• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.711-715
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• 2002

The 18q-syndrome is a deletion disorder that occurs in humans. Clinical symptoms are mental retardation, craniofacial anomalies, skeletal deformity, seizure, and hearing loss. 18q- deletion occurs over a broad region, spanning the interval from 18q22.2 to 18qter rather than a single critical region containing 18q. We experienced a case of 18q-syndrome in a male child. It was diagnosed by clinical and chromosomal study. He was a 15month-old infant who was admitted because of prolonged fever and vomiting. And he manifested a depressed midface, esotropia, anhydrosis, and developmental delay. Peripheral blood chromosome studies showed deleted chromosomal material at the distal part of the long arm of chromosome 18. He showed right hydronephroureterosis on IVP. So, he was diagnosed as 18q-syndrome with right hydronephroureterosis and anhydrosis. We report this syndrome with a review and related literature.

• The Korean Journal of Pain
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• v.8 no.1
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• pp.55-59
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• 1995

Stellate ganglion block (SGB) is applicated frequently to increase the blood flow and to reduce the pain in head, neck and upper extremity. The effects of SGB are able to be estimated by clinical signs and symptoms of Horner's syndrome, skin warmth, anhydrosis, etc. The effects are also estimated by sympathetic function and the blood flow. Blood flow velocities and pulsatility indices of common carotid,d axillary, brachial and radial artery were measured by Doppler flowmeter after SGB with 1% lidocaine at C6 level. Blood velocities of all arteries were increased and pulsatility indices of all arteries were decreased. This results suggest that SGB increase the blood flow of head and upper extremity and Doppler flowmeter is a good indicator of the effects of SGB.

• The Journal of Internal Korean Medicine
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• v.12 no.1
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• pp.123-139
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• 1991

This study has been carried out to investigate the cause and symptom of Han-So(寒嗽) by referring to 50 literature. The results were as follows ; 1. The factors causing Han-So(寒嗽) are divided into 3 groups. The 1st inner factors are lung affected by cold(寒傷肺), lung affected by wind and cold, and spleen and lung affected by cold. The 2nd outer factors are body cool, cold water drinking, lung week and cool, spleen week and cool. The 3rd non-inner non-outer factor is the pholegm accumulated. 2. The symptom of Han-so(寒嗽) is as follows ; occlusion of the naris, intolerance to cold, anhydrosis tense pulse, fever, running at the nose, headache.

• Annals of Clinical Neurophysiology
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• v.3 no.1
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• pp.43-46
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• 2001

Acute pandysautonomic neuropathy(APN) is an uncommon clinical entitiy involving vasomotor, sudomotor, pupilomotor, secretomotor and other autonomic systems. Both sympathetic or parasympathetic fibers are involved with relative preservation of somatic sensory and motor function. Although APN shares several clinical features with GBS, it is not clear whether APN is a subvariety of GBS. We report two young patients with APN. Patient 1 was a 18-year-old girl with recurrent fainting spells. Patient 2 was a 23-year-old man sufferring from unexplained nausea and vomiting. Both had a history of previous upper respiratory infection. They presented with gastroparesis, anhydrosis and orthostatic hypotension. Mild numbness and tingling sense was present, but motor power was intact. Neurologic examination showed bilateral tonic pupil, decreased pain and vibration sense, and absent tendon reflexes. Nerve conduction study indicated diffuse sensorimotor polyneuropathy. Nerve biopsy in patient 2 revealed axonal degeneration. After conservative management, gastrointestinal symptoms were improved in patient 2, however, patient 1 suffered from the symptoms lasting more than several months. These cases suggest that post-infectious dysautonomic symptoms in young patient may indicate the diagnosis of APN. Although the natural course is generally benign, accurate diagnosis and proper management may be mandatory for the better clinical outcome.

• Journal of Chest Surgery
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• v.27 no.9
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• pp.779-784
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• 1994

Vidio-assisted thoracic surgery[VATS] has recently evolved as an alternative to thoracotomy for several thoracic disorders,and the role of thoracoscopy has expanded with advances in surgical techniques and instruments. From May 1993 to May 1994, 13 patients with mediastinal mass underwent VATS for diagnosis and treatment at Gil General Hospital. There were four males and nine females, and their ages raged from 5 years to 66 years with average 38.8 years. Among 13 patients, 3 were operated for tissue diagnosis,9 for treatment,and 1 for diagnosis and treatment. Pathologic diagnoses were as follows; 5 benign neurogenic tumors, 2 thymoma, 2 sarcoidosis, 1 teratoma, 1 peripheral neuroepithelioma, 1 tbc lymphadenitis, and 1 pericardial cyst. The mean time of operation was 111.7 $\pm$ 30.7 minutes[60-160], mean duration of chest tube drainage was 2.9 $\pm$1.9days[1-9], mean hospital stay was 6.2 $\pm$2.6 days[4-13]. There was no patient needed blood transfusion or conversion to open thoracotomy. Accurate diagnosis was possible in all patients operated for diagnosis and /or treatment.[4/4,100%] Two complications occurred in two patients: 1 transient Horner,s syndrome,1 anhydrosis of left arm. Compared with those of conventional thoracotomy done for mediastinal mass during previous 2 years[May 1991 - April 1993], operative results of VATS were better in all aspects. For mediastinal mass, we concluded that VATS can be done with less morbidity,less complication,less blood loss,shorter operation time and hospital stay,and not more expensive in cost than conventional thoracotomy. Noticeably, we think that VATS is the operation of choice for the diagnosis and palliation of malignant mediastinal mass.

• The Journal of Korean Medicine
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• v.15 no.2 s.28
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• pp.321-333
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• 1994

1. Autum-dryness syndrome(秋燥) is caused by dry-warm or dry=-cool weather condition when lower-warmer Eumfen(下焦陰分) was injured by jung hyoul hatal(精血下脫) or taking medicines of dryness in autumn. 2. The symptoms of autum-dryness syndrome are headach, fever, chilling, anhydrosis, dry cough, tinnitis, dry lip, dry skin, chest discomfort in cool-dryness syndrome(凉燥) and headach, fever, dry cough, paroxymal cough, dry skin, chest pain, polydipsia, tinnitis, eye redness, sore throat in heat-dryness syndrome(溫燥). 3. Hyangsochongsitang(香蘇蔥頭湯) chn be applied for terating cool-dryness syndrome(凉燥) and chungjogoopyetang(淸燥救肺湯) for heat-dryness syndrome(溫燥). Sang Hang Tang(桑杏湯) can be administered when the evils located in the upper-warmer, and Yukmihwan(六味丸) can be administered when located in the lower-warmer(下焦) 4. According the determination of treatment based on the differentiation of symptoms and signs. When the symptoms of Weifen syndrome(衛分證) are headach, fever, dry lip or chilling or dypsia and the treatment of weifen syndrome apply Hangsosan(行蘇散) or Sang hang tang(桑杏湯). When the symptoms of Qifen syndrome(氣分證) are tinnitis, eye redness, sore throat, chest discomfort, polydipsia, dry cough, watery diarrhea, constipation and Jibaekjihwang(知栢地黃丸) when Ohingulpitang(五仁橘皮湯) Chungjogupyetang(淸燥救肺湯) can are applied for treating Qifen syndrome(氣分證). When the symptoms of younghylfen syndrome(營血分證) are dry cough, watery diarrhea, dypsia, chest discomfort, hematemesis, epistaxis and agyohwang keum tang(阿膠湯) Oknyujun(玉女煎) can be applied for treating Younghyulfen syndrome(營血分證).

• Journal of Korean Neurosurgical Society
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• v.60 no.2
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• pp.257-261
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• 2017

Objective : This study was conducted to assess the surgical results of one-stage posterior minimal laminectomy and video-assisted thoracoscopic surgery (VATS) for the treatment of thoracic dumbbell tumor and to describe its precise technique. In addition, we investigated the technique's usefulness and limitations. Methods : Seven cases of thoracic dumbbell tumor (two men and five women, mean age, 43 years) were analyzed retrospectively. Pathological findings included schwannoma in four patients, neurofibroma in two patients, and hemangioma in one patient. The location of tumors varied from T2/3 to T12/L1. Dumbbell tumors were resected by one-stage operation using posterior laminectomy followed by VATS without instrumentation. Clinical data were reviewed. Results : The mean follow-up period was 25 months (range, 3-58 months), and the operative time ranged from 255 to 385 min (mean, 331 min), with estimated blood loss ranging from 110 to 930 mL (mean, 348 mL). The tumor was completely resected without instrumentation and postoperative instability in all cases. Postoperative complications included atelectasis and facial anhydrosis in one case each. Conclusion : One-stage posterior minimal laminectomy and VATS may be a safe and less invasive technique for removal of thoracic dumbbell tumor without instability. This method has the advantage of early ambulation and rapid recovery because it reduces blood loss and postoperative pain.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.37-41
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• 2014

Fabry disease is an X-linked disease caused by deficiency of the lysosomal enzyme alpha-galactosidase A. Affected males present anhydrosis, acroparesthesia and angiokeratoma, and subsequently cardiac, cerebral and renal complications are followed. Females and atypical variants show heterogeneous clinical symptoms. In 2001, two recombinant enzymes were approved for Fabry disease: agalsidase alpha and agalsidase beta. Since the introduction of enzyme replacement therapy (ERT), the number of long-term follow-up studies has been reported. Long-term ERT showed effectiveness on renal function in patients with chronic kidney disease, decrease or stabilization of left ventricular mass, and improvement of pain and quality of life. However, there were limited effects on cerebrovascular events and their mortality. Current literatures on the clinical effect of ERT have reported limited datain adult patients who have already advanced disease. Therefore, further study for pre-symptomatic patients and atypical variants is needed to verify the impact of ERT. This review summarized recent progresses in ERT and limitations of long-term effect of ERT in patients with Fabry disease.