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Key Features

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Essentials of Diagnosis
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  • Abdominal pain, polyuria, polydipsia, hypertension, nephrocalcinosis, failure to thrive, renal stones, intractable peptic ulcer, constipation, uremia, and pancreatitis

  • Bone pain or pathologic fractures, subperiosteal bone resorption, renal parenchymal calcification or stones, and osteitis fibrosa cystica

  • Impaired concentration, altered mental status, mood swings, and coma

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General Considerations
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  • Hypercalcemia is defined as a serum calcium level > 11 mg/dL

  • Severe hypercalcemia is a level > 13.5 mg/dL

  • More than 80% of hypercalcemic children or adolescents have either hyperparathyroidism or a malignant tumor

  • Hyperparathyroidism is rare in childhood and may be primary or secondary

    • Most common cause of primary hyperparathyroidism is parathyroid adenoma

    • Most common cause of secondary hyperparathyroidism is chronic kidney disease with impaired phosphate excretion

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Clinical Findings

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Symptoms and Signs
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  • Due to hypercalcemia

    • Manifestations include hypotonicity and muscle weakness; apathy, mood swings, and bizarre behavior; nausea, vomiting, abdominal pain, constipation, and weight loss; hyperextensibility of joints; and hypertension, cardiac irregularities, bradycardia, and shortening of the QT interval

    • Coma occurs rarely

    • Calcium deposits occur in the cornea or conjunctiva (band keratopathy) and are detected by slit-lamp examination

  • Due to increased calcium and phosphate excretion

    • Loss of renal concentrating ability causes polyuria, polydipsia, and calcium phosphate deposition in renal parenchyma or as urinary calculi with progressive renal damage

  • Due to changes in the skeleton

    • Initial findings include bone pain, osteitis fibrosa cystica, subperiosteal bone absorption in the distal clavicles and phalanges, absence of lamina dura around the teeth, spontaneous fractures, and moth-eaten appearance of the skull on radiographs

    • Later, there is generalized demineralization with high risk of subperiosteal cortical bone

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Diagnosis

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  • Bone changes may be subtle in children

  • Technetium sestamibi scintigraphy is preferred over conventional procedures (ultrasound, computed tomography, and MRI) for localizing parathyroid tumors

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Treatment

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  • Initial management is vigorous hydration with normal saline and forced calcium diuresis with a loop diuretic such as furosemide (1 mg/kg given every 6 hours)

  • If response is inadequate, glucocorticoids or calcitonin may be used

  • Bisphosphonates are being used more often in refractory pediatric hypercalcemia

  • Resection of parathyroid adenoma or subtotal removal of hyperplastic parathyroid glands is the preferred treatment

  • Treatment of secondary hyperparathyroidism from chronic kidney disease is primarily directed at controlling serum phosphorus levels with phosphate binders

  • Pharmacologic doses of calcitriol are used to suppress PTH secretion

  • Long-term therapy for hypercalcemia of malignancy is the treatment of the underlying disorder

  • There is also an increasing role for bisphosphonates in treatment of chronic hypercalcemia

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Outcome

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Prognosis
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  • Excellent after resection of a single adenoma

  • Usually good following subtotal parathyroidectomy for diffuse hyperplasia or removal of multiple adenomas; depends on correction of the underlying defect

  • In patients with multiple sites of parathyroid adenoma or hyperplasia, MEN is likely, and other family ...

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