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[Unusual traumatic alterations of the tongue (author's transl)].

A baby with congenital sensoric neuropathy showed, due to missing sensitivity to pain, repeated injury of the tongue resulting from bites after the front teeth had erupted. The defect formed at the tip of the tongue and showed excessive granulation resembling at first sight a tumorous process. Such alterations of the tongue usually are the first symptom of this rare disease and therefore are most important for diagnosis. With another infant presenting with Joubert's syndrome, i.e. cerebellar malformation, macroglossy, increased motility of the tongue and severe dyspnoic attacks combined with seizures, also tumorous granulomatous alterations of the tongue were found, due to chronic injury of the tongue by bites.

Child, Preschool↗

Anesthetic management of the pediatric patient with multiple congenital anomalies including severe hemifacial hypertrophy.

An anesthesia induction technique not widely utilized is described for a pediatric patient presenting with severe hemifacial hypertrophy, severe mental retardation, as well as a seizure disorder and other congenital anomalies. The anatomically and/or pathologically altered airway as well as other anomalies increase the risks of anesthesia.

Abnormalities, Multiple↗

[Somatomedin activity in Wiedemann-Beckwith syndrome].

Two patients with Wiedemann-Beckwith-Syndrome and neonatal hypoglycemia are reported. The etiology for the impaired glucose metabolism has not been elucitated as yet. In some patients pancreatic islet-cell hyperplasia resulting in hyperinsulinemic hypoglycemia was suspected. Increased somatomedin activity was also proposed. We have measured somatomedin concentrations in both patients by bioassay and radioimmunoassay and found normal or slightly reduced plasma levels with both methods.

Asphyxia Neonatorum↗

Clinical diagnosis of malformation syndromes: syndromatology in paediatrics.

The term malformation syndrome should be retained for designation of characteristic symptom complexes of clear-cut pathogenesis. Classification of disorders into any of the three categories deformation, malformation respectively dysplasia or into any combination of them leads to better insight into morphological syndromes. Ten rules of the diagnostic procedure have been formulated and illustrated by the example of the Beckwith-Wiedemann syndrome. Disturbed organogenesis is usually accompanied by multiple signs of dysmorphy, a careful search for malformations is therefore desirable. In the case of malformation syndromes interfering with growth and development continuous care of the patient is indispensable.

Abnormalities, Multiple↗

Survey of neuromuscular disorders in Lebanon. I. Profile of the genetically determined muscular dystrophies.

The clinical and laboratory profiles of 130 cases of muscular dystrophy in Lebanon collected over 35 years (1947-1982) are presented. Data collection included hospital records; registries in the department of pathology, hospital laboratories, electron microscopy facility and electromyography laboratory; and records from a private hospital neurosurgical unit. The yield from each of these sources is presented. The data was fed into an Apple II computer and analyzed using thirty seven parameters. The results are discussed and compared with three other similar studies from India and America.

Adolescent↗