Early intervention for infants with autistic spectrum disorders in Japan

BACKGROUND: To date, many researchers in Japan have assumed that the cause of autistic spectrum disorders is attributable to some disorder in the ability of the child. However, we have been working on the premise that autistic spectrum disorders are brought about by relationship disturbances in early infancy and have been attempting to validate this hypothesis through early intervention. METHODS: We have examined the developmental process of affective communication in infants with autistic spectrum disorders. We have postulated that approach-avoidance motivational conflict (Richer) is the primary factor impeding the development of affective communication and have focused therapeutic intervention on this perspective. RESULTS: As a result, attachment behavior was markedly improved in children, but affective communication with their mothers was not. Examing the mothers' images of themselves in infancy in mother-infant psychotherapy, problems that the mothers had themselves in infacy with attachment behavior to their own mothers affected the mothers' internal representation of their children, leading to active evolution of mother-child interaction and development of affective communication between the mother and child. CONCLUSIONS: In this context, the basis and significance of the internal representation of both parties being determinants in the quality of mother-child communication are discussed. Our goal in early intervention is not the elevation of a child's linguistic-cognitive abilities, but the creation of a comforting relationship in which both parent and child can live securely, without strain.     

Continuous bilateral electrophrenic pacing in an infant with total diaphragmatic paralysis.

An infant with bilateral diaphragmatic paralysis, etiology undetermined, is presented in whom bilateral intrathoracic phrenic nerve pacemakers were inserted. He was maintained with continuous bilateral electrophrenic pacing for 142 days. A review of the literature by the authors has not revealed any previously reported cases.     

Ultrasound monitoring of diaphragm activity in bilateral diaphragmatic paralysis.

Recovery of diaphragm activity after bilateral diaphragmatic paralysis was monitored in a term infant using a mechanical sector scanner fitted with a 5 MHz transducer. The ratio of diaphragmatic excursion during spontaneous breathing and ventilator assistance was used an objective measure for comparison of diaphragmatic activity during recovery. Ultrasound assessment of diaphragm contraction may be used to study progress in diaphragmatic paralysis.     

先天性膈疝术后肺功能转归的临床研究

目的探索先天性膈疝患儿术后肺功能的转归情况。方法收集2016年11月至2019年3月在上海交通大学医学院附属新华医院小儿外科门诊随访并行肺功能检查的膈疝患儿肺功能数据,同时收集患儿治疗时的临床资料,包括患儿性别、膈疝类型、Apgar评分、出生体重、肝脏位置、血气分析、呼吸机维持时间和住院周期等进行统计分析。结果45例患儿纳入分析,其中男19例,女26例;左侧膈疝39例,右侧6例。共行89次肺功能检查,随访时间为(9.3±7.3)个月,20例患儿行1次检查,25例行2次及以上检查。以最近一次肺功能检查数据为准,45例患儿中呼吸功能正常者有7例(15.6%),呼吸功能存在异常组有38例(84.4%)。肺功能检查表现为潮气呼吸功能正常、阻塞性通气功能障碍、混合性通气功能障碍、限制性通气功能障碍的分别为10次、62次、11次和6次。肺功能检查时间、膈疝类型和肝脏疝入情况与肺功能检查结果存在相关性(P<0.05)。与限制性通气功能障碍组患儿的月龄相比,阻塞性通气功能障碍组患儿的月龄较大[(8.9±6.9)个月比(1.3±0.5)个月,P=0.001]。25例接受多次肺功能检查结果显示,随着患儿年龄的增长,其"达峰时间比"和"达容时间比"显著增加(P<0.05)。结论部分先天性膈疝患儿术后早期存在不同程度的肺功能异常,需给予高度重视;年龄、膈疝类型和肝脏疝入情况可能是影响肺功能异常的危险因素。     

Ventilation modalities in infants with congenital diaphragmatic hernia

Neonates with congenital diaphragmatic hernia are among the more complex patients to support with mechanical ventilation. They have particular features that add to the difficulties already present in the neonatal patient. A ventilation strategy tailored to the patient’s underlying physiology rather than mode of ventilation is a crucial issue for clinicians treating these delicate patients.     

Postdischarge follow-up of infants with congenital diaphragmatic hernia

Infants with congenital diaphragmatic hernia often require intensive treatment after birth, have prolonged hospitalizations, and have other congenital anomalies. After discharge from the hospital, they may have long-term sequelae such as respiratory insufficiency, gastroesophageal reflux, poor growth, neurodevelopmental delay, behavior problems, hearing loss, hernia recurrence, and orthopedic deformities. Structured follow-up for these patients facilitates early recognition and treatment of these complications. In this report, follow-up of infants with congenital diaphragmatic hernia is outlined.     

Retinol status of newborn infants with congenital diaphragmatic hernia

The etiology of congenital diaphragmatic hernia (CDH) is not yet known. Studies in the literature from 1941 have reported that nutritional deficiency of vitamin A during pregnancy could lead to CDH, associated or not with other malformations in young rats. More recently, possible correlations between expression patterns of cellular retinoid-binding protein and retinoic-acid receptors and morphologic effects of vitamin A deficiency have been suggested. The purpose of this study was to verify in human newborns the possible link between vitamin A deficiency and CDH previously observed in experimental animals. Blood samples were obtained during the first hours after birth from 11 term CDH newborns and 11 healthy controls matched for gestational age, and also from 7 mothers in each group, for a total of 7 newborn-mother pairs of matched CDH-controls. Plasma retinol was measured by high-performance liquid chromatography and retinol-binding protein (RBP) by nephelometry. In the 11 matched CDH-control newborns, plasma retinol and RBP levels in CDH newborns were 50% less than control values (P< 0.0002 and <0.006, respectively); in contrast, retinol levels in CDH mothers were significantly higher than those of control mothers (P < 0.005). The observation that the plasma concentrations of retinol and RBP are low in infants with CDH relative to controls may be clinically very relevant and may help to elucidate the mechanism of development of this congenital anomaly.     

Long-term follow up of infants with congenital diaphragmatic hernia

With improving treatment strategies for congenital diaphragmatic hernia (CDH) infants, an increase in survival of more severely affected patients can be expected. Consequently, more attention is now focused on long-term follow up of these patients. Many reports have emphasized associated morbidity, including pulmonary sequelae, neurodevelopmental deficits, gastrointestinal disorders, and other abnormalities. Therefore, survivors of CDH remain a complex patient population to care for throughout infancy and childhood, thus requiring long-term follow up. Much information has been provided from many centers regarding individual institutional improvements in overall survival. Few of these, however, have reported long-term follow up. The aim of this review is to describe the long-term outcome of survivors with CDH and to suggest a possible follow-up protocol for these patients.