Showing papers by "Boston Children's Hospital published in 1973"

Muscle Metabolism and Enzyme Activities after Training in Boys 11–13 Years Old

Abstract: The effect of training on the skeletal muscle metabolism of 11-to 13-year-old boys was examined. In one experiment changes in blood lactate, and muscle lactate, CP, ATP, and glycogen were determined at rest and following exercise before and after 4 months of training. The concentrations of glycogen, CP and ATP at rest were higher (P<0.01) following training. Blood and muscle lactate were 23 and 56 % higher after maximal work following training. A greater reduction in muscle glycogen occurred during maximal work after training but the pattern for ATP and CP depletion was unchanged. In a second experiment boys trained by pedalling a bicycle ergometer an average of 30 min 3 times a week for 6 weeks. Biopsy samples of the vastus lateralis were examined for oxidative (succinate dehydrogenase) and anaerobic (phosphofructokinase) capacity before and after training. The fiber composition and relative oxidative capacity in the fibers was determined histochemically. Succinate dehydrogenase and phosphofructokinase activities increased 30 and 83 %, respectively, following training. Fiber distribution was unchanged by training but the oxidative capacity of both fiber types appeared to increase.

A Genetic Survey of Idiopathic Scoliosis in Boston, Massachusetts

Abstract: Families of 207 index patients, including first, second, and third degree relatives were surveyed for scoliosis, and the incidence was compared with that from a similar study in Edinburgh. The data suggest a multifactorial mode of inheritance.

Temperamental characteristics as predictors of behavior disorders in children

Abstract: Sixty children, three to seven years old, each of whom had at least one mentally ill parent, were assessed for both temperamental differences and behavior disorder. Information was obtained from parents and teachers one year after the initial interviews. Certain temperamental characteristics, especially low habit regularity and low fastidiousness, were predictive of the development of later psychiatric disorder.

Production of Haemophilus influenzae b Meningitis in Infant Rats by Intraperitoneal Inoculation

Abstract: Sprague-Dawley rats have a marked age-related susceptibility to Haemophilus influenzae type b that does not correlate with serum bactericidal activity. Eighty percent of 5-day-old animals that survive to 48 h after an intraperitoneal inoculation of a mean lethal dose of bacteria have histologically documented meningitis. Animals surviving the inoculations as infants manifest cerebral dysfunction as adults. This model should facilitate experimental study of bacterial meningitis. Images

Cancer of the kidney—etiology, epidemiology, and pathology

Abstract: Renal adenocarcinoma represents approximately 1% of all malignancies exclusive of skin cancer. It is extremely rare in children or young adults and its incidence is over three times as great in men as in women. Familial occurrence is rare although there is a high frequency in patients with Lindau's disease. Naturally occurring renal adenocarcinoma is uncommon in lower animals except in rare instances of familial tumors in rodents. However, in laboratory animals renal adenocarcinomas are readily induced by a variety of carcinogens including chemical, physical, and viral agents. Implications of these studies to the epidemiology of human renal adenocarcinoma are discussed. Human, as well as naturally-occurring and experimentally-induced renal adenocarcinomas in lower animals, arise from the proximal convoluted tubule and are remarkably similar histologically and electronmicroscopically. Typically, these tumors are composed of cells which are clear or granular, a function of the cellular content of glycogen, lipids, and cytoplasmic organelles, and may be arranged in solid, cystic, trabecular, tubular, or papillary patterns. Rarely (1.8% of all renal adenocarcinomas) they are sarcomatoid mimmicking fibrosarcoma, rhabomyosarcoma, or liposarcoma. These various histologic patterns are poorly related to survival except for the sarcomatoid tumors which are regarded as highly aggressive. Renal cortical parenchymal tumors resembling the renal adenocarcinoma but less than 3 cm in diameter have historically been regarded as adenomas because of their low frequency of metastases. Based on the many points of similarities between the renal adenocarcinoma and the so-called renal adenoma, it is postulated that these tumors are simply small renal adenocarcinomas which have not yet grown large enough to metastasize.

Group b streptococcal neonatal and infant infections

Abstract: Group B streptococcal neonatal sepsis is a significantly underestimated problem. Epidemiologic studies revealed an incidence of 2 per 1,000 live births and a mortality rate of 1 per 1,000 live births. Vaginal cultures for group B were positive in 4.6 per cent of women at delivery, and 1.2 per cent of their infants had positive thorat cultures. Two distinct types of sepsis were seen in 43 infected infants. One type presented within hours of birth with respiratory distress, and the second presented as meningitis in the late neonatal period. The pattern and severity of disease appeared to be related to the group B serotype isolated. Antepartum vaginal cultures and treatment of group B-positive women will prevent most of these cases of neonatal sepsis. Eradication of the organism is dependent upon evaluation of the husband, who may harbor group B in the urethra.

Effect of physical training on hemodynamic response during submaximal and maximal exercise in 11-13-year old boys.

Abstract: Nine healthy boys, mean age 11.7 years (11.0–13.0), height 150.4 cm and weight 45.1 kg, were examined with determinations of maximal oxygen uptake (1.85 1×min-1), heart volume (499 ml) and total hemoglobin (391 g). Cardiac output was determined at rest and during exercise, including maximal exercise, using the dye-dilution technique and i.a. pressures were recorded. Cardiac output was approximately 2 1×min-1 lower and the systemic a-v oxygen difference (AVD) was correspondingly higher than for young adult men at the same sub-maximal oxygen uptake. At maximal exercise cardiac output was 12.5 1×min-1, stroke volume 67 ml, AVD 14.2 ml × 100 ml-1, systolic, diastolic and mean blood pressure 160, 71 and 105 mm Hg respectively and total peripheral resistance 8.6 mm Hg×1–1×min. After a training period of 4 months a normal increase in height was found in all boys, but body weight was unchanged. Maximal oxygen uptake increased to 2.21 1×min-1 (p < 0.01), almost entirely due to increased stroke volume (80 ml), resulting in a maximal cardiac output of 14.6 1×min-1. No significant increase in AVD was found. Mean blood pressure at maximal exercise increased significantly, total peripheral resistance was unchanged. Both heart volume and total hemoglobin showed minor increases, but the changes found were not significantly larger than expected from body growth. The hemoglobin concentration was normal (13 g%) for the age and unaffected by training.

Regulatory role of reducing-equivalent transfer from substrate to oxygen in the hepatic metabolism of glycerol and sorbitol.

Abstract: Suspensions of morphologically intact isolated rat-liver cells were used in conjunction with specific inhibitors to identify and quantitate the hepatic hy drogen-trans ocating systems involved in the transfer of reducing-equivalents from sorbitol or glycerol to O2. Rates of hydrogen translocation were derived either from measurement of the major products of substrate metabolism or from rates of substrate utilization. It was found that at saturating substrate concentrations, rates of sorbitol or glycerol 3-phosphate oxidation were closely similar (about 1.8 μmol × g wet weight−1× min−1). There was an inverse relationship between rates of sorbitol and glycerol uptake so that the rate of hydrogen flux to O2 from substrate mixtures was no greater than that from either substrate added separately. Rates of sorbitol and glycerol consumption were increased by pyruvate acting as a cytoplasmic hydrogen acceptor. It is concluded from these observations that at high substrate levels and in the absence of a cytoplasmic hydrogen acceptor, hydrogen translocation is the rate-limiting process in the hepatic metabolism of sorbitol and glycerol and that the flux of reducing equivalents to O2 from these two substrates involves shared hydrogen-translocating systems. At low levels of substrate, more likely to be encountered in vivo, the rate of sorbitol or glycerol metabolism is dependent also on substrate concentration, but even under these circumstances it was found that the capacity of the hydrogen-translocating systems governs the over-all rate of metabolism whenever substrate mixtures were present. The nature of these systems was assessed by the use of specific inhibitors. About 15% of the flux of reducing equivalents to O2 involved antimycin-insensitive pathways, presumably microsomal. A further 40% passed to O2 by a rotenone-insensitive path, most likely involving flavin-linked mitochondrial glycerolphosphate dehydrogenase. The remainder of the flux was rotenone-sensitive, but less than half of this utilized malate-oxaloacetate or malate-aspartate shuttles. The pathway for this residual rotenone-sensitive fraction (about 20–30% of the total flux) remains to be clarified. These data suggest that in parenchymal cells from normal rat liver the glycerol 3-phosphate shuttle may be more important for the transfer of reducing equivalents from cytoplasm to mitochondria than has been previously recognized. Sorbitol uptake by the cells was inhibited up to 70% by uncoupling agents. This inhibition could be overcome by addition of pyruvate as a cytoplasmic hydrogen acceptor or by artificial electron acceptors. This implies that uncoupling agents prevent the oxidation of cytoplasmic NADH by interfering with the operation of the normal hydrogen shuttles between cytoplasm and mitochondria and that these shuttles are energy-dependent. The rate-limiting and energy-dependent nature of the hydrogen translocating systems as revealed by these studies identify them as potential sites for metabolic regulation and as possible targets for hormonal action.

Changes in Cell and Organ Shape during Early Development of the Ocular Lens

Abstract: It is currently fashionable to attribute changes in organ shape during development to the actions of microtubules and microfilaments on individual cells of the organ in question. In the case of the eye lens it has been proposed that cellular elongation under the influence of microtubules and/or apical contraction by microfilaments are responsible for the remodeling of the originally low cuboidal ectoderm into the tall and wedge-shaped presumptive lens cells. Invagination of the lens is thought to follow automatically. These ideas cannot account for certain observations on lens morphogenesis, such as the relatively fixed diameter of the organ rudiment during early development, which is incompatible with the supposed contraction of the rudiment. We found that the area of contact between presumptive lens and optic cup does become fixed after a few hours of “induction.” There is a remarkable correlation in time between this fixation, and the process of lens cell elongation and increase in lens cell density. We calculated that the latter two can, in fact, be accounted for by population pressure caused by continued cell division within the defined area of the lens rudiment. A mathematical model along these lines was developed, which explains lens invagination on the basis of cell number and size, extent of the area of contact between ectoderm and optic cup, and cell population doubling times. We hypothesize that the prevention of lateral cell spreading within the lens territory, after the contact area becomes fixed, is a function of the build-up in extracellular materials in this area during the “induction period.” Both lens rudiment and presumptive retina contribute to this extracellular matrix.

Rigid spine syndrome: a muscle syndrome in search of a name.

Abstract: Comment These 2 cases illustrate the dangers of injecting into the umbilical cord substances which may cause spasm or thrombosis of the umbilical artery and sometimes also of the internal iliac artery, as occurred in Case 1. The arteria comitans nervi ischiadici, a secondary branch of the internal iliac artery after it is joined by the umbilical artery, is particularly susceptible. The possibility that other substances, such as concentrated dextrose, could cause the same effects, should not be overlooked. For a review of the condition, with references, see Sharrard (1971).

Mouse bone collagenase. The effect of heparin on the amount of enzyme released in tissue culture and on the activity of the enzyme.

Abstract: The amount of mouse bone collagenase recovered in the tissue culture medium of bone culturedin vitro was increased by the addition of heparin at an optimal concentration of approximately 50 units/ml of tissue culture medium. Dextran sulfate and Treburon (a synthetic polysaccharide-sulfuric ester) which are structurally and chemically related to heparin were as effective as heparin in increasing the amount of mouse bone collagenase recovered in the tissue culture medium. In addition to stimulating the synthesis and/or release of mouse bone collagenase, heparin was also found to increase the specific activity of both crude and purified preparations of the enzyme when assayed using collagen in the solid state as the substrate, but showed no enhancement of enzyme activity when assayed using collagen in solution as the substrate. Dextran sulfate was as effective as heparin in increasing the activity of the enzyme using collagen in the solid state as a substrate. Neither heparin or dextran sulfate enhanced the activity ofClostridium histolyticum collagenase. For the first time, a purified tissue collagenase has been shown to both degrade and solubilize undenatured, insoluble tissue collagen at 37°. Moreover, since this action was markedly enhanced by the addition of heparin, it suggests that heparin and similar substances may play an important role in the regulation of collagen degradation during the remodeling of collagenous tissuesin vivo.

Artificial blood and method for supporting oxygen transport in animals

Abstract: Perfluorocyclocarbons and emulsions containing emulsified particles of these perfluorocarbons are useful is supporting life as intravascular oxygen and carbon dioxide transport agents Stable emulsions of perfluorocarbocyclic derivatives are prepared for use as artificial bloods and perfusates for organs Intravenously infused emulsions containing perfluorocyclocarbons such as perfluoro(methylcyclohexane), perfluoro(1,3dimethylcyclohexane), perfluoro(decahydronaphthalene), perfluoro(decahydro-1-methylnaphthalene), perfluoro(decahydrodimethylnaphthalene), and the like, serve as intravascular oxygen and carbon dioxide transport agents Fluorocarbon liquids are also useful as an external respiration media for transporting oxygen-carbon dioxide necessary to support intravascular respiration Perfluorocyclocarbons have been found after intravenous emulsion infusion as artificial bloods to collect in the reticuloendothelial system (RES) of the amimal body However, it has also been found that perfluorocyclocarbon compounds leave the RES of the animal body and certain of these compounds, eg, perfluoro(decahydronaphthalene), leave the RES at a much faster rate than others Furthermore, after the intravenously infused perfluorocyclocarbon emulsions function as artificial bloods, the perfluorocyclocarbons have the capability of leaving the animal body through the tissue, eg, the lungs and skin

Malformations of kidney and urinary tract in common chromosomal aberrations. I. Clinical studies.

Abstract: A review of urinary tract malformations in chromosomal aberrations is presented. This study is based on a careful evaluation of published cases as well as of our own patients and autopsy material.

I-cell disease, mucolipidosis II. Pathological, histochemical, ultrastructural and biochemical observations in four cases.

Abstract: Pathological, histochemical, ultrastructural and biochemical studies on 4 cases of I-cell disease are reported. These studies support a lysosomal defect with deficiency of many acid hydrolases and storage of both glycolipids and mucopolysaccharides within lysosomes. The clinical, pathological and biochemical spectra of I-cell disease in these 4 cases suggest three distinct nosologic entities: a prototype, a malignant infantile form and a more benign juvenile type. Consanguinity was present in one of the cases. An autosomal recessive mode of inheritance seems most likely.

Endometrial (papillary) carcinoma of the prostatic utricle--response to orchiectomy. A case report.

Abstract: A case of papillary duct carcinoma of the prostate, termed “endometrial carcinoma of the prostatic utricle,” is presented. The bladder trigone and ureteral orifices were involved by tumor producing a nonfunctioning right kidney and left hydronephrosis. Orchiectomy produced objective regression in the tumor mass with return of right renal function, decrease in left hydronephrosis, and marked clinical improvement. The tumor displayed androgen dependence, despite the suggestion that such tumors are estrogen‐dependent derivates of the median Müllerian remnant in the prostatic urethra (the utriculus masculinus).

Unusual combined immunodeficiency syndrome exhibiting kappa IgD paraproteinemia, residual gut immunity and graft versus host reaction after plasma infusion

Abstract: Summary A 12-year-old suffering from a lymphopenic severe CID with an unusual protracted clinical course is presented. His gut-associated lympho-epithelial system was apparently normal, in contrast to the IgA deficiency in other external fluids. In addition, an elevated kappa-IgD was detected in his serum. Fresh plasma infusions from unrelated donors induced an accidental engraftment with a moderate non-fatal GvHR.

Extended combination therapy of childhood rhabdomyosarcoma.

Abstract: Nineteen children with biopsy-proven rhabdomyosarcoma were selected for a 1-2-year study utilizing surgery, supervoltage, and electron-beam radiation therapy, and combination chemotherapy with vincristine, cyclophosphamide, and dactinomycin. At the time of diagnosis, 4 patients had localized tumor, 9 had regional spread of tumor, 5 had extensive spread of tumor, and 1 had bone marrow involvement. Complete response to therapy was seen in 17 patients, 2 had partial response followed by progressive disease, and 4 died with disease at 15, 11, 8, and 6 months respectively. Eleven patients were free of disease for 4-49 months; 2 had extension of disease at 2 and 4 months. Two patients were just entered on the study and response data is being investigated currently. This approach of extended combination therapy of childhood rhabdomyosarcoma was well tolerated and offers an alternative to radical surgery in the growing child.