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

An adolescent with An adolescent with bone painbone pain

LYM, 17/M• 17 years old boy• C/O:

– 1 month history of scalp lump

• HPI:– Heel pain– Polydipsia, polyuria, nocturia

1 year

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)

LYM, 17/M

• X-ray skull

• Impression:– Hypercalcaemia with osteolytic

lesion

LYM, 17/M

• DDx:

– Primary hyperparathyroidism

– Malignancy

– Iatrogenic (excessive thiazide, Vit. D intoxication, lithium)

– Familial hypocalciuric hypercalcaemia

• 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

• Bone scan

X-Ray hands

X-Ray hands

USG Neck

• Further investigations– Blood test

• PTH level • 239 pmol/L (1.6-6.9)

LYM, 17/M

USG Kidney

• Final Diagnosis

– Primary hyperparathyroidism

– secondary to Parathyroid adenoma

– Complicated with bilateral nephrocalcinosis

LYM, 17/M

Fact sheetFact sheet

Primary hyperparathyroidism

• Etiology

– 85% parathyroid adenoma

• Associated with MEN I or IIa

– 10% parathyroid hyperplasia

– <1% parathyroid carcinoma

When do we need to further investigate for MEN syndrome?

• Multiple adenoma/hyperplasia

• Atypical parathyroid adenomas

• Parathyroid carcinoma

• Family history

Our patient

• No evidence of MEN syndrome

• Further management plan…

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

• 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

• 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

Definitive treatment

• Surgical removal of parathyroid gland

• Histology: parathyroid adenoma

Perioperative Perioperative management of management of

hyper/hypocalcaemiahyper/hypocalcaemia

Pre-operative assessment

• Monitor renal function

• Bone status

• ECG – Shortened QTc interval

Treatment options for hypercalcaemia

Rebound hypercalcaemia in hyperparathyroidismEffect occur ~48-72 hours after infusion

Severe hypercalcaemia

Inhibits osteoclast action and bone resorption

Pamidronate(60-90mg over 4 hours)

Causing hypokalaemia

Following aggressive rehydration

Inhibits calcium reabsorption in the distal renal tubule

Frusemide(10-20mg prn)

Lowers Ca by 0.25 to 0.75 mmol/LTo 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

LYM, 17/M

19001963201226502656ALP

0.830.940.360.560.63PO4

3.123.072.84.143.97Ca

4/828/719/716/714/7Date

Palmidronate

Palmidronate

Pre-operation

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

LYM, 17/M

2177

0.88

2.33

19/8

28931527482508218121161915ALP85-470 IU/l

0.891.090.810.770.70.790.45PO40.82-1.4 mmol/l

2.322.341.781.72.122.292.7Ca2.15-2.55 mmol/l

12/77/218/817/815/814/813/8Date

Rocaltrol 0.5 mcg BDRocaltrol 1 mcg BD

Oscal 1500 tidPost operation

Post-operative course

• Replacement

– Vitamin D (Rocaltrol)

– Calcium (Oscal)

– Phosphate

How to wean the medication?

• Monitor serum bone profile and urine calcium excretion

• Calcium Phosphate Keep rocaltrol until urine Calcium excretion become measurable

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

2302419ALP(IU/l) 24.2202PTH

(pmol/l) AdenomaAdenomaPathology Oral Ca+vitD IV Ca +vitDPost Op Tx

0.810.99PO4(mmol/l)

3.443.39Ca(mmol/l)Renal stoneBoneOrganAbdominal pain

Hip painSymptomsPatient SCPatient YCY

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

Post op complications

• 56% transient hypocalcemia

• 36% paresthesia

• 31% Chvostek sign +ve

• 7% Trousseau sign +ve

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

Summary

• Hyperparathyroidism is rare in children

• Nonspecific or late presentation

• Watch out for associated complications and syndrome