[The Chilean medical scientific societies join to form a common entity]. / Las Sociedades Médicas Científicas de Chile se agrupan en una entidad común.

A recent law project pretended to improve the number and distribution of clinical specialists throughout the country. Several Medical Scientific Societies felt that the diagnosis and the treatment proposed for the problem were erroneous. In Chile, there is no such lack of specialists but, unfortunately, they are badly distributed and any solution should include their better geographical distribution. This was the beginning of a common action by 30 Medical Scientific Societies, which realized that they have several problems in common, deciding to create an entity to face them: the Chilean Association of Medical Scientific Societies (ASOCIMED). This association was formed last August. In a few months of work it has studied its bylaws to be submitted for approval to the Member Societies. Also it has started to study, together with the Ministry of Public Health, the needs, number and distribution of specialists in the country and important information should be gathered from this study. ASOCIMED is already representing Medical Scientific Societies in the National Corporation for Medical Certification (CONACEM), together with the National Academy of Medicine is creating a National Award of Medicine and with the Health Insurance Companies is studying a way to face high cost diseases. During its short existence, ASOCIMED has faced a diversity of different issues and it seems reasonable to believe that it will play an important role in Chilean Medicine.

[Porphyric crisis: experience of 30 episodes]. / Crisis porfírica. Experiencia con 30 episodios.

The experience of 30 porphyric crisis is reviewed in 25 patients attended since 1967: 21 patients had 1 crisis, 3 had 2, and 1 had 3 of these episodes. In all patients, porphyria was diagnosed in relation to one crisis, even though many of them had family histories and/or previous clinical symptoms of this disease. There was clear predominance (80%) of women, but they are also a majority among acute porphyrias. The most frequent symptoms were: abdominal pain, tachycardia, dark urine, neurological and psychiatric alterations and arterial hypertension. The neurological alterations required the use of a respirator in 9 crisis (33%), which was maintained in 2 patients during 4 months. In 6 crisis (20%) there were no neurological symptoms. Among laboratory tests, hyponatremia was notable for its frequency (53.4%) and intensity. Increase in urinary porphobilinogen, a requirement for diagnosis, between 15 and 130 times the normal value was observed. Septic complications, such as pneumonia, septicemia, and urinary infection, were frequent (50%). Factors suspicious of triggering crisis episodes were: drugs, usually more than 2, in 50% of the cases; pregnancy in 30% of the women and in a lesser proportion, intense exercise, and surgery. In 10 patients, crisis triggering factors were not identified or informed. The role of pregnancy, childbirth delivery or puerperium in causing a crisis is not clear, because the patients who had a crisis related to them had 15 other pregnancies without incidents; besides, in the pregnancy which was accompanied by a crisis, there was always one or more than one potentially triggering drug present. The first therapeutic step was oral and/or parenteral administration of an overload of carbohydrates and, if there was no response, intravenous infusion of hematin was prescribed. Four (13.3%) patients died even though they had received hematin, but it had been administered too late due to a delay in diagnosis. In surviving patients, there were no organic sequels of any kind.

[Hepatitis C virus and resulting diseases]. / El virus C de la hepatitis y las enfermedades que produce.

In 1989, the main agent causing non A non B hepatitis was identified as a RNA virus of the flavivirus family, with several serotypes, and was denominated virus C. At the present moment, the knowledge about the infection features and diseases that it causes has expanded thanks to the availability of reliable laboratory techniques to detect the antibody and the virus. The prevalence of infection and the frequency of serotypes varies in different regions of the world. Chile is a country with a low prevalence. The detection of infected blood in blood banks has reduced the spreading of the disease. Other means of infection such as the use of intravenous drugs, hemodialysis and transplantation have acquired greater importance. Sexual, maternal and familial transmission is exceptional. Infected people develop an acute hepatitis, generally asymptomatic. Eighty percent remain with a chronic hepatic disease, that can be mild or progressive, evolving to cirrhosis or hepatic carcinoma. Chronic hepatitis, closely resembling an autoimmune disease, can be caused by the virus. Alcohol intake increases viral activity causing severe hepatic diseases, refractory to treatments. Several non hepatic diseases are associated to hepatitis C virus infection such as essential mixed cryoglobulinemia, mesangiocapillary glomerulonephritis, porphyria cutanea tarda, dysglobulinemias and probably type 2 diabetes mellitus. The only available treatment is interferon, that is successful in a minority of patients, frequently causing a transient improvement. The use of Ribaravine associated to interferon improve the effectiveness of therapy. Liver transplantation is the only therapy for severe hepatic disease. The use of new antiviral drugs should improve the prognosis of the disease.

Crisis porfírica. Experiencia con 30 episodios. / [Porphyric crisis: experience of 30 episodes]

The experience of 30 porphyric crisis is reviewed in 25 patients attended since 1967: 21 patients had 1 crisis, 3 had 2, and 1 had 3 of these episodes. In all patients, porphyria was diagnosed in relation to one crisis, even though many of them had family histories and/or previous clinical symptoms of this disease. There was clear predominance (80

) of women, but they are also a majority among acute porphyrias. The most frequent symptoms were: abdominal pain, tachycardia, dark urine, neurological and psychiatric alterations and arterial hypertension. The neurological alterations required the use of a respirator in 9 crisis (33

), which was maintained in 2 patients during 4 months. In 6 crisis (20

) there were no neurological symptoms. Among laboratory tests, hyponatremia was notable for its frequency (53.4

) and intensity. Increase in urinary porphobilinogen, a requirement for diagnosis, between 15 and 130 times the normal value was observed. Septic complications, such as pneumonia, septicemia, and urinary infection, were frequent (50

). Factors suspicious of triggering crisis episodes were: drugs, usually more than 2, in 50

of the cases; pregnancy in 30

of the women and in a lesser proportion, intense exercise, and surgery. In 10 patients, crisis triggering factors were not identified or informed. The role of pregnancy, childbirth delivery or puerperium in causing a crisis is not clear, because the patients who had a crisis related to them had 15 other pregnancies without incidents; besides, in the pregnancy which was accompanied by a crisis, there was always one or more than one potentially triggering drug present. The first therapeutic step was oral and/or parenteral administration of an overload of carbohydrates and, if there was no response, intravenous infusion of hematin was prescribed. Four (13.3

) patients died even though they had received hematin, but it had been administered too late due to a delay in diagnosis. In surviving patients, there were no organic sequels of any kind.

Genotipo del virus C de la hepatitis en pacientes con porfiria cutanea tarda / Genotype of the hepatitis virus C in patients with porphyria cutanea tarda

En pacientes con porfiria cutánea tarda (PCT) se ha demostrado alta prevalencia del anticuerpo del virus C de la hepatitis (anti-VCH) y del propio virus. Previamente hemos informado una prevalencia de anti-VCH de 53 por ciento, y de 80 por ciento entre aquellos pacientes con PCT esporádica. La PCT se asocia frecuentemente a daño hepático crónico,desconociéndose su origen. Se ha postulado que el VCH, directa o imdirectamente, modificaría la actividad de la uroporfirinógeno decarboxilasa(URO-D) hepática, alteración carterística de la PCT, y por lo tanto la enfermedad sería adquirida y no genética. Podría esperarse que la frecuencia e intensidad de la hepatopatías en los pacientes con PCT fuera mayor en aquellos pacientes portadores del Anti-VCH(AU)

Genotipo del virus C de la hepatitis en pacientes con porfiria cutanea tarda / Genotype of the hepatitis virus C in patients with porphyria cutanea tarda

En pacientes con porfiria cutánea tarda (PCT) se ha demostrado alta prevalencia del anticuerpo del virus C de la hepatitis (anti-VCH) y del propio virus. Previamente hemos informado una prevalencia de anti-VCH de 53 por ciento, y de 80 por ciento entre aquellos pacientes con PCT esporádica. La PCT se asocia frecuentemente a daño hepático crónico,desconociéndose su origen. Se ha postulado que el VCH, directa o imdirectamente, modificaría la actividad de la uroporfirinógeno decarboxilasa(URO-D) hepática, alteración carterística de la PCT, y por lo tanto la enfermedad sería adquirida y no genética. Podría esperarse que la frecuencia e intensidad de la hepatopatías en los pacientes con PCT fuera mayor en aquellos pacientes portadores del Anti-VCH

[Porphyrin excretion in patients with chronic hepatitis C virus infection]. / Excreción de porfirinas e infección crónica por virus C de la hepatitis.

BACKGROUND: The high prevalence of chronic hepatitis C virus infection in patients with porphyria cutanea tarda, specially in those without family history of the disease, suggests that this could be an acquired disease and one of the most frequent extra hepatic manifestations of hepatitis C virus infection. AIM: To study the excretion of porphyrins and its precursors in cirrhotic patients with and without hepatitis C virus infection. PATIENTS AND METHODS: Eighteen patients with cirrhosis Child-Pough A, eight infected with hepatitis C virus, were studied. Urinary excretion of [symbol see text] aminolevulinic acid, porphobilinogen, coproporphyrins, uroporphyrins and fecal excretion of coproporphyrins and protoporphyrins were measured. Red blood cell protoporphyrin was also measured. RESULTS: There were no differences in the measured parameters between patients with or without hepatitis C virus infection. No patient had uroporphyrin excretion values over the normal range. Some patients had slight elevations in some parameters, but always below the values observed in porphyrias. CONCLUSIONS: In these group of patients, hepatitis C virus infection of its associated liver disease, do not cause detectable alterations in porphyrin metabolism.

[Drugs and porphyric crisis]. / Fármacos y crisis porfírica.

Life threatening crisis may accompany some varieties of porphyria like the acute intermittent form, coproporphyria, porphyria variegata and that associated to deficiency of porphobilinogen synthetase. Drugs are commonly involved as precipitating factors. A classification of drugs according to their proven or probable triggering effect is offered in this paper. Insufficient information precludes the classification of some drugs.

Hepato-splenic distribution of 99Tcm-phytate and hepato-enteric distribution of 99Tcm-DISIDA in mice with carbon tetrachloride-induced acute liver damage.

The hepato-enteric distribution of 99Tcm-labelled DISIDA and the hepato-splenic distribution of 99Tcm-labelled phytate were studied in controls and in mice with carbon tetrachloride-induced acute liver damage. The test group animals showed a diminished excretion of DISIDA to intestine with retention of this tracer in the liver and an increased splenic uptake of phytate. No changes in the hepatic uptake of phytate were found. The joint evaluation of both tracers in the study of diffuse liver disease is discussed.

Deficiency of porphobilinogen synthase associated with acute crisis. Diagnosis of the first two cases in Chile by laboratory methods.

Erythrocyte porphobilinogen synthase deficiency was confirmed by the determination of its activity in blood and also by the high levels of both porphyrins and 5-aminolaevulinic acid in the urine of two siblings. They presented with a picture of porphyric attack characterized by abdominal colic pain, high blood pressure, tachycardia and severe constipation. The profile of both porphyrins and their precursors in urine and blood resembled lead poisoning. However, this was ruled out because both patients had normal blood levels of lead. Furthermore, porphobilinogen synthase activity did not normalize when it was determined in the presence of dithiothreitol or dithiothreitol plus zinc chloride. No other causes to account for a deficiency in porphobilinogen synthase activity were identified. The simultaneous occurrence of similar clinical and biochemical symptoms suggests that the same triggering factor was present. Because the activity of porphobilinogen synthase was less than 4% of normal values, it is possible that these patients were homozygotes with respect to this defect, which could explain the presence of clinical symptoms. We propose that this metabolic defect is not uncommon and it should be kept in mind when diagnosing of porphyrias or heavy metal intoxications.

Tru-cut and Menghini needles: different yield in the histological diagnosis of liver disease.

An evaluation of the information obtained by percutaneous liver biopsy performed with Travenol Tru-cut ("Tru-cut"), Menghini's 1.9 and 1.6 needles, was done using cadavers. All needles used had a similar, low number of "failures". Recognition of diffuse liver lesions, excluding fibrosis, was very high regardless of the needle used. Identification of cirrhosis was significantly more frequent in biopsies performed with the Tru-cut needle.