[A comparative study of temperament and maternal rearing pattern in preschoolers with congenital heart disease and preschoolers generally].

The purposes of this study were: (1) to understand the differences in temperamental characteristics between preschoolers with congenital heart disease (CHD) and preschoolers generally, and (2) to discuss the relationship between characteristics of children temperament and maternal rearing patterns. The research subjects based on convenience sampling were 61 preschoolers with CHD and 76 non-CHD preschoolers. The "children temperament questionnaire" and "maternal rearing pattern rating scale" were posed to the mothers of the subjects and were filled in by them. The results showed that mothers of children with mild heart symptoms tended to adopt more protective rearing patterns than mothers of non-CHD children. Children in the group "whole heart function without symptoms" scored higher points for activity than children in the non-CHD group. Preschoolers with mild heart symptoms had greater intensity of reaction and persistence than non-CHD preschoolers, but statistically, there were no significant differences in other temperament characteristics between preschoolers with and without CHD. These results could serve as reference for nursing care of children with CHD and maternal rearing practice.

Tumor necrosis factor-alpha and interleukin-6 profiles in children with pneumonia.

Pneumonia is a common cause of hospitalization and is associated with high morbidity in children. Tumor necrosis factor-alpha (TNF-alpha) and Interleukin-6 (IL-6) are primary mediators of inflammation, and have been implicated in a large number of infectious and non-infectious inflammatory diseases. The serum concentrations of TNF-alpha and IL-6 were measured by enzyme-linked immunosorbent assay (ELISA) in 27 patients with bacterial pneumonia (n = 12) or respiratory syncytial virus (RSV) pneumonia (n = 15) and in 15 healthy control subjects. TNF-alpha concentrations of patients with bacterial pneumonia in acute stage (16.94 +/- 5.70 ng/L) were significantly higher than those in convalescent stage (5.80 +/- 0.75 ng/L), in patients with RSV pneumonia (5.06 +/- 0.44 ng/L) and in healthy control subjects (5.39 +/- 0.68 ng/L) (p < 0.005). TNF-alpha concentrations of patients with RSV pneumonia were not significantly different from those of the control group. IL-6 concentrations of patients with bacterial pneumonia in acute stage (465.94 +/- 290.30 ng/L) were significantly higher than those in convalescent stage (22.04 +/- 15.08 ng/L), in patients with RSV pneumonia (7.65 +/- 2.58 ng/L), and in healthy control subjects (0.84 +/- 0.08 ng/L) (p < 0.0005). There was significant difference between patients with RSV pneumonia and the healthy control group (p < 0.005). In summary, there were significant differences in TNF-alpha and IL-6 concentrations between acute stage and convalescent stage in patients with bacterial pneumonia, making them useful as markers for bacterial pneumonia. Further studies are needed to establish the potential diagnostic and prognostic value of TNF-alpha and IL-6.

Tracheal agenesis with multiple congenital anomalies: a case report.

Tracheal agenesis (TA) is a rare congenital anomaly that is incompatible with prolonged life. It may occur alone or with other associated anomalies. A term infant presented with cyanosis, hypotonia, absence of crying and respiratory distress at birth. Intubation was difficult. Esophageal intubation was performed under laryngoscopy. As TA was suspected, a bronchoscopy was performed and the infant was found to have a normal epiglottis and vocal cords; however, there was no trachea. Cardiorespiratory deterioration developed and the patient died on the night of the second day at the postnatal age of 41 hours. Tracheal agenesis was confirmed at autopsy. Associated anomalies included bronchoesophageal fistula, double outlet of the right ventricle with ventricular septal defect, bicuspid pulmonary valve, single lobe of the right lung, imperforate anus and a rectourethral fistula. According to development theory, tracheal agenesis and VACTERL (vertebral defects, anal atresia, cardiovascular defects, tracheoesophageal fistula, radial dysplasia or renal defects and limb defects association may result from a mesodermal deficiency caused by abnormal blastogenesis.

DiGeorge syndrome with microdeletion of chromosome 22q11.2: report of one case.

DiGeorge syndrome (DGS) is a congenital anomaly involving developmental defects of the third and fourth pharyngeal pouches. Thymic aplasia or hypoplasia, parathyroid aplasia or hypoplasia, cardiac malformations, and dysmorphic facies are characteristics features. We present a case which had thymic aplasia, hypocalcemia, facial dysmorphism (hypertelorism, low set ears, cleft of soft palate, fish-like mouth and micrognathia) and congenital heart disease (ventricular septal defect, perimembranous type). The T-cell immunologic functions as a percentage of T-cell and phytohemagglutinin stimulation test were within normal range matched with age. Molecular study showed microdeletion of chromosome 22q11.2 by genotype analysis, but chromosome study of high-resolution cytogenetic analysis by G-banding technique was normal. To our knowledge, about 90% of DiGeorge syndrome patients show chromosome abnormalities, most involving chromosome 22 (monosomy of 22q11.2). In the past, most cases were proven by high-resolution cytogenetic analysis or fluorescence in situ hybridization(FISH). We report a case of DGS in Taiwan with microdeletion of chromosome 22q11.2 detected by genotype analysis.

Transcatheter embolization of coronary artery fistula: a case report.

Coronary artery fistula is rare, but it is the most common congenital coronary artery anomaly with hemodynamic significance. It usually causes no symptoms in young patients but may be associated with symptoms and complications in older patients. Surgery has been the traditional treatment. In this report, a 7-year-old girl who had a coronary artery fistula from the left circumflex coronary artery to the right atrium was successfully treated by percutaneous transcatheter technique.

Data quality of a computerized medical birth registry.

Processed by a computerized medical birth registry system, the birth records of 20,103 deliveries, from February 1992 to February 1993, were digitized with medium registry. From 1 January to 28 February 1993, the original records (n = 2840 cases) of all 10 collaborative hospitals were requested for assessment of data quality. Thirty-six items were scored, data of poor quality was found in eight; acceptable quality in four; and good quality in 28. The feasibility of data transfer by floppy disc and per modem was evaluated. This registry system had effectively shortened data processing time and improved mutual feedback between the data centre and the delivery units. Errors resulting from technical faults originating in the preparation of data for computerization at hospital level could be effectively reduced. The validity of diagnosis remained as the major source of errors.

Amphetamine poisoning in infant: report of two cases.

Amphetamine poisoning is rare in children. Here we report two male infants with acute poisoning due to accidental amphetamine ingestion. One infant had a family history of drug abuse and the other was due to poor supervision of the parents. Although typical clinical symptoms and signs (including restlessness, hyperactivity, hypertension, tachycardia and tachypnea....etc.) were found, both were completely recovered after treatment. The principle of management of amphetamine poisoning are presented.

Central venous silastic catheters in newborns and children: localization by sonography and radiology.

Central venous catheters are commonly used in neonatal and pediatric intensive care. Sonographic catheter localization is a technique without any known detrimental side effect. The positioning of silastic central venous catheters in two different diameters used in critical newborns and children was examined by sonography in this study. The sonographic findings were compared with the corresponding radiographic results. Even the very thin catheters could be easily detected due to their strong echoes. Results of forty catheter positions, including malpositions, demonstrate accuracy and practicability of this technique. Sonography may become an alternative examination to reduce the frequency of control radiography.

Selective placement of bronchial suction catheters in intubated full term and premature neonates.

Flexible straight suction catheters were passed through nasal endotracheal tubes in 30 newborn infants (gestation age 26-42 weeks; birth weight 800-4,100 g) undergoing mechanical ventilation just before chest radiographic examination for clinical purpose. With the head in midline position, 63% (19/30) and 37% (11/30) of the catheters, entered the right and left main bronchus, respectively. With the head turning to either the left or right side, 87% (26/30) and 73% (22/30) of the catheters, entered the contralateral bronchus, respectively. Both success rates increased significantly (p less than 0.05 and p less than 0.01). Thus, turning the head facilitates passage of the cather into the contralateral mainstem bronchus in full term and premature neonates.

[Retrospective study of Salmonella gastroenteritis in infants].

UNLABELLED: Sixty-four cases of Salmonellosis were retrospectively analyzed in Jan. 1985 to Dec. 1988 at the department of pediatrics, Veteran General Hospital, Taipei. Most of the cases (78%) occurred between May and October, the most common pathogenic organism isolated was B salmonella group (65.6%). Antibiotic susceptibility to ampicillin revealed remarkably decrease from 75% (1985) down to 11.7% (1988) and the most sensitive antibiotic agent was ceftriaxone (98.3%). Blood culture were obtained in 42 cases and patients with salmonella bacteremia were found in 10 cases (23.8%). Five of 17 patients under 3 month old showed positive blood culture. Four of 5 bacteremic patients were complicated individually with convulsion, failure to thrive, chronic diarrhea and one case expired. Four of 33 patients between 3 month and 1 year were bacteremic. Two of 4 bacteremic patients were respectively complicated with meningitis and hip-joint arthritis. The average duration of salmonella shedding was 37.5 days (range 4-180 days). IN CONCLUSION: 1. Salmonellosis hasn't decreased in the past 4 years and the resistance to ampicillin and chloramphenicol has increased. 2. Young infants are at particular risk for bacteremia and focal complication, so if salmonellosis is suspected in infants under 3 month old, blood culture should be obtained and proper antibiotic therapy should be given.

[Asphyxiating thoracic dystrophy: a case report].

Asphyxiating thoracic dystrophy (ATD) is a rare case of autosomal recessive disease. We report a case of full term female infant, who was noted to have small chest cage with severe respiratory distress soon after birth. On physical examination. The chest wall was fixed and small with the narrowest circumference about 29 cm in size, general cyanosis and distended abdomen were noted, there was a umbilical hernia around 1 cm in diameter. Otherwise, no other gross anomalies were found. Radiologic studies revealed short and horizontal ribs, small lung volume but depressed diaphragm, the clavicles and the spine were normal. The abdomen and long bone series all showed negative findings. The patient died of respiratory failure at 18 hours after birth. Autopsy was documented as a case of ATD. According to family history, there was one sibling die in the similar condition, although no autopsy available. Because the disease is transmitted as autosomal recessive trait, so 25% of next sibling will get the same condition. Therefore genetic consulting is necessary.

Assessment of ventricular septal defect by echocardiography.

Both 2-D echocardiography and angiocardiography were performed preoperatively in 40 children with isolated ventricular septal defect (VSD). The diagnosis of VSD was confirmed and the size of VSD was measured during operation within on month of the study. There were 27 males and 13 females (age: 10mos-9 yrs). The VSD was classified into perimembranous type (24), supracristal type (14), and AV canal type (2) by surgery. The maximal VSD areas were calculated by 1/4 pi De2 (De = maximal defect diameter measured by 2D echo). The maximal shunt flow areas were measured by color Doppler echocardiography from frame by frame search of the video tape. The maximal shunt flow area was corrected by the body surface area to be the maximal shunt flow area index. A left ventriculogram (LVG) was performed from 4-chamber and left lateral views to measure the maximal diameter of the defect. The maximal defect area of VSD on angiocardiogram was also calculated by 1/4 pi Da2 (Da = maximal defect diameter measured by angiocardiography). The VSD areas measured by different methods were compared by the linear regression analysis. We found that: (1) there is good correlation of the maximal defect diameter and the area of ventricular septal defect by 2-DE and LVG (r = 0.80, p less than 0.0001 v.s. r = 0.78, p less than 0.0001). (2) the ventricular septal defect area by 2-DE had a better correlation with the surgical measurement than that by LVG (r = 0.93, p less than 0.0001, v.s. r = 0.80, p less than 0.0001). (3) the size of ventricular septal defect measured by two-dimensional echocardiography has a better correlation with the MSFAI than the LVG did (r = 0.81, p less than 0.0001, v.s. r = 0.64, p less than 0.0001). Therefore, by measuring the maximal defect diameter, maximal defect area and maximal shunt flow area index, the echocardiography can provide an accurate and non-invasive assessment of isolated VSD in children.

A rapid cold agglutinin test in Mycoplasma pneumoniae infection.

A definite diagnosis of Mycoplasma pneumoniae infection is currently based on cultural method or complement fixation test which is usually retrospective. A rapid cold agglutinin test was developed to determine its value on the early diagnosis of M. Pneumoniae infection. One hundred and thirty patients with pneumonia aged between 5 and 14 years were studied. Blood specimens from all the patients were collected for rapid cold agglutinin test, cold hemagglutination test, and complement fixation test. Thirty patients showed positive, rapid cold agglutinin test. All the patients with positive rapid cold agglutinin test had higher (greater than or equal to 1:32) cold agglutinin titers which were simultaneously performed. The rapid cold agglutinin test had 100% sensitivity and 97% specificity when a cut-off criterion was set at cold agglutinin titer greater than or equal to 1:64. Twenty-five of the 130 cases were serologically proven to have M. pneumoniae infection using complement fixation test or/and cold agglutinin titer. M. pneumoniae was a major cause (21/28) in cold agglutinin-positive pneumonic patients. The positive predictive value of the rapid cold agglutinin test is 70% (21/30). Only 28% (7/25) of the patients with M. pneumoniae infection were diagnosed at acute stage with serological method. We conclude that the rapid cold agglutinin test is of much value in the early detection of M. pneumoniae infection in office or hospital practice in children with pneumonia.

Congenital tuberculosis: a case report.

Congenital tuberculosis is a rare disease, difficult to be correctly diagnosed from clinical manifestations alone without maternal tuberculous history. In this article we report a neonate who exhibited abdominal distension and bloody diarrhea simulating neonatal sepsis and necrotizing enterocolitis. Exploratory laparatomy was performed for the markedly distended abdomen and deteriorated condition. Calcified lymph nodes over mesentery and spleen were found, and biopsy was done to show caseous tubercles. However, this neonate deteriorated rapidly and died one day after operation. Autopsy revealed generalized discrete miliary tubercles over the lungs, liver, spleen, pancreas, adrenal glands, thyroid and lymph nodes.

Low sodium content formula for infants with congestive heart failure.

Twenty-two infants of isolated ventricular septal defect with congestive heart failure were fed with lower-sodium content formula-Lonalac (Mead-Johnson) to study the clinical response of treatment for congestive heart failure. There were no significant changes of intake, urinary output, serum sodium, potassium and osmolality before, 2 days and 6 days after Lonalac feeding. The low sodium content formula may feed the infants with congestive heart failure in addition to the traditional anticongestive therapy.

Non-Hodgkin's lymphoma in childhood--five years survey in VGH-Taipei.

From January 1981 to January 1986, 34 children between 2 and 14 years of age were diagnosed as non-Hodgkin's lymphoma by the Rapport's classification at the Department of Pediatrics of Veterans General Hospital, Taipei, R.O.C. These patients were retrospectively analysed. There was male predominance with a male to female ratio of 2.4:1. The most common symptom was the palpable mass (29 cases, 85.3%) involved the neck and abdomen equally. All the cases were diffuse patterns, DUL (diffuse undifferentiated lymphoma) and DPDL (diffuse poorly differentiated lymphoma) both were the common subtypes of histological patterns. Twenty-eight cases (82%) were in the advanced stage (stage III and IV). The incidence of BM and CNS involvement was 56% and 29.4%, respectively. CNS involvement demonstrated a higher mortality of 100%. Surgery, radiation, and chemotherapy were mainly used for stage I-II and chemotherapy for stage III-IV. The 3-year survival rate of the whole series was 26.5%, and stage I, II, III, and IV was 100%, 50%, 33%, 9%, respectively. The causes of death were CNS involvement (10 cases, 40%), extensive involvement (7 cases, 28.0%), and sepsis (3 cases, 12.0%). This study shows that NHL in children are usually diffuse rather than follicular in histological patterns, often present a palpable mass involved the cervical or abdominal area, and commonly are widespread and tend to disseminate early.

Neonatal lupus erythematosus: report of one case.

A female newborn of SLE mother developed transient typical discoid-like lupus skin lesions over her face soon after birth and had severe relapse with generalized spreading following an episode of upper respiratory tract infection at 50 days of age. Blood picture showed anemia, transient thrombocytopenia and high ESR. Cardiac echo disclosed small ASD with minimal TR. Both EKG and 24 hrs EKG monitor presented normal findings. Serological studies at the early relapse stage of this disease showed increased serum ANA, IgA and IgM level with normal IgG and decrease of C3 and C4. Both Ro(SSA) and La(SSB) antibody systems were positive in mother but only positive for La(SSB) antibody system in this baby. The alpha-anticardiolipin antibody was negative. We suggest that the Ro(SSA) and/or La(SSB) antibody systems may play a role in the pathogenesis of neonatal lupus erythematosus.

Aicardi syndrome--a case report.

The Aicardi syndrome was first described by Aicardi in in 1965, which consisted of infantile spasms, defect of the corpus callosum, a characteristic lacunar chorioretinopathy, mental subnormality, and costovertebral anomalies. All patients have been female except one male case was reported in Australia. There have been approximately over 100 cases of the Aicardi syndrome reported in the literatures since 1965. The etiology of this syndrome is unknown. The most likely cause, however, is an X-linked mutational event with lethality in hemizygous male. We report a 43-day-old female infant with classic features of Aicardi syndrome including flexion spasms, dysgenesis of corpus callosum, microphthalmia, characteristic EEG, and characteristic lacunar chorioretinopathy.

Outcome for very low birth-weight infants.

A total of 69 very low birth weight (VLBW = birth weight less than or equal to 1500gm) infants, born at Veterans General Hospital from January 1, 1984 until December 31, 1986, were studied, excluding 4 patients who were discharged before the condition was stabilized and were also lost to follow-up. The incidence of VLBW infants was 7.5 per thousand of 9214 total live birth deliveries; male to female ratio was 34:39. The hospital mortality rate for babies with birth weight less than 1001gm was 65%; for those with birth weight 1001-1500gm was 30.6%; the total mortality at hospital discharge was 40.6%. The corrected ages at final follow-up assessment ranged from eight months to four years. Among the 41 survivors one case was lost to follow-up. Of the remaining 40 cases, 5 cases had major handicap which was manifested with either severe debilitating morbidity or marked developmental delay; the incidence was 12.5%. One of them died at the postnatal age of 18 months due to ventriculo-peritoneal shunt (V-P shunt) obstruction. Six cases (15.0%) had moderate handicap; the remaining 29 cases (72.5%) were considered to be normal or to suffer from transient, mild, resolvable problems. Cases with mortality or major handicap were categorized into "worse" group, otherwise they belonged to the "better" group. Stepwise multiple discriminant function analysis showed that four factors could correctly classify the outcome in 85.1% of infants: pulmonary hemorrhage was the most important factor followed by birth weight, intraventricular hemorrhage, and maternal antepartum hemorrhage.(ABSTRACT TRUNCATED AT 250 WORDS)