Dutch guideline for the management of electrolyte disorders--2012 revision.

Electrolyte disorders are common and often challenging in terms of differential diagnosis and appropriate treatment. To facilitate this, the first Dutch guideline was developed in 2005, which focused on hypernatraemia, hyponatraemia, hyperkalaemia, and hypokalaemia. This guideline was recently revised. Here, we summarise the key points of the revised guideline, including the major complications of each electrolyte disorder, differential diagnosis and recommended treatment. In addition to summarising the guideline, the aim of this review is also to provide a practical guide for the clinician and to harmonise the management of these disorders based on available evidence and physiological principles.

Renal abnormalities in a family with Alagille syndrome.

Alagille syndrome is largely unknown to the general internist because the diagnosis is usually made by a paediatrician. Nevertheless, it is important to be aware of this syndrome because it sometimes manifests later in life with a great variability in clinical presentation and important consequences for the individual patient. We therefore discuss this syndrome using a patient with the usual characteristics of this syndrome.

Antiproteinase 3- and antimyeloperoxidase-associated vasculitis.

Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Wegener's granulomatosis, microscopic polyangiitis, and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCAs) directed against either proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO). This has led some investigators to prefer combining these diseases under the common heading of ANCA-associated vasculitides. However, it is increasingly recognized that there are characteristic differences between patients with anti-PR3 and those with anti-MPO-associated vasculitis. This review focuses on the clinical, histopathologic, and possibly pathophysiologic differences between anti-PR3- and anti-MPO-associated vasculitis. Although there is considerable overlap, the anti-PR3- and anti-MPO-associated vasculitides are each characterized by particular clinical and histopathological findings. Extrarenal organ manifestations and respiratory tract granulomas occur more frequently in patients with anti-PR3 than in those with anti-MPO. Anti-PR3-positive patients with NCGN generally have a more dramatic deterioration of their renal function compared with anti-MPO-positive patients. The term "ANCA-associated vasculitis" is considered as a useful concept in the presence of systemic vasculitis. Likewise, in the presence of vasculitis, the terms "anti-PR3-associated vasculitis" and "anti-MPO-associated vasculitis" are useful concepts.

[Myositis in the presence of slightly elevated creatine kinase values]. / Myositis bij een normale of licht verhoogde creatinekinasewaarde.

The clinical picture with, among other things, muscular swelling, fever and nocturnal sweating in three males aged 30, 37 and 52 years, suggested a neoplasm and sepsis. Ultimately, they were found to suffer from focal myositis, localized nodular myositis and polymyositis, respectively. The ESR and leukocyte counts were increased, the serum creatinine kinase (CK) activity was normal or slightly increased. The symptoms decreased after prednisone treatment. Both localized and generalized inflammatory muscular diseases may be present without the serum CK activity being raised.

The hemodynamic effect of different ultrafiltration rates in patients with cardiac failure and patients without cardiac failure: comparison between isolated ultrafiltration and ultrafiltration with dialysis.

OBJECTIVE: The increasing number of dialysis patients with cardiovascular diseases will lead to an increase in the incidence of intradialytic hypotension. Intradialytic hypotension is determined by changes in plasma volume, changes in vascular reactivity and structural cardiovascular changes. In this study the effect of two different ultrafiltration rates (UF-rate), i. e. 500 and 1000 ml/h, on plasma volume, extracellular volume and arterial blood pressure was studied during different treatments of 2 hours combined ultrafiltration + hemodialysis (UF+HD) and 2 hours isolated ultrafiltration (i-UF). PATIENTS AND METHODS: 15 Patients, 8 patients with cardiac failure, CFpts (NYHA classification III and IV) and 7 patients without cardiac failure (NCFpts) were investigated during a standardized dialysis treatment. RESULTS: The decrease in plasma volume and decrease in extracellular volume was comparable both between i-UF and UF+HD and comparable between CFpts and NCFpts and was only dependent on the UF-rate. i-UF resulted in minor blood pressure changes in both CFpts and NCFpts. In CFpts UF+HD resulted in a significant decrease in systolic blood pressure (SBP) at both UF-rates while in NCFpts SBP decreased significantly only at the higher UF-rate during UF-HD. Although there were no significant differences in hemodynamic stability during the different treatment modalities between CFpts and NCFpts, the decrease in SBP in CFpts at the higher UF-rate during UF+HD was much more pronounced. CONCLUSION: From this clinical study we conclude that differences in hemodynamic stability between i-UF and UF+HD and between CFpts and NCFpts are not related to differences in plasma volume preservation. Other factors like different changes in vascular reactivity and in CFpts structural cardiovascular changes might be responsible for the observed differences.

Acquired angio-oedema caused by IgA paraprotein.

The syndrome of acquired angio-oedema is characterized by late onset of recurrent bouts of angio-oedema or abdominal pain and may be caused by an acquired deficiency of C1-inhibitor (C1-INH), the inhibitor of the first component of complement. Acquired C1-INH deficiency has been described in approximately 50 patients and is strongly associated with malignant B-cell proliferations. We describe a patient with an 8-year history of recurrent abdominal symptoms and angio-oedema with acquired C1-INH deficiency, caused by the presence of IgA-kappa antibodies that inactivate C1-INH. Analysis of the bone marrow revealed an IgA-kappa monoclonal population of plasma cells, without evidence of overt myeloma. Angio-oedema caused by an autoantibody of the IgA isotype is extremely rare and has never been described in a Dutch patient. Recognition of angio-oedema, both hereditary and acquired, is important because of the therapeutic consequences, as will be discussed.

Protection against cardiovascular collapse in an alcoholic patient with thiamine deficiency by concomitant alcoholic ketoacidosis.

Hyperlactataemia due to thiamine deficiency has so far only been reported in the setting of full-blown cardiovascular beriberi with congestive heart failure and systemic vasodilatation. Poor tissue oxygenation and impaired lactate clearance by the liver are generally accepted as underlying causes of the elevated lactate levels. We present an alcoholic patient with thiamine deficiency-induced hyperlactataemia and accompanying alcoholic ketoacidosis, who did not display the circulatory disturbances that are characteristic of cardiovascular beriberi. The hypothesis will be presented that the concomitant presence of alcoholic ketoacidosis has prevented haemodynamic deterioration. Putative mechanisms that could explain such an effect are discussed in detail, with special reference to the role of acetyl-CoA and adenosine.

Respiratory failure in ANCA-associated vasculitis.

OBJECTIVE: To assess the prevalence, clinical manifestations, and course of respiratory failure in all patients who tested positive for antineutrophil cytoplasmic autoantibodies (ANCA) in our clinics in the period between January 1985 and January 1993. DESIGN: Case-series analysis. SETTING: Three teaching hospitals in the Netherlands. PATIENTS: Two hundred twenty consecutive patients suspected of having vasculitis and/or glomerulonephritis who tested positive for ANCA by indirect immunofluorescence and enzyme-linked immunosorbent assay. RESULTS: Sixty-two patients had pulmonary involvement. Acute respiratory failure developed in nine. Respiratory failure was related to infections in two of them and to ANCA-associated vasculitis in seven. These seven patients uniformly presented with pulmonary hemorrhage and diffuse pulmonary infiltrates. The diagnosis of systemic vasculitis was supported by the presence of a pulmonary-renal syndrome in all patients, and by detection of antibodies to the proteinase 3 or myeloperoxidase antigen in all but one patient. Antiglomerular basement membrane antibodies were absent. The mortality was high due to hypoxic respiratory failure, pulmonary superinfections, and concomitant renal failure. CONCLUSIONS: Acute respiratory failure due to vasculitis developed in one of every nine patients with ANCA-associated pulmonary disease. Patients usually present with pulmonary infiltrates and hemoptysis. A diagnosis of vasculitis may be further supported by analysis of the urinary sediment and determination of the ANCA target antigen. It remains to be proved that early detection of ANCA favorably affects the outcome.

Hydralazine-induced constrictive pericarditis.

A 59-year-old man was diagnosed as having constrictive pericarditis 17 months after a typical hydralazine-induced autoimmune syndrome. This late complication of hydralazine has been reported only once. Ten years later the patient was found to have anti-neutrophil cytoplasmic antibodies directed against myeloperoxidase.

Acute pernicious or fulminating beriberi heart disease. A report of 6 patients.

OBJECTIVE: To investigate the incidence and presentation of acute pernicious or fulminating beriberi in a general district hospital. METHODS: All patients with a diagnosis of acute pernicious or fulminating beriberi heart disease made between 1978 and 1994 were identified, and their medical records were retrospectively examined. RESULTS: Six cases of acute pernicious or fulminating beriberi heart disease were recognized. The disease was characterized by circulatory shock and peripheral cyanosis. All patients had severe lactic acidosis, in 5 of them without hypoxaemia. Four patients were alcoholics. The most important diagnostic criterion was the impressive improvement after thiamine administration. CONCLUSIONS: Acute pernicious or fulminating beriberi heart disease can be recognized occasionally. Thiamine should be administered as soon as possible in suspected cases.

Differences between anti-myeloperoxidase- and anti-proteinase 3-associated renal disease.

We performed a retrospective study of the clinical features, the pattern of the pre-treatment renal function loss, the renal morphology and the outcome in 92 patients with anti-neutrophil cytoplasmic autoantibodies directed against proteinase 3 (aPR3; N = 46) or myeloperoxidase (aMPO; N = 46). Patients with aMPO had a higher median age than patients with a PR3 (63 and 56 years; P < 0.05). The mean (+/- SD) number of affected organs in the aPR3 group exceeded that of the aMPO group (3.9 +/- 1.4 and 2.2 +/- 1.1; P < 0.01). The prevalence of renal involvement did not differ between patients with aPR3 and aMPO (83% and 67%, respectively; NS). Pre-treatment renal function deteriorated significantly faster in aPR3- than in aMPO-associated renal disease. The kidney biopsies from patients with aPR3 showed a higher activity index (10.2 +/- 3.8 and 7.3 +/- 3.2; P < 0.03) and a lower chronicity index (4.5 +/- 2.6 and 7.0 +/- 3.1; P < 0.02) than biopsies from patients with aMPO. The kidney survival at two years was 73% in patients with aPR3- and 61% in patients with aMPO-associated renal disease (NS). We conclude that renal function generally deteriorates faster in aPR3- than in aMPO-associated renal disease. This goes together with more active renal lesions in patients with aPR3 and more chronic renal lesions in patients with aMPO. Despite these differences, there is no difference in outcomes between both antibody groups.

The inflammatory abdominal aortic aneurysm. Prevalence, clinical features and diagnostic evaluation.

Eight patients with an inflammatory abdominal aortic aneurysm are described. They constituted 3.1% of all aortic aneurysms operated in the period 1981-1990. All presented with abdominal complaints and/or backache, and an elevated erythrocyte sedimentation rate (ESR); 3 had significant weight loss. The discovery of the aneurysm was considered a non-related finding except for 2 patients for whom the correct diagnosis was suggested by CT scan. Except for the aortic dilatation, the clinical and histopathological features of the inflammatory aneurysm resembled those of idiopathic retroperitoneal fibrosis with obstruction of the ureters and involvement of the duodenum by extensive perianeurysmal inflammation with infiltrates of mononuclear leukocytes and varying degrees of fibrosis. Although the triad of abdominal complaints and/or backache, an elevated ESR and weight loss has been proposed to suggest the diagnosis, the positive predictive value of these parameters was at best 22%. Additional studies, including CT scan, have to be performed to exclude, among other factors, vasculitis. Treatment should aim at repair of the aneurysm. Resolution of the inflammatory process after operation is frequently observed. Immunosuppressive therapy should only be used with caution, considering the possible increased risk of rupture.

Acute renal failure in theophylline poisoning.

A 57-yr-old woman with an intentional theophylline overdose complicated by rhabdomyolysis, renal failure and microangiopathic haemolytic anaemia is reported. Severe renal vasoconstriction, myoglobinuria and antagonism of adenosine are considered to be the principal pathophysiological renal mechanisms involved.

Clinical features and outcome in patients with glomerulonephritis and antineutrophil cytoplasmic autoantibodies.

We investigated the clinical features and outcome of 60 patients with antineutrophil cytoplasmic autoantibodies with cytoplasmic staining (C-ANCA)- and with (peri)nuclear staining (P-ANCA)-associated glomerulonephritis. Virtually all patients with C-ANCA had antibodies against proteinase 3, which corresponded with a clinical and/or histological diagnosis of Wegener's granulomatosis (WG). P-ANCA were associated with antibodies against myeloperoxidase. Although more often associated with renal-limited disease, a significant number of patients with P-ANCA had also symptoms of organs known to be preferentially affected with WG. The majority of patients presented with rapidly progressive glomerulonephritis which responded favorably to immunosuppressive therapy, albeit at the expense of a considerable number of fatal infections. Furthermore, a more protracted form of glomerulonephritis was recognized. A kidney biopsy scoring system appeared to be of value in predicting which patients would benefit most from treatment. C-ANCA and P-ANCA appear to identify a subset of patients with vasculitis that share common clinical features and a favorable response to immunosuppressive therapy.