A study of clinical and hematological profile of children with sickle cell disease in a tertiary care hospital, Valsad, India

Patel, Kinjal; Chaudhari, Chintu C.; Sharma, Diwakar

doi:10.18203/2349-3291.ijcp20172609

Cited by 5 publications

(5 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A comparable outcome was recorded among akin participants in North Maharashtra, India, where low levels of Hb concentration, RBC count, and PCV was documented [25]. Likewise, Patel and colleagues [26] also reported significant low level of hemoglobin and HCT among patients with HbS genotype. e mean cell volume recorded in this study was high, supporting the known fact that MCV usually is high in SCD patients due to the increasing need of erythropoiesis in response to chronic hemolysis leading to macrocytosis [27].…”

Section: Discussionsupporting

confidence: 53%

Assessment of Hemoglobin Variants in Patients Receiving Health Care at the Ho Teaching Hospital: A Three-Year Retrospective Study

Awaitey

Akorsu

Allotey

et al. 2020

Advances in Hematology

View full text Add to dashboard Cite

Background. It is estimated that one out of every three Ghanaians has hemoglobin genotype mutation. This change in genetic make-up may result in genotypes such as HbAS, HbSS, and HbSC. Many children in low- and middle-income countries die even before they are diagnosed with sickle cell disease (SCD). In Africa, there are limited data on the incidence and prevalence of SCD and the Volta region of Ghana is no exception. Aim. The aim of this study was to determine the prevalence of SCD and to assess the hemoglobin variants among patients attending Ho Teaching Hospital. Methods. A retrospective study design was used to extract information from the Hospital Administration and Management Systems (HAMS) on the hemoglobin electrophoresis results and corresponding full blood count results of the SCD and sickle cell anemia (SCA) patients as well as patients who were asked to do Hb electrophoresis irrespective of their sickling status. Data were collected for the period January 2016 to December 2018. Sickle cell disease status was determined using the Hb genotypes from the Hb electrophoresis results. The full blood count was used to categorize the severity of anemia based on the hemoglobin concentration in the SCA and SCD patients. Results. A total of 1,523 subjects were included in the study of which the prevalence for sickle cell disease was 16.7%. The SCD genotypes included HbS (6.2%), HbSC (7.9%), and HbSF (2.6%). Hemoglobin C disease (HbCC) constituted 0.3% out of the total prevalence of SCD. The prevalence of anemia was 99.2%, with the severest form in HbS. Also, majority of the SCD patients had severe anemia. Difference in the severity of anemia was found to be significant among both male (P=0.006) and female (P=0.004) participants with SCD. Conclusion. Patients receiving health care at the Ho Teaching Hospital had different hemoglobin variants with HbAS recording the highest prevalence. The high incidence of hemoglobin AS implies the possibility of having an increased population of individuals with sickle cell disease in future if measures are not put in place to improve screening, counseling, and education of the public about the health threat SCD poses.

show abstract

Section: Discussionsupporting

confidence: 53%

Assessment of Hemoglobin Variants in Patients Receiving Health Care at the Ho Teaching Hospital: A Three-Year Retrospective Study

Awaitey

Akorsu

Allotey

et al. 2020

Advances in Hematology

View full text Add to dashboard Cite

show abstract

“…A significant proportion of our patients had microcytic anemia. This observation has also been reported by authors in India, who associate it with martial deficiency, in a population of sickle cell children from tribes and castes with a low socioeconomic level [33,34]. In our study, two findings support a more inflammatory origin: i) the demonstration of a statistically significant association between very high CRP levels and microcytosis; ii) the observation of a simple negative linear correlation, statistically significant (P = 0.003), between CRP levels and CMV.…”

Section: Discussionsupporting

confidence: 85%

“…This trend was also reported in a study of sickle cell children admitted to a tertiary hospital in India. Indeed, Patel et al reported a mean Hb level of 8.08 ± 2.40 g/dl with H of 23.34 ± 6.40% and CMV of 75.99 ± 2.12 µ 3 [33]. In Saudi Arabia, El-Ghany et al found a mean Hb level of 8.2 ± 1.5 g/dl (23), whereas we found a mean Hb level of 7.2 ± 1.9 g/dl, an H of 22.9 ± 7.7% and a CMV of 75.7 ± 10.1µ 3 .…”

Section: Discussionmentioning

confidence: 99%

Clinical Features and Outcome of Sickle Cell Patients in Pediatric Emergency Departments in Lubumbashi, Democratic Republic of Congo

Luntadila,

Mwanet,

Natuhoyila

et al. 2024

JAMMR

View full text Add to dashboard Cite

Aims: To describe the epidemiology clinical outcome, and therapeutic aspects of sickle cell patients admitted to pediatric emergency departments, and to identify the risk factors associated with the clinical outcome. Study Design: Descriptive cross-sectional study. Place and Duration of Study: University of Lubumbashi teaching hospital, pediatric emergency departments and Jason Sendwe Referral Hospital from 10 February 2020 to 30 April 2022. Methodology: 105 children with sickle cell disease aged 6 months to 16 years were admitted and recruited in the study. The Kaplan-Meier method was used to analyze survival to D22, the log-rank test to compare survival curves, and Cox regression to identify mortality risk factors, at a significance level of p ˂ 0.05. Results: The main reasons for admission were infection (83.8%), hyperalgesic vasoocclusive crisis (73.3%) and severe anemia (36.2%). According to the Adegoke severity score, 60% of the children had a severe clinical profile, 32.4% were moderate and 7.6% were mild. Median survival was 5.9 days. Survival decreased from 80% on day 2 to 67.6% on day 22. Survival was significantly shorter for moderate and severe clinical profiles (P = 0.001), transfer from a peripheral hospital (P = 0.006), and diagnosis of an infectious syndrome (P = 0.002). The critical period was the first 2 days of hospitalization, with a mortality rate at 20% compared with an all-cause mortality rate at 12.4%. In the adjusted multivariate analyses, death risk factors were transfer (P=0.04), severe clinical profile (P=0.033), hospital stay >2 days (P=0.04), infectious syndrome (P=0.01) and suspected hepatocellular failure (P=0.009). Conclusion: Sickle cell morbidity and mortality in Lubumbashi are high and associated with mostly controllable risk factors. The prognosis for sickle cell disease can be improved by training health workers in sickle cell disease and by better organizing specific care at all levels of the health pyramid.

show abstract

“…A sample size of 100 subjects, consisting of previously diagnosed cases of SCA (SS pattern), was included in the study. The sample size was calculated using a previous study by Patel et al [ 6 ], which observed that out of 47 patients with sickle cell disease, 59.57% had an acute painful crisis, 25.53% had an acute febrile illness (AFI), 2.12% had acute chest syndrome, and 46.80% had severe anaemia. Taking this value as a reference, the minimum required sample size with a 10% margin of error and a 5% level of significance is 97 patients.…”

Section: Methodsmentioning

confidence: 99%

Clinical Profiles of Children With Sickle Cell Anaemia Presenting With Acute Clinical Events: A Single-Center Study

Singh¹,

Bokade²,

Tirpude³

et al. 2023

Cureus

View full text Add to dashboard Cite

Background: Sickle cell disease is a common genetic disorder characterised by chronic haemolytic anaemia and vaso-occlusive crisis. Sickle cell anaemia (SCA) has both short-term effects in the form of acute clinical events and long-term repercussions seen with chronic multiorgan involvement. It is associated with significant morbidity and mortality. In India, the disease is largely undocumented. Thus, there is an urgent need to highlight the features of the disease so that locally appropriate models of care may be implemented. Objective: This study aims to evaluate acute clinical events in SCA and to provide data that may help to reduce the rate of morbidity and mortality associated with this disease by early interventions. Materials and methods: A cross-sectional observational study was conducted between November 2020 and May 2022 at Indira Gandhi Government Medical College and Hospital, Nagpur, Central India. The inclusion criteria included previously diagnosed patients of SCA (homozygous sickle cell disease) on high-performance liquid chromatography (HPLC) between the age groups of six months and 12 years, presenting with acute clinical events. The exclusion criteria included patients younger than six months and older than 12 years of age, and all patients with other haemoglobinopathies and sickle cell trait. The study was approved by the Institutional Ethical Committee. All the data was entered into a well-designed Microsoft Office Excel spreadsheet (v 2019, Microsoft, Washington, USA). All the clinical, biochemical, and haematological data were tabulated and analysed. Results: A total of 100 children with sickle cell disease diagnosed by HPLC were enrolled during the study period. About 215 acute clinical events among the 100 cases were recorded, for which they were admitted to the paediatric ward or PICU. The majority (35%, n=35) were seen in the age group of six to nine years (school-going age). About 52% were male and 48% were female (male-to-female ratio= 1.08:1). Pain was the most common symptom. The highest incidence of 36.75% (n=79) was seen with acute painful crises and was the most common indication of hospitalisation, followed by acute febrile illness (AFI) (34.42%, n=74), aplastic crisis (10.23%, n=22), splenic sequestration crisis (9.77%, n=21), hepatobiliary involvement (3.72%, n=8), acute chest syndrome and haemolytic crisis (each 1.86%, n=4), and stroke (1.40%, n=3). In cases of having foetal haemoglobin (HbF) ≥20%, the incidence of acute painful crisis (p=0.0001), hand-foot syndrome (p=0.047), aplastic crisis (p=0.033), splenic sequestration crisis (p=0.039), and AFI (p=0.035) was low as compared to cases having HbF ≤20% which was statistically significant. The incidence of acute painful crisis, hand-foot syndrome, and an aplastic crisis was significantly low in patients receiving hydroxyurea therapy as compared to patients who were not on hydroxyurea. Out of 100 cases, four died during the study period, three died because of splenic sequestration crisis...

show abstract

A study of clinical and hematological profile of children with sickle cell disease in a tertiary care hospital, Valsad, India

Cited by 5 publications

References 9 publications

Assessment of Hemoglobin Variants in Patients Receiving Health Care at the Ho Teaching Hospital: A Three-Year Retrospective Study

Assessment of Hemoglobin Variants in Patients Receiving Health Care at the Ho Teaching Hospital: A Three-Year Retrospective Study

Clinical Features and Outcome of Sickle Cell Patients in Pediatric Emergency Departments in Lubumbashi, Democratic Republic of Congo

Clinical Profiles of Children With Sickle Cell Anaemia Presenting With Acute Clinical Events: A Single-Center Study

Contact Info

Product

Resources

About