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

Echocardiography is the first and is the most-available imaging modality for many cardiovascular diseases, and echocardiographic parameters can give much important information for diagnosis, treatment, and prognostic evaluations. Left ventricular ejection fraction (LVEF) is the most commonly used echocardiographic parameter for left ventricular (LV) systolic function. Although LVEF is used routinely in daily practice, it is calculated from volumetric change without representing true myocardial properties. Recently, strain echocardiography has been used to objectively measure myocardial deformation. Myocardial strain can give accurate information about intrinsic myocardial function, and it can be used to detect early-stage cardiovascular diseases, monitor myocardial changes with specific therapies, differentiate cardiomyopathies, and predict the prognosis of several cardiovascular diseases. Although strain echocardiography has been applied to measure the right ventricle and left atrium, in addition to analyzing the LV, many cardiologists who are not imaging specialists are unaware of its clinical use and importance. Therefore, this review describes the measurement and clinical utility of 2-dimensional strain analysis in various cardiovascular diseases.
Cardiomyopathies ; Cardiovascular Diseases ; Diagnosis ; Echocardiography ; Heart Atria ; Heart Ventricles ; Prognosis ; Specialization ; Stroke Volume

Though clinical practice guidelines (CPGs) for cardiac rehabilitation (CR) are an effective and widely used treatment method worldwide, they are as yet not widely accepted in Korea. Given that cardiovascular (CV) disease is the second leading cause of death in Korea, it is urgent that CR programs be developed. In 2008, the Government of Korea implemented CR programs at 11 university hospitals as part of its Regional Cardio-Cerebrovascular Center Project, and 3 additional medical facilities will be added in 2019. In addition, owing to the promotion of CR nationwide and the introduction of CR insurance benefits, 40 medical institutions nationwide have begun CR programs even as a growing number of medical institutions are preparing to offer CR. The purpose of this research was to develop evidence-based CPGs to support CR implementation in Korea. This study is based on an analysis of CPGs elsewhere in the world, an extensive literature search, a systematic analysis of multiple randomized control trials, and a CPG management, development, and assessment committee comprised of thirty-three authors—primarily rehabilitation specialists, cardiologists, and thoracic surgeons in 21 university hospitals and 2 general hospitals. Twelve consultants, primarily rehabilitation, sports medicine, and preventive medicine specialists, CPG experts, nurses, physical therapists, clinical nutritionists, and library and information experts participated in the research and development of these CPGs. After the draft guidelines were developed, 3 rounds of public hearings were held with staff members from relevant academic societies and stakeholders, after which the guidelines were further reviewed and modified. CR involves a more cost-effective use of healthcare resources relative to that of general treatments, and the exercise component of CR lowers CV mortality and readmission rates, regardless of the type of coronary heart disease and type and setting of CR. Individualized CR programs should be considered together with various factors, including differences in heart function and lifestyle, and doing so will boost participation and adherence with the CR program, ultimately meeting the final goals of the program, namely reducing the recurrence of myocardial infarction and mortality rates.
Acute Coronary Syndrome ; Cause of Death ; Consultants ; Coronary Disease ; Delivery of Health Care ; Heart ; Hospitals, General ; Hospitals, University ; Humans ; Insurance Benefits ; Korea ; Life Style ; Methods ; Mortality ; Myocardial Infarction ; Nutritionists ; Physical Therapists ; Preventive Medicine ; Recurrence ; Rehabilitation ; Secondary Prevention ; Specialization ; Sports Medicine ; Surgeons

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

Little is known about the treatment of gender dysphoria among children and adolescents in Japan. This preliminary survey aims to improve understanding of current clinical practice for treatment of children with gender dysphoria. Subjects were 315 certified child and adolescent psychiatrists in Japan. The questionnaire asked about clinical experiences concerning gender dysphoria and gender identity-related concerns. A total of 128 psychiatrists responded to the questionnaire. Mean length of clinical experience was 24.2±10.0 years in total and 16.9±11.5 years as child and adolescent psychiatry specialists. Among the respondents, 74 (57.8%) had seen children and adolescents with DSM-5 gender dysphoria, and 87 (67.7%) had examined cases with gender identity-related concerns. The mean number of experienced cases with gender dysphoria was 1.80±2.3 per respondent. We found that even among certified child and adolescent psychiatrists in Japan, experience with treatment of children with gender dysphoria was limited.
Adolescent Psychiatry ; Adolescent ; Child ; Gender Dysphoria ; Humans ; Japan ; Psychiatry ; Specialization ; Surveys and Questionnaires ; Transgender Persons