Short Stature in Renal Disease
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Pediat. Res. 7: 978-982 (1973) A Review: Short Stature in Renal Disease GUNNAR B. STICKLER1141 AND BARRY J. BERGEN Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA Introduction and of CO2 content as a measure of acid-base buffer. Urea in blood is not as precise a measure of renal Factors contributing to short stature in children with function as is serum creatinine or creatinine clearance, renal disease have been investigated by several groups and serum pH is a more reliable indicator of the acid- [1, 2, 10, 13]. Acidosis and chronic malnutrition were base balance than is the CO content. However, these believed to be among the most important causes of the 2 less reliable indicators were the only ones available in growth failure. A critical discussion of the contribut- retrospect. ing causes has been presented by Bergstrom and co- The alkaline phosphatase is uniformly recorded in workers [1], international units per liter, yet until 1956 it was ex- Previous studies, while mentioning clinically evident pressed in Bodansky units (the correction factor to renal rickets as a cause of growth failure, failed to note international units per liter is 17), and from 1956-1966, the possibility that subtle impairment of bone metabo- in King-Armstrong units (correction factor to interna- lism also may be an important factor which contri- tional units per liter is 7.08). butes to growth failure in renal disease. The heights of the patients were compared with nor- We wish to point out that renal rickets may be the mal values for height established by Reed and Stuart most important factor for short stature in renal dis- [8]. Deviations from normal were expressed as stand- ease. ard deviation from the mean for age and sex. Children were considered to have rickets if they had Our Series positive roentgenographic evidence in addition to ele- A search was made of the records of all patients who vated alkaline phosphatase values. were less than 15 years old when they were first seen at the Mayo Clinic between 1950 and 1968 and who ful- Results filled the following criteria: (1) history of renal disease of a minimum of 2 years' duration; (2) a blood urea Total Group value of more than 50 mg/100 ml; (3) disease processes Of the 64 patients who met the criteria for inclusion classified as cystinosis, bilateral hydronephrosis, ne- in the study, those with cystinosis were the shortest phrotic syndrome, and renal tubular dysfunction (not and those with chronic glomerulonephritis were the treated with steroids), familial renal disease, chronic tallest (Table I). There were patients with very short glomerulonephritis, glomerulonephritis with anaphy- stature in all disease categories but that of chronic lactoid purpura, and renal failure of unknown cause. glomerulonephritis. No cases were excluded from the study. The following The patients were separated into two groups accord- patient data were obtained at the time of their last ing to height: those whose heights were within 3 SD of examination: (1) height and weight, (2) blood pressure, the mean and those whose heights were 3 SD below the (3) hemoglobin level, (4) urea value, (5) CO2 content, mean. The first group represented normal growth (6) alkaline phosphatase level, (7) specific gravity of whereas the second represented profound growth fail- urine (first morning voided specimen), and (8) serum ure. No differences were found between children with inorganic phosphorus. profound growth failure and those with normal This retrospective study was limited by the use of growth (Table II) in regard to (1) blood pressure, (2) levels of urea in blood as a measure of renal function presence or absence of anemia, (3) azotemia, (4) sever- 978 Short stature in renal disease 979 Table I. Short stature in renal diseases whereas patients with profound growth failure who No. of patients with height had such evidence were as young as 1.5 years of age. Six patients who were short had no roentgenographic Between More Total Disease Within 2 and 3 than 3 2 SD of SD below SD below evidence of rickets. Figure 1 shows the relation be- mean mean tween age and height in roentgenographically proven Cystinosis 0 2 3 5 rickets. The difference in the severity of growth failure Hydronephrosis 4 8 3 15 between children with rickets and those without rick- Nephrotic syndrome (long 2 0 4 6 ets was significant (P < 0.01). Furthermore, there was duration) 1 evidence that younger children with renal insufficiency, Renal failure (cause unknown) 9 3 8 20 tended to be shorter than older children with the same Familial renal disease 4 1 3 82 Chronic glomerulonephritis 9 1 0 103 degree of disease. and anaphylactoid purpura Among the patients with roentgenographic evidence 1 of rickets, two had genu varum and nine had genu Includes 3 patients with hypoplastic kidneys, 5 with end-stage valgum. These probably are minimal numbers because kidney (type unknown), and 12 with probable chronic pyelo- nephritis. more subtle deformities may not have been noted on 2 Includes one patient with Alport's syndrome, one with oxalo- clinical examination. sis, one with familial nephrosis, one with Lowe's syndrome, and There was no relation between the level of inor- four with juvenile nephronophthisis. 3 ganic phosphorus and either patient age or the pres- Proved by biopsy (five patients), autopsy (three patients), ence or absence of rickets. Children who had a very and clinical examination (two patients). short stature had phosphorus values ranging from Table II. Comparison of patients with normal or short stature 2.1-12 mg/100 ml in various age groups, regardless of in renal disease whether or not they had rickets. Also there was no Patients with height Patients with height relation between the level of phosphorus and short Factor within 3 SD of mean more than 3 SD below (normal stature) mean (short stature) stature. We utilized West and Smith's [13] formula (in Systolic blood pres- 140.6 ± 39.2 116.6 ± 15.6 which chronic renal failure is assumed to be a cause of sure, mm Hg significant anorexia and failure to gain weight) to de- Diastolic blood pres- 96.9 ± 32.1 76.0 ± 19.0 sure, mm Hg termine the nutritional status of the children in our Hemoglobin, g/100 9.4 ± 2.6 9.0 ± 2.2 ml Table III. Height in relation to roentgenographic evidence of Blood urea, mg/100 175.9 ± 86.6 193.9 ± 119.08 rickets ml With rickets CO content, mEq/ 15.9 ± 4.8 12.6 zfc 3.9 Height No. of 2 patients ^ liter T Specific gravity of 1,008.4 ±3.9 1,007.3 ±3.7 Within 2 SD below mean (normal) urine 43 3 7 1 Between 2 and 3 SD below mean Alkaline phosphatase, 252.9 ± 171.U 530.2 ± 341.6 15 4 27 More than 3 SD below mean IU/liter 21 15 71 1 Statistically significant difference: P ~ 0.01. .-Without rickets (X-ray) x-With rickets(X-ray) ity of acidosis, and (5) concentrating ability of the 1 • • • kidney. Most patients in each group had specific gravi- • 0 X • Without rickets • • • ties below 1.010, and all patients had specific gravities - • • • • below 1.018. The only statistically significant differ- -2 •x With rickets ""7 X -—"— • x X x _______ • • • • • ence between the two groups was in the increase of ~~~% •• • • .—-—x* o+ X X • —-—• —^F^ -4 ___——- — X alkaline phosphatase (Table II). The mean values for X X__ X • X X blood pressure tended to be higher in the normal-sized X -6 X X children, but this was not statistically significant. 1 1 * 1 t 1 I 1 I 1 1 1 1 1 1 i i i i i " Seven patients whose heights were within 3 SD of the mean had roentgenographic evidence of renal rickets Age,years on the basis of hand and wrist roentgenograms (Table Fig. 1. Comparison of height and age of children who had III), and all seven were more than 10 years old, renal disease with or without rickets. STICKLER AND BERGEN Table IV. Relation among clinical renal rickets, short stature, sigmoid are partially reabsorbed, thus resulting in ac- and weight for height index (WHI) cumulation of hydrogen ions and the production of Cases acidosis. Three of the five such patients in our series Clinical rickets Short stature Above 0.95 Below 0.95 Total 1 had, for part of their observation period, been treated WHI WHI with sodium bicarbonate, and during other periods, no No No 24 7 17 (71) treatment was prescribed. To the best of our knowl- Yes No 3 0 3 edge, the diet was not changed. Comparison of growth No Yes2 15 6 9 (60) 2 during the periods of sodium bicarbonate therapy and Yes Yes 16 7 9 (56) the periods without it showed that the growth rates Total 58 20 38 did not differ regardless of therapy. All five patients had heights within normal limits, and none had renal 1 Percentage is shown within parentheses. failure or renal rickets. 'More than 2 SD below mean. Prader et al. [7] described a patient who had a ure- series: weight for height index (WHI) = (actual terosigmoidostomy but failed to grow. He was acidotic weight/ideal weight for 50th percentile of height age). at that time and had rickets. Alkali therapy was asso- In this formula, a normal child has a WHI of 1. West ciated with catch-up growth and the healing of rickets. and Smith [13] considered that the index was abnor- No statement was made whether the patient received mally low if it was under 0.95 and considered that vitamin D in addition to alkali.