ABSTRACT
Kawasaki disease is a vasculitis with unknown cause, which is most commonly seen in children younger than 5 years old. The incidence of the disease is between 0.8-3% and male/female ratio is 1.37. Kawasaki is diagnosed basd on prolonged fever at least five days with four of five clinical criteria. Recurrent diseases occur in 1-3% of cases. Multiple recurrences have been reported rarely. We report on a 4-year-old boy that experienced three attacks of Kawasaki disease. All three clinical features were complete diseases and he was treated with IVIG. He was healthy after 6 months of follow up after the third recurrence. We report a case of multiple recurrent Kawasaki disease with coronary aneurysm in different portion. There was no adverse sequelae, after he is treated with IVIG
ABSTRACT
Congenital adrenal hyperplasia [CAH] is an of autosomal recessive disorder of adrenal steroidogenesis leading to a deficiency of cortisol. The deficiency of cortisol results in increased secretion of corticotropin which, in turn leads to adrenocortical hyperplasia and overproduction of intermediary metabolites. In 90% of the cases, with this disease, there is a problem with the enzyme 21 hydroxylase [21 OHD]. Severe and mild forms of these disorders, caused by variations in the severity of the genetic mutations have been reported. Depending on the enzymatic step that is deficient, there may be signs, symptoms and laboratory findings of mineralocorticoid deficiency or excess; incomplete virilization or premature androgenization of the affected male and virilization or sexual infantalism in the affected female. It appears that this disease is more prevalent in Iran because of the custom of family marriages, however its exact prevalence needs correct diagnosis and documentation of cases. In this article the process of the disorders, diagnosis and treatment will be discussed