Case of Senear-Usher Syndrome (Pemphigus Foliaceus?) with Fatal Ending in One Year

Dermatology ◽  
1948 ◽  
Vol 97 (5-6) ◽  
pp. 272-278 ◽  
Author(s):  
Emery Kocsard
Keyword(s):  
Usher Syndrome ◽  
Pemphigus Foliaceus ◽  
One Year

scholarly journals Outcomes of Late Implantation in Usher Syndrome Patients

2016 ◽  
Vol 21 (02) ◽  
pp. 140-143 ◽  
Author(s):  
Ana Hoshino ◽  
Agustina Echegoyen ◽  
Maria Goffi-Gomez ◽  
Robinson Tsuji ◽  
Ricardo Bento
Keyword(s):  
Hearing Loss ◽  
Retrospective Study ◽  
Speech Perception ◽  
Cochlear Implants ◽  
Pure Tone ◽  
Autosomal Recessive ◽  
Usher Syndrome ◽  
Autosomal Recessive Disorder ◽  
Auditory Pathways ◽  
One Year

Introduction Usher syndrome (US) is an autosomal recessive disorder characterized by hearing loss and progressive visual impairment. Some deaf Usher syndrome patients learn to communicate using sign language. During adolescence, as they start losing vision, they are usually referred to cochlear implantation as a salvage for their new condition. Is a late implantation beneficial to these children? Objective The objective of this study is to describe the outcomes of US patients who received cochlear implants at a later age. Methods This is a retrospective study of ten patients diagnosed with US1. We collected pure-tone thresholds and speech perception tests from pre and one-year post implant. Results Average age at implantation was 18.9 years (5–49). Aided average thresholds were 103 dB HL and 35 dB HL pre and one-year post implant, respectively. Speech perception was only possible to be measured in four patients preoperatively, who scored 13.3; 26.67; 46% vowels and 56% 4-choice. All patients except one had some kind of communication. Two were bilingual. After one year of using the device, seven patients were able to perform the speech tests (from four-choice to close set sentences) and three patients abandoned the use of the implant. Conclusion We observed that detection of sounds can be achieved with late implantation, but speech recognition is only possible in patients with previous hearing stimulation, since it depends on the development of hearing skills and the maturation of the auditory pathways.

scholarly journals Involvement of the Areae Compositae of the Heart in Endemic Pemphigus Foliaceus

2019 ◽  
pp. 181-186
Author(s):  
Ana Maria Abreu-Velez ◽  
Yulieth A. Upegui-Zapata ◽  
Carlos A. Valencia-Yepes ◽  
Eduardo Upegui-Quiceno ◽  
Alejandra M. Jiménez-Echavarría ◽  
...  
Keyword(s):  
Left Ventricular Hypertrophy ◽  
Ventricular Hypertrophy ◽  
Usher Syndrome ◽  
Left Ventricular ◽  
Pemphigus Foliaceus ◽  
New Variant ◽  
Endemic Pemphigus Foliaceus ◽  
Microscopic Studies ◽  
Antigenic Reactivity ◽  
Control Study

Background: A new variant of endemic pemphigus foliaceus in El Bagre (El Bagre-EPF), Colombia, South America, shares features with Senear-Usher syndrome and occurs in an endemic fashion. Patients affected by El Bagre-EPF have heterogeneous antigenic reactivity not only to the skin but to other organs, including the heart. Here we test for autoantibodies to the areae compositaeof the heart (structure consisting of typical desmosomal amalgamated fascia adherensmolecules)and evaluate any possible clinical correlation. Methods: A case-control study comparing 45 patients and 45 controls from the endemic area, matched by demographics including age, gender, weight, work activities, and comorbidities, was performed. Direct and indirect immunofluorescence, immunohistochemistry, confocal microscopic studies, and echocardiogram studies were completed. Results: The main clinical abnormally seen in the El Bagre-EPF patients was left ventricular hypertrophy in 15/45 patients, compared with no such findings in the control population (P < 0.1). Seventy percent of El Bagre-EPF patients and none of the controls displayed polyclonal autoreactivity using different immunoglobulins and complement to the areae compositae of the heart using different methods and antibodies (P < 0.1). Conclusions: Patients affected by El Bagre-EPF demonstrated autoantibodies to the areae compositae of the heart. This finding was associated with left ventricular hypertrophic cardiomyopathy.The areae compositaemay play a role incell junction tension and the El Bagre-EPF patients’ autoantibodies possibly disrupting these junctions and thereby contributing to the left ventricular hypertrophy.

scholarly journals Autoantibodies in Senear-Usher Syndrome: Cross-Reactivity or Multiple Autoimmunity?

2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
María Elena Pérez-Pérez ◽  
Esperanza Avalos-Díaz ◽  
Rafael Herrera-Esparza
Keyword(s):  
Lupus Erythematosus ◽  
Usher Syndrome ◽  
Cross Reactivity ◽  
Pemphigus Foliaceus ◽  
Double Stranded Dna ◽  
Cell Clones ◽  
Single Antigen ◽  
Skin Biopsies ◽  
Serological Data ◽  
Pemphigus Erythematosus

Senear-Usher syndrome or pemphigus erythematosus is a pathology that overlaps clinically and serologically with pemphigus foliaceus and lupus erythematosus. Skin biopsies of patients with pemphigus erythematosus reveal acantholysis and deposits of immunoglobulins in desmosomes, and they are positive in the lupus band test. In the present paper, we determined whether the autoantibodies associated with pemphigus erythematosus targeted a single antigen or multiple antigens as a result of the stimulation of independent B cell clones. Our present paper demonstrates that patients with pemphigus erythematosus produce both antiepithelial antibodies specific for desmoglein 1 and 3 and antinuclear antibodies specific for Ro, La, Sm, and double-stranded DNA antigens. After eluting specific anti-epithelial or anti-nuclear antibodies, which were recovered and tested using double-fluorescence assays, a lack of cross-reactivity was demonstrated between desmosomes and nuclear and cytoplasmic lupus antigens. This result suggests that autoantibodies in pemphigus erythematosus are directed against different antigens and that these autoantibodies are produced by independent clones. Given these clinical and serological data, we suggest that pemphigus erythematosus behaves as a multiple autoimmune disease.

scholarly journals Prevalence of pemphigus incidence in the Bhuj, Kutch, Gujarat: a cross-sectional study

2017 ◽  
Vol 3 (4) ◽  
pp. 478 ◽  
Author(s):  
Devendra Parmar ◽  
Keyur J. Patel
Keyword(s):  
Total Population ◽  
Geographic Area ◽  
Cross Sectional Study ◽  
Incidence Rates ◽  
Original Research ◽  
Pemphigus Foliaceus ◽  
Inclusion Criteria ◽  
Cross Sectional ◽  
Mucous Membranes ◽  
One Year

<p class="abstract"><strong>Background:</strong> <span lang="EN-IN">Pemphigus is a group of rare skin disorders that cause blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals. In Brazil, the prevalence of the disorder is 3.4% in regions such as the Amerindian reservation of Limão Verde and approximately 15,000 patients are known to have pemphigus foliaceus. This prompted us to conduct a study to estimate the incidence of pemphigus in the central Bhuj district of the state of Gujarat in India.</span></p><p class="abstract"><strong>Methods:</strong> <span lang="EN-IN">The present study was planned and conducted between January 2016 to December 2016. The entire team of dermatologists licensed in the district were included in the study and full efforts were made to reach out to them. All the participating dermatologists were asked to fill up a simple perform prepared by the experts in the field. The Performa included the details like date, investigations required for pemphigus, diagnosis based for pemphigus</span>.<strong></strong></p><p class="abstract"><strong>Results:</strong> <span lang="EN-IN">The total population of Bhuj district is estimated to be 5 millions. At the end of the study period that is one year, all the collected performa from the licensed dermatologist were evaluated and results were prepared accordingly. A total of 24 patients who fulfilled all the inclusion criteria were included in the study. </span></p><p class="abstract"><strong>Conclusions:</strong> <span lang="EN-IN">This original research proves that the incidence of pemphigus in Bhuj district was 7.2 per million in the period of one year. This incidence is much higher than the incidence rates reported from the Germany where only 14 cases were reported over a period of 8 years. This study does give us a preliminary idea about the incidence of this rare disease in this geographic area.</span></p>

Pemphigus foliaceus in two pet Nigerian Dwarf goats

2020 ◽  
Vol 8 (3) ◽  
pp. e001162
Author(s):  
Michelle Lauren Piccione ◽  
Elizabeth A Layne ◽  
Gillian C Shaw
Keyword(s):  
Clinical Remission ◽  
Sodium Phosphate ◽  
Clinical Signs ◽  
Pemphigus Foliaceus ◽  
Caprine Arthritis Encephalitis Virus ◽  
Bacterial Cultures ◽  
History Of ◽  
One Year ◽  
Antiparasitic Treatment ◽  
Dwarf Goats

Two male Nigerian Dwarf pet goats from a group of three living in rural southern Wisconsin, USA, presented at the same time with an eight-month history of pruritus and marked crusting on head, axillae and inguinal regions. Parasite investigations were negative, and there was no response to antiparasitic treatment. Fungal and bacterial cultures did not reveal a cause. Histopathology was consistent with a diagnosis of pemphigus foliaceus, and goats were treated with immunosuppressive doses of injectable dexamethasone sodium phosphate. After six weeks of treatment, clinical remission was achieved, and immunosuppressive therapy was continued. Three months later, goat 2 developed presumptive viral pneumonia; necropsy reported presumptive caprine arthritis encephalitis virus. One year into treatment, goat 1 developed similar clinical signs and was euthanased. In goats, pemphigus foliaceus is rare and can be treated using glucocorticoids, but as in other species, chronic immunosuppression increases susceptibility to infections.

scholarly journals Post biologic scenario in pemphigus patients at a tertiary care institution

2019 ◽  
Vol 6 (1) ◽  
pp. 104
Author(s):  
Ramesh A. ◽  
Sampath V. ◽  
Shabari A.
Keyword(s):  
Complete Remission ◽  
Immunosuppressive Agents ◽  
Tertiary Care ◽  
Treatment Protocol ◽  
Oral Prednisolone ◽  
Pemphigus Vulgaris ◽  
Infectious Complications ◽  
Pemphigus Foliaceus ◽  
Long Duration ◽  
One Year

<p class="abstract"><strong>Background:</strong> Rituximab is increasingly used for the treatment of pemphigus. Data derived from single center studies following a uniform treatment protocol are limited. The effect of demography and disease type on treatment response is poorly characterized. Aim of this study was to assess the efficacy, adverse effects of rituximab, adjuvants and follow up in pemphigus patients.</p><p class="abstract"><strong>Methods:</strong> Author undertook a retrospective review of records of 26 pemphigus patients (pemphigus vulgaris 25 and pemphigus foliaceus 1) who had received rituximab infusion. Oral prednisolone was administered in doses up to 0.5 mg/kg of body weight after infusion and tapered over the next 3-4 months according to the disease activity. However, other immunosuppressive agents such as cyclophosphamide and AZT were continued for one year after clinical remission was achieved.<strong></strong></p><p class="abstract"><strong>Results:</strong> Complete remission was observed in 23 (88.5%) patients. The mean time to disease control and complete remission was 1.10 and 4.36 months, respectively. Three patients experienced relapse after a mean duration of 26 months. Infectious complications like candidiasis and furunculosis developed in two patients. Two patients had hypotension during infusion.</p><p class="abstract"><strong>Conclusions:</strong> Rituximab is an effective agent in the treatment of pemphigus and also for a long duration of remission with a lower initial dose of oral prednisolone. Severe side effects were rare.</p>

Between ‘Savage Man’ and ‘Most Faithful Englishman’ Manteo and the Early Anglo-Indian Exchange, 1584–1590

Itinerario ◽  
2000 ◽  
Vol 24 (2) ◽  
pp. 146-169 ◽  
Author(s):  
Michael Leroy Oberg
Keyword(s):  
North Carolina ◽  
Chesapeake Bay ◽  
One Year ◽  
Anglo Indian

In August of 1587 Manteo, an Indian from Croatoan Island, joined a group of English settlers in an attack on the native village of Dasemunkepeuc, located on the coast of present-day North Carolina. These colonists, amongst whom Manteo lived, had landed on Roanoke Island less than a month before, dumped there by a pilot more interested in hunting Spanish prize ships than in carrying colonists to their intended place of settlement along the Chesapeake Bay. The colonists had hoped to re-establish peaceful relations with area natives, and for that reason they relied upon Manteo to act as an interpreter, broker, and intercultural diplomat. The legacy of Anglo-Indian bitterness remaining from Ralph Lane's military settlement, however, which had hastily abandoned the island one year before, was too great for Manteo to overcome. The settlers found themselves that summer in the midst of hostile Indians.

Some Biological Applications of High Voltage Electron Microscopy

1974 ◽  
Vol 32 ◽  
pp. 298-299
Author(s):  
Hans Ris
Keyword(s):  
High Voltage ◽  
Chromosome Structure ◽  
Nerve Fibers ◽  
Good Resolution ◽  
High Voltage Electron Microscopy ◽  
Voltage Electron ◽  
University Of Wisconsin ◽  
High Voltage Electron ◽  
One Year ◽  
The University

The High Voltage Electron Microscope Laboratory at the University of Wisconsin has been in operation a little over one year. I would like to give a progress report about our experience with this new technique. The achievement of good resolution with thick specimens has been mainly exploited so far. A cold stage which will allow us to look at frozen specimens and a hydration stage are now being installed in our microscope. This will soon make it possible to study undehydrated specimens, a particularly exciting application of the high voltage microscope.Some of the problems studied at the Madison facility are: Structure of kinetoplast and flagella in trypanosomes (J. Paulin, U. of Georgia); growth cones of nerve fibers (R. Hannah, U. of Georgia Medical School); spiny dendrites in cerebellum of mouse (Scott and Guillery, Anatomy, U. of Wis.); spindle of baker's yeast (Joan Peterson, Madison) spindle of Haemanthus (A. Bajer, U. of Oregon, Eugene) chromosome structure (Hans Ris, U. of Wisconsin, Madison). Dr. Paulin and Dr. Hanna are reporting their work separately at this meeting and I shall therefore not discuss it here.

Osseointegration interface of calcified bone and endosseous implants

1992 ◽  
Vol 50 (2) ◽  
pp. 1096-1097
Author(s):  
K.E. Krizan ◽  
J.E. Laffoon ◽  
M.J. Buckley
Keyword(s):  
Structure And Function ◽  
Titanium Implants ◽  
En Bloc ◽  
Endosseous Implants ◽  
Ultrastructural Studies ◽  
The Past ◽  
Cacodylate Buffer ◽  
One Year ◽  
And Function

With increase use of tissue-integrated prostheses in recent years it is a goal to understand what is happening at the interface between haversion bone and bulk metal. This study uses electron microscopy (EM) techniques to establish parameters for osseointegration (structure and function between bone and nonload-carrying implants) in an animal model. In the past the interface has been evaluated extensively with light microscopy methods. Today researchers are using the EM for ultrastructural studies of the bone tissue and implant responses to an in vivo environment. Under general anesthesia nine adult mongrel dogs received three Brånemark (Nobelpharma) 3.75 × 7 mm titanium implants surgical placed in their left zygomatic arch. After a one year healing period the animals were injected with a routine bone marker (oxytetracycline), euthanized and perfused via aortic cannulation with 3% glutaraldehyde in 0.1M cacodylate buffer pH 7.2. Implants were retrieved en bloc, harvest radiographs made (Fig. 1), and routinely embedded in plastic. Tissue and implants were cut into 300 micron thick wafers, longitudinally to the implant with an Isomet saw and diamond wafering blade [Beuhler] until the center of the implant was reached.

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