Survival of Tunneled Double Lumen-Cuffed Catheters in Children on Maintenance Hemodialysis - A Retrospective Cohort Study.

Introduction: Survival of tunneled cuffed catheters (TCC), used widely in children, is complicated by infections and catheter dysfunction. In resource limited settings, where risk of complications could be higher and waiting period for transplantation longer, catheter survival determines patient survival. This study was conducted to determine infection free catheter survival rates, incidence of catheter failure and associated risk factors. Methods: Children <18 years of age receiving maintenance hemodialysis through TCC at nephrology division of a pediatric hospital, over a period of 6 years. Data was collected with consecutive selection by a complete enumeration technique from pre-collected data sheets in the records. Exposure detected were catheter infections, thrombosis, and mechanical complications. Results: Forty-five TCCs in 36 children studied for 12,590 catheter days showed catheter failure in 36%, due to catheter related infections in 75% and mechanical complications in 25%. The incidence of complications per 1000 catheter days was 1.19 infection, 1.03 thrombus, and 0.39 mechanical. Catheter-related blood stream infection (CRBSI) (15/36) was associated with thrombus in nine and led to mortality in three. The mean infection free catheter survival was 449 ± 42 days for cohort with 388 ± 38 days in Group A (premature catheter removal) and 593 ± 43 days in Group B (elective removal) (P = 0.03). Catheterization duration of 267 days predicted CRBSI (sensitivity 93%, specificity 66.7%) with area under the curve of 0.808. Conclusions: Median infection free catheter survival was 449 days with catheter failure in 36%. CRBSI was the main cause of failure. Duration of catheterization greater than 267 days was a predictor of CRBSI.

Role of Cinacalcet in Treating Cardiac Dysfunction Secondary to Hyperparathyroidism: A Case Series.

Cardiovascular disease is a leading cause of death in children with chronic kidney disease (CKD). A strong correlation exists between disturbed calcium-phosphate metabolism and cardiac dysfunction. Studies with use of cinacalcet in CKD are few and limited to older children and adults and in improving growth and bone deformities. We present three children with CKD on CAPD with cardiac dysfunction with refractory hyperparathyroidism. Patients were initiated on lowest adult weight-adjusted dose of 0.2 mg/kg/day. Dose was titrated every 30 days to achieve decline in iPTH to a goal of 200- 300 pg/ml. Serum calcium, phosphorus and iPTH levels were checked monthly. Complications of therapy related to cinacalcet monitored. Monthly echocardiography done to monitor cardiac dysfunction. None of them experienced significant adverse effects of cinacalcet therapy.

Intestinal Pseudo-Obstruction - An Under-Recognized Presentation of Systemic Lupus Erythematosus.

Intestinal pseudo-obstruction (IPO), characterized by obstruction without an identifiable anatomic cause, is a rare and poorly understood entity that may occur as a primary condition or secondary to other autoimmune disorders such as systemic lupus erythematosus (SLE). A12-year-old female child was brought with abdominal distension, vomiting, and fever for 15 days. Examination showed height and weight less than the third centile for age, tachypnea, tachycardia, and hypertension with severe abdominal distension, copious bilious aspirate, and very sluggish bowel sounds. Abdominal X-ray showed multiple air fluid levels. Ultrasound abdomen and unenhanced computed tomography (CT) scan revealed thickened dilated bowel loops, ascites, and pleural effusion. In view of multisystem nature of the disease, Koch's abdomen or autoimmune disease was suspected and emergency laparotomy procedure was deferred. She was evaluated and diagnosed to have SLE with lupus nephritis class V as per the International Society of Nephrology/Royal Pathology Society. She was managed conservatively with nasogastric decompression, immunosuppressive therapy and supportive hemodialysis.

Multicystic dysplastic kidney: a retrospective study.

OBJECTIVE: To report the renal structural and functional anomalies in children with multicystic dysplastic kidneys. METHODS: Retrospective descriptive analysis of 47 children with multicystic dysplastic kidney seen in a pediatric nephrology unit over a period of 6 years. RESULTS: Antenatal diagnosis of multicystic dysplastic kidney was made in 34 (72.3%) patients. On follow up of 31 children for more than 12 months, 21 (68%) had involution, 4 [13%] had non-regression, and 4 (13%) were nephrectomized. Vesico-ureteric reflux (n=13; 28%) was the commonest renal abnormality. The serum creatinine values were higher (P=0.006) in children with contralateral reflux. Sub-nephrotic proteinuria was noted in 9 (29%) and was significantly associated with complete involution (P=<0.023). None of the patients developed hypertension and 2 (6.4%) had renal failure. CONCLUSIONS: Close nephrological follow-up is needed in children with multicystic dysplasia of kidneys.

Efficacy of levamisole in children with frequently relapsing and steroid-dependent nephrotic syndrome.

OBJECTIVE: To assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome. STUDY DESIGN: Retrospective analysis of hospital case records. SETTING: Pediatric nephrology department of a tertiary referral pediatric hospital. PARTICIPANTS: 62 children with frequently relapsing nephrotic syndrome and 35 children with steroid-dependent nephrotic syndrome. METHODS: Case records of children who were diagnosed as steroid-dependant or frequently-relapsing nephrotic syndrome from June 2004 to June 2011, were reviewed. Levamisole was given daily (2 mg/kg/d) along with tapering doses of alternate day steroids after remission on daily steroids. RESULTS: Levamisole was effective in 77.3% children with a better (80.6%) efficacy in frequently relapsing nephrotic syndrome. A total of 34 children completed 1 year follow-up post levamisole therapy. The cumulative mean (SD) steroid dose 1-year before therapy was 4109(1154) mg/m2 and 1-year post therapy was 661 (11) mg/m2 (P<0.001). The relapses were also less during the period of post-levamisole therapy. CONCLUSION: Levamisole is an effective alternative therapy in frequently relapsing and steroid-dependent nephrotic syndrome.

Chronic myeloid leukemia in a child with IgA nephropathy.

We report an 11 year old boy with IgA nephropathy developing chronic myeloid leukemia on follow-up. This association suggests that a B cell defect might be involved in the pathogenesis of these two conditions.

L-carnitine in beta thalassemia.

This study was conducted to determine L-carnitine levels in regularly transfused and chelated beta thalassemia patients (n=40; mean age, 17.5+/-5.0 years). Ten age matched controls were also studied. The mean L-carnitine level in thalassemic patients was 23.71+/- 7.3 microM as compared to control 29.26+/-2.37 microM (P<0.0001). Mean carnitine was significantly lower (P=0.037) in those with ferritin greater than 2000 ng/dL (22.80+/-6.97 microM) in comparison to those with ferritin less than 2000 ng/dL (30.1+/-7.77 microM). Although carnitine levels in non vegetarians was higher (26.91+/-8.4 microM) than in vegetarians (22.34+/-6.55 microM), this difference was not statistically significant (P=0.072). We conclude that L-carnitine levels were found to be lower in thalassemics as compared to age matched controls.

Evaluation of osteopathy in thalassemia by bone mineral densitometry and biochemical indices.

OBJECTIVE: To evaluate osteopathy in thalassemia by bone mineral densitometry (BMD) and biochemical indices. METHODS: Prospective review analysis with no follow up from 2006 to 2007 of 42 regularly transfused thalassemics aged 10-25 years (27 boys, 15 girls) was done. Anthropometry, pubertal stage and symptomatology were noted. Urinary C-terminal cross-linked telopeptide of type-1 collagen (Crosslaps) by ELISA; serum 25-OH vitamin D and osteocalcin by RIA; parathyroid hormone (PTH) and ferritin by chemiluminescence and IGF-1 by Enzyme immunoassay were evaluated. Dual Energy X-ray Absorptiometry (DEXA) of lumbar spine and femur was done on Lunar prodigy system. Data was entered and analyzed using the SPSS for Windows software. Mean comparisons were done by ANOVA 1 and data was compared using Chi-square test and p value < 0.05 was taken as significant. RESULTS: Of 42 patients, 81% had osteoporosis by Z-score of DEXA. Urinary crosslaps was high in 55%; 36% had increased osteocalcin; 62% had low vitamin D levels; 38% had high parathyroid levels and IGF-1 was low in 52%. Mean serum ferritin level was 5344 ± 2855 ng/dl. There was statistical significance (p = 0.046) between chronological age and BMD. All 42 cases were divided into two groups: Group-1 (Normal DEXA), Group-2 (Abnormal DEXA) and analysis of biochemical indices between two groups showed no significant difference in any of the biochemical parameters. CONCLUSION: This study revealed majority of thalassemics with inadequate chelation have bone resorption with advancing chronological age and BMD should be evaluated regularly for early diagnosis to prevent morbidity.