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Background: Bacterial tracheitis may cause life-threatening airway obstruction. Methods: Records of patients admitted to the pediatric wards of Mackay Memorial Hospital between 1994 and 2005 with a diagnosis of bacterial tracheitis made on bronchoscopic visualization of thick membranous tracheal secretions were retrospectively reviewed. Results: A total of 40 patients (aged 1 month–8 years, 29 [73%] under 3 years old) were included. Cough, fever, dyspnea, and hoarseness were the commonest symptoms. Fourteen patients (21%) required intubation. The most frequently isolated bacteriae were α-hemolytic streptococcus (in 11, 38%), pseudomonas (5, 17%), and Staphylococcus aureus (4, 14%). Intubation was more frequent in patients seen between 1994 and 1999 compared with those seen later (8/12 early vs 9/28 late). In the early period α-hemolytic streptococcus (55%) and pseudomonas (36%) were isolated. In the later period the most frequently isolated bacteria was α-hemolytic streptococcus (28%), followed by S. aureus (22%). No patients died, but those with pseudomonas infection had more severe complications, including tracheal stenosis. The average hospital stay in the early period was 26.2 ± 20.5 days versus 9.1 ± 4.8 days in the late period. The corresponding lengths of stay in the intensive care unit were 10.5 ± 11.5 days and 2.0 ± 2.2 days. Conclusions: Bacterial tracheitis requiring hospitalization of children appeared to be milder in the second half of the study period. Pseudomonas tracheitis tends to have a severe course. [ABSTRACT FROM AUTHOR]

Background: Neonatal subgaleal hemorrhage (SGH) is a rare but potentially lethal medical emergency. The objective of the present retrospective study was to clarify the clinical presentation, management, and factors associated with morbidity and mortality. Methods: The records of all newborns from January 1995 to December 2004 were reviewed for evidence of SGH, based on the presence of a fluctuating hemorrhagic mass that crossed suture lines and extended toward the neck. Characteristics were compared between those with a poor or a good outcome. Results: Forty-two newborns with SGH were identified, 77% having had an instrumental delivery. The incidence was 0.6/1000 deliveries and 4.6/1000 vacuum-assisted deliveries. Thirteen patients (31%) had a poor outcome (five died, four had epilepsy, three with severe auditory dysfunction, two with cerebral palsy, and one with renal vein thrombosis). The group with the poor outcome had significantly more patients who had been transferred from other hospitals ( P < 0.001). Those with a poor outcome had significantly more hypotension ( P < 0.001) and seizures ( P < 0.05). Laboratory findings associated with a poor outcome included anemia, coagulopathy, metabolic acidosis, and renal impairment. Other predictors of a poor prognosis included skull bone fracture, the need for pressors, blood transfusion, ventilator support, intensive care unit admission, and a longer hospital stay. Conclusions: SGH may be associated with serious complications and death. The condition must be recognized promptly and monitored closely. Those with poor prognostic factors should be referred for intensive care, treated aggressively, and followed in the long term. [ABSTRACT FROM AUTHOR]

Background: Prader–Willi syndrome (PWS) is a congenital disorder caused by absent expression of paternal genes in 15q11-13 affecting multiple systems. The information concerning the clinical features of this genetic disorder is incomplete in Taiwan. Methods: A retrospective analysis was carried out of 70 PWS patients (39 male, 31 females; age range, 1 month–22 years) seen in four major medical centers in Taiwan from January 1980 through June 2005. All cases were confirmed by methylation-specific polymerase chain reaction. The molecular characteristics, birth history, clinical presentation and laboratory studies were analyzed. Results: Complete genetic analysis was performed in 52 of the 70 patients with PWS. The abnormalities found included deletions in 45 (87%), maternal uniparental disomy (UPD) in five (10%), and a probable imprinting center deletion or an imprinting defect in two (4%). The average weight of the patients at birth was 2588 ± 540 g. Bone age delay of >2 years and growth hormone (GH) deficiency were noted in 11/40 (28%) and 12/20 (60%), respectively. In the 18 in whom both bone age and GH were assessed, abnormalities of both were found in two (11%). In 2000, Taiwan instituted the Rare Diseases and Orphan Drugs Act and mandated a three-phase screening protocol for PWS. Of the 41 patients diagnosed with PWS before 2000, only four (10%) were diagnosed before the age of 3 months; in the 29 patients diagnosed after 2000, in 15 (52%) the syndrome was confirmed before 3 months of age ( P < 0.001). Conclusions: The present finding is in contrast to that of most previous reports that indicated a higher incidence of UPD in PWS. It is unclear whether this discrepancy in the incidence of UPD arises from under-diagnosis or because of ethnic differences, a question worthy of further study. The three-phase screening protocol has generated notable improvement in the early diagnosis of PWS in Taiwan. [ABSTRACT FROM AUTHOR]

Background: This study investigated the survival and natural history of trisomy 13 in a series of patients, comparing the management and outcome before and after the implementation of Taiwan’s National Health Insurance program (NHI). Methods: A total of 28 cases of trisomy 13 seen at Mackay Memorial Hospital, Taipei, Taiwan, from 1985 to 2004 were retrospectively reviewed. Survival and management before (12 cases) and after (16 cases) the implementation of National Health Insurance were compared, and structural defects, imaging findings, and cytogenetic results were analyzed. The cases that were diagnosed prenatally, and finally terminated, were excluded from this study. The diagnosis of trisomy 13 was based on the postnatal chromosome analysis. Results: All patients except one with trisomy 13 translocation died in their first year because of severe malformations of the cardiovascular or central nervous system. The median survival was 9 days. After implementation of National Health Insurance, survival with trisomy 13 was significantly longer than before ( P < 0.05). The three most common structural defects were abnormal auricular helices or low-set ears (89%), cryptorchidism and abnormal scrotum of male (73%) and cleft lip and/or palate (71%). Using echocardiography, the most commonly detected heart defects were patent ductus arteriosus (68%), ventricular septal defect (50%) and atrial septal defect (50%), and eight cases (36%) had complex congenital heart defects. The most common brain lesion was lenticulostriate vasculopathy (22%), followed by holoprosencephaly (17%), brain edema (13%) and subependymal cyst (13%). Conclusions: Early diagnosis and the survival patterns from the data collected should be used to inform parents and health-care professionals to assist in decision making. Although most patients with trisomy 13 die within the first weeks after birth, it is important to recognize that a few may survive the first year. When counseling families, the long-term survival prospects of trisomy 13 patients should be included. [ABSTRACT FROM AUTHOR]

Background: The objective of the present retrospective study was to clarify the clinical course, major pathogens, and other infections associated with infected cephalohematoma.Methods: From January 1978 to December 2003, records of all newborns were reviewed for evidence of an infected cephalohematoma, based either on local signs of infection or a diagnostic tap that yielded pus. Patients were divided into two groups: those seen in the early period (1978–1990) and in the late period (1991–2003).Results: Twenty-eight newborns with infected cephalohematoma were identified, 14 each in the early and late periods. The mean age at onset was 17.8 ± 13.9 days. The most common local findings were erythema (79%), increasing size of the hematoma (68%), and a fluctuant mass (46%), while fever (64%), poor appetite (39%) and irritability (18%) were the common systemic signs. Common laboratory findings included leukocytosis (82%) and an elevated C-reactive protein (61%).Escherichia coliwas the most common pathogen (16 patients, 57%) and was isolated significantly more frequently in the late period (early period: 36%, late period: 79%,P < 0.05).Staphylococcus aureuswas the second common pathogen (five patients, 18%). Ten patients (36%) had other associated infection: eight had sepsis, three had meningitis, and one had osteomyelitis. In the early period three patients (11%) died.Conclusions: Clinicians should be aware that cephalohematoma is a potential site of infection. The incidence of associated system infection is high and may result in mortality. Appropriate diagnostic and therapeutic measures should be undertaken promptly if there are infectious signs. [ABSTRACT FROM AUTHOR]