ABSTRACT
BACKGROUND: Clinical advice may suggest discontinuing breastfeeding after the diagnosis of phenylketonuria in infants as the only effective way to monitor the newborn's intake and accurate measurement of phenylalanine (Phe). This study aims to investigate the prevalence and duration of breastfeeding, as well as its effect on serum Phe levels in infants with phenylketonuria at Education and Therapy Medical Center, Be'sat Hospital, Iran. METHODS: We conducted a cross-sectional study of 34 children under two years old diagnosed with phenylketonuria between September 2018 and December 2022. Infants were categorized as breastfed and non-breastfed (bottle-fed) based on their feeding method after diagnosis. Data on age at diagnosis, medical records, demographic information, and anthropometric indices were collected, and infants with incomplete data or mixed feeding (formula + breast milk) were excluded from the study. RESULTS: Of 94 infants managed in our hospital, 34 had complete medical records. Among the all patients 13 (38%) continued to be breastfed combined with phenylalanine-free amino acid-based protein substitute, while 21 (62%) were did not receive breast milk. The mean duration of breastfeeding was 2.57 ± 0.59 (1-3) months. The mean age at diagnosis was 22.6 ± 18.4 days. Phenylalanine concentrations at diagnosis were mean 10, SD 5.44; range 4-24 mg/dL [0.22-1.33 µmol/L] in the breastfed group and mean 14.3, SD 10.2; range 5-37 mg/dL [0.27-2.05 µmol/L] in the non-breastfed group.Non-breastfed infants had lower serum Phe levels than breastfed infants: mean 3.76, SD 2.10; range 1-7 mg/dL [0.05-0.38 µmol/L] and mean 4.89, SD 3.68; range 2-19 mg/dL [0.11-1.05 µmol/L], respectively, although not statistically significant [(t (34) = 118.0, P = 0.51]. Also we found no significant associations in body measurements for weight, height, and head circumference at birth and final assessment. CONCLUSIONS: In conclusion, during treatment, there were no statistically significant associations between breastfeeding and serum Phe levels with growth in children with phenylketonuria.
Subject(s)
Breast Feeding , Phenylketonurias , Infant , Infant, Newborn , Female , Child , Humans , Child, Preschool , Breast Feeding/methods , Cross-Sectional Studies , Milk, Human , PhenylalanineABSTRACT
BACKGROUND AND OBJECTIVE: Although several studies have assessed the effect of folate supplementation on lipid profiles among patients with metabolic diseases, findings are inconsistent. This review of randomized controlled trials (RCTs) was conducted to summarize the evidence on the effects of folate supplementation on lipid profiles among patients with metabolic diseases. METHODS: Randomized-controlled trials (RCTs) published in PubMed, EMBASE, Web of Science and Cochrane Library databases up to until 20 August 2017 were searched. Two review authors independently assessed study eligibility, extracted data, and evaluated risk of bias of included studies. Heterogeneity was measured with a Q-test and with I2 statistics. Data were pooled by using the fix or random-effect model based on the heterogeneity test results and expressed as standardized mean difference (SMD) with 95% confidence interval (CI). RESULTS: A total of thirteen randomized controlled trials were included. Folate supplementation did not affect systolic blood pressure (SMD -0.87; 95% CI, -1.83, 0.09) and diastolic blood pressure (SMD -0.59; 95% CI, -1.55, 0.37), and lipid profiles including triglycerides (SMD 0.10; 95% CI, -0.42, 0.63), total- (SMD 0.06; 95% CI, -0.31, 0.43), HDL- (SMD 0.04; 95% CI, -0.36, 0.44), VLDL- (SMD 0.08; 95% CI, -0.24, 0.41), and LDL-cholesterol (SMD -0.14; 95% CI, -0.55, 0.28). CONCLUSIONS: Folate supplementation did not affect blood pressures and lipid profiles among patients with metabolic diseases. Additional prospective studies regarding the impact of folate supplementation on blood pressures and lipid profiles in patients with metabolic diseases are necessary.
