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Sample records for staphylococcal scalded-skin syndrome

  1. Staphylococcal scalded skin syndrome in a 4-year-old child: a case report.

    Science.gov (United States)

    Haasnoot, P J; De Vries, A

    2018-01-29

    Staphylococcal scalded skin syndrome is an exfoliating skin disease which primarily affects children. Differential diagnosis includes toxic epidermal necrolysis, staphylococcal scalded skin syndrome, epidermolysis bullosa, and Stevens-Johnson syndrome. Staphylococcal scalded skin syndrome primarily affects children and can cause serious morbidity. In this case report we highlight the case of a 4-year-old Caucasian boy. Diagnostic and therapeutic challenges are discussed. Differential diagnoses are considered and therapy is described and discussed. The latest treatment options are used and described. Successful results are achieved in this case due to timely and correct management. Some therapeutic options are widely used without thorough research bases. This case report highlights staphylococcal scalded skin syndrome and its treatment, and future challenges. Further research is warranted and this case report aims to further research in exfoliating skin disorders.

  2. Congenital staphylococcal scalded skin syndrome in a premature infant

    NARCIS (Netherlands)

    Haveman, LM; Fleer, A; de Vries, LS; Gerards, LJ

    2004-01-01

    A case of congenital staphylococcal scalded skin syndrome (SSSS) with fatal outcome in a premature infant is reported. An intrauterine infection with Staphylococcus aureus was probably the cause for the fulminant course of the disease. Despite adequate antibiotic treatment, the infant died within 24

  3. Staphylococcal scalded skin syndrome in a premature newborn caused by methicillin-resistant Staphylococcus aureus: case report

    Directory of Open Access Journals (Sweden)

    Andreas Hörner

    Full Text Available CONTEXT: Staphylococcal scalded skin syndrome is an exfoliative skin disease. Reports of this syndrome in newborns caused by methicillin-resistant Staphylococcus aureus are rare but, when present, rapid diagnosis and treatment is required in order to decrease morbidity and mortality. CASE REPORT: A premature newly born girl weighing 1,520 g, born with a gestational age of 29 weeks and 4 days, developed staphylococcal scalded skin syndrome on the fifth day of life. Cultures on blood samples collected on the first and fourth days were negative, but Pseudomonas aeruginosa and Enterococcus sp. (vancomycin-sensitive developed in blood cultures performed on the day of death (seventh day, and Pseudomonas aeruginosa and Serratia marcescens were identified in cultures on nasopharyngeal, buttock and abdominal secretions. In addition to these two Gram-negative bacilli, methicillin-resistant Staphylococcus aureus was isolated in a culture on the umbilical stump (seventh day. The diagnosis of staphylococcal scalded skin syndrome was based on clinical criteria.

  4. Varicella infection in a neonate with subsequent staphylococcal scalded skin syndrome and fatal shock.

    Science.gov (United States)

    Singh, Shakal Narayan; Tahazzul, Mohammad; Singh, Anita; Chandra, Surabhi

    2012-08-01

    A male term neonate, at day 23 of life, presented with vesicular lesions over the trunk, which spread to allover the body on the next day. Five days later, he started developing blistering of the skin over the trunk and extremities, which subsequently ruptured, leaving erythematous, tender raw areas with peeling of the skin. The mother had vesicular eruptions, which started on the second day of delivery and progressed over the next 3 days. Subsequently, similar eruptions were noticed in two of the siblings before affecting the neonate. On the basis of the exposure history and clinical picture, a diagnosis was made of varicella infection with staphylococcal scalded skin syndrome (SSSS). The blood culture and the wound surface culture grew Staphylococcus aureus. Treatment included intravenous fluid, antibiotics, acyclovir and wound care. However, after 72 h of hospitalisation, the neonate first developed shock, refractory to fluid boluses, vasopressors and catecholamine along with other supports; and he then succumbed. In all neonates, staphylococcal infection with varicella can be fatal due to SSSS, the toxic shock syndrome or septicaemia.

  5. Neonatal staphylococcal scalded skin syndrome: clinical and outbreak containment review.

    LENUS (Irish Health Repository)

    Neylon, Orla

    2012-01-31

    Staphylococcal scalded skin syndrome (SSSS) is a toxin-mediated exfoliating skin condition predominated by desquamation and blistering. Neonatal outbreaks have already been reported; however, our outbreak highlights the potential for SSSS following neonatal health promotion measures such as intra-muscular vitamin K administration and metabolic screening (heel prick) as well as effective case containment measures and the value of staff screening. Between February and June 2007, five confirmed cases of neonatal SSSS were identified in full-term neonates born in an Irish regional maternity hospital. All infants were treated successfully. Analysis of contact and environmental screening was undertaken, including family members and healthcare workers. Molecular typing on isolates was carried out. An outbreak control team (OCT) was assembled and took successful prospective steps to prevent further cases. All five Staphylococcus aureus isolates tested positive for exfoliative toxin A, of which two distinct strains were identified on pulsed-field gel electrophoresis analysis. Two cases followed staphylococcal inoculation during preventive measures such as intra-muscular vitamin K administration and metabolic screening (heel prick). None of the neonatal isolates were methicillin resistant. Of 259 hospital staff (70% of staff) screened, 30% were colonised with S. aureus, and 6% were positive for MRSA carriage. This is the first reported outbreak of neonatal SSSS in Ireland. Effective case containment measures and clinical value of OCT is demonstrated. Results of staff screening underlines the need for vigilance and compliance in hand disinfection strategies in maternity hospitals especially during neonatal screening and preventive procedures.

  6. Regional outbreak of staphylococcal scalded skin syndrome in healthy children

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    Hyun Jeong Do

    2010-01-01

    Full Text Available Purpose : Staphylococcal scalded skin syndrome (SSSS is a relatively uncommon superficial blistering skin disease that is due to Staphylococcus aureus. We had experienced a regional outbreak of SSSS over 3 years in healthy children. Methods : We retrospectively reviewed the medical records of those patients diagnosed as SSSS. Most of neonatal cases were nosocomial infections and excluded from the analysis. The clinical features, laboratory findings, the isolation and antibiotic resistance of S. aureus, the antibiotic management and other supportive treatments were analyzed. Results : Fifty-five patients with SSSS were admitted to our hospital from October 2001 to September 2004. The median age of patients was 3.0 years. Of the 55 patients, 9 were the generalized type, 13 were the intermediate type and 33 were the scarletiniform rash. All the patients were living in neighborhood of the Jinju area. S. aureus were isolated from 9 of the patients and all of the isolated S. aureus were methicillin resistant. All the patients except two were treated with intravenous flocloxacillin or nafcillin and/or cefotaxime. All the patients recovered during the follow-up period of 2 to 3 weeks. Conclusion : We experienced a regional outbreak of SSSS in previous healthy children. Further study for finding the carriers of S. aureus caused SSSS and preventing the spread of this disease is needed. Additionally, guidelines for treating SSSS due to methicillin resistant S. aureus should be established.

  7. Rapid containment of nosocomial transmission of a rare community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) clone, responsible for the Staphylococcal Scalded Skin Syndrome (SSSS)

    OpenAIRE

    Lamanna, Onofrio; Bongiorno, Dafne; Bertoncello, Lisa; Grandesso, Stefano; Mazzucato, Sandra; Pozzan, Giovanni Battista; Cutrone, Mario; Chirico, Michela; Baesso, Flavia; Brugnaro, Pierluigi; Cafiso, Viviana; Stefani, Stefania; Campanile, Floriana

    2017-01-01

    Background The aims of this study were to identify the source and the transmission pathway for a Staphylococcal Scalded Skin Syndrome (SSSS) outbreak in a maternity setting in Italy over 2?months, during 2014; to implement appropriate control measures in order to prevent the epidemic spread within the maternity ward; and to identify the Methicillin-Resistant Staphylococcus aureus (MRSA) epidemic clone. Methods Epidemiological and microbiological investigations, based on phenotyping and genoty...

  8. Nosocomial outbreak of staphylococcal scalded skin syndrome in neonates: epidemiological investigation and control.

    Science.gov (United States)

    El Helali, N; Carbonne, A; Naas, T; Kerneis, S; Fresco, O; Giovangrandi, Y; Fortineau, N; Nordmann, P; Astagneau, P

    2005-10-01

    Over a three-month period, 13 neonates developed staphylococcal scalded skin syndrome (SSSS) in a maternity unit, between four and 18 days after their birth. An epidemiological and descriptive study followed by a case-control study was performed. A case was defined as a neonate with blistering or peeling skin, and exfoliative toxin A Staphylococcus aureus positive cultures. Controls were selected at random from the asymptomatic, non-colonized neonates born on the same day as the cases. All staff members and all neonates born during the outbreak period were screened for carriage by nasal swabs and umbilical swabs, respectively. S. aureus isolates were polymerase chain reaction (PCR) screened for etA gene and genotyped by pulsed-field gel electrophoresis (PFGE). Two clusters of eight and five cases were identified. Receiving more than one early umbilical care procedure by the same ancillary nurse was the only risk factor identified in the case-control study (odds ratio=15, 95% confidence intervals 2-328). The ancillary nurse suffered from chronic dermatitis on her hands that favoured S. aureus carriage. Exfoliative-toxin-A-producing strains, as evidenced by PCR and indistinguishable by PFGE, were isolated from all but one of the SSSS cases, from four asymptomatic neonates, from two staff members and from the ancillary nurse's hands. Removal of the ancillary nurse from duty, infection control measures (isolation precautions, chlorhexidine handwashing and barrier protections), and treatment of the carriers (nasal mupirocin and chlorhexidine showers) led to control of the epidemic. In conclusion, this study emphasizes the need for tight surveillance of chronic dermatitis in healthcare workers.

  9. Nosocomial outbreak of staphyloccocal scalded skin syndrome in neonates in England, December 2012 to March 2013.

    Science.gov (United States)

    Paranthaman, K; Bentley, A; Milne, L M; Kearns, A; Loader, S; Thomas, A; Thompson, F; Logan, M; Newitt, S; Puleston, R

    2014-08-21

    Staphylococcal scalded skin syndrome (SSSS) is a blistering skin condition caused by exfoliative toxin-producing strains of Staphylococcus aureus. Outbreaks of SSSS in maternity settings are rarely reported. We describe an outbreak of SSSS that occurred among neonates born at a maternity unit in England during December 2012 to March 2013. Detailed epidemiological and microbiological investigations were undertaken. Eight neonates were found to be infected with the outbreak strain of S. aureus, of spa type t346, representing a single pulsotype. All eight isolates contained genes encoding exfoliative toxin A (eta) and six of them contained genes encoding toxin B (etb). Nasal swabs taken during targeted staff screening yielded a staphylococcal carriage rate of 21% (17/80), but none contained the outbreak strain. Mass screening involving multi-site swabbing and pooled, enrichment culture identified a healthcare worker (HCW) with the outbreak strain. This HCW was known to have a chronic skin condition and their initial nasal screen was negative. The outbreak ended when they were excluded from work. This outbreak highlights the need for implementing robust swabbing and culture methodswhen conventional techniques are unsuccessful in identifying staff carrier(s). This study adds to the growing body of evidence on the role of HCWs in nosocomial transmission of S. aureus.

  10. Antimicrobial Agent of Susceptibilities and Antiseptic Resistance Gene Distribution among Methicillin-Resistant Staphylococcus aureus Isolates from Patients with Impetigo and Staphylococcal Scalded Skin Syndrome

    Science.gov (United States)

    Noguchi, Norihisa; Nakaminami, Hidemasa; Nishijima, Setsuko; Kurokawa, Ichiro; So, Hiromu; Sasatsu, Masanori

    2006-01-01

    The susceptibilities to antimicrobial agents of and distributions of antiseptic resistance genes in methicillin-resistant Staphylococcus aureus (MRSA) strains isolated between 1999 and 2004 in Japan were examined. The data of MRSA strains that are causative agents of impetigo and staphylococcal scalded skin syndrome (SSSS) were compared with those of MRSA strains isolated from patients with other diseases. The susceptibilities to antiseptic agents in MRSA isolates from patients with impetigo and SSSS were higher than those in MRSA isolates from patients with other diseases. The distribution of the qacA/B genes in MRSA strains isolated from patients with impetigo and SSSS (1.3%, 1/76) was remarkably lower than that in MRSA strains isolated from patients with other diseases (45.9%, 95/207). Epidemiologic typings of staphylococcal cassette chromosome mec (SCCmec) and pulsed-field gel electrophoresis (PFGE) showed that MRSA strains isolated from patients with impetigo and SSSS had type IV SCCmec (75/76), except for one strain, and 64.5% (49/76) of the strains had different PFGE types. In addition, the patterns of restriction digestion of all tested qacA/B plasmid in MRSA isolates having different PFGE types were identical. The results showed that a specific MRSA clone carrying qacA/B was not prevalent, but qacA/B was spread among health care-associated MRSA strains. Therefore, it was concluded that the lower distribution rate of qacA/B resulted in higher susceptibilities to cationic antiseptic agents in MRSA isolated from patients with impetigo and SSSS. PMID:16757607

  11. Rapid containment of nosocomial transmission of a rare community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) clone, responsible for the Staphylococcal Scalded Skin Syndrome (SSSS).

    Science.gov (United States)

    Lamanna, Onofrio; Bongiorno, Dafne; Bertoncello, Lisa; Grandesso, Stefano; Mazzucato, Sandra; Pozzan, Giovanni Battista; Cutrone, Mario; Chirico, Michela; Baesso, Flavia; Brugnaro, Pierluigi; Cafiso, Viviana; Stefani, Stefania; Campanile, Floriana

    2017-01-06

    The aims of this study were to identify the source and the transmission pathway for a Staphylococcal Scalded Skin Syndrome (SSSS) outbreak in a maternity setting in Italy over 2 months, during 2014; to implement appropriate control measures in order to prevent the epidemic spread within the maternity ward; and to identify the Methicillin-Resistant Staphylococcus aureus (MRSA) epidemic clone. Epidemiological and microbiological investigations, based on phenotyping and genotyping methods, were performed. All neonates involved in the outbreak underwent clinical and microbiological investigations to detect the cause of illness. Parents and healthcare workers were screened for Staphylococcus aureus to identify asymptomatic carriers. The SSSS outbreak was due to the cross-transmission of a rare clone of ST5-CA-MRSA-SCCmecV-spa type t311, exfoliative toxin A-producer, isolated from three neonates, one mother (from her nose and from dermatological lesions due to pre-existing hand eczema) and from a nurse (colonized in her nose by this microorganism). The epidemiological and microbiological investigation confirmed these as two potential carriers. A rapid containment of these infections was obtained only after implementation of robust swabbing of mothers and healthcare workers. The use of molecular methodologies for typing was able to identify all carriers and to trace the transmission.

  12. Staphylococcal scalded skin syndrome hos voksne

    DEFF Research Database (Denmark)

    Mikkelsen, Carsten Sauer; Mikkelsen, Dorthe Bisgaard; Jensen, Thøger Gorm

    2010-01-01

    parts of her body. The bullae ruptured easily and left a erythematous base. The histopathological changes were characteristic for adult SSSS. The patient was well-treated with intravenous dicloxacillin, topical antibiotic and antiseptic treatment. The patient had marked thrombocytosis, but no interest...

  13. Impetigo asacause of sepsis ininfants

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    Alicja Moes-Wójtowicz

    2015-06-01

    Full Text Available Impetigo is a fairly common disease of the childhood. This infection exists in two distinct forms: bullous and non-bullous impetigo. In non-bullous impetigo, a tiny vesicle or pustule shows initially and after 4–6 days develops into a honey-coloured crusted plaque. Bullous impetigo is always caused by Staphylococcus aureus, a bacterium that produces exfoliative toxins (ETA and ETB. Infection is limited to the superficial layers of the skin, and skin changes typically occur on the face and limbs. The disease is most prevalent in children aged 2 to 5, but it can occur at any age. The treatment involves topical or systemic antibiotics depending on the severity of symptoms and the patient’s condition. The differential diagnosis of impetigo includes: staphylococcal scalded-skin syndrome, Herpes simplex infection, eosinophilic pustular folliculitis, epidermolysis bullosa, pemphigus and pemphigoid. The differentiation between impetigo and staphylococcal scalded-skin syndrome can be challenging. Both diseases are caused by the same strain of Staphylococcus aureus that produces exfoliative toxins. However, in impetigo toxins are produced only within skin lesions whereas in staphylococcal scalded-skin syndrome, toxins are produced in organs colonised by Staphylococcus aureus and then released systemically. In the course of staphylococcal scalded-skin syndrome severe tenderness of the skin and a positive Nikolsky’s sign are observed. The paper presents two cases of impetigo in infants. In both cases, the correct diagnosis was not established immediately, which delayed appropriate treatment. In one of the cases, due to the imprecise initial diagnosis and the lack of the holistic approach to the patient, a correct diagnosis was established so late, that a systemic infection developed.

  14. Re-evaluation of broiler carcass scalding protocols for impact on the recovery of Campylobacter from breast skin after defeathering

    Science.gov (United States)

    This research re-evaluated the impact of scalding protocols on the recovery of Campylobacter from breast skin following defeathering after preliminary processing trials detected Campylobacter from breast skin for 4/8 carcasses that had vents plugged and sutured prior to scalding. Published research...

  15. Experimental research and observation of the skin response of mice with a second-degree scald during irradiation by a CO2 laser

    Science.gov (United States)

    Wang, Yunxia; Wu, Shulian; Li, Zhifang; Xu, Xiaohui; Li, Hui

    2014-05-01

    Second-degree scalding is a common dermatological injury. Inappropriate treatment methods in clinical practice always produce scarring, and can lead to skin cancer and other complications in the longer term. In this study optical coherence tomography (OCT) combined with a skin detector was used to monitor the response of second-degree scalded skin tissue irradiated by a CO2 laser. The process of treatment of second-degree scalding was systematically studied from the perspective of tissue optics. The OCT signal intensity was stronger within the whole recovery period in the experimental group undergoing CO2 laser treatment, and the attenuation coefficient (μt) returned to its original value in a shorter time. The results help us to understand tissue injury in a second-degree scald and may help improve the standard treatment.

  16. Should dermal scald burns in children be covered with autologous skin grafts or with allogeneic cultivated keratinocytes?--"The Viennese concept".

    Science.gov (United States)

    Rab, Matthias; Koller, Rupert; Ruzicka, Margot; Burda, Gudrun; Kamolz, Lars Peter; Bierochs, Bettina; Meissl, Guenther; Frey, Manfred

    2005-08-01

    The treatment of scald burns in children is still under discussion. The aim of the present study was to evaluate an optimised treatment regime for scald burns in children. Between 1997 and 2002, 124 children underwent surgical intervention due to burn injuries. Thirty-six out of these 124 children were enrolled into the evaluation of our recent treatment protocol. Twenty-two children with scald burns covering an average body surface area (TBSA) of 18.5% were treated by early excision and coverage with allogeneic keratinocytes in case of partial thickness lesions (keratinocyte group). Fourteen children with a TBSA of 17.2% were treated with autologous skin grafts alone (skin graft group). Both groups were comparable according to age, burn depth and affected TBSA. The complete clinical follow-up examination of at least 17 months was performed in 12 out of 22 children of the keratinocyte group and in 9 out of 14 patients of the comparative group. Visible scar formations were classified according to the Vancouver Scar Scale (VSS) in each patient. The use of allogeneic keratinocytes led to complete epithelialisation within 12 days in 20 of the 22 cases. No secondary skin grafting procedures had to be done. Skin take rate at the sixth postoperative day was 100% in the skin graft group. Blood transfusions were administered intraoperatively according to the clinical need of the patients by the responsible anaesthesiologist. The mean volume of blood, which had to be transfused was 63.9 ml in the keratinocyte group and significantly lower than the volume of 151.4 ml, which was administered in the skin graft group (p=0.04). At follow up the VSS observed in areas covered by keratinocytes was 2.33 on the average and therefore, significantly lower than the VSS of 5.22 in skin grafted areas of the comparative group (p=0.04). In children the use of cultivated keratinocytes in partial thickness scald burns is a procedure, which renders constantly reliable results. It minimizes the

  17. Impact of broiler processing scalding and chilling profiles on carcass and breast meat yield.

    Science.gov (United States)

    Buhr, R J; Walker, J M; Bourassa, D V; Caudill, A B; Kiepper, B H; Zhuang, H

    2014-06-01

    The effect of scalding and chilling procedures was evaluated on carcass and breast meat weight and yield in broilers. On 4 separate weeks (trials), broilers were subjected to feed withdrawal, weighed, and then stunned and bled in 4 sequential batches (n = 16 broilers/batch, 64 broilers/trial). In addition, breast skin was collected before scalding, after scalding, and after defeathering for proximate analysis. Each batch of 16 carcasses was subjected to either hard (60.0°C for 1.5 min) or soft (52.8°C for 3 min) immersion scalding. Following defeathering and evisceration, 8 carcasses/batch were air-chilled (0.5°C, 120 min, 86% RH) and 8 carcasses/batch were immersion water-chilled (water and ice 0.5°C, 40 min). Carcasses were reweighed individually following evisceration and following chilling. Breast meat was removed from the carcass and weighed within 4 h postmortem. There were significant (P defeathered eviscerated weights did not differ between the scalding and chilling treatments. During air-chilling all carcasses lost weight, resulting in postchill carcass yield of 73.0% for soft-scalded and 71.3% for hard-scalded carcasses, a difference of 1.7%. During water-chilling all carcasses gained weight, resulting in heavier postchill carcass weights (2,031 g) than for air-chilled carcasses (1,899 g). Postchill carcass yields were correspondingly higher for water-chilled carcasses, 78.2% for soft-scalded and 76.1% for hard-scalded carcasses, a difference of 2.1%. Only in trials 1 and 4 was breast meat yield significantly lower for hard-scalded, air-chilled carcasses (16.1 and 17.5%) than the other treatments. Proximate analysis of skin sampled after scalding or defeathering did not differ significantly in moisture (P = 0.2530) or lipid (P = 0.6412) content compared with skin sampled before scalding. Skin protein content was significantly higher (P defeathering. The hard-scalding method used in this experiment did not result in increased skin lipid loss either

  18. Skin regeneration in deep second-degree scald injuries either by infusion pumping or topical application of recombinant human erythropoietin gel

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    Giri P

    2015-05-01

    Full Text Available Priya Giri,1 Sabine Ebert,1 Ulf-Dietrich Braumann,2 Mathias Kremer,3 Shibashish Giri,1 Hans-Günther Machens,4 Augustinus Bader1 1Department of Cell Techniques and Applied Stem Cell Biology, Center for Biotechnology and Biomedicine (BBZ, Faculty of Medicine, University of Leipzig, Leipzig, Germany; 2Interdisciplinary Center for Bioinformatics (IZBI, University of Leipzig, Leipzig, Germany; 3Department of Plastic and Hand Surgery, University of Lübeck, Lübeck, Germany; 4Department of Plastic and Hand Surgery, Technical University of Munich, Munich, Germany Abstract: Large doses of recombinant growth factors formulated in solution form directly injected into the body is usual clinical practice in treating second-degree scald injuries, with promising results, but this approach creates side effects; furthermore, it may not allow appropriate levels of the factor to be sensed by the target injured tissue/organ in the specific time frame, owing to complications arising from regeneration. In this research, two delivery methods (infusion pumping and local topical application were applied to deliver recombinant human erythropoietin (rHuEPO for skin regeneration. First, rHuEPO was given in deep second-degree scald injury sites in mice by infusion pump. Vascularization was remarkably higher in the rHuEPO pumping group than in controls. Second, local topical application of rHuEPO gel was given in deep second-degree scald injury sites in rats. Histological analysis showed that epithelialization rate was significantly higher in the rHuEPO gel-treated group than in controls. Immunohistochemical studies showed that the rHuEPO gel-treated group showed remarkably higher expression of skin regeneration makers than the control group. An accurate method for visualization and quantification of blood vessel networks in target areas has still not been developed up to this point, because of technical difficulties in detecting such thin blood vessels. A method which

  19. Effect of nano-silver hydrogel coating film on deep partial thickness scald model of rabbit.

    Science.gov (United States)

    Xi, Peng; Li, Yan; Ge, Xiaojin; Liu, Dandan; Miao, Mingsan

    2018-05-01

    Observing the effect of nano-silver hydrogel coating film on deep partial thickness scald model of rabbit. We prepared boiling water scalded rabbits with deep II degree scald models and applied high, medium and low doses of nano-silver hydrogel coating film for different time and area. Then we compared the difference of burned paper weight before administration and after administration model burns, burn local skin irritation points infection, skin crusting and scabs from the time, and the impact of local skin tissue morphology. Rabbits deep II degree burn model successful modeling; on day 12, 18, high, medium and low doses of nano-silver hydrogel coating film significantly reduced skin irritation of rabbits infected with the integral value ( P  film group significantly decreased skin irritation, infection integral value ( P  film significantly reduced film rabbits' scalded skin crusting time ( P  film on the deep partial thickness burns has a significant therapeutic effect; external use has a significant role in wound healing.

  20. [Clinical studies on flomoxef in neonates].

    Science.gov (United States)

    Tabuki, K; Nishimura, T

    1993-07-01

    Clinical studies on flomoxef (FMOX) were performed in neonates and the results obtained are summarized as follows. Treatment with FMOX was made in 4 cases of neonatal bacterial infections; 2 cases of sepsis (suspected) and 1 case each of infection of umbilicus and staphylococcal scalded skin syndrome. Results obtained were excellent in 1 case, good in 3 cases. No significant side effects due to the drug were observed in any cases.

  1. Effect of nano-silver hydrogel coating film on deep partial thickness scald model of rabbit

    OpenAIRE

    Peng Xi; Yan Li; Xiaojin Ge; Dandan Liu; Mingsan Miao

    2018-01-01

    Objective: Observing the effect of nano-silver hydrogel coating film on deep partial thickness scald model of rabbit. Method: We prepared boiling water scalded rabbits with deep II degree scald models and applied high, medium and low doses of nano-silver hydrogel coating film for different time and area. Then we compared the difference of burned paper weight before administration and after administration model burns, burn local skin irritation points infection, skin crusting and scabs from th...

  2. Effect of nano-silver hydrogel coating film on deep partial thickness scald model of rabbit

    Directory of Open Access Journals (Sweden)

    Peng Xi

    2018-05-01

    Full Text Available Objective: Observing the effect of nano-silver hydrogel coating film on deep partial thickness scald model of rabbit. Method: We prepared boiling water scalded rabbits with deep II degree scald models and applied high, medium and low doses of nano-silver hydrogel coating film for different time and area. Then we compared the difference of burned paper weight before administration and after administration model burns, burn local skin irritation points infection, skin crusting and scabs from the time, and the impact of local skin tissue morphology. Result: Rabbits deep II degree burn model successful modeling; on day 12, 18, high, medium and low doses of nano-silver hydrogel coating film significantly reduced skin irritation of rabbits infected with the integral value (P < 0.01, P < 0.05; high, medium and low doses of nano-silver hydrogel coating film group significantly decreased skin irritation, infection integral value (P < 0.01, P < 0.05; high, medium and low doses of nano-silver hydrogel coating film significantly reduced film rabbits’ scalded skin crusting time (P < 0.01, significantly shortened the rabbit skin burns from the scab time (P < 0.01, and significantly improved the treatment of skin diseases in rabbits scald model change (P < 0.01, P < 0.05. Conclusion: The nano-silver hydrogel coating film on the deep partial thickness burns has a significant therapeutic effect; external use has a significant role in wound healing. Keywords: Nano-silver hydrogel coating film, Deep degree burns, Topical, Rabbits

  3. Steam versus hot-water scalding in reducing bacterial loads on the skin of commercially processed poultry.

    Science.gov (United States)

    Patrick, T E; Goodwin, T L; Collins, J A; Wyche, R C; Love, B E

    1972-04-01

    A comparison of two types of scalders was conducted to determine their effectiveness in reducing bacterial contamination of poultry carcasses. A conventional hot-water scalder and a prototype model of a steam scalder were tested under commercial conditions. Total plate counts from steam-scalded birds were significantly lower than the counts of water-scalded birds immediately after scalding and again after picking. No differences in the two methods could be found after chilling. Coliform counts from steam-scalded birds were significantly lower than the counts from water-scalded birds immediately after scalding. No significant differences in coliform counts were detected when the two scald methods were compared after defeathering and chilling.

  4. APPLICATION OF THE HACCP METHOD IN THE SWINE ABATTOIR BY SCALDING-SKINNING ECOLOGICAL PROCESSING / APPLICATION DE LA METHODE HACCP DANS L’ABATTOIR PORCIN PAR LE PROCESSUS ECOLOGIQUE D’ÉCHAUDAGE-DÉPEÇAGE

    OpenAIRE

    IULIAN SUCEVEANU; IRINA-CLAUDIA ALEXA; LUMINITA GROSU; OANA-IRINA PATRICIU; IRINA IFRIM; MIRELA SUCEVEANU

    2017-01-01

    In the food industry development, the food safety and the valorization of by-products represent important aspects. The present study consists in the application of the Hazard Analysis and Critical Control Point (HACCP) method in pig slaughterhouses for scalding-skinning technology in order to ensure the conformity of meat quality and the by-products management and recycling. In order to optimize the scalding-skinning process of swine, all the classical technological steps of the technological...

  5. Cross-sectional study to identify staphylococcal species isolated from teat and inguinal skin of different-aged dairy heifers.

    Science.gov (United States)

    Adkins, P R F; Dufour, S; Spain, J N; Calcutt, M J; Reilly, T J; Stewart, G C; Middleton, J R

    2018-04-01

    The purpose of this study was to describe the prevalence and distribution of staphylococcal species on the teat and inguinal skin of dairy heifers across the various stages of the heifer life cycle. The cross-sectional study included 106 Holstein heifers with an age range of 0 d to 27 mo that were selected from 11 different groups, based on housing type and age, on a single dairy operation. A composite swabbing sample including all 4 teats and a second composite sample including both inguinal regions of each heifer were collected using gas-sterilized electrostatic dusters (Swiffers; Procter and Gamble, Cincinnati, OH). Swabbing samples were mixed with 10 mL of sterile saline, agitated, and cultured on mannitol salt agar plates. At 24 h, plates were read and up to 10 staphylococcal colonies were saved for further analysis. Staphylococcal isolates were speciated using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry or PCR amplification and partial sequencing of rpoB or tuf. The prevalence of staphylococci was compared between the inguinal and teat regions using the chi-squared or Fisher's exact test, as applicable. Logistic regression models were used to investigate the relationship between a heifer's age (treated as a quantitative continuous variable) and the probability of isolating a given staphylococcal species from a given body site (inguinal region or teats). Overall, the most common species identified were Staphylococcus haemolyticus followed by Staphylococcus chromogenes, Staphylococcus xylosus, Staphylococcus devriesei, and Staphylococcus sciuri. Staphylococcus aureus was more prevalent on the teat than in the inguinal region, whereas Staphylococcus arlettae was more prevalent in the inguinal region than on the teat. All other staphylococcal species were as likely to be found on the teat skin as the inguinal region skin. Isolation from the inguinal and teat skin was associated with age for Staphylococcus agnetis, S. chromogenes

  6. Bacterial recovery from breast skin of genetically feathered and featherless broiler carcasses immediately following scalding and picking.

    Science.gov (United States)

    Buhr, R J; Berrang, M E; Cason, J A

    2003-10-01

    Genetically feathered and featherless sibling broilers selected for matched BW were killed, scalded, and defeathered to determine the consequences of feathers and empty feather follicles on the recovery of bacteria from carcass breast skin. In trial 1, the vents of all carcasses were plugged and sutured before scalding to prevent the expulsion of cloacal contents during picking. In trial 2, half of the carcasses had their vents plugged and sutured. Immediately after defeathering, breast skin was aseptically removed, and bacteria associated with it were enumerated. In trial 1, the levels of bacteria recovered did not differ between feathered and featherless carcasses: Campylobacter log10 1.4 cfu/mL of rinse, coliform log10 1.8, Escherichia coli log10 1.6, and total aerobic bacteria log10 3.1. In trial 2, the carcasses that had vents plugged and sutured had lower levels of all four types of bacteria (differences of Campylobacter log10 0.7 cfu/mL, coliform log10 1.8, E. coli log10 1.7, and total aerobic bacteria log10 0.5) than those carcasses with open vents. The lower levels of bacteria recovered from carcasses with the vents plugged and sutured during picking enabled detection of small but significant differences between feathered and featherless carcasses. The level of coliform and E. coli recovered was slightly higher by log10 0.7 cfu for feathered carcasses, but featherless carcasses had marginally higher levels of total aerobic bacteria by log10 0.4 cfu. Feathered and featherless carcasses with open vents during picking did not differ in the levels of recovery of coliform, E. coli, and total aerobic bacteria from breast skin.

  7. Clinical and Molecular Epidemiology of Staphylococcal Toxic Shock Syndrome in the United Kingdom

    Science.gov (United States)

    Sharma, Hema; Smith, Debra; Turner, Claire E.; Game, Laurence; Pichon, Bruno; Hope, Russell; Hill, Robert; Kearns, Angela

    2018-01-01

    Staphylococcal toxic shock syndrome (TSS) was originally described in menstruating women and linked to TSS toxin 1 (TSST-1)–producing Staphylococcus aureus. Using UK national surveillance data, we ascertained clinical, molecular and superantigenic characteristics of TSS cases. Average annual TSS incidence was 0.07/100,000 population. Patients with nonmenstrual TSS were younger than those with menstrual TSS but had the same mortality rate. Children <16 years of age accounted for 39% of TSS cases, most caused by burns and skin and soft tissue infections. Nonmenstrual TSS is now more common than menstrual TSS in the UK, although both types are strongly associated with the tst+ clonal complex (CC) 30 methicillin-sensitive S. aureus lineage, which accounted for 49.4% of all TSS and produced more TSST-1 and superantigen bioactivity than did tst+ CC30 methicillin-resistant S. aureus strains. Better understanding of this MSSA lineage and infections in children could focus interventions to prevent TSS in the future. PMID:29350159

  8. Wound Healing Effects of Prunus yedoensis Matsumura Bark in Scalded Rats

    Directory of Open Access Journals (Sweden)

    Jin-Ho Lee

    2017-01-01

    Full Text Available Pruni Cortex has been used to treat asthma, measles, cough, urticaria, pruritus, and dermatitis in traditional Korean medicine. The objective of this study was to investigate the effects of Prunus yedoensis Matsumura bark methanol extract (PYE on scald-induced dorsal skin wounds in rats. Scalds were produced in Sprague-Dawley rats with 100°C water and treated with 5% and 20% PYE (using Vaseline as a base, silver sulfadiazine (SSD, and Vaseline once a day for 21 days, beginning 24 hours after scald by treatment group allocation. The PYE-treated groups showed accelerated healing from 12 days after scald, demonstrated by rapid eschar exfoliation compared to the control and SSD groups. PYE-treated groups showed higher wound contraction rates and better tissue regeneration in comparison with the control group. Serum analysis showed that transforming growth factor beta 1 and vascular endothelial growth factor levels remained high or gradually increased up to day 14 in both PYE groups and then showed a sharp decline by day 21, implying successful completion of the inflammatory phase and initiation of tissue regeneration. These findings suggested that PYE is effective in promoting scald wound healing in the inflammation and tissue proliferation stages.

  9. Staphylococcal superantigens; toxic shock syndrome toxin-1 and enterotoxins in pediatric otitis media effusion: a brief report

    Directory of Open Access Journals (Sweden)

    Noorbakhsh S

    2013-03-01

    Full Text Available Background: Staphylococcal superantigens (SAg's may have some role in otitis media with effusion (OME. The aim of this study was the search of staphylococcal SAg's in middle ear effusion of children with OME.  Methods: This cross sectional-analytic study was done in ENT & pediatric wards upon 64 children with otitis media with effusion (OME between 1-15 years, (mean age=7.42+4 years of Rasoul Akram University Hospital, Tehran, Iran in 2009-2011. Fifty six percent (36 of cases were male, 43.8% (28 were female. Staphylococcal SAg's; Toxic Shock Syndrome Toxin-1 (TSST-1, Staphylococcal enterotoxin A, B, C, D (Enzyme immune assay, AB Cam, USA were detected in middle ear effusion samples after conventional culture.Results: None type of SAg's found in 39% of OME cases, enterotoxin B found in: 22%; enterotoxin A: 17%, enterotoxin C: 15.6%, enterotoxin D: 12.5%, Toxic Shock Syndrome Toxin-1 (TSST-1: 7.8% Mean age of cases with positive TSST-1, enterotoxin A, B, C, and D was: 1, 5, 8.6, 9.6 and 9.6 years respectively. Positive TSST had no agreement with positive enterotoxin A and C but had weak agreement with type B and D. Mean age of cases with positive TSST was one years which had significant difference with (7.9 years in cases with negative TSST test (P<0.0001.Conclusion: At least one or more type of staphylococcal toxins had found in middle ear effusion of 70% of OME cases with negative culture for Staphylococcus aureus. Even in culture negative cases, staphylococcal toxins might have some immunologic role in middle ear effusion forming. Finding the SAg's (at least one type are important for treatment of immunosuppressive or corticosteroid in cases with resistant OME.

  10. Skin Peeling Syndrome

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    Sharma Rajeev

    2000-01-01

    Full Text Available Peeling of the skin is an uncommonly encountered disorder. Occurrence of vesicles and bullae in peeling skin syndrome is very rare. We report a case of idiopathic peeling skin syndrome with vesicular lesions.

  11. Skin regeneration with conical and hair follicle structure of deep second-degree scalding injuries via combined expression of the EPO receptor and beta common receptor by local subcutaneous injection of nanosized rhEPO

    Directory of Open Access Journals (Sweden)

    Ebert S

    2012-03-01

    Full Text Available Augustinus Bader1, Sabine Ebert1, Shibashish Giri1, Mathias Kremer2, Shuhua Liu2, Andreas Nerlich5, Christina I Günter³, Dagmar U Smith4, Hans-Günther Machens2,31Department of Applied Stem Cell Biology and Cell Techniques, Centre for Biotechnology and Biomedicine, University of Leipzig, Leipzieg, 2Department of Plastic and Hand Surgery, University of Lübeck, Lübeck, 3Department of Plastic and Hand Surgery, Technische Universität München, Munich, 4Münchner Studienzentrum, Technische Universität München, Munich, 5Institute of Pathology, Klinikum München-Bogenhausen, Munich, GermanyBackground: Acceleration of skin regeneration is still an unsolved problem in the clinical treatment of patients suffering from deep burns and scalds. Although erythropoietin (EPO has a protective role in a wide range of organs and cells during ischemia and after trauma, it has been recently discovered that EPO is not tissue-protective in the common β subunit receptor (βCR knockout mouse. The protective capacity of EPO in tissue is mediated via a heteroreceptor complex comprising both the erythropoietin receptor (EPOR and βCR. However, proof of coexpression of these heterogenic receptors in regenerating skin after burns is still lacking.Methods: To understand the role of nanosized recombinant human erythropoietin (rhEPO in wound healing, we investigated the effects of subcutaneous injections of EPO on skin regeneration after deep second-degree scalding injuries. Our aim was to determine if joint expression of EPOR and βCR is a prerequisite for the tissue-protective effect of rhEPO. The efficiency in wound regeneration in a skin scalding injury mouse model was examined. A deep second-degree dermal scald injury was produced on the backs of 20 female Balb/c mice which were subsequently randomized to four experimental groups, two of which received daily subcutaneous injections of rhEPO. At days 7 and 14, the mice were sacrificed and the effects of rhEPO were

  12. Lack of a standardised UK care pathway resulting in national variations in management and outcomes of paediatric small area scalds.

    Science.gov (United States)

    Trevatt, Alexander E J; Kirkham, Emily N; Allix, Bradley; Greenwood, Rosemary; Coy, Karen; Hollén, Linda I; Young, Amber E R

    2016-09-01

    There is a paucity of evidence guiding management of small area partial thickness paediatric scalds. This has prevented the development of national management guidelines for these injuries. This research aimed to investigate whether a lack of evidence for national guidelines has resulted in variations in both management and outcomes of paediatric small area scalds across England and Wales (E&W). A national survey of initial management of paediatric scalds ≤5% Total Body Surface Area (%TBSA) was sent to 14 burns services in E&W. Skin graft rates of anonymised burns services over seven years were collected from the international Burns Injury Database (iBID). Average skin grafting rates across services were compared. Length of stay and proportion of patients receiving general anaesthesia for dressing application at each service were also compared. All 14 burns services responded to the survey. Only 50% of services had a protocol in place for the management of small area burns. All protocols varied in how partial thickness paediatrics scalds ≤5% TBSA should be managed. There was no consensus as to which scalds should be treated using biosynthetic dressings. Data from iBID for 11,917 patients showed that the average reported skin grafting rate across all burns services was 2.3% (95% CI 2.1, 2.6) but varied from 0.3% to 7.1% (P<0.001). Service provider remained associated with likelihood of skin grafting when variations in the %TBSA case mix seen by each service were controlled for (χ(2)=87.3, P<0.001). The use of general anaesthetics across services varied between 0.6 and 35.5% (P<0.001). The median length of stay across services varied from 1 to 3 days (P<0.001). A lack of evidence guiding management of small-area paediatric scalds has resulted in variation in management of these injuries across E&W. There is also significant variation in outcomes for these injuries. Further research is indicated to determine if care pathways and outcomes are linked. An evidence

  13. Emergence of a Staphylococcus aureus Clone Resistant to Mupirocin and Fusidic Acid Carrying Exotoxin Genes and Causing Mainly Skin Infections.

    Science.gov (United States)

    Doudoulakakis, Anastassios; Spiliopoulou, Iris; Spyridis, Nikolaos; Giormezis, Nikolaos; Kopsidas, John; Militsopoulou, Maria; Lebessi, Evangelia; Tsolia, Maria

    2017-08-01

    Skin and soft tissue infections (SSTIs) caused by mupirocin-resistant Staphylococcus aureus strains have recently increased in number in our settings. We sought to evaluate the characteristics of these cases over a 43-month period. Data for all community-acquired staphylococcal infections caused by mupirocin-resistant strains were retrospectively reviewed. Genes encoding products producing high-level resistance (HLR) to mupirocin ( mupA ), fusidic acid resistance ( fusB ), resistance to macrolides and lincosamides ( ermC and ermA ), Panton-Valentine leukocidin (PVL) ( lukS/lukF -PV), exfoliative toxins ( eta and etb ), and fibronectin binding protein A ( fnbA ) were investigated by PCRs in 102 selected preserved strains. Genotyping was performed by SCC mec and agr typing, whereas clonality was determined by pulsed-field gel electrophoresis (PFGE) and multilocus sequence typing (MLST). A total of 437 cases among 2,137 staphylococcal infections were recorded in 2013 to 2016; they were all SSTIs with the exception of 1 case of primary bacteremia. Impetigo was the predominant clinical entity (371 cases [84.9%]), followed by staphylococcal scalded skin syndrome (21 cases [4.8%]), and there were no abscesses. The number of infections detected annually increased during the study years. All except 3 isolates were methicillin susceptible. The rates of HLR to mupirocin and constitutive resistance to clindamycin were 99% and 20.1%, respectively. Among the 102 tested strains, 100 (98%) were mupA positive and 97 (95%) were fusB positive, 26/27 clindamycin-resistant strains (96.3%) were ermA positive, 83 strains (81.4%) were lukS/lukF positive, 95 (93%) carried both eta and etb genes, and 99 (97%) were fnbA positive. Genotyping of methicillin-sensitive S. aureus (MSSA) strains revealed that 96/99 (96.7%) belonged to one main pulsotype, pulsotype 1, classified as sequence type 121 (ST121). The emergence of a single MSSA clone (ST121) causing impetigo was documented. Resistance to

  14. APPLICATION OF THE HACCP METHOD IN THE SWINE ABATTOIR BY SCALDING-SKINNING ECOLOGICAL PROCESSING / APPLICATION DE LA METHODE HACCP DANS L’ABATTOIR PORCIN PAR LE PROCESSUS ECOLOGIQUE D’ÉCHAUDAGE-DÉPEÇAGE

    Directory of Open Access Journals (Sweden)

    IULIAN SUCEVEANU

    2017-03-01

    Full Text Available In the food industry development, the food safety and the valorization of by-products represent important aspects. The present study consists in the application of the Hazard Analysis and Critical Control Point (HACCP method in pig slaughterhouses for scalding-skinning technology in order to ensure the conformity of meat quality and the by-products management and recycling. In order to optimize the scalding-skinning process of swine, all the classical technological steps of the technological processes were analyzed separately and globally, with the adaptation of the HACCP method for the new technology. The result of this study shows that the implementation of the new technology that combines existing technologies has many advantages from both an economic and a technological point of view. At the same time the new technology ensures food safety by taking account of the application of the food safety system, respectively, HACCP method.

  15. Genetics Home Reference: acral peeling skin syndrome

    Science.gov (United States)

    ... Home Health Conditions Acral peeling skin syndrome Acral peeling skin syndrome Printable PDF Open All Close All ... to view the expand/collapse boxes. Description Acral peeling skin syndrome is a skin disorder characterized by ...

  16. Repurposing ebselen for treatment of multidrug-resistant staphylococcal infections.

    Science.gov (United States)

    Thangamani, Shankar; Younis, Waleed; Seleem, Mohamed N

    2015-06-26

    Novel antimicrobials and new approaches to developing them are urgently needed. Repurposing already-approved drugs with well-characterized toxicology and pharmacology is a novel way to reduce the time, cost, and risk associated with antibiotic innovation. Ebselen, an organoselenium compound, is known to be clinically safe and has a well-known pharmacology profile. It has shown potent bactericidal activity against multidrug-resistant clinical isolates of staphylococcus aureus, including methicillin- and vancomycin-resistant S. aureus (MRSA and VRSA). We demonstrated that ebselen acts through inhibition of protein synthesis and subsequently inhibited toxin production in MRSA. Additionally, ebselen was remarkably active and significantly reduced established staphylococcal biofilms. The therapeutic efficacy of ebselen was evaluated in a mouse model of staphylococcal skin infections. Ebselen 1% and 2% significantly reduced the bacterial load and the levels of the pro-inflammatory cytokines tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), interleukin-1 beta (IL-1β), and monocyte chemo attractant protein-1 (MCP-1) in MRSA USA300 skin lesions. Furthermore, it acts synergistically with traditional antimicrobials. This study provides evidence that ebselen has great potential for topical treatment of MRSA skin infections and lays the foundation for further analysis and development of ebselen as a potential treatment for multidrug-resistant staphylococcal infections.

  17. [Effects of seawater immersion on the inflammatory response and oxygen free radical injury of rats with superficial partial-thickness scald at early stage].

    Science.gov (United States)

    Yang, Y X; Wang, J H; Liu, L; Zou, Q; Zhang, Y; Bai, Z

    2017-06-20

    Objective: To study the effects of seawater immersion on the inflammatory response and oxygen free radical injury of rats with superficial-thickness scald at early stage. Methods: Seventy Wistar rats were divided into healthy control group (HC, n =7), pure scald group (PS, n =21), scald+ fresh water immersion group (SF, n =21), and scald+ seawater immersion group (SS, n =21) according to the random number table. Rats in group HC did not receive any treatment, while 5% total body surface area superficial partial-thickness scald was made on the back of rats in the latter three groups. Rats in group PS lived freely immediately post burn, while wounds on the back of rats in groups SF and SS were immersed into fresh water and seawater, respectively. Serum and full-thickness skin tissue in the center of wounds on the back of 7 rats in groups PS, SF, and SS at post immersion (injury) hour (PIH) 2, 4, and 6 were collected, respectively, while serum and full-thickness skin tissue at the same position of the 7 rats in group HC were collected at PIH 6 of rats in other groups. Morphology of skin tissue was observed with HE staining; tumor necrosis factor-alpha (TNF-α) content in serum and skin tissue was determined by enzyme-linked immunosorbent assay; superoxide dismutase (SOD) content in serum and skin tissue was determined by hydroxylamine method; malondialdehyde content in serum and skin tissue was determined by thiobarbituric acid method. Data were processed with analysis of variance of factorial design, one-way analysis of variance, Welch test, LSD test, and Tamhane test. Results: (1) Epidermal cells of skin tissue of rats in group HC arranged in order and continuously, and the dermis tissue and accessory structures were clear and complete. The skin layer and epidermis of wounds of rats in group PS had no significant change, but the edema of epidermis and dermis and infiltration of inflammatory cells enhanced over time at PIH 2, 4, and 6. The horny layer of epidermis of

  18. Recurrent Skin and Lung Infections in Autosomal Dominant Hyper IgE Syndrome with Transactivation Domain STAT3 Mutation

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    Chad J. Cooper

    2014-01-01

    Full Text Available Background. Hyper IgE is a rare systemic disease characterized by the clinical triad of high serum levels of IgE (>2000 IU/mL, eczema, and recurrent staphylococcal skin and lung infections. The presentation of hyper IgE syndrome is highly variable, which makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiency disorders. Case Report. A 23-year-old Hispanic presented with history of frequent respiratory and gastrointestinal infections as a child and multiple episodes of skin and lung infections (abscess with Staphylococcus aureus throughout his adult life. He had multiple eczematous lesions and folliculitis over his entire body, oral/esophageal candidiasis, and retention of his primary teeth. The IgE was elevated (>5000 IU/mL. Genetic mutation analysis revealed a mutation affecting the transactivation domain of the STAT3 gene. Conclusion. The hallmark of hyper IgE syndrome is serum IgE of >2000 IU/mL. Hyper IgE syndrome is a genetic disorder that is either autosomal dominant or recessive. A definite diagnosis can be made with genetic mutation analysis, and in this case, it revealed a very rare finding of the transactivation domain STAT3 mutation. Hyper IgE syndrome is a challenge for clinicians in establishing a diagnosis in suspected cases.

  19. Panton-Valentine Leukocidin-Producing Staphylococcal aureus: Report of Four Siblings.

    LENUS (Irish Health Repository)

    2012-01-31

    Panton-Valentine leukocidin (PVL)-producing Staphylococcus aureus results in leukocyte destruction and tissue necrosis (Pediatric Dermatology 2007;24:401). It can be associated with a spectrum of clinical manifestations that range from localized staphylococcal skin infections to sometimes severe necrotizing pneumonia (Clin Infect Dis 1999;29:1128). We report a case of four siblings, three brothers whose atopic dermatitis was complicated by cutaneous lesions and furunculosis, while their 21-month-old sister had a fatal PVL positive staphylococcal pneumonia.

  20. Lactobacilli require physical contact to reduce staphylococcal TSST-1 secretion and vaginal epithelial inflammatory response

    NARCIS (Netherlands)

    Younes, Jessica A.; Reid, Gregor; van der Mei, Henny C.; Busscher, Henk J.

    Staphylococcus aureus biofilms can be found on vaginal epithelia, secreting toxins and causing inflammation. The co-vaginal species Lactobacillus can alter staphylococcal-induced epithelial secretion of inflammatory cytokines and quench staphylococcal toxic shock syndrome toxin-1 secretion. It is

  1. Staphylococcal enterotoxins in the Etiopathogenesis of Mucosal Autoimmunity within the Gastrointestinal Tract

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    MaryAnn Principato

    2014-04-01

    Full Text Available The staphylococcal enterotoxins (SEs are the products of Staphylococcus aureus and are recognized as the causative agents of classical food poisoning in humans following the consumption of contaminated food. While illness evoked by ingestion of the SE or its producer organism in tainted food are often self-limited, our current understanding regarding the evolution of S. aureus provokes the utmost concern. The organism and its associated toxins, has been implicated in a wide variety of disease states including infections of the skin, heart, sinuses, inflammatory gastrointestinal disease, toxic shock, and Sudden Infant Death Syndrome. The intricate relationship between the various subsets of immunocompetent T cells and accessory cells and the ingested material found within the gastrointestinal tract present daunting challenges to the maintenance of immunologic homeostasis. Dysregulation of the intricate balances within this environment has the potential for extreme consequences within the host, some of which are long-lived. The focus of this review is to evaluate the relevance of staphylococcal enterotoxin in the context of mucosal immunity, and the underlying mechanisms that contribute to the pathogenesis of gastrointestinal autoimmune disease.

  2. Epidemiological investigation of nosocomial outbreak of staphylococcal skin diseases in neonatal ward.

    Science.gov (United States)

    Kurlenda, J; Grinholc, M; Krzysztoń-Russjan, J; Wiśniewska, K

    2009-05-01

    During a 1-month period, eight neonates developed staphylococcal skin disease diagnosed as a bullous impetigo in the maternity unit of the Provincial Hospital in Gdansk. An epidemiological investigation based on phenotyping and genotyping methods was performed. All neonates involved in the outbreak, their mothers and 15 staff members were screened for carriage of Staphylococcus aureus by nasal swabs. Isolated strains were compared with strains cultured from affected skin and purulent conjunctiva of infected newborns. Isolates were analyzed for the presence of the etA and etB genes using polymerase chain reaction and genotyped by pulsed-field gel electrophoresis (PFGE) and coa gene polymorphism. The analyzed S. aureus strains were methicillin-sensitive and could be divided into two groups according to antibiotyping, phage typing, coa polymorphism and PFGE pattern. The first group consisted of etA and etB negative strains, and the second one involved only the etB positive ones. Our results have shown that there were two different clusters of infection caused by two populations of S. aureus strains. Among the 15 medical staff members screened we have found seven carriers. However, phage typing revealed that distinct strains unrelated to the outbreak isolates were carried. Although we have not been able to establish the source of bacteria involved in the outbreak, our results suggest that for both groups, mothers could be the source of the infecting strains.

  3. Use of a scald additive to reduce levels of Salmonella typhimurium during poultry processing.

    Science.gov (United States)

    McKee, S R; Townsend, J C; Bilgili, S F

    2008-08-01

    This study was conducted to evaluate the efficacy of a scald additive, RP scald, to reduce Salmonella Typhimurium (ST) levels on inoculated poultry carcasses. The RP scald (contains sodium hydroxide) in a 1% solution has a pH of 11.0, which may reduce bacteria levels on carcasses. In this study, 600 broilers (Ross 708 straight run, 6 wk of age) with 300 broilers in each of 2 experimental trials were divided into 4 scald treatments (inoculated with ST) and 2 noninoculated groups. The treatment groups included 4 scald treatments (n = 50 per experimental group per trial): soft scald (SS; 50 degrees C for 90 s), soft scald with 1.0% added RP scald (SSRP), hard scald (56.6 degrees C for 45 s; HS), and hard scald with 1.0% added RP scald. The noninoculated groups (n = 50 per group per trial) are represented by SS0 and HS0. After defeathering, carcass rinses were collected for ST detection. Results indicated that inoculated broilers from hard scald with 1.0% added RP scald had the lowest Salmonella recovery, whereas carcasses from the SS treatment with no RP additive had the highest ST recovery. In trial 1, the SSRP was more effective in reducing ST than HS alone; however, this trend was not consistent. In trial 2, HS alone was more effective in ST reduction than SSRP. Within each scald temperature, the addition of RP scald increased ST reduction; therefore, RP scald may be effective in reducing ST on broiler carcasses in poultry scalder applications, particularly when hard scald temperatures are used.

  4. Skin findings in Williams syndrome.

    Science.gov (United States)

    Kozel, Beth A; Bayliss, Susan J; Berk, David R; Waxler, Jessica L; Knutsen, Russell H; Danback, Joshua R; Pober, Barbara R

    2014-09-01

    Previous examination in a small number of individuals with Williams syndrome (also referred to as Williams-Beuren syndrome) has shown subtly softer skin and reduced deposition of elastin, an elastic matrix protein important in tissue recoil. No quantitative information about skin elasticity in individuals with Williams syndrome is available; nor has there been a complete report of dermatologic findings in this population. To fill this knowledge gap, 94 patients with Williams syndrome aged 7-50 years were recruited as part of the skin and vascular elasticity (WS-SAVE) study. They underwent either a clinical dermatologic assessment by trained dermatologists (2010 WSA family meeting) or measurement of biomechanical properties of the skin with the DermaLab™ suction cup (2012 WSA family meeting). Clinical assessment confirmed that soft skin is common in this population (83%), as is premature graying of the hair (80% of those 20 years or older), while wrinkles (92%), and abnormal scarring (33%) were detected in larger than expected proportions. Biomechanical studies detected statistically significant differences in dP (the pressure required to lift the skin), dT (the time required to raise the skin through a prescribed gradient), VE (viscoelasticity), and E (Young's modulus) relative to matched controls. The RT (retraction time) also trended longer but was not significant. The biomechanical differences noted in these patients did not correlate with the presence of vascular defects also attributable to elastin insufficiency (vascular stiffness, hypertension, and arterial stenosis) suggesting the presence of tissue specific modifiers that modulate the impact of elastin insufficiency in each tissue. © 2014 Wiley Periodicals, Inc.

  5. Development of a Consistent and Reproducible Porcine Scald Burn Model

    Science.gov (United States)

    Kempf, Margit; Kimble, Roy; Cuttle, Leila

    2016-01-01

    There are very few porcine burn models that replicate scald injuries similar to those encountered by children. We have developed a robust porcine burn model capable of creating reproducible scald burns for a wide range of burn conditions. The study was conducted with juvenile Large White pigs, creating replicates of burn combinations; 50°C for 1, 2, 5 and 10 minutes and 60°C, 70°C, 80°C and 90°C for 5 seconds. Visual wound examination, biopsies and Laser Doppler Imaging were performed at 1, 24 hours and at 3 and 7 days post-burn. A consistent water temperature was maintained within the scald device for long durations (49.8 ± 0.1°C when set at 50°C). The macroscopic and histologic appearance was consistent between replicates of burn conditions. For 50°C water, 10 minute duration burns showed significantly deeper tissue injury than all shorter durations at 24 hours post-burn (p ≤ 0.0001), with damage seen to increase until day 3 post-burn. For 5 second duration burns, by day 7 post-burn the 80°C and 90°C scalds had damage detected significantly deeper in the tissue than the 70°C scalds (p ≤ 0.001). A reliable and safe model of porcine scald burn injury has been successfully developed. The novel apparatus with continually refreshed water improves consistency of scald creation for long exposure times. This model allows the pathophysiology of scald burn wound creation and progression to be examined. PMID:27612153

  6. Contamination of pig carcasses with scalding water studied with a radiolabelled colloid

    Energy Technology Data Exchange (ETDEWEB)

    Jones, E; Nilsson, T; Ekman, L; Oestlund, K [Sveriges Lantbruksuniversitet, Uppsala. Dept. of Clinical Chemistry; Sveriges Lantbruksuniversitet, Uppsala. Abt. fuer Lebensmittelhygiene; Schwedisches Fleischforschungszentrum, Kaevlinge

    1979-10-01

    Swine carcasses at slaughter are normally scalded after bleeding, i.e. immersed in a hot-water-tank. The present study was made to investigate whether during this treatment scalding water enters the severed vessels via the stick wound and contaminates the carcass. Five experimental pigs were slaughtered and immersed in a scalding vat containing water with a dispersed radiolabelled colloid, sup(99m)Tc-sulphide. After scalding muscles and other tissues from various parts of the body were examined for radioactivity. Scalding water (radioactivity) could be demonstrated in tissues from all investigated parts of the carcass. The highest amounts were found in the lungs and in the large blood vessels. The hygienic significance of the findings is discussed.

  7. Ectodermal dysplasia-skin fragility syndrome

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    Vijay S Adhe

    2011-01-01

    Full Text Available Ectodermal dysplasia-skin fragility (EDSF syndrome is a rare and first described inherited disorder of desmosomes. It occurs due to loss-of-function mutations in PKP1 gene resulting in poorly formed desmosomes and loss of desmosomal and epidermal integrity. We report a case of a 2-year-old Indian male child who presented with palmoplantar hyperkeratosis with fissuring, short, sparse, and easily pluckable scalp hair, nail dystrophy, and multiple erosions over the skin. Skin biopsy showed epidermal hyperplasia with widening of intercellular spaces. His developmental milestones were delayed but intelligence was normal. Echocardiography, X-ray chest, and electrocardiogram were normal. Very few cases of this syndrome have been reported in the literature. We consider this as the first case report from India.

  8. Target metabolite and gene transcription profiling during the development of superficial scald in apple (Malus x domestica Borkh).

    Science.gov (United States)

    Busatto, Nicola; Farneti, Brian; Tadiello, Alice; Vrhovsek, Urska; Cappellin, Luca; Biasioli, Franco; Velasco, Riccardo; Costa, Guglielmo; Costa, Fabrizio

    2014-07-20

    Fruit quality features resulting from ripening processes need to be preserved throughout storage for economical reasons. However, during this period several physiological disorders can occur, of which superficial scald is one of the most important, due to the development of large brown areas on the fruit skin surface. This study examined the variation in polyphenolic content with the progress of superficial scald in apple, also with respect to 1-MCP, an ethylene competitor interacting with the hormone receptors and known to interfere with this etiology. The change in the accumulation of these metabolites was further correlated with the gene set involved in this pathway, together with two specific VOCs (Volatile Organic Compounds), α-farnesene and its oxidative form, 6-methyl-5-hepten-2-one. Metabolite profiling and qRT-PCR assay showed these volatiles are more heavily involved in the signalling system, while the browning coloration would seem to be due more to a specific accumulation of chlorogenic acid (as a consequence of the activation of MdPAL and MdC3H), and its further oxidation carried out by a polyphenol oxidase gene (MdPPO). In this physiological scenario, new evidence regarding the involvement of an anti-apoptotic regulatory mechanism for the compartmentation of this phenomenon in the skin alone was also hypothesized, as suggested by the expression profile of the MdDAD1, MdDND1 and MdLSD1 genes. The results presented in this work represent a step forward in understanding the physiological mechanisms of superficial scald in apple, shedding light on the regulation of the specific physiological cascade.

  9. Early childhood severe scalds in a developing country: A 3-year retrospective study

    Directory of Open Access Journals (Sweden)

    Pius Agbenorku

    2013-12-01

    Full Text Available The burns intensive care unit (BICU staff observed an increasing number of pediatric scald burn admissions as a result of increase injuries associated with the scald burns. A retrospective study was conducted to identify scalds demographics, etiologies, and mortality risk factors. This descriptive study comprised a total of 166 patients aged 0-5 years, who were admitted to the BICU of the Reconstructive Plastic Surgery and Burns Unit (RPSBU through the Accident and Emergency (A and E Centre of the Komfo Anokye Teaching Hospital (KATH from May 1 st 2009 to April 30 th 2012. Source of information was the BICU Computerized Database System. Data extracted included demographics as well as treatment methods and outcomes. The study population was 166; 92 (55.4% males and 74 (44.6% females. Scalds admissions were 141 (84.9%; 13 (9.2% of them died, 83 (58.9% discharged, and 45 (31.9% transferred-out to another burn ward and pediatric surgery ward in the hospital. Scald patients' demographics included 78 males (55.3% and 63 females (44.7%; mean age was 2.18 years. Mortality risk factors identified were age <3 years (P = 0.044; scalds from hot water (P = 0.033, total burns surface area >30% (P = 0.017, and multiple body parts affected (P = 0.049. The current study showed age, hot water, and Total Burns Surface Area (TBSA as risk factors of early childhood scalds. Education on scalds prevention targeting mothers/caregivers is needed to create awareness of the frequency, severity, and danger associated with pediatric scalds.

  10. Patterns of scald injuries in children--has anything changed?

    LENUS (Irish Health Repository)

    Yates, J

    2011-10-01

    The objective was to study presentation patterns of scald injuries in children and suggest potential countermeasures to reduce these injuries. We retrospectively studied scald injuries in children attending an urban paediatric emergency department between January 1st and December 31st 2008. Data was extracted from our emergency department database using search terms \\'burn\\

  11. Filaggrin-dependent secretion of sphingomyelinase protects against staphylococcal α-toxin-induced keratinocyte death.

    Science.gov (United States)

    Brauweiler, Anne M; Bin, Lianghua; Kim, Byung Eui; Oyoshi, Michiko K; Geha, Raif S; Goleva, Elena; Leung, Donald Y M

    2013-02-01

    The skin of patients with atopic dermatitis (AD) has defects in keratinocyte differentiation, particularly in expression of the epidermal barrier protein filaggrin. AD skin lesions are often exacerbated by Staphylococcus aureus-mediated secretion of the virulence factor α-toxin. It is unknown whether lack of keratinocyte differentiation predisposes to enhanced lethality from staphylococcal toxins. We investigated whether keratinocyte differentiation and filaggrin expression protect against cell death induced by staphylococcal α-toxin. Filaggrin-deficient primary keratinocytes were generated through small interfering RNA gene knockdown. RNA expression was determined by using real-time PCR. Cell death was determined by using the lactate dehydrogenase assay. Keratinocyte cell survival in filaggrin-deficient (ft/ft) mouse skin biopsies was determined based on Keratin 5 staining. α-Toxin heptamer formation and acid sphingomyelinase expression were determined by means of immunoblotting. We found that filaggrin expression, occurring as the result of keratinocyte differentiation, significantly inhibits staphylococcal α-toxin-mediated pathogenicity. Furthermore, filaggrin plays a crucial role in protecting cells by mediating the secretion of sphingomyelinase, an enzyme that reduces the number of α-toxin binding sites on the keratinocyte surface. Finally, we determined that sphingomyelinase enzymatic activity directly prevents α-toxin binding and protects keratinocytes against α-toxin-induced cytotoxicity. The current study introduces the novel concept that S aureus α-toxin preferentially targets and destroys filaggrin-deficient keratinocytes. It also provides a mechanism to explain the increased propensity for S aureus-mediated exacerbation of AD skin disease. Copyright © 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.

  12. 9 CFR 311.30 - Livestock suffocated and hogs scalded alive.

    Science.gov (United States)

    2010-01-01

    ... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Livestock suffocated and hogs scalded alive. 311.30 Section 311.30 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE, DEPARTMENT... PARTS § 311.30 Livestock suffocated and hogs scalded alive. All livestock which have been suffocated in...

  13. Skin peeling syndrome

    Directory of Open Access Journals (Sweden)

    Gharpuray Mohan

    1994-01-01

    Full Text Available We are reporting a case of skin peeling syndrome, a rare disorder in which sudden generalized exfoliation of the stratum corneum occurs. Histopathologically, there was well formed subcorneal pustule filled with polymorphs and nuclear dust, considering this to be a varient of subcorneal pustular dermatosis, we have put the patient on Dapsone.

  14. A case of peeling skin syndrome

    OpenAIRE

    Anil K Singhal; Devendra K Yadav; Bajrang Soni; Savita Arya

    2017-01-01

    Peeling skin syndrome is a very rare autosomal recessive disease characterized by widespread painless peeling of the skin in superficial sheets. Etiology is still unknown with an autosomal recessive inheritance. Less than 100 cases have been reported in the medical literature. We present a 32-year-old man having asymptomatic peeling of skin since birth. Sheets of skin were peeling from his neck, trunk, and extremities, following friction or rubbing especially if pre-soaked in water but sparin...

  15. Acral peeling skin syndrome: report of two cases.

    Science.gov (United States)

    García, Elena García; Carreño, Rosario Granados; Martínez González, Miguel A; Reyes, José Jiménez

    2005-01-01

    Peeling skin syndrome is a rare dermatosis characterized by spontaneous and painless peeling of the skin. The authors report two patients with history of spontaneous, asymptomatic, and noninflammatory peeling skin of the acral surfaces after soaking in water. On light microscopy, blisters were located in the mid layers of the stratum corneum, above the granular layer. Ultrastructural examination revealed increased intercellular lipids and abnormal, "moth-eaten," keratohyalin granules, but the authors were unable to determine whether the separation initiated within the horny cells or between adjacent cells. These patients represented a localized variant of peeling skin syndrome.

  16. Peeling skin syndrome associated with novel variant in FLG2 gene.

    Science.gov (United States)

    Alfares, Ahmed; Al-Khenaizan, Sultan; Al Mutairi, Fuad

    2017-12-01

    Peeling skin syndrome is a rare genodermatosis characterized by variably pruritic superficial generalized peeling of the skin with several genes involved until now little is known about the association between FLG2 and peeling skin syndrome. We describe multiple family members from a consanguineous Saudi family with peeling skin syndrome. Next Generation Sequencing identifies a cosegregating novel variant in FLG2 c.632C>G (p.Ser211*) as a likely etiology in this family. Here, we reported on the clinical manifestation of homozygous loss of function variant in FLG2 as a disease-causing gene for peeling skin syndrome and expand the dermatology findings. © 2017 Wiley Periodicals, Inc.

  17. Electroacupuncture at Zusanli Prevents Severe Scalds-Induced Gut Ischemia and Paralysis by Activating the Cholinergic Pathway

    Directory of Open Access Journals (Sweden)

    Huan Wang

    2015-01-01

    Full Text Available Severe burn injuries may result in gastrointestinal paralysis, and barrier dysfunction due to gut ischemia and lowered vagus excitability. In this study we investigate whether electroacupuncture (EA at Zusanli (ST36 could prevent severe scalds-induced gut ischemia, paralysis, and barrier dysfunction and whether the protective role of EA at ST36 is related to the vagus nerve. 35% burn area rats were divided into six groups: (a EAN: EA nonchannel acupoints followed by scald injury; (b EA: EA at ST36 after scald injury; (c VGX/EA: vagotomy (VGX before EA at ST36 and scald injury; (d VGX/EAN: VGX before EAN and scald injury; (e atropine/EA: applying atropine before scald injury and then EA at ST36; (f atropine/EAN: applying atropine before scald injury and then EA at nonchannel acupoints. EA at the Zusanli point significantly promoted the intestinal impelling ratio and increased the amount of mucosal blood flow after scald injury. The plasma diamine oxidase (DAO and intestinal permeability decreased significantly after scald injury in the EA group compared with others. However, EA after atropine injection or cervical vagotomy failed to improve intestinal motility and mucosa blood flow suggesting that the mechanism of EA may be related to the activation of the cholinergic nerve pathway.

  18. Bacteriophage-Derived Peptidase CHAPK Eliminates and Prevents Staphylococcal Biofilms

    Directory of Open Access Journals (Sweden)

    Mark Fenton

    2013-01-01

    Full Text Available New antibacterial agents are urgently needed for the elimination of biofilm-forming bacteria that are highly resistant to traditional antimicrobial agents. Proliferation of such bacteria can lead to significant economic losses in the agri-food sector. This study demonstrates the potential of the bacteriophage-derived peptidase, CHAPK, as a biocidal agent for the rapid disruption of biofilm-forming staphylococci, commonly associated with bovine mastitis. Purified CHAPK applied to biofilms of Staphylococcus aureus DPC5246 completely eliminated the staphylococcal biofilms within 4 h. In addition, CHAPK was able to prevent biofilm formation by this strain. The CHAPK lysin also reduced S. aureus in a skin decolonization model. Our data demonstrates the potential of CHAPK as a biocidal agent for prevention and treatment of biofilm-associated staphylococcal infections or as a decontaminating agent in the food and healthcare sectors.

  19. Pediatric soup scald burn injury: etiology and prevention.

    Science.gov (United States)

    Palmieri, Tina L; Alderson, Tyrone S; Ison, Dahlia; O'Mara, Michael S; Sharma, Raj; Bubba, Anthony; Coombs, Elena; Greenhalgh, David G

    2008-01-01

    One of the leading causes of scald burn injury in children is from hot soup, particularly prepackaged instant soups. The purpose of this study was to determine the demographic, socioeconomic, and situational factors that contribute to the incidence of scald burns in children. A 20-item questionnaire was given to the caregiver of children who were treated for scald burn injury at a pediatric burn center from July 2006 to March 2007. Questions included demographics (child age, gender, siblings, ethnicity), socioeconomic status (income, education), factors contributing to the injury (type of soup, child supervision, type of container), and location of injury. The mean age of the 78 children sustaining burn injury and completing the survey was 4.8 +/- 0.6 years. The majority of patients were girls (51%), and the most frequently involved ethnic group was Hispanic (44%). Households had a mean of 3.0 +/- 0.3 children in residence, and an income of less than $29,000/year (59%). The highest educational level achieved was high school for 73% of the parents. Prepackaged soup (65%) with a narrow base heated directly in the original container (46%) using the microwave (51%) was implicated in the majority of burns. Soup scald burns, especially from prepackaged instant soups, appear to predominate in lower income families with multiple children. The majority of injuries occur when the caregiver heats the soup in the original container using the microwave. Prevention of these types of injuries will require a two-pronged approach: educating families with multiple children and changing the soup packaging.

  20. Application of distilled white vinegar in the cloaca to counter the increase in Campylobacter numbers on broiler skin during feather removal.

    Science.gov (United States)

    Berrang, M E; Smith, D P; Hinton, A

    2006-02-01

    Because of the escape of highly contaminated gut contents from the cloaca of positive carcasses, Campylobacter numbers recovered from broiler carcass skin samples increase during automated feather removal. Vinegar is known to have antimicrobial action. The objective of this study was to determine the effect of vinegar placed in the cloaca prior to feather removal on the numbers of Campylobacter recovered from broiler breast skin. Broilers were stunned, killed, and bled in a pilot processing plant. Vinegar was placed in the colons of the chickens prior to scalding. Carcasses were scalded, and Campylobacter numbers were determined on breast skin before and after passage through a commercial-style feather-picking machine. Campylobacter numbers recovered from the breast skin of untreated control carcasses increased during feather removal from 1.3 log CFU per sample prior to defeathering to 4.2 log afterward. Placement of water in the colon before scalding had no effect on Campylobacter numbers. Campylobacter numbers recovered from the breast skin of carcasses treated with vinegar also increased during defeathering but to a significantly lesser extent. Treated carcasses experienced only a 1-log increase from 1.6 log CFU per sample before feather removal to 2.6 log CFU per sample afterward. Application of an effective food-grade antimicrobial in the colon prior to scald can limit the increase in Campylobacter contamination of broiler carcasses during defeathering.

  1. QTL analysis of soft scald in two apple populations

    OpenAIRE

    McClure, Kendra A; Gardner, Kyle M; Toivonen, Peter MA; Hampson, Cheryl R; Song, Jun; Forney, Charles F; DeLong, John; Rajcan, Istvan; Myles, Sean

    2016-01-01

    The apple (Malus?domestica Borkh.) is one of the world?s most widely grown and valuable fruit crops. With demand for apples year round, storability has emerged as an important consideration for apple breeding programs. Soft scald is a cold storage-related disorder that results in sunken, darkened tissue on the fruit surface. Apple breeders are keen to generate new cultivars that do not suffer from soft scald and can thus be marketed year round. Traditional breeding approaches are protracted a...

  2. The skin function: a factor of anti-metabolic syndrome

    Directory of Open Access Journals (Sweden)

    Zhou Shi-Sheng

    2012-04-01

    Full Text Available Abstract The body’s total antioxidant capacity represents a sum of the antioxidant capacity of various tissues/organs. A decrease in the body’s antioxidant capacity may induce oxidative stress and subsequent metabolic syndrome, a clustering of risk factors for type 2 diabetes and cardiovascular disease. The skin, the largest organ of the body, is one of the major components of the body’s total antioxidant defense system, primarily through its xenobiotic/drug biotransformation system, reactive oxygen species-scavenging system, and sweat glands- and sebaceous glands-mediated excretion system. Notably, unlike other contributors, the skin contribution is variable, depending on lifestyles and ambient temperature or seasonal variations. Emerging evidence suggests that decreased skin’s antioxidant and excretory functions (e.g., due to sedentary lifestyles and low ambient temperature may increase the risk for metabolic syndrome. This review focuses on the relationship between the variability of skin-mediated detoxification and elimination of exogenous and endogenous toxic substances and the development of metabolic syndrome. The potential role of sebum secretion in lipid and cholesterol homeostasis and its impact on metabolic syndrome, and the association between skin disorders (acanthosis nigricans, acne, and burn and metabolic syndrome are also discussed.

  3. Erythroderma in children

    Directory of Open Access Journals (Sweden)

    Sarkar Rashmi

    2010-01-01

    Full Text Available Although erythroderma is a well-recognized entity in the adult age groups and has been studied by various authors, there is a paucity of studies on erythroderma in the pediatric age group. It poses a greater challenge to the dermatologist and pediatrician because of its potential life threatening nature. In a study conducted by us in a large Indian hospital to delineate the causes of neonatal and infantile erythroderma, the causes identified were infections (40%, ichthyosiform erythroderma (25%, atopic dermatitis (15%, infantile seborrheic dermatitis (10% and unidentified (10%. In another study of childhood erythroderma, etiologically, drugs (29% showed the highest incidence, followed equally (18% by genodermatoses, psoriasis and staphylococcal scalded skin syndrome (SSSS. The management of childhood erythroderma is mainly supportive with correction of the hematologic, biochemical and metabolic imbalance if required. In this review, the causes of childhood erythroderma, the clinical features useful to the diagnosis and management are discussed.

  4. Skin symptoms in four ectodermal dysplasia syndromes including two case reports of Rapp-Hodgkin-Syndrome.

    Science.gov (United States)

    Knaudt, Björn; Volz, Thomas; Krug, Markus; Burgdorf, Walter; Röcken, Martin; Berneburg, Mark

    2012-01-01

    The skin, hair and nail changes in four distinct ectodermal dysplasia syndromes are compared and reviewed. These syndromes comprise Christ-Siemens-Touraine syndrome; ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome; ankyloblepharon-ectodermal defects-cleft lip/palate syndrome and Rapp-Hodgkin syndrome. A comprehensive overview of the dermatological signs and symptoms in these syndromes was generated from the database of the Ectodermal Dysplasia Network Germany, the clinical findings in the patients seen in our department and an extensive review of the literature. The findings included abnormalities of skin, sweating, hair and nails. These clinical findings are discussed in relation to the underlying molecular defects known to play a role in these four ectodermal dysplasia syndromes.

  5. Touraine Solente Gole syndrome: The elephant skin disease

    Directory of Open Access Journals (Sweden)

    T. M. Sheeja Rajan

    2013-01-01

    Full Text Available Touraine Solente Gole syndrome is a rare hereditary syndrome of primary pachydermoperiostosis, with the characteristic triad of pachydermia (or elephant like skin, periostosis and acropachia. A 27-year-old patient presented with aesthetic deformity of forehead due to deep skin folds and coarsening of facial features due to progressive thickening of skin. Associated palmoplantar hyperkeratosis with broadened of finger and toe tips and digital clubbing were noticed. Dermatologic evaluation revealed cutis verticis gyrata of scalp, seborrhoeic hyperplasia of face and hyperhidrosis. Natural history of the disease and aetiopathogenesis were reviewed. Aesthetic correction of forehead through frontal rhytidectomy was attempted.

  6. Steven johnsons syndrome and toxic epidermal necrolysis: A review

    Directory of Open Access Journals (Sweden)

    Sri ram Anne

    2014-12-01

    Full Text Available Toxic epidermal necrolysis (TEN and Stevens Johnson Syndrome (SJS are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the etiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP, disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS. Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy.

  7. Pediatric superficial scald burns--reassessment of our follow-up protocol.

    Science.gov (United States)

    Egro, Francesco M; O'Neill, Jennifer K; Briard, Robert; Cubison, Tania C S; Kay, Alan R; Estela, Catalina M; Burge, Timothy S

    2010-01-01

    The most common pediatric burn injury is a superficial scald. The current follow-up protocol for such burns includes review of the patient at 2 weeks postinjury and then 2 months later. The authors decided to review the protocol to assess the need for this second follow-up. A retrospective study reviewed the case notes of patients younger than 16 years at the time of their injury presenting with a scald over 5% TBSA. The progress of healing and scar development up to 5 years follow-up was assessed. This study showed that scalds healing within 2 weeks following injury rarely became hypertrophic. A prospective study was performed over a 10-month period. All children who suffered a superficial partial-thickness scald injury were included. At the 2-week appointment, the need for further follow-up was predicted. The accuracy of this prediction was assessed 2 months later. This study showed that an experienced member of the burns team could reliably predict at 2-week appointment those children who could be safely discharged with no subsequent need for scar management. This study suggests that it will be safe to modify the follow-up protocol, reducing the number of clinic attendances.

  8. Toxic shock syndrome

    Science.gov (United States)

    Staphylococcal toxic shock syndrome; Toxic shock-like syndrome; TSLS ... Toxic shock syndrome is caused by a toxin produced by some types of staphylococcus bacteria. A similar problem, called toxic shock- ...

  9. Granulomatous slack skin syndrome: Report of a unique case.

    Science.gov (United States)

    Maheswari, S Uma; Sampath, V; Ramesh, A

    2018-01-01

    Granulomatous slack skin syndrome is a rare variant of cutaneous T-cell lymphoma (mycosis fungoides). It is characterized clinically by redundant skin folds, which show a predilection towards flexural areas such as the axilla and the groin. Histologically, it shows a granulomatous T-cell infiltrate and loss of elastic tissue. It has an indolent but progressive course; and is usually refractory to treatment. We report a unique case of slack skin syndrome, sparing the classical sites with rapid and unusual involvement of non-intertriginous areas.

  10. Lactobacilli require physical contact to reduce staphylococcal TSST-1 secretion and vaginal epithelial inflammatory response.

    Science.gov (United States)

    Younes, Jessica A; Reid, Gregor; van der Mei, Henny C; Busscher, Henk J

    2016-06-01

    ITALIC! Staphylococcus aureusbiofilms can be found on vaginal epithelia, secreting toxins and causing inflammation. The co-vaginal species ITALIC! Lactobacilluscan alter staphylococcal-induced epithelial secretion of inflammatory cytokines and quench staphylococcal toxic shock syndrome toxin-1 secretion. It is hypothesized that these effects of lactobacilli require direct physical contact between lactobacilli, staphylococci and the epithelium. Indeed, lactobacilli only reduced ITALIC! S. aureus-induced inflammatory cytokine expression when allowed physical contact with vaginal epithelial cells. Furthermore, a reduction in toxic shock syndrome toxin-1 secretion only occurred when a probiotic ITALIC! Lactobacillusstrain was allowed contact, but not when being physically separated from ITALIC! S. aureus Bacterial-probe atomic force microscopy demonstrated that lactobacilli and staphylococci strongly adhere to epithelial cells, while lactobacilli adhere stronger to staphylococci than staphylococci to each other, giving lactobacilli opportunity to penetrate and reside in staphylococcal biofilms, as visualized using confocal laser scanning microscopy with fluorescence ITALIC! in situhybridization probes. These results identify that physical contact and biochemical signaling by lactobacilli are intrinsically linked mechanisms that reduce virulence of ITALIC! S. aureusbiofilm. © FEMS 2016. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  11. A Case of Peeling Skin Syndrome.

    Science.gov (United States)

    Singhal, Anil K; Yadav, Devendra K; Soni, Bajrang; Arya, Savita

    2017-01-01

    Peeling skin syndrome is a very rare autosomal recessive disease characterized by widespread painless peeling of the skin in superficial sheets. Etiology is still unknown with an autosomal recessive inheritance. Less than 100 cases have been reported in the medical literature. We present a 32-year-old man having asymptomatic peeling of skin since birth. Sheets of skin were peeling from his neck, trunk, and extremities, following friction or rubbing especially if pre-soaked in water but sparing palm and soles. Histologically, there was epidermal separation at the level of stratum corneum, just above the stratum granulosum. This case is being presented due to its rarity.

  12. A case of peeling skin syndrome

    Directory of Open Access Journals (Sweden)

    Anil K Singhal

    2017-01-01

    Full Text Available Peeling skin syndrome is a very rare autosomal recessive disease characterized by widespread painless peeling of the skin in superficial sheets. Etiology is still unknown with an autosomal recessive inheritance. Less than 100 cases have been reported in the medical literature. We present a 32-year-old man having asymptomatic peeling of skin since birth. Sheets of skin were peeling from his neck, trunk, and extremities, following friction or rubbing especially if pre-soaked in water but sparing palm and soles. Histologically, there was epidermal separation at the level of stratum corneum, just above the stratum granulosum. This case is being presented due to its rarity.

  13. Manipulation of Innate and Adaptive Immunity by Staphylococcal Superantigens

    Directory of Open Access Journals (Sweden)

    Stephen W. Tuffs

    2018-05-01

    Full Text Available Staphylococcal superantigens (SAgs constitute a family of potent exotoxins secreted by Staphylococcus aureus and other select staphylococcal species. SAgs function to cross-link major histocompatibility complex (MHC class II molecules with T cell receptors (TCRs to stimulate the uncontrolled activation of T lymphocytes, potentially leading to severe human illnesses such as toxic shock syndrome. The ubiquity of SAgs in clinical S. aureus isolates suggests that they likely make an important contribution to the evolutionary fitness of S. aureus. Although the apparent redundancy of SAgs in S. aureus has not been explained, the high level of sequence diversity within this toxin family may allow for SAgs to recognize an assorted range of TCR and MHC class II molecules, as well as aid in the avoidance of humoral immunity. Herein, we outline the major diseases associated with the staphylococcal SAgs and how a dysregulated immune system may contribute to pathology. We then highlight recent research that considers the importance of SAgs in the pathogenesis of S. aureus infections, demonstrating that SAgs are more than simply an immunological diversion. We suggest that SAgs can act as targeted modulators that drive the immune response away from an effective response, and thus aid in S. aureus persistence.

  14. Effects of plasma CGRP and NPY level changes on intestinal mucosal barrier injury after scald in rats

    International Nuclear Information System (INIS)

    Shao Lijian; Zhu Qingxian; He Ming; Zhang Hongyan

    2004-01-01

    Objective: To investigate the significance of plasma CGRP and NPY levels changes immediately after scald in rats. Methods: Thirty-two rat models of 30% TBSA III degree scald were prepared. Eight animals each were sacrificed at 3, 6,12 and 24 hrs; taking blood samples for determination of plasma CGRP, NPY levels and 5 cm of ileum for pathologic study. As controls, eight animals without scald were treated in the same way. Results: Plasma CGRP levels were decreased significantly after scald, reaching bottom value at 12 hr and remained lower than those in controls at 24 hr (p 0.05). Plasma levels of CGRP were negatively correlated to plasma NPY levels (p<0.01). Ileum mucosal injuries presented as edema, congestion with necrosis and slough of epithelium were most marked at 12 hr. Conclusion: Plasma CGRP and NPY levels changed significantly after scald and were mutually negatively correlated. Post-scald intestinal mucosa barrier injuries were possibly related to the changes of levels of those vasoactive peptides

  15. Facial peeling skin syndrome: a case report and a brief review.

    Science.gov (United States)

    Janjua, Shahbaz Ahmad; Hussain, Ijaz; Khachemoune, Amor

    2007-03-01

    Peeling skin syndrome is an extremely rare genodermatosis of possible autosomal recessive inheritance, characterized by asymptomatic spontaneous exfoliation of the stratum corneum at a subcorneal or intracorneal level. It usually presents at birth or appears later in early childhood. The condition may be generalized or localized. Here we describe a case of localized continual skin peeling limited to the facial skin in a 6-month-old infant, with two other members of the family affected with the same condition. A few cases of localized skin peeling limited to the acral surfaces have been described in the literature, but a familial case of localized skin peeling limited to the facial skin has not been described before. We believe that our patient represents a new subtype of peeling skin syndrome, limited to the skin of the face.

  16. Programming MapReduce with Scalding

    CERN Document Server

    Chalkiopoulos, Antonios

    2014-01-01

    This book is an easy-to-understand, practical guide to designing, testing, and implementing complex MapReduce applications in Scala using the Scalding framework. It is packed with examples featuring log-processing, ad-targeting, and machine learning. This book is for developers who are willing to discover how to effectively develop MapReduce applications. Prior knowledge of Hadoop or Scala is not required; however, investing some time on those topics would certainly be beneficial.

  17. Mild Staphylococcus aureus Skin Infection Improves the Course of Subsequent Endogenous S. aureus Bacteremia in Mice.

    Directory of Open Access Journals (Sweden)

    Sanne van den Berg

    Full Text Available Staphylococcus aureus carriers with S. aureus bacteremia may have a reduced mortality risk compared to non-carriers. A role for the immune system is suggested. Here, we study in mice the effect of mild S. aureus skin infection prior to endogenous or exogenous S. aureus bacteremia, and evaluate protection in relation to anti-staphylococcal antibody levels. Skin infections once or twice by a clinical S. aureus isolate (isolate P or S. aureus strain 8325-4 were induced in mice free of S. aureus and anti-staphylococcal antibodies. Five weeks later, immunoglobulin G (IgG levels in blood against 25 S. aureus antigens were determined, and LD50 or LD100 bacteremia caused by S. aureus isolate P was induced. S. aureus skin infections led to elevated levels of anti-staphylococcal IgG in blood. One skin infection improved the course of subsequent severe endogenous bacteremia only. A second skin infection further improved animal survival rate, which was associated with increased pre-bacteremia IgG levels against Efb, IsaA, LukD, LukE, Nuc, PrsA and WTA. In conclusion, S. aureus isolate P skin infection in mice reduces the severity of subsequent endogenous S. aureus bacteremia only. Although cellular immune effects cannot be rules out, anti-staphylococcal IgG against specified antigens may contribute to this effect.

  18. Ectodermal Dysplasia Skin Fragility Syndrome

    Directory of Open Access Journals (Sweden)

    Ayça Alan Atalay

    2014-06-01

    Full Text Available Ectodermal dysplasia-skin fragility syndrome (EDSFS is a rare autosomal recessive genodermatosis first described in 1997 by Mc Grath. EDSFS results from loss of function mutations in plakophilin-1 (PKP1. PKP1 is a structural component of desmosomes, cellcell adhesion complexes. It is also found as a nuclear protein in several cell types that are lack of desmosomes. In skin, however, PKP1 expression is confined mainly to suprabasal keratinocytes and the outer root sheath of hair follicules. Loss of function mutation in PKP1 leads to extensive skin fragility, bullae and erosions following minor trauma, focal keratoderma with painful fissures, alopecia, and nail dystrophy. In some patients hypohidrosis may also be seen. EDSFS is now considered as a specific suprabasal form of epidermolysis bullosa simplex. In this report we describe a 20 year old EDSFS case.

  19. Hot tea and tiny tots don't mix: A cross-sectional survey on hot beverage scalds.

    Science.gov (United States)

    Burgess, J D; Kimble, R M; Watt, K A; Cameron, C M

    2017-12-01

    Hot beverage scalds are a leading cause of burns in young children. The aim of this study was to look at the circumstances surrounding these injuries in terms of setting, mechanism, supervision and first aid to inform a prevention campaign. A cross-sectional study was delivered via iPad to parents and caregivers presenting with a child aged 0-36 months with a hot beverage scald at a major paediatric burns centre. Of the 101 children aged 0-36 months that presented with a hot beverage scald over a 12-month period, 54 participants were included. The scald aetiology was as expected with the peak prevalence in children aged 6-24 months, pulling a cup of hot liquid down over themselves. The majority of injuries occurred in the child's home and were witnessed by the caregiver or parent. The supervising adult was often in close proximity when the scald occurred. Less than a third (28%) of participants received recommended first aid treatment at the scene, with an additional 18% receiving this treatment with three hours of the injury-usually at an emergency department. While the aetiology of these scalds were as expected, the low use of recommended burn first aid was of concern. Although supervision was present in almost all cases, with the parent/caregiver close-by, this proximity still permitted injury. Attentiveness and continuity of supervision, which can be difficult with competing parental demands, appear to play a more important role role; as do considerations of other safety mechanisms such as hazard reduction through keeping hot drinks out of reach and engineering factors such as improved cup design. By incorporating the findings from this study and other research into a hot beverage scald prevention campaign, we hope to see a change in knowledge and behaviour in parents and caregivers of young children, and ultimately a reduction in the incidence of hot beverage scalds. Crown Copyright © 2017. Published by Elsevier Ltd. All rights reserved.

  20. Skin signs in Ehlers-Danlos syndrome: clinical tests and para-clinical methods

    DEFF Research Database (Denmark)

    Remvig, Lars; Duhn, Ph; Ullman, S

    2010-01-01

    Objective: The criteria for Ehlers-Danlos syndrome (EDS) and the hypermobility syndrome (HMS) should be reliable. Examination for general joint hypermobility has high reliability but there is only sparse information on the reliability of skin tests, and no information on the level of normal skin...

  1. Skin signs in Ehlers-Danlos syndrome: clinical tests and para-clinical methods

    DEFF Research Database (Denmark)

    Remvig, L; Duhn, Ph; Ullman, S

    2010-01-01

    The criteria for Ehlers-Danlos syndrome (EDS) and the hypermobility syndrome (HMS) should be reliable. Examination for general joint hypermobility has high reliability but there is only sparse information on the reliability of skin tests, and no information on the level of normal skin extensibility...

  2. Modeling Skin Injury from Hot Rice Porridge Spills

    Directory of Open Access Journals (Sweden)

    Torgrim Log

    2018-04-01

    Full Text Available The present work analyzes skin burns from spills of hot rice and milk products. The traditional Norwegian rice porridge serves as an example. By testing spills on objects emulating an arm, it was concluded that spills were seldom thinner than 3 mm, and stayed in place due to the viscosity of the porridge for more than one minute. The Pennes bioheat equation was solved numerically for such spills, including heat conduction to the skin and convective heat losses from the porridge surface. Temperatures were analyzed in the porridge and skin layers, and the resulting skin injury was calculated based on the basal layer temperature. Parameters influencing burn severity, such as porridge layer thickness, porridge temperature, removal of the porridge and thermal effects of post scald tempered (15 °C water cooling were analyzed. The spilled porridge resulted in a prolonged heat supply to the skin, and the skin injury developed significantly with time. The porridge temperature turned out to be the most important injury parameter. A 70 °C porridge temperature could develop superficial partial-thickness burns. Porridge temperatures at processing temperatures nearly instantly developed severe burns. It was demonstrated that prompt removal of the hot porridge significantly reduced the injury development. The general advice is to avoid serving porridge and similar products at temperatures above 65 °C and, if spilled on the skin, to remove it quickly. After such scald incidents, it is advised to cool the injured area by tempered water for a prolonged period to stimulate healing.

  3. Biobrane versus topical agents in the treatment of adult scald burns.

    Science.gov (United States)

    Krezdorn, Nicco; Könneker, Sören; Paprottka, Felix Julian; Tapking, Christian; Mett, Tobias R; Brölsch, G Felix; Boyce, Maria; Ipaktchi, Ramin; Vogt, Peter M

    2017-02-01

    Limited data is available for treatment of scald lesions in adults. The use of the biosynthetic matrix Biobrane ® has been suggested as treatment option with more benefits over topical dressings. Application of Biobrane ® in scalds in our center led to a perceived increase of infection, secondary deepening, surgery and length of stay. We therefore assessed the effect of different treatment options in adult scalds in our center. We performed a retrospective cohort study of adult patients that have been admitted with scalds in our center between 2011 and 2014. We assessed two groups, group 1 with Biobrane ® as initial treatment and group 2 with topical treatment using polyhexanid hydrogel and fatty gauze. Primary outcome variables were rate of secondary deepening, surgery, infection (defined as positive microbiological swabs and antibiotic treatment) and length of stay. Total body surface area (TBSA) as well as diabetes mellitus (DM), hypertension, smoking and alcohol consumption as potential confounders were included. A total of 52 patients were included in this study. 36 patients received treatment with Biobrane ® and 16 with ointment and fatty gauze. No significant differences were found for age and TBSA whereas gender ratio was different (25/11 male/female in group 1 vs 4/12 in group 2, p=0.003). Rate of secondary deepening, surgery, infection as well as days of hospital stay (DOHS) were comparable. Logistic and multilinear regression showed TBSA to be a predictive factor for infection (p=0.041), and TBSA and age for length of stay (age p=0.036; TBSA p=0.042) in group 1. The use of Biobrane ® in adult scald lesions is safe and non-inferior to topical treatment options. In elder patients and larger TBSA Biobrane ® may increase the risk of infection or a prolonged stay in hospital. Level 3 - retrospective cohort study. Copyright © 2016 Elsevier Ltd and ISBI. All rights reserved.

  4. [An experimental study on the prevention of enteral bacterial translocation in scalded rats by smectite powder].

    Science.gov (United States)

    Su, Hai-tao; Li, Yi-shu; Lu, Shu-liang; Sun, Man; Qing, Chun; Li, Zong-yu; Shao, Tie-bing; Huang, Li-bing; Qu, Bing; Yang, Xin-bo

    2005-04-01

    To explore the preventive and treatment effects of smectite powder on enteral bacterial translocation in scalded rats. Fifty-four Sprague-Dawley (SD) rats were randomly divided into three groups, i.e. normal control (A, n = 6), burn control (B, n = 24), and burn treatment (T, n = 24) groups. The rats in B and T groups were fed with tracing bacteria JM109, which was transfected with PUC19 plasmid in advance. The rats were subjected to 30% TBSA scald injury after the plasmid was shown to have colonized in the intestine. Smectite powder (0.6 g/day/kg) was fed to rats of T group immediately after the scalding, while those in B group received no smectite powder. Bacterial translocation in blood and mesenteric lymph nodes in all groups was observed and identified by enzyme digestion at 12 post scald hour (PSH) and on 1, 3 and 5 post-scald days (PSD). The contents of malondialdehyde (MDA) and superoxide dismutase (SOD) were determined in rat intestinal tissue. And the degree of injury to the entire small intestine was observed pathologically. The villus height of intestinal mucosa was measured, and the rate of epithelial nuclear splitting of mucosal crypts was calculated. The number of rats with positive blood bacterial culture in B group was obviously higher than that in A and T groups (P Smectite powder is beneficial to the protection of the intestinal mucosa in scalded rats, and can effectively prevent postburn intestinal bacterial translocation in rats.

  5. Skin extensibility and consistency in patients with Ehlers-Danlos syndrome and benign joint hypermobility syndrome

    DEFF Research Database (Denmark)

    Remvig, L; Duhn, P H; Ullman, S

    2009-01-01

    OBJECTIVES: The reproducibility of clinical tests for skin extensibility and consistency, essential for differentiating between types of Ehlers-Danlos syndrome (EDS) and benign joint hypermobility syndrome (BJHS), is unknown. Paraclinical methods may provide objective differential diagnostic...

  6. Monkey Feeding Assay for Testing Emetic Activity of Staphylococcal Enterotoxin.

    Science.gov (United States)

    Seo, Keun Seok

    2016-01-01

    Staphylococcal enterotoxins (SEs) are unique bacterial toxins that cause gastrointestinal toxicity as well as superantigenic activity. Since systemic administration of SEs induces superantigenic activity leading to toxic shock syndrome that may mimic enterotoxic activity of SEs such as vomiting and diarrhea, oral administration of SEs in the monkey feeding assay is considered as a standard method to evaluate emetic activity of SEs. This chapter summarizes and discusses practical considerations of the monkey feeding assay used in studies characterizing classical and newly identified SEs.

  7. Campylobacter, Salmonella, and Escherichia coli on broiler carcasses subjected to a high pH scald and low pH postpick chlorine dip.

    Science.gov (United States)

    Berrang, M E; Windham, W R; Meinersmann, R J

    2011-04-01

    The objective of this study was to determine the individual and combined effects of a high pH scald and a postpick chlorine dip on bacteria present on broiler carcasses. In each of 3 replications, a flock was sampled at several sites within a commercial broiler processing plant. Carcasses were sampled by whole carcass rinse before and after treated scalding at mean pH 9.89 or control scalding at mean pH 6.88. Other carcasses from the same flock run on both the treated and control scald lines were collected and sampled before and after a chlorine dip tank operated at mean total chlorine level of 83.3 mg/kg and pH 6.04. Rinses were cultured for numbers of Campylobacter and Escherichia coli and presence or absence of Salmonella. High pH scald was more effective than standard scald to lessen the prevalence and numbers of Campylobacter on broiler carcasses; a lower prevalence was maintained through the postpick chlorine dip tank. The pH of the scald tank made no difference in numbers of E. coli recovered from broiler carcasses at any tested point on the processing line. High pH scald was not more effective than standard scald to lessen Salmonella prevalence. Furthermore, it is unclear why the postpick chlorine dip effectively lessened Salmonella prevalence on only the control scald line. Although no evidence exists that these treatments have an additive effect when used in series, each treatment shows some promise individually. Further optimization may result in more effective decontamination of broiler carcasses.

  8. Acral peeling skin syndrome in two East-African siblings: case report.

    Science.gov (United States)

    Kiprono, Samson K; Chaula, Baraka M; Naafs, Bernard; Masenga, John E

    2012-03-19

    Acral peeling skin syndrome is a rare autosomal recessive genodermatosis due to a missense mutation in transglutaminase 5. The skin peeling occurs at the separation of the stratum corneum from the stratum granulosum. We present a case of two siblings who developed continuous peeling of the palms and soles from the first year of life. This peeling was more severe on the soles than palms and on younger sibling than elder sibling. Peeling is worsened by occlusion and sweating. Sporadic cases of Acral Peeling Skin Syndrome occur in African population. There is variability in time of presentation and clinical severity even within families.

  9. Hot soup! Correlating the severity of liquid scald burns to fluid and biomedical properties.

    Science.gov (United States)

    Loller, Cameron; Buxton, Gavin A; Kerzmann, Tony L

    2016-05-01

    Burns caused by hot drinks and soups can be both debilitating and costly, especially to pediatric and geriatric patients. This research is aimed at better understanding the fluid properties that can influence the severity of skin burns. We use a standard model which combines heat transfer and biomedical equations to predict burn severity. In particular, experimental data from a physical model serves as the input to our numerical model to determine the severity of scald burns as a consequence of actual fluid flows. This technique enables us to numerically predict the heat transfer from the hot soup into the skin, without the need to numerically estimate the complex fluid mechanics and thermodynamics of the potentially highly viscous and heterogeneous soup. While the temperature of the soup is obviously is the most important fact in determining the degree of burn, we also find that more viscous fluids result in more severe burns, as the slower flowing thicker fluids remain in contact with the skin for longer. Furthermore, other factors can also increase the severity of burn such as a higher initial fluid temperature, a greater fluid thermal conductivity, or a higher thermal capacity of the fluid. Our combined experimental and numerical investigation finds that for average skin properties a very viscous fluid at 100°C, the fluid must be in contact with the skin for around 15-20s to cause second degree burns, and more than 80s to cause a third degree burn. Copyright © 2015 Elsevier Ltd and ISBI. All rights reserved.

  10. Fancy a cup of scald? - The role of hot beverage burns in paediatric burns admissions in Ireland.

    Science.gov (United States)

    McGuire, F; Hegarty, M; Jennings, P; Marsden, P; Smith, L

    2017-06-09

    Burns and scalds are preventable injuries in children that typically occur in the home. This study aimed to examine the role of hot beverage scalds in paediatric burn admissions in order to identify key target audiences for future safety strategies. Using the Hospital Inpatient Enquiry System (HIPE) a retrospective study of paediatric burn admissions in 2014 examined demographics, cause and severity of injury and location of occurrence. There were 233 paediatric discharges (age 0-18 yrs.) with a principal diagnosis of burn injury; 57% of these occurred in children under three years and 95% of these occurred in the home. Scalds caused 74% of burn injuries; hot beverages accounted for least 33% of these of which 77% were partial thickness and 73% were upper body burns. Effective hot beverage scald prevention strategies, targeted towards caregivers in the home, are required.

  11. Defeathering of broiler carcasses subjected to delayed scalding 1, 2, 4, and 8 hours after slaughter

    Science.gov (United States)

    With implementation of farm slaughter, scalding and defeathering could be delayed for a minimum of 2 to 4 h. This research evaluated the potential for delaying scalding and defeathering up to 8 h after slaughter. Following 12 h feed withdrawal broilers were cooped and transported to the pilot plan...

  12. Acral peeling skin syndrome in two East-African siblings: case report

    Directory of Open Access Journals (Sweden)

    Kiprono Samson K

    2012-03-01

    Full Text Available Abstract Background Acral peeling skin syndrome is a rare autosomal recessive genodermatosis due to a missense mutation in transglutaminase 5. The skin peeling occurs at the separation of the stratum corneum from the stratum granulosum. Case presentation We present a case of two siblings who developed continuous peeling of the palms and soles from the first year of life. This peeling was more severe on the soles than palms and on younger sibling than elder sibling. Peeling is worsened by occlusion and sweating. Conclusions Sporadic cases of Acral Peeling Skin Syndrome occur in African population. There is variability in time of presentation and clinical severity even within families.

  13. Is cupping blister harmful?-A proteomical analysis of blister fluid induced by cupping therapy and scald.

    Science.gov (United States)

    Liu, Zhidan; Chen, Chunlan; Li, Xiaoyan; Zhao, Chuang; Li, Zunyuan; Liang, Wei; Lin, Yufang

    2018-02-01

    Cupping therapy has a long history in traditional medicine especially in Asian countries. It was controversial whether cupping induced blisters are beneficial to healing effects, and the formation and content in the blisters remain unexplored. We aimed to identify and compare the molecular components of the blister fluid from the cupping therapy and the scalds to explore the necessary of inducing cupping induced blisters. Fluid sample of blisters from fifteen patients receiving cupping therapy (Cupping group) and scald burns (Scald group) were collected in this study. Proteins from the blisters were separated by two-dimensional electrophoresis (2D-gel) and further analyzed by mass spectrometry. In addition, the changes in particular proteins were confirmed by Western blotting. The protein components are significantly different between blister from cupping therapy and scalds. The immune responses, oxidative stress and metabolic related proteins (Ig lambda-2 chain C regions, Ig gamma-1 chain C region, hemopexin, prdx2, calmodulin, succinyl-CoA ligase and tetranectin) were increased, whereas the hemoglobin subunit beta was decreased in the Cupping group compared with the Scald group. Cupping induced blisters contain several proteins which relate to the activation of certain immune pathways including anti-oxidation, anti-apoptosis, tissue repairing and metabolic regulation. This proteomic analysis may indicate a significant clue to the mechanism study of cupping. Copyright © 2017 Elsevier Ltd. All rights reserved.

  14. Modeling Skin Injury from Hot Spills on Clothing

    Directory of Open Access Journals (Sweden)

    Torgrim Log

    2017-11-01

    Full Text Available The present work analyzes scald burns from hot beverages, such as coffee and tea, spilled on the lap, i.e., an incident that may occur in daily life. The Pennes bioheat equation is solved numerically for small spills wetting the clothing, i.e., the fabric prevents the spilled liquid from draining away. Temperatures are analyzed in the wetted fabric and the skin layers and the resulting skin injury is calculated based on the basal layer temperature. Parameters influencing burn severity, such as clothing thickness, liquid temperature, removal of fabric and thermal effects of post scald water cooling are analyzed. The fabric cools the water some but represents a threat since the entrapped water results in a prolonged heat supply. The liquid temperature turned out to be the most important injury parameter, where liquid temperature of about 80–85 °C seems to be a limit for developing superficial partial-thickness burns in the present minimum case, i.e., where the liquid just wets the fabric. Spilling water in excess of just wetting the fabric, more severe burns will develop at lower liquid temperatures due to the prolonged heat supply. Higher liquid temperatures will nearly instantly develop more severe burns. It is demonstrated that removal of the clothing within the first seconds after the spill may significantly reduce the scalding severity. The general advice is therefore to avoid excessive heating of beverages and, if the beverage is spilled, to quickly remove the wetted clothing. Prolonged tempered water cooling is advised to improve the healing processes.

  15. Toksisk shock syndrom. Et tilfoelde hos et barn med forbroending

    DEFF Research Database (Denmark)

    Glazowski, M J; Ostergaard, G Z; Arpi, M

    1992-01-01

    A case of the toxic shock syndrome (TSS) in a burnt (scalded) child is presented. TSS is a condition most frequently associated with menstruating women using tampons. In recent years, however, increased knowledge of the syndrome has led to an increase in the number of reported cases associated...

  16. Prevention of Recurrent Staphylococcal Skin Infections

    OpenAIRE

    Creech, C. Buddy; Al-Zubeidi, Duha N.; Fritz, Stephanie A.

    2015-01-01

    Staphylococcus aureus infections pose a significant health burden. The emergence of community-associated methicillin-resistant S. aureus has resulted in an epidemic of skin and soft tissue infections (SSTI), and many patients experience recurrent SSTI. As S. aureus colonization is associated with subsequent infection, decolonization is recommended for patients with recurrent SSTI or in settings of ongoing transmission. S. aureus infections often cluster within households and asymptomatic carr...

  17. Skin fragility syndrome in a cat with cholangiohepatitis and hepatic lipidosis.

    Science.gov (United States)

    Daniel, Alexandre G T; Lucas, Sílvia R R; Júnior, Archivaldo R; Monteiro, Paula R G; Ramos, Daniela; Pires, Carolina G; Sinhorini, Idércio L

    2010-02-01

    A case of acquired skin fragility syndrome associated with hepatic disease in a 9-year-old, spayed female, domestic shorthair cat is described. The cat was admitted to the veterinary hospital of the University of São Paulo (Brazil) with a 6-week history of vomiting, inappetence and weight loss. Remarkable signs were weakness, lethargy and profound jaundice that had been present for 10 days according to the owner. On completion of the physical examination, when the cat was gently manipulated for blood collection the thoracic limb and interscapular skin tore. Liver enzymes and bilirubin levels were all above the normal range. On histological examination of skin and liver, Masson's trichrome stain showed collagen fibre alteration and major hepatocyte abnormalities. Findings were consistent with feline skin fragility syndrome associated with cholangiohepatitis and hepatic lipidosis. Copyright 2009 ESFM and AAFP. Published by Elsevier Ltd. All rights reserved.

  18. Harvest Date Influences Superficial Scald Development in Granny Smith Apples During Long Term Storage

    OpenAIRE

    ERKAN, Mustafa; PEKMEZCİ, Mustafa

    2014-01-01

    The effects of harvest dates on superficial scald development and postharvest quality in 'Granny Smith' apples (Malus domestica Borkh.) were investigated. Apples were harvested at 15-day intervals during 2 consecutive years (2000-2001) and stored at 0 °C with 90% relative humidity for 8 months. At the end of the 8 - month storage period plus an additional 1 week at 20 °C the percentage of superficial scald was lower (24.4%) in late harvested (November 15) apples than in ap...

  19. Study of action of biological aerococcus аutosymbiont on the model of staphylococcal infection

    Directory of Open Access Journals (Sweden)

    Stepanskyi D.O.

    2017-06-01

    Full Text Available The paper presents data on the study of the antagonistic effect of aerococcus autosymbionts in experimental models of infections caused by staphylococci. To study the antagonistic action of aerococcus autosymbiont on staphylococcus, a model of chronic staphylococcal infection in white mice and rabbits was used. In staphylococcal experimental infection, aerococcus antagonistic action against staphylococcus was tested by subcutaneous injection on white mice. Aerococci survival under the skin was studied. Aerococcus autosymbionts introduced under the skin to staphylococcus in 5 hours and 3 hours after administration of the latter cause antagonist effect and inhibit the development of infiltrates. Similar results were obtained when introducing aerococcus autosymbionts in the focus of infection in 5 minutes, 5 minutes and 3 hours, 1 and 5 hours after infection. Aerococci introduced after 24 and 48 hours shortened terms of disease manifestations as compared to the control group of mice. The therapeutic effect of aerococcus autosymbionts was tested on the experimental model of burns and wounds infected with staphylococcus. In animals with wounds treated with aerococcus autostrains the number of pathogenic staphylococci was 10 times less than in the control group. According to our observations aerococcus autosymbionts showed no irritant effect when applied on the wound surface, helped its healing, sharply reduced the percentage of staphylococcus inoculation from wound secretions.

  20. A Preliminary Analysis of the Phenomenology of Skin-Picking in Prader-Willi Syndrome

    Science.gov (United States)

    Morgan, Jessica R.; Storch, Eric A.; Woods, Douglas W.; Bodzin, Danielle; Lewin, Adam B.; Murphy, Tanya K.

    2010-01-01

    To examine the nature and psychosocial correlates of skin-picking behavior in youth with Prader-Willi Syndrome (PWS). Parents of 67 youth (aged 5-19 years) with PWS were recruited to complete an internet-based survey that included measures of: skin-picking behaviors, the automatic and/or focused nature of skin-picking, severity of skin-picking…

  1. Superficial Dsg2 Expression Limits Epidermal Blister Formation Mediated by Pemphigus Foliaceus Antibodies and Exfoliative Toxins

    Directory of Open Access Journals (Sweden)

    Donna Brennan

    2010-01-01

    Full Text Available Cell-cell adhesion mediated by desmosomes is crucial for maintaining proper epidermal structure and function, as evidenced by several severe and potentially fatal skin disorders involving impairment of desmosomal proteins. Pemphigus foliaceus (PF and staphylococcal scalded skin syndrome (SSSS are subcorneal blistering diseases resulting from loss of function of the desmosomal cadherin, desmoglein 1 (Dsg1. To further study the pathomechanism of these diseases and to assess the adhesive properties of Dsg2, we employed a recently established transgenic (Tg mouse model expressing Dsg2 in the superficial epidermis. Neonatal Tg and wild type (WT mice were injected with purified ETA or PF Ig. We showed that ectopic expression of Dsg2 reduced the extent of blister formation in response to both ETA and PF Ig. In response to PF Ig, we observed either a dramatic loss or a reorganization of Dsg1-α, Dsg1-β, and, to a lesser extent, Dsg1-γ, in WT mice. The Inv-Dsg2 Tg mice showed enhanced retention of Dsg1 at the cell-cell border. Collectively, our data support the role for Dsg2 in cell adhesion and suggest that ectopic superficial expression of Dsg2 can increase membrane preservation of Dsg1 and limit epidermal blister formation mediated by PF antibodies and exfoliative toxins.

  2. Direct, Specific and Rapid Detection of Staphylococcal Proteins and Exotoxins Using a Multiplex Antibody Microarray.

    Directory of Open Access Journals (Sweden)

    Bettina Stieber

    Full Text Available S. aureus is a pathogen in humans and animals that harbors a wide variety of virulence factors and resistance genes. This bacterium can cause a wide range of mild to life-threatening diseases. In the latter case, fast diagnostic procedures are important. In routine diagnostic laboratories, several genotypic and phenotypic methods are available to identify S. aureus strains and determine their resistances. However, there is a demand for multiplex routine diagnostic tests to directly detect staphylococcal toxins and proteins.In this study, an antibody microarray based assay was established and validated for the rapid detection of staphylococcal markers and exotoxins. The following targets were included: staphylococcal protein A, penicillin binding protein 2a, alpha- and beta-hemolysins, Panton Valentine leukocidin, toxic shock syndrome toxin, enterotoxins A and B as well as staphylokinase. All were detected simultaneously within a single experiment, starting from a clonal culture on standard media. The detection of bound proteins was performed using a new fluorescence reading device for microarrays.110 reference strains and clinical isolates were analyzed using this assay, with a DNA microarray for genotypic characterization performed in parallel. The results showed a general high concordance of genotypic and phenotypic data. However, genotypic analysis found the hla gene present in all S. aureus isolates but its expression under given conditions depended on the clonal complex affiliation of the actual isolate.The multiplex antibody assay described herein allowed a rapid and reliable detection of clinically relevant staphylococcal toxins as well as resistance- and species-specific markers.

  3. Effect of DPA and 1-MCP on chemical compounds related to superficial scald of Granny Smith apples

    Energy Technology Data Exchange (ETDEWEB)

    Moggia, C.; Moya-Leon, M. A.; Pereira, M.; Yuri, J. A.; Lobos, G. A.

    2010-07-01

    Research was carried out to study the mode of action of diphenylamine (DPA) and 1-methylcyclopropene (1-MCP), on control of superficial scald of Granny Smith apples (Malus domestica Borkh.), and its relation with chemical compounds. Fruit was harvested from a commercial orchard in Chile, 182 and 189 days after full bloom and received the following treatments: DPA (2,000 ppm); 1-MCP (1.2 ppm) and control (no treatment). All fruit was stored for 4 or 6 months at 0 degree centigrade. A completely randomized factorial design was used (2 harvest dates by 3 post harvest treatments). Monthly measurements were made on maturity indices, ethylene production rate (EPR), scald related compounds [a-farnesene (AF), conjugated trienes (CT), total anti-oxidants (AO)], and cell membrane stability. Following 4 and 6 months of storage, plus 7 days at 20 degree centigrade, scald was evaluated. After 6 months, DPA-treated fruit, from both harvests, showed similar firmness, EPR and AO, compared to the control. However, AF and CT were lower, and cell membrane stability higher. Conversely, 1-MCP-treated fruit showed a noticeable EPR suppression and AF inhibition, along with higher firmness, lower CT and AO, compared to the control and DPA. Furthermore, cell membrane stability was superior to that of the control and similar to that of the DPA. Treated fruit (DPA and 1-MCP) showed an important reduction in scald compared to the control. The effect of 1-MCP on the investigated compounds and the reduction in scald, confirms that ethylene plays a major role on its development. (Author) 50 refs.

  4. Acral peeling skin syndrome: a case of two brothers.

    Science.gov (United States)

    Wakade, Oojwala; Adams, Beth; Shwayder, Tor

    2009-01-01

    We report two brothers of Middle Eastern descent with consanguineous parents who present with numerous fragile, flaccid blisters on the hands and feet. In addition to spontaneous peeling, they can manually peel skin from acral areas without pain. The symptoms worsen with warm temperatures, excessive water exposure, and perspiration. Two biopsies from flaccid blisters on the feet were taken from the older brother, which revealed cleavage at the level of the stratum corneum. A diagnosis of acral peeling skin syndrome was made.

  5. Irradiation seed treatment reduces scald, common root rot and increases phosphorus absorption of barley

    International Nuclear Information System (INIS)

    Arabi, M.I.E.; Jawhar, M.

    2003-01-01

    The effect of low doses of gamma irradiation on severity of barley to scald and common root rot diseases, and phosphorus absorption was studied seeds were exposed to doses of 0, 10, 15, 20, 30, 40 and 50 Gy. A stimulatory effect was observed at irradiation doses of 30 and 40 Gy, which decreased the severity of barley to scald by 34% and 31% respectively. On the other hand, doses 20 and 30 Gy decreased the severity to CRR by 54% and 49% respectively, whereas, phosphorus absorption was significantly increased at doses of 15 and 20 Gy

  6. Ectodermal dysplasia-skin fragility syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    Subhash Kashyap

    2015-01-01

    Full Text Available Ectodermal dysplasia/skin fragility syndrome (ED-SFS is a newly described autosomal recessive disorder characterized by skin fragility and blistering, palmoplantar keratoderma, abnormal hair growth, nail dystrophy, and occasionally defective sweating. It results from mutations in the PKP1 gene encoding plakophilin 1 (PKP1, which is an important component of stratifying epithelial desmosomes and a nuclear component of many cell types. Only 12 cases of this rare genodermatosis have been reported so far. We present an unusual case of ED-SFS in a 12-year boy who was normal at birth but subsequently developed skin fragility, hair and nail deformities, abnormal dentition, palmoplantar keratoderma, and abnormal sweating but no systemic abnormality.

  7. A 7-year-old with indurated skin and unilateral progressive joint immobility: A case of stiff skin syndrome.

    Science.gov (United States)

    Ogunmakin, Kehinde; Vangipuram, Ramya; Sturgeon, Ashley; Shimizu, Ikue

    2015-09-17

    Stiff skin syndrome is a rare sclerotic condition that presents during infancy or early childhood. It has an insidious chronic course and may lead to significant co-morbidity and reduced quality of life. Often, affected individuals experience impaired ambulation and immobilization related to joint involvement. Clinically, it may resemble other sclerotic diseases, so histopathological evaluation is necessary to establish a diagnosis. As it is a condition with limited treatment options, prompt diagnosis and early initiation of physical therapy is crucial to prevent joint restriction and maintain quality of life. We describe a case of a 7-year-old with stiff skin syndrome, and review the literature to discuss the clinical presentation, histological findings, and management of this condition.

  8. An unsuspected cause of meal-time morbidity: instant noodle scald burns.

    Science.gov (United States)

    Koltz, Peter F; Wasicek, Philip; Mays, Chester; Bell, Derek E

    2013-01-01

    Observational analysis revealed a concerning frequency of scald burns secondary to instant noodles. A literature review reveals studies with small sample sizes of pediatric populations and analysis of container engineering. The adult cohort, treatments, and short-term outcomes have been neglected. Considering these deficiencies, we reviewed our institution's experience with burns secondary to instant noodles. Patient encounters due to instant noodle burns from January 1, 2007, through May 15, 2011, were reviewed. Demographics, burn characteristics, treatment, length of stay, number of operative interventions, and complications were analyzed. Eight hundred fifty-two patients were seen (460 were admitted) for scald burns of all pathogenesis. Of these, 121 (14%) were seen for burns secondary to noodles (63 men and 58 women). Of these, 48 were older than age 4 (group 1), and 73 were younger than age 4 (group 2). TBSA was 2.34 in group 1 and 1.64 in group 2 (P = .04). The most commonly burned areas in group 1 were extremities (n = 43) and in group 2 were chest (n = 32) and extremities (n = 31). Seven patients in group 1 and two patients in group 2 required operative intervention. Length of stay in groups 1 and 2 were 3.5 and 6 days, respectively. Noodle scald burns cause morbidity at all ages. Pediatric burns due to noodles are frequently managed conservatively but more often necessitate inpatient treatment. The nonpediatric population has larger TBSA and requires more frequent operative intervention. The morbidity of noodle burns is significant. Increased public education and container re-engineering is warranted.

  9. Nematode Infections Are Risk Factors for Staphylococcal Infection in Children

    Directory of Open Access Journals (Sweden)

    Sandra F Moreira-Silva

    2002-04-01

    Full Text Available Nematode infection may be a risk factor for pyogenic liver abscess in children and we hypothesized that the immunomodulation induced by those parasites would be a risk factor for any staphylococcal infection in children. The present study was designed to compare, within the same hospital, the frequency of intestinal nematodes and Toxocara infection in children with and without staphylococcal infections. From October 1997 to February 1998, 80 children with staphylococcal infection and 110 children with other diseases were submitted to fecal examination, serology for Toxocara sp., evaluation of plasma immunoglobulin levels, and eosinophil counts. Mean age, gender distribution, birthplace, and socioeconomic conditions did not differ significantly between the two groups. Frequency of intestinal nematodes and positive serology for Toxocara, were remarkably higher in children with staphylococcal infections than in the non-staphylococcal group. There was a significant correlation between intestinal nematodes or Toxocara infection and staphylococcal infection in children, reinforced by higher eosinophil counts and higher IgE levels in these children than in the control group. One possible explanation for this association would be the enhancement of bacterial infection by the immunomodulation induced by helminth infections, due to strong activation of the Th2 subset of lymphocytes by antigens from larvae and adult worms.

  10. Characterization of staphylococci and their role in pathology of children

    Directory of Open Access Journals (Sweden)

    M. A. Harуfulina

    2014-03-01

    Full Text Available The problem of human staphylococcal infection in the world has been considered. It is known that there are 27 species of staphylococci, 14 of them colonize the skin and mucous membranes of humans, and 3 of them may cause diseases. They are Staphylococcus aureus, S. epidermidis and S. saprophyticus. Staphylococci are causative agents of the large part of community-acquired and nosocomial bacteremia, pneumonia, infections of skin and soft tissues, bones and joints. Of all cases of nosocomial infections, S. aureus accounts for 31%. During the identification of 65 strains of bacteria isolated from people with respiratory tract lesions, it was found that 58.5% of cases were connected with presence of staphylococci. 86.8% of isolates identified belong to species S. aureus and 13.2% to S. saprophyticus. Study of pathogenicity factors manifestation revealed that all strains identified as S. aureus had plasmocoagulase; lipase and lecitinase were simultaneously detected in 78.3% of them, among S. saprophyticus strains in 20%. Presence of haemolysins was determined for 82.1% of strains of staphylococci. Ability to film formation was detected in 21 (63.6% strains of S. aureus and 2 (40% of S. saprophyticus strains. Given high frequency of carriers of staphylococci among healthy individuals, they should be considered as potentially pathogenic microorganisms and opportunistic pathogens. Due to their pathogenicity factors (adhesins, capsule, cell wall components, enzymes and toxins, staphylococci may cause diseases such as sepsis, meningitis, peritonitis, endocarditis, pneumonia, tonsillitis, and in young children they may result in serious Ritter illness (“babies scalded syndrome”, foodstuff infection and staphylococcal enterocolitis, in lactating women – mastitis, toxic shock syndrome. To solve the problem of staphylococcal infection prevention, it is necessary to use the following prophylaxis methods: to avoid decrease in immunity, to observe the

  11. Novel TGM5 mutations in acral peeling skin syndrome

    NARCIS (Netherlands)

    van der Velden, Jaap J. A. J.; van Geel, Michel; Nellen, Ruud G. L.; Jonkman, Marcel F.; McGrath, John A.; Nanda, Arti; Sprecher, Eli; van Steensel, Maurice A. M.; McLean, W. H. Irwin; Cassidy, Andrew J.

    Acral peeling skin syndrome (APSS, MIM #609796) is a rare autosomal recessive disorder characterized by superficial exfoliation and blistering of the volar and dorsal aspects of hands and feet. The level of separation is at the junction of the stratum granulosum and stratum corneum. APSS is caused

  12. Prevention of Recurrent Staphylococcal Skin Infections

    Science.gov (United States)

    Creech, C. Buddy; Al-Zubeidi, Duha N.; Fritz, Stephanie A.

    2015-01-01

    Synopsis Staphylococcus aureus infections pose a significant health burden. The emergence of community-associated methicillin-resistant S. aureus has resulted in an epidemic of skin and soft tissue infections (SSTI), and many patients experience recurrent SSTI. As S. aureus colonization is associated with subsequent infection, decolonization is recommended for patients with recurrent SSTI or in settings of ongoing transmission. S. aureus infections often cluster within households and asymptomatic carriers serve as reservoirs for transmission; therefore, a household approach to decolonization is more effective than measures performed by individuals alone. Other factors, such as environmental surface contamination, may also be considered. Novel strategies for the prevention of recurrent SSTI are needed. PMID:26311356

  13. Scalds among children in Kuwait.

    Science.gov (United States)

    Bang, R L; Ebrahim, M K; Sharma, P N

    1997-01-01

    In a prospective study of 560 children treated for burns as in-patients over a period of four-and-a-half years in specialized hospitals responsible for the majority of burn cases in Kuwait, 388 patients (69%) had sustained scalds. The mean age of these 388 children, between 0 to 12 years, was 3.02 +/- 2.08, and male to female ratio 1.5 to 1. They were categorized into three age groups, first, up to 1 year comprised 17.5% cases, who were solely dependent on parents or childminder; second 2 to 5 years of age, who were inquisitive, independent, pre-school children, and constituted the majority of cases (73%), and third 6 to 12 years who were 9.5% school children. The pre-school children (2-5 years) thus formed a highly vulnerable group in the country. Accidents (99.4%) occurred at home and the kitchen being the commonest place. The hot water from pan and pots in the kitchen was the most common etiologic factor in 229 cases (59%), followed by tea/coffee 20.7%, soup 9.0%, hot oil 6.7%, and milk 4.6% patients. The most common circumstance was the child upsetting the pan of hot fluid in the kitchen. The mean total body surface areas of second and third degree burns were 14.21 +/- 9.66 (range 1 to 60%). The average length of stay in the hospital was 16.90 +/- 15.74 days, varying from one to 109 days. Thirty-nine children were ill prior to burn, and the commonest disease was respiratory tract infection. Three patients (0.8%) with 3rd degree burns were treated with primary excision and grafting, and 137 (35.3%) needed secondary skin grafting for residual burn wounds. Four patients (1%) died, one due to burn shock, two due to septicemia and one due to multiorgan failure. There is need for general awareness through public education, which may lead to the prevention of significant number of such accidents.

  14. Constitutional mismatch repair-deficiency syndrome presenting as colonic adenomatous polyposis: clues from the skin.

    Science.gov (United States)

    Jasperson, K W; Samowitz, W S; Burt, R W

    2011-10-01

    Constitutional mismatch repair-deficiency (CMMR-D) syndrome is an autosomal recessive condition characterized by hematologic malignancies, brain tumors, Lynch syndrome-associated cancers and skin manifestations reminiscent of neurofibromatosis type 1 (NF1). In contrast to Lynch syndrome, CMMR-D syndrome is exceptionally rare, onset typically occurs in infancy or early childhood and, as described in this report, may also present with colonic polyposis suggestive of attenuated familial adenomatous polyposis (AFAP) or MUTYH associated polyposis (MAP). Here we describe two sisters with CMMR-D syndrome due to germline bi-allelic MSH6 mutations. Both sisters are without cancer, are older than typical for this condition, have NF1 associated features and a colonic phenotype suspicious for an attenuated polyposis syndrome. This report highlights the role of skin examinations in leading to an underlying genetic diagnosis in individuals with colonic adenomatous polyposis, but without mutations associated with AFAP or MAP. © 2010 John Wiley & Sons A/S.

  15. Epidemiology and outcome analysis of scalds in children caused by "guo lian kang": an 11-year review in a burn center in China.

    Science.gov (United States)

    Zhu, Liqiang; Zhang, Hong; Shi, Fusheng; Yi, Dong; Zhu, Guangjun

    2015-03-01

    The objective of this study was to investigate the epidemiological characteristics of scalds induced by "guo lian kang" (literally "stove connected to bed") in children. The demographic, burn features, outcome, and other information of 0-14-year-old children with "guo lian kang"-induced scalds admitted to the burn treatment center of the 322 Hospital of the People's Liberation Army in Datong from 30 September 2001 to 31 December 2011 were analyzed. Among the 1993 children clearly documented with scalds due to hot liquid in vessels, 795 (39.89%) had burns due to "guo lian kang." The proportion of the pediatric burns caused by "guo lian kang" was 55.56% in 2002 and 37.30% in 2011, with a decreasing trend. One-year-old children accounted for the highest proportion of children with burns due to "guo lian kang" (male:female=1.54:1). Scalding mostly occurred from October to May; the most common times were around 8:00 AM, 11:00 AM, and 7:00 PM. Most scalds occurred on the upper limbs, 90.22% of which were dermal burns. The median burn area was 11% of the total body surface area. The burn agent was mainly hot water in pots. Scalding mostly occurred when the children accidentally sat on, or fell or stepped into the pot while playing. Among the children with "guo lian kang"-induced scalds, up to 42.26% of the children did not receive any treatment before hospital admission, and 18.86% of the children were admitted to the hospital ≥6h after the occurrence of burns. In addition, 74.97% of the children's families can afford the medical expenses, while 5.28% abandoned treatment because of financial difficulties. There were no significant differences between children with scalds caused by "guo lian kang" and scalds caused due to other reasons considering the age and sex. Compared to scalds due to other reasons, children with "guo lian kang"-induced scalds had greater burn area and depth, shock incidence, surgery rate, blood transfusion rate, length of hospital stay, and

  16. Identification of QTLs for powdery mildew and scald resistance in barley

    NARCIS (Netherlands)

    Shtaya, M.J.Y.; Marcel, T.C.; Sillero, J.C.; Niks, R.E.; Rubiales, D.

    2006-01-01

    A population of 103 recombinant inbred lines (RILs, F9-derived lines) developed from the two-row spring barley cross L94 × `Vada¿ was evaluated under field conditions for resistance against powdery mildew (Blumeria graminis f.sp. hordei) and scald (Rhynchosporium secalis). Apart from the major

  17. Staphylococcus aureus α-toxin modulates skin host response to viral infection.

    Science.gov (United States)

    Bin, Lianghua; Kim, Byung Eui; Brauweiler, Anne; Goleva, Elena; Streib, Joanne; Ji, Yinduo; Schlievert, Patrick M; Leung, Donald Y M

    2012-09-01

    Patients with atopic dermatitis (AD) with a history of eczema herpeticum have increased staphylococcal colonization and infections. However, whether Staphylococcus aureus alters the outcome of skin viral infection has not been determined. We investigated whether S aureus toxins modulated host response to herpes simplex virus (HSV) 1 and vaccinia virus (VV) infections in normal human keratinocytes (NHKs) and in murine infection models. NHKs were treated with S aureus toxins before incubation of viruses. BALB/c mice were inoculated with S aureus 2 days before VV scarification. Viral loads of HSV-1 and VV were evaluated by using real-time PCR, a viral plaque-forming assay, and immunofluorescence staining. Small interfering RNA duplexes were used to knockdown the gene expression of the cellular receptor of α-toxin, a disintegrin and metalloprotease 10 (ADAM10). ADAM10 protein and α-toxin heptamers were detected by using Western blot assays. We demonstrate that sublytic staphylococcal α-toxin increases viral loads of HSV-1 and VV in NHKs. Furthermore, we demonstrate in vivo that the VV load is significantly greater (P skin inoculated with an α-toxin-producing S aureus strain compared with murine skin inoculated with the isogenic α-toxin-deleted strain. The viral enhancing effect of α-toxin is mediated by ADAM10 and is associated with its pore-forming property. Moreover, we demonstrate that α-toxin promotes viral entry in NHKs. The current study introduces the novel concept that staphylococcal α-toxin promotes viral skin infection and provides a mechanism by which S aureus infection might predispose the host toward disseminated viral infections. Copyright © 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.

  18. [Clinical application of moving cupping therapy based on skin reaction observation and syndrome differentiation].

    Science.gov (United States)

    Deng, Xiao-Lan; Chen, Bo; Chen, Ze-Lin

    2014-12-01

    The diagnostic evidence on clinical diseases and theoretic basis of moving cupping therapy were ex- plored in the paper. By the observation of the local reaction, such as skin appearance and color, the affected location, duration of sickness and nature of disease were judged. Different moving cupping methods were selected for different disorders. It was discovered that the property of syndromes should be recognized by the palpation on skin and muscle in the moving cupping therapy so that the pathogenesis and treating principle could be carefully determined. The moving cupping therapy is the important component of body surface therapy. Skin reaction observation and syndrome differentiation is the essential guidance of the moving cupping therapy.

  19. Generalized peeling skin syndrome: Case report and review of the literature.

    Science.gov (United States)

    Kharfi, Monia; Khaled, Aida; Ammar, Donia; Ezzine, Nadia; El Fekih, Nadia; Fazaa, Becima; Jaafoura, Hiabib; Kamoun, Mohamed Ridha

    2010-03-15

    Peeling skin syndrome (PSS) is a rare form of ichthyosis with a probable autosomal recessive inheritance that exhibits superficial, painless, continual, or seasonal cutaneous exfoliation. The syndrome generally appears at birth or in infancy. We report a case of generalized PSS and provide a literature review. A 34-year-old woman reported a lifelong history of generalized and painless peeling of the skin that worsened in summer. Her parents were third degree cousins. Her twin sister and her two cousins presented with the same condition. Physical examination showed widespread superficial sheets of variable size that could be easily removed without bleeding or pain. No underlying erythema was noted. Otherwise, the patient was in good health. Histological findings showed an epidermal cleavage within the stratum corneum. The generalized form of PSS is classified into 3 types, A, B, and C, according to the classification system of Traupe and Mevorah. We have tried to classify the cases of generalized PSS already reported in the literature into one of these three types. Thirteen reported cases probably presented PSS-type A. Sixteen patients are best described as PSS-type B. Two patients exhibit PSS-type C. Fifteen reported patients had an acral form of peeling skin syndrome. The classification of the eleven remaining patients was difficult to determine. Our patient presented clinical and histological features of generalized PSS-type A.

  20. Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN): could retinoids play a causative role?

    Science.gov (United States)

    Mawson, Anthony R; Eriator, Ike; Karre, Sridhar

    2015-01-12

    Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are overlapping manifestations on a spectrum of acute drug-induced conditions associated with severe blistering, skin peeling, and multi-organ damage. TEN is an eruption resembling severe scalding, with ≥30% skin detachment. SJS is a mild form of TEN, characterized histologically by epidermal keratinocyte apoptosis with dermo-epidermal separation and extensive small blisters with <10% body surface skin detachment. The syndrome can be induced by numerous medications and typically occurs 1-4 weeks after the initiation of therapy. Granulysin is found in the lesions of patients with SJS/TEN and plays a significant pathogenic role in the condition, but the overall mechanisms linking medications, granulysin, and disease manifestations remain obscure. This paper reviews evidence suggesting that the different medications implicated in SJS/TEN have the common property of interacting and synergizing with endogenous retinoids (vitamin A and its congeners), in many instances causing the latter to accumulate in and damage the liver, the main storage organ for vitamin A. It is hypothesized that liver damage leads to the spillage of toxic retinoid compounds into the circulation, resulting in an endogenous form of hypervitaminosis A and cytotoxicity with widespread apoptosis, mediated by granulysin and recognized as SJS/TEN. Subject to testing, the model suggests that symptom worsening could be arrested at onset by lowering the concentration of circulating retinoids and/or granulysin via phlebotomy or plasmapheresis or by pharmacological measures to limit their expression.

  1. Hyper Ig E syndrome (Job syndrome, HIES) – radiological images of pulmonary complications on the basis of three cases

    International Nuclear Information System (INIS)

    Jończyk-Potoczna, Katarzyna; Szczawińska-Popłonyk, Aleksandra; Warzywoda, Małgorzata; Bręborowicz, Anna; Pawlak, Bogdan

    2012-01-01

    Hyperimmunoglobulinemia E syndrome (hyper-IgE syndrome, Job syndrome, HIES) is a complex immune deficiency with multiorgan clinical manifestations and diverse genetic background. The clinical triad of symptoms observed in approximately 75% of patients with HIES includes: recurrent abscesses of staphylococcal etiology, recurrent respiratory infections and elevated immunoglobulin E in serum. The paper discusses three cases of female patients presenting typical pulmonary complications of the hyper-Ig E syndrome. In the first case, the development of aspergilloma in a postinflamatory cyst was observed, in the other one, pneumonia with pleural effusion, and as a consequence of inflammatory infiltrations – fibrotic changes, giving rise to lobectomy, while in the last of these cases, the course of lung disease was complicated by formation of staphylococcal abscess. In one of the girls, bronchiectasis appeared at follow-up. Complications of pulmonary infections are the most common causes of death in hyper-Ig E syndrome. Late diagnosis significantly worsens the respiratory function and reduces the chance for normal development of a child. Introduction of comprehensive treatment, including prophylaxis, decreases the recurrences. Therefore, the important role is attributed to the radiologist in the multidisciplinary care of patients with this syndrome

  2. Hypercalciuria in a child with acral peeling skin syndrome: a case report.

    Science.gov (United States)

    Gorczyca, Daiva; Węgłowska, Jolanta; Prescha, Anna; Woźniak, Zdzisław; Nesteruk, Dominika; Wertheim-Tysarowska, Katarzyna; Śmigiel, Robert

    2015-01-01

    We present a case of 3-year-old Caucasian boy who developed monthly cyclic attacks of skin peeling of the palms and soles over 1.5 years. The skin peeling was associated with hypercalciuria. No mutation was present in TGM5 and CSTA genes, but the typical clinical picture and the biopsy from flaccid blisters on the feet confirmed the acral peeling skin syndrome (APSS). The possible associations of rare genetic disorders and metabolic conditions in the course of APSS need to be investigated.

  3. Adenosine triphosphate hydrolysis reduces neutrophil infiltration and necrosis in partial-thickness scald burns in mice.

    Science.gov (United States)

    Bayliss, Jill; Delarosa, Sara; Wu, Jianfeng; Peterson, Jonathan R; Eboda, Oluwatobi N; Su, Grace L; Hemmila, Mark; Krebsbach, Paul H; Cederna, Paul S; Wang, Stewart C; Xi, Chuanwu; Levi, Benjamin

    2014-01-01

    Extracellular adenosine triphosphate (ATP), present in thermally injured tissue, modulates the inflammatory response and causes significant tissue damage. The authors hypothesize that neutrophil infiltration and ensuing tissue necrosis would be mitigated by removing ATP-dependent signaling at the burn site. Mice were subjected to 30% TBSA partial-thickness scald burn by dorsal skin immersion in a water bath at 60 or 20°C (nonburn controls). In the treatment arm, an ATP hydrolyzing enzyme, apyrase, was applied directly to the site immediately after injury. Skin was harvested after 24 hours and 5 days for hematoxylin and eosin stain, elastase, and Ki-67 staining. Tumor necrosis factor (TNF)-α and interferon (IFN)-β expression were measured through quantitative real-time polymerase chain reaction. At 24 hours, the amount of neutrophil infiltration was different between the burn and burn + apyrase groups (P burn group at 24 hours and 5 days. TNF-α and IFN-β expression at 24 hours in the apyrase group was lower than in the burn group (P burn site allays the neutrophil response to thermal injury and reduces tissue necrosis. This decrease in inflammation and tissue necrosis is at least partially because of TNF-α and IFN-β signaling. Apyrase could be used as topical inflammatory regulators to quell the injury caused by inflammation.

  4. Skin fragility syndrome in a cat with feline infectious peritonitis and hepatic lipidosis.

    Science.gov (United States)

    Trotman, Tara K; Mauldin, Elizabeth; Hoffmann, Vickie; Del Piero, Fabio; Hess, Rebecka S

    2007-10-01

    A 6-year-old spayed female domestic shorthair cat with a 3-week history of inappetence, weight loss, and hiding was examined. A palpable abdominal fluid wave, dehydration, and a small tear on the left flank were noted during initial examination. When the cat was gently restrained for blood sampling, the skin on the dorsal neck tore, leaving a 15 cm x 7 cm flap of skin. Clinicopathological abnormalities included nonregenerative anaemia, hypoalbuminaemia, increased globulin concentration, and mildly elevated aspartate aminotransferase and alkaline phosphatase activities. Abdominal fluid was viscous and had a total protein of 5.3 g dL(-1) with 316 cells microL(-1), consistent with a modified transudate. Cytology of the abdominal fluid revealed 86% nondegenerate neutrophils, 13% macrophages, and 1% small lymphocytes. Histopathological evaluation and indirect immunohistochemistry confirmed a diagnosis of feline infectious peritonitis, hepatic lipidosis and feline skin fragility syndrome. Feline skin fragility syndrome has not previously been reported in association with feline infectious peritonitis (FIP). Its inclusion as a clinical sign associated with FIP may facilitate a diagnosis.

  5. Acral peeling skin syndrome in two East-African siblings: case report

    OpenAIRE

    Kiprono, Samson K; Chaula, Baraka M; Naafs, Bernard; Masenga, John E

    2012-01-01

    Abstract Background Acral peeling skin syndrome is a rare autosomal recessive genodermatosis due to a missense mutation in transglutaminase 5. The skin peeling occurs at the separation of the stratum corneum from the stratum granulosum. Case presentation We present a case of two siblings who developed continuous peeling of the palms and soles from the first year of life. This peeling was more severe on the soles than palms and on younger sibling than elder sibling. Peeling is worsened by occl...

  6. Acral peeling skin syndrome associated with a novel CSTA gene mutation.

    Science.gov (United States)

    Muttardi, K; Nitoiu, D; Kelsell, D P; O'Toole, E A; Batta, K

    2016-06-01

    Acral peeling skin syndrome (APSS) is a rare autosomal recessive condition, characterized by asymptomatic peeling of the skin of the hands and feet, often linked to mutations in the gene TGM5. However, more recently recessive loss of function mutations in CSTA, encoding cystatin A, have been linked with APSS and exfoliative ichthyosis. We describe the clinical features in two sisters with APSS, associated with a novel large homozygous deletion encompassing exon 1 of CSTA. © 2015 British Association of Dermatologists.

  7. Production of staphylococcal enterotoxin A in cream-filled cake.

    Science.gov (United States)

    Anunciaçao, L L; Linardi, W R; do Carmo, L S; Bergdoll, M S

    1995-07-01

    Cakes were baked with normal ingredients and filled with cream, inoculated with different size enterotoxigenic-staphylococcal inocula. Samples of the cakes were incubated at room temperature and put in the refrigerator. Samples of cake and filling were taken at different times and analyzed for staphylococcal count and presence of enterotoxin. The smaller the inoculum, the longer the time required for sufficient growth (10(6)) to occur for production of detectable enterotoxin. Enterotoxin added to the cake dough before baking (210 degrees C, 45 min) did not survive the baking. The presence of enterotoxin in the contaminated cream filling indicated this as the cause of staphylococcal food poisoning from cream-filled cakes. Refrigeration of the cakes prevented the growth of the staphylococci.

  8. Treatment of Chronic Skin-Picking in an Adolescent With Asperger Syndrome and Borderline Intellectual Disability

    NARCIS (Netherlands)

    Lang, R.B.; Didden, H.C.M.; Sigafoos, J.; Rispoli, M.; Regester, A.; Lancioni, G.E.

    2009-01-01

    We present the case of a 17-year-old girl with Asperger syndrome and borderline intellectual disability with a 5-year history of chronic skin-picking. Our intervention approach included an initial functional assessment to identify variables maintaining skin-picking, followed by evaluation of a

  9. Toxic epidermal necrolysis and Stevens-Johnson syndrome

    Directory of Open Access Journals (Sweden)

    French Lars E

    2010-12-01

    Full Text Available Abstract Toxic epidermal necrolysis (TEN and Stevens Johnson Syndrome (SJS are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP, disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS. Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis

  10. METHICILLIN RESISTANCE IN STAPHYLOCOCCAL ISOLATES ...

    African Journals Online (AJOL)

    The study assessed the importance of Staphylococcus aureus as a urinary pathogen and the incidence of multidrug resistant (MDR), methicillin-resistant Staphylococcus aureus (MRSA). A total of 86 staphylococcal isolates made up of 50 clinical isolates from urine samples submitted to the Medical Microbiology Laboratory ...

  11. Effect of atopic skin stressors on natural moisturizing factors and cytokines in healthy adult epidermis

    DEFF Research Database (Denmark)

    Engebretsen, K A; Kezic, S; Jakasa, I

    2018-01-01

    : To explore the effect of selected exogenous skin stressors on NMF and skin cytokines levels in healthy adult epidermis. MATERIAL AND METHODS: 40 healthy volunteers (18-49 years) were exposed to hard, soft, and chlorinated water, 0.5% SLS, house dust mite, cat allergen, staphylococcal enterotoxin B (SEB...... of various skin cytokines in healthy individuals. Our data highlight environmental factors that might play a role in AD pathophysiology, but needs confirmation in AD patients. This article is protected by copyright. All rights reserved....

  12. The Histological and Immunohistochemical Features of the Skin Lesions in CANDLE Syndrome

    Science.gov (United States)

    Torrelo, Antonio; Colmenero, Isabel; Requena, Luis; Paller, Amy S.; Ramot, Yuval; Lee, Chyi-Chia Richard; Vera, Angel; Zlotogorski, Abraham; Goldbach-Mansky, Raphaela; Kutzner, Heinz

    2015-01-01

    Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a newly characterized autoinflammatory disorder, caused by mutations in PSMB8. It is characterized by early-onset fevers, accompanied by a widespread, violaceous and often annular, cutaneous eruption. While the exact pathogenesis of this syndrome is still obscure, it is postulated that the inflammatory disease manifestations stem from excess secretion of interferons. Based on preliminary blood cytokine and gene expression studies, the signature seems to come mostly from type I interferons, which are proposed to lead to the recruitment of immature myeloid cells into the dermis and subcutis. In this study, we systematically analyzed skin biopsies from 6 CANDLE syndrome patients by routine histopathology and immunohistochemistry methods. Skin lesions showed the presence of extensive mixed dermal and subcutaneous inflammatory infiltrate, composed of mononuclear cells, atypical myeloid cells, neutrophils, eosinophils and some mature lymphocytes. Positive LEDER and myeloperoxidase staining supported the presence of myeloid cells. Positive CD68/PMG1 and CD163 staining confirmed the existence of histiocytes and monocytic macrophages in the inflammatory infiltrate. CD123 staining was positive, demonstrating the presence of plasmacytoid dendritic cells. Uncovering the unique histopathologic and immunohistochemical features of CANDLE syndrome provides tools for rapid and specific diagnosis of this disorder as well as further insight into the pathogenesis of this severe, life-threatening condition. PMID:26091509

  13. 'Diffuse skin browning' in 1-MCP-treated apples: etiology and systems of control.

    Science.gov (United States)

    Larrigaudière, Christian; Vilaplana, Rosa; Recasens, Inmaculada; Soria, Yolanda; Dupille, Eve

    2010-11-01

    'Diffuse skin browning' (DSB) is a physiological disorder that affects Golden Delicious apples treated with 1-methylcyclopropene (1-MCP). Although a very high incidence is found, very little is known about the etiology of this disorder. This study aims to provide an understanding of the causes of this disorder and prevent it. A very high incidence of DSB was found in 1-MCP-treated apples independent of the location of the orchard. Similar to superficial scald, harvest maturity determines the DSB incidence, with the more mature fruit being less sensitive. The 1-MCP dose (156 nL L(-1) or 625 nL L(-1)) and the temperature at which the 1-MCP treatment was applied (0.5 or 20 °C) did not affect the incidence of DSB. Diphenylamine (DPA) treatment did not prevent DSB, contrary to superficial scald. Additionally, controlled atmosphere storage only partially reduced the incidence of DSB, whereas progressive cooling strategies completely inhibited DSB occurrence. A direct correlation was found between the sensitivity of the Golden Delicious clone to russeting and its sensitivity to develop DSB during storage. Our results indicated that DSB and superficial scald are two different disorders involving different oxidative processes. DSB can be prevented by progressive cooling and selection of russeting-resistant clones. 2010 Society of Chemical Industry

  14. Functional Assessment and Behavioral Treatment of Skin Picking in a Teenage Girl With Prader-Willi Syndrome

    NARCIS (Netherlands)

    Radstaake, M.; Didden, H.C.M.; Bolio, M.M.L.; Lang, R.B.; Lancioni, G.E.; Curfs, L.M.G.

    2011-01-01

    Skin picking is common in individuals with Prader-Willi Syndrome (PWS) but few treatment studies exist. This study reports the successful functional behavioral assessment (FBA) and treatment of skin picking in a 16-year-old female with PWS. A treatment package based on FBA results consisted of

  15. Prevalence and Characteristics of Nonblanching, Palpable Skin Lesions With a Linear Pattern in Children With Henoch-Schönlein Syndrome.

    Science.gov (United States)

    Milani, Gregorio P; Lava, Sebastiano A G; Ramelli, Vera; Bianchetti, Mario G

    2017-11-01

    Linear nonblanching skin lesions are thought to occur very rarely in patients with Henoch-Schönlein syndrome. To examine the prevalence and characteristics of linear nonblanching skin lesions in children with Henoch-Schönlein syndrome. A prospective case series was conducted at the ambulatory practice of a hospitalist between January 1, 2010, and December 31, 2015, among 31 consecutive children with Henoch-Schönlein syndrome. Thirty-one consecutive children affected with Henoch-Schönlein syndrome who were from 3.0 to 12.0 years of age (median age, 6.2 years). Children with Henoch-Schönlein syndrome underwent a careful, structured skin examination established in advance with emphasis on the presence of palpable lesions with a linear pattern. Among the 31 children in the study (12 girls and 19 boys; median age, 6.2 years [range, 3.0-12.0 years]), 8 (26%) had linear lesions on the legs, groin, waistline, wrists, or forearms. Patients with or without linear lesions did not differ significantly with respect to sex, age, and cutaneous, abdominal, articular, or renal involvement. This study illustrates the prevalence and characteristics of linear skin lesions in patients with Henoch-Schönlein syndrome. Patients with symptoms suggestive of this vasculitis should be evaluated for the presence of nonblanching, palpable lesions with a linear pattern.

  16. Michelin tire baby syndrome: a review of the literature and a proposal for diagnostic criteria with adoption of the name circumferential skin folds syndrome.

    Science.gov (United States)

    Rothman, Ilene L

    2014-01-01

    The term Michelin tire baby (MTB), named for the cartoon mascot of the Michelin Tire Company, has been used to describe babies with multiple symmetric circumferential rings of folded skin. In those reported with this phenotype who had skin biopsies, pathology has shown nevus lipomatosis, smooth muscle hamartoma, degenerative collagen, and scarring. Others did not undergo biopsy or had normal skin. Many individuals with the MTB phenotype have had a variety of other congenital anomalies. I review the literature on MTB and the history of the designation Michelin tire baby Syndrome (MTBS). Because the term MTBS has been poorly defined or not defined at all, I propose strict criteria for diagnosis. In doing so, it is recommended that the syndrome be renamed to avoid further confusion. © 2014 Wiley Periodicals, Inc.

  17. Hyperimmunoglobulin E syndrome: Genetics, immunopathogenesis, clinical findings, and treatment modalities

    Directory of Open Access Journals (Sweden)

    Hassan Hashemi

    2017-01-01

    Full Text Available The hyperimmunoglobulin E syndromes (HIESs are very rare immunodeficiency syndromes with multisystem involvement, including immune system, skeleton, connective tissue, and dentition. HIES are characterized by the classic triad of high serum levels of immunoglobulin E (IgE, recurrent staphylococcal cold skin abscess, and recurrent pneumonia with pneumatocele formation. Most cases of HIES are sporadic although can be inherited as autosomal dominant and autosomal recessive traits. A fundamental immunologic defect in HIES is not clearly elucidated but abnormal neutrophil chemotaxis due to decreased production or secretion of interferon γ has main role in the immunopathogenesis of syndrome, also distorted Th1/Th2 cytokine profile toward a Th2 bias contributes to the impaired cellular immunity and a specific pattern of infection susceptibility as well as atopic-allergic constitution of syndrome. The ophthalmic manifestations of this disorder include conjunctivitis, keratitis, spontaneous corneal perforation, recurrent giant chalazia, extensive xanthelasma, tumors of the eyelid, strabismus, and bilateral keratoconus. The diagnosis of HIES is inconclusive, dependent on the evolution of a constellation of complex multisystemic symptoms and signs which develop over the years. Until time, no treatment modality is curative for basic defect in HIES, in terms of cytokines/chemokines derangement. Of note, bone marrow transplant and a monoclonal anti-IgE (omalizumab are hoped to be successful treatment in future.

  18. Gerodermia osteodysplastica and wrinkly skin syndrome: are they the same?

    Science.gov (United States)

    Al-Gazali, L I; Sztriha, L; Skaff, F; Haas, D

    2001-07-01

    Gerodermia osteodysplastica (GO) is a connective tissue disorder characterized by premature aging, wrinkled, and lax skin with reduced elasticity which is more marked on the dorsum of the hands and feet associated with hyperextensible joints and osteoporosis. The wrinkly skin syndrome (WSS) is characterized by wrinkled skin over the dorsum of the hands, feet, and abdomen; hyperextensible joints, particularly of the hands; intrauterine growth retardation; postnatal failure to thrive; and mental and developmental delay. We report on five children from two consanguineous Arab families with features overlapping both GO and WSS. All five children had similar dysmorphic facial features consisting of broad and prominent forehead, hypotelorism with epicanthal folds, prominent bulbous nose, flat malar region, and large protruding ears. All had wrinkling of the skin more marked on the dorsum of the hands, feet, and abdomen; hyperextensibility of the joints, particularly of the hands; and aged appearance. Intrauterine growth retardation, subsequent failure to thrive, developmental delay, and variable degree of osteoporosis was also present in all of them. The older three children developed progressive prognathism. We suggest that GO and WSS could represent variable manifestation of the same disorder. Copyright 2001 Wiley-Liss, Inc.

  19. Staphylococcal superantigens stimulate immortalized human adipocytes to produce chemokines.

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    Bao G Vu

    Full Text Available BACKGROUND: Human adipocytes may have significant functions in wound healing and the development of diabetes through production of pro-inflammatory cytokines after stimulation by gram-negative bacterial endotoxin. Diabetic foot ulcers are most often associated with staphylococcal infections. Adipocyte responses in the area of the wound may play a role in persistence and pathology. We studied the effect of staphylococcal superantigens (SAgs on immortalized human adipocytes, alone and in the presence of bacterial endotoxin or staphylococcal α-toxin. METHODOLOGY/PRINCIPAL FINDINGS: Primary non-diabetic and diabetic human preadipocytes were immortalized by the reverse transcriptase component of telomerase (TERT and the E6/E7 genes of human papillomavirus. The immortal cells were demonstrated to have properties of non-immortalized pre-adipocytes and could be differentiated into mature and functional adipocytes. Differentiated adipocytes exposed to staphylococcal SAgs produced robust levels of cytokines IL-6 and IL-8, but there were no significant differences in levels between the non-diabetic and diabetic cells. Cytokine production was increased by co-incubation of adipocytes with SAgs and endotoxin together. In contrast, α-toxin alone was cytotoxic at high concentrations, but, at sub-cytotoxic doses, did not stimulate production of IL-6 and IL-8. CONCLUSIONS/SIGNIFICANCE: Endotoxin has been proposed to contribute to diabetes through enhanced insulin resistance after chronic exposure and stimulation of adipocytes to produce cytokines. Our data indicate staphylococcal SAgs TSST-1 and SEB alone and in combination with bacterial endotoxin also stimulate adipocytes to produce cytokines and thus may contribute to the inflammatory response found in chronic diabetic ulcers and in the systemic inflammation that is associated with the development and persistence of diabetes. The immortal human pre-adipocytes reported here will be useful for studies to

  20. Peeling skin syndrome: 11 cases from Saudi Arabia

    International Nuclear Information System (INIS)

    Al-Ghamdi, Fasiza; Al-Raddadi, Ali; Satti, Mohamed

    2006-01-01

    Peeling skin syndrome (PSS) is a rare genodermatoses of probable autosomal recessive inheritance in Saudi Arabia, consanguinity of parents is common and consequently the occurrence of family disease, including that of the skin, is not uncommon. To characterize the clinical and pathological features of PSS in Saudi Arabia, we reviewed the medical records and clinical photographs of patients with recurring blistering diseases and conducted a histopathologic evaluation of skin biopsies to identify the site of cleavage. Eleven persons with PSS were seen at King Khalid National Guard Hospital in Jeddah between the years 1986 and 2005. Ages ranged between 2 and 15 years and there were 9 males (81.8%) and 2 females (18.2%). The most common presentation in the majority of patients was localized spontaneous peeling of the skin. Eight patients (72.8%) had a history of vesicles that were small, dry and peeled away. Trauma did no play role in blister formation. All patients were local from Bedouin tribes where a family history of a similar complaint was documented in 8 cases (72.7%). And consanguinity of marriage was evident on 6 patients (54.5%). Histological examination of the biopsies showed either intracorneal or superficial subcorneal cleavage above the granuler layer in all biopsied patients. Although rare, PSS occurs in Saudi Arabia and is most likely related to consanguinity of marriages. This disease is generally mild and is characterized by intrascorneal cleavage within the superficial epidermis. The disease should be recognized and not confused with other vasicobullous disease. (author)

  1. [Efficacy observation on application of negative pressure therapy in the treatment of superficial partial-thickness scald wound in children].

    Science.gov (United States)

    Shen, Chuan-an; Chai, Jia-ke; Tuo, Xiao-ye; Cai, Jian-hua; Li, Dong-jie; Zhang, Lin; Zhu, Hua; Cai, Jin-dong

    2013-02-01

    To observe the effect of negative pressure therapy in the treatment of superficial partial-thickness scald in children. Three hundred and seven children with superficial partial-thickness scald hospitalized from August 2009 to May 2012 were divided into negative pressure therapy group (NPT, n = 145) and control group (C, n = 162) according to the random number table. Patients in group NPT were treated with negative pressure from within post injury day (PID) 3 to PID 9 (with -16 kPa pressure), while traditional occlusive dressing method was used in group C. Changes in body temperature, wound healing condition, frequency of dressing change were compared between group NPT and group C. Bacterial culture results of wounds were compared before and after treatment in group NPT. Volume of drained transudate per one percent of wound area was recorded in group NPT on PID 1 to PID 3. Data were processed with t test or chi-square test. The incidence of high fever was significantly lower in group NPT (26.9%, 39/145) than in group C (63.6%, 103/162, χ(2) = 41.419, P partial-thickness scald.

  2. Evolutionary paths of streptococcal and staphylococcal superantigens

    Directory of Open Access Journals (Sweden)

    Okumura Kayo

    2012-08-01

    Full Text Available Abstract Background Streptococcus pyogenes (GAS harbors several superantigens (SAgs in the prophage region of its genome, although speG and smez are not located in this region. The diversity of SAgs is thought to arise during horizontal transfer, but their evolutionary pathways have not yet been determined. We recently completed sequencing the entire genome of S. dysgalactiae subsp. equisimilis (SDSE, the closest relative of GAS. Although speG is the only SAg gene of SDSE, speG was present in only 50% of clinical SDSE strains and smez in none. In this study, we analyzed the evolutionary paths of streptococcal and staphylococcal SAgs. Results We compared the sequences of the 12–60 kb speG regions of nine SDSE strains, five speG+ and four speG–. We found that the synteny of this region was highly conserved, whether or not the speG gene was present. Synteny analyses based on genome-wide comparisons of GAS and SDSE indicated that speG is the direct descendant of a common ancestor of streptococcal SAgs, whereas smez was deleted from SDSE after SDSE and GAS split from a common ancestor. Cumulative nucleotide skew analysis of SDSE genomes suggested that speG was located outside segments of steeper slopes than the stable region in the genome, whereas the region flanking smez was unstable, as expected from the results of GAS. We also detected a previously undescribed staphylococcal SAg gene, selW, and a staphylococcal SAg -like gene, ssl, in the core genomes of all Staphylococcus aureus strains sequenced. Amino acid substitution analyses, based on dN/dS window analysis of the products encoded by speG, selW and ssl suggested that all three genes have been subjected to strong positive selection. Evolutionary analysis based on the Bayesian Markov chain Monte Carlo method showed that each clade included at least one direct descendant. Conclusions Our findings reveal a plausible model for the comprehensive evolutionary pathway of streptococcal and

  3. Differential skin test reactivity to pollens in pollen food allergy syndrome versus allergic rhinitis.

    Science.gov (United States)

    Ta, Von; Scott, David R; Chin, William K; Wineinger, Nathan E; Kelso, John M; White, Andrew A

    2015-01-01

    Pollen food allergy syndrome (PFAS), also called oral allergy syndrome, is a form of food allergy in which uncooked foods cause allergic symptoms generally limited to the oral mucosa. It occurs in a subset of patients with pollen allergy, although not all patients have prominent rhinitis symptoms. PFAS is related to antigenic similarity between the pollen and food allergen. The size of skin test reactions in a group of subjects with pollen sensitivity with PFAS was compared with a group of subjects who were pollen sensitive and without PFAS. Self-reported rhinitis symptoms between the two groups were compared to identify if symptom severity differed. Twenty subjects with PFAS and 20 subjects with seasonal allergic rhinitis without PFAS were enrolled in the study. All the subjects underwent standard skin-prick testing to a panel of common allergens, including select fresh fruits and vegetables. The subjects completed a Mini Rhinoconjunctivitis Quality of Life Questionnaire as part of their clinical evaluation. The subjects with PFAS and those without PFAS were compared statistically. The subjects with PFAS had significantly larger-sized skin-prick test results specific to pollens (p PFAS reported milder nasal symptoms in relation to pollen skin test result size when compared with allergic rhinitis controls without PFAS. Our study outlined basic differences between two seemingly similar patient groups with a particularly striking discordance between skin test result sizes and rhinitis symptoms. This discordance should be explored further to increase mechanistic understanding of allergen cross-reactivity in PFAS.

  4. DNA repair in Bloom's syndrome skin fibroblasts after ultraviolet light irradiation

    International Nuclear Information System (INIS)

    Kurihara, Takayuki; Inoue, Masao; Kawashima, Hiroko; Yagi, Takashi; Takebe, Hiraku.

    1981-01-01

    Skin fibroblasts from a patient with Bloom's syndrome (86NoKi) were assayed for various DNA repair activities after ultraviolet light (UV) irradiation. Cultured fibroblasts as well as lymphocytes obtained from this patient showed a high frequency of spontaneous sister chromatid exchanges (SCEs). There was no significant difference between 86NoKi fibroblasts and skin fibroblasts from normal donors in the sensitivity to UV as measured by inactivation of colony forming activity, the capacity of host-cell reactivation (HCR) of UV-irradiated virus, and the amount of unscheduled DNA synthesis (UDS) after UV irradiation. However, the yield of UV-induced SCEs in 86NoKi cells was significantly higher than that in normal cells. (author)

  5. Goltz syndrome: A newborn with ectrodactyly and skin lesions

    Directory of Open Access Journals (Sweden)

    Shatanik Sarkar

    2015-01-01

    Full Text Available Goltz syndrome or Focal Dermal Hypoplasia is a rare multisystem disorder, involving all the three germ cell layers. The disease is thought to be inherited in X-linked dominant fashion with heterogeneous mutations of the PORCN gene at Xp11.23 locus. Majority of the cases are sporadic, mainly due to postzygotic somatic mutations. The clinical spectrum includes characteristic cutaneous manifestations, multiple skeletal anomalies, and involvement of the eyes, hair, nails, kidneys, and so on. Considerable variability is noted in the clinical expression of the disease probably due to genomic mosaicism. Around 300 cases of Goltz syndrome have been reported in the literature. Here, we report such a case with characteristic skin lesions, multiple bony defects, distinctive facial features, coloboma of iris, and bilateral hydronephrosis. The diagnosis was evident immediately after birth due to the characteristic clinical picture of the baby.

  6. Circumferential skin folds in a child: A case of Michelin tire baby syndrome

    Directory of Open Access Journals (Sweden)

    Palit Aparna

    2007-01-01

    Full Text Available A six-month-old girl who presented with dermatitis was found to have multiple, symmetric, deep, gyrate skin folds involving her trunk and similar circumferential lesions on her extremities since birth. She had a characteristic round face with hypertelorism, depressed nasal bridge, thin, down-turned vermillion border of upper lip and short neck. Skin biopsy demonstrated increased smooth muscle fibers in the deeper dermis. A diagnosis of Michelin tire baby syndrome was made. Clinical features, histopathology, differential diagnosis and prognosis of this rare disorder have been discussed.

  7. Skin lesions simulating blue toe syndrome caused by prolonged contact with a millipede

    Directory of Open Access Journals (Sweden)

    Augusto Scardazan Heeren Neto

    2014-04-01

    Full Text Available Venomous animals are those that, by means of a hunting and defense mechanism, are able to inject their prey with a toxic substance produced in their bodies, directly from specialized glands (e.g., tooth, sting, spur through which the poison passes. Millipedes are poisonous animals; they can be harmful to humans, and their effects usually manifest as erythematous, purpuric, and cyanotic lesions; local pain; and paresthesia. Here, we report a case of skin contact with a millipede for 6h resulting in skin lesions similar to blue toe syndrome.

  8. Burning mouth syndrome: A review

    Directory of Open Access Journals (Sweden)

    Rajendra G Patil

    2017-01-01

    Full Text Available Burning mouth syndrome is a condition characterized by chronic orofacial pain without any mucosal abnormalities or other organic disease. There are numerous synonyms for this ailment such as stomatodynia, stomatopyrosis, glossodynia, glossopyrosis, sore mouth, sore tongue, oral dysesthesia, and scalding mouth syndrome. Patients usually present with burning, stinging, or numbness on the tongue or other areas of oral mucosa. The complex etiology and lack of characteristic signs and symptoms makes the diagnosis difficult. As a result of which managing such patients become a herculean task. Moreover, lack of understanding of the disease leads to misdiagnosis and unnecessary referral of patients. In this article, the authors have described the etiopathogenesis, diagnostic algorithm and management of this confusing ailment.

  9. Immunoglobulin A nephropathy in association with generalized inflammatory peeling skin syndrome.

    Science.gov (United States)

    Srinivasaraghavan, Rangan; Krishnamurthy, Sriram; Chandar, Rumesh; Mahadevan, Subramanian; Chandrashekar, Laxmisha; Rajesh, Nachiappa Ganesh

    2015-01-01

    We describe an 8-year-old girl born to second-degree consanguineous parents with complaints of recurrent episodes of hematuria for 6 months. She had generalized peeling of the skin since birth and recurrent purulent cutaneous infections. The clinical presentation and histopathology of the skin biopsy specimen were consistent with the inflammatory variant of peeling skin syndrome (PSS). She also had a single ventricle with pulmonary stenosis, for which a bidirectional Glenn shunt had been placed. The renal biopsy specimen showed immunoglobulin A (IgA) nephropathy. She responded well to enalapril and steroids, with a decrease in proteinuria. IgA nephropathy has not been previously reported in PSS. Complications such as IgA nephropathy in children with PSS would help to further delineate the diverse clinical presentations and the clinical course of this rare dermatosis. We discuss the mechanisms that could explain this hitherto unreported association. © 2014 Wiley Periodicals, Inc.

  10. Staphylococcus aureus and staphylococcal enterotoxin A in breaded chicken products: detection and behavior during the cooking process.

    Science.gov (United States)

    Pepe, Olimpia; Blaiotta, Giuseppe; Bucci, Francesca; Anastasio, Marilena; Aponte, Maria; Villani, Francesco

    2006-11-01

    In this study we examined the presence of Staphylococcus aureus and staphylococcal enterotoxin A (SEA) in 20 industrial breaded chicken products obtained from different retail butchers and supermarket stores in Italy. The levels of contamination in the products analyzed were quite low, although the pH values and water activities (a(w)) in the samples considered were in ranges favorable for S. aureus growth. As demonstrated by phenotypic and molecular characterization, in spite of the high percentage of coagulase-positive Staphylococcus strains, only three strains could be referred to the species S. aureus. Moreover, all the strains were negative in PCR assays targeting staphylococcal enterotoxin genes (seA to seE, seG to seJ, and seM to seO), as well as the toxic shock syndrome toxin 1 gene, and no SEA was detected in the retail breaded chicken samples analyzed by a reversed passive latex agglutination assay or by Western blotting. Hence, we evaluated the thermal resistance of two strains of SEA-producing S. aureus in a laboratory-scale preparation of precooked breaded chicken cutlets. The heat treatment employed in the manufacture determined the inactivation of S. aureus cells, but the preformed SEA remained active during product storage at 4 degrees C. The presence of the staphylococci and, in particular, of S. aureus in the retail breaded chicken products analyzed is a potential health risk for consumers since the pH and a(w) values of these kinds of products are favorable for S. aureus growth. The thermal process used during their manufacture can limit staphylococcal contamination but cannot eliminate preformed toxins.

  11. Adult-onset acral peeling skin syndrome in a non-identical twin: a case report in South Africa.

    Science.gov (United States)

    Mathew, Reshmi; Omole, Olufemi B; Rigby, Jonathan; Grayson, Wayne

    2014-12-31

    Acral peeling skin syndrome is a rare autosomal recessive disorder in which skin exfoliation is limited to the hands and feet. While it typically manifests from early childhood, in this first reported case from South Africa, the patient did not manifest clinically until the fourth decade of life. A 44-year-old woman of African descent, 1 of a set of non-identical twins, presented with recurrent episodes of skin peeling of the upper and lower limbs. The first episode occurred 4 years prior, followed by perennial skin peeling during the spring seasons. She was not on treatment for any chronic disease and reported no exposure to chemicals or other irritants. The family, including the non-identical twin sister, has no history of skin disorders and the patient's HIV antibody test was negative. At presentation, physical examination revealed ongoing exfoliation with new skin formation on the palms and soles. The mucous membranes and nails were spared. Other systems were normal. Skin biopsy taken from the palms confirmed peeling skin syndrome. The patient was managed with topical aqueous cream and analgesics. She was briefly counseled on the nature and prognosis of the disease, and referred for genetic testing and counseling. On follow-up, she continues to have skin peeling once or twice a year. This first reported case of this rare disease in South Africa contributes to the growing body of literature on the disease and highlights the need for clinicians to be aware of its variable clinical onset.

  12. Peeling skin syndrome: genetic defects in late terminal differentiation of the epidermis.

    Science.gov (United States)

    Bowden, Paul E

    2011-03-01

    In this issue, Israeli and colleagues confirm that homozygous mutations in corneodesmosin (CDSN) cause type B peeling skin syndrome (PSS), an autosomal recessive skin disorder. The deletion mutation described resulted in a frameshift, producing a downstream premature stop codon and early truncation of the protein. The recently described CDSN nonsense mutation in another PSS family also resulted in protein truncation and nonsense-mediated mRNA decay. Type B generalized PSS can now be clearly distinguished from acral PSS, caused by mutations in transglutaminase 5. This directly affects cornified envelope cross-linking rather than corneodesmosome adherence. These observations provide new insight into the molecular defects underlying two closely related forms of PSS.

  13. Pediatric scalds: do cooking-related burns have a higher injury burden?

    Science.gov (United States)

    Bachier, Marielena; Hammond, Sarah E; Williams, Regan; Jancelewicz, Timothy; Feliz, Alexander

    2015-11-01

    Pediatric scald burns result in frequent emergency room visits and hospitalizations. We investigated whether cooking-related burns produce greater morbidity requiring more extensive care than noncooking burns. We performed a 6-y review at our free-standing children's hospital. Children aged cooking versus noncooking burns. The Mann-Whitney U test, a chi-square test, and the negative binomial were used to compare continuous, categorical, and count data between groups. Bivariate analysis was performed to identify risk factors among patients with adverse outcomes. We identified 308 patients; 262 (85%) cooking and 46 (15%) noncooking burns. Most patients were African-American males, with public insurance, and a median age of 2 y. Cooking burns preferentially occurred over the head, neck, and upper body; noncooking burns were distributed over the lower body (P  0.11). In subgroup analysis, semisolid and grease burns resulted in increased rates of wound contractures and/or limited mobility when compared with noncooking burns (P = 0.05 and P = 0.008, respectively). Patients with complications were more likely to have third degree burns and required more consults, longer hospitalization, and more surgical debridements and clinic visits. Most accidental scald burns occurred in young children during food preparation. Greater long-term morbidity was found in patients with semisolid and grease burns. This subset of children has a higher injury burden and requires extensive care in the acute and long-term setting. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Inflammatory peeling skin syndrome caused a novel mutation in CDSN.

    Science.gov (United States)

    Telem, Dana Fuchs; Israeli, Shirli; Sarig, Ofer; Sprecher, Eli

    2012-04-01

    Generalized peeling skin syndrome (PSS) is a rare autosomal recessive dermatosis manifesting with continuous exfoliation of the stratum corneum. The inflammatory (type B) subtype of PSS was recently found to be caused by deleterious mutations in the CDSN gene encoding corneodesmosin, a major component of desmosomal junctions in the uppermost layers of the epidermis. In the present study, we assessed a 10-month-old baby, who presented with generalized superficial peeling of the skin. Using PCR amplification and direct sequencing, we identified the third PSS-associated mutation in CDSN, a homozygous 4 bp duplication in the second exon of the gene (c.164_167dup GCCT; p.Thr57ProfsX6). These data further support the notion that corneodesmosin deficiency impairs cell-cell adhesion in the upper epidermis, paving the way for an abnormal inflammatory response due to epidermal barrier disruption.

  15. Skin vasomotor hemiparesis followed by overactivity: characteristic thermography findings in a patient with Horner syndrome due to spinal cord infarction.

    Science.gov (United States)

    Kobayashi, Makoto

    2016-04-01

    We present a 21-year-old female with Horner syndrome due to spinal cord infarction. In this patient, infrared thermography revealed a hemibody skin temperature increase followed by excessive focal decreases, indicating skin vasomotor hemiparesis and overactivity.

  16. The Influence of Scalded Flour, Fermentation, and Plants Belonging to Lamiaceae Family on the Wheat Bread Quality and Acrylamide Content.

    Science.gov (United States)

    Bartkiene, Elena; Bartkevics, Vadims; Krungleviciute, Vita; Pugajeva, Iveta; Zadeike, Daiva; Juodeikiene, Grazina; Cizeikiene, Dalia

    2018-06-01

    The aim of this study was to investigate the influence of additives such as plants belonging to Lamiaceae family (Thymus vulgaris, Carum carvi, Origanum vulgare, Ocimum basilicum, and Coriandrum sativum), scalded flour (SF) or scalded flour fermented with Lactobacillus plantarum LUHS135 (SFFLp) on the quality and acrylamide formation in wheat bread. The formation of acrylamide and bread quality significantly depended on the king of plants used and the amount of SF and SFFLp used. The additives of T. vulgaris and SF increased the content of acrylamide by 3.4-fold in comparison with bread prepared without SF, whereas the addition of SFFLp significantly reduced the content of acrylamide in bread, especially using 5% of SFFLp supplemented with O. vulgare and 15% of SFFLp supplemented with C. sativum (respectively by 40% and 29.4%) therefore could be recommended for safer bread production. The addition of 5% (from total wheat flour content) of scalded wheat flour fermented with Lactobacillus plantarum LUHS135 strain (SFFLp) with Origanum vulgare addition, and 5% or 10% of SFFLp prepared with Ocimum basilicum, and 15% of SFFLp prepared with Coriandrum sativum significantly reduce the content of acrylamide in wheat bread, therefore could be recommended for safer bread production. © 2018 Institute of Food Technologists®.

  17. Burns and scalds in pre-school children attending accident and emergency: accident or abuse?

    OpenAIRE

    Benger, J; McCabe, S

    2001-01-01

    Objectives—To assess how frequently and adequately information relating to the possibility of non-accidental injury (NAI) is documented and considered by doctors assessing pre-school children with burns and scalds in the accident and emergency (A&E) department, and to determine the effect of introducing a routine reminder mechanism into the A&E notes, coupled with an improved programme of NAI education and awareness.

  18. Basal cell carcinoma of the skin (part 1): epidemiology, pathology and genetic syndromes.

    Science.gov (United States)

    Correia de Sá, Tiago Ribeiro; Silva, Roberto; Lopes, José Manuel

    2015-11-01

    Basal cell carcinoma (BCC) is the most common skin cancer worldwide with increasing incidence, but difficult to assess due to the current under registration practice. Despite the low mortality rate, BCC is a cause of great morbidity and an economic burden to health services. There are several risk factors that increase the risk of BCC and partly explain its incidence. Low-penetrance susceptibility alleles, as well as genetic alterations in signaling pathways, namely SHH pathway, also contribute to the carcinogenesis. BCC associate with several genetic syndromes, of which basal cell nevus syndrome is the most common.

  19. Attenuation of massive cytokine response to the staphylococcal enterotoxin B superantigen by the innate immunomodulatory protein lactoferrin

    Science.gov (United States)

    Hayworth, J L; Kasper, K J; Leon-Ponte, M; Herfst, C A; Yue, D; Brintnell, W C; Mazzuca, D M; Heinrichs, D E; Cairns, E; Madrenas, J; Hoskin, D W; McCormick, J K; Haeryfar, S M M

    2009-01-01

    Staphylococcal enterotoxin B (SEB) is a pyrogenic exotoxin and a potent superantigen which causes massive T cell activation and cytokine secretion, leading to profound immunosuppression and morbidity. The inhibition of SEB-induced responses is thus considered a goal in the management of certain types of staphylococcal infections. Lactoferrin (LF) is a multi-functional glycoprotein with both bacteriostatic and bactericidal activities. In addition, LF is known to have potent immunomodulatory properties. Given the anti-microbial and anti-inflammatory properties of this protein, we hypothesized that LF can modulate T cell responses to SEB. Here, we report that bovine LF (bLF) was indeed able to attenuate SEB-induced proliferation, interleukin-2 production and CD25 expression by human leucocyte antigen (HLA)-DR4 transgenic mouse T cells. This inhibition was not due to bLF's iron-binding capacity, and could be mimicked by the bLF-derived peptide lactoferricin. Cytokine secretion by an engineered SEB-responsive human Jurkat T cell line and by peripheral blood mononuclear cells from healthy donors was also inhibited by bLF. These findings reveal a previously unrecognized property of LF in modulation of SEB-triggered immune activation and suggest a therapeutic potential for this naturally occurring protein during toxic shock syndrome. PMID:19659771

  20. Aberrant distribution patterns of corneodesmosomal components of tape-stripped corneocytes in atopic dermatitis and related skin conditions (ichthyosis vulgaris, Netherton syndrome and peeling skin syndrome type B).

    Science.gov (United States)

    Igawa, Satomi; Kishibe, Mari; Honma, Masaru; Murakami, Masamoto; Mizuno, Yuki; Suga, Yasushi; Seishima, Mariko; Ohguchi, Yuka; Akiyama, Masashi; Hirose, Kenji; Ishida-Yamamoto, Akemi; Iizuka, Hajime

    2013-10-01

    Atopic dermatitis (AD), Netherton syndrome (NS) and peeling skin syndrome type B (PSS) may show some clinical phenotypic overlap. Corneodesmosomes are crucial for maintaining stratum corneum integrity and the components' localization can be visualized by immunostaining tape-stripped corneocytes. In normal skin, they are detected at the cell periphery. To determine whether AD, NS, PSS and ichthyosis vulgaris (IV) have differences in the corneodesmosomal components' distribution and corneocytes surface areas. Corneocytes were tape-stripped from a control group (n=12) and a disease group (37 AD cases, 3 IV cases, 4 NS cases, and 3 PSS cases), and analyzed with immunofluorescent microscopy. The distribution patterns of corneodesmosomal components: desmoglein 1, corneodesmosin, and desmocollin 1 were classified into four types: peripheral, sparse diffuse, dense diffuse and partial diffuse. Corneocyte surface areas were also measured. The corneodesmosome staining patterns were abnormal in the disease group. Other than in the 3 PSS cases, all three components showed similar patterns in each category. In lesional AD skin, the dense diffuse pattern was prominent. A high rate of the partial diffuse pattern, loss of linear cell-cell contacts, and irregular stripping manners were unique to NS. Only in PSS was corneodesmosin staining virtually absent. The corneocyte surface areas correlated significantly with the rate of combined sparse and dense diffuse patterns of desmoglein 1. This method may be used to assess abnormally differentiated corneocytes in AD and other diseases tested. In PSS samples, tape stripping analysis may serve as a non-invasive diagnostic test. Copyright © 2013 Japanese Society for Investigative Dermatology. Published by Elsevier Ireland Ltd. All rights reserved.

  1. Candidate biomarker discovery and selection for ‘Granny Smith' superficial scald risk management and diagnosis, poster board

    Science.gov (United States)

    Discovery of candidate biomarkers for superficial scald, a peel disorder that develops during storage of susceptible apple cultivars, is part of a larger project aimed at developing biomarker-based risk-management and diagnostic tools for multiple apple postharvest disorders (http://www.tfrec.wsu.ed...

  2. Peeling skin syndrome in eight cases of four different families from India and Bangladesh

    Directory of Open Access Journals (Sweden)

    Nilendu Sarma

    2012-01-01

    Full Text Available Peeling skin syndrome (PSS is a rare recessively inherited ichthyosiform genodermatoses characterized by superficial skin peeling. This has 2 subtypes, acral (APSS; OMIM 609796 and generalized form (OMIM 270300. The later has been subdivided into type A (non-inflammatory and type B (inflammatory. Eight cases of peeling skin syndrome in 4 families were recorded over a period of 5 years. They were diagnosed clinically and confirmed histopathologically. Disease onset ranged from birth to childhood age (mean 5.25 ± 4.528 years and age at presentation ranged from 7-35 years (mean 23.25 ± 10.471 years. Males outnumbered females (M:F - 5:3. All had non-inflammatory generalized disease of type-A PSS variety, except one who had type-B PSS. Two Muslim families (1 st and 2 nd family, total 5 patients came from nearby country Bangladesh, and the 2 Hindu families were Indian. Higher severity over acral areas in generalized type, possible autosomal dominant pattern of inheritance and improvement with age as found in this series were new manifestations and possibly unreported previously. The disease was found to be poorly responsive to oral retinoids. Prevalence of the disease may be higher than expected. Importance of mutational analysis was also highlighted.

  3. Peeling skin syndrome in eight cases of four different families from India and Bangladesh.

    Science.gov (United States)

    Sarma, Nilendu; Boler, Anup Kumar; Bhanja, Dulal Chandra

    2012-01-01

    Peeling skin syndrome (PSS) is a rare recessively inherited ichthyosiform genodermatoses characterized by superficial skin peeling. This has 2 subtypes, acral (APSS; OMIM 609796) and generalized form (OMIM 270300). The later has been subdivided into type A (non-inflammatory) and type B (inflammatory). Eight cases of peeling skin syndrome in 4 families were recorded over a period of 5 years. They were diagnosed clinically and confirmed histopathologically. Disease onset ranged from birth to childhood age (mean 5.25 ± 4.528 years) and age at presentation ranged from 7-35 years (mean 23.25 ± 10.471 years). Males outnumbered females (M:F - 5:3). All had non-inflammatory generalized disease of type-A PSS variety, except one who had type-B PSS. Two Muslim families (1 st and 2 nd family, total 5 patients) came from nearby country Bangladesh, and the 2 Hindu families were Indian. Higher severity over acral areas in generalized type, possible autosomal dominant pattern of inheritance and improvement with age as found in this series were new manifestations and possibly unreported previously. The disease was found to be poorly responsive to oral retinoids. Prevalence of the disease may be higher than expected. Importance of mutational analysis was also highlighted.

  4. [Effects of exogenous high mobility group protein box 1 on angiogenesis in ischemic zone of early scald wounds of rats].

    Science.gov (United States)

    Dai, L; Guo, X; Huang, H J; Liao, X M; Luo, X Q; Li, D; Zhou, H; Gao, X C; Tan, M Y

    2018-04-20

    Objective: To observe effects of exogenous high mobility group protein box 1 (HMGB1) on angiogenesis in ischemic zone of early scald wounds of rats. Methods: Thirty-six Sprague-Dawley rats were divided into HMGB1 group and simple scald (SS) group according to the random number table, with 18 rats in each group. Comb-like copper mould was placed on the back of rats for 20 s after being immersed in 100 ℃ hot water for 3 to 5 min to make three ischemic zones of wound. Immediately after scald, rats in HMGB1 group were subcutaneously injected with 0.4 μg HMGB1 and 0.1 mL phosphate buffer solution (PBS), and rats in SS group were subcutaneously injected with 0.1 mL PBS from boarders of ischemic zone of scald wound. At post scald hour (PSH) 24, 48, and 72, 6 rats in each group were collected. Protein expressions of vascular endothelial growth factor (VEGF) in ischemic zone of wound at PSH 24, 48, and 72 and protein expressions of CD31 in ischemic zone of wound at PSH 48 and 72 were detected by immunohistochemistry. The number of microvessel in CD31 immunohistochemical sections of ischemic zone of wound at PSH 48 and 72 was calculated after observing by the microscope. The mRNA expressions of VEGF and CD31 in ischemic zone of wound were detected by real-time fluorescence quantitative reverse transcription polymerase chain reaction at PSH 24, 48, and 72. Data were processed with analysis of variance of factorial design, t test, and Bonferroni correction. Results: (1) At PSH 24, 48, and 72, protein expressions of VEGF in ischemic zone of wound of rats in HMGB1 group were significantly higher than those of rats in SS group ( t =7.496, 4.437, 5.402, P zone of wound of rats in HMGB1 group were 0.038 8±0.007 9 and 0.057 7±0.001 2 respectively, significantly higher than 0.013 4±0.004 9 and 0.030 3±0.004 0 of rats in SS group ( t =10.257, 15.055, P zone of wound of rats in HMGB1 group was obviously more than that of rats in SS group ( t =3.536, 4.000, P zone of wound of

  5. A case report of Muir-Torre syndrome in a woman with breast cancer and MSI-Low skin squamous cell carcinoma

    OpenAIRE

    Kientz, Caroline; Joly, Marie-Odile; Faivre, Laurence; Clemenson, Alix; Dalac, Sophie; Lepage, C?me; Chapusot, Caroline; Jacquot, Caroline; Schiappa, Renaud; Lebrun, Marine

    2017-01-01

    Background The tumor spectrum in the Lynch syndrome is well defined, comprising an increased risk of developing colonic and extracolonic malignancies. Muir-Torre syndrome is a variant with a higher risk of skin disease. Patients have been described carrying mutations in the mismatch repair genes and presenting tumors with unusual histology or affected organ not part of the Lynch syndrome spectrum. Hence, the real link between Lynch syndrome, or Muir-Torre syndrome, and these tumors remains di...

  6. Acral peeling skin syndrome: a clinically and genetically heterogeneous disorder.

    Science.gov (United States)

    Pavlovic, Sasha; Krunic, Aleksandar L; Bulj, Tanja K; Medenica, Maria M; Fong, Kenneth; Arita, Ken; McGrath, John A

    2012-01-01

    Acral peeling skin syndrome (APSS) is a rare, autosomal, recessive genodermatosis characterized by painless spontaneous exfoliation of the skin of the hands and feet at a subcorneal or intracorneal level. It usually presents at birth or appears later in childhood or early adulthood. Some cases result from mutations in the TGM5 gene that encodes transglutaminase 5, which has an important role in cross-linking cornified cell envelope proteins. We report a new APSS pedigree from Jordan that contains at least 10 affected family members, although sequencing of the TGM5 gene failed to disclose any pathogenic mutation(s). On the basis of probable consanguinity, we performed homozygosity mapping and identified areas of homozygosity on chromosomes 1, 6, 10, 13, and 16, although none of the intervals contained genes of clear relevance to cornification. APSS is a clinically and genetically heterogeneous disorder, and this Jordanian pedigree underscores the likelihood of still further heterogeneity. © 2011 Wiley Periodicals, Inc.

  7. Feather retention force in broiler carcasses slaughtered and held up to 8 hours postmortem prior to scalding

    Science.gov (United States)

    One factor that could impact the feasibility of commercial on-farm slaughter of broilers is the time delay from on-farm slaughter to scalding and defeathering in the commercial plant that could be 4 h or more. This experiment evaluated feather retention force (FRF) in broilers that were slaughtered ...

  8. Hyperelastic skin

    Science.gov (United States)

    ... is most often seen in people who have Ehlers-Danlos syndrome. People with this disorder have very elastic skin. ... any member of your family been diagnosed with Ehlers-Danlos syndrome? What other symptoms are present? Genetic counseling may ...

  9. Staphylococcal Bicomponent Pore-Forming Toxins: Targets for Prophylaxis and Immunotherapy

    Directory of Open Access Journals (Sweden)

    M. Javad Aman

    2014-03-01

    Full Text Available Staphylococccus aureus represents one of the most challenging human pathogens as well as a common colonizer of human skin and mucosal surfaces. S. aureus causes a wide range of diseases from skin and soft tissue infection (SSTI to debilitating and life-threatening conditions such as osteomyelitis, endocarditis, and necrotizing pneumonia. The range of diseases reflects the remarkable diversity of the virulence factors produced by this pathogen, including surface antigens involved in the establishment of infection and a large number of toxins that mediate a vast array of cellular responses. The staphylococcal toxins are generally believed to have evolved to disarm the innate immune system, the first line of defense against this pathogen. This review focuses on recent advances on elucidating the biological functions of S. aureus bicomponent pore-forming toxins (BCPFTs and their utility as targets for preventive and therapeutic intervention. These toxins are cytolytic to a variety of immune cells, primarily neutrophils, as well as cells with a critical barrier function. The lytic activity of BCPFTs towards immune cells implies a critical role in immune evasion, and a number of epidemiological studies and animal experiments relate these toxins to clinical disease, particularly SSTI and necrotizing pneumonia. Antibody-mediated neutralization of this lytic activity may provide a strategy for development of toxoid-based vaccines or immunotherapeutics for prevention or mitigation of clinical diseases. However, certain BCPFTs have been proposed to act as danger signals that may alert the immune system through an inflammatory response. The utility of a neutralizing vaccination strategy must be weighed against such immune-activating potential.

  10. Staphylococcal bicomponent pore-forming toxins: targets for prophylaxis and immunotherapy.

    Science.gov (United States)

    Aman, M Javad; Adhikari, Rajan P

    2014-03-04

    Staphylococccus aureus represents one of the most challenging human pathogens as well as a common colonizer of human skin and mucosal surfaces. S. aureus causes a wide range of diseases from skin and soft tissue infection (SSTI) to debilitating and life-threatening conditions such as osteomyelitis, endocarditis, and necrotizing pneumonia. The range of diseases reflects the remarkable diversity of the virulence factors produced by this pathogen, including surface antigens involved in the establishment of infection and a large number of toxins that mediate a vast array of cellular responses. The staphylococcal toxins are generally believed to have evolved to disarm the innate immune system, the first line of defense against this pathogen. This review focuses on recent advances on elucidating the biological functions of S. aureus bicomponent pore-forming toxins (BCPFTs) and their utility as targets for preventive and therapeutic intervention. These toxins are cytolytic to a variety of immune cells, primarily neutrophils, as well as cells with a critical barrier function. The lytic activity of BCPFTs towards immune cells implies a critical role in immune evasion, and a number of epidemiological studies and animal experiments relate these toxins to clinical disease, particularly SSTI and necrotizing pneumonia. Antibody-mediated neutralization of this lytic activity may provide a strategy for development of toxoid-based vaccines or immunotherapeutics for prevention or mitigation of clinical diseases. However, certain BCPFTs have been proposed to act as danger signals that may alert the immune system through an inflammatory response. The utility of a neutralizing vaccination strategy must be weighed against such immune-activating potential.

  11. Transcriptome-Wide Expression Profiling in Skin Fibroblasts of Patients with Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type.

    Science.gov (United States)

    Chiarelli, Nicola; Carini, Giulia; Zoppi, Nicoletta; Dordoni, Chiara; Ritelli, Marco; Venturini, Marina; Castori, Marco; Colombi, Marina

    2016-01-01

    Joint hypermobility syndrome/Ehlers-Danlos syndrome hypermobility type (JHS/EDS-HT), is likely the most common systemic heritable connective tissue disorder, and is mostly recognized by generalized joint hypermobility, joint instability complications, minor skin changes and a wide range of satellite features. JHS/EDS-HT is considered an autosomal dominant trait but is still without a defined molecular basis. The absence of (a) causative gene(s) for JHS/EDS-HT is likely attributable to marked genetic heterogeneity and/or interaction of multiple loci. In order to help in deciphering such a complex molecular background, we carried out a comprehensive immunofluorescence analysis and gene expression profiling in cultured skin fibroblasts from five women affected with JHS/EDS-HT. Protein study revealed disarray of several matrix structural components such as fibrillins, tenascins, elastin, collagens, fibronectin, and their integrin receptors. Transcriptome analysis indicated perturbation of different signaling cascades that are required for homeostatic regulation either during development or in adult tissues as well as altered expression of several genes involved in maintenance of extracellular matrix architecture and homeostasis (e.g., SPON2, TGM2, MMP16, GPC4, SULF1), cell-cell adhesion (e.g., CDH2, CHD10, PCDH9, CLDN11, FLG, DSP), immune/inflammatory/pain responses (e.g., CFD, AQP9, COLEC12, KCNQ5, PRLR), and essential for redox balance (e.g., ADH1C, AKR1C2, AKR1C3, MAOB, GSTM5). Our findings provide a picture of the gene expression profile and dysregulated pathways in JHS/EDS-HT skin fibroblasts that correlate well with the systemic phenotype of the patients.

  12. TGM5 mutations impact epidermal differentiation in acral peeling skin syndrome.

    Science.gov (United States)

    Pigors, Manuela; Kiritsi, Dimitra; Cobzaru, Cristina; Schwieger-Briel, Agnes; Suárez, Jose; Faletra, Flavio; Aho, Heikki; Mäkelä, Leeni; Kern, Johannes S; Bruckner-Tuderman, Leena; Has, Cristina

    2012-10-01

    Acral peeling skin syndrome (APSS) is an autosomal recessive skin disorder characterized by acral blistering and peeling of the outermost layers of the epidermis. It is caused by mutations in the gene for transglutaminase 5, TGM5. Here, we report on clinical and molecular findings in 11 patients and extend the TGM5 mutation database by four, to our knowledge, previously unreported mutations: p.M1T, p.L41P, p.L214CfsX15, and p.S604IfsX9. The recurrent mutation p.G113C was found in 9 patients, but also in 3 of 100 control individuals in a heterozygous state, indicating that APSS might be more widespread than hitherto expected. Using quantitative real-time PCR, immunoblotting, and immunofluorescence analysis, we demonstrate that expression and distribution of several epidermal differentiation markers and corneodesmosin (CDSN) is altered in APSS keratinocytes and skin. Although the expression of transglutaminases 1 and 3 was not changed, we found an upregulation of keratin 1, keratin 10, involucrin, loricrin, and CDSN, probably as compensatory mechanisms for stabilization of the epidermal barrier. Our results give insights into the consequences of TGM5 mutations on terminal epidermal differentiation.

  13. Staphylococcal enterotoxin-like X (SElX is a unique superantigen with functional features of two major families of staphylococcal virulence factors.

    Directory of Open Access Journals (Sweden)

    Ries J Langley

    2017-09-01

    Full Text Available Staphylococcus aureus is an opportunistic pathogen that produces many virulence factors. Two major families of which are the staphylococcal superantigens (SAgs and the Staphylococcal Superantigen-Like (SSL exoproteins. The former are immunomodulatory toxins that induce a Vβ-specific activation of T cells, while the latter are immune evasion molecules that interfere with a wide range of innate immune defences. The superantigenic properties of Staphylococcal enterotoxin-like X (SElX have recently been established. We now reveal that SElX also possesses functional characteristics of the SSLs. A region of SElX displays high homology to the sialyl-lactosamine (sLacNac-specific binding site present in a sub-family of SSLs. By analysing the interaction of SElX with sLacNac-containing glycans we show that SElX has an equivalent specificity and host cell binding range to the SSLs. Mutation of key amino acids in this conserved region affects the ability of SElX to bind to cells of myeloid origin and significantly reduces its ability to protect S. aureus from destruction in a whole blood killing (WBK assay. Like the SSLs, SElX is up-regulated early during infection and is under the control of the S. aureus exotoxin expression (Sae two component gene regulatory system. Additionally, the structure of SElX in complex with the sLacNac-containing tetrasaccharide sialyl Lewis X (sLeX reveals that SElX is a unique single-domain SAg. In summary, SElX is an 'SSL-like' SAg.

  14. Meta-analysis of staphylococcal diarrhea in some developing African countries

    Directory of Open Access Journals (Sweden)

    George P. Einstein

    2017-06-01

    Full Text Available Staphylococcal diarrhea is a common gastro-intestinal illness caused by poor water supply and unhygienic food preparation. Although, about 70% of diarrheal cases per year have been attributed to the consumption of contaminated foods, in Africa, few studies have reported the incidence of Staphylococcus-related diarrhea and the implicated virulence factors. We investigated the implications of common factors such as age of children, age of mothers, maternal level of education, gender of children, overall bacteria isolated, sanitary condition status and feeding type of mothers on the burden of staphylococcal diarrhea. A literature search was performed using Pubmed, Googlescholar, American society for microbiology (ASM journals and other sources. The quality of studies was assessed. Adjusted odds ratios with 95% confidence intervals (CI for the occurrence of Staphylococcus aureus amongst other causes were extracted. For each study, a multivariate logistic regression analysis of the adjusted odds ratio was performed to identify the risk factors on the burden of staphylococcal diarrhea. Six case-control studies were included in the meta–analysis. Quality of individual studies rages from 0.57–0.84 (median, 0.69. meta-analysis gave pooled odds ratios 3.27 (95% CI; 2.88–4.17. The results identified inadequate sanitary conditions and type of feeding as major risk factors.

  15. Acral peeling skin syndrome resembling epidermolysis bullosa simplex in a 10-month-old boy.

    Science.gov (United States)

    Kavaklieva, S; Yordanova, I; Bruckner-Tuderman, L; Has, C

    2013-01-01

    The acral peeling skin syndrome (APSS) is a rare autosomal recessive disorder clinically characterized by asymptomatic desquamation of the skin limited to the hands and feet and histologically by cleavage at the stratum granulosum and stratum corneum level [Kiritsi et al.: J Invest Dermatol 2010;130:1741-1746]. We report on a 10-month-old boy with a history of skin peeling limited to the hands and feet since 2 months of age. Clinical examination revealed erythematous erosions with peripheral desquamation and flaccid blisters. DNA mutation analysis detected two heterozygous TGM5 mutations: c.2T>C, p.M1T in exon 1 and c.337G>T, p.G113C in exon 3 in keeping with the diagnosis of APSS. The clinical presentation of APSS alone might be confusing and strongly resemble epidermolysis bullosa simplex making the differential diagnosis difficult.

  16. The protective effects of sildenafil in acute lung injury in a rat model of severe scald burn: A biochemical and histopathological study.

    Science.gov (United States)

    Gokakin, Ali Kagan; Deveci, Koksal; Kurt, Atilla; Karakus, Boran Cihat; Duger, Cevdet; Tuzcu, Mehmet; Topcu, Omer

    2013-09-01

    Severe burn induces biochemical mediators such as reactive oxygen species that leads to lipid peroxidation which may have a key role in formation of acute lung injury (ALI). Sildenafil is a selective and potent inhibitor of cyclic guanosine monophosphate specific phosphodiesterase-5. Sildenafil preserves alveolar growth, angiogenesis, reduces inflammation and airway reactivity. The purpose of the present study was to evaluate the effects of different dosages of sildenafil in ALI due to severe scald burn in rats. Twenty-four rats were subjected to 30% total body surface area severe scald injury and were randomly divided into three equal groups as follow: control, 10 and 20mg/kg sildenafil groups. Levels of malondialdehyde (MDA), activities of glutathione peroxidase (Gpx), catalase (Cat), total oxidative stress (TOS), and total antioxidative capacity (TAC) were measured in both tissues and serums. Oxidative stress index (OSI) was calculated. A semi-quantitative scoring system was used for the evaluation of histopatological findings. Sildenafil increased Gpx, Cat, TAC and decreased MDA, TOS and OSI. Sildenafil decreased inflammation scores in lungs. Our results reveal that sildenafil is protective against scald burn related ALI by decreasing oxidative stress and inflammation and the dosage of 10mg/kg could be apparently better than 20mg/kg. Copyright © 2013 Elsevier Ltd and ISBI. All rights reserved.

  17. The integration of physiologically-targeted skin care in the management of atopic dermatitis: focus on the use of a cleanser and moisturizer system incorporating a ceramide precursor, filaggrin degradation products, and specific "skin-barrier-friendly" excipients.

    Science.gov (United States)

    Del Rosso, James Q; Kircik, Leon H

    2013-07-01

    Atopic dermatitis (AD) may be considered the "poster disease" for exemplifying the significance of abnormalities of the epidermal barrier that occur predominantly within the stratum corneum (SC) and upper epidermis. Specifically, impairments of the SC permeability barrier, antimicrobial barrier, and immunologic barrier contribute markedly to the fundamental pathophysiology of AD. The multiple clinical sequelae associated with epidermal barrier impairments inherent to AD include dry skin, pruritus, increased skin sensitivity to irritants and allergens, eczematous skin changes, staphylococcal skin and anterior nares colonization, and increase in some cutaneous infections (ie, molluscum contagiosum). This article addresses the pathophysiology of AD with clinically relevant correlations, and discusses the scientific basis of a specially designed cleanser and moisturizer system that incorporates ceramide technology and filaggrin degradation products along with other "barrier-friendly" excipients.

  18. Blue Rubber Bleb Nevus Syndrome Showing Vascular Skin Lesions Predominantly on the Face

    Directory of Open Access Journals (Sweden)

    Ayumi Korekawa

    2015-07-01

    Full Text Available An 81-year-old Japanese man presented with dark blue papules and nodules on his face. There were multiple soft papules and nodules, dark blue in color, compressive, and ranging in size from 2 to 10 mm. A few similar lesions were seen on the patient's right dorsal second toe and right buccal mucosa. There were no skin lesions on his trunk and upper limbs. The patient's past history did not include gastrointestinal bleeding or anemia. Histopathological examination showed dilated vascular spaces lined by the normal epithelium extending beneath the dermis and into the subcutaneous fat. Endoscopy of the gastrointestinal tract to check for colon involvement was not performed. X-ray images of the limbs revealed no abnormalities in the bones or joints. Laboratory investigations did not show anemia. Although we failed to confirm a diagnosis by endoscopy, the skin lesions, histopathological findings, lack of abnormal X-ray findings, and the presence of oral lesions as a part of gastrointestinal tract guided the diagnosis of blue rubber bleb nevus syndrome (BRBNS. Skin lesions of BRBNS occur predominantly on the trunk and upper limbs. However, the present case showed multiple skin lesions predominantly on the face. Therefore, it is important for clinicians to know about a possible atypical distribution of skin lesions in BRBNS.

  19. Acral Peeling Skin Syndrome Resembling Epidermolysis Bullosa Simplex in a 10-Month-Old Boy

    Directory of Open Access Journals (Sweden)

    S. Kavaklieva

    2013-08-01

    Full Text Available The acral peeling skin syndrome (APSS is a rare autosomal recessive disorder clinically characterized by asymptomatic desquamation of the skin limited to the hands and feet and histologically by cleavage at the stratum granulosum and stratum corneum level [Kiritsi et al.: J Invest Dermatol 2010;130:1741-1746]. We report on a 10-month-old boy with a history of skin peeling limited to the hands and feet since 2 months of age. Clinical examination revealed erythematous erosions with peripheral desquamation and flaccid blisters. DNA mutation analysis detected two heterozygous TGM5 mutations: c.2T>C, p.M1T in exon 1 and c.337G>T, p.G113C in exon 3 in keeping with the diagnosis of APSS. The clinical presentation of APSS alone might be confusing and strongly resemble epidermolysis bullosa simplex making the differential diagnosis difficult.

  20. Microbiology and evisceration efficiency of broiler carcasses slaughtered and held up to 8 hours postmortem prior to scalding and defeathering

    Science.gov (United States)

    The implementation of on farm slaughter could eliminate potential animal welfare issues associated with cooping, transport, dumping, and shackling live broilers. This research evaluated evisceration efficiency and the microbiological implications of delaying scalding and defeathering for up to 8 h a...

  1. Cumulative irritancy in the guinea pig from low grade irritant vehicles and the angry skin syndrome

    DEFF Research Database (Denmark)

    Andersen, Klaus Ejner; Maibach, H I

    1980-01-01

    A 4-week open cumulative irritancy test in guinea pigs discriminated between two low grade irritant vehicles, nonionic base (anhydrous) and hydrophilic ointment. The procedure might be useful as a predictive test for low grade irritants. The angry skin syndrome was established in the guinea pigs...

  2. Fournier gangrene associated with hyper IgE syndrome (Job syndrome).

    Science.gov (United States)

    Hori, Junichi; Yamaguchi, Satoshi; Watanabe, Masaki; Osanai, Hiroaki; Hori, Masako

    2008-04-01

    We report a case of a 32-year-old man with hyper IgE syndrome (Job syndrome) who developed Fournier gangrene due to infectious multiple atheromas of the scrotal skin that progressed to the right groin and thigh. The patient required surgical debridement and subsequent skin grafting. This is a rare case of Fournier gangrene associated with hyper IgE syndrome (Job syndrome). When a patient without diabetes mellitus has repeated infections and atopic-like dermatitis, Job syndrome should be considered.

  3. Rhodomyrtus tomentosa (Aiton) Hassk. leaf extract: An alternative approach for the treatment of staphylococcal bovine mastitis.

    Science.gov (United States)

    Mordmuang, Auemphon; Voravuthikunchai, Supayang Piyawan

    2015-10-01

    Antibiotic residues in dairy products as well as emergence of antimicrobial resistance in foodborne pathogens have been recognized as global public health concerns. The present work was aimed to study a potent antibacterial extract from natural product as an alternative treatment for staphylococcal bovine mastitis. Staphylococcal isolates (n=44) were isolated from milk samples freshly squeezed from individual cows. All staphylococcal isolates were resistant to ampicillin, ciprofloxacin, erythromycin, gentamicin, penicillin, except vancomycin. Rhodomyrtus tomentosa leaf ethanolic extract was accessed for its antibacterial activity and anti-inflammatory potential. The extract exhibited profound antibacterial activity against all of staphylococcal isolates with MIC and MBC values ranged from 16-64 μg/ml and 64->128 μg/ml, respectively. Moreover, the extract also exerted anti-protein denaturation and human red blood cell membrane stabilizing activity. The results support the use of R. tomentosa extract that could be applied to cure bovine mastitis and to reduce inflammatory injury caused by the bacterial infections. Copyright © 2015 Elsevier Ltd. All rights reserved.

  4. Segmental stiff skin syndrome (SSS): A distinct clinical entity.

    Science.gov (United States)

    Myers, Kathryn L; Mir, Adnan; Schaffer, Julie V; Meehan, Shane A; Orlow, Seth J; Brinster, Nooshin K

    2016-07-01

    Stiff skin syndrome (SSS) is a noninflammatory, fibrosing condition of the skin, often affecting the limb girdles. We present 4 new patients with SSS with largely unilateral, segmental distribution. To date, reported cases of SSS have been grouped based on generally accepted clinical and histopathologic findings. The purpose of this study was to analyze differences in clinical and histopathologic findings between previously reported SSS cases. This is a retrospective review of 4 new cases and 48 previously published cases of SSS obtained from PubMed search. Of 52 total cases, 18 (35%) were segmentally distributed and 34 (65%) were widespread. The average age of onset was 4.1 years versus 1.6 years for segmental versus widespread SSS, respectively. Limitation in joint mobility affected 44% of patients with segmental SSS and 97% of patients with widespread SSS. Histopathologic findings were common between the 2 groups. This was a retrospective study of previously published cases limited by the completeness and accuracy of the reviewed cases. We propose a distinct clinical entity, segmental SSS, characterized by a segmental distribution, later age of onset, and less severe functional limitation. Both segmental SSS and widespread SSS share common diagnostic histopathologic features. Copyright © 2016 American Academy of Dermatology, Inc. All rights reserved.

  5. Increased Susceptibility of Humanized NSG Mice to Panton-Valentine Leukocidin and Staphylococcus aureus Skin Infection.

    Directory of Open Access Journals (Sweden)

    Ching Wen Tseng

    Full Text Available Staphylococcus aureus is a leading cause of skin and soft-tissue infections worldwide. Mice are the most commonly used animals for modeling human staphylococcal infections. However a supra-physiologic S. aureus inoculum is required to establish gross murine skin pathology. Moreover, many staphylococcal factors, including Panton-Valentine leukocidin (PVL elaborated by community-associated methicillin-resistant S. aureus (CA-MRSA, exhibit selective human tropism and cannot be adequately studied in mice. To overcome these deficiencies, we investigated S. aureus infection in non-obese diabetic (NOD/severe combined immune deficiency (SCID/IL2rγnull (NSG mice engrafted with human CD34+ umbilical cord blood cells. These "humanized" NSG mice require one to two log lower inoculum to induce consistent skin lesions compared with control mice, and exhibit larger cutaneous lesions upon infection with PVL+ versus isogenic PVL- S. aureus. Neutrophils appear important for PVL pathology as adoptive transfer of human neutrophils alone to NSG mice was sufficient to induce dermonecrosis following challenge with PVL+ S. aureus but not PVL- S. aureus. PMX53, a human C5aR inhibitor, blocked PVL-induced cellular cytotoxicity in vitro and reduced the size difference of lesions induced by the PVL+ and PVL- S. aureus, but PMX53 also reduced recruitment of neutrophils and exacerbated the infection. Overall, our findings establish humanized mice as an important translational tool for the study of S. aureus infection and provide strong evidence that PVL is a human virulence factor.

  6. Evolutionary Origin of the Staphylococcal Cassette Chromosome mec (SCCmec)

    DEFF Research Database (Denmark)

    Rolo, Joana; Worning, Peder; Nielsen, Jesper Boye

    2017-01-01

    , many steps are still missing from this evolutionary history. In particular, it is not known how mecA was incorporated into the mobile element SCC prior to dissemination among Staphylococcus aureus and other pathogenic staphylococcal species. To gain insights into the possible contribution of several...

  7. Staphylococcal species associated with bovine mastitis in the North West of Iran: Emerging of coagulase-negative staphylococci

    Directory of Open Access Journals (Sweden)

    Somayyeh Hosseinzadeh

    2014-06-01

    Full Text Available Staphylococcal mastitis is a major and costly problem of dairy cattle all over the world. The objective of this study was to isolate and identify the main staphylococcal species causing bovine mastitis in 7 dairy herds located in 4 different areas of East and West Azerbaijan provinces, Iran. Of the 158 mastitic milk samples collected, 113 staphylococcal isolates were identified (71.5% on the basis of cultural and biochemical features as well as by genus specific PCR. Then, species level identification of staphylococcal isolates was carried out using restriction fragment length polymorphism (RFLP analysis of the gap gene (933 bp. On the basis of polymerase chain reaction-RFLP, 10 different patterns were identified. Of 113 isolates, 5 (4.4% were Staphylococcus aureus and 108 (95.6% were coagulase-negative staphylococci (CoNS. Overall, nine different species of CoNS were identified as: 44 Staphylococcus haemolyticus (40.7%, 17 Staphylococcus chromogenes (15.7%, 11 Staphylococcus epidermidis, Staphylococcus warneri and Staphylococcus cohnii each (10.2%, 6 Staphylococcus simulans (5.5%, 4 Staphylococcus hominis (3.7%, 3 Staphylococcus capitis (2.7% and 1 Staphylococcus xylosus (0.9%. S. haemolyticus, S. chromogenes and S. warneri were the only species identified from clinical mastitis. No significant difference in staphylococcal IMI was found among the studied herds and regions. This study demonstrated that CoNS, especially S. haemolyticus and S. chromogenes, were predominant and thus be considered as emerging pathogens causing mastitis in the North West of Iran. Our results also revealed that the gap PCR-RFLP was useful for identifying staphylococcal isolates derived from bovine mastitis at species level.

  8. Four Cases of Atopic Dermatitis Complicated by Sjogren's Syndrome: Link between Dry Skin and Autoimmune Anhidrosis

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    Shun Kitaba

    2011-01-01

    Full Text Available We report four adult cases of atopic dermatitis (AD complicated by Sjogren's syndrome (SS. The patients fulfilled diagnostic criteria for AD and SS. All cases showed persistent itchy dry skin and eczematous lesions complicated by sicca symptoms including dry eyes and dry mouth with moderate joint pain. One case manifested annular erythema and another manifested widespread discoid erythema. To investigate the underlying cause of dry skin in these cases, sweating function was evaluated using a quantitative sudomotor axon reflex test (QSART in which the axon reflex is stimulated by acetylcholine iontophoresis. The sweating latency time was significantly prolonged in eczematous skin of AD and AD/SS compared to normal controls. Axon reflex (AXR sweat volume was also significantly reduced in AD (normal and eczematous skin and AD/SS (normal and eczema compared to normal control. In contrast, the direct sweat volume of lesional or non-lesional AD skin induced by direct stimulation with acetylcholine was only slightly reduced compared to that in normal controls, but not in SS and lesional skin of AD/SS patients. These results suggest that the impaired sweat response in AD is attributable to an abnormal sudomotor axon reflex, which is accelerated and modulated when complicated by SS resulting in dry skin in the present cases.

  9. Identification of a novel STAT3 mutation in a patient with hyper-IgE syndrome

    DEFF Research Database (Denmark)

    Mogensen, Trine H; Jakobsen, Marianne; Larsen, Carsten S

    2013-01-01

    Here we describe a patient with hyper-IgE syndrome presenting with recurrent staphylococcal abscesses, pneumonia, and chronic mucocutaneous candidiasis, and report the identification of a novel STAT3 mutation at amino acid position 621, which has not previously been described. In addition, we...

  10. Presence of non-fibrillar amyloid beta protein in skin biopsies of Alzheimer's disease (AD), Down's syndrome and non-AD normal persons

    DEFF Research Database (Denmark)

    Wen, G Y; Wisniewski, H M; Blondal, H

    1994-01-01

    A total of 66 skin biopsies from persons with Alzheimer's disease (AD) or Down's syndrome (DS) and from persons without AD were used in this study. The age range was from 7 to 89 years. Positive immunoreactivity of skin biopsies to monoclonal antibody 4G8, which is reactive to amino acid residue 17...

  11. Ex vivo nonlinear microscopy imaging of Ehlers-Danlos syndrome-affected skin.

    Science.gov (United States)

    Kiss, Norbert; Haluszka, Dóra; Lőrincz, Kende; Kuroli, Enikő; Hársing, Judit; Mayer, Balázs; Kárpáti, Sarolta; Fekete, György; Szipőcs, Róbert; Wikonkál, Norbert; Medvecz, Márta

    2018-07-01

    Ehlers-Danlos syndrome (EDS) is the name for a heterogenous group of rare genetic connective tissue disorders with an overall incidence of 1 in 5000. The histological characteristics of EDS have been previously described in detail in the late 1970s and early 1980s. Since that time, the classification of EDS has undergone significant changes, yet the description of the histological features of collagen morphology in different EDS subtypes has endured the test of time. Nonlinear microscopy techniques can be utilized for non-invasive in vivo label-free imaging of the skin. Among these techniques, two-photon absorption fluorescence (TPF) microscopy can visualize endogenous fluorophores, such as elastin, while the morphology of collagen fibers can be assessed by second-harmonic generation (SHG) microscopy. In our present work, we performed TPF and SHG microscopy imaging on ex vivo skin samples of one patient with classical EDS and two patients with vascular EDS and two healthy controls. We detected irregular, loosely dispersed collagen fibers in a non-parallel arrangement in the dermis of the EDS patients, while as expected, there was no noticeable impairment in the elastin content. Based on further studies on a larger number of patients, in vivo nonlinear microscopic imaging could be utilized for the assessment of the skin status of EDS patients in the future.

  12. Burning Mouth Syndrome and Menopause

    Science.gov (United States)

    Dahiya, Parveen; Kamal, Reet; Kumar, Mukesh; Niti; Gupta, Rajan; Chaudhary, Karun

    2013-01-01

    Menopause is a physiological process typically occurring in the fifth decade of life. One of the most annoying oral symptoms in this age group is the burning mouth syndrome (BMS), which may be defined as an intraoral burning sensation occurring in the absence of identifiable oral lesion or laboratory findings. Pain in burning mouth syndrome may be described as burning, tender, tingling, hot, scalding, and numb sensation in the oral mucosa. Multiple oral sites may be involved, but the anterior two-third part and the tip of tongue are most commonly affected site. There is no definite etiology for BMS other than the precipitating causative factors, and it is still considered idiopathic. Various treatment options like use of benzodiazepine, anti-depressants, analgesics, capsaicin, alpha lipoic acids, and cognitive behavioral therapy are found to be effective, but definite treatment is still unknown. The present article discusses some of the recent concepts of etiopathogenesis of BMS as well as the role of pharmacotherapeutic management in this disorder. PMID:23411996

  13. Defective IL-10 signaling in hyper-IgE syndrome results in impaired generation of tolerogenic dendritic cells and induced regulatory T cells

    Science.gov (United States)

    Saito, Masako; Nagasawa, Masayuki; Takada, Hidetoshi; Hara, Toshiro; Tsuchiya, Shigeru; Agematsu, Kazunaga; Yamada, Masafumi; Kawamura, Nobuaki; Ariga, Tadashi; Tsuge, Ikuya; Nonoyama, Shigeaki; Karasuyama, Hajime

    2011-01-01

    Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by recurrent staphylococcal infections and atopic dermatitis associated with elevated serum IgE levels. Although defective differentiation of IL-17–producing CD4+ T cells (Th17) partly accounts for the susceptibility to staphylococcal skin abscesses and pneumonia, the pathogenesis of atopic manifestations in HIES still remains an enigma. In this study, we examined the differentiation and function of Th1, Th2, regulatory T cells (Treg cells), and dendritic cells (DCs) in HIES patients carrying either STAT3 or TYK2 mutations. Although the in vitro differentiation of Th1 and Th2 cells and the number and function of Treg cells in the peripheral blood were normal in HIES patients with STAT3 mutations, primary and monocyte-derived DCs showed defective responses to IL-10 and thus failed to become tolerogenic. When treated with IL-10, patient DCs showed impaired up-regulation of inhibitory molecules on their surface, including PD-L1 and ILT-4, compared with control DCs. Moreover, IL-10–treated DCs from patients displayed impaired ability to induce the differentiation of naive CD4+ T cells to FOXP3+ induced Treg cells (iTreg cells). These results suggest that the defective generation of IL-10–induced tolerogenic DCs and iTreg cells may contribute to inflammatory changes in HIES. PMID:21300911

  14. Pigmentary Markers in Danes--Associations with Quantitative Skin Colour, Nevi Count, Familial Atypical Multiple-Mole, and Melanoma Syndrome.

    Directory of Open Access Journals (Sweden)

    Peter Johansen

    Full Text Available To investigate whether pigmentation genes involved in the melanogenic pathway (melanogenesis contributed to melanoma predisposition, we compared pigmentary genetics with quantitative skin pigmentation measurements, the number of atypical nevi, the total nevus count, and the familial atypical multiple mole and melanoma (FAMMM syndrome. We typed 32 pigmentary SNP markers and sequenced MC1R in 246 healthy individuals and 116 individuals attending periodic control for malignant melanoma development, 50 of which were diagnosed with FAMMM. It was observed that individuals with any two grouped MC1R variants (missense, NM_002386:c. 456C > A (p.TYR152*, or NM_002386:c.83_84insA (p.Asn29Glnfs*14 had significantly (p<0.001 lighter skin pigmentation of the upper-inner arm than those with none or one MC1R variant. We did not observe any significant association of the MC1R variants with constitutive pigmentation measured on the buttock area. We hypothesize that the effect of MC1R variants on arm pigmentation is primarily reflecting the inability to tan when subjected to UVR. A gender specific effect on skin pigmentation was also observed, and it was found that the skin pigmentation of females on average were darker than that of males (p<0.01. We conclude that MC1R variants are associated with quantitative skin colour in a lightly pigmented Danish population. We did not observe any association between any pigmentary marker and the FAMMM syndrome. We suggest that the genetics of FAMMM is not related to the genetics of the pigmentary pathway.

  15. A Marfan syndrome gene expression phenotype in cultured skin fibroblasts

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    Emond Mary

    2007-09-01

    Full Text Available Abstract Background Marfan syndrome (MFS is a heritable connective tissue disorder caused by mutations in the fibrillin-1 gene. This syndrome constitutes a significant identifiable subtype of aortic aneurysmal disease, accounting for over 5% of ascending and thoracic aortic aneurysms. Results We used spotted membrane DNA macroarrays to identify genes whose altered expression levels may contribute to the phenotype of the disease. Our analysis of 4132 genes identified a subset with significant expression differences between skin fibroblast cultures from unaffected controls versus cultures from affected individuals with known fibrillin-1 mutations. Subsequently, 10 genes were chosen for validation by quantitative RT-PCR. Conclusion Differential expression of many of the validated genes was associated with MFS samples when an additional group of unaffected and MFS affected subjects were analyzed (p-value -6 under the null hypothesis that expression levels in cultured fibroblasts are unaffected by MFS status. An unexpected observation was the range of individual gene expression. In unaffected control subjects, expression ranges exceeding 10 fold were seen in many of the genes selected for qRT-PCR validation. The variation in expression in the MFS affected subjects was even greater.

  16. Heterogeneous response to X-ray and ultraviolet light irradiations of cultured skin fibroblasts in two families with Gardner's Syndrome

    International Nuclear Information System (INIS)

    Kinsella, T.J.; Little, J.B.; Nove, J.; Weichselbaum, R.R.; Li, F.P.; Meyer, R.J.; Marchetto, D.J.; Patterson, W.B.

    1982-01-01

    A heterogeneous response to X-ray and far UV (254 nm) light irradiations was found in cultured skin fibroblast lines from 2 separate families with Gardner's syndrome. When compared to 2 normal control cultures and cultures from 2 patients with nonfamilial colon cancer, cultures from 4 clinically affected members of family 1 showed increased sensitivity to the lethal effects of both X-ray and UV light irradiations. These cells also showed a delayed pattern of X-ray potentially lethal damage repair (PLDR) and absent UV PLDR. In contrast, cultures from 3 members of family 2 (2 of whom were clinically affected) showed a normal response of survival and PLDR to both X-ray and UV light irradiations. Thus increased sensitivity of cultured skin fibroblasts to X-ray and UV light irradiations was not a consistent in vitro finding in patients with Gardner's syndrome. However, in families with Gardner's syndrome who demonstrate in vitro radiosensitivity, additional studies are needed to assess the usefulness of these techniques in detecting affected individuals prior to the development of colon carcinoma and other manifestations

  17. Preliminary Essay on the Effect of Foliar Treatment with the Fungicide Triadimenol on Barley Culture Infected by Scald

    Directory of Open Access Journals (Sweden)

    Nasraoui, B.

    1999-01-01

    Full Text Available This study deals with the foliar treatment by the fungicide triadimenol against barley scald. Results have shown that two or three triadimenol treatments have practically stopped the infection evolution. The disease have slightly extended with only one treatment. Moreover, other assessment showed that one, two or three triadimenol treatments were significantly associated to the same increase in the yield.

  18. Variation among Staphylococcus aureus membrane vesicle proteomes affects cytotoxicity of host cells.

    Science.gov (United States)

    Jeon, Hyejin; Oh, Man Hwan; Jun, So Hyun; Kim, Seung Il; Choi, Chi Won; Kwon, Hyo Il; Na, Seok Hyeon; Kim, Yoo Jeong; Nicholas, Asiimwe; Selasi, Gati Noble; Lee, Je Chul

    2016-04-01

    Staphylococcus aureus secretes membrane-derived vesicles (MVs), which can deliver virulence factors to host cells and induce cytopathology. However, the cytopathology of host cells induced by MVs derived from different S. aureus strains has not yet been characterized. In the present study, the cytotoxic activity of MVs from different S. aureus isolates on host cells was compared and the proteomes of S. aureus MVs were analyzed. The MVs purified from S. aureus M060 isolated from a patient with staphylococcal scalded skin syndrome showed higher cytotoxic activity toward host cells than that shown by MVs from three other clinical S. aureus isolates. S. aureus M060 MVs induced HEp-2 cell apoptosis in a dose-dependent manner, but the cytotoxic activity of MVs was completely abolished by treatment with proteinase K. In a proteomic analysis, the MVs from three S. aureus isolates not only carry 25 common proteins, but also carry ≥60 strain-specific proteins. All S. aureus MVs contained δ-hemolysin (Hld), γ-hemolysin, leukocidin D, and exfoliative toxin C, but exfoliative toxin A (ETA) was specifically identified in S. aureus M060 MVs. ETA was delivered to HEp-2 cells via S. aureus MVs. Both rETA and rHld induced cytotoxicity in HEp-2 cells. In conclusion, MVs from clinical S. aureus isolates differ with respect to cytotoxic activity in host cells, and these differences may result from differences in the MV proteomes. Further proteogenomic analysis or mutagenesis of specific genes is necessary to identify cytotoxic factors in S. aureus MVs. Copyright © 2016 Elsevier Ltd. All rights reserved.

  19. Insertional inactivation of Eap in Staphylococcus aureus strain Newman confers reduced staphylococcal binding to fibroblasts.

    Science.gov (United States)

    Hussain, Muzaffar; Haggar, Axana; Heilmann, Christine; Peters, Georg; Flock, Jan-Ingmar; Herrmann, Mathias

    2002-06-01

    To initiate invasive infection, Staphylococcus aureus must adhere to host substrates, such as the extracellular matrix or eukaryotic cells, by virtue of different surface proteins (adhesins). Recently, we identified a 60-kDa cell-secreted extracellular adherence protein (Eap) of S. aureus strain Newman with broad-spectrum binding characteristics (M. Palma, A. Haggar, and J. I. Flock, J. Bacteriol. 181:2840-2845, 1999), and we have molecularly confirmed Eap to be an analogue of the previously identified major histocompatibility complex class II analog protein (Map) (M. Hussain, K. Becker, C. von Eiff, G. Peter, and M. Herrmann, Clin. Diagn. Lab. Immunol. 8:1281-1286, 2001). Previous analyses of the Eap/Map function performed with purified protein did not allow dissection of its precise role in the complex situation of the staphylococcal whole cell presenting several secreted and wall-bound adhesins. Therefore, the role of Eap was investigated by constructing a stable eap::ermB deletion in strain Newman and by complementation of the mutant. Patterns of extracted cell surface proteins analyzed both by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and by Western ligand assays with various adhesive matrix molecules clearly confirmed the absence of Eap in the mutant. However, binding and adhesion tests using whole staphylococcal cells demonstrated that both the parent and mutant strains bound equally well to fibronectin- and fibrinogen-coated surfaces, possibly due to their recognition by other staphylococcal adhesins. Furthermore, Eap mediated staphylococcal agglutination of both wild-type and mutant cells. In contrast, the mutant adhered to a significantly lesser extent to cultured fibroblasts (P Eap, whereas preimmune serum was not active. In conclusion, Eap may contribute to pathogenicity by promoting adhesion of whole staphylococcal cells to complex eukaryotic substrates.

  20. Therapeutic Down-Modulators of Staphylococcal Superantigen-Induced Inflammation and Toxic Shock

    Directory of Open Access Journals (Sweden)

    Teresa Krakauer

    2010-07-01

    Full Text Available Staphylococcal enterotoxin B (SEB and related superantigenic toxins are potent stimulators of the immune system and cause a variety of diseases in humans, ranging from food poisoning to toxic shock. These toxins bind directly to major histocompatibility complex (MHC class II molecules on antigen-presenting cells and specific Vb regions of T-cell receptors (TCR, resulting in hyperactivation of both monocytes/macrophages and T lymphocytes. Activated host cells produce massive amounts of proinflammatory cytokines and chemokines, activating inflammation and coagulation, causing clinical symptoms that include fever, hypotension, and shock. This review summarizes the in vitro and in vivo effects of staphylococcal superantigens, the role of pivotal mediators induced by these toxins in the pathogenic mechanisms of tissue injury, and the therapeutic agents to mitigate the toxic effects of superantigens.

  1. Staphylococcal superantigen-specific IgE antibodies: degree of sensitization and association with severity of asthma.

    Science.gov (United States)

    Elabras, José; Mello, Fernanda Carvalho de Queiroz; Lupi, Omar; Bica, Blanca Elena Rios Gomes; Papi, José Angelo de Souza; França, Alfeu Tavares

    2016-01-01

    To determine the presence of staphylococcal superantigen-specific IgE antibodies and degree of IgE-mediated sensitization, as well as whether or not those are associated with the severity of asthma in adult patients. This was a cross-sectional study involving outpatients with asthma under treatment at a tertiary care university hospital in the city of Rio de Janeiro, Brazil. Consecutive patients were divided into two groups according to the severity of asthma based on the Global Initiative for Asthma criteria: mild asthma (MA), comprising patients with mild intermittent or persistent asthma; and moderate or severe asthma (MSA). We determined the serum levels of staphylococcal toxin-specific IgE antibodies, comparing the results and performing a statistical analysis. The study included 142 patients: 72 in the MA group (median age = 46 years; 59 females) and 70 in the MSA group (median age = 56 years; 60 females). In the sample as a whole, 62 patients (43.7%) presented positive results for staphylococcal toxin-specific IgE antibodies: staphylococcal enterotoxin A (SEA), in 29 (20.4%); SEB, in 35 (24.6%); SEC, in 33 (23.2%); and toxic shock syndrome toxin (TSST), in 45 (31.7%). The mean serum levels of IgE antibodies to SEA, SEB, SEC, and TSST were 0.96 U/L, 1.09 U/L, 1.21 U/L, and 1.18 U/L, respectively. There were no statistically significant differences between the two groups in terms of the qualitative or quantitative results. Serum IgE antibodies to SEA, SEB, SEC, and TSST were detected in 43.7% of the patients in our sample. However, neither the qualitative nor quantitative results showed a statistically significant association with the clinical severity of asthma. Determinar a presença de anticorpos IgE específicos para superantígenos estafilocócicos e o grau de sensibilização mediada por esses, assim como se esses estão associados à gravidade da asma em pacientes adultos. Estudo transversal incluindo asmáticos adultos em acompanhamento ambulatorial em

  2. Cyclical thrombocytosis, acquired von Willebrand syndrome and aggressive non-melanoma skin cancers are common in patients with Philadelphia-negative myeloproliferative neoplasms treated with hydroxyurea.

    Science.gov (United States)

    Verner, Emma; Forsyth, Cecily; Grigg, Andrew

    2014-05-01

    Abstract Cyclical thrombocytosis, acquired von Willebrand syndrome, aggressive non-melanoma skin cancers and other hydroxyurea complications have been reported in Philadelphia-negative myeloproliferative neoplasms (MPNs), but their incidence and clinical consequences have not been defined in a large cohort of patients. We conducted a retrospective analysis of 188 consecutive patients with MPNs specifically addressing the incidence of these complications. Cyclical thrombocytosis was documented in 29 patients (15%), the majority of whom were receiving hydroxyurea. Acquired von Willebrand syndrome was identified in 17 of the 84 screened patients (20%), but was not associated with any major bleeding complications. Non-melanoma skin cancers were reported in 51 patients (27%). Hydroxyurea-related fever occurred in nine of 149 patients (6%) who received hydroxyurea. Seventy-three patients (39%) experienced a total of 98 major thrombotic events, with the majority of these occurring prior to or within 3 months of the diagnosis. Cyclical thrombocytosis, acquired von Willebrand syndrome, aggressive non-melanoma skin cancers and other hydroxyurea-related complications are not infrequent in MPNs and have important clinical consequences for management.

  3. Marfan Syndrome

    Science.gov (United States)

    Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, ... A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

  4. Use of preputial skin for coverage of post-burn contractures of fingers in children

    Directory of Open Access Journals (Sweden)

    Mohammed I Zaroo

    2011-01-01

    Full Text Available Objective: Hand burns are common injuries. Children frequently sustain burn injuries, especially to their hands. Contractures are a common sequel of severe burns around joints. The prepuce, or foreskin, has been used as a skin graft for a number of indications. We conducted this study to evaluate the feasibility of utilising the preputial skin for the management of post-burn contractures of fingers in uncircumcised male children. Materials and Methods: Preputial skin was used for the coverage of released contractures of fingers in 12 patients aged 2-6 years. The aetiology of burns was "Kangri" burn in eight patients and scalding in four patients. Six patients had contracture in two fingers, four patients in one finger, and two patients had contractures in three fingers. Results: None of the patients had graft loss, and all the wounds healed within 2 weeks. All patients had complete release of contractures without any recurrence. Hyperpigmentation of the grafts was observed over a period of time, which was well accepted by the parents. Conclusions: Preputial skin can be used successfully for male children with mild-to-moderate contractures of 2-3 fingers for restoration of the hand function, minimal donor site morbidity.

  5. FDA approved Immunosuppressants Targeting Staphylococcal Superantigens: Mechanisms and Insights

    Science.gov (United States)

    2016-12-02

    cells , resulting in polyclonal T- cell activation [4-6]. Staphylococcal superantigens hyperactivate cells of the innate immune system and adaptive T... innate host defense responses, antiviral genes, apoptotic programs, immunoproteasomes, and has many immunomodulatory functions. The cell death...1692. 104. Mendis C, Das R, Hammamieh R, Royaee A, Yang D, Peel S, et al. Transcriptional response signature of human lymphoid cells to

  6. Acral peeling skin syndrome resulting from a homozygous nonsense mutation in the CSTA gene encoding cystatin A.

    Science.gov (United States)

    Krunic, Aleksandar L; Stone, Kristina L; Simpson, Michael A; McGrath, John A

    2013-01-01

    Acral peeling skin syndrome (APSS) is a clinically and genetically heterogeneous disorder. We used whole-exome sequencing to identify the molecular basis of APSS in a consanguineous Jordanian-American pedigree. We identified a homozygous nonsense mutation (p.Lys22X) in the CSTA gene, encoding cystatin A, that was confirmed using Sanger sequencing. Cystatin A is a protease inhibitor found in the cornified cell envelope, and loss-of-function mutations have previously been reported in two cases of exfoliative ichthyosis. Our study expands the molecular pathology of APSS and demonstrates the value of next-generation sequencing in the genetic characterization of inherited skin diseases. © 2013 Wiley Periodicals, Inc.

  7. A novel unstable duplication upstream of HAS2 predisposes to a breed-defining skin phenotype and a periodic fever syndrome in Chinese Shar-Pei dogs.

    Directory of Open Access Journals (Sweden)

    Mia Olsson

    2011-03-01

    Full Text Available Hereditary periodic fever syndromes are characterized by recurrent episodes of fever and inflammation with no known pathogenic or autoimmune cause. In humans, several genes have been implicated in this group of diseases, but the majority of cases remain unexplained. A similar periodic fever syndrome is relatively frequent in the Chinese Shar-Pei breed of dogs. In the western world, Shar-Pei have been strongly selected for a distinctive thick and heavily folded skin. In this study, a mutation affecting both these traits was identified. Using genome-wide SNP analysis of Shar-Pei and other breeds, the strongest signal of a breed-specific selective sweep was located on chromosome 13. The same region also harbored the strongest genome-wide association (GWA signal for susceptibility to the periodic fever syndrome (p(raw = 2.3 × 10⁻⁶, p(genome = 0.01. Dense targeted resequencing revealed two partially overlapping duplications, 14.3 Kb and 16.1 Kb in size, unique to Shar-Pei and upstream of the Hyaluronic Acid Synthase 2 (HAS2 gene. HAS2 encodes the rate-limiting enzyme synthesizing hyaluronan (HA, a major component of the skin. HA is up-regulated and accumulates in the thickened skin of Shar-Pei. A high copy number of the 16.1 Kb duplication was associated with an increased expression of HAS2 as well as the periodic fever syndrome (p < 0.0001. When fragmented, HA can act as a trigger of the innate immune system and stimulate sterile fever and inflammation. The strong selection for the skin phenotype therefore appears to enrich for a pleiotropic mutation predisposing these dogs to a periodic fever syndrome. The identification of HA as a major risk factor for this canine disease raises the potential of this glycosaminoglycan as a risk factor for human periodic fevers and as an important driver of chronic inflammation.

  8. Waardenburg Syndrome: A Case Report

    OpenAIRE

    Hayrullah Alp; Esma Alp

    2010-01-01

    Introduction: Auditory-pigmentary syndromes are a group of diseases that effect the skin, hair, eyes and the cochlea. Waardenburg syndrome is one of the members of these autosomal dominantly inherited diseases. Waardenburg syndrome is characterized by white forelock, congenital sensorineural hearing loss, hypopigmented skin and anomalies of the intraocular tissues. How ever all these diagnostic features may not be seen in all patient. In addition, there are four subtypes of the syndrome in ea...

  9. Burning mouth syndrome and menopause

    Directory of Open Access Journals (Sweden)

    Parveen Dahiya

    2013-01-01

    Full Text Available Menopause is a physiological process typically occurring in the fifth decade of life. One of the most annoying oral symptoms in this age group is the burning mouth syndrome (BMS, which may be defined as an intraoral burning sensation occurring in the absence of identifiable oral lesion or laboratory findings. Pain in burning mouth syndrome may be described as burning, tender, tingling, hot, scalding, and numb sensation in the oral mucosa. Multiple oral sites may be involved, but the anterior two-third part and the tip of tongue are most commonly affected site. There is no definite etiology for BMS other than the precipitating causative factors, and it is still considered idiopathic. Various treatment options like use of benzodiazepine, anti-depressants, analgesics, capsaicin, alpha lipoic acids, and cognitive behavioral therapy are found to be effective, but definite treatment is still unknown. The present article discusses some of the recent concepts of etiopathogenesis of BMS as well as the role of pharmacotherapeutic management in this disorder.

  10. Probabilistic risk model for staphylococcal intoxication from pork-based food dishes prepared in food service establishments in Korea.

    Science.gov (United States)

    Kim, Hyun Jung; Griffiths, Mansel W; Fazil, Aamir M; Lammerding, Anna M

    2009-09-01

    Foodborne illness contracted at food service operations is an important public health issue in Korea. In this study, the probabilities for growth of, and enterotoxin production by, Staphylococcus aureus in pork meat-based foods prepared in food service operations were estimated by the Monte Carlo simulation. Data on the prevalence and concentration of S. aureus as well as compliance to guidelines for time and temperature controls during food service operations were collected. The growth of S. aureus was initially estimated by using the U.S. Department of Agriculture's Pathogen Modeling Program. A second model based on raw pork meat was derived to compare cell number predictions. The correlation between toxin level and cell number as well as minimum toxin dose obtained from published data was adopted to quantify the probability of staphylococcal intoxication. When data gaps were found, assumptions were made based on guidelines for food service practices. Baseline risk model and scenario analyses were performed to indicate possible outcomes of staphylococcal intoxication under the scenarios generated based on these data gaps. Staphylococcal growth was predicted during holding before and after cooking, and the highest estimated concentration (4.59 log CFU/g for the 99.9th percentile value) of S. aureus was observed in raw pork initially contaminated with S. aureus and held before cooking. The estimated probability for staphylococcal intoxication was very low, using currently available data. However, scenario analyses revealed an increased possibility of staphylococcal intoxication when increased levels of initial contamination in the raw meat, andlonger holding time both before and after cooking the meat occurred.

  11. Anti-inflammatory and organ protective effect of insulin in scalded MODS rats without controlling hyperglycemia.

    Science.gov (United States)

    Zhu, Zhongzhen; Hu, Tian; Wang, Zhanke; Wang, Jin; Liu, Rui; Yang, Qianyong; Zhang, Xiaoyun; Xiong, Yuanyuan

    2018-02-01

    Insulin, as an anti-inflammatory drug, could not be freely used in patients who experienced trauma according to the degree of inflammation, because of the side effect of hypoglycemia. In vivo experimental evidence is lacking concerning whether the effect is dosage dependent and whether it relies on controlling hyperglycemia. By adjusting the dosage ratio of glucose and insulin, different dosages of insulin were used to treat severely scalded MODS rats to achieve uncontrolled or controlled hyperglycemia. One hundred forty rats with severe scalded were randomly divided into a hyperglycemia-controlled group, hyperglycemia-uncontrolled group, and control group. The levels of inflammation response indexes and major organ dysfunction indexes were measured and compared between groups. The blood indexes of inflammatory response and major organ dysfunction did not show statistical difference between hyperglycemia-controlled groups (A) and uncontrolled groups (B) in the same dosage of insulin (all P>0.05). The blood indexes of inflammatory response and major organ dysfunction demonstrated statistical difference in different dosages of insulin with hyperglycemia-controlled groups (A1-A3 groups) and hyperglycemia-uncontrolled groups (B1-B3 groups) (all Pcontrolling hyperglycemia. The effect of anti-inflammation and organ protection of insulin is dosage dependent in vivo; it does not rely on controlling hyperglycemia. Temporary traumatic hyperglycemia itself might not be detrimental to the body. Adjusting the ratio of insulin and glucose could provide a novel train of thought for freely treating patients with severe traumatic injury with different dosages of insulin according to the degree of inflammation. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Knowledge and Perceptions About Community-acquired Staphylococcal Infections Among Health Care Workers in Hawai‘i

    Science.gov (United States)

    Tice, Alan D; Hurwitz, Eric L; Katz, Alan R

    2013-01-01

    Since the early 1990s, national rates of methicillin-resistant Staphylococcus aureus (MRSA) infections have increased dramatically.1,2 Initially identified in health care settings, community-acquired MRSA is now a major public health concern. With Hawai‘i's expanding S. aureus and MRSA epidemic closely approximating the national trend in inpatient and outpatient settings,7,8 a high level of knowledge and awareness among health care workers is essential to successfully control this evolving epidemic. Health care and related workers were surveyed to assess their knowledge and perceptions about staphylococcal and MRSA infections. Knowledge was estimated by demonstrated ability to correctly identify risk factors including diabetes and obesity, as well as to demonstrate awareness of a growing staphylococcal and MRSA epidemic.9,10 Perceptions were estimated by level of concern of antibiotic resistance as well as of the severity of the staphylococcal and MRSA epidemic. Variations in knowledge and perception concerning basic principles associated with S. aureus infections as well as characteristics of the evolving S. aureus and MRSA epidemic were observed among various occupations (advance clinical practitioners, nurses, public health professionals, athletic trainers, and non-medical workers) as well as work locations (hospital, community, and non-clinical community). Overall, health care and related workers in community settings demonstrated disparities in knowledge regarding S. aureus and MRSA infections. They were also more likely to misperceive this growing threat. These findings provide support for focused educational interventions targeting community health care and related workers to improve awareness of staphylococcal infections in order to successfully address and combat this evolving epidemic. PMID:24069572

  13. Knowledge and perceptions about community-acquired staphylococcal infections among health care workers in Hawai'i.

    Science.gov (United States)

    Dunn, Brandyn S; Tice, Alan D; Hurwitz, Eric L; Katz, Alan R

    2013-09-01

    Since the early 1990s, national rates of methicillin-resistant Staphylococcus aureus (MRSA) infections have increased dramatically.1,2 Initially identified in health care settings, community-acquired MRSA is now a major public health concern. With Hawai'i's expanding S. aureus and MRSA epidemic closely approximating the national trend in inpatient and outpatient settings,7,8 a high level of knowledge and awareness among health care workers is essential to successfully control this evolving epidemic. Health care and related workers were surveyed to assess their knowledge and perceptions about staphylococcal and MRSA infections. Knowledge was estimated by demonstrated ability to correctly identify risk factors including diabetes and obesity, as well as to demonstrate awareness of a growing staphylococcal and MRSA epidemic.9,10 Perceptions were estimated by level of concern of antibiotic resistance as well as of the severity of the staphylococcal and MRSA epidemic. Variations in knowledge and perception concerning basic principles associated with S. aureus infections as well as characteristics of the evolving S. aureus and MRSA epidemic were observed among various occupations (advance clinical practitioners, nurses, public health professionals, athletic trainers, and non-medical workers) as well as work locations (hospital, community, and non-clinical community). Overall, health care and related workers in community settings demonstrated disparities in knowledge regarding S. aureus and MRSA infections. They were also more likely to misperceive this growing threat. These findings provide support for focused educational interventions targeting community health care and related workers to improve awareness of staphylococcal infections in order to successfully address and combat this evolving epidemic.

  14. Imerslund-Grasbeck syndrome in a Chinese family with distinct skin lesions refractory to vitamin B12.

    OpenAIRE

    Lin, S H; Sourial, N A; Lu, K C; Hsueh, E J

    1994-01-01

    Two brothers in a Chinese family with selective malabsorption of vitamin B12 associated with proteinuria (Imerslund-Grasbeck syndrome) presented with widespread mottled skin pigmentation, termed poikiloderma. In contrast to anaemia, this pigmentary disturbance remained unresponsive to vitamin B12 replacement. This is different from the reported hyperpigmentation sometimes seen in vitamin B12 deficiency which is reversible following treatment. As far as is known, an irreversible and persistent...

  15. McCune Albright syndrome - association of fibrous dysplasia, café-au-lait skin spots and hyperthyroidism - case report.

    Science.gov (United States)

    Raus, Iulian; Coroiu, Roxana Elena

    2016-01-01

    McCune-Albright syndrome is a rare sporadic disease characterized by bone fibrous dysplasia, café-au-lait skin spots and a variable association of hyperfunctional endocrine disorders. Fibrous dysplasia (FD), which can involve the craniofacial, axial, and appendicular skeleton, may range from an isolated, asymptomatic monostotic lesion to a severe disabling polyostotic disease involving the entire skeleton. A twenty-five-year old male patient presented to our clinic with recently developed heart palpitations. He had also been feeling pain in the right femur since he was younger, without any trauma history, leading to difficulties of ambulation and limping occasionally. His physical examination revealed café-au-lait spots with irregular borders and right testicular agenesis. Laboratory findings identified hyperthyroidism with hyperparathyroidism. Radiographs of the pelvis revealed multiple lytic lesions of the right femur and magnetic resonance imaging (MRI) characterized these lesions as specific to fibrous dysplasia of the bone, without any insufficiency fracture at this level. The association of café-au-lait skin spots with bone fibrous dysplasia, and hyperthyroidism in this patient suggested the diagnosis of McCune - Albright syndrome.

  16. SNEDDON’S SYNDROME

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    Valentin Valtchev

    2008-10-01

    Full Text Available Sneddon’s syndrome is usually characterized by the association of an ischemic cerebrovascular disease and a widespread livedo reticularis. The incidence of Sneddon syndrome is 4/1000 000. We present 42-year-old woman with livedo reticularis, recurrence ischaemic cerebrovascular accidents, two repetitive miscarriages and positive anti-2GPi antibodies. Skin biopsy specimens reveal inflammatory changes of small- to medium-sized arteries and subendothelial proliferation and fibrosis. The diagnosis Sneddon syndrome is confirmed by skin biopsy, and MR evidence. We suggest that anti-2GPi antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with Sneddon syndrome.

  17. Basis of Virulence in Enterotoxin-Mediated Staphylococcal Food Poisoning

    Directory of Open Access Journals (Sweden)

    Emilie L. Fisher

    2018-03-01

    Full Text Available The Staphylococcus aureus enterotoxins are a superfamily of secreted virulence factors that share structural and functional similarities and possess potent superantigenic activity causing disruptions in adaptive immunity. The enterotoxins can be separated into two groups; the classical (SEA-SEE and the newer (SEG-SElY and counting enterotoxin groups. Many members from both these groups contribute to the pathogenesis of several serious human diseases, including toxic shock syndrome, pneumonia, and sepsis-related infections. Additionally, many members demonstrate emetic activity and are frequently responsible for food poisoning outbreaks. Due to their robust tolerance to denaturing, the enterotoxins retain activity in food contaminated previously with S. aureus. The genes encoding the enterotoxins are found mostly on a variety of different mobile genetic elements. Therefore, the presence of enterotoxins can vary widely among different S. aureus isolates. Additionally, the enterotoxins are regulated by multiple, and often overlapping, regulatory pathways, which are influenced by environmental factors. In this review, we also will focus on the newer enterotoxins (SEG-SElY, which matter for the role of S. aureus as an enteropathogen, and summarize our current knowledge on their prevalence in recent food poisoning outbreaks. Finally, we will review the current literature regarding the key elements that govern the complex regulation of enterotoxins, the molecular mechanisms underlying their enterotoxigenic, superantigenic, and immunomodulatory functions, and discuss how these activities may collectively contribute to the overall manifestation of staphylococcal food poisoning.

  18. Structure of the superantigen staphylococcal enterotoxin B in complex with TCR and peptide-MHC demonstrates absence of TCR-peptide contacts.

    Science.gov (United States)

    Rödström, Karin E J; Elbing, Karin; Lindkvist-Petersson, Karin

    2014-08-15

    Superantigens are immune-stimulatory toxins produced by Staphylococcus aureus, which are able to interact with host immune receptors to induce a massive release of cytokines, causing toxic shock syndrome and possibly death. In this article, we present the x-ray structure of staphylococcal enterotoxin B (SEB) in complex with its receptors, the TCR and MHC class II, forming a ternary complex. The structure, in combination with functional analyses, clearly shows how SEB adopts a wedge-like position when binding to the β-chain of TCR, allowing for an interaction between the α-chain of TCR and MHC. Furthermore, the binding mode also circumvents contact between TCR and the peptide presented by MHC, which enables SEB to initiate a peptide-independent activation of T cells. Copyright © 2014 by The American Association of Immunologists, Inc.

  19. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) with cranial vault plasmocytoma and the role of surgery in its management: a case report

    OpenAIRE

    Plata Bello, Julio; Garcia-Marin, Victor

    2013-01-01

    Introduction POEMS syndrome (an acronym of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) is a paraneoplastic disorder related to an underlying plasma cell dyscrasia. The development of such a syndrome is rare and its association with calvarial plasmocytoma is even less common, with only two previous reported cases. We describe, in detail, an unusual presentation of cranial plasmocytoma associated with POEMS syndrome and briefly discuss the possible role of s...

  20. The SaeR/S gene regulatory system induces a pro-inflammatory cytokine response during Staphylococcus aureus infection.

    Directory of Open Access Journals (Sweden)

    Robert L Watkins

    Full Text Available Community-associated methicillin-resistant Staphylococcus aureus accounts for a large portion of the increased staphylococcal disease incidence and can cause illness ranging from mild skin infections to rapidly fatal sepsis syndromes. Currently, we have limited understanding of S. aureus-derived mechanisms contributing to bacterial pathogenesis and host inflammation during staphylococcal disease. Herein, we characterize an influential role for the saeR/S two-component gene regulatory system in mediating cytokine induction using mouse models of S. aureus pathogenesis. Invasive S. aureus infection induced the production of localized and systemic pro-inflammatory cytokines, including tumor necrosis factor alpha (TNF-α, interferon gamma (IFN-γ, interleukin (IL-6 and IL-2. In contrast, mice infected with an isogenic saeR/S deletion mutant demonstrated significantly reduced pro-inflammatory cytokine levels. Additionally, secreted factors influenced by saeR/S elicited pro-inflammatory cytokines in human blood ex vivo. Our study further demonstrated robust saeR/S-mediated IFN-γ production during both invasive and subcutaneous skin infections. Results also indicated a critical role for saeR/S in promoting bacterial survival and enhancing host mortality during S. aureus peritonitis. Taken together, this study provides insight into specific mechanisms used by S. aureus during staphylococcal disease and characterizes a relationship between a bacterial global regulator of virulence and the production of pro-inflammatory mediators.

  1. Costello syndrome

    Directory of Open Access Journals (Sweden)

    Madhukara J

    2007-01-01

    Full Text Available Costello syndrome is a rare, distinctive, multiple congenital anomaly syndrome, characterized by soft, loose skin with deep palmar and plantar creases, loose joints, distinctive coarse facial features and skeletal and cardiac abnormalities. The affected patients have a predisposition to develop malignancy, developmental delays and mental retardation. Recently, a 7-year-old male child born to normal nonconsanguineous parents presented to us with abnormal facial features, arrhythmia, mitral valve dysfunction and growth retardation. His cutaneous examination revealed lax and pigmented skin over hands and feet with deep creases, acanthosis nigricans and short curly hairs. Its differentiation from other syndromes with similar clinical features is discussed in this article.

  2. Molecular Typing of Staphylococcus Aureus Isolate Responsible for Staphylococcal Poisoning Incident in Homemade Food.

    Science.gov (United States)

    Macori, Guerrino; Bellio, Alberto; Bianchi, Daniela Manila; Gallina, Silvia; Adriano, Daniela; Zuccon, Fabio; Chiesa, Francesco; Acutis, Pier Luigi; Casalinuovo, Francesco; Decastelli, Lucia

    2016-04-19

    In October 2012, two persons fell ill with symptoms consistent with staphylococcal food poisoning after eating home-canned tuna fish and tomatoes. Laboratory investigation detected the enterotoxins in the home-canned tuna and molecular analysis of the isolated Staphylococcus aureus confirmed it carried toxin genes. Qualitative enzyme-linked immunosorbent assay and enzime linked fluorescent assay methods and quantitative assay identified the enterotoxins in the food leftovers, specifically staphylococcal enterotoxins type A (SEA) and D (SED), respectively 0.49 and 2.04 ng/g. The laboratory results are discussed considering the relation to the fish in oil, survival and heat resistance of S. aureus , and presumptive microbial contamination due to improper handling during home-canning procedures. This is the first reported cluster of foodborne illnesses due to staphylococcal enterotoxins in tuna in Italy. In this study, we reported cases described and analysed for their spa -type. Showing a high heterogeneity of isolates, spa -type t 13252 is correlated in a node of the minimum spanning tree and it has never been reported as responsible for foodborne outbreak. This case underlines the importance of risk communication and dissemination of home-canning guidelines to reduce the incidence of foodborne outbreaks caused by homemade conserves.

  3. Molecular typing of Staphylococcus aureus isolate responsible for staphylococcal poisoning incident in homemade food

    Directory of Open Access Journals (Sweden)

    Guerrino Macori

    2016-06-01

    Full Text Available In October 2012, two persons fell ill with symptoms consistent with staphylococcal food poisoning after eating home-canned tuna fish and tomatoes. Laboratory investigation detected the enterotoxins in the home-canned tuna and molecular analysis of the isolated Staphylococcus aureus confirmed it carried toxin genes. Qualitative enzyme-linked immunosorbent assay and enzime linked fluorescent assay methods and quantitative assay identified the enterotoxins in the food leftovers, specifically staphylococcal enterotoxins type A (SEA and D (SED, respectively 0.49 and 2.04 ng/g. The laboratory results are discussed considering the relation to the fish in oil, survival and heat resistance of S. aureus, and presumptive microbial contamination due to improper handling during home-canning procedures. This is the first reported cluster of foodborne illnesses due to staphylococcal enterotoxins in tuna in Italy. In this study, we reported cases described and analysed for their spa-type. Showing a high heterogeneity of isolates, spa-type t13252 is correlated in a node of the minimum spanning tree and it has never been reported as responsible for foodborne outbreak. This case underlines the importance of risk communication and dissemination of home-canning guidelines to reduce the incidence of foodborne outbreaks caused by homemade conserves.

  4. Retrospective Analysis of Clinical and Cost Outcomes Associated with Methicillin-Resistant Staphylococcus aureus Complicated Skin and Skin Structure Infections Treated with Daptomycin, Vancomycin, or Linezolid

    Directory of Open Access Journals (Sweden)

    Bradley M. Wright

    2011-01-01

    Full Text Available Objective. The objective of this analysis was to compare clinical and cost outcomes associated with patients who had suspected or documented methicillin-resistant Staphylococcus aureus (MRSA infections treated with daptomycin, vancomycin, or linezolid in complicated skin and skin structure infections (cSSSIs. Design. This was a retrospective analysis conducted from February to June of 2007. Appropriate data was collected, collated, and subsequently evaluated with the purpose of quantifying length of stay, antibiotic therapy duration, clinical cure rates, adverse drug events, and cost of hospitalization. Results. All 82 patients included in the analysis experienced clinical cure. The duration of antibiotic therapy was similar among the three groups yet the length of hospitalization was slightly shorter in the daptomycin group. Conclusions. The incidence of resistant staphylococcal infections is increasing; therefore, judicious use of MRSA active agents is paramount. Future studies are necessary to determine if MRSA treatment options can be stratified based on the severity of the infectious process.

  5. Prevalence of Staphylococcal Enterotoxins in Ready-to-Eat Foods Sold in Istanbul.

    Science.gov (United States)

    Ulusoy, Beyza H; Çakmak Sancar, Burcu; Öztürk, Muhsin

    2017-10-01

    The aim of this study was to investigate the prevalence of staphylococcal enterotoxins (SEs) in ready-to-eat (RTE) foods sold in Istanbul, Turkey. A total of 5,241 samples were randomly collected from various caterers, hotels, and restaurants from 2014 to 2016. The samples were classified into four groups: (i) various cooked RTE meat and vegetable meals, (ii) various RTE salads, charcuterie, and cold appetizers, (iii) various cooked RTE bakery products (pasta, pastries, pizza, pita, ravioli, etc.), and (iv) any cooked RTE sweets and desserts (pudding, custard, cream, ashura, etc.). The samples were examined for the presence of SEs by 3M Tecra Staph Enterotoxin Visual Immunoassay method, which is a manual enzyme-linked immunosorbent assay method. Among all samples, only 1 (0.019%) RTE meal (vegetable meal with meat) was found to be contaminated with SEs, a good result in terms of staphylococcal food poisoning risk and public health.

  6. Staphylococcal endogenous endophthalmitis in association with pyogenic vertebral osteomyelitis.

    Science.gov (United States)

    Steeples, L R; Jones, N P

    2016-01-01

    PURPOSE To describe pyogenic vertebral osteomyelitis as a rare infection associated with endogenous endophthalmitis.METHODS A retrospective review of three patients with endogenous endophthalmitis and sepsis due to underlying Staphylococcal vertebral osteomyelitis presenting during a 21-month time period. The ophthalmic and systemic features and management and outcomes are presented.RESULTS One patient developed unilateral endophthalmitis with cervical spine osteomyelitis, Staphylococcus aureus being isolated from blood cultures. The second presented with bilateral endophthalmitis with disseminated Methicillin-resistant S. aureus (MRSA) infection, with thoracic and lumbar discitis and para-spinal abscesses. MRSA was cultured from vitreous, blood, and synovial fluid. Both patients received prolonged courses of intravenous antibiotics. Intravitreal antibiotic therapy was used in the second patient. Excellent visual and systemic outcomes were achieved in both cases with no ocular complications. The third patient developed lumbar osteomyelitis following spinal surgery and presented with disseminated S. aureus sepsis including unilateral endogenous endophthalmitis. Despite systemic antibiotics and intensive care the patient died.CONCLUSIONS Endogenous endophthalmitis should be suspected in septic patients developing eye symptoms. Endogenous endophthalmitis with staphylococcal bone infection is a rare but serious condition. Osteomyelitis should be considered as an infective source in any such patient reporting bone pain or reduced spinal mobility. Prompt investigation and treatment can achieve favourable visual and systemic outcomes.

  7. [Borderline structure and painful skin hemorrhages: a case of Gardner-Diamond syndrome].

    Science.gov (United States)

    Plassmann, R

    1985-01-01

    The Gardner-Diamond Syndrome is an infrequently diagnosed impressive psychosomatosis. The patients (exclusively female) suffering from this disorder are described as "angry young women". They are considered hysterical, masochistic, depressive, hostile and timid. Physically the syndrome is characterized by atypical, painful, apparently spontaneous skin bleeding in young women and these patients look like they have been beaten, injured or are seriously ill. Besides hematoma, renal hemorrhage, uterine hemorrhage, headaches or unconsciousness as well as numerous symptoms of conversion in the narrower sense of the word have been observed, e.g. loss speech, gait deviation, or feelings of discomfort. To this day the pathophysiological peculiarity that enables the patients to transform subconscious psychic events into the specific temporary alteration in the permeability of the capillary walls being a constitutional variant characteristic of females suffering from Gardner-Diamond Syndrome and activated by psychic stimuli. In the case history presented, the problem is the psychic working out of narcissistic injuries in the context of a borderline structure. These injuries turn into physical symptoms analogous to the process of conversion. Since, in this case, there is no question of a defense against oedipal fantasies one would prefer to speak of a "narcissistic conversion". Quite peculiar is the universally described resemblance of the patients to one another. The syndrome, together with the character and psychodynamic features, is repeated in almost a stereotypical manner. An explanation for this phenomenon cannot be expected even from the discovery of a pathophysiological detail in the blood vessels. Rather, we are confronted with one of those extremely rare syndromes where, in a limited field, physical and psychic events are completely blended. The physical events such as affects and defense mechanisms for which they can be substituted at random and without mutual

  8. Two QTL characterized for soft scald and soggy breakdown in apple (Malus × domestica) through pedigree-based analysis of a large population of interconnected families

    NARCIS (Netherlands)

    Howard, Nicholas P.; Weg, van de Eric; Tillman, John; Tong, Cindy B.S.; Silverstein, Kevin A.T.; Luby, James J.

    2018-01-01

    Soft scald and soggy breakdown are important postharvest physiological disorders of apple (Malus × domestica). ‘Honeycrisp’ and some of its offspring are particularly susceptible to developing these disorders. The purpose of this study was to identify molecular markers associated with high

  9. Deficient plakophilin-1 expression due to a mutation in PKP1 causes ectodermal dysplasia-skin fragility syndrome in Chesapeake Bay retriever dogs.

    Directory of Open Access Journals (Sweden)

    Thierry Olivry

    Full Text Available In humans, congenital and hereditary skin diseases associated with epidermal cell-cell separation (acantholysis are very rare, and spontaneous animal models of these diseases are exceptional. Our objectives are to report a novel congenital acantholytic dermatosis that developed in Chesapeake Bay retriever dogs. Nine affected puppies in four different litters were born to eight closely related clinically normal dogs. The disease transmission was consistent with an autosomal recessive mode of inheritance. Clinical signs occurred immediately after birth with superficial epidermal layers sloughing upon pressure. At three month of age, dogs exhibited recurrent superficial skin sloughing and erosions at areas of friction and mucocutaneous junctions; their coat was also finer than normal and there were patches of partial hair loss. At birth, histopathology revealed severe suprabasal acantholysis, which became less severe with ageing. Electron microscopy demonstrated a reduced number of partially formed desmosomes with detached and aggregated keratin intermediate filaments. Immunostaining for desmosomal adhesion molecules revealed a complete lack of staining for plakophilin-1 and anomalies in the distribution of desmoplakin and keratins 10 and 14. Sequencing revealed a homozygous splice donor site mutation within the first intron of PKP1 resulting in a premature stop codon, thereby explaining the inability to detect plakophilin-1 in the skin. Altogether, the clinical and pathological findings, along with the PKP1 mutation, were consistent with the diagnosis of ectodermal dysplasia-skin fragility syndrome with plakophilin-1 deficiency. This is the first occurrence of ectodermal dysplasia-skin fragility syndrome in an animal species. Controlled mating of carrier dogs would yield puppies that could, in theory, be tested for gene therapy of this rare but severe skin disease of children.

  10. Turner Syndrome

    Science.gov (United States)

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

  11. Inducible satellite cell depletion attenuates skeletal muscle regrowth following a scald-burn injury.

    Science.gov (United States)

    Finnerty, Celeste C; McKenna, Colleen F; Cambias, Lauren A; Brightwell, Camille R; Prasai, Anesh; Wang, Ye; El Ayadi, Amina; Herndon, David N; Suman, Oscar E; Fry, Christopher S

    2017-11-01

    Severe burns result in significant skeletal muscle cachexia that impedes recovery. Activity of satellite cells, skeletal muscle stem cells, is altered following a burn injury and likely hinders regrowth of muscle. Severe burn injury induces satellite cell proliferation and fusion into myofibres with greater activity in muscles proximal to the injury site. Conditional depletion of satellite cells attenuates recovery of myofibre area and volume following a scald burn injury in mice. Skeletal muscle regrowth following a burn injury requires satellite cell activity, underscoring the therapeutic potential of satellite cells in the prevention of prolonged frailty in burn survivors. Severe burns result in profound skeletal muscle atrophy; persistent muscle atrophy and weakness are major complications that hamper recovery from burn injury. Many factors contribute to the erosion of muscle mass following burn trauma, and we have previously shown concurrent activation and apoptosis of muscle satellite cells following a burn injury in paediatric patients. To determine the necessity of satellite cells during muscle recovery following a burn injury, we utilized a genetically modified mouse model (Pax7 CreER -DTA) that allows for the conditional depletion of satellite cells in skeletal muscle. Additionally, mice were provided 5-ethynyl-2'-deoxyuridine to determine satellite cell proliferation, activation and fusion. Juvenile satellite cell-wild-type (SC-WT) and satellite cell-depleted (SC-Dep) mice (8 weeks of age) were randomized to sham or burn injury consisting of a dorsal scald burn injury covering 30% of total body surface area. Both hindlimb and dorsal muscles were studied at 7, 14 and 21 days post-burn. SC-Dep mice had >93% depletion of satellite cells compared to SC-WT (P satellite cell proliferation and fusion. Depletion of satellite cells impaired post-burn recovery of both muscle fibre cross-sectional area and volume (P satellite cells in the aetiology of lean

  12. Genetics of Skin Cancer (PDQ®)—Health Professional Version

    Science.gov (United States)

    Genetics of Skin Cancer includes information about genes and hereditary syndromes associated with basal cell, squamous cell, and melanoma skin cancer. Get comprehensive information about the genetics of skin cancer and interventions in this summary for clinicians.

  13. Novel TGM5 mutations in acral peeling skin syndrome.

    Science.gov (United States)

    van der Velden, Jaap J A J; van Geel, Michel; Nellen, Ruud G L; Jonkman, Marcel F; McGrath, John A; Nanda, Arti; Sprecher, Eli; van Steensel, Maurice A M; McLean, W H Irwin; Cassidy, Andrew J

    2015-04-01

    Acral peeling skin syndrome (APSS, MIM #609796) is a rare autosomal recessive disorder characterized by superficial exfoliation and blistering of the volar and dorsal aspects of hands and feet. The level of separation is at the junction of the stratum granulosum and stratum corneum. APSS is caused by mutations in the TGM5 gene encoding transglutaminase-5, which is important for structural integrity of the outermost epidermal layers. The majority of patients originate from Europe and carry a p.(Gly113Cys) mutation in TGM5. In this study, we report both European and non-European families carrying other mutations in the TGM5 gene. In 5 patients, we found 3 novel mutations: c.1001+2_1001+3del, c.1171G>A and c.1498C>T. To confirm their pathogenicity, we performed functional analyses with a transglutaminase activity assay, determined alternative splicing by reverse-transcribed PCR analysis and used databases and in silico prediction tools. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  14. miR-29b promotes skin wound healing and reduces excessive scar formation by inhibition of the TGF-β1/Smad/CTGF signaling pathway.

    Science.gov (United States)

    Guo, Jingdong; Lin, Quan; Shao, Ying; Rong, Li; Zhang, Duo

    2017-04-01

    The hypertrophic scar is a medical difficulty of humans, which has caused great pain to patients. Here, we investigated the inhibitory effect of miR-29b on scar formation. The scalded model was established in mice and miR-29b mimics or a negative control was subcutaneously injected into the injury skin. Then various molecular biological experiments were performed to assess the effect of miR-29b on scar formation. According to our present study, first, the results demonstrated that miR-29b was down-regulated in thermal injury tissue and miR-29b treatment could promote wound healing, inhibit scar formation, and alleviate histopathological morphologic alteration in scald tissues. Additionally, miR-29b treatment suppressed collagen deposition and fibrotic gene expression in scar tissues. Finally, we found that miR-29b treatment inhibited the TGF-β1/Smad/CTGF signaling pathway. Taken together, our data suggest that miR-29b treatment has an inhibitory effect against scar formation via inhibition of the TGF-β1/Smad/CTGF signaling pathway and may provide a potential molecular basis for future treatments for hypertrophic scars.

  15. Bacterial populations associated with the dirty area of a South African poultry abattoir.

    Science.gov (United States)

    Geornaras, I; de Jesus, A E; von Holy, A

    1998-06-01

    Bacterial populations associated with three sample types from the neck region of poultry carcasses in the dirty area of an abattoir were characterized. Sample types before and after scalding were skin only, feathers only, and a skin and feather combination. The neck skin of carcasses after the defeathering processing stage was also sampled. Bacterial populations associated with water from the scald tank, rubber fingers at the exit of the defeathering machine, and air in the dirty area were also characterized. Bacterial colonies (751) were randomly isolated from yeast extract-supplemented tryptone soya agar plates exhibiting 30 to 300 colonies. Micrococcus spp. were isolated in the highest proportion from pre-and postscalded carcass samples (63.5 to 86.1% of isolates), regardless of the sample type. Conversely, Enterobacteriaceae (40.3%), Acinetobacter (19.4%), and Aeromonas/Vibrio (12.5%) species predominated on neck skin samples taken from mechanically defeathered carcasses. Isolates from the rubber fingers were, however, predominantly Micrococcus spp. (94.4%). Bacterial groups isolated in the highest proportion from scald tank water samples were Micrococcus spp. (38.3%), species of Enterobacteriaceae (29.1%), and lactic acid bacteria (17.0%). Corynebacterium spp., species of Enterobacteriaceae, and Micrococcus spp. were dominant on air settle plates.

  16. Pediatric Toxic Shock Syndrome

    Directory of Open Access Journals (Sweden)

    Jennifer Yee

    2017-09-01

    Full Text Available Audience: This scenario was developed to educate emergency medicine residents on the diagnosis and management of a pediatric patient with toxic shock syndrome. The case is also appropriate for teaching of medical students and advanced practice providers, as well as a review of the principles of crisis resource management, teamwork, and communication. Introduction: Toxic shock syndrome is a low-frequency, high-acuity scenario requiring timely identification and aggressive management. If patients suffering from this condition are managed incorrectly, they may progress into multi-organ dysfunction and potentially death. Toxic shock syndrome has been associated with Streptococcus and Staphylococcus aureus (Staph. Approximately half of Staph cases are associated with menstruation, which was first described in the 1970s-1980s and was associated with the use of absorbent tampons.1 Group A Streptococcus may cause complications such as necrotizing fasciitis and gangrenous myositis.2 Pediatric patients may present critically ill from toxic shock syndrome. Providers need to perform a thorough history and physical exam to discern the source of infection. Management requires aggressive care with antibiotics and IV fluids. Objectives: By the end of this simulation session, the learner will be able to: 1 Recognize toxic shock syndrome. 2 Review the importance of a thorough physical exam. 3 Discuss management of toxic shock syndrome, including supportive care and the difference in antibiotic choices for streptococcal and staphylococcal toxic shock syndrome. 4 Appropriately disposition a patient suffering from toxic shock syndrome. 5 Communicate effectively with team members and nursing staff during a resuscitation of a critically ill patient. Method: This session was conducted using high-fidelity simulation, followed by a debriefing session and lecture on toxic shock syndrome.

  17. Nosocomial coagulase-negative staphylococcal infections in bone marrow transplantation recipients with central vein catheter. A 5-year prospective study.

    Science.gov (United States)

    Engelhard, D; Elishoov, H; Strauss, N; Naparstek, E; Nagler, A; Simhon, A; Raveh, D; Slavin, S; Or, R

    1996-02-15

    The purpose of this study was to examine coagulase-negative staphylococcal infections in bone marrow transplantation (BMT) patients with central vein catheters by investigating incidence, clinical relevance, risk factors, methicillin resistance, clinical impact of initial empiric antimicrobial therapy without vancomycin, and management of documented catheter-related infections. A 5-year prospective study was conducted with daily evaluation of 242 BMT patients during hospitalization, including clinical assessment and blood culture via the Hickman/Broviac catheter. If fever or infected appearance occurred, peripheral blood cultures or exit site cultures, respectively, were done. Results showed a septicemia incidence of 7.0%, including in 6 patients following colonization, in 1 patient with tunnel infection, in 1 patient with thrombophlebitis, in 1 patient with exit site infection, and in 8 patients with septicemia of unknown origin. Total colonization incidence was 7%, with colonization only in 11 patients who had 16 episodes; incidence of exit site infection was 3.7%. Age > or = 18 years was the only identified risk factor for developing staphylococcal infection (P = 0.03). Despite a methicillin resistance rate of 45% and omission of vancomycin from the routine initial empiric antimicrobial regimen, the clinical course of coagulase-negative staphylococcal infections was relatively benign. A single patient, who experienced marrow rejection, died on day +31 with septicemia and only one patient experienced microbiological failure with recurrent colonization. Bacteria grown in both aerobic and anaerobic bottles were more likely true bacteremia than contaminant (P = 0.03). We conclude that the hazard of coagulase-negative staphylococcal infection does not mandate inclusion of a glycopeptide in the initial empiric antimicrobial regimen in BMT patients, even during febrile neutropenia. Hickman/Broviac-related staphylococcal infections, except for tunnel infection or

  18. Genetics Home Reference: Gorlin syndrome

    Science.gov (United States)

    ... for This Condition basal cell nevus syndrome BCNS Gorlin-Goltz syndrome NBCCS nevoid basal cell carcinoma syndrome Related Information ... named? Additional Information & Resources MedlinePlus (2 links) Encyclopedia: Basal Cell Nevus Syndrome Health Topic: Skin Cancer Genetic and Rare Diseases ...

  19. Analogs of human genetic skin disease in domesticated animals

    Directory of Open Access Journals (Sweden)

    Justin Finch, MD

    2017-09-01

    The genetic skin diseases we will review are pigmentary mosaicism, piebaldism, albinism, Griscelli syndrome, ectodermal dysplasias, Waardenburg syndrome, and mucinosis in both humans and domesticated animals.

  20. Lenticular disseminated dermatofibrosis and osteopoikilosis (Buschke-Ollendorf-syndrome)

    Energy Technology Data Exchange (ETDEWEB)

    Lippelt, C; Petzel, H

    1982-12-01

    Presented are 4 cases showing combined occurrence of lenticular disseminated dermatofibrosis and osteopoikilosis (Buschke-Ollendorf syndrome). Histological findings of the skin show localized increases in elastic and collageneous structures. Type I of the Buschke-Ollendorf syndrome which is characterized by disseminated small pepper-corn like changes in the skin must be differentiated from type II which is named 'dermatofibrosis nodularis xanthomatoides multilokularis' with osteopoikilosis, showing larger, single or plaque like connected changes of the skin. The Buschke-Ollendorf syndrome is a congenital autosomal dominant hereditary abnormality arising from the mutual mesodermal genesis of skin and bone changes. The Buschke-Ollendorf syndrome occasionally brings about impaired growth and mental retardation as well as rheumatoid complaints. Osteopoikilosis always appears symmetrically showing different changes in the bones usually without changes in the skin; in the contrary the lenticular disseminated dermatofibrosis is always occurring combined with findings of osteopoikilosis. During the growing years we observed patients with an increase in size and density of the bone changes and also new lesions, while the skin changes remained nearly the same.

  1. Lenticular disseminated dermatofibrosis and osteopoikilosis (Buschke-Ollendorf-syndrome)

    International Nuclear Information System (INIS)

    Lippelt, C.; Petzel, H.

    1982-01-01

    Presented are 4 cases showing combined occurrence of lenticular disseminated dermatofibrosis and osteopoikilosis (Buschke-Ollendorf syndrome). Histological findings of the skin show localized increases in elastic and collageneous structures. Type I of the Buschke-Ollendorf syndrome which is characterized by disseminated small pepper-corn like changes in the skin must be differentiated from type II which is named 'dermatodibrosis nodularis xanthomatoides multilokularis' with osteopoikilosis, showing larger, single or plaque like connected changes of the skin. The Buschke-Ollendorf syndrome is a congenital autosomal dominant hereditary abnormality arising from the mutual mesodermal genesis of skin and bone changes. The Buschke-Ollendorf syndrome occasionally brings about impaired growth and mental retardation as well as rheumatoid complaints. Osteopoikilosis always appears symmetrically showing different changes in the bones usually without changes in the skin; in the contrary the lenticular disseminated dermatofibrosis is always occurring combined with findings of osteopoikilosis. During the growing years we observed patients with an increase in size and density of the bone changes and also new lesions, while the skin changes remained nearly the same. (orig.)

  2. Molecular Typing of Staphylococcus aureus Isolated from Patients with Autosomal Dominant Hyper IgE Syndrome

    Directory of Open Access Journals (Sweden)

    Inka Sastalla

    2017-06-01

    Full Text Available Autosomal dominant hyper IgE syndrome (AD-HIES is a primary immunodeficiency caused by a loss-of-function mutation in the Signal Transducer and Activator of Transcription 3 (STAT3. This immune disorder is clinically characterized by increased susceptibility to cutaneous and sinopulmonary infections, in particular with Candida and Staphylococcus aureus. It has recently been recognized that the skin microbiome of patients with AD-HIES is altered with an overrepresentation of certain Gram-negative bacteria and Gram-positive staphylococci. However, these alterations have not been characterized at the species- and strain-level. Since S. aureus infections are influenced by strain-specific expression of virulence factors, information on colonizing strain characteristics may provide insights into host-pathogen interactions and help guide management strategies for treatment and prophylaxis. The aim of this study was to determine whether the immunodeficiency of AD-HIES selects for unique strains of colonizing S. aureus. Using multi-locus sequence typing (MLST, protein A (spa typing, and PCR-based detection of toxin genes, we performed a detailed analysis of the S. aureus isolates (n = 13 found on the skin of twenty-one patients with AD-HIES. We found a low diversity of sequence types, and an abundance of strains that expressed methicillin resistance, Panton-Valentine leukocidin (PVL, and staphylococcal enterotoxins K and Q (SEK, SEQ. Our results indicate that patients with AD-HIES may often carry antibiotic-resistant strains that harbor key virulence factors.

  3. Mupirocin in the Treatment of Staphylococcal Infections in Chronic Rhinosinusitis: A Meta-Analysis.

    Directory of Open Access Journals (Sweden)

    Jong Seung Kim

    Full Text Available Saline irrigation of the nasal cavity is a classic and effective treatment for acute or chronic rhinosinusitis. Topical antibiotics such as mupirocin have been widely used for recalcitrant chronic rhinosinusitis. Therefore, the purpose of this study was to evaluate the effect of saline irrigation using mupirocin.A systematic literature review and meta-analysis of mupirocin saline irrigation were performed using EMBASE, MEDLINE, and Cochrane library through December 2015. Data were analyzed with R 3.2.2 software. A random effects model was used because of the diversity of included studies. Sensitivity analysis of particular tested groups and single proportion tests were also performed. The main outcome measure was residual staphylococcal infection, as confirmed by culture or PCR.Two RCTs, two prospective studies and two retrospective studies were included. A random effects model meta-analysis of the pooled data identified a relative risk of residual infection of 0.13 (95% CI: 0.06-0.26, p<0.05 with low heterogeneity (I2 = 0%. The proportion of residual staphylococcal infections after 1 month was 0.08 (95% CI: 0.04-0.16. However, this proportion increased to 0.53 at 6 months (95% CI: 0.27-0.78.The short-term use of mupirocin has a strongly reductive effect on staphylococcal infection in chronic rhinosinusitis. Although there is currently a lack of clear evidence, future studies with well-designed inclusion criteria and randomized controlled trials are needed to examine mupirocin's long-term effect on chronic rhinosinusitis.

  4. The rise of methicillin resistant Staphylococcus aureus: now the dominant cause of skin and soft tissue infection in Central Australia.

    Science.gov (United States)

    Macmorran, E; Harch, S; Athan, E; Lane, S; Tong, S; Crawford, L; Krishnaswamy, S; Hewagama, S

    2017-10-01

    This study aimed to examine the epidemiology and treatment outcomes of community-onset purulent staphylococcal skin and soft tissue infections (SSTI) in Central Australia. We performed a prospective observational study of patients hospitalised with community-onset purulent staphylococcal SSTI (n = 160). Indigenous patients accounted for 78% of cases. Patients were predominantly young adults; however, there were high rates of co-morbid disease. Community-associated methicillin-resistant Staphylococcus aureus (CA-MRSA) was the dominant phenotype, accounting for 60% of cases. Hospitalisation during the preceding 6 months, and haemodialysis dependence were significant predictors of CA-MRSA infection on univariate analysis. Clinical presentation and treatment outcomes were found to be comparable for methicillin-susceptible S. aureus (MSSA) and methicillin-resistant cases. All MRSA isolates were characterised as non-multi-resistant, with this term used interchangeably with CA-MRSA in this analysis. We did not find an association between receipt of an active antimicrobial agent within the first 48 h, and progression of infection; need for further surgical debridement; unplanned General Practitioner or hospital re-presentation; or need for further antibiotics. At least one adverse outcome was experienced by 39% of patients. Clindamycin resistance was common, while rates of trimethoprim-sulfamethoxazole resistance were low. This study suggested the possibility of healthcare-associated transmission of CA-MRSA. This is the first Australian report of CA-MRSA superseding MSSA as the cause of community onset staphylococcal SSTI.

  5. [Effects of astragalus polysaccharide on intestinal immune function of rats with severe scald injury].

    Science.gov (United States)

    Huang, Cuilan; Zhan, Jianhua; Luo, Jinhua

    2015-02-01

    To observe the effects of astragalus polysaccharide (AP) on the intestinal mucosal morphology, level of secretory IgA (s-IgA) in intestinal mucus, and distribution of T lymphocyte subsets in Peyer's patch in rats with severe scald injury. One hundred and thirty SD rats were divided into sham injury group (SI, sham injured, n = 10), scald group (S, n = 30), low dosage group (LD, n = 30), moderate dosage group (MD, n = 30), and high dosage group (HD, n = 30) according to the random number table. Rats in the latter 4 groups were inflicted with 30% TBSA full-thickness scald on the back. From post injury hour 2, rats in groups LD, MD, and HD were intraperitoneally injected with 0.5 mL AP solution with the dosage of 100, 200, and 300 mg/kg each day respectively, and rats in group S were injected with 0.5 mL normal saline instead. Ten rats from group SI immediately after injury and 10 rats from each of the latter 4 groups on post injury day (PID) 3, 7, 14 were sacrificed, and their intestines were harvested. The morphology of ileal mucosa was examined after HE staining; the level of s-IgA in ileal mucus was determined with double-antibody sandwich ELISA method; the proportions of CD3⁺, CD4⁺, CD8⁺ T lymphocytes in Peyer's patches of intestine were determined with flow cytometer, and the proportion of CD4⁺ to CD8⁺ was calculated. Data were processed with one-way analysis of variance, analysis of variance of factorial design, and SNK test. (1) Villi in normal form and intact villus epithelial cells were observed in rats of group SI immediately after injury, while edema of villi and necrosis and desquamation of an enormous amount of villi were observed in groups with scalded rats on PID 3, with significant infiltration of inflammatory cells. On PID 7, no obvious improvement in intestinal mucosal lesion was observed in groups with scalded rats. On PID 14, the pathology in intestinal mucosa of rats remained nearly the same in group S, and it was alleviated obviously

  6. Skin Lesions in European Hibernating Bats Associated with Geomyces destructans, the Etiologic Agent of White-Nose Syndrome

    OpenAIRE

    Wibbelt, Gudrun; Puechmaille, S?bastien J.; Ohlendorf, Bernd; M?hldorfer, Kristin; Bosch, Thijs; G?rf?l, Tam?s; Passior, Karsten; Kurth, Andreas; Lacremans, Daniel; Forget, Fr?d?ric

    2013-01-01

    White-nose syndrome (WNS) has claimed the lives of millions of hibernating insectivorous bats in North America. Its etiologic agent, the psychrophilic fungus Geomyces destructans, causes skin lesions that are the hallmark of the disease. The fungal infection is characterized by a white powdery growth on muzzle, ears and wing membranes. While WNS may threaten some species of North American bats with regional extinction, infection in hibernating bats in Europe seems not to be associated with si...

  7. ARE THE SPIDER ANGIOMAS SKIN MARKERS OF HEPATOPULMONARY SYNDROME?

    Directory of Open Access Journals (Sweden)

    Americo de Oliveira SILVERIO

    2013-09-01

    Full Text Available Context Hepatopathies can significantly influence both veins and arteries, these changes may cause some cutaneous stigmas, such as spider angioma (SA and some systemic vascular changes, such as those observed in hepatopulmonary syndrome (HPS. Based on this common pathophysiological root we can assume that the SA can be skin markers of HPS. Objective The objective of this study is to assess whether there is a relationship between the presence of SA and HPS. Methods Records of 40 patients with liver cirrhosis who underwent contrast echocardiography were evaluated, in which we researched the description of SA, physical examination, and other clinical and laboratory data. For diagnosis of HPS we use these signs of the disease: presence of liver disease (cirrhosis in the case, abnormalities in gas exchange by arterial blood gases, and evidence of pulmonary vasodilations by the contrast echocardiography. Results The SA were found in 21/40 (52.5% patients and hepatopulmonary syndrome in 9/40 (22.5%. The HPS was observed in 8/21 (38.1% of patients with SA and 1/19 (5.3% patients were without this sign (P<0.01. We found no statistically significant difference between the SA and the presence of HPS with sex or age. Patients with SA had a higher hypoxemia [PaO2 84.8 ± 11.5 mmHg and 19.8 ± 14.7 mmHg alveolar-arterial gradient of oxygen (AAG] than those without SA (PaO2 90.8 ± 10.7 mmHg and 10.9 ± 11.7 AAG mmHg (P<0.05. Conclusion Our findings show a correlation between the presence of SA and HPS, suggesting that the SA may be cutaneous markers of HPS.

  8. The bleaching syndrome: manifestation of a post-colonial pathology ...

    African Journals Online (AJOL)

    The post-colonial root of African problems is directly related to skin color. Under the cloak of personal preference, light skin among African women has replaced dark skin as the native ideal. The aftermath is manifestation of the Bleaching Syndrome. Social Work professionals have overlooked the Bleaching Syndrome as ...

  9. Enrichment of beneficial bacteria in the skin microbiota of bats persisting with white-nose syndrome.

    Science.gov (United States)

    Lemieux-Labonté, Virginie; Simard, Anouk; Willis, Craig K R; Lapointe, François-Joseph

    2017-09-05

    Infectious diseases of wildlife are increasing worldwide with implications for conservation and human public health. The microbiota (i.e. microbial community living on or in a host) could influence wildlife disease resistance or tolerance. White-nose syndrome (WNS), caused by the fungus Pseudogymnoascus destructans (Pd), has killed millions of hibernating North American bats since 2007. We characterized the skin microbiota of naïve, pre-WNS little brown bats (Myotis lucifugus) from three WNS-negative hibernation sites and persisting, previously exposed bats from three WNS-positive sites to test the hypothesis that the skin microbiota of bats shifts following WNS invasion. Using high-throughput 16S rRNA gene sequencing on 66 bats and 11 environmental samples, we found that hibernation site strongly influenced the composition and diversity of the skin microbiota. Bats from WNS-positive and WNS-negative sites differed in alpha and beta diversity, as well as in microbiota composition. Alpha diversity was reduced in persisting, WNS-positive bats, and the microbiota profile was enriched with particular taxa such Janthinobacterium, Micrococcaceae, Pseudomonas, Ralstonia, and Rhodococcus. Some of these taxa are recognized for their antifungal activity, and specific strains of Rhodococcus and Pseudomonas are known to inhibit Pd growth. Composition of the microbial community in the hibernaculum environment and the community on bat skin was superficially similar but differed in relative abundance of some bacterial taxa. Our results are consistent with the hypothesis that Pd invasion leads to a shift in the skin microbiota of surviving bats and suggest the possibility that the microbiota plays a protective role for bats facing WNS. The detection of what appears to be enrichment of beneficial bacteria in the skin microbiota of persisting bats is a promising discovery for species re-establishment. Our findings highlight not only the potential value of management actions that

  10. [Skin and chronic kidney disease].

    Science.gov (United States)

    Rizzo, Raffaella; Mancini, Elena; Santoro, Antonio

    2014-01-01

    Kidneys and skin are seldom considered associated, but their relationship is more closer than generally believed. In some immunological diseases (SLE...) and genetic syndromes (tuberous sclerosis, Fabrys disease...) the cutaneous manifestations are integral parts of the clinical picture. In advanced uremia, besides the well-known itching skin lesions, calciphylaxis may appear, a typical example of cutaneous involvement secondary to the metabolic complications (calcium-phosphate imbalance) of the renal disease. Nephrogenic systemic fibrosis appears only in patients with renal failure and it has a very severe prognosis due to the systemic organ involvement. Moreover, there is a heterogeneous group of metabolic diseases, with renal involvement, that may be accompanied by skin lesions, either related to the disease itself or to its complications (diabetes mellitus, porphyrias). In systemic amyloidosis, fibrils may deposit even in dermis leading to different skin lesions. In some heroin abusers, in the presence of suppurative lesions in the sites of needle insertion, renal amyloidosis should be suspected, secondary to the chronic inflammation. Atheroembolic disease is nowadays frequently observed, as a consequence of the increasing number of invasive intravascular manoeuvres. Skin manifestations like livedo reticularis or the blue toe syndrome are the most typical signs, but often renal dysfunction is also present. In all these conditions, the skin lesion may be a first sign, a warning, that should arouse the suspicion of a more complex pathology, even with renal involvement. Being aware of this relationship is fundamental to accelerate the diagnostic process.

  11. Marine Collagen Peptides from the Skin of Nile Tilapia (Oreochromis niloticus): Characterization and Wound Healing Evaluation.

    Science.gov (United States)

    Hu, Zhang; Yang, Ping; Zhou, Chunxia; Li, Sidong; Hong, Pengzhi

    2017-03-30

    Burns can cause tremendous economic problems associated with irreparable harm to patients and their families. To characterize marine collagen peptides (MCPs) from the skin of Nile tilapia ( Oreochromis niloticus ), molecular weight distribution and amino acid composition of MCPs were determined, and Fourier transform infrared spectroscopy (FTIR) was used to analyze the chemical structure. Meanwhile, to evaluate the wound healing activity, in vitro and in vivo experiments were carried out. The results showed that MCPs prepared from the skin of Nile tilapia by composite enzymatic hydrolysis were composed of polypeptides with different molecular weights and the contents of polypeptides with molecular weights of less than 5 kDa accounted for 99.14%. From the amino acid composition, the majority of residues, accounting for over 58% of the total residues in MCPs, were hydrophilic. FTIR indicated that the main molecular conformations inside MCPs were random coil. In vitro scratch assay showed that there were significant effects on the scratch closure by the treatment of MCPs with the concentration of 50.0 μg/mL. In the experiments of deep partial-thickness scald wound in rabbits, MCPs could enhance the process of wound healing. Therefore, MCPs from the skin of Nile tilapia ( O. niloticus ) have promising applications in wound care.

  12. Diagnostic value of scratch-chamber test, skin prick test, histamine release and specific IgE in birch-allergic patients with oral allergy syndrome to apple

    DEFF Research Database (Denmark)

    Osterballe, M; Scheller, R; Stahl Skov, P

    2003-01-01

    BACKGROUND: The aim of the study was to examine the diagnostic value of skin prick test (SPT), scratch-chamber test (SCT), histamine release (HR) and specific immunoglobulin E (IgE) in birch-allergic patients with oral allergy syndrome to apple. METHODS: Ten birch-allergic patients with oral...... a detailed case history about symptoms of oral allergy syndrome combined with a SPT with fresh apple peel or A72 will be useful....

  13. Application of on-line nanoLC-IT-TOF in the identification of serum β-catenin complex in mice scald model.

    Directory of Open Access Journals (Sweden)

    Cheng-cai Huang

    Full Text Available Severe burn shock remains an unresolved clinical problem with an urgent need to explore novel therapeutic treatments. Intracellular β-catenin, through interaction with other proteins, has been reported to be able to regulate the size of cutaneous wounds. Higher expression of β-catenin is associated with larger sized wounds. However, the identification of serum β-catenin complex is difficult and has been rarely reported. The exploitation of more binding partners can contribute to uncovering the exact mechanisms behind serum β-catenin mediated biological effects. Here, we describe a method that consists of immunoprecipitation, SDS-PAGE, in-gel digestion, and nanoLC coupled to LCMS-IT-TOF for the investigation of serum β-catenin complex in mice scald model. Among selected gel bands obtained from the protein gels, a total of 31 peptides were identified and sequenced with high statistical significance (p<0.01. Three proteins (alpha-2-marcoglobulin, serine protease inhibitor A3K, and serine protease inhibitor A1A were identified and validated with high reliability and high reproducibility. It was inferred that these proteins might interact with serum β-catenin, which could affect the wound healing resulting from burn shock. Our study demonstrated that the on-line coupling of nano-LC with a LCMS-IT-TOF mass spectrometer was capable of sensitive and automated characterization of the serum β-catenin complex in mice scald model.

  14. Plain chest film findings of staphylococcal pneumonia in children

    International Nuclear Information System (INIS)

    Kim, Sook Young; Cha, Seong Sook; Kim, Jong Deok; Chung, Duck Hwan

    1991-01-01

    Infants during the first year of life are particularly prone to the development of staphylococcal pneumonia: in fact, it is the commonest bacterial cause of death due to respiratory tract infection in this age group. Pneumatoceles within the lungs and pleural complication such as pleural effusion, empyema, pyopneumothorax and pneumothorax are characteristics in this pneumonia. Retrospectively we reviewed 22 patients of staphylococcal pneumonia in children diagnosed by pleural fluid or blood culture, throat suction cytology, or culture of pus from thigh, joint fluid or ear discharge in one or combining them during the recent 5 years from January, 1985 to December, 1989 and obtained the following results: Male to female ratio was 1:1 and peak incidence was between 1 month and 1 year of age (45.5% : 10/22). Fever, coughing and dyspnea were the predominant symptoms, and duration of symptoms was 4 to 7 dyas in 59%. Plain chest film findings on admission day: 1) 27.3% (6/22) showed the lesion almost entirely restricted to the lung with ratio of 2:1 of right and left involvement and these were treated without complication. 2) Only one case (4.5%) showed pneumatoceles combined with pneumonic infiltration and pleural effusion. 3) Pleural lesion occurred in 72.7% (16/22) : 22.7% (5/22) without pneumonic infiltration and 50% (11/22) with pneumonic infiltration. They were empyema, pyopneumothorax, and pleural effusion in descending order. Mortalty was 4.5% (1/22)

  15. Expression of apoptosis-related genes in acute β-irradiated skin injury in rats

    International Nuclear Information System (INIS)

    Zhao Xiaoyu; He Hanliang; Qi Qiang; Lin Wei; Shen Guoliang

    2012-01-01

    Objective: To investigate the dynamic expression of apoptosis-related genes Bcl-2, Bax and P53 in acute radiation-induced skin ulcers, and to explore the underlying mechanism involved in retarded healing of the ulcer. Methods: Fifty-four female SD rats were divided into 3 groups. The model of acute radiation-induced skin injury, in rats was replicated with 45 Gy electron accelerator β-ray to the skin as radiation group (n=24); the model of deep second degree scald in rats was established as burn group (n=24); 6 normal rats were served as normal control group. From different periods skin wounds, the expression of Bcl-2, Bax and P53 were respectively assessed by means of immunohistochemical technique and. apoptosis was observed by TUNEL assay. Results: (1) The result of the TUNEL manifested that the integral absorbance (IA) of the radiation group was much higher than that of the control group. There is statistical significance between the two groups (P<0.05). (2) 0, 1, 2, 3 weeks after wound emerging, the Bax and P53 integral absorbance (IA) in radiation group was much higher than that of the control group. The Bcl-2 integral absorbance (IA) in bum group was much higher than that of the radiation group. There is statistical significance between the two groups (P<0.05). Conclusions: It was shown that apoptosis of β radiation manifested three typical characteristics, namely early occurrence, high frequency and delayed disappearance after radiation, which might explain the delayed wound healing caused by β radiation. (authors)

  16. Early burn wound excision and skin grafting postburn trauma restores in vivo neutrophil delivery to inflammatory lesions

    International Nuclear Information System (INIS)

    Tchervenkov, J.I.; Epstein, M.D.; Silberstein, E.B.; Alexander, J.W.

    1988-01-01

    This study assessed the effect of early vs delayed postburn wound excision and skin grafting on the in vivo neutrophil delivery to a delayed-type hypersensitivity (DTH) reaction and a bacterial skin lesion (BSL). Male Lewis rats were presensitized to keyhole-limpet hemocyanin. Group 1 comprised sham controls. Groups 2 through 4 were given a 30% 3 degrees scald burn, but the burn wounds were excised, and skin was grafted on days 1, 3, and 7, respectively, after the burn. Group 5 comprised burn controls. Twelve days after burn trauma, all rats were injected at different intervals (during a 24-hour period) with a trio of intradermal injections of keyhole-limpet hemocyanin, Staphylococcus aureus 502A, and saline at different sites. In vivo neutrophil delivery to these dermal lesions was determined by injecting indium in 111 oxyquinoline-labeled neutrophils isolated from similarly treated groups of rats. Neutrophil delivery to DTH and BSL lesions was restored to normal by excision and skin grafting of the burn wound one day after burn trauma. Waiting three days after burn trauma to excise and skin graft the wound partially, but not completely, restored the in vivo neutrophil delivery to DTH and BSL lesions. Waiting one week to excise and skin graft a burn wound resulted in no improvement in neutrophil delivery to DTH and BSL dermal lesions. It was concluded that burn wound excision and skin grafting immediately after burn trauma restored in vivo neutrophil delivery to a BSL and DTH dermal lesion. This may, in part, explain the beneficial effect of early aggressive burn wound debridement in patients with burn injuries

  17. Lab-on-a-chip for label free biological semiconductor analysis of Staphylococcal Enterotoxin B

    NARCIS (Netherlands)

    Yang, Minghui; Sun, Steven; Bruck, Hugh Alan; Kostov, Yordan; Rasooly, Avraham

    2010-01-01

    We describe a new lab-on-a-chip (LOC) which utilizes a biological semiconductor (BSC) transducer for label free analysis of Staphylococcal Enterotoxin B (SEB) (or other biological interactions) directly and electronically. BSCs are new transducers based on electrical percolation through a

  18. [Constitutional mismatch repair deficiency syndrome].

    Science.gov (United States)

    Jongmans, Marjolijn C; Gidding, Corrie E; Loeffen, Jan; Wesseling, Pieter; Mensenkamp, Arjen; Hoogerbrugge, Nicoline

    2015-01-01

    Constitutional mismatch repair deficiency (CMMR-D) syndrome is characterised by a significantly increased risk for developing cancer in childhood. It arises when both parents have a mutation in the same mismatch repair gene and pass it on to their child. An 8-year-old girl was diagnosed with CMMR-D syndrome after she developed a brain tumour at the age of 4 and a T-cell non-Hodgkin lymphoma at the age of 6. She had multiple hyperpigmented skin lesions and died of myelodysplastic syndrome at the age of 11. In children with cancer CMMR-D syndrome can be recognized particularly if there are multiple primary malignancies and skin hyperpigmentations and hypopigmentations. The parents of these children are at high risk for colorectal and endometrial cancer (Lynch syndrome), amongst others.

  19. Gorlin-goltz syndrome

    International Nuclear Information System (INIS)

    Ahmed, N.; Salman, M.; Mansoor, M.A.

    2007-01-01

    Multiple jaw cysts are a characteristic manifestation of basal cell nevus (Gorlin) syndrome. Gorlin-Goltz syndrome is characterized by symptoms primarily involving the skin, central nervous system, and skeletal system. In 90% of the patients, nevoid basal cell carcinoma syndrome is associated with recurring odontogenic keratocysts. This patient showed recurrent jaw and maxillary cysts, for which he was followed for 2 years. (author)

  20. Enhancement of allergic skin wheal responses in patients with atopic eczema/dermatitis syndrome by playing video games or by a frequently ringing mobile phone.

    Science.gov (United States)

    Kimata, H

    2003-06-01

    Playing video games causes physical and psychological stress, including increased heart rate and blood pressure and aggression-related feelings. Use of mobile phones is very popular in Japan, and frequent ringing is a common and intrusive part of Japanese life. Atopic eczema/dermatitis syndrome is often exacerbated by stress. Stress increases serum IgE levels, skews cytokine pattern towards Th2 type, enhances allergen-induced skin wheal responses, and triggers mast cell degranulation via substance P, vasoactive intestinal peptide and nerve growth factor. (1). In the video game study, normal subjects (n = 25), patients with allergic rhinitis (n = 25) or atopic eczema/dermatitis syndrome (n = 25) played a video game (STREET FIGHTER II) for 2 h. Before and after the study, allergen-induced wheal responses, plasma levels of substance P, vasoactive intestinal peptide and nerve growth factor, and in vitro production of total IgE, antihouse dust mite IgE and cytokines were measured. (2). In the mobile phone study, normal subjects (n = 27), patients with allergic rhinitis (n = 27) or atopic eczema/dermatitis syndrome (n = 27) were exposed to 30 incidences of ringing mobile phones during 30 min. Before and after the study, allergen-induced wheal responses, plasma levels of substance P, vasoactive intestinal peptide and nerve growth factor were measured. Playing video games had no effect on the normal subjects or the patients with allergic rhinitis. In contrast, playing video games significantly enhanced allergen-induced skin wheal responses and increased plasma levels of substance P, vasoactive intestinal peptide and nerve growth factors in the patients with atopic eczema/dermatitis syndrome. Moreover, playing video games enhanced in vitro production of total IgE and anti-house dust mite IgE with concomitant increased production of IL-4, IL-10 and IL-13 and decreased production of IFN-gamma and IL-12 in the patients with atopic eczema/dermatitis syndrome. However, exposure

  1. Michelin tire baby syndrome

    Directory of Open Access Journals (Sweden)

    Rita V Vora

    2016-01-01

    Full Text Available Michelin tire baby syndrome (MTBS, is a rare genodermatosis, characterized by generalized folding of excess skin and may be associated with various phenotypic abnormalities. The pathogenesis of this condition is unclear. Various congenital anomalies can be associated with it. It may be a clinical finding associated with various syndromes. Diagnosis is mainly clinical, and skin folds gradually diminish and disappear with age without any intervention. Here, we report a case of MTBS associated with convergent squint and hydrocephaly.

  2. Investigation of Demodex folliculorum frequency in patients with polycystic ovary syndrome.

    Science.gov (United States)

    Eser, Ayla; Erpolat, Seval; Kaygusuz, Ikbal; Balci, Hatice; Kosus, Aydin

    2017-01-01

    Background: Demodex mites are acari that reside in the pilosebaceous unit of the skin and have been associated with skin disorders. The objective of this study was to investigate the prevalence of Demodex folliculorum (D. folliculorum) mites in polycystic ovary syndrome patients as well as to examine the relationship between Demodex infestation and the presence of acne and oily or dry skin types in polycystic ovary syndrome patients. 41 polycystic ovary syndrome patients and 47 non-polycystic ovary syndrome control subjects were enrolled in the study. polycystic ovary syndrome was diagnosed according to the revised 2003 ESHRE/ASRM polycystic ovary syndrome Consensus Workshop Group diagnostic criteria. Microscopic examination of D. folliculorum mites was carried out by standardized skin surface biopsy. The result was considered positive when there were more than 5 mites per cm2. D. folliculorum was positive in 53.7% of the polycystic ovary syndrome patients and 31.9% of the non-polycystic ovary syndrome group (p=0.052). Demodex positivity was significantly associated with acne (p=0.003) and oily skin (p=0.005) in the polycystic ovary syndrome patients but not in the controls. Our study is limited by the relatively small number of subjects and the observational nature of the study design. Demodex mites might have a role in acne pathogenesis in patients with polycystic ovary syndrome. Anti-Demodex treatment may increase the response to treatment of acne. Further studies are indicated.

  3. Using Syndromic Surveillance to Investigate Tattoo-Related Skin Infections in New York City.

    Science.gov (United States)

    Kotzen, Mollie; Sell, Jessica; Mathes, Robert W; Dentinger, Catherine; Lee, Lillian; Schiff, Corinne; Weiss, Don

    2015-01-01

    In response to two isolated cases of Mycobacterium chelonae infections in tattoo recipients where tap water was used to dilute ink, the New York City (NYC) Department of Health and Mental Hygiene conducted an investigation using Emergency Department (ED) syndromic surveillance to assess whether an outbreak was occuring. ED visits with chief complaints containing the key word "tattoo" from November 1, 2012 to March 18, 2013 were selected for study. NYC laboratories were also contacted and asked to report skin or soft tissue cultures in tattoo recipients that were positive for non-tuberculosis mycobacterial infection (NTM). Thirty-one TREDV were identified and 14 (45%) were interviewed to determine if a NTM was the cause for the visit. One ED visit met the case definition and was referred to a dermatologist. This individual was negative for NTM. No tattoo-associated NTM cases were reported by NYC laboratories. ED syndromic surveillance was utilized to investigate a non-reportable condition for which no other data source existed. The results were reassuring that an outbreak of NTM in tattoo recipients was not occurring. In response to concerns about potential NTM infections, the department sent a letter to all licensed tattoo artists advising them not to dilute tattoo ink with tap water.

  4. Using Syndromic Surveillance to Investigate Tattoo-Related Skin Infections in New York City.

    Directory of Open Access Journals (Sweden)

    Mollie Kotzen

    Full Text Available In response to two isolated cases of Mycobacterium chelonae infections in tattoo recipients where tap water was used to dilute ink, the New York City (NYC Department of Health and Mental Hygiene conducted an investigation using Emergency Department (ED syndromic surveillance to assess whether an outbreak was occuring. ED visits with chief complaints containing the key word "tattoo" from November 1, 2012 to March 18, 2013 were selected for study. NYC laboratories were also contacted and asked to report skin or soft tissue cultures in tattoo recipients that were positive for non-tuberculosis mycobacterial infection (NTM. Thirty-one TREDV were identified and 14 (45% were interviewed to determine if a NTM was the cause for the visit. One ED visit met the case definition and was referred to a dermatologist. This individual was negative for NTM. No tattoo-associated NTM cases were reported by NYC laboratories. ED syndromic surveillance was utilized to investigate a non-reportable condition for which no other data source existed. The results were reassuring that an outbreak of NTM in tattoo recipients was not occurring. In response to concerns about potential NTM infections, the department sent a letter to all licensed tattoo artists advising them not to dilute tattoo ink with tap water.

  5. Revesz syndrome

    Directory of Open Access Journals (Sweden)

    Dayane Cristine Issaho

    2015-04-01

    Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

  6. Cross-Talk between Staphylococcus aureus and Other Staphylococcal Species via the agr Quorum Sensing System

    DEFF Research Database (Denmark)

    Canovas de la Nuez, Jaime; Baldry, Mara; Bojer, Martin S

    2016-01-01

    -inducing peptides (AIPs) sensed by AgrC, a two component histidine kinase. agr loci are found also in other staphylococcal species and for Staphylococcus epidermidis, the encoded AIP represses expression of agr regulated virulence genes in S. aureus. In this study we aimed to better understand the interaction...... between staphylococci and S. aureus, and show that this interaction may eventually lead to the identification of new anti-virulence candidates to target S. aureus infections. Here we show that culture supernatants of 37 out of 52 staphylococcal isolates representing 17 different species inhibit S. aureus...... suggesting that agr is an inter-species communication system. Based on these results we speculate that interactions between S. aureus and other colonizing staphylococci will significantly influence the ability of S. aureus to cause infection, and we propose that other staphylococci are potential sources...

  7. Under-recognition of acral peeling skin syndrome: 59 new cases with 15 novel mutations.

    Science.gov (United States)

    Szczecinska, W; Nesteruk, D; Wertheim-Tysarowska, K; Greenblatt, D T; Baty, D; Browne, F; Liu, L; Ozoemena, L; Terron-Kwiatkowski, A; McGrath, J A; Mellerio, J E; Morton, J; Woźniak, K; Kowalewski, C; Has, C; Moss, C

    2014-11-01

    Acral peeling skin syndrome (APSS) is a rare skin fragility disorder usually caused by mutations in the transglutaminase 5 gene (TGM5). We investigated the mutation spectrum of APSS in the U.K., Germany and Poland. We identified 59 children with APSS from 52 families. The phenotype was readily recognizable, with some variation in severity both within and between families. Most cases had been misdiagnosed as the localized form of epidermolysis bullosa simplex (EBS-loc). Eighteen different TGM5 mutations were identified, 15 of which were novel. Eight mutations were unique to a single family, nine each occurred in two families, while the common p.Gly113Cys mutation linked to a second missense variant p.Thr109Met occurred in 47 of the 52 families and was homozygous in 28. Most patients were of nonconsanguineous white European origin. We propose that APSS is under-reported and widely misdiagnosed as EBS-loc, with significant counselling implications as APSS is autosomal recessive while EBS-loc is dominant. We recommend screening for TGM5 mutations when EBS-loc is suspected but not confirmed by mutations in KRT5 or KRT14. Our report trebles the number of known TGM5 mutations. It provides further evidence that p.Gly113Cys is a founder mutation in the European population. This is consistent with the striking ethnic distribution of APSS in U.K., where the majority of patients are of nonconsanguineous white European origin, in contrast to the pattern of other recessive skin disorders. © 2014 British Association of Dermatologists.

  8. Estimating the time and temperature relationship for causation of deep-partial thickness skin burns.

    Science.gov (United States)

    Abraham, John P; Plourde, Brian; Vallez, Lauren; Stark, John; Diller, Kenneth R

    2015-12-01

    The objective of this study is to develop and present a simple procedure for evaluating the temperature and exposure-time conditions that lead to causation of a deep-partial thickness burn and the effect that the immediate post-burn thermal environment can have on the process. A computational model has been designed and applied to predict the time required for skin burns to reach a deep-partial thickness level of injury. The model includes multiple tissue layers including the epidermis, dermis, hypodermis, and subcutaneous tissue. Simulated exposure temperatures ranged from 62.8 to 87.8°C (145-190°F). Two scenarios were investigated. The first and worst case scenario was a direct exposure to water (characterized by a large convection coefficient) with the clothing left on the skin following the exposure. A second case consisted of a scald insult followed immediately by the skin being washed with cool water (20°C). For both cases, an Arrhenius injury model was applied whereby the extent and depth of injury were calculated and compared for the different post-burn treatments. In addition, injury values were compared with experiment data from the literature to assess verification of the numerical methodology. It was found that the clinical observations of injury extent agreed with the calculated values. Furthermore, inundation with cool water decreased skin temperatures more quickly than the clothing insulating case and led to a modest decrease in the burn extent. Copyright © 2015 Elsevier Ltd and ISBI. All rights reserved.

  9. The olive compound 4-hydroxytyrosol inactivates Staphyloccoccus aureus bacteria and Staphylococcal enterotoxin A (SEA)

    Science.gov (United States)

    The foodborne pathogen Staphylococcus aureus produces the virulent staphylococcal enterotoxin A (SEA), a single chain protein which consists of 233 amino acid residues with a molecular weight of 27,078 Da. SEA is a superantigen that is reported to contribute to animal (mastitis) and human (emesis, ...

  10. Expression of Staphylococcal Enterotoxins under Stress Encountered during Food Production and Preservation.

    Science.gov (United States)

    Schelin, Jenny; Susilo, Yusak Budi; Johler, Sophia

    2017-12-15

    Staphylococcal food poisoning (SFP) is the most prevalent cause of food-borne intoxications worldwide. Consumption of enterotoxins preformed in food causes violent vomiting and can be fatal in children and the elderly. While being repressed by competing bacteria in most matrices, Staphylococcus aureus benefits from crucial competitive advantages in foods with high osmolarity or low pH. During recent years, the long-standing belief in the feasibility of assessing SFP risk based on colony-forming units of S. aureus present in food products has been disproven. Instead, researchers and food business operators are acutely aware of the imminent threat arising from unforeseeable enterotoxin production under stress conditions. This paradigm shift led to a variety of new publications enabling an improved understanding of enterotoxin expression under stress conditions encountered in food. The wealth of data provided by these studies is extremely diverse, as it is based on different methodological approaches, staphylococcal strains, stressors, and enterotoxins. Therefore, in this review, we aggregated and critically evaluated the complex findings of these studies, to provide readers with a current overview of the state of research in the field.

  11. Plant-Derived Polyphenols Interact with Staphylococcal Enterotoxin A and Inhibit Toxin Activity.

    Science.gov (United States)

    Shimamura, Yuko; Aoki, Natsumi; Sugiyama, Yuka; Tanaka, Takashi; Murata, Masatsune; Masuda, Shuichi

    2016-01-01

    This study was performed to investigate the inhibitory effects of 16 different plant-derived polyphenols on the toxicity of staphylococcal enterotoxin A (SEA). Plant-derived polyphenols were incubated with the cultured Staphylococcus aureus C-29 to investigate the effects of these samples on SEA produced from C-29 using Western blot analysis. Twelve polyphenols (0.1-0.5 mg/mL) inhibited the interaction between the anti-SEA antibody and SEA. We examined whether the polyphenols could directly interact with SEA after incubation of these test samples with SEA. As a result, 8 polyphenols (0.25 mg/mL) significantly decreased SEA protein levels. In addition, the polyphenols that interacted with SEA inactivated the toxin activity of splenocyte proliferation induced by SEA. Polyphenols that exerted inhibitory effects on SEA toxic activity had a tendency to interact with SEA. In particular, polyphenol compounds with 1 or 2 hexahydroxydiphenoyl groups and/or a galloyl group, such as eugeniin, castalagin, punicalagin, pedunculagin, corilagin and geraniin, strongly interacted with SEA and inhibited toxin activity at a low concentration. These polyphenols may be used to prevent S. aureus infection and staphylococcal food poisoning.

  12. Plant-Derived Polyphenols Interact with Staphylococcal Enterotoxin A and Inhibit Toxin Activity.

    Directory of Open Access Journals (Sweden)

    Yuko Shimamura

    Full Text Available This study was performed to investigate the inhibitory effects of 16 different plant-derived polyphenols on the toxicity of staphylococcal enterotoxin A (SEA. Plant-derived polyphenols were incubated with the cultured Staphylococcus aureus C-29 to investigate the effects of these samples on SEA produced from C-29 using Western blot analysis. Twelve polyphenols (0.1-0.5 mg/mL inhibited the interaction between the anti-SEA antibody and SEA. We examined whether the polyphenols could directly interact with SEA after incubation of these test samples with SEA. As a result, 8 polyphenols (0.25 mg/mL significantly decreased SEA protein levels. In addition, the polyphenols that interacted with SEA inactivated the toxin activity of splenocyte proliferation induced by SEA. Polyphenols that exerted inhibitory effects on SEA toxic activity had a tendency to interact with SEA. In particular, polyphenol compounds with 1 or 2 hexahydroxydiphenoyl groups and/or a galloyl group, such as eugeniin, castalagin, punicalagin, pedunculagin, corilagin and geraniin, strongly interacted with SEA and inhibited toxin activity at a low concentration. These polyphenols may be used to prevent S. aureus infection and staphylococcal food poisoning.

  13. Frank-ter harr syndrome

    International Nuclear Information System (INIS)

    Saeed, M.; Shair, Q.A.; Saleem, S.M.

    2011-01-01

    Frank-Ter Haar Syndrome (FTHS) is a rare hereditary inherited disorder with many abnormalities. The main clinical features are brachycephaly, wide fontanels, prominent forehead, hypertelorism, prominent eyes, macrocornea with or without glaucoma, full cheeks, small chin, congenital heart defects, kyphoscoliosis, skeletal dysplasia, developmental delay, coccygeal skin folds and flexion deformity of the fingers. This case report describes Frank-Ter Haar syndrome in a 4 months old girl suffering from club foot, dysmorphism, prominent coccyx with skin fold, atrial septal defect, patent ductus asteriosus and megalocornea. (author)

  14. Frank-Ter Haar Syndrome.

    Science.gov (United States)

    Saeed, Muhammad; Shair, Qamar Ali; Saleem, Shah Masabat

    2011-04-01

    Frank-Ter Haar Syndrome (FTHS) is a rare hereditary inherited disorder with many abnormalities. The main clinical features are brachycephaly, wide fontanels, prominent forehead, hypertelorism, prominent eyes, macrocornea with or without glaucoma, full cheeks, small chin, congenital heart defects, kyphoscoliosis, skeletal dysplasia, developmental delay, coccygeal skin folds and flexion deformity of the fingers. This case report describes Frank-Ter Haar syndrome in a 4 months old girl suffering from club foot, dysmorphism, prominent coccyx with skin fold, atrial septal defect, patent ductus asteriosus and megalocornea.

  15. The fungus Trichophyton redellii sp. Nov. Causes skin infections that resemble white-nose syndrome of hibernating bats.

    Science.gov (United States)

    Lorch, Jeffrey M; Minnis, Andrew M; Meteyer, Carol U; Redell, Jennifer A; White, J Paul; Kaarakka, Heather M; Muller, Laura K; Lindner, Daniel L; Verant, Michelle L; Shearn-Bochsler, Valerie; Blehert, David S

    2015-01-01

    Before the discovery of white-nose syndrome (WNS), a fungal disease caused by Pseudogymnoascus destructans, there were no reports of fungal skin infections in bats during hibernation. In 2011, bats with grossly visible fungal skin infections similar in appearance to WNS were reported from multiple sites in Wisconsin, US, a state outside the known range of P. destructans and WNS at that time. Tape impressions or swab samples were collected from affected areas of skin from bats with these fungal infections in 2012 and analyzed by microscopy, culture, or direct DNA amplification and sequencing of the fungal internal transcribed spacer region (ITS). A psychrophilic species of Trichophyton was isolated in culture, detected by direct DNA amplification and sequencing, and observed on tape impressions. Deoxyribonucleic acid indicative of the same fungus was also detected on three of five bat carcasses collected in 2011 and 2012 from Wisconsin, Indiana, and Texas, US. Superficial fungal skin infections caused by Trichophyton sp. were observed in histopathology for all three bats. Sequencing of the ITS of Trichophyton sp., along with its inability to grow at 25 C, indicated that it represented a previously unknown species, described herein as Trichophyton redellii sp. nov. Genetic diversity present within T. redellii suggests it is native to North America but that it had been overlooked before enhanced efforts to study fungi associated with bats in response to the emergence of WNS.

  16. The fungus Trichophyton redellii sp. nov. causes skin infections that resemble white-nose syndrome of hibernating bats

    Science.gov (United States)

    Lorch, Jeffrey M.; Minnis, Andrew M.; Meteyer, Carol U.; Redell, Jennifer A.; White, J. Paul; Kaarakka, Heather M.; Muller, Laura K.; Lindner, David L.; Verant, Michelle L.; Shearn-Bochsler, Valerie I.; Blehert, David S.

    2014-01-01

    Before the discovery of white-nose syndrome (WNS), a fungal disease caused by Pseudogymnoascus destructans, there were no reports of fungal skin infections in bats during hibernation. In 2011, bats with grossly visible fungal skin infections similar in appearance to WNS were reported from multiple sites in Wisconsin, USA, a state outside the known range of P. destructans and WNS at that time. Tape impressions or swab samples were collected from affected areas of skin from bats with these fungal infections in 2012 and analyzed by microscopy, culture, or direct DNA amplification and sequencing of the fungal internal transcribed spacer region (ITS). A psychrophilic species ofTrichophyton was isolated in culture, detected by direct DNA amplification and sequencing, and observed on tape impressions. Deoxyribonucleic acid indicative of the same fungus was also detected on three of five bat carcasses collected in 2011 and 2012 from Wisconsin, Indiana, and Texas, USA. Superficial fungal skin infections caused by Trichophyton sp. were observed in histopathology for all three bats. Sequencing of the ITS of Trichophyton sp., along with its inability to grow at 25 C, indicated that it represented a previously unknown species, described herein as Trichophyton redellii sp. nov. Genetic diversity present within T. redellii suggests it is native to North America but that it had been overlooked before enhanced efforts to study fungi associated with bats in response to the emergence of WNS.

  17. Effect of Zicao ointment on second-degree burns in rats

    African Journals Online (AJOL)

    degree skin burns by electrical scald instrument. The animals were divided into three .... Silver sulfadiazine cream (1. %, w/w) was used as the ... hematoxylin and eosin reagent. A collagen fiber, inflammatory cell, blood vessel and granulation tissue of the skin tissues were examined under a microscope. Statistical analysis.

  18. Clinical spectrum of females with HCCS mutation: from no clinical signs to a neonatal lethal form of the microphthalmia with linear skin defects (MLS) syndrome

    NARCIS (Netherlands)

    van Rahden, V.A.; Rau, I.; Fuchs, S.; Kosyna, F.K.; de Almeida, H.L.; Fryssira, H.; Isidor, B.; Jauch, A.; Joubert, M.; Lachmeijer, A.M.A.; Zweier, C.; Moog, U.; Kutsche, K.

    2014-01-01

    Background: Segmental Xp22.2 monosomy or a heterozygous HCCS mutation is associated with the microphthalmia with linear skin defects (MLS) or MIDAS (microphthalmia, dermal aplasia, and sclerocornea) syndrome, an X-linked disorder with male lethality. HCCS encodes the holocytochrome c-type synthase

  19. Kindler′s syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    Neelam Suman

    2014-01-01

    Full Text Available Kindler syndrome is a rare hereditary disorder, associated with skin fragility. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes a 16-year-old patient with classical features like blistering and photosensitivity in childhood and the subsequent development of poikiloderma.

  20. Histopathology and culturable bacteria associated with "big belly" and "skin nodule" syndromes in ornamental Siamese fighting fish, Betta splendens.

    Science.gov (United States)

    Dong, H T; Senapin, S; Phiwsaiya, K; Techatanakitarnan, C; Dokladda, K; Ruenwongsa, P; Panijpan, B

    2018-06-02

    The Siamese fighting fish (Betta splendens) is one of the popular aquarium ornamental fish in the global trade. Large numbers of ornamental fish farmed in central Thailand suffered from two common syndromes; preliminarily named skin nodule syndrome (SNS) and big belly syndrome (BBS): they showed noticeable clinical signs of abnormal appearances resulting in depressed saleability. Since very few specifics are known about causative agents of these syndromes, this study aimed at investigating histopathological features and culturable bacteria associated with these fish infected in the process of farming. Histopathologically, SNS fish consistently exhibited necrosis and severe melanization in the muscles and multiple internal organs. Whereas BBS fish exhibited either typical granulomas or tissue damage associated with acid-fast stained bacteria and Gram negative bacteria, respectively. Six different Gram negative bacterial species were recovered from BBS fish while 23 bacterial species belonging to 14 genera were recovered from fish suffering from SNS. Most of the culturable bacteria are new to betta fish and some of them are known to be marine bacteria, suggesting possible entry route via a contaminated live feed, commercial Artemia shrimp. The true causative agents of these syndromes remain unclear. However, histopathological changes and existence of a wide range of bacteria associated with the naturally diseased fish suggest involvement of multiple bacterial infections. Copyright © 2018 Elsevier Ltd. All rights reserved.

  1. Regressing Multiple Viral Plaques and Skin Fragility Syndrome in a Cat Coinfected with FcaPV2 and FcaPV3

    Directory of Open Access Journals (Sweden)

    Alberto Alberti

    2015-01-01

    Full Text Available Feline viral plaques are uncommon skin lesions clinically characterized by multiple, often pigmented, and slightly raised lesions. Numerous reports suggest that papillomaviruses (PVs are involved in their development. Immunosuppressed and immunocompetent cats are both affected, the biological behavior is variable, and the regression is possible but rarely documented. Here we report a case of a FIV-positive cat with skin fragility syndrome and regressing multiple viral plaques in which the contemporary presence of two PV types (FcaPV2 and FcaPV3 was demonstrated by combining a quantitative molecular approach to histopathology. The cat, under glucocorticoid therapy for stomatitis and pruritus, developed skin fragility and numerous grouped slightly raised nonulcerated pigmented macules and plaques with histological features of epidermal thickness, mild dysplasia, and presence of koilocytes. Absolute quantification of the viral DNA copies (4555 copies/microliter of FcaPV2 and 8655 copies/microliter of FcaPV3 was obtained. Eighteen months after discontinuation of glucocorticoid therapy skin fragility and viral plaques had resolved. The role of the two viruses cannot be established and it remains undetermined how each of the viruses has contributed to the onset of VP; the spontaneous remission of skin lesions might have been induced by FIV status change over time due to glucocorticoid withdraw and by glucocorticoids withdraw itself.

  2. Cross contamination of turkey carcasses by Salmonella species during defeathering.

    Science.gov (United States)

    Nde, C W; McEvoy, J M; Sherwood, J S; Logue, C M

    2007-01-01

    Salmonella present on the feathers of live birds could be a source of contamination to carcass skin during defeathering. In this study, the possibility of transfer of Salmonella from the feathers of live turkeys to carcass tissue during the defeathering process at a commercial turkey processing plant was investigated. The contribution of scald water and the fingers of the picker machines to cross contamination were also examined. Over 4 visits, swab samples were collected from 174 randomly selected tagged birds before and after defeathering. Two swab samples from the fingers of the picker machines and a sample of scald water were also collected during each visit. Detection of Salmonella was carried out following standard cultural and identification methods. The DNA fingerprints obtained from pulsed field gel electrophoresis of Salmonella serotypes isolated before and after defeathering, from scald water, and from the fingers of the picker machines were compared to trace cross contamination routes. Salmonella prevalence was similar before and after defeathering during visits 2 and 3 and significantly increased after defeathering during visits 1 and 4. Over the 4 visits, all Salmonella subtypes obtained after defeathering were also isolated before defeathering. The results of this study suggest that Salmonella was transferred from the feathers to carcass skin during each visit. On each visit, the Salmonella subtypes isolated from the fingers of the picker machines were similar to subtypes isolated before and after defeathering, indicating that the fingers facilitate carcass cross contamination during defeathering. Salmonella isolated from scald water during visit 4 was related to isolates obtained before and after defeathering, suggesting that scald water is also a vehicle for cross contamination during defeathering. By using molecular subtyping, this study demonstrated the relationship between Salmonella present on the feathers of live turkeys and carcass skin after

  3. Inhibition of biological activity of staphylococcal enterotoxin A (SEA) by apple juice and apple polyphenols.

    Science.gov (United States)

    Rasooly, Reuven; Do, Paula M; Friedman, Mendel

    2010-05-12

    The foodborne pathogen Staphylococcus aureus produces the virulent staphylococcal enterotoxin A (SEA), a single-chain protein that consists of 233 amino acid residues with a molecular weight of 27 078 Da. SEA is a superantigen that is reported to contribute to animal (mastitis) and human (emesis, diarrhea, atopic dermatitis, arthritis, and toxic shock) syndromes. Changes of the native structural integrity may inactivate the toxin by preventing molecular interaction with cell membrane receptor sites of their host cells. In the present study, we evaluated the ability of one commercial and two freshly prepared apple juices and a commercial apple polyphenol preparation (Apple Poly) to inhibit the biological activity of SEA. Dilutions of freshly prepared apple juices and Apple Poly inhibited the biological activity of SEA without any significant cytotoxic effect on the spleen cells. Additional studies with antibody-coated immunomagnetic beads bearing specific antibodies against the toxin revealed that SEA added to apple juice appears to be largely irreversibly bound to the juice constituents. The results suggest that food-compatible and safe anti-toxin phenolic compounds can be used to inactivate SEA in vitro and possibly also in vivo, even after induction of T-cell proliferation by long-term exposure to SEA. The significance of the results for microbial food safety and human health is discussed.

  4. Staphylococcal poisoning foodborne outbreak: epidemiological investigation and strain genotyping.

    Science.gov (United States)

    Gallina, S; Bianchi, D M; Bellio, A; Nogarol, C; Macori, G; Zaccaria, T; Biorci, F; Carraro, E; Decastelli, L

    2013-12-01

    In June 2011, an outbreak of Staphylococcus aureus enterotoxin food poisoning gastroenteritis occurred in Turin, Italy, following a catered dinner party at a private home. Within a few hours, 26 of the 47 guests experienced gastrointestinal illness, and 9 were hospitalized. A retrospective cohort study using a standardized questionnaire was carried out, and the risk ratios for each food item were calculated. The analysis indicated consumption of seafood salad as the most probable cause of the outbreak (risk ratio = 11.72; 95 % confidence interval, 1.75 to 78.54). Biological samples were collected from four of the hospitalized guests (stool and vomit), nasal mucosa swabs from three food handlers employed with the caterer, and available food residuals. All stool and vomit samples tested positive for enterotoxigenic S. aureus. As residues of the seafood salad were no longer available for sampling, suspected contamination could not be verified. However, no other food was found contaminated by S. aureus or its enterotoxins. All isolates from the biological samples were characterized at the genomic level by means of two multiplex PCR protocols to determine the presence of genes encoding staphylococcal enterotoxins, pulsed-field gel electrophoresis and staphylococcal protein A gene (spa) typing to describe their genetic profiles. All the isolates presented genes encoding SEA and SEI; the pulsed-field gel electrophoresis genetic profiles revealed the same pulsotype in the microorganism isolated from the hospitalized guests as in one of the isolates from a food handler's nasal mucosa, and the spa typing analysis reported two closely related spa types (t701 and t267), implicating the food handler as the most likely outbreak source.

  5. Distribution and characterization of staphylococcal interspersed repeat units (SIRUs) and potential use for strain differentiation

    DEFF Research Database (Denmark)

    Hardy, K.J.; Ussery, David; Oppenheim, B.A.

    2004-01-01

    are similar to those used in the study of the evolution of Mycobacterium tuberculosis clades. Seven VNTRs, termed staphylococcal interspersed repeat units (SIRUs), distributed around the genome are described, occurring in both unique and multiple sites, and varying in length from 48 to 159 bp. Variations...

  6. Inhibition of Biological Activity of Staphylococcal Enterotoxin A (SEA) by Apple Juice and Apple Polyphenols

    Science.gov (United States)

    The foodborne pathogen Staphylococcus aureus produces the virulent staphylococcal entertoxin A (SEA), a single-chain protein that consists of 233 amino acid residues with a molecular weight of 27 078 Da. SEA is a superantigen that is reported to contribute to animal (mastitis) and human (emesis, dia...

  7. Cowden syndrome

    Directory of Open Access Journals (Sweden)

    Ravi Prakash S

    2010-01-01

    Full Text Available Cowden syndrome or multiple hamartoma syndrome is an autosomal dominant condition with variable expressions that result mainly from mutation in the PTEN gene on arm 10q. It is characterized by multiple hamartomatous neoplasms of the skin, oral mucosa, gastrointestinal tract, bones, CNS, eyes, and genitourinary tract. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here we present a case of Cowden syndrome in a 14-year-old female patient with the chief complaint of multiple oral papillomatous lesions.

  8. Stevens-Johnson syndrome and toxic epidermal necrolysis: an update on pharmacogenetics studies in drug-induced severe skin reaction.

    Science.gov (United States)

    Rufini, Sara; Ciccacci, Cinzia; Politi, Cristina; Giardina, Emiliano; Novelli, Giuseppe; Borgiani, Paola

    2015-11-01

    Stevens-Johnson syndrome and toxic epidermal necrolysis are severe, life-threatening drug reactions involving skin and membranes mucous, which are associated with significant morbidity and mortality and triggered, especially by drug exposure. Different studies have demonstrated that drug response is a multifactorial character and that the interindividual variability in this response depends on both environmental and genetic factors. The last ones have a relevant significance. In fact, the identification of new specific genetic markers involved in the response to drugs, will be of great utility to establish a more personalized therapeutic approach and to prevent the appearance of these adverse reactions. In this review, we summarize recent progresses in the Pharmacogenetics studies related to Stevens-Johnson syndrome/toxic epidermal necrolysis reporting the major genetic factors identified in the last years as associated with the disease and highlighting the use of some of these genomic variants in the clinical practice.

  9. Loss of corneodesmosin leads to severe skin barrier defect, pruritus, and atopy: unraveling the peeling skin disease.

    Science.gov (United States)

    Oji, Vinzenz; Eckl, Katja-Martina; Aufenvenne, Karin; Nätebus, Marc; Tarinski, Tatjana; Ackermann, Katharina; Seller, Natalia; Metze, Dieter; Nürnberg, Gudrun; Fölster-Holst, Regina; Schäfer-Korting, Monika; Hausser, Ingrid; Traupe, Heiko; Hennies, Hans Christian

    2010-08-13

    Generalized peeling skin disease is an autosomal-recessive ichthyosiform erythroderma characterized by lifelong patchy peeling of the skin. After genome-wide linkage analysis, we have identified a homozygous nonsense mutation in CDSN in a large consanguineous family with generalized peeling skin, pruritus, and food allergies, which leads to a complete loss of corneodesmosin. In contrast to hypotrichosis simplex, which can be associated with specific dominant CDSN mutations, peeling skin disease is characterized by a complete loss of CDSN expression. The skin phenotype is consistent with a recent murine Cdsn knockout model. Using three-dimensional human skin models, we demonstrate that lack of corneodesmosin causes an epidermal barrier defect supposed to account for the predisposition to atopic diseases, and we confirm the role of corneodesmosin as a decisive epidermal adhesion molecule. Therefore, peeling skin disease will represent a new model disorder for atopic diseases, similarly to Netherton syndrome and ichthyosis vulgaris in the recent past.

  10. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (II): thrombocytopenia and skin manifestations.

    Science.gov (United States)

    Cervera, R; Tektonidou, M G; Espinosa, G; Cabral, A R; González, E B; Erkan, D; Vadya, S; Adrogué, H E; Solomon, M; Zandman-Goddard, G; Shoenfeld, Y

    2011-02-01

    The objectives of the 'Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations' were to assess the clinical utility of the international consensus statement on classification criteria and treatment guidelines for the catastrophic APS, to identify and grade the studies that analyze the relationship between the antiphospholipid antibodies and the non-criteria APS manifestations, and to present the current evidence regarding the accuracy of these non-criteria APS manifestations for the detection of patients with APS. This article summarizes the studies analyzed on thrombocytopenia and skin manifestations, and presents the recommendations elaborated by the Task Force after this analysis.

  11. p63 in skin development and ectodermal dysplasias

    Science.gov (United States)

    Koster, Maranke I.

    2010-01-01

    The transcription factor p63 is critically important for skin development and maintenance. Processes that require p63 include epidermal lineage commitment, epidermal differentiation, cell adhesion, and basement membrane formation. Not surprisingly, alterations in the p63 pathway underlie a subset of ectodermal dysplasias, developmental syndromes in which the skin and skin appendages do not develop normally. This review summarizes the current understanding of the role of p63 in normal development and ectodermal dysplasias. PMID:20445549

  12. Plant plasma membrane-bound staphylococcal-like DNases as a novel class of eukaryotic nucleases

    Directory of Open Access Journals (Sweden)

    Leśniewicz Krzysztof

    2012-10-01

    Full Text Available Abstract Background The activity of degradative nucleases responsible for genomic DNA digestion has been observed in all kingdoms of life. It is believed that the main function of DNA degradation occurring during plant programmed cell death is redistribution of nucleic acid derived products such as nitrogen, phosphorus and nucleotide bases. Plant degradative nucleases that have been studied so far belong mainly to the S1-type family and were identified in cellular compartments containing nucleic acids or in the organelles where they are stored before final application. However, the explanation of how degraded DNA components are exported from the dying cells for further reutilization remains open. Results Bioinformatic and experimental data presented in this paper indicate that two Arabidopsis staphylococcal-like nucleases, named CAN1 and CAN2, are anchored to the cell membrane via N-terminal myristoylation and palmitoylation modifications. Both proteins possess a unique hybrid structure in their catalytic domain consisting of staphylococcal nuclease-like and tRNA synthetase anticodon binding-like motifs. They are neutral, Ca2+-dependent nucleaces showing a different specificity toward the ssDNA, dsDNA and RNA substrates. A study of microarray experiments and endogenous nuclease activity revealed that expression of CAN1 gene correlates with different forms of programmed cell death, while the CAN2 gene is constitutively expressed. Conclusions In this paper we present evidence showing that two plant staphylococcal-like nucleases belong to a new, as yet unidentified class of eukaryotic nucleases, characterized by unique plasma membrane localization. The identification of this class of nucleases indicates that plant cells possess additional, so far uncharacterized, mechanisms responsible for DNA and RNA degradation. The potential functions of these nucleases in relation to their unique intracellular location are discussed.

  13. Churg Strauss syndrome

    International Nuclear Information System (INIS)

    Lopez Rengifo, Diana Milena; Contreras Zuniga, Eduardo; Osio, Luis Fernando

    2007-01-01

    The Churg-Strauss syndrome, also called allergic granulomatosis and angiitis, is a multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. The most common organ involved is the lung, followed by the skin. The Churg-Strauss syndrome, however, can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems

  14. Nicolau Syndrome after Intramuscular Injection: 3 Cases

    Directory of Open Access Journals (Sweden)

    Seok-Kwun Kim

    2012-05-01

    Full Text Available Nicolau syndrome is a rare complication of intramuscular injection consisting of ischemicnecrosis of skin, soft tissue, and muscular tissue that arises locoregionally. The characteristicpattern is pain around the injection site, developing into erythema, a livedoid dermatitispatch, and necrosis of the skin, subcutaneous fat, and muscle tissue. Three patients wereinjected with drugs (diclofenac sodium, ketoprofen, meperidine for pain relief. Three patientscomplained of pain, and a skin lesion was observed, after which necrosis developed on theirbuttocks. Each patient underwent debridement and coverage. The wound healed uneventfully.We report three cases of Nicolau syndrome in the buttocks following diclofenac intramuscularinjection.

  15. Differences in manifestations of Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome.

    Science.gov (United States)

    Meester, Josephina A N; Verstraeten, Aline; Schepers, Dorien; Alaerts, Maaike; Van Laer, Lut; Loeys, Bart L

    2017-11-01

    Many different heritable connective tissue disorders (HCTD) have been described over the past decades. These syndromes often affect the connective tissue of various organ systems, including heart, blood vessels, skin, joints, bone, eyes, and lungs. The discovery of these HCTD was followed by the identification of mutations in a wide range of genes encoding structural proteins, modifying enzymes, or components of the TGFβ-signaling pathway. Three typical examples of HCTD are Marfan syndrome (MFS), Ehlers-Danlos syndrome (EDS), and Loeys-Dietz syndrome (LDS). These syndromes show some degree of phenotypical overlap of cardiovascular, skeletal, and cutaneous features. MFS is typically characterized by cardiovascular, ocular, and skeletal manifestations and is caused by heterozygous mutations in FBN1 , coding for the extracellular matrix (ECM) protein fibrillin-1. The most common cardiovascular phenotype involves aortic aneurysm and dissection at the sinuses of Valsalva. LDS is caused by mutations in TGBR1/2 , SMAD2/3 , or TGFB2/3 , all coding for components of the TGFβ-signaling pathway. LDS can be distinguished from MFS by the unique presence of hypertelorism, bifid uvula or cleft palate, and widespread aortic and arterial aneurysm and tortuosity. Compared to MFS, LDS cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis, a key distinguishing feature of MFS. Overlapping features between MFS and LDS include scoliosis, pes planus, anterior chest deformity, spontaneous pneumothorax, and dural ectasia. EDS refers to a group of clinically and genetically heterogeneous connective tissue disorders and all subtypes are characterized by variable abnormalities of skin, ligaments and joints, blood vessels, and internal organs. Typical presenting features include joint hypermobility, skin hyperextensibility, and tissue fragility. Up to one quarter of the EDS patients show aortic aneurysmal

  16. Differences in manifestations of Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome

    Science.gov (United States)

    Meester, Josephina A. N.; Verstraeten, Aline; Schepers, Dorien; Alaerts, Maaike; Van Laer, Lut

    2017-01-01

    Many different heritable connective tissue disorders (HCTD) have been described over the past decades. These syndromes often affect the connective tissue of various organ systems, including heart, blood vessels, skin, joints, bone, eyes, and lungs. The discovery of these HCTD was followed by the identification of mutations in a wide range of genes encoding structural proteins, modifying enzymes, or components of the TGFβ-signaling pathway. Three typical examples of HCTD are Marfan syndrome (MFS), Ehlers-Danlos syndrome (EDS), and Loeys-Dietz syndrome (LDS). These syndromes show some degree of phenotypical overlap of cardiovascular, skeletal, and cutaneous features. MFS is typically characterized by cardiovascular, ocular, and skeletal manifestations and is caused by heterozygous mutations in FBN1, coding for the extracellular matrix (ECM) protein fibrillin-1. The most common cardiovascular phenotype involves aortic aneurysm and dissection at the sinuses of Valsalva. LDS is caused by mutations in TGBR1/2, SMAD2/3, or TGFB2/3, all coding for components of the TGFβ-signaling pathway. LDS can be distinguished from MFS by the unique presence of hypertelorism, bifid uvula or cleft palate, and widespread aortic and arterial aneurysm and tortuosity. Compared to MFS, LDS cardiovascular manifestations tend to be more severe. In contrast, no association is reported between LDS and the presence of ectopia lentis, a key distinguishing feature of MFS. Overlapping features between MFS and LDS include scoliosis, pes planus, anterior chest deformity, spontaneous pneumothorax, and dural ectasia. EDS refers to a group of clinically and genetically heterogeneous connective tissue disorders and all subtypes are characterized by variable abnormalities of skin, ligaments and joints, blood vessels, and internal organs. Typical presenting features include joint hypermobility, skin hyperextensibility, and tissue fragility. Up to one quarter of the EDS patients show aortic aneurysmal

  17. The clinical spectrum and antibiotic sensitivity patterns of staphylococcal pyodermas in the community and hospital

    Directory of Open Access Journals (Sweden)

    Shireen Furtado

    2014-01-01

    Full Text Available Context: The uncontrolled use of antibiotics has resulted in a relentless spread of multiresistant strains of Staphylococcus aureus. There are studies conducted in medical colleges in Chandigarh, Chennai, Mumbai and Vellore comparing pyodermas in the community and hospital setting based on clinical and bacteriological parameters. Aims: This study, conducted over 1½ years from March 2009 to August 2010, aimed at analyzing the clinical spectrum and antibiotic sensitivity pattern of community and hospital-associated (HA staphylococcal pyoderma. It also assessed the prevalence of methicillin-resistant S. aureus (MRSA in the community and hospital cohort settings. Subjects and Methods: The study comprised of 200 cases of staphylococcal pyodermas, derived from the community (150 cases and hospital (50 cases. Patients were evaluated based on their clinical presentation; antibiotic susceptibility was tested using the Kirby-Bauer disk diffusion method. Statistical Analysis Used: Statistical significance between individual attributes between the community and HA staphylococcal pyoderma groups was analyzed using Chi-square test and mean differences using student′s t-test. Results: Factors associated with community-associated (CA pyodermas were young age (P = 0.0021, primary pyodermas, and involvement of extremities, while those with HA pyodermas were middle age, secondary pyodermas, and significantly increased body surface involvement (P = 0.041. Incidence of CA-MRSA was 11.3%, while that of HA-MRSA was 18%. Conclusions: A high level of resistance to first-line drugs such as penicillin, ciprofloxacin and cotrimoxazole was observed, more so in the hospital strain than in the community strain. S. aureus demonstrated good susceptibility to cephalosporins. Though the two strains of MRSA differed clinically, they showed 100% sensitivity to vancomycin and linezolid.

  18. Metastatic Basal Cell Carcinoma Accompanying Gorlin Syndrome

    Directory of Open Access Journals (Sweden)

    Yeliz Bilir

    2014-01-01

    Full Text Available Gorlin-Goltz syndrome or basal cell nevus syndrome is an autosomal dominant syndrome characterized by skeletal anomalies, numerous cysts observed in the jaw, and multiple basal cell carcinoma of the skin, which may be accompanied by falx cerebri calcification. Basal cell carcinoma is the most commonly skin tumor with slow clinical course and low metastatic potential. Its concomitance with Gorlin syndrome, resulting from a mutation in a tumor suppressor gene, may substantially change morbidity and mortality. A 66-year-old male patient with a history of recurrent basal cell carcinoma was presented with exophthalmus in the left eye and the lesions localized in the left lateral orbita and left zygomatic area. His physical examination revealed hearing loss, gapped teeth, highly arched palate, and frontal prominence. Left orbital mass, cystic masses at frontal and ethmoidal sinuses, and multiple pulmonary nodules were detected at CT scans. Basal cell carcinoma was diagnosed from biopsy of ethmoid sinus. Based on the clinical and typical radiological characteristics (falx cerebri calcification, bifid costa, and odontogenic cysts, the patient was diagnosed with metastatic skin basal cell carcinoma accompanied by Gorlin syndrome. Our case is a basal cell carcinoma with aggressive course accompanying a rarely seen syndrome.

  19. Happle-tinschert syndrome: Report of a case with hemimegalencephaly

    Energy Technology Data Exchange (ETDEWEB)

    Oezgur, Anil; Cabuk, Gonca; Arpaci, Rabia; Baz, Kiymet; Katar, Demet [Mersin University Faculty of Medicine, Mersin (Turkmenistan)

    2014-08-15

    Happle-Tinschert syndrome is a disorder causing unilateral segmentally arranged basaloid follicular hamartomas of the skin associated with ipsilateral osseous, dental and cerebral abnormalities including tumors. Although a case with hemimegalencephaly was previously described, this is the first report of Happle-Tinschert syndrome with discrepant short left leg, ipsilateral skin lesions, hemimegalencephaly and frontal polymicrogyria.

  20. Happle-tinschert syndrome: Report of a case with hemimegalencephaly

    International Nuclear Information System (INIS)

    Oezgur, Anil; Cabuk, Gonca; Arpaci, Rabia; Baz, Kiymet; Katar, Demet

    2014-01-01

    Happle-Tinschert syndrome is a disorder causing unilateral segmentally arranged basaloid follicular hamartomas of the skin associated with ipsilateral osseous, dental and cerebral abnormalities including tumors. Although a case with hemimegalencephaly was previously described, this is the first report of Happle-Tinschert syndrome with discrepant short left leg, ipsilateral skin lesions, hemimegalencephaly and frontal polymicrogyria.

  1. Gorlin-Goltz Syndrome

    Directory of Open Access Journals (Sweden)

    Padma Pandeshwar

    2012-01-01

    Full Text Available The Gorlin-Goltz syndrome (GGS (the nevoid basal cell carcinoma syndrome—NBCCS is a rare autosomal dominant syndrome caused due to mutations in the PTCH (patched gene found on chromosome arm 9q. The syndrome, characterized by increased predisposition to develop basal cell carcinoma and associated multiorgan anomalies, has a high level of penetrance and variable expressiveness. GGS is a multidisciplinary problem, early diagnosis of which allows introduction of secondary prophylaxis and following an appropriate treatment to delay the progress of the syndrome. The following report emphasizes the need for awareness of the diagnostic criteria of this syndrome in cases with no typical skin lesions.

  2. Weak effect of metal type and ica genes on staphylococcal infection of titanium and stainless steel implants.

    Science.gov (United States)

    Hudetz, D; Ursic Hudetz, S; Harris, L G; Luginbühl, R; Friederich, N F; Landmann, R

    2008-12-01

    Currently, ica is considered to be the major operon responsible for staphylococcal biofilm. The effect of biofilm on susceptibility to staphylococcal infection of different implant materials in vivo is unclear. The interaction of ica-positive (wild-type (WT)) and ica-negative (ica(-)) Staphylococcus aureus and Staphylococcus epidermidis strains with titanium and both smooth and rough stainless steel surfaces was studied by scanning electron microscopy in vitro and in a mouse tissue cage model during 2 weeks following perioperative or postoperative inoculation in vivo. In vitro, WT S. epidermidis adhered equally and more strongly than did WT S. aureus to all materials. Both WT strains, but not ica(-) strains, showed multilayered biofilm. In vivo, 300 CFUs of WT and ica(-)S. aureus led, in all metal cages, to an infection with a high level of planktonic CFUs and only 0.89% adherent CFUs after 8 days. In contrast, 10(6) CFUs of the WT and ica(-) strains were required for postoperative infection with S. epidermidis. In all metal types, planktonic numbers of S. epidermidis dropped to titanium cages adherent WT bacteria survived in higher numbers than ica(-) bacteria. In conclusion, the metal played a minor role in susceptibility to and persistence of staphylococcal infection; the presence of ica genes had a strong effect on biofilm in vitro and a weak effect in vivo; and S. epidermidis was more pathogenic when introduced during implantation than after implantation.

  3. Crohn's Disease Associated with Sweet's Syndrome and Sjögren's Syndrome Treated with Infliximab

    Directory of Open Access Journals (Sweden)

    Erina N. Foster

    2005-01-01

    Full Text Available The association of Crohn's disease (CD and Sweet's syndrome is rare and the presence of Sjögren's syndrome in Crohn's disease is even rarer, with only three reports found in the literature. We describe two cases of Crohn's disease associated with Sweet's syndrome, one of which is the first case of CD and Sweet's concomitantly associated with Sjögren's syndrome. Both cases responded rapidly to Infliximab therapy with complete resolution of the skin lesions.

  4. Plant-Derived Polyphenols Interact with Staphylococcal Enterotoxin A and Inhibit Toxin Activity

    OpenAIRE

    Shimamura, Yuko; Aoki, Natsumi; Sugiyama, Yuka; Tanaka, Takashi; Murata, Masatsune; Masuda, Shuichi

    2016-01-01

    This study was performed to investigate the inhibitory effects of 16 different plant-derived polyphenols on the toxicity of staphylococcal enterotoxin A (SEA). Plant-derived polyphenols were incubated with the cultured Staphylococcus aureus C-29 to investigate the effects of these samples on SEA produced from C-29 using Western blot analysis. Twelve polyphenols (0.1-0.5 mg/mL) inhibited the interaction between the anti-SEA antibody and SEA. We examined whether the polyphenols could directly i...

  5. [Influence of the self-reported skin color on the prevalence of metabolic syndrome in an urban Brazilian population].

    Science.gov (United States)

    Barbosa, Paulo José Bastos; Lessa, Ines; Almeida Filho, Naomar de; Magalhães, Lucélia Batista N Cunha; Araújo, Jenny

    2010-01-01

    The metabolic syndrome (MS) has a high prevalence in different parts of the world, with variations between different ethnic groups. This study aims at exploring the influence of the self-reported skin color on the prevalence of MS Methods: Cross-sectional study, carried out in a population subgroup (n=1,439 adults) in Salvador, Brazil. The self-reported skin color (white, mulatto or black) was used as well as the MS criterion of ATP-III. The Chi-square test for tendency was used to analyze the prevalence gradient between the groups and logistic regression, for association analysis. The general prevalence of MS, adjusted for potentially confounder variables, did not differ among whites (23.3%), mulattos (23.3%) and blacks (23.4%). The analysis by sex showed, among men, a reduction in the MS prevalence of whites (26.2%, 95%CI: 20.7-31.7), in comparison to blacks (17.5%, 95%CI: 12.3-22.8) and an intermediate prevalence among mulattos, 21.9%, 95%CI: 18.6-25.1, p tend. = 0.002. Among the women, the tendency was the opposite, being higher among the blacks, 27.0%, 95%CI: 22.2-31.8, and lower among the whites, 20.5%, 95%CI: 15.6-25.4, p tend. = 0.02. The multivariate analysis of the association between skin color and MS (white = group of reference) showed that the black color of the skin was a protective factor among black men, with a prevalence ratio (PR) = 0.60 (0.36-0.97), whereas it tended to be a risk factor among black women, with a PR = 1.33 (0.94-1.78). The prevalence of MA presented an inverse variation according to the color of skin between men and women. To be black was a protective factor among men and a risk factor among women.

  6. Costello syndrome: report and review

    NARCIS (Netherlands)

    van Eeghen, A. M.; van Gelderen, I.; Hennekam, R. C.

    1999-01-01

    We describe a 34-year-old woman with mental retardation, short stature, macrocephaly, a "coarse" face, hoarse voice, and redundant skin with deep palmar and plantar creases who had evident Costello syndrome. Lacking papillomata, she had wart-like lesions of the skin. The previously reported patients

  7. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.

    Science.gov (United States)

    Knobler, R; Moinzadeh, P; Hunzelmann, N; Kreuter, A; Cozzio, A; Mouthon, L; Cutolo, M; Rongioletti, F; Denton, C P; Rudnicka, L; Frasin, L A; Smith, V; Gabrielli, A; Aberer, E; Bagot, M; Bali, G; Bouaziz, J; Braae Olesen, A; Foeldvari, I; Frances, C; Jalili, A; Just, U; Kähäri, V; Kárpáti, S; Kofoed, K; Krasowska, D; Olszewska, M; Orteu, C; Panelius, J; Parodi, A; Petit, A; Quaglino, P; Ranki, A; Sanchez Schmidt, J M; Seneschal, J; Skrok, A; Sticherling, M; Sunderkötter, C; Taieb, A; Tanew, A; Wolf, P; Worm, M; Wutte, N J; Krieg, T

    2017-09-01

    The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum. © 2017 European Academy of Dermatology and Venereology.

  8. Double antibody solid-phase radioimmunoassay for staphylococcal enterotoxin A

    International Nuclear Information System (INIS)

    Lindroth, S.; Niskanen, A.

    1977-01-01

    A double antibody solid-phase (DASP) radioimmunoassay for staphylococcal enterotoxin A is described. In the assay the antigen-antibody complex is precipitated by anti-rabbit serum which is adsorbed onto a solid carrier (cellulose). The method is sensitive to 200 pg of enterotoxin. It was possible to detect a little as 2-5 ng of enterotoxin A/ml food extract from minced meat and sausage. Enterotoxins B and C were not found to inhibit the uptake of labled enterotoxin A at a level which might distort the results of the enterotoxin A assay. The DASP technique is sensitive, rapid, and easy to perform and thus compares favorably with other radioimmunoassays for enterotoxin. (orig.) [de

  9. Arousal Modulation in Females with Fragile X or Turner Syndrome

    Science.gov (United States)

    Roberts, Jane; Mazzocco, Michele M. M.; Murphy, Melissa M.; Hoehn-Saric, Rudolf

    2008-01-01

    The present study was carried out to examine physiological arousal modulation (heart activity and skin conductance), across baseline and cognitive tasks, in females with fragile X or Turner syndrome and a comparison group of females with neither syndrome. Relative to the comparison group, for whom a greater increase in skin conductance was…

  10. Chimeric anti-staphylococcal enterotoxin B antibodies and lovastatin act synergistically to provide in vivo protection against lethal doses of SEB.

    Directory of Open Access Journals (Sweden)

    Mulualem E Tilahun

    Full Text Available Staphylococcal enterotoxin B (SEB is one of a family of toxins secreted by Staphylococcus aureus that act as superantigens, activating a large fraction of the T-cell population and inducing production of high levels of inflammatory cytokines that can cause toxic shock syndrome (TSS and death. Extracellular engagement of the TCR of T-cells and class II MHC of antigen presenting cells by SEB triggers the activation of many intracellular signaling processes. We engineered chimeric antibodies to block the extracellular engagement of cellular receptors by SEB and used a statin to inhibit intracellular signaling. Chimeric human-mouse antibodies directed against different neutralizing epitopes of SEB synergistically inhibited its activation of human T-cells in vitro. In the in vivo model of lethal toxic shock syndrome (TSS in HLA-DR3 transgenic mice, two of these antibodies conferred significant partial protection when administered individually, but offered complete protection in a synergistic manner when given together. Similarly, in vivo, lovastatin alone conferred only partial protection from TSS similar to single anti-SEB antibodies. However, used in combination with one chimeric neutralizing anti-SEB antibody, lovastatin provided complete protection against lethal TSS in HLA-DR3 transgenic mice. These experiments demonstrate that in vivo protection against lethal doses of SEB can be achieved by a statin of proven clinical safety and chimeric human-mouse antibodies, agents now widely used and known to be of low immunogenicity in human hosts.

  11. Churg-strauss syndrome

    Directory of Open Access Journals (Sweden)

    Subhasish Ghosh

    2011-01-01

    Full Text Available Churg-Strauss syndrome (CSS is a rare granulomatous necrotizing small vessel vasculitis characterized by the presence of asthma, sinusitis, and hypereosinophilia. The cause of this allergic angiitis and granulomatosis is unknown. Other common manifestations are pulmonary infiltrates, skin, gastrointestinal, and cardiovascular involvement. No data have been reported regarding the role of immune complexes or cell mediated mechanisms in this disease, although autoimmunity is evident with the presence hypergammaglobulinemia, increased levels of IgE and Antineutrophil cytoplasmic antibody (positive in 40%. We report the case of a 27-year-old lady presenting with painful swelling of predominantly lower limbs with extensive vesicles and ecchymotic patches and fever shortly after stopping systemic steroids taken for a prolonged duration (2002--2010. The aim of this case report is to point to the possibility of CSS in patients presenting with extensive skin lesions masquerading as Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Syndrome (SJS/TENS.

  12. Diogenes Syndrome: A Case Report

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    Projna Biswas

    2013-01-01

    Full Text Available Cessation of normal skin cleansing seen in geriatric or self-neglected patients can cause accumulation of keratinous crusts on the skin. In the extreme end of this spectrum is a condition known as Diogenes syndrome (DS. These patients may have psychiatric disorders like paranoid disorders, mood affection, or temporofrontal dementia. Subjects are mainly the elderly but few cases in younger age group of patients have also been reported. Lesions of DS are usually found over upper central chest, back, and groin. In the young, lesions are mainly found over scalp, face, or arms. Absence of normal skin cleaning causes keratin and dirty debris to accumulate and with time form a thick shell. These debris can be secondarily infected by bacteria, fungus, and so forth. These skin lesions are not usually seen in individual with proper hygiene. We report a case of Diogenes syndrome in a 34-year-old young male patient who had associated schizophrenia.

  13. Nicolau syndrome following intramuscular benzathine penicillin

    Directory of Open Access Journals (Sweden)

    De Sousa R

    2008-01-01

    Full Text Available Nicolau syndrome (NS is a rare complication of an intramuscular injection characterized by severe pain, skin discoloration, and varying levels of tissue necrosis. The case outcomes vary from atrophic ulcers and severe pain to sepsis and limb amputation. We describe a case of a seven-year-old boy with diagnosis of NS after intramuscular benzathine penicillin injection to the ventrolateral aspect of the left thigh. Characteristic violaceous discoloration of skin and immediate injection site pain identified it as a case of NS. The case was complicated by rapid progression of compartment syndrome of the lower limb, proceeding to acute renal failure and death. Associated compartment syndrome can be postulated as a poor prognostic factor for NS.

  14. Nicolau Syndrome after Intramuscular Injection: 3 Cases

    Directory of Open Access Journals (Sweden)

    Seok-Kwun Kim

    2012-05-01

    Full Text Available Nicolau syndrome is a rare complication of intramuscular injection consisting of ischemic necrosis of skin, soft tissue, and muscular tissue that arises locoregionally. The characteristic pattern is pain around the injection site, developing into erythema, a livedoid dermatitis patch, and necrosis of the skin, subcutaneous fat, and muscle tissue. Three patients were injected with drugs (diclofenac sodium, ketoprofen, meperidine for pain relief. Three patients complained of pain, and a skin lesion was observed, after which necrosis developed on their buttocks. Each patient underwent debridement and coverage. The wound healed uneventfully. We report three cases of Nicolau syndrome in the buttocks following diclofenac intramuscular injection.

  15. Identification of Clinical Staphylococcal Isolates from Humans by Internal Transcribed Spacer PCR

    Science.gov (United States)

    Couto, Isabel; Pereira, Sandro; Miragaia, Maria; Sanches, Ilda Santos; de Lencastre, Hermínia

    2001-01-01

    The emergence of coagulase-negative staphylococci not only as human pathogens but also as reservoirs of antibiotic resistance determinants requires the deployment and development of methods for their rapid and reliable identification. Internal transcribed spacer-PCR (ITS-PCR) was used to identify a collection of 617 clinical staphylococcal isolates. The amplicons were resolved in high-resolution agarose gels and visually compared with the patterns obtained for the control strains of 29 staphylococcal species. Of the 617 isolates studied, 592 (95.95%) were identified by ITS-PCR and included 11 species: 302 isolates of Staphylococcus epidermidis, 157 of S. haemolyticus, 79 of S. aureus, 21 of S. hominis, 14 of S. saprophyticus, 8 of S. warneri, 6 of S. simulans, 2 of S. lugdunensis, and 1 each of S. caprae, S. carnosus, and S. cohnii. All species analyzed had unique ITS-PCR patterns, although some were very similar, namely, the group S. saprophyticus, S. cohnii, S. gallinarum, S. xylosus, S. lentus, S. equorum, and S. chromogenes, the pair S. schleiferi and S. vitulus, and the pair S. piscifermentans and S. carnosus. Four species, S. aureus, S. caprae, S. haemolyticus, and S. lugdunensis, showed polymorphisms on their ITS-PCR patterns. ITS-PCR proved to be a valuable alternative for the identification of staphylococci, offering, within the same response time and at lower cost, higher reliability than the currently available commercial systems. PMID:11526135

  16. Identification of clinical staphylococcal isolates from humans by internal transcribed spacer PCR.

    Science.gov (United States)

    Couto, I; Pereira, S; Miragaia, M; Sanches, I S; de Lencastre, H

    2001-09-01

    The emergence of coagulase-negative staphylococci not only as human pathogens but also as reservoirs of antibiotic resistance determinants requires the deployment and development of methods for their rapid and reliable identification. Internal transcribed spacer-PCR (ITS-PCR) was used to identify a collection of 617 clinical staphylococcal isolates. The amplicons were resolved in high-resolution agarose gels and visually compared with the patterns obtained for the control strains of 29 staphylococcal species. Of the 617 isolates studied, 592 (95.95%) were identified by ITS-PCR and included 11 species: 302 isolates of Staphylococcus epidermidis, 157 of S. haemolyticus, 79 of S. aureus, 21 of S. hominis, 14 of S. saprophyticus, 8 of S. warneri, 6 of S. simulans, 2 of S. lugdunensis, and 1 each of S. caprae, S. carnosus, and S. cohnii. All species analyzed had unique ITS-PCR patterns, although some were very similar, namely, the group S. saprophyticus, S. cohnii, S. gallinarum, S. xylosus, S. lentus, S. equorum, and S. chromogenes, the pair S. schleiferi and S. vitulus, and the pair S. piscifermentans and S. carnosus. Four species, S. aureus, S. caprae, S. haemolyticus, and S. lugdunensis, showed polymorphisms on their ITS-PCR patterns. ITS-PCR proved to be a valuable alternative for the identification of staphylococci, offering, within the same response time and at lower cost, higher reliability than the currently available commercial systems.

  17. The ``eco-syndrome`` and what causes it; Das Oeko-Syndrom`` und seine Ausloeser

    Energy Technology Data Exchange (ETDEWEB)

    Voack, C. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie; Borelli, S. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie; Ring, J. [Technische Univ. Muenchen (Germany). Klinik und Poliklinik fuer Dermatolologie und Allergologie

    1997-01-24

    Headache, tiredness, skin irritation etc. may be the results of adverse effects on health due to toxic substances in residential buildings and at workplaces. Possible causes of syndromes that are hard to objectify but associated with serious afflictions such as the ``eco-syndrome`` or Multiple Chemical Sensitivity (MCS) and the Sick Building Syndrome (SBS) are listed. (VHE) [Deutsch] Bei Kopfschmerzen, Muedigkeit, Hautirritationen u.ae. besteht die Moeglichkeit einer gesundheitlichen Beeintraechtigung durch Schadstoff im Wohn- oder Arbeitsbereich. Moegliche Ausloeser fuer schwer objektivierbare, aber mit hohem Leidensdruck verbundene Krankheitsbilder wie Oeko-Syndrom(Multiple Chemical Sensivity, MCS) und SBS (Sick-Building-Syndrom) werden aufgelistet. (VHE)

  18. Goldenhar syndrome

    Directory of Open Access Journals (Sweden)

    Neeraj Sharma

    2013-01-01

    Full Text Available Goldenhar syndrome is a syndrome of complex structures developing from first and second branchial arches during blastogenesis. The etiology of this rare disease is not fully understood, as it has shown itself variable genetically and of unclear causes. The disorder is characterized by a wide spectrum of symptoms and physical features that may vary greatly in range and severity from case to case. Here we present a unique case of Goldenhar syndrome with absence of left condyle, hypoplasia of the zygomatic bone, no pneumatization of the mastoid process, underdeveloped mandible, bifid tongue and the skin tags in the preauricular area.

  19. Investigation of Demodex folliculorum frequency in patients with polycystic ovary syndrome*

    OpenAIRE

    Eser, Ayla; Erpolat, Seval; Kaygusuz, Ikbal; Balci, Hatice; Kosus, Aydin

    2017-01-01

    Abstract: Background: Background: Demodex mites are acari that reside in the pilosebaceous unit of the skin and have been associated with skin disorders. Objective: The objective of this study was to investigate the prevalence of Demodex folliculorum (D. folliculorum) mites in polycystic ovary syndrome patients as well as to examine the relationship between Demodex infestation and the presence of acne and oily or dry skin types in polycystic ovary syndrome patients. Methods: 41 polycystic ...

  20. Histopathology confirms white-nose syndrome in bats in Europe.

    Science.gov (United States)

    Pikula, Jiri; Bandouchova, Hana; Novotny, Ladislav; Meteyer, Carol U; Zukal, Jan; Irwin, Nancy R; Zima, Jan; Martínková, Natália

    2012-01-01

    White-nose syndrome, associated with the fungal skin infection geomycosis, caused regional population collapse in bats in North America. Our results, based on histopathology, show the presence of white-nose syndrome in Europe. Dermatohistopathology on two bats (Myotis myotis) found dead in March 2010 with geomycosis in the Czech Republic had characteristics resembling Geomyces destructans infection in bats confirmed with white-nose syndrome in US hibernacula. In addition, a live M. myotis, biopsied for histopathology during hibernation in April 2011, had typical fungal infection with cupping erosion and invasion of muzzle skin diagnostic for white-nose syndrome and conidiospores identical to G. destructans that were genetically confirmed as G. destructans.

  1. Rauvolfia grandiflora (Apocynaceae extract interferes with staphylococcal density, enterotoxin production and antimicrobial activity

    Directory of Open Access Journals (Sweden)

    Lanamar de Almeida Carlos

    2010-10-01

    Full Text Available Staphylococci bacteria are involved in many human and animal infections and development of alternative antimicrobial drugs against pathogenic bacteria is of great interest to the pharmaceutical industry. This study investigated the in vitro effect of Rauvolfia grandiflora methanol extract (root bark fraction (RGE on the density of ATCC strains of Staphylococcus aureus and Staphylococcus epidermidis, and a clinical enterotoxin-producer, S. aureus bovine strain. The alkaloid, isoreserpiline, obtained from dichloromethane extract of R. grandiflora was ineffective against the strains tested. After incubation of staphylococci strains in the presence of 1.2 mg.mL-1 RGE, a significant inhibition of cell growth was observed using both spectrophotometry and ELISA assays. Twelve drugs were evaluated for their antimicrobial effects on culture RGE-treated cells using the disk diffusion method. Penicillin resistant strains became sensitive to the drug after RGE treatment. Furthermore, enterotoxin production by RGE-treated S. aureus was evaluated using a standardized ELISA method. Although staphylococcal LSA 88 bovine strain cells remained viable after exposure to the extract, enterotoxin production was precluded in 20% after RGE treatment. Significant interference in staphylococci cell density, drug sensitivity and enterotoxin secretion was observed after treatment. The study highlights the necessity to find new methods of disease prevention and new antibiotic therapies against staphylococcal infections.

  2. Rauvolfia grandiflora (apocynaceae) extract interferes with staphylococcal density, enterotoxin production and antimicrobial activity.

    Science.gov (United States)

    de Almeida Carlos, Lanamar; da Silva Amaral, Kenas Aguiar; Curcino Vieira, Ivo José; Mathias, Leda; Braz-Filho, Raimundo; Silva Samarão, Solange; Vieira-da-Motta, Olney

    2010-07-01

    Staphylococci bacteria are involved in many human and animal infections and development of alternative antimicrobial drugs against pathogenic bacteria is of great interest to the pharmaceutical industry. This study investigated the in vitro effect of Rauvolfia grandiflora methanol extract (root bark fraction) (RGE) on the density of ATCC strains of Staphylococcus aureus and Staphylococcus epidermidis, and a clinical enterotoxin-producer, S. aureus bovine strain. The alkaloid, isoreserpiline, obtained from dichloromethane extract of R. grandiflora was ineffective against the strains tested. After incubation of staphylococci strains in the presence of 1.2 μg.mL(-1) RGE, a significant inhibition of cell growth was observed using both spectrophotometry and ELISA assays. Twelve drugs were evaluated for their antimicrobial effects on culture RGE-treated cells using the disk diffusion method. Penicillin resistant strains became sensitive to the drug after RGE treatment. Furthermore, enterotoxin production by RGE-treated S. aureus was evaluated using a standardized ELISA method. Although staphylococcal LSA 88 bovine strain cells remained viable after exposure to the extract, enterotoxin production was precluded in 20% after RGE treatment. Significant interference in staphylococci cell density, drug sensitivity and enterotoxin secretion was observed after treatment. The study highlights the necessity to find new methods of disease prevention and new antibiotic therapies against staphylococcal infections.

  3. Contamination of beef products with staphylococcal classical enterotoxins in Egypt and Saudi Arabia

    Directory of Open Access Journals (Sweden)

    Shawish, Reyad R.

    2016-04-01

    Full Text Available Food-borne pathogens are of high concern for public health and food safety. food poisoning is one of the most economically devastating types of food poisoning globally. The purpose of this study was to detect staphylococcal classical enterotoxins (SEs in processed beef from Kingdom of Saudi Arabia (KSA and Egypt. In the present investigation a total of 250 random processed meat samples (50 each of minced meat, beef burger, beef sausage, beef kofta and beef luncheon were collected from different super markets in the study area. Using conventional cultural methods, samples were cultured for isolation and identification of . Multiplex PCR was used to detect SEs of the classical type SEA, SEB, SEC and SED from isolates.The percentage presence of in minced meat, beef burger, beef sausage, beef kofta and beef luncheon was 38%, 22%, 30%, 32% and 12%, respectively. Multiplex PCR indicated that all examined samples contain different types of classical staphylococcal enterotoxins and only minced meat samples contained all four types of toxins. Multiplex PCR is efficient in detection of SEs from food and may be used in tracing of toxins to promote food hygiene. Implications of contamination of processed meat to food hygiene in the study area are highlighted.

  4. Psychogenic Purpura (Gardner-Diamond Syndrome)

    Science.gov (United States)

    Bhattacharya, Gaurav

    2015-01-01

    Psychogenic purpura, also known as Gardner-Diamond syndrome or autoerythrocyte sensitization syndrome, is a rare condition characterized by spontaneous development of painful edematous skin lesions progressing to ecchymosis over the next 24 hours. Severe stress and emotional trauma always precede the skin lesions. The condition is most commonly seen in women, but isolated cases have been reported in adolescents and in males. Psychodermatologic evaluation and dermatology and psychiatry liaison have been successful in the treatment of these patients. This report provides an overview of psychogenic purpura and presents the case of a 15-year-old girl. PMID:26137346

  5. Identification of the first nonsense CDSN mutation with expression of a truncated protein causing peeling skin syndrome type B.

    Science.gov (United States)

    Mallet, A; Kypriotou, M; George, K; Leclerc, E; Rivero, D; Mazereeuw-Hautier, J; Serre, G; Huber, M; Jonca, N; Hohl, D

    2013-12-01

    Peeling skin disease (PSD), a generalized inflammatory form of peeling skin syndrome, is caused by autosomal recessive nonsense mutations in the corneodesmosin gene (CDSN). To investigate a novel mutation in CDSN. A 50-year-old white woman showed widespread peeling with erythema and elevated serum IgE. DNA sequencing, immunohistochemistry, Western blot and real-time polymerase chain reaction analyses of skin biopsies were performed in order to study the genetics and to characterize the molecular profile of the disease. Histology showed hyperkeratosis and acanthosis of the epidermis, and inflammatory infiltrates in the dermis. DNA sequencing revealed a homozygous mutation leading to a premature termination codon in CDSN: p.Gly142*. Protein analyses showed reduced expression of a 16-kDa corneodesmosin mutant in the upper epidermal layers, whereas the full-length protein was absent. These results are interesting regarding the genotype-phenotype correlations in diseases caused by CDSN mutations. The PSD-causing CDSN mutations identified heretofore result in total corneodesmosin loss, suggesting that PSD is due to full corneodesmosin deficiency. Here, we show for the first time that a mutant corneodesmosin can be stably expressed in some patients with PSD, and that this truncated protein is very probably nonfunctional. © 2013 British Association of Dermatologists.

  6. Novel staphylococcal glycosyltransferases SdgA and SdgB mediate immunogenicity and protection of virulence-associated cell wall proteins.

    Directory of Open Access Journals (Sweden)

    Wouter L W Hazenbos

    Full Text Available Infection of host tissues by Staphylococcus aureus and S. epidermidis requires an unusual family of staphylococcal adhesive proteins that contain long stretches of serine-aspartate dipeptide-repeats (SDR. The prototype member of this family is clumping factor A (ClfA, a key virulence factor that mediates adhesion to host tissues by binding to extracellular matrix proteins such as fibrinogen. However, the biological siginificance of the SDR-domain and its implication for pathogenesis remain poorly understood. Here, we identified two novel bacterial glycosyltransferases, SdgA and SdgB, which modify all SDR-proteins in these two bacterial species. Genetic and biochemical data demonstrated that these two glycosyltransferases directly bind and covalently link N-acetylglucosamine (GlcNAc moieties to the SDR-domain in a step-wise manner, with SdgB appending the sugar residues proximal to the target Ser-Asp repeats, followed by additional modification by SdgA. GlcNAc-modification of SDR-proteins by SdgB creates an immunodominant epitope for highly opsonic human antibodies, which represent up to 1% of total human IgG. Deletion of these glycosyltransferases renders SDR-proteins vulnerable to proteolysis by human neutrophil-derived cathepsin G. Thus, SdgA and SdgB glycosylate staphylococcal SDR-proteins, which protects them against host proteolytic activity, and yet generates major eptopes for the human anti-staphylococcal antibody response, which may represent an ongoing competition between host and pathogen.

  7. [Toxic-shock syndrome. Three cases (author's transl)].

    Science.gov (United States)

    Rapin, M; D'Enfert, J; Cabane, J

    1981-06-13

    Several cases of toxic shock syndrome (T.S.S) have been recently reported from the U.S.A. Clinical features of this new syndrome include fever, desquamative scarlatiniform rash, hypotension and involvement of central nervous system, liver, kidney and muscles. More than 90% of cases are women with staphylococcic vaginitis using tampons during menstruations. A toxin produced by staphylococcus aureus is thought to be the causative agent, because the germ has been isolated in local (vaginal, pharyngeal, subcutaneous or other sites) but not systemic (blood, cerebrospinal fluid) cultures. The mortality rate is 3-10%, and relapse can occur. We report the first three french cases of T.S.S.: a 17 year old girl with typical tampon-associated vaginitis, a 36 year old woman with a postoperative peritonitis and a 20 year old man with a popliteal abscess. Staphylococcus aureus of type I or IV was identified at the site of infection in all cases, but never in blood cultures. These three patients recovered with antistaphylococcic antibiotics and supportive therapy, but local treatment of infections seems to have been of utmost importance. These cases suggest that T.S.S. can occur with several staphylococcus serotypes and confirm that this syndrome is not always associated with tampons and vaginitis.

  8. Inflammatory peeling skin syndrome caused by homozygous genomic deletion in the PSORS1 region encompassing the CDSN gene.

    Science.gov (United States)

    Ishida-Yamamoto, Akemi; Furio, Laetitia; Igawa, Satomi; Honma, Masaru; Tron, Elodie; Malan, Valerie; Murakami, Masamoto; Hovnanian, Alain

    2014-01-01

    Peeling skin syndrome (PSS) type B is a rare recessive genodermatosis characterized by lifelong widespread, reddish peeling of the skin with pruritus. The disease is caused by small-scale mutations in the Corneodesmosin gene (CDSN) leading to premature termination codons. We report for the first time a Japanese case resulting from complete deletion of CDSN. Corneodesmosin was undetectable in the epidermis, and CDSN was unamplifiable by PCR. QMPSF analysis demonstrated deletion of CDSN exons inherited from each parent. Deletion mapping using microsatellite haplotyping, CGH array and PCR analysis established that the genomic deletion spanned 49-72 kb between HCG22 and TCF19, removing CDSN as well as five other genes within the psoriasis susceptibility region 1 (PSORS1) on 6p21.33. This observation widens the spectrum of molecular defects underlying PSS type B and shows that loss of these five genes from the PSORS1 region does not result in an additional cutaneous phenotype. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  9. Bullous Wells’ syndrome

    Directory of Open Access Journals (Sweden)

    Bengu Cevirgen Cemil

    2016-01-01

    Full Text Available Wells’ syndrome (WS is an uncommon inflammatory skin disease which typically presents single or multiple erythematous and edematous urticarial plaques similar to cellulitis. The lesions may evolve into blue-grey morphea-like lesions and may persist for weeks or months. They ultimately heal without scar. Other clinical presentations reported in literature include papular and nodular and, rarely, bullous eruptions. Previously, bullous Wells’ syndrome was rarely reported in the literature. Herein, we describe a case of a female patient with bullous Wells’ syndrome localized to the upper limbs without any associated disorders.

  10. Clinical utility of skin karyotype

    Directory of Open Access Journals (Sweden)

    Luiza E. Dorfman

    2015-08-01

    Full Text Available ABSTRACTWe report the case of a patient with Patau syndrome, diagnosed by skin karyotype, emphasizing the applications and importance of this test. The pregnancy morphology ultrasound showed face defects and of central nervous system and heart chambers asymmetry. In the postnatal evaluation it was identified microcephaly, single central nostril, and other malformations. We performed skin karyotype that resulted in full trisomy 13. Our report highlights the possibility of performing karyotype examination in cases when it is no longer possible to obtain a blood sample, thus providing the correct diagnosis and genetic counseling for the family.

  11. Anticardiolipine antibodies in skin and muscle eluates of patients with primary and secondary antiphospholipid syndrome

    Directory of Open Access Journals (Sweden)

    Z S Alekberova

    2004-01-01

    Full Text Available Objective. To detect anticardiolipin antibodies (АСА, anti-p2-GPl antibodies, C3 and C4 complement components in immune complexes including those containing АСА in skin and muscle eluates of pts with systemic lupus erythematosus (SLE and antiphospholipid syndrome (APS. Material and methods . In 7 pts (6 female and I male, 2 with primary APS, 3 with SLE+APS and 2 with SLE skin and muscle biopsies were taken. 6 from 7 pts had thrombotic complications. Eluates were obtained from frozen skin and skeletal muscle biopsies (size was 1,5x0,5 and 0,5x0,5 respectively. Because of small size of biopsies it was not possible to use traditional methods of tissue pounding such as sharp homogenization of tissues in homogenizers with pulverizing and subsequent process of freezing-unfreezing which lead to large protein loss and make impossible serological tissue analysis. Application of acid eluates method by T.E.W. Feltkamp and J,H. Boode of own modification allowed to minimize tissue protein loss and perform serological tissue analysis. Results. Serum of all 7 pts contained antiphospholipid antibodies - IgG-ACA in 3, combination of IgG- und IgM-ACA in 5. In 5 from 7 eluates lgG АСА exceeded 0,109 OO units were revealed. They contained СЗ, C4 and different protein products mostly immunoglobulines. Anti-(I2GP1 antiboddie;. were absent. Conclusion. For the first time presence of АСА in tissues of APS pts was showed which may be of particular interest in studying morphogenesis of local tissue disturbances with participation of immune complexes containing АСА.

  12. Broiler carcass contamination with Campylobacter from feces during defeathering.

    Science.gov (United States)

    Berrang, M E; Buhr, R J; Cason, J A; Dickens, J A

    2001-12-01

    Three sets of experiments were conducted to explore the increase in recovery of Campylobacter from broiler carcasses after defeathering. In the first set of experiments, live broilers obtained from a commercial processor were transported to a pilot plant, and breast skin was sampled by a sponge wipe method before and after defeathering. One of 120 broiler breast skin samples was positive for Campylobacter before defeathering, and 95 of 120 were positive after defeathering. In the second set of experiments, Campylobacter-free flocks were identified, subjected to feed withdrawal, and transported to the pilot plant. Carcasses were intracloacally inoculated with Campylobacter (10(7) CFU) just prior to entering the scald tank. Breast skin sponge samples were negative for Campylobacter before carcasses entered the picker (0 of 120 samples). After defeathering, 69 of 120 samples were positive for Campylobacter, with an average of log10 2.7 CFU per sample (approximately 30 cm2). The third set of experiments was conducted using Campylobacter-positive broilers obtained at a commercial processing plant and transported live to the pilot plant. Just prior to scalding, the cloacae were plugged with tampons and sutured shut on half of the carcasses. Plugged carcasses were scalded, and breast skin samples taken before and after defeathering were compared with those collected from control broilers from the same flock. Prior to defeathering, 1 of 120 breast skin sponge samples were positive for the control carcasses, and 0 of 120 were positive for the plugged carcasses. After passing through the picker, 120 of 120 control carcasses had positive breast skin sponge samples, with an average of log10 4.2 CFU per sample (approximately 30 cm2). Only 13 of 120 plugged carcasses had detectable numbers of Campylobacter on the breast skin sponge, with an average of log10 2.5 CFU per sample. These data indicate that an increase in the recovery of Campylobacter after defeathering can be related

  13. Skin rash and arthritis a simplified appraisal of less common associations.

    Science.gov (United States)

    Cozzi, A; Doria, A; Gisondi, P; Girolomoni, G

    2014-06-01

    Skin and joint manifestations are part of the clinical spectrum of many disorders. Well-known associations include psoriatic arthritis and arthritis associated with autoimmune connective tissue diseases. This review focuses on less common associations where skin lesions can provide easily accessible and valuable diagnostic clues, and directly lead to the specific diagnosis or limit the list of possibilities. This may also affect health care resources as diagnostic tests are often low-specific, highly expensive and poorly available. This group of diseases can be divided into two subsets, based on the presence/absence of fever, and then further classified according to elementary skin lesions (macular, urticarial, maculo-papular, vesico-bullous, pustular, petechial and nodular). In most instances joint involvement occurs as peripheral migrating polyarthritis. Erythematosus macular or urticarial rashes occur in most febrile disorders such as monogenic autoinflammatory syndromes, Schnitzler's syndrome, Still's disease and rheumatic fever and afebrile diseases as urticarial vasculitis. Pustular rash may be observed in chronic recurrent multifocal osteomyelitis (CRMO) and pyogenic arthritis with pyoderma gangrenosum and acne (PAPA) syndrome (both febrile) as well as in Behcet's disease and Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome (both non-febrile). Papular lesions are typical of secondary syphilis, sarcoidosis, interstitial granulomatous dermatitis, papular petechial of cutaneous small-vessel vasculitis and nodular lesions of polyarteritis nodosa and multicentric reticulohistiocytosis all of which are afebrile. Differential diagnosis includes infections and drug reactions which may mimic several of these conditions. To biopsy the right skin lesion at the right time it is essential to obtain relevant histological information. © 2013 European Academy of Dermatology and Venereology.

  14. Histopathology confirms white-nose syndrome in bats in Europe

    Science.gov (United States)

    Pikula, J.; Bandouchova, H.; Novotny, L.; Meteyer, C.U.; Zukal, J.; Irwin, N.R.; Zima, J.; Martinkova, N.

    2012-01-01

    White-nose syndrome, associated with the fungal skin infection geomycosis, caused regional population collapse in bats in North America. Our results, based on histopathology, show the presence of white-nose syndrome in Europe. Dermatohistopathology on two bats (Myotis myotis) found dead in March 2010 with geomycosis in the Czech Republic had characteristics resembling Geomyces destructans infection in bats confirmed with white-nose syndrome in US hibernacula. In addition, a live M. myotis, biopsied for histopathology during hibernation in April 2011, had typical fungal infection with cupping erosion and invasion of muzzle skin diagnostic for white-nose syndrome and conidiospores identical to G. destructans that were genetically confirmed as G. destructans. ?? Wildlife Disease Association 2012.

  15. An Alternative Approach to Combination Vaccines: Intradermal Administration of Isolated Components for Control of Anthrax, Botulism, Plague and Staphylococcal Toxic Shock

    National Research Council Canada - National Science Library

    Morefield, Garry L; Tammariello, Ralph F; Purcell, Bret K; Worsham, Patricia L; Chapman, Jennifer; Smith, Leonard A; Alarcon, Jason B; Mikszta, John A; Ulrich, Robert G

    2008-01-01

    ... incompatible vaccine mixtures. Intradermally administered arrays of vaccines for protection from anthrax, botulism, plague, and staphylococcal toxic shock were biocompatible in vivo, retained potent antibody responses...

  16. Unravelling the NERDS syndrome.

    Science.gov (United States)

    Singh, Achintya Dinesh; Suri, Tejas Menon; Jagdish, Rakesh Kumar; Kumar, Uma

    2018-05-12

    A 22-year-old man presented with symmetric polyarthritis, pruritus and deviation of angle of mouth to the right side since the last 7 years. His symptoms were persistent despite receiving ayurvedic medications and symptomatic therapy. Examination revealed dry skin, cutaneous nodules, xanthelasma, periarticular non-tender swellings, pitting oedema of hands and feet and lower motor neuron type right facial palsy. Haematological investigations revealed eosinophilia and skin biopsy had cutaneous eosinophilic infiltration. The constellation of above findings comprises the nodules, eosinophilia, rheumatism, dermatitis and swelling syndrome. It a rare syndrome with few reported cases in literature. The patient was started on oral corticosteroids which was subsequently tapered and methotrexate therapy. His polyarthritis and skin rashes resolved with therapy. He has been followed-up for 2 years and is presently asymptomatic for the last 1 year. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  17. Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum

    Directory of Open Access Journals (Sweden)

    Prabhas Prasun Giri

    2011-01-01

    Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS and spontaneous air leak syndrome (pnemothorax and pneumomediastinum. She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

  18. Shah-Waardenburg syndrome.

    Science.gov (United States)

    Mahmoudi, Abdelhalim; Rami, Mohamed; Khattala, Khalid; Elmadi, Aziz; Afifi, My Abderrahmane; Youssef, Bouabdallah

    2013-01-01

    Shah-Waardenburg syndrome (SWS) is a neurocristopathy and is characterized by Hirschsprung's disease (HD), deafness, and depigmentation of hairs, skin, and iris. Is a very rare congenital disorder with variable clinical expression. This report describes a 4-day-old male newborn with Waardenburg's syndrome associated with aganglionosis of the colon and terminal ileum, and review the relevant literature for draws attention to the causal relationship between these two entities.

  19. Case Report - Neonatal progeroid syndrome (Wiedemann ...

    African Journals Online (AJOL)

    Case Report - Neonatal progeroid syndrome (Wiedemann-Rautenstrauch syndrome) in an Egyptian child with premature loss of teeth, and café au lait skin ... pads in the suprabuttock areas, triangular face, pseudohydrocephalous, sparse scalp hair and eyebrows, prominent scalp veins, greatly widened anterior fontanels, ...

  20. Role of blood culture systems in the evaluation of epidemiological features of coagulase-negative staphylococcal bloodstream infection in critically ill patients.

    Science.gov (United States)

    Oud, L; Krimerman, S; Salam, N; Srugo, I

    1999-12-01

    The impact of blood culture systems on the detection of coagulase-negative staphylococcal bloodstream infections in critically ill patients prior to and following the introduction of the Bactec 9240 blood culture system (Becton Dickinson Diagnostic Instrument Systems, USA), which replaced the Bactec NR 730 (Becton Dickinson Diagnostic Instrument Systems), was investigated over a 3-year period. Following the introduction of the new culture system, the incidence of bloodstream infections doubled (P<0.001). Patient demographics, severity of illness, and mortality remained unchanged, while the annual standardized mortality ratio decreased significantly. These data suggest that blood culture systems may have a major impact on the perceived incidence of coagulase-negative staphylococcal bloodstream infections in this population.

  1. Mouse Genetic Models Reveal Surprising Functions of IκB Kinase Alpha in Skin Development and Skin Carcinogenesis

    Energy Technology Data Exchange (ETDEWEB)

    Xia, Xiaojun [The Methodist Hospital Research Institute, Houston, TX 77030 (United States); Park, Eunmi [Department of Radiation Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02115 (United States); Fischer, Susan M. [Department of Molecular Carcinogenesis, The University of Texas MD Anderson Cancer Center, Smithville, TX 78967 (United States); Hu, Yinling, E-mail: huy2@mail.nih.gov [Cancer and Inflammation Program, Center for Cancer Research, Frederick National Laboratory for Cancer Research, Frederick, MD 21701 (United States)

    2013-02-15

    Gene knockout studies unexpectedly reveal a pivotal role for IκB kinase alpha (IKKα) in mouse embryonic skin development. Skin carcinogenesis experiments show that Ikkα heterozygous mice are highly susceptible to chemical carcinogen or ultraviolet B light (UVB) induced benign and malignant skin tumors in comparison to wild-type mice. IKKα deletion mediated by keratin 5 (K5).Cre or K15.Cre in keratinocytes induces epidermal hyperplasia and spontaneous skin squamous cell carcinomas (SCCs) in Ikkα floxed mice. On the other hand, transgenic mice overexpressing IKKα in the epidermis, under the control of a truncated loricrin promoter or K5 promoter, develop normal skin and show no defects in the formation of the epidermis and other epithelial organs, and the transgenic IKKα represses chemical carcinogen or UVB induced skin carcinogenesis. Moreover, IKKα deletion mediated by a mutation, which generates a stop codon in the Ikkα gene, has been reported in a human autosomal recessive lethal syndrome. Downregulated IKKα and Ikkα mutations and deletions are found in human skin SCCs. The collective evidence not only highlights the importance of IKKα in skin development, maintaining skin homeostasis, and preventing skin carcinogenesis, but also demonstrates that mouse models are extremely valuable tools for revealing the mechanisms underlying these biological events, leading our studies from bench side to bedside.

  2. Mouse Genetic Models Reveal Surprising Functions of IκB Kinase Alpha in Skin Development and Skin Carcinogenesis

    International Nuclear Information System (INIS)

    Xia, Xiaojun; Park, Eunmi; Fischer, Susan M.; Hu, Yinling

    2013-01-01

    Gene knockout studies unexpectedly reveal a pivotal role for IκB kinase alpha (IKKα) in mouse embryonic skin development. Skin carcinogenesis experiments show that Ikkα heterozygous mice are highly susceptible to chemical carcinogen or ultraviolet B light (UVB) induced benign and malignant skin tumors in comparison to wild-type mice. IKKα deletion mediated by keratin 5 (K5).Cre or K15.Cre in keratinocytes induces epidermal hyperplasia and spontaneous skin squamous cell carcinomas (SCCs) in Ikkα floxed mice. On the other hand, transgenic mice overexpressing IKKα in the epidermis, under the control of a truncated loricrin promoter or K5 promoter, develop normal skin and show no defects in the formation of the epidermis and other epithelial organs, and the transgenic IKKα represses chemical carcinogen or UVB induced skin carcinogenesis. Moreover, IKKα deletion mediated by a mutation, which generates a stop codon in the Ikkα gene, has been reported in a human autosomal recessive lethal syndrome. Downregulated IKKα and Ikkα mutations and deletions are found in human skin SCCs. The collective evidence not only highlights the importance of IKKα in skin development, maintaining skin homeostasis, and preventing skin carcinogenesis, but also demonstrates that mouse models are extremely valuable tools for revealing the mechanisms underlying these biological events, leading our studies from bench side to bedside

  3. Outbreak of Staphylococcal food poisoning due to SEA-producing Staphylococcus aureus.

    Science.gov (United States)

    Johler, Sophia; Tichaczek-Dischinger, Petra S; Rau, Jörg; Sihto, Henna-Maria; Lehner, Angelika; Adam, Maja; Stephan, Roger

    2013-09-01

    In 2008, 150 people gathered for a wedding celebration in Baden-Württemberg, Germany. Three hours after ingestion of a variety of foods including pancakes filled with minced chicken, several guests exhibited symptoms of acute gastroenteritis such as vomiting, diarrhea, fever, and ague. Twelve guests were reported to have fallen ill, with nine of these seeking medical care in hospitals. At least four patients were admitted to the hospital and received inpatient treatment, among them a 2-year-old child and a woman in the 4th month of pregnancy. Within 24 h of the event, an investigative team collected a variety of samples including refrigerated leftovers, food in the storage unit of the caterer, nasal swabs of the caterer, as well as 21 environmental swabs. Five stool samples from patients were provided by the hospitals. Staphylococcus aureus isolates were gathered from eight samples, among them nasal swabs of the caterer, food samples, and one stool sample. Fourier transform-infrared spectroscopy was used for species identification and for primary clustering of the isolates in a similarity tree. The isolates were further characterized by spa typing and pulsed-field gel electrophoresis, and a DNA microarray was used to determine the presence/absence of genes involved in virulence and antimicrobial resistance. We were able to match an enterotoxigenic strain from the stool sample of a patient to isolates of the same strain obtained from food and the nasal cavity of a food handler. The strain produced the enterotoxin SEA and the toxic shock syndrome toxin-1, and was also found to exhibit the genes encoding enterotoxins SEG and SEI, as well as the enterotoxin gene cluster egc. This is one of only a few studies that were able to link a staphylococcal food poisoning outbreak to its source.

  4. SAPHO: syndrome or concept? Imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Earwaker, J.W.S. [Department of Medical Imaging, Holy Spirit Hospital, Brisbane (Australia); Cotten, A. [Service de Radiologie OsteoArticulaire, Hopital Roger Salengro, Lille (France)

    2003-06-01

    The SAPHO syndrome was a term coined to include a variety of musculoskeletal disorders associated with skin conditions, mainly palmoplantar pustulosis and acne conglobata. It is more correctly a spectrum which includes the following: skin lesions, osteoarticular manifestations of synovitis hyperostosis and osteitis affecting particular target sites, and.a clinical course marked by relapses and remissions. The major sites of involvement are the anterior chest wall, the spine, long bones, flat bones, and large and small joints. The distribution and severity of involvement varies from the adult to the pediatric form of chronic recurrent multifocal osteomyelitis (CRMO). The diagnosis of SAPHO syndrome is not difficult when the typical osteoarticular lesions are located in characteristic target sites. The diagnosis is more difficult if atypical sites are involved and there is no skin disease. (orig.)

  5. Short communication: Pasteurization as a means of inactivating staphylococcal enterotoxins A, B, and C in milk.

    Science.gov (United States)

    Necidova, Lenka; Bogdanovicova, Katerina; Harustiakova, Danka; Bartova, Katerina

    2016-11-01

    Our aim was to assess the effect of pasteurization temperature on inactivation of staphylococcal enterotoxins (SE). Milk samples were inoculated with log 4.38 to 5.18cfu/mL of 40 different Staphylococcus aureus strains having the ability to produce types A, B, or C SE and incubated at 37°C for 24h to develop SE. This incubation was followed by heat treatment for 15 s at 72, 85, and 92°C. Samples were analyzed for Staph. aureus count by plate method and, specifically, for SE presence. An enzyme-linked immunofluorescent assay on a MiniVIDAS analyzer (bioMérieux, Marcy l'Étoile, France) was used to detect SE, which were determined semiquantitatively based on test values. The Staph. aureus count in milk before pasteurization did not affect the amount of SE. Before pasteurization, SEB was detected in the lowest amount compared with other SE types. Staphylococcal enterotoxins were markedly reduced with pasteurization and inactivated at pasteurization temperatures to an extent depending on the amount in the sample before pasteurization. After pasteurization at 72°C, SE were detected in 87.5% of samples (35/40), after pasteurization at 85°C in 52.5% of samples (21/40), and after pasteurization at 92°C in 45.0% of samples (18/40). We determined that SE may still persist in milk even when Staph. aureus bacteria are inactivated through pasteurization. Although pasteurization may partially inactivate SE in milk, a key measure in the prevention of staphylococcal enterotoxicosis linked to pasteurized milk consumption is to avoid any cold chain disruption during milk production and processing. Copyright © 2016 American Dairy Science Association. Published by Elsevier Inc. All rights reserved.

  6. Novel types of staphylococcal cassette chromosome mec elements identified in clonal complex 398 methicillin-resistant Staphylococcus aureus strains.

    NARCIS (Netherlands)

    Li, S.; Skov, R.L.; Han, X.; Larsen, A.R.; Larsen, J.; Sorum, M.; Wulf, M.; Voss, A.; Hiramatsu, K.; Ito, T.

    2011-01-01

    The structures of staphylococcal cassette chromosome mec (SCCmec) elements carried by 31 clonal complex 398 (CC398) methicillin-resistant Staphylococcus aureus (MRSA) strains isolated from the participants at a conference were analyzed. The SCCmecs were classified into novel types, namely, IX, X,

  7. Genetics Home Reference: Noonan syndrome with multiple lentigines

    Science.gov (United States)

    ... of Noonan syndrome with multiple lentigines include brown skin spots called lentigines that are similar to freckles, heart ... individuals may have thousands of small dark brown skin spots by the time they reach puberty. Unlike freckles, ...

  8. Imaging mass spectrometry visualizes ceramides and the pathogenesis of dorfman-chanarin syndrome due to ceramide metabolic abnormality in the skin.

    Directory of Open Access Journals (Sweden)

    Naoko Goto-Inoue

    Full Text Available Imaging mass spectrometry (IMS is a useful cutting edge technology used to investigate the distribution of biomolecules such as drugs and metabolites, as well as to identify molecular species in tissues and cells without labeling. To protect against excess water loss that is essential for survival in a terrestrial environment, mammalian skin possesses a competent permeability barrier in the stratum corneum (SC, the outermost layer of the epidermis. The key lipids constituting this barrier in the SC are the ceramides (Cers comprising of a heterogeneous molecular species. Alterations in Cer composition have been reported in several skin diseases that display abnormalities in the epidermal permeability barrier function. Not only the amounts of different Cers, but also their localizations are critical for the barrier function. We have employed our new imaging system, capable of high-lateral-resolution IMS with an atmospheric-pressure ionization source, to directly visualize the distribution of Cers. Moreover, we show an ichthyotic disease pathogenesis due to abnormal Cer metabolism in Dorfman-Chanarin syndrome, a neutral lipid storage disorder with ichthyosis in human skin, demonstrating that IMS is a novel diagnostic approach for assessing lipid abnormalities in clinical setting, as well as for investigating physiological roles of lipids in cells/tissues.

  9. Artificial biofilms establish the role of matrix interactions in staphylococcal biofilm assembly and disassembly

    Science.gov (United States)

    Stewart, Elizabeth J.; Ganesan, Mahesh; Younger, John G.; Solomon, Michael J.

    2015-01-01

    We demonstrate that the microstructural and mechanical properties of bacterial biofilms can be created through colloidal self-assembly of cells and polymers, and thereby link the complex material properties of biofilms to well understood colloidal and polymeric behaviors. This finding is applied to soften and disassemble staphylococcal biofilms through pH changes. Bacterial biofilms are viscoelastic, structured communities of cells encapsulated in an extracellular polymeric substance (EPS) comprised of polysaccharides, proteins, and DNA. Although the identity and abundance of EPS macromolecules are known, how these matrix materials interact with themselves and bacterial cells to generate biofilm morphology and mechanics is not understood. Here, we find that the colloidal self-assembly of Staphylococcus epidermidis RP62A cells and polysaccharides into viscoelastic biofilms is driven by thermodynamic phase instability of EPS. pH conditions that induce phase instability of chitosan produce artificial S. epidermidis biofilms whose mechanics match natural S. epidermidis biofilms. Furthermore, pH-induced solubilization of the matrix triggers disassembly in both artificial and natural S. epidermidis biofilms. This pH-induced disassembly occurs in biofilms formed by five additional staphylococcal strains, including three clinical isolates. Our findings suggest that colloidal self-assembly of cells and matrix polymers produces biofilm viscoelasticity and that biofilm control strategies can exploit this mechanism. PMID:26272750

  10. The staphylococcal enterotoxin (SE) family: SEB and siblings.

    Science.gov (United States)

    Krakauer, Teresa; Stiles, Bradley G

    2013-11-15

    Staphylococcus aureus plays an important role in numerous human cases of food poisoning, soft tissue, and bone infections, as well as potentially lethal toxic shock. This common bacterium synthesizes various virulence factors that include staphylococcal enterotoxins (SEs). These protein toxins bind directly to major histocompatibility complex class II on antigen-presenting cells and specific Vβ regions of T-cell receptors, resulting in potentially life-threatening stimulation of the immune system. Picomolar concentrations of SEs ultimately elicit proinflammatory cytokines that can induce fever, hypotension, multi-organ failure, and lethal shock. Various in vitro and in vivo models have provided important tools for studying the biological effects of, as well as potential vaccines/therapeutics against, the SEs. This review succinctly presents known physical and biological properties of the SEs, including various intervention strategies. In particular, SEB will often be portrayed as per biodefense concerns dating back to the 1960s.

  11. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) with cranial vault plasmocytoma and the role of surgery in its management: a case report.

    Science.gov (United States)

    Plata Bello, Julio; Garcia-Marin, Victor

    2013-10-18

    POEMS syndrome (an acronym of polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) is a paraneoplastic disorder related to an underlying plasma cell dyscrasia. The development of such a syndrome is rare and its association with calvarial plasmocytoma is even less common, with only two previous reported cases. We describe, in detail, an unusual presentation of cranial plasmocytoma associated with POEMS syndrome and briefly discuss the possible role of surgery in the management of this disease. We present the case of a 45-year-old Caucasian man who was admitted to our department presenting with progressive weakness in his lower limbs, enlarged lymph nodes and a large mass on the scalp with intense bone erosion. POEMS criteria were present and pathological studies confirmed a Castleman's variant plasmocytoma. Clinical status improved noticeably after the excision of the plasmocytoma and the treatment was completed with radiotherapy and steroid pulse therapy. Cranial vault plasmocytoma and its association with POEMS syndrome are rare conditions with few previously reported cases. Although the role of surgery is not clearly defined in POEMS syndrome guidelines, the fact that there seems to be a better prognosis and clinical outcome when surgery is used as a part of the management in POEMS syndrome with cranial vault plasmocytoma is worth discussing.

  12. Novel staphylococcal glycosyltransferases SdgA and SdgB mediate immunogenicity and protection of virulence-associated cell wall proteins

    NARCIS (Netherlands)

    Hazenbos, Wouter L. W.; Kajihara, Kimberly K.; Vandlen, Richard; Morisaki, J. Hiroshi; Lehar, Sophie M.; Kwakkenbos, Mark J.; Beaumont, Tim; Bakker, Arjen Q.; Phung, Qui; Swem, Lee R.; Ramakrishnan, Satish; Kim, Janice; Xu, Min; Shah, Ishita M.; Diep, Binh An; Sai, Tao; Sebrell, Andrew; Khalfin, Yana; Oh, Angela; Koth, Chris; Lin, S. Jack; Lee, Byoung-Chul; Strandh, Magnus; Koefoed, Klaus; Andersen, Peter S.; Spits, Hergen; Brown, Eric J.; Tan, Man-Wah; Mariathasan, Sanjeev

    2013-01-01

    Infection of host tissues by Staphylococcus aureus and S. epidermidis requires an unusual family of staphylococcal adhesive proteins that contain long stretches of serine-aspartate dipeptide-repeats (SDR). The prototype member of this family is clumping factor A (ClfA), a key virulence factor that

  13. Because You Asked about Waardenburg Syndrome.

    Science.gov (United States)

    National Inst. on Deafness and Other Communications Disorders, Bethesda, MD.

    This pamphlet uses a question-and-answer format to provide information about Waardenburg syndrome, an inherited disorder often characterized by varying degrees of hearing loss and changes in skin and hair pigmentation. The pamphlet covers: causes of Waardenburg syndrome. characteristics, types, research being done, ways to help in research…

  14. Artefactual skin lesions in children and adolescents

    DEFF Research Database (Denmark)

    Ring, Hans Christian; Miller, Iben M; Benfeldt, Eva

    2015-01-01

    BACKGROUND: Self harm is a great diagnostic and treatment challenge. In addition, psychocutaneous conditions are rare in the pediatric population and may therefore be misdiagnosed. Dermatitis artefacta is a psychocutaneous syndrome, which is a subgroup of the general spectrum of self-inflicted skin...

  15. Bullous eosinophilic cellulitis (Wells' syndrome) associated with Churg-Strauss syndrome

    NARCIS (Netherlands)

    Schuttelaar, M L A; Jonkman, M F

    We report a patient with Churg-Strauss syndrome (CSS) with asthma, eosinophilia, nasal polyposis and ANCA-associated multisystem vasculitis, who's skin eruption started with erythematous urticarial-plaques followed by haemorrhagic bullae. Histology of the plaques revealed 'flame figures' in the

  16. Cross-Talk between Staphylococcus aureus and Other Staphylococcal Species via the agr Quorum Sensing System

    Directory of Open Access Journals (Sweden)

    Jaime Canovas

    2016-11-01

    Full Text Available Staphylococci are associated with both humans and animals. While most are non-pathogenic colonizers, Staphylococcus aureus is an opportunistic pathogen capable of causing severe infections. S. aureus virulence is controlled by the agr quorum sensing system responding to secreted auto-inducing peptides (AIPs sensed by AgrC, a two component histidine kinase. agr loci are found also in other staphylococcal species and for Staphylococcus epidermidis, the encoded AIP represses expression of agr regulated virulence genes in S. aureus. In this study we aimed to better understand the interaction between staphylococci and S. aureus, and show that this interaction may eventually lead to the identification of new anti-virulence candidates to target S. aureus infections. Here we show that culture supernatants of 37 out of 52 staphylococcal isolates representing 17 different species inhibit S. aureus agr. The dog pathogen, Staphylococcus schleiferi, expressed the most potent inhibitory activity and was active against all four agr classes found in S. aureus. By employing a S. aureus strain encoding a constitutively active AIP receptor we show that the activity is mediated via agr. Subsequent cloning and heterologous expression of the S. schleiferi AIP in S. aureus demonstrated that this molecule was likely responsible for the inhibitory activity, and further proof was provided when pure synthetic S. schleiferi AIP was able to completely abolish agr induction of an S. aureus reporter strain. To assess impact on S. aureus virulence, we co-inoculated S. aureus and S. schleiferi in vivo in the Galleria mellonella wax moth larva, and found that expression of key S. aureus virulence factors was abrogated. Our data show that the S. aureus agr locus is highly responsive to other staphylococcal species suggesting that agr is an inter-species communication system. Based on these results we speculate that interactions between S. aureus and other colonizing staphylococci

  17. The radiological and clinical features of Gardner's syndrome

    International Nuclear Information System (INIS)

    Peters, P.E.; Gaebler, J.; Lingemann, B.; Ritter, W.; Muenster Univ.; Muenster Univ.

    1982-01-01

    Gardner's syndrome, completely expressed, consists of a trio of familial polyposis of the colon, osteomas and mesenchymal tumours of the skin. Inheritence is autosomal dominant. In many patients with familial polyposis of the colon, only mesenchymal skin tumours or osteomas can be demonstrated. It is therefore possible that Gardner's syndrome and familial polyposis represent two extremities of a single disease which is characterised by marked variability in the expressivity of the gene. Gardner's syndrome has been considered a rare condition occurring in only about 8% of patients with familial polyposis. Amongst the 20 patients with colonic polyposis from eleven families, mesenchymal and/or osseous lesions were found in seventeen (85%). Osteomas of the mandible were shown particularly frequently by orthopantomography. Since polyposis of the colon tends to remain symptomless for many years, the finding of oesteomas in the facial skeleton, or recurrent skin tumours in young patients, should lead to further investigation. (orig.) [de

  18. Gorlin syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Patil K

    2005-01-01

    Full Text Available Gorlin syndrome is an autosomal dominant inherited condition that exhibits high penetrance and variable expressivity. It is characterized mainly by Basal cell carcinomas, Odontogenic keratocysts and skeletal anomalies. However, medical literature documents both common and lesser known manifestations of the disorder involving the skin, central nervous system, skeletal system etc. Diagnosis of the syndrome in childhood is basically through oral abnormalities. A case of Gorlin syndrome has been reported here, with review of literature.

  19. Food Grade Pimenta Leaf Essential Oil Reduces the Attachment of Salmonella enterica Heidelberg (2011 Ground Turkey Outbreak Isolate on to Turkey Skin

    Directory of Open Access Journals (Sweden)

    Divek V. T. Nair

    2017-11-01

    Full Text Available Salmonella attached to the poultry skin is a major source of carcass contamination during processing. Once attached to the poultry skin, it is difficult to detach and inactivate Salmonella by commonly used antimicrobial agents since the pathogen is entrapped deeply in the feather follicles and the crevices on the skin. Essential oils could be natural, safe, and effective alternatives to synthetic antimicrobial agents during commercial and organic processing setup. The present study evaluated the efficacy of pimenta (Pimenta officinalis Lindl. leaf essential oil (PEO, and its nanoemulsion in reducing Salmonella Heidelberg attachment on to turkey (Meleagris gallopavo skin during simulated scalding (65°C and chilling (4°C steps in poultry processing. A multidrug resistant S. Heidelberg isolate from the 2011 ground turkey outbreak in the United States was used in the study. Results showed that PEO and the nanoemulsion resulted in significant reduction of S. Heidelberg attachment on turkey skin. Turkey skin samples treated with 1.0% PEO for 5 min resulted in >2 log10 CFU/sq. inch reduction of S. Heidelberg at 65 and 4°C, respectively (n = 6; P < 0.05. Similarly, skin samples treated with 1.0% pimenta nanoemulsion (PNE for 5 min resulted in 1.5- and 1.8- log10 CFU/sq. inch reduction of S. Heidelberg at 65 and 4°C, respectively (n = 6; P < 0.05. In addition, PEO and PNE were effective in reducing S. Heidelberg on skin during short-term storage at 4 and 10°C (temperature abuse (n = 6; P < 0.05. No Salmonella was detected in the dipping solution containing 0.5 or 1.0% PEO or PNE, whereas a substantial population of the pathogen survived in the control dipping solution. The results were validated using scanning electron -, and confocal - microscopy techniques. PEO or PNE could be utilized as an effective antimicrobial agent to reduce S. Heidelberg attachment to turkey skin during poultry processing.

  20. Novel Surgical Treatment for Refractory Heel Ulcers in Werner’s Syndrome

    Directory of Open Access Journals (Sweden)

    Keisuke Oe

    2013-01-01

    Full Text Available Patients with Werner’s syndrome frequently develop chronic leg ulcers that heal poorly. We present a patient who suffered from this rare syndrome and developed typical heel ulcers. Treatment of the ulcer is challenging, as flap options are limited over the lower third of the leg and skin grafting is not easy as there is a lack of healthy granulations. We successfully treated the ulcer with osteomyelitis by drilling the bone and applying an ultrathin split thickness skin graft with the thigh skin as the donor site.

  1. Changes in bacterial flora associated with skin damage on hands of health care personnel.

    Science.gov (United States)

    Larson, E L; Hughes, C A; Pyrek, J D; Sparks, S M; Cagatay, E U; Bartkus, J M

    1998-10-01

    In a prospective observational study of 40 nurses (20 with diagnosed hand irritation and 20 without), nurses with damaged hands did not have higher microbial counts (P = .63), but did have a greater number of colonizing species (means: 3.35 and 2.63, P = .03). Although numbers were small, nurses with damaged hands were significantly more likely to be colonized with Staphylococcus hominis (P = .03). Fifty-nine percent of S hominis isolates from nurses with damaged hands were resistant to methicillin compared with 27% of isolates from those with healthy skin (P = .14). Twenty percent of nurses with damaged hands were colonized with Staphylococcus aureus compared with none of the nurses with normal hands (P = .11). Nurses with damaged hands were also twice as likely to have gram-negative bacteria (P = .20), entercocci (P = .13), and Candida (P = .30) present on the hands. Antimicrobial resistance of the coagulase-negative staphylococcal flora (with the exception of S hominis) did not differ between the 2 groups, nor did a trend toward increasing resistance exist when compared with other studies during the past decade. Skin moisturizers and protectant products were used almost universally by nurses at work, primarily products brought from home. Efforts to improve hand condition are warranted because skin damage can change microbial flora. Such efforts should include assessment or monitoring of hand care practices, formal institutional policy adoption and control of use of skin protectant products or lotions, and prudent use of latex gloves or more widespread use of powder-free and nonlatex products.

  2. Granulomatous Lymphoproliferative Disorders: Granulomatous Slack Skin and Lymphomatoid Granulomatosis.

    Science.gov (United States)

    Gangar, Pamela; Venkatarajan, Sangeetha

    2015-07-01

    Granulomatous cutaneous T-cell lymphomas (CTCL) and lymphomatoid granulomatosis are considered granulomatous lymphoproliferative disorders. The most common types of granulomatous CTCL are granulomatous mycosis fungoides and granulomatous slack skin. Lymphomatoid granulomatosis is a rare Epstein-Barr virus driven lymphoproliferative disorder. This article reviews the etiopathogenesis, clinical presentation, systemic associations, and management of both granulomatous slack skin syndrome and lymphomatoid granulomatosis. Copyright © 2015 Elsevier Inc. All rights reserved.

  3. Staphylococcal Enterotoxins, Toxic Shock Syndrome Toxin-I, and Streptococcal Pyrogenic Exotoxins: Some Basic Biology of Bacterial Superantigens

    National Research Council Canada - National Science Library

    Krakauer, Teresa

    2003-01-01

    Staphylococcus aureus and Streptococcus pyogenes are facultative gram-positive cocci that play an important role in a myriad of human illnesses, including food poisoning, skin infections, pharyngitis...

  4. Ehlers- Danlos Syndrome

    Directory of Open Access Journals (Sweden)

    Prasanta Basak

    1989-01-01

    Full Text Available A female patient had Ehlers-Danlos syndrome type II since infancy, manifesting with hyperextensible skin and ciagarette paper scars at the sites of trauma. Treatment with vitamin C 1 gm a day seemed to be useful.

  5. Ehlers-Danlos' Syndrom

    DEFF Research Database (Denmark)

    Leganger, Julie; Søborg, Marie-Louise Kulas; Farholt, Stense

    2016-01-01

    Ehlers-Danlos syndrome (EDS) comprises a group of diseases characterized by connective tissue fragility. The clinical symptoms primarily involve the skin, joints, blood vessels and internal organs. Diagnosing EDS is complicated because of the clinical variability, imprecise diagnostic criteria...

  6. Comèl-Netherton syndrome – case report

    Directory of Open Access Journals (Sweden)

    Izabela Błażewicz

    2014-11-01

    Full Text Available Introduction. Comèl-Netherton syndrome is a rare autosomal recessive genodermatosis characterized by congenital ichthyosiform erythroderma, ichthyosis linearis circumflexa, trichorrhexis invaginata, and atopic diathesis. Comèl-Netherton syndrome is caused by mutations of the SPINK5 gene, which encodes the serine protease inhibitor LEKTI. Objective. We present diagnostic and therapeutic difficulties in a patient with Comèl-Netherton syndrome. Case report. We present the case of a 3-year-old boy, in whom from the first day of life generalized ichthyosiform erythroderma and diffuse exfoliation of the skin were observed. The differential diagnosis included Omenn syndrome, and atopic and seborrheic dermatitis. Finally, based on the overall clinical picture and microscopic examination of the hair, which showed the presence of bamboo hair, Comèl-Netherton syndrome was diagnosed. Conclusions . Because of similarity to other erythroderma, diagnosis of Comèl-Netherton syndrome in the first months of life creates diagnostic problems. In older children, recurrent skin infections and atopic diathesis can cause therapeutic difficulties and require the cooperation of many specialists.

  7. Lethal genes surviving by mosaicism: a possible explanation for sporadic birth defects involving the skin.

    Science.gov (United States)

    Happle, R

    1987-04-01

    A genetic concept is advanced to explain the origin of several sporadic syndromes characterized by a mosaic distribution of skin defects. It is postulated that these disorders are due to the action of a lethal gene surviving by mosaicism. The presence of the mutation in the zygote will lead to death of the embryo at an early stage of development. Cells bearing the mutation can survive only in a mosaic state, in close proximity with normal cells. The mosaic may arise either from a gametic half chromatid mutation or from an early somatic mutation. This concept of origin is proposed to apply to the Schimmelpenning-Feuerstein-Mims syndrome, the McCune-Albright syndrome, the Klippel-Trenaunay syndrome, the Sturge-Weber syndrome, and neurocutaneous melanosis. Moreover, this etiologic hypothesis may apply to two other birth defects that have recently been delineated, the Proteus syndrome (partial gigantism of hands or feet, hemihypertrophy, macrocephaly, linear papillomatous epidermal nevus, subcutaneous hemangiomas and lipomas, accelerated growth, and visceral anomalies), and the Delleman-Oorthuys syndrome (orbital cyst, porencephaly, periorbital appendages, and focal aplasia of the skin.

  8. Reiter's Syndrome.

    Science.gov (United States)

    Savant, S S; Fernandez, J C; Dhurandhar, M W; Fernandez, R J

    1979-01-01

    A case of Reiter's syndrome occurring in a young mate aged 20 years having extensive skin lesions of keratoderina blenoffhagica is presented along with a review of literature. Although urethritis was absent, other clinical and histopathological features of the cutaneous lesions led us to the diagnosis. The-possible relationship of postural psoriasis to Reiter's syndrome is discussed. Failure of the patient to respond satisfactorily to steroids, antibiotics etc, prompted the use of rnethotrexate in the case. The result was dramatic, as the patient completely recovered within ten days of starting treatment.

  9. Sulfasalazine Attenuates Staphylococcal Enterotoxin B-Induced Immune Responses

    Directory of Open Access Journals (Sweden)

    Teresa Krakauer

    2015-02-01

    Full Text Available Staphylococcal enterotoxin B (SEB and related exotoxins are important virulence factors produced by Staphylococcus aureus as they cause human diseases such as food poisoning and toxic shock. These toxins bind directly to cells of the immune system resulting in hyperactivation of both T lymphocytes and monocytes/macrophages. The excessive release of proinflammatory cytokines from these cells mediates the toxic effects of SEB. This study examined the inhibitory activities of an anti-inflammatory drug, sulfasalazine, on SEB-stimulated human peripheral blood mononuclear cells (PBMC. Sulfasalazine dose-dependently inhibited tumor necrosis factor α, interleukin 1 (IL-1 β, IL-2, IL-6, interferon γ (IFNγ, and various chemotactic cytokines from SEB-stimulated human PBMC. Sulfasalazine also potently blocked SEB-induced T cell proliferation and NFκB activation. These results suggest that sulfasalazine might be useful in mitigating the toxic effects of SEB by blocking SEB-induced host inflammatory cascade and signaling pathways.

  10. Magnetic Bead and Fluorescent Silica Nanoparticles Based Optical Immunodetection of Staphylococcal Enterotoxin B (SEB in Bottled Water

    Directory of Open Access Journals (Sweden)

    Shiva K. RASTOGI

    2009-10-01

    Full Text Available Staphylococcal enterotoxins (SEs are a major cause of food-borne diseases, most commonly SEs assayed immunologically with ELISA. An immunoassay based on fluorescein dye doped silica dioxide nanoparticles (F-SiNPs and magnetic bead (MB is described here for the detection of staphylococcal enterotoxin B (SEB. F-SiNPs have unique optical properties which make them attractive for biosensing. The water-in-oil (W/O reverse microemulsion method was used for the synthesis of F-SiNPs (~ 95 nm of diameter. The F-SiNPs were characterized using SEM, TEM and FTIR spectroscopy. The detection of SEB is preformed in PBS buffer, and bottled drinking water using sandwich immunoassay format. Target analytes were captured using MBs modified with the antigen-specific “capture” antibody, and detected using F-SiNP labeled secondary antigen-specific antibody. We report a limit of detection down to 1 ng/mL SEB spiked sample in less than 2 hr assay time using fluorocount method. This study demonstrates the bio warfare agent SEB capture by magnetic beads and detection using F-SiNPs.

  11. Association of Shah-Waardenburgh syndrome: a review of 6 cases.

    Science.gov (United States)

    Jan, Iftikhar A; Stroedter, Lutz; Haq, Anwaar-ul; Din, Zaheer-ud

    2008-04-01

    Shah-Waardenburg syndrome (SWS) is a neurocristopathy and is characterized by Hirschsprung's disease (HD), deafness, and depigmentation of hairs, skin, and iris. The aim of the article is to study the relative frequency of associations in 6 consecutive cases of SWS. A review of 6 consecutive patients with SWS was performed to study the frequency of various components of the syndrome. Six patients had features of SWS. All patients had HD; of these, 3 had rectosigmoid HD, whereas 3 had extended HD. All patients had white forelock of hairs with skin depigmentation. One patient had sensorineural deafness, whereas other babies were less than 1 year, and thus, full evaluation of hearing deficiency was not assessed. Three patients had blue eyes, whereas other babies had normal iris pigmentation. Skin depigmentation was noted in 5 of the 6 patients. Three babies were seriously malnourished and showed higher association of enterocolitis. Shah-Waardenburg syndrome is an uncommon association of HD. Depigmentation with a white forelock and skin manifestations are common, whereas blue iris, long segment disease, and enterocolitis are present in nearly half of the patients.

  12. Ehlers-Danlos Syndrome in Orthopaedics

    Science.gov (United States)

    Shirley, Eric D.; DeMaio, Marlene; Bodurtha, Joanne

    2012-01-01

    Ehlers-Danlos syndrome is a heterogeneous connective tissue condition characterized by varying degrees of skin hyperextensibility, joint hypermobility, and vascular fragility. Joint dislocations, musculoskeletal pain, atrophic scars, easy bleeding, vessel/viscera rupture, severe scoliosis, and obstetric complications may occur. These manifestations are secondary to abnormal collagen, with specific molecular defects in types I, III, and V collagen; they may also be related to tenascin-X, which has been identified in some patients. Ehlers-Danlos syndrome has been classified into 6 types, with variable degrees of joint instability, skin hyperextensibility, wound healing difficulty, and vascular fragility. Diagnosis begins with recognition of the signs and symptoms of global hypermobility and referring appropriate patients for genetic consultation. It is important to accurately identify patients with Ehlers-Danlos syndrome to initiate appropriate musculoskeletal treatment, optimize anesthetic and postoperative management, perform appropriate vascular screening, and help families address their concerns with other families and advocacy groups. PMID:23016112

  13. Skin lesions in European hibernating bats associated with Geomyces destructans, the etiologic agent of white-nose syndrome.

    Science.gov (United States)

    Wibbelt, Gudrun; Puechmaille, Sébastien J; Ohlendorf, Bernd; Mühldorfer, Kristin; Bosch, Thijs; Görföl, Tamás; Passior, Karsten; Kurth, Andreas; Lacremans, Daniel; Forget, Frédéric

    2013-01-01

    White-nose syndrome (WNS) has claimed the lives of millions of hibernating insectivorous bats in North America. Its etiologic agent, the psychrophilic fungus Geomyces destructans, causes skin lesions that are the hallmark of the disease. The fungal infection is characterized by a white powdery growth on muzzle, ears and wing membranes. While WNS may threaten some species of North American bats with regional extinction, infection in hibernating bats in Europe seems not to be associated with significant mortality. We performed histopathological investigations on biopsy samples of 11 hibernating European bats, originating from 4 different countries, colonized by G. destructans. One additional bat was euthanized to allow thorough examination of multiple strips of its wing membranes. Molecular analyses of touch imprints, swabs and skin samples confirmed that fungal structures were G. destructans. Additionally, archived field notes on hibernacula monitoring data in the Harz Mountains, Germany, over an 11-year period (2000-2011) revealed multiple capture-recapture events of 8 banded bats repeatedly displaying characteristic fungal colonization. Skin lesions of G. destructans-affected hibernating European bats are intriguingly similar to the epidermal lesions described in North American bats. Nevertheless, deep invasion of fungal hyphae into the dermal connective tissue with resulting ulceration like in North American bats was not observed in the biopsy samples of European bats; all lesions found were restricted to the layers of the epidermis and its adnexae. Two bats had mild epidermal cupping erosions as described for North American bats. The possible mechanisms for any difference in outcomes of G. destructans infection in European and North American bats still need to be elucidated.

  14. Skin lesions in European hibernating bats associated with Geomyces destructans, the etiologic agent of white-nose syndrome.

    Directory of Open Access Journals (Sweden)

    Gudrun Wibbelt

    Full Text Available White-nose syndrome (WNS has claimed the lives of millions of hibernating insectivorous bats in North America. Its etiologic agent, the psychrophilic fungus Geomyces destructans, causes skin lesions that are the hallmark of the disease. The fungal infection is characterized by a white powdery growth on muzzle, ears and wing membranes. While WNS may threaten some species of North American bats with regional extinction, infection in hibernating bats in Europe seems not to be associated with significant mortality. We performed histopathological investigations on biopsy samples of 11 hibernating European bats, originating from 4 different countries, colonized by G. destructans. One additional bat was euthanized to allow thorough examination of multiple strips of its wing membranes. Molecular analyses of touch imprints, swabs and skin samples confirmed that fungal structures were G. destructans. Additionally, archived field notes on hibernacula monitoring data in the Harz Mountains, Germany, over an 11-year period (2000-2011 revealed multiple capture-recapture events of 8 banded bats repeatedly displaying characteristic fungal colonization. Skin lesions of G. destructans-affected hibernating European bats are intriguingly similar to the epidermal lesions described in North American bats. Nevertheless, deep invasion of fungal hyphae into the dermal connective tissue with resulting ulceration like in North American bats was not observed in the biopsy samples of European bats; all lesions found were restricted to the layers of the epidermis and its adnexae. Two bats had mild epidermal cupping erosions as described for North American bats. The possible mechanisms for any difference in outcomes of G. destructans infection in European and North American bats still need to be elucidated.

  15. A pregnancy-associated nonfamilial case of PAPA (pyogenic sterile arthritis, pyoderma gangrenosum, acne) syndrome.

    Science.gov (United States)

    Horiuchi, Isao; Fukatsu, Yuko; Ushijima, Junko; Nakamura, Eishin; Samajima, Koki; Kadowaki, Kanako; Takagi, Kenjiro

    2016-10-01

    Little is known about the influence of pregnancy on pyogenic sterile arthritis, pyoderma gangrenosum, acne (PAPA) syndrome. We experienced a rare case of pregnancy complicated with PAPA syndrome. The patient had various histories of skin and joint disorders and experienced subarachnoid hemorrhage during pregnancy; however, her skin lesion was unaffected.

  16. Evaluation of Potential Effects of NaCl and Sorbic Acid on Staphylococcal Enterotoxin A Formation

    Directory of Open Access Journals (Sweden)

    Nikoleta Zeaki

    2015-09-01

    Full Text Available The prophage-encoded staphylococcal enterotoxin A (SEA is recognized as the main cause of staphylococcal food poisoning (SFP, a common foodborne intoxication disease, caused by Staphylococcus aureus. Studies on the production of SEA suggest that activation of the SOS response and subsequent prophage induction affect the regulation of the sea gene and the SEA produced, increasing the risk for SFP. The present study aims to evaluate the effect of NaCl and sorbic acid, in concentrations relevant to food production, on SOS response activation, prophage induction and SEA production. The impact of stress was initially evaluated on steady state cells for a homogenous cell response. NaCl 2% was found to activate the SOS response, i.e., recA expression, and trigger prophage induction, in a similar way as the phage-inducer mitomycin C. In contrast, sorbic acid decreased the pH of the culture to a level where prophage induction was probably suppressed, even when combined with NaCl stress. The impact of previous physiological state of the bacteria was also addressed on cells pre-exposed to NaCl, and was found to potentially affect cell response upon exposure to further stress. The results obtained highlight the possible SFP-related risks arising from the use of preservatives during food processing.

  17. Skin manifestations of growth hormone-induced diseases.

    Science.gov (United States)

    Kanaka-Gantenbein, Christina; Kogia, Christina; Abdel-Naser, Mohamed Badawy; Chrousos, George P

    2016-09-01

    The human skin is a well-organized organ bearing different types of cells in a well-structured interference to each other including epidermal and follicular keratinocytes, sebocytes, melanocytes, dermal papilla cells and fibroblasts, endothelial cells, sweat gland cells as well as nerves. Several hormones act on different cell types of the skin, while it is also considered an endocrine organ secreting hormones that act at several sites of the organism. GH receptors are found in almost all cell types forming the skin, while IGF-1 receptors' expression is restricted to the epidermal keratinocytes. Both Growth Hormone (GH) excess, as in the case of Acromegaly in adults, or Gigantism in growing children, and GH deficiency states lead to skin manifestations. In case of GH excess the main dermatological findings are skin thickening, coarsening of facial features, acrochordons, puffy hands and feet, oily skin and hyperhidrosis, while GH deficiency, on the contrary, is characterized by thin, dry skin and disorder of normal sweating. Moreover, special disorders associated with GH excess may have specific characteristics, as is the case of café-au-lait spots in Neurofibromatosis, or big café-au-lait skin hyperpigmented regions with irregular margins, as is the case in McCune-Albright syndrome. Meticulous examination of the skin may therefore contribute to the final diagnosis in cases of GH-induced disorders.

  18. Characterization of fatty acid modifying enzyme activity in staphylococcal mastitis isolates and other bacteria

    Directory of Open Access Journals (Sweden)

    Lu Thea

    2012-06-01

    Full Text Available Abstract Background Fatty acid modifying enzyme (FAME has been shown to modify free fatty acids to alleviate their bactericidal effect by esterifying fatty acids to cholesterol or alcohols. Although it has been shown in previous studies that FAME is required for Staphylococcus aureus survival in skin abscesses, FAME is poorly studied compared to other virulence factors. FAME activity had also been detected in coagulase-negative staphylococci (CNS. However, FAME activity was only surveyed after a bacterial culture was grown for 24 h. Therefore if FAME activity was earlier in the growth phase, it would not have been detected by the assay and those strains would have been labeled as FAME negative. Results Fifty CNS bovine mastitis isolates and several S. aureus, Escherichia coli, and Streptococcus uberis strains were assayed for FAME activity over 24 h. FAME activity was detected in 54% of CNS and 80% S. aureus strains surveyed but none in E. coli or S. uberis. While some CNS strains produced FAME activity comparable to the lab strain of S. aureus, the pattern of FAME activity varied among strains and across species of staphylococci. All CNS that produced FAME activity also exhibited lipase activity. Lipase activity relative to colony forming units of these CNS decreased over the 24 h growth period. No relationship was observed between somatic cell count in the milk and FAME activity in CNS. Conclusions Some staphylococcal species surveyed produced FAME activity, but E. coli and S. uberis strains did not. All FAME producing CNS exhibited lipase activity which may indicate that both these enzymes work in concert to alter fatty acids in the bacterial environment.

  19. Costello syndrome: An overview

    NARCIS (Netherlands)

    Hennekam, Raoul C. M.

    2003-01-01

    Costello syndrome is characterized by prenatally increased growth, postnatal growth retardation, coarse face, loose skin resembling cutis laxa, nonprogressive cardiomyopathy, developmental delay, and a outgoing, friendly behavior. Patients can develop papillomata, especially around the mouth, and

  20. Rothmund–Thomson syndrome: anaesthesia considerations

    African Journals Online (AJOL)

    cases of this syndrome have been reported in the scientific literature worldwide. This study ... photosensitivity, poikilodermatous skin changes, juvenile cataracts ... Infants and young children can have gastrointestinal disturbances including ...

  1. Abnormal sensitivity of diploid skin fibroblasts from a family with Gardner's syndrome to the lethal effects of X-irradiation, ultraviolet light and mitomycin-C

    International Nuclear Information System (INIS)

    Little, J.B.; Nove, J.; Weichselbaum, R.R.

    1980-01-01

    Skin fibroblasts isolated from two members of the same family with the cancer-prone disease Gardner's Syndrome (intestinal polyposis, colon cancer, bone and soft tissue tumors) showed enhanced sensitivity to the lethal effects of X-irradiation, ultraviolet light and mitomycin-C. These cells showed no liquid-holding type recovery following UV-irradiation of confluent cultures, but were normal in their capacity for UV-induced unscheduled DNA synthesis. UV survival was not influenced by post-irradiation incubation with caffeine. (orig.)

  2. The Marfan Syndrome: A Booklet for Teachers.

    Science.gov (United States)

    Bernhardt, Barbara A.

    This booklet explains characteristics of Marfan Syndrome, an inherited disorder of connective tissue which can be life-threatening if untreated. Medical problems affecting various parts of the body such as the heart, the skeleton, the eyes and the skin associated with Marfan Syndrome are discussed. Possible medical emergencies are identified.…

  3. Profilaxis, immunoprophylaxis and therapy of staphylococcal mastitis

    Directory of Open Access Journals (Sweden)

    Vakanjac Slobodanka

    2006-01-01

    Full Text Available Inflammation of the mammary gland, mastitis, in cows presents one of the most acute problems in intensive milk production, incurring huge economic losses which reach from 20-80% even in developed countries in the past decade. On the grounds of the programme of the respective Ministry, the Veterinary Service of the Republic of Serbia is obliged to monitor, detect, curb and control infective inflammation of the mammary gland caused by staphylococcus and streptococcus. Long-term different approaches to mastitis treatment did not result in the required solution, so that the problem of mastitis is still present and acute. In order to prevent the infiltration of the pathogenic cause into the mammary gland, its populating and multiplication, it is necessary to maintain constant and regular controls of milk, as well as undertake of preventive and therapeutic measures aimed at reducing the incidence of mastitis. Immunization and immunotherapy of mastitis are new and very interesting areas for scientific investigations and work. In the recent decades vaccines against staphylococcal mastitis are being successfully developed, whose success is reflected in the reduced incidence of clinical and subcilinical mastitis.

  4. [Mastitis revealing Churg-Strauss syndrome].

    Science.gov (United States)

    Dannepond, C; Le Fourn, E; de Muret, A; Ouldamer, L; Carmier, D; Machet, L

    2014-01-01

    Churg-Strauss syndrome often involves the skin, and this may sometimes reveal the disease. A 25-year-old woman was referred to a gynaecologist for inflammation of the right breast with breast discharge. Cytological analysis of the liquid showed numerous inflammatory cells, particularly polymorphonuclear eosinophils and neutrophils. Ultrasound examination of the breast was consistent with galactophoritis. CRP was normal, and hypereosinophilia was seen. The patient was subsequently referred to a dermatology unit. Skin examination revealed inflammation of the entire breast, which was painful, warm and erythematous; the border was oedematous with blisters. Necrotic lesions were also present on the thumbs and knees. Skin biopsy of the breast showed a dermal infiltrate with abundant infiltrate of polymorphonuclear eosinophils, including patchy necrosis and intraepidermal vesicles. Histological examination of a biopsy sample from a thumb revealed eosinophilic granuloma and leukocytoclastic vasculitis. The patient was also presenting asthma, pulmonary infiltrates and mononeuropathy at L3, consistent with Churg-Strauss syndrome. Breast involvement in Churg-Strauss syndrome is very rare (only one other case has been reported). This is the first case in which the breast condition revealed the disease. Cutaneous involvement of the breast is, however, also compatible with Wells' cellulitis. The lesions quickly disappeared with 1mg/kg/d oral prednisolone. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  5. Spontaneous Pneumothorax in Birt-Hogg-Dube' Syndrome: Two Case Reports

    Energy Technology Data Exchange (ETDEWEB)

    Bae, Hyoung Ju; Woo, Ok Hee; Yong, Hwan Seok; Kang, Eun Young; Kim, Hyun Koo; Choi, Young Ho; Shin, Bong Kyung; Kim, Yoon Kyung [Korea University School of Medicine, Korea University Guro Hospital, Seoul (Korea, Republic of)

    2011-01-15

    Birt-Hogg-Dube'(BHD) syndrome is a rare autosomal dominant inherited disorder that is characterized by skin fibrofolliculomas, renal tumors and multiple lung cysts with or without spontaneous pneumothorax. The disease is caused by germline mutations in the FLCN gene that codes for a protein of unknown function called folliculin. Patients with BHD syndrome do not always have all three manifestations of the skin, kidney and lung. To the best of our knowledge, there has been no case report of the radiologic findings of the lung manifestation in a patient with BHD syndrome in Korea. We report here on two cases of BHD syndrome that presented with spontaneous pneumothorax. The pulmonary abnormalities consisted of multiple thin-walled cysts of various sizes and shapes in both lungs

  6. Brooke-Spiegler syndrome

    Directory of Open Access Journals (Sweden)

    Layegh Pouran

    2008-01-01

    Full Text Available The Brooke-Spiegler syndrome (BSS is an uncommon autosomal dominant disorder characterized by a high affinity to form multiple adnexal neoplasia (skin appendage tumors, especially trichoepitheliomas and cylindromas, and occasionally spiradenomas, which usually appear in the second or third decade of life. To date, only a few cases with this syndrome have been reported. This case report describes a 26-year-old woman who presented to the dermatology department of Qaem Hospital with tumoral lesions on her scalp, face, and forearm. Her father and younger brother were also affected. On examination, several round-to-oval skin-colored papules with a smooth pearly surface measuring 2 to 6 mm in diameter were seen on the mid-face, particularly in the nasolabial folds, the upper lip. Tumors and nodules seen on the scalp were pinkish red, dome-shaped, and to some extent, pedunculated with surface telangiectasia and induration. Histopathology of the facial papules showed trichoepithelioma while that of a scalp nodule showed cylindroma.

  7. Staphylococcal superantigen-specific IgE antibodies: degree of sensitization and association with severity of asthma

    OpenAIRE

    Elabras Filho, José; Mello, Fernanda Carvalho de Queiroz; Lupi, Omar; Bica, Blanca Elena Rios Gomes; Papi, José Angelo de Souza; França, Alfeu Tavares

    2016-01-01

    ABSTRACT Objective: To determine the presence of staphylococcal superantigen-specific IgE antibodies and degree of IgE-mediated sensitization, as well as whether or not those are associated with the severity of asthma in adult patients. Methods: This was a cross-sectional study involving outpatients with asthma under treatment at a tertiary care university hospital in the city of Rio de Janeiro, Brazil. Consecutive patients were divided into two groups according to the severity of asthma ba...

  8. Skin bioengineering and stem cells for severe burn treatment

    International Nuclear Information System (INIS)

    Lataillade, J.J.; Trouillas, M.; Alexaline, M.; Brachet, M.; Bey, E.; Duhamel, P.; Leclerc, T.; Bargues, L.

    2015-01-01

    Severely burned patients need definitive and efficient wound coverage. The outcome of massive burns has improved with cultured epithelial auto-grafts (CEA). In spite of its fragility, percentage of success, cost of treatment and long-term tendency to contracture, this surgical technique has been developed in some burn centres. The first improvements involved combining CEA and dermis-like substitutes. Cultured skin substitutes provide faster skin closure and satisfying functional results. These methods have been used successfully in massive burns. A second improvement was to enable skin regeneration by using epidermal stem cells. Stem cells can differentiate into keratinocytes, to promote wound repair and to regenerate skin appendages. Human mesenchymal stem cells foster wound healing and were used in cutaneous radiation syndrome. Skin regeneration and tissue engineering methods remain a complex challenge and offer the possibility of new treatment for injured and burned patients. (authors)

  9. Skin tags associated with obesity and diabetes mellitus in patients with chronic kidney disease

    OpenAIRE

    Sari Funda; Inci Ayca; Dolu Suleyman; Sari Ramazan

    2017-01-01

    Introduction/Objective. Both chronic kidney disease and skin tags are associated with similar cardiovascular risk factors such as obesity, diabetes mellitus, dyslipidemia, hypertension, etc. The aim of this study was to determine the prevalence of skin tags in patients with chronic kidney disease, and to assess the relationship between skin tags and cardiovascular risk factors such as diabetes, hypertension, dyslipidemia, obesity, and metabolic syndrome. Methods. We evaluated 358 patients [14...

  10. Systematic review of a patient care bundle in reducing staphylococcal infections in cardiac and orthopaedic surgery.

    Science.gov (United States)

    Ma, Ning; Cameron, Alun; Tivey, David; Grae, Nikki; Roberts, Sally; Morris, Arthur

    2017-04-01

    Surgical site infections (SSIs) are serious adverse events hindering surgical patients' recovery. In Australia and New Zealand, SSIs are a huge burden to patients and healthcare systems. A bundled approach, including pre-theatre nasal and/or skin decolonization has been used to reduce the risk of staphylococcal infection. The aim of this review is to assess the effectiveness of the bundle in preventing SSIs for cardiac and orthopaedic surgeries. The review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Published literature was searched in PubMed, Embase and Cochrane Library of Systematic reviews. Identified articles were selected and extracted based on a priori defined Population-Intervention-Comparator-Outcome and eligibility criteria. Data of randomized controlled trials (RCTs) and comparative observational studies were synthesized by meta-analyses. Quality appraisal tools were used to assess the evidence quality. The review included six RCTs and 19 observational studies. The bundled treatment regimens varied substantially across all studies. RCTs showed a trend of Staphylococcus aureus SSIs reduction due to the bundle (relative risk = 0.59, 95% confidence interval (CI) = 0.33, 1.06) with moderate heterogeneity. Observational studies showed statistically significant reduction in all-cause and S. aureus SSIs, with 51% (95% CI = 0.41, 0.59) and 47% (95% CI = 0.35, 0.65), respectively. No publication biases were detected. SSIs in major cardiac and orthopaedic surgeries can be effectively reduced by approximately 50% with a pre-theatre patient care bundle approach. © 2017 Royal Australasian College of Surgeons.

  11. Netherton syndrome in one Chinese adult with a novel mutation in the SPINK5 gene and immunohistochemical studies of LEKTI

    Directory of Open Access Journals (Sweden)

    Zhang Xi-Bao

    2012-01-01

    Full Text Available Background : Netherton syndrome (NS is a severe autosomal recessive ichthyosis. It is characterized by congenital ichthyosiform erythroderma, trichorrhexis invaginata, ichthyosis linearis circumflexa, atopic diathesis, and frequent bacterial infections. The disease is caused by mutations in the SPINK5 (serine protease inhibitor Kazal-type 5 gene, a new type of serine protease inhibitor involved in the regulation of skin barrier formation and immunity. We report one Chinese adult with NS. The patient had typical manifestation of NS except for trichorrhexis invaginata with an atopic diathesis and recurrent staphylococcal infections since birth. Aims: To evaluate the gene mutation and of its product activity of SPINK5 gene in confirmation of the diagnosis of one Chinese adult with NS. Materials and Methods: To screen mutations in the SPINK5 gene, 33 exons and flanking intron boundaries of SPINK5 were amplified with polymerase chain reaction (PCR and used for direct sequencing. In addition, immunohistochemical staining of LEKTI (lymphoepithelial Kazal-type-related inhibitor with specific antibody was used to confirm the diagnosis of NS. The results were compared with that of healthy individuals (twenty-five blood samples. Results: A G318A mutation was found at exon 5 of patient′s SPINK5 gene which is a novel missense mutation. The PCR amplification products with mutation-specific primer were obtained only from the DNA of the patients and their mother, but not from their father and 25 healthy individuals. Immunohistochemical studies indicated there was no LEKTI expression in NS patient′s skin and there was a strong LEKTI expression in the normal human skin. Conclusion: In this report, we describe heterozygous mutation in the SPINK5 gene and expression of LEKTI in one Chinese with NS. The results indicate that defective expression of LEKTI in the epidermis and mutations of SPINK5 gene are reliable for diagnostic feature of NS with atypical

  12. [Two cases of Costello syndrome].

    Science.gov (United States)

    Masuyama, Tatsuo; Matsuo, Muneaki; Kuno, Tateo; Kitsuki, Kyoko; Kan, Yuka; Ishii, Kiyohisa; Ohtani, Yoshinobu

    2003-01-01

    We report two unrelated cases of Costello syndrome, presenting with poor postnatal growth, mild mental retardation, poor feeding, curly hair, coarse characteristic face, loose skin, hypotonia, and cardiac involvement. Nasal papilloma and acanthosis nigricans were the most characteristic features of this syndrome. Both cases had atrial fibrilation from infancy to early childhood. One patient had hypertonia in the lower extremities and pes equinovarus, while the other had hypotonia and pes planovalgus.

  13. A Homozygous Missense Mutation in TGM5 Abolishes Epidermal Transglutaminase 5 Activity and Causes Acral Peeling Skin Syndrome

    Science.gov (United States)

    Cassidy, Andrew J.; van Steensel, Maurice A. M.; Steijlen, Peter M.; van Geel, Michel; Velden, Jaap van der; Morley, Susan M.; Terrinoni, Alessandro; Melino, Gerry; Candi, Eleonora; McLean, W. H. Irwin

    2005-01-01

    Peeling skin syndrome is an autosomal recessive genodermatosis characterized by the shedding of the outer epidermis. In the acral form, the dorsa of the hands and feet are predominantly affected. Ultrastructural analysis has revealed tissue separation at the junction between the granular cells and the stratum corneum in the outer epidermis. Genomewide linkage analysis in a consanguineous Dutch kindred mapped the gene to 15q15.2 in the interval between markers D15S1040 and D15S1016. Two homozygous missense mutations, T109M and G113C, were found in TGM5, which encodes transglutaminase 5 (TG5), in all affected persons in two unrelated families. The mutation was present on the same haplotype in both kindreds, indicating a probable ancestral mutation. TG5 is strongly expressed in the epidermal granular cells, where it cross-links a variety of structural proteins in the terminal differentiation of the epidermis to form the cornified cell envelope. An established, in vitro, biochemical cross-linking assay revealed that, although T109M is not pathogenic, G113C completely abolishes TG5 activity. Three-dimensional modeling of TG5 showed that G113C lies close to the catalytic domain, and, furthermore, that this glycine residue is conserved in all known transglutaminases, which is consistent with pathogenicity. Other families with more-widespread peeling skin phenotypes lacked TGM5 mutations. This study identifies the first causative gene in this heterogeneous group of skin disorders and demonstrates that the protein cross-linking function performed by TG5 is vital for maintaining cell-cell adhesion between the outermost layers of the epidermis. PMID:16380904

  14. Management of extracolonic tumours in patients with Lynch syndrome

    NARCIS (Netherlands)

    Koornstra, Jan J; Mourits, Marian Je; Sijmons, Rolf H; Leliveld-Kors, Anna; Hollema, Harry; Kleibeuker, Jan H

    Hereditary nonpolyposis colorectal cancer, or Lynch syndrome, is responsible for 2-3% of all colorectal cancers. Lynch syndrome is also associated with a high risk of extracolonic cancers, including endometrial, stomach, small bowel, pancreas, biliary tract, ovary, urinary tract, brain, and skin

  15. Degos' syndrome. Detection of intestinal lesion by digital subtraction angiography

    International Nuclear Information System (INIS)

    Bilbao, J.I.; Garcia Delgado, F.; Idoate, M.; Arejola, J.M.; Aquerreta, D.; Otero, M.

    1986-01-01

    Degos' syndrom consists in a generalized vasculitis with frequent affectation of the gut and the skin. The arteriographic findings in a patient with the diagnosis of Degos' syndroms and severe affectation of the gut are reported [fr

  16. Churg Strauss syndrome; Sindrome de Churg Strauss

    Energy Technology Data Exchange (ETDEWEB)

    Lopez Rengifo, Diana Milena; Contreras Zuniga, Eduardo; Osio, Luis Fernando

    2007-07-01

    The Churg-Strauss syndrome, also called allergic granulomatosis and angiitis, is a multisystem disorder characterized by allergic rhinitis, asthma, and prominent peripheral blood eosinophilia. The most common organ involved is the lung, followed by the skin. The Churg-Strauss syndrome, however, can affect any organ system, including the cardiovascular, gastrointestinal, renal, and central nervous systems.

  17. Crest syndrome

    International Nuclear Information System (INIS)

    Koch, B.; Roedl, W.

    1988-01-01

    If a patient has peri- and intra-articular calcinosis, as well as acro-osteolysis and esophageal hypomotility, and rheumatic symptoms, Crest syndrome should be considered as a manifestation of progressive systemic sclerosis. In connection with relevant symptoms on the skin and visceral involvement, radiological studies offer the possibility of classifying progressive systemic sclerosis more accurately. (orig.) [de

  18. Diagnosing Lynch Syndrome

    LENUS (Irish Health Repository)

    Gleeson, J

    2016-11-01

    Lynch Syndrome, also known as Hereditary Non-Polyposis Colorectal Cancer (HNPCC), is a hereditary condition that increases an individual’s risk of developing a constellation of cancers. These most commonly arise in the colon, but also involve other solid organs such as the endometrium and ovaries in women, the stomach, brain and the skin. Ireland’s small population offers an opportunity to identify all those with Lynch Syndrome (LS) in the country, which would represent a powerful preventive opportunity to meaningfully impact on the incidence of cancer in Ireland.

  19. Acquired Ulcero-Mutilating Bilateral Acro-Osteopathy (Bureau-Barrière Syndrome

    Directory of Open Access Journals (Sweden)

    Georgi Tchernev

    2017-07-01

    Full Text Available We present a 35-year-old male patient with Bureau-Barrière syndrome. Bureau-Barrière syndrome is an ulcero-mutilating acropathy almost invariably associated with excessive alcohol intake. It presents with a triad of trophic skin changes with recurrent ulcerations, bone lesions and nerve damage. The clinical presentation includes chronic painless plantar ulcerations with periulcerous hyperkeratosis, hyperhidrosis, livedoid skin colour, nail dystrophy, widening and infiltration of the toes and common interdigital mycoses. Other non-specific skin changes related to the alcohol consumption are commonly observed as well. The condition affects mainly middle-aged men suffering from alcoholism. Often a bilateral location at the lower limb of male alcoholics has been described, as in our patient. Successful treatment of the Bureau-Barrière syndrome requires an interdisciplinary approach. Cessation of alcohol intake and smoking is of paramount importance.

  20. Oculocerebrocutaneous syndrome : report of three additional cases and aetiological considerations

    NARCIS (Netherlands)

    Moog, U; deDieSmulders, C; Systermans, JMJ; Cobben, JM

    1997-01-01

    The oculocerebrocutaneous (OCC) syndrome is characterized by the presence of orbital cysts and microphthalmia/anophthalmia, focal hypo- and aplastic skin defects, skin appendages and cerebral malformations. Most of the patients suffer from psychomotor retardation and seizures. To date, 23 patients

  1. A rare cause of acromegaly: McCune-Albright syndrome

    Directory of Open Access Journals (Sweden)

    Erdal Bodakçi

    2015-06-01

    Full Text Available McCune-Albright syndrome is characterized by polyostatic fibrous dysplasia, brown spots on the skin (café au lait pigmentation and autonomous endocrine hyperfunction. Early puberty and other endocrinological manifestations, such as acromegaly, gigantism and hypercortisolism are widely observed in the syndrome. Acromegaly is seen in 20% of patients. We report a case of acromegaly accompanied with this syndrome.

  2. Association between Michelin tire baby syndrome and congenital panhyopituitarism in an Iranian girl.

    Science.gov (United States)

    Haghshenas, Zahra; Tajziehchi, Leila; Ghavami, Fakhredin

    2014-08-01

    Michelin tire baby syndrome is a rare syndrome, diagnosed clinically by multiple circumferential skin folds. Multiple noncutaneous anomalies have been described with this syndrome. We report a case of Michelin tire baby syndrome with congenital panhypopituitarism. To date, there is no report of association between these two disorders.

  3. Subconjunctival epidermoid cysts in Gorlin-Goltz syndrome.

    Science.gov (United States)

    De Craene, S; Batteauw, A; Van Lint, M; Claerhout, I; Decock, C

    2014-08-01

    Epidermoid cysts are common benign cysts which occur particularly on the skin of the face, neck and upper trunk. Subconjunctival location of these cysts is very rare and, until today, only seen in patients with Gorlin-Goltz syndrome. Histopathological examination of these cysts show similarities with odontogenic keratocysts, a typical clinical manifestation of Gorlin-Goltz syndrome.

  4. Synovis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome: A case of spine, pelvis, and anterior chest wall involvement, with overlooked plantar pustulosis

    International Nuclear Information System (INIS)

    Kim, Hyun Soo; Jeong, Soh Yong; Lee, Sujin; Baek, In Woon; Park, Jeongmi

    2017-01-01

    Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is an inflammatory clinical condition with aseptic bone lesions and characteristic skin manifestations. A 63-year-old woman presented with vague musculoskeletal symptoms including chronic buttock pain. The clinical work-up revealed multiple spine and osteoarticular involvement. Multilevel bone marrow edema and cortical erosions involving the spine, asymmetric sacroiliitis, and osteosclerosis of the sternoclavicular joint were consistent with a diagnosis of SAPHO syndrome. Considering SAPHO syndrome in the differential diagnosis, subsequent skin inspection revealed plantar pustulosis. Despite the unique feature of accompanying skin and skeletal lesions, skin lesions could be overlooked if not suspected

  5. Synovis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome: A case of spine, pelvis, and anterior chest wall involvement, with overlooked plantar pustulosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyun Soo; Jeong, Soh Yong; Lee, Sujin; Baek, In Woon; Park, Jeongmi [Yeouido St. Mary' s Hospital, College of Medicine, The Catholic University of Korea, Seoul (Korea, Republic of)

    2017-05-15

    Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is an inflammatory clinical condition with aseptic bone lesions and characteristic skin manifestations. A 63-year-old woman presented with vague musculoskeletal symptoms including chronic buttock pain. The clinical work-up revealed multiple spine and osteoarticular involvement. Multilevel bone marrow edema and cortical erosions involving the spine, asymmetric sacroiliitis, and osteosclerosis of the sternoclavicular joint were consistent with a diagnosis of SAPHO syndrome. Considering SAPHO syndrome in the differential diagnosis, subsequent skin inspection revealed plantar pustulosis. Despite the unique feature of accompanying skin and skeletal lesions, skin lesions could be overlooked if not suspected.

  6. Evaluation of lipid oxidation and microbial loads of suya as affected ...

    African Journals Online (AJOL)

    Meat samples used for this study were excised from leg cuts of 9 Red Sokoto bucks. They were starved for 16hours before slaughter and their carcasses randomly assigned to three post-mortem processing methods – scalding, skinning and singeing. The carcasses were opened up, eviscerated, washed and fabricated.

  7. Kallmann syndrome and ichthyosis: a case of contiguous gene deletion syndrome

    Directory of Open Access Journals (Sweden)

    Irene Berges-Raso

    2017-09-01

    Full Text Available Kallmann syndrome is a genetically heterogeneous form of hypogonadotropic hypogonadism caused by gonadotropin-releasing hormone deficiency and characterized by anosmia or hyposmia due to hypoplasia of the olfactory bulbs; osteoporosis and metabolic syndrome can develop due to longstanding untreated hypogonadism. Kallmann syndrome affects 1 in 10 000 men and 1 in 50 000 women. Defects in 17 genes, including KAL1, have been implicated. Kallmann syndrome can be associated with X-linked ichthyosis, a skin disorder characterized by early onset dark, dry, irregular scales affecting the limb and trunk, caused by a defect of the steroid sulfatase gene (STS. Both KAL1 and STS are located in the Xp22.3 region; therefore, deletions in this region cause a contiguous gene syndrome. We report the case of a 32-year-old man with ichthyosis referred for evaluation of excessive height (2.07 m and weight (BMI: 29.6 kg/m2, microgenitalia and absence of secondary sex characteristics. We diagnosed Kallmann syndrome with ichthyosis due to a deletion in Xp22.3, a rare phenomenon.

  8. The POEMS syndrome: Report of three cases with radiographic abnormalities

    International Nuclear Information System (INIS)

    Tanaka, O.; Ohsawa, T.

    1984-01-01

    Three cases of a unique multisystemic syndrome with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (the POEMS syndrome) are presented, along with a review of the literature. Clinical and radiographic features of this syndrome and etiological considerations are discussed. A variety of osteosclerotic lesions, nonspecific pleural effusion and ascites are characteristic radiographic manifestations. (orig.) [de

  9. Sweet syndrome revealing systemic lupus erythematosus.

    LENUS (Irish Health Repository)

    Quinn, N

    2015-02-01

    Sweet Syndrome is an acute inflammatory skin eruption which is rare in children. We report a case of childhood Systemic Lupus Erythematosus (SLE) that presented with Sweet syndrome. This case is a unique presentation of a common disorder which provides a new facet for the differential diagnosis of SLE in children. It is also the first paediatric case to be reported in a Caucasian child.

  10. Living with Ehlers-Danlos syndrome

    OpenAIRE

    Berglund, Britta

    2003-01-01

    This thesis focuses on how individuals with the Ehlers-Danlos syndrome (EDS), an inherited connective-tissue disorder, experience and describe their daily lives with focus on physical and psychosocial problems. The syndrome primarily affects the skin, ligaments, joints and blood vessels and the symptoms in EDS vary from mild to more severe forms. Individuals with EDS were recruited via the Swedish National EDS Association. Paper (I). The aim was to explore how individuals wi...

  11. Xeroderma Pigmentosum with Severe Neurological Manifestations/De Sanctis–Cacchione Syndrome and a Novel XPC Mutation

    Directory of Open Access Journals (Sweden)

    Esteban Uribe-Bojanini

    2017-01-01

    Full Text Available Several genetic disorders caused by defective nucleotide excision repair that affect the skin and the nervous system have been described, including Xeroderma Pigmentosum (XP, De Sanctis–Cacchione syndrome (DSC, Cockayne syndrome, and Trichothiodystrophy. Cutaneous photosensitivity with an increased risk of skin malignancy is a common feature of these disorders, but clinical manifestations commonly overlap these syndromes. Several genes have been found to be altered in these pathologies, but we lack more genotype-phenotype correlations in order to make an accurate diagnosis. Very few cases of DSC syndrome have been reported in the literature. We present a case of a 12-year-old Colombian male, with multiple skin lesions in sun-exposed areas from the age of 3 months and a history of 15 skin cancers. He also displayed severe neurologic abnormalities (intellectual disability, ataxia, altered speech, and hyperreflexia, short stature, and microcephaly, which are features associated with DSC. Genetic testing revealed a novel germline mutation in the XP-C gene (c.547A>T. This is the first case of an XP-C mutation causing De Sanctis–Cacchione syndrome. Multigene panel testing is becoming more widely available and accessible in the clinical setting and will help rapidly unveil the molecular etiology of these rare genetic disorders.

  12. Xeroderma Pigmentosum with Severe Neurological Manifestations/De Sanctis–Cacchione Syndrome and a Novel XPC Mutation

    Science.gov (United States)

    Hernandez-Quiceno, Sara

    2017-01-01

    Several genetic disorders caused by defective nucleotide excision repair that affect the skin and the nervous system have been described, including Xeroderma Pigmentosum (XP), De Sanctis–Cacchione syndrome (DSC), Cockayne syndrome, and Trichothiodystrophy. Cutaneous photosensitivity with an increased risk of skin malignancy is a common feature of these disorders, but clinical manifestations commonly overlap these syndromes. Several genes have been found to be altered in these pathologies, but we lack more genotype-phenotype correlations in order to make an accurate diagnosis. Very few cases of DSC syndrome have been reported in the literature. We present a case of a 12-year-old Colombian male, with multiple skin lesions in sun-exposed areas from the age of 3 months and a history of 15 skin cancers. He also displayed severe neurologic abnormalities (intellectual disability, ataxia, altered speech, and hyperreflexia), short stature, and microcephaly, which are features associated with DSC. Genetic testing revealed a novel germline mutation in the XP-C gene (c.547A>T). This is the first case of an XP-C mutation causing De Sanctis–Cacchione syndrome. Multigene panel testing is becoming more widely available and accessible in the clinical setting and will help rapidly unveil the molecular etiology of these rare genetic disorders. PMID:28255305

  13. Acute leukemia after radiotherapy in a patient with Turcot's syndrome. Impaired colony formation in skin fibroblast cultures after irradiation

    International Nuclear Information System (INIS)

    Li, F.P.; Little, J.B.; Bech-Hansen, N.T.; Paterson, M.C.; Arlett, C.; Garnick, M.B.; Mayer, R.J.

    1983-01-01

    Colonic polyposis and carcinoma developed in a woman with Turcot's syndrome at the age of 31 years; astrocytoma developed when she was 37. Her brother and sister had died of astrocytoma at the ages of 18 and 33 years, respectively. Progressive neutropenia developed in the patient three months after radiotherapy for her brain tumor and acute myelomonocytic leukemia 19 months after treatment. Three laboratories independently evaluated cultures of her skin fibroblasts for in vitro sensitivity to cell killing (loss of colony-forming ability) by x-rays. Survival assays consistently revealed slight but significant radiosensitivity in an early-passage (six to 10 doublings) fibroblast subculture. A later subculture (21 to 29 doublings) showed no abnormality, a possible effect of selective in vitro loss of radiosensitive cells

  14. Review Over a 3-Year Period of European Union Proficiency Tests for Detection of Staphylococcal Enterotoxins in Food Matrices.

    Science.gov (United States)

    Nia, Yacine; Mutel, Isabelle; Assere, Adrien; Lombard, Bertrand; Auvray, Frederic; Hennekinne, Jacques-Antoine

    2016-04-13

    Staphylococcal food poisoning outbreaks are a major cause of foodborne illnesses in Europe and their notifications have been mandatory since 2005. Even though the European regulation on microbiological criteria for food defines a criterion on staphylococcal enterotoxin (SE) only in cheese and dairy products, European Food Safety Authority (EFSA) data reported that various types of food matrices are involved in staphylococcal food poisoning outbreaks. The European Screening Method (ESM) of European Union Reference Laboratory for Coagulase Positive Staphylococci (EURL CPS) was validated in 2011 for SE detection in food matrices and is currently the official method used for screening purposes in Europe. In this context, EURLCPS is annually organizing Inter-Laboratory Proficiency Testing Trials (ILPT) to evaluate the competency of the European countries' National Reference Laboratories (NRLs) to analyse SE content in food matrices. A total of 31 NRLs representing 93% of European countries participated in these ILPTs. Eight food matrices were used for ILPT over the period 2013-2015, including cheese, freeze-dried cheese, tuna, mackerel, roasted chicken, ready-to-eat food, milk, and pastry. Food samples were spiked with four SE types (i.e., SEA, SEC, SED, and SEE) at various concentrations. Homogeneity and stability studies showed that ILPT samples were both homogeneous and stable. The analysis of results obtained by participants for a total of 155 blank and 620 contaminated samples allowed for evaluation of trueness (>98%) and specificity (100%) of ESM. Further to the validation study of ESM carried out in 2011, these three ILPTs allowed for the assessment of the proficiency of the NRL network and the performance of ESM on a large variety of food matrices and samples. The ILPT design presented here will be helpful for the organization of ILPT on SE detection by NRLs or other expert laboratories.

  15. Review Over a 3-Year Period of European Union Proficiency Tests for Detection of Staphylococcal Enterotoxins in Food Matrices

    Directory of Open Access Journals (Sweden)

    Yacine Nia

    2016-04-01

    Full Text Available Staphylococcal food poisoning outbreaks are a major cause of foodborne illnesses in Europe and their notifications have been mandatory since 2005. Even though the European regulation on microbiological criteria for food defines a criterion on staphylococcal enterotoxin (SE only in cheese and dairy products, European Food Safety Authority (EFSA data reported that various types of food matrices are involved in staphylococcal food poisoning outbreaks. The European Screening Method (ESM of European Union Reference Laboratory for Coagulase Positive Staphylococci (EURL CPS was validated in 2011 for SE detection in food matrices and is currently the official method used for screening purposes in Europe. In this context, EURLCPS is annually organizing Inter-Laboratory Proficiency Testing Trials (ILPT to evaluate the competency of the European countries’ National Reference Laboratories (NRLs to analyse SE content in food matrices. A total of 31 NRLs representing 93% of European countries participated in these ILPTs. Eight food matrices were used for ILPT over the period 2013–2015, including cheese, freeze-dried cheese, tuna, mackerel, roasted chicken, ready-to-eat food, milk, and pastry. Food samples were spiked with four SE types (i.e., SEA, SEC, SED, and SEE at various concentrations. Homogeneity and stability studies showed that ILPT samples were both homogeneous and stable. The analysis of results obtained by participants for a total of 155 blank and 620 contaminated samples allowed for evaluation of trueness (>98% and specificity (100% of ESM. Further to the validation study of ESM carried out in 2011, these three ILPTs allowed for the assessment of the proficiency of the NRL network and the performance of ESM on a large variety of food matrices and samples. The ILPT design presented here will be helpful for the organization of ILPT on SE detection by NRLs or other expert laboratories.

  16. Persistent production of TH2-type cytokines and polyclonal B cell activation after chronic administration of staphylococcal enterotoxin B in mice

    NARCIS (Netherlands)

    Florquin, S.; Amraoui, Z.; Goldman, M.

    1996-01-01

    In order to study the immunopathological consequences of repeated exposure to bacterial superantigens, we evaluated the production of cytokines, the profile of serum immunoglobulins and the tissue damage in BALB/c mice injected twice a week for 3 weeks with 50 micrograms of staphylococcal

  17. A case study on successful ayurvedic management of a rare case of reiter's syndrome

    Directory of Open Access Journals (Sweden)

    Vaishali Kuchewar

    2017-01-01

    Full Text Available Reiter's syndrome is a clinical syndrome of arthritis, urethritis, conjunctivitis, and mucocutaneous lesions. Skin and mucosal involvement is observed in about 10% of the cases. A 34-year-old male was brought in severe condition. He had red colored foul smelling maculopapular skin lesions all over body, swollen and painful knee and shoulder joints. History revealed that he was suffering from Reiter's syndrome since 2003. He used to get admitted between the months of February and May every year due to aggravation of symptoms. Every time he was treated with systemic antibiotics, corticosteroids, immunosuppressants and non-steroidal anti-inflammatory drugs. He was successfully treated with external application and internal medication with tikta (bitter and kaṣāya (astringent drugs. He got significant relief in skin lesions as well as joint pain and his quality of life was markedly improved. This case study demonstrates that Reiter's syndrome can be well managed with Ayurvedic medicines.

  18. KERATODERMA BLENORRHAGICUM IN A PATIENT WITH REITER SYNDROME

    Directory of Open Access Journals (Sweden)

    Valentina Dimitrova

    2008-10-01

    Full Text Available Reiter syndrome is a systemic disorder, originally defined as a triad of arthritis, urethritis and conjunctivitis. This symptoms complex usually follows an episode of either urethritis or dysentery. Skin and mucosal involvement is observed in about 10% of the cases. We present a case of Reiter’s syndrome in a 55-year-old man who developed the typical skin lesions - kerathoderma blenorrhagicum. The disease started with a severe asymmetric oligoarthritis a month after the patient had urethritis. Two weeks after the onset of the arthritis red patches on the palms and plants appeared, which transformed quickly in harder and elevated plaques.

  19. Nablus mask-like facial syndrome

    DEFF Research Database (Denmark)

    Allanson, Judith; Smith, Amanda; Hare, Heather

    2012-01-01

    Nablus mask-like facial syndrome (NMLFS) has many distinctive phenotypic features, particularly tight glistening skin with reduced facial expression, blepharophimosis, telecanthus, bulky nasal tip, abnormal external ear architecture, upswept frontal hairline, and sparse eyebrows. Over the last few...

  20. Facial skin follllicular hyperkeratosis of patients with basal cell carcinoma

    Directory of Open Access Journals (Sweden)

    M. V. Zhuchkov

    2016-01-01

    Full Text Available This article provides a clinical observation of paraneoplastic syndrome of a patient with basal cell carcinoma of skin. Authors present clinical features of the described for the first time, paraneoplastic retentional follicular hyperkeratosis of facial area.

  1. A Boy with Relentless Pruritus: Job's Syndrome.

    Science.gov (United States)

    Khan, Kamran; Wozniak, Susan E; Giannone, Anna Lucia; Abdulmassih, Maria Elena

    2016-02-21

    Job's syndrome (hyper IgE syndrome) is a very rare primary immunodeficiency disease that has an annual approximate incidence of less than 1/1,000,000. This manuscript aims to provide education regarding diagnosis and management strategies of this syndrome worldwide. A 6-year-old boy was seen at the clinic secondary to persistent pruritus interfering with sleep. At the age of 2 months, the patient developed diffuse eczematous and desquamating skin lesions. He was subsequently diagnosed with atopic dermatitis and managed conservatively. From 2 months to 7 years of age, intermittent exacerbations of dermatitis persisted despite an aggressive treatment regimen. The serum IgE level increased exponentially over a period of 7 years, with a peak value of 57,400 IU/ml. Molecular genetic testing revealed a dominant negative mutation within the SH2 domain of the Signal Transducer and Activator of Transcription (STAT3) gene. The patient was subsequently diagnosed with Job's syndrome. Management included proper skin care, prophylactic antibiotics, immunomodulating agents, and psychotherapy. Job's syndrome can often go unrecognized and masquerade as atopic dermatitis. Therefore, genetic testing for this condition should be obtained in all patients with treatment-refractory AD. Additionally, psychotherapy can be a successful management strategy for the grating psychological impact that can be imposed on children with excessive pruritus.

  2. [POEMS syndrome: role and value of interleukin-6].

    Science.gov (United States)

    Andrès, E; Courouau, F; Kaltenbach, G; Maloisel, F; Imler, M

    1996-01-01

    POEMS syndrome is a systemic disorder with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. The association of POEMS syndrome with lympho-proliferative disorder is very commun. The pathogenesis remains poorly understood but implication of cytokines (interleukins 1 and 6) is suspected. We report a case of a classic POEMS syndrome (with polyneuropathy, hepatomegaly, diabetes melitus, hyperpigmentation, monoclonal IgG lambda, anasarca and solitary plasmocytoma), associated with high serum levels of interleukin 6.

  3. Correlation of skin changes with hormonal changes in polycystic ovarian syndrome: A cross-sectional study clinical study

    Directory of Open Access Journals (Sweden)

    B Vijaya Gowri

    2015-01-01

    Full Text Available Background: Polycystic ovarian syndrome (PCOS is a heterogenous collection of signs and symptoms that when gathered, form a spectrum of disorder with disturbance of reproductive, endocrine and metabolic functions. Aim: The aim of this study is to correlate the skin manifestations with hormonal changes and to know the incidence and prevalence of skin manifestations in patients with PCOS. Materials and Methods: A total of 40 patients with PCOS were examined during 1 year time period from May 2008 P to May 2009. Detailed clinical history was taken from each patient. PCOS was diagnosed on the basis of ultrasonography. Hormonal assays included fasting blood sugar, postprandial blood sugar, follicle-stimulating hormone, luteinizing hormone, thyroid stimulating hormone, dehydroepiandrostenedione, prolactin, free testosterone, fasting lipid profile and sex hormone binding globulin. The results obtained were statistically correlated. Results: In our study, the prevalence of cutaneous manifestations was 90%. Of all the cutaneous manifestations acne was seen in highest percentage (67.5%, followed by hirsutism (62.5%, seborrhea (52.5%, androgenetic alopecia (AGA (30%, acanthosis nigricans (22.5% and acrochordons (10%. Fasting insulin levels was the most common hormonal abnormality seen in both acne and hirsutism, whereas AGA was associated with high testosterone levels. Conclusion: The prevalence of cutaneous manifestations in PCOS was 90%. Hirsutism, acne, seborrhea, acanthosis nigricans and acrochordons were associated with increased levels of fasting insulin, whereas AGA showed higher levels of serum testosterone.

  4. Reiter′s Syndrome

    Directory of Open Access Journals (Sweden)

    S S Savant

    1979-01-01

    Full Text Available A case of Reiter′s syndrome occurring in a young mate aged 20 years having extensive skin lesions of keratoderina blenoffhagica is presented along with a review of literature. Although urethritis was absent, other clinical and histopathological features of the cutaneous lesions led us to the diagnosis. The-possible relationship of postural psoriasis to Reiter′s syndrome is discussed. Failure of the patient to respond satisfactorily to steroids, antibiotics etc, prompted the use of rnethotrexate in the case. The result was dramatic, as the patient completely recovered within ten days of starting treatment.

  5. Apert′s Syndrome

    Directory of Open Access Journals (Sweden)

    B K Sohi

    1980-01-01

    Full Text Available A case of Apert′s syndrome in a one year old female child is described and literature reviewed. She was the first born of a young couple. She had congenital syndactyl of toes and fingers, acro-cephalic skull, flat facies, exophthalmos, hypertelorism and greasy skin. In addition to the typical radiological features of this syndrome which the patient showed, thickened first metacarpals forked at the base were also seen. There were two phalanges for each toe. Calcification was seen intracranially. These radiological features have not been mentioned so far in the literature reviewed.

  6. Risk factors for methicillin-resistant Staphylococcal aureus skin and soft tissue infections presenting in primary care: a South Texas Ambulatory Research Network (STARNet) study.

    Science.gov (United States)

    Parchman, Michael L; Munoz, Abel

    2009-01-01

    To examine skin and soft tissue infections presenting at 4 primary care clinics and assess if historical risk factors and examination findings were associated with a positive methicillin-resistant Staphylococcus aureus (MRSA) culture. During the 10-month observational study (April 2007 through January 2008), physicians in 5 practices across South Texas collected history, physical examination findings, culture results, and antibiotic(s) prescribed for all patients presenting with a skin or soft tissue infection. Analyses were conducted to determine the relationship between historical indicators, location of lesions, and examination findings with a positive MRSA culture. Across 4 practices, 164 cases of skin and soft tissue infections were collected during 10 months. Of the 94 with a culture, 63 (67%) were MRSA positive. Patients working in or exposed to a health care setting were more likely to have a culture positive for MRSA, as were those presenting with an abscess. MRSA-positive lesions were also significantly smaller in size. Because of the high prevalence of MRSA skin and soft tissue infections among patients presenting to family physicians, presumptive treatment for MRSA may be indicated. However, increasing levels of resistance to current antibiotics is concerning and warrants development of alternative management strategies.

  7. Filaggrin 2 deficiency results in abnormal cell-cell adhesion in the cornified cell layers and causes peeling skin syndrome type A.

    Science.gov (United States)

    Mohamad, Janan; Sarig, Ofer; Godsel, Lisa M; Peled, Alon; Malchin, Natalia; Bochner, Ron; Vodo, Dan; Rabinowitz, Tom; Pavlovsky, Mor; Taiber, Shahar; Fried, Maya; Eskin-Schwartz, Marina; Assi, Siwar; Shomron, Noam; Uitto, Jouni; Koetsier, Jennifer L; Bergman, Reuven; Green, Kathleen J; Sprecher, Eli

    2018-05-11

    Peeling skin syndromes form a large and heterogeneous group of inherited disorders characterized by superficial detachment of the epidermal cornified cell layers, often associated with inflammatory features. Here we report on a consanguineous family featuring non-inflammatory peeling of the skin exacerbated by exposure to heat and mechanical stress. Whole exome sequencing revealed a homozygous nonsense mutation in FLG2, encoding filaggrin 2, which co-segregated with the disease phenotype in the family. The mutation was found to result in decreased FLG2 RNA levels as well almost total absence of filaggrin 2 in the patient epidermis. Filaggrin 2 was found to be expressed throughout the cornified cell layers and to co-localize with corneodesmosin which plays a crucial role in maintaining cell-cell adhesion in this region of the epidermis. Absence of filaggrin 2 in the patient skin was associated with markedly decreased corneodesmosin expression, which may contribute to the peeling phenotype displayed by the patients. Accordingly, using the dispase dissociation assay, we showed that FLG2 down-regulation interferes with keratinocyte cell-cell adhesion. Of particular interest, this effect was aggravated by temperature elevation, consistent with the clinical phenotype. Restoration of CDSN levels by ectopic expression rescued cell-cell adhesion.Taken together, the present data suggest that filaggrin 2 is essential for normal cell-cell adhesion in the cornified cell layers. Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.

  8. The glucagonoma syndrome and necrolytic migratory erythema : A clinical review

    NARCIS (Netherlands)

    van Beek, André P.; de Haas, Ellen R.M.; van Vloten, Willem A.; Lips, Cees J.M.; Roijers, Janine F.M.; Canninga-van Dijk, Marijke R.

    2004-01-01

    The glucagonoma syndrome is a rare disease in which a typical skin disorder, necrolytic migratory erythema, is often one of the first presenting symptoms. Weight loss and diabetes mellitus are two other prevalent characteristics of this syndrome. Necrolytic migratory erythema belongs to the recently

  9. Update on antiphospholipid antibody syndrome.

    Science.gov (United States)

    Lopes, Michelle Remião Ugolini; Danowski, Adriana; Funke, Andreas; Rêgo, Jozelia; Levy, Roger; Andrade, Danieli Castro Oliveira de

    2017-11-01

    Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related to thrombotic events, which may affect small, medium or large vessels. Other clinical features like thrombocytopenia, nephropathy, cardiac valve disease, cognitive dysfunction and skin ulcers (called non-criteria manifestations) add significant morbidity to this syndrome and represent clinical situations that are challenging. APS was initially described in patients with systemic lupus erythematosus (SLE) but it can occur in patients without any other autoimmune disease. Despite the autoimmune nature of this syndrome, APS treatment is still based on anticoagulation and antiplatelet therapy.

  10. Immune evasion by a staphylococcal inhibitor of myeloperoxidase

    Science.gov (United States)

    de Jong, Nienke W. M.; Ramyar, Kasra X.; Guerra, Fermin E.; Fevre, Cindy; Voyich, Jovanka M.; McCarthy, Alex J.; Garcia, Brandon L.; van Kessel, Kok P. M.; van Strijp, Jos A. G.; Geisbrecht, Brian V.; Haas, Pieter-Jan A.

    2017-01-01

    Staphylococcus aureus is highly adapted to its host and has evolved many strategies to resist opsonization and phagocytosis. Even after uptake by neutrophils, S. aureus shows resistance to killing, which suggests the presence of phagosomal immune evasion molecules. With the aid of secretome phage display, we identified a highly conserved protein that specifically binds and inhibits human myeloperoxidase (MPO), a major player in the oxidative defense of neutrophils. We have named this protein “staphylococcal peroxidase inhibitor” (SPIN). To gain insight into inhibition of MPO by SPIN, we solved the cocrystal structure of SPIN bound to a recombinant form of human MPO at 2.4-Å resolution. This structure reveals that SPIN acts as a molecular plug that prevents H2O2 substrate access to the MPO active site. In subsequent experiments, we observed that SPIN expression increases inside the neutrophil phagosome, where MPO is located, compared with outside the neutrophil. Moreover, bacteria with a deleted gene encoding SPIN showed decreased survival compared with WT bacteria after phagocytosis by neutrophils. Taken together, our results demonstrate that S. aureus secretes a unique proteinaceous MPO inhibitor to enhance survival by interfering with MPO-mediated killing. PMID:28808028

  11. Immune evasion by a staphylococcal inhibitor of myeloperoxidase

    Energy Technology Data Exchange (ETDEWEB)

    de Jong, Nienke W. M.; Ramyar, Kasra X.; Guerra, Fermin E.; Nijland, Reindert; Fevre, Cindy; Voyich, Jovanka M.; McCarthy, Alex J.; Garcia, Brandon L.; van Kessel, Kok P. M.; van Strijp, Jos A. G.; Geisbrecht, Brian V.; Haas, Pieter-Jan A.

    2017-08-14

    Staphylococcus aureus is highly adapted to its host and has evolved many strategies to resist opsonization and phagocytosis. Even after uptake by neutrophils, S. aureus shows resistance to killing, which suggests the presence of phagosomal immune evasion molecules. With the aid of secretome phage display, we identified a highly conserved protein that specifically binds and inhibits human myeloperoxidase (MPO), a major player in the oxidative defense of neutrophils. We have named this protein “staphylococcal peroxidase inhibitor” (SPIN). To gain insight into inhibition of MPO by SPIN, we solved the cocrystal structure of SPIN bound to a recombinant form of human MPO at 2.4-Å resolution. This structure reveals that SPIN acts as a molecular plug that prevents H2O2 substrate access to the MPO active site. In subsequent experiments, we observed that SPIN expression increases inside the neutrophil phagosome, where MPO is located, compared with outside the neutrophil. Moreover, bacteria with a deleted gene encoding SPIN showed decreased survival compared with WT bacteria after phagocytosis by neutrophils. Taken together, our results demonstrate that S. aureus secretes a unique proteinaceous MPO inhibitor to enhance survival by interfering with MPO-mediated killing.

  12. Pathologies of the skin and its appendages in endocrine diseases

    Directory of Open Access Journals (Sweden)

    Hubert Arasiewicz

    2016-05-01

    Full Text Available Patients suffering from endocrine disorders often present a wide profile of skin lesions. In hyperthyroidism we observe hair loss, lower leg myxedema and onycholysis or, in the case of hormone deficiency, generalized swelling of the skin, which becomes cold and pale. Primary hyperparathyroidism is revealed by pruritus, presence of chronic urticaria or deposition of amorphous calcium salts. In hypoparathyroidism, the skin is dry while the nails become very brittle. Skin lesions in diabetes include necrobiosis lipoidica, granuloma annulare, scleroderma-like diabetic edema and acanthosis nigricans. Overactive pituitary gland is often manifested as acromegaly with hypertrophy of soft tissue thickening and hypertrichosis. The skin in the early stages of hypopituitarism feels swollen, is pale yellow and oily, and finally becomes alabaster and dry. The characteristic features of Cushing syndrome are central obesity, lunar face, buffalo hump, and striae. In Addison’s disease we observe hyperpigmentation. Hyperandrogenism in women leads to acne, hirsutism and virilization.

  13. A Case of Ectrodactyly, Ectodermal Dysplasia, Cleft Lip and Palate Syndrome Associated with Hydrocephaly

    Directory of Open Access Journals (Sweden)

    Buket Uysal Aladag

    2013-06-01

    Full Text Available Ectrodactyly, ectodermal dysplasia, cleft lip, and palate syndrome (EEC is a genetic developmental disorder characterized by ectrodactyly, ectodermal dysplasia and orofacial clefts (cleft lip/ palate. A few cases have been reported in literature. The cardinal components of the syndrome are ectrodactyly and syndactyly of the hands and feet, cleft lip with or without cleft palate, and abnormalities ectodermal structures including skin (i.e. hypopigmented and dry skin, hyperkeratosis, skin atrophy, hair (sparse hair and eye brows, teeth (small, absent or dysplastic teeth, nails (nail dystrophy and exocrine glands (reduction/ absence of sweat, sebaceous and salivary glands. A multidisciplinary approach for treatment is needed which is co-ordinated by orthopedic, plastic, dental surgeons, ophthalmologist, dermatologists and speech therapists, psychologists. We presented EEC syndrome case with hydrocephaly by the literature. [Cukurova Med J 2013; 38(3.000: 531-535

  14. Healing effect of Terminalia chebula Retz extract on second-degree ...

    African Journals Online (AJOL)

    degree skin burns by electrical scald instrument. The animals were divided into three groups as follows: ... for at least one week before use. The animal experiment was approved by the Animal Care and Use .... the burn wound effectively. In recent years, there has been a growing interest in alternative medicines and natural ...

  15. Mazabraud syndrome associated with McCune-Albright syndrome: a case report and review of the literature.

    Science.gov (United States)

    Biazzo, Alessio; Di Bernardo, Andrea; Parafioriti, Antonina; Confalonieri, Norberto

    2017-08-23

    Mazabraud syndrome is a very rare benign disorder characterized by the association of monostotic or polyostotic fibrous dysplasia and one or multiple intramuscular myxomas. McCune -Albright syndrome is a rare benign disorder characterized by the association of polyostotic fibrous dysplasia, cafè-au-lait skin pigmentations and endocrine dysfunction, such as precocious puberty, diabetes mellitus, goiter and breast fibroadenomatosis. The association of Mazabraud syndrome and McCune-Albright in the same patient is an anecdotal event. We report the case of a 28-year-old girl with Mazabraud syndrome associated with McCune-Albright syndrome. Our literature review shows that in these patients there is a higher risk of malignant transformation of fibrous dysplasia into osteosarcoma, confirming previous reports. Conversely, no malignant transformation has been reported for myxomas in isolated Mazabraud syndrome or in the association with McCune-Albright syndrome. We conclude that these patients should be scheduled to a close and long-term follow-up.

  16. Nevoid Basal-Cell Syndrome: literature review and case report in a family

    Directory of Open Access Journals (Sweden)

    Alfio José Tincani

    Full Text Available The Nevoid Basal-Cell Carcinoma Syndrome (NBCC, or as it is also referred to, basal-cell nevus syndrome or Gorlin-Goltz syndrome, is characterized by multiple early-appearing basal cell carcinomas, keratocytosis of the mandible, and anomalies of the ocular, skeletal reproductive system. We describe four patients in the same family, all of them possessing a large number of skin tumors associated with other typical clinical and X-Ray anomalies of NBCC. The definitive treatment of NBCC has yet to be established, however, early diagnosis is very important as well as the periodical follow-up examination of ten patients, mainly due to the transformations in the skin lesions that may occur.

  17. The role of defeathering in the contamination of turkey skin by Salmonella species and Listeria monocytogenes.

    Science.gov (United States)

    Clouser, C S; Doores, S; Mast, M G; Knabel, S J

    1995-04-01

    This study was undertaken to determine whether the incidence of either Salmonella spp. or Listeria monocytogenes on turkeys at three commercial processors could be related to the type of defeathering system: 1) conventional, 58 C common bath scald; 2) kosher, 7 C common bath scald; or 3) steam-spray, 62 C nonimmersion scald. Flocks were sampled before defeathering, after defeathering, and after chill at each facility. The incidence of Salmonella-positive turkeys significantly increased subsequent to conventional defeathering (10 positive out of 14) as compared with before defeathering (3/14). The number of Salmonella-positive carcasses following kosher (0/14) and steam-spray (2/14) defeathering were similar to the number of Salmonella-positive carcasses found prior to defeathering (1/14 and 3/14, respectively). The incidence of Salmonella-positive carcasses following chill was slightly lower, but not significantly different than the number of Salmonella-positive carcasses found immediately following defeathering at all processors (8/14, 0/14, 1/14 for conventional, kosher, and steam-spray processors, respectively). Although L. monocytogenes was detected on turkeys sampled before chilling (2/10, kosher) and after chilling (8/14, kosher; 1/14, conventional), no L. monocytogenes was detected on turkeys at any of the processors prior to the evisceration process. Flocks with high aerobic plate counts prior to processing were more likely to contain Salmonella-positive birds throughout processing. Aerobic plate counts of all flocks were similar after chill whether or not Salmonella spp. and L. monocytogenes were detected.

  18. Lipidomic analysis of epidermal lipids: a tool to predict progression of inflammatory skin disease in humans.

    Science.gov (United States)

    Li, Shan; Ganguli-Indra, Gitali; Indra, Arup K

    2016-05-01

    Lipidomics is the large-scale profiling and characterization of lipid species in a biological system using mass spectrometry. The skin barrier is mainly comprised of corneocytes and a lipid-enriched extracellular matrix. The major skin lipids are ceramides, cholesterol and free fatty acids (FFA). Lipid compositions are altered in inflammatory skin disorders with disrupted skin barrier such as atopic dermatitis (AD). Here we discuss some of the recent applications of lipidomics in human skin biology and in inflammatory skin diseases such as AD, psoriasis and Netherton syndrome. We also review applications of lipidomics in human skin equivalent and in pre-clinical animal models of skin diseases to gain insight into the pathogenesis of the skin disease. Expert commentary: Skin lipidomics analysis could be a fast, reliable and noninvasive tool to characterize the skin lipid profile and to monitor the progression of inflammatory skin diseases such as AD.

  19. Hepatic Artery Mycotic Aneurysm Associated with Staphylococcal Endocarditis with Successful Treatment: Case Report with Review of the Literature

    Directory of Open Access Journals (Sweden)

    Dhara Chaudhari

    2013-01-01

    Full Text Available Mycotic hepatic artery aneurysm is a vascular pathology associated with bacterial endocarditis. It is rare in occurrence after the introduction of effective antibiotics. We present a young patient with injection drug abuse associated staphylococcal endocarditis which was successfully treated with antibiotics and valve replacement who presented with abdominal pain. He was found to have mycotic aneurysm of hepatic artery which was successfully treated with coil embolization.

  20. Waardenburg syndrome type 2: an orthodontic perspective.

    Science.gov (United States)

    Şuhani, Raluca Diana; Şuhani, Mihai Flaviu; Muntean, Alexandrina; Mesaroş, Michaela Florica; Badea, Mîndra Eugenia

    2015-01-01

    Waardenburg syndrome is a rare form of neurocristopathy. It is a disorder in the development of neural crest cells, caused by an altered cellular migration during the embryonic phase. That alteration causes an association of different abnormalities such as pigmentary disturbances of the hair, iris, skin, stria vascularis of the cochlea, dystopia canthorum and sensorineural hearing loss. We report a case of a 14-year-old Romanian male, with a family history of Waardenburg syndrome (mother) and Usher syndrome (father - congenitally sensorineural hearing loss and retinal degeneration). The case particularities are: the correlation between malocclusion and Waardenburg syndrome due to hypoplastic alae nasi and also factors that produced hearing loss, which could be Waardenburg syndrome, Usher syndrome or the presence of the connexin 26 (W24X) gene mutation.

  1. Molecular Detection of Inducible Clindamycin Resistance among Staphylococcal Strains Isolated from Hospital Patients

    Directory of Open Access Journals (Sweden)

    Shadiyeh Abdollahi

    2013-04-01

    Full Text Available Background & Objectives: Macrolide, lincosamide and streptogramin B (MLSB antimicrobial agents are used in the treatment of staphylococcal infections. They prevent the microbial protein synthesis system through binding to 23 S rRNA. The aim of this study was to apply molecular methods to detect inducible clindamycin resistance genes among staphylococcal strains isolated from clinical specimens.   Methods : Two hundred staphylococcus strains were isolated from nose and throat swabs of patients in Toohid and Besat hospitals in Sanandaj . Antimicrobial susceptibilities of isolates were determined using disc diffusion method, agar screen test and D-Test. A multiplex PCR was performed using primers specific for erm (A, B, C, TR genes.   Results: Out of 200 isolates, 18.5 % were MRSA and 32% were MRCNS (methicillin resistant coagulase negative staphylococci. Of 80 erythromycin resistant isolates, 48 were coagulase negative and 32 were S. aureus. Among the 48 coagulase negative staphylococci (CONS isolates, 11.63% expressed the MLSB-inducible phenotypes. Using PCR, the frequency of different genes in the collection of isolates were as follows: ermA 5.41 % , erm B 5.41 % , and erm C 3.13%. The ermTR gene was negative in all isolates. Among the 32 S. aureus isolates, 9.38% expressed the MLSB-nducible phenotype. Using PCR, these isolates harbored erm A (2.22%, ermB (2.22%, ermC (2.22% and ermTR (2.22% .   Conclusion: This is the first study to show the rate of inducible clindamycin clinical isolates of staphylococci harboring erm genes in Sananadaj. It also demonstrated the frequency of erm genes was higher among CONS isolates than S. aureus. This data suggested the transfer of resistance gene from nonpathogenic to pathogenic strains is likely to happen. Therefore, screening and control of these resistance genes is recommended at clinical laboratories.

  2. Classification System for Individualized Treatment of Adult Buried Penis Syndrome.

    Science.gov (United States)

    Tausch, Timothy J; Tachibana, Isamu; Siegel, Jordan A; Hoxworth, Ronald; Scott, Jeremy M; Morey, Allen F

    2016-09-01

    The authors present their experience with reconstructive strategies for men with various manifestations of adult buried penis syndrome, and propose a comprehensive anatomical classification system and treatment algorithm based on pathologic changes in the penile skin and involvement of neighboring abdominal and/or scrotal components. The authors reviewed all patients who underwent reconstruction of adult buried penis syndrome at their referral center between 2007 and 2015. Patients were stratified by location and severity of involved anatomical components. Procedures performed, demographics, comorbidities, and clinical outcomes were reviewed. Fifty-six patients underwent reconstruction of buried penis at the authors' center from 2007 to 2015. All procedures began with a ventral penile release. If the uncovered penile skin was determined to be viable, a phalloplasty was performed by anchoring penoscrotal skin to the proximal shaft, and the ventral shaft skin defect was closed with scrotal flaps. In more complex patients with circumferential nonviable penile skin, the penile skin was completely excised and replaced with a split-thickness skin graft. Complex patients with severe abdominal lipodystrophy required adjacent tissue transfer. For cases of genital lymphedema, the procedure involved complete excision of the lymphedematous tissue, and primary closure with or without a split-thickness skin graft, also often involving the scrotum. The authors' overall success rate was 88 percent (49 of 56), defined as resolution of symptoms without the need for additional procedures. Successful correction of adult buried penis often necessitates an interdisciplinary, multimodal approach. Therapeutic, IV.

  3. Staphylococcal superantigen-like 5 activates platelets and supports platelet adhesion under flow conditions, which involves glycoprotein Ib alpha and alpha(IIb)beta(3)

    NARCIS (Netherlands)

    De Haas, C. J. C.; Weeterings, C.; Vughs, M. M.; De Groot, P. G.; Van Strijp, J. A.; Lisman, T.

    2009-01-01

    Objectives: Staphylococcal superantigen-like 5 (SSL5) is an exoprotein secreted by Staphylococcus aureus that has been shown to inhibit neutrophil rolling over activated endothelial cells via a direct interaction with P-selectin glycoprotein ligand 1 (PSGL-1). Methods and Results: When purified

  4. Noncontiguous Finished Genome Sequence of Staphylococcus aureus KLT6, a Staphylococcal Enterotoxin B-Positive Strain Involved in a Food Poisoning Outbreak in Switzerland

    Science.gov (United States)

    Tobes, Raquel; Manrique, Marina; Brozynska, Marta; Stephan, Roger; Pareja, Eduardo

    2013-01-01

    We present the first complete genome sequence of a Staphylococcus aureus strain assigned to clonal complex 12. The strain was isolated in a food poisoning outbreak due to contaminated potato salad in Switzerland in 2009, and it produces staphylococcal enterotoxin B. PMID:23704175

  5. Influence of holding temperature on the growth and survival of Salmonella spp. and Staphylococcus aureus and the production of staphylococcal enterotoxin in egg products.

    Science.gov (United States)

    Yang, S E; Yu, R C; Chou, C C

    2001-01-22

    In this study, growth and survival of Salmonella spp. and Staphylococcus aureus in steamed egg and scrambled egg held at 5, 18, 22, 37, 55 and 60 degrees C are investigated. The production of staphylococcal enterotoxin in steamed egg is also examined. Results reveal that Salmonella spp. and Staph. aureus in the egg products multiply best at 37 degrees C, followed closely by 22 and 18 degrees C. Neither pathogen showed growth in the egg products held at 5 degrees C. Initial inoculation dose, holding temperature and holding time affected the population of both organisms found in the egg products. Staphylococcal enterotoxin A (SEA) and B (SEB) are detected only in the egg products held at 37 or 22 degrees C. After holding at 37 degrees C for 36 h, scrambled egg inoculated with ca. 5.0 log cfu/g Staph. aureus contains the highest levels of SEA (> 64 ng/g) and SEB (> 64 ng/g). Although Salmonella spp. and Staph. aureus grow better in steamed eggs than in scrambled eggs, production of staphylococcal enterotoxin, in general, was higher in scrambled eggs than in steamed eggs. On the other hand, a repaid destruction of the test organisms in steamed eggs held at 60 degrees C was observed. Holding the steamed eggs at 60 degrees C, Salmonella spp. and Staph. aureus with an initial population of ca. 5.9 and 5.6 log cfu/g, respectively, reduced to a non-detectable level in 1 h.

  6. Worldwide distribution of Waardenburg syndrome.

    Science.gov (United States)

    Nayak, Chetan S; Isaacson, Glenn

    2003-09-01

    To clarify the multiracial occurrence of Waardenburg syndrome, we present a case series and literature review. A computerized review of the English-language literature was conducted to assess the distribution of reported occurrences of Waardenburg syndrome in populations around the world. We detail the clinical features of 2 family cohorts: one of Western European origin and the other from South Asia. A computerized literature review found sporadic cases of the syndrome in many ethnic groups, including Japanese, Taiwanese, and Middle Eastern families. The highest reported incidence is among Kenyan Africans. Waardenburg syndrome accounts for between 2% and 5% of cases of congenital deafness. It was first described in Northern European cohorts and is widely identified in fair-skinned populations. We hope to raise awareness of the worldwide distribution of this important cause of hearing loss.

  7. case report: Werner’s Syndrome

    Directory of Open Access Journals (Sweden)

    Faruk Kılınç

    2013-01-01

    Full Text Available Werner’s syndrome (WS is an extremely rare and autosomalrecessive premature aging syndrome characterizedby scleroderma-like skin changes, alopecia, legulcers, short stature, cataract, early atherosclerosis, osteoporosis,hypogonadism and increased susceptibilityto malignancies and diabetes mellitus. It can be typicallyrecognized at the third or fourth decades of life. Patientswith WS usually die at the age of 40-50 years due to malignanttumors or atherosclerotic complications. Therefore,early recognition of WS is of great importance forgenetic counseling and for the identification of malignanttumors, atherosclerosis, diabetes, or osteoporosis at anearly stage, since they are the most important factorscausing morbidity and mortality. In this article, growth retardation,premature aging, early cataract, the findings ofhypergonadotropic hypogonadism syndrome was hospitalizedand diagnosed with wermer 19-year-old male patientis presented.Key words: Werner’s syndrome, premature aging, hypogonadism

  8. Skin Diseases: Skin Health and Skin Diseases

    Science.gov (United States)

    Skip Navigation Bar Home Current Issue Past Issues Skin Diseases Skin Health and Skin Diseases Past Issues / Fall 2008 Table of Contents ... acne to wrinkles Did you know that your skin is the largest organ of your body? It ...

  9. The Use of Engineered Bilayered Skin (MyDermTM) in the Management of Massive Skin Defect in Grade III Gustilo-Anderson Open Fracture.

    Science.gov (United States)

    Mohamed Haflah, Nor Hazla; Ng, Min Hwei; Mohd Yunus, Mohd Heikal; Naicker, Amaramalar Selvee; Htwe, Ohnmar; Fahmi, Muhammad; Ishak, Mohamad Fikeri; Seet, Wan Tai; Khairoji, Khairul Anuar; Maarof, Manira; Chua, Kien Hui; Shamsuddin, Sharen Aini; Idrus, Ruszymah

    2017-09-01

    Open fracture Gustilo-Anderson grade IIIC is associated with higher risk of infection and problems with soft tissue coverage. Various methods have been used for soft tissue coverage in open fractures with large skin defect. We report a case of a patient who had grade IIIC open fracture of the tibia with posterior tibial artery injury. The patient underwent external fixation and reduction. Because of potential compartment syndrome after vascular repair, fasciotomy of the posterior compartment was performed. This wound, however, became infected and because of further debridement, gave rise to a large skin defect. A tissue engineered skin construct, MyDerm TM was employed to cover this large defect. Complete wound closure was achieved 35 days postimplantation. The patient then underwent plating of the tibia for nonunion with no adverse effect to the grafted site. The tibia eventually healed 5 months postplating, and the cosmetic appearance of the newly formed skin was satisfactory.

  10. A simplified multiplex PCR assay for fast and easy discrimination of globally distributed staphylococcal cassette chromosome mec types in meticillin-resistant Staphylococcus aureus

    NARCIS (Netherlands)

    E. Ghaznavi Rad (Ehsanollah); N.S. Mariana (Nor Shamsudin); Z. Sekawi (Zamberi); A.F. van Belkum (Alex); V. Neela (Vasanthakumari)

    2010-01-01

    textabstractA multiplex PCR assay was developed for the identification of major types and subtypes of staphylococcal cassette chromosome mec (SCCmec) in meticillin-resistant Staphylococcus aureus (MRSA) strains. The method uses a novel 9 valent multiplex PCR plus two primer pairs for S. aureus

  11. Evaluation of in-situ fatty acid extraction protocols for the analysis of staphylococcal cell membrane associated fatty acids by gas chromatography.

    Science.gov (United States)

    Crompton, Marcus J; Dunstan, R Hugh

    2018-05-01

    The composition and integrity of the bacterial cytoplasmic membrane is critical to the survival of staphylococci in dynamic environments and it is important to investigate how the cell membrane responds to changes in the environmental conditions. The staphylococcal membrane differs from eukaryotic and many other bacterial cell membranes by having a high abundance of branch fatty acids and relatively few unsaturated fatty acids. The range of available methods for extraction and efficient analyses of staphylococcal fatty acids was initially appraised to identify the best potential procedures for appraisal. Staphylococcus aureus subsp. aureus Rosenbach (ATCC® 29213) was grown under optimal conditions to generate a cell biomass to compare the efficiencies of three approaches to extract and prepare methyl esters of the membrane fatty acids: (1) acidic direct transesterification of lipids, (2) modified basic direct transesterification of membrane lipids with adjusted reaction times and temperatures, and (3) base catalysed hydrolysis followed by acid catalysed esterification in two separate chemical reactions (MIDI process). All methods were able to extract fatty acids from the cell mass effectively where these lipids represented approximately 5% of the cellular dry mass. The acidic transesterification method had the least number of steps, the lowest coefficient of variation at 6.7% and good resistance to tolerating water. Basic transesterification was the least accurate method showing the highest coefficient of variation (26%). The MIDI method showed good recoveries, but had twice the number of steps and a coefficient of variation of 16%. It was also found that there was no need to use an anti-oxidant such as BHT for the protection of polyunsaturated fatty acids when the GC-MS injection liner was clean. It was concluded that the acidic transesterification procedures formed the most efficient and reproducible method for the analyses of staphylococcal membrane fatty acids

  12. Short- versus long-duration levofloxacin plus rifampicin for acute staphylococcal prosthetic joint infection managed with implant retention: a randomised clinical trial.

    Science.gov (United States)

    Lora-Tamayo, Jaime; Euba, Gorane; Cobo, Javier; Horcajada, Juan Pablo; Soriano, Alex; Sandoval, Enrique; Pigrau, Carles; Benito, Natividad; Falgueras, Luis; Palomino, Julián; Del Toro, María Dolores; Jover-Sáenz, Alfredo; Iribarren, José Antonio; Sánchez-Somolinos, Mar; Ramos, Antonio; Fernández-Sampedro, Marta; Riera, Melchor; Baraia-Etxaburu, Josu Mirena; Ariza, Javier

    2016-09-01

    Levofloxacin plus rifampicin (L+R) is the treatment of choice for acute staphylococcal prosthetic joint infection (PJI) managed with debridement and implant retention (DAIR). Long courses have been empirically recommended, but some studies have suggested that shorter treatments could be as effective. Our aim was to prove that a short treatment schedule was non-inferior to the standard long schedule. An open-label, multicentre, randomised clinical trial (RCT) was performed. Patients with an early post-surgical or haematogenous staphylococcal PJI, managed with DAIR and initiated on L+R were randomised to receive 8 weeks of treatment (short schedule) versus a long schedule (3 months or 6 months for hip or knee prostheses, respectively). The primary endpoint was cure rate. From 175 eligible patients, 63 were included (52% women; median age, 72 years): 33 patients (52%) received the long schedule and 30 (48%) received the short schedule. There were no differences between the two groups except for a higher rate of polymicrobial infection in the long-schedule group (27% vs. 7%; P = 0.031). Median follow-up was 540 days. In the intention-to-treat analysis, cure rates were 58% and 73% in patients receiving the long and short schedules, respectively (difference -15.7%, 95% CI -39.2% to 7.8%). Forty-four patients (70%) were evaluable per-protocol: cure rates were 95.0% and 91.7% for the long and short schedules, respectively (difference 3.3%, 95% CI -11.7% to 18.3%). This is the first RCT suggesting that 8 weeks of L+R could be non-inferior to longer standard treatments for acute staphylococcal PJI managed with DAIR. Copyright © 2016 Elsevier B.V. and International Society of Chemotherapy. All rights reserved.

  13. Modified wound dressing with phyto-nanostructured coating to prevent staphylococcal and pseudomonal biofilm development

    Science.gov (United States)

    Anghel, Ion; Holban, Alina Maria; Grumezescu, Alexandru Mihai; Andronescu, Ecaterina; Ficai, Anton; Anghel, Alina Georgiana; Maganu, Maria; Lazǎr, Veronica; Chifiriuc, Mariana Carmen

    2012-12-01

    This paper reports a newly fabricated nanophyto-modified wound dressing with microbicidal and anti-adherence properties. Nanofluid-based magnetite doped with eugenol or limonene was used to fabricate modified wound dressings. Nanostructure coated materials were characterized by TEM, XRD, and FT-IR. For the quantitative measurement of biofilm-embedded microbial cells, a culture-based method for viable cell count was used. The optimized textile dressing samples proved to be more resistant to staphylococcal and pseudomonal colonization and biofilm formation compared to the uncoated controls. The functionalized surfaces for wound dressing seems to be a very useful tool for the prevention of wound microbial contamination on viable tissues.

  14. A case of Werner's syndrome associated with osteosarcoma.

    Science.gov (United States)

    Murata, K; Hatamochi, A; Shinkai, H; Ishikawa, Y; Kawaguchi, N; Goto, M

    1999-10-01

    We described a case of Werner's syndrome associated with osteosarcoma. A 37-year-old Japanese man was diagnosed as having Werner's syndrome by the presence of juvenile cataracts, skin sclerosis and hyperpigmentation of the feet, high-pitched voice, characteristic bird-like appearance of the face with beak-shaped nose, thinning of the entire skin and hyperkeratoses on soles, hyperlipemia, hyperuricemia, diabetes melitus, and the mutated responsible gene (WRN). He had a 3-month history of a tumor on his left forearm. Histologically, the tumor included four histological patterns; a malignant fibrous histiocytoma-like, a desmoid-like, a dermatofibrosarcoma protuberans-like, and a chondrosarcoma-like pattern. Tumoral osteoid formation was also found in the tumor. Therefore, the tumor was diagnosed as osteosarcoma.

  15. Methods for measuring cutaneous transfer of radionuclides through a damaged or undamaged epidermis - Application to radiotoxicology; Methodes de mesure du transfert cutane des radionucleides au travers d'un epiderme intact ou lese, application a la radiotoxicologie

    Energy Technology Data Exchange (ETDEWEB)

    Tymen, H. [Service de protection radiologique des armees, Clamart (France); Rateau, G.; Guillet, K.; Ramounet-Le Gall, B. [Lab. de Radiotoxicologie, Commissariat a l' Energie Atomique, CEA, Bruyeres le Chatel (France); Gerasimo, P. [Service de protection radiologique des armees, Clamart (France); Fritsch, P. [Lab. de Radiotoxicologie, Commissariat a l' Energie Atomique, CEA, Bruyeres le Chatel (France)

    2002-07-01

    Although skin contamination by radionuclides is the most common cause of nuclear workers accidents, few studies dealing with the penetration of radioactive contamination through the skin are available. This work is a review of experimental methods that allow to assess transfer of radionuclides through the skin in occupational conditions, with or without skin trauma. The first section describes the different methods applied for skin transfer assessment of chemicals used in pharmacology. Major radionuclide contamination accidents can be associated with skin traumas. Thus, the second section describes the adaptation of these methods to radiotoxicology. Finally, the third section is an in vivo investigation of cobalt transfer ({sup 57}CoCl{sub 2}) through undamaged and damaged skin which simulates different industrial accident conditions (excoriation, acid or alcalin burn, scalding, branding). (author)

  16. Increasing the frequency of hand washing by healthcare workers does not lead to commensurate reductions in staphylococcal infection in a hospital ward

    Directory of Open Access Journals (Sweden)

    Kerr Kevin G

    2008-09-01

    Full Text Available Abstract Background Hand hygiene is generally considered to be the most important measure that can be applied to prevent the spread of healthcare-associated infection (HAI. Continuous emphasis on this intervention has lead to the widespread opinion that HAI rates can be greatly reduced by increased hand hygiene compliance alone. However, this assumes that the effectiveness of hand hygiene is not constrained by other factors and that improved compliance in excess of a given level, in itself, will result in a commensurate reduction in the incidence of HAI. However, several researchers have found the law of diminishing returns to apply to hand hygiene, with the greatest benefits occurring in the first 20% or so of compliance, and others have demonstrated that poor cohorting of nursing staff profoundly influences the effectiveness of hand hygiene measures. Collectively, these findings raise intriguing questions about the extent to which increasing compliance alone can further reduce rates of HAI. Methods In order to investigate these issues further, we constructed a deterministic Ross-Macdonald model and applied it to a hypothetical general medical ward. In this model the transmission of staphylococcal infection was assumed to occur after contact with the transiently colonized hands of HCWs, who, in turn, acquire contamination only by touching colonized patients. The aim of the study was to evaluate the impact of imperfect hand cleansing on the transmission of staphylococcal infection and to identify, whether there is a limit, above which further hand hygiene compliance is unlikely to be of benefit. Results The model demonstrated that if transmission is solely via the hands of HCWs, it should, under most circumstances, be possible to prevent outbreaks of staphylococcal infection from occurring at a hand cleansing frequencies Conclusion Although our study confirmed hand hygiene to be an effective control measure, it demonstrated that the law of

  17. [Data mining analysis of professor Li Fa-zhi AIDS itchy skin medical record].

    Science.gov (United States)

    Wang, Dan-Ni; Li, Zhen; Xu, Li-Ran; Guo, Hui-Jun

    2013-08-01

    Analysis of professor Li Fa-zhi in the treatment of AIDS drug laws of itchy skin, provide the corresponding drug reference basis for Chinese medicine treatment of AIDS, skin itching. By using the method of analyzing the complex network of Weishi county, Henan in 2007 October to 2011 July during an interview with professor Li Fa-zhi treatment of AIDS patients with skin pruritus, etiology and pathogenesis analysis, skin itching AIDS syndrome differentiation of old Chinese medicine treatment and medication rule. The use of multi-dimensional query analysis, core drug skin itching AIDS treatment in this study as a windbreak, cicada slough, bupleurum, Qufeng solution table drug, licorice detoxification efficacy of drugs, Radix Scutellariae, Kochia scoparia, clearing away heat and promoting diuresis medicine; core prescription for Jingfang San streak virus. Professor Li Fa-zhi treatment of AIDS in the skin itching Qufeng solution table dehumidification antipruritic treatment.

  18. Paraneoplastiske syndromer

    OpenAIRE

    Røsbekk, Stein Helge

    2007-01-01

    During the last 50 years it has become clear that malignant tumours can induce symptoms unrelated to the mechanical effects of the primary tumour itself or its metastasis. Today, the name Paraneoplastic syndrome is given to those symptom complexes that may affect the blood cells, electrolytes, coagulation system, muscle, skin, nerve and the endocrine system. Endocrine symptoms were first recognised, and different hormones were isolated from the tumour tissue. However, tumour derived hormones ...

  19. Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis ...

    African Journals Online (AJOL)

    REVIEW. Introduction. Stevens-Johnson syndrome (SJS) and toxic epidermal ... that affect the skin and mucous membranes. ... Open Access article distributed under the terms of the .... pathogenic components are removed from plasma. The.

  20. Relationship between Fatigue and Gait Abnormality in Joint Hypermobility Syndrome/Ehlers-Danlos Syndrome Hypermobility Type

    Science.gov (United States)

    Celletti, Claudia; Galli, Manuela; Cimolin, Veronica; Castori, Marco; Albertini, Giorgio; Camerota, Filippo

    2012-01-01

    Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterised by joint hypermobility, skin hyperextensibility and tissue fragility. It has recently been shown that muscle weakness occurs frequently in EDS, and that fatigue is a common and clinically important symptom. The…

  1. Characterisation of virulence genes in methicillin susceptible and resistant Staphylococcus aureus isolates from a paediatric population in a university hospital of Medellín, Colombia

    Directory of Open Access Journals (Sweden)

    Judy Natalia Jiménez

    2011-12-01

    Full Text Available Virulence and antibiotic resistance are significant determinants of the types of infections caused by Staphylococcus aureus and paediatric groups remain among the most commonly affected populations. The goal of this study was to characterise virulence genes of methicillin-susceptible S. aureus (MSSA and methicillin-resistant S. aureus (MRSA strains isolated from a paediatric population of a Colombian University Hospital during 2009. Sixty MSSA and MRSA isolates were obtained from paediatric patients between zero-14 years. We identified the genes encoding virulence factors, which included Panton-Valentine leucocidine (PVL, staphylococcal enterotoxins A-E, exfoliative toxins A and B and toxic shock syndrome toxin 1. Typing of the staphylococcal chromosome cassette mec (SCCmec was performed in MRSA strains. The virulence genes were more diverse and frequent in MSSA than in MRSA isolates (83% vs. 73%. MRSA strains harboured SCCmec types IVc (60%, I (30%, IVa (7% and V (3%. SCCmec type IVc isolates frequently carried the PVL encoding genes and harboured virulence determinants resembling susceptible strains while SCCmec type I isolates were often negative. PVL was not exclusive to skin and soft tissue infections. As previously suggested, these differences in the distribution of virulence factor genes may be due to the fitness cost associated with methicillin resistance.

  2. Characterisation of virulence genes in methicillin susceptible and resistant Staphylococcus aureus isolates from a paediatric population in a university hospital of Medellín, Colombia.

    Science.gov (United States)

    Jiménez, Judy Natalia; Ocampo, Ana María; Vanegas, Johanna Marcela; Rodríguez, Erika Andrea; Garcés, Carlos Guillermo; Patiño, Luz Adriana; Ospina, Sigifredo; Correa, Margarita María

    2011-12-01

    Virulence and antibiotic resistance are significant determinants of the types of infections caused by Staphylococcus aureus and paediatric groups remain among the most commonly affected populations. The goal of this study was to characterise virulence genes of methicillin-susceptible S. aureus (MSSA) and methicillin-resistant S. aureus (MRSA) strains isolated from a paediatric population of a Colombian University Hospital during 2009. Sixty MSSA and MRSA isolates were obtained from paediatric patients between zero-14 years. We identified the genes encoding virulence factors, which included Panton-Valentine leucocidine (PVL), staphylococcal enterotoxins A-E, exfoliative toxins A and B and toxic shock syndrome toxin 1. Typing of the staphylococcal chromosome cassette mec (SCCmec) was performed in MRSA strains. The virulence genes were more diverse and frequent in MSSA than in MRSA isolates (83% vs. 73%). MRSA strains harboured SCCmec types IVc (60%), I (30%), IVa (7%) and V (3%). SCCmec type IVc isolates frequently carried the PVL encoding genes and harboured virulence determinants resembling susceptible strains while SCCmec type I isolates were often negative. PVL was not exclusive to skin and soft tissue infections. As previously suggested, these differences in the distribution of virulence factor genes may be due to the fitness cost associated with methicillin resistance.

  3. Gorlin-Goltz Syndrome: case report and review of literature

    Directory of Open Access Journals (Sweden)

    Adrianne Rahde Bischoff

    2014-10-01

    Full Text Available Description of a case report of Gorlin-Goltz Syndrome diagnosed in a male newborn who presented increased head circumference and bifid ribs. Mother and grandmother presented typical physical findings of the syndrome, including palmar pits, odontogenic cysts, and history of multiple skin cancer resections. The diagnosis was based on clinical findings of three relatives. A literature review is also presented.

  4. Paracentrin 1, a synthetic antimicrobial peptide from the sea-urchin Paracentrotus lividus, interferes with staphylococcal and Pseudomonas aeruginosa biofilm formation.

    Science.gov (United States)

    Schillaci, Domenico; Cusimano, Maria Grazia; Spinello, Angelo; Barone, Giampaolo; Russo, Debora; Vitale, Maria; Parrinello, Daniela; Arizza, Vincenzo

    2014-01-01

    The rise of antibiotic-resistance as well as the reduction of investments by pharmaceutical companies in the development of new antibiotics have stimulated the investigation for alternative strategies to conventional antibiotics. Many antimicrobial peptides show a high specificity for prokaryotes and a low toxicity for eukaryotic cells and, due to their mode of action the development of resistance is considered unlikely. We recently characterized an antimicrobial peptide that was called Paracentrin 1 from the 5-kDa peptide fraction from the coelomocyte cytosol of the Paracentrotus lividus. In this study, the chemically synthesized Paracentrin 1, was tested for its antimicrobial and antibiofilm properties against reference strains of Gram positive and Gram negative. The Paracentrin 1 was active against planktonic form of staphylococcal strains (reference and isolates) and Pseudomonas aeruginosa ATCC 15442 at concentrations ranging from 12.5 to 6.2 mg/ml. The Paracentrin 1 was able to inhibit biofilm formation of staphylococcal and Pseudomonas aeruginosa strains at concentrations ranging from 3.1 to 0.75 mg/ml. We consider the tested peptide as a good starting molecule for novel synthetic derivatives with improved pharmaceutical potential.

  5. Gorlin-Goltz syndrome.

    Science.gov (United States)

    Şereflican, Betül; Tuman, Bengü; Şereflican, Murat; Halıcıoğlu, Sıddıka; Özyalvaçlı, Gülzade; Bayrak, Seval

    2017-09-01

    Gorlin-Goltz syndrome is a rare multisystemic disease inherited in an autosomal dominant pattern. It is characterized by numerous basal cell carcinoma of the skin, jaw cysts, and skeletal anomalies such as frontal bossing, vertebral anomalies, palmoplantar pits, and falx cerebri calcification. There is a tendency to tumors including medullablastoma, fibroma, rabdomyoma, leiomyosarcoma etc.. The diagnosis is based on major and minor clinical and radiologic criteria. Early diagnosis and treatment are of utmost importance in reducing the severity of long-term sequelae of this syndrome. In this article, we present a 15-year-old boy who was admitted to our clinic with brown-black papules and plaques on his scalp and was thought to have Gorlin-Goltz syndrome. He had a history of medulloblastoma that was treated with surgical resection followed by cranial radiotherapy and unilateral retinoblastoma. We present this case, because association of Gorlin-Goltz syndrome and retinoblastoma has not been described previously in the literature and we aimed to draw attention to radiation-induced basal cell carcinomas.

  6. Poland’s syndrome

    Directory of Open Access Journals (Sweden)

    Olga E. Agranovich

    2017-03-01

    Full Text Available Poland’s syndrome is a rare congenital condition classically characterized by partial or complete absence of chest muscles on one side of the body and usually webbing of the fingers of the hand on the same side. There may also be rib (aplasia or hypoplasia and chest bone abnormalities, which may be noticeable due to less fat under the skin. Breast and nipple abnormalities may also occur, and underarm hair is sometimes sparse or abnormally placed. In most cases, the abnormalities in the chest area do not cause health problems or affect movement. Poland’s syndrome most often affects the right side of the body and occurs more frequently in males than in females. The etiology is unknown; however, interruption of the embryonic blood supply to the arteries that lie under the collarbone (subclavian arteries is the prevailing theory. There are many methods of operative correction because of the polymorphic clinical features of this syndrome. We gathered data on the etiology, pathogenesis, and clinical presentation of Poland’s syndrome and reviewed the existing surgical treatment options.

  7. A Mosaic Activating Mutation in AKT1 Associated with the Proteus Syndrome

    NARCIS (Netherlands)

    Lindhurst, Marjorie J.; Sapp, Julie C.; Teer, Jamie K.; Johnston, Jennifer J.; Finn, Erin M.; Peters, Kathryn; Turner, Joyce; Cannons, Jennifer L.; Bick, David; Blakemore, Laurel; Blumhorst, Catherine; Brockmann, Knut; Calder, Peter; Cherman, Natasha; Deardorff, Matthew A.; Everman, David B.; Golas, Gretchen; Greenstein, Robert M.; Kato, B. Maya; Keppler-Noreuil, Kim M.; Kuznetsov, Sergei A.; Miyamoto, Richard T.; Newman, Kurt; Ng, David; O'Brien, Kevin; Rothenberg, Steven; Schwartzentruber, Douglas J.; Singhal, Virender; Tirabosco, Roberto; Upton, Joseph; Wientroub, Shlomo; Zackai, Elaine H.; Hoag, Kimberly; Whitewood-Neal, Tracey; Robey, Pamela G.; Schwartzberg, Pamela L.; Darling, Thomas N.; Tosi, Laura L.; Mullikin, James C.; Biesecker, Leslie G.

    2011-01-01

    BACKGROUND The Proteus syndrome is characterized by the overgrowth of skin, connective tissue, brain, and other tissues. It has been hypothesized that the syndrome is caused by somatic mosaicism for a mutation that is lethal in the nonmosaic state. METHODS We performed exome sequencing of DNA from

  8. Autoerythrocyte Sensitization Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Pinar Ozuguz

    2014-04-01

    Full Text Available Autoerythrocyte sensitization syndrome or Gardner-Diamond syndrome is a rare syndrome, characterized by recurrent, spontaneous, painful ecchymosis. The lesions occur particularly after emotional stress or mild trauma. Psychiatric problems are commonly observed in these patients. The lower limbs and the trunk are the most often localizations; however, lesions can appear on any other skin area. It is thought to be a sensitivity to intradermally injected autoerythrocyte. The diagnosis is usually based on typical anamnesis, clinical presentation, absence of specific laboratory changes and positive intradermal test. This syndrome should be considered in the differential diagnosis of purpura, especially in patients with psychiatric problems and without any coagulopathy. Herein, a case of 38 year-old-female who has recurrent ecchymoses on her legs, fatigue, headache and sleeping problems is presented.

  9. New quantitative image analysis of staphylococcal biofilms on the surfaces of nontranslucent metallic biomaterials.

    Science.gov (United States)

    Adachi, Kouichi; Tsurumoto, Toshiyuki; Yonekura, Akihiko; Nishimura, Seisuke; Kajiyama, Shiro; Hirakata, Yoichi; Shindo, Hiroyuki

    2007-03-01

    Implant-related infection after orthopedic surgery is difficult to cure. One of the causes of infection is the bacterial biofilm that forms around biomaterials used during surgery. Therefore, it is necessary to investigate bacterial biofilms extensively to resolve the problems of these postoperative infections. However, no established culture method or quantification system exists for bacterial biofilms grown on the surface of the metallic biomaterials used in orthopedics, which are nonradiolucent. The purpose of this study was to develop a quantitative method to evaluate the difference in resistance of stainless steel versus titanium to staphylococcal biofilms and the efficacy of antibiotics against biofilms. The bacterial strains used in this study were three Staphylococcus aureus stains: strain Seattle 1945 and two clinical strains cultured from postoperative infections. Staphylococcal biofilms were formed on stainless steel washers (SUS304) and titanium washers (pure titanium). They were stained with crystal violet and were examined with a digital microscope to calculate the bacterial coverage rate (BCR) by NIH imaging. The BCR of S. aureus biofilms formed on stainless steel and titanium washers increased over time. At 24, 48, and 72 h after cultivation, the amount of biofilm on the surface of the stainless steel washers was significantly greater or tended to be greater than that on the titanium. Cefazolin was applied to the obtained biofilms of two clinically isolated S. aureus strains. Cefazolin did not eradicate the biofilms but significantly reduced the biofilm of one strain. The newly developed quantitative method (static microtube culture and measurement system) was useful for assessing the amount of bacterial biofilms on the surface of nontranslucent biomaterial. We found that titanium may be more resistant to bacterial infection than stainless steel. To control implant-related severe infections, the biomaterials should be assessed from the viewpoint of

  10. Eye Development Genes and Known Syndromes

    Science.gov (United States)

    Slavotinek, Anne M.

    2011-01-01

    Anophthalmia and microphthalmia (A/M) are significant eye defects because they can have profound effects on visual acuity. A/M is associated with non-ocular abnormalities in an estimated 33–95% of cases and around 25% of patients have an underlying genetic syndrome that is diagnosable. Syndrome recognition is important for targeted molecular genetic testing, prognosis and for counseling regarding recurrence risks. This review provides clinical and molecular information for several of the commonest syndromes associated with A/M: Anophthalmia-Esophageal-Genital syndrome, caused by SOX2 mutations, Anophthalmia and pituitary abnormalities caused by OTX2 mutations, Matthew-Wood syndrome caused by STRA6 mutations, Oculocardiafaciodental syndrome and Lenz microphthalmia caused by BCOR mutations, Microphthalmia Linear Skin pigmentation syndrome caused by HCCS mutations, Anophthalmia, pituitary abnormalities, polysyndactyly caused by BMP4 mutations and Waardenburg anophthalmia caused by mutations in SMOC1. In addition, we briefly discuss the ocular and extraocular phenotypes associated with several other important eye developmental genes, including GDF6, VSX2, RAX, SHH, SIX6 and PAX6. PMID:22005280

  11. Raman microspectroscopy as a diagnostic tool for the non-invasive analysis of fibrillin-1 deficiency in the skin and in the in vitro skin models.

    Science.gov (United States)

    Brauchle, Eva; Bauer, Hannah; Fernes, Patrick; Zuk, Alexandra; Schenke-Layland, Katja; Sengle, Gerhard

    2017-04-01

    Fibrillin microfibrils and elastic fibers are critical determinants of elastic tissues where they define as tissue-specific architectures vital mechanical properties such as pliability and elastic recoil. Fibrillin microfibrils also facilitate elastic fiber formation and support the association of epithelial cells with the interstitial matrix. Mutations in fibrillin-1 (FBN1) are causative for the Marfan syndrome, a congenital multisystem disorder characterized by progressive deterioration of the fibrillin microfibril/ elastic fiber architecture in the cardiovascular, musculoskeletal, ocular, and dermal system. In this study, we utilized Raman microspectroscopy in combination with principal component analysis (PCA) to analyze the molecular consequences of fibrillin-1 deficiency in skin of a mouse model (GT8) of Marfan syndrome. In addition, full-thickness skin models incorporating murine wild-type and Fbn1 GT8/GT8 fibroblasts as well as human HaCaT keratinocytes were generated and analyzed. Skin models containing GT8 fibroblasts showed an altered epidermal morphology when compared to wild-type models indicating a new role for fibrillin-1 in dermal-epidermal crosstalk. Obtained Raman spectra together with PCA allowed to discriminate between healthy and deficient microfibrillar networks in murine dermis and skin models. Interestingly, results obtained from GT8 dermis and skin models showed similar alterations in molecular signatures triggered by fibrillin-1 deficiency such as amide III vibrations and decreased levels of glycan vibrations. Overall, this study indicates that Raman microspectroscopy has the potential to analyze subtle changes in fibrillin-1 microfibrils and elastic fiber networks. Therefore Raman microspectroscopy may be utilized as a non-invasive and sensitive diagnostic tool to identify connective tissue disorders and monitor their disease progression. Mutations in building blocks of the fibrillin microfibril/ elastic fiber network manifest in disease

  12. p53 and the pathogenesis of skin cancer

    International Nuclear Information System (INIS)

    Benjamin, Cara L.; Ananthaswamy, Honnavara N.

    2007-01-01

    The p53 tumor suppressor gene and gene product are among the most diverse and complex molecules involved in cellular functions. Genetic alterations within the p53 gene have been shown to have a direct correlation with cancer development and have been shown to occur in nearly 50% of all cancers. p53 mutations are particularly common in skin cancers and UV irradiation has been shown to be a primary cause of specific 'signature' mutations that can result in oncogenic transformation. There are certain 'hot-spots' in the p53 gene where mutations are commonly found that result in a mutated dipyrimidine site. This review discusses the role of p53 from normal function and its dysfunction in pre-cancerous lesions and non-melanoma skin cancers. Additionally, special situations are explored, such as Li-Fraumeni syndrome in which there is an inherited p53 mutation, and the consequences of immune suppression on p53 mutations and the resulting increase in non-melanoma skin cancer in these patients

  13. Pollen food allergy syndrome in Turkey: Clinical characteristics and evaluation of its association with skin test reactivity to pollens.

    Science.gov (United States)

    Özdemir, Seςil Kepil; Özgüςlü, Selcan

    2017-09-17

    There is limited data regarding pollen food allergy syndrome (PFAS) in Turkey. To investigate the clinical characteristics and possible risk factors of PFAS in Turkey, and to evaluate if there was an association between skin test reactivity to pollens and presence of PFAS. A total of 254 consecutive adult patients with pollen sensitivity were prospectively recruited. Patients were interviewed with a questionnaire including a list of pollen-associated foods. Patients were classified as having PFAS if they reported clear allergic symptoms compatible with PFAS. All participants underwent skin prick tests (SPT) to a panel of common aeroallergens, prick-to-prick tests with culprit fresh foods were performed in patients who gave consent. Self-reported PFAS was observed in 49 patients (19.3%). The most common culprit foods were kiwi, peach, tomato, melon and watermelon. Multiple logistic regression analysis showed that potential risk factors for PFAS were having asthma (OR=2.392, P=0.044) and tree pollen sensitization (OR=2.904, P=0.004). There were no significant differences in the SPT wheal sizes to individual pollen extracts between patients with and without PFAS with a positive SPT result for that pollen extract (P> 0.05). PFAS is frequent in pollen sensitized adults in Turkey. The most commonly implicated foods are kiwi, peach,tomato, melon and watermelon, in our geographical region. SPT wheal sizes to pollen extracts seems to be similar in patients with and without PFAS.

  14. A Case of Male Goltz Syndrome

    Directory of Open Access Journals (Sweden)

    Bhaswati Ghoshal

    2012-01-01

    Full Text Available We present the case of a boy with a clinical diagnosis of Goltz syndrome (focal dermal hypoplasia, a rare genodermatosis characterized by widespread dysplasia of mesodermal and ectodermal tissues. A 9-year-old male patient with Goltz syndrome presented with typical skin lesions along with progressive dimness of vision and mental retardation since birth. It is inherited in an X-linked dominant fashion and is normally lethal in male patients, and so very few male patients, like the index case, have been reported.

  15. Staphylococcal phenotypes induced by naturally occurring and synthetic membrane-interactive polyphenolic β-lactam resistance modifiers.

    Directory of Open Access Journals (Sweden)

    Lucia Palacios

    Full Text Available Galloyl catechins, in particular (--epicatechin gallate (ECg, have the capacity to abrogate β-lactam resistance in methicillin-resistant strains of Staphylococcus aureus (MRSA; they also prevent biofilm formation, reduce the secretion of a large proportion of the exoproteome and induce profound changes to cell morphology. Current evidence suggests that these reversible phenotypic traits result from their intercalation into the bacterial cytoplasmic membrane. We have endeavoured to potentiate the capacity of ECg to modify the MRSA phenotype by stepwise removal of hydroxyl groups from the B-ring pharmacophore and the A:C fused ring system of the naturally occurring molecule. ECg binds rapidly to the membrane, inducing up-regulation of genes responsible for protection against cell wall stress and maintenance of membrane integrity and function. Studies with artificial membranes modelled on the lipid composition of the staphylococcal bilayer indicated that ECg adopts a position deep within the lipid palisade, eliciting major alterations in the thermotropic behaviour of the bilayer. The non-galloylated homolog (--epicatechin enhanced ECg-mediated effects by facilitating entry of ECg molecules into the membrane. ECg analogs with unnatural B-ring hydroxylation patterns induced higher levels of gene expression and more profound changes to MRSA membrane fluidity than ECg but adopted a more superficial location within the bilayer. ECg possessed a high affinity for the positively charged staphylococcal membrane and induced changes to the biophysical properties of the bilayer that are likely to account for its capacity to disperse the cell wall biosynthetic machinery responsible for β-lactam resistance. The ability to enhance these properties by chemical modification of ECg raises the possibility that more potent analogs could be developed for clinical evaluation.

  16. Imaging manifestations and its clinical significance in patients with synovitis acne pustulosis hyperostosis osteomyelitis syndrome

    International Nuclear Information System (INIS)

    Yu Wei; Lin Qiang; Yao Jinpeng; Chang Yinjuan; Zhou Xiaohong

    2012-01-01

    Objective: To describe the clinical and imaging manifestations of patients with synovitis acne pustulosis hyperostosis osteomyelitis (SAPHO) syndrome, and to analyze the diagnostic importance of different clinical and imaging manifestations for SAPHO syndrome. Methods: Seventeen patients (7 males and 10 females) with SAPHO syndrome were recruited in this study. Age ranged from 36 to 67 years with a mean age of (48 ± 8) years. All patients fulfilled the diagnostic criteria of Benhamou. Serum HLA B27 antigen records were reviewed for all patients. Imaging data of the abnormal bone sites were collected by conventional radiograph in all patients, CT in 13 patients as well as MR in 3 patients. Average time to take for a definite diagnosis of the syndrome was 3.7 years (ranged from O.5 to 13 years). Results: Serum HLA B27 antigen was positive in all patients. Both skin and bone abnormalities were found in all patients. Ten patients had skin palmoplantar pustulosis and two patients had acne. Involving sites of bone and joints include sacroiliac joints, anterior chest and limbs. Sacroiliac joints were asymmetrically involved with imaging features in all patients. Eight patients exhibited anterior chest wall involvement. Five patients had osteomyelitis at limbs. For all images of 17 patients, CT was superior to conventional radiography in detecting abnormal changes of bone erosion and soft tissue swelling. MR imaging was able to depict edema changes that was not detectable by CT and radiography. Conclusion: SAPHO syndrome is a rare disease, but for patients with skin and bone-joint abnormalities, especially with skin palmoplantar pustulosis, acne as well as with imaging features at the sacroiliac joint and anterior chest wall, SAPHO syndrome should be taken into a diagnostic consideration. (authors)

  17. Distinction between Pore Assembly by Staphylococcal α-Toxin versus Leukotoxins

    Directory of Open Access Journals (Sweden)

    Olivier Joubert

    2007-01-01

    Full Text Available The staphylococcal bipartite leukotoxins and the homoheptameric α-toxin belong to the same family of β-barrel pore-forming toxins despite slight differences. In the α-toxin pore, the N-terminal extremity of each protomer interacts as a deployed latch with two consecutive protomers in the vicinity of the pore lumen. N-terminal extremities of leukotoxins as seen in their three-dimensional structures are heterogeneous in length and take part in the β-sandwich core of soluble monomers. Hence, the interaction of these N-terminal extremities within structures of adjacent monomers is questionable. We show here that modifications of their N-termini by two different processes, using fusion with glutathione S-transferase (GST and bridging of the N-terminal extremity to the adjacent β-sheet via disulphide bridges, are not deleterious for biological activity. Therefore, bipartite leukotoxins do not need a large extension of their N-terminal extremities to form functional pores, thus illustrating a microheterogeneity of the structural organizations between bipartite leukotoxins and α-toxin.

  18. White-nose syndrome in bats: a primer for resource managers

    Science.gov (United States)

    Castle, K.T.; Cryan, P.M.

    2010-01-01

    White-nose syndrome emerged as a devastating new disease of North American hibernating bats over the past four winters. The disease has spread more than 1,600 kilometers (1,000 mi) since it was first observed in a small area of upstate New York, and has affected six species of bats in the caves and mines they rely on for winter survival. A newly discovered, cold-loving fungus (Geomyces destructans) causes the characteristic skin infection of white-nose syndrome and can infect presumably healthy bats when they hibernate. Although clear links between skin infection by G. destructans and death have not yet been established, the fungus is the most plausible cause of the disease. Thousands of caves and mines are administered by the National Park Service. Although bats testing positive for white-nose syndrome have been detected only at two sites in the National Park System thus far, the National Park Service (NPS) has been preparing for the spread and effects of white-nose syndrome through a proactive national program of response coordination, research support and interpretation, and education. National park areas across the nation are uniquely situated to help understand white-nose syndrome and its ecosystem impacts, and assist in the conservation and recovery of affected bat species.

  19. Neurocutaneous syndrome: A prospective study

    Directory of Open Access Journals (Sweden)

    Radheshyam Purkait

    2011-01-01

    Full Text Available Background: Neurocutaneous syndromes (NCS are a group of genetic disorders that produce a variety of developmental abnormalities of the skin along with an increased risk of neurological complications. Cutaneous manifestations usually appear early in life and progress with time, but neurological features generally present at a later age. There is a paucity of data regarding the evolution of skin lesions and their correlation with the central nervous system involvement in children. Aim: The primary objective was to track the course of skin lesions in various forms of NCS in the pediatric age group. Our secondary aim was to assess whether there was any predictive value of the lesions in relation to the neurological manifestations. Materials and Methods: This prospective longitudinal study was conducted at a tertiary care pediatric dermatology referral clinic of the Institute of Child Health, Kolkata, West Bengal. Children between the age group 0 and 12 years were included in the study on the basis of standard diagnostic criteria for different NCS, during the period from March, 2000 to February, 2004, and each of the enrolled cases were followed up for a duration of six years. Results: The study population comprised of 67 children (35 boys, 32 girls.The mean age of presentation was 33.8±27.8 months (range 10 days to 111 months. The various forms of NCS observed was neurofibromatosis 1(NF1 (n=33, tuberous sclerosis complex (TSC (n=23, Sturge Weber syndrome (n=6, ataxia telangiectasia (n=2, PHACE syndrome (n=1, incontinentia pigmenti (n=1, and hypomelanosis of Ito (n=1. The presentations were varied, ranging from predominantly cutaneous to primarily neurological, depending on the disease entity and age group concerned. There was a significant increase in the number of café au lait macules (CALMs with time (P=0.0002 in NF1, unlike that of hypopigmented macules of TSC (P=0.15. Statistically, no relation was documented between the evolution of skin

  20. In vitro and in vivo characterization of hazelnut skin prick test extracts

    NARCIS (Netherlands)

    Akkerdaas, Jaap H.; Wensing, Marjolein; Knulst, André C.; Aalberse, Rob C.; Hefle, Susan L.; van Ree, Ronald

    2003-01-01

    RATIONALE: Hazelnut allergy ranks among the most frequently observed food allergies. Clinical symptoms range from the oral allergy syndrome to life threatening anaphylaxis. Diagnosis of hazelnut allergy partially relies on in vivo testing by means of skin prick testing (SPT). The aim of this study

  1. Characterization of the residual structure in the unfolded state of the Delta 131 Delta fragment of staphylococcal nuclease

    DEFF Research Database (Denmark)

    Francis, C. J.; Lindorff-Larsen, Kresten; Best, R. B.

    2006-01-01

    dynamics simulations to characterise the residual structure of the 131 fragment of staphylococcal nuclease under physiological conditions. Our findings indicate that 131 under these conditions shows a tendency to form transiently hydrophobic clusters similar to those present in the native state of wild......The determination of the conformational preferences in unfolded states of proteins constitutes an important challenge in structural biology. We use inter-residue distances estimated from site-directed spin-labeling NMR experimental measurements as ensemble-averaged restraints in all-atom molecular...

  2. Advances in the medical management of the severe cutaneous radiation syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Carsin, H.; Stephannazi, J. [Percy Hospital, Clamart (France). Burn Treatment Centre; Gourmelon, P.

    2000-05-01

    The cutaneous radiation syndrome is a dose dependant complex pathological syndrome which follows a brief localized exposure and characterized by erythema, swelling, moist desquamation, ulceration and necrosis (25-30 Gy). Highly penetrating gamma radiation induces severe dose dependent lesions involving skin, subcutaneous tissue, muscle, vessels, nerves and occasionally, bony structures. The classical treatment of this syndrome includes the debridement of devitalized tissues, the application of bacteriostatic agents coated in non-adherent dressings, opiate-based drugs and in some cases the use of non-steroidal anti-inflammatory drugs. For ulceration and necrosis treatment, the classical surgery is ulcerectomy, necrectomy and amputation for the distal extremity injuries. For the profound et large necrosis, the lesion should be excised and the wound bed covered with a good quality, full-thickness skin graft. Unfortunately the delayed of appearance of this syndrome results often in non specialized medical treatment. Furthermore, because of the chronic evolution (months or years) the management of the cutaneous radiation syndrome has not been considered as a priority for the medical management. Recent accident like the Georgian accident demonstrated that new techniques such as artificial skin graft could change significantly patient prognosis. This technique is routinely used for thermal burn in specialized burn units. We realized the first application of this methodology in the field of radiopathology. An important factor of this technique is that in the case of recurrence of radionecrosis. Often observed, using this technique further grafting may be employed. However the success of this procedure depends on an effective control of the infection. These has sadly been illustrated in the last accident case in Peru where it was impossible to perform the artificial skin graft due to the persistence of a non-eradicated local infection. Whether this particular approach has a

  3. Advances in the medical management of the severe cutaneous radiation syndrome

    International Nuclear Information System (INIS)

    Carsin, H.; Stephannazi, J.; Gourmelon, P.

    2000-01-01

    The cutaneous radiation syndrome is a dose dependant complex pathological syndrome which follows a brief localized exposure and characterized by erythema, swelling, moist desquamation, ulceration and necrosis (25-30 Gy). Highly penetrating gamma radiation induces severe dose dependent lesions involving skin, subcutaneous tissue, muscle, vessels, nerves and occasionally, bony structures. The classical treatment of this syndrome includes the debridement of devitalized tissues, the application of bacteriostatic agents coated in non-adherent dressings, opiate-based drugs and in some cases the use of non-steroidal anti-inflammatory drugs. For ulceration and necrosis treatment, the classical surgery is ulcerectomy, necrectomy and amputation for the distal extremity injuries. For the profound et large necrosis, the lesion should be excised and the wound bed covered with a good quality, full-thickness skin graft. Unfortunately the delayed of appearance of this syndrome results often in non specialized medical treatment. Furthermore, because of the chronic evolution (months or years) the management of the cutaneous radiation syndrome has not been considered as a priority for the medical management. Recent accident like the Georgian accident demonstrated that new techniques such as artificial skin graft could change significantly patient prognosis. This technique is routinely used for thermal burn in specialized burn units. We realized the first application of this methodology in the field of radiopathology. An important factor of this technique is that in the case of recurrence of radionecrosis. Often observed, using this technique further grafting may be employed. However the success of this procedure depends on an effective control of the infection. These has sadly been illustrated in the last accident case in Peru where it was impossible to perform the artificial skin graft due to the persistence of a non-eradicated local infection. Whether this particular approach has a

  4. Clinical categories of exaggerated skin reactions to mosquito bites and their pathophysiology.

    Science.gov (United States)

    Tatsuno, Kazuki; Fujiyama, Toshiharu; Matsuoka, Hiroyuki; Shimauchi, Takatoshi; Ito, Taisuke; Tokura, Yoshiki

    2016-06-01

    Mosquito bites are skin irritating reactions, which usually resolve spontaneously without intensive medical care. However, in certain situations, mosquito bites may form a more vicious reaction, sometimes accompanying fever and systemic symptoms. In such cases, the presence of rare hematological disorders, abnormalities in eosinophils and/or association with Epstein-Barr virus (EBV) may underlie. Importantly, hypersensitivity to mosquito bites (HMB), which is characterized by necrotic skin reactions to mosquito bites with various systemic symptoms, is often observed in association with EBV infection and natural killer (NK) cell lymphoproliferative disorder. Exaggerated skin reaction to mosquito bites is also seen in Wells' syndrome. While strong Th2-skewing immune dysregulation is apparent in the patients, they also show robust CD4(+) T cell proliferation in response to mosquito salivary gland extracts, indicating close association between Wells' syndrome and mosquito bites. Similar skin reaction to mosquito bites is also noticed in certain types of B cell neoplasm, although the role of B cells in this peculiar reaction to mosquito bites is yet to be elucidated. In this review, we will discuss the current knowledge of exaggerated reaction toward mosquito bites seen in conjunction with these unique hematological disorders, and examine the scientific studies and observations reported in previous literatures to organize our current understanding of the pathogenesis of this distinct disorder. Copyright © 2016 Japanese Society for Investigative Dermatology. Published by Elsevier Ireland Ltd. All rights reserved.

  5. Sick building syndrome

    Directory of Open Access Journals (Sweden)

    Tjandra Y. Aditama

    2002-06-01

    Full Text Available Sick building syndrome describes a number of mostly unspesific complaints of some occupants of the building. The exact pathophysiological mechanism remains elusive. It is a multi factorial event which may include physical, chemical, biological as well as psycological factors. In many cases it is due to insufficient maintenance of the HVAC (heating, ventilation, air conditioning system in the building. Sign and symptoms can be uncomfortable and even disabling, which may include mucus membrane irritation, neurotoxic symptoms, asthma like symptoms, skin complaints, gastrointestinal symptoms and other related symptoms. There are various investigation methods to diagnose sick building syndrome, and on site assessment of the building is extremely useful. Prevention through a proactive air quality monitoring program is far more desirable than dealing with an actual sick building. Indoor air and the sick building symdrome serves as a paradigm of modern occupational and environmental medicine. (Med J Indones 2002; 11:124-31Keywords: indoor air pollution, sick building syndrome, building related illness

  6. [Healing of skin lesions in diabetic foot syndrome during hospitalization].

    Science.gov (United States)

    Jirkovská, A

    2006-05-01

    Wound healing during the diabetic foot disease is indicated to in-patient treatment in case of non-healing wound, in case of serious infection and/or critical ischemia and in case of necessity of surgical treatment. Diabetic foot disease is the main reason for in-patient treatment of people with diabetes, which our experience confirms. Chronic wound is characterised by non-healing for at least 4 weeks. Ischemia and recurrent trauma caused by incomplete off-loading, prolong inflammation and infection are the main reasons for difficult healing of chronic wound. Infection is also leading cause for prolonged hospitalisation of patients with diabetic foot disease. Local decrease of grow factors and increase of tissue protease are characteristics of chronic wound. The process of wound healing is characterized by a cascade of interrelated events involving infection and inflammatory factors. The results of these investigations led to the moist wound healing concept and use of growth factors and bioengineered skin substitutes. We have good experience with the use of xenotransplant skin substitues in the treatment of diabetic foot. Off loading techniques including total contact casting, local therapy by debridement and skin substitutes had the best evidence based efficacy. We are introducing new method of the treatment of diabetic foot--VAC--vacuum assisted closure. The fundamental principle in the therapy during in-patient period, is comprehensive approach; the omitting of any of the principle of the therapy--e.g. the off-loading of the ulcers, the infection and ischemia control, may contribute to its failure.

  7. The SAPHO syndrome revisited with an emphasis on spinal manifestations

    Energy Technology Data Exchange (ETDEWEB)

    Leone, Antonio; Casale, Roberto; Magarelli, Nicola; Semprini, Alessia; Colosimo, Cesare [Catholic University, School of Medicine, Department of Radiological Sciences, Rome (Italy); Cassar-Pullicino, Victor N. [The Robert Jones and Agnes Hunt Orthopaedic and District Hospital, Department of Diagnostic Imaging, Shropshire, England (United Kingdom)

    2015-01-15

    The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome includes a group of chronic, relapsing, inflammatory musculoskeletal disorders with similar manifestations, in particular synovitis, hyperostosis, and osteitis, which may or may not be associated with neutrophilic skin eruptions such as palmoplantar pustulosis and acne conglobata. The syndrome occurs at any age, can involve any skeletal site, and its imaging appearances are variable, depending on the stage/age of the lesion and imaging method. The diagnosis is difficult if there is no skin disease. Awareness of the imaging appearances, especially in the spine, may help the radiologist in avoiding misdiagnosis (e.g., infection, tumor) and unnecessary invasive procedures, while facilitating early diagnosis and selection of an effective treatment. In this article, we provide an overview of the radiological appearances of SAPHO syndrome, focusing on the magnetic resonance imaging findings of vertebral involvement, and present relevant clinical and pathological features that assist early diagnosis. (orig.)

  8. The SAPHO syndrome revisited with an emphasis on spinal manifestations

    International Nuclear Information System (INIS)

    Leone, Antonio; Casale, Roberto; Magarelli, Nicola; Semprini, Alessia; Colosimo, Cesare; Cassar-Pullicino, Victor N.

    2015-01-01

    The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome includes a group of chronic, relapsing, inflammatory musculoskeletal disorders with similar manifestations, in particular synovitis, hyperostosis, and osteitis, which may or may not be associated with neutrophilic skin eruptions such as palmoplantar pustulosis and acne conglobata. The syndrome occurs at any age, can involve any skeletal site, and its imaging appearances are variable, depending on the stage/age of the lesion and imaging method. The diagnosis is difficult if there is no skin disease. Awareness of the imaging appearances, especially in the spine, may help the radiologist in avoiding misdiagnosis (e.g., infection, tumor) and unnecessary invasive procedures, while facilitating early diagnosis and selection of an effective treatment. In this article, we provide an overview of the radiological appearances of SAPHO syndrome, focusing on the magnetic resonance imaging findings of vertebral involvement, and present relevant clinical and pathological features that assist early diagnosis. (orig.)

  9. EXPERIENCE OF ALPROSTADIL APPLICATION AGAINST RAYNAUD'S SYNDROME AMONG CHILDREN

    Directory of Open Access Journals (Sweden)

    E.I. Alexeeva

    2007-01-01

    Full Text Available The article provides the data on the causes and mechanisms of Raynaud's syndrome development. initial or idiopathic Raynaud's syndrome is characterized by the spasm of the digital arteries and thermoregulatory vessels of skin under the impact of the cold without any signs of vessel lesions. In the event of secondary Raynaud's syndrome, there is combination of Raynaud's syndrome with the symptoms of other diseases. Secondary raynaud's syndrome is most often associated with scleroderma systematica, systemic erythema centrifugum, other rheumatic diseases, hematologic disc orders and intake of some medications. There is also data on the opportunity to apply the synthetic medication prostaglandin е 1 — alprostadil to treat Raynaud's syndrome associated with rheumatic diseases. The given clinical example demonstrates high efficacy of alprostadil in case of the patient, suffering from scleroderma systematica and generalized Raynaud's syndrome.Key words: children, scleroderma systematica, alprostadil, Raynaud's syndrome.

  10. Impetigo

    Science.gov (United States)

    Impetigo is a skin infection caused by bacteria. It is usually caused by staphylococcal (staph) bacteria, but ... itchy, but scratching them can spread the sores. Impetigo can spread by contact with sores or nasal ...

  11. Tricuspid endocarditis in hyper-IgE syndrome

    Directory of Open Access Journals (Sweden)

    Gupta S

    2010-01-01

    Full Text Available Hyper-IgE syndrome is a congenitally acquired primary immune deficiency condition. We report a case of possible hyper-IgE syndrome who presented with multiple cold skin abscesses and chest infection due to Staphylococcus aureus and hyper-IgE findings. Patient also had tricuspid valve acute bacterial endocarditis with purulent pericarditis which is very rare. This case is presented to highlight that early diagnosis and treatment in such cases decreases the mortality and morbidity in phagocytic disorders.

  12. Ehlers danlos syndrome in two siblings

    Directory of Open Access Journals (Sweden)

    Das M

    2005-01-01

    Full Text Available Two cases of Ehlers-Danlos syndrome affecting two real brothers are being reported. Both of them presented with features of atrophy and hyperextensibility of skin, hypermobility of joints and scarring at the sites of trauma. The elder brother also had kyphoscoliosis and hypogonadism with testicular failure.

  13. Churg-Strauss syndrome and hemorragic vasculitis

    Directory of Open Access Journals (Sweden)

    Rui Moreira Marques

    2011-12-01

    Full Text Available Churg-Strauss syndrome (CSS is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the lefthand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome.

  14. Churg-Strauss syndrome and hemorrhagic vasculitis

    Science.gov (United States)

    Marques, Rui Moreira; Cabral, Ana Rita; Monteiro, Antonio; Henriques, Pedro

    2011-01-01

    Churg-Strauss syndrome (CSS) is a rare syndrome characterized by sinusitis, asthma and peripheral eosinophilia. This vasculitic syndrome affects medium and small-sized vessels, the lung being the most commonly affected organ, followed by the skin. The authors report a case of a 59-year-old male with a past history of asthma and allergic rhinitis. He presented necrohemorragic lesions in the distal phalanx of the 2nd, 3rd and 4th fingers of the left-hand and petechial lesions in the plant of both feet, accompanied by asthenia, anorexia and weight loss. The analytical study revealed leukocytosis with eosinophilia, elevated inflammatory parameters and p-ANCA positive antibodies. The diagnosis of CSS was established based on clinical and histopathological data. Cutaneous manifestations of hemorragic vasculitis are rare in CSS syndrome but can be the first manifestation of the disease. The recognition of this presentation is important for the early diagnosis and treatment of this syndrome. PMID:25386301

  15. Update on Hyper IgE syndrome (HIES

    Directory of Open Access Journals (Sweden)

    Javad Ghaffari

    2012-02-01

    Full Text Available Hyper IgE Syndrome (HIES is a rare primary immunodeficiency disease. Most of HIES cases are sporadic. Autosomal dominant HIES is caused by mutation in signal transducer and activator of transcription-3 (STAT-3. A number of mosaicism HIES has been reported that is associated with intermediate phenotype. Autosomal recessive HIES is due to mutation in Dock-8 or cytokine sis 8 and TYK2 or tyrosine kinase 2. The common anifestations are atopic eczema, staphylococcal dermatitis, cellulitis and folliculitis (cold dermal abscesses that are not warm, painful and without redness, recurrent pneumonia and pulmonary abscesses, osteopenia and recurrent bone fracture. The diagnosis of standard HIES is based on clinical suspicion. There is no specific treatment for HIES. The treatment should be based on the prevention of developing infections. Prophylactic antibiotics such as cotrimoxazole and IVIG are administered. Hematopoietic stem cell transplantation was done for all types of HIES, but there is a little information and experience about the long term results of this therapy.

  16. Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

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    Birgul Gumus

    2017-01-01

    Full Text Available Background. Keratitis-ichthyosis-deafness (KID syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature. Case Report. This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR, and the postoperative follow-up was conducted using pure-tone audiometry. Conclusion. Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications.

  17. Griscelli syndrome

    Directory of Open Access Journals (Sweden)

    Kumar T

    2006-01-01

    Full Text Available Partial albinism with immunodeficiency is a rare and fatal immunologic disorder characterized by pigmentary dilution and variable cellular immunodeficiency. It was initially described in 1978. Primary abnormalities included silvery grayish sheen to the hair, large pigment agglomerations in hair shafts and an abundance of mature melanosomes in melanocytes, with reduced pigmentation of adjacent keratinocytes. We describe a child with Griscelli syndrome who presented with hepatitis, pancytopenia and silvery hair. The diagnosis was confirmed by microscopic skin and hair examination.

  18. Hypothetical atopic dermatitis-myeloproliferative neoplasm (AD-MPN syndrome

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    Toshiaki eKawakami

    2015-08-01

    Full Text Available Atopic dermatitis (AD is a chronic inflammatory skin disease. Myeloproliferative neoplasms (MPNs are hematopoietic malignancies caused by uncontrolled proliferation of hematopoietic stem/progenitor cells. Recent studies have described several mutant mice exhibiting both AD-like skin inflammation and MPN. Common pathways for skin inflammation encompass overexpression of thymic stromal lymphopoietin and reduced signaling of epidermal growth factor receptor in the epidermis, while overproduction of granulocyte-colony stimulating factor by keratinocytes and constitutive activation of Stat5 in hematopoietic stem cells are important for the development of MPN. The murine studies suggest the existence of a similar human disease tentatively termed the AD-MPN syndrome.

  19. Fetal Therapy Model of Myelomeningocele with Three-Dimensional Skin Using Amniotic Fluid Cell-Derived Induced Pluripotent Stem Cells

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    Kazuhiro Kajiwara

    2017-06-01

    Full Text Available Myelomeningocele (MMC is a congenital disease without genetic abnormalities. Neurological symptoms are irreversibly impaired after birth, and no effective treatment has been reported to date. Only surgical repairs have been reported so far. In this study, we performed antenatal treatment of MMC with an artificial skin using induced pluripotent stem cells (iPSCs generated from a patient with Down syndrome (AF-T21-iPSCs and twin-twin transfusion syndrome (AF-TTTS-iPSCs to a rat model. We manufactured three-dimensional skin with epidermis generated from keratinocytes derived from AF-T21-iPSCs and AF-TTTS-iPSCs and dermis of human fibroblasts and collagen type I. For generation of epidermis, we developed a protocol using Y-27632 and epidermal growth factor. The artificial skin was successfully covered over MMC defect sites during pregnancy, implying a possible antenatal surgical treatment with iPSC technology.

  20. {sup 18}F-FDG PET/CT in POEMS syndrome

    Energy Technology Data Exchange (ETDEWEB)

    An, Young Sil; Yoon, Joon Kee; Hong, Seon Pyo; Joh, Chul Woo; Yoon, Seok Nam [Ajou University School of Medicine, Suwon (Korea, Republic of)

    2007-02-15

    POEMS syndrome is a rare disorder, also known as Crow-Fukase, PEP or Takatsuki syndrome. The acronym, POEMS, represents polyneuropathy, organomegaly, endocrinopathy, M protein and skin change. However, there are associated features not included in the acronym such as sclerotic bone lesions, Castleman disease, papilledema, thromobocytosis, peripheral edema, ascites, effusion, polycythemia, fatigue and clubbing. In most cases, osseous lesions in POEMS syndrome present as an isolated sclerotic deposit and that reveal as osteosclerotic myeloma. Several cases of {sup 18}F-FDG PET in multiple myeloma involvements were reported, but there was no previous literature that reported FDG PET findings in POEMS syndrome. We describe here a 66-year-old patient with POEMS syndrome who underwent {sup 18}F-FDG PET/CT image.