Biomedical subjects
Publications and source records attributed to R M Hurley.
A child aged 24 months had multiple congenital abnormalities and delayed development. The 28-year-old mother had been treated since childhood with anticonvulsants. Her previous pregnancies had resulted in three early spontaneous abortions and one child with severe bilateral cleft lip and palate. This case report further suggests a relationship between maternal diphenylhydantoin use and fetal anomalies.
In nine children with the Laurence-Moon-Biedl syndrome a high incidence of nephropathy was documented. Renal involvement was noted in seven patients before or simultaneously with the diagnosis of LMBS. Five have uremia and two have died. All patients had an abnormal urogrum. The spectrum of renal lesions ranged from mesangial tissue proliferation to glomerular clerosis, interstitial scarring, and medullary and cortical cyst formation. A relationship between the lesions seen in the LMBS and those of other hereditary retinal-renal conditions is suggested.
Four of 10 children with SLE kept under observation over the past nine years have developed avascular necrosis (AN) of the femoral head. The symptoms of AN are insidious and unpredictable and predate the radiologic diagnosis by weeks to months. In a comparison of these children with SLE, with and without AN, with a group of patients with nephrosis treated with corticosteroids and a group with glomerulonephritis treated with azathioprine, AN was related to the duration of daily steroid therapy rather than the total duration of steroid treatment; this was not true for azathioprine. The occurrence of AN in our patients while they were on alternate-day steroid therapy, or coincident with a relapse, suggests that its development is determined by underlying disease.
The glomerular filtration rate (GFR) was estimated using (125)I-iothalamate by the cumulative integral method in 95 subjects - 16 normal subjects and 79 patients with suspected or proved renal disease. This method is accurate, simple to perform and theoretically sound. It minimizes errors due to transit time and bladder storage. The normal range of GFR was determined to be 71 +/- 10 (two standard deviations) ml/min/m(2). In 66 patients serum creatinine levels were compared with the GFR value expressed per square metre. For any given serum creatinine level there is a wide range of GFR values. A serum creatinine value in the normal range, i.e. between 0.7 and 1.3 mg/dl, cannot be used to predict the patient's GFR. The micro blood sampling technique makes this method particularly useful in infants.
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1. The average mechanical properties of groups of lungs or lung-thorax systems from pathogen-free rats weighing approximately 200 g were determined. Static pressure-volume curves and resistances to air-flow were obtained.2. Six series, each of sixteen rats, were studied. Eight experimental rats in each series were exposed to 4 atm O(2) (OHP) in a transparent pressure chamber; the other eight rats, which served as controls, were obtained from the breeder at the same time and studied at the same time.3. In four series, the experimental animals were killed 10 min after gasping due to OHP had been definitely established. One series was a control in which experimental animals were exposed to 4 atm of pressure in an atmosphere containing oxygen at a tension of 150 mm Hg for 190 min. The experimental animals in the sixth series were exposed to 4 atm O(2) for 2 hr and none of them gasped.4. Gross and histological examination of sixteen rats, eight of which were killed after 10 min of gasping at 4 atm O(2), showed that at this stage of intoxication there was no evidence of pulmonary pathology.5. In none of the series studied were the static pressure-volume curves for deflation shifted, i.e. OHP did not affect the elastic properties of the lungs or the alveolar surfactant.6. In two series studied 10 min after gasping behaviour had been established there was a significant decrease of resistance to air-flow and a shift to the left of the static pressure-volume curve for inflation with air. The rats in both these series were sedated with pentobarbitone and then killed with pentobarbitone injected into the jugular vein.7. The decrease in resistance to air-flow was interpreted as broncho-dilatation and a possible mechanism whereby OHP produces broncho-dilatation is discussed.
The purpose of this investigation was to study further the assertion that auditory function deteriorates in the unaided ear of individuals with sensorineural hearing loss (SNHL) who receive monaural hearing aid fittings. The word recognition scores (WRSs) of 77 monaurally and 65 binaurally fitted subjects with symmetric bilateral SNHL were examined at 1, 3, and 5 years post hearing aid fitting. The deterioration in auditory function was defined as a significant change in the WRSs for Northwestern University Auditory Test No. 6 (NU-6) materials in the unaided ear of individuals fitted with a monaural hearing aid arrangement. Analyses of the data indicate that 25 percent of the monaurally fitted subjects experienced a significant change in the WRSs of their unaided ears, whereas only 6 percent of the binaurally fitted subjects experienced a significant change in the WRSs of either ear. Auditory function does deteriorate in the unaided ears of individuals with SNHL who receive monaural hearing aid fittings. Moreover, the decline in auditory function of the unaided ear does not result from a decrease in hearing sensitivity.
Cord blood thyrotropin (TSH) screening for congenital primary hypothyroidism has been in effect on the island of St. Lucia for the past three years. Umbilical cord blood samples are obtained on Guthrie filter paper and then transported 3,000 miles to Loyola University of Chicago and delivered to the Illinois State Metabolic Screening Laboratory. There TSH is measured by radioimmunoassay (RIA). After three years, 1,789 newborns have been screened, and the mean value is 6.23 +/- 0.13 microIU per ml. This mean value is less than previously reported by us in 1986 (10.23 +/- 0.29 microIU per ml).13 It is concluded that this screening service continues to be possible far removed from the population under observation. No case of primary hypothyroidism has been detected. Our decreased mean TSH value is due to the new method currently used by the Illinois State Metabolic Screening Laboratory. Congenital hypothyroidism will not be missed provided internal controls are established and rigidly observed.
The role of endotoxin in newborn septic shock is reviewed. Metabolic and cardiovascular changes, as known to us, are described with special emphasis that the newborn is not a "small adult." Developmental or maturational changes are hypothesized to be the major cause of these differences between age groups. Finally, it is our belief that newborn septic shock is an important topic for further investigation.
Gram negative sepsis is a leading cause of human newborn morbidity and mortality. The clinical signs and glucose and lactate concentration during the late phase of newborn rat endotoxicosis has not been well characterized. In order to define a late phase model of sepsis, simultaneous clinical signs (loss of response to pain [RP] and/or righting reflex [RR]), and metabolic (glucose, lactate) concentrations were studied in the 10 day old Sprague-Dawley rat. The rats were fasted for four hours and then injected with either saline (control) or 0.1 mg per kg Salmonella enteritidis (LD90 @ 24) endotoxin intraperitoneally. Rats were then examined every 15 minutes beginning two hours post injection for the presence or absence of loss of RR and/or loss of RP. Central blood samples were collected for determination of glucose and lactate at the moment of loss of the predetermined clinical parameter. Metabolic parameters were also determined on saline treated controls time matched to the experimental groups. Rats were grouped as follows: GrI, (n = 10), saline three hours post injection; GrIII, (n = 10), saline five hours post injection; GrII, (n = 45), endotoxin at the moment of loss of RR; GrVI, (n = 14), endotoxin at 8.5 hrs post injection (termination of experiment, all with loss of RR); GrIV, (n = 11) endotoxin at loss of RP; and GrV, (n = 10) saline at 8.5 hr post injection.(ABSTRACT TRUNCATED AT 250 WORDS)
Blood was drawn from 10 fasted, healthy volunteers and stored under standard blood bank conditions in citrate-phosphate-dextrose-adenine (CPDA-1). Blood was sampled before storage (Day 0) and on Days 5, 12, 19, 26, and 35. Laboratory testing for glucose, HbAla + b, HbAlc, pyruvic acid, lactic acid, adenosine triphosphate (ATP), 2, 3-diphosphoglycerate (2,3DPG), plasma free hemoglobin (Hb) and pH (blood gases) were performed. In addition, P50 was also serially measured in two of the individuals and in their stored blood. Significant elevations of HbAla + b and HbAlc (fast hemoglobins) were found on Days 12 and 19 of storage (p less than 0.05). These elevations of fast hemoglobins are due to hypoxia, acidosis, and hyperglycemia. Following the initial elevation of the fast hemoglobins (Hbs), there was a decline in their concentration, from Day 12, which could partly be explained by cell death.