URINARY 17-KETOSTEROIDS IN CYSTIC FIBROSIS
Corresponding Author
PETER STEEN PEDERSEN
University Clinic of Paediatrics (TG), Rigshospitaiet and the Hormone Department, Statens Seruminstitut, Copenhagen, Denmark
*Department of Paediatrics, TG 7313 Rigshospitalet, Tagensvej DK-2200 Copenhagen N DenmarkSearch for more papers by this authorE. WINGE FLENSBORG
University Clinic of Paediatrics (TG), Rigshospitaiet and the Hormone Department, Statens Seruminstitut, Copenhagen, Denmark
Search for more papers by this authorSVEND G. JOHNSEN
University Clinic of Paediatrics (TG), Rigshospitaiet and the Hormone Department, Statens Seruminstitut, Copenhagen, Denmark
Search for more papers by this authorCorresponding Author
PETER STEEN PEDERSEN
University Clinic of Paediatrics (TG), Rigshospitaiet and the Hormone Department, Statens Seruminstitut, Copenhagen, Denmark
*Department of Paediatrics, TG 7313 Rigshospitalet, Tagensvej DK-2200 Copenhagen N DenmarkSearch for more papers by this authorE. WINGE FLENSBORG
University Clinic of Paediatrics (TG), Rigshospitaiet and the Hormone Department, Statens Seruminstitut, Copenhagen, Denmark
Search for more papers by this authorSVEND G. JOHNSEN
University Clinic of Paediatrics (TG), Rigshospitaiet and the Hormone Department, Statens Seruminstitut, Copenhagen, Denmark
Search for more papers by this authorAbstract
ABSTRACT. Pedersen, P. S., Flensborg, E. W. and Johnsen, S. G. (University Clinic of Paediatrics (TG), Rigshospitaiet and the Hormone Department, Statens Seruminstitut, Copenhagen, Denmark). Individual 17-ketosteroids in Cystic Fibrosis. Acta Paediatr Scand, 70: 507,.–Data are presented for chromatographically separated urinary 17-ketosteroids from 184 normal children and 106 children suffering from cystic fibrosis. In the latter group the 5β-metabolites, 11-hydroxy-ethiocholanolone and 11-keto-ethiocholanolone, were found to be decreased, suggesting changes in the steroid metabolism. The relation of this finding to a number of different investigations is discussed.
References
- 1 Breslow JL, Epstein J, Fontaine JH. Dexamethasone-resistant cystic fibrosis fibroblasts show cross-resistance to sex steroids. Cell 1978; 13: 663–69.
- 2 Flensborg EW. The possibility of a specific androgen defect in cystic fibrosis. Acta Paediatr Scand 1972; 61: 381.
- 3 Gray CE, Hamilton W. Excretion of dehydroepian-drosterone in cystic fibrosis. Acta Paediatr Scand 1971; 60: 617.
- 4 di Sant'Agnese PA, Darling RC, Perera GA. Shea E. Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. Pediatrics 1953; 12: 549–63.
- 5 Pedersen PS, Johnsen SG, Flensborg EW. Udskillelse i urin af fraktioneret 17-ketosteroider hos danske børn. Ugeskr laeger (in press).
- 6 Vestergård P, Sayegh JF, Mowat JH, Hemmingsen L. Estimation after multicolumn liquid chromatography of common urinary neutral steroids with an application to the essay of plasma 17-oxosteroids. Acta Endocrinol, Suppl. 217, 1978; 88: 1–245.
- 7 Kampmann JP, Jørgensen FS, Bennet EP, Johnsen SG. Rise in dehydroepiandrosterone and oestrogens during clomiphene administration in normal men. Acta Endocrinol 1976; 83: 166–72.
- 8 Flensborg EW, Johnsen SG. Significant changes in two 17-ketosteroid fractions in cystic fibrosis. Acta Paediatr Scand 1972; 61: 382–83.
- 9 Danes BS, Beck B, Flensborg EW. Cystic fibrosis heterozygote detection: A family study. Clin Genet 1975; 8: 85–91.
- 10 Bercovici JP, Mauvais-Jarvis P. Hyperthyroidism and gynecomastia: Metabolic studies. J Clin Endocrinol Metab 1972; 35: 671–77.
- 11 Bradlow HL, Zumoff B, Fukushima DK, Hellman L, Gallagher TF. Metabolism of 11β-hydroxy-δ4-an-drostene-3, 17-dione: Effect of thyroid hormone. J Clin Endocrinol 1966; 26: 949–54.
- 12 Gold Nl, Crigler JF. Influence of L-triiodthyronine on steroid hormone metabolism: Studies in a patient with adrenal hyperplasia. J Clin Endocrinol Metab 1963; 23: 156–66.
- 13 Denef C, De Moor P. Sex differences in Cortisol metabolism. 11. The role of the gonadal hormones in the induction of this sex difference. Eur Journ Steroids 1966; 1: 217–31.
- 14 Wilson H, Schenker S. Effect of corticoids on urinary 5a and 5/3 C19 steroids in man. Acta Endocrinol 1964; 46: 197–206.
- 15 Smith RS, Bledsoe T, Chetri MK. Cortisol metabolism and the pituitaryadrenal axis in adults with protein-calorie malnutrition. J Clin Endocrinol Metab 1975; 40: 43–52.
- 16 Vanluchene E, Aertsens W, Vanderkerckhove D. Steroid excretion in anorexia nervosa patients. Acta Endocrinol 1979; 90: 133–38.
- 17 Fisher LK, Kogut MD, Moore J et al. Clinical, endocrinological and enzymatic characterization of two patients with 5a-reductase deficiency: Evidence that a single enzyme is responsible for the 5a-reduc-tion of Cortisol and testosterone. J Clin Endocrinol Metab 1978; 47: 653–64.
- 18 Savage DCL, Forsyth CC, Cameron J. Excretion of individual adrenocortical steroids in obese children. Arch Dis Child 1974; 49: 946–54.
- 19 Zumoff B, Bradlow HL, Gallagher TF, Hellman L. Cortisol metabolism in cirrhosis. J Clin Invest 1967; 46: 1735–43.
- 20 Pogue RE, Warwick WJ. Cystic fibrosis. Minn Med 1969; 52: 1551–55.
- 21 Stern RC, Boat TF, Doershuk CF, Tucker AS, Pri-miano FP, Matthews WL. Course of cystic fibrosis in 95 patients. J Pediatr 1976; 89: 406–11.
- 22 Olavsgaard P, Worm AM, Andersen E, Flensborg EW. Development of the centres of ossification in children with cystic fibrosis. Acta Paediatr Scand 1972; 61: 382.
- 23 Flensborg EW, Steinrud J, Nielsen EL. Treatment of cystic fibrosis with anabolic agents and, in particular, the favourable effect on pulmonary function as measured by peak expiratory flow rate. Acta Paediatr Scand 1972; 61: 383.
- 24 Dooley RR, Moss AJ, Wright PM, Hassakis PC. Nor-ethandrolone in cystic fibrosis of the pancreas. J Pediatr 1969; 74: 95–102.
- 25 Kopito LE, Kosasky HS, Shwachman H. Water and electrolytes in cervical mucus from patients with cystic fibrosis. Fertil Steril 1973; 24: 512–16.
- 26 Simopoulos AP, Lapey A, Boat TF, di Sant'Agnese PA, Bartter FC. The reninaldosterone system in patients with cystic fibrosis of the pancreas. Pediatr Res 1971; 5: 626–32.
- 27 Grand RJ, di Sant'Agnese PA, Talamo RC. Pallavi-cini JC. The effects of exogenous aldosterone on sweat electrolytes. J Pediatr 1967; 70: 357–68.
- 28 Lobeck CC, McSherry NR. Response of sweat electrolyte concentrations to 9 alpha-fluorohydrocorti-sone in patients with cystic fibrosis and their families. J Pediatr 1963; 62: 393–98.
- 29 Siegenthaler P, Haller J, Haller R, Hampai A, Muller AF. Effect of experimental salt depletion and aldosterone load on sodium and chloride concentration in the sweat of patients with cystic fibrosis of the pancreas and of normal children. Arch Dis Child 1964; 39: 61–65.
- 30 Dworkin BR, Mendelsohn RS. Hormonal factors in cystic fibrosis. Physiologist 1966; 9: 173.
- 31 Hirschowitz BI, Streeten DHP, London JA, Pollard HM. Effects of eighthour intravenous infusions of ACTH and adrenocortical steroids in normal man. J Clin Invest 1957; 36: 1171–82.
- 32 Griffin JE, Wilson JD. The syndromes of androgen resistance. N Engl J Med 1980; 302/4: 198–209.
- 33 Gorsheim D, Hait NW, Beas EC, Jepson JH, Gardner FH. Increased stem cell response to erythropoietin induced by androgens. Endocrinology 1972; 93: 777–80.
- 34 Rosenthal A, Button L, Khaw KT. Blood volume changes in patients with cystic fibrosis. Pediatrics 1977; 59: 588–94.
- 35 Chodos DDJ, Ely RS, Limbeck GA, Kelley VC. Cor-ticoid metabolism in cystic fibrosis. Am J Dis Child 1965; 110: 76–80.
Citing Literature
