scholarly journals Immune Thrombocytopenia and JAK2V617F Positive Essential Thrombocythemia: Literature Review and Case Report

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
M. A. Sobas ◽  
T. Wróbel ◽  
K. Zduniak ◽  
M. Podolak-Dawidziak ◽  
J. Rybka ◽  
...  

We present the case where immune thrombocytopenia (ITP) and essential thrombocythemia (ET) sequentially appeared in the space of twenty-one years of follow-up. Impaired platelet production is present in both diseases, but clinical presentation and treatment are different. On the basis of this case history a possible role of autoimmunity as a predisposing factor to myeloproliferation has been discussed.

Neurosurgery ◽  
2002 ◽  
Vol 50 (1) ◽  
pp. 191-194 ◽  
Author(s):  
Christopher J. Hammond ◽  
Bhupal Chitnavis ◽  
Christopher C. Penny ◽  
Anthony J. Strong

ABSTRACT OBJECTIVE AND IMPORTANCE Syringomyelia in association with the Dandy-Walker complex is rare and only 17 cases have been reported, mostly as autopsy findings or incidental reports in series for other conditions. We present a further case report and discuss the possible pathogenetic mechanisms of syrinx formation in the presence of the Dandy-Walker complex. We emphasize the role of foramen magnum obstruction and alteration in cerebrospinal fluid flow dynamics in syrinx formation. The therapeutic importance of disimpaction of the foramen magnum is stressed. CLINICAL PRESENTATION A 39-year-old man presented with headache. As a child, he had had cystoperitoneal and ventriculoperitoneal shunts inserted for hydrocephalus in association with the Dandy-Walker complex. On examination, the patient had limited upgaze but was otherwise normal. Magnetic resonance imaging revealed a large cervicothoracic syrinx and a Dandy-Walker cyst obstructing the foramen magnum. INTERVENTION The cystoperitoneal shunt was revised. The patient's headaches resolved, and follow-up imaging demonstrated resolution of the syrinx and disimpaction of the foramen magnum. CONCLUSION Disimpaction of the foramen magnum can resolve syringomyelia in patients with the Dandy-Walker complex. This can be achieved by a shunt procedure or by formal foramen magnum decompression. The rarity of the association between the Dandy-Walker complex and syringomyelia prevents firm conclusions from being made regarding the best management strategy for this condition.


2021 ◽  
Vol 28 (1) ◽  
pp. 5
Author(s):  
B.S. Santosh ◽  
A. Harish Kumar ◽  
Rajdeep Singh ◽  
Jerin Jose ◽  
D.M. Shivamrthy ◽  
...  

Introduction: Rhinosporidiosis is a chronic mucocutaneous infection caused by Rhinosporidium seeberi, which mainly affects the mucus membranes of the nose, oropharynx, skin, eyes, and genital mucosa. The purpose of this case report was to illustrate the importance of clinicians in understanding the unusual incidence of this fungal infection, clinical presentation, treatment modalities, and recurrence rate. Observations: A clinical case report showed rare occurrence of rhinosporidiosis in the lower third of the face. The lesion was surgically excised by electrocauterization at its base. Commentaries: The patient showed no signs of recurrence even after 3 years of follow-up. Further research should be carried out to analyze the role of genetic predisposition in causing zoonotic infection. Conclusion: Rhinosporidiosis should also be considered as the differential diagnosis when facial swelling is encountered.


2018 ◽  
Vol 24 (1) ◽  
pp. 46-54 ◽  
Author(s):  
Matei Luminita ◽  
Cojocaru Lucia ◽  
Ghinea Mihaela

Abstract We report a case of Prinzmetal angina as inaugural manifestation of coronary disease, in a young adult male patient, recently started on anagrelide for essential thrombocythemia. Moderate proximal left anterior descendent coronary artery stenosis was documented by angiography, and interventional or surgical revascularization has been discussed. Patient’s option was for medical therapy alone. Anagrelide was temporarily withdrawn and rechallenged uneventfully after a couple of months and clinical evolution is good at four years follow-up. The mechanism by which anagrelide could induce coronary spasm and ischemia remains to be clarified.


2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
T El-nakhal ◽  
S Eid ◽  
W Al-Jundi

Abstract Aim to describe good outcomes in a case with Aortic graft infection (AGI) managed with Bovine patch and outline management of AGI. Method Literature review utilising keywords ‘’Aortic graft infection’’AND’’Bovine Patch’’ revealed initially 1350 articles, all studies in English were included, all duplicates were removed, screening was performed using the PRISMA flow. To correlate with the findings, we followed up a 72-year-old patient who presented with aortic graft infection followed by graft explantation and a fashioned Bovine graft insertion and followed up for 1 year for any re-infection in the clinics. Results Literature search resulted 73 articles discussing the role of Bovine Patch as a choice in prevention of Aortic graft re-infection. In our case, there was no clinical nor radiological evidence of Bovine patch re-infection for over a year. Literature review showed that AGI is a rare but serious complication with mortality up to 60%.The main management is IV antibiotics, followed by graft explantation and consideration of re-implantation of various types of grafts. The European society of vascular surgery (ESVS) guidelines recommend re-implantation with an aortic antibiotic soaked graft, yet re-infection rate is quoted at 20%. however, in our case Bovine graft was free from re-infection at 1 year follow up. Conclusions IV anitbioics and explantation remain the mainstay for treatment of aortic grafts infection, however, there is increasing evidence that Bovine grafts show excellent freedom from re-infection at follow up. Hence, this might be the conduit of choice when performing such surgeries; however, more evidence is needed.


2020 ◽  
Vol 02 ◽  
Author(s):  
Masood Ghori ◽  
Nadya O. Al Matrooshi ◽  
Samir Al Jabbari ◽  
Ahmed Bafadel ◽  
Gopal Bhatnagar

: Infective Endocarditis (IE), a known complication of hemodialysis (HD), has recently been categorized as Healthcare-Associated Infective Endocarditis (HAIE). Single pathogen bacteremia is common, polymicrobial endocardial infection is rare in this cohort of the patients. We report a case of endocarditis caused by Enterococcus faecalis (E. faecalis) and Burkholderia cepacia (B. cepacia), a first ever reported combination of a usual and an unusual organism, respectively, in a patient on HD. Clinical presentation of the patient, its complicated course ,medical and surgical management ,along with microbial and echocardiographic findings is presented herein. The authors believe that presentation of this case of HAIE may benefit and contribute positively to cardiac science owing to the rare encounter of this organism as a pathogen in infective endocarditis and the difficulties in treating it.


1970 ◽  
Vol 5 (4) ◽  
pp. 61-66
Author(s):  
Patrícia Resende Penido ◽  
Rhanna Junqueira Westin de Carvalho ◽  
Roger Willian Moraes Mendes

RESUMOIntrodução: A Arterite de Takayasu (AT) consiste em uma vasculopatia de origem indefinida, sendo de caráter crônico, que afeta a aorta e seus ramos principais. Em gestantes é uma condição complexa, em que o tratamento clínico é realizado com restrições. A utilização de corticoides tem sido favorável no controle inflamatório, principalmente naqueles casos em que antes da gravidez se fazia uso de imunossupressores. Casuística: Foi relatado um caso de uma gestante portadora de AT, através da análise de prontuário e de exames complementares, sendo realizado o pré-natal pelas equipes de Obstetrícia e Reumatologia, onde foi realizado tratamento clínico com corticoides, mostrando uma evolução satisfatória, ocorrendo apenas uma hospitalização que foi seguida de uma cesárea na trigésima sexta semana de gravidez, com recém nato saudável de 3.810g. Discussão: A AT pode estar associada a várias etiologias, sendo a gênese pouco conhecida. O diagnóstico na maioria das vezes é demorado, pela dificuldade da suspeita clínica, além de demandar o uso de técnicas de imagem mais sofisticadas. A gestação associada é fenômeno raro, já que as portadoras são orientadas a evitarem a gravidez. O controle clínico permitiu uma gestação sem grandes complicações e serviu como meio para levar a gestação até praticamente o fim do terceiro trimestre. Conclusão: Deve-se ressaltar o papel do acompanhamento clínico, especialmente com esta pan-arterite, mostrando os medicamentos que podem ser utilizados nesse grupo, especialmente quando se usava imunossupressores antes da gravidez. Palavras chave: Arterite de Takayasu, Gravidez, Vasculite. ABSTRACT:Introduction: Takayasu's arteritis (TA) consists of a vascular disease of unknown origin and chronic nature, which affects the aorta and its main branches. In pregnant women it is a complex condition, in which the clinical treatment is performed with restrictions. The use of corticosteroids has been favorable to control inflammation, especially in those cases when immunosuppressant had been used before pregnancy. Case Report: A case of a pregnant patient with TA has been reported through the analysis of medical records and laboratory tests. The Obstetrics and Rheumatology staff performed the prenatal care, and clinical treatment with corticosteroids was done showing a satisfactory outcome. There was only one hospitalization of the patient, which was followed by a cesarean in the thirty sixth week of pregnancy.  The newborn was a healthy one, weighing 3.810g. Discussion: TA may be associated with several etiologies, and the genesis is little known. The diagnosis most often takes time due to the difficulty of clinical suspicion, and requires the use of more sophisticated imaging techniques. The pregnancy is rare phenomenon, since the carriers are advised to avoid pregnancy. The clinical management allowed a pregnancy without major complications and served as a means to carry the pregnancy to almost the end of the third quarter. Conclusion: The role of the clinical follow-up should be noticed especially with this pan-arteritis, showing the drugs that can be used in this group, especially when there is use of immunosuppressive drugs before pregnancy. Keywords: Takayasu Arteritis, Pregnancy, Vasculitis.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Zhicheng Zhang ◽  
Xiaowei Huang ◽  
Qian Chen ◽  
Demin Li ◽  
Qi Zhou ◽  
...  

Abstract Background Small intestine duplication cysts (SIDCs) are rare congenital anatomical abnormalities of the digestive tract and a rare cause of hematochezia. Case presentation We describe an adult female presented with recurrent hematochezia. The routine gastric endoscope and colonic endoscope showed no positive findings. Abdominal CT scan indicated intussusception due to the "doughnut" sign, but the patient had no typical symptoms. Two subsequent capsule endoscopes revealed a protruding lesion with bleeding in the distal ileum. Surgical resection was performed and revealed a case of SIDC measuring 6 * 2 cm located inside the ileum cavity. The patient remained symptom-free throughout a 7-year follow-up period. Conclusion SIDCs located inside the enteric cavity can easily be misdiagnosed as intussusception by routine radiologic examinations.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 689.1-690
Author(s):  
S. Monti ◽  
L. Dagna ◽  
C. Campochiaro ◽  
A. Tomelleri ◽  
G. Zanframundo ◽  
...  

Background:Giant cell arteritis (GCA) is the most frequent systemic vasculitis after the age of 50 years old. Recent interest in the processes of immune and vascular aging have been proposed as a disease risk factor. Data on the impact of age at diagnosis of GCA on the clinical course of the disease are scarceObjectives:To assess the role of age at diagnosis of GCA on the risk and time to relapseMethods:Centres participating in the Italian Society of Rheumatology Vasculitis Study Group retrospectively enrolled patients with a diagnosis of GCA until December 2019. The cohort was divided in tertiles according to age at diagnosis (≤ 72; 73-79; > 79 years old). Negative binomial regression was used to assess the relapse rate according to age groups, and Cox regression for time to first relapse.Results:Of 720 patients enrolled in 14 Italian reference centres, 711 had complete follow-up data (female 50%; mean age 75±7). Median follow-up duration was 34 months (IQR 16;70). Patients in the older group at diagnosis (> 79 years) had more frequent visual loss compared to the 73-79 and ≤ 72 age groups (31% vs 20% vs 7%; p<0.001), but lower rates of general symptoms (56% vs 70% vs 77%; p<0.001). Large-vessel (LV)-GCA was less frequent in the older group (18% vs 22% vs 43%; p<0.001). At least one relapse occurred in 47% of patients. Median time to relapse was 12 months (IQR 6;23). Age did not influence the rate of relapses [18 per 100 persons/years (95%CI 15;21) vs 19 (95% CI 17;22) vs 19 (95%CI 17;22)], nor the time to first relapse (Figure 1). LV-GCA, presentation with significantly elevated c-reactive protein (> 50 mg/L) and general symptoms were independent predictors of relapse.Conclusion:Age at diagnosis of GCA influenced the clinical presentation and risk of ischaemic complications, but did not affect the relapse rate during follow-up. LV-GCA occurred more frequently in younger patients and was an independent predictor of relapse risk, highlighting the need for a correct characterization of the clinical subtype at the early stages of disease.Disclosure of Interests:Sara Monti: None declared, Lorenzo Dagna Grant/research support from: Abbvie, BMS, Celgene, Janssen, MSD, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, SG, SOBI, Consultant of: Abbvie, Amgen, Biogen, BMS, Celltrion, Novartis, Pfizer, Roche, SG, and SOBI, Corrado Campochiaro Speakers bureau: Novartis, Pfizer, Roche, GSK, SOBI, Alessandro Tomelleri: None declared, Giovanni Zanframundo: None declared, Catherine Klersy: None declared, Francesco Muratore: None declared, Luigi Boiardi: None declared, Roberto Padoan: None declared, Mara Felicetti: None declared, Franco Schiavon: None declared, Milena Bond: None declared, Alvise Berti: None declared, Roberto Bortolotti: None declared, Carlotta Nannini: None declared, Fabrizio Cantini: None declared, Alessandro Giollo: None declared, Edoardo Conticini: None declared, angelica gattamelata: None declared, Roberta Priori: None declared, Luca Quartuccio Consultant of: Abbvie, Bristol, Speakers bureau: Abbvie, Pfizer, Elena Treppo: None declared, Giacomo Emmi: None declared, Martina Finocchi: None declared, Giulia Cassone: None declared, Ariela Hoxha Speakers bureau: Celgene, UCB, Novartis, Sanofi, Werfen, Rosario Foti Consultant of: lilly, sanofi, MSD, Janssen, Abbvie, BMS, celgene, roche, Speakers bureau: lilly, sanofi, MSD, Janssen, Abbvie, BMS, celgene, roche, Michele Colaci: None declared, Roberto Caporali Consultant of: AbbVie; Gilead Sciences, Inc.; Lilly; Merck Sharp & Dohme; Celgene; Bristol-Myers Squibb; Pfizer; UCB, Speakers bureau: Abbvie; Bristol-Myers Squibb; Celgene; Lilly; Gilead Sciences, Inc; MSD; Pfizer; Roche; UCB, Carlo Salvarani: None declared, Carlomaurizio Montecucco: None declared


2021 ◽  
pp. 115-120
Author(s):  
Melanie Ribau ◽  
Mário Baptista ◽  
Nuno Oliveira ◽  
Bruno Direito Santos ◽  
Pedro Varanda ◽  
...  

Partial physeal bars may develop after injury to the growth plate in children, eventually leading to disturbance of normal growth. Clinical presentation, age of the patient, and the anticipated growth will dictate the best treatment strategy. The ideal treatment for a partial physeal bar is complete excision to allow growth resumption by the remaining healthy physis. There are countless surgical options, some technically challenging, that must be weighted according to each case’s particularities. We reviewed the current literature on physeal bars while reporting the challenging case of a short stature child submitted to a femoral physeal bar endoscopic-assisted resection with successful growth resumption. This case dares surgeons to consider all options when treating limb length discrepancy, such as the endoscopic-assisted resection which might offer successful results.


Sign in / Sign up

Export Citation Format

Share Document