[Splenic dysfunction in sickle cell disease: An update]. / Dysfonction splénique au cours de la drépanocytose : mise au point.

The spleen filters blood cells and contributes to the immune defense. The red pulp clears the blood from altered red blood cells via its unique microcirculatory network ; while the white pulp is a secondary lymphoid organ, directly connected to the bloodstream, whose specificity is the defense against encapsulated bacteria through the production of "natural" IgM in the marginal zone. Various health conditions can cause acquired impairment of the splenic function (or hyposplenism) directly and/or through therapeutic splenectomy. Hypo/asplenia is complicated by an increased susceptibility to encapsulated germ infections, but an increased risk of thrombosis and pulmonary hypertension has also been reported after surgical splenectomy. Homozygous sickle cell disease is the most common disease associated with functional asplenia. The latter appears early in childhood likely through repeated ischemic alterations caused by the sickling of red blood cells. In addition, specific complications such as hypersplenism and acute splenic sequestration can occur and may be life-threatening. We provide here an update on the role and physiology of the spleen, which will allow a better understanding of the pathophysiology of spleen damage and its consequences in sickle cell disease.

[Contribution and limits of "OSCE", "long-case" and "global end-of-placement marking" as end-of-rotation assessment methods. Experience from two internal medicine wards]. / Apport et limites des « ECOS ¼, « examen clinique au lit du malade ¼ et « appréciation globale de stage ¼ comme modalité d'évaluation de fin de stage. Expérience de deux services de médecine interne.

INTRODUCTION: During placements, there is an opportunity to learn clinical skills and to assess their application. However, it represents two different goals. The validity of an end-of-placement assessment is questionable, as the medical competency is contextual. We decided to evaluate the contribution and limits of different assessment modalities as an end-of-placement assessment. MATERIAL AND METHODS: Internal medicine clerks were assessed using the Mini-Cex grid by a structured objective clinical examination (OSCE), a long-case clinical examination (LCE) and a global end-of-placement marking (GEPM). Following these evaluations, students and teachers fulfilled an open questionnaire. RESULTS: In 2021, 41 students and 16 teachers participated in the study. Physical examination was evaluated in 0%, 97% et 76% of cases during OSCE, LCE and GEPM, respectively; teaching skills were assessed for 100, 42 et 49% of students in OSCE, LCE and GEPM, respectively. As compared to OSCE, there was a perceived superiority of LCE regarding its formative value (P=0.07 and P=0.03) and its summative value (P=0.0007 and P=0.02), for students and teachers, respectively. Qualitative analysis highlights the breadth of clinical skills that could be assessed during OSCE stations. Integration into a team was an additional skill that could specifically be assessed during GEPM. GEPM could also take into account the progress made during placement. CONCLUSION: Despite its subjectivity, LCE seemed to be the preferred modality for an end-of-rotation assessment.

[Acute chest syndrome in adult sickle cell patients]. / Syndrome thoracique aigu chez les patients drépanocytaires adultes.

Sickle cell disease is a frequent genetic condition, due to a mutation of the ß-globin gene, leading to the production of an abnormal S hemoglobin and characterized by multiple vaso-occlusive events. The acute chest syndrome is a severe complication associated with a significant disability and mortality. It is defined by the association of one or more clinical respiratory manifestations and a new infiltrate on lung imaging. Its pathophysiology is complex and implies vaso-occlusive phenomena (pulmonary vascular thrombosis, fat embolism), infection, and alveolar hypoventilation. S/S or S/ß0-thalassemia genotype, a history of vaso-occlusive crisis or acute chest syndrome, a low F hemoglobin level (<5%), a high steady-state hemoglobin level (> 10 g/dL), or a high steady-state leukocytosis (>10 G/L) are the main risk factors. Febrile chest pain, dyspnea, sometimes cough with expectorations are its main clinical manifestations, and bi-basal crackles are found at auscultation. Inferior alveolar opacities with or without pleural effusions are identified on chest X-ray or CT-scan. Management of the acute chest syndrome should be prompt and implies, besides the recognition of severity signs, a multimodal analgesia, oxygen supplementation, sometimes a parenteral antibiotic treatment and the frequent use of blood transfusions especially in the most severe cases. Prevention is important and includes a regular monitoring of hospitalized patients and the use of incentive spirometry.

[Gallstone complications in sickle cell patients]. / Complications lithiasiques chez les patients drépanocytaires.

Chronic haemolysis exposes patients with sickle cell disease (SCD) to the development of black pigment gallstones, which can trigger biliary complications. In order to avoid these complications, elective cholecystectomy is recommended in France for all SCD patients with detected gallstones. However, all surgeries, and especially abdominal surgeries, entail an increased risk of vaso-occlusive complications in the peri- and post-operative periods, the most dreadful one being the acute chest syndrome. Preoperative transfusion has been shown in several studies to reduce acute postoperative complications, but exposes the patient to definitive alloimmunization, or even delayed post- transfusion haemolysis, justifying a recent trend towards transfusion sparing. The conditions for avoiding transfusion for a simple and frequent surgery such as cholecystectomy are based on a benefit- risk balance, and must be discussed on a case-by-case basis by the SCD specialist. In particular, it seems fully justified to perform prophylactic preoperative transfusion in patients with a history of recent vaso-occlusive crisis or acute chest syndrome (within 6 months preoperatively), and those operated on in an emergency setting, who are particularly at risk of postoperative events.

Effect of hydroxyurea exposure before puberty on sperm parameters in males with sickle cell disease.

Sperm parameters are known to be impaired in men with sickle cell disease (SCD). Although treatment with hydroxyurea (HU) has an impact on sperm quality, sperm preservation is impossible before puberty. This study's primary objective was to analyze and compare sperm parameters in male patients with SCD exposed (or not) to HU before puberty. Twenty-six sperm samples from 15 patients (median age, 17 years; range, 16-23) treated with HU during childhood were compared with 46 samples from 23 HU-naïve patients (20 years; 16-24). The median age at HU initiation was 6 years (1-14 years), the median duration of HU treatment was 4 years (0.5-10), and the mean dose of HU was 22.4 ± 3.7 mg/kg per day. Although we observed substantial quantitative and qualitative semen abnormalities in all patients, there were no significant differences in semen volume, sperm concentration, total sperm count, or spermatozoa motility, morphology, and vitality between the HU-exposed and HU-naïve groups. At the time of the semen analysis, 100% of the patients in the HU-exposed group and 52% of the patients in the HU-naïve group received transfusion therapy. The specific effect of HU on spermatogenesis in very young infants and the putative value of transfusion for reversing the toxicity of HU warrant further investigation.

[Sickle cell trait complications: A case series of 6 patients]. / Complications du trait drépanocytaire : à propos d'une série de 6 cas.

INTRODUCTION: Patients with sickle cell trait (SCT) are commonly considered as asymptomatic carriers. However, some clinical manifestations may occur. METHODS: Here we present a retrospective descriptive study about SCT subjects with at least one complication diagnosed in a sickle cell disease referral center, in Paris, between 2008 and 2019. We also performed a literature review on the complications of SCT subjects. RESULTS: Six patients (between 19 and 65 years old) were included. SCT was already known only for 4 of them at the time of the complication. Four patients presented with a splenic infarct after a stay in high altitude or a plane trip, one of them was associated with papillary necrosis; one patient had isolated papillary necrosis, and the last one had splenic sequestration. These complications happened for most of them after exposure to an unusual situation of hypoxia or deshydratation. Five out of 6 patients had a marked elevated C reactive protein. CONCLUSION: SCT may cause acute ischemic complications in a context of prolonged hypoxia or dehydration. The most commonly reported are the splenic infarct and the renal papillary necrosis. A study of hemoglobin should be considered in these clinical situations in patients with compatible ethnic origin.

[Legionella spp: An update]. / Actualités sur les infections à Legionella.

Legionella-related disease is caused by an intracellular bacteria mainly living in water. Contamination results from inhalation of Legionella sp containing aerosolized water. Main risk factors are tobacco, immunodeficiency, and advanced age. Antigenuria is the cornerstone of the diagnosis. Immunocompromised patients, more commonly infected with non pneumophilaLegionella, present negative antigenuria, and culture and PCR are essential for the diagnosis. Legionnaires' disease may be severe, especially in elderly and/or immunocompromised patients. Mortality rate varies from 10 % in the general population to 50 % in intensive care. Treatment is based on macrolides or fluoroquinolones. Antibiotic resistance is very rare.

[Opioid-induced adrenal insufficiency: Case report and synthesis of the literature]. / Insuffisance surrénale secondaire aux opioïdes : rapport de cas et synthèse de la littérature.

INTRODUCTION: Opioid therapy for pain relief is associated with several adverse effects. Herein, we report the potential consequences of opioid use on the adrenal function. OBSERVATION: A 49-year-old woman with sickle cell anemia (Hemoglobin SS) was admitted for the treatment of a vaso-occlusive crisis. Morphine was used for pain management, provided by intravenous intermittent dosing (patient-controlled analgesia). She developed during the hospitalization low blood pressure, due to secondary adrenal insufficiency (cortisol 74 nmol/L; ACTH 2.9pmol/L). Pituitary gland was normal on brain magnetic resonance imaging and adrenal function recovered after morphine discontinuation. CONCLUSION: Opioids suppress cortisol secretion, primarily mediated by direct negative effect on hypothalamus and pituitary gland. Further studies are needed to define the incidence and the clinical significance of opioid-induced adrenal insufficiency, as well as the need for hormone replacement.

[Use of pocket-sized ultrasound in internal medicine (hospitalist) practice: Feedback and perspectives]. / Échographie ultraportable en médecine interne : retour d'expérience et point de vue.

INTRODUCTION: Point of care ultrasound (POCUS) is routinely used by intensivists and emergency physicians for many years. Its interest is not arguable any more for these specialists, despite the large variety of diseases they care. Hospitalists and internists also should find some interest in POCUS, which convenience and wide range of indications responds well to the variety of their practice. However, it is still not widely used in internal medicine departments. METHODS: We here report our experience of using a pocket-sized ultrasound device in a French internal medicine department. The device used was a Vscan Dual Probe, GE, whose two probes and presets allow for cardiac, abdominal, pulmonary, obstetric, vascular, pulmonary, and superficial soft tissue exploration. One physician of the ward received a course for POCUS that was initially dedicated for emergency physicians. This study reports on the results of the examinations made between January and September 2015. For each examination performed, clinical usefulness was assessed at the time of patient discharge, by two independent physicians who reviewed the clinical course and the results of conventional imaging and rated their evaluation on a Likert scale. RESULTS: One hundred and four examinations were evaluated. The mean duration of the ultrasound examination was 9±5minutes. The POCUS conclusions were corrected by disease course or the results of conventional imaging in 10 (9.6%) cases. The presets of the device: heart, soft tissue, lung, abdomen and vascular were used respectively in 32, 30, 21, 12 and 5% of the examinations. The main indications of POCUS examination were for identification of pleural, pericardial or peritoneal effusion, and to assess the central venous pressure by inferior vena cava examination. Eighteen examinations were performed for puncture of effusion. The retrospectively evaluated clinical benefit was clearly demonstrated in 78% of cases. The agreement between the two blinded assessors was good (kappa coefficient at 0.82). CONCLUSION: Pocket-sized ultrasound device could be used in internal medicine wards. However, its limited performance compared to more sophisticated echography limits the possible explorations and their reliability, which encourages caution and makes critical the question of the initial training of doctors and medical students.

[Benign metastasizing leiomyoma: An unusual cause of aggressive femoral bone tumor]. / Léiomyome métastatique bénin : une cause inhabituelle de tumeur osseuse fémorale agressive.

BACKGROUND: Benign metastasizing leiomyoma (BML) is a rare condition characterized by histologically benign "metastatic" smooth muscle tumors, which can affect women with history of uterine surgery. We report the case of a patient with bone metastases of BML. CASE REPORT: A 78-year-old woman who had undergone uterine surgery six years before hospital admission, was diagnosed with large pulmonary and pleural metastases that necessitated surgical removal. Pathological examination allowed the diagnosis of BML with positive staining for estrogen and progesterone receptors. Three years later, a BML metastasis in the right femoral diaphysis was unexpectedly discovered and treated by osteosynthesis because of a high risk of fracture. Despite an aromatase-inhibitor treatment, new lungs lesions appeared in the next few months. CONCLUSION: BML is a potential cause of aggressive, although histologically benign, bone tumor in women with a history of uterine surgery.

Sense of extension force and angle of the knee joint are correlated between two generations of men.

Numerous motor abilities depend on the activity of proprioceptors, which has been suggested to be genetically determined. To test this hypothesis, the control of torque generated by knee extensors and knee position was studied in 30 father-son pairs both before and immediately after running. After stabilisation of the participant in a sitting position, the knee joint of his dominant leg was flexed to 90°, and the maximal voluntary torque (MVT) of the dominant knee extensors under static conditions was measured. The participant then tried five times to produce 50% of the MVT. Next, the participant extended the knee to 45° five times without visual control. Significant correlations between the reproducibility of successive trials for groups of fathers and their sons were found. The correlation coefficients for the repeatability of the knee extension torque were 0.69 (confidence interval [CI] = 0.45-0.84; P < 0.01) and 0.75 (CI = 0.54-0.87; P < 0.01) before and after the fatiguing exercise, respectively, whereas the coefficient for the reproducibility of positioning the knee was 0.49 (CI = 0.16-0.72; P < 0.01) after the fatiguing exercise. Our results indicate a significant influence of hereditary factors on the control of limb torque and position.

[Blood management in geriatric hospitalized population]. / Épargne transfusionnelle dans la population âgée hospitalisée.

INTRODUCTION: In France, nearly 50% of patients transfused in packed red blood cells are 75 or older. The benefit of restrictive transfusion policies is no longer to be demonstrated, but the practices are still far from it. The objective of our study was to show the impact of a decision support tool on transfusion practices, specifically in a hospitalized elderly population. METHOD: A clinical decision support, validated in the improvement of practices, was created, based on the latest transfusion recommendations of 2014. Our study was interventional, monocentric, within the departments of internal medicine and geriatrics of a university hospital from February to July 2016. The clinical decision support was available for any request of transfusion of packed red blood cells for 75 years old or older patient who was hospitalized in one of these two services. RESULTS: There were 134 transfusions out of 173 for which the prescriber used our tool. Comparing 2016 with the previous two years, our tool decreased the rate of packed red blood cells delivered by 11% compared to 2014 (P<0.005), but there was no significant difference compared to 2015. It has also reduced the transfusion rate of multi-unit transfusions by 35% compared with 2014 and by 29% compared with 2015 (P<0.005). CONCLUSION: Our tool, applied specifically to the elderly, is useful to improve transfusion practices and requires to be validated on a larger scale.