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Biomedical subjects

S Jansson

Publications and source records attributed to S Jansson.

88 records · Page 5Linked to original sources

Early diagnosis of and surgical strategy for adrenal medullary disease in MEN II gene carriers.

Sixteen multiple endocrine neoplasia type II (MEN II) gene carriers--12 who had undergone thyroidectomy because of medullary carcinoma of the thyroid and 4 whose thyroid glands had been removed because of C cell hyperplasia--were examined for the presence of pheochromocytomas. No patient had sought medical advice for pheochromocytoma symptoms. Fourteen patients had MEN IIa syndromes, one patient had a MEN IIb and another patient had a mixed syndrome of von Recklinghausen's neurofibromatosis and MEN II. Eight patients had undergone unilateral adrenalectomy for pheochromocytoma 11 +/- 4 years before. The patients underwent clinical examination, determination of the urinary excretion of catecholamines and metabolites, and 131I-metaiodobenzylguanidine (131I-MIBG) and CAT scans. 131I-MIBG scanning was performed with images 1, 4, and 7 days after the radionuclide injection. In seven of eight patients who had undergone unilateral adrenalectomies, the 131I-MIBG scans showed accumulation of the radionuclide in the remaining adrenal gland. Bilateral adrenal accumulation of the radionuclide was demonstrated in seven of eight MEN IIa gene carriers who had not undergone adrenalectomy. Five patients, two of whom had undergone adrenalectomy, were found to have unilateral pheochromocytomas less than 2 cm in diameter. Only one of these five patients had an elevated excretion of urinary catecholamines. Between day 4 and day 7 after 131I-MIBG injection, adrenal glands with pheochromocytomas increased their relative accumulation of the radionuclide significantly more (p less than 0.02) than did adrenal glands without any demonstrable pheochromocytomas. All the pheochromocytomas were viewed by means of CAT scans. Only one MEN IIa patient had bilateral pheochromocytomas, but our findings indicate that there is a tendency to bilateral adrenal medullary hyperfunction in most MEN II gene carriers. As 131I-MIBG and CAT scans can facilitate the early diagnosis of pheochromocytomas, unilateral adrenalectomy can safely be performed in most MEN IIa patients. Bilateral pheochromocytomas develop in a majority of patients with MEN IIb syndromes. Bilateral adrenalectomy should therefore be performed in these patients.

3-Iodobenzylguanidine↗

Methodology and dosimetry in adrenal medullary imaging with iodine-131 MIBG.

Iodine-131 MIBG scans were performed in 59 patients in order to localize intra- or extra-adrenal pheochromocytomas (pheos), or to visualize hyperplastic adrenal medulla. Images were obtained from the pelvis to the base of the skull on Days 1, 4, and 7 after tracer injection. The 15 patients with histopathologic confirmation of adrenal medullary disease had positive scans. In three of these, the pheos were visible only on images obtained on Day 7. One scan was false negative. After excluding patients with a predisposition to adrenal medullary disease, nine subjects (28%) without verification of pheo displayed adrenal uptake of the radionuclide. Late images produce a low rate of false-negative scans; the background activity diminishes and even small pheos can be detected. In order to increase the quality of late images, 40 MBq [131I]MIBG was used instead of 20 MBq. The dosimetric considerations are discussed.

3-Iodobenzylguanidine↗

Morphology of the adrenal medulla indicating multiple neuroectodermal abnormalities in pheochromocytoma patients.

25 of 85 (29.4%) consecutive patients operated on for pheochromocytoma had other neuroectodermal abnormalities. Medullary thyroid carcinoma was the most common associated neuroectodermal abnormality followed by von Recklinghausen's neurofibromatosis. Other abnormalities were intracranial tumors, parathyroid hyperplasia and midgut carcinoid. The adrenal medulla was studied to find out morphological characteristics in patients with associated neuroectodermal abnormalities. All patients with multiple pheochromocytomas (n = 7) and all patients with hyperplasia of the extratumoral adrenal medulla (n = 13) had other neuroectodermal abnormalities. It is important to detect the associated neuroectodermal abnormalities because they can be lethal. Patients with associated neuroectodermal abnormalities often have hereditary syndromes.

Adrenal Gland Neoplasms↗

Autotransplantation of diseased parathyroid glands into subcutaneous abdominal adipose tissue.

From October 1981 to the end of 1984, 13 patients with primary hyperparathyroidism (PHPT) and 17 with secondary hyperparathyroidism (SHPT) received fresh autografts of diseased parathyroid tissue into their subcutaneous abdominal adipose tissue. Because of previous surgery to treat hyperparathyroidism (HPT) (23%), concomitant thyroid surgery (26%), and a high proportion of multiglandular disease (73%), the patients were at high risk for HPT. During the follow-up period, hypercalcemia was diagnosed in five patients and successfully treated in four: by graft excision in two patients, by excision of a fourth gland from the neck in one patient, and with prednisolone in a patient with sarcoidosis. At follow-up (an average of 30 months after grafting), one patient had HPT and 29 others were euparathyroid. Parathyroid tissue can survive and function in adipose tissue, as was demonstrated by normocalcemia in 14 patients (whose only probable remaining parathyroid tissue had been transplanted into fat), by the demonstration that graft-dependent hypercalcemia could be eliminated by excision of the transplant, and by the demonstration of viable parathyroid tissue by microscopic examination of excised grafts. Autotransplantation of diseased parathyroid tissue into fat is simple and reliable. In cases of recurrent HPT, all or a portion of the graft can be removed while the patient is under local anaesthesia. If infiltrating growth occurs, broad excisions can be performed without sacrificing vital structures.

Abdomen↗

Reoperation in the treatment of asymptomatic metastasizing medullary thyroid carcinoma.

Eleven patients who continued to have elevated calcitonin (CT) levels after thyroidectomy and central node dissection for medullary carcinoma of the thyroid gland (MCT) were subjected to additional surgery. Metastatic MCT was found in the excised tissue from each patient. Normalization of CT values was obtained in four patients who had a few microscopic, unilateral metastases. In these patients an average of 13 normal lymph nodes were excised at reoperation for each metastasis. The results of the reoperative series demonstrate that it is possible, with meticulous neck dissection, to normalize CT levels in patients with microscopic metastases from MCT. Metastases that can be seen or palpated are always associated with microscopic lymph node metastases. Removal of such microscopic metastases is probably the key to achieving normal postoperative CT levels.

Adult↗

"The" pheochromocytoma: a benign, intra-adrenal, hypertensive, sporadic unilateral tumor. Does it exist?

This study aims to examine the frequency of the pheochromocytoma (pheo), defined as a "benign, intra-adrenal, hypertensive, sporadic, unilateral tumor." Three large series amounting to 310 subphrenic chromaffin tumors operated over periods of 17, 23, and 41 years, respectively, have been reviewed. Among those combined 310 pheos, 48 (15.5%) were malignant (i.e., metastatic) and 262 (84.5%) were benign; 42 (13.5%) were ectopic (35% of malignant tumors versus 9.5% of benign tumors); 230 (74.2%) were hypertensive (74% of benign tumors versus 73% of malignant tumors); 29 (9.4%) were bilateral, including 23 patients with a family history; 41 (13.2%) of patients had MEN II type A or B syndrome; and 20 (6.5%) occurred in a phacomatosis setting. Some of the patients had a non-MEN family history of pheo (n = 5), seemingly sporadic hyperparathyroidism (n = 4), or other associated neuroendocrine tumor (n = 9). Sometimes several of these features were combined. Finally 125 (40.3%) cases fitted the classic description of the tumor, 47.0% at the time of initial presentation and 40.3% at the end of follow-up. Late occurrence of metastases or metachronous diagnosis of familial disease make lifelong follow-up mandatory. Genetic studies may be indicated in pheochromocytoma patients.

Adrenal Gland Neoplasms↗

Parathyroid localization by catheterization of large cervical and mediastinal veins to determine serum concentrations of intact parathyroid hormone.

A success rate of about 90% has been achieved after primary operations for hyperparathyroidism, compared with 60% to 80% in most series of secondary operations. The present reoperative series involved 29 patients who underwent venous catheterization with blood sampling for the determination of intact parathyroid hormone before undergoing repeat parathyroid surgery. Blood samples were taken from the internal jugular veins, innominate veins, and superior caval vein. No attempt was made to perform superselective catheterization of the small neck and mediastinal veins. The reoperations were done by four surgeons who did 1, 2, 13, and 13 of the reoperations, respectively. In all patients, distinct step-ups in parathyroid hormone concentrations were found. On average, the gradient between the highest and lowest value was about 5. Close to the location of the step-ups, diseased parathyroid tissue was found in 27 of the patients. In two cases no parathyroid tissue was found, and these patients remained hyperparathyroid postoperatively. They had been treated by the surgeon who did only two of the operations. When the step-up was observed in the left innominate vein, we could not differentiate mediastinal from low cervical adenomas. No patient developed hypoparathyroidism. To avoid this complication, autotransplantation of diseased parathyroid tissue into the abdominal subcutaneous fat was done in nine patients. No case of recurrent laryngeal nerve paralysis occurred. The introduction of reliable assays for the analysis of parathyroid hormone can make selective catheterization unnecessary when localizing remaining parathyroid glands in patients with persistent hyperparathyroidism.

Adolescent↗

Management of hyperparathyroid patients with grave hypercalcemia.

During recent years the total number of patients undergoing surgery for hyperparathyroidism has markedly increased, but the annual number of cases with substantial hypercalcemia has remained unchanged. Parathyroid carcinoma and water clear cell hyperplasia cause more severe hypercalcemia than other kinds of hyperparathyroidism. Grave hypercalcemia due to hyperparathyroidism is more common among the elderly, but can occur during pregnancy and also among children. Occasionally, a patient with hyperparathyroidism can also have another cause of the hypercalcemia and does not become normocalcemic until adequately treated for both. The suspicion of grave hypercalcemia should arise due to its clinical features. Determination of serum calcium and intact parathyroid hormone concentrations establishes the diagnosis. The basic treatment of grave hypercalcemia is to rehydrate the patient and to restore the sodium losses. To further lower the serum calcium value we have found bisphosphonates to be very effective. The definitive treatment of grave hypercalcemia due to hyperparathyroidism is surgery. As a last resort, frail patients with grave hyperparathyroidism can undergo surgery under local anesthesia. Repeat operations can improve the prognosis of patients with metastatic parathyroid carcinoma. Selective venous catheterization with blood sampling for determination of intact parathyroid hormone can be helpful in localizing recurrent disease.

Acute Disease↗

Need for thyroxine in patients lobectomised for benign thyroid disease as assessed by follow-up on average fifteen years after surgery.

Thyroid lobectomy is a common procedure. It is therefore important to know whether lobectomised patients need thyroxine substitution after operation for benign disease. A follow-up examination of 95 patients on average 15 years after surgery disclosed hypothyroidism in 5%. There were seven individuals (9%) with only elevated thyrotropin values but normal thyroxine values or only borderline thyroxine values. The completeness of the lobectomy was checked by means of technetium scintigrams. Patients on thyroxine treatment were investigated twice, the second time after discontinuing thyroxine for at least six weeks. There were no goiter recurrences indicating reoperation. No patient who had been operated on for a follicular adenoma had signs of malignant conversion or follicular recurrence, regardless of thyroxine treatment or not. We conclude that routine prescription of thyroxine is not warranted for lobectomised patients. It seems preferable to offer these patients occasional follow-up for functional assessment and thyroid palpation.

Female↗