Little is known about processing mechanism of sensory input from the periodontal ligaments to the trigeminal motor nucleus for the control of chewing force and modulation of chewing pattern. Low threshold mechanoreceptive periodontal afferent was labeled with horseradish peroxidase by use of intra-axonal injection technique and investigated with electron microscopy. Quantitative ultrastructural analysis was performed on the 39 serially reconstructed labeled boutons in the trigeminal motor nucleus in cat. Labeled bouton contained clear spherical vesicles and one or two large dense cored vesicles. Most of labeled boutons were dome or round shape. All the analysed labeled boutons were presynaptic to dendritic shaft or distal dendrite and those presynaptic to soma or proximal dendrite were not observed. A large number of labeled boutons (46.2%) were postsynaptic to one or two presynaptic pleomorphic vesicle containing endings. Synaptic triad, in that a presynaptic ending which is presynaptic to the labeled bouton, in turn, is presynaptic to dendrite that is postsynaptic to the labeled bouton, was observed in 10.3% of the labeled boutons. Most of the labeled boutons showed simple synaptic organization, in that 64.1% of the labeled boutons made synaptic contacts with one or two neuronal profiles. One (2.6%) of the 39 analyzed labeled boutons showed synaptic contacts with 5 or more neuronal profiles. Labeled bouton volume, mitochondrial volume, apposed surface area and active zone area showed wide variation. These ultrastructural parameters were positively correlated with bouton volume. The values for apposed surface area and active zone area with presynaptic p-endings, in contrast to those with postsynaptic dendrites, showed narrow range and had little correlation with bouton volume. The present study revealed characteristic features on ultrastructural parameters of labeled boutons from periodontal afferent which is involved in periodontal masseteric reflex, and that influence on the postsynaptic trigeminal motoneurons showed wide variability.
Animals ; Carisoprodol ; Cats ; Dendrites ; Horseradish Peroxidase ; Mastication ; Microscopy, Electron ; Mitochondrial Size ; Neurons ; Periodontal Ligament ; Reflex ; Synapses

PURPOSE: Early diagnosis of congenital hearing loss through the neonatal hearing screening test minimizes language defect. This research intends to identify frequency of congenital hearing loss in infants through neonatal hearing screening test with the aim of communicating the importance of hearing test for infants. METHODS: From May 20, 2003 to May 19, 2004, infants were subjected to Automated Auditory Brainstem Response test during one month of birth to conduct the test with 35 dB sound. Infants who passed the 1st round of hearing test, were classified into 'pass' group whereas those who did not were classified into 'refer' group. Infants who did not 'pass' in the hearing test conducted within one month of birth were subjected to re-test one month later, and if classified as 'refer' during the re-test, they were subjected to the diagnosis for validation of hearing loss by requesting test to the hearing loss clinic. RESULTS: There was no difference among the 'pass' and 'refer' group in terms of form of childbirth, weight at birth and gestational age. In the 1st test, total of 45 infants were classified into 'refer' group. Six among 35 who were subjected to re-test (17%) did not pass the re-test, and all were diagnosed with congenital hearing loss. This corresponds to 0.35% (3.5 per 1, 000) among total number of 1, 718 subjects. CONCLUSION: In our study the congenital hearing loss tends to be considerably more frequently than congenital metabolic disorder. Accordingly, newly born infants are strongly recommended to undergo neonatal hearing screening test.
Diagnosis ; Early Diagnosis ; Evoked Potentials, Auditory, Brain Stem ; Gestational Age ; Hearing Loss* ; Hearing Tests ; Hearing* ; Humans ; Infant ; Mass Screening* ; Parturition

Synovial sarcoma is a rare malignancy in the thoracic cavity, especially in the mediastinum. In this paper, a case of primary mediastinal synovial sarcoma is reported. A 34-year-old woman was hospitalized with dyspnea. Her chest X-ray and computed tomography (CT) showed a 16x13x11 cm mass in her anterior mediastinal space. Surgical resection was performed but was incomplete. The pathological and immunohistochemical analysis confirmed the diagnosis of monophasic spindle cell synovial sarcoma. The patient underwent adjuvant radiotherapy for two months, but local recurrence and metastasis occurred in her pleural cavity. She eventually underwent chemotherapy for one year and died 18 months after her operation.
Dyspnea ; Female ; Humans ; Mediastinum ; Neoplasm Metastasis ; Pleural Cavity ; Radiotherapy, Adjuvant ; Recurrence ; Sarcoma ; Sarcoma, Synovial ; Thoracic Cavity ; Thorax

Niemann-Pick disease (NPD) is an inherited metabolic disorder caused by a deficiency of the enzyme acid sphingomyelinase coded by SMPD1 gene. In contrast with type A NPD, a severe neurodegenerative disease of infancy, type B NPD patients have little or no neurodegeneration, and frequently survive into adulthood. Although over 100 mutations have been found within the SMPD1 gene causing NPD, there was only one report about SMPD1 mutation status of a Korean NPD patient. We report a case of a 32-yr-old female, who presented with thrombocytopenia without any neurologic involvement. Hepatosplenomegaly was detected by both physical examination and imaging studies, and a thoracic radiograph examination showed a pattern of interstitial lung disease. Biochemical tests revealed increased liver enzymes, cholesterol, triglyceride, and LDL-cholesterol, and decreased HDL-cholesterol. Sea-blue or foamy vacuolated histiocytes occurred in bone marrow and liver. Sequencing analysis of SMPD1 using genomic DNA from peripheral leukocytes identified a compound heterozygote of two mutations at exon 2: p.E246K and p.A357V. The former is a known mutation in an Italian patient, and the latter has not been reported yet. She has received oral rosuvastatin to treat hyperlipidemia at a dose of 10 mg per day for 4 months. This is the second report in which the mutation of SMPD1 gene was detected in a Korean NPD patient. The active genetic analysis of SMPD1 gene in patients with typical findings of type B NPD would enable us to facilitate diagnosis as well as to accumulate data on molecular characteristics of Korean NPD patients.
Adult ; Base Sequence ; Bone Marrow Cells/pathology ; Female ; Humans ; Korea ; Liver/pathology ; Niemann-Pick Disease, Type B/*diagnosis/genetics/radiotherapy ; Pregnancy ; Sea-Blue Histiocyte Syndrome/diagnosis/pathology ; Sequence Analysis, DNA ; Sphingomyelin Phosphodiesterase/genetics ; Tomography, X-Ray Computed