1/116. Tubular stenosis of the aorta with aortic fibromuscular dysplasia.The autopsy findings in a 2-year-old girl with severe hypertension resulting from stenosis of the lower part of the thoracic and the abdominal aorta were reviewed. The lower portion of the thoracic aorta and the abdominal aorta showed uniform narrowing down to the level of the bifurcation of iliac arteries. Histologically, the aortic wall of the stenotic site showed irregular proliferation of smooth muscle cells and collagen fibers. The elastic fibers had disappeared from outside the media. No intimal thickening and inflammatory cell infiltration were observed. These histologic changes of the aortic media in this case are apparently similar to fibromuscular dysplasia (medial hyperplasia).- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/116. Unilateral macronodular adrenal hyperplasia as an unusual cause of Cushing's syndrome--a case study.To appraise clinicians of the problems that may be encountered in the diagnosis and management of Cushing's syndrome, we present a case report of a 20-year old female, who was admitted with a recently developed central obesity, ammenorrhea hirsuitism, proximal myopathy and depression. She was found to have multiple striae, thin skin, elevated blood pressure glycosuria and hyperglycaemia. Morning and mid-night plasma cortisol concentrations revealed elevated levels, with a loss of diurnal variation. There was a failure of the normal suppressibility of cortisol secretion by low doses of dexamethasone, while a significant suppression of plasma cortisol concentration was observed with high doses of dexamethasone. There were no significant abnormalities observed in the pituitary fossa on skull radiograph and on the cranial computerised tomographic scan. After a period of stabilisation, she had a bilateral adrenalectomy done, with a histopathological finding of a left adrenal macronodular hyperplasia, while the right adrenal gland was small and friable. There was an uneventful post-operative period, with a gradual return to normality of most of the presenting complaints. The hyperglycaemia and hypertension got controlled without medications, while her menstrual cycles resumed within three months of bilateral adrenalectomy. This case report illustrates that an adrenal-dependent Cushing's syndrome may mimic a pituitary-dependent one, especially as regards the suppressibility of plasma cortisol secretion by high doses of dexamethasone.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
3/116. Physiologic tailoring of therapy for resistant hypertension: 20 years' experience with stimulated renin profiling.renin profiling to assist in management of hypertension was first proposed about 27 years ago. However, it is still not widely used, perhaps because it was initially emphasized for management of primary hypertension, which is usually relatively easy to manage. Our experience in more than 4000 patients with referral hypertension screened since 1977 suggests that this approach, which I call "Physiologic Tailoring" of management, is particularly helpful in resistant hypertension, especially in cases of adrenocortical hypertension. Three cases of resistant hypertension spanning the 20 years of the clinic's existence, are presented to illustrate the contribution of renin profiling to the diagnosis and management of adrenocortical hypertension. It is suggested that adrenocortical hypertension is virtually always due to bilateral hyperplasia and that true curable solitary nodules may not exist if patients are followed long enough; and that adrenocortical hyperplasia is much more common in blacks and patients of African origin. A study to test the cost-utility of a renin-based algorithm for management of resistant hypertension is recommended.- - - - - - - - - - ranking = 13keywords = hypertension (Clic here for more details about this article) |
4/116. Nodular regenerative hyperplasia of the liver: case report of a 13-year-old girl and review of the literature.BACKGROUND: Nodular regenerative hyperplasia (NRH) of the liver is a multi-acinar regenerative nodular lesion in a non-cirrhotic liver. It is a rare entity, especially in children, and remains of unknown aetiology. OBJECTIVE: NRH is often seen in association with other diseases or drug intake. In half of patients it is complicated by portal hypertension. Radiologically, its nodular appearance may look like neoplasia. RESULTS: We report a case of NRH with enormous hepatomegaly and multiple huge nodules. CONCLUSION: We wish to emphasise the importance of open wedge biopsy to establish diagnosis, since the prognosis of NRH in the absence of portal hypertension is good. Complications such as rupture of a nodule are rare.- - - - - - - - - - ranking = 2keywords = hypertension (Clic here for more details about this article) |
5/116. Subclinical cushing syndrome due to adrenocorticotropic hormone-independent macronodular adrenocortical hyperplasia: changes in plasma cortisol levels during long-term follow-up.A 63-year-old man with bilateral adrenal tumors was treated for hypertension and diabetes mellitus. Endocrinologic examination during follow-up revealed the production of cortisol to be independent of the regulation of the hypothalamo-pituitary-adrenal axis. Therefore, he was diagnosed as having subclinical cushing syndrome, and these tumors were removed. Adrenocorticotropic hormone-independent macronodular adrenal hyperplasia (AIMAH) was endocrinologically and histopathologically diagnosed. This is the first report demonstrating that endocrinologic changes of AIMAH can be observed during long-term follow-up.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
6/116. Primary aldosteronism due to unilateral adrenal hyperplasia: report of two cases and review of the literature.Unilateral adrenal hyperplasia (UAH) is a rare, surgically correctable subset of primary aldosteronism (PA), which shows similar endocrine features to aldosterone-producing adenoma (APA). We report here two Japanese patients with UAH. Case 1 was a 62-year-old man with a four-year history of hypertension. hypokalemia and suppressed plasma renin activity (PRA) with elevated plasma aldosterone concentration (PAC) were observed, while no adrenal nodules were identified by abdominal computed tomographic (CT) scan. Adrenal scintigraphy did not reveal definite localization. The selective adrenal-vein sampling for determinations of PAC showed an over-functioning left adrenal gland, and a left adrenalectomy was performed. Diffuse micronodular adrenocortical hyperplasia was observed. Case 2 was a 61-year-old man with a six-year history of hypertension. At the first visit to our hospital, hypokalemia and suppressed PRA with elevated PAC were observed. An abdominal CT scan showed a left adrenal mass 1.5 cm in diameter, while adrenal scintigraphy did not reveal definite laterality. A left adrenalectomy was performed, and three macronodules and diffuse micronodular adrenocortical hyperplasia were observed. hypokalemia, hypertension and endocrine data became normal, and both patients have been well with no signs of recurrence for eight years (case 1) and seven months (case 2) after surgery. Clinical characteristics and endocrine features of UAH are also reviewed.- - - - - - - - - - ranking = 3keywords = hypertension (Clic here for more details about this article) |
7/116. Adrenocorticotropin-independent unilateral adrenocortical hyperplasia with Cushing's syndrome: Immunohistochemical studies of steroidogenic enzymes, ultrastructural examination and a review of the literature.A 60-year-old woman presented with a history of palpitations, headaches and severe hypertension, which was resistant to hypotensive agents. She had a 2-year history of obesity and a moon face. Her plasma adrenocorticotropic hormone level was below the limits of detection and did not respond to corticotropin-releasing hormone. Urinary-free cortisol was elevated and the circadian rhythm of serum cortisol level had completely disappeared. Imaging analysis demonstrated a unilaterally functioning mass in the left adrenal gland. serum cortisol level in the left adrenal vein was elevated. The resected adrenal mass measured 4 x 3.5 x 2.5 cm, and ranged from yellow to tan in color. The adrenal cortex adjacent to the nodule did not demonstrate cortical atrophy. The mass was well circumscribed but not encapsulated, and consisted of multiple cortical nodules. These nodules were composed predominantly of clear cortical cells, and partly of compact cortical cells. Immunoreactivity of steroidogenic enzymes including cholesterol side-chain-cleavage P450, 3beta-hydroxysteroid dehydrogenase, 21-hydroxylase cytochrome P450, 11beta-hydroxylase cytochrome P450 and 17alpha-hydroxylase cytochrome P450 was marked in cortical nodules, but minimal in non-nodular cortex. Ultrastructural examination of nodular cortical cells also demonstrated well-developed mitochondria and smooth endoplasmic reticulum, consistent with elevated steroidogenesis in these cells.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
8/116. Intermittent hyperaldosteronism in a child due to an adrenal adenoma.aldosterone producing adenoma (APA) is a rare but potentially curable form of paediatric hypertension. We report a case of APA in a 9-year-old boy, suspected due to persistent hypokalaemia. Neither BP nor initial laboratory investigations disclosed the diagnosis and the presence of an APA was suggested by functional tests and radiological findings. Histologically, a cortical tumour was found associated with a marked medullary hyperplasia of both chromaffin and ganglion cells. CONCLUSION: This case reinforces the need for further investigations in patients with misleading clinical and laboratory data.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
9/116. A case of unilateral adrenal hyperplasia: the diagnostic dilemma of hyperaldosteronism.OBJECTIVE: To report the successful laparoscopic surgical management of a case of right unilateral adrenal hyperplasia. methods: We present a case of unilateral adrenal hyperplasia and review the pertinent literature. The effectiveness of surgical treatment of unilateral adrenal hyperplasia is evaluated. RESULTS: A 50-year-old man had a 7-year history of hypertension, which had been treated with antihypertensive medications. On initial assessment, he was taking long-acting diltiazem, 240 mg twice a day, and his blood pressure was 150/84 mm Hg. He was noted to have recurrent hypokalemia, low plasma renin activity, increased plasma aldosterone concentration, and high urinary aldosterone levels. magnetic resonance imaging of the adrenal glands revealed a normal left adrenal gland and fullness of the right adrenal gland. Selective adrenal vein catheterization showed findings consistent with a right-sided adrenal gradient. Surgical removal of the right adrenal gland laparoscopically alleviated his symptoms and cured his hypertension and hypokalemia. Pathologic examination of the right adrenal gland revealed diffuse hyperplasia of the zona fasciculata and zona reticularis. plasma aldosterone, renin, and potassium levels returned to normal postoperatively, and his blood pressure declined to 120/70 mm Hg. At 1-year follow-up, the patient remained normotensive without the need for antihypertensive agents. CONCLUSIONS: This rare type of hyperaldosteronism, known as unilateral adrenal hyperplasia, is difficult to categorize not only because it fails to typify a conventional subtype but also because it is difficult to confirm the pathologic diagnosis. Recognition of the variety of provocative, biochemical, and imaging results that may be seen in this disorder is important in making the correct diagnosis. Determining unilaterality of disease by selective adrenal vein sampling can result in a cure of hypertension and hypokalemia by surgical resection.- - - - - - - - - - ranking = 3keywords = hypertension (Clic here for more details about this article) |
10/116. Nodular regenerative hyperplasia of the liver in a patient with celiac disease.We present the case of dual adult celiac disease and liver disease with portal hypertension (esophageal varices); a percutaneous liver biopsy was compatible with nonspecific reactive hepatitis. Clinically, celiac disease was characterised by poor response to a gluten-free diet, with the development of a biochemical cholestasis and marked malnutrition. Our patient died of cerebral hemorrhage, at the age of 50 years, without associated risk factors. The necropsy demonstrated the existence of a nodular regenerative hyperplasia of the liver, splenic atrophy, gelatinous transformation of the bone marrow, and lymphocytic colitis. We discuss the different types of liver disorders associated with celiac disease and the possible relation between nodular regenerative hyperplasia and celiac disease, based on immunologic mechanisms.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
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