• Journal of radiological science and technology
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• v.27 no.3
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• pp.5-12
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• 2004

I. Introduction : The constipation is one of the common gastrointestinal symptoms seen in the clinical practice. When people come to the clinic complaining of the constipation, it is generally one of the cases of infrequent, painful or difficult evacuation as well as the hardened feces and unsatisfactory evacuation sense. Since the constipation is heavily influenced by dietary habit as well as the social and medical environment, the diagnostic radiology is useful to establish the objective and standardized definition in consideration of those various factors before diagnosis and treatment of the constipation patient. This paper describes the study of such diagnosis. II. Main Subject : Testing of CTT (colon transit time) is key study of the colon performance. CTT is very helpful in classifying the pathologic physiological types and defining the treatment plan for the chronic constipation. The study methods include using the radipaque marker, multiple marker technique and scintigraphic measurement. The defecography is the functional radiologic examination a that provides not only the anatomical information of anorectal but also performance of the pelvic floor and rectal change during evacuation. Study of dynamic movement of the anorectal during evacuation is helpful for diagnosis and treatment planning as well as follow-up testing for the constipation patient. One of the issues essential for the case history is the thorough observation of whether the patient shows the psychic psychological symptoms such as the behavior disorder or emotional disturbance. In that case, the decision must be made whether or which type of medication is needed for such psychiatric problem. III. Conclusion : The main causes of the constipation are insufficient intake of fiber or liquid. The key objective of such tests is to check etiology of the constipation. In general, the radiological examination does not provde the colon or anorectal performance information. It is envisaged that this study will provide the information to decide the testing and treatment plans and predict the prognosis of the patient by classifying the pathologic physiological types.

• Clinical and Experimental Pediatrics
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• v.50 no.2
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• pp.209-212
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• 2007

Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder of early infancy, and is characterized by periorificial dermatitis, alopecia, and intractable diarrhea. Serum zinc levels are usually low in untreated patients and the oral administration of zinc sulfate can clear skin lesions and other symptoms. Although premature and cow's milk-fed infants are at particular risk of developing AE, there have been a few reports about AE in term and breast-fed infants. We report a case of transient AE in a 4-month-old breast-fed infant. This patient suffered from diarrhea and dermatitis for more than a month. Her skin lesions were erythematous, scaly, crusted, psoriasiform, eczematous, with an eruption at the chin, and a periorificial disposition with involvement of the flexural areas of lower extremities. Her serum zinc level was almost normal at $129{\mu}g/dL$ (reference range: $60-121{\mu}g/dL$), but the zinc level in her hair was low: 8 mg percent (reference range: 10-21 mg percent). Skin biopsy findings were consistent with AE. Seven days after zinc supplementation, the skin lesions and diarrhea improved. The authors recommend that a clinical trial of zinc supplementation be considered in cases where there are suspicious of AE, even when the serum zinc level is normal.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.256-261
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• 2005

Scleroderma is a connective tissue disease of unknown etiology, but known as a kind of auto-immune disease. It is most common in women especially in $30{\sim}50$ years, and very rare in childhood. It can be classified into two main classes : localized scleroderma & systemic sclerosis. Localized type has better prognosis, and usually involves skin only, or in some cases, the muscle below, except inner organs. Systemic type involves skin, oral mucosa and major internal organs. Involving facial skin, we can see small and sharp nose, expressionless stare and narrow oral aperture. Usually they have Raynaud's phenomenon, and in progress, show mouth opening limitation and sclerosis of tongue and gingiva. It is called CREST syndrome showing calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangioectasia. Treatment of scleroderma is systemic and localized steroid therapy, use of collagen-link inhibitor (D-penicillamine), immune depressor and etc. Mouth opening limitation can be improved by mouth stretching exercise. We report a 6 years old boy, diagnosed with localized scleroderma who had mouth opening limitation. We could get additional mouth opening, and have done successful restorative treatment of mandibular and maxillary 1st and 2nd deciduous molar under deep sedation with nitrous oxide and enflurane.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.103-108
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• 2017

Kabuki syndrome is a rare congenital disorder that causes multiple birth defects and mental retardation. Mutation of the lysine methyltransferase 2D (KMT2D) gene is the primary cause of Kabuki syndrome. We report a 4-year-old Korean girl diagnosed with Kabuki syndrome based on distinctive facial features (eversion of the lower lateral eyelid, arched eyebrows, depressed nasal tip, prominent ears), skeletal anomalies, short stature, and molecular analysis, which revealed a novel frameshift mutation in the KMT2D gene. A 4-year-old patient had a past history of congenital cardiac malformations (coarctation of the aorta, ventricular septal defect, atrial septal defect, patent ductus arteriosus), subclinical hypothyroidism and dysmorphic features at birth including webbed neck, short fingers, high arched palate, micrognathia and horseshoe kidney. She showed unique facial features such as a long palpebral fissure, long eyelashes, arched eyebrows with sparseness of the lateral third, broad nasal root, anteverted ears, and small mouth. Her facial features suggested Kabuki syndrome, and genetic analysis discovered a novel heterozygous frameshift mutation (c.4379dup, p.Leu1461Thrfs*30) in exon 15 of the KMT2D gene. The diagnosis of our 4-year-old patient was made through thorough physical examination and history taking, and genetic testing. It is challenging to diagnose patients with Kabuki syndrome at birth, since the characteristic facial features are expressed gradually during growth. Clinical suspicion aroused by regular follow-ups may lead to earlier diagnosis and interventions.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.1 no.1
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• pp.45-55
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• 1998

Purpose: To make objective standards of small intestinal mucosal changes in cow's milk-sensitive enteropathy (CMSE) we analyzed histological changes of endoscopic duodenal mucosa biopsy specimens from normal children and patients of CMSE. Methods: We review the medical records of patients who had been admitted and diagnosed as CMSE by means of gastrofiberscopic duodenal mucosal biopsy following cow's milk challenge and withdrawal. Thirteen babies with CMSE, ranging from 14 days to 56 days of age, were studied. Five non-CMSE patients were used as control, ranging from 22 days to 72 days of age. The morphometric parameters under study were villous height, crypt zone depth, ratio of villous height to crypt zone depth, total mucosal thickness and length of surface epithelium by using H & E stained specimens under the drawing apparatus attached microscope. In addition, the numbers of lymphocytes in the epithelium and eosinophil cells in the lamina propria and epithelium were measured. Results: In the duodenal mucosal biopsy specimens in CMSE we found partial and subtotal villous atrophy with an increased number of interepithelial lymphocytes. The mean villous height($135{\pm}59\;{\mu}m$), ratio of villous height to crypt zone depth ($0.46{\pm}0.28$), total mucosal thickness ($499{\pm}56\;{\mu}m$), length of surface epithelium of small intestinal mucosa ($889{\pm}231\;{\mu}m$) in CMSE was significantly decreased compared with the control (p<0.05). The mean crypt zone depth ($311{\pm}65\;{\mu}m$) was significantly greater than the control ($188{\pm}24\;{\mu}m$)(p<0.05). Infiltration of interepithelial lymphocytes ($34.1{\pm}10.5$) were significantly greater than the control ($13.6{\pm}3.6$)(p<0.05). The number of eosinophil cells in both lamina propria and epithelium was no significant differences between groups (p>0.05). The small intestinal mucosa in treated CMSE showed much improved enteropathy of villous height, crypt zone depth, interepithelial lymphocytes compared with the control as well as untreated CMSE. Conclusion: Quantitation of mucosal dimensions confirmed the presence of CMSE. It seems to be a limitation in the capacity of crypt cells to compensate for the loss of villous epithelium in CMSE. Specimens obtained by gastrofiberscopic duodenal mucosal biopsy were suitable for morphometric diagnosis of CMSE. Improvement of CMSE also can be confirmed histologically after the therapy of protein hydrolysate.

• Tuberculosis and Respiratory Diseases
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• v.46 no.5
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• pp.685-696
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• 1999

Background : Idiopathic pulmonary fibrosis (IPF) is a diffuse inflammatory and fibrosing process that occurs within the interstitium and alveolus of the lung with invariably poor prognosis. The major problem in management of IPF results from the variable rate of disease progression and the difficulties in predicting the response to therapy. The purpose of this retrospective study was to evaluate the short-term efficacy of steroid and immunosuppressive therapy for IPF and to identify the pre-treatment determinants of favorable response. Method : Twenty patients of IPF were included. Diagnosis of IPF was proven by thoracoscopic lung biopsy and they were presumed to have active progressive disease. The baseline evaluation in these patients included clinical history, pulmonary function test, bronchoalveolar lavage (BAL), and chest high resolution computed tomography (HRCT). Fourteen patients received oral prednisolone treatment with initial dose of 1mg/kg/day for 8 to 12 weeks and then tapering to low-dose prednisolone (0.25mg/kg/day). Six patients who previously had experienced significant side effects to steroid received 2mg/kg/day of oral cyclophosphamide with or without low-dose prednisolone. Follow-up evaluation was performed after 6 months of therapy. If patients met more than one of followings, they were considered to be responders : (1) improvement of more than one grade in dyspnea index, (2) improvement in FVC or TLC more than 10% or improvement in DLco more than 20% (3) decreased extent of disease in chest HRCT findings. Result : One patient died of extrapulmonary cause after 3 month of therapy, and another patient gave up any further medical therapy due to side effect of steroid. Eventually medical records of 18 patients were analyzed. Nine of 18 patients were classified into responders and the other nine patients into nonresponders. The histopathologic diagnosis of the responders were all nonspecific interstitial pneumonia (NSIP) and that of nonresponders were all usual interstitial pneumonia (UIP) (p<0.001). The other significant differences between the two groups were female predominance (p<0.01), smoking history (p<0.001), severe grade of dyspnea (p<0.05), lymphocytosis in BAL fluid ($23.8{\pm}16.3%$ vs $7.8{\pm}3.6%$, p<0.05), and less honeycombing in chest HRCT findings (0% vs $9.2{\pm}2.3%$, p<0.001). Conclusion : Our results suggest that patients with histopathologic diagnosis of NSIP or lymphocytosis in BAL fluid are more likely to respond to steroid or immunosuppressive therapy. Clinical results in large numbers of IPF patients will be required to identify the independent variables.

• Journal of Chest Surgery
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• v.31 no.11
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• pp.1049-1055
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• 1998

Background: Coronary artery bypass surgery is an important treatment for ischemic heart disease. Recently operative mortality and morbidity has decreased, however further improvement is necessary. Materials and methods: This study was designed to evaluate the risk of operative mortality and morbidity by retrospective method. From 1992 to 1997, eighty six patients underwent coronary artery bypass surgery. There were 61 males and 25 females aged 36~74 years(mean, 58.6). Fourteen patients(16%) had previous PTCA or stent insertion, 41 patients(48%) had unstable angina, and 45 patients(52%) had three vessel disease. Patients with low LV ejection fraction(<35%) were 7 cases and urgent or emergent operation were 10 cases. There were 6 cases of combined surgery which were mitral valve replacement(2 cases), aortic valve replacement(2 cases), ASD repair(1 case), and VSD repair(1 case). Average number of distal anastomosis was 3.5 per patient and average aortic cross clamp time was 115±38.3min. Preoperative risk factors were defined as follows: female, old age(>70 years), low body surface area(<1.5M2), PTCA or stent insertion history, hypercholesterolemia, smoking, hypertension, DM, COPD, urgent or emergent operation, left main disease, low LV ejection fraction(<35%), and combined surgery. Results: Operative mortality was 7cases(8%). As a postoperative morbidity, perioperative myocardial infarction was 6 cases, cerebrovascular accident 6 cases, reoperation for bleeding 5 cases, acute renal failure 4 cases, gastrointestinal complication 3 cases, and mediastinitis 3 cases. In the evaluation of operative risk factors, low body surface area, DM and low LV ejection fraction were found to be predictive risk factors of postoperative morbidity(p<0.05), and low ejection fraction was especially a risk factor of hospital mortality(p<0.05). Conclusions: In this study, low body surface area, DM and low LV ejection fraction were risk factors of postoperative morbidity and low ejection fraction was a risk factor of hospital mortality.

• Journal of Sasang Constitutional Medicine
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• v.8 no.1
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• pp.295-317
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• 1996

It is researched to elucidate the effects of Taeumjowuitang(TE,太陰調胃湯), Sibimikwanjungtang(SE, 十二味寬中湯) and Yangkeogsanwhatang(SY,凉膈散火湯) on the obesity induced by gold thioglucose and the differentiation and growth of preadipocyte 3T3-L1 in the mouse. The result were as follows: 1. TE,SE and SY extracts improved the blood level of transaminase in the obese mouse induced by gold thioglucose. 2. TE,SE and SY extracts inhibited the increase of liver fat and body fat in the obese mouse induced by gold thioglucose. 3. TE,SE and SY extracts inhibited the increase of body weight in the obese mouse induced by gold thioglucose. 4. TE,SE and SY extracts inhibited the growth of undifferentiate preadipocyte 3T3-L1. 5. TE,SE and SY extracts showed inhibitory effect on the differentiation of preadipocyte 3T3-L1. The above results suggest that the TE,SE and SY extracts may be used on the obesity induced by the overgrowth and differentiation of adipocyte, and the accumulation of fat in liver and body.