ABSTRACT
Early dietary treatment of phenylketonuria (PKU), an inborn error of phenylalanine (Phe) metabolism, results in normal cognitive development. Although health-related quality of life (HRQoL) of PKU patients has been reported as unaffected in high-income countries, there are scarce data concerning HRQoL and adherence to treatment of PKU children and adolescents from Brazil. The present study compared HRQoL scores in core dimensions of Brazilian early-treated PKU pediatric patients with those of a reference population, and explored possible relationships between adherence to treatment and HRQoL. Early-treated PKU pediatric patient HRQoL was evaluated by self- and parent-proxy reports of the Pediatric Quality of Life Inventory (PedsQL) core scales. Adherence to treatment was evaluated by median Phe levels and percentage of results within the therapeutic target range in two periods. Means for total and core scales scores of PedsQL self- and parent proxy-reports of PKU patients were significantly lower than their respective means for controls. Adequacy of median Phe concentrations and the mean percentage of values in the target range fell substantially from the first year of life to the last year of this study. There was no significant difference in mean total and core scale scores for self- and parent proxy-reports between patients with adequate and those with inadequate median Phe concentrations. The harmful consequences for intellectual capacity caused by poor adherence to dietary treatment could explain the observed decrease in all HRQoL scales, especially in school functioning. Healthcare system and financial difficulties may also have influenced negatively all HRQoL dimensions.
Subject(s)
Phenylketonurias/diet therapy , Quality of Life , Treatment Adherence and Compliance/statistics & numerical data , Adolescent , Age Factors , Analysis of Variance , Brazil , Child , Female , Humans , Intelligence Tests , Linear Models , Male , Parents , Phenylalanine/blood , Phenylketonurias/psychology , Proxy , Quality of Life/psychology , Secondary Prevention , Self Report , Socioeconomic Factors , Time Factors , Treatment Adherence and Compliance/psychology , Treatment OutcomeSubject(s)
Gonadotropins/pharmacology , Muscle Contraction/drug effects , Ovary/drug effects , Adult , Chorionic Gonadotropin/administration & dosage , Chorionic Gonadotropin/pharmacology , Female , Gonadotropins, Pituitary/administration & dosage , Gonadotropins, Pituitary/pharmacology , Humans , Injections, Intramuscular , Menstruation , Muscle, Smooth/drug effectsABSTRACT
PIP: In vivo studies were carried out in 8 women to ascertain the effects of prostaglandins (PGs) E2 and F2alpha on the human fallopian tubes and ovaries. Recording devices were surgically implanted into the tubal lumen through catheters. 100 mcg. of either PGE2 or PGF2alpha or a mixture of equal amounts of the 2 totaling 100 mcg. were administered intravenously and the effects recorded. Graphs of the effects are presented. The 2 pure substances and the mixture all had a stimulatory effect on the uterus at all phases of the menstrual cycle. There were, however, qualitative and potency differences between PGE2 and PGF2alpha. On a weight basis, PGE2 had more powerful effects on the myometrium. The 2 PGs had opposing effects on the fallopian tubes, with PGF2alpha stimulating while PGE2 inhibited tubal motility. A contractile response of the ovary following intravenous administration of PGF2alpha was noted; no change in intraovarian pressure was recorded following injection of up to 100 mcg. PGE2. The compound was mildly stimulatory. The ability of PGE2 to induce relaxation of the tubes while stimulating uterine activity provides a clue as to why it is more effective in inducing abortion than PGF2alpha. It is suggested that relaxation of the tubal isthmus induced by seminal PGE2 facilitates sperm penetration into the tube.^ieng
