RESUMO
Japanese encephalitis (JE) disease among children continues in central India despite vaccination implemented in the routine immunization program. Therefore, we planned to estimate the JE vaccination effectiveness among children by undertaking a 1:2 individually-matched population-based case-control study from August 2018 to October 2020. The laboratory-confirmed JE cases aged 1-15 years were enrolled along with neighborhood controls without fever and encephalitis matched on the residence area, age and sex. The JE vaccination history was enquired from parents and verified independently from the vaccination cards available at home and records at health facilities. We enrolled 35 JE cases and 70 matched controls. The vaccination effectiveness of 86.7% (95% confidence interval [CI]: 30.8-94.7) was estimated on the per-protocol analysis of 31 case-control sets. The screening method provided an effectiveness of 89.5% (CI: 78.9-94.7) on using the population vaccination coverage of 90% reported earlier in the same area. In conclusion, JE vaccination offered a moderate level of protection among children in JE medium-endemic central India, similar to reports from high-endemic areas in India. The operational aspects of vaccination program implementation need to be evaluated to assess the impact of vaccination on the disease burden of JE in medium-endemic regions of India.
Assuntos
Encefalite Japonesa , Criança , Humanos , Encefalite Japonesa/epidemiologia , Encefalite Japonesa/prevenção & controle , Estudos de Casos e Controles , Vacinação , Programas de Imunização , Índia/epidemiologiaRESUMO
COVID-19 pandemic has affected all age groups globally including pregnant women and their neonates. The aim of the study was to understand outcomes in neonates of mothers with COVID-19 during the first and second waves of COVID-19 pandemic. A retrospective analysis of 2524 neonates born to SARS-CoV-2-infected mothers was conducted during the first wave (n = 1782) and second wave (n = 742) of the COVID-19 pandemic at five study sites of the PregCovid registry in Maharashtra, India. A significant difference was noted in preterm birth, which was higher in the second wave (15.0%, 111/742) compared to the first wave (7.8%, 139/1782) (P < 0.001). The proportion of neonates requiring NICU admission was significantly higher in the second wave (19.0%, 141/742) as compared to that in the first wave (14.8%, 264/1782) (P < 0.05). On comparing regional differences, significantly higher neonatal complications were reported from Mumbai metropolitan region (P < 0.05). During the second wave of COVID-19, birth asphyxia and prematurity were 3.8- and 2.1-fold higher respectively (P < 0.001). Neonatal resuscitation at birth was significantly higher in second wave (3.4%, 25/742 vs 1.8%, 32/1782) (P < 0.05). The prevalence of SARS-CoV-2 infection in neonates was comparable (4.2% vs 4.6%) with no significant difference between the two waves. CONCLUSION: Higher incidence of adverse outcomes in neonates born to SARS-CoV-2-infected mothers in the second wave of COVID-19 as compared to the first wave. TRIAL REGISTRATION: PregCovid study is registered with the Clinical Trial Registry of India (CTRI/2020/05/025423, Registered on 28/05/2020). WHAT IS KNOWN: ⢠The second wave of COVID-19 was more lethal to pregnant women than the first wave. Newborns are at risk of developing complications. WHAT IS NEW: ⢠Birth asphyxia, prematurity, and neonatal resuscitation at birth were significantly higher in the second wave as compared to those in the first wave of the COVID-19 pandemic in India.
Assuntos
COVID-19 , Doenças do Recém-Nascido , Complicações Infecciosas na Gravidez , Nascimento Prematuro , Asfixia/epidemiologia , COVID-19/epidemiologia , Feminino , Humanos , Índia/epidemiologia , Recém-Nascido de Baixo Peso , Recém-Nascido , Doenças do Recém-Nascido/epidemiologia , Transmissão Vertical de Doenças Infecciosas , Mães , Pandemias , Gravidez , Complicações Infecciosas na Gravidez/epidemiologia , Nascimento Prematuro/epidemiologia , Ressuscitação , Estudos Retrospectivos , SARS-CoV-2RESUMO
Background & objectives: Sickle cell disease (SCD) constitutes frequently inherited haemoglobin disorders and poses a significant health burden in India. Hydroxyurea (HU), the most commonly used drug, has shown promising results in the clinical management of SCD. The present systematic review was undertaken to assess the efficacy and toxicity of HU in Indian sickle cell patients. Methods: A systematic review of studies on HU therapy was conducted to identify the application of HU and its outcome(s) across India. PubMed, Scopus and Cochrane Library was used as data sources for various studies on the efficacy and toxicity of HU therapy for treatment for SCD in India published between January 2001 and October 2021. Two authors independently extracted the data on study design, patient characteristics and therapeutic outcomes of HU in order to determine the study quality of the present review. Results: Overall, 14 studies were included for a systematic analysis. Of these 11 were prospective, two cross-sectional and one double-blind randomized controlled trial. Low-dose HU (10 mg/kg/day) was found to reduce the rates of vaso-occlusive crisis and hospitalization as well as decreased the requirement of blood transfusion in SCD patients. The foetal haemoglobin (HbF) level was recorded in 13 (80%) studies all of whom reported an elevation in the HbF levels, with a mean increase in per cent HbF from 15.8 to 21.4 per cent across studies. The common adverse events were reversible, mild-to-moderate cytopenia and anaemia. Interpretation & conclusions: The findings of the present review suggest that there is still insufficient information presently to determine the long-term or major adverse effects on organ damage, fertility as well as pregnancy on the use of HU therapy for SCD. Long-term multi-centric studies are thus required to address these problems.
Assuntos
Anemia Falciforme , Hidroxiureia , Humanos , Hidroxiureia/efeitos adversos , Antidrepanocíticos/efeitos adversos , Estudos Transversais , Estudos Prospectivos , Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/epidemiologia , Ensaios Clínicos Controlados Aleatórios como AssuntoRESUMO
Background & objectives: Homozygous sickle cell (SS) disease in Central India runs a more severe clinical course than reports from other areas of India. The current study was undertaken to compare the disease in Central India (Nagpur) with that in Jamaica, both populations defined by newborn screening. Methods: The Nagpur cohort included infants born to sickling-positive mothers from May 2008 to 2012, examined by high-pressure liquid chromatography and DNA analysis. The Jamaican cohort screened 100,000 consecutive non-operative deliveries between June 1973 and December 1981, analyzed by haemoglobin (Hb) electrophoresis and confirmed by family studies and compatible HbA2levels. Results: In Nagpur, 103 SS patients were detected, but only 78 (76%) were followed up. In Jamaica, 311 cases were followed from birth and compliance with follow up remained 100 per cent up to 45 years. In the Nagpur cohort all had the Asian haplotype, and 82 per cent of Jamaicans had at least one Benin chromosome; none had the Asian haplotype. Compared to Jamaica, Nagpur patients had higher foetal Hb, less alpha-thalassaemia, later development of splenomegaly and less dactylitis. There were also high admission rates for febrile illness and marked anaemia. Invasive pneumococcal disease occurred in 10 per cent of Jamaicans but was not seen in Nagpur. Interpretation & conclusions: There were many differences between the disease in Nagpur, Central India and the African form observed in Jamaica. The causes of severe anaemia in Nagpur require further study, and reticulocyte counts may be recommended as a routine parameter in the management of SS disease. The role of pneumococcal prophylaxis needs to be determined in Nagpur patients. Future studies in India must avoid high default rates.
Assuntos
Anemia Falciforme , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Anemia Falciforme/genética , Hemoglobina Fetal , Homozigoto , Humanos , Índia/epidemiologia , Lactente , Recém-Nascido , Jamaica/epidemiologiaRESUMO
PURPOSE OF REVIEW: In the past, milder clinical manifestations of sickle cell disease (SCD) have been described from India. However, recent data from some parts of India suggest that the severity of the disease can be compared to that of African phenotypes. This review therefore describes the varied clinical manifestation of SCD, the success of newborn screening programme, prenatal diagnosis and low dose hydroxyurea therapy in India. RECENT FINDINGS: The varied clinical manifestations such as anemia, vaso-occlusive crisis, acute chest syndrome, renal involvement, stroke and so on vary from one part of the country to the other and also among different communities of India. Strategies for improving quality of life and controlling of SCD have been suggested. Certain factors other than genetics also play an important role in clinical manifestation of the disorder. SUMMARY: The clinical diversity of SCD is described. The natural history of SCD in India is unfolding from newborn screening programme. The use of low-dose hydroxy urea therapy both in adults and children has brought down the incidences of crisis and provides great relief to the patients. The tailor-made programme for India as regards the control and management has been discussed.
Assuntos
Anemia Falciforme , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Anemia Falciforme/fisiopatologia , Anemia Falciforme/terapia , Feminino , Humanos , Índia/epidemiologia , Recém-Nascido , Masculino , Programas de RastreamentoRESUMO
BACKGROUND: The clinical phenotype of sickle cell disease (SCD) has been reported to be milder in India than in the United States. The objective of this large single-center study was to examine the rate of complications to define the phenotype of SCD in India. METHODS: The rate of complications per 100 person-years in 833 pediatric SCD patients for 1954 person-years in Nagpur, India including those diagnosed on newborn screen (NBS) and those presenting later in childhood (non-NBS) was compared to those reported in the cooperative study of sickle cell disease (CSSCD). Event rates were also compared between patients belonging to scheduled castes (SCs), scheduled tribes (STs), and other backward classes (OBC). RESULTS: Comparison of CSSCD versus Nagpur NBS versus Nagpur non-NBS for rates of pain (32.4 vs. 85.2 vs. 62.4), severe anemia (7.1 vs. 27 vs. 6.6), stroke (0.7 vs. 0.8 vs. 1.4), splenic sequestration (3.4 vs. 6.7 vs. 1.6), acute chest syndrome (24.5 vs. 23.6 vs. 1.0), and meningitis (0.8 vs. 0 vs. 0.1) revealed more frequent complications in Nagpur compared to CSSCD. Comparison of ST, SC, and OBC for rates of pain (84.6 vs. 71.9 vs. 63.5), acute chest syndrome (3.6 vs. 2.8 vs. 2.2), severe anemia (5.4 vs. 9.5 vs. 11.4), stroke (1.2 vs. 0.4 vs. 0.3), splenic sequestration (0.6 vs. 2.4 vs. 1.9), and meningitis (0.8 vs. 0 vs. 0.1) revealed significantly more frequent complications among ST. CONCLUSIONS: SCD-related complications are more frequent in Indian children than that observed in CSSCD. Further study is indicated to define SCD phenotype in India.
Assuntos
Anemia Falciforme/complicações , Adolescente , Anemia Falciforme/mortalidade , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Fenótipo , Estudos ProspectivosRESUMO
BACKGROUND: Sickle cell disease is a major health burden in India. The aim of the study was to compare the diagnostic utility of two different approaches on automated high performance liquid chromatography (HPLC) for newborn screening for sickle cell disorders and other haemoglobinopathies in India. METHODS: Newborn babies of sickle heterozygous mothers were tested by HPLC using two different kits, the ß-thal short kit, which is routinely used for screening for haemoglobinopathies in most laboratories, and the sickle cell short kit which is specific only for neonatal samples. Confirmation of the sickle and α genotypes was done by molecular analysis. RESULTS: Of the 601 babies tested, 276 were normal, 284 were sickle heterozygous and 41 were sickle homozygous using the ß-thal short kit. Using the sickle cell short kit, a discrepancy was seen in one newborn sample where a normal baby was identified as a sickle heterozygous baby. α-Genotyping was done in 42 babies and 16 of them had α gene deletions. The presence of α thalassaemia could be suspected in 15 of these 16 babies based on a spike at the start of the chromatogram using the ß-thal short kit. In comparison, using the sickle cell short kit the diagnosis of α thalassaemia was difficult based on the percentage of the FAST peak. Further, other rare α chain Hb variants were also missed. CONCLUSIONS: The ß-thal short kit was more versatile than the sickle cell short kit for screening for haemoglobinopathies in newborns in our population.
Assuntos
Cromatografia Líquida de Alta Pressão , Hemoglobina Falciforme/análise , Hemoglobinopatias/diagnóstico , Genótipo , Hemoglobina Falciforme/genética , Heterozigoto , Homozigoto , Humanos , Recém-Nascido , Triagem NeonatalRESUMO
The ß-thalassemias and sickle cell disorders are a major health burden in India. Diagnosis and management of these disorders both in adults and in newborns using appropriate approaches and uniform technology are important in different regions of a vast and diverse country as India. In view of a National Thalassemia Control Program to be launched soon, a need was felt for guidelines on whom to screen, cost-effective technologies that are to be used as well as for establishing prenatal diagnosis programs in regional centers. Newborn screening for sickle cell disorders is in its infancy in India and uniform approaches need to be followed. Also, included are guidelines for monitoring and managing patients who are now growing older and need comprehensive care as well as management of complications of the disease.
RESUMO
BACKGROUND & OBJECTIVES: Children with sickle cell disease require more frequent hospital care and younger children (<5 yr of age) are more vulnerable to mortality. There are limited data on the events leading to hospitalizations and death in younger children with sickle cell disease from India. This study was, therefore, undertaken to evaluate the morbidity pattern in hospitalized under five children with sickle cell disease in a tertiary care hospital in Maharashtra, India. METHODS: This was a prospective observational study carried out from July 2007 to June 2009. Hospitalized children below five years of age with sickle cell disease were enrolled for the study and evaluated for morbid event/s leading to hospitalization. Haematological indices were noted at baseline (most recent past when patient was not acutely sick) and at the time of hospitalization. RESULTS: Eighty five children with sickle cell disease were hospitalized during the study period. Hospitalization with acute febrile illness (31%) was the most common morbid event followed by severe anaemia (30%) and acute painful events (20%). Majority (62%) of the events occurred between August and October. Forty five patients had foetal haemoglobin (HbF) more than 20 per cent (26.80 ± 4.81%) and morbidity was significantly less in these patients. INTERPRETATION & CONCLUSION: Acute febrile illness was the most common morbid event followed by severe anaemia and acute painful event hospitalized children with sickle cell disease. There was significant seasonal variation with maximum events occurring in the monsoon season.
Assuntos
Anemia Falciforme/fisiopatologia , Hospitalização , Anemia Falciforme/epidemiologia , Anemia Falciforme/terapia , Pré-Escolar , Feminino , Humanos , Índia/epidemiologia , Lactente , Masculino , Estudos ProspectivosRESUMO
India accounts for 14.5 percent of the global SCD newborns, roughly over 42,000 a year, second to sub-Saharan Africa. Despite the availability of cheap diagnostic and treatment options, SCD remains a largely neglected disease within healthcare policy and practice. Epidemiological modeling based on small, often dated, regional studies (largely from sub-Saharan Africa) estimate that between 50 and 90 percent of affected children will/die before the age of 5 years. This premise, coupled with targets of reducing under 5 mortality (SDG 4), privileges public health interventions for screening and prevention of new births, undermining investments in long-term health and social care. This paper presents a retrospective, descriptive analysis of the socio-demographic profile of 447 patients diagnosed with sickle cell or sickle-beta thalassemia, who died following admission at a tertiary care entre in India. We used anonymized hospital records of 3,778 sickle cell patients, admitted in pediatric and adult/medical wards between January 2016 and February 2021. A majority of hospital deaths occurred in the second and third decades of life, following a hospital admission for a week. The overall mortality during 2016-2019 was 14% with little gender difference over time. Contrary to our expectations, the number of hospital deaths did not increase during the first year of the COVID-19 pandemic, between 2020 and 2021. The conclusion highlights the importance of longitudinal, socio-demo-graphic data on deaths as providing important insights for identifying ethical policy interventions focused on improving SCD outcomes over time, reducing inequities in access to care, and preventing what might be considered "excess" deaths.
Assuntos
Anemia Falciforme , Pandemias , Adulto , Criança , Humanos , Recém-Nascido , Pré-Escolar , Estudos Retrospectivos , Anemia Falciforme/epidemiologia , Anemia Falciforme/diagnóstico , Política de Saúde , Causas de MorteRESUMO
BACKGROUND: We estimated the incidence of Japanese encephalitis (JE) and acute encephalitis syndrome (AES) following routine immunization with the live-attenuated SA 14-14-2 JE vaccine. METHODS: We implemented enhanced surveillance of AES and JE hospitalizations in endemic districts in Maharashtra and Telangana States during 2015-2016 and 2018-2020. We estimated incidence and compared differences in the incidence of JE and AES between two states, and vaccinated and unvaccinated districts during two study periods. We also considered secondary data from public health services to understand long-term trends from 2007 to 2020. RESULTS: The annual AES incidence rate of 2.25 cases per 100,000 children in Maharashtra during 2018-2020 was significantly lower than 3.36 cases per 100,000 children during 2015-2016. The six JE-vaccinated districts in Maharashtra had significantly lower incidence rates during 2018-2020 (2.03, 95% CI 1.73-2.37) than in 2015-16 (3.26, 2.86-3.70). In addition, the incidence of both JE and AES in two unvaccinated districts was higher than in the vaccinated districts in Maharashtra. Telangana had a lower incidence of both JE and AES than Maharashtra. The AES incidence rate of 0.95 (0.77-1.17) during 2018-2020 in Telangana was significantly lower than 1.67 (1.41-1.97) during 2015-2016. CONCLUSIONS: The annual incidence rate of Japanese encephalitis was < 1 case per 100,000 children. It indicated accelerated control of Japanese encephalitis after routine immunization. However, the annual incidence of acute encephalitis syndrome was still > 1 case per 100,000 children. It highlights the need for improving surveillance and evaluating the impacts of vaccination.
Assuntos
Encefalopatia Aguda Febril , Encefalite Japonesa , Criança , Humanos , Encefalite Japonesa/epidemiologia , Encefalite Japonesa/prevenção & controle , Incidência , Encefalopatia Aguda Febril/epidemiologia , Índia/epidemiologia , HospitalizaçãoRESUMO
There is limited data on the efficacy of hydroxyurea (HU) in Indian sickle cell anemia patients who have severe manifestations despite high fetal hemoglobin (Hb F). Sixty sickle cell anemia children (5-18 years) with more than three episodes of vasoocclusive crises or blood transfusions per year were randomized to receive HU (n = 30) or placebo (n = 30) therapy. Fixed dose (10 mg/kg/day) of HU was administered for 18 months and the patients were followed-up monthly with clinical assessment and laboratory monitoring. In the HU group, hemoglobin (Hb) and Hb F levels increased significantly along with a significant decrease in the number of painful crises, blood transfusion requirements and hospitalizations compared to the placebo group. No major adverse events were observed in this study. In conclusion, low-fixed dose HU therapy was effective for the treatment of Indian sickle cell anemia children. However, there is a need for long-term studies to evaluate the efficacy and toxicity in a larger number of Indian sickle cell anemia patients.
Assuntos
Anemia Falciforme/tratamento farmacológico , Antidrepanocíticos/uso terapêutico , Hidroxiureia/uso terapêutico , Adolescente , Anemia Falciforme/sangue , Antidrepanocíticos/efeitos adversos , Transfusão de Sangue/estatística & dados numéricos , Criança , Feminino , Hemoglobina Fetal/metabolismo , Seguimentos , Hospitalização/estatística & dados numéricos , Humanos , Hidroxiureia/efeitos adversos , Masculino , Dor/prevenção & controle , Resultado do TratamentoRESUMO
There is limited data on the incidence of sickle cell anemia in Central India; we therefore conducted a study to estimate the incidence of this disease in Central India. Mothers who delivered a live baby at the Government Medical College, Nagpur, India were screened for the presence of the sickle cell hemoglobin {Hb S: [ß6 (A3) GluâVal, GAG>GTG]} using the solubility test within 48 hours of delivery. Infants of mothers who showed the presence of Hb S then underwent Hb analysis by high performance liquid chromatography (HPLC). A total of 8243 mothers was screened, 1178 of whom were positive. One thousand, one hundred and sixty-two infants of mothers with a positive solubility test underwent Hb analysis by HPLC; 530 infants were normal, while 536 were heterozygous for Hb S (sickle cell trait), 88 babies were homozygous for Hb S (sickle cell anemia), while another eight babies had other Hb abnormalities. The incidence of sickle cell anemia was highest in the Scheduled caste group (1:50). We concluded that the incidence of sickle cell anemia is high in central India.
Assuntos
Anemia Falciforme/genética , Testes Genéticos/métodos , Hemoglobina Falciforme/genética , Triagem Neonatal/métodos , Anemia Falciforme/diagnóstico , Anemia Falciforme/epidemiologia , Cromatografia Líquida de Alta Pressão , Genótipo , Hemoglobina Falciforme/análise , Humanos , Incidência , Índia/epidemiologia , Recém-NascidoRESUMO
BACKGROUND: We enhanced surveillance of hospitalizations of all ages for acute encephalitis syndrome (AES) along with infectious aetiologies, including the Japanese encephalitis virus (JEV). METHODS: From October 2018 to September 2020, we screened neurological patients for AES in all age groups in Maharashtra and Telangana States. AES cases were enrolled at study hospitals along with other referrals and sampled with cerebrospinal fluid, acute and convalescent sera. We tested specimens for non-viral aetiologies viz. leptospirosis, typhoid, scrub typhus, malaria and acute bacterial meningitis, along with viruses - JEV, Dengue virus (DENV), Chikungunya virus (CHIKV), Chandipura virus (CHPV) and Herpes simplex virus (HSV). RESULTS: Among 4977 neurological hospitalizations at three study site hospitals over two years period, 857 (17.2%) were AES. However, only 287 (33.5%) AES cases were eligible. Among 278 (96.9%) enrolled AES cases, infectious aetiologies were identified in 115 (41.4%) cases, including non-viral in 17 (6.1%) cases - leptospirosis (8), scrub-typhus (3) and typhoid (6); and viral in 98 (35.3%) cases - JEV (58, 20.9%), HSV (22, 7.9%), DENV (15, 5.4%) and CHPV (3, 1.1%). JEV confirmation was significantly higher in enrolled cases than referred cases (10.2%) (p < 0.05). However, the contribution of JEV in AES cases was similar in both children and adults. JE was reported year-round and from adjacent non-endemic districts. CONCLUSIONS: The Japanese encephalitis virus continues to be the leading cause of acute encephalitis syndrome in central India despite vaccination among children. Surveillance needs to be strengthened along with advanced diagnostic testing for assessing the impact of vaccination.
Assuntos
Encefalopatia Aguda Febril , Vírus da Encefalite Japonesa (Espécie) , Encefalite Japonesa , Leptospirose , Febre Tifoide , Encefalopatia Aguda Febril/epidemiologia , Encefalopatia Aguda Febril/etiologia , Adulto , Criança , Encefalite Japonesa/diagnóstico , Encefalite Japonesa/epidemiologia , Hospitalização , Humanos , Índia/epidemiologia , SimplexvirusAssuntos
Anemia Falciforme/genética , Proteínas de Transporte/genética , Hemoglobina Fetal/genética , Proteínas de Ligação ao GTP/genética , Genes myb/genética , Proteínas de Choque Térmico HSP70/genética , Proteínas Nucleares/genética , Fatores de Alongamento de Peptídeos/genética , Polimorfismo de Nucleotídeo Único/genética , Adulto , Anemia Falciforme/diagnóstico , Estudos de Coortes , Feminino , Seguimentos , Humanos , Masculino , Proteínas Repressoras , Índice de Gravidade de DoençaRESUMO
OBJECTIVES: Sickle cell anemia is the commonest genetic disorder in India, and the frequency of the sickle cell gene is very high in the remote tribal areas where facilities are generally limited. Therefore, a rapid and affordable point-of-care test for sickle cell disease is needed. METHODS: The diagnostic accuracy of HemoTypeSC was evaluated against automated high-performance liquid chromatography (HPLC) as the gold standard for its efficacy in a newborn screening program. RESULTS: A total of 1,559 individuals (980 newborns and 579 adults) from four participating centers were analyzed by both methods. HemoTypeSC correctly identified 209 of 211 total hemoglobin (Hb) SS cases, for a 99.1%/99.9% total HbSS sensitivity/specificity. Overall, HemoTypeSC exhibited sensitivity and specificity of 98.1% and 99.1% for all possible phenotypes (HbAA, HbAS, and HbSS) detected. HPLC is relatively expensive and not available in most laboratories in remote tribal areas. CONCLUSIONS: We conclude that the rapid, point-of-care testing device HemoTypeSC test is suitable for population and newborn screening for the HbS phenotype.
Assuntos
Anemia Falciforme/diagnóstico , Hemoglobina A/análise , Hemoglobina Falciforme/análise , Sistemas Automatizados de Assistência Junto ao Leito , Testes Imediatos , Adulto , Anemia Falciforme/sangue , Cromatografia Líquida de Alta Pressão , Humanos , Índia , Recém-Nascido , Fenótipo , Estudos ProspectivosRESUMO
There is clinical variability in the presentation of sickle cell disease among Indians. Vaso-occlusive crisis is common among non-tribal patients. Hydroxyurea, induces fetal hemoglobin (HbF) synthesis and reduces the clinical severity of sickle cell disease but individual patients have a variable response. This study was undertaken to investigate the efficacy and safety of hydroxyurea in Indians with severe manifestations where the beta(s) gene is linked to the Arab-Indian haplotype and is associated with higher HbF levels. Seventy-seven patients (29 adult sickle homozygous, 25 pediatric sickle homozygous, 23 adult sickle beta-thalassemia) selected for hydroxyurea therapy were evaluated for clinical, hematological, biochemical and genetic parameters and were followed for 24 months. Ninety-eight point seven percent of the sickle chromosomes were linked to the Arab-Indian haplotype, 27% of patients had associated alpha thalassemia and 65% were Xmn I +/+. Seventy-eight percent of the patients had no further crises after starting hydroxyurea. This effect was accompanied by a significant increase in HbF (p<0.001), but this increase was variable in individual cases. There was also an increase in gamma gene mRNA expression in the few cases so studied. Hemoglobin levels increased significantly (p<0.001) resulting in the cessation of blood transfusions. Leucopoenia was observed in one patient. Hydroxyurea was effective in reducing the clinical severity in Indian patients who initially had higher HbF levels and the presence of ameliorating factors, such as alpha-thalassemia and the Xmn I polymorphism. Hydroxyurea therapy with careful monitoring can thus change the quality of life of Indians with sickle cell disease.
Assuntos
Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/genética , Haplótipos , Hidroxiureia/uso terapêutico , Adolescente , Adulto , Anemia Falciforme/sangue , Criança , Pré-Escolar , Etnicidade/genética , Feminino , Hemoglobina Fetal/biossíntese , Hemoglobina Fetal/genética , Hemoglobina Falciforme/genética , Humanos , Hidroxiureia/administração & dosagem , Hidroxiureia/efeitos adversos , Índia , Masculino , Adulto Jovem , Globinas beta/genética , Talassemia beta/sangue , Talassemia beta/tratamento farmacológico , Talassemia beta/genéticaRESUMO
AIM: Establishment of baseline epidemiology of intussusception in developing countries has become a necessity with the possibility of reintroduction of rotavirus vaccine. The current study assessed the seasonal trend in cases admitted with intussusceptions and dehydrating acute watery diarrhoea in children aged 2 months to 10 years. METHODS: In a prospective surveillance study, teaching and research hospital sites in India (Lucknow and Nagpur), Brazil (Fortazela), Egypt (Ismailia) and Kenya (Nairobi) established a surveillance where a network of hospitals with surgical facilities catered to a reference population of about 1-2 million for reporting of intussusception. One large hospital per site also recruited admitted cases of acute watery diarrhoea. RESULTS: From April 2004 to March 2006, 173 and 2346 cases of intussusception and diarrhoea, respectively, were recruited. Cases of intussusception had no apparent seasonality. Most cases of intussusception (61.3%) (107/173) were in the < or =1 year age group, with males comprising 68.8% (119/173) of all cases. Hospital mortality of intussusception was 4.2% (4/96). Cases of diarrhoea peaked in March, with 56.6% (1328/2346) of admitted cases being males. Majority (83.1%) of cases of diarrhoea had received antibiotics, and the hospital mortality was 0.8% (18/2280). CONCLUSION: Intussusception in the four participating countries exhibited no seasonal trend. We found that it is feasible to establish a surveillance network for intussusception in developing countries. Future efforts must define population base before the introduction of rotavirus vaccine and continue for some years thereafter.
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Países em Desenvolvimento , Diarreia/epidemiologia , Intussuscepção/epidemiologia , Vigilância da População , Pré-Escolar , Diarreia/prevenção & controle , Feminino , Humanos , Imunoterapia Ativa , Lactente , Intussuscepção/prevenção & controle , Masculino , Estudos Prospectivos , Rotavirus/imunologia , Rotavirus/isolamento & purificação , Estações do AnoRESUMO
This study evaluated the effect of alpha thalassemia on the red cell indices and hemoglobin profiles of normal, sickle heterozygous and sickle homozygous newborn babies in central India where the sickle gene is linked to the Arab-Indian haplotype. 265 newborn babies were analysed with complete blood count and hemoglobin analysis on high performance liquid chromatography (Variant Hb Testing System, BioRad Laboratories, Hercules, CA, USA) using the ß-thal short program. The sickle genotypes was confirmed by DNA analysis. The two common alpha gene deletions (- α3.7 and - α4.2) were detected by multiplex PCR. Among the 102 normal, 106 sickle heterozygous and 57 sickle homozygous newborns, the prevalence of a single alpha gene deletion (- α/αα) was 28.3% and that of deletion of 2 alpha genes (- α/- α) was 21.5%. In all, 57 normal (55.9%), 35 (33.0%) sickle heterozygous and 41 (71.9%) sickle homozygous newborns had a normal α genotype while - α/- α was seen in 23 (22.5%) normal, 30 (28.3%) sickle heterozygous and 4 (7.0%) sickle homozygous newborns respectively. The presence of associated alpha thalassemia resulted in a reduction in the hemoglobin levels and red cell indices in normal, sickle heterozygous and sickle homozygous newborn babies, MCV and MCH being strong discriminators of alpha thalassemia with two alpha gene deletions in all the three groups. This study also helped us to know the variations in hematological parameters in normal, sickle heterozygous and sickle homozygous newborns with and without associated α thalassemia.