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Dermoid cysts (DCs) are benign cutaneous tumors that tend to persist and grow. The aim of this study was to examine the clinicopathologic features of congenital DCs. We present a case series of 75 children with a clinicopathologic diagnosis of DC. Seventy-two cysts were located on the head, one on the neck, and two on the trunk. Six cysts were located along the midline. Eight patients had symptoms other than changes in cyst size. Imaging studies were performed on 15 patients. Surgical excision was the primary treatment in all 75 cases. Neurosurgery and ophthalmology services were involved in the care of some patients. Histopathologic studies reported a foreign body giant cell reaction in 17 of the cysts. No recurrence was documented. DCs can remain stable for years, but they can become symptomatic as a result of enlargement and rupture or, more rarely, as a result of extension into surrounding tissues. Physicians should be aware that certain locations have a higher risk of DC extension, and adequate diagnostic investigations should be performed before their complete resection.

The aim of the present study is to clarify the association between environmental exposures and non-tuberculous mycobacterial (NTM) lymphadenitis, during the last decade, in a population of children. In children up to 14 years of age in a pediatric tertiary hospital, all cases of NTM lymphadenopathy with a specific microbiological diagnosis, from January 2004 to January 2015, were reviewed. This is a case-control study (1:5 proportion), in which the prevalence of environmental factors between cases and controls was compared by means of a multivariate logistic regression analysis. A total of 24 cases were diagnosed in the aforementioned period, and 18 of them included in the case-control study. Mycobacterium avium-intracellulare was the predominant mycobacterium species isolated (83.3%). Exposure to hens showed a clear trend to be significantly associated with the disease (OR = 4.33; IC95% 0.97-19.41, p = 0.055), with no significant differences for the rest of the risk factors studied.M. avium is still the predominant bacteria causing NTM lymphadenitis in children of our region. Contact with hens has been the only risk factor for NTM lymphadenitis detected in the present study. What is Known: • M. avium is the predominant bacteria causing NTM lymphadenitis in children of our region. • There is no consensus on which environmental factors are associated with NTM lymphadenitis in children. What is New: • The only risk factor for NTM lymphadenitis found in the present study was regular contact with hens. Contact with other farm animals was not associated to NTM lymphadenitis.

The common manifestations of atopic dermatitis (AD) appear sequentially with involvement of the cheeks in infancy, flexural extremities in childhood, and hands in adulthood. Although less common clinical manifestations are well described, they have not been the subject of epidemiologic studies to describe their prevalence in specific age groups. This observational, cross-sectional, comparative study included 131 children younger than 18 of both sexes with AD who attended the clinics of the Dermatology Department of the National Institute of Pediatrics in Mexico City. Patients were examined to determine the presence of infrequent clinical manifestations of AD during infancy, preschool and school age, and adolescence and stratified according to sex, age, and number of clinical signs. A chi-square test was used to detect differences according to age and sex. Logistic regression analysis was also performed. The main findings according to age were genital dermatitis and papular-lichenoid dermatitis variant in infants; atopic feet, prurigo-like, nummular pattern, and erythroderma in preschool and school-aged children; and eyelid eczema and nipple dermatitis in adolescents. The risk of development of nipple dermatitis and eyelid eczema increased with age, and the development of genital dermatitis decreased with age. The knowledge of the prevalence of less common clinical manifestations of AD according to age in different populations might be helpful in diagnosing incipient cases of AD.

v This paper describes Elops smithi, n. sp., and designates a lectotype for E. saurus. These two species can be separated from the five other species of Elops by a combination of vertebrae and gillraker counts. Morphologically, they can be distinguished from each other only by myomere (larvae) or vertebrae (adults) counts. Elops smithi has 73–80 centra (total number of vertebrae), usually with 75–78 centra; E. saurus has 79–87 centra, usually with 81–85 centra. No other morphological character is known to separate E. smithi and E. saurus, but the sequence divergence in mtDNA cytochrome b (d = 0.023–0.029) between E. smithi and E. saurus is similar to or greater than that measured between recognized species of Elops in different ocean basins. Both species occur in the western Atlantic Ocean, principally allopatrically but with areas of sympatry, probably via larval dispersal of E. smithi by oceanographic currents.

O objetivo neste trabalho foi avaliar a aplicabilidade, para búfalos (Bubalus bubalis), de testes de avaliação de temperamento, utilizados para bovinos. Foram avaliados os testes de docilidade, teste de reação a objetos novos, de tronco e de isolamento social em 12 bezerros e suas 12 mães, separadamente. Tanto os bezerros como suas mães responderam a todos os testes com vários comportamentos. Para os bezerros e as mães, os testes que promoveram maior número de comportamentos foram o de tronco e de reação a objetos novos. Houve diferenças individuais (coeficiente de variação de 32-51%) nos resultados de todos os testes tanto nos bezerros como nas mães. Os resultados individuais, em testes de isolamento social e de reação a objetos novos apresentaram correlação positiva tanto com as mães (rs = 0,76, P< 0,01) quanto com os bezerros (rs = 0,66, P< 0,01). Baseados em sensibilidade, relevância e aplicabilidade, o teste de isolamento social e o teste de tronco podem ser sugeridos para avaliarem o temperamento de bubalinos.The objective of this study was to assess the applicability of temperament tests commonly used in bovines for water buffaloes (Bubalus bubalis). The tests for docility, reaction to a novel object, squeeze cage and social isolation were applied individually with 12 calves and their 12 mothers. Both calves and their mothers responded to all tests with a variety of behaviors. The tests that elicited the larger number of behaviors were the squeeze cage and the novel object. There were large individual differences (coefficients of variation of 32-51%) in the scores for all tests for calves and adults. The individual scores for the tests of isolation and novel object were significantly correlated for calves (rs = 0.66, P< 0.01) and adults (rs = 0.76, P< 0.01). Using the criteria of sensitivity (range of individual differences), responsiveness and applicability, the squeeze cage test and the isolation test seem well suited for water buffaloes.

The exotic suckermouth armored catfishes are known to be established in Mexico. Pterygoplichthys multiradiatus is established in Campeche, Chiapas, and localities in central and western Mexico. We report the first record of another suckermouth armored catfish established in southeastern Mexico. We captured 7 specimens of Pterygoplichthys pardalis in a freshwater pond near Frontera, Tabasco, on 8 January 2005. Additional records in this region indicated that P. pardalis was spreading in the upper Grijalva-Usumacinta River basin. Further sampling and monitoring is required to assess the ecological impacts of this exotic species on the native fauna.

The effect of errors of alignment on the bearing contact of spiral bevel gear drives has been the subject of research for many years. Generally speaking, gear misalignment causes transmission errors and edge contacts, leading to incremental levels of noise and vibration, and a reduction of the gear drive service life. Apart from assembly and/or manufacturing errors, supporting shafts deflections caused by torque transmission constitute an important but predictable source of misalignments in gear drives. In the paper, a procedure of determination of the relative spatial position of spiral bevel gear supporting shafts during torque transmission will be proposed, in order to predict the relative errors of alignment between spiral bevel gears. The obtained errors of alignment will be employed as initial data in the local synthesis method, for the purpose of compensating them through modification of the pinion surface microgeometry. Finally, a numerical example will illustrate the proposed procedure, as well as the advantages of its consideration in the design of advanced spiral bevel gear drives in order to achieve the best contact pattern and function of transmission errors for nominal torque transmission.

The conditions of meshing and contact in hypoid gear drives depend substantially on the machine-tool settings to be applied. Determination of gear geometry is the first step in the design process of a hypoid gear drive. An approach for determination of basic machine-tool settings for face-hobbed and face-milled hypoid gears is proposed, covering the cases when the gear is generated and nongenerated. Gear basic machine-tool settings are determined from the blank data that can be obtained from application of Standard ANSI/AGMA 2005-C96. Some machine-tool settings are determined analytically considering the imaginary generation of the gear by a crown gear. Some other machine-tool settings are obtained numerically in order to provide some given blank data as the normal chordal tooth thickness and the normal pressure angles of the gear teeth. The developed theory is illustrated with numerical examples.

Free tissue transfer is commonly used in the reconstruction of post-ablative defects of the mandible. Due to lack of statistical power, comparing the survival of various free flaps, even in large studies, is challenging. The purpose of this study was to perform a meta-analysis comparing the survival of the most commonly used free flaps for mandibular reconstruction.We searched PubMed, EMBASE, and SCOPUS for relevant studies. A meta-analysis using the Peto one-step odds ratio (OR) with 95% confidence intervals (CI) was used to compare the pooled survival of the most commonly used free flaps for mandibular reconstruction.Of the 25,303 studies reviewed, 17 were selected for data extraction. A total of 1,221 subjects received 1,262 free flaps. Sixty-five free flaps failed. The pooled survival of all free flaps used for mandibular reconstruction was 94.8%. The deep circumflex iliac artery (DCIA) flap was associated with a seven-fold increase in failure when compared to the radial forearm free flap (Peto OR 7.40; 95% CI 1.38, 39.75, P = 0.02). There was no difference in survival when comparing other commonly used free flaps.The results of this study suggest that free flap reconstruction of the mandible is highly successful. With the exception of the increased survival of the radial forearm when compared to the DCIA, there is no difference in recipient site survival when comparing various free flaps for mandibular reconstruction.

Wetlands with rice paddies are key habitats in the conservation of biodiversity in the Mediterranean Region and are potentially suitable habitats for foraging bats, since they provide food (insects) and drinking places; nevertheless, many wetlands lack natural roosting sites. A bat-box program designed to ascertain bat-box preferences was initiated in 1999 in the Ebro Delta (NE Spain), one of the most important wetlands in Europe. A total of 69 bat-boxes of two types (single and double compartment) were placed on three supports (trees, houses, and posts) facing east or west. Pipistrellus pygmaeus occupancy rates and the number of individuals per box were monitored on 16 occasions from July 2000 to February 2004. Bat-box preferences were only detected during the breeding season. Bat abundance was higher in east-facing boxes, in double-compartment boxes, and in boxes placed on posts and houses. Boxes on natural supports (trees) were avoided. Bat-box occupancy rates were higher during the breeding season (95.6%, spring–summer) due to the formation of maternity colonies. The number of individuals in bat-boxes during the breeding season increased as the study period progressed (from summer 2000 to summer 2003), suggesting a high degree of acceptance by maternity colonies of these alternative locations. Occupancy rates observed were the highest ever reported in bat-box scientific literature. This study highlights the role of bat-box programs as useful alternative management tools for the conservation of bat populations in highly productive wetland habitats where few natural roost sites are available.

Many exotic fresh-water and brackish-water fish species have become established in Florida waters, but the red lionfish is the first entirely marine species that appears to have become established here. We give a detailed account of the initial collections of adult specimens from off St. Augustine and Jacksonville, including data on morphometrics, meristics, and gonad histology. Our review of historical sightings on Florida reefs and of the specimens reported herein suggests that the most plausible vector for the introduction of this fish was aquarium releases. We discuss our reasons for concluding that this species has probably become established in Florida, summarize the potential danger that these venomous fishes pose to the public, and examine the effect of a parasite hosted by the male lionfish. We also consider the potential effect of the red lionfish on marine communities in Florida, but limited information on its biology precludes a detailed assessment.

Of the patients with epidermal nevi, 10-18% may have disorders of the eye, nervous, and musculoskeletal systems. A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms.

The karyotypes of four gerreids of the western Atlantic Ocean are documented. A diploid chromosome complement of 48 telocentric chromosomes was found in the four species (2N=48t, fundamental number FN=48). No differences were detected either in the number of chromosomes of the standard karyotype, in their karyotype size, or between the karyotypes derived from male or female specimens of any of the species. Chromosome length decreased progressively and slightly from pair 1 to pair 24. The Ag–NOR karyotypes of E. argenteus and E. harengulus were characterized by the position of the nucleolar organizer regions next to the centromere in chromosome pair 1, whereas in E. gula and E. plumieri Ag–NORs were detected in pair 4. The other 46 chromosomes showed a light staining of the centromere with no terminal or intermediate heterochromatic blocks. All Eucinostomus species showed Ag–NORs of similar size, while Eugerres plumieri showed Ag–NORs 10–20% larger than Eucinostomus species. A combination of size and position of the Ag–NORs identified E. gula, while size alone identified E. plumieri. However, the ancestral state for size and position of Ag–NORs could not be established. There was no differential staining of the chromosomes by G-banding. The karyotype of the gerreids appears similar to the hypothetical ancestral karyotype of fish. The phylogenetic relationships among these species could not be established because of the lack of chromosome G-bands. Most likely this indicates a homogeneous distribution of GC nucleotides in the chromosomes.

In this article, we show that the dielectric constant of lead-free ferroelectric ceramics based on the solid solution (1-x)(Bi(0.5)Na(0.5))TiO(3)-xBaTiO(3), with compositions at or near the morphotropic phase boundary (MPB), can be tuned by a local applied electric field. Two compositions have been studied, one at the MPB, with x = 0.06 (BNBT6), and another one nearer the BNT side of the phase diagram, with x = 0.04 (BNBT4). The tunability of the dielectric constant is measured at microwave frequencies between 100 MHz and 3 GHz by a nonresonant method and simultaneously applying a dc electric field. As expected, the tunability is higher for the composition at the MPB (BNBT6), reaching a maximum value of 60% for an electric field of 900 V/cm, compared with the composition below this boundary (BNBT4), which saturates at 40% for an electric field of 640 V/cm. The high tunability in both cases is attributed to the fine grain and high density of the samples, which have a submicrometer homogeneous grain structure with grain size of the order of a few hundred nanometers. Such properties make these ceramics attractive for microwave tunable devices. Finally, we have tested these ceramics for their application as infrared pyroelectric detectors and we have found that the pyroelectric figure of merit is comparable to traditional lead-containing pyroelectrics.

Congenital cutaneous angioleiomyoma is an extremely rare benign smooth muscle tumor. We present a case of a firm, painful subcutaneous mass noticed at birth on the left leg that on surgical excision proved to be an angioleiomyoma. Prognosis is good, and recurrences are uncommon. To our knowledge, this is the second report of a congenital angioleiomyoma.

Primary immunodeficiency diseases (PIDs) are rare but important conditions found predominantly in children. We studied PIDs in a large pediatric hospital, their association with cutaneous alterations, and the importance of cutaneous alterations as diagnostic markers. Among 382,383 pediatric patients, 130 (0.0003%) had a PID: humoral in 27, cellular and combined in 18, phagocytic in 37, and associated with major defects in 45. An average of two cutaneous alterations were present in 90 (69%) patients: infections in 80, eczema-dermatitis in 38, and miscellaneous in 57. In 71 (79%) patients the cutaneous alterations preceded and were the basis for the clinical immunologic diagnosis. Only two PIDs were not associated with cutaneous lesions.

Cutaneous rhabdomyomatous mesenchymal hamartoma (RMH) is a rare benign tumor composed of two or more types of mesenchymal-derived cells. RMHs are generally sporadic and independent, but they can be associated with congenital abnormalities. We report a subcutaneous case of RMH in the sternoclavicular area with two recurrences after complete surgical excision. The course is variable and can range from spontaneous resolution to repeated recurrences.

A 67-year-old patient, with no respiratory symptoms, underwent coronary catheterization for revascularization of stenosis in the right coronary artery, during which we observed a large caliber artery, apparently bronchial, extending toward the left lung base (Fig. 1A). Multislice computed tomography angiography revealed a rounded heterogeneous consolidation, 3–4 cm, in the left cardiophrenic angle. Drainage veins extending toward the left atrium were observed within the consolidation (Fig. 1B). Bronchopulmonary sequestration of the left lower lobe was diagnosed. Pulmonary sequestration (PS) is a rare malformation (0.15%–6.4% of all bronchopulmonary malformations). It is defined as an area of non-functioning lung, unconnected, or poorly connected with the tracheobronchial tree, usually irrigated by the systemic circulation. PS is conventionally classified as intralobar or extralobar. Pryce1 identifies 3 types of PS, depending on its relationship with the bronchial tree and its blood supply. This was extralobar PS type 3, since the patient presented an extrabronchial pulmonary area irrigated by the systemic circulation. Irrigationwassupplied by the right coronary artery; this is an extraordinarily rare observation – only 15 cases have been reported in the medical literature.2

To give a child the ominous diagnosis of malignant melanoma is a very difficult decision for both clinician and pathologist. Difficulties in histologic diagnosis combined with a reluctance on the part of the clinician may lead to delays in diagnosis and curative surgical intervention. In fact, some melanomas in children are misdiagnosed clinically as benign lesions and recognized as melanomas only after excision. It is imperative to recognize that malignant melanoma occurs in children and to appreciate its clinical characteristics and predisposing factors. Incidence and Prognosis Approximately 2 percent of melanomas occur in patients under the age of 20 years, . . .

The reader will find in this paper a simple theoretical framework where to analyze the usual issues from environmental and economic growth literature, including the problem of global warming, the corresponding climate change, and the impact on economic system. This paper considers two environmental externalities: the first one is local and gives account of the marginal damage from emissions flow; the second one is aggregate, or global, and relates to the extreme damage which may happen if the accumulated stock of pollutants is on the threshold of a worldwide catastrophe. In this context dominated by market failures, the decentralized equilibrium path is inefficient and unsustainable, while the socially optimal balanced growth path shows a singularity with trajectories truncating and changing of course. With respect to the economy's long-run performance we s how environment matters in different ways and at different stages, and give conditions for sustainability of sustained growth.tudy

The common manifestations of atopic dermatitis (AD) appear sequentially with involvement of the cheeks in infancy, flexural extremities in childhood, and hands in adulthood. Although less common clinical manifestations are well described, they have not been the subject of epidemiologic studies to describe their prevalence in specific age groups. This observational, cross-sectional, comparative study included 131 children younger than 18 of both sexes with AD who attended the clinics of the Dermatology Department of the National Institute of Pediatrics in Mexico City. Patients were examined to determine the presence of infrequent clinical manifestations of AD during infancy, preschool and school age, and adolescence and stratified according to sex, age, and number of clinical signs. A chi-square test was used to detect differences according to age and sex. Logistic regression analysis was also performed. The main findings according to age were genital dermatitis and papular-lichenoid dermatitis variant in infants; atopic feet, prurigo-like, nummular pattern, and erythroderma in preschool and school-aged children; and eyelid eczema and nipple dermatitis in adolescents. The risk of development of nipple dermatitis and eyelid eczema increased with age, and the development of genital dermatitis decreased with age. The knowledge of the prevalence of less common clinical manifestations of AD according to age in different populations might be helpful in diagnosing incipient cases of AD.

Congenital cutaneous angioleiomyoma is an extremely rare benign smooth muscle tumor. We present a case of a firm, painful subcutaneous mass noticed at birth on the left leg that on surgical excision proved to be an angioleiomyoma. Prognosis is good, and recurrences are uncommon. To our knowledge, this is the second report of a congenital angioleiomyoma.

O objetivo neste trabalho foi avaliar a aplicabilidade, para búfalos (Bubalus bubalis), de testes de avaliação de temperamento, utilizados para bovinos. Foram avaliados os testes de docilidade, teste de reação a objetos novos, de tronco e de isolamento social em 12 bezerros e suas 12 mães, separadamente. Tanto os bezerros como suas mães responderam a todos os testes com vários comportamentos. Para os bezerros e as mães, os testes que promoveram maior número de comportamentos foram o de tronco e de reação a objetos novos. Houve diferenças individuais (coeficiente de variação de 32-51%) nos resultados de todos os testes tanto nos bezerros como nas mães. Os resultados individuais, em testes de isolamento social e de reação a objetos novos apresentaram correlação positiva tanto com as mães (rs = 0,76, P< 0,01) quanto com os bezerros (rs = 0,66, P< 0,01). Baseados em sensibilidade, relevância e aplicabilidade, o teste de isolamento social e o teste de tronco podem ser sugeridos para avaliarem o temperamento de bubalinos. The objective of this study was to assess the applicability of temperament tests commonly used in bovines for water buffaloes (Bubalus bubalis). The tests for docility, reaction to a novel object, squeeze cage and social isolation were applied individually with 12 calves and their 12 mothers. Both calves and their mothers responded to all tests with a variety of behaviors. The tests that elicited the larger number of behaviors were the squeeze cage and the novel object. There were large individual differences (coefficients of variation of 32-51%) in the scores for all tests for calves and adults. The individual scores for the tests of isolation and novel object were significantly correlated for calves (rs = 0.66, P< 0.01) and adults (rs = 0.76, P< 0.01). Using the criteria of sensitivity (range of individual differences), responsiveness and applicability, the squeeze cage test and the isolation test seem well suited for water buffaloes.

When there are pollution externalities the competitive equilibrium is not Pareto-optimal nor environmentally sustainable even if abatment activities are endogenously decided. In this paper we introduce the possibility of an ecological catastrophe like the one predicted by the global climate change, imposing the constraint of an upper-limit to the pollutants stock. We characterize this socially optimal soclution and study conditions for the sustainability of the balanced growth path. We find a trade-off between environmental quality and growth. The rate of growth depends negatively on the weight of environmental care in the utility function and positively on the population growth rate. We show that the emissions reduction recommended in the Kioto protocol is an appropriate policy to avoid the ecological catastrophe and ensure global efficiency and positive long-run growth.

In an endogenous growth model with pollution and abatement we characterize the socially optimal solution. We find that the rate of growth depends negatively on the weight of environmental care in utility and positively on the population growth rate. We also find a trade-off between growth and environmental quality beyond which an environmental Kuznets curve is derived in the long term. This one emerges from the implications of the demographic transition for the rate of population growth, and the accompanying variation in the willingness to pay for environmental quality as the economy develops.

Dermatitis artefacta is a factitious disorder in which there is deliberate conscious production of skin lesions. There are only a few reports that evaluate instances of dermatitis artefacta in the pediatric population. The aim of this retrospective study was to assess the characteristics of patients with this disorder who were seen at the National Institute of Pediatrics in Mexico City. The records of all patients diagnosed with dermatitis artefacta from January 1980 to December 1999 were analyzed. There were 29 patients (25 females, 4 males). The upper limbs and the face were the most commonly involved areas. Superficial erosions were the most frequent initial event, and residual lesions consisted of scars and crusts. Time taken to diagnosis was on average 10 months. Half of the patients were lost to follow-up. No correlation was found between the length of time from the disease onset to diagnosis, the type of lesions, and the clinical outcome. Twelve patients had an associated systemic disorder. The possible association with chronic disease has not been sufficiently stressed and demonstrates the importance of providing psychological support for these patients. Psychiatric diagnoses were anxiety, depression, and personality disorder. No correlation was found between the psychiatric diagnosis and the outcome of dermatitis artefacta. A young age at presentation, which has been considered important as a favorable prognostic sign, could not be demonstrated in our patients.

In this note, we extend Xie (JET, 1994) to solve analytically the Lucas model with a weak externality in a specific parametric case. In particular, we characterize the shape of imbalance effects in this model. Our results are mostly consistent with the findings of the related computational literature. Among other analytical findings, we prove that, while U-shaped imbalance effects are not apparent in the Lucas model for the growth rate of output per capita, the level of such a variable may exhibit this shape.

In this note, we use a technique analogous to Xie's method (1994) to solve analytically the Lucas model with externality in a specific parametric case. In particular, we characterize the shape of imbalance effects in this model. Our results are entirely consistent with the findings of the related computational literature. Moreover, our analytical investigation tends to show that these findings are robust to the presence of the Lucas externality as long as a unique equilibrium path exist.

Background: Rothmund–Thomson syndrome (RTS) is an autosomal recessive disorder associated with an increased predisposition to osteosarcoma. Children with RTS typically present with a characteristic skin rash (poikiloderma), small stature, and skeletal dysplasias. Mutations in the RECQL4 gene, which encodes a RecQ DNA helicase, have been reported in a few RTS patients. We examined whether a predisposition to developing osteosarcoma among an international cohort of RTS patients was associated with a distinctive pattern of mutations in the RECQL4 gene. Methods: We obtained clinical information about and biologic samples from 33 RTS patients (age range = 1–30 years). Eleven patients were diagnosed with osteosarcoma. All 21 exons and 13 short introns of the RECQL4 gene were sequenced from the genomic DNA of all subjects. Kaplan– Meier survival analysis was used to estimate the incidence of osteosarcoma among patients with and without mutations predicted to produce a truncated RECQL4 protein. Results: Twenty-three RTS patients, including all 11 osteosarcoma patients, carried at least one of 19 truncating mutations in their RECQL4 genes. The incidence of osteosarcoma was 0.00 per year in truncating mutation-negative patients (100 person-years of observation) and 0.05 per year in truncating mutation-positive patients (230 person-years of observation) (P = .037; two-sided log-rank test). Conclusions: Mutations predicted to result in the loss of RECQL4 protein function occurred in approximately two-thirds of RTS patients and are associated with risk of osteosarcoma. Molecular diagnosis has the potential to identify those children with RTS who are at high risk of this cancer. [J Natl Cancer Inst 2003;95: 669–74]

Due to the presence of two different clones of cells in early embryogenesis, numerous congenital and acquired dermatoses have a linear distribution following the lines of Blaschko. Acquired inflammatory skin diseases are rarely observed in linear patterns. Our patient was born with macrocephaly, left eye glaucoma, and a left facial and contralateral corporal hemihypertrophy, cerebral dysgenesis, and skeletal abnormalities. Hypopigmented S-shaped linear macules on the trunk and linear streaks on the arms and legs were compatible with hypomelanosis of Ito. At 5 years of age the patient presented with an erythematous follicular exanthem compatible with scarlet fever exclusively in the lines of Blaschko. This fact suggests a genetic mosaicism.

Objective We analyzed malfunction rates (obstruction, omental wrapping, displacement) and catheter survival for self-locating catheters as compared with other Tenckhoff catheter designs. Patients and Methods We conducted our survey at two centers, prospectively studying all self-locating catheters implanted from May 1997 to October 2000 and used for peritoneal dialysis (PD). Tenckhoff catheters of other designs used previously in our units were used as the control group. We analyzed removal causes and catheter survival. Results We studied 173 catheters (105 self-locating catheters, 53 straight catheters, and 15 coiled catheters) implanted in 139 patients (43% of them women) with a mean age of 53 ± 14 years. The analysis of catheter removal showed that 3 of 105 self-locating catheters, 3 of 15 coiled catheters, and 17 of 53 straight catheters were removed owing to malfunction (c 2: p = 0.0000). Kaplan–Meier curves showed that the bulk of removals for malfunction occurred within the first 3 months after PD start. The group of self-locating catheters showed better survival (log-rank: p = 0.0009). Other causes for catheter removal included peritonitis ( n = 22), exit-site infection alone ( n = 4), and end of PD treatment ( n = 66). No significant differences were seen in the annual peritonitis rate (straight-tip: 0.955 ± 2.315 episodes annually; coiled-tip: 0.651 ± 0.864 episodes annually; self-locating: 0.720 ± 1.417 episodes annually; t-test: p > 0.400). No gut or bladder perforations were observed. Conclusion In our survey, self-locating catheters were associated with better survival and fewer removals for malfunction than were Tenckhoff catheters of other designs.

A pediatric case of relapsing polychondritis is reported. Diagnostic features were recurrent episodes of painful inflammation of ear cartilage, episcleritis, and atopic disease. A good therapeutic response was obtained with systemic corticosteroids, dapsone, and methotrexate.