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Biomedical subjects

J Donckier

Publications and source records attributed to J Donckier.

At least 19 recordsLinked to original sources

Metastasis of a small cell carcinoma from undetected origin to the pituitary gland--the otolaryngologist's view.

Tumour metastasis to the anterior pituitary-hypothalamic area is rare. We present a patient who had severe headache, bitemporal quadrant hemianopsia and an expanding mass in the sella turcica as revealed by MRI. Partial resection via a transsphenoidal approach was performed and postoperative radiation therapy was initiated. Immunohistochemical investigation identified the tumour as a metastatic small cell carcinoma whose primary site remained undetected for more than 12 months despite repeated oncological evaluations. We reviewed the literature on metastatic disease of the pituitary gland.

Aged↗

Late-onset primary hyperoxaluria triggered by hypothyroidism and presenting as rapidly progressive renal failure--description of a new mutation.

Primary hyperoxaluria type 1 (PH1) is a rare autosomal metabolic recessive disease, caused by the deficiency of the liver peroxysomal alanine:glyoxylate aminotransferase (AGT), characterized by accumulation of calcium oxalate crystals in kidneys and others organs. We present the case of an elderly woman with PH1, presenting as acute renal failure. Precipitation of calcium oxalate crystals was probably due to amiodarone-induced severe hypothyroidism. Residual AGT activity is associated with the G170R (G630A) mutation. A new mutation of AGT, called R36C, was also discovered; the role of this new mutation is actually not known.

Age Factors↗

[Increased expression of endothelin-1 and its mitogen receptor ET(A) in thyroid papillary cancer in humans].

Since its discovery in 1988, endothelin has initiated intense research activities, showing that it was not only a vasoconstrictor and mitogenic peptide produced by the vascular endothelium but also a ubiquitous molecule with various functions. A production of ET-1 has been evidenced in the thyroid and ET-1 receptors have been detected on thyroid cells. Produced by the vessels and the thyrocytes, ET-1 could exert paracrine and/or autocrine effects on vascular tone in the thyroid and on some functions of thyrocytes. Because of proliferative effects, its role in thyroid diseases could involve goitrogenesis and carcinogenesis. Using thyroid samples obtained at the time of surgery, we demonstrated increased expressions of ET-1 and its mitogenic receptor ET(A) in papillary carcinoma of the thyroid. Immunohistochemical studies and real-time quantitative polymerase chain reaction (RTQ-PCR) were used for this purpose. As demonstrated in other cancers, activation of endothelin axis, particularly through ET(A) receptor could contribute to cell proliferation, cell survival, angiogenesis and the development of bone metastases. ET-1 is also a proinflammatory mediator and we demonstrated an overexpression of ET-1 and of its receptor ET(A) in Hashimoto's thyroiditis. This suggests a role of ET-1 as a cytokine. In nodular goitre, ET-1 is also overexpressed but to a lesser extent than in papillary thyroid carcinoma and in Hashimoto's thyroiditis. Finally, antagonists of ET-1 receptors are currently under development and could be used in man. They could open new therapeutic perspectives in the treatment of metastatic thyroid carcinoma.

Carcinoma, Papillary↗

Emergency hepato-renal artery bypass using a PTFE graft.

A 51-year-old patient suffering from Buerger's disease with bilateral lower limb amputation and Leriche syndrome presented with uncontrollable hypertension and renal failure caused by right renal artery subocclusive stenosis associated with an occluded left renal artery and atrophic kidney. He underwent a right hepato-renal bypass grafting using an externally supported polytetrafluorethylene (PTFE) graft. Renal function improved markedly and hypertension could be controlled by standard antihypertensive treatment. Normal right renal function was maintained at one-year follow-up.

Angiography↗

Decreased expression of myocardial eNOS and caveolin in dogs with hypertrophic cardiomyopathy.

Because nitric oxide (NO) regulates cardiac and vessel contraction, we compared the expression and activity of the endothelial NO synthase (eNOS) and caveolin, which tonically inhibits eNOS in normal and hypertrophic cardiomyopathic hearts. NOS activity (L-[(3)H]citrulline formation), eNOS immunostaining, and caveolin abundance were measured in heart tissue of 23 mongrel dogs before and at 3 and 7 wk of perinephritic hypertension (PHT). Hemodynamic parameters in vivo and endothelial NO-dependent relaxation of macro- and coronary microvessels in vitro were assessed in the same animals. eNOS immunostaining and total calcium-dependent NOS activity decreased at 7 wk in all four heart cavities (in left ventricle, from 17.0 +/- 1.3 to 0.2 +/- 0.2 fmol. min(-1). mg protein(-1), P < 0.001). Caveolin-1 and -3 also decreased in PHT dog hearts. Accordingly, basal vascular tone was preserved, but maximal endothelial NO-dependent relaxation was impaired in all vessels from 7-wk PHT dogs. The latter had preserved systolic function but impaired diastolic relaxation [relaxation time constant (T(1)), 25.1 +/- 0.9 vs. 22.0 +/- 1 ms in controls; P < 0.05]. Peripheral infusion of the NOS inhibitor N(G)-nitro-L-arginine methyl ester increased mean aortic pressure in both groups and reduced diastolic (T(1), 31.9 +/- 1.4 ms) and systolic function in PHT dogs (DP40, 47.5 +/- 2.5 vs. 59.4 +/- 3.8 s(-1) in control animals). In conclusion, both eNOS and caveolin proteins are decreased in the hypertrophic hearts of PHT dogs. This is associated with altered maximal (but not basal) vascular relaxation and impaired diastolic function. Further degradation of cardiac function after NOS inhibition suggests a critical role of residual NOS activity, probably supported by the concurrent downregulation of caveolin.

Animals↗

[Cardiac incidentaloma].

We describe a case of lipomatous hypertrophy of the atrial septum mimicking a tumor of the right atrium. The association of echocardiography and radiology for the detection of this disease is illustrated in this report.

Aged↗

Hantavirus infections: clinical presentation in the emergency room.

We present a retrospective review of Hantavirus infection in the emergency department. Thirteen cases of Hantavirus infections with renal syndrome from July 1989 to August 1999 were analysed. The diagnosis was confirmed by detection of Hantavirus antibodies in all cases. Fever, chills and headaches were universally present. Intense back pain was associated in 77% of the patients. Thrombocytopenia, abnormal urinalysis, hypertransaminasaemia, increased lactate dehydrogenase were the principal biological patterns. All these parameters returned to their normal level, and all the patients recovered a normal renal function without sequels. The management is supportive. Only one patient in our series had to be dialysed. Hantavirus disease should be included in the differential diagnosis of acute renal failure with thrombocytopenia, particularly in patients with suspected exposure in known endemic areas. The differential diagnosis of any perplexing case of undifferentiated febrile illness with acute renal failure and thrombocytopenia should include Hantavirus infection.

Acute Kidney Injury↗

Ingestion of antacid tablets (Rennie) and acute confusion.

The authors describe a case of milk-alkali syndrome in a man who consumed antacid tablets (Rennie) for chronic epigastric pain. Simultaneous occurrence of hypercalcemia, metabolic alkalosis, and renal insufficiency, in conjunction with the appropriate history of ingestion of calcium carbonate-containing antacids, was suggestive of the syndrome. The syndrome became uncommon with the advent of modern ulcer therapy, but currently is increasing in frequency with the calcium supplementation drugs taken to prevent osteoporosis. This syndrome may produce life-threatening hypercalcemia.

Antacids↗

Sleeping eyes.

Explore the source record for details and available documents.

Aged↗

[Evaluation of corticosteroid replacement therapy and its effect on bones in Addison's disease].

Assessment of patients on steroid replacement therapy is important to avoid the consequences of overtreatment such as osteoporosis. The aim of this prospective study is to evaluate the severity and the etiology of osteopenia in 24 patients (15 women, 9 men) with Addison's disease receiving 30 mg hydrocortisone. Mean age of patients was 55 15 years. Osteoporosis, diagnosed by the measurement of bone mineral density (BMD) at the level of lumbar spine and right hip, was found in 58% of patients, i.e. in 10 women and 4 men. The latter had normal testosterone levels while seven women had an early menopause, the etiology of their Addison's disease being autoimmune. Three were on hormonotherapy. Correlations were found between BMD in the femoral neck and hip and the dose of hydrocortisone (mg/m(2)/day; mg/kg/day), the duration of treatment and 24 hr-cortisoluria/g creatinine. Multivariate analysis shows that 24-hr cortisoluria/g creatinine is a good predictor of BMD values. Thus, osteoporosis is frequent in Addison's disease and cortisoluria could be a useful tool to predict this complication.

Addison Disease↗

[Prolactinoma in man: clinical and histological characteristics].

Prolactinoma usually occurs a small intrasellar tumor in women or as a large tumor in men. To determine whether the predominance of macroprolactinomas in men is due to a delay in diagnosis as has been suggested, or whether there is a sex-related difference in growth rate we conducted a retrospective study in 45 men and 51 women with prolactinomas. Preoperative prolactin level (PRL) was 2,789 573 ng/ml and mean tumor size was 26 2 mm. Prolactin levels and tumor size were significantly higher in women (292 74 ng/ml and 10 1 mm; p<0.01). There was no correlation with age at diagnosis or duration of symptoms. Giant tumors were only observed in men (n=8). Frequency of resistance to bromocriptine (30% vs 5%, p<0.01) and invasive tumors (52% vs 27%, p<0.001) were significantly higher in men than in women. Likewise, proliferation rate was higher for the prolactinomas in men (Ki-67: 2.6 1.1% positive nuclei vs 0.4 0.2%; p=0.08; PCNA: 5.0 2.3% vs 3.7 1.1%). In conclusion, prolactinomas in men are more aggressive than in women. They grow rapidly, often invade the cavernous sinus and are resistant to bromocriptine; proliferation rates can be increased.

Adolescent↗

Laparoscopic adrenalectomy for phaeochromocytoma: endocrinological and surgical aspects of a new therapeutic approach.

OBJECTIVES: To evaluate the results of laparoscopic resection of phaeochromocytoma with a focus on pre- and intra-operative endocrinological and pharmacological aspects. DESIGN: Retrospective study based on review of case notes and intraoperative anaesthetic records. PATIENTS: Eight patients (four men, four women) aged 13 to 70 (median: 45) years with symptomatic phaeochromocytoma (two patients with MEN IIa syndrome) diagnosed 6-36 months before adrenalectomy in four patients and just before operation in the four remaining patients. All patients presented with hypertension. MEASUREMENTS: Pre- and postoperative blood pressure (BP) was assessed using a sphygmomanometer, intraoperative measurements being obtained by intra-arterial line. Adrenaline (A), noradrenaline (NA), metadrenaline (metA), normetadrenaline (normetA) and vanillylmandelic acid (VMA) were measured in 24-h urine samples. Adrenal imagery consisted in all patients of abdominal computed tomography and [131I] metaiodobenzylguanidine (MIBG) scintigraphy, and in 2 patients, adrenal magnetic resonance imaging and octreotide scintigraphy. Determination of plasma volume was performed by isotopic method. RESULTS: Median (range) preoperative systolic and diastolic BP was 140 (130-240) and 90 (80-150) mm Hg, respectively. Predominant catecholamine secretion consisted of A in four patients (A pattern) and of NA (NA pattern) in the other four. Tumours smaller than 30 mm usually secreted A while larger ones secreted NA. Pre- and intra-operative BP control was straightforward in all but two patients with preferential NA secretion. The median number of systolic and diastolic BP peaks were higher in patients with NA pattern (n = 6, range: 3-9) than in those with A pattern (n = 1.5, range: 1-6). Total duration of pneumoperitoneum ranged from 120 to 240 (median: 195) min. Intra- and post-operative complications included intra-abdominal bleeding, laparoscopically controlled (in one case), and upper pole kidney ischaemia in another. Postoperative hospital stay ranged from 4 to 11 days. All patients were asymptomatic postoperatively and pharmacologically controlled hypertension persisted in two of them. CONCLUSIONS: Laparoscopic adrenalectomy is a safe and efficient technique to remove phaeochromocytoma in terms of intra- and postoperative morbidity, with a low complication rate. Pre- and intra-operative pharmacological blood pressure control is as effective as in conventional adrenalectomy, with greater instability in noradrenaline secreting tumours.

Adolescent↗

Hemiplegia hypoglycaemia syndrome.

We report the case of a 83-year-old man who presented to the emergency department with hypoglycaemia resembling a cerebrovascular accident. Hypoglycaemic hemiparesis is an under-recognized manifestation of hypoglycaemia. If not recognized and treated promptly, hypoglycaemia may cause irreversible central nervous system injury; it rarely results in death. It is imperative that emergency physicians consider hypoglycaemia in all patients with coma in spite of focal neurological deficit even when the findings seem to be explained initially by other aetiologies.

Aged↗

Heart failure induced by pheochromocytoma: laparoscopic treatment and intraoperative changes of several new cardiovascular hormones.

Since 1992, adrenalectomy for pheochromocytoma has been recognized as a safe and efficient technique when performed by a laparoscopic approach. Most of the cases of pheochromocytomas treated as such and published in the literature were not associated with malignant hypertension and acute heart failure. We report the case of a 23-year-old woman who presented with this clinical picture and show that laparoscopic adrenalectomy may be as safe and efficient as conventional adrenalectomy when performed in this situation. The intraoperative changes in the secretion of catecholamines, endothelin-1, angiotensin II, N- and C-terminus of atrial natriuretic factor prohormone were also analyzed. Noradrenaline release during tumor dissection was associated with a stimulation of atrial natriuretic factor.

Adrenal Gland Neoplasms↗