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Biomedical subjects

L R Sheeler

Publications and source records attributed to L R Sheeler.

At least 19 recordsLinked to original sources

Effect of 131 iodine therapy on the course of Graves' ophthalmopathy: a quantitative analysis of extraocular muscle volumes using orbital magnetic resonance imaging.

There remains uncertainty as to the effect of radioactive iodine (131I) therapy on the associated ophthalmopathy (GO). Twenty newly diagnosed patients with Graves' hyperthyroidism treated with 131I (median dose, 15.5 mCi) were followed with ophthalmologic evaluations (OE) and magnetic resonance imaging (MRI) at baseline, 2, and 6 months, and with OE alone at 3 years. For MRI, the superior, inferior, and medial rectus muscle volumes and total muscle volumes (TMV) were measured. Replacement levothyroxine was initiated as low thyroxine (T4) levels were noted. At baseline, 10 patients (50%) showed evidence of mild GO by OE and/or MRI. There was a significant difference in TMV between the 20 patients with Graves' hyperthyroidism and 10 controls (mean +/- standard error [SE]; 2,652 +/- 118 vs. 2,046 +/- 96 mm3; P = 0.002) and between the 10 patients with and 10 without GO (3,006 +/- 96 vs. 2,298 +/- 61 mm3; P = 0.001). TMV correlated with the Hertel score (r = 0.56, P = 0.01). TMV showed no significant change at 2 or 6 months posttreatment. The inferior rectus volume increased slightly at 2 months posttreatment (P = 0.03) but remained stable at 6 months. Furthermore, no significant changes occurred in Hertel scores or in clinical assessments up to 3 years posttreatment and none showed worsening or new development of GO. In conclusion, our results show no significant risk for radioiodine-induced initiation or progression of mild GO.

Adult↗

Insulin antibodies and hypoglycemia in diabetic patients. Can a quantitative analysis of antibody binding predict the risk of hypoglycemia?

We report a noninsulin-dependent diabetes mellitus (NIDDM) patient with spontaneous, severe hypoglycemic reactions and the presence of insulin antibodies. He had a remote antecedent history of beef-pork insulin therapy as well as exposure to hydralazine. Detailed insulin binding kinetic studies were performed in this patient as well as in six other insulin-treated diabetic patients with anti-insulin antibodies (three with and three without an obvious cause of hypoglycemia). Sera from the current patient and five of the six other diabetic patients (one NIDDM, four IDDM) revealed two types of binding sites: high-affinity with low capacity (Kd, 0.4-12.4 x 10(-9) mol/L; binding capacity, 0.6-659 mU/L) and low-affinity with high capacity (Kd, 0.3 to 35.7 x 10(-7) mol/L; binding capacity; 202-113,680 mU/L). One NIDDM patient had only high-affinity antibodies (Kd, 22.9 x 10(-9) mol/L; binding capacity of 78 mU/L). Type of diabetes mellitus, insulin antibody titers or their binding capacities, insulin levels (total, bound, or free), and bioavailable insulin were not related to hypoglycemic reactions. Two calculated values by the method described tended to discriminate patients with and without hypoglycemia. The calculated amount of low-affinity antibody bound insulin ranged from 69.4-2090 mU/L vs < 4-70.6 mU/L in patients with and without hypoglycemia, respectively. The best discrimination was afford by the percent saturation of low-affinity binding sites; values were clearly higher in the patients with hypoglycemia (2.5-34.4%) than in those without hypoglycemia (not detectable, 0.06, 0.15%). Consideration of the possible drug-associated insulin antibody formation in insulin-treated diabetics and the novel quantitative analysis of the antibody binding kinetics should prove helpful in evaluating patients with high insulin antibody titers and assessing the risk of hypoglycemia.

Adult↗

Cushing's syndrome and pregnancy.

Women with active Cushing's syndrome rarely conceive. Only about 69 pregnancies have been reported in women with Cushing's syndrome, 60 of whom had maternal and fetal complications. Fourteen of the women have had therapy with unilateral adrenalectomy, bilateral adrenalectomy, pituitary surgery with selective adrenomectomy, or pituitary radiation.

Adenoma↗

Familial adrenocorticotropin-independent Cushing's syndrome with bilateral macronodular adrenal hyperplasia.

Familial Cushing's syndrome is rare, and when it occurs, it is usually associated with primary micronodular dysplasia. We report two cases, a mother and daughter, who each presented with clinical features of Cushing's syndrome at age 38 yr and were found to have ACTH-independent macronodular adrenal hyperplasia. In each case, bilateral adrenalectomy revealed the massively thickened adrenal cortex with nodules up to 1.3 cm in diameter and hyperplasia between nodules. Dynamic testing showed no suppression of free cortisol with high dose dexamethasone and no stimulation of 17-hydroxycorticosteroids with metyrapone. Two samples of serum obtained preoperatively from one patient that showed ACTH immunoreactivity of 4.6 and less than 2.2 pmol/L, respectively, each showed less than 2.2 pmol/L ACTH bioactivity. The lack of suppression with high dose dexamethasone, lack of stimulation with metyrapone, and low levels of ACTH immunoreactivity and bioactivity suggest that the bilateral hyperplasia was not dependent upon ACTH. These patients represent the first cases of ACTH-independent macronodular adrenal hyperplasia occurring in two generations of one family and illustrate the expanding clinical spectrum of Cushing's syndrome.

17-Hydroxycorticosteroids↗

Adrenal carcinosarcoma presenting in a woman with clinical signs of virilization. A case report with immunohistochemical and ultrastructural findings.

An adrenal carcinosarcoma is reported in a 29-year-old female presenting with clinical signs of virilization. This is the first reported case of a functioning adrenal carcinosarcoma in the English language literature. The tumor measured 12.5 cm in greatest dimension, weighed 610 g, and consisted of large areas of typical adrenal cortical carcinoma that was, however, interspersed with multiple foci of sarcoma. Rhabdomyosarcomatous elements were identified and confirmed both immunohistochemically and ultrastructurally. After radical resection, the patient received adjuvant mitotane therapy but developed rapid local and metastatic recurrence. Systemic chemotherapy was unsuccessful, and the patient died 8 months after surgery.

Adrenal Cortex Neoplasms↗

Pleural effusion resulting from metastatic papillary carcinoma of the thyroid.

We present a patient with a symptomatic pleural effusion resulting from papillary carcinoma of the thyroid metastatic to the pleura. Distinctive features include clinically evident pleural metastasis and the presence of psammoma bodies in the pleural fluid, which are characteristic (albeit not pathognomonic) of papillary carcinoma of the thyroid.

Carcinoma, Papillary↗

Recurrent Cushing's disease after transsphenoidal surgery.

Thirty-four patients with proven Cushing's disease underwent transsphenoidal surgery between January 1976 and August 1988. Postoperatively, all the patients had biochemically proven remission. Seven patients who had recurrence of their disease (incidence, 20.6%) were evaluated clinically and biochemically and the results are presented. All of seven patients had surgically and pathologically proven pituitary adenomas. All of seven patients had postoperative adrenal insufficiency necessitating steroid replacement for a mean of 8 months. The time of recurrence varied widely and ranged from 29 to 62 months, with a mean of 40 months. We conclude that (1) the longer the period of follow-up, the higher the incidence of recurrence; (2) although the recurrence rate is low (20.6%), it is certainly appreciable; and (3) the time of recurrence can be delayed (62 months) and thus we recommend yearly follow-up with urinary free cortisol determinations.

Adenoma↗

Spontaneous resolution of Cushing's syndrome after pregnancy.

Pregnancy is a rare occurrence in women with Cushing's syndrome. Even more uncommon is the exacerbation of Cushing's syndrome during pregnancy and spontaneous resolution post partum. We report one such case in which biochemical and radiologic findings suggested adrenocorticotropic hormone-dependent disease.

Adult↗

Cushing's syndrome and pregnancy.

Pregnancy is a rare occurrence in women with Cushing's syndrome. Amenorrhea or oligomenorrhea occurs in about 75% of premenopausal women with Cushing's syndrome as a result of suppression of gonadotrophin secretion primarily by excess glucocorticoids. We have reviewed pregnancies in women with Cushing's syndrome (63 cases from the literature and four cases of our own). Since pregnant women without Cushing's syndrome develop some features of Cushing's syndrome, such as hypertension, hyperglycemia, and striae, a high index of clinical suspicion must be maintained to prevent delay in diagnosis. The physiologic changes in adrenocorticosteroid metabolism during pregnancy further complicate the diagnosis. Maternal and fetal risks increase markedly when pregnancy does occur in women with hypercortisolism. However, the wide spectrum of severity of the disease mandates an individualized approach to the therapy in each case.

Adrenalectomy↗

Cushing's syndrome.

Cushing's syndrome remains one of the great diagnostic challenges to internists and endocrinologists. Urologists have the primary role in the treatment of many of the forms of Cushing's syndrome, especially adrenal tumors and micronodular hyperplasia. As we continue to gain experience with Cushing's disease treated with pituitary surgery and with etopic ACTH syndrome and the other forms of Cushing's syndrome, urologists are likely to become even more involved in the therapy of all types of Cushing's syndrome.

Adrenal Gland Neoplasms↗

The natural history of untreated microprolactinomas.

The development of radioimmunoassay (RIA) for prolactin (PRL) and sensitive high-resolution computed tomography (HRCT) has resulted in documentation of large numbers of microprolactinomas (microPRL). To try to define the rate and prevalence of tumor growth, the authors performed serial HRCT scans in 38 untreated patients, an average of 31.7 months apart. In no patient, including two patients with exuberant rises in PRL, was there any evidence of subtle tumor growth. The authors conclude that the majority of patients with microPRL have a benign course, with tumor growth occurring, if at all, over a period of years.

Female↗

Erdheim-Chester disease. Case report and review of the literature.

Erdheim-Chester disease is a distinctive pathologic and radiographic entity characterized by bilateral, symmetric sclerosis of the metaphyseal regions of long bones and infiltration of foamy, lipid-laden histiocytes. Clinically, it ranges from an asymptomatic, focal process to a fatal, systemic disease. All previously reported cases of Erdheim-Chester disease are reviewed and a new case with diabetes insipidus, partial hypopituitarism, histiocytic skin lesions, and retro-orbital tumors is presented. An 11-year follow-up reveals progression from a benign, limited process to a progressive, systemic disease. Patients with Erdheim-Chester disease show markedly disparate clinical courses and some features similar to Hand-Schüller-Christian disease. They need careful monitoring and further attempts at treatment.

Adult↗

Pituitary apoplexy: a complication of cardiac surgery.

Pituitary apoplexy occurred in 3 patients in the immediate postoperative period following cardiac operation with cardiopulmonary bypass. In this setting, this complication is extremely rare and not widely recognized. Precipitating factors may be related to the extracorporeal bypass apparatus, anticoagulation, low cerebral blood flow, and even anesthetic agents. Neurosurgical decompression can be safely performed in the early postoperative period following open-heart operations.

Cardiopulmonary Bypass↗

Clinical significance of the association of the Von Hippel-Lindau disease with pheochromocytoma and pancreatic apudoma.

Von Hippel-Lindau disease (VHLD) is a rare disorder known to occur with pheochromocytoma. We report a case with this association and also with pancreatic apudoma. We believe that this is the fifth reported case of this triad, which represents an overlap in the multiple endocrine neoplasia (MEN) classification. Many recent reports of overlap of MEN syndromes challenge the validity of this classification. New understanding of the embryologic origin of cells of endocrine organs has led to the elucidation of the APUD (amine precursor uptake and decarboxylation)-neuroendocrine system. Integration of the APUD system may be important to understanding the pathogenesis of the MEN-like syndromes as well as their relationship to the neurocutaneous syndromes. Any endocrine tumor or neurocutaneous syndrome should be evaluated to exclude a spectrum of possible associations.

Adult↗

Adrenal insufficiency secondary to carcinoma metastatic to the adrenal gland.

Four patients are reported in whom adrenocortical insufficiency developed consequent to carcinoma metastatic to the adrenal glands. Recognition and treatment of this entity may lead to palliation of symptoms and prolongation of useful life. The computerized axial tomographic scanner is a valuable diagnostic aid in diagnosing adrenal enlargement.

Adrenal Cortex↗