Perioral Cyanosis Research Articles

Cystic hygroma of the mediastinum in children.

Cystic hygroma of the mediastinum is rare. Although 53 cases have been reported, only two of these were described in pediatric journals. 1,2 In reporting another instance of the condition, we shall stress not only the difficulty of diagnosis, but also the hazard of failure to diagnose. Report of a Case First Admission. —The patient was a white girl weighing 2,500 gm (5 lb 8 oz) at birth. At 2 months of age, she was admitted in severe respiratory distress with cough, perioral cyanosis, flaring of the alae nasi, and deep substernal retractions. Her temperature was 98.5 F (36.9 C); the heart rate was 130 beats per minute. On physical examination there was a dullness over the left anterior chest and a decrease in breath sounds over both anterior lung fields. There were no other abnormal physical findings. Laboratory Findings. —The complete blood cell count was within normal limits, and

Ocular Histopathology in Generalized Glycogenosis (Pompe's Disease)

Although glycogen has been reported previously in mural cells of the retinal vessels³with generalized glycogenosis, the present case is the first comprehensive description of the ocular changes in this disease. <h3>Report of Case *</h3><h3>Clinical Aspects.</h3> —The patient was a 5-month-old female infant on the pediatric service of l'Hôpital Saint-Pierre (Prof. R. Dubois). The presenting symptoms were dyspnea, perioral cyanosis, and palpebral edema for one month. One sister had developed symptoms similar to those of the patient and had died at the age of four months. The present patient's previous history had not been unusual. She was born at full term without incident. Physical development was normal to the age of four months. On admission the patient was 63 cm (2 ft) in length and weighed 5.9 kg (13 lb). She was listless, with generalized hypotonia; her skin was pale and swollen; she was dyspneic with a circumscribed perioral