[Health status and quality of life in ß-thalassemia adults in Marseille, France]. / État de santé et qualité de vie des patients ß-thalassémiques adultes à Marseille, France.

INTRODUCTION: The life expectancy of ß-thalassemia patients has increased over the last 20 years. In this study, we evaluated the current health status and quality of life of these patients managed in a reference center in Marseille. METHODS: This is a single-center, descriptive study conducted between June and August 2019 in patients over 18 years of age with ß-thalassemia major or intermedia. Clinical and paraclinical data were collected retrospectively and the SF-36 health survey questionnaire was proposed to each patient. RESULTS: 43 of 64 selected patients were included and divided into 2 groups: 35 patients with transfusion-dependent ß-thalassemia and 8 patients with non-transfusion-dependent ß-thalassemia. Liver iron overload is the most frequent complication, present in 80% of transfusion-dependent and 62.5% of non-transfusion-dependent patients. Cardiac iron overload is present only in the transfusion dependent ß-thalassemia group (20%). Hypogonadotropic hypogonadism remains the most common endocrine disorder (41.9%) followed by osteoporosis (37.2%). Among the 31 patients who completed the SF-36 questionnaire, physical and mental quality of life scores were lowered in transfusion dependent (respectively 42.7 and 46.8) as in non-transfusion-dependent patients (respectively 43.8 and 28.9). CONCLUSION: Despite an improvement in medical care, our patients with ß-thalassemia show an alteration in their quality of life that will need to be characterized in the entire French cohort.

[Purtscher-like retinopathy associated with adult onset still disease: Case report and review of the literature]. / Pseudo-rétinopathie de Purtscher dans le cadre d'une maladie de Still de l'adulte : à propos d'un cas et revue de la littérature.

INTRODUCTION: Putscher-like retinopathy is a retinal disease that is similar to the syndrome initially described in 1910 by Purtscher, but occurring in a non-traumatic context. CASE REPORT: We describe a case of acute, Putscher-like retinopathy in a 48-year-old woman experiencing adult onset Still's disease. The diagnosis was based on fundus examination and fluorescein angiography. Based on a review of the literature, we discuss the current available data on the pathophysiology of this syndrome and its prognostic significance. The treatment remains controversial. CONCLUSION: When visual functional signs appear during adult Still's disease, it is necessary to evoke Putscher-like retinopathy, and to ask for an ophthalmological expertise.

[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.

[Abdominal pain in progressive encephalomyelitis with rigidity]. / Encéphalomyélite progressive avec rigidité révélée par une douleur abdominale.

INTRODUCTION: Stiff-person syndrome is rare neurological disease, associating trunk rigidity and painful muscular spasms. A clinical variant of stiff person syndrome is the progressive encephalomyelitis with rigidity and myoclonus (PERM), which includes neurological cognitive disturbances. CASE REPORT: We report a 73-year-old woman initially addressed for abdominal pain, anorexia and severe weight-loss, for whom diagnosis of PERM was made. CONCLUSION: Because of its various clinical presentations, sometimes without evidence for neurological disease, the diagnosis of PERM is delayed. The presence of antineuropile antibodies associated with muscular spasms at electromyogram are strong evidence for this diagnosis.

[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.

Control of a multi-hospital outbreak of KPC-producing Klebsiella pneumoniae type 2 in France, September to October 2009.

An outbreak of Klebsiella pneumoniae carbapenemase (KPC)-producing Klebsiella pneumoniae type 2 was detected in September 2009 in two hospitals in a suburb south of Paris, France. In total, 13 KPC-producing K. pneumoniae type 2 cases (four with infections and nine with digestive-tract colonisations) were identified, including a source case transferred from a Greek hospital. Of the 13 cases, seven were secondary cases associated with use of a contaminated duodenoscope used to examine the source case (attack rate: 41%) and five were secondary cases associated with patient-to-patient transmission in hospital. All isolated strains from the 13 patients: (i) exhibited resistance to all antibiotics except gentamicin and colistin, (ii) were more resistant to ertapenem (minimum inhibitory concentration (MIC) always greater than 4 mg/L) than to imipenem (MIC: 1­8 mg/L, depending on the isolate), (iii) carried the blaKPC-2 and blaSHV12 genes and (iv) had an indistinguishable pulsed-field gel electrophoresis (PFGE) pattern. These cases occurred in three hospitals: some were transferred to four other hospitals. Extended infection control measures implemented in the seven hospitals included: (i) limiting transfer of cases and contact patients to other wards, (ii) cohorting separately cases and contact patients, (iii) reinforcing hand hygiene and contact precautions and (iv) systematic screening of contact patients. Overall, 341 contact patients were screened. A year after the outbreak, no additional case has been identified in these seven hospitals. This outbreak emphasises the importance of rapid identification and notification of emerging highly resistant K. pneumoniae strains in order to implement reinforced control measures.

[Evaluation of practices involving a cross infection risk in anaesthesia]. / Evaluation des pratiques en anesthésie exposant au risque infectieux par transmission croisée.

OBJECTIVE: The practice of anaesthesiology has the potential for transmitting a number of infectious agents to the patient. In France, several recent cases have been identified, so that a wide survey on anaesthesiology practice has been enhanced. METHODS: An anonymous questionnaire, based on the recommendations of the French Society of Anaesthesiology and Intensive Care (Sfar), was send to 8,771 anaesthesiologists and intensive care practitioners and to 2,070 nurses practicing anaesthesiology. RESULTS: A total of 1,343 questionnaires were analyzed (response rate of 12.4%). The study shows that some recommendations were routinely applied, such as: availability of alcohol-based hand hygiene solution in operating rooms (94%), use of antimicrobial filters for respiratory circuits (99%), use of single-use laryngoscope blades (77%), aseptic technique for central venous catheterization (99%), hand hygiene after contact with body fluids (96%). In contrast, the study showed that some recommendations were partially applied: hand hygiene practice (52%), wear of gloves when a risk of blood exposure exists (23%), cleaning of reusable laryngoscope blades (19%), and incorrect wear of masks (71%). The reuse of the same syringe for several patients was described in 2% of the responses. CONCLUSION: This results, similar to those previously described in the literature, must be followed by appropriate training and education of anaesthesia personnel, implementation of the recommendations, and evaluation of practices. Reuse of the same syringe for several patients have to be eradicated because of the high risk of viral transmission.

A parent as a vector of Salmonella brandenburg nosocomial infection in a neonatal intensive care unit.

A newborn baby was admitted to the Neonatal Intensive Care Unit (NICU) of St Germain en Laye Hospital (France) because of premature birth. On day 12, he contracted gastroenteritis due to Salmonella brandenbourg. The salmonellosis led to a septic shock syndrome with a brief cardiopulmonary arrest. He was treated with intravenous ceftriaxone and gentamicin, and the evolution was favorable. Microbiological investigations revealed that the mother was the vector for this nosocomial infection. S. brandenbourg was isolated from the feces of the baby, despite recent recommendations on managing stool specimens from patients hospitalized for more than three days: according to these recommendations, these stools should be processed for viruses and Clostridium difficile toxin only.

[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.

[Auriculo-ventricular conduction disorder following a continuous administration of propofol. Apropos of a case]. / Trouble de la conduction auriculo-ventriculaire lors d'une administration continue de propofol. A propos d'un cas.

A case is reported of atrioventricular conduction trouble (Möbitz I block) during propofol-fentanyl anaesthesia in a healthy 22 year old patient. All troubles disappeared after IV atropine and isoflurane administration instead of propofol infusion.

[Pocket informatics in anesthesia]. / L'informatique de poche en anesthésie.

A simple, easy to use and inexpensive computerized system could optimize the evaluation of anaesthetic activities. Full processing of information remains our ideal but its difficulties and cost make it hardly possible yet. On the other hand, each anaesthetist can use, for example, a pocket Psion Computer which allows to register 15 items for each anaesthesia. All data are subsequently transferred to a Macintosh central computer. However this activity recording cannot be a substitute for the medical anaesthesia chart which remains essential.