ISSN:
1432-198X
Keywords:
Key words Cystinosis
;
Gastrostomy button
;
Nutritional support
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Poor growth in children with cystinosis may be a combination of sub-optimal nutritional intake, gastrointestinal dysfunction and polypharmacy. We report our experience with gastrostomy buttons (GBs) in five children (4 male) with cystinosis treated at a median age of 3 years (range 1.4–8.8 years). All children received overnight GB feeding for a mean duration of 27 months (range 7–42 months). During 135 patient-months of observation, the mean life of the GB was 5.7 months (range 3–7.7 months), with a mean of 3.4 GB changes (range 1–6) per patient. An average of five (range 3–7) medications were administered daily via the button. The mean energy intake achieved was 118% of the estimated average requirement (range 86%–183%), with 58% of total energy and 55% of protein intake being delivered via the GB during the study period. Mean height standard deviation score (SDS) was –3.79 at the start of GB feeding and –3.08 at the end of the study period, with mean weight SDS of –3.79 and –2.17 (P=0.042), respectively. The buttons have been welcomed by the families and should be considered as a treatment option for providing the nutritional prescription and administering medications.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/s004670000318
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