Fecal flora of infants with biliary atresia: effects of the absence of bile on fecal flora.

The fecal flora of nine bottle-fed infants with biliary atresia were examined to determine the effects of the absence of bile on the intestinal flora. The numbers of the following fecal flora were significantly reduced compared with healthy infants: bifidobacteria, lecithinase-negative clostridia, streptococci, and staphylococci. Bifidobacteria were reduced to the greatest extent, constituting 75% of total flora in healthy comparison infants but only 2.4% in infants with biliary atresia. The most prevalent bacteria in infants with biliary atresia were enterobacteria.

Long-term prognosis in biliary atresia after hepatic portoenterostomy: analysis of 35 patients who survived beyond 5 years of age.

The long-term prognosis in 35 patients with biliary atresia, who had undergone successful hepatic portoenterostomy and survived beyond 5 years of age, was studied. Eighteen (51%) patients developed ascending cholangitis after surgery. Jaundice recurred or increased in half of the patients, mainly as a result of ascending cholangitis. Another serious problem was the development of portal hypertension. Esophageal varices developed in 16 (46%) patients, with rupture and massive hemorrhage in seven. Leukopenia resulting from hypersplenism was common. Eighteen patients had WBC counts less than 5000/microliter. Thrombocytopenia was less common than leukopenia. Height was slightly below normal, although weight was within the normal range. Three patients died of hepatic failure; all had previously had ascending cholangitis. These observations indicate that ascending cholangitis is critical in the long-term and short-term prognoses in patients who have undergone successful repair of biliary atresia.

Fat absorption in patients with surgically repaired biliary atresia.

The degree of fat absorption was studied in 4 patients with unrepaired biliary atresia and 11 with repaired biliary atresia (6 with mild jaundice, 5 with no jaundice). The percent absorption was conspicuously reduced in unrepaired patients (mean 30%, range 21 to 39%), moderately reduced in repaired patients with mild jaundice (mean 78%, range 67 to 89%), and completely normal in repaired patients with no jaundice (mean 93%, range 89 to 97%). The excretion pattern of main fecal fatty acids in unrepaired patients was very different from that of normal controls, low in stearic and high in oleic, but very similar to the pattern of fatty acids contained in ingested milk. In repaired patients with mild or no jaundice the pattern was an intermediate one between those of normal controls and unrepaired patients. The effect of medium-chain triglycerides on fat absorption was favorable in repaired patients with mild jaundice, but not in those with no jaundice. There was an inverse correlation between fat absorption and 15 min indocyanine green retention value.

Serum 25-hydroxy-vitamin D in hepatobiliary disease in infancy.

Serum 25-hydroxy-vitamin D (25-OHD) concentrations were measured in 49 patients with hepatobiliary disease in infancy. Low mean values were found in groups of patients with biliary atresia, neonatal hepatitis, choledochal cyst, and chronic intrahepatic cholestatic syndrome. In the group of patients with surgically repaired biliary atresia, the mean value did not differ from normal. Parenteral vitamin D increased 25-OHD in serum in patients with biliary atresia, but did not do so in one patient with neonatal hepatitis. In contrast, oral vitamin D did not increase serum 25-OHD concentrations in patients with biliary atresia. It is concluded that the reduction of serum 25-OHD seen in biliary atresia was largely due to the malabsorption of vitamin D, while in neonatal hepatitis it was due to impairment of 25-hydroxylation of the vitamin.

Choledochal cyst in infancy and childhood. Analysis of 16 cases.

Choledochal cyst was diagnosed before operation in 14 out of 16 infants and children by means of a combination of diagnostic tests. Ultrasonic echogram showed a cystic mass in all 11 patients examined. The characteristic displacement of the duodenum was noted in 9 of 14 patients by contrast studies of the upper gastrointestinal tract, and hepatobiliary scintiscanning confirmed choledochal cyst in 7 of 8 patients (88%). A cyst was shown by oral or intravenous cholangiography in 3 of 4 patients. Onset of symptoms occurred from 7 days of age to 9-5 years; in 10 patients onset occurred under 12 months of age, in 3 from 13 to 24 months, and in the remaining 3 from 2 to 9-5 years. The first symptoms were jaundice, vomiting, acholic stools, and poor sucking. Obstructive jaundice and acholic stools were found in more than 80% of the patients, and abdominal mass in 60%. Vomiting occurred in 50% of the patients, one-third of whom showed failure to thrive, and 70% of patients over the age of 1-5 years had abdominal pain. About half of the patients had a mild to moderate degree of fever. In all patients primary excision of the cyst was followed by hepaticojejunostomy in Roux-en-Y fashion. The postoperative course was uneventful in 14 patients; 2 patients died, one from ascending cholangitis and one from hepatic failure.

Congenital biliary atresia. Analysis of 97 cases with reference to prognosis after hepatic portoenterostomy.

The prognosis of 97 patients with congenital biliary atresia treated by hepatic portoenterostomy was studied. In 37 of 97 patients (38%), good bile excretion was observed after surgery, and jaundice cleared. Sixteen of the 37 patients developed ascending cholangitis; 11, esophageal varices; and nine died. Seven died of ascending cholangitis and two of hepatic failure. Fourteen patients had an uneventful course. In the remaining 60 patients, bile excretion was scanty, and the disease progressed to biliary cirrhosis; 52 died. Causes of death were hepatic failure (53%), intracranial hemorrhage (16%), rupture of esophageal varices (12%), severe dehydration (9%), and postoperative complications (9%). The age at death ranged from 2 to 52 months, with an average at 16 months.

Hyperparathyroidism in hepatobiliary disease in infancy.

Metabolism of calcium and magnesium may be disturbed in hepatobiliary disease because of deficient or absent bile flow into the gut, since bile is important for the intestinal absorption of these elements. In the present paper the tubular reabsorption of phosphate (TRP), calcium (TRCa), and magnesium (TRMg) were determined in an attempt to evaluate the parathyroid function of infants and children with hepatobiliary disease. In unrepaired biliary atresia TRP was conspicuously reduced (mean 49.8%, SD 15.1). In successfully repaired biliary atresia the value was increased near the normal range (mean 80.7%, SD 8.1). In neonatal hepatitis the value was variable in individual cases, but significantly lower than the normal (mean 47.6%, SD 19.9). TRCa was reduced in one third of the patients with unrepaired biliary atresia and in one fifth of the cases of neonatal hepatitis. The value was within the normal range in repaired biliary atresia. TRMg was decreased in both unrepaired and repaired biliary atresia and in neonatal hepatitis. The effect of intravenous calcium infusion on TRP, TRCa and TRMg was evaluated in 3 patients with unrepaired biliary atresia. TRP was conspicuously enhanced after infusion. TRCa was decreased in 3 to a variable extent. TRMg was moderately increased in 2 and greatly decreased in 1. These results indicate that infants with hepatobiliary disease are in a state of secondary hyperparathyroidism because of deficient or absent bile flow into the intestines.