Gastrointestinal disorders in primary immunodeficiencies

Gastrointestinal disorders in primary immunodeficiencies

Abstracts serious legal implications. Studies aimed to further define factors causing this bias are urgently required. References [1] Charlson ME, et ...

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Abstracts

serious legal implications. Studies aimed to further define factors causing this bias are urgently required. References [1] Charlson ME, et al. J Chronic Dis 1987;40:373–83.

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4. In a patient who is ‘nil by mouth’ must have a nasogastric tube for regular PD medications. Transdermal patch of rotigotine or subcutaneous apomorphine can also be used. 5. Although withdrawal of levodopa is still the most common cause of PHS, we should not forget other agents like dopamine agonists.

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ID: 115 Parkinsonism hyperpyrexia syndrome due to abrupt withdrawal of ropinirole A. Arora

ID: 124 SLE patient with respiratory symptoms — An unusual cause Y. Snir, D. Itskoviz, F. Salamon, D. Huminer, R. Tur-Kaspa

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Silicosis is an occupational lung disease, caused by inhalation of crystalline silica. The association between silicosis and several autoimmune diseases has been described, but only few reports in the literature are found regarding about the association of silicosis and systemic lupus erythematosus (SLE). In this article, we describe a 28 year old artificial stone worker, who was admitted to our department due to acute respiratory symptoms and fever that had been developed over a period of several days. Several months before his admission, he was diagnosed with SLE manifested as arthritis with positive relevant serologic markers. Investigation of our patient's symptoms has led to the diagnosis of accelerated silicosis, which explains his recent symptoms, as well as the former diagnosis of SLE. In this article we discuss the differential diagnosis and management decisions of this patient's symptoms at his first presentation, when we considered him as SLE patient, and afterwards. We also describe the known mechanisms that contribute to the immune-modifying effect of free silica.

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Introduction: The parkinsonism hyperpyrexia syndrome (PHS) is a rare but potentially fatal complication seen in Parkinson's disease (PD). It is characterised by mental status changes, muscle rigidity, hyperthermia and autonomic dysfunction. Mortality of up to 4% has been reported but an additional one-third of patients have permanent sequelae. Parkinsonism hyperpyrexia syndrome may be indistinguishable from neuroleptic malignant syndrome except that it occurs in patients with pre-existing parkinsonism and is often referred as neuroleptic malignant like syndrome (NMLS). This is potentially the first case of a dopamine agonist (ropinirole) withdrawal leading to PHS despite the patient taking levodopa and a mono-amine oxidase inhibitor. Case report: A 67 year old man with an 8 year history of PD was admitted with an episode of collapse at home. He had postural hypotension and nitrite positive urine dip. The labile blood pressure was attributed to his multiple anti-parkinsonian medications (4 mg ropinirole daily, 100/25 mg cocareldopa 5 times a day and 10 mg selagiline daily). During the admission his ropinirole was tapered off over 3 days. Four days post admission the patient was found in a ‘confused, rigid and hallucinating’ state with a temperature of 40.2 °C. He had increased tremor and stiffness, profuse sweating, tachypnoea, tachycardia and visual hallucinations. A diagnosis of “parkinsonism hyperpyrexia syndrome (PHS)” was made. He was cooled externally. A nasogastric (NG) tube was inserted and a dose of co-careldopa was given, ropinirole was restarted. The creatine kinase (CK) was 845 U/L (50–200 U/L) and urine showed blood on dipstick. The patient started recovering over the next few hours and his muscle tone and temperature returned to baseline. Discussion: “Parkinson hyperpyrexia syndrome” is a “neuroleptic malignant like syndrome” seen in Parkinson's disease. It is a rare (0.3% of PD patients/year) but a potentially lethal form characterised by mental status changes, muscle rigidity, hyperthermia and autonomic dysfunction. Parkinson hyperpyrexia syndrome is under-reported and the diagnosis remains clinical. Mild cases may be mislabelled as sepsis or worsening of parkinsonism. High fever is the most frequent clinical manifestation of PHS. Levenson et al. in 1985 suggested a definition for NMS using major minor criteria in an appropriate clinical setting and these criteria can also be helpful in diagnosing a neuroleptic malignant like syndrome condition i.e. PHS. Learning points:

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Acute Medicine, Frenchay Hospital, Bristol, United Kingdom

Department of Medicine D, Rabin Medical Center, Beilinson Campus, Petah Tiqwa, Israel

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ID: 128 Gastrointestinal disorders in primary immunodeficiencies S. Sánchez Trigo, T. Caínzos Romero, D. García Alén, C. Lijó Carballeda, I. Gómez Buela, R. Sardina Ferreiro, L. Vilariño Maneiro, H. Álvarez Díaz, A. Mariño Callejo, P. Sesma Sánchez

1. Any elevation of body temperature during the course of antiparkinsonian drug treatment should be considered as PHS until proven otherwise. 2. It is important to distinguish PHS from other potentially lethal and similar disease states such as neuroleptic malignant syndrome and serotonin syndrome. 3. Abrupt withdrawal, rapid switching, decrease in dose post deep brain stimulation (DBS) implant, non-adherence due to various factors can all trigger PHS.

Internal Medicine, Complexo Hospitalario Universitario de Ferrol (CHUF), Ferrol, Spain Introduction: Primary immunodeficiencies (PIs) are a group of diseases that are characterized by an increase in the risk of infections, with manifestations in different organs and systems. The gastrointestinal tract (GIT) is a major lymphoid organ of the body, which is frequently affected in patients with PIs. Gastrointestinal clinical manifestations such as chronic diarrhea, malabsorption, or the inflammatory bowel disease (IBD), as well as autoimmune disorders that affect the GIT (celiac disease or pernicious anemia), appear with relative frequency. Aim: In this paper, we present the case series of patients diagnosed with PIs. These patients were followed up by a specialized medical team at the Hospital Arquitecto Marcide in Ferrol (Galicia— Spain). Materials and method: This is a retrospective study that reviews the medical records of patients diagnosed with PIs. Consultations were followed up until April 30, 2013. Results: The consultation tracked 21 patients, 52.4% of whom were women. The mean age was 58.25 years old (range 34–76). Common variable immunodeficiency (CVID) was diagnosed in 76.2% of the patients IgG subclass deficiency in 9.5%, hyper-IgM syndrome in 9.5% and x-linked agammaglobulinemia in 4.8%. Recurrent respiratory infections appeared in 61.9% of the patients. Gastrointestinal disorders were diagnosed in 61.8% of the patients along the track: chronic

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Abstracts

diarrhea (28.6%), malabsorption and weight loss (23.8%), acute gastroenteritis (19.9%), sprue-like syndrome (19%), chronic atrophic gastritis (14.3%), pernicious anemia (14.3%) and IBD (4.8%). No statistically significant differences were found between the type of PI and gastrointestinal manifestations (p = 0.83). Liver ultrasound showed abnormalities in 33.3% of the patients: steatosis (14.3%), hepatomegaly (9.5%) and simple hepatic cyst (9.5%). Gastric adenocarcinoma was diagnosed in 9.5% of the patients (2 patients). Intravenous immunoglobulin replacement therapy is being used in 66.7% of the patients. Conclusion: PIs are often associated with gastrointestinal disorders. Among our patients, the most frequent was chronic diarrhea, followed by malabsorption and weight loss. Gastric neoplasms appear with relative frequency. A protocolled monitoring and active search for gastrointestinal disorders is, therefore, recommended for patients with PIs.

ID: 219 An acute presentation of tuberculous pericarditis: Diagnosis and management Z. Zielicka, L. Koizia, V. Luther, H. Burgess Accident and Emergency, West Middlesex University Hospital, London, United Kingdom

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Aims/objectives: Tuberculosis is an infectious disease caused by Mycobacterium tuberculosis, with pulmonary and extra-pulmonary manifestations; cardiovascular involvement is rare, estimated to occur in less than 8% of cases. Material and methods: A 27-year-old Indian man, with no known history of cardiac disease, presented with a 4-week history of left sided chest pain. He had associated shortness of breath, weight loss and fevers. His sister had recently been diagnosed with a tuberculous abscess, but the patient had declined participating in contact tracing. Results: Initial bloods showed a negative troponin, elevated CRP, positive Elispot and negative for HIV. The chest radiograph identified a new cardiomegaly. Electrocardiogram (ECG) demonstrated a microvoltage tachycardia and subsequent echocardiogram identified a large circumferential pericardial effusion with early signs of tamponade. The chest computed tomography (CT) identified associated mediastinal lymphadenopathy. The patient was transferred and underwent surgical pericardial drainage and window (approximately 900 mL). The patient was started on empirical quadruple anti-tuberculous therapy and a high dose oral steroid as per current guidelines. Pericardial fluid was culture negative, with cytology revealing lymphocytes. On follow-up, symptoms were resolving and subsequent chest radiograph demonstrated no evidence of cardiomegaly. Discussion and conclusion: Tuberculous pericarditis is extremely rare in western countries but may still present in migrant populations. It is a diagnostic and treatment challenge; with a high untreated mortality rate, extremely difficult to confirm a diagnosis and very little evidence based treatment.

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order to set up improvement guidelines. Material and methods: Observational, prospective, and descriptive study conducted between 17/05/2012 and 22/07/2012 in Internal Medicine Unit. We included all patients hospitalized in this unit during this period. Results: A total of 89 patients were included during this period. The mean age was 75.96 ± 14.73 years, with a gender distribution of 50 males and 38 females (56.18% males). The average consumption of drugs per patient during hospitalization was 12.69 ± 6.55 drugs. Analysis of drug consumption by Anatomical Therapeutic Chemical (ATC) classification showing the most used drug in each group and the total cost of the group was as followed: Group A: alimentary and metabolism: omeprazole, and total cost was 914.99 €; B: blood and blood forming organs: enoxaparin, and total cost was 1532.59€; C: cardiovascular system: furosemide, and total cost was 102.86€; D: dermatologicals: betamethasone, and total cost was 10.88€; G: genitourinary system and sex hormones: tamsulosin, and total cost was 3.84 €; H: systemic hormonal preparations, excl. sex hormones and insulins: methylprednisolone, and total cost was 194.98 €; L: antineoplastic and immunomodulating agents: ciclosporin, and total cost was 14.28 €; M: musculo-skeletal system: allopurinol, and total cost of the group was 5.43 €; N: nervous system: paracetamol, and total cost was 603,96 €; R: respiratory system: ipratropium bromide, and total was 555.50€; S: sensory organs: timolol, and total cost of the group was 7.32 €; V: various: polystyrene sulfonate, and the total cost was 30.96 €; and J: antiinfectives for systemic use: amoxicillin/ clavulanic, and the total cost was 11,240.53 €. The most used active ingredients were enoxaparin (66.29% of patients), omeprazole (61.8%), paracetamol (46.07%), furosemide (38.20%), and ipratropium bromide (30.34%). The therapeutic group with the most cost was Group J: antiinfectives for systemic use, being the most prescribed: amoxicillin/clavulanic (16.85% of patients), levofloxacin (13.48%), ceftriaxone (12.36%), meropenem (11.24%) and ertapenem (10.11%). The 61% of the cost of the group was due to linezolid (2867 €), daptomycin (2449 €) and ertapenem (1544 €). Discussion and conclusion: The highest costs of the study belongs to antiinfectives group, however the most used (amoxicillin/clavulanic) only represents 1.95% of the total cost of antiinfectives. Regarding the most common active ingredients, it is logical that enoxaparin ranked first since it is the therapy of choice for prevention of thromboembolic disease. The second active ingredient most used was omeprazole (61.8% of patients), maybe we should review the use of omeprazole because it may not be necessary in all patients who have been prescribed. Although it is true that in most cases omeprazole is not a new drug added during admission, it is often prescribed because it is the patient's usual medication.

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doi:10.1016/j.ejim.2013.08.616

ID: 262 Study of drug consumption in an internal medicine service A. Nieto Sáncheza, R. Manzano Lorenzob, V. Puebla Garcíab, M.L. Arias Fernándezb, N. Tobares Carrascoa, A. Fernández-Cruza a

Internal Medicine III, Hospital Clínico San Carlos, Madrid, Spain Pharmacy, Hospital Clínico San Carlos, Madrid, Spain

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Objectives: The aim of this study is to describe the drugs used in Internal Medicine Unit. Valuing the most used drugs and its costs in

doi:10.1016/j.ejim.2013.08.617

ID: 268 Overweight, obesity and other cardiovascular risk factors at a university outpatient clinic in northern Brazil T.R. Couto, R.B. Veras, M.R.M. Cavalléro, T.I. Couto Medical Specialties Clinic of Pará State University Center, Pará State University Center, Belém, Brazil Background: The prevalence of overweight and obesity has increased globally, becoming the major health problem in recent society. When compared to eutrophic individuals, overweight and obese people have greater risk of developing cardiovascular diseases (CVD). Methods and Results: Cross-sectional retrospective study, conducted through analysis of 210 medical records (72.9% women and 27.1% men) of outpatients aged 18 to 60 years, assisted at Pará State University Center Medical Specialties Clinic between February