Although susceptibility to infections in Kabuki make-up syndrome (KMS) has frequently been reported, there have been few immunological studies. We describe a 14 year old girl with KMS exhibiting chronic idiopathic thrombocytopenic purpura (chronic ITP), including immunological studies.Corticosteroid therapy was not effective therefore splenecfomy was performed. The patient's platelet count increased transiently. Immunological studies revealed normal T cell functions and low serum immunoglobulin A (1gA) levels.Because of the abnormalities of B cell functions in chronic ITP and low serum IgA levels in our patient, we speculate that there may be some abnormalities of humoral immunity in KMS.
We report two children who suffered from sodium bromate intoxication due to ingestion of the second preparation for permanent hair waving (the second permanent preparation). One child suffered from gastrointestinal symptoms only. The other exhibited slight acute renal insufficiency. Results of the histological examination of the kidney in the sick child with acute renal insufficiency showed sporadic epithelial separation of the proximal tubuli under light microscopy. In addition, we could demonstrate more clearly epithelial separation and unbroken tubular basement membranes under electron microscopy (EM). To our knowledge, this is the first report of EM findings in this disease. The mechanism of epithelial injuries by bromate is not clear.
Anti-phospholipid antibodies (aPL) are concerned with many central nervous system diseases in systemic lupus erythematosus (SLE). However, no report has described the relationship between aseptic meningitis and aPL in SLE. We report a case of SLE with aPL, presenting cerebral infarction and aseptic meningitis.A 14 year old female with SLE with aPL experienced cerebral infarction and recurrent aseptic meningitis. Combination therapy with steroids and aspirin improved the condition and prevented relapses.The aPL are associated with cerebral infarction, even in young patients with SLE. In addition, aPL may induce aseptic meningitis in SLE.
In order to prevent vertical infection between mother and infant with HBV, a method of giving passive immunity by anti‐HB human immune globulin just after birth and then active immunity by means of HB vaccine has been initiated. However, the sustained effect of antibody acquired through HB vaccine is not ascertained.
In this study, we examined the appearance rate of anti‐HBs titer and its sustained effect by administering HB vaccine three times.
Anti‐HBs appeared in all 13 cases by five months after birth, after the injection of HB vaccine three times. The titer was from 64 to 1,024. However, nine months after birth, there was a reduction of 1–2 tubes, and antibody titer was 64 to 512. Twelve months after birth a further reduction of 1–2 tubes was observed. In those cases in which anti‐HBs titer was under 256 at six months, by 12 months it decreased to 16–32. In such cases, a booster injection 12 months after birth was considered necessary.
GOT and GPT showed changes within the normal range, and no local reaction at the injection site was found.
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