ABSTRACT
Objective To summarize the clinical characteristics of neonatal asplenia syndrome to improve the understanding of the disorder. Methods Newborns with neonatal asplenia syndrome admitted to the neonatal department of our Hospital from June 2008 to July 2015 were recruited and their gender, birth weight, gestational age, clinical manifestations, imaging features, laboratory examinations, treatments and prognosis were analyzed. Results A total of 8 newborns with asplenia syndrome were studied. All the patients presented with cyanosis at birth, 5 of them with heart murmur, 3 with apical impulse in the right ( dextrocardia) thorax and complex congenital heart diseases were found in all of them by echocardiogram examinations. 2 of them were diagnosed congenital heart diseases before birth by the fetal ultrasound. 2 cases combined with congenital imperforate anus and cutaneous fistulas. 1 case was found right renal agenesis and Howell-Jolly was seen in another case. All the 8 patientsˊ parents refused medical advices about continuing treatments and left hospital. Among them, 4 patients died in the neonatal period, 2 lost in follow-up and only 2 survived, one for 19 months and the other for 4 years. Conclusions Patients with asplenia syndrome presented absence of spleen, visceral inversion, severe cardiovascular malformations and other malformations. When the cyanosis, heart murmur and abnormal position of apical impulse were found in the newborns, asplenia syndrome should be considered and imaging examinations such as chest X-ray, ultrasound, visceral angiography and more should be ordered immediately, in order to make an early diagnosis of the disease.
ABSTRACT
Objective To study the predictive validity of general movements (GMs) assessment for motor development outcomes in neonatal purulent meningitis.Methods According to the inclusion criteria,excluding other cerebral injury diseases,a total of 34 cases of neonatal purulent meningitis,who took part in our follow-up clinic after discharged from neonatal intensive care unit of the Second Affiliated Hospital of Wenzhou Medical University from December 2012 to December 2013,were confirmed as the participants.Infants' GMs recordings during writhing movements period (at least once at 0-3 months) and fidgety movements period (at least once at 3-5 months) were collected and assessed.Motor development outcomes was determined at least after one year old with Alberta Infant Motor Scale(AIMS).Predictive validity in each period were calculated with AIMS results as the golden standard.Results Among the 34 cases,there were 24 males and ten females,and four preterm infants and 30 term babies.The age at follow-up was from 12 months to 23 months old.Ultimately,one (3％) infant with spastic cerebral palsy,seven (21％) with motor retardation and 26 (76％) with normal motor development were diagnosed.The sensitivity,specificity,positive predictive value,negative predictive value,Youden index and residual error rate for the writhing movements period in prediction of motor development outcomes were 87.5％,46.2％,33.3％,95.6％,33.7％ and 12.5％,respectively.And the corresponding figures for the fidgety movements period were 87.5％,88.5％,70.0％,95.8％,76.0％ and 12.5％,respectively.Conclusions GMs assessment could be an accurate predictive tool for later motor developmental outcomes in neonates with purulent meningitis,especially in fidgety movements period.