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Biomedical subjects

K I Yau

Publications and source records attributed to K I Yau.

At least 19 recordsLinked to original sources

Neurobehavioral development at term in very low-birthweight infants and normal term infants in Taiwan.

We compared the neurobehavioral performance at term between very low-birthweight (VLBW) infants and term infants in Taiwan, and investigated the relationships between neonatal factors and neurobehavioral performance in VLBW infants. Sixty VLBW infants and 58 healthy term infants were examined using the Neonatal Neurobehavioral Examination-Chinese version (NNE-C) at 40 weeks postmenstrual age. Medical records of the VLBW infants were reviewed to assess neonatal factors. The mean total score of the preterm infants (67.4+/-5.0) was significantly lower than that of the term infants (73.8+/-3.0) (t = 8.51, P < 0.0001). Furthermore, respiratory illness had a marginal effect on the rate of low neurobehavioral score (defined as 2SD below the mean score of term infants) in the preterm infants after adjustment for gestational age (odds ratio = 7.67, chi2 = 3.36, P = 0.067). Our findings indicate that preterm infants have lower neurobehavioral scores at term than their healthy term counterparts. Furthermore, respiratory illness may be a potential risk factor for low neurobehavioral score at term in preterm infants when gestational age is adjusted for.

Apgar Score

The controversy regarding diagnostic criteria for early myoclonic encephalopathy.

To re-evaluate the diagnostic criteria for early myoclonic encephalopathy (EME), the following study was done. During the past 2 years, five patients with erratic, fragmentary myoclonus of neonatal onset, in association with other types of seizures, were analyzed with regard to etiologies, electroclinical features and their evolution, using a series of examinations including electroencephalographies (EEGs) and metabolic investigations. Of these five patients, three were diagnosed to have non-ketotic hyperglycinemia (NKH); one was pyridoxine-dependent; the other was cryptogenic. Only two cases (one NKH and one cryptogenic) had initial typical suppression-burst (S-B) EEG pattern, which subsequently evolved into multiple paroxysmal abnormalities with random asynchronous attenuation (MP-AA) pattern. The other two cases with NKH had MP-AA EEG pattern throughout both awake and sleep recordings in two consecutive EEG studies. All three cases with NKH survived with increasing microcephaly, muscle tonicity; all developed infantile spasm with hypsarrhythmia on EEGs. The patient with pyridoxine-dependency had an initial MP-AA EEG pattern, which converted into S-B pattern after the first use of pyridoxine, eventually becoming normal after a supplement with the second-dose of pyridoxine. In conclusion, either S-B or MP-AA pattern may reflect the severity of the underlying pathologies or the disease stages. These results suggest that, from both etiological and electroclinical viewpoints, EME may represent a broader spectrum than previously recognized. The still ongoing controversy regarding whether the S-B pattern should be recognized as the sole EEG criteria for the diagnosis of EME needs further experience to clarify.

Age of Onset

The human tail.

The human tail is a congenital anomaly with a protruding lesion from the lumbosacrococcygeal region. A newborn with a tail-like structure over the coccygeal area observed since birth is presented. Lipoma accompanied by tethered spinal cord were found. In reviewing the literature from 1960 to 1997, 59 cases were described. Higher incidences of spinal dysraphism (49.15%) and tethered spinal cord (20.34%) compared with previous reports were evident. This fact plays an important role in understanding the disturbance of development and regression of human tails. A new classification according to whether the anomaly appears in combination with spinal dysraphism is proposed for clinical usage. Preoperative detailed image studies are needed to clarify the possibility of tethered spinal cord syndrome developing in the future and thus prevent it. Magnetic resonance imaging is the modality of choice if available. Long-term follow-up for possible sequelae after operation, especially in cases with spinal dysraphism, is necessary.

Animals

Etiology and outcome of hydrops fetalis.

Hydrops fetalis is a morbid condition caused by a wide variety of diseases. Although treatment for some underlying pathologies is becoming available, the prognosis remains poor. To analyze the etiology and outcome of hydrops fetalis, we reviewed the records of 79 fetuses encountered at the National Taiwan University Hospital between January 1993 and October 1996. The mean +/- standard deviation gestational age at presentation was 24.9 +/- 6.3 weeks. One case was due to RhE incompatibility. Seventy-eight fetuses were diagnosed as having nonimmune hydrops fetalis; this was idiopathic in 20 fetuses. In the other 58, the causes and associated conditions were classified into eight groups: hematologic (25), cardiovascular (15), chromosomal (5), gastrointestinal (4), cystic hygroma (3), pulmonary (2), infection (2), and miscellaneous (3). One fetus with cystic hygroma also had a chromosomal abnormality (Turner's syndrome). The most common cause of nonimmune hydrops fetalis was homozygous alpha-thalassemia (31%). Twenty-nine fetuses were lost to follow-up after initial evaluation and 50 fetuses were delivered at our hospital. Of these, there were eight cases of antepartum death, 24 cases of intrapartum death, and 18 live births. Seventeen of the live-born infants were treated at our neonatal intensive care unit, of whom only seven survived.

Adult

Early outcome of extremely low birth weight infants in Taiwan.

We retrospectively evaluated the outcome and the risk factors for mortality among extremely low birth weight (ELBW) infants born at National Taiwan University Hospital. The records of all live-born infants with body birth weight of less than 1,000 g from January 1, 1993, to December 31, 1996, were evaluated. Infants with major anomalies or whose parents refused resuscitation were excluded from the analysis. There were 81 ELBW infants (0.59%) among a total of 13,835 live births during the study period, and 73 cases were enrolled for study. The mean gestational age (GA) was 27.2 (range, 24-34) weeks. Sixty-six percent of the ELBW infants were born by cesarean delivery. Respiratory distress syndrome occurred in 64% of infants and exogenous surfactant therapy was given to 47%, while intermittent mandatory ventilation was given to 85%. Symptomatic patent ductus arteriosus occurred in 34% of infants, septicemia in 30%, chronic lung disease in 48%, grade III to IV intraventricular hemorrhage in 27%, stage III to V retinopathy of prematurity in 33%, and necrotizing enterocolitis in 8%. Neonatal survival was 74%, survival to discharge was 60%, and intact survival was 50%. The survival rate was 40% for infants with a birth body of weight less than or equal to 750 g, and 68% for those with a birth body weight of greater than 750 g. While survival was 27% for infants with a GA of less than 26 weeks, it was 75% for those with a GA of greater than or equal to 26 weeks. The survival rate improved year by year for those with a GA less than 28 weeks. Cox regression analysis of survival showed that Apgar score at 1 minute (p = 0.0063), pulmonary hypertension (p = 0.012), and severe intraventricular hemorrhage (p = 0.0031) were the most important factors associated with mortality. Though the outcome of ELBW infants in our institute seems poorer than in some more developed countries, it is improving. The prognosis for ELBW infants of 24 to 25 weeks' GA remains guarded under our present care.

Female

Mature teratoma arising from an intra-abdominal undescended testis presenting as a fetal abdominal mass.

Teratoma of the intra-abdominal testis is a rare finding in infants. We describe the case of a full-term newborn treated for a calcified abdominal mass which was observed unexpectedly on prenatal sonography. An undescended right testis was also noted. During laparotomy, a twisted retroperitoneal tumor was found just above the right deep inguinal ring. Histological analysis revealed a mature teratoma of the intra-abdominal right testis. The tumor was removed and there was no recurrence at follow-up 1 year later. A teratoma should be considered in cases of fetal abdominal mass, especially when the testes cannot be detected in the scrotum by the 8th month. Prenatal sonographic diagnosis might be possible.

Adult

Tricho-rhino-phalangeal syndrome type II associated with epiglottic aplasia and congenital nephrotic syndrome.

A male neonate with tricho-rhino-phalangeal syndrome type II (Langer-Giedion syndrome) and interstitial deletion of chromosome 8 with karyotype 46, XY, del (8) (q24.11-->q24.13) is reported. In addition to hypotrichosis of the scalp hair, abnormally bulbous nose and redundant skin, which are usually found in this syndrome, aplasia of the epiglottis and non-Finnish type congenital nephrotic syndrome were also present. He died of renal failure and respiratory failure at 11 days of age. Electron microscopy of renal necropsy showed foot process loss, focal thickening and splitting of the glomerular basement membrane and mesangial expansion. These have never been reported and could be new associations in this disease that may serve to support the concept of contiguous gene syndrome in patients with tricho-rhino-phalangeal syndrome.

Epiglottis

Predictive pulmonary function of school children in an area of low air pollution in Taiwan.

The objective of this study was to determine the effect of different parameters on a predictive model of pulmonary function for elementary school children in an area of low air pollution in Taiwan. Healthy children aged 7 to 12 years from three elementary schools in low-air-pollution areas (Da-Chen, Mai-Liau and Tai-Si) participated in the study. A total of 836 children (423 boys and 413 girls) were included in the study. During summer vacation, each child underwent a physical examination including a screening spirometry. A questionnaire regarding respiratory symptoms and indoor air pollutants was also completed by the children's parents. Air monitoring showed that the levels of outdoor pollutants were relatively low. Multiple linear regression analysis was performed with FVC (forced vital capacity) and FEV1 (forced expiratory volume in 1 sec) as dependent variables. Gender, age, height, weight, technician and indoor air pollution parameters were the independent variables. The results showed that gender,height, weight and technician were the most significant variables for predicting FVC and FEV1. The various indoor air pollution parameters seemed to have no influence on the pulmonary function of children, except that mildew in bedrooms mildly decreased FEV1. Regression analysis showed that all the pulmonary function parameters measured had a positive correlation with height, whereas weight correlated only with certain parameters. Because both indoor and outdoor air pollution was relatively low, we suggest that this model could be used as a basic predictive model of pulmonary function for elementary school children in Taiwan.

Air Pollution

Reliability of the Neonatal Neurobehavioral Examination--Chinese version.

A Chinese version of the Neonatal Neurobehavioral Examination (NNE-C) was applied to 15 high-risk infants and five normal term infants for investigation of reliability. The infants were assessed by three physical therapists to examine inter-rater reliability and reassessed by one of the therapists within 2 days to examine test-retest reproducibility. The internal consistency of the NNE-C scale was high, with an alpha coefficient of 0.84. The inter-rater reliability was high for item scores (kappa coefficients > 0.75 for 81% of the items) and for section and total scores (all intraclass correlation coefficients > 0.80). The test-retest reproducibility was moderate for item scores (kappa coefficients > 0.40 for 85% of the items) and was high for section and total scores (all intraclass correlation coefficients > 0.80). We conclude that the NNE-C scale is clinically feasible and reliable for the evaluation of neurobehavioral functions of high-risk and normal term infants in Chinese-speaking societies.

Behavior

Lung mechanics in infants with left-to-right shunt congenital heart disease.

To clarify which hemodynamic measurement correlates best with lung mechanics in infants with congenital heart disease and left-to-right shunts, dynamic pulmonary function tests and echocardiography were performed in 26 infants with such disease (study infants) and in 37 normal, healthy infants (control infants). The tidal volume and pulmonary compliance (CL) were lower and airway resistance higher in infants with congenital heart disease than in control infants. A significant correlation was demonstrated between CL, expiratory resistance (Re), and the right pulmonary artery-to-aortic size ratio (RPA/DAO). CL and Re also correlated well with the corrected acceleration time square root of RR ratio (ACT/square root of RR: ACT, acceleration time and RR: length of the cardiac cycle) of pulmonary flow velocity. Stepwise multiple regression analysis revealed that RPA/DAO correlated best with both CL and Re. It is concluded that infants with congenital heart disease and left-to-right shunts have lower lung compliance and higher expiratory airway resistance than normal children, and that RPA/DAO is the echocardiographic parameter that correlates best with the changes in lung mechanics.

Airway Resistance

Nonprogressive congenital unilateral ventriculomegaly.

Congenital unilateral ventriculomegaly is a rare condition, usually caused by obstruction of the foramen of Monro. In the past, this condition required surgical intervention. We present a female newborn with nonprogressive unilateral ventriculomegaly which was initially detected by prenatal sonography. No surgical intervention was performed, and during the 9 months of follow-up, she had normal head growth and reached appropriate developmental milestones.

Cephalometry

Intrauterine intervention in a case of recurrent meconium peritonitis.

We present a case of recurrent meconium peritonitis detected in the second trimester and treated by intrauterine intervention. Antenatal ultrasound findings included fetal ascites and intra-abdominal calcification. Aspiration of fetal ascites under ultrasound guidance and determination of the bilirubin concentration established the diagnosis of meconium peritonitis. Paracentesis was repeated to remove irritating intestinal contents and to decrease pressure on the fetal thorax. Although the exact cause of the meconium peritonitis remains unknown, the recurrence of the condition suggests a genetic basis. A possibility of cystic fibrosis was not considered because the clinical picture did not suggest it. Intrauterine intervention helped to establish the diagnosis of meconium peritonitis and may have contributed to the good outcome.

Adult

Pulmonary mechanics and the energetics of breathing in healthy infants.

In order to understand the change in the pulmonary mechanics and energetics of breathing in infants, dynamic pulmonary function tests were studied on 84 occasions in 70 normal healthy infants between birth and seven months of age. All of the study infants were born at full-term and had no lower respiratory infections after birth. Airflow and transpulmonary pressure were measured simultaneously and utilized to calculate values of pulmonary mechanics and energetics of breathing. Variables of ventilation (respiratory frequency, tidal volume, minute ventilation, and inspiratory time) had good correlation with age. Lung compliance increased with age. However, when it was expressed in body weight the lung compliance was independent of age (1.34 +/- 0.37 mL/cmH2O/kg for infants studied). Pulmonary resistance did not differ statistically between different age-groups and it correlated with the level of lung compliance (r = 0.3411, p < 0.01). Older infants had a higher flow rate and esophageal pressure change. This might imply that the older the infant is, the more forceful the breath becomes. The work of breathing was higher in infants of older age and in infants with higher inspiratory pulmonary resistance or lower lung compliance (r = 0.6257, 0.3825 and 0.4579, respectively; p < 0.05). This study establishes reference values for the pulmonary mechanics and energetics of breathing in infancy which may be of assistance in the objective evaluation of respiratory status in infants.

Female

Persistent hyperinsulinemic hypoglycemia due to nesidiodysplasia in a neonate.

A large, one-hour-old female baby was noted to have hypoglycemia immediately after birth. Although early feeding and then intravenous glucose infusion with a dose gradually increased to 30 mg/kg/min were given, the baby still had recurrent hypoglycemia 14 days after birth. High serum insulin/glucose ratios (> 2.5) were noted twice during hypoglycemic episodes. At the same time, the baby's serum growth hormone, thyroid hormone and cortisol levels were within normal ranges. Hyperinsulinism due to nesidiodysplasia was diagnosed and a near-total pancreatectomy was performed at the age of 14 days. After the operation, euglycemia was achieved and there was no episode of hypoglycemia even after fasting for eight hours. Our experience confirms that surgery is the treatment of choice for patients, including neonates, with hyperinsulinemic hypoglycemia.

Female

Growth and body composition of preterm, small-for-gestational-age infants at a postmenstrual age of 37-40 weeks.

In order to understand the nutritional status of preterm, small-for-gestational-age (SGA) infants in the early postnatal period, the growth and body composition of preterm, SGA infants was followed prospectively from birth to the postmenstrual age of 37-40 weeks. The infants were stratified into different groups by gestational age, clinical condition and body proportionality. In each subgroup, the growth and changes in body composition of SGA infants were compared with appropriate-for-gestational-age (AGA) infants of a comparable postmenstrual age. At birth, the SGA infants of both the 31-33 and 34-36 week gestational-age groups were smaller than AGA infants in all body measurements, including arm area (AA), arm muscle area (AMA) and arm fat area (AFA). When the preterm SGA infants had grown to the postmenstrual age of 37-40 weeks, the amount of fat they had accumulated was as much as, or more than that in term AGA infants. Yet, they had less muscle mass and their body weight, body length and head circumference were less than those in term AGA infants. This pattern of growth and the changes in body composition had been persistently observed in SGA infants of different gestational-age groups, different clinical status and different body proportionality. Differences between postnatal enteral nutrition and placental nutrition, or different energy utilization, in preterm SGA infants are hypothesized to account for these observations. The growth of less mature (31-33 weeks gestation) SGA infants and those preterm SGA infants with an eventful clinical course was suboptimal as compared with other SGA infants in the same subgroup. In this study, the weight to length ratio (WLR) was used to define the status of nutrition in preterm SGA infants: WLR < or = 2 S.D. or > 2 S.D. off the reference mean. Infants in both groups showed some catch-up growth in body weight. Yet, at near-term their body weight were still more than 2 S.D. below the mean of term AGA. In each gestational-age group, the growth of these two body-proportionality groups did not differ from each other except for the low WLR group of 34-36 weeks gestation which had a significantly lower body weight and skinfold thickness than the group with a normal WLR. Multiple regression analysis revealed that skinfold measurements of preterm SGA infants at birth is the best factor for determining the body weight gain at near-term. After use of the skinfold thickness was set aside, WLR became the most important factor.(ABSTRACT TRUNCATED AT 400 WORDS)

Anthropometry

Weight to length ratio--a good parameter for determining nutritional status in preterm and full-term newborns.

To identify which parameter showed the strongest correlation with neonatal body fat store, when the ratios for assessing both weight-for-length and the mid-arm circumference to head circumference (MAC/HC) were included in the analysis, body anthropometrics and skinfold thickness were measured in 250 full-term and 125 preterm infants. Among the study cases, 66.7% were appropriate for gestational age, 26.7% were small for gestational age and 6.7% were large for gestational age. Sum of the skinfold thickness measured at the midtricepital and subscapular areas correlated well with body anthropometrics, weight/length ratio, body mass index, ponderal index and mid-arm circumference to head circumference ratio. Multiple stepwise regression analysis revealed that the weight/length ratio correlated best with skinfold thickness in both full-term and preterm newborn infants. Therefore, the simple weight/length ratio might be useful for evaluation of the nutritional status of intrauterine growth, and in the prediction of metabolic complications in both full-term and preterm newborns with abnormal intrauterine growth.

Adipose Tissue