Spherocytosis, splenectomy, strokes, and heart attacks

Schilling, Robert F.

doi:10.1016/s0140-6736(05)64276-6

Cited by 94 publications

(82 citation statements)

References 4 publications

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“…The 147 subjects who had the spleen removed before age 40 were not considered in this report. A previous publication from this institution [15] demonstrates that subjects who have had a splenectomy for spherocytosis have a fivefold greater rate of AS events than those who have not had a splenectomy.…”

Section: Methodsmentioning

confidence: 88%

“…We hypothesized that the combination of lower cholesterol and lower whole blood viscosity found in anemia should result in a reduction of arteriosclerotic (AS) events in persons with hereditary spherocytosis who have not undergone splenectomy. Because of a long-term commitment to the care and study of hereditary spherocytosis patients [9][10][11][12][13][14][15], we have information about the medical history of a large number of family members.…”

Section: Introductionmentioning

confidence: 99%

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Arteriosclerotic events are less frequent in persons with chronic anemia: Evidence from families with hereditary spherocytosis

2006

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Because anemic persons have lower cholesterol and whole blood viscosity than those who are not anemic, we hypothesized that subjects with hereditary spherocytosis who have not had the spleen removed should have fewer arteriosclerotic events than unaffected family members. We defined arteriosclerotic events as myocardial infarct, stroke, coronary artery surgery, and carotid artery surgery. We compared the rate of these events in affected-not splenectomized persons to the rate in unaffected family members. The relative risk of an arteriosclerotic event in hereditary spherocytosis patients with a spleen was one fifth that in unaffected family members. These data support the hypothesis that chronic anemia retards the development of arteriosclerosis. Spherocytosis is another example of an inherited condition that conveys an advantage in one system and a disadvantage in another. Am. J. Hematol. 81:315-317, 2006. V V C 2006 Wiley-Liss, Inc.

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Section: Methodsmentioning

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Arteriosclerotic events are less frequent in persons with chronic anemia: Evidence from families with hereditary spherocytosis

2006

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“…This predicted value is higher than that of patients with a clinical diagnosis of Gilbert syndrome, reflecting the existence of other inherited or acquired factors affecting bilirubin metabolism, in addition to reduced glucuronidation caused by the (TA) insertion. The awareness that the (TA) insertion is the most frequent cause of Gilbert syndrome prompted the study of its association with other pathologies, namely h-thalassemia [22,23], sickle cell anemia [24], ABO incompatibility [25], glucose-6-phosphate dehydrogenase deficiency [26 -28], hereditary spherocytosis [29,30,31] and neonatal icterus [32,33].…”

Section: Discussionmentioning

confidence: 99%

Analysis of the UDP-glucuronosyltransferase gene in Portuguese patients with a clinical diagnosis of Gilbert and Crigler–Najjar syndromes

Costa

Vieira

Martins

et al. 2006

Blood Cells, Molecules, and Diseases

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We describe the molecular study in a cohort of 120 Portuguese patients with the clinical diagnosis of Gilbert syndrome and in one with the diagnosis of Crigler -Najjar syndrome type II, as well as a prenatal diagnosis of Crigler -Najjar syndrome type I. Among the 120 unrelated patients with Gilbert syndrome, 110 were homozygous for the [TA] . Additional studies in these 9 patients revealed heterozygosity for the c.674T>G, c.488 _ 491dupACCT and c.923G>A mutations, in 1, 1 and 4 patients, respectively. The patient with Crigler -Najjar syndrome type II was a compound heterozygote for [TA]7 and the c.923G>A mutation. The undocumented polymorphisms c.-1126C>T and c.997-82T>C were also detected in the course of this study. Prenatal diagnosis in a family with a boy previously diagnosed as Crigler -Najjar syndrome type I and homozygosity for the c.923G>A mutation revealed that the fetus was unaffected.Homozygosity for the [TA] insertion was found to be the most frequent cause of GS in our population. Identification of further mutations in the UGT1A1 gene was also seen to contribute significantly towards diagnosis. D

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“…However, with the better understanding of the important role of the spleen in immune defense, changes have taken place in the surgical strategy for splenic surgery. Several studies have indicated that total splenectomy could increase the risks of postoperative infection, secondary atherosclerosis events, pulmonary hypertension, and thrombocytic events [9,10] . Therefore, splenectomy should be avoided if possible.…”

Section: Discussionmentioning

confidence: 99%

Partial splenectomy using a laparoscopic bipolar radiofrequency device: A case report

Wang

Lin

et al. 2015

WJG

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We report a 51-year-old female patient with a solitary lymphangioma located in the upper splenic pole which was managed successfully with laparoscopic partial splenectomy. Surgery lasted 170 min and did not require blood transfusions. The patient recovered well post-operatively and was asymptomatic at the 3-mo follow-up. She had a normal platelet count and no recurrence on ultrasonography or computed tomography. Laparoscopic partial splenectomy is a safe, minimally invasive technique for the treatment of solitary splenic lymphangiomas in the splenic pole. We performed the procedure using the Habib TM 4X device. This laparoscopic bipolar radiofrequency device ensured a "bloodless" splenic parenchymal resection. Core tip:We report the first case of a solitary splenic lymphangioma managed successfully by laparoscopic partial splenectomy using Habib TM 4X, a laparoscopic bipolar radiofrequency device, which allowed for a "bloodless" splenic parenchymal resection.Wang WD, Lin J, Wu ZQ, Liu QB, Ma J, Chen XW. Partial splenectomy using a laparoscopic bipolar radiofrequency device: A case report.

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Spherocytosis, splenectomy, strokes, and heart attacks

Cited by 94 publications

References 4 publications

Arteriosclerotic events are less frequent in persons with chronic anemia: Evidence from families with hereditary spherocytosis

Arteriosclerotic events are less frequent in persons with chronic anemia: Evidence from families with hereditary spherocytosis

Analysis of the UDP-glucuronosyltransferase gene in Portuguese patients with a clinical diagnosis of Gilbert and Crigler–Najjar syndromes

Partial splenectomy using a laparoscopic bipolar radiofrequency device: A case report

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