Human T-cell Lymphotropic Virus types I and II (HTLV-I/II) in French Guiana: clinical and molecular epidemiology

We review here the epidemiological studies performed by our group on human retrovirus HTLV-I and HTLV-II infections and the associated diseases in French Guiana since 1984. French Guiana is an overseas French administrative district located between Brazil and Surinam. Its population is characterized by a large variety of ethnic groups, including several populations of African origin and various populations of Amerindian origin. Several epidemiological studies of large samples of pregnant women and in remote villages showed that HTLV-I is highly endemic in this area but is restricted to groups of African origin, especially the Noir-Marrons. In this endemic population, the results of segregation analysis in a genetic epidemiological study were consistent with the presence of a dominant major gene predisposing to HTLV-I infection, especially in children. In contrast, HTLV-II infection appears to be rare in French Guiana, having been found in only a few individuals of Brazilian origin. From a molecular point of view, the HTLV-I strains present in the Noir-Marrons, Creoles and Amerindians appear to originate from Africa, as they belong to the large cosmopolitan molecular subtype A.     

Pregnancy and sickle cell disease: a combination with potentially serious complications

Three women with sickle cell disease were hospitalised during pregnancy. The first patient, 26 years old, was admitted with a sickle cell crisis at a gestational age of 23 weeks; she subsequently developed preeclampsia with foetal growth retardation. A stillborn foetus was delivered at 24 weeks after termination of pregnancy. Histopathology of the placenta showed multiple infarctions possibly due to sickle cell disease. The second patient, 22 years old, was treated for sickle cell crisis and preterm labour. She delivered healthy twins at 29 weeks by Caesarean section. Postoperatively, an acute chest syndrome was treated with antibiotics and erythrocytapheresis. The third patient, 25 years old, was treated post partum for multiple venous thromboembolic events. Management of sickle cell-related complications is the same in pregnant and nonpregnant women. We recommend counselling women with sickle cell disease prior to conception and testing partners for haemoglobinopathy. The pregnant sickle cell patient needs specialised care from a multidisciplinary team including obstetricians, haematologists, neonatologists and anaesthesiologists.     

Knowledge and Acceptability of the HPV Vaccine Among Ethnically Diverse Black Women

The purpose of the study was to examine HPV vaccine knowledge and acceptability among ethnically diverse Black women. Forty-four women were interviewed in 6 focus groups (2 African American, 2 English-speaking Caribbean, 1 Haitian, and 1 African). Thematic content analysis was used to generate common concepts and themes and to compare findings across groups. There was varied but limited knowledge and confusion across ethnic groups about the HPV infection and vaccine. African and Haitian women had the least knowledge. Overall, women were generally receptive toward the HPV vaccine for girls but unclear about the need to vaccinate boys. Concerns about the HPV vaccine were mainly related to side effects/safety and vaccinating children at a young age. Healthcare provider’s recommendation of the vaccination was important for decision making. Educational interventions with Black women about HPV vaccination should recognize cultural beliefs that vary by ethnic group.     

Iron status of children with sickle cell disease

BACKGROUND: Dietary iron requirements are unclear in children with SS-type sickle cell disease. METHODS: Iron status was assessed in 104 nontransfused African American children (aged 0.5 to 17.6 years) with sickle cell disease who receive no iron supplement. Dietary iron intake was not measured at the time of this study. RESULTS: Serum ferritin was normal or high in all children. Other hematologic and biochemical indicators of iron deficiency were in the normal range in most children. CONCLUSIONS: Unlike previous studies, this sample of children and adolescents did not show signs of iron deficiency.     

University students in Nigeria: knowledge, attitude toward sickle cell disease, and genetic counseling before marriage

Sickle cell disease is an inherited debilitating and traumatic disease with severe morbidity and mortality among people of African descent. Children with sickle cell disease have difficulty in coping with school work due to their health conditions. In this cross-sectional survey of University of Ibadan Christian Pentecostal students, a majority of study respondents (63.6%) knew their AA genotype, had a high knowledge level of sickle cell disease, knew the benefits of genetic counseling, and had a positive attitude toward sickle cell disease and genetic counseling. More education about sickle cell disease and access to genetic counseling, especially for students in tertiary institutions in Nigeria and elsewhere in Africa, is recommended.     

Neonatal screening for hemoglobinopathies in Rio de Janeiro, Brazil]

OBJECTIVE: To describe the main results obtained in the first 15 months of neonatal screening for sickle cell disease in the state of Rio de Janeiro, Brazil, from August 2000 to November 2001. METHODS: Starting in August 2000, blood samples began to be collected for sickle cell disease screening from all newborns receiving care in primary health care clinics in the state of Rio de Janeiro. The samples were submitted to high-resolution liquid chromatography. If the resulting chromatogram was compatible with sickle cell disease, the child and the parents were referred for diagnostic confirmation and treatment. RESULTS: Between August 2000 and November 2001, 99 260 newborns were screened. There was one case of homozygous Hb C. On average, one of every 27 newborns who were screened presented sickle cell trait (Hb AS). Sickle cell disease was observed in 83 cases, or one new case in each 1 196 births. The 83 consisted of: 62 Hb S, 18 Hb SC, and 3 Hb SD. One child did not appear for diagnostic confirmation. The 82 children who were followed up by the program presented 15 intercurrent illnesses (upper respiratory infections, fever, splenic sequestration crises, hand-foot syndrome, and vascular occlusion), resulting in seven hospital admissions. Blood transfusions were necessary with 15 children, but none developed alloimmunization. All the other babies were doing well with the use of prophylactic penicillin. CONCLUSIONS: Our data show the importance of early diagnosis for sickle cell disease, so as to prevent the frequent infectious complications faced by these patients.     

Psychological Abuse of Children in Health Care (Book)

We compared the knowledge of sickle cell disease, child and parent coping strategies, and parent discipline methods/knowledge in three groups of children and their parents. The groups were composed of: (a) children with sickle cell anemia who experienced disease-related significant lifestyle disruption, (b) children with sickle cell anemia who experienced disease-related minimal lifestyle disruption, and (c) non-ill Black children. The groups were matched for age, sex, and ethnicity. No differences were found among the groups on measures of child coping. Children with sickle cell anemia, regardless of level of disruption, reported more knowledge about sickle cell disease than did healthy peers. Parents of children in the significant lifestyle disruption groups scored lower on the Engagement, Emotional Engagement, and Problem-Focused Disengagement, and higher on the Medical Coping scales of the Coping Strategies Inventory than parents of healthy children and children in the minimal lifestyle disruption group. Surprisingly, the groups did not differ in discipline knowledge; however, parents of children in the minimal lifestyle disruption group reported significantly greater use of effective discipline methods than parents of healthy children. Further research on the relations among coping, discipline, and lifestyle disruption due to SCA in children is suggested.     

Neonatal screening and clinical care programmes for sickle cell disorders in sub-Saharan Africa: lessons from pilot studies

OBJECTIVES: Despite the widespread use of neonatal screening programmes for sickle cell disease in Western regions, few studies have focused on the special healthcare needs in sub-Saharan African countries. The purpose of this review is to evaluate the need for a neonatal screening programme for sickle cell disease, and if justified, to propose a realistic healthcare programme for sickle cell newborns in those countries based on personal experiences in Kinshasa (Democratic Republic of the Congo) and Ouagadougou (Burkina Faso) as well as from a review of the literature. REVIEW: There are well-established criteria for the development of neonatal screening programmes for sickle cell disease in sub-Saharan African countries. In particular, in regions where incidence of the disease is 0.5 per 1000 or higher, a sickle cell screening programme can be proposed that includes the systematic screening of all newborns, or the targeted screening of those newborns who have a mother with a sickle cell or haemoglobin C trait. Screening should be preferentially organized using cord blood, with a simple, effective and affordable screening method such as isoelectric focusing. If necessary, confirmation of results should be performed using another cost-effective technique such as citrate agar electrophoresis at an acidic pH. There is also a need for a sickle cell disease clinical care programme which should include: infection prophylaxis with penicillin and malarial prophylaxis; family training to identify early severe or persistent symptoms and the gravity of malarial crises; the evaluation of nutritional status and adequate fluid intake; and the importance of regular medical visits. Improved knowledge of the diagnosis was found to reduce the need for unnecessary and unsafe blood transfusions. CONCLUSIONS: This paper provides an overview of practices employed in neonatal screening and clinical care programmes for sickle cell disease in sub-Saharan African countries. The development of these programmes is pivotal to improving the health care of those affected by haemoglobin disorders. However, such programmes require major economic and organizational resources, which must taken into account and balanced against other local health priorities.     

Heterogeneity in the prevalence of methylenetetrahydrofolate reductase gene polymorphisms in women of different ethnic groups

OBJECTIVE: To determine the prevalence of methylenetetrahydrofolate reductase (MTHFR) gene polymorphisms in women of different ethnic groups and to relate these common mutations to plasma homocysteine, red cell folate, and serum folate. DESIGN: A one-time fasting blood sample was obtained for MTHFR genotype (C677T and A1298C) determinations (n=433). Serum folate, red cell folate, and homocysteine analyses were performed in nonfolic acid supplement users (n=215). SUBJECTS/SETTING: This study involved 433 women from four ethnic groups, including 193 Hispanic women of Mexican descent, 139 white women, 53 Asian women of mixed descent, and 48 African American women. STATISTICAL ANALYSIS PREFORMED: Chi;(2), t Test, and analysis of variance were used. RESULTS: Mexican women (18.1%) had a higher frequency of the 677 TT genotype compared with white (7.2%), Asian (3.8%), and African American (0%) women. White women (7.9%) had a higher frequency of the 1298 CC genotype than the other ethnic groups (range=1.9% to 2.6%). The frequency of compound heterozygosity (677 CT + 1298 AC) was higher in Mexican (17.6%) and white (15.1%) women than Asian and African American ( approximately 4% to 6%) women. In the era of folic acid fortification, neither genotype, independently or together, was associated with homocysteine or blood folate concentrations when ethnic groups were combined. In Mexican women, however, a linear trend (P相似文献   

Geographic differences in mortality of young children with sickle cell disease in the United States.

OBJECTIVES: Because geographic differences in health care have been found for many diseases, including those affecting children, there are probably geographic differences in the health care of young children with sickle cell disease. Consequently, survival of young children with sickle cell disease might differ among geographic areas. This study''s objective was to identify areas in the United States where young children with sickle cell disease are at especially high and low risk of dying. METHODS: Using U.S. death certificate data from 1968 through 1992, the authors calculated the mortality rates of 1- through 4-year-old black children with sickle cell disease for states, counties, and cities. Deaths from trauma, congenital anomalies, and perinatal conditions were excluded. RESULTS: From 1968 through 1980 and from 1981 through 1992, 1- through 4-year-old black children with sickle cell disease in Florida had a markedly higher risk of dying, and those in Pennsylvania had a markedly lower risk of dying, than the average 1- through 4-year-old black child with the disease in the United States. From 1981 through 1992, 1- through 4-year-old black children with sickle cell disease in Maryland had the lowest mortality rate in the nation. During the same time period, 1- through 4-year-old black children with sickle cell disease in five counties in Florida were at especially high risk, while in Baltimore no young black children with the disease died. These geographic differences in mortality of black children with sickle cell disease greatly exceeded geographic differences in mortality of black children without the disease. CONCLUSIONS: Marked differences exist across the United States in mortality of young black children with sickle cell disease. To improve survival for children with the disease in high mortality areas, evaluations should be made of the accessibility and quality of medical care, and of parents'' health care seeking behavior and compliance with antibiotic prophylaxis. In addition, efforts should be made to understand and duplicate the success of treatment programs in low mortality areas.     

Impact of Lifestyle Disruption on Parent and Child Coping, Knowledge, and Parental Discipline in Children With Sickle Cell Anemia

We compared the knowledge of sickle cell disease, child and parent coping strategies, and parent discipline methods/knowledge in three groups of children and their parents. The groups were composed of: (a) children with sickle cell anemia who experienced disease-related significant lifestyle disruption, (b) children with sickle cell anemia who experienced disease-related minimal lifestyle disruption, and (c) non-ill Black children. The groups were matched for age, sex, and ethnicity. No differences were found among the groups on measures of child coping. Children with sickle cell anemia, regardless of level of disruption, reported more knowledge about sickle cell disease than did healthy peers. Parents of children in the significant lifestyle disruption groups scored lower on the Engagement, Emotional Engagement, and Problem-Focused Disengagement, and higher on the Medical Coping scales of the Coping Strategies Inventory than parents of healthy children and children in the minimal lifestyle disruption group. Surprisingly, the groups did not differ in discipline knowledge; however, parents of children in the minimal lifestyle disruption group reported significantly greater use of effective discipline methods than parents of healthy children. Further research on the relations among coping, discipline, and lifestyle disruption due to SCA in children is suggested.     

Dépistage néonatal ciblé de la drépanocytose : limites du test de falciformation (test d’Emmel) dans le bilan prénatal en zone ouest africaine

Background

Newborn screening for sickle cell anemia is necessary in Africa where the disease is more frequent. Hemoglobin electrophoresis is used for screening, but is limited by a high cost and difficult access. Sickling test (Emmel test), which is more affordable and technically more accessible, is often requested for prenatal assessment of pregnant women in West African areas to reserve screening for newborns from mothers in whom the positive sickling test attests the presence of hemoglobin S. This study aims to evaluate the number of undetected sickle cell anemia newborns by a screening policy targeting only newborns from mothers in whom a sickling test would have been positive.

Awareness of Sickle Cell among People of Reproductive Age: Dominicans and African Americans in Northern Manhattan

Sickle cell disease is a chronic condition that is characterized by severe anemia, painful crises, and organ dysfunction. In the USA, sickle cell is a health burden typically associated with African Americans. Dominicans constitute the largest Latino group in New York City (NYC) and have the second overall highest prevalence of sickle trait—one in 20 births, compared to one in 12 African American births. We aimed to document the prevalence of sickle within the largely Dominican and African American community of Northern Manhattan (Washington Heights, Inwood, Harlem), assess and compare knowledge about sickle disease and carrier status in young adults of reproductive age between African Americans and Dominicans, and elicit preferred sources of health information. NY State Newborn Screening data in Northern Manhattan were analyzed by zip code. A brief oral survey was administered to 208 parents of young children—150 Dominicans and 58 African Americans. Significant differences were seen in knowledge about sickle—27% of Dominican parents surveyed correctly defined sickle cell disease as an inherited blood disorder, compared to 76% of African Americans (p < 0.001). Only 7% of African Americans did not know their own trait status, compared to 43% of Dominicans (p < 0.001). Parents were better informed if they or family members were affected by sickle conditions. Participants from both groups prefer receiving information from doctors and online. A separate group of 168 predominantly Dominican youth, ages 14–24, demonstrated knowledge levels similar to that of Dominican parents. These results suggest that many of reproductive age in a NYC community affected by sickle conditions frequently lack basic relevant information, with larger information gaps among Dominicans. Expanded efforts are warranted to inform young adults of diverse affected communities.     

Physician knowledge and adherence to prescribing antibiotic prophylaxis for sickle cell disease.

OBJECTIVE: The purpose of this research was to examine how physician characteristics were associated with: (i). physician knowledge of and adherence to sickle cell guidelines; and (ii). the types of educational programs about sickle cell disease desired by physicians. METHODS: A survey was developed to assess the research objective. After the survey was pre-tested and an institutional review board exemption was obtained, it was sent to a systematic random sample of 375 pediatricians and all 125 practicing hematologists in North Carolina. They were asked to answer a six-item knowledge test relating to the antibiotic prophylaxis guidelines. RESULTS: The response rate was 57%, of which 61% were pediatricians. Over half (56%) were in a practice with at least one pediatric sickle cell patient. Fifty-nine percent of physicians answered five or more questions correctly on the knowledge test. The question most physicians answered correctly (97%) pertained to the necessity of antibiotics for children with sickle cell disease. The question most frequently answered incorrectly (62%) pertained to prescribing antibiotics to a child with unconfirmed sickle cell disease. Logistic regression results indicated that the number of sickle cell patients seen in practice influenced the number of questions answered correctly. Sixty-six percent of physicians prescribed prophylactic antibiotics for 100% of their patients with sickle cell disease and therefore were 100% adherent. Eighty-one percent of pediatricians compared with 12% of hematologists were 100% adherent in prescribing antibiotics. Hematologists and those practicing at a medical school or university were less likely to be 100% adherent in prescribing antibiotic prophylaxis. CONCLUSION: The majority of physicians surveyed were relatively knowledgeable about sickle cell guidelines, however there may be a need for continuing education programs that focus on the issues of prescribing antibiotics to a child with unconfirmed sickle cell disease and penicillin dosage.     

Pregnancy in the Sickle Cell Disease and Fetomaternal Outcomes in Different Sickle cell Genotypes: A Systematic Review and Meta-Analysis

BackgroundPregnancy is a major concern among women with the sickle cell disease (SCD), and it is associated with increased adverse outcomes. The aim of the present meta-analysis is to report the fetomaternal outcomes in different sickle cell genotypes.MethodsIn this systematic review and meta-analysis, a comprehensive search of databases and search engines such as PubMed, Scopus, Web of Science, ProQuest, Cochrane Library, Science Direct and Google Scholar were performed. Any observational studies that had compared at least one outcome such as maternal outcomes, fetal outcomes, and morbidity between two groups of pregnant women with different types of sickle cell genotypes and pregnant women without SCD were evaluated.ResultsA total number of 9,827 pregnant women with SCD were examined. The results showed that pregnancy in SCD increased the risk of adverse outcomes for the mothers (including postpartum hemorrhage, prematurity, pregnancy-induced hypertension, pre-eclampsia, eclampsia, cesarean section, lower segment cesareansection, maternal death), fetus (including live births, low birth weight, intrauterine growth restriction, APGAR score at 5 min <7, stillbirth, neonatal death, perinatal mortality, acute fetal distress, intrauterine fetal death) and morbidity among the SCD(severe anemia, urinary tract infection, blood transfusion, painful crisis, acute chest syndrome, vaso-occlusive crises).ConclusionAccording to the results of this meta-analysis, pregnancy in the SCD is associated with an increased risk of maternal outcomes, fetal outcomes, and morbidity among SCD patients with different genotypes. Pregnancy in sickle cell hemoglobinopathies needs careful multidisciplinary management and cautious caring so as to decrease maternal and fetal morbidity and mortality.     

An analysis of the results of a program for the prevention of sickle-cell anemia in Camagüey Province, 1986

This paper deals with results of the programme for the prevention of anemia due to sickle cells carried out in Camagüey Province, during 1986. The programme is based on the statistical study of all pregnant women searched during 1986 (12,154), to verify those carriers (heterozygous) of hemoglobin AS and AC, as well as risk couples and to be able to analyze incidence by health area during the period comprised from March 1st. to June 30, 1986, at the Maternal Hospital and "Manuel Ascunce Domenech" Provincial Hospital, Camagüey. A data collecting model looking upon particular and familial aspects of the pregnant woman, such as origin, race, number of clinical history and gestational age, within others, was filled by them. There is 2.13% (259) pregnant women carriers of this pathology in the province. Ten risk couples were detected, but without presenting consanguinity. The concept that this disease prevails in black people is sustained. It is considered that there is need of a publishing work on this theme among patients and of preparing scientifically the nursing personnel in charge of pregnant women.     

Role of dietary factors in ethnic differences in early risk of cardiovascular disease and type 2 diabetes

BACKGROUND: The disparity in the prevalence of cardiovascular disease and type 2 diabetes between African Americans and whites has been well established, and ethnic differences in several risk factors for these diseases are evident in childhood. OBJECTIVE: The current study explored whether dietary factors explain ethnic differences in serum lipids and insulin profiles in children, independent of body composition and social class background. DESIGN: The sample included 95 African American and white children (mean age: 10.0 y). Macronutrient and food group intakes were derived from three 24-h recalls. Cardiovascular disease and type 2 diabetes risk were determined on the basis of total cholesterol, triacylglycerol, insulin sensitivity (S(i)), and acute insulin response (AIR). Data were analyzed by using t tests, analysis of covariance, and multiple regression. RESULTS: African American children had lower triacylglycerol (P < 0.01), lower S(i) (P < 0.001), and higher AIR (P < 0.001) than whites. Intake of fruit and vegetables was significantly higher, and dairy intake lower, in African American than in white children after adjustment for social class and total energy intake. Several direct relations were observed between diet and insulin action: carbohydrate and fruit intakes were positively associated with S(i) (P = 0.02), and vegetable intake was negatively associated with AIR (P = 0.01). However, neither macronutrient nor food group intake accounted for the ethnic differences in triacylglycerol and AIR. CONCLUSIONS: The African American children in our sample showed a greater disease risk than did the white children, even after body composition, social class background, and dietary patterns were adjusted for.