Biopsy-driven diagnosis in infants with cholestatic jaundice in Iran.

AIM: To determine the frequencies of diagnoses confirmed by liver biopsy in infants with cholestasis in an Iranian pediatric hospital. METHODS: This was a retrospective study conducted in a tertiary referral children's hospital in Iran. We retrieved all pathology reports of liver biopsies from children less than two years of age who had presented for evaluation of cholestatic jaundice from March 2001 to March 2011. Additional specimen samples obtained from archived pathology blocks were reviewed by a pathologist blinded to the final diagnosis. These results were compared with the pathology reports from chart records to ensure consensus and eliminate any inconsistencies in final diagnoses. A structured checklist was used to gather information on multiple variables including age, sex, gestational age at birth, birth weight, age at which hyperbilirubinemia manifested, presence and identification of associated anomalies, clinical manifestations, and histological findings from liver biopsies. The baseline data are reported using descriptive statistics, and differences between groups were assessed by Fisher's exact test and Student's t test when indicated. RESULTS: Fifty-five cases (28 females; 27 males) of infantile cholestasis (IC) were included in this study. The mean serum total bilirubin and direct bilirubin at presentation were 13.6 ± 5.9 and 7.3 ± 3.4, respectively. Forty cases (72.7%) were the product of term pregnancies. Common associated clinical findings were acholic stool in 33 cases (60.0%), hepatomegaly in 30 cases (54.5%), and dark-colored urine in 21 cases (38.2%). Biliary atresia (BA) was the most frequent diagnosis, found in 32 cases (58.2%), followed by intrahepatic bile duct paucity found in 6 cases (10.9%), metabolic disease in 6 cases (10.9%), idiopathic neonatal hepatitis in 5 cases (9.1%), choledochal cyst in 2 cases (3.6%), liver cirrhosis in 2 cases (3.6%), and progressive familial intrahepatic cholestasis and portal fibrosis each in 1 case (1.8%). The mean times for jaundice onset and liver biopsy were 43.8 and 102.0 d, respectively. In BA, the mean age at jaundice presentation was 21 d and for liver biopsy was 87.5 d, representing a mean delay of 66.5 d. CONCLUSION: A significant delay was found between IC presentation and liver biopsy, which is detrimental in conditions that can cause irreversible liver damage, such as BA.

Validation of a Persian version of the Fibromyalgia Impact Questionnaire (FIQ-P).

The aim of this study is to translate, adapt, and validate a Persian version of the Fibromyalgia (FM) Impact Questionnaire (FIQ-P). The FIQ-P was adapted following the translation and back-translation approach; then, it was administered to thirty females with FM. Participants also completed two other validated questionnaires, the Medical Outcome Survey Short Form-36 (SF-36) and the Beck Depression Inventory (BDI). Internal consistency within the FIQ-P items and its test-retest reliability were assessed with Cronbach's alpha and Spearman's correlation coefficient, respectively. Construct validity was analyzed by Spearman's r when correlating the FIQ-P to other questionnaires. The translated version was concordant. Adaptation affected two sub-items of physical function. Participants' mean age ± standard deviation was 40.4 ± 9.0 years. Internal consistency proved good with α = 0.80. Test-retest coefficient ranged from 0.50 for the item "work days missed" to 0.79 for all FIQ-P items. Fair and statistically significant (P < 0.01) correlations were found between the FIQ-P items and two other questionnaires, SF-36 (r = -0.57) and BDI (r = 0.53). We concluded that the FIQ-P is a valid and reliable instrument for measuring health status of Persian-speaking FM patients.

Common bile duct stone associated with hemolytic uremic syndrome.

Cholelithiasis is an unusual complication of hemolytic uremic syndrome. A 12-year-old boy with hemolytic uremic syndrome, established by renal biopsy, who developed cholestatic jaundice is presented here. Laboratory results for secondary causes of hemolytic uremic syndrome were normal. Abdominal ultrasonography and magnetic resonance cholangiopancreatography revealed extrahepatic obstruction. A common bile duct stone, discovered by retrograde cholangiopancreatography was extracted by sphincterotomy. In conclusion, cholelithiasis should be considered as a cause of abdominal pain and cholestasis in patients who are diagnosed as having hemolytic uremic syndrome.