Gliomatosis cerebri with spinal metastasis presenting with chronic meningitis in two boys.

Spinal cord involvement in gliomatosis cerebri (GC) is uncommon. We report two patients with GC, who initially presented with chronic meningitis and were treated with antituberculous drugs. Although tumor meningitis was suspected, due to the intractable clinical course, a correct diagnosis was established after performing a biopsy examination of the metastatic spinal lesion which was detected by magnetic resonance imaging (MRI). Cerebrospinal fluid examination, including cytology, should be performed repetitively for patients with chronic meningitis refractory to antibiotic treatment. Spinal MRI is necessary for the complete neurological workup, even when the patients do not show spinal symptoms.

A rare cause of childhood-onset nephrotic syndrome: lipoprotein glomerulopathy.

We report on a 12-year-old female patient with lipoprotein glomerulopathy (LPG) who was proven to be heterozygous for ApoE2 Kyoto (Arg25Cys). Her family members have the same variant but do not have obvious signs of renal function impairment. Six months of treatment with a statin caused significant clinical improvement in the lipid profile, proteinuria, and renal function. Our case suggests that administration of a statin is a potential therapeutic strategy for improving nephrotic syndrome in patients with LPG.

Universal hepatitis B vaccination reduces childhood hepatitis B virus-associated membranous nephropathy.

OBJECTIVE: To compare the incidence of hepatitis B virus (HBV)-associated membranous nephropathy (HBVMN) before and after universal HBV vaccination and to identify factors underlying the change. METHODS: This study included 471 hospitalized children with nephrotic syndrome (NS) and 488 long-term follow-up hepatitis B surface antigen (HBsAg)-carrier children. Horizontal transmission (negative maternal HBsAg status) of HBVMN and HBV was assessed, and the incidence of HBVMN was compared before and after initiation of the universal HBV vaccination program started in 1984. RESULTS: The frequency of HBVMN in children with NS was 11.6% between 1974 and 1984, 4.5% between 1984 and 1994, 2.1% between 1994 and 2004, and 0% between 2004 and 2009. Similarly, the number of HBsAg-seropositive children with NS (mainly via horizontal infection) decreased after universal vaccination. The prevaccination frequency of HBV horizontal transmission in chronic HBsAg carriers from the general population was 36.5% compared with 5% in the postvaccination period. The incidence of HBVMN in these carriers revealed a parallel decline. CONCLUSIONS: Our results revealed a significant decrease in the frequency of HBVMN in children with NS and in long-term follow-up HBsAg carriers. Children with HBVMN are primarily infected with HBV via horizontal transmission; thus, the significant reduction in horizontal transmission in HBsAg-carrier children in the general population after universal HBV vaccination may explain the reduction of HBVMN in the vaccinated population. These findings confirm the effectiveness of HBV vaccination on reducing the incidence of HBVMN, possibly through a significant decline in horizontal HBV infection.