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At least 37 records · Page 2Linked to original sources
PubMed2001

Focal dermal hypoplasia: management of complex dental features.

A 17 year-old female who presented for treatment of grossly carious lower first molar teeth had multiple features of Focal Dermal Hypoplasia (FDH). These included enamel pitting and hypoplasia, anomalies of shape, size and positioning of teeth, as well as soft tissue papillomas and telangiectasis of tongue. A case report and a synopsis of the syndrome (FDH) are presented, the oral aspects of the condition are reviewed and the management of the complex dental anomalies is discussed.

Adolescent↗
PubMed1985

Ablepharon macrostomia syndrome.

The ablepharon macrostomia syndrome is a severe congenital condition that includes total absence of the upper and lower eyelids, failure of lip fusion that results in an enlarged, fish-like mouth, abnormally shaped ears and nose, absence of lanugo, ventral hernia, and ambiguous genitalia. In one such patient we were able to reconstruct the eyelids in a three-stage procedure. Redundant skin from the retroauricular area was used to create full-thickness grafts. The child later underwent successful mouth reconstruction. Although developmentally delayed, the child was eventually able to sit unassisted, to grasp objects, and to follow light with some fixation. Nystagmus was severe. The retina was attached in one eye and detached in the other. Corneal opacities present initially improved in one eye, allowing a view of the pupil and a normal anterior chamber.

Cornea↗
PubMed1999

Oral mucosa lesions in Mazahua Indian adolescents.

The epidemiologic data on oral lesions in native Indians remain unknown in many countries around the world. This paper reports the prevalence and distribution of oral congenital anomalies and pathologic lesions found in a survey of 107 schoolchildren (ages 12 to 17), from two isolated communities in the ethnographic Mazahua area in the State of Mexico. The main entities identified were: pigmented lesions (47.6%), lingual anomalies (17.4%) and developmental tooth alterations (6.9%). The remaining 24.4% of the lesions were gingival inflammatory hyperplasia, partial ankilosis of the tongue, lichen planus, focal epithelial hyperplasia and the double lip. The most frequent localization was lips and tongue. These findings suggest the high prevalence of oral anomalies in this Indian population. Therefore, we suggest that health programs should emphasize the prevention, diagnosis and treatment of these pathologies in Indians groups.

Adolescent↗
PubMed1989

Minor physical anomalies in schizophrenia.

This study was conducted to investigate the value of using physical anomalies (PAs) to evaluate early prenatal injury in schizophrenia. PAs are minor abnormalities in development of the head, hands, and feet that are presumably associated with insult during the first trimester. Sixty-seven schizophrenic inpatients and 88 normal controls were evaluated for PAs. The schizophrenic patients showed significantly more anomalies than the controls. The difference remained significant even when patients were compared to controls of low socioeconomic status. Both male and female patients showed a high incidence of mouth abnormalities, and female patients showed a high incidence of abnormalities in head circumference. Patients with early age of onset (less than or equal to 18 years) had more physical anomalies than did later onset patients. This relationship was most noticeable for males. Physical anomalies were not associated with deficits on measures of vigilance, selective attention, or orientation.

Adult↗
PubMed2004

Idiopathic autonomic neuropathy: comparison of cases seropositive and seronegative for ganglionic acetylcholine receptor antibody.

BACKGROUND: The clinical characteristics of autoimmune autonomic neuropathy are only partially defined. More than 50% of patients with high levels of ganglionic acetylcholine receptor (AChR) autoantibodies have a combination of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light response, upper gastrointestinal tract symptoms, and neurogenic bladder. OBJECTIVE: To compare patients with idiopathic autonomic neuropathy who were seropositive (n = 19) and seronegative (n = 87) for ganglionic AChR antibodies. DESIGN: Retrospective review of autonomic programmatic database. SETTING: Autonomic Disorders Program Project at Mayo Clinic College of Medicine, Rochester, Minn. PATIENTS: We evaluated a cohort of 87 patients with idiopathic autonomic neuropathy who had undergone full autonomic testing and neurological evaluation and who had a complete panel of paraneoplastic and ganglionic AChR antibodies. We compared patients seropositive (n = 19) and seronegative (n = 87) for ganglionic AChR antibodies. RESULTS: The seropositive group had a significant overrepresentation of abnormal pupillary responses (12/18 [67%] vs 12/87 [14%]; P<.001), sicca complex (9/15 [60%] vs 11/47 [23%]; P =.01), and lower gastrointestinal tract dysautonomia (16/19 [84%] vs 48/85 [56%]; P =.02). A subacute mode of onset was more common in the seropositive group (12/19 [63%] vs 23/84 [27%]; P =.004). Results of quantitative autonomic function tests differed significantly in the 2 groups only in the cardiovagal domain. Because subacute onset was overrepresented in the seropositive group, we analyzed the data separately, controlling for temporal profile (ie, the relationship between antibody status and symptoms while controlling for rate of onset). The relationships between antibody status and clinical profile (eg, presence of sicca complex, pupillary abnormalities, and lower gastrointestinal tract symptoms) generally remained significant regardless of onset rate, indicating that the associations are not due to temporal profile. CONCLUSIONS: These observations support the concept that ganglionic AChR antibodies are diagnostically and pathophysiologically important. Patients with orthostatic hypotension and prominent cholinergic dysautonomia are most likely to be seropositive for ganglionic AChR antibody.

Autoantibodies↗
PubMed2003

The spectrum of autoimmune autonomic neuropathies.

We analyzed the clinical characteristics of 18 patients (13 female, 5 male) who had autoimmune autonomic neuropathy (AAN) and ganglionic acetylcholine receptor (AChR) autoantibodies. Mean age was 61.4 years (standard deviation, 12.0 years). Ten patients had subacute symptom onset, six with an antecedent event. Eight patients had chronic AAN, characterized by insidious symptom onset, without antecedent event, and gradual progression. A majority of patients with high antibody values (>1.00 nmol/L) had a combination of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light response, upper gastrointestinal symptoms, and neurogenic bladder. Chronic AAN segregated into two subgroups. One subgroup (N = 4) had low antibody titer (0.09 +/- 0.01 nmol/L) and a paucity of cholinergic symptoms. It was indistinguishable from pure autonomic failure. The other subgroup (N = 4) had high antibody titer (11.6 +/- 2.08 nmol/L), sicca complex, abnormal pupils, and neurogenic bladder; three had severe upper gastrointestinal dysfunction. Higher antibody titers correlated with greater autonomic dysfunction and more frequent cholinergic dysautonomia. These observations expand the clinical spectrum of AAN to include chronic cases, some being indistinguishable from pure autonomic failure, and support the concept that ganglionic AChR antibodies are important diagnostically and pathophysiologically in acquired dysautonomia.

Adolescent↗
PubMed1988

Comparison of parotid and minor salivary gland biopsy specimens in the diagnosis of Sjögren's syndrome.

We conducted a prospective study comparing minor salivary gland and parotid gland biopsy specimens obtained simultaneously from 24 patients who were undergoing evaluation for primary Sjögren's syndrome (SS). Adequate tissue for study was obtained with all minor salivary gland biopsies and 19 of 24 parotid gland biopsies. Parotid inflammation was seen in 6 of 11 patients whose minor salivary gland biopsy results indicated SS, but in none of 8 patients who had normal findings on minor salivary gland biopsy. Patients with parotid inflammation were older and had a higher frequency of dry eyes and mouth, abnormal results on Schirmer's test, serious extraglandular involvement, and serologic abnormalities. We conclude that parotid gland biopsy adds very little to the minor salivary gland biopsy in the diagnosis of primary SS, but that parotid inflammatory changes may reflect disease duration and/or severity.

Aged↗
PubMed1994

Reconstruction of oropharyngeal defects utilizing a free radial forearm flap.

The authors report their experience with free radial forearm flaps for oropharyngeal reconstruction. Fifteen patients who submitted to intraoral reconstruction with this flap were followed for periods ranging from 3 to 36 months, with a mean of 14 months. Ages ranged from 15 to 58 years with a mean of 41. The defects were secondary to tumor ablation (11 patients), complications of conventional treatment for congenital deformities (3 patients) and trauma resulting from a gunshot wound to the upper lip and palate (1 patient). Total necrosis of 1 flap occurred and 1 patient required reoperation on the first postoperative day for revision of the anastomoses. Our results enable us to recommend the radial forearm flap as the flap of choice for reconstruction of extensive oropharyngeal defects.

Adolescent↗
PubMed1977

Rehabilitation of the individual with oral-facial anomalies and communicative disorders.

The Lancaster Cleft Palate Clinic was organized for the purposes of establishing, equipping, maintaining, and operating an institution, hospital for special services, or clinic for the treatment of all defective formations of the mouth, teeth, palate, and face; for the establishment and operation of a school for the retraining of those with speech or hearing defects associated with or without oral defects; and for the conduct of research in, and publication of information relative to, the above fields.

Face↗
PubMed1990

Persistent buccopharyngeal membrane with cleft palate. A case report.

A case of persistent buccopharyngeal membrane associated with cleft palate is reported. Persistence of the buccopharyngeal membrane results in partial fusion of the jaws and inability in opening and should be labeled as partial syngnathia. An association of this condition with cleft palate occurs as a part of the aglossia-adactylia and the popliteal pterygium syndromes. Though infrequently reported in the literature, presence of a persistent buccopharyngeal membrane associated with only cleft palate, without the other characteristics of the syndromes, does occur and should be recognized as a separate developmental defect.

Adolescent↗
PubMed1993

Chromosomal DNA probes for the identification of Bacteroides tectum and Bacteroides fragilis from the oral cavity of cats.

A dot-blot hybridisation assay using high molecular weight DNA as whole chromosomal probes was used to differentiate Bacteroides tectum from Bacteroides fragilis. 32P-labelled probes were compared with digoxigenin (DIG)-labelled probes. The whole chromosomal probes were specific--differentiating B. tectum from B. fragilis and both from a variety of other species (including other members of the genera Bacteroides, Fusobacterium, Eubacterium, and Prevotella) found in normal and abnormal mouths of cats and horses. However, even at very high stringencies, B. tectum homology groups I, II and III were not distinguishable from one another using either 32P-labelled or DIG-labelled probes. Thus, DIG-labelled whole chromosome probes directed against cellular DNA released directly onto nitrocellulose membranes is considered a useful method for diagnostic veterinary laboratories wishing to identify B. tectum and distinguish it from B. fragilis and other oral anaerobic flora of cats.

Animals↗