Long-term outcomes and associated prognostic risk factors of childhood-onset lupus nephritis.

Background: This study investigated the clinical characteristics and kidney outcomes of childhood-onset lupus nephritis (LN), and risk factors associated with prognosis. Methods: We enrolled 216 patients with histologically diagnosed LN during childhood. The Korean Society of Pediatric Nephrology organized a retrospective cohort study of childhood-onset LN in 13 major pediatric nephrology centers in South Korea. Results: The mean age at kidney biopsy was 13.2 ± 3.22 years. The main forms of presentation were nephrotic syndrome and/or hematuria in 152 patients (70.4%), and the most common histological finding was World Health Organization (WHO) class IV in 138 patients (63.9%), followed by WHO class III in 34 patients (15.7%). In the outcome analysis, the mean follow-up period of the patients was 7.8 ± 5.11 years. At last follow-up, 32 patients (14.8%) developed advanced chronic kidney disease (CKD). Male sex and failure to achieve remission at 12 months of treatment were significant risk factors for developing advanced CKD (hazard ratio of 2.57 and 2.29, respectively). Conclusion: Our study demonstrated the clinical characteristics and long-term outcomes of patients with childhood-onset LN. Male sex and failure to achieve remission in the first year of treatment were predictive of advanced CKD. Therefore, prompt awareness and close monitoring of these high-risk patients are needed, which may further improve the prognosis of children with LN.

Three cases of multicentric carpotarsal osteolysis syndrome: a case series.

BACKGROUND: Multicentric carpotarsal osteolysis syndrome (MCTO) is characterized by progressive destruction and disappearance of the carpal and tarsal bones associated with nephropathy. MCTO is caused by loss-of-function mutations in the MAF bZIP transcription factor B (MAFB) gene. CASE PRESENTATION: This report describes three unrelated patients with MAFB mutations, including two male and one female patient. Osteolytic lesions in the carpal and tarsal bones were detected at 2 years, 12 years, and 14 months of age, respectively. Associated proteinuria was noted at 4 years, 12 years, and 3 months of age, respectively. Kidney biopsy was performed in two of them and revealed focal segmental glomerulosclerosis (FSGS). One patient showed progression to end-stage renal disease, that is by 1 year after the detection of proteinuria. The second patient had persistent proteinuria but maintained normal renal function. In the third patient, who did not undergo a kidney biopsy, the proteinuria disappeared spontaneously. The bony lesions worsened progressively in all three patients. Mutational study of MAFB revealed three different mutations, two novel mutations [c.183C > A (p.Ser61Arg) and c.211C > G (p.Pro71Ala)] and one known mutation [c.212C > T (p.Pro71Leu)]. CONCLUSION: We report three cases with MCTO and two novel MAFB mutations. The renal phenotypes were different among the three patients, whereas progressive worsening of the bony lesions was common in all patients. We also confirmed FSGS to be an early renal pathologic finding in two cases. A diagnosis of MCTO should be considered in patients with progressive bone loss concentrated primarily in the carpal and tarsal bones and kidney involvement, such as proteinuria.

Hyponatremia may reflect severe inflammation in children with febrile urinary tract infection.

BACKGROUND: Hyponatremia is the most common electrolyte abnormality in clinical practice, but little is known about the association between febrile urinary tract infection (UTI) and hyponatremia or its significance to clinical outcomes. METHODS: Data from 140 children with febrile UTI between 2000 and 2010 were retrospectively analyzed. Laboratory examinations [white blood cell (WBC) count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serum sodium concentration], renal ultrasonography, (99m)Technetium-dimercaptosuccinic acid (DMSA) scintigraphy, and voiding cystourethrogram were performed. Culture growing >50,000 colonies of one single bacterial species on a urine sample obtained by catheter or >100,000 colonies on two clean-catch samples was required to establish diagnosis of UTI. RESULTS: In children with renal cortical defects diagnosed after DMSA scintigraphy (group 1), duration of fever was significantly longer (P = 0.038) and WBC (P = 0.047) and CRP (P < 0.0001) levels significantly higher than in those without renal cortical defects (group 2). However, serum sodium levels were significantly lower in group 1 than group 2 (135.9 ± 2.4 vs 137.4 ± 2.7 mEq/L, P = 0.007). Hyponatremia (serum sodium ≤ 135 mEq/L) was also more frequent in group 1 than in group 2 (74.1 % vs 45.3 %, P = 0.012). Serum sodium concentration was negatively correlated with WBC count (r = -0.156, P = 0.011) and CRP levels (r = -0.160, P= 0.028). CONCLUSIONS: Our study indicates that hyponatremia may be a substantial inflammatory marker and is significantly and independently associated with the degree of inflammation in children with febrile UTI.

Urinary bladder rupture during voiding cystourethrography.

Voiding cystourethrography (VCUG) is a commonly performed diagnostic procedure for the evaluation of vesicoureteral reflux with urinary tract infection or congenital renal diseases in children. The procedure is relatively simple and cost-effective, and complications are very rare. The iatrogenic complication of VCUG range from discomfort, urinary tract infection to bacteremia, as well as bladder rupture. Bladder rupture is a rare complication of VCUG, and only a few cases were reported. Bladder rupture among healthy children during VCUG is an especially uncommon event. Bladder rupture associated with VCUG is usually more common in chronically unused bladders like chronic renal failure. Presented is a case of bladder rupture that occurred during a VCUG in a healthy 9-month-old infant, due to instilled action of dye by high pressure. This injury completely healed after 7 days of operation, and it was confirmed with a postoperative cystography. The patient's bladder volume, underlying disease, velocity of the contrast media instilled, catheter size, and styles of instillation are important factors to prevent bladder rupture during VCUG. Management of bladder rupture should be individualized, but the majority of infants are treated with the operation. In conclusion, bladder rupture is a rare complication, however, delicate attention is needed in order to prevent more dire situations.