RESUMO
Recent studies have shown that the claim that King George III suffered from acute porphyria is seriously at fault. This article explores some of the causes of this misdiagnosis and the consequences of the misleading claims, also reporting on the nature of the king's recurrent mental illness according to computer diagnostics. In addition, techniques of cognitive archaeology are used to investigate the nature of the king's final decade of mental illness, which resulted in the appointment of the Prince of Wales as Prince Regent. The results of this analysis confirm that the king suffered from bipolar disorder type I, with a final decade of dementia, due, in part, to the neurotoxicity of his recurrent episodes of acute mania.
Assuntos
Transtorno Bipolar/diagnóstico , Transtorno Bipolar/história , Demência/diagnóstico , Demência/história , Pessoas Famosas , Porfirias/diagnóstico , Porfirias/história , Diagnóstico por Computador , Inglaterra , Escrita Manual , História do Século XVIII , História do Século XIX , Humanos , MasculinoAssuntos
Mordeduras Humanas/história , Sangue , Folclore , História da Medicina , Mitologia , Europa (Continente) , História do Século XVI , História do Século XVII , História do Século XVIII , História do Século XIX , História do Século XX , História Medieval , Humanos , Transtornos Mentais/história , Pelagra/história , Porfirias/história , Raiva/históriaRESUMO
The aim of the present study is to describe recent issues with Yusho disease in Japan, describe the state of dioxin accumulation and the intake of dioxin via food in Japan, and introduce the Japan Environment and Children's Study. Yusho disease manifested in western Japan in 1968. The causes of Yusho are believed to be dioxin-related compounds, mainly polychlorinated biphenyls (PCBs) and polychlorinated dibenzofurans (PCDFs), via the ingestion of rice oil produced in February 1968. As of March 31, 2011, there were 1961 registered Yusho cases, but of these 539 are deceased. A retrospective cohort study on registered Yusho cases reported that the standardized mortality ratios (SMRs) for the major causes of death were not significantly elevated, with the exception of all-cancer (SMR=1.26; 95% confidence interval [CI]: 1.03-1.53) and lung cancer mortality (SMR=1.56; 95% CI: 1.03-2.27) in males. The results of the Yusho mortality study show that the SMR for liver cancer in males tends to decrease over time. In 2011, the Ministry of the Environment of Japan reported that the average concentration of dioxins in the blood (2002-2010) of the Japanese people was 19 pg-TEQ/g-fat, demonstrating a range of 0.10-130 pg-TEQ/g-fat, and that the average dioxin intake from food (2002-2010) was 0.82 pg-TEQ/kg-body weight/day, demonstrating a range of 0.031-6.2 pg-TEQ/kg-body weight/day according to 2006 WHO TEFs. The Japan Environment and Children's Study Project was launched in 2011 and is supported by the Ministry of the Environment of Japan. In this project, 100,000 mother and child pairs will be recruited over 3 years from designated study areas. Follow-up examinations will be carried out from pregnancy until the children are 13 years of age (a so-called birth-cohort study). This project will be implemented by the National Center at the National Institute for Environmental Studies and is supported by the Medical Support Center at the National Center for Child Health and Development. Field operations will be performed at 15 designated regional centers nationwide.
Assuntos
Dioxinas/toxicidade , Porfirias/história , Animais , Dioxinas/farmacologia , Contaminação de Alimentos , História do Século XX , História do Século XXI , Humanos , Japão/epidemiologia , Neoplasias/induzido quimicamente , Neoplasias/epidemiologia , Porfirias/induzido quimicamente , Porfirias/mortalidadeRESUMO
The porphyrias are a group of disorders resulting from defective haem biosynthesis. One form, variegate porphyria, is common in South Africa as a result of a founder effect. Over the past 50 years, the University of Cape Town Faculty of Health Sciences has built and maintained an international reputation for excellence in the field of porphyria. The porphyria group is respected for its research and for its accumulated experience in the management of these disorders. Equally important has been the comprehensive and holistic care offered to patients with porphyria, and to their families.
Assuntos
Porfirias/história , Universidades , Flavoproteínas/genética , História do Século XX , História do Século XXI , Hospitais Universitários , Humanos , Proteínas Mitocondriais/genética , Porfiria Variegada/genética , Porfirias/diagnóstico , Porfirias/terapia , Protoporfirinogênio Oxidase/genética , África do SulRESUMO
The diagnosis that George III suffered from acute porphyria has gained widespread acceptance,but re-examination of the evidence suggests it is unlikely that he had porphyria.The porphyria diagnosis was advanced by Ida Macalpine and Richard Hunter, whose clinical symptomatology and historical methodology were flawed.They highlighted selected symptoms, while ignoring, dismissing or suppressing counter-evidence.Their claims about peripheral neuropathy, cataracts, vocal hoarseness and abdominal pains are re-evaluated; and it is also demonstrated that evidence of discoloured urine is exceedingly weak. Macalpine and Hunter believed that mental illnesses were primarily caused by physical diseases, and their diagnosis of George III formed part of a wider agenda to promote controversial views about past, contemporary and future methods in psychiatry.
Assuntos
Pessoas Famosas , Historiografia , Transtornos Mentais/história , Doenças do Sistema Nervoso Periférico/história , Porfirias/história , Inglaterra , História do Século XVIII , História do Século XIX , Humanos , MasculinoRESUMO
This research, based on a study of King George III's medical records and of contemporary diaries of his courtiers and equerries, further confirms the considerable doubt on the claim of Richard Hunter and Ida Macalpine that the King suffered from recurrent attacks of acute porphyria.The present study examines the above records from a psychiatric viewpoint, together with some additional reports, to re-assess the nature of the King's maladies. It concludes that he suffered from recurrent mania (four episodes), with chronic mania and possibly a degree of fatuity during the last decade of his life.This is in agreement with previous reports that he suffered from manic-depressive psychosis.
Assuntos
Transtorno Bipolar/história , Pessoas Famosas , Historiografia , Transtornos Mentais/história , Porfirias/história , Inglaterra , História do Século XVIII , História do Século XIX , Humanos , MasculinoAssuntos
Bioquímica/história , História do Século XX , Humanos , Minnesota , Fisiologia/história , Porfirias/história , Estados UnidosRESUMO
The first case of porphyria on record in Japan was a patient with congenital erythropoietic porphyria (CEP) reported by Sato and Takahashi in 1920. Since then until the end of December 2002, 827 cases of porphyrias have been diagnosed from characteristic clinical and/or laboratory findings (463 males, 358 females, and 6 of unknown sex). Essentially all inherited porphyrias have been found in Japan, with the incidences and clinical symptoms generally being similar to those reported for other countries. The male-female ratio was approximately 1:1 for CEP, whereas it was higher for erythropoietic protoporphyria. In contrast, preponderances of female patients exist with acute hepatic porphyrias, such as acute intermittent porphyria (AIP), variegate porphyria (VP), and hereditary coproporphyria (HCP), and with undefined acute porphyria. Although porphyria cutanea tarda (PCT) is believed to be increasing recently in women in other countries because of smoking and the use of contraceptives, it is still by far more prominent in males in Japan than in females. The recent increasing contribution of hepatitis C virus infection to PCT in Japan has also been recognized. but there have been no PCT cases in Japan with HFE gene mutations. Familial occurrence and consanguinity were high for CEP, as expected; however, significant consanguinity was also noted in families where CEP, AIP, HCP, VP, or PCT occurred as a single isolated case without a family history of disease. This survey also revealed that as many as 71% of acute hepatic porphyria cases were initially diagnosed as nonporphyria and later revised or corrected to porphyria, indicating the difficulty of diagnosing porphyria in the absence of specific laboratory testing for porphyrins and their precursors in urine, stool, plasma, and erythrocyte samples.
Assuntos
Porfirias/patologia , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Consanguinidade , Análise Mutacional de DNA , Coleta de Dados , Feminino , História do Século XX , Humanos , Incidência , Lactente , Recém-Nascido , Japão , Masculino , Pessoa de Meia-Idade , Linhagem , Porfirias/genética , Porfirias/história , Porfirias Hepáticas , Fatores SexuaisRESUMO
Although the biosynthetic pathway to heme has been well elucidated and errors along that route have been identified and firmly connected to specific diseases, the porphyrias, slight but nonspecific abnormalities, are occasionally invoked as proof of porphyria or in support of other diagnoses. An errant patient with a conundrum of symptoms but without an explanation for them might have to take iatrogenic detours only to learn after what are at times ulyssean vagaries that the initial diagnosis of porphyria is in the end untenable. Thus the porphyrias are superb examples of the interface between laboratory and clinical medicine, in which the occurrence of the Ulysses syndrome can be curtailed through the careful ordering of tests and cogent interpretation of their results.
Assuntos
Medicina Clínica/métodos , Porfirias/diagnóstico , Pessoas Famosas , História Antiga , Humanos , Masculino , Porfirias/históriaRESUMO
Porphyrias are a group of inherited disorders of heme biosynthesis classified as neurovisceral, cutaneous, or mixed. A deficiency of any of the 8 enzymes in the biosynthetic pathway can lead to a variety of clinical symptoms. Classification depends on the defective enzyme. Porphyrias often are misdiagnosed because patients have vague symptoms. However, acute forms of porphyria can be life-threatening, so it is important to make an accurate diagnosis and initiate proper medical management. We discuss the history, pathogenesis, clinical manifestations, diagnosis, and treatment of porphyrias and then briefly describe the 8 types of porphyrias and their distinguishing features.
Assuntos
Porfirias/história , Diagnóstico Diferencial , História do Século XVIII , História do Século XIX , História do Século XX , Humanos , Porfirias/etiologia , Porfirias/patologia , Porfirias/terapiaRESUMO
We present the main diagnosis concerning Rousseau's, with an emphasis on his urologic disorders.
Assuntos
Pessoas Famosas , Filosofia/história , Porfirias/história , Doenças Urológicas/história , Doença Aguda , França , História do Século XVIII , Humanos , Masculino , Porfirias/complicações , Doenças Urológicas/etiologiaRESUMO
Historical evidence has been collected attempting to diagnose members of royal houses, perhaps most publicized by Macalpine and Hunter (1969) for George III and his assumed porphyria, claiming that his insanity was a classic case of thereof. This rare metabolic disease presents with a variety of signs and symptoms: skin disease, abdominal pain, tachycardia, and neuro-psychiatric findings. The porphyrias are hereditary and since George III and Frederick William I share ancestors it seemed reasonable to investigate if the latter may also have suffered from porphyria. The pathography of both kings is meticulous, showing for both that abdominal pain, erratic behavior, restlessness, and discolored urine were frequently observed and complete recovery interictally was common. Intercurrent illnesses, fasting, alcohol and even tobacco smoking have been shown to be inducers of attacks and these risk factors are well documented in royal history.--The diagnosis of porphyria was not recognized then and other names were used, such as Cachexia hypochondriaca, Asthma spasmodico flatulentum, dolores arthritici.--We propose that Frederick William I suffered from an inducible porphyria.
Assuntos
Pessoas Famosas , Porfirias/história , Alemanha , História do Século XVII , História do Século XVIII , Humanos , MasculinoRESUMO
Black urine is recorded in all ancient urology as a negative prognostic sign, often linked with the presence of blood; its presence can also be considered as a sign of massive hemolytic crisis, especially if associated with specific nosological patterns. The Hippocratic case of Epidemics III, 11 has recently been diagnosed as an intermittent acute porphyria. Despite the difficult 'retrospective' diagnosis of an ancient case, it seems likely that the Hippocratic physicians empirically knew clinical associations of symptoms that modern medicine could consider as the first descriptions of porphyria.