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ORIGINAL ARTICLE Table of Contents   
Year : 2003  |  Volume : 37  |  Issue : 3  |  Page : 14
Has the improvement in the economic status changed the pattern and severity of bone and joint complications in sickle cell disease - A comparative study of 2 decades


Department of Orthopaedic Surgery, College of Medicine, King Faisal University, Dammam; and King Fahd University Hospital, Al-Khobar, Saudi Arabia

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   Abstract 

We studied 110 patients of Sickle Cell Disease (SSD), born in seventies and eighties. They were matched for age and sex in both groups. The haematological and orthopaedic complications were compared. Group I patients belonged to the economic times of the country with Gross National Product (GNP) per capita $13,139.00, whereas the GNP per capita of Group II patients was $7086.00. The average age in Group I and II was 12.2 years (1-15 years) and 11.92 years (1-16 years). Mean haemoglobin concentration was marginally lower in Group I 9.42 to 9.8 G % in group II. The bone and joint infection was seen in 38 patients in group I and 36 in Group II. Avascular necrosis of the femoral head (ANFH) was diagnosed in 14 patients in group I and 22 in group II and similar was the affection of spine was 12 and 17 patients in group I and II respectively. There was no difference in the type of infection except in 15 patients of group I had multifocal as compared to 9 in group II. The majority of infecting organism was salmonellla species in both groups. Twenty-two patients in group I and 32 in group II were admitted due to painful crisis.
We conclude that the haematological parameters in both the groups and the severity of orthopaedic complications in different economic times were very similar, indicating that economic status did not influence the outcome in our patients of sickle cell disease with regard to pattern nor the severity.

Keywords: Sickle cell disease - Economic status- Severity - Bone and joint

How to cite this article:
Sadat-Ali M, Al-Habdan I. Has the improvement in the economic status changed the pattern and severity of bone and joint complications in sickle cell disease - A comparative study of 2 decades. Indian J Orthop 2003;37:14

How to cite this URL:
Sadat-Ali M, Al-Habdan I. Has the improvement in the economic status changed the pattern and severity of bone and joint complications in sickle cell disease - A comparative study of 2 decades. Indian J Orthop [serial online] 2003 [cited 2019 Dec 9];37:14. Available from: http://www.ijoonline.com/text.asp?2003/37/3/14/48551

   Introduction Top


Lehman, Maranjan and Mourant [1] reported for the first time the existence of sickle cell gene in the Eastern province of Saudi Arabia. Initially the disease was believed to be mild in comparison to other parts of the world, but experience of the last decades has given physicians a better understanding to suggest that SSD is as severe as seen elsewhere. [2],[3][4],[5],[6],[7],[8],[9],[10] It was suggested that patients with low socio- economic group who live in poor sanitary conditions have repeated infections which lead to severe disease. [10] Okany and Akinyanju [11] reported that socio-economic impact on the influence on the severity of the disease whereas Serjeant [12] believed among other factors, socio-economic factor to be responsible in expression of severity of the disease.

The objective of this retrospective study is to compare patients seen in eighties and nineties to assess of any difference in the pattern and severity of bone and joint complications in sickle cell disease.


   Patients and Methods Top


Fifty-five patients treated in 1980's who were born in seventies (group I) were matched for age, sex and residence with a similar number of patients born in 1980's and seen for the first time in 1990's (group II) with bone and joint complications in sickle cell disease. All patients were seen at King Fahd Hospital of the University, Al­Khobar of College of Medicine, King Faisal University, Dammam, Saudi Arabia. Laboratory data available for comparison was haemoglobin concentration, level of sickle cell haemoglobin, fetal haemoglobin and reticulocyte count. Clinical data included in both groups were painful crisis, osteomyelitis, septic arthritis and avascular necrosis of femoral and humeral head and spinal involvement. In bone and joint infection, the infective organism, site of infection and chronicity was also analyzed. For all statistical analysis BMDP Statistical Software Package was used. [13]


   Results Top


The laboratory data of all patients are given in [Table 1]. There were 32 males in group I and 36 in group II. Rest were females. The mean age in group I was 12.2 yrs. (1-15 yrs) where as in group II, the average age was 11.92 years (1-16). There was no significant difference in comparison of haemoglobin concentration, Sickle haemoglobin and Fetal haemoglobin and reticulocyte count.

There were 74 patients with bone and joint infection 51.3% (38) belonged to group I and 48.6% (36) were of group II [Table 2]. Site of infection is given in [Table 3], the most common site being the lower limbs in both groups. In the Group I 15 patients had multifocal site of infection whereas in group II only 9 patients had multifocal infection. In Group I  Salmonella More Details species was the infecting organism in 12 patients and in Group II 10 patients. Gross National Product (GNP) per capita income of group I patients was $13139.00 and that of group II patients was $7086.00. [14]


   Discussion Top


Sickle cell disease which is primarily a disease of hemopoietic system in which the skeleton bears the brunt of its complications. Over the decades report in the literature suggested the severity of the disease to be in different in different regions of the world. The causes of this difference was believed to be from genetic to local factors. Initially it was reported that the disease in Saudi Arabia was mild as compared to other parts of the world. [2],[15],[16],[17],[18] The mildness of the disease was believed to be due to high level of fetal haemoglobin. This was proved to be incorrect in later reports published from Saudi Arabia. [5],[6],[19],[20],[21],[22],[23] One another factor which was implicated was low economic status. Adeyokunnu and Hendrickse [10] reported poor sanitary conditions of Africa in the lower economic group which lead to severe and repeated salmonella osteomyelitis. Recently Okany and Akinyanju [11] found that the socio-economic status does influence the expression of severity of the disease. The economic conditions in the kingdom of Saudi Arabia was at the peak between 1973-1983 and the health care of patients has benefited due to this economic boom. Comparison of patients between the two decades of orthopaedic complications did not show any significant difference in the severity nor the pattern. Both groups showed very similar affection. We believe the differences of ANFH and spine in group II is due to early diagnosis of this complication due to availability of Magnetic Resonance Imaging in 90's. The chronicity and multifocal sites were comparatively less in number in group II patients because early diagnosis and aggressive treatment. [24]

It was believed that incidence and severity of VOC which causes chronic organ damage was an important factor in the morbidity, severity and mortality in sickle cell disease as a whole. Recent studies have refuted this belief. [25],[26] In our patients there was no significant differences between the number of episodes in both groups. Other factor such as of high persistence fetal haemoglobin have been shown not to be an important parameter in the expression of severity. [5],[6],[7],[8],[27]

There is no doubt that there is upward surge in the life expectancy of sickle cell patients not because of a change in severity of disease but due to better understanding and correct management of the complications. Our comparative study proves that improvement in the economic status of our patients did not make the disease mild, compelling us to believe that genetic factors play the role in the pattern and severity of sickle cell disease.

 
   References Top

1.Lehmann H, Maranjian G, Mourant AE. Distribution of sickle cell hemoglobin in Saudi Arabia. Nature 1963; 198:492-93.  Back to cited text no. 1    
2.Acquae JK, Omer A, Ganeshaguru K, Sejeny SA, Hoffbrand V. Non-benign sickle cell anemia in Western Saudi Arabia. Br J Haematol 1985; 60:99-108.  Back to cited text no. 2    
3.Al-Awamy BH, El-Mouzan M, Al-Torki MT, Serjeant GR: Neonatal screening for sickle cell disease in Eastern Province of Saudi Arabia. Trans R Soc Trop Med Hyg 1984; 78:792-94.  Back to cited text no. 3    
4.Al-Awamy BH. Sickle Cell Anemia: It's clinical manifes­ tations and their management. Saudi Med J 1987; 8:553-62.   Back to cited text no. 4    
5.Sadat-Ali M. Severe orthopaedic complications of the be­ nign sickle cell disease. Paper presented at the first scien­ tific symposium of Sickle Cell Anemia, Dammam, Saudi Arabia. 1990.  Back to cited text no. 5    
6.Sadat-Ali M, Al-Salem AH. Orthopaedic complications in sickle cell disease in Eastern Saudi Arabia. Ind Prac 1991; 54:214-20.  Back to cited text no. 6    
7.Sadat-Ali M, Geeranavar SS, As-Suhaimi S. Orthopaedic complications in sickle cell disease. A comparative study from two regions in Saudi Arabia. Int Orthop 1992; 16:307-10.  Back to cited text no. 7    
8.Abu-Srair HA, El-Bashier AM, Al-Dabous I, Al-Khater M. Incidence of major infection in sickle cell paediatric patients at Qatif Central Hospital. Ann Saudi Med 1991; 12:267-70.  Back to cited text no. 8    
9.Sadat-Ali M. The status of acute osteomyelitis in sickle cell disease. A 15-year review. Int Surg 1998; 83:84-87.  Back to cited text no. 9    
10.Adeyokunnu AA, Hendrickse RG. Salmonella osteomy­elitis in childhood. A report of 63 cases seen in Nigerian children of whom 57 had sickle cell anemia. Arch Dis Child 1980; 55:175-84.  Back to cited text no. 10    
11.Okany CC, Akinyanju OO. The influence of socio-eco­nomic status on the severity of sickle cell disease. Afr J Med Sci 1993; 22:57-60.  Back to cited text no. 11    
12.Serjeant GR. Natural history and determinants of clinical severity of sickle cell disease. Curr Opin hematol 1995; 2: 103-8.  Back to cited text no. 12  [PUBMED]  
13.Dixon WJ, Brown MB, Engelman L, Jennrich RI. BMDP statistical software manual. Berkeley; University of California Press.1990.  Back to cited text no. 13    
14.Gross national product per capita for Saudi Arabia between 1973-1992. Internet communication 1999 at http:// www.bized.ac.uk/dataserve/ Penn World Tables and http:/ /www.who.org/ The WHO statistical information system, Geneva 1999.  Back to cited text no. 14    
15.Gelpi RP. Sickle cell disease in Saudi Arabs. Acta Haematol 1970; 43:89-99.  Back to cited text no. 15    
16.Perrine RP, Brown MJ, Clegg JB, Weatherall DJ, May A. Benign sickle cell anaemia. Lancet 1972; 2:1163-67.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]
17.Perrine RP, Pembrey ME, John P, Perrine S, Shoup F. Natural history of sickle cell anaemia in Saudi Arabs. A study of 270 subjects. Ann Intern Med 1978; 88:1-6.  Back to cited text no. 17    
18.Serjeant GR, Serjeant BE. The natural history of sickle cell disease. Med Digest 1982; 8:5-14.  Back to cited text no. 18    
19.Al-Awamy BH. Sickle cell disease in early childhood in the eastern province of Saudi Arabia. Paper presented at the First Scientific Symposium on sickle cell anaemia, Dammam, Saudi Arabia. 1990.  Back to cited text no. 19    
20.Al-Salem AH, Ahmed HA, Qaisaruddin S, Al-Jam'a A, El-Bashier AM, Al-Dabbous I. Osteomyelitis and septic arthritis in sickle cell disease in eastern province of Saudi Arabia. Int Orthop 1992; 16:398-402.  Back to cited text no. 20    
21.Sadat-Ali M. Avascular necrosis of the femoral head in sickle cell disease. An integrated classification. Clin Orthop 1993; 290:200-205.  Back to cited text no. 21    
22.Sadat-Ali M. Avascular necrosis of humeral head. A com­plication of sickle cell disease. Ind J Orthop 1993; 27:149­-151.  Back to cited text no. 22    
23.Sadat-Ali M. Sickle cell disease- Orthopaedic complica­tions and their management. India; Premier publishing com­pany. 1994:55-74.  Back to cited text no. 23    
24.Sadat-Ali M, Al-Umran K, Al-Habdan I, Al-Mulhim F. Ultrasonography : Can it differentiate between vaso-oc­clusive crisis and acute osteomyelitis in sickle cell disease. J Paedtr Orthop, 1998, 18:552-54.  Back to cited text no. 24    
25.Schnog JJ, Lard LR, Rojer RA, Van der Dijs FP, Masket FA, Duits AJ. New concepts in assessing sickle cell dis­ease severity. Am J Hematol 1998; 58:61-66.  Back to cited text no. 25    
26.Steinberg MH. Management of sickle cell disease. New Eng J Med 1999; 340:1021-30.  Back to cited text no. 26  [PUBMED]  [FULLTEXT]
27.Al-Awamy BH. Observations on the clinical features of sickle cell disease in early childhood. Saudi Med J 1999; 20:277-82.  Back to cited text no. 27    

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Correspondence Address:
M Sadat-Ali
Department of Orthopaedic Surgery, College of Medicine, King Faisal University, Dammam; and King Fahd University Hospital, Al-Khobar
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


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    Abstract
    Introduction
    Patients and Methods
    Results
    Discussion
    References
    Article Tables
 

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