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Endocrine emergencies in adolescents G. Chiumello, M.P.Guarneri

Endocrine emergencies in adolescents G. Chiumello, M.P.Guarneri. Paediatric Department, Endocrine Unit Vita –Salute San Raffaele University, Milan, ITALY. 1656 children (age 0-15 yrs) affected by diabetes type 1 DKA 19 %, severe DKA 4% DKA 30 %, severe DKA 8 % less 2 yrs

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Endocrine emergencies in adolescents G. Chiumello, M.P.Guarneri

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  1. Endocrine emergencies in adolescentsG. Chiumello, M.P.Guarneri Paediatric Department, Endocrine Unit Vita –Salute San Raffaele University, Milan, ITALY

  2. 1656 children (age 0-15 yrs) affected by diabetes type 1 • DKA 19 %, severe DKA 4% • DKA 30 %, severe DKA 8 % less 2 yrs Diabetes Care 2010

  3. M.S. 14 yrs • 3rd year of junior high school  poor school performances • large family • disadvantaged socio-economic conditions • Diabetes (onset 6 years of age)  poor glycometabolic control respiratory distress progressive worsening of general conditions with state of consciousness alteration

  4. EMERGENCY ROOM: - pH 6,97 pCO2 17 HCO3 3,9 BEB -26,4 • glycemia 714 mg/dL • Na+ 126mmol/L, K+ 5,7 mmol/L, creatinine 1,46 mg/dL Intensive Care Unit discontinuous insulin therapy during last 10-15 days and probably interruption of treatment in the last 24-48 hours

  5. TREATMENT III PERIOD: fluid replacement and maintenance (16-24 hours): • Saline solution 0,45% + 40 mEq/L K+ • I PERIOD: volume expansion / shock treatment (1-2 hours): • 10-20 ml/Kg saline solution 0,9% • II PERIOD: fluid replacement and maintenance (6 hours): • Saline solution 0,9% + 40 mEq/L K+ (50% KCl + 50% KPO4)

  6. Gradual glycemia reduction AFTER THE FIRST PERIOD • e.v insulin (0,1 U/Kg/h) until DKAresolution then switch to subcutaneous insulin therapy

  7. A.M. 18 yrs • diabetes 13 yrs duration • HbA1c> 10% • poor glycometabolic control • good social conditions • optimal level of family care During a relative’s acute illness, she voluntarily suspended the administration of insulin for 48 hours EMERGENCY ROOM: loss of consciousness

  8. Serious DKA with oligoanuria and hyperkalemia. Admitted to intensive care unit Psychological support Persistence of poor glycometabolic control and onset of eating disorders. Found lifeless in bed dead in bed syndrome

  9. J.P.B. 17 yrs • diabetes 13 yrs duration • poor glycometabolic control with frequent asymptomatic hypoglycemia • HbA1c> 9% • arrived in Italy 1 year ago from Morocco • unsatisfactory familiar and personal compliance After insulin injection, eveningwith friends, loss of consciousness with generalized convulsions  glycemia 22 mg/dl The following year, during another serious hypoglycemia, involved in a traffic accident.

  10. Hyperthyroidism 0.1:100.000/yr (early childhood) Incidence  1:100.000/yr 3:100.000/yr (adolescence) • initial symptoms can be mild: nervousness and anxiety, irritability, emotional lability, fatigue, palpitations, tremors, insomnia, profuse sweating. Often drop in school performance • variable thyroid size, usually symmetrical increase with tense consistence • ophthalmic abnormalities less severe than in adults, with staring eyes, puffiness around the eyes, increased tear formation, irritation and unusual sensitivity to light. True exophthalmos is quite unusual

  11. SYMPTOMATIC THERAPY  beta-blocker during the first 2 weeks of therapy children  propranolol adults  atenolol METHIMAZOLE / PROPYLTHIOURACIL (not available in Italy)

  12. Thyrotoxic crisis

  13. Causes surgery diabetes ketoacidosis sepsis status epilecticus burns thyroxine overdose is not a cause of thyrotoxic crisis

  14. METHIMAZOLO/PROPYLTHIOURACIL ev: block of thyroid hormones synthesis LUGOL’S IODINE: oral or nasogastric administration inibition of FT4 / FT3 conversion CORTISONICS: useful for their collateral inhibition of peripheral conversion of T4 to T3. Dexamethasone: 8-10mg/die in 3 administrations Hydrocortisone: 100-300mg/die in 3 administrations • BETA-BLOCKERS: • if signs of marked adrenergic activity (psychosis, extreme agitation, hyperexcitability) • PROPRANOLOL: • 0.1 mg/kg (up to 2mg) in 5-10 min ev • 2 mg/kg/die every 4-6 hours (max 6mg/kg/die)

  15. Acute adrenal insufficiency • DEFECTS OF ADRENAL STEROIDOGENESIS - congenital adrenal insufficiency- 21OHD- other enzyme defects • ADDISON D. • - autoimmune disease • - genetic defects • - adrenal hemorrhage • - adrenoleukodystrophy • SECONDARY ADDISON D. Panhypopituitarism

  16. P.G. 17 yrs ICS • Holidays in Egypt with friends • Fails to inform friends about his underlying disease • Acute gastroenteritiswith severe evolution and fever • Admitted after 36 hours of symptoms in intensive care unit

  17. loss of consciousness, • severe hypotension • dyselectrolitemia Parents, alerted by friends, informed local doctors about son’s disease (ICS) Diagnosis:Acute adrenal insufficiency in ICS Adequate therapy

  18. Replacement therapy: • Glucocorticoid (hydrocortisone) • Mineralcorticoid (deoxycorticosterone) • Absolute need of: • good therapeutic compliance • therapeutic management

  19. Acute adrenal crisis • Causes: • Infections • Surgery • Severe stress • Accidents and injuries • Strenuous physical activity • Therapeutic non-compliance • Clinical features: • Anorexia • Nausea • Vomiting • Abdominal pain • Hypotension • Dyselectrolitemia • Hypoglycemia • Fever • Lethargy • Shock

  20. GLUCOSALINE SOLUTION Glucosesolution 5% withNaCl (10-5mEq/kg/die) 120-150 ml/kg/die, half in first threehours GLUCOCORTICOID Flebocortidendovenous 2-3 mg/kg (bolus) then 5mg/kg in threehours then 10mg/kg/die MINERALCORTICOID Deoxycorticosteroneacetatei.m. 3 mg/die

  21. PANCREATIC NEUROENDOCRINE TUMORS IN PEDIATRIC AGE • Prevalence in children: 0.005-0.007 per 100,000 (M:F = 9:10) • 32,8 % of pancreatic tumors • Unusual before 10 years (1.2%). Average age at diagnosis>40 years • More aggressive in children • Survival rate at 15 years: 50% J. Pediatric Surgery 2008

  22. 87% accidentally found Sugical choice:distal pancreatectomy / DCP

  23. CLINICAL PRESENTATION • Accidental ultrasound/radiologic diagnosis (lesions < 1 cm) • Clinical syndromes related to endocrine mediators peptides production (sometimes accidentally discovered at blood tests) MAIN PANCREATIC NET AND ASSOCIATION TO MEN1 Tumor Secreting Peptides Symptomatology -Insulinoma (5-10%) Insulin, PP, VIP Hypoglycemia signs and symptoms -Glucagonoma (10%) Glucagone, PP Erythema Calcitonin Hyperglycemia Gloss/stomatitis -Gastrinoma (25-40%) Gastrin, VIP, PP Zollinger Ellison Neurotensina Syndome -VIPoma (5%) VIP Verner MorrisonSyndrome -Silent functional endocrine tumor (20%)

  24. CLINICAL PRESENTATION Specific symptoms not always present: catch down growth, weight loss. Possible accidental diagnosis. Confounding clinical features for multiple hormons. GLUCAGONOMA •necrolytic migrant erythema(70%) •hyperglycemia (83%) •glossitis and stomatitis(34%) •weight loss (66%) •thromboembolic disease(30%) •occasionally, but not always present, nausea and vomiting • ZOLLINGER ELLISON SYNDROME • Multiple peptic ulcers prone to perforation • Diarrhea • Identification of tumor • Weight loss • VERNER MORRISON SYNDROME • •Pancreatic cholera: non continues diarrhea, even while fasting, with faecal volume over 700 ml/die • Electrolytic alterations(ipokaliemia, acloridia) • Dyspepsia (inhibition of acid and peptic secretion)

  25. DIAGNOSTIC PROBLEMS • multiple hormonal involvement • MIXED TUMOR: neuroendocrineand neoplastic exocrinetumors • possible changes in tumor secretion (spontaneous or treatment-induced)

  26. A.O. 9 yrs Catch down growth  GH: Clonidine 29.3 ng/ml, • GRF + Arginine > 35 ng/ml • IGF1 50-90° p.le • TSH 6.6 mcu/ml; FT4 1.08 ng/dl. • Thyroid Ultrasound: image suggestive of chronic mild diffuse thyroiditis. • Bone age = statural age •  Hypothalamic-pituitaryRM: normal • Persistence velocity rate ≈ - 2SDS • TSH: 9.36…6.28; FT4: 1.45…1.12 • Eutirox 1,4 mcg/kg/die TSH normalization • urinary cortisol 24h: 122...125…240 mcg/24h. (v.n. 25 – 75), ACTH: normal (34.4 pg/ml) • HypercortisolismHypothesis:dexamethasone suppression test at low doses: adequate inhibition of cortisol and ACTH T

  27. Hypercortisolism Hypothesis? • Suppression test at low doses (Dexamethasone 0,5 mg x 4/die for 2 days) • Cortisol 3 mcg/dl (v.n. < 1,4)  Cushing syndrome  admission to our Department • Absence of circadian rhythm • Cortisol 12 p.m. (242…162 ng/ml v.n. <44) • Urinary and salivary cortisol: • DexamethasoneTest: appropriate cortisol and ACTH inhibition • FUNCTIONAL HYPERCORTICISM • Abdomen ultrasound:adenoma 5x4x4,5 cm (pancreatic tale) • TAC e RM: NEUROENDOCRINE TUMOR • Markers: Glucagon +++  Glucagonoma • Laparotomic partial pancreatectomy

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