Volume 88 Number 1
Letters to the Editor
DISCUSSION
15 5
Somatomedin in cerebral gigantism
In conditions such as the viral subglottic croup syndrome, which may be evidenced by a narrowing of the airway lucency in the subglottic area, the epiglottic shadow remains narrow and "fingeflike." We have used the terms "thumb sign" and "little finger sign" in describing these diagnostic lateral neck radiographs and have been impressed by the reliability of these signs in evaluating patients in whom epiglottitis is suspected. The "thumb sign" has always been present in our patients as early as 9 when the diagnosis was first suspected, and lateral neck radiographs were taken to substantiate the diagnosis. In instances where acute epiglottltis is obvious from the clinical presentation, we immediately consult the otolaryngology and anesthesiology services for the establishment o f a secure airway either by tracheostomy or by nasotracheal intubation. W h e n the diagnosis is in question and the child's clinical condition allows, a lateral neck radiograph is obtained. We have found that the use of the "thumb sign" has been a simple and reliable aid to us in the evaluation o f patients with acute upper airway obstruction and hope it may be o f interest to others.
John K. Podgore, D.O. Infectious Disease Section Department of Pediatrics Madigan Army Medical Center Tacoma, Wash. 98431 James W. Bass, M.D. Infectious Disease Section Department of Pediatrics Trifler Army Medical Center Honolulu, Hawaii
REFERENCES 1. Rapkin RH: The diagnosis o f epiglottitis: Simplicity and reliability of radiographs of the neck in the differential diagnosis of the croup syndrome, J PEDIATR 80:96, 1972. 2. Meschan I: Analysis of roentgen signs in general radiology, Philadelphia, 1973, WB Saunders Company, p 790. 3. Caffey J: Pediatric x-ray diagnosis, ed 6, Chicago, 1972, Yearbook Medical Publishers, Inc, p 236.
To the Editor: Cerebral gigantism is a clinical syndrome which is characterized by typical facial features (hypertelorism, prominent supraorbital ridge, antimongoloid slant, high-arched palate, and prognathism), unusually large hands and feet, excessive growth in height starting from birth, advanced bone age, enlarged ventricles, nonprogressive cerebral dysfunction, and mental retardation.' The etiology of cerebral gigantism is not clear. Increased growth hormone levels have not been documented. Normal growth hormone suppression by glucose has been reported by Hook and associates.'-' Only one patient has had a paradoxical elevation of serum growth hormone during glucose loading. Adrenal and thyroid function studies were normal; insulin values during glucose loading were also normal. -~Bejar and associates 3 noted elevation of essential plasma amino acids in these patients. In view Of the normal secretion of growth hormone, it was deemed of interest to measure somatomedin activity in these patients. The endocrine evaluation consisted of measurements of basal levels of serum growth hormone and during stimulation with insulin and arginine and suppression with glucose. Somatomedin activity in the serum was determined according to the method of Wiedemann and Schwartz? RESULTS
AND COMMENTS
The five patients studied were above the ninety-seventh percentile for height (Table I). Bone age was advanced in four patients. Growth hormone responses to stimulation tests were normal in Patients K. D., M. P. and K. S. Serum somatomedin activity was found to be within the normal range defined previously (1.05 U/ml, • 2 SD 0.72--1.28) ~ in four patients. The somewhat lower value in Patient R. S. may be a reflection of the younger age of this patient. Age dependency of somatomedin activity has been suggested. ~The birth weight was increased in all five patients as evidence of excessive fetal growth. Cerebral gigantism is characterized by certain features of acromegaly but is otherwise a nonprogressive disorder, the cause
Table I
Patient K. O.
M.P.
R.S, T.S. K.S.
Sex
Height (era)
Weight (kg)
Birth weight (kg)
F F M M M
126.5 169.5 125 177 193
25.5 76.4 30 85 84
-4.8 5.4 4.5 4.4
Pubertal development (Tanner stage) I II I IV IV
Chronologic age (yr) 5 10 4 11 15
4/12 1/I2 2/12 3/12 2/12
Bone age (yr)
Baseline growth hormone (ng/ml)
8 10/12 13 5 15 6/12 17
15 8 1.3 1.2
Nadir of growth hormone during glucose loading (ng/ml) 1.2
Letters to the Editor
DISCUSSION
15 5
Somatomedin in cerebral gigantism
In conditions such as the viral subglottic croup syndrome, which may be evidenced by a narrowing of the airway lucency in the subglottic area, the epiglottic shadow remains narrow and "fingeflike." We have used the terms "thumb sign" and "little finger sign" in describing these diagnostic lateral neck radiographs and have been impressed by the reliability of these signs in evaluating patients in whom epiglottitis is suspected. The "thumb sign" has always been present in our patients as early as 9 when the diagnosis was first suspected, and lateral neck radiographs were taken to substantiate the diagnosis. In instances where acute epiglottltis is obvious from the clinical presentation, we immediately consult the otolaryngology and anesthesiology services for the establishment o f a secure airway either by tracheostomy or by nasotracheal intubation. W h e n the diagnosis is in question and the child's clinical condition allows, a lateral neck radiograph is obtained. We have found that the use of the "thumb sign" has been a simple and reliable aid to us in the evaluation o f patients with acute upper airway obstruction and hope it may be o f interest to others.
John K. Podgore, D.O. Infectious Disease Section Department of Pediatrics Madigan Army Medical Center Tacoma, Wash. 98431 James W. Bass, M.D. Infectious Disease Section Department of Pediatrics Trifler Army Medical Center Honolulu, Hawaii
REFERENCES 1. Rapkin RH: The diagnosis o f epiglottitis: Simplicity and reliability of radiographs of the neck in the differential diagnosis of the croup syndrome, J PEDIATR 80:96, 1972. 2. Meschan I: Analysis of roentgen signs in general radiology, Philadelphia, 1973, WB Saunders Company, p 790. 3. Caffey J: Pediatric x-ray diagnosis, ed 6, Chicago, 1972, Yearbook Medical Publishers, Inc, p 236.
To the Editor: Cerebral gigantism is a clinical syndrome which is characterized by typical facial features (hypertelorism, prominent supraorbital ridge, antimongoloid slant, high-arched palate, and prognathism), unusually large hands and feet, excessive growth in height starting from birth, advanced bone age, enlarged ventricles, nonprogressive cerebral dysfunction, and mental retardation.' The etiology of cerebral gigantism is not clear. Increased growth hormone levels have not been documented. Normal growth hormone suppression by glucose has been reported by Hook and associates.'-' Only one patient has had a paradoxical elevation of serum growth hormone during glucose loading. Adrenal and thyroid function studies were normal; insulin values during glucose loading were also normal. -~Bejar and associates 3 noted elevation of essential plasma amino acids in these patients. In view Of the normal secretion of growth hormone, it was deemed of interest to measure somatomedin activity in these patients. The endocrine evaluation consisted of measurements of basal levels of serum growth hormone and during stimulation with insulin and arginine and suppression with glucose. Somatomedin activity in the serum was determined according to the method of Wiedemann and Schwartz? RESULTS
AND COMMENTS
The five patients studied were above the ninety-seventh percentile for height (Table I). Bone age was advanced in four patients. Growth hormone responses to stimulation tests were normal in Patients K. D., M. P. and K. S. Serum somatomedin activity was found to be within the normal range defined previously (1.05 U/ml, • 2 SD 0.72--1.28) ~ in four patients. The somewhat lower value in Patient R. S. may be a reflection of the younger age of this patient. Age dependency of somatomedin activity has been suggested. ~The birth weight was increased in all five patients as evidence of excessive fetal growth. Cerebral gigantism is characterized by certain features of acromegaly but is otherwise a nonprogressive disorder, the cause
Table I
Patient K. O.
M.P.
R.S, T.S. K.S.
Sex
Height (era)
Weight (kg)
Birth weight (kg)
F F M M M
126.5 169.5 125 177 193
25.5 76.4 30 85 84
-4.8 5.4 4.5 4.4
Pubertal development (Tanner stage) I II I IV IV
Chronologic age (yr) 5 10 4 11 15
4/12 1/I2 2/12 3/12 2/12
Bone age (yr)
Baseline growth hormone (ng/ml)
8 10/12 13 5 15 6/12 17
15 8 1.3 1.2
Nadir of growth hormone during glucose loading (ng/ml) 1.2
