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Indian Journal of Pediatrics Dec 1971
Topics: Child; Humans; Male; Spherocytosis, Hereditary
PubMed: 5144579
DOI: 10.1007/BF02813177 -
Pediatric Blood & Cancer Oct 2016To compare the clinical effectiveness of total splenectomy (TS) or partial splenectomy (PS) in pediatric hereditary spherocytosis, a systematic review and meta-analysis... (Comparative Study)
Comparative Study Meta-Analysis Review
To compare the clinical effectiveness of total splenectomy (TS) or partial splenectomy (PS) in pediatric hereditary spherocytosis, a systematic review and meta-analysis was performed (PROSPERO registration CRD42015030056). There were 14 observational studies comparing pre- and postoperative hematologic parameters. Secondary outcomes include in-hospital infections, surgical complications, symptomatic recurrence, and biliary disease. TS is more effective than PS to increase hemoglobin (3.6 g/dl vs. 2.2 g/dl) and reduce reticulocytes (12.5% vs. 6.5%) after 1 year; outcomes following PS are stable for at least 6 years. There were no cases of overwhelming postsplenectomy sepsis. A population-based patient registry is needed for long-term follow-up.
Topics: Child; Humans; Publication Bias; Quality of Life; Reticulocyte Count; Spherocytosis, Hereditary; Splenectomy
PubMed: 27300151
DOI: 10.1002/pbc.26106 -
Haematologica Sep 2008
Topics: Genotype; Humans; Phenotype; Spherocytosis, Hereditary
PubMed: 18757847
DOI: 10.3324/haematol.13344 -
The Journal of the Oklahoma State... Dec 1963
Topics: Adolescent; Geriatrics; Humans; Infant; Spherocytosis, Hereditary; Splenectomy
PubMed: 14086192
DOI: No ID Found -
New Jersey Medicine : the Journal of... Feb 1994We report the development of myeloproliferative disorders in two patients with hereditary spherocytosis diagnosed in adulthood. Eight previously reported cases of the... (Review)
Review
We report the development of myeloproliferative disorders in two patients with hereditary spherocytosis diagnosed in adulthood. Eight previously reported cases of the coexistence of hereditary spherocytosis and hematologic malignancy are reviewed.
Topics: Humans; Leukemia; Male; Middle Aged; Myeloproliferative Disorders; Neoplasms; Spherocytosis, Hereditary
PubMed: 8164881
DOI: No ID Found -
British Journal of Haematology Jun 2009Splenectomy is indicated in hereditary spherocytosis to relieve symptoms due to anaemia or splenomegaly, reverse growth failure or skeletal changes due to over-robust... (Review)
Review
Splenectomy is indicated in hereditary spherocytosis to relieve symptoms due to anaemia or splenomegaly, reverse growth failure or skeletal changes due to over-robust erythropoiesis, and prevent recurrent gallstones. A life-long risk of bacterial infection has been recognised for many years as a concomitant cost of splenectomy. The scope of this risk has expanded to include a number of organisms beyond the triad of pneumococcus, meningococcus, and haemophilus influenzae. Recently, it has been demonstrated that splenectomy also confers a significant risk of delayed adverse vascular events in patients with hereditary spherocytosis, just as it does in patients undergoing splenectomy for other indications. Further, these same studies demonstrated a benefit of avoiding splenectomy: hereditary spherocytosis patients with a spleen have significantly fewer adverse vascular events than unaffected family members, probably because of the protective effect of chronic, mild anaemia. Accordingly, this review marshals the evidence favouring a conservative approach to splenectomy in spherocytosis.
Topics: Adult; Bacterial Infections; Child; Humans; Myocardial Infarction; Patient Selection; Risk; Spherocytosis, Hereditary; Splenectomy; Treatment Outcome
PubMed: 19388926
DOI: 10.1111/j.1365-2141.2009.07694.x -
Acta Dermato-venereologica Nov 2021
Review
Topics: Anemia, Hemolytic; Humans; Pyoderma Gangrenosum; Spherocytosis, Hereditary; Splenectomy
PubMed: 34694421
DOI: 10.2340/actadv.v101.438 -
Pediatric Blood & Cancer Jan 2021
Topics: COVID-19; Child, Preschool; Erythrocyte Transfusion; Hemolysis; Humans; Male; SARS-CoV-2; Spherocytosis, Hereditary
PubMed: 32710684
DOI: 10.1002/pbc.28548 -
The Turkish Journal of Pediatrics 2018Güngör A, Yaralı N, Fettah A, Ok-Bozkaya İ, Özbek N, Kara A. Hereditary spherocytosis: Retrospective evaluation of 65 children. Turk J Pediatr 2018; 60: 264-269....
Güngör A, Yaralı N, Fettah A, Ok-Bozkaya İ, Özbek N, Kara A. Hereditary spherocytosis: Retrospective evaluation of 65 children. Turk J Pediatr 2018; 60: 264-269. Hereditary spherocytosis (HS) is a common cause of congenital hemolytic anemia in Caucasians and it could be diagnosed at any age. The aim of this study is to examine the demographic characteristics, clinical features and laboratory findings of children with HS and their complications observed during follow up. Sixty-five patients, with hereditary spherocytosis between January 2008 and September 2013, were enrolled into this retrospective study. The age of patients at the time of diagnosis varied between 15 days and 17 years. The median age of patients at diagnosis was 48 months (IQR 2-78). The female/male ratio was 1.1. Forty-seven patients (72.3%) had a family history of HS. The patients were classified according to laboratory findings: 13 of them (20%) were diagnosed as mild HS, 36 (55.4%) as moderate HS and of 16 (24.6%) as severe HS. During follow-up, nine patients (13.8%) experienced an aplastic crisis. Megaloblastic crisis was not observed in any patient. Twenty patients (30.8%) had cholelithiasis. Splenectomy was performed in 20% of patients and the mean age for splenectomy was 8.3 years. Complications such as sepsis or thrombosis were not detected after splenectomy. Hereditary spherocytosis should be kept in mind in patients with anemia, jaundice and splenomegaly and the family history must be questioned. The most common complication was gallstone; even patients without severe hemolysis should be followed intermittently by abdominal ultrasonography in order to control the development of gallstone.
Topics: Adolescent; Blood Cell Count; Child; Child, Preschool; Erythrocyte Transfusion; Female; Humans; Infant; Infant, Newborn; Male; Retrospective Studies; Spherocytosis, Hereditary; Splenectomy; Ultrasonography
PubMed: 30511538
DOI: 10.24953/turkjped.2018.03.005 -
The Western Journal of Medicine Jan 1978
Review
Topics: Disease Outbreaks; Erythrocytes, Abnormal; Hemolysis; Humans; Osmotic Fragility; Spherocytosis, Hereditary; Spleen; Splenectomy; Virus Diseases
PubMed: 343392
DOI: No ID Found