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 Table of Contents  
ORIGINAL ARTICLE
Year : 2012  |  Volume : 9  |  Issue : 1  |  Page : 14-17

Transabdominal ultrasonographic findings in children with sickle cell anemia in Sokoto, North-Western Nigeria


1 Department of Radiology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria
2 Department of Pediatrics, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Date of Web Publication10-Oct-2012

Correspondence Address:
Sadisu M Ma'aji
Department of Radiology, Usmanu Danfodiyo University Teaching Hospital, Sokoto
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0331-8540.102106

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  Abstract 

Background: Sickle cell anemia (SCA) is an inherited disease caused by production of abnormal hemoglobin chains within the red blood cell. In Nigeria, West Africa, the incidence of SCA is 3%. The most common abdominal manifestations include abdominal pain (mesenteric crisis), hepatomegaly, splenomegaly, autosplenomegaly, and cholelithiasis. Objective: To evaluate the abnormal transabdominal ultrasonographic (TAUS) findings in children with sickle cell anemia in Sokoto, Northwestern Nigeria. Materials and Methods: Pediatric patients with SCA attending the sickle cell clinic at the Department of Pediatrics, Usmanu Danfodiyo University Teaching Hospital Sokoto were scanned at the Radiology Department of the Hospital by transabdominal ultrasonography (TAUS) using Apogee 800 plus Ultrasound scanner with a variable frequency probes at 5-12 MHz. The patients were in their steady states. They were also screened for viral hepatitis B and C. Results: The commonest TAUS findings in this study was hepatomegaly in 70 (98.6%) patients followed by splenomegaly in 15 (21.1%), autosplenomegaly in 3 (4.2%), and gallbladder sludge in 2 (2.8%) patients. Conclusion: Transabdominal ultrasonographic imaging of patients with sickle cell anemia revealed a high prevalence of abdominal abnormalities, especially of the liver, spleen, and gallbladder.

Keywords: Children, sickle cell anemia, transabdominal ultrasonography


How to cite this article:
Ma'aji SM, Jiya NM, Saidu SA, Danfulani M, Yunusa GH, Sani UM, Jibril B, Musa A, Gele HI, Baba MS, Bello S. Transabdominal ultrasonographic findings in children with sickle cell anemia in Sokoto, North-Western Nigeria. Niger J Basic Clin Sci 2012;9:14-7

How to cite this URL:
Ma'aji SM, Jiya NM, Saidu SA, Danfulani M, Yunusa GH, Sani UM, Jibril B, Musa A, Gele HI, Baba MS, Bello S. Transabdominal ultrasonographic findings in children with sickle cell anemia in Sokoto, North-Western Nigeria. Niger J Basic Clin Sci [serial online] 2012 [cited 2019 Mar 21];9:14-7. Available from: http://www.njbcs.net/text.asp?2012/9/1/14/102106


  Introduction Top


Sickle cell anemia (SCA) is an inherited disease caused by production of abnormal hemoglobin chains within the red blood cell where there is replacement of glutamic acid with valine at the 6 th position of the β chain. This then results in rigid sickling of the cell, leading to vascular occlusion and ischemia in multiple organs. Sickle cell anemia is frequently encountered among populations in West Africa where the incidence of SCA has been estimated to be about 3%. [1] Repeated vaso-occlusion accounts for majority of the clinical manifestations of the disease. [1],[2],[3] The most common abdominal manifestations include abdominal pain (mesenteric crisis), hepatomegaly, splenomegaly, autosplenectomy, cholelithiasis, renal enlargement, and increased renal echogenicity. [3],[4] There is paucity of data on transabdominal ultrasonographic findings in children with sickle cell anemia in the study area. The objective of this study was to evaluate the prevalence of abnormal transabdominal ultrasonographic (TAUS) findings in children with sickle cell anemia in Sokoto, North-Western, Nigeria, because ultrasound is cheap, readily available, and reproducible and does not use ionizing radiation.


  Materials and Methods Top


A total of 71 patients with SCA attending sickle cell clinic at the Department of Pediatrics, Usmanu Danfodiyo University Teaching Hospital, Sokoto referred to the radiology department for abdominal sonography were recruited for the study. Sixty-three patients were homozygous for sickle cell disease (HbSS) while 8 patients had HbSS+F.

All the patients were scanned with Apogee 800 plus US scanner with a variable frequency probes at 5-12 MHz. After the patient fasted overnight, the examination was performed with the patient in the supine, right, or left side position to obtain an optimal view of the abdominal viscera. Measurement of the liver, spleen, and kidneys were performed in all the patients as follows: Long axis of the right lobe of the liver; long axis of the spleen at the level of the hilum; and long axis of both kidneys respectively. Informed consent was obtained from all patients or parents. Splenomegaly was defined as long axis of spleen longer than 88 mm and 84 mm in boys and girls, respectively. [5] Hepatomegaly was defined as long axis of the liver longer than 122 mm and 120 mm in boys and girls, respectively. [5] The upper limit for normal right and left kidneys was accepted as 70 mm and 68 mm approximately in both sexes. [6] In addition to the renal size, the renal parenchyma changes and any other abnormal abdominal findings were documented.


  Results Top


A total of 71 patients with sickle cell anemia were scanned. The age range was 1-15 years (mean age 6.1΁ SD 4.3). The commonest TAUS finding in this study was hepatomegaly in 70 (98.6%) patient, followed by splenomegaly in 15 (21.1%), gallbladder wall thickening in 7 (9.7%), and gallbladder stone seen in 4 (5.6%) patients. Autosplenectomy was demonstrated in 3 (4.2%) patients, gallbladder sludge was seen in 2 (2.8%) and increased renal medullary echogenicity was seen in 1 (1.4%) patient. The types of hemoglobin electrophoresis pattern from the patients under this study are shown in [Figure 1]. Sixty-three patients were homozygous for sickle cell disease (SS), and 8 patients had HbSS+F.
Figure 1: A histogram showing the Types of Haemoglobin Electrophoresis seen in the patients

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[Figure 2] is a pie chart showing sex distributions. [Figure 3] is a transabdominal sonographic image showing hepatomegaly, while [Figure 4] shows an echogenic right- sided ectopic kidney in a sickler. [Figure 5] is also an ultrasound scan demonstrating multiple gallbladder stones.
Figure 2: A pie chart showing sex distribution

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Figure 3: Longitudinal transabdominal ultra sound showing hepatomegly

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Figure 4: Transabdominal ultra sound showing right ectopic kidney with grade 1 nephropathy and left normal kidney

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Figure 5: Transabdominal ultra sound showing multiple gallblader stones

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  Discussion Top


Sickle cell anemia is an inherited autosomal recessive disorder characterized primarily by chronic anemia and periodic episodes of pain. This disorder produces abnormal hemoglobin, which causes the red cell to sickle or become crescent-shaped. These sickle-shaped red blood cells (RBCs) are much more rigid and, therefore, obstruct microcirculation and cause tissue infarction. Sickle-cell anemia (SCA) is particularly common among people whose ancestors come from sub-Saharan Africa, India, Saudi Arabia, and Mediterranean countries. Migration raised the frequency of the gene in the American continent. In some areas of sub-Saharan Africa, up to 2% of all children are born with the condition. [7] In Nigeria, sickle cell anemia is common and constitutes a major challenge in ultrasonographic examination. Sickle cell anemia manifests in the variety of abdominal problems, including hepatomegaly, splenomegaly, autosplenectomy, biliary tract abnormalities, renal enlargement, and increased renal and pancreatic echogenicity. [3],[4] Transabdominal ultrasonography (TAUS) has advantages over other imaging modalities in assessing the abdominal manifestations of sickle cell disease.

In one study, the TAUS appearances of the liver in SCA and thalassemia intermedia was described in 105 patients, with hepatomegaly demonstrated in 70.5% of the patients. [4] Hepatomegaly was also the most common pathologic finding in the current study with 70 (98.6%) prevalence. However, hepatomegaly is a common finding in SCA patients. Most of the other causes of hepatomegaly are due to infections, infiltrative and granulomatous disease, malignancy and other hematologic diseases. [8] Blood transfusion-related viral infections such as hepatitis B and C may also cause hepatomegaly. However, only 3 patients were positive for hepatitis B and C in this current study. The prevalence of cholelithiasis is usually secondary to chronic hemolysis seen in SCD. The prevalence of gallstones reported in homozygous SCD has varied widely from 34% to 70% in the United States, [9],[10],[11] 29% in Jamaica, [11] 4% to 25% in Africa, [12],[13] and 8% in Saudi Arabia. [14] The study among the pediatric age group in Ilorin showed a prevalence of 4.2%. [15] Our findings in this study are similar to that of Ilorin with 4.2% prevalence of cholelithiasis. Earlier studies have also pointed out the low incidence of cholelithiasis in Africans with SCD. [16],[17] The prevalence of biliary sludge in our study is 2.8%, which is lower than the value reported by Nzeh et al, who reported 7.5%. [17] Gallbladder wall thickening was demonstrated in 7 (9.7%) in our study with similar findings in the study carried out in Ilorin with 8.1%. [17] A study from Sudan showed a prevalence of gallbladder wall thickening of 2.2%. [20]

Splenomegaly was another common finding, seen in 15 patients (21.1%) in this study. In a similar study carried out in Turkey by Ali et al, the prevalence of splenomegaly was 17.9% and 16.7% in SS and S β thalassemia, respectively. [6] Autosplenectomy was demonstrated in 3 (4.2%) of our patients, which is similar to a study done by Bakhieta in Sudan who reported a prevalence of 4.8. [20] SCA is associated with many structural and functional abnormalities of the kidney, [21] which may progress to chronic renal failure and end-stage renal disease. [22] Several studies have reported medullary or diffuse increase reflectivity on renal ultrasound in patients with SCA. [3],[23],[24],[25] Medullary-increased renal echogenicity was seen in 1 (1.4%) of our patient. However, in a similar study carried out in Turkey, the reported prevalence was 10% in SS and 11% in S beta thalassemia groups. [6] Medullary hyperechogenicity have been reported in many conditions including papillary necrosis, hyperparathyroidism, medullary sponge kidney, and hypercalciuria.

Few reports have described pancreatic hemosiderosis in patients with SCA or thalassemia syndromes, [3] but no pancreatic abnormalities were seen in the current study.


  Conclusion Top


In conclusion, transabdominal ultrasonographic imaging of patients with sickle cell anemia revealed a high prevalence of abdominal abnormalities, especially in the liver, gallbladder, and spleen with a low prevalence in the kidney and pancreas. These abnormalities are related to hemolysis, anemia, and other hemoglobin abnormalities.

 
  References Top

1.Effiong CE. Sickle cell in childhood. In: Isaac SA, editors. Sickle cell disease. A handbook for the general clinician. Ibadan: Caxton; 1995.  Back to cited text no. 1
    
2.Lonergon GJ, Cline DB, Abbondanzo SL, Sickle cell anemia. Radiographic 2001;21:971-94.  Back to cited text no. 2
    
3.Fixler J, Styles L. Sickle cell disease. Pediatrics Clin N Am 2002;49:1193-210.  Back to cited text no. 3
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4.Papadaki MG, Kattamis AC, Papadaki IG, Menegas DG, Georgakopoulou TP, Mavrommati-Metaxotou A, et al. Abdominal ultrasonographic findings in patients with Sickle cell anemia and thalassemia intermedia. Pediatr Radiol 2003;33:515-21.  Back to cited text no. 4
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5.World Health Organization. 59 th World Health Assembly, Provisional Agenda Item 11.4; 24 th Apr. Geneva: WHO; 2006.  Back to cited text no. 5
    
6.Balci A, Karazincir S, Sangün O, Gali E, Daplan T, Cingiz C, et al. Prevalence of abdominal ultrasonographic abnormalities in patients with sickle cell disease. Diagn Interv Radiol 2008;14:133-7.  Back to cited text no. 6
    
7.Dick R, Watkinson A. The liver and spleen In: Sutton WD. Text book of Radiology and Imaging. 7 th ed. New York: Elsevier; 2002. p. 737-86.  Back to cited text no. 7
    
8.Stephans CG, Scott RB, Cholelithiasis In: SCA: Surgical and Medical Management. Arch Intern Med 1980;140:648-51.  Back to cited text no. 8
    
9.Barret CE. Cholelithiasis in SCA. Amer J Med 1968;45:889-98.  Back to cited text no. 9
    
10.Cameron TL, Maddery WC, Zuidema GB. Biliary tract disease and SCA: Surgical consideration. Am Surg 1971;74:702-10.  Back to cited text no. 10
    
11.MacCall IW, Desai P, Serjeant GR. Cholelithiasis in Jamaican patients with homozygous SCD. Amer J Heamatol 1977;3:15-21.  Back to cited text no. 11
    
12.Archampong EQ, Konotey-Ahulu FID. Biliary tract disease and SCA in Korle Bu Teaching Hospital, Accra. Ghana Med J 1975:14:175-80.  Back to cited text no. 12
    
13.Adekile AD. Experience with cholelithiasis in patients with SCA in Nigeria. Amer J Med 1973;54:327-32.  Back to cited text no. 13
    
14.Perrine RP. Cholelithiasis in SCA in a Caucasian population. Amer J Med 1973;54:327-32.  Back to cited text no. 14
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15.Nzeh DA, Adedoyin MA. Sonographic pattern of gallbladder disease in children with SCA. Paediatr Radiol 1989;19:290-2.  Back to cited text no. 15
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16.Adekile AD, Makanjuola D. Ultrasonography in children with sickle cell Anaemia. Nig J Paed 1983;10:35.  Back to cited text no. 16
    
17.Lagundoye SB. Radiological features of sickle cell Anaemia and related heamoglobinopathies in Nigeria. Afr J Med Sci 1970;1:315.  Back to cited text no. 17
    
18.Bakhieta IT. Sonographic findings in Sudanese children with sickle cell anaemia. J Diagn Med Sonography 2010;6:281-5.  Back to cited text no. 18
    
19.Ataga KI, Orringer EP. Renal abnormalities in sickle cell disease. Am. J Heamatology 2000;63:205-11.  Back to cited text no. 19
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20.Mapp E, Karasick S, Pollack H, Wechsler RJ, Karasick D. Uroradiological manifestations of haemoglobinopathies. Semin Roentgenol 1987;22:186-94.  Back to cited text no. 20
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21.Walker TM, Serjeant GR. Increased renal reflectivity in sickle cell disease: Prevalence and characteristic. Clin Radiol 1995;50:566-9.  Back to cited text no. 21
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22.Zinn D, Haller JO, Cohen HL. Focal and diffuse increased echogenicity in the renal parenchyma in patients with sickle cell heamoglobinopathies-an observation. J. Ultrasound Med 1993;12:211-4.  Back to cited text no. 22
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23.Namjoshi SP. Punctate echogenic foci in spleen and increase echogenicity in renal cortex in sickle cell Ameamia. J Clin Ultrasound 1999;27:52.  Back to cited text no. 23
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24.Shultz PK, Strife JL, Mc Daniel JD. Hyperechoic renal medullary pyramids in infants and children. Radiology 1991;181:163-7.  Back to cited text no. 24
    
25.Siegelman ES, Outwater E, Hanau CA, Ballas SK, Steiner RM, Rao VM, et al. Abdominal iron distribution in sickle cell disease: MR findings in transfusion and non transfusion dependent patients. J Comput Assist Tomogr 1994;18:63-7.  Back to cited text no. 25
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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