Severe congenital neutropenia (SCN) is a heterogeneous group of disorders with a defect in granulopoiesis causing marked neutropenia and severe bacterial infections. A 17-month-old girl (patient 1) was admitted due to cervical lymphadenitis caused by methicillin-resistant Staphylococcus aureus, with neutropenia. She had Pseudomonas aeruginosa sepsis and peritonitis with perforated appendicitis at 8-month of age. Her sister, a 37-month-old girl (patient 2), had recurrent stomatitis with profound neutropenia, and her mother, a 32-yr-old woman (patient 3), had had recurrent stomatitis until her early 20s with neutropenia. We found an ELANE gene mutation (c.597+1G > A) from them in direct DNA sequencing analysis. Patients 1 and 2 did not respond to granulocyte colony stimulating factor and patient 1 was treated with prolonged antibiotics and excision. We demonstrated inherited SCN cases showing different severity even with the same mutation of the ELANE gene in a family.
Adult ; Child, Preschool ; DNA Mutational Analysis ; Female ; Granulocyte Colony-Stimulating Factor/therapeutic use ; Humans ; Infant ; Leukocyte Elastase/*genetics ; Methicillin-Resistant Staphylococcus aureus/isolation & purification ; Mutation/genetics ; Neutropenia/*congenital/diagnosis/drug therapy/genetics ; Pedigree ; *Phenotype ; Polymorphism, Single Nucleotide ; Recurrence ; Staphylococcal Infections/diagnosis/microbiology ; Stomatitis/diagnosis ; Tomography, X-Ray Computed

Women in the reproductive age group diagnosed with cervical cancer can receive radical trachelectomy for fertility preservation. Extremely short cervix following radical trachelectomy could result in cervical incompetence. Although prophylactic cervicoisthmic cerclage is placed at the time of radical trachelectomy, it might not be sufficient to prolong pregnancy. We present a successful term pregnancy after laparoscopic radical trachelectomy and concurrent cervicoisthmic cerclage for early stage cervical cancer.
Cervix Uteri ; Female ; Fertility Preservation ; Humans ; Pregnancy ; Trachelectomy ; Uterine Cervical Neoplasms

BACKGROUND: A light plane of general anesthesia is chosen for fetal safety during a cesarean section. Therefore an experience of wakefulness and pain perception is not infrequent and can be distressful to patients. The electroencephalogram-derived bispectral index (BIS) is a promising new method to assess anesthetic adequacy. This study was designed to assess the adequacy of general anesthesia and to evaluate the usefulness of the BIS in monitoring the awareness during a cesarean section. METHODS: Twenty one parturients undergoing general anesthesia for an elective caesarean section were examined. Anesthesia was induced with 4 mg/kg thiopental and 1 mg/kg succinylcholine, and then followed with a mixture of 50% N2O, 50% O2 and 0.8% enflurane at a flow of 4 L/min. Before administration of succinylcholine, a tourniquet was applied to the free arm and inflated to 250 mmHg. Responsiveness to verbal commands using the Tunstall isolated forearm technique was detected and the BIS was monitored throughout the study period. RESULTS: The incidence of responsiveness to verbal commands were 33% at the time of skin incision and fetal delivery respectively. BIS values of response to commands were significantly higher than that of no respose at both time points (p < 0.05). Fisher's exact test suggested that the BIS value less than 75 was related to unresponsiveness to verbal commands at time of skin incision (p < 0.05) and the BIS less than 85 at fetal delivery (p < 0.05). CONCLSIONS: These results suggest that adequate anesthesia is not provided with a mixture of 50% N2O, 50% O2 and 0.8% enflurane at a flow of 4 L/min at the time of skin incision and fetal delivery. In addition BIS could be a predictor of awareness during cesarean section under general anesthesia.
Anesthesia ; Anesthesia, General* ; Arm ; Cesarean Section* ; Consciousness Monitors* ; Enflurane ; Female ; Forearm ; Humans ; Incidence ; Pain Perception ; Pregnancy ; Skin ; Succinylcholine ; Thiopental ; Tourniquets ; Wakefulness

A long-term effect of pentobarbital on the atrial natriuretic peptide (ANP) system was investigated. The experimental group of rats (Sprague-Dawley, male) was one week previously treated with pentobarbital (50 mg/kg, intraperitoneal), and the control was an age-matched group of rats which had never been anesthetized. ANP reaponse to volume-expansion (VE) induced by intravenous infusion of iso-oncotic saline over 30 min (total volume infused amounted up to 5% body weight) was examined under thiopental anesthesia (50 mg/kg, intraperitoneal). Basal plasma ANP level did not significantly differ between the experimental and control groups. Following VE, while the plasma ANP five-fold increased in the control, it rather decreased in the experimental group. Despite the different ANP responses, the magnitude of urinary responses (volume and sodium excretion) to VE did not differ between the two groups. Right and left atrial tissue contents of ANP were significantly lower in the experimental group than in the controL In another series of experiments, the two-kidney, one clip rats were made under either pentobarbital or ether anesthesia and the blood pressure and ANP responses were compared. While the magnitude of blood pressure increases did not differ, the plasma ANP level measured on Day 12 after the clipping was lower in the pentobarbital group than in the ether group. These results suggest that pentobarbital has a long-term inhibitory effect on the ANP system. Its physiological significance in blood pressure and body fluid homeostasis remains to be determined.
Anesthesia ; Animals ; Atrial Natriuretic Factor ; Blood Pressure ; Body Fluids ; Ether ; Homeostasis ; Infusions, Intravenous ; Pentobarbital* ; Plasma ; Rats* ; Sodium ; Thiopental

BACKGROUND: The risk of bleeding during extracorporeal membrane oxygenation (ECMO) is a potential deterrent in performing tracheostomy at many centers. To evaluate the safety of surgical tracheostomy (ST) in critically ill patients supported by ECMO, we reviewed the clinical correlation between preoperative coagulation status and bleeding complication-related ST during ECMO. METHODS: From April 1, 2012 to March 31, 2016, ST was performed on 38 patients supported by ECMO. We retrospectively reviewed and analyzed the medical records including complications related to ST. RESULTS: Heparin was administered to 23 patients (60.5%) for anticoagulation during ECMO, but 15 patients (39.5%) underwent ECMO without anticoagulation. Of the 23 patients administered anticoagulation therapy, heparin infusion was briefly paused in 13 prior to ST. The median platelet count, international normalized ratio, and activated partial thromboplastin time before ST were 126 ×109/L (range, 46 to 434 ×109/L), 1.2 (range, 1 to 2.3) and 62 seconds (27 to 114.2 seconds), respectively. No peri-procedural clotting complications related to ECMO were observed. Two patients (5.3%) suffering from ST-related major bleeding required surgical hemostasis. Minor bleeding after ST occurred in two cases (5.3%). No significant difference was found according to anticoagulation management (P = 0.723). No fatality was attributable to ST. CONCLUSIONS: The complication rates of ST in the patients supported by ECMO were low. Therefore, ST performed by an experienced operator, and with careful optimization of coagulation status, is a relatively safe procedure; the use of ST with ECMO should thus not be dismissed on account of the potential for bleeding caused by the administration of anticoagulants.
Anticoagulants ; Critical Illness ; Extracorporeal Membrane Oxygenation* ; Hemorrhage ; Hemostasis, Surgical ; Heparin ; Humans ; International Normalized Ratio ; Medical Records ; Partial Thromboplastin Time ; Platelet Count ; Retrospective Studies ; Tracheostomy*

BACKGROUND: Stellate ganglion block (SGB) has been used to treat over 150 diseases which include diabetes mellitus and gout. This study was planned to investigate whether stellate ganglion block (SGB) could lower the levels of blood glucose, uric acid, epinephrine, and norepinephrine. METHODS: Sixty Sprague-Dawley rats within the weight of 250-350 gm were randomly devided into four groups. CS group was normal group with sham SGB with normal saline, CL group was normal group with SGB with lidocaine, DS group was diabetic group with SGB with normal saline, DL group was diabetic group with SGB with lidocaine. The diabetes was induced by intraperitoneal injection of 40 mg/kg of streptozotocin in citrate buffer (0.01 M, pH 4.5). Nondiabetic groups were given same amount of the citrate buffer. Seven days after the last injection of the streptozotocin blood glucose level was checked and more than 300 mg/dl was considered diabetic. The SGB was performed three times at right superior cervical ganglion two days apart from two days after the conformation of diabetes. Successful SGB was conformed by the ipsilateral ptosis or conjunctival congestion. Blood samplings from tail vein for the check of glucose, uric acid, and catecholamines were done before the injection of streptozotocin, seven days after the last injection of streptozotocin, and two days after the last SGB. RESULTS: The SGB with lidocaine reduced the blood glucose level only in the diabetic rats while SGB with the saline did not. The epinephrine levels were increased in the diabetics and decreased by the SGB with lidocaine without any statistical significance. Norepinephrine and uric acid levels had not been effected by the SGB and both of them had no correlationship with the glucose level. CONCLUSIONS: SGB in the diabetic rats decreases the blood glucose level. But for the effects of the SGB on the level of epinephrine further study would be needed.
Animals ; Blood Glucose* ; Catecholamines* ; Citric Acid ; Diabetes Mellitus ; Epinephrine ; Estrogens, Conjugated (USP) ; Glucose ; Gout ; Hydrogen-Ion Concentration ; Injections, Intraperitoneal ; Lidocaine ; Norepinephrine ; Rats* ; Rats, Sprague-Dawley ; Stellate Ganglion* ; Streptozocin ; Superior Cervical Ganglion ; Uric Acid* ; Veins

BACKGROUND: The purpose of this study was to assess the effect of rocuronium pretreatment on the succinylcholine induced biochemical changes and fasciculations, myalgia and to compare it with vecuronium pretreatment. METHODS: We have studied 60 patients undergoing minor elective surgery, in a prospective double blinded method. Three groups of 20 patients each were pretreated with saline 0.01 ml/kg (group C), rocuronium 0.05 mg/kg (group R), or vecuronium 0.007 mg/kg (group V). Three minutes after the pretreatment, 1.5 mg/kg succinylcholine was injected. Serum potassium five minutes after succinylcholine and creatine kinase 24 hr after operation were measured. Fasciculations and postoperative myalgia at 24 and 48 h were evaluated. RESULTS: The increase in creatine kinase and incidence of fasciculations were lowest in the rocuronium group (33 IU/L; 15%) and followed by vecuronium group (58 IU/L; 50%) and highest in the control group (101 IU/L; 100%). The increase in serum potassium concentration (0.3 mEq/L) was not attenuated by any regimen. The incidence of postoperative myalgia on day 1 and day 2 was lower in the rocuronium (40%, 30%) and the vecuronium (50%; 35%) group than control group (85%; 75%). CONCLUSIONS: Rocuronium pretreatment is more effective in reducing creatine kinase rise and fasciculations after succinylcholine than vecuronium. However rocuronium and vecuronium are also effective in preventing postoperative myalgia.
Creatine Kinase ; Fasciculation ; Humans ; Incidence ; Myalgia* ; Potassium ; Prospective Studies ; Succinylcholine* ; Vecuronium Bromide

BACKGROUND: There is high interest in a new approach to improve children's lifestyle habits in order to prevent childhood obesity. A smartphone application (app) is a new technology platform that can be used to prevent childhood obesity. METHODS: The key messages for healthy eating habits and physical activities were selected by experts in nutrition, physical activity, and psychology and were adjusted for use in elementary school students. Gamification techniques were used to increase app user engagement. A total of 249 students in grades 4-6 were asked to rate their satisfaction for the app after 4 weeks of use. RESULTS: The main menu of "HAPPY ME" was composed of user profile, "EVERYDAY QUEST," exercise diary, diet diary, settings, and notifications. The app users were asked to complete 6-9 daily missions regarding healthy eating habits and physical activities through "EVERYDAY QUEST." The mean satisfaction scores for each variable were distributed from 3.6 to 4.0 points, and most of the students responded that they would continue to use the app (4.0 points). CONCLUSION: We developed a smartphone app (HAPPY ME) for 4th-6th grade elementary students aimed to provide educational information about healthy eating habits and physical activities in order to prevent childhood.
Child ; Diet ; Eating ; Humans ; Life Style ; Motor Activity ; Pediatric Obesity ; Psychology ; Religious Missions ; Smartphone

PURPOSE: Chlamydia pneumoniae is a common intracellular bacterial pathogen and plays an important role in acute respiratory infections. The purpose of this study was to investigate clinical presentations of C. pneumoniae in children with acute respiratory infections. METHODS: We examined the medical records of pediatric patients (age<18 years) admitted with acute respiratory infections of C. pneumoniae to Gachon University Gil Medical Center between March 1, 2011 and August 31, 2014. We compared the clinical features of C. pneumoniae infection with that of Mycoplasma pneumoniae infection. RESULTS: We confirmed acute respiratory infections of C. pneumoniae in 110 patients out of 2,156 patients (5.1%) admitted with acute respiratory infections. The mean age was 37.2+/-30.1 months. More than half of them (54.5%) had coinfection. C. pneumoniae infection had mild and subacute courses. The mean duration of symptoms prior to admission was 8.5+/-13.8 days. There were remarkable seasonal variations and prevalence was higher in December and April (P=0.03 and P=0.02, respectively). Although rhinorrhea and pharyngeal injection were more common in C. pneumoniae infection (P<0.05), clinical signs and symptoms were similar between C. pneumoniae and M. pneumoniae. Extrapulmonary manifestations such as skin lesion, Gastrointestinal symptoms, hepatitis, and neurologic symptoms were common (41.0%) in C. pneumoniae infection and, had similar incidence in M. pneumoniae infection. CONCLUSION: C. pneumoniae is an important infectious agent of acute respiratory infections in children. Clinical pictures of C. pneumoniae are similar to M. pneumoniae, even in extrapulmonary manifestations. C. pneumoniae should be taken into consideration in differential diagnosis of acute respiratory infection in children.
Child* ; Chlamydia* ; Chlamydophila pneumoniae* ; Coinfection ; Diagnosis, Differential ; Hepatitis ; Humans ; Incidence ; Medical Records ; Mycoplasma pneumoniae ; Mycoplasma* ; Neurologic Manifestations ; Pneumonia ; Pneumonia, Mycoplasma* ; Prevalence ; Respiratory Tract Infections* ; Seasons ; Skin

Japanese-B viral encephalitis (JE) usually has a monophasic illness pattern. A 45-year-old woman in an altered mentality had improved over 1 month. About 1 week after the initial improvement, the patient became comatose with aggravated EEG and MRI findings. Assays of cerebrospinal fluid and serum were positive for the IgM antibody to Japanese-B virus. After intravenous immunoglobulin (IVIG) infusion, the patient recovered. We report a patient with JE who showed a biphasic illness pattern and recovered after IVIG therapy.
Cerebrospinal Fluid ; Coma ; Electroencephalography ; Encephalitis, Viral* ; Female ; Humans ; Immunization, Passive* ; Immunoglobulin M ; Immunoglobulins ; Immunoglobulins, Intravenous ; Magnetic Resonance Imaging ; Middle Aged