bluuebunny/crossref_metadata_2025_split huggingface dataset:
STATEMENT ON TREATMENT OF PHENYLKETONURIA COMMITTEE ON THE HANDICAPPED CHILD
Authors:   COMMITTEE ON THE HANDICAPPED CHILD
Published: December 2021

IN RESPONSE to many requests from individuals and agencies, the following statement on the present status of treatment of phenylketonuria (PKU) has been prepared. The Committee on Fetus and Newborn has reviewed the present status of neonatal screening for inborn errors of metabolism (e.g., PKU and related problems) and is reporting separately.
There is considerable discrepancy of opinion regarding the treatment of phenylketonuria. The enthusiasts say that with adequate mass screening, diagnosis, and early treatment, phenylketonuria can be eliminated as a cause of mental retardation; the doubters believe that there is need to improve screening procedures and that the efficacy of treatment leaves much to be desired.
AREAS OF AGREEMENT ON TREATMENT
In spite of discrepancies in the available data, certain facts appear to warrant acceptance, namely:
1. If PKU is detected early, and the infant is started on the proper diet before 6 months of age, and then is "adequately" maintained, the child usually will demonstrate borderline to average intelligence at 5 years of age. The earlier treatment is begun, in general, the better the result.
2. For the infant being treated with a diet low in phenylalanine, the acceptable concentration of phenylalanine in the serum probably lies above 3 mg/100 ml and below 8 mg/100 ml. Some insist that it be kept below 4-6 mg/100 ml. Concentrations over 12 mg/100 ml are almost certainly too high to achieve best results.
3. For optimum results the diet must be maintained rigidly and constantly, and at the same time the parents must also offer the child the usual affection, stimulation, discipline, and security necessary for normal behavioral development.
SERUM BUTANOL-EXTRACTABLE IODINE CONCENTRATIONS IN PREMATURES
Authors: Marks Alan N. , Man Evelyn B. 
Published: December 2021

Normal, full-term neonates have the highest circulating thyroxine-like iodine concentrations observed during the life span of humans (7.2-15.2 µg/100 ml) These elevated serum Butanol-Extractable Iodine concentrations have also occurred in neonates over 2,500 gm in birth weight who required exchange transfusions.
Data are presented on low BEI's of 20 neonates under 2,000 gm in birth weight in a series of 201 BEI measurements on 187 newborns. Mean BEI values increased progressively through 500 gm increments in birth weight from the 2,000-2,500 gm to the 3,500-4,000 gm group, without further rise for infants over 4,000 gm. Requirement of thyroid hormones has been discussed in relation to development of the central nervous system.
PRENATAL TREATMENT OF ERYTHROBLASTOSIS FETALIS FOLLOWING HYSTEROTOMY
Authors: Adamsons Karlis , Freda Vincent J. , James L. Stanley , Towell Molly E. 
Published: December 2021

In recent years considerable progress has been made in diagnosis and treatment of the erythroblastotic fetus. Many aspects of the problem, however, remain to be solved. Perhaps the most urgent one is earlier diagnosis: delaying treatment until demise is imminent is unlikely to lead to optimal outcome. Interference in the transfer of bilirubin across the placenta is probably a late phenomenon, present only when placental perfusion is impaired and the diffusion distance is increased by stromal edema of villi. Hence, the currently accepted indications for operative intervention are present only when the fetus has been severely affected for some time. It is possible that changes in fetal heart rate, either spontaneous or in response to a given stimulus, might reveal an incipient anemia not yet reflected in the composition of amniotic fluid. Determination of the excretion pattern of chorionic gonadotropin might also be of value. Changes in the appearance of placental villi obtained by needle biopsy and examined by phase contrast microscopy have been helpful in diagnosing impending hydrops. The benefits of this procedure, however, must be weighed against the potential danger of increasing maternal sensitization.
Optimal technique and timing for administration of red cells remain to be determined. Different approaches may be preferable at various gestational ages. Close to the time of extrauterine viability and in the absence of hydrops the method introduced by Liley may prove to be the most satisfactory. Earlier in gestation techniques which provide the fetus with an indwelling catheter, either intravascular or intraperitoneal, probably hold greater promise. Bone marrow as the site of infusion might offer advantages and should also be considered. Whether the insertion of a catheter is accomplished better during hysterotomy or by means of a transabdominal needle puncture with the aid of a fluoroscope or amnioscope, remains to be determined. For the hydropic fetus near term, exchange transfusion in utero might be lifesaving.
Editor'S Note
Authors: Dodge Warren F. 
Published: December 2021

Dr. Dodge commented as follows:
Thank you for the opportunity to comment upon the letter from Dr. McCrory. There appears to be no major disagreement between us n the treatment of infants with chronic renal osteodystrophy. Our reasoning in submitting his short report was not to recommend dietary estriction of phosphorus as the sole means of therapy, but, rather, to once again call attention to its inherent dangers if pursued too zealously.
Most investigators appear to be in agreement hat renal osteodystrophy may accompany chronic renal insufficiency.
THE PLASMA FREE FATTY ACID COMPOSITION AND BLOOD GLUCOSE OF NORMAL AND DIABETIC PREGNANT WOMEN AND OF THEIR NEWBORNS
Authors: Chen Chiung H. , Adam Peter A. J. , Laskowski Donald E. , McCann Michael L. , Schwartz Robert 
Published: December 2021

The blood glucose and plasma FFA content and composition of the newborns of normal women, gestational diabetics, and insulin-dependent diabetics were analyzed. Normal maternal FFA levels were twice the fetal values; by 2 hours of age FFA increased fourfold over initial values in normal infants, while infants of gestational diabetics had a threefold rise, and those of insulin dependent diabetics only twofold. Blood sugar at the same time decreased, and there was an inverse relationship between blood glucose in the normal infants and FFA. This relationship is altered in the infant of the diabetic mother.
Gas chromatography of plasma indicated a higher ratio of unsaturated to saturated FFA in mothers compared to infants. No pattern differences were noted between the normal and diabetic mothers, except that the latter had higher oleic acid. In the initial 2 hours of life, the patterns changed, although no differences were noted between infants of diabetic mothers and normals.
The observations suggest: (1) The metabolic interrelationships of glucose and FFA in the normal infant are different from those in the infant of the diabetic mother. The latter infant has a state of physiological hyperinsulinism. (2) Plasma FFA in the fetus is derived from both fetal fat synthesis as well as placental transfer. (3) The infant of the diabetic mother does not differ from the normal as regards FFA composition.
SURVIVAL TIME OF CIRCULATING LONG-ACTING THYROID STIMULATOR IN NEONATAL THYROTOXICOSIS: IMPLICATIONS FOR DIAGNOSIS AND THERAPY OF THE DISORDER
Authors: Sunshine Philip , Kusumoto Howard , Kriss Joseph P. 
Published: December 2021

We have described an infant with neonatal thyrotoxicosis who developed supraventricular tachycandia, goiter, and periorbital edema. Both the baby's and mother's sera contained the long-acting thyroid stimulator (LATS). While the level of LATS remained unchanged in the serum of the mother, it rapidly fell in the serum of the infant. The biological half-life of LATS in the infant's serum was calculated to be 6 days, and by the age of 21 days no LATS activity could be detected. Based on these findings, antithyroidal treatment which had been instituted soon after birth was discontinued on the thirty-eighth day without recurrence of signs or symptoms of thyrotoxicosis.
In addition to conventional antithyroid therapy for thyrotoxicosis, exchange transfusion and prenatal corticosteroid therapy of the mother may be considered when neonatal or maternal serum LATS titers, respectively, are very high.
GROWTH HORMONE
Authors: Root Allen 
Published: December 2021

Growth hormone influences protein, fat, carbohydrate, and mineral metabolism. It promotes nitrogen retention, growth of cartilage, transportation of amino acids through the cell wall, and incorporation of amino acids into protein. This factor mobilizes free fatty acids from adipose tissue and increases the serum concentration of these substances; long-term administration of this hormone is followed by depletion of body fat stores and inhibition of fatty acid synthesis. In diabetic subjects growth hormone administration is followed by hyperglycemia, glycosuria, and ketosis; its effect on carbohydrate metabolism in normal subjcets is more subtle. Sodium, potassium, and inorganic phosphate are retained following the administration of growth hormone. Hypercalciuria also accompanies such treatment, an effect mediated through the parathyroid glands.
Human growth hormone may be detected in the serum through the use of the radioimmunoassay. The hypothalamus is intimately involved with the control of the secretion and release of growth hormone from the pituitary. There is a correlation between the availability of glucose for metabolism and the plasma concentration of growth hormone; when glucose is unavailable growth hormone is released in order to provide a substitute source of energy, fatty acids.
The administration of growth hormone to the patient with hypopituitarism is followed by growth in many instances, but it has not usually been effective in promoting growth in individuals with other abnormalities. Acquired resistance to the effect of growth hormone is accompanied by the development of antibodies directed against this protein.
School Health Services
Authors: McLendon Prestion A. 
Published: December 2021

Those who have read the article of the late Dr. H. H. Mitchell (edited by Dr. Frazier) and published in Pediatrics, June, 1965, cannot help but be grateful for his long and determinded efforts in the cause of quality school health services to children. His efforts deserve far greater recognition than was given during his lifetime.
His review of the progress of school health services, as they have developed, is clear and definitive for authoritative reference, and reflects commendation on the Academy for its part during its short consideration of this most important national problem.
Pulmonary Vasoconstriction and the Pharmacology of Respiratory Distress
Authors: Defoe Edward C. 
Published: December 2021

The stimulating controversy over the R.D.S. problem between Dr. Ivanhoe and Dr. Clements and associates (Pediatrics, 36:451, 1965) prompts expression of a few additional thoughts.
Through a proponent of the theory that the basic problem in these infants is lack of normal sympathetic neurohormonal activity at time of initial inspiratory efforts, I must agree with Dr. Clements that direct evidence for such a deficiency is tenuous. However, the evidnce cited1 against the possibility is questionable.
Teen Skin, by Gordon C. Sauer, M.D. Springfield, Illinois: Charles C Thomas, 1965, 57 pp., $3.75
Authors: Perlman Henry Harris 
Published: December 2021

There has been a long-felt need for a "down-to-earth" manual discussing the cause, clinical course, the manifestations and management of "teen-age" acne not just for the average person unfamiliar with medical terms; but also for the dermatologist, the pediatrician, and the general practitioner, who all too often cannot find ample time to discuss all phases of skin care with the patients. Dr. Sauer, a wellknown dermatologist, has admirably filled this gap.
In his preface, Dr. Sauer states that "teenagers are hungry for answers to their questions about skin problems."
Les Chromosomes Humains (Caryotype Normal et Variations Pathologiques) by Raymond Turpin and Jerome Lejeune. Paris: Gautier-Villars, 1965, 535 pp. 100 figures, 54 F
Authors: Migeon Barbara Ruben 
Published: December 2021

From two of the pioneers in human cytogenetics comes an extensive discussion of the normal human karyotype and the known pathological variations in these karyotypes. Well aware of the difficulties inherent in critically reviewing papers pertaining to their subject before all data are available, the authors, nevertheless, wish to transmit to physicians and biologists the information which has accumulated to date.
The first part of the book consists of the necessary preliminary discussions by Lejeune: (1) an enumeration of observations relating to the number of human chromosome prior to 1956 including those of Tijo and Levan, (2) a description of tissue culture and cytological techniques requisite to the study of chromosome in various tissues, and (3) a definition of the normal karyotype with several of its nonpathological variations.
The Child Who Does Not Talk-Clinics in Develpmental Medicine 13, edited by Catherine Renfrew and Kevin Murphy. London, England: The Spastics Society Medical Education and Information Unit in Association with Heinemann Medical Books Ltd., 1964, 206 pp., $5.00
Authors: Pickering Ruth 
Published: December 2021

Communication being crucial to the human way of life, and considered by some authorities to be the most recent evolutionary development, it is significant that The National Spastics Society held one of their Clinics in Developmental Medicine on this problem.
The Child Who Does Not Talk is the report of an International Study Group on the Development and the Disorder of Hearing, Language and Speech in children held in St. Mary's College, Durham, England, in 1963.
HEREDITARY ANGIONEUROTIC EDEMA: A CLINICAL SURVEY
Authors: Donaldson Virginia H. , Rosen Fred S. 
Published: December 2021

Inasmuch as hereditary angioneurotic edema has generally been overlooked in the pediatric literature, it is hoped that this report may alert pediatricians to the possibiity of the existence of the disease in children of families with appropriate histories. It is likely that the symptomatology will not be severe in childhood; nonetheless, swellings and abdominal attacks do occur. Most important, the possibility that abdominal surgery will be helpful during bouts of abdominal pain due to hereditary angioneurotic edema should be considered unlikely. Markedly edematous viscera may provide technical difficulty during surgery, and undue physical trauma is not helpful.
Hereditary angioneurotic edema differs from other non-allergic angioneurotic edema in that the tendency to attacks of edema is inherited as an autosomal dominant trait and swellings tend to be much more severe. The failure of anti-allergic medications and lack of evidence for allergy as a cause of attacks of hereditary angioneurotic edema tend to differentiate this disease from the more common allergic forms. Absence of serum inhibitor of C'lesterase further characterizes hereditary angioneurotic edema. Sera from persons with non-familial allergic and non-allergic angioneurotic edema have contained normal or greater than normal amounts of this inhibitor.7
In view of the definition of biochemical alterations in the blood of patients with hereditary angioneurotic edema and the possibility of identifying affected individuals before the onset of symptoms, it is to be hoped that a rational means for the prevention of symptoms can be devised.
ENZYME INDUCTION IN A CASE OF GLYCOGEN STORAGE DISEASE
Authors: Moses Shimon W. , Levin Stanley , Chayoth Reuben , Steinitz Kurt 
Published: December 2021

In cases of glycogen storage disease Type III, low levels of liver glucose-6-phosphatase activity have frequently been found in addition to the complete absence of amylo-l, 6-glucosidase activity. Glucose-6-phosphatase regulates a key reaction in the homeostatic control of gluconeogenesis. It belongs to the group of adaptive enzymes, and it has been shown in rats that its activity can be induced by steroids and suppressed by insulin. Increasing evidence is accumulating to indicate that the hormonal control of this enzyme activity affects de novo synthesis at the gene level.
The subnormal activity of glucose-6-phosphatase in cases of amylo-1, 6-glucosidase deficiency could be the result of a double enzyme deficiency due to two structural gene defects, or secondary to a regulatory gene disorder resulting in suppression of glucose-6-phosphatase biosynthesis. If the low levels of glucose-6-phosphatase activity result from a structural gene mutation, hormonal induction of enzyme biosynthesis would be unlikely; whereas, if the markedly depressed activity of this enzyme is due to abnormal control affecting the regulation of enzyme synthesis, hormonal induction may be possible.
A case of amylo-1, 6-glucosidase type A is presented in which, in addition to complete absence of the debrancher enzyme, exceedingly low values of liver glucose-6-phosphatase were repeatedly demonstrated. Low insulin-like activity was found in the patient's plasma, but no significant abnormality of steroid metabolism could be shown. Triamcinolone administration resulted in fourfold increase of liver glucose-6-phosphatase activity. This enzyme induction by Triamcinolone suggests that the markedly reduced levels of glucose-6-phosphatase activity prior to steroid administration were due to a regulatory disorder of enzyme biosynthesis and not to a structural gene defect. It is suggested that the Triamcinolone induction test can be used in similar situations as a diagnostic tool to differentiate between a regulatory gene disorder affecting enzyme synthesis, and a structural gene defect.
HYPERGLYCINEMIA WITH KETOACIDOSIS AND LEUKOPENIA
Authors: Soriano Juan Rodriguez , Taitz Leonard S. , Finberg Laurence , Edelmann Chester M. 
Published: December 2021

Metabolic investigations are reported in a patient with the clinical and biochemical features of "idiopathic hyperglycinemia." During ketoacidosis elevated concentrations in serum of numerous amino acids were noted, especially leucine, isoleucine, valine, glycine, and lysine. Hyperammonemia was found in association with ketoacidosis.
Clinical and biochemical amelioration was induced by restriction of protein intake to 1 gm/kg or less. Although exacerbation was produced by increasing the protein intake to 1.5 gm/kg, the patient was able to tolerate as much as 3.0 gm/kg of an amino acid mixture in which leucine, isoleucine, valine, methionine, and threonine were absent.
It is postulated that this disease, in contrast to hyperglycinemia caused by a specific disorder in glycine metabolism, represents a generalized defect in utilization of amino acids resulting in excessive deamination of certain amino acids in the muscle, with consequent hyperammonemia and ketoacidosis. The nature of the defect in amino acid metabolism is unknown.
STUDIES ON INTESTINAL DIGESTION OF STARCH IN MAN
Authors: Auricchio Salvatore , Pietra Domenico Della , Vegnente Angela 
Published: December 2021

In 14 infants and small children the α-amylase activity and the carbohydrates of duodenal juice were studied after a test meal containing amylopectin.
In infants at the end of the first year of life and in small children the amylopectin is very rapidly hydrolyzed into glucose, maltose, maltotriose, and branched dextrins; these compounds are probably hydrolyzed into glucose by the action of mucosal glucosidases.
In infants 6 months old or younger the intestinal hydrolysis of amylopectin in the intestine is incomplete, with large amounts of dextrins containing more than 30 glucose units and, in the small oligosaccharides group, a decrease in the glucose and maltose content as well as an increase in the maltotetraose content.
The analysis of carbohydrates which accumulate in intestinal lumen during digestion of starch will be of value in studying pathogenesis of starch malabsorption.
AN UNUSUAL DISEASE OF THE NEWBORN DESCRIBED IN 1785
Authors: C. T. E. 
Published: December 2021

Almost two centuries ago Dr. Bassignot described an unusual skin disease of newborn infants in these words:
Seyne or Sedna, a small town in Provence is the theatre of a very peculiar disease, which attacks almost all the new born infants at that place. Some authors have spoken of it under the name of crinons or comedons; but it is known by the people of the country under the name of cees, a corruption of ceddes, a provincial term which signifies a bristle. It sometimes manifests itself within the space of twelve hours, sometimes. however, not till the end of fifteen days, or even a month.
The symptoms by which this disease is known, are a very considerable itching, which is augmented by the heat of the bed, and prevents the infant from sleeping; a perpetual agitation; an inability to suck, the child's tongue not being able to accommodate itself to the nipple; and, at last, the noise of its cries being diminished, which become hoarse, and are indeed almost extinguished. This last sign appears to be the most certain; and they in general judge of the severity of the disease by the degree to which the voice is extinguished, and by the weakness of the cries of the infant.
When, by these signs, the disease is known to be present, they proceed immediately to the cure. This consists in frictions, which are performed by the women of the country, and who are so much in the habit of treating this disease, that they do not in general call in the aid of any medical practitioner. These frictions are performed on different parts of the body, according to the state of the disease that is present. And they distinguish these stages of it, which are sometimes very distinct, sometimes united.
In the first, the diminution of the noise in crying is conjoined with a total incapability of sucking. This required friction at the upper part of the sternum, on the fore and back parts of the neck, on the cheeks towards the angle of the inferior jaw, and on the temples. In the second state of the disease, the infant still enjoys a certain facility in moving the tongue, without, however, being able properly to seize the nipple; when the arms are set at liberty they are extended, the fingers are spread out with a considerable degree of tension, or the hand is firmly clenched. This state requires friction of the fore-arm, from the shoulder to the wrist. The third stage is distinguished merely by a diminution of the cries; then frictions are directed to the arms, the shoulders, the back, and even to the calves of the legs; which probably, as well as the hands, demonstrate the existence of crinons in these parts, by some particular movement, but which has not yet been sufficiently attended to.