[Tubular renal acidosis]. / Les acidoses tubulaires rénales.

Renal tubular acidosis (RTAs) are a group of metabolic disorders characterized by metabolic acidosis with normal plasma anion gap. There are three main forms of RTA: a proximal RTA called type II and a distal RTA (type I and IV). The RTA type II is a consequence of the inability of the proximal tubule to reabsorb bicarbonate. The distal RTA is associated with the inability to excrete the daily acid load and may be associated with hyperkalaemia (type IV) or hypokalemia (type I). The most common etiology of RTA type IV is the hypoaldosteronism. The RTAs can be complicated by nephrocalcinosis and obstructive nephrolithiasis. Alkalinization is the cornerstone of treatment.

[Oncogenic osteomalacia: increased production of fibroblast growth factor 23 is not the unique actor]. / Ostéomalacie oncogénique : le FGF 23 ne fait pas tout.

The importance of fibroblast growth factor 23 (FGF 23) has been highlighted in the mechanism of urinary leakage of phosphate in the oncogenic osteomalacia (OO). It is now a component of diagnosis of this disease. We report a 58-year-old man who presented with osteomalacia and hypophosphatemia secondary to urinary leakage of phosphorus. Although serum FGF 23 was normal, the diagnosis of OO was obtained after another cause of acquired prolonged hypophosphatemia has been excluded (hyperparathyroidism and Fanconi syndrome in particular). The search for a deep tumor was performed, allowing the detection of a 12 mm hemangiopericytoma in the upper thigh. Its removal allowed the rapid resolution of clinical symptoms and laboratory abnormalities. The importance of functional sequelae in OO depends on prompt diagnosis. Tumorectomy remains the optimal treatment. Thus, the search for a secreting tumor is essential even in the absence of elevated serum FGF 23.

[Management of cytomegalovirus infections in patients treated with immunosuppressive drugs for chronic inflammatory diseases]. / Prise en charge de l'infection à cytomégalovirus symptomatique chez les patients traités par immunosuppresseurs pour une maladie inflammatoire chronique.

INTRODUCTION: For patients with chronic inflammatory disease treated by immunosuppressive agents (for example: rheumatoid arthritis or systemic lupus erythematosus), there are no available guidelines in medical literature on the use of antiviral agents for the management of symptomatic cytomegalovirus (CMV) infection. EXEGESIS: A patient treated by methotrexate for a spondylarthritis presented a CMV infection manifested with persistent fever and pneumonia. CMV pp65 antigenemia was of 120 positive nuclei for 100,000 cells. Treatment with valganciclovir allowed a prompt recovery, while treatment by methotrexate was maintained. CONCLUSION: Symptomatic CMV infection evolution is unpredictable and potentially severe in patients with chronic inflammatory diseases receiving immunosuppressive agents. Although there is no data issued from clinical trials, the observation reported in this article and the publications of similar cases in the medical literature indicate that treatment with valganciclovir seems worth to be used in this context. Stopping immunosuppressive therapy does not seem mandatory.

[Hemoglobin targets in anemic patients with chronic kidney disease treated with erythropoietin]. / Erythropoïétine et insuffisance rénale: jusqu'où ne pas aller?

PURPOSE: Erythropoietin is the treatment of the anaemia in chronic kidney disease. A target rate of haemoglobin higher than 11 g/dl was usually proposed, but recent recommendations stated that higher limit of haemoglobin was to be reached, with the aim to improve the quality of life of the patients and to reduce their risks of cardiovascular diseases. These objectives are to be revised, according to the results of recently published clinical trials. CURRENT KNOWLEDGE AND KEY POINTS: Patients treated to reach a high rate of haemoglobin (between 13 and 14,5 g/dl) have an improved quality of life, but a 30% higher mortality rate, compared to patients treated with a lower objective of haemoglobin rate (10-12 g/dl). Hypertension and vascular access thromboses were also more frequent in the patients with the highest haemoglobin rate. Two to three times more erythropoietin was necessary to reach the higher rate of haemoglobin. These results favour a target rate of haemoglobin not higher than 12 g/dl. A polemic followed the results of these clinical trials, mostly in the United States, questioning the way in which the higher limit had been fixed whereas precise data were unavailable. The role of pharmaceutical industry and of for profit dialysis centres was underlined. FUTURE PROSPECTS AND PROJECTS: The next step is now to explain if the excess in cardiovascular morbimortality is related to the haemoglobin rate or to a direct effect of the erythropoietin. Such an understanding is important, the more so as new erythropoiesis-stimulating agents are being developed.

[Hypokalaemia and paralysis: think about the thyroid]. / Hypokaliémie et paralysie: penser à la thyroïde.

Periodic hypokalemic paralysis can be of genetic origin or secondary to other causes of hypokalaemia. The thyreotoxic hypokalemic periodic paralysis (THPP) usually occurs among asian subjects. It is a diagnostic and therapeutic emergency which may lead to life-threatening complications due to hypokalaemia and muscle weakness. The potassium supplementation is followed by a complete recovery after a few hours. We underlined the interest of thyroid assays in patients having an acute muscular paralysis associated with hypokalaemia.

[Systemic corticosteroid treatment and risk of infectious diseases]. / Corticothérapie systémique et risque infectieux.

PURPOSE: It was shown that corticosteroids alter the inflammatory and immune responses. Many publications report on serious infections occurring in patients receiving corticosteroids or presenting with Cushing's syndrome. This information is synthesized in this article. CURRENT KNOWLEDGE AND KEY POINTS: The demonstration of the infectious risk associated with corticosteroids relies on observational data and on biological plausibility. However, this risk remains difficult to quantify, because of many confusing factors such as the patients' associated conditions and immunosuppressive treatments, and the highly variable dose and duration of the corticosteroid treatment. Taking into account the published data, the screening for a chronic infection seems licit among patients receiving a systemic corticosteroid treatment, in particular for those who will receive more than 10 mg of prednisone per day. FUTURE PROSPECTS: Although no clinical trials of prevention of infections in corticosteroid treated patients has been published, a strategy aiming at minimizing the infectious risk of corticosteroid treated patients is proposed, based on the analysis of the literature presented in this article.

[Is there an evidence-based management of idiopathic retroperitoneal fibrosis?]. / Peut-on proposer une prise en charge de la fibrose rétropéritonéale idiopathique validée par les données de la littérature?

BACKGROUND: Nowadays it is quite easy to diagnose idiopathic retroperitoneal fibrosis (IRF), particularly with the help of medical imaging. However there is no guideline about the treatment. PURPOSE: Looking for data about an evidence-based management. METHODS: Screening of the database Medline. Titles and abstracts of articles published between 01/01/1985 and 31/12/2004 have been read to identify clinical trials and series about more than ten patients. RESULTS: No record of any therapeutic trials has been found. Eight series in total, which included 177 patients, were identified. Two of the patients have been treated by an ureteral desobstruction only (endoscopy or nephrostomy), 45 by surgery (ureterolysis), 65 by corticotherapy and 64 both by surgery and steroids. For 38 patients, immunosuppressive drugs were combined with corticotherapy (azathioprine, cyclophosphamide or D-penicillamine). According to the authors, doses and duration of corticotherapy varied. Median follow-up lasted 56 months. The outcome is satisfactory in 73% for surgery alone, 86% for medical treatment alone and 73% for both. The association between steroids therapy and immunosuppressive drugs is efficient in 97% of the cases. No clear data about side effects was mentioned. DISCUSSION: Treatment of the IRF is still empirical, based on surgery and corticotherapy. There is no guideline about the treatment strategy. Although tamoxifen has been proposed, efficacy evidence is lacking. Prospective multicenter studies will help us to progress in the management of the IRF.

[Use of nitrates in hospitalised elderly patients aged 65 and over]. / Utilisation des dérivés nitrés chez les patients âgés de 65 ans et plus hospitalisés en médecine interne.

BACKGROUND: Nitrates are frequently prescribed drugs, although their indications are limited. We studied nitrates' prescription in elderly patients hospitalised in the internal medicine ward of a French teaching hospital. METHODS: Hospitalised patients aged 65 years and more and receiving nitrates in their usual treatment were identified prospectively. A standardised questionnaire was used during a structured medical interview conducted by the same physician for all patients. Informations regarding nitrates' prescription were studied according to the actual recommendations for their use: angina in patients with contraindication to betablockers, acute myocardial infarction and acute pulmonary oedema. RESULTS: Among 256 hospitalised elderly patients, 49 (19% [IC95%: 15-25]) were under nitrates therapy, because of either angina pectoris, heart failure or unknown reason. Cardiologists prescribed nitrates in accordance with guidelines significantly more frequently than non-cardiologists. Transdermal treatment was used in 69% of patients. DISCUSSION: In hospitalised patients aged 65 years and more who are prescribed nitrates in their usual treatment, at least one quarter have no recommended indication for its use. As almost one fifth of patients are receiving nitrates in their usual treatment, the medicoeconomic impact of these useless prescriptions could be significant. Nitrates prescribing can be optimized by following guidelines for their use, and restraining from prescribing the transdermal treatment which is more costly and without evidence-based clinical benefit compared to the oral route.

Clinical significance of tolerant strains of streptococci in adults with infective endocarditis.

OBJECTIVES: To compare the characteristics of patients with endocarditis due to tolerant and non-tolerant Streptococcus strains. PATIENTS AND METHODS: A retrospective nine-year study was conducted in a single tertiary-care hospital. The study included 24 cases of streptococcal endocarditis with known beta-lactam minimal inhibitory and bactericidal concentrations. RESULTS: Ten of the 24 patients concerned were infected with tolerant streptococcal strains, and 14 with non-tolerant strains. Bacterial tolerance was not associated with higher mortality or increased frequency of surgery. Fewer patients infected with tolerant than non-tolerant strains had serum bactericidal titers reaching success-predictive levels, and more of these experienced failure of initial antibiotic treatment and needed longer treatment. CONCLUSIONS: The results of this study strongly suggest that penicillin tolerance of the streptococci responsible for endocarditis has a clinical impact. Consequently, pending a larger prospective study addressing the problem of tolerance, it is clinically relevant to determine the minimal inhibitory and bactericidal penicillin concentrations for all streptococcal isolates causing endocarditis.

A survey of physicians' vaccine risk perception and immunization practices for subjects with immunological diseases.

Various publications have caused concern by implying that immunization may be linked to new cases or flare-ups of immunological diseases (IDs). In view of the resulting uncertainty, we studied physicians' vaccine risk perception and immunization practices for adults with IDs. A questionnaire was mailed to three groups of physicians in France: internal medicine specialists, general practitioners, and travel clinic physicians. Thirteen vaccines currently used for adults in France were studied. Risk perception was rated on a 10 cm visual analog scale (VAS). The distribution of the answers was compared between and within groups of physicians. Potential associations between risk perception and reported practices were investigated by multivariate analysis. In the three groups of physicians (n=762), the tetanus and Salk poliomyelitis vaccines had the lowest risk perception. The yellow fever, BCG and Sabin poliomyelitis vaccines were the least well perceived. The distribution of risk perception for these three live vaccines and the hepatitis B vaccine was uniform according to VAS grading. For the other vaccines studied, the distribution was skewed to the low-risk perception side of the VAS. Risk perception was greater for physicians who stated: (1) that certain IDs carried a high risk of adverse events following immunization; (2) that they sought the advice of the referent physician before immunization; (3) warned their patients of the risk of an ID flare-up after vaccination; (4) sought information about recent immunization in patients with a flare-up; and (5) had experience of the side effects of immunization in adults with ID. Risk perception was lower for physicians who said they updated immunizations, and for the internists. The worse the vaccine risk perception by physicians, the more uniform the distribution of perception, thus reflecting the disagreement of the scientific community about the risk of using such vaccines for adults with an ID. Risk perception and immunization practices were related in adults with ID. Understanding of decisions concerning immunization may help to improve immunization updating and prevent risk amplification when evidence is lacking.

[Systemic lupus erythematosus and risk of hepatitis B vaccination: from level of evidence to prescription]. / Lupus érythémateux systémique et risque de la vaccination contre l'hépatite B: du niveau de preuve à la prescription.

INTRODUCTION: Case reports focusing on immunological diseases occurring subsequently to vaccination are often described in the literature. Reporting of such cases may influence physicians' perception of risks related to immunization, and thereby immunization practices. The decision to vaccinate a patient with an immunological disease should not rely on such case reports, but on the level of evidence of a causal relationship between vaccination and the occurrence of an adverse event. This article describes the search for available data supporting such causality before taking the decision to introduce vaccination against hepatitis B in a female patient with systemic lupus erythematosus (SLE). CURRENT KNOWLEDGE AND KEY POINTS: Data extracted from Medline and surveillance system showed that: 1) biologic plausibility of a relationship between the HBs antigen and SLE was unlikely; 2) case reports or case series were seldom and not convincing regarding potential causality; and 3) there were neither controlled observational studies nor controlled clinical trials. The only available clinical study was of poor quality and did not show any adverse event. The level of evidence of a causal relationship between vaccination against hepatitis B and the occurrence of an adverse event in patients with SLE was low, in-between levels 4 and 5 as defined by the Center for evidence-based medicine. The risk-benefit ratio may therefore rely on these results and guide the decision whether or not vaccination should be introduced. FUTURE PROSPECTS AND PROJECTS: The type of reasoning reported in this paper can be used for other vaccines or other immunological diseases, and have wider applicability in terms of therapeutic risk management when data and evaluation are lacking that could guide decisions.

Severe valvular heart disease in patients on chronic dialysis. A five-year multicenter French survey.

A retrospective multicenter survey of the 230 chronic dialysis centers in metropolitan France, conducted between January 1 1998 and December 31 1992, to assess the incidence, causes and features of severe valvular heart disease among chronic dialysis patients, identified 98 patients. The annual incidence was estimated to be 15 to 19 cases per 10,000 dialysed patients. The most common etiologies were calcific valvular disease (69%) and endocarditis (19%). Calcific valvular disease led mostly to aortic stenosis, whereas endocarditis primarily caused mitral insufficiency. Two valves were damaged in 32% of the endocarditis patients versus 9% of those with calcific valvular disease. Sixty-one patients underwent surgery. Median overall survival after surgery was 25 +/- 3.0 months. Patients who underwent surgery for calcific valvulopathy, aortic stenosis or only aortic valve replacement had a median survival of 36 months. Patients who underwent surgery for endocarditis or replacement of 2 valves had a median survival of < 12 months. Actuarial survival of surgical patients differed significantly between: i) the patients for whom presurgical evaluation showed a single valvular lesion and those with multiple valvular lesions (p = 0.002), ii) the patients who had surgery to replace a single heart valve and those who had another type of surgery (p = 0.001), and iii) the patients who had surgery to insert a single aortic prosthetic heart valve and those who had another type of surgery (p = 0.004). Multivariate analysis (including etiologies, number of valvular lesions and type of surgery) showed that survival was significantly dependent only on the number of severe valvular lesions (p = 0.002). Five patients with severe calcific aortic stenosis died before scheduled surgery could be performed. These data suggest that, for patients on chronic dialysis, calcific aortic stenosis is the most frequent form of severe valvular disease. Because aortic stenosis progresses rapidly in these patients and thus quickly leads to irreversible cardiac failure, the operative risk, although high in this population, seems acceptable when only one valve is affected.