive cells were high in 21 cases and low in 17. The mean of CD57-positive cell numbers was 191.85 +/- 115.33 in the disease-free group and 84.44 +/- 57.90 in the relapsing group. Log rank analysis showed statistical significance between event-free survival and number of CD57-positive cells (P = .0207). In multivariate analysis, CD57 expression proved to be a prognostic factor independent from otherfactors. As a result, CD57 expression by background natural killer cells may be used as a prognostic parameter in mixed cellularity and nodular sclerosing Hodgkin's disease.

  7. Vascular sclerosing effects of bleomycin on cutaneous veins: a pharmacopathologic study on experimental animals.

    Science.gov (United States)

    AlGhamdi, Khalid M; Kumar, Ashok; Ashour, Abdelkader E; Al-Rikabi, Ammar C; AlOmrani, Abdullah Hasan; Ahamed, Shaik Shaffi

    2017-01-01

    Varicose veins and the complications of venous disease are common disorders in humans. To study the effects of bleomycin as a potential new sclerosing agent and its adverse events in treating varicose veins. Bleomycin-loaded liposomes 0.1ml was injected in the dorsal ear veins of white New Zealand rabbits. Sodium tetradecyl sulfate was used as a positive control. Normal saline was used as negative control. The blood vessels of the treated ears were photographed before and at one hour and two, eight and 45 days after treatment. Biopsies from the treated areas were obtained for histological examination. Blood samples were collected to determine any possible toxicity. Bleomycin by itself was ineffective; therefore, liposomes were used as a vector to deliver bleomycin to the vein lumen. Subsequently, bleomycin started showing its sclerosing effects. Toxicity monitoring showed no apparent hematologic, pulmonary, hepatic or renal toxicities. This study revealed that bleomycin induced vasculitis, which led to vascular occlusion, which was observed on day 1 and day 8. No bleomycin-related injury was noted by histopathological examination of lung sections. The calculation of the lung/body weight coefficient indicated that edema was present in the experimental groups compared with the negative and positive controls. Relatively small number of experimental animals used. This study showed that bleomycin-loaded liposomes were able to induce vasculitis and vascular occlusion without any toxicity or complications. It might be useful, hence, to treat patients suffering from Varicose veins and other ectatic vascular diseases with this agent.

  8. Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis

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    P J Lorenzoni

    2011-01-01

    Full Text Available Myasthenia gravis (MG is an immune-mediated disease that compromises the postsynaptic membrane of the neuromuscular junction. Primary sclerosing cholangitis (PSC is considered an immune-mediated cholestatic liver disease. Both MG and PSC include an autoimmune pathogenesis, so there is some evidence that patients with MG or PSC have a higher risk of developing autoantibodies and other immune disorders than normal controls, but the coexistence of these two disorders has never been documented. We report a 40-year-old woman who presented with MG when she was 20 years old and developed PSC 20 years after a thymectomy. Liver biochemistry revealed cholestasis. Magnetic resonance imaging showed multifocal strictures and beads involving the intrahepatic bile ducts. A liver biopsy confirmed sclerosing cholangitis. Serological analysis demonstrated positive autoantibodies (Anti-nuclear antibodies, anti-smooth muscle antibodies. Repetitive stimulation had a decremental response, and antibodies to acetylcholine receptors were detectable. To our knowledge, this is the first case of PSC in a patient with MG. The main characteristics of both MG and PSC combination are discussed.

  9. Contrasting Pattern of Chronic Inflammatory Bowel Disease in Primary and Autoimmune Sclerosing Cholangitis.

    Science.gov (United States)

    Bjarnason, Ingvar; Hayee, Bu; Pavlidis, Polychronis; Kvasnovsky, Charlotte; Scalori, Astrid; Sisson, Guy; Charlesworth, Annika; Shaikh, Hizbullah; Bjornsson, Einar; Heneghan, Michael A

    2015-10-01

    Primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (AISC) are related, but distinct chronic liver diseases. PSC is associated with a high prevalence of ulcerative colitis while the intestinal inflammation associated with AISC is less well characterised. To assess and contrast aspects of intestinal inflammation in patients with AISC and PSC and compare the clinical features with those of patients with ulcerative colitis and Crohn's disease. 23 and 22 patients with AISC and PSC, respectively, underwent review of colonoscopy and biopsy findings, capsule enteroscopy and assessment of clinical and inflammatory (faecal calprotectin) disease activity, which was compared with that of patients with ulcerative colitis and Crohn's disease (n = 55 each). Five and 6 patients with AISC and PSC, respectively, had normal colonoscopy and faecal calprotectin levels of 34.4 ± 8.3 and 39.7 ± 8.4 μg/g, respectively (normal 0.05) between patients with intestinal inflammation in AISC (588 ± 549 μg/g), PSC (421 ± 351 μg/g), ulcerative colitis (501 ± 656 μg/g) or Crohn's disease (476 ± 571 μg/g). Capsule enteroscopy showed that 7 of 18 (39%) (p < 0.03) of those with AISC had small bowel mucosal breaks whereas no patient with PSC had these findings. Collectively these findings lend support to the suggestion that the chronic inflammatory bowel disease associated with PSC and in particular AISC may represent a distinct nosologic entity different from classic ulcerative colitis and Crohn's disease.

  10. Serum S-100B levels in children with Subacute Sclerosing Panencephalitis

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    İbrahim Hocanlı

    2012-09-01

    Full Text Available Objectives: S-100B a protein prevalent in the centralnervous system is a peripheral biomarker for blood-brainbarrier disruption and neuronal damage. The objective ofthe study was to investigate the S-100B levels in patientswith subacute sclerosing panencephalitis.Materials and methods: A group of 40 patients withSSPE and 40 healthy controls were recruited. SerumS-100B protein concentrations were measured using acommercially available electrochemiluminescence immunoassay(ECLIA kit, as supplied and according to themanufacturer’s standards.Results: Median S-100B levels were 0.095 ± 0.017 μg/Lin patients with SSPE and 0.097±0.019 μg/L in the controlgroup. This difference was not statistically significant(p>0.05. The patient group was further subdivided intotwo subgroups according to the presence or absence ofbrain atrophy. The S-100B levels were 0.096 ± 0.018 μg/Lin the subgroup with atrophy and 0.094±0.014 μg/L in thesubgroup without atrophy. This difference was also notstatistically significant (p>0.05.Conclusions: Our results suggest that serum S-100B isnot a reliable marker for neuronal damage in SSPE. J ClinExp Invest 2012; 3 (3: 331-334Key words: Subacute sclerosing panencephalitis, S-100B level, serum biomarker

  11. IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material.

    Science.gov (United States)

    Deshpande, Vikram; Sainani, Nisha I; Chung, Raymond T; Pratt, Daniel S; Mentha, Gilles; Rubbia-Brandt, Laura; Lauwers, Gregory Y

    2009-10-01

    IgG4-associated cholangitis is a steroid-responsive hepatobiliary inflammatory condition associated with autoimmune pancreatitis that clinically and radiologically mimics primary sclerosing cholangitis. In this study, we conducted a morphological and immunohistochemical analysis of liver material obtained from individuals with IgG4-associated cholangitis, and compared these with well-characterized cases of primary sclerosing cholangitis. The study group consisted of 10 patients (9 biopsy and 1 hepatectomy case) with IgG4-associated cholangitis and 17 patients with primary sclerosing cholangitis (16 needle biopsy and 1 hepatectomy case). All patients with IgG4-associated cholangitis had pancreatic involvement as well, and six pancreatectomy samples revealed characteristic histopathological features of autoimmune pancreatitis. Primary sclerosing cholangitis cases were defined by the presence of a characteristic ERCP appearance. Clinical, pathological, radiological, and follow-up data were recorded for all cases. Portal and periportal inflammation was graded according to Ishak's guidelines. Immunohistochemical stains for IgG and IgG4 were performed. The cohort of patients with IgG4-associated cholangitis (mean age: 63 years) was older than individuals with primary sclerosing cholangitis (mean age: 44 years). Seven of these cases showed intrahepatic biliary strictures. IgG4-associated cholangitis liver samples showed higher portal (P=0.06) and lobular (P=0.009) inflammatory scores. Microscopic portal-based fibro-inflammatory nodules that were composed of fibroblasts, plasma cells, lymphocytes, and eosinophils were exclusively observed in five of the IgG4-associated cholangitis cases (50%). More than 10 IgG4-positive plasma cells per HPF (high power field) were observed in 6 of the IgG4-associated cholangitis cases (mean: 60, range: 0-140 per HPF), whereas all primary sclerosing cholangitis cases showed significantly lesser numbers (mean: 0.08, range: 0-1 per HPF). On a

  12. Perspective: Update on Idiopathic Intracranial Hypertension

    Science.gov (United States)

    Bruce, Beau B.; Biousse, Valérie; Newman, Nancy J.

    2011-01-01

    Purpose Provide an update on various features of idiopathic intracranial hypertension. Design Perspective. Methods Selected articles on the epidemiology, clinical and imaging features, natural history, pathophysiology, and treatment of idiopathic intracranial hypertension were reviewed and interpreted in the context of the authors’ clinical and research experience. Results Idiopathic intracranial hypertension is primarily a disease of obese women of childbearing age, but it can affect patients of any weight, sex, and age. Although a relatively rare disorder, idiopathic intracranial hypertension’s associated costs in the U.S. entail hundreds of millions of dollars. Even following treatment, headaches are frequently persistent and may require the continued involvement of a neurologist. Quality of life reductions and depression are common among idiopathic intracranial hypertension patients. However, visual dysfunction, especially visual field abnormalities, represents the major morbidity of this disorder, and serial automated perimetry remains the primary mode of patient monitoring. Patients who are men, black, very obese, or anemic are at higher risk of visual loss. Vitamin A metabolism, adipose tissue as an actively secreting endocrine tissue, and cerebral venous abnormalities are areas of active study regarding idiopathic intracranial hypertension’s pathophysiology. Treatment studies show that lumbar puncture is a valuable treatment (in addition to its crucial diagnostic role) and that weight management is critical. However, open questions remain regarding the efficacy of acetazolamide, CSF diversion procedures, and cerebral venous stenting. Conclusions Many questions remain unanswered about idiopathic intracranial hypertension. Ongoing studies, especially an ongoing NIH-funded clinical trial of acetazolamide, should provide more insight into this important, yet poorly understood syndrome of isolated intracranial hypertension. PMID:21696699

  13. Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease

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    Cristina Suárez-Ferrer

    2016-02-01

    Full Text Available Introduction: The possibility of developing idiopathic portal hypertension has been described with thiopurine treatment despite compromises the prognosis of these patients, the fact its true prevalence is unknown. Material and methods: A cross-sectional study was conducted in a cohort of inflammatory bowel disease (IBD patients followed at our unit, to determine the prevalence of diagnosis of idiopathic portal hypertension (IPH and its relationship with thiopurine treatment. Results: At the time of the analysis, 927/1,419 patients were under treatment with thiopurine drugs (65%. A total of 4 patients with IBD type Crohn's disease with idiopathic portal hypertension probably related to the thiopurine treatment were identified (incidence of 4.3 cases per 1,000. Seventy-five percent of patients started with signs or symptoms of portal hypertension. Only one patient was asymptomatic but the diagnosis of IPH because of isolated thrombocytopenia is suspected. However, note that all patients had thrombocytopenia previously. Abdominal ultrasound with fibroscan, hepatic vein catheterization and liver biopsy were performed on all of them as part of the etiology of portal hypertension. In the abdominal ultrasound, indirect portal hypertension data were observed in all patients (as splenomegaly cirrhosis was also ruled out. The fibroscan data showed significant liver fibrosis (F2-F3. Conclusion: Idiopathic portal hypertension following thiopurine treatment in IBD patients is a rare occurrence, but it must be borne in mind in the differential diagnosis for early diagnosis, especially in patients undergoing thiopurine treatment over a long period. The presence of thrombocytopenia is often the only predictor of its development in the preclinical stage.

  14. Idiopathic aneurysm of pulmonary artery

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    Pacheco, Julio B. Cota; Pimentel, Patricia N.; Knust, Beatriz S., E-mail: jcota@uol.com.br [Clinica de Cardiologia Cota Pacheco, Mogi das Cruzes, SP (Brazil)

    2015-07-15

    Because it is a very rare isolated lesion, we decided to present this case of idiopathic pulmonary artery aneurysm (IPAA) and review the cases published in the literature in order to correlate our clinical and imaging findings, as well as management based on patient data. IPAA was first described in a case of autopsy by Bristowe in 1860 and later in 1947 by Deterling and Claggett, whose prevalence was lower than eight to one hundred thousand. Although the use of diagnostic imaging methods has been applied in a very large population in recent decades, this lesion has been most often described in postmortem examinations. Therefore, it is important to be aware of possible clinical symptoms, at times non-specific, as well as the signs on imaging studies. In this study, therefore, the report of a case of an asymptomatic patient whose diagnosis was done through color Doppler echocardiography in a routine test in 2012, later confirmed by simple chest computed tomography (chest CT) and cardiac catheterization as IPAA and its branches. We discussed the literature available and the possibilities for treatment and the use of color Doppler echocardiography as an initial diagnostic tool for such a rare and intriguing disease. (author)

  15. Meningoceles in Idiopathic Intracranial Hypertension

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    Bialer, Omer Y.; Rueda, Mario Perez; Bruce, Beau B.; Newman, Nancy J.; Biousse, Valérie; Saindane, Amit M.

    2014-01-01

    OBJECTIVE MRI abnormalities have been described in patients with increased intracranial pressure (ICP), including in those with idiopathic intracranial hypertension (IIH). Spontaneous cerebral spinal fluid (CSF)-filled outpouchings of the dura (meningoceles), and secondary CSF leaks can occur from elevated ICP in patients with IIH, however, few studies have evaluated these findings. Our objective was to evaluate the frequency of spontaneous intracranial meningoceles among IIH patients and determine their association with visual outcome. SUBJECTS AND METHODS We performed a retrospective case-control study of consecutive IIH patients between 2000 and 2011 who underwent MRI including T2-weighted imaging. Demographics, presenting symptoms, CSF opening pressure, and visual outcome were collected for the first and last evaluations. Controls included patients without headache or visual complaints with normal brain MRIs. Stratified analysis was used to control for potential confounding by age, gender, race, and body mass index. RESULTS We included 79 IIH patients and 76 controls. Meningoceles were found in 11% of IIH patients versus 0% of controls (pmeningoceles were found in 9% of IIH patients versus 0% of controls (pmeningocele or prominent Meckel’s caves was not associated with demographics, symptoms, degree of papilledema, CSF opening pressure, visual acuity, or visual field defect severity. CONCLUSION Meningoceles are significantly more common in IIH patients than in controls, and can be considered an additional imaging sign for IIH. Meningoceles are not, however, associated with decreased CSF opening pressure or better visual outcome in IIH. PMID:24555598

  16. Autophagy in idiopathic pulmonary fibrosis.

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    Avignat S Patel

    Full Text Available Autophagy is a basic cellular homeostatic process important to cell fate decisions under conditions of stress. Dysregulation of autophagy impacts numerous human diseases including cancer and chronic obstructive lung disease. This study investigates the role of autophagy in idiopathic pulmonary fibrosis.Human lung tissues from patients with IPF were analyzed for autophagy markers and modulating proteins using western blotting, confocal microscopy and transmission electron microscopy. To study the effects of TGF-β(1 on autophagy, human lung fibroblasts were monitored by fluorescence microscopy and western blotting. In vivo experiments were done using the bleomycin-induced fibrosis mouse model.Lung tissues from IPF patients demonstrate evidence of decreased autophagic activity as assessed by LC3, p62 protein expression and immunofluorescence, and numbers of autophagosomes. TGF-β(1 inhibits autophagy in fibroblasts in vitro at least in part via activation of mTORC1; expression of TIGAR is also increased in response to TGF-β(1. In the bleomycin model of pulmonary fibrosis, rapamycin treatment is antifibrotic, and rapamycin also decreases expression of á-smooth muscle actin and fibronectin by fibroblasts in vitro. Inhibition of key regulators of autophagy, LC3 and beclin-1, leads to the opposite effect on fibroblast expression of á-smooth muscle actin and fibronectin.Autophagy is not induced in pulmonary fibrosis despite activation of pathways known to promote autophagy. Impairment of autophagy by TGF-β(1 may represent a mechanism for the promotion of fibrogenesis in IPF.

  17. Idiopathic scoliosis and the vestibular system.

    Science.gov (United States)

    Hawasli, Ammar H; Hullar, Timothy E; Dorward, Ian G

    2015-02-01

    Despite its high prevalence, the etiology underlying idiopathic scoliosis remains unclear. Although initial scrutiny has focused on genetic, biochemical, biomechanical, nutritional and congenital causes, there is growing evidence that aberrations in the vestibular system may play a role in the etiology of scoliosis. In this article, we discuss putative mechanisms for adolescent idiopathic scoliosis and review the current evidence supporting a role for the vestibular system in adolescent idiopathic scoliosis. A comprehensive search of the English literature was performed using PubMed ( http://www.ncbi.nlm.nih.gov/pubmed ). Research articles studying interactions between adolescent idiopathic scoliosis and the vestibular system were selected and evaluated for inclusion in a literature review. Eighteen manuscripts of level 3-4 clinical evidence to support an association between adolescent idiopathic scoliosis (AIS) and dysfunction of the vestibular system were identified. These studies include data from physiologic and morphologic studies in humans. Clinical data are supported by animal model studies to suggest a causative link between the vestibular system and AIS. Clinical data and a limited number of animal model studies suggest a causative role of the vestibular system in AIS, although this association has not been reproduced in all studies.

  18. Zonisamide monotherapy for idiopathic epilepsy in dogs.

    Science.gov (United States)

    Chung, J Y; Hwang, C Y; Chae, J S; Ahn, J O; Kim, T H; Seo, K W; Lee, S Y; Youn, H Y

    2012-11-01

    To evaluate the efficacy of zonisamide as a monotherapy in dogs with idiopathic epileptic seizure. The experiment was conducted on 10 dogs with idiopathic epilepsy that were treated at the Seoul National University Hospital for Animals. A diagnosis was conducted based on physical and neurologic examination, complete blood count and chemical analysis, magnetic resonance imaging and cerebrospinal fluid analyses. Idiopathic epilepsy was diagnosed when all of these examinations were normal. Oral zonisamide was administrated to 10 dogs with idiopathic epilepsy at 5-15 mg/kg per os every 12 h to achieve a concentration of zonisamide in serum of 10-40 μg/mL. The frequency of seizures before and after the administration of zonisamide therapy was recorded and the concentrations of zonisamide in serum were measured. Six (60%) of the dogs were favourable responders to treatment, showing a ≥50% reduction in monthly frequency of seizures. Of the remaining four, two dogs did not show a reduction and the other two showed an increase in frequency of seizures. The mean dosage of zonisamide for favourable responders was 7.92 (SD 3.79) mg/kg, which was administered orally twice a day. Only one dog, which was one of the unfavourable responders in the whole study, experienced mild side effects. Among the dogs treated with oral zonisamide, 60% responded favourably. The effect of zonisamide as an anticonvulsant drug was demonstrated in this study. Based on these results, zonisamide monotherapy is effective in some dogs with idiopathic epilepsy.

  19. Three Ulcerative Colitis Susceptibility Loci Are Associated with Primary Sclerosing Cholangitis and Indicate a Role for IL2, REL, and CARD9

    NARCIS (Netherlands)

    Janse, Marcel; Lamberts, Laetitia E.; Franke, Lude; Raychaudhuri, Soumya; Ellinghaus, Eva; Boberg, Kirsten Muri; Melum, Espen; Folseraas, Trine; Schrumpf, Erik; Bergquist, Annika; Bjornsson, Einar; Fu, Jingyuan; Westra, Harm Jan; Groen, Harry J. M.; Fehrmann, Rudolf S. N.; Smolonska, Joanna; van den Berg, Leonard H.; Ophoff, Roel A.; Porte, Robert J.; Weismueller, Tobias J.; Wedemeyer, Jochen; Schramm, Christoph; Sterneck, Martina; Guenther, Rainer; Braun, Felix; Vermeire, Severine; Henckaerts, Liesbet; Wijmenga, Cisca; Ponsioen, Cyriel Y.; Schreiber, Stefan; Karlsen, Tom H.; Franke, Andre; Weersma, Rinse K.

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts. Both environmental and genetic factors contribute to its pathogenesis. To further clarify its genetic background, we investigated susceptibility loci recently

  20. Prevalence of glaucoma suspects and pattern of intra-ocular ...

    African Journals Online (AJOL)

    Background: Glaucoma is the commonest cause of irreversible blindness in the world. Some glaucoma patients start out as glaucoma suspects for years. Aim: To determine the prevalence of glaucoma suspects and pattern of intra-ocular pressure distribution in glaucoma suspects. Methods: This survey was carried out in ...

  1. Idiopathic bronchocentric granulomatosis in an asthmatic adolescent

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    Hiroki Umezawa

    2015-01-01

    Full Text Available Bronchocentric granulomatosis in asthmatic patients has been generally considered to be associated with allergic bronchopulmonary aspergillosis and represent a histopathologic manifestation of fungal hypersensitivity. Here we report a case of an idiopathic bronchocentric granulomatosis in a 17-year-old man with a history of asthma. He was admitted to the hospital with a fever and cough, and a chest CT scan showed peribronchial consolidation in the pulmonary parenchyma, which was unresponsive to antibiotic therapy. The pathological findings obtained by video-assisted thoracoscopic lung biopsy revealed necrotizing granulomatous inflammation centered on bronchi and bronchioles and there was no evidence of fungal colonization, resulting in a diagnosis of idiopathic bronchocentric granulomatosis. Systemic corticosteroid therapy led to clinical and radiological recovery. Physicians should take into account the possibility of the idiopathic process in bronchocentric granulomatosis of asthmatic patients.

  2. Nuclear Pedigree Criteria of Suspected HNPCC

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    Kładny Józef

    2003-01-01

    Full Text Available Abstract The criteria for the diagnosis of HNPCC established by the ICG-HNPCC are very restrictive as they do not allow for the diagnosis of a large number of "suspected HNPCC" cases - these are families which do no fulfill the strict diagnostic "Amsterdam criteria", but do present with several pedigree and clinical features characteristic for HNPCC. Several series of families suspected of harboring germline mutations in DNA mismatch repair genes have been studied for germline changes in DNA mismatch repair genes and a mutation rate of somewhere between 8-60% was found. Therefore a subgroup of members of the ICG-HNPCC has been working on pedigree/clinical diagnostic criteria for suspected HNPCC. Materials and methods Part I The study was based on two series of colorectal cancer (CRC cases: 1 HNPCC - this group comprised 190 patients affected by CRC from randomly selected families which fulfilled the Amsterdam II criteria registered in Düsseldorf, Germany (102 cases of CRC, Denmark (18 CRCs, Leiden, Holland (23 CRCs and Szczecin, Poland (47 CRCs. 2 Consecutive CRCs - this group comprised 629 (78.0% of 806 individuals with CRC diagnosed in 1991-1997 in the city of Szczecin (ca. 400,000 of inhabitants, Poland. Nuclear pedigrees in both groups were compared for frequency of occurrence of clinical features, that have been shown to be associated with HNPCC. Part II 52 consecutive CRC cases from Szczecin, matching the criteria recognized in part I as appropriate for diagnosis of cases "suspected of HNPCC" were studied for the occurrence of germline hMSH2/hMLH1 constitutional mutations using "exon by exon" sequencing. Results The combination of features - i.e. the occurrence of an HNPCC associated cancer (CRC or cancer of the endometrium, small bowel or urinary tract in a 1st degree relative of a CRC patient; at least one of the patients being diagnosed under age of 50 - appeared to be strongly associated to HNPCC with an OR - 161. Constitutional

  3. Pharmacological interventions for primary sclerosing cholangitis: an attempted network meta-analysis.

    Science.gov (United States)

    Saffioti, Francesca; Gurusamy, Kurinchi Selvan; Hawkins, Neil; Toon, Clare D; Tsochatzis, Emmanuel; Davidson, Brian R; Thorburn, Douglas

    2017-03-28

    Primary sclerosing cholangitis is a chronic cholestatic liver disease that is associated with both hepatobiliary and colorectal malignancies, which can result in liver cirrhosis and its complications. The optimal pharmacological treatment for patients with primary sclerosing cholangitis remains controversial. To assess the comparative benefits and harms of different pharmacological interventions in people with primary sclerosing cholangitis by performing a network meta-analysis, and to generate rankings of available pharmacological interventions according to their safety and efficacy. Given that it was not possible to assess whether potential effect modifiers were similar across comparisons, we did not perform the network meta-analysis but instead used standard Cochrane methods.When trials begin to provide an adequate description of potential effect modifiers, we will attempt to conduct network meta-analysis. We searched CENTRAL, MEDLINE, Embase, Science Citation Index - Expanded, the WHO International Clinical Trials Registry Platform, and randomised controlled trials registers until February 2017 to identify randomised clinical trials (RCT) on pharmacological interventions for primary sclerosing cholangitis. We included only RCTs, irrespective of language, blinding, or publication status, in which participants were given a diagnosis of primary sclerosing cholangitis. We excluded trials that included previously liver-transplanted participants. We considered any of various pharmacological interventions compared with one other or with placebo. We excluded trials that compared different doses of various pharmacological interventions or that reported different treatment durations, except for ursodeoxycholic acid (UDCA). As UDCA is the drug most commonly investigated for primary sclerosing cholangitis, we performed a second analysis in which we stratified the dose of UDCA. We calculated the odds ratio and the rate ratio with 95% confidence intervals (CIs) using both

  4. Metabolomic profiling of 17 bile acids in serum from patients with primary biliary cirrhosis and primary sclerosing cholangitis: a pilot study.

    Science.gov (United States)

    Trottier, Jocelyn; Białek, Andrzej; Caron, Patrick; Straka, Robert J; Heathcote, Jenny; Milkiewicz, Piotr; Barbier, Olivier

    2012-04-01

    Primary biliary cirrhosis and primary sclerosing cholangitis are two cholestatic diseases characterised by hepatic accumulation of bile acids. This study compares serum bile acid levels in patients with primary biliary cirrhosis and primary sclerosing cholangitis and from age and sex-matched non cholestatic donors. Seventeen bile acids were quantified using liquid chromatography coupled to tandem mass spectrometry. Serum samples from cholestatic patients were compared with those of non-cholestatic donors. The concentration of total bile acids, taurine and glycine conjugates of primary bile acids was elevated in both patients with primary biliary cirrhosis and primary sclerosing cholangitis when compared to non-cholestatic donors. Samples from primary sclerosing cholangitis patients displayed reduced levels of secondary acids, when compared to non cholestatic and primary biliary cirrhosis sera. The ratio of total glycine versus total taurine conjugates was reduced in patients with primary biliary cirrhosis, but not in primary sclerosing cholangitis. The present study suggests that circulating bile acids are altered differentially in primary biliary cirrhosis and primary sclerosing cholangitis patients. Copyright © 2011 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.

  5. Stellate nonhereditary idiopathic foveomacular retinoschisis.

    Science.gov (United States)

    Ober, Michael D; Freund, K Bailey; Shah, Manthan; Ahmed, Shareef; Mahmoud, Tamer H; Aaberg, Thomas M; Zacks, David N; Gao, Hua; Mukkamala, Krishna; Desai, Uday; Packo, Kirk H; Yannuzzi, Lawrence A

    2014-07-01

    To describe a new classification of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR). Retrospective case series and literature review. A total of 17 patients from 5 institutions. Detailed case history, multimodal imaging, and genetic testing were reviewed for patients with macular schisis without a known predisposing condition. Patients with a stellate appearance centered on the fovea with correlating confirmed expansion of the outer plexiform layer (OPL) by optical coherence tomography (OCT) were included. Exclusion criteria included a family history of macular retinoschisis, a known genetic abnormality associated with retinoschisis, myopic traction maculopathy, epiretinal membrane, vitreoretinal traction, optic or scleral pit, or advanced glaucomatous optic nerve changes. Clinical features, anatomic characteristics, and visual acuity. A total of 22 eyes from 16 female patients and 1 male patient with foveomacular schisis were reviewed from 5 institutions. Initial visual acuity was ≥ 20/50 in all eyes (mean, 20/27), but visual acuity in a single eye decreased from 20/20 to 20/200 after the development of subfoveal fluid. The refractive status was myopic in 16 eyes, plano in 3 eyes, and hyperopic in 2 eyes. Three eyes had a preexisting vitreous separation, and 19 eyes had an attached posterior hyaloid. Follow-up ranged from 6 months to >5 years. In this largest known series of patients with SNIFR, all patients demonstrated splitting of the OPL in the macula with relatively preserved visual acuity (≥ 20/40) except in a single patient in whom subretinal fluid developed under the fovea. Copyright © 2014. Published by Elsevier Inc.

  6. [Bracing in Adolescent Idiopathic Scoliosis].

    Science.gov (United States)

    Lo, Yi-Fang; Huang, Yu-Chu

    2017-04-01

    Scoliosis is a common medical problem, with an incidence of between 0.47% and 5.2% in the general population globally. Adolescent idiopathic scoliosis (AIS) accounts for nearly 80% of all scoliosis. Young people with AIS often experience negative social consequences in association with their condition. Without proper and timely treatment, the potential resulting disabilities range from trunk deformity, pain, and neurological complications to compromised cardiopulmonary function, all of which may cause lifelong suffering. Scoliosis may be treated either conservatively or surgically, based on the severity of the disease. Bracing is the most widely adopted method of conservative treatment. However, the main goal of bracing is to inhibit the progression of the spinal curvature rather than to cure scoliosis. The clinical effectiveness of bracing in Taiwan has often been underutilized as a result of financial or other factors such as the availability of the treatment. The purpose of the present review is to clarify the effectiveness of bracing for AIS by elucidating the pathophysiology of scoliosis and examining the recent clinical evidence. The importance of preventative care and the unique contribution of nursing care to treatment has to date been under-recognized. The positive support that nurses provide to the families of the patients during the early phases of treatment as well as to the patients themselves, including helping them exercise appropriately and wear the brace correctly, is an essential component of effective treatment. Learning how to work with and to adapt to the brace being part of the body is an important part of the treatment as well as a way to avoid pain. Nurses are in an ideal position to facilitate this learning process and, overall, to provide health education.

  7. Idiopathic Perforation of the Sigmoid Colon in a 2.5 Years Old Girl: A Case Report

    Directory of Open Access Journals (Sweden)

    Sanaz Mehrabani

    2017-09-01

    Full Text Available Idiopathic colon perforation is rare in children. It is more common at the extremes of age. Splenic flextures, ileocecal and lower sigmoid regions are the most common sites of perforation. Delay in proper management of this condition is associated with high mortality and morbidity rate. We report on the case of a 2.5 years old girl who presented with fever, diarrhea, nausea and vomiting and progressive abdominal distention.Finally, she underwent an exploratory laparotomy for suspected peritonitis because of the patient's worsening condition. An area of perforation was found in the sigmoid colon that segmental resection and an end double barrel colostomy was done.

  8. Subacute sclerosing panencephalitis with bilateral inferior collicular hyperintensity on magnetic resonance imaging brain

    Directory of Open Access Journals (Sweden)

    Maya Thomas

    2012-01-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is chronic encephalitis occurring after infection with measles virus. An 8-year-old boy presented with progressive behavioral changes, cognitive decline and myoclonic jerks, progressing to a bed bound state over 2 months. Magnetic resonance imaging (MRI brain showed T2-weighted hyperintensities in the subcortical areas of the left occipital lobe and brachium of the inferior colliculus on both sides. EEG showed bilateral, synchronous periodic discharges. Serum/cerebrospinal fluid measles IgG titer was significantly positive. The overall features were suggestive of SSPE. MRI finding of bilateral inferior colliculus changes on MRI without significant involvement of other commonly involved areas suggests an uncommon/rare imaging pattern of SSPE.

  9. [Primary sclerosing cholangitis: present views of its pathogenesis, diagnosis and treatment].

    Science.gov (United States)

    Aleksandrova, E A; Avdeev, V G; Burnevich, E Z; Arion, E A

    2012-01-01

    Primary sclerosing cholangitis (PSC) is a chronic slowly progressive cholestatic liver disease characterized by non-purulent destructive inflammation and sclerosis of the extra- and intrahepatic bile ducts, leading to secondary biliary cirrhosis. The etiology of the disease is unknown; however, bacterial and genetic factors are presumed to be implicated. In 66-84% of cases, PSC is accompanied by inflammatory bowel diseases, such as ulcerative colitis and Crohn's disease. The diagnosis of PSC is based on the clinical presentation of the disease and on the data of magnetic resonance imaging or endoscopic retrograde cholangiography. The treatment of PSC has not been developed; there is evidence that ursodeoxycholic acid reduces cholestasis. Orthotopic liver transplantation is indicated at the stage of decompensated liver cirrhosis.

  10. Panencefalite esclerosante sub-aguda Subacute sclerosing panencephalitis: a case report

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    Charles Peter Tilbery

    1970-09-01

    Full Text Available É relatado um caso de penencefalite esclerosante sub-aguda, sendo salientadas as 4 fases da moléstia. O diagnóstico foi feito pela evolução, baseado no exame de líquido cefalorraqueano (eletroforese das proteínas e no eletrencefalograma e confirmado pela necrópsia, sendo verificada a presença de inclusões intranucleares.A case of subacute sclerosing panencephalitis is reported. The diagnosis was achieved by the disease's course, based on the laboratory data, so as cerebrospinal fluid examination (protein eletrophoretic study, eletroencephalogram and confirmed by histopathological study demonstrating the presence of intranuclear inclusions.

  11. Pathogenesis of Primary Sclerosing Cholangitis and Advances in Diagnosis and Management

    Science.gov (United States)

    Eaton, John E.; Talwalkar, Jayant A.; Lazaridis, Konstantinos N.; Gores, Gregory J.; Lindor, Keith D.

    2013-01-01

    Primary sclerosing cholangitis (PSC), first described in the mid-1850’s, is a complex liver that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis, associated with chronic inflammation of the biliary epithelium, resulting in multi-focal bile duct strictures that can affect the entire biliary tree. Chronic inflammation leads to fibrosis involving the hepatic parenchyma and biliary tree, which can lead to cirrhosis and malignancy. The etiology of PSC is not fully understood which in part explains a lack of effective medical therapy for this condition. However, we have begun to better understand the molecular pathogenesis of PSC. The recognition of specific clinical subtypes and their pattern of progression could improve phenotypic and genotypic classification of the disease. We review our current understanding of this enigmatic disorder and discuss important topics for future studies. PMID:23827861

  12. Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: a Review Featuring a Women's Health Perspective

    Science.gov (United States)

    Marchioni Beery, Renée M.; Vaziri, Haleh; Forouhar, Faripour

    2014-01-01

    Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are two major types of chronic cholestatic liver disease. Each disorder has distinguishing features and variable progression, but both may ultimately result in cirrhosis and hepatic failure. The following offers a review of PBC and PSC, beginning with a general overview of disease etiology, pathogenesis, diagnosis, clinical features, natural course, and treatment. In addition to commonly associated manifestations of fatigue, pruritus, and fat-soluble vitamin deficiency, select disease-related topics pertaining to women's health are discussed including metabolic bone disease, hyperlipidemia and cardiovascular risk, and pregnancy-related issues influencing maternal disease course and birth outcomes. This comprehensive review of PBC and PSC highlights some unique clinical considerations in the care of female patients with cholestatic liver disease. PMID:26357630

  13. Recurrence of primary sclerosing cholangitis, primary biliary cholangitis and auto-immune hepatitis after liver transplantation.

    Science.gov (United States)

    Visseren, T; Darwish Murad, S

    2017-04-01

    Liver transplantation is a well-accepted treatment for decompensated chronic liver disease due to primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC) and auto-immune hepatitis (AIH). Survival after liver transplantation is generally good with 1 and 5-year survival rates around 90% and 70-85%. After transplantation, however, these diseases recur in 8.6-27% (rPSC), 10.9-42.3% (rPBC) and 7-42% (rAIH), and this poses significant challenges in terms of management and graft outcome in these patients. In this review we discuss the incidence, clinical presentation, challenges in diagnosis, reported risk factors and impact on post-transplant outcomes of recurrence of PSC, PBC and AIH after liver transplantation. We also discuss some of the limitations of current investigations and formulate idea's for future research objectives. Copyright © 2017 Elsevier Ltd. All rights reserved.

  14. Primary sclerosing cholangitis and the microbiota: current knowledge and perspectives on etiopathogenesis and emerging therapies

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    Tabibian, James H.; O’hara, Steven P.; Lindor, Keith D.

    2014-01-01

    Primary sclerosing cholangitis (PSC) is a chronic, fibroinflammatory, cholestatic liver disease of unknown etiopathogenesis. PSC generally progresses to liver cirrhosis, is a major risk factor for hepatobiliary and colonic neoplasia, and confers a median survival to death or liver transplantation of only 12 years. Although it is well recognized that approximately 75% of patients with PSC also have inflammatory bowel disease (IBD), the significance of this association remains elusive. Accumulating evidence now suggests a potentially important role for the intestinal microbiota, and enterohepatic circulation of molecules derived there from, as a putative mechanistic link between PSC and IBD and a central pathobiological driver of PSC. In this concise review, we provide a summary of and perspectives regarding the relevant basic, translational, and clinical data, which, taken together, encourage further investigation of the role of the microbiota and microbial metabolites in the etiopathogenesis of PSC and as a potential target for novel pharmacotherapies. PMID:24990660

  15. Apical Sodium-Dependent Transporter Inhibitors in Primary Biliary Cholangitis and Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Hegade, Vinod S; Jones, David E J; Hirschfield, Gideon M

    2017-01-01

    Bile acids (BAs) have gained mainstream attention since the discovery of their key role as signalling molecules in health and disease. The apical sodium-dependent transporter (ASBT) protein located in the terminal ileum plays an important physiological role in the enterohepatic circulation of BAs and therefore essential for the BA homeostasis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), the 2 most common cholestatic liver diseases are characterised by altered BA flow and BA composition, which contribute to disease progression and symptom (pruritus) development. Therefore, changing the circulating BA pool in patients with PBC and PSC may have therapeutic implications. To this end, pharmacological inhibition of ASBT is fast emerging as an interesting target. In this review, we discuss the recent evidence for potential therapeutic use of ASBT inhibitors to treat PBC and PSC patients. © 2017 S. Karger AG, Basel.

  16. Severe infection with multidrug-resistant Salmonella choleraesuis in a young patient with primary sclerosing cholangitis.

    Science.gov (United States)

    Ferstl, Philip G; Reinheimer, Claudia; Jozsa, Katalin; Zeuzem, Stefan; Kempf, Volkhard Aj; Waidmann, Oliver; Grammatikos, Georgios

    2017-03-21

    Massive global spread of multidrug-resistant (MDR) Salmonella spp. expressing extended-spectrum beta-lactamase (ESBL) and additional resistance to fluoroquinolones has often been attributed to high international mobility as well as excessive use of oral antibiotics in livestock farming. However, MDR Salmonella spp. have not been mentioned as a widespread pathogen in clinical settings so far. We demonstrate the case of a 25-year-old male with primary sclerosing cholangitis who tested positive for MDR Salmonella enterica serotype Choleraesuis expressing ESBL and fluoroquinolone resistance. The pathogen was supposedly acquired during a trip to Thailand, causing severe fever, cholangitis and pancreatitis. To our knowledge, this is the first report of Salmonella enterica serotype Choleraesuis in Europe expressing such a multidrug resistance pattern. ESBL resistance of Salmonella enterica spp. should be considered in patients with obstructive biliary tract pathology and travel history in endemic countries.

  17. A Review of the Challenges Associated with the Diagnosis and Therapy of Primary Sclerosing Cholangitis

    Science.gov (United States)

    Saadi, Mohammed; Yu, Christine

    2014-01-01

    Primary sclerosing cholangitis (PSC) is a chronic and progressive cholestatic liver disease that often leads to the development of cirrhosis. Complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, dominant biliary strictures, gallstones, and hepatobiliary malignancies, most commonly cholangiocarcinoma (CCA). Despite the presumed autoimmune etiology of PSC, a clear benefit from immunosuppressive agents has not yet been established, and their use is limited by their side effects. Endoscopy is required in evaluation of biliary strictures in PSC to rule out the possibility of CCA. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease. However, disease recurrence can be a source of morbidity and mortality as transplanted patients survive longer. Further studies are needed to develop an optimal therapeutic strategy for patients with PSC to decrease the incidence of complications of the disease, to decrease the need for transplantation, and to extend life expectancy. PMID:26357617

  18. Cholestatic Liver Injury: Care of Patients With Primary Biliary Cholangitis or Primary Sclerosing Cholangitis.

    Science.gov (United States)

    Larson, Laurie; James, Michelle; Gossard, Andrea

    2016-10-01

    The most common causes of chronic cholestatic liver disease are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both disease processes are characterized by a destruction of intrahepatic and/or extrahepatic biliary ducts. The etiology is not entirely clear; however, there is an underlying autoimmune component contributing to both disease processes. Although PBC and PSC are often diagnosed and managed in the outpatient setting, in some instances, a patient may have jaundice, fatigue, and pruritus requiring evaluation and determination of the cholestatic cause. Patients with PSC should be monitored for evidence of cholangiocarcinoma, colon cancer, and gallbladder polyps as they are at an increased risk of malignant neoplasms. Liver transplant has the potential for improving quality of life, although disease recurrence is a risk. ©2016 American Association of Critical-Care Nurses.

  19. Clinical case report: Sclerosing hemangioma of the liver, a rare but great mimicker

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    Siavash Behbahani, MD

    2016-06-01

    Full Text Available Atypical hemangioma (including sclerosing and/or hyalinizing hemangioma of the liver is a rare variant of hepatic hemangioma, which is the most common benign hepatic tumor. Atypical hemangioma can be indistinguishable from malignancy, primary, or metastatic, based on imaging characteristics. We describe a case of a 70-year-old man with weight loss, occasional bloody stool, change in caliber of stool, and laboratory abnormalities who was found to have multiple hepatic lesions concerning for metastases. We demonstrate that knowledge of the appearance of atypical hemangioma and its inclusion in the differential diagnosis of hepatic lesions can alter patient management and be important to consider before invasive therapies are planned.

  20. Subacute Sclerosing Panencephalitis in an Infant: Diagnostic Role of Viral Genome Analysis

    Science.gov (United States)

    Baram, Tallie Z.; Gonzalez-Gomez, Ignacio; Xie, Zong-De; Yao, Dapeng; Gilles, Floyd H.; Nelson, Marvin D.; Nguyen, Hahn T.; Peters, Julius

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is related to “defective” measles virus or vaccination, though an association with parainfluenza viruses has been reported. SSPE is characterized by a slow, erratic course and elevated cerebrospinal fluid measles titers. An immunocompetent, vaccinated infant, with onset of symptoms in parainfiuenza virus season and a catastrophic course is described. Cerebrospinal fluid titers were negative, but postmortem brain had typical SSPE lesions. Patient brain-derived RNA, subjected to reverse transcription followed by polymerase chain reaction yielded polymerase chain reaction products with measles virus but not parainfluenza virus genes. The sequenced fragment revealed multiple mutations, typical for SSPE. SSPE can thus present in infants, with short latency and no cerebrospinal fluid antibodies. Viral genomic analysis may be diagnostic, permitting early therapy. PMID:8024248

  1. Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis and pseudotumour cerebri.

    Science.gov (United States)

    Panda, Akhila Kumar; Mehta, Vachan Jayant; Maheshwari, Siddharth; Kar, Sujit Kumar

    2013-08-20

    Subacute sclerosing panencephalitis (SSPE) is a chronic progressive encephalitis of childhood and young adults due to persistent measles virus infection. The usual age of onset is between 5 and 15 years. There are wide varieties of presentations of SSPE described in the literatures. Variable clinical presentations may lead to diagnostic dilemma and unnecessary investigations especially in developing countries, where the measles is quite endemic and vaccination status is not up to the mark because of poor literacy and socioeconomic status. Good clinical correlations, neuroimaging findings, EEG and cerebrospinal fluid (CSF) marker for SSPE yield the clue to diagnosis. This case illustrates a 13-year-old boy presented with short history of intellectual decline, headache, papilloedema, cranial nerve palsy, myoclonus with suggestive neuroimaging mimicking acute disseminated encephalomyelitis (ADEM) and pseudotumour cerebri. Subsequently he was diagnosed to be a case of SSPE on the basis of CSF and serum measles antibody titer.

  2. Subacute sclerosing panencephalitis with parkinsonian features in a child: A case report.

    Science.gov (United States)

    Bozlu, Gulcin; Cobanogullari Direk, Meltem; Okuyaz, Cetin

    2015-10-01

    Subacute sclerosing panencephalitis (SSPE) can present with atypical clinical signs which may result in delayed diagnosis and treatment. We present a child with SSPE whose initial manifestation was parkinsonism. This 12-year-old boy presented with the complaint of difficulty in standing up and walking for 2 months. Neurological examination revealed generalized rigidity, bradykinesia, impaired postural reflexes, and a mask-like facies. The initial diagnosis of Juvenile Parkinson Disease was made. He had no improvement with levodopa, trihexyphenidyl, tetrabenazine and clonazepam. The EEG showed irregular background activity with generalized slow waves which were not suppressed with diazepam injection. SSPE was considered and the diagnosis was confirmed with the identification of measles antibodies in cerebrospinal fluid. SSPE should be considered in children and adolescents with parkinsonian symptoms, particularly in the absence of a history of vaccination against measles. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  3. Marked Atrophic Changes of the Brain in a Patient with Subacute Sclerosing Panencephalitis.

    Science.gov (United States)

    Yasin, Faizan; Assad, Salman; Nadeem, Muhammad; Zahid, Mehr

    2017-08-21

    Subacute Sclerosing Panencephalitis (SSPE) is a debilitating disorder associated with the measles infection in childhood. It is a very rare manifestation in children. It usually presents with measles before the age of two. We report a similar case of SSPE in a 14-year-old girl who developed this life-threatening condition in spite of receiving the measles vaccination. Despite the vaccination, the patient had suffered from measles before the age of two. This highlights the dilemma of ineffective vaccinations in developing countries. We also describe the radiologic features of SSPE in this patient, with marked atrophy seen in the occipital region following hyperintensities noticed at a relatively earlier stage.

  4. Subacute sclerosing panencephalitis with bilateral inferior collicular hyperintensity on magnetic resonance imaging brain.

    Science.gov (United States)

    Thomas, Maya; Sivadasan, Ajith; Alexander, Mathew; Patil, Anil Kumar B

    2012-10-01

    Subacute sclerosing panencephalitis (SSPE) is chronic encephalitis occurring after infection with measles virus. An 8-year-old boy presented with progressive behavioral changes, cognitive decline and myoclonic jerks, progressing to a bed bound state over 2 months. Magnetic resonance imaging (MRI) brain showed T2-weighted hyperintensities in the subcortical areas of the left occipital lobe and brachium of the inferior colliculus on both sides. EEG showed bilateral, synchronous periodic discharges. Serum/cerebrospinal fluid measles IgG titer was significantly positive. The overall features were suggestive of SSPE. MRI finding of bilateral inferior colliculus changes on MRI without significant involvement of other commonly involved areas suggests an uncommon/rare imaging pattern of SSPE.

  5. Subacute sclerosing panencephalitis in a child with human immunodeficiency virus (HIV) infection on antiretroviral therapy

    Science.gov (United States)

    Muthusamy, Karthik; Yoganathan, Sangeetha; Thomas, Maya Mary; Alexander, Mathew; Verghese, Valsan Philip

    2015-01-01

    Subacute Sclerosing Panencephalitis (SSPE) in HIV-infected children is a scarcely reported entity with previous reports describing fulminant course. The impact of highly active antiretroviral therapy (HAART) in altering its course remains unknown. We describe a child with HIV infection, who developed measles at 5 months of age and later developed SSPE at 14 years of age, remaining stable at 7 month follow-up, while on HAART for WHO (World Health Organisation) stage IV disease. The dynamics of HIV-related immunosuppression has an impact on the clinical course of SSPE. Contrary to reported cases of fulminant progression, a classic presentation with slow progression can be expected in children on HAART. We reemphasize the recommendation of “early measles vaccination” to prevent measles infection and subsequent SSPE in these children with an increasingly good life expectancy in the era of HAART. PMID:25745323

  6. Neuro-ophthalmology of subacute sclerosing panencephalitis: two cases and a review of the literature.

    Science.gov (United States)

    Colpak, Ayse I; Erdener, Sefik E; Ozgen, Burce; Anlar, Banu; Kansu, Tulay

    2012-11-01

    To review the literature on early visual manifestations of subacute sclerosing panencephalitis (SSPE) with regard to two patients who had visual problems preceding the onset of neurological symptoms. One patient had cortical visual disturbances and the other had visual loss due to retinal pigment epithelial changes. SSPE is a chronic encephalitis characterized by a history of measles infection and a progressive disease of the central nervous system that still occurs frequently in countries with insufficient measles immunization. Visual manifestations can occur as a result of involvement of the pathways that lead from the retina to the occipital cortex during the course of the disease, but are rare as a presenting sign. Fundus changes, especially macular retinitis and macular pigment disturbances, appear to be the most common ocular manifestations of SSPE. Ophthalmologists must be aware that SSPE can knock their door with ocular findings of SSPE, months or years before the onset of neurological symptoms.

  7. An initial MRI picture of limbic encephalitis in subacute sclerosing panencephalitis.

    Science.gov (United States)

    Lebon, Sébastien; Maeder, Philippe; Maeder-Ingvar, Malin; Poloni, Claudia; Mayor-Dubois, Claire; Roulet-Perez, Eliane; Jeannet, Pierre-Yves

    2011-11-01

    Subacute sclerosing panencephalitis (SSPE) is a rare and severe long-term complication of measles. Hallmarks of this entity include progressive cognitive decline, myoclonia, a generalized periodic pattern on EEG and deep white matter abnormalities on MRI. However, imaging can be normal in early stages. We report herein the case of a previously healthy 13-years-old girl with an unusual radiological presentation. She presented with unilateral myoclonia, cognitive decline with memory impairment and a first brain MRI with swelling of both hippocampi mimicking limbic encephalitis. Measles antibodies were positive in CSF and the EEG showed slow periodic complexes. This unusual radiological presentation has never been described in SSPE. Relationship between virus and limbic system are discussed. Copyright © 2011 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

  8. Regional cerebral metabolic rate for glucose and cerebrospinal fluid monoamine metabolites in subacute sclerosing panencephalitis

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    Yanai, Kazuhiko; Miyabayashi, Shigeaki; Iinuma, Kazuie; Tada, Keiya; Fukuda, Hiroshi; Ito, Masatoshi; Matsuzawa, Taiju

    1987-06-01

    Regional cerebral metabolic rate for glucose (rCMRglu) and cerebrospinal fluid monoamine metabolites were measured in two cases of subacute sclerosing panencephalitis (SSPE) with different clinical courses. A marked decrease in rCMRglu was found in the cortical gray matter of a patient with rapidly developing SSPE (3.6 - 4.2 mg/100 g brain tissue/min). However, the rCMRglu was preserved in the caudate and lenticular nuclei of the patient (7.7 mg/100 g/min). The rCMRglu in a patient with slowly developing SSPE revealed patterns and values similar to those of the control. Cerebrospinal fluid monoamine metabolites ; homovanilic acid and 5-hydroxyindoleacetic acid, were decreased in both rapidly and slowly developing SSPE. These data indicated that rCMRglu correlated better with the neurological and psychological status and that dopaminergic and serotonergic abnormalities have been implicated in pathophysiology of SSPE.

  9. Epidemiology of subacute sclerosing panencephalitis (SSPE) in Germany from 2003 to 2009: a risk estimation.

    Science.gov (United States)

    Schönberger, Katharina; Ludwig, Maria-Sabine; Wildner, Manfred; Weissbrich, Benedikt

    2013-01-01

    Subacute sclerosing panencephalitis (SSPE) is a fatal long-term complication of measles infection. We performed an estimation of the total number of SSPE cases in Germany for the period 2003 to 2009 and calculated the risk of SSPE after an acute measles infection. SSPE cases were collected from the Surveillance Unit for Rare Paediatric Diseases in Germany and the Institute of Virology and Immunobiology at the University of Würzburg. The total number of SSPE cases was estimated by capture-recapture analysis. For the period 2003 to 2009, 31 children with SSPE who were treated at German hospitals were identified. The capture-recapture estimate was 39 cases (95% confidence interval: 29.2-48.0). The risk of developing SSPE for children contracting measles infection below 5 years of age was calculated as 1∶1700 to 1∶3300. This risk is in the same order of magnitude as the risk of a fatal acute measles infection.

  10. Subacute sclerosing panencephalitis resembling Rasmussen’s encephalitis on magnetic resonance imaging

    Science.gov (United States)

    Jakkani, Ravi Kanth; Sureka, Jyoti; Panwar, Sanuj

    2015-01-01

    Subacute sclerosing panencephalitis (SSPE) is a rare, slowly progressing but invariably fatal disease that is related to a prior measles virus infection and most commonly affects paediatric patients. Magnetic resonance (MR) imaging is the modality of choice for determining such changes in white matter. SSPE typically demonstrates bilateral but asymmetric periventricular and subcortical white matter involvement. We herein report a rare case of unilateral white matter involvement in a 13-year-old boy with SSPE that closely simulated Rasmussen’s encephalitis. To the best of our knowledge, this is the first report of an atypical presentation on MR imaging in which SSPE was a rare cause of unilateral brain parenchymal involvement in a patient with intractable seizures. PMID:26451061

  11. Chorioretinitis: a potential clue to the early diagnosis of subacute sclerosing panencephalitis.

    Science.gov (United States)

    Jeevagan, Vijayabala; Dissanayake, Athula

    2017-08-01

    We describe a 36-year-old man with subacute sclerosing panencephalitis (SSPE) presenting with chorioretinitis two years before onset of other neurological features. He had neither myoclonus nor the typical EEG features of SSPE. The diagnosis was confirmed in the appropriate clinical setting by detecting elevated measles antibody titres in cerebrospinal fluid and serum. Clinicians should consider SSPE among the differential diagnoses in chorioretinitis. This is particularly so if there is macular or perimacular involvement with concurrent involvement of the optic nerve in young patients, even without other characteristic neurological symptoms. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  12. A case of fulminant subacute sclerosing panencephalitis presenting with acute myoclonic-astatic epilepsy.

    Science.gov (United States)

    Magurano, Fabio; Marella, Gian Luca; Marchi, Antonella; Filia, Antonietta; Marsella, Luigi Tonino; Potenza, Saverio; Massa, Roberto; Bucci, Paola; Baggieri, Melissa; Nicoletti, Loredana

    2017-01-01

    The neurologic sequelae post-measles are less common than other complications measles-related and can lead to severe disability or death: primary measles encephalitis (PME), acute post-infectious measles encephalomyelitis (APME), measles inclusion body encephalitis (MIBE), and subacute sclerosing panencephalitis (SSPE). SSPE syndrome can affect people years from the acute measles virus infection, as result of the persistence of defective viral particles in brain cells. Clinical onset typically manifests with progressive intellectual deterioration, behavioral changes, and myoclonic jerks. The course of SSPE in the majority of affected children is that of a progressive worsening with fatal outcome within two years. This report described an Italian case of fulminant SSPE syndrome that led to death within few months from the initial onset.

  13. Evolution of certain typical and atypical features in a case of subacute sclerosing panencephalitis

    Science.gov (United States)

    Raut, Tushar Premraj; Singh, Maneesh Kumar; Garg, Ravindra Kumar; Rai, Dheeraj

    2012-01-01

    Subacute sclerosing panencephalitis (SSPE) is a slowly progressive inflammatory disease of the central nervous system caused by a persistent measles virus usually affecting the childhood and adolescent age group. Clinical features at onset are very subtle and non-specific. Certain atypical features can occur at onset or during the course of illness which can be misleading. Neuroimaging features often are non-specific. Features like myoclonic jerks, cognitive decline and typical EEG findings lead to a strong suspicion of SSPE. Here, we describe the stagewise progression of a case of SSPE in a 14-year-old girl who had myoclonic jerks and cognitive decline at onset. During the course of disease, the patient developed cortical vision loss, atypical extrapyramidal features like segmental and hemifacial dystonia ultimately leading to a bedbound vegetative state. EEG showed typical periodic discharges along with positive cerebrospinal fluid serology for measles. PMID:23266775

  14. Subacute sclerosing panencephalitis in a child with human immunodeficiency virus (HIV infection on antiretroviral therapy

    Directory of Open Access Journals (Sweden)

    Karthik Muthusamy

    2015-01-01

    Full Text Available Subacute Sclerosing Panencephalitis (SSPE in HIV-infected children is a scarcely reported entity with previous reports describing fulminant course. The impact of highly active antiretroviral therapy (HAART in altering its course remains unknown. We describe a child with HIV infection, who developed measles at 5 months of age and later developed SSPE at 14 years of age, remaining stable at 7 month follow-up, while on HAART for WHO (World Health Organisation stage IV disease. The dynamics of HIV-related immunosuppression has an impact on the clinical course of SSPE. Contrary to reported cases of fulminant progression, a classic presentation with slow progression can be expected in children on HAART. We reemphasize the recommendation of “early measles vaccination” to prevent measles infection and subsequent SSPE in these children with an increasingly good life expectancy in the era of HAART.

  15. [Sclerosing epithelioid fibrosarcoma of the paravertebral column. Case report and literature review].

    Science.gov (United States)

    Puerta Roldán, Patricia; Rodríguez Rodríguez, Rodrigo; Bagué Rossell, Silvia; de Juan Delago, Manel; Molet Teixidó, Joan

    2013-01-01

    Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of low-grade fibrosarcoma, with specific histological and immunohistochemical features and a poor prognosis. We report a case of SEF of the paravertebral column in a 49-year old male who presented a paraspinal mass with extension into the L4-L5 neural foramen and invasion of the L5 nerve root. Histology of the tumourectomy specimen and its immunohistochemical study led to the diagnosis of SEF. This case was particularly unusual due to its paravertebral column location and, despite its low grade, illustrates the malignant potential of SEF. Copyright © 2012 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  16. Sclerosing peritonitis: A case report with a review of the literature

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    Anita Sharan

    2016-01-01

    Full Text Available Sclerosing peritonitis (SP, also known as abdominal cocoon, is a rare form of peritoneal inflammation involving both the visceral and parietal surfaces of the abdominal cavity. It has an unknown etiology, resulting in an intestinal obstruction due to complete or incomplete encapsulation of the small bowel by a fibrocollagenous membrane. A high index of clinical suspicion is needed for its preoperative diagnosis. It is not easy to make a preoperative diagnosis since the early clinical features are not specific. Repeated episodes of small intestinal obstruction, relevant imaging findings, and absence of other plausible etiologies may aid in clinical suspicion. We are presenting the case of a 14-year-old girl presenting with pain in the abdomen since 5-6 days, vomiting since 2 days, and passing worms per vomitus since 2 days. Diagnosis of SP was done. This case is being highlighted for the rarity of its occurrence and distinct morphological spectrum.

  17. Combined Interferon-α and Inosiplex Treatment of Subacute Sclerosing Panencephalitis: A Case Report

    Directory of Open Access Journals (Sweden)

    Chy-Yuan Han

    2003-08-01

    Full Text Available Subacute sclerosing panencephalitis (SSPE is rare in Taiwan. On admission to hospital, a 15-year-old boy was diagnosed with SSPE based on the clinical picture, electroencephalogram, cerebrospinal fluid studies, and brain biopsy. The initial clinical picture was a decline in school performance and a change in personality, followed by progressive tic-like involuntary movements and mental impairment for 8 months, then a rapidly progressive course. After the patient was treated with oral inosiplex and intraventricular interferon-! (IFN-!, his condition stabilized and the neurologic disability index score improved slightly. There were no major side effects during treatment except for a transient initial elevation of body temperature that lasted for several days. Oral inosiplex and intraventricular IFN-! appear to be safe and effective. Early identification and aggressive treatment of SSPE is important.

  18. Diversity of matrix protein in subacute sclerosing panencephalitis and measles virus-infected cells.

    Science.gov (United States)

    Haga, T; Yoshikawa, Y; Yamanouchi, K

    1990-01-01

    Expression of the viral matrix (M) proteins in Vero cells infected with 18 strains of subacute sclerosing panencephalitis (SSPE) virus and measles virus was examined by immunocytochemistry and Western blot analysis using an anti-M monospecific serum and two sera against the M protein specific synthetic peptides. By immunocytochemistry using the anti-M monospecific serum, M protein was detected in all of the virus-infected cells regardless of cell-free virus production. M proteins of the seven non-productive strains were found to vary significantly in their epitope, in their reactivity to different assay systems, and in their molecular weight, whereas M proteins of the other 11 productive strains were detected consistently. These results suggest diversification of M protein of the non-productive strains.

  19. Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: a Review Featuring a Women's Health Perspective.

    Science.gov (United States)

    Marchioni Beery, Renée M; Vaziri, Haleh; Forouhar, Faripour

    2014-12-01

    Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are two major types of chronic cholestatic liver disease. Each disorder has distinguishing features and variable progression, but both may ultimately result in cirrhosis and hepatic failure. The following offers a review of PBC and PSC, beginning with a general overview of disease etiology, pathogenesis, diagnosis, clinical features, natural course, and treatment. In addition to commonly associated manifestations of fatigue, pruritus, and fat-soluble vitamin deficiency, select disease-related topics pertaining to women's health are discussed including metabolic bone disease, hyperlipidemia and cardiovascular risk, and pregnancy-related issues influencing maternal disease course and birth outcomes. This comprehensive review of PBC and PSC highlights some unique clinical considerations in the care of female patients with cholestatic liver disease.

  20. Sclerosing angiomatoid nodular transformation of the spleen masquerading as a sarcoma metastasis

    Directory of Open Access Journals (Sweden)

    Lalitha Sitaraman

    2010-12-01

    Full Text Available We report a case of sclerosing angiomatoid nodular transformation (SANT of the spleen presenting as an incidental splenic mass in a patient with a history of retroperitoneal spindle cell sarcoma. Imaging studies and preoperative fine needle biopsy failed to differentiate this lesion from other vascular splenic lesions or a metastatic focus of a prior sarcoma. The patient was treated with splenectomy, which has proved both diagnostic and therapeutic in this and other cases of SANT. Although histology can lead to the diagnosis of vascular tumor, immuno­histochemistry is the only way to confirm the diagnosis of SANT. The etiology of SANT is unknown. SANT of the spleen is a benign lesion that does not recur after splenectomy.

  1. Chemotherapy-induced sclerosing cholangitis: long-term response to endoscopic therapy.

    Science.gov (United States)

    Alazmi, Waleed M; McHenry, Lee; Watkins, James L; Fogel, Evan L; Schmidt, Suzette; Sherman, Stuart; Lehman, Glen L

    2006-04-01

    Hepatic arterial infusion of fluoropyrimidines has been widely used for the treatment of hepatic metastasis from colorectal cancer. One major complication of such treatment is biliary sclerosis resembling primary sclerosing cholangitis, which has an incidence ranging from 8% to 26%. We evaluated the efficacy and long-term outcome of endoscopic therapy in the management of chemotherapy-induced sclerosing cholangitis (CISC). With the use of an endoscopic retrograde cholangiopancreatography (ERCP) database, all patients with a diagnosis of CISC who had endoscopic therapy between March 1995 and March 2005 were identified. The indications, findings, therapies, and complications for all patients undergoing ERCP were recorded in this database. Additional information was obtained by review of medical records. Eleven patients (six men and five women) were identified. The mean age at presentation was 59.5 years (range, 36-76 years). Cholangiogram findings revealed stricture confined to the common hepatic duct in two patients, involving the hilum in seven patients, involving the right and/or left main hepatic ducts in nine patients, and extending to the intrahepatic radicals in two patients. All patients had successful endoscopic therapy to alleviate the presenting symptom. The grade and extent of biliary strictures did not change in five patients, improved in one patient, recurred in two patients, and progressed in two patients over the follow-up period of 28.2 months (range, 4-60 months). Although long-term follow-up of patients with CISC is limited by the dismal prognosis of the underlying malignancy, CISC has a recalcitrant pattern that rarely improves with endoscopic therapy. However, endoscopic therapy seems to be an effective method of palliation.

  2. A sclerosing stromal tumor of the ovary with masculinization in a premenarchal girl

    Directory of Open Access Journals (Sweden)

    Soo Min Park

    2011-05-01

    Full Text Available A sclerosing stromal tumor of the ovary is an extremely rare benign tumor; it usually is found during the second and third decades of life. Patients present with pelvic pain or a palpable abdominal mass. Hormonal effects such as masculinization are uncommon. Here, an 11-year old premenarchal girl presented with deepening of the voice. In addition, clitoromegaly and hirsutism with a male suprapubic hair pattern were observed. The laboratory findings showed that the testosterone level was elevated to 3.67 ng/mL, andostenedione to above 10 ng/mL, dehydroepiandrosterone-sulfate to 346 μg/dL and 17-hydroxy progesterone (17-OHP to 11.28 ng/mL. The chromosome evaluation revealed a 46,XX female karyotype. An adrenocorticotropic hormone stimulation test was performed. The 17-OHP to cortisol ratio in 30 minutes was 0.045, which suggested a heterozygote for the 21-hydroxylase deficiency. However, the CYP21A2 gene encoding steroid 21-hydroxylase showed normal. The pelvic ultrasound showed a heterogeneous mass consisting of predominantly solid tissue in the pelvic cavity. The pelvic magnetic resonance imaging revealed an 8.9× 6.2×6.6 cm mass of the left ovary. A left oophrectomy was performed and microscopic examination confirmed a sclerosing stromal tumor. Immunohistochemical studies showed that the tumor was positive for smooth muscle actin and vimentin, but negative for S-100 protein and cytokeratin. Following surgery, the hormone levels returned to the normal range and the hirsutism resolved.

  3. Vascular sclerosing effects of bleomycin on cutaneous veins: a pharmacopathologic study on experimental animals*

    Science.gov (United States)

    AlGhamdi, Khalid M; Kumar, Ashok; Ashour, Abdelkader E.; AL-Rikabi, Ammar C.; AlOmrani, Abdullah Hasan; Ahamed, Shaik Shaffi

    2017-01-01

    Background Varicose veins and the complications of venous disease are common disorders in humans. Objective To study the effects of bleomycin as a potential new sclerosing agent and its adverse events in treating varicose veins. Methods Bleomycin-loaded liposomes 0.1ml was injected in the dorsal ear veins of white New Zealand rabbits. Sodium tetradecyl sulfate was used as a positive control. Normal saline was used as negative control. The blood vessels of the treated ears were photographed before and at one hour and two, eight and 45 days after treatment. Biopsies from the treated areas were obtained for histological examination. Blood samples were collected to determine any possible toxicity. Results Bleomycin by itself was ineffective; therefore, liposomes were used as a vector to deliver bleomycin to the vein lumen. Subsequently, bleomycin started showing its sclerosing effects. Toxicity monitoring showed no apparent hematologic, pulmonary, hepatic or renal toxicities. This study revealed that bleomycin induced vasculitis, which led to vascular occlusion, which was observed on day 1 and day 8. No bleomycin-related injury was noted by histopathological examination of lung sections. The calculation of the lung/body weight coefficient indicated that edema was present in the experimental groups compared with the negative and positive controls. Study limitations Relatively small number of experimental animals used. Conclusions This study showed that bleomycin-loaded liposomes were able to induce vasculitis and vascular occlusion without any toxicity or complications. It might be useful, hence, to treat patients suffering from Varicose veins and other ectatic vascular diseases with this agent. PMID:28954096

  4. Knockout of the primary sclerosing cholangitis-risk gene Fut2 causes liver disease in mice.

    Science.gov (United States)

    Maroni, Luca; Hohenester, Simon D; van de Graaf, Stan F J; Tolenaars, Dagmar; van Lienden, Krijn; Verheij, Joanne; Marzioni, Marco; Karlsen, Tom H; Oude Elferink, Ronald P J; Beuers, Ulrich

    2017-08-01

    The etiopathogenesis of primary sclerosing cholangitis is unknown. Genetic variants of fucosyltransferase 2 (FUT2) have been identified in genome-wide association studies as risk factors for primary sclerosing cholangitis. We investigated the role of Fut2 in murine liver pathophysiology by studying Fut2-/- mice. Fut2-/- mice were viable and fertile, had lower body weight than wild-type (wt) littermates and gray fur. Half of the Fut2-/- mice showed serum bile salt levels 40 times higher than wt (Fut2-/-high ), whereas the remainder were normocholanemic (Fut2-/-low ). Fut2-/- mice showed normal serum liver tests, bile flow, biliary bile salt secretion, fecal bile salt loss, and expression of major hepatocellular bile salt transporters and cytochrome P450 7a1, the key regulator of bile salt synthesis, indicating that elevated serum bile salts in Fut2-/-high mice were not explained by cholestasis. Fut2-/-high mice, but not Fut2-/-low mice, were sensitive to hydrophobic bile salt feeding (0.3% glycochenodeoxycholate); they rapidly lost weight and showed elevation of serum liver tests (alkaline phosphatase, aspartate aminotransferase, alanine aminotransferase) and areas of liver parenchymal necrosis. Histomorphological evaluation revealed the presence of paraportal shunting vessels, increased numbers of portal vascular structures, wall thickening of some portal arteries, and periductal fibrosis in Fut2-/-high mice more than Fut2-/-low mice and not wt mice. Unconjugated bilirubin and ammonia were or tended to be elevated in Fut2-/-high mice only. Portosystemic shunting was demonstrated by portal angiography, which disclosed virtually complete portosystemic shunting in Fut2-/-high mice, discrete portosystemic shunting in Fut2-/-low mice, and no shunting in wt littermates. Liver pathology in Fut2-/- mice is dominated by consequences of portosystemic shunting resulting in microcirculatory disturbances, mild (secondary) periductal fibrosis, and sensitivity toward human bile

  5. Health-related quality of life among patients with primary sclerosing cholangitis.

    Science.gov (United States)

    Haapamäki, Johanna; Tenca, Andrea; Sintonen, Harri; Barner-Rasmussen, Nina; Färkkilä, Martti A

    2015-09-01

    To assess health-related quality of life (HRQoL) of patients with primary sclerosing cholangitis (PSC), and to compare it with that of the general population. Also, to examine changes in HRQoL in newly diagnosed PSC patients at a follow-up 1-2 years later, and to compare their HRQoL with HRQoL of newly diagnosed inflammatory bowel disease (IBD) patients. Furthermore, sources of and need for disease-related information among PSC patients were surveyed. Primary sclerosing cholangitis patients filled in the survey questionnaire when attending an endoscopic retrograde cholangiography examination. The 15D served as a general HRQoL instrument. The follow-up questionnaire was mailed to the newly diagnosed patients 1-2 years later. No significant difference was seen in 15D scores between PSC patients and general population, but the dimensions of excretion (P < 0.001), depression (P = 0.003), distress (P = 0.003) and vitality (P = 0.005) were significantly lower in PSC. Age and symptoms affected HRQoL but severity of biliary changes did not. Those with newly diagnosed IBD had lower 15D scores than those with PSC. No significant changes were observed in 15D scores of new PSC patients in the follow-up. Many patients were dissatisfied with information received. Newly diagnosed PSC patients have better HRQoL than do IBD patients, and no significant HRQoL changes were observed in the mean follow-up of 1.58 years after PSC diagnosis. ERC findings did not correlate with HRQoL or symptoms. HRQoL of PSC patients was mostly comparable with that of general population, but special attention should be paid to patients' psychological well-being. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. Sclerosing Encapsulated Peritonitis: A devastating and infrequent disease complicating kidney transplantation, case report and literature review.

    Science.gov (United States)

    Caicedo, Liliana; Delgado, Alejandro; Caicedo, Luis A; Bravo, Juan Carlos; Thomas, Laura S; Rengifo, Martin; Villegas, Jorge I; Serrano, Oscar; Echeverri, Gabriel J

    2017-01-01

    Sclerosing Encapsulating Peritonitis (SEP) is a rare condition with an incidence of up to 3% and a mortality of up to 51% among peritoneal dialysis (PD) patients (Brown et al., Korte et al. and Kawanishi et al.). In the last ten years, the incidence of SEP in kidney transplant recipients has increased (Nakamoto, de Sousa et al. and Korte et al.). A 31-year old male with a 15 years history of PD and later kidney retransplantation was admitted to the emergency service after experiencing several weeks of diffuse abdominal pain which had escalated to include vomiting and diarrhea during the 24h previous to admission. The patient underwent an exploratory laparotomy where severe peritoneal thickening was found, in addition to signs of chronic inflammation and blocked intestinal loops. Histopathologic findings were suggestive of sclerosing peritonitis. After two months of treatment in hospital, the patient presented an obstructed intestine, with a rigid and thickened peritoneum compromising all the intestinal loops. Despite being rare, SEP, represents a significant complication due to its high mortality and recurrence. It is insidious in its early stages and culminates in an intestinal obstruction (Fieren). Risk factors for its development in kidney transplant recipients include a history of prolonged treatment with PD and the use of calcineurin inhibitors as an immunosuppressive treatment (Korte et al.). Given the increase in the incidence of SEP in kidney transplant recipients, the clinician should be alert to the presence of this complication. A greater number of multi-centre studies are required to identify the risk factors for SEP that are inherent in renal transplant recipients. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  7. Risk factors and outcome in patients with primary sclerosing cholangitis with persistent biliary candidiasis.

    Science.gov (United States)

    Rupp, Christian; Bode, Konrad Alexander; Chahoud, Fadi; Wannhoff, Andreas; Friedrich, Kilian; Weiss, Karl-Heinz; Sauer, Peter; Stremmel, Wolfgang; Gotthardt, Daniel Nils

    2014-10-23

    Candidiasis is commonly observed in patients with primary sclerosing cholangitis (PSC), but the clinical risk factors associated with its presence have not been fully investigated. In this study, we aimed to analyse the incidence, risk factors, and transplantation-free survival in primary sclerosing cholangitis (PSC) patients with persistent biliary candidiasis. We retrospectively analysed patients diagnosed with PSC who were admitted to our department during 2002 to 2012. One-hundred fifty patients whose bile cultures were tested for fungal species were selected, and their clinical and laboratory parameters were investigated. The results of endoscopic retrograde cholangiography (ERC) and bile cultures were analysed using chart reviews. The cases of biliary candidiasis were sub-classified as transient or persistent. Thirty out of 150 (20.0%) patients had biliary candidiasis. Although all patients demonstrated comparable baseline characteristics, those with biliary candidiasis showed significantly reduced transplantation-free survival (p candidiasis. A subgroup analysis showed reduced survival with a greater necessity for orthotopic liver transplantation (OLT) only in patients with persistence of Candida (p = 0.007). The survival in the patients with transient biliary candidiasis was comparable to that in candidiasis-free patients. In a multivariate regression analysis that included Mayo risk score (MRS), sex, age, dominant stenosis, inflammatory bowel disease, autoimmune hepatitis overlap syndrome, and number of times ERC was performed, biliary candidiasis was an independent risk factor for reduced survival (p = 0.008). Risk factors associated with acquisition of biliary candidiasis were age at PSC diagnosis and number of ERCs. The persistence of biliary candidiasis is associated with markedly reduced transplantation-free survival in PSC patients. By contrast, actuarial survival in patients with transient biliary candidiasis approaches that for patients without any

  8. Idiopathic intracranial hypertension: case report

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    Iacob G.

    2015-12-01

    Full Text Available Idiopathic intracranial hypertension - IIH (synonymous old terms: benign intracranial hypertension - BIH, pseudotumor-cerebri - PTC it’s a syndrome, related to elevated intracranial pressure, of unknown cause, sometimes cerebral emergency, occuring in all age groups, especially in children and young obese womans, in the absence of an underlying expansive intracranial lesion, despite extensive investigations. Although initial symptoms can resolve, IIH displays a high risk of recurrence several months or years later, even if initial symptoms resolved. Results: A 20-year-old male, obese since two years (body mass index 30, 9, was admitted for three months intense headache, vomiting, diplopia, progressive visual acuity loss. Neurologic examination confirmed diplopia by left abducens nerve palsy, papilledema right > left. At admission, cerebral CT scan and cerebral MRI with angio MRI 3DTOF and 2D venous TOF was normal. Despite treatment with acetazolamide (Diamox, corticosteroid, antidepressants (Amitriptyline, anticonvulsivants (Topiramate three weeks later headache, diplopia persist and vision become worse, confirmed by visual field assessment, visual evoked potential (VEP. A cerebral arteriography demonstrate filling defect of the superior sagittal sinus in the 1/3 proximal part and very week filling of the transverse right sinus on venous time. Trombophylic profile has revealed a heterozygote V factor Leyden mutation, a homozygote MTHFR and PAI mutation justifying an anticoagulant treatment initiated to the patient. The MRI showed a superior sagittal sinus, right transverse and sigmoid sinus thrombosis, dilatation and buckling of the optic nerve sheaths with increased perineural fluid especially retrobulbar, discrete flattening of the posterior segment of the eyeballs, spinal MRI showed posterior epidural space with dilated venous branches, with mass effect on the spinal cord, that occurs pushed anterior on sagittal T1/T2 sequences cervical and

  9. Idiopathic erythrocytosis in IgA nephropathy

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    E Mahesh

    2017-01-01

    Full Text Available We report a case of idiopathic erythrocytosis in a 31-year-old male who was incidentally detected to have hypertension during his preemployment checkup. Urine routine showed proteinuria and hematuria. Biochemical parameters revealed raised serum creatinine, and histological findings of the renal biopsy showed IgAN.

  10. Idiopathic erythrocytosis in IgA nephropathy.

    Science.gov (United States)

    Mahesh, E; Madhyastha, P R; Kalashetty, M; Gurudev, K C; Bande, S; John, M M

    2017-01-01

    We report a case of idiopathic erythrocytosis in a 31-year-old male who was incidentally detected to have hypertension during his preemployment checkup. Urine routine showed proteinuria and hematuria. Biochemical parameters revealed raised serum creatinine, and histological findings of the renal biopsy showed IgAN.

  11. [Idiopathic pulmonary hemosiderosis with dendriform pulmonary ossification].

    Science.gov (United States)

    Barrera, Ana Madeleine; Vargas, Leslie

    2016-12-01

    Pulmonary ossification is a rare and usually asymptomatic finding reported as incidental in lung biopsies. Similarly, idiopathic pulmonary hemosiderosis is a rare cause of pulmonary infiltrates. We report the case of a 64-year old man with chronic respiratory symptoms in whom these two histopathological findings converged.

  12. Exercise therapy in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Takken, T.; van Brussel, M.; Engelbert, R. H. H.; van der Net, J.; Kuis, W.; Helders, P. J. M.

    2008-01-01

    Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). To assess the effects of exercise therapy on functional ability,

  13. Infections and Thrombocytopenia in Chronic Idiopathic ...

    African Journals Online (AJOL)

    This is a case report to study the association between episodes of thrombocytopenia and bacterial infections in a patient with chronic idiopathic thrombocytopenic purpura (ITP).The case was a 31 year old female patient, with a history of chronic ecchymosis, melaena stool and menorrhagia, who presented at the female ...

  14. GENETIC PREDICTORS OF IDIOPATHIC SICK SINUS SYNDROME

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    A. A. Chernova

    2012-01-01

    Full Text Available Published data demonstrating genetic determination of sick sinus syndrome is presented. The definition of this pathology is presented; the main symptoms are described, as well as genes that influence the development of idiopathic sick sinus syndrome, their polymorphisms and role in disorders of the cardiovascular system.

  15. Acute surgical management in idiopathic intracranial hypertension.

    LENUS (Irish Health Repository)

    Zakaria, Zaitun

    2012-01-01

    Idiopathic intracranial hypertension is a headache syndrome with progressive symptoms of raised intracranial pressure. Most commonly, it is a slow process where surveillance and medical management are the main treatment modalities. We describe herein an acute presentation with bilateral sixth nerve palsies, papilloedema and visual deterioration, where acute surgical intervention was a vision-saving operation.

  16. Idiopathic first seizure in adult life

    NARCIS (Netherlands)

    C.A. van Donselaar (Cees)

    1990-01-01

    textabstractThis thesis is based on a prospective investigation of 226 patients aged 15 years and older, who were referred to one of the participating hospitals due to a possible idiopathic first seizure. Only those patients were admitted to the study in whom an obvious cause on clinical grounds

  17. Idiopathic hypertrophic pachymeningitis presenting with occipital ...

    African Journals Online (AJOL)

    Abstract: Background: Although occipital neuralgia is usually caused by degenerative arthropathy, nearly 20 other aetiologies may lead to this condition. Methods: We present the first case report of hypertrophic pachymeningitis revealed by isolated occipital neuralgia. Results and conclusions: Idiopathic hypertrophic ...

  18. Idiopathic hypertrophic pachymeningitis presenting with occipital ...

    African Journals Online (AJOL)

    Background: Although occipital neuralgia is usually caused by degenerative arthropathy, nearly 20 other aetiologies may lead to this condition. Methods: We present the first case report of hypertrophic pachymeningitis revealed by isolated occipital neuralgia. Results and conclusions: Idiopathic hypertrophic ...

  19. Juvenile idiopathic arthritis and the temporomandibular joint

    African Journals Online (AJOL)

    Yasser Mohammed

    e Pediatric Immunology Units, Ain Shams University hospitals, Faculty of Medicine, Ain Shams University, Cairo, Egypt. Received 1 October 2011; accepted 23 November 2011. Available online 1 February 2012. KEYWORDS. Juvenile idiopathic arthritis;. Temporomandibular joint;. Magnetic resonance imaging. Abstract ...

  20. A Case of Idiopathic Spontaneous Intratesticular Hemorrhage

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    Takumi Takeuchi

    2017-09-01

    On visual inspection, the whole testis was black. The spermatic cord was neither distorted nor black. Testicular torsion could not be completely ruled out; thus, left orchiectomy was performed. Histopathology revealed diffuse intratesticular hemorrhage without the necrosis of seminiferous tubular cells. We encountered a case of idiopathic spontaneous intratesticular hemorrhage.