Gastroesophageal Variceal Bleeding as a Complication of Cystic Fibrosis in a 3-Month-Old Patient
Abstract
Cystic fibrosis (CF) is a hereditary disease of mucous and sweat glands, which affects the respiratory and gastrointestinal systems. Herein, we describe a 3-month-old girl with a history of recurrent episodes of urinary tract infections that required hospitalization. She was referred to our center at the age of three months, with massive gastroesophageal variceal bleeding. In physical examination, she had clubbing, hepatosplenomegaly, and mild ascites. Laboratory studies revealed high serum levels of liver enzymes and low level of Albumin. As of suspicious to CF, sweat tests were performed twice which confirmed the diagnosis of CF. Gastrointestinal bleeding due to gastroesophageal varices is a rare complication of CF, which could result as a consequence of hepatobiliary involvement of disease. Early diagnosis of CF could prevent severe complications and even death in this group of patients.
Marshall SG, Debley JS. The respiratory system. In: Kliegman RM, Marcdante KJ, Jenson HB, Behrman RE,editors. Nelson essentials of pediatrics. 5th ed.Philadelphia: Elsevier Saunders 2006: 648-9.
Grossman S, Grossman LC. Pathophysiology of cystic fibrosis. Crit Care Nurse 2005;25:46-51.
McAuley DF, Elborn JS. Cystic fibrosis: basic science. Paediatr Respir Rev 2000;1:93-100 .
Flora KD, Benner KG. Liver disease in cystic fibrosis. Clin Liver Dis 1998;2:51-61 .
Diwakar V, Pearson L, Beath S. Liver disease in children with cystic fibrosis. Paediatr Respir Rev 2001;2:340-9.
Wilschanski M, Durie PR. Patterns of GI disease in adulthood associated with mutations in the CFTR gene. Gut 2007;56:1153-63.
Dogan AS, Conway JJ, Lloyd-Still JD. Hepatobiliary scintigraphy in children with cystic fibrosis and liver disease. J Nucl Med 1994;35:432-5.
Fitzsimmons SC. Cystic Fibrosis Foundation, Patient Registry1996. Annual Data Report Bethesda, Maryland. 1997.
Karrer FM, Holland RM, Allshouse MJ, Lilly JR. Portal vein thrombosis: treatment of variceal hemorrhage by endoscopic variceal ligation. J Pediatr Surg 1994;29:1149-51.
Kowdley KV. Ursodeoxycholic acid therapy in hepatobiliary disease. Am J Med 2000;108:481-6.
Files | ||
Issue | Vol 54, No 3 (2016) | |
Section | Case Report(s) | |
Keywords | ||
Cystic fibrosis Gastrointestinal bleeding Gastroesophageal varices Hepatomegaly |
Rights and permissions | |
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |