Hsiao-Ping Wang

Multiple Clinical Manifestations and Diagnostic Challenges of Incontinentia Pigmenti—12 Years' Experience in 1 Medical Center

Background: Incontinentia pigmenti (IP) is a rare X‐linked dominant disorder that involves ectodermal tissues of multiple systems. Previous reports are few in Taiwan. To contribute toward better understanding of IP, we describe and discuss the clinical features of cases that were diagnosed in a medical center during the past 12 years. Methods: The medical records of all patients with IP between July 1995 and June 2007 were reviewed retrospectively. The demographics, physical findings, pathology reports, molecular study reports, eosinophil counts and outcome were recorded. Results: A total of 4 patients, 3 female and 1 male neonate, who met the criteria for the diagnosis of IP were enrolled. Among these cases, 3 were not diagnosed with IP at initial presentation but were regarded to have infectious diseases. A definite family history of 3 consecutive generations was proved not only by clinical manifestations but also by molecular study in 1 patient. The patient also had retinal and vitreous body hemorrhage, which rapidly progressed to retinal detachment of the right eye in 2 months. Another patient presenting with stage III hyperpigmentation at birth had an extremely rare finding of left foot deformity. The male patient had unilateral and localized vesicular lesions over his left thigh. Conclusion: Diagnosis of IP is difficult in the neonatal period. Referral to experienced specialists is necessary. Multiple clinical characteristics of IP and rapid progression of ophthalmologic manifestations can be demonstrated through our study. Furthermore, 3 of the 4 cases in our study are the very first reports in Taiwan.

Pulmonary hemorrhage in very low‐birthweight infants: Risk factors and management

Background:  Beginning 2007, the intratracheal route of epinephrine to end massive pulmonary hemorrhage (MPH) in very low‐birthweight (VLBW) infants was modified at Kaohsiung Veterans General Hospital. The aim of the present study was to assess the change in outcomes for these infants, and to evaluate the risk factors of MPH.

Reappraisal of the Prostaglandin E1 Dose for Early Newborns with Patent Ductus Arteriosus-Dependent Pulmonary Circulation

OBJECTIVES The usual initial dose of prostaglandin E1 (PGE1) for ductal-dependent congenital heart disease (CHD) is 50-100 ng/kg/minute. The aim of this study was to review our experience of a low initial dose of PGE1 treatment in early newborns with congenital heart disease and patent ductus arteriosus (PDA)-dependent pulmonary flow. METHODS We reviewed the clinical data of 33 newborns with CHD and PDA-dependent pulmonary circulation who were admitted from January 2005 to December 2010. Clinical parameters were collected, including, PGE1 dosage, oxygenation condition, vital signs, and other related clinical parameters during admission. Echocardiography was employed to assess the status of the PDA as clinically indicated. RESULTS Thirty-three newborns, including 17 males and 16 females, with CHD and PDA-dependent pulmonary circulation were enrolled in the study. Their mean age was 2.9 ± 5.1 (within the range of 1-26) days with a median of 1.0 day. Among the 33 cases, 25 were diagnosed with pulmonary atresia and eight with critical pulmonary stenosis. Twenty-five of our patients were treated with the initial low-dosage regimen of 20.0 ± 7.4 ng/kg/minute in our neonatal intensive care unit. None of these 25 patients with had significant apnea necessitating intubation and none had hypotension, fever, convulsion or cortical hyperostosis. Three of the eight patients who were treated with high-dose PGE1 (39 ± 13.2 ng/kg/minute) before referral to our unit had apnea and intubation after PGE1 use. All patients had adequate PDA patency with a low maintenance dose of 10.5 ± 5.3 ng/kg/minute before operation under our protocol. CONCLUSION In our experience, adequate PDA flows in early newborns with CHD and PDA-dependent pulmonary circulation could be achieved at a much lower dose than recommended in the literature. The lower dose of PGE1 also causes much fewer complications, such as apnea, fever, and hypotension. For early newborns with CHD and PDA-dependent pulmonary circulation, treatment with a lower initial dose of PGE1 of 20 ng/kg/minute and a maintenance dose of 10 ng/kg/minute is recommended.

Urinary tract infection in infants: a single-center clinical analysis in southern Taiwan.

BACKGROUND This study summarized the epidemiology, etiology, and susceptibility of pathogens to antibiotics, and specific characteristics in infants aged less than 4 months diagnosed with urinary tract infection in the past decade in Taiwan. METHODS The medical charts of patients aged less than 4 months admitted for urinary tract infection to Kaohsiung Veterans General Hospital between January 2001 and December 2009 were retrospectively reviewed. RESULTS A total of 132 patients, with male predominance (68.9%), were enrolled. The top three pathogens were similar to those identified in previous studies in Taiwan. The most common pathogen, Escherichia coli (85.3%), was resistant to ampicillin (75.9%), followed by sulfamethoxazole/trimethoprim (31.7%), and cefazolin (28.5%). Dimercaptosuccinic acid (DMSA) renal scan revealed 34.5% positive findings, while the vesicoureteral reflux (VUR) rate was 37.8% by direct radionuclide voiding cystography and/or voiding cysto-urethrography. Positive DMSA findings significantly correlated with VUR (p<0.001) and higher C-reactive protein level (p<0.05). CONCLUSIONS E coli was the most common pathogen in the present cohort, and the top three pathogens were similar to those found in general pediatric population in Taiwan. VUR was the most common genitourinary tract anomaly in this age group. Positive DMSA was well correlated with VUR and higher C-reactive protein level.

Perinatal factors in patent ductus arteriosus in very low-birthweight infants

The relationship between hemoglobin and patent ductus arteriosus (PDA) has not been discussed before. The aim of this study was to assess the influence of hemoglobin and perinatal factors on PDA in very low‐birthweight (VLBW) infants.

A potentially fatal complication during subclavian vein catheterization in an infant with congenital heart disease—puncture to pulmonary artery directly: a case report

The most frequent and acute complications of subclavian vein catheterization are arterial puncture to the subclavian artery and pneumothorax. We report an arterial puncture directly to the pulmonary artery in infant during subclavian vein catheterization.

Hemopericardium from right atrial laceration in a newborn

We report the case of a newborn who suffered right atrial laceration during delivery. To our knowledge, there has been no previous report of blunt injury causing right atrial laceration with hemopericardium complicated by cardiac tamponade in the perinatal period.

The Epidemiological Trends and Impact of Resistance on the Outcome of Neonatal Omphalitis: 12 Years Experience in a Medical Center

Objectives: To understand the epidemiological trends and impact of resistance on the outcome of neonatal omphalitis, we investigated the features of omphalitis cases that occurred in a medical center over the past twelve years. Materials and Methods: The medical records of patients with omphalitis between January 1994 and December 2005 were reviewed retrospectively. A total of 64 patients with inflammatory change of the umbilical stump or the surrounding tissue were enrolled. The patients were divided into two groups in different time periods to compare the epidemiological trends. Results: Staphylococcus aureus and Escherichia coli were the leading pathogens of neonatal omphalitis. Infections of S. aureus increased significantly in the latter 6 years. The proportion of patients with resistant bacteria did not increase (42.4% vs. 46.4%, p=0.754) over time, while the occurrences of those with methicillin resistant staphylococcus aureus (MRSA) did increase (23.5% vs. 43.5%, p=0.191). There was no difference in the systemic signs, laboratory data or hospital stay between the presence and absence of resistant bacteria groups. Conclusions: S. aureus caused the majority of omphalitis infections and the emergence of MRSA is noteworthy. In vitro resistance is not indicative of clinical severity and further studies are necessary to aid decisions on the optimal management of omphalitis.

An Outbreak of Coxsackievirus B5 Infection in a Nursery for the Newborn

An outbreak of coxsackievirus B5 infection among neonates occurred during October 31 to November 5, 2001. Four infants were referred from the same nursery of a maternal hospital to the sick baby room of our hospital with a diagnosis of neonatal sepsis. Another infant staying in the same sick baby room had a fever on November 7, 2001. Meningitis caused by coxsackievirus B5 was diagnosed in all 5 patients thereafier Clinical manifestations of these infants included fever, irritable crying, and lethargy. Neither the white blood cell (WBC) count nor C-reactive protein (CRP) was significantly elevated in any of the 5 patients. The WBC counts in the cerebrospinal fluid (CSF) ranged from 1 to 1505/mm^3 with lymphocytic predominance. Hypoglycorrhachia was noted in 40% (2/5) of patients. The virus was isolated from the CSF and at least 1 other site (throat, urine, or stool) in all the patients. The median duration of fever was 5.6 (range, 4~7) d. A brain sonogram performed at the acute stage revealed no brain edema. No neurological sequelae had been noted by 1 year of age. The outbreak subsided after a policy of strict hand-washing before and after contact with infants was applied in both hospitals.