Marfan syndrome is an autosomal dominant disorder involving mutation in the FBN1 gene, which encodes fibrillin-1, a protein critical to maintain the integrity of connective tissue. A mutation in this gene can affect multiple organ systems, but it is not classically associated with gastrointestinal complications. We describe a man with Marfan syndrome with multiple small bowel diverticula leadin...

22q 11 deletion syndrome (22q11DS; Online Mendelian inheritance in man #192430) also known as velocardiofacial or DiGeorge syndrome, is a genetic disorder resulting from a hemizygous microdeletion of the long arm of chromosome 22. It has an estimated prevalence of 1 out of 4000 live births and results in a heterogeneous clinical presentation that is irrespective of deletion size and can be asso...

The Marfan syndrome is an autosomal dominant disorder of connective tissue characterized by a large number of possible mutations, and by heterogeneity of clinical presentation primarily in skeletal, ocular and cardiovascular organ systems. Cardiovascular complications of the disease are responsible for high mortality. The case of a young patient with a progressive advanced abdominal aorta dilat...

Prader-Willi syndrome (PW) is a rare genetic disorder with multi-organ system involvement. These patients present many perioperative challenges including sleep-related breathing disorders, morbid obesity, thick salivary secretions, mental retardation, and difficult intravenous access. PW has been suggested to be associated with central adrenal insufficiency. We report a novel case of persistent...

Carcinoid syndrome is a rare disorder caused when elevated levels of vasoactive substances secreted by a carcinoid tumor fail to be metabolized by the liver. This can occur for a variety of reasons including metastatic invasion of the organ. Carcinoid syndrome results in elevated levels of 5-hydroxyindoleacetic acid in the urine. Clinical manifestations include: flushing, diarrhea, bronchospasm...

Solid organ transplantation (SOT) is the preferred treatment for an expanding range of conditions whose successful therapy has produced a growing population of chronically immunosuppressed patients with potential neurological problems. While the spectrum of neurological complications varies with the type of organ transplanted, the indication for the procedure, and the intensity of long-term req...

Churg-Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg-Strauss syndrome. The propositus included, 50 cases of childhood Churg-Strauss syndrome have been reported. The patient characteristics and ...

how to cite this article: hakim shoushtari m, ghalebandi mf, tavasoli a, pourshams m. kleine-levin syndrome in an 8 year old girl with autistic disorder does autism account a primary or secondary cause? iran j child neurol. autumn 2015;9(4):61-64. abstract objective kleine–levin syndrome (kls) is a rare disorder with an unknown etiology. autism spectrum disorder is characterized by various degr...

olanzapine is the frequently prescribed drug in children and adolescents with bipolar disorder, but unfortunately it has metabolic side-effects. on the other hand, in a number of melatonin studies on sleep cycle, regulation of metabolic abnormalities has been reported. therefore, we aimed to study effects of melatonin in reducing metabolic side-effects of olanzapine in 11-17 year-old patients w...

introduction: the aim of this study is essay, the association between borderline ersonality disorder and premenstrual syndrome in patients with borderline personality.  method: in this case -control study, 400 students at high schools stage of education in tehran, randomly selected. all the students were aged between 19-15 y , did not use ocp, regular menstrual cycles and cycle lengths (between...