A Case of Photosensitivity Diagnosed as Xeroderma Pigmentosa Type A after 5 Years without Medical Follow-up

2011 ◽  
Vol 73 (4) ◽  
pp. 354-357
Author(s):  
Futoshi KOHDA ◽  
Makoto NOZAKI ◽  
Rikako SASAKI ◽  
Mieko HATA ◽  
Chikako NISHIGORI
Keyword(s):  
Type A ◽  
2021 ◽  
pp. 152660282098527
Author(s):  
Jan Stana ◽  
Carlota Fernandes Prendes ◽  
Ramin Banafsche ◽  
Nikolaos Konstantinou ◽  
Barbara Rantner ◽  
...  

Purpose: To demonstrate the feasibility of urgent endovascular treatment of a chronic type A dissection and contained rupture of the false lumen using a noncustomized triple-branched arch endograft, which necessitated reassignment of the branches to the supra-aortic vessels. Case Report:: A 57-year-old patient with a contained rupture of the descending thoracic aorta, in the setting of a chronic type A dissection and a maximum aortic diameter of 85 mm, was converted to endovascular repair after failure of an open surgical approach. A custom-made triple-branched arch endograft designed for another patient was employed, with concomitant occlusion of the false lumen using a Candy Plug occluder. To adjust the graft’s configuration to the patient’s anatomy, the supra-aortic vessels were not assigned to the originally planned branches. The 12-month follow-up angiography demonstrated a satisfactory result. Conclusion: A noncustomized triple-branched arch endograft can be used in an emergency setting to treat chronic type A dissection, reassigning the branches to the supra-aortic vessels as needed.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1341.2-1341
Author(s):  
B. Hernández-Cruz ◽  
F. J. Olmo Montes ◽  
M. J. Miranda García ◽  
M. D. Jimenez Moreno ◽  
M. A. Vázquez Gómez ◽  
...  

Background:The Virgen Macarena University Hospital belongs to the Public Health System of Andalusia and serves 481,296 inhabitants in Seville, Spain. In 2018 the Fracture Liaison Service switched to a multidisciplinary unit.Objectives:To describe FLS, to know the characteristics of patients with emphasis on gender differences and to know the completion of International Osteoporosis Foundation quality standards.Methods:Prospective, observational, analytical, research of usual clinical practice. All the consecutive patients attended from May 2018 to October 2019, ≥50 years, with a fragility fracture (occurred in the previous 24 months) were included. The study was approved by the Ethics Committee, Code 1084-N-16.Results:Our FLS is a type A multidisciplinary Unit, with a high level of intervention in the evaluation, estimation of fracture risk and fall risk, treatment prescription and follow-up of the patients. We included 408 patients, 80% females, one third with ≥80 years. Fragility fractures recorded in 328 women were hip (132, 40%), clinical vertebral (81, 25%) and no hip no vertebral (115, 35%). Those recorded in 82 males were hip (53, 66%), clinical vertebral (20, 24%) and no hip no vertebral (9, 10%), p=0.0001. Males had a higher rate of secondary causes of OP, drinker, and smoking. The most relevant gender difference was the low percentage of patients receiving pre-FF OP treatment. Forty-nine (16%) women versus 9 (7%) males had received it at some point in their life, p=0.04. Two hundred and seventy-one (86%) women vs 48 males (63%) had received it at after their FF in their reference unit, and all them were treated after the FLS evaluation. The probability of a male not receiving prior treatment was 2.5 (95% CI 1.01- 6.51); p=0,04. This probability was 0.64 (0.38-1.09) after the FF. After twelve months of follow-up in FLs, 96% continued treatment, with no differences between men and women. The completion of IOF quality standards was bad (red light) for patient identification items and FLS reference time. It was poor (amber traffic light) for initial OP screening standard and was good (green light) for the remaining 10 indicators. The completion of IOF quality standards was bad (red light) for patient identification items and FLS reference time. It was poor (amber traffic light) for initial OP screening standard and was good (green light) for the remaining 10 indicators (Figure 1).Figure 1.Figure 1.Conclusion:The FLS is a multidisciplinary type A. Its operation has narrowed the gap in diagnosis, treatment, and follow-up of FF patients, especially males. It is essential to improve patient recruitment, reduce referral times and increase the overall assessment of the patients.References:[1]Ganda K. et al. Models of care for the secondary prevention of osteoporotic fractures: a systematic review and meta-analysis, Osteoporos Int 2013;24:293-406.[2]Javaid MK et al. A patient-level key performance indicator set to measure the effectiveness of fracture liaison services and guide quality improvement: a position paper of the IOF Capture the Fracture Working Group, National Osteoporosis Foundation and Fragility Fracture Network. Osteoporos Int. 2020 Jul;31(7):1193-1204.Acknowledgements:Spanish Society of Research in Mineral and Bone Metabolism for its support through the competitive project FLS Excellence 2018 to obtain a training grant from the case management nurse.Disclosure of Interests:Blanca Hernández-Cruz Speakers bureau: Sociedad Española de Reumatología, Abbvie, Roche, Bristol, MSD, Lilly, Pfizer, Amgen, Sanofi, Consultant of: Abbvie, Lilly, Sanofi, STADA, UCB, Amgen, Galapagos., Grant/research support from: Fundación para la Investigación Sevilla, Junta de AndalucíaFundación Andaluza de Reumatología, Sociuedad Española de Reumatología., Francisco Jesús Olmo Montes: None declared., Maria José Miranda García: None declared., María Dolores Jimenez Moreno: None declared., María Angeles Vázquez Gómez: None declared., Mercedes Giner García: None declared., Miguel Angel Colmenero Camacho: None declared., José Javier Pérez Venegas: None declared., María José Montoya García: None declared.


2021 ◽  
pp. 175319342098321
Author(s):  
Anyuan Wang ◽  
Jian Ding ◽  
Long Wang ◽  
Tinggang Chu ◽  
Zhipeng Wu ◽  
...  

We present the MRI findings for 39 Wassel Type IV duplicated thumbs in 38 patients. We found that MRI revealed the morphology of the cartilaginous connection between the thumb anlages and the location of the deviation corresponding to the classification of Horii, which allowed precise preoperative planning of corrective osteotomies. All 39 thumbs were available for follow-up after surgical reconstruction at a mean of 29 months (range 25 to 39). Four out of nine Horii Type A cases and all 12 Type B, as well as the six Type C and the six Type D cases, achieved good results according to the Tada scoring system. Five Type A cases achieved fair results with residual stiffness of the interphalangeal joint. No secondary operations were needed. We conclude that MRI proved useful in subclassifying Wassel Type IV duplicated thumbs and may aid in planning the osteotomies needed for their reconstruction. Level of evidence: IV


Stroke ◽  
2021 ◽  
Author(s):  
Yueshan Feng ◽  
Jiaxing Yu ◽  
Jiankun Xu ◽  
Chuan He ◽  
Lisong Bian ◽  
...  

Background and Purpose: Paravertebral arteriovenous shunts (PVAVSs) are rare. Whether the intradural venous system is involved in drainage may lead to differences in clinical characteristics through specific pathophysiological mechanisms. This study aims to comprehensively evaluate the natural history and clinical outcomes of PVAVSs with or without intradural drainage. Methods: Sixty-four consecutive patients with PVAVSs from 2 institutes were retrospectively reviewed. Lesions were classified as type A (n=28) if the intradural veins were involved in drainage; otherwise, they were classified as type B (n=36). The clinical course from initial presentation to the last follow-up was analyzed. Results: The patients with type A shunts were older at presentation (52.5 versus 35.5 years, P <0.0001) and more likely to have lower spinal segments affected than patients with type B PVAVSs (67.8% versus 13.9%, P =0.00006). After presentation, the deterioration rates related to gait and sphincter dysfunction were significantly higher in patients with type A than type B shunts (gait dysfunction: 71.8%/y versus 17.0%/y, P =0.0006; sphincter dysfunction: 63.7%/y versus 11.3%/y, P =0.0002). According to the angiogram at the end of the latest treatment, 79% of type A and 75% of type B PVAVSs were completely obliterated. If the lesions were partially obliterated, a significantly higher clinical deterioration rate was observed in patients with type A shunts than those with type B shunts (69.9%/y versus 3.2%/y, P =0.0253). Conclusions: Type A PVAVSs feature rapid progressive neurological deficits; therefore, early clinical intervention is necessary. For complex lesions that cannot be completely obliterated, surgical disconnection of all refluxed radicular veins is suggested.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Andrea Colli ◽  
Laura Besola ◽  
Lorenzo Bagozzi ◽  
Erica Manzan ◽  
Eleonora Bizzotto ◽  
...  

Introduction: TOP-MINI is a new micro invasive surgical procedure to treat degenerative mitral valve regurgitation due to flail/prolapse. Hypothesis: This prospective single centre study sought to assess the safety and effectiveness of the TOP-MINI procedure up to one year follow-up. Methods: Clinical and Echocardiographic outcomes were evaluated at 1, 3, 6 months and 1 year follow-up for all patients underwent TOP-MINI procedure from November 2013 to March 2015. Procedural success was defined as residual MR≤2+ at any time. Results: Sixty-one patients were treated during study period. One year survival was 96.7±2.3%. Freedom from MR>2+ is shown in figure 1 Panel A, Freedom from MR>2+ according to valve anatomy (Type A isolated P2 disease, Type B posterior multisegment disease, Type C anterior or bileaflet and/or calcified disease) is shown in Figure 1 Panel B. Freedom from MR>2+ according to STS risk profile is shown in Figure 2. The trend of Echocardiographic parameters is shown in Figure 3. Conclusions: TOP-MINI is a safe and effective procedure at 1 year FU. Residual MR is influenced by valve anatomy showing good results in Type A and B patients. Future techniques refinements are needed in order to improve outcomes of Type C patients. The lack of annuloplasty procedure does not influence negatively left ventricle reverse remodeling.


Aorta ◽  
2017 ◽  
Vol 05 (02) ◽  
pp. 61-63
Author(s):  
George Samanidis ◽  
Meletios Kanakis ◽  
Constantinos Ieromonachos ◽  
George Stavridis

AbstractA 48-year-old man was admitted to our hospital with chronic aortic dissection Stanford Type A. His diagnosis was confirmed by chest multi-detector computed tomography (CT). The patient underwent combined (i.e., hybrid) open and endovascular repair (frozen elephant trunk) in a one-stage operation with moderate hypothermic circulatory arrest and antegrade cerebral perfusion. His postoperative course was uneventful, and he was discharged home on postoperative day 9. At 2-year follow-up, chest CT angiography revealed complete shrinkage of the obliterated false lumen in the distal aortic arch and descending thoracic aorta.


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