Deficiency in the second component of complement (C2) is the most common homozygous complement deficiency. While approximately half of the affected individuals are apparently healthy, C2 deficiency may be associated with autoimmune diseases and rarely increased susceptibility to infection. We report 5 patients who had homozygous type I C2 deficiency in two families. Three of them suffered from frequent infections. These symptomatic patients had additional risk factors; the index cases in the first and the second family had IgG2 deficiency and IgA deficiency, respectively, and alternative complement pathway hemolytic activity was also low in both of them and in the sibling of the first index case. These results emphasize the probable role of other immunologic defects in the clinical presentation of C2 deficiency.

The clinical features of three children with Griscelli syndrome and autopsy findings of two are presented. The patients were 5 years, 9 months, and 3 months old, respectively. Clinical features included partial albinism, hepatosplenomegaly, and various neurological symptoms. Light and electron microscopic studies of the skin were compatible with Griscelli syndrome. Postmortem examination of the viscera and central nervous system revealed lymphohistiocytic infiltration with erythrophagocytosis. Bilateral diffuse involvement of the central nervous system, cranial nerve, and spinal cord was detected in both cases.

2. Materials and methods 2.1. Strains and susceptibility testing Random amplified polymorphic DNA (RAPD) analysis was used to determine the genetic relatedness of 58 E. coli isolates from cattle, goats, sheep, cats, and dogs. Twenty different RAPD patterns were observed among these isolates and one representative isolate was chosen from each pattern based on resistance genotype.

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Immune thrombocytopenia (ITP) is a frequently encountered disease in childhood. Recent reports pointed to the benefit of high-dose steroid in ITP treatment since it resulted in a better outcome in a shorter time than IV immunoglobulin therapy. In the authors' clinic, mainly after 1984, megadose methyl prednisolone (MDMP) has been used for ITP treatment. There is no report that includes an extensive immune system examination of megadose steroid effect in childhood ITP. The purpose of this study is to determine the effects of MDMP therapy on the immune system in childhood ITP for serum immunoglobulins, absolute lymphocyte and lymphocyte subclass counts, and in vitro blastogenic responses to mitogens. The authors have demonstrated that lymphocyte subtypes (CD3, CD4, CD8, CD19) and serum immunoglobulin G, A, M increased after a short-course MDMP therapy in comparison to pretreatment values in mixed group (acute + chronic) of childhood ITP patients. Also blastic transformation function of lymphocytes with Conca-A and phytohemaglutinin showed an upward trend in 6 of the 9 patients. Steroid are thought to have a suppressive effect on the immune system but this study suggests that short-course MDMP may not be hazardous to the immune system.