Sample records for medular tiroideo hereditario

  1. Como Lo Hago Yo: Lipomas Medulares

    PubMed Central

    Portillo, Santiago


    Basados en la experiencia de 82 casos; en 5% se observaron anomalías pélvicas asociadas. En los menores de 3 años el motivo de consulta (85%) fue la tumoración. En los mayores de 3 años (42%) tenía problemas neurológicos. Solo el 24% preocupados por la tumoración. El objetivo de la cirugía es desanclar la médula y no remover la totalidad del lipoma. El lipoma de filum es el que es mas simple para operar. Aún cuando la escuela francesa propone operar solamente cuando hay síntomas favorezco cirugía preventiva. Favorezco una segunda cirugía si hay signos de anclaje postoperatorio, aún cuando observamos empeoramiento postoperatorio motor en 2.5% de los operados y urológico a largo plazo en 6% de los operados. PMID:24791218

  2. [Bone marrow transplantation in Mexico. Report of the 1st successful case in acute myeloblastic leukemia. Grupo de Trasplante Medular Oseo del INNSZ].


    León, E; Sosa, R


    The first case of allogeneic bone marrow transplantation in acute myelogenous leukemia (AML) done in Mexico is reported. The patient was a 26 year old Mexican woman who in October 1987 was diagnosed of having AML of the M2 subtype. After three cycles of the TADOP regimen (6-thioguanine, cytosine-arabinoside, doxorubicin, vincristine & prednisone), the patient entered complete remission. Unfortunately, after a seven month period of remission she suffered a relapse which was refractory to a new chemotherapy cycle. On 9/14/88 an allogeneic BMT from her HLA identical brother was performed. The conditioning regimen consisted of busulfan and cyclophosphamide. Prophylaxis for GVHD consisted of cyclosporine and methylprednisolone. The posttransplantation course was satisfactory, reaching > 500 neutrophils x 10(9)/L on day 14 and > 50,000 platelets x 10(9)/L without support on day 23 posttransplant. The patient developed fever of unknown etiology, which was satisfactorily resolved with ceftazidime, vancomycin and metronidazole. She also presented a grade II oral and esophageal mucositis. As a late complication, on day 90 posttransplant, she developed a bilateral pneumonia which was resolved with sulfamethoxazole-trimethoprim administration. Up to the time of this report (40 months posttransplant) the patient is completely asymptomatic, is under no immunosuppression, and shows no evidence of graft versus host disease or recurrent leukemia. PMID:1488582

  3. [Morbus Albers Schonberg osteopetrosis].


    Saracević, Ediba; Hasanbegović, Edo


    The patient presented has bone disease, so called "mramor disease". The disease is characterized by insuffiticnty of ostcoclasts which results n persisting of primary spongiosis without normal eroding and bone rebuilding. The discase is diagnosed by X rays where the bone density is increased. The medular space is completly closed by dense bone knitting which, with soft cortical space, seems like bone in bone. This is autosomal recessive disease with serious clinical picture in infants. PMID:15875483

  4. [Spinal cord compression disclosing rib hydatidosis].


    Ousehal, A; Adil, A; El Azhari, A; Kadiri, R


    The authors report an exceptional case of spinal compression following an isolate rib hydatidosis. The CT scan has suspected the diagnosis. The authors recall the anatomoclinic features and specify the radiologic aspects of the osseous hydatidosis, especially the rib's localization which is very rare. MR imaging in addition of its diagnosis role showing a very evocative cyst images, is the exam of choice in order to appreciate the disease's extent and the degree of medular sufferance. PMID:8676297

  5. [Proposal for a survey for assisting the family and caregivers of patients with spinal cord injuries].


    Mancussi e Faro, A C


    This study proposes a basis survey for assistance to the family and caregivers because we believe there is necessity of family participation on the treatment, trying to understand and share the disease or deficiency situation. We objectified to sketch the relationship degree and the people's gender that accompanied the spinal cord injured hurt medular patient in nursing consultations and to discuss the necessity of basis survey to the assistance family and to the caregiver. 101 nursing consultations were accomplished, in clinic health, to the spinal cord injured patient and his/her relative and 36 patients were totalized, from this number 26 (72.22%) were male and 10 (27.78%), were female. It was verified that the men with medular lesion, in the greater number (27-80.7 O/o), were accompanied with his relatives specially mother and wife, while the women with medular lesion, in the greater number too (7-70%) were accompanied with her relatives of diversified proximity. In reference to the basis survey that comprehend the care at house and the caregiver necessity, we can affirm its relevance, trying to context the family support identified attending the caregivers. PMID:11337805

  6. Combined procedure of vascularized bone marrow transplantation and mesenchymal stem cells graft - an effective solution for rapid hematopoietic reconstitution and prevention of graft-versus-host disease.


    Coliţă, Andrei; Coliţă, Anca; Zamfirescu, Dragos; Lupu, Anca Roxana


    Hematopoietic stem cell transplantation (HSCT) is a a standard therapeutic option for several diseases. The success of the procedure depends on quality and quantity of transplanted cells and on stromal capacity to create an optimal microenvironment, that supports survival and development of the hematopoietic elements. Conditions associated with stromal dysfunction lead to slower/insufficient engraftment and/or immune reconstitution. A possible solution to this problem is to realize a combined graft of hematopoietic stem cells along with the medular stroma in the form of vascularized bone marrow transplant (VBMT). Another major drawback of HSCT is the risk of graft versus host disease (GVHD). Recently, mesenchymal stromal cells (MSC) have demonstrated the capacity to down-regulate alloreactive T-cell and to enhance the engraftment. Cotransplantation of MSC could be a therapeutic option for a better engraftment and GVHD prevention. PMID:22677297

  7. [Participation of the adrenals in the pathogenesis of metabolic acid-base disorders].


    Iluchev, D; Shtereva, S


    The authors examined in dynamics the changes in the functional state of the adrenals on 240 rabbits, which served as models for acute metabolic deviations in the acid-base balance. The obtained results showed that the acute metabolic acidosis increased moderately the values of ACTH and 17-hydroxycorticosteroids in blood without changing their concentration on the adrenal tissue. It lowered strongly the content of catecholamines (adrenaline and noradranaline) in the adrenal medular part. The metabolic alkalosis raised the concentration of ACTH in blood plasma and increased the amount of corticosteroids in blood and adrenals. There was no well formed parallelism in normalizing acid-base and hormonal indices. As a consequence of this a stage of postaciodotic catecholamine adrenal deficit was formed as well as metabasic hypercorticism in the experimental animals. PMID:14819

  8. [Shouldn't the Achilles' heel be on the foot - regarding a clinical case].


    Cavaco, Raquel; Machado, Célia; Gorjão Clara, J


    Clinical suspicion is the bedrock of a thorough clinical work-up, and a review of the clinical files with no definitive diagnosis is a must. Such a review can uncover rare diagnoses, such as Waldenstrom's macroglobulinaemia, a proliferative B-cell disease characterised by medular infiltration and monoclonocal IgM production. 1% of these are biclonal. We present a case of a 74 year old patient diagnosed in October 2004 with acute cholecystitis. Surgical evaluation revealed bilateral pleural effusion with an inconclusive aetiology. Patient underwent a right pleurodesis in May 2005 and the aetiology remained inconclusive. The third evaluation, in July 2005, led to a final diagnosis of Waldenstrom's macroglobulinaemia. PMID:19023500

  9. [Cushing syndrome due to ectopic ACTH secretion].


    Mendonça, B B; Madureira, G; Bloise, W; Albergaria, A; Halpern, A; Liberman, B; Villares, S M; Batista, M C; Avancini, V F; Nitterdorfi, C T


    The authors studied 8 patients (4 males and 4 females) with Cushing's syndrome due to ectopic ACTH secretion. Chronological age ranged from 15 to 45 years and duration of the disease ranged from 3 to 48 months. All patients presented typical signs of Cushing's syndrome, blood hypertension, and four of them had hyperpigmentation of the skin. Five patients had fasting hyperglycemia and all patients but one had serum hypokalemia (serum K = 2.2 to 3.9mEq/l). The circadian rhythm of cortisol was absent in all patients and basal cortisol levels were elevated in all patients but one. Basal ACTH levels evaluated in 7 patients were elevated in 6 (29 to 1050 pg/ml-MRC). One patient presented normal depression of urinary 17-OH after two days of dexamethasone and normal increase of urinary 17-OH and serum 11-dexycortisol after methyrapone. Four patients had carcinoid tumor (3 thymic and 1 bronchial), two had pancreatic islets cell tumors, one had bilateral pheochromocytoma and medular carcinoma of the thyroid, and one had oat cell carcinoma of the lung and medular carcinoma of the thyroid. Thoracic X-rays identified the ectopic ACTH secretion tumor in four cases, all confirmed by CT scan. Abdominal CT showed a difuse enlargement of the adrenals in seven cases and bilateral nodules in one case (pheochromocytomas). Six patients died within 3 years of the diagnosis. The authors concluded that clinical and hormonal findings could mislead the findings of ACTH ectopic secretion and Cushing's disease, and suggest that thoracic X-rays and CT scans of the skull, thorax, and abdome should be done in all cases of Cushing's syndrome. PMID:2559451

  10. [Traumatic rupture of the thoracic aorta].


    Glock, Y; Roux, D; Soula, P; Cerene, A; Fournial, G


    This is a retrospective analysis of 50 postraumatic aortic rupture (1968-1996, 39 males, mean age: 34.5). Group A is composed of 35 patients with an acute aortic rupture and a prompt diagnosis. Group B includes 13 patients with a chronic rupture. All patients from group A had a severe politraumatism with abdominal, cranial, extremities or hip fractures. Mediastinal thickening with or without hemothorax indicated an angiography or a transesophageal echocardiography lately. In group A, 36 patients have been operated on urgently (12-24 hours); cardiopulmonary bypass was performed on 20 patients; an aorto-aortical bypass was done in 27 cases and a direct suture in the remaining 9. In group B, cardiopulmonary bypass was performed on 9 patients; a aorto-aortical bypass was done in 11 cases and a direct suture in 2. Overall hospital mortality was 16%; 19% in group A and 7.6% in group B. Ischemic paraplejia appeared in 5 patients (10%), all from group A. No false aneurysm developed after 4.5 years of follow-up (3-135 months) in the 38 survivors. The usefulness of transesophageal echocardiography, the importance of medular protection and the utility of several interventionist radiologic techniques are discussed. PMID:9053930

  11. [Histopathology of Q rickettsiosis in sheep].


    Belchev, L; Pavlov, N


    Examined was material taken from five sheep (ewes) and two weaned lambs having naturally contracted Qu rickettsiosis. Described are the clinical symptoms of the disease and the morphologic changes. The diseased animals showed rise in temperature (39.5--40.5 degrees C), loss of appetite, and depression. Some of the weaned lambs manifested slight cough and digestive troubles. Part of the animals showed nervous symptoms--tic movements of the head and limbs. Morphologically, the liver was edematired, of lower compactness, and the spleen was enlarged, the meninges being hyperemic and peppered with pinpointed hemorrhages. Histologically, a strong diffuse activation and proliferation of the liver capillary endothelium was established along with necrobiosis of the liver epithelial cells and a diffuse leukocyte infiltration. Established was also hyperplasia of the reticular cells and the lymph follicles of the spleen and the bronchial lymph nodes. The epithelial cells of the kidney tubules were involved in vacuolar dystrophy, and in the medular section there were fibroblastic proliferations with hyperemia. Inflammatory changes in the brain were also found. PMID:607554

  12. [A urinary outbreak of Acinetobacter baumanii in a spinal cord injury unit].


    Pedraza, F; Andreu, A; Saune, M; Moreno, A; Ramírez, L; García, L


    From January 1990 to April 1992, 114 urinary strains of Acinetobacter baumanii were isolated in 57 patients with traumatic spinal cord [correction of medular] injury. The strains were characterized by having all of them the same biochemical identification, except for citrate, maltose and tryptophan-desaminase. Until December 1990, (5 strains) were resistant to all antibiotics, except to tobramicine, amikacine, cotrimoxazol and imipenem (6.3%, 33.9%, 26.7% and 0% of resistances, respectively); since January 1991, (99 strains) became resistant to all of them, except to imipenem. 39.5% of AB were isolated in pure cultures, 46% of them with pyuria. Between February 1991 and January 1992, we observed the highest number of affected patients, although without seasonal predominance. We observed as well a higher incidence among males (46 males, 11 females). 80% of them carried a permanent probe. Only 6 patients presented clinical signs directly related to AB. The environmental study could not demonstrate any source of contagion or transmission mechanism. PMID:8452972

  13. [Buerger's disease or thromboangiitis obliterans].


    Léger, P; Pathak, A; Hajji, L; Faivre-Carrere, C; Boccalon, H


    The Buerger's disease or thromboangiitis obliterants (TAO) is a non atheromatous inflammatory disease which alters medium and small-sized arteries and veins. It can be found world-wide, but it is more frequent in Eastern Europe, Middle East, Asia and Southeast Asia. Young men and smokers are the most affected. The incidence of this disease is increasing among women. The cause of this disease is unknown yet. The most striking fact is the relationship between TAO and tobacco. The diagnostic is most often late in front of a digital leg ischemia. Complementary exams help to its diagnostic and management but none of them are specific out of the pathology. The affection is evolving towards distal gangrene with amputation in 5 to 10% of cases. Prostacyclin demonstrated its efficiency. Revascularization surgery is difficult but sometimes possible. Sympathectomy, medular stimulation must be suggested. The patient survival is not at stake and the prognosis is above all functional. The most important element in the treatment is stop smoking definitively. PMID:12555498

  14. [Secondary biliary cirrhosis in a patient with histiocytosis X].


    Romão, Zita; Pontes, J; Andrade, P; Leitão, M C; Donato, A; Freitas, Diniz


    Histiocitose X or Histiocytosis of the Langerhans cells represents a complex spectrum of clinical alterations, resulting from infiltration by anomalous histiocytes of various organs, including the skin, bones, lungs, lymphatic ganglia and liver. Liver disease is rare and the mechanism by which lesions appear is unknown. Cholestasis results from phenomena of sclerosant colangitis, which affects the intrahepatic ducts, or from proliferation of histiocytic cells in the periportal areas. Some patients develop biliar cyrrhosis. The authors present the clinical case of a 62-year-old female patient, hospitalized for chronic cholestasis, diabetes and gallstone in the main bile duct. She had metastatic lesions of the hypophysis and bones, the biopsies of which revealed infiltration by histiocytic cells. Endoscopic Retrograde Colangiopancreatography (E.R.C.P.) revealed dilatation of intrahepatic bile ducts and stenosis of left hepatic duct. A histological study of the hepatic biopsy showed chronic cholestasis and areas of fibrosis, without infiltration by histiocytic granulomas, which were observed in the medular biopsy and in the thyroid nodule cytology. PMID:12025455

  15. [Immune complex glomerulonephritis associated with pulmonary tuberculosis].


    Villar, I; Hernández, E; Cozzi, J; Paletta, C; Mathurín, S


    A 32 year old man was admitted for dyspnea, hemoptysis, macroscopic hematuria, hypertension (140/100), peripheral edema and hemodynamic decompensation. Lung Xrays revealed pulmonary edema and a cavity in the left apex. Laboratory determinations revealed an altered renal function with increased creatinine and urea levels and nephrotic syndrome. There was leucocyturia, hematuria and cylindruria. The sputum showed a large number of acid-fast bacilli. The patient began anti-tuberculosis treatment with three drugs (isoniacid, rifampicin, pirazinamide). On ultrasonography, both kidneys revealed ecogenic lesions with size, shape and cortico-medular relationship preserved. The patient persisted with altered renal function, steady levels of urea nitrogen, creatinine and potassium, preserved diuresis and hypertension. Bidimensional echocardiogram: LVDD 55 mm, hypoquinetic septum, pericardic effusion, thickened pericardium, pleural effusion, shortening fraction decreased. He received treatment for this congestive cardiac failure and hypertension with enalapril, nifedipine and fursemide. A percutaneous renal biopsy was performed with anatomopathologic diagnosis of diffuse encocapillar proliferative glomerulonephritis with crescents (15%) and total glomerular sclerosis (33%). Immunofluorescence: positive, immune-complexes with IgM and C3. The patient gradually recovered his normal renal function, improved his pleural effusions and normalized his cardiac function. He was discharged in good clinical condition on the 69th day of anti-tuberculosis treatment. An association between pulmonary tuberculosis and glomerulonephritis is discussed. It is proposed that renal lesions might be the consequence of the tuberculosis due to the sedimentation of circulating immune-complexes. PMID:7854090

  16. Morpho-functional patterns of kidney injury in the experimental leptospirosis of the guinea-pig (L. icterohaemorrhagiae).


    Dávila de Arriaga, A J; Rocha, A S; Yasuda, P H; De Brito, T


    Thirty-seven guinea-pigs experimentally infected with a virulent strain of L. icterohaemorrhagiae, were submitted to a renal function study as evaluated through the maximal urinary concentration (MUC) test, blood urea nitrogen (BUN) and afterwards had their kidneys examined by light and electron microscopy. Vascular changes were also studied after the administration of colloidal carbon as a marker. Through the MUC test and BUN determination, two groups of tubulo-interstitial lesions can be visualised, one in animals without renal sufficiency, manifested chiefly by cell edema with RE dilation and another, in animals with renal insufficiency, characterised not only by marked cell edema and mitochondrial changes, but also by proximal tubule regenerative aspects without overt tubular necrosis. Interstitial edema and focal nephritis was prominent in both groups, a finding which minimises their role in the pathogenesis of renal failure in experimental leptospirosis. Vascular injury, affecting the vessels of the renal microcirculation chiefly at the cortico-medular junction, was observed in both groups. Its severity and extension ran parallel to the intensity of the tubular injury. This suggests a simultaneous action of a noxious agent liberated by the leptospires over both structures, tubular damage being accentuated by the local circulatory changes. PMID:7131130

  17. [Clinical effects of the transfusion of leukocytes isolated by filtration from continuous flux].


    Malinvaud, G; Gailiard, S; Gualde, N


    The present work studies clinical effects of leukocyte transfusions to patients with medular aplasis. Leukocytes were collected by filtration on a continuous flow, according to the technique earlier described in this review [9]. Two major points are stressed on tolerance by the patients of the injected products and clinical efficiency. Seventy eight suspensions were prepared and transfused to 30 patients in the course of 36 incidents of myeloid insufficiency. All patients but two evidenced by the time of transfusion a number of polynuclears inferior to 500 per cubic millimeter. The infection was quite serious with increased gravity despite the antibiotherapy. Intolerance was noticeable in about one third of the cases, half of which consisted only in chillis by the end or after transfusion. Only one accident consisting in acute respiration troubles and shock was observed. This however does not occur by chance. It involves sensitization which may be related to HLA system but may also be of different nature, although not clearly identified. Nevertheless is efficiency of the injected products demonstrated by recirculation of the transfused leukocytes. This was noticed within an hour following transfusion for more than 50 percent of the cases. Furthermore it lasted for 16 hours in more than one fourth of the patients. In addition following results are in favour of real clinical efficiency. Certainly in the course of 16 aplasic incidents, no improvement was observed. For most patients however transfusions were late and not renewed or the patients were highly immunized. Conversely the infection state did improve in 8 patients, the disease responsible for aplasia running its course on its own. Lastly in the course of 12 aplasic incidents, infection and acute aplasia did cure. All these observations should lead one to study with great care the immunological state of the recipient. Instructions being known, the number of transfused leukocytes should be sufficient and

  18. Behavioral coping strategies in response to social stress are associated with distinct neuroendocrine, monoaminergic and immune response profiles in mice.


    De Miguel, Zurine; Vegas, Oscar; Garmendia, Larraitz; Arregi, Amaia; Beitia, Garikoitz; Azpiroz, Arantza


    Individual variation in behavioral coping strategies to stress implies that animals may have a distinct physiological adaptation to stress; these differences may underlie differences in vulnerability to stress-related diseases. This study was designed to test the hypothesis that different behavioral coping strategies (active vs. passive) are stable over time and that they would be associated with differences in hypothalamic-pituitary-adrenal (HPA) and sympathetic-adreno-medular (SAM) axes, and monoaminergic and immune activity. Male mice were subjected to social stress. Twelve days after the first social interaction, mice were subjected to a second identical social stress interaction. Behavior was videotaped and assessed during both sessions. One hour after the final social interaction, serum was collected for corticosterone and adrenaline concentrations and brains were collected for hypothalamic corticotrophin-releasing hormone (CRH) mRNA expression. Monoaminergic system activity was determined by mRNA expression of serotonin, dopamine and noradrenaline synthetic enzymes in the brain stem. Immune system activity was determined by mRNA expression of hypothalamic interleukin-1β (IL-1β) and splenic IL-1β and interleukin-2 (IL-2). Mice engaging in a passive strategy had higher serum corticosterone and lower serum adrenaline concentrations than the active group. The passive group showed lower hypothalamic mRNA expression of IL-1β and CRH and lower splenic mRNA expression of IL-2 and IL-1β relative to mice in the active group. An active strategy was associated with higher expression of the dopaminergic synthetic enzyme, while a passive strategy was associated with decreased expression of the serotonergic synthetic enzyme. These findings indicate that individual coping strategies are stable over time and are related to differences in the physiological stress response and immune activity. PMID:21864582