Chromosomal Microarray Analysis as a First-Tier Clinical Diagnostic Test in Patients With Developmental Delay/Intellectual Disability, Autism Spectrum Disorders, and Multiple Congenital Anomalies: A Prospective Multicenter Study in Korea

BACKGROUND: To validate the clinical application of chromosomal microarray analysis (CMA) as a first-tier clinical diagnostic test and to determine the impact of CMA results on patient clinical management, we conducted a multicenter prospective study in Korean patients diagnosed as having developmental delay/intellectual disability (DD/ID), autism spectrum disorders (ASD), and multiple congenital anomalies (MCA). METHODS: We performed both CMA and G-banding cytogenetics as the first-tier tests in 617 patients. To determine whether the CMA results directly influenced treatment recommendations, the referring clinicians were asked to complete a 39-item questionnaire for each patient separately after receiving the CMA results. RESULTS: A total of 122 patients (19.8%) had abnormal CMA results, with either pathogenic variants (N=65) or variants of possible significance (VPS, N=57). Thirty-five well-known diseases were detected: 16p11.2 microdeletion syndrome was the most common, followed by Prader-Willi syndrome, 15q11-q13 duplication, Down syndrome, and Duchenne muscular dystrophy. Variants of unknown significance (VUS) were discovered in 51 patients (8.3%). VUS of genes putatively associated with developmental disorders were found in five patients: IMMP2L deletion, PTCH1 duplication, and ATRNL1 deletion. CMA results influenced clinical management, such as imaging studies, specialist referral, and laboratory testing in 71.4% of patients overall, and in 86.0%, 83.3%, 75.0%, and 67.3% of patients with VPS, pathogenic variants, VUS, and benign variants, respectively. CONCLUSIONS: Clinical application of CMA as a first-tier test improves diagnostic yields and the quality of clinical management in patients with DD/ID, ASD, and MCA.

Effects of Anticonvulsant Monotherapy on Bone Mineral Density in Children / 대한소아신경학회지

PURPOSE: The use of anticonvulsants can cause side effects such as reduction of bone mineral density, requiring attention in growing children. The aim of our study is to investigate the effects of different anticonvulsants on bone mineral density in epileptic patients treated with monotherapy. METHODS: We retrospectively reviewed medical records of 60 subjects who visited the Pediatric Epilepsy Clinic of Bucheon St. Mary's Hospital from January 2013 to December 2017. Bone mineral density was measured with dual photon absorptiometry every 6 months. RESULTS: The number of patients treated with oxcarbazepine, valproate and levetiracetam was 31, 16 and 13, respectively. Reduction of bone mineral density was seen in 8 out of 31 patients (25.8%, P=0.10) treated with oxcarbazepine, 9 out of 16 patients treated with valproate (56.3%, P=0.04) and 4 out of 13 patients treated with levetiracetam (30.8%, P=0.50). CONCLUSION: There was a significant reduction of bone mineral density in patients treated with valproate compared to the other anticonvulsants in our study. We believe attention to bone mineral density is required in children treated with anticonvulsants.

The Effectiveness of Community-based Muscle and Joint Self Management Program for Older Adults

PURPOSE: The aim of this study was to determine the effectiveness of a community-based muscle and joint self-management program with muscle stretching and muscle strengthening exercises for community-dwelling older adults. METHODS: The study was a pre-and-post design in a single group, which examined the effects after the intervention of muscle and joint self-management program for 6 weeks, on the 42 subjects of the elderly who registered in a Community Senior Center in J city. Data analyses were conducted with paired t-test by using a SAS (version 9.2 for Windows) program. RESULTS: The effectiveness of muscle and joint self-management program on shoulder flexibility and right knee extension (flexibility) were significantly improved (t=2.72, p=.010; t=−2.26, p=.029). Joint symptoms (pain, stiffness), physical functioning, depression, fatigue, and left knee extension were not significantly improved after the muscle and joint self-management program. CONCLUSION: The results showed the possibility of this 6-week exercise program in improving shoulder and knee flexibility for community-dwelling older adults.

Effect of Watching 3-Dimensional Television on Refractive Error in Children

PURPOSE: To investigate the effect of watching 3-dimensional (3D) television (TV) on refractive error in children. METHODS: Sixty healthy volunteers, aged 6 to 12 years, without any ocular abnormalities other than refractive error were recruited for this study. They watched 3D TV for 50 minutes at a viewing distance of 2.8 meters. The image disparity of the 3D contents was from -1 to 1 degree. Refractive errors were measured both before and immediately after watching TV and were rechecked after a 10-minute rest period. The refractive errors before and after watching TV were compared. The amount of refractive change was also compared between myopes and controls. The refractive error of the participants who showed a myopic shift immediately after watching TV were compared across each time point to assure that the myopic shift persisted after a 10-minute rest. RESULTS: The mean age of the participants was 9.23 ± 1.75 years. The baseline manifest refractive error was -1.70 ± 1.79 (-5.50 to +1.25) diopters. The refractive errors immediately after watching and after a 10-minute rest were -1.75 ± 1.85 and -1.69 ± 1.80 diopters, respectively, which were not different from the baseline values. Myopic participants (34 participants), whose spherical equivalent was worse than -0.75 diopters, also did not show any significant refractive change after watching 3D TV. A myopic shift was observed in 31 participants with a mean score of 0.29 ± 0.23 diopters, which resolved after a 10-minute rest. CONCLUSIONS: Watching properly made 3D content on a 3D TV for 50 minutes with a 10-minute intermission at more than 2.8 meters of viewing distance did not affect the refractive error of children.

Neonatal Meningoencephalitis caused by Herpes Simplex Virus Type 2 / 소아감염

Despite its rare occurrence, early diagnosis and appropriate treatment for neonatal herpes simplex virus infection are mandatory due to its high morbidity and mortality. In Korea, there has been no epidemiologic data on neonatal herpes simplex virus infection, and even case reports are rare. We observed a 16-day-old neonate who presented with fever and seizures. We diagnosed her with meningoencephalitis caused by herpes simplex virus type 2 based on the polymerase chain reaction test, and treated her with intravenous acyclovir and anticonvulsants. The seroprevalence of herpes simplex virus type 2 sharply increases in women in their 30s, and the average age for childbirth has increased to older than 30 years of age in Korea; we therefore expect that the incidence of neonatal herpes simplex virus type 2 infection will rise in Korea, and more attention should be directed to neonatal herpes simplex virus type 2 infection. We report this newborn patient's case along with a literature review.

Clinical Characteristics of Attention Deficit Hyperactivity Disorder in Childhood Malignant Hematologic Diseases

PURPOSE: The purpose of this study is to identify the prevalence of attention-deficit hyperactivity disorder (ADHD) among children treated for childhood malignant hematologic diseases, and determine the different characteristics of the patients with ADHD as opposed to the patients without ADHD, which will help to predict the development of ADHD and to help treat them. METHODS: 172 patients diagnosed as childhood malignant hematologic diseases went through the test battery including diagnostic criteria for ADHD in pediatric neurology department of Seoul St. Mary's hospital from March 2009 to May 2012. Age, sex, ADHD type, hematologic diseases type, age at onset of hematologic diseases, treatment with/without chemotherapy and relapse were investigated. These data were compared between the groups of the patients without ADHD and with ADHD. RESULTS: Out of 172 patients, Fifty one patients (29.6%) had both ADHD and childhood malignant hematologic diseases. Sex (male), age at onset of hematologic diseases ( or =11 years (P=0.007). The patients with chemotherapy had about 3.4 fold increased risk for ADHD compared to the patients without chemotherapy (P=0.023). CONCLUSION: This study showed childhood malignant hematologic disorders has significant correlation with ADHD. In addition, Male, < or =5 years of age at onset and use of chemotherapy in the patients can be necessary to predict ADHD. Therefore, early detection and establishment of the countermeasures for ADHD are necessary.

Outcomes of the Monoconversion to Valproate or Lamotrigine for Absence Seizures

PURPOSE: Ethosuximide (ESX) is currently not available due to various reasons in Korea. The aim of this study is to compare the efficacy of valproate (VPA) and lamotrigine (LTG) when ESX monotherapy was replaced by VPA or LTG. METHODS: A retrospective study was done for a total of 34 patients treated with ESX in 5 different hospitals affiliated with Catholic University of Korea from January, 2010 to December, 2012. They all were initially treated with ESX, but later switched to VPA or LTG. The subjects were selected based on clinical symptoms and electroencephalography findings. RESULTS: Among 34 patients, VPA was prescribed to 17 patients (50.0%) and LTG to 17 patients (50.0%). Twenty patients (58.8%) achieved the seizure freedom after 3 months of the treatments, 13 patients (76.5%) by VPA and 7 (41.2%) by LTG respectively. Four patients (23.5%) with VPA and 10 (58.8%) with LTG were replaced by other anticonvulsants due to ineffectiveness and/or side effects of medication. When we compare the efficacy of seizure reduction between VPA and LTG after 3 month period of the treatment, the efficacy of VPA was better than that of LTG (P=0.04). CONCLUSION: The results of this study suggest that the VPA is a better alternative anticonvulsant than LTG for the patients with absence epilepsy who are unable to continue ESX.

The Impact of Anticonvulsants on Neurologic Outcomes in Neonatal Seizures / 대한주산의학회잡지

PURPOSE: Although neonatal seizures can cause epilepsy, neurodevelopmental disability, and mortality with high frequency, the use of anti-epileptic drug is limited and the side effect of the drug is unidentified. Thus, authors investigated the prognosis of the neonatal seizures related with anti-epileptic drugs and electroencephalography. METHODS: Retrospective medical records of 37 infants with neonatal seizures under 44 weeks of gestational age who were hospitalized at neonatal intensive care unit in Seoul St. Mary' Hospital from January to June 2012 were analyzed. RESULT: The mean gestational age was 32.5+/-1.9 weeks and the mean birth weight was 2,010+/-82 g. Seizures occurred in 65% in infants within 7 days of birth and subtle seizures were most common type. Hypoxic ischemic encephalopathy was the most common cause of seizures and 62% showed abnormal electrographic findings. Among 37 of patients, seizures of 57% were able to be controlled by levetiracetam, but 19% were controlled by co-administration of more than three anti-epileptic drugs. After 6 months of treatment, epilepsy was diagnosed in 6% and developmental delay occurred in 19% among patients controlled by one anti-epileptic drug. Epilepsy and developmental delay occurred in 29% and 86%, respectively, among patients with more than three anti-epileptic drugs. In addition 86% of patients with normal electroencephalographic findings show normal development, but 3 patients with severe abnormalities showed abnormal development. CONCLUSION: Neurologic outcome was not good when the number of anti-epileptic drugs were added due to uncontrolled seizures, and the result of electroencephalography showed severe abnormalities.

Characteristics of Children with Epilepsy and Concomitant Attention-Deficit Hyperactivity Disorder

PURPOSE: To investigate the prevalence of ADHD (attention-deficit hyperactivity disorder) in children with epilepsy and to determine the difference of characteristics in patients with epilepsy and concomitant ADHD as opposed to the patients without ADHD for better management. METHODS: We retrospectively reviewed 184 patients diagnosed as epilepsy and treated with antiepileptic drugs in pediatric neurology department of Seoul St. Mary's hospital from March, 2009 to May, 2012. Their ages ranged from 6 to 18 years. The subjects were included in the study those who made a regular visit for more than a year. RESULTS: 1) Out of 184 patients, 69 patients (37.5%) had both ADHD and epilepsy. 2) In epilepsy children with ADHD, male outnumbered female by almost two fold (male 67: female 33) (P=0.022). 3) In epilepsy children with ADHD, epileptiform discharges on EEG was focused in central regions in 39% of them (P=0.014). 4) In 56% of patients without ADHD, their seizures remained under the control with single anticonvulsant, as opposed to 36% of patients with both ADHD and epilepsy (P=0.001). Therefore, the presence of ADHD in patients with epilepsy might be related to the therapeutic response to anticonvulsants, and be a useful predictive factor for the response to early treatment. CONCLUSION: Patients with epilepsy and concomitant ADHD showed a significant difference and poor response to epilepsy treatment, as opposed to patients without ADHD. Therefore, early detection and establishment of countermeasures for ADHD is necessary.

Outcome of allogeneic hematopoietic stem cell transplantation for childhood acute lymphoblastic leukemia in second complete remission: a single institution study / 소아과

PURPOSE: The survival rate for childhood acute lymphoblastic leukemia (ALL) has improved significantly. However, overall prognosis for the 20 to 25% of patients who relapse is poor, and allogeneic hematopoietic stem cell transplantation (HSCT) offers the best chance for cure. In this study, we identified significant prognostic variables by analyzing the outcomes of allogeneic HSCT in ALL patients in second complete remission (CR). METHODS: Fifty-three ALL patients (42 men, 79%) who received HSCT in second CR from August 1991 to February 2009 were included (26 sibling donor HSCTs, 49%; 42 bone marrow transplantations, 79%). Study endpoints included cumulative incidence of acute and chronic graft-versus-host disease (GVHD), relapse, 1-year transplant-related mortality (TRM), disease-free survival (DFS), and overall survival (OS). RESULTS: Cumulative incidences of acute GVHD (grade 2 or above) and chronic GVHD were 45.3% and 28.5%, respectively. The estimated 5-year DFS and OS for the cohort was 45.2+/-6.8% and 48.3+/-7%, respectively. Only donor type, i.e., sibling versus unrelated, showed significant correlation with DFS in multivariate analysis (P=0.010). The rates of relapse and 1 year TRM were 28.9+/-6.4% and 26.4+/-6.1%, respectively, and unrelated donor HSCT (P=0.002) and HLA mismatch (P=0.022) were significantly correlated with increased TRM in univariate analysis. CONCLUSION: In this single institution study spanning more than 17 years, sibling donor HSCT was the only factor predicting a favorable result in multivariate analysis, possibly due to increased TRM resulting from unrelated donor HSCT.

Outcome of allogeneic hematopoietic stem cell transplantation for childhood acute lymphoblastic leukemia in second complete remission: a single institution study / 소아과

PURPOSE: The survival rate for childhood acute lymphoblastic leukemia (ALL) has improved significantly. However, overall prognosis for the 20 to 25% of patients who relapse is poor, and allogeneic hematopoietic stem cell transplantation (HSCT) offers the best chance for cure. In this study, we identified significant prognostic variables by analyzing the outcomes of allogeneic HSCT in ALL patients in second complete remission (CR). METHODS: Fifty-three ALL patients (42 men, 79%) who received HSCT in second CR from August 1991 to February 2009 were included (26 sibling donor HSCTs, 49%; 42 bone marrow transplantations, 79%). Study endpoints included cumulative incidence of acute and chronic graft-versus-host disease (GVHD), relapse, 1-year transplant-related mortality (TRM), disease-free survival (DFS), and overall survival (OS). RESULTS: Cumulative incidences of acute GVHD (grade 2 or above) and chronic GVHD were 45.3% and 28.5%, respectively. The estimated 5-year DFS and OS for the cohort was 45.2+/-6.8% and 48.3+/-7%, respectively. Only donor type, i.e., sibling versus unrelated, showed significant correlation with DFS in multivariate analysis (P=0.010). The rates of relapse and 1 year TRM were 28.9+/-6.4% and 26.4+/-6.1%, respectively, and unrelated donor HSCT (P=0.002) and HLA mismatch (P=0.022) were significantly correlated with increased TRM in univariate analysis. CONCLUSION: In this single institution study spanning more than 17 years, sibling donor HSCT was the only factor predicting a favorable result in multivariate analysis, possibly due to increased TRM resulting from unrelated donor HSCT.

Neonatal Seizures in Preterm Infants

PURPOSE: Neonatal seizures are the most prominent feature of neonatal neurologic dysfunction, and the lifespan risk for seizures is highest in the neonatal period, especially in preterm infants. This study was aimed to find and analyze the risk factors and clinical profiles of seizures in preterm infants. METHODS: Sixteen cases of preterms with seizures were retrospectively reviewed between Mar. 2009 and Feb. 2012. RESULTS: The average gestational age was 30.3+/-4.1 weeks and 11 patients (68.7%) experienced seizures within 1 week after the birth. Eight cases (50%) had a perinatal asphyxia during the delivery and 7 cases (43.8%) had a history of perinatal maternal illness. Various types of seizures were observed of which subtle seizures were the most common (50%). Electroecephalographies were performed in 12 cases, which showed abnormal findings in 9 cases (75%). Neuroimaging studies were performed in 16 cases and showed abnormal findings in 10 cases (62.5%). Thirteen patients were discharged in the improved state and 3 patients expired. Eight cases had normal outcome, while 3 cases showed developmental delay. Prognosis was unknown in 2 cases due to follow up loss. CONCLUSION: Neonatal asphyxia is the most important risk factor. Furthermore, infants with a history of perinatal maternal illness appear to be at risk for neonatal seizures. EEG is a sensitive method for assessing seizure activity and prognosis. Monitoring high risk infants with asphyxia and a history of perinatal maternal illness, and early postnatal tracing with EEG is required to detect and manage high risk preterms.

A Survey for Changed Control Policies of Hepatitis B in Republic of Korea / 소아감염

PURPOSE: We have tried to search all concerning data on the policies of hepatitis B vaccination or surveillance of medical equipments that can transmit blood mediated diseases, including disposable syringe, in Republic of Korea, to propose references to other medical professionals. METHODS: Data from domestic journals, government websites, computerized newspapers, etc. were reviewed. The data were arranged into four categories (governmental policies on hepatitis B control, hepatitis B mass immunization program in school, using process of hepatitis B vaccine, and policies on medical equipments surveillance, including disposable syringes, which is considered as source of blood mediated infection), and the periodic changes on each subjects in policies were reviewed. RESULTS: Due to growing social concerns on hepatitis B in early 1980s, swift introduction and production of hepatitis B vaccine was made. After establishment of "5 year project for eradication of hepatitis B" in late 1983, number of vaccinated population jumped up to 6 million within 2 years (1984-85). However, since the immunization targets were mainly adult, not infant, this project was annulled in late 1985. Following this project, hepatitis B mass immunization program in school was carried out. In year 1995, hepatitis B vaccine was included in national immunization program. The use of disposable syringe was recommended from year 1980 but legislated in year 1985, finally. CONCLUSION: The mainstay in controlling hepatitis B in Republic of Korea was coordination of prompt introduction of vaccine, mass immunization, high vaccination coverage rate, or use of disposable syringes. However, since there is lack of official data available, it is urgent to arrange and computerize all government data related to infectious diseases.

Limitation of Prediction on Intravenous Immunoglobulin Responsiveness in Kawasaki Disease / 소아감염

PURPOSE: We aimed to evaluate predictive parameters for non-response to intravenous immunoglobulin (IVIG) in patients with Kawasaki disease (KD) before IVIG use using two controls. METHODS: We evaluated 229 consecutive KD patients who were treated with 2 g/kg of IVIG at a single center. Those who had persistent fever >24 hours after IVIG infusion made up the 23 IVIG non-responders; the first control included a total 206 defervesced cases and the second control included 46 cases that were matched for age and pre-treatment fever duration to non-responders. RESULTS: Demographic and clinical characteristics were similar in IVIG non-responders and responders at presentation. As for laboratory findings, the neutrophil differential, CRP, AST, ALT, and LDH were higher, and lymphocyte differential, total protein, albumin, platelet count, and total cholesterol were significantly lower in IVIG non-responders compared to responders by univariate analysis in both study designs. However in multivariate analysis, non-responders showed a significantly higher neutrophil differential (cutoff value, >77%, sensitivity 68.4% and specificity 79.5%) and lower cholesterol (<124 mg/dL, sensitivity 79% and specificity 70.5%). Whereas plasma albumin (<3.6 g/dL, sensitivity 73.7% and specificity 60%) was the sole laboratory parameter of non-responders in the second study design. CONCLUSION: Severity of inflammation in KD was reflected by higher or lower laboratory values at presentation. Because the multivariate analysis for these indices may be influenced by some confounding factors, including the numbers of patients of different ages and fever duration, other assessment modalities are needed for KD patients with the greatest risk of coronary artery lesions.

A Case fo Disseminated Malignant Melanoma Arising From Pigmented Nevus / 대한피부과학회지

Malignant melanoma is a tumor arising within the melanocyte system of the skin, eyes and juxtacutaneous mucous membrane. Tbrough the lymphatic channel developed early metastasis and regional lymphade- nopzthy developed. Malignant melanoma may arise on apparently normal skin, in a junction nevua, rareh in a compound nevus and extremeIy rarely in an intradermaI nevus and 20% of total incidence of malignant melanoma are preceded by a nevocellular nevus. A 41 year-old male patient who had multiple, pea to walnut sized, black colored. nodules developing on entire body visited our Dermto. dept. About one year ago, solitary black colored nodule arose from preexisting pigmented nevus which had been from 20 yrs ago on Rt. ant. axillary area and then nodule remcved by wide exeision and regional lymphadenectomy at a Hosp. 2 months after surgical operation, multiple pea to walnut sized, black colored nodules has developed on entire body. There was no lymphadenopathy on entire body. In H-E & Fontana-Masson stain, numerous bizzarre large cells with hyperchromatic variable sized melanin pigments are seen in the dermis and subcutaneous tissues Mitotic figutes are present. Diagnosis was confirmed by the clinical and histological feature. The patient was treated with BCG waccination(4*10

Two Cases of Solitary Type Keratoacanthoma / 대한피부과학회지

The solitary type of Koratoacanthoma is a common, rapidly growing, benign tumor with a natural history of spontaneous involution which was first described by Hutchinson in 1889. It was well known that this Keratoacanthoma is close resemblence to squamous cell carcinoma clinicallt & histopathology. The etiology of Kertoacanthoma is ucertain but it have been associated with many exogenous factors, including sun exposure, occupational exposure to heat and trauma, and tar and with autoimmune etiology and viral infection. We experienced 2 cases of soitary type of Keratoacantoma with typical clinical and histopathologic findings which presented on the pubic area and forehead but had no predisposing factors of Keratoacanthoma. The one was 66-year-old woman who had been a history of proritic, rapidly growing tumor on Mons Pubis since about 6 months ago. The another one was 40 year-old male who had been a history of asymromatic, growing tomor on forehead since about 3 months ago. In the H-E stained sections, there were horn filled invagination, dyskeratotic cell, horn pearl in the epidermis and an inflammatory infiltration in dermis. Diagnosis was cinfirmedby the clinical and histopatholigical pictures. The former was treated by electrocautery after simple excision and the latter by curretage and electrodesiccation.

A Case Report of 3 Clinical Types of Cutaneous Tuberculosis Simultaneously Appeared on One Person / 대한피부과학회지

Tuberculosis of the skin may present clinically as plaques, ulcers, verrucous, lesions, nodules, tumors, vegetatives reactions, and cicartricial infiltration. The reaction of the host to the presence of Mycobacterium tuberculosis also varies with the extent and the degree of invasion, the virulence of the bacilli, and nonspecific factors such as age and generaI state of health. The incidence of cutaneous tuberculosis has tendency to decline in the world because of thc availability of effective antiTB drugs, elimination of infected milk herbs, and an elevation of living standards, but in Korea, cutaneous tuberculosis remained to be a significant medical problem. We experienced 21 year-old male who had a case of 3 clinical types including Lupus vulgaris, Scrofuloderma, Papulonecrotic tuberculid. Diagnosis of 3 clinical types vere established by clinical history and signs, histo pathology, tuberculin test and effective of treatment vith INH, streptomycin and literature were reviewed.

Statistical Consideration in Syphilitic Patients in Ewha Womans University Hospital (1974. 8. - 1977. 8.) / 대한피부과학회지

The recent prevalence of syphilis has known to be increased progressively throughout the world, since middle of 1950. In Korea, current trend of increased syphilic infection has became a major public health problem. The authors selected sero-positive patients, among the outpaients of department of dermatology, urology, OB & GY, and physical examination, and performed statistical analysis of this sero-positive patients. Results are as following; 1) Of the 2,007 patients taken physical examination, 30 patient(1.49%) were reactive to VDRL. 2) 0.71% of the total out patients of the dermatologic dcpartment were sero-positive. Annual ratio of the syphilitic patient were increased progressively. 3) Ratio of the syphilitic patients to the total outpatients of OB & GY department was 0.55% and somewhat increased since 1976. 4) In the age distribution, the third decade shared 51.8% of the total sero-positive patients 5) In the sexual ratio. male:female=l: l. 62 in the total sero-positive patients. (male:female=l: 1.06 in the dermatologic department.) 6) In the stage distribution, latent syphilis patients were 59.14% of the total syphilitic patients.

Clinical Effect of Glutathione (Tathion) on Melasma / 대한피부과학회지

Tatbion is a tripeptide, reduced form of Glutathione(GSH or p-glutamyl-cysteiny1 -glycine). Glutathione(SH compound)is believed to inhibit melanin formation by combining witb the copper in tyrnsinase whicb is essential in the conversion of tyrosine to DOPA (3, 4-dihydroxyphenylalanine) and DOPA to DOPA-quinone or by forming cornplex with the intermediate in the tyrosine-to-melaa.in reaction. The effect of Tathion in the treatment of melasma has not been reported in Korea. We have observed the effect of Tatbion in 150 patients with melasma. After the average duration of Gwks of treatment(50-100mg tree tirnes/daily), we were abIe to grade the results as follow. Excellent(Pigmentation almost disappeared): 17. 4 % Good (Pigmentation markedly improved): 56. 7% Fair (Pigmentation slightly improved: 7. 3% None (No effect) : 18.6% The result showed relatively good effects of Tathion in the treatment of 122pts (81.4%) with melasma in total. The brief review of literature on the treatment of melasma was undertaken.

Two Cases of Paget's Disease / 대한피부과학회지

Pagets disease is charaterized by a unilateral sharply defined eczema with or without an underlying ductal adenocarcinoma of the female nipple which was described by James Paget on 1874. We experienced 2 cases of Pagets disease confined to the nipple & areola with or without a palpable mass. The one was 44-year-old female, who had been suffered from pea. sized, eczematous lesion and crust formation on the Lt. nipple and areola with a painful palpable mass in the Lt, breast and nipple retraction since about 3years ago(Fig. 1). The another one was 52-year-old female, who had been suffered frorn palm sized, same lesions of the former on the Lt. breast with accompanying itching sensation and without a palpable mass and nipple retraction since about 7years ago(Fig. 5). Histopathologically, there were large, round, clear staining cells with Iarge nuclei in the epidersi and an inflammatory infiltration in the dermis. Diagnosis was confirmed by the clinical and histological pictures. The former was treated by simple mastectomy and the latter by radical mastectorny.