scholarly journals Sickle Cell Disease-Induced Pulmonary Hypertension: A Review of Pathophysiology, Management, and Current Literature

Pulse ◽  
2021 ◽  
pp. 1-7
Author(s):  
Abu Baker Sheikh ◽  
Adeel Nasrullah ◽  
Erick Daniel Lopez ◽  
Mian Tanveer Ud Din ◽  
Shazib Sagheer ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Treatment Options ◽  
Diagnostic Techniques ◽  
Prognostic Indicators ◽  
Cell Disease ◽  
Hemoglobin S ◽  
Individualized Care ◽  
Progression Of Disease

Sickle cell disease is an inherited hemoglobinopathy leading to the synthesis of hemoglobin S. Hemoglobin S results in the formation of abnormal sickle-shaped erythrocytes that lead to hematologic abnormalities such as hemolytic anemia and increased risks of thrombosis. Another particular problem encountered with the disease is pulmonary hypertension. The objective of this narrative review is to discuss the prevalence, pathophysiology mechanisms, diagnostic techniques, treatment options, and prognostic indicators in the setting of sickle cell disease with pulmonary hypertension. Additionally, the review also highlights other advancements that are being investigated. Considering the significant morbidity, mortality, and prevalence of pulmonary hypertension in patients with sickle cell disease, it is important to account for the aforementioned domains in the future guidelines to provide optimal and individualized care to the high-risk individuals as well as reduce the progression of disease, morbidity, and mortality rates.

Lead Neuropathy and Sickle Cell Disease

PEDIATRICS ◽  
1974 ◽  
Vol 54 (4) ◽  
pp. 438-441
Author(s):  
Gerald Erenberg ◽  
Steven S. Rinsler ◽  
Bernard G. Fish
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Lead Poisoning ◽  
Cell Disease ◽  
Hemoglobin S ◽  
Increased Risk ◽  
Rare Manifestation ◽  
Poisoning In Children

Four cases of lead neuropathy in children with hemoglobin S-S or S-C disease are reported. Neuropathy is a rare manifestation of lead poisoning in children, and only ten other cases have been well documented in the pediatric literature. The last previous case report of lead neuropathy was also in a child with hemoglobin S-S disease. The neuropathy seen in the children with sickle cell disease was clinically similar to that seen in the previously reported cases in nonsicklers, but differed in both groups from that usually seen in adult cases. It is, therefore, postulated that children with sickle cell disease have an increased risk of developing neuropathy with exposure to lead. The exact mechanism for this association remains unknown, but in children with sickle cell disease presenting with symptoms or signs of peripheral weakness, the possibility of lead poisoning must be considered.

scholarly journals Sickle Retinopathy in a Person with Hemoglobin S/New York Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Donovan Calder ◽  
Maryse Etienne-Julan ◽  
Marc Romana ◽  
Naomi Watkins ◽  
Jennifer M. Knight-Madden
Keyword(s):  
New York ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Laboratory Diagnosis ◽  
Compound Heterozygote ◽  
Cell Disease ◽  
Hemoglobin S

A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease. This recently reported form of sickle cell disease was not previously known to cause retinopathy and surprisingly was observed in a non-Asian individual. The ophthalmological findings, the laboratory diagnosis, and possible pathophysiology of this disorder are discussed. Persons diagnosed with sickle cell trait who present with symptoms of sickle cell disease may benefit from specific screening for this variant.

scholarly journals Pulmonary Hypertension of Sickle Cell Disease Beyond Classification Constraints

2014 ◽  
Vol 63 (25) ◽  
pp. 2881-2882 ◽  
Author(s):  
Elizabeth S. Klings ◽  
Claudia R. Morris ◽  
Lewis L. Hsu ◽  
Oswaldo Castro ◽  
Mark T. Gladwin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease
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