1. An adolescent with bone pain

2. LYM, 17/M 17 years old boy C/O: –1 month history of scalp lump HPI: –Heel pain –Polydipsia, polyuria, nocturia 1 year

3. LYM, 17/M P/E: –2cm lump over left occipital area Investigations: –Blood test Very abnormal bone profile (Ca 4.2mmol/L, ALP 2377IU/L, Cr 124umol/L)

4. LYM, 17/M X-ray skull

5. Impression: –Hypercalcaemia with osteolytic lesion LYM, 17/M

6. DDx: –Primary hyperparathyroidism –Malignancy –Iatrogenic (excessive thiazide, Vit. D intoxication, lithium) –Familial hypocalciuric hypercalcaemia

7. Further investigations –Blood test PTH level –Urine Ca/Cr 24 hr Ur Ca pending 2.5 (<0.6) 33.1 (2-7.4mmol/d) 1324mg/day (18mg/kg/day) LYM, 17/M

8. Bone scan

9. X-Ray hands

11. USG Neck

12. Further investigations –Blood test PTH level 239 pmol/L (1.6-6.9) LYM, 17/M

13. USG Kidney

14. Final Diagnosis –Primary hyperparathyroidism –secondary to Parathyroid adenoma –Complicated with bilateral nephrocalcinosis LYM, 17/M

15. Fact sheet

16. Primary hyperparathyroidism Etiology –85% parathyroid adenoma Associated with MEN I or IIa –10% parathyroid hyperplasia –<1% parathyroid carcinoma

17. When do we need to further investigate for MEN syndrome? Multiple adenoma/hyperplasia Atypical parathyroid adenomas Parathyroid carcinoma Family history

18. Our patient No evidence of MEN syndrome Further management plan…

19. Criteria for surgery in Primary hyperparathyroidism BIOCHEMICAL –Serum total calcium > 3mmol/L –Marked hyperclaciuria (urinary calcium excretion more than 400mg per day) –Impaired renal function –Nephrolithiasis Age under 50 Guidelines from the National Institutes of Health Consensus Development Conference

20. BONE –Osteitis fibrosa cystica –Reduced cortical bone density –Bone mass more than two standard deviations below age-matched controls (Z score less than 2) Criteria for surgery in Primary hyperparathyroidism Guidelines from the National Institutes of Health Consensus Development Conference

21. SYMPTOMS –Classic neuromuscular symptoms –Proximal muscle weakness and atrophy, hyperreflexia, and gait disturbance Criteria for surgery in Primary hyperparathyroidism Guidelines from the National Institutes of Health Consensus Development Conference

22. Definitive treatment Surgical removal of parathyroid gland Histology: parathyroid adenoma

23. Perioperative management of hyper/hypocalcaemia

24. Pre-operative assessment Monitor renal function Bone status ECG –Shortened QTc interval

25. Treatment options for hypercalcaemia Rebound hypercalcaemia in hyperparathyroidism Effect occur ~48-72 hours after infusion Severe hypercalcaemiaInhibits osteoclast action and bone resorption Pamidronate (60-90mg over 4 hours) Causing hypokalaemiaFollowing aggressive rehydration Inhibits calcium reabsorption in the distal renal tubule Frusemide (10-20mg prn) Lowers Ca by 0.25 to 0.75 mmol/L To achieve urine output ~4ml/kg/hr Ca>3.5mmol/L or symptomatic Enhances filtration and excretion of Ca Normal Saline CautionsIndication in Hypercalcaemia Mode of action Agent

26. LYM, 17/M 19001963201226502656ALP 0.830.940.360.560.63PO4 3.123.072.84.143.97Ca 4/828/719/716/714/7Date Palmidro nate Pre-operation

27. Acute post-operative care Admission to ICU –risk of severe hypocalcaemia with laryngospasm –Higher risk: Preoperative iPTH >25 pmol/L Frequent monitoring of clinical symptoms serum calcium –Hypocalcaemia could be delayed Early start of oral Vitamin D and calcium supplement

28. LYM, 17/M 2177 0.88 2.33 19/8 28931527482508218121161915 ALP 85-470 IU/l 0.891.090.810.770.70.790.45 PO4 0.82-1.4 mmol/l 2.322.341.781.72.122.292.7 Ca 2.15- 2.55 mmol/l 12/77/218/817/815/814/813/8Date Rocaltrol 0.5 mcg BD Rocaltrol 1 mcg BD Oscal 1500 tid Post operation

29. Post-operative course Replacement –Vitamin D (Rocaltrol) –Calcium (Oscal) –Phosphate

30. How to wean the medication? Monitor serum bone profile and urine calcium excretion Calcium  Phosphate  Keep rocaltrol until urine Calcium excretion become measurable

31. Our experiences 2 more cases in the past one year All male Age 14,16 All symptomatic All have end organ damage: nephrocalcinosis, nephrolithiasis, bone involvement All are single adenoma

32. 2302419ALP(IU/l) 24.2202PTH (pmol/l) Adenoma Pathology Oral Ca+vitD IV Ca +vitDPost Op Tx 0.810.99PO4(mmol/l) 3.443.39Ca(mmol/l) Renal stoneBoneOrgan Abdominal painHip painSymptoms Patient SCPatient YCY

33. Mayo clinic experiences 52 patients (<19 years old) 65% adenoma, 27% hyperplasia 44% of end organ damage Common symptoms: fatigue, headache, nausea and vomiting, polydipsia, etc Unremarkable physical examination J Kollars, A E Zarroug, et al Pediatrics Vol 115 No.4 April 2005

34. Post op complications 56% transient hypocalcemia 36% paresthesia 31% Chvostek sign +ve 7% Trousseau sign +ve

35. Risk factors of severe post-op hypocalcemia In adult series 2 or more parathyroid glands involved Thyroid operation iPTH >25pmol/l Previous OT on parathyroid gland Bengt Ahringberg Kald et al. Eur J Surg 2002; 168: 552-556

36. Summary Hyperparathyroidism is rare in children Nonspecific or late presentation Watch out for associated complications and syndrome