Atul Sharma

The use of pamidronate in three children with renal disease

Abstract. The successful use of pamidronate, a bisphosphonate, for the treatment of hypercalcemia and/or osteopenia is reported in three children with renal failure or following renal transplant. Patient 1 was an 11-year-old post renal transplant male who received a single dose of IV pamidronate (0.5u2005mg/kg) for the treatment of acute hypercalcemia associated with a pathological fracture and subsequent immobilization. Prompt resolution of the hypercalcemia was seen. He received a second course of pamidronate (0.5u2005mg/kg per day for 3 days) for the treatment of osteopenia and has had a subsequent 15% increase in lumbar spine bone mineral content (BMC). Patient 2, a 14-year-old male on peritoneal dialysis, presented with symptomatic hypercalcemia associated with tertiary hyperparathyroidism. A single dose of IV pamidronate (0.4u2005mg/kg) was given with prompt resolution and prolonged control of his hypercalcemia. The third patient was a 16-year-old female, also in renal failure on peritoneal dialysis. Her course had been complicated by marked osteopenia. IV pamidronate (0.5u2005mg/kg per dose) was given on 3 successive days before and after renal transplant in an attempt to stabilize her bone mineral density (BMD) around the time of renal transplantation, when additional glucocorticoid was necessary. Her total body BMC and BMD remained stable pre and post transplant. The treatment was effective and well tolerated in all three patients. Hence pamidronate is safe and effective for the management of hypercalcemia and osteopenia in children with renal failure and/or renal transplant.

Moderate renal failure in association with prolonged acquired hypothyroidism in children

We evaluated five children with prolonged primary hypothyroidism and noted a significant reduction in renal function (40%), which was reversible with hormonal replacement. This decline was higher than reported in adults and was of sufficient magnitude to warrant altering drug‐dosing schedules. Furthermore, patients with moderately reduced renal function should be carefully evaluated for signs and symptoms of hypothyroidism. □Glomerular filtration rate, primary hypothyroidism, renal insufficiency

Osteopenia and fractures in cystinotic children post renal transplantation

Abstract.Many of the end-organ effects of cystinosis are known to be risk factors for osteopenia; these include deposition of cystine crystals in bone, hypothyroidism, diabetes mellitus, primary hypogonadism, urinary phosphate wasting, and chronic renal failure. While transplantation may correct the latter, it exposes the child to other risk factors for diminished bone mass, notably the use of high-dose glucocorticoids. Our objective was to determine if these multiple risk factors translate into an increased occurrence of osteopenia, as measured by dual-energy X-ray absorptiometry (DEXA), and/or fractures in this population. We examined the charts, X-rays, and bone mineral density (BMD) of all cystinotic patients post renal transplant for whom this information was available. Lumbar spine BMD was measured by DEXA scan (Hologic 4500). Z-scores were corrected for growth parameters using previously published reference data. Fracture history and pertinent serum markers of bone metabolism were also analyzed. Of the 63 renal transplants performed at our institution, 11 children were transplanted due to cystinosis. Nine of these patients, 5 male and 4 female, had had BMD evaluations, with an average age of 14.3xa0years (range 5–17xa0years) at the time of initial BMD post transplant. The mean interval between transplant and BMD evaluation was 39xa0months (range 3–90xa0months). Surprisingly, 7 of 9 patients had normal uncorrected BMD values (z-scores −1.92 to +0.02) and 7 of 9 patients had normal corrected values (z-scores –1.20 to +1.93). Three patients suffered from a total of eight fractures. Of the 3 fracture patients, 2 had normal BMD. All patients maintained good graft function and had normal calcium/phosphate mineral status. Of note, 3 of 5 male patients had evidence of primary testicular failure at earlier ages than often described, and this may be an unrecognized risk factor for bone disease in this population. Despite the numerous risk factors for developing osteopenia, these results suggest that the majority of cystinotic patients post renal transplant do not experience reduced bone mineral content as measured by DEXA. However, the significant fracture history among these patients demonstrates that DEXA cannot be used to assess fracture risk in patients with nephropathic cystinosis.

Systemic juvenile rheumatoid arthritis complicated by two different renal lesions

Abstract. Systemic-onset juvenile rheumatoid arthritis (JRA) is a complex disease which affects many organ systems. Associated renal lesions are unusual, with the possible exception of amyloidosis. We describe a girl with systemic-onset JRA who developed first membranous nephropathy and then, 3.5 years later, a severe crescentic glomerulonephritis. The membranous lesion followed therapy with intravenous immune globulin, and the possibility that this intervention caused the renal disease must be considered. It appears that both of these lesions should be added to the list of possible complications of systemic-onset JRA.

Reassessing hemodialysis adequacy in children : the case for more

Abstractu2002Several lines of evidence support an upward revision in pediatric hemodialysis dose guidelines: Although current recommendations are derived largely from studies of dialysis mortality and morbidity in adults, recent reports of improved growth and pubertal development with more intensive dialysis highlight the need for appropriate pediatric outcome measures in the assessment of dialysis adequacy, particularly in prepubertal patients. Even if adult studies can be extrapolated directly to younger patients, reappraisal of these data would appear to justify an increase in recommended dialysis clearances, based on higher dietary protein intake and accumulating evidence that adults, too, benefit from more intensive therapy. Suboptimal dialysis may also occur when dialysis dose is overestimated by urea kinetic models that fail to account for compartment effects and post-treatment urea rebound. Studies comparing the available models in pediatric patients have appeared recently, and a few models have been developed specifically for pediatric applications. These should permit more reliable estimates of solute clearance for a much-needed multicenter trial to clarify optimal dialysis therapy for growing children.

Infusion clearance of subcutaneous iothalamate versus standard renal clearance

Abstractu2003Accurate, timed urine collections for the measurement of glomerular filtration rate (GFR) may be impractical in infants or in patients with urological abnormalities. GFR may be measured without urine collection using a constant subcutaneous infusion of iothalamate. We compare the infusion clearance with conventional renal clearance in 14 children and young adults. The mean clearance ratio (infusion clearance/renal clearance ± 1 SD) was 0.99±0.1 and the mean discrepancy between the two methods was 8.5%±4.7%. The 95% limits of agreement for the ratio of the two methods are 0.83–1.23. These data indicate that subcutaneous infusion of iothalamate is a practical method for measuring GFR in children without a urine collection.

Programme to provide Quebec infants with free vitamin D supplements failed to encourage participation or adherence

The aim of this study was to evaluate the uptake of a free vitamin D infant prescription programme and to determine the incidence of nutritional rickets.

Impact of Vitamin D Supplementation on Gross Motor Development of Healthy Term Infants: A Randomized Dose-Response Trial

ABSTRACT In addition to benefits for bone health, vitamin D is implicated in muscle function in children and adults. Aims: To determine if vitamin D dosage positively correlated with gross motor development at 3 and 6 months of age. We hypothesized that higher doses would be associated with higher scores for gross motor skills. Methods: A consecutive sample of 55 healthy, term, and breastfed infants from Montreal, Canada were recruited from a randomized trial of vitamin D supplementation between 2009 and 2012. Infants were randomized to 400 International Units (IU) (n = 19), 800 IU (n = 18) or 1,200 IU (n = 18) vitamin D3/day. Motor performance at 3 and 6 months was quantified by the Alberta Infant Motor Scale (AIMS). Plasma vitamin D3 metabolites were measured by tandem mass spectrometry. Results: AIMS scores did not differ at 3 months. However, total AIMS scores and sitting subscores were significantly higher at 6 months in infants receiving 400 IU/day compared to 800 IU/day and 1,200 IU/day groups (p < .05). There were weak negative correlations with length and C-3 epimer of 25(OH)D. Conclusions: In contrast to our hypothesis, gross motor achievements were significantly higher in infants receiving 400 IU/day vitamin D. Our findings also support longer infants being slightly delayed.