Acute Abdominal Manifestations in Sickle Cell Disease

Crastnopol, Philip; Stewart, Charles F.

doi:10.1001/archsurg.1949.01240041003001

Cited by 9 publications

(3 citation statements)

References 12 publications

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“…Its etiology has been attributed to a myriad of sources including mesenteric and retroperitoneal adenopathy, infarction in vertebral bodies, hepatobiliary disease and splenic infarction [5]. While the above may at some point in the natural history of the disease contribute to abdominal pain, it is more likely that intermittent ischemia, stemming from transient ‘low-flow’ states due to red blood cell deformity, is the primary source of the abdominal pain.…”

Section: Discussionmentioning

confidence: 99%

Small Bowel Ischemia in a Sickle Cell Patient

Abadin

Zhu²,

Connolly³

et al. 2009

Case Rep Gastroenterol

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Section: Discussionmentioning

confidence: 99%

Small Bowel Ischemia in a Sickle Cell Patient

Abadin

Zhu²,

Connolly³

et al. 2009

Case Rep Gastroenterol

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“…Although pancreatitis during childhood is not common, the high incidence of cholelithiasis in patients with SCD should be considered in the differential diagnosis of SCD with abdominal pain (4,9). Cholecystectomy is an appropriate treatment in symptomatic patients (2).…”

Section: Discussionmentioning

confidence: 99%

“…The common causes of abdominal pain include bone marrow hyperplasia, mesenteric and retroperitoneal lymphadenitis, ischemia and infarction of the abdominal or-gans such as the spleen, liver, small intestine, colon, and pancreas. Other causes include viral hepatitis, acute cholecystitis, cholelithiasis, and peptic ulcers (4). Available treatments include transfusions, hydroxycarbamide, stem cell transplantation, and several new therapeutic options such as gene therapy and gene editing in development (5,6).…”

Section: Introductionmentioning

confidence: 99%

Abdominal pain in a patient with sickle cell disease with multiple complications

Mehrabani¹

2019

Turk Pediatri Ars

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Sickle cell disease is an inherited autosomal recessive hemoglobinopathy. Acute abdominal pain is the cause of hospitalization in 10% of patients with sickle cell disease and usually occurs during vaso-occlusion or distal tissue ischemia. Determining the etiology of abdominal pain is very difficult in these patients because it is associated with several rare diagnoses, such as pancreatitis and splenic abscess in some patients. We represent a 14-year-old boy with sickle cell disease who was hospitalized due to acute abdominal pain and indicated multiple and scarce disturbances in the spleen and hepatobiliary system.

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Treatment of Sickle Cell Anemia

Leavell¹

1954

Arch Intern Med

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SICKLE cell anemia was described first by Herrick in 1910.1 Since then, surveys of Negroes in the United States have revealed the incidence of the symptomless sickle cell trait to be about 9% and incidence of sickle cell anemia to be about 2.25 per thousand.2 In the past decade sickle cell anemia has been the subject of many studies, which have contributed greatly to our understanding of many of the abnormalities that occur in the disease. Conclusions drawn from these studies form the basis for our current ideas of the pathogenesis of the disease. Since our present therapy is directed toward this aspect of the disease, the pathogenesis of sickle cell anemia will be reviewed briefly before treatment is discussed. It now seems well established that the sickling phenomenon is due to a gene which produces sickle cell anemia when it is present in the homozygous state and sickle cell trait when it occurs in the heterozygous state.* The erythrocytes in both conditions are abnormal and assume the characteristic "sickle shape" when the hemoglobin is in the reduced form.5 This characteristic of the cells has been shown to be due to hemoglobin that is different from the normal electrophoretically.f Erythrocytes in patients with sickle cell anemia contain only the abnormal "sickle cell" hemoglobin, while those of persons with the sickle cell trait contain a mixture of the abnormal "sickle cell" hemoglobin and normal hemoglobin. Studies of erythrocyte survival in vivo indicate that the increased hemolysis in sickle cell anemia is due to a defect in the erythrocytes, which have a survival time much shorter than normal, in contrast with those in sickle cell trait, which apparently have a normal life span.4; A hemolytic type of anemia occurs because hematopoiesis, although increased, is not able to compensate for the excessive erythrocyte destruc¬ tion. In many patients hemoglobin and erythrocyte levels remain essentially the same over a period of years because the life span of the red cells and the maximum compensatory effort that the bone marrow is able to make remain unchanged. § On occasions this balance is upset, and the anemia rapidly becomes much more severe, either as a result of increased hemolysis or as a consequence of the develop¬ ment of a temporary aplastic state in the bone marrow.15 Aside from the sickling property of the erythrocytes and the presence of anemia, probably the most charac¬ teristic feature of sickle cell anemia is the occurrence of sudden attacks of pain or other localized manifestations in almost any part of the body. These episodes appear to be explained by the capillary engorgement and areas of infarction that are found

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Acute Abdominal Manifestations in Sickle Cell Disease

Cited by 9 publications

References 12 publications

Small Bowel Ischemia in a Sickle Cell Patient

Small Bowel Ischemia in a Sickle Cell Patient

Abdominal pain in a patient with sickle cell disease with multiple complications

Treatment of Sickle Cell Anemia

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