Background: Primary cutaneous CD30+ lymphoproliferative disorders (pcCD30-LPDs) are a diseases with various clinical and prognostic characteristics. Objective: Increasing our knowledge of the clinical characteristics of pcCD30-LPDs and identifying potential prognostic variables in an Asian population. Methods: Clinicopathological features and survival data of pcCD30-LPD cases obtained from 22 hospitals in South Korea were examined. Results: A total of 413 cases of pcCD30-LPDs (lymphomatoid papulosis [LYP], n=237; primary cutaneous anaplastic large cell lymphoma [C-ALCL], n=176) were included. Ninety percent of LYP patients and roughly 50% of C-ALCL patients presented with multiple skin lesions. Both LYP and C-ALCL affected the lower limbs most frequently. Multiplicity and advanced T stage of LYP lesions were associated with a chronic course longer than 6 months. Clinical morphology with patch lesions and elevated serum lactate dehydrogenase were significantly associated with LPDs during follow-up in LYP patients. Extracutaneous involvement of C-ALCL occurred in 13.2% of patients. Lesions larger than 5 cm and increased serum lactate dehydrogenase were associated with a poor prognosis in C-ALCL. The survival of patients with C-ALCL was unaffected by the anatomical locations of skin lesions or other pathological factors. Conclusion The multiplicity or size of skin lesions was associated with a chronic course of LYP and survival among patients with C-ALCL.

Background: In 2006, the Korean Atopic Dermatitis Association (KADA) working group released the diagnostic criteria for Korean atopic dermatitis (AD). Recently, more simplified, and practical AD diagnostic criteria have been proposed. Objective: Based on updated criteria and experience, we studied to develop and share a consensus on diagnostic criteria for AD in Koreans. Materials and Methods: For the diagnostic criteria, a questionnaire was constructed by searching the English-language literature in MEDLINE and the Cochrane Database of Systematic Reviews. A modified Delphi method composed of 3 rounds of email questionnaires was adopted for the consensus process. Fifty-four KADA council members participated in the 3 rounds of votes and expert consensus recommendations were established. Results: Diagnostic criteria for AD include pruritus, eczema with age-specific pattern, and chronic or relapsing history. Diagnostic aids for AD encompass xerosis, immunoglobulin E reactivity, hand–foot eczema, periorbital changes, periauricular changes, perioral changes, nipple eczema, perifollicular accentuation, and personal or family history of atopy. Conclusion This study streamlined and updated the diagnostic criteria for AD in Korea, making them more practicable for use in real-world clinical field.

PURPOSE: Pseudomembranous colitis (PMC) occurs rarely in children, but its incidences are increasing due to frequent antibiotic use. We investigated the incidence and clinical characteristics of PMC accompanied by bacterial enteritis-like symptoms in children. METHODS: Between November 2003 and July 2007 at the Department of Pediatrics, Dongsan Medical Center, we analyzed the medical records of consecutive patients who received antibiotics in the past 1 month, developed bacterial enteritis-like symptoms, and were diagnosed with PMC based on sigmoidoscopy examination and histological findings. RESULTS: Among 22 patients who underwent sigmoidoscopy and biopsy examinations, 11 (50%) were diagnosed with PMC. These 11 patients were aged 2 months-12 years, among whom 5 patients (45.5%) were less than 1 year old. The clinical symptoms were bloody diarrhea (28.6%), abdominal pain or colic (28.6%), watery or mucoid diarrhea (23.8%), vomiting (9.5%), and fever (9.5%). The antibiotics used were penicillins (55.6%), macrolides (27.8%), cephalosporins (11.1%), and aminoglycosides (5.6%). The period of antibiotic use was 3-14 days. The interval between the initial antibiotic exposure and the onset of symptoms was 5-21 days. The results of stool examination of all patients were negative for Clostridium difficile toxin A. Patient distribution according to the degree of PMC was as follows: grade I, 18.2% (2 cases); grade II, 27.3% (3); grade III, 36.4% (4); and grade IV, 18.2% (2). PMC did not recur in any case. CONCLUSION: PMC is not a rare disease in children. If pediatric patients receiving antibiotics manifest symptoms like bacterial enteritis, PMC should be suspected. Endoscopy and biopsy should be applied as aggressive diagnostic approaches to detect this condition.
Abdominal Pain ; Aged ; Aminoglycosides ; Anti-Bacterial Agents ; Bacterial Toxins ; Biopsy ; Cephalosporins ; Child ; Clostridium difficile ; Colic ; Diarrhea ; Endoscopy ; Enteritis ; Enterocolitis, Pseudomembranous ; Enterotoxins ; Fever ; Humans ; Incidence ; Korea ; Macrolides ; Medical Records ; Pediatrics ; Penicillins ; Rare Diseases ; Sigmoidoscopy ; Vomiting

Perianastomotic ulceration (PAU) rarely occurs after small bowel resection in infancy. Since the understanding of its pathogenesis is incomplete, an effective method of treatment has not yet been discovered. We report the first case in Korea of a 10-year-old girl with chronic iron deficiency anemia (IDA) and growth failure who was diagnosed with PAU at colonoscopy. Seven years were required to identify the cause of IDA. After surgical resection and revision of anastomosis, a close follow-up is being conducted due to the risk of recurrence. Here, we also review reports on 25 pediatric patients with PAU derived from a search of the English-language literature and describe the clinical features of PAU along with the results of treatment.
Anemia, Iron-Deficiency ; Child ; Colonoscopy ; Follow-Up Studies ; Humans ; Iron ; Korea ; Recurrence ; Ulcer

Cowden's syndrome is a harmatomatous polyposis syndrome with characteristic mucocutaneous lesions and among the spectra of clinical disorders that has been attributed to germline mutations in the PTEN gene. Although Cowden's syndrome has rarely been reported, immunologic studies have revealed that patients with this syndrome have humoral and/or cellular immune abnormalities. We recently identified a 21-year-old woman with Cowden's syndrome who was diagnosed with candida esophagitis without a history of diabetes, carcinoma, or steroid therapy. We report the immunologic status of this patient and the relationship with candida esophagitis on the basis of a literature review.
Candida ; Esophagitis ; Female ; Germ-Line Mutation ; Hamartoma Syndrome, Multiple ; Humans ; Young Adult

BACKGROUNDS/AIMS: Peginterferon alpha-2a or -2b is the standard treatment regimen in chronic hepatitis C. However, there have been few comparative studies of the efficacies of these two types of peginterferon. We evaluated their efficacies in combination with ribavirin as a initial treatment for chronic hepatitis C. METHODS: Ninety-seven patients were treated with peginterferon alpha-2a (180 microgram/week, n=48) or peginterferon alpha-2b (1.5 microgram/kg/week, n=49) plus ribavirin (800 mg/day for 24 weeks in genotype non-1 or 1,000-1,200 mg/day for 48 weeks in genotype 1). Virologic responses including the early virologic response (EVR), end-of-treatment response (ETR), sustained virologic response (SVR), and adverse effects were analyzed retrospectively. RESULTS: The virologic response rates did not differ significantly between peginterferon alpha-2a and -2b: 89.6% and 89.7% for EVR, 79.2% and 79.5% for ETR, 72.9% and 73.5% for SVR, respectively. Analysis of the virologic responses according to genotype also revealed no significant differences in SVR between peginterferon alpha-2a and -2b (59.3% vs. 59.7% for genotype 1 and 90.5% vs. 83.3% for genotype non-1, respectively), or in adverse effects including flu-like symptom, rash, itching, neutropenia, and thrombocytopenia. CONCLUSIONS: We found no significant differences in therapeutic efficacies and adverse effects between the alpha-2a and -2b types of peginterferon as the initial treatment regimen in naive chronic hepatitis C patients.
Adult ; Antiviral Agents/*administration & dosage ; Combined Modality Therapy ; Genotype ; Hepacivirus/drug effects/genetics ; Hepatitis C, Chronic/diagnosis/*drug therapy ; Humans ; Interferon Alfa-2a/*administration & dosage ; Interferon Alfa-2b/*administration & dosage ; Korea ; Middle Aged ; Polyethylene Glycols/*administration & dosage ; Retrospective Studies ; Ribavirin/*administration & dosage ; Risk Factors

BACKGROUND: Many AML patients have received hematopoietic stem cell transplantation (HSCT) from HLA-matched unrelated donors. According to many of the previous reports, those patients could achieve long-term, disease-free survival after HSCT from multinational unrelated donors with tolerable transplant-related complications, even when there are HLA-mismatches. METHODS: We present the results of 35 unrelated hematopoietic stem cell transplantations from multiple international donor banks including the Korean (n=24), and Japan Marrow Donor Program (n=3), the Taiwan Tzu Chi Marrow Donation Registry (n=6), as well as using Caucasian donors from the National Marrow Donor Program (n=2), for the treatment of AML patients. RESULTS: The median age of patients was 36 (range: 16~53) and the median follow-up duration was 21 months (range: 5~60). Also, the median age of the donors was 28 (range: 20~53). The majority of the patients had intermediate or unfavorable cytogenetic features. The main conditioning regimen we used consisted of cyclophosphamide plus TBI (n=31) with our standard GvHD prophylaxis that contained tacrolimus plus a short course of methotrexate. Some patients (n=10) received an additional two-day course of ATG (thymoglobulin, Sangstat) in addition to the standard regimen. All the transplanted patients achieved engraftment. The incidence of acute GvHD was 42%, and that of chronic GvHD was 56%. Four (11%) patients have relapsed to date. The two-year non-relapse transplant-related mortality was 26%. The estimated probability of DFS and the event-free survival at five-years were 80% and 53%, respectively. CONCLUSION: These results suggest that multinational unrelated donors HSCT may provide a feasible option for the treatment of high-risk Korean AML patients.
Bone Marrow ; Cyclophosphamide ; Cytogenetics ; Disease-Free Survival ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Incidence ; Japan ; Leukemia, Myeloid, Acute* ; Methotrexate ; Mortality ; Tacrolimus ; Taiwan ; Tissue Donors ; Unrelated Donors*