Body growth in primary de Toni-Debré-Fanconi syndrome
| Autor: | Hans-Joachim Bremer, Rüdiger Waldherr, Otto Mehls, Dieter Haffner, Achim Weinfurth, F Manz, Christoffer Seidel, Karl Schärer, H. Schmidt |
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| Rok vydání: | 1997 |
| Předmět: |
Male
medicine.medical_specialty Adolescent Kidney Function Tests Weight Gain Gastroenterology Internal medicine medicine Humans Hypercalciuria Magnesium Child Growth Disorders Acidosis Retrospective Studies business.industry Metabolic disorder Inulin Infant Metabolic acidosis Phosphorus medicine.disease Fanconi Syndrome Hypokalemia Body Height Bicarbonates Endocrinology El Niño Nephrology Child Preschool Pediatrics Perinatology and Child Health Potassium Calcium Female medicine.symptom business Hypophosphatemia Kidney disease |
| Zdroj: | Pediatric nephrology (Berlin, Germany). 11(1) |
| ISSN: | 0931-041X |
| Popis: | Body growth in nine children with primary de Toni-Debre-Fanconi syndrome was followed from birth to adolescence or adult life. At the time of diagnosis, corresponding to the start of treatment, the median age was 2.3 (range 0.4 – 13.9) years and height standard deviation score (SDS) was always decreased (median – 3.5, range – 6.8 to – 2.1). Despite continuous electrolyte and bicarbonate supplementation only four patients showed a slight improvement in growth. At the time of the last observation at the age of 17.2 (4.5 – 20.1) years median height was – 4.7 ( – 5.9 to – 1.8) SDS. The median difference between height at last observation and target height was – 4.5 SDS. Final height (n = 5) ranged between – 1.8 and – 5.5 (median –4.3) SDS. The pubertal growth spurt was absent in two children. Metabolic acidosis was identified as a significant growth-retarding factor. Mean serial blood bicarbonate levels and height SDS at the last observation were correlated (r = – 0.87, P |
| Databáze: | OpenAIRE |
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