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Neonatal Teeth

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501. Ectodermal dysplasia syndrome

of two or more ectodermal derivatives, including hair, teeth, nails, sweat glands and their modified structures (i.e. ceruminous, mammary and ciliary glands). The spectrum of clinical manifestations is wide and may include additional manifestations from other ectodermal, mesodermal and endodermal structures. ORPHA:79373 Classification level: Group of disorders Synonym(s): Ectodermal dysplasia Prevalence: 6-9 / 10 000 Inheritance: - Age of onset: Neonatal , Infancy ICD-10: - OMIM: - UMLS: C0013575 (...) , with a tendency towards congenital or early-onset non-inflammatory alopecia. Teeth are slow-growing and defective in number (i.e. hypo-, oligo- and anodontia; see these terms); they are often dysplastic (i.e. peg shaped) and display enamel dysplasia. Nails manifest a wide range of features, comprising but not limited to dystrophic, thickened, and absent nails. Eccrine glands may be reduced in number or totally absent leading to hypohidrosis and anhidrosis, respectively. Hypoplastic breast and nipples

2005 Orphanet

502. Incontinentia pigmenti

(BL) followed by verrucous plaques evolving over time to hyperpigmented swirling patterns. It is further characterized by teeth abnormalities, alopecia, nail dystrophy and affects occasionally the retina and the central nervous system (CNS). ORPHA:464 Classification level: Disorder Synonym(s): Bloch-Siemens syndrome Bloch-Sulzberger syndrome Prevalence: 1-9 / 1 000 000 Inheritance: X-linked dominant Age of onset: Neonatal ICD-10: Q82.3 OMIM: UMLS: C0021171 C0022283 C2930820 MeSH: D007184 GARD (...) of information on the Orphanet website are accepted. For all other comments, please send your remarks via . Only comments written in English can be processed. Check this box if you wish to receive a copy of your message * " for="captcha" >Please reproduce the text below: * Incontinentia pigmenti Disease definition Incontinentia pigmenti (IP) is a rare X-linked dominant multi-systemic ectodermal dysplasia usually lethal in males and presenting neonatally in females with a bullous rash along Blashko's lines

2005 Orphanet

503. Dyskeratosis congenita

(BMF) and cancer. ORPHA:1775 Classification level: Disorder Synonym(s): DC DKC Zinsser-Engman-Cole syndrome Prevalence: 1-9 / 1 000 000 Inheritance: Autosomal dominant or Autosomal recessive or X-linked recessive Age of onset: Neonatal , Infancy , Childhood , Adolescent , Adult ICD-10: Q82.8 OMIM: UMLS: C0265965 MeSH: D019871 GARD: MedDRA: 10062759 Summary Epidemiology Dyskeratosis congenita (DC) prevalence is unknown. More than 400 families are reported in the world. Clinical description DC has (...) or anogenital cancer. Additional clinical findings have been reported and may include: developmental delay, short stature, microcephaly, blepharitis, epiphora, periodontal disease, taurodontism, decreased teeth/root ratio, esophageal stenosis, urethral stenosis, osteoporosis, avascular necrosis of femur and/or humerus, premature hair greying/alopecia, or abnormal eyelashes. Individuals with Patients with DC may also develop pulmonary fibrosis, pulmonary arteriovenous malformations, gastrointestinal

2005 Orphanet

504. Amelogenesis imperfecta

of information on the Orphanet website are accepted. For all other comments, please send your remarks via . Only comments written in English can be processed. Check this box if you wish to receive a copy of your message * " for="captcha" >Please reproduce the text below: * Amelogenesis imperfecta Disease definition A rare genetic odontal or periodontal disorder that represents a group of developmental conditions affecting the structure and clinical appearance of the enamel of all or nearly all the teeth (...) in a more or less equal manner, and which may be associated with morphologic or biochemical changes elsewhere in the body. ORPHA:88661 Classification level: Disorder Synonym(s): - Prevalence: Unknown Inheritance: Autosomal recessive or Autosomal dominant or X-linked dominant Age of onset: Infancy , Neonatal ICD-10: K00.5 OMIM: UMLS: C0002452 MeSH: D000567 GARD: MedDRA: - Summary Epidemiology The prevalence varies from 1:700 to 1:14,000, according to the populations studied. Clinical description

2005 Orphanet

505. Pachyonychia congenita

. The first signs of the disease usually are thickened nails or neonatal teeth. At least 3 phenotypes of hypertrophic nail dystrophy of feet and hands can be observed: nail grows to full length but a distal prominent hyperkeratosis causes an upward slant with an accentuated curvature of the nail; nail plate terminates prematurely leaving a distal region of hyperkeratosis and an exposed finger tip; or nail plate is thin with little or no hyperkeratosis. When children start walking, typically in the first

2005 Orphanet

506. Oral-facial-digital syndrome, type 1

anomalies including external malformations (craniofacial and digital), and possible involvement of the central nervous system (CNS) and of viscera (kidneys, pancreas and ovaries) in females. ORPHA:2750 Classification level: Disorder Synonym(s): OFD1 OFDI OFDSI Oral-facial-digital syndrome type 1 Papillon-L�age-Psaume syndrome Prevalence: Unknown Inheritance: X-linked dominant or Not applicable Age of onset: Infancy , Neonatal ICD-10: Q87.0 OMIM: UMLS: C1510460 C2931426 MeSH: - GARD: MedDRA: - Summary (...) % (lobed tongue, tongue hamartomas or lipomas, ankyloglossia, cleft or highly arched palate, accessory gingival frenulae, missing (hypodontia, see this term) or extra teeth, enamel dysplasia, and malocclusion), craniofacial abnormalities in about 87% including facial dysmorphism (ocular hypertelorism or telecanthus, hypoplasia of the alae nasi, median cleft or pseudocleft upper lip, micrognathia, downslanting palpebral fissures), abnormal hair/alopecia, evanescent facial milia, digital malformations

2004 Orphanet

507. In childen with herpetic stomatitis should all be treated as soon as first lesions appear with antiviral;if so which treatment is recommended?

section on recurrent attacks (see guideline for further information). PRODIGY also has an overview of the management of herpes simplex gingivostomatitis, which reports: “Soft diet and adequate fluid intake are important. In severe cases, intravenous hydration may be required. Paracetamol or ibuprofen is effective in treating pain and fever. Chlorhexidine mouthwash helps to prevent plaque accumulation if brushing teeth is painful. Oral antivirals are not usually needed in uncomplicated primary (...) gingivostomatitis. They are useful in severe cases or in immunocompromised people. (Hospital admission may be needed.) Discuss the course of the disease, the possibility of recurrence, transmission risks (advise people to avoid kissing and to wash their hands often), and stress the importance of avoiding immunocompromised people and neonates.” References 1) PRODIGY. Herpes simplex – oral. 2005 ( ) Answered 19 June 2006 Follow us: © 2019 Trip Database Ltd. company number 04316414. Trip is proud to be made

2006 TRIP Answers

508. Ellis-Van Creveld syndrome (PubMed)

and cardiac defects. After birth, cardinal features are short stature, short ribs, polydactyly, and dysplastic fingernails and teeth. Heart defects, especially abnormalities of atrial septation, occur in about 60% of cases. Cognitive and motor development is normal. This rare condition is inherited as an autosomal recessive trait with variable expression. Mutations of the EVC1 and EVC2 genes, located in a head to head configuration on chromosome 4p16, have been identified as causative. EVC belongs (...) to the short rib-polydactyly group (SRP) and these SRPs, especially type III (Verma-Naumoff syndrome), are discussed in the prenatal differential diagnosis. Postnatally, the essential differential diagnoses include Jeune dystrophy, McKusick-Kaufman syndrome and Weyers syndrome. The management of EVC is multidisciplinary. Management during the neonatal period is mostly symptomatic, involving treatment of the respiratory distress due to narrow chest and heart failure. Orthopedic follow-up is required

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2007 Orphanet journal of rare diseases

509. Selectively preventing development of third molars in rats using electrosurgical energy. (PubMed)

Selectively preventing development of third molars in rats using electrosurgical energy. Third molars are teeth with questionable value. People who never develop third molars avoid confronting the disease and pain these teeth often cause. Since third molars do not begin to develop until children are approximately five years of age, a window of opportunity exists to therapeutically prevent this tooth's development. The dentition of neonate rats possess developmental stages of molars similar (...) to that of five-year-old children. This pilot study tests the hypothesis that third molars can be selectively prevented from developing.Thirty-three neonate rats received a momentary pulse of electrosurgical energy to one of their maxillary tuberosities. The tuberosities on the contralateral sides received no treatment. Intraoral and radiographic examinations of sacrificed animals occurred when they were between 47 and 52 days old. Several tuberosity areas from sacrificed animals underwent histologic

2004 Journal of the American Dental Association

510. Loss of desmoplakin tail causes lethal acantholytic epidermolysis bullosa. (PubMed)

be indispensable, because DP-/- mice are early abortive. Here, we report a patient with severe fragility of skin and mucous membranes caused by genetic truncation of the DP tail. The new phenotype is lethal in the neonatal period because of immense transcutaneous fluid loss. The phenotype also comprised universal alopecia, neonatal teeth, and nail loss. Histology showed suprabasal clefting and acantholysis throughout the spinous layer, mimicking pemphigus. Electron microscopy revealed disconnection of keratin

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2005 American Journal of Human Genetics

511. Intra-oral colonization of macaque monkeys by Actinobacillus actinomycetemcomitans (PubMed)

Intra-oral colonization of macaque monkeys by Actinobacillus actinomycetemcomitans Actinobacillus actinomycetemcomitans was acquired by captive Macaca fascicularis 3 to 6 months after birth, and all monkeys aged over 6 months harbored detectable levels. This microorganism was most frequently isolated from the gingival plaque of the incisor (and other) teeth compared with other oral sites. Strains were leukotoxic by bioassay and Western blot analysis. Antibodies in macaque serum contained (...) neutralized the leukotoxin of a human A. actinomycetemcomitans strain. High titres of maternal neutralizing anti-leukotoxin antibodies were detected in neonates; the titre then fell rapidly so that by 6 months the antibody titer was zero. Antileukotoxin antibody production was detected after 6 months of age, rapidly reaching a high level within 2 years after birth. The presence of leukotoxic strains of A. actinomycetemcomitans in the gingival region did not appear to be correlated with an increase

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1989 Oral Microbiology and Immunology

512. Neonatal screening for hearing impairment--The Oman experience. (PubMed)

. In Phase III, 36 neonates were tested with ABR. Eleven were lost to follow up and eight children were advised to undergo further investigations. Ten children were found normal and 26 had hearing impairment. Six neonates had sensory-neuronal hearing loss, 17 children had otitis media with effusion and three children had atresia of the middle ear. The yield of hearing screening was 1.2/1000. The cost of screening was US$7.1/newborn.Universal hearing screening in Oman was useful but had teething problems (...) Neonatal screening for hearing impairment--The Oman experience. Oman introduced universal hearing screening at a national level in 2002 after piloting it in limited regions. Authors present their experiences.The screening had three phases. In Phase I, trained health staff of the delivery suits screened newborns by transient evoked otoacoustic emissions (TEOAE) test. In Phase II, otologists examined ears and repeated hearing tests after 6 weeks. Those who failed the repeat test were referred

2006 International Journal of Pediatric Otorhinolaryngology

513. Maturation of primary and permanent teeth in preterm infants. (PubMed)

Maturation of primary and permanent teeth in preterm infants. To elucidate the development of primary and permanent teeth and to interpret the effect of different calcium, phosphorus, and vitamin D supplementation in the neonatal period on dental maturation in preterm children.Preterm infants were randomised to four groups to receive a vitamin D dose of 500 or 1000 IU/day and calcium and phosphorus supplemented or unsupplemented breast milk. The maturity of the primary and permanent teeth (...) was not delayed compared with the controls (mean Demirjian SDS 0.16 v 0.49, p = 0.14). Early dietary intake of either mineral or vitamin D did not affect maturation of the primary dentition in preterm children. Children receiving the higher vitamin D dose in the neonatal period had more mature permanent dentition than those receiving the lower dose, but mineral intake did not affect maturation of the permanent teeth. Dental maturation did not correlate with bone mineral status.This is the first longitudinal

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2000 Archives of disease in childhood. Fetal and neonatal edition Controlled trial quality: uncertain

514. Hypoplasia of primary and permanent teeth following osteitis and the implications of delayed diagnosis of a neonatal maxillary primary molar. (PubMed)

Hypoplasia of primary and permanent teeth following osteitis and the implications of delayed diagnosis of a neonatal maxillary primary molar. A 2-year-and-6-month-old Japanese girl with delayed eruption of a maxillary right primary canine and disturbance in the development of maxillary right primary molars was examined. The crown of the maxillary right first primary molar was severely decayed and hypoplastic. The periodontal tissues on the maxillary right posterior region were swollen (...) and slight erythrogenic change was observed. Radiographic examination revealed a disturbance in the development of the permanent successor and the primary teeth in the maxillary right quadrant. According to the history, the maxillary right first primary molar had erupted on day seven after birth. It is suggested that the disturbance in development of the permanent successor and the primary teeth was secondary to osteitis caused by infection of the neonatal tooth. Appropriate dental treatment had not been

2003 International Journal of Paediatric Dentistry

515. PRENATAL AND NEONATAL VARIABLES ASSOCIATED WITH ENAMEL HYPOPLASIA IN DECIDUOUS TEETH IN LOW BIRTH WEIGHT PRETERM INFANTS (PubMed)

PRENATAL AND NEONATAL VARIABLES ASSOCIATED WITH ENAMEL HYPOPLASIA IN DECIDUOUS TEETH IN LOW BIRTH WEIGHT PRETERM INFANTS This study investigated possible prenatal and neonatal variables that may influence the prevalence of tooth enamel hypoplasia in preterm and low birth weight children (LBW) and a matched control group of term children with normal birth weight (NBW). The study sample consisted of 61 children born preterm and with LBW examined at 18-34 months of age. The control group (...) % had hypoplasia. LBW preterm infants presented a higher prevalence of hypoplasia than NBW controls. The deciduous teeth most affected by hypoplasia were maxillary incisors. There was no significant association with prenatal variables; among neonatal variables there was a significant association with respiratory distress syndrome and neurological examination at discharge with an altered result.

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2007 Journal of Applied Oral Science

516. Natal and neonatal teeth (PubMed)

Natal and neonatal teeth 12391001 2002 12 17 2008 11 20 1359-2998 87 3 2002 Nov Archives of disease in childhood. Fetal and neonatal edition Arch. Dis. Child. Fetal Neonatal Ed. Natal and neonatal teeth. F227 Sureshkumar R R Department of Paediatrics, Poole Hospital, Dorset, UK. drrsuresh@aol.com McAulay A H AH eng Case Reports Journal Article England Arch Dis Child Fetal Neonatal Ed 9501297 1359-2998 AIM IM Humans Infant, Newborn Infant, Premature Natal Teeth 2002 10 23 4 0 2002 12 18 4 0 2002

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2002 Archives of disease in childhood Fetal and neonatal edition

517. Natal and neonatal teeth in relation to environmental toxicants. (PubMed)

Natal and neonatal teeth in relation to environmental toxicants. Infants born to mothers heavily exposed to polychlorinated biphenyls (PCBs) and dibenzofurans (PCDFs) have earlier been reported to have increased prevalences of natal and neonatal teeth. Some tendency toward higher prevalence figures of natal and neonatal teeth can be seen in the literature in normal child populations during the last 40 y. We therefore decided to determine the present prevalence of these teeth in a Finnish (...) population and to evaluate whether infants with natal and neonatal teeth are more exposed to PCBs, PCDFs, and polychlorinated dibenzo-p-dioxins (PCDDs) than infants on average. A total of 34,457 infants born in 1997-2000 in four hospitals in southern Finland were examined for natal and neonatal teeth. The exposure of the infant to PCBs and PCDD/Fs was evaluated by measuring the levels of 17 most toxic PCDD/F and 36 PCB congeners in his or her mother's milk sample when the child was 4-8 wk old. A total

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2002 Pediatric Research

518. Ectopic teeth in the orbit of a neonate. (PubMed)

Ectopic teeth in the orbit of a neonate. 8562558 1996 03 01 2018 11 13 0007-1161 79 12 1995 Dec The British journal of ophthalmology Br J Ophthalmol Ectopic teeth in the orbit of a neonate. 1144-5 Reuser T T TT Cruysberg J R JR eng Case Reports Letter England Br J Ophthalmol 0421041 0007-1161 IM Humans Infant, Newborn Male Orbital Diseases congenital diagnostic imaging Tomography, X-Ray Computed Tooth Eruption, Ectopic diagnostic imaging 1995 12 1 1995 12 1 0 1 1995 12 1 0 0 ppublish 8562558

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1995 The British journal of ophthalmology

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