Morphological features of the human umbilical vein in normal, sickle cell trait, and sickle cell disease pregnancies

Decastel, Monique; Leborgne-Samuel, Yannick; Alexandre, L.; Mérault, G.; Berchel, C

doi:10.1016/s0046-8177(99)90294-0

Cited by 23 publications

(17 citation statements)

References 18 publications

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“…However, there also is small vessel disease in the sickle brain (106,141,144). Moreover, similar pathologic changes have been observed in lung (68), umbilical cord (39), spleen (37,104), penis (70), and perhaps kidney (102). Correspondingly, these organs show clinical disease: stroke (141), pulmonary hypertension (30), fetal growth retardation and mortality (107), autosplenectomy (41), priapism (70), and renal failure (102), respectively.…”

Section: Chronic Vasculopathysupporting

confidence: 59%

The Endothelial Biology of Sickle Cell Disease: Inflammation and a Chronic Vasculopathy

2004

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A single amino acid substitution in hemoglobin comprises the molecular basis for sickle cell anemia, but evolution of the corresponding clinical disease is extraordinarily complicated and likely involves multiple pathogenic factors. Sickle disease is fundamentally an inflammatory state, with activation of the endothelium, probably through proximate effects of reperfusion injury physiology and chronic molestation by adherent red cells and white cells. The disease also involves enhanced angiogenic propensity, activation of coagulation, disordered vasoregulation, and a component of chronic vasculopathy. Sickle cell anemia is truly an endothelial disease, and it is likely that genetic differences in endothelial function help govern its astonishing phenotypic diversity.

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Section: Chronic Vasculopathysupporting

confidence: 59%

The Endothelial Biology of Sickle Cell Disease: Inflammation and a Chronic Vasculopathy

2004

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“…After nucleation, which depends on the 10th power of HbS concentration (i.e., an increase from 86 to 87 g=L would result in a more than a 12% increase of the effective concentration), fibrillar strands are formed that have been clearly demonstrated in the present case. Similar light and electron microscopic studies of sickle cells are known [9][10][11][12][13][14]. It is obvious that in the present case, anesthesia and=or surgery caused a fatal reduction of the ratio of HbS gelation delay time=capillary transit time of erythrocytes.…”

Section: Discussionsupporting

confidence: 75%

“…In addition to the classical vasoocclusion model of sickle cell anemia [5,13], recent studies revealed additional mechanisms of disturbed organ perfusion, especially under decreasing oxygen saturation. These include higher sickle cell adhesion to endothelial cells, higher viscosity and lower deformability of sickle cell membranes, and altered intracellular viscosity of the affected cells.…”

Section: Discussionmentioning

confidence: 99%

Sickle Cell Anemia: Conclusions from a Forensic Case Report of a Young African Woman Who Died After Anesthesia

Hammer

Wegener

Nizze

et al. 2006

Ultrastructural Pathology

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A 20-year old African woman underwent anesthesia for interruption of an unwanted pregnancy. As a consequence of the anesthesia, she went into coma because of an as yet unknown and untested homozygotic state of sickle cell anemia. Her vital functions were maintained for more than 1 year by intensive medicine, but she died finally in multiorgan failure and aspiration pneumonia. Because of the complications under anesthesia and the missing preanesthetic test for hemoglobinopathy, autopsy was conducted in the forensic medicine department and not in the department of pathology. The sickle cell disease was diagnosed by electrophoresis of the blood, by molecular detection of mutation in the hemoglobin gene, as well as by postmortem light and electron microscopy. Sickle cells were found in capillaries of brain, liver, lung, bone marrow, and spleen. Electrophoretic analysis revealed 80.2% HbS in addition to 3.2% HbA2 and 16.6% HbF, whereas no HbA0 could be detected in blood, confirming the homozygosity of sickle cell anemia. Because of sickle cell crisis, occluded blood vessels, and severe brain cortex necrosis, the patient died in spite of reanimation and intensive medicine. This case demonstrates that it is still important to realize the possibility of this disease and diagnostic obstacles even in regions where its manifestation is not endemic, as in Northern and Central Europe.

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“…38 Placental dysfunction in this setting is likely a consequence of both vaso-occlusion and impaired placental development, 4,39 creating a condition of relative fetal hypoxia. 40,41 The lack of impact of prophylactic transfusion on fetal growth restriction and preeclampsia demonstrated in this systematic review may potentially be explained by the circumstances under which prophylactic transfusion was instituted. The wide variation among studies in the timing of prophylactic transfusion commencement is of specific relevance, given that aberrant early placentation is typically the pathophysiologic origin of these conditions.…”

Section: Discussionmentioning

confidence: 95%

Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis

Malinowski

Shehata

D’Souza

et al. 2015

Blood

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Key Points• Prophylactic transfusion in pregnant women with SCD may reduce maternal mortality, vaso-occlusive pain events, and pulmonary complications.• Prophylactic transfusion in pregnant women with SCD may similarly reduce perinatal mortality, neonatal death, and preterm birth.Pregnancy in women with sickle cell disease is associated with adverse maternal and neonatal outcomes. Studies assessing the effects of prophylactic red blood cell transfusions on these outcomes have drawn inconsistent conclusions. The objective of this systematic review was to assess the effect of prophylactic compared with on-demand red blood cell transfusions on maternal and neonatal outcomes in women with sickle cell disease. A systematic search of several medical literature databases was conducted. Twelve studies involving 1291 participants met inclusion criteria. , and preterm birth (9 studies, 1123 participants; OR, 0.59; 95% CI, 0.37-0.96). Event rates for most of the results were low. Prophylactic transfusions may positively impact several adverse maternal and neonatal outcomes in women with sickle cell disease; however, the evidence stems from a relatively small number of studies with methodologic limitations. A prospective, multicenter, randomized trial is needed to determine whether the potential benefits balance the risks of prophylactic transfusions. (Blood. 2015;126(21):2424-2435

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Morphological features of the human umbilical vein in normal, sickle cell trait, and sickle cell disease pregnancies

Cited by 23 publications

References 18 publications

The Endothelial Biology of Sickle Cell Disease: Inflammation and a Chronic Vasculopathy

The Endothelial Biology of Sickle Cell Disease: Inflammation and a Chronic Vasculopathy

Sickle Cell Anemia: Conclusions from a Forensic Case Report of a Young African Woman Who Died After Anesthesia

Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis

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