Abstracts from 8th Congress of the European Federation of Internal Medicine / European Journal of Internal Medicine 20S (2009), S1–S283 and hypertrophic myocardiopathy in AA. 5. Renal and rectal biopsy are useful in the diagnosis of amyloidosis with cardiac affectation. 6. Admitance is carry out by nephrology (in secondary amyloidosis) and internal medicine (in primary amyloidosis).
P0257 CLINICAL MANIFESTATIONS AND PREDISPOSING DISEASES IN PATIENTS WITH SECONDARY AMILOIDOSIS. ANALYSIS OF 67 PATIENTS
Alejandro Junco García, Elpidio Calvo Manuel. Medicina Interna Introduction: Amyloidosis is a rare disease of unknown etiology, characterized by the deposition of amyloid in the extracellular spaces of various organs and tissues, and with an incidence of about 8 per million person per year. Objectives: 1.To review Systemic Amyloidosis cases (Primary amyloidosis: AL and Secondary amyloidosis: AA) admitted to our hospital over a period of twelve years. 2.To determine the most frequent clinical manifestations in patients with secondary amyloidosis. 3.To gain knowledge of predisposing diseases associated with secondary amyloidosis. 4.To analysis the medical services implied in this disease. Patients and methods: We performed a retrospective descriptive statistical study of 67 patients admitted to the Hospital Clínico San Carlos from January 1996 to December 2007 with a diagnosis of Systemic Amyloidosis. Data were collected on clinical manifestations and predisposing diseases in secondary amyloidosis. The diagnosis of secondary amyloidosis was established on positive biopsy for amyloidosis or negative biopsy but meeting the following inclusion criteria: to present infection or chronic inflammation and proteinuria, hepathomegaly or gastrointestinal disease of unknown origin. Outcomes: In systemic amyloidosis: 27 (40.3%) were AL and 40 (59.7%) AA. – Anthropometric data: Men: 41 (61.2%), women: 26 (38.8%).In AL: 19 (70.4%) were males and 8 (29.6%) females and in AA: 22 (55.0%) were males and 18 (45.0%) females. The median age of diagnosis was 67 years old (25-75 percentile: 54-75). The median age at diagnosis in AL was 73 years old (25-75 percentile: 61-81) and in AA was 62.5 years old (25-75 percentile: 43,2-73,7). – Predisposing diseases in patients with AA: Inflamathory bowel disease: 8 (20.0%); Rheumatoid arthritis: 6 (15.0%); Bronchiectasis: 4 (10.0%); TBC: 4 (10.0%); Ankylosing spondylitis B27 +: 4 (10.0%), Cutaneous fistula: 3 (7.5%), Xantogranulomatous chronic pyelonephritis: 3 (7.5%); Cancer: 2 (5.0%); Osteomyelitis: 2 (5,0%); prosthetic infection: 2 (5.0%) Seronegative spondylitis: 1 (2.5%), Chronic hidrosadenitis: 1 (2.5%), Familial mediterranean fever: 1 (2.5%); X-Histiocytosis: 1 (2.5%). – Clinical manifestations in patients with AA: General events: 13 (35%), renal manifestations: 33 (82.5%), cardiac manifestations: 18 (45.0%), digestive manifestations: 22 (55.0%), liver disorders: 13 (32.5%), splenomegaly: 4 (10.0%); dysautonomy: 7 (17.5%), peripheral nerve affectations: 8 (20.0%); endocrinology disorders: 2 (5.0%), pulmonary affectations: 1 (2.5%). – Distribution of patients in hospital servicies: Nephrology: 21 (52.5%); MIN: 11 (27.5%); Digestive: 3 (7.5%); Urology: 2 (5.0%); Rest of them: less than 3% each one. Conclusions: 1. Secondary amyloidosis is more common in men with a median age of 62.5 years old. 2. Inflammatory bowel disease and rheumatoid arthritis are the most frequent diseases. 3. The most frequent clinical manifestations are renal, gastrointestinal and cardiac. 4. Admitance is carry out by internal medicine and nephrology.
P0258 ACUTE THYROIDITIS – CASE REPORT
Catarina Graça Rodrigues, Marinela Félix Major, João Mascarenhas Araújo. Fernando Da Fonseca Hospital Acute thyroiditis is due to suppurative infection of the thyroid and it is rare. In adults it usually complicates a septicaemia. The patient presents with fever, chills and a painful and relatively enlarged thyroid, sometimes accompanied by erythema over the gland, respiratory distress and dysphagia. Inflammatory parameters are usually increased, but thyroid function is characteristically normal. It may be confounded with sub-acute thyroiditis, which is treated with NSAIDs or glucocorticoids, but the abrupt presentation of acute thyroiditis facilitates the differential diagnosis. The responsible organism should be identified. Antibiotic treatment must be started as soon as possible and surgery may be needed to drain an abscess. The authors present the case of a woman, aged 74, admitted at the hospital
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by lypothimia due to moderate anemia which was in study. Six days after the admission she developed a bilateral pneumonia. An empiric antibiotic treatment was promptly started but 48 hours later an asymmetric and very painful enlargement of the thyroid was found on physical examination and documented by ultrasound and CT scan of the neck, with a liquid collection in the right lobe of the gland extending to the soft tissues around. The blood cultures were negative and so a fine-needle aspiration biopsy of the lesion was performed, with isolation of Klebsiella pneumoniae sensitive to the antibiotic in course. No surgical drainage was performed because she died in septic shock in 24 hours. We discuss the seriousness of complications and the demand of a timely diagnosis.
P0259 ACUTE ISCHEMCIC STROKE IN YOUNG ADULTS – CASE REPORT
Catarina Graça Rodrigues, Marinela Félix Major, João Mascarenhas Araújo. Fernando Da Fonseca Hospital Stroke in young adults is not as uncommon as we might expect and atherosclerosis is responsible for an important percentage of cases (as in the elderly), in association with the high incidence of risk factors for atherosclerotic vascular disease in western countries. But a large etiologic spectrum should be always considered and the diagnosis is challenging. The authors present a case of a black woman aged 47 with no atherosclerosis risk factors known and no history of trauma or of drug abuse that was admitted at the hospital due to sudden-onset of dysarthria, labial commissural deviation and right hemiparesis. CT imaging of the brain revealed absence of intracranial haemorrhage. All complementary study was normal except the Doppler Echocardiography. The Transthoracic mode gave limited information about the atrial septum and so a Transesophageal exame was performed revealing a patent foramen oval and an aneurysm of the atrial septum, without evidences of thrombus. A deep venous trombosis was excluded by Doppler ultrasound of the members. She started anticoagulation with varfarine and three months later the foramen oval was closed by a percutaneous intervention without complications.
P0260 NUTRITIONAL RISK ASSESSMENT AND ATRIAL FIBRILLATION (AF)
Jesus Diez-Manglano. Internal Medicine Unit. Hospital Royo Villanova. Zaragoza. Spain Introduction: CONUT is a screening tool for CONtrolling NUTritional status for early detection of hospital undernutrition. It includes total cholesterol, serum albumin and total lymphocyte count. Objective: To know if CONUT predicts mortality in patients with AF admitted in an internal medicine unit. Methods: Retrospective analysis of records of patients admitted in a unit of internal medicine from January 1, through December 31, 2007 with AF as first or secondary discharge diagnosis. Only the first admission for each patient has been included. For each patient age, sex, previous AF, hypertension, diabetes, heart failure, total lympchocyte count, serum albumin, total cholesterol and mortality during admission were registered and CONUT index was calculated. Data were compared using X2 and Student tests. Results: From 2,073 admissions 392 (18.9%) had an AF diagnosis. CONUT could be calculated in 282 patients with AF diagnosis, 126 male and 156 female with mean age of 81±8 (38-96 years). 181 cases were known AF and 101 were new onset AF. Mean total cholesterol was 147±41 mg/dL, mean serum albumin 3.3±0.5 g/dL and mean total lymphocyte count 1182±685. During admission died 38 patients (13,5%). There were no differences in mortality by sex, previous AF, heart failure, hypertension, diabetes of previous stroke. Died patients had lower serum albumin 3.1±0.5 vs 3.4±0.5; p =0.0006) and CONUT Parameter Serum Albumin (g/dL) Score Total Lymphocytes/μL Score Cholesterol (mg/dL) Score Screening Total Score
Undernutrition Degree Normal
Light
Moderate
Severe
3.5-4.5 0 >1600 0 > 180 0 0-1
3.0-3.49 2 1200-1599 1 140-180 1 2-4
2.5-2.9 4 800-1199 2 100-139 2 5-8
< 2.5 6 < 800 3 < 100 3 9-12
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Abstracts from 8th Congress of the European Federation of Internal Medicine / European Journal of Internal Medicine 20S (2009), S1–S283
lower total lymphocyte count (878±518 vs 1230±697; p=0.003). Mortality was higher among patients with severe undernutrition grade. Conclusions: CONUT is useful for early detection of undernutrition and predicts mortality during admission for patients with AF. Keywords: Nutrition. Atrial fibrillation
P0261 CHOROID PLEXUS CARCINOMA – A CLINICAL CASE
Pedro Figueiredo, Corinna Lohmann, Henrique Santos. Hospital Garcia De Orta - Serviço De Medicina 1 - Almada/Portugal A 75 year old caucasian woman with a history of Sjogren Syndrome, osteoporosis and head trauma, was admitted for a two -month history of weight loss, dysarthria, dysphagia, convergent strabismus and walking incapacity. MRI and CT-scan of the head showed a mass involving the majority of the cerebellar surface, following the course of the fifth and seventh nerves towards the para-sellar regions and along the infundibulum. The Cerebrospinal fluid showed a predominance of small lymphocytes, without clonal characteristics, investigation for cerebral and pulmonary tuberculosis was negative. The patient had been started on antibacilar and dexamethasone therapy, needing ventilatory support because of progressive deterioration of mental and clinical status, with consequent death. Autopsy was performed, revealing macroscopically white nodules of soft consistency and irregular size, involving the cerebellar peduncles, the upper portion of the brain stem (exit of cranial nerves) and the sella turca/optic chiasm. Further macroscopic examination of solid organs showed no evidence of malignancy. The immunohistochemical and cytokeratin analysis, in consideration of the lack of evidence of solid organ malignancy, suggests Choroid Plexus carcinoma (CPC). The CPC is an uncommon, aggressive, malignant tumour of the central nervous system and is associated with a poor prognosis. The presentation of this case is illustrated by explicit images of the autopsy and the histological exams.
P0263 INFECTIOUS DISEASES IN A HOME CARE UNIT
Maria Jose Esquillor 1 , Jorge Gutierrez 1 , Aitziber Etxeberria 1 , Ruth Caballero 1 , Maria Teresa Rubio 1 , Catalina Isabel Gonzalez 1 , Valentina Fernandez 2 . 1 Hospital Reina Sofia; 2 Hospital De Navarra Background: Nowadays, with the development of Units of Care Home, some infectious diseases, what traditionally they needed treatment in the hospital, can be managed in patient’s house. Those patients with known and steady diseases, are taken care better at home, avoiding hospital secondary effects and improving welfare patient. Aims: The primary end point was to know the activity and the kind of infectious pathology in the unit of Care home of Hospital Reina Sofia de Tudela during a period of 26 months. Methods: Between November 2006 and January 2009, we have collected data on infectious diseases from all consecutively enrolled patients who have been seen after the introduction of Care Home in our Health Area. It is a descriptive study and we have pick up the following information: age; sex; number of days in the unit, who has decided the admission in the Unit, diagnostic, treatment received and destination Results: During this period of time, 751 patients have been admitted in the Unit 322 with infectious diseases (55% men) with an average age: 76.16 years. The average stay in the Unit was 8.25 days. With respect the main infectious diseases were: Respiratory infections 243 admittances (124 of them with pneumonia), urinary infection: 21, cellulites: 14, infected of ulcers: 12 incidents, diverticulitis: 7, unknown origin fever: 8, infection of biliar tract: 6, septic arthritis: 5 (3 of them infection of knee prosthesis), hepatic abscess: 3, one patient with oral candidiasis, one with herpetic encephalitis and one with maxillary sinusitis. As regard the final destination: 20 patients (6.2%) were admitted in the hospital because getting worse or a new process, 23 (7%) patients died and the rest were discharged. Conclusions: Pneumonias and respiratory infection disease, with and without COPD, represent the most frequent infectious pathology (75.5%) of admitted patients in the Unit. Morbi – mortality is similarly to other series, with a mean significantly lower average stay.
P0262 SKIN INVOLVEMENT IN SARCOIDOSIS
Pedro Figueiredo, Corinna Lohmann, Henrique Santos. Departamento De Medicina Interna - Serviço De Medicina 1, Hospital Garcia De Orta, Almada, Portugal Sarcoidosis is a multisystemic granulomatous disease of unknown etiology, appears in young adults (20-40 years), histologically characterized by the presence of non-caseating granulomas in the organs involved. The isolated skin involvement occurs in 1/3 of patients at the time of presentation, the involvement of other organs comes after several months. The lesions frequently present in the form of erythema nodosum (15-34%), usually associated with bilateral hilar adenopathy and polyarthralgia - Löfgren syndrome. The authors describe a case of a female patient, Caucasian race, 55-year-old clerk, married, no children, no smoking. No allergies. Personal background of: Dyslipidemia, Hypertention, polio in childhood (sequelae in the right lower limb). Medical consultation observed in about 3 years by myalgia and increase of transaminases, was discharged keeping with usual medication sinvastatin. It was sent by your family doctor for the consultation of the plastic surgery in my hospital with the diagnosis of synovial cyst. Excision of the tumor allowed for the right wrist on 04/04/2008, the piece was sent for histopathological examination, with the result of granulomatous dermatitis compatible with sarcoidosis. The patient was referred to the consultation of Internal Medicine for additional study and follow. Of tests: TAC chest with discrete granulomas dispersed. Broncho-alveolar lavage with bronchial lining cells, alveolar macrophages, neutrophils and lymphocytes. Bronchial biopsy, outbreaks of metaplasia decks and mild nonspecific chronic inflammatory infiltrate. With this event aims to present a rare form of cutaneous involvement of this disease, that the biopsy allows the diagnosis. The decision to treat the skin manifestations depend on the location and nature of injuries. When it requires an attitude therapy, corticosteroids are the drugs of choice. The presentation of this case is illustrated by explicit images of the histological exams.
P0264 DRUG RASH WITH EOSINOPHILIA AND SYSTEMIC SYMPTOMS SYNDROME INDUCED BY SULFASALAZINE
Gulistan Bahat 1 , Hulya Gamze Celik 2 , Bulent Saka 1 , Fatih Tufan 1 , Cemil Tascioglu 1 . 1 Department of Internal Medicine, Istanbul Medical Faculty, Istanbul University; 2 Institute of Cardiology, Istanbul University Introduction: Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a delayed idiosyncratic systemic hypersenstivity syndrome reported with the use of several drugs. It is a rare but serious reaction which may be cause visceral organ involvements. It is most commonly associated with anticonvulsants and antibacterial sulphonamides. Sulfasalazine is a drug commonly prescribed in rheumatology and gastroenterology practice. Besides its common dose related non-serious side effects, it causes a rare but sometimes fatal immunoallergic reaction named DRESS syndrome. Case: A 68 year old woman presented with oliguric acute renal failure, hypotension, fever and widespread maculopapular rash. She had a three year history of bilateral knee pain. She was given sulfasalazine (1 g/d) and prednisolone (5 mg/d) with a presumptive diagnosis of rheumatoid arthritis seven weeks prior to admission. One week before her presentation she developed acute tonsillopharyngitis with fever for which she was given peroral sulbactam-ampicillin. Three days later a maculopapular rash appeared on her back and spread upwards and downwards to the whole body. In follow-up, rash persisted despite withdrawal of ampicillin and salazopyrine, and she developed marked eosinophilia, atypical lymphocytosis, hypotension, acute renal failure and cytolytic hepatitis. Viral serology for CMV, EBV, HIV, HBV and HCV were negative. DRESS syndrome was considered and she responded very well to moderate dose of methyl prednisolone which was tapered and ceased at the end of fourth week. In the follow up she was free of symptoms and complete blood count, creatinine, liver enzymes and function tests and electrolytes were all within normal limits. Conclusion: We conclude that DRESS syndrome is a rare but serious complication of sulfasalazine treatment. The genetic susceptibility, viral infections or additional antibiotic use may be involved in its pathopysiology. Its treatment mainly consist of discontinuation of sulfasalazine and administration of glucocorticoid therapy. Increased awareness of this syndrome is essential among