Index of Suspicion in the Nursery * Case 1: Fatal Outcome of Hepatomegaly in a Newborn

Source: NeoReviews recent issues - Category: Pediatrics Authors: Tags: Pediatric Drug Labeling Update Articles Source Type: news

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AbstractThe aim of this work is to investigate the different expression patterns of B cell-specifics moloney murine Leukemia virus integration site-1 (BMI-1) and brain and acute leukemia, cytoplasmic (BAALC) genes, their prognostic and clinical significance in newly diagnosed cytogenetically heterogenous adult acute myeloid leukemia patients. BMI-1 and BAALC expression was detected in the bone marrow of patients using quantitative real-time reverse transcription polymerase chain reaction with cut off value set at 50th percentile for both genes. BMI-1 and BAALC overexpression was detected in 50% of cases which suggest their...
Source: Indian Journal of Hematology and Blood Transfusion - Category: Hematology Source Type: research
Hepatic signs – MCQ – Answer Pick out the wrong statement – Correct answer: b) Presystolic hepatic pulsations are noted in tricuspid regurgitation Presystolic hepatic pulsations are noted in tricuspid stenosis with sinus rhythm, due to forceful right atrial contraction which occurs just before systole. Systolic hepatic pulsations are noted in tricuspid regurgitation. Though hepatomegaly is usually tender in recent onset heart failure, in chronic cases it becomes non tender. Pulsations of liver in constrictive pericarditis follow that pattern in jugular venous pulse. In abdominojugular reflux, firm pressur...
Source: Cardiophile MD - Category: Cardiology Authors: Tags: Medicine MCQ - CVS Source Type: blogs
Hepatic signs – MCQ Pick out the wrong statement: a) Hepatomegaly in chronic heart failure can be non tender b) Presystolic hepatic pulsations are noted in tricuspid regurgitation c) Pulsatile hepatomegaly can be seen in constrictive pericarditis d) Abdominojugular reflux (earlier called “hepatojugular reflux”) can be noted in right heart failure Post your answer as a comment below
Source: Cardiophile MD - Category: Cardiology Authors: Tags: Medicine MCQ - CVS Source Type: blogs
Discussion Tachycardia is a rapid heart rate that is above normal for age and level of exertion. Tachycardia is common, particularly sinus tachycardia due to normally encountered circumstances such as pain, fever or exercise. It is usually a normal physiologic process but sustained tachycardia often indicates a potentially abnormal underlying cause. Sinus tachycardia has a rapid heart rate with normal P waves and P-R intervals and variations from moment to moment and respiration. Generally it is not over 200 beats/minute. Vagal stimulation can slow the heart rate; this is a gradual slowing, not an abrupt slowing seen in ...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Abstract Gaucher disease (GD) is a common lysosomal storage disorder caused by deficiency of glucocerebrosidase (GCase) due to the pathogenic variants in the GBA gene. The aim of this study was to evaluate the performance of high risk screening program for GD by measuring the enzyme activities of GCase and chitotriosidease in dried blood spots of patients with splenomegaly and/or thrombocytopenia. A total of 787 subjects (364 females and 423 males) with unexplained splenomegaly and/or thrombocytopenia were enrolled in this study from May 2016 to Aug 2019. The cutoff value of GCase activity was set as less than 3.0...
Source: International Journal of Clinical Chemistry - Category: Chemistry Authors: Tags: Clin Chim Acta Source Type: research
Myelofibrosis (MF) is a clonal myeloproliferative neoplasm (MPN) that occurs in a primary form (PMF), or develops during progression of essential thrombocythemia (ET, post-ET MF) or polycythemia vera (PV, post-PV MF). MF results in insufficient hematopoiesis, bone marrow fibrosis, and extramedullary hematopoiesis. It can be accompanied by splenomegaly, hepatomegaly, pulmonary fibrosis, and constitutional symptoms. Circulating blasts are observed. Although the clinical course is heterogeneous, the prognosis of MF is poor with a high rate of transformation into blast phase.
Source: Biology of Blood and Marrow Transplantation - Category: Hematology Authors: Source Type: research
Quick diagnosis units (QDU) have become an alternative hospital-based ambulatory medicine strategy to inpatient hospitalization for potentially serious illnesses in Spain. Whether diagnosis of pancreatic cancer is better accomplished by an ambulatory or inpatient approach is unknown. The main objective of this retrospective study was to examine and compare the diagnostic effectiveness of a QDU or inpatient setting in patients with pancreatic cancer. Patients with a diagnosis of pancreatic adenocarcinoma who had been referred to a university, tertiary hospital-based QDU or hospitalized between 2005 and 2018 were eligible. ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Observational Study Source Type: research
We report a 3-year-old boy with recurrent hemoptysis for 2 years. The child had tachycardia, tachypnea, tender hepatomegaly, and left-sided decreased air entry with crepitations. Coagulation and Koch's workup was negative. X-ray of the chest showed a small left hemithorax. Computed tomography with angiography showed left unilateral PVA with pulmonary artery hypoplasia and dysplastic left lung. The child has been posted for left pneumonectomy. Although uncommon, PVA should be diagnosed early to prevent life-threatening complications such as hemoptysis and pulmonary hypertension. The patient can be managed conservatively...
Source: Lung India - Category: Respiratory Medicine Authors: Source Type: research
AbstractThe distribution ofBCR –ABL1 transcript variants e13a2 ( “b2a2”) and e14a2 (“b3a2”) in Nigerians with chronic myeloid leukemia (CML) had not been previously studied. In addition, there is paucity of data on the impact ofBCR –ABL1 transcript variants on clinical presentation and survival in CML patients in Nigeria. TheBCR –ABL1 transcript variants were analyzed in 230 Imatinib-treated CML patients at diagnosis. Patients with incomplete data (n  = 28), e19a2 (n = 3) and e1a2 (n = 1) were excluded from analysis of transcript variant on...
Source: Indian Journal of Hematology and Blood Transfusion - Category: Hematology Source Type: research
This study identified the oxidative stress response transcription factor NRF2 as a common mediator of hepatomegaly. NRF2 activation results in elevated expression of several growth factors that belong to the Platelet-derived growth factor (PDGF) and epidermal growth factor (EGF) families. These growth factors are made by hepatocytes and activate their receptors in an autocrine fashion to stimulate the accumulation of glycogen and lipid that lead to hepatocytes and liver enlargement. The protein kinase AKT plays a key role in this process and its inhibition leads to reversal of hepatomegaly.
Source: Journal of Hepatology - Category: Gastroenterology Authors: Source Type: research
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