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Biomedical subjects

Chuen-Ming Lee

Publications and source records attributed to Chuen-Ming Lee.

6 recordsLinked to original sources

Dizygotic twins discordant for early-onset Citrobacter koseri and group B streptococcal sepsis.

Early-onset neonatal sepsis is usually a multisystem fulminant illness with prominent respiratory symptoms, and typically the infant has acquired the organism from the maternal genital tract during the intrapartum period. In this article, we report a rare case of dizygotic twins where each individual suffered early-onset sepsis caused by a different pathogen. Group B streptococcal (GBS) sepsis was diagnosed in twin A 1 day after birth; sepsis and meningitis caused by Citrobacter koseri was diagnosed in twin B at the age of the 4 days. The mother developed pre-eclampsia and fever and the twins were delivered via cesarean section at 35 week's gestation. Twin A received ampicillin treatment for 14 days and recovered fully. Twin B was treated with ceftriaxone for 4 weeks and follow-up brain ultrasound revealed persistent enlargement of the bilateral-lateral ventricles. When empiric antibiotic is considered for the symptomatic twin of a sibling with early-onset GBS infection, samples of blood and cerebrospinal fluid (CSF) should be obtained for culture study before treatment. Adjustment of antibiotic treatment based on the results of cultures and CSF Gram stain and antibiotic susceptibility test is essential.

Adult↗

Use of magnetic resonance cholangiopancreatography to diagnose neonatal congenital choledochal cyst.

Antenatal identification of choledochal cysts has become increasingly common with advances in prenatal ultrasonography. However, the antenatal diagnosis needs to be confirmed postnatally and a preoperative radiologic evaluation of the cyst prior to complete resection is required. There have been few reports of the application of magnetic resonance cholangiopancreatography in neonates, or in neonates with large choledochal cysts. We describe a neonate with a progressively growing giant choledochal cyst, which was initially detected at 28 weeks' gestation. After delivery, the baby did not feed well and suffered from jaundice and frequent postprandial bilious vomiting. At the age of 11 days, magnetic resonance cholangiopancreatography was used to confirm the prenatal diagnosis and provide a thorough preoperative evaluation. He underwent early resection of the cyst at 15 days of age and recovered uneventfully. Magnetic resonance cholangiopancreatography is an alternative diagnostic method for confirming the antenatal diagnosis of large choledochal cysts in neonates. It may also provide useful diagnostic information for preoperative evaluation.

Cholangiopancreatography, Magnetic Resonance↗

X-linked centronuclear myopathy.

We report the cases of two male preterm newborns with X-linked centronuclear myopathy (CNM). This is the most severe type of CNM. Each of them presented with generalized hypotonia, weakness, difficulty in swallowing, and respiratory distress at birth. Physical examination of both newborns revealed long thin face, high-arched palate, mild bilateral ptosis, frog-leg posture, and absence of deep tendon reflex. Diagnosis of the disease was made according to fetal history, family history, muscle histopathology, electron microscopy, and genetic analysis. Subdural hemorrhage of brain and subcapsular hematoma of the liver were found at autopsy of Case 1. The results of molecular analysis of Case 2 and his family favored the diagnosis of X-linked CNM. Molecular studies can be easily performed with only minute amount of DNA of patients, and may help the clinician to predict which patients may be at the risk for medical complications.

Fatal Outcome↗

Early diagnosis of Prader-Willi syndrome in a newborn.

Prader-Willi syndrome (PWS) is a multiple-systemic disorder with many manifestations related to hypothalamic insufficiency, with obesity and behavioral problems as the major causes of morbidity and mortality. We describe a 2-day-old boy who initially presented with neonatal hypotonia and was diagnosed as PWS based on abnormal DNA methylation patterns in the small nuclear ribonucleoprotein polypeptide N (SNRPN) gene at the age of one week, despite the absence of other classical features. Molecular diagnosis for PWS, which has become available in recent years, should be considered for neonates with undiagnosed central hypotonia.

Chromosome Deletion↗

Cat-scratch disease in children at a medical center.

BACKGROUND: Cat-scratch disease (CSD) is a well-recognized, benign, self-limited regional lymphadenopathy occurring in immunocompetent patients. Several retrospective studies have demonstrated that CSD occurs at all ages, however, the disease appears to be more common in children. We conducted a retrospective case study of CSD in children presenting at our hospital, and reviewed the relevant literature. METHODS: The medical records for eight children (age range 4-13 years) diagnosed with cat-scratch disease at the Tri-service General Hospital in Taipei from September 1, 1986 to September 1, 2002 were retrospectively reviewed. Clinical manifestations, diagnostic methods, and treatment types were assessed. RESULTS: The male gender predominated (75%); the median age was 8.6 years. The latency period ranged from 7 days to 1 month, with a median diagnostic interval of 8.5 days. There were no deaths. All patients had lymphadenopathy, and the axillary node was the most prevalent site (62.5%). The most common locations for the scratch or inoculation lesions were the hands and/or fingers (62.5%). Three patients were diagnosed from clinical symptoms and history (37.5%), three from serological study (37.5 %), and two using biopsy or aspiration (25%). All patients were given antibiotic treatment. The most commonly used antibiotic was gentamicin (75%), with the average duration of treatment 8.7 days. The average hospital stay was 8 days, with the lymph-node size reduced relative to pretreatment baseline in all except one case. CONCLUSION: In our experience, the long-term prognosis for children with typical CSD is favorable, and gentamicin may be a good antibiotic option.

Adolescent↗