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Sample records for dentin dysplasia type

  1. Dentin dysplasia type I : Five cases within one family

    NARCIS (Netherlands)

    Kalk, WWI; Batenburg, RHK; Vissink, A

    Five cases of dentin dysplasia type I within one family are described. Clinically and radiologically, such patients are characterized by a delayed eruption pattern, opacity of the incisional margins, hypermobility of the teeth, short and defective roots, and obliterated pulp chambers. A conservative

  2. Dentinal Dysplasia Type I: A Case Report with a 6-Year Followup

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    Sezin Ozer

    2013-01-01

    Full Text Available Introduction. Dentin dysplasia is a rare disturbance of dentin formation characterized by normal enamel but atypical dentin formation with abnormal pulpal morphology that is inherited as an autosomal pulpal morphology. Case Presentation. A 7-year-old female who had problems in chewing function was referred to Oral and Maxillofacial Surgery Department at the Faculty of Dentistry in Ondokuz Mayıs University. In the radiographic examination, it was determined that some of the unerupted permanent teeth of the patient had short, blunted, and malformed roots with obliterated pulp chambers, although the bone below the teeth showed well-defined margins. This unusual case of generalized short roots presents a case demonstrating both classic and atypical features of dentinal dysplasia type I (DDI in the mixed and permanent dentitions. Conclusion. There are still many issues in the diagnosis and management of patients with dentin dysplasia. Early diagnosis, clinical and radiographic findings, as well as treatment of this condition and the initiation of effective preventive strategies may help prevent or delay loss of dentition.

  3. Implant-based oral rehabilitation of a variant model of type I dentinal dysplasia: A rare case report

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    Sowmya Nettem

    2014-01-01

    Full Text Available Dentin dysplasia is an exceptionally rare, autosomal-dominant, hereditary condition, primarily characterized by defective dentin formation affecting both the deciduous and permanent dentitions. The etiology remains imprecise to date, in spite of the numerous hypotheses put forward and the constant updates on this condition. This case report of type I dentin dysplasia exhibits radiographic findings that are unique and diverse from the classical findings of various subtypes of this disease reported to date. This article also depicts the implant-based oral rehabilitation of the young patient diagnosed with this variant model of dentin dysplasia type I. Early diagnosis and implementation of this preventive and curative therapy is vital for avoiding premature exfoliation of deciduous and permanent dentition and the associated residual ridge resorption, thereby overcoming functional and esthetic deficits and ensuring protection of the remaining dentition from further harm.

  4. Dentin dysplasia type I with pyogenic granuloma in a 12-year-old girl

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    Nirmala SVSG

    2009-06-01

    Full Text Available This paper presents a case of dentin dysplasia, a rarely reported disorder that is also associated with pyogenic granuloma in a 12-year-old girl. The case presented as excessively mobile teeth that appeared radiographically as rootless teeth and also as a soft tissue lesion in the right maxillary anterior region.

  5. Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia

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    MacKie Iain

    2008-11-01

    Full Text Available Abstract The hereditary dentine disorders, dentinogenesis imperfecta (DGI and dentine dysplasia (DD, comprise a group of autosomal dominant genetic conditions characterised by abnormal dentine structure affecting either the primary or both the primary and secondary dentitions. DGI is reported to have an incidence of 1 in 6,000 to 1 in 8,000, whereas that of DD type 1 is 1 in 100,000. Clinically, the teeth are discoloured and show structural defects such as bulbous crowns and small pulp chambers radiographically. The underlying defect of mineralisation often results in shearing of the overlying enamel leaving exposed weakened dentine which is prone to wear. Currently, three sub-types of DGI and two sub-types of DD are recognised but this categorisation may change when other causative mutations are found. DGI type I is inherited with osteogenesis imperfecta and recent genetic studies have shown that mutations in the genes encoding collagen type 1, COL1A1 and COL1A2, underlie this condition. All other forms of DGI and DD, except DD-1, appear to result from mutations in the gene encoding dentine sialophosphoprotein (DSPP, suggesting that these conditions are allelic. Diagnosis is based on family history, pedigree construction and detailed clinical examination, while genetic diagnosis may become useful in the future once sufficient disease-causing mutations have been discovered. Differential diagnoses include hypocalcified forms of amelogenesis imperfecta, congenital erythropoietic porphyria, conditions leading to early tooth loss (Kostmann's disease, cyclic neutropenia, Chediak-Hegashi syndrome, histiocytosis X, Papillon-Lefevre syndrome, permanent teeth discolouration due to tetracyclines, Vitamin D-dependent and vitamin D-resistant rickets. Treatment involves removal of sources of infection or pain, improvement of aesthetics and protection of the posterior teeth from wear. Beginning in infancy, treatment usually continues into adulthood with a

  6. Jansen type of spondylometaphyseal dysplasia

    International Nuclear Information System (INIS)

    Campbell, J.B.; Kozlowski, K.; Lejman, T.; Sulko, J.

    2000-01-01

    Metaphyseal dysplasia, type Jansen (JMD), is a rare skeletal dysplasia with characteristic radiographic abnormalities. Of the various types of metaphyseal dysplasia, JMD shows the most severe alteration in metaphyseal architecture. All of the long tubular bones, including those of the hands and feet, show metaphyseal irregularity with a fragmented appearance and slight widening. The adjacent physes are abnormally widened, while the epiphyses tend to be slightly enlarged, rounded but otherwise normal. The spine in infancy and childhood usually appears normal. This report describes a young girl with metaphyseal changes typical of JMD except for the hands and feet, which appeared normal. She also showed very unusual abnormalities of the spine. This appears, therefore, to represent a unique osteochondrodysplasia for which we propose the term spondylometaphyseal dysplasia, type Jansen. (orig.)

  7. Interradicular dentin dysplasia associated with amelogenesis imperfecta with taurodontism or trichodentoosseous syndrome: A diagnostic dilemma

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    Veda Hegde

    2014-01-01

    Full Text Available Amelogenesis imperfecta is a hereditary disorder with diverse clinical presentation, where enamel is the tissue that is primarily affected either quantitatively or qualitatively. Hypomaturation/hypoplastic amelogenesis imperfecta with taurodontism is a rare variant of amelogenesis imperfecta which is often confused with trichodentoosseous syndrome. We report a rare case of hereditary enamel defect with taurodontism associated with interradicular dentin dysplasia.

  8. Interradicular dentin dysplasia associated with amelogenesis imperfecta with taurodontism or trichodentoosseous syndrome: a diagnostic dilemma.

    Science.gov (United States)

    Hegde, Veda; Srikanth, K

    2014-01-01

    Amelogenesis imperfecta is a hereditary disorder with diverse clinical presentation, where enamel is the tissue that is primarily affected either quantitatively or qualitatively. Hypomaturation/hypoplastic amelogenesis imperfecta with taurodontism is a rare variant of amelogenesis imperfecta which is often confused with trichodentoosseous syndrome. We report a rare case of hereditary enamel defect with taurodontism associated with interradicular dentin dysplasia.

  9. Acro-mesomelic dysplasia - a new type

    International Nuclear Information System (INIS)

    Brahimi, L.; Bacha, L.; Kozlowski, K.; Massen, R.; Zenati, M.

    1988-01-01

    Two siblings who represent a new type of acro-mesomelic dysplasia are reported. The unique pattern of the acro-mesomelic hypoplastic/dysplastic changes allows us to designate as a new syndrome. (orig.)

  10. A new type of spondylo-metaphyseal dysplasia - Algerian type

    International Nuclear Information System (INIS)

    Kozlowski, K.; Bacha, L.; Massen, R.; Ayati, M.; Sator, S.; Brahimi, L.

    1988-01-01

    A new, dominantly inherited, severe form of spondylometaphyseal dysplasia in five members of an Algerian family is reported. Another child, not investigated, was also probably affected. The disease is characterised by a unique clinical and radiological set of features: dwarfism, genu valgum deformity, progressive kypho-scoliosis, wrist deformity, myopia and severe metaphyseal dysplasia, with moderate spinal changes and minimal changes in the hands and feet. In view of the geographical localisation of the disorder and the anatomical distribution we propose the name Algerian type of spondylo-metaphyseal dysplasia. (orig.)

  11. Multiple Epiphyseal Dysplasia (MED: A Rare Type of Skeletal Dysplasia

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    Mohammad Imnul Islam

    2012-06-01

    Full Text Available Multiple epiphyseal dysplasia (MED is a congenital disorder of skeletal development that primarily affects the ends of long bones, causing progressive joint and bone inflammation and short stature. Mutations in several genes are responsible for pathogenesis of this disease. We are reporting a case of MED who presented with the complaints of multiple swelling of the joints which was associated with pain during movement for last seven years. The patient had flexion deformity of all the affected joints along with restriction of movement. These were associated with kyphosis, pectus carnitum, knock-knee and short stature. Radiological findings were suggestive of MED. Counseling was done with the parents regarding the etiology, progression and outcome of the disease.DOI: http://dx.doi.org/10.3329/bsmmuj.v5i1.11025 BSMMU J 2012; 5(1:57-60 

  12. Bond strength of resin modified glass ionomer cement to primary dentin after cutting with different bur types and dentin conditioning

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    Rebeca Di Nicoló

    2007-10-01

    Full Text Available The aim of this in vitro study was to evaluate the effect of different bur types and acid etching protocols on the shear bond strength (SBS of a resin modified glass ionomer cement (RM-GIC to primary dentin. Forty-eight clinically sound human primary molars were selected and randomly assigned to four groups (n=12. In G1, the lingual surface of the teeth was cut with a carbide bur until a 2.0-mm-diameter dentin area was exposed, followed by the application of RM-GIC (Vitremer - 3M/ESPE prepared according to the manufacturer's instructions. The specimens of G2, received the same treatment of G1, however the dentin was conditioned with phosphoric acid. In groups G3 and G4 the same procedures of G1 and G2 were conducted respectively, nevertheless dentin cutting was made with a diamond bur. The specimens were stored in distilled water at 37ºC for 24h, and then tested in a universal testing machine. SBS. data were submitted to 2-way ANOVA (= 5% and indicated that SBS values of RM-GIC bonded to primary dentin cut with different burs were not statistically different, but the specimens that were conditioned with phosphoric acid presented SBS values significantly higher that those without conditioning. To observe micromorphologic characteristics of the effects of dentin surface cut by diamond or carbide rotary instruments and conditioners treatment, some specimens were examined by scanning electron microscopy. Smear layer was present in all specimens regardless of the type of rotary instrument used for dentin cutting, and specimens etched with phosphoric acid presented more effective removal of smear layer. It was concluded that SBS of a RM-GIC to primary dentin was affected by the acid conditioning but the bur type had no influence.

  13. Altered inhibition in Tuberous Sclerosis and Type IIb cortical dysplasia

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    Talos, Delia M.; Sun, Hongyu; Kosaras, Bela; Joseph, Annelise; Folkerth, Rebecca D.; Poduri, Annapurna; Madsen, Joseph R.; Black, Peter M.; Jensen, Frances E.

    2012-01-01

    Objective The most common neurological symptom of tuberous sclerosis complex (TSC) and focal cortical dysplasia (FCD) is early-life refractory epilepsy. As previous studies have shown enhanced excitatory glutamatergic neurotransmission in TSC and FCD brains, we hypothesized that neurons associated with these lesions may also express altered GABAA receptor (GABAAR)-mediated inhibition. Methods Expression of the GABAAR subunitsα1 and α4, the Na+-K+-2Cl− (NKCC1), and the K+−Cl− (KCC2) transporters in human TSC and FCD Type II specimens were analyzed by Western blot and double label immunocytochemistry. GABAAR responses in dysplastic neurons from a single case of TSC were measured by perforated-patch recording and compared to normal-appearing cortical neurons from a non-TSC epilepsy case. Results TSC and FCD Type IIb lesions demonstrated decreased expression of the GABAAR α1, increased NKCC1 and decreased KCC2 levels. In contrast, FCD Type IIa lesions showed decreased α4, and increased expression of both NKCC1 and KCC2 transporters. Patch clamp recordings from dysplastic neurons in acute slices from TSC tubers demonstrated excitatory GABAAR responses that were significantly attenuated by the NKCC1 inhibitor bumetanide, in contrast to hyperpolarizing GABAAR-mediated currents in normal neurons from non-TSC cortical slices. Interpretation Expression and function of GABAARs in TSC and FCD IIb suggests the relative benzodiazepine insensitivity and more excitatory action of GABA compared to FCD IIa. These factors may contribute to resistance of seizure activity to anticonvulsants that increase GABAergic function, and may justify add-on trials of the NKCC1 inhibitor bumetanide for the treatment of TSC and FCD Type IIb related epilepsy. PMID:22447678

  14. A new syndrome of 'spondylo-epi-metaphyseal dysplasia: mixed type''

    International Nuclear Information System (INIS)

    Sharma, B.G.

    2003-01-01

    A new type of rare bone dysplasia is described, which shares some common features with spondylo-meta-epiphyseal dysplasia: short limb-abnormal calcification type and lethal metatropic dysplasia. Besides these features, the present case has some additional unusual features. Facial malformation was very obvious and of a different type. The nose and nares were completely flattened. Hypertrophied acetabular bones, round densities on the ilia, premature ossification of many epiphyses and carpal bones, curvilinear calcifications in some joints, fusion of the ischiopubic rami, calcification of many costal cartilages and thick sclerotic base of the skull were a few of the significant findings. On the basis of the clinical and radiological features, the condition has been named ''spondylo-epi-metaphyseal dysplasia: mixed type''. (orig.)

  15. Observer variability in the assessment of type and dysplasia of colorectal adenomas, analyzed using kappa statistics

    DEFF Research Database (Denmark)

    Jensen, P; Krogsgaard, M R; Christiansen, J

    1995-01-01

    . The kappa values for Observer A vs. B and Observer C vs. B were 0.3480 and 0.3770, respectively (both type and dysplasia). Values for type were better than for dysplasia, but agreement was only fair to moderate. CONCLUSION: The interobserver agreement was moderate to almost perfect, but the intraobserver...... agreement was only fair to moderate. A simpler classification system or a centralization of assessments would probably increase kappa values....... of adenomas were assessed twice by three experienced pathologists, with an interval of two months. Results were analyzed using kappa statistics. RESULTS: For agreement between first and second assessment (both type and grade of dysplasia), kappa values for the three specialists were 0.5345, 0.9022, and 0...

  16. Unusual manifestations of ectodermal dysplasia-syndactyly syndrome type I in two Yemeni siblings.

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    Mohammad, Alshami

    2015-01-15

    Ectodermal dysplasias (EDs) are a group of genodermatoses characterized by malformations of tissues derived from the ectoderm, including the skin, its appendages (hair, nails, sweat glands), teeth, and the breasts. Ectodermal dysplasia syndactyly syndrome (EDSS) is a rare, newly described type of ED involving syndactyly. We report 2 Yemeni siblings with typical EDSS manifestations, including bilateral, partial cutaneous syndactyly of the fingers and toes; sparse, coarse, brittle scalp hair, eyebrows, and eyelashes; and conical, widely spaced teeth with enamel notches. In addition, the siblings presented with other features hitherto not described for this syndrome, such as adermatoglyphia, onychogryphosis, hypoplastic widely spaced nipples, hypoplastic thumbs, and red scalp hair.

  17. Thanatophoric dysplasia type II with encephalocele and aortic hypoplasia diagnosed in an anatomical specimen

    NARCIS (Netherlands)

    Jap-A-Joe, Simone M. E. A. A.; Oostra, Roelof-Jan; Maas, Mario; Stoker, Jaap; van der Horst, Chantal M. A. M.

    2003-01-01

    A hitherto unknown combination of congenital anomalies was found in an anatomical specimen of a female neonate. External examination and additional CT and MRI studies showed thanatophoric dysplasia type II with cloverleaf skull and concomitant parietal meningoencephalocele and hypoplasia of the

  18. Adult-onset epilepsy in focal cortical dysplasia of Taylor type

    NARCIS (Netherlands)

    Siegel, A. M.; Cascino, G. D.; Elger, C. E.; Devinsky, O.; Laff, R.; Najjar, S.; Sperling, M. R.; LoRusso, G.; Cossu, M.; Urbach, H.; Aronica, E.; Meyer, F. B.; Scheithauer, B. W.; Dubeau, F.; Andermann, F.

    2005-01-01

    Focal cortical dysplasia of Taylor type (FCDT) usually presents with seizures at an early age, whereas adult onset of epilepsy is uncommon. We reviewed the medical records of 213 patients with FCDT. In 21 patients (10%), age at seizure onset ranged from 18 to 55 years (mean 25.3). The outcome of

  19. Bent bone dysplasia (BBD)-FGFR2 type: the radiologic manifestations in early gestation

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    Handa, Atsuhiko; Okajima, Yuka; Kurihara, Yasuyuki [St. Luke' s International Hospital, Department of Radiology, Tokyo (Japan); Izumi, Noriko; Yamanaka, Michiko [St. Luke' s International Hospital, Department of Integrated Women' s Health, Tokyo (Japan)

    2016-02-15

    Bent bone dysplasia-fibroblast growth factor receptor 2 type (BBD-FGFR2) is a recently identified skeletal dysplasia caused by specific FGFR2 mutations, characterized by craniosynostosis and prenatal bowing of the long bones. Only a few cases have been published. We report an affected fetus terminated at 21 weeks of gestation. The clinical and radiologic manifestations mostly recapitulate previous descriptions; however we suggest additional hallmarks of this disorder in early gestation. These hallmarks include distinctive short, thick clavicles and wavy ribs, as well as vertebral bodies that showed striking anteroposterior shortening. Femoral fractures were also present in our case. Although craniosynostosis is a hallmark of the disease, clinicians should be aware that craniosynostosis might not be readily apparent on plain films early in gestation. (orig.)

  20. Familial temporal lobe epilepsy due to focal cortical dysplasia type IIIa.

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    Fabera, Petr; Krijtova, Hana; Tomasek, Martin; Krysl, David; Zamecnik, Josef; Mohapl, Milan; Jiruska, Premysl; Marusic, Petr

    2015-09-01

    Focal cortical dysplasia (FCD) represents a common cause of refractory epilepsy. It is considered a sporadic disorder, but its occasional familial occurrence suggests the involvement of genetic mechanisms. Siblings with intractable epilepsy were referred for epilepsy surgery evaluation. Both patients were examined using video-EEG monitoring, MRI examination and PET imaging. They underwent left anteromedial temporal lobe resection. Electroclinical features pointed to left temporal lobe epilepsy and MRI examination revealed typical signs of left-sided hippocampal sclerosis and increased white matter signal intensity in the left temporal pole. PET examination confirmed interictal hypometabolism in the left temporal lobe. Histopathological examination of resected tissue demonstrated the presence FCD type IIIa, i.e. hippocampal sclerosis and focal cortical dysplasia in the left temporal pole. We present a unique case of refractory mesial temporal lobe epilepsy in siblings, characterized by an identical clinical profile and histopathology of FCD type IIIa, who were successfully treated by epilepsy surgery. The presence of such a high concordance between the clinical and morphological data, together with the occurrence of epilepsy and febrile seizures in three generations of the family pedigree points towards a possible genetic nature of the observed FCD type IIIa. Copyright © 2015 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.

  1. Comparative X-ray morphometry of prenatal osteogenesis imperfecta type 2 and thanatophoric dysplasia: a contribution to prenatal differential diagnosis.

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    Bondioni, Maria Pia; Pazzaglia, Ugo Ernesto; Izzi, Claudia; Di Gaetano, Giuseppe; Laffranchi, Francesco; Baldi, Maurizia; Prefumo, Federico

    2017-11-01

    The purpose of the paper was to assess the morphometric parameters to improve the specificity of the ultrasound (US) signs for the early differential diagnosis between two lethal dysplasias, as thanatophoric dysplasia (TD) and osteogenesis imperfecta type 2 (OI-2). The diaphyseal length and the bowed shape of long bones associated with vertebral body dimension assessment were investigated in a group of 14 pregnancy terminations carried out in the time period 2007-2013. The definitive diagnosis was established after pregnancy termination by means of skeletal standardized X-rays, histopathology and gene analysis. TD and OI-2 long bones were significantly shorter than controls. No significant differences were observed between the two dysplasias. The bowing angle was higher in OI-2; a true angulation or eventually axial displacement was present only in the latter. Furthermore, they did not show any evidence of vertebral collapse. The thanatophoric dysplasia presented less bowed long bones, and never true angulation. The spine was steadily characterized by flattened anterior vertebral bodies. Long bone shortening is not a sufficient and accurate sign for early sonographic differential diagnosis between TD and OI-2. Angled diaphysis, axial diaphyseal displacement and a conserved vertebral body height in the prenatal period support the diagnosis of osteogenesis imperfecta type 2, while moderately regular bowed diaphysis associated with platyspondyly that of thanatophoric dysplasia.

  2. Sphenoid dysplasia in neurofibromatosis type 1: a new technique for repair.

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    Concezio, Di Rocco; Amir, Samii; Gianpiero, Tamburrini; Luca, Massimi; Mario, Giordano

    2017-06-01

    Sphenoid bone dysplasia in neurofibromatosis type 1 is characterized by progressive exophthalmos and facial disfiguration secondary to herniation of meningeal and cerebral structures. We describe a technique for reconstruction of the sphenoid defect apt at preventing or correcting the ocular globe dislocation. After placement of spinal cerebrospinal fluid drainage to reduce intracranial pressure, the temporal pole is posteriorly dislocated extradurally. The greater sphenoid wing defect is identified. A titanium mesh covered by lyophilized dura, modeled in a curved fashion, is interposed between the bone defect and the cerebro-meningeal structures with its convex surface over the retracted temporal pole. The particular configuration of the titanium mesh allows a self-maintaining position due to the pressure exerted by the brain over its convex central part with its lateral margins consequently pushed and self-anchored against the medial and lateral walls of the temporal fossa. Screw fixation is not needed. The technique utilized in four cases proved to be reliable at the long-term clinical and neuroradiological controls (6 to 19 years). Sphenoid bone dysplasia in NF1, resulting in proptosis and exophthalmos, is usually progressive. It can be surgically repaired using a curved titanium mesh with the convexity faced to the temporal pole that is in the opposite fashion from all the techniques previously introduced. When utilized early in life, the technique can prevent the occurrence of the orbital and facial disfiguration.

  3. Ectodermal dysplasias

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    Anhidrotic ectodermal dysplasia; Christ-Siemens-Touraine syndrome; Anondontia; Incontinentia pigmenti ... These resources can provide more information on ectodermal dysplasias: Ectodermal Dysplasia Society -- www.ectodermaldysplasia.org National Foundation for ...

  4. Prognosis of vulvar dysplasia and carcinoma in situ with special reference to histology and types of human papillomavirus (HPV)

    DEFF Research Database (Denmark)

    Junge, Jette; Poulsen, H; Horn, T

    1997-01-01

    is the involvement of the resection borders. The location of the lesion, the degree and type of dysplasia, and the type of HPV seem to play a minor role. Local excision and subsequent intensive control with removal of any visible new lesion probably prevents development of vulvar invasive carcinoma....... were classified according to the WHO (mild, moderate, severe dysplasia or carcinoma in situ) and Toki et al. (1991) (warty, basaloid, combined warty/basaloid or mixed (warty, basaloid and simple). No pure types of Toki (1991) could be demonstrated. There were no differences regarding recurrences in any...... of these groups. HPV DNA was detected in the initial lesions by PCR in 50/56 (89%) (44 with HPV type 16 and 6 with HPV type 33) and by ISH in 23/61 (38%). The same type of HPV could be demonstrated in all first recurrences except in two, where HPV types 33 was shown in specimens harboring HPV type 16...

  5. Effect of immediate and delayed dentin sealing on the fracture strength, failure type and Weilbull characteristics of lithiumdisilicate laminate veneers

    NARCIS (Netherlands)

    Gresnigt, Marco M.M.; Cune, Marco S.; de Roos, Joanne G.; Özcan, Mutlu

    OBJECTIVES: Adhesion on dentin is less reliable than on enamel, which could affect the durability of laminate veneers (LV). Immediate dentin sealing (IDS) is suggested instead of delayed dentin sealing (DDS) to overcome hypersensitivity and prevent debonding from dentin. This study evaluated the

  6. Effects of type I collagen degradation on the durability of three adhesive systems in the early phase of dentin bonding.

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    Lin Hu

    Full Text Available This study was designed to evaluate the effects of type I collagen degradation on the durability of three adhesive systems in the early phase of dentin bonding.Bonded dentin specimens were prepared using three different types of adhesive systems. Micro-tensile bond strength and degradation of collagen were tested before, and after 1 month or 4 months of aging in artificial saliva. The relationship between micro-tensile bond strength and collagen degradation was analyzed by calculating their Pearson's correlation coefficient.Aging induced time-dependent reduction in micro-tensile bond strengths for all the tested adhesive systems, although such reduction for the single-step self-etching adhesive G-Bond (GB was not statistically significant. The bond strength of the two-step self-etching primer adhesive system Clearfil SE Bond (SEB was similar to that of the two-step etch-and-rinse self-priming adhesive system Single Bond 2 (SB, and they were both significantly reduced after one or four months of aging. A negative correlation was found between the degree of collagen degradation and magnitude of micro-tensile bond strength (r = -0.65, p = 0.003. The Pearson's correlation coefficient was 0.426, indicating that 42.6% of the aging-induced reduction in bond strength can be explained by the degradation of collagen.In the early phase of dentin bonding, there was a negative correlation between the degree of collagen degradation and the magnitude of micro-tensile bond strength. The reduction of bond strength was accompanied by the degradation of collagen. These results provide evidence for the causative relationship between the degradation of collagen and the deterioration of dentin-adhesive interface.

  7. Porcine dentin sialoprotein glycosylation and glycosaminoglycan attachments.

    Science.gov (United States)

    Yamakoshi, Yasuo; Nagano, Takatoshi; Hu, Jan Cc; Yamakoshi, Fumiko; Simmer, James P

    2011-02-03

    Dentin sialophosphoprotein (Dspp) is a multidomain, secreted protein that is critical for the formation of tooth dentin. Mutations in DSPP cause inherited dentin defects categorized as dentin dysplasia type II and dentinogenesis imperfecta type II and type III. Dentin sialoprotein (Dsp), the N-terminal domain of dentin sialophosphoprotein (Dspp), is a highly glycosylated proteoglycan, but little is known about the number, character, and attachment sites of its carbohydrate moieties. To identify its carbohydrate attachment sites we isolated Dsp from developing porcine molars and digested it with endoproteinase Glu-C or pronase, fractionated the digestion products, identified fractions containing glycosylated peptides using a phenol sulfuric acid assay, and characterized the glycopeptides by N-terminal sequencing, amino acid analyses, or LC/MSMS. To determine the average number of sialic acid attachments per N-glycosylation, we digested Dsp with glycopeptidase A, labeled the released N-glycosylations with 2-aminobenzoic acid, and quantified the moles of released glycosylations by comparison to labeled standards of known concentration. Sialic acid was released by sialidase digestion and quantified by measuring β-NADH reduction of pyruvic acid, which was generated stoichiometrically from sialic acid by aldolase. To determine its forms, sialic acid released by sialidase digestion was labeled with 1,2-diamino-4,5-methyleneoxybenzene (DMB) and compared to a DMB-labeled sialic acid reference panel by RP-HPLC. To determine the composition of Dsp glycosaminoglycan (GAG) attachments, we digested Dsp with chondroitinase ABC and compared the chromotagraphic profiles of the released disaccharides to commercial standards. N-glycosylations were identified at Asn37, Asn77, Asn136, Asn155, Asn161, and Asn176. Dsp averages one sialic acid per N-glycosylation, which is always in the form of N-acetylneuraminic acid. O-glycosylations were tentatively assigned at Thr200, Thr216 and Thr

  8. Porcine dentin sialoprotein glycosylation and glycosaminoglycan attachments

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    Yamakoshi Fumiko

    2011-02-01

    Full Text Available Abstract Background Dentin sialophosphoprotein (Dspp is a multidomain, secreted protein that is critical for the formation of tooth dentin. Mutations in DSPP cause inherited dentin defects categorized as dentin dysplasia type II and dentinogenesis imperfecta type II and type III. Dentin sialoprotein (Dsp, the N-terminal domain of dentin sialophosphoprotein (Dspp, is a highly glycosylated proteoglycan, but little is known about the number, character, and attachment sites of its carbohydrate moieties. Results To identify its carbohydrate attachment sites we isolated Dsp from developing porcine molars and digested it with endoproteinase Glu-C or pronase, fractionated the digestion products, identified fractions containing glycosylated peptides using a phenol sulfuric acid assay, and characterized the glycopeptides by N-terminal sequencing, amino acid analyses, or LC/MSMS. To determine the average number of sialic acid attachments per N-glycosylation, we digested Dsp with glycopeptidase A, labeled the released N-glycosylations with 2-aminobenzoic acid, and quantified the moles of released glycosylations by comparison to labeled standards of known concentration. Sialic acid was released by sialidase digestion and quantified by measuring β-NADH reduction of pyruvic acid, which was generated stoichiometrically from sialic acid by aldolase. To determine its forms, sialic acid released by sialidase digestion was labeled with 1,2-diamino-4,5-methyleneoxybenzene (DMB and compared to a DMB-labeled sialic acid reference panel by RP-HPLC. To determine the composition of Dsp glycosaminoglycan (GAG attachments, we digested Dsp with chondroitinase ABC and compared the chromotagraphic profiles of the released disaccharides to commercial standards. N-glycosylations were identified at Asn37, Asn77, Asn136, Asn155, Asn161, and Asn176. Dsp averages one sialic acid per N-glycosylation, which is always in the form of N-acetylneuraminic acid. O-glycosylations were

  9. Spondyloepiphyseal dysplasia congenita

    International Nuclear Information System (INIS)

    Macpherson, R.I.; Wood, B.P.

    1980-01-01

    Spondyloepiphyseal dysplasia congenita is a form of primarily short trunk dwarfism, that is manifest at birth but generally has not been regarded as a cause of lethal neonatal dwarfism. Seven neonates with severe dwarfism are presented. The first survived the newborn period, but the other six were early neonatal deaths. All displayed the clinical and radiologic features of spondyloepiphyseal dysplasia congenita. The striking similarities between spondyloepiphyseal dysplasia congenita and achondrogenesis type 2 are discussed. (orig.) [de

  10. Fracture Resistance of Lithium Disilicate Ceramics Bonded to Enamel or Dentin Using Different Resin Cement Types and Film Thicknesses.

    Science.gov (United States)

    Rojpaibool, Thitithorn; Leevailoj, Chalermpol

    2017-02-01

    To investigate the influence of cement film thickness, cement type, and substrate (enamel or dentin) on ceramic fracture resistance. One hundred extracted human third molars were polished to obtain 50 enamel and 50 dentin specimens. The specimens were cemented to 1-mm-thick lithium disilicate ceramic plates with different cement film thicknesses (100 and 300 μm) using metal strips as spacers. The cements used were etch-and-rinse (RelyX Ultimate) and self-adhesive (RelyX U200) resin cements. Compressive load was applied on the ceramic plates using a universal testing machine, and fracture loads were recorded in Newtons (N). Statistical analysis was performed by multiple regression (p enamel showed the highest mean fracture load (MFL; 1591 ± 172.59 N). The RelyX Ultimate groups MFLs were significantly higher than the corresponding RelyX U200 groups (p enamel (p enamel. Reduced resin film thickness could reduce lithium disilicate restoration fracture. Etch-and-rinse resin cements are recommended for cementing on either enamel or dentin, compared with self-adhesive resin cement, for improved fracture resistance. © 2015 by the American College of Prosthodontists.

  11. Three-dimensional imaging of acetabular dysplasia: diagnostic value and impact on surgical type classification

    International Nuclear Information System (INIS)

    Smet, Maria-Helena; Marchal, Guy J.; Baert, Albert L.; Hoe, Lieven van; Cleynenbreugel, Johan van; Daniels, Hans; Molenaers, Guy; Moens, Pierre; Fabry, Guy

    2000-01-01

    Objective: To investigate the diagnostic value and the impact on surgical type classification of three-dimensional (3D) images for pre-surgical evaluation of dysplastic hips. Materials and methods: Three children with a different surgical type of hip dysplasia were investigated with helical computed tomography. For each patient, two-dimensional (2D) images, 3D, and a stereolithographic model of the dysplastic hip were generated. In two separate sessions, 40 medical observers independently analyzed the 2D images (session 1), the 2D and 3D images (session 2), and tried to identify the corresponding stereolithographic hip model. The influence of both image presentation (2D versus 3D images) and observer (degree of experience, radiologist versus orthopedic surgeon) were statistically analyzed. The SL model choice reflected the impact on surgical type classification. Results: Image presentation was a significant factor whereas the individual observer was not. Three-dimensional images scored significantly better than 2D images (P=0.0003). Three-dimensional imaging increased the correct surgical type classification by 35%. Conclusion: Three-dimensional images significantly improve the pre-surgical diagnostic assessment and surgical type classification of dysplastic hips

  12. Three-dimensional imaging of acetabular dysplasia: diagnostic value and impact on surgical type classification

    Energy Technology Data Exchange (ETDEWEB)

    Smet, Maria-Helena E-mail: marleen.smet@uz.kuleuven.ac.be; Marchal, Guy J.; Baert, Albert L.; Hoe, Lieven van; Cleynenbreugel, Johan van; Daniels, Hans; Molenaers, Guy; Moens, Pierre; Fabry, Guy

    2000-04-01

    Objective: To investigate the diagnostic value and the impact on surgical type classification of three-dimensional (3D) images for pre-surgical evaluation of dysplastic hips. Materials and methods: Three children with a different surgical type of hip dysplasia were investigated with helical computed tomography. For each patient, two-dimensional (2D) images, 3D, and a stereolithographic model of the dysplastic hip were generated. In two separate sessions, 40 medical observers independently analyzed the 2D images (session 1), the 2D and 3D images (session 2), and tried to identify the corresponding stereolithographic hip model. The influence of both image presentation (2D versus 3D images) and observer (degree of experience, radiologist versus orthopedic surgeon) were statistically analyzed. The SL model choice reflected the impact on surgical type classification. Results: Image presentation was a significant factor whereas the individual observer was not. Three-dimensional images scored significantly better than 2D images (P=0.0003). Three-dimensional imaging increased the correct surgical type classification by 35%. Conclusion: Three-dimensional images significantly improve the pre-surgical diagnostic assessment and surgical type classification of dysplastic hips.

  13. Ceramic inlays : effect of mechanical cycling and ceramic type on restoration-dentin bond strength

    NARCIS (Netherlands)

    Trindade, F.Z.; Kleverlaan, C.J.; da Silva, L.H.; Feilzer, A.J.; Cesar, P.F.; Bottino, M.A.; Valandro, L.F.

    2016-01-01

    This study aimed to evaluate the bond strength between dentin and five different ceramic inlays in permanent maxillary premolars, with and without mechanical cycling. One hundred permanent maxillary premolars were prepared and divided into 10 groups (n=10) according to the ceramic system (IPS e.Max

  14. Focal cortical dysplasia type IIa and IIb: MRI aspects in 118 cases proven by histopathology

    Energy Technology Data Exchange (ETDEWEB)

    Colombo, Nadia; Citterio, Alberto [Ospedale Ca Granda Niguarda, Department of Neuroradiology, Milano (Italy); Tassi, Laura; Mai, Roberto; Sartori, Ivana; Cardinale, Francesco; Lo Russo, Giorgio [Ospedale Niguarda, Claudio Munari Epilepsy Surgery Center, Milano (Italy); Deleo, Francesco; Spreafico, Roberto [IRCCS Foundation Neurological Institute ' ' C. Besta' ' , Department of Epilepsy Clinic and Experimental Neurophysiology, Milano (Italy); Bramerio, Manuela [Ospedale Niguarda, Department of Pathology, Milano (Italy)

    2012-10-15

    This study aims to review the magnetic resonance imaging (MRI) aspects of a large series of patients with focal cortical dysplasia type II (FCD II) and attempt to identify distinctive features in the two histopathological subtypes IIa and IIb. We retrospectively reviewed the MRI scans of 118 patients with histological proven FCD IIa (n = 37) or IIb (n = 81) who were surgically treated for intractable epilepsy. MRI was abnormal in 93 patients (79 %) and unremarkable in 25 (21 %). A dysplastic lesion was identified in 90 cases (97 %) and classified as FCD II in 83 and FCD non-II in seven cases. In three cases, the MRI diagnosis was other than FCD. There was a significant association between the presence of cortical thickening (p = 0.002) and the ''transmantle sign'' (p < 0.001) and a correct MRI diagnosis of FCD II. MRI positivity was more frequent in the patients with FCD IIb than in those with FCD IIa (91 % vs. 51 %), and the detection rate of FCD II was also better in the patients with type IIb (88 % vs. 32 %). The transmantle sign was significantly more frequent in the IIb subgroup (p = 0.003). The rates of abnormal MRI results and correct MRI diagnoses of FCD II were significantly higher in the IIb subgroup. Although other MRI stigmata may contribute to the diagnosis, the only significant correlation was between the transmantle sign and FCD IIb. (orig.)

  15. Prenatal diagnosis of boomerang dysplasia.

    Science.gov (United States)

    Wessels, Marja W; Den Hollander, Nicolette S; De Krijger, Ronald R; Bonifé, Luisa; Superti-Furga, Andrea; Nikkels, Peter G; Willems, Patrick J

    2003-10-01

    Boomerang dysplasia, atelosteogenesis type 1 and Piepkorn dysplasia are bone dysplasias with an overlapping clinical spectrum characterized by deficient formation and ossification of specific elements of the skeleton. Typical symptoms include micromelia with diminished ossification, and a characteristic bowed and boomerang-like aspect of the long tubular bones. We report here a new case of boomerang dysplasia, which was detected prenatally in the 16th week of gestation by ultrasound. Copyright 2003 Wiley-Liss, Inc.

  16. The time of onset of abnormal calcification in spondylometaepiphyseal dysplasia, short limb-abnormal calcification type

    Energy Technology Data Exchange (ETDEWEB)

    Tueysuez, Beyhan [Istanbul University, Department of Pediatric Genetics, Cerrahpasa Medical School, Istanbul (Turkey); Gazioglu, Nurperi [Istanbul University, Department of Neurosurgery, Cerrahpasa Medical School, Istanbul (Turkey); Uenguer, Savas [Istanbul University, Department of Pediatric Radiology, Cerrahpasa Medical School, Istanbul (Turkey); Aji, Dolly Yafet [Istanbul University, Department of Pediatrics, Cerrahpasa Medical School, Istanbul (Turkey); Tuerkmen, Seval [Istanbul University, Department of Pediatric Genetics, Cerrahpasa Medical School, Istanbul (Turkey); Universitatsklinikum Berlin, Charite Virchow-Klinik, Berlin (Germany)

    2009-01-15

    A 1-month-old boy with shortness of extremities on prenatal US was referred to our department with a provisional diagnosis of achondroplasia. His height was normal but he had short extremities and platyspondyly, premature carpal epiphyses on both hands, and short tubular bones with irregular metaphyses on radiographs. Re-evaluation of the patient at the age of 1 year revealed very short height and premature calcification of the costal cartilages and epiphyses. Spondylometaepiphyseal dysplasia (SMED), short limb-abnormal calcification type was diagnosed. This condition is a very rare autosomal recessively inherited disorder, and most of the patients die in early childhood due to neurological involvement. At the age of 2 years and 5 months, a CT scan showed narrowing of the cervical spinal canal. One month later he died suddenly because of spinal cord injury. In conclusion early diagnosis is very important because the recurrence risk is high and patients may die due to early neurological complications. The time of onset of abnormal calcifications, a diagnostic finding of the disease, is at the age of around 1 year in most patients. When abnormal calcifications are not yet present, but radiological changes associated with SMED are present, this rare disease must be considered. (orig.)

  17. The time of onset of abnormal calcification in spondylometaepiphyseal dysplasia, short limb-abnormal calcification type

    International Nuclear Information System (INIS)

    Tueysuez, Beyhan; Gazioglu, Nurperi; Uenguer, Savas; Aji, Dolly Yafet; Tuerkmen, Seval

    2009-01-01

    A 1-month-old boy with shortness of extremities on prenatal US was referred to our department with a provisional diagnosis of achondroplasia. His height was normal but he had short extremities and platyspondyly, premature carpal epiphyses on both hands, and short tubular bones with irregular metaphyses on radiographs. Re-evaluation of the patient at the age of 1 year revealed very short height and premature calcification of the costal cartilages and epiphyses. Spondylometaepiphyseal dysplasia (SMED), short limb-abnormal calcification type was diagnosed. This condition is a very rare autosomal recessively inherited disorder, and most of the patients die in early childhood due to neurological involvement. At the age of 2 years and 5 months, a CT scan showed narrowing of the cervical spinal canal. One month later he died suddenly because of spinal cord injury. In conclusion early diagnosis is very important because the recurrence risk is high and patients may die due to early neurological complications. The time of onset of abnormal calcifications, a diagnostic finding of the disease, is at the age of around 1 year in most patients. When abnormal calcifications are not yet present, but radiological changes associated with SMED are present, this rare disease must be considered. (orig.)

  18. Left frontal meningioangiomatosis associated with type IIIc focal cortical dysplasia causing refractory epilepsy and literature review.

    Science.gov (United States)

    Roux, Alexandre; Mellerio, Charles; Lechapt-Zalcman, Emmanuelle; Still, Megan; Zerah, Michel; Bourgeois, Marie; Pallud, Johan

    2018-03-29

    We report the surgical management of a lesional drug-resistant epilepsy caused by a meningioangiomatosis associated with a type IIIc focal cortical dysplasia located in the left supplementary motor area in a young male patient. A first anatomical-based partial surgical resection was performed at 11 years old under general anaesthesia without intraoperative mapping, which allowed for postoperative seizure control (Engel IA) for six years. The patient then presented with intractable right sensatory and aphasic focal onset seizures despite two appropriate antiepileptic drugs. A second functional-based surgical resection was performed using intraoperative cortico-subcortical functional mapping with direct electrical stimulation under awake conditions. A complete surgical resection was performed and a left partial supplementary motor area syndrome was observed. At six postoperative months, the patient is seizure free (Engel IA) with an ongoing decrease in antiepileptic drug therapy. Intraoperative functional brain mapping can be applied to preserve the brain function and networks around a meningioangiomatosis to facilitate the resection of potentially epileptogenic perilesional dysplastic cortex and to tailor the extent of resection to functional boundaries. Copyright © 2018. Published by Elsevier Inc.

  19. Pseudoaneurysm Accompanied by Crowe Type IV Developmental Dysplasia of the Hip: A Case Report

    Directory of Open Access Journals (Sweden)

    Hirotake Yo

    2012-01-01

    Full Text Available We report the case of a 72-year-old woman whose pseudoaneurysm was difficult to diagnose and treat. The patient had a history of congenital dislocated hip and was undergoing anticoagulation therapy with warfarin due to the mitral valve replacement. Her chief complaint was pain and enlargement of the left buttock, and the laboratory tests revealed severe anemia. However, her elderly depression confused her chief complaint, and she was transferred to a psychiatric hospital. Two months after the onset of the symptoms, she was finally diagnosed with a pseudoaneurysm by contrast-enhanced CT and angiography. IDC coils were used for embolization. A plain CT showed hemostasis as well as a reduced hematoma at 2 months after the embolization. The possible contributing factors for the pseudoaneurysm included bleeding due to warfarin combined with an intramuscular hematoma accompanied by Crowe type IV developmental dysplasia of the hip that led to an arterial rupture by impingement between pelvis and femoral head. Since the warfarin treatment could not be halted due to the valve replacement, embolization was chosen for her treatment, and the treatment outcome was favorable.

  20. Surgical outcomes in two different age groups with Focal Cortical Dysplasia type II: Any real difference?

    Science.gov (United States)

    Ramírez-Molina, Jorge Luis; Di Giacomo, Roberta; Mariani, Valeria; Deleo, Francesco; Cardinale, Francesco; Uscátegui-Daccarett, Angélica María; Lorenzana, Pablo; Tassi, Laura

    2017-05-01

    Focal Cortical Dysplasias (FCDs) represent a common architectural cortical disorder underlying drug-resistant focal epilepsy. So far, studies aimed at evaluating whether age at surgery is a factor influencing surgical outcome are lacking, so that data on the comparison between patients harboring Type II FCD operated at younger age and those operated at adult age are still scarce. We compared presurgical clinical features and surgical outcomes of patients with histopathologically diagnosed Type II FCD undergoing surgery at an earlier age with those operated after 20 years of age. We retrospectively analyzed 1660 consecutive patients operated at the "Claudio Munari" Epilepsy Surgery Centre. There were 289 patients (17.4%) with a neuropathological diagnosis of Type II FCD. We included two different groups of patients, the first one including patients operated on at less than 6years, the second sharing the same seizure onset age but with delayed surgery, carried out after the age of 20. Seizure characteristics and, neuropsychological and postoperative seizure outcomes were evaluated by study group. Forty patients underwent surgery before the age of 6 and 66 patients after the age of 20. Surgical outcome was favorable in the whole population (72.6% were classified in Engel's Class Ia+Ic), independently from age at surgery. In the children group, 32 patients were classified in Class I, including 30 (75%) children in classes Ia and Ic. In the adult group, 53 belonged to Class I of whom 47 (71%) were in classes Ia and Ic. The percentage of permanent complications, the surgical outcomes, and AED withdrawal did not significantly differ by study group. Our results indicate that there is no difference between the groups, suggesting that outcome depends mainly on the histological findings and not on timing of surgery. Copyright © 2017 Elsevier Inc. All rights reserved.

  1. Ceramic Inlays: Effect of Mechanical Cycling and Ceramic Type on Restoration-dentin Bond Strength.

    Science.gov (United States)

    Trindade, F Z; Kleverlaan, C J; da Silva, L H; Feilzer, A J; Cesar, P F; Bottino, M A; Valandro, L F

    2016-01-01

    This study aimed to evaluate the bond strength between dentin and five different ceramic inlays in permanent maxillary premolars, with and without mechanical cycling. One hundred permanent maxillary premolars were prepared and divided into 10 groups (n=10) according to the ceramic system (IPS e.Max Press; IPS e.Max CAD; Vita PM9; Vita Mark II; and Vita VM7) and the mechanical cycling factor (with and without [100 N, 2 Hz, 1.2×10(6) cycles]). The inlays were adhesively cemented, and all of the specimens were cut into microbars (1×1 mm, nontrimming method), which were tested under microtensile loading. The failure mode was classified and contact angle, roughness, and microtopographic analyses were performed on each ceramic surface. The mechanical cycling had a significant effect (p=0.0087) on the bond strength between dentin and IPS e.max Press. The Vita Mark II group had the highest bond strength values under both conditions, with mechanical cycling (9.7±1.8 MPa) and without (8.2±1.9 MPa), while IPS e.Max CAD had the lowest values (2.6±1.6 and 2.2±1.4, respectively). The adhesive failure mode at the ceramic/cement interface was the most frequent. Vita Mark II showed the highest value of average roughness. IPS e.max Press and Vita Mark II ceramics presented the lowest contact angles. In conclusion, the composition and manufacturing process of ceramics seem to have an influence on the ceramic surface and resin cement bond strength. Mechanical cycling did not cause significant degradation on the dentin and ceramic bond strength under the configuration used.

  2. C-type natriuretic peptide plasma levels are elevated in subjects with achondroplasia, hypochondroplasia, and thanatophoric dysplasia.

    Science.gov (United States)

    Olney, Robert C; Prickett, Timothy C R; Espiner, Eric A; Mackenzie, William G; Duker, Angela L; Ditro, Colleen; Zabel, Bernhard; Hasegawa, Tomonobu; Kitoh, Hiroshi; Aylsworth, Arthur S; Bober, Michael B

    2015-02-01

    C-type natriuretic peptide (CNP) is a crucial regulator of endochondral bone growth. In a previous report of a child with acromesomelic dysplasia, Maroteaux type (AMDM), caused by loss-of-function of the CNP receptor (natriuretic peptide receptor-B [NPR-B]), plasma levels of CNP were elevated. In vitro studies have shown that activation of the MAPK kinase (MEK)/ERK MAPK pathway causes functional inhibition of NPR-B. Achondroplasia, hypochondroplasia, and thanatophoric dysplasia are syndromes of short-limbed dwarfism caused by activating mutations of fibroblast growth factor receptor-3, which result in overactivation of the MEK/ERK MAPK pathway. The purpose of this study was to determine whether these syndromes exhibit evidence of CNP resistance as reflected by increases in plasma CNP and its amino-terminal propeptide (NTproCNP). This was a prospective, observational study. Participants were 63 children and 20 adults with achondroplasia, 6 children with hypochondroplasia, 2 children with thanatophoric dysplasia, and 4 children and 1 adult with AMDM. Plasma levels of CNP and NTproCNP were higher in children with achondroplasia with CNP SD scores (SDSs) of 1.0 (0.3-1.4) (median [interquartile range]) and NTproCNP SDSs of 1.4 (0.4-1.8; P achondroplasia (CNP SDSs of 1.5 [0.7-2.1] and NTproCNP SDSs of 0.5 [0.1-1.0], P < .005). In children with hypochondroplasia, CNP SDSs were 1.3 (0.7-1.5) (P = .08) and NTproCNP SDSs were 1.9 (1.8-2.3) (P < .05). In children with AMDM, CNP SDSs were 1.6 (1.4-3.3) and NTproCNP SDSs were 4.2 (2.7-6.2) (P < .01). In these skeletal dysplasias, elevated plasma levels of proCNP products suggest the presence of tissue resistance to CNP.

  3. Small Lesion Size Is Associated with Sleep-Related Epilepsy in Focal Cortical Dysplasia Type II

    Directory of Open Access Journals (Sweden)

    Bo Jin

    2018-02-01

    Full Text Available ObjectiveTo investigate the neuroimaging and clinical features associated with sleep-related epilepsy (SRE in patients with focal cortical dysplasia (FCD type II.MethodsPatients with histopathologically proven FCD type II were included from three epilepsy centers. SRE was defined according to the video EEG findings and seizure history. Cortical surface reconstruction and volume calculation were performed using FreeSurfer. The lesions were manually delineated on T1 volumetric MRI using the ITK-SNAP software. The lesion volumes were normalized by the intracranial volume of each patient. The lesions were classified as small or large by placing a threshold based on quantitative (whether the lesion was detected on MRI report and qualitative (volume criteria.ResultsA total of 77 consecutive patients were included. Of them, 36 had SRE and 41 had non-SRE. An earlier age of epilepsy onset, high seizure frequency, regional interictal EEG findings, and favorable surgical outcome were characteristic in both groups. Small lesions were defined as those having a volume <3,217 mm3. In total, 60.9% of the patients with SRE (25/41 had small FCD lesion, which was significantly higher than the non-SRE group (9/34, 26.5%, p = 0.005. Small lesion size was the only predictor significantly associated with SRE in the overall type II group by multivariate analyses (p = 0.016. Although the proportion of patients who had frontal FCD and SRE was higher than non-frontal FCD (54.5 vs. 27.3%, p = 0.043, the relationship between SRE and lesion location was not confirmed by multivariate analysis. Thalamic volume and seizure semiology were not statistically different between the SRE and non-SRE group. The significant association between lesion size and SRE was reproducible in type IIb and IIa subgroups.SignificanceSRE is common in patients with FCD type II. Small FCD type II lesions are significantly associated with SRE. Although our findings cannot be applied to

  4. Familial temporal lobe epilepsy due to focal cortical dysplasia type IIIa

    Czech Academy of Sciences Publication Activity Database

    Fábera, Petr; Krijtová, H.; Tomášek, M.; Krýsl, D.; Zámečník, J.; Mohapl, M.; Jiruška, Přemysl; Marusič, P.

    2015-01-01

    Roč. 31, Sep 2015 (2015), s. 120-123 ISSN 1059-1311 R&D Projects: GA MZd(CZ) NT14489 Institutional support: RVO:67985823 Keywords : familial temporal lobe epilepsy * focal cortical dysplasia * epilepsy surgery * genetics of epilepsy Subject RIV: FH - Neurology Impact factor: 2.109, year: 2015

  5. Presentation of hypohidrotic ectodermal dysplasia in two siblings

    Directory of Open Access Journals (Sweden)

    Uday Ginjupally

    2015-01-01

    Full Text Available Ectodermal dysplasias are a large hereditary group of disorders which are usually manifested as X-linked recessive disorders and have a full expression in males, whereas females show little to no signs of the disorder. The two most common types of ectodermal dysplasias are hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome and hidrotic ectodermal dysplasia (Clouston syndrome. Hypohidrotic ectodermal dysplasia is characterized by hypodontia, hypotrichosis, and hypohidrosis. Here, we present two female sibling cases of hypohidrotic ectodermal dysplasia.

  6. Kidney Dysplasia

    Science.gov (United States)

    ... whose mothers used certain prescription medications or illegal drugs during pregnancy What are the signs of kidney dysplasia? Many ... the use of certain prescription medications or illegal drugs during pregnancy. Pregnant women should talk with their health care ...

  7. Type IX Collagen Gene Mutations Can Result in Multiple Epiphyseal Dysplasia That Is Associated With Osteochondritis Dissecans and a Mild Myopathy

    NARCIS (Netherlands)

    Jackson, Gail C.; Marcus-Soekarman, Dominique; Stolte-Dijkstra, Irene; Verrips, Aad; Taylor, Jacqueline A.; Briggs, Michael D.

    Multiple epiphyseal dysplasia (MED) is a clinically variable and genetically heterogeneous disease that is characterized by mild short stature and early onset osteoarthritis. Autosomal dominant forms are caused by mutations in the genes that encode type IX collagen, cartilage oligomeric matrix

  8. Type IX collagen gene mutations can result in multiple epiphyseal dysplasia that is associated with osteochondritis dissecans and a mild myopathy.

    NARCIS (Netherlands)

    Jackson, G.C.; Marcus-Soekarman, D.; Stolte-Dijkstra, I.; Verrips, A.; Taylor, J.A.; Briggs, M.D.

    2010-01-01

    Multiple epiphyseal dysplasia (MED) is a clinically variable and genetically heterogeneous disease that is characterized by mild short stature and early onset osteoarthritis. Autosomal dominant forms are caused by mutations in the genes that encode type IX collagen, cartilage oligomeric matrix

  9. Spondylo-meta-epiphyseal dysplasia, short limbs, abnormal calcification type: a new case with severe neurological involvement

    Energy Technology Data Exchange (ETDEWEB)

    Fano, V.; Lejarraga, H. [Hospital Garrahan, Buenos Aires (Argentina). Service of Growth and Development; Barreiro, C. [Hospital Garrahan, Buenos Aires (Argentina). Service of Genetics

    2001-01-01

    A case of an affected girl with spondylo-meta-epiphyseal dysplasia (SMED) is reported. The disease was detected at birth as a congenital dysplasia with generalized lesions. At 10 months of age, abnormal calcifications appeared in both wrists. The patient evolved with severe growth retardation and multiple neurological and respiratory complications, followed by death at 21 months of age. (orig.)

  10. Polymer Nanocarriers for Dentin Adhesion

    Science.gov (United States)

    Osorio, R.; Osorio, E.; Medina-Castillo, A.L.; Toledano, M.

    2014-01-01

    To obtain more durable adhesion to dentin, and to protect collagen fibrils of the dentin matrix from degradation, calcium- and phosphate-releasing particles have been incorporated into the dental adhesive procedure. The aim of the present study was to incorporate zinc-loaded polymeric nanocarriers into a dental adhesive system to facilitate inhibition of matrix metalloproteinases (MMPs)-mediated collagen degradation and to provide calcium ions for mineral deposition within the resin-dentin bonded interface. PolymP-nActive nanoparticles (nanoMyP) were zinc-loaded through 30-minute ZnCl2 immersion and tested for bioactivity by means of 7 days’ immersion in simulated body fluid solution (the Kokubo test). Zinc-loading and calcium phosphate depositions were examined by scanning and transmission electron microscopy, elemental analysis, and x-ray diffraction. Nanoparticles in ethanol solution infiltrated into phosphoric-acid-etched human dentin and Single Bond (3M/ESPE) were applied to determine whether the nanoparticles interfered with bonding. Debonded sticks were analyzed by scanning electron microscopy. A metalloproteinase collagen degradation assay was also performed in resin-infiltrated dentin with and without nanoparticles, measuring C-terminal telopeptide of type I collagen (ICTP) concentration in supernatants, after 4 wk of immersion in artificial saliva. Numerical data were analyzed by analysis of variance (ANOVA) and Student-Newman-Keuls multiple comparisons tests (p calcium regardless of zinc incorporation. Nanoparticles failed to infiltrate demineralized intertubular dentin and remained on top of the hybrid layer, without altering bond strength. Calcium and phosphorus were found covering nanoparticles at the hybrid layer, after 24 h. Nanoparticle application in etched dentin also reduced MMP-mediated collagen degradation. Tested nanoparticles may be incorporated into dental adhesive systems to provide the appropriate environment in which dentin MMP

  11. Immunohistochemical Evaluation of Glucose Transporter Type 1 in Epithelial Dysplasia and Oral Squamous Cell Carcinoma.

    Science.gov (United States)

    Pereira, Karuza Maria Alves; Feitosa, Sthefane Gomes; Lima, Ana Thayssa Tomaz; Luna, Ealber Carvalho Macedo; Cavalcante, Roberta Barroso; de Lima, Kenio Costa; Chaves, Filipe Nobre; Costa, Fábio Wildson Gurgel

    2016-01-01

    Oral squamous cell carcinoma (OSCC) is the most common malignancy of the oral cavity and some of these have been documented in association or preceded by oral epithelial dysplasia (OED). Aggressive cancers with fast growth have demonstrated overexpression of some glucose transporters (GLUTs). Thus, the aim of this study was to analyze the immunohistochemical expression of the glucose transporter, GLUT-1, in OEDs and OSCCs, seeking to better elucidate the biological behavior of neoplasias. Fifteen cases were selected this research of both lesions. Five areas were analyzed from each case by counting the percentage of positive cells at 400x magnification. Immunoreactivity of GLUT-1 was observed in 100% of the samples ranging from 54.2% to 86.2% for the OSCC and 73.9% to 97.4% for the OED. Statistical test revealed that there was greater overexpression of GLUT-1 in OED than the OSCC (p=0.01). It is believed the high expression of GLUT-1 may reflect the involvement of GLUT-1 in early stages of oral carcinogenesis.

  12. A novel splice site mutation in the dentin sialophosphoprotein gene in a Chinese family with dentinogenesis imperfecta type II

    International Nuclear Information System (INIS)

    Wang Haoyang; Hou Yanning; Cui Yingxia; Huang Yufeng; Shi Yichao; Xia Xinyi; Lu Hongyong; Wang Yunhua; Li Xiaojun

    2009-01-01

    Twenty-four individuals were investigated that spanned six generations in a Chinese family affected with an apparently autosomal dominant form of dentinogenesis imperfecta type II (DGI-II, OMIM 125490). All affected individuals presented with typical, clinical and radiographic features of DGI-II, but without bilateral progressive high-frequency sensorineural hearing loss. To investigate the mutated molecule, a positional candidate approach was used to determine the mutated gene in this family. Genomic DNA was obtained from 24 affected individuals, 18 unaffected relatives of the family and 50 controls. Haplotype analysis was performed using leukocyte DNA for 6 short tandem repeat (STR) markers present in chromosome 4 (D4S1534, GATA62A11, DSPP, DMP1, SPP1 and D4S1563). In the critical region between D4S1534 and DMP1, the dentin sialophosphoprotein (DSPP) gene (OMIM *125485) was considered as the strongest candidate gene. The first four exons and exon/intron boundaries of the gene were analyzed using DNA from 24 affected individuals and 18 unaffected relatives of the same family. DNA sequencing revealed a heterozygous deletion mutation in intron 2 (at positions -3 to -25), which resulted in a frameshift mutation, that changed the acceptor site sequence from CAG to AAG (IVS2-3C→A) and may also have disrupted the branch point consensus sequence in intron 2. The mutation was found in the 24 affected individuals, but not in the 18 unaffected relatives and 50 controls. The deletion was identified by allele-specific sequencing and denaturing high-performance liquid chromatography (DHPLC) analysis. We conclude that the heterozygous deletion mutation contributed to the pathogenesis of DGI-II

  13. Ectodermal dysplasia

    Directory of Open Access Journals (Sweden)

    Sonia Saggoo

    2009-01-01

    Full Text Available Hereditary hypohidrotic ectodermal dysplasia, also called the Christ-Siemens-Touraine Syndrome is characterized by congenital dysplasia of one or more ectodermal structures and is manifested by hypohidrosis, hypotrichosis and hypodontia. It is usually an X-linked recessive mendelian character which is rarely seen in males. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Patients with this disorder exhibit smooth , thin and dry skin, fine and blond scanty hair. Intra-orally anodontia or hypodontia, with impaired development of alveolar process is seen. A case report of a rare case of this disorder in a female patient aged 18 years is hereby presented.

  14. Sponastrime dysplasia

    International Nuclear Information System (INIS)

    Lachman, R.S.; California Univ., Los Angeles, Torrance, CA; Stoss, H.; Spranger, J.

    1989-01-01

    The 2nd family with Sponastrime Dysplasia is described. The clinical, radiologic and chondro-osseous morphology of boy and girl siblings are presented. The facial appearance is an 'oriental look' with midface hypoplasia and a saddle nose. The radiological findings include the spinal changes of lordosis, osteoporosis and pear-shaped vertebrae, as well as striated metaphyses (osteopathia striata). The morphological findings suggest a disturbance in the formation of cartilage, with a defect in collagen and proteoglycans synthesis in this rare autosomal recessive skeletal dysplasia. (orig.)

  15. Acromesomelic dysplasia (Marotaeux type associated with craniovertebral junction anomaly: A report of a rare case and review of literature

    Directory of Open Access Journals (Sweden)

    Jayesh C Sardhara

    2014-01-01

    Full Text Available Acromesomelic dysplasia Maroteaux type (AMDM is a rare autosomal recessive osteochondrodysplasia. The responsible gene, AMDM gene, in human beings, has been mapped on 9p13-q12 chromosome by homozygous mapping and pathogenic mutation was later identified in natriuretic receptor B (NPR-B which has been implicated in the regulation of skeletal growth. Till now, around 40 to 50 cases of AMDM have been described in the world literature. Association of the congenital craniovertebral (CV junction anomaly has not been reported. Here we are presenting a case of AMDM, with CV junction anomaly. A 10-year boy presented with short stature (122 cm with short distal limbs, symptomatic for thoracic kyphoscoliosis with back pain. On examination there were no neurological deficits. On radiological investigation, he was found to have short and broad phalanges and toes, thoracic kyphoscoliosis, abnormal pelvic ring, mild ventriculomegaly, cervical syringomyelia and tonsillar descent below foramen magnum, hydrocephalus, os odontoideum with Klippel-Feil anomaly. This was diagnosed as AMDM with congenital os odontoideum, Klippel-Feil anomaly with Arnold-Chiari malformation (ACM type-1. The patient underwent posterior fossa decompression by removal of foramen magnum ring along with C1 arch for ACM type-1. Kyphosis was left for conservative treatment till further observation and required orthopedic correction in his further age. To the best of our knowledge this is a very rare entity of AMDM with congenital CV junction anomaly.

  16. Procollagen type-III aminoterminal peptide in serum and synovial fluid of dogs with hip dysplasia and coxarthrosis

    DEFF Research Database (Denmark)

    Madsen, J S; Jensen, L T; Strøm, H

    1990-01-01

    Hip dysplasia is an affection of the coxofemoral joint that progresses until stabilization is caused by fibrosis and osteoarthritic changes. This stabilization process can be examined by clinical and radiographic methods. The capability of evaluating the procollagen concentrations in liquids......-III-NP) concentration was measured in serum and in synovial fluid from coxofemoral joints in 20 dogs. Dogs were grouped on the basis of evidence of dysplasia and osteoarthritic changes of the hip: (1) a control group of 6 dogs without clinical or radiographic signs of hip dysplasia, and (2) dysplastic group of 14 dogs...

  17. Genetics Home Reference: metatropic dysplasia

    Science.gov (United States)

    ... is a skeletal disorder characterized by short stature (dwarfism) with other skeletal abnormalities. The term "metatropic" is ... my area? Other Names for This Condition metatropic dwarfism metatropic dysplasia type 1 Related Information How are ...

  18. [New classification of Crowe type IV developmental dysplasia of the hip].

    Science.gov (United States)

    Ma, Hai-yang; Zhou, Yong-gang; Zheng, Chong; Cao, Wen-zhe; Wang Sen; Wu, Wen-ming; Piao, Shang; Du, Yin-qiao

    2016-02-01

    To compare differences between Crowe IV developmental dysplasia of the hip (DDH) with secondary acetabulum and Crowe IV DDH without secondary acetabulum,and determine whether it is necessary to divide Crowe IV DDH into two subtypes. From June 2007 to May 2015,145 hips of 112 Crowe N patients who underwent total hip arthroplasty (THA) using S-ROM stem were divided into two groups: secondary acetabulum formaton group (group A) and no secondary acetabulum formaton group (group B). In group A,there were 12 females, 96 males,with an average age of (39.38 ± 11.19) years old. In group B, there were 2 females, 35 males, with an average age of (38.19 ± 10.92) years old. All the patients were evaluated by using Harris Hip Score. Radiographic evaluations were made preoperatively and during follow up. The differences between two groups were compared on dislocation height, canal flare index (CFI), subtrochanteric shortening osteotomy (SSTO) usage, pre- and post-operation Harris scores, complications. The dislocation height for group A was (4.74 ± 1.57) cm, while the dislocation height for group B was (3.12 ± 1.15) cm. Significantly difference was detected between two groups. The CFI for group A was 2.69 ± 0.68, while the CFI for group B was 3.42 ± 0.79, and the significantly difference was detected between two groups. Harris scores were totally improved from 58.18 ± 15.67 preoperatively to 91.20 ± 3.79 post-operatively and the difference was significant. Pre-operative Harris scores was 58.1 ± 15.3 in group A, 58.3 ± 16.9 in group B. Post-operative Harris scores was 91.0 ± 4.1 in group A, 91.0 ± 5.1 in group B. No significant difference was found on Harris scores between A and B preoperatively and post-operatively. Complications of 4 cases peri-prosthesis fracture, 4 cases dislocation and 4 cases nerve injury occur in group A; While only one case dislocation and one case nerve injury occur in group B. No statistical significance was detected. Crowe IV DDH with

  19. Focal cortical dysplasia – review

    International Nuclear Information System (INIS)

    Kabat, Joanna; Król, Przemysław

    2012-01-01

    Focal cortical dysplasia is a malformation of cortical development, which is the most common cause of medically refractory epilepsy in the pediatric population and the second/third most common etiology of medically intractable seizures in adults. Both genetic and acquired factors are involved in the pathogenesis of cortical dysplasia. Numerous classifications of the complex structural abnormalities of focal cortical dysplasia have been proposed – from Taylor et al. in 1971 to the last modification of Palmini classification made by Blumcke in 2011. In general, three types of cortical dysplasia are recognized. Type I focal cortical dysplasia with mild symptomatic expression and late onset, is more often seen in adults, with changes present in the temporal lobe. Clinical symptoms are more severe in type II of cortical dysplasia usually seen in children. In this type, more extensive changes occur outside the temporal lobe with predilection for the frontal lobes. New type III is one of the above dysplasias with associated another principal lesion as hippocampal sclerosis, tumor, vascular malformation or acquired pathology during early life. Brain MRI imaging shows abnormalities in the majority of type II dysplasias and in only some of type I cortical dysplasias. The most common findings on MRI imaging include: focal cortical thickening or thinning, areas of focal brain atrophy, blurring of the gray-white junction, increased signal on T2- and FLAIR-weighted images in the gray and subcortical white matter often tapering toward the ventricle. On the basis of the MRI findings, it is possible to differentiate between type I and type II cortical dysplasia. A complete resection of the epileptogenic zone is required for seizure-free life. MRI imaging is very helpful to identify those patients who are likely to benefit from surgical treatment in a group of patients with drug-resistant epilepsy. However, in type I cortical dysplasia, MR imaging is often normal, and also in both

  20. Pseudoachondroplastic dysplasia.

    Directory of Open Access Journals (Sweden)

    Khungar A

    1993-04-01

    Full Text Available Pseudoachondroplasia is a heterogeneous inherited skeletal dysplasia in which dwarfism is a major feature. We report here a case of a 7 year old girl misdiagnosed as rickets, who presented with short stature, lordosis, genu varum and flexion deformities at both the elbows. Skeletal survey revealed epiphyseal and metaphyseal irregularities. A review of literature is also presented.

  1. Frontofacionasal Dysplasia

    African Journals Online (AJOL)

    rme

    226. Frontofacionasal Dysplasia: Another Observation eye. Other ocular features include small eyes, iris colobomas and cataracts. A frontally situated lipoma, causing a swelling at the nasion, was reported in one patient and an encephalocele has been described.3. Mental retardation has been reported in one patient with.

  2. Fibromuscular dysplasia

    Directory of Open Access Journals (Sweden)

    Jeunemaitre Xavier

    2007-06-01

    Full Text Available Abstract Fibromuscular dysplasia (FMD, formerly called fibromuscular fibroplasia, is a group of nonatherosclerotic, noninflammatory arterial diseases that most commonly involve the renal and carotid arteries. The prevalence of symptomatic renal artery FMD is about 4/1000 and the prevalence of cervicocranial FMD is probably half that. Histological classification discriminates three main subtypes, intimal, medial and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string-of-beads' appearance that is related to medial FMD, and tubular and focal types, which are not clearly related to specific histological lesions. Renovascular hypertension is the most common manifestation of renal artery FMD. Multifocal stenoses with the 'string-of-beads' appearance are observed at angiography in more than 80% of cases, mostly in women aged between 30 and 50 years; they generally involve the middle and distal two-thirds of the main renal artery and in some case also renal artery branches. Cervicocranial FMD can be complicated by dissection with headache, Horner's syndrome or stroke, or can be associated with intracerebral aneurysms with a risk of subarachnoid or intracerebral hemorrhage. The etiology of FMD is unknown, although various hormonal and mechanical factors have been suggested. Subclinical lesions are found at arterial sites distant from the stenotic arteries, and this suggests that FMD is a systemic arterial disease. It appears to be familial in 10% of cases. Noninvasive diagnostic tests include, in increasing order of accuracy, ultrasonography, magnetic resonance angiography and computed tomography angiography. The gold standard for diagnosing FMD is catheter angiography, but this invasive procedure is only used for patients in whom it is clinically pertinent to proceed with revascularization during the same procedure. Differential diagnosis include

  3. Spondylometaepiphyseal dysplasia in a mother and her child

    Energy Technology Data Exchange (ETDEWEB)

    Pettersson, H; Nilsson, K O [Section of Pediatric Radiology, Department of Diagnostic Radiology, and the Department of Pediatrics, Malmoe Allmaenna Sjukhus, Malmoe, Sweden

    1979-01-01

    Variant types of spondylometaepiphyseal dysplasia in a mother and her child is reported. Several stages of the disorder are presented, demonstrating the principal difficulties in distinguishing variant types of skeletal dysplasia.

  4. A distinct form of spondyloepimetaphyseal dysplasia with joint laxity (SEMDJL)-leptodactylic type: radiological characteristics in seven new patients

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ok-Hwa [Ajou University Hospital, Department of Radiology, Suwon (Korea); Cho, Tae-Joon [Seoul National University Children' s Hospital, Department of Orthopaedic Surgery, Seoul (Korea); Song, Hae-Ryong [Korea University Guro Hospital, Department of Othopaedic Surgery, Seoul (Korea); Chung, Chin Youb [Seoul National University Bundang Hospital, Department of Orthopaedic Surgery, Gyeonggi (Korea); Miyagawa, Shin-Ichiro [National Hospital Organization Kure Medical center, Department of Pediatrics, Hiroshima (Japan); Nishimura, Gen [Kiyose Children' s Hospital, Department of Radiology, Tokyo (Japan); Superti-Furga, Andrea [University of Freiburg, Departement of Pediatrics, Freiburg (Germany); Unger, Sheila [Institute of Human Genetics, University of Freiburg, Freiburg (Germany)

    2009-08-15

    This study presents seven cases of a rare but distinctive form of spondyloepimetaphyseal dysplasia with joint laxity-leptodactylic or Hall type to emphasize the characteristic clinical and radiological findings. A multiinstitutional retrospective review was performed on seven patients. The patient population consisted of one family with an affected mother and two siblings and four unrelated patients; there were one adult, aged 40 years, and six children, ranging in age from 3 to 12 years. The gender ratio of females to males was 5 to 2. We reviewed the clinical data and skeletal surveys and focused on radiographs of the pelvis, knees, hands, and spine. The outstanding clinical features were short stature, midface hypoplasia, and multiple dislocations and/or ligamentous laxity of the large joints, particularly at the knees with a genu valgum or varum deformity. Of seven patients, six patients showed normal intellect but one patient had mild mental retardation. The main radiological features included small, irregular epiphyses, metaphyseal irregularity with vertical striations that was a constant finding at the knees, constricted femoral necks, delayed ossification of the carpal bones, and slender metacarpals. Progressive thoracolumbar scoliosis was evident with aging; however, the vertebral bodies appeared normal in height or mild platyspondyly was noted. In view of the orthopedic management of multiple joint dislocations and ligamentous laxity of the large joints, awareness of this disease entity and diagnostic precision solely based on radiological findings is of importance, particularly as the disorder is currently more common than initially reported. (orig.)

  5. A distinct form of spondyloepimetaphyseal dysplasia with joint laxity (SEMDJL)-leptodactylic type: radiological characteristics in seven new patients

    International Nuclear Information System (INIS)

    Kim, Ok-Hwa; Cho, Tae-Joon; Song, Hae-Ryong; Chung, Chin Youb; Miyagawa, Shin-Ichiro; Nishimura, Gen; Superti-Furga, Andrea; Unger, Sheila

    2009-01-01

    This study presents seven cases of a rare but distinctive form of spondyloepimetaphyseal dysplasia with joint laxity-leptodactylic or Hall type to emphasize the characteristic clinical and radiological findings. A multiinstitutional retrospective review was performed on seven patients. The patient population consisted of one family with an affected mother and two siblings and four unrelated patients; there were one adult, aged 40 years, and six children, ranging in age from 3 to 12 years. The gender ratio of females to males was 5 to 2. We reviewed the clinical data and skeletal surveys and focused on radiographs of the pelvis, knees, hands, and spine. The outstanding clinical features were short stature, midface hypoplasia, and multiple dislocations and/or ligamentous laxity of the large joints, particularly at the knees with a genu valgum or varum deformity. Of seven patients, six patients showed normal intellect but one patient had mild mental retardation. The main radiological features included small, irregular epiphyses, metaphyseal irregularity with vertical striations that was a constant finding at the knees, constricted femoral necks, delayed ossification of the carpal bones, and slender metacarpals. Progressive thoracolumbar scoliosis was evident with aging; however, the vertebral bodies appeared normal in height or mild platyspondyly was noted. In view of the orthopedic management of multiple joint dislocations and ligamentous laxity of the large joints, awareness of this disease entity and diagnostic precision solely based on radiological findings is of importance, particularly as the disorder is currently more common than initially reported. (orig.)

  6. Type VII collagen is enriched in the enamel organic matrix associated with the dentin-enamel junction of mature human teeth.

    Science.gov (United States)

    McGuire, Jacob D; Walker, Mary P; Mousa, Ahmad; Wang, Yong; Gorski, Jeff P

    2014-06-01

    The inner enamel region of erupted teeth is known to exhibit higher fracture toughness and crack growth resistance than bulk phase enamel. However, an explanation for this behavior has been hampered by the lack of compositional information for the residual enamel organic matrix. Since enamel-forming ameloblasts are known to express type VII collagen and type VII collagen null mice display abnormal amelogenesis, the aim of this study was to determine whether type VII collagen is a component of the enamel organic matrix at the dentin-enamel junction (DEJ) of mature human teeth. Immunofluorescent confocal microscopy of demineralized tooth sections localized type VII collagen to the organic matrix surrounding individual enamel rods near the DEJ. Morphologically, immunoreactive type VII collagen helical-bundles resembled the gnarled-pattern of enamel rods detected by Coomassie Blue staining. Western blotting of whole crown or enamel matrix extracts also identified characteristic Mr=280 and 230 kDa type VII dimeric forms, which resolved into 75 and 25 kDa bands upon reduction. As expected, the collagenous domain of type VII collagen was resistant to pepsin digestion, but was susceptible to purified bacterial collagenase. These results demonstrate the inner enamel organic matrix in mature teeth contains macromolecular type VII collagen. Based on its physical association with the DEJ and its well-appreciated capacity to complex with other collagens, we hypothesize that enamel embedded type VII collagen fibrils may contribute not only to the structural resilience of enamel, but may also play a role in bonding enamel to dentin. Copyright © 2014 Elsevier Inc. All rights reserved.

  7. A novel loss-of-function mutation in Npr2 clarifies primary role in female reproduction and reveals a potential therapy for acromesomelic dysplasia, Maroteaux type.

    Science.gov (United States)

    Geister, Krista A; Brinkmeier, Michelle L; Hsieh, Minnie; Faust, Susan M; Karolyi, I Jill; Perosky, Joseph E; Kozloff, Kenneth M; Conti, Marco; Camper, Sally A

    2013-01-15

    We discovered a new spontaneous mutant allele of Npr2 named peewee (pwe) that exhibits severe disproportionate dwarfism and female infertility. The pwe phenotype is caused by a four base-pair deletion in exon 3 that generates a premature stop codon at codon 313 (L313X). The Npr2(pwe/pwe) mouse is a model for the human skeletal dysplasia acromesomelic dysplasia, Maroteaux type (AMDM). We conducted a thorough analysis of the female reproductive tract and report that the primary cause of Npr2(pwe/pwe) female infertility is premature oocyte meiotic resumption, while the pituitary and uterus appear to be normal. Npr2 is expressed in chondrocytes and osteoblasts. We determined that the loss of Npr2 causes a reduction in the hypertrophic and proliferative zones of the growth plate, but mineralization of skeletal elements is normal. Mutant tibiae have increased levels of the activated form of ERK1/2, consistent with the idea that natriuretic peptide receptor type 2 (NPR2) signaling inhibits the activation of the MEK/ERK mitogen activated protein kinase pathway. Treatment of fetal tibiae explants with mitogen activated protein kinase 1 and 2 inhibitors U0126 and PD325901 rescues the Npr2(pwe/pwe) growth defect, providing a promising foundation for skeletal dysplasia therapeutics.

  8. Tibial lengthening for unilateral Crowe type-IV developmental dysplasia of the hip

    Directory of Open Access Journals (Sweden)

    Jun Wan

    2014-01-01

    Conclusions: Tibial lengthening may effectively correct gait and satisfactorily improve body image in young patients with unilateral Crowe type-IV DDH. Mono-lateral external fixator allows for accelerated postoperative rehabilitation and optimal preservation of ankle movements. Lengthening along with intramedullary nails may significantly reduce the external fixation time and the risk of fixator-related complications.

  9. Peritubular dentin lacks piezoelectricity.

    Science.gov (United States)

    Habelitz, S; Rodriguez, B J; Marshall, S J; Marshall, G W; Kalinin, S V; Gruverman, A

    2007-09-01

    Dentin is a mesenchymal tissue, and, as such, is based on a collagenous matrix that is reinforced by apatite mineral. Collagen fibrils show piezoelectricity, a phenomenon that is used by piezoresponse force microscopy (PFM) to obtain high-resolution images. We applied PFM to image human dentin with 10-nm resolution, and to test the hypothesis that zones of piezoactivity, indicating the presence of collagen fibrils, can be distinguished in dentin. Piezoelectricity was observed by PFM in the dentin intertubular matrix, while the peritubular dentin remained without response. High-resolution imaging of chemically treated intertubular dentin attributed the piezoelectric effect to individual collagen fibrils that differed in the signal strength, depending on the fibril orientation. This study supports the hypothesis that peritubular dentin is a non-collagenous tissue and is thus an exception among mineralized tissues that derive from the mesenchyme.

  10. Analysis of the shelf life of chitosan stored in different types of packaging, using colorimetry and dentin microhardness.

    Science.gov (United States)

    da Cruz-Filho, Antonio Miranda; Bordin, Angelo Rafael de Vito; Souza-Flamini, Luis Eduardo; Guedes, Débora Fernandes da Costa; Saquy, Paulo César; Silva, Ricardo Gariba; Pécora, Jesus Djalma

    2017-05-01

    Chitosan has been widely investigated and used. However, the literature does not refer to the shelf life of this solution. This study evaluated, through the colorimetric titration technique and an analysis of dentin micro-hardness, the shelf life of 0.2% chitosan solution. Thirty human canines were sectioned, and specimens were obtained from the second and third slices, from cemento-enamel junction to the apex. A 0.2% chitosan solution was prepared and distributed in 3 identical glass bottles (v1, v2, and v3) and 3 plastic bottles (p1, p2, and p3). At 0, 7, 15, 30, 45, 60, 90, 120, 150, and 180 days, the specimens were immersed in each solution for 5 minutes ( n = 3 each). The chelating effect of the solution was assessed by micro-hardness and colorimetric analysis of the dentin specimens. 17% EDTA and distilled water were used as controls. Data were analyzed statistically by two-way and Tukey-Kramer multiple comparison ( α = 0.05). There was no statistically significant difference among the solutions with respect to the study time ( p = 0.113) and micro-hardness/time interaction ( p = 0.329). Chitosan solutions and EDTA reduced the micro-hardness in a similar manner and differed significantly from the control group ( p < 0.001). Chitosan solutions chelated calcium ions throughout the entire experiment. Regardless of the storage form, chitosan demonstrates a chelating property for a minimum period of 6 months.

  11. Prognosis of vulvar dysplasia and carcinoma in situ with special reference to histology and types of human papillomavirus (HPV)

    DEFF Research Database (Denmark)

    Junge, Jette; Poulsen, H; Horn, T

    1997-01-01

    Sixty-one women with vulvar dysplasia or carcinoma in situ were treated with local laser excision of the initial lesion and of the recurrences, and followed at intervals of from 3 increasing to 12 months. Recurrences were observed in 16 (26%) patients. No case of invasive carcinoma was seen. Pati...

  12. Mondini dysplasia

    International Nuclear Information System (INIS)

    Takeda, Taizo; Kakigi, Akinobu; Takeuchi, Shunji; Saito, Haruo

    1992-01-01

    Recent advances in imaging technique, including high resolution thin section computed tomography scanning and magnetic resonance imaging (MRI), permit the easy diagnosis of congenital malformations of the osseous labyrinth, which have so far been lumped together as 'Mondini dysplasia'. In the present study, the anatomic patterns from the radiogrpahic appearance and the clinical manifestation were examined in 18 patients (23 ears) with radiographic abnormalities of the inner ear. The most common abnormal configuration was a large vestibule (20 of 23 ears). This anomaly of the vestibule often involved the lateral semicircular canal, revealed as a round high signal zone on T2-weighted MRI. However, a large vestibule was not always associated with an abnormal cochlea. Abnormal cochleas were found in 8 of the 20 ears with a large vestibule, and most of these 8 ears had total or profound deafness. But 4 of the 10 ears with residual hearing had low tone deafness and 6 had fluctuating hearing loss, which was frequently associated with attacks of dizziness. These clinical manifestations of Mondini dysplasia are similar to those of patients with endolymphatic hydrops. (author)

  13. Clinical application of entire gastrointestinal barium meal combined with multi-temporal abdominal films in patients with intestinal neuronal dysplasia type B.

    Science.gov (United States)

    Li, Xiao-Yun; Li, Xiao-Gang; Du, Ming-Guo; Chen, Zhi-Dan; Tao, Zheng-Gui; Liao, Xiao-Feng

    2013-01-01

    To report and evaluate the application of entire gastrointestinal barium meal combined with multi-temporal abdominal films in the diagnosis of patients with intestinal neuronal dysplasia type B (IND type B). Thirty-six patients with symptoms of long-standing constipation were enrolled in this study. The study took place at the Department of General Surgery, Xiangyang Central Hospital, Hubei Province, China from July 2007 to October 2012. All of them had already been subjected to the tests of barium enema and anorectal manometry and were suspected to be IND type B, but were not confirmed by mucous membrane acetylcholinesterase determination. All underwent the entire gastrointestinal barium meal combined with multi-temporal abdominal films. The data was collected and then analyzed retrospectively. After entire gastrointestinal barium meal combined with multi-temporal abdominal films, 30 out of 36 cases in this group were diagnosed with intestinal neuronal diseases, and then were treated with appropriate surgical treatment. The postoperative pathological diagnosis was IND type B. The other 6 patients in this group still could not be diagnosed explicitly after the test; thus, we treated them with conservative treatment. Entire gastrointestinal barium meal combined with multi-temporal abdominal films has the advantage of being able to test the gastrointestinal transfer capabilities and to find physiological and pathological changes simultaneously. It could provide important proof for the diagnosis of patients with intestinal neuronal dysplasia type B.

  14. Dentin-cement Interfacial Interaction

    Science.gov (United States)

    Atmeh, A.R.; Chong, E.Z.; Richard, G.; Festy, F.; Watson, T.F.

    2012-01-01

    The interfacial properties of a new calcium-silicate-based coronal restorative material (Biodentine™) and a glass-ionomer cement (GIC) with dentin have been studied by confocal laser scanning microscopy (CLSM), scanning electron microscopy (SEM), micro-Raman spectroscopy, and two-photon auto-fluorescence and second-harmonic-generation (SHG) imaging. Results indicate the formation of tag-like structures alongside an interfacial layer called the “mineral infiltration zone”, where the alkaline caustic effect of the calcium silicate cement’s hydration products degrades the collagenous component of the interfacial dentin. This degradation leads to the formation of a porous structure which facilitates the permeation of high concentrations of Ca2+, OH-, and CO32- ions, leading to increased mineralization in this region. Comparison of the dentin-restorative interfaces shows that there is a dentin-mineral infiltration with the Biodentine, whereas polyacrylic and tartaric acids and their salts characterize the penetration of the GIC. A new type of interfacial interaction, “the mineral infiltration zone”, is suggested for these calcium-silicate-based cements. PMID:22436906

  15. New perspectives about molecular arrangement of primary and permanent dentin

    Energy Technology Data Exchange (ETDEWEB)

    Sanches Borges, Ana Flavia [Department of Dental Materials at Piracicaba Dental School, State University of Campinas, Piracicaba, SP (Brazil); Andrade Bitar, Renata [Biomedical Engineering, Valley of Paraiba University (UNIVAP) (Brazil); Kantovitz, Kamila Rosamilia [Department of Pediatric Dentistry at Piracicaba Dental School, State University of Campinas, Piracicaba, SP (Brazil); Bortollazo Correr, Americo [Department of Dental Materials at Piracicaba Dental School, State University of Campinas, Piracicaba, SP (Brazil); Martin, Airton Abrahao [Biomedical Engineering Post Graduation, Valley of Paraiba University (UNIVAP) (Brazil); Puppin-Rontani, Regina Maria [Department of Pediatric Dentistry at Piracicaba Dental School, State University of Campinas, Piracicaba, SP (Brazil)], E-mail: rmpuppin@fop.unicamp.br

    2007-12-30

    The dentin quality of primary and permanent teeth was inspected by Fourier transformed Raman spectroscopy (FT-Raman); scanning electron microscopy/energy-dispersive spectroscopy (SEM/EDS) and hardness test. Middle dentin of crowns were reached by carbide bur abrading providing a uniform smear layer. Phosphoric acid was applied in order to simulate the etching of total etching adhesive systems. The groups were (n = 10): G1 (primary dentin smear layer); G2 (35% phosphoric acid etched primary dentin); G3 (permanent dentin smear layer); G4 (35% phosphoric acid etched permanent dentin). FT-Raman results were subjected to cluster analysis. SEM/EDS were made in order to add the data obtained by FT-Raman. The hardness data were subjected to ANOVA and Tukey test. FT-Raman showed differences among groups, either to organic or inorganic content. For the organic content, primary and permanent dentin became similar after the etching; conversely, the inorganic content showed differences for the two substrates. Hardness test showed no significant differences between primary and permanent dentin, before or after etching, but the etching decreased these values. The mineral content arrangement of primary dentin is different from permanent dentin, independently of the etching. The substrate type did no influence the hardness, but the etching decreased it.

  16. Dysmorphic choroid plexuses and hydrocephalus associated with increased nuchal translucency: early ultrasound markers of de novo thanatophoric dysplasia type II with cloverleaf skull (Kleeblattschaedel).

    Science.gov (United States)

    Tonni, Gabriele; Palmisano, Marcella; Ginocchi, Vladimiro; Ventura, Alessandro; Baldi, Maurizia; Baffico, Ave Maria

    2014-11-01

    Prenatal diagnosis of thanatophoric dysplasia (TD) type II presenting in the first trimester with increased nuchal translucency (NT) and cloverleaf skull (Kleeblattschaedel) have been scantly reported in the medical record. Abnormal choroid plexus has been seen in association with fetal anomalies. Here we described a case of increased NT associated with indented choroid plexuses, early onset hydrocephalus and cloverleaf skull in a fetus subsequently diagnosed at early second trimester to carry a de novo mutation encoding for TD type II. The findings of dysmorphic choroid plexus, early onset hydrocephalus and cloverleaf skull at first trimester scan may be early, useful ultrasound markers of TD type II. Molecular analysis to control for possible overlapping syndromes were performed and resulted negative. Postmortem X-ray and 3D-CT scan confirmed the cloverleaf skull, narrow thorax, straight femur with rhizomelic shortening of the limbs and the presence of a communicating hydrocephalus. © 2014 Japanese Teratology Society.

  17. Oculodentodigital dysplasia

    Directory of Open Access Journals (Sweden)

    Dharmil C Doshi

    2016-01-01

    Full Text Available Oculodentodigital dysplasia is a rare, autosomal dominant disorder with high penetrance and variable expressivity, caused by mutations in the connexin 43 or gap junction protein alpha-1 gene. It has been diagnosed in fewer than 300 people worldwide with an incidence of around 1 in 10 million. It affects many parts of the body, particularly eyes (oculo, teeth (dento, and fingers and/or toes (digital. The common clinical features include facial dysmorphism with thin nose, microphthalmia, syndactyly, tooth anomalies such as enamel hypoplasia, anodontia, microdontia, early tooth loss and conductive deafness. Other less common features are abnormalities of the skin and its appendages, such as brittle nails, sparse hair, and neurological abnormalities. To prevent this syndrome from being overlooked, awareness of possible symptoms is necessary. Early recognition can prevent blindness, dental problems and learning disabilities. Described here is the case of a 21-year-old male who presented to the ophthalmology outpatient department with a complaint of bilateral progressive loss of vision since childhood.

  18. Spondylometaphyseal dysplasia with hypercalcemia

    International Nuclear Information System (INIS)

    Bagga, A.; Srivastava, R.N.; Gupta, S.; Gupta, A.

    1989-01-01

    Kozlowski's spondylometaphyseal dysplasia is characterised by short-trunk dwarfism, platyspondyly, metaphyseal dysplasia and retarded bone age. We report an association of this syndrome with asymptomatic, hypocalciuric hypercalcemia, a previously undocumented finding. (orig.)

  19. Asphyxiating thoracic dysplasia

    International Nuclear Information System (INIS)

    Franzcr, J.; Kozlowski, K.

    2008-01-01

    Asphyxiating Thoracic Dysplasia is the most frequent form of Small Thorax - Short Rib Syndromes. Asphyxiating Thoracic Dysplasia in two patients with different clinical course is reported. Radiographic examination is the only method to diagnose Asphyxiating Thoracic Dysplasia with certainty. The correct diagnosis is important for prognostication and genetic counseling. It also excludes the necessity of further, often expensive investigations. (author)

  20. Spondylo-megaepiphyseal-metaphyseal dysplasia: an unusual bone dysplasia

    International Nuclear Information System (INIS)

    Agarwal, Prachi Pragya; Srinivasan, Ashok; Sharma, Raju; Gupta, Arun Kumar; Kabra, Madhulika

    2003-01-01

    A rare case of spondylo-megaepiphyseal-metaphyseal dysplasia is reported in a 10-year-old boy. The features were metaphyseal dysplasia, markedly defective ossification of vertebral body centres and enlarged epiphyses. Although it shares some features with spondylo-metaphyseal dysplasia, oto-spondylo-megaepiphyseal dysplasia and cleidocranial dysplasia, the presence of several unusual radiological findings sets it apart. (orig.)

  1. Multilineage dysplasia is associated with a poorer prognosis in patients with de novo acute myeloid leukemia with intermediate-risk cytogenetics and wild-type NPM1.

    Science.gov (United States)

    Rozman, María; Navarro, José-Tomás; Arenillas, Leonor; Aventín, Anna; Giménez, Teresa; Alonso, Esther; Perea, Granada; Camós, Mireia; Navarrete, Mayda; Tuset, Esperanza; Florensa, Lourdes; Millá, Fuensanta; Nomdedéu, Josep; de la Banda, Esmeralda; Díaz-Beyá, Marina; Pratcorona, Marta; Garrido, Ana; Navarro, Blanca; Brunet, Salut; Sierra, Jorge; Esteve, Jordi

    2014-10-01

    Acute myeloid leukemia (AML) with myelodysplasia-related changes is characterized by the presence of multilineage dysplasia (MLD), frequently related to high-risk cytogenetics and poor outcome. However, the presence of MLD does not modify the favorable prognostic impact of NPM1 mutation. The prognosis of patients with AML presenting marked dysplasia lacking high-risk cytogenetics and NPM1 mutation is uncertain. We evaluated the prognostic impact of MLD in 177 patients with intermediate-risk cytogenetics AML (IR-AML) and wild-type NPM1. Patients were categorized as MLD-WHO (WHO myelodysplasia criteria; n = 43, 24 %), MLD-NRW (significant MLD non-reaching WHO criteria; n = 16, 9 %), absent MLD (n = 80, 45 %), or non-evaluable MLD (n = 38, 22 %). No differences concerning the main characteristics were observed between patients with or without MLD. Outcome of patients with MLD-WHO and MLD-NRW was similar, and significantly worse than patients lacking MLD. The presence of MLD (66 vs. 80 %, p = 0.03; HR, 95 % CI = 2.3, 1.08-4.08) and higher leukocyte count at diagnosis was the only variable associated with lower probability of complete remission after frontline therapy. Concerning survival, age and leukocytes showed an independent prognostic value, whereas MLD showed a trend to a negative impact (p = 0.087, HR, 95 % CI = 1.426, 0.95-2.142). Moreover, after excluding patients receiving an allogeneic stem cell transplantation in first CR, MLD was associated with a shorter survival (HR, 95 % CI = 1.599, 1.026-2.492; p = 0.038). In conclusion, MLD identifies a subgroup of patients with poorer outcome among patients with IR-AML and wild-type NPM1.

  2. Keratoprosthesis in Ectodermal Dysplasia.

    Science.gov (United States)

    Wozniak, Rachel A F; Gonzalez, Mithra; Aquavella, James V

    2016-07-01

    To describe the complex surgical management and novel medical approach for a keratoprosthesis (KPro Boston type I) in a monocular, 73-year-old patient with ectodermal dysplasia and chronic, noninfectious corneal necrosis. Best-corrected visual acuity (BCVA) was measured with Snellen letters. Surgical intervention included an amniotic membrane graft, complete replacement of the KPro, conjunctival flap graft, corneal donor tissue grafts combined with inferior rectus muscle advancement, periosteal tissue graft, tarso-conjunctival flap construction, and symblepharolysis. Infliximab was used as a medical adjunctive therapy. Initial KPro placement provided a BCVA of 20/25 and long-term stability. Subsequent chronic melting at the optic border necessitated numerous surgeries to prevent extrusion and failure. Ultimate fistulization was addressed with the formation of a surgical pocket. The addition of infliximab promoted ocular surface stability, and the patient has maintained a BCVA of 20/80. Ectodermal dysplasia can result in eyelid and corneal abnormalities, requiring a KPro for visual restoration. In the setting of chronic, sterile corneal melt, novel surgical approaches and the off-label use of infliximab allowed for visual rehabilitation.

  3. Mondini Dysplasia Presenting as Otorrhea without Meningitis

    Directory of Open Access Journals (Sweden)

    Chien-Yu Lin

    2012-12-01

    Full Text Available Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emphasized. Temporal bone computed tomography should be considered in children with unilateral sensorineural or mixed-type hearing impairment.

  4. Mondini dysplasia presenting as otorrhea without meningitis.

    Science.gov (United States)

    Lin, Chien-Yu; Lin, Hung-Ching; Peng, Chun-Chih; Lee, Kuo-Sheng; Chiu, Nan-Chang

    2012-12-01

    Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emphasized. Temporal bone computed tomography should be considered in children with unilateral sensorineural or mixed-type hearing impairment. Copyright © 2012. Published by Elsevier B.V.

  5. An RNA-splicing mutation (G{sup +51VS20}) in the Type II collagen gene (COL2A1) in a family with spondyloepiphyseal dysplasia congenita

    Energy Technology Data Exchange (ETDEWEB)

    Tiller, G.E.; Polumbo, P.A. [Vanderbilt Univ. School of Medicine, Nashville, TN (United States); Weis, M.A.; Eyre, D.R. [Univ. of Washington, Seattle, WA (United States); Gruber, H.E.; Rimoin, D.L.; Cohn, D.H. [Cedars-Sinai Medical Center, Los Angeles, CA (United States)]|[Univ. of California School of Medicine, Los Angeles, CA (United States)

    1995-02-01

    Defects in type II collagen have been demonstrated in a phenotypic continuum of chondrodysplasias that includes achondrogenesis II, hypochondrogenesis, spondyloepiphyseal dysplasia congenita (SEDC), Kniest dysplasia, and Stickler syndrome. We have determined that cartilage from a terminated fetus with an inherited form of SEDC contained both normal {alpha}1(II) collagen chains and chains that lacked amino acids 256-273 of the triple-helical domain. PCR amplification of this region of COL2A1, from genomic DNA, yielded products of normal size, while amplification of cDNA yielded a normal sized species and a shorter fragment missing exon 20. Sequence analysis of genomic DNA from the fetus revealed a G{yields}T transversion at position +5 of intron 20; the affected father was also heterozygous for the mutation. Allele-specific PCR and heteroduplex analysis of a VNTR in COL2A1 independently confirmed the unaffected status of a fetus in a subsequent pregnancy. Thermodynamic calculations suggest that the mutation prevents normal splicing of exon 20 by interfering with binding of U{sub 1} small-nuclear RNA to pre-mRNA, thus leading to skipping of exon 20 in transcripts from the mutant allele. Electron micrographs of diseased cartilage showed intracellular inclusion bodies, which were stained by an antibody to {alpha}1(II) procollagen. Our findings support the hypothesis that {alpha}-chain length alterations that preserve the Gly-X-Y repeat motif of the triple helix result in partial intracellular retention of {alpha}1(II) procollagen and produce mild to moderate chondrodysplasia phenotypes. 50 refs., 6 figs., 1 tab.

  6. Regulation of Reactionary Dentine Formation.

    Science.gov (United States)

    Neves, V C M; Sharpe, P T

    2018-04-01

    During the treatment of dental caries that has not penetrated the tooth pulp, maintenance of as much unaffected dentine as possible is a major goal during the physical removal of decayed mineral. Damage to dentine leads to release of fossilized factors (transforming growth factor-β [TGF-β] and bone morphogenic protein [BMP]) in the dentine that are believed to stimulate odontoblasts to secrete new "tertiary" dentine (reactionary dentine). This is formed on the pulpal surface of existing dentine and rethickens the dentine. We have previously shown that activation of Wnt/β-catenin signaling is pivotal for tooth repair in exposed pulp injury, and the pathway can be activated by small-molecule GSK-3 antagonists, resulting in enhanced reparative dentine formation. Here, we use a nonexposed pulp injury model to investigate the mechanisms of reactionary dentine formation in vivo, using small molecules to modulate the Wnt/β-catenin, TGF-β, and BMP pathways. We found that a local increase of Wnt activation at the injury site enhances reactionary dentine secretion. In addition, inhibition of TGF-β, BMP, or Wnt pathways does not impede reactionary dentine formation, although inhibition of TGF-β and/or BMP signaling does result in more disorganized, nontubular reactionary dentine. This suggests that Wnt/β-catenin signaling plays no major role in the formation of reactionary dentine, but in common with reparative dentine formation, exogenous elevation of Wnt/β-catenin signaling can enhance tertiary dentine formation. Release of latent TGF-β or BMPs from dentine is not required for the deposition of mineral to form reactionary dentine but does play a role in its organization.

  7. Genetics Home Reference: boomerang dysplasia

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Boomerang dysplasia Boomerang dysplasia Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Boomerang dysplasia is a disorder that affects the development ...

  8. Progressive pseudorheumatoid dysplasia misdiagnosed as ...

    African Journals Online (AJOL)

    Progressive pseudorheumatoid dysplasia misdiagnosed as seronegative juvenile idiopathic arthritis. Ozgur Taspinar, Fatih Kelesoglu, Yasar Keskin, Murat Uludag. Abstract. Background: Progressive pseudorheumatoid dysplasia (PPD) is a rare spondylo- epi-metaphyseal dysplasia (SEMD). It can be confused with juvenile ...

  9. TREATMENT OF HIP DYSPLASIA

    OpenAIRE

    Iulian ICLEANU; Mariana CORDUN

    2015-01-01

    In this thesis, our purpose is to show that using physiotherapy on patients with hip dysplasia from the very beginning, in the first months of life, helps treating them faster. Common literature proposes to use physiotherapy on patients with hip dysplasia either after their recovery or in the terminal phase of recovery, claiming that any earlier intervention will prolong the hip recovery. The effects of hip dysplasia reflect over the whole musculoskeletal system, while it hinders the knees (g...

  10. Validity of Serum Pepsinogen I/II Ratio for the Diagnosis of Gastric Epithelial Dysplasia and Intestinal Metaplasia during the Follow-Up of Patients at Risk for Intestinal-Type Gastric Adenocarcinoma

    Directory of Open Access Journals (Sweden)

    Mário Dinis-Ribeiro

    2004-09-01

    Full Text Available A cohort of individuals (n = 136 with lesions as severe as atrophic chronic gastritis (ACG was cross-sectionally evaluated for the validity assessment of pepsinogen I (PGI and pepsinogen II (PGII serum levels for the diagnosis of intestinal metaplasia (IM and gastric dysplasia. PGI/PGII ratio [median (range] was 4 (0.57.5 in patients with ACG (n = 35; 4.6 (1.9-6.8 in type I IM (n = 18; 4.2 (1.4-5.9 in type II or type III IM limited to the antrum and incisura (n = 20; 2.4 (0.4-5.6 in extensive incomplete IM (n = 38; and 1.3 (0.4-6.4 in low-grade dysplasia (n = 23 (P = .002. Using histopathologic data as a reference test, the area under the receiver operating characteristic curves (CI 95% was 0.73 (0.64-0.82 for extensive IM, 0.72 (0.58-0.85 for the diagnosis of dysplasia, and 0.81 (0.66-0.95 for the diagnosis of high-grade dysplasia. Using a PGI/PGII ratio of ≤3 as the cutoff for dysplasia diagnosis, the sensitivity was 70% (62-78%, the specificity was 65% (57-73%, and the negative predictive value estimates were over 90%. No differences in PG levels according to age or gender were observed. Helicobacter pylori did not significantly influence validity measurement estimates. PGI/PGII serum level ratio can be used even in the management of patients with a high a priori probability for a positive test. It may be useful for the exclusion of more advanced lesions (extensive IM and neoplastic lesions.

  11. Genetics Home Reference: osteoglophonic dysplasia

    Science.gov (United States)

    ... 1 link) Genetic Testing Registry: Osteoglophonic dysplasia Other Diagnosis and Management Resources (1 link) Seattle Children's Hospital: Dwarfism and Bone Dysplasias General Information from MedlinePlus (5 ...

  12. The Difference of Structural State and Deformation Behavior between Teenage and Mature Human Dentin

    Directory of Open Access Journals (Sweden)

    Peter Panfilov

    2016-01-01

    Full Text Available Objective. The cause of considerable elasticity and plasticity of human dentin is discussed in the relationship with its microstructure. Methods. Structural state of teenage and mature human dentin is examined by using XRD and TEM techniques, and their deformation behavior under compression is studied as well. Result. XRD study has shown that crystallographic type of calcium hydroxyapatite in human dentin (calcium hydrogen phosphate hydroxide Ca9HPO4(PO45OH; Space Group P63/m (176; a = 9,441 A; c = 6,881 A; c/a = 0,729; Crystallite (Scherrer 200 A is the same for these age groups. In both cases, dentin matrix is X-ray amorphous. According to TEM examination, there are amorphous and ultrafine grain phases in teenage and mature dentin. Mature dentin is stronger on about 20% than teenage dentin, while teenage dentin is more elastic on about 20% but is less plastic on about 15% than mature dentin. Conclusion. The amorphous phase is dominant in teenage dentin, whereas the ultrafine grain phase becomes dominant in mature dentin. Mechanical properties of human dentin under compression depend on its structural state, too.

  13. Parallel post-space preparation in different tooth types ex vivo : deviation from the canal centre and remaining dentine thickness

    NARCIS (Netherlands)

    Huysmans, M. C. D. N. J. M.; Klein, M. H. J.; Kok, G. F.; Whitworth, J. M.

    2007-01-01

    Aim To determine the deviation of parallel-sided twist-drills during post-channel preparation and relate this to tooth type and position. Methodology Human teeth with single root canals were selected: maxillary second premolars (group i); maxillary lateral incisors (group ii); mandibular canines

  14. Hypohidrotic and hidrotic ectodermal dysplasia: a report of two cases.

    Science.gov (United States)

    Vasconcelos Carvalho, Marianne; Romero Souto de Sousa, José; Paiva Correa de Melo, Filipe; Fonseca Faro, Tatiane; Nunes Santos, Ana Clara; Carvalho, Silvia; Veras Sobral, Ana Paula

    2013-07-14

    Ectodermal dysplasias are a large group of syndromes characterized by anomalies in the structures of ectodermal origin. There are 2 major types of this disorder, based on clinical findings: hypohidrotic ectodermal dysplasia and hidrotic ectodermal dysplasia. This clinical classification is very important because clinical professionals involved with this disease need first a clear and practical method of diagnosis. The main oral manifestation of ectodermal dysplasia may be expressed as hypodontia. Thus, dental professionals may be the first to diagnose ectodermal dysplasia. The present article reports one case of each of the main types (hypohidrotic and hidrotic) of ectodermal dysplasia and the authors review the literature regarding the pathogenesis, clinical features, and therapeutic management of this condition.

  15. Spondyloepiphyseal dysplasia congenita. A cause of lethal neonatal dwarfism

    Energy Technology Data Exchange (ETDEWEB)

    Macpherson, R.I.; Wood, B.P.

    1980-07-01

    Spondyloepiphyseal dysplasia congenita is a form of primarily short trunk dwarfism, that is manifest at birth but generally has not been regarded as a cause of lethal neonatal dwarfism. Seven neonates with severe dwarfism are presented. The first survived the newborn period, but the other six were early neonatal deaths. All displayed the clinical and radiologic features of spondyloepiphyseal dysplasia congenita. The striking similarities between spondyloepiphyseal dysplasia congenita and achondrogenesis type 2 are discussed.

  16. The fate of the hip in spondylo-epi-metaphyseal dysplasia: clinical and radiological evaluation of adults with SEMD Handigodu type

    International Nuclear Information System (INIS)

    Siddesh, N.D.; Shah, Hitesh; Joseph, Benjamin

    2012-01-01

    This study was undertaken to document the fate of the hip with reference to its structure and function in patients with spondylo-epi-metaphyseal dysplasia tarda Handigodu type (SEMD HG ). Radiographs of 271 adult patients with SEMD HG were studied to identify the pattern of long-term sequelae in the hips. Several measurements of the proximal femur and acetabulum were made to quantify morphological alterations in the hip. Fifty-four adult patients were examined and administered a questionnaire to evaluate the extent of disability attributable to the hips. Three patterns of changes in the hips were noted: 35% had acetabular protrusio, 33% had subluxation of the hip, and 32% had no protrusio or subluxation. Distinctly different anthropometric measurements and dimensional alterations around the hip were noted in these three patterns. Patients with protrusio were relatively tall while those with subluxation were the shortest. All the patients had developed degenerative arthritis of the hips by the fourth decade of life irrespective of the pattern of hip involvement. The reduction in the range of hip motion and fixed deformities were most severe in patients with protrusio. All the patients had significant disability and very low functional hip scores. Degenerative arthritis of the hip develops in the majority of patients with SEMD HG ; the symptoms are severe enough to warrant reconstructive surgery by the fourth decade of life. Protrusio or subluxation develops in a third of the patients each; both these complications will influence the surgical approach if total hip arthroplasty is planned. (orig.)

  17. Oculoauriculovertebral dysplasia (Goldenhar's syndrome).

    Science.gov (United States)

    Nkrumah, F K

    1971-03-01

    A case of Goldenhar's Syndrome or Oculoauriculovertebral dysplasia in a Ghanaian infant is described. Significant were the additional findings of congenital esophageal atresia and arthrogryposis which have so far not been reported in association with the syndrome.

  18. Periapical Cemento-osseous Dysplasia Is Rarely Diagnosed on Orthopantomograms of Patients with Neurofibromatosis Type 1 and Is Not a Gender-specific Feature of the Disease.

    Science.gov (United States)

    Friedrich, Reinhard E; Reul, Anika

    2018-04-01

    Several skeletal aberrations of the skull have been described for the tumor predisposition syndrome neurofibromatosis type 1 (NF1). Recently, periapical cemental/cemento-osseous dysplasia (COD) has been described in females affected with NF1. This reactive lesion of the hard tissues in tooth-bearing areas of the jaw has been proposed to represent a gender-specific radiological feature of NF1. The aim of this study was to investigate the prevalence of COD in patients with NF1. The orthopantomograms (OPGs) of 179 patients with a confirmed diagnosis of NF1 were analyzed for COD. The results were compared to radiographic findings obtained in OPGs of age- and sex-matched controls. The NF1 patient group was further differentiated according to the evidence of facial plexiform neurofibroma. COD was a very rare finding in both groups. The extension of the diagnostic criteria including radiologically-healthy teeth and a widened periodontal gap in the periapical area only marginally increased the number of considered cases. Although there was a somewhat more common occurrence of such changes in the patient group compared to the control group and the number of affected women was greater than the number of men, none of these differences reached statistical significance. Furthermore, COD or widening of the periradicular periodontal space was not found to be associated with facial tumor type in NF1. The investigation revealed that COD is not a diagnostic feature of NF1. There is no clear association of the rare finding of COD with gender. These studies should be compared with patient groups of other ethnic backgrounds. Copyright© 2018, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  19. Expansive focal cemento-osseous dysplasia.

    Science.gov (United States)

    Bulut, Emel Uzun; Acikgoz, Aydan; Ozan, Bora; Zengin, Ayse Zeynep; Gunhan, Omer

    2012-01-01

    To present a case of expansive focal cemento-osseous dysplasia and emphasize the importance of differential diagnosis. Cemento-osseous dysplasia is categorized into three subtypes on the basis of the clinical and radiographic features: Periapical, focal and florid. The focal type exhibits a single site of involvement in any tooth-bearing or edentulous area of the jaws. These lesions are usually asymptomatic; therefore, they are frequently diagnosed incidentally during routine radiographic examinations. Lesions are usually benign, show limited growth, and do not require further surgical intervention, but periodic follow-up is recommended because occasionally, this type of dysplasia progresses into florid osseous dysplasia and simple bone cysts are formed. A 24-year-old female patient was referred to our clinic for swelling in the left edentulous mandibular premolarmolar region and felt discomfort when she wore her prosthetics. She had no pain, tenderness or paresthesia. Clinical examination showed that the swelling in the posterior mandible that was firm, nonfluctuant and covered by normal mucosa. On panoramic radiography and computed tomography, a well defined lesion of approximately 1.5 cm in diameter of mixed density was observed. The swelling increased slightly in size over 2 years making it difficult to use prosthetics and, therefore, the lesion was totally excised under local anesthesia, and surgical specimens were submitted for histopathological examination. The histopathological diagnosis was focal cemento-osseous dysplasia. In the present case, because of the increasing size of the swelling making it difficult to use prosthetics, young age of the patient and localization of the lesion, in the initial examination, cemento-ossifying fibroma was suspected, and the lesion was excised surgically; the histopathological diagnosis confirmed it as focal cemento-osseous dysplasia. We present a case of expansive focal cemento-osseous dysplasia. Differential diagnosis

  20. Gracile bone dysplasias

    International Nuclear Information System (INIS)

    Kozlowski, Kazimierz; Masel, John; Sillence, David O.; Arbuckle, Susan; Juttnerova, Vera

    2002-01-01

    Gracile bone dysplasias constitute a group of disorders characterised by extremely slender bones with or without fractures. We report four newborns, two of whom showed multiple fractures. Two babies had osteocraniostenosis and one had features of oligohydramnios sequence. The diagnosis in the fourth newborn, which showed thin long bones and clavicles and extremely thin, poorly ossified ribs, is uncertain. Exact diagnosis of a gracile bone dysplasia is important for genetic counselling and medico-legal reasons. (orig.)

  1. Ectodermal dysplasia (ED) syndrome.

    Science.gov (United States)

    Chee, Siew-Yin; Wanga, Chung-Hsing; Lina, Wei-De; Tsaia, Fuu-Jen

    2014-01-01

    Ectodermal dysplasia (ED) syndrome comprises a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of 2 or more tissues derived from the embryonic ectoderm. The tissues primarily involved are the skin and its appendages (including hair follicles, eccrine glands, sebaceous glands, nails) and teeth. The clinical features include sparse hair, abnormal or missing teeth, and an inability to sweat due to lack of sweat glands. One such case report of ectodermal dysplasia is presented here.

  2. Gracile bone dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, Kazimierz [Department of Medical Imaging, The Children' s Hospital at Westmead, Locked Bag 4001, Westmead 2145, NSW (Australia); Masel, John [Department of Radiology, Royal Children' s Hospital, Brisbane (Australia); Sillence, David O. [Department of Paediatrics and Child Health, The University of Sydney (Australia); Arbuckle, Susan [Department of Anatomical Pathology, The Children' s Hospital at Westmead, NSW (Australia); Juttnerova, Vera [Oddeleni Lekarske Genetiky, Hradec Kralove (Czech Republic)

    2002-09-01

    Gracile bone dysplasias constitute a group of disorders characterised by extremely slender bones with or without fractures. We report four newborns, two of whom showed multiple fractures. Two babies had osteocraniostenosis and one had features of oligohydramnios sequence. The diagnosis in the fourth newborn, which showed thin long bones and clavicles and extremely thin, poorly ossified ribs, is uncertain. Exact diagnosis of a gracile bone dysplasia is important for genetic counselling and medico-legal reasons. (orig.)

  3. Mandibulo-acral dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Hoeffel, J.C.; Mainard, L. [Dept. of Radiology, Children' s Hospital, Vandoeuvre (France); Chastagner, P. [Dept. of Medicine, Children' s Hospital, Vandoeuvre (France); Hoeffel, C.C. [UFR Faculte de Medecine Cochin, Paris (France)

    2000-11-01

    We report on a 7 year-old-girl with mandibulo-acral dysplasia. When she was 3 years of age it mimicked scleroderma because of skin atrophy and later on a Hutchinson-Gilford progeria syndrome (HGP). Acro-mandibular dysplasia was diagnosed because of facial hypoplasia and mandibular hypoplasia. The bilateral proximal mid-humeral notch seen in this case is unusual. (orig.)

  4. The fate of the hip in spondylo-epi-metaphyseal dysplasia: clinical and radiological evaluation of adults with SEMD Handigodu type

    Energy Technology Data Exchange (ETDEWEB)

    Siddesh, N.D.; Shah, Hitesh; Joseph, Benjamin [Kasturba Medical College, Paediatric Orthopaedic Service, Manipal, Karnataka State (India)

    2012-08-15

    This study was undertaken to document the fate of the hip with reference to its structure and function in patients with spondylo-epi-metaphyseal dysplasia tarda Handigodu type (SEMD{sub HG}). Radiographs of 271 adult patients with SEMD{sub HG} were studied to identify the pattern of long-term sequelae in the hips. Several measurements of the proximal femur and acetabulum were made to quantify morphological alterations in the hip. Fifty-four adult patients were examined and administered a questionnaire to evaluate the extent of disability attributable to the hips. Three patterns of changes in the hips were noted: 35% had acetabular protrusio, 33% had subluxation of the hip, and 32% had no protrusio or subluxation. Distinctly different anthropometric measurements and dimensional alterations around the hip were noted in these three patterns. Patients with protrusio were relatively tall while those with subluxation were the shortest. All the patients had developed degenerative arthritis of the hips by the fourth decade of life irrespective of the pattern of hip involvement. The reduction in the range of hip motion and fixed deformities were most severe in patients with protrusio. All the patients had significant disability and very low functional hip scores. Degenerative arthritis of the hip develops in the majority of patients with SEMD{sub HG}; the symptoms are severe enough to warrant reconstructive surgery by the fourth decade of life. Protrusio or subluxation develops in a third of the patients each; both these complications will influence the surgical approach if total hip arthroplasty is planned. (orig.)

  5. Temperature increase beneath etched dentin discs during composite polymerization.

    Science.gov (United States)

    Karaarslan, Emine Sirin; Secilmis, Asli; Bulbul, Mehmet; Yildirim, Cihan; Usumez, Aslihan

    2011-01-01

    The purpose of this in vitro study was to measure the temperature increase during the polymerization of a composite resin beneath acid-etched or laser-etched dentin discs. The irradiation of dentin with an Er:YAG laser may have a positive effect on the thermal conductivity of dentin. This technique has not been studied extensively. Forty dentin discs (5 mm in diameter and 0.5 or 1 mm in height) were prepared from extracted permanent third molars. These dentin discs were etched with 20% orthophosphoric acid or an Er:YAG laser, and were then placed on an apparatus developed to measure temperature increases. The composite resin was polymerized with a high-intensity quartz tungsten halogen (HQTH) or light-emitting diode unit (LED). The temperature increase was measured under the dentin disc with a J-type thermocouple wire that was connected to a data logger. Five measurements were made for each dentin disc, curing unit, and etching system combination. Differences between the initial and the highest temperature readings were taken, and the five calculated temperature changes were averaged to determine the value of the temperature increase. Statistical analysis was performed with a three-way ANOVA and Tukey HSD tests at a 0.05 level of significance. Further SEM examinations were performed. The temperature increase values varied significantly, depending on etching systems (p < 0.05), dentin thicknesses (p < 0.05), and curing units (p < 0.05). Temperature increases measured beneath laser-etched discs were significantly higher than those for acid-etched dentin discs (p < 0.05). The HQTH unit induced significantly higher temperature increases than the LED unit (p < 0.05). The LED unit induced the lowest temperature change (5.2°C) in the 1-mm, acid-etched dentin group. The HQTH unit induced the highest temperature change (10.4°C) for the 0.5-mm, laser-etched dentin group. The risk of heat-induced pulpal damage should be taken into consideration

  6. Cloverleaf skull with generalised bone dysplasia

    International Nuclear Information System (INIS)

    Kozlowski, K.; Warren, P.S.; Fisher, C.C.; Royal Hospital for Women, Camperdown

    1985-01-01

    A case of cloverleaf skull with generalised bone dysplasia is reported. The authors believe that bone dysplasia associated with cloverleaf is neither identical with thanatophoric dysplasia nor achondroplasia. Until identity of thanatophoric dysplasia and cloverleaf skull with generalised bone dysplasia is proved the diseases should be looked upon as separate entities and the wording ''thanatophoric dysplasia with cloverleaf skull'' should be abolished. (orig.)

  7. Temperature rise produced by different light-curing units through dentin.

    Science.gov (United States)

    Yazici, A Rüya; Müftü, Ali; Kugel, Gerard

    2007-11-01

    This study investigated the temperature rise caused by different light curing units and the temperature increase in dentin of different thicknesses. Dentin discs of 1.0 and 2.0 mm thicknesses were prepared from extracted human mandibular molars. Temperatures were recorded directly at the surface of the light guide tip, under dentin discs with different thicknesses, and through a sandwich composed of 2 mm thick cured composite and dentin using a K-type thermocouple. The curing units used were two quartz-tungsten-halogen lights (Spectrum and Elipar Trilight-ET) and a light-emitting diode (LED). The highest temperature rise was observed under a Mylar strip using ET standard mode. Under 1 and 2 mm thick dentin barriers, the lowest temperature rise was measured for the LED curing light. Significant differences in temperature rise existed among all curing units except between the Spectrum and ET exponential modes under a 1 mm thick dentin barrier with cured composite. Temperature rises were insignificant between the Spectrum and ET exponential modes and between two modes of Trilight when the same experimental setup was used under a 2 mm thick dentin barrier. For all curing units, temperature elevation through 2 mm of dentin was less than for 1 mm of dentin thickness. The ET standard mode produced the highest and the LED produced the lowest temperature rise for all tested conditions. The thickness of dentin and light-curing unit might affect temperature transmission.

  8. In vivo model for microbial invasion of tooth root dentinal tubules

    Directory of Open Access Journals (Sweden)

    Jane L. BRITTAN

    2016-04-01

    Full Text Available ABSTRACT Objective Bacterial penetration of dentinal tubules via exposed dentine can lead to root caries and promote infections of the pulp and root canal system. The aim of this work was to develop a new experimental model for studying bacterial invasion of dentinal tubules within the human oral cavity. Material and Methods Sections of human root dentine were mounted into lower oral appliances that were worn by four human subjects for 15 d. Roots were then fixed, sectioned, stained and examined microscopically for evidence of bacterial invasion. Levels of invasion were expressed as Tubule Invasion Factor (TIF. DNA was extracted from root samples, subjected to polymerase chain reaction amplification of 16S rRNA genes, and invading bacteria were identified by comparison of sequences with GenBank database. Results All root dentine samples with patent tubules showed evidence of bacterial cell invasion (TIF value range from 5.7 to 9.0 to depths of 200 mm or more. A spectrum of Gram-positive and Gram-negative cell morphotypes were visualized, and molecular typing identified species of Granulicatella, Streptococcus, Klebsiella, Enterobacter, Acinetobacter, and Pseudomonas as dentinal tubule residents. Conclusion A novel in vivo model is described, which provides for human root dentine to be efficiently infected by oral microorganisms. A range of bacteria were able to initially invade dentinal tubules within exposed dentine. The model will be useful for testing the effectiveness of antiseptics, irrigants, and potential tubule occluding agents in preventing bacterial invasion of dentine.

  9. Similar PDK1-AKT-mTOR pathway activation in balloon cells and dysmorphic neurons of type II focal cortical dysplasia with refractory epilepsy.

    Science.gov (United States)

    Lin, Yuan-xiang; Lin, Kun; Kang, De-zhi; Liu, Xin-xiu; Wang, Xing-fu; Zheng, Shu-fa; Yu, Liang-hong; Lin, Zhang-ya

    2015-05-01

    Dysmorphic neurons and balloon cells constitute the neuropathological hallmarks of type II focal cortical dysplasias (FCDs) with refractory epilepsy. The genesis of these cells may be critical to the histological findings in type II FCD. Recent work has shown enhanced activation of the mTOR cascade in both balloon cells and dysmorphic neurons, suggesting a common pathogenesis for these two neuropathological hallmarks. A direct comparative analysis of balloon cells and dysmorphic neurons might identify a molecular link between balloon cells and dysmorphic neurons. Here, we addressed whether PDK1-AKT-mTOR activation differentiates balloon cells from dysmorphic neurons. We used immunohistochemistry with antibodies against phosphorylated (p)-PDK1 (Ser241), p-AKT (Thr308), p-AKT (Ser473), p-mTOR (Ser2448), p-P70S6K (Thr229), and p-p70S6 kinase (Thr389) in balloon cells compared with dysmorphic neurons. Strong or moderate staining for components of the PDK1-AKT-mTOR signaling pathway was observed in both balloon cells and dysmorphic neurons. However, only a few pyramidal neurons displayed weak staining in control group (perilesional neocortex and histologically normal neocortex). Additionally, p-PDK1 (Ser241) and p-AKT (Thr308) staining in balloon cells were stronger than in dysmorphic neurons, whereas p-P70S6K (Thr229) and p-p70S6 kinase (Thr389) staining in balloon cells was weaker than in dysmorphic neurons. In balloon cells, p-AKT (Ser473) and p-mTOR (Ser2448) staining was comparable with the staining in dysmorphic neurons. Our data support the previously suggested pathogenic relationship between balloon cells and dysmorphic neurons concerning activation of the PDK1-AKT-mTOR, which may play important roles in the pathogenesis of type II FCD. Differential expression of some components of the PDK1-AKT-mTOR pathway between balloon cells and dysmorphic neurons may result from cell-specific gene expression. Copyright © 2015 Elsevier B.V. All rights reserved.

  10. Artificial intelligence estimates the impact of human papillomavirus types in influencing the risk of cervical dysplasia recurrence: progress toward a more personalized approach.

    Science.gov (United States)

    Bogani, Giorgio; Ditto, Antonino; Martinelli, Fabio; Signorelli, Mauro; Chiappa, Valentina; Leone Roberti Maggiore, Umberto; Taverna, Francesca; Lombardo, Claudia; Borghi, Chiara; Scaffa, Cono; Lorusso, Domenica; Raspagliesi, Francesco

    2018-01-22

    The objective of this study was to determine whether the pretreatment human papillomavirus (HPV) genotype might predict the risk of cervical dysplasia persistence/recurrence. Retrospective analysis of prospectively collected data of consecutive 5104 women who underwent the HPV-DNA test were matched with retrospective data of women undergoing either follow-up or medical/surgical treatment(s) for genital HPV-related infection(s). Artificial neuronal network (ANN) analysis was used in order to weight the importance of different HPV genotypes in predicting cervical dysplasia persistence/recurrence. ANN simulates a biological neuronal system from both the structural and functional points of view: like neurons, ANN acquires knowledge through a learning-phase process and allows weighting the importance of covariates, thus establishing how much a variable influences a multifactor phenomenon. Overall, 5104 women were tested for HPV. Among them, 1273 (25%) patients underwent treatment for HPV-related disorders. LASER conization and cervical vaporization were performed in 807 (59%) and 386 (30%) patients, respectively, and secondary cervical conization in 45 (5.5%). ANN technology showed that the most important genotypes predicting cervical dysplasia persistence/recurrence were HPV-16 (normalized importance: 100%), HPV-59 (normalized importance: 51.2%), HPV-52 (normalized importance: 47.7%), HPV-18 (normalized importance: 32.8%) and HPV-45 (normalized importance: 30.2%). The pretreatment diagnosis of all of those genotypes, except HPV-45, correlated with an increased risk of cervical dysplasia persistence/recurrence; the pretreatment diagnosis was also arrived at using standard univariate and multivariable models (P<0.01). Pretreatment positivity for HPV-16, HPV-18, HPV-52 and HPV-59 might correlate with an increased risk of cervical dysplasia persistence/recurrence after treatment. These data might be helpful during patients' counseling and to implement new vaccination

  11. Genetics Home Reference: thanatophoric dysplasia

    Science.gov (United States)

    ... thanatophoric dysplasia and their potential therapeutic implications for achondroplasia. Am J Med Genet A. 2010 Jan;152A( ... of fibroblast growth factor receptor 3 disorders: the achondroplasia family of skeletal dysplasias, Muenke craniosynostosis, and Crouzon ...

  12. TREATMENT OF HIP DYSPLASIA

    Directory of Open Access Journals (Sweden)

    Iulian ICLEANU

    2015-11-01

    Full Text Available In this thesis, our purpose is to show that using physiotherapy on patients with hip dysplasia from the very beginning, in the first months of life, helps treating them faster. Common literature proposes to use physiotherapy on patients with hip dysplasia either after their recovery or in the terminal phase of recovery, claiming that any earlier intervention will prolong the hip recovery. The effects of hip dysplasia reflect over the whole musculoskeletal system, while it hinders the knees (genu valgum, the ankles (ankle valgus, calcaneal valgus and the spine (scoliosis especially at the lumbar level. The most spectacular are at the hip level, that is why we made an analytical evaluation only for this joint. To show the importance of physiotherapy for children with hip dysplasia we started from the hypothesis: untimely treatment for children with hip dysplasia has improved results in functional recovery and in obtaining a better stability, without the necessity of orthopedics or surgical interventions. The research methods used in this study are: the observation method, the bibliographic study method, the experimental method, the graphics method and the statistical mathematical method to process the data and to represent the results graphically. In the end, the results obtained are significantly different from the initial evaluations and we came to the conclusion that starting an untimely analytical kinetic treatment and globally personalizing it to every patient improves stability and biomechanical parameters for the hip.

  13. Occipital projections in the skeletal dysplasias

    International Nuclear Information System (INIS)

    Takamine, Yuji; Field, Fiona M.; Lachman, Ralph S.; Rimoin, David L.

    2004-01-01

    Occipital projections of the cranium have been reported in a number of skeletal dysplasias and syndromes. We observed two cases of atelosteogenesis type I with a bony occipital projection. This finding has neither been noted nor reported in any form of atelosteogenesis. This led us to search the International Skeletal Dysplasia Registry for occipital projections, and we found them in four other syndromes in which they had not been reported. Thus occipital spurs are a non-diagnostic feature that can be found in at least ten distinct disorders as well as a normal variant. (orig.)

  14. Upper-extremity phocomelia reexamined: a longitudinal dysplasia.

    Science.gov (United States)

    Goldfarb, Charles A; Manske, Paul R; Busa, Riccardo; Mills, Janith; Carter, Peter; Ezaki, Marybeth

    2005-12-01

    In contrast to longitudinal deficiencies, phocomelia is considered a transverse, intercalated segmental dysplasia. Most patients demonstrate severe, but not otherwise classifiable, upper-extremity deformities, which usually cannot be placed into one of three previously described phocomelia groups. Additionally, these phocomelic extremities do not demonstrate true segmental deficits; the limb is also abnormal proximal and distal to the segmental defect. The purpose of this investigation was to present evidence that upper-extremity abnormalities in patients previously diagnosed as having phocomelia in fact represent a proximal continuum of radial or ulnar longitudinal dysplasia. The charts and radiographs of forty-one patients (sixty extremities) diagnosed as having upper-extremity phocomelia were reviewed retrospectively. On the basis of the findings on the radiographs, the disorders were categorized into three groups: (1) proximal radial longitudinal dysplasia, which was characterized by an absent proximal part of the humerus, a nearly normal distal part of the humerus, a completely absent radius, and a radial-sided hand dysplasia; (2) proximal ulnar longitudinal dysplasia, characterized by a short one-bone upper extremity that bifurcated distally and by severe hand abnormalities compatible with ulnar dysplasia; and (3) severe combined dysplasia, with type A characterized by an absence of the forearm segment (i.e., the radius and ulna) and type B characterized by absence of the arm and forearm (i.e., the hand attached to the thorax). Twenty-nine limbs in sixteen patients could be classified as having proximal radial longitudinal dysplasia. Systemic medical conditions such as thrombocytopenia-absent radius syndrome were common in those patients, but additional musculoskeletal conditions were rare. Twenty limbs in seventeen patients could be classified as having proximal ulnar longitudinal dysplasia. Associated musculoskeletal abnormalities, such as proximal femoral

  15. Meyer's dysplasia epiphysealis

    International Nuclear Information System (INIS)

    Toro P, Alvaro de Jesus; Lopez C, Meisser A

    2005-01-01

    The skeletal dysplasias are a group of heterogeneous conditions since the clinical and genetic point of view, that to date they are about 200 different disorders. They have as common denominator an inconvenience of the normal process of growth and development of the bony weaving and their cartilaginous precursors. In this article the case of an adolescent of 16 years is presented with Meyer's dysplasia epiphysealis whose diagnostic was delayed and that as opposed to the cases reported in the literature required surgical treatment due to the persistent symptoms, and the literature is reviewed

  16. Dentine tubule infection and endodontic therapy implications.

    Science.gov (United States)

    Oguntebi, B R

    1994-07-01

    A critical review of the literature suggests that the microenvironment of dentinal tubules appears to favour the selection of relatively few bacterial types irrespective of the aetiology of the infection process; coronal dental caries or pulpar necrosis. These bacteria may constitute an important reservoir from which root canal infection and reinfection may occur following pulp necrosis or during and after endodontic treatment. Previous studies of this microflora have utilized microbiological culture techniques which need to be supplemented by those that allow in situ demonstration as well as identification of the bacteria. Newer treatment strategies that are designed to eliminate this microflora must include agents that can penetrate the dentinal tubules and destroy these microorganisms, since they are located in an area beyond the host defence mechanisms where they cannot be reached by systemically administered antimicrobial agents.

  17. Fibrous Dysplasia versus Juvenile Ossifying Fibroma: A Dilemma

    Directory of Open Access Journals (Sweden)

    Sreelakshmi N. Nair

    2016-01-01

    Full Text Available Fibrous dysplasia (FD is a condition characterized by excessive proliferation of bone forming mesenchymal cells which can affect one bone (monostotic type or multiple bones (polyostotic type. It is predominantly noticed in adolescents and young adults. Fibrous dysplasia affecting the jaws is an uncommon condition. The most commonly affected facial bone is the maxilla, with facial asymmetry being the chief complaint. The lesion in many instances is confused with ossifying fibroma (OF. Diagnosis of these two lesions has to be done based on clinical, radiographic, and microscopic findings. Here, we present a case of fibrous dysplasia of maxilla in a nine-year-old boy mimicking juvenile ossifying fibroma.

  18. Human papilloma virus infection and cervical dysplasia.

    Science.gov (United States)

    Melinte-Popescu, Alina; Costăchescu, Gh

    2012-01-01

    Pap testing is considered to be the best screening tool for cervical cancer but there is currently great interest in the possible application of human papilloma virus (HPV) testing to supplement Pap screening for cervical cancer. To determine the prevalence of high-risk HPV types in the studied population and to explore the association between high-risk HPV types and cervical dysplasia. Cross-sectional study conducted at the Iasi Cuza Voda Obstetrics-Gynecology Hospital and Suceava County Hospital. 332 women who underwent colposcopy for cervical lesions between 2006 and 2011 were included in this study. The overall prevalence of HPV was 57.23%. HPV prevalence differs significantly in the three age groups up to 50 years. It was highest in patients below the age of 40 and progressively lower with advancing age. The overall prevalence of cervical dysplasia was 56.62%. The prevalence of cervical dysplasia was highest in the age groups up to 40 years. The most important determinant of HPV infection is age. Persistence of HPV appears to be associated with progression to squamous intraepithelial lesion. Dysplasia is often missed in a cervical sample either because of human error in screening and interpretation, or because of suboptimal quality of Pap smear. Incorporation of HPV testing into the present Pap screening program has the potential of making screening for cervical cancer more effective, and a necessary prelude to assessing this is by determining the prevalence of the high-risk types.

  19. Thanatophoric dysplasia: Antenatal to postmortem

    Directory of Open Access Journals (Sweden)

    Chanabasappa V Chavadi

    2015-01-01

    Full Text Available Thanatophoric dwarfism (TD, literally meaning death seeking dwarf, is the most common form of lethal bone dysplasia characterized by severe micromelia, extra folds of skin and narrow chest. Other signs include small ribs, underdeveloped lungs, cloverleaf skull, hypertelorism and protuberant abdomen. A short neck, depressed nasal bridge and hypoplastic mandible may also be present. Hydrocephalus is uncommon but is another poor prognostic sign. The diagnosis is usually made with the ultrasonography in the second trimester. Based on the morphological characters, this condition is sub-divided into type 1 and type 2. Fetuses with this condition are either still-born or die shortly after birth. Antenatal sonographic, postpartum radiological and autopsy findings of a case of type 1 TD with a relatively uncommon association of hydrocephalus is discussed.

  20. Relationship between mineral distributions in dentine lesions and subsequent remineralization in vitro

    NARCIS (Netherlands)

    Kawasaki, K; Ruben, J; Tsuda, H; Huysmans, MCDNJM; Takagi, O

    2000-01-01

    Though the mineral distribution of the dentine carious lesion varies largely from tooth to tooth and from patient to patient, there are two main distribution profiles that characterize natural carious lesions in dentine. These profiles include softened and subsurface lesion types. The mineral

  1. Functional remineralization of carious dentin

    Science.gov (United States)

    Pugach, Megan Kardon

    A primary goal of dental tissue engineering is the biological reconstruction of tooth substrate destroyed by caries or other diseases affecting tooth mineralization. Traditionally, dentists treat caries by using invasive techniques to remove the diseased dental tissue and restore the lesion, ideally preventing further progression of decay. Success in strategies associated with remineralization of enamel and root caries have contributed to the less invasive prospect of remineralization of dentinal carious lesions. The central hypothesis of this dissertation is that carious dentin lesions can be remineralized if the lesions contain residual mineral. Caries Detector (CD) stained zones (pink, light pink, transparent and normal) of arrested carious dentin lesions were characterized according to microstructure by atomic force microscopy (AFM) imaging, mineral content by digital transverse microradiography, and nanomechanical properties by AFM-based nanoindentation. CD-stained and unstained zones had significantly different microstructure, mineral content and nanomechanical properties. Furthermore, the most demineralized carious zone contained residual mineral. To obtain reproducible, standardized dentin caries lesions, we characterized the lesions from an artificial carious dentin lesion model using a 0.05M acetate demineralization buffer. The artificial caries-like lesions produced by the buffer had similar mineral content and nanomechanical properties in the stained and unstained zones as natural dentin lesions. Both natural and artificial lesions had significant correlations between mineral content and nanomechanical properties. Mineral crystallite size and shape was examined by small angle x-ray scattering. Both natural and artificial carious dentin had different mineral sizes than normal dentin. Collagen in natural and artificial carious dentin lesions was examined by trichrome stain, AFM high-resolution imaging, and UV resonance Raman spectroscopy, to determine if

  2. Anomalies of tooth formation in hypohidrotic ectodermal dysplasia

    DEFF Research Database (Denmark)

    Lexner, Michala O; Bardow, Allan; Hertz, Jens Michael

    2007-01-01

    OBJECTIVE: The X-linked hypohidrotic ectodermal dysplasia (HED) is the most common type of ectodermal dysplasia. The clinical identification of possible heterozygous females can be difficult because of the varying degrees of clinical signs caused by X-chromosome inactivation. This study is the fi......OBJECTIVE: The X-linked hypohidrotic ectodermal dysplasia (HED) is the most common type of ectodermal dysplasia. The clinical identification of possible heterozygous females can be difficult because of the varying degrees of clinical signs caused by X-chromosome inactivation. This study...... affected males had multiple missing permanent teeth and tooth malformations. The heterozygous females had a significantly higher frequency of agenesis of permanent teeth compared to normative data. The heterozygous females had an increased prevalence of tooth malformations and reduced tooth size...

  3. Ectodermal Dysplasia: A Case Report

    Science.gov (United States)

    2011-01-01

    Ectodermal dysplasia is a hereditary disease characterized by dysplasia of tissues of ectodermal origin. The incidence of ectodermal dysplasia is rare (1 in 100,000 birth). This case report discusses the features, classification and prosthetic treatment plan (upper partial denture and lower complete denture for upper partial and lower complete edentulous arches respectively). This treatment plan would be able to provide psychological and functional boost to the sufferer. PMID:27678241

  4. Familial ectodermal dysplasia: a peers' agony.

    Science.gov (United States)

    Hegde, Karthik; Kashyap, Roopashri Rajesh; Nair, Gopakumar; Nair, Preeti P

    2013-07-23

    Ectodermal dysplasias include a various group of inherited disorders which share primary defect in the development of two or more tissues of embryonic ectodermal origin. Though there are many subtypes, ectodermal dysplasias are mainly hidrotic ectodermal dysplasia and hypohidrotic ectodermal dysplasia, among which the most common variety is X linked hypohidrotic ectodermal dysplasia. We report a rare case of X linked hypohidrotic ectodermal dysplasia occurring in a family with various skin, hair and oral abnormalities.

  5. Cloverleaf skull with generalised bone dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kozlowski, K.; Warren, P.S.; Fisher, C.C.

    1985-09-01

    A case of cloverleaf skull with generalised bone dysplasia is reported. The authors believe that bone dysplasia associated with cloverleaf is neither identical with thanatophoric dysplasia nor achondroplasia. Until identity of thanatophoric dysplasia and cloverleaf skull with generalised bone dysplasia is proved the diseases should be looked upon as separate entities and the wording ''thanatophoric dysplasia with cloverleaf skull'' should be abolished.

  6. Ectodermal Dysplasia Skin Fragility Syndrome

    Directory of Open Access Journals (Sweden)

    Ayça Alan Atalay

    2014-06-01

    Full Text Available Ectodermal dysplasia-skin fragility syndrome (EDSFS is a rare autosomal recessive genodermatosis first described in 1997 by Mc Grath. EDSFS results from loss of function mutations in plakophilin-1 (PKP1. PKP1 is a structural component of desmosomes, cellcell adhesion complexes. It is also found as a nuclear protein in several cell types that are lack of desmosomes. In skin, however, PKP1 expression is confined mainly to suprabasal keratinocytes and the outer root sheath of hair follicules. Loss of function mutation in PKP1 leads to extensive skin fragility, bullae and erosions following minor trauma, focal keratoderma with painful fissures, alopecia, and nail dystrophy. In some patients hypohidrosis may also be seen. EDSFS is now considered as a specific suprabasal form of epidermolysis bullosa simplex. In this report we describe a 20 year old EDSFS case.

  7. The fracture properties and toughening mechanisms of bone and dentin

    Science.gov (United States)

    Koester, Kurt John

    The mechanical properties of bone and dentin and in particular their fracture properties, are the subject of intense research. The relevance of these properties is increasing as our population ages and fracture incidence impacts the lives of a greater portion of the population. A robust framework is needed to understand the fracture properties of bone and dentin to guide researchers as they attempt to characterize the effects of aging, disease, and pharmaceutical treatments on the properties of these mineralized tissues. In the present work, this framework is provided and applied to human bone, human dentin, and animal bone. In situ electron microscopy was also used to identify the salient toughening mechanisms in bone and dentin. It was found that bone and dentin are extrinsically toughened materials and consequently their fracture properties are best characterized utilizing a crack-growth resistance approach. A description of the different mechanical measurements commonly employed when using small animal models (rats and mice) to evaluate the influence of drug therapies on bone fragility is provided. A study where these properties were measured for a large population of wild-type rats and mice was also conducted. Given my findings, it was determined that for the most complete understanding of small animal bone it was necessary to measure strength and toughness. Strength measurements probe the flaw distribution and toughness measurements to evaluate the resistance to facture in the presence of a single dominant worst-case flaw.

  8. Dentin Permeability of Carious Primary Teeth

    African Journals Online (AJOL)

    primary dental pulp make it difficult to determine which modality offers the best ... The most common pathology of the dentine is dental caries. ... to evaluate dentine permeability is to calculate its hydraulic conductance (Lp) using fluid filtration ...

  9. Improved reactive nanoparticles to treat dentin hypersensitivity.

    Science.gov (United States)

    Toledano-Osorio, Manuel; Osorio, Estrella; Aguilera, Fátima S; Luis Medina-Castillo, Antonio; Toledano, Manuel; Osorio, Raquel

    2018-05-01

    The aim of this study was to evaluate the effectiveness of different nanoparticles-based solutions for dentin permeability reduction and to determine the viscoelastic performance of cervical dentin after their application. Four experimental nanoparticle solutions based on zinc, calcium or doxycycline-loaded polymeric nanoparticles (NPs) were applied on citric acid etched dentin, to facilitate the occlusion and the reduction of the fluid flow at the dentinal tubules. After 24 h and 7 d of storage, cervical dentin was evaluated for fluid filtration. Field emission scanning electron microscopy, energy dispersive analysis, AFM and Nano-DMA analysis were also performed. Complex, storage, loss modulus and tan delta (δ) were assessed. Doxycycline-loaded NPs impaired tubule occlusion and fluid flow reduction trough dentin. Tubules were 100% occluded in dentin treated with calcium-loaded NPs or zinc-loaded NPs, analyzed at 7 d. Dentin treated with both zinc-NPs and calcium-NPs attained the highest reduction of dentinal fluid flow. Moreover, when treating dentin with zinc-NPs, complex modulus values attained at intertubular and peritubular dentin were higher than those obtained after applying calcium-NPs. Zinc-NPs are then supposed to fasten active dentin remodeling, with increased maturity and high mechanical properties. Zinc-based nanoparticles are then proposed for effective dentin remineralization and tubular occlusion. Further research to finally prove for clinical benefits in patients with dentin hypersensitivity using Zn-doped nanoparticles is encouraged. Erosion from acids provokes dentin hypersensitivity (DH) which presents with intense pain of short duration. Open dentinal tubules and demineralization favor DH. Nanogels based on Ca-nanoparticles and Zn-nanoparticles produced an efficient reduction of fluid flow. Dentinal tubules were filled by precipitation of induced calcium-phosphate deposits. When treating dentin with Zn-nanoparticles, complex modulus

  10. Pacman dysplasia: a lethal skeletal dysplasia with variable radiographic features

    Energy Technology Data Exchange (ETDEWEB)

    Miller, S.F. [Dept. of Radiology, Children' s Hospital of the King' s Daughters, Norfolk (United States); Proud, V.K. [Dept. of Genetics, Children' s Hospital of the King' s Daughters, Norfolk (United States); Werner, A.L. [Dept. of Pathology, Children' s Hospital of the King' s Daughters, Norfolk (United States); Field, F.M.; Wilcox, W.F.; Lachman, R.S.; Rimoin, D.L. [International Skeletal Dysplasia Registry, Cedars-Sinai Medical Center, Los Angeles (United States)

    2003-04-01

    Background: Punctate or stippled cartilaginous calcifications are associated with many conditions, including chromosomal, infectious, endocrine, and teratogenic etiologies. Some of these conditions are clinically mild, while others are lethal. Accurate diagnosis can prove instrumental in clinical management and in genetic counseling. Objective: To describe the diagnostic radiographic features seen in Pacman dysplasia, a distinct autosomal recessive, lethal skeletal dysplasia. Materials and methods: We present the fourth reported case of Pacman dysplasia and compare the findings seen in our patient with the three previously described patients. Results: Invariable and variable radiographic findings were seen in all four cases of histologically proven Pacman dysplasia. Conclusion: Pacman dysplasia presents both constant and variable diagnostic radiographic features. (orig.)

  11. Two-Stage Progressive Femoral Lowering Followed by Cementless Total Hip Arthroplasty for Treating Crowe IV-Hartofilakidis Type 3 Developmental Dysplasia of the Hip.

    Science.gov (United States)

    Binazzi, Roberto

    2015-05-01

    High developmental dysplasia of the hip is commonly treated with total hip arthroplasty and shortening osteotomy. We present a two stage technique, consisting of progressive femoral lowering followed by total hip arthroplasty. The clinico-radiographic results of eleven patients (twelve hips) who were operated on with the two-stage technique were evaluated at a mean follow-up of 11 ± 5 years. At the final follow-up, ten patients (eleven hips) had a mean Harris hip score of 85 ± 5 points with no implant loosening. One patient (one hip) was revised at 5 years due to infection. No neurovascular complications were observed in any patients. With this technique, we could place the cup in the anatomical position and obtain complete limb symmetry with excellent clinical results at long-term. Copyright © 2014 Elsevier Inc. All rights reserved.

  12. Developmental coxa vara associated with spondylometaphyseal dysplasia (DCV/SMD): ''SMD-Corner Fracture Type'' (DCV/SMD-CF) demonstrated in most reported cases

    International Nuclear Information System (INIS)

    Currarino, G.; Birch, J.G.; Herring, J.A.

    2000-01-01

    Background. This paper reports three children with short stature: developmental coxa vara unilateral in the first case and bilateral in the other two; somewhat squared and ''ovoid'' vertebral bodies in the first patient, and normal to slightly tall vertebral bodies in the third; metaphyseal changes in some long tubular bones including bone fragments similar to the corner fractures seen in child abuse in all three patients. Materials and methods. The first and second patients were sisters; their mother, also quite short, had surgical procedures in early life for bilateral ''coxa vara''; their brother, also of short stature, had bilateral coxa valga with otherwise normal femoral heads and necks, and mild metaphyseal changes associated with two minute ''corner fractures'' in the proximal metaphysis of the left tibia. Results. A review of reported cases of developmental coxa vara associated with spondylometaphyseal dysplasia revealed that simulated corner fractures were present in most instances. (orig.)

  13. Ictal Generalized EEG Attenuation (IGEA and hypopnea in a child with occipital type 1 cortical dysplasia - Is it a biomarker for SUDEP?

    Directory of Open Access Journals (Sweden)

    Ganne Chaitanya

    2015-01-01

    Full Text Available An interesting association of ictal hypopnea and ictal generalized EEG attenuation (IGEA as possible marker of sudden unexpected death in epilepsy (SUDEP is reported. We describe a 5-years-old girl with left focal seizures with secondary generalization due to right occipital cortical dysplasia presenting with ictal hypopnea and IGEA. She had repeated episodes of the ictal apnoea in the past requiring ventilator support and intensive care unit (ICU admission during episodes of status epilepticus. The IGEA lasted for 0.26-4.68 seconds coinciding with the ictal hypopnea during which both clinical seizure and electrical epileptic activity stopped. Review of literature showed correlation between post-ictal apnoea and post ictal generalized EEG suppression and increased risk for SUDEP. The report adds to the growing body of literature on peri-ictal apnea, about its association with IGEA might be considered as a marker for SUDEP. She is seizure free for 4 months following surgery.

  14. Fetal MR imaging of Kniest dysplasia

    International Nuclear Information System (INIS)

    Yazici, Zeynep; Kline-Fath, Beth M.; Laor, Tal; Tinkle, Bradley T.

    2010-01-01

    We present a case of Kniest dysplasia, a rare form of the type II collagenopathies, with prenatal MRI. Sonography revealed only short limbs in the fetus. Fetal MRI findings included enlarged hyaline cartilaginous structures with abnormally high T2 signal intensity, delayed ossification of the pubic and ischial bones, and platyspondyly. By delineating the cartilaginous abnormalities, fetal MRI can contribute to the prenatal diagnosis of chondrodysplasias. (orig.)

  15. Fetal MR imaging of Kniest dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Yazici, Zeynep [Uludag University, Faculty of Medicine, Department of Radiology, Gorukle (Turkey); Kline-Fath, Beth M.; Laor, Tal [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Tinkle, Bradley T. [Cincinnati Children' s Hospital Medical Center, Division of Human Genetics, Cincinnati, OH (United States)

    2010-03-15

    We present a case of Kniest dysplasia, a rare form of the type II collagenopathies, with prenatal MRI. Sonography revealed only short limbs in the fetus. Fetal MRI findings included enlarged hyaline cartilaginous structures with abnormally high T2 signal intensity, delayed ossification of the pubic and ischial bones, and platyspondyly. By delineating the cartilaginous abnormalities, fetal MRI can contribute to the prenatal diagnosis of chondrodysplasias. (orig.)

  16. The effect of trochlear dysplasia on patellofemoral biomechanics: a cadaveric study with simulated trochlear deformities.

    Science.gov (United States)

    Van Haver, Annemieke; De Roo, Karel; De Beule, Matthieu; Labey, Luc; De Baets, Patrick; Dejour, David; Claessens, Tom; Verdonk, Peter

    2015-06-01

    Trochlear dysplasia appears in different geometrical variations. The Dejour classification is widely used to grade the severity of trochlear dysplasia and to decide on treatment. To investigate the effect of trochlear dysplasia on patellofemoral biomechanics and to determine if different types of trochlear dysplasia have different effects on patellofemoral biomechanics. Controlled laboratory study. Trochlear dysplasia was simulated in 4 cadaveric knees by replacing the native cadaveric trochlea with different types of custom-made trochlear implants, manufactured with 3-dimensional printing. For each knee, 5 trochlear implants were designed: 1 implant simulated the native trochlea (control condition), and 4 implants simulated 4 types of trochlear dysplasia. The knees were subjected to 3 biomechanical tests: a squat simulation, an open chain extension simulation, and a patellar stability test. The patellofemoral kinematics, contact area, contact pressure, and stability were compared between the control condition (replica implants) and the trochlear dysplastic condition and among the subgroups of trochlear dysplasia. The patellofemoral joint in the trochlear dysplastic group showed increased internal rotation, lateral tilt, and lateral translation; increased contact pressures; decreased contact areas; and decreased stability when compared with the control group. Within the trochlear dysplastic group, the implants graded as Dejour type D showed the largest deviations for the kinematical parameters, and the implants graded as Dejour types B and D showed the largest deviations for the patellofemoral contact areas and pressures. Patellofemoral kinematics, contact area, contact pressure, and stability are significantly affected by trochlear dysplasia. Of all types of trochlear dysplasia, the models characterized with a pronounced trochlear bump showed the largest deviations in patellofemoral biomechanics. Investigating the relationship between the shape of the trochlea and

  17. Fibrous dysplasia of bone

    International Nuclear Information System (INIS)

    Kim, Kyung Soo; Lee, Sang Wook; Cho, Young Jun; Kim, Young Sook

    1983-01-01

    Fibrous dysplasia of bone is a skeletal development anomaly of unknown etiology characterized by single or multiple areas of fibrous tissue replacement of medullary cavity of one or more bones. The disease may be localized to single bone (monostotic form) or may affect multiple bones (polyostotic form). Eighteen cases of fibrous dysplasia diagnosed by roentgenlogic or histologic assessment at Chosun University Hospital, Chosun University Hospital and Kwangju Christian Hospital during recent ten tears were analyzed clinically and radiologically. The results were as follows: 1. 16 case of them had monostotic involvement, and 2 cases showed polyostotic disease, but none of our series presented Albright's syndrome. 2. The male to female ratio in this series was 10 : 8, but then 2 polyostotic forms of them were females. In age distribution, peak incidence at the time of diagnosis was in the age group of second decade (10 cases). 3. Maxilla (6 cases) and femur (4 case) were frequently involved sites in patients with monostotic lesion, whereas polyostotic lesions diffusely affected skull, pelvis, ribs and limb bones. 4. The clinical symptoms according to the extent and site of disease were very variable, which were localized painless or painful swelling, nasal obstruction, deformity of face or extremity and incidentally during routine roentgen study. 5. The chemical abnormality of blood serum was moderate degree of elevated serum alkaline phosphatase in only one patients with monostotic lesion. 6. The main radiologic findings of fibrous dysplasia were relatively well circumscribed single or multiloculated cystilike appearance, bone expansion, cortical thinning and/or erosion, bony deformity and pathologic fracture, but especially in maxilla, dense homogenous area with expanding lesion was observed in our series

  18. Evaluation of inactivation of intracanal antiseptics by dentin, demineralized dentin, dentin matrix and mineral component of dentin

    Directory of Open Access Journals (Sweden)

    Razmi H

    2007-01-01

    Full Text Available Background and Aim: Many studies have shown that microorganisms are the main cause of pulpal diseases and the main purpose of root canal therapy is their elimination from the root canal system. Antiseptic agents are used to reduce bacteria but their antibacterial activities differ from in vivo to in vitro studies and might be inactivated by dentin and its components in root canal space. This study was designed to investigate the effect of dentin on antibacterial activity of different antimicrobial agents. Materials and Methods: In this experimental study, two antibacterial agents (sodium hypochlorite and chlorhexidine with different concentrations were used in four experimental groups: Group 1: dentin, Group 2: demineralized dentin with EDTA, Group 3: dentin matrix and Group 4: dentin mineral component. The species used in this study was Entrococcus faecalis. Different concentration of agents were added to mixture of each experimental group and bacteria. At the baseline and after one and 24 hours, samples were collected and cultured. After incubation period, colonies were counted. Data were analyzed by Tukey test with p<0.05 as the limit of significance. Results: 2% and 0.2% chlorhexidine, and 5% sodium hypochlorite solutions at the three studied times eliminated Entrococcus faecalis completely. 1% sodium hypochlorite eliminated all bacteria in 1h and 24 hs. Statistical analysis showed significant differences between experimental and control groups (P<0.05. Sodium 1% hypochlorite at time 0, could reduce bacteria significantly (P<0.05 but didn’t eliminate them completely. Conclusion: Inactivation of intracanal antiseptics was not observed in this study. As elimination of bacteria occurred, application of these antibacterial agents are recommended in endodontic treatment. Further investigations on other antibacterial agents, other concentrations and shorter time intervals are recommended.

  19. Femtosecond laser ablation of dentin

    International Nuclear Information System (INIS)

    Alves, S; Vilar, R; Oliveira, V

    2012-01-01

    The surface morphology, structure and composition of human dentin treated with a femtosecond infrared laser (pulse duration 500 fs, wavelength 1030 nm, fluences ranging from 1 to 3 J cm -2 ) was studied by scanning electron microscopy, x-ray diffraction, x-ray photoelectron spectroscopy and Fourier transform infrared spectroscopy. The average dentin ablation threshold under these conditions was 0.6 ± 0.2 J cm -2 and the ablation rate achieved in the range 1 to 2 µm/pulse for an average fluence of 3 J cm -2 . The ablation surfaces present an irregular and rugged appearance, with no significant traces of melting, deformation, cracking or carbonization. The smear layer was entirely removed by the laser treatment. For fluences only slightly higher than the ablation threshold the morphology of the laser-treated surfaces was very similar to the dentin fracture surfaces and the dentinal tubules remained open. For higher fluences, the surface was more porous and the dentin structure was partially concealed by ablation debris and a few resolidified droplets. Independently on the laser processing parameters and laser processing method used no sub-superficial cracking was observed. The dentin constitution and chemical composition was not significantly modified by the laser treatment in the processing parameter range used. In particular, the organic matter is not preferentially removed from the surface and no traces of high temperature phosphates, such as the β-tricalcium phosphate, were observed. The achieved results are compatible with an electrostatic ablation mechanism. In conclusion, the high beam quality and short pulse duration of the ultrafast laser used should allow the accurate preparation of cavities, with negligible damage of the underlying material. (paper)

  20. Bizarre cell dysplasia of the cervix.

    Science.gov (United States)

    Ondič, Ondrej; Ferko, Radoslav; Kašpírková, Jana; Švajdler, Marián; Rýchly, Boris; Talarčík, Peter; Bouda, Jiří; Michal, Michal

    2017-02-01

    The aim of this study was the characterization of a new subtype of high-grade cervical squamous intraepithelial lesion (HSIL) with enlarged cells containing bizarre nuclei: so-called bizarre cell dysplasia (BCD). A total of 29 cervical cone biopsy samples of this type of dysplasia were studied. Multi-target polymerase chain reaction and in situ hybridization human papillomavirus (HPV) detection was performed in all cases. BCD was defined as a subtype of HSIL characterized by the presence of large dysplastic cells with abnormal, large pleomorphic nuclei or multinucleation causing nucleomegaly. This results in bizarre nuclear shapes. Bizarre cells are scattered throughout the whole thickness of the dysplastic squamous epithelium. The BCD lesions arise within the conventional/classic high grade or "bland" type squamous dysplasia HSIL. Statistically they were significantly associated with HVP type 16. A significant association with other studied viruses (Herpes simplex virus [HSV]1, HSV2, Varicella zoster virus, Epstein-Barr virus, cytomegalovirus, human herpesvirus 6, and human polyomaviruses BK and JC) was not confirmed. BCD involves cytologically characteristic morphologic changes that are recognizable, but which may pose some risk of misdiagnosis as low-grade squamous intraepithelial lesion due to the enlargement of dysplastic cells and multinucleation. Based on the unique histological, cytological and biological features of BCD including strong association with HPV 16 infection, we believe that this is a specific, and so far unrecognized variant of HSIL. © 2017 Japan Society of Obstetrics and Gynecology.

  1. Buffering action of human dentin in vitro.

    Science.gov (United States)

    Camps, J; Pashley, D H

    2000-01-01

    The purpose of this study was to determine the relative contributions of the mineral and organic phases of dentin to its total buffering capacity and to compare the buffering abilities of normal and caries-affected dentin for acids used in adhesive dentistry. Disks of normal and caries-affected human coronal dentin 0.6 mm thick were prepared. Fifty microL of various acids were applied to the surface of mineralized or completely demineralized dentin for varying lengths of time. They were collected from the surface and combined with water rinses to permit titration of the total amount of acid applied, the amount recovered, the total amount that was taken up by the dentin, and the amount that diffused across dentin into 1 mL of water. Equal volumes of acids were applied to mineralized or demineralized dentin powder or hydroxyapatite powder. About 88% to 90% of applied acid was recovered from the surface; only 10% to 12% of the acid was taken up by dentin. Of the H+ that was taken up, only 1% to 2% actually diffused across 0.6 mm of dentin. Increasing the application time of 37% phosphoric acid did not increase the amount of H+ that diffused across dentin. Increasing the concentration of phosphoric acid from 10% to 65% produced only slight increases in H+ diffusion across dentin. There was no difference in the buffering capacity of normal vs caries-affected dentin disks. Almost all of the buffering capacity of dentin is due to its mineral phase. The high buffering capacity of dentin and the high reactivity of H+ insure that little H+ diffuses through dentin more than 0.6 mm thick.

  2. Central ossifying fibroma, periapical cemento-osseous dysplasia and complex odontoma occurring in the same jaw

    Directory of Open Access Journals (Sweden)

    Farzaneh Agha Hosseini

    2011-05-01

    Full Text Available Central ossifying fibroma is a rare, benign fibro-osseous lesion that arises from the periodontal ligament. Periapical cemento-osseous dysplasia is another variant of fibro-osseous lesion which occurs in the anterior region of the mandible of females. Odontoma is a benign odontogenic tumor that contains enamel, dentine cement and pulp tissue. A 46-year-old woman was referred to the Department of Oral Medicine, School of Dentistry, Tehran University of Medical Sciences, with two nonpainful swellings on both sides of the mandible, which had been slowly growing over a period of one year. Our differential diagnosis was florid cemento-osseous dysplasia, focal cemento-osseous dysplasia for the right side, complex odontoma for the left side and periapical cement-osseous dysplasia for the anterior side. The historical feature revealed ossifying fibroma, complex odontoma and periapical cemento-osseous dysplasia. The occurrence of these three lesions in the same jaw has been rarely reported in the literature. The relationship between the occurrence of these three lesions is not obvious it could be coincidental. It seems that more case reports are needed to establish the relationship between them.

  3. Effect of desensitizing toothpastes on dentin

    Directory of Open Access Journals (Sweden)

    Shelon Cristina Souza Pinto

    2012-10-01

    Full Text Available The objective of this study was to analyze the effects of toothbrushing with desensitizing toothpastes on dentin permeability and dentinal tubule occlusion. Fifty rats provided two hundred incisor teeth divided into five groups: DW, brushed with distilled water (control; FT, brushed with fluoride toothpaste; SCT, brushed with strontium chloride toothpaste; PCT, brushed with potassium citrate toothpaste; and PNT, brushed with potassium nitrate toothpaste. Cavities were prepared to expose the dentinal tubules, and the incisor teeth were brushed using the experimental agents. After each treatment, Evans blue dye solution was applied to the teeth. Dentin permeability was analyzed using scanning electron microscopy and energy-dispersive X-rays (EDX. There were significant differences (p < 0.0001, ANOVA among the groups regarding dentin permeability, number of dentinal tubules, diameter of dentinal tubules, and opened tubular area. In the SCT, PCT and PNT groups, opened and partially occluded tubules, deposits, and a few smear layers were observed. In the DW and FT groups, most of the dentinal tubules were open, with no deposits or smear layers on the dentin. EDX revealed peaks of calcium and phosphorus in all of the groups, as well as traces of strontium in the SCT group and of potassium in the PCT and PNT groups. Desensitizing toothpaste decreased dentin permeability, although it produced only partial dentin tubule occlusion.

  4. Cyclophilin B Deficiency Causes Abnormal Dentin Collagen Matrix.

    Science.gov (United States)

    Terajima, Masahiko; Taga, Yuki; Cabral, Wayne A; Nagasawa, Masako; Sumida, Noriko; Hattori, Shunji; Marini, Joan C; Yamauchi, Mitsuo

    2017-08-04

    Cyclophilin B (CypB) is an endoplasmic reticulum-resident protein that regulates collagen folding, and also contributes to prolyl 3-hydroxylation (P3H) and lysine (Lys) hydroxylation of collagen. In this study, we characterized dentin type I collagen in CypB null (KO) mice, a model of recessive osteogenesis imperfecta type IX, and compared to those of wild-type (WT) and heterozygous (Het) mice. Mass spectrometric analysis demonstrated that the extent of P3H in KO collagen was significantly diminished compared to WT/Het. Lys hydroxylation in KO was significantly diminished at the helical cross-linking sites, α1/α2(I) Lys-87 and α1(I) Lys-930, leading to a significant increase in the under-hydroxylated cross-links and a decrease in fully hydroxylated cross-links. The extent of glycosylation of hydroxylysine residues was, except α1(I) Lys-87, generally higher in KO than WT/Het. Some of these molecular phenotypes were distinct from other KO tissues reported previously, indicating the dentin-specific control mechanism through CypB. Histological analysis revealed that the width of predentin was greater and irregular, and collagen fibrils were sparse and significantly smaller in KO than WT/Het. These results indicate a critical role of CypB in dentin matrix formation, suggesting a possible association between recessive osteogenesis imperfecta and dentin defects that have not been clinically detected.

  5. Displasia broncopulmonar Bronchopulmonary dysplasia

    Directory of Open Access Journals (Sweden)

    Luciana F. Velloso Monte

    2005-04-01

    Full Text Available OBJETIVO: Apresentar uma ampla revisão da literatura sobre displasia broncopulmonar, abordando novas definições, fisiopatologia, prevenção, tratamento, prognóstico e evolução. FONTE DOS DADOS: Foram selecionados os artigos mais relevantes sobre o tema, desde a sua descrição inicial, em 1967, pesquisados na MEDLINE. SÍNTESE DOS DADOS: A displasia broncopulmonar é considerada uma das principais causas de doença pulmonar crônica em lactentes. Está associada a hospitalizações freqüentes e prolongadas, especialmente por doenças pulmonares, altos índices de mortalidade e alterações no desenvolvimento neuropsicomotor e no crescimento pôndero-estatural. A patogênese é complexa e influenciada principalmente por prematuridade, infecção, oxigênio suplementar e ventilação mecânica. A prevenção envolve o acompanhamento pré-natal adequado, a prevenção do parto prematuro, o uso pré-natal do corticosteróide, a terapia de reposição de surfactante e o uso de estratégias ventilatórias "protetoras". O tratamento do paciente com displasia broncopulmonar demanda uma equipe multidisciplinar. Quando indicada, a suplementação de oxigênio é de extrema importância. Apesar de maior risco de morbimortalidade nos primeiros anos de vida, a evolução em longo prazo é favorável na maioria das vezes. CONCLUSÕES: A displasia broncopulmonar vem sendo profundamente estudada na tentativa de identificação das suas causas e possibilidades de prevenção e de tratamento. Ainda existem controvérsias quanto a esses assuntos e também em relação ao prognóstico desses pacientes, especialmente quando se trata da evolução tardia da "nova" displasia broncopulmonar.OBJECTIVE: To present a wide-ranging review of the literature on bronchopulmonary dysplasia, covering new definitions, pathophysiology, prevention, treatment, prognosis and progression. SOURCES OF DATA: The most relevant articles published on the subject since it was first

  6. Fibrous dysplasia and cherubism

    Directory of Open Access Journals (Sweden)

    Surajit Bhattacharya

    2015-01-01

    Full Text Available Fibrous dysplasia (FD is a non-malignant fibro-osseous bony lesion in which the involved bone/bones gradually get converted into expanding cystic and fibrous tissue. The underlying defect in FD is post-natal mutation of GNAS1 gene, which leads to the proliferation and activation of undifferentiated mesenchymal cells arresting the bone development in woven phase and ultimately converting them into fibro-osseous cystic tissue. Cherubism is a hereditary form of fibrous dysplasia in which the causative factor is transmission of autosomal dominant SH3BP2 gene mutation. The disease may present in two distinct forms, a less severe and limited monostotic form, and a more aggressive and more widespread polyostotic form. Polyostotic form may be associated with various endocrine abnormalities, which require active management apart from the management of FD. Management of FD is not free from controversies. While total surgical excision of the involved area and reconstruction using newer micro-vascular technique is the only definitive treatment available from the curative point of view, but this can be only offered to monostotic and very few polyostotic lesions. In polyostotic varieties on many occasions these radical surgeries are very deforming in these slow growing lesions and so their indication is highly debated. The treatment of cranio-facial fibrous dysplasia should be highly individualized, depending on the fact that the clinical behavior of lesion is variable at various ages and in individual patients. A more conservative approach in the form of aesthetic recontouring of deformed bone, orthodontic occlusal correction, and watchful expectancy may be the more accepted form of treatment in young patients. Newer generation real-time imaging guidance during recontouring surgery adds to accuracy and safety of these procedures. Regular clinical and radiological follow up is required to watch for quiescence, regression or reactivation of the disease process

  7. Type VII Collagen is Enriched in the Enamel Organic Matrix Associated with the Dentin-Enamel Junction of Mature Human Teeth

    OpenAIRE

    McGuire, Jacob D.; Walker, Mary P.; Mousa, Ahmad; Wang, Yong; Gorski, Jeff P.

    2014-01-01

    The inner enamel region of erupted teeth is known to exhibit higher fracture toughness and crack growth resistance than bulk phase enamel. However, an explanation for this behavior has been hampered by the lack of compositional information for the residual enamel organic matrix. Since enamel-forming ameloblasts are known to express type VII collagen and type VII collagen null mice display abnormal amelogenesis, the aim of this study was to determine whether type VII collagen is a component of...

  8. Evaluation of Ectodermal Dysplasia

    Directory of Open Access Journals (Sweden)

    Zelal Baskan

    2006-04-01

    Full Text Available This case series report outlines possible cranio-maxillofacial deformation consequences associated with ectodermal dysplasia (ED and embryonic malformations, including dental agenesis. Also described are the oral aspects and rehabilitation. A total of 14 ED patients (7 males and 7 females, aged 5-45 years underwent clinical examination before assessment and treatment. Lateral cephalometric radiography, Steiner's analysis, and respiratory capacity tests were performed. Most of the patients had sparse or absent hair, a short face with an unusual facial concavity, a maxillary retrusion, and a relative mandible protrusion. Depending on age and orthopedic abnormalities, patients were treated with prosthodontic and orthodontic approaches or implant treatment. Therapists should take a comprehensive and multidisciplinary approach with these patients to improve their dental, masticatory, growth, and orthognathic conditions, as well as esthetic appearance.

  9. Arrhythmogenic right ventricular dysplasia

    International Nuclear Information System (INIS)

    Vignolo Puglia, W.; Freire Colla, D.; Rivara Urrutia, D.; Lujambio Grene, M.; Arbiza Bruno, T.; Oliveira, G.; Cobas Rodriguez, J.

    1997-01-01

    The arrhythmogenic right ventricular dysplasia is a condition predominantly well defined with arrhythmic events. We analyze three cases diagnosed by the group. These cases were presented as ventricular tachycardia with a morphology of left bundle branch block, presenting one of them aborted sudden death in evolution. The baseline electrocardiogram and signal averaging were abnormal in two of the three cases, like the echocardiogram. The electrophysiological study was able to induce in the three patients with sustained monomorphic ventricular tachycardia morphology of left bundle branch block. The definitive diagnosis was made by right ventriculography in two cases and magnetic resonance imaging in the other. Treatment included antiarrhythmic drugs in the three cases and the placement of an automatic defibrillator which survived a sudden death (Author)

  10. Polyostotic Fibrous Dysplasia of Cranio-Maxillofacial Area

    Energy Technology Data Exchange (ETDEWEB)

    Han, Jin Woo; Kwon, Hyuk Rok; Lee, Jin Ho; Park, In Woo [Dept. of Oral and Maxillofacial Radiology, College of Dentistry, Kangnung National University, Kangnung (Korea, Republic of)

    2000-06-15

    Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

  11. Polyostotic Fibrous Dysplasia of Cranio-Maxillofacial Area

    International Nuclear Information System (INIS)

    Han, Jin Woo; Kwon, Hyuk Rok; Lee, Jin Ho; Park, In Woo

    2000-01-01

    Fibrous dysplasia is believed to be a hamartomatous developmental lesion of unknown origin. This disease is divided into monostotic and polyostotic fibrous dysplasia. Polyostotic type can be divided into craniofacial type, Lichtenstein-Jaffe type, and McCune-Albright syndrome. In this case, a 31-year-old female presented spontaneous loss of right mandibular teeth before 5 years and has shown continuous expansion of right mandibular alveolus. Through the radiographic view, the coarse pattern of the mixed radiopaque-lucent lesion was seen on the right mandibular body, and there was diffuse pattern of the mixed radiopaque-lucent lesion with ill-defined margin in the left mandibular body. In the right calvarium, the lesion had cotton-wool appearance. Partial excision for contouring, multiple extraction, and alveoloplasty were accomplished under general anesthesia for supportive treatment. Finally we could conclude this case was polyostotic fibrous dysplasia of cranio-maxillofacial area based on the clinical, radiologic finding, and histopathologic examination.

  12. The scapula as a window to the diagnosis of skeletal dysplasias

    International Nuclear Information System (INIS)

    Mortier, G.R.; Rimoin, D.L.; Lachman, R.S.

    1997-01-01

    Evaluation of the scapula can be useful in the diagnosis of skeletal dysplasias and helpful for the classification and delineation of new entities. A review of 2100 computerized cases of skeletal dysplasias in the International Skeletal Dysplasia Registry was performed. We found that the Luton type of platyspondylic lethal skeletal dysplasia differed radiographically from the San Diego type and Torrance type by the presence of two spikes at the inferior angle of the scapula. Hypoplasia of the body of the scapula, which is characteristic for campomelic dysplasia but not for kyphomelic dysplasia, is also present in Antley-Bixler syndrome. Radiographic and clinical similarities between campomelic dysplasia and Antley-Bixler syndrome suggest that they might be related disorders and that the latter condition should be included in the bent-bone dysplasia group. Similarity between the metaphyseal regions of the scapula and the metaphyses of the long tubular bones in the different types of short-rib polydactyly syndrome illustrates the importance of evaluation of the scapula in this group as well as in other well-defined or unknown osteochondrodysplasias. (orig.). With 8 figs

  13. Oral epithelial dysplasia classification systems

    DEFF Research Database (Denmark)

    Warnakulasuriya, S; Reibel, J; Bouquot, J

    2008-01-01

    At a workshop coordinated by the WHO Collaborating Centre for Oral Cancer and Precancer in the United Kingdom issues related to potentially malignant disorders of the oral cavity were discussed by an expert group. The consensus views of the Working Group are presented in a series of papers....... In this report, we review the oral epithelial dysplasia classification systems. The three classification schemes [oral epithelial dysplasia scoring system, squamous intraepithelial neoplasia and Ljubljana classification] were presented and the Working Group recommended epithelial dysplasia grading for routine...... use. Although most oral pathologists possibly recognize and accept the criteria for grading epithelial dysplasia, firstly based on architectural features and then of cytology, there is great variability in their interpretation of the presence, degree and significance of the individual criteria...

  14. Septooptic dysplasia : a case report

    International Nuclear Information System (INIS)

    Kim, Jung Hae; Choi, Dae Seob; Lee, Chang Wook; Kim, Soon; Kim, Seung Hyeon; Lee, Sung Woo; Ha, Jung Ho; Sakong, Jung Kyu; Lee, Hyeon Kyeong

    2001-01-01

    Septooptic dysplasia is a rare anterior midline anomaly considered to be a mild form of lobar holoprosencephaly. We describe a case with unilateral optic nerve hypoplasia and the absence of a septum pellucidum

  15. Genetics Home Reference: frontonasal dysplasia

    Science.gov (United States)

    ... respective protein. As a result, the regulation of cell organization during development of the head and face is ... Craniofacial Association: A Guide to Understanding Frontonasal Dysplasia (PDF) Disease InfoSearch: ... Organization for Rare Disorders (NORD) Operation Smile Resource List ...

  16. Fibromuscular dysplasia of renal arteries

    International Nuclear Information System (INIS)

    Akhtar, N.; Ahmed, T.M.

    2007-01-01

    This case reports a young child having uncontrolled hypertension, resulting from bilateral renal artery stenosis due to fibromuscular dysplasia presenting with abdominal pain, headache and visual disturbance. Diagnostic features and management is discussed. (author)

  17. Dyssegmental dysplasia in siblings: Prenatal ultrasonic diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Andersen, P.E. Jr.; Hauge, M.; Bang, J.

    1988-01-01

    Two cases of dyssegmental dysplasia (type Silverman-Handmaker) in siblings are presented. The first-born died at the age of 3 months and the second fetus was followed during pregnancy with ultrasound examinations. In the 20th week of gestation marked shortening of the extremities was found; a female infant showing the same radiologic bony malformations as the firstborn was born by cesarean section. These cases support the autosomal recessive inheritance and demonstrate the possibility of prenatal diagnosis in this type of micromelic dwarfism. (orig.)

  18. [Cochleovestibular dysplasia: a case report].

    Science.gov (United States)

    Darbi, A; Cenoui, F; Atmane, A; Amil, T; Hanine, A

    2010-04-01

    Cochleovestibular dysplasia or Mondini malformation is exceptional. Cochlear dysplasia is due to early cessation of the development of the inner ear during embryonic life. We report on the case of an infant who presented with perception deafness and repeated meningitis. CT allowed the diagnosis of Mondini malformation. We underline the importance of high-resolution CT in the diagnosis of Mondini malformation. Copyright 2010 Elsevier Masson SAS. All rights reserved.

  19. Intraoperative Magnetic-Resonance Tomography and Neuronavigation During Resection of Focal Cortical Dysplasia Type II in Adult Epilepsy Surgery Offers Better Seizure Outcomes.

    Science.gov (United States)

    Roessler, Karl; Kasper, Burkhard S; Heynold, Elisabeth; Coras, Roland; Sommer, Björn; Rampp, Stefan; Hamer, Hajo M; Blümcke, Ingmar; Buchfelder, Michael

    2018-01-01

    Focal cortical dysplasia (FCD) is one important cause of drug-resistant epilepsy potentially curable by epilepsy surgery. We investigated the options of using neuronavigation and intraoperative magnetic-resonance tomographical imaging (MRI) to avoid residual epileptogenic tissue during resection of patients with FCD II to improve seizure outcome. Altogether, 24 patients with FCD II diagnosed by MRI (16 female, 8 male; mean age 34 ± 10 years) suffered from drug-resistant electroclinical and focal epilepsy for a mean of 20.7 ± 5 years. Surgery was performed with preoperative stereoelectroencephalography (in 15 patients), neuronavigation, and intraoperative 1.5T-iopMRI in all 24 investigated patients. In 75% of patients (18/24), a complete resection was performed. In 89% (16/18) of completely resected patients, we documented an Engel I seizure outcome after a mean follow-up of 42 months. All incompletely resected patients had a worse outcome (Engel II-III, P < 0.0002). Patients with FCD IIB had also significant better seizure outcome compared with patients diagnosed as having FCD IIA (82% vs. 28%, P < 0.02). In 46% (11/24) of patients, intraoperative second-look surgeries due to residual lesions detected during the intraoperative MRI were performed. In these 11 patients, there were significant more completely seizure free patients (73% vs. 38% Engel IA), compared with 13 patients who finished surgery after the first intraoperative MRI (P < 0.05). Excellent seizure outcome after surgery of patients with FCD II positively correlated with the amount of resection, histologic subtype, and the use of intraoperative MRI, especially when intraoperative second-look surgeries were performed. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. Progressive pseudorheumatoid dysplasia

    International Nuclear Information System (INIS)

    Mampaey, S.; De Schepper, A.; Vanhoenacker, F.; Boven, K.; Hul, W. van

    2000-01-01

    A rare case of progressive pseudorheumatoid dysplasia (PPD) in a 9-year-old girl is presented. Clinically, chronic painless swollen joints, accompanied by progressive motion restriction and progressive walking difficulties, were found. Radiologically, there was enlargement of the epimetaphyseal portions of the large joints, metacarpal heads, and phalanges, and generalized platyspondyly with irregular delineation of the endplates of the vertebral bodies. The radioclinical features at the peripheral joints were originally misdiagnosed as juvenile rheumatoid arthritis (JRA), and the structural spinal abnormalities were neglected and interpreted as Scheuermann's disease. However, the absence of active inflammatory parameters argues against JRA, whereas the low age of onset of the irregularities at the vertebral endplates is an argument against the diagnosis of Scheuermann's disease. The combination of the dysplastic abnormalities of the spine, with platyspondyly and Scheuermann-like lesions at an unusually low age of onset, and radiological features mimicking JRA of the peripheral joints, is the clue to the diagnosis of this rare autosomal-recessive disease. This case is the first to document the MRI features of PPD of the spine. (orig.)

  1. Ureaplasma and bronchopulmonary dysplasia.

    Science.gov (United States)

    Gancia, Paolo; Delogu, Antonio; Pomero, Giulia

    2014-03-01

    Advances in neonatal intensive care have greatly improved survival rates for children born in a very early stage of lung development (i.e. less than 26 weeks of gestation). In these premature babies, even low levels of oxygen and methods of minimally invasive ventilation may disrupt the growth of the distal airways, a condition described as "new" bronchopulmonary dysplasia (BPD). Ureaplasma infection can occur in utero or in the perinatal period in premature infants, in some of which the infection with these organisms triggers an important lung pro-inflammatory and pro-fibrotic response, and may increase the risk of developing BPD. The inflammation may be worsened by exposure to oxygen and mechanical ventilation. At present, clinical studies have not clarified the role of Ureaplasma in the pathogenesis of BPD and there is insufficient evidence to determine whether antibiotic treatment of Ureaplasma has influence on the development of BPD and its comorbidities. Future research in the context of well-designed and controlled clinical trials of adequate statistical power should focus on how to determine whether the treatment of Ureaplasma decreases lung inflammation, reduces rates of BPD, and improves long-term neurodevelopment. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  2. Recurring fibrous dysplasia of anthro maxillary with cranial base invasion

    Directory of Open Access Journals (Sweden)

    Sousa, Kátia Maria Marabuco de

    2009-09-01

    Full Text Available Introduction: Fibrous dysplasia is an osseous lesion with an unknown etiology. It is characterized by the osseous maturation insufficiency. It may affect any bone, but the affection of craniofacial bones is the most critical for otorhinolaryngology. Maxilla is the most affected facial bone and the orbitary invasion is an uncommon event. The symptoms are unspecific and for its low suspicion and uncommonness, the diagnosis is generally late. The monostotic form presents a slow growth and asymptomatic course and needs to be followed up. The polyostotic type has a progressive behavior and is associated to recurrence and complications. Objective: To present two cases of patients with fibrous dysplasia diagnosis and describe the clinical presentation, radiological findings and the treatment of this pathology. Cases Report: Two cases of fibrous dysplasia are reported, which initially presented unspecific symptomatology, but with characteristic radiologic signs. They were submitted to surgical treatment for resection of the lesions and evolved with frequent recurrences with extensive affection of the facial sinuses, one patient had cranial base invasion and frontal craniotomy was needed for tumoral excision. Final Comments: Fibrous dysplasia is an uncommon osteopathy. The tomography is the choice method for characterization of the tumoral expansion, and helps in the surgical planning. The surgical strategy is indicated for symptomatic lesions, functions alterations or anatomic disorders. This article describes two uncommon manifestations of recurrent fibrous dysplasia with an extensive affection of anthro maxillary, ethmoidal and sphenoid sinuses, in addition to orbitary and cranial base invasion.

  3. Craniofacial fibrous dysplasia - A review of current management techniques

    Directory of Open Access Journals (Sweden)

    Yadavalli Guruprasad

    2012-01-01

    Full Text Available Fibrous dysplasia is a pathologic condition of bone of unknown etiology with no apparent familial, hereditary or congenital basis. Lichtenstein first coined the term in 1938 and in 1942 he and Jaffe separated it from other fibro-osseous lesions. It is a bone tumor that, although benign, has the potential to cause significant cosmetic and functional disturbance, particularly in the craniofacial skeleton. Its management poses significant challenges to the surgeon. Craniofacial fibrous dysplasia is 1 of 3 types of fibrous dysplasia that can affect the bones of the craniofacial complex, including the mandible and maxilla. Fibrous dysplasia is a skeletal developmental disorder of the bone-forming mesenchyme that manifests as a defect in osteoblastic differentiation and maturation. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Fibrous dysplasia represents about 2, 5% of all bone tumors and over 7% of all benign tumours. Over the years, we have gained a better understanding of its etiology, clinical behavior, and both surgical and non-surgical treatments.

  4. Spondylometaphyseal dysplasia with hypercalcemia. [Radiological studies

    Energy Technology Data Exchange (ETDEWEB)

    Bagga, A.; Srivastava, R.N.; Gupta, S.; Gupta, A.

    1989-08-01

    Kozlowski's spondylometaphyseal dysplasia is characterised by short-trunk dwarfism, platyspondyly, metaphyseal dysplasia and retarded bone age. We report an association of this syndrome with asymptomatic, hypocalciuric hypercalcemia, a previously undocumented finding. (orig.).

  5. Ectodermal dysplasia: a genetic review.

    Science.gov (United States)

    Deshmukh, Seema; Prashanth, S

    2012-09-01

    Ectodermal dysplasia is a rare hereditary disorder with a characteristic physiognomy. It is a genetic disorder affecting the development or function of the teeth, hair, nails and sweat glands. Depending on the particular syndrome ectodermal dysplasia can also affect the skin, the lens or retina of the eye, parts of the inner ear, the development of fingers and toes, the nerves and other parts of the body. Each syndrome usually involves a different combination of symptoms, which can range from mild to severe. The history and lessons learned from hypohidrotic ectodermal dysplasia (HED) may serve as an example for unraveling of the cause and pathogenesis of other ectodermal dysplasia syndromes by demonstrating that phenotypically identical syndromes can be caused by mutations in different genes (EDA, EDAR, EDARADD), that mutations in the same gene can lead to different phenotypes and that mutations in the genes further downstream in the same signaling pathway (NEMO) may modify the phenotype quite profoundly. The aim of this paper is to describe and discuss the etiology, genetic review, clinical manifestations and treatment options of this hereditary disorder. How to cite this article: Deshmukh S, Prashanth S. Ectodermal Dysplasia: A Genetic Review. Int J Clin Pediatr Dent 2012; 5(3):197-202.

  6. Autosomal recessive anhidrotic ectodermal dysplasia: A rare entity

    Directory of Open Access Journals (Sweden)

    Sangita Ghosh

    2014-01-01

    Full Text Available We describe a case of anhidrotic ectodermal dysplasia (AED with an autosomal recessive mode of inheritance, a very rare entity, in a 2-year-old female child of two asymptomatic, consanguineous parents. Their previous child also had a similar condition. Autosomal recessive AED (AR-AED can have its full expression both in males and females and it is clinically indistinguishable from the x-linked recessive AED (XL-AED, which is the most common type of ectodermal dysplasia. Unlike the partially symptomatic carriers of XL-AED, the heterozygotes of AR-AED are phenotypically asymptomatic.

  7. Radiographic signs of acetabular dysplasia of the adult hip

    Energy Technology Data Exchange (ETDEWEB)

    Lingg, G.; von Torklus, D.

    1981-06-01

    Many papers have been published about the different types of measurements used in the evaluation of hip dysplasia in the pediatric age group. However, there are no publications that deal with this topic regarding adults. The authors present an initial series of measurements of the angle of the roof of the acetabulum in 100 randomized asymptomatic adult males. The normal value ranged from -10/sup 0/ to +10/sup 0/. These measurements were correlated with those described by Wiberg. The combination of both methods yields highly reliable information in the evaluation of hip dysplasia.

  8. Microshear bonding effectiveness of different dentin regions

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    Anelise F Montagner

    2015-01-01

    Full Text Available Aim: The aim of this in vitro study was to evaluate the influence of dentin surfaces with different tubule orientation on bond strength of a multimode adhesive system. Materials and Methods: Twenty human molars were selected and prepared in different ways in order to produce different dentin regions. The teeth were randomized (n = 5 according to the application modes of a multimode adhesive system (etch-and-rinse and self-etching and to the dentin region (occlusal and proximal - 1.5 mm depth from dentinoenamel junction. Cylindrical restorations were performed on dentin with a starch tube. The samples remained immersed in distilled water for 24 h and the microshear bond strength (μSBS test was performed. The μSBS values were expressed in MPa and analyzed with ANOVA and Tukey′s test (P < 0.05. Results: There was no significant difference in the bond strength values between the application modes of the adhesive system (P = 0.19; however, the dentin regions (P < 0.05 significantly affected the μSBS. The proximal dentin presented higher bond strength values than occlusal dentin. Conclusion: The dentin surfaces with different tubule orientation influenced the bonding effectiveness of the adhesive system tested.

  9. Unicystic ameloblastoma with the presence of dentin

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    Sivapathasundharam B

    2007-01-01

    Full Text Available We present a case of unicystic ameloblastoma of the posterior mandible in a 28 year-old female, histologically showing luminal and intramural plexiform epithelial proliferation with typical dentin in the connective tissue capsule. The characteristics of hard tissue formation in ameloblastomas reported in existing literature and the possible origin of the dentin mass seen in our case are discussed.

  10. Raman spectra of human dentin mineral

    NARCIS (Netherlands)

    Tsuda, H; Ruben, J; Arends, J

    Human dentin mineral has been investigated by using micro-Raman spectroscopy. Fluorescence and thermal problems were largely avoided by preparing dentin samples by grinding and ultrasonic agitation in acetone. The Raman spectral features were consistent with those of impure hydroxyapatite containing

  11. Effect of etching on bonding of a self-etch adhesive to dentine affected by amelogenesis imperfecta.

    Science.gov (United States)

    Epasinghe, Don Jeevanie; Yiu, Cynthia Kar Yung

    2018-02-01

    Dentine affected by amelogenesis imperfecta (AI) is histologically altered due to loss of hypoplastic enamel and becomes hypermineralized. In the present study, we examined the effect of additional acid etching on microtensile bond strength of a self-etch adhesive to AI-affected dentine. Flat coronal dentine obtained from extracted AI-affected and non-carious permanent molars were allocated to two groups: (a) Clearfil SE Bond (control); and (b) Clearfil SE Bond and additional etching with 34% phosphoric acid for 15 seconds. The bonded teeth were sectioned into .8-mm 2 beams for microtensile bond strength testing, and stressed to failure under tension. The bond strength data were analyzed using two-way analysis of variance (dentine type and etching step) and Student-Newman-Keuls multiple comparison test (P<.05). Representative fractured beams from each group were examined under scanning electron microscopy. Both factors, dentine substrate (P<.001) and etching step (P<.05), and their interactions (P<.001), were statistically significant. Additional etching had an adverse effect on the bond strength of Clearfil SE Bond to normal dentine (P<.005), and no significant improvement was found for AI-affected dentine (P=.479). Additional acid etching does not improve the bond strength of a self-etch adhesive to AI-affected dentine. © 2017 John Wiley & Sons Australia, Ltd.

  12. Chondroectodermal dysplasia: a rare syndrome.

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    Dana Tahririan

    2014-06-01

    Full Text Available Chondroectodermal dysplasia (Ellis-Van Creveld syndrome is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. Clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities.

  13. Aspects of dentinal and pulpal pain. Pain of dentinal and pulpal origin--a review for the clinician.

    Science.gov (United States)

    Figdor, D

    1994-04-01

    Recent advances in understanding the mechanisms of pain arising from the dental pulp serve to benefit patients by improving the clinician's ability to diagnose and treat pain. There are two types of pain arising from the pulp which are mediated by entirely different nerve fibres, each with their own individual characteristics. One is a short, sharp fast pain which is induced by stimuli which cause a rapid fluid flow within the dentinal tubules. Such stimuli include cold, heat, air, drilling, and osmotic stimuli. Once the affected teeth are identified, they can often be treated by sealing the open, exposed dentine. The second type of pain is experienced as a slow, dull, aching, poorly localized pain which is mediated by pain fibres activated by stimuli which are noxious to the pulp, such as prolonged damaging heat and inflammatory mediators. Pain of this character can be difficult to diagnose and often indicates serious pulp damage necessitating removal of the offending pulp by endodontic therapy.

  14. Thanatophoric Dysplasia: A Rare Entity

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    N.S. Naveen

    2011-05-01

    Full Text Available Thanatophoric dysplasia is the lethal skeletal dysplasia characterized by marked underdevelopment of the skeleton and short-limb dwarfism. The child will be having a short neck, narrow thoracic cage and protuberant abdomen. Other anatomical features include a relatively enlarged head with frontal bossing, prominent eyes, hypertelorism and the depressed nasal bridge. The diagnosis is usually made with the ultrasonography in the second trimester. In this study we report a case of this rare entity with emphasis on its anatomical features, abnormalities and clinical profile with relevant review of literature.

  15. Managing Children with Bronchopulmonary Dysplasia

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    A. A. Baranov

    2016-01-01

    Full Text Available Bronchopulmonary dysplasia is one of the most significant early childhood chronic respiratory diseases. The article features modern approaches to preventing, diagnosing and treating broncho-pulmonary dysplasia, as well as ways of preventing complications and undesirable disease outcomes in patients older than 3 years. Members of professional associations — Union of Pediatricians of Russia and Russian Association of Perinatal Medicine Specialists — have summarized the experience of managing this category of patients at leading Russian pediatric centers according to the principles of evidence-based medicine and have provided scientific and practical data corresponding to the world level of knowledge with regard to the present problem.

  16. Central ossifying fibroma, periapical cemento-osseous dysplasia and complex odon-toma occurring in the same jaw.

    Science.gov (United States)

    Hosseini, Farzaneh Agha; Moslemi, Elham

    2011-05-16

    Central ossifying fibroma is a rare, benign fibro-osseous lesion that arises from the periodontal ligament. Periapical cemento-osseous dysplasia is another variant of fibro-osseous lesion which occurs in the anterior region of the mandible of females. Odontoma is a benign odontogenic tumor that contains enamel, dentine cement and pulp tissue. A 46-year-old woman was referred to the Department of Oral Medicine, School of Dentistry, Tehran University of Medical Sciences, with two non-painful swellings on both sides of the mandible, which had been slowly growing over a period of one year. Our differential diagnosis was florid cemento-osseous dysplasia, focal cemento-osseous dysplasia for the right side, complex odontoma for the left side and periapical cement-osseous dysplasia for the anterior side. The historical feature revealed ossifying fibroma, complex odontoma and periapical cemento-osseous dysplasia. The occurrence of these three lesions in the same jaw has been rarely reported in the literature. The relationship between the occurrence of these three lesions is not obvious it could be coincidental. It seems that more case reports are needed to establish the relationship between them.

  17. Effect of photoactivated riboflavin on the biodegradation-resistance of root-dentin collagen.

    Science.gov (United States)

    Priyadarshini, Balasankar Meera; Lu, Thong Beng; Fawzy, Amr S

    2017-12-01

    This study was conducted to evaluate the effect of UVA-activated 1% riboflavin solution on structural integrity; mechanical properties and stability; and collagenase-mediated collagen solubilisation resistance of demineralized root dentin collagen matrix. Root dentin specimens demineralized with 17% EDTA for 7days were treated with 1% RF for 1min followed by UVA photo-activation at intensity 7mW/cm 2 for 1min. Control specimens were completely devoid of riboflavin and/or UVA treatments. Specimens were challenged with bacterial collagenase type-I solution for different time-periods at 37°C. Collagen solubilisation resistance was evaluated in terms of hydroxyproline (HYP) liberation. Mechanical characterization of dentin specimens before and after 24h of exposure to collagenase solution was done in terms of apparent-elastic modulus (E appr ) and ultimate tensile strength (UTS). Variations in dentin collagen-network structure with exposure time in collagenase were visualized by TEM. Crosslinking dentin with UVA-activated riboflavin significantly decreased HYP release and increased E appr and UTS compared to control specimens with storage time in collagenase. Moreover, crosslinked specimens showed higher structural resistance to collagenase effect reflected from dense, well-formed collagen fibrils-network with characteristic collagen cross-banding. UVA-activated riboflavin treatment increased collagenase-mediated collagen degradation resistance and enhanced mechanical stability against collagenase challenges of root dentin after EDTA demineralization. Copyright © 2017 Elsevier B.V. All rights reserved.

  18. Dentin-bonding agents

    Directory of Open Access Journals (Sweden)

    João Carlos Gomes

    2008-01-01

    Full Text Available New dental restorative materials have been developed to meet not only the functional demands, but esthetics as well, and in the last few years an enormous range of new materials has appeared for use in dentistry. Among them, several adhesive systems, and different operative techniques for each group materials. Therefore, is indispensable for the professional to know about the properties, characteristics, and association of these materials with the dental structures, in order to select and use them correctly. Should conventional self-etching adhesive systems be used? This question encouraged this literature review to be conducted, with the aim of comparing the conventional adhesive systems with the self-etching systems and to look for scientific data that would help professionals to choose which adhesive system to use. When compared to conventional systems, it was noted that the self-etching systems show less sensitivity to technique, especially as regards errors the operator could commit. The self-etching systems, particularly the 2-step type, have shown equivalent values of bond strength, marginal microleakage and performance, therefore, will be an option for direct composite resin restorations in posterior teeth.

  19. Pulpal progenitors and dentin repair.

    Science.gov (United States)

    Harichane, Y; Hirata, A; Dimitrova-Nakov, S; Granja, I; Goldberg, A; Kellermann, O; Poliard, A

    2011-07-01

    Mesenchymal stem cells are present in the dental pulp. They have been shown to contribute to dentin-like tissue formation in vitro and to participate in bone repair after a mandibular lesion. However, their capacity to contribute efficiently to reparative dentin formation after pulp lesion has never been explored. After pulp exposure, we have identified proliferative cells within 3 zones. In the crown, zone I is near the cavity, and zone II corresponds to the isthmus between the mesial and central pulp. In the root, zone III, near the apex, at a distance from the inflammatory site, contains mitotic stromal cells which may represent a source of progenitor cells. Stem-cell-based strategies are promising treatments for tissue injury in dentistry. Our experiments focused on (1) location of stem cells induced to leave their quiescent state early after pulp injury and (2) implantation of pulp progenitors, a substitute for classic endodontic treatments, paving the way for pulp stem-cell-based therapies.

  20. Infra-red and Raman spectroscopic studies of infected and affected dentine

    International Nuclear Information System (INIS)

    Aminzadeh, A.; Aminzadeh, A.; Khosravy, K.

    2002-01-01

    Diagnosis of infected and affected dentine is an important factor in clinical restorative treatment of dentine. In this study, the IR and Raman spectra of the sound dentine, infected dentine and affected dentine are reported. The structure of infected dentine and affected dentine has been compared with the sound dentine and hydroxyapatite. It is shown while the infected dentine has lost its structure, the affected dentine has more or less a structure similar to the sound dentine. The molecular structure of collagen remains unchanged in both infected and affected dentine

  1. The use of acetone to enhance the infiltration of HA nanoparticles into a demineralized dentin collagen matrix.

    Science.gov (United States)

    Besinis, Alexandros; van Noort, Richard; Martin, Nicolas

    2016-03-01

    This study investigates the role of acetone, as a carrier for nano-hydroxyapatite (nano-HA) in solution, to enhance the infiltration of fully demineralized dentin with HA nanoparticles (NPs). Dentin specimens were fully demineralized and subsequently infiltrated with two types of water-based nano-HA solutions (one containing acetone and one without). Characterization of the dentin surfaces and nano-HA particles was performed using scanning electron microscopy (SEM) and transmission electron microscopy (TEM). The surface wettability and infiltration capacity of the nano-HA solutions were quantified by means of contact angle measurements and energy dispersive X-ray spectroscopy (EDS), respectively. Contact angle measurements were taken at baseline and repeated at regular intervals to assess the effect of acetone. The P and Ca levels of infiltrated dentin specimens were measured and compared to sound dentin and non-infiltrated controls. The presence of acetone resulted in an eight-fold decrease in the contact angles of the nano-HA solutions recorded on the surface of demineralized dentin compared to nano-HA solutions without acetone (one-way ANOVA, pacetone. Infiltration of demineralized dentin with the nano-HA solution containing acetone restored the lost mineral content by 50%, whereas the mean mineralization values for P and Ca in dentin treated with the acetone-free nano-HA solution were less than 6%. Acetone was shown to act as a vehicle to enhance the capacity to infiltrate demineralized dentin with HA NPs. The successful infiltration of dentin collagen with HA NPs provides a suitable scaffold, whereby the infiltrated HA NPs have the potential to act as seeds that may initiate heterogenous mineral growth when exposed to an appropriate mineral-rich environment. Copyright © 2015 Academy of Dental Materials. Published by Elsevier Ltd. All rights reserved.

  2. Dentomaxillofacial characteristics of ectodermal dysplasia.

    Science.gov (United States)

    Nakayama, Yumiko; Baba, Yoshiyuki; Tsuji, Michiko; Fukuoka, Hiroki; Ogawa, Takuya; Ohkuma, Mizue; Moriyama, Keiji

    2015-02-01

    The aim of this retrospective hospital-based study was to elucidate the dentomaxillofacial characteristics of ectodermal dysplasia. Six Japanese individuals (one male and five female; age range, 12.7-27.2 years) underwent comprehensive examinations, including history recording, cephalometric analysis, panoramic radiography, and analysis of dental models. All the subjects had two or more major manifestations for clinical diagnosis of ectodermal dysplasia (e.g., defects of hair, teeth, nails, and sweat glands). They presented hypodontia (mean number of missing teeth, 9.5; range, 5-14), especially in the premolar region, and enamel dysplasia. Five subjects had bilateral molar occlusion, whereas one subject had unilateral molar occlusion. The common skeletal features were small facial height, maxillary hypoplasia, counterclockwise rotation of the mandible, and mandibular protrusion. Interestingly, the maxillary first molars were located in higher positions and the upper anterior facial height was smaller than the Japanese norm. The results suggest that vertical and anteroposterior maxillary growth retardation, rather than lack of occlusal support due to hypodontia, leads to reduced anterior facial height in individuals with ectodermal dysplasia. © 2014 Japanese Teratology Society.

  3. Cranioectodermal Dysplasia : A Probable Ciliopathy

    NARCIS (Netherlands)

    Konstantinidou, Anastasia E.; Fryssira, Helen; Sifakis, Stavros; Karadimas, Charalampos; Kaminopetros, Petros; Agrogiannis, Georgios; Velonis, Stylianos; Nikkels, Peter G. J.; Patsouris, Efstratios

    2009-01-01

    Cranioectodermal dysplasia (CED), also known as Sensenbrenner syndrome, is a rare autosomal recessive genetic disorder characterized by typical craniofacial, skeletal and ectodermal defects, and tubulointerstitial nephritis leading to early end-stage renal failure. We report on a new familial case

  4. Treatment of hip dysplasia in patients with mucopolysaccharidosis type I after hematopoietic stem cell transplantation: results of an international consensus procedure

    NARCIS (Netherlands)

    Langereis, Eveline J.; Borgo, Andrea; Crushell, Ellen; Harmatz, Paul R.; van Hasselt, Peter M.; Jones, Simon A.; Kelly, Paula M.; Lampe, Christina; van der Lee, Johanna H.; Odent, Thierry; Sakkers, Ralph; Scarpa, Maurizio; Schafroth, Matthias U.; Struijs, Peter A.; Valayannopoulos, Vassili; White, Klane K.; Wijburg, Frits A.

    2013-01-01

    Mucopolysaccharidosis type I (MPS-I) is a lysosomal storage disorder characterized by progressive multi-organ disease. The standard of care for patients with the severe phenotype (Hurler syndrome, MPS I-H) is early hematopoietic stem cell transplantation (HSCT). However, skeletal disease, including

  5. Congenital heart defects in oculodentodigital dysplasia: Report of two cases.

    Science.gov (United States)

    Izumi, Kosuke; Lippa, Andrew M; Wilkens, Alisha; Feret, Holly A; McDonald-McGinn, Donna M; Zackai, Elaine H

    2013-12-01

    Oculodentodigital dysplasia is caused by mutations in the GJA1 gene. Oculodentodigital dysplasia presents with a spectrum of clinical features including craniofacial, ocular, dental, and limb anomalies. Although recent findings implicate the major role of GJA1 during cardiac organogenesis, congenital heart defects are infrequently reported in oculodentodigital dysplasia. Here we report on two patients with GJA1 mutations presenting with cardiac malformations and type III syndactyly. Patient 1 presented with pulmonary atresia, an intact septum, right ventricular hypoplasia and tricuspid stenosis. The infant had a small nose, thin columella and bilateral 4-5 syndactyly of the fingers. A de novo c.226C>T (p.Arg76Cys) mutation was identified. Patient 2 presented at 6 months with a ventricular septal defect. The child had hypoplastic alae nasi with a thin columella and bilateral 4-5 syndactyly of the digits. A de novo missense mutation, c.145C>G (p.Gln49Glu) was found. Our two patients underscore the importance of cardiac evaluations as part of the initial workup for patients with findings of oculodentodigital dysplasia. Conversely, those patients with type III syndactyly and congenital heart defect should be screened for GJA1 mutations. © 2013 Wiley Periodicals, Inc.

  6. Longevity of Self-etch Dentin Bonding Adhesives Compared to Etch-and-rinse Dentin Bonding Adhesives: A Systematic Review.

    Science.gov (United States)

    Masarwa, Nader; Mohamed, Ahmed; Abou-Rabii, Iyad; Abu Zaghlan, Rawan; Steier, Liviu

    2016-06-01

    A systematic review and meta-analysis were performed to compare longevity of Self-Etch Dentin Bonding Adhesives to Etch-and-Rinse Dentin Bonding Adhesives. The following databases were searched for PubMed, MEDLINE, Web of Science, CINAHL, the Cochrane Library complemented by a manual search of the Journal of Adhesive Dentistry. The MESH keywords used were: "etch and rinse," "total etch," "self-etch," "dentin bonding agent," "bond durability," and "bond degradation." Included were in-vitro experimental studies performed on human dental tissues of sound tooth structure origin. The examined Self-Etch Bonds were of two subtypes; Two Steps and One Step Self-Etch Bonds, while Etch-and-Rinse Bonds were of two subtypes; Two Steps and Three Steps. The included studies measured micro tensile bond strength (μTBs) to evaluate bond strength and possible longevity of both types of dental adhesives at different times. The selected studies depended on water storage as the aging technique. Statistical analysis was performed for outcome measurements compared at 24 h, 3 months, 6 months and 12 months of water storage. After 24 hours (p-value = 0.051), 3 months (p-value = 0.756), 6 months (p-value=0.267), 12 months (p-value=0.785) of water storage self-etch adhesives showed lower μTBs when compared to the etch-and-rinse adhesives, but the comparisons were statistically insignificant. In this study, longevity of Dentin Bonds was related to the measured μTBs. Although Etch-and-Rinse bonds showed higher values at all times, the meta-analysis found no difference in longevity of the two types of bonds at the examined aging times. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome).

    Science.gov (United States)

    Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

    2014-01-01

    Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

  8. Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome)

    OpenAIRE

    Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

    2014-01-01

    Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

  9. Maxillo-nasal dysplasia (Binder syndrome) and associated malformations of the cervical spine

    Energy Technology Data Exchange (ETDEWEB)

    Olow-Nordenram, M.A.K.; Raadberg, C.T.

    1984-01-01

    Forty-three patients with maxillo-nasal dysplasia have been subjected to a radiographic examination of the cervical spine. In 44.2 per cent malformations of the cervical vertebrae of a minor or major type were revealed. Dysplasia of the vertebral bodies related to persistence of the chorda dorsalis, a very rare malformation, was found in six cases. No correlation between the incidence or serverity of the malformations and the degree of malocclusion of the jaws and facial deformity, characteristic of Binder syndrome, were noted. The maxillo-nasal dysplasia and the spinal malformations probably have a common cause during the embryologic stage.

  10. Ectodermal dysplasia with alopecia, onychodysplasia, hypohidrosis, keratoderma, abnormal teeth and deafness

    Directory of Open Access Journals (Sweden)

    Akhyani Maryam

    2007-01-01

    Full Text Available The ectodermal dysplasias are a heterogeneous group of disorders with primary defect in hair, teeth, nail and sweat gland function. Numerous types have been described and several classifications exist. Here, we present a patient with ectodermal dysplasia with alopecia, dysplastic nails, hypohidrosis, sensorineural deafness, palmoplantar keratoderma, abnormal teeth and dry skin. To our knowledge, combination of all these features in ectodermal dysplasia has not been reported in the past. The etiology is unknown, but consanguinity of parents points to an autosomal recessive inheritance.

  11. Maxillo-nasal dysplasia (Binder syndrome) and associated malformations of the cervical spine

    International Nuclear Information System (INIS)

    Olow-Nordenram, M.A.K.; Raadberg, C.T.

    1984-01-01

    Forty-three patients with maxillo-nasal dysplasia have been subjected to a radiographic examination of the cervical spine. In 44.2 per cent malformations of the cervical vertebrae of a minor or major type were revealed. Dysplasia of the vertebral bodies related to persistence of the chorda dorsalis, a very rare malformation, was found in six cases. No correlation between the incidence or serverity of the malformations and the degree of malocclusion of the jaws and facial deformity, characteristic of Binder syndrome, were noted. The maxillo-nasal dysplasia and the spinal malformations probably have a common cause during the embryologic stage. (orig.)

  12. Bond strength and microleakage of current dentin adhesives.

    Science.gov (United States)

    Fortin, D; Swift, E J; Denehy, G E; Reinhardt, J W

    1994-07-01

    The purpose of this in vitro study was to evaluate shear bond strengths and microleakage of seven current-generation dentin adhesive systems. Standard box-type Class V cavity preparations were made at the cemento-enamel junction on the buccal surfaces of eighty extracted human molars. These preparations were restored using a microfill composite following application of either All-Bond 2 (Bisco), Clearfil Liner Bond (Kuraray), Gluma 2000 (Miles), Imperva Bond (Shofu), OptiBond (Kerr), Prisma Universal Bond 3 (Caulk), Scotchbond Multi-Purpose (3M), or Scotchbond Dual-Cure (3M) (control). Lingual dentin of these same teeth was exposed and polished to 600-grit. Adhesives were applied and composite was bonded to the dentin using a gelatin capsule technique. Specimens were thermocycled 500 times. Shear bond strengths were determined using a universal testing machine, and microleakage was evaluated using a standard silver nitrate staining technique. Clearfill Liner Bond and OptiBond, adhesive systems that include low-viscosity, low-modulus intermediate resins, had the highest shear bond strengths (13.3 +/- 2.3 MPa and 12.9 +/- 1.5 MPa, respectively). Along with Prisma Universal Bond 3, they also had the least microleakage at dentin margins of Class V restorations. No statistically significant correlation between shear bond strength and microleakage was observed in this study. Adhesive systems that include a low-viscosity intermediate resin produced the high bond strengths and low microleakage. Similarly, two materials with bond strengths in the intermediate range had significantly increased microleakage, and one material with a bond strength in the low end of the spectrum exhibited microleakage that was statistically greater. Thus, despite the lack of statistical correlation, there were observable trends.

  13. Machinability of some dentin simulating materials.

    Science.gov (United States)

    Möllersten, L

    1985-01-01

    Machinability in low speed drilling was investigated for pure aluminium, Frasaco teeth, ivory, plexiglass and human dentin. The investigation was performed in order to find a suitable test material for drilling experiments using paralleling instruments. A material simulating human dentin in terms of cuttability at low drilling speeds was sought. Tests were performed using a specially designed apparatus. Holes to a depth of 2 mm were drilled with a twist drill using a constant feeding force. The time required was registered. The machinability of the materials tested was determined by direct comparison of the drilling times. As regards cuttability, first aluminium and then ivory were found to resemble human dentin most closely. By comparing drilling time variances the homogeneity of the materials tested was estimated. Aluminium, Frasaco teeth and plexiglass demonstrated better homogeneity than ivory and human dentin.

  14. Fracture mechanism of coronal teenage dentin

    Science.gov (United States)

    Panfilov, P. E.; Kabanova, A. V.; Borodin, I. N.; Guo, J.; Zang, Z.

    2017-10-01

    The structure of coronal teenage dentin and the development of cracks in it are studied on microand nanolevels. The material is found to fail according to a ductile mechanism on a microlelvel and according to a ductile-brittle mechanism on a nanoscale. This behavior is similar to the failure of a polyethylene film and rubber, when significant elastic and irreversible deformation precedes crack growth. The viscoelastic behavior can be considered as the reaction of dentin to an applied mechanical load.

  15. Complex orthopaedic management of patients with skeletal dysplasias

    Directory of Open Access Journals (Sweden)

    A. G. Baindurashvili

    2014-01-01

    Full Text Available Skeletal dysplasias are challenging for diagnostics and treatment. We present a series of fifteen patients with different forms of skeletal dysplasias with age ranged from 6 to 17 years with variable clinical presentations managed as a part of the project of scientific cooperation between Turner Paediatric Orthopaedic Institute and Orthopaedic Hospital Vienna-Speising. The spectrum of diagnoses included multiple epiphyseal dysplasia, spondyloepiphyseal dysplasia congenita, diastrophic dysplasia, metaphyseal dysplasia, spondylometaphyseal dysplasia, Stickler syndrome, Kniest dysplasia, and anauxetic dysplasia. Complex treatment, which included axial correction and juxta-articular realignment, was performed as a single-stage, or consecutive surgery. Surgical techniques included corrective osteotomies with internal fixation, guided growth technique and external fixation devices. Best results (full axial correction, normal alignment of the joint were achieved in 8 patients, including 2 patients with metaphyseal dysplasia, 2 patients with multiple epyphyseal dysplasia, 2 patients with spondyloepyphyseal dysplasia, patient with Stickler syndrome and patient with spondylometaphyseal dysplasia. Good results (partial correction at the present time were seen in 4 patients (2 patients with Kniest dysplasia, 1 - with multiple epyphyseal dysplasia and 1 - with anauxetic dysplasia. Satisfactory results (non-progressive condition in previous progression were obtained in 2 patients with diastrophic dysplasia, and poor results (progression of the deformity - in 1 patient with diastrophic dysplasia. Positive results in most of the cases of our series make promising future for usage of complex approach for orthopedic management of children with skeletal dysplasias; advanced international cooperation is productive and helpful for diagnostics and management of rare diseases.

  16. Effect of desensitizing agents on dentin permeability.

    Science.gov (United States)

    Ishihata, Hiroshi; Kanehira, Masafumi; Nagai, Tomoko; Finger, Werner J; Shimauchi, Hidetoshi; Komatsu, Masashi

    2009-06-01

    To investigate the in vitro efficacy of two dentin desensitizing products at reducing liquid permeability through human dentin discs. The tested hypothesis was that the products, in spite of different chemical mechanisms were not different at reducing or eliminating flow through dentin discs. Dentin slices (1 mm thick) were prepared from 16 extracted human third molars and their permeability was indirectly recorded in a split chamber model, using a chemiluminescence technique, after EDTA treatment (control), after soaking with albumin, and after desensitizer application. Two products were studied: MS Coat, a self-curing resin-containing oxalate product, and Gluma Desensitizer, a glutaraldehyde/HEMA-based agent without initiator. The dentin slices were mounted between an upper chamber, filled with an aqueous solution of 1% potassium ferricyanide and 0.3% hydrogen peroxide, and a lower chamber filled with 1% sodium hydroxide solution and 0.02% luminol. The upper solution was pressurized, and upon contact with the luminol solution a photochemical signal was generated and recorded as a measure of permeability throughout two consecutive pressurizing cycles at 2.5 and 13 kPa (26 and 133 cm H2O), respectively. The permeability of the control and albumin-soaked samples was similarly high. After application of the desensitizing agents, dentin permeability was reduced to virtually zero at both pressure levels (P < 0.001).

  17. Craniofacial features of cleidocranial dysplasia

    Directory of Open Access Journals (Sweden)

    Chin-Yun Pan

    2017-12-01

    Full Text Available Cleidocranial dysplasia (CCD is an autosomal-dominant malformation syndrome affecting bones and teeth. The most common skeletal and dental abnormalities in affected individuals are hypoplastic/aplastic clavicles, open fontanelles, short stature, retention of primary teeth, delayed eruption of permanent teeth, supernumerary teeth, and multiple impacted teeth. Treatment of CCD requires a multidisciplinary approach that may include dental corrections, orthognathic surgery and cranioplasty along with management of any complications of CCD. Early diagnosis of this condition enables application of the treatment strategy that provides the best quality of life to such patients. Notably, Runx2 gene mutations have been identified in CCD patients. Therefore, further elucidation of the molecular mechanism of supernumerary teeth formation related to Runx2 mutations may improve understanding of dental development in CCD. The insights into CCD pathogenesis may assist in the development of new treatments for CCD. Keywords: cleidocranial dysplasia, mutation, Runx2, supernumerary teeth

  18. Cleidocranial dysplasia: a case report

    International Nuclear Information System (INIS)

    Kim, Jae Duk; Lee, Chang Yul; You, Choong Hyun

    2004-01-01

    Cleidocranial dysplasia is a rare and autosomal dominant disorder characterized by aplasia or hypoplasia of the clavicles, an open fontanelle, dental abnormalities, and short stature. A 17-year-old female who presented with short stature and subsequent delay in eruption of permanent teeth is described. she showed the abnormal hypermobility of the shoulder, ocular hypertelorism and concave nasal bridge. Radiographs revealed the underdeveloped maxilla, defect of the cranium in the fontanelle region, and aplasia of the clavicles. Characteristically, panoramic view revealed near parallel-sided borders of the ascending ramus and downward curvature of the zygomatic arch with hypoplasia. The prolonged retention of deciduous teeth with delayed eruption of permanent teeth and multiple embedded supernumerary teeth were striking. Radiographic and clinical investigations revealed Cleidocranial dysplasia.

  19. Cleidocranial dysplasia: a case report

    International Nuclear Information System (INIS)

    Han, Jin Won

    2005-01-01

    Cleidocranial dysplasia is a rare, autosomal dominant congenital disorder. A 12-year-old female visited with chief complaint of unerupted permanent teeth. Also her father showed severe class III malocclusion. The extraoral radiography and computed tomography showed delayed closure of the cranial sutures and underdevelopment of maxilla, maxillary sinuses, and frontal sinus. Both clavicles were underdeveloped and thoracic rib cage was bell-shaped. Both zygomatic process appeared as hypoplastic feature. There were many unerupted permanent and supernumerary teeth in the maxilla and mandible. We examined location and number of the unerupted teeth using 3D CT. Finally we could conclude this case was cleidocranial dysplasia based on the clinico-radiologic findings.

  20. [Bronchopulmonary dysplasia: definitions and classifications].

    Science.gov (United States)

    Sánchez Luna, M; Moreno Hernando, J; Botet Mussons, F; Fernández Lorenzo, J R; Herranz Carrillo, G; Rite Gracia, S; Salguero García, E; Echaniz Urcelay, I

    2013-10-01

    Bronchopulmonary dysplasia is the most common sequelae related to very low birth weight infants, mostly with those of extremely low birth weight. Even with advances in prevention and treatment of respiratory distress syndrome associated with prematurity, there is still no decrease in the incidence in this population, although a change in its clinical expression and severity has been observed. There are, however, differences in its frequency between health centres, probably due to a non-homogeneously used clinical definition. In this article, the Committee of Standards of the Spanish Society of Neonatology wishes to review the current diagnosis criteria of bronchopulmonary dysplasia to reduce, as much as possible, these inter-centre differences. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  1. DENTINE CARIES: ACID-TOLERANT MICROORGANISMS AND ASPECTS ON COLLAGEN DEGRADATION.

    Science.gov (United States)

    Lager, Anders Hedenbjörk

    2014-01-01

    Dental caries is a common disease all over the world, despite the fact that it can be both effectively prevented and treated. It is driven by acids produced by oral microorganisms as a consequence of their metabolism of dietary carbohydrates. Given enough acid challenge, eventually the tooth enamel barrier will be broken down, and the carious lesion will extend into underlying hard tissue, forming a macroscopic cavity in the dentine. In comparison to biofilm on enamel, a dentine carious lesion provides a vastly different environment for the residing microorganisms. The environment influences the types and numbers of microorganisms that can colonize the dentine caries lesion. The overall aims for this thesis are to enumerate and further study microorganisms found in established dentine caries lesions and also to illuminate how host-derived proteolytic enzymes might contribute to this degradation, not only to better understand the caries process in dentine but also to find incitements for new methods to influence the natural progression of caries lesions. In Paper I, the numbers of remaining viable microorganisms after completed excavation using two excavation methods were investigated. Samples of carious dentine tissue were collected before and after excavation and cultivated on different agar media in different atmospheres. Analysis was performed by counting the number of colony-forming units (CFUs). Key findings: The number of remaining microorganisms after excavation was low for both methods, but some microorganisms always remained in the cavity floors even when the cavities were judged as caries free using normal clinical criteria. In Paper II, the acid tolerant microbiota in established dentine caries lesions was investigated. Samples were taken as in Paper I, but on three levels (superficial, center of lesion, floor of lesion after completed excavation). The samples were cultivated in anaerobic conditions on solid pH-selective agar media of different acidity

  2. Tensile bond strength of hydroxyethyl methacrylate dentin bonding agent on dentin surface at various drying techniques

    Directory of Open Access Journals (Sweden)

    Kun Ismiyatin

    2010-06-01

    Full Text Available Background: There are several dentin surface drying techniques to provide a perfect resin penetration on dentin. There are two techniques which will be compared in this study. The first technique was by rubbing dentin surface gently using cotton pellet twice, this technique is called blot dry technique. The second technique is by air blowing dentin surface for one second and continued by rubbing dentin surface gently using moist cotton. Purpose: This experiment was aimed to examine the best dentin surface drying techniques after 37% phosphoric acid etching to obtain the optimum tensile bond strength between hydroxyethyl methacrylate (HEMA and dentin surface. Method: Bovine teeth was prepared flat to obtain the dentin surface and than was etched using 37% phosphoric acid for 15 seconds. After etching the dentin was cleaned using 20 cc plain water and dried with blot dry techniques (group I, or dried with air blow for one second (group II, or dried with air blow for one second, and continued with rubbing gently using moist cotton pellet (group III, and without any drying as control group (group IV. After these drying, the dentin surfaces were applied with resin dentin bonding agent and put into plunger facing the composite mould. The antagonist plunger was filled with composite resin. After 24 hours, therefore bond strength was measured using Autograph. Result: Data obtained was analyzed using One-Way ANOVA with 95% confidence level and continued with LSD test on p≤0.05. The result showed that the highest tensile bond strength was on group I, while the lowest on group IV. Group II and IV, III and IV, II and III did not show signigicant difference (p>0.05. Conclusion: Dentin surface drying techniques through gentle rubbing using cotton pellet twice (blot dry technique gave the greatest tensile bond strength.Latar belakang masalah: Tehnik pengeringan permukaan dentin agar resin dapat penetrasi dengan sempurna adalah dengan cara pengusapan secara

  3. Mechanical Ventilation and Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Keszler, Martin; Sant'Anna, Guilherme

    2015-12-01

    Mechanical ventilation is an important potentially modifiable risk factor for the development of bronchopulmonary dysplasia. Effective use of noninvasive respiratory support reduces the risk of lung injury. Lung volume recruitment and avoidance of excessive tidal volume are key elements of lung-protective ventilation strategies. Avoidance of oxidative stress, less invasive methods of surfactant administration, and high-frequency ventilation are also important factors in lung injury prevention. Copyright © 2015 Elsevier Inc. All rights reserved.

  4. Ectodermal dysplasia in identical twins

    OpenAIRE

    Puttaraju, Gurkar Haraswarupa; Visveswariah, Paranjyothi Magadi

    2013-01-01

    Hereditary hypohidrotic ectodermal dysplasia (HED) is typically inherited as an X-linked recessive trait, characterized by deformity of at least two or more of the ectodermal structures - hair, teeth, nails and sweat glands. Two cases of hereditary HED involving identical male twins, is being documented for the rarity of its occurrence with special attention given to genetics, pathophysiology, clinical, intraoral manifestations and to the methods to improve the masticatory function, the facia...

  5. Mondini Dysplasia Presenting as Otorrhea without Meningitis

    OpenAIRE

    Chien-Yu Lin; Hung-Ching Lin; Chun-Chih Peng; Kuo-Sheng Lee; Nan-Chang Chiu

    2012-01-01

    Mondini dysplasia is a rare inner ear malformation that is usually only diagnosed after recurrent meningitis. Surgical intervention is mandatory. This report highlights the case of a patient with Mondini dysplasia who presented with hearing impairment and otorrhea and was diagnosed and treated before the occurrence of meningitis, thus preventing morbidity and neurologic sequelae. Hearing impairment may be the only manifestation of Mondini dysplasia, and the benefit of hearing screening is emp...

  6. Hypohidrotic ectodermal dysplasia with ankylosis of temporomandibular joint and cleft palate: A rare presentation

    Science.gov (United States)

    Goyal, Manisha; Pradhan, Gaurav; Gupta, Sunita; Kapoor, Seema

    2015-01-01

    The ectodermal dysplasias are a heterogenous group of diseases, which have one or more anomalies of the hair, teeth, nails, and sweat glands. Hypohidrotic ectodermal dysplasia (HED) is the most common type and is usually transmitted as an X-linked recessive trait. It is characterized by classical triad of hypotrichosis, anhidrosis/hypohidrosis, and hypodontia/anodontia. Here, we describe an Indian boy affected with HED and rare features including ankylosis of temporomandibular joint and cleft palate. PMID:25684924

  7. Hypohidrotic ectodermal dysplasia with ankylosis of temporomandibular joint and cleft palate: A rare presentation

    Directory of Open Access Journals (Sweden)

    Manisha Goyal

    2015-01-01

    Full Text Available The ectodermal dysplasias are a heterogenous group of diseases, which have one or more anomalies of the hair, teeth, nails, and sweat glands. Hypohidrotic ectodermal dysplasia (HED is the most common type and is usually transmitted as an X-linked recessive trait. It is characterized by classical triad of hypotrichosis, anhidrosis/hypohidrosis, and hypodontia/anodontia. Here, we describe an Indian boy affected with HED and rare features including ankylosis of temporomandibular joint and cleft palate.

  8. Congenital distal humeral dysplasia: a case report

    International Nuclear Information System (INIS)

    Joseph, Benjamin; Varghese, Renjit A.

    2003-01-01

    Congenital dysplasia of the humerus is very rare. It is characteristically seen in omodysplasia and has also been reported as one of the associated features of Larsen's syndrome. We report a 4-year-old girl with bilateral humero-ulnar dysplasia, with dislocation of the elbows, facial dysmorphism, ball-and-socket ankles and foot deformities. Although the elbow dysplasia is similar to that seen in Larsen's syndrome, other pathognomic features of Larsen's syndrome were absent. The changes seen in the elbows in this patient are also different from those encountered in omodysplasia. We believe that this condition may be a distinct form of skeletal dysplasia hitherto undescribed. (orig.)

  9. Lower Lid Ectropion in Hypohidrotic Ectodermal Dysplasia

    Directory of Open Access Journals (Sweden)

    Xiaoyun Zhang

    2015-01-01

    Full Text Available We report a case of a lower lid ectropion with ectodermal dysplasia and ectropion blepharoplasty surgery experience. A 14-year-old Han nationality male patient with typical characteristics of hypohidrotic ectodermal dysplasia presented to our clinic for his right lower lid eversion. The patient was diagnosed as having hypohidrotic ectodermal dysplasia and underwent an uneventful blepharoplasty surgery. The lower lid maintained normal position during the 10-month follow-up period. Patients with ectodermal dysplasia could firstly visit ophthalmologist for their ectropion and blepharoplasty surgery could be useful for the disease.

  10. A case report of the fibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    You, Dong Soo [Department of Radiology, College of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    1975-11-15

    The author observed a rare case of fibrous dysplasia in 12 year old female who came to the Infirmary of Dental College, Seoul National University, complaining of facial asymmetry of 3 years' duration in right maxillofacial region. The serial radiograms has been taken, and the nature of the lesion established as a typical fibrous dysplasia according to the interpreted findings in their images. The author has obtained the results as follows: 1. Fibrous dysplasia occurred at 3 years of age in this case. 2. On familial tendency, traumatic history and endocrine disturbances were not noted in this patient. 3. The serial radiograms revealed a typical fibrous dysplasia encroaching right zygomatic bone.

  11. Pelvic radiograph in skeletal dysplasias: An approach

    Directory of Open Access Journals (Sweden)

    Manisha Jana

    2017-01-01

    Full Text Available The bony pelvis is constituted by the ilium, ischium, pubis, and sacrum. The pelvic radiograph is an important component of the skeletal survey performed in suspected skeletal dysplasia. Most of the common skeletal dysplasias have either minor or major radiological abnormalities; hence, knowledge of the normal radiological appearance of bony pelvis is vital for recognizing the early signs of various skeletal dysplasias. This article discusses many common and some uncommon radiological findings on pelvic radiographs along with the specific dysplasia in which they are seen; common differential diagnostic considerations are also discussed.

  12. A case report of the fibrous dysplasia

    International Nuclear Information System (INIS)

    You, Dong Soo

    1975-01-01

    The author observed a rare case of fibrous dysplasia in 12 year old female who came to the Infirmary of Dental College, Seoul National University, complaining of facial asymmetry of 3 years' duration in right maxillofacial region. The serial radiograms has been taken, and the nature of the lesion established as a typical fibrous dysplasia according to the interpreted findings in their images. The author has obtained the results as follows: 1. Fibrous dysplasia occurred at 3 years of age in this case. 2. On familial tendency, traumatic history and endocrine disturbances were not noted in this patient. 3. The serial radiograms revealed a typical fibrous dysplasia encroaching right zygomatic bone.

  13. Challenges of stem cell-based pulp and dentin regeneration: a clinical perspective.

    Science.gov (United States)

    Huang, George T-J; Al-Habib, Mey; Gauthier, Philippe

    2013-03-01

    There are two types of approaches to regenerate tissues: cell-based and cell-free. The former approach is to introduce exogenous cells into the host to regenerate tissues, and the latter is to use materials other than cells in an attempt to regenerate tissues. There has been a significant advancement in stem cell-based pulp and dentin regeneration research in the past few years. Studies in small and large animals have demonstrated that pulp/dentin-like tissues can be regenerated partially or completely in the root canal space with apical openings of 0.7-3.0 mm using dental pulp stem cells, including stem cells from apical papilla (SCAP) and subpopulations of pulp stem cells. Bone marrow mesenchymal stem cells (BMMSCs) and adipose tissue-derived MSCs (ADMSCs) have also been shown to regenerate pulp-like tissue. In contrast, the cell-free approach has not produced convincing evidence on pulp regeneration. However, one crucial concept has not been considered nor defined in the field of pulp/dentin regeneration and that is the critical size defect of dentin and pulp. Without such consideration and definition, it is difficult to predict or anticipate the extent of cell-free pulp regeneration that would occur. By reasoning, cell-free therapy is unlikely to regenerate an organ/tissue after total loss. Similarly, after a total loss of pulp, it is unlikely to regenerate without using exogenously introduced cells. A cell homing approach may provide a limited amount of tissue regeneration. Although stem cell-based pulp/dentin regeneration has shown great promise, clinical trials are difficult to launch at present. This article will address several issues that challenge and hinder the clinical applications of pulp/dentin regeneration which need to be overcome before stem cell-based pulp/dentin regeneration can occur in the clinic.

  14. Effects of matrix metallproteinases on dentin bonding and strategies to increase durability of dentin adhesion

    Directory of Open Access Journals (Sweden)

    Jung-Hyun Lee

    2012-02-01

    Full Text Available The limited durability of resin-dentin bonds severely compromises the longevity of composite resin restorations. Resin-dentin bond degradation might occur via degradation of water-rich and resin sparse collagen matrices by host-derived matrix metalloproteinases (MMPs. This review article provides overview of current knowledge of the role of MMPs in dentin matrix degradation and four experimental strategies for extending the longevity of resin-dentin bonds. They include: (1 the use of broad-spectrum inhibitors of MMPs, (2 the use of cross-linking agents for silencing the activities of MMPs, (3 ethanol wet-bonding with hydrophobic resin, (4 biomimetic remineralization of water-filled collagen matrix. A combination of these strategies will be able to overcome the limitations in resin-dentin adhesion.

  15. Genetics Home Reference: spondyloepimetaphyseal dysplasia, Strudwick type

    Science.gov (United States)

    ... White L, Hills N, Nicol M, Evans G, Cole T, Chapman S, Pope FM. A glycine to aspartic ... medicine? What is newborn screening? New Pages Lyme disease Fibromyalgia White-Sutton syndrome All New & Updated Pages ...

  16. Temperature rise induced by various light curing units through human dentin.

    Science.gov (United States)

    Dogan, Arife; Hubbezoglu, Ihsan; Dogan, Orhan Murat; Bolayir, Giray; Demir, Hakan

    2009-05-01

    This study investigated temperature rises caused by different light curing units (LCUs) in dentin of different thicknesses. The different LCUs tested in this study were namely: quartz-tungsten-halogen (QTH) (Heliolux DLX) LCU, plasma arc (PAC) (Apollo 95E Elite) LCU, and light emitting diode (LED) (Mini LED) in standard curing mode as well as pulse and soft-start modes. One hundred and forty dentin disks of 0.5, 1, 1.5, and 2 mm thickness were prepared from mandibular molars (n=7). Temperatures were recorded using a L-type thermocouple in direct contact with the light guide tip. For all curing units/modes, dentin thickness was inversely proportional to temperature rise and that QTH light gave significantly higher values compared to PAC and LED in all the test conditions. The highest temperature rise was observed under 0.5-mm-thick dentin disk with QTH, whereas the lowest temperature rise was registered with LED light in pulse mode under 2-mm-thick dentin.

  17. Collagen Orientation and Crystallite Size in Human Dentin: A Small Angle X-ray Scattering Study

    Energy Technology Data Exchange (ETDEWEB)

    Pople, John A

    2001-03-29

    The mechanical properties of dentin are largely determined by the intertubular dentin matrix, which is a complex composite of type I collagen fibers and a carbonate-rich apatite mineral phase. The authors perform a small angle x-ray scattering (SAXS) study on fully mineralized human dentin to quantify this fiber/mineral composite architecture from the nanoscopic through continuum length scales. The SAXS results were consistent with nucleation and growth of the apatite phase within periodic gaps in the collagen fibers. These mineralized fibers were perpendicular to the dentinal tubules and parallel with the mineralization growth front. Within the plane of the mineralization front, the mineralized collagen fibers were isotropic near the pulp, but became mildly anisotropic in the mid-dentin. Analysis of the data also indicated that near the pulp the mineral crystallites were approximately needle-like, and progressed to a more plate-like shape near the dentino-enamel junction. The thickness of these crystallites, {approx} 5 nm, did not vary significantly with position in the tooth. These results were considered within the context of dentinogenesis and maturation.

  18. The dynamics of TGF-β in dental pulp, odontoblasts and dentin.

    Science.gov (United States)

    Niwa, Takahiko; Yamakoshi, Yasuo; Yamazaki, Hajime; Karakida, Takeo; Chiba, Risako; Hu, Jan C-C; Nagano, Takatoshi; Yamamoto, Ryuji; Simmer, James P; Margolis, Henry C; Gomi, Kazuhiro

    2018-03-13

    Transforming growth factor-beta (TGF-β) is critical for cell proliferation and differentiation in dental pulp. Here, we show the dynamic mechanisms of TGF-β in porcine dental pulp, odontoblasts and dentin. The mRNA of latent TGF-β1 and TGF-β3 is predominantly expressed in odontoblasts, whereas the mRNA expression level of latent TGF-β2 is high in dental pulp. TGF-β1 is a major isoform of TGF-β, and latent TGF-β1, synthesized in dental pulp, is primarily activated by matrix metalloproteinase 11 (MMP11). Activated TGF-β1 enhances the mRNA expression levels of MMP20 and full-length dentin sialophosphoprotein (DSPP) in dental pulp cells, coinciding with the induction of odontoblast differentiation. Latent TGF-β1 synthesized in odontoblasts is primarily activated by MMP2 and MMP20 in both odontoblasts and dentin. The activity level of TGF-β1 was reduced in the dentin of MMP20 null mice, although the amount of latent TGF-β1 expression did not change between wild-type and MMP20 null mice. TGF-β1 activity was reduced with the degradation of DSPP-derived proteins that occurs with ageing. We propose that to exert its multiple biological functions, TGF-β1 is involved in a complicated dynamic interaction with matrix metalloproteinases (MMPs) and/or DSPP-derived proteins present in dental pulp, odontoblasts and dentin.

  19. Paleopathological Study of Dwarfism-Related Skeletal Dysplasia in a Late Joseon Dynasty (South Korean Population.

    Directory of Open Access Journals (Sweden)

    Eun Jin Woo

    Full Text Available Skeletal dysplasias related to genetic etiologies have rarely been reported for past populations. This report presents the skeletal characteristics of an individual with dwarfism-related skeletal dysplasia from South Korea. To assess abnormal deformities, morphological features, metric data, and computed tomography scans are analyzed. Differential diagnoses include achondroplasia or hypochondroplasia, chondrodysplasia, multiple epiphyseal dysplasia, thalassemia-related hemolytic anemia, and lysosomal storage disease. The diffused deformities in the upper-limb bones and several coarsened features of the craniofacial bones indicate the most likely diagnosis to have been a certain type of lysosomal storage disease. The skeletal remains of EP-III-4-No.107 from the Eunpyeong site, although incomplete and fragmented, provide important clues to the paleopathological diagnosis of skeletal dysplasias.

  20. Paleopathological Study of Dwarfism-Related Skeletal Dysplasia in a Late Joseon Dynasty (South Korean) Population.

    Science.gov (United States)

    Woo, Eun Jin; Lee, Won-Joon; Hu, Kyung-Seok; Hwang, Jae Joon

    2015-01-01

    Skeletal dysplasias related to genetic etiologies have rarely been reported for past populations. This report presents the skeletal characteristics of an individual with dwarfism-related skeletal dysplasia from South Korea. To assess abnormal deformities, morphological features, metric data, and computed tomography scans are analyzed. Differential diagnoses include achondroplasia or hypochondroplasia, chondrodysplasia, multiple epiphyseal dysplasia, thalassemia-related hemolytic anemia, and lysosomal storage disease. The diffused deformities in the upper-limb bones and several coarsened features of the craniofacial bones indicate the most likely diagnosis to have been a certain type of lysosomal storage disease. The skeletal remains of EP-III-4-No.107 from the Eunpyeong site, although incomplete and fragmented, provide important clues to the paleopathological diagnosis of skeletal dysplasias.

  1. Dentin and pulp sense cold stimulus.

    Science.gov (United States)

    Tokuda, Masayuki; Tatsuyama, Shoko; Fujisawa, Mari; Morimoto-Yamashita, Yoko; Kawakami, Yoshiko; Shibukawa, Yoshiyuki; Torii, Mistuso

    2015-05-01

    Dentin hypersensitivity is a common symptom, and recent convergent evidences have reported transient receptor potential (TRP) channels in odontoblasts act as mechanical and thermal molecular sensor, which detect stimulation applied on the exposed dentin surface, to drive multiple odontoblastic cellular functions, such as sensory transduction and/or dentin formation. In the present study, we confirmed expression of TRP melastatin subfamily member-8 (TRPM8) channels in primary cultured cells derived from human dental pulp cells (HPCs) and mouse odontoblast-lineage cells (OLCs) as well as in dentin matrix protein-1 (DMP-1) and dentin sialoprotein (DSP) positive acutely isolated rat odontoblasts from dental pulp tissue slice culture by immunohistochemical analyses. In addition, we detected TRPM8 channel expression on HPCs and OLCs by RT-PCR and Western blotting analyses. These results indicated that both odontoblasts and dental pulp cells express TRPM8 channels in rat, mouse and human, and therefore we hypothesize they may contribute as cold sensor in tooth. Copyright © 2015 Elsevier Ltd. All rights reserved.

  2. [Mantle dentin as biomodel of materials for structural teeth restoration].

    Science.gov (United States)

    Starodubova, A V; Vinnichenko, Yu A; Pourovskaya, I Ya; Rusanov, F S

    The article describes a structural element of natural teeth - mantle dentin. It has been shown that the presence of this element in the structure of a natural tooth largely ensures its strength under the influence of repeated loads in a functional oral environment and arrests crack growth at the enamel/dentine interface. This later effect is explained by the influence of a thin layer of mantle dentine, which has physical and mechanical characteristics different from that of the main dentin.

  3. Fibrous dysplasia of the femoral neck

    International Nuclear Information System (INIS)

    Savage, P.E.; Stoker, D.J.

    1984-01-01

    Fibrous dysplasia of the femur is usually observed in the intertrochanteric region. It is rarely confined to the femoral neck. We present four cases illustrating the radiographic appearance and spectrum of this condition which all showed the relatively lucent variety of fibrous dysplasia with varying degrees of expansion and surrounding sclerosis. The natural history of this condition is discussed. (orig.)

  4. Bone scintigraphy in polyostotic fibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Wadhwa, S.S.; Mansberg, R.; Fernandes, V.B. [Illawarra Regional Hospital, Wollongong, NSW, (Australia)

    1998-03-01

    Fibrous dysplasia is a benign skeletal disorder of unknown aetiology. Fibrous dysplasia characteristically involves the fibrous replacement of portions of the medullary cavities of a single bone (monostotic) or multiple bones (polyostotic). Bones typically involved include the femurs, tibiae, ribs and maxillae. The polyostotic form may be accompanied by skin pigmentation and endocrine abnormalities (McCune Allbright Syndrome). Radiological findings in fibrous dysplasia are variable, ranging from completely radiolucent to radio-opaque lesions, depending on the amount of fibrous or osseous tissue deposited in the medulla. The most common radiographic finding is that of a ground glass-like semi-opaque lesion. Case reports on scintigraphic manifestation of fibrous dysplasia are scanty. We present radiological and scintigraphic findings of polyostotic fibrous dysplasia in a young male. (authors). 3 refs., 1 fig.

  5. Mondini dysplasia with recurrent meningitis.

    Science.gov (United States)

    Lu, M Y; Lee, P I; Lee, C Y; Hsu, C J

    1996-01-01

    Mondini dysplasia is a congenital malformation of the inner ear, commonly associated with hearing impairment, cerebrospinal fluid otorrhea/rhinorrhea and recurrent meningitis. Two such cases are described, with hearing impairment, cerebrospinal fluid rhinorrhea, and several episodes of meningitis. Diagnosis was confirmed by high-resolution computed tomography. After surgical correction of the malformation, there was no recurrent episode of meningitis at subsequent follow-up. To avoid the suffering and the sequelae of recurrent meningitis, an early diagnosis and prompt surgical intervention are crucial for such patients.

  6. Cochlear implantation in Mondini dysplasia.

    Science.gov (United States)

    Daneshi, Ahmad; Hassanzadeh, Saeid; Abasalipour, Parvaneh; Emamdjomeh, Hessamaddin; Farhadi, Mohammad

    2003-01-01

    The use of cochlear implantation to treat patients with inner ear malformations such as Mondini dysplasia has been increasingly successful. Until now, conventional hearing aids in these patients have not performed well. Consequently, the hearing problem for patients with this condition has been somewhat improved with the use of cochlear implants. Various results of cochlear implantation have been reported in these patients so far. This is a report of 5 patients with Mondini malformation who have undergone cochlear implant surgery. Copyright 2003 S. Karger AG, Basel

  7. Hypoxic Episodes in Bronchopulmonary Dysplasia.

    Science.gov (United States)

    Martin, Richard J; Di Fiore, Juliann M; Walsh, Michele C

    2015-12-01

    Hypoxic episodes are troublesome components of bronchopulmonary dysplasia (BPD) in preterm infants. Immature respiratory control seems to be the major contributor, superimposed on abnormal respiratory function. Relatively short respiratory pauses may precipitate desaturation and bradycardia. This population is predisposed to pulmonary hypertension; it is likely that pulmonary vasoconstriction also plays a role. The natural history has been well-characterized in the preterm population at risk for BPD; however, the consequences are less clear. Proposed associations of intermittent hypoxia include retinopathy of prematurity, sleep disordered breathing, and neurodevelopmental delay. Future study should address whether these associations are causal relationships. Copyright © 2015 Elsevier Inc. All rights reserved.

  8. Cleidocranial dysplasia: A family report

    Directory of Open Access Journals (Sweden)

    Chelvan H

    2009-01-01

    Full Text Available A 10-year-old girl presented with a chief complaint of many unerupted teeth. Complete clinical and radiological examination of this patient confirmed the diagnosis of cleidocranial dysplasia (CCD. Her father also presented similar features with a lesser clinical severity. CCD is an autosomal-dominant heritable skeletal disease caused by heterozygous mutations in the osteoblast-specific transcription factor RUNX2 gene. Failure of tooth eruption is probably mainly due to this mutated gene in CCD patients. Interdisciplinary treatment approach is obligatory for rehabilitation of these patients. In confirmed cases, genetic counseling for family planning should certainly be advised.

  9. Ectodermal dysplasia in identical twins

    Directory of Open Access Journals (Sweden)

    Gurkar Haraswarupa Puttaraju

    2013-01-01

    Full Text Available Hereditary hypohidrotic ectodermal dysplasia (HED is typically inherited as an X-linked recessive trait, characterized by deformity of at least two or more of the ectodermal structures - hair, teeth, nails and sweat glands. Two cases of hereditary HED involving identical male twins, is being documented for the rarity of its occurrence with special attention given to genetics, pathophysiology, clinical, intraoral manifestations and to the methods to improve the masticatory function, the facial esthetics and psychology of patients affected by this disease.

  10. MRI of focal cortical dysplasia

    International Nuclear Information System (INIS)

    Lee, B.C.P.; Hatfield, G.A.; Bourgeois, B.; Park, T.S.

    1998-01-01

    We studied nine cases of focal cortical dysplasia (FCD) by MRI, with surface-rendered 3D reconstructions. One case was also examined using single-voxel proton MR spectroscopy (MRS). The histological features were reviewed and correlated with the MRI findings. The gyri affected by FCD were enlarged and the signal of the cortex was slightly increased on T1-weighted images. The gray-white junction was indistinct. Signal from the subcortical white matter was decreased on T1- and increased on T2-weighted images in most cases. Contrast enhancement was seen in two cases. Proton MRS showed a spectrum identical to that of normal brain. (orig.) (orig.)

  11. Prevention of root caries with dentin adhesives.

    Science.gov (United States)

    Grogono, A L; Mayo, J A

    1994-04-01

    This in vitro investigation determined the feasibility of using dentin adhesives to protect root surfaces against caries. The roots of 22 recently extracted human teeth were all painted with a protective lacquer leaving two unprotected small windows. On each specimen, one window (control) was left untreated and the other window (experimental) was treated using a dentin adhesive (Scotchbond Multi-Purpose). The roots were then immersed in an in vitro acetate/calcium/phosphate demineralization model at pH 4.3. After 70 days, the samples were removed and sectioned through the windows. The undecalcified ground sections were examined under transmitted and polarized light. Lesions characteristic of natural root caries were seen in the untreated control windows. No such lesions were apparent in the experimental windows. The results of this preliminary study suggest that dentin adhesives may provide protection against root caries.

  12. Dentine microhardness after different methods for detection and removal of carious dentine tissue

    Directory of Open Access Journals (Sweden)

    Fernanda Brandão Mollica

    2012-08-01

    Full Text Available There are several methods for identifying carious dentinal tissue aiming to avoid removal of healthy dentinal tissue. OBJECTIVES: The purpose of this study was to test different methods for the detection of carious dentinal tissue regarding the amount of carious tissue removed and the remaining dentin microhardness after caries removal. MATERIAL AND METHODS: The dentin surfaces of 20 bovine teeth were exposed and half of the surface was protected with nail polish. Cariogenic challenge was performed by immersion in a demineralizing solution for 14 days. After transverse cross-section of the crown, the specimens were divided into four groups (n=10, according to the method used to identify and remove the carious tissue: "Papacárie", Caries-detector dye, DIAGNOdent and Tactile method. After caries removal, the cross-sectional surface was included in acrylic resin and polished. In a microhardness tester, the removed dentin thickness and the Vickers microhardness of the following regions were evaluated: remaining dentin after caries removal and superficial and deep healthy dentin. RESULTS: ANOVA and Tukey's test (α=0.05 were performed, except for DIAGNOdent, which did not detect the presence of caries. Results for removed dentin thickness were: "Papacárie" (424.7±105.0; a, Caries-detector dye (370.5±78.3; ab, Tactile method (322.8±51.5; bc. Results for the remaining dentin microhardness were: "Papacárie" (42.2±10.5; bc, Caries-detector dye (44.6±11.8; abc, Tactile method (24.3±9.0; d. CONCLUSIONS: DIAGNOdent did not detect the presence of carious tissue; Tactile method and "Papacárie" resulted in the least and the most dentinal thickness removal, respectively; Tactile method differed significantly from "Papacárie" and Caries-detector dye in terms of the remaining dentin microhardness, and Tactile method was the one which presented the lowest microhardness values.

  13. Effect of dentin bonding agent diffusing through dentin slices on the reactive oxygen species production and apoptosis of pulpal cells

    Directory of Open Access Journals (Sweden)

    Bor-Shiunn Lee

    2015-04-01

    Conclusion: The three DBAs after diffusion through 0.2- or 0.5-mm dentin slice still exhibit cytotoxicity to dental pulp cells. However, the 0.5-mm dentin slice is found to be a better barrier than the 0.2-mm dentin slice to protect dental pulp cells from DBA-induced cytotoxicity.

  14. Shear bond strength of porcelain laminate veneers to enamel, dentine and enamel-dentine complex bonded with different adhesive luting systems.

    Science.gov (United States)

    Öztürk, Elif; Bolay, Şükran; Hickel, Reinhard; Ilie, Nicoleta

    2013-02-01

    The aim of this study was to evaluate the shear bond strength of porcelain laminate veneers to 3 different surfaces by means of enamel, dentine, and enamel-dentine complex. One hundred thirty-five extracted human maxillary central teeth were used, and the teeth were randomly divided into 9 groups (n=15). The teeth were prepared with 3 different levels for bonding surfaces of enamel (E), dentine (D), and enamel-dentine complex (E-D). Porcelain discs (IPS e.max Press, Ivoclar Vivadent) of 2mm in thickness and 4mm in diameter were luted to the tooth surfaces by using 2 light-curing (RelyX Veneer [RV], 3M ESPE; Variolink Veneer [VV], Ivoclar Vivadent) and a dual-curing (Variolink II [V2], Ivoclar Vivadent) adhesive systems according to the manufacturers' instructions. Shear bond strength test was performed in a universal testing machine at 0.5mm/min until bonding failure. Failure modes were determined under a stereomicroscope, and fracture surfaces were evaluated with a scanning electron microscope. The data were statistically analysed (SPSS 17.0) (p=0.05). Group RV-D exhibited the lowest bond strength value (5.42±6.6MPa). There was statistically no difference among RV-D, V2-D (13.78±8.8MPa) and VV-D (13.84±6.2MPa) groups (p>0.05). Group VV-E exhibited the highest bond strength value (24.76±8.8MPa). The type of tooth structure affected the shear bond strength of the porcelain laminate veneers to the 3 different types of tooth structures (enamel, dentine, and enamel-dentine complex). When dentine exposure is necessary during preparation, enough sound enamel must be protected as much as possible to maintain a good bonding; to obtain maximum bond strength, preparation margins should be on sound enamel. Copyright © 2012 Elsevier Ltd. All rights reserved.

  15. Reconstruction of the Acetabulum in Developmental Dysplasia of the Hip in Total Hip Replacement

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    Vasileios Sakellariou

    2014-09-01

    Full Text Available Developmental dysplasia of the hip (DDH or congenital hip dysplasia (CDH is the most prevalent developmental childhood hip disorder. It includes a wide spectrum of hip abnormalities ranging from dysplasia to subluxation and complete dislocation of the hip joint. The natural history of neglected DDH in adults is highly variable. The mean age of onset of symptoms is 34.5 years for dysplastic DDH, 32.5 years for low dislocation, 31.2 years for high dislocation with a false acetabulum, and 46.4 years for high dislocation without a false acetabulum. Thorough understanding of the bony and soft tissue deformities induced by dysplasia is crucial for the success of total hip arthroplasty. It is important to evaluate the existing acetabular deformity three-dimensionally, and customize the correction in accordance with the quantity and location of ace tabular deficiencies. Acetabular reconstruction in patients with DDH is hallenging. Interpretation of published data is difficult and should be done with caution because most series include patients with different types of hip disease. In general, the complication rate associated with THA is higher in patients with hip dysplasia than it is in patients with osteoarthritis. Overall, clinical and functional outcomes following THA in patients hip dysplasia (DDH differ from those treated for primary hip osteoarthritis, possibly due to the lower age and level of activity. Although function scores decline with age, the scores for pain and range of motion presented with a statistically significant improvement in the long-term.

  16. US of the hips in skeletal dysplasias and chromosomal aberrations

    International Nuclear Information System (INIS)

    Langer, R.; Langer, M.F.J.; Zwicker, C.

    1987-01-01

    Since January 1984 all newborns and infants with skeletal dysplasias and chromosomal aberrations were investigated by hip US, in addition to plain x-ray surveys. The authors observed one chondroectodermal dysplasia, one congenital spondyloepiphysial dysplasia, one cleidocranial dysplasia, one fibrochondrogenesis, two diastrophic dysplasias, and eight trisomies. The abnormalities of the hip joints could be demonstrated, and were compared with the findings on plain films. Especially skeletal dysplasias with abundant presence of cartilage were well visible. The newborn with trisomies showed normal hip joints. In the authors' opinion, all newborns with skeletal dysplasias should be investigated by hip sonography, in addition to skeletal radiography

  17. Temperature changes under demineralized dentin during polymerization of three resin-based restorative materials using QTH and LED units.

    Science.gov (United States)

    Mousavinasab, Sayed-Mostafa; Khoroushi, Maryam; Moharreri, Mohammadreza; Atai, Mohammad

    2014-08-01

    Light-curing of resin-based materials (RBMs) increases the pulp chamber temperature, with detrimental effects on the vital pulp. This in vitro study compared the temperature rise under demineralized human tooth dentin during light-curing and the degrees of conversion (DCs) of three different RBMs using quartz tungsten halogen (QTH) and light-emitting diode (LED) units (LCUs). Demineralized and non-demineralized dentin disks were prepared from 120 extracted human mandibular molars. The temperature rise under the dentin disks (n = 12) during the light-curing of three RBMs, i.e. an Ormocer-based composite resin (Ceram. X, Dentsply DeTrey), a low-shrinkage silorane-based composite (Filtek P90, 3M ESPE), and a giomer (Beautifil II, Shofu GmbH), was measured with a K-type thermocouple wire. The DCs of the materials were investigated using Fourier transform infrared spectroscopy. The temperature rise under the demineralized dentin disks was higher than that under the non-demineralized dentin disks during the polymerization of all restorative materials (p 0.05). Although there were no significant differences in the DCs, the temperature rise under demineralized dentin disks for the silorane-based composite was higher than that for dimethacrylate-based restorative materials, particularly with QTH LCU.

  18. Temperature changes under demineralized dentin during polymerization of three resin-based restorative materials using QTH and LED units

    Directory of Open Access Journals (Sweden)

    Sayed-Mostafa Mousavinasab

    2014-08-01

    Full Text Available Objectives Light-curing of resin-based materials (RBMs increases the pulp chamber temperature, with detrimental effects on the vital pulp. This in vitro study compared the temperature rise under demineralized human tooth dentin during light-curing and the degrees of conversion (DCs of three different RBMs using quartz tungsten halogen (QTH and light-emitting diode (LED units (LCUs. Materials and Methods Demineralized and non-demineralized dentin disks were prepared from 120 extracted human mandibular molars. The temperature rise under the dentin disks (n = 12 during the light-curing of three RBMs, i.e. an Ormocer-based composite resin (Ceram. X, Dentsply DeTrey, a low-shrinkage silorane-based composite (Filtek P90, 3M ESPE, and a giomer (Beautifil II, Shofu GmbH, was measured with a K-type thermocouple wire. The DCs of the materials were investigated using Fourier transform infrared spectroscopy. Results The temperature rise under the demineralized dentin disks was higher than that under the non-demineralized dentin disks during the polymerization of all restorative materials (p 0.05. Conclusions Although there were no significant differences in the DCs, the temperature rise under demineralized dentin disks for the silorane-based composite was higher than that for dimethacrylate-based restorative materials, particularly with QTH LCU.

  19. Ectodermal dysplasia: otolaryngologic evaluation of 23 cases.

    Science.gov (United States)

    Yildirim, Muzeyyen; Yorgancilar, Ediz; Gun, Ramazan; Topcu, Ismail

    2012-02-01

    The aim of this prospective study was to improve the quality of life of and reduce morbidity for patients with ectodermal dysplasia by assessing their actual and potential ENT pathologies, and offering methods of prevention and treatment. The study was conducted between 2006 and 2008 and included 23 patients diagnosed with ectodermal dysplasia. The major symptoms of ectodermal dysplasia were evaluated. Patient histories were obtained in all cases, and a complete head and neck examination was carried out. Of the 23 patients (11 males and 12 females, aged 5 to 45 years) diagnosed with ectodermal dysplasia, 22 had hypohidrotic ectodermal dysplasia and 1 had ectrodactyly-ectodermal dysplasia-clefting syndrome. In all patients diagnosed with hypohidrotic ectodermal dysplasia, the salivary glands were examined by ultrasonography and, when necessary, by scintigraphy. Hearing defects in patients with otologic problems were determined by audiometric examination: 39.1% of the patients had hearing loss, 43.5% had otitis media, and 39.1% had impacted cerumen. The most common rhinologic findings were saddle nose deformity in 56.5%, nasal obstruction and nasal dryness (52.2% each), and chronic rhinitis/rhinosinusitis (34.8%). The most common oral and oropharyngeal findings were difficulty chewing in 82.6% and dry mouth in 78.3%. All 23 patients had required dental work. Because this disorder affects several aspects of the body, its treatment requires a multidisciplinary approach, with the otolaryngologist being a vital part of the management team.

  20. Effect of Nanofiller Addition to an Experimental Dentin Adhesive on Microtensile Bond Strength to Human Dentin

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    SH. Kasraei

    2009-06-01

    Full Text Available Objective: The purpose of the study was to evaluate the influence of adding nanofiller particles to a dentin bonding agent on resin-dentin bond strength.Materials and Methods: Fifty-four human intact premolar teeth were divided in to 6 groups of nine. The teeth were ground on occlusal surfaces and polished with 320 and then 600 grit silicon carbide papers. An experimental bonding system based on acetone/alcoholsolvent was provided with filler contents of 0.0, 0.5, 1.0, 2.5, 5.0, and 10.0 weight percent fumed silica nanofiller. After dentin surface etching, rinsing and blot drying, the experimentalbonding agents were applied to dentin surface. A composite resin was, then,bonded to the dentin on the bonding agent. The specimens were thermocycled for 500 cycles and sectioned in stick form. After two week of storage in distilled water, resin-dentin microtensile bond strength of the specimens was measured. Data were analyzed by one way ANOVA and DunnettT3 tests.Results: Bond strength to dentin was significantly affected by the filler level. Minimum and maximum resin-microtensile bond strength was in the experimental bonding agent with no filler (5.88 MPa and with filler level of 1.0 weight percent (15.15 MPa, respectively,and decreased with the increase of filler content down to 8.95 MPa for the filler level of 10.0 weight percent.Conclusion: Filler content seems to be one of the important factors influencing the bond strength of dental adhesives. Maximum dentin bond strength was obtained with 1% silanized nanofiller silica added to experimental adhesive system.

  1. Optimization of the etch-and-rinse technique: New perspectives to improve resin-dentin bonding and hybrid layer integrity by reducing residual water using dimethyl sulfoxide pretreatments.

    Science.gov (United States)

    Stape, Thiago Henrique Scarabello; Tjäderhane, Leo; Abuna, Gabriel; Sinhoreti, Mário Alexandre Coelho; Martins, Luís Roberto Marcondes; Tezvergil-Mutluay, Arzu

    2018-04-13

    To determine whether bonding effectiveness and hybrid layer integrity on acid-etched dehydrated dentin would be comparable to the conventional wet-bonding technique through new dentin biomodification approaches using dimethyl sulfoxide (DMSO). Etched dentin surfaces from extracted sound molars were randomly bonded in wet or dry conditions (30s air drying) with DMSO/ethanol or DMSO/H 2 O as pretreatments using a simplified (Scotchbond Universal Adhesive, 3M ESPE: SU) and a multi-step (Adper Scotchbond Multi-Purpose, 3M ESPE: SBMP) etch-and-rinse adhesives. Untreated dentin surfaces served as control. Bonded teeth (n=8) were stored in distilled water for 24h and sectioned into resin-dentin beams (0.8mm 2 ) for microtensile bond strength test and quantitative interfacial nanoleakage analysis (n=8) under SEM. Additional teeth (n=2) were prepared for micropermeability assessment by CFLSM under simulated pulpar pressure (20cm H 2 O) using 5mM fluorescein as a tracer. Microtensile data was analyzed by 3-way ANOVA followed by Tukey Test and nanoleakage by Kruskal-Wallis and Dunn-Bonferroni multiple comparison test (α=0.05). While dry-bonding of SBMP produced significantly lower bond strengths than wet-bonding (padhesives to demineralized air-dried dentin beyond conventional wet-bonding. Less porous resin-dentin interfaces with higher bond strengths on air-dried etched dentin were achieved; nonetheless, overall efficiency varied according to DMSO's co-solvent and adhesive type. DMSO pretreatments permit etched dentin to be air-dried before hybridization facilitating residual water removal and thus improving bonding effectiveness. This challenges the current paradigm of wet-bonding requirement for the etch-and-rinse approach creating new possibilities to enhance the clinical longevity of resin-dentin interfaces. Copyright © 2018 The Academy of Dental Materials. Published by Elsevier Inc. All rights reserved.

  2. Anhidrotic ectodermal dysplasia presenting as atrophic rhinitis.

    Science.gov (United States)

    Barman, Debasis; Mandal, Satadal; Nandi, Santanu; Banerjee, Pranabashish; Rashid, M A

    2011-11-01

    Ectodermal dysplasia is a complex group of familial disorders with numerous clinical characteristics, with an incidence of 7 in 10000 born alive children. Ectodermal dysplasia affects structures of ectodermal origin like the skin and its appendages as well as other non-ectodermal structures. The most common sites of involvement are the defects in the skin, hair, teeth, nails and sweat glands,which are of ectodermal origin. Though the dermatologists and paediatricians often manage such cases, we report one case of ectodermal dysplasia presenting with atrophic rhinitis.

  3. Cleidocranial Dysplasia: Report of Two Cases

    Directory of Open Access Journals (Sweden)

    Avinash Kshar

    2010-01-01

    Full Text Available Cleidocranial dysplasia constitutes a congenital disorder manifested primarily in the development of facial and cranial bones, as well as partial development or complete absence of the clavicles and problems also arise on the number and eruption of teeth. It is a rare disease with autosomal dominant but 40% cases represented spontaneous mutations. This disease has no sex predilection. It is characterized by a generalized skeletal dysplasia. 1 In this article we are reporting two cases of cleidocranial dysplasia in son and father with characteristic clinical and radiographic features.

  4. p53 expression and mutation analysis of odontogenic cysts with and without dysplasia.

    Science.gov (United States)

    Cox, Darren P

    2012-01-01

    Overexpression of p53 protein is well described in odontogenic cystic lesions (OCLs), including those with epithelial dysplasia; however, most p53 antibodies stain both wild-type and mutated p53 protein and may not reflect genotype. Direct sequencing of the p53 gene has not identified mutations in OCLs with dysplasia. The purpose of this study was to determine the molecular basis of p53 expression in several types of OCLs with and without dysplasia. The study material comprised 13 OCLs: odontogenic keratocyst (n = 5), orthokeratinized odontogenic cyst (n = 5), dentigerous cyst (n = 2), lateral periodontal cyst (n = 1), and unspecified developmental odontogenic cyst (UDOC) (n = 1). Five of these had features of mild or moderate epithelial dysplasia. One intraosseous squamous cell carcinoma (SCC) that was believed to have arisen from an antecedent dysplastic orthokeratinized OC was also included. Immunohistochemistry was performed using the DO7 monoclonal antibody that recognizes wild-type and mutated p53. DNA was extracted from microdissected tissue for all samples and exons 4 to 8 of the p53 gene direct sequenced. In 4 of 5 OCLs with dysplasia there was strong nuclear staining of basal and suprabasal cells. In all cases without dysplasia, nuclear expression in basal cells was either negative or weak and was absent in suprabasal cell nuclei. A mutation in exon 6 of the p53 gene (E224D) was identified in both the dysplastic orthokeratinized OC and the subsequent intraosseous SCC. OCLs with features of dysplasia show increased expression of p53 protein that does not reflect p53 mutational status. One dysplastic OC shared the same p53 mutation with a subsequent intraosseous SCC, indicating that p53 mutation may be associated with malignant transformation in this case. Copyright © 2012 Elsevier Inc. All rights reserved.

  5. Effects of endodontic tri-antibiotic paste on bond strengths of dentin adhesives to coronal dentin

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    Parvin Mirzakoucheki

    2015-05-01

    Full Text Available Objectives The aim of this study was to evaluate the effects of tri-antibiotic paste (TAP on microtensile bond strengths (MTBS of dental adhesives to dentin. Materials and Methods Sixty extracted molars had their occlusal surfaces flattened to expose dentin. They were divided into two groups, i.e., control group with no dentin treatment and experimental group with dentin treatment with TAP. After 10 days, specimens were bonded using self-etch (Filtek P90 adhesive or etch-and-rinse (Adper Single Bond Plus adhesives and restored with composite resin. Teeth were sectioned into beams, and the specimens were subjected to MTBS test. Data were analyzed using two-way ANOVA and post hoc Tukey tests. Results There was a statistically significant interaction between dentin treatment and adhesive on MTBS to coronal dentin (p = 0.003. Despite a trend towards worse MTBS being noticed in the experimental groups, TAP application showed no significant effect on MTBS (p = 0.064. Conclusions The etch-and-rinse adhesive Adper Single Bond Plus presented higher mean bond strengths than the self-etch adhesive Filtek P90, irrespective of the group. The superior bond performance for Adper Single Bond when compared to Filtek P90 adhesive was confirmed by a fewer number of adhesive failures. The influence of TAP in bond strength is insignificant.

  6. Dentine hypersensitivity: real or imagined | Gbadebo | Nigerian ...

    African Journals Online (AJOL)

    Background: Dentine hypersensitivity is a common presentation of cause of pain and or discomfort with mastication which has been shown to affect the quality of life of the affected individual. It is also a common cause of presentation at the dental clinics. However, the cause, diagnosis and possible management to give relief ...

  7. Hydrofluoric acid on dentin should be avoided.

    NARCIS (Netherlands)

    Loomans, B.A.C.; Mine, A.; Roeters, F.J.M.; Opdam, N.J.M.; Munck, J. De; Huysmans, M.C.D.N.J.M.; Meerbeek, B. Van

    2010-01-01

    Hydrofluoric acid can be used for intra-oral repair of restorations. Contamination of tooth substrate with hydrofluoric acid cannot always be avoided. OBJECTIVES: To investigate the bonding effectiveness to hydrofluoric acid contaminated dentin by, micro-tensile bond strength testing, SEM and TEM.

  8. Penetration of amalgam constituents into dentine

    NARCIS (Netherlands)

    Scholtanus, Johannes D.; Ozcan, Mutlu; Huysmans, Marie-Charlotte D. N. J. M.

    Objectives: Amalgam restorations are replaced by adhesively placed composite resin restorations at an increasing rate. After the removal of amalgam dentine often shows marked dark discoloration that is attributed to the penetration of corrosion products from overlying amalgams. it is questioned

  9. Penetration of amalgam constituents into dentine.

    NARCIS (Netherlands)

    Scholtanus, J.D.; Ozcan, M.; Huysmans, M.C.D.N.J.M.

    2009-01-01

    OBJECTIVES: Amalgam restorations are replaced by adhesively placed composite resin restorations at an increasing rate. After the removal of amalgam dentine often shows marked dark discoloration that is attributed to the penetration of corrosion products from overlying amalgams. It is questioned

  10. Dentin Hypersensitivity: Recent Concepts in Management

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    Vijay Mantri

    2011-01-01

    Full Text Available Tooth sensitivity is a very common clinical presentation which can cause considerable concern for patients. Dentin hypersensitivity (DH is characterized by short sharp pain arising from exposed dentin in response to stimuli. The most widely accepted theory of how the pain occurs is Brannstrom′s hydrodynamic theory, fluid movement within the dentinal tubules. The condition generally involves the facial surfaces of teeth near the cervical aspect and is very common in premolars and canines. This condition is frequently encountered by dentists, periodontists, hygienists and dental therapists. Some dental professionals lack confidence in treating DH. The management of this condition requires a good understanding of the complexity of the problem, as well as the variety of treatments available. This review considers the etiopathogenesis, incidence, diagnosis, prevention and management of dentinal hypersensitivity. DH is diagnosed after elimination of other possible causes of the pain. Any treatment plan for DH should include identifying and eliminating predisposing etiologic factors. Professionals should appreciate the role causative factors play in localizing and initiating hypersensitive lesions. It is important to identify these factors so that prevention can be included in the treatment plan. Treatments can be self-administered by the patient at home or be applied by a dental professional in the dental office. At-home methods tend to be simple and inexpensive and can treat simultaneously generalized DH affecting many teeth Desensitizing treatment should be delivered systematically, beginning with prevention and at-home treatments. The latter may be supplemented with in-office modalities.

  11. The Possible Role of Dentin as a Piezoelectric Signal Generator by Determining the Elec-tromechanical Coupling Factor of Dentin

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    Atabak Shahidi

    2011-08-01

    Full Text Available Introduction: This article aimed at calculation of the electromechanical coupling factor of dentin which is an indicator of the effectiveness with which a piezoelectric material converts electrical en-ergy into mechanical energy, or vice versa. The hypothesis: The electro-mechanical coupling factor of dentin was determined in mode 11 and 33 by calculating the ratio of the produced electrical energy to the stored elastic energy in dentin under applied pressure. This study showed that the electromechanical coupling factor of dentin was affected by the direction of the applied force and the moisture content of dentin. Also dentin was a weak electromechanical energy converter which might be categorized as a piezoelectric pressure sensor.Evaluation of the hypothesis: Determination of the electrome-chanical coupling factor of dentin and its other piezoelectric constants is essential to investigate the biologic role of piezoelectricity in tooth.

  12. Developmental hip dysplasia in adolescence

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    Vukašinović Zoran

    2009-01-01

    Full Text Available The authors define adolescence and developmental dysplasia of the hip (DDH. Special attention is paid to pathological findings characteristic of DDH in adolescence (unrecognized and untreated DDH; treated DDH, but non-terminated treatment; DDH diagnosed with delay, inadequately treated, with complications. The authors emphasise that DDH treatment has to be successfully terminated well before the adolescence; possibilities are explained on management modes at the time of adolescence, and possible persons guilty for the persistence of later hip problems are indicated. Based on the authors' experience and having in mind all surgical possibilities for the treatment (pelvic osteotomies, femoral osteotomies, trochanteroplasties, leg length equalization procedures the authors propose treatment protocols. The intention is to provide better treatment results and to prevent secondary hip arthrosis. Furthermore, how to improve the struggle against DDH is suggested.

  13. Diastrophic dysplasia: prenatal diagnosis and review of the literature

    Directory of Open Access Journals (Sweden)

    Jonathan Celli Honório

    Full Text Available CONTEXT Diastrophic dysplasia is a type of osteochondrodysplasia caused by homozygous mutation in the gene DTDST (diastrophic dysplasia sulfate transporter gene. Abnormalities occurring particularly in the skeletal and cartilaginous system are typical of the disease, which has an incidence of 1 in 100,000 live births. CASE REPORT The case of a pregnant woman, without any consanguineous relationship with her husband, whose fetus was diagnosed with skeletal dysplasia based on ultrasound findings and DNA tests, is described. An obstetric ultrasound scan produced in the 16th week of gestation revealed characteristics that guided the clinical diagnosis. Prominent among these characteristics were rhizomelia of the lower and upper limbs (shortening of the proximal portions and mesomelia (shortening of the intermediate portions. Both upper limbs showed marked curvature, with the first finger of the upper limbs in abduction and clinodactyly of the fifth finger. Molecular analysis using the polymerase chain reaction (PCR and gene sequencing detected mutations that had already been described in the literature for the gene DTDST, named c.862C > T and c.2147_2148insCT. Therefore, the fetus was a compound heterozygote, carrying two different mutations. CONCLUSIONS Prenatal diagnosis of this condition allowed a more realistic interpretation of the prognosis, and of the couple's reproductive future. This case report shows the contribution of molecular genetics towards the prenatal diagnosis, for which there are few descriptions in the literature.

  14. Anesthesia for a patient of acromesomelic dysplasia with associated hydrocephalus, Arnold Chiari malformation and syringomyelia

    Directory of Open Access Journals (Sweden)

    Rudrashish Haldar

    2013-01-01

    Full Text Available Acromesomelic dysplasias are autosomal recessive osteochondrodysplasias. Acromesomelic dysplasia Maroteaux-type (AMDM, also known as St Helena dysplasia, is of two types: The classical and the mild variety. About 50 cases of AMDM have been reported till date, most of them being the classical variety. There is scarcity of literature on anesthesia for such patients. We are reporting a case of general anesthetic management of AMDM, associated with hydrocephalus, Arnold Chiari malformation type-1 and syringomyelia. The patient was a 10-year-old short-statured boy who presented with symptomatic thoracic kyphoscoliosis, gibbus deformity and back pain. On examination, there was no neurological deficit. Radiology revealed thoracic kyphoscoliosis, mild ventriculomegaly and upper cervical syringomyelia. The patient underwent posterior fossa decompression in the prone position under general anesthesia. We will discuss the anesthetic considerations for such patients and review the pertinent literature.

  15. Characterization of Genipin-Modified Dentin Collagen

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    Hiroko Nagaoka

    2014-01-01

    Full Text Available Application of biomodification techniques to dentin can improve its biochemical and biomechanical properties. Several collagen cross-linking agents have been reported to strengthen the mechanical properties of dentin. However, the characteristics of collagen that has undergone agent-induced biomodification are not well understood. The objective of this study was to analyze the effects of a natural cross-linking agent, genipin (GE, on dentin discoloration, collagen stability, and changes in amino acid composition and lysyl oxidase mediated natural collagen cross-links. Dentin collagen obtained from extracted bovine teeth was treated with three different concentrations of GE (0.01%, 0.1%, and 0.5% for several treatment times (0–24 h. Changes in biochemical properties of NaB3H4-reduced collagen were characterized by amino acid and cross-link analyses. The treatment of dentin collagen with GE resulted in a concentration- and time-dependent pigmentation and stability against bacterial collagenase. The lysyl oxidase-mediated trivalent mature cross-link, pyridinoline, showed no difference among all groups while the major divalent immature cross-link, dehydro-dihydroxylysinonorleucine/its ketoamine in collagen treated with 0.5% GE for 24 h, significantly decreased compared to control (P< 0.05. The newly formed GE-induced cross-links most likely involve lysine and hydroxylysine residues of collagen in a concentration-dependent manner. Some of these cross-links appear to be reducible and stabilized with NaB3H4.

  16. Camptomelic dysplasia: A case report

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    Koš Radmila

    2007-01-01

    Full Text Available Campomelic/camptomelic dysplasia is a very rare, severe osteochondrodysplasia characterised by severe skeletal and nonskeletal malformations and lethal outcome mainly in neonatal period. Characteristic abnormality by which the syndrome got its name is short, bowed long bones of lower extremities, most often of femur, manifested by short and bowed legs. Skin dimpling on tibial anterior side is another prominent characteristic of this syndrome. Severe cases are inherited by autosomal dominant trait, by mutation Sox9 gene on chromosome 17, with lethal outcome in the first days of life. Less severe forms of the disease are due to balanced translocation t (13;17 with life span up to the third decade of life. A majority of karyotypic males present as phenotypic females. We report a case of a female neonate, without consanguinity between parents, with characteristic signs of camptomelic dysplasia with short birth length of 46 cm, macrocephaly (head circumference 39 cm, dolichocephaly, hydrocephalus, short trunk and legs. Narrow rib cage, bowed lower extremities, short hand and foot phalanges, nail hypoplasia were noticed. Anterior fontanelle was enlarged, high forehead, face small and flat, hypertelorism, low nasal bridge, micrognathia, low set ears, cleft palate, were found. Characteristic skin dimpling on anterior side of tibia was present on both legs. Bone X-ray studies presented the following changes: anterior bowing of shortened femurs, hip dislocation, cervical vertebrae, scapulas, eleven pairs of slender ribs. Hip luxation. Karyotype was normal for a female, 46 XX. Respiratory insufficiency was present since birth, exacerbated, and led to lethal outcome in the second day of life, as described in the majority of these patients. .

  17. Limb malformations with associated congenital constriction rings in two unrelated Egyptian males, one with a disorganization-like spectrum and the other with a probable distinct type of septo-optic dysplasia.

    Science.gov (United States)

    Temtamy, Samia A; Aglan, Mona S; Ashour, Adel M; El-Badry, Tarek H

    2010-01-01

    In this report, we describe two unrelated Egyptian male infants with limb malformations and constriction rings. The first case is developing normally but has severe limb anomalies, congenital constriction rings, scoliosis because of vertebral anomalies, a left accessory nipple, a small tumor-like swelling on his lower back with tiny skin tubular appendages, a hypoplastic scrotum, and an anchored penis. The second case is developmentally delayed with limb malformations, congenital constriction rings, a lumbar myelomeningeocele, hemangioma, and tiny tubular skin appendages on the back. The patient also had bilateral optic atrophy. The constellation of features in our patients cannot be fully explained by the amniotic disruption complex. The first patient may represent an additional case of the human homolog of the mouse disorganization mutant. The presence of bilateral optic atrophy in the second case, although without an absent septum pellucidum nor other brain anomalies resembles the infrequently reported disorder of septo-optic dysplasia with limb anomalies. Both cases were sporadic and could be caused by a new dominant mutation because of the high paternal age of case 1 and the history of paternal occupational exposure to heat for both fathers. We draw attention to the phenotypic overlap between the disorganization-like syndrome and septo-optic dysplasia with limb anomalies.

  18. Bonding of the Polymer Polyetheretherketone (PEEK) to Human Dentin: Effect of Surface Treatments.

    Science.gov (United States)

    Rocha, Regina Furbino Villefort; Anami, Lilian Costa; Campos, Tiago Moreira Bastos; Melo, Renata Marques de; Souza, Rodrigo Othávio de Assunção E; Bottino, Marco Antonio

    2016-01-01

    Polyetheretherketone (PEEK) is a material suitable for frameworks of fixed dental prostheses. The effect of different surface treatments on the bond strength of PEEK bonded to human dentin was evaluated. One hundred PEEK cylinders (3 mm×3 mm) were divided into five groups according to surface treatment: silica coating, sandblasting with 45 μm Al2O3 particles, etching with 98% sulfuric acid for 5, 30 and for 60 s. These cylinders were luted with resin cement onto 50 human molars. First, each tooth was embedded in epoxy resin and the buccal dentin surface was exposed. Then, two delimited dentin areas (Æ:3 mm) per tooth were etched with 35% phosphoric acid and bonded with a two-step self-priming adhesive system. After the luting procedure the specimens were stored in water (24 h/37 °C). Shear bond strength (SBS) was tested using a universal testing machine (crosshead speed 0.5 mm/min; load cell 50 kgf) and failure types were assessed. Stress data (MPa) were analyzed using the Kruskal-Wallis test. Comparison of the proportions of different failure types was performed using the Bonferroni method (pPEEK, resin cement and dentin.

  19. Andhidrotic ectodermal dysplasia-autosomal recessive form

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    Inamadar Arun

    1994-01-01

    Full Text Available Anhidrotic ectodermal dysplasia with classical features in 2 sisters is reported. The mode of inheritance in these seems to be autosomal recessive; which is a very rare occurrence.

  20. CT Imaging of Craniofacial Fibrous Dysplasia

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    Zerrin Unal Erzurumlu

    2015-01-01

    Full Text Available Fibrous dysplasia is a benign fibroosseous bone dysplasia that can involve single (monostotic or multiple (polyostotic bones. Monostotic form is more frequent in the jaws. It is termed as craniofacial fibrous dysplasia, when it involves, though rarely, adjacent craniofacial bones. A 16-year-old girl consulted for a painless swelling in the right posterior mandible for two years. Panoramic radiography revealed ground-glass ill-defined lesions in the three different regions of the maxilla and mandible. Axial CT scan (bone window showed multiple lesions involving skull base and facial bones. Despite lesions in the skull base, the patient had no abnormal neurological findings. The lesion was diagnosed as fibrous dysplasia based on radiological and histopathological examination. In this paper, CT findings and differential diagnosis of CFD are discussed. CT is a useful imaging technique for CFD cases.

  1. Bilateral anophthalmia with septo-optic dysplasia

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    Manisha Jana

    2010-01-01

    Full Text Available Bilateral anophthalmia is a rare entity and association with septo-optic dysplasia is an even rare condition. The condition is characterized by absent eyeballs in the presence of eyelids, conjunctiva or lacrimal apparatus. Though anophthalmia can be diagnosed clinically, imaging plays a crucial role in delineating the associated anomalies. In addition, often clinical anophthalmia may prove to be severe microphthalmia on imaging. We describe the imaging findings in an infant with bilateral anophthalmia and septo-optic dysplasia.

  2. Renal dysplasia in a Rhodesian Ridgeback dog

    International Nuclear Information System (INIS)

    Lobetti, R.G.; Pearson, J.; Jimenez, M.

    1996-01-01

    A six-month-old Rhodesian ridgeback dog was presented for evaluation of facial swelling. Chronic renal failure was clinically diagnosed based on urinalysis, biochemical changes and ultrasonography. The facial swelling was due to fibrous osteodystrophy, which was evident on survey radiographs of the skull. On post mortem examination, chronic renal failure as a result of renal dysplasia was confirmed. This is the first reported case of renal dysplasia in this breed of dog

  3. Hip dysplasia and congenital hip dislocation

    Energy Technology Data Exchange (ETDEWEB)

    Lingg, G.; Nebel, G.; von Torklus, D.

    1981-11-01

    In human genetics and orthopedics quite different answers have been given to the question of hereditary transmission and frequency of hip dysplasia in families of children with congenital hip dislocation. We therefore have made roentgenometric measurements of 110 parents of children with congenital hip dislocation. In 25% we found abnormal flat acetabulae, whereas 12% had pathologic deep hips. This may propose a new concept of morphology of congenital hip dysplasia.

  4. Dysplasia epiphysealis hemimelica of the tibial tubercle

    Energy Technology Data Exchange (ETDEWEB)

    Thacker, M.M.; Scully, S.P.; Pitcher, J.D.; Temple, H. Thomas [University of Miami, Department of Orthopedics and Rehabilitation, FL (United States); Azouz, E.M. [University of Miami, Department of Radiology, FL (United States)

    2006-03-15

    Dysplasia epiphysealis hemimelica (DEH) is a rare skeletal dysplasia with epiphyseal involvement first described by Mouchet and Belot in 1926. Lower extremity involvement is common and might involve a single or multiple epiphyses in the affected extremity. We report an unusual case of involvement of the tibial tubercle in a girl aged 4 years 8 months, and we present the clinical, radiographic and pathologic findings. We discuss the role of MRI in the diagnosis and treatment plan. (orig.)

  5. Dentinal tubule occluding capability of nano-hydroxyapatite; The in-vitro evaluation.

    Science.gov (United States)

    Baglar, Serdar; Erdem, Umit; Dogan, Mustafa; Turkoz, Mustafa

    2018-04-29

    In this in-vitro study, the effectiveness of experimental pure nano-hydroxyapatite (nHAP) and 1%, 2%, and 3% F¯ doped nano-HAp on dentine tubule occlusion was investigated. And also, the cytotoxicity of materials used in the experiment was evaluated. Nano-HAp types were synthesized by the precipitation method. Forty dentin specimens were randomly divided into five groups of; 1-no treatment (control), 2-specimens treated with 10% pure nano-HAp and 3, 4, 5 specimens treated with 1%, 2%, and 3% F - doped 10% nano-HAp, respectively. To evaluate the effectiveness of the materials used; pH, FTIR, and scanning electron microscopy evaluations were performed before and after degredation in simulated body fluid. To determine cytotoxicity of the materials, MTT assay was performed. Statistical evaluations were performed with F and t tests. All of the nano-HAp materials used in this study built up an effective covering layer on the dentin surfaces even with plugs in tubules. It was found that this layer had also a resistance to degradation. None of the evaluated nano-HAp types were have toxicity. Fluoride doping showed a positive effect on physical and chemical stability until a critical value of 1% F - . The all evaluated nano-HAp types may be effectively used in dentin hypersensitivity treatment. The formed nano-HAp layers were seem to resistant to hydrolic deletion. The pure and 1% F - doped nano-HAp showed the highest biocompatibility thus it was assessed that pure and 1% F - doped materials may be used as an active ingredient in dentin hypersensitivity agents. © 2018 Wiley Periodicals, Inc.

  6. Shear bond strength of amalgam to dentin using different dentin adhesive systems

    Directory of Open Access Journals (Sweden)

    Farimah Sardari

    2012-01-01

    Full Text Available Background and Aims: The aim of this in vitro study was to assess the shear bond strength of amalgam to dentin using four dentin adhesive systems.Materials and Methods: One hundred human molars were selected. After enamel removal, a dentin cylinder with 3 mm thickness was prepared. Eighty specimens were resorted with amalgam and four dentin adhesive systems as follows (n=20: group 1, Scotch Bond Multi-Purpose; group 2, One Coat Bond; group 3, PQ1; and group 4, Panavia-F. In group 5, 20 specimens were resorted with amalgam and varnish as control group. The specimens were incubated at 37°C for 24 h. The shear bond strengths were then measured by using push out method. The data were analyzed by one-way ANOVA and post hoc Duncan's tests.Results: Mean values for bond strengths of test groups were as follows: group 1=21.03±8.9, group 2=23.47±9, group 3=13.16±8.8, group 4=20.07±8.9 and group 5=14.15±8.7 MPa±SD. One-way ANOVA showed the statistically significant difference between the bond strengths of five groups (P=0.001. Post hoc Duncan's test showed significant difference between groups 1and 3 (P=0.008, groups 1 and 5 (P=0.019, groups 2 and 5 (P=0.0008, groups 4 and 5 (P=0.042, and groups 3 and 4 (P=0.018.Conclusion: Results of this study showed that the bond strength of amalgam to dentin using One Coat Bond as dentin adhesive system was higher than that observed in other dentin adhesive systems.

  7. [Clinical and molecular study in a child with X-linked hypohidrotic ectodermal dysplasia].

    Science.gov (United States)

    Callea, Michele; Yavuz, Izzet; Clarich, Gabriella; Cammarata-Scalisi, Francisco

    2015-12-01

    Ectodermal dysplasia encompasses more than 200 clinically distinct entities, which affect at least two structures derived from the ectoderm, including the skin, hair, nails, teeth, sweat glands, and sebaceous glands. X-linked hypohidrotic ectodermal dysplasia is the most common type and is caused by mutation of the EDA gene that encodes Ectodysplasin-A. It occurs in less than 1 in 100 000 individuals and is clinically characterized by hypodontia, hypohidrosis, hypotrichosis, and eye dis orders. We present a child evaluated in a multidisciplinary manner with clinical and molecular diagnosis of X-linked hypohidrotic ectodermal dysplasia with type missense mutation c.1133C> T; p.T378M in EDA gene.

  8. Florid cemento-osseous dysplasia and peripheral giant cell granuloma in a patient with neurofibromatosis 1.

    Science.gov (United States)

    Sarmento, Dmitry José de Santana; Carvalho, Sérgio Henrique Gonçalves de; Araújo, José Cadmo Wanderley Peregrino de; Carvalho, Marianne de Vasconcelos; Silveira, Éricka Janine Dantas da

    2017-01-01

    We report a 35-year-old mulatto female patient with neurofibromatosis Type 1 who presented with facial asymmetry. The patient had two lesions: florid cemento-osseous dysplasia associated with peripheral giant cell granuloma. She was referred for surgical treatment of the peripheral giant cell granuloma and the florid cemento-osseous dysplasia was treated conservatively by a multidisciplinary team. So far, no changes have been observed in the patient's clinical status. We observed no recurrence of peripheral giant cell granuloma. To the best of our knowledge, the present case is the first report of a patient with neurofibromatosis Type 1 associated with a giant cell lesion and florid cemento-osseous dysplasia.

  9. Florid cemento-osseous dysplasia and peripheral giant cell granuloma in a patient with neurofibromatosis 1*

    Science.gov (United States)

    Sarmento, Dmitry José de Santana; de Carvalho, Sérgio Henrique Gonçalves; de Araújo Filho, José Cadmo Wanderley Peregrino; Carvalho, Marianne de Vasconcelos; da Silveira, Éricka Janine Dantas

    2017-01-01

    We report a 35-year-old mulatto female patient with neurofibromatosis Type 1 who presented with facial asymmetry. The patient had two lesions: florid cemento-osseous dysplasia associated with peripheral giant cell granuloma. She was referred for surgical treatment of the peripheral giant cell granuloma and the florid cemento-osseous dysplasia was treated conservatively by a multidisciplinary team. So far, no changes have been observed in the patient's clinical status. We observed no recurrence of peripheral giant cell granuloma. To the best of our knowledge, the present case is the first report of a patient with neurofibromatosis Type 1 associated with a giant cell lesion and florid cemento-osseous dysplasia. PMID:28538890

  10. Radiological features of bilateral hereditary micro-epiphyseal dysplasia - a distinct entity in the skeletal dysplasias

    NARCIS (Netherlands)

    Morstert, AK; Dijkstra, PF; van Horn, [No Value; Jansen, BRH; Heutink, P; Lindhout, D

    Aim: To prove that bilateral hereditary micro-epiphyseal dysplasia (BHMED), first described by Elsbach in 1959 [1], is a distinct disorder radiologically as well as clinically, compared with multiple epiphyseal dysplasia (MED). Material and Methods: We used the data of the revised pedigree with 84

  11. Effect of chlorhexidine on the adhesion of dental cements in dentin

    International Nuclear Information System (INIS)

    Carvajal Villalobos, Marco Vinicio; Torres Montoya, Jeimy; Vindas Ramirez, Cinthya Carolina

    2013-01-01

    The effect of pretreatment with 0.2% chlorhexidine was evaluated in the adhesion strength in the dentin shear of three self-adhesive cements after one week of storage. 48 healthy teeth (premolars and molars) extracted were used. The occlusal surface was removed, exposing the dentin. Self-adhesive cements RelyX U100 (3M ESPE), SpeedCEM (Ivoclar) and BisCem (Bisco) were placed in an Ultradent cylindrical abutment (2mm diameter) in dentin pretreated with 0.2% chlorhexidine for 60 seconds and in dentin without treatment for 40 seconds. Removed the cylinder all the samples were photocured an additional 40 seconds. After one week, the adhesion force was in eight groups on a universal test machine at 0.01 cm/min. The ANOVA tests were used to analyze the data. The use of chlorhexidine at 0.2% did not affect the average strength of adhesion in shear, while the type of cement if the affection [es

  12. Osteogenesis imperfecta with ectopic mineralizations in dentin and cementum and a COL1A2 mutation.

    Science.gov (United States)

    Kantaputra, Piranit Nik; Sirirungruangsarn, Yuddhasert; Intachai, Worrachet; Ngamphiw, Chumpol; Tongsima, Sissades; Dejkhamron, Prapai

    2018-04-10

    We report a Thai father (patient 1) and his daughter (patient 2) affected with osteogenesis imperfecta type IV and dentinogenesis imperfecta. Both were heterozygous for the c.1451G>A (p.Gly484Glu) mutation in COL1A2. The father, a Thai boxer, had very mild osteogenesis imperfecta with no history of low-trauma bone fractures. Scanning electron micrography of the primary teeth with DI of the patient 2, and the primary teeth with DI of another OI patient with OI showed newly recognized dental manifestations of teeth with DI. Normal dentin and cementum might have small areas of ectopic mineralizations. Teeth affected with DI have well-organized ectopic mineralizations in dentin and cementum. The "French-fries-appearance" of the crystals at the cemento-dentinal junction and abnormal cementum have never been reported to be associated with dentinogenesis imperfecta, either isolated or osteogenesis imperfecta-associated. Our study shows for the first time that abnormal collagen fibers can lead to ectopic mineralization in dentin and cementum and abnormal cementum can be a part of osteogenesis imperfecta.

  13. Effect of Enamel and Dentin Surface Treatment on the Self-Adhesive Resin Cement Bond Strength.

    Science.gov (United States)

    Mushashe, Amanda Mahmmad; Gonzaga, Carla Castiglia; Cunha, Leonardo Fernandes da; Furuse, Adilson Yoshio; Moro, Alexandre; Correr, Gisele Maria

    2016-01-01

    The aim of this study was to evaluate the effect of enamel and dentin surface treatment on the micro-shear bond strength of self-adhesive cement. Seventy-two extracted third molars had their crowns embedded in acrylic resin and worn to obtain a flat enamel or dentin surface. The enamel and dentin specimens were randomly assigned to 8 groups (n=12) that were based on surface treatment (11.5% polyacrylic acid solution or no treatment), substrate condition (wet or dry) and storage period (1 day or 90 days), and treated accordingly. Cylinders (1 × 1 mm) were fabricated using self-adhesive resin cement (RelyX U200) following the manufacturer's instructions. The specimens were stored in distilled water at 37 °C for either 1 day or 90 days and subjected to micro-shear bond strength test (EMIC DL 2000 at 0.5 mm/min). After this, the failure type of the specimens was determined. Data were subjected to statistical analysis (a=0.05). According to the results, the 11.5% polyacrylic acid application decreased the bond strength in both enamel and dentin samples. The moist groups showed higher bond strength than the dry ones, regardless of the substrate and surface treatment. Storage period did not influence bond strength. In conclusion, surface treatment with 11.5% polyacrylic acid and absence of moisture decreased the bond strength of the resin-cement (RelyU200), regardless of the storage period.

  14. Effect of adhesive resin cements on bond strength of ceramic core materials to dentin.

    Science.gov (United States)

    Gundogdu, M; Aladag, L I

    2018-03-01

    The aim of the present study was to evaluate the effects of self-etch and self-adhesive resin cements on the shear bond strength of ceramic core materials bonded to dentin. Extracted, caries-free, human central maxillary incisor teeth were selected, and the vestibule surfaces were cut flat to obtain dentin surfaces. Ceramic core materials (IPS e.max Press and Prettau Zirconia) were luted to the dentin surfaces using three self-etch adhesive systems (Duo-Link, Panavia F 2.0, and RelyX Ultimate Clicker) and two self-adhesive resin systems (RelyX U200 Automix and Maxcem Elite). A shear bond strength test was performed using a universal testing machine. Failure modes were observed under a stereomicroscope, and bonding interfaces between the adhesive resin cements and the teeth were evaluated with a scanning electron microscope. Data were analyzed with Student's t-test and one-way analysis of variance followed by Tukey's test (α = 0.05). The type of adhesive resin cement significantly affected the shear bond strengths of ceramic core materials bonded to dentin (P materials when the specimens were luted with self-adhesive resin cements (P materials.

  15. [Mondini dysplasia: recurrent bacterial meningitis in adolescence].

    Science.gov (United States)

    Vargas-Dĭaz, J; Garófalo-Gómez, N; Rodríguez, U; Parra, M; Barroso-García, E; Novoa-López, L; Rojas-Massipe, E; Sardiñas-Hernández, N L

    Episodes of recurrent bacterial meningitis can occur in patients due to either congenital or acquired disorders. Congenital deformity of the bony labyrinth can be linked to a fistulous tract communicating it with the intracranial subarachnoid space. Mondini deformity is a frequent malformation in congenitally deaf patients. We report the case of an adolescent with a history of being unable to hear in one ear who, from the age of 10 years, began to suffer repeated bacterial meningoencephalitis with microbiological recovery of Streptococcus pneumoniae on three occasions. The type of germ recovered in the cerebrospinal fluid (CSF) and the history of congenital deafness that was detected when the patient was 3 years old were the diagnostic clues to the possible anomaly of the inner ear with a CSF fistula. The clinically proven CSF rhinorrhea contributed to the diagnosis of an ear anomaly with a fistula. Computerised axial tomography and magnetic resonance studies of the petrous portion of the temporal bone revealed the malformation that was later found and closed during the surgical intervention on the affected ear. The clinical absence of rhinorrhea, a year's progression without new infections after operating on the patient and post-surgery imaging studies were all proof that the fistula had closed. Mondini dysplasia with CSF fistula must be included as a possible diagnosis when faced with a patient with recurrent bacterial meningoencephalitis. Imaging studies, especially magnetic resonance, enable the clinician to check the diagnosis and the CSF fistula can be closed with ear surgery.

  16. In vivo Dentin Caries Model using Rat Molars

    OpenAIRE

    Takashi, HIGASHI; Junji, TAGAMI; Nobuhiro, HANADA; Cariology and Operative Dentistry, Department of Restorative Sciences, Tokyo Medical and Dental University; Cariology and Operative Dentistry, Department of Restorative Sciences, Tokyo Medical and Dental University; Department of Oral Science The National Institute of Infectious Diseases(NIID)

    2000-01-01

    Experimental dentinal caries was induced in rat molars which were inoculated orally with Streptococcus mutans and maintained on a carionenic diet 2000. After 30 days on the diet, the rats were sacrificed. The caries lesions were confirmed with a caries detector, then nanohardness determination of caries dentin were measured with nanoindentation. After hardness measurement, the lesion was examined by SEM and EDS. Dentin caries in sixteen fissures was induced among 20 fissures in the mandibular...

  17. Deformation Behavior of Human Dentin under Uniaxial Compression

    Directory of Open Access Journals (Sweden)

    Dmitry Zaytsev

    2012-01-01

    Full Text Available Deformation behavior of a human dentin under compression including size and rate effects is studied. No difference between mechanical properties of crown and root dentin is found. It is mechanically isotropic high elastic and strong hard tissue, which demonstrates considerable plasticity and ability to suppress a crack growth. Mechanical properties of dentin depend on a shape of samples and a deformation rate.

  18. Comprehensive proteomic analysis of human dentin

    Czech Academy of Sciences Publication Activity Database

    Jágr, Michal; Eckhardt, Adam; Pataridis, Statis; Mikšík, Ivan

    2012-01-01

    Roč. 120, č. 4 (2012), s. 259-268 ISSN 0909-8836 R&D Projects: GA ČR(CZ) GA203/08/1428; GA ČR(CZ) GAP206/12/0453 Institutional research plan: CEZ:AV0Z50110509 Institutional support: RVO:67985823 Keywords : dentin * mass spectrometry * proteomics * tooth * two-dimensional gel electrophoresis Subject RIV: CB - Analytical Chemistry, Separation Impact factor: 1.420, year: 2012

  19. A standardized imaging protocol for the endoscopic prediction of dysplasia within sessile serrated polyps (with video).

    Science.gov (United States)

    Tate, David J; Jayanna, Mahesh; Awadie, Halim; Desomer, Lobke; Lee, Ralph; Heitman, Steven J; Sidhu, Mayenaaz; Goodrick, Kathleen; Burgess, Nicholas G; Mahajan, Hema; McLeod, Duncan; Bourke, Michael J

    2018-01-01

    Dysplasia within sessile serrated polyps (SSPs) is difficult to detect and may be mistaken for an adenoma, risking incomplete resection of the background serrated tissue, and is strongly implicated in interval cancer after colonoscopy. The use of endoscopic imaging to detect dysplasia within SSPs has not been systematically studied. Consecutively detected SSPs ≥8 mm in size were evaluated by using a standardized imaging protocol at a tertiary-care endoscopy center over 3 years. Lesions suspected as SSPs were analyzed with high-definition white light then narrow-band imaging. A demarcated area with a neoplastic pit pattern (Kudo type III/IV, NICE type II) was sought among the serrated tissue. If this was detected, the lesion was labeled dysplastic (sessile serrated polyp with dysplasia); if not, it was labeled non-dysplastic (sessile serrated polyp without dysplasia). Histopathology was reviewed by 2 blinded specialist GI pathologists. A total of 141 SSPs were assessed in 83 patients. Median lesion size was 15.0 mm (interquartile range 10-20), and 54.6% were in the right side of the colon. Endoscopic evidence of dysplasia was detected in 36 of 141 (25.5%) SSPs; of these, 5 of 36 (13.9%) lacked dysplasia at histopathology. Two of 105 (1.9%) endoscopically designated non-dysplastic SSPs had dysplasia at histopathology. Endoscopic imaging, therefore, had an accuracy of 95.0% (95% confidence interval [CI], 90.1%-97.6%) and a negative predictive value of 98.1% (95% CI, 92.6%-99.7%) for detection of dysplasia within SSPs. Dysplasia within SSPs can be detected accurately by using a simple, broadly applicable endoscopic imaging protocol that allows complete resection. Independent validation of this protocol and its dissemination to the wider endoscopic community may have a significant impact on rates of interval cancer. (Clinical trial registration number: NCT03100552.). Copyright © 2018 American Society for Gastrointestinal Endoscopy. Published by Elsevier Inc. All

  20. Chemical treatment of the intra-canal dentin surface: a new approach to modify dentin hydrophobicity

    Directory of Open Access Journals (Sweden)

    Cesar GAITAN-FONSECA

    2013-01-01

    Full Text Available Objective This study evaluated the hydrophobicity of dentin surfaces that were modified through chemical silanization with octadecyltrichlorosilane (OTS. Material and Methods An in vitro experimental study was performed using 40 human permanent incisors that were divided into the following two groups: non-silanized and silanized. The specimens were pretreated and chemically modified with OTS. After the chemical modification, the dentin hydrophobicity was examined using a water contact angle measurement (WCA. The effectiveness of the modification of hydrophobicity was verified by the fluid permeability test (FPT. Results and Conclusions Statistically significant differences were found in the values of WCA and FPT between the two groups. After silanization, the hydrophobic intraradicular dentin surface exhibited in vitro properties that limit fluid penetration into the sealed root canal. This chemical treatment is a new approach for improving the sealing of the root canal system.

  1. Clinical efficacy of toothpaste containing potassium citrate in treating dentin hypersensitivity

    Directory of Open Access Journals (Sweden)

    Shih-Ya Shen

    2009-12-01

    Conclusion: The prevalence of dentin hypersensitivity in this study was 38%. The use of desensitizing toothpaste containing potassium citrate with oral hygiene instruction can effectively reduce dentin hypersensitivity.

  2. Efficacy of Modified Bioactive Glass for Dentin Remineralization and Obstruction of Dentinal Tubules

    Directory of Open Access Journals (Sweden)

    Mahshid Saffarpour

    2017-10-01

    Full Text Available Objectives: This study assessed the efficacy of modified bioactive glass (MBG for dentin remineralization and obstruction of dentinal tubules.Materials and Methods: Thirty-six dentin discs were made from 20 third molars and were stored in 12% lactic acid solution for two weeks to induce demineralization. The samples were divided into three groups (n=12: 1- BG, 2- BG modified with 5% strontium (Sr and 3- BG modified with 10% Sr. After applying the BG, the samples were stored in artificial saliva for 7, 14 and 21 days. Attenuated Total Reflection-Fourier Transform Infrared Spectroscopy (ATR-FTIR, X-ray Diffraction (XRD analysis, Scanning Electron Microscopy (SEM, and Energy-Dispersive X-ray (EDX analysis were used to assess remineralization. Also, 6 dentin discs were divided into three groups of BG, BG modified with 5% Sr and BG modified with 10% Sr, to examine tubular occlusion. The discs were etched using 0.5M of EDTA for two minutes and were stored in artificial saliva for 7 days. Changes in dentin surface morphology were evaluated under SEM.Results: Group 3 showed high rates of remineralization at days 7 and 14, although the rate decreased at day 21. Group 2 exhibited high rates of remineralization at days 7, 14 and 21. Dentinal tubules were partially occluded by BG and BG modified with 5% Sr, while they were almost completely obstructed after the use of BG modified with 10% Sr.Conclusions: Strontium increases remineralization. Addition of 10% Sr to BG enhances apatite formation; however, the apatite dissolves over time. Addition of 5% Sr to BG stabilizes the apatite lattice and increases the remineralization.

  3. Epileptogenicity of cortical dysplasia in temporal lobe dual pathology: an electrophysiological study with invasive recordings.

    Science.gov (United States)

    Fauser, Susanne; Schulze-Bonhage, Andreas

    2006-01-01

    Hippocampal sclerosis is often associated with macroscopic or microscopic dysplasia in the temporal neocortex (TN). The relevance of such a dual pathology with regard to epileptogenesis is unclear. This study investigates the role of both pathologies in the generation of ictal and interictal activity. Ictal (113 seizures) and interictal data from invasive EEG recordings with simultaneous depth electrodes in the hippocampus and subdural electrodes over the TN were analysed retrospectively in 12 patients with variable degrees of hippocampal sclerosis and different types of histologically confirmed temporal cortical dysplasia [all male, age at epilepsy onset 25 Hz) and repetitive sharp waves. The interictal patterns over the TN were similar to those seen over extratemporal focal cortical dysplasias. Simultaneous recordings from the hippocampus and the TN strongly suggest that dysplastic tissue in the TN is often epileptogenic. The quantitative contribution of the hippocampus to seizure generation corresponded with the degree of hippocampal pathology, whereas different subtypes of cortical dysplasia did not affect its relative contribution to seizure generation and even mild forms of dysplasia were epileptogenic.

  4. Anesthetic management during a cesarean section in a patient with cleidocranial dysplasia: a case report.

    Science.gov (United States)

    Nishio, Yumiko; Hiraki, Teruyuki; Taniguchi, Hiroko; Ushijima, Kazuo

    2018-01-01

    Cleidocranial dysplasia is a type of skeletal dysplasia, which is primarily characterized by delayed ossification of skeletal structures. It causes facial and oral abnormalities, resulting in difficult airway management and neuraxial anesthesia. The patient was a 24-year-old primipara (height 138 cm, weight 42 kg) with a hypoplastic right clavicle, patent fontanelles, dental malalignment, and a high palate. She was diagnosed with cleidocranial dysplasia at birth, although gene examination has not been performed. The fetus was confirmed to have short limbs and large fontanelles during an examination performed at 28 weeks gestation, suspected to have cleidocranial dysplasia. The mother was scheduled for a cesarean section at 37 weeks and 1 day due to cephalopelvic disproportion. Preoperative radiography and magnetic resonance imaging revealed no vertebral and spinal abnormalities, which allowed combined spinal-epidural analgesia (CSEA) to be performed. The surgery was safely concluded under CSEA with no intraoperative respiratory or circulatory problems. Patients with cleidocranial dysplasia exhibit facial, oral abnormalities, and often vertebral abnormalities. Imaging assessments before neuraxial anesthesia and careful preparation for airway management are required.

  5. Spondyloepiphyseal Dysplasia Tarda and Osteoporosis: A Case Report - Case Report

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    Şükran Kurtulmuş

    2006-03-01

    Full Text Available Spondyloepiphyseal dysplasia (SED tarda is a rare disease of which symptoms begin at childhood. It causes shortness of body and extremities, and kyphoscoliosis deformation due to delayed formation of epiphyses. Coexistence of osteopenia and the different types of this disease is also reported. According to our knowledge, the patient having both SED tarda and osteoporosis cured with the antiresorptive agents as well as his follow-up data are presented for the first time in the literature. (Osteoporoz Dünyasından 2006; 12 (1: 18-21

  6. Progressive pseudorheumatoid dysplasia in North and West Africa ...

    African Journals Online (AJOL)

    Progressive pseudorheumatoid dysplasia is a rare autosomal recessive spondyloepiphyseal dysplasia characterized by predominant involvement of articular cartilage with progressive joint stiffness and enlargement in the absence of inflammation. Short stature, joint contractures, gait disturbance, and scoliosis and/or ...

  7. Locally Aggressive Fibrous Dysplasia Mimicking Malign Calvarial Lesion.

    Science.gov (United States)

    Ogul, Hayri; Keskin, Emine

    2018-05-01

    Fibrous dysplasia is an unusual benign bone tumor. It is divided into 3 groups as monostotic, polyostotic, and craniofacial form. The authors reported an unusual patient with fibrous dysplasia with an aggressive radiologic appearance.

  8. Light-emitting diode assessment of dentinal defects: the role of presumed extraction forces

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    Marcelo Santos Coelho

    2017-07-01

    Full Text Available Objectives The evaluation of iatrogenic dentinal defects in extracted teeth may be influenced by extraction forces and prolonged dry times. The purpose of this study was to compare the presence of dentinal defects in freshly extracted, periodontally compromised teeth with those in a group of teeth with uncontrolled extraction forces and storage time. Materials and Methods The experimental group consisted of eighteen roots obtained from teeth extracted due to periodontal reasons with class II or III mobility. They were kept in saline and sectioned within 1 hour following extraction. The control group consisted of matched root types obtained from an anonymous tooth collection, consistent with previous dentinal defect studies. The slices were obtained at 3, 6, and 9 mm from the apex. The imaging process exposed all specimens to no more than 60 seconds of dry time. The × 12.8 magnification was used for the 9 mm slices and × 19.2 magnification for the 3 mm and 6 mm slices under light-emitting diode (LED transillumination. The root canal spaces and periodontal tissues were masked to minimize extraneous factors that might influence the evaluators. Chi-square test was used for statistical analysis. Results Dentinal defects were detected in 17% of the experimental group teeth, compared to 61% of control teeth (p = 0.015. Conclusions LED transillumination assessment of freshly extracted roots with class II or III mobility showed smaller number of dentinal defects than roots with uncontrolled storage time and extraction forces. The use of freshly extracted roots with mobility should be considered for future dental defect assessment studies.

  9. Epithelial dysplasia in oral lichen planus. A preliminary report of a Dutch-Hungarian study of 100 cases.

    Science.gov (United States)

    De Jong, W F; Albrecht, M; Bánóczy, J; van der Waal, I

    1984-06-01

    In a combined study of the Free University, Amsterdam and the Semmelweis Medical University, Budapest, the presence of epithelial dysplasia was studied in 100 cases of oral lichen planus. The criteria of epithelial dysplasia which were used in this study correspond with those reported by the WHO Collaborating Centre for Oral Precancerous Lesions in 1978. In approximately 25% of all cases, moderate or at least mild dysplasia was observed. The number of dysplastic changes per section did not show any significant correlation with the clinical type, nor with age or sex. There were no marked differences between the Amsterdam and Budapest material. Long-term data on the follow-up were not available yet. No comment can therefore be given about the meaning of the finding of epithelial dysplasia in lichen planus being a sign of premalignancy or not.

  10. Topical application of bFGF on acid-conditioned and non-conditioned dentin: effect on cell proliferation and gene expression in cells relevant for periodontal regeneration

    Directory of Open Access Journals (Sweden)

    Fernanda Regina Godoy Rocha

    Full Text Available Abstract Periodontal regeneration is still a challenge in terms of predictability and magnitude of effect. In this study we assess the biological effects of combining chemical root conditioning and biological mediators on three relevant cell types for periodontal regeneration. Material and Methods: Bovine dentin slices were conditioned with 25% citric acid followed by topical application of basic fibroblast growth factor (bFGF, 10 and 50 ng. We used ELISA to assess the dynamics of bFGF release from the dentin surface and RT-qPCR to study the expression of Runx2, Col1a1, Bglap and fibronectin by periodontal ligament (PDL fibroblasts, cementoblasts and bone marrow stromal cells (BMSC grown onto these dentin slices. We also assessed the effects of topical application of bFGF on cell proliferation by quantification of genomic DNA. Results: Acid conditioning significantly increased the release of bFGF from dentin slices. Overall, bFGF application significantly (p<0.05 increased cell proliferation, except for BMSC grown on non-conditioned dentin slices. Dentin substrate discretely increased expression of Col1a1 in all cell types. Expression of Runx2, Col1a1 and Fn was either unaffected or inhibited by bFGF application in all cell types. We could not detect expression of the target genes on BMSC grown onto conditioned dentin. Conclusion: Acid conditioning of dentin improves the release of topically-applied bFGF. Topical application of bFGF had a stimulatory effect on proliferation of PDL fibroblasts, cementoblasts and BMSC, but did not affect expression of Runx2, Col1a1, Bglap and fibronectin by these cells.

  11. Fibrous Dysplasia of the Temporal Bone with External Auditory Canal Stenosis and Secondary Cholesteatoma.

    Science.gov (United States)

    Liu, Yu-Hsi; Chang, Kuo-Ping

    2016-04-01

    Fibrous dysplasia is a slowly progressive benign fibro-osseous disease, rarely occurring in temporal bones. In these cases, most bony lesions developed from the bony part of the external auditory canals, causing otalgia, hearing impairment, otorrhea, and ear hygiene blockade and probably leading to secondary cholesteatoma. We presented the medical history of a 24-year-old woman with temporal monostotic fibrous dysplasia with secondary cholesteatoma. The initial presentation was unilateral conductive hearing loss. A hard external canal tumor contributing to canal stenosis and a near-absent tympanic membrane were found. Canaloplasty and type I tympanoplasty were performed, but the symptoms recurred after 5 years. She received canal wall down tympanomastoidectomy with ossciculoplasty at the second time, and secondary cholesteatoma in the middle ear was diagnosed. Fifteen years later, left otorrhea recurred again and transcanal endoscopic surgery was performed for middle ear clearance. Currently, revision surgeries provide a stable auditory condition, but her monostotic temporal fibrous dysplasia is still in place.

  12. Protein-losing enteropathy with intestinal lymphangiectasia in skeletal dysplasia with Lys650Met mutation.

    Science.gov (United States)

    Yang, Chen; Dehner, Louis P

    2016-11-01

    Protein-losing enteropathy is a primary or secondary manifestation of a group of conditions, and etiologies which are broadly divisible into those with mucosal injury on the basis of inflammatory and ulcerative conditions, mucosal injury without erosions or ulcerations, and lymphatic abnormalities. We describe the first case of protein-losing enteropathy in a pediatric patient, with severe skeletal dysplasia consistent with thanatophoric dysplasia type I and DNA analysis that revealed a c.1949A>T (p.Lys650Met) in exon 15 of the FGFR3 gene. She presented with protein-losing enteropathy in her 6th month. Post-mortem examination revealed lymphangiectasia in the small intestine. To our knowledge, this is the first report of intestinal lymphangiectasia as a complication of skeletal dysplasia resulting in severe protein-losing enteropathy. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  13. Hereditary ectodermal dysplasia: A retrospective study

    Science.gov (United States)

    More, Chandramani B.; Bhavsar, Khusbhu; Joshi, Jigar; Varma, Saurabh N.; Tailor, Mansi

    2013-01-01

    Background: Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat. Objective: To review and analyze cases of ED with an emphasis on clinical manifestations and parent's marriage history. Methodology: The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations. Results: It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offspring's affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offspring's affected with ED. The clinical manifestations observed were- dry skin(94.74%); scaly skin(42.11%); sparse hair on scalp, eyebrows and eyelashes(100%); frontal bossing(63.18%); saddle nose (57.89%); hypertelorism (47.37%); nail abnormality(52.63%); normal sweat glands(21.05%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19. Conclusion: ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients. PMID:24082749

  14. Hereditary ectodermal dysplasia: A retrospective study.

    Science.gov (United States)

    More, Chandramani B; Bhavsar, Khusbhu; Joshi, Jigar; Varma, Saurabh N; Tailor, Mansi

    2013-07-01

    Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat. To review and analyze cases of ED with an emphasis on clinical manifestations and parent's marriage history. The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations. It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offspring's affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offspring's affected with ED. The clinical manifestations observed were- dry skin(94.74%); scaly skin(42.11%); sparse hair on scalp, eyebrows and eyelashes(100%); frontal bossing(63.18%); saddle nose (57.89%); hypertelorism (47.37%); nail abnormality(52.63%); normal sweat glands(21.05%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19. ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients.

  15. Evaluation of Dentin Defect Formation during Retreatment with Hand and Rotary Instruments: A Micro-CT Study.

    Science.gov (United States)

    Yilmaz, Ayca; Helvacioglu-Yigit, Dilek; Gur, Cansu; Ersev, Handan; Kiziltas Sendur, Gullu; Avcu, Egemen; Baydemir, Canan; Abbott, Paul Vincent

    2017-01-01

    The purpose of this study was to compare the incidence and longitudinal propagation of dentin defects after gutta-percha removal with hand and rotary instruments using microcomputed tomography. Twenty mandibular incisors were prepared using the balanced-force technique and scanned in a 19.9  μ m resolution. Following filling with the lateral compaction technique, gutta-percha was removed with ProTaper Universal Retreatment (PTUR) or hand instruments. After rescanning, a total of 24,120 cross-sectional images were analyzed. The numbers, types, and longitudinal length changes of defects were recorded. Defects were observed in 36.90% of the cross sections. A total of 73 defects were comprised of 87.67% craze lines, 2.73% partial cracks, and 9.58% fractures. No significant difference in terms of new defect formation was detected between the retreatment groups. The apical and middle portions of the roots had more dentin defects than the coronal portions. Defects in three roots of the PTUR instrument group increased in length. Under the conditions of this in vitro study, gutta-percha removal seemed to not increase the incidence of dentin defect formation, but the longitudinal defect propagation finding suggests possible cumulative dentinal damage due to additional endodontic procedures. Hand and rotary instrumentation techniques caused similar dentin defect formation during root canal retreatment.

  16. Evaluation of Dentin Defect Formation during Retreatment with Hand and Rotary Instruments: A Micro-CT Study

    Directory of Open Access Journals (Sweden)

    Ayca Yilmaz

    2017-01-01

    Full Text Available The purpose of this study was to compare the incidence and longitudinal propagation of dentin defects after gutta-percha removal with hand and rotary instruments using microcomputed tomography. Twenty mandibular incisors were prepared using the balanced-force technique and scanned in a 19.9 μm resolution. Following filling with the lateral compaction technique, gutta-percha was removed with ProTaper Universal Retreatment (PTUR or hand instruments. After rescanning, a total of 24,120 cross-sectional images were analyzed. The numbers, types, and longitudinal length changes of defects were recorded. Defects were observed in 36.90% of the cross sections. A total of 73 defects were comprised of 87.67% craze lines, 2.73% partial cracks, and 9.58% fractures. No significant difference in terms of new defect formation was detected between the retreatment groups. The apical and middle portions of the roots had more dentin defects than the coronal portions. Defects in three roots of the PTUR instrument group increased in length. Under the conditions of this in vitro study, gutta-percha removal seemed to not increase the incidence of dentin defect formation, but the longitudinal defect propagation finding suggests possible cumulative dentinal damage due to additional endodontic procedures. Hand and rotary instrumentation techniques caused similar dentin defect formation during root canal retreatment.

  17. Strontium effects on root dentin tubule occlusion and nanomechanical properties.

    Science.gov (United States)

    Saeki, Kuniko; Marshall, Grayson W; Gansky, Stuart A; Parkinson, Charles R; Marshall, Sally J

    2016-02-01

    Dentin hypersensitivity often is treated by promotion of dentin tubule occlusion. In this in vitro study we evaluated nanomechanical properties and degree of tubule occlusion conferred to sound and demineralized human root dentin following treatment with a 10% (w/w) strontium acetate solution and its relation to the treatment duration and delivery method. 24 human cervical root dentin disks (8 groups of 3) were polished through 0.25 μm. 12 disks were subjected to an acid challenge (1% citric acid, pH 3.8) for 2 min. The specimens were incubated in artificial saliva, treated by soaking or brushing with deionized (DI) water or a solution of 10% strontium acetate for 2 min twice a day for 28 days. The occlusion percent and nanomechanical properties were determined at the baseline, 5, 14 and 28 days. Cross-sectioned specimens were prepared to evaluate the depth affected by strontium acetate / dentin interaction by SEM. Statistical analysis was performed using linear mixed effects models. A 10% strontium acetate treatment over 5-28 days significantly increased tubule occlusion for normal root dentin and to a lesser extent for demineralized dentin and increased the AFM based nanomechanical properties of demineralized dentin. Brushing was more effective than soaking in recovery of properties of demineralized dentin when treated with strontium. No difference in tubuleocclusion was found between the two delivery methods. Strontium acetate itself proved to have the ability to occlude dentin tubules and result in small changes in the mechanical properties of dentin. Copyright © 2015 Academy of Dental Materials. Published by Elsevier Ltd. All rights reserved.

  18. Ectodermal dysplasia associated with sickle cell disease.

    Science.gov (United States)

    Volpato, Luiz Evaristo Ricci; Volpato, Maria Carmen Palma Faria; de Carvalhosa, Artur Aburad; Palma, Vinicius Canavarros; Borges, Alvaro Henrique

    2014-01-01

    Ectodermal dysplasia and sickle cell anaemia are inherited disorders that affect, respectively, the tissues derived from the embryonic ectoderm and the production of erythrocytes by the bone marrow. The simultaneous occurrence of both disorders is extremely rare. This is a case of both ectodermal dysplasia and sickle cell anaemia reported in a 6-year-old. The patient had been diagnosed with sickle cell anaemia for only six months when he sought treatment presenting with the following: hypotrichosis, dry skin, periocular hyperpigmentation, protruding lips, hypodontia, and morphologically altered teeth. The clinical features combined with his medical history led to the diagnosis of ectodermal dysplasia. Dentists should be prepared to recognise patterns that escape normality to aid in the diagnosis of systemic changes, even in patients with other previous diagnoses.

  19. Ectodermal Dysplasia Associated with Sickle Cell Disease

    Directory of Open Access Journals (Sweden)

    Luiz Evaristo Ricci Volpato

    2014-01-01

    Full Text Available Ectodermal dysplasia and sickle cell anaemia are inherited disorders that affect, respectively, the tissues derived from the embryonic ectoderm and the production of erythrocytes by the bone marrow. The simultaneous occurrence of both disorders is extremely rare. This is a case of both ectodermal dysplasia and sickle cell anaemia reported in a 6-year-old. The patient had been diagnosed with sickle cell anaemia for only six months when he sought treatment presenting with the following: hypotrichosis, dry skin, periocular hyperpigmentation, protruding lips, hypodontia, and morphologically altered teeth. The clinical features combined with his medical history led to the diagnosis of ectodermal dysplasia. Dentists should be prepared to recognise patterns that escape normality to aid in the diagnosis of systemic changes, even in patients with other previous diagnoses.

  20. Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma

    International Nuclear Information System (INIS)

    Kahn, Leonard B.

    2003-01-01

    For just over 100 years, adamantinoma has been recognized as a primary bone tumor with epithelial characteristics and predominantly involving the tibia. Osteofibrous dysplasia is a fibro-osseous lesion also predominantly confined to the tibia with radiologic features similar to those of adamantinoma. This lesion has been shown by immunohistochemical studies to frequently contain cytokeratin-positive epithelial cells. More recently, a third group of cases with clinical and radiologic features similar to those of osteofibrous dysplasia have demonstrated more overt strands of epithelial cells within a fibro-osseous background and have been categorized as ''differentiated'', ''regressive'', ''juvenile intracortical'' or ''osteofibrous dysplasia-like'' adamantinoma. Cytokeratin subset immunohistochemical stains and cytogenetic studies performed in recent years suggest a common histogenesis for these three entities. This article reviews the clinical, radiologic and pathologic features of these entities as well as their prognostic significance. It also reviews the results of the immunohistochemical and cytogenetic studies which establish a common histogenetic relationship. (orig.)

  1. Variable manifestations of dysplasia epiphysealis hemimelica

    Energy Technology Data Exchange (ETDEWEB)

    Azouz, E.M.; Slomic, A.M.; Marton, D.; Rigault, P.; Finidori, G.

    1985-01-01

    Dysplasia epiphysealis hemimelica (DEH) is an osteocartilaginous overgrowth involving one or multiple epiphyses or ossification centers, usually in a lower extremity on one side of the body. Characteristically the involvement is hemimelic, i.e., either the medial or lateral part of the ossification center is involved. The authors have studied 24 patients with DEH and are adding 15 new cases to the literature. Because of the variable manifestations of the dysplasia and its different degrees of involvement in the affected children, they have subdivided it into localized, classical and generalized. In the generalized form, there is involvement of a whole lower extremity from the pelvis to the foot, and some of these patients show megaepiphyses with enlargement of a whole epiphyseal center, not only its medial or lateral part. The authors have also described and illustrated other special features of the dysplasia especially the advanced bone age and the metaphyseal and growth plate involvement.

  2. Scanning Electron Microscopic Hair Shaft Analysis in Ectodermal Dysplasia Syndromes.

    Science.gov (United States)

    Hirano-Ali, Stefanie A; Reed, Ashley M; Rowan, Brandon J; Sorrells, Timothy; Williams, Judith V; Pariser, David M; Hood, Antoinette F; Salkey, Kimberly

    2015-01-01

    The objective of the current study was to catalog hair shaft abnormalities in individuals with ectodermal dysplasia (ED) syndromes using scanning electron microscopy (SEM) and to compare the findings with those in unaffected controls. This is the second of a two-part study, the first of which used light microscopy as the modality and was previously published. Scanning electron microscopy was performed in a blinded manner on hair shafts from 65 subjects with seven types of ED syndromes and 41 unaffected control subjects. Assessment was performed along the length of the shaft and in cross section. Hair donations were collected at the 28th Annual National Family Conference held by the National Foundation for Ectodermal Dysplasia. Control subjects were recruited from a private dermatology practice and an academic children's hospital outpatient dermatology clinic. SEM identified various pathologic hair shaft abnormalities in each type of ED and in control patients. When hairs with all types of ED were grouped together and compared with those of control patients, the difference in the presence of small diameter and shallow and deep grooves was statistically significant (p < 0.05). When the EDs were separated according to subtype, statistically significant findings were also seen. SEM is a possible adjuvant tool in the diagnosis of ED syndromes. There are significant differences, with high specificity, between the hairs of individuals with ED and those of control subjects and between subtypes. © 2015 Wiley Periodicals, Inc.

  3. Multiple epiphyseal dysplasia: radiographic abnormalities correlated with genotype

    Energy Technology Data Exchange (ETDEWEB)

    Unger, S.L. [Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research Center, Los Angeles, CA (United States); Briggs, M.D.; Holden, P. [Wellcome Trust Centre for Cell-Matrix Research, University of Manchester (United Kingdom); Zabel, B. [Children' s Hospital, Univ. of Mainz (Germany); Ala-Kokko, L.; Paassilta, P.; Lohiniva, J. [Dept. of Medical Biochemistry, Univ. of Oulu (Finland); Rimoin, D.L. [Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research Center, Los Angeles, CA (United States); Department of Pediatrics, UCLA School of Medicine, Los Angeles, CA (United States); Lachman, R.S. [Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research Center, Los Angeles, CA (United States); Department of Pediatrics, UCLA School of Medicine, Los Angeles, CA (United States); Department of Radiology, UCLA School of Medicine, Los Angeles, CA (United States); Cohn, D.H. [Ahmanson Department of Pediatrics, Steven Spielberg Pediatric Research Center, Los Angeles, CA (United States); Department of Human Genetics, UCLA School of Medicine, Los Angeles, CA (United States)

    2001-01-01

    Multiple epiphyseal dysplasia (MED) is an osteochondrodysplasia characterized clinically by mild short stature and early-onset degenerative joint disease and radiographically by epiphyseal hypoplasia/dysplasia. MED is genetically heterogeneous, with autosomal dominant cases resulting from mutations in at least three genes: the cartilage oligomeric matrix protein (COMP) gene (EDM1) and the COL9A2 (EDM2) and COL9A3 (EDM3) genes of type IX procollagen. We present here a comparison of the radiographic phenotypes of MED patients with type IX collagen gene mutations and those with COMP gene mutations. We reviewed radiographs from two patients with MED produced by COMP mutations, two families with COL9A2 mutations, and one family with a mutation in COL9A3. The data demonstrated that the patients with type IX collagen defects had more severe joint involvement at the knees and relative hip sparing, while the patients with COMP mutations had significant involvement at the capital femoral epiphyses and irregular acetabuli. This pattern of joint involvement was consistent regardless of overall degree of severity of the phenotype. (orig.)

  4. Recessive mutations in PTHR1 cause contrasting skeletal dysplasias in Eiken and Blomstrand syndromes

    DEFF Research Database (Denmark)

    Duchatelet, Sabine; Ostergaard, Elsebet; Cortes, Dina

    2005-01-01

    Eiken syndrome is a rare autosomal recessive skeletal dysplasia. We identified a truncation mutation in the C-terminal cytoplasmic tail of the parathyroid hormone (PTH)/PTH-related peptide (PTHrP) type 1 receptor (PTHR1) gene as the cause of this syndrome. Eiken syndrome differs from Jansen...

  5. Thin and thick layers of resin-based sealer cement bonded to root dentine compared: Adhesive behaviour.

    Science.gov (United States)

    Pane, Epita S; Palamara, Joseph E A; Messer, Harold H

    2015-12-01

    This study aims to evaluate tensile and shear bond strengths of one epoxy (AH) and two methacrylate resin-based sealers (EZ and RS) in thin and thick layers bonded to root dentine. An alignment device was prepared for accurate positioning of 20 root dentine cylinders in a predefined gap of 0.1 or 1 mm. Sealer was placed in the interface. Bond strength tests were conducted. Mode of failures and representative surfaces were evaluated. Data were analysed using anova and post-hoc tests, with P thick layer of sealer produced higher bond strength, except for the shear bond strength of EZ. Significant differences between thin and thick layers were found only in tensile bond strengths of AH and RS. Mixed type of failure was constantly found with all sealers. Bond strengths of thick layers of resin-based sealers to root dentine tended to be higher than with thin layers. © 2015 Australian Society of Endodontology.

  6. Lung volume reduction surgery in bronchopulmonary dysplasia.

    Science.gov (United States)

    Siaplaouras, J; Heckmann, M; Reiss, I; Schaible, T; Waag, K L; Gortner, L

    2003-06-01

    We report on a female preterm infant of 29 wk gestational age, who developed acquired lobar emphysema after prolonged artificial ventilation secondary to respiratory disease syndrome and bronchopulmonary dysplasia. The infant underwent atypical segmentectomy at the age of 12 mo because of life-threatening hypoxaemia with pulmonary hypertension and failure of conservative treatment. Lung volume reduction surgery (LVRS) dramatically improved the respiratory function and resulted in adequate weight gain and psychomotor development. In selected cases LVRS can be an option for lobar emphysema in premature infants with severe bronchopulmonary dysplasia.

  7. Rasmussen's encephalitis presenting as focal cortical dysplasia

    Directory of Open Access Journals (Sweden)

    D.J. O'Rourke

    2014-01-01

    Full Text Available Rasmussen's encephalitis is a rare syndrome characterized by intractable seizures, often associated with epilepsia partialis continua and symptoms of progressive hemispheric dysfunction. Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention. Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management. We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD and discuss the literature on this topic.

  8. Rasmussen's encephalitis presenting as focal cortical dysplasia

    Science.gov (United States)

    O'Rourke, D.J.; Bergin, A.; Rotenberg, A.; Peters, J.; Gorman, M.; Poduri, A.; Cryan, J.; Lidov, H.; Madsen, J.; Harini, C.

    2014-01-01

    Rasmussen's encephalitis is a rare syndrome characterized by intractable seizures, often associated with epilepsia partialis continua and symptoms of progressive hemispheric dysfunction. Seizures are usually the hallmark of presentation, but antiepileptic drug treatment fails in most patients and is ineffective against epilepsia partialis continua, which often requires surgical intervention. Co-occurrence of focal cortical dysplasia has only rarely been described and may have implications regarding pathophysiology and management. We describe a rare case of dual pathology of Rasmussen's encephalitis presenting as a focal cortical dysplasia (FCD) and discuss the literature on this topic. PMID:25667877

  9. Sponastrime dysplasia. A radiologic-pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Lachman, R S; Stoss, H; Spranger, J

    1989-07-01

    The 2nd family with Sponastrime Dysplasia is described. The clinical, radiologic and chondro-osseous morphology of boy and girl siblings are presented. The facial appearance is an 'oriental look' with midface hypoplasia and a saddle nose. The radiological findings include the spinal changes of lordosis, osteoporosis and pear-shaped vertebrae, as well as striated metaphyses (osteopathia striata). The morphological findings suggest a disturbance in the formation of cartilage, with a defect in collagen and proteoglycans synthesis in this rare autosomal recessive skeletal dysplasia. (orig.).

  10. Sponastrime dysplasia. A radiologic-pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Lachman, R.S.; Stoss, H.; Spranger, J.

    1989-07-01

    The 2nd family with Sponastrime Dysplasia is described. The clinical, radiologic and chondro-osseous morphology of boy and girl siblings are presented. The facial appearance is an 'oriental look' with midface hypoplasia and a saddle nose. The radiological findings include the spinal changes of lordosis, osteoporosis and pear-shaped vertebrae, as well as striated metaphyses (osteopathia striata). The morphological findings suggest a disturbance in the formation of cartilage, with a defect in collagen and proteoglycans synthesis in this rare autosomal recessive skeletal dysplasia. (orig.).

  11. Developmental coxa vara associated with spondylometaphyseal dysplasia (DCV/SMD): ''SMD-Corner Fracture Type'' (DCV/SMD-CF) demonstrated in most reported cases

    Energy Technology Data Exchange (ETDEWEB)

    Currarino, G. [Texas Univ., Dallas, TX (United States). Dept. of Radiology; Department of Radiology, Texas Scottish Rite Hospital for Children, 2222 Welborn Street, Dallas, TX 75219 (United States); Birch, J.G.; Herring, J.A. [Department of Orthopedic Surgery, Texas Scottish Rite Hospital for Children, Dallas, TX (United States)

    2000-01-01

    Background. This paper reports three children with short stature: developmental coxa vara unilateral in the first case and bilateral in the other two; somewhat squared and ''ovoid'' vertebral bodies in the first patient, and normal to slightly tall vertebral bodies in the third; metaphyseal changes in some long tubular bones including bone fragments similar to the corner fractures seen in child abuse in all three patients. Materials and methods. The first and second patients were sisters; their mother, also quite short, had surgical procedures in early life for bilateral ''coxa vara''; their brother, also of short stature, had bilateral coxa valga with otherwise normal femoral heads and necks, and mild metaphyseal changes associated with two minute ''corner fractures'' in the proximal metaphysis of the left tibia. Results. A review of reported cases of developmental coxa vara associated with spondylometaphyseal dysplasia revealed that simulated corner fractures were present in most instances. (orig.)

  12. Polymerization contraction stress in dentin adhesives bonded to dentin and enamel

    NARCIS (Netherlands)

    Hashimoto, M.; de Gee, A.J.; Feilzer, A.J.

    2008-01-01

    Objective In a previous study on of polymerization contraction stress determinations of adhesives bonded to dentin a continuous decline of stress was observed after the adhesives had been light-cured. The decline was ascribed to stress relief caused by diffusion into the adhesive layer of water

  13. An unusual oral habit presenting as Dentin Hypersensitivity | Afolabi ...

    African Journals Online (AJOL)

    We present the case of a 30-year-old man with an unusual oral habit- office pin chewing and filing of the front tooth which resulted in dentine hypersensitivity. Clinical relevance: The role of daily oral habits and techniques of cessation were suggested in the management of dentine hypersensitivity. Keywords: Unusual oral ...

  14. Staining of dentin from amalgam corrosion is induced by demineralization

    NARCIS (Netherlands)

    Scholtanus, J.D.; van der Hoorn, W.; Özcan, M.; Huysmans, M.C.D.N.J.M.; Roeters, J.F.M.; Kleverlaan, C.J.; Feilzer, A.J.

    2013-01-01

    PURPOSE: To evaluate the effect of artificial demineralization upon color change of dentin in contact with dental amalgam. METHODS: Sound human molars (n = 34) were embedded in resin and coronal enamel was removed. Dentin was exposed to artificial caries gel (pH 5.5) at 37 degrees C for 12 weeks (n

  15. Staining of dentin from amalgam corrosion is induced by demineralization

    NARCIS (Netherlands)

    Scholtanus, Johannes D.; van der Hoorn, Wietske; Huysmans, Marie-Charlotte D. N. J. M.; Roeters, Joost F. M.; Kleverlaan, Cornelis J.; Feilzer, Albert J.; Ozcan, Mutlu

    Purpose: To evaluate the effect of artificial demineralization upon color change of dentin in contact with dental amalgam. Methods: Sound human molars (n= 34) were embedded in resin and coronal enamel was removed. Dentin was exposed to artificial caries gel (pH 5.5) at 37 degrees C for 12 weeks (n=

  16. The effect of propolis fluoride on caries dentine activity

    Science.gov (United States)

    Darwita, Risqa Rina; Soekanto, Sri Angky; Finisha, Andanali Rhukul; Wahyuni, Hardiati Nur; Andiani, Salsabila Ghina

    2018-02-01

    This research was conducted to analyze the effect of propolis fluoride on the occurrence of arrested caries dentine surface. The design of the study was a clinical trial experimental. The respondent were primary school students aged 6-8 years, from primary school in Kukusan, Depok, West Java, Indonesia. They were screened of dental examination and the total number of 296 children with an age range of 6-8 year old were included in the study. All students who had dentin caries were get dental treatment with propolis fluoride topical application in dentin caries surface. After one month, two months and four months all students who were got propolis fluoride topical application in dentin caries surface were controlled. All data were analyzed by Wilcoxon test. The total number of dentinal decay of 296 student was 1740 surfaces indicated to Flolis topical application. After one, two and three months of Flolis application, all arrested of dentinal decay was evaluated, the result of evaluation from the second month to the third month evaluation was found that the arrested dentinal caries surfaces were decreased significantly at 29.36% (pcaries surface from the first month to the third month was decreased significantly at 38.62% (pcaries, and their ability to arrest dental caries was significantly different. Flolis was found to be effective in arresting dentinal caries surface until three months application, and should be repeated after 3 months of Flolis application.

  17. Water evaporation from substrate tooth surface during dentin treatments.

    Science.gov (United States)

    Kusunoki, Mizuho; Itoh, Kazuo; Gokan, Yuka; Nagai, Yoshitaka; Tani, Chihiro; Hisamitsu, Hisashi

    2011-01-01

    The purpose of this study was to evaluate changes in the quantity of water evaporation from tooth surfaces. The amount of water evaporation was measured using Multi probe adapter MPA5 and Tewameter TM300 (Courage+Khazaka Electric GmbH, Köln, Germany) after acid etching and GM priming of enamel; and after EDTA conditioning and GM priming of dentin. The results indicated that the amount of water evaporation from the enamel surface was significantly less than that from the dentin. Acid etching did not affect the water evaporation from enamel, though GM priming significantly decreased the evaporation (83.48 ± 15.14% of that before priming). The evaporation from dentin was significantly increased by EDTA conditioning (131.38 ± 42.08% of that before conditioning) and significantly reduced by GM priming (80.26 ± 7.43% of that before priming). It was concluded that dentin priming reduced water evaporation from the dentin surface.

  18. Overexpression of Shox2 Leads to Congenital Dysplasia of the Temporomandibular Joint in Mice

    Directory of Open Access Journals (Sweden)

    Xihai Li

    2014-07-01

    Full Text Available Our previous study reported that inactivation of Shox2 led to dysplasia and ankylosis of the temporomandibular joint (TMJ, and that replacing Shox2 with human Shox partially rescued the phenotype with a prematurely worn out articular disc. However, the mechanisms of Shox2 activity in TMJ development remain to be elucidated. In this study, we investigated the molecular and cellular basis for the congenital dysplasia of TMJ in Wnt1-Cre; pMes-stop Shox2 mice. We found that condyle and glenoid fossa dysplasia occurs primarily in the second week after the birth. The dysplastic TMJ of Wnt1-Cre; pMes-stop Shox2 mice exhibits a loss of Collagen type I, Collagen type II, Ihh and Gli2. In situ zymography and immunohistochemistry further demonstrate an up-regulation of matrix metalloproteinases (MMPs, MMP9 and MMP13, accompanied by a significantly increased cell apoptosis. In addition, the cell proliferation and expressions of Sox9, Runx2 and Ihh are no different in the embryonic TMJ between the wild type and mutant mice. Our results show that overexpression of Shox2 leads to the loss of extracellular matrix and the increase of cell apoptosis in TMJ dysplasia by up-regulating MMPs and down-regulating the Ihh signaling pathway.

  19. Dentine bond strength and antimicrobial activity evaluation of adhesive systems.

    Science.gov (United States)

    André, Carolina Bosso; Gomes, Brenda Paula Figueiredo Almeida; Duque, Thais Mageste; Stipp, Rafael Nobrega; Chan, Daniel Chi Ngai; Ambrosano, Glaucia Maria Bovi; Giannini, Marcelo

    2015-04-01

    This study evaluated the dentine bond strength (BS) and the antibacterial activity (AA) of six adhesives against strict anaerobic and facultative bacteria. Three adhesives containing antibacterial components (Gluma 2Bond (glutaraldehyde)/G2B, Clearfil SE Protect (MDPB)/CSP and Peak Universal Bond (PUB)/chlorhexidine) and the same adhesive versions without antibacterial agents (Gluma Comfort Bond/GCB, Clearfil SE Bond/CSB and Peak LC Bond/PLB) were tested. The AA of adhesives and control groups was evaluated by direct contact method against four strict anaerobic and four facultative bacteria. After incubation, according to the appropriate periods of time for each microorganism, the time to kill microorganisms was measured. For BS, the adhesives were applied according to manufacturers' recommendations and teeth restored with composite. Teeth (n=10) were sectioned to obtain bonded beams specimens, which were tested after artificial saliva storage for one week and one year. BS data were analyzed using two-way ANOVA and Tukey test. Saliva storage for one year reduces the BS only for GCB. In general G2B and GCB required at least 24h for killing microorganisms. PUB and PLB killed only strict anaerobic microorganisms after 24h. For CSP the average time to eliminate the Streptococcus mutans and strict anaerobic oral pathogens was 30 min. CSB showed no AA against facultative bacteria, but had AA against some strict anaerobic microorganisms. Storage time had no effect on the BS for most of the adhesives. The time required to kill bacteria depended on the type of adhesive and never was less than 10 min. Most of the adhesives showed stable bond strength after one year and the Clearfil SE Protect may be a good alternative in restorative procedures performed on dentine, considering its adequate bond strength and better antibacterial activity. Copyright © 2015 Elsevier Ltd. All rights reserved.

  20. The Bone Dysplasia Ontology: integrating genotype and phenotype information in the skeletal dysplasia domain

    Directory of Open Access Journals (Sweden)

    Groza Tudor

    2012-03-01

    Full Text Available Abstract Background Skeletal dysplasias are a rare and heterogeneous group of genetic disorders affecting skeletal development. Patients with skeletal dysplasias suffer from many complex medical issues including degenerative joint disease and neurological complications. Because the data and expertise associated with this field is both sparse and disparate, significant benefits will potentially accrue from the availability of an ontology that provides a shared conceptualisation of the domain knowledge and enables data integration, cross-referencing and advanced reasoning across the relevant but distributed data sources. Results We introduce the design considerations and implementation details of the Bone Dysplasia Ontology. We also describe the different components of the ontology, including a comprehensive and formal representation of the skeletal dysplasia domain as well as the related genotypes and phenotypes. We then briefly describe SKELETOME, a community-driven knowledge curation platform that is underpinned by the Bone Dysplasia Ontology. SKELETOME enables domain experts to use, refine and extend and apply the ontology without any prior ontology engineering experience--to advance the body of knowledge in the skeletal dysplasia field. Conclusions The Bone Dysplasia Ontology represents the most comprehensive structured knowledge source for the skeletal dysplasias domain. It provides the means for integrating and annotating clinical and research data, not only at the generic domain knowledge level, but also at the level of individual patient case studies. It enables links between individual cases and publicly available genotype and phenotype resources based on a community-driven curation process that ensures a shared conceptualisation of the domain knowledge and its continuous incremental evolution.

  1. Cervical dentin hypersensitivity: a cross-sectional investigation in Athens, Greece.

    Science.gov (United States)

    Rahiotis, C; Polychronopoulou, A; Tsiklakis, K; Kakaboura, A

    2013-12-01

    The purpose of this study was to identify the prevalence of cervical dentin hypersensitivity in a cross-sectional investigation of Greek adults. Seven hundred and sixty-seven subjects were examined. Participants were patients processed for first examination in the Clinic of Oral Diagnosis and Radiology at the Faculty of Dentistry, University of Athens. The evaluation of hypersensitivity was performed using two methods: for each tooth, the response to a) tactile stimulus and b) air-blast stimulus was measured. Additional factors such as smoking habits, oral health behaviour, consumption of acidic foods, type of toothbrush, daily use of fluoride solution and of desensitising toothpaste, gingival recession and non-carious cervical lesions were recorded and evaluated as causative factors. Descriptive statistics on the demographics of the study sample, of oral health behaviour characteristics and of oral examination findings were performed. Comparisons of these characteristics in the presence or absence of hypersensitivity were conducted with the chi-square test. Data were further analysed using multiple logistic regression modelling. Among study participants, 21·3% had at least one cervical dentin hypersensitivity reaction to the tactile stimulus, and 38·6%, to the air-blast stimulus. Multivariate analysis detected association of the hypersensitivity in tactile or air-blast stimulus with the non-carious lesions and with the gingival recessions. Additionally, a relation between hypersensitivity and air-blast stimulus with gender (female) was found. There was no association between the hypersensitivity in both of the stimuli and the level of education, smoking, consumption of acidic foods, type of toothbrush and daily use of fluoride solution or desensitising toothpaste. The overall prevalence of cervical dentin hypersensitivity in the adult population in Athens ranged from 21·3% to 38·6% depending on the type of stimuli. Cervical non-carious lesions and gingival

  2. Changes in dimensions and weight of human dentine after different drying procedures and during subsequent rehydration

    NARCIS (Netherlands)

    Van der Graaf, E R; Ten Bosch, J J

    Shrinkage of dentine and the decrease in its weight after drying were measured. Three drying procedures were used, freeze-drying and drying in nitrogen at 60 and 100 degrees C. The dentine was subsequently rehydrated while measuring its dimensions. Rehydrated dentine was weighed. Dentine weight

  3. Simplified Classification of Focal Cortical Dysplasia

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2002-09-01

    Full Text Available Sections of cortex from 52 of 224 (23% patients with cortical dysplasia, operated on for drug-resistant partial epilepsy, were retrospectively re-examined histologically at Niguarda Hospital, and Istituto Nazionale Neurologico ‘C. Besta’, Milan, Italy.

  4. Ceramide profile in hypohidrotic ectodermal dysplasia

    DEFF Research Database (Denmark)

    Jungersted, J. M.; Høgh, Julie Kaae; Hellgren, Lars

    2012-01-01

    Background. Hypohidrotic ectodermal dysplasia (HED) is a rare genetic disease. The clinical presentation includes lack of sweating ability, and an often widely spread dermatitis resembling atopic dermatitis (AD). In AD, the skin‐barrier defect is partly ascribed to the altered lipid profile...

  5. Screening for Developmental Dysplasia of the Hip

    NARCIS (Netherlands)

    Boere-Boonekamp, Magdalena M.; Verkerk, Paul H.

    1998-01-01

    The success rates of screening programmes for Developmental Dysplasia of the Hip (DDH) vary widely. Studies on screening programmes for DDH based on a Medline search for the years 1966–1997 are reviewed. The percentage treated in most studies, especially those using ultrasound, are high and suggest

  6. Total hip reconstruction in acetabular dysplasia.

    NARCIS (Netherlands)

    Schller, H.M.; Dalstra, M.; Huiskes, R.; Marti, R.K.

    1993-01-01

    In acetabular dysplasia, fixation of the acetabular component of a cemented total hip prosthesis may be insecure and superolateral bone grafts are often used to augment the acetabular roof. We used finite element analysis to study the mechanical importance of the lateral acetabular roof and found

  7. The patellofemoral joint: from dysplasia to dislocation

    Science.gov (United States)

    Zaffagnini, Stefano; Grassi, Alberto; Zocco, Gianluca; Rosa, Michele Attilo; Signorelli, Cecilia; Muccioli, Giulio Maria Marcheggiani

    2017-01-01

    Patellofemoral dysplasia is a major predisposing factor for instability of the patellofemoral joint. However, there is no consensus as to whether patellofemoral dysplasia is genetic in origin, caused by imbalanced forces producing maltracking and remodelling of the trochlea during infancy and growth, or due to other unknown and unexplored factors. The biomechanical effects of patellofemoral dysplasia on patellar stability and on surgical procedures have not been fully investigated. Also, different anatomical and demographic risk factors have been suggested, in an attempt to identify the recurrent dislocators. Therefore, a comprehensive evaluation of all the radiographic, MRI and CT parameters can help the clinician to assess patients with primary and recurrent patellar dislocation and guide management. Patellofemoral dysplasia still represents an extremely challenging condition to manage. Its controversial aetiology and its complex biomechanical behaviour continue to pose more questions than answers to the research community, which reflects the lack of universally accepted guidelines for the correct treatment. However, due to the complexity of this condition, an extremely personalised approach should be reserved for each patient, in considering and addressing the anatomical abnormalities responsible for the symptoms. Cite this article: EFORT Open Rev 2017;2. DOI: 10.1302/2058-5241.2.160081. Originally published online at www.efortopenreviews.org PMID:28630757

  8. Cochlear implantation in a bilateral Mondini dysplasia.

    Science.gov (United States)

    Turrini, M; Orzan, E; Gabana, M; Genovese, E; Arslan, E; Fisch, U

    1997-01-01

    We report the speech perception progress and programming procedures of a case of congenital profound deafness and bilateral Mondini dysplasia implanted with a Nucleus 20 + 2 cochlear implant at the age of six. Unclear relations between electrodes array and cochlear partition made implant programming difficult and non-standard procedures were set. Cochlear implantation may give excellent rehabilitative results also in cochleae with malformation.

  9. A new lethal sclerosing bone dysplasia

    International Nuclear Information System (INIS)

    Kingston, H.M.; Freeman, J.S.; Hall, C.M.

    1991-01-01

    A neonate is described with a lethal sclerosing bone dysplasia associated with prenatal fractures and craniofacial abnormalities including microcephaly, exophthalmos, hypoplastic nose and mid-face, small jaw and nodular hyperplasia of the gums. Parental consanguinity suggests that an autosomal recessive mutation is the likely aetiology. (orig.)

  10. Ureaplasma urealyticum colonization, prematurity and bronchopulmonary dysplasia

    NARCIS (Netherlands)

    vanWaarde, WM; Brus, F; Okken, A; Kimpen, JLL

    The aim of the present study was to determine the association between the presence of Ureaplasma urealyticum in endotracheal aspirates and bronchopulmonary dysplasia (BPD). In addition, a review of similar studies from the English literature is presented. During the period February 1990 until March

  11. In vitro evaluation of the contact angle formed between AH Plus, Hybrid Root Seal and mineral trioxide aggregate Plus sealer with dentin and gutta-percha.

    Science.gov (United States)

    Nikhil, Vineeta; Jaiswal, Shikha; Bajpai, Gauravi

    2018-01-01

    The purpose of this study was evaluation and comparison of the contact angle of new root canal sealers - Hybrid Root Seal, mineral trioxide aggregate (MTA) Plus, and the conventional AH Plus sealer with dentin and gutta-percha. Two groups (Group D - dentin and Group G - gutta-percha) of 18 samples each were further randomly divided into 3 subgroups based on the type of sealer used, that is, AH Plus, Hybrid Root Seal, and MTA Plus. Contact angle measurement device (Phoenix 300) was used to measure the contact angle of the sealers on both dentin and gutta-percha. The results thus obtained were analyzed using one-way analysis of variance and Student's t -test. MTA Plus recorded significantly higher values of contact angle on both the substrates, that is, dentin and gutta-percha when compared to AH Plus and Hybrid root canal sealer. The lowest value of contact angle in gutta-percha and dentin was shown by Hybrid root canal sealer and AH Plus, respectively. Both AH Plus and Hybrid Root Seal exhibited lower contact angle values, and hence, better wettability on both dentin and gutta-percha as compared to MTA Plus.

  12. Effect of Er,Cr:YSGG laser on the microtensile bond strength of two different adhesives to the sound and caries-affected dentin.

    Science.gov (United States)

    Ergücü, Z; Celik, E U; Unlü, N; Türkün, M; Ozer, F

    2009-01-01

    This study examined the effect of Er,Cr:YSGG laser irradiation on the microtensile bond strength (microTBS) of a three-step etch-and-rinse and a two-step self-etch adhesive to sound and caries-affected dentin. Sixteen freshly extracted human molars with occlusal dentin caries were used. The caries lesion was removed by one of the following methods: conventional treatment with burs or Er,Cr:YSGG laser (Waterlase MD, Biolase). The adhesive systems (AdheSE, Ivoclar Vivadent and Scotchbond Multi Purpose, 3M ESPE) were applied to the entire tooth surface according to the manufacturers' instructions. Resin composites were applied to the adhesive-treated dentin surfaces and light-cured. Each tooth was sectioned into multiple beams with the "non-trimming" version of the microtensile test. The specimens were subjected to microtensile forces (BISCO Microtensile Tester, BISCO). The data was analyzed by three-way ANOVA and independent t-tests (p=0.05). Er,Cr:YSGG laser irradiation exhibited similar microTBS values compared to that of conventional bur treatment, regardless of the adhesive system and type of treated dentin. The self-etch system revealed lower microTBS values, both with conventional and laser treatment techniques, compared to the etch-and-rinse adhesive in sound and caries-affected dentin (padhesive systems to sound and caries-affected dentin.

  13. The shear bond strength of self-adhesive resin cements to dentin and enamel: an in vitro study.

    Science.gov (United States)

    Rodrigues, Raphaela F; Ramos, Carla M; Francisconi, Paulo A S; Borges, Ana Flávia S

    2015-03-01

    Clinicians continue to search for ways to simplify bonding procedures without compromising clinical efficacy. The purpose of this study was to evaluate the shear strength of self-adhesive cements RelyX U100 and RelyX U200, and conventional resin cement RelyX ARC to enamel and dentin after different surface treatments. The crowns of 120 bovine incisor teeth were separated from the roots and embedded in epoxy resin in polyvinyl chloride tubes. In each tooth, the area to be cemented was delimited with central holed adhesive tape. The teeth were distributed into 12 groups (n=10) according to the substrate; etched or not with 37% phosphoric acid; and cement type of enamel-U100, enamel-phosphoric acid-U100, enamel-U200, enamel-phosphoric acid-U200, enamel-ARC, enamel-phosphoric acid-ARC, dentin-U100, dentin-phosphoric acid-U100, dentin-U200, dentin-phosphoric acid-U200, dentin-ARC, and dentin-phosphoric acid-ARC. After 7 days of storage in artificial saliva, shear strength tests were performed by using a universal testing machine (0.5 mm/min). The data were analyzed with 3-way ANOVA and the Tukey test (α=.05). Fracture analysis was performed with a light microscope. Two specimens from each group were analyzed with a scanning electron microscope. In enamel, ARC (9.96 MPa) had higher shear strength (P=.038) than U100 (5.14 MPa); however, after surface etching, U100 (17.81 MPa) and U200 (17.52 MPa) had higher shear strength (Padhesive type. U200 self-adhesive cement had similar bond strength to the ARC in enamel, but the combination with phosphoric acid had the best bond strength. For dentin, self-adhesive resin cements are equally effective alternatives to conventional resin cement. Copyright © 2015 Editorial Council for the Journal of Prosthetic Dentistry. Published by Elsevier Inc. All rights reserved.

  14. Influence of light energy density on heat generation during photoactivation of dental composites with different dentin and composite thickness

    Directory of Open Access Journals (Sweden)

    Ricardo Danil Guiraldo

    2009-08-01

    Full Text Available OBJECTIVE: The aim of this study was to determine the influence of different energy densities on the heat generated during photoactivation of Filtek Z250 (3M/ESPE and Z100 (3M/ESPE composite resins with different dentin and composite thickness. MATERIAL AND METHODS: The temperature increase was registered with a type-K thermocouple connected to a digital thermometer (Iopetherm 46. A chemically polymerized acrylic resin base was prepared to serve as a guide for the thermocouple and as a support for 0.5-, 1.0-, and 1.5-mm-thick bovine dentin discs. Circular elastomer molds (1.0 mm-height x 3.0-mm diameter or 2.0-mm height x 3.0-mm diameter were adapted on the acrylic resin base to standardize the composite resin thickness. A conventional halogen light-curing unit (XL 2500, 3M/ESPE was used with light intensity of 700 mW/cm². Energy density was calculated by the light intensity applied during a certain time with values of 28 J/cm² for Z100 and 14 J/cm² for Filtek Z250. The temperature change data were subjected to three-way ANOVA and Tukey's test at 5% level. RESULTS: The higher energy density (Z100 promoted greater temperature increase (p<0.05 than the lower energy density (Filtek Z250. For both composites and all composite thicknesses, the lowest dentin thickness (0.5 mm yielded significantly higher (p<0.05 temperature increase than the other two dentin thicknesses. The 1-mm-thick composite resin layer yielded significantly higher (p<0.05 temperature changes for both composites and all dentin thicknesses. CONCLUSIONS: Temperature increase was influenced by higher energy density and dentin/composite thickness.

  15. Three-dimensional computed tomography analysis of non-osteoarthritic adult acetabular dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Ito, Hiroshi; Matsuno, Takeo; Hirayama, Teruhisa; Tanino, Hiromasa; Yamanaka, Yasuhiro [Asahikawa Medical College, Department of Orthopaedic Surgery, Asahikawa (Japan); Minami, Akio [Hokkaido University School of Medicine, Department of Orthopaedic Surgery, Sapporo (Japan)

    2009-02-15

    Little data exists on the original morphology of acetabular dysplasia obtained from patients without radiographic advanced osteoarthritic changes. The aim of this study was to investigate the distribution and degree of acetabular dysplasia in a large number of patients showing no advanced degenerative changes using three-dimensional computed tomography (3DCT). Eighty-four dysplastic hips in 55 consecutive patients were studied. All 84 hips were in pre- or early osteoarthritis without radiographic evidence of joint space narrowing, formation of osteophytes or cysts, or deformity of femoral heads. The mean age at the time of CT scan was 35 years (range 15-64 years). 3D images were reconstructed and analyzed using recent computer imaging software (INTAGE Realia and Volume Player). Deficiency types and degrees of acetabular dysplasia were precisely evaluated using these computer software. The average Harris hip score at CT scans was 82 points. Twenty-two hips (26%) were classified as anterior deficiency, 17 hips (20%) as posterior deficiency, and 45 hips (54%) as lateral deficiency. No significant difference was found in the Harris hip score among these groups. The analysis of various measurements indicated wide variations. There was a significant correlation between the Harris hip score and the acetabular coverage (p < 0.001). Our results indicated wide variety of deficiency type and degree of acetabular dysplasia. Hips with greater acetabular coverage tended to have a higher Harris hip score. (orig.)

  16. Three-dimensional computed tomography analysis of non-osteoarthritic adult acetabular dysplasia

    International Nuclear Information System (INIS)

    Ito, Hiroshi; Matsuno, Takeo; Hirayama, Teruhisa; Tanino, Hiromasa; Yamanaka, Yasuhiro; Minami, Akio

    2009-01-01

    Little data exists on the original morphology of acetabular dysplasia obtained from patients without radiographic advanced osteoarthritic changes. The aim of this study was to investigate the distribution and degree of acetabular dysplasia in a large number of patients showing no advanced degenerative changes using three-dimensional computed tomography (3DCT). Eighty-four dysplastic hips in 55 consecutive patients were studied. All 84 hips were in pre- or early osteoarthritis without radiographic evidence of joint space narrowing, formation of osteophytes or cysts, or deformity of femoral heads. The mean age at the time of CT scan was 35 years (range 15-64 years). 3D images were reconstructed and analyzed using recent computer imaging software (INTAGE Realia and Volume Player). Deficiency types and degrees of acetabular dysplasia were precisely evaluated using these computer software. The average Harris hip score at CT scans was 82 points. Twenty-two hips (26%) were classified as anterior deficiency, 17 hips (20%) as posterior deficiency, and 45 hips (54%) as lateral deficiency. No significant difference was found in the Harris hip score among these groups. The analysis of various measurements indicated wide variations. There was a significant correlation between the Harris hip score and the acetabular coverage (p < 0.001). Our results indicated wide variety of deficiency type and degree of acetabular dysplasia. Hips with greater acetabular coverage tended to have a higher Harris hip score. (orig.)

  17. Effect of Adhesive Cementation Strategies on the Bonding of Y-TZP to Human Dentin.

    Science.gov (United States)

    Alves, Mll; Campos, F; Bergoli, C D; Bottino, M A; Özcan, M; Souza, Roa

    2016-01-01

    This study evaluated the effects of different adhesive strategies on the adhesion of zirconia to dentin using conventional and self-adhesive cements and their corresponding adhesive resins. The occlusal parts of human molars (N=80) were sectioned, exposing the dentin. The teeth and zirconia cylinders (N=80) (diameter=3.4 mm; height=4 mm) were randomly divided into eight groups according to the factors "surface conditioning" and "cement type" (n=10 per group). One conventional cement (CC: RelyX ARC, 3M ESPE) and one self-adhesive cement (SA: RelyX U200, 3M ESPE) and their corresponding adhesive resin (for CC, Adper Single Bond Plus; for SA, Scotchbond Universal Adhesive-SU) were applied on dentin. Zirconia specimens were conditioned either using chairside (CJ: CoJet, 30 μm, 2.5 bar, four seconds), laboratory silica coating (RC: Rocatec, 110 μm, 2.5 bar, four seconds), or universal primer (Single Bond Universal-UP). Nonconditioned groups for both cements acted as the control (C). Specimens were stored in water (37°C, 30 days) and subjected to shear bond strength (SBS) testing (1 mm/min). Data (MPa) were analyzed using two-way analysis of variance and a Tukey test (α=0.05). While surface conditioning significantly affected the SBS values (p=0.0001) (Cadhesive. Air-abrasion and the use of the universal primer improved the bond strength of zirconia to dentin compared to the control group, regardless of the type of resin cement used.

  18. Carbodiimide Inactivation of MMPs and Effect on Dentin Bonding

    Science.gov (United States)

    Mazzoni, A.; Apolonio, F.M.; Saboia, V.P.A.; Santi, S.; Angeloni, V.; Checchi, V.; Curci, R.; Di Lenarda, R.; Tay, F.R.; Pashley, D.H.; Breschi, L.

    2014-01-01

    The use of protein cross-linking agents during bonding procedures has been recently proposed to improve bond durability. This study aimed to use zymography and in situ zymography techniques to evaluate the ability of 1-ethyl-3-(3-dimethylaminopropyl) carbodiimide (EDC) cross-linker to inhibit matrix metalloproteinase (MMP) activity. The hypotheses tested were that: (1) bonding procedures increase dentin gelatinolytic activity and (2) EDC pre-treatment prevents this enzymatic activity. The zymographic assay was performed on protein extracts obtained from dentin powder treated with Optibond FL or Scotchbond 1XT with or without 0.3M EDC pre-treatment. For in situ zymography, adhesive/dentin interfaces were created with the same adhesives applied to acid-etched dentin slabs pre-treated or not with EDC conditioner. Zymograms revealed increased expression of dentin endogenous MMP-2 and -9 after adhesive application, while the use of EDC as a primer inactivated dentin gelatinases. Results of in situ zymograpy showed that hybrid layers of tested adhesives exhibited intense collagenolytic activity, while almost no fluorescence signal was detected when specimens were pre-treated with EDC. The correlative analysis used in this study demonstrated that EDC could contribute to inactivate endogenous dentin MMPs within the hybrid layer created by etch-and-rinse adhesives. PMID:24334409

  19. Dentin biomodification: strategies, renewable resources and clinical applications.

    Science.gov (United States)

    Bedran-Russo, Ana K; Pauli, Guido F; Chen, Shao-Nong; McAlpine, James; Castellan, Carina S; Phansalkar, Rasika S; Aguiar, Thaiane R; Vidal, Cristina M P; Napotilano, José G; Nam, Joo-Won; Leme, Ariene A

    2014-01-01

    The biomodification of dentin is a biomimetic approach, mediated by bioactive agents, to enhance and reinforce the dentin by locally altering the biochemistry and biomechanical properties. This review provides an overview of key dentin matrix components, targeting effects of biomodification strategies, the chemistry of renewable natural sources, and current research on their potential clinical applications. The PubMed database and collected literature were used as a resource for peer-reviewed articles to highlight the topics of dentin hierarchical structure, biomodification agents, and laboratorial investigations of their clinical applications. In addition, new data is presented on laboratorial methods for the standardization of proanthocyanidin-rich preparations as a renewable source of plant-derived biomodification agents. Biomodification agents can be categorized as physical methods and chemical agents. Synthetic and naturally occurring chemical strategies present distinctive mechanism of interaction with the tissue. Initially thought to be driven only by inter- or intra-molecular collagen induced non-enzymatic cross-linking, multiple interactions with other dentin components are fundamental for the long-term biomechanics and biostability of the tissue. Oligomeric proanthocyanidins show promising bioactivity, and their chemical complexity requires systematic evaluation of the active compounds to produce a fully standardized intervention material from renewable resource, prior to their detailed clinical evaluation. Understanding the hierarchical structure of dentin and the targeting effect of the bioactive compounds will establish their use in both dentin-biomaterials interface and caries management. Copyright © 2013 Academy of Dental Materials. Published by Elsevier Ltd. All rights reserved.

  20. Remineralizing Efficacy of Fluorohydroxyapatite Gel on Artificial Dentinal Caries Lesion

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    Qianqian Wang

    2015-01-01

    Full Text Available The aim was to evaluate the remineralizing efficacy of fluorohydroxyapatite (FHA gel on artificial dentinal caries lesion in vitro. Artificial carious lesions were made on occlusal cavities of teeth by exposing the dentin surface to a demineralizing solution. Each cavity was capped with a 3 mm thick FHA gel for 4 weeks. After the FHA gel was removed, the surface morphology and structure of the dentin were characterized by scanning electron microscopy (SEM, energy-dispersive X-ray spectroscopy (EDX, X-ray diffraction (XRD, and Fourier transform infrared spectroscopy (FT-IR. The dentin mineral density (DMD was measured by micro-computed tomography (Micro-CT. A layer of dense and orderly hexagonal crystal structure, with average diameter of 1 μm and thickness of 4~5 μm, could be observed on dentin surface. These crystals exhibited elemental peaks for calcium, phosphorus, carbon, and oxygen and characteristic peaks of hydroxyapatite (HA and fluorapatite (FA via XRD and FT-IR. The DMD of dentin surface layer significantly increased after it was capped with FHA gel (P<0.05. In the present study, the FHA gel could rapidly construct apatite on the artificial dentin caries surface and significantly increase the mineral density, which suggests that FHA gel might be a proper IPT material with remineralizing function.

  1. Association between Severity of Tooth Wear and Dentinal Hypersensitivity

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    Ashok Ayer

    2016-11-01

    Full Text Available Background & Objectives: Tooth wear (attrition, abrasion, erosion, and abfraction is perceived globally as ever increasing problem. Several outcome of the tooth wear are hypersensitivity, esthetic problems, functional impairment, annoyance to the patient, and fracture of the tooth. Among these, the measurable and more commonly reported outcome is hypersensitivity to stimuli. Although dentin hypersensitivity is a common clinical condition and is generally reported by the patient after experiencing a sharp, short pain caused by one of the several different external stimuli, it is often inadequately understood. None of the scientific literature available till date attempted to establish the relationship between tooth wear and dentin hypersensitivity which could be a key factor in monitoring those patients.  The aim of the study was to estimate the association between severity of teeth wear and sensitivity in the patients with reported dentinal hypersensitivity.Materials & Methods: Fifty patients with dentin hypersensitivity were investigated for tooth wear. Tooth wear measured using exact tooth wear index and level of sensitivity to stimuli was recorded using a numerical rating scale. Results: Enamel wear at cervical region of teeth showed a positive correlation (p=.010, similarly, dentin wear at cervical region of teeth showed positive correlation and significant association (p<.001 with dentinal hypersensitivity.Conclusion: The observation supports a significant association between severities of tooth surface wear and dentinal hypersensitivity.

  2. A micromechanics model of the elastic properties of human dentine

    Energy Technology Data Exchange (ETDEWEB)

    Kinney, J. H. [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States); Balooch, M. [Lawrence Livermore National Lab. (LLNL), Livermore, CA (United States); Marshall, G. W. [Univ. of California, San Francisco, CA (United States). Dept. of Restorative Dentistry; Marshall, S. J. [Univ. of California, San Francisco, CA (United States). Dept. of Restorative Dentistry

    1999-10-01

    A generalized self-consistent model of cylindrical inclusions in a homogeneous and isotropic matrix phase was used to study the effects of tubule orientation on the elastic properties of dentin. Closed form expressions for the five independent elastic constants of dentin were derived in terms of tubule concentration, and the Young's moduli and Poisson ratios of peri- and intertubular dentin. An atomic force microscope (AFM) indentation technique determined the Young's moduli of the peri- and intertubular dentin as approximately 30 GPa and 15 GPa, respectively. Over the natural variation in tubule density found in dentin, there was only a slight variation in the axial and transverse shear moduli with position in the tooth, and there was no measurable effect of tubule orientation. We conclude that tubule orientation has no appreciable effect on the elastic behavior of normal dentin, and that the elastic properties of healthy dentin can be modeled as an isotropic continuum with a Young's modulus of approximately 16 GPa and a shear modulus of 6.2 GPa.

  3. Characterization of Dentine to Assess Bond Strength of Dental Composites

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    Saad Liaqat

    2015-04-01

    Full Text Available This study was performed to develop alternating dentine adhesion models that could help in the evaluation of a self-bonding dental composite. For this purpose dentine from human and ivory was characterized chemically and microscopically before and after acid etching using Raman and SEM. Mechanical properties of dentine were determined using 3 point bend test. Composite bonding to dentine, with and without use of acid pre-treatment and/or the adhesive, were assessed using a shear bond test. Furthermore, micro gap formation after restoration of 3 mm diameter cavities in dentine was assessed by SEM. Initial hydroxyapatite level in ivory was half that in human dentine. Surface hydroxyapatites decreased by approximately half with every 23 s of acid etch. The human dentine strength (56 MPa was approximately double that of ivory, while the modulus was almost comparable to that of ivory. With adhesive use, average shear bond strengths were 30 and 26 MPa with and without acid etching. With no adhesive, average bond strength was 6 MPa for conventional composites. This, however, increased to 14 MPa with a commercial flowable “self–bonding” composite or upon addition of low levels of an acidic monomer to the experimental composite. The acidic monomer additionally reduced micro-gap formation with the experimental composite. Improved bonding and mechanical properties should reduce composite failures due to recurrent caries or fracture respectively.

  4. Dietary adaptions in the ultrastructure of dinosaur dentine.

    Science.gov (United States)

    Brink, Kirstin S; Chen, Yu-Cheng; Wu, Ya-Na; Liu, Wei-Min; Shieh, Dar-Bin; Huang, Timothy D; Sun, Chi-Kuang; Reisz, Robert R

    2016-12-01

    Teeth are key to understanding the feeding ecology of both extant and extinct vertebrates. Recent studies have highlighted the previously unrecognized complexity of dinosaur dentitions and how specific tooth tissues and tooth shapes differ between taxa with different diets. However, it is unknown how the ultrastructure of these tooth tissues contributes to the differences in feeding style between taxa. In this study, we use third harmonic generation microscopy and scanning electron microscopy to examine the ultrastructure of the dentine in herbivorous and carnivorous dinosaurs to understand how the structure of this tissue contributes to the overall utility of the tooth. Morphometric analyses of dentinal tubule diameter, density and branching rates reveal a strong signal for dietary preferences, with herbivorous saurischian and ornithischian dinosaurs consistently having higher dentinal tubule density than their carnivorous relatives. We hypothesize that this relates to the hardness of the dentine, where herbivorous taxa have dentine that is more resistant to breakage and wear at the dentine-enamel junction than carnivorous taxa. This study advocates the detailed study of dentine and the use of advanced microscopy techniques to understand the evolution of dentition and feeding ecology in extinct vertebrates. © 2016 The Author(s).

  5. Adhesion of resin composite core materials to dentin.

    Science.gov (United States)

    O'Keefe, K L; Powers, J M

    2001-01-01

    This study determined (1) the effect of polymerization mode of resin composite core materials and dental adhesives on the bond strength to dentin, and (2) if dental adhesives perform as well to dentin etched with phosphoric acid as to dentin etched with self-etching primer. Human third molars were sectioned 2 mm from the highest pulp horn and polished. Three core materials (Fluorocore [dual cured], Core Paste [self-cured], and Clearfil Photo Core [light cured]) and two adhesives (Prime & Bond NT Dual Cure and Clearfil SE Bond [light cured]) were bonded to dentin using two dentin etching conditions. After storage, specimens were debonded in microtension and bond strengths were calculated. Scanning electron micrographs of representative bonding interfaces were analyzed. Analysis showed differences among core materials, adhesives, and etching conditions. Among core materials, dual-cured Fluorocore had the highest bond strengths. There were incompatibilities between self-cured Core Paste and Prime & Bond NT in both etched (0 MPa) and nonetched (3.0 MPa) dentin. Among adhesives, in most cases Clearfil SE Bond had higher bond strengths than Prime & Bond NT and bond strengths were higher to self-etched than to phosphoric acid-etched dentin. Scanning electron micrographs did not show a relationship between resin tags and bond strengths. There were incompatibilities between a self-cured core material and a dual-cured adhesive. All other combinations of core materials and adhesives produced strong in vitro bond strengths both in the self-etched and phosphoric acid-etched conditions.

  6. Dentinal tubules revealed with X-ray tensor tomography.

    Science.gov (United States)

    Jud, Christoph; Schaff, Florian; Zanette, Irene; Wolf, Johannes; Fehringer, Andreas; Pfeiffer, Franz

    2016-09-01

    Dentin is a mineralized material making up most of the tooth bulk. A system of microtubules, so called dentinal tubules, transverses it radially from the pulp chamber to the outside. This highly oriented structure leads to anisotropic mechanical properties directly connected to the tubules orientation and density: the ultimate tensile strength as well as the fracture toughness and the shear strength are largest perpendicular to dentinal tubules. Consequently, the fatigue strength depends on the direction of dentinal tubules, too. However, none of the existing techniques used to investigate teeth provide access to orientation and density of dentinal tubules for an entire specimen in a non-destructive way. In this paper, we measure a third molar human tooth both with conventional micro-CT and X-ray tensor tomography (XTT). While the achievable resolution in micro-CT is too low to directly resolve the dentinal tubules, we provide strong evidence that the direction and density of dentinal tubules can be indirectly measured by XTT, which exploits small-angle X-ray scattering to retrieve a 3D map of scattering tensors. We show that the mean directions of scattering structures correlate to the orientation of dentinal tubules and that the mean effective scattering strength provides an estimation of the relative density of dentinal tubules. Thus, this method could be applied to investigate the connection between tubule orientation and fatigue or tensile properties of teeth for a full sample without cutting one, non-representative peace of tooth out of the full sample. Copyright © 2016 The Academy of Dental Materials. All rights reserved.

  7. Adhesive sealing of dentin surfaces in vitro: A review

    Science.gov (United States)

    Abu-Nawareg, Manar M; Zidan, Ahmed Z; Zhou, Jianfeng; Agee, Kelli; Chiba, Ayaka; Tagami, Jungi; Pashley, David H

    2016-01-01

    Purpose The purpose of this review is to describe the evolution of the use of dental adhesives to form a tight seal of freshly prepared dentin to protect the pulp from bacterial products, during the time between crown preparation and final cementum of full crowns. The evolution of these “immediate dentin sealants” follows the evolution of dental adhesives, in general. That is, they began with multiple-step, etch-and-rinse adhesives, and then switched to the use of simplified adhesives. Methods Literature was reviewed for evidence that bacteria or bacterial products diffusing across dentin can irritate pulpal tissues before and after smear layer removal. Smear layers can be solubilized by plaque organisms within 7–10 days if they are directly exposed to oral fluids. It is likely that smear layers covered by temporary restorations may last more than one month. As long as smear layers remain in place, they can partially seal dentin. Thus, many in vitro studies evaluating the sealing ability of adhesive resins use smear layer-covered dentin as a reference condition. Surprisingly, many adhesives do not seal dentin as well as do smear layers. Results Both in vitro and in vivo studies show that resin-covered dentin allows dentinal fluid to cross polymerized resins. The use of simplified single bottle adhesives to seal dentin was a step backwards. Currently, most authorities use either 3-step adhesives such as Scotchbond Multi-Purposea or OptiBond FLb or two-step self-etching primer adhesives, such as Clearfil SEc, Unifil Bondd or AdheSEe, respectfully. PMID:26846037

  8. Use of poly (amidoamine dendrimer for dentinal tubule occlusion: a preliminary study.

    Directory of Open Access Journals (Sweden)

    Tianda Wang

    Full Text Available The occlusion of dentinal tubules is an effective method to alleviate the symptoms caused by dentin hypersensitivity, a significant health problem in dentistry and daily life. The in situ mineralization within dentinal tubules is a promising treatment for dentin hypersensitivity as it induces the formation of mineral on the sensitive regions and occludes the dentinal tubules. This study was carried out to evaluate the in vitro effect of a whole generation poly(amidoamine (PAMAM dendrimer (G3.0 on dentinal tubule occlusion by inducing mineralization within dentinal tubules. Dentin discs were treated with PAMAM dendrimers using two methods, followed by the in vitro characterization using Attenuated total reflection Fourier-transform infrared spectroscopy (ATR-FTIR, X-ray diffraction (XRD, Field emission scanning electron microscopy (FE-SEM and Energy-Dispersive X-ray Spectroscopy (EDS. These results showed that G3.0 PAMAM dendrimers coated on dentin surface and infiltrated in dentinal tubules could induce hydroxyapatite formation and resulted in effective dentinal tubule occlusion. Moreover, crosslinked PAMAM dendrimers could induce the remineralization of demineralized dentin and thus had the potential in dentinal tubule occlusion. In this in vitro study, dentinal tubules occlusion could be achieved by using PAMAM dendrimers. This could lead to the development of a new therapeutic technique for the treatment of dentin hypersensitivity.

  9. Dentin-bonded all-ceramic crowns: current status.

    Science.gov (United States)

    Burke, F J; Qualtrough, A J; Hale, R W

    1998-04-01

    Dentin-bonded all-ceramic crowns employ contemporary techniques to lute the crown to the tooth using a resin luting material and dentin-bonding system. The advantages of these crowns are that they provide good esthetics and fracture resistance and can be used in cases of substantial tooth loss. Their principal disadvantages are that the luting procedure is more time-consuming and that these crowns should not be used where margins are subgingival. Dentin-bonded all-ceramic crowns may be a useful addition to the dentist's armamentarium, but long-term clinical studies are needed to fully assess their performance.

  10. Influence of EDC on Dentin-Resin Shear Bond Strength and Demineralized Dentin Thermal Properties

    Directory of Open Access Journals (Sweden)

    Lin Tang

    2016-11-01

    Full Text Available This study aimed to evaluate the bonding strength and thermal properties of demineralized dentin with and without EDC treatment. Sound human molars were randomly divided into seven treatment groups (n = 20: control, 80% ethanol, and five EDC ethanol solutions (0.01–1.0 M. In each group, 16 samples were used for bond strength assessment and 4 samples were used for scanning electron microscopy (SEM analysis. A further 70 intact molars were used to obtain a fine demineralized dentin powder, treated with the same solutions and were evaluated the crosslink degree by ninhydrin test and denaturation temperature (Td by differential scanning calorimetry. EDC-treated specimens (<1.0 M had a higher bond strength, especially 0.3 and 0.5 M group, than the control counterpart. There was a significant drop in bond strength of 1.0 M EDC group. SEM revealed a homogeneous and regular interface under all treatments. EDC treatment significantly increased the demineralized dentin cross-link degree and Td compared with the control and ethanol treatments. The 0.3 and 0.5 M treatments showed the highest cross-link degree and Td. In terms of mechnical and theramal properties consideration, 0.3 and 0.5 M EDC solutions may be favorable for when applied with etch-and-rinse adhesives, but it is still needed further long-term study.

  11. Network-Based Method for Identifying Co- Regeneration Genes in Bone, Dentin, Nerve and Vessel Tissues.

    Science.gov (United States)

    Chen, Lei; Pan, Hongying; Zhang, Yu-Hang; Feng, Kaiyan; Kong, XiangYin; Huang, Tao; Cai, Yu-Dong

    2017-10-02

    Bone and dental diseases are serious public health problems. Most current clinical treatments for these diseases can produce side effects. Regeneration is a promising therapy for bone and dental diseases, yielding natural tissue recovery with few side effects. Because soft tissues inside the bone and dentin are densely populated with nerves and vessels, the study of bone and dentin regeneration should also consider the co-regeneration of nerves and vessels. In this study, a network-based method to identify co-regeneration genes for bone, dentin, nerve and vessel was constructed based on an extensive network of protein-protein interactions. Three procedures were applied in the network-based method. The first procedure, searching, sought the shortest paths connecting regeneration genes of one tissue type with regeneration genes of other tissues, thereby extracting possible co-regeneration genes. The second procedure, testing, employed a permutation test to evaluate whether possible genes were false discoveries; these genes were excluded by the testing procedure. The last procedure, screening, employed two rules, the betweenness ratio rule and interaction score rule, to select the most essential genes. A total of seventeen genes were inferred by the method, which were deemed to contribute to co-regeneration of at least two tissues. All these seventeen genes were extensively discussed to validate the utility of the method.

  12. New endodontic obturation systems and their interfacial bond strength with intraradicular dentine - ex vivo studies.

    Science.gov (United States)

    Pawińska, M; Kierklo, A; Tokajuk, G; Sidun, J

    2011-01-01

    To comparatively evaluate adhesive properties of selected root canal fillings through the measurement of the material-dentine interfacial bond strength. Fifty extracted single-rooted human teeth with one canal each were prepared using Hero instruments to size 30.04. Teeth were divided into four subgroups depending on the root canal filling material and the method of obturation: Resilon/Epiphany - a thermoplastic method (IA), Resilon/Epiphany - a matching single-point method (IB), gutta-percha/Roeko Seal Automix - a thermoplastic method (IIA) and gutta-percha/Roeko Seal Automix - a matching single-point method (IIB). The obturated roots were cut perpendicular to the long axis to create 1.7 mm thick slices. The bond strength was measured for each test slice with push -out testing machine. The highest push-out bond strength was registered in subgroup IB (3.98 ± 1.33 MPa). Significantly lower bond strength was observed in subgroups IA (0.50 ± 0.24 MPa), IIA (0.33 ± 0.18 MPa) and IIB (0.08 ± 0.03 MPa) (pmaterial-dentine interfacial bond strength values were observed between IA and IIA, IA and IIB, IIA and IIB subgroups (p > 0.05). The push-out bond strength of the material-dentine interface was dependent on the type of material used and the root canal filling technique. The R/E system exhibited better adhesion ability to intraradicular dentine than G/RSA. The highest bond strength was observed for Resilon/Epiphany introduced with the single-cone technique.

  13. Common clinical features of children with enlarged vestibular aqueduct and Mondini dysplasia.

    Science.gov (United States)

    Wu, Chen-Chi; Chen, Yuh-Shyang; Chen, Pei-Jer; Hsu, Chuan-Jen

    2005-01-01

    The purpose of the study was to investigate the etiological factors and the audiological data of different types of inner ear malformations, which the authors thought might be helpful in elucidating the inter-relation among malformations and shedding light on pathogenesis. Retrospective study from 1998 to 2002 at a tertiary care university hospital. One hundred sixty consecutive children with a total of 302 affected ears undergoing high-resolution computed tomography of the temporal bone for sensorineural hearing loss were enrolled. The image results were correlated with causes and origins, hearing loss patterns, hearing levels, and audiogram configurations. Inner ear malformation was present in 114 (38%) ears. The most common malformations were enlarged vestibular aqueduct, incomplete partition of cochlea (Mondini dysplasia), large vestibule, and semicircular canal dysplasia, presenting either as isolated finding or in combination. Eighty-four (74%) ears had abnormalities confined to these four malformations; only 30 (26%) ears showed other malformations. Patients with complex of enlarged vestibular aqueduct, Mondini dysplasia, large vestibule, and semicircular canal dysplasia (EMVS complex) demonstrated a significantly higher incidence of fluctuating hearing loss (93%) and a better hearing level compared with those with other malformations. Homogeneity in audiological features among these four malformations was also disclosed. The authors identified a distinct clinical entity, the EMVS complex, which is characterized by fluctuating hearing loss and a better hearing level. The authors proposed that malformations belonging to this complex result from a common pathogenetic mechanism.

  14. Diagnosis of ALG12-CDG by exome sequencing in a case of severe skeletal dysplasia

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    Chaya Murali

    2014-01-01

    Full Text Available Congenital Disorder of Glycosylation type Ig (ALG12-CDG is part of a group of autosomal recessive conditions caused by deficiency of proteins involved in the assembly of dolichol-oligosaccharides used for protein N-glycosylation. In ALG12-CDG, the enzyme affected is encoded by the ALG12 gene. Affected individuals present clinically with neurodevelopmental delay, growth retardation, immune deficiency, male genital hypoplasia, and cardiomyopathy. A total of six individuals have been reported in the literature. Here, we present an infant with rhizomelic short stature, talipes equinovarus, platyspondyly, and joint dislocations. The infant had marked under-ossification of the pubic bones. Exome sequencing was performed and two deletions, each resulting in a frameshift, were found in ALG12. A review of the literature revealed two infants with ALG12-CDG and a severe skeletal dysplasia, including under-ossification of cervical vertebrae, pubic bones, and knees; in addition to talipes equinovarus and rhizomelic short stature. The phenotype of the individual we describe resembles pseudodiastrophic dysplasia and we discuss similarities and differences between ALG12-CDG and pseudodiastrophic dysplasia. The differential diagnosis in selected undiagnosed skeletal dysplasias should include CDGs.

  15. Dental Erosion and Dentin Hypersensitivity among Adult Asthmatics and Non-asthmatics Hospital-based: A Preliminary Study.

    Science.gov (United States)

    Farag, Zahra Hassan Abdelaziz; Awooda, Elhadi Mohieldin

    2016-01-01

    Asthma is a chronic inflammatory condition affecting the airways leading to spasm and swelling of the airways. The medications taken for the treatment of asthma can result in dental erosion and dentin hypersensitivity. The aims of this study were to investigate the severity of dental erosion amongst adult asthmatics according to: gender, type and duration of medication taken and to compare dental erosion and dentin hypersensitivity between asthmatics and non-asthmatics. Comparative, cross-sectional hospital based study among 40 asthmatics (M=15 & F=25) and 40 non-asthmatics (M=18 & F=22) in the age range of 18-60 year selected purposefully from Al-Shaab Teaching Hospital in Khartoum city. The Basic Erosive Wear Index was used for dental erosion assessment. Dentine hypersensitivity was determined by giving ice cold water and rated using the Visual Analogue Scale. Chi-square and Student's t-test were used for statistical analysis with P value ≤.05. There was an association between severity of dental erosion and presence of asthma (P=0.03), where asthmatics had a higher degree of erosion (moderate and severe) and non-asthmatics a lower degree. No significant association was found between dental erosion and gender, type and duration of medication among asthmatics group. A statistically significant difference was revealed in the degree of dentin hypersensitivity (P=0.00) among asthmatics (35.13%) and non-asthmatics (14.13%). Asthmatic patients had a higher degree of dental erosion and dentin hypersensitivity compared to non-asthmatics. Among asthmatic patients there was no association between severity of dental erosion and gender, type and duration medication was taken for.

  16. Joint space width in dysplasia of the hip

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig; Søballe, K

    2005-01-01

    In a longitudinal case-control study, we followed 81 subjects with dysplasia of the hip and 136 control subjects without dysplasia for ten years assessing radiological evidence of degeneration of the hip at admission and follow-up. There were no cases of subluxation in the group with dysplasia....... Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia...... and controls in regard to age, body mass index or occupational exposure to daily repeated lifting at admission.We found no significant differences in the reduction of the joint space width at follow-up between subjects with dysplasia and the control subjects nor in self-reported pain in the hip...

  17. Effect of bur-cut dentin on bond strength using two all-in-one and one two-step adhesive systems.

    Science.gov (United States)

    Koase, Kaori; Inoue, Satoshi; Noda, Mamoru; Tanaka, Toru; Kawamoto, Chiharu; Takahashi, Akiko; Nakaoki, Yasuko; Sano, Hidehiko

    2004-01-01

    To compare the microtensile bond strength (MTBS) of two all-in-one adhesive systems and one experimental two-step self-etching adhesive system to two types of bur-cut dentin. Using one of the three adhesives, Xeno CF Bond (Xeno), Prompt L-Pop (PL), or the experimental two-step system ABF (ABF), resin composite was bonded to flat buccal and root dentin surfaces of eight extracted human premolars. These surfaces were produced using either regular-grit or superfine-grit diamond burs. After storage overnight in 37 degrees C water, the bonded specimens were sectioned into six or seven slices approximately 0.7 mm thick perpendicular to the bonded surface. They were then subjected to microtensile testing. The surfaces of the fractured specimens were observed microscopically to determine the failure mode. In addition, to observe the effect of conditioning, the two types of bur-cut dentin surfaces were conditioned with the adhesives, rinsed with acetone, and observed with SEM. When Xeno and PL were bonded to dentin cut with a regular-grit diamond bur, MTBS values were lower than to superfine bur-cut dentin, and failures occurred adhesively at the interface, whereas the experimental two-step adhesive showed no significant difference in microtensile bond strength between two differently cut surfaces. The all-in-one adhesives tested here improved bond strengths when bonded to superfine bur-cut dentin as a substrate, whereas the experimental two-step adhesive system showed unchanged bonding to both regular and superfine bur-cut dentin surfaces.

  18. Prosthodontic management of anhidrotic ectodermal dysplasia

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    Shilpy Gupta

    2011-01-01

    Full Text Available Ectodermal dysplasia is characterized by the absence or defects of two or more ectodermally derived structures. Anodontia or hypodontia is the most striking dental manifestation. In severe hypodontia, there is lack of alveolar development with consequent protrusion and eversion of the lips. Patients with anhidrotic forms suffer from heat intolerance due to lack of sweat glands and mild infections may lead to death in infancy from hyperthermia. A case of a 4-year-old child with anhidrotic ectodermal dysplasia with partial anodontia is presented. Dental, oral, and physical features were taken into consideration in diagnosis and treatment planning for this patient. Clinical management consisted of removable partial prosthesis in maxillary arch and complete denture prosthesis in mandibular arch. The main aim of the treatment was to improve psychological development and to promote better functioning of the stomatognathic system.

  19. Mutations in FLNB cause boomerang dysplasia.

    Science.gov (United States)

    Bicknell, L S; Morgan, T; Bonafé, L; Wessels, M W; Bialer, M G; Willems, P J; Cohn, D H; Krakow, D; Robertson, S P

    2005-07-01

    Boomerang dysplasia (BD) is a perinatal lethal osteochondrodysplasia, characterised by absence or underossification of the limb bones and vertebrae. The BD phenotype is similar to a group of disorders including atelosteogenesis I, atelosteogenesis III, and dominantly inherited Larsen syndrome that we have recently shown to be associated with mutations in FLNB, the gene encoding the actin binding cytoskeletal protein, filamin B. We report the identification of mutations in FLNB in two unrelated individuals with boomerang dysplasia. The resultant substitutions, L171R and S235P, lie within the calponin homology 2 region of the actin binding domain of filamin B and occur at sites that are evolutionarily well conserved. These findings expand the phenotypic spectrum resulting from mutations in FLNB and underline the central role this protein plays during skeletogenesis in humans.

  20. Arrhythmogenic right ventricular dysplasia: A case report

    Directory of Open Access Journals (Sweden)

    Tessa Negrín Valdés

    2015-10-01

    Full Text Available Arrhythmogenic right ventricular dysplasia is a heart muscle disease that predominantly affects the right ventricle, bringing about the replacement of normal myocardium with fatty or fibrofatty tissue and causing sudden death in young individuals. Ventricular tachycardia is an important clinical manifestation, although there are reports of right or global heart failure. The diagnosis is confirmed by echocardiography and magnetic resonance imaging. The case of a 65-year-old former smoker, with hypertension and ischemic heart disease, a history of effort syncope symptoms and proven non-sustained ventricular tachycardia, with morphology of left bundle branch block, is reported. Relevant diagnostic studies were performed, and echocardiographic elements which were compatible with arrhythmogenic right ventricular dysplasia were found. Therefore, an implantable cardioverter defibrillator was implanted, after which the patient has had a favorable outcome.

  1. MRA of fibromuscular dysplasia in cervical vessels

    International Nuclear Information System (INIS)

    Link, J.; Steffens, J.C.; Mueller-Huelsbeck, S.; Brossmann, J.; Heller, M.

    1996-01-01

    In 386 selective angiograms of cervical vessels fibromuscular dysplasia was revealed in 4 female patients in the age of 30-54 years. FMD was located in the carotid artery (n=5) and in the vertebral artery (n=2) with a total of 8 lesions. 6/8 of the lesions of the seven cervical vessels were located typically in the mid cervical portion of the vessels and 2/6 lesions were located in the atlas loop of the vertebral artery. 4 lesions showed moderate stenosis and 4 vessels showed only mild stenosis. These patterns which demonstrated the typical morphology of fibromuscular dysplasia with alternating irregular zones of widening and narrowing were evaluated well with MR angiography, the others were missed. (orig./MG) [de

  2. Influence of Immediate Dentin Sealing on the Shear Bond Strength of Pressed Ceramic Luted to Dentin with Self-Etch Resin Cement

    Directory of Open Access Journals (Sweden)

    Robert Dalby

    2012-01-01

    Full Text Available Objectives. To examine the effect of immediate dentin sealing (IDS, with dentin bonding agents (DBAs applied to freshly cut dentin, on the shear bond strength of etched pressed ceramic luted to dentin with RelyX Unicem (RXU cement. Method. Eighty extracted noncarious third molars were ground flat to expose the occlusal dentin surfaces. The teeth were randomly allocated to five groups (A to E of sixteen teeth each. Groups A to D were allocated a dentin bonding agent (Optibond FL, One Coat Bond, Single Bond, or Go! that was applied to the dentin surface to mimic the clinical procedure of IDS. These specimen groups then had etched glass ceramic discs (Authentic luted to the sealed dentin surface using RXU. Group E (control had etched glass ceramic discs luted to the dentin surface (without a dentin bonding agent using RXU following the manufacturer’s instructions. All specimens were stored for one week in distilled water at room temperature and then shear stressed at a constant cross-head speed of 1 mm per minute until failure. Statistical analysis was performed by ANOVA followed by post hoc Tukey HSD method (0.05 in the SBS between the test groups (A–D or the control (group E. Conclusion. IDS using the dentin bonding agents tested does not statistically (>0.05 affect the shear bond strength of etched pressed ceramic luted to dentin with RXU when compared to the control.

  3. Management of developmental dysplasia of the hip in less than 24 months old children

    Directory of Open Access Journals (Sweden)

    Mehmet Bulut

    2013-01-01

    Full Text Available Background: There is no consensus on the treatment of developmental dysplasia of the hip in children less than 24 months of age. The aim of this study was to present the results of open reduction and concomitant primary soft-tissue intervention in patients with developmental dysplasia of the hip in children less than 24 months of age. Materials and Methods: Sixty hips of 50 patients (4 male, 46 female with mean age of 14.62 ± 5.88 (range 5-24 months months with a mean followup of 40.00 ± 6.22 (range 24-58 months months were included. Twenty five right and 35 left hips (10 bilaterally involved were operated. Open reduction was performed using the medial approach in patients aged < 20 months (with Tönnis type II-III and IV hip dysplasias and for those aged 20-24 months with Tönnis type II and III hip dysplasias ( n = 47. However for 13 patients aged 20-24 months with Tönnis type IV hip dysplasias, anterior bikini incision was used. Results: Mean acetabular index was 41.03 ± 3.78° (range 34°-50° in the preoperative period and 22.98 ± 3.01° (range 15°-32° at the final visits. Mean center-edge angle at the final visits was 22.85 ± 3.35° (18°-32°. Based on Severin radiological classification, 29 (48.3% were type I (very good, 25 (41.7% were type II (good and 6 (10% were type III (fair hips. According to the McKay clinical classification, postoperatively the hips were evaluated as excellent ( n = 42; 70%, good ( n = 14; 23.3% and fair ( n = 4; 6.7%. Reduction of all hip dislocations was achieved. Additional pelvic osteotomies were performed in 14 (23.3% hips for continued acetabular dysplasia and recurrent subluxation. (Salter [ n = 12]/Pemberton [ n = 2] osteotomy was performed. Avascular necrosis (AVN developed in 7 (11.7% hips. Conclusion: In DDH only soft-tissue procedures are not enough, because of the high rate of the secondary surgery and AVN for all cases aged less than 24 months. Bone procedures may be necessary in the walking

  4. Optothermal transfer simulation in laser-irradiated human dentin.

    Science.gov (United States)

    Moriyama, Eduardo H; Zangaro, Renato A; Lobo, Paulo D C; Villaverde, Antonio Balbin; Pacheco, Marcos T; Watanabe, Ii-Sei; Vitkin, Alex

    2003-04-01

    Laser technology has been studied as a potential replacement to the conventional dental drill. However, to prevent pulpal cell damage, information related to the safety parameters using high-power lasers in oral mineralized tissues is needed. In this study, the heat distribution profiles at the surface and subsurface regions of human dentine samples irradiated with a Nd:YAG laser were simulated using Crank-Nicolson's finite difference method for different laser energies and pulse durations. Heat distribution throughout the dentin layer, from the external dentin surface to the pulp chamber wall, were calculated in each case, to investigate the details of pulsed laser-hard dental tissue interactions. The results showed that the final temperature at the pulp chamber wall and at the dentin surface are strongly dependent on the pulse duration, exposure time, and the energy contained in each pulse.

  5. Detection of dentin antigenic fractions by salivary immunoglobulin G ...

    African Journals Online (AJOL)

    SAM

    2014-05-28

    May 28, 2014 ... non-collagenous proteins (NCP) and signaling molecules ... periodontal tissues and by signals and cytokines production ... Dentin extract was stored at -80°C until use. .... An effective mechanism to prevent self-reactivity is the.

  6. Amalgam shear bond strength to dentin using different bonding agents.

    Science.gov (United States)

    Vargas, M A; Denehy, G E; Ratananakin, T

    1994-01-01

    This study evaluated the shear bond strength of amalgam to dentin using five different bonding agents: Amalgambond Plus, Optibond, Imperva Dual, All-Bond 2, and Clearfil Liner Bond. Flat dentin surfaces obtained by grinding the occlusal portion of 50 human third molars were used for this study. To contain the amalgam on the tooth surface, cylindrical plastic molds were placed on the dentin and secured with sticky wax. The bonding agents were then applied according to the manufacturers' instructions or light activated and Tytin amalgam was condensed into the plastic molds. The samples were thermocycled and shear bond strengths were determined using an Instron Universal Testing Machine. Analysis by one-way ANOVA indicated significant difference between the five groups (P < 0.05). The bond strength of amalgam to dentin was significantly higher with Amalgambond Plus using the High-Performance Additive than with the other four bonding agents.

  7. Comparison of removed dentin thickness with hand and rotary instruments

    Science.gov (United States)

    Shahriari, Shahriar; Abedi, Hasan; Hashemi, Mahdi; Jalalzadeh, Seyed Mohsen

    2009-01-01

    INTRODUCTION: The aim of this study was to evaluate the amount of dentine removed after canal preparation using stainless steel (SS) hand instruments or rotary ProFile instruments. MATERIALS AND METHODS: Thirty-six extracted human teeth with root canal curvatures less than 30º were embedded in clear polyester resin. The roots were cut horizontally at apical 2, 4 and 7 mm. Dentin thickness was measured at each section and the sections were accurately reassembled using a muffle. Root canals were randomly prepared by SS hand instruments or rotary ProFile instruments. Root sections were again separated, and the remaining dentin thickness was measured. Mann-Whitney U and t tests were performed for analytic comparison of the results. RESULTS: The thickness of removed dentin was significantly different between the two used methods (Pinstrumentation group (Protary instrumentation prepares root canals with a greater conservation of tooth structure. PMID:23940489

  8. Role of Alcohol on the Fracture Resistance of Dentin

    Energy Technology Data Exchange (ETDEWEB)

    Nalla, Ravi K.; Kinney, John H.; Tomsia, Antoni P.; Ritchie,Robert O.

    2006-05-01

    Healthy dentin, the mineralized tissue that makes up the bulk of the tooth, is naturally hydrated in vivo; however, it is known that various chemical reagents including acetone and ethanol can induce dehydration and thereby affect its properties. Here, we seek to investigate this in light of the effect alcohol can have on the mechanical properties of dentin, specifically by measuring the stiffness, strength and toughness of dentin in simulated body fluid and scotch whisky. Results indicate that chemical dehydration induced by the whisky has a significant beneficial effect on the elastic modulus, strength and fracture toughness of dentin. Although this makes teeth more resistant to fracture, the change in properties is fully reversible upon rehydration. This effect is considered to be associated with increased cross-linking of the collagen molecules from intermolecular hydrogen-bonding where water is replaced with weaker hydrogen-bond forming solvents such as alcohol.

  9. KDF1, encoding keratinocyte differentiation factor 1, is mutated in a multigenerational family with ectodermal dysplasia

    KAUST Repository

    Shamseldin, Hanan E.; Khalifa, Ola; Binamer, Yousef M.; Almutawa, Abdulmonem; Arold, Stefan T.; Zaidan, Hamad; Alkuraya, Fowzan S.

    2016-01-01

    Ectodermal dysplasia is a highly heterogeneous group of disorders that variably affect the derivatives of the ectoderm, primarily skin, hair, nails and teeth. TP63, itself mutated in ectodermal dysplasia, links many other ectodermal dysplasia

  10. MR imaging features of craniodiaphyseal dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    Marden, Franklin A. [Mallinckrodt Institute of Radiology, Washington University Medical Center, 510 South Kingshighway Blvd., MO 63110, St. Louis (United States); Department of Radiology, St. Louis Children' s Hospital, Children' s Place, MO 63110, St. Louis (United States); Wippold, Franz J. [Mallinckrodt Institute of Radiology, Washington University Medical Center, 510 South Kingshighway Blvd., MO 63110, St. Louis (United States); Department of Radiology, St. Louis Children' s Hospital, Children' s Place, MO 63110, St. Louis (United States); Department of Radiology/Nuclear Medicine, F. Edward Hebert School of Medicine, Uniformed Services University of the Health Sciences, MD 20814, Bethesda (United States)

    2004-02-01

    We report the magnetic resonance (MR) imaging findings in a 4-year-old girl with characteristic radiographic and computed tomography (CT) features of craniodiaphyseal dysplasia. MR imaging exquisitely depicted cranial nerve compression, small foramen magnum, hydrocephalus, and other intracranial complications of this syndrome. A syrinx of the cervical spinal cord was demonstrated. We suggest that MR imaging become a routine component of the evaluation of these patients. (orig.)

  11. Bilateral anophthalmia with septo-optic dysplasia

    OpenAIRE

    Jana, Manisha; Sharma, Sanjay

    2010-01-01

    Bilateral anophthalmia is a rare entity and association with septo-optic dysplasia is an even rare condition. The condition is characterized by absent eyeballs in the presence of eyelids, conjunctiva or lacrimal apparatus. Though anophthalmia can be diagnosed clinically, imaging plays a crucial role in delineating the associated anomalies. In addition, often clinical anophthalmia may prove to be severe microphthalmia on imaging. We describe the imaging findings in an infant with bilateral ano...

  12. Distinctive skeletal dysplasia in Cockayne syndrome

    International Nuclear Information System (INIS)

    Silengo, M.C.; Franceschini, P.; Bianco, R.; Biagioli, M.; Pastorin, L.; Vista, N.; Baldassar, A.; Benso, L.

    1986-01-01

    Cockayne syndrom is a well-known autosomal recessive form of dwarfism with senile-like appearance. Skeletal changes such as flattening of vertebral bodies, ivory epiphyses and thickening of cranial vault, have been observed in some patients with this condition. We describe here a 5.5-year-old girl with the typical clinical signs of Cockayne syndrome and a distinctive form of bone dysplasia with major involvment of the spine. (orig.)

  13. Distinctive skeletal dysplasia in Cockayne syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Silengo, M.C.; Franceschini, P.; Bianco, R.; Biagioli, M.; Pastorin, L.; Vista, N.; Baldassar, A.; Benso, L.

    1986-03-01

    Cockayne syndrome is a well-known autosomal recessive form of dwarfism with senile-like appearance. Skeletal changes such as flattening of vertebral bodies, ivory epiphyses and thickening of cranial vault, have been observed in some patients with this condition. We describe here a 5.5-year-old girl with the typical clinical signs of Cockayne syndrome and a distinctive form of bone dysplasia with major involvement of the spine.

  14. Malignant transformation of fibrous dysplasia into chondroblastic osteosarcoma

    International Nuclear Information System (INIS)

    Kaushik, Shaifali; Smoker, Wendy R.K.; Frable, William J.

    2002-01-01

    A case of malignant transformation of polyostotic fibrous dysplasia into maxillary chondroblastic osteosarcoma is presented. The clinical, radiographic, CT, MR imaging features and pathological findings of polyostotic fibrous dysplasia and its malignant transformation are described. Malignant transformation of fibrous dysplasia is rare and has not previously been described in the English literature in this location in McCune-Albright syndrome and in the absence of radiation treatment. (orig.)

  15. Florid osseous dysplasia of the jaws

    International Nuclear Information System (INIS)

    Cho, Su Beom; Koh, Kwang Joon

    1995-01-01

    Few cases of florid osseous dysplasia has been described as a condition that characteristically affects the jaws. It usually manifests as multiple radiopaque masses distributed throughout the jaws. Confusion exists about the relationship of florid osseous dysplasia, gigantiform cementoma, chronic sclerosing osteomyelitis, sclerosing osteitis or multiple enostosis. Authors experienced a case of florid osseous dysplasia of the jaws in 52-year-old female on the basis of clinical, radiographic and histopathologic findings. The characteristic features are as follows: 1. In clinical examination, there was no clinical sign and symptoms except extrated area. And there was no facial asymmetry. 2. Radiograms show round or lobular dense radiopaque masses surrounded by radiolucent bands in lower molar teeth area bilaterally. And slight increased radiopacities in maxillary molar teeth area bilaterllay. There was no expansion or thinning of buccal and lingual cortical bones. There is no displacement or resorption of involved teeth. In right side of mandible, mandibular canal is displaced inferiorly due to mass. 3. Photomicrograms show densely mineralized sclerotic acellular masses with empty lacunae. Pattern is suggestive of cementum, although it could be considered sclerotic bone. In the periphery, lesion consisting of moderately cellular fibrous tissue in calcified products are deposited.

  16. Determination of some variables of human dentin for dosimetric purposes

    International Nuclear Information System (INIS)

    Peres, C.

    1990-01-01

    The initial objective of this work is to determine the atomic composition of human dentin, using various techniques of measurements such as X-Ray fluorescence, atomic absorption, spectrophotometry, volumetric analysis, chemistry, colorimetry and thermogravimetry. With the values of the effective atomic number Z bar dependent of the quality of the ionizing radiation, one can find the kind of material that can be used for a phantom equivalent to dentin. (author)

  17. Bonding stability of adhesive systems to eroded dentin

    Directory of Open Access Journals (Sweden)

    Janaina Barros CRUZ

    2015-01-01

    Full Text Available This in vitro study evaluated the immediate and 6 months microshear bond strength (µSBS of different adhesive systems to sound and eroded dentin. Sixty bovine incisors were embedded in acrylic resin and ground to obtain flat buccal dentin surfaces. Specimens were randomly allocated into two groups: sound dentin (immersion in artificial saliva and eroded dentin (erosive challenge following a pH cycling model comprising 4 ×/day Sprite Light® drink for 10 days. Then, specimens were reassigned according to the adhesive system: etch-and-rinse adhesive (Adper Single Bond, two-step self-etch system (Clearfil SE Bond, or one-step self-etch adhesive (Adper Easy One. Polyethylene tubes with an internal diameter of 0.76 mm were placed over pre-treated dentin and filled with resin composite (Z250. Half of the specimens were evaluated by the µSBS test after 24 h, and the other half 6 months later, after water storage at 37°C. Failure mode was evaluated using a stereomicroscope (400×. Data were analyzed by three-way repeated measures analysis of variance and Tukey’s post hoc tests (α = 0.05. After 6 months of water aging, marked reductions in µSBS values were observed, irrespective of the substrate. The µSBS values for eroded dentin were lower than those obtained for sound dentin. No difference in bonding effectiveness was observed among adhesive systems. For all groups, adhesive/mixed failure was observed. In conclusion, eroded dentin compromises the bonding quality of adhesive systems over time.

  18. Treatment of oral dysplasia with 5% imiquimod cream: short communication.

    Science.gov (United States)

    Mullins, R; Ansell, M; Laverick, S

    2016-11-01

    We report what we think is the first treatment of oral dysplasia with 5% imiquimod cream. A 60-year-old man presented with varying degrees of dysplasia on the soft palate. A cover plate was fabricated and the patient was prescribed 5% imiquimod cream, a topical imunomodulator, for six weeks. The lesion improved and histological examination of an incisional biopsy found no features of dysplasia. This case highlights the efficacy of imiquimod cream in the treatment of dysplasia, and the need for development of a preparation suitable for the oral mucosa. Copyright © 2016. Published by Elsevier Ltd.

  19. The role of the acetabular labrum in hip dysplasia

    DEFF Research Database (Denmark)

    Hartig-Andreasen, Charlotte; Søballe, Kjeld; Troelsen, Anders

    2013-01-01

    A periacetabular osteotomy (PAO) is the preferred joint preserving treatment for young adults with symptomatic hip dysplasia and no osteoarthritis. In symptomatic dysplasia of the hip, there is labral pathology in up to 90% of cases. However, no consensus exists as to whether a labral tear should...... be treated before the periacetabular osteotomy (PAO), treated simultaneously with the PAO, or left alone and only treated if symptoms persist after the PAO. This review is an update of aspects of labral anatomy and function, the etiology of labral tears in hip dysplasia, and diagnostic assessment of labral...... tears, and we discuss treatment strategies for coexisting labral tears and hip dysplasia....

  20. Thanatophoric Dysplasia; a Rare Case Report on a Congenital Anomaly

    Directory of Open Access Journals (Sweden)

    Maria Francis Yuvaraj

    2017-01-01

    Full Text Available The rare form of skeletal dysplasia is thanatophoric dysplasia. The meaning for thanatophoric dysplasia is death bearing which is derived from Greek word. It occurs 1in 20,000 to 50,000. It is mainly due to mutations in the fibroblast growth factor receptor 3gene. Features of thanatophoric dysplasia are frontal bossing, prominent eyes, narrow thorax, protruded abdomen and bowed legs. The knowledge about this condition is useful in the fields of Anatomy, Paediatrics, Obstetrics and Gynaecology, Ultrasonagraphy and Genetics, for future research purpose.

  1. Focal cemento-osseous dysplasia: review and a case report.

    Science.gov (United States)

    Salem, Y M Y; Osman, Y I; Norval, E J G

    2010-10-01

    Focal cemento-osseous dysplasia is a benign fibro-osseous condition that can be seen in dentate and edentulous patients. It is an asymptomatic lesion and needs no treatment; however follow-up is essential due to the possibility that focal cemento-osseous dysplasia can progress to a condition called florid osseous dysplasia that involves multiple sites. A case report is presented here, along with a review of the differential diagnoses considered in order to reach a final diagnosis of focal cemento-osseous dysplasia.

  2. Comparison of three methods to diagnose hip dysplasia in dogs

    International Nuclear Information System (INIS)

    Sharma, Vikas; Mohindroo, J.

    2009-01-01

    The present study was designed to compare the usefulness of goniometry, radiography and distraction index in diagnosis of hip dysplasia in dogs. During the study 25 clinical cases (50 joints) suspected for hip dysplasia were evaluated. Norberg angle was found to have a significant positive correlation with extension, flexion, abduction, and adduction angles and a significant negative correlation with distraction index (DI) measurements. It could be inferred that all the six parameters (NA, DI, extension, flexion, abduction, and adduction) were reliable indicators for early diagnosis of hip dysplasia.Goniometry could be used as a safe and easy method for preliminary suspicion of hip dysplasia

  3. [Mondini dysplasia: traumatic cerebrospinal fluid otorrhea with meningitis].

    Science.gov (United States)

    Kaftan, H; Adamaszek, M; Hosemann, W

    2006-08-01

    Mondini dysplasia is a rare malformation of the inner ear commonly associated with loss of hearing and vestibular function. Children with Mondini dysplasia are predisposed to developing a spontaneous cerebrospinal fluid (CSF) leak and recurrent meningitis. If there is no CSF leak but a unilateral hearing loss, the condition may go undiagnosed for years. We describe a 65-year-old man with unrecognized unilateral Mondini dysplasia who presented with CSF leak and meningoencephalitis after minor head trauma. Two operative interventions were undertaken to close the defect properly. Patients with Mondini dysplasia or their parents should be cautioned about the potential hazards of any head trauma.

  4. The Ectodermal Dysplasias : Severe Palmoplantar Hyperkeratosis And Chronic Angular Cheilitis

    Directory of Open Access Journals (Sweden)

    Mahajan Vikram K

    2003-01-01

    Full Text Available The ectodermal dysplasias are congenital, non-progressive and diffuse disorders affecting primarily the tissues derived from ectoderm. Over a period, their classification has become confusing due to indiscriminate use of them “ectodermal dysplasia” for numerous syndromes with a defect in one or more epidermal defect in each element of skin; their precise classification appears difficult as yet. Only X-linked recessive ectodermal dysplasia (Christ-Siemens-Touraine syndrome remains best defined. This paper describes three cases of ectodermal dysplasias highlighting their overlapping features.

  5. Measurement of tibial tuberosity-trochlear groove distance: evaluation of inter- and intraobserver correlation dependent on the severity of trochlear dysplasia.

    Science.gov (United States)

    Dornacher, Daniel; Reichel, Heiko; Lippacher, Sabine

    2014-10-01

    Excessive tibial tuberosity-trochlear groove distance (TT-TG) is considered as one of the major risk factors in patellofemoral instability (PFI). TT-TG characterises the lateralisation of the tibial tuberosity and the medialisation of the trochlear groove in the case of trochlear dysplasia. The aim of this study was to assess the inter- and intraobserver reliability of the measurement of TT-TG dependent on the grade of trochlear dysplasia. Magnetic resonance imaging (MRI) scans of 99 consecutive knee joints were analysed retrospectively. Hereof, 61 knee joints presented with a history of PFI and 38 had no symptoms of PFI. After synopsis of the axial MRI scans with true lateral radiographs of the knee, the 61 knees presenting with PFI were assessed in terms of trochlear dysplasia. The knees were distributed according to the four-type classification system described by Dejour. Regarding interobserver correlation for the measurements of TT-TG in trochlear dysplasia, we found r=0.89 (type A), r=0.90 (type B), r=0.74 (type C) and 0.62 (type D) for Pearson's correlation coefficient. Regarding intraobserver correlation, we calculated r=0.89 (type A), r=0.91 (type B), r=0.77 (type C) and r=0.71 (type D), respectively. Pearson's correlation coefficient for the measurement of TT-TG in normal knees resulted in r=0.87 for interobserver correlation and r=0.90 for intraobserver correlation. Decreasing inter- and intraobserver correlation for the measurement of TT-TG with increasing severity of trochlear dysplasia was detected. In our opinion, the measurement of TT-TG is of significance in low-grade trochlear dysplasia. The final decision to perform a distal realignment procedure based on a pathological TT-TG in the presence of high-grade trochlear dysplasia should be reassessed properly. Retrospective study, Level II.

  6. Bond strength of compomers to dentin using acidic primers.

    Science.gov (United States)

    Tate, W H; You, C; Powers, J M

    1999-10-01

    To determine the in vitro bond strengths of seven compomer/bonding agent restorative systems to human dentin. Seven compomer/bonding agents were bonded to human dentin, stored in water at 37 degrees C for 24 hours, and debonded in tension. Bonding conditions were with and without phosphoric acid etching, with and without the use of combined primer/bonding agents, and under moist and wet bond interfaces. Without phosphoric acid etching, F2000/F2000 Compomer Primer/Adhesive and F2000/Single Bond Dental Adhesive System were less sensitive to dentin wetness. With moist dentin, bond strengths of Dyract/Prime & Bond 2.1, Dyract AP/Prime & Bond 2.1, Hytac/OSB light-curing, one-component bonding agent, F2000/Single Bond, and Freedom/STAE single component light-cured dentin/enamel adhesive system, were improved with phosphoric acid etching. Also, with moist dentin, the bond strength of F2000/F2000 Compomer Primer/Adhesive in the 3M Clicker dispensing system was higher without phosphoric acid etching, whereas bonds of Compoglass/Syntac Single-component were not affected by phosphoric acid etching. Bonding did not occur without primer/bonding agent, regardless of surface condition or use of phosphoric acid etching.

  7. Osseous dysplasia (cemento-osseous dysplasia) of the jaw bones in western Pennsylvania patients: analysis of 35 cases.

    Science.gov (United States)

    Owosho, Adepitan A; Potluri, Anitha; Bilodeau, Elizabeth A

    2013-01-01

    The purpose of this study is to analyze the demographic, clinical, and radiographic presentations of osseous dysplasia of the jaws in western Pennsylvania patients and its associated complications. The clinical records and radiographs of patients diagnosed with osseous (cement-osseous) dysplasia were retrieved from the electronic health record of the University of Pittsburgh, School of Dental Medicine from 2007 to 2012. All cases were reviewed; the WHO criteria and classification for osseous dysplasia was used. Clinical and demographic data, radiographic findings, and final diagnoses were collected and analyzed. 35 cases of osseous dysplasia were retrieved over the six-year period.The majority (33) were females [94.3%], with ages ranging from 26 to 89 years, with a mean age of 53.9 years +/- standard deviation of 15.6 years, 32 [91.4%] were African Americans and 3 [8.6%] were Caucasians. 17 [48.6%] were florid osseous dysplasia, 13 [37.1%] periapical osseous dysplasia and 5 [14.3%] focal osseous dysplasia. Of the 35 patients only 8 [22.9%] patients were symptomatic. All florid osseous dysplasia patients were African American females, with 7 of the patients being symptomatic and the commonest symptom being pain. Also, all periapical osseous dysplasia patients were African Americans (12 females and 1 male), with 1 of the patients presenting with widening of the diastema. Of the focal osseous dysplasia patients, 3 were Caucasians and 2 African American (4 females and 1 male). The cases occurred mostly in African American females with a peak incidence in the fifth and sixth decades of life; most cases occurred in the mandible. The commonest form of osseous dysplasias was the florid osseous dysplasia which is most likely to present with symptoms.

  8. Ectodermal dysplasia-skin fragility syndrome: A rare case report

    Directory of Open Access Journals (Sweden)

    Subhash Kashyap

    2015-01-01

    Full Text Available Ectodermal dysplasia/skin fragility syndrome (ED-SFS is a newly described autosomal recessive disorder characterized by skin fragility and blistering, palmoplantar keratoderma, abnormal hair growth, nail dystrophy, and occasionally defective sweating. It results from mutations in the PKP1 gene encoding plakophilin 1 (PKP1, which is an important component of stratifying epithelial desmosomes and a nuclear component of many cell types. Only 12 cases of this rare genodermatosis have been reported so far. We present an unusual case of ED-SFS in a 12-year boy who was normal at birth but subsequently developed skin fragility, hair and nail deformities, abnormal dentition, palmoplantar keratoderma, and abnormal sweating but no systemic abnormality.

  9. Laboratory evaluation of the effect of unfilled resin after the use of self-etch and total-etch dentin adhesives on the Shear Bond Strength of composite to dentin.

    Science.gov (United States)

    Nasseri, Ehsan Baradaran; Majidinia, Sara; Sharbaf, Davood Aghasizadeh

    2017-05-01

    Based on the frequent application of composite resins as tooth-colored fillings, this method is considered a viable alternative to dental amalgam. However, this method has the low bond strength of the composite to dentin. To solve this issue, various dental adhesive systems with different characteristics have been developed by dentistry experts. To assess the effect of an additional layer of unfilled resin in self-etch and total-etch dentin adhesives on the shear bond strength (SBS) of composite to dentin. Moreover, we assessed the effects of sample storage in artificial saliva on the SBS of composite to dentin. Methods: This experimental study was conducted on 160 freshly extracted human first or second premolar teeth, which were randomly divided into 16 groups. The teeth were prepared from Mashhad University of Medical Sciences, Mashhad, Iran (2008-2009). Scotchbond Multi-purpose (SBMP), single bond (SB), Clearfil SE Bond, and Clearfil S3 Bond were applied to dentin surface with or without the placement of hydrophobic resin (Margin Bond) in accordance with the instructions of the manufacturers. To expose the coronal dentin, the teeth were abraded with 600 grit SiC paper. Immediately after restoration, half of the samples were tested in terms of SBS, while the other samples were evaluated in terms of SBS after three months of storage in artificial saliva. SBS rates of dental composites evaluated by universal testing machine and samples were studied by optical stereomicroscopy to verify the failure type. Data analysis was performed in SPSS V.16 using Kolmogorov-Smirnov test, independent-samples t-test, ANOVA, and Duncan's logistic regression test. In this study, a significant reduction was observed in the SBS rates of SB and S3 bond adhesive systems after storage with and without hydrophobic resin (p>0.000). Without storage in normal saline, a significant increase was observed in the SBS rate of the SE bond (p=0.013). In addition, SBS rate of SBMP significantly

  10. The expanding spectrum of COL2A1 gene variants IN 136 patients with a skeletal dysplasia phenotype.

    Science.gov (United States)

    Barat-Houari, Mouna; Dumont, Bruno; Fabre, Aurélie; Them, Frédéric Tm; Alembik, Yves; Alessandri, Jean-Luc; Amiel, Jeanne; Audebert, Séverine; Baumann-Morel, Clarisse; Blanchet, Patricia; Bieth, Eric; Brechard, Marie; Busa, Tiffany; Calvas, Patrick; Capri, Yline; Cartault, François; Chassaing, Nicolas; Ciorca, Vidrica; Coubes, Christine; David, Albert; Delezoide, Anne-Lise; Dupin-Deguine, Delphine; El Chehadeh, Salima; Faivre, Laurence; Giuliano, Fabienne; Goldenberg, Alice; Isidor, Bertrand; Jacquemont, Marie-Line; Julia, Sophie; Kaplan, Josseline; Lacombe, Didier; Lebrun, Marine; Marlin, Sandrine; Martin-Coignard, Dominique; Martinovic, Jelena; Masurel, Alice; Melki, Judith; Mozelle-Nivoix, Monique; Nguyen, Karine; Odent, Sylvie; Philip, Nicole; Pinson, Lucile; Plessis, Ghislaine; Quélin, Chloé; Shaeffer, Elise; Sigaudy, Sabine; Thauvin, Christel; Till, Marianne; Touraine, Renaud; Vigneron, Jacqueline; Baujat, Geneviève; Cormier-Daire, Valérie; Le Merrer, Martine; Geneviève, David; Touitou, Isabelle

    2016-07-01

    Heterozygous COL2A1 variants cause a wide spectrum of skeletal dysplasia termed type II collagenopathies. We assessed the impact of this gene in our French series. A decision tree was applied to select 136 probands (71 Stickler cases, 21 Spondyloepiphyseal dysplasia congenita cases, 11 Kniest dysplasia cases, and 34 other dysplasia cases) before molecular diagnosis by Sanger sequencing. We identified 66 different variants among the 71 positive patients. Among those patients, 18 belonged to multiplex families and 53 were sporadic. Most variants (38/44, 86%) were located in the triple helical domain of the collagen chain and glycine substitutions were mainly observed in severe phenotypes, whereas arginine to cysteine changes were more often encountered in moderate phenotypes. This series of skeletal dysplasia is one of the largest reported so far, adding 44 novel variants (15%) to published data. We have confirmed that about half of our Stickler patients (46%) carried a COL2A1 variant, and that the molecular spectrum was different across the phenotypes. To further address the question of genotype-phenotype correlation, we plan to screen our patients for other candidate genes using a targeted next-generation sequencing approach.

  11. Mandibuloacral dysplasia: A premature ageing disease with aspects of physiological ageing.

    Science.gov (United States)

    Cenni, Vittoria; D'Apice, Maria Rosaria; Garagnani, Paolo; Columbaro, Marta; Novelli, Giuseppe; Franceschi, Claudio; Lattanzi, Giovanna

    2018-03-01

    Mandibuloacral dysplasia (MAD) is a rare genetic condition characterized by bone abnormalities including localized osteolysis and generalized osteoporosis, skin pigmentation, lipodystrophic signs and mildly accelerated ageing. The molecular defects associated with MAD are mutations in LMNA or ZMPSTE24 (FACE1) gene, causing type A or type B MAD, respectively. Downstream of LMNA or ZMPSTE24 mutations, the lamin A precursor, prelamin A, is accumulated in cells and affects chromatin dynamics and stress response. A new form of mandibuloacral dysplasia has been recently associated with mutations in POLD1 gene, encoding DNA polymerase delta, a major player in DNA replication. Of note, involvement of prelamin A in chromatin dynamics and recruitment of DNA repair factors has been also determined under physiological conditions, at the border between stress response and cellular senescence. Here, we review current knowledge on MAD clinical and pathogenetic aspects and highlight aspects typical of physiological ageing. Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.

  12. Effect of different surface treatments on adhesion of In-Ceram Zirconia to enamel and dentin substrates.

    Science.gov (United States)

    Saker, Samah; Ibrahim, Fatma; Ozcan, Mutlu

    2013-08-01

    Resin bonding of In-Ceram Zirconia (ICZ) ceramics is still a challenge, especially for minimally invasive applications. This study evaluated the adhesion of ICZ to enamel and dentin after different surface treatments of the ceramic. ICZ ceramic specimens (diameter: 6 mm; thickness: 2 mm) (N = 100) were fabricated following the manufacturer's instructions and randomly assigned to 5 groups (n = 20), according to the surface treatment methods applied. The groups were as follows: group C: no treatment; group SB: sandblasting; group SCS-S: CoJet+silane; group SCS-P: CoJet+Alloy Primer; group GE-S: glaze+ hydrofluoric acid etching (9.6%) for 60 s+silane. Each group was randomly divided into two subgroups to be bonded to either enamel or dentin (n = 10 per group) using MDP-based resin cement (Panavia F2.0). All the specimens were subjected to thermocycling (5000x, 5°C-55°C). The specimens were mounted in a universal testing machine and tensile force was applied to the ceramic/cement interface until failure occurred (1 mm/min). After evaluating all the debonded specimens under SEM, the failure types were defined as either "adhesive" with no cement left on the ceramic surface (score 0) or "mixed" with less than 1/2 of the cement left adhered to the surface with no cohesive failure of the substrate (score 1). The data were statistically evaluated using 2-way ANOVA and Tukey's tests (α = 0.05). The highest tensile bond strength for the enamel surfaces was obtained in group GE-S (18.1 ± 2 MPa) and the lowest in group SB (7.1 ± 1.4 MPa). Regarding dentin, group CSC-P showed the highest (12 ± 1.3 MPa) and SB the lowest tensile bond strength (5.7 ± 0.4 MPa). Groups SB, CSC-S, CSC-P, and GE-S did not show significant differences between the different surface treatments on either enamel or dentin surfaces (p enamel and dentin substrates (p enamel substrates, exclusively adhesive failures from ICZ (score 0) were found, on dentin exclusively mixed failures were observed (score

  13. A new form of spondyloperipheral dysplasia with facial dysmorphism, flattened vertebrae, hypoplastic pelvis, brachydactyly and soft tissue syndactyly

    International Nuclear Information System (INIS)

    Kitoh, Hiroshi

    2001-01-01

    We report the case of a 9-year-old Japanese boy with spondyloperipheral skeletal dysplasia associated with facial dysmorphism, pelvic abnormalities, and distinctive hands and feet. Radiographic manifestations included mild platyspondyly with posterior scalloping, small flared ilia with shallow acetabulae, mesomelic shortening of long bones, marked delay of carpal bone maturation, and brachydactyly with hypoplastic middle and terminal phalanges bilaterally in both hands and feet. There was bilateral soft tissue syndactyly of the 2nd and 3rd interdigital spaces of the hands, the 2nd interdigital space of the feet, with hypoplastic nails. The clinical and radiographic manifestations in this case appear to represent a unique type of skeletal dysplasia. (orig.)

  14. Hipótesis actuales relacionadas con la sensibilidad dentinal

    Directory of Open Access Journals (Sweden)

    Andrés Pérez Ruiz

    1999-08-01

    Full Text Available Se hace una revisión sobre las hipótesis actuales concernientes a la sensibilidad dentinal, centrando el análisis en las teorías acerca de la presencia de nervios en la dentina, la transmisión del impulso nervioso mediante el odontoblasto y el movimiento de fluidos dentro de la dentina. Por ser la explicación de la sensibilidad de la dentina uno de los más complejos problemas de la fisiología y la histología del diente, se exponen los hechos morfológicos y funcionales que apoyan la validez de los mecanismos mencionados. Se concluye que aunque la teoría hidrodinámica tiene una sólida base experimental y es la de mayor aceptación en la actualidad, pudieran estar involucrados en la génesis del dolor dentinal más de una de estas explicaciones probables.A review of the present hypotheses of dentinal sensitivity was carried out. The analysis was centered on the theories of the existance of nerves in dentin, the transfer of nervous impulse through odontoblast and the fluid flow in the dentin. Since the explanation of dentin sensitivity is one of the most complex problems of dental physiology and histology; the morphological and functional aspects that support the validity of the mentioned mechanisms are stated. It is concluded that although the hydrodynamic theory has a sound experimental basis and is widely accepted at present, more than one of these likely theories could be involved in the genesis of dentinal pain.

  15. In vitro mechanical stimulation facilitates stress dissipation and sealing ability at the conventional glass ionomer cement-dentin interface.

    Science.gov (United States)

    Toledano, Manuel; Osorio, Raquel; Osorio, Estrella; Cabello, Inmaculada; Toledano-Osorio, Manuel; Aguilera, Fátima S

    2018-06-01

    The aim of this study was to evaluate the induced changes in the chemical and mechanical performance at the glass-ionomer cement-dentin interface after mechanical load application. A conventional glass-ionomer cement (GIC) (Ketac Bond), and a resin-modified glass-ionomer cement (RMGIC) (Vitrebond Plus) were used. Bonded interfaces were stored in simulated body fluid, and then tested or submitted to the mechanical loading challenge. Different loading waveforms were applied: No cycling, 24 h cycled in sine or loaded in sustained hold waveforms. The cement-dentin interface was evaluated using a nano-dynamic mechanical analysis, estimating the complex modulus and tan δ. Atomic Force Microscopy (AFM) imaging, Raman analysis and dye assisted confocal microscopy evaluation (CLSM) were also performed. The complex modulus was lower and tan delta was higher at interfaces promoted with the GIC if compared to the RMGIC unloaded. The conventional GIC attained evident reduction of nanoleakage. Mechanical loading favored remineralization and promoted higher complex modulus and lower tan delta values at interfaces with RMGIC, where porosity, micropermeability and nanoleakage were more abundant. Mechanical stimuli diminished the resistance to deformation and increased the stored energy at the GIC-dentin interface. The conventional GIC induced less porosity and nanoleakage than RMGIC. The RMGIC increased nanoleakage at the porous interface, and dye sorption appeared within the cement. Both cements created amorphous and crystalline apatites at the interface depending on the type of mechanical loading. Remineralization, lower stress concentration and resistance to deformation after mechanical loading improved the sealing of the GIC-dentin interface. In vitro oral function will favor high levels of accumulated energy and permits micropermeability at the RMGIC-dentin interface which will become remineralized. Copyright © 2018 Elsevier Ltd. All rights reserved.

  16. Effect of root canal rinsing protocol on dentin bond strength of two resin cements using three different method of test.

    Science.gov (United States)

    Khoroushi, Maryam; Sheikhi, Mohammadreza; Khalilian-Gourtani, Amirhossein; Soleimani, Bahram

    2016-07-01

    Different studies have used different tests to evaluate bond strength of resin cements to root dentin. In this in vitrostudy, three different tests were used to evaluate the bond strength of two resin cements to root dentin using two root dentin irrigation protocols. Ninety-six intact single-rooted teeth were selected for this study. Forty-eight teeth, with a root length of 15mm, were randomly divided into two groups and irrigated with normal saline or 2.5% sodium hypochlorite solutions during root canal preparation, respectively. For each 12 specimens from each group, fiber post #1 was bonded using an etch-and-rinse (Duo-Link) and a self-adhesive (BisCem) resin cement, respectively. After incubation, two specimens were prepared for the push-out test from the middle thirds of the roots. In another 24 teeth, after two 1.5-mm sections were prepared from the middle thirds of the prepared roots, sections of the post were bonded in two subgroups with each of the cements mentioned above and the samples were prepared for the pull-out test. For shear test, the crowns of 48 teeth were cut away, the dentin surfaces were prepared, the two irrigation solutions were used, and the resin cements were bonded. Data collected from the three tests were evaluated by ANOVA, post-hoc Tukey and Weibull tests (α=0.05). There were significant differences in the mean bond strength values between the three bond strength tests (Pstrength in all tests (P>0.05). Under the limitations of the present study, the method of the test used had an effect on the recorded bond strength between the resin cement and root dentin. Cement type and irrigation protocol resulted in similar variations with all the tests. Push-out and shear tests exhibited more coherent results. Bond strength, endodontically treated tooth, fiber post, resin cement, sodium hypochlorite.

  17. Evaluation of bond strength of self-etching adhesives having different pH on primary and permanent teeth dentin.

    Science.gov (United States)

    Ozmen, Bilal; Koyuturk, Alp Erdin; Tokay, Ugur; Cortcu, Murat; Sari, Mustafa Erhan

    2015-10-16

    The purpose of this in vitro study was to evaluate the dentin shear bond strength of 4 self-etching adhesives having a different pH on primary and permanent teeth dentin. The occlusal enamel was removed from 60 freshly extracted third molar and 60 primary second molar human teeth, which were randomly separated into 4 groups (n = 15). Four adhesive systems were applied: G-Bond (GC Corporation, Tokyo, Japan, pH: 1.5), Futura Bond M (Voco, Cuxhaver, Germany, pH: 1.4), Adper Prompt L-Pop (3M/ESPE, St Paul, MN, USA, pH: 0.8), and Clearfil S(3) Bond (Kuraray Medical, Tokyo, Japan, pH: 2.7) according to the manufacturer's instructions. After the application of dentin bonding agents, a composite resin material (Z250 Restorative A2, 3M ESPE, St. Paul, MN, USA) for permanent teeth and a compomer resin material (Dyract Extra A2, Dentsply, Konstanz, Germany) for primary teeth was applied onto the prepared dentin surfaces. The data were obtained by using a universal test machine at a crosshead speed of 1 mm/min. The mean values were compared using Tukey's multiple comparison test. Although there was no difference between adhesives on the permanent teeth, Clearfil S3 adhesive showed higher bond (18.07 ± 0.58 MPa) (P>0.05). Lower bond strength values were obtained from primary teeth and especially G-Bond adhesive (9.36 ± 0.48 MPa) (Padhesives with different pH and solvent types can be used successfully for permanent teeth dentin but adhesives with low pH did not provide greater shear bond strength values.

  18. The Effect of Laser Irradiation on Shear Bond Strength of GI to Dentin After CPP-ACP Treatment

    Directory of Open Access Journals (Sweden)

    Moezizadeh

    2016-02-01

    Full Text Available Background Dentin sensitivity is one of the most important problems in dentistry. Enamel loss due to root exposure is serious issue and common exposure is one of the reasons for dentin hypersensitivity. There are different methods for solving this problem. One of the most conservative and least expensive methods is use of casein phosphopeptide-amorphous calcium phosphate (CPP-ACP paste. Objectives The aim of this study was to evaluate shear bond strength of GIC to dentin, with or without laser, CPP-ACP paste and polyacrylic acid treatments. Materials and Methods Fifty sound human third molars were bisected in a mesiodistal direction using a diamond disk. Using 400, 600 and 800 grit silicon carbide paper, dentin surfaces were exposed. The teeth were divided into five groups. In groups A, B, D and H, CPP-ACP (GC tooth mousse Itabashi-Ku, Tokyo, Japan was applied for one hour the first day and repeated at the same time of day for a total of five days. In groups B, C, D and E, the specimens were subjected to laser for 10 seconds using Er, Cr: YSGG laser. In groups B, C, H and G, specimens were treated with 10% polyacrylic acid for 20 seconds. A plastic tube containing GI was positioned over the tooth. Samples were loaded in shear bond using a Universal Testing Machine (Zwick/Roell, Germany, at a 0.5 mm/minute crosshead speed. Results Despite the failing of groups A and D, group analysis showed that there were no significant differences between the groups. The predominant type of fracture in all groups was adhesive. Conclusions Application of CPP-ACP, without preconditioning with polyacrylic acid, can decrease shear bond strength. Laser irradiation has no effect on shear bond strength of GIC to dentin in this condition.

  19. Cleidocranial dysplasia: Report of 4 cases and review

    Directory of Open Access Journals (Sweden)

    Virender Gombra

    2008-01-01

    Full Text Available Patients with cleidocranial dysplasia commonly present with significant dental problems such as retention of multiple deciduous teeth, impaction or delay in eruption of permanent teeth and often, the presence of supernumerary teeth. We report 4 cases of 2 families presenting with cleidocranial dysplasia disorder with their clinical and radiological diagnosis and illustrating its pathogenesis and various treatment modalities, review of literatures.

  20. Marfan syndrome with multiseptate pneumothorax and mandibular fibrous dysplasia

    Directory of Open Access Journals (Sweden)

    Kate A

    2009-01-01

    Full Text Available We describe a rare case of pneumothorax due to Marfan syndrome associated with fibrous dysplasia of the mandible. Marfan syndrome and fibrous dysplasia were possibly due to a common etiological factor. The association between the two and other tumors described in literature related to Marfan syndrome is discussed.

  1. Prevalence of Cervical Dysplasia among Women in Kano Municipal ...

    African Journals Online (AJOL)

    Fifty-seven women had cervical dysplasia giving a prevalence rate of 10.63%. Of the 57 women with cervical dysplasia, 21 (36.8%) had a low grade squamous intraepithelial lesions (LGSIL), while 36(63.2%) had a high grade squamous intraepithelial lesion (HGSIL). The disease was higher in grand multiparous clients ...

  2. Further delineation of spondylometaphyseal dysplasia with cone-rod dystrophy

    NARCIS (Netherlands)

    Sousa, Sérgio B.; Russell-Eggitt, Isabelle; Hall, Christine; Hall, Bryan D.; Hennekam, Raoul C. M.

    2008-01-01

    There are several entities that combine a skeletal dysplasia with a retinal dystrophy. Recently, another possibly autosomal recessive entity was added to this group characterized by a specific spondylometaphyseal dysplasia and a cone-rod dystrophy, without other significant impairments. The entity

  3. The Possible Relationship Between Mammary Dysplasia and Breast ...

    African Journals Online (AJOL)

    Aim: There is need to resolve the continuing difficult question regarding the possible relationship between mammary dysplasia and breast cancer. Method: This is a 30-year study of the incidences of both mammary dysplasia and breast cancer occurring among the Igbos, a major ethnic group in Nigeria, West Africa. Results: ...

  4. Joint space width in dysplasia of the hip

    DEFF Research Database (Denmark)

    Jacobsen, Steffen; Sonne-Holm, Stig; Søballe, K

    2005-01-01

    . Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia...

  5. Genetics Home Reference: X-linked spondyloepiphyseal dysplasia tarda

    Science.gov (United States)

    ... Educational Resources (6 links) Cincinnati Children's Hospital: Coxa Vera Disease InfoSearch: Spondyloepiphyseal dysplasia tarda X-linked Johns ... Free article on PubMed Central Savarirayan R, Thompson E, Gécz J. Spondyloepiphyseal dysplasia tarda (SEDL, MIM #313400). ...

  6. Knee radiography in the diagnosis of skeletal dysplasias

    International Nuclear Information System (INIS)

    Kwee, Thomas C.; Beek, Frederik J.A.; Nievelstein, Rutger A.J.; Beemer, Frits A.

    2006-01-01

    Flattening of the epiphyses of long bones is seen in several skeletal dysplasias and standardized measurements on a radiograph of the knee to detect skeletal dysplasias using this feature have been described. Since then only two other studies in which this method was used have been published, and both included only a small number of children and neither had a control group. In addition, the Dutch National Working Group on Skeletal Dysplasias began to have doubts about the reliability of the method. We therefore decided to re-evaluate its accuracy in a population of children with and without a skeletal dysplasia. To determine the diagnostic value of standardized measurements on conventional AP radiographs of the knee in children with a skeletal dysplasia. Subjects and methods: We measured the distal femoral metaphysis and epiphysis according to the published method on conventional AP radiographs of the knee in 45 healthy children and 52 children with a skeletal dysplasia. We compared graphically the height of the distal femoral epiphysis with its width and with the width of the femoral metaphysis. Receiver operating characteristic (ROC) curves were calculated for each group of children. All graphs showed a considerable overlap between children with a skeletal dysplasia and healthy children. The size of the area under the ROC curves for the different groups was small, varying between 0.567 and 0.653. This method does not discriminate between children with a skeletal dysplasia and healthy children. We therefore consider it to be of little diagnostic value. (orig.)

  7. Management of high-grade dysplasia in Barrett's esophagus.

    Science.gov (United States)

    Palley, S L; Sampliner, R E; Garewal, H S

    1989-08-01

    When Barrett's esophagus is complicated by adenocarcinoma, surgery is indicated in appropriate patients. Until now, high-grade dysplasia in Barrett's esophagus has been managed in a similar fashion. We explore this approach and review reported cases of high-grade dysplasia to suggest guidelines for collection of data to make future clinical decisions more rational.

  8. A study of the clinical and radiological features in a cohort of 93 patients with a COL2A1 mutation causing spondyloepiphyseal dysplasia congenita or a related phenotype

    DEFF Research Database (Denmark)

    Terhal, Paulien A; Nievelstein, Rutger Jan A J; Verver, Eva J J

    2015-01-01

    -like dysplasia (n = 2). The remaining 13 patients had normal stature with mild SED, Stickler-like syndrome or multiple epiphyseal dysplasia. Over 50% of the patients had undergone orthopedic surgery, usually for scoliosis, femoral osteotomy or hip replacement. Odontoid hypoplasia was present in 56% (95% CI 38......Type 2 collagen disorders encompass a diverse group of skeletal dysplasias that are commonly associated with orthopedic, ocular, and hearing problems. However, the frequency of many clinical features has never been determined. We retrospectively investigated the clinical, radiological......, and genotypic data in a group of 93 patients with molecularly confirmed SEDC or a related disorder. The majority of the patients (80/93) had short stature, with radiological features of SEDC (n = 64), others having SEMD (n = 5), Kniest dysplasia (n = 7), spondyloperipheral dysplasia (n = 2), or Torrance...

  9. Cerebral infarction attributable to cerebrovascular fibromuscular dysplasia

    International Nuclear Information System (INIS)

    Shimazaki, Haruo

    2008-01-01

    Fibromuscular dysplasia (FMD) is a noninflammatory, nonatheromatous segmental angiopathy. The renal arteries are affected most commonly, followed by the internal carotid and vertebral arteries. FMD of the internal carotid and vertebral arteries usually occurs in the extracranial portions and is mostly observed at the level of the second cervical vertebra. FMD of the intracranial arteries is rare, but tends to occur in children and young adults. FMD is more common in females than in males, and it is often observed in middle-aged women. Although the etiology of FMD is not well understood, several mechanisms have been proposed, such as genetic predisposition, hormonal factors, and arterial wall ischemia. The pathology of FMD is characterized by smooth muscle hyperplasia or thinning, elastic fiber destruction, fibrous tissue proliferation, and arterial wall disorganization. Cerebrovascular fibromuscular dysplasia (cFMD) is relatively rare in Japan but is regarded as one of the cardinal causes of stroke in the younger population. cFMD without complications causes nonspecific symptoms such as headache or vertigo, but when it results in an arterial dissection or aneurysm, it leads to cerebral infarction or subarachnoid hemorrhage. Conventional angiographic findings mostly reveal a pattern called the 'string of beads', which is pathologically correlated to medial fibromuscular dysplasia. Doppler echography, computed tomography and magnetic resonance angiography (MRA) may be useful for detecting cFMD lesions in some cases. MRA should be performed to rule out the presence of intracranial aneurysms. Antiplatelet and anticoagulation agents are prophylactics against cFMD complications. Surgical treatments such as graduated intraluminal dilatation had previously been the mainstays for treating cFMD. Percutaneous transluminal angioplasty with or without stenting has now become the preferred invasive treatment for symptomatic cFMD. (author)

  10. Effect of salivary contamination and decontamination on bond strength of two one-step self-etching adhesives to dentin of primary and permanent teeth.

    Science.gov (United States)

    Santschi, Katharina; Peutzfeldt, Anne; Lussi, Adrian; Flury, Simon

    2015-02-01

    To evaluate the effects of human saliva contamination and two decontamination procedures at different stages of the bonding procedure on the bond strength of two one-step self-etching adhesives to primary and permanent dentin. Extracted human primary and permanent molars (210 of each) were ground to mid-coronal dentin. The dentin specimens were randomly divided into 7 groups (n = 15/group/molar type) for each adhesive (Xeno V+ and Scotchbond Universal): no saliva contamination (control); saliva contamination before or after light curing of the adhesives followed by air drying, rinsing with water spray/air drying, or by rinsing with water spray/air drying/reapplication of the adhesives. Resin composite (Filtek Z250) was applied on the treated dentin surfaces. The specimens were stored at 37°C and 100% humidity for 24 h. After storage, shear bond strength (SBS) was measured and data analyzed with nonparametric ANOVA followed by exact Wilcoxon rank sum tests. Xeno V+ generated significantly higher SBS than Scotchbond Universal when no saliva contamination occurred. Saliva contamination reduced SBS of Xeno V+, with the reduction being more pronounced when contamination occurred before light curing than after. In both situations, decontamination involving reapplication of the adhesive restored SBS. Saliva contamination had no significant effect on Scotchbond Universal. There were no differences in SBS between primary and permanent teeth. Rinsing with water and air drying followed by reapplication of the adhesive restored bond strength to saliva-contaminated dentin.

  11. Evaluation of caries progression in dentin treated by fluoride-containing materials using an in-air micro-PIGE and micro-PIXE measurement system

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, H., E-mail: yhiroko@dent.osaka-u.ac.jp [Department of Restorative Dentistry and Endodontology, Osaka University Graduate School of Dentistry, 1-8 Yamada-Oka, Suita 565-0871 (Japan); Iwami, Y.; Yagi, K.; Hayashi, M. [Department of Restorative Dentistry and Endodontology, Osaka University Graduate School of Dentistry, 1-8 Yamada-Oka, Suita 565-0871 (Japan); Komatsu, H.; Okuyama, K.; Matsuda, Y. [Graduate School of Dental Medicine, Hokkaido University, Kita 13, Nishi 7, Kita-ku, Sapporo 060-8586 (Japan); Yasuda, K. [The Wakasa Wan Energy Research Center, 64-52-1 Nagatani, Tsuruga 914-0192 (Japan)

    2015-04-01

    It is well-known that fluorine (F) is involved in the progression of caries. The evaluation of caries progression has conventionally been based on the change in mineral content using transverse microradiography (TMR). The purpose of this study was to evaluate the progression of dentinal caries by the change in calcium (Ca) content using Particle-Induced Gamma-ray Emission/Particle-Induced X-ray Emission (PIGE/PIXE) techniques at the Wakasa Wan Energy Research Center. We also assessed the relationship between caries progression rate and the concentration of F penetration into dentin from dental fluoride-containing materials (FCMs). Dentin sections of six extracted human teeth were prepared to obtain various amounts of F uptake using three types of FCMs. F and Ca distribution of specimens were obtained using PIGE/PIXE techniques. After evaluation, the specimens were immersed in 10 ml of demineralizing solution (pH 4.5) to simulate caries attack. To estimate caries progression rates, the same portions of the specimens were evaluated after caries attack treatment using PIGE/PIXE. A negative correlation between the F uptake in dentin and the rate of caries progression was observed. Therefore, caries progression in dentin was reduced by increasing the amount of F uptake from FCMs. This demonstrates that PIGE/PIXE techniques are valuable for estimating caries progression rates.

  12. Evaluation of caries progression in dentin treated by fluoride-containing materials using an in-air micro-PIGE and micro-PIXE measurement system

    International Nuclear Information System (INIS)

    Yamamoto, H.; Iwami, Y.; Yagi, K.; Hayashi, M.; Komatsu, H.; Okuyama, K.; Matsuda, Y.; Yasuda, K.

    2015-01-01

    It is well-known that fluorine (F) is involved in the progression of caries. The evaluation of caries progression has conventionally been based on the change in mineral content using transverse microradiography (TMR). The purpose of this study was to evaluate the progression of dentinal caries by the change in calcium (Ca) content using Particle-Induced Gamma-ray Emission/Particle-Induced X-ray Emission (PIGE/PIXE) techniques at the Wakasa Wan Energy Research Center. We also assessed the relationship between caries progression rate and the concentration of F penetration into dentin from dental fluoride-containing materials (FCMs). Dentin sections of six extracted human teeth were prepared to obtain various amounts of F uptake using three types of FCMs. F and Ca distribution of specimens were obtained using PIGE/PIXE techniques. After evaluation, the specimens were immersed in 10 ml of demineralizing solution (pH 4.5) to simulate caries attack. To estimate caries progression rates, the same portions of the specimens were evaluated after caries attack treatment using PIGE/PIXE. A negative correlation between the F uptake in dentin and the rate of caries progression was observed. Therefore, caries progression in dentin was reduced by increasing the amount of F uptake from FCMs. This demonstrates that PIGE/PIXE techniques are valuable for estimating caries progression rates

  13. Renal dysplasia and MRI: a clinician's perspective

    Energy Technology Data Exchange (ETDEWEB)

    Greenbaum, Larry A. [Emory University, Division of Pediatric Nephrology, Children' s Healthcare of Atlanta, Atlanta, GA (United States)

    2008-01-15

    Renal dysplasia is a common abnormality in children. The role of MRI in evaluating children with renal dysplasia is evolving. More information is clearly necessary before MRI replaces conventional imaging modalities. In order to appropriately use MRI, the radiologist must have an understanding of the clinical questions that are important in the management of children with renal dysplasia. This review provides background information on renal dysplasia for the pediatric radiologist. The focus is on unilateral disease, especially multicystic dysplastic kidneys, and bilateral dysplasia, which is the most common cause of kidney failure in children. The emphasis is on the important clinical issues, and the potential of MRI as a methodology for providing clinically useful information not otherwise available from other imaging modalities. (orig.)

  14. An Uncommon Reason of Osteoporosis: Spondyloepiphyseal Dysplasia Congenita

    Directory of Open Access Journals (Sweden)

    Onur Elbasan

    2017-12-01

    Full Text Available Skeletal dysplasia is a complex and rare disease group that presents with clinical and radiological findings that differ from classical metabolic bone diseases in which bone and cartilage tissue are affected together. Spondyloepiphyseal dysplasia conjenita with involvement of the spine and long bone epiphyses is seen with short stature and short body from birth. Although bone deformities such as shortness of neck and vertebrae, kyphosis, scoliosis, pectus carinatum, genu varum or valgum are frequent, association with osteopenia/osteoporosis has been rarely reported. Although spondyloepiphyseal dysplasia tarda and osteopenia are coexisting in the literature, there is no evidence of the association of spondyloepiphyseal dysplasia tarda and conjunctiva with osteoporosis. In our case report, we presented a patient who was applied to our center with short stature, diagnosed with osteoporosis and spondyloepiphyseal dysplasia congenita by detecting femur head aplasia in radiological imaging.

  15. Innovative Approaches to Regenerate Enamel and Dentin

    Directory of Open Access Journals (Sweden)

    Xanthippi Chatzistavrou

    2012-01-01

    Full Text Available The process of tooth mineralization and the role of molecular control of cellular behavior during embryonic tooth development have attracted much attention the last few years. The knowledge gained from the research in these fields has improved the general understanding about the formation of dental tissues and the entire tooth and set the basis for teeth regeneration. Tissue engineering using scaffold and cell aggregate methods has been considered to produce bioengineered dental tissues, while dental stem/progenitor cells, which can differentiate into dental cell lineages, have been also introduced into the field of tooth mineralization and regeneration. Some of the main strategies for making enamel, dentin, and complex tooth-like structures are presented in this paper. However, there are still significant barriers that obstruct such strategies to move into the regular clinic practice, and these should be overcome in order to have the regenerative dentistry as the important mean that can treat the consequences of tooth-related diseases.

  16. Sealing Occlusal Dentin Caries in Permanent Molars

    DEFF Research Database (Denmark)

    Qvist, Vibeke; Borum, Mette Kit; Møller, Kirsten Dynes

    2017-01-01

    The purpose of this study was to investigate the possibility of postponing restorative intervention of manifest occlusal caries in young, permanent dentition by non-invasive sealing. This RCT-designed study included 521 occlusal lesions in 521 patients aged 6 to 17 y. Based on clinical and radiog......The purpose of this study was to investigate the possibility of postponing restorative intervention of manifest occlusal caries in young, permanent dentition by non-invasive sealing. This RCT-designed study included 521 occlusal lesions in 521 patients aged 6 to 17 y. Based on clinical...... longevity of sealings and restorations until retreatments, and 3) compare effectiveness of sealings and restorations to halt caries progression in sealed lesions and beneath restorations. Furthermore, we aimed to identify factors influencing longevity and the effectiveness of sealings and restorations.......001). The median survival time for sealings not replaced by restorations was 7.3 y (CI, 6.4 y to NA). Survival of sealings was increased in patients with low caries risk and/or excellent oral hygiene, second molars compared with first molars, and lesions not extending the middle one-third of the dentin. Survival...

  17. Florid cemento-osseous dysplasia (FCOD: case report

    Directory of Open Access Journals (Sweden)

    Tai-Min Lin

    2010-12-01

    Full Text Available Florid cemento-osseous dysplasia (FCOD is a type of fibro-osseous lesion and represents a reactive process in which normal bone is replaced by poorly cellularized cementum-like materials and cellular fibrous connective tissues. It is strictly localized to the tooth-bearing or edentulous areas, often occurring bilaterally with symmetric involvements. In this case report, a mixed radiolucent and radiopaque lesion at the periapical area of the right mandibular second molar of a 46-year-old female patient was misdiagnosed as chronic apical periodontitis initially by clinical manifestation and radiographic finding on a periapical radiograph. The tooth #47 was a distal abutment tooth of a three-unit bridge from teeth #45–#47. No decay of teeth #45 and #47 was found when the bridge was removed. Electric pulp test revealed that the tooth #47 was a vital-pulp tooth. Panoramic radiography showed a similar mixed radiolucent and radiopaque lesion at the edentulous area of tooth #37. The symmetric distribution of the two lesions with no alveolar bone expansion and the positive vitality test of the involved tooth supported the diagnosis of FCOD for this case. No endodontic treatment or surgical biopsy was performed. The patient was instructed to undergo regular clinical and radiographic follow-up to monitor the change of the lesion. We suggest that when a radiolucent or mixed lesion occurs at the periapical area of a vital-pulp tooth, panoramic radiography may help differentiate an inflammatory periapical lesion from a lesion of cemento-osseous dysplasia (COD.

  18. Autosomal dominant craniometaphyseal dysplasia with atypical features.

    Science.gov (United States)

    McKay, D R; Fialkov, J A

    2002-03-01

    Craniometaphyseal dysplasia (CMD) is a rare genetic disorder of bone modelling characterised by hyperostosis and sclerosis of the craniofacial bones, and abnormal modelling of the metaphyses. Clinically, autosomal dominant (AD) CMD is characterised by facial distortion and cranial-nerve compression. The goals of surgical treatment for AD CMD are cosmetic recontouring of the sclerotic craniofacial bones, correction of nasal obstruction and correction or prevention of neurological manifestations. We describe the successful correction of AD CMD craniofacial manifestations in an individual with atypical findings, and outline an approach for correcting the craniofacial deformities associated with this rare disorder. Copyright 2002 The British Association of Plastic Surgeons.

  19. Fibromuscular Dysplasia Presenting with Bilateral Renal Infarction

    International Nuclear Information System (INIS)

    Doody, O.; Adam, W. R.; Foley, P. T.; Lyon, S. M.

    2009-01-01

    Fibromuscular dysplasia (FMD) describes a group of conditions which cause nonatheromatous arterial stenoses, most commonly of the renal and carotid arteries, typically in young women. We report a rare case of bilateral segmental renal infarction secondary to FMD in a young male patient. His initial presentation with loin pain and pyrexia resulted in a delay in the definitive diagnosis of FMD. He was successfully treated with bilateral balloon angioplasty. The delayed diagnosis in this patient until the condition had progressed to bilateral renal infarcts highlights the need for prompt investigation and diagnosis of suspected cases of FMD.

  20. The natural history of developmental dysplasia of the hip: sonographic findings in infants of 1-3 months of age

    NARCIS (Netherlands)

    Roovers, E.A.; Boere-Boonekamp, Magdalena M.; Mostert, Adriaan K.; Castelein, René M.; Zielhuis, Gerhard A.; Kerkhoff, Antoon

    2005-01-01

    The natural history of sonographic developmental dysplasia of the hip was determined in a population-based study in which 5170 infants were screened by ultrasound using Graf's method. Of the normal hips at the age of 1 month, 99.6% were still normal at the age of 3 months. Of the immature type

  1. Fibrous dysplasia with cartilaginous differentiation (''fibrocartilaginous dysplasia''): a review, with an illustrative case followed for 18 years

    International Nuclear Information System (INIS)

    Kyriakos, Michael; McDonald, Douglas J.; Sundaram, Murali

    2004-01-01

    A 21-year-old man with an 18-year history of progressive, and deforming, monomelic fibrous dysplasia with massive cartilaginous differentiation (fibrocartilaginous dysplasia) is described. A review is made of all prior reported examples of this entity in the English language medical literature. The radiologic and histologic differential diagnoses are described, distinguishing the lesion from chondrosarcoma and from fibrocartilaginous mesenchymoma. (orig.)

  2. DNA Fingerprinting Abnormalities Can Distinguish Ulcerative Colitis Patients with Dysplasia and Cancer from Those Who Are Dysplasia/Cancer-Free

    Science.gov (United States)

    Chen, Ru; Rabinovitch, Peter S.; Crispin, David A.; Emond, Mary J.; Koprowicz, Kent M.; Bronner, Mary P.; Brentnall, Teresa A.

    2003-01-01

    Patients with extensive ulcerative colitis (UC) of longer than 8 years duration are at high risk for the development of colorectal cancer. The cancers in these patients appear to develop in a stepwise manner with progressive histological changes from negative for dysplasia → indefinite for dysplasiadysplasia → cancer. The aim of this study was to determine the timing and extent of genomic instability in the progression of UC dysplasia and cancer. Using two polymerase chain reaction (PCR)-based DNA fingerprinting methods, arbitrarily primed PCR and intersimple sequence repeat PCR, we assessed DNA sequence variation in biopsies across the spectrum of cancerous, dysplastic, and nondysplastic mucosa. UC patients with dysplasia/cancer had substantial genomic instability in both their dysplastic and nondysplastic colonic mucosa, whereas instability was not present in the majority of UC patients without dysplasia/cancer. The degree of instability in nondysplastic tissue was similar to that of dysplastic/cancerous mucosa from the same patient, suggesting that this instability was widespread and reached the maximum level early in neoplastic progression. These results suggest that UC patients who develop dysplasia or cancer have an underlying process of genomic instability in their colonic mucosa whereas UC patients who are dysplasia-free do not. PMID:12547724

  3. Repair Responses of Dental Pulp to Tooth Injury and Biological Properties of Dentin-pulp Complex

    OpenAIRE

    大島, 勇人; Ohshima, Hayato

    2004-01-01

    Regeneration-the creation of a new tissue after the original one has been lost-is the fundamental biological capability in an organism. Numerous organs are considered to contain stem cells referred to as adult stem cells, even in the adult. Adult stem cells can give rise to a limited set of adult tissue types. In the field of clinical dentistry, it is well-known that the dentin-pulp complex is capable of repair after tooth injuries such as tooth replantation/transplantation or restorative pro...

  4. The effect of air thinning on dentin adhesive bond strength.

    Science.gov (United States)

    Hilton, T J; Schwartz, R S

    1995-01-01

    The purpose of this study was to determine if air thinning three dentin adhesives would affect bond strength to dentin. Ninety human molars were mounted in acrylic and the occlusal surfaces ground to expose a flat dentin surface. Thirty teeth were randomly assigned to one of the following dentin bonding agent/composite combinations: A) Universal Bond 3/TPH (Caulk), B) All-Bond 2/Bis-Fil-P (Bisco), and C) Scotchbond Multi-Purpose/Z-100 (3m). The primers were applied following the manufacturers' instructions. The adhesives were applied by two methods. A thin layer of adhesive was applied with a brush to 15 specimens in each group and light cured. Adhesive was brushed on to the remaining 15 teeth in the group, air thinned for 3 seconds, and then polymerized. The appropriate composite was applied in 2 mm increments and light cured utilizing a 5 mm-in-diameter split Teflon mold. Following 3 months of water storage, all groups were shear tested to failure on an Instron Universal Testing Machine. Bond strength was significantly higher in all groups when the dentin bonding agent was painted on without being air thinned. Scotchbond Multi-Purpose had significantly higher bond strength than All-Bond 2, which had significantly higher bond strength than Universal Bond 3.

  5. EFFECT OF SURFACE CONDTIONINGON BOND STRENGTH TO ENAMEL AND DENTIN

    Directory of Open Access Journals (Sweden)

    M MOUSAVINASAB

    2002-09-01

    Full Text Available Introduction. Compoglass is a trade mark of dental compomers and because of its partially resinus structure, surface conditioning of dental surfaces is needed for a better bonding process. In this study, the effect of enamel and dentin conditioning procedure on shear bond strength (SBS of compoglass to tooth surfaces was studied. Methods. four groups each one including 11 sound premolars were chosen and their surfaces were prepared as following groups: group1, unconitioned dentin; group 2, dentin conditioning with phosphoric acid 35%; group 3, dentin conditioning with polyacrylic acid 20% group 4, unconditioning enamel; group 5, enamel conditioning with phosphoric acid 35%; and group 6, enamel conditioning with polyacrylic acid 20%. Compoglass was bonded to prepared surfaces and after fixation of the samples in acrylic molds, all samples were tested under shear force of instron testing machine at a rate of 1 mm/min speed. Results. The mean SBS obtained in these 6 groups were 6.207, 8.057, 10.146, 25.939 and 11.827 mpa. the mode of fracture also studied using a streomicroscope. Statistical analysis of the results showed that the maximum SBS obtained in group 5 and the lowest SBS about 6.207 mpa obtained in group 1. Despite increase in SBS group 2 and 3, there was no statistical differncies between group 1, 2 and 3. Discussion. Based on results of this study, conditioning of enamel and dentin surface due to improve SBS is recommeneded.

  6. Enamel and dentin bond strength following gaseous ozone application.

    Science.gov (United States)

    Cadenaro, Milena; Delise, Chiara; Antoniollo, Francesca; Navarra, Ottavia Chiara; Di Lenarda, Roberto; Breschi, Lorenzo

    2009-08-01

    To evaluate the effects of gaseous ozone application on enamel and dentin bond strength produced by two self-etching adhesive systems. The shear bond strength test was conducted to assess adhesion on enamel (protocol 1), while the microtensile bond strength test was performed on dentin (protocol 2). Protocol 1: 96 bovine incisors were randomly divided into 4 groups, and enamel surfaces were bonded in accordance with the following treatments: (1E) ozone + Clearfil Protect Bond; (2E) Clearfil Protect Bond (control); (3E) ozone + Xeno III; (4E) Xeno III (control). Ozone gas was applied for 80 s. Shear bond strength was measured with a universal testing machine. Protocol 2: 40 noncarious human molars were selected. Middle/deep dentin was exposed and bonded in accordance with the following treatments: (1D) ozone+Clearfil Protect Bond; (2D) Clearfil Protect Bond (control); (3D) ozone+Xeno III (4D) Xeno III (control). Four-mm-thick buildups were built on the adhesives, then specimens were sectioned in accordance with the nontrimming technique. Specimens were stressed until failure occurred, and failure modes were analyzed. Shear bond and microtensile bond strength data were analyzed using two-way ANOVA and Tukey's post-hoc test. No statistical differences were found between ozone treated specimens and controls, neither on enamel nor on dentin irrespective of the tested adhesive. Clearfil Protect Bond showed higher bond strength to enamel than Xeno III, irrespective of the ozone treatment (p enamel and dentin bond strength.

  7. Dentin and dental pulp regeneration by the patient's endogenous cells.

    Science.gov (United States)

    Kim, Sahng G; Zheng, Ying; Zhou, Jian; Chen, Mo; Embree, Mildred C; Song, Karen; Jiang, Nan; Mao, Jeremy J

    2013-03-01

    The goal of regenerative endodontics is to restore the functions of the dental pulp-dentin complex. Two approaches are being applied toward dental pulp-dentin regeneration: cell transplantation and cell homing. The majority of previous approaches are based on cell transplantation by delivering ex vivo cultivated cells toward dental pulp or dentin regeneration. Many hurdles limit the clinical translation of cell transplantation such as the difficulty of acquiring and isolating viable cells, uncertainty of what cells or what fractions of cells to use, excessive cost of cell manipulation and transportation, and the risk of immune rejection, pathogen transmission, and tumorigenesis in associated with ex vivo cell manipulation. In contrast, cell homing relies on induced chemotaxis of endogenous cells and therefore circumvents many of the difficulties that are associated with cell transplantation. An array of proteins, peptides, and chemical compounds that are yet to be identified may orchestrate endogenous cells to regenerate dental pulp-dentin complex. Both cell transplantation and cell homing are scientifically valid approaches; however, cell homing offers a number of advantages that are compatible with the development of clinical therapies for dental pulp-dentin regeneration.

  8. New Classification of Focal Cortical Dysplasia: Application to Practical Diagnosis

    Science.gov (United States)

    Bae, Yoon-Sung; Kang, Hoon-Chul; Kim, Heung Dong; Kim, Se Hoon

    2012-01-01

    Background and Purpose: Malformation of cortical development (MCD) is a well-known cause of drug-resistant epilepsy and focal cortical dysplasia (FCD) is the most common neuropathological finding in surgical specimens from drug-resistant epilepsy patients. Palmini’s classification proposed in 2004 is now widely used to categorize FCD. Recently, however, Blumcke et al. recommended a new system for classifying FCD in 2011. Methods: We applied the new classification system in practical diagnosis of a sample of 117 patients who underwent neurosurgical operations due to drug-resistant epilepsy at Severance Hospital in Seoul, Korea. Results: Among 117 cases, a total of 16 cases were shifted to other FCD subtypes under the new classification system. Five cases were reclassified to type IIIa and five cases were categorized as dual pathology. The other six cases were changed within the type I category. Conclusions: The most remarkable changes in the new classification system are the advent of dual pathology and FCD type III. Thus, it will be very important for pathologists and clinicians to discriminate between these new categories. More large-scale research needs to be conducted to elucidate the clinical influence of the alterations within the classification of type I disease. Although the new FCD classification system has several advantages compared to the former, the correlation with clinical characteristics is not yet clear. PMID:24649461

  9. Different methods of dentin processing for application in bone tissue engineering: A systematic review.

    Science.gov (United States)

    Tabatabaei, Fahimeh Sadat; Tatari, Saeed; Samadi, Ramin; Moharamzadeh, Keyvan

    2016-10-01

    Dentin has become an interesting potential biomaterial for tissue engineering of oral hard tissues. It can be used as a scaffold or as a source of growth factors in bone tissue engineering. Different forms of dentin have been studied for their potential use as bone substitutes. Here, we systematically review different methods of dentin preparation and the efficacy of processed dentin in bone tissue engineering. An electronic search was carried out in PubMed and Scopus databases for articles published from 2000 to 2016. Studies on dentin preparation for application in bone tissue engineering were selected. The initial search yielded a total of 1045 articles, of which 37 were finally selected. Review of studies showed that demineralization was the most commonly used dentin preparation process for use in tissue engineering. Dentin extract, dentin particles (tooth ash), freeze-dried dentin, and denatured dentin are others method of dentin preparation. Based on our literature review, we can conclude that preparation procedure and the size and shape of dentin particles play an important role in its osteoinductive and osteoconductive properties. Standardization of these methods is important to draw a conclusion in this regard. © 2016 Wiley Periodicals, Inc. J Biomed Mater Res Part A: 104A: 2616-2627, 2016. © 2016 Wiley Periodicals, Inc.

  10. Modification of dentin surface to enamel-like structure: A potential strategy for improving dentin bonding durability, desensitizing and self-repairing

    Directory of Open Access Journals (Sweden)

    Hongye Yang

    2014-01-01

    Full Text Available Introduction: Current theories of dentin bonding are based on the concept of "hybrid layer". However, the histological complexity of dentin, as well as the vulnerability of the hybrid layer, goes against the long-term effect of dentin bonding. At the same time, post-operative sensitivity is more likely to occur after traditional adhesive restoration. The Hypothesis: Compared to dentin bonding, enamel bonding exhibits a more optimal immediate and long-term performance, owing to its higher degree of mineralization, well-arranged enamel crystals and the porous structure after etching. Moreover, "enamel hypersensitivity" is never going to happen due to the lack of tubules existing in dentin. In light of this phenomenon, we brought up the concept and the proposal method to form an "enamel-like" dentin, simulating enamel structure to achieve satisfying durability of dentin bonding and obtain good performance for preventing post-operative sensitivity. With the application of mesoporous silicon bi-directionally binding to hydroxyapatite of dentin itself and hydroxyapatite nanorods synthetized in vitro, we may be able to form an enamel-like "functional layer" via ion-regulating self-assembly. Evaluation of Hypothesis: This paper explains how to achieve dentin enamel-like modification by chemical methods, especially, details the strategies and possible mechanisms of the hypothesis. Validation of the hypothesis is more likely to eliminate the adverse effect of dentinal fluid, improve long-term performance of dentin bonding, offer strategies for desensitizing treatment and self-repairing carious-affected dentin, and furthermore, provide the possibility to introduce new theories of dentin bonding.

  11. Arrhythmogenic right ventricular dysplasia: MRI findings

    International Nuclear Information System (INIS)

    Wall, E.E. van der; Bootsma, M.M.; Schalij, M.J.; Kayser, H.W.M.; Roos, A. de

    2000-01-01

    Arrhythmogenic right ventricular dysplasia (ARVD) is a heart muscle disorder of unknown cause that is characterized pathologically by fibrofatty replacement of the right ventricular myocardium. Clinical manifestations include structural and functional malformations of the right ventricle, electrocardiographic abnormalities, and presentation with ventricular tachycardias with left bundle branch pattern or sudden death. The disease is often familial with an autosomal inheritance. In addition to right ventricular dilatation, right ventricular aneurysms are typical deformities of ARVD and they are distributed in the so-called ''triangle of dysplasia'', i. e., right ventricular outflow tract, apex, and infundibulum. Ventricular aneurysms at these sites can be considered pathognomonic of ARVD. Another typical hallmark of ARVD is fibrofatty infiltration of the right ventricular free wall. These functional and morphologic characteristics are relevant to clinical imaging investigations such as contrast angiography, echocardiography, radionuclide angiography, ultrafast computed tomography, and magnetic resonance imaging (MRI). Among these techniques, MRI allows the clearest visualization of the heart, in particular because the right ventricle is involved, which is usually more difficult to explore with the other imaging modalities. Furthermore, MRI offers the specific advantage of visualizing adipose infiltration as a bright signal of the right ventricular myocardium. MRI provides the most important anatomic, functional, and morphologic criteria for diagnosis of ARVD within one single study. As a result, MRI appears to be the optimal imaging technique for detecting and following patients with clinical suspicion of ARVD. (orig.) [de

  12. Mondini dysplasia and congenital cytomegalovirus infection.

    Science.gov (United States)

    Bauman, N M; Kirby-Keyser, L J; Dolan, K D; Wexler, D; Gantz, B J; McCabe, B F; Bale, J F

    1994-01-01

    We report a case of bilateral temporal bone anomalies in a child with symptomatic congenital cytomegalovirus infection and severe, bilateral sensorineural hearing loss identified at 3 months of age. High-resolution temporal bone computed tomography (HRCT) revealed bilateral findings of a short, malformed cochlea lacking an interscalar septum, a short and wide internal auditory canal, and an enlarged vestibular aqueduct, features diagnostic of bilateral Mondini dysplasia. To determine the importance of this observation, we completed HRCT in five additional children between 7 months and 9 years of age who had evidence of symptomatic congenital cytomegalovirus infection. One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplasia with a small cochlea lacking an interscalar septum, an abnormal vestibule, and a large cochlear aqueduct. Of the remaining four children, hearing thresholds ranged from normal to profoundly decreased, but their HRCT scans were normal to visual inspection. When inner ear dimensions of these temporal bones were compared with norms established by Pappas and coworkers, however, seven of the eight ears had short cochleas and narrow lateral semicircular canals, and three ears had short or narrow vestibules. These results indicate that congenital cytomegalovirus infection may cause anomalies or growth disturbances of the temporal bone.

  13. Placental Mesenchymal Dysplasia: A Case Report

    Directory of Open Access Journals (Sweden)

    Rachna Agarwal

    2012-01-01

    Full Text Available Introduction. A rare case of histologically proven placental mesenchymal dysplasia (PMD with fetal omphalocele in a 22-year-old patient is reported. Material and Methods. Antenatal ultrasound of this patient showed hydropic placenta with a live fetus of 17 weeks period of gestation associated with omphalocele. Cordocentesis detected the diploid karyotype of the fetus. Patient, when prognosticated, choose to terminate the pregnancy in view of high incidence of fetal and placental anomalies. Subsequent histopathological examination of placenta established the diagnosis to be placental mesenchymal dysplasia. Conclusion. On clinical and ultrasonic grounds, suspicion of P.M.D. arises when hydropic placenta with a live fetus presents in second trimester of pregnancy. Cordocentesis can detect the diploid karyotype of the fetus in such cases. As this condition is prognostically better than triploid partial mole, continuation of pregnancy can sometimes be considered after through antenatal screening and patient counseling. However, a definite diagnosis of P.M.D. is made only on placental histology by absence of trophoblast hyperplasia and trophoblastic inclusions.

  14. Trochleoplasty in major trochlear dysplasia: current concepts

    Directory of Open Access Journals (Sweden)

    Beaufils Philippe

    2012-02-01

    Full Text Available Abstract Trochleoplasty is the theoretical solution to persistent symptoms (pain and/or instability related to trochlear dysplasia where there is not only a trochlear flatness but also a trochlear prominence. The threshold of prominence indicating surgical intervention has as yet not been determined. A bump of 5 mm is generally accepted as the inferior limit. Given the interventional nature of this demanding procedure, it should be proposed in selected cases after considerable discussion with the patient. Trochleoplasty is indicated as a primary procedure for major trochlear dysplasia with a prominence > 5 mm. Stabilization is obtained in most of the cases with the risk of residual mild anterior knee pain. It is also indicated as a salvage procedure when a previous surgery failed. Despite the reputation of the procedure, the published results are encouraging in terms of prevention of re-dislocation, satisfaction index, and radiological outcomes. Post-operative stiffness is the main complication, which may require manipulation under anaesthesia or arthroscopic arthrolysis. There are few other complications reported and to date secondary necrosis of the trochlea has not been reported. Technically speaking, the deepening trochleoplasty is a difficult procedure without reliable landmarks. We propose a recession wedge trochleoplasty which is an easier procedure. It is never undertaken as an isolated procedure, but always in conjunction with other realignment procedures of the extensor apparatus according to the "a la carte" surgery concept.

  15. Septo-optic dysplasia with pachygyria

    Directory of Open Access Journals (Sweden)

    Abhay A Lune

    2014-01-01

    Full Text Available Optic nerve hypoplasia can be associated with other central nervous system malformations. When associated with the absence of the septum pellucidum or thinning of corpus callosum and pituitary hypoplasia, it is termed septo-optic dysplasia. Pachygyria is a rare congenital disorder characterized by a defect in migration of cerebral neurons resulting in failure of cortical gyri to develop.We report a case of a young learning-disabled male presenting with decreased vision, nystagmus, esotropia,, and seizures since childhood. Fundoscopy revealed bilateral optic nerve hypoplasia. Growth hormone levels were reduced. Magnetic resonance imaging revealed thinned optic nerves, optic chiasma and pituitary infundibulum, absent septum pellucidum, box-shaped ventricles and right frontal cortical thickening with few and broad gyri.This case report highlights a rare case of septo-optic dysplasia with pachygyria. It also emphasizes that every patient with optic nerve hypoplasia should have an endocrinal and neurological evaluation and neuro-radiographic imaging for associated abnormalities.

  16. Effect of sonic application mode on the resin-dentin bond strength and dentin permeability of self-etching systems.

    Science.gov (United States)

    Mena-Serrano, Alexandra; Costa, Thays Regina Ferreira da; Patzlaff, Rafael Tiago; Loguercio, Alessandro Dourado; Reis, Alessandra

    2014-10-01

    To compare manual and sonic adhesive application modes in terms of the permeability and microtensile bond strength of a self-etching adhesive applied in the one-step or two-step protocol. Self-etching All Bond SE (Bisco) was applied as a one- or a two-step adhesive under manual or sonic vibration modes on flat occlusal dentin surfaces of 64 human molars. Half of the teeth were used to measure the hydraulic conductance of dentin at 200 cm H₂O hydrostatic pressure for 5 min immediately after the adhesive application. In the other half, composite buildups (Opallis) were constructed incrementally to create resin-dentin sticks with a cross-sectional area of 0.8 mm² to be tested in tension (0.5 mm/min) immediately after restoration placement. Data were analyzed using a two-way ANOVA and Tukey's test (α = 0.05). The fluid conductance of dentin was significantly reduced by the sonic vibration mode for both adhesives, but no effect on the bond strength values was observed for either adhesive. The sonic application mode at an oscillating frequency of 170 Hz can reduce the fluid conductance of the one- and two-step All Bond SE adhesive when applied on dentin.

  17. Craniofacial morphometric analysis of individuals with X-linked hypohidrotic ectodermal dysplasia.

    Science.gov (United States)

    Goodwin, Alice F; Larson, Jacinda R; Jones, Kyle B; Liberton, Denise K; Landan, Maya; Wang, Zhifeng; Boekelheide, Anne; Langham, Margaret; Mushegyan, Vagan; Oberoi, Snehlata; Brao, Rosalie; Wen, Timothy; Johnson, Ramsey; Huttner, Kenneth; Grange, Dorothy K; Spritz, Richard A; Hallgrímsson, Benedikt; Jheon, Andrew H; Klein, Ophir D

    2014-09-01

    Hypohidrotic ectodermal dysplasia (HED) is the most prevalent type of ectodermal dysplasia (ED). ED is an umbrella term for a group of syndromes characterized by missing or malformed ectodermal structures, including skin, hair, sweat glands, and teeth. The X-linked recessive (XL), autosomal recessive (AR), and autosomal dominant (AD) types of HED are caused by mutations in the genes encoding ectodysplasin (EDA1), EDA receptor (EDAR), or EDAR-associated death domain (EDARADD). Patients with HED have a distinctive facial appearance, yet a quantitative analysis of the HED craniofacial phenotype using advanced three-dimensional (3D) technologies has not been reported. In this study, we characterized craniofacial morphology in subjects with X-linked hypohidrotic ectodermal dysplasia (XLHED) by use of 3D imaging and geometric morphometrics (GM), a technique that uses defined landmarks to quantify size and shape in complex craniofacial morphologies. We found that the XLHED craniofacial phenotype differed significantly from controls. Patients had a smaller and shorter face with a proportionally longer chin and midface, prominent midfacial hypoplasia, a more protrusive chin and mandible, a narrower and more pointed nose, shorter philtrum, a narrower mouth, and a fuller and more rounded lower lip. Our findings refine the phenotype of XLHED and may be useful both for clinical diagnosis of XLHED and to extend understanding of the role of EDA in craniofacial development.

  18. Temperature changes across CO2-lased dentin during multiple exposures

    Science.gov (United States)

    Zakariasen, Kenneth L.; Barron, Joseph R.; Boran, Thomas L.

    1990-06-01

    The literature increasingly indicates that lasers will have a multitude of applications for dental hard tissue procedures, e.g. preventive therapy, caries removal, laser etching and endodontic therapy. However, it is critical that such laser therapies avoid the production of heat levels which will be damaging to the surrounding vital tissues, such as the dental pulp and periodontal tissues. Our preliminary research on temperature changes across C02 lased dentin indicated that for single preventive therapeutic exposures (1.2 W., 0. 1 sec., 1.0 mm focal spot) the mean temperature rise across 350 j.tm of dentin was 0.57 0C while across 1000 .tm of dentin the mean rise was only 0.18 °C. Further research utilizing multiple preventive therapeutic exposures (1.2 W., 0. 1 sec., 1.0 mm focal spot, 3 x 1.0 sec. intervals) showed mean temperature elevations of 1.56 0C across 350 m of dentin and 0.66 O across 1000 xm of dentin. While these temperature elevations, which would be associated with preventive therapy, are very low and would be biologically acceptable, it must be noted that exposures of higher intensities are required to fuse enamel and porcelain, or remove decay. This current research investigates temperature elevations which occuT during C02 lasing utilizing the following exposure parameters: 8.0 W., 1.0 mm focal spot, 0.1 sec. exposures, 2 or 4 exposures per site pulsed 1.0 sec. apart. Three dentin thicknesses were utilized, i.e. 1000 jim, 1500 p.tm and 2000 .tm. Four sections of each thickness were utilized with four exposure sites per specimen (2 with 2 exposures, 2 with 4 exposures). All dentin sections were prepared from non-carious third molars using a hard tissue microtome. A thermistor was placed on the dentin surface opposite each lased site and temperature changes were recorded for approximately 50 sec. following lasing. Mean temperature elevations ranged from a high of 3.07 C for the 1000 xm section utilizing four exposures to a low of 0.37 0C for the

  19. A study of the clinical and radiological features in a cohort of 93 patients with a COL2A1 mutation causing spondyloepiphyseal dysplasia congenita or a related phenotype

    NARCIS (Netherlands)

    Terhal, Paulien A.; Nievelstein, Rutger Jan A. J.; Verver, Eva J. J.; Topsakal, Vedat; van Dommelen, Paula; Hoornaert, Kristien; Le Merrer, Martine; Zankl, Andreas; Simon, Marleen E. H.; Smithson, Sarah F.; Marcelis, Carlo; Kerr, Bronwyn; Clayton-Smith, Jill; Kinning, Esther; Mansour, Sahar; Elmslie, Frances; Goodwin, Linda; van der Hout, Annemarie H.; Veenstra-Knol, Hermine E.; Herkert, Johanna C.; Lund, Allan M.; Hennekam, Raoul C. M.; Mégarbané, André; Lees, Melissa M.; Wilson, Louise C.; Male, Alison; Hurst, Jane; Alanay, Yasemin; Annerén, Göran; Betz, Regina C.; Bongers, Ernie M. H. F.; Cormier-Daire, Valerie; Dieux, Anne; David, Albert; Elting, Mariet W.; van den Ende, Jenneke; Green, Andrew; van Hagen, Johanna M.; Hertel, Niels Thomas; Holder-Espinasse, Muriel; den Hollander, Nicolette; Homfray, Tessa; Hove, Hanne D.; Price, Susan; Raas-Rothschild, Annick; Rohrbach, Marianne; Schroeter, Barbara; Suri, Mohnish; Thompson, Elizabeth M.; Tobias, Edward S.; Toutain, Annick; Vreeburg, Maaike; Wakeling, Emma; Knoers, Nine V.; Coucke, Paul; Mortier, Geert R.

    2015-01-01

    Type 2 collagen disorders encompass a diverse group of skeletal dysplasias that are commonly associated with orthopedic, ocular, and hearing problems. However, the frequency of many clinical features has never been determined. We retrospectively investigated the clinical, radiological, and genotypic

  20. A Study of the Clinical and Radiological Features in a Cohort of 93 Patients with a COL2A1 Mutation Causing Spondyloepiphyseal Dysplasia Congenita or a Related Phenotype

    NARCIS (Netherlands)

    Terhal, Paulien A.; Nievelstein, Rutger Jan A. J.; Verver, Eva J. J.; Topsakal, Vedat; van Dommelen, Paula; Hoornaert, Kristien; Le Merrer, Martine; Zankl, Andreas; Simon, Marleen E. H.; Smithson, Sarah F.; Marcelis, Carlo; Kerr, Bronwyn; Clayton-Smith, Jill; Kinning, Esther; Mansour, Sahar; Elmslie, Frances; Goodwin, Linda; van der Hout, Annemarie H.; Veenstra-Knol, Hermine E.; Herkert, Johanna C.; Lund, Allan M.; Hennekam, Raoul C. M.; Megarbane, Andre; Lees, Melissa M.; Wilson, Louise C.; Male, Alison; Hurst, Jane; Alanay, Yasemin; Anneren, Goeran; Betz, Regina C.; Bongers, Ernie M. H. F.; Cormier-Daire, Valerie; Dieux, Anne; David, Albert; Elting, Mariet W.; van den Ende, Jenneke; Green, Andrew; van Hagen, Johanna M.; Hertel, Niels Thomas; Holder-Espinasse, Muriel; den Hollander, Nicolette; Homfray, Tessa; Hove, Hanne D.; Price, Susan; Raas-Rothschild, Annick; Rohrbach, Marianne; Schroeter, Barbara; Suri, Mohnish; Thompson, Elizabeth M.; Tobias, Edward S.; Toutain, Annick; Vreeburg, Maaike; Wakeling, Emma; Knoers, Nine V.; Coucke, Paul; Mortier, Geert R.

    Type 2 collagen disorders encompass a diverse group of skeletal dysplasias that are commonly associated with orthopedic, ocular, and hearing problems. However, the frequency of many clinical features has never been determined. We retrospectively investigated the clinical, radiological, and genotypic

  1. Effects of different desensitizing treatments on root dentin permeability

    Directory of Open Access Journals (Sweden)

    Raydsa Raíssa Moura ROSA

    Full Text Available Abstract The objective of this study was to evaluate the effects of diode laser and a desensitizing dentifrice on dentin permeability. Fifty-two root dentin fragments were obtained (5 × 5mm and treated with 24% EDTA gel. The samples were divided into 4 groups (n = 13: G1, control (no treatment; G2, diode laser (λ = 908 nm, 1.5 W, continuous mode, 20s; G3, application of abrasive dentifrice for 1 minute (Elmex Sensitive Professional (International Gaba; and G4, application of abrasive dentifrice for 1 minute followed by irradiation with diode laser. Ten samples per group were immersed in 2% methylene blue solution for 4h. The specimens were washed, longitudinally sectioned, observed under optical microscopy, photographed and assessed based on the degree of dye leakage. The remaining samples were observed under scanning electron microscopy (SEM. The leakage data were subjected to ANOVA test, followed by Tukey’s t-test (α = 5%. Groups 2, 3 and 4 showed less dye penetration than the control group (p < 0.05, but were similar among each other. SEM images showed that dentinal tubules were open in G1, and fused and occluded in G2. Group 3 showed dentinal tubules that were occluded by the metal ions from the toothpaste. G4 presented similar characteristics to G3, and the presence of fused dentin. The diode laser and the dentifrice were effective in reducing dentinal permeability, and the combination of the two treatments did not show better results than either one used alone.

  2. Relationship between flexible flat foot and developmental hip dysplasia.

    Science.gov (United States)

    Ponce de León Samper, M C; Herrera Ortiz, G; Castellanos Mendoza, C

    2015-01-01

    To evaluate the possible relationship between flexible flat foot and developmental hip dysplasia in children between six and 15 years of age. Cross-sectional study including 65 patients that had undergone surgery due to residual hip dysplasia or hip dislocation and compared against 75 healthy patients. Flexible flat foot prevalence was measured in each group, with the results showing that 61% of the group with residual hip dysplasia or hip dislocation had this condition, vs. 12% in the healthy group. The statistical analysis shows that the chances of suffering from flexible flat foot, are five times greater in the hip dysplasia or hip dislocation group, than in the healthy group. There is no evidence in the literature showing a relationship between these two conditions, even though they have a common etiology. This study shows a potential measurable relation between this two conditions. Patients with hip dysplasia or dislocation may have a higher chance of presenting flexible flat foot during late childhood, adolescence and adulthood, a fact that suggests a relationship between these two pathologies. Also, patients who seek assistance for the first time because of a flexible flat foot condition without having been evaluated during the first year of life for hip dysplasia, would be better off if evaluated for residual hip dysplasia. Copyright © 2014 SECOT. Published by Elsevier Espana. All rights reserved.

  3. Expanding the phenome and variome of skeletal dysplasia.

    Science.gov (United States)

    Maddirevula, Sateesh; Alsahli, Saud; Alhabeeb, Lamees; Patel, Nisha; Alzahrani, Fatema; Shamseldin, Hanan E; Anazi, Shams; Ewida, Nour; Alsaif, Hessa S; Mohamed, Jawahir Y; Alazami, Anas M; Ibrahim, Niema; Abdulwahab, Firdous; Hashem, Mais; Abouelhoda, Mohamed; Monies, Dorota; Al Tassan, Nada; Alshammari, Muneera; Alsagheir, Afaf; Seidahmed, Mohammed Zain; Sogati, Samira; Aglan, Mona S; Hamad, Muddathir H; Salih, Mustafa A; Hamed, Ahlam A; Alhashmi, Nadia; Nabil, Amira; Alfadli, Fatima; Abdel-Salam, Ghada M H; Alkuraya, Hisham; Peitee, Winnie Ong; Keng, W T; Qasem, Abdullah; Mushiba, Aziza M; Zaki, Maha S; Fassad, Mahmoud R; Alfadhel, Majid; Alexander, Saji; Sabr, Yasser; Temtamy, Samia; Ekbote, Alka V; Ismail, Samira; Hosny, Gamal Ahmed; Otaify, Ghada A; Amr, Khalda; Al Tala, Saeed; Khan, Arif O; Rizk, Tamer; Alaqeel, Aida; Alsiddiky, Abdulmonem; Singh, Ankur; Kapoor, Seema; Alhashem, Amal; Faqeih, Eissa; Shaheen, Ranad; Alkuraya, Fowzan S

    2018-04-05

    PurposeTo describe our experience with a large cohort (411 patients from 288 families) of various forms of skeletal dysplasia who were molecularly characterized.MethodsDetailed phenotyping and next-generation sequencing (panel and exome).ResultsOur analysis revealed 224 pathogenic/likely pathogenic variants (54 (24%) of which are novel) in 123 genes with established or tentative links to skeletal dysplasia. In addition, we propose 5 genes as candidate disease genes with suggestive biological links (WNT3A, SUCO, RIN1, DIP2C, and PAN2). Phenotypically, we note that our cohort spans 36 established phenotypic categories by the International Skeletal Dysplasia Nosology, as well as 18 novel skeletal dysplasia phenotypes that could not be classified under these categories, e.g., the novel C3orf17-related skeletal dysplasia. We also describe novel phenotypic aspects of well-known disease genes, e.g., PGAP3-related Toriello-Carey syndrome-like phenotype. We note a strong founder effect for many genes in our cohort, which allowed us to calculate a minimum disease burden for the autosomal recessive forms of skeletal dysplasia in our population (7.16E-04), which is much higher than the global average.ConclusionBy expanding the phenotypic, allelic, and locus heterogeneity of skeletal dysplasia in humans, we hope our study will improve the diagnostic rate of patients with these conditions.GENETICS in MEDICINE advance online publication, 5 April 2018; doi:10.1038/gim.2018.50.

  4. COHESIVE STRENGTH OF DENTIN RESISTÊNCIA COESIVA DA DENTINA

    Directory of Open Access Journals (Sweden)

    Flávio Fernando DEMARCO

    1997-07-01

    Full Text Available The bond strength of dentin adhesives to dentin has increased after each generation. Although dentin substratum is part of the bonding process, little importance has been given to measure dentin cohesive strength. The aim of this study was to evaluate the cohesive strength of dentin in human canines. Seventeen non carious canines were selected. All of them had been extracted for more than one year. The teeth were ground until dentin square samples with approximately 2 X 2 mm were obtained. They were embedded in acrylic resin and subjected to shear stress, in a Wolpert Machine, at a crosshead speed of 0.5 mm/min. The mean cohesive strength of dentin in shear mode was 33.95 (+-9.72 MPa. The fracture surfaces were observed under a X40 magnification. A finite element analysis was performed to observe the stress distribution as related to the shear test. The failure pattern was compatible with the shear test and also with the stress distribution in the finite element analysisA resistência de união dos adesivos dentinários tem sido aumentada com o desenvolvimento de cada nova geração. Pouca importância tem sido dada à resistência coesiva da dentina. A proposta deste estudo foi avaliar a resistência coesiva da dentina. Dezessete caninos humanos hígidos, os quais tinham sido extraídos há mais de um ano, foram usados. Os dentes foram desgastados até a obtenção de corpos-de-prova em dentina, de formato quadrangular, com tamanho aproximado de 2 X 2 mm. Os dentes foram incluídos em resina acrílica e, então, submetidos ao teste de cisalhamento em uma máquina de ensaios universais Wolpert, com uma velocidade de 0,5 mm/min. A resistência coesiva média da dentina no teste de cisalhamento foi de 33,95 (+- 9,72 MPa. O tipo de fratura foi analisado com um aumento de 40X. Foi realizada uma análise com elemento finito, para observar a distribuição do estresse relacionada com o teste de cisalhamento. O padrão de fratura encontrado foi compat

  5. Bonding of universal adhesives to dentine--Old wine in new bottles?

    Science.gov (United States)

    Chen, C; Niu, L-N; Xie, H; Zhang, Z-Y; Zhou, L-Q; Jiao, K; Chen, J-H; Pashley, D H; Tay, F R

    2015-05-01

    Multi-mode universal adhesives offer clinicians the choice of using the etch-and-rinse technique, selective enamel etch technique or self-etch technique to bond to tooth substrates. The present study examined the short-term in vitro performance of five universal adhesives bonded to human coronal dentine. Two hundred non-carious human third molars were assigned to five groups based on the type of the universal adhesives (Prime&Bond Elect, Scotchbond Universal, All-Bond Universal, Clearfil Universal Bond and Futurabond U). Two bonding modes (etch-and-rinse and self-etch) were employed for each adhesive group. Bonded specimens were stored in deionized water for 24h or underwent a 10,000-cycle thermocycling ageing process prior to testing (N=10). Microtensile bond testing (μTBS), transmission electron microscopy (TEM) of resin-dentine interfaces in non-thermocycled specimens and scanning electron microscopy (SEM) of tracer-infused water-rich zones within hybrid layers of thermocycled specimens were performed. Both adhesive type and testing condition (with/without thermocycling) have significant influences on μTBS. The use of each adhesive in either the etch-and-rinse or self-etch application mode did not result in significantly different μTBS to dentine. Hybrid layers created by these adhesives in the etch-and-rinse bonding mode and self-etch bonding mode were ∼5μm and ≤0.5μm thick respectively. Tracer-infused regions could be identified within the resin-dentine interface from all the specimens prepared. The increase in versatility of universal adhesives is not accompanied by technological advances for overcoming the challenges associated with previous generations of adhesives. Therapeutic adhesives with bio-protective and bio-promoting effects are still lacking in commercialized adhesives. Universal adhesives represent manufacturers' attempt to introduce versatility in product design via adaptation of a single-bottle self-etch adhesive for other application

  6. Prosthodontic management of a patient with ectodermal dysplasia.

    Science.gov (United States)

    Nandini, Yamini

    2013-12-01

    Ectodermal dysplasia is a rare congenital disease that affects the ectodermal structures. It is characterized by hypotrichosis, hypohidrosis and hypodontia. A 14-year-old boy with ectodermal dysplasia presenting with oligodontia and marked resorption of the maxillary and mandibular alveolar ridges is reported. Prosthetic rehabilitation in the form of a maxillary and mandibular partial denture was made with metal crowns on existing lower teeth to achieve appropriate vertical dimension. Significant improvement in speech, masticatory function and facial esthetics was achieved. Removable prosthodontics can provide an acceptable solution to esthetic, functional and psychological rehabilitation in patients with ectodermal dysplasia.

  7. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome

    Directory of Open Access Journals (Sweden)

    Reema Sharma Dhar

    2014-01-01

    Full Text Available Ectrodactyly-ectodermal dysplasia-cleft (EEC syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report.

  8. Ectrodactyly-ectodermal dysplasia-cleft lip and palate syndrome.

    Science.gov (United States)

    Dhar, Reema Sharma; Bora, Amitava

    2014-01-01

    Ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome is an autosomal dominant disorder characterized by the triad of ectrodactyly-ectodermal dysplasia, and facial clefting along with some associated features. Presence of all the three major features in a single individual is extremely rare. We report a case of 4 year 11 months old child with EEC syndrome having ectodermal dysplasia-cleft lip and cleft palate and ectrodactyly with some associated features. Clinical features, diagnosis and role of a dentist in the multidisciplinary treatment approach have been elaborated in this case report.

  9. Polypoidal Intestinal Metaplasia and Dysplasia of the External Urethral Meatus

    Directory of Open Access Journals (Sweden)

    Mary Mathew

    2012-01-01

    Full Text Available Urethral mucosa with intestinal metaplasia and dysplasia is a rare occurrence. To date only a single case has been reported in a male with long-standing urethral stricture. We present a 33-year-old female with polypoid intestinal metaplasia and dysplasia of the external urethral meatus in the absence of an inciting factor. Intestinal metaplasia of the urethral mucosa may undergo dysplasia, emphasizing the necessity of a high degree of clinical suspicion and vigilant pathological examination of these lesions.

  10. Focal cemento-osseous dysplasia masquerading as a residual cyst.

    Science.gov (United States)

    Bhandari, Rajat; Sandhu, Simarpreet V; Bansal, Himanta; Behl, Rashi; Bhullar, Ramanpreet Kaur

    2012-04-01

    Focal cemento-osseous dysplasia (FCOD) is a benign fibroosseous condition that can be seen in dentulous and edentulous patients. It is an asymptomatic lesion and needs no treatment; however, follow-up is essential due to the possibility that it can progress to a condition called florid cemento-osseous dysplasia. We report a case of FCOD of mandible in a 25-year-old female. Clinically, the lesion resembled periapical pathosis of odontogenic origin. An attempt has been made to discuss the clinical and histopathologic features along with differential diagnosis of cemento-osseous dysplasia.

  11. Focal cemento-osseous dysplasia masquerading as a residual cyst

    Directory of Open Access Journals (Sweden)

    Rajat Bhandari

    2012-01-01

    Full Text Available Focal cemento-osseous dysplasia (FCOD is a benign fibroosseous condition that can be seen in dentulous and edentulous patients. It is an asymptomatic lesion and needs no treatment; however, follow-up is essential due to the possibility that it can progress to a condition called florid cemento-osseous dysplasia. We report a case of FCOD of mandible in a 25-year-old female. Clinically, the lesion resembled periapical pathosis of odontogenic origin. An attempt has been made to discuss the clinical and histopathologic features along with differential diagnosis of cemento-osseous dysplasia.

  12. [Florid cemento-osseous dysplasia of the jaws].

    Science.gov (United States)

    Benazzou, S; Boulaadas, M; El Ayoubi, A; Nazih, N; Essakalli, L; Kzadri, M

    2011-06-01

    Florid cemento-osseous dysplasia is a benign and rare tumor of the jaws. It is more commonly seen in middle-aged black women. Most cases are asymptomatic and are found during routine radiographic examination. We report two complicated cases of florid cemento-osseous dysplasia, one with facial deformity and the other with chronic osteitis. The diagnosis of florid cemento-osseous dysplasia is based on clinical and radiological features. The lesions are commonly bilateral and symmetrical. Copyright © 2011. Published by Elsevier Masson SAS.

  13. [Protein-energy malnutrition in patients with connective tissue dysplasia].

    Science.gov (United States)

    Lialiukova, E A

    2013-01-01

    In the conditions of the specialized Center of a dysplasia of a connecting tissue the assessment of an protein--energy malnutrition at 121 patients with signs of a dysplasia of a connecting tissue is carried out. High frequency of an oligotrophy at patients with a dysplasia of a connecting tissue is registered. The I degree of a gipotorofiya is taped at 26.21% of the patients, II degree--at 18.44%, the III degree--at 3.88% of patients.

  14. Bilateral Cerebellar Cortical Dysplasia without Other Malformations: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Oh, Jung Seok; Ahn Kook Jin; Kim, Jee Young; Lee, Sun Jin; Park, Jeong Mi [Catholic University Yeouido St. Mary' s Hospital, College of Medicine, Seoul (Korea, Republic of)

    2010-06-15

    Recent advances in MRI have revealed congenital brain malformations and subtle developmental abnormalities of the cerebral and cerebellar cortical architecture. Typical cerebellar cortical dysplasia as a newly categorized cerebellar malformation, has been seen in patients with Fukuyama congenital muscular dystrophy. Cerebellar cortical dysplasia occurs at the embryonic stage and is often observed in healthy newborns. It is also incidentally and initially detected in adults without symptoms. To the best of our knowledge, cerebellar dysplasia without any related disorders is very rare. We describe the MRI findings in one patient with disorganized foliation of both cerebellar hemispheres without a related disorder or syndrome

  15. Radiological features of bilateral hereditary micro-epiphyseal dysplasia - a distinct entity in the skeletal dysplasias

    Energy Technology Data Exchange (ETDEWEB)

    Mostert, A.K. [Isala Clinics, Location Weezenlanden, Dept. of Orthopaedic Surgery, Zwolle (Netherlands); Dijkstra, P.F. [Jan van Breemen Inst., Dept. of Radiology, Amsterdam (Netherlands); Horn, J.R. van [Univ. Hospital Groningen, Dept. of Orthopaedic Surgery, Groningen (Netherlands); Jansen, B.R.H. [Reinier de Graaf Hospital, Dept. of Orthopaedic Surgery, Delft (Netherlands); Heutink, P. [Erasmus MCRotterdam, Dept. of Clinical Genetics, Rotterdam (Netherlands); Lindhout, D. [Univ. Medical Centre Utrecht, Dept. of Medical Genetics, Utrecht (Netherlands)

    2002-07-01

    Aim: To prove that bilateral hereditary micro-epiphyseal dysplasia (BHMED), first described by Elsbach in 1959, is a distinct disorder radiologically as well as clinically, compared with multiple epiphyseal dysplasia (MED). Material and Methods: We used the data of the revised pedigree with 84 family members, performed a medical history, physical examination and made a radiological evaluation for defining a clinical and radiological phenotype of BHMED family members. We used blood samples for genetic analysis. Results: Although there is a clear clinical picture of the dysplasia, the radiological signs are more reliable for making the diagnosis. Especially the typical deformity of the hip and knee joint are diagnostic for BHMED. By linkage analysis we excluded linkage with the three known MED-loci (EDM1, EDM2 and EDM3). Conclusion: BHMED is indeed an entity that is distinct from common multiple epiphyseal dysplasia (MED), clinically, as well as radiologically and genetically. (orig.) [German] Ziel: Es sollte dargelegt werden, dass sich eine vererbliche, laterale Mikro-Epiphysendysplasie (BHMED), Erstbeschreibung durch Elsbach 1959, klinisch, radiologisch und genetisch von einer mutiplen Epiphysendysplasie (MED) unterscheidet. Material und Methode: Anhand der Daten eines ueberarbeiteten Stammbaumes mit 84 Familienmitgliedern wurde der medizinische Werdegang rekonstruiert. Es erfolgte eine physische Untersuchung der Familienmitglieder. Schliesslich wurde eine radiologische Auswertung durchgefuehrt, um einen klinischen und radiologischen Phaenotyp der von BHMED betroffenen Familienmitglieder zu definieren. Fuer eine genetische Analyse wurden Blutproben entnommen. Ergebnisse: Obwohl es ein deutliches klinisches Bild einer Dysplasie gibt, sind die radiologischen Kennzeichen fuer die Diagnose zuverlaessiger. Insbesondere die typische Deformation der Huefte und des Kniegelenks ist diagnostisch fuer BHMED. Durch Linkage-Analyse konnte eine Verbindung zu den drei bekannten

  16. Triple pelvic osteotomy in the treatment of hip dysplasia

    Directory of Open Access Journals (Sweden)

    Vukašinović Zoran

    2009-01-01

    Full Text Available Introduction. Insufficient femoral head coverage is found in a variety of diseases, with acetabular dysplasia as the most frequent disorder and triple pelvic osteotomy as the most recently introduced surgical treatment. Objective. This study analyses pre- and postoperative pathoanatomic characteristics of triple in comparison to Salter and Chiari osteotomies, with a logistic regression analysis of outcome predictor and effect explanator factors in relation to the chosen type of operation. Methods. The study involved 136 adolescents treated with Salter and Chiari osteotomies or a triple pelvic osteotomy at the Institute of Orthopaedic Surgery 'Banjica' in Belgrade. The patients were between 10-20 years old at the time of operation. We collected and analyzed data from all the patients: illness history, operative parameters, preoperative and postoperative pathoanatomic data. The data was statistically processed using the statistical software SPSS, defining standard descriptive values, and by using the appropriate tests of analytic statistics: t-test for dependent and independent variables, χ2-test, Fisher's exact test, Wilcoxon's test, parameter correlation, one-way ANOVA, multi-factorial ANOVA and logistic regression, according to the type of the analyzed data and the conditions under which the statistical methods were applied. Results. The average CE angle after triple pelvic osteotomy was 43.5°, more improved than after the Salter osteotomy (33.0° and Chiari osteotomy (31.4° (F=16.822; p<0.01. Postoperative spherical congruence was also more frequent after the triple osteotomy than after the other two types of operations, and with a high significance. Preoperative painful discomfort was found to be a valid predictor of indications for the triple osteotomy over both Chiari and Salter osteotomies. The valid explanators of effect for the triple osteotomy are: postoperative joint congruence (compared to the Chiari osteotomy and increase in joint

  17. Influence of blood contamination during multimode adhesive application on the microtensile bond strength to dentin.

    Science.gov (United States)

    Kucukyilmaz, E; Celik, E U; Akcay, M; Yasa, B

    2017-12-01

    The present study evaluated the effects of blood contamination performed at different steps of bonding on the microtensile bond strength (μTBS) of multimode adhesives to dentin when using the self-etch approach. Seventy-five molars were randomly assigned to three adhesive groups comprising 25 specimens each: two multimode adhesives [Single Bond Universal (SBU) and All-Bond Universal (ABU)] and a conventional one-step self-etch adhesive [Clearfil S3 Bond Plus (CSBP)]. Each group was subdivided as follows: (1) uncontaminated (control): bonding application/light curing as a positive control; (2) contamination-1 (cont-1): bonding application/light curing/blood contamination/dry as a negative control; (3) contamination-2 (cont-2): bonding application/light curing/blood contamination/rinse/dry; (4) contamination-3 (cont-3): bonding application/blood contamination/dry/bonding re-application/light curing; and (5) contamination-4 (cont-4): bonding application/blood contamination/rinse/dry/bonding re-application/light curing. Dentin specimens were prepared for μTBS testing after the composite resin application. Data were analyzed with two-way ANOVA and post-hoc tests (α = 0.05). μTBS values were similar in cont-3 groups, and ABU/cont-4 and corresponding control groups, but were significantly lower in the other groups than in their control groups (P groups showed the lowest μTBS values (P blood contaminants and reapplying the adhesive may regain the dentin adhesion when contamination occurs before light curing. Alternatively, rinsing and drying contaminants followed by adhesive re-application may be effective depending on adhesive type.

  18. Transmission electron microscopical study of teenage crown dentin on the nanometer scale

    Energy Technology Data Exchange (ETDEWEB)

    Panfilov, Peter, E-mail: peter.panfilov@urfu.ru [Ural Federal University, Ekaterinburg (Russian Federation); Kabanova, Anna [Ural Federal University, Ekaterinburg (Russian Federation); Guo, Jinming; Zhang, Zaoli [Erich Schmid Institute for Materials Science, Austrian Academy of Sciences, Leoben (Austria)

    2017-02-01

    Statement of significance: This is the first transmission electron microscopic study of teenage crown dentin on the nanometer scale. Samples for TEM were prepared by mechanical thinning and chemical polishing that allowed obtaining the electron transparent foils. It was firstly shown that human dentin possesses the layered morphology: the layers are oriented normally to the main axis of a tooth and have the thickness of ~ 50 nm. HA inorganic phase of teenage crown dentin is in the amorphous state. The cellular structure, which was formed from collagen fibers (diameter is ~ 5 nm), are observed near DEJ region in teenage dentin, whereas bioorganic phase of teenage crown dentin near the pulp camera does not contain the collagen fibers. Cracks in dentin thin foils have sharp tips, but big angles of opening (~ 30{sup °}) with plastic zone ahead crack tip. It means that young crown human dentin exhibits ductile or viscous-elastic fracture behavior on the nanometer scale. - Highlights: • Dentin has layered morphology. • Mineral component of dentin is in amorphous state. • Collagen fibers form cellular structure in dentin. • Cracks in dentin behave by elastic-plastic manner.

  19. Transmission electron microscopical study of teenage crown dentin on the nanometer scale

    International Nuclear Information System (INIS)

    Panfilov, Peter; Kabanova, Anna; Guo, Jinming; Zhang, Zaoli

    2017-01-01

    Statement of significance: This is the first transmission electron microscopic study of teenage crown dentin on the nanometer scale. Samples for TEM were prepared by mechanical thinning and chemical polishing that allowed obtaining the electron transparent foils. It was firstly shown that human dentin possesses the layered morphology: the layers are oriented normally to the main axis of a tooth and have the thickness of ~ 50 nm. HA inorganic phase of teenage crown dentin is in the amorphous state. The cellular structure, which was formed from collagen fibers (diameter is ~ 5 nm), are observed near DEJ region in teenage dentin, whereas bioorganic phase of teenage crown dentin near the pulp camera does not contain the collagen fibers. Cracks in dentin thin foils have sharp tips, but big angles of opening (~ 30 ° ) with plastic zone ahead crack tip. It means that young crown human dentin exhibits ductile or viscous-elastic fracture behavior on the nanometer scale. - Highlights: • Dentin has layered morphology. • Mineral component of dentin is in amorphous state. • Collagen fibers form cellular structure in dentin. • Cracks in dentin behave by elastic-plastic manner.

  20. Deformation behavior of human dentin in liquid nitrogen: a diametral compression test.

    Science.gov (United States)

    Zaytsev, Dmitry; Panfilov, Peter

    2014-09-01

    Contribution of the collagen fibers into the plasticity of human dentin is considered. Mechanical testing of dentin at low temperature allows excluding the plastic response of its organic matrix. Therefore, deformation and fracture behavior of the dentin samples under diametral compression at room temperature and liquid nitrogen temperature are compared. At 77K dentin behaves like almost brittle material: it is deformed exclusively in the elastic regime and it fails due to growth of the sole crack. On the contrary, dentin demonstrates the ductile response at 300K. There are both elastic and plastic contributions in the deformation of dentin samples. Multiple cracking and crack tip blunting precede the failure of samples. Organic phase plays an important role in fracture of dentin: plasticity of the collagen fibers could inhibit the crack growth. Copyright © 2014 Elsevier B.V. All rights reserved.

  1. Structure and microstructure of coronary dentin in non-erupted human deciduous incisor teeth

    Directory of Open Access Journals (Sweden)

    Costa Luciane R.R S.

    2002-01-01

    Full Text Available The dentin structure of non-erupted human deciduous mandibular and maxillary central and lateral incisor teeth was studied employing light and scanning electron microscopy. For light microscopy, nitric-acid-demineralized and ground sections were used. The sections were stained by hematoxylin-eosin, picrosirius and azo-carmim methods, and ground specimens were prepared using a carborundum disk mounted in a handpiece. For SEM study, teeth were frozen in liquid nitrogen and fractured at longitudinal and transversal directions. Structurally, demineralization and ground methods revealed tubules with primary and secondary curvatures, canaliculi, giant tubules, interglobular dentin, predentin, and intertubular dentin. Scanning electron microscopy showed three-dimensional aspects of dentinal tubules, canaliculi, peritubular dentin, intertubular dentin, and predentin. This study contributes to knowledge about dentin morphology showing characteristics of teeth not yet submitted to mastication stress.

  2. MRI findings of dysplasia epiphysealis hemimelica

    Energy Technology Data Exchange (ETDEWEB)

    Iwasawa, T. [Dept. of Radiology, School of Medicine, Yokohama City Univ., Kanagawa (Japan); Aida, N. [Dept. of Radiology, School of Medicine, Yokohama City Univ., Kanagawa (Japan)]|[Kanagawa Children`s Medical Center, Yokohama (Japan); Kobayashi, N. [Dept. of Radiology, School of Medicine, Yokohama City Univ., Kanagawa (Japan)]|[Kanagawa Children`s Medical Center, Yokohama (Japan); Nishimura, G. [Dept. of Radiology, School of Medicine, Yokohama City Univ., Kanagawa (Japan)]|[Dept. of Radiology, School of Medicine, Dokkyou Univ., Tochigi (Japan)

    1996-01-01

    We report a boy with dysplasia epiphysealis hemimelica of the knee, particularly emphasizing MRI findings. When he was 14 months of age, plain radiography exhibiting juxta-articular soft tissue mass with small, punctate, calcified foci did not warrant the definitive diagnosis, while MRI clearly demonstrated osteocartilaginous overgrowth of the femoral epiphysis. The epiphyseal mass mostly showed the same signal intensity as normal cartilage but contained low signal spots corresponding to calcified foci. The cartilaginous cap was depicted as a mottled area of high intensity on a T2-weighted image. When the patient was 4 years of age, this ossified mass was resected surgically and pathologically identified as osteochondroma incorporated into the epiphysis. (orig.)

  3. Florid Cementoosseous Dysplasia: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Mehmet Fatih Şentürk

    2013-01-01

    Full Text Available Florid cementoosseous dysplasia (FCOD is a rare, benign, fibroosseous, and multifocal dysplastic lesion of the jaw that consists of cellular fibrous connective tissue with bone and cementum-like tissue. FCOD is most commonly found in middle-aged black women, is generally asymptomatic, and is usually detected during radiological examination. FCOD associated with multiple impacted teeth and bone expansion is a very rare phenomenon, and there are only a few familial cases reported in the literature. In this report, a 35-year-old male Turkish patient is presented who was diagnosed with nonfamilial FCOD from clinical, radiological, and histopathological findings. To our knowledge this is the first case of the nonfamilial FCOD with this many impacted teeth and severely expanded bones.

  4. Ectodermal dysplasia-skin fragility syndrome

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    Vijay S Adhe

    2011-01-01

    Full Text Available Ectodermal dysplasia-skin fragility (EDSF syndrome is a rare and first described inherited disorder of desmosomes. It occurs due to loss-of-function mutations in PKP1 gene resulting in poorly formed desmosomes and loss of desmosomal and epidermal integrity. We report a case of a 2-year-old Indian male child who presented with palmoplantar hyperkeratosis with fissuring, short, sparse, and easily pluckable scalp hair, nail dystrophy, and multiple erosions over the skin. Skin biopsy showed epidermal hyperplasia with widening of intercellular spaces. His developmental milestones were delayed but intelligence was normal. Echocardiography, X-ray chest, and electrocardiogram were normal. Very few cases of this syndrome have been reported in the literature. We consider this as the first case report from India.

  5. Four cases of right ventricular dysplasia

    International Nuclear Information System (INIS)

    Takamura, Ichiro; Ando, Joji; Miyamoto, Atsushi; Kobayashi, Takeshi; Sakamoto, Sanya; Yasuda, Hisakazu

    1985-01-01

    Finding of 81 Kr right ventriculography and 201 Tl myocardial perfusion imaging in 4 patients with right ventricular dysplasia (RVD) were compared with those in 28 patients with dilated cardiomyopathy. Remarkably dilated right ventricle was detected on 201 Tl myocardial perfusion imaging in the RVD group. In a patient with RVD who died suddenly, perfusion defect of the left ventricular myocardium, a decreased right ventricular ejection fraction, and an increased right ventricular end diastolic volume were seen. Perfusion defect of the left ventricular myocardium was seen in 10 of the 28 patients with dilated cardiomyopathy, 4 of whom died suddenly. In these 4 patients, a decreased left ventricular ejection fraction and an increased right ventricular end diastolic volume were seen. These findings obtained by the radionuclide techniques suggested that there are differences in cardiac dysfunction of the both ventricles between the groups with RVD and dilated cardiomyopathy. (Namekawa, K.)

  6. Focal cemento-osseous dysplasia of mandible.

    Science.gov (United States)

    Cankaya, Abdülkadir Burak; Erdem, Mehmet Ali; Olgac, Vakur; Firat, Deniz Refia

    2012-09-03

    Fibro-osseous lesions are disturbances in bone metabolism in which normal bone is replaced by a connective tissue matrix that then gradually develops into cemento-osseous tissue. Typically, the lesion is asymptomatic and is detected on routine radiographic examination. Radiologically, this lesion has three stages of maturation: pure radiolucent, radiopaque/mixed radiolucent, and radiopaque appearance. During these stages the lesion can be misdiagnosed. In this case report a 69-year- old patient with a a complaint of painless swelling of the left mandibular molar and premolar area is presented along with a review of the differential diagnoses considered in order to reach a final diagnosis of focal cemento-osseous dysplasia.

  7. Nonfamilial cleidocranial dysplasia (dysostosis): a case report

    International Nuclear Information System (INIS)

    Salem, G

    1990-01-01

    Cleidocranial dysplasia, previously known as cleidocranial dysostosis, is a rare hereditary disease of unknown etiology characterized by abnormalities in the skull, jaws, shoulder girdle, as well as abnormalities of the dentition. The disease usually follows an autosomal dominant mode of transmission. This is a report on a case in a 25-year-old Saudi female from Gizan, Saudi Arabia, which seemes to be the first case reported in the Kingdom. An outstanding feature of this case is that it did not follow a familial pattern of inheritance since the patient is the only member of the family suffering from such disorder. The abnor malities present in the dentition are described together with the associated skeletal malformations. The clin ical and radiographic findings, as well as the hereditary pattern of the disease as described in the literature, are discussed. The dental management of these cases is reviewed. (author)

  8. Guidelines for genetic skeletal dysplasias for pediatricians

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    Sung Yoon Cho

    2015-12-01

    Full Text Available Skeletal dysplasia (SD is a kind of heterogeneous genetic disorder characterized by abnormal growth, development, differentiation, and maintenance of the bone and cartilage. The patients with SD most likely to be seen by a pediatrician or orthopedic surgeon are those who present with short stature in childhood. Because each category has so many diseases, classification is important to understand SD better. In order to diagnose a SD accurately, clinical and radiographic findings should be evaluated in detail. In addition, genetic diagnosis of SD is important because there are so various SDs with complex phenotypes. To reach an exact diagnosis of SDs, cooperative approach by a clinician, a radiologist and a geneticist is important. This review aims to provide an outline of the diagnostic approach for children with disproportional short stature.

  9. The Demographics of Canine Hip Dysplasia in the United States and Canada

    Directory of Open Access Journals (Sweden)

    Randall T. Loder

    2017-01-01

    Full Text Available Canine hip dysplasia (CHD is a common problem in veterinary medicine. We report the demographics of CHD using the entire hip dysplasia registry from the Orthopedic Foundation for Animals, analyzing differences by breed, sex, laterality, seasonal variation in birth, and latitude. There were 921,046 unique records. Each dog was classified using the American Kennel Club (AKC and Fédération Cynologique Internationale (FCI systems. Statistical analysis was performed with bivariate and logistic regression procedures. The overall CHD prevalence was 15.56%. The OR for CHD was higher in females (1.05, those born in spring (1.14 and winter (1.13, and those in more southern latitudes (OR 2.12. Within AKC groups, working dogs had the highest risk of CHD (OR 1.882 with hounds being the reference group. Within FCI groups, the pinscher/molossoid group had the highest risk of CHD (OR 4.168 with sighthounds being the reference group. The similarities between CHD and DDH are striking. Within DDH there are two different types, the typical infantile DDH and the late onset adolescent/adult acetabular dysplasia, with different demographics; the demographics of CHD are more similar to the later onset DDH group. Comparative studies of both disorders should lead to a better understanding of both CHD and DDH.

  10. Development of angle-resolved low coherence interferometry for clinical detection of dysplasia

    Directory of Open Access Journals (Sweden)

    Yizheng Zhu

    2011-01-01

    Full Text Available This review covers the development of angle-resolved low coherence interferometry (a/LCI from initial development through clinical application. In the first applications, the approach used a time-domain interferometry scheme and was validated using animal models of carcinogenesis to assess the feasibility of detecting dysplasia in situ. Further development of the approach led to Fourier-domain interferometry schemes with higher throughput and endoscope-compatible probes to enable clinical application. These later implementations have been applied to clinical studies of dysplasia in Barrett′s esophagus tissues, a metaplastic tissue type that is associated with an increased risk of esophageal adenocarcinoma. As an alternative to systematic biopsy, the a/LCI approach offers high sensitivity and specificity for detecting dysplasia in these tissues while avoiding the need for tissue removal or exogenous contrast agents. Here, the various implementations of a/LCI are discussed and the results of the preliminary animal experiments and ex vivo human tissue studies are reviewed. A review of a recent in vivo clinical study is also presented.

  11. Modelling borderline and mild dysplasia associated with HPV 6 and 11 infection.

    Science.gov (United States)

    Chapman, Ruth; Soldan, Kate; Jit, Mark

    2011-04-05

    Low risk HPV types 6/11 are responsible for some low-grade cytological abnormalities. Most economic analyses of HPV vaccination have estimated the additional benefit of HPV 6/11 protection by the quadrivalent vaccine, over the bivalent, based on reduction of genital warts but have not included reduction in repeat smears and colposcopies due to low-grade abnormalities. We investigate the contribution of HPV types 6/11 to abnormal smears and associated costs in England. The risk of borderline or mild dysplasia due to HPV 6/11 infection was estimated from a study of type-specific HPV DNA in cervical screening specimens collected throughout England. A Markov model representing 10 million women with HPV 6/11 or with no HPV infection from 24 to 64 years was developed to estimate the number of abnormal smears, subsequent repeat smears and colposcopies due to HPV 6/11 associated with borderline or mild dysplasia. Fitting was achieved by varying the force of infection, probability of borderline or mild dysplasia if HPV-uninfected or infected with HPV 6/11 and the duration of infection. The relative risks of borderline or mild dysplasia when infected with HPV 6/11 compared to not being HPV infected were 6.32 (95% credible interval 1.56-25.6) and 17.5 (1.02-300) respectively. Using best fitting parameters we find the costs incurred are between £170 and £195 per abnormal smear due to infection with HPV 6/11. In England, the impact of cytological abnormalities due to HPV 6/11 is relatively small, but not negligible. A vaccine that protects against HPV 6/11 infections could reduce costs associated with borderline and mild dysplasia, and associated colposcopies. These benefits should be considered when formulating immunisation policy, if possible. Smears and colposcopies in those uninfected with HPV far outnumber those in women infected with HPV 6/11. Copyright © 2011 Elsevier Ltd. All rights reserved.

  12. Hypohidrotic ectodermal dysplasia: a multidisciplinary approach.

    Science.gov (United States)

    Bildik, Tezan; Ozbaran, Burcu; Kose, Sezen; Koturoglu, Guldane; Gokce, Bulent; Gunaydin, Asli; Altintas, Inci

    2012-01-01

    Hypohidrotic ectodermal dysplasia (HED; Christ-Siemens-Touraine syndrome) is a genetic disorder characterized by sparse hair, oligodontia with peg-shaped teeth, reduced sweating, and defects in a number of other ectodermal organs. A partial or complete absence of eccrine glands can lead to recurrent severe overheating that may cause seizures and neurological deficits. This clinical report presents a 14-year-old male patient with hypohidrotic ectodermal dysplasia, including the clinical and radiographic findings, and multidisciplinary treatment. The Schedule for Affective Disorders and Schizophrenia for School Aged Children-Present and Lifetime Version (K-SADS-PL) was administered to patient along with mother to assess for any psychiatric disorders. The screening and rating scales completed by mother and two teachers to evaluate the severity of attention deficit hyperactivity disorder (ADHD) symptoms, and other behavioral problems. Patient's academic performance, adaptive functioning, and problem behavior was evaluated using. The Teacher Report Form. Mental capacity was assessed with the Wechsler Intelligence Scale for Children (WISC-R). Illness Perception Questionnaire Revised was used to systematically assess illness representation attributes and emotional representations of illness. On the psychiatric diagnosis assessment using K-SADS-PL sub-threshold attention deficits and anxiety symptoms were determined. In this case we established a multidisciplinary approach in his treatment with pediatric, dermatological, and dental examinations, beside his psychiatric evaluation. The prosthetic rehabilitation included restoring upper teeth with copings and fabrication of upper and lower complete dentures. Metal framework was not incorporated in the partial denture design allowing modifications as the oral and maxillofacial development continued. Removable complete or partial dentures without metal framework is a treatment of choice until the completion of facial growth at

  13. Penetration of varnishes into demineralized root dentine in vitro

    NARCIS (Netherlands)

    Arends, J; Duschner, H; Ruben, JL

    1997-01-01

    In this paper the penetration of three different varnishes employed in caries prevention (Duraphat(R), Fluor Protector(R) and Cervitec(R)) into demineralized dentine is quantified using confocal laser scanning microscopy. The results show that the varnish penetration into lesions about 85 mu m in

  14. Bonding strategies for MIH-affected enamel and dentin.

    Science.gov (United States)

    Krämer, Norbert; Bui Khac, Ngoc-Han Nana; Lücker, Susanne; Stachniss, Vitus; Frankenberger, Roland

    2018-02-01

    Aim of the present study was to evaluate resin composite adhesion to dental hard tissues affected by molar incisor hypomineralisation (MIH). 94 freshly extracted human molars and incisors (53 suffering MIH) were used. 68 teeth (35 with MIH) were used for μ-TBS tests in enamel and dentin, 26 (18 with MIH) for qualitative evaluation. Specimens were bonded with Clearfil SE Bond, Scotchbond Universal, and OptiBond FL. For MIH affected enamel, additional OptiBond FL groups with NaOCl and NaOCl+Icon were investigated. Beside fractographic analysis, also qualitative evaluations were performed using SEM at different magnifications as well as histological sectioning. Highest μ-TBS values were recorded with dentin specimens (ANOVA, mod. LSD, p0.05). Pre-test failures did not occur in dentin specimens. Sound enamel specimens exhibited significantly higher μ-TBS values than MIH enamel (p0.05), however, it caused less pre-test failures (pMIH enamel is the limiting factor in adhesion to MIH teeth. MIH-affected dentin may be bonded conventionally. Copyright © 2017 The Academy of Dental Materials. Published by Elsevier Ltd. All rights reserved.

  15. Amalgam stained dentin: a proper substrate for bonding resin composite?

    NARCIS (Netherlands)

    Scholtanus, J.D.

    2016-01-01

    Nowadays the use of dental amalgam is mostly abandoned and substituted by tooth colored resin composites that can be bonded to teeth tissues by adhesive techniques. The aim of this thesis was to find out whether dark stained dentin, as often observed after removal of amalgam restorations and

  16. Bio-reconstruction of root canal using dentin post

    Directory of Open Access Journals (Sweden)

    Navin Mishra

    2013-01-01

    Full Text Available This case report describes the successful management of complicated crown fracture of left maxillary central incisor (#21 of 23-year-old male using dentin (biological post made from human tooth. Endodontic treatment was initiated and sectional obturation was done using ProTaper gutta-percha and AH Plus sealer. The coronal space was modified to receive a human dentin post. A two-step procedure comprising direct and indirect technique of post fabrication was done to achieve the accuracy of biological post. A maxillary cuspid from an institutional tooth bank was taken, sectioned mesiodistally using a diamond disc simulating the length and thickness of mock post. Dentin post was first verified on the plaster model and then cemented in tooth #21. Core build up was done with composite and porcelain fused to metal (PFM crown was luted. On the follow-up visits, patient was asymptomatic and radiographic evaluation revealed normal periradicular architecture. Biological posts may be good alternatives to conventional post systems as they preserve internal dentin walls, provide excellent adhesion, and resilience similar to natural tooth structure.

  17. Effect of dentin desensitizing procedures on methyl methacrylate ...

    African Journals Online (AJOL)

    Background: Acrylic and bisacryl resins are widely used both during the temporization phase as well as for provisional restorations and the effect of external agents on dentin sensitivity can be reduced by the ... Materials and Methods: Forty extracted restoration and caries free human premolar teeth were used in this study.

  18. Müllerian agenesis with hypohidrotic ectodermal dysplasia syndrome.

    Science.gov (United States)

    Whaley, Katie; Winter, Jordan; Eyster, Kathleen M; Hansen, Keith A

    2012-04-01

    To describe the association of müllerian agenesis with hypohidrotic ectodermal dysplasia. Case report. University medical center. A 17-year-old woman with hypohidrotic ectodermal dysplasia referred for evaluation of primary amenorrhea. History, physical examination, and ultrasound. Physical findings of these two syndromes. Physical examination and ultrasound demonstrated müllerian agenesis with findings of hypohidrotic ectodermal dysplasia. This is the first description of the association of müllerian agenesis with ectodermal dysplasia. This rare case might provide further insight into the development of the uterus and the ectoderm as well as its derivatives. Copyright © 2012 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.

  19. Anhidrotic Ectodermal Dysplasia with Palmo-plantar Keratoderma

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    Kamlesh Kumar

    1982-01-01

    Full Text Available A patient with anhidrotic ectodermal dysplasia and palmo-plantar keratoderma is presented. Palmo-planta keratoderma is an unusual association with this disease. Atopic dermatitis was another associated condition in this patient.

  20. Ehlers-Danlos syndrome with monostotic fibrous dysplasia

    Directory of Open Access Journals (Sweden)

    Rao A

    1979-01-01

    Full Text Available An unusual case of Ehlers-Danlos syndrome with monostotic fibrous dysplasia of the humorus is presented. The other orthopae-dic manifestations, its complications and associated features are re-viewed and summarised.

  1. Cemento-osseous dysplasia in Jamaica: review of six cases.

    Science.gov (United States)

    Ogunsalu, C; Miles, D

    2005-09-01

    Six cases of cemento-osseous dysplasia (COD) of the jaw bone in Jamaicans are reviewed. Five were documented over a 15-year period (1980-1995). These include a case of florid cemento-osseous dysplasia (previously called gigantiform cementoma). Three of the initial cases were histologically diagnosed as gigantiform cementoma. There was no indication in the patient's case file whether these were familial or non-familial. The other two cases were diagnosed histologically as periapical cemento-osseous dysplasia and cementoblastoma respectively. Based on the current understanding of the nature of florid-cemento-osseous dysplasia (FLCOD), a new case was diagnosed as such solely on radiological findings. This single case of FLCOD is reported and discussed against the background of other cemento-osseous lesions. Special emphasis is placed on the radiology of COD in this paper. The confirmative role of radiology without the need for histophathology and treatment for asymptomatic FLCOD is emphasized.

  2. Omphalocele and alveolar capillary dysplasia: a new association.

    NARCIS (Netherlands)

    Gerrits, L.C.; Mol, A.C. de; Bulten, J.; Staak, F.H.J.M. van der; Heijst, A.F.J. van

    2010-01-01

    OBJECTIVE: First report of an infant with coexistent omphalocele and alveolar capillary dysplasia. DESIGN: Descriptive case report. SETTING: Neonatal intensive care unit of a tertiary care children's hospital. PATIENT: We describe a term infant with omphalocele and respiratory insufficiency

  3. Electrocorticographic discharge patterns in glioneuronal tumors and focal cortical dysplasia

    NARCIS (Netherlands)

    Ferrier, Cyrille H.; Aronica, Eleanora; Leijten, Frans S. S.; Spliet, Wim G. M.; van Huffelen, Alexander C.; van Rijen, Peter C.; Binnie, Colin D.

    2006-01-01

    PURPOSE: To determine whether highly epileptiform electrocorticographical discharge patterns occur in patients with glioneuronal tumors (GNTs) and focal cortical dysplasia (FCD) and whether specific histopathological features are related to such patterns. METHODS: The series consists of operated

  4. Urethral dysontogenic metaplasia in cat with bilateral renal dysplasia

    Directory of Open Access Journals (Sweden)

    Carolina da Fonseca Sapin

    2017-10-01

    Full Text Available This paper to describe a case of dysontogenic urethral metaplasia in a one month old mongrel feline who also had bilateral renal dysplasia. Dysontogenic metaplasia in cats are scarce and this change may be associated with renal dysplasia and/or lower urinary tract. The animal had history of abdominal enlargement since birth and dysuria, eliminating urine only dropwise. Due to the poor prognosis we opted for euthanasia. At necropsy was observed enlarged and distended bladder, reduced kidneys and dilated and tortuous ureters. The urethra was thickened, hard to cut, and histologically, was replacing the connective tissue, cartilage and endochondral ossification areas, which features dysontogenic metaplasia. Both kidneys presented primitive appearance featuring dysplasia. Dysontogenic metaplasia in urinary tract feline with renal dysplasia, has not been described.

  5. p63 in skin development and ectodermal dysplasias

    Science.gov (United States)

    Koster, Maranke I.

    2010-01-01

    The transcription factor p63 is critically important for skin development and maintenance. Processes that require p63 include epidermal lineage commitment, epidermal differentiation, cell adhesion, and basement membrane formation. Not surprisingly, alterations in the p63 pathway underlie a subset of ectodermal dysplasias, developmental syndromes in which the skin and skin appendages do not develop normally. This review summarizes the current understanding of the role of p63 in normal development and ectodermal dysplasias. PMID:20445549

  6. Hereditary hypohidrotic ectodermal dysplasia: report of a rare case.

    Science.gov (United States)

    Paramkusam, Geetha; Meduri, Venkateswarlu; Nadendla, Lakshmi Kavitha; Shetty, Namratha

    2013-09-01

    Hereditary Hypohidrotic Ectodermal Dysplasia (HHED), an X-linked, recessive, Mendelian character, is seen usually in males and it is inherited through female carriers. It is characterised by congenital dysplasia of one or more ectodermal structures and it is manifested by hypohidrosis, hypotrichosis and hypodontia. It results from abnormal morphogenesis of cutaneous and oral embryonic ectoderm. Here, we are presenting a rare case of HHED in a 19 year female with classic features of this condition.

  7. Brachial artery aneurysm and thrombosis secondary to fibromuscular dysplasia

    Directory of Open Access Journals (Sweden)

    Julia Louise Jones, MBBS

    2016-09-01

    Full Text Available Fibromuscular dysplasia is a pathologic process causing stenosis and dilation of medium-caliber arteries of unknown etiology. It most commonly affects the renal and carotid arteries; however, it has been described in virtually all anatomic areas, including, rarely, the brachial artery. We describe a case of brachial artery aneurysm and thrombosis in a 29-year-old man secondary to fibromuscular dysplasia, treated surgically with excision, embolectomy, interposed vein graft, and anticoagulation.

  8. Adult-onset Rasmussen encephalitis associated with focal cortical dysplasia.

    Science.gov (United States)

    Hohenbichler, Katharina; Lelotte, Julie; Lhommel, Renaud; Tahry, Riëm El; Vrielynck, Pascal; Santos, Susana Ferrao

    2017-12-01

    Rasmussen encephalitis is a rare, devastating condition, typically presenting in childhood. Cases of adult-onset Rasmussen have also been described, but the clinical picture is less defined, rendering final diagnosis difficult. We present a case of adult-onset Rasmussen encephalitis with dual pathology, associated with focal cortical dysplasia and encephalitis. We interpreted the Rasmussen encephalitis to be caused by severe and continuous epileptic activity due to focal cortical dysplasia. The best therapeutic approach for such cases remains unclear.

  9. Chondrosarcoma occurring in a patient with polyostotic fibrous dysplasia

    Energy Technology Data Exchange (ETDEWEB)

    De Smet, A.A.; Travers, H.; Neff, J.R.

    1981-12-01

    A 36-year-old white man with polyostotic fibrous dysplasia was found to have a high-grade chondrosarcoma arising from the left ilium. Although a left hemipelvectomy was performed, the patient subsequently developed sacral and pulmonary metastases and succumbed to his disease. This patient represents the first documented example of an unequivocally high-grade chondrosarcoma arising in an area of fibrous dysplasia without prior irradiation.

  10. Ectrodactyly, ectodermal dysplasia, cleft lip, and palate (EEC syndrome

    Directory of Open Access Journals (Sweden)

    Mohita Marwaha

    2012-01-01

    Full Text Available The ectodermal dysplasias (EDs are a large and complex group of diseases. More than 170 different clinical conditions have been recognized and defined as ectodermal dysplasias. Commonly involved ectodermal-derived structures are hair, teeth, nails, and sweat glands. In some conditions, it may be associated with mental retardation. We report a case of 10-year-old male child with ectrodactyly, syndactyly, ED, cleft lip/palate, hearing loss, and mental retardation.

  11. The association between gender and familial prevalence of hip dysplasia in Danish patients

    DEFF Research Database (Denmark)

    El Jashi, Rima; Gustafson, Maria B; Jakobsen, Mette B

    2017-01-01

    BACKGROUND: The development of hip dysplasia is associated with several risk factors. 1 of these risk factors is gender, since 80% of patients with symptomatic hip dysplasia are females. Another risk factor for hip dysplasia is familial predisposition of hip dysplasia. Several studies indicate...... that the risk of hip dysplasia is increased with familial prevalence of hip dysplasia. However, little is known about the association between the familial prevalence and gender and the development of hip dysplasia. PURPOSE: The aim of the study was to estimate the prevalence of hip dysplasia among relatives...... to Danish patients with hip dysplasia operated with periacetabular osteotomy (PAO), and the degree of relationship of affected family members. Furthermore, to assess the association between gender and family predisposition in the same group of patients. METHOD: The study is a cross-sectional study...

  12. Fracture toughness of dentin/resin-composite adhesive interfaces.

    Science.gov (United States)

    Tam, L E; Pilliar, R M

    1993-05-01

    The reliability and validity of tensile and shear bond strength determinations of dentin-bonded interfaces have been questioned. The fracture toughness value (KIC) reflects the ability of a material to resist crack initiation and unstable propagation. When applied to an adhesive interface, it should account for both interfacial bond strength and inherent defects at or near the interface, and should therefore be more appropriate for characterization of interface fracture resistance. This study introduced a fracture toughness test for the assessment of dentin/resin-composite bonded interfaces. The miniature short-rod specimen geometry was used for fracture toughness testing. Each specimen contained a tooth slice, sectioned from a bovine incisor, to form the bonded interface. The fracture toughness of an enamel-bonded interface was assessed in addition to the dentin-bonded interfaces. Tensile bond strength specimens were also prepared from the dentin surfaces of the cut bovine incisors. A minimum of ten specimens was fabricated for each group of materials tested. After the specimens were aged for 24 h in distilled water at 37 degrees C, the specimens were loaded to failure in an Instron universal testing machine. There were significant differences (p adhesives tested. Generally, both the fracture toughness and tensile bond strength measurements were highest for AllBond 2, intermediate for 3M MultiPurpose, and lowest for Scotchbond 2. Scanning electron microscopy of the fractured specimen halves confirmed that crack propagation occurred along the bond interface during the fracture toughness test. It was therefore concluded that the mini-short-rod fracture toughness test provided a valid method for characterization of the fracture resistance of the dentin-resin composite interface.

  13. Is There a Relationship between Ovarian Epithelial Dysplasia and Infertility?

    Directory of Open Access Journals (Sweden)

    Gautier Chene

    2012-01-01

    Full Text Available Aim. Ovarian epithelial dysplasia was initially described in material from prophylactic oophorectomies performed in patients at genetic risk of ovarian cancer. Similar histopathological abnormalities have been revealed after ovulation stimulation. Since infertility is also a risk factor for ovarian neoplasia, the aim of this study was to study the relationship between infertility and ovarian dysplasia. Methods. We blindly reviewed 127 histopathological slides of adnexectomies or ovarian cystectomies according to three groups—an exposed group to ovulation induction (n = 30, an infertile group without stimulation (n = 35, and a spontaneously fertile control group (n = 62—in order to design an eleven histopathological criteria scoring system. Results. The ovarian dysplasia score was significantly higher in exposed group whereas dysplasia score was low in infertile and control groups (resp., 8.21 in exposed group, 3.69 for infertile patients, and 3.62 for the controls. In the subgroup with refractory infertility there was a trend towards a more severe dysplasia score (8.53 in ovulation induction group and 5.1 in infertile group. Conclusion. These results raise questions as to the responsibility of drugs used to induce ovulation and/or infertility itself in the genesis of ovarian epithelial dysplasia.

  14. Hypohidrotic ectodermal dysplasia: a clinical case with a longitudinal approach.

    Science.gov (United States)

    Fraiz, Fabian Calixto; Gugisch, Renato Cordeiro; Cavalcante-Leão, Bianca Lopes; Macedo, Liliane Moreira

    2014-11-01

    This paper describes a clinical case of a male with hypohidrotic ectodermal dysplasia submitted to rehabilitation and occlusal dental interventions with follow-up from 3 to 14 years of age. Due to the severe effects on function and esthetics, the clinical manifestations of ectodermal dysplasia exert a negative impact on quality of life. However, oral rehabilitation in childhood poses a challenge due to growth and development. A male with hypohidrotic ectodermal dysplasia began dental intervention at the age of 3 years. The clinical and radiographic exams revealed the absence of several primary and permanent teeth and abnormal shape of the primary maxillary incisors. The facial characteristics were compatible with hypohidrotic ectodermal dysplasia, such as a prominent brow, everted lips, fattened bridge of the nose and small vertical facial height. The treatment proposed involved rehabilitation through successive temporary partial dentures, functional orthopedics of the jaws, esthetic reconstruction of the anterior teeth, timely occlusal intervention and preventive actions for the control of dental caries and plaque. The present case demonstrates that early care plays a fundamental role in minimizing the biopsychosocial consequences of hypohidrotic ectodermal dysplasia and preparing the patient for future oral rehabilitation. Although, the literature offers a number of papers describing dental treatment for ectodermal dysplasia, few cases include long-term follow-up and the use of a functional orthopedic appliance in combination with removable dentures in such patients.

  15. Assessment of hip dysplasia and osteoarthritis: Variability of different methods

    International Nuclear Information System (INIS)

    Troelsen, Anders; Elmengaard, Brian; Soeballe, Kjeld; Roemer, Lone; Kring, Soeren

    2010-01-01

    Background: Reliable assessment of hip dysplasia and osteoarthritis is crucial in young adults who may benefit from joint-preserving surgery. Purpose: To investigate the variability of different methods for diagnostic assessment of hip dysplasia and osteoarthritis. Material and Methods: By each of four observers, two assessments were done by vision and two by angle construction. For both methods, the intra- and interobserver variability of center-edge and acetabular index angle assessment were analyzed. The observers' ability to diagnose hip dysplasia and osteoarthritis were assessed. All measures were compared to those made on computed tomography scan. Results: Intra- and interobserver variability of angle assessment was less when angles were drawn compared with assessment by vision, and the observers' ability to diagnose hip dysplasia improved when angles were drawn. Assessment of osteoarthritis in general showed poor agreement with findings on computed tomography scan. Conclusion: We recommend that angles always should be drawn for assessment of hip dysplasia on pelvic radiographs. Given the inherent variability of diagnostic assessment of hip dysplasia, a computed tomography scan could be considered in patients with relevant hip symptoms and a center-edge angle between 20 deg and 30 deg. Osteoarthritis should be assessed by measuring the joint space width or by classifying the Toennis grade as either 0-1 or 2-3

  16. Assessment of hip dysplasia and osteoarthritis: Variability of different methods

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    Troelsen, Anders; Elmengaard, Brian; Soeballe, Kjeld (Orthopedic Research Unit, Univ. Hospital of Aarhus, Aarhus (Denmark)), e-mail: a_troelsen@hotmail.com; Roemer, Lone (Dept. of Radiology, Univ. Hospital of Aarhus, Aarhus (Denmark)); Kring, Soeren (Dept. of Orthopedic Surgery, Aabenraa Hospital, Aabenraa (Denmark))

    2010-03-15

    Background: Reliable assessment of hip dysplasia and osteoarthritis is crucial in young adults who may benefit from joint-preserving surgery. Purpose: To investigate the variability of different methods for diagnostic assessment of hip dysplasia and osteoarthritis. Material and Methods: By each of four observers, two assessments were done by vision and two by angle construction. For both methods, the intra- and interobserver variability of center-edge and acetabular index angle assessment were analyzed. The observers' ability to diagnose hip dysplasia and osteoarthritis were assessed. All measures were compared to those made on computed tomography scan. Results: Intra- and interobserver variability of angle assessment was less when angles were drawn compared with assessment by vision, and the observers' ability to diagnose hip dysplasia improved when angles were drawn. Assessment of osteoarthritis in general showed poor agreement with findings on computed tomography scan. Conclusion: We recommend that angles always should be drawn for assessment of hip dysplasia on pelvic radiographs. Given the inherent variability of diagnostic assessment of hip dysplasia, a computed tomography scan could be considered in patients with relevant hip symptoms and a center-edge angle between 20 deg and 30 deg. Osteoarthritis should be assessed by measuring the joint space width or by classifying the Toennis grade as either 0-1 or 2-3

  17. Papillary bile duct dysplasia in primary sclerosing cholangitis.

    Science.gov (United States)

    Ludwig, J; Wahlstrom, H E; Batts, K P; Wiesner, R H

    1992-06-01

    A 62-year-old man with a 20-year history of chronic ulcerative colitis and a 9-year history of primary sclerosing cholangitis (PSC) underwent orthotopic liver transplantation because of symptoms related to PSC and cholangiographic features compatible with a biliary neoplasm. Study of the excised liver revealed papillary mucosal lesions in the common hepatic duct and the right and left hepatic ducts as well as cholangiectases and other features typically associated with PSC. The papillary lesions consisted of abundant fibrovascular stroma covered by biliary epithelium with low-grade and high-grade dysplasia. Some periductal glands were also dysplastic. These features distinguished papillary dysplasia from classic biliary papillomatosis. Only one focus of microinvasion was found; there were no metastases. Among 60 cases of PSC in whom the entire liver could be studied after orthotopic liver transplantation, this was the only instance of unequivocal dysplasia. However, in one specimen, papillary hyperplasia was found. Detailed macroscopic and microscopic rereview of 23 livers from our patients with the longest history of PSC (range, 5-24 years) failed to reveal any additional cases with dysplasia. It is concluded that (a) papillary mucosal lesions in PSC may represent papillary dysplasia without invasion; (b) these lesions may evolve from papillary hyperplasia; (c) the process may be largely, if not entirely, in situ; and (d) the prevalence of dysplasia and carcinoma of bile ducts may be less than the 7%-9% reported in the literature for malignancies associated with PSC.

  18. TRPV4 related skeletal dysplasias: a phenotypic spectrum highlighted byclinical, radiographic, and molecular studies in 21 new families

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    Sillence David

    2011-06-01

    Full Text Available Abstract Background The TRPV4 gene encodes a calcium-permeable ion-channel that is widely expressed, responds to many different stimuli and participates in an extraordinarily wide range of physiologic processes. Autosomal dominant brachyolmia, spondylometaphyseal dysplasia Kozlowski type (SMDK and metatropic dysplasia (MD are currently considered three distinct skeletal dysplasias with some shared clinical features, including short stature, platyspondyly, and progressive scoliosis. Recently, TRPV4 mutations have been found in patients diagnosed with these skeletal phenotypes. Methods and Results We critically analysed the clinical and radiographic data on 26 subjects from 21 families, all of whom had a clinical diagnosis of one of the conditions described above: 15 with MD; 9 with SMDK; and 2 with brachyolmia. We sequenced TRPV4 and identified 9 different mutations in 22 patients, 4 previously described, and 5 novel. There were 4 mutation-negative cases: one with MD and one with SMDK, both displaying atypical clinical and radiographic features for these diagnoses; and two with brachyolmia, who had isolated spine changes and no metaphyseal involvement. Conclusions Our data suggest the TRPV4 skeletal dysplasias represent a continuum of severity with areas of phenotypic overlap, even within the same family. We propose that AD brachyolmia lies at the mildest end of this spectrum and, since all cases described with this diagnosis and TRPV4 mutations display metaphyseal changes, we suggest that it is not a distinct entity but represents the mildest phenotypic expression of SMDK.

  19. Genetics Home Reference: platyspondylic lethal skeletal dysplasia, Torrance type

    Science.gov (United States)

    ... and an exaggerated curvature of the lower back ( lordosis ). Infants with this condition are born with a ... Diagnosis and Management Resources (1 link) MedlinePlus Encyclopedia: Lordosis General Information from MedlinePlus (5 links) Diagnostic Tests ...

  20. Morphological alterations of radicular dentine pretreated with different irrigating solutions and irradiated with 980-nm diode laser.

    Science.gov (United States)

    Alfredo, Edson; Souza-Gabriel, Aline E; Silva, Silvio Rocha C; Sousa-Neto, Manoel D; Brugnera-Junior, Aldo; Silva-Sousa, Yara T C

    2009-01-01

    The topographical features of intraradicular dentine pretreated with sodium hypochlorite (NaOCl) or ethylenediamine tetraacetic acid (EDTA) followed by diode laser irradiation have not yet been determined. To evaluate the alterations of dentine irradiated with 980-nm diode laser at different parameters after the surface treatment with NaOCl and EDTA. Roots of 60 canines were biomechanically prepared and irrigated with NaOCl or EDTA. Groups were divided according to the laser parameters: 1.5 W/CW; 1.5 W/100 Hz; 3.0 W/CW; 3.0 W/100 Hz and no irradiation (control). The roots were splited longitudinally and analyzed by scanning electron microscopy (SEM) in a quali-quatitative way. The scores were submitted to two-way Kruskal-Wallis and Dunn's tests. The statistical analysis demonstrated that the specimens treated only with NaOCl or EDTA (control groups) were statistically different (P laser-irradiated specimens, regardless of the parameter setting. The specimens treated with NaOCl showed a laser-modified surface with smear layer, fissures, and no visible tubules. Those treated with EDTA and irradiated by laser presented absence of smear layer, tubules partially exposed and melting areas. The tested parameters of 980-nm diode laser promoted similar alterations on dentine morphology, dependent to the type of surface pretreatment. Copyright 2008 Wiley-Liss, Inc.