Congenital absence of pituitary gland and adrenal hypoplasia.
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Biomedical subjects
Publications and source records attributed to M W Moncrieff.
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Three children with the haemolytic-uraemic syndrome were treated with intravenous heparin. Peritoneal dialysis was required for two of them, one of whom died after 26 days of therapy. Renal biopsy specimens from the two survivors showed widespread glomerular disease, which appeared permanent in one case, but only occasional thrombi. It is suggested that heparin therapy, by preventing further intrarenal thrombosis, allowed the normal fibrinolytic mechanisms to remove previously formed thrombi.
The clinical, laboratory, and renal biopsy findings in 47 children with symptomless haematuria are reported. In 41 the haematuria was recurrent. Local causes were excluded by means of intravenous urography, which was normal in all but one child, who had a horseshoe kidney.Since all the patients had presented in a similar manner they were classified into four groups according to the severity of glomerular changes on renal biopsy. In group I the glomeruli were optically normal. In group 2 they showed a variable degree of mesangial thickening with absent or minimal cellular proliferation. In group 3 there was diffuse mesangial thickening and proliferation-an appearance indistinguishable from that of subsiding post-streptococcal glomerulonephritis. Compared with groups 1 and 2, more patients in this group had persistent proteinuria, as well as evidence of streptococcal infection preceding the initial haematuria. Only two patients showed severe proliferative glomerulonephritis on biopsy (group 4); both had heavy proteinuria and one repeatedly had low serum beta1(c)-globulin levels.
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Forty-six children with the nephrotic syndrome whose renal biopsy specimens showed minimal changes and whose response to corticosteroid therapy was unsatisfactory were treated with cyclophosphamide. Three patients were completely steroid-resistant from the outset and the remainder were steroid-dependent. In several patients steroids controlled the condition less effectively with time. Most patients showed signs of steroid toxicity, and growth retardation was striking.A moderate leucopenia was induced with cyclophosphamide, and treatment was maintained for three to four months in the majority of cases. Thirty-eight children (83%) have remained in complete remission off all treatment for periods of 3 to 23 months, 33 after one course of cyclophosphamide and five after a second course. Two other patients who remitted but relapsed later are still on treatment. In only six patients was full remission not obtained, and three of these were steroid-resistant from the start. Two died from pneumonia and adrenal failure and four continued to have proteinuria, though in one an impressive reduction occurred.The results indicate that cyclophosphamide therapy is an effective alternative for nephrotic children with normal glomeruli on light microscopy who develop steroid dependence or resistance, and who exhibit toxic effects of steroid therapy.
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