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)
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© 2009 Springer-Verlag Berlin Heidelberg
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Le Coultre, C., Otte, JB., Tica, C. (2009). Liver and Pancreas. In: Zachariou, Z. (eds) Pediatric Surgery Digest. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-34033-1_27
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DOI: https://doi.org/10.1007/978-3-540-34033-1_27
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