Shin-Nan Cheng

Polymorphisms of the β2-Adrenergic Receptor Correlated to Nocturnal Asthma and the Response of Terbutaline Nebulizer

BACKGROUND Inhaled β(2)-adrenergic receptor (β(2)-AR) agonists are the mainstay of treatment of acute asthma. Polymorphisms of the β(2)-AR, especially codons 16, 27, and 164, may affect the functions of the receptor. This study was conducted to investigate whether different polymorphisms of the β(2)-AR are related to the treatment responses of an inhaled β(2)-AR agonist in children with nocturnal and nonnocturnal asthma in Taiwan. METHODS The nocturnal asthma group consisted of 27 children (mean age of 10.3±2.4 years), and the nonnocturnal asthma group consisted of 24 patients (mean age of 9.9±3.0 years). Allele-specific polymerase chain reaction was performed to determine 16, 27, and 164 loci alleles of β(2)-AR genetic polymorphisms, and peak expiratory flow (PEF) was measured before and 1 hour after inhalation of 0.2mg/kg/dose of terbutaline to determine the treatment response in these patients. RESULTS The polymorphisms of β(2)-AR 27 but not 16 or 164 were significantly associated with the response to terbutaline nebulizer (p<0.05). The polymorphism of β(2)-AR 16 was associated with nocturnal asthma (p=0.027). The Gly16 allele was more prevalent in the nocturnal asthma group (9/27; 33.3%) than in the nonnocturnal asthma group (3/24; 12.5%). Arg16 allele was less prevalent in the nocturnal asthma (3/27; 11.1%) than in the nonnocturnal asthma group (10/24; 41.7%). There was also a linkage disequilibrium found between β(2)-AR 16 (Arg/Arg) and β(2)-AR 27 (Gln/Gln). CONCLUSION These findings suggest that polymorphisms of β(2)-AR 16 are related to nocturnal asthma and polymorphisms of β(2)-AR 27 are associated with the variable responses to the inhaled terbutaline in children with nocturnal and nonnocturnal asthma.

Concomitant use of acyclovir and intravenous immunoglobulin rescues an immunocompromised child with disseminated varicella caused multiple organ failure.

Varicella is a common and mild disease in healthy children. However, when patients are in immunocompromised conditions, such as receiving chemotherapy for cancer treatment, they are highly vulnerable and it can even prove lethal. Herein, we report a 14-year-old boy with acute lymphoblastic leukemia who was receiving chemotherapy for induction with vincristine, idarubicin, L-asparaginase, and prednisolone, presented with typical varicella skin lesions and varicella-zoster virus was detected in his serum by real-time polymerase chain reaction (PCR). His condition was advanced to multiple organs failure, including fulminant hepatitis, disseminated intravascular coagulation, and myocarditis despite acyclovir administration. After a combined therapy with intravenous acyclovir and high-dose intravenous immunoglobulin, his condition was dramatically improved. We suggest that IVIG may be used immediately with acyclovir when immunocompromised patients with varicella advanced to dissemination are identified.

Xanthoma of Bone in a Normolipidemic Child: Report of One Case

Xanthoma invasion of the bone is a very rare disease especially in normolipidemic children. Bone erosion can be found in patients with this disease. However, due to the similarity of the symptoms of xanthoma with many other diseases including malignancy, the other diseases may initially be to be suggested and xanthoma may not even be considered. In this paper, we present an 8-year-old normolipidemic male child with a parietal bone xanthoma proved using tissue diagnosis. The clinical, radiographic and histological findings are also reviewed.

Bacillus cereus septicemia in a patient with acute lymphoblastic leukemia: A case report and review of the literature

Bacillus cereus is an aerobic Gram-positive, spore-forming, rod-shaped bacterium that is responsible for foodborne illnesses. We report on a 15-year-old girl with B-cell acute lymphoblastic leukemia, who fell into a somnolent state after presenting with a 12-hour history of fever, muscle soreness, myalgia in both calves, sore throat, and vomiting. Fulminant septicemic syndrome caused by B. cereus was finally identified. The aim of this work is the introduction of B. cereus as a differential diagnosis of sepsis in patients with acute leukemia in induction chemotherapy, to prevent delayed treatment.

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.

Subglottic hemangioma associated with cutaneous and cerebellar hemangiomas detected by MRI: report of one case.

Subglottic hemangioma (SGH) is a benign neoplasm that may cause severe and life-threatening respiratory obstruction in infants. However, patients usually present with inspiratory stridor in the first few months of life and may be mistakenly diagnosed as recurrent or persistent croup. Definitive diagnosis is made by image studies, endoscopic examination and biopsy or all. We report a 2-month-old female infant of SGH with initial clinical manifestations of dyspnea and inspiratory stridor co-existing with cutaneous and cerebellar hemangiomas. Clinicians must be alert the possibility of SGH when associated with cutaneous hemangioma. This patient has received oral steroid treatment for more than two months with improvement of the airway obstruction. Although purplish patch lesions over left side of face, eyelid, cheek, and peri-oral regions regressed, the size of the SGH on the followed MRI was slightly enlarged. The diagnosis and various treatments of SGH are discussed and reviewed in this paper.

Cellulitis resulting from infection by Haemophilus influenzae type b: report of two cases.

Cellulitis resulting from infection by Haemophilus influenzae type b (Hib) is uncommon but may be a characteristic of such infection. Especially in the post-Hib vaccine era, such infection represents a potentially life-threatening infectious process due to the relatively high incidence of accompanying bacteremia and meningitis. Here, we report on two apparently previously healthy children who developed Hib cellulitis, one of whom revealed lower-leg cellulitis while the other exhibited buccal cellulitis associated with an oral ulcer. To the best of our knowledge, there have been no formal reports pertaining to buccal cellulitis following infection by Hib yet published in Taiwan, and reports of Hib cellulitis of the extremities still appear to be rare, hence we report on these two cases.

Effective Radiotherapy Cured Cauda Equina Syndrome Caused by Remitted Intracranial Germinoma Depositing

Cauda equina syndrome (CES) in children is very rare and can permanently disable. A remitted intracranial germinoma depositing on the spinal cord, leading to CES, has never been reported. We discuss the case of a 10-year-old girl who presented with sudden ataxia, low back pain, sensory deficits of the left lower extremity, and difficulty urinating and defecating 7 months after totally remitted intracranial germinoma postintracranial surgery and cranial irradiation. Magnetic resonance imaging (MRI) of the brain and spine showed multiple intradural extramedullary homogeneous masses from the cervical to lumbar levels, compressing the conus medullaris and cauda equina. After emergent craniospinal irradiation, the patients neurologic symptoms dramatically subsided. A remitted intracranial germinoma depositing on her spinal cord could be the cause of CES. Early identification and a proper craniospinal irradiation may halt the progression of symptoms.

Acute Respiratory Distress Syndrome in a Full-Term Newborn: Report of One Case

Respiratory distress syndrome, previously called hyaline membrane disease, is a common cause of morbidity and mortality associated with premature delivery. The incidence and severity of respiratory distress syndrome generally increase with decreasing gestational age at birth, and its occurrence in the at-term baby is rare. We report a male newborn who was born via elective cesarean section at 38 4/7 weeks gestation. After birth, he subsequently had severe respiratory distress typical of surfactant deficiency severe enough to be managed with ventilator support. He showed no evidence of intrapartum infection or aspiration. After two doses of surfactant were instilled via endotracheal tube, his respiratory condition showed dramatic improvement and recovered fully without complication before discharge. We report this case to emphasize the possibility that a term baby may develop surfactant-deficient respiratory failure and to suggest that elective delivery before 39 weeks gestation should be undertaken only for good medical reasons. In addition, the pathogenesis of acute respiratory distress syndrome in full-term neonates is discussed.

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.