ABSTRACT
BACKGROUND: Tubulointerstitial nephritis (TIN) is an uncommon condition in which the aetiology, treatment and outcome is not well defined. We describe a large series of children with biopsy-proven TIN. METHODS: All children with biopsy-proven TIN presenting to our institution during a 23-year period were retrospectively reviewed for aetiology, symptoms, treatment, and long-term outcome. RESULTS: A total of 27 children (16 girls) were described. Median age was 12 years (range 8 months to 15 years). A potentially adverse drug reaction was found in 12 (44 %) and infection in 8 (30 %). In 13 (48 %) no initiating factor was identified. All but 1 patient were treated with corticosteroids owing to worsening kidney function and 4 patients with other immunosuppressive agents. Fifteen children (56 %) had an estimated glomerular filtration rate (eGFR) of less than 80 ml/min/1.73 m(2) at last follow-up. Fifteen of the 23 children investigated (65 %) had coexistent uveitis. CONCLUSION: This series represents a subset of paediatric TIN patients in whom there was a clinical indication for a renal biopsy, hence presenting with more severe disease than previously reported. This group were more likely to have no identifiable underlying cause and an increased requirement for corticosteroid treatment. Furthermore, more than half of the cases developed chronic kidney disease (CKD) with impaired kidney function.
Subject(s)
Nephritis, Interstitial/diagnosis , Nephritis, Interstitial/therapy , Adolescent , Adrenal Cortex Hormones/therapeutic use , Biopsy , Child , Child, Preschool , Disease Progression , Drug-Related Side Effects and Adverse Reactions , Female , Follow-Up Studies , Glomerular Filtration Rate , Humans , Immunosuppressive Agents/therapeutic use , Infant , Infections/complications , Kidney Function Tests , Male , Nephritis, Interstitial/pathology , Proteinuria/etiology , Renal Insufficiency, Chronic/complications , Retrospective Studies , Treatment Outcome , Uveitis/etiologyABSTRACT
OBJECTIVE: To describe the presentation, management, and outcome of 43 cases of pneumococcal-associated hemolytic uremic syndrome (P-HUS). An increased incidence of P-HUS has been noted in the United Kingdom between January 1998 and May 2005. STUDY DESIGN: Cases with microangiopathic hemolytic anemia (Hb <10 g/dL with fragmented RBCs), thrombocytopenia (platelet count < 130 x 10(9)/L), acute renal impairment with oliguria and elevated plasma creatinine for age, confirmed or suspected pneumococcal infection and/or T-activation were included. RESULTS: The median age at presentation was 13 months (range, 5-39 months). Pneumococcus was identified in 34 of 43 cases; T-activation was identified in 36 of 37 cases. Twelve strains were serotyped: serotypes 3 (n = 2), 6A (n = 2), 12F (n = 1), 14 (n = 1), 19A (n = 6). Empyema was present in 23 of 35 pneumonia cases; 13 cases had confirmed (9) or suspected (4) pneumococcal meningitis; 36 cases required dialysis (median, 10 days; range, 2-240 days). The mortality rate was 11%, comprising 3 cases of meningitis, 1 case of sepsis and 1 case of pulmonary embolism at 8 months follow up while on dialysis. Follow-up data were available for 35 of 38 patients who survived (median follow-up period, 9 months; range, 1-63 months); of these, 10 patients had renal dysfunction, 1 patient was dialysis-dependent, 5 patients had hypertension and 8 patients had at least 1+ proteinuria on urinalysis. CONCLUSION: P-HUS has increased compared with historic surveys (0/288 in 1985-1988; 8/413 in 1997-2001, 43/315 in 1998-May 2005). Early mortality remains high (8-fold that of VTEC-induced HUS). Ten of 12 strains identified would not be covered by the PCV7 vaccine.