19 research outputs found

    Haemophagocytic lymphohistiocytosis in patients with human immunodeficiency virus infection: to treat or not to treat

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    Haemophagocytic lymphohistiocytosis (HLH) in Human Immunodeficiency Virus (HIV) infected individuals can either be due to the disease itself or due to associated infections/malignancies. The treatment for HLH requires immunosuppressive therapy but administering immunosuppressive therapy to an already immunosuppressed patient (HIV infection) is complex. We present two such cases of HLH in patients infected with HIV. In the first case, no alternate cause for HLH was found even after extensive investigations and it was attributed to the uncontrolled HIV replication. Patient was started on dexamethasone for the same but succumbed to hospital acquired pneumonia. The second patient was diagnosed with Hodgkin's lymphoma but he succumbed to his illness before initiating immunosuppressive therapy for HLH. We report these cases to highlight the dilemma and a need for further research in this direction

    Fatal familial hemophagocytic lymphohistiocytosis with perforin gene (PRF1) mutation and EBV-associated T-cell lymphoproliferative disorder of the thyroid

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    Familial hemophagocytic lymphohistiocytosis (FHL) is a rare fatal autosomal recessive disorder of immune dysregulation. The disease presents most commonly in the first year of life; however, symptomatic presentation throughout childhood and adulthood has also been identified. Biallelic mutation in the perforin gene is present in 20%–50% of all cases of FHL. Secondary hemophagocytic lymphohistiocytosis (HLH) in association with hematological malignancies is known; however, whether mutations in HLH-associated genes can be associated with FHL and hematolymphoid neoplasms is not well documented. Also, Epstein–Barr-virus- (EBV) positive systemic T-cell lymphoproliferative disease (SE-LPD) in the setting of FHL is not clearly understood. Here, we present the case of a young boy who presented with typical features of childhood FHL harboring the perforin gene (PRF1) mutation, and had SE-LPD diagnosed on autopsy, along with evidence of recent EBV infection. The patient expired due to progressive disease. Five siblings died in the second or third decade of life with undiagnosed disease. Genetic counseling was provided to the two surviving siblings and parents, but they could not afford genetic testing. One surviving sibling has intermittent fever and is on close follow-up for possible bone marrow transplantation

    National hepatitis registry in Pakistan: a dire need for hepatitis surveillance and control

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    Abstract Hepatitis is a major public health issue in Pakistan, with an estimated 11.55% prevalence of HCV infection in the adult population. The country ranks second globally in terms of hepatitis C virus (HCV) infections, with approximately one in every 20 Pakistanis already infected. The mortality rates due to HBV and HCV stand at 563,000 and 366,000 annually, respectively. However, the absence of a national registry or database system and the lack of coordination among provinces pose significant obstacles in combating this disease effectively. To address this issue, the establishment of a centralized national database registry is crucial, allowing comprehensive analysis, tracking of hepatitis prevalence, and identification of high-risk areas for targeted interventions. By fostering collaboration among provinces, the government, and non-governmental organizations, the registry would facilitate joint decision-making, minimize duplication of efforts, and address inconsistencies in diagnosis and treatment. Collaborating with student-run organizations and leveraging enhanced laboratory capacities post-COVID era can strengthen the hepatitis control program. The centralized approach and unified efforts are necessary to achieve the goal of a hepatitis-free Pakistan, where a healthier future can be realized

    Feasibility Study of Expanded Clay Aggregate Lightweight Concrete for Nonstructural Applications

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    In nonstructural infill panels, common materials like expanded polystyrene panels face fire susceptibility, autoclaved aerated concrete (AAC) incurs high production costs, and traditional bricks come with a significant carbon footprint and weight. So, there is a requirement for infill panels that are not just resilient and lightweight but sustainable as well. This study seeks to address these issues by introducing sustainable and lightweight expanded clay aggregate (ECA) in concrete. Firstly, eight ECA mix designs were prepared by integrating fly ash and kerosene with clay, and ECA with a bulk density of 0.59 g/cm³ and compressive strength of up to 1.73 MPa were prepared. The lightest ECA mix was then chosen to explore their use in lightweight aggregate concrete (LWAC) along with fly ash as a secondary cementitious material. The resulting LWAC had a minimum density of 1,050 kg/m³ and a compressive strength of 6.8 MPa, fulfilling the standard requirements of a minimum of 3.5 MPa for nonstructural concrete

    Identification of Three Novel QTLs Associated with Yellow Rust Resistance in Wheat (<i>Triticum aestivum</i> L.) Anong-179/Khaista-17 F<sub>2</sub> Population

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    Wheat yellow rust (YR) caused by Puccinia striiformis is lethal for the leaf photosynthetic process, which substantially affects yield components and ultimately causes drastic yield reduction. The current study aimed to identify all-stage YR resistance linked QTLs in the best cross-combination. Experimental materials were phenotyped for disease severity in YR-hot spot area at Cereal Crops Research Institute, Pirsabak Pakistan in Khyber Pakhtunkhwa province in 2019 and 2020 and 2020 and 2021 Rabi seasons. The AN179 × KS17 was found to be the best cross combination, which showed high resistance to YR, whereas crosses AN179 × PK15 and PR129 × PK15 demonstrated susceptibility to YR with high disease severity. The recombinant inbred lines (RIL) F2 wheat population Annong-179/Khaista-17 demonstrated highly desirable YR resistance and yield component traits. Simple sequence repeat (SSR) markers were used to genotype the RIL population and their parents. Three novel QTLs linked to all-stage YR resistance were found on chromosomes 2BS, 3BS and 6BS, which explained 1.24, 0.54, and 0.75 phenotypic variance, respectively. Incorporation of the newly identified novel YR-resistance associated QTLs into hybridization wheat breeding program could be effective for marker-assisted selection of the improved and sustainable resistance
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