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A mediastinal neuroblastoma developed in a female infant with Wiedemann-Beckwith syndrome. Careful and close examinations in children with Wiedemann-Beckwith syndrome should therefore not only include screening for Wilms tumour but also for neuroblastoma.
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A syndrome was observed in two sisters which was characterized by a median defect of the hair of the head, hypoplasia of the ala nasi, absence of the permanent teeth, microdontia of the deciduous teeth, and exocrine insufficiency of the pancreas. This syndrome is probably autosomal regressive; the symptoms are similar to those of Johanson-Blizzard syndrome.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
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Obstructive sleep apnea (OSA) is relatively common in children with Down Syndrome due to anatomical and immunological variations which place them at particular risk. Secondary cardio-respiratory complications of upper airway obstruction may be found at the time of initial diagnosis; these require medical therapy before surgical correction of the OSA is attempted. The selection of appropriate surgical therapy is influenced by the anatomical structure of the individual child. Tonsillectomy and adenoidectomy alone infrequently provide lasting relief of obstruction for these children. We have found that uvulopalatopharyngoplasty (UPPP), with or without simultaneous anterior reduction of the tongue, alleviates this form of airway obstruction. This paper describes the procedures and reports our experience of six patients with Down syndrome.
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An improved simplified technique is described for reducing the volume of the tongue by operation. A plastic support has been designed on which the tongue can be spread out symmetrically and fixed in position. Gallbladder forceps are applied to the base of the tongue to avoid hemorrhage. An initial incision is made through the whole thickness of the tongue down to the support, and a partial resectomy performed, followed by apposition of the wound edges. The results obtained with this procedure are far superior to those previously reported using other techniques: the operation takes less time and there is almost no loss of blood.
A case of 17 years old female patient with the open bite connected with dimension enlargement and changed function of the tongue has been described. After the operation of the tongue by means of Reinwald method an active upper base with barrier and rubber traction between paracentral upper and lower incisors have been applied. The open bite was eliminated within one year the speech and appearance of the patient improved at the same time.
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Amyloidosis associated with myeloma and its current classification are examined. The clinical example is reported of a patient with isolated micromolecular plasmocytoma in whom the amyloidosis appeared during the remission of the primary disease. The patient was not seen until a late stage when he was admitted to hospital with severe cardiac decompensation. Echocardiography revealed the location to be the cardiac muscle. The increase in left ventricular mass revealed by the ECG was in contrast with the low ECG voltages. Biopsy showed involvement of the oral cavity. The condition resisted all treatment and the patient died a few months after the onset of the amyloidosis. The view that amyloidosis associated with plasma cell dyscrasias should be classified with primary amyloidosis is supported.