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

No abstract available.
Lung*

We report a case of secretory carcinoma with axillary lymph node metastasis in a 21-year old woman. She was aware of a mass in her breast for 10 years and noticed a rapid growth of the preexisting mass during the last years. Histologically, the tumor was composed of micropapillary and microcystic or cribriform glandular structures which contained eosinophilic, mucinous, intraluminal secretions. The center had a dense hyalinized strama with a solid infiltrative growth of tumor cells with intracytoplasmic secretory vacuoles at the periphery. In addition, marked intraductal papillary epithelial proliferations were present at the superficial portions of the tumor near the nipple. Prognostic factors and their relationship to juvenile papillomatosis are discussed with a review of the literature.
Female ; Humans ; Neoplasm Metastasis ; Breast Neoplasms

Bromidrosis is a disorder characterized by rancid body odor which influences a patient's social life and mental health. The therapeutic modalities and the mechanism of bromidrosis have been carefully studied, however, there have been few reports about the genetic inheritance of bromidrosis. We investigated the family history of 42 patients who were operated on for bromidrosis and followed up to the third generation in 10 cases. The results were as follows: Results of investigation which were followed up the second generation. The fathers of five patients and the mothers of 11 patients had bromidrosis in 18 male patients. The fathers of six patients and the mothers of 12 patients had bromidrosis in 24 female patients. Thirty-four patient (81.0%) among a total of 42 have a single parent with bromidrosis. Result of investigation which were followed up to the third generation Bromidrosis was occurred in 17 of 42 patients (40.5%) in the second generation, and 18 of 27 patients (66.7%) in the third generation. In one case, a father transmitted bromidrosis to his three sons, and as a result, X-linked inheritance could be ruled out Bromidrosis was not skipped in every generation of all families. We on conclude that bromidrosis is an autosomal dominant inherited disorder.
Fathers ; Female ; Genes, X-Linked ; Humans ; Male ; Mental Health ; Mothers ; Odors ; Single Parent ; Wills*

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

No abstract available.
Coronary Vessels* ; Pulmonary Artery*