One author's affiliation is misspelled in original article.

The congenital renal cystic disease encompasses a complex group of pathologic and clinical entities. We retrospectively reviewed 42 cases of congenital renal cystic lesions classified into four Potter types in a series of 2,063 consecutive autopsies from 1981 to 1996. According to our study based on morphologic, clinical, genetic features and associated anomalies, type I and III are relatively compatible with Potter's original definition. However, it was reasonable that type II and IV are classified to the same group because of: 1) very similar histologic findings representing dysplastic kidney, 2) many associated anomalies, 3) no evidence of inheritance, and 4) presence of a combined type. Syndrome associated cysts, such as Meckel-Gruber syndrome, were also separately classified. If the dysplastic evidence was insufficient for diagnosis to the dysplastic kidney in type II and IV, then these cases would be better classified into a cystic disease associated with congenital hydronephrosis. We propose a classification of the congenital cystic disease of the kidney to be: 1) dysplastic kidney, 2) cystic disease associated with congenital hydronephrosis, 3) polycystic kidney, and 4) syndromic cystic disease.
Autopsy ; Classification* ; Diagnosis ; Hydronephrosis ; Kidney* ; Polycystic Kidney Diseases ; Retrospective Studies ; Wills

No abstract available.
Neurofibromatoses* ; Pseudarthrosis* ; Ulna*

Fulminant hepatic failure (FHF) is a rare but fatal disease with a 40%-80% mortality, often requiring liver transplantation, which is hard to perform in children. A therapeutic intervention alternative to liver transplantation is an extracorporeal artificial liver support system. Molecular Adsorbent Recirculation Systems® (MARS) has emerged as a bridge therapy for adult FHF, whereas in pediatric FHF, there have been a small number of cases of implementing MARS. Recently, we witnessed the recovery of 2 teenagers with FHFs caused by acetaminophen and a diet aid. Both patients were treated uneventfully, with early use of MARS. These cases suggest that early use of MARS may be a promising therapeutic intervention in pediatric drug-induced FHF.

PURPOSE: This study was conducted to develop a comprehensive cognitive rehabilitation program that can be easily applied to brain injured patients by family members or nurses in community or hospital settings. METHODS: A Systemic literature review design was used. Thirty-three related studies were reviewed. RESULT: Based on the results of the literature review, the training tasks for attention were designated to enhancing 4 hierarchical areas, i.e., focused, selective, alternating, and divided attention. On the other hand, the memory rehabilitation tasks mainly consisted of mnemonic skills, such as the association method which helps patients memorize given information by linking together common attributes, the visual imagery method, and self-instruction method. The problem solving rehabilitation program included a task of games or plays which stimulated the patients' curiosity and interest. The training tasks for problem solving were to encourage the process of deriving reasonable solutions for a problematic situation resembling real problems that the patients were faced with in their everyday life. CONCLUSION: It is expected that the cognitive rehabilitation program developed from this study could help patients having difficulty in their every day life, due to a reduced cognitive ability resulting from brain injury, to effectively adapt to every day life.
Problem Solving ; Memory ; Humans ; *Cognitive Therapy ; Cognition Disorders/etiology/*rehabilitation ; Brain Injuries/complications/nursing/*rehabilitation

We experienced a patient who manifested Miller-Fisher syndrome initially, and progressed relapsing ataxia later, There was electrophysiologic evidence of distal sensory polyneuropathy with completely sparig of somatic motor nerves. Sural nerve biopsy showed severe segmental demyelination. All the symptoms improved by intravenous immunoglobulin dramatically but after a period of remission, progressive ataxia reappeared. Plama exchange had some effect and prednisolone alone showed no definite improvement. High dose of prednisolone combined with IVIgG made remission for a long time. We believe that this patient had an unusual form of inflammatory polyneuropathy, a relapsing variant of the Miller-Fisher syndrome of acute idiopathic polyneuritis. In this case, IVIgG was the most effective therapy and prednisolone was added as a long term therapy successfully.
Ataxia* ; Biopsy ; Demyelinating Diseases ; Humans ; Immunoglobulins ; Miller Fisher Syndrome* ; Neuritis ; Polyneuropathies ; Prednisolone ; Sural Nerve

No abstract available.
Esophageal Atresia*

No abstract available.
Arteriovenous Fistula* ; Telangiectasia, Hereditary Hemorrhagic*

PURPOSE: The purpose of this study was to identify the factors affecting shiftwork tolerance among nurses and provide basic data, ultimately, in formulating a plan for improving their shiftwork tolerance. METHODS: The participants were 317 nurses who were working at three general hospitals in Daejeon and Seoul. Data analyzed t-test, ANOVA, correlation analysis, and regression analysis using SPSS 20.0 program. RESULTS: As for the sub-areas of shiftwork tolerance for the respondents, more than a half of nurses working at shifts suffered from insomnia, felt drowsy, complained of chronic fatigue, and were at the anxious and depressed state. Shiftwork tolerance differed by the daily intake of coffee and exercise. Shiftwork tolerance was significantly positively correlated with sleep-wake habits, hardiness, and flexibility and negatively correlated with a languidity. Regression analysis revealed that shiftwork tolerance was affected by age, sleep-wake habits, languidity, flexibility, and hardiness. CONCLUSION: To put the results, nurses had shiftwork tolerance affected by sleep-wake habits, languidity, and hardiness. It is therefore necessary to make a scheme for allowing nurses to improve hardiness and flexibility and decrease the languidity with the objective of improving their shiftwork tolerance.
Circadian Rhythm ; Coffee ; Fatigue ; Hospitals, General ; Pliability ; Seoul ; Sleep Initiation and Maintenance Disorders ; Sleep Wake Disorders ; Surveys and Questionnaires ; Work Schedule Tolerance

No abstract available.
Coronary Vessels* ; Pulmonary Artery*