American Journal of Diseases of Children (1960)

Published by American Medical Association
Online ISSN: 0002-922X
Publications
Article
Societal change swirls about us. We are reeling under current events, both those that affect society at large and medicine in particular. Just a few years ago, it would have been unthinkable to envision a Berlin Wall thatwas crumbling under the pressure ofhundreds of thousands of East Germans who now pass freely through its gates. In the same time frame, medicine has seen obstetricians leave their practices in the United States, because of the risk, cost, and torment of potential malpractice actions. How many of us would have predicted that Colombia would go to "war" with the drug barons and turn the country into a bloody area in the interest of stemming drug abuse? Which of us would have predicted that a major congressional thrust might result in a change in physician reimbursement that equates payment with effort, irrespective of a procedural component? These mixed examples cannot be equated in terms of the impact on the world, but they all illustrate that change is inevitable, and the only predictability in our lives is that change will occur. From the time the first bipeds walked the earth, humans have attempted to change the relationships of one individual to another, of one group of individuals to another group, to alter the environment, and to develop ways to fend off harmful elements in that environment. Our capacity to think, make judgments, solve problems, and simply answer questions posed by the world about us, irrespective of the usefulness of the answers, has always resulted in change. Why, then, are we continually surprised by change? Why do we seem unprepared to accept the inevitable? Why do we resist such change until forced to accept it, since it will happen, whether we accept it or not?
 
Article
Sir.—Brown's discussion1 on the P value continues his elegant series of lucid dissertations on medical statistics. A brief clarification is needed, however, concerning the issue of multiple statistical tests and the risk of type I error. Such corrections to compensate for multiple tests on the same data set are important during "data dredging" expeditions, explorations of a data set in search of statistical differences that were not anticipated by the original study design or hypothesis. As Brown points out, there is one chance in 20 that any such comparison will be significant at the P=.05 level. The corrected P values also protect against the type I error, which can occur when "in examining the treatment means we notice a combination that we did not intend to test but which seems unexpectedly large."2 In contrast to the data dredger is the investigator who designs a prospective study
 
Article
• Before undertaking a research comparison, investigators may wish to estimate the sample sizes needed to assure that the research is feasible and is worth the effort and expense. Such calculations require several decisions by the researchers: (1) the acceptable level of the type I error (P value), (2) the desired power of the test, (3) the difference between the samples that is considered to be important, and (4) the variability expected among the values to be studied. Some recipes for estimating approximate sample sizes are suggested. (AJDC 1988;142:1213-1215)
 
Article
Sir.—In his article on P values1 published in the April 1990 issue of AJDC, Brown has made some very helpful comments on the meaning of P values and has drawn attention to the difficulties associated with performing repeated statistical comparisons on the same set of subjects. However, twice in his article he does not pay sufficient heed to his own advice. First, he correctly points out that when a t test is used to compare the birth weights of two groups of infants and the significant P value is set at .05, the random variation in birth weights among the infants would suggest, wrongly, in about one comparison of 20, that an important difference between the two groups in average weight was present. He goes on to consider what happens when a second test, eg, on head circumference, is performed on the same group, and says "The chance of
 
Article
Several years ago, AJDC reversed a long-standing policy and discontinued publication of a large number of single patient reports (many call these "case" reports, but this term is too dehumanizing, and is not used by AJDC). We did so because most of these were of the "me too," or "nth instance of ..." type. We did not feel that such contributions were useful to our readers, nor were they important enough to occupy valuable editorial space. We still do publish patient reports, either as full original articles, when they are unique, or as a Pediatric Forum submission when they are valuable reminders of an important point in the care of children. Since this change in policy we have had multiple conversations and letters concerning the value of patient reports and we continue to receive some 100 such reports each year (we sincerely believe that authors do not read the
 
Article
The use of P values (eg, P≤.05 or similar notation) is commonplace in research reports in medical journals. There are two kinds of P values of interest to journal readers. One is the value selected by the researcher as the accepted "risk of the type I error," "α risk," or "rejection region." It is a widespread custom to use.05 as a convenient cut-point for "statistical significance." (This convention is a part of the statistical legacy of the British statistician, Sir Ronald A. Fisher.) The second kind of P value is the observed value from a statistical test, that is, the value resulting from application of a test (eg, t or Χ2 test) to observations. Since the "P" in P value implies a probability, most medical journals1 use an uppercase P, reserving a lowercase p to signify proportions. The aim here is to look at some slippery features
 
Article
Clinical, radiological, histochemical, ultrastructural, and biochemical studies were conducted on three cases of I-cell disease. I-cell disease can be readily distinguished from Hurler syndrome (mucopolysaccharidosis I) by the presence of hypertrophic gums, vacuolated lymphocytes in peripheral blood, and a normal level of urinary mucopolysaccharides. Accumulation of proteoglycans was more prominent in the inclusion bodies of I-cell chondrocytes in comparison to cultured fibroblasts, which contained a large amount of glycolipids and a small amount of proteoglycans. An autosomal recessive mode of inheritance was suggested in two of the cases.
 
Article
Case 1.—A 13-year-old boy developed a purulent otitis media and was given 29 tablets of 0.5 gm of Retasulfin over a period of ten days. On the fourth day thereafter he developed a morbilliform rash and was referred to the clinic. The next day numerous vesicles filled with serous fluid appeared on the skin. These became confluent over a large area and broke when rubbed, leaving a raw surface denuded of epidermis. Three days later the boy's entire body was nearly completely denuded of epidermis. At this time he developed purulent conjunctivitis, an ulcerated inflammation of the oral membranes, purulent exudate of the urethra, and pneumonia. The boy was given antibiotics, prednisone, methandrostenolone (Dianabol), and blood transfusions. The boy survived, and after 35 days of hospitalization he went home in a good state. Case 2.—A 2½-year-old boy developed acute bronchitis for which he was given an unknown dose of Retasulfin.
 
Article
A variety of substances claimed to possess growth-promoting activity have aroused much interest in recent years. The most interesting among these substances is the family of somatomedins (SM) (including SM-A, SM-B, and SM-C, IGF I and II, and MSA), which may actually be classified as hormones. Should purified SMs become available in larger quantities so that clinical trials become possible, these substances may eventually prove to be important therapeutic agents. Clinical observations strongly suggest the role of as-yet-undefined growth factors in a number of conditions. A field only recently subjected to investigation and that promises to yield findings of particular interest is the possible role played by factors governing the growth of elements of the epidermis and the nervous system.
 
Article
Sir.—The optimal dosage of growth hormone (GH) for the treatment of GH deficiency has not been determined. As little as 2.5 U given once weekly can result in catch-up growth.1 Raben2 established a dosage of 2 U three times per week regardless of body size as the standard that was used into the 1980s. In a series of reports the US Collaborative Study Group concluded that a dosage of 0.06 U/kg of body weight three times per week was appropriate. These and other studies were summarized by Frasier3 in a comprehensive review. He developed a log-dose response curve, based on the published data, that had a very shallow slope, indicating a decreasing cost benefit of increasing dosage. The purpose of this study was to compare what had been a relatively high dosage of pituitary extract GH (0.12 U or 0.06 mg/kg three times per week) recommended by one manufacturer
 
Article
Recently, Kessner et al1 reported an extensive epidemic due to enteropathogenic Escherichia coli (EPEC) which occurred during the winter of 1960-1961 in the metropolitan Chicago-northwestern Indiana region. This report prompted us to recount our experience with a nosocomial epidemic, sparked by the Chicago outbreak, which plagued our hospital for a period of three months. Our epidemic started late in July, 1961, and was attributed to 8-month-old twins admitted with diarrhea. Their 18-month-old sibling had been discharged from a Chicago hospital two weeks previously. The epidemic involved 27 infants (25 of them cross-infections in our hospital) and contributed to the death of six children. During the preceding six years, suppression of contagion of EPEC by a neomycin sulfate "umbrella" was successful in reducing contagion in diarrhea patients admitted to the Children's Hospital, Columbus, Ohio.2 All newly admitted diarrhea patients under 2 years of age were given oral neomycin
 
Article
• For a 24-month period (1977 through 1978), the determinants of neonatal intensive care unit (NICU) mortality were examined retrospectively in 133 consecutively admitted newborn infants who weighed less than 1,000 g at birth. Seventy-one (53.4%) died during the first five days of life, 27 (20.3%) died after the first five days of life, and 35 (26.3%) were eventually discharged from the unit. Neonatal infection was the key determinant of increased mortality after the first five days of life. In these patients, grampositive organisms were recovered from 54% (14/26) of all body fluid cultures from 21 infected infants. Infections included Staphylococcus epidermidis (27% [7/26]), Staphylococcus aureus (15% [4/26]), and Escherichia coli (11% [3/26]). We propose that reducing the incidence of neonatal infections is necessary to substantially improve late NICU mortality in these very-low-birth-weight neonates. (Am J Dis Child 1983;137:838-841)
 
Article
• Twenty-five of the 45 long-term survivors with birth weights of 1,000 g or less who were cared for in the University of Washington, Seattle, Neonatal Intensive Care Unit from 1960 to 1972 were examined at a mean age of 10.6 years. Seven of the 25 children (28%) had one or more major neurologic or sensory handicaps. Sixteen (64%) have been or presently are in a special education program. Only seven children (28%) are currently rated by their teachers to be achieving at or above grade level. Arithmetic reasoning, mathematics achievement, and reading comprehension were specific weaknesses. Fine and gross motor skills were impaired. Perceptual skills were impaired to a lesser degree. (Am J Dis Child 1982;136:105-110)
 
Article
• Reported survival rates of infants with birth weights under 1,001 g vary greatly; by implication, high survival rates may be used as a measure of the standard of perinatal care. To illustrate the importance of precisely defining the population sample, we determined the survival rate to 2 years of 238 infants with birth weights of 501 to 1,000 g, born between 1977 and 1980. The rate was 33.6%; however, by excluding certain categories of patients, the survival rate was augmented to 46.9%. There were 36 infants who died in the delivery room and an additional 34 who died before the age of 3 hours in the intensive care unit; together they contributed 29.4% to the total mortality of 66.4%. This group is especially liable to influence reported survival rates. Although exclusion of patients is legitimate, the criteria need to be precisely defined if data between centers are to be compared. (AJDC 1985;139:470-471)
 
Article
• Of 56 infants weighing less than 1,001 g who were born in 1980, 30 (54%) survived the neonatal period and 29 (52%) survived the first year. At 12 to 16 months of age, 44% of the measured survivors were below the fifth weight percentile and 32% were below the fifth head circumference percentile for their adjusted ages. Of the first-year survivors, 21 (72%) were developmentally normal, four (14%) were mildly handicapped, and four (14%) were moderately to severely handicapped at 12 to 34 months of age. Handicapped infants differed significantly from normal infants in their neonatal requirements for mechanical ventilation, but did not differ in birth weight, gestational age, route of delivery, Apgar scores, maternal age or marital status, maternal education or income, gender, race, place of birth, or proportions below the tenth percentile in weight or head circumference at birth. (AJDC 1984;138:837-842)
 
Article
• A controlled two-year study of the effectiveness of well-child care by pediatric nurse practitioners (PNPs) of 1,152 children, newborns through age 22 months, was made at the Kaiser-Permanente Medical Centers in San Francisco and Oakland, Calif. In this setting of a large, prepaid, group practice health care plan, the PNPs were found to be entirely competent in maintaining the health of their patients, and were generally accepted by the parents. Effects on utilization of medical care facilities were minimal. Costs of well-child care were reduced. (Am J Dis Child 130:51-55, 1976)
 
Article
This study by one individual of 1,200 patients with cat-scratch disease provides a heretofore unavailable realistic evaluation of a common infectious disease. All patients had lymphadenopathy, a prerequisite for diagnosis. Suppuration occurred in 11.8% of patients. Cat contact was established for 99.1%, and the cat was immature in the vast majority. An inoculation site, the most neglected feature in the study of the patients, was detected in 92.6%. The results of a skin test, considered as specific as the standard tuberculin test and to be safe but not standardized, was positive in 99%. The 12 patients with negative skin tests probably were tested too early in the course of the disease to have developed reactivity. Skin tests of 578 family members of patients, who served as controls, gave positive results in 18.5%. Of 60 patients with unusual manifestations, 48 had the oculoglandular syndrome of Parinaud. Other manifestations included erythema nodosum, encephalopathy, osteolytic lesions, thrombocytopenic purpura, and erythema marginatum. Most patients in this series had received antibiotics of many types during the course of the disease. None appeared beneficial. The disease is benign in character in a majority of patients. Surgical removal of involved lymph nodes or biopsy of lymph nodes or inoculation sites is not necessary for diagnosis or management. A survey of hospitals in the United States discharging more than 750 pediatric patients annually indicates that cat-scratch disease is a problem in all sections of the country.
 
Article
• To delineate the mineral metabolism of renal hypophosphatemic rickets and to update progress in linear growth after calcitriol (1,25-dihydroxyvitamin D3) therapy, the medical records of 19 patients were examined retrospectively from January 1978 through December 1985. With a mean (± SD) follow-up period of 42.0±5.4 months after calcitriol had been administered for at least 12 months, the growth measurements were as follows: the percentile weight (mean ± SD) remained unchanged, with the initial being 12.3%±17.3% and the final being 15.3%±18.6%, and the length/height percentiles were -2.7%±5.9% and -2.4%±4.4%. The growth velocity index showed a significant improvement from mean values of 61.7% (at age 2 years) to mean values of 101.2% (at age 11.2 years). Serum phosphate concentrations rose from the initial value of 2.9±0.6 to 3.5±0.8 mg/dL (0.9±0.2 to 1.1±0.3 mmol/L). The effects of calcitriol on renal function were tested by creatinine clearance values, which were 127±22 mL/min/1.73 m2 (2.12±0.37 mL/s/1.73m2) at the conclusion of the study, compared with 128±25 mL/min/1.73 m2 (2.13±0.41 mL/s/1.73m.2) obtained at the initiation of calcitriol therapy. We conclude that calcitriol treatment of renal hypophosphatemic rickets in children results in improvement of growth velocity and serum phosphate concentration without deterioration of renal function. (AJDC 1987;141:108-110)
 
Article
• Serum 25-hydroxyvitamin D (25-OH-D) and 1,25-dihydroxyvitamin D (1,25-(OH)2D) and bone mineral content by the photon-absorption technique were determined in eight patients with X-linked hypophosphatemic rickets treated for at least 24 months with oral sodium phosphate and high-dosage ergocalciferol (vitamin D2). Mean 25-OH-D2 level was 129.5 ± 67.5 ng/mL (mean ± SD); the level of 25-OH-D3 was 10.5 ± 5.8 ng/mL. These values were significantly higher than in normal subjects (total 25-OH-D mean of 27 ± 10 ng/mL). Serum 1,25-(OH)2D was 16.9 ± 8.5 pg/ml (mean ± SD) in the eight patients, significantly lower than 47 ± 16 pg/mL in 27 age-matched controls. Values indicative of significant demineralization were found in seven of the eight phosphate-treated patients, who had no radiologic evidence of rickets. These results suggest that any theory of the pathogenesis of this disorder must account for inappropriate renal vitamin D metabolism and for renal hyperphosphaturia. The failure of high-dosage oral phosphate and ergocalciferol to fully correct demineralization may suggest a role for calcitriol (1,25-(OH)2D3) as a therapeutic agent. (Am J Dis Child 134:140-143, 1980)
 
Article
Rickets, hypocalcemia, hypophosphatemia, and hyperparathyroidism were found in a low-birth-weight premature infant. The concentration of plasma calcitriol (1,25-dihydroxyvitamin D) was 145 pg/mL. With additional exogenous calcitriol (37.5 ng/kg/24 hr) given for eight weeks, the biochemical abnormalities were corrected and healing of rickets was evident. Three months later, while receiving only 400 IU of ergocalciferal daily, the patient had normal levels of serum calcium, phosphate, and alkaline phosphatase with a serum calcitriol concentration of 36 pg/mL. These observations suggest that rickets of prematurity may involve a malabsorption of calcium and phosphorus with an elevated calcitriol level needed to overcome this inadequate absorption. Additional doses of calcitriol may be of benefit in these infants, although it must be given carefully. Furthermore, the role of phosphate supplementation in these infants requires consideration.
 
Article
Using a precise assay for 1,25-dihydroxyvitamin D in serum, the levels in 103 children, aged 13 months to 16 years, were found to be 43 +/- 2 pg/mL (mean +/- SE). This value is higher than reported values in adults and in neonates. Age-related changes in 1,25-dihydroxyvitamin D levels during childhood were also evident. Older children have significantly higher levels than children less than 11 years, possibly indicating changes with puberty and the adolescent growth spurt. The values were significantly reduced in childhood uremia (13 +/- 5 pg/mL), in hypoparathyroidism (16 +/- 1 pg/mL), and in children with Fanconi's syndrome. Treatment with oral calcitriol in these three groups of patients led to increased serum levels.
 
Article
• The hormonal changes in the development of pseudohypoparathyroidism (PSH) have not, to our knowledge, been previously reported. The male sibling of a child with PSH was studied for 2½ years. At 1 year of age he had generalized subcutaneous calcifications that subsequently migrated over his body. At 3 years of age and over a six-month period, serum calcium levels fell; serum phosphorus, parathyroid hormone (PTH), and 1,25-dihydroxyvitamin D (1,25-[OH]2D) concentrations increased. There was no calcemic, phosphaturic, or urinary cyclic adenosine monophosphate response to PTH. The concentration of serum PTH was suppressed by infusion of calcium and doubled with edetic acid infusion, indicating that the parathyroids were sensitive to changes in calcium levels. Thus, increasing PTH and increased 1,25-(OH)2D concentrations occur in the development of PSH. Migratory skin calcifications may occur. We speculate that increasing the serum PTH level reflects increasing compensatory parathyroid production to overcome a progressive PTH receptor defect and serves, with increased 1,25-(OH)2D concentrations, to prevent severe falls in serum calcium concentrations in the early stage of the disease. (AJDC 1984;138:654-658)
 
Article
To evaluate the effects of calcitriol (1,25-dihydroxyvitamin D3) therapy for the bone disease induced by long-term treatment with anticonvulsants, we reviewed the medical records of 330 institutionalized oligophrenic children and young adults under 26 years of age to identify the 144 children who required anticonvulsant therapy. Of this latter group, 52 children were found to have serum alkaline phosphatase levels elevated more than 2 SDs above normal and were enrolled into this prospective three-year study. To achieve rapid resolution of the bone disease, we elected to use calcitriol at 0.25 to 0.75 micrograms/d. After 1195 patient-months of treatment, our data suggest that the dystrophic process was reversed in 42.3% of the cases, as judged by decreases in serum alkaline phosphatase levels at six months, 65.4% of cases at 12 months, and 83.3% of cases at 13 to 18 months. By 30 months of follow-up, all patients showed significant lowering of serum alkaline phosphatase levels. The improvements were slow and gradual. Twenty-six patients in the treatment series of 52 patients initially showed signs of rickets or osteomalacia on roentgenograms of the wrists. Of these 26 patients, 12 (46%) showed improvement on roentgenograms within 24 months of the beginning of treatment. With reference to complications, hypercalcemia (calcium level, greater than 11 mg/dL [2.74 mmol/L]) was encountered at the rate of one episode per 44 patient-months of treatment. Our results strongly suggest that calcitriol is effective in healing anticonvulsant-related osteomalacia among children and youths, with a low incidence of complications.
 
Article
It has been shown that long-term administration of anticonvulsant drugs may result in bone demineralization,1,2 and abnormal vitamin D metabolism has been implicated as the main cause.3 Recently, however, serum 1,25-dihydroxyvitamin D (1,25-[OH]2D) levels were found to be within normal range or slightly elevated in patients who were receiving anticonvulsant drugs.4 Other studies have suggested that metabolic acidosis produced by anticonvulsant drugs is a causative factor of bone demineralization.5-7 However, in these reports, the serum levels of vitamin D metabolites were not measured. We observed a case of anticonvulsant-induced rickets associated with renal tubular acidosis with a normal level of 1,25-(OH)2D and a reduced level of 25-hydroxyvitamin D (25-OH-D) in the serum. Report of a Case.—A severely handicapped institutionalized 12-year-old girl
 
Article
To evaluate whether tuberculosis-associated hypercalcemia is related to abnormal synthesis of 1,25-dihydroxyvitamin D (1,25[OH]2D) and whether ketoconazole administration may be useful in treating tuberculosis-associated hypercalcemia. Case study. Endocrine Unit, Pediatric Clinic, University of Pisa (Italy). Two boys (aged 10.5 years and 14.7 years) with active tuberculosis and hypercalcemia. At admission, serum 1,25-dihydroxyvitamin D levels were elevated. Oral ketoconazole administration (3.0 mg/kg every 8 hours) decreased 1,25-dihydroxyvitamin D levels within the first week of therapy (from 208.8 to 57.6 pmol/L [-72.4%] in one boy and from 321.6 to 115.2 pmol/L [-64.2%] in the other). We also found a coincident normalization of serum ionized calcium concentration (from 1.45 to 1.24 mmol/L [-13.0%] in one boy and from 1.55 to 1.26 mmol/L [-17.0%] in the other). Abnormal elevated levels of 1,25-dihydroxyvitamin D caused hypercalcemia in our patients; ketoconazole administration may be effective in the treatment of hypercalcemia in patients with tuberculosis, which decreases 1,25-dihydroxyvitamin D synthesis.
 
Article
Sir.—Pseudohypoparathyroidism (PHP) is a heterogeneous syndrome characterized by the resistance of the target tissues to the action of parathyroid hormone (PTH). Although PHP is usually considered to be a disorder of hypocalcemic patients, normocalcemic patients with PHP have been described recently.1 This report describes an 11-year-old normocalcemic girl with PHP. In this patient, the serum concentration of 1,25-dihydroxycholecalciferol was within the normal range, in contrast to the abnormally low values of this metabolite previously reported in hypocalcemic patients with PHP.2 The purpose of this article is to report the acute response of calciferol metabolite concentrations to PTH and dibutyryl cyclic adenosine 3′,5′-monophosphate (cAMP) infusion in a normocalcemic patient with PHP. Patient Report.—An 11-year-old girl presented for evaluation of short stature. She was a short, stocky girl with rounded facies; she was 126.0 cm tall (2.9 SDs below normal mean for age), weighed 28.9 kg (14% above average weight
 
Article
Urine calcium excretion was evaluated in 19 patients before and after calcitriol (1,25-dihydroxyvitamin D3) treatment that was followed up for a five- to seven-year period. The effects of increases of calcitriol dosage and modifications of calciuria with hydrochlorothiazide were systematically examined. The urine calcium excretion before calcitriol therapy was 2.3 +/- 0.8 mg/kg/day (mean- +/- SEM) and the urine calcium-creatinine concentration ratio was 0.12 +/- 0.04. With the dose of calcitriol at 23 ng/kg/day, these values increased to 3.2 +/- 0.8 mg/kg/day and 0.19 +/- 0.04, respectively. Following double dose of calcitriol (44 ng/kg/day), increments in calciuria and urinary calcium/creatinine concentration of 1.4 +/- 0.6 mg/kg/day and 0.10 +/- 0.03, respectively, were observed. With concomitant administration of hydrochlorothiazide (1 to 2 mg/kg/day) therapy at maintenance dose and calcitriol (31 ng/kg/day), the urine calcium excretion effectively decreased by 1.3 +/- 0.6 mg/kg/day and the urine calcium-creatinine concentration ratio by 0.05 +/- 0.02. The results suggest that children with calcium-phosphate disorders who require long-term treatment with calcitriol must be carefully monitored in terms of urine calcium excretion, especially when the dosages are increased to achieve maximal therapeutic efficacy. Calciuria induced by calcitriol administration is effectively reversed by addition of hydrochlorothiazide to the treatment regimen.
 
Article
In 1938 Gross performed the first successful ligation of a patent ductus arteriosus. Since that time techniques have varied1 and been perfected. Medical and surgical authors have discussed the radical and conservative treatment of this anomaly.2 Few corrections of this anomaly have been successfully carried out in the premature infant with secondary complications. Most authors prefer to operate on the child free of cardiac failure, infection, and the technical hazard of extremely small size. But, if these adverse conditions exist and are medically irreversible, then operative intervention should be attempted. The following case is an example of the successful repair of a patent ductus arteriosus in a premature infant with penumonitis and medically uncontrollable congestive heart failure. The patient weighed 3 lb 2 oz (1,417 gm) at the time of surgery. This case is believed to represent the smallest child ever to be successfully repaired.3 Report of
 
Article
THE PRESENT STUDY was concerned with the relationships of perinatal and environmental factors to subsequent mental development of infants with birth weights from 1,500 gm to 2,500 gm. A companion study in this clinic on infants with birth weights under 1,500 gm suggested that certain types of respiratory difficulty, toxemia of pregnancy in the mothers, and a limited formal education of the parents were possibly important factors in the failure of some infants to develop normal intellectual capacity.¹ Previous studies by others have indicated that the incidence of late unfavorable neurogenic sequelae among premature infants was inverse to birth weight, suggesting that different factors might be operating as birth weight increases or that the same factors were operating but with different intensity.²⁻¹¹ The present investigation, like the companion study previously reported, was a prospective one that placed special emphasis on respiratory difficulties in the neonatal period, but also
 
Article
A girl aged 3 years and 11 months, with recurrent episodes of unexplained metabolic acidosis, hepatomegaly, and fasting hypoglycemia unresponsive to glucagon, showed profound falls in blood glucose levels in response to oral fructose and glycerol challenge. In vitro analysis of her hepatic glycolytic and gluconeogenic enzymes demonstrated absent fructose-1,6-diphosphatase activity. A therapeutic trial of orally given folic acid, 30 mg daily, did not improve her tolerance for fructose and glycerol. Over the next two years she showed improvement in tolerance to fasting, and to fructose and glycerol loading on dietary management.
 
Article
We evaluated the use of Bayesian forecasting for gentamicin therapy in outborn infants weighing 1000 g or less irrespective of postnatal age. Dosages were individualized using a computer program, guided by early serum gentamicin assays after a loading dose and a database of population kinetics. Steady-state gentamicin levels achieved were compared with those from a regimen based on guidelines. A total of 26 gentamicin courses were individualized in 19 infants of 22 to 33 weeks' gestation, weighing 500 to 1000 g at 1 to 41 days of age. All steady-state trough levels were between 1 and 2.4 mg/L; peak levels were between 4.4 and 9.3 mg/L. The 95% confidence intervals were in almost identical ranges. The prevalence of toxic and suboptimal trough levels was less when compared with that of 23 gentamicin courses based on guidelines in 17 control infants. We conclude that early individualized gentamicin dosage over a range of postnatal age is a practical alternative and serum level distributions appear superior.
 
Article
The clinical records of all patients with blood cultures positive for a bacterial pathogen were retrospectively examined during an 11-year period to determine the rate of and clinical features associated with polymicrobial bacteremia. During this period, bacteria were isolated in 6302 blood cultures. Of these cultures, 38 instances (0.6%) of polymicrobial bacteremia occurred in 38 patients. In 37 patients (97%), an underlying condition was identified that was considered a predisposing factor for polymicrobial bacteremia--18 patients (42%) had lesions of the gastrointestinal tract, 13 patients (34%) had an indwelling central venous catheter, nine patients (24%) had a malignant neoplasm or were receiving chemotherapy, and nine patients (24%) had neutropenia. A total of 98 pathogenic organisms were isolated; 52 were gram-negative and 46 were gram-positive, and 18 patients (47%) had more than two organisms isolated. Polymicrobial bacteremia was usually clinically indistinguishable from monomicrobial septicemia. Overall mortality was 32%. Polymicrobial bacteremia continues to be a rare, but serious, infectious disease that usually affects children with underlying medical problems and is associated with a high rate of mortality.
 
Article
The number and causes of unintentional infant deaths were determined to identify common, preventable infant deaths. Retrospective autopsy review. Infants aged 1 day to 1 year undergoing complete autopsies. Autopsies performed by the Louisville Office of the Kentucky Medical Examiner's Program from 1979 through 1989. The manner of death was designated as an "accident" based on review of autopsy findings, scene investigation, and investigation by law enforcement officials. The cases were divided into groups based on the nature of the unintentional injury. Causes of death included asphyxia in mechanically unsafe sleeping environments, overlying, drowning, scald burns, plastic bag suffocation, house fires, motor vehicle collisions, aspiration of foreign bodies, hypothermia, blunt head trauma, and alcohol toxicity. The largest group of deaths in this series resulted from mechanically unsafe sleeping environments. The majority of deaths in this series could have been prevented by minor changes in the household environment. The causes of fatal unintentional injury to infants are different from those in older children. Pediatricians should be aware of hazards unique to this age group.
 
Article
Two infants with fatal echovirus type 11 infections are described. Disseminated intravascular coagulation developed in both patients, and at postmortem examination, diffuse hemorrhagic necrosis of multiple organ systems was evident, most strikingly in the liver. A 3-month-old child is described, in whom lethargy, vomiting, pitting edema of the occipital scalp and neck, and subsequent diffuse echovirus disease developed. The clinical manifestation in this infant of focal myositis with histologic documentation at postmortem examination is unique to echovirus 11 disease. To our knowledge, this child represents the first described patient with nonparalytic, fatal echovirus type 11 infection occurring beyond the immediate neonatal period.
 
Article
Two premature infants in a special care nursery acquired late-onset group B streptococcal (GBS) sepsis within a 24-hour period. The infecting strains were serotype III organisms with bacteriophage type 7/11/12. Cultures of the mothers of the two affected infants were negative for GBS, implying nosocomial acquisition of infection. Although 32% of nursery personnel had mucosal carriage of GBS, none of the seven isolates of GBS type III was the same bacteriophage type as the two infecting strains. Of the other infants hospitalized in the nursery, five were asymptomatically colonized with GBS. These infants were in bassinets adjacent to the affected infants; all five of their isolates were identical to the two infecting strains. We conclude that infant-to-infant transmission may result in nosocomial late-onset GBS septicemia.
 
Article
An 11-year-old girl with keratitis and plantar keratosis had tyrosinemia. The concentration of tyrosine in the plasma was 16.5 mg/dL. Dietary intake of phenylalanine and tyrosine was systematically varied, and the plasma concentrations of tyrosine and nitrogen balance were studied. It was necessary to achieve a total intake of phenylalanine and tyrosine less than 100 mg/kg/day to obtain plasma concentrations of tyrosine of less than 10 mg/dL. After dietary therapy was started, the keratitis resolved promptly, and the patient remained asymptomatic during a period of 16 months in which the mean plasma concentration of tyrosine was 11.1 mg/dL. The dietary management of a child at this age presents a different problem from that of a young infant. It can be successfully pursued at home, as well as in the carefully regulated environment of a clinical research center.
 
Top-cited authors
Reginald Tsang
  • Cincinnati Children's Hospital Medical Center
William Paul Glezen
  • Baylor College of Medicine
Lawrence D Hammer
  • Stanford University
Henry R Shinefield
  • Finlay Instituto - Center for Vaccine Research and Production
Michael Weitzman
  • NYU Langone Medical Center