1/90. The elevated serum alkaline phosphatase--the chase that led to two endocrinopathies and one possible unifying diagnosis.A 39-year-old Chinese man with hypertension being evaluated for elevated serum alkaline phosphatase (SAP) levels was found to have an incidental right adrenal mass. The radiological features were characteristic of a large adrenal myelolipoma. This mass was resected and the diagnosis confirmed pathologically. His blood pressure normalised after removal of the myelolipoma, suggesting that the frequently observed association between myelolipomas and hypertension may not be entirely coincidental. Persistent elevation of the SAP levels and the discovery of hypercalcaemia after surgery led to further investigations which confirmed primary hyperparathyroidism due to a parathyroid adenoma. The patient's serum biochemistry normalised after removal of the adenoma. The association of adrenal myelolipoma with primary hyperparathyroidism has been reported in the literature only once previously. Although unconfirmed by genetic studies this association may possibly represent an unusual variation of the multiple endocrine neoplasia type 1 syndrome.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/90. Unilateral adrenal medullary hyperplasia: another form of curable hypertension?A case of unilateral adrenal medullary hyperplasia is presented in a 49-year-old caucasian female without multiple endocrine neoplasia association. The patient presented with episodic hypertension and paroxysms suggesting an underlying phaeochromocytoma. Biochemical supported this diagnosis but no discrete tumour was found on preoperative localising studies or at the time of surgery. The patient underwent a unilateral adrenalectomy with confirming adrenal medullary hyperplasia with complete resolution of her symptoms for six months.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
3/90. Approach to a patient with suspected primary aldosteronism.A typical case of a patient with suspected mineralocorticoid hypertension is discussed. The presentation follows sequential steps through the diagnostic evaluation and treatment of primary aldosteronism. The diagnostic approach can be classified into three phases: screening tests, confirmatory tests, and subtype differentiation studies. It is essential to follow the evaluation steps to elucidate the cause of primary aldosteronism, as this determines the appropriate treatment. Another lesson to learn is the need to critically appraise the value of any endocrine test, particularly if it is likely to influence the management strategy in an individual patient.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
4/90. Bone amyloidoma in a diabetic patient with morbid obesity.Bone localisations of amyloidosis are rare, usually diffuse and associated with myeloma. We report the case of a patient with massive obesity complicated by diabetes, hypertension, sleep apnea and liver steatosis, who complained of rapidly worsening bilateral polyradiculalgia of the lower limbs. After sufficient weight loss made nuclear magnetic resonance imaging feasible, a spinal tumour was visualised on the 5th lumbar vertebra, extending to soft tissues. Total excision was performed, and pathological studies revealed an amyloid bone tumour with no evidence of myeloma.- - - - - - - - - - ranking = 0.0080738200281778keywords = bone (Clic here for more details about this article) |
5/90. prognosis of children with malignant pheochromocytoma. Report of 2 cases and review of the literature.Malignant pheochromocytomas are rare in childhood and the prognosis of children with this tumor is not well known. We present 2 pediatric observations of malignant pelvic pheochromocytoma. Symptoms in both cases were headache and hypertension. The tumor invaded the sacral bone. Angiogram helped to localize the tumor and metastases, and allowed preoperative embolization of the tumor in 1 case. The first child underwent incomplete surgical resection, (131)I-MIBG therapy and radiotherapy, and is still alive 2 years after diagnosis. The second child died from metastatic invasion a few weeks after discovery of the tumor. We reviewed previous reports of children with malignant pheochromocytomas (30 cases). Primary tumor was extraadrenal in 50% of cases. The 3-year survival rate was 73 /- 9% (mean /- SD). Apart from surgical resection, no particular treatment appeared to be more effective than others in reducing mortality.- - - - - - - - - - ranking = 0.0080738200281778keywords = bone (Clic here for more details about this article) |
6/90. multiple endocrine neoplasia type 2a. Study of a family.INTRODUCTION: Pheochromocytomas (Pheo) can occur sporadically, isolated or in association with other neuroendocrine lesions. In multiple endocrine neoplasia type 2a (MEN-2A), Pheo is associated to medullary thyroid carcinoma (MTC) or its precursor, C-cell hyperplasia (CCH) and parathyroid hyperplasia. Genetic screening provides early diagnosis and preventive treatment. In order to validate dna analysis as a reliable method of early identification of gene carriers, we compared the results of genetic screening with clinical, biochemical, imaging and pathological findings in the members of an affected family. population AND methods: The diagnosis of a bilateral necrotic Pheo in a female patient led to the study of a family with four generations, aged 3 to 78 years (mean = 30.3 yrs). The study included a clinical examination; basal and pentagastrin stimulated calcitonin values; urinary catecholamines and their metabolites; serum calcium and a genetic study (direct sequence of PCR products from genomic dna isolated from leucocytes using specific primers in exon 11 of the RET protooncogene of chromosome 10). The radiologic study, gammagraphic study (131I-MIBG) and magnetic resonance study were performed in members with clinical suspicion of Pheo. RESULTS: Seven out of nine patients had a mutation on codon 634 of exon 11 of RET (TGC-CGC), leading to cysteine arginine substitution in the codified protein; all gene carriers had biochemical markers of MTC/CCH and four of Pheo. The Pheo patients underwent adrenalectomy (bilateral in three) and all the gene carriers underwent prophylactic thyroidectomy. The pathologic findings were: MTC in four (metastasized in one); CCH in three and parathyroid hyperplasia in one. CONCLUSIONS: Phenotypic penetration of RET mutation was 100% for MTC/CCH, but only 57% of the gene carriers had Pheo. Genetic screening allowed early prophylactic treatment in four out of seven patients; pathologic findings revealed several evolutionary stages of the disease. patients not yet showing Pheo are under close clinical and laboratory surveillance.- - - - - - - - - - ranking = 6keywords = endocrine (Clic here for more details about this article) |
7/90. hypertension in the young adult--come feel the pulse.hypertension occurring in teenagers and young adults is uncommon. Though the most common form is still essential hypertension, secondary causes are more commonly found here than in older adults. Renal, cardiovascular and endocrine diseases constitute most of these causes. Coarctation of the aorta is the most common cardiovascular cause of hypertension, and its importance lies in the fact that it is correctable, and that its persistence often leads to dangerous complications and early death. The cardinal sign of differential pulse and blood pressures between the upper and lower limbs can be detected clinically. Hence, the importance of a detailed physical examination in all young hypertensives, including palpation of all the pulses, cannot be overemphasized. We present 2 hypertensive young men who were found to have isolated coarctation of the aorta.The lesion in the first patient was located postductally just distal to the left subclavian artery.This area has been found to be the most common site of coarctation.The second patient had an unusual mid-thoracic coarctation. The clinical and radiological features as well as complications are highlighted. In young hypertensive patients, a high index of suspicion may enable the physician to make a timely diagnosis and hence avert the potentially disastrous complications that may arise in undetected cases.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
8/90. Poorly controlled hypertension in a painter with chronic lead toxicity.In 1984, a 56-year-old house painter developed intractable pain in his back and other joints. After several unrevealing medical work-ups, he was found to have a high blood lead level (122 microg/dL); he has a history of scraping and sanding lead paint without adequate protective measures. The patient was hospitalized and chelated with EDTA four times over the next 5 years; each time he felt better at the end of his treatment, but he returned to largely the same working conditions. He developed hypertension in April 1989, underwent a final chelation, and retired. He was subsequently followed on a regular basis with repeated measurement of lead levels in blood and bone (using a K-x-ray fluorescence instrument) as well as clinical parameters. In 1995 his blood pressure became difficult to control despite a sequential increase in his antihypertensive medication dosages and the addition of new medications. In 1997 he began calcium supplementation and a high-calcium diet; his blood pressure declined markedly, allowing him to taper off of two of his four antihypertensive medications. This case demonstrates an occupational activity (construction) that has now become the dominant source of lead exposure for U.S. adults, the importance of a good occupational history to suspecting and making a diagnosis, the possible outcomes of chronic lead toxicity, and the importance of preventing further exposure and using proper methods to treat acute toxicity. It also highlights a current major etiologic question, that is, whether and to what degree lead exposure contributes to the development of hypertension, and raises the issue of whether lead-induced hypertension constitutes a subset of hypertension that is especially amenable to therapy with dietary calcium.- - - - - - - - - - ranking = 0.0080738200281778keywords = bone (Clic here for more details about this article) |
9/90. Cardiac phaeochromocytoma presenting with severe hypertension and chest pain.Cardiac phaeochromocytoma is a rare cause of endocrine hypertension. We report a case of a 25-year-old woman, who presented with severe hypertension and intermittent chest pain. The patient denied typical phaeochromocytoma spells of palpitation, headache, and diaphoresis. The 24-hr urinary excretion of norepinephrine was increased sevenfold above the upper limit of normal; however, the excretion of total metanephrines, epinephrine, and dopamine were normal. Computed tomography (CT) scan of the abdomen was normal. An 131I-labelled metaiodobenzylguanidine (MIBG) scan was falsely negative while the patient was taking labetalol. The cardiac phaeochromocytoma was localized with indium-111-pentetreotide scintigraphy and chest magnetic resonance imaging scan. Repeat 123I-MIBG scintigraphy was positive after discontinuing labetalol. The cardiac phaeochromocytoma was located in the right atrial groove, adjacent to the tricuspid valve, and contained multiple feeder arteries from the right coronary artery. After treatment with volume expansion, alpha-methyl-p-tyrosine, and alpha- and beta-adrenergic blockade, surgical resection was performed. While under cardiopulmonary bypass, coronary bypass grafting and tricuspid annuloplasty were performed to facilitate the complete surgical resection of the 4.5-cm tumour. The surgical course was uncomplicated, with complete cure of hypertension and normalization of catecholamine excretion. Post-operative cardiac function, as measured by echocardiogram, was normal. Although cardiac phaeochromocytoma may be highly vascular, invasive and difficult to resect, it can be cured.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
10/90. Immunotactoid glomerulopathy with massive bone marrow deposits in a patient with IgM kappa monoclonal gammopathy and hypocomplementemia.A case of immunotactoid glomerulopathy with an amyloid-like material in the glomeruli and bone marrow is described. Clinically the patient was diagnosed as having severe nephrotic syndrome, hypocomplementemia, and IgM kappa monoclonal gammopathy. Immunotactoid glomerulopathy is an unusual cause of glomerulonephritis, characterized by congo red-negative, amyloid-like deposits in the glomeruli. This unusual case presentation shows that immunotactoid glomerulopathy may be a manifestation of systemic disease. This patient also presented with hypocomplementemia, an extremely rare associated finding that has been reported previously in only four cases of immunotactoid glomerulopathy.- - - - - - - - - - ranking = 0.040369100140889keywords = bone (Clic here for more details about this article) |
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