ICNC Abstracts, ICNC 2018

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Prevalence of smooth muscle dysfunction among children with Duchenne muscular dystrophy
Ranjith Kumar Manokaran, Biswaroop Chakrabarty, Prashant Jauhari, Rakesh Kumar, Sandeep Aggarwala, Arun Gupta, Manisha Jana, R M Pandey, Sheffali Gulati

Last modified: 2018-09-09

Abstract


Introduction:

DMD patients suffer from smooth muscle related co-morbidities. With a need to unravel further, we embarked on a cross sectional study to evaluate smooth muscle dysfunction amongDMD patients.

Objective:

To estimate prevalence of smooth muscle dysfunction in children with DMD (genetically /biopsy proven) aged 5 years and above in terms of vascular, upper gastrointestinal and bladder smooth muscle on basis of questionnaires using a pre-structured proforma(Pediatric bleeding questionnaire, dysfunctional voiding scoring system, pediatric gastroesophageal symptom questionnaire (PGSQ)). To estimate bleeding time (mean ± SD) using Duke`s method, prevalence of gastroesophageal reflux using technetium 99 gastroesophageal nuclear scintigraphy and prevalence of urodynamic abnormalities using uroflowmetric assessment

Methods:

Prestructured proforma was administered among 99 patients. They underwent bleeding time test, GER scan, uroflowmetry and ultrasonographic evaluation for post void residual urine.

Results:

Prevalence of smooth muscle dysfunction in terms of vascular, upper GI and bladder smooth muscle by administering  a pre-structured proforma was 27.2 % (95 % C.I - 18.5 to 35.7). Nocturnal enuresis and urinary frequency were reported in 16 and 12 % respectively.Mean bleeding time was 387.5 seconds (Mildly prolongation). Prevalence of gastroesophageal reflux on GER scan was 21%.On uroflowmetry, we foundvoided volume/estimated bladder capacity  > 15 %  and abnormal flow curves were seen in 18.2 % and  9.7 % respectively. Excessive post void residual urine volume was seen in 25.6 %.

Conclusions:

Smooth muscle dysfunctionwas seen in atleast one third of study population highlighting theneed for addressing these issues in patient care.


Keywords


Smooth muscle dysfunction, Duchenne muscular dystrophy, children

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