Gout is the most common inflammatory arthritis among Filipinos, characterized by hyperuricemia leading to mono- sodium urate crystal deposition and an ensuing inflammatory response. Though typically a disorder of middle- aged and older adults, tophaceous gout presenting before the age of 30 is rare and suggests aggressive disease progression. Allopurinol, a first-line urate-lowering therapy, is generally effective but may cause rare, potentially life-threatening adverse reactions such as allopurinol hypersensitivity syndrome (AHS). Febuxostat, a non-purine xanthine oxidase inhibitor, is an alternative for patients intolerant to allopurinol. Although hypersensitivity reactions to febuxostat are extremely rare, isolated case reports document their occurrence in both patients with prior AHS and in allopurinol-naïve individuals. Hypersensitivity to both agents is exceedingly uncommon and presents a major therapeutic challenge. In such cases, febuxostat desensitization, conducted in collaboration with allergy specialists, may permit a viable solution to safely reintroduce urate-lowering therapy and prevent further disease progression. This case report describes a patient with young-onset, tophaceous gout who developed severe hypersensitivity reactions to both allopurinol and febuxostat — an unusual and challenging therapeutic dilemma. The case highlights the need for individualized management strategies, including the consideration of drug desensitization, in patients with limited urate-lowering options.

BACKGROUND

Waiting time of patients from a consult with a primary care physician to a specialist is poorly understood. It is one indicator of health service delivery and patient satisfaction. Patients consider waiting for a specialist consult for more than three months too long and unacceptable.

To describe the sociodemographic and clinical factors associated with length of referral waiting time.

Cross-sectional retrospective chart review of patient records in a tertiary government hospital from 2015 to 2019.

A total of 366 charts were reviewed. Many of the patients referred to other specialty clinics were middle-aged adults and females. Median wait times for medical and surgical specialties were 11 (IQR: 0-29) and 18 (IQR: 6-35) days, respectively (p=0.003). Nutrition, rehabilitative medicine, and family health unit received the most number of referrals among non-surgical fields. Ophthalmology, otorhinolaryngology, and general surgery received the highest number of referrals among the surgical fields. Referral waiting times were longest for cardiology (median: 125, IQR: 91-275 days) and shortest for nutrition (median: 0, IQR: 0-6 days).

Waiting times from a primary care clinic to a specialty clinic at a tertiary government hospital vary based on urgency, specialty clinic, purpose of referral, presence of comorbidities, and chronicity of condition. Clinical factors found to be significantly associated with referral waiting time include urgency, type of clinic, and purpose of referral.

Immune checkpoint inhibitors (ICIs) have been changing the paradigm of cancer treatment. However, immune-related adverse effects (irAEs) have also increased with the exponential increase in the use of ICIs. ICIs can break up the immunologic homeostasis and reduce T-cell tolerance. Therefore, inhibition of immune checkpoint can lead to the activation of autoreactive T-cells, resulting in various irAEs similar to autoimmune diseases. Gastrointestinal toxicity, endocrine toxicity, and dermatologic toxicity are common side effects. Neurotoxicity, cardiotoxicity, and pulmonary toxicity are relatively rare but can be fatal. ICI-related gastrointestinal toxicity, dermatologic toxicity, and hypophysitis are more common with anti- CTLA-4 agents. ICI-related pulmonary toxicity, thyroid dysfunction, and myasthenia gravis are more common with PD-1/PD-L1 inhibitors. Treatment with systemic steroids is the principal strategy against irAEs. The use of immune-modulatory agents should be considered in case of no response to the steroid therapy. Treatment under the supervision of multidisciplinary specialists is also essential, because the symptoms and treatments of irAEs could involve many organs. Thus, this review focuses on the mechanism, clinical presentation, incidence, and treatment of various irAEs.
Autoimmune Diseases ; Cardiotoxicity ; Homeostasis ; Incidence ; Myasthenia Gravis ; Organization and Administration ; Specialization ; Steroids ; T-Lymphocytes ; Thyroid Gland

PURPOSE: We used a questionnaire to explore perceptions and clinical practice patterns of Korean pediatric ophthalmologists in terms of amblyopia.METHODS: From September to November 2018, we conducted a web-based questionnaire survey of 99 specialists of the Korean Association for Pediatric Ophthalmology and Strabismus who operated ophthalmology clinics in Korea. We received 56 responses (56.57%) and retrospectively analyzed the data.RESULTS: The average specialist age was 44.0 ± 9.7 years. The mean age of treated amblyopia patients was 3 to 5 years (69.6%); the most common amblyopia was refractive anisometropic amblyopia (75.0%). On average, treatment commenced at 4 years of age (53.6%); child and parent co-operation most significantly influenced treatment success (46.4%). The preferred test was cycloplegic refraction (96.4%) and the preferred treatment occlusion therapy (100%) with glasses correction (98.2%). Occlusion therapy was most commonly performed for 2 hours/day (69.6%); the minimum age for eyeglasses prescription was 2.10 ± 1.18 years. Only three respondents (5.36%) prescribed contact lenses and only one (1.79%) performed refractive surgery.CONCLUSIONS: In Korea, amblyopia treatment is based on occlusion therapy and glasses correction. However, the time of treatment commencement, the duration of occlusion therapy, and the glasses used for correction varied. It is necessary to develop guidelines for amblyopia treatment; these should reflect current medical conditions.
Amblyopia ; Child ; Contact Lenses ; Eyeglasses ; Glass ; Humans ; Korea ; Ophthalmology ; Parents ; Practice Patterns, Physicians' ; Prescriptions ; Refractive Surgical Procedures ; Retrospective Studies ; Specialization ; Strabismus ; Surveys and Questionnaires

BACKGROUND: The incidence of asthma exacerbation (AE) and the predictive value of spirometry and fractional exhaled nitric oxide (FeNO) in school children have not been evaluated.OBJECTIVE: We sought to evaluate the efficacy of spirometry measurement and FeNO monitoring for predicting AE in school children in the Cheongju area in Korea.METHODS: With parental agreement, we studied 170 students aged 7–12 years. Children were evaluated by an asthma specialist using baseline spirometry, skin prick test, seasonal FeNO measurement, and asthma control test. The study participants underwent a physical examination and their medical history was also evaluated by the specialist. They were assessed for asthma control status during regular doctor visits for 1 year.RESULTS: In total, 160 children (94.1%) completed follow-up and FeNO monitoring. Of which, 26 children (16.3%) had AE. AE was associated with male children and children with allergic rhinitis (p < 0.05). While, children with AE tended to have higher FeNO than those without AE, no significant difference was found. The maximum value of FeNO ≥35 ppb was associated with AE (p < 0.05). Children with AE had a significantly decreased baseline forced expiratory volume in 1 second/forced vital capacity (FEV₁/FVC), %predicted, forced expiratory flow at 25%–75% of FVC (FEF(25%–75%)). FEV₁/FVC < 80% was associated with AE in children regardless of inhalant allergen sensitization (all p < 0.05).CONCLUSION: Baseline spirometry had a predictive value of AE in school children. Sensitive spirometric parameters such as FEV₁/FVC and FEF(25%–75%) can be used as prognostic factors to predict future childhood AE. FeNO value ≥ 35 ppb during monitoring was associated with AE in school children.
Asthma ; Child ; Chungcheongbuk-do ; Follow-Up Studies ; Forced Expiratory Volume ; Humans ; Incidence ; Korea ; Male ; Nitric Oxide ; Parents ; Physical Examination ; Rhinitis, Allergic ; Seasons ; Skin ; Specialization ; Spirometry ; Vital Capacity

BACKGROUND/AIMS: School nurses’ knowledge of inflammatory bowel disease (IBD) has not been evaluated. We aimed to investigate school nurses’ knowledge of IBD and determine whether education could improve this knowledge. METHODS: School nurses were invited to complete self-reported questionnaires on IBD. Then, IBD specialists from tertiary referral hospitals provided a 60-minute lecture with educational brochures on two occasions, with a 3-month interval. Within 6 months after the educational interventions, school nurses were asked to complete the same IBD questionnaire via e-mail. RESULTS: Among 101 school nurses who were invited to participate, 54 nurses (53.5%) who completed two consecutive questionnaires were included in this study (median age, 45 years; range, 25 to 59 years; 100% female); 11.1% and 7.4% of the study participants had no knowledge regarding ulcerative colitis and Crohn’s disease, respectively. They had heard of IBD most frequently from doctors (33.3%), followed by internet sources (25.9%). After 6 months, the number of nurses who could explain IBD to students with over 30% confidence increased from 24 (44.5%) to 42 (77.8%) (p < 0.001). Most nurses (81.5%) reported that the educational intervention was helpful for managing students with abdominal pain or diarrhea. The number of students who received IBD-related welfare services from the Daegu Metropolitan Office of Education doubled when compared with the corresponding number during the prior educational year. CONCLUSIONS: There is room for improvement in school nurses’ knowledge of IBD. A systematic educational program on IBD should be implemented for these nurses.
Abdominal Pain ; Colitis, Ulcerative ; Crohn Disease ; Daegu ; Diarrhea ; Education* ; Electronic Mail ; Humans ; Inflammatory Bowel Diseases* ; Internet ; Pamphlets ; Schools, Nursing ; Specialization ; Tertiary Care Centers

Scholarly article writing and publishing in international peer-reviewed journals can become an overwhelming task for many medical, nursing, and healthcare professionals in a university setting, especially in countries whose native language is not English. To help improve their scientific writing skills and publishing capacity, a university-based editing system and writing programs can be developed as educational platforms. These are delivered by a team of specialist editors composed of tenured faculty members who have a strong medical background and extensive experience in teaching courses on medical research, editing, writing, and publishing. For the editing system, the specialist editors provide comprehensive editing, personalized consultation, full editorial support after peer review, guidance with online submissions/resubmissions, and detailed editorial review at different stages of the manuscript writing. In addition, the specialist editors can develop writing programs such as medical writing and editing internships, academic courses in medical writing or research study designs and reporting standards, special interactive lectures and sessions on predatory publishing, seminars on updated editorial guidance of global editorial associations, academic visits on medical writing and editing, medical writing mentoring program, networking programs in scholarly communication, and publication resources in medical writing and scholarly publishing. These editing system and writing programs can serve as integrated platforms for improving scientific writing skills and publishing capacity by providing continuing education in medical writing, editing, publishing, and publication ethics.
Delivery of Health Care ; Education, Continuing ; Ethics ; Humans ; Internship and Residency ; Lectures ; Medical Writing ; Mentors ; Nursing ; Peer Review ; Publications ; Specialization ; Writing*

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.
Autism Spectrum Disorder ; Autistic Disorder ; Cytogenetics ; Diagnostic Tests, Routine ; Down Syndrome ; Humans ; Intellectual Disability ; Korea ; Microarray Analysis ; Muscular Dystrophy, Duchenne ; Prader-Willi Syndrome ; Prospective Studies ; Referral and Consultation ; Specialization

Infantile hemangioma (IH) usually presents solely as a cutaneous manifestation, and rarely accompanies diverse anomalies such as spinal dysraphism. A 2-month-old girl presented with IH on her lumbar skin as a coin-sized red plaque with adjacent depressed skin and a child-palm-sized red plaque on her left ankle since birth. Considering the coexistence of IH and depressed skin on the midline in her lumbosacral area, magnetic resonance imaging of her spine was performed, which showed intraspinal/dermal vascular tumors with spina bifida occulta at the 12th thoracic vertebrae level. Furthermore, no neurologic deficits were observed. She has been taking oral propranolol with topical timolol to prevent neural complications and the lesions clinically improved. However, additional surgery for the intraspinal lesions was considered due to urination/defecation abnormalities since she was 13 months of age. In cases of midline IH, particularly with additional skin lesions, appropriate imaging studies to identify accompanying anomalies should be performed, and referrals to neurosurgical specialists should be considered.
Ankle ; Female ; Hemangioma ; Humans ; Infant ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Parturition ; Propranolol ; Referral and Consultation ; Skin ; Specialization ; Spina Bifida Occulta ; Spinal Dysraphism ; Spine ; Thoracic Vertebrae ; Timolol

PURPOSE: Pediatric palliative care (PPC) is emphasized as standard care for children with life-limiting conditions to improve the quality of life. In Korea, a government-funded pilot program was launched only in July 2018. Given that, this study examined various PPC delivery models in other countries to refine the PPC model in Korea. METHODS: Target countries were selected based on the level of PPC provided there: the United Kingdom, the United States, Japan, and Singapore. Relevant literature, websites, and consultations from specialists were analyzed by the integrative review method. Literature search was conducted in PubMed, Google, and Google Scholar, focusing publications since 1990, and on-site visits were conducted to ensure reliability. Analysis was performed on each country's process to develop its PPC scheme, policy, funding model, target population, delivery system, and quality assurance. RESULTS: In the United Kingdom, community-based free-standing facilities work closely with primary care and exchange advice and referrals with specialized PPC consult teams of children's hospitals. In the United States, hospital-based specialized PPC consult teams set up networks with hospice agencies and home healthcare agencies and provide PPC by designating care coordinators. In Japan, palliative care is provided through several services such as palliative care for cancer patients, home care for technology-dependent patients, other support services for children with disabilities and/or chronic conditions. In Singapore, a home-based PPC association plays a pivotal role in providing PPC by taking advantage of geographic accessibility and cooperating with tertiary hospitals. CONCLUSION: It is warranted to identify unmet needs and establish an appropriate PPD model to provide need-based individualized care and optimize PPC in South Korea.
Adolescent ; Child ; Delivery of Health Care ; Disabled Children ; Financial Management ; Great Britain ; Health Services Needs and Demand ; Home Care Services ; Hospice Care ; Hospices ; Humans ; Japan ; Korea ; Methods ; Palliative Care ; Pediatrics ; Primary Health Care ; Quality of Life ; Referral and Consultation ; Singapore ; Specialization ; Tertiary Care Centers ; United States