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https://www.readbyqxmd.com/read/29157612/tafro-syndrome
#1
REVIEW
Takuro Igawa, Yasuharu Sato
TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD...
February 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29154419/clinical-profiles-and-prognosis-of-acute-heart-failure-in-adult-patients-with-dystrophinopathies-on-home-mechanical-ventilation
#2
Abdallah Fayssoil, Rabah Ben Yaou, Adam Ogna, France Leturcq, Olivier Nardi, Bernard Clair, Karim Wahbi, Frederic Lofaso, Pascal Laforet, Denis Duboc, David Orlikowski, Djillali Annane
AIMS: Duchenne muscular dystrophy (DMD) is characterized by respiratory and heart involvements. In the context of permanently wheelchair bound and on mechanical ventilation (MV) patients, the clinical presentation of acute heart failure (AHF) syndrome may be atypical. We sought to describe clinical and genetic profiles and to determine prognosis of DMD and Becker muscular dystrophy (BMD) patients on home MV (HMV), hospitalized for AHF. METHODS AND RESULTS: We included genetically proven DMD and BMD patients on HMV admitted for AHF...
November 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/29145282/autosomal-dominant-polycystic-kidney-disease-combined-with-hypertrophic-cardiomyopathy-a-case-report
#3
Yingjing Shen, Chenggang Xu
INTRODUCTION: This report describes the novel sampling of autosomal dominant polycystic kidney disease (ADPKD) combined with hypertrophic cardiomyopathy (HCM). SYMPTOMS AND CLINICAL FINDINGS: A 48-year-old Chinese man presented with anasarca, hypourocrinia, gross hematuria, and weight gain by 10 kg subsequently developed acute kidney injury after struck by acute respiratory distress syndrome, really a threat to his heart. DIAGNOSES: Abdominal ultrasound revealed multiple small cysts in both kidneys, with the right kidney measuring 11...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29123888/cardiac-arrest-caused-by-rapidly-increasing-ascites-in-a-patient-with-tafro-syndrome-a-case-report
#4
Masatoshi Okumura, Atsushi Ujiro, Yasunori Otsuka, Hiroshi Yamamoto, Sho Wada, Hirofumi Iwata, Toshiaki Kan, Seiji Miyauchi, Atsushi Hashimoto, Yuko Sato, Yoshihito Fujita, Yoshihiro Fujiwara, Hideki Shimaoka
Case: Thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly (TAFRO) syndrome is a newly defined systemic inflammatory disorder with gradual progression of symptoms. A 59-year-old man with fever and ascites of unknown cause developed sudden-onset shock and respiratory failure in the general ward. Cardiac arrest immediately followed. Although he was resuscitated, frequent administration of adrenaline was required to maintain his blood pressure. His circulation was most effectively stabilized by drainage of fluid from his distended abdomen...
July 2017: Acute Medicine & Surgery
https://www.readbyqxmd.com/read/29021431/a-case-of-tocilizumab-resistant-tafro-syndrome-complicated-by-type-ii-respiratory-failure
#5
Tatsuya Aoki, Mikio Wada, Atsushi Kawashima, Kouichi Hirakawa, Akihiro Nagata, Keizo Kagawa
A 66-year-old man with a several year history of thrombocytopenia, pleural effusion and ascites, anasarca, and organomegaly presented with general fatigue, appetite loss, dyspnea with type II respiratory failure. The precise history of the patient and the re-evaluation of lymph node and bone marrow biopsies conducted by the previous physician indicated TAFRO syndrome. The patient's laboratory data improved for a year with tocilizumab, but then worsened to the point that the patient required artificial ventilation due to the deterioration of type II respiratory failure...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29018798/tafro-syndrome-in-caucasians-a-case-report-and-review-of-the-literature
#6
Céline Louis, Sandrine Vijgen, Kaveh Samii, Yves Chalandon, Louis Terriou, David Launay, David C Fajgenbaum, Jörg D Seebach, Yannick D Muller
BACKGROUND: TAFRO syndrome has been reported in Japan among human herpesvirus 8 (HHV-8)-negative/idiopathic multicentric Castleman's disease (iMCD) patients. To date, the majority of iMCD patients with TAFRO syndrome originate from Japan. CASE PRESENTATION: Herein, we report a 67-year-old HIV/HHV-8-negative Caucasian iMCD patient diagnosed with TAFRO. He presented with marked systemic inflammation, bicytopenia, terminal renal insufficiency, diffuse lymphadenopathies, and anasarca...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28984775/clinicopathological-features-of-progressive-renal-involvement-in-tafro-syndrome-a-case-report-and-literature-review
#7
REVIEW
Mari Tanaka, Hiraku Tsujimoto, Kojiro Yamamoto, Saeko Shimoda, Kazumasa Oka, Hiroya Takeoka
RATIONALE: TAFRO syndrome is a systemic inflammatory disease characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, MyeloFibrosis, Renal dysfunction, and Organomegaly. Progressive renal insufficiency is a predominant symptom; however, the mechanism of acute kidney injury (AKI) remains unclear, probably because severe thrombocytopenia prevents kidney biopsy. We report a rare case of TAFRO syndrome with histologically confirmed renal involvement. PATIENTS CONCERNS: A 70-year-old man developed fever, anasarca, AKI, thrombocytopenia, and hepatosplenomegaly...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28977667/successful-use-of-entecavir-in-hepatitis-b-associated-membranous-nephropathy
#8
Vidushi Mahajan, Sanjay D'Cruz, Ritambara Nada, Atul Sachdev
We report the case of a 7-year-old unimmunized boy who presented with generalized anasarca for the first time, along with nephrotic-range proteinuria, hypoalbuminemia, microscopic hematuria and hypertension. Special investigations revealed ELISA test to be positive for hepatitis B surface antigen (HBsAg) and hepatitis B envelope antigen (HBeAg); hepatitis B viral DNA load (HBV DNA) level (real-time polymerase chain reaction) was 54 360 903 IU/ml. For hepatitis B virus (HBV)-related glomerulopathy, he was started on enalapril and lasilactone, and percutaneous renal biopsy was performed, which revealed membranous nephropathy (MN)...
July 27, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28969188/primary-intestinal-lymphangiectasia-waldmann-s-disease-presenting-with-chylous-effusions-in-a-15-year-old
#9
Vijay Surampalli, Srinath Ramaswamy, Deepanjali Surendran, Chanaveerappa Bammigatti, Rathinam Palamalai Swaminathan
Primary Intestinal Lymphangiectasia (PIL) is a rare disease of unknown aetiology which presents in the paediatric age group with anasarca, diarrhoea, hypoproteinaemia, lymphoedema and chylous effusions. Tuberculosis, filariasis, chest trauma, malignancies and haematological disorders usually contribute to most cases of secondary lymphangiectasia and chylous effusions. We hereby describe a case of PIL presenting with chylous effusions which remained undiagnosed for eight years.
August 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28956126/a-comparison-of-tafro-syndrome-between-japanese-and-non-japanese-cases-a-case-report-and-literature-review
#10
REVIEW
Fabrice Coutier, Nadine Meaux Ruault, Thomas Crepin, Kevin Bouiller, Helder Gil, Sébastien Humbert, Isabelle Bedgedjian, Nadine Magy-Bertrand
TAFRO syndrome was first described as a variant of multicentric Castleman's disease with thrombocytopenia, anasarca, fever, renal dysfunction, and organomegaly. We report the case of a 25-year-old Caucasian male with diagnosis of TAFRO syndrome and present a literature review. The objective of the study was to compare TAFRO syndrome between Japanese and non-Japanese patients. Cases were included by searching the term "TAFRO" in the Medline database using PubMed between 2010 and 2016. The Student t test and Mann-Whitney U test were used to compare continuous variables...
September 27, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28912039/acquired-heart-disease-superimposed-on-congenital-heart-disease
#11
D Luke Glancy
A 50-year-old man with a murmur since birth developed systemic arterial hypertension as an adult. He came to the hospital because of dyspnea. He had a pulmonic valve ejection click and a murmur of pulmonic stenosis. His echocardiogram showed biventricular hypertrophy, a flat ventricular septum, a D-shaped left ventricle, systolic doming of the pulmonic valve, and Doppler evidence of a 70 mm Hg peak systolic pressure gradient across the pulmonic valve and a peak right ventricular systolic pressure of 100 mm Hg...
November 15, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28860961/case-report-a-toddler-with-anasarca-caused-by-congenital-nephrotic-syndrome
#12
Tumelo M Satekge, Olivia Kiabilua, Gertruida van Biljon, Komala Pillay, Tahir S Pillay
Congenital nephrotic syndrome is a rare inherited disorder arising from defects in the proteins of the cells in the glomerular basement membrane and develops either in utero or at birth. The clinical presentation is the result of massive protein loss in the urine with associated compensatory mechanisms. Here we present a clinical case of a female toddler with a history of anasarca (severe generalised edema) from birth and who presents with the classical biochemical laboratory findings of nephrotic syndrome, together with the more pronounced features that arise from protein loss including abnormal thyroid function testing and a marked hypercholesterolaemia...
May 2017: EJIFCC
https://www.readbyqxmd.com/read/28855215/gemcitabine-induced-chronic-systemic-capillary-leak-syndrome
#13
Ravneet Bajwa, Jason Starr, Karen Daily
A 56-year-old woman presented with anasarca, hypoalbuminaemia and hypotension following cycle 3 day 1 of adjuvant gemcitabine for stage II pancreatic cancer. Due to the temporal nature of presentation, suspicion for gemcitabine-induced capillary leak syndrome was included in the differential diagnosis. Vascular endothelial growth factor levels were elevated at 707 pg/mL (reference range: 9-86 pg/mL). Corticosteroids were initiated, resulting in complete resolution of symptoms and hypotension. The patient suffered relapse of symptoms on discontinuation of steroids, further supporting chronic capillary leak syndrome...
August 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28820362/characterization-of-patients-with-systemic-lupus-erythematosus-who-meet-the-diagnostic-criteria-for-tafro-syndrome
#14
E Hasegawa, H Sato, Y Wada, K Takai, A Wakamatsu, Y Nozawa, T Nakatsue, T Kuroda, Y Suzuki, M Nakano, I Narita
Purpose TAFRO syndrome is a novel disorder manifesting as fever, anasarca, thrombocytopenia, renal insufficiency and organomegaly, and its etiology has not been clarified. The aim of this study was to elucidate similarities and differences between systemic lupus erythematosus (SLE) and TAFRO syndrome. Methods We examined 46 consecutive patients diagnosed with SLE and determined whether they meet the proposed diagnostic criteria for TAFRO syndrome (2015 version). Results Of the 46 patients with SLE, four (8...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28797399/a-54-year-old-man-with-anasarca-dyspnea-and-recurrent-bilateral-pleural-effusions
#15
Pradnya D Patil, Yvette M Cua, Carol Farver, Rafael L Perez, Atul C Mehta, Tanmay S Panchabhai
A 54-year-old African-American man presented with 2 years of progressively worsening dyspnea and anasarca. Over the past 6 months he gained 30 lbs with worsening lower extremity, abdominal wall, and scrotal edema. A recent workup for cardiac, renal, and liver disease, including two-dimensional echocardiogram, liver and renal function tests, and abdominal ultrasound, was unremarkable. He reported a 15-pack year history of smoking and quit 3 years ago. Chest radiograph at that time revealed bilateral pleural effusions that were both reportedly milky in appearance when drained by thoracenteses...
August 2017: Chest
https://www.readbyqxmd.com/read/28755680/capillary-leak-syndrome-in-neuromyelitis-optica-treated-with-rituximab
#16
Ignacio Fuentes Fernandez, Rocio Hernandez-Clares, Ester Carreón Guarnizo, Jose E Meca Lallana
61-year-old woman with Neuromyelitis optica (NMO) diagnosis treated with rituximab was referred to our hospital with severe hypovolemic shock and anasarca. The laboratory findings showed marked hemoconcentration and a decrease in total serum protein. She developed a multiple organ failure and died three hours later. We diagnosed the patient as having capillary leak syndrome (CLS). CLS is a very rare condition caused by unexplained episodic capillary hyperpermeability, which can be idiopathic or secondary to some conditions like infection, malignant disease and some drugs like monoclonal antibodies...
August 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28740835/severe-pulmonary-hypertension-due-to-combined-pulmonary-fibrosis-and-emphysema-another-cause-of-death-among-smokers
#17
REVIEW
André Carramenha de Góes Hirano, Eduardo Pelegrineti Targueta, Fernando Peixoto Ferraz de Campos, João Augusto Dos Santos Martines, Dafne Andrade, Silvana Maria Lovisolo, Aloisio Felipe-Silva
In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents...
April 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28723802/tocilizumab-for-uncontrollable-systemic-inflammatory-response-syndrome-complicating-adult-onset-still-disease-case-report-and-review-of-literature
#18
REVIEW
Asami Masui-Ito, Ryuji Okamoto, Kaoru Ikejiri, Mika Fujimoto, Muneyoshi Tanimura, Shiro Nakamori, Tomohiro Murata, Eiji Ishikawa, Norikazu Yamada, Hiroshi Imai, Masaaki Ito
RATIONALE: Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown etiology characterized by evanescent salmon-pink rash, fever spikes, arthralgia, and lymphadenopathy. AOSD usually has a good prognosis, but it can sometimes be fatal, especially when it is complicated by systemic inflammatory response syndrome (SIRS) and multiple organ failure. PATIENT CONCERNS: A previously healthy 26-year-old woman was referred to our hospital for persistent high fever and mild systemic edema...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28717524/anasarca-fever-thrombocytopenia-organomegaly-and-multiorgan-failure-in-a-24-year-old-pregnant-woman
#19
Guillaume Morel, Joy Mootien, Philippe Guiot, Khaldoun Kuteifan
TAFRO syndrome is a distinct idiopathic multicentric Castleman disease characterized by the association of thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. We report the first case occurring in a Caucasian pregnant woman. At 34 weeks of gestation, our patient presented with all clinical and biological symptoms compatible with a TAFRO syndrome. Tough quick cesarean section was performed as symptoms got worse with onset of multiorgan failure requiring mechanical ventilation for acute respiratory distress, continuous renal replacement, and vasopressors...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28706863/non-functional-tricuspid-valve-disease
#20
REVIEW
Dale S Adler
Only 75% of severe tricuspid regurgitation is classified as functional, or related primarily to pulmonary hypertension, right ventricular dysfunction, or a combination of both. Non-functional tricuspid regurgitation occurs when there is damage to the tricuspid leaflets, chordae, papillary muscles, or annulus, independent of right ventricular dysfunction or pulmonary hypertension. The entities that cause non-functional tricuspid regurgitation include rheumatic and myxomatous disease, acquired and genetic connective tissue disorders, endocarditis, sarcoid, pacing, RV biopsy, blunt trauma, radiation, carcinoid, ergot alkaloids, dopamine agonists, fenfluramine, cardiac tumors, atrial fibrillation, and congenital malformations...
May 2017: Annals of Cardiothoracic Surgery
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