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https://www.readbyqxmd.com/read/28446488/eculizumab-in-a-child-with-atypical-haemolytic-uraemic-syndrome-and-haemophagocytic-lymphohistiocytosis-triggered-by-cytomegalovirus-infection
#1
Gloria M Fraga-Rodriguez, Sonia Brió-Sanagustin, Eulalia Turón-Viñas, Bradley P Dixon, Eduardo Carreras-González
We present the case of a 21-month-old girl with two rare and life-threatening conditions, atypical haemolytic uraemic syndrome (aHUS) and haemophagocytic lymphohistiocytosis (HLH), triggered by a cytomegalovirus (CMV) infection. Soon after admission, the girl became anuric and required continuous venovenous haemodiafiltration.Initial treatments included methylprednisolone, fibrinogen and plasma infusion (for HLH), plasmapheresis (for thrombotic microangiopathy), immunoglobulins (for inflammation), ganciclovir (for CMV infection) and the antibiotic cefotaxime...
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28444683/-atypical-guillain-barre-syndrome-clustering-is-it-necessary-to-reconsider-the-diagnostic-criteria-and-microbiological-protocol
#2
A Dominguez-Mayoral, C Gutierrez, J M Lopez-Dominguez, S Eichau, J Abril, G Navarro-Mascarell, M A Quesada-Garcia, M Ramos, M Alvarez-Lopez, C Menendez-De Leon, G Izquierdo
INTRODUCTION: Guillain-Barre syndrome is classically defined as a symmetrical ascending acute polyradiculoneuropathy, although there are atypical variants that make diagnosis difficult. CASE REPORTS: The medical data of six patients in our hospital area are collected during the first quarter of 2013. Lumbar punctures, imaging, neurophysiological studies, ganglioside antibodies and serologies have been proposed in all cases. We focus on the atypical features as late hyporeflexia, increased frequency of asymmetry and distal paresis and initial fever...
May 1, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28444411/-acute-liver-failure
#3
A Koch, C Trautwein, F Tacke
Acute liver failure (ALF) is a rare, but life-threatening disease that is characterized by the acute onset of jaundice, coagulopathy, and hepatic encephalopathy (HE) in patients without pre-existing liver disease. Main causes in Germany are drug toxicity, acetaminophen overdose, and viral hepatitis (A, B, E). For the initial assessment of patients with ALF and the diagnostic algorithm, the early detection of HE, exclusion of liver cirrhosis, immediate diagnosis of the underlying etiology, and evaluation for the necessity of liver transplantation (LT) are critical...
April 25, 2017: Medizinische Klinik, Intensivmedizin und Notfallmedizin
https://www.readbyqxmd.com/read/28443948/relapse-of-congenital-thrombotic-thrombocytopenic-purpura-after-spontaneous-remission-in-a-second-trimester-primigravida-case-report-and-review-of-the-literature
#4
Donavan de Souza Lúcio, Jacqueline Foelkel Pignatari, Marcelo Gil Cliquet, Henri Augusto Korkes
CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities...
April 20, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28443263/a-1-month-old-infant-with-chylomicronemia-due-to-gpihbp1-gene-mutation-treated-by-plasmapheresis
#5
Mo Kyung Jung, Juhyun Jin, Hyun Ok Kim, Ahreum Kwon, Hyun Wook Chae, Seok Jin Kang, Duk Hee Kim, Ho-Seong Kim
Chylomicronemia is a severe type of hypertriglyceridemia characterized by chylomicron accumulation that arises from a genetic defect in intravascular lipolysis. It requires urgent and proper management, because serious cases can be accompanied by pancreatic necrosis or persistent multiple organ failure. We present the case of a 1-month-old infant with chylomicronemia treated by plasmapheresis. His chylomicronemia was discovered incidentally when lactescent plasma was noticed during routine blood sampling during a hospital admission for fever and irritability...
March 2017: Annals of Pediatric Endocrinology & Metabolism
https://www.readbyqxmd.com/read/28439852/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-a-concise-review-with-a-comprehensive-summary-of-therapeutic-interventions-emphasizing-supportive-measures
#6
REVIEW
Jeremy A Schneider, Philip R Cohen
INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are two of the most severe dermatologic conditions occurring in the inpatient setting. There is a lack of consensus regarding appropriate management of SJS and TEN. PURPOSE: The scientific literature pertaining to SJS and TEN (subsequently referred to as SJS/TEN) is summarized and assessed. In addition, an interventional approach for the clinician is provided. METHODS: PubMed was searched with the key words: corticosteroids, cyclosporine, etanercept, intravenous immunoglobulin, Stevens-Johnson syndrome, and toxic epidermal necrolysis...
April 24, 2017: Advances in Therapy
https://www.readbyqxmd.com/read/28438876/thrombotic-microangiopathy-following-chemotherapy-with-s-1-and-cisplatin-in-a-patient-with-gastric-cancer-a-case-report
#7
Jun Muto, Hiroshi Kishimoto, Yasuo Kaizuka, Mitsuru Kinjo, Hidefumi Higashi, Fumiaki Kishihara
BACKGROUND: Thrombotic microangiopathy (TMA) represents a spectrum of serious disorders characterized by occlusive microvascular thrombosis, thrombocytopenia and end-organ damage. TMA is associated with a broad range of conditions and is also a well-described complication of both cancer and its treatment. CASE REPORT: A 77-year-old Japanese woman underwent S-1 and cisplatin chemotherapy for treatment of advanced gastric cancer with multiple lymph node and liver metastases...
May 2017: In Vivo
https://www.readbyqxmd.com/read/28438465/isaacs-syndrome-with-caspr2-antibody-a-series-of-three-cases
#8
Jie Song, Sisi Jing, Chao Quan, Jun Lu, Xiangyang Qiao, Kai Qiao, Jiahong Lu, Jianying Xi, Chongbo Zhao
Isaacs syndrome is a form of peripheral nerve hyperexcitability, characterized by spontaneous muscle twitching and stiffness. Some patients are reported to be positive for CASPR2 antibody that may be one of the pathogenic autoantibodies in Isaacs syndrome. We reported a series of three patients with Isaacs syndrome, including their clinical features, electrophysiologic findings, laboratory parameters and therapeutic responses. All the three patients were positive for CASPR2 antibodies examined on transfected human embryonic kidney 293 cells by indirect immunofluorescence method...
April 21, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28432047/delayed-lgi1-seropositivity-in-voltage-gated-potassium-channel-vgkc-complex-antibody-limbic-encephalitis
#9
Michael Sweeney, Jonathan Galli, Scott McNally, Anne Tebo, Thomas Haven, Perla Thulin, Stacey L Clardy
We utilise a clinical case to highlight why exclusion of voltage-gated potassium channel (VGKC)-complex autoantibody testing in serological evaluation of patients may delay or miss the diagnosis. A 68-year-old man presented with increasing involuntary movements consistent with faciobrachial dystonic seizures (FBDS). Initial evaluation demonstrated VGKC antibody seropositivity with leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (CASPR2) seronegativity. Aggressive immunotherapy with methylprednisolone and plasmapheresis was started early in the course of his presentation...
April 20, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28428030/complement-inhibition-with-eculizumab-for-thrombotic-microangiopathy-rescues-a-living-donor-kidney-transplant-in-a-patient-with-antiphospholipid-antibody-syndrome
#10
Praveen Ramakrishnan Geethakumari, Patrick Mille, Rakesh Gulati, Srikanth Nagalla
Antiphospholipid antibody syndrome (APS) is an enigmatic heterogeneous disorder despite several revelations in its pathobiology. Renal transplantation in patients with APS has been notoriously difficult due to the high risk of development of thrombotic microangiopathy (TMA), which is often refractory to conventional treatment modalities such as aggressive anticoagulation and plasmapheresis. We describe a case of a 58-year-old male with secondary APS undergoing living unrelated renal transplantation for end-stage renal disease from lupus nephritis...
March 10, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28427710/clinical-outcomes-and-predictive-factors-related-to-good-outcomes-in-plasma-exchange-in-severe-attack-of-nmosd-and-long-extensive-transverse-myelitis-case-series-and-review-of-the-literature
#11
Saharat Aungsumart, Metha Apiwattanakul
OBJECTIVE: To investigate the predictive factors associated with good outcomes of plasma exchange in severe attacks through neuromyelitis optica spectrum disorder (NMOSD) and long extensive transverse myelitis (LETM). In addition, to review the literature of predictive factors associated with the good outcomes of plasma exchange in central nervous system inflammatory demyelinating diseases (CNS IDDs). METHODS: Retrospective study in 27 episodes of severe acute attacks myelitis and optic neuritis in 24 patients, including 20 patients with NMOSD seropositive, 1 patient with NMOSD seronegative and 3 patients with LETM...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28424014/semi-quantitative-analysis-of-cerebral-fdg-pet-reveals-striatal-hypermetabolism-and-normal-cortical-metabolism-in-a-case-of-vgkcc-limbic-encephalitis
#12
Patrick Moloney, Ruth Boylan, Marwa Elamin, Sean O'Riordan, Ronan Killeen, Christopher McGuigan
In the context of delayed autoimmune encephalitis antibody results, functional imaging can support the diagnosis of limbic encephalitis associated with anti-voltage-gated potassium channel complex (VGKCC) antibodies. Here we present a typical case of VGKCC encephalitis in a 69-year-old woman whose symptoms responded to plasmapheresis. A cerebral 18F-fluoro-2-deoxy-d-glucose positron emission tomography (FDG-PET) scan performed prior to commencing treatment revealed striatal hypermetabolism assessed qualitatively and semi-quantitatively, with normal uptake in the cortex and cerebellum when analysed semi-quantitatively...
April 2017: Neuroradiology Journal
https://www.readbyqxmd.com/read/28418206/anti-nmda-receptor-encephalitis-during-pregnancy-a-case-report-and-literature-review
#13
Xue Xiao, Shunping Gui, Peng Bai, Yi Bai, Dan Shan, Yayi Hu, Tri M Bui-Nguyen, Rong Zhou
Anti-N-methyl-d-aspartate receptor (anti-NMDA-R) encephalitis is an autoimmune disorder that was first described by Dr Vitaliani in 2005. In 2007, Dalmau et al. found anti-NMDA-R antibody expressed both in the hippocampus and prefrontal nerve cell membrane, finally proposing the diagnosis of autoimmune anti-NMDA-R encephalitis. Most of the patients are female (91%), with ages ranging from 4 to 76 years. The average age is 23 years, a birth peak age, although anti-NMDA-R encephalitis is rare during pregnancy...
April 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/28414683/recurrent-hypertriglyceridemic-pancreatitis-htgp-and-the-use-of-insulin-drip-as-treatment
#14
T Tran, N Lee
INTRODUCTION: Chronic pancreatitis by definition is a recurrent episode of acute pancreatitis and is commonly associated with alcoholism in the US. Another cause of chronic pancreatitis is hypertriglyceridemia (HTGP);, occurring in 1-5 percent of cases. The incidence of HTGP is higher in patients with diabetes and HIV, usually requiring triglyceride levels > 1000 mg/dL. CASE: A 33 year old man with uncontrolled type 1 diabetes and recurrent pancreatitis, first diagnosed 5 years prior, presented as a transfer from an outside hospital for a recurrent episode of HTGP...
March 2017: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#15
REVIEW
Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito
Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates...
April 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28405538/henoch-sch%C3%A3-nlein-purpura-presenting-as-severe-gastrointestinal-and-renal-involvement-with-mixed-outcomes-in-an-adult-patient
#16
Raj Shah, Madhuri Ramakrishnan, Alexis Vollmar, Amanda Harrell, Richard Van Trump, Amgad Masoud
Henoch-Schönlein purpura (HSP) is typically seen as a self-limiting disease in children, but can present more severely in adults, especially when there is renal involvement. Management of HSP in adults also remains a controversial topic with very few studies evaluating available therapies. In this case, HSP presenting as a combination of severe gastrointestinal involvement and a rapid decline in renal function in an adult patient directed our therapy. The patient was a 48-year-old Caucasian male with no known past medical history, who presented with a combination of purpuric rash over the lower extremities, severe abdominal pain with upper gastrointestinal bleeding and a rapidly increasing serum creatinine, with hematuria...
March 9, 2017: Curēus
https://www.readbyqxmd.com/read/28405484/thyroid-associated-ophthalmopathy
#17
REVIEW
Esra Şahlı, Kaan Gündüz
Thyroid-associated ophthalmopathy is the most frequent extrathyroidal involvement of Graves' disease but it sometimes occurs in euthyroid or hypothyroid patients. Thyroid-associated ophthalmopathy is an autoimmune disorder, but its pathogenesis is not completely understood. Autoimmunity against putative antigens shared by the thyroid and the orbit plays a role in the pathogenesis of disease. There is an increased volume of extraocular muscles, orbital connective and adipose tissues. Clinical findings of thyroid-associated ophthalmopathy are soft tissue involvement, eyelid retraction, proptosis, compressive optic neuropathy, and restrictive myopathy...
April 2017: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/28393729/granulomatous-polyangitis-wegener-granulomatosis-clinical-findings-and-results-of-long-term-follow-up
#18
Nazan Şen, Müge Aydın Tufan, Reyhan Yıldız, Emine Duygu Ersözlü Bozkırlı, Eftal Yücel
Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. Patients and Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. Result: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72...
September 2016: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/28390833/successful-treatment-of-propafenone-induced-cardiac-arrest-by-calcium-gluconate-a-case-report
#19
Xiaoyu Chen, Zaixing Yang
Propafenone is prescribed for the control of cardiac ventricular arrhythmias. Poisoning from propafenone intoxication is rare, but the survival rate of patients is low. We present a case of a 37-year-old man who developed cardiac arrest due to propafenone intoxication. Cardiopulmonary resuscitation, plasmapheresis, and other medical treatments had no effect on cardiac arrest. After repeated administrations of calcium gluconate, the patient achieved a full recovery. To the best of our knowledge, this is the first case report in which a full recovery from cardiac arrest was achieved by administration of calcium gluconate...
April 3, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28380082/heparin-induced-thrombocytopenia-as-a-cause-of-prolonged-low-platelet-count-in-a-patient-with-thrombotic-thrombocytopenic-purpura-treated-with-plasmapheresis
#20
Agata Winiarska, Norbert Kwella, Tomasz Stompór
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder belonging to thrombotic microangiopathies (TMA) and is caused by functional deficiency of the ADAMTS-13 metalloproteinase. Plasma exchange (PE) remains the treatment of choice in this disease. Here, were describe the case of a patient who apparently recovered from TTP following multiple sessions of PE, but remained thrombocytopenic. Careful analysis revealed the development of heparin-induced thrombocytopenia (HIT) that precluded platelet count (PLT) normalization...
April 5, 2017: Acta Biochimica Polonica
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