OBJECTIVE: As an electronic health record system is implementing in Korean health care sectors, concerns about key functionalities of electronic health record systems are increasing. The purpose of this study was to identify core functions and set the priority in electronic health record systems under the Korean contexts in order to assure and improve the quality of the systems. METHODS: A survey was conducted using questionnaire developed by the study team based on literature review. The subjects were medical record administrators working at medical record department in general hospitals. RESULTS: The response rate was 59.8%(55/92). The functions which more than ninety percent of subjects responded as necessary right now and/or in near future related to 'drug alert', 'clinical guideline', 'chronic disease management', 'automated real-time surveillance', 'coded data', 'result reporting', 'de-identifying data', 'disease registry', and 'provider-provider communication and connectivity'. CONCLUSION: The results showed the high prioritized functions were decision support and health information/data management.
Electronic Health Records* ; Health Care Sector ; Hospitals, General* ; Humans ; Medical Record Administrators ; Medical Records ; Needs Assessment* ; Surveys and Questionnaires

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

Purpose: Surgeons can treat debilitating conditions of uncontrollable complex anorectal fistulas with sepsis, even after repeated fistula surgeries, for curative intention. Adipose-derived stem cells have shown good outcomes for refractory Crohn fistula. Unfortunately, cell therapy has some limitations, including high costs. We have therefore attempted immediate cell-assisted lipotransfer (CAL) in treating refractory complex anal fistulas and observed its outcomes. Methods: In a retrospective study, CAL, using a mixture of freshly extracted autologous stromal vascular fraction (SVF) and fat tissues, was used to treat 22 patients of refractory complex anal fistula from March 2018 to May 2021. Preoperative and postoperative assessments were performed with direct visual inspection, digital palpation, and endoanal ultrasonography. A fistula was considered completely healed if (1) the patient had no symptoms of discharge or inflammation; (2) there were no visible secondary openings of fistula tract inside and outside of the anorectal unit and even in the perineum; and (3) there was no primary opening in the anus. The endpoint of complete remission was wound healing without signs of inflammation 3 months after CAL treatment. Results: In a total of 22 patients who received CAL treatment, 19 patients showed complete remission, 1 patient showed partial improvement, and 2 patients showed no improvement. One of the 2 patients without improvement at primary endpoint showed complete remission 9 months after CAL. There were no significant adverse effects of the procedure. Conclusion We found that the immediately-collected CAL procedure for refractory complex anal fistula showed good outcomes without adverse side effects. It can be strongly recommended as an alternative surgical option for the treatment of complex anal fistula that is uncontrollable even after repeated surgical procedures. However, considering the unpredictable characteristics of SVF, long-term follow-up is necessary.

Purpose: This study investigated the incidence and risk factors of cataract in people with diabetes mellitus (DM) using data from Ansan cohort of the Korean Genome and Epidemiology Study (KoGES). Methods: Data from a total of 329 patients with type 2 DM without cataract who participated in Ansan cohort of the KoGES from baseline survey (2001–2002) to fifth follow-up visit (2011–2012) were examined.The characteristics of the subjects were analyzed with frequency and percentage, and mean and standard deviation. Cataract incidence was measured as incidence proportion (%). For risk factors of cataract, hazard ratio (HR) and 95% confidence interval (CI) were obtained using the Cox proportional hazard model. Results: The cataract incidence over a 10-year follow-up period was 19.1% (15.1 in males and 25.8 in females), and mean age at the incidence of cataract was 63.48 years (61.58 years in males and 65.31 years in females). Age (HR=1.09, 95% CI=1.05–1.13) and HbA1c (HR=1.21, 95% CI=1.07–1.37) or the duration of DM (HR=1.05, 95% CI=1.00–1.09) were found to be independently associated with cataract development. Conclusion Cataract development in people with DM is common, and its likelihood increases with age, HbA1c, and the duration of DM. Considering negative effect of cataract on their quality of life and economic burden, nurses should identify people with DM at a higher risk of cataract development, and plan individual eye examination programs to detect cataract development as early as possible.

OBJECTIVE: To compare the efficacy of suprapapillary and transpapillary meth-ods of transhepatic biliary metallic stent placement in malignant biliary strictures and to specify the indications of each method applied. MATERIALS AND METHODS: Stents were placed in 59 patients. Strictures were cat-egorized as type A (within 3 cm of the ampulla, n = 27), type B (over 3 cm from ampulla, n = 7), type C (within 3 cm of the bending portion, n = 9), or type D (over 3 cm above the bending portion, n=16). The stenting method was suprapapillary in 34 cases and transpapillary in 25. The rates of initial and long-term patency and of early recurrence were compared. RESULTS: Initial patency rates for the suprapapillary and transpapillary methods were 1/7 (14.3%) and 20/20 (100%) respectively for type A (p < 0.0001), 4/5 (80.0%) and 2/2 for type B, 3/7 (42.9%) and 2/2 for type C, and 15/16 (93.8%) and 0/0 for type D. Early recurrence rates were 7/30 (23.3%) using the suprapap-illary method and 4/29 (13.8%) using the transpapillary method (p = 0.51). The long-term patency rate did not differ significantly according to either type (p =0.37) or method (p = 0.62). CONCLUSION: For good initial patency, the transpapillary method is recommended for strictures of the distal extrahepatic duct near the ampulla and just above the bending portion. Long-term patency is not influenced by the stenting method employed.
Bile Duct Neoplasms/complications ; Bile Duct Obstruction, Extrahepatic/etiology/*therapy ; Cholangiography ; Comparative Study ; Digestive System Neoplasms/complications ; Female ; Human ; Male ; Middle Age ; Palliative Care/*methods ; *Radiology, Interventional ; Retrospective Studies ; *Stents ; Tomography, X-Ray Computed

OBJECTIVE: To compare the efficacy of suprapapillary and transpapillary meth-ods of transhepatic biliary metallic stent placement in malignant biliary strictures and to specify the indications of each method applied. MATERIALS AND METHODS: Stents were placed in 59 patients. Strictures were cat-egorized as type A (within 3 cm of the ampulla, n = 27), type B (over 3 cm from ampulla, n = 7), type C (within 3 cm of the bending portion, n = 9), or type D (over 3 cm above the bending portion, n=16). The stenting method was suprapapillary in 34 cases and transpapillary in 25. The rates of initial and long-term patency and of early recurrence were compared. RESULTS: Initial patency rates for the suprapapillary and transpapillary methods were 1/7 (14.3%) and 20/20 (100%) respectively for type A (p < 0.0001), 4/5 (80.0%) and 2/2 for type B, 3/7 (42.9%) and 2/2 for type C, and 15/16 (93.8%) and 0/0 for type D. Early recurrence rates were 7/30 (23.3%) using the suprapap-illary method and 4/29 (13.8%) using the transpapillary method (p = 0.51). The long-term patency rate did not differ significantly according to either type (p =0.37) or method (p = 0.62). CONCLUSION: For good initial patency, the transpapillary method is recommended for strictures of the distal extrahepatic duct near the ampulla and just above the bending portion. Long-term patency is not influenced by the stenting method employed.
Bile Duct Neoplasms/complications ; Bile Duct Obstruction, Extrahepatic/etiology/*therapy ; Cholangiography ; Comparative Study ; Digestive System Neoplasms/complications ; Female ; Human ; Male ; Middle Age ; Palliative Care/*methods ; *Radiology, Interventional ; Retrospective Studies ; *Stents ; Tomography, X-Ray Computed

PURPOSE: We studied the plasma neuron-specific enolase (NSE) and glutamic acid levels as a marker of the severity of acute ischemic stroke (AIS). METHODS: We enrolled 93 patients who visited to the emergency department from April to September, 2005. The AIS patients included those who visited the emergency department within 24 hours due to ischemic stroke symptoms. The AIS patients was subclassified according to large-vessel, small-vessel, cardioembolic, or unclassified infarction. RESULTS: The plasma NSE and glutamic acid level were 15.1+/-7.9 ng/ml and 204.5+/-86.5 nM/ml, respectively, in the AIS patients. Plasma NSE and Glutamic acid in the was higher than reference range (NSE 0-12 ng/ml, Glutamic acid 0-130 nM/ml). According to the type of infarction, no differences were observed in the plasma NSE and glutamic acid levels. CONCLUSION: In cases of AIS, NSE and glutamic acid have no statistical usefulness in classifying the type of infarction. However, the value of plasma NSE and glutamic acid levels have statistical usefulness in deciding on the existence or nonexistence of an AIS.
Cerebral Infarction ; Emergency Service, Hospital ; Glutamic Acid* ; Humans ; Infarction ; Phosphopyruvate Hydratase* ; Plasma* ; Reference Values ; Stroke*

BACKGROUND: The present study was performed to elicit what effect nalbuphine would have on isoflurane MAC (minimum alveolar concentration) in the patients undergoing lower abdominal surgery. METHODS: Sixty-two women were randomly allocated to one of five study groups to receive an intravenous injection of no nalbuphine (group I), 0.25 mg/kg (group II), 0.5 mg/kg (group III), 1.0 mg/kg (group IV), 1.5 mg/kg (group V). Anesthesia and tracheal intubation were induced with propofol 2 mg/kg, succinylcholine 1 mg/kg. Patients were inhaled at a preset end-tidal concentration of isoflurane, which was maintained for 20 min. Response to skin incision, movement or no movement, was determined 30 minutes after nalbuphine injection. The isoflurane concentration of the next patient in the same group moved up or down in steps of 0.1~0.3%, according to the previous patient's response. MAC was determined using the "up-down" method and logistic regression. RESULTS: The MAC's of isoflurane were 1.09 vol% end-tidal in the control group, 0.89 vol% in group II, 0.65 vol% in group III, 0.55 vol% in group IV, and 0.51 vol% in group V. CONCLUSIONS: It would be suggested that nalbuphine dose-dependently reduce the isoflurane MAC, and have ceiling effect on the reduction of isoflurane MAC.
Anesthesia ; Female ; Humans ; Injections, Intravenous ; Intubation ; Isoflurane* ; Logistic Models ; Nalbuphine* ; Propofol ; Skin ; Succinylcholine

PURPOSE: To report a case of Joubert syndrome associated with Leber's congenital amaurosis. CASE SUMMARY: A 7-month-old boy was referred for poor eye contact and esodeviation since birth. He could not fixate with his eyes or follow objects. Nystagmus in both eyes and moderate angle esotropia were present. Slit lamp examination and fundus examination showed no significant findings. Visual evoked potential and electroretinogram were extinguished in both eyes. The patient showed abnormal respiratory patterns, developmental delay, and hypotonia. Brain MRI showed aplasia of the cerebellar vermis, and he was diagnosed with Joubert syndrome. At 2 years of age, he was moderately able to fixate with his eyes and to crudely follow objects. Nystagmus and esotropia had diminished, but the visual evoked potential and electroretinogram still showed no response in both eyes. CONCLUSIONS: Leber's congenital amaurosis can be associated with Joubert syndrome, and children with Leber's congenital amaurosis should be evaluated for associated systemic abnormalities.
Blindness ; Brain ; Cerebellar Diseases ; Child ; Esotropia ; Evoked Potentials, Visual ; Eye ; Eye Abnormalities ; Humans ; Infant ; Kidney Diseases, Cystic ; Muscle Hypotonia ; Parturition ; Retina ; Spinocerebellar Degenerations

In order to identify the characteristic ocular findings in Down's syndrome(DS), 123 Korean children with DS prospectively underwent ocular examination, including visual acuity assessment, slit-lamp biomicroscopy, ocular motility, cycloplegic refraction, and ophthalmoscopy. The ocular findings in decreasing prevalence were the following:upward slanting of the palpebral fissure(78 patients, 63%), epicanthus(75 patients, 61%), epiblepharon(66 patients, 54%), astigmatism(38 patients, 31%), hyperopia(35 patients, 28%), myopia(31 patients, 25%), strabismus(31 patients, 25%, 18 esotropia and 13 exotropia), nystagmus(27 patients, 22%), nasolacrimal duct obstruction(21 patients, 17%), blepharoconjunctivitis(20 patients, 16%), retinal abnormalities(18 patients, 15%), cataract(4 patients, 13%), and glaucoma(1 patient, 0.8%). Brushfield spots and keratoconus were not found. The ocular findings in Korean children with DS are characterized as an unreported, high incidence of epiblepharon, the highest rate of exotropia as reported, and no cases of Brushfield spots. The difference in the incidence of these ocular abnormalities according to race and age should be considered in every patient with DS.
Child* ; Continental Population Groups ; Down Syndrome* ; Esotropia ; Exotropia ; Humans ; Incidence ; Keratoconus ; Nasolacrimal Duct ; Ophthalmoscopy ; Prevalence ; Prospective Studies ; Retinaldehyde ; Visual Acuity