[Gaucher Disease type 1 mimicking immune thrombocytopenia: Role of hyperferritinemia and hypergammaglobulinemia in the initial evaluation of an isolated thrombopenia]. / Maladie de Gaucher de type 1 simulant une thrombopénie immunologique : intérêt de l'hyperferritinémie et de l'hypergammaglobulinémie dans le bilan d'une thrombopénie isolée.

INTRODUCTION: Gaucher 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 REPORT: A 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. CONCLUSION: Gaucher 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.

[Chronic lymphoid leukemia and renal complication: Report on 10 cases from Marseille over 16 years]. / Néphropathies au cours de la leucémie lymphoïde chronique : à propos d'une étude monocentrique de 10 cas.

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.

Reducing pre-operative fasting while preserving operating room scheduling flexibility: feasibility and impact on patient discomfort.

BACKGROUND: The need to preserve operating room (OR) scheduling flexibility can challenge adherence to the 2-h pre-operative fasting period recommendation before elective surgery. Our primary objective was to assess the feasibility of a pre-operative carbohydrate (CHO) drink delivery strategy preserving OR scheduling flexibility. METHODS: During the 1st study phase, patients admitted for elective surgery fasted overnight (Control group); during the 2nd phase, patients fasted overnight and received a pre-operative CHO drink (CHO group). CHO delivery time was set to allow any patient to be ready for surgery 30 min ahead of the scheduled time and any patient with an operation scheduled in the afternoon to be ready at 13:00 hours; patients admitted the morning of an early morning operation would not be allowed to take a CHO drink. RESULTS: We included 194 patients in the Control group and 199 in the CHO group. In the CHO group, the morning CHO dose was delivered to 66.3% of the patients (95% CI 59.3-72.9%), with a median pre-operative fasting time period of 4 h 57 min. After excluding patients admitted the morning of an operation scheduled before 10:00 hours, the delivery rate was 77.2% (70.2-83.3%). Patients in the CHO group experienced significantly less pre-operative thirst (median 2 vs. 5 on a 0-10 scale, P < 0.0001) and hunger (0 vs. 2, P < 0.0001) than those in the Control group. CONCLUSION: Although preservation of OR scheduling flexibility resulted in a longer fasting time than recommended, CHO drink can be made available to a large proportion of patients with significantly reduced perioperative discomfort.

[Thymic disease associated with nephrotic syndrome: a new case with membranous nephropathy and literature review]. / Les syndromes néphrotiques para-thymiques : revue de la littérature à propos d'une observation.

INTRODUCTION: Malignant thymoma or thymic hyperplasia is associated with various autoimmune diseases. Renal disease has rarely been reported in this condition. We report a new case with improvement of renal disease after thymectomy. CASE REPORT: A 77-year-old-women with nephritic syndrome was found to have associated thymic mass. Renal pathology showed membranous nephropathy. The thymic mass pathology showed a B2 type thymoma. After thymectomy the nephrotic syndrome improved. CONCLUSION: Glomerulopathy can be secondary to an acquired thymic disease. Membranous nephropathy but also other glomerular diseases can be observed often presenting with nephritic syndrome. Despite the rarity of this association this clinical observation underlines that a thymoma should be searched in the presence of a glomerulopathy. The glomerulopathy can be improved by the treatment of the thymoma.

[Audit of preoperative antibiotic prophylaxis in a surgical site infections surveillance network]. / Audit des pratiques de l'antibioprophylaxie chirurgicale dans un réseau de surveillance des infections du site opératoire.

OBJECTIVE: To evaluate the preoperative antibiotic prophylaxis (PAP) prescriptions in a surgical site infection (SSI) surveillance network. STUDY DESIGN: Auto-evaluative audit in a prospective multicenter cohort included in a surveillance system. PATIENTS AND METHODS: Since 1997, surgical wards in volunteer centers monitored all surgery patients each year during a period of two months. Patients were evaluated for SSI during the 30 days following surgery. Participating centers were asked in 2000 to participate to a PAP practice assessment. For each surgery patient, a questionnaire was completed. The "Guidelines for Antibiotic Prophylaxis Prescription in Surgery" edited in 1999 by the Société française d'anesthésie et de réanimation was used as gold standard. RESULTS: 6109 patients were included in the survey from 34 health care centers and 3881 received PAP. 90% of patients received PAP intravenously and 63% received twice the curative dose. PAP was administered within 90 minutes prior to incision in 70% of cases. 78% of PAP lasted less than 24 hours. PAP indication with regards to the type of surgical procedures was assessed in 4629 patients. PAP guidelines were observed in 1573 (34%) patients: 999 patients in whom PAP was not indicated did not receive PAP and 574 received it in compliance with recommended dose and indications. CONCLUSION: Efforts should be made to improve PAP prescription according to standards guidelines.

Probable transmission of HIV from an orthopedic surgeon to a patient in France.

BACKGROUND: Transmission of HIV from infected health care workers to patients has been documented in only one cluster involving 6 patients of a dentist in Florida. In October 1995, the French Ministry of Health offered HIV testing to patients who had been operated on by an orthopedic surgeon in whom AIDS was recently diagnosed. OBJECTIVE: To determine whether the surgeon transmitted HIV to patients during operations. DESIGN: Epidemiologic investigation. SETTING: The practice of an orthopedic surgeon in a French public hospital. PARTICIPANTS: 1 surgeon and 983 of his former patients. MEASUREMENTS: 3004 patients who had undergone invasive procedures were contacted by mail for counseling and HIV testing. One HIV-positive patient was interviewed, and DNA sequence analysis was performed to compare the genetic relation of the patient's and the surgeon's viruses. Infection-control precautions and the surgeon's practices were assessed. RESULTS: Of 983 patients in whom serologic status was ascertained, 982 were HIV negative and 1 was HIV positive. The HIV-positive patient, a woman born in 1925, tested negative for HIV before placement of a total hip prosthesis with bone graft (a prolonged operation) performed by the surgeon in 1992. She had no identified risk for HIV exposure. Molecular analysis indicated that the viral sequences obtained from the surgeon and the HIV-infected woman were closely related. Infection-control precautions were in accordance with recommendations, but blood contact between the surgeon and his patients occurred commonly during surgical procedures. CONCLUSIONS: An HIV-infected surgeon may have transmitted HIV to one of his patients during surgery.

[The influence of fat emulsions in ternary mixing during total parenteral feeding on biological and galenic parameters]. / Influence de l'émulsion lipidique en mélange ternaire lors de la nutrition parenterale totale sur les paramètres galéniques et biologiques.

Visual and microscopic inspection, pH, osmolality, measurements of particle size were made on one formulation of parenteral admixture. Based on examination of particle size distribution, admixtures prepared from Intrapilide or Ivelip were stable for up to four days. The results of visual and microscopic inspection in case of using Endolipide concluded to a shorter stability in that case. In the second part of this work the two fat emulsions Intralipide and Ivelip were studied to blow out any clinical or biochemical differences between two groups of patients. This clinical study was carried on fourty two patients recovering from digestive surgery. Patient metabolic parameters such as albumin and prealbumin remained the same in the two groups. The serum alkaline phosphatase has significantly been increasing in group Ivelip. Serum phosphoremia has been increasing in group Intralipide.