bluuebunny/crossref_metadata_2025 huggingface dataset:
Panniculitis with pseudomonas septicaemia in AIDS
Authors: Smith Ruth A. , Ross Janet S. , Branfoot Anthony C. , Asboe David , Shanson David C. , Gazzard Brian G. , Bunker Christopher B.
Published: August 2010

A 27 year old female with acquired immunodeficiency syndrome (AIDS) developed tender erythematous nodules on her thighs. A skin biopsy of the lesions showed septal panniculitis and venulitis and Pseudomonas species was cultured from both the urine and the skin biopsy tissue. The lesions resolved on antibiotic therapy. Panniculitic nodules may represent an early stage of ecthyma gangrenosum which is a well recognised cutaneous complication of pseudomonas septicaemia.
Toxic epidermal necrolysis successfully treated with cyclosporine. Report of three cases
Authors: Szepietowski Jacek , Wąsik Feliks , Szybejko‐Machaj Grażyna , Maj Joanna
Published: August 2010

Background Toxic epidermal necrolysis (TEN) is a severe, life‐threatening disease with many similarities to graft‐versus‐host disease (GVHD). Although the pathogenesis of TEN is still unclear, it is widely accepted that TEN is an immunologically mediated disease. The treatment of TEN is often unsatisfactory. Cyclosporin was demonstrated to affect the early phase of T‐cell activation and was recently shown to be effective in GVHD.

Observations Three cases of TEN were treated with 8–10 mg/kg/day cyclosporine in conjunction with corticosteroids. Cyclosporin therapy was continued for 10–21 days, depending on the severity of the disease. Rapid improvement in skin condition was observed in all patients; skin and mucous lesions healed completely after this treatment.

Conclusions Our findings suggest that cyclosporin can have a beneficial effect in TEN, and should he considered as treatment of choice in the early phase of TEN to arrest primary immunopathological processes.
Malignant proliferating tricholemmal tumor: report of three cases
Authors: Demirkesen Cuyan , Filizel Füsun , Ilvan Şennur , Kaner Gültekin , Oruç Nafi
Published: August 2010

We report three cases of malignant proliferating tricholemmal tumors, two of which displayed rapid growth of long‐standing scalp nodules. The third case was a nodule on the scapula with axillary lymph node metastasis. Although some investigators believe that the diagnosis of malignancy is certain only with evidence of metastasis: in our first two cases morphologic features suggested malignancy which included infiltration of adjacent tissues, high degree of cytologic atypia, abundant dyskeratotic cells, monstrous multinucleated cells and atypical mitotic figures. We emphasize the possibility of malignancy in long‐standing proliferating tricholemmal tumors that show rapid enlargement.
Comparison of SureSkin®, DuoDerm E® and Jelonet® Gauze in split skin donor sites – a clinical and histologial evaluation
Authors: Steenfos Henrik , Partoft Søren , Timshel Susanne , Balslev Eva
Published: August 2010

Background : The hydrocolloid dressings DuoDerm E®, and SureSkin® were compared to each other and Jelonet®/Gauze dressing served as the control.

Methods : In 10 split skin donor sites the 3 dressings were tested.

Results : DuoDerm E® and SureSkin® healed the donor sites in 8.5 ± 0.8 days compared to the control with a heating time of 12 ± 1.6 days. Histological biopsies confirmed the clinical results.

Conclusions : It was concluded that SureSkin® is equal to DuoDerm E®in healing split donor sites, and that hydrocolloid dressings produce 33% faster healing limes.
Lone circinate balanitis and genital keratoderma: underdiagnosed presentations of chlamydia infection in men?
Authors: Brook M. Gary , Kell Philip D. , Atia Waheeb A.
Published: August 2010

Objectives To describe the experience in a genito‐urinary clinic of lone circinate balanitis and genital keratoderma.

Design Retrospective analysis of all male GU clinic attenders diagnosed as circinate balanitis and genital keratoderma. chlamydia infection or bulanitis in a 12 month period.

Subjects Attenders at a North London genitor‐urinary clinic.

Main outcome measures Proportion of male patients with chlamydia or balanitis who had circinate balanitis and/or genital keratoderma.

Results Six men had circinate balanitis and/or keratoderma of the glans. All six were chlamydia positive whereas of 179 men with balanitis of other causes, only two had chlamydia (P < 0.001). Only one of the men with circinate balanitis had symptoms of urethritis although all had microscopic urethritis. Five of the 6 men (83%) were from ethnic minorities who constitute only 35% of our total male clinic attenders. Only one patient had any other manifestation of Reiter's syndrome (oral ulceration) and none had arthritis or conjunctivitis.

Conclusions Circinate balanitis or genital keratoderma occurring as single entities amongst our clinic population seems to be more common and more strongly associated with chlamydia infection than previously published literature would suggest.
Part Two: The development and implementation of the Australian and New Zealand Songlines and Haerenga training model
Authors: Norton Joy
Published: August 2010

Translations of Abstract

Les deux articles présentent les modèles de formation de la Société australienne et néo‐zélandaise d'analystes jungiens pour l'année 2006. Les auteurs y décrivent le processus et la réflexion qui sous‐tendent les modifications récentes apportées à la formation.

Dans le premier article, sont retracées les influences de la psychologie analytique et de la formation analytique sur le développement de l'ANZSJA (Australian and New Zealand Society of Jungian Analysts). La révision de la formation récemment entreprise révélait le désir de l'ANZSJA de continuer à former des analystes jungiens en explorant de nouveaux modes de conceptualisation de la formation. La pensée, les images et les métaphores qui sous‐tendent ce modèle innovateur de formation sont analysées. Le défi de proposer et de développer la formation dans une région géographique d'une vaste étendue, avec de faibles ressources a été relevé. L'ANZSJA a pris en compte les pratiques traditionnelles des peuples indigènes Australiens et Néo‐Zélandais. Le motif du voyage et le mouvement sont au coeur du modèle de l'ANZSJA. La formation dispensée par l'ANZSJA n'est pas contenue dans un ou plusieurs bâtiments, elle se tient sur des ≪sites de rencontre≫à travers les paysages de ces deux pays.

La responsabilité et la transparence sont au coeur de ce modèle de formation. Dans le second article, la structure et la documentation produites à l'occasion de cette révision sont contextualisées, exposées dans leurs grandes lignes et mises en rapport avec l'ethos qui étaye l'avancée de la formation analytique en Australie et en Nouvelle‐Zélande. L'accent porte ici sur les mécanismes d'ordre pratique et sur les processus, que nous avons fait évoluer pour donner naissance à notre nouveau modèle de formation. Les procédures d'engagement des analystes dans le nouveau modèle sont en cours de discussion.
The Innermost Kernel: Depth Psychology and Quantum Mechanics. Wolfgang Pauli's Dialogue with C.G. Jung, by Gieser, Suzanne
Authors: Hogenson George B.
Published: August 2010

Edited by Linda Carter and Marcus West
Doose syndrome (myoclonic–astatic epilepsy): 40 years of progress
Authors: KELLEY SARAH A , KOSSOFF ERIC H
Published: August 2010

Doose syndrome, otherwise traditionally known as myoclonic–astatic epilepsy, was first described as a unique epilepsy syndrome by Dr Hermann Doose in 1970. In 1989, the International League Against Epilepsy classified it formally as a symptomatic generalized epilepsy, and 20 years later it was renamed ‘epilepsy with myoclonic–atonic seizures’. In this review, we discuss the components of this unique disorder including its incidence, clinical features, and electroencephalographic findings. Recent evidence has suggested possible genetic links to the GEFS+ (generalized epilepsy with febrile seizures plus) family, and, additionally, some children with structural brain lesions can mimic the Doose syndrome phenotype. Treatment strategies such as corticosteroids, ethosuximide, and valproate have been described as only partially effective, but newer anticonvulsants, such as levetiracetam and zonisamide, may provide additional seizure control. The most effective treatment reported to date appears to be the ketogenic diet. Prognosis is quite varied in this disorder; however, many children can have a remarkably normal neurodevelopmental outcome.
Febrile seizures are a syndrome of secondarily generalized hippocampal epilepsy
Authors: NEVILLE BRIAN , GINDNER DIANE
Published: August 2010

Aim  The objective of this study was to examine in detail the semiology of febrile seizures, particularly to look for features that might suggest focality. In prolonged febrile seizures there is acute evidence of hippocampal involvement. Retrospective data relates mesial temporal sclerosis to such early prolonged febrile seizures. Animal models of prolonged seizures causing hippocampal damage show limbic seizures at low dose of the precipitants.

Method  A detailed history of the early ictal phase of 10 children with typical febrile seizures and of the behavioural components of 10 children with high fever was taken by two independent observers and a consensus reached.

Results  There were seven males and three females aged 1 year to 2y 8mo (mean age 1y 11mo). In seven of the 10 children with febrile seizures there was an early phase of the attack compatible with focal origin and in four of these there were clear mesial temporal features. No such features were seen in the children with fever alone.

Interpretation  We conclude that the majority of febrile seizures have evidence of focal origin and many appear to arise in the hippocampus.
3β‐HSD in songbird brain: subcellular localization and rapid regulation by estradiol
Authors: Pradhan Devaleena S. , Lau Loretta Y. M. , Schmidt Kim L. , Soma Kiran K.
Published: August 2010

J. Neurochem. (2010) 115, 667–675.

The enzyme 3β‐hydroxysteroid dehydrogenase/Δ5–Δ4 isomerase (3β‐HSD) catalyzes the conversion of dehydroepiandrosterone to androstenedione, thereby playing a key role in sex steroid synthesis. In peripheral tissues, 3β‐HSD is membrane‐bound, is present in both mitochondria and microsomes, and is regulated differentially in these two subcellular compartments. In the brain, 3β‐HSD is present, but its subcellular compartmentalization is unknown. Here, in Study 1, we examined the subcellular localization of 3β‐HSD in the brain of a songbird, the zebra finch. In Study 2, in males and females, we determined whether 3β‐HSD activity in different subcellular compartments is rapidly regulated by in vitro treatment with estradiol (E2), which has many rapid effects on the brain. Brain 3β‐HSD was enriched primarily in microsomes and secondarily in mitochondria and synaptosomes. In both males and females, E2 treatment rapidly (within 5 min) inhibited 3β‐HSD activity in both mitochondria/synaptosomes and microsomes, with greater inhibition in microsomes. We also assessed the activity of 5β‐reductase, which acts on androstenedione. E2 rapidly inhibited 5β‐reductase activity in microsomes only. This is the first study to examine the subcellular localization of 3β‐HSD in the brain, and the data demonstrate the importance of subcellular localization for the regulation of steroidogenic enzymes in the brain.
Health–illness transition among persons using advanced medical technology at home
Authors: Fex Angelika , Flensner Gullvi , Ek Anna‐Christina , Söderhamn Olle
Published: August 2010

Scand J Caring Sci; 2011; 25; 253–261
 
Health–illness transition among persons using advanced medical technology at home

This study aimed to elucidate meanings of health–illness transition experiences among adult persons using advanced medical technology at home. As an increasing number of persons perform self‐care while using different sorts of advanced medical technology at home, knowledge about health–illness transition experiences in this situation may be useful to caregivers in supporting these patients. A qualitative design was used. Five women and five men, all of whom performed self‐care at home, either using long‐term oxygen therapy from a ventilator or oxygen cylinder, or performing peritoneal or haemodialysis, were interviewed. Ethics committee approval was obtained. Informed consent was received from all participants, and ethical issues concerning their rights in research were raised. The interviews were analysed using a phenomenological hermeneutical methodology, including both an inductive and a deductive structural analysis. This method offers possibilities to obtain an increased understanding by uncovering a deeper meaning of lived experiences through interviews transcribed as texts. The health–illness transition for adult persons in this context was found to mean a learning process of accepting, managing, adjusting and improving daily life with technology, facilitated by realizing the gain from technology at home. Further, the meaning of the health–illness transition experience was interpreted as contentment with being part of the active and conscious process towards transcending into a new state of living, in which the individual and the technology were in tune. The healthy transition experience was characterized by human growth and becoming. This study elucidates one meaning of health–illness transition experiences in relation to the use of advanced medical technology on a more generic level, independent of the specific type of technology used. A positive attitude towards technology at home facilitates the transition.
Microarray analysis of a microbe–mineral interaction
Authors: OLSSON‐FRANCIS K. , VAN HOUDT R. , MERGEAY M. , LEYS N. , COCKELL C. S.
Published: August 2010

The weathering of volcanic minerals makes a significant contribution to the global silicate weathering budget, influencing carbon dioxide drawdown and long‐term climate control. Basalt rocks may account for over 30% of the global carbon dioxide drawdown in silicate weathering. Micro‐organisms are known to play a role in rock weathering yet the genomics and genetics of biological rock weathering are unknown. We apply DNA microarray technology to determine putative genes involved in weathering using the heavy metal‐resistant organism, Cupriavidus metallidurans CH34; in particular we investigate the sequestering of iron. The results show that the bacterium does not depend on siderophores. Instead, the up‐regulation of porins and transporters which are employed concomitantly with genes associated with biofilm formation suggests that novel passive and active iron uptake systems are involved. We hypothesize that these mechanisms induce rock weathering by changes in chemical equilibrium at the microbe–mineral interface, reducing the saturation state of iron. We also demonstrate that low concentrations of metals in the basalt induce heavy metal‐resistant genes. Some of the earliest environments on the Earth were volcanic. Therefore, these results not only elucidate the mechanisms by which micro‐organisms might have sequestered nutrients on the early Earth but also provide an explanation for the evolution of multiple heavy metal resistance genes long before the creation of contaminated industrial biotopes by human activity.
Endothelial function and hemodynamics in systemic sclerosis
Authors: Rossi Pascal , Granel Brigitte , Marziale Dominique , Le Mée Fanny , Francès Yves
Published: August 2010

Introduction:  Systemic sclerosis (SSc) is characterized by the development of fibrosis of skin and internal organs that is associated with vascular damage. However, its related parameters have not been fully explored. The aim of this study was to investigate endothelial function in SSc and its relationship with systolic pulmonary artery pressure and systemic arterial compliance (SAC).

Methods:  We studied 14 SSc females (4 with diffuse and 10 with limited cutaneous form of the disease) and 14 healthy controls matched for age and for cardiovascular risk factors. Endothelium‐dependent dilation (i.e. flow‐mediated) and endothelium‐independent (i.e. nitroglycerin‐induced) dilation of the brachial artery were measured as the percentage of change from baseline (FMD and NMD, respectively). In patients with SSc, SAC, cardiac output (CO), systemic arterial resistance and pulmonary artery pressure were estimated using echocardiography Doppler.

Results:  Heart rate, brachial artery pressure and body mass index did not differ between patients with SSc and controls. Flow‐mediated vasodilation (FMD) and NMD were significantly decreased in patients with SSc (10·3 ± 8·6 versus 26·6 ± 7·4%, P<0·001; 24·2 ± 8·4 versus 33·3 ± 10·1%, P<0·001, respectively). Postischaemia reactive hyperaemia was lower in patients with SSc (275 ± 185 versus 618 ± 366%, P<0·001). FMD and nitrate‐mediated dilation (NMD) were associated with CO, but not with SAC; moreover, FMD correlated with pulmonary artery pressure and peripheral arterial resistance conversely to NMD.

Conclusions:  Endothelium function in SSc is impaired independently to SAC. Furthermore, the severity of both small artery and pulmonary artery involvement may impact on endothelium‐dependant function.
Breed differences in the proportional morbidity of testicular tumours and distribution of histopathologic types in a population‐based canine cancer registry
Authors: Nødtvedt A. , Gamlem H. , Gunnes G. , Grotmol T. , Indrebø A. , Moe L.
Published: August 2010

Histologically verified tumours submitted to the Norwegian Canine Cancer Register from 1990 to 1998 were studied (n = 14 401). The proportion of testicular tumours (n = 345) was 2.4%, and the breakdown of histological tumour diagnoses is presented. The frequency of the most common histopathological types was 33% interstitial (Leydig), 26.4% Sertoli and 33.9% seminomas/germ cell tumours. The average age at diagnosis was 10 years, but was significantly lower for Sertoli cell tumours (8.6 years) than for the other tumour types. Following a histopathological re‐evaluation, 22.5% of the original tumor diagnoses were modified. Proportional morbidity ratios were calculated and individuals from the breeds Shetland sheepdog and Collie were five times more likely to have testicular tumours than the overall average for the registry. Breed differences in the distribution of histopathologic types were observed. Shetland sheepdog and Collie were most commonly diagnosed with Sertoli cell tumours, while all tumours from Norwegian elkhound in this material were seminomas.
Immunotherapy with autologous tumour antigen‐coated microbeads (large multivalent immunogen), IL‐2 and GM‐CSF in dogs with spontaneous B‐cell lymphoma
Authors: Henson M. S. , Curtsinger J. M. , Larson V. S. , Klausner J. S. , Modiano J. F. , Mescher M. F. , Miller J. S.
Published: August 2010

Cytotoxic T‐lymphocyte responses to subcellular antigens are enhanced when antigens are presented on cell‐sized silica microbeads called large multivalent immunogens (LMIs). LMIs prepared with tumour cell membrane fragments have induced partial remissions in humans with melanoma and renal cell carcinoma. The purpose of this phase I study was to evaluate the safety of LMIs, prepared with autologous lymphoma cell membranes, along with subcutaneous interleukin 2 (IL‐2) and granulocyte‐macrophage colony stimulating factor (GM‐CSF) in dogs with untreated B‐cell lymphoma. After lymph node excision and induction chemotherapy, five dogs were vaccinated with three weekly doses of LMI alone; five with LMI and subcutaneous IL‐2 and five with LMI, IL‐2 and GM‐CSF. No significant toxicity was noted, treatment did not adversely affect disease‐free interval and half of the dogs showed measurable delayed‐type hypersensitivity reactions to intradermal challenge with LMI, suggesting specific cell‐mediated immunity.
Subcutaneous Phycomycosis Caused byBasidiobolus haptosporus: A Clinicopathologic and Mycologic Study in a Child
Authors: de Leon‐Bojorge Beatriz , Ruiz‐Maldonado Ramon , Lopez‐Martinez Ruben
Published: August 2010

Abstract:The clinical, histopathologic, and mycologic aspects of a case of subcutaneous phycomycosis caused byBasidiobolus haptosporusin a 3‐year‐old boy are reported. This is the first documented case from Central and North America. Oral potassium iodide and trimethoprim‐sulfamethoxazole gave excellent therapeutic results.
Diamond‐Gardner Syndrome: A Case Report
Authors: Regazzini R. , Malagoli P.G. , Zerbinati N. , De Filippi C. , Serra F. , Donadiin A.
Published: August 2010

Abstract: We present a 13‐year‐old girl who came to our attention for an erythematous bruised lesion which appeared spontaneously without any apparent coagulopathy. The history, histology, and positive “skintest,” carried out by subcutaneously injecting autologous erythrocytes obtained from heparinized blood, confirmed the suspected diagnosis of Diamond‐Gardner syndrome. No alterations of blood vessel walls or thromboses were found. Evaluation of hemocoagulative parameters revealed an increased factor VIII level and reduced platelet aggregation after stimulation by adenosine diphosphate (ADP.)
Cutaneous Myiasis Caused by Dermatobia hominis
Authors: Veraldi Stefano , Gorani Alberto , Süss Luciano , Tadini Gianluca
Published: August 2010

Abstract: We report a case of myiasis caused by larvae of Dermatobia hominis in a 12‐year‐old boy. The infestation was acquired in Uruguay and was characterized by a single, large, inflammatory, nodular lesion located on the scalp. The lesion was accompanied by local pruritus and pain as well as diffuse headache and regional lymphadenopathy. From the lesion a single larva in stage III, of noteworthy size, was removed. Very few pediatric cases of myiasis due to D. hominis have been reported in the literature. Furthermore, as far as we know, just one adult case of myiasis caused by D. hominis acquired in Uruguay has been published.
Cervicofacial Hemangioma and a Minor Sternal Malformation: Inclusion in PHACES Syndrome?
Authors: Boulinguez S. , Teillac‐Hamel D. , Bédane C. , Bennaceur S. , De Prost Y.
Published: August 2010

Abstract: Hemangiomas are frequent in childhood, but those associated to dysmorphic anomalies are rare. We report a young girl with hemangiomas of the face, neck, and upper trunk, severe laryngeal dyspnea, and sternal atrophic skin without sternal nonunion, probably corresponding to a minor form of PHACES syndrome.
Fatal Pseudomonas Septicemia and Vasculitis in a Premature Infant
Authors: Hughes J. R. , Newbould M. , du Vivier A. W. P. , Greenough A.
Published: August 2010

Abstract: A characteristic vasculitis is an uncommon but wellrecognized complication of severe Pseudomonas aeruginosa infection in the neonate. Although it is one of the most common gram‐negative organisms responsible for overall nosocomial infection, it is a rare cause of intrauterine or intrapartum infection of the newborn. We describe a fatal Pseudomonas aeruginosa infection occurring in a very premature infant with widespread skin necrosis. Although this organism is relatively uncommon in the neonate, premature infants are at increased risk of infection, with a significant associated mortality. Early recognition and treatment are therefore very important.
Congenital Lipoblastomatosis of the Lower Extremity in a Neonate
Authors: Calobrisi Stella D. , Garland Jeffery S. , Esterly Nancy B.
Published: August 2010

Abstract: Lipoblastomatosis is a benign tumor of embryonic fat that is more common in male infants. It occurs more frequently in the soft tissues of the extremities. The diagnosis is made by biopsy, which shows globules of lipocytes and lipoblasts mixed with spindled and myxoid cells. MRI demonstrates fat infiltrating fascia and muscle. The infant described had clinical, histologic, and radiologic findings consistent with this diagnosis. Because of concern that total excision would compromise function, a debulking procedure is planned.
Keratitis, Ichthyosis, and Deafness (KID) Syndrome in Half Sibs
Authors: Koné‐Paut Isabelle , Hesse Sylvie , Palix Christian , Rey Réjane , Rémédiani Karine , Garnier Jean‐Marc , Berbis Philippe
Published: August 2010

Abstract: The keratitis, ichthyosis, and deafness (KID) syndrome is a rare congenital disorder of the ectoderm characterized by diffuse hyperkeratotic erythroderma, keratitis with neovascularization of the cornea, and severe neurosensory hearing loss. A familial occurrence of this syndrome has been mentioned in four reports including three of vertical transmission and one of two affected sisters born from consanguineous, unaffected parents. We report for the first time a familial case of KID syndrome in two half siblings born to the same unaffected mother. This new observation allows us to propose various hypotheses about its mode of inheritance.
Clinical Features and Long‐Term Follow‐Up of 20 Cases of Parakeratosis Pustulosa
Authors: Tosti Antonella , Peluso Anna Maria , Zucchelli Valeria
Published: August 2010

Abstract: We report the clinical features and long‐term follow‐up of 20 pediatric patients with parakeratosis pustulosa (PKP). In eight children PKP was considered as a clinical manifestation of psoriasis. Of these, three children had skin psoriasis at the time of our observation, two developed nail psoriasis, and three developed psoriatic pitting during follow‐up. Parakeratosis pustulosa was considered a symptom of allergic contact dermatitis in four patients, whereas atopic dermatitis was possibly responsible for PKP in two patients. A complete recovery from disease was observed in 11 patients. The results of our study suggest that PKP is not a single entity but rather represents a nail symptom that can be produced by several inflammatory diseases, including nail psoriasis, atopic dermatitis, and contact dermatitis.
Mucocutaneous Manifestations in Children with HIV Infection and AIDS
Authors: Hachem May El , Bernardi Stefania , Pianosi Giuseppe , Krzysztofiak Andrzej , Livadiotti Susanna , Gattinara Guido Castelli
Published: August 2010

Abstract: Mucocutaneous diseases are more frequent in HIV/AIDS‐infected children than in the normal population. We analyze mucocutaneous disorders with atypical presentations in a large population of HIV‐infected children, with or without full‐blown AIDS, compared to a population of HIV seroreverted children. The majority of these cutaneous disorders have an infectious etiology and their frequency is related to the degree of deterioration of the immune system. Some diseases commonly observed in adults are rare in children; neoplasms are an exception.
Congenital Lower Lip Pits (Van der Woude Syndrome): Presentation of 10 Cases
Authors: Nagore Eduardo , Sánchez‐Motilla José M. , Febrer M. Isabel , Serrano Gabriel , Bonillo Juan , Aliaga Adolfo
Published: August 2010

Abstract: Congenital lower lip pits are a rare developmental malformation of the lower lip. Clinically they present as bilateral depressions in the vermilion zone of the lower lip. It is important to be aware of this disorder because lower lip pits have also been reported in a variety of other congenital disorders and are associated with other malformations. Ten cases of this entity are presented. Nine of the 10 patients are members of two related families. The clinical and pathologic picture as well as some of the genetic and therapeutic aspects of this peculiar condition are discussed.
Childhood Vulval Lichen Sclerosus in a Patient with Ectodermal Dysplasia and Uncombable Hair
Authors: Powell Jenny , Wojnarowska Fenella , Dawber Rodney , Slavotinek Anne , Huson Susan
Published: August 2010

Abstract: An 11‐year‐old girl was diagnosed as having ectodermal dysplasia, with abnormalities of her nails, teeth, and hair noted from infancy. Her hair was unruly and investigations confirmed the changes seen in “cheveux incoiffables”—the uncombable hair syndrome. She also had atopy requiring treatment to control asthma and eczema. When she was 8 years old she developed vulval lichen sclerosus, which was controlled easily with early treatment. The coexistence of these conditions is discussed.
Unnecessary Milk Elimination Diets in Children with Atopic Dermatitis
Authors: Sinagra J.L. , Bordignon V. , Ferraro C. , Cristaudo A. , Di Rocco M. , Amorosi B. , Capitanio B.
Published: August 2010

Abstract:  Milk elimination diets are frequently adopted in the treatment of atopic dermatitis, although the real prevalence of clinically relevant food allergy remains unclear and reports from different authors are often in disagreement. We investigated the percentage of children allergic to cow's milk compared with the rate of milk exclusion diets in a group of patients with atopic dermatitis. We enrolled 206 children (79 girls, 127 boys), mean age 45.8 (4–68) months, affected by atopic dermatitis into our study. All children underwent radioallergosorbent test for casein, alpha‐lactalbumin and beta‐lactoglobulin, prick test, atopy patch test, and oral provocation test. Children were followed up at 1, 3, 6, and 12 months. Of the 206 patients, 20 were excluded from statistical analysis, leaving 186. Forty‐five (24.2%) were on a milk elimination diet and 141 on a normal diet. Four patients on the milk‐free diet (8.9%), accounting for 2.2% of all patients, were found to be allergic. In the others, milk reintroduction did not cause the disease to worsen during the follow‐up period. No children on a normal diet were found to be allergic. Our results demonstrated an actual prevalence of cow's milk allergy in patients on milk elimination diets (4%) to be significantly lower than the number of patients prescribed such diets (24.2%)—confirming that this measure is being applied excessively.
Trichorrhexis Nodosa Secondary to Argininosuccinicaciduria
Authors: Fichtel Jill C. , Richards Judith A. , Davis Loretta S.
Published: August 2010

Abstract:  Argininosuccinicaciduria is a rare metabolic disorder of the urea cycle associated with the inability to excrete nitrogenous waste in the form of urea. Along with low serum arginine, hepatomegaly, and mental retardation, congenital trichorrhexis nodosa is a distinguishing feature of the disorder. We present a 3.5‐year‐old girl diagnosed with argininosuccinicaciduria who presented to the dermatology clinic with hair thinning and loss since birth. Microscopic evaluation revealed nodular swellings on the hair shafts and frayed cortical fibers consistent with the diagnosis of trichorrhexis nodosa occurring in the setting of argininosuccinicaciduria.
Cushing Syndrome from Percutaneous Absorption of 1% Hydrocortisone Ointment in Netherton Syndrome
Authors: Halverstam Caroline P. , Vachharajani Akshaya , Mallory Susan Bayliss
Published: August 2010

Abstract:  Netherton syndrome is a congenital skin disease associated with decreased skin barrier function and increased percutaneous absorption. We report an 11‐year‐old boy with Netherton syndrome who developed Cushing syndrome after application of 1% hydrocortisone ointment to his entire body for more than 1 year. This presentation illustrates that even low‐potency steroid ointments should be used with caution in Netherton syndrome and warns about the use of long‐term topical medications with potential systemic side effects when used in large quantities in any chronic skin disease.
Pityriasis Lichenoides Chronica: An Association with Autoimmune Hepatitis?
Authors: Saltik‐Temizel Inci Nur , Ersoy‐Evans Sibel , Demir H. , Yüce A.
Published: August 2010

Abstract:  Pityriasis lichenoides is an uncommon, benign skin disorder with two major variants: acute and chronic. Autoimmune hepatitis comprises heterogeneous forms of chronic hepatitis that are generally progressive and often fluctuating. Two major forms of this entity are recognized. Herein, we report the concurrence of pityriasis lichenoides chronica with type I autoimmune hepatitis in a child, which, to the best of our knowledge, has not been previously reported. Although it is hard to say whether an etiologic relationship or coincidental coexistence occurred between the two entities in our patient, some common mechanisms may be involved in both diseases.
MCI‐154: A Second Generation Ca2+Sensitizer That Does Not Impair Relaxation — A Novel Approach to the Treatment of Heart Failure
Authors: Kitada Yoshimi
Published: August 2010

With the negative results of the cyclic AMP‐dependent positive inotropic agents in clinical trials, great interest has been focused on the development of agents that directly activate cardiac myofilaments. These drugs are called “Ca2+sensitizers”; they are expected to represent a possible new pharmacological approach to the therapy of chronic heart failure. MCI‐154 is one of the most powerful and promising Ca2+sensitizers currently in clinical trials. In preclinical studies, the positive inotropic action of MCI‐154 was observed at the concentrations at which the drug did not increase intracellular Ca2+. In skinned cardiac muscle fiber bundles from various animal species MCI‐154 shiftedpCa(‐log [Ca2+] M)‐force relation upward and to the left, suggesting that this drug not only increases sensitivity of myofibrils to Ca2+, but also enhances cross‐bridge interaction. In regard to the molecular mechanism of action of MCI‐154, the earliest experiments showed that MCI‐154 at the high concentration (10−4M) stimulated binding of Ca2+to troponin (Tn) C. However, MCI‐154‐(at concentrations lower than 10−5M)‐induced increase in the affinity of TnC to Ca2+led to the complex formation of TnC with TnI and TnT. This effect of the drug on Ca2+regulation and interaction between troponin subunits was discovered using fluorescence spectroscopy. At 10−4M MCI‐154 decreased binding of Ca2+to TnC. The Ca2+sensitizing effect of MCI‐154 disappeared when cardiac TnI was exchanged for skeletal TnI. Taken together, it can be concluded that TnI may represent a target protein for the positive inotropic action of MCI‐154. The long‐term treatment with MCI‐154 prolonged the life span of cardiomyopathic hamsters, Biol 14.6, a model that resembles human heart failure. Unlike PDE inhibitors, MCI‐154 did not aggravate arrhythmias generated in the two‐stage coronary ligation‐, digitalis‐ and catecholamine‐induced canine arrhythmia models. In clinical studies MCI‐154 improved contractile function in patients with left ventricular dysfunction after myocardial infarction. Its effect was not associated with an increase in myocardial oxygen consumption or impaired relaxation. In conclusion, MCI‐154 could be promising in the treatment of chronic heart failure. Clinical studies with MCI‐154 are currently in progress.
Fluvastatin, HMG‐CoA Reductase Inhibitor: Antiatherogenic Profiles Through Its Lipid‐Lowering–Dependent and ‐Independent Actions
Authors: Mitani Hironobu , Kimura Masaaki
Published: August 2010

Fluvastatin, the first totally synthetic inhibitor of 3‐hydroxy‐3‐methylglutaryl coenzyme A (HMG‐CoA) reductase, has the unique structure of a mevalonolactone derivative of a fluorophenyl‐substituted indole ring. Fluvastatin markedly reduces plasma lipid levels in animals and humans by inhibiting the activity of HMG‐CoA reductase following the up‐regulation of low density lipoprotein receptors, as it occurs with other HMG‐CoA reductase inhibitors. Several lines of evidence indicate that the hypolipidemic action of fluvastatin results in antiatherosclerotic effects in hyperlipidemic animal models. Recent findings have shown, however, that fluvastatin inhibits the progression of atherosclerosis independent of its lipid‐lowering effects. In addition, fluvastatin inhibits key events or factors in the initiation or progression of atherosclerosis. These effects include antioxidant and antithrombotic properties, improvement of endothelial function, inhibition of activated monocyte‐endothelial cell interaction, and inhibition of activated macrophage and smooth muscle cell function.

Recent large clinical trials with fluvastatin demonstrated its beneficial effects on the incidence of clinical events and on the progression of atherosclerotic plaques in patients with hyperlipidemia. Although angiographic changes (e.g., the decrease in minimum lumen diameter) in response to therapy were modest, the accompanying clinical benefit appeared to be significant. The lipid‐lowering therapy can not fully explain the results of clinical trials. The antiatherogenic profiles of fluvastatin might contribute to the decrease in the clinical events by directly inhibiting the atherosclerotic plaque progression and stabilizing the atherosclerotic plaque lesion.

Elucidation of pleiotropic effects of fluvastatin other than lipid‐lowering properties encourages the clinical use of fluvastatin not only for the reduction of plasma cholesterol levels but also for the secondary prevention of coronary heart disease in patients with hypercholesterolemia.
Pharmacology of the Thromboxane Receptor Antagonist and Thromboxane Synthase Inhibitor BM‐531
Authors: Dogné Jean‐Michel , Rolin Stéphanie , de Leval Xavier , Benoit Patricia , Neven Philippe , Delarge Jacques , Kolh Philippe , Damas Jacques , David Jean‐Louis , Masereel Bernard
Published: August 2010

BM‐531 (N‐tert‐butyl‐N'‐[(2‐cyclohexylamino‐5‐nitrobenzene)sulfonyl]urea), a torasemide derivative, is a novel noncarboxylic thromboxane receptor antagonist and thromboxane synthase inhibitor. Indeed, its affinity for human washed platelet TXA2receptors labeled with [3H]SQ‐29548 (IC50= 0.0078 μM) is higher than sulotroban (IC50= 0.93 μM) and SQ‐29548 (IC50= 0.021 μM). Moreover, BM‐531 is characterized by a potent antiaggregatory property. Indeed, on one hand, in human citrated platelet‐rich plasma BM‐531 prevents platelet aggregation induced by arachidonic acid (600 μM) (ED100= 0.125 μM), U‐46619, a stable TXA2agonist (1 μM) (ED50= 0.482 μM) or collagen (1 μg/mL) (percentage of inhibition: 42.9% at 10 μM) and inhibits the second wave of ADP (2 μM)‐induced aggregation. On the other hand, when BM‐531 is incubated in whole blood from healthy donors, the closure time measured by the recently developed platelet function analyser (PFA‐100®) is significantly prolonged. In addition, at the concentrations of 10 and 1 μM, BM‐531 totally prevents the production of TXB2by human platelets activated by arachidonic acid. Finally, at 10 μM, BM‐531 significantly prevents rat fundus contractions induced by U‐46619 but not by prostacyclin. These results suggest that BM‐531, which is devoid of the diuretic property of torasemide, can be regarded as a promising antiplatelet agent.
Ifetroban Sodium: An Effective TxA2/PGH2Receptor Antagonist
Authors: Rosenfeld Louis , Grover Gary J. , Stier Charles T.
Published: August 2010

This review presents a comprehensive discussion on the chemistry, pharmacokinetics, and pharmacodynamics of ifetroban sodium, a new thomboxane A2/prostaglandin H2receptor antagonist. Thromboxane A2is an arachidonic acid product, formed by the enzyme cyclooxygenase. In contrast to other cyclooxygenase products, thromboxane A2has been shown to be involved in vascular contraction and has been implicated in platelet activation. In general, results of clinical studies and animal experiments indicate that hypertension is associated with hyperaggregability of platelets and increased thomboxane A2levels in blood, urine, and tissues. The precursors to thromboxane A2, prostaglandin G2, and prostaglandin H2, also bind and activate the same receptors. Thus, a receptor antagonist was thought to be an improved strategy for reversing the actions of thromboxane A2/prostaglandin H2, rather than a thromboxane synthesis inhibitor. This review describes new methods for the synthesis and analysis of ifetroban, its tissue distribution, and its actions in a variety of animal models and disease states. We describe studies on the mechanisms of how ifetroban relaxes experimentally contracted isolated vascular tissue, and on the effects of ifetroban on myocardial ischemia, hypertension, stroke, thrombosis, and its effects on platelets. These experiments were conducted on several animal models, including dog, ferret, and rat, as well as on humans. Clinical studies are also described. These investigations show that ifetroban sodium is effective at reversing the effects of thromboxane A2– and prostaglandin H2‐mediated processes.
Sotalol: The Mechanism of Its Antiarrhythmic‐Defibrillating Effect
Authors: Manoach Mordechai , Tribulova Narcis
Published: August 2010

This minireview deals with the role of intercellular communication and synchronization in the initiation and maintenance of ventricular fibrillation. It is proposed that myocardial cell junctions might represent a therapeutic substrate for the prevention of this fatal arrhythmia. This hypothesis is supported by the results of recent experimental studies involving elucidation of the mechanism of antiarrhythmic‐defibrillating effects of sotalol. Enhancement of intercellular communication and myocardial synchronization are thought to play critical role in the mechanism of action of this drug.
Eplerenone: A Selective Aldosterone Receptor Antagonist (SARA)
Authors: Delyani John A. , Rocha Ricardo , Cook Chyung S. , Tolbert Dwain S. , Levin Stuart , Roniker Barbara , Workman Diane L. , Sing Yuen‐lung L. , Whelihan Brian
Published: August 2010

Aldosterone, the final product of the renin‐angiotensin‐aldosterone system (RAAS), is a mineralocorticoid hormone that classically acts, via the mineralocorticoid (aldosterone) receptor, on epithelia of the kidneys, colon, and sweat glands to maintain electrolyte homeostasis. Aldosterone has also been shown to act at nonepithelial sites where it can contribute to cardiovascular disease such as hypertension, stroke, malignant nephrosclerosis, cardiac fibrosis, ventricular hypertrophy, and myocardial necrosis. Although angiotensin‐converting enzyme (ACE) inhibitors and angiotensin type 1 (AT1) receptor antagonists act to suppress the RAAS, these agents do not adequately control plasma aldosterone levels — a phenomenon termed “aldosterone synthesis escape.” Spironolactone, a nonselective aldosterone receptor antagonist, is an effective agent to suppress the actions of aldosterone; its use is, however, associated with progestational and antiandrogenic side effects due to its promiscuous binding to other steroid receptors. For these reasons, eplerenone — the first agent of a new class of drugs known as the selective aldosterone receptor antagonists (SARAs) — is under development. In rodent models, eplerenone provides marked protection against vascular injury in the kidney and heart. In phase II clinical trials, eplerenone demonstrates 24‐h control of blood pressure with once or twice daily dosing, and is safe and well tolerated in patients with heart failure when given with standard of care agents. Pharmacokinetic studies reveal that eplerenone has good bioavailability with low protein binding, good plasma exposure, and is highly metabolized to inactive metabolites and excreted principally in the bile. Eplerenone is well tolerated in acute and chronic safety pharmacology studies. Ongoing phase III trials of eplerenone in the treatment of hypertension and heart failure are underway. These studies will extend our understanding of selective aldosterone receptor antagonism in the treatment of chronic cardiovascular disease.
Norbormide: a Calcium Entry Blocker with Selective Vasoconstrictor Activity in Rat Peripheral Arteries
Authors: Bova Sergio , Cima Lorenzo , Golovina Vera , Luciani Sisto , Cargnelli Gabriella
Published: August 2010

Norbormide is a unique vasoactive substance endowed with species‐ and tissue‐specific, endothelium independent, vasoconstrictor activity that is restricted to the peripheral arteries of rat. In rat aorta and in all tested arteries of other species norbormide exhibits vasorelaxant property presumably due to the blockade of calcium channels. A calcium entry blocker effect of norbormide has also been described in isolated, perfused guinea pig hearts. In these preparations norbormide produced coronary vasodilator, as well as negative inotropic and dromotropic effects. In single ventricular myocytes of guinea pigs norbormide reduces L‐type calcium current. The mechanism underlying the selective vasoconstrictor effect of norbormide is unknown. In rat caudal artery, a vessel contracted by norbormide, the drug activates phospholipase C (PLC) signal cascade which is the biochemical pathway involved in the contractile effect triggered by most receptor‐activating vasoactive agents. Therefore, norbormide‐ induced contraction of rat peripheral vessels is likely to be due to the activation of a PLC‐coupled receptor abundantly or selectively expressed in vascular smooth muscle cells. The identification of this putative receptor could facilitate the development of tissue‐selective pharmacological agents.
Cardiovascular Actions of Berberine
Authors: Lau Chi‐Wai , Yao Xiao‐Qiang , Chen Zhen‐Yu , Ko Wing‐Hung , Huang Yu
Published: August 2010

Berberine, is an alkaloid fromHydrastis canadensisL., Chinese herb Huanglian, and many other plants. It is widely used in traditional Chinese medicine as an antimicrobial in the treatment of dysentery and infectious diarrhea. This manuscript describes cardiovascular effects of berberine and its derivatives, tetrahydroberberine and 8‐oxoberberine. Berberine has positive inotropic, negative chronotropic, antiarrhythmic, and vasodilator properties. Both derivatives of berberine have antiarrhythmic activity. Some of cardiovascular effects of berberine and its derivatives are attributed to the blockade of K+channels (delayed rectifier and KATP) and stimulation of Na+‐Ca2+exchanger. Berberine has been shown to prolong the duration of ventricular action potential. Its vasodilator activity has been attributed to multiple cellular mechanisms. The cardiovascular effects of berberine suggest its possible clinical usefulness in the treatment of arrhythmias and/or heart failure.
Cilostazol (Pletal®): A Dual Inhibitor of Cyclic Nucleotide Phosphodiesterase Type 3 and Adenosine Uptake
Authors: Liu Yongge , Shakur Yasmin , Yoshitake Masuhiro , Kambayashi Jun‐ichi
Published: August 2010

Cilostazol (Pletal®), a quinolinone derivative, has been approved in the U.S. for the treatment of symptoms of intermittent claudication (IC) since 1999 and for related indications since 1988 in Japan and other Asian countries. The vasodilatory and antiplatelet actions of cilostazol are due mainly to the inhibition of phosphodiesterase 3 (PDE3) and subsequent elevation of intracellular cAMP levels. Recent preclinical studies have demonstrated that cilostazol also possesses the ability to inhibit adenosine uptake, a property that may distinguish it from other PDE3 inhibitors, such as milrinone. Elevation of interstitial and circulating adenosine levels by cilostazol has been found to potentiate the cAMP‐elevating effect of PDE3 inhibition in platelets and smooth muscle, thereby augmenting anti‐platelet and vasodilatory effects of the drug. In contrast, elevation of interstitial adenosine by cilostazol in the heart has been shown to reduce increases in cAMP caused by the PDE3‐inhibitory action of cilostazol, thus attenuating the cardiotonic effects. Cilostazol has also been reported to inhibit smooth muscle cell proliferationin vitroand has been demonstrated in a clinical study to favorably alter plasma lipids: to decrease triglyceride and to increase HDL‐cholesterol levels. One, or a combination of several of these effects may contribute to the clinical benefits and safety of this drug in IC and other disease conditions secondary to atherosclerosis. In eight double‐blind randomized placebo‐controlled trials, cilostazol significantly increased maximal walking distance, or absolute claudication distance on a treadmill. In addition, cilostazol improved quality of life indices as assessed by patient questionnaire. One large randomized, double‐blinded, placebo‐controlled, multicenter competitor trial demonstrated the superiority of cilostazol over pentoxifylline, the only other drug approved for IC. Cilostazol has been generally well‐tolerated, with the most common adverse events being headache, diarrhea, abnormal stools and dizziness. Studies involving off‐label use of cilostazol for prevention of coronary thrombosis/restenosis and stroke recurrence have also recently been reported.