x-linked cutaneous amyloidosis: Topics by WorldWideScience.org

Cutaneous Scarring: A Clinical Review

β₂-microglobulin (β₂m) amyloid deposits are linked to dialysis related amyloidosis (DRA) in hemodialysis patients. The mechanism by which β₂m...Full Text Available

2009-10-21

X-linked nephrogenic diabetes insipidus mutations in North America and the Hopewell hypothesis.

Cutaneous scarring can cause patients symptoms ranging from the psychological to physical pain. Although the process of normal scarring is well described the ultimate cause of pathological scarring...Full Text Available

2009-01-01

RISK-XLR: A Microcomputer-Based Genetic Risk Program for X-Linked Recessive Traits

In X-linked nephrogenic diabetes insipidus (NDI) the urine of male patients is not concentrated after the administration of the antidiuretic hormone arginine-vasopressin. This disease is due to mutations...Full Text Available

1993-09-01

Therapeutic effects of topical application of ozone on acute cutaneous wound healing.

A computer program, RISK-XLR, which calculates genetic risk for carrier status of a Mendelian X-linked recessive condition has been written for the Macintosh series of microcomputers. The program,...Full Text Available

1987-11-04

KoreaScience (Korea)

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2009-06-01

Use of oral miltefosine for cutaneous leishmaniasis in Canadian soldiers returning from Afghanistan

Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.

Old world cutaneous leishmaniasis (CL) is caused by Leishmania major and Leishmania tropica, and is endemic to several Asian and Middle-Eastern countries where the...Full Text Available

2008-11-01

Effects of Human Adipose-derived Stem Cells on Cutaneous Wound Healing in Nude Mice

PURPOSE: To determine the frequency of ophthalmic abnormalities in patients with cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome) and T-cell lymphoma involving the skin and...Full Text Available

1998-01-01

Bacterial infections in suspected cutaneous leishmaniasis lesions.

BackgroundDespite numerous treatments available for deteriorated cutaneous wound healing such as a diabetic foot, there is still the need for more effective therapy. Adipose-derived...Full Text Available

2011-05-01

Vascular Effects of Photodynamic and Pulsed Dye Laser Therapy Protocols

In Iran, microscopic examination of skin scrapings from 2202 individuals with clinically diagnosed cutaneous leishmaniasis (CL) lesions revealed the presence of amastigotes in 1123 cases (51.0%). Bacteriological...Full Text Available

1990-01-01

Thermal neutron capture therapy for human cutaneous malignant melanoma at Kyoto university reactor. {sup 10}B{sub 1}-BPA administration and radiation dose determination

Background and ObjectivePulsed dye laser (PDL) treatment of cutaneous vascular lesions is associated with variable and unpredictable efficacy. Thus, alternative treatment...Full Text Available

2008-11-01

Scanning Electron Microscopy of Sarcophagid (Diptera) Larvae Recovered from a Case of Human Cutaneous Myiasis

Short communication.

1994-06-01

... point dried and mounted on aluminum stubs using colloidal silver paste. Mounted specimens were observed and photographed using ... ...

Amyloidosis

The red-green visual pigment gene region in adrenoleukodystrophy.

The subjects covered in this Symposium range through almost every clinical medical specialty. From an average of one paper in each of the past three Symposiums, the explosive interest in cerebral amyloidosis has led to the presentation of 12 papers on this subject in the present volume. The genetically predisposed familial amyloidotic processes, such as the polyneuropathies and familial Mediterranean fever have also stimulated extensive and intriguing investigations which have revealed the striking effect of a single amino acid substitution in transforming a normal protein into a lethal ''amyloidogenic'' one. This Symposium clearly depicts the advances since the first amyloid fibril protein was definitively identified and defined 14 years ago. Since all amyloid fibril proteins so far described are variants of normal proteins, attention to gene abnormalities now becomes a significant focus as well as the pathogenic sequences which lead in these cases to twisted ...

1984-11-09

Ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome

Although recent data established that a specific very-long-chain fatty acyl-CoA synthetase is defective in X-linked adrenoleukodystrophy (ALD), the ALD gene is still unidentified. The ALD locus has...Full Text Available

1990-03-01

Radionuclide demonstration of ventriculoatrial shunt-cutaneous fistula with cerebrospinal fluid leak

The IFAP syndrome is a rare X-linked genetic disorder reported in nearly 40 patients. It is characterized by the triad of Ichthyosis Follicularis, Alopecia, and Photophobia from birth. Other features...Full Text Available

Cutaneous manifestations in brucellosis.

Radionuclide techniques have commonly been used to assess ventriculovenous shunt function, cerebrospinal fluid dynamics, and for the diagnosis of cerebrospinal fluid leaks. All of these techniques were utilized in this unusual case of retrograde migration of a ventriculoatrial catheter into the neck, with cutaneous fistulization and CSF leak. (orig.).

Clinical and laboratory experience of vorinostat (suberoylanilide hydroxamic acid) in the treatment of cutaneous T-cell lymphoma

Brucellosis is a common worldwide zoonotic disease. Cutaneous manifestations are not specific and affect 1-14% of patients with brucellosis. Here, we describe 49-year-old female with fever and a diffuse maculopapular rash due to Brucella melitensis infection. Histopathology of skin biopsy revealed leukocytoclastic vasculitis; positive blood cultures for B. melitensis established the diagnosis of brucellosis. We provide a review of the relevant literature. PMID:21772606

2011-05-01

Total skin electron beam therapy for cutaneous lymphomas and leukemias

The most common cutaneous T-cell lymphomas (CTCLs) – mycosis fungoides (MF) and Sézary Syndrome – are characterised by the presence of clonally expanded, skin-homing helper-memory...Full Text Available

2006-12-01

Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings

Total skin electron beam therapy (TSEB) was used in the treatment of 33 patients with lymphoma and 13 patients with leukemia involving extensive segments of the skin surface. Twenty-two of 23 had lesions as a primary manifestation of lymphoma (primary cutaneous lymphoma-PCL) and 11 developed cutaneous lesions following disseminated nodal lymphoma (secondary cutaneous lymphoma-SCL). A once weekly fractionation scheme was employed to irradiate the entire skin surface with 3.5 to 4 MeV electron beam from a 6 MeV linear accelerator. During each weekly session, 400 rad were delivered to the entire skin and a complete course consisted of 4-6 consecutive weekly sessions. The majority of patients have been previously treated elsewhere for various periods and all patients have been at risk for a median of 12 months, range from 12-117 months following TSEB. Striking predominance of the diffuse pattern (76%) was demonstrated in both ...

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Amyloid oligomers: spectroscopic characterization of amyloidogenic protein states

Extraskeletal Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are widely regarded as clinically and histologically identical tumors which consist of small blue round cells. Extraskeletal ESs/PNETs usually occur in the deep soft tissues of the paraspinal region, chest wall, or lower extremities. However, superficially located cases, so-called cutaneous ESs/PNETs, are exceedingly rare, and the vast majority of the reported cases present as a single small mass. We present magnetic resonance imaging (MRI) findings and clinical course of a unique case of primary cutaneous ES/PNET presenting as numerous huge masses with severe ulceration on them. (orig.)

2010-06-01

British Library Electronic Table of Contents (United Kingdom)

It is assumed that protein fibrils manifested in amyloidosis result from an aggregation reaction involving small misfolded protein sequences being in an `oligomeric' or `prefibrillar' state. This review covers recent optical spectroscopic studies of amyloid protein misfolding, oligomerization and amyloid fibril growth. Although amyloid fibrils have been studied using established protein-characterization techniques throughout the years, their oligomeric precursor states require sensitive detection in real-time. Here, fluorescent staining is commonly performed using thioflavin T and other small fluorescent molecules such as 4-(dicyanovinyl)- julolidine and 1-amino-8-naphtalene sulphonate that have high affinity to hydrophobic patches. Thus, populated oligomeric intermediates and related `pre...

2010-01-01

The treatment of mycosis fungoides and related conditions with particular emphasis on electron therapy.

Scalp Angiosarcoma Remission with Bevacizumab and Radiotherapy without Surgery: A Case Report and Review of the Literature

The difficulties created by the lack of generally accepted views on classification, diagnostic criteria and natural history of the cutaneous lymphomata are discussed. Only the lymphomata which remain...Full Text Available

1975-03-01

New common variants affecting susceptibility to basal cell carcinoma

Angiosarcoma (AS) is a rare and aggressive vascular neoplasm with very poor prognosis. Patients with extensive cutaneous AS who are not surgical candidates have very limited options since there is no...Full Text Available

2011-01-01

Multicentre, randomised, controlled study of the impact of continuous sub-cutaneous glucose monitoring (GlucoDay) on glycaemic control in type 1 and type 2 diabetes patients

In a follow-up to our previously reported genome-wide association study of cutaneous basal cell carcinoma (BCC)1, we describe here several new susceptibility...Full Text Available

2009-08-01

British Library Electronic Table of Contents (United Kingdom)

ResumeObjectif determiner dans une etude randomisee l'impact d'un enregistrement continu du glucose sous-cutane pendant 48 heures sur le controle glycemique trois mois plus tard chez des patients presentant un diabete de type 1 (DT1) ou de type 2 (DT2). Methodes Quarante-huit patients avec un mauvais equilibre glycemique (HbA1c : 8-10,5 %) ont beneficie d'un enregistrement continu du glucose sous-cutane pendant 48 heures avec le systeme GlucoDay (A. Menarini Diagnostics) et ont ete randomises en deux groupes : ajustement de traitement en fonction de l'autosurveillance glycemique capillaire (groupe ASG) ou du profil d'enregistrement continu du glucose sous-cutane (groupe CGM). Le dosage d'HbA1c et le port du GlucoDay ont ete renouveles trois mois plus tard. Resultats Trente-quatre patients ...

2009-01-01

Endocrine tumours in neurofibromatosis type 1, tuberous sclerosis and related syndromes

Effects of 3 biologic dressings on healing of cutaneous wounds on the limbs of horses

Neurofibromatosis type 1 (NF-1) and tuberous sclerosis complex (TSC) are two familial syndromes known as phakomatoses that may be associated with endocrine tumors. These hereditary cutaneous...Full Text Available

2010-06-01

Distribution Frequency of Pathogenic Bacteria Isolated from Cutaneus Leishmaniasis Lesions

AbstractThree biologic dressings [split-thickness allogeneic skin (STS)], allogeneic peritoneum (P), and xenogenic porcine small intestinal submucosa (PSIS)] were...Full Text Available

2004-01-01

Cutaneous expression of Thy-1 in mycosis fungoides.

Cutaneous leishmaniasis (CL) is a parasitic disease characterized by single or multiple ulcerations. Secondary bacterial infections are one of the complications that can increase the tissue destruction...Full Text Available

2008-09-01

Cutaneous leishmaniasis associated with jungle training.

Dermal dendritic cells from eleven cases of mycosis fungoides (MF) (six patch and five plaque stage), two cases of pre-MF, and five specimens of normal human skin, were characterized immunohistochemically...Full Text Available

1992-12-01

Cutaneous gallium uptake in patients with AIDS with mycobacterium avium-intracellulare septicemia

In November 1977, 627 soldiers belonging primarily to the First Battalion, 82nd Airborne Division, stationed at Fort Bragg, were sent to the Canal Zone, Panama, for jungle warfare training. A medical surveillance program incorporating pre- and post-evaluations over a 6-month period with dermatologic examinations, questionnaires, and serologic tests was established. Ten cases of cutaneous leishmaniasis (1.6/100 men) were diagnosed by positive Leishmania culture. The demonstrated lack of sensitivity and specificity of the indirect fluorescent antibody test and the direct agglutination test render these serological methods useless as diagnostic screening methods in the early stages of this disease. PMID:7406101

1980-07-01

Serum Globotriaosylceramide Assay as a Screening Test for Fabry Disease in Patients with ESRD on Maintenance Dialysis in Korea

Gallium imaging is increasingly being used for the early detection of complications in patients with AIDS. A 26-year-old homosexual man who was HIV antibody positive underwent gallium imaging for investigation of possible Pneumocystis carinii pneumonia. Widespread cutaneous focal uptake was seen, which was subsequently shown to be due to mycobacterium avium-intracellulare (MAI) septicemia. This case demonstrates the importance of whole body imaging rather than imaging target areas only, the utility of gallium imaging in aiding the early detection of clinically unsuspected disease, and shows a new pattern of gallium uptake in disseminated MAI infection.

Disruption of the ATP-binding Cassette B7 (ABTM-1/ABCB7) Induces Oxidative Stress and Premature Cell Death in Caenorhabditis elegans*

Background/AimsFabry disease is an X-linked recessive and progressive disease caused by α-galactosidase A (α-GaL A) deficiency. We sought to assess the prevalence...Full Text Available

2010-12-01

Comparative analysis of the ATRX promoter and 5' regulatory region reveals conserved regulatory elements which are linked to roles in neurodevelopment, alpha-globin regulation and testicular function

X-linked sideroblastic anemia with ataxia (XLSA/A) is a rare inherited disorder characterized by mild anemia and ataxia. XLSA/A is caused by mutations in the ABCB7 gene, which encodes...Full Text Available

2011-06-17

Vitiligo during Treatment of Crohn's Disease with Adalimumab: Adverse Effect or Co-Occurrence?

BackgroundATRX is a tightly-regulated multifunctional protein with crucial roles in mammalian development. Mutations in the ATRX gene cause ATR-X syndrome, an X-linked...Full Text Available

British Library Electronic Table of Contents (United Kingdom)

Abstract Adalimumab is a fully human monoclonal anti-tumor necrosis factor-a agent that is approved for the treatment of Crohn's disease. It has a good safety profile, injection site reactions being the most common adverse effect. We report a case of a 54-year-old woman with a 30-year history of Crohn's disease who developed achromic patches on the trunk and upper extremities after initiating treatment with adalimumab. Cutaneous biopsy confirmed diagnosis of vitiligo and laboratory testing ruled out thyroid disease. Concomitant occurrence of vitiligo and inflammatory bowel disease, although rare, has been described. A common autoimmune basis could explain this fact. Moreover, multiple cutaneous adverse effects have been described in the literature secondary to biologic treatments, includin...

2011-01-01

Relationships of the Lateral Femoral Cutaneous Nerve to Bony Landmarks

British Library Electronic Table of Contents (United Kingdom)

Background The lateral femoral cutaneous nerve (LFCN) can be at risk during, for example, the insertion of pins in the anterior superior iliac spine (ASIS) during external fixation of the pelvis, total hip arthroplasty through a direct anterior approach, open surgery for impingement in the hip through an anterior approach, and periacetabular osteotomy. During surgery, the surgeon usually assumes the location of the LFCN by using the ASIS as a landmark. Questions/purposes We investigated (1) whether there is any relationship between the LFCN and the ASIS and (2) the anatomy of the LFCN at the lateral border of the psoas major. Methods Using 25 formalin-fixed cadavers, we determined the location of the LFCN emergence point as above, same level with, or below the iliac crest (IC). We measured...

2011-01-01

Amicrobial Pustulosis-Like Rash in a Patient with Crohn's Disease under Anti-TNF-Alpha Blocker

British Library Electronic Table of Contents (United Kingdom)

Abstract Amicrobial pustulosis of the folds (APF) is a recently described entity characterized by relapsing pustular lesions predominantly involving the cutaneous flexures and scalp. This disease typically occurs in association with systemic lupus erythematosus and a variety of other autoimmune diseases. We here describe an APF-like pustular eruption predominantly affecting the scalp, face and trunk, occurring during long-term infliximab treatment for Crohn's disease. Immunohistochemical staining of skin biopsy specimens for myxovirus resistance protein A, a marker for type 1 interferon-inducible proteins, showed increased staining in the epidermis and dermal mononuclear inflammatory infiltrate. Our observation further extends the spectrum of cutaneous adverse reactions potentially related...

2011-01-01

Adverse cutaneous reactions secondary to tyrosine kinase inhibitors including imatinib mesylate, nilotinib, and dasatinib

British Library Electronic Table of Contents (United Kingdom)

Abstract Imatinib mesylate is the first of a novel group of drugs that specifically target protein tyrosine kinases, which are central to the pathogenesis of human cancer. It has been approved for the treatment of chronic myeloid leukemia and gastrointestinal stromal tumor and has been found efficacious in other neoplastic diseases. Nilotinib and dasatinib, a second-generation of tyrosine kinase inhibitors (TKIs), were developed in response to findings of emerging imatinib resistance or intolerance to the drug. Cutaneous reactions are the most common nonhematologic side effect of these drugs, and their management is challenging especially in the absence of alternative anticancer agents. The present review focuses on the clinical characteristics and the hypothesized molecular pathogenesis o...

2011-01-01

Weekly total skin electron beam therapy for mycosis fungoides and other cutaneous lymphomata: further experience.

Extracorporeal Photochemotherapy as a Challenging Treatment for Cutaneous T-Cell Lymphoma, Acute and Chronic Graft-versus-Host Disease, Organ Rejection and T-Lymphocyte-Mediated Autoimmune Diseases

Lymphomatous processes involving the skin can be managed effectively by total skin electron beam irradiation. A technique using weekly doses (400 rad for each of 6 successive weeks) has been employed...Full Text Available

1975-03-01

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Evaluation of T-cell receptor gene rearrangements in patients with recurrent patch/plaque (T2) CTCL (mycosis fungoides).

Summary20 years ago, in 1987, Edelson and co-workers published their first report on the effectiveness of a new procedure, called extracorporeal photochemotherapy (ECP), in patients...Full Text Available

2008-01-01

Cutaneous and subcutaneous Ewing's sarcoma: an indolent disease

Cutaneous T-cell lymphoma is typically a clonal neoplasm of epidermotropic CD4+ T-lymphocytes that includes the entity mycosis fungoides (MF). After identification of patients with recurrent MF treated...Full Text Available

1999-11-01

Total skin electron beam and total nodal irradiation for treatment of patients with cutaneous T-cell lymphoma

Purpose: The occurrence of extraosseous Ewing's sarcoma (ES) in deep soft tissues has been well described, but cases in which this tumor occurs in a primary cutaneous or subcutaneous site have rarely been reported. The superficial variant may be less aggressive than are the more common bony and deep soft tissue counterparts with an apparently favorable outcome. A retrospective review of patients with cutaneous or subcutaneous ES was conducted to analyze outcome and patterns of failure. Methods and Materials: Between July 1985 and March 1997, 14 patients with cutaneous or subcutaneous ES were treated at St. Jude Children's Research Hospital. The median age at presentation was 16 years (range 7-21 years). Anatomic locations included trunk and pelvis (7), upper or lower extremity (4), and head and neck (3). The median size of the lesion was 3 cm (range, 1-12 cm). Thirteen had definitive surgical resections, and one had biopsy ...

2000-01-15

Effect of external pressure on intramuscular blood flow at rest and after running

Sixteen patients with advanced cutaneous T-cell lymphoma (CTCL) with or without lymph node involvement, but without evidence of extranodal manifestations, were treated with a combination of total skin electron beam therapy (TSEB) and total nodal irradiation (TNI). Fourteen (87%) patients achieved a complete response (CR) lasting from 1 to 84+ months (median, 8+ months) from the completion of treatment. The best results occurred in 6 patients with pretumorous intracutaneous CTCL (Stages IB and IIA) where the CR has lasted in all patients from 8 to 84+ months (median about 27+ months). Radiotherapy was well tolerated with the major toxicity being bone marrow suppression. The authors conclude that combined TSEB and TNI is a relatively safe and effective treatment for patients with CTCL prior to the development of lymph node involvement. Long-term follow-up is needed to assess the curative potential of this treatment.

1985-06-01

Mary Lyon and the hypothesis of random X chromosome inactivation

Local blood flow in the thigh was measured with /sup 133/Xe clearance technique in eight male distance runners after compression with a foam rubber compress and a standard elastic bandage. Two degrees of compression were tested, and an initial experiment with rested subjects was followed by a similar experiment immediately after running. Maximum compression exerted a cutaneous pressure of 85 (+/- 8) mm Hg and caused an immediate cessation of intra-muscular blood flow in the compressed area. Moderate compression gave a cutaneous pressure of 40 (+/- 5) mm Hg and resulted in a reduction of blood flow by approximately 50%. During compression, there were no significant differences in the blood flow of rested subjects compared to subjects immediately after running. In acute soft tissue injuries, a maximum compression bandage should effectively reduce or eliminate the formation of an intra-muscular hematoma, and an additive effect on blood flow of ice ...

1987-10-01

British Library Electronic Table of Contents (United Kingdom)

The 50th anniversary of Mary Lyon?s 1961 Nature paper, proposing random inactivation in early embryonic life of one of the two X chromosomes in the cells of mammalian females, provides an opportunity to remember and celebrate the work of those involved. While the hypothesis was initially put forward by Lyon based on findings in the mouse, it was founded on earlier studies, notably the work of Susumu Ohno; it was also suggested independently by Beutler and colleagues using experimental evidence from a human X-linked disorder, glucose-6-phosphate dehydrogenase deficiency, and has proved to be of as great importance for human and medical genetics as it has for general mammalian genetics. Alongside the hypothesis itself, previous cytological studies of mouse and human chromosomes, and the obse...

2011-01-01

The successful treatment of lupus pernio with the flashlamp pulsed dye laser.

Dual-field rotational (DFR) technique for total-skin electron-beam therapy (TSEBT)

Lupus pernio of the nose is the most characteristic cutaneous lesion of sarcoidosis. It is cosmetically disfiguring and can be the cause of significant morbidity. In particular, the affected skin is often red or purple due to increased vasculature. It is particularly resistant to both surgical and medical therapy. We describe a patient with lupus pernio affecting her nose, which showed a dramatic improvement following treatment with the pulse dye laser (PDL). A biopsy taken after treatment showed the continued presence of sarcoidal granulomas. We therefore feel that treatment with the PDL is an effective tool in improving the cosmetic appearance of lupus pernio, but does not influence the underlying disease process. PMID:11360426

1999-01-01

Where the sun does not shine: Is sunshine protective against melanoma of the vulva?

Comparison of dosimetry measured in a noncylindrical Rando-Alderson phantom by two-field, four-field, and six-field total-skin electron-beam therapy (TSEBT) techniques with our dual-field rotational (DFR) technique reveals a superior dosimetry for the latter. Our technique of dual-field rotational DFR-TSEBT is described in detail, and its advantages and indications for the primary management of cutaneous T-cell lymphoma (CTCL) and Kaposi's sarcoma (KS) are discussed.

1987-08-01

British Library Electronic Table of Contents (United Kingdom)

Intermittent sun exposure and sunburn are strong predictors of cutaneous malignant melanoma (CMM). On the other hand, melanomas may arise also in non-sun-exposed areas such as the vulva. However, little is known about a possible relationship between sun exposure and vulvar melanoma. Temporal and latitudinal dependencies of the incidence rates of vulvar melanoma were studied in comparison with those of CMM among Caucasians in Sweden, East Germany, USA and Victoria (Australia). The ratios of vulvar melanoma incidence rates to those of CMM tend to decrease with increasing CMM rates. The incidence rates of CMM have increased with time until recently, while those of vulvar melanoma have either decreased or remain constant. In USA vulvar melanoma incidence rates seem to increase from south to no...

2010-01-01

Total skin electron beam therapy in mycosis fungoides. Evaluation of a technique for deceleration of electron beam energy and clinical study

Total skin electron beam therapy in mycosis fungoides

The studies using phantoms confirmed that the reduction of electron beam energy and minimization of X-ray contamination could be achieved when electron beam was interposed by an acrylic plate placed 20 cm anterior to a patient. Four patients of mycosis fungoides were treated with 8 MeV electron beam of a linear accelerator at UOEH Hospital from October 1981 to December 1986. Two of them were treated with this technique by placing 2 cm thick acrylic plate anterior to the patients and satisfactory results were obtained. Cutaneous lesions subsided remarkably with the dosage of 2000 cGy given in 2 months. Leucopenia due to bone marrow suppression was mild and the patients tolerated the treatment well.

1988-08-01

The dermal leishmaniases of Brazil, with special reference to the eco-epidemiology of the disease in Amazonia

Scientific Electronic Library Online (English)

Abstract in english Six species of Leishmania are at present known to cause cutaneous and/or mucocutaneous leishamniasis in Brazil, and they are all to be found in the Amazon region of this country. The eco-epidemiology of each is discussed, with the observation that the Amazonian leishmaniases are all zoonoses, with their source in silvatic mammals and phlebotomine sandfly vectors. With man's destruction of the natural forest in southern Brazil, some sandfly species have survived by adapti (more) ng to a peridomestic or domiciliary habitat in rural areas. Some domestic animals, such as dogs and equines are seemingly now involved in the epidemiology of the disease. No such process has yet been reported in the Amazon region, but may well take place with the continuing devastation of its forest.

1994-09-01

Pentoxyphylline in association with vitamin E reduces cutaneous fibrosis in systemic sclerosis

British Library Electronic Table of Contents (United Kingdom)

Systemic sclerosis (SSc) is a disorder characterized by skin thickness and vasculopathy. The objective of the study was to evaluate the therapeutic effect and safety of the association of pentoxyphylline and vitamin E in SSc patients. Twelve SSc patients (American College of Rheumatology criteria) enrolled this 24-week open-label study. Patients received daily 800?mg of pentoxyphylline and 800?UI of vitamin E and were evaluated at 4-week interval. The primary efficacy endpoint was the change in Modified Rodnan Skin Score (MRSS) at week 24. Nine diffuse SSc patients treated 6?months with cyclophosphamide were used as a historical control group. The mean age of the treated group was 43.6?years, and ten of 12 (84%) patients were women. Their mean MRSS reduced from 25.7 to 18.7 (p?=?0.03) at 1...

2009-01-01

Evaluation of antiasthmatic activity of Clitoria ternatea L. roots

British Library Electronic Table of Contents (United Kingdom)

Aim of study: Clitoria ternatea L. (Family: Fabaceae) is being used in traditional medicine for the treatment of severe bronchitis and asthma. So the aim of study was to evaluate antiasthmatic activity of ethanol extract of Clitoria ternatea roots. Materials and methods: In the present study ethanol extract of Clitoria ternatea root (ECTR) was evaluated for preliminary phytochemical screening, acute toxicity studies and antiasthmatic activity using milk induced leucocytosis and eosinophilia in mice, egg albumin induced mast cell degranulations in rats and passive cutaneous anaphylaxis in rats at doses (100-150mg/kg ip). Results: The results of present investigation showed that the LD50 of ECTR is more than 1300mg/kg. ECTR significantly decreases milk induced leucocytosis and eosinophilia, ...

2011-01-01

Borrelia Burgdorferi ''sensu lato'' in Brazil: Occurrence confirmed by immunohistochemistry and focus floating microscopy

British Library Electronic Table of Contents (United Kingdom)

In the present study, we report the occurrence of Lyme's borreliosis in patients from the Brazilian Amazon Region. Borreliosis was investigated by immunohistochemistry and focus floating microscopy for Borrelia burgdorferi in skin biopsy samples from 22 patients with both clinical and histopathology evidences compatible with Erythema Migrans. Spirochetes were detected by specific immunohistochemistry and focus floating microscopy for B. burgdorferi in samples from five patients. Clinical cure of the cutaneous lesions was observed in all the patients after treatment with doxycycline regimen as proposed by the Center Disease Control guidelines. A limitation of our study was the fact that we were not able to isolate and culture these organisms. These are the first known Brazilian cases of bor...

2010-01-01

Successful treatment of skin infiltration in childhood hematological malignancies with total skin electron beam therapy. A report of two cases

Two children with cutaneous lymphoma and leukemia who were treated with total skin electron beam therapy (TSEB) are described here. Patient 1. A 7-year-old boy was admitted because of a mass over the bilateral parotis and anemia. The white blood cell count on admission was 5,000/ul. Bone marrow examination revealed 70 per cent monoblasts (M5a type by FAB classification). Complete remission was obtained following the regimen of daunomycin, cytosine arabinoside, 6-mercaptopurine and prednisolone. He was again admitted because of a skin nodule on the left thigh 19 months after initial diagnosis. A biopsy of the skin nodule demonstrated monoblastic infiltration. He received 20 Grays (Gy) to the left thigh and this led to resolution of the skin nodule. At that time, other skin nodules appeared on the right upper and lower extremities. He was treated with TSEB. Daily doses of 1 Gy were given twice a week with a 4 MeV electron beam and a total dose of 10 Gy was ...

1988-07-01

p53 protein in aggressive and non-aggressive basal cell carcinoma.

Glycosaminoglycan synthesis was studied in cell populations of ultraviolet light-induced murine cutaneous fibrosarcoma cells under conditions of varying growth rates in vitro. After labeling with the precursors, /sup 3/H-glucosamine and /sup 35/SO/sub 4/, sulfated glycosaminoglycans recoverable by direct proteolysis of the culture monolayers increased approximately 5-fold on a per cell basis from sparsely populated, exponential cell cultures (greater than 85% of cells in S, G2, or M phases) to stationary cultures inhibited by high cell density (greater than 50% of cells in G1). Within this cell surface-associated material, the relative ratio of heparan sulfate to the chondroitin sulfates was approximately 60/40% under conditions of exponential growth; in the growth-arrested cultures, the reverse ratio was found. The substratum attached material, obtained from the flask surface after ethyl glycol bis(beta-aminoethyl ether)-N,N'-tetraacetic acid ...

1985-08-01

Experimental studies on reconstruction of peripheral arteries by high voltage cathode ray irradiated allografts

Basal cell carcinoma (BCC) is the most frequent cutaneous neoplasm, with a generally favorable clinical behavior. Sometimes, indeed, it recurs after therapy and/or metastasizes. As point mutations in the coding sequence of the p53 tumor suppressor gene have been implicated in the progression of many human tumors, we studied the expression of p53 protein on this neoplasia. We tested immunohistochemically the positivity for p53 protein (NCL-p53-CM1, YLEM) on 19 cases of morphologically "non aggressive" BCC (BCC1) and on 19 "aggressive" BCC (BCC2), all with one or more relapses and 3 with distant metastases also. Results were related to clinico-pathological and follow-up data. All but one BCC2 were found positive for p53 protein. Conversely, only 2 cases of BCC1 exhibited low immunoreactivity for p53 protein, with high statistical differences between the two groups. No correlation was found between the immunoreactivity, age of patients, and site of the lesions. The ...

1993-10-01

Electron beam therapy in squamous and basal cell carcinoma of the face. L'electrontherapie des epitheliomas cutanes de la face

The use of cathode ray irradiated arterial allografts on the reconstruction of small peripheral arteries is described. The fate of irradiated arterial allografts implanted subcutaneously in rats was compared histologically with that of allografts which were fresh, frozen or stored in 70% alcohol. Follow-up studies were made of 10 dogs in which irradiated arterial allografts had been implanted in the femoral artery to be evaluated; long term patency by arteriography, gross and histologic changes of the implanted allografts, and the luminal surface of the implanted allografts by scanning electron microscopic studies. For the purpose of antigenic studies, extracts of canine arteries which had been irradiated, frozen or stored in 70% alcohol, were prepared and tested by the following immunological methods: precipitation, Ouchterlony gel diffusion, and passive cutaneous anaphylaxis. From the results obtained in these experiments, it was concluded that, compared with ...

1976-01-01

An evaluation of the pendulum technique of electron beam therapy

This work is a retrospective study of 86 patients with squamous cell (51) and basal cell (35) carcinoma of the face. The median age was 67 years. The staging is as following: 31 cases T{sub 1} and T{sub 2}, 55 patients T{sub 3} and T{sub 4}. All patients were considered NO. Electron beam was used in all cases and energy ranged from 5 to 15 MeV depending on the thickness of the tumor. The total tumor dose was 60-70 Gy delivered in 6 weeks. In 7 cases of very old patients the authors have used concentrated irradiation: 3 patients received 21 Gy in one fraction and 4 patients 48 Gy in 8 fractions. 96.8% patients T{sub 1} i T{sub 2} are NED contrary to 56.4% T{sub 3} i T{sub 4}. Cosmetic results were considered as good in 52, fairly good in 7 and bad in 2 patients.

1989-01-01

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Aarskog-Scott syndrome: Clinical update and report of nine novel mutations of the FGD1 gene

Electron beam therapy of lesions such as cutaneous metastatic carcinoma and reticulum cell sarcoma is performed via multiple portals for a wide irradiation field. However, the dose distribution in border areas is generally uneven, resulting in hot and cold dose areas depending on the intervals of the portals. We performed electron irradiation by the pendulum technique, especially on curved areas, and evaluated the technique in various respects. Pendulum electron beam irradiation is indicated when the length of the cone must be reduced in order to deliver the target volume near the skin surface as well as to avoid the occurrence of hot spots deep in the body. We prepared a 15cm rotating cone by cutting a 30cm fixed irradiation cone. The modification posed no disadvantages in terms of extent and flatness of the beam, or stability during rotation. After determining the relationship between the rotation speed and the dose, the technique was successfully applied to a ...

DEFF Research Database (Denmark)

Mutations in the FGD1 gene have been shown to cause Aarskog-Scott syndrome (AAS), or facio-digito-genital dysplasia (OMIM#305400), an X-linked disorder characterized by distinctive genital and skeletal developmental abnormalities with a broad spectrum of clinical phenotypes. To date, 20 distinct mutations have been reported, but little phenotypic data are available on patients with molecularly confirmed AAS. In the present study, we report on our experience of screening for mutations in the FGD1 gene in a cohort of 60 European patients with a clinically suspected diagnosis of AAS. We identified nine novel mutations in 11 patients (detection rate of 18.33%), including three missense mutations (p.R402Q; p.S558W; p.K748E), four truncating mutations (p.Y530X; p.R656X; c.806delC; c.1620delC), one in-frame deletion (c.2020_2022delGAG) and the first reported splice site mutation (c.1935+3A>C). A recurrent mutation (p.R656X) was detected in three independent families. ...

2010-01-01

ATRX ADD domain links an atypical histone methylation recognition mechanism to human mental-retardation syndrome

Total skin electron beam therapy followed by adjuvant psoralen/ultraviolet-a light in the management of patients with T1 and T2 cutaneous T-cell lymphoma (mycosis fungoides)

ATR-X (alpha-thalassemia/mental retardation, X-linked) syndrome is a human congenital disorder that causes severe intellectual disabilities. Mutations in the ATRX gene, which encodes an ATP-dependent chromatin-remodeler, are responsible for the syndrome. Approximately 50% of the missense mutations in affected persons are clustered in a cysteine-rich domain termed ADD (ATRX-DNMT3-DNMT3L, ADD{sub ATRX}), whose function has remained elusive. Here we identify ADD{sub ATRX} as a previously unknown histone H3-binding module, whose binding is promoted by lysine 9 trimethylation (H3K9me3) but inhibited by lysine 4 trimethylation (H3K4me3). The cocrystal structure of ADD{sub ATRX} bound to H3{sub 1-15}K9me3 peptide reveals an atypical composite H3K9me3-binding pocket, which is distinct from the conventional trimethyllysine-binding aromatic cage. Notably, H3K9me3-pocket mutants and ATR-X syndrome mutants are defective in both H3K9me3 binding and localization at ...

2011-07-19

Immunoscintigraphy with anti-225.28S for ocular melanoma - a comparison with histology and immunohistochemistry

Purpose: Patients with mycosis fungoides [cutaneous T-cell lymphoma (CTCL)] may benefit from adjuvant therapy after completing total skin electron beam therapy (TSEBT). We report the results for (T1(T2)) CTCL patients treated with adjuvant oral psoralen plus ultraviolet light (PUVA) with respect to overall survival (OS), disease-free survival (DFS), salvage of recurrence, and toxicity. Methods and Materials: Between 1974 and 1993, TSEBT was administered to a total of 213 patients with CTCL. Records were reviewed retrospectively, and a total of 114 patients were identified as having T1 or T2 disease. Radiotherapy was provided via a 6-MeV linac to a total of 36 Gy, 1 Gy/day, 4 days/week, for 9 weeks. Beginning in 1988, patients were offered adjuvant PUVA within 2 months of completing TSEBT. This was started at 0.5-2 J/m"2, 1-2 treatments/week, with a taper over 3-6 months. Therapy then continued once per month. There were 39 T1 and 75 T2 patients. Six T1 (15%) and ...

1997-07-15