Study of Cardiac Dysfunction in Portal Hypertension: A Single-Center Experience From Eastern India.

INTRODUCTION: Cardiac functional abnormalities are common in patients with cirrhosis of the liver. Nonetheless, the effect of portal hypertension and liver disorder on cardiac abnormalities is yet to be investigated. The current study evaluated the contribution of cirrhotic and non-cirrhotic portal hypertension as the potential cause of cardiac abnormalities. METHODS: The present study was a cross-sectional observational study. After excluding known heart diseases, 128 patients with portal hypertension from different causes were enrolled in the study. Cardiac functional activity was assessed by electrocardiogram (ECG) and transthoracic echocardiography (TTE).  Results: This study included a total of 128 patients, out of which 24 had extrahepatic portal vein obstruction (EHPVO), four patients had Budd-Chiari syndrome and 100 had liver cirrhosis. Normal ventricular function was observed in patients with EHPVO and Budd-Chiari syndrome. Sixty-eight percent of cases had liver cirrhosis diastolic abnormalities. The mean QTc interval in patients with cirrhotic cardiomyopathy (CCM) was 0.49 ± 0.05 sec which was significantly increased when compared to patients without CCM with 0.432 ± 0.07 at p=0.0016. The Child Turcotte Pugh (CTP) score and MELD (Model for End-Stage Liver Disease) score in patients with CCM were significantly higher as compared to patients without CCM. All alcoholic cirrhotic and non-alcoholic cirrhotic patients had equal prevalence of diastolic dysfunction (p-value >0.05). CONCLUSION:  Patients with Child class C or a high MELD score are associated with a higher prevalence rate of CCM while normal cardiac function was observed among patients having portal hypertension due to extrahepatic causes. We recommend cardiac evaluation by echocardiography in all cirrhotic patients. Institution of specific medical therapy and early referral for liver transplantation should be considered to improve survival in patients with decompensated cirrhosis.

The Clinico-histopathological Spectrum of Tumors and Tumor-Like Lesions in Adult Intussusception.

BACKGROUND: Intussusception is an uncommon cause of intestinal obstruction in adults. The etiology of this disease differs from the children. Thus, its management depends on the possible etiology and is different from pediatric cases. AIMS: We aimed to study the clinico-histopathological spectrum of the tumors and tumor-like lesions in the intussusception in adults. MATERIAL AND METHODS: A retrospective review of the adult (> 18 years) intussusception cases was performed. The clinical data and follow-up were obtained. The histopathology was reviewed along with the special stains and immunohistochemistry for ascertaining a histopathological diagnosis. RESULTS: Fifteen cases of adult intussusception were identified from 107 resected specimens of adult intestinal obstruction. The mean age was 44.5 years with a male/female ratio of 1.1:1. A definitive pathology could be ascertained in 80% of the cases (n = 12/15). Eight cases had benign non-neoplastic etiology (53.3%) (33.3% tumor-like lesions) while seven cases (46.7%) had neoplastic etiology (20% benign neoplastic; 26.7% malignant neoplastic). All cases of colonic or enterocolic intussusceptions were associated with neoplasia whereas 90% of the enteric intussusceptions occurred due to benign non-neoplastic causes.  CONCLUSIONS: Non-neoplastic causes are predominant in the enteric intussusceptions while neoplastic causes are more commonly associated with colonic or enterocolic intussusceptions. The post-operative histopathological examination concludes on the adequacy of the index surgery or the provision of further management if required.

Enhancer of Zeste Homolog 2 (EZH2) in Malignant Progression of Gallbladder Carcinoma.

PURPOSE: Data related to the role of epigenetic modifications in gallbladder carcinoma (GBC) is limited. We intended to assess the role of crucial epigenetic modifiers pertaining to histone modification and DNA-methylation system in gallbladder carcinogenesis. METHODS: The expression of EZH2, H3K27me3, and DNA methyltransferases (DNMTs) was analyzed by immunohistochemistry in cases of GBC (n = 39), gallbladder dysplasia (GBD, n = 12), and benign mucosa (BM, n = 16). A semi-quantitative scoring system was used for assessing the immunohistochemical expression. RESULTS: The expression of EZH2 was significantly higher in cases of GBC than GBD (p value 0.001). The cases of BM were negative. Its expression was also higher in poorly differentiated tumors and positively correlated with the proliferative activity (MIB-1 labeling index) (p value 0.03 and 0.01, respectively). There was no significant difference in the expression levels of H3K27me3, DNMT-1, and DNMT-3B among GBC, GBD, and BM cases. Although GBC cases with strong EZH2 expression showed a shorter overall survival, the difference was not statistically significant. CONCLUSION: This study highlights the crucial role of the key epigenetic regulators EZH2 in the pathobiology and evolution of gallbladder carcinogenesis. Given the reversibility of epigenetic alterations, EZH2 may be a novel therapeutic target for gallbladder carcinogenesis.

Thyroid paraganglioma: A case-based systematic review of literature.

Thyroid paragangliomas are distinctly rare primary thyroid neoplasms with nearly 75 cases reported worldwide. Due to their similar embryological origin and cytohistomorphology with other thyroid neoplasms, they may pose great diagnostic challenges for pathologists, radiologists, endocrinologists, as well as surgeons leading to unnecessary aggressive therapy. With recent advances in molecular genetics, the prognostic significance of such seemingly innocuous thyroid neoplasms has been better understood. In this manuscript, we describe such a case and present a systematic review of all reported cases till date giving an update on our current knowledge regarding their diagnostic pitfalls, pathology, and molecular genetics.

Acquired Pure Red Cell Aplasia Following Recombinant Erythropoietin (Darbepoetin-alfa) Therapy.

Acquired pure red cell aplasia (PRCA) following use of recombinant erythropoietin (rEPO) is distinctly rare and sporadically reported in the literature. We discuss a case of PRCA following the usage of rEPO (darbepoetin-α) during the management of anemia of chronic kidney disease in an elderly male subject with review of literature and a brief insight into proposed pathophysiologic mechanism, diagnosis, and management.

Pediatric neuroblastic tumors: A critical evaluation of cytomorphological features for risk stratification on aspiration cytology.

BACKGROUND: Neuroblastic tumor (NT) is the most common extracranial solid tumor of childhood with variable outcome which again depends on risk stratification related to distinct biology of the tumor. The use of fine-needle aspiration (FNA) material for evaluation of cytomorphological parameters and risk stratification in NTs using cytology prognostic score (PS) is limited in routine practice. METHODS: We reviewed 38 FNA cytology cases diagnosed as pediatric small round cell tumor between time period June 2017 to December 2019 for clinical, cytomorphological and immunohistochemical features. RESULTS: Ten out of 38 small round cell tumors were NTs. All 10 cases were further subclassified according to International Neuroblastoma Pathology Classification into undifferentiated neuroblastoma (n = 1), poorly differentiated neuroblastoma (n = 5), differentiating neuroblastoma (n = 2), and ganglioneuroblastoma (n = 2). Cytologic PS was done using the morphological criteria as described previously in literature. The patients were divided into favorable and unfavorable cytomorphological prognostic categories with a cut off scoring of 12. A score of more than 12 is associated with high risk morphology and advanced stage. All cases with PS > 12 (n-4) (unfavorable cytomorphology) had a poor outcome compared to six patients with PS < 12 (n-6) (favorable cytomorphology). CONCLUSION: Aspiration cytology can be used as a first line investigation to evaluate the cytomorphological features for risk stratification and diagnosis in patients with NTs using cytological prognostic scoring system. A larger multi-centric validation study necessitates for cytological risk stratification.

Factors Influencing the Age at Discharge of Very Low Birth Weight Preterm Neonates From a Neonatal Intensive Care Unit in Eastern India: A Cohort Study.

OBJECTIVE: To study factors that influence the age of discharge of very low birth weight (VLBW) preterm neonates from the Neonatal Intensive Care Unit (NICU). SETTING: This cohort study was conducted in the NICU, Department of Pediatrics, Kalinga Institute of Medical Sciences, Bhubaneswar, India, from April 2019 to December 2019. PATIENTS: Neonates whose birth weight was <1500 g and gestation <37 weeks were enrolled. Those having major congenital malformation were excluded. OUTCOME: Age at discharge (in days). RESULTS: Out of 114 neonates enrolled, 84 neonates completed the study and discharged, 29 neonates died during the study, and one patient left against medical advice. Demographic, antenatal, neonatal factors, and mother's participation in care of the baby were compared with respect to age at discharge in univariate analysis. Those found significant on univariate analysis were subjected to multivariate analysis. In the multivariate analysis model two independent predictors were observed, birth weight and gestation, which were inversely related to age at discharge. CONCLUSION: Birth weight and gestation are the two most important factors which significantly influence the age at discharge from hospital of VLBW preterm neonates.

Giant Cell Tumor with Secondary Aneurysmal Bone Cyst of the Patella: A Case Report.

A 15-year-old girl presented with pain and swelling on the anterior aspect of the right knee for one year. The radiological evaluation with x-rays and magnetic resonance imaging suggested a benign aggressive lesion of the right patella with a cortical breach. Core needle biopsy of the lesion revealed it to be a giant cell tumor (GCT). She was treated with total patellectomy and end-to-end repair of quadriceps to the patellar tendon. The histopathological report of the whole specimen revealed it to be a GCT with secondary aneurysmal bone cyst (ABC). After 24 months, she was asymptomatic, and there was no evidence of local recurrence or distal metastasis. An extensive review of the literature revealed only four cases of combined GCT with secondary ABC in the patella. Though rare, GCT with secondary ABC of the patella should be kept as a differential diagnosis for anterior knee pain and swelling in young patients. The diagnosis is solely based on histopathological findings. It is imperative to obtain a precise tissue diagnosis in the preoperative period to plan appropriate treatment.