An Unusual Amyloid Goiter in a 48-Year-Old Woman with Rheumatoid Arthritis, Secondary Amyloidosis and Renal Failure.

Amyloid goiter (AG) is characterized by the presence of deposits of amyloid protein in the thyroid tissue in sufficient amounts to produce enlargement of the gland, accompanied by fat deposition or thyrolipomatosis. It can be seen in long-standing inflammatory disorders, with the common characteristic of amyloidotic renal failure. In daily practice, practitioners should pay attention to the differential diagnosis in patients with suggestive co-morbidities for amyloidosis. The clinic is a progressive increase of the thyroid gland with compressive symptomatology (dyspnea, dysphagia, and changes in the voice). The main imaging finding is diffuse fatty infiltration of the thyroid. The amyloid goitre was most probably in the general context of amyloidosis, regardless of the other complications. We present a case of a 48-years-old female with amyloid goiter secondary to rheumatoid arthritis and renal failure.

[Seip-Lawrence syndrome associated with polyostotic fibrous dysplasia. Report of a case]. / Síndrome de Seip-Lawrence asociado a displasia fibrosa poliostótica. A propósito de un caso.

We report the case of a 28-year-old woman attending for hirsutism and diabetes mellitus. Diabetes was a casual finding 2 years before consulting and was treated with diet and antidiabetic drugs. Acromegalic appearance, facial acne, penty, curled and rude hair, hypertrichosis, ade I diffuse goitre, prominent abdomen with umbilical hernia, severe hepatomegaly, prominent muscles and veins with normal genitalia appeared in the physical examination. No other abnormalities were found. Hypophysis, thyroid, suprarenal and ovaric hormonal functional studies were normal. An insulin-resistant diabetes mellitus was found in the metabolic study. Ultrasound and TAC showed severe diffuse hepatomegaly and visceral fat lack. Bone radiographies showed diffuse lesions compatible with polyostotic dysplasia. Subcutaneous, hepatic and bone biopsy revealed lack of fat tissue, hepatic steatosis and osteal fibrosis. Patient s diagnosis was Berardinelli-Seip syndrome, Seip-Lawrence or lipoatrophic diabetes associated with polyostotic fibrotic dysplasia. Case is studies and bibliographic references are reviewed.

[Adrenal mass and adrenal insufficiency]. / Masa suprarrenal e insuficiencia suprarrenal.

Primary adrenal insufficiency is a non frequent disease, that is declared in young adults and in the most of the cases is produced from an autoimmune mechanism or a tuberculous disease. The incidence of these forms in the different geographic areas is dependent of degree of irradication of the tuberculosis. We report the case of a patient with latent chronic adrenal insufficiency of tuberculous origin who was affected for an addisonian crisis during an intercurrent infectious disease, which permitted the diagnosis of the addisonian crisis, and Mal of Pott was moreover detected. Evolution with corticosteroid and specific treatment was very favorable.

[Acute acalculous cholecystitis complicating the presentation of cardiac tamponade as the initial manifestation of a pulmonary carcinoma]. / Colecistitis aguda alitiásica complicando la presentación de un taponamiento cardiaco, como manifestación inicial de un carcinoma pulmonar.

The finding of a cardiac tamponade (CT) as initial manifestation of lung cancer is rare, being its most frequent manifestations dyspnea, cough and edemas. The presence of alithiasic acute cholecystitis (AAC) as early manifestation of CT is extremely rare, despite this having being described related to other situations of low cardiac output. We present the case of a patient who underwent emergency surgery due to AAC as a form of presentation of CT, this being the initial manifestation of a pulmonary adenocarcinoma. The histopathological study of the liver and the vesicle were compatible with signs of short evolution venous stasis, and the diagnosis was established through pericardium biopsy and thoracic CAT.

[The assessment of the nutritional status of patients with familial amyloid neuropathy type I (Corino Andrade's disease) who are candidates for orthotopic liver transplantation]. / Valoración del estado nutricional de los pacientes con polineuropatía amiloidótica familiar tipo I (enfermedad de Corino Andrade) candidatos a trasplante ortotópico de hígado.

Corino Andrade's disease (Type I Family amyloidotic polyneuropathy) (FAP I) is a slow-evolving hereditary amyloidosis affecting, among other things, the digestive system, with the appearance of an amyloid deposit which produces a malabsorptive syndrome with diarrhea in those affected. At present, the only effective therapeutic option is orthotopic liver transplant (OLT): clearance from the hospital's transplant commission as a candidate requires, among other things, a prior nutritional study. We therefore proposed to carry out a nutritional assessment of these patients, comparing them with a group of terminal liver patients who are OLT candidates. The PAF I group showed a high level of calorie energy malnutrition (86%) and, to a lesser extent, visceral and protein malnutrition. The liver patient group showed 67% of visceral malnutrition and lower levels of calorific energy and protein malnutrition. All the OLT candidates showed a high rate of nutritional deterioration. The greater presence of calorific energy malnutrition in the PAF I patients might be due to the neuropathic gastro-intestinal condition. The high level of malnutrition encountered suggest the need for pre-transplant nutritional intervention designed to minimise post-surgical risk.

[The assessment of the nutritional status of patients in the terminal stage of liver disease who are candidates for orthotopic liver transplantation]. / Valoración del estado nutricional de los pacientes en estadio terminal de enfermedad hepática, candidatos a trasplante ortotópico de hígado.

Orthotopic liver transplant (OLT) is nowadays an accepted procedure for the treatment of patients with end-stage liver disease. Because of the significant state of malnutrition of such patients, we decided to evaluate the nutritional condition of patients accepted by the hospital's Transplants Committee as candidates for this type of therapy, with the eventual aim of discovering the types of malnutrition in the different pathologies, and which were the most frequent. The sample studied showed a significant prevalence of malnutrition. Patients with viral hepatitis showed only visceral type malnutrition, which affected 100% of them. The ethanolic cirrhosis group presented all types of malnutrition-energy-calorific (35%), protein (24%) and visceral (53%), while the group of other liver diseases presented 15% of energy-calorific malnutrition and 85% visceral. From these results, we conclude that the sample studied has a high prevalence of malnutrition and that the importance of its detection makes it possible to reduce pre- and post-surgical morbidity and mortality rates with an appropriate nutritional support.

[Disorders secondary to iodine deficiency in the autonomous community of the Murcia area]. / Trastornos derivados de la deficiencia de yodo en la comunidad autónoma de la región de Murcia.

Hypothesis. To know goiter prevalence, iodine excretion and disorders associated to iodine deficiency in the region of Murcia, with the aim to define the need for establishing iodine prophylaxis programs. Method. Transversal study on 1956 pupils of the region of Murcia, with an age between four and seventeen years old, stratified by sex, age and area of origin, evaluating thyroid size, grade, weight, height, school performance and problems, menarche age, fish and iodine salt intake and iodine excretion. Results. Global prevalence of goiter was 29%, we found a mean ioduria of 93.92 +/- 56.83 micrograms/l. Anthropometric mean values for each age group did not differ between pupils affected by the lack of iodine and the others. A lower school performance was found in pupils with iodine deficit. Fish intake did not play a role in goiter prevalence. Conclusions. Murcia is an area affected by disorders derived from mild iodine deficiency, which implies the need to establish a continuous plan of effective prophylaxis with iodine salt.

[Nutritional parameters and morbimortality in clinical hospitalization]. / Parámetros nutricionales y morbimortalidad en hospitalización clínica.

The prevalence of malnutrition in patients admitted to hospital in the region of Northwest Murcia and its repercussion on the morbidity rate and length of admission are studied. A protocol of nutritional evaluation according to the computer programme EDECAN-MED was applied, and the corresponding statistical treatment of data and results was done. We find that anthropometric data lack predictive value and that deterioration in the values for albumin, basal energy expenditure and nutritional prognostic index are associated with increased morbidity/mortality and prolongation of the length of hospital stay. The most valuable predictive datum of the length of hospital stay was the nutritional prognostic index, in spite of it being an index of surgical risk.

[Anthropometric study of the school population of the autonomic community of Murcia]. / Estudio antropométrico de la poblacíon escolar de la Comunidad Autónoma de Murcia.

BACKGROUND: The aim of this study was to know the anthropometric study of school children in Murcia in order to obtain parameters of present reference for our country and region. METHODS: A transversal study of the school population of the community of Murcia at Preschool, EGB, BUP and Technical School levels was carried out including 1,930 children between the ages of 4 and 17. These children were selected from a total of 242,596 proportionally chosen with respect to demography of each of the twelve regions in the area with weight, height and bicipital, tricipital, subscapular and abdominal skin folds being measured. RESULTS: The values obtained and classified by age, sex and regions of the autonomic community of Murcia exposed in tables, demonstrate the differences existing in all the parameters measured with those routinely used at an international and national level. CONCLUSIONS: The differences found clearly justify the need to compare the regional antropometric alterations with the "reference parameters" obtained in Spain and Murcia for which other studies concerning the same would be advisable.