1/59. listeria monocytogenes and recurrent mycobacterial infections in a child with complete interferon-gamma-receptor (IFNgammaR1) deficiency: mutational analysis and evaluation of therapeutic options.We describe the history of a girl with interferon-gamma-receptor (IFNgammaR1) deficiency and studies performed to identify the molecular and clinical characteristics of this recently discovered disorder. This is the first report of a child from Northern europe with IFNgammaR1 deficiency. The patient, now 7 years old, first presented with swelling and reddening at the Bacille Calmette-Guerin (BCG) vaccination site, swelling of lymph nodes, hepatomegaly, and an unusually severe varicella rash at the age of 4 months. At that time, she was diagnosed with BCG histiocytosis without typical granuloma formation and was treated with antituberculous agents. During the clinical course of her illness, several different types of atypical mycobacteria and (for the first time in an IFNgammaR1-deficient patient) listeria monocytogenes were detected. Flow cytometric analysis showed that the patient's monocytes could not bind a monoclonal antibody specific for the IFNgamma-receptor. Our analysis of mRNA derived from the alpha-chain (IFNgammaR1) gene of this receptor revealed deletions of 173 bp and 4 bp in cDNA sequences originating from individual alleles. The 173 bp deletion was located between nucleotide positions 200 and 372, exactly matching those of exon 3, and the 4 bp deletion was located between nucleotide positions 561 and 564 of the coding region of the cDNA. Analysis of genomic dna revealed the presence of a G to T transition at the 5'end of the splice consensus sequence of intron 3, which explains the absence of exon 3. The other allele carried the 4-base-pair deletion (ACTC) at nucleotide positions 15-18 of exon 5. Twelve months after an allogeneic bone marrow transplantation, the patient had clinically improved.- - - - - - - - - - ranking = 1keywords = varicella (Clic here for more details about this article) |
2/59. Varicella-zoster virus vasculitis: a case of recurrent varicella without epidermal involvement.New types of diseases due to the varicella-zoster virus (VZV) are increasingly recognized. A case of cutaneous VZV vasculitis without epidermal involvement is presented. The patient received chemotherapy for a large B cell lymphoma. He presented a few painless papules on one hand and in the axilla. A lymphocytic vasculitis was evidenced. immunohistochemistry revealed the presence of VZV in endothelial cells and dermal dendrocytes. Nerves and keratinocytes were free of the virus infection. Such a presentation probably represents a mild form of recurrent varicella with prominent but limited vascular involvement.- - - - - - - - - - ranking = 6keywords = varicella (Clic here for more details about this article) |
3/59. Varicella followed by glomerulonephritis. Treatment with corticosteroids and azathioprine resulting in recurrence of varicella.The present report outlines the clinical features of a 2-year-old boy who following varicella developed purpura of the lower extremities, transient gastrointestinal bleeding and glomerulonephritis. The triad of symptoms suggests Schonlein-Henoch syndrome, but coagulation studies and renal biopsy did not confirm this, and varicella is thought to be the cause of the complications. Therapy with corticosteroids and azathioprine had only a minor effect on the nephritis but caused depression of serum IgG and specific antibody resulting in reinfection or reactivation of varicella.- - - - - - - - - - ranking = 7keywords = varicella (Clic here for more details about this article) |
4/59. Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature.Immunodeficiency with thymoma (Good syndrome, GS) is a rare, adult-onset condition that is characterized by thymoma, hypogammaglobulinemia, and low numbers of peripheral B cells. CD4 T lymphopenia and an inverted CD4:CD8 T-cell ratio may be present. Here we report 5 patients with GS and infectious complications who were seen at 3 institutions between 1983 and 1999. Three patients had recurrent sinopulmonary infections, 3 had severe cytomegalovirus (CMV) disease, and 1 had pneumocystis carinii pneumonia. review of the literature identified 46 other reports of infections in GS patients. The infections reported in all 51 patients included recurrent sinopulmonary infection (19 cases with documented respiratory pathogens), generally with encapsulated bacteria, most often haemophilus influenzae (11 cases); CMV disease (5 cases); bacteremia (7 cases); oral or esophageal candidiasis (6 cases); persistent mucocutaneous candidiasis (5 cases); chronic diarrhea (5 cases with documented stool pathogens); urinary tract infections (4 cases); P. carinii pneumonia (3 cases); tuberculosis (2 cases); Kaposi sarcoma (1 case); disseminated varicella (1 case); candidemia (1 case); wound infection with clostridium perfringens (1 case); mycoplasma arthritis (1 case); and other infections. patients with GS present with a spectrum of sinopulmonary infections and pathogens similar to common variable immunodeficiency (CVID). Compared with patients with CVID, opportunistic infections, including severe CMV disease, P. carinii pneumonia, and mucocutaneous candidiasis, appear to be more common in patients with GS, and patients with GS may have a worse prognosis. GS should be ruled out in patients with thymoma or CVID who develop severe, especially opportunistic, infections. Treatment with intravenous immune globulin is recommended for all patients with GS.- - - - - - - - - - ranking = 1keywords = varicella (Clic here for more details about this article) |
5/59. encephalitis related to primary varicella-zoster virus infection in immunocompetent children.INTRODUCTION: encephalitis is a rare complication of primary varicella-zoster virus (VZV) infection in immunocompetent children. methods: The clinical and laboratory findings of two girls with VZV-related encephalitis are reported. RESULTS: Both children presented with focal epileptic seizures, corresponding to cortical/subcortical as well as white matter lesions. The first showed a typical vesicular skin rash. She was easily diagnosed and made a rapid recovery during acyclovir and steroid treatment. In the second girl, a preceding measles-mumps-rubella virus vaccination and the absence of skin vesicles were misleading with respect to the diagnosis, which was finally proven by IgG seroconversion and intrathecal synthesis of IgG antibodies to VZV. She developed left parieto-occipital tissue necrosis and recovered only transiently during initial acyclovir/steroid treatment. Eight weeks after onset, progressive white matter demyelination and the occurrence of erythema nodosum in the lower limbs necessitated a second 4-month course of oral steroids. The VZV PCR from cerebrospinal fluid was negative in both children. CONCLUSIONS: Primary VZV infection may cause severe encephalitis that may occur without skin vesicles and lead to a chronic course with systemic vasculitis. The coincidence of vaccination and neurologic diseases offers no proof per se of a causal relationship.- - - - - - - - - - ranking = 5keywords = varicella (Clic here for more details about this article) |
6/59. Disseminated herpes simplex virus and varicella zoster virus coinfection in a patient taking thalidomide for relapsed multiple myeloma.Disseminated herpes simplex virus (HSV) and varicella zoster virus (VZV) have been reported individually in immunosuppressed adults. We present a case of coinfection with disseminated HSV and VZV infection in a patient taking thalidomide for relapsed multiple myeloma. This is the first report of opportunistic infection associated with thalidomide.- - - - - - - - - - ranking = 5keywords = varicella (Clic here for more details about this article) |
7/59. Second varicella infections: are they more common than previously thought?OBJECTIVE: To describe the epidemiology and clinical characteristics of varicella reinfections reported to a surveillance project. methods: We investigated varicella cases reported to a surveillance project between January 1, 1995, and December 31, 1999--with more extensive investigation of cases reporting previous varicella with onset between January 1, 1998, and September 30, 1998--to provide a more detailed description of first and second varicella infections. A simple decision tree was used to assess the likelihood that reported first and second infections were varicella. RESULTS: Among varicella cases reported to the surveillance project, 4.5% of cases in 1995 and 13.3% of cases in 1999 reported previous varicella. More than 95% of first infections were physician diagnosed, epidemiologically linked to another case, or had a rash description consistent with varicella; the same was true for reported second infections. People who reported reinfections were generally healthy. There was a family history of repeat infections in 45% of people who reported reinfections. CONCLUSIONS: Clinical varicella reinfections may occur more commonly than previously thought. Additional studies of the predictive value of a positive varicella history and laboratory studies of reported reinfections are indicated to guide varicella vaccination policy.- - - - - - - - - - ranking = 15keywords = varicella (Clic here for more details about this article) |
8/59. Corneal epithelial keratitis in herpes zoster ophthalmicus: "delayed" and "sine herpete". A non-contact photomicrographic in vivo study in the human cornea.PURPOSE: To investigate the origin of corneal epithelial keratitis occurring without accompanying herpes zoster ophthalmicus (HZO) cutaneous rash. methods: Corneal epithelial lesions in seven patients (four with a history of classical HZO with cutaneous rash, one of herpes zoster oticus, and two with no history of herpes zoster, were examined with the slit lamp and photographed by non-contact in vivo photomicrography. The findings were compared with lesions in classical acute HZO. polymerase chain reaction (PCR) was done in three patients. RESULTS: Slit lamp appearance, morphology at higher magnification, and kinetics of the lesions were indistinguishable from classical acute HZO. PCR was positive for varicella-zoster virus dna in all three samples. CONCLUSIONS: The findings strongly suggest that HZO typical corneal epithelial lesions occurring in the absence of cutaneous rash are in fact recurrent episodes of virus shedding.- - - - - - - - - - ranking = 1keywords = varicella (Clic here for more details about this article) |
9/59. Atypical recurrent varicella in 4 patients with hemopathies.Relapsing varicella may occur in children with hiv infection and more rarely in younger adults. Our aim was to report unusual clinical, histologic, and virologic aspects of 4 elderly patients with malignant hemopathies who had an unusual form of recurrent varicella develop. Conventional microscopy, immunohistochemistry, and in situ hybridization were applied to smears and skin biopsy specimens. The patients presented a few dozen, scattered, large, papulovesicular lesions with central crusting. No zoster-associated pain or dermatomal distribution of the lesions was noted. Conventional microscopy revealed vascular changes and epidermal alterations typical for alpha-herpes virus infection. The varicella zoster virus major viral envelope glycoproteins gE and gB, and the immediate-early varicella zoster virus IE63 protein and the corresponding genome sequence for gE were detected on Tzanck smears; they were localized in endothelial cells and keratinocytes on skin biopsy specimens. The varicella zoster virus infection in endothelial cells, the vascular involvement, and the widespread distribution of the lesions suggest that the reported eruptions are vascular rather than neural in origin. These findings invalidate the diagnosis of herpes zoster but strongly support the diagnosis of recurrent varicella in an indolent and yet unreported presentation. Furthermore, these eruptions differ from relapsing varicella in children and young adults by the age of the patients, the paucity of clinical lesions, the larger diameter of the lesions and their peculiar clinical aspect, the significantly longer time interval between primary varicella and the recurrence, the prolonged healing time of the lesions, their mild disease course, and the fact that all the lesions are in the same stage of development.- - - - - - - - - - ranking = 12keywords = varicella (Clic here for more details about this article) |
10/59. Delayed onset of varicella keratitis.Although varicella is one of the most common infectious diseases in the united states, systemic and ocular complications are rare. We report a patient who developed disciform edema followed by microdendritic keratitis 1 and 2 months, respectively, after resolution of the acute phase of varicella. Cultures were negative, but serologic analysis found positive antibodies against varicella zoster virus and negative antibodies against herpes simplex virus. Based on this case and on a review of the literature, we believe that this delayed onset of keratitis represents a distinct category of varicella corneal complications.- - - - - - - - - - ranking = 8keywords = varicella (Clic here for more details about this article) |
| | Next -> |