kostman syndrome

OBJECTIVE: The aim of this study was to assess the susceptibility of HIV to two HIV monoclonal antibodies (bnAbs), 3BNC117 and 10-1074, in individuals with chronically suppressed HIV infection. DESIGN: The susceptibility of bnAbs was determined using the PhenoSense mAb Assay, which is a cell-based infectivity assay designed to assess the susceptibility of luciferase-reporter pseudovirions. This assay is the only CLIA/CAP compliant screening test specifically developed for evaluating bnAb susceptibility in people with HIV infection...

We report on the post-hoc analysis of three clinical studies (NCT01935089, NCT00594880, and NCT00051818) with chronically HIV-infected, immune-reconstituted individuals with similar entry criteria, and demographics interrupting antiretroviral therapy (ART) without or with 5 weeks of weekly pegylated (Peg)-IFN-α2b or Peg-IFN-α2a immunotherapy added onto ART. Results show similar rates of viral suppression between both immunotherapies when continued during a 4-week ART interruption, despite Peg-IFN-α2a maintaining significantly higher trough blood levels...

In the pilot NCT01935089 trial, we tested whether pegylated interferon alpha2b (Peg-IFN-α2b) with antiretroviral therapy (ART) was safe and could impact HIV and immune measures in blood and in gut-associated lymphoid tissue (GALT). Twenty HIV-1+ ART-suppressed individuals received 1 μg/kg/week Peg-IFN-α2b with ART for 20 weeks, with intermediate 4-week analytical ART interruption (ATI). Safety, immune activation, HIV viral load and integrated HIV DNA in blood, and HIV RNA and DNA in gut biopsies were measured...

Antimicrobial peptides play an important role in the innate immune response. Deficiency in salivary LL-37 antimicrobial peptide has been implicated in periodontitis in patients with morbus Kostman syndrome. Down syndrome is associated with periodontitis, diminished salivary flow, and salivary immunoglobulin deficiency. In the present study, levels of LL-37 and its hCAP18 precursor were measured in saliva samples from young individuals with Down syndrome and compared with levels in those from age-matched healthy controls...

The development of body weight gain and lipodystrophy due to antiretroviral therapy may lead to disturbances in sleep, particularly the obstructive sleep apnoea (OSA) syndrome. A retrospective review of the medical records of consecutively identified HIV-infected subjects who were diagnosed with OSA by overnight polysomnography between January 1, 2003 and December 31, 2004 was performed. Twelve HIV-infected subjects with OSA confirmed by polysomnography (total apnoea/hypopnoea index > or = 5) were identified...

BACKGROUND/AIMS: Kostmann syndrome, or severe congenital neutropenia, is an autosomal recessive disease of neutrophil production and is associated with severe periodontal pathology. The aim of this study was to determine whether human cytomegalovirus (HCMV) and Epstein-Barr virus (EBV) contribute to the pathogenesis of Kostmann syndrome periodontitis. METHODS: Supragingival plaque and saliva samples were taken from a 6-year-old boy and his 3-year-old sister suffering from Kostmann syndrome, and from two age- and gender-matched healthy children serving as controls...

OBJECTIVE: To describe recruitment and baseline epidemiologic characteristics of volunteers in the first phase 3 placebo-controlled trial of a recombinant gp120 HIV vaccine (AIDSVAX B/B). METHODS: Volunteers were gay/bisexual men or women at risk for sexually transmitted HIV infection. Recruitment strategies, demographics, and risk factors were assessed. HIV status was determined by standard HIV-1 antibody assays. Seronegative/viremic HIV infection at enrollment was determined using the HIV-1 nucleic acid test...

Hypereosinophilia, defined as an absolute eosinophil count exceeding 1,500/mm(3), is caused by a limited number of disorders. Its association with malignancy is a rare occurrence that typically denotes extensive metastasis and a poor prognosis. Several theories have been proposed to explain the hypereosinophilia associated with malignancy, focusing on the production of eosinophilopoietic cytokines by tumors. We describe a patient with hypereosinophilia associated with a cardiac rhabdomyosarcoma, review the etiologies of hypereosinophilia, and discuss the possible mechanisms...

BACKGROUND: Congenital neutropenia is characterized by an almost total absence of neutrophils and increased susceptibility to infection. Oral manifestations include ulcerations of mucous membranes, acute gingival inflammation with focal necrosis, and rapid loss of attachment. Treatment with recombinant human granulocyte colony-stimulating factor (rhG-CSF) increases neutrophil numbers and ameliorates the periodontal status. METHODS: We report the treatment of a 22-year-old male with congenital neutropenia (Kostman syndrome), referred to us due to periodontal disease, and the effect of treatment on peripheral neutropenia...

Interleukin-12 (IL-12) is a potentially critical factor in the immune response against human immunodeficiency virus (HIV) because it is important for regulating proliferation and interferon-gamma (IFN-gamma) production by T cells and natural killer (NK) cells, antigen presentation and accessory cell function by macrophages and dendritic cells, and cytolytic activities of cytotoxic T-lymphocyte cells and NK cells, which are all functions known to be dysfunctional in patients with acquired immune deficiency syndrome...

PURPOSE: To describe nodular lymphangitis by reviewing the clinical and epidemiologic features of this disease with an emphasis on distinguishing specific etiologic agents. DATA SOURCES: English-language articles were identified through a MEDLINE search (1966 to September 1992) using sporotrichosis, lymphangitis, and sporotrichoid as key words; additional references were selected from the bibliographies of identified articles. In addition, three new patients with nodular lymphangitis are described...

BACKGROUND: There has been a recent resurgence of severe infections with Streptococcus pyogenes, including bacteremias and a toxic shock-like syndrome. The purpose of this study was to examine the influence of intravenous drug use on the incidence, clinical epidemiology, and severity of S pyogenes bacteremia. METHODS: We conducted a retrospective survey of medical records at a 500-bed tertiary care urban hospital and at a nearby 500-bed suburban university-affiliated hospital...

Morphological changes of the thymus, tonsils, lymph nodes, spleen, and other organs in two observations of severe combined insufficiency (SCIN) in infants of the first year of life are described. In both cases there was hypoplasia of the thymus with the lack or occasional thymic bodies and poor content of thymocytes in the lobules. In one infant, hypoplasia of the thymus was combined with intestinal angiomatosis which suggested the syndrome of ataxia-teleangiectasia. In the other infant, SCIN was associated with severe granulocytopenia of the Kostman type...

Children with congenital neutropenia (Kostman disease) present a syndrome of recurrent and eventually fatal bacterial infections. The disease is uniformly lethal and the mean age at death is 24 months. To attempt to better understand the nature of this inborn error, we have studied marrow and peripheral blood of 3 children with the disease. Along with classic morphologic analysis we have used the technic of soft agar bone marrow culture in vitro. All 3 patients showed marked deficiency of numbers of neutrophils in the peripheral blood, intermittent monocytosis especially with infection, and predominant nitroblue tetrazolium (NBT) dye staining in monocytes during infection...