P394 Impact of gastrointestinal and urological problems in children with myotonic dystrophy type 1
Gastrointestinal and urological problems impact on daily life of patients with myotonic dystrophy type 1 (DM1) but have remained understudied. In a cross-sectional study, frequency and nature of gastrointestinal and urological problems in children with DM1 aged 5-18 years were assessed by a combinat...
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Published in | Neuromuscular disorders : NMD Vol. 33; pp. S156 - S157 |
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Main Authors | , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
Elsevier B.V
01.10.2023
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Abstract | Gastrointestinal and urological problems impact on daily life of patients with myotonic dystrophy type 1 (DM1) but have remained understudied. In a cross-sectional study, frequency and nature of gastrointestinal and urological problems in children with DM1 aged 5-18 years were assessed by a combination of the Childhood Bladder and Bowel Dysfunction Questionnaire (CBBDQ), supplementary BBD questionnaire previously used in Duchenne muscular dystrophy and the Clinical Rating Scale for Gastrointestinal Symptoms (GSRS). Parents rated the influence of urological and gastrointestinal problems on daily life, both from the patient's and family perspective. We included 58 children (30 males, 28 females) with a mean age of 13 years; 74.1% reported at least one gastrointestinal problem. Abdominal pain was the most frequently reported problem (51.7%), followed by dysphagia (41.8%), diarrhoea (36.2%), encopresis (36.0%), constipation (32.7%), bloating and flatulence (both 25.9%). The most frequently reported urological problem was urinary incontinence (22.0%), followed by enuresis nocturna (10.3%) and voiding symptoms (23.5% hesitancy, 4.8% intermittency and 13.8% dysuria). The process of toilet training was described as difficult by 59.3% of the parents and 13.8% of the children was not successfully toilet-trained at enrolment. The majority considered urological and gastrointestinal symptoms to have a negative influence on their daily life; 22.4% of parents reported severe influence on daily family life (shame, social restrictions, school absence and concerns for their children's future). Considering the high prevalence of urological and gastrointestinal problems in children with DM1 and their influence on daily life it is key to correctly recognize, diagnose and treat these problems. We recommend standard screening for gastrointestinal and urological complaints in children with DM1. |
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AbstractList | Gastrointestinal and urological problems impact on daily life of patients with myotonic dystrophy type 1 (DM1) but have remained understudied. In a cross-sectional study, frequency and nature of gastrointestinal and urological problems in children with DM1 aged 5-18 years were assessed by a combination of the Childhood Bladder and Bowel Dysfunction Questionnaire (CBBDQ), supplementary BBD questionnaire previously used in Duchenne muscular dystrophy and the Clinical Rating Scale for Gastrointestinal Symptoms (GSRS). Parents rated the influence of urological and gastrointestinal problems on daily life, both from the patient's and family perspective. We included 58 children (30 males, 28 females) with a mean age of 13 years; 74.1% reported at least one gastrointestinal problem. Abdominal pain was the most frequently reported problem (51.7%), followed by dysphagia (41.8%), diarrhoea (36.2%), encopresis (36.0%), constipation (32.7%), bloating and flatulence (both 25.9%). The most frequently reported urological problem was urinary incontinence (22.0%), followed by enuresis nocturna (10.3%) and voiding symptoms (23.5% hesitancy, 4.8% intermittency and 13.8% dysuria). The process of toilet training was described as difficult by 59.3% of the parents and 13.8% of the children was not successfully toilet-trained at enrolment. The majority considered urological and gastrointestinal symptoms to have a negative influence on their daily life; 22.4% of parents reported severe influence on daily family life (shame, social restrictions, school absence and concerns for their children's future). Considering the high prevalence of urological and gastrointestinal problems in children with DM1 and their influence on daily life it is key to correctly recognize, diagnose and treat these problems. We recommend standard screening for gastrointestinal and urological complaints in children with DM1. |
Author | Klinkenberg, S. Vrijens, D. van den Berg, J. Maagdenberg, S. Altena-Rensen, S. Janssen, E. Braakman, H. Gierenz, N. de Wall, L. |
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Title | P394 Impact of gastrointestinal and urological problems in children with myotonic dystrophy type 1 |
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