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BACKGROUND: Involuntary weight loss frequently poses a diagnostic challenge. Patient and physician alike want to exclude malignant and other major organic illness. The present study aimed to evaluate whether a negative baseline evaluation (consisting of clinical examination, standard laboratory examination, chest X-ray, and abdominal ultrasound) lowers the probability of evolving organic illness in patients with significant unexplained weight loss. METHODS: Prospective observational study of 101 consecutive patients presenting to a general internal medicine department of a university hospital with an unexplained unintentional weight loss of at least 5% within 6-12 months. Laboratory tests of interest included C-reactive protein, albumin, haemoglobin, and liver function tests. RESULTS: Weight loss of the 101 patients [age (mean, interquartile range): 64 (51-71) years, 46% male] averaged 10 (7-15) kg. Organic causes were found in 57 patients (56%), including malignancy in 22 (22%). In 44 patients without obvious organic cause for the weight loss (44%), a psychiatric disorder was implicated in 16 (16%) and no cause was established in 28 (28%), despite vigorous effort and follow-up of at least 6 months. Baseline evaluation was entirely normal in none of the 22 patients (0%) with malignancy, in 2 of the 35 (5.7%) with non-malignant organic disease, and in 23 of the 44 (52%) without physical diagnosis. Additional testing, oftentimes extensive, after a normal baseline evaluation led to one additional physical diagnosis (lactose intolerance). CONCLUSION: In patients presenting with substantial unintentional weight loss, major organic and especially malignant diseases seem highly unlikely when a baseline evaluation is completely normal. In this setting, a watchful waiting approach may be preferable to undirected and invasive testing.

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Recurrent fever of unknown origin is mostly caused by rather rare diseases and many cases remain unexplained. The very limited literature data do not allow one to construct a diagnostic algorithm. A number of general principles should be kept in mind before starting the investigation for this rare subtype of fever of unknown origin.

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BACKGROUND: We present a case of severe neurological symptoms caused by hyperammonemia, secondary to a urinary tract infection with urea-splitting bacteria. INVESTIGATIONS: Blood analysis, urinalysis, urine culture, abdominal ultrasonography, cystography, CT. DIAGNOSIS: Hyperammonemia as a result of urinary tract infection with urea-spliting bacteria. MANAGEMENT: Desobstruction of the urinary tract and bladder or pouch rinsing, antibiotics, reduction of the dietary and endogenous nitrogen load, and endogenous nitrogen breakdown. Identification, prevention and treatment of underlying causes.

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Systemic inflammatory diseases represent a large group of rare diseases that may involve all organs and also the heart. The three layers of the heart can be affected and some manifestations such as pericarditis in systemic lupus erythematosus are rather well known, yet others are not known even for cardiologists. Modern sophisticated imaging techniques reveal cardiac abnormalities in most of these diseases and the reported frequency of cardiac involvement ranges widely depending upon the applied diagnostic methods and selection of patients. The clinical significance of these findings is not always established. However, better knowledge and awareness of cardiac involvement is necessary because it conveys a major risk for mortality in several of these rare diseases.

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BACKGROUND: C-reactive protein (CRP) is a widely used inflammatory marker. Yet, the clinical significance and outcome of extremely elevated CRP levels are poorly characterized. METHODS: We collected all patients seen at a university hospital in 2004 with at least one CRP level above 500 mg/l and retrospectively analyzed their electronic files, focusing on patient characteristics, clinical diagnosis, microbiology and vital outcome. RESULTS: CRP was above 500 mg/l in 130 patients with a median age of 62 years. Patient characteristics, settings, etiologies of inflammation, comorbidities and microbiology varied widely. Infections, mainly bacterial, accounted for 88% of episodes. Outcome was fatal in 36% of all patients and in 61% of patients with active malignancies. CONCLUSION: A wide variety of infections, especially bacterial, that are generally readily identified account for the majority of instances of extreme CRP elevation. Mortality is high, certainly in oncological patients.

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C1-esterase inhibitor deficiency is a rare yet classic medical cause of acute abdominal pain mimicking a surgical emergency. A hereditary form and a very rare acquired form of the disease exist, and both give rise to a similar clinical syndrome despite a different pathogenic mechanism. We describe a typical case of acquired C1-esterase inhibitor deficiency in a 65-year-old woman presenting with recurrent acute abdomen and ascites who had undergone two negative surgical interventions before diagnosis was eventually established. Both the diagnostic and therapeutic approach to this rare condition should be known by emergency physicians for two reasons: (1) it may present as an acute abdominal emergency resulting in unnecessary surgical intervention; and (2) it may cause life-threatening upper airway obstruction as a result of laryngeal oedema.

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