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Query: UMLS:C0221002 (primary hyperparathyroidism)
 4,921 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although primary hyperparathyroidism has rarely been described in pediatric patients, prompt diagnosis can avoid severe CNS and metabolic consequences. The aim of this paper is to report a 6 year-old girl whose first symptoms began at eight days of age with cyanosis, hypotonia, and upward gaze deviation. At 4 months, she was admitted due to neurologic disorders and recurrent infection, but the definite diagnosis was made only six years later. Her serum calcium levels are among the highest ever reported in the medical literature, reaching 25.5 mg/dl (6.36 mmol/l). Hypercalcemia, very high levels of parathormone (1550 ng/l--normal range 10-65) and bone deformities posed no problem to diagnosis when she first came to our attention. Nephrocalcinosis and impaired renal function were detected and this child had to be treated with diuretics (furosemide) and hydration that were able to lower her serum calcium levels. Imaging studies including 99mTc-sestamibi scan were not diagnostic. At surgery, the four parathyroid glands were mildly enlarged, with primary hyperplasia. The four glands were removed, cryopreserved, and 14 fragments (1 mm each) were autotransplanted to the braquioradial muscle of the left forearm. After a first phase of hypocalcemia (hungry-bone syndrome), treated with calcium and calcitriol, the calcium levels stabilized. The question is whether she will experience some degree of recovery from her neurological problems, since her severely high calcium levels have been maintained for such a long time.
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PMID:Primary hyperparathyroidism in children: patient report and review of the literature. 964 34

Neonatal primary hyperparathyroidism (NPHP) is a rare disease that presents in the first 6 months of life. It is almost invariably fatal unless a prompt diagnosis is made and urgent surgical intervention is instituted. Patients with hyperparathyroidism require special preoperative considerations. They require proper work-up from cardiac, renal and neurological point of view, correction of intravenous volume and electrolytes irregularities. We are presenting a case of an infant who started to have cyanosis and lethargy at 21st day of life. Initial diagnosis of pneumonia was made but later on diagnosed as hyperparathyroidism. He was planned for parathyroidectomy under general anaesthesia. He was induced with sevoflurane followed by fentanyl and atracurium and intubated with size 3.5 mm endotracheal tube (ETT). Later, he was maintained with isoflurane and O2/NO2. He was successfully extubated at the end of operation and was shifted to intensive care unit (ICU) for close monitoring.
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PMID:Anaesthetic management of neonatal primary hyperparathyroidism. 2386 56