Showing papers in "JAMA Pediatrics in 1969"
TL;DR: It is likely, on the basis of the following literature review and additional case material, that multiple lentigenes commonly represent one facet of a more generalized hereditary syndrome.
Abstract: MULTIPLE lentigenes have been reported in association with a variety of abnormalities and a familial occurrence. It is likely, on the basis of the following literature review and additional case material, that multiple lentigenes commonly represent one facet of a more generalized hereditary syndrome. Zeisler and Becker 1 described a 24-year-old woman who experienced a marked increase in number of lentigenes from birth to puberty. There were none on the face, but otherwise they were generally distributed. The patient also had pectus carinatum, ocular hypertelorism, and mandibular prognathism. Rosen 2 reported three siblings (two male and one female) with large numbers of lentigenes, but no mention was made of other disorders. A large pedigree in which eight individuals in three generations had lentigenes was reported by Pipkin and Pipkin. 3 Seven affected members had nystagmus, and photographs of some members gave evidence of mandibular prognathism. The pedigrees suggested an autosomal
273 citations
TL;DR: Pedigree pattern and demonstration of biochemical abnormality in four presumed heterozygotes are evidence of the autosomal recessive nature of the syndrome.
Abstract: IN seven members of four related Amish sibships, each bearing the name Byler, a disorder characterized by intrahepatic cholestasis has been identified.1The purpose of the present report is to describe the clinical, biochemical, and histologic findings. Pedigree pattern and demonstration of biochemical abnormality in four presumed heterozygotes are evidence of the autosomal recessive nature of the syndrome. Report of Cases Case1.—A girl born in 1947 (VI,5 in Fig 1) was noted from early infancy to have loose, foul-smelling stools. At 9 months, jaundice was observed which subsequently cleared. For the next two years she had recurrent bouts of jaundice associated with light stools, dark urine, and pruritus. The "attacks" were often associated with infection and the jaundice persisted for periods varying from two weeks to two months. At 2 ⅔ years she was hospitalized for laparotomy. An enlarged lymph node thought to be compressing the common bile
273 citations
TL;DR: The findings of a positive relationship between the concentration of serum bilirubin and the caudad progression of dermal icterus suggests that simple inspection of the skin of the newborn infant provides useful information of the actual serum bilIRubin level.
Abstract: IN NEWBORN infants, progressive hyperbilirubinemia is accompanied by a caudad advancement of dermal icterus which begins at the face and proceeds to the trunk, the extremities, and finally to the palms and soles. This clinical observation was described by Rolleston and McNee 1 in 1929, but has not been previously correlated with actual levels of serum bilirubin. In this report, the results of such an investigation on a large series of infants is presented. Our findings of a positive relationship between the concentration of serum bilirubin and the caudad progression of dermal icterus suggests that simple inspection of the skin of the newborn infant provides useful information of the actual serum bilirubin level. Materials and Methods The study population comprised icteric full term and low birth weight newborn infants. Approximately 80% of the infants were white. The full term group of infants included (1) 85 infants with no hemolytic disease
186 citations
TL;DR: This communication reports on a longitudinal study of 20 infants with CID acquired during intrauterine life or in the immediate postnatal period, where isolation of cytomegalovirus from a urine specimen or from other sites was a prerequisite for inclusion in the study.
Abstract: REFINEMENTS of virological and serological diagnostic techniques have led to a more complete understanding of the congenital syndrome caused by the cytomegalovirus. The various clinical manifestations of congenital cytomegalic inclusion disease (CID) during the early weeks of life include hepatosplenomegaly, jaundice, hemolytic anemia, thrombocytopenia purpura, pneumonitis, chorioretinitis, and central nervous system (CNS) disease.1,2A number of studies have emphasized the mental and motor retardation resulting from chronic intrauterine encephalitis,1,2but knowledge of the natural history of congenital CID is incomplete. This communication reports on a longitudinal study of 20 infants with CID acquired during intrauterine life or in the immediate postnatal period. Materials and Methods Clinical Sample.—Twenty infants were selected for study during the first weeks of life by presenting signs and symptoms suggesting the diagnosis of congenital CID. Isolation of cytomegalovirus from a urine specimen or from other sites was a prerequisite for inclusion in the
167 citations
TL;DR: It is believed that the familial disorder described below is not only different from known disturbances of growth, including achondroplasia, but that it shows, in addition, several characteristic nonskeletal defects that justify its description as a new dwarfing syndrome.
Abstract: FOUR CASES of a previously unrecognized dwarfing syndrome have been observed in one family. Four additional cases are thought to have occurred in preceding generations. We believe that the familial disorder described below is not only different from known disturbances of growth, including achondroplasia, but that it shows, in addition, several characteristic nonskeletal defects that justify its description as a new dwarfing syndrome. Report of Cases Case1 (VI 3 , Fig 1).—The propositus is the son of nonconsanguineous parents. The mother was 20, the father 26 years old at the time of birth. The pregnancy, the mother's first, had been uneventful. Because of her short stature, 147.5 cm (4 ft 10 inches), a roentgenographic pelvimetry was performed. Interpretation was as follows: "transverse diameter, 13.5 cm; true conjugate, 11.9 cm; and bispinous diameter, 9.9 cm. Midpelvis and outlet appear adequate. No disproportion between fetal skull and pelvis." Labor was started at
157 citations
TL;DR: SIR Norman McAlister Gregg's report of "Congenital Cataract Following German Measles in the Mother"contained accurate and detailed descriptions of the clinical manifestations of congenital rubella which still provide a firm foundation for the intensive investigations completed during the subsequent 27 years.
Abstract: SIR Norman McAlister Gregg's report of "Congenital Cataract Following German Measles in the Mother"1contained accurate and detailed descriptions of the clinical manifestations of congenital rubella which still provide a firm foundation for the intensive investigations completed during the subsequent 27 years. Gregg detected cataract, microphthalmia, nystagmus, retinopathy, intolerance to atropine, transient corneal clouding, patent ductus arteriosus, congestive heart failure, low birth weight, feeding difficulty, dermatitis, and high death rate which are characteristic of congenital rubella in early infancy; correlated these findings with maternal rubella "most frequently in the first or second month"; and pointed out that "other defects... may show up as development proceeds." Within two years Gregg could report that deafness and retardation were also important components of the rubella syndrome.2 Retrospective studies by many investigators indicated that the risk of congenital defect following maternal rubella approached 100% for infection during the first eight gestational weeks,
144 citations
TL;DR: There are several instances in which a low protein diet simultaneously caused clinical improvement and reduction or elimination of hyperammonemia.
Abstract: THE Krebs-Henseleit urea cycle is the only biochemical pathway for the conversion of ammonia to urea in man (Fig 1). It is known to involve five enzymatic reactions and, so far, four genetically determined disease states have been described, each of which is characterized by a deficiency of one of the urea cycle enzymes. 1 All of these states are associated with varying degrees of hyperammonemia which, as would be expected, are most severe in the disorders of steps 1 and 2, and least severe in argininosuccinic aciduria where it usually occurs only after a protein load. The clinical picture of all these disorders includes a reluctance to eat high protein foods, intermittent ataxia, irritability, lethargy or even coma, and mental retardation. There are several instances in which a low protein diet simultaneously caused clinical improvement and reduction or elimination of hyperammonemia. For this and other reasons, it is likely
141 citations
TL;DR: The author has encountered 12 cases of a strange syndrome heretofore not described in available medical literature, which occurs in infants and consists of recurrent attacks, during which the baby keeps the head tilted to one side, either left or right, and often rotated slightly toward the opposite side.
Abstract: IN RECENT years, the author has encountered 12 cases of a strange syndrome heretofore not described in available medical literature. The condition occurs in infants and consists of recurrent attacks, during which the baby keeps the head tilted to one side, either left or right, and often rotated slightly toward the opposite side (Figure). In some cases there is no distress, the child appearing perfectly content unless the mother attempts to straighten the head, whereupon the baby cries and opposes the attempt. In other cases, the onset of head-tilting is accompanied by vomiting, pallor, and great agitation, after which the baby regains composure but keeps the head tilted for some time, often several days. Eventually the head spontaneously returns to its normal position, and the child appears none the worse for his experience, until again, without apparent reason, the whole process repeats itself. Eventually, after months or years, the attacks
103 citations
TL;DR: The third edition of Gleason, Gosselin, and Hodge remains an essential volume for any Pediatric Emergency Service or for any doctor dealing at all commonly with poisonings.
Abstract: The third edition of Gleason, Gosselin, and Hodge remains an essential volume for any Pediatric Emergency Service or for any doctor dealing at all commonly with poisonings. With the addition of one more author, Roger P. Smith, PhD, and a change in cover color to bright blue, this new volume should be placed alongside the two prior editions rather than replace them since some of the previously listed products have been dropped as obsolete and yet, still may exist to cause a rare poisoning. Basically, the third edition is unchanged as to format; the same seven sections: (1) first aid and general treatment, (2) ingredient index and toxicity ratings, (3) therapeutic discussions of specific poisons, (4) supportive treatment, (5) trade name product index and contents, (6) general formulations of common classes of commercial products such as drainpipe cleaners or hair sprays, and (7) index of manufacturers with addresses. Much of
102 citations
TL;DR: The RA 27/3 attenuated rubella strain was isolated directly from naturally infected material in WI-38 human diploid fibroblasts and its in vitro characteristics and its behavior when inoculated in man are examined.
Abstract: THE PROVENANCE of the RA 27/3 attenuated rubella strain has already been described in print. 1,2 Detailed information was given at the London Conference just three months ago. 3 Therefore we shall only outline the history of this strain, before proceeding to examine its in vitro characteristics and its behavior when inoculated in man. In order to avoid the problem of passenger viruses, the RA 27/3 strain was isolated directly from naturally infected material in WI-38 human diploid fibroblasts. 4 Explant cultures were made of the dissected organs of a particular fetus aborted because of rubella, the 27th in our series of fetuses aborted during the 1964 epidemic. The third explant, which happened to be from kidney, was selected arbitrarily for further study. Fibroblast cells that grew out from this explant could be subcultivated after several weeks. The presence of rubella virus in the supernatant fluids was confirmed. After four
100 citations
TL;DR: According to Crocker, the classical infantile form is the only one showing increased sphingomyelin in the brain, and the well documented adult Neimann-Pick disease can be added as the fifth form.
Abstract: NIEMANN-Pick disease is an inborn error of lipid metabolism characterized by abnormal accumulation of sphingomyelin in various organs. Crocker 1 divided the disease into the following four subgroups based on the combined clinical and chemical studies: classical infantile form (type A), visceral form (type B), subacute or juvenile form (type C), and Nova Scotian variant (type D). This classification seems to be generally accepted, but the well documented adult Neimann-Pick disease 2,3 can be added as the fifth form. A recent investigation 4 indicated that the deficiency of sphingomyelinase, an enzyme which hydrolyzes sphingomyelin, is the basic enzymatic defect of Niemann-Pick disease. A more comprehensive study 5 of this enzyme revealed its deficiency only in the infantile and visceral forms. According to Crocker, 1 the classical infantile form is the only one showing increased sphingomyelin in the brain. A characteristic pathological change of the central nervous system in infantile Niemann-Pick
TL;DR: The investigators designed a common protocol and standard case report forms for use at the five collaborating centers that permitted an evaluation of the effects of the corticosteroid in bronchiolitis and provided further insight into the natural history of the disease.
Abstract: A DOUBLE-BLIND study on the effect of betamethasone sodium phosphate in the treatment of 297 infants and children with acute bronchiolitis was conducted at five hospitals from December 1963 to June 1965. For this study, the investigators designed a common protocol and standard case report forms for use at the five collaborating centers. The information obtained permitted an evaluation of the effects of the corticosteroid in bronchiolitis and provided further insight into the natural history of the disease. The study was not designed to explore the causes of bronchiolitis or the effect of supportive treatment. In recent years, corticosteroids have been used in the treatment of bronchiolitis on the hypothesis that their anti-inflammatory action would reduce bronchiolar inflammation and swelling. These drugs have been regarded as ineffective by some investigators and lifesaving by others. In 1964, Sussman et al1reported no change in the clinical course of 49 bronchiolitic
TL;DR: A synthesis of this material may provide a predicted biologic behavior of neuroblastoma, so that the effectiveness of newer drugs to treat this tumor can be predicted.
Abstract: SECOND only to accidents, cancer is the most frequent cause of death in children older than 1 year. 1 In the California Tumor Registry neuroblastoma accounts for 5% of the childhood malignancies, and in children's centers 10% to 12% of the malignant neoplasms are neuroblastomas. 2,3 This tumor is preceded in frequency only by leukemia and brain tumor. However, there are only five series of more than 90 patients reported. Cases of neuroblastoma recorded in the California Tumor Registry are reviewed in this paper. This series is compared with the other large series reported from single medical centers: namely, the Hospital for Sick Children, London; the Children's Medical Center, Boston; the Memorial Center for Cancer and Allied Diseases, New York; the Children's Hospital, Philadelphia; and the Children's Hospital, Columbus, Ohio. 4-8 A synthesis of this material may provide a predicted biologic behavior of neuroblastoma, so that the effectiveness of newer
TL;DR: The recognition of the etiologic relationship between rubella and congenital defects by Gregg, 7 in 1941, established the clinical and public health importance of the disease, and documentation of the severe teratogenic impact of the Disease has stimulated efforts toward control of rubella.
Abstract: RUBELLA was first reported to be a distinct disease entity early in the 19th century in Germany. 1 Subsequently, it has become recognized as one of the common infectious diseases of childhood. Outbreaks of rubella have occurred in military recruits, 2,3 in boarding schools, 4 and other confined populations. 5,6 These have proved disruptive but hardly catastrophic. The recognition of the etiologic relationship between rubella and congenital defects by Gregg, 7 in 1941, established the clinical and public health importance of the disease. More recently, documentation of the severe teratogenic impact of the disease has stimulated efforts toward control of rubella. Initially, efforts were directed toward the isolation of rubella virus. This was accomplished in 1962 by Parkman et al 8 at the Walter Reed Hospital and by Weller and Neva 9 at the Harvard School of Public Health. Four years later Parkman, Meyer, and colleagues 10,11 at the National
TL;DR: The namefrontometaphyseal dysplasiato is chosen to designate this new syndrome of multiple bony defects which is apparently distinct from Pyle's disease, craniometaphYseal Dysplasia, craniodiaphySEal dys plasia, and osteopetrosis.
Abstract: THIS REPORT was prompted by the discovery of a patient with an unknown osseous dysplasia. We have chosen the namefrontometaphyseal dysplasiato designate this new syndrome of multiple bony defects which is apparently distinct from Pyle's disease, craniometaphyseal dysplasia, craniodiaphyseal dysplasia, and osteopetrosis. Report of a Case A 19-year-old white male patient was admitted for cardiac evaluation (Fig 1). On admission, he was noted to have multiple skeletal anomalies. The patient's father, mother, sister, and two brothers were normal. Consanguinity was denied. The patient was known to have a heart murmur since birth. Five years before admission, increased fatigability and exertional dyspnea were noted. Past history indicated that the patient had a 50% to 60% hearing loss bilaterally. Physical examination revealed a well-nourished boy with a marked supraorbital ridge, slight facial asymmetry, wide nasal bridge, mild antimongoloid obliquity, hirsutism above the eyebrows, and a small pointed chin (Fig 2).
TL;DR: Signs and symptoms of rubella arthritis range from subjective "morning stiffness" to full-blown arthritis characterized by swelling, redness, tenderness, and effusion, which is less common in men and is uncommon in children.
Abstract: TRANSIENT joint manifestations, ranging from mild arthralgia to severe extensive polyarthritis are a troublesome feature of rubella These complaints, described as early as 1906 in Osler's textbook,1have been a source of considerable concern in the past, since they may simulate the picture of acute rheumatoid polyarthritis Numerous reports have defined the epidemiologic and clinical characteristics of rubella arthritis, and a rather characteristic pattern now is well recognized2 Polyarthralgia and polyarthritis are so common among women with rubella as to be considered typical manifestations of the disease Joint involvement is less common in men and is uncommon in children Symptoms most typically appear with the rash or within several days after its onset, but rarely may precede the onset of rash by several days Involvement which is frequently symmetrical may range from subjective "morning stiffness" to full-blown arthritis characterized by swelling, redness, tenderness, and effusion Objective signs and
TL;DR: This study has studied growth following surgical correction of nine different congenital cardiac defects and provided information on postoperative growth acceleration and identified clinical situations in which accelerated postoperatively growth was less likely to occur.
Abstract: CHILDRENwith congenital heart disease may have significant growth disturbance.1-3Longitudinal studies have shown that the majority of children will have accelerated growth following correction of patent ductus arteriosus.4-6There are no reported longitudinal studies of the response of growth following the surgical correction of other cardiac defects. We have studied growth following surgical correction of nine different congenital cardiac defects. The results have provided information on postoperative growth acceleration and have identified clinical situations in which accelerated postoperative growth was less likely to occur. Materials and Methods The 463 patients in this study were selected from a group of children with congenital heart disease who were seen consecutively from April 1, 1963, through Sept 30, 1963, and followed to Jan 1, 1968. Their ages ranged from 1 week to 17 years. Children were admitted to the study if they had one of nine cardiac defects (Table 1) and
TL;DR: A 17-year-old married white mother was referred to the University of Alabama Medical Center for treatment of acute renal failure 24 hours after she ingested approximately 30 ml of arsenic trioxide with total elemental arsenic 1% (Cowley's Rat and Mouse Poison).
Abstract: INORGANIC arsenic has been a common poison since ancient times and even to this day remains a problem in clinical toxicology. Although considerable information is available regarding most aspects of acute arsenic intoxication, 1-3 little is known concerning placental transfer of this metaloid and its effects upon the human fetus. Although organic arsenic compounds enter the fetus in negligible amounts, 4,5 animal studies suggest that, in contrast, inorganic arsenic compounds readily transverse the placenta in quantities capable of producing fetal damage. 6 This report describes the case of a mother who ingested inorganic arsenic and the subsequent death of her newborn infant. Report of a Case A 17-year-old married white mother was referred to the University of Alabama Medical Center for treatment of acute renal failure 24 hours after she ingested approximately 30 ml of arsenic trioxide with total elemental arsenic 1.32% (Cowley's Rat and Mouse Poison). Immediately following
TL;DR: A study of infants with hemolytic disease of the newborn or neonatal hyperbilirubinemia was undertaken to describe all CNS related abnormalities which were detected during at least the four years thereafter, and those other neonatal factors which may have had an influence on the CNS were also documented.
Abstract: THE etiologic relationship of neonatal bilirubin exposure to classic kernicterus (athetosis, sensori-neural hearing loss, and defective ocular supraversion) is well known, but the relationship to other manifestations of central nervous system (CNS) dysfunction has not been well documented. 1-4 The possible interrelationship of neonatal bilirubin exposure and other neonatal factors, such as maturity, hypoxia, and acidosis on the later occurrence of CNS abnormalities, has not been clearly established. 5-10 At the Childrens Hospital of Los Angeles, a study of infants with hemolytic disease of the newborn or neonatal hyperbilirubinemia was undertaken to describe all CNS related abnormalities which were detected during at least the four years thereafter. In addition to bilirubin exposure, those other neonatal factors which may have had an influence on the CNS were also documented. The observations provided an opportunity for analyzing the above relationships and for providing the clinician with data which might be useful in
TL;DR: Comments on the author's statement that retinal changes have not been previously described in patients with GSD Type I, and multiple bilateral symmetrical, yellowish, nonelevated discrete paramacular lesions in the occular fundi of their cases.
Abstract: To the Editor .—I read with interest Dr. Richard Fine's article inThe Journal(Amer J Dis Child115:238-331 [March] 1968). I would like to make some comments with regard to the author's statement: "Retinal changes have not been previously described in patients with GSD Type I." Newer mentioned Von Gierke's disease among conditions in which lipemia retinalis occurs. To quote from his book 1 : "Lipemia retinalis occurs most commonly in diabetic acidosis, usually in growth onset diabetes. It may occur also in essential familial hyperlipidemia, secondary hyperlipidemias of the nephrotic syndrome, pancreatitis, Von Gierke's disease, or other conditions with increased triglyceride concentration of the blood." Dr. Fine and his associates described: "Multiple bilateral symmetrical, yellowish, nonelevated discrete paramacular lesions" in the occular fundi of their cases and found them most striking in patients with the highest serum triglyceride levels. These changes are not consistent with the classical description of
TL;DR: Five children challenged by intranasal administration of pharyngeal secretions containing 100 tissue culture interfering dose 50's of rubella virus 8 to 12 months after the five had received HPV-77 vaccine had significant increases in HI, neutralizing, and complement fixing antibodies.
Abstract: STUDIES by others have indicated that attenuated strain high passage virus-77 (HPV-77) or HPV-77 derived attenuated rubella vaccines are capable of inducing significant (fourfold or greater) increases in hemagglutination inhibiting (HI) and neutralizing antibody titers in at least 95% of susceptible children. 1-3 Although this acquisition of antibody is often accompanied by pharyngeal shedding of the vaccine viruses, spread of these viruses to susceptible contacts has not been reported. 1-3 To estimate the protective efficacy of HPV-77 induced antibodies, Meyer et al 1 challenged five children by intranasal administration of pharyngeal secretions containing 100 tissue culture interfering dose 50's (InD 50 ) of rubella virus 8 to 12 months after the five had received HPV-77 vaccine. Although none of these five children with HPV-77 induced antibodies developed clinical rubella or shed rubella virus after challenge with "wild" virus, two had significant increases in HI, neutralizing, and complement fixing (CF) antibodies. 1
TL;DR: The hypothesis that there is an association among small head circumference, mental subnormality, and growth failure was tested and the reliability of using motor-development milestones to predict ultimate mental development in children with congenital heart disease was evaluated.
Abstract: PREVIOUS reports in the literature have suggested that children with significant congenital heart defects, especially of the cyanotic types, may have delayed motor development and a higher than expected incidence of mental subnormality.1-3Two of the factors that have been considered to affect the mental development of these children have been the hemodynamic severity of the lesion and environmental deprivation due to physical incapacity.1-3 This study was designed not only to assess the roles of the hemodynamic abnormality of the defect and environment, but also to evaluate other factors that may make a crucial contribution to the mental status of these children. In this study of children with congenital heart disease, the hypothesis that there is an association among small head circumference, mental subnormality, and growth failure was tested.4The study also evaluated the reliability of using motor-development milestones to predict ultimate mental development in children with
TL;DR: These patients are the first recognized examples of dysgammaglobulinemia associated with a chromosomal deletion of the long arm of the same chromosome.
Abstract: IN CONTRAST to the X chromosome, little is known about the autosomal loci for various genetic traits. Recently Gerald et al1 and Bloom et al 2 have presented suggestive evidence that the controlling formation of locus the α-chain of haptoglobin is situated on one end of a No. 13 chromosome. We have studied two patients with chromosome defects and dysgammaglobulinemia. One had a No. 18 ring chromosome, which is formed by breakage in both arms with reunion of the broken ends and loss of a variable amount of material distal to each break. The other had a partial deletion of the long arm of the same chromosome. In both patients IgA was not detectable, and, in one, the level of IgG was very low. These patients are the first recognized examples of dysgammaglobulinemia associated with a chromosomal deletion. Report of Cases Case 1.—A 3-year-old white boy was first admitted to
TL;DR: The initial efforts of several groups to produce inactivated rubella virus vaccines were disappointing, and recipients contracted rubella with rash and, in many instances, transmitted their infection to susceptible contacts.
Abstract: AFTER THE isolation of rubella virus in 1962, 1,2 many investigators directed their attention toward studies that were to characterize the agent, define the nature of the disease, and eventually lead to vaccine development. The initial efforts of several groups to produce inactivated rubella virus vaccines were disappointing. Completely inactivated materials were usually not antigenic, and experimental preparations that did elicit an antibody response were thought either to contain residual live virus or to confer no protection to a rubella challenge. 3,4 Early attempts to develop a live rubella virus vaccine were also unrewarding. A number of separate research teams prepared experimental materials from virus strains arbitrarily passed varying numbers of times in cell cultures. 5-9 Unfortunately, these viruses were not attenuated, and recipients contracted rubella with rash and, in many instances, transmitted their infection to susceptible contacts. At this time, our laboratory was likewise engaged in passing strains of
TL;DR: No firm decision can be made on the proper age for immunization against rubella until more is known about the length of immunity from rubella vaccines, but most of the trials have been carried out in children in enclosed communities.
Abstract: THE EVENTS of the past six years have made clear the need for prophylactic measures against rubella. In 1966, Parkman and Meyer and their colleagues 1,2 reported the development of a live rubella vaccine which had been attenuated by passage in African green monkey kidney cultures. Clinical trials in susceptible children showed that the vaccine strain was immunogenic and produced very few reactions. Although virus excretion occurred in a proportion of the vaccinees, no transmission of infection was detected in susceptible contacts. Recently, further attenuated vaccines have been developed in different cell substrates, 3 and, on account of the potential risk of nasopharyngeal excretion to pregnant women, most of the trials have so far been carried out in children in enclosed communities. Until more is known about the length of immunity from rubella vaccines, no firm decision can be made on the proper age for immunization against rubella. We therefore
TL;DR: In 1963, Burian described what he called the "whistling face" or "cranio-facio-corporal" syndrome in four children, which would certainly suggest that this is the same condition described by the other authors.
Abstract: IN 1938, Freeman, an orthopedic surgeon, and Sheldon, a pediatrician, described two children with microsomia, increased philtrum length, small nose and nostrils, flattening of facial bones, deep set eyes, ocular hypertelorism, epicanthus, ulnar deviation of the hands without bony abnormality, finger contractures, and bilateral talipes equinovarus.1They employed the term "cranio-carpo-tarsal dystrophy" to describe the syndrome. Otto,2in 1953, and Kulz,3in 1961, each described a similar case and, cognizant of the earlier report by Freeman and Sheldon, continued to use the same term as well as the eponym, Freeman-Sheldon syndrome. In 1963, Burian,4a Czechoslovakian plastic surgeon, apparently unaware of the other reports, described what he called the "whistling face" or "cranio-facio-corporal" syndrome in four children. Unfortunately, the children were not described in detail. Examination of his published photographs, however, would certainly suggest that this is the same condition described by the other authors (Fig
TL;DR: The editors make no claim that the volume is a textbook of pediatric oncology, but indicate that it is written to describe the clinical and pathological manifestations of a variety of tumors in children and to discuss methods of treatment based on personal observations rather than "on an exhaustive review of the literature."
Abstract: The editors make no claim that the volume is a textbook of pediatric oncology, but indicate that it is written to describe the clinical and pathological manifestations of a variety of tumors in children and to discuss methods of treatment based on personal observations rather than "on an exhaustive review of the literature." The contributors' experience is derived mainly from the material and studies of the Manchester Children's Tumor Registry and includes a variety of rare as well as common tumors in childhood. Most of the chapters are stated to have resulted from collaboration between the Manchester contributors and after discussion with four distinguished physicians, three in the United Kingdom and one in the United States, concerning certain special facets of the subject matter. Written from the English viewpoint, the book includes chapters on: "Problems of Children's Tumors in Britain"; "Some Variations in Childhood Cancers Throughout the World"; "The Leukemias";
TL;DR: The authors want to advance observations on Shigella infections in neonates from a semiprimitive Mayan village of Guatemala, because the behavior of infections in these infants departs from that described by the American authors.
Abstract: SEVERAL cases of neonatal shigellosis have been reported recently in industrialized areas. 1-3 Practically all the Shigella infections reported were accompanied by serious clinical manifestations, mainly diarrhea, with several deaths. In the present paper, the authors want to advance observations on Shigella infections in neonates from a semiprimitive Mayan village of Guatemala, because the behavior of infections in these infants departs from that described by the American authors. The cases herein reported were observed during a longitudinal study in which a cohort of children is followed in their natural environment from the time of birth, in order to observe their general health condition, their growth, their diet, and the colonization of the intestine by parasites, yeasts, bacteria, and viruses. Only the infection by Shigella , observed in infants under 1 month of age, will be described and discussed in the present paper. Methods Study Area .—Since February 1964, children born in
TL;DR: This study studies the transplacental passage of attenuated rubella vaccines in humans using patients from the department of obstetrics and gynecology, University of Helsinki or The State Institute of Midwifery, Helsinki to study the potential communicability and danger of accidental vaccination during pregnancy.
Abstract: THE CURRENT candidate strains of attenuated rubella vaccines have proved capable of eliciting a demonstrable antibody response. At their present level of attenuation all the strains are known to cause viremia and virus shedding from the respiratory tract, although no transmission of the vaccine virus to susceptible contacts has been demonstrated with certainty. In addition to the potential communicability of attenuated rubella virus from a vaccinee to a pregnant mother, the danger exists of accidental vaccination during pregnancy. This has prompted our studies on the transplacental passage of attenuated rubella vaccines in humans. Materials and Methods The general scheme of the study is shown in Table 1 and the Figure. The vaccinees were patients from the department of obstetrics and gynecology, University of Helsinki or The State Institute of Midwifery, Helsinki. As of January 1969, 32 patients have received by subcutaneous inoculation (1 ml) either HPV-77-DK12 (dog kidney grown) rubella
TL;DR: A patient with a normal karyotype, who satisfied the usual diagnostic criteria for congenital leukemia, but who had a transient spontaneous reversal of her symptoms for a period of eight months is reported.
Abstract: A GREAT deal of interest in congenital leukemia exists, several features of which differ from leukemia in later life. Thus, a disproportionately large number of cases have myelogenous leukemia, and the disease has been observed in far greater number than expected by chance in children with Down's syndrome (mongolism) and other chromosomal anomalies. The general impression of the disease is that of a rapid downhill course, 1-6 but there have been a number of apparent spontaneous remissions or even spontaneous cures in infants with Down's syndrome. 7-14 We report a patient with a normal karyotype, who satisfied the usual diagnostic criteria for congenital leukemia, but who had a transient spontaneous reversal of her symptoms for a period of eight months. Report of a Case The child was born to a 19-year-old unwed mother, para 1, gravida 1, having ABO blood group A positive, after a full pregnancy. The pregnancy was