bluuebunny/crossref_metadata_2025_split huggingface dataset:
GENETICS OF THE MECKEL SYNDROME (DYSENCEPHALIA SPLANCHNOCYSTICA)
Authors: Hsia Y. E. , Bratu M. , Herbordt A. 
Published: December 2021

Seven cases in two families are reported of the Meckel syndrome; two sets of male identical twins in one family, and three affected siblings in another. The key features of this syndrome are occipital encephalocele, cleft lip and palate, polydactyly, and polycystic kidneys. Additional malformations frequently found include microcephaly, micrognathia, absence of the olfactory lobes, microphthalmia, absent pituitary with consequent thyroid hypoplasia and suprarenal agenesis, short-limbed dwarfism, microgenitalia or ambiguous genitalia, and accessory spleen. No chromosomal abnormality has been found in this syndrome, although it is otherwise remarkably similar to the trisomy D1 syndrome.
More than 50 cases have been described in the literature that are recognizable as having the Meckel syndrome. Almost half of these were familial, being clustered in sibships. Eight cases tested have all had normal chromosomes. The concordant involvement of identicial twins, and consanguinity of some parents provide further evidence strongly supporting the hypothesis that the Meckel syndrome is a recessively inherited condition which is determined by homozygous expression of a single autosomal gene.
Recognition of this syndrome is important for proper genetic counseling of parents having this risk.
This mutant gene may disrupt normal fetal development by affecting an embryonic organizer system.
"SUICIDE" AS SEEN IN POISON CONTROL CENTERS
Authors: McIntire Matilda S. , Angle Carol R. 
Published: December 2021

Of 1,103 cases of poisoning, ages 6 to 18, admitted to 50 poison control centers during 1 year, 13% were considered unintentional, 13% "trips," 26% suicide attempts, and 48% suicide gestures or affect reactions. The youngest age group, 6 to 10, is 63% male, 40% Negro, and about one-half give a history of precipitating stress or current or prior referral for behavior problems. The abrupt increase in self-poisoning in girls at age 12 peaks at age 16, while male paisonings continue to increase with age. An admittedly immature concept of death was retained by 16% of the 17 to 18-year-olds. In the five deaths (mortality 1: 220 hospitalized self-poisonings), lethality of intent was presumed low with death the result of a toxicologic mishap.
The estimate of about 115,000 self-poisonings annually in the United States, ages 6 to 18, defines a mental health problem of the first magnitude, but open to epidemiologic analysis by relatively simple techniques.
UMBILICAL ARTERY CATHETERIZATION: DEMONSTRATION OF ARTERIAL THROMBOSIS BY AORTOGRAPHY
Authors: Neal William A. , Reynolds John W. , Jarvis Charles W. , Williams Hugh J. 
Published: December 2021

One hundred consecutive admissions to a neonatal intensive care unit were included in a prospective study designed to ascertain the frequency of clinically silent thrombosis among infants with umbilical artery catheters.
Eighteen of 19 infants (95%) had distinct arterial thrombus formation demonstrated by hand injection of contrast media at the time of catheter removal. In addition, 7 of the 12 patients (58%) who died were found to have clots present by necropsy.
Only one patient had clinical evidence of vascular occlusion prior to the pullout aortogram, though a second infant became symptomatic 4 hours after the study and subsequently died.
Careful selection of patients in whom umbilical artery catheters are inserted is emphasized because of the unexpectedly high incidence of arterial thrombosis associated with this procedure.
URINARY TRACT INFECTIONS IN CHILDREN I. FEMALES AGED 2 THROUGH 14, FIRST TWO INFECTIONS
Authors: Cohen Matthew 
Published: December 2021

In January of 1964 a 20-year prospective study of urinary tract infections was begun at the Southern California Permanente Medical Group in Panorama City, California. This paper is concerned with 368 females, aged 2 through 14 years, who had their first infection diagnosed between January 1964 and August 31, 1969.
No benefit could be demonstrated to Sulfisoxazole therapy exceeding 2 weeks for the first infection and 6 weeks for the second infection nor to treating with ampicillin instead of sulfisoxazole.
A higher recurrence rate was found in those patients who, at the time of the first infection, had minimal abnormalities on urinalysis, infection with an organism other than E. coli, fever, or no symptoms.
Recurrences of 40% and 60% were diagnosed after the first two infections respectively. Usually the patients with a recurrent infection were asymptomatic. It is therefore felt that the physician must take an active part in informing the patient of the need to return and contacting her if she fails to do so. The follow-up period should extend for at least 6 years after the last infection.
USE OF d-AMPHETAMINE AND RELATED CENTRAL NERVOUS SYSTEM STIMULANTS IN CHILDREN
Authors: Yaffe Sumner J. , Bierman Charles W. , Cann Howard M. , Gold Arnold P. , Kenny Frederic M. , Riley Harris D. , Schafer Irwin , Stern Leo , Weiss Charles F. , Chudzik Gregory , Shirkey Harry C. , Soyka Lester F. , Ballin John C. , Done Alan K. , Farchione Louis , Leger Jacques , Lockhart Jean D. , Sawchuk Steven , Simopoulos Artemis P. 
Published: December 2021

The abuse of amphetamines has become a problem of international significance. Japan was the first country to recognize this problem, and by 1954 there were an estimated 500,000 to 600,000 abusers in Japan. More than ten years ago Japan banned the use of amphetamines. The United Kingdom restricted distribution of amphetamines to hospital pharmacies in 1968. Sweden categorized amphetamine as a narcotic in 1944 because of abuse; and in 1965 phenmetrazine (Preludin) and in 1968 methylphenidate (Ritalin) were removed from the market. Patients now requiring amphetamines are registered with the government. Sweden has about 10,000 drug addicts (almost all between 15 and 30 years of age) using central stimulants intravenously; this is about the same percentage of their population as the estimated percentage of heroin addicts in New York City.1 In contrast, the number of heroin and opiate addicts in Sweden is estimated to be less than 500.
In 1970, the Food and Drug Administration (FDA) responded to the problem of amphetamine abuse in the United States by limiting the package insert labeling for amphetamines to three indications: narcolepsy, hyperkinesis in children, and the short-term treatment of obesity. Currently, the latter indication is being reviewed and may no longer be valid.
Among the related agents there is some specificity in labeling, e.g., methylphenidate is approved for use in adults with mild depression, narcolepsy at any age, and children with minimal brain dysfunction but not obesity; phenmetrazine for use only in obesity, etc. However, in the broad view there is a similarity in the pharmacologic properties, side effects, and abuse liability of dextro-amphetamine, methamphetamine, methylphenidate, and phenmetrazine.
NURSERY EPIDEMIC CAUSED BY A NONTYPABLE GRAY COLONY VARIANT OF STAPHYLOCOCCUS AUREUS
Authors: Ramamurthy Rajam S. , Pildes Rosita S. , Gorbach Sherwood L. 
Published: December 2021

A nursery epidemic caused by coagulase-positive Staphylococcus aureus and involving 32 infants is reported. The epidemic occurred between March 16, 1971, and May 3, 1971, in spite of hexachlorophene bathing. No breaks in technique could be identified. The causative pathogen was sensitive to hexachlorophene in a 1:10,000 dilution and its morphological characteristics were unique. The colonies, on human blood agar, were intensely hemolytic and had a grayish white color. These characteristics proved useful markers for identification of the carriers.
The epidemic was brought under control by several measures. Reinforcement of nursery techniques was ineffective. Nasal antibiotics decreased the number of cases but the persistent carriers required prolonged treatment. Cohort system was effective, but complete eradication of the pathogenic organism occurred, only after the carriers were removed from the nursery.
The emergence of a virulent strain of S. aureus was considered an important factor in this epidemic. The maintenance of a low colonization rate may, by itself, be inadequate in the prevention of epidemics caused by a virulent strain.
A LONGITUDINAL STUDY OF THE GROWTH OF LOW BIRTH WEIGHT INFANTS
Authors: Cruise Mary O. 
Published: December 2021

Physicians may need to consider the growth of children–especially those of unusual size for age–from two different viewpoints. Most pediatricians are used to thinking of what may be called distance growth: how "normal" is a child's actual length at 2 years? The other aspect, velocity growth, expresses the rate or speed at which the child has grown. This may be equal for children of corresponding ages, whereas their distance growth may vary greatly. Failure of growth at a normal rate in one child should be investigated whereas another child with horizontal growth measurement outside ± 2 SD, but with normal velocity growth may require no investigation.
Data from this study provide not only mean measurements for weight, length, and head circumference of low birth weight infants who are grouped by sex and gestational age, but also provide velocity growth from birth through 3 years of age. Preterm infants (gestational age &lt; 37 weeks) had greater velocity growth rates than small-for-date infants (full-term, low birth weight). At 1, 2, and 3 years the SFD infants had the smallest mean measurements of all the study groups of low birth weight infants. Comparative full-term infants were larger than low birth weight infants from birth through 3 years of age.
TWO CURRENT POISONINGS: TRICYCLIC DRUGS AND METHADONE
Authors: Arena Jay M. 
Published: December 2021

One of the most serious types of poisoning, occurring with increasing frequency in children as well as in adults in the United States, arises from the wide usage and increased accessibility of antidepressant drugs, specifically the tricyclic compounds. These include imipramine (Tofranil), desipramine (Norpramin, Pertofrane), amitriptyline (Elavil), nortriptyline (Aventyl), protriptyline (Vivactyl), trimipramine (Surmontil), and opipramol. Doxepin hydrochloride, a dibenzoxepin compound, is structured similarly to amitriptyline except for the replacement of one carbon atom by an oxygen atom in the tncyclic ring.
There have been a number of serious cardiac arrhythmias, conduction disturbances, and heart blocks associated with intoxications from tricyclic antidepressant tranquilizers.
The most commonly seen disturbances of cardiac function from overdoses of these drugs are supraventricular tachycardia, widening of the QRS complexes, depression of the S-T segments, and abnormal T waves. Disturbances of rhythm and conduction which have been reported include atrial and ventricular tachycardias, flutters, and fibrillations, wandering pacemaker, multifocal extrasystoles (ectopic beats), complete or partial atrioventricular and intraventricular blocks, and cardiac arrest.
In addition to the cardiac disturbances, intoxication with tricyclic antidepressants also produces the peripheral signs of atropinism and symptoms of vomiting, thirst, drowsiness or dizziness, and signs of mydriasis, nystagmus, agitation or stupor, bowel and bladder paralysis, muscle rigidity, tremors, hyperreflexia, athetoid or clonic movements, convulsions, hypertension or hypotension, depressed respiration, cyanosis, hyperthermia or hypothermia, and coma. The most serious phase appears to be the first 12 hours, but during recovery a deep lethargy may alternate with tremors, agitation, delirium, hallucinations, mental confusion, and persistent insomnia.
Letters to the Editor
Authors: Baden Melvin , Stern Leo 
Published: December 2021

Drs. Farrell and Zachman have presented a very lucid explanation of how the corticosteroids might act as an enzyme inducer in the therapy of RDS. Nevertheless, before one could recommend such usage in the postnatal period on a wholesale basis, data indicative of its efficacy are required and they have indeed wisely pointed out the need for such confirmation in their letter.
With respect to their interpretation of the findings in our paper,1 we should like to reiterate the following which are worthy of careful consideration by any prospective user.
SYSTEMIC HYPERTENSION FOLLOWING OCULAR ADMINISTRATION OF 10% PHENYLEPHRINE IN THE NEONATE
Authors: Borromeo-McGrail Virginia , Bordiuk Joseph , Keitel Hans 
Published: December 2021

The effect of ophthalmic instillation of 10% phenylephrine on systemic blood pressure in the neonate has not been previously evaluated, despite reports of adverse reactions. The effect of 10% and 2½% phenylephrine eye drops in healthy low birth weight neonates was compared in a double-blind study. Blood pressure showed a rise of 12 to 16 mm Hg in systole and 10 to 14 mm Hg in diastole following administration of 10% phenylephrine. Blood pressure remained stable in infants receiving 2½% phenylephrine.
Consistent, although more variable, increases in blood pressure were noted in eight low birth weight infants given 10% phenylephrine eye drops in a nonblind study. Complete mydriasis was achieved with 2½% phenylephrine as well as with 10%.
Neither concentration had an effect on pulse or respiratory rates. Cutaneous administration of either 10% or 2½% phenylephrine to abdominal skin produced local blanching but no systemic effect.
To avoid potentially dangerous sequelae from iatrogenic hypertension, it is recommended that not more than one drop of 2½% phenylephrine in each eye be administered to neonates for mydriasis.
PLASMA GLUCOSE AND INSULIN RESPONSES TO ORAL GLUCOSE WITH CHRONIC DIPHENYLHYDANTOIN THERAPY
Authors: Cummings Nancy P. , Rosenbloom Arlan L. , Kohler William C. , Wilder B. J. 
Published: December 2021

The hyperglycemic effect of massive doses of diphenylhydantoin (DPH) was noted in rabbits in 1965.1 The following year Klein2 described a patient with DPH-induced hyperglycemia: a child of 20 months who had ingested 800 mg of the drug during 24 hours. The peak plasma glucose level was 726 mg/100 ml and returned to normal a few days after the cessation of DPH. Later, a dose of 100 mg of DPH was shown not to affect oral glucose tolerance in this child. Dahl3 described a 47-yearold-man who increased his own anticonvulsant dose of DPH to 700 mg daily; he had a blood glucose level of 230 mg/100 ml two hours after eating. Two days after reduction of the dose to 200 mg daily, the blood glucose was normal and while he was taking 300 mg daily he had a normal oral glucose tolerance test and no side effects. A 26-month-old retarded child receiving 300 mg DPH daily was the next to be reported to have hyperglycemia.1 Two fatal instances of hyperosmolar coma related to DPH induced hyperglycemia were described in seriously ill elderly patients given large amounts of DPH intravenously.5 Peters and Samaan6 observed relative hypoinsulinism and abnormal oral glucose tolerance four days after stopping DPH (500 to 700 mg daily) in a 17-year-old girl with lethargy, headache, and nystagmus. Her plasma DPH level at the time of glucose tolerance testing was 65µg/ml.
Several studies have shown the dose dependency of the hyperglycemic and hypoinsulinemic effect of DPH. In a 42-year-old woman blood glucose was elevated on 400 mg/day.7
THE THIRTEEN-YEAR-OLD LOUISA MAY ALCOTT WRITES OF HER WISH FOR A ROOM OF HER OWN
Authors: C. T. E. 
Published: December 2021

One of Louisa Alcott's (1832-1888) strongest desires as a young adolescent was for a room of her own. The following is taken from her Journal and was written when she was 13 years old.
Fruitlands
March, 1846,–I have at last got the little room I have wanted so long, and am very happy about it. It does me good to be alone, and mother has made it very pretty and neat for me. My workbasket and desk are by the window, and my closet is full of dried herbs that smell very nice. The door that opens into the garden will be very pretty in summer, and I can run off to the woods when I like.
I have made a plan for my life, as I am in my teens, and no more a child. I am old for my age, and don't care much for girl's things. People think I'm wild and queer; but mother understands and helps me. I have not told any one about my plan; but I'm going to be good. I've made so many resolutions, and written sad notes, and cried over my sins, and it does n't (sic) seem to do any good! Now I'm going to work really, for I feel a true desire to improve, and be a help and comfort, not a care and sorrow, to my dear mother.1
PSYCHOLOGICAL AND EDUCATIONAL SEQUELAE OF PREMATURITY
Authors: Rubin Rosalyn A. , Rosenblatt Cynthia , Balow Bruce 
Published: December 2021

Psychological and educational correlates of prematurity were assessed in a prospective longitudinal study of 241 infants classified by birth weight, gestational age, and sex. Eighty per cent of the 78 low birth weight (LBW) infants weighed between 2,001 and 2,500 gm; three weighed less than 1,500 gm.
It was found that (1) LBW was associated with a number of indicators of abnormal conditions during the neonatal period; (2) birth weight rather than gestational age was the major correlate of psychological and educational impairment; (3) LBW males and small-for-date (SFD) subjects of both sexes had a significantly higher incidence of school problems warranting special school placement and special school services than did full birth weight (FBW) full-term control subjects; (4) LBW subjects scored lower than FBW subjects on all measures of mental development, language development, school readiness, and academic achievement through 7 years of age; (5) while there were no sex differences among LBW subjects on objective measures of psychological development and academic achievement, males had a higher incidence of school identified problems than did females; and (6) LBW and preterm infants did not differ significantly from control subjects on socioeconomic status.
It was concluded that low birth weight preterm males and small-for-date subjects of both sexes constitute a "high risk" population in terms of eventual impairment of school functioning.
RICKETS AND MYOPATHY CURED BY REMOVAL OF A NONOSSIFYING FIBROMA OF BONE
Authors: Pollack James A. , Schiller Alan L. , Crawford John D. 
Published: December 2021

A 9-year-old boy presented with progressive muscle weakness, waddling gait, and diminishing growth velocity of 16 months' duration.
Radiographs demonstrated active rickets and a lytic lesion of the distal radius. Normocalcemia (9.6 mg/100 ml), hypophosphatemia (1.7 mg/100 ml), and elevated alkaline phosphatase (31.6 Bodansky units) suggested X-linked resistant rickets but bone structure in films taken prior to age 7 was normal. No other family members had hypophosphatemia. Glomerular function was normal; generalized aminoaciduria and glycosuria were absent, but urinary glycine was elevated. Normal serum parathyroid hormone levels were suppressed by induced hypercalcemia. Chemical improvement and radiological healing were not obtained on vitamin D2 in doses to 200,000 units/day, but occurred promptly, with complete symptomatic relief, when oral phosphate, 1,250 mg daily, was added. The lytic radial lesion, initially not excised because of its benign radiological appearance and proximity to the metaphysis, responded to therapy with increasing sclerosis. Nonetheless, even after three years of medical treatment, chemical rickets exacerbated when vitamin D, but not phosphate, was omitted. When the patient was 12 years old, all therapy was discontinued and the lesion, a benign, nonossifying fibroma of bone, was excised. Within four days all biochemical indices of rickets returned to normal, except for the hyperglycinuria which declined more slowly over three weeks. One year after surgery the patient was without clinical or biochemical evidence of recurrent disease. The data suggest the acquired, hypophosphatemic rickets with myopathy was induced by elaboration in the tumor of a humoral substance, not parathyroid hormone, the nature of which is unknown.
Thanatophoric Dwarfism–Maybe Genetic but not Polygenic
Authors: Hall Judith G. 
Published: December 2021

I cannot agree with the conclusions reached by Pena and Goodman1 that thanatophoric dwarfism is a polygenic condition. They imply that thanatophoric dwarfism is a homogenous entity. If one reviews the cases cited by them, a wide range of severity of bony involvement and body proportions is seen. Conditions with different etiologies are probably represented in the cases published thus far as thanatophoric dwarfism.
In order to establish the genetic nature and recurrence risk of thanatophoric dwarfism, Pena and Goodman cite reports of two families2,3 in each of which parents were normal and two sibs were affected.
IMMUNIZATION AGAINST HEMOPHILUS INFLUENZAE
Authors: Mortimer Edward A. 
Published: December 2021

This issue contains a description of progress toward the development of a vaccine for Hemophilus influenzae, type b.1 The present status of this vaccine and the successive steps that must be taken to determine the safety and efficacy of any such product before it can be recommended for use are outlined, and a description of the antigenic anatomy of H. influenzae is included. Acquaintance with this information should be of value to pediatricians in the not-too-distant future when immunization against this organism may become available.
Why should attempts be made to develop a vaccine against H. influenzae? Review of the literature suggests three reasons: the frequency of meningitis due to this organism, the apparent increase in that frequency, and the severity of the disease in spite of current treatment.
PLASMA CORTICOSTEROIDS IN INFANTS WITH THE RESPIRATORY DISTRESS SYNDROME
Authors: Baden Melvin , Bauer Charles R. , Colle Eleanor , Klein George , Papageorgiou Apostolos , Stern Leo 
Published: December 2021

Plasma total corticosteroid concentrations were measured in 44 premature infants with the respiratory distress syndrome (RDS). Further assay of serum concentrations of cortisol, cortisone, corticosterone, corticosterone sulfate, and 11-deoxycorticosterone sufate was carried out in 24 of these infants and in 10 healthy, nonstressed premature controls. Infants with RDS had higher concentrations of cortisol (22.1±4.3µg/100 ml) and corticosterone sulfate (7.7 ± 0.8 µg/100 ml) than control infants (6.6 ± 0.8 µg/100 ml, 1.2 ± 0.3 µg/100 ml, and 4.2 ± 0.4 µg/100 ml, respectively).
A significant correlation was found with the five-minute Apgar score (negative correlation, p = 0.005) and a possible correlation with the A-aDO2 gradient. Among premature infants with RDS, those of 32 weeks' gestation or less had higher concentrations of cortisol (28.3 ± 23.6 µg/100 ml) and corticosterone (3.9 ± 2.7 µg/100 ml) than those of more than 32 weeks (11.5 ± 5.4 µg/100 ml and 1.7 ± 0.7 µg/100 ml, respectively), suggesting either elevated response to stress or a diminished ability to metabolize these compounds.