an adolescent with bone pain. lym, 17/m 17 years old boy c/o: –1 month history of scalp lump hpi:...
Post on 24-Dec-2015
221 Views
Preview:
TRANSCRIPT
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
top related