An atypical presentation of a mesenteric Meckel's diverticulum in a 7-year-old warmblood mare: case report.

BACKGROUND: Meckel's diverticula are a rare cause of small intestinal strangulation, diagnosed at laparotomy or necropsy. This congenital anomaly of the gastrointestinal tract originates from a remnant of the vitelline duct. In reported equine cases, they present as a full-thickness diverticulum on the antimesenteric border of the distal jejunum or proximal ileum. CASE PRESENTATION: On laparotomy a Meckel's diverticulum positioned at the mesenteric side was found to be the cause of small intestinal strangulation. This position is very uncommon and to the best knowledge of the authors there is no unambiguous description of another case. CONCLUSIONS: Meckel's diverticula should be on the list of differential diagnoses in cases of small intestinal strangulation. As in humans, equine Meckel's diverticula can have the standard antimesenteric as well as a more exceptional mesenteric location. This case adds to the series of anecdotal reports of anomalies with regard to Meckel's diverticula in the horse.

Dietary beta-carotene, vitamin C and E intake and breast cancer risk in the European Prospective Investigation into Cancer and Nutrition (EPIC).

So far, studies on dietary antioxidant intake, including beta-carotene, vitamin C and vitamin E, and breast cancer risk are inconclusive. Thus, we addressed this question in the European Prospective Investigation into Cancer and Nutrition. During a median follow-up time of 8.8 years, 7,502 primary invasive breast cancer cases were identified. Cox proportional hazard models were used to estimate hazard ratios (HR) and 95% confidence intervals (CI). All analyses were run stratified by menopausal status at recruitment and, additionally, by smoking status, alcohol intake, use of exogenous hormones and use of dietary supplements. In the multivariate analyses, dietary intake of beta-carotene, vitamin C and E was not associated with breast cancer risk in premenopausal [highest vs. lowest quintile: HR, 1.04 (95% CI, 0.85-1.27), 1.12 (0.92-1.36) and 1.11 (0.84-1.46), respectively] and postmenopausal women [0.93 (0.82-1.04), 0.98 (0.87-1.11) and 0.92 (0.77-1.11), respectively]. However, in postmenopausal women using exogenous hormones, high intake of beta-carotene [highest vs. lowest quintile; HR 0.79 (95% CI, 0.66-0.96), P (trend) 0.06] and vitamin C [0.88 (0.72-1.07), P (trend) 0.05] was associated with reduced breast cancer risk. In addition, dietary beta-carotene was associated with a decreased risk in postmenopausal women with high alcohol intake. Overall, dietary intake of beta-carotene, vitamin C and E was not related to breast cancer risk in neither pre- nor postmenopausal women. However, in subgroups of postmenopausal women, a weak protective effect between beta-carotene and vitamin E from food and breast cancer risk cannot be excluded.

[The interdisciplinary treatment of Merkel cell carcinoma: a retrospective case analysis and review of the current literature]. / Interdisziplinäre Behandlung des Merkelzellkarzinoms: Eine retrospektive Fallanalyse und aktuelle Literaturübersicht.

BACKGROUND: Merkel cell carcinoma is a rare, -aggressive, neuroendocrine malignancy of the skin. Over the period from June 2005 to January 2007 (18 months) 7 Merkel cell carcinomas in different tumour stages and localisations were treated in our department for hand, plastic and reconstructive surgery. Given an incidence rate of approximately 0.1-0.4 per 100 000 inhabitants, this accumulation of cases is considerably higher than would be statistically expected. This led us to analyse our cases retrospectively and provided the opportunity to discuss the treatment modalities on the basis of the current literature. PATIENTS AND METHODS: All patients were referred to our clinic after incomplete tumour excision and histopathological diagnosis elsewhere, for further surgical treatment. Two female and 5 male patients, aged 63 to 83 years, were treated. The patients' data were collected and analysed retrospectively. RESULTS: In all cases an R0 resection could be achieved, with safety resection margins between 1.5 cm and 4.5 cm. The reconstruction of the resulting defects was achieved by skin transplants in six cases and a primary suture in one case. All patients received adjuvant radiotherapy. Furthermore, three patients received adjuvant chemotherapy due to metastases of the progressed Merkel cell carcinoma. Three patients remained free of recurrence and in two patients a progression of the tumour disease could be impeded, whilst two patients succumbed to their disease. CONCLUSIONS: In the case of an early diagnosis, an R0 resection can usually be achieved. A safety resection margin of a minimum of 1.5 cm in the face and 3 cm for localisation on the extremities should be obtained. To reduce the local recurrence rate, adjuvant radiotherapy should be conducted. The benefit from chemotherapy still remains unclear.

Severe respiratory dysfunction in a patient with relapsing polychondritis: should we treat the saddle nose deformity?

SUMMARY: We report a case of surgical correction of a saddle nose deformity, causing severe ventilation restrictions in a 42-year-old man diagnosed with relapsing polychondritis. Relapsing polychondritis is an autoimmune disorder, in which antibodies to type II collagen cause an inflammatory destruction of cartilage. If septal cartilage of the nose is involved, destruction leads to collapse of the dorsum of the nose, causing a saddle nose deformity. Patients suffer from a ventilation disorder of varying degree depending on the response to or onset of immunosuppressive therapy. In the described patient, the destruction of the nasal septum, in addition to unstable tracheal cartilage, caused a severe restriction in ventilation, with total collapse of the internal nasal valves during forced inspiration. To improve the function of the external airways the patient underwent surgery to reconstruct the nasal septum. Although cartilage grafts are the state of the art to reconstruct the nasal septum, we used a bone graft from the iliac crest, because the autoimmune polychondritis precludes cartilage grafting due to expected cartilage destruction. At follow up 2 years postoperatively no signs of bone resorption or deterioration of the improved airway were observed. We conclude that the use of bone grafts is a promising method to restore and improve ventilation disorders caused by a saddle nose deformity in relapsing polychondritis.

PKA- and PKC-dependent regulation of angiopoietin 2 mRNA in human granulosa lutein cells.

New blood vessels develop from preexisting vessels in response to growth factors or hypoxic conditions. Recent studies have shown that angiopoietin 2 (ANGPT-2) plays an important role in the modulation of angiogenesis and vasculogenesis in humans and mice. The signaling pathways that lead to the regulation of ANGPT-2 are largely unclear. Here, we report that protein kinase C and protein kinase A activators (ADMB, 8-Cl-cAMP) increased the mRNA levels of ANGPT-2 in human Granulosa cells, whereas PKC and PKA Inhibitors (Rp-cAMP, GO 6983) decreased markedly the level of ANGPT-2 mRNA. Due to varying specificity of the modulators for certain protein kinases subunits, we conclude that the conventional PKCs, but not PKC alpha and beta1, the atypical PKCs and the PKA I, are involved in the regulation of ANGPT-2. These findings may help to explain the role of both PKA and PKC dependent signaling cascades in the regulation of ANGPT-2 mRNA.

Digital constriction bands in pseudoainhum: morphological radiographic, and histological analysis.

Pseudoainhum is a rare condition of unknown etiology that produces digital constricting rings, most commonly on the small fingers. A thorough discussion of pseudoainhum in the plastic surgical literature is apparently lacking at this time. The authors describe the gross morphology, radiographic and laboratory features, and surgical pathology of the disease, and provide well-defined guidelines for its treatment.

Composite grafting and hyperbaric oxygen therapy in pediatric nasal tip reconstruction after avulsive dog-bite injury.

It is estimated that more than four million people are bitten by dogs in the United States each year. The majority of such injuries are minor, and their treatment does not usually require surgical consultation. However, the authors report a case in which a Rottweiler inflicted a mutilating nasal tip/alar rim avulsion on a 5-year-old boy. They report their experience with immediate reconstruction of the nasal defect using a large ipsilateral auricular cartilage composite graft (crus helix). Adjunctive hyperbaric oxygen therapy (without sedation or anesthesia) was used to maximize the stimulus for graft revascularization. Reconstructive goals were achieved while avoiding the need for a central facial donor site defect.

The evolution of velopharyngeal imaging.

Currently, functional visualization of the velopharynx requires tests that are either invasive (endoscopy) or that impart ionizing radiation (speech videofluoroscopy). The overall intrusiveness of endoscopy may limit its clinical utility, especially in young children. As a resut of growing awareness of the long-range effects of radiation exposure associated with X-ray imaging, radiographic research on subjects and studies not judged to be clinically necessary have been all but abandoned. The static nature of lateral radiographs precludes temporal assessment, and the two dimensionally of images derived from both of these diagnostic modalities may limit understanding of spatial anatomic relationships and may preclude quantitative analysis. The need for a noninvasive, rapid, and easily repeatable method for examination of the velopharynx has fomented the innovative application of existing technologies, especially magnetic resonance imaging. We present an updated overview of techniques for imaging the velopharyngeal mechanism, with a focus on residual velopharyngeal dysfunction after initial palatoplasty. We provide a comprehensive perspective of the role of currently available instrumentation, summarize the work in our center regarding the technological developments of magnetic resonance imaging, and speculate about future applications of magnetic resonance imaging systems for evaluation of velopharyngeal dysfunction. The limitations of each of these measures discussed are emphasized.

Treatment of chronic pain syndrome.

We address the psychological and pharmacological treatment of chronic pain syndrome. The commonly used pain management protocols in each area are described. Psychological management procedures that are covered include relaxation methods, patient education, cognitive therapy techniques, and patient compliance enhancement. Concerning pharmacological management, the World Health Organization ladder analgesic guidelines are described, as well as adjunct medications, such as antidepressants, anxiolytics, and hypnotics.

Confirmation of linkage of Van der Woude syndrome to chromosome 1q32: evidence of association with STR alleles suggests possible unique origin of the disease mutation.

Van der Woude syndrome (VWS) is an autosomal dominant craniofacial disorder with high penetrance and variable expression. Its clinical features are variably expressed, but include cleft lip and/or cleft palate, lip pits and hypodontia. All VWS families studied to date map the disease gene to a < 2 cM region of chromosome 1q32, with no evidence of locus heterogeneity. The aim of this study is to refine the localization of the VWS gene and to further assess possible heterogeneity. We analyzed four multiplex VWS families. All available members were clinically assessed and genotyped for 19 short tandem repeat markers on chromosome 1 in the VWS candidate gene region. We performed two-point and multipoint limit of detection (LOD) score analyses using a high penetrance autosomal dominant model. All families showed positive LOD scores without any recombination in the candidate region. The largest two-point LOD score was 5.87. Our assay method for short tandem repeat (STR) markers provided highly accurate size estimation of marker allele fragment sizes, and therefore enabled us to determine the specific alleles segregating with the VWS gene in each of our four families. We observed a striking pattern of STR allele sharing at several closely linked loci among our four Caucasian VWS families recruited at three different locations in the US. These results suggest the possibility of a unique origin for a mutation responsible for many or most cases of VWS.

Surgical treatment of pseudosyndactyly of the hand in epidermolysis bullosa: histological analysis of an acellular allograft dermal matrix.

Recessive dystrophic epidermolysis bullosa is an inherited mechanobullous disorder of skin and mucous membranes. The most striking clinical characteristic of the disease is the formation of blisters following trivial trauma. Repeated cycles of blistering and scarring result in gradual encasement of the hand in an epidermal "cocoon." The authors treated an 11-year-old boy with recessive dystrophic epidermolysis bullosa who presented with hand contractures and interdigital pseudosyndactyly. Treatment included release of contractures and application of a biosynthetic dermal analog. This report is a histological analysis of the dermal matrix 1 year after initial placement of the allograft. Fibroblasts repopulating the dermal allograft had a normal synthetic phenotype and lacked the myofibroblastic features seen in the ungrafted control biopsy. Collagen and elastin in the repopulated dermal allograft had normal dermal orientation and maturity in contrast to the sparse, immature collagen and lack of elastin compared with the dermis of an ungrafted control region. Results of this histological study indicate that treatment of recessive dystrophic epidermolysis bullosa with an acellular human dermal allograft may restore some features of normal dermal architecture. Although the initial results are encouraging, longer follow-up is required before definitive conclusions can be made.

Long-term stability of postpalatoplasty perceptual speech ratings: a prospective study.

This prospective study was undertaken to assess the long-term stability of velopharyngeal perceptual speech ratings of patients with repaired cleft palate. All patients were evaluated and managed at the Cleft Palate and Craniofacial Deformities Institute, St. Louis Children's Hospital. Patients alternately received palatoplasty with or without intravelar veloplasty. Two senior surgeons standardized their operative procedures and performed or supervised directly all operations. Perceptual speech and language evaluations were conducted by the same experienced speech pathologist when the children were 6 years old and 12 years or older. Data were analyzed from the 28 patients available for long-term follow-up. The intravelar veloplasty (N = 14) and nonintravelar veloplasty (N = 14) groups were similar with respect to cleft anatomy and mean age at palatoplasty and at the second perceptual speech evaluation. Evaluation of the 12-year-old and older ratings indicated that the overwhelming majority of patients improved or maintained clinical stability in perceptual ratings of velopharyngeal function. When assessing direction and magnitude of change (i.e., incremental improvement vs. deterioration), the intravelar veloplasty and nonintravelar veloplasty groups had a similar distribution of perceptual speech ratings at both the 6-year and 12-year or older speech evaluations. Results were consistent with previously published data from our center, that the intravelar veloplasty procedure did not affect demonstrably the incidence of postpalatoplasty auditory perceptual symptoms of velopharyngeal dysfunction.

Sphincter pharyngoplasty for the surgical management of speech dysfunction associated with velocardiofacial syndrome.

There are no reports in the literature that document the effectiveness of sphincter pharyngoplasty as a surgical alternative to pharyngeal flap for management of velopharyngeal dysfunction in patients with velocardiofacial syndrome. A retrospective review of patients with velocardiofacial syndrome was undertaken at our tertiary cleft care centre. All patients were managed between 1984 and 1996 at the Cleft Palate and Craniofacial Deformities Institute, St Louis Children's Hospital. Subjects (n = 19) underwent velopharyngeal surgical management on the basis of perceptual speech evaluations and instrumental assessments of inadequate velopharyngeal closure. All patients had a molecular diagnosis of velocardiofacial syndrome based on fluorescent in situ hybridisation analysis of peripheral blood lymphocytes and independent evaluation by a medical geneticist. Surgical outcome was classified as successful if perceptual speech assessment indicated elimination of hypernasality, nasal emission and turbulence, and instrumental assessment indicated 100% velopharyngeal closure. Results showed that 18 of 19 patients were managed successfully with sphincter pharyngoplasty. Our data corroborate that sphincter pharyngoplasty is a reasonable alternative to pharyngeal flap in patients with velopharyngeal dysfunction secondary to velocardiofacial syndrome.

Comparative study of the pharmacodynamic and pharmacologic effects of Betaseron and AVONEX.

The aim of this 1 week study was to compare the biologic effects induced by Betaseron and AVONEX using their approved dose, route, and schedule. Sixteen healthy volunteers were randomly assigned to receive either a single i.m. dose of AVONEX (6 million International Units [MIU]) or, every other day s.c. doses of Betaseron (8 MIU). Common side effects associated with interferon-beta (IFN-beta) treatment and biologic response parameters (neopterin, beta2-microglobulin, interleukin-10 [IL-10], and MxA protein levels in blood) were measured. Ibuprofen was administered to all subjects throughout the study. Fever, chills, and myalgia occurred most frequently and with the greatest severity between 6 and 12 h after the first dose of either IFN-beta. Despite the additional dosing of subjects in the Betaseron group, the incidence, duration, and severity of the side effects were not significantly different from those in the AVONEX group. Biologic response parameters reached similar maximum concentrations in both treatment groups. In the Betaseron group, neopterin and beta2-microglobulin levels remained significantly greater than baseline throughout the 7 day study, whereas those in the AVONEX group were elevated only through day 5. Betaseron treatment significantly increased IL-10 levels above baseline, but AVONEX treatment did not. The overall induction of neopterin, beta2-microglobulin, and IL-10 (as measured by area under the concentration-time curve) was significantly greater in the Betaseron group than the AVONEX group (p = 0.031). The results of this study demonstrate that the approved Betaseron dosing regimen, in combination with ibuprofen use, provided a significantly greater and more consistently elevated biologic response compared with that of AVONEX and did so with a side effects profile comparable to that of once a week AVONEX dosing.

Simultaneous palpation of the craniosacral rate at the head and feet: intrarater and interrater reliability and rate comparisons.

BACKGROUND AND PURPOSE: The main purpose of this study was to determine the interrater and intrarater reliability of measurements obtained during palpation of the craniosacral rate at the head and feet. Palpated craniosacral rates of head and feet measured simultaneously were also compared. Subjects. Twenty-eight adult subjects and 2 craniosacral examiners participated in the study. METHODS: A within-subjects repeated-measures design was used. A standard cubicle privacy curtain, hung over the subject's waist, was used to prevent the examiners from seeing each other. RESULTS: Interrater intraclass correlation coefficients (ICCs) were .08 at the head and .19 at the feet. Intrarater ICCs ranged from .18 to .30. Craniosacral rates simultaneously palpated at the head and feet were different. CONCLUSION AND DISCUSSION: The results did not support the theories that underlie craniosacral therapy or claims that craniosacral motion can be palpated reliably.