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Congenital hepatic fibrosis usually presents in adolescent or young adulthood, but onset of signs and symptoms can range from early childhood through mid-life. Clinical features may vary but commonly include cholangitis, hepatomegaly and signs of portal hypertension. [citation needed]
Hepatomegaly is enlargement of the liver. [4] It is a non-specific medical sign, having many causes, which can broadly be broken down into infection, hepatic tumours, and metabolic disorder. Often, hepatomegaly presents as an abdominal mass. Depending on the cause, it may sometimes present along with jaundice. [1]
Liver transplantation is an option for those children whose liver function and symptoms fail to respond to a Kasai operation. [29] Recent large-scale studies by Davenport et al. (Annals of Surgery, 2008) show that the age of the patient is not an absolute clinical factor affecting prognosis. The influence of age differs according to the disease ...
The severity of the disorder can vary within the same family, with symptoms ranging from so mild as to go unnoticed, to severe heart and/or liver disease that requires transplantation. [6] It is uncommon, but Alagille syndrome can be a life-threatening disease with a mortality rate of 10%. [ 7 ]
[11] [12] The build-up of tissue in the liver, known as hepatic fibrosis, may cause symptoms such as jaundice, ascites, abnormal bleeding and enlarged spleen. [12] Kidney complications in the form of polycystic kidney or nephronophthisis is estimated to affect 77% of patients with COACH syndrome, and is therefore a highly significant symptom of ...
Consequently, liver disease and neuropsychiatric symptoms are the main features that lead to diagnosis. [5] People with liver problems tend to come for medical attention earlier (generally as children or teenagers) than those with neurological and psychiatric symptoms, who tend to be in their 20s or older.
The treatment of chronic liver disease depends on the cause. Specific conditions may be treated with medications including corticosteroids , interferon , antivirals , bile acids or other drugs. Supportive therapy for complications of cirrhosis include diuretics , albumin , vitamin K , blood products , antibiotics and nutritional therapy.
Hepatoblastoma is a malignant liver cancer occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells. They usually present with an abdominal mass. The disease is most commonly diagnosed during a child's first three years of life. [1]