Histological Analysis of Serial Renal Biopsy Specimens from Children with Immunoglobulin A Nephropathy Not Treated with Immunosuppressants.

BACKGROUND: Although pediatric immunoglobulin A nephropathy (IgAN) is considered to have a good prognosis, few studies have investigated histological changes over time in IgAN. Serial renal biopsies were performed during the course of the disease and histological changes were observed in patients who did not receive immunosuppressive treatment. To our knowledge, this is the first report of two or more histological evaluations of renal biopsies from patients with pediatric IgAN who did not receive immunosuppressive drugs. METHODS: Forty-two patients with biopsy-proven IgAN who did not receive immunosuppressive agents and underwent serial renal biopsies were followed in our hospital between 1990 and 2003. This retrospective study evaluated findings from renal biopsy specimens and medical records. RESULTS: Analysis of histological findings showed that 19 of 42 patients improved and 16 showed exacerbation of mesangial proliferation. Seven patients showed no obvious histological changes. Of the improved cases, 11 showed spreading of chronic lesions, and there was a significant difference between patients with and without segmental glomerular sclerosis or adhesion at the first biopsy. Of the exacerbated cases, only 5 of 16 patients showed strong active lesions at the first renal biopsy. CONCLUSIONS: Histological changes were investigated in pediatric IgAN patients not receiving immunosuppressive treatment. The results suggest that, even if mesangial hypercellularity improves, chronic lesions may spread during the natural history of the disease. Predicting histological changes by using findings from renal biopsies performed early after onset is difficult; therefore, patients should be carefully followed.

A case of IgA vasculitis with necrotizing arteritis in a 13-year-old girl.

IgA vasculitis (IgAV) is the most frequent form of vasculitis in childhood which classically presents with purpura of the lower extremities, joint pain or swelling and abdominal pain. Though it is a self-limiting disease, and its prognosis is generally good, glomerulonephritis is one of the most important complications. IgAV is classified as a small vessel vasculitis, and though glomerulonephritis develops in IgAV, necrotizing arteritis is rarely seen. Here, we present a case of a 13-year-old girl with IgAV, glomerulonephritis, and necrotizing arteritis in the small renal arteries. There have been only a few reports of adult cases of IgAV with necrotizing arteritis in the kidneys, but there have been no pediatric cases. Some previous reports showed a high mortality rate and implied the possibility of overlap with other vasculitides. In the current report, a rare case of IgAV is described which exhibited necrotizing arteritis rather than overlap with another vasculitis, with a relatively typical clinical course for IgAV and laboratory tests.

A case of anhidrotic ectodermal dysplasia presenting with pyrexia, atopic eczema, and food allergy.

Anhidrotic ectodermal dysplasia (AED) is a rare hereditary disorder with a triad of sparse hair, dental hypoplasia, and anhidrosis. Here we report a case of AED with food allergy and atopic eczema. The patient was a 11-month-old boy admitted to our hospital with pyrexia for 2 weeks. He presented with a history of dry skin, eczema, and food allergy to egg. On clinical examination, his body temperature was 38.8°C, with dry skin and eczema almost all over the body, sparse eyebrows, and scalp hair. Laboratory investigations and physical examination did not show any evidence of infection. Radioallergosorbent test was positive to egg yolk, egg white, ovomucoid, milk, house dust, and house dust mite. As the child did not sweat despite the high fever, we performed the sweat test which revealed a total lack of sweat glands. Genetic examination revealed a mutation of the EDA gene and he was diagnosed as AED. His pyrexia improved upon cooling with ice and fan. His mother had lost 8 teeth and her sweat test demonstrated low sweating, suggestive of her being a carrier of AED. Atopy and immune deficiencies have been shown to have a higher prevalence in patients with AED. Disruption of the skin barrier in patients with AED make them more prone to allergic diseases such as atopic eczema, bronchial asthma, allergic rhinitis and food allergy. Careful assessment of the familial history is essential to differentiate AED when examining patients with pyrexia of unknown origin and comorbid allergic diseases.