Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries

Background and Aim:  A major challenge in neonatal cholestasis (NC) is to differentiate biliary atresia (BA) from other non-atretic causes. In developing countries there are considerable problems of late referral of NC cases and performing surgery without prelaparotomy liver biopsy that contributes to a high proportion of negative laparotomy and increased morbidity. We evaluated the hepatic histopathology for presence of features that correlate best with the diagnosis of BA and assessed the accuracy of percutaneous liver biopsy.
Methods:  Fifty-five cases of NC that fulfilled the selection criteria and had liver biopsy available were analyzed. Among the 49 adequate liver biopsies, 28 cases were diagnosed as BA, 15 neonatal hepatitis (NH) and 6 were due to other causes. Validity of percutaneous liver biopsy diagnoses was compared with confirmed cases by laparotomy findings and 1-year follow up. Twelve histological parameters of confirmed cases of BA and NH were evaluated by logistic regression analyses.
Results:  Ductular proliferation ( P  = 0.0002), bile duct and ductular bile plugs ( P  = 0.009), and portal fibrosis ( P  = 0.002) were the best indicators of BA and among them ductular proliferation was the most important in distinguishing BA from NH. Ductal plate malformation was observed in 17.9% cases of BA. Sensitivity and specificity of percutaneous liver biopsy for diagnosing BA was 88.2% each.
Conclusion:  Percutaneous liver biopsy is highly accurate (88.2%) in diagnosing BA. In developing countries. This investigation should be done to decrease the frequency of negative laparotomy and to achieve cost–benefit with reduced morbidity.     

GOLD Classification of COPD: Discordance in Criteria for Symptoms and Exacerbation Risk Assessment

The new A-B-C-D Global Initiative for Chronic Obstructive Lung Disease (GOLD) classification of severity of chronic obstructive pulmonary disease (COPD) is based on combined symptoms and exacerbation risk assessment. The assumed equivalence between dyspnoea modified Medical Research Council (mMRC) grade ≥2 and COPD Assessment Test (CAT) score ≥ 10 to identify more symptoms has been questioned. Whether the exacerbation risk assessment criteria, old GOLD spirometry staging and frequency of exacerbations, are equivalent has not been examined. We evaluated the extent of agreement between these alternative criteria and whether it improved by redefining the equivalence between mMRC grade and CAT score. CAT scores, mMRC grades of dyspnoea, frequency of exacerbations and spirometry stages were computed in 400 patients with COPD. Receiver operating characteristic curve was analysed to determine the best CAT score to identify more symptoms. CAT scores across mMRC grades and the frequency of exacerbations across spirometry stages showed substantial overlaps. The symptoms criteria gave discordant classification in 88 (22%) patients (kappa 0.62) and the exacerbation risk assessment criteria in 181 (45%) patients (kappa 0.12). A CAT score of ≥10 had 82% sensitivity but 24% specificity to identify mMRC grade ≥ 2, while a score of 17 had 98% specificity but a low sensitivity of 52% and did not improve the agreement. We conclude that symptoms and exacerbation risk assessment criteria of the new GOLD classification yield discordant group categorisations. Lack of any satisfactory equivalence between CAT score and mMRC grades implies that the former cannot be used alone. Using the higher of mMRC ≥ 2 and CAT score ≥ 17 to identify more symptoms would avoid discordant categorisation.     

Sequential occurrence of combined pulmonary fibrosis and emphysema syndrome in a non‐smoker female patient

The combined pulmonary fibrosis and emphysema (CPFE) syndrome is a unique and an under‐recognized disorder characterized by emphysema in the upper lobes and interstitial fibrosis in the lower lobes of the lung. It occurs predominantly in males and almost exclusively in smokers. This rare combination of a restrictive and an obstructive mechanical defect carries a poorer prognosis than either of the two components. We present a case of CPFE syndrome in a non‐smoker female patient who developed lower lobe emphysema subsequent to development of interstitial fibrosis. The case was remarkable for the extreme rarity of several presenting features, namely, a lower lobe occurrence of emphysema subsequent to pre‐existent interstitial fibrosis, female gender and absence of a history of smoking.     

Efficacy of Single Low-Dose Rasburicase in Management of Tumor Lysis Syndrome in Leukemia and Lymphoma Patients

BackgroundTumor lysis syndrome (TLS) is a metabolic emergency in hematology patients. The recommended dose of rasburicase for the management of TLS is 0.2 mg/kg per day for 5 days, which is cost prohibitive for many patients. We sought to determine the efficacy of single low-dose rasburicase in the prevention and treatment of hyperuricemia in TLS.Patients and MethodsWe planned a prospective study for the safety and efficacy of fixed (weight based) dose of rasburicase to manage TLS. Patients diagnosed with leukemia/lymphoma with laboratory or clinically confirmed TLS or presence of ≥ 2 high-risk factors and serum uric acid > 7.5 mg/dL were included. The primary endpoint was uric acid normalization (< 7.5 mg/dL) within 24 hours of rasburicase administration.ResultsFifty-five patients were recruited for this study. Pediatric patients (< 18 years) accounted for 43.6% of cases. Rasburicase was provided prophylactically to 43 patients (78.2%) and for treating TLS to 12 (21.8%). Mean ± standard deviation serum uric acid at baseline and 24 hours was 9.2 ± 1.8 mg/dL and 3.2 ± 2.1 mg/dL, respectively. There was significant reduction in the serum uric acid and creatinine (P < .001) within 24 hours of rasburicase administration. The response was maintained up to 72 hours. A single dose of rasburicase was effective in 94.5% of patients. Single low-dose rasburicase led to 95% direct cost savings compared to the recommended dose.ConclusionSingle-dose rasburicase with frequent laboratory monitoring is effective in the management of TLS and offers significant cost reductions.     

Airway compromise during central venous cannulation in an undiagnosed tubercular retropharyngeal abscess: A case report

Central venous cannulation is often associated with complications during insertion even by expert''s hand and with the aid of ultrasound. We encountered a patient for central line insertion through the right internal jugular vein having a retropharyngeal abscess of tubercular origin. We accidentally punctured the abscess cavity leading to increased respiratory distress and subsequent need of intubation to the patient. This kind of complication during central line insertion has never been reported before. We intend to report such a case to alert everyone about the grave complications it can lead to and the methods to minimize them in the times ahead.