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.

The condition of work environment (patient room) is often out of concern to the management so that they do not meet the requirements of Regulation of Minister of Health of the Republic of Indonesia (Permenkes) No. 1204 of 2004 concerning Hospital Environmental Health Requirements. It will have implications for the performance of the doctor (specialist). This study aimed to describe the conditions of the work environment (temperature, lighting, and dust) and the doctor's performance and the relationship between the work environment and the doctor's performance. This research was a quantitative study with a cross sectional design. The research was conducted in 3 teaching hospitals in Medan, namely H. Adam Malik Hospital, dr. Pirngadi and RSU USU during April - November 2018. The study population was 236 people with a sample of 22 people taken using incidental sampling technique. The data obtained by measuring the conditions of the work environment (temperature, lighting, and dust) and interviews using a questionnaire to measure the performance of doctors. Data analysis used Fisher's Exact Test. The results showed that there was no influence of work environment conditions on the doctor's performance, however the temperature and lighting conditions did not meet the requirements. Meanwhile, the temperature of the work room affected the behavior of doctors. Thus, the hospital management needs to take corrective action on working environment conditions by replacing old air conditioners or regularly maintaining air conditioners, setting up a barrier against noise sources, and replacing the bulb with a lighter intensity. The medical committee needs to regularly supervise the work of doctors so that their performance is achieved according to the target.
effect of working environment, specialist physicians&rsquo ; , performance

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

Sleep is well known to be important to health and well-being, creativity, memory consolidation, and cognitive functions. However, sleep disorder patients sometimes had some limitation to get proper diagnosis and treatments. Now we live in an era of big change, so called the Fourth Industrial Revolution, which is characterized by mobile internet connectivity and artificial intelligence. Sleep medicine also started to change to patients-centered medicine with technical enhancement. To date, lots of smartphone applications and wearable device for monitoring sleep have appeared but not been validated enough against polysomnography. As another topic, big data receives lots of attention among sleep specialists. It is believed that big data would provide the basis of personalized healthcare. Here, we will discuss about new trend of sleep medicine involving mobile health such as telemedicine, smartphone, wearable device, and big data.
Artificial Intelligence ; Cognition ; Creativity ; Delivery of Health Care ; Diagnosis ; Humans ; Internet ; Memory Consolidation ; Polysomnography ; Sleep Wake Disorders ; Smartphone ; Specialization ; Telemedicine

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

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

PURPOSE: This study was performed to investigate the current status of pediatric palliative care provision and how it is perceived by the palliative care experts. METHODS: A descriptive study was conducted with 61 hospice institutions. From September through October 2017, a questionnaire was completed by experts from the participating institutions. Data were analyzed using SPSS 21.0. RESULTS: Among 61 institutions, palliative care is currently provided for pediatric cancer patients by 11 institutions (18.0%), all of which are concentrated in Seoul, Incheon and Gyeonggi and Gyengsang provinces; 85.2% of all do not plan to provide specialized pediatric palliative care in the future. According to the experts, the main barriers in providing pediatric palliative care were the insufficient number of trained specialists regardless of the delivery type. Experts said that it was appropriate to intervene when children were diagnosed with cancer that was less likely to be cured (33.7%) and to move to palliative care institutions when their conditions worsened (38.2%); and it was necessary to establish a specialized pediatric palliative care system, independent from the existing institutions for adult patients (73.8%). CONCLUSION: It is necessary to develop an education program to establish a nationwide pediatric palliative care centers. Pediatric palliative care intervention should be provided upon diagnosis rather than at the point of death. Patients should be transferred to palliative care institutions after intervention by their existing pediatric palliative care team at the hospital is started.
Adult ; Child ; Diagnosis ; Education ; Gyeonggi-do ; Hospices ; Humans ; Incheon ; Palliative Care ; Republic of Korea ; Seoul ; Specialization ; Terminal Care