[The causes of splenic infarction: An almost systematic review of the literature]. / Les causes des infarctus spléniques : une revue quasi systématique de la littérature.

INTRODUCTION: Splenic infarction is a rare event in clinical practice, diagnosed by CT scan. There are many causes. They often determine the treatment given. However, there is no consensus on etiological investigations. METHODS: We present here an almost systematic review of the literature, based on data available on Pubmed from 1991 to 2022. Using the keywords "splenic infarct", from 1893 references, 11 cohort studies and 867 clinical cases were included in this review. Articles written in languages using alphabets other than Latin were excluded. RESULTS AND CONCLUSIONS: Analysis of these various studies has enabled us to draw up a list that is intended to be as exhaustive as possible of the causes of splenic infarction. The most frequent are emboligenic heart disease, hematological malignancies, solid neoplasia and certain infections. The descriptions available in the literature were mainly based on isolated clinical cases, not always making it possible to establish a causal link with the disease described, especially as around 20% of reported cases of splenic infarction were asymptomatic and potentially of incidental discovery. Based on the findings of this literature review, we propose a protocol for the etiological assessment of splenic infarcts.

Treatment with Hizentra in patients with primary and secondary immunodeficiencies: a real-life, non-interventional trial.

BACKGROUND: Although Hizentra is indicated for immunoglobulin replacement therapy in patients with primary and secondary immunodeficiencies, phase III trials have focused on patients with primary immunodeficiencies. In this 9-month, real-life, prospective, non-interventional, longitudinal, multicenter study of patients with primary and secondary immunodeficiencies in France, treatment modalities (primary endpoint), efficacy, safety, tolerability, quality of life, and treatment satisfaction were evaluated using descriptive statistics. RESULTS: Starting in January 2012, 117 patients were enrolled (99 adults, 18 children). Secondary immunodeficiencies were present in 48.7 % of patients. At follow-up, injections were administered every 7 days in 92.2 % of patients. Nine patients (7.8 %) were taking Hizentra every 10-14 days. The median dose of Hizentra administered was 0.1 g/kg/injection. Fifty-six patients were administered doses <0.1 g/kg/injection and 13 patients were administered doses >0.2 g/kg/injection. Mean trough IgG titers were 9.0 ± 3.3 g/L (median 8.3 g/L). The mean yearly rate of infection was 1.2 ± 1.9. Mean scores on the Short Form-36 physical and mental component summaries were 46.3 ± 10.0 and 46.6 ± 9.3, respectively. Scores on the Treatment Satisfaction Questionnaire for Medication ranged from 69.9 ± 19.9 to 88.3 ± 21.2 depending on the domain. Treatment with Hizentra was well tolerated. No single drug-related systemic reaction occurred in more than one patient and few local reactions were reported (n = 5). CONCLUSIONS: Under real-life conditions and in a cohort that included patients with primary and secondary immunodeficiencies, treatment with Hizentra was effective and well tolerated and patients were generally satisfied with the treatment.

Infections Revealing Complement Deficiency in Adults: A French Nationwide Study Enrolling 41 Patients.

Complement system is a part of innate immunity, its main function is to protect human from bacterial infection. As genetic disorders, complement deficiencies are often diagnosed in pediatric population. However, complement deficiencies can also be revealed in adults but have been poorly investigated. Herein, we describe a case series of infections revealing complement deficiency in adults to study clinical spectrum and management of complement deficiencies.A nationwide retrospective study was conducted in French university and general hospitals in departments of internal medicine, infectious diseases enrolling patients older than 15 years old who had presented at least one infection leading to a complement deficiency diagnosis.Forty-one patients included between 2002 and 2015 in 19 different departments were enrolled in this study. The male-to-female ratio was 1.3 and the mean age at diagnosis was 28 ±â€Š14 (15-67) years. The main clinical feature was Neisseria meningitidis meningitis 75% (n = 31/41) often involving rare serotype: Y (n = 9) and W 135 (n = 7). The main complement deficiency observed was the common final pathway deficiency 83% (n = 34/41). Half of the cohort displayed severe sepsis or septic shock at diagnosis (n = 22/41) but no patient died. No patient had family history of complement deficiency. The mean follow-up was 1.15 ±â€Š1.95 (0.1-10) years. Half of the patients had already suffered from at least one infection before diagnosis of complement deficiency: meningitis (n = 13), pneumonia (n = 4), fulminans purpura (n = 1), or recurrent otitis (n = 1). Near one-third (n = 10/39) had received prophylactic antibiotics (cotrimoxazole or penicillin) after diagnosis of complement deficiency. The vaccination coverage rate, at the end of the follow-up, for N meningitidis, Streptococcus pneumonia, and Haemophilius influenzae were, respectively, 90% (n = 33/37), 47% (n = 17/36), and 35% (n = 14/34).This large study emphasizes that complement deficiencies can be revealed in adults by infectious episodes. Most of them were meningococcal infections revealing common final pathway deficiency. To avoid undiagnosis or late diagnosis, adult displaying first episode of N meningitidis infection should be tested for complement deficiency.

[Shifting cellulitis in a patient with X-linked hypogammaglobulinemia]. / Dermo-hypodermite bactérienne « à bascule ¼ chez un patient atteint d'hypogammaglobulinémie liée à l'X.

BACKGROUND: In cases of immunodeficiency, a systemic infection may be revealed by atypical symptoms, particularly those involving the skin. PATIENTS AND METHODS: The present case describes a 19-year-old male with X-linked hypogammaglobulinemia, or Bruton agammaglobulinemia, treated with intravenous immunoglobulin G antibodies. Over a 6-week period, the patient developed recurrent plaques in both legs, first on one and then on the other, without fever. Blood cultures were repeated and the fifth pair proved positive for Campylobacter jejuni. An abdominal scan showed appendicitis without intestinal signs. The patient was treated with azithromycin for 2 weeks, which resulted in full recovery from the skin lesions. DISCUSSION: Campylobacter bacteremia infections are severe and carry a 15% mortality rate at 30 days. The majority of affected patients present humoral immunodeficiency. The literature contains reports of 10 patients with C. jejuni-associated cellulitis, of whom 6 presented hypogammaglobulinemia. We postulate that the cutaneous manifestations were caused by septic metastases. The immunoglobulin replacement therapy mainly comprised IgG antibodies; IgA and IgM antibodies appear to play a key role in the response to C. jejuni infection, which could explain the susceptibility observed. The American guidelines recommend blood and skin cultures in patients with cellular immune defects. We suggest that this recommendation be extended to patients with humoral immunodeficiency.

[Role of PET-scan in the positive diagnosis of sarcoidosis in the work-up of uveitis]. / Apport du TEP-scan dans le diagnostic positif de la sarcoïdose, au cours du bilan d'uvéite indéterminée.

INTRODUCTION: Sarcoidosis is a multisystem granulomatous inflammatory disease, which may present as uveitis. Work-up includes the search for pulmonary and extra-pulmonary sites, with the assistance of PET-scanning. MATERIAL AND METHODS: We present six patients enrolled retrospectively from March 2012 to November 2013 with uveitis, for whom a systemic work-up was performed, along with specific tests for sarcoidosis. 18-FDG PET-scan was performed when histology was inconclusive for epithelioid granulomata and giant cells, or when CT and/or chest X-ray were normal, in cases of uveitis clinically suggestive of sarcoidosis. RESULTS: Hypermetabolic foci were found in all patients, in inflammatory areas with foci mainly located in the mediastinal (65%), hilar (33%), cervical, and supraclavicular regions. There was no correlation between ACE levels and positivity of the PET-scan, since only two patients exhibited ACE > 70 IU/L. DISCUSSION: PET-scan is a promising technique in the field of sarcoid uveitis; however, histologic proof remains the gold standard. CONCLUSION: This study demonstrates the value of PET-scan in the assessment of indeterminate uveitis, by screening for sarcoidosis, and permits an assessment of the extent of the disease.

[Quick diagnostic and therapeutic assistance unit: activity and impact on relationship between internal medicine and general practitioners]. / Unité d'assistance diagnostique et thérapeutique rapide : bilan d'activité et impact sur les relations médecine générale et médecine interne hospitalière.

INTRODUCTION: General practitioners report difficulties to work efficiently with hospital physicians. We created a phone number dedicated to general practitioners to contact directly a hospital physician in the general internal medicine department entitled "quick diagnostic and therapeutic assistance". The aim of this study was to assess the first year activity of this professional support and its impact on general practitioners. RESULTS: We received 663 phone calls from February 2005 to February 2006. This led to a simple medical advice (41%), and immediate (26%) or delayed (32%) consultation or admission. Results of the mail survey showed that this "quick diagnostic and therapeutic assistance" was helpful for the general practioners. CONCLUSION: Quick diagnostic and therapeutic assistance improve the quality of clinical care through better continuity of care between the public hospital and the general practitioners.

[Gaucher's disease in Rennes University hospital: a 10-year retrospective study]. / Etude rétrospective sur dix ans des patients atteints de maladie de Gaucher au CHU de Rennes.

INTRODUCTION: Gaucher's disease (GD) remains rare and cohort studies are essential to improve our knowledge of this disease. METHODS: We performed a 10-year retrospective study of patients with GD followed-up in the Rennes University teaching hospital. RESULTS: Among a population of 1,500,000 inhabitants, 12 patients with GD were identified. Eight were men, and four were women. Mean age at diagnosis was 32.3 years and the first symptoms appeared around 31 years old. Main symptoms were: splenomegaly (82%), hepatomegaly (64%), thrombocytopenia (73%), anemia (64%), deterioration of general status (45%), bone pain (27%). Parkinsonism was noted in two patients, polyclonal gammopathy in two others, and monoclonal gammopathy was evidenced in four patients, with chronic lymphocytic lymphoma in one of them. Enzymatic activity dosage confirmed the diagnosis of GD for eight patients. For the remaining four patients, diagnosis was obtained by identification of Gaucher's cells on tissue examination. Substitutive enzymotherapy (SE) was performed for seven patients, with great improvement of initial symptoms. For two of these seven patients, SE is changed for miglustat with persistent improvement of clinical status. CONCLUSION: Association between GD and Parkinsonism or between GD and gammopathy was confirmed in our study. Other cohort studies are needed to improve the knowledge of GD.

[Efficiency of rituximab in acquired hemophilia: report of two cases and review of literature]. / Efficacité du rituximab dans les hémophilies acquises: à propos de deux cas et revue de la littérature.

INTRODUCTION: Acquired hemophilia due to an inhibitor of factor VIII is a rare clinical situation. EXEGESIS: Rituximab is now used in the treatment of acquired hemophilia. We report two cases of acquired hemophilia treated by rituximab with efficiency. CONCLUSION: Rituximab appears to be a first line immunosuppressive therapy in acquired hemophilia, especially in post-partum hemophilia.

[Diagnosis of solid malignancies in an internal medicine unit: a series of 114 consecutive cases]. / Le diagnostic des cancers solides dans un service de médecine interne: étude descriptive de 114 cas consécutifs.

PURPOSE: A deterioration of the general condition, a prolonged fever or an unexplained inflammatory syndrome are frequent reasons for hospitalization in a internal medicine unit. In these situations, it is not rare to make a diagnosis of cancer. PATIENTS AND METHODS: A descriptive study was carried out over a three years period (1st October 1999 to 30th September 2002) in an internal medicine unit. Every week, all patients in whom a cancer was diagnosed were enrolled in the study. RESULTS: During this period, 165 patients were identified (3.8% of the in-patients). A histological proof was obtained in 114 patients. Digestive and bronchopulmonary cancers were the most frequent. The first signs were very varied but digestive disorders and ferriprive anaemia were the most frequent. The number of investigations necessary to diagnosis were weak (1.56 procedures) when a sign was identified but were high (5.12 procedures) when no information was provided by interview, clinical examination or usual biological tests. CONCLUSION: Diagnosis of cancer is an usual situation in an internal medicine unit. Interview and clinical examination are essential in the diagnostic step. It could decrease the number of procedures. Internal unit services are fully concerned by the announcement of cancer.