No abstract available.
Histiocytosis*

No abstract available.
Neurofibromatoses* ; Peripheral Nerves*

No abstract available.
Cyclosporine* ; Humans ; Vitiligo*

Dystrophinopathy, caused by mutations in the DMD gene, presents with variable clinical phenotypes ranging from the severe Duchenne muscular dystrophy (DMD) to the milder Becker muscular dystrophy(BMD) forms. DMD is a recessive X-linked form of muscular dystrophy. Two-thirds of mothers of affected males are thought to be DMD carriers. Approximately 2.5-7.8% of female DMD carriers have muscle weakness and are categorized as manifesting DMD carriers. The symptoms of female carriers of DMD range from mild muscle weakness to severe gait problems. The most commonly presented symptom is mild proximal muscle weakness, which is often asymmetric and progressive, but shows variable clinical spectrum with BMD of more severe DMD-like phenotype. Atypical presentations in manifesting carriers are myalgia or cramps without limb weakness, isolated cardiomyopathy and camptocormia. Multiplex PCR and MLPA analysis are common techniques to identify mutations in the DMD gene. Relationship between X-chromosome inactivation and clinical severity is not clear. Female carriers of DMD are not less common, and they have an important role of birth of a male DMD.
Cardiomyopathies ; Extremities ; Female* ; Gait ; Humans ; Male ; Mothers ; Multiplex Polymerase Chain Reaction ; Muscle Cramp ; Muscle Weakness ; Muscular Dystrophies ; Muscular Dystrophy, Duchenne* ; Myalgia ; Parturition ; Phenotype

PURPOSE: Long-term survival of biliary atresia patients after Kasai's portoenterostomy is being increasingly reported. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly. The authors investigated 5-year survival rates and prognostic factors for survival after Kasai's portoenterostomy using univariate and multivariable methods. METHODS: The authors analyzed 5-year survival rates and prognostic factors in 56 patients who underwent Kasai's portoenterostomy during the period from 1991 to 2005 by the Kaplan-Meier model and Cox proportional hazards model, respectively. Both clinical factors and histological features of the liver and periportal fibrous mass were analyzed as prognostic factors. RESULTS: Younger ages of less than 90 days at surgery, clearance of jaundice and absence of post-Kasai cholangitis in 6 months were predictive of a favorable outcome. A significant difference between the live and the dead was found with regard to an average of 7 or more bile ductules in periportal fibrous mass in 200 HPF (P=0.013). The external diameter of the longest bile ductule in the periportal fibrous mass is not correlated to the prognosis (P=0.49). Independent factors for good prognosis were jaundice-free in 6 months and over 7 bile ductules density in periportal fibrous mass. CONCLUSION: Favorable outcome after Kasai's portoenterostomy for biliary atresia is determined by bilirubin clearance in 6 months after Kasai operation and seven or more bile ductules in periportal fibrous mass. The 5-year post-Kasai survival rate is 77.2%.
Bile ; Biliary Atresia ; Bilirubin ; Cholangitis ; Humans ; Jaundice ; Liver ; Prognosis ; Proportional Hazards Models ; Survival Rate

No abstract available.
Multiple Myeloma* ; Bortezomib

Subdural hemorrhage (SDH) can manifest various neurologic symptoms. However, SDH presenting with only hand weakness has rarely been reported. We report two SDH cases with only hand weakness mimicking peripheral neuropathy. Since SDH can present with hand weakness only, we suggest the clinicians to do a careful history taking and recommend a CT scan in the elderly patients.
Aged ; Hand ; Hematoma, Subdural* ; Humans ; Neurologic Manifestations ; Paresis ; Peripheral Nervous System Diseases* ; Tomography, X-Ray Computed

No abstract available.
Animals ; Horns* ; Keratoacanthoma*

In removable partial denture with crossed occlusion, it is difficult to meet the satisfaction of patient due to variable movement of denture and unfavorable stability under loading. In a case with few unilaterally teeth remaining, additional treatments such as implantation or residual root are required to assure bilaterally stable support. However, due to the medical condition of the patient with hypertension, angina and old age etc., removable partial denture was planned as treatment in this case. Proper diagnosis, accurate analysis and full understanding of the adaptation for neuromuscular system are necessary to recover the vertical dimension of patient from a decreased vertical dimension due to crossed occlusion.
Denture, Partial, Removable* ; Dentures ; Diagnosis ; Humans ; Hypertension ; Mouth Rehabilitation* ; Mouth* ; Tooth ; Vertical Dimension

No abstract available.
Acneiform Eruptions*