Biomedical subjects

Shin-Nan Cheng

Publications and source records attributed to Shin-Nan Cheng.

21 records · Page 2Linked to original sources

PubMedDate not supplied

Moyamoya disease initially mimicking MELAS syndrome in a 14-month-old child.

Moyamoya disease is a neurological disease rarely seen in children outside Japan. It is difficult to differentiate moyamoya disease in its early stage from mitochondrial disorders in children when nontraumatic ischemic stroke is considered. We present a 14-month-old Taiwanese female child who had suffered from sudden onset of nontraumatic seizure attack, after which progressive left limb weakness was noted. Initial brain imaging and a series of laboratory studies were performed which suggested mitochondrial disorders, especially mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome. The patient underwent an oral glucose lactate stimulation test (OGLST), and mitochondrial disorders were not favored. MR angiography of the circle of Willis showed moyamoya disease. The patient then underwent an encephalo-duro-arterio-myo-synangiosis (EDAMS) operation. Evaluation of the neurodevelopmental and intelligence outcome required close long-term follow-up.

Brain↗

PubMedDate not supplied

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↗

PubMedDate not supplied

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↗