Adult Sickle Cell Disease: A Five-Year Experience of Intensive Care Management in a University Hospital in Oman

Tawfic, Qutaiba A.; Kausalya, Rajini; Al‐Sajee, Dhuha; Burad, Jyoti; Mohammed, Ahmed Kh.; Narayanan, Aravind

doi:10.12816/0003110

Cited by 21 publications

(27 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A total of 16.3% of our patients needed NIV/mechanical ventilation, which is indicative of increased disease severity in these patients. Importantly, another study from our institution showed that 69.6% of all SCA patients admitted to intensive care had ACS 7 . It was also reported that the need for inotropic support and mechanical ventilation was a predictor of mortality in these patients 7 .…”

Section: Discussionmentioning

confidence: 86%

“…Importantly, another study from our institution showed that 69.6% of all SCA patients admitted to intensive care had ACS 7 . It was also reported that the need for inotropic support and mechanical ventilation was a predictor of mortality in these patients 7 . Previously, mechanical ventilation was found to predict mortality and the increased utilization of hospital resources 18 .…”

Section: Discussionmentioning

confidence: 86%

“…SCA is the most prevalent hereditary haemoglobin disorder in Oman 5,6,12,13 . Furthermore, ACS is the leading cause of death among patients with SCA [7][8][9] . It is characterized by acute onset of chest pain, associated with tachypnea, fever, reduced oxygen saturation, dyspnea, cough, elevated WBC counts and not infrequently, with a decreased Hb level and new infiltrates on chest X-ray.…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Predictors of impending acute chest syndrome in patients with sickle cell anaemia

Alkindi

Al-Busaidi

Al-Salami

et al. 2020

Sci Rep

View full text Add to dashboard Cite

Acute chest syndrome (AcS) is a major complication of sickle cell anaemia (ScA) and a leading cause for hospital admissions and death. We aimed to study the spectrum of clinical and laboratory features of AcS and to assess the predisposing factors and predictors of severity. A retrospective case-control cohort was studied by retrieving patient information from electronic medical records after ethical approval. One hundred adolescents and adults with SCA and hospital admissions for ACS were identified through the discharge summaries, along with 20 additional patients presenting with VOC, but without ACS (controls). Among the patients with ACS, fever (>38.5 °C), reduced oxygen saturation (<95) and asplenia significantly differed when compared to those of controls (p < 0.05, chi-squared test). The degree of severity was reflected in the use of non-invasive ventilation (NIV), simple and exchange transfusions, and the presence of bilateral pleural effusions and multi-lobar atelectasis/consolidation, which were significantly higher in the cases with ACS than in the controls. Lower haemoglobin (Hb) and high WBC counts were also significantly different between the two groups (p < 0.05, Student's t test). Using logistic regression, our study further demonstrated that asplenia, fever, and reduced O 2 saturation, along with low Hb and leukocytosis, were important predictors for the development of ACS. Sickle cell anaemia (SCA) is an autosomal recessive disorder characterized by a point mutation in codon 6 of the beta globin chain, where glutamic acid is replaced by valine, resulting in the formation of HbS with varied clinical manifestations 1. The abnormal Hb is insoluble and polymerizes when exposed to low O 2 tension, leading to the major clinical manifestations of SCA, including recurrent vaso-occlusive crisis (VOC), predisposition to infections and reduced red cell survival with anaemia 2. Sickle cell disease (SCD) is prevalent in sub-Saharan Africa and the Indian sub-continent, but human population mobility has spread the disorder throughout Europe, Asia and the Americas 3,4. SCD is highly prevalent in Oman, with an estimated sickle cell gene frequency of 5.8% and a prevalence of sickle cell traits and SCA in Omani children of 4.8% and 0.3%, respectively 5,6. It is thus estimated that one neonate of every 323 newborns in Oman has SCA 6. Acute chest syndrome (ACS) is defined as the presence of fever and/or new respiratory symptoms accompanied by the presence of a new pulmonary infiltrate on chest X-ray and is one of the most important causes of death and the second most common cause of hospitalization in SCA patients 7. In a retrospective study, ACS was found to be responsible for 69.6% of SCA ICU admissions and was associated with a 16% mortality rate 7. Furthermore, ACS is associated with a high risk of sickle cell-related mortality and morbidity, including prolonged hospitalization 8. Although the aetiology of ACS is multifactorial, the Cooperative Study of Sickle Cell Disease (CSSCD) in the United States identified...

show abstract

Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Predictors of impending acute chest syndrome in patients with sickle cell anaemia

Alkindi

Al-Busaidi

Al-Salami

et al. 2020

Sci Rep

View full text Add to dashboard Cite

show abstract

“…The majority of patients who develop acute MOF often have co-morbid processes such as an infection or acute chest [27]. There are no systematic reviews or randomized controlled trials evaluating management and outcomes, but a retrospective chart review from 1993 and scattered case reports describe this devastating process [13,[27][28][29]. It typically presents with fever, anemia, thrombocytopenia and encephalopathy, all of which were present in the above case.…”

Section: Discussionmentioning

confidence: 98%

“…Acute multi-organ failure syndrome is a severe and often lifethreatening sequela of SCD, characterized by vaso-occlusive crisis (VOC) complicated by failure of two additional organ systems, including the lungs, liver or kidney [27][28][29]. The majority of patients who develop acute MOF often have co-morbid processes such as an infection or acute chest [27].…”

Section: Discussionmentioning

confidence: 99%

Untitled

2016

HTIJ

View full text Add to dashboard Cite

Patients with sickle cell disease are at increased risk of invasive infection despite gains made through penicillin prophylaxis, pneumococcal immunization and newborn screening. We present the case of a 42-year old male with hemoglobin SS disease, who developed acute multi-organ failure (MOF) secondary to Streptococcus pyogenes. He presented to the emergency department complaining of fever three times in five days, although afebrile at each presentation. On his third visit he became hemodynamically unstable and encephalopathic requiring intubation and admission to the ICU. His course was complicated by cardiomyopathy, acute renal insufficiency and bilateral trans metatarsal amputations. He was successfully discharged home on hospital day #37. We report on our experience with MOF from unrecognized infection in an adult with SCD and fever and review evidence-based and consensus treatment practices for management of sickle disease and fever in the emergency department. In conclusion, we strongly recommend that all febrile adults with sickle cell disease be treated as a medical emergency and receive empiric antibiotic therapy in addition to routine work-up and investigations.

show abstract

Phenotypes of sickle cell intensive care admissions: an unsupervised machine learning approach in a single-center retrospective cohort

et al. 2022

View full text Add to dashboard Cite

Adult Sickle Cell Disease: A Five-Year Experience of Intensive Care Management in a University Hospital in Oman

Cited by 21 publications

References 14 publications

Predictors of impending acute chest syndrome in patients with sickle cell anaemia

Predictors of impending acute chest syndrome in patients with sickle cell anaemia

Untitled

Phenotypes of sickle cell intensive care admissions: an unsupervised machine learning approach in a single-center retrospective cohort

Contact Info

Product

Resources

About