Clinical and pathological challenges in the diagnosis of late-onset biliary atresia: four case studies

Biliary atresia (BA) is classically described at the neonatal age. However, rare cases of BA in older infants have also been reported. We report four cases of late-onset BA in infants older than 4 weeks (3 males, 1 female), and describe the diagnostic and management difficulties. One of the cases had a late-onset (29 weeks) presentation with a successful surgical procedure. We highlight the importance of this unusual differential diagnosis in infants with cholestatic syndrome, who may benefit from Kasai surgery, regardless of age.

Steroid-induced cataracts in patients with autoinmune hepatitis

BACKGROUND: Posterior subcapsular cataract (PSC) has been reported in patients on corticosteroids therapy for various pathologies. Autoimmune hepatitis (AIH) is a progressive inflammatory hepatic disease for which corticosteroids are recommended but PSC has not yet been reported in AIH patients on corticosteroids therapy. METHODS: The AIH patients at our service underwent an ophthalmological assessment and their files were analyzed establish the dosage and duration of medication, the presence of visual complaints and the type of AIH, in order to correlate these with the ophthalmological findings. Patients were examined with a slit lamp following pupil dilation. RESULTS: 17 AIH patients evaluated, only one patient had PSC. CONCLUSION: Although the frequency of PSC was low (1/17) in AIH patients on costicosteroid therapy, these individuals should undergo occasional ophthalmological assessment for the presence of cataracts since their visual acuity and quality of life may be adversely affected.

Caroli’s disease: 6 case studies

Caroll’s disease is characterized by congenital non-obstructive dilatation of the intrahepatic bile ducts of undefined etiology. It is a rare disease usually affecting the whole liver but it may affect a lobe or a segment (11). This study shows the evolution of 6 cases (2 boys and 4 giris) that were diagnosed with Caroli’s disease at a referral service. Their ages ranged from 2 to 16 years--median age 10 years. One of the patients presented with cholangitis, while hepatomegaly was observed in 83

of the cases. Four of the patients presented biliary lithiasis and in one of these cholesterol crystals could be observed in the duodenal secretion. The diagnosis was confirmed in 4 cases by endoscopic retrograde cholangiopancreatography, in 1 by cholangioresonance and 1 by echography. Two of the patients also presented congenital hepatic fibrosis. The outpatient clinic follow-up indicated that all the patients evolved well with the exception of one patient who was selected as a candidate for liver transplantation.

Gastric emptying in rats with acetaminophen-induced hepatitis

The objective of this work was to study the gastric emptying (GE) of liquids in fasted and sucrose-fed rats with toxic hepatitis induced by acetaminophen. The GE of three test meals (saline, glucose and mayonnaise) was evaluated in Wistar rats. For each meal, the animals were divided into two groups (N = 24 each). Group I was fed a sucrose diet throughout the experiment (66 h) while group II was fasted. Forty-two hours after the start of the experiment, each group was divided into two subgroups (N = 12 each). Subgroup A received a placebo and subgroup B was given acetaminophen (1 g/kg). Twenty-four hours later, the GE of the three test meals was assessed and blood samples were collected to measure the serum levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST) and acetaminophen. In group IB, the mean AST and ALT values were 515 and 263 IU/l, respectively, while for group IIB they were 4014 and 2472 IU/l, respectively. The mean serum acetaminophen levels were higher in group IIB (120 µg/ml) than in group IB (87 µg/ml). The gastric retention values were significantly higher in group IIB than in group IIA for all three test meals: saline, 51 vs 35 percent; glucose, 52 vs 38 percent and mayonnaise, 51 vs 29 percent(median values). The correlation between gastric retention and AST levels was significant (P<0.05) for group IIB for the three test meals: r = 0.73, 0.67 and 0.68 for saline, glucose and mayonnaise, respectively. We conclude that GE is altered in rats with hepatic lesions induced by acetaminophen, and that these alterations may be related to the liver cell necrosis caused by the drug

Correlation between the severity of acute hepatic necrosis induced by acetaminophen and serum aminotransferase levels in fasted and sucrose-fed rats

The relationship between serum aminotransferase levels and the acute hepatic necrosis induced by acetaminophen was studied in 24 male Wistar rats (220-265 g). The animals were divided into two groups, one of which was fasted for 66 h (group I) while the other was fed only sucrose cubes ad libitum (group II). The animals received 1 g acetaminophen per Kg body weight 42 h after the onset of the experiment. Twenty-four hours later, blood was drawn to measure aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels and the liver was removed for both macro- and microscopic examination. The intensity of the hepatic necrosis was scored according to the extent of the lesion. The hepatic necrosis was more frequent and intense in group I, with the aminotransferase levels being higher in this group (median AST and ALT levels were 3900 IU/l and 2511 IU/l, respectively, for group I and 119 IU/l and 79 IU/l, respectively, for group II). There was a positive correlation (rs) between the intensity of hepatic necrosis assessed microscopically and the levels of AST (group I, rs = 0.83; group II, rs = 0.79) and ALT (group I, rs = 0.58; group II, rs = 0.80). These findings suggest that aminotransferase levels are a reliable indicator of the degree of hepatic necrosis in this model of acetaminophen intoxication.