Ernest M. Post

Psychosocial aspects of constitutional short stature: Social competence, behavior problems, self-esteem, and family functioning

To determine the psychosocial effects of short stature, we administered a battery of psychologic tests to 24 children (ages 6 to 12 years) with constitutional short stature. Their results were compared to those of a group of 23 healthy children with normal stature matched for age, IQ, sex, and socioeconomic status. The short children had significantly higher scores on parental ratings of behavioral difficulties, especially somatic complaints, social withdrawal, and schizoidal tendencies. There were also indications of impaired self-concept as expressed by feelings of unpopularity and dissatisfaction. Parental responses suggested a tendency to set less clear limits on behavior, but not necessarily toward overprotectiveness. Parents of short children also gave responses indicating poorer communication and cooperation among family members. In contrast to recent studies of growth hormone-deficient children, in which no maladjustment surfaced, these results indicate that children with constitutional delay have characteristic behavioral difficulties.

Activity of the alternative pathway of complementin the newborn infant

Levels of C3, properdin, factor B, and C3 to C9 activity were markedly reduced in cord sera taken from 94 normal newborn infants. Nevertheless, cord serum supported complete activation of its own alternative pathway by zymosan or CoF. Lysis of a target cell, however, was defective; nearly 75% of cord sera had reduced rabbit erythrocyte CH50 titers. These were partially increased by the addition of factor B and properdin, and totally restored by adding factor B, properdin, and C3 to C9. Therefore, although the alternative pathway of the neonate is intact, it appears to be limited in its ability to generate an adequate number of stable and active enzymatic sites on a target cell membrane.

Treatment of neonatal hypertension with Captopril

C A P T O P R I L (SQ 14,225) is an an t ihype r t ens ive agent which acts p r imar i ly by inh ib i t ing the enzyme that converts ang io tens in I to ang io tens in II. It has b e e n effective in the t r ea tmen t o f m a n y hype r t ens ive adul ts wi th high and n o r m a l ren in hyper t ens ion , re f rac tory to o the r fo rms o f therapy . 1 ~ Recent ly , Cap topr i l has b e e n used to t reat severe ly hyper t ens ive ch i ldren? . ~ We repor t he re three cases o f severe h igh r en in h y p e r t e n s i o n in n e w b o r n infants , all o f w h o m r e s p o n d e d to Cap topr i l t he r apy w h e n o the r modal i t i es o f t r e a t m e n t had failed.

Alteration of the complement system in children with Henoch Schönlein Purpura

Abstract The complement system was studied in detail in 24 patients with Henoch Schonlein Purpura. Serum levels of C3 and C3–C9 hemolytic titers were normal or elevated. Classical pathway activity was determined by quantitation of serum C1, C4, and C2 titers as well as by the ability of serum to support C3–C9 consumption with an immune precipitate and lysis of sensitized erythrocytes (CH 50 ). All of these studies were normal. Alternative pathway activity was determined by measurement of serum levels of properdin, properdin convertase, and factor B as well as the ability of serum to support C3–C9 activation with zymosan or cobra venom factor and the lysis of unsensitized rabbit erythrocytes. Only the serum levels of properdin and properdin convertase were abnormal. In 11 of 12 patients studied within the first week of the disease, levels of serum properdin (P) were reduced more than 3 SD from the normal mean; similarly, 5 of 12 patients had reduced serum levels of properdin convertase (PC). In addition, of five patients whose initial blood was obtained between the first and second week of the disease, four had low levels of either P or PC. Finally, seven samples were obtained 14 days or more after onset; four had low serum levels of P or PC and three of these were patients assayed from 1–5 years after onset. The length of time required for the P or PC to return to normal was extremely variable.

Association of Arterial Hypertension with Poor Outcome in Children with Acute Brain Injury

We studied intensive care unit (ICU) records of 42 comatose children with acute brain injuries to define the relationship between high arterial blood pressure and poor neurologic recovery. Diagnoses included head trauma, anoxia, Reyes syndrome, and central nervous system infection. The highest systolic blood pressure in all 42 patients exceeded the 95th percentile. In those whose highest systolic pressure exceeded 95th percentile by more than 20 torr, severe neurologic deficit or death occurred in 19 of 34 (56%), while in those with milder hypertension, poor outcome occurred in only one of eight (13%, p = 0.0316). Of those with high blood pressure persisting until ICU discharge, 14 of 19 (74%) had poor outcome, while those with blood pressure normalizing prior to ICU discharge had poor outcome in only six of 23 (26%, p = 0.0026). High blood pressure was not usually a reflex effect of elevated intracranial pressure. This finding suggests that measures to control high blood pressure are indicated in the management of the acutely brain-damaged child.

Thyroxine Screening Values in Premature Infants

OBJECTIVES Premature infants often have low thyroxine levels when compared to fullterm infants. We sought to determine gestational age specific normal ranges for thyroxine screening results for premature infants in neonatal intensive care units. METHODS Thyroid screening results for infants less than 38 weeks gestation admitted to two NICUs were examined. For each sample the thyroxine Z-score was computed using parameters from fullterm infants. The mean thyroxine Z-score was calculated for each gestational age for days of life 1, 2, 3-7, 8-14, 15-21, 22-28, and 29-60. RESULTS There were 1144 specimens obtained from 543 premature infants. The mean thyroxine Z-score was below 0 for almost every gestational age and days-of-life category. The mean thyroxine Z-score increased with gestational age, but did not rise with increasing postpartum age. CONCLUSION The data show that normal thyroxine Z-scores for premature infants are lower than for fullterm infants and remain low at least as long as the infants remain ill.

Acquired hypothyroidism due to iodine deficiency in an American child

Abstract Acquired hypothyroidism secondary to iodine deficiency is rarely reported in iodine-replete environments. The case of a 9-year-old patient with severe acquired hypothyroidism due to iodine deficiency is presented. His deficiency occurred because of a restrictive diet used to control eosinophilic esophagitis. Hypothyroidism and iodine deficiency were quickly corrected with a kelp supplement.

411 IDENTIFYING THE ACADEMIC AND EMOTIONAL DIFFICULTIES ASSOCIATED WITH SHORT STATURE

To determine if children with growth retardation are at greater risk for academic and emotional problems than those of normal stature, we studied twenty-five children of normal intelligence with constitutional short stature or growth hormone deficiency. On the Child Behavior Checklist, 44% of these children scored in the 90th percentile or higher on the overall index of behavioral difficulty, a level typical of children referred for mental health services. Our patients had specific elevations on indices of somatic complaints, schizoidal tendencies, obsessive-compulsive traits and depression. They also had a disproportionate incidence of excessive clowning (45%), being teased (65%), unhappiness (48%), and underachievement (41%). Another striking finding was that the children had a high prevalence of grade retention, 28% having repeated at least one grade, in spite of having normal intelligence (mean full scale IQ=105.3, S.D. 14.9). Furthermore, 30% of the subjects had a verbal IQ that was 20 or more points higher than the performance score, a discrepancy occurring in only 10% of the population. In conclusion, short children seem to be at increased risk for developing academic and emotional difficulties. To better define the development problems unique to these children, we are now administering an extensive battery of intelligence, achievement, and personality tests to our patients and a control group matched for age, sex and socioeconomic class.

1701 DEVELOPMENT OF AN OLFACTORY TEST FOR CHILDREN

To determine if olfaction could be reliably measured in young children, we tested 114 normal children, ages 4-10 years. We used 41 microfragrance (“scratch ‘n’ sniff”) cards, and photographs to depict the test substances. The cards were randomized into 41 blocks of five. Each subject was scored on 5 trials of one block of five odorants, and identified each odorant by selecting one of the photographs. Children ages 4-5 identified 10, children ages 5-6 identified 13, and children ages 8-10 identified 22 of the odorants correctly more than 70% of the time. We then selected 5 of the more frequently recognized odorants and tested 175 additional children. The odorants were candy cane, fish, baby powder, bubble gum, and orange. We also administered the Peabody Picture Vocabulatory Test-Revised to determine the relationship, if any, between olfactory scores and general intellectual ability. Children between the ages of 3 6/12 and 4 years had only 68% correct. By age 5, the percent correct had reached a plateau of 93%. An adult with Kallmann syndrome (hypogonadotropic hypogonadism and anosmia) had only 24% correct. There was no relationship between the Peabody Test scores and percent correct on the olfactory test. In conclusion, we have developed an olfactory test for evaluating young children. The improvement in olfactory scores with age may be due to maturation of the olfactory process rather than intellectual development.

682 COMPUTER-BASED MANAGEMENT OF A PEDIATRIC SUBSPECIALTY CLINIC

To improve patient care and reduce the time required for administrative functions, we developed a computerized system for our Pediatric Endocrinology Clinic. Using a Hewlett-Packard 9845A mini-computer, we store background data and add new information accrued at each patient contact. We maintain a tabular record of past, current, and pending data for each patient and enter test results as they are received. Prior to each weekly clinic, we enter the names or hospital numbers of the scheduled patients into the computer and generate a patient list. For each patient the computer prints a customized guide sheet by assembling selected skeleton sentences from a list stored in memory. The subset of sentences chosen for each patient is retained, but can be modified as desired. The guide sheet instructs residents and students by highlighting the pertinent aspects of the history and physical examination and also serves as the draft of the letter to the referring physician. After the patient visit, we enter new findings, diagnoses, tests and treatment plans into the computer. A preliminary letter is printed and reviewed before the computer types the final copy on letterhead stationery. Implementation of this system has enhanced our ability to evaluate our patients, provided a new teaching instrument and reduced the time consumed by administrative tasks. It is applicable to other clinical settings and the programs, written in BASIC, can be adapted to equipment costing less than