Clinico-Radiologic, Laboratory, And Histopathologic Profile Of Patients Diagnosed With Neonatal Cholestasis At Philippine Children&#039;S Medical Center.

Gil Bryan  B. Galvan,; Manuelito  A. Madrid,

Clinico-Radiologic, Laboratory, And Histopathologic Profile Of Patients Diagnosed With Neonatal Cholestasis At Philippine Children'S Medical Center.

Gil Bryan B. Galvan | Manuelito A. Madrid

Abstract:

BACKGROUND: Neonatal cholestasis warrants early, accurate and prompt intervention and comprises a wide spectrum of differential diagnosis which present with overlapping features, thus making a diagnosis difficult. OBJECTIVE: To evaluate the clinical and laboratory parameters that could aid to differentiate between intrahepatic and extrahepatic neonatal cholestasis. METHODS: Retrospective and descriptive study of neonatal cholestasis patients who underwent liver biopsy and were admitted at the Philippine Children's Medical Center from January 2007 to December 2011. RESULTS: Factors that favor an intrahepatic cause of cholestasis are ultrasound finding of a normal gallbladder, marked degree of giant cell transformation and presence of extramedullary hematopoiesis. Factors that favor an extrahepatic cause of cholestasis are presence of splenomegaly, markedly elevated GGT, and histopathology findings of portal and periportal ductal proliferation, bile plugs, lesser degree of giant cell transformation, septal fibrosis and cirrhosis, portal and neoductular cholestasis, and portal-portal bridges. CONCLUSION: In this study, meticulous history and physical examination aid in the diagnosis of neonatal cholestasis. Splenomegaly and markedly elevated serum GGT are suggestive of biliary atresia, and a normal gallbladder by ultrasound favors neonatal hepatitis. Although there is significant overlap of histopathologic findings of patients with neonatal cholestasis, certain parameters favor an extrahepatic over an intrahepatic process.

References:

Heejin Lee, Kim KM, Jang JY, Jang S, Yu S. The clinicopathological parameters for making the differential diagnosis of neonatal cholestasis. Korean Journal of Pathology. 2009.
Brandao MA, Amaut LT, De Tommaso AM, Hessel G. Differential diagnosis of neonatal cholestasis: Clinical and laboratory parameters. Journal de Pediatria (Rio J). 2010;86(1):40–41.
Santos JL, Almeida H, Cerski CTS, Silveira TR. Histopathological diagnosis of intrahepatic and extrahepatic neonatal cholestasis. Brazilian Journal of Medical and Biological Research. 1998;31:911–919.
Wang JS, Wang ZL, Wang XH, Zhu QR, Zheng S. The prognostic value of serum gamma-glutamyl transferase activity in Chinese infants with previously diagnosed idiopathic neonatal hepatitis. HK J Pediatr. 2008;13:39–45.
Moyer V, Freese DK, Whitington PF, Olson A, Brewer F, Colletti R, Heyman M. Guideline for the evaluation of cholestatic jaundice in infants: Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology and Nutrition. 2004;39:115–128.
Urganci N, Çetinkaya F, Kalyoncu D, Cakir EP, Yilmaz B. Infants with cholestasis: Diagnosis, management and outcome. Marmara Medical Journal. 2012;25:83–.
Lee WS, Chai PF. Clinical features differentiating biliary atresia from other causes of neonatal cholestasis. Ann Acad Med Singapore. 2010;39:648–654.
Redkar RG, Masarweh M, Howard ER, Karani J, Mele-Vergani G, Davenport M. Inspissated bile in infants: Recent advances and current concepts. Department of Paediatric Surgery, Radiology & Institute of Liver Studies, King’s College Hospital, London. Pediatric Gastro Conf. 2005.
Gilbert-Barnes E. (2007). Pathology of the fetus, infant and child. 2nd ed. Mosby Elsevier.
Burt AD, Portmann BC, Ferrell LD. (2002). MacSween’s pathology of the liver. 4th ed. Churchill Livingstone, Elsevier.
Humphrey TM, Stringer MD, et al. Biliary atresia: US diagnosis. Radiological Society of North America. 2007;244:845–851.