Clinical presentation of acute chest syndrome in sickle cell disease

Abstract

In this study the records of 45 patients with sickle cell disease involved in 63 presentations of acute chest syndrome at the Princess Margaret Hospital in Nassau, the Bahamas, between 1997 and 2001 were examined. Patients were divided into three groups on the basis of age (<13 years, 13–18 years, ⩾19 years) with a view to assessing clinical presentation. The incidence of symptoms, physical signs, and laboratory findings were enumerated and significant differences between age groups determined. The data were analysed using analysis of variance, t test, and χ2 test and compared with existing knowledge on the subject. This study proposed to evaluate the clinical presentation of acute chest syndrome with emphasis on historical and physical findings, and to encourage the physician to maintain a high index of suspicion for the condition in susceptible patients. It was found that presentation varied significantly with age groups, children presenting most classically with fever and cough and adults, with chest pain. The 13–18 age group emerged as the group which presented most frequently with the typical symptoms of chest infection, thus potentially making diagnosis easier. Of note, the most frequent finding was a normal examination, while the second commonest physical finding was crepitations on auscultation of the chest.

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

“…I studied the summation of results in this study together with those done in Qatif and Hofuf previously[2123] versus the results reported by Taylor, et al and Bernard Maitre, et al [2425] Tables 2 and 3. I found 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 [Figure 2].…”

Painful Vaso-occlusive Crisis as a Prodromal Phase of Acute Chest Syndrome. Is Only One Chest X-ray Enough? A Case Report

“…The diagnosis of ACS in sickle cell disease is an important challenge to the physician and especially to the pediatrician. Similar with our case, it may present insidiously and non-specifically, often complicating other conditions (Taylor et al, 2004).…”

“…Bernard et al (2007) suggested that up to 50% of patients diagnosed with ACS, are initially admitted to the hospital for other reasons and subsequently develop the disease. The most common admission's reason in these cases, similar to our case, is a vaso-occlusive painful crisis (Taylor et al, 2004;Bernard et al, 2007). Therefore, the patients admitted to the hospital for painful crisis, should be considered to be in the prodromal phase of ACS and should be monitored closely in case they develop this syndrome.…”

New pulmonary infiltrates in a 19 year-old with sickle cell crisis

“…The three most common symptoms at the time of diagnosis were fever in 80%, cough in 62% and chest pain in 44%. The three most common findings on physical examination were: respiratory rate >30/min in 67% (mean peak respiratory rate of 38/min); a mean peak temperature of 38.9 • C; and rales on examination in 76% [5,6]. Laboratory findings at the time of diagnosis included a mean initial hemoglobin of 7.7 g/dl, a mean decrease in hemoglobin from steady state value of about 0.78 g/dl, a mean white cell count of 23,300 per cu mm, relative thrombocytopenia (200,000/cu mm) and a mean partial pressure of arterial oxygen at diagnosis of 70 mmHg.…”