Cerebral current-source distribution associated with pain improvement by non-invasive painless signaling therapy in patients with failed back surgery syndrome

Background@#Non-invasive painless signaling therapy (NPST) is an electro-cutaneous treatment that converts endogenous pain information into synthetic non-pain information. This study explored whether pain improvement by NPST in failed back surgery syndrome (FBSS) patients is related to cerebral modulation. @*Methods@#Electroencephalography (EEG) analysis was performed in 11 patients with FBSS. Subjects received daily NPST for 5 days. Before the first treatment, patients completed the Brief Pain Inventory (BPI) and Beck Depression Inventory and underwent baseline EEG. After the final treatment, they responded again to the BPI, reported the percent pain improvement (PPI), and then underwent post-treatment EEG. If the PPI grade was zero, they were assigned to the ineffective group, while all others were assigned to the effective group. We used standardized low-resolution brain electromagnetic tomography (sLORETA) to explore the EEG current-source distribution (CSD) associated with pain improvement by NPST. @*Results@#The 11 participants had a median age of 67.0 years, and 63.6% were female. The sLORETA images revealed a beta-2 CSD increment in 12 voxels of the right anterior cingulate gyrus (ACG) and the right medial frontal area. The point of maximal CSD changes was in the right ACG. The alpha band CSD increased in 2 voxels of the left transverse gyrus. @*Conclusions@#Pain improvement by NPST in FBSS patients was associated with increased cerebral activity, mainly in the right ACG. The change in afferent information induced by NPST seems to be associated with cerebral pain perception.

Cerebral current-source distribution associated with pain improvement by non-invasive painless signaling therapy in patients with failed back surgery syndrome

Background@#Non-invasive painless signaling therapy (NPST) is an electro-cutaneous treatment that converts endogenous pain information into synthetic non-pain information. This study explored whether pain improvement by NPST in failed back surgery syndrome (FBSS) patients is related to cerebral modulation. @*Methods@#Electroencephalography (EEG) analysis was performed in 11 patients with FBSS. Subjects received daily NPST for 5 days. Before the first treatment, patients completed the Brief Pain Inventory (BPI) and Beck Depression Inventory and underwent baseline EEG. After the final treatment, they responded again to the BPI, reported the percent pain improvement (PPI), and then underwent post-treatment EEG. If the PPI grade was zero, they were assigned to the ineffective group, while all others were assigned to the effective group. We used standardized low-resolution brain electromagnetic tomography (sLORETA) to explore the EEG current-source distribution (CSD) associated with pain improvement by NPST. @*Results@#The 11 participants had a median age of 67.0 years, and 63.6% were female. The sLORETA images revealed a beta-2 CSD increment in 12 voxels of the right anterior cingulate gyrus (ACG) and the right medial frontal area. The point of maximal CSD changes was in the right ACG. The alpha band CSD increased in 2 voxels of the left transverse gyrus. @*Conclusions@#Pain improvement by NPST in FBSS patients was associated with increased cerebral activity, mainly in the right ACG. The change in afferent information induced by NPST seems to be associated with cerebral pain perception.

Evaluating Postoperative Muscle Strength Using Surface Electromyography in Hip Fracture Patient

Background@#To compare the muscle strength of patients with a hip fracture according to the presence of sarcopenia after surgery and the correlation of measured values between a Biodex and surface electromyography (sEMG) in postoperative measurement of muscle strength. @*Methods@#Seventy-one patients who underwent hip fracture surgery were included in this study. Muscle mass was measured using dual energy X-ray absorptiometry and the grip strength was evaluated using a dynamometer. The diagnosis of sarcopenia followed the Asian Working Group for Sarcopenia criteria. We evaluated the Biodex to assess muscle strength according to the presence of sarcopenia and at the same time measured the sEMG to evaluate the correlation of muscle strength between Biodex and sEMG. @*Results@#We assigned 34 patients with sarcopenia and 37 without sarcopenia to 2 groups. In the comparison of muscle strength using Biodex and sEMG between the 2 groups, it was confirmed that muscle strength of sarcopenia group was decreased compared with that of the non-sarcopenia group, although there was no statistical significance between the groups. However, Biodex and sEMG showed very close correlation with muscle strength in all variables. @*Conclusions@#We suggest that using sEMG for the evaluation of muscle strength after hip fracture surgery may be an excellent tool alternative to isokinetic testing machines such as the Biodex.

Fall Risk Assessment of Rural Elderly Population in Korea

OBJECTIVE: To investigate the risk factors for fall in the elderly population residing in rural areas of Korea and provide useful data for their prevention. METHODS: As part of farmers’ health promotion project, a retrospective study was conducted with a total of 350 elderly people recruited from March 2016 to December 2016. These subjects were divided into two groups: 254 non-fallers and 96 fallers. A person who fell to the floor at least once in the past year was defined as a faller. Participants were asked to visit the hospital once. The demographic characteristics, social environment, and educational levels were surveyed using a questionnaire. Physical examination was performed in the following order: cognitive function, lower leg strength and torque, body composition, and knee image test. RESULTS: Statistically significant factors for falls in univariate analysis were female gender, age, living alone, educational level less than middle school, skeletal muscle mass, Mini-Mental State Exam, knee osteoarthritis, hip torque, hip power mean, knee torque, and knee power mean. Multivariate analysis was performed to identify variables most relevant to falls among statistically significant factors in univariate logistic analysis. It was confirmed that female gender and age of 70–79 years were statistically significant factors related to falls. CONCLUSION: Female gender and elderly status (70–79 years) are important risk factors for falls in rural areas underscoring the need for special attention when considering risk factors for falls among the elderly living in rural areas.

Measurement of Uncertainty Using Standardized Protocol of Hand Grip Strength Measurement in Patients with Sarcopenia

BACKGROUND: The aim of this study was to determine the accuracy and error range of hand grip strength measurement using various methods. METHODS: Methods used for measurement of hand grip strength in 34 epidemiologic studies on sarcopenia were analyzed. Maximum grip strength was measured in a sitting position with the elbow flexed at 90 degrees, the shoulder in 0 degrees flexion, and the wrist in neutral position (0 degrees). Maximum grip strength in standing position was measured with the shoulder in 180 degrees flexion, the elbow fully extended, and the wrist in neutral position (0 degrees). Three measurements were taken on each side at 30 sec intervals. The uncertainty of measurement was calculated. RESULTS: The combined uncertainty in sitting position on the right and left sides was 1.14% and 0.38%, respectively, and the combined uncertainty in standing position on the right and left sides was 0.35 and 1.20, respectively. The expanded uncertainty in sitting position on the right and left sides was 2.28 and 0.79, respectively, and the expanded uncertainty in standing position on the right and left sides was 0.71 and 2.41, respectively (k=2). CONCLUSIONS: Uncertainty of hand grip strength measurement was identified in this study, and a significant difference was observed between measurement. For more precise diagnosis of sarcopenia, dynamometers need to be corrected to overcome uncertainty.

Dentinal Tubules Occluding Effect Using Nonthermal Atmospheric Plasma

Nonthermal atmospheric plasma has been studied for its many biomedical effects, such as tooth bleaching, wound healing, and coagulation. In this study, the effects of dentinal tubules occlusion were investigated using fluoride-carboxymethyl cellulose (F-CMC) gel, nano-sized hydroxyapatite (n-HA), and nonthermal atmospheric plasma. Human dentin specimens were divided to 5 groups (group C, HA, HAF, HAP, and HAFP). Group HA was treated with n-HA, group HAF was treated with n-HA after a F-CMC gel application, group HAP was treated with n-HA after a plasma treatment and group HAFP was treated with n-HA after a plasma and F-CMC gel treatment. The occlusion of dentinal tubules was investigated using scanning electron microscopy (SEM) and energy dispersive x-ray spectroscopy (EDS), which shows Ca/P ratio. In the EDS results, a higher Ca/P ratio was shown in the groups including n-HA than in the control group. The specimens of group HAP and HAFP had a higher Ca/P ratio in retentivity. In the SEM results, there was not a significant difference in the amount of times applied. Therefore, this study suggests F-CMC gel and n-HA treatment using nonthermal atmospheric plasma will be a new treatment method for decreasing hypersensitivity.

Change of Femoral Anteversion Angle in Children With Intoeing Gait Measured by Three-Dimensional Computed Tomography Reconstruction: One-Year Follow-Up Study

OBJECTIVE: To evaluate femoral anteversion angle (FAA) change in children with intoeing gait depending on age, gender, and initial FAA using three-dimensional computed tomography (3D-CT). METHODS: The 3D-CT data acquired between 2006 and 2016 were retrospectively reviewed. Children 4 to 10 years of age with symptomatic intoeing gait with follow-up interval of at least 1 year without active treatment were enrolled. Subjects were divided into three groups based on age: group 1 (≥4 and <6 years), group 2 (≥6 and <8 years), and group 3 (≥8 and <10 years). Initial and follow-up FAAs were measured using 3D-CT. Mean changes in FAAs were calculated and compared. RESULTS: A total of 200 lower limbs of 100 children (48 males and 52 females, mean age of 6.1±1.6 years) were included. The mean follow-up period was 18.0±5.4 months. Average initial and follow-up FAA in children with intoeing gait was 31.1°±7.8° and 28.9°±8.2°, respectively. The initial FAA of group 1 was largest (33.5°±7.7°). Follow-up FAA of group 1 was significantly reduced to 28.7°±9.2° (p=0.000). FAA changes in groups 1, 2, and 3 were −6.5°±5.8°, −6.4°±5.1°, and −5.3°±4.0°, respectively. These changes of FAA were not significantly (p=0.355) different among the three age groups. However, FAA changes were higher (p=0.012) in females than those in males. In addition, FAA changes showed difference depending on initial FAA. When initial FAA was smaller than 30°, mean FAA change was −5.6°±4.9°. When initial FAA was more than 30°, mean FAA change was −6.8°±5.4° (p=0.019). CONCLUSION: FAA initial in children with intoeing gait was the greatest in age group 1 (4–6 years). This group also showed significant FAA decrease at follow-up. FAA changes were greater when the child was a female, younger, and had greater initial FAA.

Collet-Sicard Syndrome With Hypoglossal Nerve Schwannoma: A Case Report

Collet-Sicard syndrome is a rare syndrome that involves paralysis of 9th to 12th cranial nerves. We report an uncommon case of schwannoma of the hypoglossal nerve in a 39-year-old woman presented with slurred speech, hoarse voice, and swallowing difficulty. Physical examination revealed decreased gag reflex on the right side, decreased laryngeal elevation, tongue deviation to the right side, and weakness of right trapezius muscle. MRI revealed a mass lesion in the right parapharyngeal space below the jugular foramen. The tumor was surgically removed. It was confirmed as hypoglossal nerve schwannoma via pathologic examination. Videofluoroscopic swallowing study revealed aspiration of liquid food and severe bolus retention in the vallecula and piriform sinus. Laryngoscopy revealed right vocal cord palsy. Electrodiagnostic study revealed paralysis of the right 11th cranial nerve. In summary, we report an uncommon case of schwannoma of the hypoglossal nerve with 9th to 12th cranial nerve palsy presenting as Collet-Sicard syndrome.

Prevalence and Related Characteristics of Carpal Tunnel Syndrome Among Orchardists in the Gyeongsangnam-do Region

OBJECTIVE: To determine the prevalence and related characteristics of carpal tunnel syndrome (CTS) in orchardists and to investigate the association between electrodiagnostic severity and physical examinations. METHODS: Between July 2013 and September 2014, 377 subjects (174 men and 203 women) visited the Gyeongsang National University Hospital's Center for Farmer's Safety and Health. All the subjects underwent electrodiagnostic tests and physical examination, including Phalen's test, Tinel's sign, and Durkan's carpal compression test (CCT). The subjects were classified into 2 groups, the normal group and the CTS group, according to electrodiagnostic test results. To determine the related characteristics of CTS, potential variables, including age, sex, drinking, smoking, body mass index, waist circumference, and total work time, were compared between the 2 groups. The association between electrodiagnostic severity and physical examinations was analyzed. RESULTS: CTS was diagnosed in 194 subjects based only on electrodiagnostic test results, corresponding to a prevalence of 51.5%. Among the variables, mean age (p=0.001) and total work time (p=0.007) were significantly correlated with CTS. With respect to the physical examinations, low specificities were observed for Tinel's sign, Phalen's test, and Durkan's CCT (38.4%, 36.1%, and 40.9%, respectively) in the subjects aged ≥65 years. In addition, Phalen's test (p=0.003) and Tinel's sign (p=0.032) in men and Durkan's CCT (p=0.047) in women showed statistically significant differences with increasing CTS severity. The odds ratio was 2.066 for Durkan's CCT in women according to the multivariate logistic regression analysis. CONCLUSION: CTS prevalence among orchardists was high, and Durkan's CCT result was significantly quantitatively correlated with the electrodiagnostic test results. Therefore, Durkan's CCT is another reliable examination method for CTS.

Ureteral Rupture Caused by a Suprapubic Catheter in a Male Patient With Spinal Cord Injury: A Case Report

Spinal cord injury (SCI) may lead to urinary system disturbances. Patients with SCI usually have neurogenic bladder, and treatment optionss for this condition include clean intermittent catheterization and a permanent indwelling urethral or suprapubic catheter. Complications of catheterization include urinary tract infection, calculi, urinary tract injury, bladder contraction, bladder spasm, renal dysfunction, bladder cancer, and so forth. To the best of our knowledge, ureteral rupture is an unusual complication of catheterization, and ureteral rupture has been rarely reported in SCI patients. Therefore, here we report a case of ureteral rupture caused by a suprapubic catheter used for the treatment of neurogenic bladder with vesicoureteral reflux in a male patient with SCI. Due to SCI with neurogenic bladder, ureteral size can be reduced and the suprapubic catheter tip can easily migrate to the distal ureteral orifice. Thus, careful attention is required when a catheter is inserted into the bladder in patients with SCI.

Usefulness of a Hanging Position With Internal Rotation of Shoulder in Ultrasonography-Guided Intra-articular Steroid Injection for Adhesive Capsulitis

OBJECTIVE: To evaluate the feasibility of a new position (internal rotation in hanging) in ultrasonography, we compared the length of the glenohumeral joint space and the effectiveness of steroid injection with the hanging position and with the commonly used abdomen or cross position. METHODS: A prospective, randomized controlled trial was performed in 42 patients with adhesive capsulitis of shoulder. We used three arm positions for the posterior approach as follows: the patient's palm on thigh, other hand on abdomen (abdomen position); hand on patient's opposite shoulder (cross position); arm in hanging position with internal rotation of shoulder (hanging position). The order of shoulder position was randomized and blinded. Real-time ultrasonography-guided intra-articular steroid injection was performed by posterior approach at the first position in each patient. The Brief Pain Inventory (BPI), the Shoulder Pain and Disability Index (SPADI), and range of motion (ROM) were measured before steroid injection and 2 weeks after injection. RESULTS: The lengths of the joint space were 2.88±0.75, 2.93±0.89, and 2.82±0.79 mm in abdomen, cross, and hanging position respectively, with no significant difference among the three positions (p=0.429). Treatment efficacy was significantly improved in ROM, total BPI, and SPADI in all three positions (p<0.001). The changes in ROM for shoulder abduction were 23.6°±19.7°, 22.2°±20.9°, and 10.0°±7.8° in abdomen, cross, and hanging position, respectively. Changes in total BPI scores were 25.1±15.7, 23.6.±18.0, 11.6±6.1, and changes in total SPADI score were 35.0±14.2, 30.9±28.9, and 16.5±10.3 in abdomen, cross, and hanging position, respectively. There were no significant difference among the three positions for all parameters (p=0.194, p=0.121, and p=0.108, respectively. CONCLUSION: For patients with adhesive capsulitis who cannot achieve or maintain abdomen or cross position, scanning and injection with the shoulder in internal rotation with hanging position may be a useful alternative.

Usefulness of a Hanging Position With Internal Rotation of Shoulder in Ultrasonography-Guided Intra-articular Steroid Injection for Adhesive Capsulitis

OBJECTIVE: To evaluate the feasibility of a new position (internal rotation in hanging) in ultrasonography, we compared the length of the glenohumeral joint space and the effectiveness of steroid injection with the hanging position and with the commonly used abdomen or cross position. METHODS: A prospective, randomized controlled trial was performed in 42 patients with adhesive capsulitis of shoulder. We used three arm positions for the posterior approach as follows: the patient's palm on thigh, other hand on abdomen (abdomen position); hand on patient's opposite shoulder (cross position); arm in hanging position with internal rotation of shoulder (hanging position). The order of shoulder position was randomized and blinded. Real-time ultrasonography-guided intra-articular steroid injection was performed by posterior approach at the first position in each patient. The Brief Pain Inventory (BPI), the Shoulder Pain and Disability Index (SPADI), and range of motion (ROM) were measured before steroid injection and 2 weeks after injection. RESULTS: The lengths of the joint space were 2.88±0.75, 2.93±0.89, and 2.82±0.79 mm in abdomen, cross, and hanging position respectively, with no significant difference among the three positions (p=0.429). Treatment efficacy was significantly improved in ROM, total BPI, and SPADI in all three positions (p<0.001). The changes in ROM for shoulder abduction were 23.6°±19.7°, 22.2°±20.9°, and 10.0°±7.8° in abdomen, cross, and hanging position, respectively. Changes in total BPI scores were 25.1±15.7, 23.6.±18.0, 11.6±6.1, and changes in total SPADI score were 35.0±14.2, 30.9±28.9, and 16.5±10.3 in abdomen, cross, and hanging position, respectively. There were no significant difference among the three positions for all parameters (p=0.194, p=0.121, and p=0.108, respectively. CONCLUSION: For patients with adhesive capsulitis who cannot achieve or maintain abdomen or cross position, scanning and injection with the shoulder in internal rotation with hanging position may be a useful alternative.

Ultrasonographic Diagnosis of Non-displaced Avulsion Fracture of the Acromion: A Case Report

Avulsion fracture of the acromion is rare. It is difficult to diagnosis because there is little displacement and it occurs even without direct trauma. We experienced a case without direct trauma that was diagnosed with ultrasonography. A 55-year-old male patient visited our outpatient clinic with shoulder pain resulting from a significant stress at the trapezius muscle during lifting of a steel reinforcement. Simple radiography revealed a calcific deposit over the acromion rather than a fracture. Avulsion fracture was identified with ultrasonography. This is the first report demonstrating that ultrasonography has an advantage over radiographs in the diagnosis of an avulsion fracture of the acromion of the scapula.

A Case of Paraplegia Associated with Intrathecal Methotrexate : A case report

The treatment and prophylactic therapy of meningeal leukemia with intrathecal methotrexate in acute lymphoblastic leukemia is the standard method. Intrathecal overdose of methotrexate may produce severe toxicities such as paraplegia. We experienced a case of paraplegia diagnosed by magnetic imaging examination of the spine in a 24-year-old woman received repeated intrathecal methotrexate for meningeal leukemia.

Clinical Characteristics of Retinal Arterial Macroaneurysms

PURPOSE: To determine the clinical features of retinal arterial macroaneurysm (RAM). METHODS: Clinical characteristics of RAM, and its managements and results were evaluated. RESULTS: Of a total of 17 patients (17 eyes), 11 patients were women and 12 were over seventh decade. Mean follow-up period was 9.2 months. Hypertension was associated in 6 patients. Single aneurysm was temporally located in 15 eyes, which was along the temporal vascular arcade. It was on the arteriovenous crossing site in 8 eyes and followed retinal branch vein occlusion in 4 eyes. Aneurysms were located nasally in 2 eyes. Type was hemorrhagic in 15 eyes and exudative in 2 eyes. Pars plana vitrectomy was performed in 5 eyes and intraocular gas was injected in 2 eyes. Photocoagulation was performed directly in 2 eyes and indirectly in 3 eyes. Five eyes received no special managements. Visual acuity was less than 0.1 in 11 eyes and 0.3 or better in 4 on the initial visit, and less than 0.1 in 1 eyes and 0.3 or better in 10 on the final visit. Twelve eyes showed visual improvement of 2 lines or more. CONCLUSIONS: RAM usually develops along the temporal vascular arcade and induces visual disturbance due to hemorrhage and exudate. However, visual prognosis is relatively good.

Phacofragmentation for the treatment of a completely posterior dislocation of the total crystalline lens

In order to evaluate the effectiveness and safety of intravitreal phacofragmentation, we analyzed cases of pars plana vitrectomy (PPV) with intravitreal phacofragmentation and scleral fixation of an intraocular lens (IOL) performed in patients with a crystalline lens completely dislocated into the vitreous cavity without any damage to the lens capsule. Of the 23 eyes examined, the dislocated lens was related with a hypermature cataract in 4 eyes and Marfan syndrome in 2 eyes, developed after argon and Nd:YAG laser iridotomy in 2 eyes, and was due to ocular trauma in 15 eyes. The dislocated lens was present for more than a year in 6 eyes and less than 4 weeks in 13 eyes. PPV, intravitreal phacofragmentation and scleral fixation of IOL were performed in all 23 eyes. Additionally, trabeculectomy was performed in 4 eyes, iridoplasty in 1 eye and cryopexy with intravitreal injection of sulfur hexafluoride in 2 eyes. Perfluorocarbon liquid was used in 9 eyes. The mean follow-up period was 11.7 months. On final examination, the visual acuity was better than 20/40 in 17 eyes (73.9%) and counting fingers in 1 eye due to traumatic optic neuropathy. There was no postoperative retinal detachment. These results demonstrated that PPV with intravitreal phacofragmentation and other combined procedures is a safe and effective surgical method for treating a completely dislocated crystalline lens.

Strabismus and Refractive Errors in Cerebral Palsy Patients

OBJECTIVE: The purpose of this study was to compare the patients in the rehabilitation center with the patients who had visited the Department of Ophthalmology of Chonnam University Hospital to investigate incidence and type of ocular abnormalities in cerebral palsy patients. METHODS: We compared the seventy patients who had visited the Department of Ophthalmology of Chonnam University Hospital from January 1990 to July 2000 with one hundred and six patients in the rehabilitation center, and analyzed the incidence and type of ocular abnormalities between the two groups of cerebral palsy patients. RESULTS: In rehabilitation center group, spastic type was 51 patients among 90 patients (56.7%). In outpatient group, spastic type was 48 patients among 52 patients (92.3%). In rehabilitation center group, strabismus was detected in 35 patients among 106 patients (33.0%). In outpatient group, strabismus was detected in 31 patients among 70 patients (44.3%). In rehabilitation center group, refractive errors were detected in 27 patients among 85 patients (31.8%). In outpatient group, refractive errors were detected in 28 patients among 60 patients (46.7%). CONCLUSION: We concluded that because of the high incidence of ocular abnormalities in both rehabilitation center and outpatient groups, early detection through routine screening and early treatment for the ocular abnormalities of the cerebral palsy patients would be needed.

A Case of Hemolytic Disease in the Newborn Due to Anti-Dia Antibody

The Diego blood group is expressed as Dia antigen and Dib antigen. The frequency of Dia antigen expression in Korean is estimated to be 6-15%. We report the first case of severe hemolytic disease caused by anti-Dia antibody in Korea. Full-term male infant was admitted due to hyperbilirubinemia on the first day of life. Total bilirubin level was elevated to 20 mg/dL at 20 hours of age and exchange transfusion was performed. Total bilirubin decreased to 12.7 mg/dL after exchange transfusion, but increased to 19.0 mg/ dL at 43 hours of age. After second exchange transfusion, total bilirubin was 6.0 mg/dL. The blood group of mother was type A, Rh D (+ and that of newborn was type O, Rh D (+. The direct antiglobulin test showed strong positive, and anti-Dia antibody was eluted from the baby's red blood cell. Due to higher incidence of Dia antigen expression in oriental population, we suggest that the Diego positive cell panel should be included in the diagnosis of hemolytic disease of the newborn.

A Case of Clostridium difficile Pseudomembranous Colitis / 대한소아소화기영양학회지

Pseudomembranous colitis, thought to be uncommon in children, is a bacterial, toxin-mediated inflammatory process resulting in acute or chronic diarrhea and is characterized by colonic pseudomembranes. It is mediated by toxins produced by Clostridium difficile and is increasingly recognized in pediatric population. Diagnosis is based on positive culture of C. difficile in selective media and positive test of C. difficile toxin. Oral metronidazole or vancomycin are the main treatment options but avoidance of further antibiotics should also be encouraged where possible. We have experienced a case of pseudomembranous colitis in a 4-year-old female presented with septic shock and colitis. This case was diagnosed with positive test of C. difficile toxin B and confirmed by isolation of the organism on cultire in selective media. Symptoms have been ameliorated by discontinuation of antibiotics and administration of metronidazole and oral vancomycin, and ICU care.

Two Cases of ARC (Arthrogryposis, renal dysfunction and cholestasis) Syndrome / 대한소아소화기영양학회지

ARC (arthrogryposis multiplex congrnita, renal dysfunction and cholestasis) syndrome was first described by Lutz-Richner and Landolt in 1973 and after then similar cases were scarcely reported worldwide. There's no reported case in Korea. It was first suggested that ARC syndrome was inherited by X-linked recessive trait but now it was thought to be inherited by autosomal recessive trait also. Most parents were consanguineous but some unconsanuineous parents were also reported. We report on 1 male and 1 female infant who had ARC syndrome from 2 unrelated families. They were born from unconsanguineous parents.