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Biomedical subjects

K C Kuban

Publications and source records attributed to K C Kuban.

33 records · Page 2Linked to original sources

Septo-optic-dysplasia-schizencephaly. Radiographic and clinical features.

The anatomical anomalies that characterize septo-optic-dysplasia (deMorsier Syndrome) are variable and often subtle. We report imaging studies of nine patients with septo-optic-dysplasia which provide radiologic and ultrasonographic clues to this disorder. In addition, we propose that cerebral schizencephaly may be a component of the dysmorphogenesis that results in septo-optic-dysplasia. Septo-optic-dysplasia-schizencephaly complex is frequently associated with endocrinologic, ophthalmologic, and neurologic symptoms and signs.

Adolescent↗

Maternal toxemia and neonatal germinal matrix hemorrhage in intubated infants less than 1751 g.

Two hundred seventy-two intubated infants who weighed less than 1751 g were enrolled in a clinical trial of phenobarbital prophylaxis of postnatal germinal matrix hemorrhage. The incidence of germinal matrix hemorrhage was 3.1% (one of 32) among infants born to women with toxemia, and 23% (55 of 240) among those born to women without toxemia. The apparent protective effect of toxemia could not be explained by intrauterine growth retardation, mode of delivery, or maternal receipt of any medication. Infants born to toxemic women were less likely than their peers to develop pneumothorax, become acidotic, and to require extensive respiratory assistance. This apparently protective effect of maternal toxemia was not seen in infants born to nontoxemic, hypertensive women. Thus, maternal toxemia, but not hypertension, might reduce the risk of germinal matrix hemorrhage by reducing the occurrence and/or severity of pulmonary and related problems that place infants at high risk of germinal matrix hemorrhage.

Cerebral Hemorrhage↗

Hemorrhage, phenobarbital, and fluctuating cerebral blood flow velocity in the neonate.

Fifty-one sequential intubated babies with birth weights of less than 1,751 were evaluated by serial Doppler ultrasound during the first three days of life. These babies were part of a phenobarbital prophylaxis trial cohort study. Subependymal-intraventricular hemorrhage developed in 17 of the babies. Infants with subependymal-intraventricular hemorrhage, whether or not they received pancuronium or phenobarbital, had coefficients of variation comparable to those of babies without hemorrhage. Coefficient of variation values of the right were comparable to values obtained from the left anterior cerebral artery complex and did not appear to be consistently altered by the presence of subependymal-intraventricular hemorrhage. Coefficient of variation values appeared to be consistently greatest on day 1 and lowest on day 2. In addition, the values overall increased as the number of waves used to determine the coefficient of variation enlarged from five to 20. This phenomena, however, was not seen among pancuronium recipients and suggests that movement artifact may be a determinant of coefficient of variation values. We conclude that, when the best 20 waves are chosen to evaluate the coefficient of variation, no association exists between coefficient of variation values and development of subependymal-intraventricular hemorrhage or administration of phenobarbital.

Blood Flow Velocity↗

Arterial blood gas derangements associated with death and intracranial hemorrhage in premature babies.

We evaluated to what extent acidosis and alkalosis and their respiratory and metabolic components during the first 12 hours of life occurred prior to early neonatal death and postnatal intracranial hemorrhage among 206 low birth weight, intubated premature babies participating in a clinical trial of phenobarbital prophylaxis for intracranial hemorrhage. Time-weighted indices included the time each baby spent with abnormal values of pH, PaCO2 and HCO3-. Babies whose birth weight was less than 1 kg suffered adversities associated with prolonged pH less than 7.35. Heavier birth weight babies were at increased risk of adversity if their pH fell below 7.2. Babies who were not severely acidotic initially, but became so within hours, were at prominently increased risk of death and hemorrhage. Babies who had a mild increase of PaCO2 between 45 and 60 mmHg were less likely to develop germinal matrix hemorrhage than their peers who had more severe hypercapnia. A time-weighted measure of metabolic deficit correlated with death, but not with hemorrhage. Prolonged exposure to pH greater than 7.55 was associated with reduced risk of subependymal/intraventricular hemorrhage and death, especially in babies below 1 kg birth weight. We conclude that acidosis is an antecedent of intracranial hemorrhage in low birth weight premature babies, that duration of exposure might convey important risk information, and that birth weight is a correlate of vulnerability to some pH disturbances.

Acidosis↗

Bilirubin, intraventricular hemorrhage, and phenobarbital in very low birth weight babies.

The relationships among serum bilirubin concentration on days 5 and 7, birth weight, the presence of intraventricular hemorrhage, and the receipt of phenobarbital were examined in a group of 232 newborns weighing less than 1,751 g who were intubated, mechanically ventilated by 12 hours after birth, and whose parents had given permission for a randomized trial of phenobarbital prophylaxis of intraventricular hemorrhage. The ratio of serum bilirubin concentration to birth weight (the bilirubin divided by birth weight index [BBI]) was used to examine the impact of 25 variables on a clinical guideline for therapy of hyperbilirubinemia in newborn infants. A linear regression model was used; the most powerful covariate was a birth weight less than 1.0 kg. The only other variable that reduced the BBI was phenobarbital receipt. The presence of intraventricular hemorrhage and ecchymoses had a significant influence increasing the BBI.

Bilirubin↗

Respiratory complications in low-birth-weight infants who received phenobarbital.

We compared the ventilatory requirements of 127 infants who received phenobarbital for five days with those of 111 infants who received placebo. All infants were intubated, weighted less than 1750 g at birth, and survived the first ten days of life. Those infants who received phenobarbital did not require ventilatory assistance for more days than did placebo receivers. However, a pneumothorax or pulmonary interstitial emphysema was more likely to develop in infants who received phenobarbital than in infants who received placebo, even when adjustment was made for the presence of subependymal-intraventricular hemorrhage. We believe this is the first report of this relationship and recommend additional studies to test the hypothesis that phenobarbital contributes to the occurrence/recognition of pneumothorax or pulmonary interstitial emphysema in very-low-birth-weight infants if phenobarbital continues to be used routinely as prophylaxis or treatment.

Cerebral Hemorrhage↗

The prophylactic clinical trial as a epidemiologic resource.

We encourage investigators to use data collected in a clinical trial of a prophylactic agent or procedure to study the epidemiology of the disease or event the prophylactic was meant to prevent. Making additional use of previously collected data is economically attractive. Problems can arise, however, if the sample is not representative of the universe of all people at risk of the disorder, the data set is not adequate to the task, or the prophylactic and/or its correlates influence the risk of the disorder. Investigators should consider modifying data collection procedures in future prophylactic trials so that they are suitable for an epidemiologic study. They and readers of their reports, however, are advised to be cautious in drawing inferences from epidemiologic studies extracted from prophylactic trials.

Clinical Trials as Topic↗

Schilder's disease: additional aspects and a therapeutic option.

An 8-year-old boy presented with large diffuse myelinoclastic bifrontal and right parietal peripherally enhancing white matter lesions with high intracranial pressure. Intravenous treatment with ACTH and high dose cyclophosphamide (cytoxin) resulted in shrinkage of the lesion, loss of peripheral enhancement and clear clinical improvement. After nine months following the onset of the first episode, an isolated left frontal enhancing lesion appeared along with a new right hemiparesis and speech difficulty. The same therapy again resulted in prompt definitive improvement of the CT scan and reversal of the clinical course. This case was extensively investigated and characterized as the 1912 variety of Schilder's disease, to be distinguished from post-infectious encephalopathy and other forms of white matter disease such as adrenoleukodystrophy. No elevation of CSF IgG, oligoclonal bands, or characteristic abnormality of T-cell subset distribution were identified. However, the pathological picture most closely resembled that of multiple sclerosis. A striking feature of this child's clinical history was the relatively rapid clinical improvement with immunosuppression.

Brain Diseases↗

Neonatal intracranial hemorrhage and phenobarbital.

We enrolled 280 intubated babies with birth weights of less than 1,751 g in a double-blind randomized prospective clinical trial to evaluate whether phenobarbital influences the likelihood of developing subependymal-intraventricular-intraparenchymal hemorrhage. Phenobarbital was associated with an increased risk of developing any subependymal-intraventricular-intraparenchymal hemorrhage and was not associated with a diminished risk of either severe hemorrhage or germinal matrix hemorrhage. This increased risk was apparent even after we considered the influence of phenobarbital levels, timing of phenobarbital administrations, institutional differences, quality of ultrasound scans, gestational age- and birth weight-specific effects, ascertainment bias, and other possible confounders of phenobarbital administration.

Cerebral Hemorrhage↗

Human telencephalic angiogenesis.

We provide new observations regarding the histogenesis and regional development of the human telencephalic microvasculature during the last half of gestation. Endothelium-lined trunks extending from pia to the subventricular plexus, evident early in gestation, persist during the last half of gestation. The courses of these trunks are modified and become complex as the bulk of the telencephalon, striatum, and thalamus increases and as gyri grow. New cerebral tissue is supplied by increasing numbers of shorter penetrating vessels. All extrastriatal vessels have many lateral right- and acute-angle branches that join nearby trunks and shorter vessels. Striatal vessel branches predominantly have acute angles. Most extrastriatal channels remain devoid of apparent muscularis until the final weeks of gestation. In contrast, striatal arteries begin to muscularize at about 24 weeks of gestation. Muscularization appears to occur in a centripetal direction and is apparent in the caudate at approximately 30 weeks' gestation. We did not identify transventricular, paraventricular, or recurrent arteries ending in deep white matter.

Arachnoid↗

Syndrome of opsoclonus-myoclonus caused by Coxsackie B3 infection.

Coxsackie B3 was cultured from 2 children who presented within two weeks of each other from the Cape Cod, Massachusetts, area showing opsoclonus and myoclonus. The organism was recovered from the cerebrospinal fluid of 1 patient and from the stools of both. Both children had cerebrospinal fluid pleocytosis and gradual, spontaneous resolution of their symptoms.

Cerebrospinal Fluid↗

Deep tendon reflexes in premature infants.

Ten classic deep tendon reflexes (DTRs) were evaluated in 62 premature infants of greater than 27 weeks post-conceptional age. The pectoralis major was the most readily elicitable reflex in all infants (100%), regardless of maturity. Achilles, patellar, biceps, thigh adductors, and brachioradialis reflexes also were obtained in at least 98% of babies of greater than 33 weeks gestation. Among these reflexes, less mature infants (less than 33 weeks gestation) had decreased elicitation rates for patellar and biceps reflexes and overall had diminished reflex intensity when compared to older infants (33-36 weeks gestation). By order of decreasing rate, finger flexors, jaw, crossed adductors, and triceps reflexes were less frequently elicited in both groups. Equal DTRs were obtained often in healthy and previously ill infants of less than 33 weeks gestation. Head position had no apparent affect on the ability to elicit reflexes. Theophylline therapy tended to intensify the Achilles reflex and the quiet, wakeful state appeared to be the most optimal state for the elicitation of DTRs.

Gestational Age↗

Etiologic heterogeneity of intracranial hemorrhages in preterm newborns.

Hemorrhages in brain parenchyma and ventricles in preterm infants tend to be grouped as one entity. To help determine whether these hemorrhages should be viewed as one or more entities, we compared the risk profiles of 3 groups of hemorrhages defined by location and time of occurrence: early germinal matrix hemorrhage (GMH), late GMH, and late peri/intraventricular hemorrhage excluding GMH. These 3 groups were determined to have sufficient differences in various risk factors to warrant separate epidemiologic study.

Anesthesia, Spinal↗