Kimura's disease is a granulomatous disease which develops in the skin, subcutaneous tissues and lymph nodes and is characterized histologically by the presence of lymphoid follicles, vascular proli- feration and infiltration with eosinophils. The disease shows geographical predilection to Japan, China and South East Asia. The exact etiology and pathogenesis remain uncertain. Some patients had proteinuria or nephrotic syndrome. We have recently experienced the superimposed oliguric acute renal failure associated with Kimura's disease in a male patient with chronic renal failure who had been managed conservatively. Inguinal lymph node biopsy revealed Kimura's disease. He recovered from acute renal failure after being treated with hemodialysis and prednisolone. Lymphadeno- pathy and fever subsided with steroid treatment. We report a case of Kimura's disease which was complicated by acute renal failure in the patient with chronic renal failure.
Acute Kidney Injury* ; Biopsy ; China ; Eosinophils ; Far East ; Fever ; Humans ; Japan ; Kidney Failure, Chronic* ; Lymph Nodes ; Male ; Nephrotic Syndrome ; Prednisolone ; Proteinuria ; Renal Dialysis ; Skin ; Subcutaneous Tissue

The Proteus syndrome is a recently described congenital harmatosis consisting of numerous clinical features of great variety. Mainly affected are the musculoskeletal system, primarily by hemihypertrophy, macrodactyly, exostoses and kyphoscoliosis, and the skin and the subcutaneous tissue, primarily by pigmented nevi and subcutaneous tumors. These findings are diagnostic features of Proteus syndrome. We report typical manifestations of Proteus syndrome in a 12-year-old boy with brief review of literature.
Child ; Exostoses ; Humans ; Male ; Musculoskeletal System ; Nevus, Pigmented ; Proteus Syndrome* ; Proteus* ; Skin ; Subcutaneous Tissue

OBJECTIVE: To determine the prevalence, location, and risk factors for the peripheral nerve entrapments of upper extremity among the crutch or cane users. METHOD: We performed the clinical and electrodiagnostic assessments of both upper extremities in 43 crutch or cane users and 49 able-bodied controls. RESULTS: The prevalence for the nerve entrapment of upper extremity among the crutch or cane users was 27.9% by the clinical criteria and 86.0% by the electrodiagnostic criteria. Electrodiagnostically, the median nerve entrapment was 76.7%, and the ulnar nerve entrapment was 72.1% among the crutch or cane users. The carpal tunnel was the most common site for the entrapment. Body weight, duration of disability, and duration of crutch or cane use were found to be significantly correlated with the emtrapments of median nerve, whereas duration of crutch or cane use alone was significantly correlated with the entrapments of ulnar nerve. CONCLUSION: The peripheral nerve entrapments of upper extremity is associated with the chronic crutch or cane use and the preventive strategies need to be developed for the patients with risks.
Body Weight ; Canes* ; Humans ; Median Nerve ; Nerve Compression Syndromes ; Peripheral Nerves* ; Prevalence ; Risk Factors ; Ulnar Nerve ; Ulnar Nerve Compression Syndromes ; Upper Extremity*

PURPOSE: Microdeletions on the Y chromosome have been associated with infertile in men. The deletions cluster in three regions, named azoospermia factor (AZF): AZFa, AZFb and AZFc. It has been suggested that deletions in AZFa result in Type I Sertoli cell-only (SCO) infertility (no spermatogonia present), deletions in AZFb in spermatogenic arrest, and deletions in AZFc Type II SCO (some spermatogonia present with limited spermatogenesis). The purpose of this study was to determine the prevalence of Y chromosome microdeletions and to correlate of the pathologic presentation with specific deletions in infertile Korean men. MATERIALS AND METHODS: We analyzed 115 non-obstructive azoospermic (NOA), 30 obstructive azoospermic (OA), 30 severe oligospermic (sperm concentration <5 x 10(6)/ml) patients and 50 fathered men. We tested leukocyte DNA by PCR for the presence of STS markers, AZFa (sY84, 85, 86), AZFb (sY129, 134, 135, 143, RBM1) and AZFc (DAZ, sY242). The PCR results were confirmed by Southern hybridization and were investigated by SSCP analysis for DAZ gene muations. RESULTS: None of 30 OA and 50 fertile men had microdeletions, but 15 (13.0%) of the 115 NOA and 4 (13.3%) severely oligospermic patients had one or more microdeletions. Deletions involving only the AZFc region were found in 9 men (3 severe oligospermia, 4 spermatogenic arrest, 1 Type I SCO and 1 Type II SCO). Deletions involving only the AZFb were found in 4 (1 severe oligospermia and 3 spermatogenic arrest), and deletions involving only AZFa were found in 1 (Type I SCO). Also, deletions involving the AZFb and AZFc were found in 5 (2 severe oligospermia and 3 Type I SCO). CONCLUSIONS: The prevalence of Y chromosome microdeletion was 13.0% and 13.3% in NOA and severely oligo spermia patients. The earlier reported association with particular types of infertility was not confirmed. The region of the deletions does not correlate with severity of spermatogenic failure or the presence of visible sperm. Deletions involving more proximal regions of the Y chromosome (AZFa) seemed to be rare.
Azoospermia ; DNA ; Fathers ; Humans ; Infertility ; Leukocytes ; Male ; Oligospermia ; Polymerase Chain Reaction ; Polymorphism, Single-Stranded Conformational ; Prevalence ; Spermatogonia ; Spermatozoa ; Y Chromosome*

The effects of chimeric monoclonal antibodies (cMAbs), prostaglandin E, (PGE,), and indomethacin (INDO) on antibody-dependent cellular cytotoxicity (ADCC) against human squamous cell carcinoma of head and neck (SCCHN) cell line were examined. Using the PCI-50 SCCHN cell line as target and normal human peripheral blood mononuclear cells as effector, ADCC was enhanced by the treatment of cMAbs (1.25 p,g/ml), but was inhibited by exogenous PGE (5 X 10' M). The effects of cMAb and PGE were dose-dependent. Maximal suppression of activity occured when PGE was present during the entire 4-hr 'Cr-release assay period, whereas pretreatment of effector cells with PGE had minimal inhibitory effect after washing. These results indicate that decreased ADCC seen with SCCHN targets treated with PGE is related to post-binding events, such as binding of effector and target cells. Pre-treatment of effector cells with INDO (1 ug/ml) resulted in restoration of NK activity which was inhibited by PGE. Our in vitro results suggest that INDO can increase tumor cell killing by the reversal of the suppression for many imrnune functions by PGE.
Antibodies, Monoclonal ; Antibody-Dependent Cell Cytotoxicity ; Carcinoma, Squamous Cell* ; Cell Line ; Head* ; Homicide ; Humans* ; Indomethacin ; Neck* ; Prostaglandins E

Primary frontal sinus lymphoma is a very uncommon disease. In all the previously reported cases, the presenting symptoms have been due to the tumor mass effect. We present an unusual case report of an immunocompetent patient who presented with facial palsy, and then progressively developed other cranial nerve palsies over several months. He was later diagnosed with diffuse large B cell lymphoma originating from the frontal sinus. The patient underwent chemotherapy, but eventually had to receive autologous peripheral blood stem cell transplantation. He is currently disease-free. The clinical course, diagnostic workup, and therapeutic outcome are described.
Adult ; Cranial Nerve Diseases/*diagnosis/pathology ; Frontal Sinus/*pathology ; Humans ; Lymphoma/*diagnosis/pathology ; Male

PURPOSE: To evaluate characteristic MR findings of craniocerebral anomaly and its relationship with neurologic manifestations. MATERIALS AND METHODS: We retrospectively reviewed MR images of 36 patients with craniocerebral anomaly diagnosed by MRI and clinical courses. We correlated the characteristic MR findings in 41 lesions with neurologic manifestastions focusing on seizures and developmental delay. RESULTS: Twenty-three patients with seizures consisted of 14 patients(60%) with neuronal migration disorders and seven(30%) with phakomatosis, among which 18 patients(78%) had generalized type of seizures. Locations of the lesions were the parietal lobes in 11 patients(52%) and the subependymal or periventricular regions in seven(33%). Two patients with tuberous sclerosis had the lesions in both parietal and subependymal areas. Nine patients had the signs of developmental delay that were seen in the four(44%) with schizencephaly, two (22%) with tuberous sclerosis, two(22%) with heterotopia, and one(ll %) with pachygyria. CONCLUSION: Neuronal migration anomaly was relatively common lesion that presented neurologic manifestations such as seizures and developmental delay. Generalized type of seizures was common. We were able to diagnose these anomalies using the MRI that helped establish therapeutic plans.
Humans ; Lissencephaly ; Magnetic Resonance Imaging ; Malformations of Cortical Development ; Neurocutaneous Syndromes ; Neurologic Manifestations ; Neuronal Migration Disorders ; Neurons ; Parietal Lobe ; Rabeprazole ; Retrospective Studies ; Seizures* ; Tuberous Sclerosis

No abstract available.
Transplantation* ; Tuberculosis*

No abstract available.
Transplantation* ; Tuberculosis*

OBJECTIVE: We sought to determine the predictors of midpalatal suture expansion by miniscrew-assisted rapid palatal expansion (MARPE) in young adults. METHODS: The following variables were selected as possible predictors: chronological age, palate length and depth, midpalatal suture maturation (MPSM) stage, midpalatal suture density (MPSD) ratio, the sella-nasion (SN)-mandibular plane (MP) angle as an indicator of the vertical skeletal pattern, and the point A-nasion-point B (ANB) angle for anteroposterior skeletal classification. For 31 patients (mean age, 22.52 years) who underwent MARPE treatment, palate length and depth, MPSM stage and MPSD ratio from the initial cone-beam computed tomography images, and the SN-MP angle and ANB angle from lateral cephalograms were assessed. The midpalatal suture opening ratio was calculated from the midpalatal suture opening width measured in periapical radiographs and the MARPE screw expansion. Statistical analyses of correlations were performed for the entire patient group of 31 subjects and subgroups categorized by sex, vertical skeletal pattern, and anteroposterior skeletal classification. RESULTS: In the entire patient group, the midpalatal suture opening ratio showed statistically significant negative correlations with age, palate length, and MPSM stage (r = −0.506, −0.494, and −0.746, respectively, all p < 0.01). In subgroup analyses, a strong negative correlation was observed with the palate depth in the skeletal Class II subgroup (r = −0.900, p < 0.05). CONCLUSIONS The findings of this study indicated that age, palate length, and MPSM stage can be predictors of midpalatal suture expansion by MARPE in young adults.