Liver and Pancreas

Abstract

27.1

General considerations

■ Preferred term is congenital hyperinsulinism

■ Incidence 1:50,000 live births

■ Unregulated secretion of insulin in pancreatic β-cell hyperplasia due to alterations of the K+-ATP channel (mutations of four genes, for the following: Kir6.2 and sulfonylurea, glucokinase, glutamate dehydrogenase receptor)

■ Persistent hypoglycemia is often resistant to therapy

27.2

General considerations

■ Incidence: 1:10,000 live births

■ End result of a destructive inflammatory process, unknown etiology (viral, toxic)

27.3

General considerations

■ Incidence: 1:100,000 live births

■ Female:male ratio 3:1 to 4:1

■ 60% of cases are diagnosed before 10 years of age

■ Most frequent etiology

• Common pancreaticobiliary channel

• Pressure in pancreatic duct higher than in the bile duct

■ Reflux of pancreatic juice in the common bile duct damages endothelium, causing cystic dilatation

■ Other etiologies are also possible, such as obstruction of the distal common bile duct and genetic reasons

27.4

General considerations

■ Not as frequent as in adults. More frequently discovered within the last three decades, probably because of improvements in diagnostic techniques (ultrasonography)

■ Two peaks of appearance: first in infancy and second in early adolescence with a steady increase thereafter

■ Female:male ratio 1:1 in infancy, 2–4:1 in prepuberty

■ Table 27.5 equates pathogenic mechanisms with risk factors for cholelithiasis

27.5

General considerations

■ Echinococcus granulosus is found more commonly in endemic areas such as Mediterranean shores of Europe, but Echinococcus alveolaris can also be found

■ Humans are the intermediate host contaminated orally by ingestion of parasitic eggs

■ The eggs liberate scoleces, which migrate through the bowel wall into the portal vein and then to the following organs where they develop into a hydatid

• 70% in the liver

• 20% in the lung

• 10% in other organs

■ The hydatid cyst usually presents as a space-occupying mass, either univesicular with a thin capsule or multivesicular with a thick capsule (Fig. 27.7)

■ The disease can be complicated if there is a biliary fistula or rupture into a hollow cavity occurs

■ Echinococcus alveolaris behaves like a tissue-invading malignant tumor

■ The liquid tumor has a variable size and, with time, the host considers it a foreign body and tends to push it to the organ’s periphery

27.6

General considerations

■ Congenital cysts, originating from the biliary system

■ Clear liquid content; wall covered with biliary-type epithelium

■ Localization most often superficial

27.7

General considerations

■ General considerations are given in Table 27.6

27.8

Indications

■ End-stage chronic liver diseases

• Cholestatic origin (biliary atresia is the most frequent indication followed by Byler’s disease-progressive familial intrahepatic cholestasis)

• Alagille’s syndrome

• Cholestatic cirrhosis of unknown etiology

• Cirrhosis of unknown etiology, post-hepatitis B cirrhosis

• Inborn error of metabolism

– With normal liver function, i.e., Crigler–Najjar, Wilson disease

– With evolution towards cirrhosis, i.e., alpha-1-antitrypsin deficiency, tyrosinemia, glycogenosis cystic fibrosis

■ Acute liver failure

■ Unresectable liver tumor (mostly hepatoblastoma)