Search

Background: Improved management of growth impairment might have resulted in less growth retardation after pediatric kidney transplantation (KT) over time. We aimed to analyze recent longitudinal growth data after KT in comparison to previous eras, its determinants, and the association with transplant outcome in a large cohort of transplanted children using data from the European Society for Paediatric Nephrology/European Renal Association and European Dialysis and Transplant Association Registry., Methods: A total of 3492 patients transplanted before 18 years from 1990 to 2012 were included. Height SD scores (SDS) were calculated using recent national or European growth charts. We used generalized equation models to estimate the prevalence of growth deficit and linear mixed models to calculate adjusted mean height SDS., Results: Mean adjusted height post-KT was -1.77 SDS. Height SDS was within normal range in 55%, whereas 28% showed moderate, and 17% severe growth deficit. Girls were significantly shorter than boys, but catch-up growth by 5 years post-KT was observed in both boys and girls. Children <6 years were shortest at KT and showed the greatest increase in height, whereas there was no catch-up growth in children transplanted >12., Conclusions: Catch-up growth post-KT remains limited, height SDS did not improve over time, resulting in short stature in nearly half of transplanted children in Europe.

Background: Cystinosis is an autosomal recessive disorder characterized by intralysosomal cystine accumulation. Growth retardation is more pronounced in cystinosis than in other chronic kidney diseases and is mostly not corrected by cysteamine., Methods: Growth was evaluated in nine cystinosis patients, all treated with cysteamine, both after cysteamine and recombinant human growth hormone (rhGH) therapy initiation. Growth hormone (GH) secretion was studied by nocturnal GH measurements in four of nine patients and by glucagon test in four of nine patients., Results: RhGH was administered to seven of nine patients. At rhGH initiation, height was below -2 SDS in five of seven patients, final height was above -2 SDS in six of seven. In two patients not treated with rhGH, final height remained below -4 SDS despite cysteamine treatment being started at the age of 6.1 and 8.1 years, respectively. Nocturnal GH secretion was normal in all patients. Glucagon tests revealed GH deficiency in one patient; two of four patients had abnormal GH peak timing., Conclusions: We present the first reported case of GH deficiency in cystinosis. Although no overt GH deficiency was detected in other patients, abnormal GH peak timing can indicate a subclinical GH secretion problem. RhGH significantly improved growth in cystinosis patients and should be initiated early in life.

Aims: Chronic renal failure in childhood jeopardizes both growth and development. In children with chronic renal failure from birth, growth in height and weight have been found to be generally poor. Few data on head circumference are available., Material: A cohort of 19 children with chronic renal failure from birth was studied from the early weeks of life to the age of 5 years. There were 18 boys; and 18 patients had congenital renal hypoplasia or hypodysplasia associated with obstructive uropathies. Eight patients received recombinant growth hormone (rhGH) after the age of 2 years. Only 2 patients needed renal replacement therapy before the age of 5 years. Data after transplantation were not included., Methods: The following variables were analyzed: body height, body mass index and head circumference. Data were expressed in median values of standard deviations scores (SDS)., Results: In the first 3 months of life there was a significant drop in height SDS, body mass index SDS and head circumference SDS. Thereafter, a stable growth velocity was observed for the rest of the study period, except for body mass index SDS. which improved after 36 months. There was a striking difference between patients who needed treatment or not with recombinant human growth hormone (rhGH). Patients without rhGH displayed a stable growth after the age of 3 months until 5 years of age. In the remaining 8 patients, rhGH treatment resulted in a significant increase not only in height SDS but also in head circumference SDS., Conclusions: In infants and young children with chronic renal failure from birth, growth in head circumference parallels growth in body height. This applies to all patients and to data before and during rhGH treatment.

We retrospectively analyzed the effects of recombinant human growth hormone (rhGH) in a Belgian population of 36 short children with renal allografts. Seven children were dropped from the growth study: 1 had skeletal dysplasia and in 6 cases rhGH was given for less than 1 yr (1 died, 1 developed genu valgum, 2 were non-compliant and 2 grafts deteriorated). Final height was reached in 17 patients, and 12 children were still growing at the end of the study. Median height standard deviation score (SDS) in the 29 patients was -2.3 at the time of transplantation, and -2.7 when rhGH therapy was initiated. During rhGH therapy (median duration 3.2 yr, range 0.6-7.7 yr), height SDS increased by a mean of 0.4 per year, and bone maturation was not accelerated. Final height reached was 162.7 (149.0-169.5) cm (median SDS -1.8) in males and 151.0 (130.5-169.5) cm (median SDS -1.9) in females. Final height is significantly greater in males than females compared with a historical control group of untreated patients. Final height is within the parental target height range in 6 out of the 17 patients. The increase in height SDS in patients who were at an advanced stage of puberty (Tanner stages 4-5) when rhGH therapy was initiated exceeded our expectations (mean height gain 14.2 cm in boys and 10 cm in girls). In the cohort of 36 children, 4 patients developed an acute allograft rejection, all of whom had an underlying chronic rejection. This resulted in 3 graft losses within 5 yr. Our results indicate that rhGH treatment has a positive effect in short children with renal allografts, even if it is started in late puberty. In the presence of underlying chronic rejection, rhGH treatment needs careful monitoring to minimize the risk of graft loss.

Unlabelled: Twenty infants with chronic renal failure from the first weeks of life, received strict conservative treatment consisting of a protein-restricted, calorie-enriched diet, supplements of essential amino acids, sodium chloride, sodium bicarbonate, calcium and vitamin D. The last 10 patients also received erythropoietin. Neither nasogastric nor gastrostomy tubes were used in any of these patients. Four patients needed dialysis in the second half of the 1st year of life. We analysed the patients' growth in weight, height and head circumference from birth until the age of 12 months. At the age of 12 months, mean values of height, weight and head circumference SDS were -1.63, -1.53 and -1.01 respectively as compared to healthy children. The body length data also compare favourably with those from a large cohort of chronic renal failure patients collected by the European Study Group for Nutritional Treatment of Chronic Renal Failure in Childhood; here the mean height SDS at 12 months of age is -3.3.Conclusion: This retrospective analysis shows that the generally observed progressive growth retardation in infants with chronic renal failure can be prevented by early and adequate conservative management. [ABSTRACT FROM AUTHOR]