Aneurysm of the internal carotid artery is a rare disease and is known to be associated with congenital arterial anomalies such as neurofibromatosis type I (NF-I). NF-I is an autosomal dominant neurocutaneous disorder characterized by a variety of manifestations that involve the central and peripheral nervous systems, skin, vascular system, and skeleton. In particular, the involvement of vascular abnormalities in NF-I is well known. Any vessel may be affected by this condition, although the renal artery is most frequently involved. The vascular abnormality can be occlusive or an aneurysmal degenerative change. Therefore, symptomatic presentations might assume an indolent pathophysiologic course such as hypertension, or manifest as a catastrophic event such as arterial rupture that could result in sudden death. We report a rare autopsy case of an aneurysmal rupture of the internal carotid artery in a woman with suspected NF-I, who collapsed in her home.
Aneurysm ; Autopsy ; Carotid Artery, Internal ; Death, Sudden ; Female ; Glycosaminoglycans ; Humans ; Hypertension ; Neurocutaneous Syndromes ; Neurofibromatoses ; Neurofibromatosis 1 ; Peripheral Nervous System ; Rare Diseases ; Renal Artery ; Rupture ; Skeleton ; Skin

No abstract available.
Anesthesia* ; Anesthesia, General* ; Humans

PURPOSE: To evaluate the predictive value of the early response of 18F-flurodeoxyglucose positron emission tomography (FDG PET) during concurrent chemoradiotherapy (CCRT) for locally advanced non-small cell lung cancer (NSCLC). MATERIALS AND METHODS: FDG PET was performed before and during CCRT for 13 NSCLC patients. Maximum standardized uptake value (SUVmax), mean standardized uptake value (SUVmean), metabolic tumor volume (MTV), and total lesion glycolysis (TLG) were measured and the changes were calculated. These early metabolic changes were compared with the standard tumor response by computed tomograms (CT) one month after CCRT. RESULTS: One month after the completion of CCRT, 9 patients had partial response (PR) of tumor and 4 patients had stable disease. The percent changes of SUVmax (%DeltaSUVmax) were larger in responder group than in non-responder group (55.7% +/- 15.6% vs. 23.1% +/- 19.0%, p = 0.01). The percent changes of SUVmean (%DeltaSUVmean) were also larger in responder group than in non-responder group (54.4% +/- 15.9% vs. 22.3% +/- 23.0%, p = 0.01). The percent changes of MTV (%DeltaMTV) or TLG (%DeltaTLG) had no correlation with the tumor response after treatment. All the 7 patients (100%) with %DeltaSUVmax > or = 50% had PR, but only 2 out of 6 patients (33%) with %DeltaSUVmax < 50% had PR after CCRT (p = 0.009). Likewise, all the 6 patients (100%) with %DeltaSUVmean > or = 50% had PR, but only 3 out of 7 patients (43%) with %DeltaSUVmean < 50% had PR after CCRT (p = 0.026). CONCLUSION: The degree of metabolic changes measured by PET-CT during CCRT was predictive for NSCLC tumor response after CCRT.
Carcinoma, Non-Small-Cell Lung* ; Chemoradiotherapy* ; Glycolysis ; Humans ; Lung Neoplasms ; Positron-Emission Tomography ; Tumor Burden

To investigate effects of cytokines on rheumatoid synovial cells, proliferation and expression of cytokine and metalloproteinase genes were studied with the primary culture of rheumatoid synovial cells which was treated with TNF-alpha, GM-CSF, TGF-alpha, PDGF and IL-B. By [3H] thymidine incorporation assay, TGF-beta and PDGF increased proliferation of synovial cells by 1.5 and 2.5 folds respectively. Cytokine gene expression was assessed by RT-PCR. Rheumatoid synovial cells expressed constitutively TGF-beta and IL-B at a high level and IL-1B, GM-CSF, and MIP-1a at a relatively low level. TGF-beta, GM-CSF and PDGF increased IL-B expression. Expression of pro-inflammatory cytokines and chemokines was increased by GM-CSF and PDGF. Both GM-CSF and PDGF increased the expression of IL-1B, GM-CSF MIP-la and IL-8. In addition, GM-CSF enhanced expression of TNF-alpha. Stromelysin and collagenase are the major proteinases responsible for destruction ot joints in rheumatoid arthritis (RA). These genes were expressed constitutivefy in rheumatoid synovial cells. In summary, PDGF and GM-CSF may piay an important role by inducing or increasing expression of IL-1B, TGF-beta and PDGF by increasing proliferation of rheumatoid synovial cells.
Tumor Necrosis Factor-alpha

PURPOSE: To evaluate the findings and the role of CT in plexiform neuro-fibromatosis of the mediastinum. MATERIALS AND METHODS: We retropectively reviewed the CT scans of five patients with plexiform neurofibromatosis of the mediastinum. The CT scans were reviewed with attention to the distribution of the lesions, appearance and attenuation of mediastinal lesions, enhancement pattern after intravenous contrast infusion and associated findingssuch as intercostal neurofibroma. RESULTS: In all five patients CT scans demonstrated fusiform low attenuated masses which were oriented longitudinally and extended over multiple contiguous scans along the distribution of major mediastinal nerves. In four patients, mediastinal lesions appeared infiltrative, obliterating adjacent mediastinal fat plane. One patient had well defined fusiform masses along the major mediastinal nerves. Postcontrast enhanced CT scans revealed slight central enhancement in two patient and no contrast enhancement in three patients. Associated findings such as neurofibromas of intercostal nerves and sympathetic trunk, or subcutaneous neurofibromas were detected on CTscans in all five patients. CONCLUSION: Characteristic CT findings of low attenuation masses along the major mediastinal nerves are helpful to differentiate plexiform neurofibromatosis from mediastinal lymphadenopathy and to prevent from misreading as a malignant disease.
Humans ; Intercostal Nerves ; Lymphatic Diseases ; Mediastinum* ; Neurofibroma ; Neurofibromatoses* ; Tomography, X-Ray Computed

Large-cell acanthoma is a generally hyperkeratotic, sharply demarcated patch on sun-exposed skin with the outstanding pathologic feature being composed of large, relatively uniform keratinocytes. We describe a case of large-cell acanthoma that involved the skin of the nasal bridge. Patient was a 56-year-old women with a tannish brown patch, 2 cm in size and of 5 years' duration. Controversial issues about nosologic entity of large cell acanthoma are discussed.
Female ; Humans

One of the most important prognostic factors in LCP disease is the extent of epiphyseal involvement. Magnetic resonance imaging is considered to be the technique of choice for early diagnosis of Legg-Calve-Perthes disease. Gadolinium-enhanced spin-echo MR images were obtained after nonenhanced Tl-weighted(spin-echo) and T2-weighted(gradient-echo) images. Four different areas were identified in the femoral epiphysis(necrosis, regenerative, cartilaginous and normal fatty bone tissue). The histological evolution of LCP is well described by Catterall and others. Comparing their description with our MRI finding, we suggest classification of LCP into three phases: (I) necrosis, (II) regeneration(IIa-early and IIb-late) and (III) reossification and sequale. T2 weighted image was useful in the early stage and Tl weighted image was useful in the later stage for evaluation of involved extent of the disease. With MRI, we think that we can find out the stage of LCP more early and rationally, pathological factors more easily and appropriate time for operation exactly. we believe that MRI is more adequate method to decide the stage of LCP disease.
Classification ; Early Diagnosis ; Legg-Calve-Perthes Disease ; Magnetic Resonance Imaging* ; Necrosis

Basal Cell Nevus Syndrome ; Family Characteristics ; Female ; Follow-Up Studies ; Genetic Counseling ; Humans ; Mandible ; Mass Screening ; Mothers ; Odontogenic Cysts ; Recurrence ; Siblings ; Skin ; Young Adult

OBJECTIVE: In the present study, it was hypothesized that the sleep electroencephalogram (EEG) characteristics of young (<30 yrs) and elderly (>55 yrs) OSAS patients would differ. METHODS: We analyzed 76 sleep EEG recordings from OSAS patients (young group: n=40, mean age: 24.3±4.9 yrs; elderly group: n=36, mean age: 59.1±4.9 yrs), which were obtained during nocturnal polysomnography. The recordings were assessed via spectral analysis in the delta (0.5–4.5 Hz), theta (4.5–8 Hz), alpha (8–12 Hz), beta (12–32 Hz), slow sigma (11–13 Hz), and fast sigma (13–17 Hz) frequency bands. RESULTS: Apnea Hypopnea Index (AHI) and sleep efficiency (%) did not differ significantly between the two groups (19.8±14.4 vs. 25.9±16.0, p=0.085; 84.4±12.6 vs. 80.9±11.0, p=0.198, respectively). After adjusting for gender, the slow/fast sigma ratio was not significantly correlated with AHI in the elderly group (r=-0.047, p=0.790) but AHI was inversely correlated with the slow/fast sigma ratio in the young group (r=-0.423, p=0.007). A multiple linear regression analysis revealed that a higher AHI was related with a lower slow/fast sigma ratio in the young group (β=-0.392, p=0.028) but not the elderly. CONCLUSION: In the present study, sleep EEG activity differed between young and elderly OSAS patients. The slow/fast sigma ratio was associated with OSAS severity only in young patients, suggesting that young OSAS patients may have a distinctive brain plasticity compared with elderly patients.
Aged* ; Apnea ; Brain ; Electroencephalography* ; Humans ; Linear Models ; Plastics ; Polysomnography ; Sleep Apnea, Obstructive*

PURPOSE: We investigated the clinical course of infants with congenital heart disease (CHD) who experienced thyroid dysfunction within 100 days of birth. METHODS: We performed retrospective medical reviews of 54 CHD patients (24 male patients) who underwent a thyroid function test (TFT) between January 2007 and July 2016. Data were collected on birth history, diagnosis of CHD, underlying chromosomal or genetic abnormalities, medication history, surgery, ventilator care, and exposure to iodine contrast media (ICM). Results of neonatal screening tests (NSTs) and TFTs were reviewed. RESULTS: A total of 36 patients (29 transient, 7 permanent) showed thyroid dysfunction. Among the seven patients with permanent hypothyroidism, three had an underlying syndrome, three showed abnormal NST results, and one was admitted to the intensive care unit for macroglossia and feeding cyanosis. We found that infants with transient thyroid dysfunction had a lower birth weight and were more commonly exposed to thyroid disrupting medication and/or ICM. However, these risk factors were not significant. A total of 8 patients with a history of ICM exposure showed thyroid dysfunction. Excluding 3 patients with elevated thyroid stimulating hormone before ICM exposure, 5 patients recovered from transient thyroid dysfunction. CONCLUSIONS: We observed thyroid dysfunction in two-thirds of CHD infants (53.7% transient, 13.0% permanent) who had risk factors and received TFT screening within 100 days, despite normal NSTs. Further studies with larger sample sizes are required to revise the criteria for TFT screening in CHD infants.
Birth Weight ; Contrast Media ; Cyanosis ; Diagnosis ; Heart ; Heart Defects, Congenital* ; Humans ; Hypothyroidism ; Infant* ; Infant, Newborn ; Intensive Care Units ; Iodine ; Macroglossia ; Male ; Mass Screening ; Neonatal Screening ; Parturition ; Reproductive History ; Retrospective Studies ; Risk Factors ; Sample Size ; Thyroid Function Tests ; Thyroid Gland* ; Thyrotropin ; Ventilators, Mechanical