An 11-month-old with anti-glomerular basement membrane disease

Bigler, Steven A.; Parry, Wilson M.; Fitzwater, Douglas S.; Baliga, Radhakrishna

doi:10.1016/s0272-6386(97)90497-2

Cited by 13 publications

(16 citation statements)

References 6 publications

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“…There is also a strong HLA association, with over 80% of individuals carrying HLA DR15 or DR4 alleles [12]. A number of case reports have described the development of anti-GBM disease in adult patients following lithotripsy, suggesting that antigen release or exposure from damage to the kidney may initiate anti-GBM disease in susceptible individuals [8, 9, 13, 14, 15, 16, 17, 18, 19]. In these cases, the affected individuals presented between 3 and 12 months after lithotripsy.…”

Section: Discussionmentioning

confidence: 99%

Anti-GBM disease after nephrectomy for xanthogranulomatous pyelonephritis in a patient expressing HLA DR15 major histocompatibility antigens: a case report

O’Hagan¹,

Mallett²,

Convery³

et al. 2015

CNCS

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Antiglomerular basement membrane (anti-GBM) antibody disease is uncommon in the pediatric population. There are no cases in the literature describing the development of anti-GBM disease following XGP or nephrectomy. We report the case of a 7-year-old boy with no past history of urological illness, treated with antimicrobials and nephrectomy for diffuse, unilateral xanthogranulomatous pyelonephritis (XGP). Renal function and ultrasound scan of the contralateral kidney postoperatively were normal. Three months later, the child represented in acute renal failure with rapidly progressive glomerulonephritis requiring hemodialysis. Renal biopsy showed severe crescentic glomerulonephritis with 95% of glomeruli demonstrating circumferential cellular crescents. Strong linear IgG staining of the glomerular basement membranes was present, in keeping with anti-GBM disease. Circulating anti-GBM antibodies were positive. Treatment with plasma exchange, methylprednisolone, and cyclophosphamide led to normalization of anti-GBM antibody titers. Frequency of hemodialysis was reduced as renal function improved, and he is currently independent of dialysis with estimated glomerular filtration rate 20.7 mls/min/1.73 m2. Case studies in the adult literature have reported the development of a rapidly progressive anti-GBM antibody-induced glomerulonephritis following renal surgery where patients expressed HLA DR2/HLA DR15 major histocompatibility (MHC) antigens. Of note, our patient also expresses the HLA DR15 MHC antigen.

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Section: Discussionmentioning

confidence: 99%

Anti-GBM disease after nephrectomy for xanthogranulomatous pyelonephritis in a patient expressing HLA DR15 major histocompatibility antigens: a case report

O’Hagan¹,

Mallett²,

Convery³

et al. 2015

CNCS

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“…Some authors have proposed that structural differences in the GBM exist in childhood, which transition to the adult type over ages 3 months to 3 years, contributing to the exceedingly rare diagnosis of anti-GBM disease in younger children [5]. However, patients have been reported in the literature aged from 11 months to 5 years [6][7][8]. The youngest reported patient (11 months) presented with acute kidney injury, requiring plasmapheresis and progressing to end-stage renal disease (ESRD) and ultimately renal transplantation [6].…”

Section: Introductionmentioning

confidence: 99%

“…However, patients have been reported in the literature aged from 11 months to 5 years [6][7][8]. The youngest reported patient (11 months) presented with acute kidney injury, requiring plasmapheresis and progressing to end-stage renal disease (ESRD) and ultimately renal transplantation [6]. The U.S. Renal Data System (USRDS) reports that 0.4% (24/6,560) ESRD cases in children (2002)(2003)(2004)(2005)(2006) were attributable to Goodpasture syndrome, presumably anti-GBM disease, as other causes of pulmonary-renal syndrome are listed separately [9].…”

Section: Introductionmentioning

confidence: 99%

A 25-year experience with pediatric anti-glomerular basement membrane disease

et al. 2010

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Anti-glomerular basement membrane (anti-GBM) disease, which is extremely uncommon in children, is characterized by rapidly progressive glomerulonephritis (RPGN) and autoantibodies against GBM collagen. Pulmonary hemorrhage is the third component in Goodpasture Syndrome. Cigarette smoking and exposure to hydrocarbons have been linked to anti-GBM disease in adults, but such an association has not been established in children. We reviewed renal biopsy and autopsy specimens over 25 years from a major tertiary care U.S. children's hospital, diagnosing anti-GBM by clinical RPGN, crescentic glomerulonephritis, and linear immunofluorescence (IF) immunoglobulin G staining in patients under 18 years of age. We identified four patients, with and without pulmonary manifestations. The sole autopsy case showed diagnostic IF despite undetectable serum anti-GBM antibodies and positive testing for serum anti-neutrophil cytoplasmic antibodies (ANCA). Three patients have survived 1-18 years following diagnosis, one of whom is recovering renal function. One adolescent had a history of smoking cigarettes and one had a probable hydrocarbon exposure. Anti-GBM disease is unusual in children, and the relationship to inhaled agents is incompletely understood. Serum anti-GBM antibodies are typically present, but cases with undetectable levels can occur. Some patients are anti-GBM and ANCA positive, with a small subset ANCA-positive, anti-GBM-negative. Ours is the first such described pediatric case.

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“…GPS is rare in children, and usually occurs from ages 6-13. 4 , although the youngest reported case was 11 months old 5 . Onestudy of GPS in children reported a male to female ratio of 0.43, with positive anti-GBM antibody levels in all patients who were tested 4 .…”

Section: Discussionmentioning

confidence: 99%