scholarly journals Localized Breast Amyloidosis Associated with Sjörgren Syndrome

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Edgar G. Fischer

Sjörgren syndrome is a systemic autoimmune disease that is rarely associated with amyloid deposits, and in most reported cases, these deposits are localized to a single organ. Amyloidosis of the breast is a rare and unexpected finding, and only 5 case series with 63 patients have been published in the past 40 years. To date, only 6 cases have been reported in which Sjörgren syndrome is associated with amyloid deposits in the breast. A 61-year-old female diagnosed with Sjörgren syndrome underwent a breast needle core biopsy for calcifications. Microscopic examination revealed amyloid deposits in the periductular basement membranes, in the walls of arteries and veins, and in the surrounding connective tissue. No malignancy was found. Clinical workup revealed the amyloid deposits to be localized to the breast and did not reveal an underlying hematolymphoid neoplasm. The association between Sjörgren syndrome and breast amyloidosis is rare, but few reports have appeared in recent years, and it may be an emerging disease association. The finding of localized amyloid in the breast and other organs should lead to a clinical workup not only for hematopoietic neoplasms but also for autoimmune diseases such as Sjörgren syndrome.

2015 ◽  
Vol 93 (5) ◽  
pp. 425-429 ◽  
Author(s):  
Heather J. Dean ◽  
Elizabeth A.C. Sellers

Prior to 1985, type 2 diabetes was a disease of adults. Simultaneously with the global epidemic of childhood obesity, type 2 diabetes has increased in children. Initially, the presentation of small case series of type 2 diabetes in children was met with skepticism. As the number and size of the case series grew and the first long-term outcomes of end-stage complications in young adults appeared in the literature, the international community took notice with guarded interest. Type 2 diabetes disproportionately affects the children of specific ethnic groups and from disadvantaged socioeconomic environments, especially Indigenous populations. The past decade has seen unprecedented intense global interest in the etiology, treatment, and prevention of type 2 diabetes in children.


2000 ◽  
Vol 124 (3) ◽  
pp. 353-356
Author(s):  
J. Peter R. Pelletier ◽  
Julie A. Plumbley ◽  
Elizabeth A. Rouse ◽  
Stephen J. Cina

Abstract Context.—Clostridium septicum infections are rare but often associated with serious if not fatal outcomes. Clostridium septicum infection does not appear to be associated with a single specific defect in cellular or humoral immunity. It has been associated with multiple medical problems, including but not limited to leukemia, malignancy of the bowel, other solid tumors, cyclic neutropenia with enterocolitis, diabetes mellitus, and severe arteriosclerosis. Most cases of C septicum are associated with malignancy, and mortality approaches 100% if care is not rendered within 12 to 24 hours. Objectives.—To evaluate outcomes of patients with C septicum bacteremia, whether treated medically or surgically or both, and to note associated conditions. Design.—Retrospective evaluation of patients found to have C septicum bacteremia in the past 6 years. Setting.—Two teaching hospitals, Brooke Army Medical Center (250 beds) and Wilford Hall Medical Center (292 beds), were the source of our patients. Patients.—All patients found to have C septicum bacteremia during hospitalization or postmortem examination were included in the study. There were no exclusion criteria. Main Outcome Measure.—Mortality associated with C septicum infection. Results.—In our case series, mortality was 33%, which is slightly lower than reported in prior studies (43%–70%). Conclusion.—Presumptive identification based on Gram stain, awareness of C septicum infection as a paraneoplastic syndrome, and prompt, clear communication between laboratory personnel and clinicians are necessary for early diagnosis of C septicum infection. Early institution of antibiotic therapy improves prognosis.


Materials ◽  
2019 ◽  
Vol 12 (9) ◽  
pp. 1524 ◽  
Author(s):  
Gabriele Cervino ◽  
Luca Fiorillo ◽  
Ines Paola Monte ◽  
Rosa De Stefano ◽  
Luigi Laino ◽  
...  

Background: Nowadays, patients involved in antiplatelet therapy required special attention during oral surgery procedures, due to the antiplatelet drugs assumption. The motivations of the assumption may be different and related to the patient’s different systemic condition. For this reason, accordingly to the current international guidelines, different protocols can be followed. The aim of this work is to analyze how the dentist’s approach to these patients has changed from the past to the present, evaluating the risk exposure for the patients. Methods: This review paper considered different published papers in literature through quoted scientific channels, going in search of “ancient” works in such a way as to highlight the differences in the protocols undertaken. The analyzed manuscripts are in the English language, taking into consideration reviews, case reports, and case series in such a way as to extrapolate a sufficient amount of data and for evaluating the past therapeutic approaches compared to those of today. Results: Colleagues in the past preferred to subject patients to substitution therapy with low molecular weight anticoagulants, by suspending antiplatelet agents to treatment patients, often for an arbitrary number of days. The new guidelines clarify everything, without highlighting an increased risk of bleeding during simple oral surgery in patients undergoing antiplatelet therapy. Conclusion: Either patients take these medications for different reasons, because of cardiovascular pathologies, recent cardiovascular events, or even for simple prevention, although the latest research shows that there is no decrease of cardiovascular accidents in patients who carry out preventive therapy. Surely, it will be at the expense of the doctor to assess the patient’s situation and risk according to the guidelines. For simple oral surgery, it is not necessary to stop therapy with antiplatelet agents because the risk of bleeding has not increased, and is localized to a post-extraction alveolus or to an implant preparation, compared to patients who do not carry out this therapy. From an analysis of the results it emerges that the substitutive therapy should no longer be performed and that it is possible to perform oral surgery safely in patients who take antiplatelet drugs, after a thorough medical history. Furthermore, by suspending therapy, we expose our patients to more serious risks, concerning their main pathology, where present.


2014 ◽  
Vol 48 (3) ◽  
pp. 132-138
Author(s):  
Sunil Dogra ◽  
Samir Malhotra ◽  
Promila Pandhi ◽  
Sharonjeet Kaur ◽  
Sujit Rajagopalan ◽  
...  

ABSTRACT Background Toxic epidermal necrolysis (TEN) is a rare idiosyncratic mucocutaneous reaction associated with high mortality. Drugs are most commonly implicated in TEN. The treatment constitutes stopping the offending drug, along with symptomatic management. In this study, we searched for case reports/series of TEN and analyzed data to find the most commonly implicated drugs in TEN, effects of use of corticosteroids on mortality in TEN patients, changing trends in mortality over the past 3 decades and difference in mortality rates in both developing and developed countries. Materials and methods We searched for case reports/series of TEN to evaluate most commonly implicated drugs in TEN, effects of use of corticosteroids on mortality in TEN patients, changing trends in mortality over the past three decades and difference in mortality rates in both developing and developed countries. Results Antibiotics (28.6%) followed by antiepileptics (17.4%) and nonsteroidal anti-inflammatory drugs (9.6%) are most commonly implicated. There was nonsignificant decrease in mortality among steroids users as compared to nonusers (OR = 2.0, CI 0.96-4.24). During the period between 1980 and 1989 the reported mortality in TEN cases was approximately 33.4%, which decreased to 27% in the next two decades. There was a nonsignificant difference in mortality in develo- ping countries as compared to developed countries (OR: 0.70, CI 0.32-1.53). Conclusion Corticosteroids have been associated with non significant reduction in the mortality. Apart from this, mortality did not differ over years together in both developing and developed countries. How to cite this article Kaur S, Rajagopalan S, Shafiq N, Dogra S, Srinivasan A, Pandhi P, Malhotra S. Drugs Implicated, Mortality and Use of Corticosteroids in Toxic Epidermal Necrolysis Cases: A Systematic Review of Published Case Reports and Case Series. J Postgrad Med Edu Res 2014;48(3):132-138.


2020 ◽  
Vol 21 (21) ◽  
pp. 8247
Author(s):  
Alina Christine Hilger ◽  
Gabriel Clemens Dworschak ◽  
Heiko Martin Reutter

The treatment of major birth defects are key concerns for child health. Hitherto, for the majority of birth defects, the underlying cause remains unknown, likely to be heterogeneous. The implicated mortality and/or reduced fecundity in major birth defects suggest a significant fraction of mutational de novo events among the affected individuals. With the advent of systematic array-based molecular karyotyping, larger cohorts of affected individuals have been screened over the past decade. This review discusses the identification of disease-causing copy-number variations (CNVs) among individuals with different congenital malformations. It highlights the differences in findings depending on the respective congenital malformation. It looks at the differences in findings of CNV analysis in non-isolated complex congenital malformations, associated with central nervous system malformations or intellectual disabilities, compared to isolated single organ-system malformations. We propose that the more complex an organ system is, and the more genes involved during embryonic development, the more likely it is that mutational de novo events, comprising CNVs, will confer to the expression of birth defects of this organ system.


2018 ◽  
Vol 5 (suppl_1) ◽  
pp. S669-S669
Author(s):  
Nathan Summers ◽  
Colleen Kelley ◽  
Wendy Armstrong ◽  
Vincent Marconi ◽  
Minh Ly Nguyen

2019 ◽  
Vol 15 (2) ◽  
pp. 713-733 ◽  
Author(s):  
Johannes Hepp ◽  
Lorenz Wüthrich ◽  
Tobias Bromm ◽  
Marcel Bliedtner ◽  
Imke Kathrin Schäfer ◽  
...  

Abstract. Causes of the Late Glacial to Early Holocene transition phase and particularly the Younger Dryas period, i.e. the major last cold spell in central Europe during the Late Glacial, are considered to be keys for understanding rapid natural climate change in the past. The sediments from maar lakes in the Eifel, Germany, have turned out to be valuable archives for recording such paleoenvironmental changes. For this study, we investigated a Late Glacial to Early Holocene sediment core that was retrieved from the Gemündener Maar in the Western Eifel, Germany. We analysed the hydrogen (δ2H) and oxygen (δ18O) stable isotope composition of leaf-wax-derived lipid biomarkers (n-alkanes C27 and C29) and a hemicellulose-derived sugar biomarker (arabinose), respectively. Both δ2Hn-alkane and δ18Osugar are suggested to reflect mainly leaf water of vegetation growing in the catchment of the Gemündener Maar. Leaf water reflects δ2H and δ18O of precipitation (primarily temperature-dependent) modified by evapotranspirative enrichment of leaf water due to transpiration. Based on the notion that the evapotranspirative enrichment depends primarily on relative humidity (RH), we apply a previously introduced “coupled δ2Hn-alkane–δ18Osugar paleohygrometer approach” to reconstruct the deuterium excess of leaf water and in turn Late Glacial–Early Holocene RH changes from our Gemündener Maar record. Our results do not provide evidence for overall markedly dry climatic conditions having prevailed during the Younger Dryas. Rather, a two-phasing of the Younger Dryas is supported, with moderate wet conditions at the Allerød level during the first half and drier conditions during the second half of the Younger Dryas. Moreover, our results suggest that the amplitude of RH changes during the Early Holocene was more pronounced than during the Younger Dryas. This included the occurrence of a “Preboreal Humid Phase”. One possible explanation for this unexpected finding could be that solar activity is a hitherto underestimated driver of central European RH changes in the past.


2018 ◽  
Vol 34 (03) ◽  
pp. 312-317 ◽  
Author(s):  
Andrew Frankel ◽  
Oren Friedman ◽  
Leo Wang

AbstractPolydioxanone (PDS) foil is widely recognized as a septal cartilage replacement during rhinoplasties and is thought to be completely resorbable and biodegradable. Since its United States Food and Drug Administration approval in 2010, PDS foil has drawn significant enthusiasm and many surgeons consider it an ideal implantable biomaterial as reflected in numerous studies highlighting its benefits. However, scant literature exists highlighting relevant complications of PDS plates that may potentially lead to cavalier overuse. This descriptive case series assesses the outcomes of PDS foil usage in three patients seen for septoplasty at two independent institutions over the past 5 years. Our results demonstrate that PDS plate usage can lead to septal cartilage loss and resultant saddle nose deformıty associated with prolonged postoperative edema and inflammation. To our knowledge, this is the largest case series of this reported phenomenon.


1977 ◽  
Author(s):  
H. Koestering ◽  
H. Girndt ◽  
K.U. Schnuett ◽  
M.A. Guerrero

In patients with chronic renal insufficiency undergoing chronic hemodialysis or heme— filtration, shunts uith great blood flous are always needed. In the past feu years ue have preferred Brescia-Cimino shunts (BCS). As there are only a feu arteries and veins, from uhich shunts are able to be formed, ue aluays try to save clotted shunts. Ide have performed thrombolytic therapy uith StreptokinaseR 24 times. Immediately following the clotting of a BCS ue began hemodialytic treatment for about 10-12 hours. Afterwards, according to the ASTK titres, an initial dose of StreptokinaseR uas applied; this uas folloued by continuous doses of about 100,000 units/hour. If another hemodialytic treatment uas necessary before reopening the shunt, ue started this only after 3 days uith a reduced dose of StreptokinaseR (50% of the original amount). In 22 cases this thrombolytic therapy uas successful uithin 3-120 hours, in 2 cases the shunts remained clotted. In three of the successfully treated patients, the shunts had been clotted for 19, 28 and 40 days. Ide believe that thrombolytic therapy uith StreptokinaseR in patients uith clotted BCS has proven to be successful and dependable.


2019 ◽  
Vol 57 (5) ◽  
pp. 646-655 ◽  
Author(s):  
Puneet Batra ◽  
Bruno Frazāo Gribel ◽  
B. A. Abhinav ◽  
Anika Arora ◽  
Sreevatsan Raghavan

Presurgical infant orthopedics (PSIO) is done to reduce the size of the cleft defect along with improving the arch alignment and nasolabial aesthetics in patients with cleft lip and palate, leading to an improvement of nasolabial aesthetics allowing for a tidier and more aesthetic reparative procedure and postsurgical scar. Since the 2000s, clear aligners have slowly and steadily treaded their way as an acceptable orthodontic modality, with their usage and acceptability increasing considerably over the past decade. Thus, from the knowledge gathered in its 10 years working with 3-dimensional (3-D) diagnosis, treatment planning, and 3-D Printing services, Compass 3D (Belo Horizonte, Brazil) developed the OrthoAligner NAM system. This case series highlights one of the world’s first documented cases of PSIO treated with a series of clear aligners.


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