Severe pemphigoid gestationis responsive to intravenous immunoglobulins after initial treatment failure of combined oral steroids and cyclosporin

2021 ◽  
Vol 31 (2) ◽  
pp. 245-246
Author(s):  
Lisa Killion ◽  
Paula Beatty ◽  
Emma Tierney ◽  
Caitriona Hackett ◽  
Anne Marie Tobin ◽  
...  
Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012511
Author(s):  
Zachary M Grinspan ◽  
Kelly G Knupp ◽  
Anup D Patel ◽  
Elissa G Yozawitz ◽  
Courtney J Wusthoff ◽  
...  

Objective.Compare the effectiveness of initial treatment for infantile spasms.Methods.The National Infantile Spasms Consortium prospectively followed children with new onset infantile spasms that began at age 2-24 months at 23 US centers (2012-2018). Freedom from treatment failure at 60 days required no second treatment for infantile spasms and no clinical spasms after 30 days of treatment initiation. We managed treatment selection bias with propensity score weighting and within-center correlation with generalized estimating equations.Results.Freedom from treatment failure rates were: ACTH 88/190 (46%), oral steroids 42/95 (44%), vigabatrin 32/87 (37%), and non-standard therapy 4/51 (8%). Changing from oral steroids to ACTH was not estimated to affect response (observed 44% estimated to change to 44% [95% CI 34–54]). Changing from non-standard therapy to ACTH would improve response from 8% to 39 [17–67]%, and to oral steroids from 8% to 38 [15–68]%. There were large but not statistically significant estimated effects of changing from vigabatrin to ACTH (29% to 42 [15–75]%), vigabatrin to oral steroids (29% to 42 [28–57]%), and non-standard therapy to vigabatrin (8% to 20 [6-50]%). Among children treated with vigabatrin, those with tuberous sclerosis complex (TSC) responded more often than others (62% vs 29%; p<0.05)Conclusion.Compared to non-standard therapy, ACTH and oral steroids are superior for initial treatment of infantile spasms. The estimated effectiveness of vigabatrin is between ACTH / oral steroids and non-standard therapy, though the sample was underpowered for statistical confidence. When used, vigabatrin worked best for TSC.Classification of Evidence:This study provides Class III evidence that for children with new onset infantile spasms, ACTH or oral steroids were superior to non-standard therapies.


2020 ◽  
Vol 105 (9) ◽  
pp. 886-890 ◽  
Author(s):  
Stuart Haggie ◽  
Hasantha Gunasekera ◽  
Chetan Pandit ◽  
Hiran Selvadurai ◽  
Paul Robinson ◽  
...  

ObjectiveEmpyema is the most common complication of pneumonia. Primary interventions include chest drainage and fibrinolytic therapy (CDF) or video-assisted thoracoscopic surgery (VATS). We describe disease trends, clinical outcomes and factors associated with reintervention.Design/setting/patientsRetrospective cohort of paediatric empyema cases requiring drainage or surgical intervention, 2011–2018, admitted to a large Australian tertiary children’s hospital.ResultsDuring the study, the incidence of empyema increased from 1.7/1000 to 7.1/1000 admissions (p<0.001). We describe 192 cases (174 CDF and 18 VATS), median age 3.0 years (IQR 1–5), mean fever duration prior to intervention 6.2 days (SD ±3.3 days) and 50 (26%) cases admitted to PICU. PICU admission increased during the study from 18% to 34% (p<0.001). Bacteraemia occurred in 23/192 (12%) cases. A pathogen was detected in 131/192 (68%); Streptococcus pneumoniae 75/192 (39%), S. aureus 25/192 (13%) and group A streptococcus 13/192 (7%). Reintervention occurred in 49/174 (28%) and 1/18 (6%) following primary CDF and VATS. Comparing repeat intervention with single intervention cases, a continued fever postintervention increased the likelihood for a repeat intervention (OR 1.3 per day febrile; 95% CI 1.2 to 1.4, p<0.0001). Younger age, prolonged fever preintervention and previous antibiotic treatment were not associated with initial treatment failure (all p>0.05).ConclusionWe report increasing incidence and severity of empyema in a large tertiary hospital. One in four patients required a repeat intervention after CDF. Neither clinical variables at presentation nor early investigations were able to predict initial treatment failure.


2012 ◽  
Vol 32 (1) ◽  
pp. 114-119 ◽  
Author(s):  
Bradley N. Gaynes ◽  
Stacie B. Dusetzina ◽  
Alan R. Ellis ◽  
Richard A. Hansen ◽  
Joel F. Farley ◽  
...  

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Sadie Henry

Introduction. Pemphigoid gestationis (PG) is an autoimmune blistering disease that occurs in approximately 1 in 50,000 pregnancies. Failing to recognize PG may lead to inadequate maternal treatment and possible neonatal complications.Case Report. At 18 weeks of gestation, a 36-year-old otherwise healthy Caucasian G4P1 presented with pruritic papules on her anterior thighs, initially treated with topical steroids. At 31 weeks of gestation, she was switched to oral steroids after her rash and pruritus worsened. The patient had an uncomplicated SVD of a healthy female infant at 37 weeks of gestation and was immediately tapered off steroid treatment, resulting in a severe postpartum flare of her disease.Discussion. This case was similar to reported cases of pruritic urticarial papules followed by blisters; however, this patient had palm, sole, and mucous membrane involvement, which is rare. Biopsy for direct immunofluorescence or ELISA is the preferred test for diagnosis. Previous case reports describe severe postdelivery flares that require higher steroid doses. Obstetrical providers need to be familiar with this disease although it is rare, as this condition can be easily confused with other dermatoses of pregnancy. Adequate treatment is imperative for the physical and psychological well-being of the mother and infant.


2003 ◽  
Vol 98 (2) ◽  
pp. 337-341 ◽  
Author(s):  
Kelly D. Foote ◽  
William A. Friedman ◽  
Thomas L. Ellis ◽  
Frank J. Bova ◽  
John M. Buatti ◽  
...  

Object. The goal of this study was to evaluate the outcomes of patients who underwent repeated radiosurgery to treat a residual intracranial arteriovenous malformation (AVM) after an initial radiosurgical treatment failure. Methods. The authors reviewed the cases of 52 patients who underwent repeated radiosurgery for residual AVM at the University of Florida between December 1991 and June 1998. In each case, residual arteriovenous shunting persisted longer than 36 months after the initial treatment; the mean interval between the first and second treatment was 41 months. Each AVM nidus was measured at the time of the original treatment and again at the time of retreatment, and the dosimetric parameters of the two treatments were compared. After retreatment, patients were followed up and their outcomes were evaluated according to a standard posttreatment protocol for radiosurgery for AVMs. The mean original lesion volume was 13.8 cm3 and the mean volume at retreatment was 4.7 cm3, for an average volume reduction of 66% after the initial treatment failure. Only two AVMs (3.8%) failed to demonstrate size reduction after the primary treatment. The median doses on initial and repeated treatment were 12.5 and 15 Gy, respectively. Five patients were lost to follow up and five refused neuroimaging follow up. One patient died of a hemorrhage shortly after retreatment. Of the remaining 41 patients, 24 had evidence of cure, 15 on angiographic studies and nine on magnetic resonance (MR) images. Seventeen had evidence of treatment failure, 10 on angiographic studies and seven on MR images. By angiographic criteria alone, the cure rate after retreatment was 60%, whereas according to angiographic and MR imaging results, the cure rate was 59%. Conclusions. Although initial radiosurgical treatment failed to obliterate the AVM in these 52 patients, it did produce a substantial therapeutic effect (volume reduction). This size reduction commonly allowed higher doses to be delivered during radiosurgical retreatment. The results show rates of angiographically confirmed cure comparable to primary treatment and a low incidence of complications, indicating that salvage radiosurgical retreatment is a safe and effective therapy in cases of failed AVM radiosurgery.


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