جستجوی مقالات مرتبط با کلیدواژه "آترزی مجاری صفراوی" در نشریات گروه "پزشکی"

Children with cholestasis experience a wide spectrum of disease, ranging from transient liver function abnormalities to life-threatening conditions. These children may require hospitalization or specialized care at various times due to liver enzyme disorders and associated clinical issues. In severe cases, liver damage can lead to acute liver failure, necessitating liver transplantation or resulting in death. Early diagnosis of the etiology of cholestasis can be crucial for improving prognosis. This study aimed to determine the frequency, causes, and outcomes of cholestasis in infants referred to Amir Kola Children's Hospital.

This retrospective descriptive study included all infants with cholestasis admitted to AmirKola Children's Hospital between 2012 and 2022. Inclusion criteria were infants under 2 years of age diagnosed with cholestasis. Infants with incomplete medical records were excluded from the study. Hepatic cholestasis was diagnosed based on the infant's medical history and confirmed by a pediatric gastroenterologist. All infants underwent diagnostic evaluation tests and abdominal ultrasound. Liver biopsy was performed when necessary to assess liver pathology. Short-term outcomes of hepatic cholestasis, including length of hospitalization, frequency of hospitalization, need for special care, liver transplantation, and mortality, were evaluated. Demographic information, along with the causes and outcomes of cholestasis, were reported using measures of central tendency (mean) and dispersion (standard deviation), or as frequencies and percentages.

During the study period (2012-2022), 3,650 infants under 2 years of age were admitted to Amir Kola Children's Hospital. The frequency of cholestasis among hospitalized children was 54 (1.47%). The most common symptom of cholestasis was hepatomegaly, observed in 35 (64%) infants. The leading causes of cholestasis included biliary atresia in 21 (38.8%) infants, idiopathic hepatitis in 17 (31.4%) infants, and congenital metabolic disorders in 7 (12.96%) infants. Of the children in the study, 23 (42%) were hospitalized at least once, and 10 (18.5%) were hospitalized twice. Liver biopsy was performed in 32 (60.4%) of the 54 children; 11 had neonatal cholestasis, 7 had normal ultrasound results, and 13 had giant cell hepatitis and neonatal hepatitis (10 with normal ultrasound results), with 2 showing normal pathology reports. Additionally, 20 (37%) of these children died, 24 (44%) recovered, and 9 required liver transplantation.

According to the results of this study, more than one-third of children with cholestasis died, with biliary atresia and idiopathic hepatitis identified as the most common causes. Biliary atresia was also the most frequent indication for liver transplantation. These findings highlight the need for timely diagnostic examinations, testing, and specialized evaluations to support early diagnosis and treatment in infancy.

Early diagnosis of biliary atresia is very important for better outcome of patients. Ultrsonography is one of the tools for early differentiating of biliary atresia from other causes of neonatal cholestasis. It has been reported that triangular cord sing (TACS) in sonography is a reliable sign for diagnosis of biliary atresia. The aim of this study was to reassess the accuracy of TACS alone and coupled with abnormal gallbladder in the diagnosis of biliary atresia. Infants with prolonged cholestatic jaundice underwent ultrasonography and liver biopsy. Results of ultrasound scans (TASC and gallbladder abnormality) were compared with histopathologic findings. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of ultrasonographic findings for diagnosis of biliary atresia based on liver histopathology were calculated. The sensitivity, specificity and accuracy of TACS for diagnosis of biliary atresia were 36%, 95% and 77% respectively. Positive predictive value and negative predictive value were 77%. The sensitivity and specificity of gallbladder abnormality for diagnosis of biliary atresia were 36% and 88% respectively. Positive predictive value of TACS couple with gallbladder abnormality for diagnosis of biliary atresia was 100%. The ultrasonographic TACS is very specific for diagnosis of biliary atresia. Positive predictive value and accuracy of this sign is much higher than of gallbladder abnormality. The sensitivity of TACS for diagnosis of biliary atresia is very low.

Cholestatic jaundice in early infancy is an important clinical condition. Idiopathic neonatal hepatitis and extra-hepatic biliary atresia (EHBA) is two main of different etiology, and it's necessary to distinguish between these two entities. It is important that EHBA be rapidly diagnosed, because an immediate surgery may prevent further potentially fatal consequences. A rapid, ubiquitous, non-invasive and accurate technique with the least complications is crucial for early diagnosis of EHBA. The hepatobiliary scan identifies diseased gallbladders and bile drainage problems, and ultrasonography is an easy and cheap method of survey of biliary tract. In this study, we evaluated and compared two different diagnostic method, ultrasonography and HIDA scan for this purpose. Method & Materials: In these two years prospective study ultrasonography, HIDA scan and liver biopsy was performed in a group of patients with cholestasis. We considered the result of liver biopsy as the gold standard. Accordingly sensitivity, specificity, positive and negative predictive values and accuracy of ultrasonography and HIDA scan were calculated and compared together. Sixty infants, 35 males and 25 females mean age (56.1±17.8 days) were studied According to the results of liver biopsy, there were 16 (26.7%) cases of EHBA, and 24 (40%) cases of idiopathic hepatitis. Ultrasonography detected EHBA in 15 cases and HIDA scan diagnosed it in 38 patients. Sensitivity, specificity, positive predictive values, negative predictive value and accuracy of ultrasonography were 87.5%, 97.7%, 93.3%, 95.7%, and HIDA scan, these value were 100%, 50%, 42.1%, 100% respectively. This study showed a lower sensitivity but higher specificity of ultrasonography for diagnosis of EHBA in compared with HIDA scan