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About 1,073 results

ALLMedicine Hereditary Spherocytosis Center

Research & Reviews  533 results

Genotype-phenotype correlation in children with hereditary spherocytosis.
https://doi.org/10.1111/bjh.16750
British Journal of Haematology References; Tole S, Dhir P et. al.

May 19th, 2020 - Hereditary spherocytosis (HS) is a common inherited haemolytic anaemia attributed to disturbances in five different red cell membrane proteins. We performed a retrospective study of 166 children with HS and describe the clinical phenotype accordin...

Erythrocyte deformability reduction in various pediatric hematologic diseases.
https://doi.org/10.3233/CH-200817
Clinical Hemorheology and Microcirculation; Kim YK, Lim YT et. al.

Mar 31st, 2020 - Previously, hemorheology studies using Rheoscan mainly focused on chronic kidney disease, cardiovascular disease, and endocrine disease in adults. The study using LORCA focused on erythrocyte disease. There were no studies using Rheoscan in childr...

Long-Term Evaluation of the Outcomes of Subtotal Laparoscopic and Robotic Splenectomy i...
https://doi.org/10.1007/s00268-020-05485-3
World Journal of Surgery; Manciu S, Nae GA et. al.

Mar 25th, 2020 - Hereditary spherocytosis (HS) is a common inherited disease affecting the erythrocyte membrane. Total splenectomy (TS) is effective in reducing hemolysis and decreasing the need of transfusions, but total removal of the spleen represents a potenti...

Beta-Spectrin Deletion Responsible for Hereditary Spherocytosis: When New Technologies ...
https://doi.org/10.1097/MPH.0000000000001742
Journal of Pediatric Hematology/oncology; Panizo Morgado E, Darnaude MT et. al.

Feb 18th, 2020 - Hereditary spherocytosis arises from alterations in the genes encoding red blood cell membrane proteins. Although its diagnosis is mostly clinical, recent advances in next-generation sequencing (NGS) technologies have allowed for a faster cost-eff...

Successful Splenectomy Management in a Patient With Moderate Factor VII Deficiency and ...
https://doi.org/10.1097/MPH.0000000000001738
Journal of Pediatric Hematology/oncology; Goudarzipour K, Amiri V et. al.

Feb 7th, 2020 - By the advent of the effective therapies for many coagulation diseases and hereditary spherocytosis (HS), patient's survival has been improved significantly; however, if patients are diagnosed late or left untreated, both diseases could ominously ...

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Guidelines  2 results

ICSH guidelines for the laboratory diagnosis of nonimmune hereditary red cell membrane ...
https://doi.org/10.1111/ijlh.12335
International Journal of Laboratory Hematology; King MJ, Garçon L et. al.

Mar 19th, 2015 - Hereditary spherocytosis (HS), hereditary elliptocytosis (HE), and hereditary stomatocytosis (HSt) are inherited red cell disorders caused by defects in various membrane proteins. The heterogeneous clinical presentation, biochemical and genetic ab...

Guidelines for the diagnosis and management of hereditary spherocytosis--2011 update.
https://doi.org/10.1111/j.1365-2141.2011.08921.x
British Journal of Haematology; Bolton-Maggs PH, Langer JC et. al.

Nov 7th, 2011 - Guidelines on hereditary spherocytosis (HS) published in 2004 (Bolton-Maggs et al, 2004) are here replaced to reflect changes in current opinion on the surgical management, (particularly the indications for concomitant splenectomy with cholecystec...

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Clinicaltrials.gov  535 results

Genotype-phenotype correlation in children with hereditary spherocytosis.
https://doi.org/10.1111/bjh.16750
British Journal of Haematology References; Tole S, Dhir P et. al.

May 19th, 2020 - Hereditary spherocytosis (HS) is a common inherited haemolytic anaemia attributed to disturbances in five different red cell membrane proteins. We performed a retrospective study of 166 children with HS and describe the clinical phenotype accordin...

Erythrocyte deformability reduction in various pediatric hematologic diseases.
https://doi.org/10.3233/CH-200817
Clinical Hemorheology and Microcirculation; Kim YK, Lim YT et. al.

Mar 31st, 2020 - Previously, hemorheology studies using Rheoscan mainly focused on chronic kidney disease, cardiovascular disease, and endocrine disease in adults. The study using LORCA focused on erythrocyte disease. There were no studies using Rheoscan in childr...

Long-Term Evaluation of the Outcomes of Subtotal Laparoscopic and Robotic Splenectomy i...
https://doi.org/10.1007/s00268-020-05485-3
World Journal of Surgery; Manciu S, Nae GA et. al.

Mar 25th, 2020 - Hereditary spherocytosis (HS) is a common inherited disease affecting the erythrocyte membrane. Total splenectomy (TS) is effective in reducing hemolysis and decreasing the need of transfusions, but total removal of the spleen represents a potenti...

Beta-Spectrin Deletion Responsible for Hereditary Spherocytosis: When New Technologies ...
https://doi.org/10.1097/MPH.0000000000001742
Journal of Pediatric Hematology/oncology; Panizo Morgado E, Darnaude MT et. al.

Feb 18th, 2020 - Hereditary spherocytosis arises from alterations in the genes encoding red blood cell membrane proteins. Although its diagnosis is mostly clinical, recent advances in next-generation sequencing (NGS) technologies have allowed for a faster cost-eff...

Successful Splenectomy Management in a Patient With Moderate Factor VII Deficiency and ...
https://doi.org/10.1097/MPH.0000000000001738
Journal of Pediatric Hematology/oncology; Goudarzipour K, Amiri V et. al.

Feb 7th, 2020 - By the advent of the effective therapies for many coagulation diseases and hereditary spherocytosis (HS), patient's survival has been improved significantly; however, if patients are diagnosed late or left untreated, both diseases could ominously ...

see more →

News  3 results

Blueberry Muffin Rash Secondary to Hereditary Spherocytosis
https://www.mdedge.com/dermatology/article/157898/pediatrics/blueberry-muffin-rash-secondary-hereditary-spherocytosis/page/0/1

Feb 5th, 2018 - Comment Dermal extramedullary hematopoiesis is a normal component of embryologic development up until the fifth month of gestation. 1 The term blueberry muffin rash typically is used to describe the cutaneous manifestations of extramedullary hemato.

Darier Disease: Sustained Improvement Following Reduction Mammaplasty
https://www.mdedge.com/dermatology/article/66988/darier-disease-sustained-improvement-following-reduction-mammaplasty
Cohen PR

Darier disease is an autosomal-dominant inherited genodermatosis. A woman is described who had Darier disease and hereditary spherocytosis whose inframammary Darier disease had dramatic and sustained improvement following reduction mammaplasty.

Hereditary Spherocytosis with Leg Ulcers Healing after Splenectomy
https://www.medscape.com/viewarticle/736752

Abstract and Introduction Abstract Hereditary spherocytosis (HS) is a familial hemolytic disorder with marked heterogeneity. A refractory chronic leg ulcer is an uncommon complication of HS, reported in fewer than two percent of patients. We prese...

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