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  1. Article ; Online: Eculizumab for paroxysmal nocturnal haemoglobinuria

    Sugat Adhikari / Surendra Sapkota / Suraj Shrestha / Kshitiz Karki / Anjan Shrestha

    Orphanet Journal of Rare Diseases, Vol 18, Iss 1, Pp 1-

    catastrophic health expenditure in Nepalese patients

    2023  Volume 4

    Abstract: Abstract Paroxysmal nocturnal hemoglobinuria (PNH) results from a mutation in the phosphatidylinositol glycan class-A gene which causes uncontrolled complement activation with resultant intravascular hemolysis and its sequelae. Eculizumab is a terminal ... ...

    Abstract Abstract Paroxysmal nocturnal hemoglobinuria (PNH) results from a mutation in the phosphatidylinositol glycan class-A gene which causes uncontrolled complement activation with resultant intravascular hemolysis and its sequelae. Eculizumab is a terminal complement inhibitor that blocks this complement activation and has revolutionized the treatment of PNH but comes with an enormous price which can have catastrophic health expenditure in low-middle income countries (LMIC) like Nepal. Here, we discuss the potential way forwards in the treatment of PNH in Nepal and other LMICs.
    Keywords Catastrophic health expenditure ; Eculizumab ; Low and middle-income countries ; Orphan drug ; Paroxysmal nocturnal hemoglobinuria ; Nepal ; Medicine ; R
    Language English
    Publishing date 2023-06-01T00:00:00Z
    Publisher BMC
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  2. Article ; Online: Undiagnosed polycythemia, an uncommon cause of Wallenberg syndrome

    Aadesh Rayamajhi / Biraj Pokhrel / Shambhu Khanal / Anjan Shrestha

    Clinical Case Reports, Vol 10, Iss 4, Pp n/a-n/a (2022)

    A case report

    2022  

    Abstract: Abstract A 26‐year‐old man presented with difficulty swallowing, dizziness, hiccups, and Horner's syndrome. Clinical and neuroimaging collaboration confirmed lateral medullary syndrome. Polycythemia was identified as the only attributable risk factor. ... ...

    Abstract Abstract A 26‐year‐old man presented with difficulty swallowing, dizziness, hiccups, and Horner's syndrome. Clinical and neuroimaging collaboration confirmed lateral medullary syndrome. Polycythemia was identified as the only attributable risk factor. However, the cause of polycythemia could not be assessed further. Polycythemia was managed with phlebotomy.
    Keywords case report ; ischemic stroke ; lateral medullary syndrome ; polycythemia ; Medicine ; R ; Medicine (General) ; R5-920
    Language English
    Publishing date 2022-04-01T00:00:00Z
    Publisher Wiley
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  3. Article ; Online: Tuberculosis with Evans syndrome

    Sagar Gyawali / Utsav Joshi / Zeni Kharel / Shambhu Khanal / Anjan Shrestha

    Clinical Case Reports, Vol 9, Iss 5, Pp n/a-n/a (2021)

    A case report

    2021  

    Abstract: Abstract Evans syndrome and tuberculosis could be predisposing factors for one another, or there may be a common pathophysiological denominator for the co‐occurrence. Further research is needed for a better understanding of pathophysiology and treatment. ...

    Abstract Abstract Evans syndrome and tuberculosis could be predisposing factors for one another, or there may be a common pathophysiological denominator for the co‐occurrence. Further research is needed for a better understanding of pathophysiology and treatment.
    Keywords autoimmune hemolytic anemia ; Evans syndrome ; tuberculosis ; Medicine ; R ; Medicine (General) ; R5-920
    Language English
    Publishing date 2021-05-01T00:00:00Z
    Publisher Wiley
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  4. Article ; Online: Multiple myeloma with plasmacytoma of the clivus bone presenting with multiple cranial nerve III, IV, and VI palsy

    Chiranjiwi Prasad Shah / Rajan Chamlagain / Sangam Shah / Subodh Paudel / Sanjit Kumar Sah / Bipin Koirala / Kamal Pandit / Sanjeeta Sitaula / Anjan Shrestha

    Clinical Case Reports, Vol 11, Iss 2, Pp n/a-n/a (2023)

    A diagnostic dilemma

    2023  

    Abstract: Abstract Central nervous system (CNS) manifestation with cranial nerve palsy in multiple myeloma (MM) is a rare manifestation. Plasmacytoma originates from the bones of the skull base in 3% patients with MM but rarely develops from the soft tissues of ... ...

    Abstract Abstract Central nervous system (CNS) manifestation with cranial nerve palsy in multiple myeloma (MM) is a rare manifestation. Plasmacytoma originates from the bones of the skull base in 3% patients with MM but rarely develops from the soft tissues of the nasal cavity and paranasal sinuses. Here, we present a case of 68‐year‐old male patient with multiple myeloma, clivus bone plasmacytoma, and cavernous sinus syndrome.
    Keywords clivus bone ; multiple myeloma ; palsy ; plasmacytoma ; Medicine ; R ; Medicine (General) ; R5-920
    Language English
    Publishing date 2023-02-01T00:00:00Z
    Publisher Wiley
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  5. Article ; Online: Hemochromatosis in a β‐thalassemia minor patient with H63D homozygous mutation

    Nishan Babu Pokhrel / Shambhu Khanal / Parikshit Chapagain / Biraj Pokhrel / Anjan Shrestha

    Clinical Case Reports, Vol 8, Iss 12, Pp 2341-

    A case report

    2020  Volume 2345

    Abstract: Abstract β‐thalassemia heterozygosity can cause significant iron overload when accompanied by HFE gene mutations and inappropriate iron supplementation. ...

    Abstract Abstract β‐thalassemia heterozygosity can cause significant iron overload when accompanied by HFE gene mutations and inappropriate iron supplementation.
    Keywords anemia ; h63d mutation ; hereditary hemochromatosis ; HFE gene ; iron overload ; β‐thalassemia ; Medicine ; R ; Medicine (General) ; R5-920
    Language English
    Publishing date 2020-12-01T00:00:00Z
    Publisher Wiley
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  6. Article ; Online: Essential Thrombocythemia among Patients with Myeloproliferative Neoplasms in Haematology Unit of a Tertiary Care Centre

    Sanjit Kumar Sah / Sangam Shah / Sansar Babu Tiwari / Basanta Sharma Poudel / Biranmol Singh / Prakash Sharma / Sunil Sharma Acharya / Hritik Murarka / Sabin Thapaliya / Anjan Shrestha

    Journal of Nepal Medical Association, Vol 60, Iss

    A Descriptive Cross-sectional Study

    2022  Volume 248

    Abstract: Introduction: Essential thrombocythemia, a myeloproliferative condition with an increased number of circulating platelets, is a rare hematological malignancy. The aim of the study is to find out the prevalence of essential thrombocythemia among patients ... ...

    Abstract Introduction: Essential thrombocythemia, a myeloproliferative condition with an increased number of circulating platelets, is a rare hematological malignancy. The aim of the study is to find out the prevalence of essential thrombocythemia among patients with myeloproliferative neoplasms presenting in haematology unit of a tertiary care centre. Methods: This was a descriptive cross-sectional study at a tertiary care centre from September, 2020 to September, 2021 (Reference number: 48 (6-11) E2077/076). All the patients with a diagnosis of essential thrombocythemia and willing to give consent were included in the study while the patients with incomplete investigations were excluded. A sample size of 72 patients was taken and convenience sampling was done. Data were entered in Microsoft Excel 2010 and analysis was done by the Statistical Package for the Social Sciences Version 22.0. Point estimate at 95% Confidence Interval was calculated along with frequency and proportion for binary data along with mean and standard deviation for continuous data. Results: Among 72 patients with myeloproliferative neoplasms, the prevalence of essential thrombocythemia was found to be 17 (23.61%) (13.80-33.42 at a 95% Confidence Interval). The mean age of patients was 55.41±11.20 years with a male to female ratio of 9:8. The mean hemoglobin level and platelet count in patients were found to be 11.20±2.1 g/dl and 677000±262067.70 cells/mm3. Twelve (70.58%) of total patients were under low risk of essential thrombocythemia while 3 (17.64%) of them were at high risk. Conclusions: The prevalence of essential thrombocythemia was similar to other studies done in similar settings.
    Keywords essential thrombocythemia ; hematology ; mutation ; Medicine (General) ; R5-920
    Subject code 610
    Language English
    Publishing date 2022-04-01T00:00:00Z
    Publisher Nepal Medical Association
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  7. Article ; Online: Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease)

    Rabindra Bhakta Pradhananga / Kripa Dangol / Anjan Shrestha / Dharma Kanta Baskota

    International Archives of Otorhinolaryngology, Vol 18, Iss 04, Pp 406-

    A Case Report and Literature Review

    2014  Volume 408

    Abstract: Abstract Introduction Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive ... ...

    Abstract Abstract Introduction Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. Objective To report a case of a 12-year-old patient with multiple sites of cervical lymphadenitis, which was diagnosed as RDD histopathologically as well as immunohistologically. Resumed Report A 12-year-old girl presented with multiple painless sites of cervical lymphadenitis without any systemic and other ear, nose, and throat manifestations. The biopsy report of the lymph node showed dilatation of the sinuses, filled with histiocytes having foamy cytoplasm. Many of the histiocytes were engulfing mature lymphocytes. The sinus histiocytes were strongly positive for S-100 protein. Conclusion RDD must be considered in the differential diagnosis of massive or multiple lymphadenopathies.
    Keywords sinus histiocytosis ; massive lymphadenopathy ; rosai-dorfman disease ; Medicine ; R ; Otorhinolaryngology ; RF1-547
    Subject code 610
    Language English
    Publishing date 2014-08-01T00:00:00Z
    Publisher Thieme Revinter Publicações Ltda.
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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