Billy F Andrews

Serum amino acid nitrogen in infancy and childhood.

Summary Determinations of serum amino acid nitrogenby a ninhydrin microtitration method for 109 infants and children were made in this study. Thirty-two control determinations were obtained from infants and children 6 weeks to 11 years of age. Values ranged from 3.0 to7.5 mg. per cent, with a mean of 5.01 mg. per cent. Twenty-five determinations were made oncord sera of normal term infants. Values ranged from 2.3 to 12.0 mg. per cent, with a mean of 7.68 mg. per cent. The values for cord sera differed significantly from those of the control group sera (t=6.07 p Sixty-six determinations were made on52 infants and children during and after illness. Values ranged from 2.5 to 40.0 mg. per cent, with a mean of 9.12 mg. per cent. Five of 7 patients with diseases involving the liver had elevated levels; 3 of these 5 died. Two patients with acute salicylate intoxication, who survived, demonstrated elevations which may also be attributed to hepatic injury. Four patients with renal disease presented normal values. Eight children with severe infectious diseases demonstrated increased levels. One of 2 patients with hyperthyroidism demonstrated an increased level. No patients were observed with alterationof intermediary metabolism of specific amino acids.

758 HOW TO ASSURE ENDING FOR SURVIVAL OF A PEDIATRIC DEPARTMENT

Funding for departments of pediatrics has been severly affected by recent economic changes. Institutional, State, National, and Foundation support has been reduced requiring non-traditional measures to assure teaching, research, and even service needs. Departments were forced to evaluate and prioritize all of their activities to assure maximal use of funds to assure goals. Two major approaches have emerged, Group Practices and Foundations. Our department of 165, with 40 faculty, 5 fellows, and 120 staff representing administration, clerical, research technicians, and paramedical personnel has remainded intact despite reductions in excess of 10% and forced salary increases. A foundation board of 14 members, chaired by the department chairman representing sections and single persons with four community members, was established with Internal Revenue Code 501(C)(3) approval. Daily operation is carried out by the chairman and executive board. The business arm of the foundation provides total care for poverty patients, tertiary, and consult care for all groups of children and is non-competitive with private practitioners. Extra compensation was provided for units of activity outside daily duties, irrespective of actual fees obtained for services. An unselfish, equitable, hardworking, and committed department of pediatrics has remained intact with academic performance, although stressed, not decreased and with an actual improvement on national tests by students and residents.

NONHYPEROXIC RETROLENTAL FIBROPLASIA

Retrolental fibroplasia has traditionally required the combination of prematurity and exposure to high ambient oxygen concentrations. However, cases of RLF in stillborn infants as well as an infant near term never given supplimental oxygen have been reported. Two recent papers have emphasized an increased incidence of RLF in populations of preterm infants with respiratory distress syndrome who received exchange transfusions.This report describes two infants near term who received exchange transfusions for hyperbilirubinemia and subsequently developed severe RLF in the absence of environmental hyperoxia and clinical respiratory distress. The case histories, clinical courses and retinal photographs will be presented. The possible etiologic role of the shift of oxygen-hemoglobin affinity caused by transfusions with adult erythrocytes with increased oxygen availability to the retinal vessels and tissues will be discussed.The medical and legal implications of our cases and the recent reports are many. For one, the levels of oxygen considered safe for neonates receiving exchange transfusion nay have to be re-evaluated. Secondly, serial thorough ophthalmologic evaluations in any neonate receiving adult erythrocytes by exchange transfusion or multiple transfusion replacement is crucial. Finally, the traditional relationship of hyperoxia and RLF must be expanded and re-evaluated to include other parameters like the hematologic status of the neonate and should include appreciation that this may be a phenomenon independent of environmental hyperoxia.

THE IMPACT OF NEONATAL INTENSIVE CARE

Application of new understanding, equipment and techniques to the care of high risk newborns has led to a generalized decrease in neonatal mortality. Studies related to morbidity and follow-up are under way in our own and in other centers. In Kentucky, a state which ranks very low economically, a reduction of approximately 30% in neonatal mortality has occurred in 10 years. In one of the first nurseries to develop neonatal intensive care a reduction of over 40% was recorded.Major contributing factors in this achievement have been the development of neonatal intensive care units in the states two medical schools, intra- and extra-mural programs for education of medical and nursing personnel, improvement in regional care with few infants born in hospitals with under 1000 deliveries, and family practitioner, obstetric and pediatric cooperation.Tables, graphs, pictures will be utilized to illustrate changes in patterns of mortality, equipment, education, care and types of problems encountered.

The Rights of Children 150

Thirty years ago “The Childrens Bill of Rights” was written to call attention to the lack of attention given to the rights of children and when most of our states had more rights against abuse for animals than they did for children. Accomplishments have been made for childrens rights. The United Nations Convention on the Rights of the Child, 1989, has been accepted, except for the United States and one other nation. Currently there are many groups in the United States who are seeking a Constitutional Amendment to secure rights for our children. “The Childrens Bill of Rights” is again being used to promote this venture. “The Childrens Bill of Rights” will be presented and discussed. Copies will be provided.

Neonatology--A Six Finger Exercise Revisited 149

In 1958 at Walter Reed General Hospital the cartoon, “Neonatology--A Six Finger Exercise” was first used with special emphasis on the sixth finger--pediatrica iatrogenica. This area had been a major factor in the development of this new field and must always remain a concern for those who care for the newborn. The cartoon below will be discussed in light of the time. Figure

The Story of the Concept of the Small-for-Date Infant. 802

“Why did you think of the concept for small-for-date infants (SGA and IUGR)? has been a frequently asked question of me for nearly 40 years. It happened in October 1958 at the Walter Reed Army Hospital. It had possibly been fermenting since my mentor in physiology, Otto H. Gauer, had forced me to read the works of William Harvey prior to initiating research while a medical student at Duke University School of Medicine and where the Dean Wilburt C. Davison constantly mentioned that he weighed in at less than a Kg. Harry G. Gordon on rounds routinely emphasized, “Many problems of the newborn are related to the degree of maturity which they possess at birth, irrespective of size.” When presented an infant who appeared emaciated, Ogden C. Bruton would question, “Why did this infant fail to thrive in the natural habitat of the uterus?” Stuart H. Clifford had just written about postmaturity. The widely used and reprinted cartoon, devised to present a concept of small-for-date infants for the medical students of Frederick Burke at Georgetown University and of Roland Scott at Howard University, Schools of Medicine and residents at Walter Reed, will be explained in light of the times when infants under 2.5 Kg. were considered premature.

A NEW APPROACH TO SOLVING ETHICAL PROBLEMS IN MEDICINE. 229

New medical, scientific and technological advances, ever present fiscal restrictions and changes in the practice such as managed care by insurance companies and increased restrictions of government force physicians to examine each change in light of their moral and ethical values. “Ethics is the highest possible level of moral thought and action” and every pediatrician who is a member of the American Academy of Pediatrics has accepted an advocacy role for children. Irrespective of our multiple races, religions and languages, all can find basic values and standards of accepted conduct which are held universally. Thought does not extend beyond language and this is also true for medical ethics. There is a need for a basic cluster of words or definitions which we can follow in making ethical decisions.

712 MIDTRIMESTER DIAGNOSIS OF OSTEOGENESIS IMPERFECTA, TYPE 2

We report two affected offspring with Osteogenesis Imperfecta, Type 2. The first child was born near term and expired shortly thereafter. Prenatal radiologic examination was consistent with O.I., Type 2. The second affected fetus was diagnosed at 19 weeks gestation by sonographic and radiographic imaging even though the amniotic fluid pyrophosphate was negative at 14½ weeks gestation. The skull was poorly mineralized and compressed. Long bones were shortened, angulated, and markedly irregular. There was deficient ossification of the spine, extremities, and minimal patchy ossification of the calvarium. Pregnancy was terminated at 21 weeks following the radiographic diagnosis. Radiologic and pathologic examination of the abortus was consistent with the prenatal diagnosis.Although the diagnosis of this, as well as other bone dysplasias has been made in utero during late pregnancy, we conclude that radiographic and sonographic imaging may be the only method of diagnosis of O.I., Type 2 during midtrimester gestation.

725 A NEW SYNDROME OF RETINITIS PIGMENTOSA, HEARING LOSS, MENTAL RETARDATION, AND DYSMORPHOLOGY

Two siblings born to nonconsanguinous parents were evaluated for mental retardation(MR). Findings included retinitis pigmentosa(RP), sensorineural hearing loss(HL) and similar dysmorphism.JA was 4½ and LA was 2. There was delayed closure of the anterior fontanelle. Length and weight were 5th% and OFC for JA was 50th% and LA was 20th%. They had frontal bossing, down-slanting palpebral fissures, epicanthal folds, flattened nasal bridge, lowset ears, and small feet. JA had strabismus and LA had proptosis. JA had small externalia genitalia. Both had hypotonia and hearing loss. Mental ages were 19 and 9 months. Both had extinguished ERGs, normal CT scans, amino acids, OGTTs and chromosomes. LA had normal skull films and thyroid studies.RP is the common demoninator in a number of genetic syndromes with overlapping clinical characteristics:Although our patients shared some of these features, the dysmorphology separates them and reflects a new recessive syndrome which should be added to this group.