摘要:

Arterial concentrations of glycerol, FFA, glucose, lactate and β‐hydroxybutyrate were serially measured during the first two hours after birth in normal fullterm infants in a thermo‐controlled environment. Blood gas tensions, acid‐base balance, pulmonary gas exchange, motor activity and heart rate were also determined: a detailed report of these data will be published separately. In 22 infants the glycerol concentrations showed a rapid immediate increase after birth wheras the rises in FFA concentrations were delayed until between 30 and 120 minutes, indicating a prompt increase in lipolysis and a suppression of lipid mobilization during the first half hour after birth. This suppression might be explained by a high rate of reesterification or oxidation of FFA within adipose tissue. The influence of environmental temperature (2 8.7‐3 4.8oC) and degree of acidosis on the pattern of changes in FFA and glycerol were only marginal. No inhibition of lipolysis and lipid mobilization was shown in an infant who developed postnatal asphyxia.At 120 min after birth, when acidosis had been eliminated, an inverse correlation was found between the rise in FFA from birth to 120 min and the respiratory exchange ratio (V̇co2/V̇o2).The glucose concentrations were related neither to the FFA nor to the glycerol concentrations. The rate of elimination of lactate and β‐hydroxybutyrate was not influenced by environmental temperature or acidosis. Minute amounts of administered heparin caused an increased rise in FFA and glycerol concentrations which were associated with the appearance of lipoprotein li

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06677.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryGangliosides and neutral aminoglycolipids were determined in several organs from two subjects with classical Tay‐Sachs disease. Ganglioside GM2was stored in all organs investigated. Of the extraneural organs, the highest concentration was found in the adrenals, spleen and liver. The rectal muscular layer had a high concentration of gangliosides. Rectal biopsy might be used for studies of the nervous ganglioside pattern.The brain tissue showed a many‐fold increase of the activities of p‐nitrophenylglycosi‐dases. The activity of ganglioside sialidase in the brain was not increased in Tay‐Sachs and some other neurometabolic diseases. Subfrac‐tionation of the glycohydrolases by ultracen‐trifugation showed no hexosaminidase activity in the supernatant fraction of the brain in Tay‐Sachs disease compared with about 2/3 of the total activity in normal human brain.A simple agarose electrophoretic method was developed for the separation of the hexosaminidases into two fractions, A and B. Hexosaminidase A was virtually absent in Tay‐Sachs disease.The known forms of gangliosidoses and their biochemical characteristics are reviewed and a scheme is given for their classificat

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06678.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryA controlled therapeutic trial was performed in newborns with birthweight of 750‐1 250 g. 40 infants (the “treatment” group) received an i.v. infusion with glucose and NaHCO3from the 1st to the 4th‐7th day of life, and increasing amounts of human milk from the 2nd day of life. They also received, during the newborn period, 3 antibiotics (sodium penicillin G 500 000 U + methicillin 100 mg + colistin sulf. 8 mg per kg/day), a gamma globulin preparation (0.5 ml/day for 3 days) and fresh human plasma (3 ml every 2nd day in the first 5 days of birth). 40 infants (the “control” group) received only oral feeding with 10% glucose and human milk, starting in the 2nd day of life, and i.m. kanamycin (20 mg/kg/day for 5 days).No difference between treated and control babies was observed with respect to the following findings: neonatal mortality rate and survival curve on the whole series; neonatal mortality rate in babies with birthweight above or below 1.04 kg, with gestational age below 28 weeks, with above or below median respiratory rate or arterial pH on admission, with or without marked abnormality on chest film, and in males or females (but, in subjects with gestational age above 27 weeks, a significantly lower mortality rate was found in controls); postmortem findings; incidence of apnoeic spells, tremors and cloni, and pattern of muscular tonus. Bradycardia associated with 2: 1 atrioventricular block was observed in 6 controls in the 2nd day of birth. No adverse effects presumably due to the therapeutic procedures so contrasted were demonstrated.It was concluded that in the present series of newborns with very low weight: (a) the massive anti‐infectious therapy was not superior to the administration of a single broadspectrum antibiotic;(b)the administration of fresh human plasma was not effective in preventing hemorrhage (but further evaluation is needed); (c) the routine i.v. infusion with glucose and NaHCO3did not improve neonatal survival as compared with oral feeding.However, previous studies, as well as observations on the present series reported elsewhere (11), have shown that several biochemical abnormalities commonly seen in orally fed newborns with very low weight can be prevented or corrected, at least in part, by the early infusion with glucose and NaHCO3. Since some of these abnormalities are potentially dangerous to the central nervous system, the final evaluation of this therapy must await long‐term follow‐up studies in survivors. It is therefore suggested that, before such studies are available, the early i.v. infusion with glucose and NaHCO3should be given routinely to newborns wit

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06679.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryA controlled therapeutic trial was performed in newborns with birthweight of 750‐1 250 g. 40 infants (the “treatment” group) received an i.v. infusion with glucose and NaHCO, from the 1st to the 4th‐7th day of life, and increasing amount of human milk from the 2nd day of life. They also received in the newborn period 3 antibiotics (penicillin, methicillin, and colistin), gamma globulin, and fresh human plasma. 40 infants (the “control” group) received only oral feeding with 10% glucose and human milk, starting in the 2nd day of life, and i.m. kanamycin.As compared to controls, in treated infants acidemia decreased more rapidly in the first two days of birth, but hypercarbia developed after the 3rd day.Blood electrolytes and ECG studies were performed in part of the infants in the 2nd day of life. Markedly increased serum K and and BUN levels were observed in controls, and significantly lower values in treated infants. Severe ECG abnormalities were associated with hyperkalemia in controls, and were never observed in treated infants. Serum Ca levels were low and similar in the two groups. Hyperbilirubinemia occurred more frequently controls, and exchange‐transfusion was performed only in this group of infants.Since many metabolic abnormalities commonly occurring in fasting newborns with very low weight are potentially dangerous to the central nervous system, the conclusive evaluation of the i.v. infusion with glucose and NaHC03 must await long‐term follow‐up studies in survivors. While waiting for this evidence, it is suggested that this therapy should be given routinely to newborns with very low weight. The present observations also suggest that, if a reasonably fast oral feeding schedule is provided, in most circumstances the infusion therapy should not be continued after the

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06680.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryThe effect of hexahydrocoenzyme Q4(CoQ) has been studied in 4 cases of Duchenne muscular dystrophy, including a pair of identical twins.CoQ was given by the oral route in doses increasing from 50 mg to 1 g daily. In the case of the twins one received CoQ, whereas the other was given corn oil as a control.During a period of treatment of more than one year no improvement in muscular strength was observed. On the contrary, there was a steady progression of the disease in 3 of the 4 cases, with an identical clinical course in the twins.No overall consistent decrease in CPK or aldolase activities was observed during the period of treatment, but there was a statistically significant decrease of CPK and aldolase for one boy.The absence of overall beneficial changes during this particular trial might have been because (a) hexahydrocoenzyme Q4is intrinsically inactive in Duchenne dystrophy; (b) the dose level was too low; (c) the disease in these particular boys was too advanced.Besides these limitations, this trial also does not exclude the possibility that other homologs of the coenzyme Q group may have therapeutic benefit in human muscular dystrophy.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06681.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryThe present data suggest that perinatal asphyxia may result in a transfer of blood,in utero,from placenta to fetus. The data contain no suggestion that asphyxia causes pooling of fetal blood in the placenta.

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06682.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryFree and conjugated components of the total urinary glucuronic acid were analyzed daily during the first 4 days of life in 62 newborn male infants. The newborn were divided into two groups: “jaundiced” 37 infants in whom the serum bilirubin level on the fourth day of life was either the same or higher than the level on the third day; and “non‐jaundiced” 25 infants, in whom the serum bilirubin level showed a fall on the fourth day after an initial rise during the first 3 days. The percentage of free glucuronic acid excreted was found significantly higher in the jaundiced infants.Premature infants studied for comparison demonstrated an even higher percentage of free glucuronic acid excreted in the urine than the jaundiced infants. On the other hand, in healthy adult males the percentage of free glucuronic acid excreted was found to be the lowest.No significant difference was found in the total glucuronic acid concentrations of the jaundiced and non‐jaundiced infants.It could be concluded, therefore, that when a high percentage of free glucuronic acid is excreted in the urine this probably indicates impaired conjugating capacity o

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06683.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryDescription of a case of “chronic hypomag‐nesemia” with secondary hypocalcemia, tetany and convulsions followed for a period of 3 years, from the age of 7 month. Primary malabsorption of magnesium has been demonstrated. Mitochondria of intestinal mucosa cells were found to be swollen. Mg‐con‐centration was increased

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06684.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummaryA study has been carried out on the relationship between M, Ca and Sr in a case of primary Mg‐malabsorption and in control children on high‐ and low‐Mg diets.Probably more than one transfer system exists for these cations in intestine and in Mg‐malabsorption the simple diffusion of both Mg and Sr is impaired.The influence of Mg on Ca‐P homeostasis is not clearly understood but a resistance to parathormone and vitamin D in Mg‐deficiency is

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06685.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY

摘要:

SummarySixty‐nine children suffering from superficial fungal infections were treated with four pimari‐cin preparations: pimafucin vaginal tablets, pimafucin 1 % suspension, pimafucin, and pimafucort ointments. Complete cure was achieved in fifty‐three children (76.8%), improvement in twelve (17.3%). In four (5.7%) children with serious basic diseases (leukaemia, diabetes) the treatment was unsuccessful. No signs of intolerance were obs

ISSN:0803-5253    

DOI:10.1111/j.1651-2227.1971.tb06686.x

出版商:Blackwell Publishing Ltd    

年代:1971

数据来源: WILEY