phenylephrine-hydrochloride and Myotonic-Dystrophy

The aim of this study is to assess if, and to what extent, myotonic dystrophy can affect the craniofacial growth pattern.. The research was conducted on a sample of 27 patients with Steinert's myotonic dystrophy (study group). Each subject underwent a clinical examination with impression-taking and intra- and extraoral photographs. A latero-lateral projection teleradiography in the mirror position was also taken and a cephalometric examination was performed. The assessed values were compared with those obtained from a group of healthy subjects (control group).. Statistical analysis of the data obtained from the myotonic patients who developed the disease during the growth phase revealed alterations in the transversal plane and, to an even greater extent, the vertical one, with a high frequency of anterior open bite. Discussion and conclusions Regarding the pathogenesis of these types of skeletal dysplasias, the authors hypothesise a posterior rotation growth pattern, resulting from gravitational force prevailing over the deficit of the elevator muscles.

Topics: Adolescent; Adult; Age of Onset; Cephalometry; Child; Chin; Female; Humans; Male; Mandible; Maxilla; Maxillofacial Development; Models, Dental; Myotonic Dystrophy; Nose; Open Bite; Photography, Dental; Skull; Skull Base; Young Adult

We investigated the relationship between nasal flaring and SpO2 in 19 patients with Duchenne muscular dystrophy (DMD) and 26 patients with myotonic dystrophy (DM1). In DMD patients, nasal flaring was observed when SpO2 was lower than 96%, while it was not seen even at 82% of SpO2 in DM1. None of the DM1 patients could perform voluntary nasal flaring. Nasal flaring is a useful indicator of hypoxemia in DMD but not in DM1. It remains to be elucidated whether the lack of nasal flaring in DM1 patients is due to abnormal respiratory central mechanism or nasal muscle weakness.

Topics: Adult; Aged; Blood Gas Analysis; Facial Muscles; Humans; Hypoxia; Middle Aged; Muscular Dystrophy, Duchenne; Myotonic Dystrophy; Nose; Oxygen; Respiratory Center

In this report we present two infants with congenital myotonic dystrophy (CMD) who were successfully weaned from prolonged ventilatory support using nasal continuous positive airway pressure (N-CPAP). The first infant received 127 days of endotracheal mechanical ventilation as part of 141 days of total ventilatory support, including N-CPAP; the second infant received 27 days of endotracheal mechanical ventilation as part of 84 days of total ventilatory support. Noninvasive N-CPAP facilitated weaning these two infants from ventilatory support, thereby minimizing the morbidity associated with prolonged intubation. The developmental outcomes of our two infants were comparable to infants not requiring prolonged endotracheal mechanical ventilation. We suggest that this noninvasive modality of ventilatory support may be advantageous in the management and beneficial to the outcome of infants with CMD who are respirator-dependent >30 days.

Topics: Adult; Female; Humans; Infant, Newborn; Intubation, Intratracheal; Male; Myotonic Dystrophy; Nose; Positive-Pressure Respiration; Pregnancy; Respiratory Insufficiency; Ventilator Weaning