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1.

Introduction

Lymphogranuloma venereum (LG) is a sexually transmitted infection (STI) caused by Chlamydia trachomatis L serovar.

Methods

These five consecutive cases aim to highlight the risk of LG misdiagnosis, in case of initial presentation with isolated inguinal adenitis.

Results

Five men (mean age: 30 ± 7 years) were seen in an internal medicine department, for inguinal adenopathy. One patient had clinical signs of urethritis. None presented an associated rectitis. Three patients had a history of STI, and two had a discovery of related HIV disease. Urinary polymerase chain reaction (PCR) was positive for the symptomatic patient and negative for the others. Lymph node PCR was positive in all patients within a L2b serotype (searched in 4 out of 5 cases).

Conclusion

LG should be evoked in any patient with inguinal adenomegaly, particularly in case of STI history or risk factors. Negativity of urinary PCR should lead to further investigations, essentially a lymph node cytopuncture to evidence Ctrachomatis.  相似文献   

2.

Introduction

Trophic disorders of the extremities are a common complication of systemic sclerosis (SSc), mainly related to microvascular damage. However, SSc seems to be a risk factor for premature athero-thrombotic disease that can affect the peripheral arteries, participate in the occurrence of trophic disorders and promote the occurrence of infectious complications. The objective of this study was to assess the prevalence of arterial disease of the limbs in SSc patients.

Methods

Consecutive inclusions in the context of a multidisciplinary consultation centered on disability of the hand with collection of clinical data [cardiovascular risk factors (CVRF), history of trophic disorders of ischemic origin, peripheral pulse palpation, Allen maneuver the upper (UL) and lower limbs (LL)], and hemodynamic data (flow recorded by Doppler in radial, ulnar, anterior and posterior tibial arteries, and measurement of systolic indices ankles).

Results

Fourteen patients were included (11 right-handers, 2 left-handers, 1 ambidextrous). The sex-ratio male/female was 0.27 and the average age of 58.1 ± 10.4 years. The main CVRF were age and smoking. In the UL, 42.8% of patients had a history of trophic disorders, Allen maneuver was abnormal for 35.7% of the superficial palmar arch, 42.9% of ulnar pulse were not perceived and there was no recordable flow in 25% of ulnar artery. In the LL, 14.3% of patients had already presented trophic disorders toes, Allen maneuver was abnormal for 15.4% of the posterior tibial artery, 25.6% of posterior tibial pulse were not perceived and flow of 15.4% of posterior tibial arteries was pathological.

Conclusion

The distal macrovascular disease preferentially affecting the ulnar and posterior tibial arteries with a high frequency to the UL and two times less at LL. The pathophysiology is unclear but it could be a proper manifestation of SSc. It seems necessary that SSc patients have a strict balance of their CVRF and a screening of macrovascular arterial lesions. There is also the question of the place of an anti-atherosclerotic therapy in these patients.  相似文献   

3.
4.

Introduction

Myeloid sarcomas are uncommon proliferations of immature myeloid cells occurring in any extramedullary organ. We report here two cases of myeloid sarcomas in patients with, respectively, a polycythemia vera and a myelodysplastic syndrome.

Case reports

The first is an 81-year-old woman who presented with osteolytic lesions. Diagnosis has been highlighted using anatomopathological study after bone marrow biopsy, but it was delayed because of a very localized basin lesion and few positive myeloid markers. The second patient is an 86-year-old man who presented with pancytopenia and several lymph nodes. Lymph node cytology failed because of the rarity of blast cells. Diagnosis was done after anatomopathological study on lymph node biopsy which revealed a localized form of myeloid sarcoma.

Conclusion

The diagnosis of myeloid sarcoma must be considered when unusual tumors occur in patients with a chronic myeloid disease. In that case, therapeutic options are those of an acute myeloid leukemia.  相似文献   

5.

Objectives

Ocular tuberculosis is a rare form of extra pulmonary tuberculosis. It represents 1–2% of all clinical forms. The aim of this work was to focus on diagnostic and therapeutic characteristics of ocular tuberculosis.

Methods

We report a case series of 14 patients with ocular tuberculosis seen in an infectious diseases department between 2006 and 2015. The diagnosis was retained on clinical data and a positive tuberculin skin test or interferon-gamma release assay.

Results

The patient's mean age was 40.7 ± 9 years. The most common clinical presentation was uveitis (11 patients and 16 eyes). An extra ocular involvement was associated in three patients. The mean duration of antitubercular therapy was 10 ± 2.5 months. Corticosteroid therapy was associated in 11 cases. The outcome was favorable in all cases. Two patients had maintained visual sequelae.

Conclusion

Ocular tuberculosis is a rare disease but still remains a diagnostic problem. It should be considered in case of any chronic ocular symptoms, especially in endemic countries. Early management can improve the visual prognosis.  相似文献   

6.

Introduction

Anti-3-hydroxy-3-méthylglutaryl-coenzyme A reductase antibody-associated necrotizing autoimmune myopathy has been recently described (2011). This myopathy is distinct from statin toxic myopathy. Our objective is to report on the clinical and para-clinical characteristics of this myopathy and to show the difficulties of therapeutic care.

Case reports

We describe 4 cases of patients followed-up in Brittany, France. All data have been analyzed retrospectively. The mean age of our patients was 59.5 years, with a sex ratio of 1. The clinical presentation was homogeneous, with a subacute painful proximal and symmetrical weakness, without extra-muscular involvement. Other presentations have been described (including pseudo-dystrophic presentation). All patients had a previous statin medication (mean duration of 3.75 years) although this criteria is not a requisite. All patients had high levels of creatine kinase and abnormal electromyographic examination. The pathological pattern on muscular biopsy was a necrotizing myopathy without significant inflammatory cells infiltration. Cardio-respiratory function was normal and no associated neoplasia was found. Over the follow-up, we observed a marked corticosteroid-dependence, not improved by immunosuppressive drugs (azathioprine and methotrexate). The benefit of intravenous immunoglobulin was clear with, sometimes, prolonged responses.

Conclusion

An early diagnosis of this myopathy is necessary in order to introduce an immunotherapy associated with a close monitoring. The therapeutic strategy (within which the stead of intravenous immunoglobulin seems increased) remains to be defined and long-term prospective studies are thus needed.  相似文献   

7.

Introduction

Nocardiosis are uncommon. The diagnosis may be difficult, with significant morbidity and mortality, often occurring on frail patients. Few data are available in France.

Methods

A retrospective single center study was conducted from 2002 to 2014 and included all patients with at least one positive microbiological sample for Nocardia with a follow-up in our hospital.

Results

Nineteen patients, including 15 men, were included with a mean age of 58 years (25–85). Seventeen had a risk factor (lung diseases [13], corticosteroids [12], solid neoplasia [2], HIV infection [2], diabetes mellitus [3], kidney transplant [2], lymphopenia [1]). Infections’ locations were: pulmonary (12), brain (3), skin (2), lymph node (1) and corneal (1). The slow growth leads to a median of 35 days for a positive result (3–95). Nine species were identified. Fifteen patients (79%) received one or more lines of antibiotics including: cotrimoxazole (9), amoxicillin (7) cefotaxime/ceftriaxone (7) imipenem (3), or amikacin (3). The average duration of antibiotic therapy was 207 days. Four patients did not receive antibiotics due to a late result or a bacterial co-infection masking nocardiosis. Five patients died (26%) including 2 with cerebral nocardiosis. Six patients were cured, 4 suffered a relapse, 4 had an unknown evolution, and 1 was still treated.

Conclusion

Our study shows that nocardiosis is a disease difficult to treat. A better understanding of this type of infection is necessary.  相似文献   

8.
9.
10.
IntroductionGaucher disease type 1 is a rare genetic disease. It can cause thrombocytopenia. Current guidelines do not support bone marrow examination in front of isolated thrombocytopenia if no evidence suggests malignant hemopathy. This strategy aiming at sparing unnecessary investigations makes such rare diseases more difficult to diagnose.Case reportA 31-year-old woman was diagnosed with immune thrombocytopenia according to current guidelines. She presented later with mild splenomegaly. Bone marrow aspirate smears showed Gaucher cells. Gaucher disease was then confirmed. Looking backward, initial biological clues (hyperferritinemia, hypergammaglobulinemia) should have enabled to consider the diagnosis.ConclusionGaucher disease type 1 can be responsible for apparently isolated thrombocytopenia. The disease must be looked for if the thrombocytopenia is associated with unexplained hypergammaglobulinemia or hyperferritinemia. Diagnosing immune thrombocytopenia without bone marrow sample requires to systematically pay attention to any clinical or biological abnormality, not to ignore rare differential diagnoses.  相似文献   

11.

Introduction

Although most infections occur within the first 2 years after splenectomy, the relatively short follow-up reported in many studies may underestimate the frequency of infections. The objective of the study was to determine the incidence of infective outcomes and factors associated with infection after splenectomy by studying a group of patients who underwent splenectomy over a 10-year period.

Methods

A retrospective and monocentric study of patients who underwent splenectomy between January 1st, 1997 and December 31st, 2004 in a French university hospital. Age, sex, indication for splenectomy, infectious events, death, vaccination and antibiotic prophylaxis were collected in January 2015.

Results

One hundred and sixty-five patients were included. The most common reasons for splenectomy were therapeutic hematological indications (37.5%). Ninety-seven per cent received pneumococcal vaccine. Prophylactic antibiotics were prescribed in 78% of patients. Thirty-seven patients had 42 severe infections with a median incidence rate of 4 years after splenectomy (2 days–12 years). The rate of infection after splenectomy declined over time but 57% occurred after 2 years and 14.3% after 10 years. Respiratory infections were the most common sites of infections. The incidence of infection differed according to age was highest among the elderly (HR = 6.2; 95%CI: 1.4–27.1; after 65 years old) and underlying reason for splenectomy (P = 0.02). There is no difference with or without prophylactic antibiotics.

Conclusion

After splenectomy, the incidence of severe infection declined over time but can occur after 10 years. The onset of infection is linked to age and reason for splenectomy.  相似文献   

12.

Introduction

Meningoencephalitis (ME) are central nervous system inflammations with many etiologies. The etiology remains unknown in nearly half of the cases. The last French study was performed in 2007. Besides some rare or emerging causes, such as limbic ME, which are curable, infectious ME are the most frequent. Long term ME outcome remains partly known. The study objectives were to assess etiologies and outcome of ME in a University hospital between 01/01/2011 and 31/12/2014.

Methods

This is a retrospective epidemiological study based on computerized files from patients hospitalized in internal medicine, infectious diseases and intensive care departments. Included patients presented compatible clinical symptoms with ME (delirium, focal deficits, epilepsy) and confirmed meningitis after lumbar puncture.

Results

The files of 79 patients were studied. In 37 patients (46.8%) ME was infectious: viral in 16 cases (mainly VZV), bacterial in 16 (mainly pneumococcus). Eight patients (10.1%) presented with a noninfectious cause, 2 of which being limbic ME. Etiology remains undetermined in 34 patients. The patients presenting with a ME of unknown etiology had a lesser serious initial presentation, a shorter time in intensive care department, and a better long-term outcome.

Conclusion

Etiologic diagnosis of ME is difficult. Aside infectious ME, diagnosis of others curable ME is very important.  相似文献   

13.
14.
15.

Introduction

Chronic lymphoid leukemia (CLL) is a hematological malignant disease, associated with a clonal B cell proliferation. The incidence is 4400 new cases per year in France. The prevalence increases with age with a median age at diagnostic of 65 years. Renal involvement is rare and estimated at 1.2% of patients with CLL. Renal pathological diagnoses associated with CLL are variable and are not always related to the hematological disease. We report here on cases of patients with CLL who underwent a renal biopsy over the past 16 years in Marseille.

Methods

All cases of renal biopsies performed in patients with CLL between2000 and 2016 in Marseille were included. Pathological analysis was performed by the same experimented pathologist. Data were collected at the time of biopsy and after treatment.

Results

Ten patients were included in this study. The reason for renal biopsy was acute kidney injury or the onset of nephrotic syndrome. We report on 4 cases of membranous nephropathy, 1 minimal change disease, 1 cryglobulinemia-related membrano-proliferative glomerulonephritis, 1 light chain amyloidosis, 1 fibrillary glomerulonephritis, 1 interstitial monoclonal infiltration and one case of non-specific tubular lesions. Only one patient was treated before the biopsy, 7 patients received a specific hematological treatment of CLL because of its renal involvement. Renal and hematological responses were variable.

Conclusion

Renal involvement of CLL is rare and is not mentioned in the Binet classification. Yet, it can be severe, with acute kidney injury or nephrotic syndrome, and can lead to the initiation of a specific treatment. The most frequent presentation this series was secondary MN, which differs from previous series.  相似文献   

16.
The global population is aging and intensive care unit admission rate of elderly patients is dramatically increasing. The objective of this review is to provide an overview of the literature about the management of elderly patients in intensive care unit and more specifically about epidemiology, admission criteria, mortality, functional prognosis and ethical aspects. We also discuss the data on cardiorespiratory arrest, shock, acute respiratory failure and delirium. The mortality rate of patients over 80 years old in intensive care unit can reach up to 70% at 1 year, but is dependent on many factors, such as comorbidities or frailty. Above all, more than half of elderly patients recover their long-term autonomy. Their quality of life is comparable to that of the same age population. Considering that the first 3 months after an intensive care unit stay are the most decisive in terms of vital and functional prognosis, we will discuss strategies to improve care through the creation of dedicated intensive care-geriatrics networks.  相似文献   

17.
18.
Paget's disease of bone is the second most common metabolic bone disease after osteoporosis. Its pathogenesis is not yet clearly understood. Geographic distribution and epidemiological variations suggest a role of genetic and environmental factors in its pathophysiology. The frequency of the Paget's disease of bone increases with age. Its discovery can be fortuitous. Prognosis mainly depends on the occurrence of complications involving bones and joints, neurological, cardiovascular or metabolic systems. Treatment of symptomatic forms currently relies on bisphosphonates that have transformed its prognosis.  相似文献   

19.
20.
The auto-inflammatory diseases linked to NLRC4 mutations are recently described entities. Transmission is autosomal dominant in 80 % of cases; cases of somatic mutation have already been reported. The disease may display two very different clinical phenotypes: the phenotype 1 (30 %), severe, is dominated by a multisystemic inflammation starting in the first year of life with symptoms of chronic inflammatory bowel disease (IBD), macrophagic actication syndrome (MAS), or even a presentation suggesting a cryopyrinopathy in its CINCA form; the mortality of this phenotype is high (25 %). The phenotype 2 (70 %), mild, usually starts after the age of 3 and is characterized by cold urticaria, arthralgia, ocular features and fever in 50 % of cases without visceral failure. Anti-interleukin-1 inhibitors are effective in most cases (83 %). Interleukin-18 (IL-18) levels are very high in both clinical forms. Interleukin-18 inhibitors and anti-interferon-gamma inhibitors were remarkably effective in two very severe phenotype 1 patients. Thus, NLRC4 mutations can induce various clinical manifestations with two distinct phenotypes. Although still rare, because very recently described, this group of diseases could be evoked by an internist in front of cold familial urticarial; probably more and more cases will be diagnosed thanks to the major progresses of genetic diagnostic tools such as next generation sequencing.  相似文献   

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