[Membranous nephropathy following allogeneic peripheral blood stem cell transplantation in a boy with anaplastic large cell lymphoma].

We report the case of 13-year-old boy who had been diagnosed as having anaplastic large cell lymphoma (ALCL) when he was 11 years old. He suffered a relapse despite the chemotherapy regimens he had been subjected to. Since anaplastic lymphoma kinase (ALK), one of the important prognostic factors of ALCL, was not expressed in the tumor cells, allogeneic peripheral blood stem cell transplantation (PBSCT) from his HLA-matched elder brother was performed. Eleven months after PBSCT, the patient developed nephrotic syndrome as a consequence of chronic graft-versus-host disease (GVHD). He was diagnosed as having membranous nephropathy (MN) based on the results of histological examinations. Soluble interleukin-2 receptor and anti-nuclear antibody closely reflected the clinical course of MN, therefore some immune mechanisms closely related to chronic GVHD seemed to contribute to the occurrence of MN after PBSCT.

[A boy with occipital lobe epilepsy showing prolonged QTc in the ictal ECG].

We report a 9-year-old boy with occipital lobe epilepsy who showed a prolonged QTc on the ictal electrocardiogram (ECG). He complained of sudden onset of blindness accompanied with vomiting and headache. Since ECG revealed a prolonged QTc, he was transferred to our pediatric emergency center. Occipital spike and slow wave complexes on the electroencephalogram (EEG) suggested the diagnosis of occipital lobe epilepsy. Administration of carbamazepine (190 mg) resulted in the disappearance of the blindness and abnormal waves on EEG. This is the first report to describe the occurrence of prolonged QTc during seizures of occipital lobe epilepsy. Because of the high risk of life-threatening ventricular arrhythmia induced by prolongation of QTc such as torsades de pointes, attention should be paid to the ECG findings in patients with occipital lobe epilepsy.

Up-regulation of interleukin-2 mRNA in children with idiopathic nephrotic syndrome.

The current hypothesis for the pathogenesis of childhood idiopathic nephrotic syndrome (INS) favors the involvement of a T cell-mediated immune response. Various cytokines derived from T cells may play a critical role in INS. Previous studies have measured serum or urine cytokine levels and suggest an imbalance of the T cell-mediated immune response. To elucidate the true profile of T cell-derived cytokines, we determined interleukin (IL)-2, interferon (IFN)-gamma, IL-4, IL-10, and tumor necrosis factor (TNF)-alpha mRNA expression in children with INS. We collected mRNA from peripheral blood mononuclear cells together with plasma and urine from nine children in the acute and remission phases of INS. Expression of IL-2, IFN-gamma, IL-4, IL-10, and TNF-alpha mRNA was determined by a quantitative real-time PCR assay. Plasma and urine cytokine concentrations were measured using a specific enzyme-linked immunosorbent assay. These data were compared between the acute and remission phase in the same patients. The IL-2 mRNA levels were significantly higher in the acute phase than in the remission phase, whilst no significant difference was found in the other cytokines investigated. There was no significant difference in the plasma and urine cytokine concentrations between the acute and remission phase. Our results indicate increased expression of IL-2 mRNA in the acute phase of INS, suggesting that IL-2, at least in part, might be involved in the pathophysiology of childhood INS.