[Post-acute and rehabilitation care in young patients with multiple comorbidities: An observational study]. / Soins de suite et réadaptation (SSR) de Médecine Interne pour sujets jeunes polypathologiques : étude observationnelle d'une structure pilote.

INTRODUCTION: A unique structure devoted to post-acute and rehabilitation care for patients under 75 with multiple comorbidities has been created within the Department of Internal Medicine, Bichat Hospital, Paris. We aim to report on demographic factors, clinical characteristics and outcomes of patients hospitalized in this pilot structure. METHODS: All consecutive adult patients admitted between May 2017 and May 2018 were retrospectively reviewed. RESULTS: Analysis was performed on 61 (61 [24-75] years-old) admitted patients. The median length of hospital stays was 108 [13-974] days. At admission, the median Charlson comorbidity index was 6 [0-12] predicting a 10-year survival of 21 [0-99]%. Most patients were unemployed (83.6%) and had very low-income (< national minimum wage in 65.6% of cases). At hospital discharge, most patients (85.4%) were able to return home. The complete resolution of health problems occurred in most cases (65.6%) and was associated with a lower probability of both hospital readmission and death 1-year after discharge. CONCLUSION: The structure served a high percentage of patients with major and complex health needs but limited access to care due to individual disabilities, low-income and underinsured status. However, despite major health disorders, functional limitations, and vulnerability, admission improved patient outcomes and reduced excess hospital readmissions in most cases.

Soluble CD163 is a biomarker for accelerated atherosclerosis in systemic lupus erythematosus patients at apparent low risk for cardiovascular disease.

Objective: This study aimed to determine whether sCD163, a soluble macrophage marker up-regulated in numerous inflammatory disorders, is predictive of accelerated atherosclerosis associated with systemic lupus erythematosus (SLE).Methods: Carotid ultrasound was prospectively performed, at baseline and during follow-up, in 63 consecutive SLE patients asymptomatic for cardiovascular disease (CVD) and 18 volunteer health workers. Serum sCD163 level was determined at baseline using enzyme-linked immunosorbent assay. The primary outcome was the presence of a carotid plaque. Factors associated with carotid plaques were identified through multivariate analysis.Results: Despite a low risk for cardiovascular events according to Framingham score in both groups (2.1 ± 3.8% in SLE vs 2.1 ± 2.9% in controls; p = 0.416), ultrasound at baseline showed a carotid plaque in 23 SLE patients (36.5%) and two controls (11.1%) (p = 0.039). Multivariate analysis showed that SLE status increased the risk for carotid plaque by a factor of 9 (p = 0.017). In SLE patients, sCD163 level was high (483.7 ± 260.8 ng/mL vs 282.1 ± 97.5 ng/mL in controls; p < 0.001) and independently associated with carotid plaques, as assessed by stratification based on sCD163 quartile values (p = 0.009), receiver operating characteristics (p = 0.001), and multivariate analysis (p = 0.015). sCD163 at baseline was associated with the onset of carotid plaque during follow-up (3 ± 1.4 years) in SLE patients who had no carotid plaque at the first evaluation (p = 0.041).Conclusion: sCD163 is associated with progressing carotid plaque in SLE and may be a useful biomarker for accelerated atherosclerosis in SLE patients at apparent low risk for CVD.

[Combined heart and kidney transplantation in Fabry's disease: Long-term outcomes in two patients]. / Transplantation combinée cœur­rein au cours de la maladie de Fabry : suivi à long terme de deux patients.

INTRODUCTION: Fabry disease is a lysosomal storage disorder linked to an alpha-galactosidase A deficiency that can lead to heart and kidney failure. There is little data about the prognosis of patients who undergo a combined heart and kidney transplantation. CASE REPORTS: Two brothers who were diagnosed with Fabry disease after the age of 30 years underwent a combined heart and kidney transplantation at respectively 49 and 42 years of age because of a severe hypertrophic cardiomyopathy with end stage renal failure. They are alive respectively 4 and 9 years after the transplantation. No recurrence of the disease in the transplanted organs has been found. CONCLUSION: Combined heart and kidney transplantation in Fabry disease is an efficient therapy for the cardiomyopathy and kidney failure. Its prognosis can be good when the patients are carefully selected. However, an early diagnosis is critical in order to avoid a procedure associated with a high perioperative mortality.

Pituitary-adrenal function after prolonged glucocorticoid therapy for systemic inflammatory disorders: an observational study.

CONTEXT: Glucocorticoid therapy is being used in a wide variety of systemic disorders. Reference papers, published more than 20 years ago, showed no correlation between adrenal insufficiency risk and dose or duration of glucocorticoid therapy. OBJECTIVE: Our objective was to evaluate the extent to which long-term glucocorticoid therapy damages the pituitary-adrenal axis in patients with systemic inflammatory disorders. DESIGN: We conducted a retrospective observational study from January 2011 to August 2012. SETTING: This was a monocentric study at the Department of Internal Medicine, Bichat Hospital, Paris-Diderot University, Paris, France. PARTICIPANTS: Sixty consecutive patients who were receiving long-term prednisone therapy for systemic inflammatory disorders and in whom discontinuation of glucocorticoid treatment was planned. INTERVENTION: A short Synacthen test was performed. A bolus of 0.25 mg 1-24-ACTH was injected in the morning, 24 hours after the most recent dose of prednisone. Cortisol was measured at baseline and 60 minutes after Synacthen injection. MAIN OUTCOME MEASURES: We assessed frequency and risk estimate of pituitary-adrenal dysfunction. RESULTS: Twenty-nine patients (48.3%) had adrenal insufficiency defined by a plasmatic cortisol <100 nmol/L (n = 13) at baseline (time 0) or <550 nmol/L (n = 16) 60 minutes after Synacthen injection. Cumulative dose (area under the receiver operating characteristic curve = 0.77 [95% confidence interval = 0.62-0.91], P = .007) and exposure (area under the receiver operating characteristic curve 0.80 [95% confidence interval = 0.67-0.93], P = .002) to prednisone were predictive for adrenal insufficiency based on a T0 <100 nmol/L. Prednisone was stopped in 29 of 31 patients (93.5%) showing a normal response to short Synacthen test; none of these patients required hydrocortisone replacement with a mean follow-up of 10 (± 6) months. CONCLUSION: Adrenal insufficiency is frequent in patients treated with long-term glucocorticoids for systemic inflammatory disorders and is related to duration and cumulative dose of steroids.

[Long-term follow-up of a French cohort of retroperitoneal fibrosis]. / Fibrose(s) rétropéritonéale(s) : stratégie diagnostique, pathologies associées et suivi à long terme d'une cohorte française.

PURPOSE: Retroperitoneal fibrosis (RPF) is a rare disease with an expanding etiologic spectrum. We aimed to analyze non-invasive diagnosis strategy, associated disorders, monitoring, treatment and prognosis. METHODS: Retrospective cohort study in a single tertiary center. RESULTS: Eighteen RPF cases (11 males) followed between 1996 and 2009 were reviewed. Blood CRP level was high in all cases before treatment. CT scan, associated or not with MRI or 18-FDG PET-scan, confirmed the diagnosis in 15 patients. Histological analysis of a surgical biopsy specimen was performed in only three cases. Ten patients suffered retroperitoneal fibrosis secondary to systemic vasculitis (granulomatosis with polyangeitis, n=1, Takayasu aortitis, n=2), systemic fibrosis with Riedel thyroiditis (n=1) and atheromatous periaortitis (n=6). Fifteen patients were treated with corticosteroids with a mean treatment duration of 60 months (12-228). Dependency to corticosteroids was recorded in ten patients. Patients with fibrosis related to vasculitis were younger, had a higher CRP level, more frequent corticosteroid dependency and a higher relapse rate. Relapses were successfully treated with steroids. Immunosuppressive treatment was only prescribed in the setting of systemic vasculitis. No patient died, after a 6±2 years follow-up. Late relapses could occur, sometimes years after steroid therapy cessation. CONCLUSION: In our study, RPF occurred as a secondary disorder in 60% of the cases. Disease extension, relapse rate and treatment response varied according to the underlying cause of RPF, pleading for an extensive and systematic initial assessment. Since no death or end-stage renal insufficiency was observed, RPF might be considered as a steroid-sensitive and benign disorder.

Pharmacokinetic and pharmacodynamic variability of fluindione in octogenarians.

In the PREPA observational study, we investigated the factors influencing pharmacokinetic and pharmacodynamic variability in the responses to fluindione, an oral anticoagulant drug, in a general population of octogenarian inpatients.Measurements of fluindione concentrations and international normalized ratio (INR ) were obtained for 131 inpatients in whom fluindione treatment was initiated. Treatment was adjusted according to routine clinical practice. The data were analyzed using nonlinear mixed-effects modeling, and the parameters were estimated using MONOLI X 3.2. The pharmacokinetics (PK) of fluindione was monocompartmental, whereas the evolution of INR was modeled in accordance with a turnover model (inhibition of vitamin K recycling). Interindividual variability (II V) was very large. Clearance decreased with age and with prior administration of cordarone. Patients who had undergone surgery before the study had lower IC50 values, leading to an increased sensitivity to fluindione. Pharmacokinetic exposure is substantially increased in elderly patients, warranting a lower dose of fluindione.

[Auditory and vestibular findings in Fabry disease: a study of 25 patients]. / Atteintes ORL de la maladie de Fabry : à propos de 25 observations.

PURPOSE: Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disorder due to deficient activity of the enzyme alpha-galactosidase A. Males and females exhibit severe organ involvement. The high incidence of otological symptoms was recently reported. PATIENTS AND METHODS: Monocentric and retrospective study of twenty-five patients with FD (13 families; seven males and 18 females). The patients underwent audiological assessment before initiation and during enzyme replacement therapy. We also analysed neurologic heart and kidney status. RESULTS: Twenty patients (80%; 13 females and seven males) complained of otologic symptoms. Audiological evaluation showed a sensorineural hearing loss in 17 patients, bilateral in 16 out of them. Vestibular examination showed a functional impairment in two patients (one female, one male). Correlations were found between hearing loss and either kidney disease (73,3%), neurological complications (100%) and cardiomyopathy (80%). Fourteen patients (56%; seven females, seven males) received enzyme replacement therapy. Improvement or stabilization of the audiological evaluation was reported in seven patients, whereas worsening was observed in three patients. CONCLUSION: This study confirms the high frequency of audiological involvements in females and males with FD. Our analysis suggests that the frequency of hearing loss is increased in the presence of renal or neurologic involvement or cardiomyopathy. There is no clinically significant efficacy of enzyme replacement therapy on hearing function. Although the pathophysiology remains unknown, a vascular mechanism responsible of the inner ear involvement seems to be privileged.

[Acute cytomegalovirus infection revealing systemic lupus erythematosus]. / Infection aiguë à cytomégalovirus révélatrice d'un lupus érythémateux systémique.

Systemic lupus erythematosus (SLE) remains of unknown origin. Herpes viridae infections seem to play a role in the pathogenesis of this disease. We report a 31-year-old man who presented an acute cytomegalovirus (CMV) infection with persistent fever and myopericarditis as the presenting manifestation of SLE. This case report emphasizes a difficult differential diagnosis between SLE and an acute CMV infection and suggests a possible role of this virus in the pathogenesis of SLE.

Aseptic meningitis and ischaemic stroke in Fabry disease.

BACKGROUND: Fabry disease (OMIM 301 500) is an X-linked lysosomal storage disease. Neurological symptoms in Fabry disease mainly include stroke, acroparesthesia, cranial nerve palsies and autonomic dysfunction. We report on aseptic meningitis in Fabry patients. METHODS: Clinical analysis, brain magnetic resonance imaging, cerebrospinal fluid analysis, treatment and outcome data were analysed in three cases of meningitis associated with Fabry disease. FINDINGS: Mean age at meningitis onset was 26.6 (24-28) years. Headache was present in all cases and fever in two cases. Meningitis was always diagnosed before Fabry disease. A familial history of Fabry disease was present in two cases. Non-neurological symptoms caused by Fabry disease were present in all cases. All patients also suffered stroke and sensorineural hearing loss. Cerebrospinal fluid (CSF) analysis showed pleocytosis (mean, 36; range: 8-76 cells/mm(3)) and a high protein level (mean, 63; range, 47-70 mg/dl). C-reactive protein blood levels and erythrocyte sedimentation rate were raised. Diagnosis was assessed by low alpha-galactosidase A dosage and/or gene mutation analysis in all cases. All patients were treated with enzyme replacement therapy (ERT). In two cases, lumbar puncture was repeatedly performed and there was no normalisation of CSF under ERT alone, at 9 and 24 months of follow-up, respectively. One patient who suffered intracranial hypertension was treated efficiently with steroids, associated with azathioprine. The fact that Fabry disease could be an auto-inflammatory disorder is discussed. INTERPRETATION: Fabry disease may cause aseptic meningitis.

[Adenosine deaminase is useful for the diagnosis of peritoneal tuberculosis in patients with end-stage renal failure]. / Le dosage d'adénosine déaminase est utile pour le diagnostic de tuberculose péritonéale chez le patient dialysé

INTRODUCTION: End-stage renal failure patients are particularly at risk for tuberculosis, especially for peritoneal tuberculosis. Microbiological diagnosis remains hazardous in many cases. CASE REPORT: We report on a case of peritoneal tuberculosis in an end-stage renal failure patient. The diagnosis was suspected on the basis of adenosine deaminase dosage in peritoneal fluid, allowing an early presumptive treatment and a favourable outcome with a 3 years follow-up. DISCUSSION: The measurement of adenosine deaminase activity in ascites represents a diagnostic advance in tuberculous peritonitis among end-stage renal failure patients.

[Iatrogenic central serous chorioretinopathy during glucocorticoid therapy for temporal arteritis]. / Baisse de l'acuité visuelle chez une patiente traitée par corticoïdes pour maladie de Horton : choriorétinopathie séreuse centrale iatrogène.

INTRODUCTION: Visual complications of temporal arteritis are frequent and serious. Their risk prompts glucocorticoid therapy, but this treatment may also cause ophthalmologic troubles. EXEGESIS: A sudden and isolated monocular visual blur, occurring in a 66 years old woman after 4 month of glucocorticoid treatment for temporal arteritis, revealed a case of iatrogenic central serous chorioretinopathy. The diagnosis of this disease is established by fluorescein angiography and its functional prognosis is excellent. Tapering the doses of glucocorticoids, as fast as the underlying disease allows, hastens visual recovery. CONCLUSION: When the treatment of temporal arteritis is commenced for more than a month, new visual complications are rare. Central serous chorioretinopathy induced by glucocorticoids belongs to the diagnoses that should be evoked in this case, especially if there is no clinical manifestation of arteritis and no inflammatory markers.

[Sirolimus-associated interstitial pneumonitis in a renal transplant patient]. / Pneumopathie organisée au sirolimus: un diagnostic à évoquer chez le patient transplanté d'organe.

INTRODUCTION: Sirolimus is a new immunosuppressive drug used in organ transplantation, particularly in renal transplantation. In the future, it could replace calcineurin inhibitors such as cyclosporine. It is currently associated with side effects, such as thrombocytopenia and hyperlipidemia. Several interstitial pneumonitis associated with sirolimus has been previously described in renal transplant recipients associated with marked general symptoms. EXEGESIS: We report on a 65-year-old renal recipient presenting with a non typical case of sirolimus interstitial pneumonitis. He presented with fever and marked general symptoms for several months. CT scan showed a unilateral interstitial pneumonitis. After infectious, inflammatory and tumoral diseases were ruled out, sirolimus associated interstitial pneumonitis was evoked. The patient improved quickly after discontinuation of sirolimus. CONCLUSION: It is important to evoke, after eliminating other aetiologies, sirolimus induced pneumonitis in face of an organ transplant recipient presenting with marked general symptoms even if the pulmonary symptoms are not predominant.

Familial inflammatory inclusion body myositis.

OBJECTIVE: To compare familial inflammatory inclusion body myositis (IBM) with hereditary inclusion body myopathies and sporadic IBM. PATIENTS AND METHODS: Clinical, biological, MRI, and histological data were analysed in two siblings with inflammatory IBM and compared with those of patients with sporadic and hereditary IBM. RESULTS: Both patients had a clinical phenotype of sporadic IBM, which differs from hereditary myopathies because of late age of onset--respectively 65 and 66 years, and different pattern of muscular involvement--asymmetric, mainly distal but also involving quadriceps. MRI showed selective fatty infiltration and oedema in the extensor compartment of thigh muscles. The diagnosis of IBM was confirmed by muscle biopsy, showing muscle fibres containing numerous rimmed vacuoles, a characteristic shared by all types of IBM. In contrast with hereditary IBM, histological analysis also showed inflammatory mononuclear infiltrate invading non-necrotic fibres, ragged red and oxidase c negative fibres, and positive Congo red staining. Moreover, HLA class II typing disclosed DR beta 1 0301 haplotype, which is significantly related to sporadic but not to hereditary IBM. With steroid treatment and monthly intravenous immunoglobulins, the disease was stabilised in both patients at protracted follow up. CONCLUSION: Sporadic and familial inflammatory IBM share the same clinical, biological, MRI, and histological features.

Subacute clinical forms of Plasmodium falciparum malaria in travelers receiving chloroquine-proguanil prophylaxis.

We have observed 4 French travelers, returning from African countries, who were not immune to malaria and were receiving chloroquine-proguanil prophylaxis, in whom the diagnosis of malaria could easily have been missed because the clinical signs were uncommon. These cases suggest that chloroquine-proguanil prophylaxis is not always effective and that travelers with unexplained symptoms should be monitored closely for malaria.

[Splenic infarction in a HIV-infected patient. Apropos of a case and review of the literature]. / Infarctus splénique chez un patient infecté par le virus VIH. A propos d'un cas et revue de la littérature.

Incomplete ischemia of the celiac trunk due to arterial thrombosis occurred in a patient infected with the HIV. Ischemia led to infarct of the spleen and pancreatitis. Endoluminal desobstruction of the arterial trunk then medical management after exploratory laparoscopy were successful without splenectomy. The causes, diagnostic methods and treatments for splenic infarction in HIV-infected patients are discussed with a review of the literature.