Nocturnal enuresis and albuminuria or proteinuria are markers of renal damage in sickle cell disease (SCD) and commonly develop early on in life. Proteinuria progresses with age, leading to chronic kidney disease in adulthood. The aims of this study were to identify the prevalence of enuresis and albuminuria/proteinuria in paediatric patients with SCD in London and to determine the relationship between these and various demographic and clinical variables.

Methods

A cross sectional, single centre study was conducted. Questionnaire-based interviews themed on nocturnal enuresis were undertaken for patients between the ages of 6 and 17. Urinalysis was performed for the presence of albuminuria or proteinuria. Hospital patient records were accessed for clinical data.

Results

A total of 56 patients were recruited to the study, of which 27 (51.8%) were female. Twenty patients (35.7%) had a history of enuresis and met the DSM IV (Diagnostic and Statistical Manual of Mental Disorders, fourth edition) criteria for nocturnal enuresis compared to 5% prevalence in children in the general population1.

There was a statistically significant association between enuresis and age, overactive bladder (OAB) symptoms, sleep-disordered breathing (SDB), painful crises and regular transfusions (Table 1). Fourteen out of 29 patients (48.3%) with OAB symptoms reported nocturnal enuresis compared to six out of 27 patients (22.2%) who did not (p<0.05). Of the patients reporting SDB, 48.6% were enuretic compared to 14.3% who did not (p<0.01). Incidence of painful crises per month was higher for the enuretic group (2.29 vs. 0.88, p<0.05) but the mean number of emergency admissions in the preceding 18 months was similar. Of the 14 regular blood transfusion patients, only one (7.1%) was currently enuretic compared to 14 out of 32 (40.6%) who were not receiving any treatment (p<0.05).

Table 1

Prevalence of nocturnal enuresis against various patient characteristics (n=56)

Patient FeatureHistory of enuresis%Current enuresis (n)%P value
Gender Male 11/29 37.9    
 Female 9/27 33.3   0.72 
Age group (years) 6-9   6/15 40.0  
 10-15   8/26 30.8  
 16-17   1/15 6.7 <0.05 
Phenotype HbSS, HbSβ0 thalassaemia 13/43 30.2    
 HbSC, HbSβ+ thalassaemia 7/13 53.8   0.119 
OAB symptoms Yes 29 14 48.3   
 No 27 6 22.2  <0.05 
SDB Yes 35 17 48.6   
 No 21 3 14.3  <0.05 
Hydroxycarbamide Yes  1/10 10.0  
 No  14/32 40.6 0.073 
Regular blood transfusion Yes  1/14 7.1  
 No  14/32 40.6 <0.05 
Patient FeatureHistory of enuresis%Current enuresis (n)%P value
Gender Male 11/29 37.9    
 Female 9/27 33.3   0.72 
Age group (years) 6-9   6/15 40.0  
 10-15   8/26 30.8  
 16-17   1/15 6.7 <0.05 
Phenotype HbSS, HbSβ0 thalassaemia 13/43 30.2    
 HbSC, HbSβ+ thalassaemia 7/13 53.8   0.119 
OAB symptoms Yes 29 14 48.3   
 No 27 6 22.2  <0.05 
SDB Yes 35 17 48.6   
 No 21 3 14.3  <0.05 
Hydroxycarbamide Yes  1/10 10.0  
 No  14/32 40.6 0.073 
Regular blood transfusion Yes  1/14 7.1  
 No  14/32 40.6 <0.05 
View Large

Seven patients (13.4%) had albuminuria/proteinuria. (Table 2)

Table 2

Prevalence of albuminuria or proteinuria across various patient characteristics (n=52)

Patient FeatureAlbuminuria or proteinuria%P value
Gender Male 2/28 10.7  
 Female 5/24 37.5 0.149 
Age group 6-9 1/15 6.7  
 10-15 3/26 11.5  
 16-17 3/11 27.3 0.146 
Hydroxycarbamide Yes 1/9 11.1  
 No 3/31 9.7 0.900 
Regular blood transfusion Yes 3/12 25.0  
 No 3/31 9.7 0.193 
Haematuria - or trace 2/45 4.4  
 +, ++, +++ 5/7 71.4 <0.001 
BP Normotensive 5/41 12.2  
 Hypertensive 2/9 22.2 0.432 
Current enuresis Yes 0/15 0.0  
 No 7/37 18.9 0.070 
Patient FeatureAlbuminuria or proteinuria%P value
Gender Male 2/28 10.7  
 Female 5/24 37.5 0.149 
Age group 6-9 1/15 6.7  
 10-15 3/26 11.5  
 16-17 3/11 27.3 0.146 
Hydroxycarbamide Yes 1/9 11.1  
 No 3/31 9.7 0.900 
Regular blood transfusion Yes 3/12 25.0  
 No 3/31 9.7 0.193 
Haematuria - or trace 2/45 4.4  
 +, ++, +++ 5/7 71.4 <0.001 
BP Normotensive 5/41 12.2  
 Hypertensive 2/9 22.2 0.432 
Current enuresis Yes 0/15 0.0  
 No 7/37 18.9 0.070 
View Large

There was no difference in albuminuria/proteinuria prevalence between the hydroxycarbamide or blood transfusion group compared to the non-treatment group. There was no difference in HbF percentage, systolic BP, frequency of emergency admissions, painful crises per month, haemoglobin levels and estimated glomerular filtration rates (eGFR) in patients with albuminuria/proteinuria and those without. The prevalence of haematuria increased with age; 6.7% in the 6-9 age category compared to 36.4% in the 16 to 17 age category (p=<0.05).

Conclusions

Nocturnal enuresis and albuminuria or proteinuria is prevalent at an early age in many children with SCD. Early identification and initiation of treatment such as Angiotensin Converting Enzyme inhibitors2 may delay onset of complications especially alongside beneficial sickle cell treatments such as hydroxycarbamide and regular blood transfusions. Questioning parents on enuresis, OAB and SDB sumptoms and undertaking regular urinalysis on younger age groups is a practical and cost-effective surveillance method.

References

1. American Psychiatric Association. Diagnostic and statistical manual of mental disorders: DSM-IV-TR. American Psychiatric Publishing, Inc.; 2000.

Angiotensin-converting enzyme (ACE) inhibitors for proteinuria and microalbuminuria in people with sickle cell disease Cochrane Database of Systematic Reviews, 2013.

Disclosures:

No relevant conflicts of interest to declare.

Topics:
child, enuresis, pain, proteinuria, sickle cell anemia, albuminuria, blood transfusion, hydroxyurea, hematuria, thalassemia
© 2013 by The American Society of Hematology
2013
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