bluuebunny/crossref_metadata_2025 huggingface dataset:
Processing of sound location in human cortex
Authors: Lewald Jörg , Riederer Klaus A. J. , Lentz Tobias , Meister Ingo G.
Published: March 2008

This functional magnetic resonance imaging study was focused on the neural substrates underlying human auditory space perception. In order to present natural‐like sound locations to the subjects, acoustic stimuli convolved with individual head‐related transfer functions were used. Activation foci, as revealed by analyses of contrasts and interactions between sound locations, formed a complex network, including anterior and posterior regions of temporal lobe, posterior parietal cortex, dorsolateral prefrontal cortex and inferior frontal cortex. The distinct topography of this network was the result of different patterns of activation and deactivation, depending on sound location, in the respective voxels. These patterns suggested different levels of complexity in processing of auditory spatial information, starting with simple left/right discrimination in the regions surrounding the primary auditory cortex, while the integration of information on hemispace and eccentricity of sound may take place at later stages. Activations were identified as being located in regions assigned to both the dorsal and ventral auditory cortical streams, that are assumed to be preferably concerned with analysis of spatial and non‐spatial sound features, respectively. The finding of activations also in the ventral stream could, on the one hand, reflect the well‐known functional duality of auditory spectral analysis, that is, the concurrent extraction of information based on location (due to the spectrotemporal distortions caused by head and pinnae) and spectral characteristics of a sound source. On the other hand, this result may suggest the existence of shared neural networks, performing analyses of auditory ‘higher‐order’ cues for both localization and identification of sound sources.
Noradrenergic modulation in the olfactory bulb influences spontaneous and reward‐motivated discrimination, but not the formation of habituation memory
Authors: Mandairon Nathalie , Peace Shane , Karnow Alexandra , Kim Jane , Ennis Matthew , Linster Christiane
Published: March 2008

The mammalian main olfactory bulb receives a significant noradrenergic input from the locus coeruleus. Norepinephrine is involved in acquisition of conditioned odor preferences in neonatal animals and in some species‐specific odor dependent behaviors. Thus far, the role of norepinephrine in odor processing in adult rats remains less studied. We tested the role of noradrenergic modulation in the olfactory bulb of cannulated rats by bilateral injections of vehicle (6 µL saline), the α noradrenergic receptor antagonist phentolamine (3.15 or 10 mm), the β noradrenergic receptor antagonist alprenolol (12 or 120 mm), the α1 noradrenergic receptor antagonist prazosin (1 or 10−2 mm) and the α2 noradrenergic receptor antagonist yohimbine (2 or 0.02 mm) 20 min before two different behavioral tasks. We found that local blockade of noradrenergic receptors in the olfactory bulb did not affect the formation of habituation memory to an odorant over sequential presentations separated by 5‐min intertrial intervals. However, spontaneous discrimination between chemically related odorants was impaired when noradrenergic receptors, and in particular α1 receptors, were blocked by local antagonist infusion into the olfactory bulb. By contrast, discrimination was improved when β receptors were blocked. These results show that although the formation of a habituation memory to odorants is not affected by noradrenergic modulation, the specificity of this memory is affected. In contrast, reward‐motivated discrimination learning was not impaired, but slowed down in rats in which both α and β receptors had been blocked.
Characterizing the regulation of the Pu promoter in Acinetobacter baylyi ADP1
Authors: Huang Wei E. , Singer Andrew C. , Spiers Andrew J. , Preston Gail M. , Whiteley Andrew S.
Published: March 2008

Effective gene trapping and screening requires sensory and regulatory compatibility of both host and exogenous systems. The naturally competent bacterium Acinetobacter baylyi ADP1 is able to efficiently take up and integrate exogenous DNA into the chromosome, making it an attractive host system for a wide range of metagenomic applications. To test the ability of A. baylyi ADP1 to express the XylR‐regulated Pu promoter from Pseudomonas putida mt‐2, we have constructed and examined an A. baylyi ADP1 strain, ADPWH‐Pu‐lux‐xylR. The Pu promoter in ADPWH‐Pu‐lux‐xylR was specifically induced by toluene, m‐, p‐ and o‐xylene. The substrate‐induced Pu promoter was highly dependent on the growth medium: it was repressed in rich media until stationary phase, but was immediately induced in minimal medium with glucose as the sole carbon source (MMG). However, the Pu promoter was repressed in MMG when it was supplemented with 5 g l−1 yeast extract. Further investigation showed that the Pu promoter in MMG was repressed by 0.5 g l−1 aspartic acid or asparagine, but not repressed by glutamine. Changing the carbon/nitrogen ratios by addition of ammonia did not significantly affect the Pu promoter activity but addition of nitrate did. These results show that A. baylyi ADP1 reproduced characteristics of the XylR‐regulated Pu promoter observed in its original host. It demonstrates that A. baylyi could provide an excellent genetic host for a wide range of functional metagenomic applications.
Pricing‐to‐Market and Market Structure*
Authors: Bugamelli Matteo , Tedeschi Roberto
Published: March 2008

Stimulated by imperfect competition/sticky prices framework of the new open economy macroeconomics, empirical research has reconsidered the role of exchange rates in international adjustment. This paper reassesses the link between exchange rates and traded good prices by estimating pricing‐to‐market equations for the five main euro area countries over the period 1990–99. We minimize selection biases by keeping all manufacturing products and all destination markets and show that exchange rate pass‐through (ERPT) is much larger, almost complete, than previously estimated. Thanks to a huge variability in terms of exchange rate variations, products and destination markets, we can map differences in ERPT into market structures and, at the same time, reconcile our results with the empirical literature. We find that ERPT is highly incomplete for sales by oligopolistic industries into advanced economies, indeed in the order of 50–60% as previously estimated. ERPT is instead almost complete in emerging and developing economies where therefore exchange rate movements can help adjust external imbalances. We also find that ERPT is largely asymmetric: it is almost complete after an appreciation of the exporter's currency, rather incomplete after a depreciation. This result is very robust across specifications.
Verbal ability and delinquency: testing the moderating role of psychopathic traits
Authors: Muñoz Luna C. , Frick Paul J. , Kimonis Eva R. , Aucoin Katherine J.
Published: March 2008

Background:  Impaired verbal abilities are one of the most consistent risk factors for serious antisocial and delinquent behavior. However, individuals with psychopathic traits often show serious antisocial behavior, despite showing no impairment in their verbal abilities. Thus, the aim of the current study was to examine whether psychopathy moderates the relationship between verbal abilities and delinquent behavior in a sample of detained youth.

Methods:  The sample included 100 detained adolescent boys who were assessed on self‐reported delinquent acts and psychopathic traits, as well as their age at first offense based on official records. Participants also completed a competitive computer task involving two levels of provocation, during which skin conductance was measured. A standard measure of receptive vocabulary was individually administered.

Results:  As predicted, there was a significant interaction between callous‐unemotional (CU) traits (a critical dimension of psychopathy) and verbal ability when predicting violent delinquency. Individuals who were high on CU traits with higher scores on the measure of verbal abilities reported the greatest violent delinquency. These individuals also showed the lowest level of skin conductance reactivity during the provocation task.

Conclusions:  The results suggest CU traits are an important moderator of the relation between verbal abilities and violent delinquency.
Peer victimization as a predictor of depression and body mass index in obese and non‐obese adolescents
Authors: Adams Ryan E. , Bukowski William M.
Published: March 2008

Background:  The current study examined the pathway from peer victimization to depressive symptoms and body mass index (BMI) as mediated by self‐concept for physical appearance in both obese and non‐obese adolescents. It was thought that this pathway would be particularly important for obese adolescents because, compared to non‐obese adolescents, they are at risk for being victimized and because the victimization would be more likely to lead to lower self‐concept.

Method:  Utilizing data from the National Longitudinal Survey of Children and Youth, the current study examined self‐reports of peer victimization, self‐concept for physical appearance, depressive symptoms, height, and weight in 1,287 adolescents at three time periods over four years starting when the participants were between the ages of 12 and 13.

Results:  For non‐obese adolescents, victimization did not predict changes in depressive symptoms and body mass index (BMI) four years later. For obese females, the mediated pathway was found from victimization to self‐concept to both depressive symptoms and increases in BMI. For obese males, the findings were more complicated. In this group, the mediated pathway was found from victimization to self‐concept to decreases in BMI, but a mediated pathway was not found for depressive symptoms.

Conclusions:  The current study suggests that a risk‐factor for being victimized, such as obesity, may play an important role in the long‐term effects of victimization by making it more likely that the adolescent will be victimized over the long term but also that victimization can reinforce the negative self‐perceptions that the adolescent already has. It is important to go beyond using obesity as a predictor of long‐term adjustment and examine the processes and experiences of obese individuals that might more directly cause depression or changes in health.
Research Review: The relation between child and parent anxiety and parental control: a meta‐analytic review
Authors: Van Der Bruggen Corine O. , Stams Geert Jan J.M. , Bögels Susan M.
Published: March 2008

Background:  There is growing research interest in the association between parental control and child anxiety. Parental control may enhance child anxiety and parents may exert control in anticipation of their child’s anxiety‐related distress. Moreover, high levels of anxiety in parents could influence the development of parental control. Whereas past reviews have solely examined the relation between child anxiety and parental control, this meta‐analysis focuses on the associations between both child and parent anxiety and parental control.

Methods:  The associations of parent anxiety and child anxiety with observed parental control (k = 23 studies, N = 1,305 parent–child dyads) were investigated using a meta‐analytic approach. Moreover, factors were identified that may function as moderators of these relations, such as parent and child gender, family socioeconomic status, child age, and design and measurement characteristics.

Results:  A substantial association between child anxiety and parental control (d = .58) was found. Moderator analyses yielded the strongest effect sizes for studies with an overrepresentation of girls, for school‐aged children, for families from higher socioeconomic backgrounds, and for studies using a discussion task to assess parental control. Although a nonsignificant relation was found for the relation between parent anxiety and parental control (d = .08), small but significant effects were found for school‐aged children, for studies using a discussion task to assess parental control, and for samples with an overrepresentation of boys.

Conclusions:  As the direction of the association between child anxiety and parental control is unknown, future studies should use experimental designs to further explore the causal link between child anxiety and parental control.
Na+/H+ exchanger inhibition modifies dopamine neurotransmission during normal and metabolic stress conditions
Authors: Rocha Marcelo A. , Crockett David P. , Wong Lai‐Yoong , Richardson Jason R. , Sonsalla Patricia K.
Published: March 2008

Na+/H+ exchanger (NHE) proteins are involved in intracellular pH and volume regulation and may indirectly influence neurotransmission. The abundant NHE isoform 1 (NHE1) has also been linked to brain cell damage during metabolic stress. It is not known, however, whether NHE1 or other NHE isoforms play a role in striatal dopamine (DA) neurotransmission under normal or metabolic stress conditions. Our study tested the hypothesis that NHE inhibition with cariporide mesilate (HOE‐642) modifies striatal DA overflow and DAergic terminal damage in mice caused by the mitochondrial inhibitor malonate. We also explored the expression of NHE1–5 in the striatum and substantia nigra. Reverse microdialysis of HOE‐642 elicited a transient elevation followed by a reduction in DA overflow accompanied by a decline in striatal DA content. HOE‐642 pre‐treatment diminished the malonate‐induced DA overflow without reducing the intensity of the metabolic stress or subsequent DAergic axonal damage. Although NHE isoforms 1–5 are expressed in the striatum and midbrain, NHE1 protein was not co‐located on nigrostriatal DAergic neurons. The absence of NHE1 co‐location on DAergic neurons suggests that the effects of HOE‐642 on striatal DA overflow are either mediated via NHE1 located on other cell types or that HOE‐642 is acting through multiple NHE isoforms.
The Ichthyoses—Pathogenesis and Prenatal Diagnosis: A Review of Recent Advances
Authors: Williams Mary L.
Published: March 2008

Abstract:Disturbances in the process of normal cornification leading to pathologic scaling provide the pathophysiologic basis for the ichthyoses. These disturbances may result from either abnormalities in protein metabolism (keratinization) (i.e., the “bricks”) or in lipid metabolism (i.e., the “mortar”) (Fig. 1). The evidence linking the various ichthyoses to defects in protein or lipid metabolism have been reviewed. It is likely that future advances will lead not only to a more complete understanding of the pathogenesis of these disorders, but also will shed significant light on the normal stratum corneum functions of barrier formation and desquamation, as well as lead the way to more rational and effective therapies. In recent years, prenatal diagnosis has been successfully performed in several of the ichthyoses. It is likely that improvements in our ability to prenatally diagnose those disorders will advance hand‐in‐hand with further progress in unraveling their underlying causes.
The Pathogenesis, Diagnosis, and Treatment of Histiocytosis Syndromes
Authors: Lipton Jeffrey M.
Published: March 2008

Abstract: The histiocytosis syndromes previously known, and often still referred to, as histiocytosis‐X were originally categorized by many as malignant neoplasms. They have been treated as such with aggressive chemotherapy and radiation therapy. Although these modalities are still used, there are significant differences between the histiocytosis syndromes and true malignant disease that suggest conservative management for many patients. The clinical course of malignant neoplasia is relentlessly progressive with virtually no survival in untreated patients. The histiocytosis syndromes are characterized by frequent spontaneous remissions and exacerbations, with varying morbidity and survival in untreated patients, depending on the extent of the disease. Pathologically, the lesions of histiocytosis appear as reactive infiltrates, possessing little of the cellular atypicality and homogeneity characteristic of malignancy. Although the etiology of these phenomena is unknown, histiocytosis syndromes appear to represent a reactive autoimmune disorder triggered by unknown stimuli. (1–3)
Chronic Blepharitis and Pyoderma of the Scalp: An Immune Deficiency State in a Father and Son with Hypercupremia and Decreased Intracellular Killing
Authors: Wheeland Ronald G. , Thurmond Roger D. , Gilmore William A. , Blackstock Rebecca
Published: March 2008

Abstract: An immune deficiency state is proposed as the cause of a disorder affecting a father and son with chronic dermatitis, purulent blepharitis with corneal ulceration, and scarring pyodermatous alopecia of the scalp. The results of immunologic investigation revealed abnormal neutrophil function with a variable decrease in intracellular killing, decreased lymphocyte transformation, increased serum IgG and IgE, and elevated serum copper levels. These findings will be compared with previously described immune deficiency disorders.
Progressive Cardiomyopathic Lentiginosis: Report of Six Cases and One Autopsy
Authors: Ruiz‐Maldonado Ramon , Trevizo Lourdes , Tamayo Lourdes , de los Rios Miguel F. , Skurovich Mario , Carrillo Joaquin , Dominguez Dagoberto , del Castillo Victoria
Published: March 2008

Abstract: Progressive Cardiomyopathic lentiginosis (PCL) is a syndrome with progressive multisystem abnormalities. Lentigines, the cutaneous hallmark, are usually present from early age and are of utmost diagnostic value.

Six children with PCL arc reported. The most constant diagnostic features in this age group were multiple cutaneous lentigines, structural cardiac anomalies, and electrocardiographic alterations. Less constant findings were neurosensory deafness, short stature, and osseous, ocular, and genitourinary abnormalities.
Resistant Childhood Psoriasis: An Analysis of Patients Seen in a Day‐care Center
Authors: Menter M. Alan , Whiting David A. , McWilliams Joyce
Published: March 2008

Abstract: During a three‐and‐one‐half‐year period, 54 children aged 1 to 16 years, all of whom had resistant psoriasis, were seen at a day‐care center. The female: male ratio was 3:2. In 74% of the patients, the onset of psoriasis occurred when they were below 10 years of age. Papules and plaques occurred in 78% of cases, guttate lesions in 16%, and nail involvement in 21%. A family history of psoriasis was present in 69% of cases, preceding upper respiratory tract infections occurred in 35%, and prior cradle cap and/or diaper rash in 50%. In the day‐care center, Goeckerman therapy was given to 31 children. The average duration of treatment was 12 days, producing maximum clearing of over 90% in 64% of patients, with substantial improvement in the remainder. After three months, the psoriasis in 83% of these patients was over 90% clear; 43% showed maximum clearing at one year. Two patients were treated with anthralin and ultraviolet light alone, one with good results. One patient with nail psoriasis alone was not treated. The remaining 20 patients were either unable to attend for treatment at the center or had less extensive psoriasis. Home treatment was prescribed for them, with less satisfactory results.
Cicatricial Pemphigoid in a 6‐Year‐Old Child: Report of a Case and Review of the Literature
Authors: Rosenbaum Marjorie Mintz , Esterly Nancy Burton , Greenwald Mark J. , Gerson Carol Roberts
Published: March 2008

Abstract: Cicatricial pemphigoid has been documented previously in only four patients under the age of 20 years. We report a 6‐year‐old male who had erosions of the oral and genital mucosa, conjunctivitis, hoarseness, dysphagia, recurrent vomiting, and weight loss. Upper airway obstruction due to a chronically inflamed and scarred epiglottis necessitated tracheostomy. Biopsy of tissue from a solitary cutaneous lesion demonstrated a subepidennal bulla. Direct and indirect immunofluorescence confirmed a diagnosis of cicatricial pemphigoid. Therapy was begun consisting of prednisone, 2 mg per kg per day, in combination with dapsone, 2 mg per kg per day. He did not improve until the prednisone dosage was increased to 4 mg per kg per day.
Multiple Pilomatricomas (Calcifying Epitheliomas of Malherbe)
Authors: Schlechter Robert , Hartsough Nicole A. , Guttman Frank M.
Published: March 2008

Abstract: Three patients with multiple pilomatricoma were seen. One had 10 nodules occurring sporadically over a four‐year period. This is the greatest number of lesions reported in a single individual. Discussion of all three cases and a review of the literature are included.
Immunopathologic Demonstration of T Lymphocyte Subpopulations and Interleukin 2 in Graneloma Annulare
Authors: Modlin Robert L. , Horwitz David A. , Jordan Robert R. , Gebhard John F. , Taylor Clive R. , Rea Thomas H.
Published: March 2008

Abstract: Immunopathologic aspects of granuloma annulare were studied in frozen sections of nine skin biopsy specimens with monoclonal antibodies directed against T lymphocytes, Langerhans'cells, interleukin 2, and interleukin 2 receptors in conjunction with immunoperoxidase techniques. The predominant lymphocyte was an activated T lymphocyte (Leu 1+, HLA‐DR−) with an excess of helper/inducer phenotype (Leu 3a+) as compared with suppressor/cytotoxic phenotype (Leu 2a+). Langerhans'cells were increased in the epidermis and numerous 0KT6+ cells were observed in the perivascular and granulomatous infiltrate. Both interleukin 2‐positive cells and interleukin 2 receptor‐positive cells were identified in the dermal lesions according to observed reactivity with the corresponding monoclonal antibodies. These findings suggest that a cell‐mediated immune response producing cytokines may be important in the pathogenesis of granuloma annulare. Comparison of theese results with skin specimens from patients with sarcoidosis and from a patient with granuloma annulare having some of the histologic features of sarcoidosis, suggests that the cutaneous infiltrate in granuloma annulare represents a response distinct from that of sarcoidosis.
Green Foot
Authors: LeFeber William P. , Golitz Loren E.
Published: March 2008

Abstract: Pseudomonas aeruginosa may infect the skin surface, nails, hair follicles, or deeper tissues. We report a 13‐year‐old male with an asymptomatic green discoloration of the toenails and sole of the right foot. Pseudomonas aeruginosa was cultured from the shoe, but not from the discolored skin. We stispect that constant wearing of occlusive, rubber‐soled, basketball shoes associated with hyperhidrosis allowed colonization of his shoe with pseudomonas. This case is unique in that colonization resulted in a green color of the foot not associated with infection of the skin.
Woolly‐Hair Nevus: A Case Report and Study by Scanning Electron Microscopy
Authors: Goldin Harry M. , Bronson Darryl M. , Fretzin David F.
Published: March 2008

Abstract: Woolly‐hair is a congenital defect that produces a localized patch of lightly colored, curly scalp hair. Scanning electron microscopy of the abnormal hairs demonstrates twisting of the hair shaft and abnormal cuticle formation.
Disseminated Cutaneous Nocardia Brasiliensis Infection
Authors: Cailen Jeffrey P. , Kingman John
Published: March 2008

Abstract: Nocardia brasiliensis is an uncommon pathogen in the United States despite its presence in soil samples. It is most frequently seen in patients who are immunosuppressed, but can occur in the immunocompetent host also. Usually in a healthy host the infection is localized to one area of the body. Disseminated infection was diagnosed in a healthy child who recovered fully after initiation of oral sulfonamide therapy. We suspect that the source was soil to which the patient was exposed during a family camping trip.

Nocardia asteroides is the most frequent cause of nocardial infections in North America (1). These infections are unusual in healthy individuals, occurring more often in immunologically compromised hosts (1). They are usually acquired by a pulmonary route and may disseminate widely, most commonly to the central nervous system and skin. Nocardia brasiliensis, when it does occur in the United States, usually causes a chronic cutaneous and subcutaneous infection manifesting itself as mycetoma, abscess, or ulcer (2). We saw acute suppurative lymphocutaneous Nocardia brasiliensis infection in an otherwise healthy 2‐year‐old boy.
Necrotizing Fasciitis in Infancy: Report of Three Cases and Review of the Literature
Authors: Goldberg G. N. , Hansen R. C. , Lynch P. J.
Published: March 2008

Abstract: Necrotizing fasciitis exists on a continuum of soft tissue infections between ceUuiitis and myonecrosis. It has been uncommonly reported in infancy (1–10). The diagnosis depends on recognition of the characteristic rapidly progressive clinical course and demonstration of fUll‐thickness skin necrosis with involvement of the fascial planes overlying the muscle. The organisms most often recovered are beta‐hemolytic Streptococcus or Staphylococcus aureus, but other aerobic and anaerobic organisms in single or mixed infection have heen reported (1, 2, 11, 12). This infection has a significant mortality rate, necessitating early, aggressive, specific medical and surgical treatment.
Pediculosis and the Pediatrician
Authors: Rasmussen James E.
Published: March 2008

Abstract: Head lice commonly evoke feelings of disgust, revulsion, anger, and shame among parents and patients. There should, however, be no great cause for such alarm if a physician suspects pediculosis capitis. The recent introduction of several new pediculicidal drugs now allows a choice among four distinct therapeutic agents, which should substantially improve control of isolated cases and epidemics. Physicians must be aware that consumer groups are pressing public health authorities and drug manufacturers to establish proper treatment standards and safety warnings for the use of these agents. In addition, some controversy surrounds the use of lindane in children. This paper reviews the epidemiology and clinical appearance of pediculosis capitis in children, with emphasis on these recent developments. Pubic lice (Phthirus pubis) and body lice (Pedicutus humanus corporis), both of which are much less common pediatric infestations, are mentioned only briefly.
Epidermolysis Bullosa: A Review
Authors: Cooper Thomas W. , Bauer Eugene A.
Published: March 2008

Abstract:Epidermolysis bullosa is a group of inherited disorders characterized by blistering of the skin as a result of minor trauma. The disease can be divided into three anatomical categories: Epidermolytic, where blister cleavage occurs within the epidermis; functional, which has blister cleavage within the lamina lucida; and Dermolytic, where blister cleavage occurs below the basal lamina in the upper papillary dermis. Each of these three categories can be divided into several distinct entities based on clinical and histologic criteria. Basic biochemical studies have increased our understanding of several of these diseases, most notably recessive dystrophic epidermolysis bullosa. Although therapy for patients with EB is largely supportive, increased knowledge of the biochemistry of these disorders is making direct therapeutic interventions possible.
Systemic Glucocorticosteroid Therapy of Skin Disease in Children
Authors: Fritz Karen A. , Weston William L.
Published: March 2008

The role of systemic glucocorticosteroid therapy in the managetnent of dermatologic disorders in children is limited. Most skin diseases requiring the antiinflammatory or antiproliferative effects of steroids are best managed with topical preparations, because they exert local effects almost exclusively and cause few if any systemic side effects when used properly. There are, however, certain skin diseases, which because of their severity or their intrinsic nature, do not respond adequately to these agents. We propose the indications for pharmacologic doses of systemic glucocorticosteroids in dermatologic disease, the preferred route of administration, the most common as well as the more rare side effects of this therapy. Withdrawal of patients from chronic use of these drugs is also discussed.
Dyslipoproteinemia and Xanthomatosis
Authors: Haber Craig , Kwiterovich Peter O.
Published: March 2008

Abstract: The cutaneous markers associated with dyslipoproteinemia are reviewed in the context of the current view of lipid and lipoprotein metabolism. The utility of determining the plasma levels of lipoprotcins and certain apoproteins in children or adults with xanthomas or xanthelasma is discussed. We hope that early identification and appropriate treatment of such patients will reduce the morbidity and mortality from the two major complications of dyslipoproteinemia—atherosclerotic cardiovascular disease and pancreatitis.
Psoriatic Arthritis in Children
Authors: Perlman Susan G.
Published: March 2008

Abstract: This paper reviews the typical features of psoriatic arthritis, with emphasis on the clinical manifestations in children. The concepts of seronegative spondyloarthropathy and enthesopathy are described. Recent information is presented on genetics, possible pathophysiologic mechanisms, and treatment.
Melanoma Masquerading as Spitz Nevus Following Acute Lymphoblastic Leukemia
Authors: Goldes Jeffry , Holmes Spencer , Satz Mark , Cich John , Dehner Louis
Published: March 2008

Abstract: A malignant melanoma originally diagnosed as a Spitz nevus led to the death of a 10‐year‐old boy. The melanoma developed four years after therapy was begun for acute lymphoblastic leukemia. Melanomas in children are rare. Melanomas histologically resembling Spitz ncvi have been reported. Deep contiguous growth and melanization are suspicious features. Lymphoproliferative malignancies are most commonly reported to occur in patients surviving treatment for acute lymphoblastic leukemia. Melanoma following acute lymphoblastic leukemia has not been described previously.
Transient Zinc Deficiency in a Full‐Term Breast‐Fed Infant of Normal Birth Weight
Authors: Bye A.M.E. , Goodfellow A. , Atherton D.J.
Published: March 2008

Abstract: A full‐term male infant was seen at age 5 months with symptomatic zinc deficienty. He was breast fed and the mother's milk zinc levels were low. The infant responded to oral zinc suppliments and has continued to be asymptomatic for 12 months after their withdrawal. This is the first report of transient zinc deficiency in an otherwise healthy, breast‐fed, full‐term infant of normal birth weight.
Aplasia Cutis Congenita: A Report of 12 New Families and Review of the Literature
Authors: Sybert Virginia P.
Published: March 2008

Abstract: Aplasia cutis congenita (ACC) is a heterogeneous group of disorders whose common characteristic is focal absence of skin. In the majority of instances this is limited to the scalp, although other areas of the body may also be involved. Other congenital malformations have been reported to occur with AAC; limb decects appear to be a specific association. Given our experience with AAC, we suggest a classification based on genetically distinct entities. Type I AAC is limited to the scalp. Type II involves body or scalp; IIA invoves body or limb defects. Type III is limited to the scalp or limbs. Type IV is associated with epidermolysis bullosa; type IVA is Bart syndrome. Although most reported cases have been sporasdic, there are may familial occurrences of all types of ACC. Most published pedigrees are consistent with authosomal dominant inheritance with reduced penetrance, or autosomal recessive inheritance. Careful examination of family member of affected individuals is warranted.
Inflammatory Linear Verrucous Epidermal Nevus: Report of Seven New Cases and Review of the Literature
Authors: Morag C. , Metzker Arie
Published: March 2008

Abstract: In seven patients with inflammatory linear verrucous epithelial nevus (ILVEN) examined, the condition demonstrated the typical clinical and histologic picture. Based on this experience, we suggest revised criteria for the disease. Dermatologists and pediatricians aware of ILVEN can easily differentiate it from similar lesions and thus be able to suggest effective therapy.
Cutaneous Histiocytosis Syndromes
Authors: Roper Susan S. , Spraker Mary K.
Published: March 2008

Abstract: Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self‐healing histiocytosis X, the Letterer‐Siwe‐like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xantho‐granuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.
Cutaneous Manifestations of Diabetes Mellitus in Children
Authors: Edidin Deborah V.
Published: March 2008

Abstract: This article reviews skin changes occurring in diabetic children. Skin changes may be related to metabolic alterations associated with diabetes. Others may be manifestations of macro or microvascular disease. Insulin injections, required for management of most diabetes in the pediatric age group, may also cause cutaneous changes. There remain several cutaneous abnormalities which are observed with increased frequency among diabetics, the significance of which is unknown. The skin is a potentially invaluable tool for understanding certain diabetic omplications: however, the value of observations and experimental data relating to cutaneous changes in diabetics depends upon documentation of the type of diabetes mellitus employing the currently accepted classification system.
Skin Surface Stripping in Diagnosing and Monitoring Inflammatory, Xerotic, and Neoplastic Diseases
Authors: Pierard‐Franchimont Claudine , Pierard Gérald E.
Published: March 2008

Abstract: Skin surface stripping is a useful and simple diagnostic tool for the diagnosis of a number of diseases that affect the skin during childhood and adolescence. The procedure has proved to be especially valuable in inflammatory, xerotic, and neoplastic conditions as it provides information regarding pathologic changes as well as diagnosis.
Verrucous Hemangioma
Authors: Klein Jeffrey A. , Barr Ronald J.
Published: March 2008

Abstract: Two patients with verrucous hemangioma, a congenital vascular malformation, were seen. The typical lesion is a unilateral group of hyperkeratotic papules and noduies on a lower extremity. Unlike the more common capillary or cavernous hemangiomas, verrucous hemangiomas tend to enlarge and spread with time, and to recur after excision.
Processioeary Caterpillar Dermatitis
Authors: Garty Ben Zion , Danon Yehuda L.
Published: March 2008

Abstract: This work is the first description of processionary caterpillar dermatitis in pediatric patients. Seven children were exposed to caterpillar spines of the genus Thamatopoea Wilkinson; Tams, a pine tree caterpillar that is the main causative agent of caterpillar dermatitis in Israel. In all seven patients avoidance of re‐exposure caused the symptoms to disappear within a few days. This disease should be considered in the differential diagnosis of dermatitis in children in endermic zones.
Toxic Epidermal Necrolysis in a 6‐week‐old Infant
Authors: Hawk Roberta J. , Storer James S. , Daum Robert S.
Published: March 2008

Abstract: Toxic epidermal necrolysis was documented in a 6‐week‐old infant with Klebsiella pneumoniae sepsis who received many medications. We inoculated infant mice with the K. pneumoniae isolate but were unable to produce histologic changes resembling those seen in our patient. This condition should be included in the differential diagnosis of severe drug reactions in very young infants with clinical scalded‐skin syndromes.
Dermatologic Uses of Methotrexate in Children: Indications and Guidelines
Authors: Paller Amy S.
Published: March 2008

Abstract: Although methotrexate is frequently employed as an antimetabolic chemotherapeutic agent for childhood cancer, it is rarely used for dermatologic disorders in children. In the pediatrie patient whose skin disease is recalcitrant to more conservative treatment, a trial of methotrexate is often appropriate. There are few reports of the drug's use for skin disorders in childhood upon which to base rational therapeutic management, however. In setting guidelines for use in children, we must consider pharmacokinetic studies and methotrexate toxicity observed in managing pediatric cancer.
The Effects of Wearing Diapers on Skin
Authors: Zimmerer R. E. , Lawson K. D. , Calvert C. J.
Published: March 2008

Abstract: Wearing dry and wet cloth and disposable diaper materials has certain effects on the degree of skin wetness. These, in turn, affect the coefficient of skin friction, the skin's susceptibility to abrasion damage, its permeability, and its support of microbial growth. These effects were explored using an adult model wearing forearm patches. The adult model was validated by comparisons of skin wetness and friction values for infants and adults determined under similar conditions. Skin wetness was proportional to diaper wetness. With increased skin wetness, there were increased coefficients of friction and increased abrasion damage, skin permeability, and microbial growth. Cloth diaper material produced wetter skin than did disposable diaper material at equivalent loadings.
Etiologic Factors in Diaper Dermatitis: The Role of Urine
Authors: Berg Ronald W. , Buckingham Kent W. , Stewart Robert L.
Published: March 2008

Abstract: Diaper dermatitis may result after repeated or prolonged contact of skin with urine and feces. A hairless mouse model was used to elucidate the role of urine in this process. The results of this work suggest that an important function of urine in the etiology of diaper dermatitis is to increase the pH of the diaper environment by breaking down urea in the presence of fecal urease. This rise in pH increases the activities of fecal proteases and lipases, which can damage skin. Urine can also increase the permeability of diapered skin to irritants and can directly irritate skin when exposure is prolonged.
Etiologic Factors in Diaper Dermatitis: The Role of Feces
Authors: Buckingham Kent W. , Berg Ronald W.
Published: March 2008

Abstract: While the etiology of diaper dermatitis in infants is complex, it is generally believed to involve an interaction between skin and irritants in feces and urine. Proteases and lipases were identified as the major irritants in the feces of infants, and bile salts were found to potentiate the damage produced by the action of fecal enzymes on skin. Fecal enzymes also increased the permeability of skin, thereby increasing potential susceptibility to other irritants in the diaper environment.
Bart's Syndrome: Microscopic, Ultrastructural, and Immunofluorescent Mapping Features
Authors: Butler David F. , Berger Timothy G. , James William D. , Smith T. Larry , Stanley John R. , Rodman Orlando G.
Published: March 2008

Abstract: Bart's syndrome has been clinically described as the association of congenital localized absence of skin (CLAS), epidermolysis bullosa (EB), oral mucosal lesions, and dystrophic nails. Transmission occurs through an auto‐somal dominant gene with complete penetrance but variable expression. It has been difficult to classify this type of EB because of lack of microscopic and ultrastructural studies on affected family members. This is the first report of microscopic, ultrastructural, and immunofluorescent mapping studies of an affected individual with the complete inherited syndrome initially described by Bart. This study is also the first to document the association of CLAS and dominant dystrophic epidermolysis bullosa by histology, electron microscopy, and immunofluorescent mapping. Our two patients and one other affected family member had diminution or absence of a specific basement membrane antigen as defined by immunofluorescence with a monoclonal antibody (KF‐1) in perilesional skin.
Waardenburg's Syndrome with Hirschsprung's Disease: A Neural Crest Defect
Authors: Mallory Susan Bayliss , Wiener Eugene , Nordiund James J.
Published: March 2008

Abstract:Waardenburg's syndrome and Hirschsprung's disease are both conditions involving a defect of migrating neural crest cells and may be seen together. We report a patient with the combination of these two diseases and review the literature on the subject. Physicians should be aware that deafness can be associated with pigmentary anomalies.
Protein‐Losing Enteropathy in Atopic Dermatitis
Authors: Jenkins Huw R. , Walker‐Smith John A. , Atherton David J.
Published: March 2008

Abstract: We evaluated a child with atopic dermatitis associated with protein‐losing enteropathy, which responded to a chicken‐based diet. Allergic gastroenteropathy is a potentially hazardous disease that may be more frequent in children with atopic dermatitis than is currently realized.
Diaper Dermatitis: Frequency and Severity Among a General Infant Population
Authors: Jordan W. E. , Lawson K. D. , Berg R. W. , Franxman J. J. , Marrer A. M.
Published: March 2008

Abstract: The frequency and severity of diaper dermatitis was measured among a midwestem suburban population of 1089 infants ranging in age from 1 to 20 months. No diagnosis of specific etiology was made. Fecal samples were collected and analyzed for Candida albicans, and information on family characteristics, infant diet, general health, history of rash, and diapering habits and practices was collected by questionnaire. The distribution of the severity of observed diaper rash can be described as a logarithmic‐normal function, implying several multiplicative causative factors. Within the total severity range, there appear to be three subcategories of diaper rash, differing in some manner, perhaps reflecting different etiologies. The frequency of observed diaper rash was a function of the maturity of the infant, reaching a maximum around 9 to 12 months of age. The prevalence of severe rash correlated with the presence and level of fecal C. albicans. Infants diapered exclusively in disposable diapers showed less rash (P<0.001) than those diapered exclusively or sometimes in cloth diapers.
Multiple Hamartomas Associated with Intracranial Malformation
Authors: Mimouni Francis , Han Bokyung K. , Barnes Louise , Ballard Jeanne L. , Dignan Peter S. , Kiessling Mary A. , Lucky Anne W.
Published: March 2008

Abstract: We examined a newborn infant with multiple hamartomas, including an epidermal nevus syndrome and a giant pigmented congenital nevocellular nevus, associated with other structural developmental abnormalities such as nevus flammeus, vascular malformation, cutis aplasia congenita of the scalp, cartilage hamartoma, and a lipodermoid of the conjunctiva. This child had a significant brain malformation, diagnosed by sonography and computerized tomography, consisting of a significant enlargement of the left hemisphere not associated with asymmetry of the skull or facial bones. We suggest a careful investigation of the intracranial structures by computerized tomography and/or ultrasonography in case of either extensive linear nevus sebaceous sequence and/or giant pigmented nevocellular nevus.
Embryogenesis of the Dermis in Human Skin
Authors: Smith Lynne T. , Holbrook Karen A.
Published: March 2008

Abstract: The process of human dermal development has been examined from the stage of the embryonic mesenchymal dermis (5–8 weeks of gestation) through the formation of the fibrous dermal connective (end of the first trimester), and during the growth of the fetal dermis (second and third trimesters) until birth. The elaboration of dermal organization and the overall increase in dermal thickness, largely consequences of extracellular, fibrous matrix accumulation, parallel the development and growth of the fetus as a whole. Although the mechanisms that determine the formation of the connective tissue architecture are not fully understood, the structural, histochemical, and biochemical changes that occur throughout development suggest processes that may be important in human dermal embryogenesis.
The Association of Aplasia Cutis Congenita with Therapy of Maternal Thyroid Disease
Authors: Kalb Robert E. , Grossman Marc E.
Published: March 2008

Abstract: Aplasia cutis congenita, the localized absence of skin at birth, usually is an isolated scalp defect. We examined an infant with aplasia cutis congenita associated with maternal Grave's disease and the use of methimazole during pregnancy. This association was reported twice before. It has certain implications with respect to therapy of pregnant hyperthyroid women.
Ataxia‐telangiectasia Associated with Sarcoidosis
Authors: Fleck Robin M. , Myers Linda K. , Wasserman Richard L. , Tigelaar Robert E. , Freeman Robert G.
Published: March 2008

Abstract: Ataxia‐telangiectasia and sarcoidosis are diseases characterized by abnormalities of the immune system. We examined a 13‐year‐old female with ataxia‐telangiectasia who developed atrophic, violaceous plaques of the skin and a destructive, polyarticular arthritis. The histologic features of both skin and synovium were most consistent with a diagnosis of sarcoidosis. Interaction of these two immunologic disorders may have resulted in the unusual features of this patient's disease.
Discoid Lupus Erythematosus and X‐linked Chronic Granulomatous Disease
Authors: Barton Leslie L. , Johnson Courtney R.
Published: March 2008

Abstract: X‐linked chronic granulomatous disease (CGD), a defect of leukocyte bactericidal capacity, was seen in three generations of a large kindred. The association of discoid lupus erythematosus (DLE) with CGD was noted. Recurrent antigenic stimulation leading to autoantibody formation may explain the apparently increased frequence of DLE in female carriers of CGD. A screen for CGD, the nitroblue tetrazolium test, is suggested for females with DLE who have experienced suppurative infections or who have a family history of early childhood deaths or recurrent infections.
Polymorphic Light Eruption in Pediatric Patients with American Indian Ancestry
Authors: Draelos Zoe Kececioglu , Hansen Ronald C.
Published: March 2008

Abstract: Polymorphic (polymorphous) light eruption (PMLE) is a cutaneous reaction to a broad spectrum of light energies that is found in persons of American Indian ancestry. We treated PMLE in 11 pediatric patients of American Indian ancestry.
Palmoplantar Milia: Sequelae of Early Congenital Syphilis
Authors: Noppakun Nopadon , Hendrick Sophia J. , Raimer Sharon S. , Sanchez Ramon L.
Published: March 2008

Abstract: A 5‐month‐old male was examined in our dermatology clinic with numerous palmoplantar milia. The child had early congenital syphilis with palmoplantar desquamation, which was followed by the development of milia. Histologic and electron microscopic features suggested that these secondary milia probably derived from the epidermis.
Hay‐Wells Syndrome
Authors: Shwayder Tor A. , Lane Alfred T. , Miller Marvin E.
Published: March 2008

Abstract: Hay‐Wells syndrome is an autosomal dominant constellation of facial clefting, ankyloblepharon filiforme adnatum (fused eyelids), and ectodermal defects. Our patient, a child who had these unusual features at birth, led us to consider possible differential diagnoses based on clinical features and review of the literature.