Osteopetrosis (Marble bone disease) is a very rare congenital genetic disease of skeleton, resulting from defective bone resorption, due to functionally defective osteoclast, leading to accumulation of excessive bone mass. Malignant infantile osteopetrosis (MIO) is one of the varieties of osteopetrosis, which is fatal and is diagnosed in early infancy. Malignant infantile osteopetrosis is present with abnormal bone remodeling, hematological abnormities, features of extramedullary hematopoiesis. Radiology is the key of diagnosis. In this case, we present a 5-month-old male infant diagnosed as malignant infantile osteopetrosis, who presented with bronchopneumonia, anemia, thrombocytopenia, hepatosplenomegaly, failure to thrive (FTT).
Summary:The outcome of anaesthesia in coronary artery surgery depends partly on the duration of invasive manipulations including endotracheal intubation. It is ideal to avoid prolonged mechanical ventilation and attempt early extubation. Depressant effect of some anaesthetic agents and narcotics makes it a common practice to ventilate the patients of coronary artery bypass graft (CABG) surgery overnight resulting in unsatisfactory respiratory and haemodynamic performance. This study was aimed at overcoming the effects of prolonged mechanical ventilation after CABG surgery by using Total Intravenous Anaesthesia (TIVA) and by extubating the patient early to achieve a better postoperative respiratory cardiovascular performance. The study showed that in CABG surgery, TIVA produces non-significant depression of post-operative respiratory performance which helps in early extubation compared to those receiving conventional anaesthesia and electively ventilated overnight.
Purpose
Perinatal cytomegalovirus (CMV) infection can lead to biliary atresia (BA) in different entities. This study aimed to compare the clinical, hematological, biochemical, and histological features of infants with BA based on their CMV immunoglobulin M (IgM) status at presentation.
Methods
This cross-sectional descriptive study was carried out between January 2019 and June 2020 at the Department of Pediatric Gastroenterology and Nutrition at the Bangabandhu Sheikh Mujib Medical University (BSMMU) in Dhaka. Forty-three patients with BA were selected purposively and categorized into either the CMV IgM-positive or CMV IgM-negative BA group. Categorical variables were compared using Fisher’s exact test and chi-square tests, while the Student’s
t
-test and Mann–Whitney U-test were used to compare continuous variables. For all statistical tests, a
p
-value <0.05 was considered statistically significant.
Results
Thirty-three (76.7%) of the cases were between 2 and 3 months of age on admission. The clinical, hematological, and biochemical parameters did not differ significantly between the CMV IgM-positive and CMV IgM-negative BA groups. Most (50.0%) of the CMV IgM-positive cases had fibrosis stage F2, while 43.5% of the CMV IgM-negative cases had fibrosis stage F3, with no significant difference between the groups (
p
=0.391).
Conclusion
Our data shows no significant distinction between CMV IgM-positive and CMV IgM-negative BA, suggesting that CMV does not contribute to BA pathogenesis.
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