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Biomedical subjects

J Pietz

Publications and source records attributed to J Pietz.

25 records · Page 2Linked to original sources
PubMed1989

Continuous negative pressure in the treatment of infants with pulmonary hypertension and respiratory failure.

We report the successful use of continuous negative pressure (CNP) with standard intermittent mandatory ventilation (IMV) in five patients suffering from respiratory failure and persistent pulmonary hypertension of the newborn (PPHN). These infants all fulfilled criteria for use of extracorporeal membrane oxygenation (ECMO) with PaO2 less than 40 torr, alveolar-arterial oxygen difference (AaDO2) greater than 620 mm Hg, and oxygenation index (OI) greater than 50. Despite a considerable amount of conventional ventilation with mean airway pressures (PAW) between 14 and 26 cm water, none of these patients were able to improve oxygenation. All infants demonstrated significant improvement in ventilation requirements after initiation of CNP as reflected by a decrease in PAW, proximal inspiratory pressure (PIP), and IMV. Oxygenation dramatically improved in all infants. All five patients survived without any pulmonary or neurological complications at discharge. Availability of CNP may circumvent the need for ECMO in infants with severe lung disease and PPHN.

Extracorporeal Membrane Oxygenation↗
PubMed1988

Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early.

In 34 children with phenylketonuria (PKU) treated early the prognostic value of the age on institution of the diet (within the first 3 months of life) and of the quality of dietary treatment was determined in two different ways: 1) following intelligence closely (IQ) and (2) evaluating the EEG development up to their 12th (n = 34) and 15th (n = 18) years of life as appropriate. In general, IQ scores were found to be normal from the 4th-15th years of life. In our group of patients there was no effect on the IQ of the timing of diet onset. Children with "strict" dietary control showed a significantly higher IQ than those with "loose" control. One hundred and fifty-four EEGs (10/20 system, awake with eyes closed) were recorded at intervals of 2 years and conventionally evaluated. The development of alpha-activity was found to be normal. Beta-activity was enhanced. Abnormal EEG findings like general slowing and generalized paroxysmal activity (GPA) with or without spikes were more frequent in children with PKU than in controls, with the exception of focal abnormalities. EEG abnormalities increased with advancing age independently of IQ development and showed no relation to either the age at the onset nor the quality of dietary treatment.

Adolescent↗
PubMed1988

Visual evoked potentials of mildly mentally retarded and control children.

Visual evoked potentials (VEPs) were recorded from 25 10- to 13-year-old mildly mentally retarded children and compared with those from 31 control children of the same age-range. Correlations of VEPs with age were weak, but a relationship between VEPs and IQ was demonstrated for the control group. The retarded group had significantly longer latencies and higher amplitude peaks than the control group, with the differences occurring primarily over non-specific cortex and for secondary components. Analysis also showed that the retarded group were neurophysiologically heterogeneous. Since the same children had been analyzed earlier by quantitative EEG methods, comparisons are made with respect to these two methods of investigating brain function.

Adolescent↗
PubMed1988

Hypoxanthine: a marker for asphyxia.

It has been hypothesized that hypoxanthine concentrations in the blood of newborn infants are a marker of asphyxia. To test this hypothesis, we measured serum hypoxanthine levels in relationship to perinatal and neonatal asphyxia, and compared arterial hypoxanthine levels with arterial pH and base deficit. We also compared hypoxanthine levels of survivors with those of asphyxiated non-survivors. Forty-two newborns were classified as asphyxiated by either of two methods: 1) Infants from whom umbilical cord hypoxanthine levels were taken were classified as asphyxiated if they had an Apgar score of 6 or less at 1 or 5 minutes, fetal heart rate below 100 beats per minute, or meconium-stained amniotic fluid; and 2) infants from whom peripheral arterial hypoxanthine samples were taken were classified by clinical assessment, whereby one author, blinded to the infants' hypoxanthine levels, prospectively assessed each patient's condition for evidence of asphyxia. Hypoxanthine levels correlated with increased base deficit (P less than .001; r = 0.8) and with decreased pH (P less than .001; r = -0.5). By both of our asphyxia classification methods, hypoxanthine levels were significantly higher (P less than .002) in the asphyxiated groups. We also noted a higher hypoxanthine level in asphyxiated non-survivors as compared with all survivors (P less than .02). We propose that serum hypoxanthine levels may help define asphyxia. Because hypoxanthine, when metabolized by xanthine oxidase, generates oxygen radicals that are highly destructive to tissue, hypoxanthine levels may have important therapeutic implications for asphyxiated patients.

Asphyxia Neonatorum↗
PubMedDate not supplied

Treatment of infantile spasms with high-dosage vitamin B6.

High-dose vitamin B6 (pyridoxine-HCl, 300 mg/kg/day orally) was introduced as the initial treatment of recently manifested infantile spasms in 17 children (13 symptomatic cases with identified brain lesion and 4 cryptogenic cases). 5 of 17 children (2 cryptogenic, 2 with severe pre/perinatal brain damage and one with Sturge-Weber syndrome) were classified as responders to high-dose vitamin B6. In all 5 cases the response to vitamin B6 occurred within the first 2 weeks of treatment and within 4 weeks all patients were free of seizures. Two patients developed other seizures (partial seizures, etiologically unclear blinking attacks), but no relapse of infantile spasms was observed among the five responders to vitamin B6. No serious adverse reactions were noted. Side effects were mainly gastrointestinal symptoms, which were reversible after reduction of the dosage. Considering the life-threatening side effects of treatment with ACTH/corticosteroids or valproate, a controlled clinical trial with high-dose vitamin B6 would appear justified to either prove or disprove efficacy.

Administration, Oral↗
PubMedDate not supplied

Propionic acidemia without acidemia: a case report.

Propionic acidemia is a recessively inherited disorder of organic acid metabolism characterized by a spectrum of clinical and biochemical findings. Metabolic acidosis is a key feature of this disease and is useful in differentiating it from nonketotic hyperglycinemia. This case report is presented because significant metabolic acidosis was not a prominent or persistent finding in this patient.

Acidosis↗