medigraphic.com
Órgano Oficial de la Asociación Mexicana de Hepatología

2007, Number 2

<< Back Next >>

Ann Hepatol 2007; 6 (2)

Anterior pituitary hormone effects on hepatic functions in infants with congenital hypopituitarism

Karnsakul W, Sawathiparnich P, Nimkarn S, Likitmaskul S, Santiprabhob J, Aanpreung P

Language: English
References: 32
Page: 97-103
PDF size: 65.57 Kb.


Text Extraction

Background: Congenital hypopituitarism is an uncommon cause of neonatal cholestasis. Little is known about the effect of anterior pituitary hormone on hepatic functions. Methods: A retrospective review of the medical charts of eight infants with congenital hypopituitarism and neonatal cholestasis was performed. The results of endocrinological investigations, eye examinations, and magnetic resonance imaging were used to classify these infants. Results: Eight infants (4 male and 4 female; mean age, 1.7 weeks) who presented with cholestatic jaundice subsequently (mean age, 7.6 weeks) developed isolated or multiple anterior pituitary hormone deficiencies. Persistent hypoglycemia, ocular abnormalities, and microphallus were often clinical signs prompting further endocrinological and radiological investigations. Septo-optic dysplasia was prevalent, occurring in five cases. Cholestasis and hepatosplenomegaly resolved within a mean of 9.7 and 10 weeks, respectively, in the majority of cases after replacement of glucocorticoid and thyroid hormones. However, transaminase levels remained high after hormone replacement. Cortisol deficiency and hypoglycemia were noted in all cases, often following stress. Hyperlipidemia persisted in one case after the resolution of cholestasis and after corticosteroid and thyroid hormone replacement therapy. Growth hormone deficiency was not corrected due to the absence of hypoglycemia after corticosteroid hormone, an infant’s age, and/or a lack of financial resources. Conclusions: In our series, it appears that glucocorticoid and thyroid hormones play a significant role in the resolution of cholestasis and hepatosplenomegaly. A persistently elevated transaminase level and hyperlipidemia after corticosteroid and thyroid hormone replacement may indicate the need for long-term follow-up and/or growth hormone therapy.


REFERENCES

  1. Krähe J, Hauffa BP, Wollmann HA, Käser H. Transient elevation of urinary catecholamine excretion and cholestatic liver disease in a neonate with hypopituitarism. J Pediatr Gastroenterol Nutr 1992; 14: 153-9.

  2. Prabhakar VK, Shalet SM. Aetiology, diagnosis, and management of hypopituitarism in adult life. Postgrad Med J 2006; 82: 259-66.

  3. Daaboul JJ, Kartchner W, Jones KL. Neonatal hypoglycemia caused by hypopituitarism in infants with congenital syphilis. J Pediatr 1993; 123: 983-5.

  4. Coppola A, Spera C, Varcaccio Garofalo G, Gargiulo A, Nasti A. Partial anterior hypopituitarism caused by toxoplasmosis congenita. Description of a clinical case. Minerva Med 1987; 78: 403-10.

  5. Massa G, Vanderschueren-Lodeweyckx M, Van Vliet G, Craen M, de Zegher F, Eggermont E. Hypothalamo-pituitary dysfunction in congenital toxoplasmosis. Eur J Pediatr 1989;148:742-4.

  6. Hanna CE, Krainz PL, Skeels MR, et al. Detection of congenital hypopituitary hypothyroidism: ten-year experience in The Northwest Regional Screening Program. J Pediatr 1986; 109: 959-64.

  7. Parks JS, Brown MR, Hurley DL, Phelps CJ, Wajnrajch MP. Heritable disorders of pituitary development. J Clin Endocrinol Metab 1999; 84(12): 4362-70.

  8. Izenberg N, Rosenblum M, Parks J. The endocrine spectrum of SOD. Clin Pediatr 1984; 23: 632-6.

  9. Gunduz K, Gunalp I, Saatci I. Septo-optic dysplasia associated with bilateral complex microphthalmos. Ophthalmic Genet 1996; 17: 109-13.

  10. Trauner M, Meier PJ, Boyer JL. Molecular pathogenesis of cholestasis. N Engl J Med 1998; 339: 1217-27.

  11. Ellaway CJ, Silink M, Cowell CT, et al. Cholestatic jaundice and congenital hypopituitarism. J Paediatr Child Health 1995; 31: 51-3.

  12. Brewer DB. Congenital absence of the pituitary gland and its consequence. J Pathol Bacteriol 1957; 73: 59-67.

  13. Giacoia GP, MacGillvary MH. Neonatal hypothalamic hypopituitarism and liver dysfunction. NY State J Med 1981; 81: 1625-5.

  14. Lanes R, Blanchette V, Edwin C, et al. Congenital hypopituitarism and conjugated hyperbilirubinemia in two infants. Am J Dis Child 1978; 132: 926-8.

  15. Bauman JW, Chang BS, Hall FR. The effects of adrenalectomy and hypophysectomy on bile flow in the rats. Acta Endocrinol 1966; 52: 404.

  16. Macarol V, Morris TQ, Baker KJ, Bradley SE. Hydrocortisone choleresis in the dog. J Clin Invest 1970; 49: 1714.

  17. Dumont M, Erlinger S. Influence of hydrocortisone on bile formation in the rat. Biol Gastroenterol 1973; 6: 197.

  18. Gartner LM, Arias IM. Hormonal control of hepatic bilirubin transport and conjugation. Am J Physiol 1972; 222: 1091-9.

  19. Shaffer EA, Zahavi I, Gall DG. Postnatal development of hepatic bile formation in the rabbit. Dig Dis Sci 1985; 30(6): 558-63.

  20. Drop SLS, Colle E, Guyda HJ. Hyperbilirubinemia and idiopathic hypopituitarism in the newborn period. Acta Paediatr Scand 1979; 68: 277-80.

  21. Hodges S, Buckler JMH. Neonatal cholestasis and hypopituitarism. Arch Dis Child 1984; 59: 1200.

  22. Sheehan AG, Martin SR, Stephure D. Neonatal cholestasis, hypoglycaemia and congenital pituitarism. J Pediatr Gastroenterol Nutr 1992; 14: 426-30.

  23. Poley JR, Smith JD, Thompson JB, Seely JR. Improved micellar dispersal of dietary lipid by bile acids during replacement therapy in growth hormone deficient children. Pediatr Res 1977; 12: 1186-91.

  24. Greco F, Finocchiaro M, Polizzi A, Caruso M. Early onset of septo-optic dysplasia. Minerva Pediatr 2002; 54: 57-62.

  25. Ishibashi S, Murase T, Yamada N, Tanaka K, Taikaku F, Sato K. Hyperlipidaemia in patients with hypopituitarism. Acta Endocrinol (Copenh) 1985; 110: 456-60.

  26. Abdu TA, Neary R, Elhadd TA, Akber M, Clayton RN. Coronary risk in growth hormone deficient hypopituitary adults: increased predicted risk is due largely to lipid profile abnormalities. Clin Endocrinol (Oxf) 2001; 55: 209-16.

  27. Herman SP, Baggenstoss AH, Cloutier MD. Liver dysfunction and histologic abnormalities in neonatal hypopituitarism. J Pediatr 1975; 87: 892-5.

  28. Lovinger RD, Kaplan SL, Grumbach MM. Congenital hypopituitarism associated with neonatal hypoglycemia and microphallus: four cases secondary to hypothalamic hormone deficiencies. J Pediatr 1975; 87: 1171-81.

  29. Johnson JD, Hansen RC, Albritton DW, et al. Hypoplasia of the anterior pituitary and neonatal hypoglycemia. J Pediatr 1973; 82: 634-41.

  30. Choo-Kang LR, Sun CC, Counts DR. Cholestasis and hypoglycemia: manifestations of congenital anterior hypopituitarism. J Clin Endocrinol Metab 1996; 81: 2786-9.

  31. Kaufman FR, Costin G, Thomas DW, Sinatra FR, Roe TF, Neustein HB. Neonatal cholestasis and hypopituitarism. Arch Dis Child 1984; 59: 787-9.

  32. Minami K, Izumi G, Yanagawa T, Shimoyamada Y, Yoshigawa N. Septo-optic dysplasia with congenital hepatic fibrosis. Pediatr Neurol 2003; 29: 157-9.




2020     |     www.medigraphic.com