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Biomedical subjects

C Mize

Publications and source records attributed to C Mize.

10 recordsLinked to original sources
PubMed1995

Defective molybdopterin biosynthesis: clinical heterogeneity associated with molybdenum cofactor deficiency.

A patient with molybdenum cofactor deficiency (producing the biochemical abnormalities associated with deficiencies of sulphite oxidase and xanthine dehydrogenase) clinically expressed Marfan-like habitus with dislocated lenses, vertebral abnormality, learning disability, moderate hemiplegia, increased medial lentiform MRI signal and intermittent microscopic haematuria. S-Sulphocysteine was present in plasma and urine, and the oxidized derivative of a molybdopterin precursor (precursor Z), together with xanthine and hypoxanthine, were elevated in urine. Blood uric acid was < 1 mg/dl, while urinary urothione was not detected. These data indicate a functionally inadequate terminal enzyme for converting precursor Z to active molybdopterin (complementation group B of general molybdenum cofactor deficiency). Although the biochemical parameters were indicative of a severe deficiency state, the patient has survived into the third decade with a less severe clinical spectrum than has generally been associated with this disease.

Adult↗
PubMed1992

Adaptation of feeding to a low fat yield in breast milk.

The effect of a low milk fat yield was assessed in a blinded prospective study of healthy term infants and mothers encouraged to breast-feed. Fat yield index was calculated as milk volume collected by Egnell pump multiplied by the "creamatocrit." Two weeks after delivery mothers who had a relatively low fat yield index (less than or equal to 30th percentile) were matched with mothers with a higher fat yield index and with formula-feeding mothers. Between 2 and 6 weeks the low fat yield group had a marginally lower weight gain but similar growth in length and head circumference to that of the higher fat yield group. The low fat yield group spent more time per feeding and had more complete breast emptying, resulting in a fat yield index comparable with the higher fat yield group for the mean milk volume ingested by the infant at 6 weeks. A low fat yield had no adverse effect on maternal satisfaction or maternal-infant interaction during feeding. Formula feeders spent the least time in feeding and en face gazing. Adaptation of breast-feeding to a low fat yield sustains infant intake and growth, maternal-infant interaction, and maternal satisfaction. However, there is little reserve if milk production diminishes. Further study is needed to define the limits of adaptation and effective interventions if fat yield is inadequate.

Breast Feeding↗
PubMed1991

Metabolic tolerance to arginine: implications for the safe use of arginine salt-aztreonam combination in the neonatal period.

Two similar cohorts of low birth weight infants whose size was appropriate for gestational age randomly received either aztreonam-arginine plus ampicillin (n = 15) or gentamicin plus ampicillin (n = 15) for empiric treatment of neonatal sepsis. The regimens were infused together with glucose at greater than 5 mg/kg per minute, and immediate (4 hours) and cumulative (3 days) effects were assessed. Serum arginine and insulin values rose immediately after administration of aztreonam (containing 0.15 mmol of arginine per kilogram), but there were no changes in the gentamicin-treated cohort; no differences occurred in either cohort in serum concentrations of glucose, ammonia, potassium, creatinine, and bilirubin. After 3 days of antibiotic therapy (n = 13), the baseline serum arginine concentration was almost twice as high in the aztreonam group and showed a similar further rise and fall during the 4 hours after infusion; arginine urinary fractional excretion (normalized to creatinine clearance) decreased in the gentamicin group. The indirect bilirubin concentration rose more (p less than 0.001) in the aztreonam-treated infants (5.1 to 11.5 mg/dl (87 to 196 mumol/L] than in the gentamicin-treated infants (5.5 to 8.1 mg/dl (94 to 138 mumol/L)). Thus a modest differential bilirubin response and modestly elevated baseline serum arginine level occurred after the 3-day low-arginine doses of this study; serum ammonia and glucose concentrations were not affected. Aztreonam-arginine in neonates was well tolerated metabolically, and we believe that it can be used safely in conjunction with attention to glucose and bilirubin metabolism.

Ammonia↗
PubMed1989

Use of 31P magnetic resonance spectroscopy to characterize evolving brain damage after perinatal asphyxia.

We investigated postasphyxial brain damage with 31P magnetic resonance spectroscopy (MRS) and correlated it with neurologic assessment and standard laboratory evaluation during the first 10 months of life in 1 infant, baby G. We compared these observations to 31P MRS data from 7 healthy term newborns, 1 normal infant examined serially over the first 8.5 months of life, and 5 other term infants following perinatal asphyxia. MRS noninvasively provides biochemical correlates of the evolution of brain damage following perinatal asphyxia and suggests that pH derived from the inorganic phosphate peak may serve as a marker for brain injury.

Asphyxia Neonatorum↗
PubMed1989

Randomized trial of taurine supplementation for infants less than or equal to 1,300-gram birth weight: effect on auditory brainstem-evoked responses.

Taurine may be important to the developing eye and brain of the small preterm infant. A blinded randomized trial was conducted to determine whether taurine supplementation of healthy infants of less than or equal to 1,300 g birth weight until their discharge from the hospital increases their growth rate, neurobehavioral development, electroretinographic development, or maturation of auditory brainstem-evoked responses. Infants were fed with Similac Special Care as desired, which was prepared to contain less than 5 mg/L of taurine or 45 mg/L of taurine, a concentration similar to that of human milk. Infants who did not receive taurine supplementation (n = 19) and those who did (n = 18) were similar with respect to condition at study entry, caloric intake, and growth rates throughout the study, and electroretinographic findings and scores on the Brazelton Behavioral Assessment Scale at 37 weeks' postmenstrual age. Infants who received taurine supplementation had greater overall plasma taurine concentrations. The group receiving taurine supplementation also had more mature auditory-evoked responses at 37 weeks' postmenstrual age with a modest (0.2 to 0.5 ms) but consistent reduction (P less than .05) in the interval between stimulus and response at two different stimulation rates. Although further study is needed, taurine intake appears to influence auditory system maturation of preterm infants.

Amino Acids↗
PubMed1989

More on taurine.

Explore the source record for details and available documents.

Drug Evaluation↗
PubMed1978

Erythropoiesis: comparison of in vivo and in vitro amplification.

1. Amplification is defined as the phase of erythropoiesis that includes all cell divisions of the recognizable erythron. 2. In vivo, amplification always takes place surrounding a central histiocyte (erythroblastic island). It is made up of four to five successive synchronous divisions. 3. In vitro, complete maturation and amplification of erythropoietic cells can be obtained without apparent association with a central histiocyte. 4. The functional significance of the 'erythroblastic island' and disorders of amplification are discussed in relation to clinical disorders of erythropoiesis.

Animals↗
PubMedDate not supplied

[In vitro studies on amplification of rat erythropoiesis (author's transl)].

From rat bone marrow in which erythropoiesis has been depressed (by suppression of erythropoietin production), liquid culture has yielded a predominantly synchronous amplification of erythroblast development. Groups of 2, 4, 8, 16 and 32 erythrolbasts are observed at 9-15 hour intervals in successive stages of maturation, similar (by their staining characteristics and nuclear size) to that seen in vivo.

Animals↗