Respiratory syncytial virus infection cases in congenital heart disease patients / 소아과

PURPOSE: Respiratory syncytial virus (RSV) is one of the main pathogens causing lower respiratory infections (LRI) in young children, usually of limited severity. However, in congenital heart disease (CHD) patients, one of the high-risk groups for RSV infection, RSV can cause serious illnesses and fatal results. To elucidate the effects of RSV infection in CHD patients, we observed RSV infection cases among CHD patients and non-CHD patients. METHODS: On admission of 343 LRI patients over 3 years, 77 cases of RSV infection were detected by the RSV antigen rapid test of nasopharyngeal secretion. We compared RSV infection cases among groups of CHD and non-CHD patients. RESULTS: During the winter season, RSV caused 20-50% of LRI admissions in children. In patients with completely repaired simple left to right (L-R) shunt diseases such as ventricular septal defect, atrial septal defect, and patent ductus arteriosus, RSV infections required short admission days similar to non-CHD patients. In patients with repaired CHD other than simple L-R shunt CHD, for whom some significant hemodynamic problems remained, RSV infection required long admission days with severe clinical course. In children with unrepaired CHD, RSV infection mostly occurred in early infant age, with long admission days. RSV infections within a month after cardiac surgery also required long admission days and severe clinical course. CONCLUSION: To avoid the tragedic outcome of severe RSV infection in the CHD patients, efforts to find the subgroups of CHD patients at high risk to RSV infection are needed, and effective preventive treatment should be applied.

Electrodiagnostic Findings and Clinical Characteristics of Familial Hyperkalemic Periodic Paralysis With a SCN4A Met1592Val Mutation

BACKGROUND: Hyperkalemic periodic paralysis (HYPP) is characterized by episodic flaccid paralysis of skeletal muscles that is exacerbated by the consumption of potassium-containing foods, fasting, or rest following exercise. HYPP is largely diagnosed based on clinical features and electrodiagnostic findings. METHODS: Seven patients from three families were assessed by interviews and clinical examinations. Standardized protocols comprising short and long exercise tests were applied to 15 unaffected control subjects and the 7 patients with familial HYPP. RESULTS: Exercise of short duration induced an immediate increase in the amplitude of the compound motor action potential (CMAP) in the patients, and this was significantly larger and lasted longer than that observed in controls within 50 seconds (p<0.05). A long exercise test induced a large increase in the CMAP amplitude in patients immediately after exercise completion, which decreased to normal values with 1 minute. In contrast, controls showed a decreased CMAP amplitude immediately after exercise, which subsequently also returned to the normal value. Precipitants of attacks were vigorous exercise and hunger in all patients, and cold and potassium-rich foods in five patients. All patients experienced clinical myotonia at the eyelids or lips. CONCLUSIONS: We conclude that exercise tests may be helpful in confirming abnormal excitability of muscle membranes in patients with HYPP. We have described the clinical and electromyographic characteristics in familial HYPP with the Met1592Val mutation in the SCN4A gene.

Congenital Myotonic Dystrophy Diagnosed in an Adolescent Period

Type 1 myotonic dystrophy (DM1) is an autosomal-dominant inherited disorder with multisystem involvement, caused by an abnormal expansion of CTG sequence of the dystrophia myotonica protein kinase (DMPK) gene on chromosome 19. Congenital myotonic dystrophy (CDM) is the most severe phenotypic form of DM1. CMD tends to be observed in congenitally affected fetus or neonates born to affected mothers. We report a patient confirmed as CDM during the adolescent period.

A case of maloccluded incisor teeth in a beaver: Castor canadensis

A three-year-old female beaver (Castor canadensis) was referred to the Veterinary Teaching Hospital of Chungbuk National University. It had been raised in the Cheong-ju zoo and had a history of malocclusion caused by improper feeding. General anesthesia was induced, and preoperative intraoral dental radiographs of the rostal maxillary and mandibular dentition were taken and lateral and ventrodorsal extraoral radiographs of the cheek teeth were also taken. The radiographs were negative for apical pathology and revealed a normal appearance of the cheek teeth. The lesion was likely to be related to the excessive length of the maxillary and mandibular incisors. Odontoplasty was performed to reduce overgrowth of the crowns of the incisors. Sequential transverse sections were removed until the crown was reduced by approximately its original length. The pulp chamber was not approached during the operation, as confirmed by postoperative intraoral radiographic evaluation of the incisors. Recovery from anesthesia was uneventful and the beaver returned to normal masticatory activities immediately after the operation.