scholarly journals Difficulties in the treatment of recurrent juvenile laryngeal papillomatosis associated with pemphigus vulgaris

2020 ◽  
Vol 10 (38) ◽  
pp. 38-44
Author(s):  
Codrut Sarafoleanu ◽  
Gheorghe Lapicus ◽  
Ana Isabela Lupu ◽  
Vlad Budu

AbstractRecurrent laryngeal papillomatosis is a disease caused by human papillomaviruses (HPVs), which currently does not benefit from a curative treatment. Due to the fact that HPV has the action of modifying cellular DNA, with changes in the expression of interleukins and interferon, with insufficient maturation of T cells and intracellular overpopulation of immunosuppressive cells, the association of RRP (Recurrent Respiratory Papillomatosis) with an autoimmune disease may cause particular difficulties in the therapeutic management of patients diagnosed with RRP.Immunosuppressive medication negatively influences the development of papillomatosis, increasing the number of local relapses and, respectively, the need for surgical intervention due to the increased viral multiplication and the proliferation of papillomatous lesions. In order to exemplify the difficulties encountered in treating RRP associated with an autoimmune disorder, the authors present the case of a 21-year-old patient diagnosed with juvenile recurrent laryngeal papillomatosis genotype 6, with multiple antecedent surgeries, who was diagnosed with pemphigus vulgaris at the age of 19. The peculiarity of the case lies in the difficulty of managing the RRP associated with an immunosuppressive disorder whose therapeutic indication is cortisone and immunosuppressive treatment, which led to exacerbation of viral multiplication and proliferation of papillomatous lesions.

2019 ◽  
Vol 64 (6) ◽  
pp. 499
Author(s):  
Brijesh Takkar ◽  
Mihika Dube ◽  
DineshP Asati ◽  
Sourabh Jain

2021 ◽  
Author(s):  
Marie-Kristin Schwaegermann ◽  
Lukas Hobohm ◽  
Johanna Rausch ◽  
Michael Reuter ◽  
Thomas-Friedrich Griemert ◽  
...  

AbstractImmune thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder characterized by severely reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to autoantibodies. This leads to the development of pathogenic multimers of VWF, causing a thrombotic microangiopathy with decreased number of platelets, hemolysis, and life-threatening tissue ischemia of mostly brain, heart, and kidneys. Standard treatment of iTTP involves daily plasma exchange to remove ultra large multimers of VWF, inhibitors, substituting ADAMTS13, and the accompaniment of an immunosuppressive treatment with steroids. Recently, caplacizumab was approved for iTTP. Caplacizumab is a nanobody binding the A1 domain of VWF, blocking its interaction with glycoprotein Ib–IX–V platelet receptor and therefore preventing platelet aggregation. VWF activities may serve as therapeutic drug monitoring of caplacizumab, whereas ADAMTS13 activities may be used for biomarkers to guide caplacizumab treatment modalities and overall treatment duration. Additional immunosuppressive treatment by inhibiting autoantibody formation (e.g., the use of Rituximab, a chimeric monoclonal antibody directed against the B-cell antigen CD20) is a further treatment option. Infections are well-known causes for an acute episode for patients with iTTP. The novel SARS-CoV-2 virus is mainly associated with acute respiratory distress as well as diffuse endothelial inflammation and increased coagulopathy. However, little is known about an infection with SARS-CoV-2 virus triggering iTTP relapses. We herein report the case of an acute iTTP episode accompanying a SARS-CoV-2 infection.


1989 ◽  
Vol 11 (5) ◽  
pp. 132-159

Laryngeal papillomatosis is a difficult disease to manage, and patients with this disease are at risk of cancer and death. These infections are caused by human papillomaviruses of which there are more than 30. Human papillomavirus type 11 causes both genital warts (condyloma accuminata) and laryngeal papillomas. Available data indicate that neonates acquire this virus as they pass through a birth canal infected with human papillomavirus type 11. Laryngeal papillomas occur in children younger than 10 years of age, and the incidence peaks between the ages of 2 and 5 years. Signs of the disease are voice change, abnormal cry, and airway obstruction; it spontaneously regresses or becomes malignant.


2019 ◽  
Vol 12 (9) ◽  
pp. e230948
Author(s):  
Uthara Mathew ◽  
Ankit Mittal ◽  
Surabhi Vyas ◽  
Animesh Ray

Interstitial pneumonia with autoimmune features (IPAF) is a recently proposed terminology for interstitial lung disease (ILD) with evidence of autoimmunity that does not meet the criteria for a defined connective tissue disease (CTD). Although ILD is well recognised in patients with established CTD, it is rarely the sole presenting feature of CTD. We report a case of 22-year-old male patient, who presented with progressive shortness of breath for 2 months and had features suggestive of platypnea-orthodeoxia syndrome (POS). Imaging revealed ILD with usual interstitial pneumonia pattern. Patient had features of autoimmune disorder but did not fulfil the criteria for any CTD and hence was labelled as IPAF. His POS was attributed predominantly to the lower lobe disease. The patient responded well to immunosuppressive treatment. A systematic review of literature of all cases with POS due to pulmonary parenchymal involvement has also been done.


2008 ◽  
Vol 82 (18) ◽  
pp. 9056-9064 ◽  
Author(s):  
Sally Roberts ◽  
Sarah R. Kingsbury ◽  
Kai Stoeber ◽  
Gillian L. Knight ◽  
Phillip H. Gallimore ◽  
...  

ABSTRACT Productive infections by human papillomaviruses (HPVs) are restricted to nondividing, differentiated keratinocytes. HPV early proteins E6 and E7 deregulate cell cycle progression and activate the host cell DNA replication machinery in these cells, changes essential for virus synthesis. Productive virus replication is accompanied by abundant expression of the HPV E4 protein. Expression of HPV1 E4 in cells is known to activate cell cycle checkpoints, inhibiting G2-to-M transition of the cell cycle and also suppressing entry of cells into S phase. We report here that the HPV1 E4 protein, in the presence of a soluble form of the replication-licensing factor (RLF) Cdc6, inhibits initiation of cellular DNA replication in a mammalian cell-free DNA replication system. Chromatin-binding studies show that E4 blocks replication initiation in vitro by preventing loading of the RLFs Mcm2 and Mcm7 onto chromatin. HPV1 E4-mediated replication inhibition in vitro and suppression of entry of HPV1 E4-expressing cells into S phase are both abrogated upon alanine replacement of arginine 45 in the full-length E4 protein (E1^E4), implying that these two HPV1 E4 functions are linked. We hypothesize that HPV1 E4 inhibits competing host cell DNA synthesis in replication-activated suprabasal keratinocytes by suppressing licensing of cellular replication origins, thus modifying the phenotype of the infected cell in favor of viral genome amplification.


2020 ◽  
Vol 5 (2) ◽  
pp. 77-80
Author(s):  
Dumitru Costel ◽  
Dana Ghiga ◽  
Septimiu Voidazan ◽  
Alexandra Grosan ◽  
Dan Simpalean ◽  
...  

AbstractBackground: Although the clinical evolution of a patient with heart failure is initially improved by transplantation, a number of potential complications may occur in the post-transplant period, which may be directly related to the effects of chronic immunosuppression. The purpose of this study was to analyze the occurrence and frequency of post-transplant complications related to immunosuppressive treatment in the Institute of Cardiovascular Diseases and Transplantation of Târgu Mureș, Romania.Material and methods: This is a descriptive study including 53 patients out of a total of 71 patients who underwent cardiac transplantation between 2000 and 2017 in the Institute of Cardiovascular Disease and Cardiac Transplantation in Târgu Mureș, Romania. Data were collected from the patient files and included demographic, clinical and laboratory data, as well as information about post-transplant complications related to immunosuppressive treatment.Results: The mean age of patients undergoing heart transplantation was 40.72 ± 14.07 years, the majority of patients being male (84.91%) and living in an urban environment (56.60%). The average length of hospital stay was 33.6 days. From the total number of patients, 7 (13.2%) presented post-transplantation bacterial infections, while antibodies indicating the presence or history of B hepatitis, toxoplasma, and cytomegalovirus infection were identified with a relatively high incidence in the study population.Conclusions: Infections following surgery are probably the most common post-transplant pathology, the primary reason being the administration of immunosuppressive medication.


2019 ◽  
Vol 13 (1) ◽  
pp. 108-115 ◽  
Author(s):  
Lennerling Annette ◽  
Kisch Annika ◽  
Forsberg Anna

Background: After lung transplantation, life-long treatment with immunosuppressive medication is required to prevent rejection and graft loss but adherence to immunosuppressive treatment may be difficult for the lung recipient. Adherence is essential and non-adherence to immunosuppressive treatment can lead to graft loss and death. Objective: The aim of this cross-sectional study was to investigate the prevalence of non-adherence 1 to 5 years after lung transplantation in relation to symptom burden, health literacy, psychological well-being and relevant demographic variables. Methods: 117 adult lung recipients, due for their annual follow-up 1-5 years after lung transplantation, participated. Four self-report instruments were used for assessment: the Basel Assessment of Adherence with Immunosuppressive Medication Scale, the Newest Vital Sign, the Psychological General Well-Being and the Organ Transplant Symptom and Wellbeing Instrument. Statistical analysis was performed. Results: Thirty percent of the lung recipients were non-adherent. The most common non-adherence dimension was not taking a dose (43%) and not being punctual with the regimen (80%). Of those working full time or part time, 43% were non-adherent (p=.032). A higher level of non-adherence was reported a long time after LuTx with the highest level at the 3-year follow-up. Conclusion: The level of non-adherence among lung recipients was high. The highest levels were found among those who had returned to work. Non-adherence increased with time after lung transplantation.


2018 ◽  
Vol 69 (8) ◽  
pp. 2251-2253
Author(s):  
Paul Salahoru ◽  
Cristina Mihaela Ghiciuc ◽  
Cristina Grigorescu ◽  
Marius Valeriu Hinganu ◽  
Catalina Elena Lupusoru

Myasthenia gravis is an autoimmune disorder which presents a series of clinical manifestations which are generally motor. The hallmark of the disorder is muscle weakness, which typically worsens during physical exercise and improves upon rest. Approximatively 80% of the patients presenting this affection test positive for Acetylcholine antibody anti-receptor (AChR-ab).Treatment of patients with myasthenia gravis consists of:administration of oral medica-tion, immunomodulating treatment by removal of AChR-ab through plasmapheresis or surgical intervention (thymectomy).This article presents a retrospective study in which the authors attempted to identify factors which can influence the effect of thymectomy on AChR-ab titration and on the clinical presentation of the patients suffering with myasthenia gravis.


mBio ◽  
2019 ◽  
Vol 10 (2) ◽  
Author(s):  
Marit Orav ◽  
David Gagnon ◽  
Jacques Archambault

ABSTRACTHuman papillomaviruses (HPVs) are important pathogens with a significant medical burden. HPV genomes replicate in infected cells via bidirectional theta replication and a poorly understood unidirectional mechanism. In this report, we provide evidence that the previously described interaction between the viral E1 helicase and the cellular UAF1-USP1 deubiquitinating enzyme complex, a member of the Fanconi anemia DNA damage response pathway, is required for the completion of the bidirectional theta replication of the HPV11 genome and the subsequent initiation of the unidirectional replication. We show that unidirectional replication proceeds via theta structures and is supported by the cellular Bloom helicase, which interacts directly with E1 and whose engagement in HPV11 replication requires UAF1-USP1 activity. We propose that the unidirectional replication of the HPV11 genome initiates from replication fork restart events. These findings suggest a new role for the Fanconi anemia pathway in HPV replication.IMPORTANCEHuman papillomaviruses (HPVs) are important pathogens that replicate their double-stranded circular DNA genome in the nucleus of infected cells. HPV genomes replicate in infected cells via bidirectional theta replication and a poorly understood unidirectional mechanism, and the onset of viral replication requires the engagement of cellular DNA damage response pathways. In this study, we showed that the previously described interaction between the viral E1 helicase and the cellular UAF1-USP1 complex is necessary for the completion of bidirectional replication and the subsequent initiation of the unidirectional replication mechanism. Our results suggest HPVs may use the cellular Fanconi anemia DNA damage pathway to achieve the separation of daughter molecules generated by bidirectional theta replication. Additionally, our results indicate that the unidirectional replication of the HPV genome is initiated from restarted bidirectional theta replication forks.


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