Clinical profile of 128 subjects operated for primary hyperparathyroidism

From 1960 to 1990, one hundred twenty eight (128) subjects with primary hyperparathyroidism were operated in the University Hospital. The medical records were reviewed. Serum and urine chemistries were done by conventional methods, serum PTH was done by RIA's (N-, C-, and midregion) and intact by IRMA and 1,25 dihydroxycholecalciferol by a non equilibrium receptor assay from calf thymus and preceded by double Sep-Pak chromatography. The distal third of the radius (nondominant arm) was used to evaluate radial bone density (RBD), using single photon absorptiometry (Norland) and the lumbar bone density (LBD) was measured by dual energy X Ray absorptiometry (DEXA). The RBD was done in 41 females and 15 males and the LBD in 12 females and 4 males. The series comprised 95 females, age range from 15 to 79 years, and 33 males, age range from 14 to 69 years. Prominent clinical features included nephrolithiasis in 72 subjects (56 per cent), osteitis fibrosa cystica in 2, isolated familial hyperparathyroidism in 4 subjects in one family, 7 subjects with MEN-1 in 3 families, and 4 subjects with MEN-2 in one family. Only 7 subjects were asymptomatic. Serum calcium was elevated in all, serum alkaline phosphatase was elevated in 24 per cent and urinary hydroxiproline was increased in 48 per cent. Serum phosphorus was low in 92 per cent. PTH assay was either elevated or inappropriately normal for the serum calcium in all patients tested. Serum 1,25 D was elevated in 57 per cent. The PTH level was positively correlated with the serum calcium (r = 0.70), but had no significant correlation with the serum phosphorus and the 1,25 D. The RBD expressed as the standard deviation from that of the mean for age and sex matched controls was > or = 2 SD below the mean in 39 per cent of females and in 40 per cent of males. In contrast to the RBD none of the subjects tested had a LBD > or = 2 SD below the age and sex adjusted mean. 103 subjects had adenomas, 20 primary hyperplasia, 2 carcinomas and in 3 surgical exploration was unsuccessful. As to the outcome of Surgery, 117 (93 per cent) were cured. Thus, in this series, successful surgery for primary hyperparathyroidism is the rule. Primary hyperparathroidism is rarely asymptomatic and appendicular bone disease and nephrolithiasis are commonly seen.

Unusual cases of hyperparathyroidism

The history, physical and radiologic findings, treatment and pathology in five unusual cases of hyperparathyroidism is presented. The hyperparathyroidism was caused by a large (113 grams) mediastinal adenoma in the first patient, who is alive 25 years after surgery. A parathyroid carcinoma with compression of the esophagus was documented in the second patient. This patient is alive and normocalcemic 23 years after surgical treatment. A third patient with hyperplasia returned with hypercalcemia 20 years postsurgery requiring reoperation. A fourth patient with advanced bone findings was found to have a parathyroid adenoma. The fifth case is a patient with tertiary hyperparathyroidism secondary to hypophosphatemic rickets

Pheochromocytoma: a twenty year experience at the University Hospital

Durante los últimos 20 años (1970-90), hemos tenido 24 pacientes con feocromocitomas: 19 diagnosticados clínicamente y 5 en autopsia. Sus edades variaron de 17 a 74 años (promedio de 43.2) y el número de varones (14), fueron mayor que el de las hembras (n=10). La mayoría de los sujetos tuvieron síntomas (95%) consistentes en la tríada típica de dolor de cabeza, palpitaciones y sudoración. El hallazgo más frecuente fue la hipertensión (95%), la cual fue sostenida en 60% y paroxística en 35% de los casos. Un 25% (n=6) de casos tenía tumores bilaterales, todos familiares. Quince eran tumores adrenales solitarios (63%) y 3 (12.5%) eran extra-adrenales: 2 en el abdomen (órgano de Zuckerbandl) y uno intratorácico (paraganglioma del atrio derecho del corazón). De los 6 casos familiares, 4 estaban asociados a la enfermedad de Von Hipper-Lindau (VHL), mientras que 2 tenían el síndrome de neoplasia múltiple tipo 2 (MEN-II. Todos los casos familiares eran bilaterales y se hallaban en las adrenales. No hubo tumores malignos. Entre los 19 casos diagnosticados pre-operatoriamente, fueron positivas las pruebas en orina del ácido vanililmandélico (VMA) en 95% y la de catecolaminas totales en 100%. Se logró visualizar los tumores pre-operatoriamente por medio de tomografía computadorizada (CT) o por imagen de resonancia magnética (MRI) en 62% de los pacientes más recientes. En 5 casos más antiguos sólo se hizo el diagnóstico post-mortem: 3 murieron de hemorragias cerebrales, 1 de infarto del pons y otro en fallo cardíaco congestivo. Hubo dos muertes post-operatorias y otra, 13 años post-operación, de carcinoma medular del tiroides. De los 19 operados 13 (68% fueron curados. Se concluye que los feocromocitomas aún retienen un considerable potencial de morbilidad y alguna mortalidad. Estos raros tumores constituyen un reto diagnóstico clínico, pero conllevan una gran satisfacción para el médico que los atiende

Localization of parathyroid lesions: blind study for surgeons

Success in the treatment of primary hyperparathyroidism rest in the accurate localization and removal of the diseased gland or glands. Computerized tomography and nuclear imaging scans are being used to localize abnormal parathyroid tissue. In the present study, fifteen consecutive patients undergoing surgery for primary hyperparathyroidism were all subjected to these ancillary studies. Results were not revealed to the operating team. In all instances an adenoma was localized during neck exploration. CT Scan failed to localize 73% of the affected glands. Nuclear scans missed almost fifty percent of the parathyroid adenomas. The low yield of these ancillary localizing tests makes them unnecessary in the routine evaluation of patients undergoing surgery for primary hyperparathyroidism

A syndrome of familial intrathyroidal primary parathyroid hyperplasia: case reports and critical review of literature

Intrathyroidal hyperplastic parathyroid glands were responsible for primary hyperparathyroidism (PHPT) in two of three members in a family. The third had an extrathyroidal parathyroidal "adenoma". Both intrathyroidal parathyroid (IThP) hyperplastic glands were the largest ones removed at the time of surgical cure. A review of the literature confirmed our postulate of a higher incidence of familial cases among patients with hyperparathyroidism and IThP with an incidence of 10.34% of IThP in familial cases versus a 4.2% in non-familial cases with PHPT. This contrasts with an incidence of 0.1% of IThP in normal patients. We hypothesize that stimulation of IThP tissue by surrounding calcitonin-producing C-cells might play a role in the seemingly preferential IThP hyperplasia. Recognition of this syndrome of Familial IthP Hyperplasia is important in order to avoid unnecessarily aggressive surgery for hyperparathyroidism