No abstract available.
Anemia, Iron-Deficiency* ; Erythrocyte Indices* ; Iron*

No abstract available.

No abstract available.
Heart Atria*

PURPOSE: Deletion of chromosome 22q11 is associated with DiGeorge syndrome, velocardiofacial syndrome, and conotruncal anomaly face syndrome. This study was performed to determine the criteria of clinical phenotype as recognizable syndrome and to research the loss of heterozygosity in CATCH 22 patients and their family. METHODS: An evaluation of the clinical and genetic profiles of 30 persons of CATCH 22 syndrome or their family referred with a diagnosis of either congenital heart disease or cleft palate was undertaken. The deletions of 22q11 were analyzed using the fluorescences in situ hybridization(N25, Oncor) and short tandem-repeat polymorphic makers(STRP, D22S941). RESULTS: The dysmorphic features of CATCH 22 showed considerable overlap and intrafamilial difference was common. The familial cases of CATCH 22 were transmitted maternally as autosomal dominant. The target gene study using the STRP maker(D22S941) in these series showed good clinico-genetic correlation but some heterogeneity. CONCLUSION: Although 22q11 deletion was large in size and high variable in polymorphic markers, extensive evaluation clinically as well as genetically will be necessary for subgrouping of CATCH 22 syndrome due to good clinicogenetic correlation. Furthermore, we also suggest the development of new polymorphic markers to research the unknown characteristics of polymorphic markers in Korean patients with CATCH 22 syndrome.
Cleft Palate ; Diagnosis ; DiGeorge Syndrome ; Heart Defects, Congenital ; Humans ; Loss of Heterozygosity ; Phenotype ; Population Characteristics

No abstract available.
Aconitum* ; Arrhythmias, Cardiac* ; Cardiopulmonary Resuscitation*

Limited data are available on improved outcomes after initiation of neurointensivist co-management in neurosurgical intensive care units (NSICUs) in Korea. We evaluated the impact of a newly appointed neurointensivist on the outcomes of neurosurgical patients admitted to an intensive care unit (ICU). This retrospective observational study involved neurosurgical patients admitted to the NSICU at Samsung Medical Center between March 2013 and May 2016. Neurointensivist co-management was initiated in October 1 2014. We compared the outcomes of neurosurgical patients before and after neurointensivist co-management. The primary outcome was ICU mortality. A total of 571 patients were admitted to the NSICU during the study period, 291 prior to the initiation of neurointensivist co-management and 280 thereafter. Intracranial hemorrhage (29.6%) and traumatic brain injury (TBI) (26.6%) were the most frequent reasons for ICU admission. TBI was the most common cause of death (39.0%). There were no significant differences in mortality rates and length of ICU stay before and after co-management. However, the rates of ICU and 30-day mortality among the TBI patients were significantly lower after compared to before initiation of neurointensivist co-management (8.5% vs. 22.9%; P = 0.014 and 11.0% vs. 27.1%; P = 0.010, respectively). Although overall outcomes were not different after neurointensivist co-management, initiation of a strategy of routine involvement of a neurointensivist significantly reduced the ICU and 30-day mortality rates of TBI patients.
Brain Injuries ; Cause of Death ; Critical Care Outcomes ; Critical Care* ; Humans ; Intensive Care Units* ; Intracranial Hemorrhages ; Korea ; Mortality ; Neurosurgery ; Observational Study ; Retrospective Studies

PURPOSE: Treatment of Kawasaki disease with intravenous gamma globulin(IVGG), together with aspirin, has been dernonstrated to be safe and effective in preventing coronary artery lesion and systemic inflarnmation, but optimal IVGG dosage and administration method are still controversial. We compared the therapeutic efficacy and clinical response of single IVGG 1g/kg to that of IVGG lg/kg for comparable risk group of Kawasaki disease. METHODS: We conducted a prospective study involving 63 children with Kawasaki disease requiring IVGG treatment(Harada score> or =4) at Chungnam National University Hospital from February 1996 to January 1999. The children were assigned to receive IVGG either as a single infusion of 1g/kg(A group, 32 person) or 2g/kg(B group, 31 person) and aspirn(100mg/kg/day through acute phase, then 3 to 5mg/kg/day for 8 weeks of duration). RESULTS: There were no significant difference between the two groups according to clinical and laboratry data, including coronary artery lesions(group A, 31.3% and group B, 29.0%) before treatment. After IVGG treatment ratio of complication with coronary artery lesion(group A 1/32=3.1% and group B, 2/31=6.5%) and that of retreatment(group A, 4/32=12.5%, group B, 2/31=6.5%), duration of fever(group A, 1.3+/-1.6 days and group B, 0.7+/-1.4 days), hospital stay(group A, 7.0+/-1.4 days and group B, 6.5+/-2.0 days), laboratory finding and side effects of IVGG were not significantly different(P>0.05). The total dosage of IVGG was significantly lower in group A than group B(group A, 1.16+/-0.37g/kg, 375,421+/-207,351won and group B, 2.10+/-0.40g/kg, 641,498+/-274,750won (P<0.05). CONCLUSION: The therapeutic efficacy and clinical response of single 1g/kg therapy are comparable to that of single 2g/kg therapy.
Aspirin ; Child ; Chungcheongnam-do ; Coronary Vessels ; gamma-Globulins* ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Prospective Studies

PURPOSE: We performed a study on clinical benefits of Harada's scoring method, problems of present criteria and requirement of the revision of that criteria for intrvenos gamma-globulin (IVGG) treatment of Kawasaki disease (KD) in Korea. METHODS: The patients of 104 cases who visited to Department of Pediatrics of Chungnam National University Hospital and diagnosed as KD were objective group. We classified the patients to high and low risk groups as two methods of Harada's scoring method and present criteria for IVGG treatment of KD in Korea, and analyzed the results after treatment by means of echocardiogram. RESULTS: 1. Characteristics of the patients 1) Duration to diagnosis: Mean duration was 6.0 3.5days after onset of fever. 2) Age of onset and male to female ratio: the patients of 84.6% were the age of 4 years or less, and male to female ratio was 1.8 to 1. 2. Coronary aitery lesion (CAL) abnormalities according to items of Harada's scoring method and classification of risk groups 1) The age of less than 12 months and CRP of 3+ or more were significant predictive factors 2) Classification of risk groups was significantly predictive in CAL development. 3. Classification of total cases The patients reserved to administration for IVGG according to Harada's scoring method and present criteria for IVGG treatment of KD in Korea were 63.5% and 50% respectively. If so in present criteria for IVGG treatment of KD in Korea, 50% of the KD patients were excluded for that application. 4. Outcome after treatment according to selective IVGG use by two methods 1) Selective IVGG use by Harada's scoring method: There was no significant difference in development of CAL between low risk group and high risk group, resulting in 1 case (14.3%) of 7 cases and 5 cases (9.4%) of 53 cases respectively. 2) Selective IVGG use by present criteria for IVGG treatment of KD in Korea: There were more significant cases at low risk group than those at high risk group in development of CAL, resulting in 3 cases (42.9%) of 7 cases and 5 cases (12.2%) of 41 cases respectively. 3) Outcome after treatment in patients not administered IVGG as low risk groups according to two methods: There were more significant cases in low risk group at present criteria for IVGG treatment of KD in Korea than those at Harada's scoring method in the development of CAL, resulting in 3 cases (42.9%) of 7 cases and 1 case (14.3%) of 7 ases respectively. 4) Outcome after treatment in patients administered IVGG as high risk groups according to two methods: There was no significant difference between high risk groups at both methods in the CAL development, resulting in 5 cases (12.2%) of 41 cases at present criteria for IVGG treatment of KD in Korea and 5 cases (9.4%) of 53 cases at Harada's scoring method. CONCLUSIONS: Harada's scoring method had more benefits in clinical application, selection of risk group, and outcome after treatment, So we propose the revision of present criteria for IVGG treatment of KD in Korea, suggesting another method as indication for selective IVGG treatment.
Age of Onset ; Chungcheongnam-do ; Classification ; Diagnosis ; Female ; Fever ; gamma-Globulins ; Humans ; Korea* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Pediatrics ; Research Design* ; Risk Factors

BACKGROUND: Several methods have been used to evaluate the engraftment and to monitor residual disease after bone marrow transplantation (BMT). Among them, karyotyping have been useful in gauging engraftment following opposite sex BMT. More recently, fluorescence in situ hybridization (FISH) has also been applied to determine engraftment and residual status. In order to establish the utility of this method in clinical practice, we have evaluated the data from FISH and several methods. METHODS: We performed FISH using chromosome X alpha-satellite probe (Oncor , USA) on twenty eight peripheral blood and nine bone marrow nuclear cells from eleven patients who underwent sex mis-matched transplant and from a patient who had a loss of X chromosome. RESULTS: In nine patients with well engrafted BMT, signals of host cells showed less than 5% in all patients, evaluated 21-210 days post-transplant. Mixed chimerism was detected in six patients; transiently in early post-transplant period in four, in a patient with engraftment failure, and in a patient with relapse, respectively. CONCLUSION: FISH using X probe is a rapid, quantitative and sensitive 'interphase cytogenetic method' for the evaluation of engraftment and monitoring of residual disease following sex mis-matched BMT or BMT in a patient with a loss of X chromosome; It is especially useful in early post-transplant period when ony a few cells are available during severe cytopenia.
Bone Marrow Transplantation* ; Bone Marrow* ; Chimerism ; Cytogenetics ; Fluorescence* ; Humans ; In Situ Hybridization* ; Karyotyping ; Recurrence ; X Chromosome

No abstract available.
Catheters* ; Cystic Adenomatoid Malformation of Lung, Congenital* ; Fetal Therapies* ; Lung*