scholarly journals Melioidosis and sickle cell disease: Description of a rare association

2020 ◽  
Vol 3 (1) ◽  
pp. 57-62
Author(s):  
Mick Ya-Pongombo Shongo ◽  
◽  
Mimi Mujing Yav ◽  
Olivier Mukuku ◽  
Gaston Kankolongo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Democratic Republic ◽  
Democratic Republic Of Congo ◽  
Central Africa ◽  
Cell Disease ◽  
Clinical Form ◽  
Rare Association ◽  
Wide Range ◽  
Clinical Cases

Melioidosis and its germ are increasingly reported on the African continent and particularly in Central Africa, probably due to the increased awareness of clinicians and microbiologists and the growing recognition of the organism. It is called "Great Mimicker" because it produces a wide range of clinical characteristics such as would be found in patients living with sickle cell disease (SCD) in particular. However, to date, no publication presents this association between melioidosis and SCD. The authors describe here 3 clinical cases presenting this very rare association between melioidosis and SCD. These are 3 children with SCD (homozygous SS) residing in Lubumbashi in Haut-Katanga province in the Democratic Republic of Congo. One patient presented with sepsis as a clinical form of the disease. All 3 had presented a pulmonary form. Only one patient was treated specifically after the diagnosis of melioidosis; for the other two, this diagnosis was confirmed after their death. Thus the death rate is 66.67%. This article describes, through these 3 clinical cases, a very rare first association between melioidosis and SCD. This association requires research to establish whether, like Thalassemia, SCD can be considered a risk factor for melioidosis. A screening of cases of melioidosis in the general population should allow us to focus on this.

scholarly journals Improvement of SCD morbimortality in children: experience in a remote area of an African country

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Benoît Mukinayi Mbiya ◽  
Didier Kalenda Kalombo ◽  
Yannick Nkesu Mukendi ◽  
Valery Daubie ◽  
John Kalenda Mpoyi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
African Country ◽  
Democratic Republic ◽  
Democratic Republic Of Congo ◽  
Public Health Problem ◽  
Cell Disease ◽  
Republic Of Congo ◽  
Reference Center

Abstract Background Sickle cell disease (SCD) is a public health problem in the Democratic Republic of Congo. While reference sickle cell centers have been implemented in capital cities of African countries and have proven to be beneficial for SCD patients. In the Democratic Republic of Congo, they have never been set up in remote areas for families with low or very low sources of income. Method A cohort of 143 children with SCD aged 10 years old (IQR (interquartile range): 6–15 years) (sex ratio male/female = 1.3) were clinically followed for 12 months without any specific intervention aside from the management of acute events, and then for 12 months with a monthly medical visit, biological follow-up, and chemoprophylaxis (folic acid/penicillin), adequate fluids and malaria prevention. Results The median age of patients at the diagnosis of SCD was 2 years (IQR: 1–5). The implementation of standardized and regular follow-ups in a new sickle cell reference center in a remote city showed an increase in the annual mean hemoglobin level from 50 to 70 g/L (p = 0.001), and a decrease in the lymphocyte count and spleen size (p < 0.001). A significant decrease (p < 0.001) in the average annual number of hospitalizations and episodes of vaso-occlusive crises, blood transfusions, infections, and acute chest syndromes were also observed. Conclusions The creation of a sickle cell reference center and the regular follow-up of children with sickle cell disease are possible and applicable in the context of a remote city of an African country and represent simple and accessible measures that can reduce the morbimortality of children with sickle cell disease.

Barriers to healthcare for sickle cell disease patients in the Democratic Republic of Congo

2017 ◽  
Vol 1 (1) ◽  
pp. 3-9
Author(s):  
Salomon Agasa Batina ◽  
◽  
Paul Kombi Kambale ◽  
Marcel Poyo Sabiti ◽  
Charles Tshilumba Kayembe ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Democratic Republic ◽  
Democratic Republic Of Congo ◽  
Cell Disease ◽  
Republic Of Congo ◽  
Barriers To Healthcare

scholarly journals Neonatal screening for sickle cell disease in Central Africa: a study of 1825 newborns with a new enzyme-linked immunosorbent assay test

2007 ◽  
Vol 14 (3) ◽  
pp. 113-116 ◽  
Author(s):  
Léon Mutesa ◽  
François Boemer ◽  
Louis Ngendahayo ◽  
Stephen Rulisa ◽  
Emmanuel K Rusingiza ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Immunosorbent Assay ◽  
Neonatal Screening ◽  
Democratic Republic Of Congo ◽  
Central Africa ◽  
High Specificity ◽  
Elisa Test ◽  
Enzyme Linked Immunosorbent Assay ◽  
Cell Disease

Objectives: To evaluate the feasibility of systematic neonatal screening for sickle cell disease in the region of Great Lakes in Central Africa using a new approach with limited costs. Methods: Between July 2004 and July 2006, 1825 newborn dried blood samples were collected onto filter papers in four maternity units from Burundi, Rwanda and the East of the Democratic Republic of Congo. We tested for the presence of haemoglobin C and S in the eluted blood by an enzyme-linked immunosorbent assay (ELISA) test using a monoclonal antibody. All ELISA-positive samples (multiple of the median (MoM)≥ 1.5) were confirmed by a simple molecular test. The statistica software version 7.1 was used to create graphics and to fix the MoM cut-off, and the χ2 of Pearson was used to compare the genotype incidences between countries. Results: Of the 1825 samples screened, 97 (5.32%) were positive. Of these, 60 (3.28%) samples were heterozygous for Hb S, and four (0.22%) for Hb C; two (0.11%) newborns were Hb SS homozygotes. Conclusions: The lower cost and the high specificity of ELISA test are appropriate for developing countries, and such systematic screening for sickle cell anaemia is therefore feasible.

scholarly journals Sickle Cell Disease in the Democratic Republic of Congo: Assessing Physicians’ Knowledge and Practices

2020 ◽  
Vol 5 (3) ◽  
pp. 127
Author(s):  
Benoît Mukinayi Mbiya ◽  
Ghislain Tumba Disashi ◽  
Béatrice Gulbis
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Democratic Republic ◽  
Democratic Republic Of Congo ◽  
Clinical Care ◽  
Diagnostic Tools ◽  
Public Health Issue ◽  
Cell Disease ◽  
Specific Training ◽  
Republic Of Congo

Background: Sickle cell disease is a major public health issue in the Democratic Republic of Congo (DRC), but it is still poorly understood by health professionals. The objective of this study was to assess the knowledge and practices of Congolese physicians treating sickle cell disease (SCD), in order to identify the areas for improvement in clinical care. Methods: This was a descriptive observational study conducted among Congolese physicians using a questionnaire. Participants were evaluated using a pre-established answer grid. Results: A total of 460 physicians participated, including 81 women (18%), with an average age of 35 years (range 25–60 years). Most physicians were general practitioners. Although self-assessment of their level of knowledge on SCD was estimated as average to good, less than half of the participants (n = 460; 46%) reported adequate management of vaso-occlusive crises, and only 1% of them had received specific training on SCD. Most physicians reported difficulties both in terms of diagnostic (65%) and management (79%) options of SCD patients. This study also showed that 85% of these physicians did not have access to the diagnostic tools for SCD. Conclusions: Insufficient knowledge on SCD and poor diagnostic and treatment options might contribute to increased morbidity and mortality of patients living in the DRC. Interventions aiming to improve physicians’ knowledge, patient follow-up, and treatment access are needed. Specific training alongside existing programs (HIV, malaria), early diagnosis of the disease, and the creation of patient advocacy groups should be implemented to improve SCD patient care.

Attention Deficits in Children with Sickle Cell Disease

2002 ◽  
Vol 95 (1) ◽  
pp. 57-67 ◽  
Author(s):  
Nina A. Nabors ◽  
Angela K. Freymuth
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Adaptive Functioning ◽  
Prior History ◽  
Cell Disease ◽  
Attentional Deficits ◽  
Wide Range Achievement Test ◽  
Wide Range ◽  
History Of ◽  
Timed Test

Previous research has suggested that children with sickle cell disease may exhibit cognitive deficits even in the absence of direct cerebrovascular involvement (stroke). This study was designed to assess specific attentional deficits in children with sickle cell disease. 12 children with sickle cell disease (Hb SS) with a prior history of stroke, 14 children with sickle cell disease (Hb SS) without evidence of stroke, and 13 similar aged siblings (Hb AA or Hb AS) were compared on measures of attention, intellectual functioning, achievement, and adaptive Functioning, Significant differences were found between children with sickle cell disease (with or without stroke) and healthy controls on a timed test of visual scanning, the Coding subtest of the Wechsler Intelligence Scale for Children—Revised, and subtests of Reading, Arithmetic, and Spelling from the Wide Range Achievement Test-Revised. The differences between children with sickle cell disease and their healthy siblings appear to be the result of strokes rather than sickle cell disease itself as children with sickle cell disease without strokes did not significantly differ from controls. Implications for the effects of sickle cell disease and stroke on academic performance are discussed.

scholarly journals Microfluidics in Sickle Cell Disease Research: State of the Art and a Perspective Beyond the Flow Problem

2021 ◽  
Vol 7 ◽  
Author(s):  
Anupam Aich ◽  
Yann Lamarre ◽  
Daniel Pereira Sacomani ◽  
Simone Kashima ◽  
Dimas Tadeu Covas ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Flow Problem ◽  
Red Cells ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Intercellular Interaction ◽  
Blood Disorder ◽  
Resource Setting ◽  
Wide Range

Sickle cell disease (SCD) is the monogenic hemoglobinopathy where mutated sickle hemoglobin molecules polymerize to form long fibers under deoxygenated state and deform red blood cells (RBCs) into predominantly sickle form. Sickled RBCs stick to the vascular bed and obstruct blood flow in extreme conditions, leading to acute painful vaso-occlusion crises (VOCs) – the leading cause of mortality in SCD. Being a blood disorder of deformed RBCs, SCD manifests a wide-range of organ-specific clinical complications of life (in addition to chronic pain) such as stroke, acute chest syndrome (ACS) and pulmonary hypertension in the lung, nephropathy, auto-splenectomy, and splenomegaly, hand-foot syndrome, leg ulcer, stress erythropoiesis, osteonecrosis and osteoporosis. The physiological inception for VOC was initially thought to be only a fluid flow problem in microvascular space originated from increased viscosity due to aggregates of sickled RBCs; however, over the last three decades, multiple molecular and cellular mechanisms have been identified that aid the VOC in vivo. Activation of adhesion molecules in vascular endothelium and on RBC membranes, activated neutrophils and platelets, increased viscosity of the blood, and fluid physics driving sickled and deformed RBCs to the vascular wall (known as margination of flow) – all of these come together to orchestrate VOC. Microfluidic technology in sickle research was primarily adopted to benefit from mimicking the microvascular network to observe RBC flow under low oxygen conditions as models of VOC. However, over the last decade, microfluidics has evolved as a valuable tool to extract biophysical characteristics of sickle red cells, measure deformability of sickle red cells under simulated oxygen gradient and shear, drug testing, in vitro models of intercellular interaction on endothelialized or adhesion molecule-functionalized channels to understand adhesion in sickle microenvironment, characterizing biomechanics and microrheology, biomarker identification, and last but not least, for developing point-of-care diagnostic technologies for low resource setting. Several of these platforms have already demonstrated true potential to be translated from bench to bedside. Emerging microfluidics-based technologies for studying heterotypic cell–cell interactions, organ-on-chip application and drug dosage screening can be employed to sickle research field due to their wide-ranging advantages.

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