We studied to assess the relationship between intrauterine growth retardation and theincreased nucleated red blood cell counts (NRBC) in small for gestational age (SGA) and appropriatefor gestational age (AGA) neonates with low birth weight. We also evaluated the nucleated red blood cell counts in low birth weight neonates who had either perinatal asphyzia or hyaline membrane disease (HMD) or died within 7 days after birth. The results were as follows: 1) In low birth weight neonates, the mean value for NRBC counts was 9.02/100 WBCs and the mean absolute value for NRBC counts was 0.9210E9/L. 2) The mean values for NRBC counts were 13.4/100 WBCs in SGA and 6.4/100WBCs in AGA. The mean absolute values for NRBC were 1.32x10E9/L in AGA neonates 3) In SGA neonates with low birth weight, the mean NRBC counts wers 19.6/100WBCs in asphyxiated group and 4.5/100WBCs in control group. The mean absolute NRBC counts were 1.9810E9/L in control group. 4) In AGA neonates with low birth weight, the mean NRBC countswere 9.1/100WBCs in asphyxiated group and 2.4/100WBCs in control group. The meanabsolute NRBC counts were 0.98x10E9/L in asphyxiated group and o.23x10E9/L in controlroup. 5) The mean NRBC counts were 13.8/100WBCs in neonates with HMD and 7.1/100WBCs in control group. The mean absolute NRBC counts were 1.50x10E9/L in neonates withHMD and 0.70x10E9/L in control group. 6) The mean NRBC counts were 19.9/100 WBCs in expired group and 6.8/100WBCs in suvived group. The mean absolute NRBC counts were 2.1810E9/L in expired group and 0.66x10E9/L in survived group. 7) The NRBC counts of SGA neonates were significantly higher than that of AGA neonates with low birth weight. 8) The NRBC counts of asphyxiated neonates were significantly higher than that of the control group. 9) The NABC counts of expired neonates were significantly higher than that of the control group. 10) The NRBC counts of expired neonates were significantly higher than that of the survived neonates.
Birth Weight* ; Erythrocyte Count* ; Erythrocytes* ; Fetal Growth Retardation ; Gestational Age ; Humans ; Hyaline Membrane Disease ; Infant, Low Birth Weight ; Infant, Newborn* ; Parturition*

No abstract available.

No abstract available.
Humans ; Hypoglycemia* ; Infant* ; Infant, Newborn* ; Iron*

BACKGROUND: This report describes the clinicopathologic findings of six hepatic masses that developed after Kasai hepatic portoenterostomy (HPE) in six patients with longstanding biliary atresia (BA). METHODS: Hepatic masses were found in six of 55 pediatric patients who underwent liver transplantation for BA after Kasai HPE from 1997 to 2009. Clinicopathologic analysis was performed and immunohistochemical staining was carried out for CD34, smooth muscle actin (SMA) and cytokeratin 7. RESULTS: Of the six hepatic masses, two were diagnosed as focal nodular hyperplasia (FNH)-like lesions, two were large regenerative nodules (LRN), one was a mesenchymal hamartoma (MH) and one was a cholangiocarcinoma. The immunohistochemical staining findings for SMA and CD34 were more prominent for the FNH-like nodules than for the cirrhotic background liver. Dysplastic biliary epithelium arising from intestinal metaplasia was found in the cholangiocarcinoma. CONCLUSIONS: Our findings suggest that FNH-like lesions, LRNs and MH are the results of vascular hemodynamic changes after Kasai HPE and that cholangiocarcinoma is due to recurrent cholangitis after BA. All the lesions in this series must be included in the differential diagnosis of a newly formed hepatic mass in patients after portoenterostomy.
Actins ; Biliary Atresia ; Child ; Cholangiocarcinoma ; Cholangitis ; Diagnosis, Differential ; Epithelium ; Focal Nodular Hyperplasia ; Hamartoma ; Hemodynamics ; Humans ; Keratins ; Liver ; Liver Transplantation ; Metaplasia ; Muscle, Smooth ; Portoenterostomy, Hepatic

No abstract available.
Epidermolysis Bullosa ; Muscular Dystrophy, Duchenne ; Ornithine Carbamoyltransferase ; Polymerase Chain Reaction* ; Preimplantation Diagnosis*

Objective: : Gamma Knife radiosurgery (GKRS) is an effective and noninvasive treatment for high-risk arteriovenous malformations (AVMs). Since differences in GKRS outcomes by nidus type are unknown, this study evaluated GKRS feasibility and safety in patients with brain AVMs. Methods: : This single-center retrospective study included patients with AVM who underwent GKRS between 2008 and 2021. Patients were divided into compact- and diffuse-type groups according to nidus characteristics. We excluded patients who performed GKRS and did not follow-up evaluation with magnetic resonance imaging or digital subtraction angiography within 36 months from the study. We used univariate and multivariate analyses to characterize associations of nidus type with obliteration rate and GKRS-related complications. Results: : We enrolled 154 patients (mean age, 32.14±17.17 years; mean post-GKRS follow-up, 52.10±33.67 months) of whom 131 (85.1%) had compact- and 23 (14.9%) diffuse-type nidus AVMs. Of all AVMs, 89 (57.8%) were unruptured, and 65 (42.2%) had ruptured. The mean Spetzler-Martin AVM grades were 2.03±0.95 and 3.39±1.23 for the compact- and diffuse-type groups, respectively (p<0.001). During the follow-up period, AVM-related hemorrhages occurred in four individuals (2.6%), three of whom had compact nidi. Substantial radiation-induced changes and cyst formation were observed in 21 (13.6%) and one patient (0.6%), respectively. The AVM complete obliteration rate was 46.1% across both groups. Post-GKRS complication and complete obliteration rates were not significantly different between nidus types. For diffuse-type nidus AVMs, larger AVM size and volume (p<0.001), lower radiation dose (p<0.001), eloquent area location (p=0.015), and higher Spetzler-Martin grade (p<0.001) were observed. Conclusion : GKRS is a safe and feasible treatment for brain AVMs characterized by both diffuse- and compact-type nidi.

OBJECTIVE: Embryonic stem (ES) cells could be differentiated into the specific cell types by alternation of culture condition and modification of gene expression. This study was performed to evaluate the differentiation protocol for mouse and human ES cells to insulin secreting cells. METHODS: Undifferentiated mouse (JH-1) and human (Miz-hES1) ES cells were cultured on STO feeder layer, and embryoid bodies (EBs) were formed by suspension culture. For the differentiation, EBs were cultured by sequential system with three stage protocol. The differentiating ES cells were collected and marker gene expressions were analyzed by semi-quantitative RT-PCR in each stage. Amount of secreted insulin levels in culture media of human ES cells were measured by human insulin specific RIA kit. RESULTS: During the differentiation process of human ES cells, GATA-4, alpha-fetoprotein, glucose transporter-2 and Ngn-3 expression were increased whereas Oct-4 was decreased progressively. Insulin and albumin mRNAs were expressed from stage II in mouse ES cells and from stage III in human ES cells. We detected 3.0~7.9 microU/ml secretion of insulin from differentiated human ES cells by in vitro culture for 36 days. CONCLUSION: The sequential culture system could induce the differentiation of mouse and human ES cells into insulin secreting cells. This is the first report of differentiation of human ES cells into insulin secreting cells by in vitro culture with serum and insulin free medium.
alpha-Fetoproteins ; Animals ; Culture Media ; Embryoid Bodies ; Embryonic Stem Cells* ; Feeder Cells ; Gene Expression ; Glucose ; Humans ; Insulin* ; Insulin-Secreting Cells* ; Mice ; RNA, Messenger

Acute generalized exanthematous pustulosis (AGEP) is characterized by acute nonfollicular sterile pustules on a background of edematous erythema. Hydroxychloroquine (HCQ), an antimalarial drug, widely used to treat rheumatic and dermatologic diseases. HCQ has been reported to be an uncommon cause of AGEP. We report a 60-year-old woman with rheumatoid arthritis requiring the use of HCQ presented fever and erythematous eruption on the trunk with sterile pustules. Leukocytosis and elevated erythrocyte sedimention rate noted on laboratory examination. On the histopathological examination of the skin biopsy specimen showed neutrophilic infiltration and scattered eosinohpils. The lesions were resolved with removal of HCQ. The clinical course was consistent with the diagnosis of AGEP associated with HCQ. We reported a case of typical AGEP associated with HCQ in a patient with Rheumatoid arthritis. The patient presented resolution from cutaneous lesions with withdrawal of culprit drug, without the need of systemic steroid.
Acute Generalized Exanthematous Pustulosis ; Arthritis, Rheumatoid ; Biopsy ; Erythema ; Erythrocytes ; Female ; Fever ; Humans ; Hydroxychloroquine ; Leukocytosis ; Neutrophils ; Skin

BACKGROUND AND OBJECTIVES: Atrial fibrillation(AF) is the most frequently encountered arrhythmia in clinical practice. Pharmacologic therapy has been advocated for both immediate restoration of sinus rhythm and prevention of recurrent AF. Because conventional antiarrhythmic therapy is often ineffective in maintaining sinus rhythm or is associated with adverse side effects in patients with AF, recent interest has focused on the use of class III antiarrhythmic agents. This study investigated the efficacy and safety of sotalol and amiodarone for conversion of chronic AF and prevention of recurrent AF. MATERIALS AND METHOD: Thirty six patients with AF were firstly received sotalol by prospective study protocol. The patients were classified as having paroxysmal AF(PAF, N=2) or chronic AF(CAF, N=4) based on AF pattern. If the patients with CAF did not convert to sinus rhythm or the patients with PAF recurred in AF, the patients were received second agents(amiodarone). Patients were followed up for one year. RESULTS: Among the 12 patients with PAF receiving sotalol, 10(83.3%) patients remained in normal sinus rhythm for average 9.4+/-3.6 months. Sotalol was replaced by amiodarone in the remaing 2 patients with arrhythmia recurrence and 1 of the 2 patients remained in sinus rhythm during the follow-up period. In the case of 24 patients with CAF, conversion to sinus rhythm was achieved in 5(20.8%) patients with sotalol. Among the patients with CAF who were not respond to sotalol, 17 patients received amiodarone subsquently and 3 patients successfully converted to sinus rhythm. There were no proarrhythmic effects related to both agents during the study period. CONCLUSION: Both sotalol and amiodarone appear to be less effective in the termination of CAF, but sequential use of these two agents seem to be very effective for the prevention of recurrence of PAF.
Amiodarone* ; Arrhythmias, Cardiac ; Atrial Fibrillation* ; Follow-Up Studies ; Humans ; Prospective Studies ; Recurrence ; Sotalol*

OBJECTIVE: Preimplantation genetic diagnosis (PGD) is reserved for couples with a risk of transmitting a serious and incurable disease, and hence avoids the undesirable therapeutic abortion. In this study, we evaluated the efficacy of PGD for Duchenne muscular dystrophy (DMD) cases by the fluorescent PCR with polymorphic linked markers and the conventional duplex-nested PCR methods. METHODS: Biopsy of one or two blastomeres was done from the embryos fertilized by ICSI on the third day after fertilization. We performed two cases of PGD-DMD by the duplex-nested PCR for the causative mutation loci and the SRY gene on Y chromosome. The triplex fluorescent PCR for the mutation loci, the SRY gene and the polymorphic microsatellite marker on X chromosome was applied for two cases of PGD-DMD. RESULTS: By the duplex-nested PCR, successful diagnosis rate was 95.5% (21/22), but we could not discriminate the female embryos whether normal or carrier in this X-linked recessive disease. However, the triplex fluorescent PCR method showed 100% (27/27) of successful diagnosis rate, and all female embryos (n=17) were distinguished normal (n=10) from carrier (n=7) embryos. Unaffected and normal embryos were transferred into mother's uterus after diagnosis. A healthy normal male was achieved after PGD with the duplex-nested PCR method and a twin, a male and a female, were delivered with triplex fluorescent PCR method. The normality of dystrophin gene was confirmed by amniocentesis and postnatal genetic analysis in all offsprings. CONCLUSION: The fluorescent PCR with polymorphic marker might be useful in improving the specificity and reliability of PGD for single gene disorders.
Abortion, Therapeutic ; Amniocentesis ; Biopsy ; Blastomeres ; Diagnosis ; Dystrophin ; Embryonic Structures ; Family Characteristics ; Female ; Fertilization ; Genes, sry ; Humans ; Male ; Microsatellite Repeats ; Muscular Dystrophy, Duchenne* ; Polymerase Chain Reaction* ; Pregnancy ; Preimplantation Diagnosis* ; Prostaglandins D ; Sensitivity and Specificity ; Sperm Injections, Intracytoplasmic ; Twins ; Uterus ; X Chromosome ; Y Chromosome