No abstract available.

No abstract available.

Congenital epulis of the newborn refers to a very rare gingival tumor that occurs along the anterior alveolar ridge in newborn infants. We report a neonate with 3*2*1.5cm mass protruding from the mouth. This was a pinky, red solid pedunculated mass, attached to the maxillar left canine region of the alveolus at birth. The tumor's large size partially obliterated the oral cavity and caused oral feeding difficulty but did not cause respiratory problems. We described the postnatal MR imaging findings, electromicroscopic, histochemical and immunohistochemical studies of this tumor in order to search for the histogenesis of the tumor. In our case, histochemically, individual tumor cells contained numerous PAS positive cytoplasmic granules. Immunohistochemically, strong and diffuse cytoplasmic staining for vimentin and NSE was observed. Staining with S-100 protein, cytokeratin, desmin, CEA, factor VIII-related antigen, lysozyme, EMA were negative. After total excision of the lesion in our case, we had no complication or recurrence either early or late(up to one year).
Alveolar Process ; Cytoplasm ; Cytoplasmic Granules ; Desmin ; Gingival Neoplasms* ; Humans ; Infant, Newborn* ; Keratins ; Magnetic Resonance Imaging ; Mouth ; Muramidase ; Parturition ; Recurrence ; S100 Proteins ; Vimentin ; von Willebrand Factor

BACKGROUND: Congenital heart disease may be complicated by pulmonary hypertension. We assessed whether inhaled nitric oxide would produce selective pulmonary vasodilation in pediatric patients with congenital heart disease and pulmonary hypertension. METHODS: Inhaled low dose (10 20 ppm) nitric oxide was administrated in patients who were at risk of pulmonary hypertension after operations for congenital heart disease. To identify the nitric oxide effects, we evaluated hemodynamic and ABGA data before (T0) and after (T1) inhaled nitric oxide and just before (T2) decreasing concentration of inhaled nitric oxide. RESULTS: Inhaled nitric oxide decreased pulmonary arterial pressure and increased PaO2/FiO2 without decreasing systemic arterial pressure. CONCLUSIONS: Inhaled nitric oxide selectively decreased pulmonary arterial pressure in patients with congenital heart disease complicated by pulmonary artery hypertension.
Arterial Pressure ; Heart Defects, Congenital ; Heart Diseases* ; Heart* ; Hemodynamics ; Humans ; Hypertension ; Hypertension, Pulmonary* ; Nitric Oxide* ; Pulmonary Artery ; Vasodilation

Paroxysmal choreoathetosis(dyskinesia) is classified into two subtypes: paroxysmal kinesigenic choreoathetosis(PKC) and paroxysmal dystonic choreoathetosis(PDC). PDC consist of attacks of dystonia and /or choreoathetosis during which the patients are dysarthric or anarthric, have irregular clonic movement and dystonic posturing of extremities, and these not precipitated by sudden movement but rather by alocohol, coffee and stress. Attacks are longer (2ninute-4hours), but less frequently(3-4times/day) than PKC, they are not responsive to anticonvulsants(eg, phenytoin, carbamazepine) but controlled by clonazepam.This disease is rare, in the reported families, the transmission was clearly autosomal dominant with high penetrance.
Coffee ; Dystonia ; Extremities ; Humans ; Penetrance ; Phenytoin

Repetitive nerve stimulation test(RNS) was performed on 32 patients with myasthenia gravis who showed positive response to the tensilon test. The result of the repetitive stimulation test before and after the tensilon injection was compared with the point of improvement of the CMAP and decremental response. The results were as follows: 1. All patients with myasthenis gravis showed decremental responses to the low rate of stimulation in RNS. 2. The results after tensilon injections showed significant improvements of the CMAP in ocularis oculi(OOC), flexor carpi ulnaris(FCU)(P <0.0005), deltoid and abductor digiti quinti muscles(ADQ)(P < 0.005). 3. There were improvements of decremental responses after tensilon injection. 1) At 2/sec, there were significant improvements in the muscles of ADQ(P <0.005), deltoidl OOC and FCU(P < 0.0005). 2) At 3/sec, there were significant improvements in the muscles of OOC(P <0.005), deltoid, FCU and ADQ(P < 0.0005). 3) At 5/sec, there were also significant improvements in all the muscles of OOC, deltoid, ADQ, FCU(P <0.0005). These results showed that tensilon improves the RNS abnorrnalities in myasthenic patients with positive tensilon test. We think these findings before and after the tensilon injection are helpful to diagnose and differentiate a patient with suspected myasthenia gravis who has a negative tensilon test or insignificant RNS findings.
Edrophonium* ; Humans ; Muscles ; Myasthenia Gravis*

Repetitive nerve stimulation test(RNS) was performed on 32 patients with myasthenia gravis who showed positive response to the tensilon test. The result of the repetitive stimulation test before and after the tensilon injection was compared with the point of improvement of the CMAP and decremental response. The results were as follows: 1. All patients with myasthenis gravis showed decremental responses to the low rate of stimulation in RNS. 2. The results after tensilon injections showed significant improvements of the CMAP in ocularis oculi(OOC), flexor carpi ulnaris(FCU)(P <0.0005), deltoid and abductor digiti quinti muscles(ADQ)(P < 0.005). 3. There were improvements of decremental responses after tensilon injection. 1) At 2/sec, there were significant improvements in the muscles of ADQ(P <0.005), deltoidl OOC and FCU(P < 0.0005). 2) At 3/sec, there were significant improvements in the muscles of OOC(P <0.005), deltoid, FCU and ADQ(P < 0.0005). 3) At 5/sec, there were also significant improvements in all the muscles of OOC, deltoid, ADQ, FCU(P <0.0005). These results showed that tensilon improves the RNS abnorrnalities in myasthenic patients with positive tensilon test. We think these findings before and after the tensilon injection are helpful to diagnose and differentiate a patient with suspected myasthenia gravis who has a negative tensilon test or insignificant RNS findings.
Edrophonium* ; Humans ; Muscles ; Myasthenia Gravis*

Sparganosis of the central nervous system is an uncommon parasitic zoonosis caused by the migrating larva of the genus Spirometra mansonoides. The patient, a 53-year-old woman, complained of focal involuntary movement involving the right face and headache. Three months after first admission, paraparesis developed. Serial MRI revealed cerebral and spinal lesions and micro-ELISA test disclosed positive reaction to sparganus antigen.
Central Nervous System ; Dyskinesias ; Female ; Headache ; Humans ; Larva ; Magnetic Resonance Imaging ; Middle Aged ; Paraparesis ; Sparganosis* ; Spinal Cord* ; Spirometra

We treated a 66 year-old-male patient with non-traumatic spontaneous E. coli meningitis, whose cerebrospinal fluid showed turbid, and from which E. coli was cultured, and who had urinary tract infection and fatty liver disease which were thought to be predisposing factors to the meningitis. Gram-negative bacillary meningitis is a very rare condition after the neonatal period, and may be complicated by penetrating cranial injuries, neurosurgical interventions or such debilitating diseases as diabetes, liver cirrhosis, urinary tract infection, malignancy and alcholism, etc. It has a high mortality rate in spite of using various antibiotics.
Adult* ; Anti-Bacterial Agents ; Causality ; Cerebrospinal Fluid ; Fatty Liver ; Humans ; Liver Cirrhosis ; Meningitis* ; Mortality ; Urinary Tract Infections

Hereditary progressive dystonia develope in the first decade of ?life and is characterized by marked diurnal fluctuation of symptoms(dystonic postures and movements), i.e.symptoms aggrevated towards evening and alleviated after sleep. These symptoms and signs responded dramatically to levodopa. With the high incidence of familial cases this disorder is considers to be hereditary, but inheritance is not clear yet. Segawa regarded this disorder as a dystonia different from Parkinson's disease and other types of dystonic movement disorders, thereafter it has been reported under name of hereditary progressive dystonia with marked diurnal fluctuation.
Dystonia* ; Incidence ; Levodopa ; Movement Disorders ; Parkinson Disease ; Posture ; Wills