Introduction And Importance: Gaucher disease is a rare autosomal recessive lysosomal storage disorder marked by a substantial reduction in beta-glucocerebrosidase activity. Historically, supportive treatments such as splenectomy and orthopedic interventions were employed, whereas recent advances have led to the approval of Enzyme Replacement Therapy (ERT) and Substrate Reduction Therapy (SRT) as therapeutic options.
Case Presentation: The authors present the case of a 61-year-old female with chronic abdominal pain, abdominal fullness, pancytopenia, and hepatosplenomegaly, all indicative of Gaucher's disease, later confirmed by histopathological examination.
JNMA J Nepal Med Assoc
October 2021
Herniation of bladder mucosa through the bladder wall muscle layer is known as bladder diverticulum. The incidence of bladder diverticulum is 1.7.
View Article and Find Full Text PDFIt is high time now to discover novel drugs due to the increasing rate of drug resistance by the pathogen organisms and target cells as well as the dependence or tolerance of the body towards the drug. As it is obvious that significant numbers of the modern day pharmaceuticals are derived from natural products, it is equally astonishing to accept that venoms of various origins have therapeutic potentials. Wasp venoms are also a rich source of therapeutically important toxins which includes short cationic peptides, kinins, polyamines and polyDNA viruses, to name a few indentified.
View Article and Find Full Text PDFPrimary extra-osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is rare. The tumor has poor prognosis with high risk of recurrence and hematogenous spread, most commonly to the lungs. We report a case of primary osteosarcoma of the breast occurring in a 58-year old woman.
View Article and Find Full Text PDFHornet stings are medically important stings which can cause allergic manifestations and, in severe cases, may lead to the unusual complication of acute renal failure (ARF) and other systemic complications. ARF results from toxic or ischaemic acute tubular necrosis in a setting of haemolysis or rhabdomyolysis or both and acute allergic interstitial nephritis. Venom from hornet stings can also contribute to myocardial injury or liver impairment.
View Article and Find Full Text PDFPrimary small cell carcinoma of esophagus (SCC) is a rare disease but has more aggressive behavior than esophageal squamous cell carcinoma (SQC). The distinction of SCC from SQC is very important therapeutically. Few systematic studies of immunohistochemical analysis to differentiate primary esophageal SCC with concomitant SQC, and adjacent normal esophageal epithelium have been reported.
View Article and Find Full Text PDFWe examined 23 patients with columnar-lined esophagus (2 with long segment and 21 with short segment) endoscopically. After staining with Lugol's iodine solution, squamous islands were identified in the columnar mucosa in 18 (78%) of the 23 patients. When biopsy specimens were obtained from these islands, esophageal glands proper or their ducts were seen in 12 (67%) of the 18 cases.
View Article and Find Full Text PDFArch Pathol Lab Med
February 2005
Many histologic changes have been described in the esophageal squamous mucosa in patients with gastroesophageal reflux disease (GERD), including dilated intercellular spaces, balloon cells, intrapapillary vessel dilation, elongated papillae, basal cell hyperplasia, acanthosis, intraepithelial eosinophils, Langerhans cells, and p53 protein overexpression. To define a set of histologic changes that are invariably reflux associated, we examined the histologic changes in esophageal specimens from normal controls, patients with GERD, patients without GERD but with a suspicion of other pathology, and patients with esophageal carcinoma. We also examined biopsy specimens from sites with differing endoscopic features, including cloudy white and reddened mucosa.
View Article and Find Full Text PDFThe incidence of peripheral pulmonary adenocarcinoma has increased in recent years. Clara cell has been known as target for carcinogens and source of pulmonary tumors. One of the presumed roles of the bronchiolar Clara cell is the secretion of pulmonary surfactant into the bronchiolar lumen.
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