Surgical management of severe coarctation of the aorta in the first month of life.

Between March, 1978, and April, 1982, 48 neonates under 4 weeks of age were operated upon for severe coarctation of the aorta in our unit. Of these, 36 babies were in severe congestive cardiac failure and 11 had renal failure. Angiocardiography and echocardiography demonstrated intracardiac lesions in 29 patients.

Effects of an optokinetic background on pursuit eye movements.

The effects of an optokinetic background on pursuit eye movements was studied in four normal human subjects and seven patients with impaired pursuit and/or optokinetic nystagmus (OKN). Eye movements were recorded by DC electro-oculography and eye movement velocity was analyzed by a digital, microprocessor system. Tracking of a small laser target was performed against a featureless, white screen, a stationary OKN background that filled the entire visual field and an OKN background moving at constant velocity.

Effect of eccentric gaze on pursuit.

The effect of eccentric gaze on pursuit eye movements was studied with electro-oculography in normal human subjects and a patient with defective pursuit. Sinusoidal target trajectories were centered about the primary position of gaze and eccentric, horizontal positions of gaze. Eccentric gaze decreased pursuit gain (eye velocity/target velocity) when the eyes moved farther eccentrically in the orbits and increased pursuit gain when the eyes moved toward the centers of the orbits.

Fatal infantile mitochondrial myopathy due to cytochrome c oxidase deficiency.

A case of cytochrome c oxidase deficiency primarily affecting skeletal muscle is described. The child was admitted at 4 weeks due to failure to thrive and examination at that time revealed weakness and hypotonia. His condition deteriorated until at 11 weeks respiratory arrest necessitated artificial ventilation and death occurred at 14 weeks.

The cytochrome b and flavin content and properties of the O2- -forming NADPH oxidase solubilized from activated neutrophils.

NADPH-dependent O2- -generating activity was extracted and partially purified from guinea pig polymorphonuclear leukocytes. The most active preparation generated 202.8 nmol O2- min/min per mg protein.

Binding of dansyl propranolol to the (Ca2+ + Mg2+)-ATPase.

We have studied the binding of dansyl propranolol to lipid bilayers and to the (Ca2+ + Mg2+)-ATPase of sarcoplasmic reticulum. The fluorescence emission spectra for dansyl propranolol bound to the ATPase system can be fitted to the sum of three peaks, characteristic of probe bound to lipid and to protein and free in solution, respectively. Titrations show that binding to the lipid component of the ATPase system is comparable to binding to simple lipid bilayers.

Changes in the cytochrome composition of Rhodopseudomonas sphaeroides grown aerobically, photosynthetically and on dimethyl sulphoxide.

Several strains and mutants of Rhodopseudomonas sphaeroides can be grown anaerobically in the dark in the presence of dimethyl sulphoxide as an electron acceptor. During adaptation to this fermentative mode of growth, two major c-type cytochromes are synthesized, one with Mr 45 000 and the second with Mr 20 000 and a midpoint potential of +120 mV. These cytochromes are barely detectable in membranes prepared from cells grown in aerobic or photosynthetic conditions.

A variant form of X-linked chronic granulomatous disease with normal nitroblue tetrazolium slide test and cytochrome b.

Chronic granulomatous disease was diagnosed in a boy who suffered from severe generalized infections. Family investigations revealed the inheritance of the disease to be X-linked. However, unlike other cases of X-linked chronic granulomatous disease, the membrane oxidase of the neutrophils from this patient was not totally defective and sufficient activity was left to result in a normal phorbol myristate acetate-stimulated nitroblue tetrazolium slide test.

Internal jugular catheterisation in small children. The use of a posterior approach.

Internal jugular catheterisation, using a posterior approach, was attempted in 50 infants and children aged from 1 day to 12 years, weighing 1.25 to 30 kg. Thirty-five of the patients weighed less than 10 kg.

Gonorrhea in the United States: 1967-1979.

Sex-, race-, and age-specific gonorrhea cases and rates reported by public and private sources were studied. The role of the age, sex, and race composition of the population was used to explain changing trends of morbidity due to gonorrhea. Beginning in 1967, reported cases of gonorrhea in the United States increased at an annual rate of 13% through 1975, with increases in women twice those in men.

Absence of cytochrome b-245 in chronic granulomatous disease. A multicenter European evaluation of its incidence and relevance.

The heme-containing protein cytochrome b-245 has been proposed as a primary component of the microbicidal oxidase system of phagocytes that normally generates superoxide-free radicals but when defective is associated with chronic granulomatous disease. We measured this cytochrome in granulocytes from 27 patients with chronic granulomatous disease and from 64 members of their families. It was undetectable in all 19 of the men in whom the defect appeared to be located on the X chromosome.

Annular and non-annular binding sites on the (Ca2+ + Mg2+)-ATPase.

Quenching of the fluorescence of the (Ca2+ + Mg2+)-ATPase purified from muscle sarcoplasmic reticulum can be used to measure relative binding constants of hydrophobic compounds to the phospholipid-protein interface. We show that the binding constant for cholesterol is considerably less than that for phosphatidylcholine, so that cholesterol is effectively excluded from the phospholipid annulus around the ATPase. However, dibromocholestan-3 beta-ol causes quenching of the fluorescence of the ATPase, and so has access to other, non-annular sites.

The association of FAD with the cytochrome b-245 of human neutrophils.

A plasma membrane fraction prepared from human neutrophils had a fluorescence resembling that of a fluorescent flavoprotein, with emission maximum near 520nm and excitation maxima near 380 and 460nm. The fluorescence emission and excitation properties of Triton N-101-solubilized membrane fraction resembled those of FAD. FAD was present in the membranes at a concentration of 417pmol/mg of protein and cytochrome b(-245) at a concentration of 407pmol/mg of protein.

Interaction of fatty acids with the calcium-magnesium ion dependent adenosinetriphosphatase from sarcoplasmic reticulum.

The fluorescence emission spectrum of dansylundecanoic acid is sensitive to the environment and appears at a lower wavelength when the fatty acid is bound to protein than when it is bound to phospholipid. When bound to the (Ca2+-Mg2+)-ATPase of sarcoplasmic reticulum, the emission spectrum can be resolved into separate components assigned to fatty acid bound to protein and to lipid. Efficiency of energy transfer from the tryptophan residues of the ATPase to dansylundecanoic is higher for protein-bound probe than for lipid-bound probe.

Development of cytochrome b and an active oxidase system in association with maturation of a human promyelocytic (HL-60) cell line.

The human HL-60 myeloid leukaemia cell line developed, during maturational changes induced by dimethyl sulphoxide, an enhanced capacity for phorbol myristate acetate- stimulated oxidative activity and acquired a cytochrome b. Titration of the absorbance at 559 nm at potentials of-190 to -370 mV indicated that this cytochrome had a very low potential, differentiating it from mitochondrial and endoplasmic reticulum cytochromes and identifying it as the cytochrome b(-245) that has been recently found in other phagocytic cells. Subcellular fractionation studies of mature HL-60 cells showed that cytochrome b had a dual distribution within the cell.

The use of N-methylprotoporphyrin dimethyl ester to inhibit ferrochelatase in Rhodopseudomonas sphaeroides and its effect in promoting biosynthesis of magnesium tetrapyrroles.

N-Methylprotoporphyrin dimethyl ester inhibits ferrochelatase in isolated membranes of Rhodopseudomonas sphaeroides at low concentrations (around 10 nm). Full inhibition developed after a short lag phase. The inhibition was non-competitive with porphyrin substrate.

Human amniotic fluid cells grown in a hormone-supplemented medium: suitability for prenatal diagnosis.

A new supplemented medium has been developed to improve human amniotic fluid cell growth and to reduce the dependence on exogenously added serum. The medium consists of a mixture of Ham's F12 medium and Dulbecco's modified Eagle's medium supplemented with Hepes, antibiotics, and 10 growth-promoting factors at 4% fetal bovine serum. Good clonal growth is achieved consistently in 8--13 days and is associated with large numbers of metaphase cells.

The enzymic reduction and kinetics of oxidation of cytochrome b-245 of neutrophils.

1. The absorption coefficient of human neutrophil plasma-membrane reduced-minus-oxidized cytochrome b-245 was determined [delta epsilon (mM; 559-540 nm) = 21.6 cm-1].