Subject(s)
Lung/physiopathology , Respiratory Distress Syndrome, Newborn/physiopathology , Child , Child, Preschool , Humans , Infant, Newborn , Intensive Care Units , Lung/diagnostic imaging , Oxygen Inhalation Therapy/adverse effects , Radiography , Respiratory Distress Syndrome, Newborn/complications , Respiratory Distress Syndrome, Newborn/diagnostic imaging , Respiratory Distress Syndrome, Newborn/therapy , Respiratory Function TestsABSTRACT
Ninety-four patients with classical phenylketonuria are reviewed. Early treatment with a low phenylalanine diet usually results in normal or near-normal I.Q. Twenty-seven of the 38 patients diagnosed before 2 months of age have an I.Q. in the normal range. Of the other 11, six are dull normal, four are borderline and one is frankly retarded. Adequate reasons are given for the less than optimal results in these 11 early-treated patients. Twenty-one of the 28 patients presenting with abnormal electroencephalograms showed normal tracings soon after initiation of diet and four of the other seven improved. Nine of 12 on continuous anticonvulsant medication at the time of presentation were able to discontinue this soon after institution of dietary measures. Eighteen of the 19 sibling pair comparisons revealed a better ultimate I.Q. in the early-treated sib. A plea for early diagnosis by continuation of newborn screening programs and for centralization of diagnostic confirmation and treatment is made.
Subject(s)
Diet Therapy , Phenylketonurias/therapy , Age Factors , Anticonvulsants/therapeutic use , Brain Damage, Chronic/etiology , Child, Preschool , Electroencephalography , Humans , Infant , Infant, Newborn , Infant, Newborn, Diseases/diagnosis , Infant, Newborn, Diseases/therapy , Intellectual Disability/etiology , Intellectual Disability/prevention & control , Intelligence Tests , Ontario , Phenylketonurias/complications , Phenylketonurias/diagnosis , Time FactorsSubject(s)
Respiration, Artificial , Respiratory Distress Syndrome, Newborn/therapy , Arteries , Birth Weight , Blood , Carbon Dioxide/blood , Clinical Trials as Topic , Female , Gestational Age , Humans , Hydrogen-Ion Concentration , Infant, Newborn , Male , Oxygen/blood , Positive-Pressure Respiration , Prognosis , Respiratory Distress Syndrome, Newborn/mortality , Respiratory Insufficiency/mortality , Respiratory Insufficiency/therapy , Ventilators, MechanicalSubject(s)
Diet Therapy/adverse effects , Nutrition Disorders/etiology , Phenylketonurias/complications , Anemia/etiology , Bone Diseases, Developmental/etiology , Child, Preschool , Deficiency Diseases/etiology , Growth Disorders/etiology , Humans , Hypoproteinemia/etiology , Infant , Intellectual Disability/etiology , Phenylalanine/blood , Phenylketonurias/therapyABSTRACT
Forty-five newborn infants in respiratory failure with respiratory distress syndrome were treated with intermittent negative pressure ventilation (INPV). There was a survival rate of 38% (17/45).All infants were initially treated without nasotracheal intubation. However, 24 of these developed a Paco(2) greater than 70 mm. Hg and were subsequently intubated. Intubation was followed by a decrease in the degree of hypercarbia in each instance and simultaneous increase in Pao(2).COMPLICATIONS ENCOUNTERED DURING VENTILATION WERE: emphysema (one patient), aspiration pneumonia (two patients), septicemia (two patients), misplaced nasotracheal tube (one patient).Follow-up of the 17 surviving patients for periods of four to 36 months disclosed two patients with post-intubation hoarseness. One infant initially had spastic quadriplegia with EEG abnormalities, both of which cleared by 5 months of age. In the remaining 14 infants, the results of physical, neurological and psychological examinations have remained within normal limits.
