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Insulinoma in Childhood

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Insulinoma in Childhood Arch Dis Child: first published as 10.1136/adc.44.236.435 on 1 August 1969. Downloaded from Arch. Dis. Childh., 1969, 44, 435. Insulinoma in Childhood JILLIAN R. MANN, P. H. W. RAYNER, and A. GOUREVITCH From the Children's Hospital, Birmingham Hypoglycaemia is an important cause of cerebral sulthiame, and primidone, she continued to have damage in childhood. While much attention has generalized convulsions approximately twice weekly, recently been directed to its detection and treatment frequently in the early morning. She attended a in the neonatal period, in later childhood the junior school where she was noted at times to appear tired and drowsy. diagnosis is often delayed. There are many In July 1967, at the age of 124 years, she was again possible causes of hypoglycaemia in childhood, admitted to hospital. On the day before admission but in only a minority of cases is a precise diagnosis she had been unusually drowsy, and the following made, the majority being labelled 'idiopathic'. morning she was completely unrousable. On exami- An insulin-secreting islet cell adenoma of the nation she was in deep coma, responding only to pancreas (insulinoma) is a rare cause of childhood painful stimuli. She was flushed and sweating, pulse hypoglycaemia; Drash and Schultz (1967) found 135/min. Blood pressure 110/80 mm. Hg. There fewer than 30 cases in their review. The case of a were no other abnormal physical signs. Height 12-year-old girl with an insulinoma is described in 144-8 cm., weight 35-8 kg., early pubertal development to establish the incidence present. She is the third child in a family of 7. She this paper. In an attempt was born by vertex delivery at term, birthweight of the condition in the United Kingdom, the heads 2947 g. (64 lb.). An exchange transfusion had been of undergraduate and postgraduate Paediatric performed after birth for Rhesus incompatibility. A copyright. Departments were asked if any cases of insulinoma younger brother had 'febrile' convulsions until 4 years had been encountered by them during the previous of age, and one cousin developed diabetes mellitus in 10 years. 4 cases were reported to us and brief childhood. details of these patients are also presented. In the adult the incidence of insulinoma is Initial investigations and treatment. The history higher and the diagnostic criteria more clearly and physical findings suggested hypoglycaemic coma. defined than in childhood. Our experience and Initial investigations gave the following results: blood Floyd et sugar 28 mg./100 ml., CSF sugar 23 mg./100 ml., http://adc.bmj.com/ that of others (Drash and Schultz, 1967; CSF protein 40 mg./100 ml. Plasma electrolytes, al., 1964) suggests that diagnostic procedures, such urea, calcium, and phosphorus were normal, but the as the tolbutamide test, are frequently unhelpful in plasma alkaline phosphatase was 60 KA units. Serum childhood. The diagnosis and treatment of the salicylate 1 4 mg./100 ml., and plasma proteins and condition in childhood is discussed. transaminases were normal. The skull and chest x-rays were normal, and the skeletal maturation was Case History assessed as 13 years (Greulich and Pyle). An EEG revealed slow activity of high amplitude bilaterally. Christine H. was first seen in July 1966 at 114 years on September 28, 2021 by guest. Protected of age with a 1-month history of attacks during which The hypoglycaemia was controlled with difficulty; she stared vacantly or became unsteady on her feet intravenous dextrose 10 g. (as 50% solution) and hydro- for a few seconds. On the day of admission she had a cortisone 100 mg. were given, after which the blood generalized convulsion which was preceded by giddiness sugar rose to 66 mg./100 ml. Glucagon 1 mg. was given, and nausea. No abnormal physical signs were found. and normoglycaemia was maintained with an intra- Investigations revealed fasting whole blood sugar venous infusion of dextrose 10% and hydrocortisone, 54 mg./100 ml., serum calcium 9 5 mg./100 ml., serum together with dextrose feeds via an intragastric tube. phosphorus 4-5 mg./100 ml., serum alkaline phos- Restoration of the blood sugar to normal produced no phatase 32 KA units. Skull x-ray was normal. An immediate change in her conscious level which improved EEG was abnormal and suggestive of centrencephalic slowly during the subsequent 2 days. Corticosteroid epilepsy. A diagnosis of idiopathic epilepsy was made therapy was discontinued after 4 days. A period of and anticonvulsant therapy started. Despite a variety extreme restlessness and aphasia followed and a left of drugs which included phenytoin, ethosuximide, hemiparesis was present. During the following 3 weeks her speech improved, though remaining slurred, Received December 13, 1968. and she became able to walk with support. 435 Arch Dis Child: first published as 10.1136/adc.44.236.435 on 1 August 1969. Downloaded from 436 Mann, Rayner, and Gourevitch TABLE I 140 0 Plasma Insulin and Blood Sugar Levels 130 %0 - Pre-operative 120 %% 6O-bmonths post-operative 110 - °XX Glucose(m /IOOml.) Plasma Blood Insulin (yUml.) Insulin Sugar Comment 100- (,uU/ml.) (mg./100 ml.) I 90 \ 28 On admission before treatment 80' 32 After intravenous dextrose 196 66 After intravenous dextrose and 70' hydrocortisone 60 213 134 After 3-hour fast, while on oral steroids 50 - 91 112 After 8-hour fast 85 52 After 10-hour fast 401 120 After 10-hour fast 30 4 98 148 Fasting, day of operation 20 - 91 160 During operation 49 148 Immediately after operation 10- 10 Fasting, 1 day after operation 30 Fasting, 4 days after operation 9 Fasting, 4 weeks after operation 0 15 30 45 60 75 90 105 120 22 Fasting, 6 weeks after operation Time after injection (min.) FIG.-Tolbutamide tests (O 5 g./kg. intravenously). * Before operation; 0 6 months after operation. Further investigations and treatment. A pro- limit of normal fasting levels in children in this age- visional diagnosis of insulinoma was made on the basis group is 25 MU/ml. in our laboratory.) A glucose of the severity of the initial hypoglycaemia, and the tolerance test (Table II) 2 weeks after admission lack of evidence of other potential causes in this age- showed a diabetic curve, but this result was felt to group such as poisoning, pituitary or adrenal disease, reflect the corticosteroid therapy and glucose supple- liver disease, and diabetes. This was supported by ments which had been required to control the hypo- plasma insulin determinations which were performed glycaemia. An intravenous tolbutamide test (Fig.) copyright. on a number of occasions, consistently raised random showed a pronounced hypoglycaemic response, the and fasting levels being obtained (Table I). (The upper blood glucose of 11 mg./100 ml. at 30 minutes rising to TABLE II Glucose Tolerance Tests http://adc.bmj.com/ Time (hours) ! . I . Dose of Glucose 0 1 1i 2 2i 3 4 (g./kg.) Pre-operative - Sugar (mg./100 ml.) 84 276 186 117 76 88 68 61 1-0 1 mth. after operation i Sugar (mg./100 ml.) 109 236 244 132 112 80 100 110 110 on September 28, 2021 by guest. Protected 2 mth. after operation Glucose (mg./100 ml.) 102 121 72 44 100 - - 63 1-75 Insulin (tiU/ml.) 18 220 49 18 70 - I _ 6 Growth hormone (ng./ml.) 13 1 1 1 2 _ - 13 5 mth. after operation Glucose (mg./100 ml.) 75 141 108 90 - 55 - - 1-75 Insulin (,uU/ml.) 34 212 198 150 - 28 _ 7 mth. after operation Glucose (mg./100 ml.) 69 107 100 80 59 - _ - 1 75 Insulin (,uU/ml.) 24 180 159 91 32 - 12 mth. after operation Glucose (mg./100 ml.) 63 106 100 - 78 - 60 56 1-75 Insulin (,tU/ml.) 14 58 82 - 43 - 14 17 Growth hormone (ng./ml.) 15 <1 <1 - 1 < 1 17 Arch Dis Child: first published as 10.1136/adc.44.236.435 on 1 August 1969. Downloaded from Insulinoma in Childhood 437 only 18 mg./100 ml. at 120 minutes. A slightly raised formed at intervals, and the results are given in Table II. fasting plasma insulin level of 36 1uU/ml. was recorded, The glucose response has remained within normal but the subsequent insulin response was within normal limits but the insulin response, which was initially limits. Despite the absence of tolbutamide-induced raised, has progressively returned to normal during a hyperinsulinism, the clinical picture, together with 12-month follow-up period. A tolbutamide test (Fig.) increased random and fasting insulin levels, was felt to performed 6 months after operation again provoked a be sufficiently typical of pancreatic insulinoma to justify pronounced fall in the blood glucose levels, and surpri- an exploratory laparotomy, which was performed by singly the insulin response was within the abnormal one of us (A.G.) 1 month after admission. range (136 ptU/ml. at 71 minutes). However, no further episodes of hypoglycaemia have been recorded, Operative findings and procedure. The laparo- and x-rays of the chest and bones have shown no evi- tomy was performed through a right paramedian dence of metastases, so we do not consider that another incision. A round, dark red tumour, 1 cm. in dia- insulinoma is present. meter, was found in the pancreas at the junction of The hypoglycaemia produced evidence of cerebral the body and the tail. An accessory spleen was noted. damage which was initially severe, but her recovery has Partial pancreatectomy and splenectomy were per- been better than was anticipated. Aphasia and left formed, leaving the head of the pancreas and the hemiparesis were apparent when she regained conscious- accessory spleen intact.
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