ABSTRACT
Wilson disease (WD) is an autosomal recessive disorder of copper metabolism with diverse clinical manifestations. Zinc (Zn) has been used for treatment of WD. Recent studies showed low serum zinc level in patients suffering from WD than the normal. This cross-sectional analytical study has been designed to compare the serum zinc level between paediatric patients suffering from WD but yet not started treatment and children who have normal ALT level. This study was carried out at the Department of Pediatric Gastroenterology and Nutrition, BSMMU, Dhaka, Bangladesh from July 2018 to June 2019. Total 51 children were included in this study. Among them 27 were diagnosed case of WD aged between three to eighteen years and 24 children of same ages who were suffering from other than liver disease having normal ALT were included as volunteers. The patients of WD were divided into four groups according to their presentation as acute hepatitis, chronic liver disease (CLD), acute liver failure & neuropsychiatric manifestation. Informed written consent was obtained from all patients and volunteers for participation in this study. Along with other physical findings and laboratory investigations 3 ml of venous blood were collected for estimation of serum zinc level. After estimation of serum zinc level results were analyzed statistically. The difference in serum zinc levels were compared between the groups. Serum zinc level was significantly lower in Wilson disease patients (43.8±19.7µg/dl; range: 13-83) compared to volunteers group (67.8±11.8µg/dl; range: 47-97) p<0.001. Among the diseased group, serum zinc level were significantly lower in 18 CLD (38.4±17.4µg/dl) and in 4 acute liver failure (33.1±3.7µg/dl) compared to 4 acute hepatitis (71.8±4.3µg/dl) (p=0.001) and (p<0.001) respectively. Mean serum zinc level was low in 4 Wilsonian acute liver failure (33.1±3.7µg/dl), which was significant compared to those (23) who presented as Wilson disease non acute liver failure (45.7±20.8µg/dl) (p=0.013). Serum zinc level was significantly lower in Wilson disease children compared to the volunteers. Zinc level was also found significantly low in Wilson disease presented as CLD and acute liver failure in comparison to Wilson disease presented as acute hepatitis.
Subject(s)
Hepatolenticular Degeneration , Liver Failure, Acute , Humans , Child , Child, Preschool , Adolescent , Bangladesh , Cross-Sectional Studies , VolunteersABSTRACT
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-monthold male infant diagnosed as malignant infantile osteopetrosis, who presented with bronchopneumonia, anemia, thrombocytopenia, hepatosplenomegaly, failure to thrive (FTT).
Subject(s)
Anemia , Osteopetrosis , Infant , Humans , Male , Child, Preschool , Osteopetrosis/diagnosis , Osteopetrosis/diagnostic imaging , Bone Marrow , Splenomegaly/diagnostic imaging , Splenomegaly/etiology , RadiographyABSTRACT
The most devastating pandemic of this era coronavirus disease-2019 (COVID-19) is caused by a novel virus named severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2). Although it is primarily a respiratory pathogen, it can also result in several extra-pulmonary manifestations includes gastrointestinal symptoms, hepatocellular injury. Angiotensin-converting enzyme-2 (ACE-2) receptor and transmembrane serine protease 2 (TMPRSS2), the entry receptor for the causative coronavirus SARS-CoV-2 is co-express in the gastrointestinal tract, hepatocyte, and cholangiocytes similar to the respiratory mucosa. The presence of these receptors facilitates the entry into the tissue and causes direct viral tissue damage, which is a proposed mechanism of injury. Diarrhoea, nausea, vomiting, abdominal discomfort are common gastrointestinal manifestations, whereas derangement of liver function tests is the most hepatic manifestation in COVID-19. In this article, we reviewed on SARS-CoV-2 disease COVID-19 regarding gastrointestinal, hepatic, and pancreatic manifestation, the mechanisms by which the virus may inflict damage, and their management perspective.
