Congenital hepatic fibrosis (CHF) is an inherited, noncirrhotic liver disease.
Prominent clinical features include the following:
Portal hypertension (portal HTN)
Increased risk of asce...
Severely affected patients are usually diagnosed in utero or shortly after birth due to massively enlarged cystic kidneys.
Prenatal renal enlargement and dysfunction may result in olig...
Monitor growth, weight gain, nutritional status, and for vitamin A, D, E, K malabsorption, with supplementation as needed.
Vaccinate against encapsulated bacteria as patients ...
Acute upper GI tract bleeding or melena requires urgent esophagogastroduodenoscopy (EGD) for bleeding control, frequent Hgb/Hct checks, blood cu...
Chandar J, Garcia J, Jorge L, et al. Transplantation in autosomal recessive polycystic kidney disease: liver and/or kidney? Pediatr Nephrol. 2015;30(8):1233–1242. [View Abstra...
777.8 Other specified perinatal disorders of digestive system
572.3 Portal hypertension
576.1 Cholangitis
P78.89 Other specified perinatal digestive system disorders
K76.6 Portal hypertensi...
Q: Will other children of mine be affected?
A: Maybe. If the inheritance pattern is autosomal recessive, the possibility of having an affected sibling is 1:4.
Q: Is my child at increased risk if he ...
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