Urinary Citrate Excretion in Children With Hypercalciuria

Urinary Citrate Excretion in Children With Hypercalciuria

PEDIATRIC UROLOGY weeks of gestation. It is of major interest that the abdominal wall defect in fetuses with the prune belly syndrome is reversible i...

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PEDIATRIC UROLOGY

weeks of gestation. It is of major interest that the abdominal wall defect in fetuses with the prune belly syndrome is reversible if fetal bladder drainage is instituted relatively early in pregnancy. L. R. K.

Urinary Citrate Excretion in Children With Hypercalciu.ria

tients in whom constipation and other gastrointestinal symptoms are exceptionally severe. W. J. C. 12 references

Virilizing Adrenocortical Tumors in Childhood: Eight Cases and a Review of the Literature P. D. K. LEE, R. J. WINTER AND 0. C. GREEN, Division of

Endocrinology, Department of Pediatrics, Children's Memorial Hospital and Northwestern University Medical School, Chicago, Illinois

L. A. MILLER AND F. B. STAPLETON, Pediatric Research Laboratory, Memphis, Tennessee

J. Ped., 107: 263-266 (Aug.) 1985 The pathogenesis of calcium-containing urinary stones involves an imbalance between promoting and inhibiting factors of calcium crystal growth and aggregation in the urine. Urinary citrate, in particular, is important in the inhibition of the growth of calcium oxalate and calcium phosphate crystals in urine. Hypocitraturia is present in children with distal renal tubular acidosis. However, little information is available concerning urinary citrate excretion in various forms of childhood nephrolithiasis. The authors measured 24-hour urinary citrate and calcium excretion in 40 children while they were ingesting a standardized low calcium, low sodium diet. The results revealed a normal urinary citrate excretion in children with hypercalciuria. The difference in urinary citrate excretion between children with renal and absorptive hypercalciuria was unexplained. Their findings also suggest that differences in urinary citrate excretion cannot explain why some children with hypercalciuria have urolithiasis and others have only hematuria. It is possible that differences in urinary citrate excretion between children and adults with hypercalciuria may partly explain the apparent reduced risk for calculous formation in children. W. J. C. 2 tables, 16 references

Meningomyelocele and Hirschprung Disease: Theoretical and Clinical Significance R. G. MERKLER, S. B. SOLISH AND A. L. SCHERZER, Department of Pediatrics, Cornell University Medical Center, New York, New York Pediatrics, 76: 299-300 (Aug.) 1985 Many combinations of neural tube-derived abnormalities with other congenital anomalies have been reported, including neurenteric cysts, bladder exstrophy, diaphragmatic hernia, renal agenesis and tracheoesophageal fistula. The authors present a child with a combination of hydrocephalus associated with meningomyelocele as well as Hirschsprung's disease. They propose the concept of "neurocristopathy" -a disorder of the neural crest with pleuralistic expressions in other systems. They propose that in cases of meningomyelocele, a condition in which the neural crest has failed to form completely, a secondary aganglionic megacolon could arise as an expression of a neurocristopathy. It also is possible that a single underlying cause could render a section of the neural crest abnormal, although formed, and simultaneously inhibit the formation of a lower portion of the neural crest. While the authors acknowledge that many children with meningomyelocele have constipation owing to a neurogenic bowel, that is abnormal function in a colon normally endowed with ganglionic cells, they recommend more extensive investigation for aganglionosis in pa-

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Pediatrics, 76: 437-444 (Sept.) 1985 The authors report their 20-year experience with 8 children with adrenocortical neoplasms. All 8 patients presented with virilization and excessive linear growth. Serum levels of dehydroepiandrosterone, testosterone and cortisol appeared to be useful in the diagnosis. Sonography and computerized tomography were helpful in tumor localization. Of the 8 children 7 had a benign course. G. W. K. 1 table, 118 references

Monozygotic Siblings Discordant for N euroblastoma: Etiologic Implications B.

H. KUSHNER AND L. HELSON, Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, New York, New York

J. Ped., 107: 405-409 (Sept.) 1985

The authors describe a case of disseminated neuroblastoma in 1 of monozygotic triplets. The literature was reviewed with regard to twins who were concordant and discordant for neuroblastoma. The information obtained supports the thesis that hereditary factors may be important in neuroblastoma diagnosed in infants, while random mutational events may be important in cases of neuroblastoma diagnosed after infancy. G. W. K. 2 tables, 24 references

Effect of Donor-Specific Transfusions on Renal Transplantation in Children D.

POTTER, M. GAROVOY, S. HOPPER, P. TERASAKI AND 0. SALVATIERRA, Departments of Surgery and Pediatrics, University of California, San Francisco, California

Pediatrics, 76: 402-405 (Sept.) 1985 During a 6-year period the immunological consequences of donor-specific transfusion were studied. Three transfusions of whole blood or packed cells (10 ml./kg.) were given at 2-week intervals. A total of 17 patients received l mg./kg. azathioprine beginning 1 week before donor-specific transfusion and continuing until after transplantation. Ten patients became sensitized to the donor blood. Of those who did not receive azathioprine 26 per cent became sensitized, compared to only 11 per cent of those receiving azathioprine. A total of 37 children received transplants from their blood donors. Actuarial kidney survival in this group was 97 per cent at 1 year, and 93 per cent at 2 and 5 years. During the same period actuarial kidney survival in children receiving cadaver kidneys was 71 per cent at 1 year and 64 per cent at 2 years. G. W. K. 2 figures, 1 table, 13 references