Syndrome d'immunodéficience acquise (AIDS) révélé par un herpes génital sévère. A propos de deux cas.

The authors present 2 cases of AIDS revealed by severe recurrent genital herpes simplex. The patients are 2 young, previously healthy, African women without histories of homosexuality or drug abuse. The first patient died after 5 months of follow-up (post mortem findings: viral bronchopneumonia with positive cultures for herpes and cytomegalovirus (CMV), viral colitis due to CMV). The second patient survived. She has been treated, during the last 11 months, for filariasis, buccal and vaginal candidiasis and cerebral toxoplasmosis.

Helper T cell dysfunctions in a patient with Omenn's syndrome: modulation by IFN-gamma therapy.

Case Reports

Antibody deficiency due to a missense mutation in CD19 demonstrates the importance of the conserved tryptophan 41 in immunoglobulin superfamily domain formation.

Immunoglobulin superfamily (IgSF) domains are conserved structures present in many proteins in eukaryotes and prokaryotes. These domains are well-capable of facilitating sequence variation, which is most clearly illustrated by the variable regions in immunoglobulins (Igs) and T cell receptors (TRs). We studied an antibody-deficient patient suffering from recurrent respiratory infections and with impaired antibody responses to vaccinations. Patient's B cells showed impaired Ca(2+) influx upon stimulation with anti-IgM and lacked detectable CD19 membrane expression. CD19 sequence analysis revealed a homozygous missense mutation resulting in a tryptophan to cystein (W52C) amino acid change. The affected tryptophan is CONSERVED-TRP 41 located on the C-strand of the first extracellular IgSF domain of CD19 and was found to be highly conserved, not only in mammalian CD19 proteins, but in nearly all characterized IgSF domains. Furthermore, the tryptophan is present in all variable domains in Ig and TR and was not mutated in 117 Ig class-switched transcripts of B cells from controls, despite an overall 10% amino acid change frequency. In vitro complementation studies and CD19 western blotting of patient's B cells demonstrated that the mutated protein remained immaturely glycosylated. This first missense mutation resulting in a CD19 deficiency demonstrates the crucial role of a highly conserved tryptophan in proper folding or stability of IgSF domains.

Echovirus meningoencephalitis in X-linked hypogammaglobulinemia.

Echovirus meningoencephalitis and polymyositis are classical complications of X-linked agammaglobulinemia (1). The treatment of meningoencephalitis is troublesome since intravenous (2), intrathecal (3) and intraventricular (4) administration of gammaglobulins have been reported successful, but failure also occurred in some cases (5). We report our experience of high dose intravenous treatment.

An Immunohistochemical Study of the Distribution of the Measles Virus Receptors, CD46 and SLAM, In Normal Human Tissues and Subacute Sclerosing Panencephalitis

We have compared the expression of the known measles virus (MV) receptors, membrane cofactor protein (CD46) and the signaling lymphocyte-activation molecule (SLAM), using immunohistochemistry, in a range of normal peripheral tissues (known to be infected by MV) as well as in normal and subacute sclerosing panencephalitis (SSPE) brain. To increase our understanding of how these receptors could be utilized by wild-type or vaccine strains in vivo, the results have been considered with regard to the known route of infection and systemic spread of MV. Strong staining for CD46 was observed in endothelial cells lining blood vessels and in epithelial cells and tissue macrophages in a wide range of peripheral tissues, as well as in Langerhans' and squamous cells in the skin. In lymphoid tissues and blood, subsets of cells were positive for SLAM, in comparison to CD46, which stained all nucleated cell types. Strong CD46 staining was observed on cerebral endothelium throughout the brain and also on ependymal cells lining the ventricles and choroid plexus. Comparatively weaker CD46 staining was observed on subsets of neurons and oligodendrocytes. In SSPE brain sections, the areas distant from lesion sites and negative for MV by immunocytochemistry showed the same distribution for CD46 as in normal brain. However, cells in lesions, positive for MV, were negative for CD46. Normal brain showed no staining for SLAM, and in SSPE brain only subsets of leukocytes in inflammatory infiltrates were positive. None of the cell types most commonly infected by MV show detectable expression of SLAM, whereas CD46 is much more widely expressed and could fulfill a receptor function for some wild-type strains. In the case of wild-type stains, which are unable to use CD46, a further as yet unknown receptor(s) would be necessary to fully explain the pathology of MV infection.

Nonlinear propagation of modulated ion-acoustic plasma waves in the presence of an electron beam

Theoretical and numerical studies are presented of the amplitude modulation of ion-acoustic waves (IAWs) in a plasma consisting of warm ions, Maxwellian electrons, and a cold electron beam. Perturbations parallel to the carrier IAW propagation direction have been investigated. The existence of four distinct linear ion acoustic modes is shown, each of which possesses a different behavior from the modulational stability point of view. The stability analysis, based on a nonlinear Schrodinger equation (NLSE) reveals that the IAW may become unstable. The stability criteria depend on the IAW carrier wave number, and also on the ion temperature, the beam velocity and the beam electron density. Furthermore, the occurrence of localized envelope structures (solitons) is investigated, from first principles. The numerical analysis shows that the two first modes (essentially IAWs, modified due to the beam) present a complex behavior, essentially characterized by modulational stability for large wavelengths and instability for shorter ones. Dark-type envelope excitations (voids, holes) occur in the former case, while bright-type ones (pulses) appear in the latter. The latter two modes are characterized by an intrinsic instability, as the frequency develops a finite imaginary part for small ionic temperature values. At intermediate temperatures, both bright- and dark-type excitations may exist, although the numerical landscape is intertwined between stability and instability regions.(c) 2006 American Institute of Physics.

Familial ossification of the stylohyoid ligament in a three generation family--a new clinical entity displaying autosomal dominant inheritance

Ossification of the stylohyoid ligament is very common in the Caucasian population. More than 9000 descriptions of apparently isolated case reports on PubMed have been cited over the last 20 years, often associated with an incidental finding on imaging after neck trauma. No cases of familial ossification have been described. We document a family with several affected members, each with an ossified stylohyoid ligament, confirming that ossification may be hereditary in some families and is most likely due to an autosomal dominant gene.

Corticosteroids and peritonsillar abscess formation in infectious mononucleosis

Peritonsillar abscess formation is an uncommon complication of infectious mononucleosis (IM). Early case reports implicated corticosteroids in the development of such abscesses, however, subsequent studies suggested that these drugs do not promote the formation of abscesses at several sites outside the central nervous system. It has recently been demonstrated that zwitterionic polysaccharides, in bacterial capsules, form complexes with CD4(+) T lymphocytes leading to abscess formation. A patient is presented who developed peritonsillar abscess a few days after initiation of corticosteroid therapy for IM; the medical literature was reviewed in respect of this subject. It appears that the occurrence of these abscesses in IM is not strongly linked to corticosteroid treatment. The authors, therefore, recommend that steroids should not be withheld from patients with severe IM on the basis that they may precipitate the development of peritonsillar abscess.

Non-Acanthamoeba amebic keratitis

Purpose. To describe the clinical presentation and outcome of two cases with presumed non-Acanthamoeba amebic keratitis. Methods. Case reports. Results. Both patients presented with typical symptoms and signs of Acanthamoeba keratitis. The patients' soft contact lenses, lens cases, open solutions, and conjunctival samples were cultured. Diagnosis of non- Acanthamoeba amebic keratitis was based on the presence of keratitis and amebic growth from patients' contact lenses of the affected eyes. Amebic culture from the contralateral contact lens was negative. Vahlkampfia cysts were identified in case 1, and Naegleria cysts in case 2. Topical treatment with polyhexamethylene biguanide and propamidine resolved the keratitis in case 1. Case 2 was lost to follow-up. Conclusion. Non-Acanthamoeba amebic keratitis was diagnosed in two patients based on the clinical presentation (resembling Acanthamoeba keratitis), culture of the contact lens, and response to antiamebic treatment (in one case). Keratitis associated with Naegleria contamination of contact lenses has not been previously reported.

Plateau iris syndrome associated with multiple ciliary body cysts:Report of 3 cases

Objective: To describe plateau iris syndrome associated with multiple neuroepithelial cysts of the pars plicata. Methods: Case reports of 3 patients with plateau iris syndrome who were found to have multiple bilateral ciliary body cysts on ultrasound biomicroscopic examination. Results: Ultrasound biomicroscopy revealed classic features of plateau iris syndrome in each patient but also showed multiple neuroepithelial cysts of the ciliary body in each eye. Conclusion: Plateau iris syndrome may be associated with multiple ciliary body cysts.

Postoperative pulmonary calcification in a child with truncus arteriosus

Pulmonary calcification is uncommon in children. It is rarely described following cardiac surgery. Here, the authors describe the case of an infant who developed extensive pulmonary calcification following the repair of truncus arteriosus.

Clozapine-induced liver injury and pleural effusion

<p>Clozapine, whilst associated commonly with a transient and benign increase in liver enzymes, has also been associated with varying presentations of hepatitis in existing case reports. This report describes what we believe to be the first documented case of acute liver injury and pleural effusion associated with clozapine, resolving after cessation of the agent. The case supports existing literature in advocating a high index of suspicion, particularly in the 4-5 weeks following clozapine initiation, when considering nonspecific clinical symptoms and signs.p>

Immune modulation in advanced radiotherapies: Targeting out-of-field effects

<p>By virtue of being a localized treatment modality, radiotherapy is unable to deliver a tumoricidal radiation dose to tissues outside of the irradiated field. Nevertheless, ionizing radiation may result in radiation damage mediated by a bystander like effect away from the irradiated field, but this response is likely to be modest when radiotherapy is the sole treatment modality. Over the last decade there has been a re-emergence of immune modulating therapies as anti-cancer treatment modalities. Clinical trials on vaccines have on the whole been largely disappointing, but greater response rates have been observed from the immune checkpoint modulators. A clinical benefit of using such agents has been shown in disease sites such as melanoma and non-small cell lung cancer. There is growing pre-clinical data and a number of case reports which suggest the presence of abscopal effects when radiotherapy is co-administered with immune checkpoint inhibitors, suggesting that this combination may lead to an enhanced tumour response outside of the primary treatment field. In this review, the mechanisms of such an enhanced out-of-field tumour response, the potential clinical utilities, the optimal radiotherapy delivery and considerations for clinical follow-up following treatment are discussed.p>