bluuebunny/crossref_metadata_2025 huggingface dataset:
Age‐related changes in gene expression patterns of matrix metalloproteinases and their collagenous substrates in mandibular condylar cartilage in rats
Authors: Bae Jin‐Wan , Takahashi Ichiro , Sasano Yasuyuki , Onodera Kazuyuki , Mitani Hidetoshi , Kagayama Manabu , Mitani Hideo
Published: July 2003

Matrix metalloproteinases (MMPs) have been implicated in physiological cartilage matrix remodelling as well as in pathological and invasive extracellular matrix remodelling of tissue. Age‐related changes in the gene expression patterns of MMPs in mandibular condylar cartilages (MCCs) were analysed. We examined the gene expression patterns of Mmp‐8 and ‐13 and their substrates, Col1a1, Col2a1 and Col10a1, in MCC of growing and ageing rats. Temporomandibular joints of male Wistar rats aged 4, 8, 16 and 32 weeks were subjected to in situ hybridization analysis. Histologically, MMCs showed characteristics of growth plate cartilage at ages 4 and 8 weeks, and more closely resembled articular cartilage thereafter. Mmp‐8 was expressed in the cells in all cartilaginous cell layers at ages 4 and 8 weeks, and then was localized only in the mature cells at ages 16 and 32 weeks. Whereas Mmp‐13 expression was limited to the lowermost hypertrophic chondrocytes in the growth stage, mature chondrocytes instead of hypertrophic chondrocytes expressed Mmp‐13 in adult non‐hypertrophic MCC. Because Mmp‐8 and ‐13 expression overlapped with Col2a1 and Col10a1, chondrocytes could play a pivotal role in degradation as well as production of the cartilaginous matrix in MCC.
Angiogenesis in the distal femoral chondroepiphysis of the rabbit during development of the secondary centre of ossification
Authors: Doschak M. R. , Cooper D. M. L. , Huculak C. N. , Matyas J. R. , Hart D. A. , Hallgrimsson B. , Zernicke R. F. , Bray R. C.
Published: July 2003

In the developing chondroepiphyses of long bones, the avascular cartilaginous anlage is invaded by numerous blood vessels, through the process of angiogenesis. The objective of this study was to investigate the chronology of this vascular invasion with the spontaneous calcification of the cartilaginous epiphysis during development of the secondary ossification centre in the rabbit distal femur. The time‐course of chondroepiphyseal vascular invasion was determined histologically and standardized for eight gestational and four postnatal intervals by plotting kit body mass against crown–rump length. Similarly, microcomputed tomography (µ‐CT) helped to visualize calcification at those same gestational and postnatal intervals. To confirm the angiogenic nature of the avascular chondroepiphysis, such samples were assayed on the chick chorio‐allantoic membrane (CAM). Neovascular outgrowths from the CAM were apparent 48 h following introduction of an 18‐day (gestational) chondroepiphyseal sample. Chondroepiphyseal samples were assayed for the potent developmental angiogenic factors bFGF and VEGF, with the mRNA expression for both these mediators being confirmed using RT‐PCR. As angiogenesis and calcification during chondroepiphyseal development occur in a defined tissue environment initially devoid of blood vessels and mineral, those processes provided a unique opportunity to study their progression without complication of injury‐related inflammation or extant vasculature and mineral. Furthermore, the discovery of angiogenic, angiostatic or mineral‐regulating mediators specific to developing connective tissue may prove useful for analysing the regulation of vascular and mineral pathogenesis in articular tissues.
The histological structure of the malleolar groove of the fibula in man: its direct bearing on the displacement of peroneal tendons and their surgical repair
Authors: Kumai T. , Benjamin M.
Published: July 2003

The peroneal (fibularis) tendons are held in place within the malleolar groove by the superior peroneal retinaculum. If this is torn, the tendons can subluxate or dislocate. Understanding the anatomy of the region is important for treating these injuries when it becomes necessary to reconstruct the malleolar groove surgically. Serial transverse sections of the groove were cut from 10 dissecting room cadavers after routine histology processing. The structure of the malleolar groove differed significantly in its proximal and distal parts. Distally, the bone is convex and the shape of the groove is determined by a thick periosteal cushion of fibrocartilage that covers the bone surface. Proximally, the groove shape is determined by the bone itself, and the periosteum is thin and fibrous. The restriction of a periosteal fibrocartilage to the distal end suggests that it serves to adapt the shape of the malleolar groove to that of the tendons within it and thus promotes stress dissipation. Paradoxically, however, it increases the risk of damage to subluxated tendons, because these can be sliced longitudinally by a sharp ridge created from periosteal fibrocartilage when the retinaculum is torn. Our results suggest that if bone‐block surgical procedures are used to reconstruct the malleolar groove, they are best restricted to its proximal part.
The growth and morphogenesis of the early mouse mandible: a quantitative analysis
Authors: Ramaesh Thaya , Bard Jonathan B. L.
Published: July 2003

Three‐dimensional reconstruction and BrdU incorporation have been used to quantify the development and growth of the mouse mandible and to analyse its relationship to Meckel's cartilage and the molar teeth. The mandible anlage is first histologically detectable at E13.5 as paired plates of osteoid tissue within condensed mesenchyme (∼0.9 mm long and ∼0.36 mm deep) that are lateral to the two arms of Meckel's cartilage. Over the next 3 days, each plate lengthens to ∼3.6 mm, and extends medially at its superior and inferior edges, folding over to enclose the alveolar nerve and Meckel's cartilage and producing additional processes that form the molar tooth sockets (E15.5). At around E15.5, the first molar tooth socket forms from two processes that extend from the medial and distal parts of the mandible to surround the tooth. By E16.5, this process is complete in the distal region where Meckel's cartilage is beginning to degenerate. Mandible ossification begins at E14 with proliferation restricted to the outer surface. BrdU incorporation rates are particularly high at the proximal and distal ends where lengthening occurs, and at the superior and inferior edges as they extend medially to surround Meckel's cartilage. Incorporation rates slow at the distal ends of each mandible at E16.5 as they approach each other at the symphysis. The results indicate that the mandible mainly grows at its periphery, and the pattern of mandibular growth and morphogenesis suggests that these processes are mainly directed and constrained by paracrine signalling from Meckel's cartilage and the tooth buds.
Detecting microdamage in bone
Authors: Lee T. C. , Mohsin S. , Taylor D. , Parkesh R. , Gunnlaugsson T. , O'Brien F. J. , Giehl M. , Gowin W.
Published: July 2003

Fatigue‐induced microdamage in bone contributes to stress and fragility fractures and acts as a stimulus for bone remodelling. Detecting such microdamage is difficult as pre‐existing microdamage sustained in vivo must be differentiated from artefactual damage incurred during specimen preparation. This was addressed by bulk staining specimens in alcohol‐soluble basic fuchsin dye, but cutting and grinding them in an aqueous medium. Nonetheless, some artefactual cracks are partially stained and careful observation under transmitted light, or epifluorescence microscopy, is required. Fuchsin lodges in cracks, but is not site‐specific. Cracks are discontinuities in the calcium‐rich bone matrix and chelating agents, which bind calcium, can selectively label them. Oxytetracycline, alizarin complexone, calcein, calcein blue and xylenol orange all selectively bind microcracks and, as they fluoresce at different wavelengths and colours, can be used in sequence to label microcrack growth. New agents that only fluoresce when involved in a chelate are currently being developed – fluorescent photoinduced electron transfer (PET) sensors. Such agents enable microdamage to be quantified and crack growth to be measured and are useful histological tools in providing data for modelling the material behaviour of bone. However, a non‐invasive method is needed to measure microdamage in patients. Micro‐CT is being studied and initial work with iodine dyes linked to a chelating group has shown some promise. In the long term, it is hoped that repeated measurements can be made at critical sites and microdamage accumulation monitored. Quantification of microdamage, together with bone mass measurements, will help in predicting and preventing bone fracture failure in patients with osteoporosis.
Intrapopulation variability in mineralization density at the human femoral mid‐shaft
Authors: Goldman H. M. , Bromage T. G. , Boyde A. , Thomas C. D. L. , Clement J. G.
Published: July 2003

One of several microstructural variables known to affect the mechanical properties of bone is the degree of mineralization of bone matrix. The aim of this study was to examine mineralization density, and its variability with age and sex, from a biomechanical perspective. Histological sections, prepared from mid‐shaft femora obtained at autopsy from 40 individuals, were imaged using quantitative backscattered electron microscopy. Each cross‐section montage was divided into 48 segments according to anatomical position. Mean grey‐level values were quantified for each segment. One‐way anova with Tukey HSD post hoc tests were used to test for differences in mineralization between segments, age groups and sexes. Results showed a decrease in overall degree of mineralization density with adult age, but an increase in its coefficient of variation. Degree of mineralization was significantly lower in the periosteal third of the cortex, particularly in the antero‐lateral aspect. This pattern was most prevalent amongst the youngest individuals in the sample. Whereas males between ages 45–64 years had a higher average degree of mineralization than females, the opposite was true of the older age group. Mineralization significantly decreased between middle and older age groups in males, but not in females. Despite limited consistencies in the location of high and low average mineralization bone through the cortex, the degree of interindividual variation, even within a single age and sex group, overwhelmed population level trends. The patterns of variability identified in this study are consistent with results of an analysis of collagen fibre orientation using the same sample material.
The real response of bone to exercise
Authors: Boyde Alan
Published: July 2003

This review presents findings made in studies of large mammalian bones, especially from racehorse training experiments (2–8 years old, third metacarpal, tarsal) and human autopsy orthopaedic femoral implant retrievals and other human biopsy and autopsy cases. Samples were cleaned to analyse mineralized matrix in three dimensions, or poly methyl‐methacrylate embedded and micromilled to delete topography and study the superficial c. 0.5‐µm two‐dimensional section using quantitative backscattered electron imaging. With experimental implant studies in rabbits, observations were also made in vivo using confocal microscopy. Cracks in both calcified cartilage and bone may be removed by infilling with calcified matrix. This may be a general repair mechanism for calcified connective tissue crack repair. The fraction of the organ volume occupied by any form of bone tissue in equine distal third metacarpal extremities was increased in the more exercised groups by bone deposited within former marrow adipocytic space. Where deposited upon prior lamellar bone surfaces, this occurred without the intervention of prior resorption and without the formation of a hypermineralized cement line. Exercise inhibited osteoclastic resorption at external anatomical growth modelling sites where it normally occurs. Addition is not coupled to time‐wasting resorption: both internally and externally, it occurs both by layering on existing cancellous surfaces and by creation of new immature scaffold, with de novo incorporation of a rich, capillary blood vessel supply. The real response within bone organs subjected to mechanical overload exercise within normal physiological limits is to make more, and to lose less, bone.
WRN, the protein deficient in Werner syndrome, plays a critical structural role in optimizing DNA repair
Authors: Chen Lishan , Huang Shurong , Lee Lin , Davalos Albert , Schiestl Robert H. , Campisi Judith , Oshima Junko
Published: July 2003

Werner syndrome (WS) predisposes patients to cancer and premature aging, owing to mutations in WRN. The WRN protein is a RECQ‐like helicase and is thought to participate in DNA double‐strand break (DSB) repair by non‐homologous end joining (NHEJ) or homologous recombination (HR). It has been previously shown that non‐homologous DNA ends develop extensive deletions during repair in WS cells, and that this WS phenotype was complemented by wild‐type (wt) WRN. WRN possesses both 3′ → 5′ exonuclease and 3′ → 5′ helicase activities. To determine the relative contributions of each of these distinct enzymatic activities to DSB repair, we examined NHEJ and HR in WS cells (WRN–/–) complemented with either wtWRN, exonuclease‐defective WRN (E–), helicase‐defective WRN (H–) or exonuclease/helicase‐defective WRN (E–H–). The single E– and H– mutants each partially complemented the NHEJ abnormality of WRN–/– cells. Strikingly, the E–H– double mutant complemented the WS deficiency nearly as efficiently as did wtWRN. Similarly, the double mutant complemented the moderate HR deficiency of WS cells nearly as well as did wtWRN, whereas the E– and H– single mutants increased HR to levels higher than those restored by either E–H– or wtWRN. These results suggest that balanced exonuclease and helicase activities of WRN are required for optimal HR. Moreover, WRN appears to play a structural role, independent of its enzymatic activities, in optimizing HR and efficient NHEJ repair. Another human RECQ helicase, BLM, suppressed HR but had little or no effect on NHEJ, suggesting that mammalian RECQ helicases have distinct functions that can finely regulate recombination events.
Are mice calorically restricted in nature?
Authors: Austad Steven N. , Kristan Deborah M.
Published: July 2003

An important question about traditional caloric restriction (CR) experiments on laboratory mice is how food intake in the laboratory compares with that of wild mice in nature. Such knowledge would allow us to distinguish between two opposing views of the anti‐aging effect of CR – whether CR represents, in laboratory animals, a return to a more normal level of food intake, compared with excess food consumption typical of laboratory conditions or whether CR represents restriction below that of animals living in nature, i.e. the conditions under which house mice evolved. To address this issue, we compared energy use of three mouse genotypes: (1) laboratory‐selected mouse strains (= laboratory mice), (2) house mice that were four generations or fewer removed from the wild (= wild‐derived mice) and (3) mice living in nature (= wild mice). We found, after correcting for body mass, that ad libitum fed laboratory mice eat no more than wild mice. In fact, under demanding natural conditions, wild mice eat even more than ad libitum fed laboratory mice. Laboratory mice do, however, eat more than wild‐derived mice housed in similar captive conditions. Therefore, laboratory mice have been selected during the course of domestication for increased food intake compared with captive wild mice, but they are not particularly gluttonous compared with wild mice in nature. We conclude that CR experiments do in fact restrict energy consumption beyond that typically experienced by mice in nature. Therefore, the retarded aging observed with CR is not due to eliminating the detrimental effects of overeating.
Selective silencing by RNAi of a dominant allele that causes amyotrophic lateral sclerosis
Authors: Ding Hongliu , Schwarz Dianne S. , Keene Alex , Affar El Bachir , Fenton Laura , Xia Xugang , Shi Yang , Zamore Phillip D. , Xu Zuoshang
Published: July 2003

RNA interference (RNAi) can achieve sequence‐selective inactivation of gene expression in a wide variety of eukaryotes by introducing double‐stranded RNA corresponding to the target gene. Here we explore the potential of RNAi as a therapy for amyotrophic lateral sclerosis (ALS) caused by mutations in the Cu, Zn superoxide dismutase (SOD1) gene. Although the mutant SOD1 is toxic, the wild‐type SOD1 performs important functions. Therefore, the ideal therapeutic strategy should be to selectively inhibit the mutant, but not the wild‐type SOD1 expression. Because most SOD1 mutations are single nucleotide changes, to selectively silence the mutant requires single‐nucleotide specificity. By coupling rational design of small interfering RNAs (siRNAs) with their validation in RNAi reactions in vitro and in vivo, we have identified siRNA sequences with this specificity. A similarly designed sequence, when expressed as small hairpin RNA (shRNA) under the control of an RNA polymerase III (pol III) promoter, retains the single‐nucleotide specificity. Thus, RNAi is a promising therapy for ALS and other disorders caused by dominant, gain‐of‐function gene mutations.
Metalloporphyrins improve the survival of Sod2‐deficient neurons
Authors: Patel Manisha N.
Published: July 2003

The objective of this study was to determine whether metalloporphyrin catalytic antioxidants influence the survival of neuronal cultures in an in vitro model of age‐related mitochondrial oxidative stress. Neuronal cultures were prepared from cerebral cortices of manganese superoxide dismutase (MnSOD or Sod2) knockout (homozygous –/–, heterozygous –/+ or wild‐type +/+) mice. The ability of catalytic antioxidants, manganese tetrakis‐(4‐benzoic acid) porphyrin (MnTBAP) and manganese tetrakis‐(N‐ethyl‐2‐pyridyl) porphyrin (MnTE‐2‐PyP) to influence the survival of cultured cerebrocortical neurones from Sod2‐replete (+/+) and Sod2‐deficient (+/– or –/–) mice was assessed. Sod2–/– cultures showed accelerated cell death in serum‐free conditions when grown in ambient oxygen. MnTBAP and MnTE‐2‐PyP delayed the death of Sod2–/– cultures and improved the survival of Sod2+/+ and Sod2+/– cultures in serum‐free conditions. The results suggest that metalloporphyrin antioxidants can delay neuronal death resulting specifically from increased mitochondrial oxidative stress. Furthermore, Sod2‐deficient neuronal cultures provide a simple model system to screen the biological efficacy of mitochondrial antioxidants.
Genomic regions responsible for manganese superoxide dismutase regulation in Drosophila melanogaster
Authors: Paul Anirban , Duttaroy Atanu
Published: July 2003

The transcription of manganese superoxide dismutase (MnSOD), expression of which is essential for detoxification of superoxide radicals from mitochondria, has been shown to be regulated in vitro by many factors and conditions including oxidative stress, cytokines, lipopolysaccharide, cytoplasmic myc (c‐myc), p53 and tumour necrosis factors. Here we describe genomic regions in Drosophila melanogaster with regulatory effects on transcription of the MnSOD gene at an organism‐wide level. To understand the integrated regulation of MnSOD expression we screened chromosomes of D. melanogaster to locate deficiencies that altered the expression of MnSOD. Suppressors of MnSOD were screened by assessing the relative message abundance of MnSOD in 149 deletions covering approximately 81% of the Drosophila genome. The chromosomal deficiency Df(2R)017 significantly up‐regulated MnSOD mRNA by 1.7‐fold. Deficiency in four other genomic intervals, Df(1)ct‐J4, Df(2L)BSC4, Df(3L)66C‐G28 and Df(3R)Scr, down‐regulated MnSOD expression. Changes in MnSOD expression were positively associated with paraquat sensitivity of the deletion genotypes. Thus, at least one candidate enhancer and four candidate suppressors exist in the Drosophila genome to regulate the transcriptional activity of the MnSOD gene in vivo.
Mucocutaneous Findings in Pediatric AIDS Related to Degree of Immunosuppression
Authors: Wananukul Siriwan , Deekajorndech Tawatchai , Panchareon Chitsanu , Thisyakorn Usa
Published: July 2003

Abstract: The normal value of the absolute CD4‐positive T‐lymphocyte count is relatively high in normal infants and declines steadily until 6 years of age, whereas the CD4 percentage of the total lymphocyte count is constant. The immunologic categories according to the 1994 revised pediatric human immunodeficiency virus (HIV) classification, based on CD4‐positive percentage of the total lymphocyte count, is classified into three categories: no evidence of suppression (≥25%), moderate suppression (15–24%), and severe suppression (1–14%). Our objective was to determine the prevalence of mucocutaneous findings in pediatric acquired immunodeficiency syndrome (AIDS) related to the degree of immunosuppression. We prospectively examined 120 children less than 13 years of age who were born to HIV‐seropositive women and developed definite HIV infection. The prevalence of mucocutaneous findings in those children who had severe, moderate, and no evidence of immunosuppression were 62%, 43%, and 20%, respectively. The mucocutaneous findings in patients in the moderate and severe suppression groups were significantly more common than in patients without evidence of immunosuppression (p < 0.001). In the moderate immunosuppression group, 11% had two mucocutaneous findings while 21% in the severe immunosuppression group had two or more mucocutaneous findings. The most common mucocutaneous finding was oral candidiasis (33%), which had a mean corresponding CD4 percentage of the total lymphocyte count of 11.3%. Herpes zoster was found in 6% of the patients (mean CD4 percentage of the total lymphocyte count = 13.5%). Chronic herpes simplex virus (HSV) stomatitis was found in 3% of the patients (mean CD4 percentage of the total lymphocyte count = 3%). Mucocutaneous manifestations are common in pediatric AIDS. The majority of these findings have an infectious etiology. The prevalence increases as the CD4‐positive percentage of the total lymphocyte count decreases. More than one mucocutaneous finding can be found at the same time in patients with moderate or severe immunosuppression.
Childhood Discoid Lupus Erythematosus: A Tunisian Retrospective Study of 16 Cases
Authors: Cherif Faïka , Mebazaa Amel , Mokni Mourad , El Euch Dalenda , Azaiz Mohamed Iadh , Dhahri Amel Ben Osman
Published: July 2003

Abstract: Discoid lupus erythematosus (DLE) is uncommon in children. The clinical features of childhood DLE are similar to those of adult DLE in presentation and chronic course. However, children have a particularly high level of transition to systemic disease. We undertook a retrospective study of 16 children with DLE ranging in age from 2 to 15 years, seen over a 9‐year period. Six were less than 10 years old at the onset of the disease. The sex ratio was equal. The frequency of childhood DLE was about 7% of the total number of DLE patients seen in our department. Photosensitivity was defined as a clinical history of induction or exacerbation of discoid lesions following sun exposure, and was present in 81% of patients. There was no progression to systemic lupus erythematosus (SLE); an average follow‐up time was 10.5 months (2–30 months). We would like to emphasize the increased frequency of childhood DLE in our country and the importance of photosensitivity. However, follow‐up data regarding transition to SLE is lacking, therefore we are unable to offer a prognosis to our patients.
Superficial Mycotic Infections of the Foot in a Native Pediatric Population: A Pathogenic Role for Trichosporon cutaneum?
Authors: Archer‐Dubon Carla , Orozco‐Topete Rocío , Leyva‐Santiago Jaime , Arenas Roberto , Carbajosa Josefina , Ysunza Alberto
Published: July 2003

Abstract: Superficial mycotic infections of the feet are usually caused by Tricophyton rubrum, predominantly affecting adults and resulting from the use of occlusive footwear. We carried out a mycologic study of superficial foot infections in a rural school in Mexico where most people wear a leather, nonocclusive sandal. Forty students had clinical signs of 50 fungal infections of the foot: 39 athlete's foot and 11 onychomycosis. Thirty‐one boys and 9 girls were studied. Hyphae were seen in 11 cases of athlete's foot and 5 of onychomycosis. Twenty‐one cultures were positive (42%). The most frequently isolated fungi were the opportunistic Trichosporon cutaneum in 42.8%, Candida sp. (23.8%), Trichophyton mentagrophytes (23.8%), and Candida glabrata (9.5%). Superficial mycotic infections of the feet and nails were most frequent in children and adolescents who usually wear nonocclusive shoes. The most frequent pathogens were Candida sp. and T. mentagrophytes. It is interesting to note the prevalence of T. cutaneum that has recently been implicated in mycoses of the feet and nails. We did not isolate T. rubrum in any patient.
Are Children with Recessive Dystrophic Epidermolysis Bullosa of Low Birthweight?
Authors: Fox A. T. , Alderdice F. , Atherton D. J.
Published: July 2003

Abstract: To compare the birthweight of children affected by recessive dystrophic epidermolysis bullosa (RDEB) to a sibling control group, we designed a questionnaire‐based case‐control study. As participants we used patients with RDEB attending the Great Ormond Street Hospital for Children, London, England, and their nearest unaffected siblings. We found that children with RDEB are of significantly lower birthweight than their unaffected siblings, with 30% being small for their gestational age compared to 12% of controls (McNamar χ2 = 4.9, d f = 1, p = 0.02). A conditional logistic regression model was used to examine the possible effects of confounding variables. The relationship between the RDEB and standardized birthweight groups, smoking status of the mother at the time of birth, and the previous number of live births showed that the standardized birthweight group was the only significant variable in the model and was unaffected by confounding variables. Based on these findings, we concluded that the compromise in growth seen in RDEB begins in utero.
Juvenile Xanthogranuloma of the Proximal Nail Fold
Authors: Piraccini Bianca Maria , Fanti Pier Alessandro , Iorizzo Matilde , Tosti Antonella
Published: July 2003

Abstract: An 18‐month‐old Caucasian boy presented with a firm 0.5 mm nodule, pink‐red in color, with a yellow hue and some telangiectases on the surface, localized on the right thumbnail. The nodule involved all of the proximal nail fold and covered the proximal third of the nail. Pathology showed a dense dermal infiltrate of histiocytes, some of which had foamy nuclei, and multinucleated Touton giant cells. The lesion progressively decreased in size and had completely disappeared after 3 years. Periodic follow‐up was important not only to monitor evolution of the juvenile xanthogranuloma, but also to avoid excessive growth of the lesion with possible definitive nail matrix damage.
Recalcitrant Scrofuloderma Due to Rib Tuberculosis
Authors: Kaur Sukhjot , Thami Gurvinder P. , Gupta P. N. , Kanwar Amrinder J.
Published: July 2003

Abstract: Tuberculosis (TB) of the ribs is extremely rare and its diagnosis is often delayed due to the late appearance of radiologic signs. Although antituberculous drugs are the mainstay of treatment, surgery has a definite therapeutic role in recalcitrant or recurrent cases. A 5‐year‐old boy with scrofuloderma of the chest wall due to underlying TB of the rib, who responded partially to antituberculous treatment is reported. Surgical resection of the affected rib led to complete resolution. Early diagnosis of TB of this rare site and the role of surgical intervention in such cases is emphasized.
Nevus Lipomatosus Cutaneus Superficialis
Authors: Lane Joshua E. , Clark Edward , Marzec Tomasz
Published: July 2003

Abstract: Nevus lipomatosus cutaneus superficialis (NLCS) is a rare developmental anomaly characterized by isolated ectopic mature adipose tissue in the dermis. We describe a child with a NLCS present from birth that was growing in size. The lesion was removed by simple excision and has not recurred.
Tinea Nigra: Report of Two Cases in Infants
Authors: Pegas José Roberto , Criado Paulo Ricardo , Lucena Suzana Kilian , De Oliveira Marco Antônio
Published: July 2003

Abstract: Tinea nigra, a relatively uncommon mycosis caused by Phaeoannelomyces werneckii, is typically seen as an asymptomatic brown or black macule on the hands and feet. We present two cases of tinea nigra in children in São Paulo, Brazil, and alert readers to the potential for confusion with melanocytic lesions.
Necrotic Facial Papules in an Adolescent: C2 Deficiency with Eventual Development of Lupus Erythematosus
Authors: Lyon Valerie B. , Nocton James J. , Drolet Beth Ann , Esterly Nancy B.
Published: July 2003

Abstract: A 14‐year‐old girl was admitted to the hospital because of persistent throat pain, fever, fatigue, 25 pound weight loss, and leukopenia. On physical examination she was thin, ill‐appearing, and had necrotic papules on the face and palpable cervical lymph nodes. Presumptive differential diagnosis included occult malignancy and infection. Numerous investigative procedures failed to elucidate a source. Vasculitis was eventually appreciated after repeat skin biopsy. Numerous serologic studies were performed and were notable for a very low level of the second component of complement without direct evidence of lupus erythematosus (LE) or other autoimmune conditions. A diagnosis of C2 deficiency‐associated vasculitis was made. She was treated with high‐dose prednisone and cyclophosphamide with resolution of her symptoms. Two years later she returned with marked malar erythema. Antinuclear and Smith antibodies were then detected and a diagnosis of LE was made. She was treated with hydroxychloroquine and sun‐avoidance measures with clearance of the malar rash.
Olmsted Syndrome: The Clinical Spectrum of Mutilating Palmoplantar Keratoderma
Authors: Bergonse Fabiane N. , Rabello Suzy M. , Barreto Rodrigo L. , Romiti Ricardo , Nico Marcello Menta S. , Aoki Valéria , Reis Vitor Manoel S. , Rivitti Evandro A.
Published: July 2003

Abstract: Olmsted syndrome is a rare variety of transgredient palmoplantar keratoderma with an undefined inheritance pattern. It starts in the neonatal period or in childhood and has a progressive and extremely disabling course. We report two new, unrelated patients with Olmsted syndrome, one with the early signs and symptoms of the disorder, the other showing the full‐blown spectrum of the syndrome during a 30‐year follow‐up period.
A Pediatric Case of Sclerodermatous Chronic Graft‐versus‐Host Disease
Authors: Terasaki Kenjiro , Kanekura Takuro , Setoyama Mitsuru , Kanzaki Tamotsu
Published: July 2003

Abstract: We report a rare case of sclerodermatous chronic graft‐versus‐host disease (GVHD) in a 6‐year‐old boy that occurred after bone marrow transplantation for his aplastic anemia. The clinical manifestation and histopathologic findings were typical of scleroderma. Although various kinds of treatment have been tried for scleroderma, no established therapy exists. Furthermore, treating this disease is even more difficult in children. In the future, clarification of the pathogenesis of chronic GVHD and establishment of therapy will be necessary.
Neurocutaneous Melanosis with Transposition of the Great Arteries and Renal Agenesis
Authors: Köksal Nilgün , Bayram Yusuf , Murat Işık , Doğru Murat , Bostan Özlem , Sevinir Betül , Yazıcı Zeynep
Published: July 2003

Abstract: Neurocutaneous melanosis (NCM) is rare and is characterized by the proliferation of melanocytes in the central nervous system. A 6‐day‐old infant boy was referred to our department with giant congenital melanocytic nevi and convulsions. On physical examination the patient had a giant black‐brown pigmented nevus covering his face, neck, scalp, shoulders, back, chest, and abdomen. Numerous satellite lesions were noted on the face, neck, and upper extremities. In the right bulbar conjunctiva, a brown plaque was present. Magnetic resonance imaging (MRI) showed hyperintense areas in the brain on short repetition time/short echo time sequences, compatible with intraparenchymal melanin deposits. No leptomeningeal abnormality was seen. Further investigation also revealed agenesis of the right kidney and transposition of the great arteries. Transposition of the great arteries, which has never been reported in NCM, may be an incidental finding. We present a case of NCM associated with agenesis of the right kidney and transposition of the great arteries.
Congenital Primary Cutaneous Rhabdomyosarcoma in a Neonate
Authors: Brecher Alexandra R. , Reyes‐Mugica Miguel , Kamino Hideko , Chang Mary Wu
Published: July 2003

Abstract: We report a case of congenital primary cutaneous rhabdomyosarcoma, solid alveolar type, presenting as a solitary skin lesion on the right upper lip of a 2‐week‐old infant boy. Rhabdomyosarcoma originates from the embryonic mesenchyme precursor of striated muscle. Histologically it belongs to the group of “small round cell tumors.” Its myogenic origin is ascertained by immunohistochemical studies positive for myogenin, muscle‐specific actin, desmin, and myoglobin. Malignancy in the neonatal period is uncommon and the clinical management presents considerable challenges. Congenital alveolar rhabdomyosarcoma is a highly malignant tumor with no record of long‐term survivors. Treatment options include chemotherapy, excision, and radiotherapy. This infant's tumor was responsive to chemotherapy and surgery and he was free of disease at the 6‐month follow‐up.
Stiff Skin Syndrome: A Case Report
Authors: Mat Cem , Kalayciyan Aylin , Arzuhal Nurcan , Demirkesen Cuyan , Karaçorlu Murat , Tüzün Yalçın
Published: July 2003

Abstract: Stiff skin syndrome (SSS) is a disease similar to scleroderma with an unknown etiology. Stone‐hard areas of skin are observed from birth or in early childhood. In this article we describe a 15‐year‐old girl with skin hardening and limitation of movement. We diagnosed the case as SSS, of which we have not encountered a similar report in the Turkish literature.
Nail Dystrophy in Congenital Cutaneous Candidiasis
Authors: Clegg Herbert W. , Prose Neil S. , Greenberg David N.
Published: July 2003

Abstract: Congenital cutaneous candidiasis (CCC) is usually a benign condition characterized by various skin manifestations and is rarely associated with nail changes. We report a premature infant with CCC who developed dystrophy of all 20 nails at about 1 month of age. Nail dystrophy due to Candida albicans in the young infant may be differentiated from other congenital or hereditary nail malformations by appearance, recovery of the organism in nail culture, and complete resolution over a period of several months.
Infantile Myofibroma in a Prematurely Born Twin: A Case Report
Authors: Jurčić Vesna , Perković Tanja , Pohar‐Marinšek Živa , Hvala Asta , Lazar Inka
Published: July 2003

Abstract: Infantile myofibromatosis is a rare benign tumor of infancy and childhood that occurs in solitary, multiple, and generalized forms with similar histology but different clinicopathologic and prognostic implications. Even solitary tumors need follow‐up, as the type of presentation will be determined over time. It is necessary to differentiate this entity from other more aggressive tumors, especially rhabdomyosarcoma, which is treated by chemotherapy prior to excision. We describe a prematurely born twin girl who had at birth a solitary tumor of the cervicoscapular region, involving the dermis and subcutis. A fine‐needle aspiration biopsy (FNAB) specimen obtained soon after her birth suggested a diagnosis of benign neoplasm. The tumor was excised 1 month later, at which time it was significantly enlarged, ulcerated, and also exhibited worrisome histologic features including mitoses and infiltrative growth. It had the characteristic histologic pattern of infantile myofibromatosis, and myofibroblastic features of tumor cells were confirmed immunohistochemically and ultrastructurally. During the follow‐up period of 39 months, there was no sign of recurrence or new tumors.
Two Cases for Diagnosis: Asymmetric Childhood Scleredema or Stiff Skin Syndrome?
Authors: Morrell Dean S. , Challgren Eric , Nijhawan Anita , Olson Judyann , Laumann Anne , Medenica Maria , Spencer Charles , Esterly Nancy B.
Published: July 2003

Abstract: Two children with chronic and asymmetric skin indurations are presented. The clinical and pathologic features are suggestive of asymmetric childhood scleredema and stiff skin syndrome. The key features of scleredema and stiff skin syndrome are discussed.
Phthirus pubis Infestation of the Eyelashes and Scalp Hairs in a Girl
Authors: Ikeda Nariko , Nomoto Hiroo , Hayasaka Seiji , Nagaki Yasunori
Published: July 2003

Abstract: A 4‐year‐old girl was seen because of foreign bodies on the eyelashes. Parasites and their nits were seen on the patient's eyelashes and scalp. Her parents denied having Phthirus pubis infection of the axillary, pubic, or body hair, despite confirmation of the infestation in their child. The parasites were removed and the lashes with nits were cut off at the base. The parasite was identified as P. pubis. The scalp was washed with phenothrin shampoo. One month later no P. pubis infestation was found. Removal of the parasites, cutting the lashes at the base, and using phenothrin shampoo on the scalp were effective in resolving phthiriasis.
Lidocaine Iontophoresis for Topical Anesthesia Before Dermatologic Procedures in Children: A Randomized Controlled Trial
Authors: Wagner Annette M. , Cunningham Bari , Zempsky William T. , Parkinson Thomas M.
Published: July 2003

Abstract: Local anesthesia by injection in pediatric patients undergoing dermatologic procedures is not well received because of the pain of injection and the fear of needles. Lidocaine iontophoresis is a method of topical anesthesia where lidocaine is driven into the skin under the influence of electric current. We performed a prospective double‐blind, placebo‐controlled evaluation of iontophoresis of 2% lidocaine with 1:100,000 epinephrine. Sixty children requiring dermatologic procedures were enrolled (50 shave biopsy, 7 curettage, 2 injection, 1 punch biopsy). Twenty‐nine of 31 patients in the lidocaine group versus 2 of 29 placebo patients required no supplemental anesthesia (p < 0.001). The pain reported by the patients on the Oucher pain scale subsequent to the procedure was significantly lower in the lidocaine group (p < 0.001). Investigators and parents also rated pain lower in the lidocaine group (p < 0.001). Blanching and/or erythema occurred in 58 of 60 patients, but resolved within 1 hour in all patients. There were no other adverse events. Lidocaine iontophoresis is a safe and effective method of topical anesthesia prior to dermatologic procedures in children.
Evaluation of Left Ventricular Systolic and Diastolic Global Function: Peak Positive and Negative Myocardial Velocity Gradients in M‐Mode Doppler Tissue Imaging
Authors: Ueno Yoshiki , Nakamura Yasuyuki , Ohbayashi Yasunori , Kinoshita Masahiko
Published: July 2003

Objectives: To evaluate a new indicator of left ventricular global function: Myocardial velocity gradient (MVG) M‐mode Doppler tissue imaging (DTI). Background: MVG is a new indicator of regional left ventricular function and global left ventricular diastolic function. However, it is unclear whether MVG also is an indicator of left ventricular global function in comparison with invasive indices. Methods: We performed conventional imaging and M‐mode DTI in 85 subjects and calculated MVG at the posterior wall. We obtained satisfactory images in 65 subjects, who we divided into three groups: Noninvasive study group, invasive study group, and hemodialysis group. The noninvasive study group was divided into three subgroups (a younger normal subgroup, an older normal subgroup, and a cardiomyopathy subgroup), and MVG was compared with indices of conventional imaging. In the invasive study group, we compared MVG and indices of conventional imaging with hemodynamic data (peak positive and negative dp/dt, and the time constant T) using a high fidelity micromanometer‐tipped catheter. In the hemodialysis group, we compared indices before hemodialysis with those after hemodialysis. Results: Peak positive MVG correlated well with peak positive dp/dt (r = 0.79), and this did not change with hemodialysis (P = 0.87). Peak negative MVG also correlated well with peak positive dp/dt and the time constant T (r = 0.88 and r = 0.80), and this did not change with hemodialysis (P = 0.97). Conclusions: Peak positive and negative MVG are sensitive and load‐insensitive indicators of left ventricular function.
Quantitative Assessment of Aortic Stenosis by Three‐Dimensional Anyplane and Three‐Dimensional Volume‐Rendered Echocardiography
Authors: Handke Michael , Schäfer Dietrich Markus , Heinrichs Gudrun , Magosaki Etsuko , Geibel Annette
Published: July 2003

Aortic stenosis is a challenge for three‐dimensional (3‐D) echocardiographic image resolution. This is the first study evaluating both 3‐D anyplane and 3‐D volume‐rendered echocardiography in the quantification of aortic stenosis. In 31 patients, 3‐D echocardiography was performed using a multiplane transesophageal probe. Within the acquired volume dataset, five parallel cross sections were generated through the aortic valve. Subsequently, volume‐rendered images of the five cross sections were reconstructed. The smallest orifice areas of both series were compared with the results obtained by two‐dimensional (2‐D) transesophageal planimetry and those calculated by Doppler continuity equation. No significant differences were found between Doppler (0.76 ± 0.18 cm2), 2‐D echocardiography (0.78 ± 0.24 cm2), and 3‐D anyplane echocardiography (0.72 ± 0.29 cm2). The orifice area measured smaller (0.54 ± 0.31 cm2, P < 0.001) by 3‐D volume‐rendered echocardiography. Bland‐Altmann analysis indicated that for 3‐D anyplane echocardiography, the mean difference from Doppler and 2‐D echocardiography was − 0.04 ± 0.24 cm2 and − 0.06 ± 0.23 cm2, respectively. For 3‐D volume‐rendered echocardiography, the mean difference was −0.23 ± 0.24 cm2 and − 0.25 ± 0.26 cm2, respectively. In the subgroup with good resolution in the 3‐D dataset, close limits of agreement were obtained between 3‐D echocardiography and each of the reference methods, while the subgroup with poor resolution showed wide limits of agreement. In conclusion, planimetry of the stenotic aortic orifice by 3‐D volume‐rendered echocardiography is feasible but tends to underestimate the orifice area. Three‐dimensional anyplane echocardiography shows better agreement with the reference methods. Accuracy is influenced strongly by the structural resolution of the stenotic orifice in the 3‐D dataset.
Atypical Bland‐White‐Garland Syndrome: A 58‐Year‐Old Woman with Stenosis of the Pulmonary Origin of the Left Coronary Artery
Authors: Wild Philipp S. , Stahl Fabian , Gunkel Oliver , Zotz Rainer J.
Published: July 2003

The anomalous origin of the left coronary artery from the main pulmonary trunk (also known as Bland‐White‐Garland syndrome) is a rare congenital malformation that occurs in 0.4% of patients with cardiac anomalies. We present an adult case (a 58‐year‐old woman) of atypical Bland‐White‐Garland syndrome. The patient displayed a stenosis at the ostium of the anomalous origin of the left coronary artery and an aortopulmonary fistula. Using conventional angiography, it was not possible to differentiate between an anomalous origin of the pulmonary coronary artery and total stenosis of the left main coronary artery in combination with a pulmonary fistula. However, transesophageal echocardiography (TEE) succeeded in making this differential diagnosis. Conclusion: If there is subtotal or total occlusion, TEE can be used for detection of coronary vessel morphology, particularly in cases of coronary anomalies.
Transesophageal Echocardiographic Identification of Thrombus Producing Obstruction of Left Pulmonary Artery Descending Lobar Branches and Bronchial Artery Dilatation
Authors: Kang Seung‐Wan , Nekkanti Rajasekhar , Nanda Navin C. , Gupta Himanshu
Published: July 2003

We report an elderly patient in whom a thrombus in the distal left pulmonary artery was shown by transesophageal echocardiography to extend and produce obstruction of the descending lobar branches as well as dilatation of the left bronchial artery.
The Rupture of Periaortic Infective Aneurysm into the Left Atrium and the Left Ventricular Outflow Tract: Preoperative Diagnosis by Transthoracic Echocardiography
Authors: Lastowiecka Ewa , Biederman Andrzej , Szajewski Tomasz , Rydlewska‐Sadowska Wanda
Published: July 2003

We present a rare complication of infective endocarditis, perforated periaortic abscess with fistulous communication between the aortic root, the left atrium, and the left ventricular outflow tract. Preoperative transthoracic echocardiographic diagnosis was confirmed intraoperatively. The patient was treated successfully by aortic homograft implantation.
Use of Cyclic Variation of Integrated Backscatter to Assess Contractile Reserve and Myocardial Viability in Chronic Ischemic Left Ventricular Dysfunction
Authors: Yuda Satoshi , Dart Jared , Najos Ofelia , Marwick Thomas H.
Published: July 2003

The detection of viable myocardium has important implications for management, but use of stress echocardiography to detect this is subjective and requires exposure to dobutamine. We investigated whether cyclic variation (CV) of integrated backscatter (IB) from the apical views could provide a resting study for detection of contractile reserve (CR) and prediction of myocardial viability in 27 patients with chronic ischemic left ventricular (LV) dysfunction. Repeat echocardiography was performed after 6.7 ± 3.8 months of follow‐up; 14 patients underwent revascularization and 13 were treated medically. Using a standardized dobutamine echocardiography (DbE) protocol, images from three apical views were acquired at 80–120 frames/sec at rest and during stress. CR was identified if improvement of wall motion was observed at low dose (5 or 10 μg/kg/min) DbE. Myocardial viability was characterized by improvement at follow‐up echocardiography in patients with revascularization. CVIB at rest and low dose dobutamine were assessed in 194 segments with resting asynergy (severe hypokinesis or akinesis), of which 88 (45%) were in patients who underwent revascularization. Of these, CVIB could be measured in 190 (98%) segments at rest and 185 (95%) at low dose dobutamine. Sixty‐two (33%) segments had CR during low dose DbE and 50 (57%) segments showed wall‐motion recovery (myocardial viability) at follow‐up echocardiography. Segments with CR had significantly higher CVIB at rest (P < 0.001) and low dose dobutamine (P < 0.005) than segments without CR. Using optimal thresholds of CVIB (> 8.2 dB) at rest, the accuracy of CVIB for detecting CR was 70%. Compared with nonviable segments, viable segments had significantly higher CVIB at rest (P < 0.001) and low dose dobutamine (P < 0.001). Using optimal thresholds of CVIB (>5.3 dB) at rest, the accuracy of CVIB for detecting myocardial viability was 85%, which was higher than that in conventional DbE (62%, P < 0.01). Thus, assessment of CVIB from the apical views is a feasible and accurate tool for detecting CR and predicting myocardial viability in chronic LV dysfunction.