A child is reported with adrenocortical unresponsiveness to ACTH and autonomic dysfunction. The latter consisted of cold extremities, progressive loss of tear production, the development of achalasia of the esophagus, pupillary dysfunction, and an abnormal histamine skin test. These findings suggest progressive parasympathetic denervation as a cause for the adrenocortical abnormality. (J Child Neurol 1988;3:174-176).
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References
1.
Migeon CJ, Kenny FM, Kowarski A., et al: The syndrome of congenital adrenocortical unresponsiveness. Pediatr Res1968; 2:501.
Franks RC, Nance WE: Hereditary adrenocortical unresponsiveness to ACTH. Pediatrics1970;45:43-48.
4.
Monteleone JA , Monteleone PL: Hereditary adrenocortical unresponsiveness to ACTH—another case. Pediatrics1970 ; 46:321-322.
5.
Kelch RP, Kaplan SL, Biglieri EG, et al: Hereditary adrenocortical unresponsiveness to adrenocorticotropic hormone. J Pediatr1972;81:726-736.
6.
Kershnar AK, Roe TF, Kogut MD: Adrenocorticotropic hormone unresponsiveness: Report of a girl with excessive growth and review of 16 reported cases. J Pediatr1972;80:610-619.
Allgrove J., Clayden GS, Grant DB, Macaulay JC: Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet1978;1:1284-1286.
9.
DiGeorge A. : Disorders of the adrenal glands, in Berman RE, Vaughn VC (eds): Nelson Textbook of Pediatrics. Philadelphia, WB Saunders Co, 1983 , p 1479.
10.
Baum JC: Systemic disease associated with tear deficiencies. Int Opthalmol Clin1973;13:157-184.
11.
Axelrod FB, Branom N., Becker M., et al: Treatment of familial dysautonomia with bethanecol (urecholine) . J Pediatr1972;81: 573-578.
12.
Croll WF, Duthie RJ, MacWilliam JA: Postural hypotension: Report of a case. Lancet1935;1:194-198.
13.
Guttman L.: Topographic studies of disturbances of sweat secretion after complete lesions of peripheral nerves. J Neurol Neurosurg Psychiatry1940;3:197-210.
14.
Moser HW, Moser AE, Singh I., O'Neill BP: Adrenoleukodystrophy: Survey of 303 cases: Biochemistry, diagnosis, and therapy . Ann Neurol1984;16:628-641.
15.
Brunt PW, McKusick VA: Familial dysautonomia. A report of genetic and clinical studies and a review of the literature. Medicine1970;49:343-374.
16.
Kure K., Wada Y., Okinaka S.: The spinal parasympathetic: The nerve supply of the suprarenal gland . Q J Exp Physiol1931; 21:227.
17.
Unsicker K. , Groschel-Stewart U.: Distribution of contractile proteins and adrenergic nerves in the adrenal gland of guinea pig, rat and ox as revealed by immunofluorescence and the glyoxylic acid technique . Experientia1977;34:102-105.
18.
Karteszi M. , Dallman MF, Makara GB, Stark E.: Regulation of the adrenocortical response to insulin-induced hypoglycemia. Endocrinology1982;111:535-541.
19.
Ottenweller JE, Meier AH: Adrenal innervations may be an extrapituitary mechanism able to regulate adrenocortical rhythmicity in rats . Endocrinology1982;111:1334-1338.
20.
Appenzeller O. : Clinical Autonomic Failure. New York, Elsevier, 1986:125.
