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Devika Gupta, Supreet Mohanty, Deepshi Thakral, Arvind Bagga, Naveet Wig, Dipendra Kumar Mitra
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) in the background of systemic lupus erythematosus (SLE) is rare. Inability to discriminate between these two entities may be fatal for the patient. Here we report two cases of SLE with secondary HLH, one of which manifested HLH as the initial presentation, and the significance of HLH's timely diagnosis. CASE REPORT We describe two cases of SLE secondarily affected by HLH, which were diagnosed by various laboratory parameters and detection of profoundly reduced NK cell activity by using flow cytometry...
October 13, 2016: American Journal of Case Reports
Imane El Mezouar, Fatima Zahra Abourazzak, Najoua Ghani, Taoufik Harzy
Adult-onset Still's disease (AOSD) is a multisystem inflammatory disease of unknown etiology. It is characterized by arthritis, hectic fever, transient rash and visceral lesions such as pleuropericarditis, lymphadenopathy and hepato splenomegaly. Although kidney involvement may appear in some cases of AOSD, minimal glomerular lesion (MGL) has not been described. We describe a female patient, who presented with multisystemic manifestations, including high spiking fever, arthralgias, striking hyperferritinemia, and proteinuria...
September 2014: Eur J Rheumatol
Moushumi Lodh, Joshi Anand Kerketta
BACKGROUND: In clinical medicine, ferritin is predominantly utilized as a serum marker of total body iron stores. In cases of iron deficiency and overload, serum ferritin serves a critical role in both diagnosis and management. Elevated serum and tissue ferritin are linked to coronary artery disease, malignancy, and poor outcomes following stem cell transplantation. Ferritin is directly implicated in less common but potentially devastating human diseases including sideroblastic anemias, neurodegenerative disorders, and hemophagocytic syndrome...
July 2012: EJIFCC
Bethina Isasi Gasser
BACKGROUND: Serum ferritin is a commonly used clinical biochemical parameter and hyperferritinemia is used as a surrogate marker for iron overload, acute or chronic inflammation, malignancy or cell death. The aim of the present study was to develop purification strategies of ferritin from sera to determine if micro-heterogeneity of serum ferritin can be used to differentiate the underlying cause of the hyperferritinemia. PATIENTS MATERIALS AND METHODS: Sera from patients with hemochromatosis, rheumatologic diseases, aceruloplasminemia, ferroportin disease or iron loading anemia have been collected and stored and ferritin purified by negative affinity followed by ion exchange and size exclusion chromatography...
August 2009: EJIFCC
Roberto Catanzaro, Biagio Cuffari, Angelo Italia, Francesco Marotta
After the first description of fatty pancreas in 1933, the effects of pancreatic steatosis have been poorly investigated, compared with that of the liver. However, the interest of research is increasing. Fat accumulation, associated with obesity and the metabolic syndrome (MetS), has been defined as "fatty infiltration" or "nonalcoholic fatty pancreas disease" (NAFPD). The term "fatty replacement" describes a distinct phenomenon characterized by death of acinar cells and replacement by adipose tissue. Risk factors for developing NAFPD include obesity, increasing age, male sex, hypertension, dyslipidemia, alcohol and hyperferritinemia...
September 14, 2016: World Journal of Gastroenterology: WJG
Piero Ruscitti, Paola Cipriani, Francesco Ciccia, Francesco Masedu, Vasiliki Liakouli, Francesco Carubbi, Onorina Berardicurti, Giuliana Guggino, Paola Di Benedetto, Salvatore Di Bartolomeo, Marco Valenti, Giovanni Triolo, Roberto Giacomelli
Macrophage activation syndrome (MAS) is a rare, life-threatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome. Due to its rarity, epidemiologic data are still lacking. Hyperferritinemia is frequently associated with MAS and might modulate the cytokine storm, which is involved in the development of multiple organ failure. In this paper, we investigated clinical data, treatments, and outcome of a homogeneous cohort of 41 adult MAS patients, complicating autoimmune rheumatic diseases...
September 21, 2016: Autoimmunity Reviews
Jérémy Flais, Edouard Bardou-Jacquet, Yves Deugnier, Guillaume Coiffier, Aleth Perdriger, Gérard Chalès, Martine Ropert, Olivier Loréal, Pascal Guggenbuhl
OBJECTIVES: Hyperuricemia is becoming increasingly frequent in the population, and is known to be sometimes the cause of gout. The impact of uric acid is still not clearly understood, however. The iron metabolism may interact with the uric acid metabolism. The aim of this study was to examine the relationship between the serum uric acid and serum ferritin levels in a cohort of hemochromatosis patients who were homozygous for the HFE p.Cys282Tyr mutation. METHODS: 738 patients with the HFE gene mutation Cys282Tyr in the homozygous state were included in the study...
September 19, 2016: Joint, Bone, Spine: Revue du Rhumatisme
Nicholas J Fordham, Richa Ajitsaria, Leena Karnik, Subarna Chakravorty
BACKGROUND: This is the first documented case of a patient with hemophagocytic lymphohistiocytosis in association with coeliac disease. There was complete clinical and biochemical remission of hemophagocytic lymphohistiocytosis following the introduction of a gluten-free diet. CASE PRESENTATION: A 7-year-old white girl presented with fevers and maculopapular rash with a recent history of tonsillitis. Blood tests revealed thrombocytopenia (64×10(9)/L), anemia (80 g/L), hypofibrinogenemia (1 g/L), and hyperferritinemia (71,378 μg/L)...
2016: Journal of Medical Case Reports
Dídac Casas-Alba, Antonio Martínez-Monseny, Laura Monfort, Carmen Muñoz-Almagro, María Cabrerizo, Àngela Deyà, Cristian Launes
Human parechovirus-3 has been associated with severe clinical manifestations in infants, such as sepsis-like illness and meningoencephalitis. Nevertheless, the vast majority of patients have a favorable outcome. We report the occurrence of this infection in dizygotic infants with extreme hyperferritinemia and a transient impairment of natural-killer cell cytotoxicity.
September 15, 2016: Pediatric Infectious Disease Journal
Oswald Moling, Andrea Piccin, Martina Tauber, Peter Marinello, Mariagrazia Canova, Marco Casini, Giovanni Negri, Bernd Raffeiner, Raffaella Binazzi, Latha Gandini, Cinzia Vecchiato, Giovanni Rimenti, Atto Billio
BACKGROUND: Silicone implants have been successfully used for breast augmentation and reconstruction in millions of women worldwide. The reaction to the silicone implant is highly variable; it can lead to local inflammatory symptoms, and sometimes to systemic symptoms and disease. Over 80 cases of anaplastic lymphoma kinase-negative anaplastic large cell lymphoma have been reported in patients with silicone breast implants and have been accepted as a new clinical entity. To the best of our knowledge, an intravascular large B-cell lymphoma associated with a silicone breast implant has not been reported previously...
2016: Journal of Medical Case Reports
Noriyuki Yamakawa, Kengo Oe, Naoichiro Yukawa, Kosaku Murakami, Ran Nakashima, Yoshitaka Imura, Hajime Yoshifuji, Koichiro Ohmura, Yasuo Miura, Naohisa Tomosugi, Hiroshi Kawabata, Akifumi Takaori-Kondo, Tsuneyo Mimori
Hereditary hemochromatosis (HH) is an inherited disorder usually seen in Northern Europeans, which results in iron overload syndrome. A few cases have also been reported in Japan. We herein report a Japanese man presenting with fever, arthritis, liver dysfunction, and hyperferritinemia who was diagnosed with type 4 HH. He was heterozygous for the 1520A>G (His507Arg) mutation in the ferroportin-1 gene (SLC40A1). He had a familial cataract as an infant, but hereditary hyperferritinemia cataract syndrome was excluded...
2016: Internal Medicine
Anna Waszczuk-Gajda, Krzysztof Mądry, Rafał Machowicz, Joanna Drozd-Sokołowska, Beata Stella-Hołowiecka, Andrzej Mital, Agata Obara, Anna Szmigielska-Kapłon, Anna Sikorska, Edyta Subocz, Wiesław W Jędrzejczak, Jadwiga Dwilewicz-Trojaczek
BACKGROUND: Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders characterized by ineffective hematopoiesis, cytopenias and a risk of progression to acute myeloid leukemia (AML). Anemia is the most frequent cytopenia diagnosed in patients with MDS. Regular RBC transfusions are the only treatment option for about 40% of patients. Transfusion-dependent patients develop secondary iron overload. The influence of serum ferritin (SF) concentration on survival and acute myeloid leukemia transformation in MDS patients remains controversial...
July 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
Uroosa Ibrahim, Muhammad N Siddique, Gautam Valecha, Masoud Asgari, Edhan Isaac, Meekoo Dhar
A 46‑year‑old obese male with a medical history of thalassemia minor presented to the emergency room with complaints of severe fatigue and jaundice worsening over two weeks. On further evaluation, the patient was found to have significant hyperbilirubinemia and transaminitis. The hospital course was further complicated by pancytopenia requiring multiple transfusions, worsening hyperbilirubinemia, severe hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia. He was also found to have splenomegaly and evidence of hemophagocytosis on bone marrow biopsy...
2016: Curēus
Norma E González, Silvia Álvarez Ponte, Mariela López, Pablo Fronti, Silvina Smith, Victor Pawluk
The secondary hemophagocytic syndrome is rare in children and even rarer associated with tuberculosis. e report the case of a patient with acquired immunodeficiency syndrome, disseminated tuberculosis and hemophagocytic syndrome. An 8-year-old girl, diagnosed with acquired immunodeficiency syndrome, was admitted due to fever, vomiting and abdominal pain. She presented abdominal distension, dehydration, tachypnea, crackles and wheezing in both lungs, anemia, thrombocytopenia and coagulopathy. She received broad-spectrum antibiotics and exploratory laparotomy was performed with appendectomy and lymph node biopsy...
October 1, 2016: Archivos Argentinos de Pediatría
Jung Sook Yeom, Ji Sook Park, Ji-Hyun Seo, Eun Sil Park, Jae-Young Lim, Chan-Hoo Park, Hyang-Ok Woo, Hee-Shang Youn, Ok Jeong Lee, Tae-Hee Han, Ju-Young Chung
We report a human parechovirus-3 (HPeV-3) infection in 2 neonates who had prolonged fever (>5 days) with palmar-plantar erythema. This distinctive rash was observed 4-5 days after fever onset, just before defervescence. Elevated aspartate aminotransferase, lactate dehydrogenase, and ferritin levels were characteristic laboratory findings in the 2 cases, suggesting tissue damage caused by hypercytokinemia. Case 1 was treated with intravenous immunoglobulin, considering the possibility of severe systemic inflammatory responses...
July 2016: Korean Journal of Pediatrics
Masayuki Ochiai, Hiroaki Kurata, Hirosuke Inoue, Koichi Tanaka, Yuki Matsushita, Junko Fujiyoshi, Yoshifumi Wakata, Kiyoko Kato, Tomoaki Taguchi, Hidetoshi Takada
BACKGROUND: The substantial risk of iron overload is not routinely monitored in most of the neonatal intensive care units (NICUs) in Japan; however, blood transfusion is an essential strategy for successfully treating preterm low-birth-weight infants. OBJECTIVE: The aim of this study was to investigate the iron status and clinical features of infants with a birth weight of <1,500 g, i.e. very-low-birth-weight infants (VLBWIs). METHODS: This prospective observational study enrolled 176 (82...
August 23, 2016: Neonatology
Licínio Manco, Celeste Bento, Bruno L Victor, Janet Pereira, Luís Relvas, Rui M Brito, Carlos Seabra, Tabita M Maia, M Letícia Ribeiro
Glucose-6-phosphate isomerase (GPI) deficiency cause hereditary nonspherocytic hemolytic anemia (HNSHA) of variable severity in individuals homozygous or compound heterozygous for mutations in GPI gene. This work presents clinical features and genotypic results of two patients of Portuguese origin with GPI deficiency. The patients suffer from a mild hemolytic anemia (Hb levels ranging from 10 to 12.7g/mL) associated with macrocytosis, reticulocytosis, hyperbilirubinemia, hyperferritinemia and slight splenomegaly...
September 2016: Blood Cells, Molecules & Diseases
Selin Aytaç, Ezgi Deniz Batu, Şule Ünal, Yelda Bilginer, Mualla Çetin, Murat Tuncer, Fatma Gümrük, Seza Özen
Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years...
October 2016: Rheumatology International
Kyung Hee Kim, Ki Young Oh
Phlebotomy is the removal of blood from the body, and therapeutic phlebotomy is the preferred treatment for blood disorders in which the removal of red blood cells or serum iron is the most efficient method for managing the symptoms and complications. Therapeutic phlebotomy is currently indicated for the treatment of hemochromatosis, polycythemia vera, porphyria cutanea tarda, sickle cell disease, and nonalcoholic fatty liver disease with hyperferritinemia. This review discusses therapeutic phlebotomy and the related disorders and also offers guidelines for establishing a therapeutic phlebotomy program...
2016: Journal of Blood Medicine
N E Karaca, G Aksu, E Ulusoy, C Cavusoglu, C Oleaga-Quintas, A Nieto-Patlan, M E Richard, C Deswarte, J L Casanova, J Bustamante, N Kutukculer
No abstract text is available yet for this article.
2016: Journal of Investigational Allergology & Clinical Immunology
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