254 resultados para Congenital Deafness


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Patients with congenital nasolacrimal obstruction (CNLO) were evaluated at the Lacrimal Apparatus Service of Botucatu Faculty of Medicine - UNESP from 1990 to 1993. 45 children were seen at this period with CNLO. The diagnosis was confirmed by dacryocystography (DCG). Obstruction was unilateral in 77,7% of the patients; 68,1% at Arlt Sinus and 43,6% had lacrimal sac grade 1. Probing was made in children older than 6 months, under general anaesthesia, after DCG was performed. Cure was greater in children age 6 to 12 months with obstruction at Hasner's Valve and without dilation of lacrimal sac.

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A presentation of two cases of Dubowitz syndrome in monozigous twin girls. The syndrome is a rare congenital disorder, the main clinical aspects of which include retarded intrauterine and post-natal growth, microcephaly, peculiar face and an eczemic rash, resulting from photosensitivity of the regions exposed to sunlight.

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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.

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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.

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Introduction: The study of otolaryngologic manifestations in children HIV + can lead to early diagnosis of AIDS, allowing specific treatment, responsible by reduced morbidity and mortality. Objectives: Detect the otolaryngologic manifestations in children with AIDS and alert to the importance of the early diagnosis. Study design: Clinical prospective. Material and method: We evaluated 22 children with AIDS assisted at Faculdade de Medicina de Botucatu (São Paulo, Brasil). The medical records were reviewed and the children were submitted to otolaryngologic and hearing acuity exams. Results: We evaluated 12 boys and 10 girls, whose ages ranged from 8 months to 12 years. In B and C clinical classification were included 18 children who were using anti-retroviral medicaments. Physical examination mainly indicated cervical lymphadenopathy (18 cases), paleness of the nasal mucous membrane with abundant mucous secretion over the nasal epithelium (15 cases) and retraction of tympanic membranes (seven cases). The main otolaryngologic diagnoses were: rhinosinusitis (16 cases), oral candidiasis (13 cases), inadequate eustachian tube function (seven cases) and recurrent tonsillar infections (six cases). Conductive hearing loss were detected in 4 children. No child presented sensorineural hearing loss. Conclusions: The main otolaryngologic manifestations presented by the children with HIV virus were rhinosinusitis, oral candidiasis, inadequate eustachian tube function and recurrent tonsillar infections. The allergic aspect of the nasal mucous membrane and the cervical lymphadenopathy were frequent signs and could alert the otolaryngologyst to AIDS during the exam.

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Vitamin A and its derivatives, retinoic acid, tretinoin and isotretinoin, are currently used in dermatological treatments. The administration of high doses of this vitamin provokes congenital malformations in mice: cleft palate, maxillary and mandibular hypoplasia and total or partial fusion of the maxillary incisors. This study compares the tooth germs of the first maxillary and mandibular molars of fetal mice submitted to isotretinoin during organogenesis. Twelve 60-day-old female Mus musculus were divided into two groups on the 7th day of pregnancy: treated group--1 mg isotretinoin per kg body weight, dissolved in vegetable oil, was administered from the 7th to the 13th day of pregnancy; control group--vegetable oil in equivalent volume was administered orally for the same period. On the 16th day of pregnancy, the females were sacrificed, the fetuses were removed and their heads amputated. After standard laboratory procedures, 6-micron thick serial slices were stained with hematoxylin and eosin for optical microscopy examination. The results showed that both groups had closed palates with no reminiscence of epithelial cells; however, the first molar germs of the isotretinoin-treated animals showed delayed development compared to the control animals.

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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.

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Study model: observacional, retrospective. Objective: to determine the frequence of the ametropic errors and other ocular problems in children with 2 to 8 year-old at Piracicaba - SP. Patients and Method: During the school year of 2000, 1001 children enrolled at the public schools of Piracicaba - SP, age ranged from 2 to 8 years old, were referred to complete ophthalmological exam. Visual acuity was previously determined using Snellen chart, applied by school teachers. Those children presenting visual acuity equal or less than 0.8, visual complaints or visual disorders were selected to appointment. Results: 51 children (5.09%) did not attended to examination. 950 children were submitted to complete ophthalmological exam. Ametropic errors were found 70.84% of the children. The most prevalent refractive errors were Hypermetropic Astigmatism (49.62%) and Hypermetropia (32,98%). Anisometropia was found in 1.78% children. Other ocular disabilities accounted for 10.21% of the examined children, such as strabismus (3.36%), eyelid changes, allergic conjunctivitis, congenital dacryostenosis, optic atrophy, corioretinitis and congenital glaucoma. Conclusion: The frequence of ocular problems observed let us to conclude the screening programs are valid surveys on decreasing rates of preventable blindness in our country.

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The pregnant women presenting Diabetes mellitus develop metabolic alterations, that may cause damage to the fetal well-being and provoke anomalies and/or malformations. The antioxidant treatment has improved the embryonic development from streptozotocin diabetic rats. Several studies have shown that a Gingko biloba extract presents antioxidant effects and, in the present study, one of the G. biloba extract formulations was used (EGb761) - Tebonin (200 mg/Kg/day), given to the diabetic pregnant female rats. The aim was to evaluate the effect of the EGb761 treatment on the of anomalies and/ or malformations incidence of the offspring. Diabetes was induced in female rats using streptozotocin in a dose of 40 mg/kg. The rats were mated, and the pregnant animals were divided in two groups: Control (water) and experimental (G. biloba). At day 21 of pregnancy, the rats were killed, and their fetuses were analyzed and processed for anomalies and/or malformations incidence. The results demonstrated that control and experimental groups presented no external anomalies and malformations; increased incidence of skeletal anomalies and of visceral malformations, and lower rate of visceral anomalies and skeletal malformations. These data confirm no statistical difference and, therefore, EGb761 treatment did not cause changes. Thus, a dose of 200 mg/Kg/day of a Gingko biloba extract given during the pregnancy rat was ineffective in the prevention of the anomalies and/or malformations related to the diabetes.

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Objective: To review the literature on inhaled nitric oxide and to describe its main clinical applications in pediatrics. Sources of data: A 10 year literature review with selection of the most important publications on inhaled nitric oxide, using the Medline and Cochrane Systematic Review databases. Summary of the findings: This review was organized as follows: introduction; metabolism and biological effects; clinical applications; dosage, gas administration and weaning; precautions and side-effects. Inhaled nitric oxide use was described in persistent pulmonary hypertension and hypoxia of the newborn, acute respiratory distress syndrome, primary pulmonary hypertension, heart surgery, chronic obstructive pulmonary disease, sickle cell anemia, and bronchospastic disease. Conclusions: Inhaled nitric oxide is a therapeutic approach with wide clinical applications in pediatrics. Its use is safe when administered in pediatric intensive care units under strict monitoring. As a pulmonary vasodilator, nitric oxide has beneficial effects on gas exchange and ventilation. Controlled trials, focusing on early gas administration should be performed under many clinical conditions, especially acute respiratory distress syndrome.

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Double lip is a developmental abnormality which may occur either isolated or as a component of Ascher's syndrome. A case of congenital double lip associated to hemangiomas and enlargement of the thyroid is reported. The oral and facial hemangiomas were excised under local anesthesia. Surgical reconstruction of the upper lip was done in order to reduce the interference with speech and mastication. The recognition and treatment of the abnormalities are discussed.

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Purpose: To analyze the cinedacryocystography in children with congenital nasolacrimal obstruction suspicion. Methods: The cinedacryocystographic examination was done in 143 children (58% of girls and 42% of boys) younger than 5 years old, suspected of congenital nasolacrimal obstruction. The exams were done under general anesthesia and the children were probed immediately when obstruction was detected. Results: Epiphora, discharge or both were the most common complaints. Nevertheless 11% children had undergone unsuccessfully probing once, no one had any radiologic evaluation previously. The cinedacryocystographic exam showed nasolacrimal duct obstruction in 65,73%. Permeable lacrimal vies were presented in 34,3% of the children with epiphora. The obstruction was mainly at the Arlt's sinus. Using the cinedacryocystography, it was possible to observe the lacrimal sac enlargement and alterations in the contiguous structures such as hypertrophic turbinates (91,1%), sinusitis (44,6%) and septum deviation (24,1%). Conclusion: Cinedacryocystographic evaluation provides important information about lacrimal system obstruction in children, therefore, it is useful in all children with suspected congenital lacrimal obstruction.

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The midline cervical cleft is an unusual congenital anomaly of the ventral neck and fewer than 100 cases have been reported overall and the first described by Bailey in 1924. This anomaly is report in association with median cleft of lower lip, cleft mandible and tongue, and hypoplasia of other midline neck structures. Its considered an anomaly originated from the two first branchial arches. The treatment of this cleft is a vertical complete excision and a closure with multiple Z-plasty. Many authors recommend avoid linear closure and prefer multiple Z-plasty for evicted fibrosis and local retraction. In this paper we report 2 case of this anomaly and the literature is reviewed.

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Arsenic is an environmental pollutant that influences the male reproductive function, affecting the spermatogenesis process in the testis and causing alterations in the sperm. The objective of this work was evaluate the reproductive efficiency in adult male rats exposed to arsenic and the possible male-mediated structural malformations. The animal were treated with sodium arsenato (3,6 mg/Kg, i.p.), twice a weak, for 15, 30, 45, 60 or 75 days whereas control received only distilled water. Results have showed a reduced pregnancy rate associated to a decrease in the fertility potencial and to a increase in the preimplantation loss. There was a decrease in the fetal weights, as showed by its average. Malformations rate, postimplantation loss and resorptions were unaffect by treatment. These results suggest that chronic exposure to arsenic provokes reduction on fertility of male rats. Further studies are required to clarify the arsenic effects on male reproductive system and male-mediated effects on progeny.

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This article assembled the congenital lacrimal obstruction according embryology, clinical presentation, propedeutic exams and other differential anomalies.