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Sahin Erdol, Mehmet Ture, Birol Baytan, Tahsin Yakut, Halil Saglam
There are published reports stating that some of the congenital metabolic diseases, such as lysinuric protein intolerance, multiple sulphatase deficiency, galactosemia, Gaucher disease, Pearson syndrome, and galactosialidosis, might lead to secondary hemophagocytic lymphohistiocytosis (HLH). However, to date, to our knowledge, the long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency has never been investigated among patients with HLH. Here, we report on a patient who was referred to our institution for a differential diagnosis of pancytopenia, liver failure, and rhabdomyolysis...
November 2016: Journal of Pediatric Hematology/oncology
Gregory Plotnikoff, Melissa Barber
INTRODUCTION: Single-disorder or single-organ-system clinical practice guidelines are often of limited usefulness in guiding effective management of patients with chronic multidimensional signs and symptoms. The presence of multiple long-standing medical problems in a given patient despite intensive medical effort suggests that addressing systemic core imbalances could complement more narrowly focused approaches. CASE PRESENTATION: A 72-year-old man experiencing longstanding depression, fatigue, irritable bowel syndrome, and chronic pain in the context of additional refractory illnesses was assessed and treated, guided by a system-oriented approach to underlying core imbalances termed functional medicine...
October 14, 2016: Permanente Journal
Arun Agarwal, Samiksha Sharma, Mala Airun
Selective immunoglobulin M deficiency(sIgMD) is a rare form of dysgammaglobulinaemia characterized by an isolated low level of serum immunoglobulin M (IgM). It was an incidence of less than 0.03% in the general population and 1% in hospitalized patients. sIgMD may occur as a primary or secondary condition. sIgMD is much more common than primary .Hemophagocytic lymphohistiocytosis (HLH) is also a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes and can be primary or secondary, characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations...
July 2016: Journal of the Association of Physicians of India
Laetitia Piane, Marie-Laure Théron, Marcel Aumann, Catherine Trumel
A 9-year-old, female Maltese dog was referred to the Veterinary School of Toulouse with a 2-day history of anorexia and weakness. On clinical examination, the dog had hyperthermia (39.7°C), abdominal discomfort, and polypnea. Significant laboratory findings included pigmenturia, hyperbilirubinemia, hypercreatininemia, hyperfibrinogenemia, abnormal Snap canine pancreas-specific lipase, and pancytopenia with a nonregenerative anemia. A peripheral blood smear revealed numerous intraerythrocytic large Babesia but no polychromasia...
October 18, 2016: Veterinary Clinical Pathology
Mohamed Gaber, Anselm Wong, Zeff Koutsogiannis, Shaun L Greene
No abstract text is available yet for this article.
December 2016: European Journal of Emergency Medicine: Official Journal of the European Society for Emergency Medicine
Ruixue Miao, Zhiling Wang, Qin Guo, Yang Wen, Qiong Liao, Yu Zhu, Min Shu, Chaomin Wan
BACKGROUND: Visceral Leishmaniasis (VL) is a life-threatening parasitic infection transmitted by phlebotomine sandflies. We undertook this study to analyze the clinical features of pediatric VL in a population of Chinese children. METHODS: A retrospective study was performed with pediatric patients (≤14years) diagnosed with VL based on bone-marrow biopsy, serology and diagnosis based on clinical manifestation and the improvement after the experimental drug when negative bone marrow and serology results were shown...
September 30, 2016: Pediatric Infectious Disease Journal
Corey J Langer, Shirish M Gadgeel, Hossein Borghaei, Vassiliki A Papadimitrakopoulou, Amita Patnaik, Steven F Powell, Ryan D Gentzler, Renato G Martins, James P Stevenson, Shadia I Jalal, Amit Panwalkar, James Chih-Hsin Yang, Matthew Gubens, Lecia V Sequist, Mark M Awad, Joseph Fiore, Yang Ge, Harry Raftopoulos, Leena Gandhi
BACKGROUND: Limited evidence exists to show that adding a third agent to platinum-doublet chemotherapy improves efficacy in the first-line advanced non-small-cell lung cancer (NSCLC) setting. The anti-PD-1 antibody pembrolizumab has shown efficacy as monotherapy in patients with advanced NSCLC and has a non-overlapping toxicity profile with chemotherapy. We assessed whether the addition of pembrolizumab to platinum-doublet chemotherapy improves efficacy in patients with advanced non-squamous NSCLC...
October 10, 2016: Lancet Oncology
Lindert Benedictus, Charlotte R Bell
Bovine neonatal pancytopenia (BNP) is a hemorrhagic disease that emerged in calves across Europe in 2007. Its occurrence is attributed to immunization of the calf's mother with a vaccine produced using an allogeneic cell line. Vaccine-induced alloantibodies specific for major-histocompatibility class I antigens are transferred from the mother to the calf via colostrum, leading to profound depletion of peripheral blood and bone marrow cells that is often fatal. Areas covered: Pubmed and Web of Science were used to search for literature relevant to BNP and the use of allogeneic vaccine cell lines...
October 17, 2016: Expert Review of Vaccines
Venkatesh Krishnamurthy Tekur
Methotrexate forms one of the main drugs in the pharmacological management of rheumatoid arthritis, psoriasis, and some neoplastic diseases. Methotrexate rarely causes cutaneous ulceration and most cases are reported in patients with psoriasis and have been accompanied by pancytopenia. The author here reports occurrence of multiple (two) cutaneous ulcers due to methotrexate in a nonpsoriatic patient. The patient was on methotrexate for seronegative rheumatoid arthritis for 10 years. To the best of the Author's knowledge, this is a rare case of cutaneous ulceration due to methotrexate in a nonpsoriatic patient reported in the literature so far, and probably one of its kind without pancytopenia or other hematological abnormalities...
September 2016: Indian Dermatology Online Journal
Nagappa H, Shasthara P
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
M S Vaidya, S A Khandagale, Basant K Yadav
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Sunu Alex, Madhumathi, Tharun Tom
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Mohanty Bijaya, Prasad S K, Mohanty Rajesh, Singh Ritu, Kumari Sareeta
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Divendu Bhushan
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Yoshiaki Takahashi, Toshiharu Matsuura, Yusuke Yanagi, Koichiro Yoshimaru, Tomoaki Taguchi
BACKGROUND/PURPOSE: There is currently no unified view regarding whether liver transplantation or splenectomy should be performed for hypersplenism before liver transplantation in biliary atresia (BA) patients. We herein describe the efficacy of splenectomy before liver transplantation. METHODS: Splenectomy was performed in ten patients with hypersplenism associated with BA. We retrospectively reviewed their perioperative and postoperative courses, the number of leukocytes and thrombocytes, and the MELD score...
September 26, 2016: Journal of Pediatric Surgery
Yuichi Katanami, Satoshi Kutsuna, Atsuko Horino, Takehiro Hashimoto, Yoshikazu Mutoh, Kei Yamamoto, Nozomi Takeshita, Kayoko Hayakawa, Shuzo Kanagawa, Yasuyuki Kato, Norio Ohmagari
Melioidosis, an infectious disease with high mortality, caused by Burkholderia pseudomallei, is endemic in southeast Asia and northern Australia. In Indonesia, autochthonous cases have been rarely reported, with most cases being sporadic and occurring in travelers. Herein, we report a fatal case of neurological melioidosis in a traveler from Indonesia presenting with septic shock.
October 5, 2016: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
Frederike Reichmann, Annette Pfitzner, Guenter Rademacher, Elke Schwedinger, Klaus Cussler, Carola M Sauter-Louis
BACKGROUND: Several research groups from different European countries have worked on the aetiopathogenesis of bovine neonatal pancytopenia (BNP) and an association between the use of the vaccine PregSure BVD (Pfizer, Germany) and the development of this haemorrhagic disease was confirmed. Because BNP is not a notifiable disease, it is difficult to obtain information on its incidence. Based on pharmacovigilance (PhV) data, which are the only officially available data at the national level, the incidence of BNP is considered low...
October 7, 2016: BMC Veterinary Research
Ji Won In, Nuri Lee, Eun Youn Roh, Sue Shin, Kyoung Un Park, Eun Young Song
OBJECTIVES: Aplastic anemia (AA) is characterized by pancytopenia and bone marrow failure, and most acquired AA is an immune-mediated disorder. Regulatory T cells (Tregs) suppressing autoreactive T cells were decreased in AA patients. FoxP3 is a major regulator for the development and function of Tregs. Polymorphism in FoxP3 was shown to be associated with various autoimmune diseases, however, has not yet been studied in AA. In this study, we examined the association between FoxP3 polymorphisms and AA in Korean patients...
October 5, 2016: Hematology (Amsterdam, Netherlands)
Maria Fernanda Pascutti, Martje N Erkelens, Martijn A Nolte
The ability of the bone marrow (BM) to generate copious amounts of blood cells required on a daily basis depends on a highly orchestrated process of proliferation and differentiation of hematopoietic stem and progenitor cells (HSPCs). This process can be rapidly adapted under stress conditions, such as infections, to meet the specific cellular needs of the immune response and the ensuing physiological changes. This requires a tight regulation in order to prevent either hematopoietic failure or transformation...
2016: Frontiers in Immunology
K Rakul Nambiar, Sreejith G Nair, R Nandini Devi
Acute myeloid leukemia (AML) presents with symptoms related to pancytopenia (weakness, infections, bleeding diathesis) and organ infiltration with leukemic cells. Galactorrhea is an uncommon manifestation of AML. We report a case of AML presenting with galactorrhea.
October 2016: Proceedings of the Baylor University Medical Center
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