폐형성 부전이 동반된 Trisomy 22 1례

최명범,강기수,박찬후,우향옥,이정희,이재익,백원영,윤희상,Choi, Myoung-Bum,Kang, Ki Su,Park, Chan-Hoo,Woo, Hyang-Ok,Lee, Jeong-Hee,Lee, Jae-Ik,Paik, Won Young,Youn, Hee-Shang 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.6

We report a case of trisomy 22 in a liveborn male infant which was confirmed by fluorescence in situ hybridization(FISH), macrocultures and GTG-banding, and RHA-banding procedures of peripheral white blood cells. The infant showed lung hypoplasia, which is a unique presentation, with other clinical manifestations of previously reported cases of trisomy 22, such as intrauterine growth retardation, cleft palate, micrognathia, large atrial septal defect, limb anomalies, imperforate anus, and hypospadias. Our report gives weight to the previously reported observation that pulmonary hypoplasia may be associated in trisomy 22.

Helicobacter pylori에 의한 만성위염의 항균요법 후 위십이지장점막의 병리조직학적 변화

최명범(Myoung Bum Choi),김윤옥(Yoon Ok Kim),조윤경(Yoon Kyung Cho),신선경(Sun Kyeong Sin),김송자(Song Ja Kim),우향옥(Hyang Ok Woo),김미령(Mi Ryeung Kim),김승환(Seung Hwan Kim),윤희상(Hee Shang Youn),고경혁(Gyung Hyuck Ko),박철근(Cheo 대한소화기학회 1997 대한소화기학회지 Vol.29 No.1

Background; We examined the histopathological changes of the gastroduodenal mucosa after chemotherapy against Helicobacter pylori to evaluate what happened to them, because most of patients with chronic gastritis who received the triple chemotberapy felt improvement of upper gastrointestinal symptoms in spite of its 45% clearance rate of Helicobacter pylori in our previous study. Methods; Gastric and duodenal mucosal biopsy specimens were obtained before and 2-4 weeks after the triple chemotherapy. Helicobacter pylori colonization was evaluated with urease test and histopatho]ogical examinations including hematoxy]in-eosin and Warthin-Starry silver staining. Histopathological grading of gastric and duodenal mucosa was done according to the Sydney Syste and Whitehead's criteria, respectively. Resu)ts; Decreasing pathological grades of Helicobacrer pylori density, gastric neutrophil infiltration and chronic inflammatory cell infiltration was observed in 100%, 100% and 57.6% of 26 Helicobacter pylori-cleared patients after chemotherapy, respectively. And we a]so observed the decreasing pathological grades of Helicobacter pylori density, gastric neutrophil infiltration and chronic inflammatory cell infiltration in 70.8%, 63.6% and 29.4% of 34 Helicobacter pylori-persisted patients after chemotherapy, respectively. The histopathological changes of neutrophil and chronic inflamrnatory cell infiltration after chemotherapy were correlated with the histopathological changes of Helicobacter pylori density. Chronic inflammatory cell infiltration of duodenal mucosa was decreased only in 8.6% of patients after chemotherapy. Conclusions; After chemotherapy, the histopathological grades of Helicobacter pylori density, neutrophil infiltration and chronic inflammatory cell infiltration of gastric mucosa were decreased in both Helicobacter pylori-cleared and persisted groups. (Korean J Gastroenterol 1997;29:41 - 52)

적혈구 이형성증(Dyserythropoiesis)의 소견을 보이는 부분 21-monosomy 증후군

임재영,서지현,최명범,박찬후,우향옥,윤희상,Lim, Jae-Young,Seo, Ji-Hyoun,Choi, Myoung-Bum,Park, Chan-Hoo,Woo, Hyang-Ok,Youn, Hee-Shang 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.4

All complete monosomy 21 appear to be lethal early in their development in humans and only survive in mosaic forms. Complete monosomy 21 is a very rare and usually debilitating genetic disorder. Partial monosomy 21 is also rare and is thought to constitute a clinical syndrome consisting of peculiar faces, hypertonia, psychomotor retardation, and slow growth. We experienced a case of monosomy 21 mosaicism. Chromosome analysis demonstrated mosaicism for cell lines in the lymphocytes examined; 45, XX, -21/46, XX. The main clinical features were craniofacial dysmorphism including high arched palate, submucosal cleft, micrognathia and arthrogryposis-like symptoms including flexion deformity of fingers. And hematological findings were revealed dyserythropoiesis, thrombocytopenia and eosinophilia. Currently, the patient has nearly compatible growth, but a mild degree of mental retardation. We report here an 8 years old female child with apparent monosomy 21 mosaicism associated with dyserythropoiesis, thrombocytopenia and eosinophilia, with a review of the associated literatures.

고혈압과 뇌혈관염이 동반된 상태에서 뇌증을 보인 Henoch-Schonlein Purpura Nephritis 1례

최희라,김어진,최명범,임재영,박찬후,우향옥,윤희상,Choi, Hee Ra,Kim, Eo Jin,Choi, Myoung Bum,Lim, Jae Young,Park, Chan Hoo,Woo, Hyang Ok,Youn, Hee Sang 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.10

Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested as possible causes of the neurologic manifestation. Diagnosis of vasculitic involvement of CNS is difficult. Magnetic resonance imaging of the brain is the modality of choice for the evaluation of the CNS disease. Steroid or plasmapheresis are used in treatment of cerebral vasculitis. We experienced a case of 9-year-old boy who had presented with Henoch-Schonlein purpura nephritis complicating encephalopathy accompanied by hypertension and cerebral vasculitis. Brain MRI showed multiple small nodular-linear pattern enhancing lesions in whole cerebral hemispheres and focal increased T2 signal in the right basal ganglia. We used intravenous immunoglobulin in treatment of cerebral vasculitis. We report this case with a brief review of related literature.

건강한 소아에서 발생한 원발성 폐렴구균성 복막염 1례

양정수,이민혜,최명범,박찬후,우향옥,윤희상,Yang, Jeong-Soo,Lee, Min-Hae,Choi, Myoung-Bum,Park, Chan-Hoo,Woo, Hyang-Ok,Youn, Hee-Shang 대한소아소화기영양학회 2002 Pediatric gastroenterology, hepatology & nutrition Vol.5 No.1

Primary peritonitis usually refers to a bacterial infection of the peritoneal cavity without a demonstrable intra-abdominal source. Most cases occur in children with ascites resulting from nephrotic syndrome or cirrhosis. Rarely, it may occur in previously healthy children less than 7years of age, usually a girl. Distinguishing primary peritonitis from appendicitis may be impossible in patients without a history of nephrotic syndrome or cirrhosis. Accordingly, the diagnosis of primary peritonitis is made only at laparotomy. We report one case of primary pneumococcal peritonitis in a 27-month-old female who underwent explorative laparotomy to discover the cause of suspicious intestinal perforation and mechanical ileus. Later, pneumococci were cultured in blood and gram-positive diplococci were isolated from the pus of peritoneal cavity.

상부 위장관 증세가 있는 소아의 위십이지장병변 및 Helicobacter pylori 감염

윤영란,김미령,임재영,최명범,박찬후,우향옥,윤희상,고경혁,강형련,백승철,이우곤,조명제,이광호,Yoon, Young-Ran,Kim, Mi-Ryeung,Lim, Jae-Young,Choi, Myoung-Bum,Park, Chan-Hoo,Woo, Hyang-Ok,Youn, Hee-Shang,Ko, Gyung-Hyuck,Kang, Hyung-Lyun,Baik, S 대한소아소화기영양학회 2003 Pediatric gastroenterology, hepatology & nutrition Vol.6 No.2

Purpose: This study was undertaken to evaluate the gastroduodenal pathology and Helicobacter pylori infection in children with upper gastrointestinal symptoms. Methods: One hundred and seven pediatric patients with upper gastrointestinal symptoms were undergone endoscopy at the Gyeongsang National University Hospital from June 1990 to April 1991. Histopathologic examination was done by H & E staining of gastric antral biopsy specimen and gastritis was defined according to the Sydney System. Tissue H. pylori status was evaluated with the urease test using Christensen's urea broth and H & E or Warthin-Starry silver staining of gastric antral biopsy specimen. IgG Immunoblotting were also performed to detect specific anti-H. pylori antibody in these patients. Results: The reasons for endoscopy were recurrent abdominal pain, acute abdominal pain, sallow face, hunger pain, and frequent nausea. Variable degrees of gastric mucosal hyperemia were found in most of the patients. Gastric hemorrhagic spots, gastric ulcer, duodenal ulcer, duodenal erosion, and hemorrhagic duodenitis were rare endoscopic findings. Histologic chronic gastritis was found in 88% of 107 patients. Histologic chronic duodenitis was observed in all 99 patients whose tissue were available. Gastric tissue H. pylori was positive in 57% of 107 patients by one of the ureasetest, H & E staining and Warthin-Starry silver staining. However, gastric tissue H. pylori detection rate was lower in the younger age groups. Anti-H. pylori IgG antibodies were detectable in 96% of 107 patients. Conclusion: Chronic gastroduodenitis and anti-H. pylori IgG antibody were ubiquitous in children with upper gastrointestinal symptoms.

경남 지역 가와사끼병의 역학적 조사

양정수,조은영,정혜성,황지영,이동진,노은석,최명범,박찬후,윤희상,우향옥,Yang, Jeong Soo,Cho, Eun Young,Jung, Hae Sung,Hwang, Ji Young,Lee, Dong-Jin,No, Eun Suk,Choi, Myoung-Bum,Park, Chan-Hoo,Youn, Hee-Shang,Woo, Hyang-Ok 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.7

Purpose : The aim of this study was to determine the epidemiology of Kawasaki disease in the Kyung Nam area and to evaluate whether the results of this epidemiological study could support infectious etiology. Methods : We sent a questionnaire to three training hospitals in the Kyung Nam area and retrospectively reviewed their medical records of Kawasaki disease from Jun. 1995 to Dec. 1999. Results : The total number of patients was 717 cases, with little differences of annual prevalence during the five years. In all cases, the monthly prevalence of Kawasaki disease was high in Apr. and Jul. At the eastern of Kyung Nam, the monthly prevalence was high in Apr. and Jul. in 1995 and 1996, Jul. in 1997, Apr. in 1998 and Apr. and Jul. in 1999. In the central area of Kyung Nam, the monthly prevalence was high in Apr. in 1995 and 1996, Apr. and Jul. in 1997 and Jul. in 1998 and 1999. In the western Kyung Nam, the monthly prevalence was high in Nov. in 1995, Aug. in 1996, Oct. in 1997, Dec. in 1998 and Nov. in 1999. Conclusion : In the eastern and central areas of Kyung Nam, the monthly prevalence of Kawasaki disease was similarly high in Apr. and Jul. However, in the western district, the prevalence was high in late fall and winter. We could not prove the hypothesis that Kawasaki disease occurred with the spread of single infectious agent, but the a nnually similar prevalence in eastern and central Kyung Nam supported the infection theory for the etiology of the disease.

Pre-embedding Immunoelectron Microscopy를 이용한 Helicobacter pylori의 발견 - Immunoblotting법과 비교 -

정혜성,김은정,김은아,박지회,전진수,서지현,임재영,최명범,우향옥,윤희상,고경혁,백승철,이우곤,조명제,이광호,Jung, Hae-Seoung,Kim, Eun-Jung,Kim, Eun-A,Park, Ji-Hoe,Jun, Jin-Su,Seo, Ji-Hyoun,Lim, Jae-Young,Choi, Myoung-Bum,Woo, Hyang-Ok,Youn, Hee-Sha 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.7

Purpose : We tried to evaluate whether the detection rate of Helicobacter pylori in gastric biopsy specimens could be improved by using pre-embedding immunoelectron microscopy. Methods : A total of 119 children who complained of upper gastrointestinal symptoms were endoscoped at the Gyeongsang National University Hospital from July, 1996 to July, 1999. Five biopsy specimens(three for urease test, one for hematoxylin-eosin(H & E) staining, and one for preembedding immunoelectron microscopy) were obtained from each antrum and body. Immunoblotting analysis were also performed. Results : Among the 119 patients, H. pylori were found in 116 patients(97.5%) by the immunoelectron microscopy. Among three patients who were found H. pylori negative in immunoelectron microscopy, two patients showed H. pylori in H & E stained slides and one patient was urease test positive(color change within six hours). Urease tests were positive in 107 patients(89.9 %). The positive rate of immunoblotting tests was 81.5%. However, only 13 patients(10.9%) showed H. pylori on the H & E stained antrum or body tissue. Conclusion : In this study, we found H. pylori histopathologically in most of the pediatric patients who complained of upper gastrointestinal symptoms. This study showed that pre-embedding immunoelectron microscopic examinations can be used as a gold standard in the diagnosis of childhood H. pylori infection. However, this method also has limited capacity to detect widely scattered H. pylori compared to the other histopathologic diagnostic methods.

뇌교량 형성 부전 및 심기형을 동반한 ARCI 증후군 1례

김어진,윤영란,이민혜,강기수,임재영,최명범,박찬후,우향옥,윤희상,Kim, Eo-Jin,Yoon, Young-Ran,Lee, Min-Hae,Kang, Ki-Su,Lim, Jae-Young,Choi, Myoung-Bum,Park, Chan-Hoo,Woo, Hyang-Ok,Youn, Hee-Shang 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.8

ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized due to pneumonia at four months of age. Corpus callosum hypoplasia and atrial septal defect were detected. The case was treated with a mechanical ventilator because pneumonia was aggravated and respiratory failure occurred. The patient expired at five months of age.

소아 식도 이물의 내시경적 적출방법 변화에 대한 고찰

김점수,양정수,정혜성,이민혜,박찬후,최명범,우향옥,윤희상,Kim, Jum Su,Yang, Jung Soo,Jung, Hae Sung,Lee, Min Hye,Park, Chan-Hoo,Choi, Myoung Bum,Woo, Hyang-Ok,Youn, Hee-Shang 대한소아청소년과학회 2002 Clinical and Experimental Pediatrics (CEP) Vol.45 No.4

Purpose : The aim of this study was to evaluate the latest tendency of esophageal foreign body's extraction and to obtain a consensus from recent trends of indications and techniques of flexible endoscopy of esophageal FB in children. Methods : We retrospectively reviewed medical records of 127 cases with foreign bodies in esophagus at Dept. of Pediatrics and Otorhinolaryngology, Gyeongsang National University Hospital (GNUH) from Jun, 1987 to July, 2001. They were divided into two groups by the kinds of endoscopy : flexible endoscope(66 cases) or rigid endoscope(61 cases). Rigid endoscopy was performed under general anesthesia at Dept. of Otorhinolaryngology but flexible endoscopy was performed without general anesthesia or sedative drugs(midazolam or diazepam). Results : An annual number of cases of two groups were similar from 1991 to 1998. But from 1999, flexible endoscopy was performed actively. Asymptomatic cases were frequently observed in flexible endoscopy(28 cases/66 cases) but swallowing difficulties were frequently observed in the rigid endoscopy group(25 cases/61 cases). Other symptoms were vomiting, irritability, chest discomfort and abdominal pain. The total number of cases with underlying disease(esophageal stenosis, cerebral palsy) was 8. The total number of cases with complications (erosion, ulcer, bleeding, perforation) was 11. The above cases were not correlated between the two groups. In 55 cases(83.3%) of the flexible endoscopic group and 53 cases(86.8%) of the rigid endoscopic group, foreign bodies in the esophagus were removed within 24 hours. Conclusion : We could not find any benefit in rigid endoscopic technique. Flexible endoscopic FB removal can be performed safely and effectively in children by an experienced endoscopist.