Indmedica Home | About Indmedica | Medical Jobs | Advertise On Indmedica
Search Indmedica Web
Indmedica - India's premier medical portal

Current Pediatric Research

Acute chest syndrome in children with sickle cell disease: Saudi Arabian experience

Author(s): Hassan A. Al-Trabolsi and Mohammed Alshehri

Vol. 9, No. 1 (2005-10 - 2005-12)

Curr Pediatr Res 2005; 9 (1 & 2): 23-26

Hassan A. Al-Trabolsi and Mohammed Alshehri

٭Department of Pediatrics (Division of Hematology/ Oncology), College of Medicine, King Khalid University, Abha, Saudi Arabia.
٭٭Department of Pediatrics (Division of Pulmonology), College of Medicine, King Khalid University, Abha, Saudi Arabia.

Key words: Sickle cell disease, children, acute chest syndrome

Accepted June 14, 2005

Abstract

Acute chest syndrome (ACS) is a known complication of sickle cell disease (SCD). It carries high morbidity and mortality. We conducted a retrospective study to evaluate the frequency, clinical, laboratory, and radiological features of this complication among children with sickle cell disease in the Southern Province of Saudi Arabia. Our results were generally comparable to international published data among similar population. Our results revealed that the frequency of ACS episodes are age dependent, which occurred more frequently in young children i.e. < 4 years of age (57%). Fever and cough were the most frequent symptoms; 93% and 86% respectively. Most of the cases experienced respiratory distress such as tachypnea (86%), chest retraction (64%), and decreased breath sounds (57%). On the other hand only small number of patients (11%) had complete normal chest examination. ACS, as other sickle cell crises, usually develops in association with other complication. In our study, painful crisis was the most commonly associated complication along with ACS (79%). All of the chest X-rays were positive at different anatomical sites; bilateral involvement was observed frequently (36%). This study is unique being the first description of such problem in children in the Southern Province of Saudi Arabia. It will serve as a base s for subsequent studies.

Introduction

Acute chest syndrome is an acute pulmonary illness in patients with sickle cell disease. It is defined as an acute episode associated with clinical and radiological evidence of new pulmonary abnormalities in patients with sickle cell disease (SCD) and often accompanied by fever, bone pain, pleuritic chest pain, cough, dyspnea, hypoxia, leukocytosis and decline in hemoglobin below the usual steady-state level [1-3]. It is a common problem, causing significant morbidity and mortality. Many factors may cause this syndrome. It is responsible for up to 25% of deaths, and for more than 90% of hospital admission [4-7].

The term “acute chest syndrome” was first suggested by Charache et al in 1979 [8], acknowledging the difficulties in determining its pathogenesis. Treatment is primarily supportive. Therapy includes hydration, analgesia, supplemental oxygen, antibiotics, blood transfusion and mechanical ventilation. Early detection and aggressive management may limit its severity and prevent its complications. It is estimated that ACS will occur in nearly one third of patients at risk [9-11]

Materials and Methods

This is a retrospective study performed at Kamiss Military Hospital and Aseer Central Hospital, Southern province, Saudi Arabia. Medical records of children with SCD who were diagnosed with acute chest syndrome between 1995-2001 were carefully reviewed. The following information were extracted: Sex, age, presenting symptoms, and signs, laboratory values, radiological findings as well as associated complications. Patients were divided into three groups on the basis of age (< 4 years, 4-8 years, and 9-12 years) with a view to assessing clinical presentation. The incidence of symptoms, signs, laboratory and radiological findings were studied. Treatment including Blood transfusion, exchange, antibiotics, course of the patients, intensive care admission, intubations and duration of hospital stay were noted.

Data was entered into IBM compatible computer at the Department of Family and Community Medicine, College of Medicine, King Khalid University. The Statistical Package for Social Science software (SPSSversion 10) was used for analysis of the data.

Results

Patients: Most of the episodes occurred in young children < 4 years of age (57%) and least common in older children > 9 years (11%). ACS noted to occur in both sexes with slight male predilection i.e. 54% compared to 46% in females. Most of the patients in the study had single episode of ACS (71%), while repeated episodes (second or more) have occurred in 28% (Table 1).

Table 1: Patient Characteristics

Age Group No. of Patient %
< 4 year 16 57
4 – 8 year 9 32
9 – 12 year 3 11
Male 15 54
Female 13 46
Single episode 20 71
Repeated episodes 8 28

Table 3: Presenting Signs

Signs Number %
Temperature 22 79
>39° C 8 21
< 39° C 24 86
Tachypnea 18 64
Retraction 26 93
Tachycardia 15 54
Dullness to percussion 16 57
Decreased breath sound 20 71
Wheezing 7 29
Bronchial breathing 9 32
Normal exam 3 11

Table 4: Baseline and ACS laboratory values

Test Mean CI
Hb (g/dl) 7.6 6.41-8.90
WBC (X 109/L) 18.9 16.70-20.81
Platelet (X 109/L) 345.7 321.91-393.50
Reticulocytes (%) 9.8 7.40-10.60
Serum bilirubin 3.8 3.10-4.21

Table 5: Associated problems with ACS

Problems Number %
Painful Crisis 22 79
Infection
Bacteria 15 50
URTS 10 33
UTI 2 7
Acute Cholecystitis 2 7
Postoperative 4 14
Sequestration crisis 2 7
Bronchial asthma 4 14

Table 6: Radiological Manifestations

Chest radiograph Number %
Upper zone involvement 2 7
Middle zone involvement 8 27
Lower zone involvement 4 14
More than one zone in one side 6 20
Bilateral involvement 10 33
Pleural effusion 7 31

Presenting symptoms: The most common presenting symptoms were fever, cough, and chest pain. Some patients experienced shortness of breath, wheezing, chills and productive cough. Fever was the most common presentation, (93%), cough and chest pain were found in 86% and 71% respectively while dyspnea was recognized in 61%. The frequency of presenting symptoms was agedependent with fever and cough being more common in young children (age 2-4 years) and the incidence of chest pain, shortness of breath, productive cough occurred in less than quarter of patients. (Table 2).

Table 7: Presenting symptoms

  Number %
Fever 26 93
Cough 24 86
Chest pain 20 71
Productive cough 16 57
Shortness of breath 17 61

Physical findings: Vital signs at the time of hospitalization were age dependent with young children experiencing higher temperature, pulse rate, and respiratory rate than older children. The most frequent physical exam findings were an abnormal chest examination in the form of signs of respiratory distress, decreased air entry, rales and dullness to percussion, whereas normal chest examination was observed in only (11%) of the cases (Table 3). Painful vasoocclusive crisis was associated with (79%) of patients. It has been considered the most common associated event with ACS followed by an underlying infectious process (55%).

Laboratory findings: Blood count documented during acute chest syndrome was compared with steady state value. Hemoglobin and white blood counts showed significant changes with the severity of the disease. (Table 4)

Radiographic findings

Radiographic findings vary by age. The predominant radiological findings were bilateral lungs involvement (36%). Young children had isolated upper and middle lobe disease significantly more often and lower disease less often than older children (Table 6).

Discussion

Episodes of acute chest pain in patients with SCD associated with a new infiltrate on chest film are called “acute chest syndrome” (ACS). These episodes are second only to acute painful episodes in term of incidence and need for hospitalization. These episodes are more common in children than adults and more common in patients with low levels of fetal hemoglobin and high level of total hemoglobin. [12].

Acute chest syndrome consists of combination of signs and symptoms. It is a form of lung injury that can lead to adult respiratory distress syndrome (ARDS). Pulmonary disease is the leading cause of death in sickle cell disease [13]. There are both acute and chronic pulmonary manifestations of sickle cell disease. The acute syndrome consists of dyspnea, chest pain, fever, tachypnea, pulmonary infiltrate on radiography and leukocytosis. It affects approximately 30% of patients with sickle cell disease and may be lifethreatening. [14]. There is scanty data about the acute chest syndrome among Saudi children with SCD. Al-Dabbous et al reported the frequency of ACS in Qatif (a medium size city in the Eastern Province of the Saudi Arabia) in the order of 5- 7.7% [11, 15]. It affects both boys and girls, with predominance of males [11,16]. The process is usually due to infection or vasoocclusion but may also be the result of non cardiogenic pulmonary embolization from a distant thrombus or infracted bone marrow. [14,17]. Approximately 50% of patients experience at least one episode of ACS and they ultimately have a higher chance of dying at an early age, and the mortality rate after such an event can be as high as 10-12% [18-20]. It is important to recognize and treat these events aggressively since it is the leading cause of death among sickle cell patients [21].

This retrospective study of 28 patients with sickle cell disease who developed ACS is a representative sample of such patients. Our data revealed that the incidence of ACS was strongly influenced by patient age, being most common in younger children and least frequent in older children. The association of ACS with young children could also be explained by reasoning that the increased susceptibility to viral respiratory infection in young children could precipitate ACS in children who are also more likely to have significant abnormal respiratory finding on chest exam at presentation [22].

Other factors associated with a high ACS incidence were a higher steadystate leukocyte counts. The reason for the association between high leukocyte counts and ACS incidence is not clear. It could be explained by the increasing susceptibility to viral respiratory infection in young children as a precipitating factor for ACS [23].

The predominant radiological findings in our study revealed diffuse lung involvement, unlike lower lobes being involved in other studies [24,25]. This difference in the site of the lung involvement could be attributed the fact that most of the cases in this study are very young children and the possibility of an associated infectious process rendering both lung to be affected.

This is the first study in Aseer Central Hospital to record the experience of ACS in children with SCD, and to the best of our knowledge, it has not been reported from the Southern Province of the Kingdom of Saudi Arabia. This retrospective study demonstrates the clinical presentation of ACS in children with SCD in this part of Saudi Arabia. It is of great value as baseline study. Nevertheless, further studies of such condition are required to clearly understand this important complication of SCD.

References

  1. Haynes J, Manci E, Voelkel N. Pulmonary Complications. In: Embury EH, Habbel RP, Mohandas N, Steinberg MH, editors. Sickle Cell Disease - basic principles and clinical practice. 1st ed. New York (NY): Raven Press; 1994. p. 623-631.
  2. Al-Jam’a AH, Al-Dabbous IA. Management of ACS. In: Al-Jam’a AH, Al-Dabbous IA, editors. Management Manual of Sickle Cell Disease. 1st ed. Dammam (KSA): Al-Shati Modern Press; 1992. p. 52-58.
  3. Poncz M, Kane E, Gill FM. Acute chest syndrome in sickle cell disease: Etiology and clinical correlates. J Pediatr 1985; 107: 861-866.
  4. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug PP. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994; 330: 1639-1644.
  5. Vichinsky EP. Comprehensive care in sickle cell disease: its impact on morbidity and mortality. Semin Hematol. 1991; 28: 220-226.
  6. Gray A, Anionwu EN, Davies SC, Brozovic M.. Patterns of mortality in sickle cell disease in the United Kingdom. J Clin Pathol. 1991; 44: 459-463.
  7. Thomas AN, Pattison C, Serjeant GR. Causes of death in sickle-cell disease in Jamaica. Br Med J (Clin Res Ed). 1982 28; 285: 633-635.
  8. Charache S, Scott JC, Charache P. “Acute chest syndrome” in adults with sickle cell anemia. Microbiology, treatment, and prevention. Arch Intern Med. 1979; 139: 67-69.
  9. Weil JV, Castro O, Malik AB, Rodgers G, Bonds DR, Jacobs TP. Pathogenesis of lung disease in sickle hemoglobinopathies. Am Rev Respir Dis 1993; 148: 249-256.
  10. Poncz M, Greenberg J, Gill FM, Cohen A. Hematologic changes during ACS in sickle cell disease. Am J Pediatr Hematol Oncol 1985; 7: 96-99.
  11. Al-Dabbous IA. Acute chest sydnrome in sickle cell disease children in Saudi Arab children in Eastern Province. Annals of Saudi Medicine. In press 2002.
  12. Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, Vera JC, Levy PS. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994 15; 84: 643-649.
  13. Vichinsky EP. Comprehensive care in sickle cell disease: its impact on morbidity and mortality. Semin Hematol. 1991; 28: 220-226.
  14. Charache S, Scott JC, Charache P. “Acute chest syndrome” in adults with sickle cell anemia. Microbiology, treatment, and prevention. Arch Intern Med. 1979; 139: 67-69.
  15. Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, Vera JC, Levy PS . The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994 15; 84: 643-649.
  16. Srair HA, Owa JA, Aman HA, Madan MA. Acute chest syndrome in children with sickle cell disease. Indian J Pediatr 1995; 62: 195-197.
  17. Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, Nickerson B, Orringer E, McKie V, Bellevue R, Daeschner C, Manci EA. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000: 14; 343: 824.
  18. Al-Dabbous IA. Acute chest syndrome. Saudi Med J. 2002; 23:1037-1044.
  19. Vichinsky E, Willimas R, Das M, Earles AN, Lewis N, Adler A et al. Pulmonary fat embolism: A distinct cause of severe ACS in sickle cell anemia. Blood 1994; 83: 3107-3112.
  20. Poncz M, Greenberg J, Gill FM, Cohen A. Hematologic changes during ACS in sickle cell disease. Am J Pediatr Hematol Oncol 1985; 7: 96-99.
  21. Thomas AN, Pattison C, Serjeant GR. Causes of death in sickle cell disease in Jamaica. Br Med J (Clin Res Ed). 1982 28; 285: 633-635.
  22. Sprinkle RH, Cole T, Smith S, Buchanan GR. Acute chest syndrome in children with sickle cell disease. A retrospective analysis of 100 hospitalized cases. Am J Pediatr Hematol Oncol. 1986; 8: 105-1010.
  23. Al-Dabbous IA, Abu-Srair HA, Al-Faris SS. Pattern of admissions of children with sickle cell disease in Qatif Central Hospital, Saudi Arabia. Bahrain Medical Bulletin 1994; 16: 3-6.
  24. Al-Hawsawi ZM Acute chest syndrome in sickle cell disease. Saudi Med J. 2004; 25: 116-117.
  25. Koren A, Wald I, Halevi R, Ben Ami M. Acute chest syndrome in children with sickle cell anemia. Pediatr Hematol Oncol 1990; 7: 99-107

Correspondence:
Dr. Hassan A. Al-Trabolsi
Department of Pediatrics
College of Medicine
P. O. Box 641
Abha, Saudi Arabia
e-mail: haltrabolsi(at )hotmail.com
Phone: +966 7 224-7800, Fax: +966 7224-7570
Al-Trabolsi/ Alshehri

Access free medical resources from Wiley-Blackwell now!

About Indmedica - Conditions of Usage - Advertise On Indmedica - Contact Us

Copyright © 2005 Indmedica