Defining health law or the edgewood syndrome

ABSTRACT - The authors’ main purpose is to present ideas on defining Health Law by highlighting the particularities of the field of Health Law as well as of the teaching of this legal branch, hoping to contribute to the maturity and academic recognition of Health Law, not only as a very rich legal field but also as a powerful social instrument in the fulfillment of fundamental human rights. The authors defend that Health Law has several characteristics that distinguish it from traditional branches of law such as its complexity and multidisciplinary nature. The study of Health Law normally covers issues such as access to care, health systems organization, patients’ rights, health professionals’ rights and duties, strict liability, healthcare contracts between institutions and professionals, medical data protection and confidentiality, informed consent and professional secrecy, crossing different legal fields including administrative, antitrust, constitutional, contract, corporate, criminal, environmental, food and drug, intellectual property, insurance, international and supranational, labor/employment, property, taxation, and tort law. This is one of the reasons why teaching Health Law presents a challenge to the teacher, which will have to find the programs, content and methods appropriate to the profile of recipients which are normally non jurists and the needs of a multidisciplinary curricula. By describing academic definitions of Health Law as analogous to Edgewood, a fiction house which has a different architectural style in each of its walls, the authors try to describe which elements should compose a more comprehensive definition. In this article Biolaw, Bioethics and Human Rights are defined as complements to a definition of Health Law: Biolaw because it is the legal field that treats the social consequences that arise from technological advances in health and life sciences; Bioethics which evolutions normally influence the shape of the legal framework of Health; and, finally Human Rights theory and declarations are outlined as having always been historically linked to medicine and health, being the umbrella that must cover all the issues raised in the area of Health Law. To complete this brief incursion on the definition on Health Law the authors end by giving note of the complex relations between this field of Law and Public Health. Dealing more specifically on laws adopted by governments to provide important health services and regulate industries and individual conduct that affect the health of the populations, this aspect of Health Law requires special attention to avoid an imbalance between public powers and individual freedoms. The authors conclude that public trust in any health system is essentially sustained by developing health structures which are consistent with essential fundamental rights, such as the universal right to access health care, and that the study of Health Law can contribute with important insights into both health structures and fundamental rights in order to foster a health system that respects the Rule of Law.-------------------------- RESUMO – O objectivo principal dos autores é apresentar ideias sobre a definição de Direito da Saúde, destacando as particularidades desta área do direito, bem como do ensino deste ramo jurídico, na esperança de contribuir para a maturidade e para o reconhecimento académico do mesmo, não só como um campo juridicamente muito rico, mas, também, como um poderoso instrumento social no cumprimento dos direitos humanos fundamentais. Os autores defendem que o Direito da Saúde tem diversas características que o distinguem dos ramos tradicionais do direito, como a sua complexidade e natureza multidisciplinar. O estudo do Direito da Saúde abrangendo normalmente questões como o acesso aos cuidados, a organização dos sistemas de saúde, os direitos e deveres dos doentes e dos profissionais de saúde, a responsabilidade civil, os contratos entre instituições de saúde e profissionais, a protecção e a confidencialidade de dados clínicos, o consentimento informado e o sigilo profissional, implica uma abordagem transversal de diferentes áreas legais, incluindo os Direitos contratual, administrativo, antitrust, constitucional, empresarial, penal, ambiental, alimentar, farmacêutico, da propriedade intelectual, dos seguros, internacional e supranacional, trabalho, fiscal e penal. Esta é uma das razões pelas quais o ensino do Direito da Saúde representa um desafio para o professor, que terá de encontrar os programas, conteúdos e métodos adequados ao perfil dos destinatários, que são normalmente não juristas e às necessidades de um currículo multidisciplinar. Ao descrever as várias definições académicas de Direito da Saúde como análogas a Edgewood, uma casa de ficção que apresenta um estilo arquitectónico diferente em cada uma de suas paredes, os autores tentam encontrar os elementos que deveriam compor uma definição mais abrangente. No artigo, Biodireito, Bioética e Direitos Humanos são descritos como complementos de uma definição de Direito da Saúde: o Biodireito, dado que é o campo jurídico que trata as consequências sociais que surgem dos avanços tecnológicos na área da saúde e das ciências da vida; a Bioética cujas evoluções influenciam normalmente o quadro jurídico da Saúde; e, por fim, a teoria dos Direitos Humanos e as suas declarações as quais têm estado sempre historicamente ligadas à medicina e à saúde, devendo funcionar como pano de fundo de todas as questões levantadas na área do Direito da Saúde. Para finalizar a sua breve incursão sobre a definição de Direito da Saúde, os autores dão ainda nota das complexas relações entre este último e a Saúde Pública, onde se tratam mais especificamente as leis aprovadas pelos governos para regular os serviços de saúde, as indústrias e as condutas individuais que afectam a saúde das populações, aspecto do Direito da Saúde que requer uma atenção especial para evitar um desequilíbrio entre os poderes públicos e as liberdades individuais. Os autores concluem afirmando que a confiança do público em qualquer sistema de saúde é, essencialmente, sustentada pelo desenvolvimento de estruturas de saúde que sejam consistentes com o direito constitucional da saúde, tais como o direito universal ao acesso a cuidados de saúde, e que o estudo do Direito da Saúde pode contribuir com elementos

Transformation of a Cutaneous Follicle Center Lymphoma to a Diffuse Large B-Cell Lymphoma - An Unusual Presentation

Primary cutaneous follicle center lymphoma (PCFCL) is characterized by a proliferation of follicle center cells in the skin. A definitive diagnosis is frequently delayed because of difficulties in interpretation of the histopathologic findings. It has an excellent prognosis with a 5-year survival over 95% and its risk of transformation has not been established. We describe a case report of man with a gastric diffuse large B-cell lymphoma (DLBCL) referred to our clinic because of nodules in the back that had gradually developed over a period of 10 years. A biopsy performed 3 years before was interpreted as reactive follicular hyperplasia. A new skin biopsy revealed a diffuse large B-cell lymphoma and immunoglobulin heavy chain gene rearrangements from the initial skin biopsy (PCBCL) and the DLBCL gastric biopsy were studied by polymerase chain reaction and an identical clonal rearrangement was detected which was highly suggestive of a transformation lymphoma.

Hand Involvement in Ollier Disease and Maffucci Syndrome: a Case Series

Ollier Disease and Maffucci Syndrome are two rare diseases that can cause tumors in several organs, having a special predilection for the hand. However, there have been very few reports in the literature focusing on hand manifestations of these diseases. We report the cases of three female patients: one with Ollier Disease, and two other with Maffucci Syndrome. All patients had hand involvement as their initial primary complaint. The Ollier Disease patient developed chondrosarcomas of two digits and had to have these fingers amputated. One of the Maffucci patients died one year after presentation from a brain glioblastoma. These cases emphasize the importance of early diagnosis of Ollier Disease and Maffucci Syndrome, as these two conditions are associated not only to crippling hand deformity, but also to a significant risk of chondrosarcoma, and other malignant tumors.

A Posterior Interosseous Nerve Syndrome Associated With Spontaneous Rupture of the Extensor Pollicis Longus Tendon

Posterior interosseous nerve entrapment syndrome and spontaneous rupture of the extensor pollicis longus tendon are rare conditions. The authors describe the bizarre combination of a spontaneous rupture of the extensor pollicis longus tendon in a 82-year-old lady with a posterior interosseous nerve syndrome. As far as the authors know, this is the first description of such an association in the literature. Surgical exploration revealed compression of the posterior interosseous nerve at the proximal portion of the supinator muscle and at Henry's leash. The nerve was freed, and the tendon of the extensor index proprius was transferred to the extensor pollicis longus. Six months after the procedure, the patient had resumed her daily activities, showing a good functional result.

Dapsone hypersensitivity syndrome in an adolescent during treatment during of leprosy

A 12 y old girl was admitted 24 days after start a WHO multidrug therapy scheme for multibacillary leprosy (dapsone, clofazimine and rifampicin) with intense jaundice, generalized lymphadenopathy, hepatoesplenomegaly, oral erosions, conjunctivitis, morbiliform rash and edema of face, ankles and hands. The main laboratory data on admission included: hemoglobin, 8.4 g/dL; WBC, 15,710 cells/mm³; platelet count, 100,000 cells/mm³; INR = 1.49; increased serum levels of aspartate and alanine aminotransferases, gamma-glutamyl transpeptidase, alkaline phosphatase, direct and indirect bilirubin. Following, the clinical conditions had deteriorated, developing exfoliative dermatitis, shock, generalized edema, acute renal and hepatic failure, pancytopenia, intestinal bleeding, pneumonia, urinary tract infection and bacteremia, needing adrenergic drugs, replacement of fluids and blood product components, and antibiotics. Ten days after admission she started to improve, and was discharged to home at day 39th, after start new supervised treatment for leprosy with clofazimine and rifampicin, without adverse effects. This presentation fulfils the criteria for the diagnosis of dapsone hypersensitivity syndrome (fever, generalized lymphadenopathy, exfoliative rash, anemia and liver involvement with mixed hepatocellular and cholestatic features). Physicians, mainly in geographical areas with high prevalence rates of leprosy, should be aware to this severe, and probably not so rare, hypersensitivity reaction to dapsone.

Cutaneous anthrax in Lima, Peru: retrospective analysis of 71 cases, including four with a meningoencephalic complication

Anthrax is a zoonosis produced by Bacillus anthracis, and as an human infection is endemic in several areas in the world, including Peru. More than 95% of the reported naturally acquired infections are cutaneous, and approximately 5% of them can progress to meningoencephalitis. In this study we review the clinical and epidemiological characteristics of the patients with diagnosis of cutaneous anthrax evaluated between 1969 and 2002 at the Hospital Nacional Cayetano Heredia (HNCH) and the Instituto de Medicina Tropical Alexander von Humboldt in Lima, Peru. Seventy one patients were included [49/71 (69%) of them men], with a mean age of 37 years. The diagnoses were classified as definitive (44%) or probable (56%). The most common occupation of the patients was agriculture (39%). The source of infection was found in 63 (88.7%) patients. All the patients had ulcerative lesions, with a central necrosis. Most of the patients (65%) had several lesions, mainly located in the upper limbs (80%). Four patients (5.6%) developed meningoencephalitis, and three of them eventually died. In conclusion, considering its clinical and epidemiological characteristics, cutaneous anthrax must be included in the differential diagnosis of skin ulcers. A patient with clinical suspicion of the disease should receive effective treatment soon, in order to avoid neurological complications which carry a high fatality rate.

The histopathology of cutaneous leishmaniasis due to Leishmania (Leishmania) mexicana in the Yucatan peninsula, Mexico

Localized Cutaneous Leishmaniasis (LCL) known as "chiclero's ulcer" in southeast Mexico, was described by SEIDELIN in 1912. Since then the sylvatic region of the Yucatan peninsula has been documented as an endemic focus of LCL. This study of 73 biopsies from parasitological confirmed lesions of LCL cases of Leishmania (Leishmania) mexicana infection was undertaken: 1) to examine host response at tissue level; and 2) to relate manifestations of this response to some characteristics of clinical presentation. Based on Magalhães' classification we found that the most common pattern in our LCL cases caused by L. (L.) mexicana was predominantly characterized by the presence of unorganized granuloma without necrosis, (43.8%). Another important finding to be highlighted is the fact that in 50/73 (68.5%) parasite identification was positive. There was direct relation between the size of the lesion and time of evolution (r s = 0.3079, p = 0.03), and inverse correlation between size of the lesion and abundance of amastigotes (r s = -0.2467, p = 0.03). In view of the complexity of clinical and histopathological findings, cell-mediated immune response of the disease related to clinical and histopathological features, as so genetic background should be studied.

Síndrome de Hipersensibilidade ao Alopurinol Simulando Linfoma Cutâneo

As reacções cutâneas secundárias às drogas são frequentes (em 2 a 3% dos doentes hospitalizados); embora na maioria sem gravidade, num pequeno número de casos podem atingir elevada morbilidade e mortalidade. É por isso essencial o rápido reconhecimento das reacções mais graves, como por exemplo síndrome de Stevens-Johnson, necrólise epidérmica tóxica ou síndrome de hipersensibilidade. Neste último as drogas mais frequentemente responsáveis são os antiepilépticos, sulfonamidas e alopurinol. Os síndromes de hipersensibilidade a fármacos assumem raramente características clínicas e sobretudo histológicas quase indistinguíveis dos verdadeiros linfomas cutâneos. Nestes casos a diferenciação entre estas duas entidades, embora difícil, é muito importante pelas naturais implicações terapêuticas. A este propósito os autores descrevem o caso clínico de um doente de 51 anos, com febre elevada de início abrupto e eritrodermia, em que o exame histológico das biópsias cutânea e ganglionar foi de linfoma cutâneo de células T com envolvimento ganglionar específico. Veio no entanto a apurar-se que o doente tinha sido medicado com alopurinol duas semanas antes do início da febre. As características clínico-laboratoriais e a evolução vieram a comprovar o diagnóstico de síndrome de hipersensibilidade ao alopurinol.

Sensitivity of an immunoenzymatic test for detection of ant-L. brasiliensis antibodies compared to other tests used for the diagnosis of American cutaneous leishmaniasis

The diagnosis of American cutaneous leishmaniasis (ACL) is frequently based on clinical and epidemiological data associated with the results of laboratory tests. Some laboratory methods are currently being applied for the diagnosis of ACL, among them the indirect immunofluorescence reaction (IIFR), the Montenegro skin test (MST), histopathological examination, and the polymerase chain reaction (PCR). The performance of these methods varies in a considerable proportion of patients. After the standardization of an immunoenzymatic test (ELISA) for the detection of IgG in the serum of patients with ACL using a crude Leishmania braziliensis antigen, the results obtained were compared to those of other tests routinely used for the diagnosis. The tests revealed the following sensitivity, when analyzed separately: 85% for ELISA IgG, 81% for PCR, 64.4% for MST, 58.1% for IIFR, and 34% for the presence of parasites in the biopsy. ELISA was positive in 75% of patients with ACL presenting a negative MST, in 84.8% of ACL patients with negative skin or mucous biopsies for the presence of the parasite, and in 100% of cases with a negative PCR. Thus, ELISA presented a higher sensitivity than the other tests and was useful as a complementary method for the diagnosis of ACL.

Disseminated cutaneous cryptococcosis in a patient with AIDS

The authors study a patient carrying Aids, with exuberant dermatological manifestations of cryptococcosis. They stress the therapeutic effectiveness of short-term amphotericin B. The authors reviewed cases of cutaneous infection with Cryptococcus reported in the national and international literature, verifying that the frequency has increased with the AIDS epidemic. Also, they discuss about the differential diagnosis with some cases of dermatosis, particularly with the disseminated giant molluscum contagiosum. In relation to the therapy, they affirm that the choice of drug depends on the organ involved, as well as the immune state of the patient.