scholarly journals Death due to sickle cell anemia: autopsy diagnosis

2017 ◽  
Vol 5 (7) ◽  
pp. 3185
Author(s):  
Manjusha Shripad Dhawle ◽  
Ashwini Radhakrishan Tangde ◽  
Santosh Govind Rathod ◽  
Rajan S. Bindu
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Tertiary Care ◽  
Medical Officer ◽  
Clinical History ◽  
Care Hospital ◽  
Cell Disease ◽  
Unexpected Death ◽  
Mortality And Morbidity ◽  
History Of

Background: Sickle cell disease (SCD) is well known and is the commonest hereditary hematological disorder which is associated with increased mortality and morbidity.  They are group of inherited haemoglobinopathies caused by the occurrence of hemoglobin S (Hbs) in homozygous or heterozygous form or in combinations of Hbs with another hemoglobin such as Hbsc or beta thalassaemia (Hbs-thal). Sickle cell syndromes are remarkable for their clinical heterogenecity including their presentations as sudden and unexpected death due to sickle cell crises. While doing autopsy in cases of deaths with no apparent cause and physical over activity medical officer must keep in mind the possibility of death due to vasoocclusive crisis in sickle cell disease. Aim of the study was to create awareness among the physicians and relatives / public and to minimize future unexpected death from complications or crisis from SCD.Methods: This is a retrospective study of 10 cases carried in the department of pathology, in tertiary care hospital and covers a period from January 2009 to December 2016. These cases were brought dead to the casualty with a history of sudden death. After post mortem examination, the specimens were sent for histopathological examination.Results: The record of 10 cases was reviewed. Out of our ten cases 7 were male and 3 were females. The youngest person was 17-year female and oldest was 65 years male. In clinical history 3 cases had complains of chest pain (30%), 3 others had complained of breathlessness (30%), 2 had history of unconsciousness (20%), one case had complained of abdominal pain (10%) and one case had a history of fall and injury (10%). Microscopic examination of each organ was carried out.  Organs like lungs, liver, spleen, kidneys, heart and brain showed wide spread congested vessels which were stuffed with RBC.Conclusions: We present this study to emphasize that sickle cell crisis is one of the cause of sudden unexplained death and highlight the importance of considering sickle cell disease as a cause of death in cases with no apparent cause.

scholarly journals Epidemiological and clinical study of sickle cell disease at tertiary care centre in western India

2019 ◽  
Vol 7 (1) ◽  
pp. 97
Author(s):  
Varsha P. Patel ◽  
Archana U. Gandhi ◽  
Chineen Shah
Keyword(s):  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Tertiary Care ◽  
Clinical Profile ◽  
Acute Chest Syndrome ◽  
Care Hospital ◽  
Cell Disease ◽  
Tribal Communities

Background: Sickle cell disorders are structural hemoglobinopathies, rendering red blood cells sickle shaped, less deformable and sticky leading to microvascular vaso-occlusion and premature red blood cells destruction which leads to varied clinical manifestations. It leads to lifelong morbidity thus affecting quality of life and contributes to early mortality thereby reducing the key national resources- the healthy workforce. This study was done to evaluate epidemiological and clinical profile of sickle cell disease attending the centre.Methods: This study was cross-sectional, observational study conducted at tertiary care hospital in Gujarat. After taking ethical clearance patients were enrolled as per inclusion and exclusion criteria and epidemiological and clinical profile of sickle cell disease patients was studied.Results: Mean age of sickle cell disease was 22.58 years. It was found in tribal communities of Gujarat like Rathwa, Baria, Tadvi etc. Commonest symptom was musculoskeletal pain (86.84%), followed by jaundice (71.05%), fever, dyspnoea, abdominal pain and chest pain. Most common systemic manifestation was pain crises (60.66%), followed by hemolytic anemia (31.15%), acute chest syndrome (30%), consolidation (11.67%), hepatopathy (10%) and avascular necrosis of hip. (6.56%).Conclusions: Sickle cell disease is seen in younger patients. In Gujarat mainly tribal communities are affected. Major systemic manifestations of sickle cell disease include pain crisis followed by hemolytic crisis, acute chest syndrome, hepatopathy and AVN of hip.    

Validation of a Non-Invasive Hemoglobin Estimation in Patients with Sickle Cell Disease

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4770-4770
Author(s):  
Murtadha K. Al-Khabori ◽  
Abdulhakeem Al-Hashim ◽  
Zeba Jabeen ◽  
Khalil Al Farsi ◽  
Mohammed Al-Huneini ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Correlation Coefficient ◽  
Sickle Cell ◽  
Conflicts Of Interest ◽  
Pearson Correlation ◽  
Tertiary Care ◽  
Coefficient Of Determination ◽  
Care Hospital ◽  
Cell Disease ◽  
Non Invasive

Abstract Abstract 4770 Introduction: The care of patients with Sickle Cell Disease (SCD) is frequently complicated by difficult venous access complicating blood sampling for laboratory investigations including hemoglobin (Hb) measurement. Non-invasive hemoglobin concentration monitoring is a potential solution, albeit not validated yet in patients with SCD. The primary objective of this study was to validate non-invasive pulse CO-oximetry based hemoglobin estimation in this patient population. Methods: We conducted a prospective observational study on patients with SCD admitted to the inpatient wards over 4 weeks in a tertiary care hospital. We estimated a spot Hemoglobin (Sp Hb) measurement using Masimo Pronto-7 Pulse CO-oximetry device (two measurements per patient) and compared it to a venous sample Hb (Reference Hemoglobin; Ref Hb) measured using Abbott CELL-DYN Sapphire hematology analyzer. We calculated Pearson correlation coefficient and coefficient of determination (R2). The multivariable linear regression model of predicting the estimation differences included age, gender, weight, height, blood pressure and reference hemoglobin. Results: We enrolled 98 patients (45 males, 53 females) with a mean age of 26 years (SD 8.8; 14–75) and a mean Ref Hb of 9.2 g/dL (SD 1.5; 5.3–13). The mean Sp Hb was 10.1 g/dL (SD 2.0; 5.3–14.5). The correlation coefficient between the Sp Hb and Ref Hb was 0.54 (R2 = 29%) with a mean difference of 0.9 g/dL (SD 1.7; −4.8 to 4.5). In the multivariable model, gender (p =0.042) and Ref Hb level (p=0.001) were statistically significant predictors for the difference in measurement. A strong correlation between the two CO-oximetry Hb measurements was obtained (correlation coefficient = 0.81, R2 = 65%). Conclusions: Our study demonstrated the validity of the CO-oximetry Hb measurement in adult patients with SCD. Larger prospective studies are needed to confirm our results. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Troponin elevation in sickle cell disease

2021 ◽  
Author(s):  
Nuri I. Akkus ◽  
Saurabh Rajpal ◽  
Jeffrey Hilbun ◽  
Ashish Dwary ◽  
Thomas R. Smith ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Troponin I ◽  
Tertiary Care ◽  
Care Hospital ◽  
Elevated Troponin ◽  
Cell Disease ◽  
Troponin Elevation ◽  
Pain Crisis

Abstract: Objective: Sickle Cell Disease (SCD) is associated with cardiovascular abnormalities. Troponin is not typically measured in this population and significance of having abnormal troponin is unknown. We wanted to evaluate use of troponin and factors that predispose troponin elevation in patients admitted with Sickle Cell Pain Crisis (SCPC). Methods: We reviewed data of consecutive patients admitted to a tertiary care hospital between 2006-2011 with a diagnosis of SCPC. Subjects with Elevated Troponin (ET) group (troponin I > 0.04 ng/ml) were compared with those with Normal Troponin (NT group) for demographics, risk factors, presence of echocardiography-derived tricuspid regurgitant jet velocity (TRV) ≥3 m/s suggesting pulmonary hypertension (PH)and laboratory tests. Mann-Whitney U test was used to compare groups. Results: 283 of 724 patients admitted with SCPC had chest pain. Troponin I was measured in 63 patients,51 had normal troponin and 12 had elevated troponin ranging from 0.06 to 3.42 ng/ml. ET was associated lower hemoglobin (p=0.02), lower Hematocrit (p=0.02), lower platelet number (P<0.001), higher LDH (p=0.012), higher AST levels (p=0.004), higher bilirubin (p=0.006) level and TRV ≥3 m/s (p=0.028). Conclusions: In less than 10% of SCPC patient’s troponin was measured and among those about 1 out of 5 of them had an elevated troponin. Troponin elevation was not associated with traditional cardiovascular risk factors but associated with lower hematocrit, elevated LDH, bilirubin levels and TRV ≥3 m/s.

Cognitive Functioning and Brain Magnetic Resonance Imaging in Children With Sickle Cell Disease

PEDIATRICS ◽  
1996 ◽  
Vol 97 (6) ◽  
pp. 864-870 ◽  
Author(s):  
F. Daniel Armstrong ◽  
Robert J. Thompson ◽  
Winfred Wang ◽  
Robert Zimmerman ◽  
Charles H. Pegelow ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Brain Magnetic Resonance Imaging ◽  
Clinical History ◽  
Neuropsychological Evaluation ◽  
Resonance Imaging ◽  
Cell Disease ◽  
Only Children ◽  
History Of ◽  
Neuropsychological Evaluations

Objective. Brain magnetic resonance imaging (MRI) and neuropsychological evaluations were conducted to determine whether neuroradiographic evidence of infarct in children with sickle cell disease between ages 6 and 12 years would result in impairment in cognitive and academic functioning. Method and Design. Children enrolled in the Cooperative Study of Sickle Cell Disease were evaluated with brain MRI and neuropsychological evaluation. Completed studies were obtained for 194 children, 135 with HbSS. MRIs were categorized according to the presence of T2-weighted, high-intensity images suggestive of infarct and were further categorized on the basis of a clinical history of cerebrovascular accident (CVA). An abnormal MRI but no clinical history of CVA was classified as a silent infarct. Neuropsychological evaluations included assessment of both global intellectual functioning and specific academic and neuropsychological functions. Results. Central nervous system (CNS) abnormalities were identified on MRI in 17.9% of the children (22.2% of children homozygous for HbS), and a clinical history of CVA (N = 9, 4.6%) was identified in only children with HbSS disease. Subsequent analyses examined only children with HbSS. Children with a history of CVA performed significantly poorer than children with silent infarcts or no MRI abnormality on most neuropsychological evaluation measures. Children with silent infarcts on MRI performed significantly poorer than children with no MRI abnormality on tests of arithmetic, vocabulary, and visual motor speed and coordination. Conclusions. These results substantiate the importance of careful evaluation, educational planning, and medical intervention for CNS-related complications in children with sickle cell disease.

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

2017 ◽  
Vol 4 (4) ◽  
pp. 1317 ◽  
Author(s):  
Kinjal G. Patel ◽  
Chintu Chaudhari ◽  
Diwakar Sharma
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Trait ◽  
Tertiary Care ◽  
Morbidity And Mortality ◽  
Care Hospital ◽  
Age Group ◽  
Cell Disease ◽  
Significant Difference ◽  
The Common

Background: Sickle cell disease is commonly seen in rural population of south part of Gujarat in India. It is one of the common causes of recurrent hospitalization, morbidity and mortality in pediatric population. This study was therefore undertaken to evaluate the clinical profile of sickle cell disease in a tertiary care hospital.Methods: This was the prospective observational study done from November 2015 to October 2016. All the hospitalized diagnosed case of sickle cell disease and trait in age group of 6 months to 14 years were taken in this study. Sickle cell disease with some genetic or metabolic disease and sickle-beta-thalassemia patients were not included in this study.Results: Total 61 patients were admitted over a one year of study period, out of which 47 were sickle cell disease and 14 sickle cell trait patients. Morbidity events were commonly observed in 5-12 years of age groups (68.85%). Seasonal variation also observed, 47.54% of total cases are seen in winter season. Pain (60.65%) was the most common presenting symptom. Severe pallor (39.34%) and splenomegaly (24.59%) was the most common sign in both groups. Vaso-occlusive crisis (59.01%) was the most common morbidity event observed, of which abdominal pain was the most common site of pain involvement. On laboratory analysis, there was statistically significant difference observed in disease and trait. In patients with sickle cell disease acute painful crisis (59.57%) was the common morbidity event observed while in sickle cell trait patients acute febrile illness (71.42%) observed.Conclusions: Vaso-occlusive crisis is the commonest manifestation in pediatric age group. Comprehensive medical care and management is required to decrease the morbidity and mortality. 

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