Identification of Clinical and Laboratory Parameters Associated with the Development of Acute Chest Syndrome during Vaso-Occlusive Episodes in Children with Sickle Cell Disease: A Preliminary Step before Assessing Specific and Early Treatment Strategies

This prospective observational study sought to ascertain clinical and laboratory parameters associated with the development of acute chest syndrome (ACS) during vaso-occlusive episodes (VOE) in children with sickle cell disease (SCD). It was performed at the pediatric department of the university Intercommunal Créteil hospital. All children with SCD (all sickle genotypes) consecutively admitted from November 2013 to December 2016 for painful VOEs and no evidence of ACS were included. Clinical and laboratory parameters collected at admission and within 48 h after admission were compared for children in whom ACS developed or not. Variables that were statistically significant on univariate analysis or considered to be clinically relevant were included in a multivariable model to ascertain the risk factors associated with the development of ACS during a VOE. The variables retained in the multivariate model were used to construct a predictive score for ACS. For each included child and during the study period, only data from the first VOE and/or the first ACS were analyzed. Among 191 hospitalizations for painful VOEs, for 176 children with SCD, ACS developed in 35 during hospitalization. Mean hospital stay was longer for children with ACS versus VOEs alone (7.6 (±2.3) vs. 3.3 (±1.8) days, p < 0.0001), and all children with ACS versus 28/156 (17.9%) with VOEs alone received red blood cell transfusion (p < 0.0001). The multivariate model retained pain score (≥9/10), pain localization (abdominal or spinal pain or involving more than two limbs), and high reticulocyte (≥260 × 109/L) and neutrophil (>10 × 109/L) counts, at admission, as independently associated with ACS development. The area under the receiver operating characteristic curve for the ACS predictive score was 0.82 (95% CI: 0.74–0.89), and the negative predictive value was 97.7%. The evolution profiles during the first 48 h differed between children with ACS and VOEs alone, with a more rapid decline of pain score and leucocytosis in children with VOEs. Clinical and laboratory measurements at admission may be simple parameters to identify children with increased risk of ACS development during VOEs and to facilitate early diagnosis of this respiratory complication. Also, the persistent elevation of leukocyte count on day 2 may be considered a sign of evolving ACS.

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A case of severe multi-system disease in a sickle cell SC patient caused by parvovirus B19 infection

Journal of Clinical Images and Medical Case Reports ◽

10.52768/2766-7820/1314 ◽

2021 ◽

Vol 2 (5) ◽

Author(s):

Mohamed Almuqamam ◽

◽

Swetha Madhavarapu ◽

Nataly Apollonsky ◽

◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Parvovirus B19 ◽

Acute Infection ◽

Organ Injury ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Splenic Sequestration ◽

Increased Risk ◽

Parvovirus B19 Infection

Sickle Cell Disease (SCD) is an inherited hemoglobinopathy, which results in production of abnormal hemoglobin S. HbSC disease is a variant of SCD, which shares a similar clinical complication profile to HbSS disease, but often thought to be a milder condition. In patients with SCD, Hb S in deoxygenated state undergoes polymerization, leading to hemolysis, vaso-occlusive events, and eventually end-organ damage. Among other complications in patients with SCD is increased risk of complications caused by parvovirus B19. We present a case of a 14-year-old female with HbSC disease who presented to the emergency room with complaint of abdominal pain and found to have splenic sequestration. Splenic sequestration progressed rapidly, Hemoglobin (hb) dropped to 4.6 g/dl and acute chest syndrome (ACS) developed. She was treated following the ACS protocol, received 4 units of Packed Red Blood Cells (PRBC) and subsequently underwent a single volume PRBC exchange transfusion. Considering her unusual presentation, with severe ARDS from alveolar hemorrhage requiring mechanical ventilation and multi-organ injury, several autoimmune and infectious conditions with a cytokine storm component including COVID-19 disease, were considered. Results of viral testing revealed parvovirus B19 IgM antibodies signifying an acute infection. She fully recovered with supportive care and was discharged home. Multisystem involvement simulating connective tissue disorders or malignancies with acute parvovirus B19 infection has been reported and is considered extremely rare. To our knowledge, there were no reports of pediatric patients with SC disease presenting with splenic sequestration and ACS in the setting of parvovirus B19 multisystem disease. Keywords: sickle cell disease; acute respiratory distress syndrome; acute chest syndrome; parvovirus B19.

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Sickle crisis in the critically ill

10.1093/med/9780199600830.003.0275 ◽

2016 ◽

Author(s):

Shilpa Jain ◽

Mark T. Gladwin

Keyword(s):

Sickle Cell Disease ◽

Blood Cell ◽

Sickle Cell ◽

Red Blood Cell ◽

Systemic Infection ◽

Red Blood Cell Transfusion ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Ischaemia Reperfusion Injury ◽

Transfusion Therapy

Sickle cell disease crises are precipitated by an acute occlusion of microvessels, which can lead to end organ ischaemia reperfusion injury and acute haemolysis. Acute fat emboli syndrome, acute lung injury (the acute chest syndrome), acute pulmonary hypertension, and cor pulmonale, haemorrhagic and occlusive stroke, and systemic infection represent the most common life-threatening complications observed in current ICU practice. General principles of management in all patients admitted to the critical care unit are hydration, antibiotics, pain control, and maintenance of oxygenation and ventilation. Red blood cell transfusion therapy is the treatment of choice for most complications of sickle cell disease requiring intensive care management. Transfusion of sickle negative, leukoreduced red blood cells, phenotypically matched for Rhesus and Kell antigens is the minimum standard of care in sickle cell disease patients as they have a high incidence of red blood cell alloimmunization.

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Polymorphisms in TNFα Are Associated with Cerebrovascular Events in Sickle Cell Disease.

Blood ◽

10.1182/blood.v114.22.1540.1540 ◽

2009 ◽

Vol 114 (22) ◽

pp. 1540-1540 ◽

Cited By ~ 1

Author(s):

Latorya A Barber ◽

Allison E Ashley-Koch ◽

Melanie E. Garrett ◽

Karen L Soldano ◽

Marilyn J. Telen

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Conflicts Of Interest ◽

Umbilical Vein ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Data Set ◽

Clinical Complications ◽

Cerebrovascular Events ◽

Increased Risk

Abstract Abstract 1540 Poster Board I-563 Tumor necrosis factor alpha (TNFα) is a pro-inflammatory cytokine that stimulates phagocytosis, neutrophil recruitment, and expression of adhesion molecule VCAM-1. Plasma levels of TNFα have been found to be increased in sickle cell disease (SCD), and in vitro studies show that TNFα causes increased adherence of sickle red blood cells to human umbilical vein endothelial cells. A polymorphism in the promoter region of the TNFα gene has previously been associated with stroke in children with SCD (Hoppe et al., 2007). The current study was designed to identify associations of additional TNFα single nucleotide polymorphisms (SNPs) with SCD clinical complications. We analyzed five SNPs in the TNFα gene in 509 DNA samples of SCD patients from Duke University, University of North Carolina at Chapel Hill, and Emory University. In our data set, cerebrovascular events (CVEs), including overt stroke, seizures, and transient ischemic attacks, occurred in 133 out of 509 SCD patients (26.1%). SNP genotyping was performed using Taqman genotyping assays from Applied Biosystems. Due to low minor allele frequencies (<0.05) for all the SNPs examined, genetic associations with SCD clinical complications were examined by using allele tests. After controlling for age, gender, and use of hydroxyurea, two of the five TNFα SNPs, rs2228088 and rs3093665, were significantly associated with CVEs (p=0.013 and 0.029, respectively). The odds that SCD patients with a G allele at rs2228088 suffered from CVEs were 0.485 times that for patients with the T allele, suggesting that the G allele had a protective effect. The odds of having the A allele at rs3093665 and suffering from CVEs was also reduced, at 0.45 compared to the C allele. Neither SNP was found to be in linkage disequilibrium (LD) with any of the other SNPs analyzed (r2≤0.002). There was also strong association of SNP rs2228088 with acute chest syndrome (ACS; p=0.003), occurring in 382 out of 509 SCD patients (75%). However, in this analysis, the G allele was associated with increased risk for ACS (OR=2.313). In addition to the association with CVEs, the SNP rs3093665 was also significantly associated with priapism (p=0.03), reported by 86 of 223 male SCD patients (38.6%). In this analysis, the A allele was protective, as had been observed for CVE (OR=0.188). Additionally, we found no difference in steady state plasma TNFα levels between genotypes for the two SNPs. The functional significance of these SNPs is presently unknown. SNP rs2228088 is a synonymous SNP located in the coding region, and rs3093665 is located in the 3' untranslated region of the TNFα gene. While the G to T change at SNP rs2228088 does not translate to a change in amino acid sequence, the A to C change at SNP rs3093665 may affect mRNA stability due to its location. It is also possible that one or both of these SNPs is in LD with another functionally relevant SNP. Our findings thus support previous data implicating TNFα polymorphisms in risk for central nervous system events. Interestingly, ACS has been previously associated with seizures, stroke and altered mental status in adults and children with SCD (Vinchinsky et al., 2000) and with silent cerebral infarcts and reversible posterior leukoencephalopathy syndrome in children with SCD (Henderson et al., 2003). However, in our dataset, ACS and the occurrence of CVEs were not associated (p=0.24). Further studies are required to elucidate these and other factors that potentially correlate with SCD clinical complications. Disclosures No relevant conflicts of interest to declare.

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Regulatory Genetic Variation at the S100B Gene Associates with Vaso-Occlusive Manifestations in Sickle Cell Disease

Blood ◽

10.1182/blood-2018-99-115395 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 1063-1063 ◽

Cited By ~ 1

Author(s):

Xu Zhang ◽

Wei Zhang ◽

Santosh L. Saraf ◽

Sergei Nekhai ◽

Mark T Gladwin ◽

...

Keyword(s):

Linkage Disequilibrium ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Research Funding ◽

Genetic Regulation ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Thrombotic Risk ◽

Increased Risk

Abstract In sickle cell disease (SCD) polymerization of hemoglobin S under deoxygenated conditions causes vaso-occlusion, which can manifest as acute pain crisis and progressive bone/organ damage. Molecular studies have attributed vaso-occlusion to elevated vascular adhesion and inflammatory responses, whereas the genetic regulation has only recently been assessed. Genomic DNA isolated from peripheral blood mononuclear cells (PBMCs) was hybridized to Illumina Human 610-Quad SNP array for the PUSH and Walk-PHaSST cohorts and to Affrymetrix SNP 6.0 array for the Howard SCD expression cohort. Single nucleotide polymorphisms (SNPs) for 381 PUSH, 525 Walk-PHaSST, and 55 Howard patients were imputed to 1000 genomes project phase 3 data. Messenger RNA from PBMCs was profiled using Affymetrix Human Exon 1.0 ST Array for the Howard expression cohort and Affymetrix Human gene 2.0 ST array for the UIC expression cohort. Patients within the PUSH and Walk-PHaSST cohorts were classified to four groups according to a cumulative pain score, calculated based on pain frequency and questionnaire description of pain intensity. Pain grouping was examined for correlation with other SCD complications using Cochran Armitage test. History of acute chest syndrome (ACS, PUSH P=3.8×10-9, Walk-PHaSST P=2.4×10-5) and avascular necrosis (AVN, PUSH P=4.1×10-4, Walk-PHaSST P=3.7×10-5) were the most significant clinical manifestations that consistently associated with pain in the two cohorts. To investigate the genetic control of vaso-occlusive manifestations with appropriate power, we leveraged genetic association of pain, ACS, and AVN with genetic regulation of disease-specific gene expression. We mapped expression quantitative trait loci (eQTL) in the Howard expression cohort for SNPs<1 Mb away from gene ends per expression trait. At a permutation based false discovery rate of 5%, 1004 independent eQTL (linkage disequilibrium r2 ≤0.3 per trait) were identified for 880 genes. Focusing on 129 genes whose expression was altered in PBMCs in sickle cell anemia by at least 1.5-fold [1], we identified six eQTL for five differential genes (up-regulated: OSBP2, SLC14A1, RNF182, CCRL2; down-regulated: S100B). The six eQTL were assessed for association with pain, ACS, and AVN, using the Walk-PHaSST cohort for discovery and the PUSH cohort for validation. At a significance of Bonferroni corrected P=0.05 (nominal P=0.0083), an eQTL of S100B (rs2154586) significantly associated with AVN in the Walk-PHaSST cohort (OR=1.8, P=0.00061) and the association was replicated in the PUSH cohort (OR=2.7, P=0.0052). The A allele of the eQTL (frequency=0.18) associated with increased risk for AVN and increased expression level of S100B in the Howard expression cohort (β=0.40, P=1.6 ×10-6). In an additional 64 sickle cell anemia patients without hydroxyurea treatment from the UIC expression cohort, expression levels of S100B were significantly elevated in the individuals with AVN (β=0.28, P=0.029). The 24 SNPs in linkage disequilibrium with the eQTL (r2 >0.7) constituted the third most significant peak in a meta-analysis of genome-wide association of AVN in the PUSH and Walk-PHaSST cohorts. To test the hypothesis that genes involved in vaso-occlusion in SCD may affect thrombotic risk in non SCD individuals, we examined the association of the locus with venous thromboembolism (VTE) in the ARIC, JHS and CHS cohorts from dbGaP. The locus was imputed in African Americans and VTE was defined as being told by a doctor to have a blood clot in the leg or lung as answered in questionnaires during medical exams. The SNPs were associated with VTE using logistic linear regression adjusting for age, gender, enrollment site, and the first 15 principal components per cohort. The risk allele of the leading SNP for AVN consistently associated with increased risk of VTE across the cohorts, with a combined P=0.0041 and OR=1.4. S100B encodes a calcium sensor that appears to intervene in a variety of biological functions. S100B can mediate the inflammatory effects of damage-associated molecular pattern molecules (DAMPs) produced by erythrocyte hemolysis [2, 3]. Serum concentration of S100B correlates with LDH and with TCD-determined peak velocity of the left middle cerebral artery in thalassemia patients[4]. Polymorphisms of S100B that lead to increased serum levels are associated with increased risk of ischemic stroke in the Chinese population[5]. Disclosures Nekhai: NIMHD, NIH: Research Funding; NHLBI, NIH: Research Funding; NIAID, NIH: Research Funding.

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Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease

Pediatric Blood & Cancer ◽

10.1002/pbc.21336 ◽

2008 ◽

Vol 50 (5) ◽

pp. 1006-1012 ◽

Cited By ~ 66

Author(s):

John J. Strouse ◽

Clifford M. Takemoto ◽

Jeffrey R. Keefer ◽

Gregory J. Kato ◽

James F. Casella

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Increased Risk

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Safety and Efficacy of Adjuvant Low-Dose Ketamine Therapy for Patients with Sickle Cell Disease

Blood ◽

10.1182/blood.v120.21.3226.3226 ◽

2012 ◽

Vol 120 (21) ◽

pp. 3226-3226 ◽

Cited By ~ 1

Author(s):

Caitlin M. Neri ◽

Sophie Pestieau ◽

Heather Young ◽

Angelo Elmi ◽

Deepika S. Darbari

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Pain Control ◽

Low Dose ◽

Red Blood Cell Transfusion ◽

Bivariate Analysis ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Ketamine Infusion ◽

Pain Scores

Abstract Abstract 3226 Background/Objectives: Opioids are the mainstay of therapy for painful vasoocclusive crises (VOCs) in sickle cell disease (SCD). For some patients, opioid-induced hyperalgesia caused by activation of N-methyl-D-aspartate (NMDA) receptors has been considered to contribute to poor analgesia. Ketamine, an NMDA receptor antagonist, could be a useful adjunct therapy; however safety concerns remain with its use. We examined records of SCD patients at our institution who received ketamine as an adjuvant to opioids at the discretion of anesthesia pain services over the past 4 years. We sought to explore the safety of ketamine and determine its effect on daily opioid requirement in SCD patients hospitalized for VOCs. Methods: The Institutional Review Board at Children's National Medical Center approved this study. A retrospective case-crossover study was conducted through review of the electronic medical record. For each patient we selected 2-paired hospitalizations occurring within 2 years of each other. One hospitalization where the patient received low-dose ketamine infusion in addition to opioid patient controlled analgesia (PCA), and a second hospitalization where the same patient received opioid PCA alone. We compared clinical characteristics of hospitalizations where patients did or did not receive ketamine. Exploratory bivariate analysis (paired t-test and McNemar's test) was used to compare variables between the pairs. Results: Thirty-three patients were identified to have at least 2 hospitalizations for VOC within 20 months of each other where they received adjuvant ketamine infusion during one, while not during the other. Average age was 15.6 ± 3.4 years, 67 % were females, and 36 % were on hydroxyurea therapy. SCD genotypes included homozygous SS 70%, SC 24 %, S-beta-zero thalassemia 3%, and S-beta-plus thalassemia 3%. Mean number of admissions in the 6 months prior to the ketamine hospitalization was 2 (range 0–5) and 64 % had ≥ 2 prior admissions within 6 months. Ketamine dose was 0.1 mg/kg/h for all patients except one who briefly received 0.15 mg/kg/hr. During the ketamine and opioid PCA hospitalization patients reported overall higher pain scores (6.53 vs. 5.94 out of 10; p = 0.0356), required higher doses of opioid (0.0395 mg/kg/hr vs. 0.0323 mg/kg/hr; p = 0.0038), and had a longer length of stay (LOS) (5.6 vs. 4.4 days; p =0.0148) as compared to the PCA alone hospitalization. Patients were more likely to have a diagnosis of acute chest syndrome (ACS) at some point during the ketamine and opioid PCA hospitalization (42% vs. 15 %; p = 0.0126) as compared to the opioid PCA alone hospitalization. Patients were more likely to be treated with additional adjuvant pain control agents (diazepam, lorazepam, gabapentin, pregabalin, amitripyline, or duloxetine) during the ketamine and opioid PCA admission as compared with opioid PCA alone admissions (45% vs. 9% p = 0.0013). Rates of red blood cell transfusion and ICU transfer were not different between the hospitalizations. In 3 patients ketamine was discontinued due to vivid dreams delusions, or dizziness. Nausea, pruritis, sedation, and use of complementary therapies were similar between hospitalizations. Conclusions: We did not observe an opioid sparing effect of ketamine infusion as hypothesized in this group of frequently hospitalized patients. Low-dose ketamine is a safe adjuvant medication for SCD patients hospitalized for VOCs. Higher opioid use during ketamine and opioid PCA admissions is likely due to patients experiencing more severe VOCs as indicated by higher pain scores involving multiple sites, higher rates of ACS, and longer LOS. These severity measures may have contributed to the decision of the pain medicine service to add low-dose ketamine infusion to standard opioid PCA in this retrospective sample. Finally, VOCs in this group of frequently admitted individuals may represent chronic pain which is known to be minimally responsive to most pharmacologic therapies. Patients receiving ketamine appear to be using additional adjuvant pain agents and may be underutilizing hydroxyurea. Prospective randomized studies of adjuvant ketamine therapy in patients with SCD are warranted to determine true efficacy. Disclosures: Off Label Use: Ketamine is a non-barbiturate phencyclidine derivative that is approved for use as a surgical anesthetic. It is not approved for use in pain management, however is commonly used in low-doses as an adjunct to traditional pain control therapies.

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Pulmonary Complications of Sickle Cell Anemia

Chest Imaging ◽

10.1093/med/9780199858064.003.0025 ◽

2019 ◽

pp. 141-145

Author(s):

Constantine Raptis

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Community Acquired Pneumonia ◽

Pulmonary Complications ◽

Acute Chest Syndrome ◽

Globin Chain ◽

Cell Disease ◽

Risk Of Mortality ◽

Increased Risk ◽

History Of

“Sickle cell disease” describes the spectrum of pathology in patients with at least one HbS chain and one other abnormal β‎ globin chain. Although patients with sickle cell disease often present with a simple community acquired pneumonia, acute chest syndrome must be considered in patients presenting with chest pain and fever, as it carries an increased risk of mortality, especially in adults. A few other entities, including rib infarction and subdiaphragmatic pathologies, can mimic the symptoms of acute chest syndrome. Finally, the findings of sickle cell disease on chest radiography will be discussed. Radiologists must be familiar with these findings in order to accurately interpret imaging studies, especially when the history of sickle cell is not provided.

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Smoking is associated with an increased risk of acute chest syndrome and pain among adults with sickle cell disease

Blood ◽

10.1182/blood-2010-01-265819 ◽

2010 ◽

Vol 115 (18) ◽

pp. 3852-3854 ◽

Cited By ~ 20

Author(s):

Robyn T. Cohen ◽

Michael R. DeBaun ◽

Morey A. Blinder ◽

Robert C. Strunk ◽

Joshua J. Field

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Increased Risk

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THE BURDEN OF SLEEP DISORDERED BREATHING IN CHILDREN WITH SICKLE CELL DISEASE

10.22541/au.162577109.95312179/v1 ◽

2021 ◽

Author(s):

Ilaria Liguoro ◽

Michele Arigliani ◽

Hui-leng Tan ◽

Atul Gupta

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Sleep Disordered Breathing ◽

Selection Process ◽

Specific Treatment ◽

Acute Chest Syndrome ◽

Clinical Implications ◽

Cell Disease ◽

Increased Risk ◽

Disordered Breathing

Children with sickle cell disease (SCD) have an increased risk of sleep disordered breathing (SDB) compared with the general pediatric population. There has been a growing research interest on this field in recent years, yet many questions regarding risk factors and clinical implications of SDB remain unclear. The aim of this review is to provide a concise narrative and systematic synthesis of the available evidence on the epidemiology, clinical presentation, complications and management, of SDB in children with SCD. An electronic search was conducted on studies published from the 1st of January 2000 to the 31st of December 2020 in PubMed/Medline, Scopus and Cochrane databases. All studies focusing on SDB in children with SCD aged from 0 to 20 years were included. Studies were eligible for inclusion if available in the English language. A quantitative synthesis of the included studies was performed. Only studies focusing on specific treatment outcomes were included in a meta-analytic process. A total of 190 papers were initially identified. After screening the title and abstract, 112 articles were evaluated for eligibility. At the end of the selection process, 62 studies were included in the analysis. Sleep-disordered breathing is associated with worse neurological, neurocognitive and cardiological outcomes, whereas the association with frequency or severity of vaso-occlusive pain events and acute chest syndrome was not clarified. Therapeutic interventions like adenotonsillectomy or oxygen supply may result in a significant increase in mean nocturnal oxygen saturation but effective clinical implications remain still unclear.

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Fetomaternal outcome in sickle cell disease in a tertiary care centre

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20210315 ◽

2021 ◽

Vol 10 (2) ◽

pp. 619

Author(s):

Rahi S. Modi ◽

Srushti S. Patel ◽

Dipti A. Modi ◽

Heena Talesara

Keyword(s):

Sickle Cell Disease ◽

Perinatal Mortality ◽

Sickle Cell ◽

Gestational Hypertension ◽

Tertiary Care ◽

Acute Chest Syndrome ◽

Medical Complications ◽

Cell Disease ◽

Tertiary Care Centre ◽

Increased Risk

Background: Sickle cell disease is a hereditary haematological disorder prevalent in tribal regions of India. Sickle cell disease can increase complications during pregnancy and in turn negatively influence pregnancy outcomes. This study reports the analysis of tribal maternal admissions in the tertiary centre S.S.G. Hospital, Baroda, Gujarat. Hence this study was conducted to assess complications in pregnancy and maternal and perinatal outcome among women with Sickle cell disease.Methods: It was a retrospective observational study including all pregnant women with sickle cell disease after 20 weeks of gestation who delivered at S.S.G. Hospital, Baroda from August 2019 to August 2020.Results: There were 43 antenatal women with Sickle cell disease during the study period. There was increased risk of obstetric complications like gestational hypertension (11.62%), preeclampsia (9.3%), eclampsia (6.97%), HELLP syndrome (4.65%), intrauterine growth retardation (23.25%), and oligohydramnios (11.62%). Medical complications observed were mainly anaemia (53.48%), vaso-occlusive crisis (18.16%), acute chest syndrome (4.65%) and infections like urinary tract infection (6.97%) and pneumonia (4.65%). The incidence of low birth weight babies (56.94%), low APGAR score (11.62%) and neonatal ICU admissions (23.25%) was high. 6.5% cases of maternal mortality and 4.65% cases of perinatal mortality was observed.Conclusions: Pregnancy in Sickle cell disease is associated with an increased maternal morbidity and high perinatal mortality due to obstetric and medical complications.

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