Posterior segment involvement in cat-scratch disease: A case series.

INTRODUCTION: Cat-scratch disease (CSD) is a systemic infectious disease. The most well-known posterior segment presentation is neuroretinitis with a macular star. In this study, we present a case series emphasising the heterogeneity of the disease and the various posterior segment manifestations. MATERIALS AND METHODS: A retrospective case series of consecutive patients presenting with posterior segment CSD, over a 5-year period (2010 to 2015), at two ophthalmological centres in Midi-Pyrénées. RESULTS: Twelve patients (17 eyes) were included, of whom 11 (92%) presented with rapidly decreasing visual acuity, with 6 of these (50%) extremely abrupt. CSD was bilateral in 5 (42% of all patients). Posterior manifestations were: 12 instances of optic nerve edema (100%), 8 of focal chorioretinitis (67%) and only 6 of the classic macular edema with macular star (25% at first examination, but 50% later). Other ophthalmological complications developed in three patients; one developed acute anterior ischemic optic neuropathy, one a retrohyaloid hemorrhage and one a branch retinal artery occlusion, all secondary to occlusive focal vasculitis adjacent to focal chorioretinitis. CONCLUSION: Classical neuroretinitis with macular star is not the only clinical presentation of CSD. Practitioners should screen for Bartonella henselae in all patients with papillitis or focal chorioretinitis.

[Recurrent bilateral anterior uveitis due to Leishmania infantum in a patient with immune deficiency related to HIV infection: a case report and literature review]. / Uvéite antérieure bilatérale récidivante à Leishmania infantum chez un patient présentant un déficit immunitaire lié à une infection à VIH : à propos d'un cas et revue de la littérature.

We report the case of a 56-year-old patient, presenting with bilateral hypertensive anterior uveitis due to Leishmania infantum, in the setting of immune deficiency related to HIV infection. The etiology of the uveitis was diagnosed following detection of L. infantum DNA on anterior chamber paracentesis. Initially, the patient received an intravitreal injection of liposomal amphotericin B, systemic antiparasitic treatment, topical steroid and ocular hypotensive treatment. Due to unfavourable disease progression in the right eye, we re-evaluated the anti-inflammatory, antiretroviral and antiparasitic medications (beginning meglumine antimoniate): the uveitis in the left eye was thus able to be controlled. We followed the efficacy of treatment with weekly quantification of Leishmania DNA in the aqueous humor. Uveitis secondary to leishmaniasis is rare and serious. Physicians must be aware of this cause of uveitis, particularly in immunodeficient patients. The quantitation of Leishmania DNA in the aqueous humor is an indispensible tool for monitoring the disease.

FDG-PET/CT findings during immune reconstitution in an HIV-1 patient infected with visceral leishmaniasis.

Visceral leishmaniasis can rarely be unmasked by immune reconstitution in human immunodeficiency virus (HIV)-1-infected patients. We report the first case of immune reconstitution associated with leishmaniasis in an HIV patient to be imaged with [(18)F]fluorodeoxyglucose positron emission tomography (FDG/PET), at both baseline and after therapy.

[Inappropriate low glycated hemoglobin and hemolysis]. / L'hémolyse : une cause d'erreur dans l'interprétation de l'hémoglobine glycosylée.

Hemoglobin A1c (HbA1c), measured regularly in the patients with diabetes, is the major form of stable glycated hemoglobin and has to be maintained below 6.5% to prevent or decrease the risk of chronic complications. HbA1c reflects mean blood glucose levels of the previous 3 months. We report the case of a particularly low HbA1c in a diabetic patient despite high plasma glucose levels, that was induced by auto-immune hemolysis related to an Evans syndrome. All hemolytic disorders can be responsible for falsely reassuring HbA1c values. Clinicians must be aware that hematologic status has to be considered for the correct interpretation of HbA1c results.

Peripheral blood natural killer cell count is associated with clinical outcome in patients with aaIPI 2-3 diffuse large B-cell lymphoma.

BACKGROUND: Lymphocytopenia is a prognostic factor in Hodgkin's disease. In diffuse large B-cell lymphoma (DLBCL), data are much less established, in spite of numerous reports on immune system-lymphoma interactions. This study addresses the prognostic value of blood lymphocyte subsets at diagnosis in DLBCL. PATIENTS AND METHODS: Absolute values of blood lymphocyte subsets and monocytes were prospectively determined by flow cytometry in 140 patients with 2 or 3 adverse age-adjusted International Prognostic Index (aaIPI) factors included in a Groupe d'Etude des Lymphomes de l'Adulte protocol (LNH98B3). Absolute cell counts at diagnosis and aaIPI were evaluated with regard to clinical outcome. RESULTS: Low median cell counts of 337, 211, and 104/mul were evidenced for the CD4+, CD8+ T, and natural killer (NK) cells, respectively. In univariate analysis, only NK cell count [odds ratio (OR) = 1.81 (1.27, 2.57), P = 0.001] and aaIPI [OR = 2.29 (0.95, 5.45), P = 0.06] were associated with induction treatment response. Low NK cell count [Hazard ratio (HR) = 1.27 (1.06, 1.52), P = 0.01] and aaIPI 3 [HR = 1.95 (1.20, 3.16), P = 0.01] were also associated with a shorter event free survival (EFS). In multivariate analysis, NK cell count was associated with response [OR = 1.77 (1.24, 2.54), P = 0.002] and EFS [HR = 1.25 (1.04, 1.50) P = 0.02] independently of aaIPI. CONCLUSIONS: This study shows an association between circulating NK cell number and clinical outcome in DLBCL, possibly important in the context of the broadening use of rituximab, a likely NK-dependent therapy.

Use of allogenic epidermal sheets for difficult wound healing: selection and testing of relevant growth factors.

The clinical interest of using allogenic epidermal sheets (AES) has largely been shown [1,2,3]. As well as covering, they also stimulate healing, by simultaneously secreting numerous growth factors (GFs), although little is known on their mechanism of action. Our objectives were to: (a) devise a test for the efficacy of AES release, (b) select keratinocyte-secreting strains and optimal culture conditions. Three GFs were selected: IL-1alpha, IL-8 and VEGF. Three different keratinocyte strains were cultured for 3 and 6 days after confluence for 3 passages. Assays were performed after 3 h and 24 h+3 h after dispase treatment (AES conservation for 24 h then change of medium and sampling after 3 h). AES were found to secrete GFs in DMEM and the amounts were greater when cultured for 6 rather than 3 days after confluence. Each strain had different secretory patterns depending on passage and time in culture, this variability being explained by inter-individual heterogeneity.

An in vitro model to evaluate the cytokine response in Echinococcus infections.

With the aim of proposing an alternative model to animal experimentation, we investigated cytokine production in response to antigens in an in vitro system. This is a co-culture system of healthy human leukocytes and enterocyte-like Caco-2 cells. The antigens tested, EgA31, EgTrp, and FABP1, are candidates for vaccines in infections caused by Echinococcus spp. in the gut. All three have previously been described in the protoscolex stage and belong to protein families which confer protective immunity against several helminths. In this study, we evaluate the Th1/Th2 profile (Th1: IL-12, IFN-gamma; Th2: IL-6, IL-10) in response to protoscoleces, EgA31 and the mixture of EgA31, EgTrp and FABP1. No cytokine production was detected in response to protoscoleces. Neither IFN-gamma nor IL-6, but a significant IL-10 and IL-12 concentration was detected in response to both types of antigens. These findings suggest that EgA31 and the mixture EgA31/EgTrp/FABP1 generated an immunogenic response associated with a mixed Th1/Th2 cytokine.

[Preliminary results in standardization of flow cytometry protocols for monocytic HLA-DR measurement and their application in the follow up of septic shock]. / Standardisation de la mesure de l'antigène HLA-DR monocytaire par cytométrie en flux: résultats préliminaires et application dans le suivi des chocs septiques.

In septic shock, the diminished HLA-DR expression on monocytes has been proposed as a marker of immunoparalysis that correlates with an increased risk for fatal outcome. The present study was designed to determine whether some differences in protocol procedures could lead to discrepant results in HLA-DR measurement. After establishing a reliable protocol, the second objective was to illustrate the immunoparalysis in patients with septic shock. HLA-DR measurement on monocytes was determined by means of flow cytometry in 54 healthy donors and 16 patients with septic shock. We demonstrated that storage temperature, storage duration before staining and red cells lysis constitute crucial steps in HLA-DR measurement. The precision results with coefficients of variation below 5%, were quite convincing for a manual immunoassay. At 48 hours after diagnosis of septic shock, we found severely decreased percentages of monocytes expressing HLA-DR in septic patients (24 +/- 4%, mean +/- SEM) in comparison with healthy donors (90 +/- 1%), p < 0.001). Furthermore, the persistence of a low level of monocytic HLA-DR (less than 50 %) at day 9 after admittance was associated with patients who died. This study illustrates the state of immunoparalysis in patients with septic shock and supports the potential interest in measuring HLA-DR expression on monocytes. However, multicenter studies are now needed to validate this parameter. Based on our analytical results, we conclude that a critical issue in such studies will be the capacity in each center to perform standardized measurement of HLA-DR. It should be remembered that this determination requires the definition of a common analytical procedure between laboratories participating in the trial.

Human gammadelta T cells express a higher TCR/CD3 complex density than alphabeta T cells.

The aim of our study was to compare CD3 expression on gammadelta T cells and alphabeta T cells in human patients. The antigen density of TCR and CD3 on both subsets was assessed by a quantitative method in eight patients. In parallel, we developed and validated a reliable direct tricolor staining protocol that we tested on samples from hospitalized and healthy individuals (n = 60). Our results demonstrate that human gammadelta T cells constitutively express approximately twofold more of the TCR/CD3 complex than alphabeta T cells. We suggest that this enhanced expression of the TCR/CD3 complex could contribute to the higher reactivity of gammadelta T cells compared to alphabeta T cells. These clinical laboratory results confirm the fundamental data described elsewhere. gammadelta T cells deserve further clinical investigations to understand their precise role in human immunity.

Serum levels of YKL-40 and C reactive protein in patients with hip osteoarthritis and healthy subjects: a cross sectional study.

BACKGROUND: YKL-40 is a 40 kDa glycoprotein secreted by chondrocytes and synoviocytes. It has been suggested that it is a surrogate marker of synovial inflammation and joint destruction in rheumatoid arthritis (RA) and osteoarthritis (OA) and related to C reactive protein (CRP) serum levels in RA. OBJECTIVE: To study serum levels of YKL-40 in patients with hip OA and its relation with CRP. METHODS: YKL-40 and CRP were assayed in serum samples from 45 patients (24 women, 21 men, mean age 65) with symptomatic OA of the hip and 33 healthy controls. YKL-40 was assayed by immunoassay and CRP by ultrasensitive immunonephelometry. OA severity was assessed by the measurement of joint space width with a computer analysis system of digitised hip radiographs. Statistical analysis was performed to determine correlations between serum markers and radiological joint space width. RESULTS: The mean (standard error) YKL-40 level was 90.3 (8.2) ng/ml in patients with hip OA and 66.9 (8.2) ng/ml in controls (p=0.03). The mean CRP level was 2.93 (3.03) mg/l in OA and 1.40 (1.61) mg/l in controls (p=0.006). The serum levels of YKL-40 and CRP increased with age and were significantly correlated (Spearman test: r(s)=0.42, p=0.005) in patients but not in controls. Neither YKL-40 nor CRP correlated with radiographic joint space width. CONCLUSIONS: Serum YKL-40 was significantly increased in patients with hip OA. The correlation between YKL-40 and CRP suggests that YKL-40 may be a marker of joint inflammation in OA. Longitudinal studies are required to assess the usefulness of YKL-40 in the monitoring of patients with hip OA.

[Standardization and automation of HLA B27 typing by flow cytometry: validation and comparison with microlymphocytotoxicity]. / Standardisation et automatisation du typage HLA B27 par une technique cytofluorimétrique: validation par comparaison à la microlymphocytoxicité.

One of the strongest known association between human leukocyte antigen (HLA) phenotype and disease is that of ankylosing spondylitis and HLA-B27. Thus, the determination of HLA-B27 status is an useful tool in the diagnosis of ankylosing spondylitis. To date, the 2 reference methods for HLA typing (microlymphocytotoxicity and molecular biology techniques), are costly in terms of both technician time and materials, and require a great deal of experience. In total, these techniques are not well-suited for routine application in clinical immunology laboratories. Use of flow cytometry has recently been applied for HLA-B27 typing. Nevertheless, it requires an extensive validation protocol. We developed a flow cytometry technique as standardized as possible (whole blood, automated lysing system, automated photomultiplier voltage calibration, definition of thresholds stable with time) and validated our results by comparison with microlymphocytotoxicity. In total, 326 samples were analyzed. We found 99% of concordant results between the 2 techniques, and neither false positive results nor false negative results with flow cytometry could be observed. These results illustrate the reliability of the protocol. It should be remembered that reference technique remains necessary to confirm the few results (< 1%) found in "grey zone" by flow cytometry. Standardization of flow cytometry techniques, as described in this work for HLA B27, seems to be a reasonable goal for the next decade in clinical immunology laboratories.

[Mothers rooming-in in a department of pediatrics (author's transl)]. / L'hospitalisation du jeune enfant avec sa mère dans un service de pédiatrie générale. I. Caractéristiques de la population du secteur "mère-enfant" dans un service de pédiatrie générale.

The socio-economic and medical characteristics of a population of 100 children hospitalized in a "mother and child" section of a general pediatric department were studied in order to point out some objective data. This population appears socio-economically privileged. The majority of them are single children, with ages ranging from 0 to 10 years and with many babies under 6 months. There are as many working mothers in this group as in the average population of the department. Types of diseases are varied as the children were not chosen on medical criteria. Lastly, the length of the stay in hospital becomes noticeably shorter.

[Mothers rooming-in in a department of pediatrics (author's transl)]. / L'hospitalisation du jeune enfant avec sa mère dans un service de pédiatrie générale. IL Les parents, l'équipe soignante et les médecins face à l'hospitalisation de l'enfant avec sa mère.

In order to better understand the reactions arising from the admission of a child with his mother to the hospital, the reactions of the families, of the hospital staff and of doctors to the permanent presence of the mothers have been analysed. It appears that, in spite of the constraints linked with this type of hospital treatment, it does have positive aspects as compared to the usual type of hospitalization : the organization of the admission, the existence of a single medical contact, a closer observation, a team which works closer together, better possibilities of obtaining information, a shorter stay in hospital; all these improvements could be generalized. However, this concept of hospital treatment is quite demanding for the hospital staff, who must have the support of all the doctors concerned and of the administration.

[Propionicacidemia. A report on two cases (author's transl)]. / Acidémie propionique. A propos de deux observations.

Two cases of propionicacidemia are reported. The first patient was a child aged 16 months with psychomotor retardation, severe disturbances of wakefulness, and vomiting; the second case concerned a new-born baby with metabolic acidosis and neurological disorders. In both cases the diagnosis of propionicacidemia was made after the discovery of ketonuria, and raised blood ammonia and glucose levels. Appropriate dietary measures led to great improvement in the first case, and nearly normal psychomotor development in the second case at 7 months of age. Problems related to biotin sensitivity, leucine intolerance, and raised blood ammonia levels are discussed.

[Bourneville's tuberose sclerosis in children (author's transl)]. / La sclérose tubéreuse de Bourneville chez l'enfant. Etude de trente cas.

In 20 out of a series of 30 children with Bourneville's disease, the diagnosis was made from the association of epilepsy and multiple achromic spots, before the appearance of adenomas. A total of 27 of these 30 children had these spots, and their characteristics are reviewed, as well as the average age of onset and frequency of the other typical cutaneous signs noted in this group: sebaceous adenomas, Koenen's tumors, and leather-grained skin plaques. Severe epilepsy was a constant finding and was often resistant to treatment: West's syndrome (18 cases), and Lennox Gastaut's syndrome (16 cases). Severe mental deficiency was also an almost constant finding and was increased by behavioural disorders: 11 out of the 13 children aged more than 5 years. Other locations of the disease included more particularly the ocular (8 cases), skeletal (11 cases), and renal (1 case) regions. In 5 cases, the disease had been transmitted by a parent, but in 3 of these it had not been recognized.