Immunedeficiencies

Sickle cell disease patients are prone to infection and overwhelming sepsis because an immunedeficient state arises from asplenia (autosplenectomy/surgical splenectomy) and functional hyposplenism. The common pathogen encountered in sepsis with asplenic/hyposplenism patients is encapsulated organism, gram-negative bacilli, but in developing countries like India, there are many possibilities of infection by an uncommon organism that make it difficult to diagnose. Here, we have described a case of sickle cell disease presented with persistent fever and later, found to have an atrophic spleen with involvement of respiratory system and osteoarticular system...

Numerous animal models of colitis have provided important insights into the pathogenesis of inflammatory bowel disease (IBD), contributing to a better understanding of the underlying mechanisms for IBD. As aberrant CD4+ T cell responses play a critical role in the pathogenesis and development of IBD, T cell adoptive transfer models of colitis have become a valuable tool in investigating the immunopathogenesis of intestinal inflammation. While the adoptive transfer of CD4+ CD45RBhi T cells into immunedeficient recipient mice was the first discovered and is currently the most widely used model, several variations of the T cell transfer model have also been developed with distinct features...

AIM: We compared soluble transferrin receptors (sTfR), serum ferritin, mean cell volume (MCV) of red cells and the sTfR-ferritin index with the intensive method bone marrow trephine (BMT) iron stores in the diagnosis of iron deficiency anaemia (IDA) in HIV-positive hospitalised participants. METHODS: In this cross-sectional study, we recruited hospitalised Human Immunedeficiency Virus(HIV)-positive and coronavirus of 2019 (COVID-19)-negative adults with anaemia who required a bone marrow examination as part of their diagnostic workup...

Skin cancers represent the most common human tumors with a worldwide increasing incidence. They can be divided into melanoma and non-melanoma skin cancers (NMSCs). NMSCs include mainly squamous cell (SCC) and basal cell carcinoma (BCC) with the latest representing the 80% of the diagnosed NMSCs. The pathogenesis of NMSCs is clearly multifactorial. A growing body of literature underlies a crucial correlation between skin cancer, chronic inflammation and immunodeficiency. Intensity and duration of immunodeficiency plays an important role...

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Epstein-Barr virus infection is the most common viral latent infection in humans and represents one prototypical model to study immunity to viral infections. In that respect, inborn errors of immunity (IEIs) or primary immunodeficiencies (PIDs) predisposing to severe and chronic EBV infections provide peculiar examples to decipher-specific molecular and cellular components involved in the immune control of EBV-infected cells. Herein, we discuss the recent knowledge and concepts arising from these studies, with a particular focus on 'atypical' EBV infections when EBV enters T, NK and smooth muscle cells, instead of the common 'typical' infection of B cells...

With significant advancement in the tools and strategies available for diagnosis and management, there is an expected change in the epidemiological profile of patients living with HIV/AIDS (Human immunodeficiency syndrome/Acquired immunedeficiency syndrome). We retrospectively analyzed the changing epidemiological pattern of HIV infection over a period of 13 years in the anti-retroviral (ART) center of a tertiary care hospital in India. The study included a total of 9419 patients (8811 adults and 608 children) who were registered at our ART center between 2005 and 2017...

Sütçü M, Aktürk H, Gülümser-Şişko S, Acar M, Erol OB, Somer A, Bilgiç B, Salman N. Granulomatous amebic encephalitis caused by Acanthamoeba in an immuncompetent child. Turk J Pediatr 2018; 60: 340-343. Acanthamoeba may lead to granulomatous amebic encephalitis (GAE) with high mortality rates generally in patients with immunosupression and/or chronic disease. Here, we present a rare GAE case, who was a previously healthy child. A Georgian 9 year old boy presented with focal seizure on his left arm and confusion...

Composition of the gut microbiota has been linked with human immunedeficiency virus (HIV)-infected patients on antiretroviral therapy (ART). Evidence suggests that ART-treated patients with poor CD4+ T-cell recovery have higher levels of microbial translocation and immune activation. However, the association of the gut microbiota and immune recovery remains unclear. We performed a cross-sectional study on 30 healthy controls (HC) and 61 HIV-infected individuals, including 15 immunological ART responders (IRs), 20 immunological ART non-responders (INRs) and 26 untreated individuals (VU)...

BACKGROUND: There is a strong rationale to pursue the use of neonatal porcine islets (NPIs) as an unlimited source of islets for clinical xenotransplantation. Since NPIs are composed of immature insulin producing beta (ß) cells and ductal precursor cells, they provide an ideal model to examine culture conditions to enhance ß cell proliferation and/or ß cell neo-formation from ductal cells. In an attempt to optimize the potential of NPIs as a source of ß cell grafts, we utilized an in vitro differentiation protocol and measured its effect on the functional maturation and differentiation of NPIs...

Kawasaki Disease (KD) is a systemic autoimmune vasculitis that affects small and medium sized vessels. Main complication of Kawasaki Disease is coronary artery aneurism, which has higher risk in case of delayed diagnosis and treatment. Although, complete and incomplete KD cases in different types of immune deficiency diseases have been presented up to date, clinical course of KD in patients with hypogammaglobulinemia (HG) has not been reported. Herein, a case diagnosed as incomplete KD in a child with transient HG of infancy has been reported...

Hepatocellular carcinoma (HCC) is the most commonly diagnosed malignancy of the liver. A more thorough understanding of HCC pathogenesis will provide novel targets for development of cancer drugs to effectively treat HCC. To further this goal, we carried out a proteomic profiling of HCC cell lines Huh-7.4 and Huh-7.5. These two cell lines were derived from subgenomic HCV RNA-replicating Huh-7 cells upon clearance of HCV RNA by antiviral drug treatment. Initially, the tumorigenicity of each cell line was determined and compared in parallel in the same immunedeficient mice...

To obtain background data of NOD/Shi-scid IL-2Rγnull (NOG) mice, severely immunedeficient mice, a total of 120 animals were examined at 7, 26 and 52 weeks-old (20 mice/sex/group). The survival rate at 52 weeks-old was 95% (19/20) in both sexes. Clinically, circling behavior in one direction along the cage wall was observed in males after 8 weeks and females after 47 weeks-old, and hunchback position was found in males after 32 weeks-old. Hematologically, lymphocyte count markedly decreased at all ages, while white blood cell count increased in several mice at 52 weeks-old...

We report an HIV-negative, 55-year-old manwith recurrent Kaposi's sarcoma (KS) of the lowerextremities, who does not fit into any of thefour previously described variants of KS: classicKS, AIDS-related KS, iatrogenic KS, and AfricanendemicKS. There are reports in the literature ofchildhood-onset KS, which is thought to be dueto an inherited immune deficiency that confers ahigher susceptibility to human Herpesvirus-8 (HHV-8), which is the virus that is known to cause KS. Ourpatient may be affected with an inherited immunedeficiency that has predisposed him to KS, and thismutation also may account for his prostate andbladder cancer...

Germinotropic lymphoproliferative disorders were previously described as localized disorders associated with coinfection by human herpes virus 8 and Epstein-Barr virus and characterized by good clinical outcome. We report the clinical, morphological, phenotypical, and molecular features of three cases of a hitherto unreported variant of Epstein-Barr virus (EBV)-positive, human herpes virus 8 (HHV8)-negative large B cell lymphoma with exclusive intrafollicular localization. All cases occurred in elderly individuals (63, 77, and 65 years old; one male, two females) without obvious immunedeficiency, who presented with high stage disease...

Although, erythema nodosum is a common skin manifestation associated with syphilis, nodular vasculitis is a rare feature. Here, we describe a case of a 22-year-old, human immunedeficiency virus negative, non-immunocompromised man who developed recurrent oral and scrotal ulcers with nodular lesions of the lower extremitie. Behçet's disease was initially suspected, however, his serologic test for syphilis was positive, and he was thus diagnosed with secondary syphilis, with a skin biopsy showing nodular vasculitis...

BACKGROUND: Mitochondrial DNA (mtDNA) encodes key proteins of the electron transfer chain (ETC), which produces ATP through oxidative phosphorylation (OXPHOS) and is essential for cells to perform specialised functions. Tumor-initiating cells use aerobic glycolysis, a combination of glycolysis and low levels of OXPHOS, to promote rapid cell proliferation and tumor growth. Glioblastoma multiforme (GBM) is an aggressively malignant brain tumor and mitochondria have been proposed to play a vital role in GBM tumorigenesis...

Primary (inborn) immunodeficiency is caused by gene defects that impact both the innate and the adaptive immune system. Individuals with an immunedeficiency primarily come to medical attention with recurrent infections. Most diagnoses are first made in childhood and include cellular immunodeficiency, defects of phagocyte function and other primary immunodeficiencies. Antibody deficiencies, particularly common variable immunodeficiency (CVID) and complement defects may, however, not become manifested until adulthood...

BACKGROUND: Development of high affinity and specificity molecular imaging probes that increase accuracy for early detection of lymph node (LN) metastases is important for improving survivorship in prostate cancer. We evaluated the specificity, sensitivity, and accuracy of fluorescence-labeled bombesin (BBN) peptides to detect LN and systematic metastases in orthotopic mouse models bearing gastrin releasing peptide receptor (GRPR)-positive human prostate cancer. METHODS: PC-3 cells were orthotopically implanted in severe combined immunedeficient or thymic nude (nu/nu) male mice...

BACKGROUND: Laos has a high prevalence of tuberculosis (TB) and a slowly increasing prevalence of human immunodeficiency virus/acquired immunedeficiency syndrome (HIV/AIDS). Sputum smear microscopy is the only method currently available for routine screening of pulmonary TB, although it only detects one in three cases among persons living with HIV (PLWH). Bleach treatment of sputum samples (bleach method) has been shown to significantly improve the sensitivity of the test; however, its effectiveness in PLWH remains to be determined in Laos...