Anaesthetic Management of a Patient with Synchronous Kartagener Syndrome and Biliary Atresia.
نویسندگان
چکیده
Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated.
منابع مشابه
The Role of Appendix in the Management of Biliary Atresia Associated with Bowel Atresia
Biliary atresia is characterized by the progressive obliteration of extra- and intrahepatic biliary duct system leading to the obstruction of bile flow in infancy. The cause(s) of biliary atresia remain unclear and many surgical options for bypassing the atretic segment have been described.1-3 Biliary atresia may be associated with small bowel atresia. The surgical interventions for biliary dr...
متن کاملPeri-Operative High-Dose v Post-Operative Low Dose Steroid Therapy in the Management of Biliary Atresia: a Preliminary Report
Background: The use of high-dose steroid therapy peri portoenterostomy may have a positive impact on the frequency of cholangitis and survival rate. Methods: A prospective study was conducted on two groups of patients (less than three months of age) suffering from biliary atresia from 1999 to 2005. The patients in group I (G I) were managed peri-operatively by high-dose methylprednisolone while...
متن کاملUltrasonographic Triangular Cord Sign and Gallbladder Abnormality in Diagnosis of Biliary Atresia
Introduction: Early diagnosis of biliary atresia is very important for better outcome of treatment. Ultrasonography is one of the diagnostic tools for early differentiation of biliary atresia from other causes of neonatal cholestasis. It has been reported that triangular cord sign (TACS) in sonography is a reliable sign for diagnosis of biliary atresia. The aim of this study was to re-assess ...
متن کاملApplication of ursodeoxycholic acid in hepatobiliary scintigraphy for neonatal hyperbilirubinemia: Comparison with phenobarbital
Introduction: Early differentiation of biliary atresia from neonatal hepatitis is of utmost importance, since on time surgery of biliary atresia significantly improves the outcome. Hepatobiliary scintigraphy is an integral part of diagnosis work-up of these patients; however its specificity for diagnosis of biliary atresia is suboptimal. In this study we evaluated t...
متن کاملBiliary atresia associated with meconium peritonitis caused by perforation of small bowel atresia.
BACKGROUND/PURPOSE This report describes our experiences with 5 cases of biliary atresia associated with meconium peritonitis caused by perforation of small bowel atresia. METHODS A review of medical records was undertaken in an effort to recognize cases of biliary atresia associated with meconium peritonitis. RESULTS Five patients of 171 with biliary atresia (2.9%) were detected to have me...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Turkish journal of anaesthesiology and reanimation
دوره 43 3 شماره
صفحات -
تاریخ انتشار 2015