• Title/Summary/Keyword: 이형성증

Search Result 200, Processing Time 0.027 seconds

Optimal Management of Patent Ductus Arterisus in Premature (미숙아 동맥관개존증의 효과적 치료)

  • 전태국;박표원
    • Journal of Chest Surgery
    • /
    • v.30 no.6
    • /
    • pp.585-590
    • /
    • 1997
  • Between December 1994 and October 1996, 57 premature infants with evidence of a hemodynamically significant PDA associated with cardiopulmonary compromise underwent indomethcin therapy(Group I, n=48) or surgical ligation(Group II, n=9) because of indomethacin failure. The gestational alee(29.6$\pm$ 3.1weeks vs. 28.1 $\pm$ 1.6weeks) and birth weight(1,413 $\pm$ 580gm, ,098 $\pm$ 235gm) showed no significant differences between the two groups. Medical management included fluid restriction, diuretics, and indomethacin therapy(one or two cycles). Surgical libation was done at the neonatal intensive care unit(NICU) without moving the patient to the operation room. There was no complication associated with the operation. There were 9 deaths in Group I(19%, 9/48) and 2 deaths in Group II(22% , 219). The main causes of deaths were persistent bronchopulmonary dysplasia with sepsis(n=8) and intrapulmonary hemorrhage(n=3). The rate of medical treatment failure including death and complication in premature infants whose body weights were less than 1500gm was higher(41%, 15/38) than in premature infants whose body weights were more than 1500gm(16%, 3/19). Early surgical ligation of PDA may be applicable in the premature infant with a large size, low birth weight(<1500 gm), or associated intracardiac anomalies. Perfoming the operation in the NICU may be safe in s ead of moving the patient to the operating room.

  • PDF

Engraftment of Intraperitoneally Injected Bone Marrow Cells to Newborn Mice Injected with an Angiogenesis Inhibitor (혈관생성 억제제를 주사한 마우스 모델에서의 골수 세포의 복강 내 주입 후 생착)

  • Cho, Su-Jin;Ju, Sun-Young;Woo, So-Youn;Kang, Hyoung-Jin;Ahn, Hyo-Seop;Ryu, Kyung-Ha;Park, Eun-Ae
    • Neonatal Medicine
    • /
    • v.15 no.1
    • /
    • pp.22-31
    • /
    • 2008
  • Purpose : Bronchopulmonary dysplasia (BPD) is characterized by arrested vascular and alveolar growth in the premature lung. Considering the consequences of arrested lung growth, the idea of administering bone marrow cells to enhance the inborn repair mechanism is promising as this may reduce the morbidity and mortality of BPD. We followed enhanced green fluorescent protein (EGFP)-labeled bone marrow cells (BMC) injected intraperitoneally into non-EGFP mice in order to determine their fate after transplantation. Methods : An angiogenesis inhibitor, SU1498, was injected subcutaneously on day 3 in non-EGFP C57BL/6 newborn mice to create a model of arrested alveolar development. On the following day, $1{\times}10^6$ BMCs isolated from major histocompatibility complex (MHC)- matched syngenic EGFP mice were injected intraperitoneally to non-EGFP BPD mice. Morphometric analysis, immunostaining, and confocal microscopy were performed to determine the fate of EGFP-positive stem cells in the injured lung. Results : SU1498 injection reduced alveolar surface area and mean alveolar volume in newborn mice. BMC injection resulted in recovery of lung structure comparable to controls. EGFP-positive BMCs were identified in the lungs of the recipient mice after intraperitoneal injection. The injected EGFP cells were co-stained with endothelial and epithelial cells of the developing lung as determined by confocal microscopy. Conclusion : Our results illustrated that EGFP-positive BMCs engrafted and trans-differentiated into epithelial and endothelial cells after intraperitoneal injection in a mouse model of arrested alveolar development.

The Usefulness of Capsule Endoscopy in Diagnosis of Small Bowel Diseases (소장질환의 진단에 캡슐내시경 검사의 유용성)

  • Eun, Jong-Ryul;Jang, Byung-Ik
    • Journal of Yeungnam Medical Science
    • /
    • v.23 no.1
    • /
    • pp.45-51
    • /
    • 2006
  • Background: This study was conducted to evaluate the usefulness of capsule endoscopy (CE) for the diagnosis of small bowel diseases. Materials and Methods: We reviewed the medical records of 66 patients (mean age: 52.1 years, male/female: 39/27), who underwent CE at Yeungnam University Hospital from August 2003 to March 2006. Results: Suspicious gastrointestinal (GI) bleeding presenting as anemia or history of gross bleeding was the most common reason to perform CE (71.2%). Other indications included GI symptoms (21.2%) such as abdominal pain/discomfort, nausea, diarrhea, and others (7.6%). In studies performed for GI bleeding (n=47), ulcer/erosion was the most common finding (n=22, 46.8%) followed by tumor (n=5, 10.6%), angiodysplasia (n=3, 6.4%), polyp (n=3, 6.4%), active bleeding (n=1, 2.1 %), ulcer with stenosis (n=1, 2.1%), and normal findings (n=12, 25.5%). Of these, a bleeding focus was detected in 32 cases (68.1%) undergoing CE studies. Among 14 patients with GI symptoms, only two patients had typical findings related with symptoms. Surgical resection was performed in five cases with tumor. Of these, four were diagnosed as gastrointestinal stromal tumor and the other one was a lymphangioma. There were no complications associated with the CE procedure. Conclusion: Capsule endoscopy is a safe, noninvasive diagnostic tool for small bowel diseases and may be useful for the diagnosis of small bowel hemorrhage including obscure bleeding. However, further studies are needed to confirm its utility for abdominal symptoms other than hemorrhage because of the low diagnostic yield.

  • PDF

Assessment of Risk Factors for Developmental Defects of the Enamel in Preterm (조산아의 발육성 법랑질 결함의 위험 요인 평가)

  • Shang-yon Park;Jaeho Lee;Hyung-Jun Choi;Chung-Min Kang
    • Journal of the korean academy of Pediatric Dentistry
    • /
    • v.50 no.2
    • /
    • pp.192-204
    • /
    • 2023
  • This study aimed to determine the criteria for quantifying developmental defects of enamel in primary teeth in premature babies and to investigate the severity of developmental defects according to the gestational age, birth weight, systemic complications, and treatments received after preterm birth. Birth information, a history of complications, the duration of parenteral nutrition, and endotracheal intubation were investigated by retrospectively reviewing the admission and discharge records of premature babies in the neonatal intensive care unit. The Preterm Developmental Defects of Enamel (PDDE) index was designed by modifying the existing developmental defects of enamel index. Based on PDDE index, the evaluator scored developmental defects of enamel by classifying them as enamel hypomineralization and hypoplasia. The PDDE scores in the extremely preterm and extremely low birth weight groups were significantly higher than those in other groups. Furthermore, PDDE scores of premature babies with bronchopulmonary dysplasia, rickets, intraventricular hemorrhage, or necrotizing colitis were significantly higher than those in the control group. In addition, more than 50 days of endotracheal intubation and more than 20 days of parenteral nutrition were associated with significantly higher PDDE scores than those in the control group and were risk factors for developmental defects of enamel. This study provides basic information for identifying risk factors for developmental defects of enamel in premature babies.

The factors associated with the efficacy of indomethacin treatment in premature infants with patent ductus arteriosus (인도메타신 투여 시 미숙아 동맥관개존 치료효과에 영향을 미치는 인자)

  • Yoon, Min Jeong;Yoon, Hye Sun;Chung, Sung Hoon;Han, Mi Young;Bae, Chong Woo
    • Clinical and Experimental Pediatrics
    • /
    • v.50 no.6
    • /
    • pp.531-535
    • /
    • 2007
  • Purpose : Indomethacin treatment is successful in about 90% of patent ductus arteriosus (PDA) in premature infants, but in some, repeated administration or surgical closure is required. The object of the present study is to determine the factors affecting the efficacy of indomethacin treatment and to predict the treatment result. Method : The 29 preterm neonates, admitted to neonatal intensive care unit of Kyunghee university medical center and Eulji university hospital between September 2002 and April 2006 were diagnosed of PDA and treated with indomethacin. The risk factors that might affect the efficacy of treatment were studied retrospectively. Results : The single-administered group was 19 patients out of 29 (65.5%) and among the repeated-administered group, 5 patients (17.2%) had repeated indomethacin administration and the other 5 patients (17.2%) underwent surgery due to reopening of the duct after repeated medical treatment. In repeated-administered group, the diameter of PDA was significantly larger ($3.66{\pm}0.8mm$ vs $2.55{\pm}0.8mm$, P<0.01), especially when larger than 3.5 mm (sensitivity: 70%, specificity: 89%). Also, the mean postnatal age of the first indomethacin administration was significantly delayed in repeated-administered group (19.7 days vs 12.5 days, P<0.05). There were no significant differences in gestational age (32 wk 5 days vs 30 wk 8 days) and in birth weight (1598.9 g vs 1750.5 g). There were no significant differences in associated morbidities. Conclusion : In patients with larger diameter of PDA (>3.5 mm) and older postnatal age (>7 days), the effect of indomethacin was decreased. Therefore in such cases, repeated dose of indomethacin or surgical ligation should be considered earlier.

Characterization of B Cells of Lymph Nodes and Peripheral Blood in a Patient with Hyper IgM Syndrome (Hyper IgM Syndrome 환자에서 얻은 림프절 및 말초혈액 B세포의 특성)

  • Kim, Dong Soo;Shin, Kyuong Mi;Yang, Woo Ick;Shin, Jeon-Soo;Song, Chang Hwa;Jo, Eun Kyeong
    • Clinical and Experimental Pediatrics
    • /
    • v.46 no.2
    • /
    • pp.128-136
    • /
    • 2003
  • Purpose : Hyper IgM syndrome(HIGM) is characterized by severe recurrent bacterial infections with decreased serum levels of IgG, IgA, and IgE but elevated IgM levels. Recently, it has been classified into three groups; HIGM1, HIGM2 and a rare form of HIGM. HIGM1 is a X-linked form of HIGM and has now been identified as a T-cell deficiency in which mutations occur in the gene that encodes the CD40 ligand molecule. HIGM2 is an autosomal recessive form of HIGM. Molecular studies have shown that the mutation of HIGM2 is in the gene that encodes activation-induced cytidine deaminase(AID). Recently, another rare form of X-linked HIGM syndrome associated with hypohydrotic ectodermal dysplasia has been identified. We encountered a patient with a varient form of HIGM2. To clarify the cause of this form of HIGM, we evaluated the peripheral B cells of this patient. Methods : The lymphocytes of the patient were prepared from peripheral blood. B cells were immortalized with the infection of EBV. Cell cycle analysis was done with the immortalized B cells of the patient. Peripheral mononuclear cells were stained with monoclonal anti-CD40L antibody. Total RNA was extracted from the peripheral mononuclear cells. After RT-PCR, direct sequencing for CD40L gene and HuAID gene were done. Immunostainings of a lymph node for CD3, CD23, CD40, Fas-L, bcl-2, BAX were done. Results : The peripheral B cells of this patient showed normal expression of CD40L molecule and normal sequencing of CD40L gene, and also normal sequencing of AID gene. Interestingly, the peripheral B cells of this patient showed a decreased population of G2/mitosis phase in cell cycles which recovered to normal with the stimulation of IL-4. Conclusion : We suspect that the cause of increased serum IgM in this patient may be from a decrease of G2/mitosis phase of the peripheral B cells, which may be from the decreased production or secretion of IL-4. Therefore, this may be a new form of HIGM.

Cortical Dysplasia: Tc-99m ECD SPECT Findings and Comparative Study with MRI according to Pathologic Grading (뇌피질 이형성증: Tc-99m ECD SPECT 소견과 병리적 등급에 따른 MRI와 비교 연구)

  • Park, Soon-Ah;Lim, Seok-Tae;Sohn, Myung-Hee;Chung, Gyung-Ho
    • The Korean Journal of Nuclear Medicine
    • /
    • v.35 no.1
    • /
    • pp.23-32
    • /
    • 2001
  • Purpose: Cortical dysplasia (CD) designates a diverse group of malformations resulting from one or more abnormalities in the development of the cerebral cortex. We investigated the findings of interictal SPECT and the diagnostic usefulness of interical and ictal SFECT according to pathological grading (PG) in comparison with MRI. Materials and Methods: This study included 16 patients (M:F=9:7, age: $19.9{\pm}11.8$ yrs) with pathologically proven CD. Tc-99m ECD SPECT was performed in all patients: interictal 11, interictal and ictal 3, ictal 2. MRI were obtained in all patients and image analysis was done blindly as to the result of SPECT. Pathologic findings of CD were classified into grade 1 G1, dyslamination), grade 2 (G2, dysplastic neurons) and grade 3 (G3, balloon cells). We compared SFECT with MRI in lesions-to-lesions and analyzed the result according to PG. Results: In SFECT and MRI. 38 and 27 lesions were visually recognized. In 14 interictal SPECT, variable findings in 35 lesions were demonstrated: 25 were hypoperfusion, 7 hyperperfusion, 2 heterotopic perfusion in the white matter. By comparison between two studios, missed lesions were founded: SPECT were 1 lesion, MRI 12. Review of missed 12 lesions of MRI were followed according to PG: G1 patients were 16.7% (4/19), G2 40.0% (6/15), and G3 50% (2/4). Conclusion: Interictal SFECT in CD showed variable findings such as hypoperfusion, hyperperfusion or heterotopic perfusion. However, for detection of missed CD on MRI, SFECT may help to detect a functional abnormality of the lesion with high PG.

  • PDF

Early Result of Hydroxyapatite in Bone Defect after Operative Treatment of Benign Bone Tumor (양성 골종양의 수술적 치료 후 발생한 골결손에서 시행한 Hydroxyapatite의 조기 결과)

  • Chung, So-Hak;Kwon, Young-Ho;Park, Young-Gyun;Kim, Jae-Do
    • The Journal of the Korean bone and joint tumor society
    • /
    • v.14 no.1
    • /
    • pp.25-32
    • /
    • 2008
  • Purpose: This study was performed to evaluate the efficiency of Hydroxyapatite ($Bongros^{(R)}$-HA) what use for bone defect after operative treatment of benign bone tumor. Materials and Methods: Hydroxyapatite, was used to treat bone defect after operative treatment of benign bone tumor from September, 2006 to December, 2007. A total of 17 benign bone tumor cases (10 males and 7 females) with mean age of 28.5 was observed studied. The diagnoses were fibrous dysplasia in 8 cases, solitary bone cyst in 5 cases, and giant cell tumor in 4 cases. In categorization by location of tumor, there were 6 cases of proximal femur, 3 cases of proximal femur, 3 cases of proximal tibia, 4 cases of proximal humerus, and 1 case of hip bone. Autogenous bone was used with Hydroxyapatite in 4 cases, and only Hydroxyapatite used in 13 cases. Periods of Follow-up were from 3 months to 15 months, and mean period were 7.5 months. Amount of graft resorption and bone formation was observed with compare of post operation radiograph and the difference was shown by percentage. Results: More than 98% bone uptake was observed after mean 4.5 months, and more than 98% bone formation was observed after mean 6.2 months. Lesser bone defect size showed faster bone formation and it was statistically significant result (P=0.012). But other comparative studies on other factors such as sex, age of patients and combination of autogenous bone were no statistically significant differences in graft resorption and bone formation. And there was no significant complications in periods of follow-up. Conclusion: Hydroxyapatite is considered as one of useful method of bone defect after operative treatment of benign bone tumor.

  • PDF

Review of Primary Chest wall Tumors (원발성 흉벽종양의 임상적 고찰)

  • Sohn, Sang-Tae;Chon, Soon-Ho;Shin, Sung-Ho;Kim, Hyuck;Chung, Won-Sang;Kim, Young-Hak;Kang, Chung-Ho;Park, Moon-Hang;Jee, Heng-Ok
    • Journal of Chest Surgery
    • /
    • v.31 no.10
    • /
    • pp.988-994
    • /
    • 1998
  • Background: Chest wall tumors can classified into soft tissue tumors and bone tissue tumors and can be subclassified into benign and malignant tumors. Materials and methods: We report an analysis of 68 patients with primary chest wall tumors treated at the department of thoracic and cardiovascular surgery at Hanyang University Hospital from January, 1973 to September 1997. Results: Among a total of 68 patients 33(48.5%) were males and 35(51.5%) were females. The ages of the patients ranged from 10 to 79 years with a mean age of 39.3 years. According to the age distribution, 23 patients (33.8%) were from the 4th decade, 12 patients(17.6%) were from the 6th decade, and 10 patients(14.7%) were from the 5th decade. Among the primary chest wall tumors, 53 cases were benign and 15 cases were malignant. Among the benign tumors, 17 cases(32.1%) were in the 4th decade and among the malignant tumors, 6 cases(40%) were in the 4th decade. In both malignant and benign tumors the most common ages were in the 4th decade. The most common tumors were fibrous dysplasia and chondroma, each with a total of 14 cases(26.4%). Osteochondroma and lipoma each had 8 cases(15.1%). Among malignant tumors, osteosarcoma was most common with 8 cases (53.3%). According to location, 49 cases occured in both bone and cartilage tissue, 19 cases occurred in cartilage. Among the presenting symptoms, palpable mass was present in all cases. Fifty-one patients complained of tenderness and among cases with involvement of the lung, 3 patients had complained of respiratory distress. Among the malignant tumors 6 cases underwent a radical operation and 4 cases of benign tumors underwent a radical operation. Postoperativly, there was one case with recurrence from a desmoid tumor. There were no deaths postoperativly and no deaths due to complications(and their postoperative courses were uneventful). Conclusions: Most patients with primary chest wall tumors initially present with mass at admission. Resection is sufficient treatment for benign tumors but in malignant tumors wide resection of the chest wall is needed and mchest wall reconstruction.

  • PDF

Treatment of Benign Bone Lesions with Autologous Bone Marrow Stromal Cell Transplantation (자가 골수 기질 세포 이식을 이용한 장관골 양성 골 병변의 치료)

  • Rhee, Seung-Koo;Kang, Yong-Koo;Kim, Yong-Sik;Bahk, Won-Jong;Chung, Yang-Guk;Kim, Hyoung-Jun;Ok, Ji-Hoon
    • The Journal of the Korean bone and joint tumor society
    • /
    • v.10 no.1
    • /
    • pp.13-21
    • /
    • 2004
  • Purpose: We analyzed the result of autologous bone marrow stromal cell transplantation with or without cancellous chip bone allograft for benign long bone lesions. Materials and methods: Since July 1996, eight benign bone lesions treated by curettage, cancellous chip bone allograft and bone marrow or marrow stromal cell transplantation were observed for resolution of clinical symptoms, new bone formation and consolidation. There were 6 males and 2 females. Average age was 24 (range 8 to 47) years old. Histologic diagnoses were 5 fibrous dysplasia, 2 simple bone cysts and one chondroblastoma and fibrous cortical defect each. Mean follow-up period was 16.3 (range 3 to 84) months. Results: In all four symptomatic patients, the pain was subsided in two weeks after surgery. New bone formation in the lesion was observed at 4 weeks, which incorporated into surrounding normal bone around 8 weeks. There were one pathologic fracture through the lesion at 3 weeks and one recurrence of simple bone cyst at 5 months postoperatively. Conclusion: Bone marrow or marrow stromal cell transplantation for bone defects from curettage of benign bone lesions, with or without cancellous chip bone allograft revealed rapid healing. Though it was the result of short-term follow up, it supports that bone marrow stromal cell transplantation will be very useful for the treatment of benign long bone cysts or other lesions. The complete curettage of inner cystic wall is important to prevent later recurrence, and the rigid internal fixation is also needed in selected high risk lesions of fracture.

  • PDF