Subject(s)
Air Pollution, Indoor/adverse effects , Allergens , Respiratory Sounds/etiology , Child , Female , Humans , MaleABSTRACT
Respiratory mechanics were studied in nine infants with glossoptosis-apnoea syndrome to determine whether glossoptosis may account for signs of both inspiratory and expiratory airway obstruction. Airflow, oesophageal pressure, inspiratory and expiratory time (Ti and Te), and inspiratory and expiratory resistance (Ri and Re) were measured before and during ventilatory phases characterised by glossoptotic pharyngeal obstruction, induced by turning the infants onto their backs. In addition, an attempt was made to correlate the abnormalities in pulmonary mechanics with the clinical features. During partial glossoptotic pharyngeal obstruction, a significant increase was observed in Te and Re and variable changes in Ti and Ri. During severe obstruction, the infants displayed obstructed inspiratory efforts often associated with stridor, as well as obstructed expiratory efforts often associated with audible grunting and retarded expiratory flow pattern. The expiratory grunt was loudest over the neck and mimicked bronchospasm over the chest. These findings indicate that glossoptotic pharyngeal obstruction induces functional airway obstruction which may affect both inspiration and expiration. Expiratory airway obstruction seems, at least in part, to be due to active braking of expiratory flow.
Subject(s)
Airway Obstruction/etiology , Posture/physiology , Respiratory Mechanics/physiology , Tongue Diseases/complications , Airway Obstruction/physiopathology , Choanal Atresia/complications , Esophageal Atresia/complications , Female , Humans , Infant , Infant, Newborn , Inhalation/physiology , Male , Micrognathism/complications , Syndrome , Tongue Diseases/physiopathologyABSTRACT
The natural history of primary megaureter was evaluated in 26 infants (20 boys, 6 girls) with 38 nonrefluxing primary megaureters. Prenatal ultrasound diagnosis had been made in 21 infants. In all patients, primary megaureter was associated with pelvicaliceal dilatation. Two patients with urinary tract infection and two with agenesis of the contralateral kidney underwent surgical treatment. Asymptomatic infants with good differential renal function on DTPA scanning were managed conservatively regardless of their postfurosemide drainage curve. One of these infants later developed a slight deterioration in function and was submitted to surgery. One patient was lost to follow-up. Twenty patients, after a period ranging from 4 months to 8 years, showed partial or complete regression of ureteral dilatation with stable good renal function. In conclusion, many asymptomatic primary megaureters in infancy improve spontaneously and do not require surgical treatment.
Subject(s)
Hydronephrosis/congenital , Ureteral Obstruction/congenital , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Hydronephrosis/surgery , Infant, Newborn , Kidney Function Tests , Male , Pregnancy , Prenatal Diagnosis , Ureteral Obstruction/surgery , Urinary Tract Infections/surgery , UrographyABSTRACT
Patients with esophageal atresia (EA) or choanal atresia/stenosis (CA) present with many clinical features of maturational dysautonomia (DY). Since CA and DY are considered manifestations of cephalic neurocristopathy, we tested the hypothesis that EA may also be related to faulty development of cephalic neural crest. Forty-eight patients with EA and 53 with CA were followed up to study the frequency of the facial anomalies which are regarded as the phenotypic expression of an abnormal cephalic neural crest contribution to facial embryogenesis. Forty-eight patients with EA and 51 with CA had clinical manifestations of DY. Forty-four patients with EA (91%) and 49 with CA (92%) had one or more facial anomalies. Comparing the groups, patients with EA had an increased frequency of unilateral facial anomalies of branchial arch derivatives (P < .01); those with CA had an increased frequency of anomalies of frontonasal process derivatives (P < .01). These findings support the hypothesis that EA may be related to an abnormal contribution from the cephalic neural crest. The presence of facial anomalies may facilitate the diagnosis of subclinical DY.
Subject(s)
Abnormalities, Multiple/embryology , Autonomic Nervous System Diseases/etiology , Choanal Atresia/embryology , Esophageal Atresia/embryology , Facial Bones/abnormalities , Neural Crest/embryology , Autonomic Nervous System Diseases/complications , Choanal Atresia/complications , Embryonic and Fetal Development , Esophageal Atresia/complications , Humans , Infant, Newborn , Skull/abnormalitiesABSTRACT
Patients with esophageal atresia (EA) or choanal atresia (CA) manifest similar clinical and pathophysiological features. To determine the significance of this observation, the clinical records of 80 patients with EA and 57 with CA were reviewed. This survey showed that similarities between the two conditions included inspiratory and expiratory dyspnea, episodes of reflex apnea and/or bradycardia, oropharyngeal dysphagia, vomiting, convulsions, hyperhydrosis, hyperthermia, sialorrhea, and sudden death. After the second year of life most symptoms disappeared spontaneously. In both conditions, respiratory effort resulted in partial or complete obstruction affecting both the inspiratory and expiratory phases of the respiratory cycle. Support for this finding was obtained by studying the breathing pattern of 3 patients with EA and 3 with CA, before and during postural respiratory loading. The data suggest that patients with EA are similar to those with CA, having upper airway instability that may result in obstructive hypopnea or apnea associated with expiratory grunting. It is possible that this upper airway instability is a manifestation of more general maturational dysautonomia previously not recognised in patients with EA.
