[Evaluation of fresh frozen plasma consumption in children medical center]

FFP [Fresh Frozen Plasma] containing all coagulation factors is used to treat coagulation disorders. In the present study, FFP consumption in Children Medical Center was studied to evaluate transfusion indications leading to blood orders some of which might have been unnecessary bringing about inappropriate blood use or unused blood donations. In this retrospective study, 1262 FFP order and reservation requests during one year for being administered to 735 patients were reviewed by census method. The results were analyzed by Chi square and SPSS. Out of 1262 requested FFP units, 952 [75.4%] were transfused, and 90 units [7.13%] though thawed were not. Out of 220 reserved units, only 22 units were transfused. Taking more attention in ordering will prevent blood loss and increase economic saving. The blood bank of Children Medical Center does not embark on thawing FFP before its usage is finally confirmed so that blood loss is somehow avoided

Congenital self healing cutaneous langerhans cell histiocytosis with multiple hypopigmented macules after recovery in a neonate

Langerhans cell histiocytosis [LCH] is the most common type of childhood histiocytic disorder with an incidence of 0.2 to 1 per 100,000 children under the age of 15 years [1]. The clinical picture of LCH varies from single system [S-S]bone or skin disease to multi-system disease [M-S] [2]. We report a neonate with disseminated papulonodular eruption containing mononuclear CD1a and S100 positive histiocytic cells infiltration at epidermis and underlying dermis. The diagnosis of a congenital self healing Langerhans cell histiocytosis [CSHLCH] was made and follow up showed a complete recovery of the eruptions, leaving hypopigmented macules in the sites corresponding to the initial findings

[ case report of acute hemolytic crisis as the initial manifestation of Wilson disease]

Wilson disease is a hereditary disorder of copper metabolism which can present with hepatic, neurologic or psychiatric symptoms and in rare cases as a hemolytic disturbance. Free copper can result in red blood cell damage and hemolysis which is a rare feature of the disease affecting less than 10% of patients. In this condition the liver is usually involved and liver transplantation can be life saving. This article is a case report of acute hemolytic crisis as the initial manifestation of Wilson disease. An 8 years old girl was admitted in pediatric ward of Golestan hospital because of abdominal pain, icterus, anemia and tea color urine. Hepatitis or Glucose 6 Phosphate Dehydrogenase deficiency was the first diagnosis. Because of unresponsiveness to transfusion, Wilson disease was considered and the diagnosis was established with the presence of Kayser Fleischer ring. Chronic or acute hemolytic anemia is a rare or unusually presentation of Wilson disease. In any child especially older than 5 years with liver disease or hemolytic anemia, Wilson disease should be considered and appropriate diagnostic tests performed

Intracranial rhabdomyosarcoma presented as chronic subdural hematoma: a case report

Intracranial rhabdomyosarcoma is a very rare disorder. Subdural rhabdomyosarcoma has not been reported yet. It can be misdiagnosed with chronic subdural hematoma in CT images. Herein, we presented a 2.5-year-old boy with intractable chronic subdural hematoma who were treated with burr hole insertion, needle aspiration and shunting for about 2 years with partial resolution of his symptoms after each procedure. The final pathologic evaluation after extensive bilateral craniotomy revealed rhabdomyosarcoma. The role of CT and MRI in early diagnosis and management of this rare situation is discussed

Report a rare hemifacial atrophy-romberg disease

Romberg disease is a rare progressive disease with hemifacial atrophy of skin, soft tissue and bone. It was first described by parry in 1825 and later in 1846 by Romberg and in 1871 named as a progressive hemifacial atrophy by Eulenbery. First signs of this rare disease usually appear before the age of 20 and initially skin and subcutaneous parts are involved and later, there is muscle and bone involvement. In 95% of cases there is unilateral atrophy. Different theories are mentioned about the exact cause of this rare disease such as Rubeolla infection, trigeminal neuralgia, sclerodermia, cervical sympathetic anomaly, but the exact cause is not known yet. Progression of disease usually lasts between 2 to 10 years, but in some cases it takes longer. Treatment includes skeletal and soft tissue reconstruction, although in most of cases soft tissue rehabilitation is enough. The patient is a 20 year old woman who was suffering from atrophy of soft tissue and skull in the right frontal and parietal areas plus loss of eyebrow for the past 9 years. In biopsy skin and adnexal atrophyed was identified. The progression of disease ceased after 2 years and reached a steady state. The patient was a scheduled for tissue expander insertion in scalp area [left side] to expand hair full area and flap rotation and covering hairless area of scalp [right side]. In 2 steps operation minimal surgical scar with satisfaction of patient was achieved and this way may be an effective way for limited form of this disease