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M Telenius-Berg

Publications and source records attributed to M Telenius-Berg.

At least 19 recordsLinked to original sources
PubMed1990

Incidence of sporadic and familial medullary thyroid carcinoma in Sweden 1959 through 1981. A nationwide study in 126 patients. Swedish MCT Study Group.

Medullary thyroid carcinoma (MTC) was identified in 276 patients (in 27 diagnosed at autopsy) by a review of virtually all 6,513 notifications of primary thyroid cancer ot the National Cancer Registry in Sweden 1959 through 1981. The diagnosis was confirmed in 268 of the 276 cases by histopathological and histochemical reexamination. Anamnestic data and morphological characteristics indicated that 208 (75%) patients had sporadic and 68 (25%) familial MTC. The mean ages at diagnosis of these two groups were 57.0 and 42.6 years respectively. The age-standardized incidence rate per 10(5) inhabitants was 0.18 for males and 0.23 for females. The age-specific incidence of sporadic MTC increased markedly with age, whereas no unequivocal rise was found after the age of 20 for familial disease. Standardized morbidity ratios (SMR), calculated separately for each of the six Swedish health care regions, revealed a roughly two-fold and mostly non-significant geographical variation in the occurrence of sporadic MTC. SMR for familial disease varied, however, between 0 and 306 and deviated highly significantly from the national average in four of the six regions. Regional differences in diagnostic intensity were considered unlikely as the sole explanation of this finding.

Adult↗
PubMed1989

Clinical characteristics in sporadic and familial medullary thyroid carcinoma. A nationwide study of 249 patients in Sweden from 1959 through 1981.

All patients with medullary thyroid carcinoma (MTC) diagnosed in Sweden during 1959 through 1981 were recruited for study from the Swedish Cancer Registry. Among a total of 249 patients, 66 were diagnosed in 1959 through 1969 and 183 from 1970 through 1981. Apparently sporadic MTC was present in 186 patients, and familial MTC in 63. Twenty-seven patients with familial MTC were diagnosed from clinical symptoms and 36 by screening. In both the sporadic group and the symptomatic familial group, approximately 80% of the patients had palpable thyroid tumors. Lymph node metastases were present in 44% of the sporadic group and in 37% of the group with symptomatic familial MTC, and distant metastases in approximately 20% of the patients in each of these groups. The patients detected by screening differed significantly from the two groups of symptomatic cases by having a lower frequency of palpable thyroid lesions (50%), smaller tumors, and lower frequencies of cervical lymph node metastases (14%) and distant metastases (0%). Multivariate analyses revealed no significant differences between sporadic cases and symptomatic familial cases regarding tumor size or stage. Large tumors (greater than 3 cm) were more frequently accompanied by palpable cervical lymph nodes and were associated with an approximately four times higher risk of distant metastases than tumors smaller than 1 cm. Women had significantly smaller tumors and a more favorable stage distribution than age-related men.

Adult↗
PubMed1989

The clinical and screening age-at-onset distribution for the MEN-2 syndrome.

The decision to screen for multiple endocrine neoplasia type 2 (MEN-2) is generally based on family history, the rationale for this approach being the presumed 100% penetrance of the disease. To determine the validity of this presumption we have estimated--by applying modifications of the life-table method--the clinical and screening age-at-diagnosis distributions for MEN-2, using families from the Cancer Research Campaign Medullary Thyroid Cancer Register and one large American family. The clinical penetrance of MEN-2 is shown to be incomplete, an estimated 41% of gene carriers not presenting with symptoms by age 70 on the basis of clinical history. Screening by the standard tests for detecting the earliest manifestations of the syndrome increases the penetrance to an estimated 93% by age 31. There is no evidence of a difference in the age-at-diagnosis distributions between maternal and paternal transmission, or among different families, but there is some suggestion of an earlier onset of medullary thyroid cancer in female gene carriers, and of a tendency of pheochromocytoma to cluster in families. These results can be used to calculate risks to relatives of affected individuals, which in turn can be used to guide decisions on which individuals to screen.

Adrenal Gland Neoplasms↗
PubMed1989

Quality of life after bilateral adrenalectomy in MEN 2.

Pheochromocytoma is a major cause of morbidity and mortality in the multiple endocrine neoplasia type 2 (MEN 2) syndrome. For the physician, surgical treatment seems well justified even though bilateral adrenalectomy will induce iatrogenically complete loss of adrenocortical function. For the patient this treatment may be a cause of medical problems as well as worry. We have evaluated quality of life after bilateral adrenalectomy in 27 MEN 2 patients through a combined oral and written approach. Mortality was low (one of 27), as was serious morbidity. Most patients had adapted well to the postadrenalectomy state. However, fatigue, worry, and noncompliance with daily medication often caused problems.

Adaptation, Psychological↗
PubMed1988

Risk estimation and screening in families of patients with medullary thyroid carcinoma.

Many gene carriers for multiple endocrine neoplasia type 2a (MEN 2a) do not manifest the disease, even into old age. Thus, a negative family history in a patient presenting with medullary thyroid carcinoma does not reliably exclude familial disease. Data are reported for the probability that the MEN 2a gene will either have manifested clinically or be detectable by stimulated calcitonin screening by a given age. These probabilities can be used to calculate individual risks and to guide screening.

Adolescent↗
PubMed1987

Exclusion of linkage of loci on chromosome 19 with multiple endocrine neoplasia, type 2.

Linkage between seven loci on chromosome 19 and multiple endocrine neoplasia type 2a (MEN2A) was examined in a single large Swedish pedigree. A total of 50 cM was excluded from the male genetic map by pairwise analysis and an estimated 63 cM by multipoint analysis. Using existing data on the likelihood of different marker-marker distances and taking into account current exclusions on other chromosomes, the probability that the gene for MEN2A segregating in this pedigree could still be located on chromosome 19 is approximately 0.28%.

Chromosome Mapping↗
PubMed1987

Catecholamine release after physical exercise. A new provocative test for early diagnosis of pheochromocytoma in multiple endocrine neoplasia type 2.

A simple and practical provocative test is needed for early asymptomatic pheochromocytoma, which is a major risk for patients with multiple endocrine neoplasia type 2 (MEN-2). We measured plasma catecholamines before and after submaximal exercise in 26 MEN-2 gene carriers, eight of whom with asymptomatic pheochromocytoma, nine with medullary thyroid carcinoma and 10 after uni- or bilateral adrenalectomy. Seventeen clinically healthy individuals and 11 patients with neurovegetative lability and symptoms mimicking pheochromocytoma served as controls. Plasma adrenaline, noradrenaline and dopamine increased after exercise except for adrenaline after bilateral adrenalectomy. The post-exercise levels of adrenaline and the adrenaline/dopamine ratio were significantly higher in the pheochromocytoma patients compared to the healthy controls and the patients with neurovegetative lability, while the patients with medullary thyroid carcinoma represented an intermediate group with a high probability of developing adrenal tumors. The present method is a physiological test with a high sensitivity and specificity. It is practical and well suited for repeated examinations and seems to be of value for the detection of early pheochromocytoma in MEN-2 patients. Furthermore, the test could be used in the differential diagnosis between pheochromocytoma and neurovegetative lability.

Adrenal Gland Neoplasms↗
PubMed1986

Immunoextracted calcitonin in milk and plasma from totally thyroidectomized women. Evidence of monomeric calcitonin in plasma during pregnancy and lactation.

The level of immunoreactive calcitonin in the first produced breast milk was in totally thyroidectomized (TX) women 713 +/- 307 pg-eq/ml (mean +/- SD, N = 7) and in control women 772 +/- 329 pg-eq/ml (N = 33), i.e. about 45 times higher than in plasma (see below). On gel chromatography of immunoextracted milk from TX women, immunoreactive calcitonin appeared as high molecular weight forms in the same pattern as in milk from healthy women when the same antiserum was used for immunoextraction and radioimmunoassay (RIA). In another series of experiments, a new antiserum raised in rabbits was used for measurement of immunoreactive calcitonin after immunoextraction with 1. Plasma levels of immunoreactive calcitonin in the TX women during pregnancy were 16 +/- 6 pg-eq/ml (N = 6) and during lactation 14 +/- 7 pg-eq/ml (N = 5). Immunoreactive calcitonin was undetectable (less than 8 pg/ml) in plasma from those TX women in whom lactation had stopped (N = 5). Immunoextraction and gel chromatography of plasma collected during pregnancy and lactation from the TX women showed that the immunoreactive calcitonin present eluted in the region of monomeric calcitonin with both antiserum 1 and 2. In conclusion, high concentrations of high molecular weight forms of of immunoreactive calcitonin have been demonstrated in milk from TX patients, most probably devoid of any calcitonin-producing thyroid C-cells. This points to a local production site in the mammary gland.(ABSTRACT TRUNCATED AT 250 WORDS)

Calcitonin↗
PubMed1977

Screening for medullary carcinoma of the thyroid in families with Sipple's syndrome: evaluation of new stimulation tests.

In search of new practical diagnostic methods for the early diagnosis of hereditary medullary carcinoma of the thyroid (MCT) calcitonin release has been studied following induction by pentagastrin, cholecystokinin-pancreozymin (the C-terminal octapeptide, C8-CCK, and the native swine extract), and ethanol in eighteen cases of MCT (all but one clinically occult), three 'borderline cases', seven first degree relatives of patients with hereditary MCT and thirty-five healthy controls. Pentagastrin, subcutaneous (s.c.) or intravenous (i.v.), induced a pronounced and rapid increase of serum calcitonin within 2-5 min. The elevation was roughly proportional to the tumour mass as estimated at operation. Seventeen out of eighteen MCT patients responded to s.c. pentagastrin with a significant increase in serum calcitonin and the response correlated well with that induced by calcium infusion test. Only two blood samples, at times 0 and 5 min, were necessary for diagnosis. In the MCT patients, i.v. pentagastrin produced more pronounced elevations of serum calcitonin than did s.c. pentagastrin, whereas no increase was seen in the control group. The subjective discomfort caused by i.v. pentagastrin was somewhat more intense but lasted shorter than that induced by s.c. administration. No serious complications were seen. All of nine MCT patients responded to C8-CCK with increments in serum calcitonin exceeding those of the control group and both of two responded similarly to the native cholecystokinin-pancreozymin extract. Generally the serum calcitonin response was lower and more variable after C8-CCK than after s.c. or i.v. pentagastrin, and the subjective discomfort was also more pronounced with abdominal cramps during the injection. Ethanol in the dose used was the least effective stimulator for serum calcitonin release. Clinically suspected MCT carriers with palpable tumours can be diagnosed by determination of the basal, i.e. non-stimulated serum calcitonin levels. Other possible Sipple genome carriers, who are at the time clinically healthy with normal basal serum calcitonin, should be subjected to a s.c. or i.v. pentagastrin stimulation test at each examination. These tests are much simpler to perform than a calcium infusion, test, but seem to have about the same sensitivity.

Adolescent↗
PubMed1975

Serum calcitonin response to induced hypercalcemia.

The rise in serum calcitonin (delta-CT240 min) has been measured during hypercalcemia induced by i.v. infusion of calcium gluconate. This calcium infusion test was used in a prospective screening for medullary carcinoma of the thyroid (MCT) in 4 families with Sipple's syndrome as well as in 3 sporadic cases of MCT. In 16 normal controls delta-CT240 was minus 0.2-+ 0.5 ng/ml (mean plus or minus 2 S.D.). Delta-CT240 was normal in 2 patients with chronic hypocalcemia. In all 14 MCT patients delta-CT240 was markedly higher (min-max 2.2-630 ng/ml), i.e. no false negatives were found. However, in these cases, the diagnosis was already evident from basal serum calcitonin (S-CT), which up to now has been our most sensitive diagnostic technique for MCT. 19 first-degree relatives of patients with Sipple's syndrome presented no signs of MCT. In 14 of these delta-CT240 was normal ("healthy relatives"), but in 5 it was slightly elevated, intermediate between the controls and the MCT patients. These 5 borderline cases were more sharply delineated from normal by delta-CT240 than by S-CT. Thus our calcium infusion test seems to be the most sensitive method for early diagnosis of occult MCT. We recommend the calcium infusion test for: (a) screening for MCT in all Sipple relatives with normal or only slightly elevated basal S-CT, (b) postoperative control in both sporadic and hereditary MCT, (c) investigation of supposed non-MCT tumours with calcitonin production.

Adolescent↗