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ACROMEGALY and GIGANTISM Including a NEW SYNDROME in CHILDHOOD by S

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ACROMEGALY and GIGANTISM Including a NEW SYNDROME in CHILDHOOD by S Postgrad Med J: first published as 10.1136/pgmj.26.294.201 on 1 April 1950. Downloaded from 201 ACROMEGALY AND GIGANTISM Including A NEW SYNDROME IN CHILDHOOD By S. LEONARD SIMPSON, M.A., M.D.(Cantab.), F.R.C.P.(Lond.) Physician (with charge of Endocrine & Diabetic Clinics), Willesden General Hospital ; Endocrinologist, Princess Louise Children's Hospital; Consultant Endocrinologist, St. Mary's Hospital The characteristic endocrine giant is a patient his eighteenth birthday, i80 cm. (6 ft.), and at 22 with an eosinophil adenoma of the anterior years, 224 cm. (7 ft. 6 in.). Extreme prognathism pituitary gland, the hypersecretion of growth hor- and coarsening of the features were indicative mone having been operative before union of the of acromegaly, but the bones remained slender. ephiphyses of the long bones and subsequently The genitals were entirely infantile, and no producing the added complication of acromegaly secondary sexual characteristics were present. if the hypersecretion continues in late adolescence The sella turcica was deformed and three times or adult life. In relatively minor degree both the normal size. gigantism and acromegaly occur more frequently Acromegaly, not unexpectedly, tends to occur without an actual eosinophil adenoma and tend to in tall people and Davidoff (1926) found in one have a high incidence in some families and indeed series that the average height of men before the in some races, e.g. the Swedes. These are ob- disease commenced was 6 ft. 2 in., and in women viously genetically determined variations of nor- 5 ft. 6 in. mality. by copyright. Clinical accounts of gigantism go back to 1567 Acromegaly and the Thyroid (quoted by Sternberg, 1897), but fables and folk- The thyroid gland may be appreciably enlarged lore carry them back to much earlier times and in acromegaly and the basal metabolism moderately we may include the biblical tale of Goliath and raised. If there is any truth in the pituitary thyro- David. Pierre Marie used the name 'acro- trophic cause of thyrotoxicosis, it might well be megalie ' in i866, Minkowski connected the disease substantiated by typical findings in acromegaly, with the pituitary gland in 1887, and Benda but clinical thyrotoxicosis is a rarity, especially the associated it with the eosinophil cells in I900. picture of Graves' disease. Cushing and Davidoff and Davidoff established found an increased basal metabolism Cushing (1927) firmly (1927) http://pmj.bmj.com/ the relation with eosinophil cell adenoma, or hyper- (average i8 per cent.) in 49 of 72 cases of acro- plasia; and Cushing in 1912 recorded an interest- megaly. Davis (I940) in a series of 135 cases (of ing case of acromegaly in a man of 33, with a a total of i66 cases) found an average increase of primary cerebellar cystic tumour, a secondary i8 per cent. in 16 cases, and an average decrease hydrocephalus, and hyperplasia of the eosinophil of minus 7 per cent. in 17 cases, the remaining 2 cells of the anterior pituitary gland. being normal. He found enlargement of the It is not the author's intention to spend time on thyroid gland in 86 of the i66 cases and obvious the conventional descriptions of these disorders adenomata in 53 of these 86 cases, the adenomata on September 26, 2021 by guest. Protected which are to be found in any good textbook, but varying from small nodules to large tumours that rather to draw attention to less obvious features caused tracheal compression. As to correlation, and problems. the group of cases in which the thyroid gland con- Acromegaly does not usually manifest itself tained definite adenomata comprised the cases in until adolescence or adult life, the excess of growth which there was the greatest increase of meta- hormone having its initial point of action on the bolism (mean B.M.R. plus 24 per cent.); in the epiphyseal cartilage of the long bones. However, group of cases in which the thyroid gland was pal- Atkinson (I93I) recorded one case at the age of pable, the B.M.R. average was plus i per cent.; eight years and noted in the literature 25 cases in and the group in which the thyroid was not pal- childhood. F. Mainzer and E. Yalausis (I937) pable clinically had an average B.M.R. of plus 9 describe a case of giantism and acromegaly in a per cent. Resection of the thyroid gland was boy of 14 following a severe fall, concussion and carried out in 27 cases for 'one or more of the unconsciousness for three days. This was fol- following reasons :-respiratory obstruction, pre- lowed by progressive weakness and very rapid sent in 7 cases; evidence of hyperthyroidism, growth, although there was no measurement until present in 24 cases, and hypertensive heart disease Postgrad Med J: first published as 10.1136/pgmj.26.294.201 on 1 April 1950. Downloaded from 202 POSTGRADUATE MEDICAL JOURNAL April 1950 in association with hyperthyroidism which was also varies in intensity with the spontaneous present in 5 cases.' There were five deaths in the variations in the course of the primary acromegaly. series, and Davis regards these cases as poor sur- The diabetes of acromegaly may be very resistant gical risks. The B.M.R. after thyroidectomy was to insulin and several hundred units may be re- recorded in only 12 cases and of these it decreased quired daily. The renal threshold may be high in io and was unchanged in 2. The decrease and frequent blood sugars essential for an accurate varied from 2 to 53 with an average of 13 per assay of the severity of the disorder. Fasting blood cent. sugars may be normal or near normal and yet be Fortunately, only five patients received pre- followed by a severe diabetic type of carbohydrate operative iodine so that the pathological anatomy tolerance curve. Polyuria, polyphagia and poly- of the thyroid gland was undistorted. In no less dipsia may be symptoms of acromegaly even in the than 23 cases the histological picture was des- absence of diabetes. Coggeshall and Root (I940) cribed as ' adenoma in colloid thyroid'; in I as describe I6 cases of diabetes mellitus in acro- colloid thyroid gland, and in 3 only as diffuse megalics of which 3 died in diabetic coma. The parehchymatous hypertrophy. Cushing and average interval between the onset of acromegaly Davidoff (1927) found that surgical removal of a and diabetes was nine years, but varied between pituitary eosinophil adenoma in patients with one and 22 years. Three of their cases were acromegaly, goitre and raised B.M.R. resulted in a secondary to giantism without acromegaly. They fall of B.M.R. as striking as the effect of thy- quote White's findings to the effect that a con- roidectomy. It would therefore appear probable siderable proportion of diabetic children are that the thyrotropic hormone is secreted in excess above the average height at the onset of their in acromegaly, and we then must ask ourselves the diabetes and also show earlier bony, dental and question why does it in the majority of cases sexual development. This obviously raises the produce a colloid adenomatous goitre and not the question of the primary pituitary origin of or- diffuse parenchymatous hyperplasia of Graves' dinary diabetes mellitus. disease ? We cannot reject the thyrotropic by copyright. stimulus mechanism theory of Graves' disease Acromegaly and the Parathyroids since injection of thyrotropic hormone in some Bauer and Aub (1940) reviewed the experimental species of animals does produce this hyperplrsia and clinical evidence of correlation between the picture. We must, therefore, conclude that the two. An alkaline extract of the anterior pituitary character of the response of the thyroid gland is at gland caused hypertrophy ofthe parathyroid glands least as important a factor as the stimulus. in rabbits and rats. They noted a case reported by The above facts gleaned from acromegaly, as Scriver and Bryan of acromegaly with osteoporosis well as others learned from certain types of and a negative calcium balance, and studied four in own series. found an Cushing's disease, cannot be ignored in any acromegalics their They http://pmj.bmj.com/ attempts to theorize about the problems of toxic increased urinary excretion of calcium and phos- goitre. phorus but no change in faecal or serum values. They excluded hyperthyroidism as a factor, as in Acromegaly and Diabetes Mellitus this disorder the faecal as well as the urinary ex- The occurrence of diabetes mellitus as a com- cretion of calcium and phosphorus is increased. plication of acromegaly has long been known, and They found that the excretion of calcium varied the work of Young (1937) on the pituitary dia- with the phase of activity of the acromegaly and betogenic hormone gives a theoretical basis for the could be reduced by deep radiation of the pituitary on September 26, 2021 by guest. Protected clinical facts. Cushing and Davidoff (I927) gland, but did not regard proof of secondary found 12 cases of diabetes mellitus in a group of hyperfunction of the parathyroids as absolute. o00 acromegalics. It is often stated that the diabetes of acromegaly is relatively mild and in- Acromegaly and the Gonads nocuous, but in a series of autopsies Cushing and In adults there may be an initial increase in Davidoff (1927) found that of 44 acromegalics no libido and potency, but soon impotence follows, less than ii died in diabetic coma. The initial or it may be one of the earliest symptoms. These diabetes may well be entirely pituitary in origin, two phases may also be found in adolescence, and but later there is probably degeneration of the in spite of normal or large genitals there may be islets as in Young's dogs, and a superimposed incomplete secondary sexual characteristics.
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