• Clinical and Experimental Pediatrics
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• v.45 no.7
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• pp.828-835
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• 2002

Purpose : The outcomes of infants weighing less than 1,500 gm(very low birth weight infant : VLBWI) reflect recent progress in neonatal intensive care. In this study, we analyzed changes over time in survival rate and morbidity of VLBWIs during the past seven years. Methods : A retrospective review of medical records was analyzed for VLBWIs admitted to the neonatal intensive care unit of Samsung Medical Center within three days from birth. We compared the outcomes of previous corresponding data(period I : Oct. 1994 to Sept. 1996), with the outcomes of period II(Oct. 1996 to Dec. 1998) and period III(Jan. 1999 to Dec. 2000). Results : As shown in Tables 1 and 3, the distribution of birth weight, gestational age(GA), gender, and inborn admissions did not change during the 7-year study. The overall survival rate of VLBWI increased significantly over time(period I : 72% vs period III : 88.3%, P<0.05). Between period I and period II, the birth weight-specific survival rate increased by 23.6%(75% vs 92.7%, P<0.05) for infants 1,000 to 1,249 gm. Between period II and period III, the birth weight-specific survival rate increased three times(20% vs 66.7%, P<0.05) for infants <750 gm. The survivors of lowest birth weight included infants at 624 gm(GA : $26^{+5}$ weeks), 667 gm(GA : $25^{+6}$ weeks) and 480 gm(GA : $26^{+2}$ weeks) in each period. The gestational age-specific survival rate in period III increased significantly in GA 25-26 weeks and 29-30 weeks(vs period I & period II, P<0.05). The survivors of lowest gestational age included infants at GA 26 weeks(970 gm), GA $23^{+5}$ weeks(791 gm) and GA $24^{+1}$ weeks(740 gm) in each period. The incidence of severe IVH(grade III, IV) and the early death rate(< seven days) decreased in period III(vs period I; P<0.05), reflecting improvements in neonatal intensive care. Conclusion : The survival rate of VLBWI continues to increase, particularly for BW < 750 gm, GA < 26 weeks. This increase in survival is not associated by any increase in major morbidities.

• Journal of Oral Medicine and Pain
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• v.30 no.3
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• pp.301-317
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• 2005

The number of patients with tongue carcinoma is increasing rapidly among young individuals in many parts of the world. Oral carcinoma progresses from hyperplastic lesion through dysplasia to invasive carcinoma and the concept of "field cancerization" with molecular alteration has been suggested for oral cavity carcinogenesis. Significant improvement in treatment and prognosis will depend on more detailed understanding of the multi-step process leading to cancer development. To induce tongue carcinoma in rat by 4-NQO, each drinking water was made to 10 ppm, 25 ppm, 50 ppm and control (only D.W. without 4-NQO). Specimens were classified into 4 groups such as control, I (mild & moderate dysplasia), II (severe dysplasia and carcinoma in situ), III (carcinoma). The mRNA expressions of Bcl-2 family were evaluated by RT-PCR technique. For anti-apoptotic Bcl-2 family, mRNA expression of Bcl-w was down-regulated in all stages of tongue carcinogenesis model. However, mRNA expression of Bcl-2 was up-regulated. For pro-apoptotic Bcl-2 family, all members were down-regulated in all stages of tongue carcinogenesis model except for Bad mRNA in group III. In terms of BH3 only protein, mRNA expressions of Bok and Mcl-1 were down regulated in all stages of specimen, but Bmf in group II and BBC3 in group III were up-regulated. Our current findings demonstrated the involvements of mRNA expression of Bcl-2 family in multi-step tongue carcinogensis. This highlights the necessity for continued efforts to discover suitable biomakers (Bcl-2 family) for early diagnosis of the disease, and to understand its pathogenesis as a first step in improving methods of treatment. The discovery of these potential biomarkers and molecular targets for cancer diagnostics and therapeutics has the potential to significantly change the clinical approach and outcome of the disease.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.1
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• pp.7-13
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• 2014

Purpose: As well as patient survival, the restoration of postoperative function such as ambulation is important in limb salvage operations for treatment of malignant bone tumors involving the proximal femur. The authors analyzed clinical outcomes of limb salvage operations using tumor prostheses for metastatic or primary malignant bone tumors in the proximal femur. Materials and Methods: From February 2005 to January 2014, 20 cases (19 patients) with malignant bone tumor involving the proximal femur with pain or complicated pathologic fracture were treated with segmental resection and limb salvage operations with tumor prostheses. Mean age was 63.1 years (range 35-86). Fourteen patients were male and six ones were female. The mean follow-up period was 20 months (1-94 months). There were 15 cases of metastatic bone tumor, 4 cases of osteosarcoma, and 1 case of multiple myeloma. The primary tumors of the metastatic bone tumors included 4 lung cancers, 3 hepatocellular carcinomas, and 3 renal cell carcinomas. Other primary tumors were breast cancer, thyroid cancer, colon cancer, prostate cancer, and malignant spindle cell tumor, each in 1 case. Modular tumor prostheses were used in all cases; (Kotz's$^{(R)}$ Modular Tumor prosthesis (Howmedica, Rutherford, New Jersey) in 3 cases, MUTARS$^{(R)}$ proximal femur system (Implantcast, Munster, Germany) in 17 cases). Perioperative pain was assessed with Visual Analogue Scales (VAS). Postoperative functional outcome was assessed with Musculoskeletal Tumor Society (MSTS) grading system. Results: Out of 20 cases (19 patients), 11 cases (10 patients) survived at the last follow-up. Average postoperative survival of the 9 deceased patients was 10.1 months (1-38 months). VAS score improved from pre-operative average of 8.40 (5-10) to 1.35 (0-3) after operation. Average postoperative MSTS function score was 19.65 (65.50%, 7-28). The associated complications were 2 local recurrences, 3 hematomas, 3 infections, 2 scrotal swellings, and 1 dislocation. There was no case of periprosthetic fracture or loosening. Conclusion: Limb salvage operation with tumor prosthesis is an appropriate treatment for early pain reduction and functional restoration in malignant bone tumors in the proximal femur with pain an/or complicated pathologic fractures.

• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1047-1051
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• 2008

Purpose : This study aims to evaluate the efficacy and safety of sedatives for pediatric patients using noninvasive procedures. Methods : We performed a prospective study in 446 (aged 1 month-21 y) consecutive pediatric patients undergoing sedation to study noninvasive sedation techniques from February to August 2007. We reviewed demographic data, sedative drugs, dosage, complications, and successful rates of sedation according to the underlying diseases. Results : The overall successful rate of sedation was 435/446 (97.5%). The overall rate of successful sedation using chloral hydrate was 99.1% (420/424), and was 70.6% (12/17) and 60.0% (3/5) with ketamine and midazolam, respectively. Of the neurologic patients (n=172, aged 1 month to 21 years), 136 patients were sedated for EEGs, 5 patients for renal scans, and 31 patients for neuroimaging studies such as brain CT or MRI. All non-neurological patients (n=274, aged 1 month to 5 years) were diagnosed with urinary tract infection and sedated for renal scan. The overall success rate of sedation for this group was 99.6% (273/274). A total of 14 adverse events were observed (3.1%). Most adverse reactions were mild in severity and clinically insignificant. Conclusion : Using chloral hydrate alone has enough effect to sedate non-neurologic patients. However, neurologic patients in the severe course group, especially those suffering from intractable epilepsy, autism, or severe cerebral palsy, must be medicated with chloral hydrate 2 times at most; instead, injections of ketamine or midazolam in the early stage may result in a more promising outcome.

• Journal of Chest Surgery
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• v.39 no.5 s.262
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• pp.387-393
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• 2006

Background: Esophageal perforation is an uncommon problem, but it is associated with high mortality. We performed a retrospective review of patients with instrumental esophageal perforation to assess the outcome of current management techniques. Material and Method: We retrospectively analyzed all cases of instrumental esophageal perforation diagnosed at our hospital from January 1999 through to March 2005. The study group consisted of 12 patients (8 women and 4 men) with a mean age of 48.8 years (range, $21{\sim}83$ years). We reviewed the effects of the surgical or medical treatments in various conditions of patients, such as of various sites of perforation and time delayed after injury. Result: Perforations were due to diagnostic endoscopy (50.0%, 6/12), esophageal bougination for benign stricture (33.3%, 4/12), endoscopic port insertion (8.3%, 1/12), and tracheal intubation (8.3%, 1/12). The perforated sites were thoracic in 7 patients and cervical in 5. The treatment included resection and reconstruction (5 cases), incision and drainage (4 cases), medical treatment (2 cases), and closed thoracostomy drainage only (1 case). Post-operative complications of transient pneumonia and wound infection were developed in 1 patient respectively. Both occurred in two patients with diffuse mediastinal abscess formation. The overall mortality was 8.3% (1/12) in one old patient who was managed medically for cervical esophageal perforation. Conclusion: We concluded that surgical treatment for esophageal perforations was safe and effective whether diagnosed early or lately.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.259-264
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• 2003

Purpose : We have performed an analysis on patients who received Rastelli operation in our institute and reviewed their progress postoperatively. Various factors with suspected relationship to the outcome have been considered to help in future treatment and follow-up. Methods : We analyzed retrospectively 43 patients who either received Rastelli operation in Yonsei University Cardiovascular Center from March 1995 to April 1997 or who received post-procedural cardiac catheterization and follow-up echocardiography in the out-patient department after the procedure. Results : No statistically valid relationships were found between the age of the patient, their body weight, preoperative pulmonary arterial index and pressure, presence of pulmonary branchial stenosis and postoperative results. Cases with atrioventricular concordance showed lower age and body weight, and discordant cases exhibited lower ejection fraction 3 days postoperatively. Upon follow up, lower NYHA score was seen in patients with severe residual stenosis. In the group that received cardiac catheterization after the procedure, residual stenosis and right ventricular pressure measurement in echocardiography showed good correlation with the catheterization data. Conclusion : In cases where conduit insertions of the right ventricular outflow tract are required to achieve total correction in complex cardiac deformity, early operation does not seem to provide a clear risk to the patient. In patients with atrioventricular discordance, careful postoperative observation of the ventricular function seems to be needed. Also, echocardiography appears to be a sound method in follow-up of patients after the correctional procedure.

• The Journal of Dong Guk Oriental Medicine
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• v.6 no.2
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• pp.167-190
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• 1998

Nowadays there were two tendencies of studies about prognostic factors in stroke. One way was to define prognostic factors according to the radiological features. And the other way was to define according to the mental state, recognition, perception, motors, language, urinary&bowel incontinence etc.. The former could be objectively investigated, while the latter was difficult. The purpose of this study was to determine which variables would be predictors of stroke and which factors would be affect predictions most. The subjects of this study were 32 patients who were admitted to the Dept. of Internal Medicine, Dongguk Univ. College of Oriental Medicine whthin 48 hours from attack, Medical records were reviewed FIM, CNS, NIH stroke scale. We compared each sub-items of FIM, CNS, NIH stroke scale about mental state, recognition, perception, motors, language, urinary&bowel incontinence with MBI score at 4 weeks from admission. Also, we analyzed the correlations of sub-items and groups which devided into 5 according to independence of MBI score. And we found out the most influent factors with multiple regression analysis. The major results were as follows; 1. In mean of MBI score at 4 weeks of each groups devided low, middle, high score at mental state, recognition, perception, motors, language, urinary&bowel incontinence items, there were statistical differences in all items. 2. The mental state and lim ataxia sub-items had no significant correlations with groups divided according to independence of MBI score. All the other items were significantly correlated. 3. The most influent factors was recognition. The second was sensory and the third was bowel incontinence. 4. The most influent scales was FIM, and the second was CNS, and NlH had no statistical significancy.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.178-185
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• 2005

Purpose : The purpose of this study was to evaluate the outcome of children with juvenile myelomonocytic leukemia(JMML) treated with allogeneic hematopoietic stem cell transplantation(allo-HSCT). Methods : Eleven JMML patients aged 8-39 months underwent allo-HSCT. The sources of grafts were unrelated donors(n=7), HLA-matched siblings(n=3) and an HLA 1-antigen mismatched familial donor. All patients had received chemotherapy ${\pm}13$-cis-retinoic acid(CRA) before transplant, and CRA was used, posttransplant, in six patients. Results : Only three patients were in complete remission(CR) at the time of transplantation. Initial chimeric status revealed complete donor chimerism(CC) in five patients, mixed chimerism(MC) in five and autologous recovery(AR) in one. One patient with MC having persistent splenomegaly eventually turned to CC and CR after rapid tapering of cyclosporine, combined with daily use of CRA. An AR case relapsed shortly after transplant but was rescued with second, unrelated cord blood transplantation. Ultimately, six patients are alive, event-free, with a median follow-up of 15.5 months posttransplant. All three deaths occurred in patients who failed to achieve CC, leading to disease progression. Conclusion : We suggest that graft-versus-leukemia effect play an important role and CRA a possible role in posttransplant leukemic involution in JMML. In patients whose leukemic burden is still high with MC after transplant, early tapering of immunosuppressants and introduction of CRA might provide a chance of a cure for some patients.

• Korean Circulation Journal
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• v.54 no.4
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• pp.189-200
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• 2024

Background and Objectives: Concerns remain that early aspirin cessation may be associated with potential harm in subsets at high risk of ischemic events. This study aimed to assess the effects of P2Y12 inhibitor monotherapy after 3-month dual antiplatelet therapy (DAPT) vs. prolonged DAPT (12-month or longer) based on the ischemic risk stratification, the CHADS-P2A2RC, after percutaneous coronary intervention (PCI). Methods: This was a sub-study of the SMART-CHOICE trial. The effect of the randomized antiplatelet strategies was assessed across 3 CHADS-P2A2RC risk score categories. The primary outcome was a major adverse cardiac and cerebral event (MACCE), a composite of all-cause death, myocardial infarction, or stroke. Results: Up to 3 years, the high CHADS-P2A2RC risk score group had the highest incidence of MACCE (105 [12.1%], adjusted hazard ratio [HR], 2.927; 95% confidence interval [CI], 1.358-6.309; p=0.006) followed by moderate-risk (40 [1.4%], adjusted HR, 1.786; 95% CI, 0.868-3.674; p=0.115) and low-risk (9 [0.5%], reference). In secondary analyses, P2Y12 inhibitor monotherapy reduced the Bleeding Academic Research Consortium (BARC) types 2, 3, or 5 bleeding without increasing the risk of MACCE as compared with prolonged DAPT across the 3 CHADS-P2A2RC risk strata without significant interaction term (interaction p for MACCE=0.705 and interaction p for BARC types 2, 3, or 5 bleeding=0.055). Conclusions: The CHADS-P2A2RC risk score is valuable in discriminating high-ischemic-risk patients. Even in such patients with a high risk of ischemic events, P2Y12 inhibitor monotherapy was associated with a lower incidence of bleeding without increased risk of ischemic events compared with prolonged DAPT.