RESUMO
OBJECTIVE: To summarize the experience, performance and scientific output of long-running telemedicine networks delivering humanitarian services. METHODS: Nine long-running networks--those operating for five years or more--were identified and seven provided detailed information about their activities, including performance and scientific output. Information was extracted from peer-reviewed papers describing the networks' study design, effectiveness, quality, economics, provision of access to care and sustainability. The strength of the evidence was scored as none, poor, average or good. FINDINGS: The seven networks had been operating for a median of 11 years (range: 5-15). All networks provided clinical tele-consultations for humanitarian purposes using store-and-forward methods and five were also involved in some form of education. The smallest network had 15 experts and the largest had more than 500. The clinical caseload was 50 to 500 cases a year. A total of 59 papers had been published by the networks, and 44 were listed in Medline. Based on study design, the strength of the evidence was generally poor by conventional standards (e.g. 29 papers described non-controlled clinical series). Over half of the papers provided evidence of sustainability and improved access to care. Uncertain funding was a common risk factor. CONCLUSION: Improved collaboration between networks could help attenuate the lack of resources reported by some networks and improve sustainability. Although the evidence base is weak, the networks appear to offer sustainable and clinically useful services. These findings may interest decision-makers in developing countries considering starting, supporting or joining similar telemedicine networks.
Assuntos
Altruísmo , Eficiência Organizacional , Eficiência , Pesquisa sobre Serviços de Saúde/estatística & dados numéricos , Qualidade da Assistência à Saúde/estatística & dados numéricos , Telemedicina/organização & administração , Comportamento Cooperativo , Saúde Global , Pesquisas sobre Atenção à Saúde , Humanos , Modelos Organizacionais , Cultura Organizacional , Inquéritos e Questionários , Telemedicina/economia , Telemedicina/estatística & dados numéricosRESUMO
OBJECTIVE: The aim of this study was to evaluate the impact on pediatric care of the Pacific Asynchronous TeleHealth (PATH) system, a provider-to-provider teleconsultation platform utilized by military medical facilities throughout the Pacific Region. This review focuses on access to care, quality of care, and cost savings for the Department of Defense as a result of ongoing development of the PATH system from 2006 to 2009. METHODS: This is a retrospective review of 1,000 consecutive teleconsultations occurring from January 2006 to March 2009. Three pediatric subspecialists reviewed the characteristics of each teleconsultation and the ultimate outcome. RESULTS: The PATH system processed > 300 pediatric teleconsultations in 2009 from 20 hospitals and clinics throughout the Pacific Region. The number of teleconsultations has grown significantly since 2006. Median teleconsultation response time was 14.5 h with 97% of teleconsultations answered within 1 week. The majority (75%) of teleconsultations came from areas without host nation pediatric subspecialty resources. Most teleconsultations (72%) involved diagnostic questions, whereas 21% were primarily for treatment issues. Teleconsultations originated predominantly from outpatient clinics (90%), with question resolution in 60% of cases without a face-to-face subspecialty evaluation. Fifteen percent of teleconsultations resulted in patient transfer to our center for definitive diagnosis and treatment. The diagnostic and/or treatment plan was modified in 74% of teleconsultations. PATH precluded patient transfer in 12%-43% of teleconsultations (annual savings: $208,283-$746,348 per year) and generated an average of 1.7 relative value units per teleconsultation. CONCLUSIONS: PATH provided patient access to pediatric subspecialty expertise via provider-to-provider asynchronous teleconsultation. Internet-based pediatric subspecialty teleconsultation provides fast, convenient, cost-effective, quality pediatric care to populations of patients who might otherwise require transfer to a distant medical facility for more advanced care. PATH serves as a model for future asynchronous teleconsultation platforms in both the military and civilian healthcare arenas.
Assuntos
Pediatria/estatística & dados numéricos , Telemedicina/estatística & dados numéricos , Adolescente , Criança , Pré-Escolar , Atenção à Saúde/organização & administração , Atenção à Saúde/estatística & dados numéricos , Feminino , Guam , Havaí , Humanos , Lactente , Recém-Nascido , Japão , Coreia (Geográfico) , Masculino , Ilhas do Pacífico , Oceano Pacífico , Pediatria/organização & administração , Pediatria/normas , Avaliação de Programas e Projetos de Saúde/estatística & dados numéricos , Qualidade da Assistência à Saúde/normas , Qualidade da Assistência à Saúde/estatística & dados numéricos , Estudos Retrospectivos , Inquéritos e Questionários , Telemedicina/organização & administração , Telemedicina/normasRESUMO
Neurofibromatosis type 1 is an autosomal dominant disorder affecting the ras proto-oncogene. It is characterized by the overgrowth of nervous tissue and skin discoloration. While it is associated with a variety of phenotypic presentations, it is the plexiform variant that is particular concerning, as it can become extremely disfiguring and has a propensity for malignant degeneration. A case of a Pacific Islander with a large plexiform type 1 neurofibroma is presented. The patient was ultimately treated with surgical resection, negative pressure wound therapy, and split-thickness skin grafting with good results. A review of the literature concerning the diagnosis and treatment of neurofibromatosis is included.
Assuntos
Neurofibroma Plexiforme/patologia , Neurofibromatose 1/cirurgia , Neoplasias de Tecidos Moles/patologia , Adulto , Humanos , Masculino , Tratamento de Ferimentos com Pressão Negativa , Neurofibroma Plexiforme/cirurgia , Proto-Oncogene Mas , Transplante de Pele , Neoplasias de Tecidos Moles/cirurgiaRESUMO
BACKGROUND: Congenital diaphragmatic hernia (CDH) usually presents at birth with respiratory distress syndrome (RDS) and has a high mortality rate if not promptly recognized and treated. The incidence of CDH is reported to be 0.8 - 1.0/10,000 registered births. Less than 3% present after the neonatal period. In the latter, ie. late-presenting CDH (L-pCDH), the prognosis is improved because pulmonary hypoplasia does not develop. With the creation and application of a store-and-forward telemedicine system to the Pacific Island Health Care Project (PIHCP), we have come to realize that this rare condition ie., (L-pCDH) occurs with unusual frequency in the United States Associated Pacific Islands (USAPIs), especially those of the Federated States of Micronesia (FSM). METHODS: Information concerning CDH was identified from the archived data base of the over 3, 100 cases of the PIHCP as well as the composite data base of Tripler Army Medical Center (TAMC). The cases of CDH diagnosed after the neonatal period (> 30 days of age) were considered to have L-pCDH. The study period was from 1997 - 2006. RESULTS: During the study period (1997-2006), 12 cases of CDH were referred from the PIHCP. There was 1 case of CDH born at TAMC (beneficiary from Micronesia) during the same period. The number of births over the 10-year period of study was similar for the referral base (PIHCP) and TAMC. Five of the 12 cases in which the age at diagnosis could be established, were L-pCDH from the PIHCP. The one case of CDH born at TAMC, during the 10 year period, was diagnosed in the neonatal period (< 30 days of age). DISCUSSION: These results confirm that CDH occurs with increased frequency in the USAPIs as contrasted to the frequency in the developed world. The incidence of L-p CDH is far in excess in the USAPIs. This review highlights the fact that this group of patients can be easily identified using tele-techology (attached chest X-rays demonstrating stomach and/or bowel gas in the chest) thus allowing remote diagnosis and referral for ultimate surgical correction of this potentially lethal condition. Infants and children with CDH: provided excellent graduate medical education (GME) for residents-in-training; are afforded advanced surgical treatment unavailable in the remote jurisdictions of the Pacific; and, following surgery, are restored to health to return to their homes so as to become contributing members of society. CONCLUSION: CDH, especially L-pCDH occurs with increased frequency in the USAPIs. The Web-based-store-and-forward telemedicine PIHCP allows for remote diagnosis. Patients so identified are transported to TAMC for definitive treatment. These patients provide excellent GME to our residents-in-training. Following surgical correction these patients are restored to health to return to their homes to mature and become contributing members of society.
Assuntos
Hérnias Diafragmáticas Congênitas , Feminino , Hérnia Diafragmática/epidemiologia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Ilhas do Pacífico/epidemiologia , PrognósticoRESUMO
A young Marshallese woman presented with the insidious development of fever, cough, fatigue, profound weakness, massive weight loss, cachexia, alopecia, amenorrhea, and periumbilical hyperpigmentation. Limited laboratory studies revealed anemia, leukocytosis, and hyponatremia. Imaging studies, as well as digital photographs, transmitted over the Internet, using the secure Pacific Island Health Care Project (PIHCP), store-and-forward telemedicine system, suggested the diagnosis of disseminated tuberculosis, and antimycobacterial antibiotics were begun. Sputum cultures eventually grew Mycobacterium tuberculosis. Based on the constellation of clinical signs and symptoms, the transmitted images, and limited laboratory data, adrenal tuberculosis (Addison's disease) with adrenal insufficiency was diagnosed and corticosteroids were initiated. The patient responded dramatically This case underscores the utility of telemedicine in the diagnosis and treatment of patients with unusual conditions, rarely seen today in the United States, from remote sites in the Developing World.
Assuntos
Doença de Addison/etiologia , Telemedicina/organização & administração , Tuberculose Pulmonar/complicações , Doença de Addison/tratamento farmacológico , Adulto , Antituberculosos/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Humanos , Micronésia , Tuberculose Pulmonar/tratamento farmacológico , Adulto JovemRESUMO
In 1990, an 18-month-old Micronesian girl was initially diagnosed with a right adrenocortical carcinoma. More than a decade later (2003), she was diagnosed with metastatic osteosarcoma with the primary in her right proximal fibula. Given this child's remarkable history of malignancy, she underwent testing for a genetic mutation that is associated with increased cancer formation. One such cancer syndrome is called Li-Fraumeni syndrome where approximately 70% of patients carry a genetic mutation in the p53 tumor suppressor gene. Patients with LFS are at risk for developing cancers of the breast, soft tissues, brain, bone, adrenal gland, and blood cells. Mutational analysis of our patient did reveal the presence of a germline mutation of the p53 tumor suppressor gene. She was found to have a base pair change (A-->C) at nucleotide 394 resulting in a lysine to glutamine amino acid change at codon 132 (K132Q), which remarkably has never been described in association with either adrenocortical carcinoma or osteosarcoma.
Assuntos
Neoplasias do Córtex Suprarrenal/genética , Carcinoma Adrenocortical/genética , Neoplasias Ósseas/genética , Mutação em Linhagem Germinativa/genética , Segunda Neoplasia Primária/genética , Osteossarcoma/genética , Proteína Supressora de Tumor p53/genética , Neoplasias do Córtex Suprarrenal/patologia , Carcinoma Adrenocortical/patologia , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Códon , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Micronésia , Segunda Neoplasia Primária/diagnóstico por imagem , Segunda Neoplasia Primária/patologia , Osteossarcoma/diagnóstico por imagem , Osteossarcoma/patologia , RadiografiaRESUMO
Rheumatic heart disease (RHD) continues to be a major health problem in developing countries. The burden of disease in many countries, especially those of Oceania, is very high and is still the leading cause of heart-related deaths. Several factors contribute to the prevalence of RHD in the Pacific Basin including poverty, poor access to care, distance for travel, and limited resources. The Pacific Island Health Care Project (PIHCP) at Tripler Army Medical Center (TAMC) is a unique program which provides indigenous, medically under-served peoples in the United States Associated Pacific Islands (USAPIs) with definitive medical and surgical care. The program has been an important source of patients to enhance Graduate Medical Education (GME) at TAMC. Beginning in 1998, a secure, Web-based, store-and-forward telemedicine network was developed. It was ultimately deployed to 11 sites in the USAPIs. This unique platform has facilitated the selection and definitive care of Pacific Islanders at TAMC. The purpose of this study was to review our experience with RHD in patients referred from the USAPIs utilizing a unique telemedicine system. All patient records that were archived in the PIHCP database were retrospectivelyreviewed for the diagnosis of RHD from 1998 (telemedicine program began) to 2006. Descriptive analysis of the data is displayed in a tabular format. Of the 150 patient consults with RHD in the PIHCP 76 were accepted for care at TAMC and 74 came to Honolulu. Most patients were younger than 40 years of age. Almost all patients evaluated at TAMC had mitral valve involvement, and 81% of patients underwent a surgical procedure to correct the valvular disease. Our experience with RHD and its management illustrates a number of challenges that must be addressed by those who attempt to provide technically advanced care to persons from the developing world. In the case of RHD, patient selection, choice of intervention, and early return of the patient home are critical to the success of any such program. Despite these problems the PIHCP has restored many patients to health and returned them to their island homes as contributing members of society.
Assuntos
Cardiopatia Reumática/diagnóstico , Cardiopatia Reumática/terapia , Telemedicina/estatística & dados numéricos , Adolescente , Adulto , Criança , Demografia , Feminino , Pesquisa sobre Serviços de Saúde , Humanos , Masculino , Insuficiência da Valva Mitral/diagnóstico , Insuficiência da Valva Mitral/cirurgia , Oceania , Consulta Remota/estatística & dados numéricosRESUMO
BACKGROUND: Pediatric subspecialists are often separated from the children who need them by distance, time, or socioeconomic factors. The Electronic Children's Hospital of the Pacific is an Internet-based store-and-forward pediatric consultation system established to overcome these barriers. OBJECTIVE: To characterize the use of the Electronic Children's Hospital of the Pacific and its impact on access to specialty care, the quality of the care provided, and cost savings. DESIGN: Prospective trial. SETTING: Twenty-two military treatment facilities in the Pacific. PARTICIPANTS: Primary care providers, pediatric consultants, and 5 reviewers. MAIN OUTCOME MEASURES: Consult response time, physician panel review, and evacuation cost avoidance. RESULTS: There were 267 cases from 16 sites. The mean +/- SD response time by a consultant was 32 +/- 8 hours. The panel review deemed that the initial diagnosis was changed or modified in 15% (39/267) of the cases, the diagnostic plan was changed or modified in 21% (57/267), and the treatment plan was changed or modified in 24% (64/267) (P < .01 for all). Routine air evacuations to a tertiary care medical center were avoided in 32 cases (12%), with an estimated cost savings of $185 408. CONCLUSIONS: The Electronic Children's Hospital of the Pacific improved the quality of patient care by providing expeditious specialty consultation. Significant cost avoidance in this military pediatric population was documented. Store-and-forward Internet-based teleconsultation is an effective means of providing pediatric subspecialty consultation to a population of underserved children.
Assuntos
Hospitais Pediátricos , Internet , Telemedicina/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Acessibilidade aos Serviços de Saúde , Humanos , Lactente , Recém-Nascido , Estudos Prospectivos , Consulta Remota/economia , Consulta Remota/métodos , Telemedicina/economia , Comunicação por VideoconferênciaRESUMO
To evaluate the benefit of the Pacific Island Health Care Project (PIHCP) to our orthopedic graduate medical education program, we performed a retrospective review of our 8-year operative experience with patients referred through this program. Between July 1994 and June 2002, 69 patients underwent 79 orthopedic operative procedures. Patients were categorized by primary diagnosis, anatomic site involved, and surgical treatment rendered. Because many of the patients referred from the PIHCP with tumors were noted to have either unusually large lesions or advanced-stage disease, further analyses of tumor stage and pathologic grade were made. Seven of the 14 oncologic cases surgically treated in our department in the past 8 years were referrals from the PIHCP. Unique operative procedures performed for these tumor patients included one forequarter amputation, one hip disarticulation, one hemipelvectomy, two partial scapulectomies, and one distal ulna excision. We conclude that the PIHCP referrals provide an important and relatively unique contribution to the clinical and operative experience of our orthopedic residents. These patients from the Pacific basin also enhance our orthopedic graduate medical education program by exposing our residents to the special socioeconomic and cultural issues related to caring for people from developing insular countries.
Assuntos
Hospitais Militares/estatística & dados numéricos , Internet , Medicina Militar/educação , Procedimentos Ortopédicos/estatística & dados numéricos , Ortopedia/educação , Encaminhamento e Consulta , Consulta Remota , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Educação de Pós-Graduação em Medicina , Feminino , Havaí , Acessibilidade aos Serviços de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Ilhas do Pacífico , Avaliação de Programas e Projetos de Saúde , Estudos Retrospectivos , Transporte de PacientesRESUMO
INTRODUCTION: Tripler Army Medical Center (TAMC) in Honolulu, Hawaii, is uniquely situated to serve patients from the United States Associated Pacific Islands (USAPIs) through the congressionally funded Pacific Island Health Care Project (PIHCP). Because of time differences and distance, a web-based store-and-forward consultation and referral network was established using the internet to more efficiently and economically facilitate patient care. Using both electronic and hard copy records, we sought to establish the incidence of Hirschsprung Disease (HD) in children from the USAPI and contrast it to that of the developed world. METHODS: PIHCP website records as well as all the inpatient and outpatient medical records of patients referred to TAMC fortreatment of HD from 1994 to 2002 were reviewed. A diagnosis of HD was confirmed in all cases with full thickness biopsy. Incidence figures for HD are based on this review and on the birth rates for these islands from the International Data Base of the U.S. Bureau of the Census. RESULTS: There were 14 cases of short-segment HD referred over a nine year study period. Nine patients came from the Federated States of Micronesia (FSM) with an average annual incidence of 1:3190, which is 1.5 to 2 times the reported incidence in Western nations. Remarkably, seven of these nine were from Pohnpei State, capital of the FSM (annual incidence of 1:1370 or3-5 times that in the West). Three patients came from the Republic of the Marshall Islands (RMI), and two came from American Samoa (AS). There were no reported consanguineous marriages, associated syndromes, or complications of surgery. CONCLUSION: HD was found to be up to 2-3 times more common among people from the FSM than has been reported in the developed world. Given the limitations of providing care and obtaining data from all the USAPls with a population that is spread over a massive expanse of ocean larger than the continental United States, this incidence is likely an underestimation of HD among Pacific Islanders. A secure web-based referral network developed in 1998 has been invaluable in collecting epidemiologic data from these islands as well as in providing health care workers in the USAPI with an efficient and inexpensive means to seek consultation from specialists and sub-specialists at a major tertiary care medical facility.
Assuntos
Doença de Hirschsprung/epidemiologia , Havaiano Nativo ou Outro Ilhéu do Pacífico/estatística & dados numéricos , Doença de Hirschsprung/etnologia , Humanos , Incidência , Estudos Retrospectivos , Estados Unidos/epidemiologiaRESUMO
In a series of recent articles, we suggest that family dentists, military dentists and psychiatrists with expertise in posttraumatic stress disorder (especially in the Veterans Health Administration) are likely to see an increased number of patients with symptomatic jaw-clenching and early stages of tooth-grinding (Bracha et al., 2005). Returning warfighters and other returnees from military deployment may be especially at risk for high rates of clenching-induced masticatory muscle disorders at early stages of incisor grinding. The literature we have recently reviewed strongly supports the conclusion that clenching and grinding may primarily be a manifestation of experiencing extreme fear or severe chronic distress (respectively). We have recently reviewed the clinical and paleoanthropological literature and have noted that ancestral warfare and ancestral combat, in the early Paleolithic Environment of Evolutionary Adaptedness (EEA) may be a neglected factor explaining the conservation of the archaic trait of bite-muscle strengthening. We have hypothesized that among ancestral warriors, jaw clenching may have rapidly strengthened the two primary muscles involved in biting, the masseter muscles and the much larger temporalis muscles. The strengthening of these muscles may have served the purpose of enabling a stronger, deeper, and therefore more lethal, defensive bite for early Paleolithic humans. The neuroevolutionary perspective presented here may be novel to many dentists. However, it may be useful in patient education and in preventing progression from jaw-clenching to chronic facial pain.
Assuntos
Força de Mordida , Bruxismo/etiologia , Dor Facial/etiologia , Músculos da Mastigação/fisiologia , Guerra , Evolução Biológica , HumanosRESUMO
BACKGROUND: Telemedicine networks, which deliver humanitarian services, sometimes need to share expertise to find particular experts in other networks. It has been suggested that a mechanism for sharing expertise between networks (a 'clearing house') might be useful. OBJECTIVE: To propose a mechanism for implementing the clearing house concept for sharing expertise, and to confirm its feasibility in terms of acceptability to the relevant networks. DESIGN: We conducted a needs analysis among eight telemedicine networks delivering humanitarian services. A small proportion of consultations (5-10%) suggested that networks may experience difficulties in finding the right specialists from within their own resources. With the assistance of key stakeholders, many of whom were network coordinators, various methods of implementing a clearing house were considered. One simple solution is to establish a central database holding information about consultants who have agreed to provide help to other networks; this database could be made available to network coordinators who need a specialist when none was available in their own network. RESULTS: The proposed solution was examined in a desktop simulation exercise, which confirmed its feasibility and probable value. CONCLUSIONS: This analysis informs full-scale implementation of a clearing house, and an associated examination of its costs and benefits.
Assuntos
Altruísmo , Redes de Comunicação de Computadores/organização & administração , Encaminhamento e Consulta/organização & administração , Telemedicina/organização & administração , Redes de Comunicação de Computadores/normas , Comportamento Cooperativo , Bases de Dados Factuais , Países em Desenvolvimento , Estudos de Viabilidade , Humanos , Disseminação de Informação/métodos , Avaliação das Necessidades , Encaminhamento e Consulta/normas , Especialização , Telemedicina/normasRESUMO
Seven long-running telemedicine networks were surveyed. The networks provided humanitarian services (clinical and educational) in developing countries, and had been in operation for periods of 5-15 years. The number of experts serving each network ranged from 15 to 513. The smallest network had a total of 10 requesters and the largest one had more than 500 requesters. The networks operated in nearly 60 countries. The seven networks managed a total of 1857 cases in 2011, i.e. an average of 265 cases per year per network. There was a significant growth in total activity, amounting to 100.3 cases per year during the 15 year study period. In 2011, network activity was 50-700 teleconsultations per network. There were clear differences in the patterns of activity, with some networks managing an increasing caseload, and others managing a slowly reducing caseload. The seven networks had published a total of 44 papers listed in Medline which summarized the evidence resulting from the delivery of services by telemedicine. There was a dearth of information about clinical and cost-effectiveness. Nevertheless, the services were widely appreciated by referring doctors, considered to be clinically useful, and there were indications that clinical outcomes for telemedicine patients were often improved. Despite a lack of formal evidence, the present study suggests that telemedicine can provide clinically useful services in developing countries.
Assuntos
Altruísmo , Países em Desenvolvimento/estatística & dados numéricos , Telemedicina/organização & administração , Pesquisa sobre Serviços de Saúde , Humanos , Modelos Organizacionais , Inquéritos e Questionários , Telemedicina/normas , Telemedicina/estatística & dados numéricosAssuntos
Neoplasias da Mama/complicações , Hipoglicemia/etiologia , Insulina/metabolismo , Tumor Filoide/complicações , Adulto , Neoplasias da Mama/metabolismo , Neoplasias da Mama/patologia , Neoplasias da Mama/cirurgia , Feminino , Humanos , Secreção de Insulina , Mastectomia , Tumor Filoide/metabolismo , Tumor Filoide/patologia , Tumor Filoide/cirurgiaRESUMO
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.
Assuntos
Artrite Juvenil/epidemiologia , Lúpus Eritematoso Sistêmico/epidemiologia , Ambulatório Hospitalar , Pediatria/métodos , Encaminhamento e Consulta/estatística & dados numéricos , Reumatologia/métodos , Adolescente , Adulto , Artrite Juvenil/diagnóstico , Criança , Pré-Escolar , Havaí/epidemiologia , Hospitais Rurais , Hospitais Urbanos , Humanos , Lactente , Lúpus Eritematoso Sistêmico/diagnóstico , Prevalência , Encaminhamento e Consulta/tendências , População Rural , Saúde da População UrbanaRESUMO
OBJECTIVE AND IMPORTANCE: Carcinoma of the adnexal structures of the skin is a rare malignancy, and is even more unusual in the scalp. We report an unusual case of scalp adnexal carcinoma of eccrine origin that went untreated for years, resulting in a giant tumor with extension through the cranium. The tumor resection and reconstruction of the cranium and scalp defects posed unique challenges. CLINICAL PRESENTATION: A 54-year-old woman experienced a large recurrence of her scalp adnexal carcinoma after an incomplete wide local excision, which invaded through the cranium. INTERVENTION: The entire vertex of the scalp and cranium were removed en bloc. After cranioplasty, a free vascularized muscle flap was used for soft tissue coverage, but failed owing to poor vascular inflow. A large area of dura was left open, using a vacuum-assisted wound closure device to generate granulation tissue by secondary intention. Another split thickness skin graft was used to provide a cosmetically acceptable outcome. CONCLUSION: Scalp adnexal tumors of eccrine origin rarely metastasize and can be resected for cure with complete removal. Reconstruction options for large scalp and cranial tumors may be limited, and allowing the dura to granulate by secondary intention has been very rarely described. The novel use of a vacuum-assisted wound closure device was a very useful adjunct in this situation, and may be beneficial in the reconstruction of other patients with large scalp and cranial defects after neurosurgical procedures. It should be used with caution, since it may risk injury to a major venous sinus, especially when used in the midline, or cerebrospinal fluid leakage.
Assuntos
Adenocarcinoma/cirurgia , Neoplasias Ósseas/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Neoplasias Cutâneas/cirurgia , Curetagem a Vácuo , Adenocarcinoma/patologia , Neoplasias Ósseas/secundário , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologia , Transplante de Pele/métodos , Derrame Subdural/etiologia , Derrame Subdural/cirurgiaRESUMO
AVP synthesis, storage, and osmotically stimulated release are reduced in young adult rats exposed prenatally to ethanol (PE). Whether the reduced release of AVP to the osmotic stimulus is due to impairment of the vasopressin system or specifically to an osmoreceptor-mediated release is not known. The present experiments were done, therefore, to determine whether a hemorrhage-induced AVP response would also be diminished in PE-exposed rats. Pregnant rats were fed either a control liquid diet [no prenatal ethanol (NPE)] or a liquid diet with 35% of the calories from ethanol from days 7-21 of pregnancy. Offspring were weaned at 3 wk of life. At 11 wk of age, femoral arterial catheters were surgically placed, and blood volumes were determined at 12 wk. Three days later, two hemorrhages of 10% of the blood volume were performed with samples taken before and 10 min after the hemorrhages. After a 20% blood loss, plasma AVP was 19% higher in NPE rats than in the PE rats despite no differences in mean arterial blood pressure (MABP). Also, hypothalamic AVP mRNA and pituitary AVP content were reduced in PE rats. Furthermore, confirming an earlier report of sex differences in AVP release, the hemorrhage-induced hormone response was twofold greater in female rats than male rats, regardless of previous ethanol exposure. These studies demonstrate that the AVP response to hemorrhage is reduced in PE rats independently of differences in MABP. The data are compatible with a theory of a reduced number of hemorrhage-responsive vasopressinergic neurons capable of stimulated AVP release in PE rats.
Assuntos
Arginina Vasopressina/metabolismo , Etanol/farmacologia , Hemorragia/metabolismo , Efeitos Tardios da Exposição Pré-Natal/metabolismo , Caracteres Sexuais , Animais , Pressão Sanguínea , Peso Corporal , Feminino , Frequência Cardíaca , Hematócrito , Masculino , Gravidez , Ratos , Ratos Sprague-DawleyRESUMO
OBJECTIVE: To examine the genotypic and phenotypic characteristics of a Micronesian kindred with autosomal dominant precocious osteoarthritis (OA). METHODS: We reviewed records and radiographs of 3 index patients and their parents, administered questionnaires to 16 additional kindred members, performed whole-genome scans of 24 family members, and sequenced relevant genes from 16 family members. RESULTS: The kindred displayed early onset OA, enlarged epiphyses, platyspondyly, and brachydactyly with dysplastic findings consistent with mild spondyloepiphyseal dysplasia. Genetic analysis revealed an arginine to cysteine substitution at position 75 of the collagen 2A1 gene, a mutation that has been described in 4 other geographically distinct families. The major phenotypic differences among the families were in height (ranging from short to tall) and hearing loss noted in 3 of the 5 families. CONCLUSION: The presence of the COL2A1 Arg75Cys mutation in 5 geographically distinct areas helps to confirm a potential mutational hotspot. The diverse phenotypic spectrum suggests that modifier genes and environmental factors play a role in the expression of this mutation.
Assuntos
Colágeno Tipo II/genética , Predisposição Genética para Doença , Mutação de Sentido Incorreto/genética , Osteoartrite do Quadril/genética , Osteoartrite do Joelho/genética , Osteocondrodisplasias/genética , Arginina/genética , Cisteína/genética , Saúde da Família , Feminino , Testes Genéticos , Humanos , Masculino , Repetições de Microssatélites , Osteoartrite do Quadril/diagnóstico por imagem , Osteoartrite do Quadril/fisiopatologia , Osteoartrite do Joelho/diagnóstico por imagem , Osteoartrite do Joelho/fisiopatologia , Osteocondrodisplasias/diagnóstico por imagem , Linhagem , Reação em Cadeia da Polimerase , RadiografiaRESUMO
Physicians from the Republic of Palau have participated in the Pacific Island Health Care Project (PIHCP) sponsored by Tripler Army Medical Center (TAMC) since the projects inception in 1990. The PIHCP was developed to provide exceptional graduate medical educational (GME) benefit to physicians-in-training at TAMC while at the same time to supply the medically underserved indigenous peoples of the United States Associated Pacific Islands (USAPIs) with advanced medical and surgical care. Coordination and management of patient referrals utilizing standard means (telephone, fax, letter) had become virtually impossible. A patient referral submitted via the Internet in December 1997 to the PIHCP from Palau was the stimulus needed to develop a telemedicine solution to the whole referral process. With the technical assistance of Project Akamai and the support of the US Congress, I was able to develop a simple, store-and-forward platform for the consultation and referral of patients Palau. Palau, a country of about 20,000 or 4% of the total population of the USAPIs (> 500,000) submitted > 20% of the cases. from the USAPIs to TAMC. Since that time 2500 cases have been submitted to the PIHCP. Of those cases 511 have been from A spectrum patients with of well described, well documented disease conditions has been provided by the physicians from Palau. Many cases have supporting imagery. Both sexes and all ages are well represented. Patients with cancer lead the list of referred patients. For the last several years the PIHCP provided Palauans with nearly $1,000,000 annually in medical care. The long and collegial relationship between the Republic of Palau and TAMC has been rewarding. This relationship has been strengthened by the PICHP.