macrophage activating syndrome (mas) is a life-threatening disease seen in autoimmune diseases including lupus erythematosus, rheumatoid arthritis, still's disease, polyarteritis nodosa. it is characterized by fever, pancytopenia, liver failure, coagulopathy, and neurologic symptoms and high serum ferritin. a 27 yr. old female patient was admitted in shahid mostafa khomeini hospital (tehra...

primary immunodeficiencies (pid) are a group of disorders, characterized by an unusual susceptibility to infections. delay in diagnosis results in increased morbidity and mortality in affected patients. the purpose of this study was to determine the mortality rate of iranian immunodeficient patients referred to children medical center hospital affiliated to tehran university of medical sciences...

superior vena cava (svc) syndrome is not an uncommon occurrence in patients with malignancy and it is often described as a medical emergency. in majority of the cases, svc syndrome occurs due to mechanical obstruction of the svc by extraluminal compression with primary intrathoracic malignancies. however, intraluminal obstruction due to thrombosis can also produce symptoms and signs of svc synd...

in this case report, we present the first diagnosed case of galloway- mowat syndrome in iran. a 7 month old infant boy with microcephaly that had prominently stunted head growth after birth, gastroesophageal reflux, multiple craniofascial characters, hypothyroidism and nephrotic syndrome diagnosed at 5 months of age associated with rapid decline in renal function and heavy proteinuria in 2 mont...

a female one month old with features supporting a diagnosis of neonatal progeroid syndrome(wrs)  presented to our neonatology section of gb pant children hospital srinagar .she had prenatal and post natal growth failure, generalized lipotrophy, triangular face, psedohydrocephalous, sparse scalp hair and eye brows, prominent scalp veins and greatly widened anterior fontenella.

reports which describe sick sinus syndrome due to malignant lymphoma have been rare and only eight cases have been reported until now. this is a case of sick sinus syndrome and superior vena cava syndrome secondary to invasion of occult malignant lymphoma of the lung in a 60 years old male. there were no symptoms or signs of malignancy before the first presentation with sick sinus syndrome. pat...

case presentation the patient was a 65-year-old female with sps diagnosed 1 year before. assessments were performed, such as range of motion, muscle strength, pain, balance, and functional mobility. she had shoulder pain, with a score of 5/10 on the numerical pain rating scale for both shoulders. moreover, her muscle strength was impaired. although the patient could not maintain an upright posi...

nevoid basal cell carcinoma syndrome (bcns) is an autosomal dominant inherited disorder. multiple organ systems may be affected in this syndrome including abnormalities of the skin, skeletal system, genitourinary system and central nevus system. in this report, we present a case of nevoid basal cell carcinoma syndrome in a 26-year-old male patient. the patient had multiple odontogenic keratocys...

autoerythrocyte sensitization syndrome cabs) is a rare purpuric disorder of women characterized by inflammatory and painful ecchymotic lesions unrelated to blood clotting or vascular abnormalities. gastrointestinal bleeding, hematuria, headache or syncopal attacks may also be observed. our patient is a 33-year-old woman presenting with recurrent severe painful ecchymotic lesions on both lower e...