Primary Hyperparathyroidism in an Adolescent Girl

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Article Information:


Group: 2006
Subgroup: Volume 4, Issue 3, Summer
Date: January 2006
Type: Original Article
Start Page: 176
End Page: 179

Authors:

  • A Rashaud
  • Institute of Endocrinology and Metabolism, Iran University of Medical Sciences, Tehran, IR.Iran
  • M Najafizadeh
  • Institute of Endocrinology and Metabolism, Iran University of Medical Sciences, Tehran, IR.Iran
  • MA Ghoraian
  • Institute of Endocrinology and Metabolism, Iran University of Medical Sciences, Tehran, IR.Iran

      Correspondence:

      Affiliation: Institute of Endocrinology and Metabolism, Iran University of Medical Sciences
      City, Province: Tehran,
      Country: IR.Iran
      Tel:
      Fax:
      E-mail: meh_naj2000@yahoo.com

Abstract:


The aim of this paper is to present the case of a 15.5 year old girl with primary hyperparathy-roidism (PHPT) with symptoms of bone pain that appeard 2-3 years prior to admission; she had suffered bone fracture six months earlier and had high serum calcium, very high serum PTH and low serum phosphorous levels, all re-sults being in agreement with PHPT. The diagnosis was confirmed by imaging studies with 99m TC-Sestamibi scan, showing an ade-noma in parathyroid tissue. The adenoma was removed by surgical operation. After surgery the patient was treated by high doses of calcium and vitamin D to avoid postoperative hypocalcemia.

Keywords: Primary hyperparathyroidism;Hy-percalcemia

Manuscript Body:


Introduction

Primary Hyperparathyroidism (PHPT) is a generalized disorder of calcium, phosphate and bone metabolism that results from increased secretion of the parathyroid hormone; it most commonly occurs in adults especially the elderly. However primary hyperparathyroidism does occasionally occur in children.1,2 PHPT may go undetected for years because of the minimal symptoms of the disease.2 The infrequency of screening lab tests in pediatric patients’ laboratory studies, including calcium and PTH levels, results in significant delays that often occur between onset of the symptoms and the time of diagnosis of primary hyperparathyroidism. Early diagnosis of the disease can help avoid metabolic consequences of hypercalcemia like hypercalcuria, nephrolithiasis, nephrocalcinosis, CNS damages, as well as bone fractures and other complications2. In a study conducted by Kollars et al3 on 52 patients who underwent parathyroid resection between 1970 and 2000, the most common presenting symptoms reported were fatigue and lethargy, headache, nephrolithiasis, nausea, abdominal pain, vomiting and polydipsia.3 An adenoma of one or more parathyroid glands is the most common cause of the disease.4 It has been suggested that bone disease is more common whereas renal disease is less common in children.5,6 The classical presentation of PHPT with nephrolithiasis, cystic bone disease and soft tissue calcification has become less common and has been replaced increasingly by patients presenting with mild hypercalcemia and few or no symptoms.7

Case Report

A 15.5 year old girl was admitted to the Institute of Endocrinology and Metabolism for evaluation of short stature. Her menstruation onset was three years prior to admission. Height was 147cm and weight 34 kg. On admission the patient had genovalgus and rather severe pain in her knees, that had begun about 2-3 years prior to admission. About 6 months before admission there was a fracture on the femur just above the knee following mild trauma (pathologic fracture). On physical examination diffuse bilateral goiter was noticed. There was a nodule under the lower pole of the right lobe of the thyroid, dimensions approx 2x2x2cm. Laboratory examinations revealed the following results: T3: 1.8ng/mL (normal:0.8-2.0), T4: 4.9 µg/dL, (normal: 4.5-12), T3 Resin-uptake: 25% (normal:25-35%), TSH, IRMA: 1.2 mIU/L (normal: 0.3-4.0).

Results for evaluation of parathyroid function showed high serum and 24-hour urine Ca levels, low inorganic phosphorous, high alkaline phosphatase activity, and alkaline pH of all urine samples. The most important finding was extremely high serum PTH levels; (Table 1). Radioisotope scan of thyroid gland with Technesium 99m reported decreased iodine uptake by thyroid lobes and increased background activity with a non functioning nodule. Radiography of carpal and metacarpal bones, revealed osteoporosis and preosteal resorption of these bones, especially on the radial part, suggesting hyperparathyroidism (Fig. 1). According to the MIBI parathyroid scan report following injection of TC-99 MIBI, early images showed a focus of tracer uptake in the lower portion of the right thyroid lobe. On delayed images, an area of increased uptake adjacent to the lower pole of the right thyroid lobe was noticed, a finding in favor of abnormal functioning tissue (parathyroid adenoma) (Fig. 2).

 

Fig.1. Radiography of hand

 

Fig.2. MIBI Parathyroid scan

Surgical operation was performed for dissection of the nodule (right lower parathyroid ectomy). The dimensions of the adenoma were 1x 2 cm and its weight was 2 grams. The pathology report confirmed parathyroid adenoma. After surgery, high doses of calcium and vitamin D3 were prescribed to avoid hypocalcemia due to hungry bone syndrome. Postoperative laboratory results (2 weeks after surgery) are shown in Table 1

 

Table 1. Laboratory findings prior to surgical and after operation

Variable  
Before After Reference range
Serum Ca (mg/dL)  
12.4 9.5 8.5-10.5
Serum Phosphorus (mg/dL)    2.7 3.2

Child.: 4.5-6.5

Adults: 2.5-5.0

Serum alkaline phosphatase
(IU/L)
1555
- Up to 444
24hr urine Ca (mg/24hr)  
432 65 100-300
 24 hr urine phosphorous
(mg/24hr) 
600
189
400-1000
 Serum iPTH (ng/mL)  
2048
51
13-54
 24 hr urine creatinine (g/24hr)  
0.6
0.5
 0.6-1.8

Discussion

Primary hyperparathyroidism is a rare but serious disease, which in most cases is diagnosed by chance. Symptoms are often minimal or the disease is asymptomatic.8In the study by Kollars et al,3 it was found that PHPT is more common in adolescents than younger children (median age was 16.8 years) and the most common presenting features reported in their study were fatigue and lethargy, headache, nephrolithiasis, nausea, abdominal pain, vomiting and polydipsia. In the present study, patient symptoms included fatigue, lethargy, abdominal pain and polydipsia. Most of the symptoms are consistent with the Kollars et al report. They found that PHPT is most commonly due to a single adenoma and, in all cases, surgery had been curative. In a retrospective analysis of 16 cases of PHPT aged 10.5-20 years, 11 (85%) had single adenomas and surgery was curative in all cases. In a similar study 80% of patients with PHPT had parathyroid adenoma.9,10
All the above mentioned findings are consistent with our report. Hyperparathyroid syndromes including multiple endocrine neoplasia (MEN) type I and type IIA, familial hyperparathyroidism and familial cystic parathyroid adenomatosis may be seen in children.4 When familial PHPT is diagnosed in a child, other members of the family should be screened for MEN types I and IIA.In assessing children with hypercalcemia, it is important to exclude familial hypocalcuric hypercalcemia, which may occasionally cause elevated PTH levels. This syndrome has a benign course and parathyroid surgery is not recommended for these patients.11 Considering elevated 24 urine calcium levels, the patient in present study did not qualify for this category.
According to the recommendations of the panel of world experts on issues related to PHPT, at a 2 day conference, the value for the total calcium concentration to be regarded as a criterion for surgery was set at 1mg/dL (0.25mM) above the upper limits of normal;12 it was also suggested that a number of possible risk factors be considered for development of complications of primary hyperparathyroidism including:

-Serum calcium concentrations of 1 mg/dL above accepted normal range.

-Confirmed 24hour urinary calcium of more than 400mg.

-Patients under 50 years of age and

-Bone density at the lumbar spine, hip, or distal radius of more than 2.5SD below peak bone mass.
Surgery was recognized as the only definitive therapy for primary hyperparathyroidism and was acknowledged to be virtually an appropriate course of action. Accurate localization of adenoma prior to surgery is necessary for a successful operation. Using 99m Tc-sestamibi scintigraphy, a sensitive and specific parathyroid scan may be performed, resulting in reduction of operation time and morbidity.13,14 This technique was successfully used for localization of the adenoma in our patient.Postoperative hypocalcemia may be caused by permanent or transient hypoparathyroidism or extensive skeletal remineralisation15 and should be treated with high doses of calcium and vitamin D.

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