Medical School Location and Sex Affect the In-State Retention of Pediatric Residency Program Graduates in Hawai'i.

The objective of this study was to assess the impact of medical school, sex, career choice, and location of practice of one pediatric residency program on physician workforce. This is a retrospective study of all categorical pediatric graduates of a residency program located in Honolulu, Hawai'i from 1968 to 2015. Information on medical school training, sex, career choice (general pediatrics or specialty), and location of practice were studied by examining data into five 10-year graduation periods. The program graduated 319 residents over nearly a 50-year timespan. Of these, 181 (56.7%) residents remained in Hawai'i to practice (adjusted odds ratio [OR] = 7.46, 95% confidence interval [CI]: 3.61-15.43). There were 125 (39.1%) graduates who relocated to the continental US with the majority moving to the West (55.2%), while other graduates moved to the South, Midwest, and Northeast (25.6%, 13.6%, and 5.6%, respectively). The remaining 13 (4.1%) graduates moved internationally. Female residents steadily increased over time (P < .001), with females significantly choosing general pediatrics (OR = 3.05, 95% CI: 1.91-4.89). In the time periods with the highest percentage of University of Hawai'i medical school graduates, there was an increased percentage of graduates staying in Hawai'i. This study examined the regional and national impact of a small residency program. The results indicated that trends in gender and the impact of medical school location were important in establishing a pediatrician workforce for local communities. Support of both medical school and residency education should be considered when assessing future workforce needs.

Incidence of Acute Post-Streptococcal Glomerulonephritis in Hawai'i and Factors Affecting Length of Hospitalization.

Acute post-streptococcal glomerulonephritis (APSGN) is a disorder of inflammation in the glomeruli and vasculature of the kidneys that is caused by immune-complex formation after Streptococcus pyogenes infection. Most patients with APSGN present with macroscopic hematuria, edema, and hypertension, however presentation can vary from no symptoms to severe proteinuria, or even acute renal failure. This study sought to estimate the incidence of APSGN among children in Hawai'i, to identify populations at increased risk for APSGN, and to recognize risk factors correlated with the length of hospitalization by subtype of APSGN (eg, pyoderma-associated, pharyngitis-associated). This retrospective review of 106 patients found that the incidence of APSGN in Hawai'i is greater than 4 per 100,000 children, which is significantly higher than the incidence of APSGN in high-income countries at 0.3 per 100,000 children. This increased incidence may be due to Hawai'i's unique racial group composition and therefore the unique immunologic response of the children of Hawai'i (particularly Pacific Islanders, who represent 62% of patients with APSGN in this study, but only represent 10% of Hawai'i's general population). In addition, there may be increased prevalence of nephritogenic strains of Streptococcus pyogenes in Hawai'i. The length of hospitalization was significantly increased in children with elevated serum creatinine levels (P <.0001) and lower bicarbonate levels (P =.0003).

Presence, resilience, and compassion training in clinical education (PRACTICE): Evaluation of a mindfulness-based intervention for residents.

Background: Residents' well-being tends to decline in the course of training, yet research on preventive and restorative interventions for residents is limited. Mindfulness-based interventions have been successfully employed to support well-being in practicing physicians, but their impacts on residents are not well established. Objective: This paper describes the structure, content, and evaluation of a pilot mindfulness-based intervention program designated PRACTICE (presence, resilience and compassion training in clinical education) that was designed specifically to support resident well-being. Methods: A combined sample of 14 postgraduate year one residents from two residency training programs participated in a four-session (8 h) mindfulness-based intervention in the Fall/Winter of 2018. Participants were surveyed before, after, and at 3 months postintervention, on measures of wellness (Professional Fulfillment Index) and mental health (Patient Health Questionnaire-4), along with measures of program engagement. Results: Participants demonstrated a significant reduction in burnout at the conclusion of the program. Depression and anxiety screening scores also trended toward improvement. However, participants were not able to sustain these gains. Three months after the conclusion of the program wellness measures had returned to preintervention levels. Conclusions: The results of this study support the use of mindfulness-based interventions in resident wellness programs. The lack of an enduring effect indicates the need for a maintenance phase intervention.

Engaging a Community Chaplaincy Resource for Interprofessional Health Care Provider Training in Facilitating Family Decision Making for Children at End-of-Life.

Coordinating the care of terminally ill children is difficult for both parents and the health care team. An underutilized resource is spiritual care, such as that provided by Pacific Health Ministry, a community-based nonprofit established to develop hospital ministry training programs in Hawai'i and provide chaplaincy services to local facilities. This paper describes a training exercise, called the Pediatric Interprofessional Program (PIPP), which is modeled after an adult program, the Hawai'i Interprofessional Training for End of Life Communication in the intensive care unit (HITEC-ICU). Both programs were developed to introduce teams of learners consisting of Pacific Health Ministry spiritual care residents, internal medicine or pediatric residents, undergraduate students in nursing, and graduate students in social work to techniques in delivering serious, life-altering information, and the dynamics of working as an interprofessional team through use of progressively unfolding clinical simulations. PIPP facilitators included chaplaincy instructors at Pacific Health Ministry, university faculty, and community practitioners in pediatrics, nursing, and social work. The simulations were conducted at the Translational Health Science Simulation Center (THSSC) of the University of Hawai'i at Manoa (UHM) School of Nursing and Dental Hygiene (SONDH), with simulated patients from the HealthCAST (Collaborative Acting Simulation Training) program, a collaborative agreement between SONDH and the UHM Department of Theatre and Dance. The training is ongoing, but has thus far demonstrated that interprofessional education programs are feasible across community, academic, and clinical lines, and benefit from the engagement of community resources.

Diagnosis of Systemic Lupus Erythematosus in a Polynesian Male with a History of Rheumatic Fever: A Case Report and Literature Review.

The presence of rheumatic heart disease (RHD) and systemic lupus erythematosus (SLE) has rarely been described in one patient. This report describes an adolescent Polynesian male with RHD who developed SLE years later. Initially, he fulfilled modified Jones criteria for rheumatic fever with aortic insufficiency, transient arthritis, elevated streptococcal titers, and a high erythrocyte sedimentation rate with a negative antinuclear antibody (ANA). He responded well to nonsteroidal anti-inflammatory and penicillin prophylaxis, which supported the diagnosis of rheumatic fever. Five years after his RHD diagnosis, he developed pancreatitis with glomerulonephritis, nephrosis, and pancytopenia. In addition, laboratory results revealed that he had multiple autoantibodies: anti-Sm and extremely elevated anti-dsDNA and ANA, fulfilling diagnostic criteria for SLE. The patient was treated, and he responded to pulse steroids followed by oral steroid therapy. To our knowledge, there are no known reported cases of a patient who was diagnosed with both RHD and SLE and met the clinical criteria for both diseases. The rarity of this concurrent disease process in one patient suggests a possible overlap in humoral immunity toward self-antigens as well as ethnic variability that increases predisposition to rheumatologic diseases.

Chest Pain From Hypermobility Responding to Physical Therapy in an Adolescent.

Hypermobility syndrome usually causes pain in limbs from extension type injuries. The authors report on a 16-yr-old female adolescent with incapacitating chest pain secondary to extreme hypermobility of the chest. This pain led the patient to see multiple specialists without improvement or diagnosis. Physical examination results revealed a very hypermobile patient who was able to internally rotate her shoulders inward until her elbows touched. This unusual hyperextension maneuver was achieved by holding the shoulders in anteversion with her hands on her hips (see figures in the article). Currently, there is no literature reporting hypermobility as a cause for chronic chest pain. Pain medication including opioids did not reduce the patient's chronic chest pain. Specific physical therapy to strengthen core and chest wall muscles in addition to working on proper breathing techniques with the diaphragm decreased pain and resulted in a resolution of this condition. We report that hypermobility can cause significant chest pain and may require creative physical therapy to strengthen the specific musculature.

Cardiac Myosin Epitopes Recognized by Autoantibody in Acute and Convalescent Rheumatic Fever.

BACKGROUND: Acute rheumatic fever (ARF) is an autoimmune disorder associated with Streptococcus pyogenes infection. A prevailing hypothesis to account for this disease is that epitopes of self-antigens, such as cardiac myosin react to antibodies against S. pyogenes. The goal of our study was to confirm disease epitopes of cardiac myosin, identify immunodominant epitopes and to monitor the epitope response pattern in acute and convalescent rheumatic fever. METHODS: Enzyme-linked immunosorbant assays were used to determine epitopes immunodominant in acute disease and to track the immune response longitudinally to document any changes in the epitope pattern in convalescent sera. Multiplex fluorescence immunoassay was used to correlate anti-streptolysin O (ASO) and anti-human cardiac myosin antibodies. RESULTS: Disease-specific epitopes in rheumatic fever were identified as S2-1, 4 and 8. Epitopes S2-1, 4, 8 and 9 were found to be immunodominant in acute sera and S2-1, 8, 9, 29 and 30 in the convalescent sera. Frequency analysis showed that 50% of the ARF subjects responded to S2-8. S2-8 responders tended to maintain their epitope pattern throughout the convalescent period, whereas the S2-8 nonresponders tended to spread their responses to other epitopes later in the immune response. There was a significant correlation between anti-cardiac myosin and ASO titers. In addition, S2-8 responders showed elevated ASO titers compared with S2-8 non responders. CONCLUSION: Our studies confirm the existence of S2-1, 4 and 8 as disease-specific epitopes. We provide evidence that cardiac myosin S2-8 responders remain epitope stable in convalescence, whereas S2-8 nonresponders shift to neoepitopes. Multiplex data indicated a correlation between elevated ASO and anti-human cardiac myosin antibody titers. Mapping of cardiac myosin epitopes recognized in rheumatic fever sera may identify immunophenotypes of rheumatic fever.

The evolution of the Japanese medical education system: a historical perspective.

The Japanese Medical Education system has been influenced by political events throughout the country's history. From long periods of isolation from the western world to the effect of world wars, Japan's training system for physicians has had to adapt in many ways and will continue to change. The Japanese medical education system was recently compared to the "Galapagos Islands" for its unusual and singular evolution, in a speech by visiting professor Dr. Gordon L. Noel at the University of Tokyo International Research center.1 Japanese medical schools are currently working to increase their students' clinical hours or else these students may not be able to train in the United States for residencies. Knowing the history of the Japanese Medical education system is paramount to understanding the current system in place today. Studying the historical foundation of this system will also provide insight on how the system must change in order to produce better clinicians. This article provides a glimpse into the medical system of another nation that may encourage needed reflection on the state of current healthcare training in the United States.

Pulmonary hemosiderosis in children with bronchopulmonary dysplasia.

We describe a possible association between pulmonary hemosiderosis (PH) and a history of bronchopulmonary dysplasia (BPD). Both patients were born at 28-week gestation and presented with PH at ages 22 months and 6 years, respectively. Both initially presented with cough and tachypnea, and bronchoalveolar lavage showed evidence of hemosiderin-laden macrophages. Initial hemoglobin levels were < 4 g/dL and chest radiographs showed diffuse infiltrates that cleared dramatically within days after initiation of intravenous corticosteroids. In the first case, frank pulmonary blood was observed upon initial intubation, prompting the need for high frequency ventilation, immediate corticosteroids, and antibiotics. The mechanical ventilation wean was made possible by the addition of mycophenolate mofetil (MMF) and hydroxychloroquine. Slow tapering off of medications was accomplished over 6 years. These cases represent a possible correlation between prematurity-associated BPD and PH. We present a review of the literature regarding this possible association. In addition, MMF proved to be life-saving in one of the PH cases, as it has been in pulmonary hemorrhage related to systemic lupus erythematosus. Further studies are warranted to investigate the possible association between PH and prematurity-related BPD, as well as the use of MMF in the treatment of PH.

An intra-articular ganglion cyst in a patient with juvenile idiopathic arthritis.

We report an intra-articular ganglion cyst (IAGC) presenting as knee pain and a mass in a patient with longstanding Juvenile Idiopathic Arthritis (JIA). We could not find a similar case of an IAGC occurring in the knee of JIA patients in the literature. IAGC may need to be included as a possibility in patients with inflammatory arthritis with new-onset knee pain, especially in those with a palpable mass. MRI was useful in distinguishing IAGC from more worrisome causes of a knee mass. Orthopedic input was helpful in diagnosis and treatment. In addition, methotrexate therapy was effective in bringing about a long-lasting remission.

A pediatric residency research requirement to improve collaborative resident and faculty publication productivity.

Involvement in a research project can teach training physicians about the scientific process involved in medicine. For this reason, the University of Hawai'i pediatrics department developed a Residency Research Requirement and Program (RRRP) in 2001. We studied a 14-year time period before and after the RRRP was initiated, and found a greater then ten-fold increase in resident publications and faculty involvement in these projects. Many of these manuscripts were the result of resident collaboration and this also increased significantly. The residents who later went into fellowship training were found to be more likely to publish their work. An RRRP encourages residents and faculty to become involved in research publications and other scholarly activities. Its development may help to motivate training physicians to learn important research skills.

Priming the immune system for heart disease: a perspective on group A streptococci.

Although immune responses against group A streptococci and the heart have been correlated with antibodies and T cell responses against cardiac myosin, there is no unifying hypothesis about carditis caused globally by many different serotypes. Our study identified disease-specific epitopes of human cardiac myosin in the development of rheumatic carditis in humans. We found that immune responses to cardiac myosin were similar in rheumatic carditis among a small sample of worldwide populations, in which immunoglobulin G targeted human cardiac myosin epitopes in the S2 subfragment hinge region within S2 peptides containing amino acid residues 842-992 and 1164-1272. An analysis of rheumatic carditis in a Pacific Islander family confirmed the presence of potential rheumatogenic epitopes in the S2 region of human cardiac myosin. Our report suggests that cardiac myosin epitopes in rheumatic carditis target the S2 region of cardiac myosin and are similar among populations with rheumatic carditis worldwide, regardless of the infecting group A streptococcal M serotype.

An insert in the covS gene distinguishes a pharyngeal and a blood isolate of Streptococcus pyogenes found in the same individual.

Expression of the extensive arsenal of virulence factors by Streptococcus pyogenes is controlled by many regulators, of which CovRS is one of the best characterized and can influence ∼15 % of the genome. Animal models have established that mutants of covRS arise spontaneously in vivo resulting in highly invasive organisms. We analysed a pharyngeal and a blood isolate of S. pyogenes recovered from the same individual 13 days apart. The two isolates varied in many phenotypic properties including SpeB production, which were reflected in transcriptomic analyses. PFGE, multilocus sequence typing and partial sequencing of some key genes failed to show any differences except for an 11 bp insert in the covS gene in the blood isolate which caused a premature termination of transcription. Complementation of a fully functional covS gene into the blood isolate resulted in high expression of CovS and expression of speB. These results, showing a pharyngeal and a blood isolate from a single individual differing by a simple insertion, provide evidence for the model that regulatory gene mutations allow S. pyogenes to invade different niches in the body.

Group A streptococcal isolates temporally associated with acute rheumatic fever in Hawaii: differences from the continental United States.

BACKGROUND: The annual incidence of acute rheumatic fever (ARF) in Hawaii has remained several times higher than that in the continental United States, particularly among ethnic Polynesians. The emm types of Streptococcus pyogenes that are associated with this nonsuppurative complication have, to our knowledge, not been previously reported in Hawaii. METHODS: Patients with ARF were identified through an active surveillance system at Kapiolani Medical Center (Honolulu, HI), the only pediatric tertiary care referral hospital in Hawaii. Specimens were obtained by throat culture from patients who met the Jones criteria for ARF at the time of presentation (63 patients), prior to penicillin treatment, and from consenting family contacts (10 individuals). Eight patients and 2 close family contacts with positive throat culture results were identified from February 2000 through December 2005. Group A streptococci isolates were characterized by emm sequence typing. RESULTS: Unusual emm types were temporally associated with the onset of ARF. Emm types 65/69 (from 2 patients), 71, 92, 93, 98, 103, and 122 were isolated from the 8 patients with ARF, and emm types 52 and 101 were isolated from the 2 household contacts. CONCLUSIONS: So-called rheumatogenic emm types and/or serotypes, which were previously associated with ARF in the continental United States, were not found in this study. Instead, emm types that are not commonly included among group A streptococci isolates in the continental United States and that are seldom, if ever, temporally associated with ARF were identified. These findings suggest that unusual group A streptococci emm types play a significant role in the epidemiology of ARF in Hawaii.

Visiting consultant clinics to study prevalence rates of juvenile rheumatoid arthritis and childhood systemic lupus erythematosus across dispersed geographic areas.

OBJECTIVE: Visiting consultant clinics (VCC) may provide pediatric rheumatologic care to children in rural populations, but the clinical demands have not been studied. We studied whether these clinics could be effective in determining prevalence rates of rheumatic illness like juvenile rheumatoid arthritis (JRA) and childhood systemic lupus erythematosus (SLE) across large dispersed geographic areas. METHODS: The study population included children diagnosed with JRA or SLE at the only civilian pediatric rheumatology center in the State of Hawaii. Prevalence rates of these illnesses were then calculated for the urban and more rural neighbor island areas. VCC and prevalence data were calculated over a 10-year period. RESULTS: We found a lower prevalence of JRA in the urban area (38.3 per 100,000) when compared to the rural neighbor islands (63.2 per 100,000). However, an equivalent prevalence of SLE was found in the urban (24.0 per 100,000) and neighboring islands (21.8 per 100,000). Clinical demands increased significantly with the success of the VCC, and with an increase in pediatric rheumatologic staffing. CONCLUSION: We found an increased prevalence of JRA in rural areas when compared to urban areas. Similar prevalence rates of SLE suggested the finding was not due to referral bias alone. VCC are useful to estimate disease prevalence over large areas, and therefore make it possible to identify areas at greater risk. Further investigations are needed to elucidate the possible environmental and genetic factors that may explain the regional differences in JRA prevalence.

Streptococcal mimicry and antibody-mediated cell signaling in the pathogenesis of Sydenham's chorea.

Recent evidence suggests that the pathogenesis of Sydenham's chorea following group A streptococcal infection is due to antibodies which develop due to the infection and infiltrate the brain and basal ganglia. Antibodies present in acute chorea react with the surface of neuronal cells and signal the induction of calcium calmodulin dependent protein kinase II with elevation of tyrosine hydroxylase and subsequent dopamine release which may lead to the movement disorder. The antibodies present in disease recognize lysoganglioside and the group A streptococcal epitope, N-acetyl-glucosamine. Monoclonal antibodies (mAbs) from Sydenham's chorea demonstrated the mimicry between lysoganglioside and the group A streptococcal carbohydrate epitope. A group of antibodies present in pediatric autoimmune neuropsychiatric disorders (PANDAS) were similar but not identical to the antibodies observed in chorea.

Ethnic differences for developing rheumatic fever in a low-income group living in Hawaii.

UNLABELLED: The risk factors responsible for acute rheumatic fever (ARF) are complex, in part, because group A streptococcus (GAS) infection is a prerequisite for this disease. We attempted to differentiate socioeconomic from genetic risk factors by studying subjects in a Hawaii pediatric cardiology clinic who qualified for Medicaid. This ethnically diverse group was unique because they maintained a low socioeconomic but generally healthy lifestyle with more limited risks than those living in extremely impoverished conditions. METHODS: Questionnaires were administered to consenting subjects in the clinic, who were divided into those diagnosed with ARF (n = 26) and those with other (primarily congenital) heart diseases (n = 41). RESULTS: The socioeconomic status of the ARF and non-ARF groups was lower than that of the Hawaii population in general, and little differences were noted between the groups. The ARF group, however, had slightly larger household sizes and more children than the non-ARF group. The greatest difference was in ethnicity. By the Fisher exact test, the number of Polynesians belonging to the ARF group was significantly greater than all other ethnicities (P = .005). Polynesians had an odds ratio > 4.80 of developing ARF, which increased to 6.33 when number of children per household was considered. CONCLUSION: The potential contribution of genetic predisposing factors for developing ARF was analyzed in subjects living in a homogeneously low socioeconomic level relative to the general Hawaii population. Polynesians were at highest risk when compared to other ethnicities living in similar socioeconomic conditions.

Demographic and clinical characteristics of adolescents in Hawaii with obsessive-compulsive disorder.

BACKGROUND: A high prevalence rate of obsessive-compulsive disorder (OCD) among Hawaiian adolescents, particularly Native Hawaiians, has been reported. Because Native Hawaiian and other Polynesian youth are at an increased risk for rheumatic fever, caused by an autoimmune response to group A beta-hemolytic streptococci, we hypothesized that the genetic and environmental risk factors for streptococcal infections and their autoimmune sequelae potentially may be associated with the presence of OCD and may partially explain this high OCD prevalence. OBJECTIVE: To describe, among the adolescents in Hawaii diagnosed as having OCD through a previous study, OCD prevalence by ethnicity, household crowding and other measures of socioeconomic status, various measures of physical health and health-seeking behavior, and comorbid psychopathologic features. DESIGN: Six hundred nineteen adolescents from 5 high schools in the state of Hawaii were interviewed from April 15, 1993, to May 7, 1996. Interview instruments included the Diagnostic Interview Schedule for Children and other measurements of psychopathology. Obsessive-compulsive disorder diagnoses, based on current and past 6-month symptoms elicited via structured interview of the adolescents, were reported. RESULTS: Relative to other ethnicities, Native Hawaiians had a 2-fold higher risk (odds ratio = 2.03) for OCD. Degree of Polynesian ancestry correlated positively with OCD prevalence. Obsessive-compulsive disorder prevalence also correlated positively with crowding in the household; measures of physical illness; and measures of depression, anxiety, aggression, and illicit substance use. CONCLUSIONS: The characteristics of OCD in this sample suggest the need to consider the possibility of a streptococcal origin and the need for further studies to clarify the genetic and environmental risk factors for OCD in Hawaiian and other Polynesian youth.

Ethnic differences in risk for pediatric rheumatic illness in a culturally diverse population.

OBJECTIVE: To analyze the differences of occurrence of pediatric rheumatic disease among various ethnic groups in a culturally diverse isolated geographic area. METHODS: A retrospective study of pediatric rheumatic diseases in a multiethnic area during a 6 year period. RESULTS: A group of 922 patients was categorized based on predominant ethnicity, and their risk of having acute rheumatic fever (ARF), juvenile rheumatoid arthritis (JRA), and systemic lupus erythematosus (SLE) was studied. Odds ratios (OR) were computed for each illness with Caucasians as the reference group. Results indicated that Polynesians were overrepresented among patients with ARF, having elevated OR that were significantly different from Caucasians (22.5-120.7, p < 0.0001). For SLE, the highest OR were obtained for Samoans, Filipinos, and Japanese. In contrast, for JRA, Filipinos and Japanese had OR less than one, and no Samoans were diagnosed with JRA, possibly indicating a protective effect against developing JRA. CONCLUSION: This unique retrospective study examined the ethnic variations of expression of certain rheumatic diseases in an isolated region. Results reveal that certain ethnic groups are at risk for ARF and SLE, but are protected against JRA. These findings suggest investigating possible immunogenetic similarities and differences in these illnesses.