Bilirubin binding, free fatty acids and a new concept for the pathogenesis of kernicterus.
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Biomedical subjects
Publications and source records attributed to L M Gartner.
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A group of 412 infants with birthweights between 501 and 2,500 gm and gestational ages of 36 weeks or less were studied for the influence of both prolonged ruputre of amniotic membranes and maternal hypertension on the incidence of idiopathic respiratory distress syndrome (IRDS). The occurrence of these complications was associated with a significant decrease in the incidence of IRDS only in infants with birthweights between 1,501 and 2,500 gm (37.4% in the no complications group versus 12.8% in the complications group; P less than .01) or gestational ages of 33 to 36 weeks (35.2% in the no complications group versus 13.1% in the complications group; P less than .01). In infants with birthweights of 1,500 gm or less or gestational ages of 32 weeks or less, the specific antecedent complications of pregnancy did not alter the incidence of IRDS. Rupture of the membranes for more than 72 hours had no greater effect on the incidence of IRDS than those lasting 24 to 72 hours.
An analysis of birthweights of 337 neonates in relation to history of maternal narcotic usage was undertaken. Mean birthweight of infants born to mothers abusing heroin during the pregnancy was 2,490 gm, an effect primarily of intrauterine growth retardation. Low mean birthweight (2,615 gm) was also seen in infants born to mothers who had abused heroin only prior to this pregnancy, and mothers who had used both heroin and methadone during the pregnancy (2,535 gm). Infants born to mothers on methadone maintenance during the pregnancy had significantly higher mean birthweights (2.961 gm), but lower than the control group (3,176 gm). A highly significant relationship was observed between maternal methadone dosage in the first trimester and birthweight, i.e., the higher the dosage, the larger the infant. Heroin causes fetal growth retardation, an effect which may persist beyond the period of addiction. Methadone may promote fetal growth in a dose-related fashion after maternal use of heroin.
A simple, rapid microfluorometric method utilizing a fluorescent dye, Direct Yellow 7, for quantitative measurement of serum albumin-binding capacity for bilirubin has been developed. The dye binds exclusively to serum albumin with enhancement of fluorescence. The dye shares bilirubin-binding sites on albumin with up to 2 mols of bilirubin per mol of albumin with differing affinities to the first and second sites. Lowered pH reduces albumin-binding capacity and exposure to light increases binding capacity of icteric sera. The binding capacity of adult human sera is greater than that of human cord sera or purified human serum albumin compared on albumin molar basis.
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A solitary hepatic abscess was found at postmortem examination in a neonate who died at 25 days of age. Elevation of the right diaphragm was noted radiologically from the first day of life, suggesting congenital presence of the abscess. Recognition of the previously unsuspected association between hepatic abscess and congenital diaphragmatic elevation might have led to surgical intervention and have increased the infant's chance of survival.
The cerebro-hepato-renal syndrome is a rare familial malady with cerebral, renal, and skeletal abnormalities, severe hypotonia, cirrhosis, iron and lipid storage, and death within 6 months. Correlated electron microscopic, histochemical, and biochemical studies demonstrate defects in two oxidative organelles. Peroxisomes cannot be found in hepatocytes and renal proximal tubules. In hepatocytes and cortical astrocytes, mitochondria are distorted in their appearance and glycogen stores are increased. Oxygen consumnption of brain and liver mitochondrial preparations with succinate and with substrates reducing nicotinamide adenine dinucleotide is markedly diminished, but the consumption is normal with ascorbate and tetramethylphenylenediamine, which suggests a defect in electron transport prior to the cytochromes. Histochemical studies of mitochondrial oxidation point to a defect between the succinate dehydrogenase flavoprotein and coenzyme Q, possibly in the region of nonheme iron protein.
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