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On the occasion of a new case and review of 9 others in the literature, the authors seek to establish the hallmarks of the nephritis occurring after M. pneumoniae infection. It often consists of an acute nephritic syndrome, 10 to 40 days after a respiratory tract infection; hematological abnormalities are not constant. Histological examination of the kidney shows a type I membranoproliferative glomerulonephritis in 2, dense deposit disease in 2 and tubulo-interstitial nephritis in one. Immunofluorescence studies have shown the microbial antigen 3 times out of 4. Specific antibiotherapy does not charge the prognosis of the extrarespiratory manifestations, for which an immunologic mechanism is likely. 相似文献
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Saha A Bagri N Mehera N Dubey NK Batra V 《Journal of pediatric endocrinology & metabolism : JPEM》2011,24(9-10):789-792
We present a rare case of membranoproliferative glomerulonephritis (MPGN) associated with autoimmune hypothyroidism in a child. The exact pathogenesis of glomerulonephritis remains unclear. Thyroxine replacement therapy along with steroids may lead to significant decrease in proteinuria and resolution of edema. Thyroid status should be evaluated in all cases with MPGN. 相似文献
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Membranoproliferative glomerulonephritis in childhood cirrhosis associated with alpha1-antitrypsin deficiency. 总被引:3,自引:0,他引:3
Three alpha1-antitrypsin (alpha1AT) deficient, protease inhibitor type ZZ children who died from cirrhosis and its complications had membranoproliferative glomerulonephritis at postmortem examination. During life, all three had clinical and laboratory evidence of renal disease which became apparent when hepatic decompensation developed. Immunofluorescence studies and electron microscopy performed in one patient revealed subendothelial deposits of alpha1AT, complement, and immune globulins along the glomerular basement membrane. The pathogenesis of these renal lesions is speculative. Glomerular lesions were not observed in kidney sections of 16 children who died from cirrhosis but who were not alpha1AT-deficient. The present study suggests that renal involvement may be yet another manifestation of disease associated with alpha1AT deficiency. 相似文献
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Membranoproliferative glomerulonephritis: the Cincinnati experience--cumulative renal survival from 1957 to 1989 总被引:1,自引:0,他引:1
P T McEnery 《The Journal of pediatrics》1990,116(5):S109-S114
Seventy-six children with idiopathic MPGN have been followed at this medical center since 1957 (mean 10.6 years of disease). Seventy-one of the children have been treated with a regimen of prednisone (mean 7.7 years of treatment). Monotherapy with alternate-day prednisone was used in 50 of the children. Other immunosuppressive and cytotoxic drugs have not been used since 1981. In the 71 treated patients, the cumulative renal survival (creatinine less than 3.0 mg/dl (265.5 mumol/L)) was 82% in the tenth year and 56% in the twentieth year after disease onset. Cumulative renal survival, calculated from the date of initiation of the prednisone regimen, was 75% in the tenth year and 59% in the twentieth year. As our experience with this disease has increased, long-term cumulative renal survival has improved. 相似文献
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Thirty-eight families with cancer in two or more children were identified from records of the Sidney Farber Cancer Center and the Children's Hospital, Boston. Neoplasms developed in one pair of identical twins and in 37 sets of sibs (2 with 4 affected sibs, 4 with 3, and 31 sib-pairs). Predisposition to cancer extended to their parents and other relatives, and subsequently to other sibs who were unaffected at the time of ascertainment of the cancer-prone families. Genetic susceptibility was suggested in individual families by parental consanguinity or by an inherited disorder predisposing to cancer, but in other instances no oncogenic factors were identified. Familial aggregates of childhood provide opportunities for etiologic study and cancer control. 相似文献
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15 patients with typhoid glomerulonephritis were studied and compared with a group of children with poststreptococcal nephritis. Useful criteria distinguishing the two diseases are given. The diseases may present in a similar manner and therefore it is important to remember typhoid as a cause of glomerulonephritis in endemic areas or in patients travelling from endemic areas. 相似文献
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Three sibs all presented in the early neonatal period with a salt-losing syndrome. The salt-losing form of congenital adrenal hyperplasia was diagnosed and appropriate treatment with glucocorticosteroids, mineralocorticosteroids, and additional dietary salt started. Although early life was maintained with difficulty, with age all 3 children required decreasing amounts of replacement steroids to maintain normal plasma electrolyte balance. They were reinvestigated at the ages of 15 years and 8 years (twins), when cortisol synthesis and metabolism proved normal, but aldosterone synthesis was blocked by deficiency of 18-dehydrogenase. Rational treatment of these cases of a salt-losing syndrome in which aldosterone synthesis alone is blocked due to lack of the enzyme 18-dehydrogenase requires the administration of a mineralocorticosteroid drug only. Since deoxycorticosterone (acetate or pivalate) requires intramuscular administration, as life-long therapy oral fludrocortisone is preferable. Although fludrocortisone has glucocorticoid activity, the "hydrocortisone equivalent" effect of the small dosage used was unlikely to inhibit either pituitary corticotrophin or growth hormone production. 相似文献
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D T Leung R Y Tseng S H Go G L French C W Lam 《Archives of disease in childhood》1987,62(10):1075-1076
Of 74 paediatric inpatients with acute glomerulonephritis, 58 (78%) had a raised (greater than 1/200) antistreptolysin O titre. Serum C3 concentration was low in 73, but returned to normal within six weeks. Streptococcal infection remains the commonest cause of acute nephritis in children in Hong Kong, possibly due to overcrowded living conditions. 相似文献
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The clinical course and renal pathology of 17 children with acute extracapillary proliferative glomerulonephritis is reported. Patients with systemic diseases associated with renal involvement were excluded. The onset followed streptococcal infection in ten; of these, four have died, one has been transplanted, and the remaining five have completely healed. The mean follow-up of the latter five patients was 32 months (range, 18 to 57 months). Of the other seven patients without evidence of precedeing streptococcal infection, two have died, two have been transplanted, and the remaining three all have hypertension, proteinuria, and reduced creatinine clearance. The mean follow-up of the latter three patients was 29 months (range, 14 to 38 months). The initial renal histopathologic changes and their progress in later renal biopsies is described. The role of various therapeutic agents is discussed. The prognosis in acute extracapillary proliferative glomerulonephritis following streptococcal infection appears to be better than in ones without preceding streptococcal infection. 相似文献
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Three clinically normal sibs were discovered to have type II hyperprolinaemia in a routine serum amino acid screening programme in Sicily. In addition to the basic biochemical features of type II hyperprolinaemia, all 3 children had marked hyperglycinaemia, whereas their parents had both normal blood proline and glycine concentrations. Clinical normality in individuals with hyperprolinaemia may suggest that these two metabolic disorders (types I and II) are benign entities. Furthermore, the absence of clinical abnormality in these sibs, despite the presence of marked hyperprolinaemia and hyperglycinaemia, may suggest that neither of these findings alone causes brain damage. The hyperglycinaemia in these sibs is unexplained and is an unusual if not unique finding in association with hyperprolinaemia. 相似文献
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M D Walters C O Savage M J Dillon C M Lockwood T M Barratt 《Archives of disease in childhood》1988,63(7):814-817
Antineutrophil cytoplasm antibody (ANCA) has been reported in the sera of adults with Wegener''s granulomatosis and microscopic polyarteritis, but this phenomenon has not so far been described in children. We report three children with crescentic glomerulonephritis in whom ANCA concentrations were raised at presentation. Two cases were idiopathic, and the other later developed features of Wegener''s granulomatosis. In all three, plasma exchange and immunosuppression removed ANCA, but in only one case was there clinical improvement. This child later developed classical nasal lesions of Wegener''s granulomatosis associated with a decline in renal function and a rise in ANCA. Plasma exchange and immunosuppression again produced a good clinical response and removed ANCA. This suggests that ANCA is either a marker of disease activity, or is involved in the pathogenesis of disease. 相似文献