FG syndrome in a Brazilian child with additional previously unreported signs.

We report on a Brazilian child with typical manifestations of the FG syndrome. Pigmentary dysplasia, metacarpal fusion and peculiar anatomopathological findings are additional undescribed signs.

Interstitial long-arm deletion of chromosome 7 and ectrodactyly

An interstitial deletion of 7q21 was found in a boy with mental retardation, microcephaly, convergent strabismus, micrognathia, genital anomalies, and other findings, including ectrodactyly.

New treatment for frontal sinus hypertrophy

A new treatment of frontal sinus hypertrophy is described. The anterior wall is removed, inverted, and attached again. The resulting depression is filled with bone dust. Details are discussed, and a case is presented.

Prevalência de anodontia entre estudantes do 2O grau da cidade de São José dos Campos-Correlação dessa anomalia entre terceiros molares e outros órgão dentários.

The authors studied 201 school children form both sexes, aging 12 to 14 years, in order to identify anodontia, including in third molars. The results indicated a congenital absence of teeth in 24.37% of the examined children, distributed into 10.44% of boys and 13.93% of girls. Anodontia was observed concerning third molar teeth and other teeth, and the correlation was calculated by the total, sex, and hemiarch. The data were presented and discussed, and the results included several conclusions.

Freqüência de raízes fusionadas, separadas divergentes, separadas convergentes, separadas retilíneas e dilaceradas dos terceiros molares.

In a sample of Brazilian white young adults between 18-23 years old, the A.A. studied the third molar roots, in anatomical aspects, using the orthopantomographic radiographic method. They concluded that the superior third molar shows a biggest number of fusionated roots (19.50% in the right side and 19.66% in the left side) and the inferior third molar shows a biggest number of separated roots (21.48% in the right side and 19.66% in the left side).

Incontinentia pigmenti (Bloch-Sulzberger syndrome): a case report.

A syndrome--incontinentia pigmenti--of probable genetic etiology, is discussed and a new case report presented. The syndrome presents systemic and dental manifestations, which are separate although similar to those found in other disease entities, i.e., congenital syphilis, hereditary ectodermal dysplasia.

Fístula arteriovenosa pulmonar maciça. Causa rara, potencialmente curável de hipóxia neonatal.

The case of a six-day-old neonate admitted in an emergency situation because of dyspnea and increasing cyanosis is reported. Despite abnormal opacification on the chest X-ray and left ventricular overload on the electrocardiogram and echocardiogram, features compatible with the disease, the diagnosis of massive pulmonary arteriovenous fistula affecting the whole left superior lobe, was made possible only after necroscopic examination.

Natal teeth: Case report

The natal teeth are defined as the presence of teeth at birth and the etiology is rather obscure. Clinically, the teeth show normal size and shape, although they reveal an immature appearance. The histological aspect show enamel with the possibility of presenting normal mineralization or being hypoplastic. The dentin may show alterations. The incidence of natal teeth varies greatly, with a predisposition for the female sex. This paper relates a case report of two partially erupted natal teeth in a female baby 9 days-old. The presence of teeth made the parents anxious. In their mind, that meant the child was abnormal. On the radiograph the erupted teeth showed little radioopacity, without a radicular formation. The therapy utilized was the keeping of the teeth and a periodic monitoring, cleaning and daily topical applications sodium fluoride. The case was monitored by monthly consultations. At 9 months of age the primary central lower did not present mobility. On the radiograph examination incomplete radicular formation was observed.

Sindrome das bandas ou constricoes amnioticas: Relato de caso

The authors present a case of constriction band syndrome in a 20-year-old male. There were constriction rings in the interphalangeal joints of hands and in the thigh, shortening of the lower limb and atrophic left gastrocnemius muscle. These findings belong to the described syndrome, which is uncommon and has no genetic implication. Personal antecedent: convulsive syndrome and corrective surgery for congenital bent foot.

Espinha bífida e mielodisplasia em ruminantes: Relato de caso

Spina bifida with mielodysplasia are described in calf and lamb in this report. Locomotor abnormalities in the pelvic limbs and vertebral malformation were found in both animals, and arthrogryposis was also present in the calf. The mielodysplasia was very similar in these animals, and both were characterized by non separation of ventral and dorsal horns, areas with medular canal duplication and syringomyelia. No cases of spina bifida was previously described in Brazil.

Testicular ultrastructure and morphology of the seminal pathway in Prochilodus scrofa

The seminiferous tubules of Prochilodus scrofa present a coiled morphological arrangement with intertubular anastomoses and unrestricted spermatogonial distribution. The structural pattern of the seminiferous tubules is cystic, with cysts formed by cytoplasmic prolongations of Sertoli cells. Inside the cysts are observed different types of germ cells. The seminiferous tubules open individually on the ventral surface of the main testicular duct present in each testis. Each main testicular duct prolongs as a spermatic duct, fusing with the spermatic duct of the opposite side to form the common spermatic duct which opens into the urogenital papilla. The mature sperm cysts break and extravasate their content into the lumen of the seminiferous tubules from which the seminal fluid and the spermatozoa penetrate the main testicular duct, the spermatic duct and the common spermatic duct for semen ejaculation.

Seqüência de moebius: Manifestações clínicas e avaliação auditiva

Introduction: Children with Moebius syndrome may present paralysis of cranial nerves III, IV, V, VI, VII, VIII, IX, X and XII, compromising motor and sensorial functions. Hands and feet defects (syndactyly, equinovarus and arthrogryposis) are frequently associated. These manifestations can be attributed to the use of misoprostol during pregnancy to induce abortion. Study design: Clinical prospective. Aim: To evaluate the main clinical manifestations, hearing acuity and possible etiologic factors in children with Moebius syndrome. Material and method: The children were submitted to clinical, otolaryngological and hearing acuity assessment. Hearing acuity was evaluated through behavioral tests, pure tone audiometry, tympanometry and auditory brainstem response (ABR). We investigated possible etiologic factors. Results: Three boys and two girls were evaluated. The main manifestations were: facial paralysis, paralysis of masseter muscle, defects in dental occlusion, retraction of tympanic membrane, equinovarus, oblique palpebral fissure and tongue atrophy. Conductive hearing loss was detected in three children and sensorineural hearing loss in one child. The use of misoprostol during pregnancy was reported by four mothers. Conclusions: The children with Moebius syndrome evaluated in the present study manifested palsies of various cranial nerves, especially V, VII and XII nerves, responsible for motor and sensorial alterations. Inadequate eustachian tube function associated to conductive hearing loss was frequent. The use of misoprostol during pregnancy was reported by the mothers and it was considered a possible etiologic factor.

Natal and neonatal teeth: Review of the literature

Child development from conception through the first years of life is marked by many changes. Tooth eruption follows a chronology corresponding to the date when the tooth erupts into the oral cavity. These dates have been established in the literature and are subjected to small variations depending on hereditary, endocrine and environmental features. At times, however, the chronology of tooth eruption suffers a more significant alteration in terms of onset, and the first teeth may be present at birth or arise during the first month of life. The expectations about the eruption of the first teeth are great and even greater when the teeth appear early in the oral cavity. The objective of the present study was to present a review of the literature with important aspects about natal and neonatal teeth.

Cytokeratins in epithelia of odontogenic neoplasms

Neoplasms and tumours related to the odontogenic apparatus may be composed only of epithelial tissue or epithelial tissue associated with odontogenic ectomesenchyme. The immunohistochemical detection of different cytokeratins (CKs) polypeptides and vimentin has made it easier to explain the histogenesis of many epithelial diseases. The present study aimed to describe the immunohistochemical expression of cytokeratins 7, 8, 10, 13, 14, 18, 19 and vimentin in the epithelial components of the dental germ and of five types of odontogenic tumours. The results were compared and histogenesis discussed. All cells of the dental germ were positive for CK14, except for the preameloblasts and secreting ameloblasts, in which CK14 was gradually replaced by CK19. CK7 was especially expressed in the cells of the Hertwig root sheath and the stellate reticulum. The dental lamina was the only structure to express CK13. The reduced epithelium of the enamel organ contained CK14 and occasionally CK13. Cells similar to the stellate reticulum, present in the ameloblastoma and in the ameloblastic fibroma, were positive for CK13, which indicates a nature other than that of the stellate reticulum of the normal dental germ. The expression of CK14 and the ultrastructural aspects of the adenomatoid odontogenic tumour probably indicated its origin in the reduced dental epithelium. Calcifying odontogenic epithelial tumour is thought to be composed of primordial cells due to the expression of vimentin. Odontomas exhibited an immunohistochemical profile similar to that of the dental germ. In conclusion, the typical IF of odontogenic epithelium was CK14, while CK8, 10 and 18 were absent. Cytokeratins 13 and 19 labelled squamous differentiation or epithelial cells near the surface epithelium, and CK7 had variable expression.