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Showing papers in "JAMA Pediatrics in 1968"


Journal ArticleDOI
TL;DR: Nine patients with remarkably similar facies with a previously unrecognized syndrome of valvular pulmonary stenosis and multiple extra cardiac anomalies with similar associated lesions are reported on in 1962.
Abstract: AT THE meeting of the Mid-Western Society for Pediatric Research in 1962, I reported on nine patients with remarkably similar facies with a previously unrecognized syndrome of valvular pulmonary stenosis and multiple extra cardiac anomalies.1Review of the literature at that time showed that several males resembling these patients had been reported under several different headings, most commonly under the designation of, "the male Turner syndrome."2-5There were no definite reports of similarly affected female patients. In that report I spoke in terms of a "new syndrome" in order to stress that this condition could affect not only males but also females, and that it was also clinically and etiologically different from the true Turner6syndrome. Since that time, a further eight patients, five males and three females, with valvular pulmonary stenosis and similar associated lesions have been studied. An additional two patients, one of each sex,

515 citations


Journal ArticleDOI
TL;DR: The present case is reported to provide evidence of a similar experience with a patient who also had this rare disease complex and was followed at Wilford Hall USAF Hospital from Oct 5, 1964, until his death on May 1, 1967.
Abstract: AN UNTOWARD response to radiotherapy in a patient with ataxia telangiectasia and malignancy was reported recently. 1 The present case is reported to provide evidence of a similar experience with a patient who also had this rare disease complex. Report of a Case The patient was a 9-year-old white boy who was followed at Wilford Hall USAF Hospital from Oct 5, 1964, until his death on May 1, 1967. He was the product of a full-term gestation and appeared normal during the first year of life. When he started to walk at 15 months of age, ataxia was noted. The child had frequent episodes of bronchitis and ear infections during the first four years of life, and when he was 6 years old, he began having repeated bouts of pneumonia. At age 7, telangiectasia of the auricles and of the palpebral and bulbar conjunctiva became evident, and the diagnosis of

181 citations


Journal ArticleDOI
TL;DR: Since 1956, a new syndrome which they called "a syndrome of obesity, short growth, cryptorchidism, and mental retardation, with an amyotonia-like condition in the neonatal period" has been described.
Abstract: IN 1956, Prader et al 1 described a new syndrome which they called "Ein Syndrom von Adipositas, Kleinwuchs, Kryptorchismus und Oligophrenie nach myatonieartigem Zustand im Neugeborenenalter" (a syndrome of obesity, short growth, cryptorchidism, and mental retardation, with an amyotonia-like condition in the neonatal period). Since then, other cases have been reported 2-24 (Table 1) under various titles. (Synonyms for this condition are: Syndrome of Willi-Prader, 8 of Willi, Prader, and Labhart, 18 of Prader-Willi, 13-15,23,24 of Prader, Labhart, and Willi, 7 and of Prader, Labhart, Willi, and Fanconi. 21 [Fanconi, with Prader, Labhart, and Willi, was coauthor of an exhibit of this syndrome at the Eighth International Congress of Paediatrics, July 22-27, 1956, in Copenhagen.] Some of the more descriptive titles are: hypotonia, mental retardation, obesity, and cryptorchidism associated with dwarfism and diabetes in children 5 ; hypogenital dystrophy with diabetic tendency 12 ; congenital muscular weakness and hypotonia with mental retardation,

161 citations


Journal ArticleDOI
TL;DR: Otitis media is a common problem in infants and children and although antibiotics seem to be effective in controlling suppurative complications, otitis is still a significant cause of morbidity and hearing loss and treatment is largely empirical.
Abstract: ACUTE otitis media is a common problem in infants and children. Although antibiotics seem to be effective in controlling suppurative complications, otitis is still a significant cause of morbidity and hearing loss. The etiology remains unsettled and treatment is largely empirical. Bacteriologic studies have repeatedly demonstrated that in only 60% to 70% of cases is it possible to isolate a pathogen from middle ear fluid.1-5Unproved explanations for the sterility of the remaining 30% to 40% are diverse and include a possible viral etiology.5There have been only sporadic accounts of virus isolation from middle ear fluid; few systematic attempts to isolate virus from middle ear fluid have been reported.5-8The association ofMycoplasmawith bullous myringitis has been observed in adult volunteers infected with artificially propagatedM pneumoniae.9In naturally acquiredMpneumoniaeinfection the organism has been recovered on several occasions from the middle

154 citations


Journal ArticleDOI
TL;DR: In the hope that a relatively long follow-up of a group of patients with precocious puberty would shed some light on the natural history of the process, the present study undertook.
Abstract: Sexual precocity is an uncommon condition which is characterized by the appearance of the changes of puberty before the anticipated age of puberty. It affects girls more often than boys, although a familial occurrence of the disease among boys has been reported. 1 "Constitutional," "cryptogenic," and "idiopathic" are qualifying terms to designate precocious puberty of unknown cause. "Neurogenic" and "cerebral" are terms applied to precocious puberty associated with organic lesions of the central nervous system (CNS). In the hope that a relatively long follow-up of a group of patients with precocious puberty would shed some light on the natural history of the process, we undertook the present study. Moreover, the frequent association of sexual precocity with organic lesions of the CNS prompted a desire to gain information about the possible development of lesions of the CNS among patients who, at the time of their original examination, had uncomplicated precocious puberty.

151 citations


Journal ArticleDOI
TL;DR: The role of the spleen in bodily defenses and the hazard of infection associated with the asplenic state has been discussed in the literature as mentioned in this paper, with a gross rate of infection of 5% to 8% following splenectomy, though how many infections have been attributed to splenectectomy has depended largely on the judgment of the author.
Abstract: IN 1952 King and Shumacker observed that of five infants who had splenectomy in the first six months of life all five later developed serious infections, three of them fatal. 1 Subsequently, many other clinical series as well as many experimental observations appeared in the literature, all designed to demonstrate the role of the spleen in bodily defenses and the hazard of infection associated with the asplenic state. Reported morbidity rates in persons who have had splenectomy have varied from 5% in King and Shumacker's original series to zero in a few small series. Most authors have suggested a gross rate of infection of 5% to 8% following splenectomy, though how many infections have been attributed to splenectomy has depended largely on the judgment of the author. Most writers have recognized that at least some of the postsplenectomy infections were related to the primary disease or were operative complications. Still,

121 citations


Journal ArticleDOI
TL;DR: Interest in streptococcal impetigo or pyoderma as an important predisposing infection of acute glomerulonephritis has appeared to be greater among physicians and investigators in the southern and southeastern section of the US reflecting perhaps a higher incidence of impetiosa in warmer climates, and its relatively more important role in nephritis.
Abstract: OUR PRESENT knowledge of impetigo contagiosa extends from descriptions now over 100 years old.1,2The defining of "streptococcal" and "staphylococcal" forms of impetigo has been principally the work of dermatologists,3-5while others, including bacteriologists and epidemiologists, have described the characteristics of infecting organisms.6-10Fewer examples of studies of impetigo are found in the pediatric literature and the above mentioned studies may not be widely appreciated by physicians caring for children. Interest in streptococcal impetigo or pyoderma as an important predisposing infection of acute glomerulonephritis has appeared to be greater among physicians and investigators in the southern and southeastern section of the US reflecting perhaps a higher incidence of impetigo in warmer climates, and its relatively more important role in nephritis.11-16The reports of pyoderma and nephritis among Indians at Red Lake, Minn, indicate however that skin infection is by no means limited to the south.17,18

106 citations


Journal ArticleDOI
TL;DR: A comprehensive review of clinical and laboratory findings of the oculocerebro-renal syndrome is compiled and certain as yet undocumented characteristics of the syndrome are added.
Abstract: THE OCULO-CEREBRO-RENAL syndrome is a congenital hereditary affectation limited to males. It is characterized by growth retardation, mental deficiency, hypotonia, mild or severe metabolic acidosis, generalized hyperaminoaciduria, proteinuria, rickets, and characteristic eye changes, the most prominent of which are bilateral congenital cataracts and glaucoma. Since the original description of this syndrome by Lowe et al1in 1952 many other reports have appeared. Recently a disturbance in the metabolism of ornithin has been described in a patient affected with this syndrome.2The term oculocerebro-renal syndrome3and Lowe's syndrome4have both been used interchangeably, though the former is preferable. In some published reports a diversity of clinical and metabolic manifestation from the original description has been noted.5 The purpose of the present communication is to compile a comprehensive review of clinical and laboratory findings of the syndrome and to add certain as yet undocumented characteristics of the

97 citations


Journal ArticleDOI
TL;DR: By handsorting all death certificates for US children dying of cancer, 1960 to 1964, it is able recently to separate deaths attributed to neuroblastoma and, in conjunction with information from 504 hospital charts of children with this neoplasm, to examine its patterns of occurrence for etiologic implications.
Abstract: NEUROBLASTOMA is among the most frequent cancers of childhood; yet in contrast to leukemia, very little effort has been made to seek clues to its origins through epidemiologic research. The failure to assign neuroblastoma a single exclusive code number in the Manual of the International Statistical Classification of Diseases, Injuries and Causes of Death , 1 has been a great handicap in studying its distribution nationally. By handsorting all death certificates for US children dying of cancer, 1960 to 1964, we have been able recently to separate deaths attributed to neuroblastoma and, in conjunction with information from 504 hospital charts of children with this neoplasm, to examine its patterns of occurrence for etiologic implications. Methods The mortality study was based on about 22,000 copies of childhood cancer death certificates provided by the National Vital Statistics Division, US Public Health Service. The certificates concerned all deaths from malignant neoplasms in children under 15

93 citations


Journal ArticleDOI
TL;DR: The American Board of Pediatrics is being exhorted to raise its training requirements and standards of examination to assure that its diplomates are competent consultants who are able to use the latest technologic developments and to lower its training required so as to encourage an immediately larger flow of pediatricians into the void left by the vanishing general practitioner.
Abstract: PERHAPS more than any of the other specialty boards, the American Board of Pediatrics is being buffetted by the winds of change blowing with increasing force from various quarters of the American medical scene. Simultaneously, the Board is being exhorted (1) to raise its training requirements and standards of examination to assure that its diplomates are competent consultants who are able to use the latest technologic developments, (2) to lower its training requirements and standards of examination so as to encourage an immediately larger flow of pediatricians into the void left by the vanishing general practitioner, and (3) to modify its standards in order to accommodate candidates who wish to be trained both in pediatrics and in some other field such as cardiology, allergy, neurology, psychiatry, mental retardation, public health, family medicine, community medicine, and others. While trying to keep a roof over its head during this tumult, the Board

89 citations


Journal ArticleDOI
TL;DR: The nephrotic syndrome has been selected for initial presentation in some depth because it is of inherent clinical and epidemiological interest and because it illustrates so graphically the type of information found about some of the other conditions studied.
Abstract: THE incidence of the nephrotic syndrome and its impact on the patient, his family, and the community were topics investigated as part of a survey of selected long-term diseases and conditions of children The survey encompassed the total child population under 16 years of age in Erie County, a large metropolitan area in western New York state The nephrotic syndrome has been selected for initial presentation in some depth because it is of inherent clinical and epidemiological interest and because it illustrates so graphically the type of information found about some of the other conditions studied The Erie County Survey of Long-Term Childhood Illness was undertaken in 1962 by the Erie County Department of Health in cooperation with the New York State Department of Health The major objective of the three-year study was the determination of the incidence and prevalence of about 70 long-term conditions of childhood for which services

Journal ArticleDOI
TL;DR: The experience illustrates the spectrum of clinical and radiological manifestations, emphasizes associated renal disease, includes pulmonary function data, and documents the survival of one patient to an advanced age in patients with TAD.
Abstract: IN 1964 Maroteaux and Savart 1 reported three instances of children surviving with a generalized, previously lethal, developmental abnormality of bone called the thoracic asphyxiant dystrophy (TAD). This condition was first described and named by Jeune et al in 1954. 2 Their patients died at early ages due to respiratory insufficiency. The hallmarks of the dystrophy are extreme constriction of the thorax and short-limbed dwarfism with abnormalities of the bone in the pelvis and extremities. Associated abnormalities reported are polydactyly and dental anomalies (Table). We have collected data on three patients with this dystrophy. Our experience illustrates the spectrum of clinical and radiological manifestations, emphasizes associated renal disease, includes pulmonary function data, and documents the survival of one patient to an advanced age. From our own experience and from information provided to us, it is clear that renal failure is a significant hazard in patients with TAD. Report of Cases

Journal ArticleDOI
TL;DR: The epidemiology of fatal disseminated herpes simplex virus infection occuring in children after the neonatal period has been reviewed and the association with malnutrition and measles stressed.
Abstract: THE NATURAL history of herpes simplex virus infection, which has been well established since the virus was first incriminated as a cause of stomatitis in children, 1 was reviewed recently. 2,3 Primary infection occurs most often in a subclinical or clinically unrecognized form in early childhood. If the primary infection is overt it may present in a variety of ways and recovery usually follows, but occasionally permanent damage or death results. The epidemiology of fatal disseminated herpes simplex virus infection occuring in children after the neonatal period has been reviewed and the association with malnutrition and measles stressed. 4 Little is known about the pathogenesis of this fatal form of infection which was initially recognized in Cape Town, South Africa in 1957. 5 Detailed virological and pathological studies have been carried out in 33 cases, including one neonate, seen in the subsequent nine years, and results of these studies are

Journal ArticleDOI
TL;DR: The literature has revealed that several cases of this syndrome have been published as other disorders, chiefly Albers-Schonberg disease and cleidocranial dysostosis and still others will be considered under differential diagnosis.
Abstract: MAROTEAUX and Lamy, 1 in 1962, defined pycnodysostosis as a syndrome consisting of the following characteristics: (1) dwarfism; (2) osteopetrosis; (3) partial agenesis of the terminal digits of the hands and feet; (4) cranial anomalies, such as persistence of fontanelles and failure of closure of cranial sutures; (5) frontal and occipital bossing; and (6) hypoplasia of the angle of the mandible. The literature has revealed that several cases of this syndrome have been published as other disorders, chiefly Albers-Schonberg disease and cleidocranial dysostosis. Still others will be considered under differential diagnosis. The first reported case appears to be that of Montanari 2 in 1923. Collado-Otero, 3 in 1956, accurately described a case as "another form of osseous dystrophy," and credit should be given to this author as well as to Maroteaux and Lamy for their recognition of this disorder as a distinct entity. Our literature survey yielded a total of

Journal ArticleDOI
TL;DR: The patient presented was delivered after an attempted abortion with aminopterin and showed such close resemblance to a case report by Warkany et al as persuasively to exclude other causes for fetal damage.
Abstract: IT HAS long been known that administration of aminopterin in early pregnancy usually causes fetal anomalies, fetal death, and miscarriage1,2A number of years ago this drug was occasionally employed for therapeutic abortion until it was discovered that a fetus which survived to term showed serious abnormalities of development3Probably this drug has not been employed as an abortifacient in recent years except by the very inept The patient we present was delivered after an attempted abortion with aminopterin and showed such close resemblance to a case report by Warkany et al4as persuasively to exclude other causes for fetal damage At the age of 4½ years she is perhaps the only living example of this syndrome; the patient of Warkany et al died at 29 hours and Meltzer's3patient survived the neonatal period but the final outcome has not been reported Report of a Case

Journal ArticleDOI
TL;DR: The prognosis and factors affecting the outlook of infants with neonatal hepatitis are investigated, with a focus on the long-term outlook.
Abstract: THE neonatal, or giant cell, form of hepatitis is characterized by elevations of conjugated serum bilirubin levels during the earliest months of life. Until the etiologies of this condition are discovered, it seems simplest to employ this single diagnostic label. While the clinical and histological features of neonatal hepatitis have been fairly clearly defined, 1-3 little is known about the long-term outlook. Significant numbers of infants with neonatal hepatitis develop chronic liver disease. 4 We therefore investigated the prognosis and factors affecting the outlook of infants with this condition. Materials and Methods Charts, autopsy protocols, and microscopic sections collected over a 15-year period (July 1950 to June 1965) at the Hospital for Sick Children, Toronto, were examined. Infants with idiopathic obstructive jaundice of at least two weeks' duration during the first 4 months of life were considered as having cases of neonatal hepatitis, whether diagnosed with the aid of surgical

Journal ArticleDOI
TL;DR: Seven patients with virilizing adrenal tumors are described, and in one case, virilization was present from birth, a unique instance of in utero virilized attributable to a tumor.
Abstract: FUNCTIONING neoplasms of the adrenal cortex are clinically manifested by virilization, Cushing's syndrome, feminization, or hyperaldosteronism. In the present report, seven patients with virilizing adrenal tumors are described. In one case, virilization was present from birth. This is a unique instance of in utero virilization attributable to a tumor. Other unusual clinical, pathological, and laboratory findings are being reported. Methods The method used for 17-ketosteroid (17-KS) measurements was a modification of that of Callow et al. 1 The procedure of Allen et al 2 was used for dehydroisoandrosterone (DHA). Pregnanetriol was measured at the BioScience Laboratories, Los Angeles, by Cox's method. 3 The 17-hydroxycorticosteroids (17-OHCS) were measured by a modification 4 of the method of Glenn and Nelson. 5 Aldosterone was estimated in patient 1 by a modification of the procedure of Neher and Wettstein. 6 Cathecholamines were measured by the method of Jacobs et al, 7 and vanillyl mandelic acid as described

Journal ArticleDOI
TL;DR: Factors contributing to pathogenesis and pathophysiology of hypertonic dehydration were studied in individual patients and controversial therapeutic recommendations, some of which are based on theoretical considerations, were considered.
Abstract: HYPERTONIC DEHYDRATION with infantile diarrhea has long attracted interest in Buffalo because here the incidence seems to be higher than that reported in the literature from other areas in the USA and it occurs predominantly in winter. The sodium concentration in the serum of infants hospitalized with severe diarrhea is above 150 mEq/liter in more than one third of the cases. The high incidence of hypertonic dehydration in Buffalo and controversial therapeutic recommendations, some of which are based on theoretical considerations, aroused interest in planning a controlled study which would help to evaluate causes and therapy of this disturbance. Factors contributing to pathogenesis and pathophysiology of hypertonic dehydration were studied in individual patients. Plan of Study Patients admitted to the Children's Hospital of Buffalo with dehydration and a concentration of sodium in the serum above 150 mEq/liter were admitted to the study. The patients were referred to the investigators by

Journal ArticleDOI
TL;DR: No treatment is required for cystic nodules in the median palatine raphe at the junction of hard and soft palates and histologic examination revealed cysts containing keratin.
Abstract: CLINICALLY evident cysts of the alveolar and palatal mucosa are apparently a very common finding in newborns and infants and are often referred to as Epstein's pearls or Bohn's nodules. The cystic lesions have been described as white, raised, multiple nodules of maxillary and mandibular alveolar ridges and midpalatal region varying in size from a pin head to 2 or 3 mm. The majority of these cysts are inconspicuous and usually only the larger lesions are noticed. No treatment of these lesions is required as they do not interfere with tooth eruption and usually disappear in a few months. Palatal mucosal cysts of fetuses and infants have been studied in detail by several investigators.1-3Monteleone and McLellan1found 79% to 85% of 393 1-day-old infants had cystic nodules in the median palatine raphe at the junction of hard and soft palates and histologic examination revealed cysts containing keratin. Burke

Journal ArticleDOI
TL;DR: It is hypothesized that these deaths occur in those infants who are unable to breathe through their mouths and who make violent spasmodic efforts to establish a nasal airway, and the resemblance to neonatal deaths of infants with choanal atresia strongly supports this hypothesis.
Abstract: Sudden unexpected death (SUD) occurs annually in the United States in more than 15,000 infants less than 6 months old—the greatest mortality during the first year after the neonatal period. These deaths are not only unexpected but mostly unexplained, and most of the hypotheses proposed for causation have been disproved. A previous communication reported 28 instances among 18,000 infants who were under regular supervision; additional cases may simply not have been retrieved. Almost all of these were diagnosed at autopsy as interstitial pneumonia with findings indicating asphyxia. It is hypothesized that these deaths occur in those infants (30%±) who are unable to breathe through their mouths and who make violent spasmodic efforts to establish a nasal airway. The resemblance to neonatal deaths of infants with choanal atresia strongly supports this hypothesis.

Journal ArticleDOI
TL;DR: This prospective study was designed in 1953 to determine the frequency of spontaneous remission of bronchial asthma in patients in the pediatric allergic clinic of the Strong Memorial Hospital.
Abstract: THE POSSIBILITY of "outgrowing" bronchial asthma at the time of adolescence has been the subject of much speculation and clinical study.1-5This concern takes on special importance if one recalls that asthma and related respiratory allergic syndromes make up more than a quarter of all chronic diseases among American children.6 This prospective study was designed in 1953 to determine the frequency of spontaneous remission of bronchial asthma in patients in the pediatric allergic clinic of the Strong Memorial Hospital. Methods As part of a controlled study of the effectiveness of hyposensitization therapy in perennial bronchial asthma in children, the half of the patients who received only placebo injections provided a group upon whom the natural history of the disease could be evaluated. There were 104 such children. Although not representing a random sample of all children in the community with asthma, this study does have the virtue of

Journal ArticleDOI
TL;DR: Children whose mothers had received PTU during pregnancy were studied and compared to siblings who were born at a time when the mothers were not receiving PTU and who were presumably euthyroid.
Abstract: LONG-TERM follow up of children born to thyrotoxic mothers treated with propylthiouracil (PTU) during pregnancy has not been reported previously. Since the goiters in the newborn associated with PTU therapy are usually small and, unlike iodide goiters, do not cause mechanical difficulties, 1 the major concern is whether the presence of goiter with accompanying hypothyroidism has an adverse effect on subsequent intellectual and physical development. This in turn is part of the larger question as to whether PTU therapy in the pregnant patient with thyrotoxicosis has any harmful effects on the subsequent development of the offspring. In an effort to answer this question, children whose mothers had received PTU during pregnancy were studied and compared to siblings who were born at a time when the mothers were not receiving PTU and who were presumably euthyroid. Materials and Methods From a previously reported group of 37 children whose mothers had received

Journal ArticleDOI
TL;DR: A particularly interesting patient with a complex dysmorphogenetic syndrome and an elevated level of serum alkaline phosphatase who developed a malignant schwannoma is concerned.
Abstract: THIS report concerns a particularly interesting patient with a complex dysmorphogenetic syndrome and an elevated level of serum alkaline phosphatase who developed a malignant schwannoma. Report of a Case The patient (No. 501867, born Dec 22, 1951) was first admitted to the University of Wisconsin Hospitals at the age of 5 years for dyspnea on exertion and cyanosis. The patient was delivered by caesarean section due to placenta previa after a full-term pregnancy. His birth weight was 3,345 gm (7 lb 6 ounces); his birth length is unknown. During the first year of life, he failed to gain weight satisfactorily; a cardiac murmur was first heard around the age of 12 months. He became cyanotic primarily during breath-holding spells. His penis had been noted to be large since birth. At the time of his first admission to the University Hospitals, he appeared to be a small, pale child, measuring 104

Journal ArticleDOI
TL;DR: There was a 4:1 preponderance of females over males who presented with these clinical features in patients with Turner's syndrome, and the sexual infantilism had not been apparent to Ullrich, whose patients were females.
Abstract: MOST of the phenotypic characteristics of what is now called Turner's syndrome were first described by Ullrich, 1 in 1930, in an 8-year-old girl. Webbing of the neck, cubitus valgus, congenital lymphangiectatic edema, prominent ears, small mandible, ptosis, dystrophy of the nails, and hypoplastic nipples were among the physical findings he reported. In 1949, Ullrich 2 summarized two decades of observations on such patients and noted that there was a 4:1 preponderance of females over males who presented with these clinical features. Other observers, such as Bizarro 3 had also recorded the presence of this syndrome in males. Several years after Ullrich's original report, Turner, 4 in 1938, specified certain features in older patients, all of whom were females, which are considered by some to be the cardinal signs of Turner's syndrome: webbed neck, cubitus valgus, and sexual infantilism. The sexual infantilism had not been apparent to Ullrich, whose patients

Journal ArticleDOI
TL;DR: The study was designed to determine whether GH responsiveness estimated by established stimuli becomes normal prior to, concurrent with, or after the clinical and growth improvements which accompany thyroid replacement.
Abstract: RETARDED linear growth in hypothyroidism has been attributed to three causes: (1) secondary hypopituitarism with decreased growth hormone (GH) secretion; (2) reduced sensitivity of peripheral tissues to GH; and (3) fall in the caloric intake.1Shiekholislam et al2have reported abnormally low levels of plasma GH in seven of ten hypothyroid children after stimulation with insulin hypoglycemia. The children with impaired GH responses were subsequently restudied when they were euthyroid and experiencing normal growth. Their GH responses to hypoglycemia were then found to be normal. This article is concerned with the results of serial evaluations of plasma GH responses in eight children with hypothyroidism. The study was designed to determine whether GH responsiveness estimated by established stimuli becomes normal prior to, concurrent with, or after the clinical and growth improvements which accompany thyroid replacement. Therefore, GH responses, growth rates, thyroid hormone levels, and bone maturation were assessed

Journal ArticleDOI
TL;DR: An instance of a response to steroid therapy which suggests a direct action of this hormone on the hemangioma is presented, which represents an unusual clinical variant of this syndrome.
Abstract: APPROXIMATELY 60 cases of hemangioma associated with thrombopenia have been reported in the literature since the original description of the syndrome in 1940. The intimate relationship between tumor and platelet count is well known; however, the precise mechanism for the inverse relationship between the size of the tumor and the number of circulating platelets has not been defined. Various forms of therapy have been attempted in this disorder. Contrary to several discouraging reports, the role of corticosteroid treatment is unclear. It is the purpose of this paper to present an instance of a response to steroid therapy which suggests a direct action of this hormone on the hemangioma. In addition, the case itself represents an unusual clinical variant of this syndrome. Report of a Case The patient was a 13-year-old white boy who presented with the syndrome of multiple hemangiomata and severe thrombopenia. He was the product of a full-term

Journal ArticleDOI
TL;DR: America, as a nation, is demanding that the medical profession provide high quality, yet compassionate medical care for all its citizens, and a double standard has developed for low-income families.
Abstract: THE POOR are demanding that America consider and deal with their problems, social, economic, and medical. America, as a nation, is demanding that the medical profession provide high quality, yet compassionate medical care for all its citizens. While the doctor-patient relationship always has been considered the sine qua non of medical practice, a double standard has developed for low-income families. The care of this group of patients is described as episodic, fragmented, crisis oriented, and anonymous. 1 The absence of a single physician to provide both preventive and curative services precludes the development of any relationship between doctor and patient. In the attempt to supply this need, large scale comprehensive medical care programs for low-income families have been established. These programs are based upon the assumption that such programs inevitably will improve the health of the patients served. The supporting evidence for this hypothesis is at best scanty; the need

Journal ArticleDOI
TL;DR: The phenotype of this patient is sufficiently similar to that of another patient with a G-chromosomal deletion 5 and different from "antimongolism" to suggest a new G-deletion syndrome, but four loci were excluded from such assignment.
Abstract: DELETION of part of a G-group chromosome causes a syndrome, 1,2 which has been termed "antimongolism," 3,4 because some of the phenotypic features appear antithetical to those observed in the G-trisomy syndrome of mongolism (Down's syndrome) This paper describes a patient with multiple anomalies and mental retardation associated with a ring and hence a deleted G chromosome The phenotype of this patient is sufficiently similar to that of another patient with a G-chromosomal deletion 5 and different from "antimongolism" to suggest a new G-deletion syndrome In our case no genetic locus could be assigned to the deleted segment, but four loci were excluded from such assignment Report of a Case Labor and delivery of the patient, a girl born on Nov 17, 1964, were normal Her birth weight was 298 kg (657 lb) Shortly after birth she was noted to have bilateral ptosis of the eyelids At 15 months of

Journal ArticleDOI
TL;DR: This manuscript, the second in this series of three articles, summarizes work completed by May 1967 by the groups and deals with various new data obtained in a series of trials being conducted by the laboratory.
Abstract: DURING the past year our group reported the development of an attenuated rubella virus and described the results of preliminary studies using this strain as an experimental live virus vaccine. 1,2 Since the initial clinical trials yielded promising results, a concerted effort was made to confirm and extend these observations. Accordingly, the attenuated strain high passage virus-77 (HPV-77) was made available immediately and has been widely distributed. To speed this work further, HPV-77 experimental vaccine produced in this laboratory was supplied to five groups of clinical investigators, three in this country and two abroad. Our manuscript, the second in this series of three articles, summarizes work completed by May 1967 by the groups and deals with various new data obtained in a series of trials being conducted by our laboratory. The accompanying papers 3,4 provide detailed information concerning the clinical studies performed by the three other groups in this country.

Journal ArticleDOI
TL;DR: In this paper, the authors acknowledge the assistance of William B. Ware, PhD, of the School of Education of Northwestern University, who performed the statistical evaluation of their data, and a question by a reviewer of the original manuscript led to an exchange of letters with the senior author.
Abstract: In a footnote, the authors of this paper acknowledge the assistance of William B. Ware, PhD, of the School of Education of Northwestern University, who performed the statistical evaluation of their data. A question by a reviewer of the original manuscript led to an exchange of letters with the senior author. Because a problem was involved which in the past has been and in the future will be encountered by many others in the statistical treatment of observational data and because Mr. Ware was helpfully lucid in clarifying the problem, a comment by the editor is justified. After perusing the manuscript and the query of the reviewer, your editor wrote to the senior author as follows: "You speak of using the analysis of variance as modified by Winer. Does this mean that unaccounted-for random variability (variance) was determined as the difference between total variance and the sum of the variances