bluuebunny/crossref_metadata_2025_split huggingface dataset:
PHENYLKETONURIA: A STUDY OF HUMAN BIOCHEMICAL GENETICS
Authors: Hsia David Yi-Yung 
Published: December 2021

ONE of the privileges extended to me upon receiving the E. Mead Johnson Award is to have an opportunity to express in public my gratitude to the several teachers who have contributed so heavily to my development. First and foremost, I want to thank Dr. Sydney Gellis, who first took me on as a research fellow, subsequently fired in me an excitement and enthusiasm for research, and has remained through the years a loyal friend and colleague. Next, I want to thank Dr. Charles Janeway who had the vision of urging me to become interested in the then embryonic field of "inborn errors of metabolism." In the pursuit of this interest, I want to thank Dr. Eugene Knox for teaching me the biochemistry and Professor Lionel Penrose for teaching me the genetics which subsequently led to the work on phenylketonuria. Finally, the late Dr. John Bigler and Dr. Robert Lawson gave me the encouragement and the freedom to pursue these studies in Chicago. On this occasion I would also like to express my appreciation to the Mead Johnson Company for having provided me with my first fellowship, which permitted me to go into research, and also for having manufactured the product which has played such a vital role in our understanding of phenylketonuria.
The concept of "inborn errors of metabolism" was first suggested by Sir Archibald Garrod in 1908. In the Croonian Lectures delivered at the Royal College of Physicians, he suggested that four metabolic disorders—albinism, alkaptonuria, cystinuria, and pentosuria—had certain features in common.
STUDIES ON HYALINE MEMBRANE DISEASE
Authors: Ambrus Clara M. , Weintraub David H. , Ambrus Julian L. 
Published: December 2021

Fibrinolytic enzyme therapy of respiratory distress syndrome was explored in a controlled, randomized, double-blind clinical study. Of 100 infants entered in the study, 60 corresponded to all of the predetermined criteria and were included in the final evaluation. Infants with respiratory distress syndrome were admitted to the study if they were (1) premature, (2) from diabetic mothers, or (3) from mothers with hemorrhagic complications of pregnancy. In regard to sex, delivery route, maternal status, pretreatment respiratory rate, Silverman score, venous pressure, electrolytes, protein levels, and circulating levels of members of the fibrinolysin system, the placebo-treated control group and the experimental group were found to be comparable. The groups were comparable in regard to pretreatment blood pH and pCO2 as well, except that the subgroup that was treated with the enzyme preparation and expired showed a greater degree of acidosis than the other groups.
Patients in the experimental group received 5 RPMI units/kg of human plasmin activated with human urokinase (UK-plasmin) intravenously in 4 hours, plus 60 RPMI units/kg/day of enzyme by aerosol. In the placebo-treated control group, 11 of 28 infants recovered (39%) and in the UK-plasmin treated group, 23 of 32 recovered (72%). The difference is statistically significant at less than 5% level. Of the infants with a birth weight of 2 kg or less, 3 of 16 recovered in the placebo-treated control group (19%), and 10 of 16 recovered in the UK-plasmin treated group (63%); the difference is statistically significant at less than 5% level. No clinical side effects were seen in any of the patients.
Autopsy findings indicated a high degree accuracy of diagnosis. The incidence of hemorrhage diagnosed at autopsy was the same in the control and experimental groups. In all but one of the patients that expired in the UK-plasmin treated group, sufficient pathologic findings were obtained to account for death regardless of the presence of hyaline membrane disease. Similar findings were obtained in 9 of 17 patients who expired in the control group.
UK-plasmin therapy of respiratory distress syndrome of infants due to hyaline membrane disease appears to be worthy of further exploration. A diagram correlates certain features of the pathophysiology of this disease and the mechanism of action of therapy.
SEROLOGICAL EVIDENCE OF RESPIRATORY VIRUS INFECTIONS IN MOSCOW CHILDREN, 1948 TO 1956
Authors: Ritova V. V. , Zhdanov V. M. , Schastny E. I. 
Published: December 2021

1. The study of sera collected between 1948 and 1956 from children with acute respiratory disease showed that outbreaks were caused by adenoviruses, parainfluenza viruses, reoviruses, rhinoviruses, ECHO viruses, and Coxsackie viruses.
2. Adenovirus infections were the most prevalent. Other viral infections were prevalent in certain years.
3. Mixed infections with two viruses constituted about 10% of all cases.
A CONTROLLED STUDY OF EARLY FLUID ADMINISTRATION ON SURVIVAL OF LOW BIRTH WEIGHT INFANTS
Authors: Cornblath Marvin , Forbes Audrey E. , Pildes Rosita S. , Luebben Gertrude , Greengard Joseph 
Published: December 2021

A controlled study was done to evaluate the effects of early administration of fluids versus 24 to 72 hours starvation on the survival at 14 days of 90 premature infants with birth weights less than 1,500 gm. Mortality was 30% in the 30 infants who were given intravenous fluids as compared to 50% in those who were given nasogastric fluids or those who were starved.
A reduction in mortality was noted in the group of infants between 765 and 1,250 gm who received parenteral fluids early (mean, 6 hours; range, 2 to 15 hours). The early administration of fluids via a nasogastric tube did not influence mortality. Since limitations to those lower-weight groups had not been included in the protocol, a further controlled study is indicated.
There was no significant difference in the 72-hour weight loss, the biochemical determinations, the morbidity, or the autopsy findings of the three groups studied.
COLD AGGLUTININS, EATON AGENT, AND RESPIRATORY INFECTIONS OF CHILDREN
Authors: Sussman Sidney J. , Magoffin Robert L. , Lennette Edwin H. , Schieble Jack 
Published: December 2021

Twenty-eight of 444 children under 4 years of age developed cold agglutinins in association with a respiratory illness. Infection with the Eaton agent, Mycoplasma pneumoniae, could not be established by C-F tests in any of these 28 cases. In 18 cases, pathogens other than the Eaton agent were implicated. A positive cold agglutinin test was of no value in making an etiologic diagnosis. Complement-fixation tests for antibody to the Eaton agent in 106 children with respiratory illnesses showed an antibody titer rise in 1 case and stationary titers in 12 others.
These observations covered a 4-year period on the pediatric inpatient service of a large metropolitan general hospital. Sporadic cases of pneumonia due to Mycoplasma pneumoniae, confirmed by C-F antibody titer rises, were observed in older children and adults in the same metropolitan area during this period, but no evidence of epidemic occurrence was observed.
The Use of THAM in the Treatment of Status Asthmaticus
Authors: STRAUSS JOSE , FINE RICHARD N. , MEDINA DAVID A. , DONNELL GEORGE N. 
Published: December 2021

The treatment of status asthmaticus (epinephrine-fast state in an acutely ill asthmatic patient) has been described frequently; however, the need to correct the respiratory and metabolic acidosis which accompanies this condition has not been emphasized. Recently such recommendations have appeared in the nonpediatric literature of this country and of Europe. The purpose of this paper is to report two pediatric patients with status asthmaticus who recovered after administration of Tris (hydroxymethyl) aminomethane (THAM), an amine alkalinizing agent.
Methods
The Astrup technique employing the ME-l equipment and Sigaard-Andersen's nomogram were used to determine pH, pCO2, Base Excess (B.E.), Buffer Base (B.B.) and Standard Bicarbonate (S.B.) of capillary blood. Serum electrolytes were determined by conventional methods. [SEE TABLE I AND II IN SOURCE PDF].
PROBABILITY, RARITY, INTEREST, AND SURPRISE
Authors: Weaver Warren 
Published: December 2021

THE unusual is often interesting. Moreover, the frequency definition of probability, which is the one consciously or tacitly used in all applications to experience, makes it clear that an improbable event means precisely a rare event. Hence, an improbable event is often interesting. But is an improbable event always interesting? We shall see that is is not. If an event actually occurs, and if its probability, as reckoned before its occurrence, is very small, is the fact of its occurrence surprising? The answer is that it may be, or it may not be.
Suppose one shuffles a pack of cards and deals off a single bridge hand of thirteen cards. The probability, as reckoned before the event, that this hand contain any thirteen specified cards is 1 divided by 635,013,559,600. Thus the probability of any one specified set of thirteen cards is, anyone would agree, very small.
When one hand of thirteen cards is dealt in this way there are, of course, precisely 635,013,559,600 different hands that can appear. All these billions of hands are, furthermore, equally likely to occur; and one of them is absolutely certain to occur every time a hand is so dealt. Thus, although any one particular hand is an improbable event, and so a rare event, no one particular hand has any right to be called a surprising event. Any hand that occurs is simply one out of a number of exactly equally likely events, some one of which was bound to happen. There is no basis for being surprised at the one that did happen, for it was precisely as likely (or as unlikely, if you will) to have happened as any other particular one.
EFFECT OF EARLY FEEDING OF SOLID FOODS ON NUTRITIVE INTAKE OF INFANTS
Authors: Guthrie Helen A. 
Published: December 2021

The early introduction of solid foods to the diet of bottle-fed infants living in a college town causes some significant changes in the nutritive content of the diet but does not increase the adequacy of the diet before 3 months as evaluated by allowances recommended for infants by the National Research Council. Diets containing solid foods provide significantly more iron and thiamine, two of the nutrients used to enrich infant cereal products. Most of the infants were receiving nutritional supplements of vitamin A and D in spite of the fact that their diets were adequate in these nutrients. The need for a supplement of ascorbic acid was evident for some infants.
The feeding of solid foods apparently had no effect on the sleeping pattern of the infants as evidenced by the number of feedings, nor did it increase the cost of the food itself. It did, however, increase the time and effort involved for the mother. Since there was no increase in the caloric content of the diet, the solid foods must have replaced milk rather then to have supplemented it, a fact confirmed by the observations of many mothers. At least 10% of the infants experienced some form of allergic reaction to solid foods.
All of the infants in the study had been introduced to solid foods by 9 weeks, 2 to 4 weeks prior to the age at which the American Academy of Pediatrics suggests that there is a rational basis from a nutritional and developmental point of view to begin supplementing the diet. Although there was no evidence of adverse reactions other then allergies to the pattern of early feeding observed in this group, there was no evidence to indicate that the infant benefitted from a nutritional standpoint.
Lack of evidence of benefits and an accumulating body of information on hazards from the early introduction of solid foods suggest a re-examination of current emphasis on the early supplementation of the milk diet in infants.
CYTOLOGIC STUDY OF URINARY SEDIMENT IN THE EVALUATION OF SEX HORMONE PRODUCTION IN CHILDHOOD
Authors: Silver Henry K. , Kirchner Robert L. 
Published: December 2021

Cytologic examination of desquamated cells in voided urine was performed in 236 children. The urinary sediment of 95% of girls and 80.5% of boys of all ages exhibited changes consistent with sex-hormone stimulation. Cytologic changes were most marked in the neonatal period and at puberty; during the first week of life, the most striking alteration occurred in low-birth-weight infants.
Our findings suggest that the quantity of sex hormones known to be produced in both sexes throughout childhood may be sufficient to cause discernible changes in cells derived from the urogenital tract.
FACTITIAL HYPERKALEMIA DUE TO ICING BEFORE ANALYSIS
Authors: Oliver T. K. , Young G. A. , Bates G. D. , Adamo J. S. 
Published: December 2021

Both adult and newborn blood plasma potassium concentration rose significantly when whole blood was kept on ice for periods of 5 hours. This appears to be due to inhibition of red cell glycolysis. The rise in plasma potassium concentration of blood from newborn infants is significantly greater than that occurring in blood from adults; this difference is a function of the neonate's increased red cell volume. Meaningful interpretation of potassium concentration, particularly in the newly born, demands that the plasma be separated immediately.
The plasma potassium concentration of normal newborns at birth does not differ significantly from adults; in this study the mean value in 20 infants was 4.81 mEq/l.
Locking the Medicine Cabinet
Authors: CROOK WILLIAM G. 
Published: December 2021

I would like to commend Dr. J. Albright Jones for his suggestion (Pediatrics, 38:124, 1966) for poison control in the pediatric age group. Certainly, parents who are willing to purchase such a box and outfit with a lock are apt to be parents whose children will rarely suffer from accidental poisoning.
But, as Dr. Jones pointed out, "there are some [familiesj who have to be urged and cajoled to protect their children properly." For this reason I would hope that Dr. Jones' suggestion might be carried one step further, so that the job would be made easy for any family who has a medicine chest in the house.
THE RESPONSE OF THE THYROID GLAND TO THYROID-STIMULATING HORMONE (TSH) IN INFANTS WITH MALNUTRITION
Authors: Beas Francisco , Mönckeberg Fernando , Horwitz Isidoro 
Published: December 2021

The thyroid response to TSH was studied in a group of 16 infants with severe marasmus and 9 normal controls of the same age.
The radioiodine uptake, the BEI, and the oxygen consumption were determined in both groups before and after the administration of a single dose of TSH.
The results suggest that the low function of thyroid gland found in marasmic infants is due mainly to a decrease of TSH stimulation but there is also a deficit of thyroid function per se.
These results are discussed in relation to previous data.
XX/XO MOSAICISM IN NINE PREADOLESCENT GIRLS
Authors: Leāo José Carneiro , Voorhess Mary L. , Schlegel Robert J. , Gardner Lytt I. 
Published: December 2021

Clinical and chromosomal studies are presented on nine preadolescent girls with XX/XO mosaicism, investigated because of short stature alone or in association with other complaints. Short stature was the sole reason for seeking medical advice in most of the cases and was the most important clue in making the diagnosis, since these patients do not have the "textbook picture" of Turner's syndrome. All nine patients were under the 3rd percentile for height. No lymphedema, web neck, or congenital heart disease was observed in the present series.
The quantitative buccal smear for sex chromatin was found to be a useful preliminary screening test in indentifying these patients. The possibility of XX/XO mosaicism should be explored in all girls with unexplained short stature.
MANAGEMENT OF INFANTS WITH VENTRICULAR SEPTAL DEFECT, A CONTROVERSY
Authors: NADAS ALEXANDER S. 
Published: December 2021

THE provocative paper by Sigmann, et al. in this issue is an excellent exposé of the activist point of view in pediatric cardiology. One cannot but congratulate the authors on their diagnostic and surgical skills. Still, being a reluctantly adopted citizen of conservative New England, this member of the Editorial Board feels called upon to present a critique of the Ann Arbor publication and to propose an alternate, more restrained, approach to treatment of infants with ventricular septal defect.
First, we ought to compare the mortality rates of Doctor Sigmann's patients who were operated on, if possible, with those they treated medically themselves and with those so managed elsewhere.
Depigmented Nevi and Tuberous Sclerosis
Authors: GARCÍA MANUEL ANADOR 
Published: December 2021

I read with real interest the paper by Drs. Arnold P. Gold and John M. Freeman on the early diagnostic values of depigmented areas of the skin in tuberous sclerosis (Pediatrics 35:1003, 1965).
In a revision of cases in this Hospital from 1960 through 1964, we found this sign in six out of seven cases. We consider this a remarkable fact, since, prior to the reference appearing in your magazine, we have found no indication as to the value of the cutaneous manifestations in the early diagnosis of the disease.
THE REVERSIBILITY OF PULMONARY HYPERTENSION IN PATIENTS WITH CYSTIC FIBROSIS
Authors: Kelminson Leslie L. , Cotton Ernest K. , Vogel John H. K. 
Published: December 2021

1. The present study was concerned with the evaluation of the acute and chronic effects of tolazoline, O2, and sleep on the pulmonary circulation of six subjects with cystic fibrosis and pulmonary hypertension.
2. Right heart catheterization was performed and observations were made at rest, after tolazoline and after O2. In four subjects, changes in pulmonary artery pressure (PAP) induced by various agents and sleep were observed for periods ranging from 3 hours to 4 days.
3. Resting mean PAP ranged from 22 to 67 mm Hg; arterial PO2 from 37 to 47 mm Hg; pH from 7.252 to 7.408, cardiac index from 2.64 to 3.91 1/min/m2, and total pulmonary resistance (TPR) from 440 to 1,330 dynes sec cm-5-m2.
4. Following tolazoline, the average mean PAP dropped from 51 to 28 mm Hg, average cardiac output rose from 3.20 to 4.08 1/min/m2; TPR dropped from 880 to normal. This decrease in PAP and TPR was achieved with little change in either pH or arterial pO2.
5. The addition of O2 to tolazoline lowered the mean PAP further by 8 mm Hg.
6. Observations were made during sleep in three of the patients. In each, there was an increase in PAP ranging from 9 to 23 mm Hg.
7. Repeated administration of tolazoline resulted both in progressively lower PAP and less tendency for its return to control level.
8. Postmortem pulmonary arteriograms and histological examination of the pulmonary vessels were performed in one patient. Despite the presence of numerous abscesses and diffuse bronchopneumonia, the pulmonary vessel was found to be relatively normal. Only medial hypertrophy of the small arterioles was seen.
9. The factors involved in the development of hypoxic pulmonary hypertension and possible mechanisms by which tolazoline effects acute and sustained reduction of PAP are discussed.
10. It is concluded that acute and repetitive pharmacologic treatment is effective in the reduction of pulmonary hypertension in patients with cystic fibrosis. Further, it is suggested that patients with cystic fibrosis and reactive pulmonary hypertension would benefit from residing at sea level.
SUBTOTAL PANCREATECTOMY IN THE MANAGEMENT OF SEVERE PERSISTENT IDIOPATHIC HYPOGLYCEMIA IN CHILDREN
Authors: Hamilton James P. , Baker Lester , Kaye Robert , Koop C. Everett 
Published: December 2021

Twelve cases of severe persistent idiopathic hypoglycemia of infancy treated by partial pancreatectomy since 1950 are reviewed. Eleven of these maintained normal blood sugars postoperatively. Six patients of this group required medical supportive treatment for various periods of time in addition to the surgery.
A total of 45 other operative cases are reviewed and presented in table form. Overall, 89% of these cases were able to achieve acceptable control of their hypoglycemia. Subtotal pancreatectomy in an infant is a technically simple procedure and should not be equated with the formidable procedure of pancreatectomy in adults.
It is concluded that partial pancreatectomy deserves a role in the treatment of severe persistent idiopathic hypoglycemia of infancy. Subtotal pancreatectomy should not be considered as an alternative to a medical regimen. It should not be entered into lightly but should be considered as one of several means which may have to be used, often simultaneously, in the vigorous treatment of these children.
MIST THERAPY
Authors: AVERY MARY ELLEN , GALINA MORTON , NACHMAN RICHARD 
Published: December 2021

AN ARTICLE on mist tent therapy in this issue opens with the remarkable comment that no objective evidence of the efficacy of mist in patients with cystic fibrosis has been published. The authors conducted a clinical study which led to the conclusion that under the conditions of their study mist was effective as judged by improvement in some aspects of pulmonary function. It may be even more remarkable to note the absence of objective evidence of the role of mist in the wide variety of circumstances in which it is commonly used on most pediatric services.
A review of some physical principles involved in water exchange, of observations on the deposition of aerosolized particles in the lung, and of the uses and hazards of mist therapy seems pertinent.
SOLVENT SNIFFING
Authors: Press Edward , Done Alan K. 
Published: December 2021

THOUSANDS of adolescents and teen-aged youngsters in many cities throughout the United States and other countries are deliberately inhaling vapors of a wide variety of volatile organic solvents in order to induce repeated states of inebriation. Although the practice itself is not new, its occurrence in epidemic proportions in many areas and the passage of legislation prohibiting the act in many cities and states in the United States have brought the problem into nationwide prominence.
Some of the solvents used (such as carbon tetrachloride, trichlorethane, benzene, and acetone) have been implicated in previous industrial exposures as the cause of serious toxic effects, including fatalities. The possibility of similar ill effects from these and other solvents when used in much higher concentrations at shorter, but frequently repeated intervals as currently practiced, is one that has been raised repeatedly. Consequently, the Panel on Household and Economic Chemicals of the American Medical Association's Section on Adverse Reactions (Council on Drugs) invited this appraisal of the problem. Included is an assessment of the potential problem from the standpoints of incidence, acute effects, behavioral difficulties, possible chronic or cumulative toxic effects, and possible remedial measures.
SOURCES OF INFORMATION
This report summarizes the authors' investigations of the problem over the period of the last several years. In addition, it includes a review of published reports, the results of extensive discussions and correspondence with medical and law enforcement personnel in many areas of the United States, Sweden, and elsewhere. Also direct medical and hospital studies of a sample of habitual sniffers by one of us (A.K.D.) in Salt Lake City, Utah, and a detailed comparison of four other similar studies and a personal experiment on simultaneous electroencephalographic tracings and blood level measurements during inhalation by the other (E.P.).