Hideo Ogura

Infliximab treatment for refractory Kawasaki disease with coronary artery aneurysm.

Tumor necrosis factor-alpha (TNF-alpha) is considered to be 1 of the factors that induce vasculitis, including coronary artery aneurysm (CA), in Kawasaki disease (KD), because the blood concentration of TNF-alpha is higher in patients with CA compared with those without. Therefore, an anti-TNF-alphaagent (infliximab) was administered to a 1-month-old girl with refractory KD complicated by CA and subsequently, the CA improved and KD was controlled without complications 20 months after the onset.

Turner's Syndrome with Agenesis of the Corpus Callosum, Hashimoto's Thyroiditis and Horseshoe Kidney

We present a 14‐year‐old girl with Turners syndrome, agenesis of the corpus callosum, Hashimotos thyroiditis and horseshoe kidney. She was admitted to our hospital because of short stature. She had some of the somatic stigmata of Turners syndrome, was slightly mentally retarded, but had no neurological abnormalities. Chromosome study showed a 45, x karyotype. Her thyroid function was normal, but both the antithyroglobulin and antimicrosomal antibody titers were elevated, suggesting lymphocytic chronic thyroiditis. An intravenous pyelogram and an abdominal CT scan showed a horseshoe kidney. CT scan of the brain showed agenesis of the corpus callosum.

Two primary doses of diphtheria-tetanus-acellular pertussis vaccine induce immunological responses to Bordetella pertussis as strong as those induced by three primary doses

Pertussis vaccinations are administered worldwide under various conditions and schedules with diphtheria-tetanus-pertussis (DTP). In Japan, a general vaccination with three primary doses of diphtheria-tetanus-acellular pertussis (DTaP) at 4-week intervals and one booster dose 12 months after the primary series have been used since 1981. Decreasing the number of doses of the vaccination would lessen the physical and economic costs. To compare the immunological response to two versus three primary doses, we assessed antibody and cellular immune responses in health children. The anti-filamentous hemagglutinin (anti-FHA) and anti-pertussis toxin (anti-PT) antibody responses to two primary doses of DTaP before a booster were significantly lower than the responses to three primary doses. Although these antibody levels were low in children who received two primary doses, the FHA-induced DNA synthesis was equal to that of the children who received three doses. The anti-FHA and anti-PT antibody levels 4 weeks after the booster following two doses were similar to the levels following three doses, and high antibody titers were maintained over a long period. In areas where contact with bacteria is expected, two primary doses of DTaP may be adequate to induce the necessary level of immunological responses.

Idiopathic thrombocytopenic purpura associated with Helicobacter pylori infection

Recent reports show that Helicobacter pylori infection is associated with some immunological disorders. Furthermore, Gasbarrini et al. 1 and Emilia et al. 2 had independently suggested that the eradication of H. pylori leads to the remission of chronic idiopathic thrombocytopenic purpura (ITP). Although many cases of adulthood chronic ITP achieving remission after eradication of H. pylori have been reported, little is known about childhood chronic ITP. 3 Here, we report a 16-year-old boy with chronic ITP complicated by H. pylori infection.

Renal complications in two patients with dentatorubral-pallidoluysian atrophy.

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder characterized by various combinations of myoclonus epilepsy, ataxia, choreoathetosis and dementia. No specific therapy has been established and renal complication is rare. We report two cases of DRPLA with renal complications. Hematuria and proteinuria had gradually progressed for 2 and 13 years in these patients. Renal biopsy findings revealed focal glomerulosclerosis in one case and end-stage kidney disease in the other case. Angiotensin-converting enzyme inhibitor and angiotensin receptor II antagonist were administered to both patients, resulting in improved proteinuria and preserved renal function in one patient, while renal function continued to deteriorate in the other patient. Although renal complication is rare in patients with DRPLA, the presence of renal disease has to be suspected in patients with persistent proteinuria.