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Biomedical subjects

S W Moses

Publications and source records attributed to S W Moses.

At least 55 records · Page 3Linked to original sources

Necrotizing enterocolitis beyond the neonatal period.

Necrotizing enterocolitis (NEC) is usually considered to be a neonatal disease, and is rarely described beyond the newborn period. During the last 15 years, 19 infants from the Negev region, Israel, with NEC were beyond the neonatal age group (range = 34-616 days, median = 90 days). Of this group only 16% were born prematurely, and only 16% had perinatal or neonatal pathology. Diarrhea and dehydration preceding NEC were common as was malnutrition. Bacteremia was detected in 42%. The mortality rate was 90%. Malnutrition, supposedly contributed to the formation of NEC and to the high mortality rate among these infants.

Age Factors↗

The dietary treatment of children with type I glycogen storage disease with slow release carbohydrate.

The effect of ingestion of uncooked cornstarch (2 g/kg body weight) in water, uncooked starch (1 g/kg) added to a meal, and glucose (2 g/kg) in water, was studied in eight patients with type IA glycogen storage disease (GSD) and one patient with type IB GSD. Blood glucose concentrations were determined at 30-min intervals during each tolerance test; blood lactate, blood insulin, and expiratory hydrogen were determined at 60-min intervals. The glucose levels remained in the normal range (greater than or equal to 1.8 mM) during approximately 6.5-9.0 h, 3.5-6.5 h, and 2.25-4.0 h during the three tolerance tests, respectively. The lactate levels differed markedly for the different tests per patient, and for the same type of test between the patients. Blood insulin concentrations after starch administration did not exceed values of 50 mU/liter above fasting levels and were markedly lower than those after glucose administration (maximum levels of 280 mU/liter). The expiratory hydrogen excretion did not increase or only slightly increased after cornstarch administration (less than 20 ppm).

Adolescent↗

Infant feeding practices among Bedouins in transition from seminomadic to settlement conditions in the Negev area of Israel.

Infant feeding practices among 353 Bedouin families in transition from seminomadic to settlement conditions in the Negev area of Israel were compared with those of 302 Jewish families from the same area. Over 99% of the Bedouin infants were initially breast-fed, in contrast to 79% of the Jewish infants; none of the Jewish infants continued to be breast-fed by the end of the first year of life, while 63% of the Bedouins were. Rice was the first solid food to be introduced to Bedouin infants, while fruits and vegetables were the first solids introduced to the Jewish infants. Rice was not an important constituent of the Jewish infants' diet. By the age of 6 months, 93% of the Jewish infants were eating fruits and vegetables, 78% meat, 49% bread and 55% eggs, in contrast to 20, 13, 8 and 18% among the Bedouins, respectively. Introduction of meat lagged significantly among the Bedouin infants, taking place after the 8th month of life for greater than 50%. Bedouin infant-feeding practices resembled those prevalent among rural populations in developing countries. It is likely that with increasing modernization this pattern will gradually disappear and will be replaced by that prevalent among Western populations.

Adult↗

Growth and nutritional status of Bedouin infants in the Negev Desert, Israel: evidence for marked stunting in the presence of only mild malnutrition.

Growth and feeding practices of 353 Bedouin infants from the Negev Desert, Israel, were compared to those of 302 Jewish infants from the same area and to American standards. These two populations differed in their cultures and educational backgrounds. The use of medical and health services was lower among the Bedouin population. The feeding practices of the Bedouin infants were markedly different from those of their controls. The Bedouin infants show a progressive decrease in weight, length, and head circumference (means of all three parameters were around the 5th percentile) while the Jews were comparable to Americans. The nutritional status was assessed by three different anthropometric measurements. The weight to length ratio showed that 88% of Bedouin and 96% of Jewish infants were above the 10th percentile. Tricep skinfold measurements showed that 96% of the Bedouins and 99% of the Jews were above the 5th percentile. The midarm circumference to head circumference ratio was in the range between 0.280 and 0.310 (mild malnutrition range) while that of the Jews was above 0.310 (well-nourished range). These data show marked stunting in the presence of only mild malnutrition. This observation argues against the general belief that marked stunting is the result of prolonged severe malnutrition. Differences in cultural and genetic backgrounds, as well as different feeding practices and increased morbidity, could contribute to this phenomenon.

Anthropometry↗

Dexamethasone and salbutamol in the treatment of acute wheezing in infants.

Thirty-two infants, aged 1 to 12 months, hospitalized with acute wheezing, were studied. They were randomly divided into four treatment groups of eight patients each. The treatments were intramuscular dexamethasone or placebo (double-blind), and salbutamol (oral and inhaled), or none (open), in all four possible combinations. The study was carried out as a randomized block design with eight blocks of four infants each, matched by age and clinical score. Average daily improvements, as reflected by changes in the clinical score and length of hospital stay, was essentially the same for infants treated with placebo, salbutamol alone, and dexamethasone alone. However, combined salbutamol-dexamethasone treatment resulted in more than twice the rate of improvement of the other treatments. The difference was statistically highly significant (P less than .01). Furthermore, the response of this combined treatment was observed within 24 hours; none of the ten infants in whom there was no significant improvement within 48 hours and neither of the two patients who developed respiratory failure received the combined salbutamol-dexamethasone treatment. A potentiating effect of corticosteroids on the beta-adrenergic responsiveness is a possible explanation for the advantage of this combined treatment in the management of acute wheezing in infancy.

Acute Disease↗

Clinical variability of congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency.

Studies in 18 Jewish families from Morocco, Tunis, Turkey and Iran revealed 26 patients with congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. The clinical expression of androgen excess varied widely in affected females, and range from solely enlarged clitoris in the mildest forms to severely hypertrophied clitoris with penile urethra and fused labial-scrotal folds in the most extreme forms of masculinization. Intermediate degrees of severity were manifested by ambiguous genitalia. There was no correlation between the degree of virilization and the signs of mineralocorticoid excess. Severe volume-induced hypertension leading to vascular accidents and death were also observed in severe as well as in mildly virilized patients, while completely masculinized females were sometime normotensive. Overt hypokalemia was present in 6 patients but was not a constant feature of hypertensives. However, all affected individuals, except for 2 infants, had very low levels of plasma renin activity suggesting that a state of volume expansion was indeed present in the majority of cases, even though changes in blood pressure did not always occur. The clinical expression of this disorder is characterized by a wide range of variability in the signs of both androgen and mineralocorticoid excess, which do not necessarily correlate with the quantity of hormones secreted.

Adolescent↗

Permanent tetraplegia as a consequence of tetanus neonatorum. Evidence for widespread lower motor neuron damage.

It is generally believed that no permanent neurological damage is found among survivors of tetanus neonatorum. Newborns dying shortly after the onset of tetanus also lack significant neurological abnormalities. In adults a variety of neuromuscular lesions have been reported; however, a uniform pathological picture is absent. We report a case of a newborn with severe tetanus in whom striking evidence of anterior horn neuronal damage was documented, causing permanent nonprogressive tetraplegia. We suggest that the mechanism responsible for this lesion involves the retrograde axoplasmic flow of tetanus toxin reaching the spinal cord via nerve endings in the infected umbilical cord stump.

Atrophy↗

Glycogenosis due to liver and muscle phosphorylase kinase deficiency.

A four-year-old Israeli Arab boy was found to have glycogen accumulation in both liver and muscle without clinical symptoms. Liver phosphorylase kinase (PK) activity was 20% of normal, resulting in undetectable activity of phosphorylase a. Muscle PK activity was about 25% of normal, resulting in a marked decrease of phosphorylase a activity. Two sisters showed a similar pattern, whereas one brother had normal PK activity. The patient's liver protein kinase activity was normal Addition of exogenous protein kinase did not affect PK activity, whereas exogenous PK restored phosphorylase activity to normal. These findings indicate that these patients are affected by a rare variant of PK deficiency, which involves both muscle and liver and which apparently is not sex linked. It is possible that this defect represents an unusual mutation of a subunit of the phosphorylase kinase enzyme.

Child↗

Haemorrhagic infarction of the myocardium in a newborn with haemoglobin H disease and erythroblastosis.

Extensive haemorrhagic myocardial infarction developed in a newborn, apparently as a result of anoxia due to erythroblastosis fetalis, associated with haemoglobin H disease. Acute massive myocardial infarction in the neonatal period is rare and usually is associated with congenital malformation of the heart or its blood supply. Neonatal myocardial infarction in the anatomically normal heart with normal coronary vessels, has been described in only 8 patients (1). The communication describes a newborn with acute haemorrhagic myocardial infarction due to anoxia believed to be caused by the combined effect of erythroblastosis fetalis and haemoglobin H disease.

Erythroblastosis, Fetal↗

Caffeine pharmacokinetics in young and adult dogs.

The pharmacokinetics of caffeine were studied in young (age: 1 day, 7 days, 14 days, 30-45 days) and adult dogs following a single intravenous dose of 50 mg/kg. Mean (+/-SE) plasma elimination half life (T1/2) was 47.5 (+/-5.35) h in 1-day-old puppies, as opposed to 6.66(+/-0.85) h in adult dogs. A rapid decrease in plasma T1/2 values occurred during the first 2 weeks of life. At about 14 days of age caffeine plasma T1/2 was similar to that of adults. The volume of distribution was greatest (0.94+/- 0.03 liters/kg) and the total body clearance was smaller (2.81 +/- 0.73 liters/kg/min X 10(-4)) in the 1-day-old animals. The much smaller body clearance of caffeine in the newborn as compared to the adult dog could be due to a lower rate of metabolism and/or renal excretion of this drug in the young.

Age Factors↗

Salt conservation in familial dysautonomia (Riley-Day syndrome).

In some patients with familial dysautonimia, plasma renin activity shows a paradoxical response to postural stimuli, i.e., levels of plasma renin activity are high when the patient is in the supine position and fall significantly during subsequent ambulation. Furthermore, there is no coordinated release of plasma renin activity and aldosterone. The aim of the present study was to determine whether these findings are accompanied by a disturbance of salt conservation. Six patients were studied in a summer camp while on normal and low-salt diets. Plasma and urinary aldosterone levels rose sharply and appropriately when four of the patients were placed on a low-sodium diet. In these subjects, urinary sodium output fell sharply although three of them failed to attain sodium equilibrium by the third day of the low-sodium regimen. Elevation of early morning plasma renin activity appeared to correlate with an inversion in the normal day-night rhythm in urinary volume.

Adolescent↗

Effect of hydrocortisone and glucagon on glycogen metabolism in the fetal rat liver.

1. Hydrocortisone increases in vivo incorporation of [14C] glucose into fetal liver glycogen in the last days of gestation, whereas in glucagon-treated fetuses, a slight decrease in the incorporation rate was found. 2. Hydrocortisone increases total synthetase activity as that of synthetase a but was without effect on fetal liver glycogen phosphorylase. 3. Glucagon causes a slight increase in phosphorylase a activity on days 19-21, and was without effect on the activities of synthetase a and total synthetase. 4. Dibutyryl cyclic AMP had no effect on the key enzymes of glycogen metabolism 1 h after injection in utero, whereas after 6 h an increase in phosphorylase a activity was found without any change in synthetase a activity.

Animals↗