Ultrasonic assessement of the prevalence of gall stones in sickle cell disease children seen at the University of Benin Teaching Hospital Benin City

Niger J Paed 2014; 41 (4): 370 - 374

ORIGINAL

Odunvbun ME

Ultrasonic assessment of the

Adeyekun AA

prevalence of gall stones in sickle cell

disease children seen at the University

of Benin Teaching Hospital, Benin

City, Nigeria

DOI:http://dx.doi.org/10.4314/njp.v41i4,16

Accepted: 17th July 2014

Abstract Background: Gallstone

patients

with

gallstones

were

is a common problem in patients

HbSS, none with HbSC had stone

Odunvbun ME (

)

with sickle cell disease. Preva-

although only three were studied.

Department of Child Health

lence of this problem among

Only one child was symptomatic

sickle cell disease (SCD) children

for the presence of gallstones. One

Adeyekun AA

Department of Radiology

may vary with age, and geo-

patient had biliary sludge. The

University of Benin Teaching Hospital

graphic location. Studies on gall-

male: female ratio was 4:1. Preva-

Benin-City, Edo State, Nigeria

stone prevalence in SCD children

lence increased with age; 2.9%

Email: magodunvbun@yahoo.com

are scanty in the South-South

4.5% and 9.1% in patients aged

zone of Nigeria.

less than 5,6-10 and 11-18 years

Aim: To determine by ultrasono-

respectively. Body weight was

graphy the prevalence of gall-

significantly associated with the

stones among steady state sickle

presence of gallstones.

cell disease children attending the

Conclusion: Gallstone was found

Paediatric Outpatient Clinic of

in 5% of the SCD children aged 1-

the University of Benin Teaching

18years. This value is low com-

Hospital, Benin-City, south south

pared to those from Europe and

Nigeria.

North America. It however con-

Methodology: The study was a

firmed the relative low prevalence

descriptive cross sectional study.

in the general population of Nige-

The prevalence of gall stones was

rians. The clinical significance of

studied

prospectively on

101

gallstones in these SCD children is

sickle cell disease patients who

that it may contribute significantly

attended the Paediatric Outpatient

to the morbidity and mortality seen

clinic of the University of Benin

in these children.

Teaching Hospital by means of

Recommendation:

Routine

ab-

ultrasonography. They were aged

dominal scan is recommended for

1-18 years. All except three pa-

sickle cell disease children to de-

tients were Haemoglobin (Hb) SS

tect gallstone whose presence can

while the remaining three were

mimic abdominal crisis. The op-

HbSC. Statistical analysis was

tion of elective cholecystectomy

done by means, percentages and

with reduced morbidity compared

tests of significance was done

to emergency cholecystectomy can

using students t- test.

thus be exploited.

Results: Five of the 101 children

studied had gallstones and one

Key words: Sickle cell disease,

had biliary sludge. The preva-

gallstones, biliary, sludge, Benin-

lence of gallstone was 5%. All

City, ultrasound.

Introduction

SC. The disease is a chronic haemolytic disorder associ-

ated with increased bilirubin production and jaundice .

Sickle cell disease (SCD) is the commonest haemoglobi-

Hyper-haemolytic crisis usually following sepsis con-

nopathy seen in Nigeria with a prevalence of 3% in the

tributes greatly to increase bilirubin production. Third

newborn population . The commonest phenotypes are

generation cephalosporins are known to crystallize in the

the

homozygous SS or Sickle cell anemia, and less

gallbladder

2-3

and their use in treating infections which

common are the compound heterozygote Haemoglobin

is a common problem in SCD is thought to contribute to

371

the high prevalence of stones seen in these patients .

Statistical analysis was done using means and percent-

Vaso-occlusive events, acute sequestration crisis and

ages. Students’t-test was used to test for significance. At

recurrent febrile episodes from bacterial infections con-

95% confidence interval, p values less than or equal to

stitute the common acute events associated with the dis-

0.5 were considered significant.

ease. However, chronic events also occur and formation

of gallstone as a result of the chronic haemolysis is one

of such complication. Gallstone in SCD patients are

usually pigment stones composed largely of calcium

Results

bilirubinate. It is thought that gallstones create a nidus of

infection, predisposing affected patients to higher mor-

One hundred and one sickle cell disease (SCD) children

bidity and mortality . In addition, infection is thought to

aged 1-18years (mean age was 8.2 ±4.5 yrs) were stud-

play a major role in the genesis of pigment stones in

ied; 62 were males, while 39 were females. Ninety eight

SCD children. Studies have shown that 5-10% of bile

were haemoglobin (Hb) SS, while 3 were SC. Thirty

obtained from patients at cholecystectomy culture

five were aged 1-5 years, 44 were 6-10years and 22

bacteria.

were 11-18years (Table 1).

The prevalence of this disorder among the populations

Table1: Prevalence of gallstones by age in Sickle cell disease

of SCD children vary widely, from 34% to 70 % in the

patients

USA , 29% in Jamaica and from 4 to 25% in

6-7

Age (yrs)

No (%)

No with gallstone (%)

Africa

9,10

Symptomatic biliary tract disease is difficult to

diagnose in SCD because symptoms of gallstones mimic

1-5

35(100)

1(2.9%)

those of vaso-occlusive crisis involving the abdomen.

6-10

44(100)

2(4.5%)

In addition, the morbidity and mortality of emergency

11-18

22(100)

2 (9.1%)

Total

101

5 (5%)

cholecystectomy in SCD is much higher than in elective

cholecystectomy, hence the need for early detection .

Early detection allows for elective cholecytectomy

which is associated with lower morbidity compared to

Gallstones were seen in five HbSS children, aged 5, 8,

emergency cholecytectomy.

10, and in two 18 year olds respectively. This consists

The prevalence of this condition among children with

of four males and one female with a male/ female ratio

sickle cell disease children in Benin City by means of

of 4:1. The overall prevalence of gall stones in this study

ultrasonography is not known hence the need for this

is thus 5%. The prevalence of gallstones increased with

study. This study therefore aims to determine the preva-

increase in the patients age (Table 1); 2.9%, 4.5% and

lence of gallstones among steady state sickle cell disease

9.1% respectively in patients aged 1-5, 6-10, 11-18

children attending Outpatient clinic at the University of

years respectively. All the patients with gallstone were

Benin Teaching Hospital, Benin-City, Nigeria.

HbSS. One child, a 14 year old female had biliary

sludge. All except one were asymptomatic for the pres-

ence of the gallstone. He had right upper quadrant ab-

dominal pain. This child was a five year old with sebor-

Methods

rhoiec dermatitis in addition to the sickle cell disease.

This study was conducted at the University of Benin

The weight of the children studied ranged between 10-

Teaching Hospital, Edo State Nigeria, a tertiary health

56kg (mean 25.29 ±10.5kg). Their height ranged 77-

facility in the South-South Nigeria. The study was a

172cm with a mean of 123.27 ±22.78. The body mass

descriptive cross sectional study. It was conducted be-

index (BMI) ranged from10.6-22.5 with a mean of 15.71

tween April and September 2010. Sickle cell disease

± 1.87, while the body surface area (BSA) ranged from

children attending the out- patient clinic and in steady

0.47-1.67 with a mean of 0.92. The mean haemoglobin

state were recruited consecutively. Informed consent

concentration (Hb) was 7.4 ± 1.1g/dl, range was 5.4-

was obtained from the parents or care givers and the aim

10.0g/dl. The mean packed cell volume (PCV) was 22.7

of the study was explained. Ethical committee approval

±3.4% (range 16-32%). There was a statistically signifi-

was obtained. Exclusion criteria included presence of

cant difference in the mean weight of patients that had

fever, pain crisis, and recent hospitalizations. Each pa-

stones compared to patients that did not have stones;

tient was scanned just before breakfast. Scanning was

p=0.02. However, there was no significant difference in

done trans-abdominally using a Fukuda Sonic machine

the mean height, BMI, BSA, Hb and PCV values

(Fukuda Denshi; Japan) with a 3.5 MHz convex probe,

amongst the patents that had stones and those without

with the subjects in supine position. Each measurement

have stones (Table 2).

was taken three times and the average taken to minimize

Of the five patients with gallstone, one had undergone

inter- observer’s error. Gallstones was identified as dis-

autosplenectomy, the spleen was present in the rest.

crete hyper-echoic foci with acoustic shadowing while

sludge appeared as echoic material that layers or settles

along the posterior gallbladder wall without shadowing .

The antero-posterior, transverse and longitudinal diame-

ters of the gallbladder were measured.

372

Table 2: Comparison of anthropometric and hematologic

stone was 26% (57/217) amongst

parameters of patients with and without gallstones and sludge

subjects aged 5-19year . Again, the prevalence of gall-

Parameters

Patients with

Patients without

student

p-value

(mean)

gallstone/sludge

t-test

stones in the above studies was also much higher than

that of this study although the Turkey study had much

Weight(kg)

34.6

24.7

2.221

0.02

older patients. In the United Kingdom (UK), in a study

Height(cm)

138.5

122.2

1.827

0.07

of 131 patients with SCD aged 10-65 years, the preva-

BMI(kg/m2

16.4

15.6

1.021

0.31

lence of gallstones among the 95 SS subjects was 58% .

BSA(m2)

1.1

0.9

1.638

0.1

Hb(g/dl)

7.03

7.4

0.795

0.4

Again, the patients are older than the subjects in this

PCV(%)

22.8

22.7

0.07

0.9

study. In addition, UK is an European country where

refined foods with high cholesterol and low fibre is con-

sumed. Prevalence rates in the USA was 29% in SCD

aged 2-18 years, 28 of the 31 subject studied had ultra-

sound scan. It is thought that the difference in dietary

Discussion

cholesterol and or fibre consumption account for the

Chronic haemolysis associated with sickle cell disease

difference in the prevalence of gallstones in Africa and

Europe and USA .

facilitates the formation of gallstones and ultrasonogra-

phy is a reliable, safe, reproducible, and non invasive

method for investigating the biliary tract. It provides

The prevalence of gallstones is usually increased with

88% sensitivity and 80% specificity for the diagnosis of

increase age and by the age of 18 years 30% of SCD are

expected to have developed gallstones

30,31

gallstones . The incidence of gallstones in SCD chil-

. In this study,

dren also appears to vary worldwide. In this study, the

the prevalence of gallstone amongst the children aged

overall prevalence of gallstones was 5%. This is similar

11-18 years was 9.1%.

to the 6% reported by Akinyanju and Ladapo in SCD

The youngest child with of gallstones in this study was

children younger than 15 years in western Nigeria , the

five years. This is similar to that in the Ghanaian study,

4.4% , 5% ,and 4.2%

respectively in reported by

however gallstones has been reported in a 2.5 year old

SCD child , and in an SCD toddler who was sympto-

three other Nigerian studies and the 4% documented in

matic .

Ghana.

The patients studied in the Ghana were aged

2-13 years and hence were younger than the 1-18 years

Only one patient, a 5 year old, in this study was sympto-

old in this study. In the study conducted by Akinyanju,

matic, the complaint was that of pain in the right upper

oral cholecystography was used and but this method is

quadrant of the abdomen, this child additionally had

now abosolete. Studies by Adekile et al

and Akama-

seborrhoeic dermatitis. In Ghana, all the patients were

asymptomatic , while in other studies, some of the sub-

guna et al

used both cholecystography and ultrasono-

graphy while Nzeh et al used only ultrasonograghy. In

jects had already done cholecystectomy or were sympto-

matic

25-27

Senegal, the prevalence of gallstones amongst SCD chil-

dren aged 11months to 22 years was 9.4%. This is

higher than reports from Ghana and Nigeria. This is

One child who had undergone autosplenectomy had

probably because the age cohort in the Senegalese study

gallbladder stone, unlike other studies where those with

autosplenectomy did not have gallstones .

was higher.

In another study conducted in Sudan

amongst 90 homozygous SCD children aged six months

Biliary sludge is a complex mixture of mucous, calcium

to 16 years, the prevalence of gallstones was 11.1%.

bilirubinate, and cholesterol in which hypersecretion of

This is higher than that which was documented in Ghana

mucous leads to precipitation of bilirubin, forming a

viscous material detectable by ultrasound . Its natural

and this present study. Sudan is a north African country

with probably different dietary habit as studies have

history is variable; it may resolve spontaneously or may

progress to gallstone formation . Only one child was

estimated the prevalence of gallstones even amongst

their healthy people as high

compared to Nigeria and

noted to have biliary sludge. Sludge is usually a precur-

Ghana both of which are West African countries. Indeed

sor to the development of biliary stones as has been

reported by some authors . Hence, there is a need for

the pattern of cholelithiasis in SCD children seems to

mirror that of the general population where they reside.

this child to be followed up with routine scans for possi-

In Nigeria, the prevalence of gallstones is low in the

ble development of stone.

general population (0.8-1.8%)

while that of the Suda-

nese people is 5.2% . In Congo, a Central Africa coun-

Previous studies have reported a male predominance in

try, in a study of 190 young SCD individuals aged

cases of gallstones in SCD children; the male: female

ratio was 4:1 in this study, 12:1 in Ghana , 1.5:1 in

3-24yrs, 58.4% were found to have gallstones . The

Sudan , and 1.7:1 in Jamaica .

prevalence of gallstones in this study was lower than the

13% seen amongst the SCD children in Jamaican aged 5

Hemoglobin concentration was the only hematologic

years to 13 years . Again a difference in the dietary

parameter that was done in this study because it is a rou-

intake could be accountable for this, In Turkey, the

tine investigation conducted at every clinic visit. In this

prevalence of cholelithiasis amongst the 84 homozygous

study, the haemoglobin concentration was similar in

SCD aged 3-46 years that were studied was 50%, of

patients with and without gallstones. This finding is

similar to that reported by Attalla et al , Bond et al

these 15/84 had had cholecystectomy for gallstones and

and Sarnaik et al . However, Webb et al reported a

27/84 had ultrasound evidence of gallstones . In Italy,

in a study of 527 SCD subjects, the prevalence of gall

lower haemoglobin concentration in SCD patients with

373

gallstones compared to patients without stones.

Author’s Contributions

None of the patients in this study received cholecystec-

Odunvbun ME: Conceptualization of the work, Data

tomy. However, in SCD, prophylactic cholecystectomy

collection, Literature review, analysis, manuscript

is advised even for asymptomatic gallstones as morbid-

writing.

ity and mortality of emergency cholecystectomy in this

Adeyekun AA: Conceptualisation of the work,

setting is much higher than in elective cholecytectomy .

Ultrasound scanning of patients

Conflict of interest: None

Funding: None

Conclusion

Recommendation

In this study, prevalence of gallstones is low but consis-

tent with other studies from Nigeria and Ghana. Gall-

Gallstone can mimic abdominal crisis and other causes

stones were commoner in males than females and the

of surgical abdomen, therefore, there is need for routine

prevalence increased with increase in age. Haematocrit

screening with ultrasonography which is safe and inex-

and haemoglobin concerntration were similar amongst

pensive. In addition, screening allows for early detection

patients with and without gallstones.

and elective surgery for gallstones in asymptomatic

patients which is safer than emergency surgery for

symptomatic cases.

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