chronic pulmonary complications of sickle cell disease

Sickle cell disease (SCD) is the most common monogenic disease worldwide, with a variable prevalence in each continent. A single nucleotide substitution leads to an amino-acid change in the β-globin chain, altering the normal structure ofhemoglobin, which is then called hemoglobin S inherited in homozygosity (HbSS) or double heterozygosity (HbSC, HbSβ), and leads to chronic hemolysis, vaso-occlusion, inflammation, and endothelium activation. Pregnant women with SCD are at a higher risk of developing maternal and perinatal complications...

Sickle cell disease is a disorder characterized by chronic hemolytic anemia and multiorgan disease complications. Although vaso-occlusive episodes, acute chest syndrome, and neurovascular disease frequently result in complication and have well-documented guidelines for management, the management of chronic hemolytic and vascular-related complications, such as priapism, leg ulcers, and pulmonary hypertension, is not as well recognized despite their increasing reported prevalence and association with morbidity and mortality...

Both Sickle cell disease (SCD) and Tuberculosis (TB) are prevalent, and mostly concentrated in similar geographic areas. Since there is an overlap of the possible presenting symptoms of SCD complications and TB, a low threshold for suspicion of TB in these patients is recommended. We present a case of an active TB infection in a SCD patient that presented mainly with chronic cough.

Sickle leg ulcer (SLU) occurs as a result chronic occlusion of the vasculature with consequent necrosis of the skin and subcutaneous tissue usually in proximity of the malleoli. The description of clinical associations and the simultaneous occurrence of SLU and other complications of SCD compared to the non-SLU patients was the aim of this work. A total of 272 (60.8% males and 39.2% females) patients were captured during this time period out of which 68 (51 males and 17 females) had SLU out of whom 20 patients had bilateral leg ulcers...

Background: Although obstructive sleep apnea (OSA) could act as a modulator of clinical severity in sickle cell disease (SCD), few studies focused on the associations between the two diseases. Research Question: The aims of this study were: (1) to explore the associations between OSA, nocturnal oxyhemoglobin saturation (SpO2) and the history of several acute/chronic complications, (2) to investigate the impact of OSA and nocturnal SpO2 on several biomarkers (hematological, blood rheological, and coagulation) in patients with SCD...

Chronic hypoxia is a major driver of cardiovascular complications, including heart failure. The nitric oxide (NO) - soluble guanylyl cyclase (sGC) - cyclic guanosine monophosphate (cGMP) pathway is integral to vascular tone maintenance. Specifically, NO binds its receptor sGC within vascular smooth muscle cells (SMC) in its reduced heme (Fe2+ ) form to increase intracellular cGMP production, activate protein kinase G (PKG) signaling, and induce vessel relaxation. Under chronic hypoxia, oxidative stress drives oxidation of sGC heme (Fe2+ →Fe3+ ), rendering it NO-insensitive...

BACKGROUND: Sickle cell anaemia affects about 4 million people across the globe, making it an inherited disorder of public health importance. Red cell lysis consequent upon haemoglobin crystallization and repeated sickling leads to anaemia and a baseline strain on haemopoiesis. Vaso-occlusion and haemolysis underlies majority of the chronic complications of sickle cell. We evaluated the clinical and laboratory features observed across the various clinical phenotypes in adult sickle cell disease patients...

Background Sickle cell disease (SCD) is an autosomal recessive disease resulting in hemolytic anemia and recurrent vaso-occlusive events. Consequently, it can result in a broad range of functional and structural renal and cardiac alterations. Chronic kidney disease (CKD), in SCD, is associated with proteinuria, microalbuminuria, and hemoglobinuria. Cardiac complications in SCD include pulmonary hypertension, left ventricular diastolic heart disease, dysrhythmia, and sudden death. In patients with advancing age, cardio-renal dysfunction can have substantial effects on morbidity and mortality...

Sickle cell disease (SCD), a haemoglobinopathy characterized by chronic haemolysis with acute exacerbations and vascular occlusion episodes, may be complicated by pulmonary hypertension. The latter may be caused by chronic thromboembolic disease of pulmonary artery branches and its management is not well-defined. Herein, we present a case of SCD complicated by chronic thromboembolic pulmonary hypertension of subsegmental pulmonary artery branches successfully treated with endothelin receptor antagonists, orally administered prostacyclin analogs and balloon pulmonary angioplasty...

Red cell transfusion represents one of the cornerstones of the chronic management of sickle cell disease, as well as its acute complications. Automated red cell exchange can rapidly lower the number of circulating sickle erythrocytes, without causing iron overload. Here, we describe our experience, having offered this intervention since 2011. A transient reduction in the platelet count by 61% was observed after the procedure. This was not associated with any haemorrhagic complications. Despite exposure to large volumes of blood, the alloimmunisation rate was only 0...

SARS-CoV-2 or COVID-19 is a novel coronavirus, which is the cause of the current pandemic with 107,411,561 infections and 2,351,195 death worldwide so far. There are multiple symptoms that are linked with the infection of COVID-19 such as coughing, shortness of breath, congestion together with fatigue, fever, loss of taste or smell, headaches, diarrhea, vomiting, and loss of appetite. The lack of or early stage of development of a cure for COVID-19 illness, there is need for insuring the best possible position of health to be able to fight the virus naturally through a robust immune system to limit severe complication...

Non-myeloablative haematopoietic progenitor cell transplantation (HPCT) from matched related donors (MRD) has been increasingly utilized in sickle cell disease (SCD). A total of 122 patients received 300 cGy of total body irradiation (TBI), alemtuzumab, unmanipulated filgrastim-mobilized peripheral blood HPC and sirolimus. The median follow-up was four years; median age at HPCT was 29 years. Median neutrophil and platelet engraftment occurred on day 22 and 19 respectively; 41 patients required no platelet transfusions...

BACKGROUND: The most commonly isolated organisms in a parapneumonic effusion include S. pneumoniae, H. influenzae, and S. aureus. If unusual organisms are isolated from the pleural space, further investigation is warranted to locate the primary source. We present a patient with an infected chronic renal cyst found to have an empyema secondary to Proteus mirabilis to highlight the importance of further diagnostic workup when encountering unusual organisms in the pleural space. CASE PRESENTATION: A 40-year-old African-American female, with a past medical history of asthma and sickle cell trait, presented with 5 weeks of upper respiratory tract symptoms and chest pain...

STUDY OBJECTIVE: We provide an updated assessment of trends in sickle cell disease (SCD)-related mortality, a significant source of mortality in the United States among black persons, using 1979 to 2017 US mortality data. METHODS: SCD-related deaths were identified with International Classification of Diseases codes. Because SCD-related death is rare in other races, the analysis focused on black decedents. Age-specific and average annual SCD-related death rates were calculated...

This is a descriptive cross-sectional unicentric study, with a prospective collection of data on the frequency of chronic complications of sickle cell disease in patients monitored at Souro Sanou University Hospital in Bobo-Dioulasso in the department of medicine conducted from April 1, 2017 to July 31, 2018. Patients with confirmed adult sickle cell disease, at least 16 years of age, who had given oral consent, was seen at least twice in the inter-critical period during the study, and who had performed a biological and / or radiological screening for chronic complications...

Sickle cell disease (SCD) is a life-threatening hereditary blood disorder that affects millions of people worldwide, especially in sub-Saharan Africa. This condition has a multi-organ involvement and highly vascularised organs, such as the lungs, are particularly affected. Chronic respiratory complications of SCD involve pulmonary vascular, parenchymal and airways alterations. A progressive decline of lung function often begins in childhood. Asthma, sleep-disordered breathing and chronic hypoxaemia are common and associated with increased morbidity...

BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...

According to a widespread theory, thrombotic masses are not formed in the pulmonary artery (PA) but result from migration of blood clots from the venous system. This concept has prevailed in clinical practice for more than a century. However, a new technologic era has brought forth more diagnostic possibilities, and it has been shown that thrombotic masses in the PA could, in many cases, be found without any obvious source of emboli. Chronic obstructive pulmonary disease, asthma, sickle cell anemia, emergency and elective surgery, viral pneumonia, and other conditions could be complicated by PA thrombosis development without concomitant deep vein thrombosis (DVT)...

As the COVID-19 pandemic continues to claim lives across the globe, insufficient data exists regarding the optimal treatment. It is well known that patients 55 years of age or older and patients with certain chronic diseases are at higher risk of severe illness, including acute respiratory distress syndrome and death. A potentially fatal pulmonary complication of sickle cell disease, acute chest syndrome, can be precipitated by acute infections, including respiratory viruses. We report the case of a patient with sickle cell disease (HbSC) who developed COVID-19 pneumonia and acute chest syndrome who was treated with emergent red blood cell exchange in order to avoid endotracheal intubation...

BACKGROUND: The management of sickle cell disease (SCD), an inherited, chronic, and multifaceted condition, is associated with considerable health care resource utilization (HRU) and costs, especially for Medicaid. Anemia affects most patients with SCD and correlates with end-organ damage (EOD), such as stroke, chronic kidney disease (CKD), end-stage renal disease (ESRD), and pulmonary hypertension (PH). Limited research has been conducted to quantify the economic burden of EOD among patients with SCD...