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Biomedical subjects

D Shulman

Publications and source records attributed to D Shulman.

At least 19 recordsLinked to original sources
PubMed2001

Congenital hyperreninemic hypoaldosteronism unlinked to the aldosterone synthase (CYP11B2) gene.

Isolated hyperreninemic hypoaldosteronism presenting in infancy is usually caused by mutations in the CYP11B2 gene encoding aldosterone synthase. We studied five patients in four unrelated kindreds with hyperreninemic hypoaldosteronism, in whom we were unable to find such mutations. All presented in infancy with failure to thrive, hyponatremia, hyperkalemia, markedly elevated plasma renin activity, and low or inappropriately normal aldosterone levels. All had normal cortisol levels and no signs or symptoms of congenital adrenal hyperplasia. All responded to fludrocortisone treatment. There were no mutations detected in exons or splice junctions of CYP11B2. Linkage of the disorder to CYP11B2 was studied in two unrelated consanguineous patients and in an affected sib pair. The consanguineous patients were each heterozygous for at least one of three polymorphic microsatellite markers near CYP11B2, excluding linkage to CYP11B2. However, linkage of the disease to CYP11B2 could not be excluded in the affected sib pair. Genes involved in the regulation of aldosterone biosynthesis, including those encoding angiotensinogen, angiotensin-converting enzyme, and the AT1 angiotensin II receptor were similarly excluded from linkage. These results demonstrate the existence of an inherited form of hyperreninemic hypoaldosteronism distinct from aldosterone synthase deficiency. The affected gene(s) remain to be determined.

Adult↗
PubMed1997

Demonstration of a leptin binding factor in human serum.

Serum leptin levels are elevated in subjects with exogenous obesity, indicating that obesity is associated with leptin resistance. Since in man no abnormalities have yet been found in either the genes for leptin or its receptor, the mechanism of leptin resistance in obesity remains unknown. To determine if resistance might be related to leptin binding by a serum component, we assessed the carrier status of leptin in serum. The presence of a specific leptin binding factor in human serum has been established by (1) demonstrating [125I]-leptin binding to a serum component that is saturable and specifically displaceable only by unlabeled leptin and not by human growth hormone, pork insulin, insulin-like growth factors I and II, luteinizing or follicle stimulating hormones, transforming growth factor-beta 1, interleukin-6, or leukemia inhibiting factor; (2) fractionating the leptin bound serum complex and the serum leptin binding component on a molecular sieving column revealing a mass of approximately 450 kDa; and (3) identifying an inverse correlation between the concentration of serum leptin and the quantity of the leptin binding component. It is suggested that binding of leptin by this serum component may influence the physiologic response to leptin.

Animals↗
PubMed1992

Non-nutritive sucking does not increase blood levels of gastrin, motilin, insulin and insulin-like growth factor 1 in premature infants receiving enteral feedings.

Non-nutritive sucking in premature infants accelerates weight gain for unclear reasons. The effects of non-nutritive sucking on enteral hormone secretion may augment digestion and/or absorption of nutrients. Blood concentrations of gastrin, motilin, insulin and insulin-like growth factor-1 were measured before and 72 h after the initiation of nasogastric feedings in 21 premature infants randomly assigned to either a non-nutritive suckling or control group. Gastrin and motilin concentrations increased significantly after feedings in all infants (mean +/- SEM) (gastrin, 41 +/- 4 to 73 +/- 9 pg/ml, p < 0.01; motilin, 141 +/- 5 to 181 +/- 3 pg/ml, p < 0.01) Pre- and post-feed insulin concentrations were greater in the non-nutritive sucking group receiving bolus feeds than in control infants who were bolus-fed (P < 0.01). Non-nutritive sucking in premature infants does not appear to alter blood concentrations of motilin, gastrin, insulin or insulin-like growth factor-1 three days after initiation of feedings. If changes in the secretion of these hormones are induced by non-nutritive sucking, they may be at a local paracrine level.

Enteral Nutrition↗
PubMed1990

Growth hormone by daily injection in patients previously treated for growth hormone deficiency.

When recombinant-DNA-derived methionyl growth hormone (met-GH) became available for patients resuming treatment, we were able to compare dose intervals in 47 prepubertal children with growth hormone deficiency. Patients were randomly assigned to one or three doses weekly or to daily injections for a total dosage of 0.3 mg/kg weekly. If the patient's annual growth failed to increase more than 2 cm above baseline, the dose interval was changed from weekly to three times a week or from three times a week to daily. In the second year of the study, all patients received daily injections. Despite a mean duration of previous treatment with growth hormone of more than 3 years, daily injections in 16 patients throughout the first year of the study resulted in a mean growth velocity (9.6 +/- 2.4 cm) comparable to that in newly treated patients given met-GH three times weekly in other trials. Administration by daily injection was more effective than injection three times per week (P less than .05) in 13 patients (7.9 +/- 2.1 cm) or once a week in four patients (7.7 +/- 1.2 cm). Second year growth velocities in 21 patients who had received once-a-week or three-times-a-week injections the first year of the study, increased significantly with a change to daily injection (7.7 +/- 2.2 vs 8.8 +/- 1.9 cm) (P less than .05).

Age Determination by Skeleton↗
PubMed1990

Height screening in the community. The commercialization of growth. The role of the pediatrician.

The availability of unlimited but costly supplies of biosynthetic growth hormone has led to pressure for pharmacologic use (as opposed to replacement therapy in proven deficiency states). Commercial and altruistic motives have converged to promote community height screening among individuals who have been perceived by themselves or parents as short. This does not meet accepted criteria for health screening. Height screening of large populations of children yields few unrecognized medical conditions. If the goal of community screening is to identify abnormally short individuals (less than 3%) who might benefit from growth hormone treatment and if the unproven assumption is correct that stature correlates with success and happiness, then those less likely to appear for screening need to be recruited to avoid elitist domination. The annual cost of such growth promotion would be greater than $10 billion, with no evidence for substantial health benefits. Growth monitoring of all children through improved height measurement in schools and in physicians offices, as part of health supervision, is a more sound community approach than height screening.

Body Height↗
PubMed1988

Pulmonary barotrauma including orbital emphysema following inhalation of toxic gas.

Severe pulmonary barotrauma occurred following smoke and toxic gas inhalation in a 20-year-old male. He developed pneumothorax, pneumomediastinum, and extensive facial subcutaneous emphysema which intensified during treatment with positive pressure ventilation. Following the appearance of diplopia and exotropia, orbital emphysema was demonstrated radiologically. The diplopia and exotropia were manifestations of mechanical interference in extra-ocular muscle function by the intra-orbital air, an unusual expression of pulmonary barotrauma.

Adult↗
PubMed1988

Drive and timing components of respiration in young children following induction of anaesthesia with halothane or ketamine.

Timing and drive components of respiration were studied in 18 young children following induction of anaesthesia with ketamine and were compared with results from ten children following induction of anaesthesia with halothane. During one minute of quiet breathing, signals from a pneumotachograph attached to the anaesthetic mask were analysed for tidal volume (Vt), respiratory frequency (f), minute volume (Ve), inspiratory and expiratory times (Ti, Te) and flow pattern. Following induction of anaesthesia with ketamine, children breathed more slowly and deeply than children receiving halothane, but there was no significant difference in Ve or in Vt/Ti, suggesting that respiratory drive was similar in the two groups of children. In the children receiving ketamine, Ti was more than twice as long, and thus the ratio Ti/Te was significantly increased, in comparison with the group receiving halothane. In addition to the prolonged Ti in the children induced with ketamine, there was a more rapid increase in volume in early inspiration than in late inspiration, which is an apneustic breathing pattern. There was a slower decrease in volume in early expiration, with occasional early expiratory breath holding lasting up to three seconds, in the ketamine-induced children. The unique breathing pattern demonstrated with ketamine, consisting of large Vt, increased Ti/Te ratio, apneustic inspiratory pattern, and expiratory braking, contributed to an increased mean lung volume above functional residual capacity, of 2.40 ml.kg-1 body weight, in comparison to 1.27 ml.kg-1 in the children receiving halothane.

Anesthesia, Inhalation↗
PubMed1988

Anaesthetic considerations in facial reconstruction for Down's syndrome.

Sixty-three patients with Down's syndrome underwent facial reconstructive surgery under general anaesthesia in order to improve their acceptability and potential for functioning effectively in society. Preoperatively, one-third of the patients had respiratory illnesses, 11 (17.5%) had cardiac anomalies, and 5 (7.9%) had endocrinological abnormalities. Anaesthesia was based on spontaneous ventilation of halothane and N2O in oxygen via an endotracheal tube with appropriate monitoring. Only one patient had an intraoperative complication, an episode of ventricular dysrhythmia, but postoperatively 9 patients required nasopharyngeal airways or endotracheal intubation in order to maintain a patent upper airway. The anaesthetic considerations for facial reconstructive surgery in Down's syndrome are discussed.

Adolescent↗
PubMed1987

Pulmonary barotrauma during cardiopulmonary resuscitation.

Two cases are presented of pulmonary barotrauma developing during cardiopulmonary resuscitation. This was attributed to high airway pressures developed during ventilation. One patient was ventilated with a self-inflating bag and the second with the Robertshaw demand valve. Both devices were used appropriately. Three other cases of pulmonary barotrauma during resuscitation have been recorded with the use of the latter device during CPR by ambulance personnel in Israel. The Robertshaw demand valve may generate excessively high airway pressures during normal use or when malfunctioning. For these reasons, the use of the Robertshaw demand valve has been discontinued in the Israeli Emergency Medical Services ambulances.

Aged↗
PubMed1987

Determinants of end expiratory volume in young children during ketamine or halothane anesthesia.

The expiratory time (Te) and the rate of lung emptying in expiration are important determinants of functional residual capacity (FRC) in infants. In order to determine whether these factors also influence FRC in children during anesthesia, 20 preschool children were studied, 10 while receiving ketamine, and 10 during halothane anesthesia. Te was measured during quiet breathing and the passive expiratory time constant (tau) was determined from the passive expiratory flow volume (V/V) curve following a brief end inspiratory airway occlusion. The number of time constants available for expiration, Te/tau, was then calculated. The difference between FRC and the relaxation volume of the respiratory system (Vrs) (FRC-Vrs) was measured by extrapolating the linear segment of the V/V curve to zero flow, and measuring FRC-Vrs. During ketamine anesthesia, tau was markedly prolonged (1.15 s, range 0.73-2.29 s), with the result that Te/tau was, in all subjects, less than 2. Children anesthetized with halothane had shorter tau (0.38 s, range 0.24-0.65 s), and Te/tau was more than 2 in most subjects. FRC-Vrs was significantly greater in the subjects from the ketamine group (203 ml, range 115-392 ml) than in those from the halothane group (32 ml, range 1-71 ml). For the 20 subjects, there was a significant relationship between FRC-Vrs (ml) and Te/tau described by the equation. FRC-Vrs = 845.0e-1.28(Te/tau) The authors conclude that, in children during ketamine anesthesia, tau is prolonged and, in these children, the relationship of Te to tau is an important determinant of FRC-Vrs. Te/tau was not related causally to FRC-Vrs in the halothane group.(ABSTRACT TRUNCATED AT 250 WORDS)

Anesthesia, General↗
PubMed1986

In vitro activity of antibiotics alone and in combination against Actinobacillus actinomycetemcomitans.

The MICs for 90% of the organisms tested (MIC90S) of 11 antibiotics against 24 clinical isolates of Actinobacillus actinomycetemcomitans were determined by the MIC 2000 system. The lowest MIC90S (16 micrograms/ml) were observed with ceftriaxone and rifampin. The next lowest MIC90S were found with cephapirin, tetracycline, and chloramphenicol (3.12 micrograms/ml). The MIC90S of penicillin, ampicillin, ticarcillin, piperacillin, and amikacin were each greater than or equal to 12.5 micrograms/ml. Antibiotic synergy was studied by the killing curve method and was defined as a greater than or equal to 2 log10 reduction in CFU when two antibiotics were used in combination at one-fourth the MBC for each compared with the effect of each antibiotic alone at one-half the MBC. Synergism between rifampin and penicillin, cephapirin, or ceftriaxone was tested for with 12 A. actinomycetemcomitans strains. In 7 of 37 instances, synergism was demonstrated for the combinations rifampin plus ceftriaxone (n = 3) or rifampin plus penicillin (n = 4); in 9 instances, an additive effect was noted, and impaired killing with drug combinations compared with the effect of a single antibiotic was suggested in 4 strains. The majority of strains were indifferent to the combinations. Similarly, variable results were observed when the combination of trimethoprim and cephapirin was tested against eight A. actinomycetemcomitans strains. Our data suggest that rifampin and cephapirin are the most active of the 11 antibiotics studied against A. actinomycetemcomitans. In addition, in vitro synergism between rifampin and other antibiotics or between trimethoprim and cephapirin was not consistently demonstrable.

Actinobacillus↗
PubMed1986

Growth hormone (GH) provocative testing frequently does not reflect endogenous GH secretion.

GH secretion was studied in 73 children with classical GH deficiency or GH neurosecretory dysfunction (GHND), intrinsic short stature, or normal stature. The GH-deficient group was defined by a peak GH secretory response below 10 ng/ml to all provocative tests (arginine, L-dopa, insulin hypoglycemia, and clonidine). GHND was defined by a mean serum 24-h GH concentration below 3 ng/ml, with a normal response (greater than or equal to 10 ng/ml) to provocative testing. Twenty-one GH-deficient children, 21 children with GHND, and 18 short control children underwent provocative GH testing and a 24-h study with GH sampling every 20 min. A group of 13 normal stature control children also underwent 24-h GH sampling. The mean stimulated peak serum GH level [4.7 +/- 0.6 (+/- SEM) ng/ml] in the GH-deficient group was significantly below that in the GHND (19.5 +/- 1.7 ng/ml) and short control groups (24.0 +/- 3.5 ng/ml; P less than 0.01). The mean 24-h serum GH concentration was reduced in GH-deficient (1.5 +/- 0.2 ng/ml) and GHND (2.0 +/- 0.1 ng/ml) children compared to those in short (5.6 +/- 0.5 ng/ml) and normal stature (5.8 +/- 0.8 ng/ml) control children (P less than 0.01). Peak GH concentrations after provocative testing correlated poorly with 24-h mean concentrations in GH-deficient, GHND, and short control children (r = 0.38, 0.23, and 0.41, respectively; P = NS for all groups). Mean serum GH concentrations from blood sampling intervals of 12 h (day/night; 0800-2000/2000-0800 h, respectively) or even 6 h (day; 0900-1500 h) were statistically different in GHND or GH-deficient groups compared to those in control children; however, there was significantly more overlap for individual children using the 6- and 12-h daytime intervals than for the 24-h data. Plasma somatomedin-C/insulin-like growth factor I correlated with mean 24-h GH concentration endogenous secretion (r = 0.7; P less than 0.001). These data suggest that provocative GH testing frequently does not correlate with endogenous GH secretion.

Adolescent↗
PubMed1986

The interrelationships of thyroid and growth hormones: effect of growth hormone releasing hormone in hypo- and hyperthyroid male rats.

Growth hormone (GH) and the thyroid hormones interact in the hypothalamus, pituitary and peripheral tissues. Thyroid hormone exerts a permissive effect upon the anabolic and metabolic effects of GH, and increases pituitary synthesis of this protein hormone. GH depresses the secretion of thyrotropin and the thyroid hormones and increases the peripheral conversion of thyroxine to triiodothyronine. In the adult male rat experimental hypothyroidism produced by ingestion of propylthiouracil depresses the GH secretory response to GH-releasing hormone in vivo and in vitro, reflecting the lowered pituitary stores of GH in the hypothyroid state. Short term administration of large amounts of thyroxine with induction of the hyperthyroid state does not affect the in vivo GH secretory response to GH-releasing hormone in this animal.

Animals↗
PubMed1985

The effect of ketamine on the functional residual capacity in young children.

The effect of ketamine on the functional residual capacity (FRC) was measured in nine ASA class I children prior to elective surgery. FRC was determined by the closed-circuit helium dilution method on the day prior to surgery in the awake state and also following induction of anesthesia on the day of the operation. Anesthesia consisted of ketamine by continuous intravenous infusion following preanesthetic sedation with atropine and triclofos or flunitrazepam. There were no significant differences in FRC between the measurements in the awake state and anesthetized (392 +/- 43 SEM ml, and 411 +/- 53 SEM ml, respectively), and the authors conclude that ketamine does not affect resting lung volume in young children.

Anesthesia, General↗
PubMed1984

Capnography in the early diagnosis of carbon dioxide embolism during laparoscopy.

Venous embolism of carbon dioxide occurred during elective diagnostic laparoscopy in a healthy adult female. The diagnosis of gas embolism was made on the basis of the sudden abrupt onset of systolic and diastolic murmurs. The continuously recorded end-tidal carbon dioxide concentration (FETCO2) increased abruptly from 3.8 to 4.2 per cent and then slowly decreased to 4.0 per cent over the subsequent 30 seconds. CO2 insufflation was terminated immediately following the establishment of the diagnosis. The patient recovered uneventfully. A transient but rapid rise in FETCO2 is suggested as a useful early sign of venous CO2 embolism during laparoscopy.

Adult↗