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Purpura fulminans

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https://www.readbyqxmd.com/read/29683961/acquired-versus-congenital-neonatal-purpura-fulminans-a-case-report-and-literature-review
#1
Tina Findley, Minal Patel, John Chapman, Deborah Brown, Andrea F Duncan
Neonatal purpura fulminans (PF) is a life-threatening disorder caused by congenital or acquired deficiencies of protein C (PC) or S. PF presents as a cutaneous manifestation of disseminated intravascular coagulation. We describe a case of PF in a newborn with left leg ischemia and undetectable PC levels soon after birth. Despite anticoagulation therapy and PC concentrate, left foot amputation was required. Genetic testing of PROC for congenital PC deficiency was normal. This case highlights the course of PF due to acquired PC deficiency in a newborn treated with PC concentrate which is rarely described in the literature...
April 20, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29676380/fatal-meningococcal-septicemia-without-meningeal-signs-contribution-of-the-peripheral-smear-in-diagnosis-report-of-a-case
#2
Deepti Mutreja, Nikhil Moorchung, S J Manasa, Jeenu Varghese
Acute meningococcemia is characterized by extensive purpurae consisting of both petechiae and ecchymoses. This condition can be rapidly fatal without treatment due to shock and severe consumptive coagulopathy. We report a case of fatal meningococcal septicemia in a military recruit who presented with fever and associated rapidly progressive purpuric rash (purpura fulminans) without any meningeal signs. Evaluation revealed evidence of disseminated intravascular coagulopathy and multiorgan failure. Diplococci were demonstrated in peripheral blood neutrophils and monocytes...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29664383/two-cases-of-israeli-spotted-fever-with-purpura-fulminans-sharon-district-israel
#3
Regev Cohen, Frida Babushkin, Maurice Shapiro, Martina Uda, Yafit Atiya-Nasagi, Dar Klein, Talya Finn
We report a series of 5 case-patients who had Israeli spotted fever, of whom 2 had purpura fulminans and died. Four case-patients were given a diagnosis on the basis of PCR of skin biopsy specimens 3-4 days after treatment with doxycycline; 1 case-patient was given a diagnosis on the basis of seroconversion. Rickettsia spp. from the 2 case-patients who died were sequenced and identified as Rickettsia conorii subsp. israelensis. Purpura fulminans has been described in association with R. rickettsii and R. indica, but rarely with R...
May 2018: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/29657898/slow-elevation-in-protein-c-activity-without-a-proc-mutation-in-a-neonate-with-intracranial-hemorrhage
#4
Erika Uehara, Hiro Nakao, Yusuke Tsumura, Hisaya Nakadate, Shoichiro Amari, Hideshi Fujinaga, Yoshiyuki Tsutsumi, Dongchon Kang, Shouichi Ohga, Akira Ishiguro
Severe protein C (PC) deficiency leads to purpura fulminans and stroke in newborns. However, the clinical impact of plasma PC activity on the development of neonatal cerebral disease remains elusive. We report a case of hemorrhagic stroke associated with neonatal asphyxia and severe PC deficiency. Plasma PC and protein S activity 7 days after birth was 12% and 43%, respectively. No PROC mutation was found. PC levels did not exceed 20% until 2 months of age, even in the absence of consumption coagulopathy or vitamin K deficiency...
April 2018: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29593006/purpura-fulminans-causing-acute-cortical-necrosis-in-postpartum-period
#5
Mohan Kumar, Kundan Mishra, Vikas Suri, Savita Kumari
No abstract text is available yet for this article.
March 28, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29540887/neonatal-purpura-fulminans-a-rare-genetic-disorder-due-to-protein-c-deficiency-a-case-report
#6
Sayyeda Ghazala Irfan Kazi, Emaduddin Siddiqui, Irfan Habib, Saadia Tabassum, Badar Afzal, Irum Qamar Khan
Neonatal Purpura Fulminans is a rare and fatal disorder associated with perivascular haemorrhage and disseminated intravascular coagulation. Early clinical recognition, timely investigation and treatment is utmost important. A 6 days old baby boy was brought to emergency with blackish ulcers all over the body. Initially these were over the feet and scalp but later appeared on the abdomen. On examination, child was vitally stable, mildly icteric and had multiple erythematous large bullous blackish lesions on scalp, lower abdomen, perineum, back and soles...
March 2018: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/29540648/acute-kidney-injury-in-a-case-of-purpura-fulminans-developing-secondary-to-antithrombin-3-deficiency
#7
Gülsüm Özkan, Gaye Kübra Emeksiz, Reşit Volkan Atar, Samet Sedef, Pınar Sonat Kara, Meltem Öznur, Burhan Turgut
Purpura fulminans associated with antithrombin 3 (AT 3) deficiency is very rare in adults and neonates. It can be categorized into three principal forms - neonatal, idiopathic and acute infectious. Purpura fulminans has been reported to cause cardiac, pulmonary and renal damage in rare cases. We describe an adult case of purpura fulminans developing in association with AT 3 deficiency without infection following a surgical procedure, and acute kidney injury (AKI) developing secondary to rhabdomyolysis and disseminated intravascular coagulation (DIC)...
2018: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/29437318/acute-infectious-purpura-fulminans-secondary-to-a-dog-bite
#8
Antonio Andreu Ruiz, Tomás Ros Argente Del Castillo, José Moya Sánchez
No abstract text is available yet for this article.
February 2018: Emergencias: Revista de la Sociedad Española de Medicina de Emergencias
https://www.readbyqxmd.com/read/29381878/purpura-fulminans-it-s-not-always-sepsis
#9
Martin Olivieri, Sebastian Huetker, Karin Kurnik, Christoph Bidlingmaier, Julia Keil, Karl Reiter, Florian Hoffmann
No abstract text is available yet for this article.
January 30, 2018: Klinische Pädiatrie
https://www.readbyqxmd.com/read/29356699/a-novel-compound-heterozygous-mutations-in-protein-c-gene-causing-neonatal-purpura-fulminans
#10
Huifei Zhang, Xiaojie Bi, Zhengxian Su, Xi Tu, Lizhen Wang, Bo Shen
: Neonatal purpura fulminans is a rare, life-threatening disease caused by severe congenital deficiency of protein C (PC) because of homozygous or compound heterozygous mutations in the PROC gene. Mutation analysis plays a critical role in diagnosing the disorder and offering prenatal guidance. In this study, we identified a genetic defect in the PROC gene leading to neonatal purpura fulminans. The propositus had very low PC activity (4%) and PC antigen activity (5%). DNA screening of the whole PROC gene revealed two compound heterozygous mutations in exon8 (c...
March 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29174007/-acute-hemorrhagic-edema-of-infancy-associated-with-coxsackie-virus-infection
#11
A Debray, V Ollier, A Coutard, F Arditty, S Bekkar, C Bodemer, M Leruez-Ville, A Mirand, F Lesage, P Foucaud
Acute hemorrhagic edema of infancy is a rare but benign vasculitis occurring in infants aged from 4 to 24 months. Skin lesions can take various forms, including extensive hemorrhagic purpura, and can therefore be mistaken for purpura fulminans if associated with fever, which leads to initiating broad-spectrum antibiotic treatment. In the present case, we describe a 7-month-old boy with acute hemorrhagic edema of infancy and rapidly extensive purpura lesions that led to intravenous cefotaxime and amikacin treatment...
December 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29157918/purpura-fulminans-mechanism-and-management-of-dysregulated-hemostasis
#12
REVIEW
Meaghan E Colling, Pavan K Bendapudi
Purpura fulminans (PF) is a highly thrombotic subtype of disseminated intravascular coagulation that can accompany severe bacterial, and more rarely, viral infections. PF is associated with an extremely high mortality rate, and patients often die of overwhelming multisystemic thrombosis rather than septic shock. Survivors typically experience amputation of involved extremities and significant scarring in affected areas. Despite the devastating clinical course associated with this hemostatic complication of infection, the mechanism of PF remains poorly understood...
April 2018: Transfusion Medicine Reviews
https://www.readbyqxmd.com/read/29108905/-maternal-deaths-due-to-infectious-cause-results-from-the-french-confidential-enquiry-into-maternal-deaths-2010-2012
#13
A Rigouzzo, V Tessier, L Zieleskiewicz
Over the period 2010-2012, maternal mortality from infectious causes accounted for 5% of maternal deaths by direct causes and 16% of maternal deaths by indirect causes. Among the 22 deaths caused by infection occurred during this period, 6 deaths were attributed to direct causes from genital tract origin, confirming thus the decrease in direct maternal deaths by infection during the last ten years. On the contrary, indirect maternal deaths by infection, from extragenital origin, doubled during the same period, with 16 deaths in the last triennium, dominated by winter respiratory infections, particularly influenza: the 2009-2010 influenza A (H1N1) virus pandemic was the leading cause of indirect maternal mortality by infection during the studied period...
December 2017: Gynecologie, Obstetrique, Fertilite & Senologie
https://www.readbyqxmd.com/read/29099305/peripheral-blood-vessels-are-a-niche-for-blood-borne-meningococci
#14
Elena Capel, Jean-Philippe Barnier, Aldert L Zomer, Christine Bole-Feysot, Thomas Nussbaumer, Anne Jamet, Hervé Lécuyer, Daniel Euphrasie, Zoé Virion, Eric Frapy, Philippe Pélissier, Olivier Join-Lambert, Thomas Rattei, Sandrine Bourdoulous, Xavier Nassif, Mathieu Coureuil
Neisseria meningitidis is the causative agent of cerebrospinal meningitis and that of a rapidly progressing fatal septic shock known as purpura fulminans. Meningococcemia is characterized by bacterial adhesion to human endothelial cells of the microvessels. Host specificity has hampered studies on the role of blood vessels colonization in N. meningitidis associated pathogenesis. In this work, using a humanized model of SCID mice allowing the study of bacterial adhesion to human cells in an in vivo context we demonstrate that meningococcal colonization of human blood vessels is a prerequisite to the establishment of sepsis and lethality...
November 17, 2017: Virulence
https://www.readbyqxmd.com/read/29082047/protein-c-deficiency-caused-by-a-novel-mutation-in-the-proc-gene-in-an-infant-with-delayed-onset-purpura-fulminans
#15
Mariam S Al Harbi, Ayman W El-Hattab
Protein C is an anticoagulant that is encoded by the PROC gene. Protein C deficiency (PCD) is inherited in an autosomal dominant or recessive pattern. Autosomal dominant PCD is caused by monoallelic mutations in PROC and often presents with venous thromboembolism. On the other hand, biallelic PROC mutations lead to autosomal recessive PCD which is a more severe disease that typically presents in neonates as purpura fulminans. In this report, we describe an 8-month-old infant with autosomal recessive PCD who presented with multiple lumps on his lower extremities at the age of 2 months and later developed purpura fulminans after obtaining a muscle biopsy from the thigh at the age of 5 months...
2017: Case Reports in Dermatological Medicine
https://www.readbyqxmd.com/read/29072871/disseminated-intravascular-coagulation-in-an-under-recognised-zoonotic-infection
#16
Sarah Lawrence, Andrew Claxton, Mark Holland, Jack Hodd
A 51 year old man presented with severe sepsis, disseminated intravascular coagulation (DIC) and multiorgan dysfunction after a 24 hour history of diarrhoea and malaise. Despite fluid resuscitation and receiving a platelet transfusion, freshfrozen plasma and intravenous broad-spectrum antibiotics, he remained anuric with a worsening metabolic acidosis. He was transferred to critical care for organ support including renal replacement therapy. He subsequently developed purpura fulminans. Blood cultures were positive for Captocytophaga carnimorsis, a gram-negative canine zoonosis that is an underdiagnosed cause of severe sepsis, for which DIC at presentation is characteristic...
2017: Acute Medicine
https://www.readbyqxmd.com/read/29062669/reconstruction-of-near-total-loss-of-the-upper-and-lower-lips-due-to-purpura-fulminans-with-local-tissue-and-a-dual-skin-paddled-anterolateral-thigh-flap
#17
Masamitsu Kuwahara, Satoshi Yurugi, Yuji Yamanaka, Chikako Sasaki, Takashi Nakanishi
It is difficult to totally reconstruct the lip, achieving good functional and aesthetic results. There have been few reports of reconstructing complete lip defects. Moreover, upper and lower lip necrosis by purpura fulminans has not been reported. We present a case of a 60-year-old male purpura fulminans patient with upper and lower lip necrosis. Fortunately, our patient had retained his oral commissure function. We reconstructed this defect with an orbicularis oris muscle-skin-mucosal pedicled flap derived from the region between the nasolabial folds for upper lip; a similar bipedicled flap for the lower lip and the donor site was closed with a dual-skin paddled anterolateral thigh flap...
September 2017: Plastic and Reconstructive Surgery. Global Open
https://www.readbyqxmd.com/read/29054893/purpura-fulminans-a-rare-presentation-of-streptococcus-pneumoniae-infection
#18
Hock Gin Teo, Jun Yet Wong, Tracy Lee Lyee Ting
A previously healthy man presented with fever for 2 days and rapidly progressive purpuric rash for 1 day. He progressed into hypotension, disseminated intravascular coagulation and refractory shock despite resuscitation and early antibiotic commencement. Blood culture grew Streptococcus pneumoniae This case report highlights the fact that purpura fulminans can be a rare presentation of S. pneumoniae infection as well.
October 20, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29024990/myocarditis-and-purpura-fulminans-in-meningococcaemia
#19
A A Gawalkar, S Tale, B A Chhabria, A Bhalla
No abstract text is available yet for this article.
November 1, 2017: QJM: Monthly Journal of the Association of Physicians
https://www.readbyqxmd.com/read/28915308/mutilating-purpura-fulminans-in-an-adult-with-meningococcal-sepsis
#20
María Elena Arnáiz-García, Ana María Arnáiz-García, Francisco Gutierrez-Diez, Juan Francisco Nistal, Jose María González-Santos, Ivana Pulitani, Carlos Amado-Diago, Javier Arnáiz
We report a dramatic case of meningococcal sepsis manifesting as purpura fulminans in an elderly diabetic woman. Hemodynamic instability and severe bilateral cutaneous lesions involving her hands and feet developed rapidly. Specific antibiotic therapy and the administration of inotropic and vasopressor drugs were initiated. The severity and extension of the cutaneous lesions (attributed to purpura fulminans) worsened because of the need for vasoconstrictors for the treatment of septic shock. Bilateral transmetatarsal and metacarpal amputations were required to stabilize the patient...
September 2017: Puerto Rico Health Sciences Journal
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