scholarly journals AB0858 SUCCESSFUL TERM PREGNANCY IN A PATIENT WITH GRANULOMATOSIS WITH POLYANGIITIS AFTER UNDERGOING RITUXIMAB THERAPY - CASE REPORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1452.2-1453
Author(s):  
O. Sirenko ◽  
O. Kuryata ◽  
T. Lysunets ◽  
A. Legkobyt ◽  
H. Mostova

Background:Granulomatosis with polyangiitis (GWP) is a relatively rare disease with the lack of experience in the management of pregnancy currently. Potentially negative factors of pregnancy prognosis are both disease-related and teratogenic effects of specific vasculitis therapy [1].Objectives:To describe a clinical case of Successful term pregnancy in a patient with Granulomatosis with polyangiitis after undergoing rituximab therapy.Methods:19 years-old woman was admitted to the rheumatology department of Clinical Regional Hospital After Mechnicov in 2013 with the debut of Granulomatosis with polyangiitis. Her disease course included recurrent rhinitis and sinusitis, Granulomatosis of the eye orbits. Positive ANCA titers were present in the disease onset. In the preceding 7 years she had been treated with corticosteroids, cyclophospan (discontinuated in 2017 because of lack of efficiency), than azathioprine. Aseptic necrosis of the both femoral head was estimated on fifth disease year. In 2018 azathioprine therapy had been discontinued owing to the disease progression and biological agents were prescribed – rituximab with positive effect. The disease remission was achived by rituximab therapy, the patient was warned about the need for contraception. The last dose of rituximab was introduced in February 2020. However, in March 2020, the patient reported pregnancy and therapy was discontinued. At the onset of pregnancy she was treated with 6 mg oral methylprednisolone. Daily and this dose was stable till all pregnancy period. Fetal growth assessment, congenital abnormalities screening test, and laboratory tests for gestational diabetes and preeclampsia were unremarkable during all gestational trimesters. The ANCA titers remained negative and renal function was normal and there was no flare during all pregnancy period.Results:At 38 weeks’ gestation a spontataneous labor started without induction. A 3270-g healthy boy delivered with APGAR score of 9 at 1 minute and 5 minutes. Postpartum the disease remained in remission, and the patient was maintained on corticosteroids. The infant was healthy, with normal development.Conclusion:Thus, the use of biological agents therapy in patients with GWP shows possible ways to safe reproductive potential with disease remission achievement.References:[1]Daher A, Sauvetre G, Girszyn N, Verspyck E, Levesque H, Le Besnerais M. Granulomatosis with polyangiitis and pregnancy: A case report and review of the literature. Obstetric Medicine. 2020;13(2):76-82. doi:10.1177/1753495X18822581Disclosure of Interests:None declared

Author(s):  
Enzo Lüsebrink ◽  
Leonhard Binzenhöfer ◽  
Stefan Brunner ◽  
Jörg Hausleiter ◽  
Steffen Massberg ◽  
...  

Abstract Background Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic disease characterized by fibro-adipose degeneration of ventricular myocardium. Initial clinical presentation is variable and ranges from asymptomatic cases to chronic heart failure and sudden cardiac death due to malignant arrhythmias. Case summary Here, a 67-year-old male patient who started extensive physical training upon retirement and presented with ventricular tachycardia and progressive heart failure as a first sign of his disease. ARVC/D diagnosis was established according to the 2010 modified Task Force Criteria and supported by HRS/EHRA Consensus based genotyping. After initial discharge on optimal medical therayp and prophylactic ICD-implantation according to his individual ARVC risk score, the patient reported rapid decline in physical capacity on a regular follow-up four months later. To better understand the etiology of his clinical deterioration, we performed stress echocardiography, coronary angiogram and exercise right heart catheterization, which conclusively suggest impaired left ventricular (LV) filling secondary to RV failure as a main cause of global circulatory failure. Discussion The present case report focuses on relation of physical activity to disease onset and the concomitant advent of symptoms during exercise as well as a structured and guideline-aided diagnostic work-up in ARVC/D and staged treatment options. Continuous ARVC/D-center-oriented re-assessment and treatment planning including lifestyle intervention, psychological support, medical, surgical and interventional options are key elements of sustained long-term care for ARVC/D patients.


Author(s):  
V.M. Bolotskih, E.R. Semenova

A case of umbilical vein thrombosis is presented. Thrombotic masses were detected inside umbilical vein during ultrasound examination on the gestation age 40 weeks and 4 days. Such serious complication probably caused by decompensation of chronic placenta insufficiency in post-term pregnancy. In result reduce blood speed and forming thrombus inside umbilical vein


BMJ Open ◽  
2020 ◽  
Vol 10 (10) ◽  
pp. e037691
Author(s):  
Line Zinckernagel ◽  
Annette Kjær Ersbøll ◽  
Teresa Holmberg ◽  
Susanne S Pedersen ◽  
Helle Ussing Timm ◽  
...  

ObjectivesPsychosocial healthcare is recommended, but little is known about how patients perceive the level of care and whether subgroups of patients experience less psychosocial healthcare than others. We examined the prevalence of patient-reported psychosocial healthcare and factors predicting patient-reported lack of psychosocial healthcare among patients with heart disease.DesignA cohort study.SettingDenmark, nationwide.ParticipantsA registry-based random sample of 5000 patients with incident heart disease in 2013.MeasuresPatient-reported psychosocial healthcare was obtained from a survey and potential predictors before disease onset from registries. We used multivariable logistic regression analysis to determine predictors of patient-reported lack of care.ResultsWe received responses from 56%; 40% reported lacking information on psychosocial aspects, 51% lacking psychosocial rehabilitation and support and 32% reported lacking both types of psychosocial healthcare. The type of heart disease was the strongest predictor of patient-reported lack of psychosocial healthcare, especially among patients with atrial fibrillation (OR: 3.11–3.98). Older age (OR: 1.48–2.05), female gender (OR: 1.27–1.53) and no contact with general practitioner (OR: 1.47–1.84) also predicted patient-reported lack of psychosocial healthcare. Patients outside the labour force (OR: 1.29) and living in the capital region (OR: 1.50) more frequently reported lacking psychosocial rehabilitation and support, and patients with recent (OR: 1.63) or past (OR: 1.33) anxiety or depression and severe comorbidities (OR: 1.34) more frequently reported lacking both types of psychosocial healthcare.ConclusionsMany patients with heart disease reported lacking psychosocial healthcare. Importantly, patients who most need psychosocial healthcare are not those who report receiving it. Our results call for action to translate guidelines into clinical practice.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 668.2-669
Author(s):  
S. Monti ◽  
P. Delvino ◽  
C. Klersy ◽  
G. Coppa ◽  
A. Milanesi ◽  
...  

Background:Patient-reported outcomes (PROs) are currently poorly integrated in the clinical evaluation of disease activity in patients with ANCA-associated vasculitis (AAV).Objectives:To assess the distribution of the Patient Global Assessment (PtGA) in patients with AAV in stable remission, and to identify correlates of PtGA; to assess the discordance between PtGA score and PhGA.Methods:Patients with a diagnosis of AAV [eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis] in stable, complete remission (defined by a BVAS=0) and with a Physician Global Assessment (PhGA)=0 were included. A questionnaire including several aspects of disease captured by PROs was collected. PtGA on a 0-100 mm visual analogue scale (VAS) was assessed, with higher scores representing higher/worse levels of disease activity. Similarly, VAS for pain, chronic damage according to the patient’s opinion, general health (GH), fatigue, and sleep quality were collected. The worst symptom in the patient’s opinion affecting the overall assessment of disease activity was recorded. The Cragg Hurdle model was used to assess the predictors of PtGA.Results:65 patients were included, female 57%, mean age 61±12 years. Mean disease duration at enrollment was 8±6 years. Mean vasculitis damage index (VDI) was 4.4 ±2.3, with 45% of patients having a VDI ≥ 5. Despite having been classified as being in remission, PtGA was elevated in 37% of patients. We explored several correlates of PtGA. Higher degree of damage accrual (VDI) did not influence the patient’s evaluation of current disease activity. Similarly, we did not identify a correlation between older age, educational level, number of organ-systems involved, number of comorbidities, the number of previous major or minor relapses, higher disease duration, nor the type of AAV diagnosis (figure 1, panel A). Only sex significantly correlated with PtGA scores: 19 (51%) of female patients reported an elevated PtGA compared to only 5 (18%) of male (p=0.009). PtGA resulted to be significantly correlated with other (mostly modifiable) PROs including VAS pain, perception of the level of chronic damage accrual, GH, and fatigue (figure 1, panel B). The agreement between patients’ and physicians’ assessments of disease activity was 63%. Patients reported pain, followed by chronic respiratory symptoms to be the worst-experienced ongoing manifestations affecting their evaluation of disease activity.Conclusion:A significant proportion of patients with AAV considered to be in remission by the physician still declares to have persistent aspects of uncontrolled disease. PtGA is significantly influenced by persistent pain and fatigue, which warrant better assessment in the future.Disclosure of Interests:None declared


Neurosurgery ◽  
2019 ◽  
Vol 87 (5) ◽  
pp. E573-E577
Author(s):  
C Michael Honey ◽  
Marie T Krüger ◽  
Alan R Rheaume ◽  
Josue M Avecillas-Chasin ◽  
Murray D Morrison ◽  
...  

Abstract BACKGROUND AND IMPORTANCE Hemi-laryngopharyngeal spasm (HeLPS) has been recently described but is not yet widely recognized. Patients describe intermittent coughing and choking and can be cured following microvascular decompression of their Xth cranial nerve. This case report and literature review highlight that HeLPS can co-occur with glossopharyngeal neuralgia (GN) and has been previously described (but not recognized) in the neurosurgical literature. CLINICAL PRESENTATION A patient with GN and additional symptoms compatible with HeLPS is presented. The patient reported left-sided, intermittent, swallow-induced, severe electrical pain radiating from her ear to her throat (GN). She also reported intermittent severe coughing, throat contractions causing a sense of suffocation, and dysphonia (HeLPS). All her symptoms resolved following a left microvascular decompression of a loop of the posterior inferior cerebellar artery that was pulsating against both the IXth and Xth cranial nerves. A review of the senior author's database revealed another patient with this combination of symptoms. An international literature review found 27 patients have been previously described with symptoms of GN and the additional (but not recognized at the time) symptoms of HeLPS. CONCLUSION This review highlights that patients with symptoms compatible with HeLPS have been reported since 1926 in at least 4 languages. This additional evidence supports the growing recognition that HeLPS is another neurovascular compression syndrome. Patients with HeLPS continue to be misdiagnosed as conversion disorder. The increased recognition of this new medical condition will require neurosurgical treatment and should alleviate the suffering of these patients.


PEDIATRICS ◽  
1982 ◽  
Vol 70 (1) ◽  
pp. 135-136
Author(s):  
Deborah Tolchin ◽  
Mordecai Koenigsberg ◽  
Maria Santorineou

There have been many reports of the association between hemihypertrophy and intraabdominal masses, including Wilms' tumor, hepatoma, and adrenal cortical neoplasias.1 The present report describes a patient with segmental hemihypertrophy, multiple ovarian cysts, and bilateral Wilms' tumor and suggests a screening regimen for patients with hemihypertrophy. CASE REPORT The patient was a 9 lb 14 oz product of a term pregnancy, who was well until a mass filling the entire right side of the abdomen was discovered on routine examination at 4 months of age. Intravenous pyelogram (IVP) confirmed a large prerenal mass which on ultrasound was felt to be an ovarian cyst.


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