Onychomadesis in a patient with immunoglobulin class switch recombination deficiency

Immunoglobulin class switch recombination deficiencies [Ig CSR deficiencies] or Hyper IgM syndromes [HIGM] are a group of primary immunodeficiency diseases, characterized by defective CD40 signaling of B cells resulting into a CSR and a somatic hypermutation. The affected patients are characterized with reduced serum levels of IgG and IgA, and normal or elevated level of IgM, which lead to increased susceptibility to infections. We describe a 3 year-old boy with frequent bacterial infections of the skin and respiratory tract, mucosal ulcers, and diarrhea. He experienced onychomadesis in both fingernails and toenails during recent bacterial infection. Quantitative immunoglobulin levels revealed high levels of serum IgM and very low levels of IgG, IgA, and IgE. Clinical and immunologic studies supported the diagnosis of HIGM. Onychomadesis as a finding in HIGM could be considered. Considering exclusion of CD40L, CD40, AID and UNG genes by molecular analysis, new CSR selective deficiencies could be suspected in this case

[Prevalence of allergic rhinitis in 13-14 year old school children in Hamedan]

Allergic Rhinitis [AR] is an inflammatory disorder of the nasal mucosa characterized by nasal congestion, rhinorrhea and itching and often accompanied by sneezing and conjunctival irritation. Two prerequisites for the expression of AR are sensitivity to an allergen and its presence in the environment. Recognition of allergens and removal and avoidance of them can decrease the prevalence of AR. This study aims to evaluate the prevalence of AR in Hamedan, Iran, and recognition of effective factors involved in expression of AR. The study was undertaken between October 2005 and June 2006 among 13- to 14-year-old children in middle-schools of Hamedan. Data were collected using the standardized ISAAC written questionnaire for prevalence of AR and another questionnaire for effective factors on AR including gender, season of expression of AR, smoking parents, keeping pets and birds, apartment plants, breast milking and family size. In the second phase the students with AR were examined for signs of AR. A total of 1600 [900 girls and 700 boys] children participated in the study. 17.7% of the children had symptoms of allergic rhinitis sometime in their life. There was no significant difference between prevalence of AR between boys and girls [18.6% in the boys and 17% in the girls] [p>0.05]. 37.2% of the cases were perennial and 62.8% of them were seasonal or mixed type. AR was more prevalent in spring [21%]. The prevalence of the disease between students with smoking parents and non smoking parents were 30.6% and 11.2% respectively [P=0.001]. The prevalence of the disease between students with pets and without pets were 23.1% and 17.7% respectively [P=0.001]. Presence of apartment plants, type of nutrition and family size had no effect on prevalence of AR [P>0.05]. The most common signs of AR were allergic shiners [54%] and clear and dilute nasal secretion [51%]. Compared with previous studies conducted in other Iranian cities using similar method, the prevalence of allergic rhinitis was lower in Hamedan. The prevalence of AR may be reduced with avoidance of smoking and removal of pets

Hair loss as a sign of Kawasaki disease

Kawasaki disease is a multi system disorder with varying clinical expressions. This disease is an acute systemic vasculitis of unknown etiology that has recently recognized as a leading cause of acquired heart disease in children of many developed countries. We describe an unusual instance of hair loss in a patient with Kawasaki disease. A 26 months old boy developed prolonged high fever, bilateral conjunctival infection, arthralgia and erythromatosis skin rash. He was admitted to the hospital with the diagnosis of Kawasaki disease. Laboratory results included an erythrocyte sedimentation rate [ESR] above 100 and platelet count > 1000,000. The patient developed acute and unprovoked scalp hair loss. He was treated with intravenous immunoglobulin [IVIG] 2 g/kg and aspirin 100 mg/kg/day with complete improvement of signs and symptoms. This report documents hair loss as an uncommon presentation of Kawasaki disease

Synergistic effect of intravenous immunoglobulins and iodinated contrast media on renal function

Acute renal failure is one of the side effects while using intravenous immunoglobulins. This complication is also observed with iodinated contrast media. Herein, we describe a patient with acute renal failure who received intravenous immunoglobulins and iodinated contrast media concomitantly. Both drugs are responsible for osmotic nephrosis. The same effect on renal cells may explain a synergistic effect on renal function