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    Growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula in infants with cow's milk protein allergy
    (Frontiers Media Sa, 2023) Kansu, Aydan; Urganci, Nafiye; Bukulmez, Aysegul; Kutluk, Gunsel; Taskin, Didem Gulcu; Keskin, Lutfiye Sahin; Igde, Mahir
    ObjectiveTo evaluate growth, tolerance and safety outcomes with use of an extensively hydrolyzed casein-based formula (eHCF) in infants with cow's milk protein allergy (CMPA). MethodsA total of 226 infants (mean & PLUSMN; SD age: 106.5 & PLUSMN; 39.5 days, 52.7% were girls) with CMPA who received eHCF comprising at least half of the daily dietary intake were included. Data on anthropometrics [weight for age (WFA), length for age (LFA) and weight for length (WFL) z-scores] were recorded at baseline (visit 1), while data on infant feeding and stool records, anthropometrics and Infant Feeding and Stool Patterns and Formula Satisfaction Questionnaires were recorded at visit 2 (on Days 15 & PLUSMN; 5) and visit 3 (on Days 30 & PLUSMN; 5). ResultsFrom baseline to visit 2 and visit 3, WFA z-scores (from -0.60 & PLUSMN; 1.13 to -0.54 & PLUSMN; 1.09 at visit 2, and to -0.44 & PLUSMN; 1.05 at visit 3, p < 0.001) and WFL z-scores (from -0.80 & PLUSMN; 1.30 to -0.71 & PLUSMN; 1.22 at visit 2, and to -0.64 & PLUSMN; 1.13 at visit 3, p = 0.002) were significantly increased. At least half of infants never experienced irritability or feeding refusal (55.7%) and spit-up after feeding (50.2%). The majority of mothers were satisfied with the study formula (93.2%), and wished to continue using it (92.2%). ConclusionsIn conclusion, eHCF was well-accepted and tolerated by an intended use population of infants & LE; 6 months of age with CMPA and enabled adequate volume consumption and improved growth indices within 30 days of utilization alongside a favorable gastrointestinal tolerance and a high level of parental satisfaction.
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    Prevalence of congenital sucrase-isomaltase deficiency in Turkey may be much higher than the estimates
    (Indian Acad Sciences, 2023) Taskin, Didem Gulcu; Civan, Hasret Ayyildiz; SarI, Emine Ergul; Altuntas, Cansu; Ersoy, Melike; Tuncel, Tolga; Onay, Huseyin
    Congenital sucrase-isomaltase deficiency (CSID) is a rare autosomal carbohydrate malabsorption disorder caused by mutations in the sucrase-isomaltase gene. While the prevalence of CSID is high in the indigenous populations of Alaska and Greenland, it is imprecise and ambiguous in the Turkish pediatric population. In this cross-sectional case-control study, which is retrospective in nature, next-generation sequencing (NGS) results obtained from records of 94 pediatric patients with chronic nonspecific diarrhea were reviewed. Demographic characteristics, clinical symptoms and treatment responses of those diagnosed with CSID were evaluated. We identified one new, homozygous frame-shift mutation and 10 other heterozygous mutations. Two cases were from the same family and nine were from different families. While the median age at onset of symptoms was 6 months (0-12), median age at diagnosis was 60 months (18-192) with a median delay of 5 years and 5 months (10 months -15 years and 5 months) in diagnosis. Clinical symptoms included diarrhea (100%), abdominal pain (54.5%), vomiting after consuming sucrose (27.2%), diaper dermatitis (36.3%) and growth retardation (81%). Our clinical study revealed that sucrase-isomaltase deficiency may have been underdiagnosed in patients with chronic diarrhea in Turkey. In addition, the frequency of heterozygous mutation carriers was significantly higher than that of homozygous mutation carriers and those with a heterozygous mutations responded well to the treatment.