PURPOSE: The pathophysiology of hypogammaglobulinemia in nephrotic syndrome (NS) remains unknown. We evaluated the differences in the distribution of anti-bacterial antibodies and anti-viral antibodies, and those of immune antibodies and natural antibodies in steroid-sensitive NS. MATERIALS AND METHODS: We examined the antibody status of 18 children who had routine vaccinations. The levels of immnunoglobulin G (IgG), the IgG subclasses, and the antibodies induced by vaccinations such as diphtheria-pertussis-tetanus and measles-mumpsrubella were analyzed in children with steroid-sensitive NS. RESULTS: There was a positive correlation between the albumin and IgG values (r = 0.6, p < 0.01), and the four IgG subclasses were all evenly depressed in the nephrotic children during the acute stage of the disease. The antibodies induced by bacterial antigens were depressed and the seropositivity of anti-viral antibodies tended to be lower than those of age-matched control children during the acute stage. The depressed immune antibody status recovered rapidly in the remission stage of NS, despite corticosteroid treatment. CONCLUSIONS: IgG levels correlated positively with albumin levels, and all antibodies, including immune and natural antibodies, were depressed in the acute stage of NS. Our results suggest that hypogammaglobulinaemia in NS may be associated with intravascular homeostasis of oncotic pressure.
Adolescent ; Antibodies, Bacterial/immunology ; Antibodies, Viral/immunology ; Case-Control Studies ; Child ; Child, Preschool ; Female ; Humans ; Immunoglobulin G/immunology ; Male ; Nephrotic Syndrome/*drug therapy/*immunology ; Steroids/*therapeutic use

Nephrotic syndrome associated with Tsutsugamushi disease has not been previously reported. We are describing a case of Tsutsugamuchi disease presenting with nephrotic syndrome. A 72-year-old woman presented with fever and generalized edema. Laboratory studies revealed a leukocytosis, hypoalbuminemia, and hypercholesterolemia. Her urine protein excretion was 5.4 g/day. The anti-Tsutsugamushi antibody test was strongly positive (1:2,560). A renal biopsy was performed, and pathologic findings revealed membranous glomerulonephritis. The patient's clinical symptoms improved markedly after treatment with doxycycline.
Aged ; Anti-Bacterial Agents/therapeutic use ; Antibodies, Bacterial/blood ; Biopsy ; Doxycycline/therapeutic use ; Female ; Glomerulonephritis, Membranous/diagnosis/*etiology ; Humans ; Nephrotic Syndrome/diagnosis/*etiology ; Orientia tsutsugamushi/immunology ; Scrub Typhus/*complications/diagnosis/drug therapy/microbiology ; Treatment Outcome

OBJECTIVERecent studies suggest that T cell dysfunction, especially IL-4, may be involved in the pathogenesis of steroid sensitive nephrotic syndrome (SSNS). The aim of this study was to investigate the association between the polymorphisms in variable numbers of tandem repeat region (VTR) of IL-4 gene and childhood SSNS.

METHODThe polymorphism in the IL-4 gene was identified by using the polymerase chain reaction and direct sequencing methods in 55 Chinese children with SSNS, who were followed-up for at least 1 year, and 115 healthy Chinese adult blood donors as controls. A variable number of tandem repeat (VNTR) region polymorphisms of IL-4 gene were detected, and alleles were designated as IL-4 B1 and B2, corresponding to 2 and 3 repeats, respectively. The serum IgE was also examined in 48 patients before the steroid treatment.

RESULT(1) There were no significant differences in the genotype and allele frequencies between patients with SSNS and normal controls (P > 0.05). (2) The frequencies of B1B1 (96.4%) were significantly higher in SSNS children with frequent relapses (28 patients had more than 3 relapses during the first year of the disease) than in 27 patients without frequent relapses (P < 0.05). (3) Twenty-three patients with frequent relapses showed higher levels of serum IgE (1.98 +/- 0.23 g/L) than 25 patients (IgE: 1.05 +/- 0.19 g/L) without frequent relapses (P < 0.05), and the patients with B1B1 (38 patients) also had higher serum IgE levels than patients with B1B2 and B2B2 (10 patients) (P < 0.05).

CONCLUSIONThe results suggest that IL-4 VNTR B1B1 genotype might be a predictor for the frequent relapse in childhood SSNS in Chinese.

Adolescent ; Asian Continental Ancestry Group ; Child ; Child, Preschool ; Female ; Gene Frequency ; Genetic Predisposition to Disease ; Genotype ; Humans ; Immunoglobulin E ; blood ; Infant ; Interleukin-4 ; genetics ; Male ; Minisatellite Repeats ; Nephrotic Syndrome ; drug therapy ; genetics ; immunology ; Polymerase Chain Reaction ; Polymorphism, Genetic ; Recurrence ; Steroids ; therapeutic use