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We designed this prospective randomized controlled study to evaluate the effects of premature and standard term formula on growth, nutrient intake and biochemical response in premature infants from hospital discharge to 6 months of corrected age. Premature infants with a gestational age of < or =35 weeks and a birth weight < or =1850 gm were assigned to receive premature infant formula (n = 19) or a standard term infant formula (n = 15). No differences were found between the two groups in weight, length, or head circumference at baseline or on follow-up. Infants fed premature formula had higher blood urea nitrogen and phosphorus at 3 months of corrected age. Those on the premature formula also had higher energy intake at 1 month of corrected age. We suggest that premature infants, especially very low birth weight infants, fed preterm infant formula after discharge until 6 months of corrected age tolerate the formula well and may benefit over those standard term formula.

Nosocomial meningitis is uncommon in children. We reviewed the medical records of all children who developed bacterial meningitis at least 72 hours after admission to Mackay Memorial Hospital for the period July 1992 through June 2000. Clinical manifestations, predisposing factors, pathogens, and outcomes were analyzed. Twenty-two cases of nosocomial meningitis were identified, comprising 9.2% (22/239) of all pediatric cases of bacterial meningitis during the study period. The male-to-female ratio was 14:8. All patients were younger than 6 months of age except for one, who was 7 years old. The mean duration between admission and onset of meningitis was 15.3 days (range, 3 to 58 days). Twenty-two organisms were isolated, including 13 Gram-negative bacteria (59%) and 9 Gram-positive bacteria (41%). The most common pathogen was Escherichia coli (5 cases), followed by Enterobacter cloacae (3), Staphylococcus aureus (3), and Chryseobacterium meningosepticum (3). Seventeen patients (77%) had concomitant bacteremia. Predisposing factors for acquisition of nosocomial meningitis included previous treatment with broad-spectrum antibiotics (68%), prematurity with very low birth weight (41%), and total parenteral nutrition (32%). Two patients (9%) had previous neurosurgical intervention. Four patients (18%) died, 3 of whom were low birth weight premature infants. Nine patients (41%) had sequelae, including developmental delay, hydrocephalus, hearing impairment, and epilepsy. Neurosurgery was not a significant risk factor for the development of nosocomial meningitis, while very low birth weight played an important role. Previous intraventricular hemorrhage or hydrocephalus, prematurity with very low birth weight, infection with Gram-negative bacteria, and prior broad-spectrum antibiotic administration were associated with poor outcome.

The purpose of the current study was to examine where arginine vasopressin (AVP) inhibits respiration by direct action on the areas of the ventrolateral medulla (VLM) in the rat. The animal was anesthetized by urethane (1.2 g/kg, i.p.), paralyzed with gallamine triethiodide, and artificially ventilated. Catheterization of the femoral artery and vein, and bilateral vagotomy were performed. The rat was then placed upon a stereotaxic instrument in a prone position. The phrenic nerve was separated and cut peripherally. Phrenic nerve activity (PNA) was monitored at normocapnia and hypercapnia in hyperoxia. Microinjection of AVP into various subregions of the VLM was then performed. In response to AVP microinjection, a transient period of apnea and then a significant decrease in PNA amplitude were observed. Arterial blood pressure was unchanged. This inhibition of PNA with AVP treatment was site-specific, attenuated by raising CO2 concentration, and totally abolished by pretreatment with AVP V1A receptor antagonist. Data of the present study indicate that endogenous resource of AVP may produce an inhibitory effect upon respiration via AVP receptors presented on neurons within the VLM.

Background and Purpose: Despite the development of new adjuvant therapies, meconium aspiration syndrome (MAS) remains a serious respiratory disorder in neonates. Surfactant inactivation by meconium can be overcome by use of exogenous surfactant. This study sought to assess the efficacy and safety of dilute surfactant lavage at 2 different concentrations to treat severe MAS., Methods: We retrospectively reviewed the charts of all term infants with a diagnosis of MAS who had an oxygenation index (OI) > 20 during a 2-year period. Tracheobronchial lavage was performed with a dilute surfactant suspension (5 mg/mL or 10 mg/mL) to reach a total dose of 60 to 70 mg/kg of phospholipid, administered in aliquots of 2 mL., Results: The records of 22 patients were reviewed, of whom 12 had undergone lavage. These patients were subdivided into low-concentration (surfactant concentration, 5 mg/mL; n = 6) and high-concentration (surfactant concentration, 10 mg/mL; n = 6) subgroups. There were no significant differences in demographic characteristics between these 2 subgroups. The lavaged infants had a significantly higher arterial partial pressure of oxygen (PaO(2)) 24 hours after lavage than the infants without lavage (178.3 mm Hg vs 80.6 mm Hg, p < 0.05). The incidence of pneumothorax (1/12 vs 7/10, p < 0.05) and requirement for inhaled nitric oxide (5/12 vs 9/10, p < 0.05) were significantly lower in the lavaged group. All infants tolerated the procedure well except for 2 with transient complications. There were no significant differences in duration of lavage, response and complications between subgroups lavaged at low and high surfactant concentration., Conclusions: Early lavage with dilute surfactant solution at a phospholipid concentration of either 5 mg/mL or 10 mg/mL is effective for the treatment of severe MAS. Further large-scale, prospective, randomized, controlled trials are necessary to establish the optimal dose, concentration, surfactant product, and instillation method of this treatment before it can be recommended for routine use.

Infantile subglottic hemangioma is a rare, life threatening disease of the larynx. Various treatments have been described in the literature, including tracheostomy, systemic steroids, intralesional steroid injection, carbon dioxide laser therapy, and interferon alfa-2a. The best treatment, however, remains controversial. We report a case of congenital subglottic hemangioma successfully managed with a single intralesional steroid injection, which resulted in rapid involution of the hemangioma without recurrence after 18 months of follow up. This therapy appears to minimize both short and long term morbidity associated with treatment of this lesion.

Unlabelled: The purpose of this study was to analyse the clinical differences between omphalocele and gastroschisis in Taiwan, with special reference to associated anomalies and outcomes. A retrospective review was conducted of 115 cases seen between January 1990 and June 2000 at two tertiary medical centres in Taiwan. Data included perinatal events and associated anomalies. Of 115 patients, 65 were classified as having gastroschisis and 50 as having omphalocele. Other anomalies were found in 24 omphalocele cases, compared with 23 gastroschisis cases. The range of anomalies associated with omphalocele varied more widely than in the gastroschisis cohort. Of patients with omphalocele and associated anomalies, six had chromosomal abnormalities compared with none of the patients with gastroschisis. In patients with gastroschisis and additional malformations, 17 had gastrointestinal anomalies, the most common of which was intestinal malrotation. A comparison of perinatal data revealed that infants with gastroschisis were more likely to be small for gestational age. Gastroschisis was associated with a younger overall maternal age than omphalocele and a lower birth weight. There was a male predominance among omphalocele patients, but this did not reach statistical significance., Conclusion: Gastroschisis was frequently associated with intestinal anomalies and transient dysfunction, and outcomes were related to postoperative complications. Infants with gastroschisis more frequently required prolonged parenteral nutrition supplement, resulting in longer hospital stay. Omphalocele was often accompanied by chromosomal disorders leading to early neonatal death, so we recommend that amniocentesis should be indicated if omphalocele is suspected on fetal ultrasonography.

Intrauterine infections with varicella-zoster virus following maternal varicella in early pregnancy and resulting in congenital malformations are rare. Herein we report a child with congenital varicella syndrome characterized by low birth weight, cicatricial scarring, hypoplasia of both lower extremities with joint contracture, congenital hip dislocation, corneal opacity, atresia of the sigmoid colon and a rarely associated cloaca anomaly. The varicella IgG remained positive after she was seven months old. Her mother developed chickenpox at the 14th week of gestation. The purpose of this article is to raise pediatricians' index of suspicion for congenital varicella syndrome when an infant is born with multiple congenital malformations with an apparent history of maternal varicella infection.

Objectives: To compare the accuracy of standard and hemocytometer white blood cell (WBC) counts and urinalyses for predicting urinary tract infection (UTI) in febrile infants., Methods: Enrolled were 230 febrile infants < 12 months of age. All urine specimens were obtained by suprapubic bladder aspiration and microscopically analyzed by the standard urinalysis (UA) and by hemocytometer WBC counts simultaneously, and quantitative urine cultures were performed. Receiver-operating characteristic (ROC) curves were constructed for each method of UA. The optimal cutoff point of the UA test in predicting UTI was determined by ROC analysis., Results: There were 37 positive urine cultures of at least 1,000 CFU/ml. Of these 37 patients, 9 females and 28 males, 1 had a positive blood culture (Escherichia coli). Thirty (81%) of the positive urine cultures had a bacterial colony count > or = 100,000 colony-forming units/ml, whereas the remaining had between 1,000 and 50,000 colony-forming units/ml. The area under the ROC curve for standard UA was 0.790 +/- 0.053, compared with 0.900 +/- 0.039 for hemocytometer WBC counts (P < 0.05). For hemocytometer WBC counts, the presence of < or =10 WBC/microl appeared to be the most useful cutoff point, yielding a high sensitivity (83.8%) and specificity (89.6%). Standard UA, with a cutoff point of 5 WBC/high power field, had a lower sensitivity (64.9%) and similar specificity (88.1%). The hemocytometer WBC counts showed significantly greater sensitivity and positive predictive value (83.8 and 60.8%, respectively) than the standard urinalysis (64.9 and 51.1%, respectively) (P < 0.05). The accuracy, specificity and likelihood ratio of hemocytometer WBC counts were also greater than that of standard UA (88.7, 89.6 and 8.08% vs. 84.3, 88.1 and 5.44%)., Conclusion: Hemocytometer WBC counts provide more valid and precise prediction of UTI in febrile infants than standard UA. The presence of > or =10 WBC/microl in suprapubic aspiration specimens is the optimum cutoff value for identifying febrile infants for whom urine culture is warranted.

Objective: To assess the usefulness of laboratory parameters, including peripheral white blood cell (WBC) count, C-reactive protein (CRP) concentration, erythrocyte sedimentation rate (ESR), and microscopic urinalysis (UA), for identifying febrile infants younger than 8 weeks of age at risk for urinary tract infection (UTI), and comparison of standard UA and hemocytometer WBC counts for predicting the presence of UTI., Methods: A total of 162 febrile children <8 weeks of age were enrolled in this prospective study. All underwent clinical evaluation and laboratory investigation, including WBC count and differential; ESR; CRP; blood culture; a lumbar puncture for cell count and differential, glucose level, protein level, Gram stain, and culture; and a UA and urine culture. All urine specimens were obtained by suprapubic aspiration and microscopically analyzed with standard UA as well as with hemocytometer WBC counts. Quantitative urine cultures were performed. Sensitivity, specificity, accuracy, likelihood ratios, and receiver operating characteristic (ROC) curves were determined for each of the screening tests., Results: There were 22 positive urine culture results of at least 100 colony-forming unit/mL. Eighteen of these 22 patients were males, and all were uncircumcised. There were significant differences for pyuria >/=5 WBCs/hpf, pyuria >/=10 WBC/microL, CRP >20 mg/L, and ESR >30 mm/hour between culture-positive and culture-negative groups (P <.05). The ROC area for hemocytometer WBC count, standard UA, peripheral WBC count, ESR, and CRP concentration were.909 +/-.045,.791 +/-.065,.544 +/-.074,. 787 +/-.060, and.822 +/-.036, respectively. The ROC curve analysis indicates that the CRP, ESR, and standard UA were powerful but imperfect tools with which to discriminate for UTI in potentially infected neonates. Hemocytometer WBC counts had the highest sensitivity, specificity, accuracy, and likelihood ratios for identifying very young infants with positive urine culture results. For all assessments, hemocytometer WBC counts were significantly different, compared with the standard urinalysis. ESR, CRP, and peripheral WBC counts were not helpful in identifying UTI in febrile infants., Conclusion: UTI had a prevalence of 13.6% in febrile infants <8 weeks of age. The CRP, ESR, and standard UA were imperfect tools in discriminating for UTI, and the sensitivity of these laboratory parameters was relatively low. Hemocytometer WBC count was a significantly better predictor of UTI in febrile infants.

Background: Vallecular cyst is fairly uncommon in neonates and infants. Although benign in nature, it may cause severe airway obstruction and even death. This study retrospectively analyzed the clinical manifestations of vallecular cyst in neonates and discussed its management., Methods: From June 1993 through January 1997, 11 cases of vallecular cyst were collected and reviewed retrospectively. There were 8 male infants and 3 female infants. Their clinical manifestations, age at the onset of symptoms, age at diagnosis, and surgical management were analyzed. Fibrolaryngoscopy was used for preoperative diagnosis and postoperative follow-up., Results: The infants' initial presentations were inspiratory stridor, respiratory distress, noisy breathing, feeding difficulty, and failure to thrive. There was a high incidence of patients with coexisting signs of laryngomalacia (10/11). Ten patients received laryngomicrosurgery with CO2 laser for deroofing the cyst. Additional supraglottoplasty was performed at the same time in 4 patients with laryngomalacia type A + C and in one patient with severe laryngomalacia type C. Their symptoms all resolved soon after surgery. The phenomenon of laryngomalacia also disappeared. There has been no recurrence up to the present., Conclusion: Although fairly uncommon, vallecular cyst should be included in the differential diagnosis of congenital laryngeal stridor in neonates. The use of fibroendoscopy can promptly diagnose vallecular cyst and any synchronous airway lesions. Although most of the synchronous laryngomalacia (type C) in this study was secondary to vallecular cyst, we suggest that supraglottoplasty be taken into consideration during cyst deroofing when the signs and symptoms of laryngomalacia type A are also present.

This study was undertaken to determine postnatal changes in thyroid function in very low-birth-weight (VLBW) infants. A total of one-hundred VLBW infants participated. Serial examination of serum levels of thyroxine (T4), free T4, triiodothyronine (T3), and thyroid-stimulating hormone (TSH) was performed in the neonatal period. A total of eighty-nine infants survived to discharge, while eleven died during hospitalization. Transient hypothyroxinemia was found in forty-one (46.1 percent) of the survivors. One of the surviving infants had primary hypothyroidism. His data was excluded from the analysis. In the other eighty-eight surviving infants, TSH levels were within normal limits throughout the six-week study period. T4 and free T4 values decreased after the first day of life, reaching a nadir at one week of age, followed by progressive increases. The mean cord blood T3 level was very low; the serum T3 value increased progressively in the postnatal period. We found a correlation between low T4 and free T4 values and mortality and neonatal illness. Hypothyroxinemia was associated with critical illness. In conclusion, the postnatal changes in thyroid function in VLBW infants were characteristic, with transient hypothyroxinemia being common in these infants. Further investigation of the relationship between thyroid function, death, neonatal illness, and developmental outcome is warranted.

Prolonged hospitalization in infants suffering from chronic lung disease who require continuous oxygen therapy can be avoided by oxygen administration at home. In the period from August 1995 to September 1996, 155 very low-birth-weight (VLBW) infants were consecutively admitted to the neonatal intensive care unit of Mackay Memorial Hospital. Of the 155 infants, 72% (111/155) survived to discharge. However, 34% (38/111) of the survivors developed chronic lung disease. Twenty-three infants with chronic lung disease underwent home oxygen therapy after 107.0 +/- 43.6 days of hospitalization. The mean duration of home oxygen therapy was 4.3 +/- 3.4 months. In the first year after discharge, 91% of the patients required rehospitalization. One patient died during the fourth hospitalization. Follow-up information on growth and development at one year of corrected age was available for 19 patients. Five of the 19 patients had a body weight below the 5th percentile. Five of the 19 infants were mentally retarded and 12 of the 19 patients had significantly delayed motor development. In conclusion, carefully supervised home oxygen therapy permits safe early discharge of selected VLBW infants with chronic lung disease. Their somatic and psychomotor development should be carefully followed up.

Atrial flutter is a rare arrhythmia in the neonate and early infancy. We retrospectively reviewed the clinical presentations, treatment and outcome of seven patients who presented clinically with atrial flutter. The age of onset ranged from 1 day to 3 months. Atrial flutter was diagnosed in the first 3 days of life in 4. Three cases presented as atrial flutter with 2:1 atrioventricular conduction and the remaining 4 with variable AV block. Heart failure was present in 3 patients and 6 patients showed normal intracardiac structure on echocardiography. Electrical cardioversion was attempted as the first treatment in 4 cases, followed by digoxin in three of the four. Digoxin was given as an initial therapy in 2 patients. One patient recovered spontaneously without treatment. In the 6 patients who received therapy, 5 converted to normal sinus rhythm within 2 days. The remaining patient had ventricular ectopic beats for about 4 months. Only 2 cases were maintained on oral digoxin for at least 4 months after discharge. No patient had a recurrence of atrial flutter during the follow-up period which ranged from 6 months to 7 years. We conclude that there is a good long-term prognosis for atrial flutter in the neonate. Digoxin and DC cardioversion may be effective as initial therapy. Long-term digoxin prophylaxis after conversion to sinus rhythm may be not necessary.

Intussusception found in the 1st month of life is rare and usually discussed as one entity, neonatal intussusception, but in fact, includes the intussusceptions occurring both prenatally and neonatally, of which the clinical presentations and results are different. Four full-term babies with prenatal intussusception presenting as intestinal atresia (IA) and three premature babies with neonatal intussusception mimicking necrotizing enterocolitis (NEC) are presented. Prenatal intussusception, as one of the causes of IA produces prominent signs of intestinal obstruction immediately after birth. Preoperative evaluation usually fails to yield a definitive diagnosis, but surgery is usually performed in time and is successful. In neonatal intussusception, full-term infants usually have a pathological lead point and the colon is almost always involved. A barium enema is thus useful in diagnosis. Premature babies, on the other hand, rarely have a colonic component, and the clinical features are insidious and similar to NEC. This results in diagnostic confusion that may lead to a dangerous delay in appropriate surgical correction. A high level of suspicion about this condition in cases diagnosed presumptively with NEC is important. Serial abdominal sonograms may be helpful in the early diagnosis of neonatal intussusception.

Aside from congenital heart disease, anomalies associated with unilateral hypoplasia of the depressor anguli oris muscle have not been well-documented in large series. We evaluated the associated anomalies in 50 infants or children with this disorder (male:female = 2:1) and found accompanying anomalies in 35 (70%) of 50 cases. They included anomalies of the head and neck (48%), heart (44%), skeleton (22%), genitourinary tract (24%), central nervous system (10%), gastrointestinal tract (6%), and miscellaneous minor anomalies (8%). Nearly half of our cases (22/50) had at least 2 associated systemic anomalies. Failure to thrive and psychomotor retardation were found in 5 (10%) and 3 (6%) patients, respectively, on follow-up. Three infants died neonatally of severe heart disorders, and the other one died of central nervous system anomalies. The above findings indicate that a thorough search for associated anomalies, particularly in the cardiovascular system, should be performed in all newborns with asymmetric crying face.

The primary objective of this study was to evaluate the safety and benefit of early enteral feeding in very-low-birth-weight (VLBW) infants without parenteral nutrition. Weight gain, feeding intolerance, nosocomial infection rate and a postnatal growth curve were recorded for 61 VLBW premature infants who were admitted to the Neonatal Intensive Care Unit of Mackay Memorial Hospital from September 1, 1995 to February 28, 1997. Nine infants were unable to complete the study and three were excluded because of severe bronchopulmonary dysplasia; therefore only 49 infants could be evaluated. They were divided into two groups based on birth weight: 1001 gm to 1250 gm (Group A, mean birth weight 1153 +/- 64 gm, mean gestational age 29.0 +/- 2.2 weeks), and less than or equal to 1000 gm (Group B, mean birth weight 911 +/- 82 gm, mean gestational age 27.1 +/- 1.5 weeks). They received breast milk or premature formula by intermittent nasogastric or continuous nasogastric feeding. Growth was followed over the first 30 postnatal days. Group A reached 100 kcal/kg/day of enteral feeding at a mean age of 17 days as compared with a mean age of 20 days for group B. Infants regained their birth weight at 20 and 25 days in Groups A and B, respectively. By the 30th postnatal day, weight gain exceeded birth weight by 218.2 +/- 143.1 gm and 95.3 +/- 81.5 gm in groups A and B respectively. No definite episodes of necrotizing enterocolitis (NEC) developed. Two cases of Escherichia coli sepsis and one of Klebsiella sepsis occurred. The conclusion was that early enteral feeding in very-low-birth-weight infants does not increase the risk of NEC. It was also demonstrated that enteral feeding alone can produce biphasic postnatal growth curves in very-low-birth-weight infants. Although early enteral feeding was well tolerated in the study infants, the occurrence of feeding intolerance in some (36%) would suggest that additional parenteral nutrition may benefit some infants until full enteral feeding can be achieved.

We present a premature newborn with congenital rickets, born to a mother with untreated chronic renal insufficiency. X-ray films showed blurred metaphyseal ends and decreased bone density in the femurs and ribs. With treatment including calcium, phosphate, and vitamin D, her rickets healed and she grew normally.

A 34-year-old woman with an abnormal maternal serum screening result and a Down syndrome risk of 1:60 calculated from a maternal serum alpha-fetoprotein (AFP) value of 1.4 multiples of the median (MoM) and a human chorionic gonadotrophin (hCG) level of 4.32 MoM at 18 weeks' gestation was found to have isolated fetal ascites at 23 weeks' gestation. Spontaneous resolution occurred 10 weeks after the initial presentation. After birth, the neonate had generalized cutis marmorata telangiectatica congenita (CMTC), large vascular plaques on the scalp with superficial ulceration and crusts, a small atrial septal defect, a patent ductus arteriosus, hepatomegaly, micrognathia, seizures, an abnormal electroencephalogram, congenital retinal detachment, glaucoma and widely spaced toes. Our patient illustrates that CMTC in utero may be associated with a markedly elevated maternal serum hCG level as well as transitory isolated fetal ascites. However, such associations can be coincidental and further collaborative studies and cases will be necessary before it can be determined that a disproportionately elevated hCG level and transitory isolated fetal ascites are predictive of CMTC in utero.

A term male neonate had an uncommon congenital vascular disorder of the skin covering the whole body and extremities. These skin lesions were characterized by mottling and persistent telangiectasia. A skin biopsy showed nevus vascularis reticularis which was recognized as cutis marmorata telangiectatica congenita. In addition to the cutaneous involvement, the patient also had fetal ascites, pleural effusion, patent ductus arteriosus, glaucoma, retinal detachment in the left eye, and telangiectasia in the right eye. Neonatal focal seizure developed and expired on the 34th day of life. The cerebral image studies suggested vascular anomalies with secondary cerebral parenchymal damage. Cutis marmorata telangiectatica congenita may be solitary but is frequently associated with other developmental defects. However, the specific findings of cerebral and ophthalmic lesions in our case were rather unusual.

Unlabelled: Pancreatitis in children is not common and can be associated with severe morbidity and mortality. We encountered 43 children, ranging in age from 2 to 18 years, with pancreatitis over the past 10 years. The diagnosis of pancreatitis was made in those patients who showed: (1) significant intra-operative pathology or; (2) clinical findings of pancreatic inflammation and laboratory confirmation. More than one third (16 cases) of the cases were due to trauma, other causes included systemic disease (10), structural disease (8), and toxins or drugs (4). Five cases were classified as idiopathic. Most of the patients presented with abdominal pain (95%) and vomiting (56%). Jaundice was found in 7 patients and an abdominal mass in 2. Morbidity included pseudocyst (10), relapse (4), hyperglycaemia (4) and miscellaneous problems. Eight (50%) of the patients with trauma and 6 (86%) of the patients with structural diseases required surgery. Other patients were managed conservatively with bowel rest, gastric decompression, intravenous fluid and total parenteral nutrition. One case had a fatal outcome. All the survivors did well in long term follow up. Relevant literature has been reviewed and the sensitivity of various diagnostic modalities compared and discussed. A lesser known association between pancreatitis and structural anomalies such as choledochal cyst is discussed. To our knowledge, the present review is the first on pancreatitis in Chinese children., Conclusion: Pancreatitis can occur from a wide variety of causes and may result in severe complications. Early diagnosis, close monitoring and aggressive intervention are mandatory to reduce morbidity and mortality.

Forty-six patients with two or more features of the VATER association admitted to the Mackay Memorial Hospital from May, 1983 to Mar, 1992 were retrospectively enrolled in this study. We compared the incidence of major features with that reported in the literature. Imperforate anus, congenital heart disease, and renal anomalies were the three most common major features in our study. Thirteen patients died. Heart failure was the major cause of mortality. We noted many patients associated minor features, and among them there was a relatively high ratio of cleft lip, cleft palate, and hypospadius. The overall outcome and development were good among the survivals. We suggest that children who have any congenital anomaly included in the VATER association should get a careful examination and evaluation of their heart, genitourinary tract, limbs and vertebrae. Early correction if possible is indicated in such patients.

Ninety-five infants, less than 2 months of age, diagnosed as urinary tract infections, from July 1984 to June 1991, were reviewed. Their urinary cultures, obtained either by suprapubic puncture or via catheterization, all had bacterial colony counts of over 10(5)/ml. In this survey, males predominated (91.6%). Fever and gastrointestinal problems were the two most prevalent signs. E. coli was the most common causative organism, and gentamicin was the most effective antibiotic. Vesicoureteral reflux (VUR), the most common anomaly, was found in one-third (25/76) of patients on voiding cystourethrography, with 20% being high grade (Gr. IV or Gr. V). Eleven cases (11%) had bacteremia, and one case had bacterial meningitis. Sixty-seven cases were followed up in our hospital and seven of them had second infections within a year of their first UTI. The mean period between episodes was less than two months. All these patients had urinary tract anomalies and received oral chemoprophylactic drugs for variable lengths of time. Five of the seven recurrences were caused by resistant bacilli. Continuous oral antibiotic prophylaxis and regular follow-up examinations were the rules of prevention for further infection and future renal impairment. These preventive methods are especially important in young infants with UTI.

From July 1987 to October 1991, we experienced 10 full-term newborn infants with severe adult respiratory distress syndrome (ARDS). The triggering events were intrauterine/perinatal asphyxia in 6 and sepsis in 4. All had severe respiratory distress/failure and were mechanically ventilated with high concentration of inspired oxygen and positive end-expiratory pressure. Radiography of the chest all showed bilateral dense consolidation (white out lungs) and reduced lung volume. Persistent pulmonary hypertension (PPHN) was documented in 9 cases (90%). The concomittent occurrence of ARDS and PPHN rendered respiratory management extremely difficult. High-rate ventilation and tolazoline infusion were used in all these 9 PPHN cases. Acute complication of respiratory therapy (pneumothorax) was encountered in 5 patients. Only 3 cases survived, all belonging to the asphyxia group. Of these 3 survivors, 1 developed bronchopulmonary dysplasia, 1 had cerebral palsy on follow up and the other one was lost on follow up. The outcome of neonatal ARDS was generally poor.

Enterovirus infection has been recognized as one of the most common viral infections in the perinatal and neonatal periods. It frequently leads to significant mortality. One fatal case of neonatal enteroviral infection was experienced in last year. The patient was a one-day-old male, presenting with neonatal sepsis. He has a biphasic illness, first with a mild febrile prodrome then followed by severe systemic involvement, with meningitis, myocarditis, hepatosplenomegaly and disseminated intravascular coagulation. All bacterial cultures were negative, but the rectal swab isolated enterovirus. The echocardiogram revealed depressed cardiac function, and he finally expired at the age of 10 days. The autopsy findings supported the diagnosis of perinatal enteroviral infection (coxsackievirus B infection was highly suspected). Clinically, if a neonate presents as sepsis, but has the following conditions, enteroviral infection should be considered: (1) negative bacterial cultures; (2) multiple organ involvement; (3) proven enteroviral infection in the same nursery or ward; (4) a mild febrile illness in the mother within the last antepartum 10 days or the first postpartum 5 days; (5) any family members with fever or signs of upper respiratory infection within 15 days before delivery.

Aspergillus sp. are the most common contaminants of the paranasal sinuses with the maxillary sinus as the site most frequently involved. Fungal sphenoid sinusitis can be a life-threatening situation, thus aggressive therapy is indicated. The treatment is primarily surgical. The transnasal endoscopic technique offers excellent visualization and an atraumatic approach to the sphenoid sinus. Surgical exteriorization and aeration of the involved sinus, without using antifungal chemotherapeutic agents is curative. A rare case of sphenoid sinus aspergillosis which was successfully treated by transnasal endoscopic sphenoidotomy is reported.

In order to delineate the efficacy of plasmin-treated intravenous gamma-globulin (IVGG) in the treatment of Kawasaki syndrome, we compared the frequency of coronary artery abnormalities in children treated or not with IVGG for Kawasaki syndrome. Among 291 cases of Kawasaki syndrome diagnosed during the period of 1987 to 1991 without coronary abnormalities within 10 days of the onset of illness, 128 were treated with IVGG and aspirin and were compared with 163 treated with aspirin alone. IVGG was given in a dosage of 400 mg/kg/day for 4 consecutive days. The detection of coronary abnormalities was monitored by two dimensional echocardiography. Two weeks after enrollment coronary artery abnormalities were present in 37 (22.7%) of 163 children in the aspirin group and in 9 (9%) of 128 in the gamma-globulin group (P < 0.05). Seven weeks after enrollment, abnormalities were present in 20 (12.3%) of 163 children in the aspirin group and in 6 (4.6%) of 128 in the IVGG group (P < 0.05). We conclude that plasmin-treated IVGG is effective in reducing the prevalence of coronary artery abnormalities in Kawasaki syndrome and suggest a predominant role of the Fc gamma fragment of IgG in the therapeutic effect.

Transient asymmetric hypertrophy of the interventricular septum was found by echocardiography in a 20-day-old infant whose mother was not diabetic. The electrocardiogram showed first-degree atrioventricular block and QT prolongation. Electrocardiograms on all first degree relatives were within normal limits. With the medication of propranolol, the infant did well. Both echocardiographic and electrocardiographic findings returned to normal two months later (Acta Paed Sin 1993; 34:38-44).

A seven-month-old girl was admitted to the Pediatrics Department of Mackay Memorial Hospital with the following symptoms and signs: (1) high fever for more than five days; (2) injection of bilateral conjunctiva; (3) bright red lips with strawberry tongue; (4) edematous change of palms and soles, followed by digit desquamation; (5) an ill-defined, erythematous plaque on the scar of the BCG. Kawasaki disease was diagnosed, and high dose aspirin (100 mg/kg/day) and intravenous gamma-globulin (IVIG) (400 mg/kg/day) were given for four days. The patient was afebrile on the second day after IVIG infusion, and was discharged six days after admission. A small single daily dose of aspirin (10 mg/kg/day) was given after the afebrile days. Unfortunately, vomiting and consciousness disturbance were noted one day after discharge. Laboratory data showed elevated aspartate aminotransferase (AST), alanine aminotransferase (ALT) and ammonia. Hypoglycemia and prolonged PT and PTT were also noted. Reye syndrome was suspected, and the patient was admitted to the intensive care unit for further management. A liver biopsy gave findings consistent with Reye syndrome. In spite of intensive treatment, the infant expired on the second day after admission. In a review of the literature, no correlation between these two syndromes was found. This rare case is presented to warn that Reye syndrome may follow Kawasaki disease when aspirin has been prescribed at a high dose.

We studied 2561 cases of otolaryngologic emergencies in the Far Eastern Memorial Hospital from January 1, 1985, to December 31, 1989, looking for the distribution and epidemiology of these emergency diseases. Our conclusions were as follows: 1. Total emergency otolaryngologic cases [otologic cases and pharyngolaryngologic cases] demonstrated a tendency to increase from year to year. 2. Acute otitis media and foreign bodies of the nose also showed an increase from year to year. 3. The "top five" emergency otolaryngologic diseases in order of frequency were: epistaxis (22.7%), foreign bodies of the throat (18.7%), acute tonsillitis (11.4%), acute otitis media (7.7%), and foreign bodies of the ear (6.4%). 4. Patients admitted to the emergency department (7.2%) ranked in order of frequency were: epistaxis (29%), acute tonsillitis (24.5%) and esophageal foreign bodies (14.1%). 5. Emergency visits occurred most frequently between 20-24 o'clock (26.5%) daily; the least from 4-8 o'clock (9.3%).

A retrospective study was undertaken of 175 patients (119 males, 56 females) admitted to the neonatal intensive care unit of Mackay Memorial Hospital during the period of July 1, 1985 to June 30, 1986 who received mechanical ventilation during their stay at the hospital. Upon reviewing the clinical histories of these patients, the complication rate of mechanical ventilation was 31.9%. The percentages of each complication were: pneumothorax 50.0%, pneumomediastinum 5.2%, pulmonary interstitial emphysema 1.7%, atelectasia 13.8%, pneumonia 13.8%, chronic lung disease 13.8%, nasopharyngeal infection 1.7%. Survival rate of these ventilated patients with or without complication was not significant statistically (69.2% vs 65.6%). However, with regard to the hospital course, cases with complication had a significantly longer duration of ventilator usage, hospital stay and oxygen usage than uncomplicated cases. In conclusion, experienced personnel are needed to supervise the use of mechanical ventilation in neonates, and a team of well-trained nurses working in the neonatal intensive care unit are essential to minimize complications.

Congenital cutaneous candidiasis is a very rare disease. We reported two newborn infants in whom generalized skin eruption was noted at birth, characteristics of erythematous papules and pustules. The eruption involved head, face, neck, trunk and extremities. Candida albicans was demonstrated on direct KOH smear, by surface fungal cultures and skin biopsy. The disease implies a congenital intrauterine infection and is different from neonatal candidiasis which manifests as thrush, diaper dermatitis. The route of infection is ascending in congenital cutaneous candidiasis. The skin eruption is usually noted at birth or within 12 hours after delivery as a diffuse erythematous maculopapula, with pustules or vesicles distributed over head, face, neck, trunk and extremities. There is no fever; other constitutional signs are lacking. No evidence of impaired immunological responsiveness has been noted in previous study. Clinical features, direct smear examination of specimen and appropriate cultures are useful in differentiating the lesions from other more common dermatoses of the neonatal period. Topical antifungal therapy is sufficient unless systemic candidiasis is present. Prognosis for congenital cutaneous candidiasis is good.

From January 1986 to December 1988, 5 cases of oligohydramnios with pulmonary hypoplasia were experienced. Clinically, all of the newborn infants had unique faces and evidence of respiratory distress with tachypnea, cyanosis, intercostal retractions, nasal flaring and grunting respiration presented within minutes after birth. Usually, they had no response to respiratory therapy. Most of them showed small lung volume by chest roentgenogram and were prone to develop pneumothorax. All 5 cases did not survive more than a few hours. Autopsy findings disclosed pulmonary hypoplasia. Four of them had urinary tract malformation, and the other one had the history of prolonged leakage of amniotic fluid.

Triazolopyrimidine sulfanilides are a class of highly active herbicides whose primary target is acetolactate synthase. Spontaneous mutants of tobacco (Nicotiana tabacum) (KS-43) and cotton (Gossypium hirsutum) (PS-3 and DO-2) resistant to triazolopyrimidine sulfonanilide were selected in tissue culture. Acetolactate synthase partially purified from the three mutants were 80- to 1000-fold less sensitive to inhibition by the compound compared with the corresponding wild-type enzyme. The mutants also varied in the cross-resistance pattern to other acetolactate synthase inhibiting herbicides in the sulfonylurea, imidazolinone, and pyrimidyl-oxy-benzoate chemical families. Thus, acetolactate synthase from KS-43, PS-3, and DO-2 cultures have different mutations. The affinities for pyruvate, thiamine pyrophosphate, as well as the activity of the mutant enzymes were found to be comparable to the corresponding wild-type enzymes. However, the enzyme from PS-3 was highly resistant to feedback inhibition by valine and leucine. In contrast, acetolactate synthase from KS-43 and DO-2 were inhibited by valine and leucine to nearly the same extent as the wild-type enzymes. Also, PS-3 cultures accumulated much higher levels of the branched chain amino acids compared to the wild-type cotton culture. The mutation in the PS-3 enzyme has therefore rendered it insensitive to feedback regulation by valine and leucine.

A 4-year-old boy developed nephrotic syndrome following varicella infection. Serologic studies during the early phase of the disease demonstrated a decrease in serum C3, C4, and properdin factor B. Renal biopsy revealed an acute proliferative glomerulonephritis with deposition of immunoglobulins A (IgA) and M, C3, C1q, and varicella virus antigen in the glomerulus, suggesting an immune complex deposition. Ultrastructurally, this suggested a postinfectious immune complex glomerulonephritis. These phenomena suggested that varicella virus antigen antibody complexes were deposited in the glomerulus and activated the classic and alternative pathway of complements, leading to an immune complex glomerulonephritis. During the nephrotic phase, an increase in OKT8 cells and decrease of the OKT4 cells were demonstrated. Two months later, this alteration returned to normal as the renal disease was in remission. This change of lymphocyte subsets during varicella infection may play a role in the pathogenesis of nephrotic syndrome.

Three cases of duplications of the alimentary tract are presented. Case 1 was a 5-month-old male baby. He was admitted due to copiously painless rectal bleeding. Tc-99m pertechnetate scanning revealed unusual tubular and spherical lesions. Laparotomy findings were that a tubular ileal duplication m assured 100cm or so in length and communicated with ileal lumen by its distal orifice. Moreover, there were 2 spherical duplications like ping-pong ball in morphology located in neighboring mesentery. Case 2 was a 2-day-old male newborn. He was admitted because of persistent bilious vomiting on his second day of life. Plain film x-ray revealed dilated stomach and scanty bowel gas. Laparotomy finding was that a cystic duplication measured 1.5cm in length which located in and obstructed the lumen of proximal jejunum. Also there was a distal orifice of duplication in communication with jejunal lumen. Case 3 was a 4-year-and-4-month-old boy who admitted after intermittently non-projectile vomiting for a period of 1 1/2 year. Sonography showed a calcification lesion at right upper quadrant of abdomen. Panendoscopy saw a refraction at second part of duodenum. The findings of laparotomy were that 2 tumor masses laid upon gastrocolic ligament. One was ossified soft tissue (gastrocolic ligament) with 1.5 x 1 x 0.8cm3 in size, and the other was cystic duplication of transverse colon in contact with beneath mesocolon. All duplications of 3 cases were lined with ectopic gastric and small intestinal mucosa, and ulcerative lesion was found in case 3 only. All lesions were resected and removed with good results.

Six of 83 asphyxiated neonates showed a diffuse increase of echodensity in bilateral thalami with or without other lesions in the basal ganglia on sonographic examination. The thalamic image still had a fairly high echogenecity compared with the surrounding brain parenchyma on follow up examination and was hence termed the bright thalamus. These six patients had a poor neurological outcome, including psychomotor retardation, spastic diplegia or quadriplegia, microcephaly, failure to thrive, seizures, and one death. The bright thalamus is not an occasional or an isolated sonographic finding of hypoxic-ischaemic encephalopathy, but it may serve as a landmark of severe hypoxic brain damage with adverse outcome.