Sickle cell patient with an acute chest syndrome and a negative chest X‐ray: Potential role of the ventilation and perfusion (V/Q) lung scan

Feldman, Lawrence E.; Gross, Robert E.; Garon, Jack; Nallari, Anitha; Kaur, Navleen; Motwani, Bharat; Sukumaran, Sunitha; Allen, Sandra; Westerman, Maxwell P.

doi:10.1002/ajh.10411

Cited by 8 publications

(6 citation statements)

References 3 publications

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“…Those admitted to this study fulfilled two criteria at presentation or on previous episodes: (a) new infiltrate on chest radiograph (X-ray) and (b) new respiratory symptom or sign mainly chest pain, cough, wheezing, tachypnea and/or fever (>38.5°C). Although it is reported that rare cases of ACS presented with normal chest X-ray,[19] but still the selected criteria is keeping with the most widely accepted definition of ACS. [6] Historical data, clinical features, CBC (using automated counter), Hb-electrophoresis (using sickling test and alkaline ± acid gel-electrophoresis methods), G6PD activity (using fluorescent screening test), Cultures (whenever needed), radiological studies such as; plain chest X-ray; abdominal ultrasound (measurement of splenic long axis length and evaluation of hepatobiliary tree), room air arterial blood gases (were done on admission whenever possible), blood transfusion rate/year, hospitalization rate, severity status (average number of acute admission / year for crisis, ACS, priapism, stroke, etc.…”

Section: Methodsmentioning

confidence: 99%

Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes

Alabdulaali

2007

Ann Thorac Med

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BACKGROUND AND OBJECTIVES:Genetic studies suggest that the sickle cell mutation has arisen on at least four separate occasions in Africa and as a fifth independent mutation in the Eastern Province of Saudi Arabia or India. The pathophysiology of sickle cell disease (SCD) is essentially similar in these different areas although the frequency and severity of complications may vary between areas. The aim of this study was to evaluate the prevalence and outcome of acute chest syndrome (ACS) in SCD patients from Eastern province of Saudi Arabia in comparison with patients with African haplotypes.MATERIALS AND METHODS:This was a retrospective study involving 317 SCD patients who were two years or older, admitted to King Fahad Hospital Hofuf between January-May 2003 for different etiologies. Twenty six patients presented with different causes of ACS; 11 patients presented with different pathologies other than ACS, but had past history of ACS; 280 patients presented with different pathologies and never presented with ACS. Clinical features, CBC, Hb-electrophoresis, G6PD activity, cultures, chest X-ray, arterial oxygen saturation, blood transfusion rates and outcome were studied. Univariate and multiple regression analysis were carried out to evaluate influence on ACS. Comparison between SCD patients with ACS from this study and from Eastern province of Saudi Arabia to patients with African haplotypes were carried out, using data reported in the literature.RESULTS:During the period of this study, 37 patients with new or previous episodes of ACS were studied (accounting for 11.67% of admitted SCD patients). Most of the patients with ACS had only one episode, but five patients (13.51%) had had episodes or more. One patient died giving an in-hospital mortality rate of 1/26 (3.85%). Comparison of recurrence of ACS and mortality between SCD patients in Eastern province of Saudi Arabia to that of patients with African haplotype showed that recurrence is significantly lower (P<0.025) in patients from Eastern province compared to patients with African haplotype, mortality also is lower but not statistically significant.CONCLUSION:Acute chest syndrome in SCD patients in Eastern province of Saudi Arabia is relatively uncommon, but causes significant morbidity and mortality. Its prevalence and recurrence is low if compared to that of patients with African haplotypes.

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Section: Methodsmentioning

confidence: 99%

Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes

Alabdulaali

2007

Ann Thorac Med

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“…This usually reveals widespread perfusion defects with normal ventilation (Noto, ; Feldman et al , ; Kaur et al , ). However, the appearances may be confused with pulmonary emboli or diminished by the presence of other pathology, such as pleural effusions.…”

Section: Monitoring and Investigationsmentioning

confidence: 99%

Guideline on the management of acute chest syndrome in sickle cell disease

et al. 2015

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“…We believe that a combination of vasoocclusion and increased marrow blood flow is adequate to dislodge necrotic marrow material into the circulation. A ventilation and perfusion scan may be useful when this is initially suspected [87]. Once a vaso-occlusive crisis develops, the ensuing hypoxia and tissue hypoxemia cause sickling of erythrocytes.…”

Section: Review Of Literaturementioning

confidence: 99%

Bone marrow embolism in sickle cell disease: A review

et al. 2005

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The fat embolism syndrome is an important complication of patients with sickle cell hemoglobinopathies because of severe morbidity and mortality. Our recent experience with three cases that survived with intensive supportive care and prompt use of transfusion stimulates this review. A high index of suspicion, prompt use of diagnostic tools, and aggressive clinical management are the keys to a successful outcome. Am.

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Sickle cell patient with an acute chest syndrome and a negative chest X‐ray: Potential role of the ventilation and perfusion (V/Q) lung scan

Cited by 8 publications

References 3 publications

Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes

Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes

Guideline on the management of acute chest syndrome in sickle cell disease

Bone marrow embolism in sickle cell disease: A review

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