The Alagille Syndrome (AGS) is a heritable disorder affecting the liver and other organs. Causative dominant mutations in human Jagged 1 have been identified in most AGS patients. Related organ defects occur in mice that carry jagged 1 and notch 2 mutations. Multiple jagged and notch genes are expressed in the developing zebrafish liver. Compound jagged and notch gene knockdowns alter zebrafish...

Prolonged eosinophilia of unknown cause has generally been described as the hypereosinophilic syndrome, and is characterised by peripheral blood and bone marrow infiltration and frequent multisystem disease. The nature of this disorder has been questioned, and the clinical features are quite variable, suggesting its heterogeneity and probable neoplastic aetiology. A patient with severe eosinoph...

objectives: tourette syndrome is a disease with vocal and motor tics. this disorder is co-morbid with many psychiatric disorders, among which obsessive-compulsive disorder is the most common. methods: during a one-year period, 30 patients with tourette disorder were studied at an adolescent psychiatry referral clinic. it was an analytical-descriptive study. subjects were selected by convenience...

background due to the lack of enough researches on the impaired underlying mechanisms in the obsessive-compulsive disorder (ocd) and also existence of new psychological therapies such as metacognitive therapy and neurofeedback, the application of mentioned mechanisms in the new therapies can be introduced as a possible effective option with more long term effects to treat ocd. objectives the cu...

Multicentric Castleman's disease (MCD) is a rare lymphoproliferative disorder of unknown etiology and characterized by various clinical manifestations and multiple organ involvement. It has been reported in association with POEMS syndrome and can progress to Kaposi's sarcoma or malignant lymphoma. The disease runs a more aggressive course and a poor prognosis. Optimal therapies have not been we...

Systemic lupus erythematosus (SLE) is a multisystem, autoimmune disorder that poses a challenge to the emergency physician with its protean and occasionally dangerous manifestations. Morbidity for SLE patients is typically mediated through organ inflammation and destruction or the consequences of therapeutic immunosuppression. Further complicating lupus is the frequent association with the anti...

Malignancy is a common cause of disseminated intravascular coagulation and usually presents as a chronic disorder in solid organ tumours. We present a rare case of recurrent acute disseminated intravascular coagulation in neuroendocrine carcinoma after manipulation, firstly, by core biopsy and, later, by cytotoxic therapy causing a release of procoagulants and cytokines from lysed tumour cells....

Tacrolimus, a novel immunosuppressant agent, has been widely used in organ transplantation and autoimmune diseases. We herein present a case of neuromyelitis optica spectrum disorder (NMOSD) combined with Sjögren's syndrome (SS) successfully treated with tacrolimus. This patient repeatedly presented with recurrent longitudinally extensive transverse myelitis. Her NMO-IgG and anti-SSA and anti-S...

Pregnancy-induced hypertension is a disorder of unknown etiology unique to pregnant women. Classic clinical manifestations include hypertension, proteinuria, and edema. Early recognition and proper management of this disease may serve to avoid serious maternal complications. Ultimate maternal treatment depends on delivery of the fetus and placenta. Advanced stages of this disease result in mult...

There are three categories of blood eosinophilia: reactive (non-clonal), clonal and Idiopathic hypereosinophilic syndrome (HES). The empirical diagnostic criteria of HES are: blood eosinophilia exceeding 1.5xl09/l for more than six consecutive months; absence of an underlying cause of hypereosinophilia despite extensive diagnostic evaluation and organ damage or dysfunction as a result of local ...