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Biomedical subjects

D Tenenbaum

Publications and source records attributed to D Tenenbaum.

At least 19 recordsLinked to original sources
PubMed1988

Alterations of serum high-density lipoproteins and hepatic lipase activity in congenital hypothyroidism.

Fourteen term newborns and infants with congenital hypothyroidism were studied before (T0) and after (T1) 1 month of replacement therapy. From T0 to T1 serum total cholesterol and high-density-lipoprotein cholesterol remained constant and at normal levels whereas low-density-lipoprotein apolipoprotein B and triacyglycerol concentrations increased slightly. The proportion of large-size high-density-lipoprotein subfraction was high at T0 and decreased very significantly at T1. Conversely, postheparin serum hepatic lipase activity was low before treatment and increased after thyroxine therapy. Lipoprotein lipase activity remained low throughout the study. These results suggest that, in contrast to adult hypothyroidism, low-density-lipoprotein catabolism is not altered in congenital hypothyroidism. However, as in adults, a defect of lipolytic enzyme activities is present and can induce an impairment of the elimination of cholesterol via the high-density-lipoprotein route and, potentially, of the catabolism of triglyceride-rich lipoproteins. Beyond the scope of congenital hypothyroidism, one should keep in mind these results when attending infants with transient low thyroid hormone levels and lipid intolerance.

Congenital Hypothyroidism↗
PubMed1988

[Neurophysiological study of peripheral nerves in newborn infants with congenital hypothyroidism. Value in the surveillance of replacement therapy].

The authors studied prospectively with electrophysiological means, peripheral nerve maturation of 18 neonates with congenital hypothyroidism compared to 18 controls. Before replacement therapy, a large reduction of sural nerve action potential amplitude and of Hoffmann's reflex was observed. It was accompanied by a moderate reduction in nerve conduction velocity. These results imply a myelinic and even more an axonal pathology. It is related to the anatomical type of hypothyroidism and to serum T4 deficiency. It is transient and disappears after 6 months of hormonal replacement therapy. In conclusion, this study gives new informations on the consequences of congenital hypothyroidism on peripheral nerves.

Congenital Hypothyroidism↗
PubMed1987

Hepatic response to insulin in control of glucose kinetics in the neonatal lamb.

Imprecise control of neonatal glucose homeostasis may be partially due to decreased hepatic response to insulin. In prior kinetic studies the effect of that response could not be determined because the hormonal effects of insulin could not be separated from those of glucagon. Somatostatin (SRIF) suppresses secretion of both and has been used to differentiate the hormonal effects. Eighteen term lambs (age 4.2 +/- 0.3 days and birth weight 4.0 +/- 0.2 kg (M +/- SEM] were infused with 0.9% NaCl at 0.06 mL.kg-1 min-1 plus 100 microCi/kg D[6-3H] glucose by prime plus constant infusion. Ra (production) and Rd (utilization) were measured during infusion of SRIF or SRIF plus replacement insulin (0.2 mU.kg-1 min-1). There was a rise in pl. glucose (98 +/- 10 to 119 +/- 10 mg/dL (P less than .0001)); a fall (46.2%) in pl insulin [13 +/- 2 to 7 +/- 1 microU/mL (P less than .0004); a rise in Ra (7.8 +/- 1.5 to 13.2 +/- 4.1 mg.kg-1 min-1 P less than .047); and a rise in Rd (7.7 +/- 1.4 to 11.3 +/- 3.0 mg.kg-1 min-1 (P less than .047)) in SRIF treated animals compared to nontreated controls. There was no change in plasma glucagon (454 +/- 182 to 255 +/- 141 pg/mL) in SRIF treated animals compared to nontreated controls. All perturbations were eliminated when SRIF plus replacement insulin produced control insulin levels. Insulin suppression in the neonatal period resulted in glucagon being unopposed which produced an elevated rate of glucose production and elevated plasma glucose concentration.

Animals↗
PubMed1987

[Treatment of citrullinemia. Apropos of a case followed from birth. Importance of alpha-ketonic acids].

From day 1 to day 3, the protein intake of this neonate was restricted to 1 g/kg/d. It included a) essential amino acids (i.e. histidine, lysine, threonine, tryptophan), b) arginine (1,000 mg/d), c) alphaketoisovaleric 500 mg/d, alpha-ketoisocaproic (500 mg/d), alphaketobetamethylvaleric (500 mg/d), alphaketogammamethylthiobutyric (200 mg/d), betaphenylpyruvic (400 mg/d) acids. 250 mg/kg/d of sodium benzoate were given. Caloric and water intakes were 120 cal/kg/d and 120 ml/kg/d respectively. Afterwards, this procedure was modified according to clinical and biological data including serum ammonia and amino acid levels. Alpha-ketonic acid absorption and metabolism were studied on day 29. Both were fast. The detection of alloisoleucine, which is not metabolized was the consequence of the use of alphaketobetamethylvaleric acid. Until the age of 21 months, clinical and metabolic status was satisfactory. At this time, repeated seizures without metabolic failure were accompanied by psychomotor damages.

Amino Acid Metabolism, Inborn Errors↗
PubMed1987

[Second prenatal diagnosis in a familial form of male pseudohermaphroditism caused by 17 keto-reductase deficiency: prediction confirmed by a normal third male infant].

In the first child of this family, the diagnosis of male pseudo-hermaphroditism due to 17 keto-reductase deficiency was established at two months of age after HCG test. During the second pregnancy, amniocentesis was performed for fetal karyotype and steroid determination in the amniotic fluid: an affected male fetus was suspected and this prediction was confirmed at birth. For the third pregnancy, a prenatal diagnosis was requested again and made, according to the same procedure: a normal male fetus was predicted and this diagnosis was confirmed at birth; this study demonstrates the feasibility and reliability of a prenatal diagnosis for 17 keto-reductase deficiency.

17-Hydroxysteroid Dehydrogenases↗
PubMed1987

[Gastric teratoma disclosed by neonatal digestive hemorrhage].

A gastric teratoma diagnosed after a gastro-intestinal tract bleeding in a neonate is reported. The endogastric tumor was shown by gastric endoscopy. The tumor was pediculated and a simple tumorectomy was performed, without trouble later. Fifty-three other cases have been found in the literature. Most of them presented with abdominal distension and a palpable mass; diagnosis was always made after surgery and the diagnosis of mature gastric teratoma was confirmed by histological examination. These rare tumors are always of benign nature, but are often revealed by complications. Their frequency is less than 1% of infants teratoma and 85% are found in the first year of life; they are more frequent in males.

Gastrointestinal Hemorrhage↗
PubMed1987

[An XX male newborn infant. A genetic and endocrinologic study].

The second child of a non consanguineous couple had a male phenotype with two intrascrotal testes of normal size however a scrotum bifidum was noted. The karyotype of the child was 46 XX and the parents one's was normal. No Y specific sequence was detected by using four Y specific probes (47 B, 12 F3, 52 D and 118). During the first semester of life, hormonal investigations showed a normal testicular function.

Androgens↗
PubMed1986

[Post-crisis elevation of adrenocorticotropic hormone and prolactin in epileptic children].

In six children, aged from 8 to 12 years, presenting with primary generalized epilepsy a significant rise in plasma ACTH and prolactin levels (as compared with levels measured 5 days later) was observed either during a generalized seizure demonstrated by EEG (3 cases) or within 1 hour of a generalized seizure (3 cases). The neurophysiological basis for these post-epileptic endocrine disturbances is a sudden depression of dopaminergic and GABA-ergic pathways. As a result, these endocrine changes, already well documented in adults, may be regarded as a good biological marker of the epileptic origin of a paroxysmal attack.

Adrenocorticotropic Hormone↗
PubMed1986

Comparison of morphologic features and mitotic rate to cytometrically determined DNA content of poorly differentiated lymphocytic lymphomas.

A direct correlation between the percentage of cells in S phase of the cell cycle and the clinical behavior of lymphocytic lymphomas of low, intermediate, and high grade malignancy has been described. The histopathologist has used the mitotic rate and other morphologic criteria such as size of cells and nuclear characteristics as predictors/indicators of the aggressiveness of a tumor. We compared the S phase values of 22 cases of poorly differentiated lymphocytic lymphoma (PDL) of the B cell type, using flow cytometric measurement of DNA content, to morphologic features and mitotic rate (MR). The 22 cases were divided into 3 histologic groups: nodular PDL composed of small, cleaved lymphocytes (11 cases); follicular mantle zone lymphoma and those of intermediate differentiation (6 cases); and "blastic" PDL (5 cases). In Group 1 there was excellent correlation of MR, percentage of cells in S phase, and proportion of large cells (transformed lymphocytes) per high power field (HPF). In Group 2, this correlation was not found between MR and percentage of cells in S phase in five of the six cases. The high S phase in this group did correlate with the large proportion of large cells found primarily in pseudofollicular proliferation centers and in remnants of true follicular centers. These cells may have a prolonged S phase and thus fewer mitoses were seen. In Group 3, although both MR and S phases were high, a direct correlation between them as noted in the Group 1 cases was not seen, but an excellent correlation of the high S phase and the number of blasts was present. The fact that three of the five patients in this group died rapidly (within less than 2 years of presentation) and the two survivors were experiencing rapid progression of disease, supports the concept that this group represents a clearly different, more aggressive subclass of PDL.

DNA↗
PubMed1986

[Gestational bone age of newborn infants].

A method for bone age calculation for neonates is reported. Criteria for maturation and growth are separately analysed. The chosen X ray charts were thorax including mandibula and profile of the right leg. 126 neonates 28 to 41 weeks of gestational age were studied. A linear regression is given. An area of confidence for growth and maturation for each gestational age has been designed. When gestational age is known, it gives the opportunity to appreciate the impact of a given pathology on these 2 data.

Age Determination by Skeleton↗
PubMed1985

Mechanisms of beta sympathomimetic action on neonatal glucose homeostasis in the lamb.

Beta sympathomimetic drugs are used clinically to inhibit premature labor. Significant alterations in glucose homeostasis have been documented in both mother and offspring. To determine the mechanism(s) of beta sympathomimetic drugs on neonatal glucose homeostasis, ritodrine hydrochloride was infused in a newborn term lamb model of glucose kinetics by a crossover design. There was a progressive rise in plasma glucose concentration and plasma insulin concentration during ritodrine hydrochloride infusion compared with the control (saline) infusion. The rise in plasma glucose concentration was associated with a progressive rise in Ra (rate of appearance, production) and Rd (rate of disappearance, utilization). The ritodrine hydrochloride-infused animals had a progressive rise in [insulin/glucose] compared with our prior studies in the newborn lamb, suggesting direct stimulation of pancreatic beta cell secretion. In the neonatal lamb, beta sympathomimetic drugs directly stimulate both hepatic glucose production and pancreatic beta cell secretion. These effects explain, in part, why infants of mothers infused with ritodrine hydrochloride may evidence alterations in neonatal glucose homeostasis.

Adrenergic beta-Agonists↗
PubMed1985

[Prenatal diagnosis in a familial form of male pseudohermaphroditism due to 17-keto reductase deficiency].

In an infant considered at birth as a female but with easily palpable gonads in the labia major, the XY karyotype and the endocrine studies (determination of plasma levels of steroid hormones under basal conditions and during hCG stimulation) were consistent with the diagnosis of male pseudohermaphroditism due to 17-keto reductase deficiency. During the second pregnancy an amniocentesis revealed a 46 XY karyotype. Endocrine studies performed on the amniotic fluid at midgestation suggested that the fetus was affected by the same enzyme defect. After birth, the diagnosis was demonstrated with anatomical an endocrine studies.

17-Hydroxysteroid Dehydrogenases↗