Background: The high prevalence and heterogeneity of clinical and haematological phenotypes of sickle-cell anaemia and thalassaemia intermedia (TI) syndromes in the Greek population constitute a major challenge in routine medical practice.
Purpose: To investigate the spectrum and prevalence of abdominal US findings in these patients.
Materials And Methods: The study included 105 patients, 13 homozygous for sickle-cell anaemia (S/S) and 92 with TI syndromes (Th/S, beta(thal)/beta(thal )or beta(thal)/deltabeta(thal)). The patients' ages ranged from 1 to 54 years, with 49 males and 56 females.
Results: At the time of the examination, 41% of the patients had undergone splenectomy and 23.8% had undergone cholecystectomy. The most frequent US findings (percentages refer to all patients) were: hepatomegaly (70.5%), splenomegaly (48.6%) and cholelithiasis (20%). A bright liver was identified in 3.8% and focal hepatic lesions were present in two patients of the TI group. Functional disorders of the gallbladder concerned mainly patients with sickle-cell syndromes. Of those with S/S, 30.8% had a shrunken spleen. An echogenic pancreas was identified in one patient in each group. Increased renal echogenicity was observed in 17.6% of patients with sickle-cell syndromes. One case of polycystic kidney disease was diagnosed and coexisted with S/S.
Conclusions: The value of US imaging in detecting complications of sickle-cell and TI syndromes is still debated. To our knowledge, there are no previous reports concerning the frequency and spectrum of abdominal US findings in patients with these syndromes.
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http://dx.doi.org/10.1007/s00247-003-0950-5 | DOI Listing |
Mediterr J Hematol Infect Dis
January 2025
Center for Human Genetics, Faculty of Medicine, KU Leuven, Leuven, Belgium.
Mediterr J Hematol Infect Dis
January 2025
Pediatric Intensive Care Unit, Hospital Professor Doutor Fernando Fonseca, Unidade Local de Saúde Amadora/Sintra, Portugal.
Background: Red Blood Cell Exchange (RBCX) is a common treatment for pediatric sickle cell disease (SCD). Since inflammation with elevated proinflammatory cytokines plays a crucial role in SCD, this study hypothesized that RBCX might lower these cytokines and aimed to assess the impact of this technique on these markers.
Methods: Prospective and observational study of pediatric SCD patients (HbSS genotype) enrolled in a chronic RBCX program at a Portuguese Hospital from October 2022 to August 2024.
Ann Hematol
January 2025
Department of Internal Medicine, Section of Hematology/Oncology, University of Missouri-Kansas City, Kansas City, MO, 64108, USA.
Sickle cell disease (SCD) is an inherited hematologic disease caused by sickle hemoglobin as the predominant RBC hemoglobin or by sickle hemoglobin in combination with other abnormal β-hemoglobin variants like HbC, HbD and others. Sickling of erythrocytes under deoxygenated conditions is the basis of inflammatory and thrombotic cascades which result in multiple serious complications, leading to early morbidity and mortality. While HLA-matched allogeneic bone marrow transplantation is potentially curative, it has considerable limitations due to potential severe toxicities.
View Article and Find Full Text PDFJ Pain
January 2025
Center for Child Health, Behavior and Development, Seattle Children's Research Institute, Seattle, Washington, USA; Department of Anesthesiology & Pain Medicine, University of Washington, Seattle, Washington, USA.
Treatment expectations (TE) are predictive of patient outcomes in clinical practice and suggested to moderate treatment responses in chronic pain clinical trials. However, evidence is mainly derived from studies conducted with adult populations with musculoskeletal pain, primarily focused on pharmacological treatments and a few alternative intervention modalities (e.g.
View Article and Find Full Text PDFAnn Diagn Pathol
January 2025
Stanford Medical Center; Stanford, CA, United States of America.
Gamna-Gandy (GG) bodies are sclerosiderotic nodules composed of iron pigment and calcium, that have been described predominantly in the spleens of patients with sickle cell disease. Their formal depiction in the kidney is mainly limited to case reports and small series. We aimed to investigate the incidence of GG bodies and associated clinicopathologic features in consecutive nephrectomies performed for renal tumors.
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