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Non-neuronopathic Gaucher disease (Type I) in an elderly female: a case report.
Ann Med Surg (Lond)
November 2024
Department of General and Gastrointestinal Surgery, Nepal Mediciti, Lalitpur, Nepal.
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.
Unveiling three accessory spleens in one patient: a rare case report and literature review.
Int J Emerg Med
November 2024
Department of Surgery, Faculty of Medicine, University of Aleppo, Aleppo, Syria.
Article Synopsis
- The spleen develops from mesenchymal cells in the dorsal mesentery during embryogenesis, and accessory spleens are common anomalies that can be mistaken for tumors; this case is the first report of three accessory spleens found in Syria in a patient who had a prior splenectomy.
- A 33-year-old male experienced right-sided abdominal pain, and after medical evaluation, imaging tests showed a lobulated mass in the right adrenal gland and elevated levels of metanephrine, suggesting a pheochromocytoma; laparoscopic surgery ultimately revealed multiple accessory spleens without signs of cancer.
- The case emphasizes the importance of considering accessory spleens in patients with masses near the adrenal glands, as they can complicate diagnosis and treatment
Predictors for spontaneous remission in childhood chronic immune thrombocytopenia.
Ann Hematol
December 2024
Department of Pediatrics, College of Medicine, National Cheng Kung University Hospital, National Cheng Kung University, No. 138, Shengli Road, Tainan, 704302, Taiwan.
This study examined the factors associated with spontaneous remission in children with chronic immune thrombocytopenia (ITP). We retrospectively analyzed the medical records of patients diagnosed with ITP from January 1988 to December 2019 at our institute. A total of 104 children with chronic ITP were identified.
View Article and Find Full Text PDFSplenic Torsion From a Wandering Spleen.
Cureus
September 2024
General Surgery, Keesler Medical Center, Biloxi, USA.
Wandering spleen is a unique condition defined as a hypermobile spleen with a sole attachment to its vascular pedicle. The most common complication is torsion of the spleen, resulting in a surgical emergency. Early diagnosis and prevention of splenic infarction are the mainstays of treatment to help preserve immunologic function.
View Article and Find Full Text PDFExperience with Pediatric Chronic Immune Thrombocytopenia over 30 Years in the Era before Eltrombopag.
Children (Basel)
August 2024
Department of Pediatric Hematology and Oncology, Istinye University, Istanbul 34320, Turkey.
Background: There is limited information on the natural course of chronic ITP in children. We aimed to evaluate the clinical and demographic characteristics of children with chronic ITP in the era before the availability of eltrombopag.
Methods: A total of 86 children with chronic ITP between 1978-2014 were included.
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