0000000000074326

AUTHOR

W. Schönberger

Transient hypothyroidism associated with prematurity, sepsis, and respiratory distress.

Serial TSH and T4 determinations were performed in sixty neonates admitted to our hospital for neonatal intensive care within a period of three months. Seven patients (12%) showed transient hypothyroidism on the basis of low T4 and high TSH values. Only one of these patients, who had meconium aspiration and pneumonia, did not have the respiratory distress syndrome. In addition, 4 of these patients had sepsis. All of the patients were born before 37 gestational weeks and had birth weights under 2200 g. In addition, two patients of this gestational age and birth weight group had a progressive fall of T4 to extremely hypothyroid values without simultaneous elevation of TSH. Two of the 5 patien…

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Serial measurements of transient evoked otoacoustic emissions (TEOAEs) in healthy newborns and in newborns with perinatal infection.

Detection of hearing impairment in early childhood is difficult. We serially recorded transient evoked otoacoustic emissions (TEOAEs) to search for signs of ototoxicity in term, healthy newborns and compared the results to a second group of term babies treated for perinatally acquired bacterial infection with ampicillin plus either cefotaxime or plus aminoglycoside. At initial evaluation, in the group of 45 healthy children born at term, well reproducible emissions were observed in all but two children. In each of these two, initially well reproducible TEOAEs were detected in one ear only. At the time of the second recording (mean at day 8.5) excellent emissions were seen in all ears of all…

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Differenzierung zwischen prämaturer Thelarche und Pubertas praecox anhand klinischer, hormoneller und radiologischer Befunde

In a retrospective study of 39 girls (aged 10 months to 7 10/12 years) with premature breast development criteria for distinguishing between premature thelarche and precocious puberty were analysed. Serum estradiol levels and bone age were determined and a test with luteinizing hormone-releasing hormone (LHRH) performed (inclusion criteria). On the basis of the LHRH test and bone age, premature thelarche was diagnosed in 29 patients and precocious puberty in ten: while those with premature thelarche had a follicle-stimulating hormone (FSH) pattern of rise, in those with precocious puberty the rise in gonadotropin was of the LH type. The LH/FSH ratio 30 min after stimulation was 1 (median 2.…

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The effect of nacom (L-dopa and L-carbidopa) on growth hormone secretion in 75 patients with short stature.

The stimulatory effect of Nacom (250 mg L-Dopa and 25 mg L-Carbidopa) on the HGH secretion was evaluated in 75 short stature patients. The number of blood samples was restricted to only three (0, 45 and 90 min). 63 patients reached adequate HGH concentrations after the ingestion of 1 tablet Nacom (84%). Somatotropin levels increased from 2.08 (Sx 0.28) to a maximal HGH value of 14.22 (Sx 0.87) ng/ml. When the stimulatory effect of Nacom was compared with the standard method of arginine infusion in children with normal stature the arginine test was not superior to the Nacom-test. The Nacom-test appears to be a simple and reliable screening method for HGH deficiency, particularly in outpatien…

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Familial insulin resistant diabetes associated with acanthosis nigricans, polycystic ovaries, hypogonadism, pigmentary retinopathy, labyrinthine deafness, and mental retardation.

Two sibs, whose parents are first cousins, had diabetes mellitus with hyperinsulinism, insensitive insulin receptors, and acanthosis nigricans. Both patients had pigmentary retinopathy, secondary cataracts, labyrinthine deafness, mental retardation, and cerebral atrophy. They were disproportionately short with relatively broad hands and feet and slightly coarse face. The young woman had secondary amenorrhea and polycystic ovaries and the boy gynecomastia and hypergonadotrophic hypogonadism. This appears to be the second family with a new autosomal recessive disorder differing from Alstrom syndrome by the presence of mental retardation and absence of renal insufficiency. Impaired insulin rec…

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HGH secretion after oral application of l-Dopa and l-Carbiodopa

The stimulatory effect of L-Dopa and L-Carbidopa (Nacom) on HGH secretion was determined in 12 children of normal height aged from 6 to 14 years. Each child received a standard dose of 250 mg L-Dopa and 25 mg L-Carbidopa p.o. HGH concentration in the serum was determined at standard intervals. All subjects showed a sufficient increase of HGH. The mean value was 19.6 ng/ml. According to the maximum values of the HGH concentration the sample can be divided into two groups; the first group reached the highest values after 20--40 min, the second one after 60--90 min. On evaluation of the curve of the mean values it appears that 2 blood samples taken 40 and 90 min after the ingestion of L-Dopa a…

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Reduction of mortality rate in premature infants by substitution of thyroid hormones.

Our previous examinations had shown that 9 of 13 premature infants with severe respiratory distress had hypothyroid T4-values. On the basis of these results a prospective study was initiated. Every second neonate born after less than 37 weeks gestation or weighing less than 2200 g and admitted to our intensive care unit since Janary 1979 received a prophylactic dose of 25 μg l-Thyroxine and 5 μg Tri-iodothyronine daily. Five of the patients inadvertently did not receive the drug and were included in the non-treated group which thus numbered 55. Both groups were nearly identical with regard to gestational age, birth weight and Apgar score. In the treated group of 45 infants three (=6.6%) die…

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Hypoparathyreoidismus und Innenohrschwerhörigkeit

Es werden 3 Kinder mit nicht-familiarem Hypoparathyreoidismus beschrieben, die durch eine hochgradige Horstorung cochlearer Genese auffielen. Wahrend bei einem der Kinder klinisch und molekulargenetisch ein Kearns-Sayre-Syndrom nachgewiesen werden konnte, bleibt die Ursache des gemeinsamen Auftretens der Storungen bei 2 Kindern ungeklart. Die Assoziation scheint haufiger als bisher angenommen: sie betrifft 3 der in der Kinderklinik Mainz betreuten 9 Kinder mit primarem Hypoparathyreoidismus. Genetisch determinierte Storungen der Haarzellmotilitat der Cochlea sowie Haarzellschadigung als Folge chronisch verringerter Kalziumkonzentrationen in der Peri- und Endolymphe werden als pathogenetisch…

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