Search PubMedSearch

Biomedical subjects

R Abs

Publications and source records attributed to R Abs.

At least 37 records · Page 2Linked to original sources

[Surgery and curietherapy of keloids].

Postoperative irradiation of keloids allows a decrease of the recurrence rate by about 50%, compared to surgery alone. A review of the literature illustrates the benefits due to the irradiation, and describes the techniques available. The Iridium 192 interstitial brachytherapy, with per-operative implantation of the plastic tubes and immediate irradiation of 20 Gy at 5 mm depth, is detailed as used by French teams. The analysis of the published results allows to recommend this technique which is tailored to each clinical situation, safe, and easy to perform by the surgeon.

Brachytherapy

Association of isolated hypogonadotropic hypogonadism, pronounced hypodontia and the Wolff-Parkinson-White syndrome.

Diagnosis of idiopathic isolated hypogonadotropic hypogonadism was made in a 22-year-old female patient referred for primary amenorrhoea. It was considered a separate entity from Kallmann's syndrome, because it was not accompanied by anosmia or other specific pleiotropic features. On the other hand, the patient showed severe hypodontia and an intermittent Wolff-Parkinson-White syndrome. To our knowledge, this association has never been reported before. This unusual phenotype points to a nonrandom association. However, no information in the literature is available to consider a new single gene defect or a contiguous gene syndrome.

Adult

Variable regions of chromosome 11 loss in different pathological tissues of a patient with the multiple endocrine neoplasia type I syndrome.

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant inherited disorder characterized by nodular proliferation of the parathyroid glands and tumors of the anterior pituitary gland, the endocrine pancreas, and the neuroendocrine cell system of the gut. Loss of the putative tumor suppressor effect of the MEN1 gene is probably responsible for the development of MEN1-associated tumors. We report here a genetic study of a female MEN1 patient with the association of nodular hyperplasia of two parathyroid glands, an insulinoma, multiple duodenal gastrinomas, a prolactinoma, and a gastric carcinoid. We performed loss of heterozygosity (LOH) studies of chromosome 11 on all affected tissues except the insulinoma. Allelic losses of chromosome 11 were detected in several tumors, but the chromosomal regions of LOH were different, suggesting that different somatic mutational events are involved in the pathogenesis of these tumors. LOH of chromosome 11 was also detected in the prolactinoma of this patient, which indicates that the MEN1 gene has a tumor suppressor effect in the pituitary.

Calcium

Autonomously functioning thyroid nodules in a patient with a thyrotropin-secreting pituitary adenoma: possible cause--effect relationship.

A 51-year-old female patient with long-standing hyperthyroidism due to a thyrotropin-secreting pituitary adenoma is reported, who became thyrotoxic again shortly after successful pituitary surgery. Functional testing and scintigraphy suggested the diagnosis of autonomous functioning thyroid nodules, which was confirmed by pathological examination of the resected thyroid tissue. This is the first report revealing the transition from a pituitary-dependent to a thyroid-dependent hyperthyroidism. Autonomous functioning thyroid nodules are, however, considered an intrinsic thyroid defect. In similarity with other disorders, in which trophic hormones may induce an autonomous secretion by the target gland, this report opens the possibility that a humoral factor may play a role in the development of autonomous functioning thyroid nodules.

Adenoma

Magnetic resonance characterization of a long-standing pituitary abscess.

A 56-year-old male patient with panhypopituitarism is presented. Magnetic resonance (MR) examination of the pituitary region revealed a pituitary mass with markedly increased signal intensity on T1-weighted images and low signal intensity on T2-weighted images. At surgery, a large amount of purulent material was removed. At light microscopy, the tissue was demonstrated to be a pituitary abscess occurring in an otherwise normal pituitary gland. The high signal intensity on T1-weighted images does not correspond to the only MR description of a pituitary abscess given until now, reporting a mass isointense to white matter. These signal characteristics could emanate from the presence of small amounts of blood breakdown products in the proteinaceous content of the abscess. MR appearance of a pituitary abscess is possibly aspecific and could depend on the pathological composition of the lesion.

Aged

Benign intracranial hypertension as a cause of transient partial pituitary deficiency.

A male patient is reported with benign intracranial hypertension, who presented with three periods of partial pituitary deficiency, corresponding to episodes of headaches and papilledema. No radiological signs of the empty sella syndrome were observed. Since benign intracranial hypertension has been implicated in the development of the empty sella syndrome, it is postulated that the endocrine deficiency syndrome encountered in the empty sella syndrome can be provoked by chronic intracranial hypertension in itself and is not necessarily secondary to the anatomical changes occurring in the sellar region.

Adult

The association of meningioma and pituitary adenoma: report of seven cases and review of the literature.

Seven patients with the co-occurrence of meningioma and pituitary adenoma are reported. Diagnosis was based on histological examination and if not available on MR imaging. Extensive review of the literature reveals only 18 other cases with this association if meningiomas appearing after radiation therapy for pituitary tumors are excluded. As in isolated meningiomas, the female-to-male ratio is high. A preponderance of perisellar meningiomas is apparent. Another distinct characteristic of this combination is not revealed, since no specific typing of the meningioma and no specific endocrine syndrome is uncovered. Two patients presented with multiple meningiomas, of which one showed two histologically different tumor types. The presence of other tumors in association with pituitary adenomas and meningiomas in our series is striking and could suggest a genetic dysregulation leading to the development of these tumors.

Adenoma

Hyperfunctioning unilateral adrenal macronodule in three patients with Cushing's disease: hormonal and imaging characterization.

We aimed to investigate the dynamics of adrenocorticotropin (ACTH) and cortisol secretion in pituitary-dependent Cushing's syndrome with bilateral macronodular adrenal hyperplasia presenting as a single adrenal macronodule, and to determine the imaging characteristics of this syndrome. Three female patients were studied. Plasma ACTH and serum cortisol secretion were studied by determining their rhythmicity and pulsatility and their responses to the administration of ovine corticotropin-releasing factor, thyrotropin-releasing hormone, metyrapone, tetracosactrin, insulin and dexamethasone. Techniques used to localize the anatomical lesion were bilateral simultaneous inferior petrosal sinus sampling, magnetic resonance examination of the pituitary, computed tomography (CT) scanning and [75Se]cholesterol scintigraphy of the adrenal glands. Plasma ACTH and serum cortisol levels were measured using a commercial radioimmunoassay and an immunoradiometric assay. The ACTH and cortisol pulse number and amplitude were calculated using established computer software. In all three patients ACTH and cortisol secretory dynamics fulfilled the requirements for diagnosis of pituitary-dependent Cushing's syndrome. A close relationship between ACTH and cortisol pulses also favored a pituitary dependency. Study of the amplitude of cortisol pulses classified two patients in the group of hypopulsatile Cushing's disease. Adrenal CT scanning demonstrated the presence of a large single nodule. [75Se]Cholesterol scintigraphy showed bilateral radionuclide uptake, although mostly localized over the adrenal nodule. All patients underwent successful trans-sphenoidal hypophysectomy. Over a period of 1 year, a slow shrinkage of the adrenal nodule was observed in two patients, while no change in volume was observed in one patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Glands

The association of astrocytoma and pituitary adenoma in a patient with alcaptonuria.

A female patient with a juvenile pilocytic astrocytoma and a pituitary adenoma is described. The patient also has alcaptonuria, a rare inborn error of metabolism with autosomal recessive mode of inheritance. The association of these three disorders has never been reported previously. The possible existence of a common genetic factor in the development of both tumors and alcaptonuria is discussed.

Adenoma

Treatment of macroprolactinomas with the long-acting and repeatable form of bromocriptine: a report on 29 cases.

Twenty-nine patients with macroprolactinomas were treated by monthly intramuscular injections of the long-acting and repeatable form of bromocriptine (Parlodel-LAR) in doses ranging from 50-150 mg. They were divided into two groups: group I consisted of 22 patients who received Parlodel LAR before transsphenoidal adenomectomy; group II was composed of 7 patients with earlier neurosurgery and of 2 patients from group I not cured by transsphenoidal adenomectomy. Duration of therapy varied from 1-12 months, and a total of 104 injections was given. At nadir day, serum PRL levels were situated between less than 1% and 43% of pretreatment values. At day 28 after the first injection, serum PRL levels varied between less than 1% to 139% of initial values. No difference could be detected between the two groups regarding the percent of PRL inhibition. Long-term treatment with Parlodel-LAR resulted in a sustained inhibition of PRL secretion, except for 1 case. Resumption of menstrual cycles occurred in 4 out of 15 women and correction of hypogonadism in 4 out of 14 men. Amelioration of disturbed visual fields was recorded in 3 out of 8 patients. Diminution of the adenoma volume was radiologically documented in 14 out of 22 cases. Only few and mild side effects were recorded. One patient with partial adrenal deficiency suffered from a syncope, but this was prevented by hydrocortisone supplementation during the subsequent Parlodel-LAR administration. In conclusion, Parlodel-LAR proved effective in the treatment of macroprolactinomas, achieving rapid inhibition of PRL secretion, and in some patients amelioration of hypopituitarism, reduction in tumor size, and improvement in visual fields, and caused no serious side effects. It is a valuable preparation to surgery and can also be used in long-term medical therapy.

Adult

Aldosterone-secreting adrenal adenoma as part of multiple endocrine neoplasia type 1 (MEN1): loss of heterozygosity for polymorphic chromosome 11 deoxyribonucleic acid markers, including the MEN1 locus.

A 63-year-old female presented with the extremely rare occurrence of an aldosterone-secreting adrenocortical adenoma as part of the syndrome of multiple endocrine neoplasia type 1 (MEN1). Only two other MEN1 patients were reported in the literature with hyperaldosteronism. The patient's MEN1 syndrome consisted of the association of primary hyperparathyroidism due to parathyroid adenoma, a prolactinoma, and a toxic multinodular goiter. Elevated basal and meal-stimulated serum PP levels without demonstrable pancreatic tumor were also found. Genetic analysis of the aldosterone-secreting adenoma with DNA markers localized on chromosome 11 showed loss of heterozygosity in tumor DNA. Since the MEN1 syndrome is caused by loss of the tumor suppressor gene on chromosome 11 in the 11q13 region, it is probable that the same mechanism is associated with the formation of the adrenocortical adenoma.

Adenoma

Nephrogenic diabetes insipidus. An unusual presentation of recurrent rectal cancer.

The occurrence of diabetes insipidus (DI) in patients with systemic cancer is caused usually by tumor metastasis to the hypothalamus or posterior pituitary. A 43-year-old man with DI 8 months after radical surgery for a poorly differentiated adenocarcinoma of the distal rectum is reported. A therapeutic trial of intranasal desmopressin acetate did not correct the hyposthenuria, thus localizing the defect to the kidneys. A large tumor recurrence in the pelvis caused bilateral hydroureteronephrosis, resulting in nephrogenic DI. This report shows that not all cases of DI in cancer patients are of central origin.

Adenocarcinoma

Hyperfunctioning metastatic follicular thyroid carcinoma in Pendred's syndrome.

A 66-year-old woman with Pendred's syndrome underwent a partial thyroidectomy when she was 17 years old. At the age of 52 years, she had a second thyroid operation because of hyperthyroidism due to a toxic multinodular goiter with a mediastinal extension consisting of several separate nodules. Five years later a hyperfunctioning metastatic follicular carcinoma was diagnosed histologically. After treatment with radioactive iodine, the patient was well. To the authors' knowledge, this is the first description of a metastatic follicular thyroid carcinoma in Pendred's syndrome and the first report of hyperthyroidism occurring after malignant degeneration of a dyshormonogenetic goiter.

Adenocarcinoma

Acute effects of Parlodel-LAR and response to long-term treatment with bromocriptine in a patient with a follicle stimulating hormone-secreting pituitary adenoma.

A 68-year-old male patient presented with visual impairment due to a large pituitary tumor. After transsphenoidal adenomectomy the elevated serum FSH levels were lowered but not normalized. Deterioration of the vision was detected five years later and tumor regrowth was evidenced. The patient was treated with the long-acting and repeatable form of bromocriptine (Parlodel-LAR). Three days after the first intramuscular injection it already resulted in an important improvement of the visual field defects. Serum FSH concentration was suppressed during a prolonged period, but no change in the size of the pituitary adenoma was recorded on CT scan. Long-term oral treatment with bromocriptine resulted in a sustained suppression of the serum FSH levels, without further visual improvement, but with a significant reduction of the volume of the adenoma. The rapid and prolonged effect of Parlodel-LAR upon the FSH secretion, with a possible correction of the visual field defects and a reduction of the tumor mass, could make this medication appropriate as adjunctive treatment in some gonadotroph cell adenomas.

Adenoma

Changes in serum thyroid hormone autoantibody concentrations during pregnancy: a case report.

A 29-year-old female patient with Graves' disease who developed thyroid hormone autoantibodies (THAA) under treatment with methimazole is presented. THAA were identified as IgG-kappa. During a first pregnancy that ended by miscarriage in the 3rd month, the titer of anti-thyroxine autoantibodies decreased by about 30%. Relapse of Graves' disease occurred 2 months later and an increase in serum THAA concentration to the initial titer was observed. THAA titer remained unchanged during treatment with methimazole and afterwards during thyroxine supplementation for radioiodine-induced hypothyroidism. During a second pregnancy, a decrease in anti-thyroxine autoantibody titer reached 45% at the time of delivery and an increase by 20% was noted 5 months later. A similar decline in THAA concentration was shown during a third pregnancy. The changes in THAA concentrations observed during pregnancy suggest an immunological influence of pregnancy on the THAA production, as previously demonstrated in other autoimmune diseases, like Hashimoto's thyroiditis.

Adult