PURPOSE: This study aimed to identify the early predictors of neurodevelopmental outcomes in infants undergoing therapeutic hypothermia for neonatal hypoxic ischemic encephalopathy. METHODS: The medical records of 24 neonates who underwent hypothermia therapy for hypoxic ischemic encephalopathy at the neonatal intensive care unit of Yeouido St. Mary's Hospital of the Catholic University of Korea between August 2013 and May 2016 were reviewed. Patients were divided into two groups according to their neurological outcome at the age of 18 to 24 months: a normal group (n=14), which included patients with normal neurological function, and an abnormal group (n=10), which included patients with neurological deficits. The clinical characteristics, clinical outcomes, and laboratory findings before and after hypothermia treatment were compared between the groups. RESULTS: There were no significant differences in the demographic characteristics between the two groups. With regard to clinical outcomes, only brain magnetic resonance imaging (MRI) findings showed significant differences between the normal and abnormal groups (21.4% vs. 100.0%, P < 0.001). With regard to laboratory findings, there were significant differences in the white blood cell (WBC) count after hypothermia treatment between the normal and abnormal groups (9.78±3.52 vs. 14.90±3.48, P=0.003). However, logistic regression analysis showed that the WBC count was not an independent risk factor for abnormal neurodevelopment (P>0.05). CONCLUSION: The presence of abnormal lesions on MRI was the most useful predictor of poor neurodevelopmental outcome in infants treated with therapeutic hypothermia after perinatal asphyxia.
Asphyxia ; Brain ; Humans ; Hypothermia ; Hypothermia, Induced ; Hypoxia-Ischemia, Brain ; Infant ; Infant, Newborn ; Intensive Care, Neonatal ; Korea ; Leukocytes ; Logistic Models ; Magnetic Resonance Imaging ; Medical Records ; Risk Factors ; Treatment Outcome

PURPOSE: The purpose of this report is to organize the history of Korean Mothersafe, a professional teratology information center. Throughout its domestic and international activities, Mothersafe has firm role to write journals and books, to provide counseling, to run educational courses on maternal and fetal toxicology, and so on. This paper delineates the achievements in last ten years and discusses the vision of Mothersafe. METHODS: In order to formulate the accomplishments of Korean Mothersafe, the volume of counseling calls are carefully reviewed by their contents, counselees, and the consultation results, etc. Textbooks and journals based on the data from Mothersafe are evaluated. Other roles of the Mothersafe, such as hosting symposium and running public programs are also noted. RESULTS: Korean Mothersafe provided 76,555 counseling to 45,933 expectant women and 30,622 breast-feeding women. The database has total 52,130 enrollments from 2010 to 2019. A total of 33 papers are published regarding medication, alcohol, smoking, radiation, etc. A specialized book on maternal-fetal toxicology was published and teratology part of obstetrics textbook was updated. Education programs and symposiums were held and primary care programs for expectant parents are run by Mothersafe. CONCLUSION Korean Mothersafe has contributed to safe environments for numerous pregnant and breast-feeding women regarding medication, drinking, smoking, and other hazardous substances. The huge database provided evidence for researches, textbooks and seminars, etc. Korean Mothersafe is now facing a new challenge to go forward through social dynamics with many issues regarding prevention and continuance of pregnancy, abortion, and so on.

BACKGROUND: In patients with HIV, CD4+ T cell count and viral load are the main laboratory tests performed to assess clinical management. However, they require extensive resources. In this study, we aimed to determine whether hematological parameters measured using a hematology analyzer are useful as surrogate markers of CD4+ T cell count and viral load in HIV-infected patients. METHODS: Peripheral blood samples were obtained from 14 HIV-naïve, 105 HIV-treated, and 103 uninfected individuals. Hematological parameters were measured using the ADVIA 2120i hematology analyzer (Siemens Healthcare Diagnostics, USA). RESULTS: In HIV-naïve and -treated patients, the percentage of large unstained cells (%LUCs) was 2.5±1.6% and 1.9±0.7%, respectively, compared to 1.6±0.5% in HIV-uninfected controls. The %LUCs was higher in HIV patients with low CD4⁺ T cell count below 200/μL (2.4±1.0%) or high viral load ≥200 copies/mL (2.4±0.8%) than in other infected groups. Significant differences in lymphocyte count were observed between the HIV-naïve (1.5±0.6×10⁹/L) and uninfected (2.0±0.6×10⁹/L) groups as well as between HIV patients with CD4⁺ T cells ≥500/μL (2.5±0.6×10⁹/L) and other infected groups. Neutrophil count varied between high viral load (3.0±1.4×10⁹/L) and low viral load (3.7±1.3×10⁹/L) groups. The CD4⁺ T cell count correlated with lymphocyte count (r=0.642, P<0.0001) and %LUCs (r=-0.287, P=0.002). CONCLUSIONS: %LUCs, lymphocyte count, and neutrophil count are probable surrogate markers of CD4⁺ T cells and viral load.
Biomarkers ; Cell Count ; Delivery of Health Care ; Disease Progression* ; Hematology ; HIV Infections* ; HIV* ; Humans ; Lymphocyte Count ; Neutrophils ; T-Lymphocytes ; Viral Load

Eosinophilic cholangiopathy is a rare disease characterized by dense transmural eosinophilic infiltration of the gallbladder and bile duct. It's clinical and laboratory manifestations are not different from those of other causes of cholangiopathy, and the diagnosis is usually made based on pathologic findings after cholecystectomy. Moreover, the occurrence of eosinophilic cystitis accompanied by cholangiopathy is extremely rare. We report a rare case of hypereosinophilic syndrome manifested as eosinophilic cholangiopathy accompanied with eosinophilic cystitis, for the first time in Korea, in a 49-year-old woman who presented with persistent right upper quadrant pain. After performing imaging study to validate the diagnosis of acute acalculous cholecystitis and cholangitis, an urgent cholecystectomy was performed. Pathologic examination of the excised gallbladder was consistent with eosinophilic cholecystitis. The patient underwent bladder biopsy because there was persistant irritative voiding symptoms combined with constant mild peripheral eosinophilia even after cholecystectomy, and the pathologic findings revealed eosinophilic cystitis. Symptoms and peripheral eosinophilia were improved after steroid therapy for an indicated period.
Acalculous Cholecystitis ; Bile Ducts ; Biopsy ; Cholangitis ; Cholecystectomy ; Cholecystitis ; Cystitis* ; Diagnosis ; Eosinophilia ; Eosinophils* ; Female ; Gallbladder ; Humans ; Hypereosinophilic Syndrome* ; Korea ; Middle Aged ; Rare Diseases ; Urinary Bladder

PURPOSE: In this single center study, we assessed the timing of presentation of necrotizing enterocolitis (NEC) diagnosed by sonography according to the gestational age. METHODS: We retrospectively reviewed the medical records of 49 newborn patients who were diagnosed with NEC (modified Bell's stage II and higher according to abdominal sonography and simple abdominal radiography) and were admitted to the neonatal intensive care unit of St. Mary's Hospital between January 2008 and December 2012. Infants were grouped according to their gestational age (GA): Group I (GA <28 weeks), Group II (GA, 28-32 weeks), Group III (GA, 33-36 weeks), and Group IV (GA > or =37 weeks); early-onset NEC was considered when NEC developed at <14 days of age and late-onset NEC was considered when NEC developed at > or =14 days of age. RESULTS: The number of infants in each group were: Group I (n = 16), Group II (n = 20), Group III (n = 11), and Group IV (n = 2). The mean age at diagnosis of NEC was: Group I (31.6 days), Group II (15.9 days), Group III (11.0 days), and Group IV (2.0 days). Early-onset NEC developed at a mean of 5.5 days of age, whereas late-onset NEC developed at a mean of 36.1 days of age. CONCLUSION: Based on early sonographic diagnosis, infants with lower gestational ages developed NEC at a more advanced postnatal age as compared to more mature infants. However, further studies are needed to understand the etiology of this disease process.
Diagnosis ; Enterocolitis, Necrotizing* ; Epidemiology ; Gestational Age* ; Humans ; Infant ; Infant, Newborn ; Intensive Care, Neonatal ; Medical Records ; Retrospective Studies ; Ultrasonography

Familial Mediterranean fever (FMF) is an inherited autosomal recessive disorder, ethnically restricted and commonly found among populations surrounding the Mediterranean Sea. FMF is the most prevalent autoinflammatory disease; is characterized by recurrent, self-limited episodes of fever with serositis; and is caused by Mediterranean fever gene (MEFV) mutations on chromosome 16. We describe a case of adult-onset FMF with complete symptomatic remission during pregnancy, without the use of colchicine. A 25-year-old woman had presented with periodic fever, abdominal pain, and vomiting since she was 21. Her abdominal computed tomography scan showed intestinal nonrotation. She underwent exploratory laparotomy and appendectomy for her symptoms 1 year prior. She had a symptom-free pregnancy period, but abdominal pain and fever recurred after delivery. Mutation analysis of the MEFV gene revealed two point mutations (p.Leu110Pro and p.Glu148Gln). We report an adult female patient with FMF in Korea with complete symptomatic remission during pregnancy.
Abdominal Pain ; Adult ; Appendectomy ; Chromosomes, Human, Pair 16 ; Colchicine ; Familial Mediterranean Fever* ; Female ; Fever ; Humans ; Korea ; Laparotomy ; Mediterranean Sea ; Point Mutation ; Pregnancy* ; Serositis ; Vomiting

PURPOSE: The purpose of this study was to examine the usefulness of abdominal sonography in the diagnosis of necrotizing enterocolitis (NEC). METHODS: We reviewed the medical records of 51 neonates who were diagnosed with NEC in the neonatal intensive care unit at Yeouido St. Mary's Hospital of the Catholic University in Korea between January 2008 and December 2012. The neonates underwent abdominal ultrasonography on the day of their diagnosis and on the third day after diagnosis. Simple abdominal radiography was performed on the same day as the sonography. The neonates were diagnosed with NEC in accordance with the abdominal sonographic findings. Abdominal radiography and sonography were used to assess the NEC stages in the neonates. RESULTS: On the day of NEC diagnosis by abdominal sonography, 50 neonates were diagnosed with stage II NEC and 1 was diagnosed with stage III NEC. However, simple radiography diagnosed 49 neonates with stage I NEC, 1 with stage II NEC, and 1 with stage III NEC. On the third day after NEC diagnosis by abdominal sonography, 48 neonates were diagnosed with stage II NEC and 3 were diagnosed with stage III NEC. On the other hand, simple radiography diagnosed 26 neonates with stage I NEC, 24 with stage II NEC, and 1 with stage III NEC. CONCLUSION: Abdominal sonography can be used as a tool for accurately diagnosing NEC and treating neonates showing ambiguous symptoms during the early stages of NEC.
Diagnosis* ; Early Diagnosis ; Enterocolitis, Necrotizing* ; Hand ; Humans ; Infant, Newborn ; Intensive Care, Neonatal ; Korea ; Medical Records ; Radiography ; Radiography, Abdominal ; Ultrasonography

The occurrence of valporic acid (VPA)-induced pancreatitis is a rare condition, predominantly observed in adolescent. Also, the occurrence of VPA-associated with hemorrhagic pseudocyst is extremely rare. We report the case of a 54-year-old man who had been taking VPA for uncontrolled seizures. He was admitted to our hospital with complaints of abdominal pain and diagnosed with acute on chronic pancreatitis. There were no other causes explaining pancreatitis, and it was thought to be due to VPA therapy. Despite of cessation of VPA, there was ongoing severe abdominal pain with fever. The patient underwent follow-up CT, which revealed a large loculated fluid collection that was observed with intra-cystic hemorrhage. After treatment with percutaneous catheter drainage, he was discharged with regression of the pancreatic pseudocyst. VPA-associated pancreatitis with hemorrhagic pseudocyst is rare but possible. Therefore, this possibility should be considered in the cause of hemorrhagic pseudocyst in a patient taking VPA.
Abdominal Pain ; Adolescent ; Catheters ; Drainage ; Fever ; Follow-Up Studies ; Hemorrhage ; Humans ; Middle Aged ; Pancreatic Pseudocyst ; Pancreatitis* ; Pancreatitis, Chronic ; Seizures ; Valproic Acid

Anaphylaxis to proton pump inhibitors (PPIs) has rarely been reported. Different patterns of cross-reactivity between PPIs have also been demonstrated using skin tests. Here, we report a case of anaphylaxis to lansoprazole with tolerance to other commercially available PPIs, which was proved by skin tests and oral provocation tests (OPTs). A 47-year-old female patient visited our Emergency Department with a sudden onset of whole body urticaria, facial swelling, dyspnea, and loss of consciousness that developed 1 hour after ingestion of 30 mg of lansoprazole for her episodic epigastric soreness. The skin prick test (SPT) and the intradermal test (IDT) with lansoprazole, esomeprazole, rabeprazole, and pantoprazole were performed. Lansoprazole showed positive reactions in both the SPT (3 mg/mL) and the IDT (0.003 mg/mL). Rabeprazole (3 mg/mL) showed a positive reaction only in IDT. The SPT and the IDT with esomeprazole and pantoprazole were all negative. The OPT with 30 mg of lansoprazole was positive (showing generalized rash and facial swelling 30 minuites after ingestion), while OPTs with esomeprazole, pantoprazole, and rabeprazole were all negative. Other PPIs could be safe alternatives in cases of anaphylaxis to 1 PPI. Skin tests seem to be helpful to define cross-reactivity between PPIs.
Anaphylaxis* ; Dyspnea ; Eating ; Emergency Service, Hospital ; Esomeprazole ; Exanthema ; Female ; Humans ; Intradermal Tests ; Lansoprazole* ; Middle Aged ; Proton Pump Inhibitors* ; Rabeprazole ; Skin ; Skin Tests ; Unconsciousness ; Urticaria

PURPOSE: The aim of this study was to analyze parental decisions regarding pregnancies in which the fetus had sex chromosome abnormalities (SCA) over a ten-year period. MATERIALS AND METHODS: We collected and reviewed records from our hospital for 2001-2010 and a genetic specialist provided-genetic counseling. RESULTS: We diagnosed 130 cases (0.71%) with SCA out of 18,376 prenatal cases from 2001 to 2010. We reviewed the records and the results of all pregnancies. We also included cases (n=84) of apparently normal anatomic fetuses to analyze the factors influencing parental decisions. We excluded 34 cases with an obvious anomaly or a presumably bad outcome and 12 cases that were not followed up. Forty-three couples (51.2%) continued their pregnancies while forty-one (48.8%) terminated them. Of 38 mosaicism cases, 21 (55.3%) were continued. Among the 20 pregnancies assisted by reproductive techniques, 15 (75%) were continued (P=0.02). More pregnancies were continued when genetic counseling was provided (61.9%) compared to cases in which it was not provided (19%) (P=0.01). CONCLUSION: Genetic counseling is important in providing appropriate information to parents. Establishing guidelines and protocols will help both obstetricians and parents to make informed decisions.
Family Characteristics ; Fetus ; Genetic Counseling ; Humans ; Mosaicism ; Parents ; Pregnancy ; Prenatal Diagnosis ; Reproductive Techniques ; Sex Chromosome Aberrations ; Sex Chromosomes ; Specialization