It has long been assumed that children with short stature have more social, academic, and psychological problems than those with normal stature (Drash, 1969; Gold, 1978; Money & Pollitt, 1966; Pollitt & Money, 1964). They were believed to experience low self-esteem, a high degree of social isolation, withdrawal, immaturity, and disturbances of body image. However, recent studies have shown that growth hormone deficient children had no greater occurrence of abnormalities in general psychological adjustment, sex role development, body image (Drotar, Owens, & Gotthold, 1980), anxiety, or locus of control (Stabler & Underwood, 1977). Although such children seem to have normal psychological adjustment, they represent less than 1% of the children with growth disorders (McArthur & Fagan, 1971). Growth hormone deficient children probably experience short stature differently from other short children, because their condition is being ameliorated by medical therapy. Most short children have constitutional short stature (Horner, Thorsson, & Hintz, 1978), which is characterized by (a) normal birth weight; (b) growth failure between 6 months and 3 years; (c) subsequent normal growth velocity; but (d) height below the 5th percentile for age throughout childhood. This condition is also referred to as constitutional growth delay and constitutional delay of growth and adolescence. Because the period of growth failure is associated with a deceleration in skeletal maturation, the potential for attaining normal adult height remains excellent. The onset of pubertal changes, including the growth spurt, is usually delayed. Children with constitutional short stature usually become adults of normal stature, but may suffer lasting psychologic effects from their previous short stature.

The Gordon Diagnostic System (GDS) is a single-component microcomputer-based instrument that can be used to administer 11 psychological tests. The game-like tasks provide objective data for evaluating possible attention-deficit hyperactivity disorder or other conditions that affect a person’s ability to sustain attention and exert self-control. The core of the instrument is an integrated circuit with a microprocessor, a random access memory, and an erasable, programmable read-only memory (EPROM). It can be programmed with an IBM PC and an EPROM programmer. The assembly language source is compiled into machine language, which is used to simulate the GDS on the IBM PC or is “burned” into the ROM. The memory is erasable with ultraviolet light, so revisions can be made easily. The GDS illustrates that “custom” programming a ROM is not limited to large industrial concerns, and that it can be done within small research groups.

An odor identification task was used to determine whether individuals with cleft palate (with or without cleft lip) also have an increased prevalence of olfactory deficits. Olfactory responses of 35 affected subjects (7 to 22 years of age) were compared with those of 68 subjects of comparable age without cleft palates. Subjects were requested to identify the smell of ten common household odors. They selected their responses from an alphabetized list of the test odorants. After a practice trial, the set of odorants was presented five times in randomized sequences. The percentage of correct responses increased with age for prepubertal and pubertal subjects without cleft palates. Although the olfactory scores of girls without cleft palates continued to increase after puberty, this trend was absent in boys. On the average, the girls with cleft palates, compared with only three of 34 boys without cleft palates, had olfactory scores less than 60% correct. There was no evidence of heterogeneity in the magnitude or direction of the relationship between any of the subtypes of cleft palate and olfactory dysfunction. In this study, cleft palate is more strongly associated with olfactory deficits in boys than in girls, suggesting the possibility that the deficit may be a sex-influenced trait.

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, Reye's 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.

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.

To assess the effects of constitutional short stature on intellectual, academic and visual-motor functioning, we administered a battery of psychological tests to a group of 24 such children. Their responses were compared to those of 23 subjects with normal height matched for age, sex and socioeconomic status. Contrary to the conclusions of uncontrolled studies, our data showed short and control groups to be comparable on all variables. While children with constitutional delay have been shown to exhibit low self-esteem and high levels of internalizing behavior problems, these difficulties do not appear to intrude significantly into the intellectual or academic realms.

We describe 12 of 1130 infants with Down syndrome in whom various degrees of thyroid dysfunction were detected by neonatal screening. These aberrations were confirmed subsequently in 11 patients. In eight of 11 children, persistent primary hypothyroidism, was diagnosed, whereas in the remaining three patients transient thyroid abnormalities were noted. The twelfth patient died and could not be retested. We found an incidence of persistent primary congenital hypothyroidism in infants with Down syndrome of 1:141, or about 28 times more than in the general population. The cause of thyroid aberrations in these infants remains unclear; none of the studied patients had agenesis or ectopia of the thyroid gland. On initial screening most infants with Down syndrome had only mild biochemical abnormalities, with gradual decompensation occurring thereafter. Infants with Down syndrome are therefore at high risk for congenital hypothyroidism and should have careful follow-up to prevent further deterioration of their mental development or growth.

We studied intensive care unit (ICU) records of 42 children with Glasgow Coma Score ≤ 7 to better define the relation between high blood pressure (BP) and poor neurologic outcome following acute brain injury. Diagnoses included Reyes Syndrome (10), head trauma (16), anoxia (13) and CNS infection (3) and excluded those dying of circulatory failure. Corrected peak BP (in mmHg): BPc=(peak systolic BP)-(systolic BP 95%ile for age), was recorded in each 6 hour interval. Persistence of high BP was defined as BP >95%ile in 2 successive 6 hour intervals. Outcome was classified as good (mild or no deficit) or poor (death or severe neurologic deficit making patient dependent on special care). All patients' highest BP exceeded 95%ile. Highest BPc was: 1-20 in 8/42 (19%), 21-40 in 19/42 (45%), and >40 in 15/42 (36%). In those with highest BPc >20, poor outcome occurred in 19/34 (56%) while in those with highest BPc ≤ 20, poor outcome occurred in only 1/8 (13%)(p = .0316). Of those with high BP persisting until ICU discharge, 14/19 (74%) had poor outcome, while those with BP normalizing prior to ICU discharge had poor outcome in only 6/23 (26%) (p =.0026). Mean BPc in those with poor outcome tended to exceed mean BPc in patients with good outcome in each time interval from day 4 onward, with the difference reaching p<.05 on days 7,9 and 10. Severity as well as persistence of high BP identify patients with poor outcome after acute brain injury. This finding justifies further attempts to test the benefit of controlling high BP in the brain injured patient.

To characterize the children with enuresis likely to respond to desmopressin acetate, we performed a double-blind crossover study that included the use of a placebo. During the two weeks of desmopressin administration, six children (12%) had 13 or 14 dry nights, and 15 children (29%) had eight to 12 dry nights. Among the 17 children aged 9 years or older, with four to seven dry nights during the two-week baseline period, 12 children (71%) responded to desmopressin (eight to 14 dry nights). In contrast, none of the 15 children younger than 9 years of age with fewer than three dry nights before therapy responded. During the posttreatment period, only four of the 21 drug responders reported a persistent effect. Desmopressin may be effective in reducing the frequency of enuresis, especially in children older than 9 years of age without nightly enuresis.

To determine if children with growth retardation are at greater risk for academic and emotional problems than thoseof normal stature, we compared twenty-five children of normal intelligence with constitutional short stature or growth hormone deficiency to a control group with normal height matched for age, sex and socioeconomic status. On the Child Behavior Checklist, 44% of the short 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.

Hearing loss has been noted as a complication of cranial irradiation in adults,1-7but to our knowledge has not previously been described in children.8We studied a preadolescent child in whom moderate to severe hearing loss developed after radiation therapy. Report of a Case.—A 12-year-old girl had been in good health until 5 years of age, when she had begun to experience recurrent headaches. At age 6½ years, papilledema developed; skull roentgenograms showed a partially calcified suprasellar mass. She underwent subtotal resection of an optic glioma, and received postoperative radiation, 5,000 rad total tumor dose, in 25 fractions over five weeks. The radiation field included the petrous portion of both temporal bones. Examination immediately after therapy revealed alopecia in the left temporal region, and desquamation behind both pinnae. Over the following three months, headaches again developed that were treated with aspirin, 80 to 100 mg/kg/day. When

To determine the effectiveness of desmopressin (DDAVP, a vasopressin analogue) for treating enuresis, we performed a double-blind crossover study at three medical centers. Subjects (N=43) ranged from 6 to 16 years old, had normal renal function and denied polyuria, polydipsia and daytime incontinence. The study encompassed four consecutive two-week periods: pre-treatment, treatment with placebo or DDAVP (40 mcg intranasally at 8 PM), crossover treatment, and post-treatment. DDAVP did not produce adverse side effects or significantly alter blood pressure, body weight, serum osmolality or urine osmolality. The results were as follows: Thirteen of 43 children responded to DDAVP (number of wet nights <6). In 6 of the responders, the drug effect persisted into the next study period. Only 2 of 24 children responded to placebo during the first treatment period. In conclusion, DDAVP appears safe for treating enuresis. A child's response to the drug can be determined by a two-week therapeutic trial.

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 $5,000.

We simplified the procedures for identifying children with diabetes insipidus by using the relationship between urine and plasma osmolality (Uosm and Posm). We defined the normal Uosm-Posm relationship as the area within the 95% confidence regions for pairs of osmolality measurements obtained from normal children during periods of free access to fluids and after oral hydration. In 13 patients with diabetes insipidus, Uosm-Posm coordinates were outside these regions in 50 of 64 pairs (one to ten per patient) during periods of free access to fluids. After three hours of fluid deprivation, the Uosm-Posm relationship was abnormal in 12 patients; the 13th was unable to void. For those with a normal Posm, and Uosm less than Posm, another pair of osmolality measurements after three hours of fluid restriction should determine the diagnosis.

Urinary excretion of lead following administration of calcium ethylenediaminetetraacetic acid (EDTA) is widely employed as an index of the body lead burden. In our experience, as well as that of others, the relationship of blood lead to that removed in the EDTA mobilization test has been imprecise. In an attempt to identify the variables multiple factors were examined in 117 lead mobilization tests conducted in 110 patients. Variables included: age, hemoglobin, free erythrocyte porphyrin (FEP), serum iron, blood lead, and urinary lead excretion expressed as μg urine lead per mg of EDTA administered (Pb/EDTA). Analysis indicates that mobilization results can be best predicted from the blood lead and serum iron values (r = .79; F = 96.5, p & z.Lt;.001). The multiple correlation equation is:The multiple correlation equation indicates that urinary lead excretion is greater with either greater blood lead or serum iron, or both. Correlations with lead alone (r = 0.67), iron (0.65), or FEP (0.19) were not as great as the combination of lead and iron. The enhancement of lead excretion in the presence of normal, or increased serum iron concentrations suggests that iron and lead share similar binding sites and that iron displaces lead from these sites making it more available for chelation.

The complement system was studied in detail in 24 patients with Henoch Schönlein 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 (CH50). 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.