• Clinical and Experimental Pediatrics
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• 제48권1호
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• pp.93-96
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• 2005

저자들은 출생 시부터 전신에 분포하는 밀크 커피색 반점이 있고, 액와부에 작은 주근깨를 보이며 정신 지체와 발달 지연등 제1형 신경섬유종증의 소견을 보이는 환아에서 급성 신경학적 쇠약 증세가 있어 시행한 뇌자기공명영상 촬영과 뇌동맥조영 촬영상 모야모야병의 소견이 동반된 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제57권7호
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• pp.323-328
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• 2014

Purpose: Adenoid hypertrophy is a physical alteration that may affect speech, and a speech disorder can have other negative effects on a child's life. Airway obstruction leads to constricted oral breathing and causes postural alterations of several oro-facial structures, including the mouth, tongue, and hyoid bone. The postural modifications may affect several aspects of speech production. Methods: In this study, we compared articulation errors in 19 children with adenoid hypertrophy (subject group) to those of 33 children with functional articulation disorders independent of anatomical problems (control group). Results: The mean age of the subject group was significantly higher (P=0.016). Substitution was more frequent in the subject group (P=0.003; odds ratio [OR], 1.80; 95% confidence interval [CI], 1.23- 2.62), while omission was less frequent (P<0.001; OR, 0.43; 95% CI, 0.27-0.67). Articulation errors were significantly less frequent in the palatal affricative in the subject group (P=0.047; OR, 0.25; 95% CI, 0.07-0.92). The number of articulation errors in other consonants was not different between the two groups. Nasalization and aspiration were significantly more frequent in the subject group (P=0.007 and 0.014; OR, 14.77 and 0.014; 95% CI, [1.62-135.04] and NA, respectively). Otherwise, there were no differences between the two groups. Conclusion: We identified the characteristics of articulation errors in children with adenoid hypertrophy, but our data did not show the relationship between adenoid hypertrophy and oral motor function that has been observed in previous studies. The association between adenoid hypertrophy and oral motor function remains doubtful.

• Clinical and Experimental Pediatrics
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• 제57권7호
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• pp.310-316
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• 2014

Purpose: Prader-Willi syndrome (PWS) is a complex genetic disorder that results from the lack of paternally expressed genes in the chromosome 15q11-q13 region. This study was performed to delineate the clinical features of PWS infants and toddlers and the effects of two-year growth hormone (GH) treatment according to gender and age at the start of treatment. Methods: The clinical characteristics and the results of the GH treatment were reviewed retrospectively for 30 PWS patients diagnosed by molecular genetic testing and clinical manifestations. Results: The mean age at diagnosis with PWS was 13.7 months (2-47 months of age). All patients showed the characteristics of facial dysmorphism, including brown hair and almond-shaped eyes. Most patients showed developmental delays/mental retardation (93.3%), cryptorchidism (75%), feeding problems in infancy (73.3%), and neonatal or infantile hypotonia (66.7%). Among 30 patients, 14 PWS infants and toddlers had been treated with GH for more than two years. Two years of GH treatment resulted in an improvement in head circumference-standard deviation score (HC-SDS), body weight-SDS, insulin-like growth factor-1 (IGF-1) SDS, IGF binding protein-3 (IGFBP-3) SDS, lean body mass, and bone mineral content, especially in IGFBP-3 SDS and motor development in PWS patients younger than two years of age. There was significant increase in IGF-1 SDS and IGFBP-3 SDS among male PWS patients after GH treatment. Conclusion: Our study showed increases in IGFBP-3 SDS and an improvement in motor development among individuals under two years of age after GH treatment, and significant difference in IGF-1 SDS and IGFBP-3 SDS by gender.

• Journal of Chest Surgery
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• 제42권2호
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• pp.272-274
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• 2009

칼돌기 부위에 고통을 느끼는 칼돌기 통증은 흔하지 않은 근골격계 질환으로 전흉부와 상복부, 목, 양측 어깨, 등으로 통증이 방사될 수 있다. 진단에서 중요한 것은 이러한 통증이 칼돌기의 이상에 의해서 발생할 수 있다는 것을 임상적으로 알고 있어야 한다는 것이다. 2 증례의 칼돌기 통증 환자를 경험하였는데, 칼돌기 부위를 가볍게 누르면 전흉부와 상복부의 통증이 반복적으로 재생산될 때 진단하였다. 수년간 칼돌기 통증으로 고통 받아 왔던 첫번째 증례 환자에서는 칼돌기 제거수술을 하였고, 두번째 증례 환자에서는 국소마취제를 주사하여 치료하였다.

• 대한세포병리학회지
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• 제7권1호
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• pp.79-83
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• 1996

Gaucher's disease is an autosomal recessive disorder resulting from mutation at the glucocerebrosidase locus on chromosome 1q21. As a result, glucocerebroside accumulates principally in the phagocytic cells known as Gaucher cells. In our case, a five-year old girl was admitted with seven days' history of fever and abdominal distension. At physical examination the patient had hepatosplenomegaly. Laboratory tests revealed a hemoglobin concentration of 2.8g/L: platelet counts of $23,000/{\mu}l$: normal range of white cell and differential counts, and negative Coombs' test. Liver enzymes were normal. For the evaluation of hepatosplenomegaly, fine needle aspiration was performed blindly against the palpable spleen. Wet-fixed hematoxylin and eosin-stained smears are made. The smears from the spleen showed predominantly macrophages with abundant cytoplasm and rather small, uniform, often eccentric nuclei with small nucleoli. The multinucleated cells were often found. The cytoplasm was pale, with more or less distinct fibrillarity. The cells had the characteristic appearance of Gaucher cells. Gaucher cells were also found in the tissue section from the liver, spleen and lymph node and the bone marrow aspirate. The diagnosis was later confirmed by determination of bela-glucosidase activity in peripheral blood leucocytes. Fine needle aspiration of the spleen is considered as a convenient procedure with a low complication rate for the diagnosis of lysosomal storage disease.

• Clinics in Shoulder and Elbow
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• 제14권2호
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• pp.242-247
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• 2011

목적: 골내 침범이 동반된 비전형적인 견관절 석회화 건염 1예를 보고하고자 한다. 대상 및 방법: 만성적인 좌측 견관절 동통의 급성 악화와 운동 제한을 주소로 내원한 59세 여자 환자로 단순 방사선 상 견봉하 석회 침착과 자기 공명 영상 상 감염이나 종양과 유사하게 골 피질을 침범하고 석회 물질이 골 내부로 연장되어 있었다. 관절경하 석회 제거술과 골내 석회의 소파술, 건 결손부에 봉합 나사못을 이용한 회전근 개 봉합술을 시행하였다. 결과: 환자의 급성 견관절 통증은 술 후 즉각적인 호전을 보였고 순조로운 재활 경과와 관절 운동범위의 회복을 보였다. 술 후 5개월에 촬영한 단순 방사선 상 석회 침착의 재발은 관찰되지 않았다. 결론: 견관절 석회화 건염은 주위 골조직으로 연장된 다양한 영상 소견을 나타 낼 수 있으며, 질환을 정확히 판별함으로써 불필요한 검사나 치료를 피할 수 있을 것으로 생각된다.

• Clinical and Experimental Pediatrics
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• 제46권6호
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• pp.606-609
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• 2003

저자들은 특징적인 진찰 소견과 피부 조직 생검에서 제 I형 교원질 유전자 발현의 감소를 보인 Goltz 증후군 1례를 경험하였기에 보고하는 바이다.

• Imaging Science in Dentistry
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• 제46권3호
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• pp.167-171
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• 2016

Purpose: Obstructive sleep apnea (OSA) is a common medical disorder with serious complications if untreated. Dentists play a vital role in the early diagnosis of this condition, thereby improving patients' prognoses. The purpose of this study was to identify patients with a high risk of OSA using simple cephalometric measurements in patients receiving routine dental care. Materials and Methods: The present study was conducted on 206 patients divided into a high-risk group and a control group after answering the Berlin questionnaire. Cephalometric analysis of a digital cephalogram was performed to measure the upper airway diameter (UAD) and mandibular-to-hyoid bone distance (MP-H) by 2 observers at 2 different times. Results: Among 206 patients, 93 (45%) were included in the high-risk group and 113 (55%) were in the control group. No significant difference was present between the groups with regard to gender, and the patients ranged in age from 18 to 65 years. The UAD measurements in the high-risk group were significantly lower than in the control group, and the MP-H measurements were significantly higher in the high-risk group than in the control group. The UAD was lower in middle-aged patients in both groups. Conclusion: Our study found that the UAD was lower in individuals with a high risk of OSA. Also, we found that middle-aged individuals of both genders were more likely to develop OSA. Dentists play a vital role in diagnosing patients at a high risk for OSA via thorough clinical examinations, risk factor analyses, and simple cephalometric analyses.

• Tuberculosis and Respiratory Diseases
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• 제42권1호
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• pp.110-114
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• 1995

저자등은 26세 남자환자에서 폐농양과 동반된 속발성 비대성 골관절병증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제18권4호
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• pp.734-740
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• 1996

본과에 내원하여 LCH로 진단된 13증례를 고찰해 보면, 10세 미만의 남아에서 호발되었으며, 주증상은 부종 및 동통이었고, 임상적으로 악성종양과의 감별이 중요하며, 국소적 소파 및 약물 치료로 대체로 좋은 예후를 보였다. 그러나, 이는 악골에서만의 경우를 보았을 때이며, LCH는 기본적으로 망상내피계가 존재하는 신체내 어느 곳에서나 생길 수 있으며, 실제 본과의 증례에서도 폐에서 LCH의 소견이 확인된 증례가 있었으며, 다른 증례의 환자에서도, 백내장, 결핵, 중이염 등의 병력이 있었던 바 이것이 LCH의 또다른 병소이였을 가능성을 배재할 수 없다. LCH는 비교적 좋은 예후를 기대할 수는 있으나, 좀더 장기간의 추적조사 및 전신적 검토, 관련 타과와의 협조가 필요하다.