PORPHYRIAS (porphyria, singular; grech, porphyra purple paint) — group of diseases, hereditary or with hereditary predisposition, the porphyrines resulting from disturbance of porphyrinic exchange and followed by increase in contents in an organism or their predecessors. It is not necessary to carry acquired diseases and intoxications to P., at to-rykh the purpurinuria (the increased content of porphyrines in urine) or a porfirinemiya is observed (the increased content of porphyrines in erythrocytes).
The main quantity is normal porphyrines (see) it is synthesized in marrow and a liver. Items divide on erythropoietic and hepatic. Treat erythropoietic P. an erythropoietic uroporfiriya, erythropoietic protoporphyry iya p an erythropoietic koproporfiriya; to hepatic P. — the acute alternating porphyria, a hereditary hepatic koproporfiriya, to - a pro-protoporphyria, or the mixed (variyegatny) porphyria, and an urokopro-porphyria (a late skin porphyria).
The first case of P. is described in 1874 by Schultz: at the patient since the early childhood hypersensitivity to solar radiation, increase in the sizes of a spleen, urine of red color was revealed. In several years diseases with signs of a polyneuritis, abdominal pains, mental disorders were described. Such patients also had urine of red or pink color. Sometimes the exacerbation of a disease came after reception of hypnotic drugs, however in nek-ry cases symptoms of a disease appeared without reception of any pharmaceuticals. E. Goppe-Zeyler called the pigment found in urine of patients, urohematoporphirin, assuming that at urine there is a haematoporphyrin — substance, a cut it turns out artificially during the processing of blood sulfuric to - that. Therefore the disease was called a gekhmato-porphyria or gematoporfir an in-uriya in the beginning. Further it was established that the substances emitted with urine, a stake at different types of P. belong not to haematoporphyrin, and to an uroporfirin, coproporphyrin, protoporphyrin and their predecessors therefore the terms «haematoporphyrin» and «hematoporphyrinuria» are not used.
The significant contribution to P.'s studying was made by Günter (N. of Gunther, 1911, 1922) who described inborn P. Günter's porphyria, or an erythropoietic uroporfiriya). For the first time established by Valden-str (J. G. Waldenstrom, 1937) that with urine at patients with nek-ry forms P., napr, the acute alternating P., is allocated to hl. obr. the predecessor of porphyrines — porphobilinogen which in trace amount is found in urine of healthy faces. It also showed that the acute alternating porphyria is inherited on dominant type, allocated latent forms of a disease. Valden-str in detail described the most common form of a porphyria which is shown at adult persons a photosensitization, easy vulnerability of skin, increase in the sizes of a liver and disturbance of its functional state. He called it a late skin porphyria. Byrd-zher and Goldberg (N. of Berger, A. Goldberg, 1955) reported about the unknown to a form earlier — the hereditary hepatic koproporfiriya having much in common with the acute alternating porphyria. J. A. Magnus with soavt, in 1961 described an erythropoietic protoporphyria, at a cut the content of porphyrines in urine does not exceed norm, however increase in content of protoporphyrin in erythrocytes and Calais is found.
The used earlier generalizing term «porphyrinic disease» is not applied since P. represent group of diseases with various pathogeny, a wedge, a picture and the forecast.
Erythropoietic uroporfiriya (synonym: the inborn porphyria, Günter's porphyria, Günter's disease) — the disease which is characterized by damage of skin, hemolitic anemia with an intracellular hemolysis, adjournment in erythrocytes and allocation with urine of an uroporfirin of I (isomer I of an uroporfirin) meets seldom, proceeds hard.
The erythropoietic uroporfiriya is inherited on autosomal recessively type. The disease is observed in one generation, sometimes at several children. At parents, heterozygous carriers patol, a gene, a wedge, displays of a disease are absent though in some cases the increased content of porphyrines in urine is found.
In a pathogeny of a disease, most likely, matter a superactivity of enzyme of synthase 6-aminolevulinic to - you, edges a little reduced activity of enzyme of a kosintaza of uroporphyrinogen III can be connected with disturbance of repression of synthesis of enzyme, and. As a result uroporphyrinogen I which cannot be used in an organism for further transformations is formed of excess amount of porphobilinogen. The amount of the formed uroporphyrinogen III is enough for synthesis gem (see. Gemoglobin ); therefore patients have no hypochromia anemia with the high content of iron. Excess adjournment of an uroporfirin in erythrocytes leads to shortening of duration of their life, i.e. to raised to hemolysis (see). At the same time from erythrocytes (see) a large amount of uroporphyrinogen which is oxidized in uroporfirin and is laid in skin that causes is released photosensitization (see).
The disease can be shown at newborns. At the same time find urine of red color, hypersensitivity of skin to solar radiation. In several weeks or months after the birth on various body parts of the child there are bubbles to dia, from 1 to 10 mm which quite often ulcerate, at the same time ulcers badly heal, consecutive infection joins. Further ulcers cicatrize, sites of the sclerosed skin which lead to formation of atrophic hems are formed and can cause a contracture of joints, and also a blindness (at damage of eyes). Quite often the child has no hair and nails, sklerodermopodobny changes on a face develop.
Darkening of teeth is characteristic, note a reddish luminescence them in UV rays. Almost at all patients at a palpation of a stomach reveal increase in the sizes of a spleen. There are typical signs hemolitic anemia (see), proceeding with an intracellular hemolysis (level of an indirect bilirubin, the maintenance of reticulocytes increases, the irritation of a red sprout of marrow in the absence of increase in content of free hemoglobin in a blood plasma is found and at absence in urine of hemosiderin, osmotic resistance of erythrocytes is lowered, shortening of life expectancy of erythrocytes is noted). It is in some cases observed thrombocytopenia (see).
At biochemical, a research find a large number of an uroporfirin of I and to a lesser extent coproporphyrin I in urine; a large number of an uroporfirin of I in erythrocytes. In UV rays erythrocytes shine red light.
A certain effect renders splenectomy (see), leads edges to lengthening of life expectancy of erythrocytes. Besides, from the destroyed erythrocytes the smaller quantity of an uroporfirin is released that leads to reduction of a photosensitization. Forecast of a disease serious.
Erythropoietic protoporphyria — the disease which is characterized by hypersensitivity to solar radiation, emergence of a cutaneous dropsy soon after radiation, as a rule, without formation of ulcers and hems, the high content of protoporphyrin in erythrocytes and Calais in the absence of it in urine. Meets quite often, in most cases proceeds it is good-quality, sometimes — it is heavy.
The disease is inherited on autosomal dominantly type. In a pathogeny disturbance of synthesis gem from protoporphyrin, most likely, regarding eritrokario-tsit matters, and also, apparently, increase in synthesis 6-aminolevulino-howl to - you. Adjournment of protoporphyrin in skin leads to disturbances, however toxic effect of protoporphyrin on skin is expressed to a lesser extent, than uroporfiri-on I.
Wedge, manifestations: on open parts of a body in a few minutes after stay in the sun hypostasis, an itch, erubescence develops; quite often later a nek-swarm time raises temperature. At long stay in the sun hemorrhagic rashes are observed. Occasionally on places of burns bubbles which ulcerate are formed and leave further small hems. Burn at such patients can be got through window glass, synthetic linen since the light radiation (380 nanometers) freely passes through them.
From complications it is noted hypochromia anekhlshya with the high content of iron in blood serum (see. Zhelezorefrakternaya anemia ), tendency to formation of concrements in a gall bladder, seldom or never — adjournment of a huge amount of porphyrines in a liver with development liver failure (see).
From biochemical, signs increase in content of protoporphyrin IX in erythrocytes by 20 — 100 times is characteristic (norm to 50 mkg! 100 ml) at the normal maintenance of an uroporfirin and coproporphyrin. Significant increase in amount of protoporphyrin and coproporphyrin in Calais is found in nek-ry patients. Differential diagnosis is carried out with an iron deficiency anemia (see), hemolitic anemia (see), with lead intoxication, at to-rykh slight increase of protoporphyrin IX in erythrocytes is observed (to 100 — 150 mkg 100 ml).
Protection against solar radiation (wearing hats, gloves) and use of sunblock creams («Beam», «Board»),
the Forecast favorable is recommended.
Erythropoietic koproporfiriya meets exclusively seldom, it is described by Geylmeyer (L. Heil-meyer, 1964) and L. I. Idelso-n (1968).
The disease is probably inherited on autosomal dominantly type. The pathogeny is unknown. A wedge, the same manifestations, as at an erythropoietic protoporphyria. Content of protoporphyrin in erythrocytes increases by 30 — 80 times in comparison with norm. Content of porphyrines in urine increases slightly, generally at the expense of coproporphyrin. Treatment is not developed. Forecast favorable.
The acute alternating porphyria it is characterized by abdominal pains, increase in the ABP, release of urine of pink color in connection with a large number of the predecessor of porphyrines in it — porphobilinogen, especially in the period of an aggravation, defeat of a nervous system.
The disease is inherited on autosomal dominantly type. Disturbance of activity of enzyme of synthase of an uroiorfirinogen of I and increase in activity of enzyme of synthase 8-aminolevulinic to - you is the cornerstone of a pathogeny, most likely. A wedge, manifestations are connected, apparently, with accumulation in nervous cells of toxicant — 6-aminolevulinic to - you since it is shown that 6-aminolevulinic to - that concentrates in a hypothalamus and brake activity sodium - kaliyzavisimy adenosin phosphate-zy that leads to disturbance of transport of ions through membranes and breaks function of nerve fibril. Its demyelination develops (see. Nerve fibrils ), the axonal neuropathy, as causes a wedge, displays of a disease.
The most characteristic sign of the acute alternating porphyria — abdominal pains which can be localized in epigastric area, reminding a picture of perforated stomach ulcer, or pain in the right ileal area that it simulates a picture of an acute appendicitis. Pains can be localized in right hypochondrium, reminding pains at cholelithiasis, or in lumbar area, simulating renal colic and sometimes an extrauterine pregnancy since severe pains are quite often preceded by a delay of periods.
Increase in the ABP is found in a considerable part of patients, the expressed arterial hypertension with increase in systolic and diastolic arterial pressure, and also locks sometimes comes to light.
Defeat of a nervous system is shown by also porfiriyny polyneuropathy. Patol, changes happen in many sites of an axon (see. Neuropathy ). Its degeneration can begin in terminal departments and extend proksimalno along an axon to nervous cells. The basic nevrol, a symptom complex at the acute alternating P. is the axonal polyneuropathy of a time-firiynaya. In the beginning the disease is characterized by weakness in muscles of legs, nagging pains in them, paresthesias. Paresis of muscles of the lower extremities quickly accrues, weakness in muscles of hands joins. Deep symmetric paresis and paralyzes are more expressed in distal departments (see. Paralyses, paresis ). Function of muscles of a back suffers to a lesser extent, than muscles of a stomach which sharply weaken that leads to disturbance of a tussive reflex. Tendon jerks are absent. Intolerable hand and legs pains, symptoms of a tension, morbidity of all nervous trunks and disorder of sensitivity on polyneuritic type with loss of muscular and joint feeling are constantly observed. If the porfiriyny polyneuropathy is limited only to paresis of muscles of a trunk and extremities, then its current is good-quality. Gradually there is a recovery of muscular functions though in distal departments, especially feet, recovery continues within many months and even years. At extremely heavy current of a polyneuropathy at P., especially at the induced forms caused by reception of barbiturates, alcohol, high doses of analgetics, insolation, and sometimes and an operative measure dysfunction craniocereberal joins the developed tetraparesis (cranial, T.) nerves. It is observed anisocoria (see), non-constant ptosis (see), paresis of oculomotor muscles (see. Third cranial nerve ), a periodic blindness, a promination and a stushevannost of disks of optic nerves (see. Optic nerve ). There is paresis masseters (see), bulbar frustration with signs of an aphagia (see. Dysphagies ), aphonias (see), anarthrias (see. Dysarthtia ). Paresis of intercostal muscles and (in more expressed degree) diaphragms joins these symptoms. Paresis of muscles of extremities and a trunk at an extreme current of a porfiriyny polyneuropathy are combined with bulborespi-ratorny disturbances. Breath becomes frequent to 40 — 50 in 1 min. Vital capacity of lungs (see) catastrophically decreases. Death in the absence of treatment comes from an apnoea and cordial activity. Sharp tachycardia with pulse rate 140 — 180 in 1 min. is always noted. In this phase of a disease sharp excitement, sleeplessness, visual and auditory hallucinations, spotty memory defects are characteristic. Mental disturbances after development of respiratory insufficiency (see) are caused by an arterial anoxemia (see. Hypoxia ). Structure cerebrospinal liquid (see) without aberrations, at long existence of a polyneuropathy proteinaceous and cellular dissociation is possible.
Most often the disease is shown at girls and young women; it is provoked by pregnancy, childbirth, reception of a number of pharmaceuticals (barbiturates, sulfanamide drugs, analginum). The course of a disease happens various. Especially strong aggravations come after operations when for premedication use thio-pental-sodium (see), at the same time if before operation the patient complained only of severe pains in a stomach, then after it appear heavy nevrol. frustration. The aggravation of this form P. can spontaneously come or under the influence of female sex hormones, medicines, napr, griseofulvin, valocordin, a bellaspon, Belloidum, Theophedrinum, and also nek-ry chemical substances (especially a hlorofosa). At development heavy nevrol, frustration there can come death, however sometimes they gradually disappear, there occurs remission therefore the disease was called the acute alternating P.
Klinicheski the disease is shown not at all carriers patol, a gene. Quite often at relatives of patients, especially at men, symptoms of a disease in the absence of clinical come to light biochemical (a latent form of the acute alternating porphyria). At influence of adverse factors at them the severe form of a disease can develop.
The diagnosis is established on the basis by a wedge, pictures and a lab. data: detection in urine of predecessors of synthesis of porphyrines — porphobilinogen and 6-aminolevulinic to - you which are defined by simple qualitative test. In a test tube with 1 — 2 ml of fresh urine add as much Ehrlich's reactant (2% solution of the paradimethylaminobenzaldehyde dissolved in 4 N solution salt to - you); in the presence of porphobilinogen cerise coloring appears, a cut it can be caused also by a large number in urine of urobilin. For specification add chloroform, after upholding it appears below, and a water layer from above. If coloring is connected with urobilin, it passes into a sublayer, at P. it remains in a top water coat.
Differential diagnosis is carried out with a hereditary hepatic koproporfiriya and a koproproto-porphyria (see below), for to-rykh substantial increase of content in urine 6-aminolevuli-new to - you and porphobilinogen in the period of an exacerbation of a disease and normalization of contents them is characteristic during remission, in some cases signs of a photosensitization. The acute alternating P. is differentiated also with a plumbism (see Lead), a cut differs in hypochromia anemia with existence of basphilic stippling of erythrocytes and with the high content of iron in blood serum; with an infectious and allergic polyradiculoneuritis — a syndrome of Giyena — of Barret — Shtrolya (see. Polyneuritis ), miyelopoliradikulonevrity (see), recurrent polyneurites and various secondary polyneuropathies.
At severe pains apply morphine, aminazine; at sharp tachycardia, at substantial increase of the ABP — inderal or Obsidanum; at strong locks — prozerin.
The diet with the high content of carbohydrates is recommended (within a day not less than 400 g of glucose, for the best portability together with juice; if swallowing is broken, it is entered via the nazogastralny probe). At the same time intravenously enter high doses of glucose (10% solution of glucose to 1 l within a day), edges reduces production of porphyrines. Adenosine monophosphate and inosine (inosine) possess the same action. Adenozin-monofosfat enter intramusculary 50 — 60 mg in the form of drugs of adenil or a fos-faden, inosine is appointed inside on 200 mg by 3 — 4 times a day. At improvement of a state the dose of injection drugs is reduced at the expense of peroral. For temporary switching off of a menstrual cycle if it leads to an exacerbation of a disease, it is reasonable to use androgens. At emergence of respiratory insufficiency and bulborespiratorny frustration apply measures of resuscitation; make tracheostomy (see), artificial ventilation of the lungs (see. Artificial respiration ), a toilet of a tracheobronchial tree, feeding via the nazogastralny probe, massage of a thorax for recovery of the mechanism of expectoration (see. Massage ). For recovery of movements do the general massage and remedial gymnastics.
The forecast at severe defeat of a nervous system serious. If the polinevropagiya is limited only to paresis of muscles of extremities and trunks, there can occur spontaneous remission. Gradually there is a recovery of movements, but approximately at a half of patients residual paresis in distal departments of hands and legs is noted. After accession of bulborespirator-ny frustration the forecast sharply worsens. So, according to Markovic (M. of Markovitz, 1954), the lethality reaches 90%.
Prevention of aggravations — an exception of the means causing them; use of adenil during pregnancy. It is necessary to investigate blood and urine of relatives of patients for identification biochemical, signs of the Item. Prohibit patients with a latent form P. reception of the pharmaceuticals capable to cause an aggravation of the Item.
Hereditary hepatic koproporfiriya — rather rare form P.
The disease is inherited on autosomal dominantly type; often proceeds latentno. Disturbance of activity of enzyme of a kopro-porfirinogen-oxidase is characteristic; in a liver at the same time increase in activity of synthesis 6-aminole-vulinovoy to - you is found out what, most likely, defeat of a nervous system is connected with. On a wedge, reminds manifestations the acute alternating porphyria. The most frequent symptom of a disease — abdominal pains, sometimes come to light mental disorders, paresis which are more rare and not so expressed, as at the acute alternating porphyria. The ABP can raise, appear tachycardia. A part of patients has signs of a violent photodermatitis (see Photodermatoses). According to Brody and Goldberg (M. J. Vgo-die, A. Goldberg, 1980), at patients with a hereditary koproporfiriya in 80% of cases observes abdominal pains, at one third of patients hypersensitivity to the sun comes to light and paresis, mental disorders, vomiting, a lock, at 20% — small fervescence, at 15% — epileptic seizures, a hyperbilirubinemia, arterial hypertension, tachycardia, at 10% — a diarrhea develop.
In the period of an aggravation in urine the increased quantity - aminolevulinic to - you is found and porphobilinogen, a cut is lower, than at the acute alternating porphyria. In urine and Calais the amount of coproporphyrin is sharply increased. During remission the content of porphobilinogen and 6-aminolevuli-new to - you can be normal at the increased content of coproporphyrin in urine and Calais.
Treatment of aggravations is similar to treatment at the acute alternating porphyria.
Koproprotoporfiriya (variyegatny porphyria) occurs most often at the persons of white race burning in South Africa, descendants of immigrants from Holland in 17 century P. Mustajoki in 1976 found patients with this form P. in Finland.
The disease is inherited on autosomal dominantly type. Disturbance of activity of enzyme protoporphyry of an inogen-oxidase, in charge of synthesis of protoporphyrin from a protoporfirinogen is the cornerstone of a pathogeny, apparently. At the same time increase in synthesis 6-amino-levulinic to - you due to increase in activity of a sintetaza 6-aminolevu-linovoy to - you takes place.
The disease is characterized same nevrol, symptomatology, as at the acute alternating P., and also skin symptomatology. Skin manifestations meet at men, nevrol, disturbances more often — at women. Abdominal pains, nevrol, frustration arise as well as at the acute alternating porphyria (under the influence of barbiturates, sulfanamide drugs, analginum, griseofulvin). Exacerbations of a disease remind manifestations acute alternating the Item. Unlike the acute alternating form at a koproprotoporfi-riya sometimes develops renal failure (see).
Skin manifestations same as at an urokoproporfiriya, were observed at 80% of the persons living in South Africa, and according to Mustayoki, they occur at the persons living in Europe much less often and are expressed much less.
Constant significant increase in amount of protoporphyrin in Calais is characteristic of a koproprotoporfiriya. Contents 6-aminolevuli-new to - you and porphobilinogen in urine increases in the period of an aggravation and can go down, sometimes to datum level, after reduction of abdominal pains, recession nevrol, symptoms.
Treatment, prevention and the forecast same, as at a hereditary hepatic koproporfiriya.
Urokoproporfiriya (a synonym a late skin porphyria) — the most often found form P. in the USSR.
The disease is often observed at the persons who are abusing alcoholic beverages, had hepatitis, dealing with hepatotoxic poisons, and also with gasoline (e.g., at drivers). Urokoproporfiriya sometimes develops at a tumor of a liver. At most of persons with a porphyria disturbance of a functional condition of a liver is observed. These facts allowed to assume that an urokoproporfiriya — not the hereditary, and acquired form P. However only an insignificant part of the persons abusing alcoholic beverages gets sick with P.; at heavy hepatitises in urine the content of coproporphyrin, but not an uroporfirin whereas at an urokoproporfiriya in urine the maintenance of an uroporfirin at slight increase of level of coproporphyrin is increased generally increases; increase in maintenance of an uroporfirin in urine and coproporphyrin in Calais is found in most of relatives of patients with an uro-koproporfiriya; in several families 2 — 3 persons suffering from an uroko-pro-porphyria are found — all this speaks about hereditary predisposition to a disease. During all life at patients the increased content of porphyrines can be allocated with a stake, however at a normality of a liver the content of porphyrines in skin and in urine remains normal. At an abnormal liver function porphyrines are not removed in enough in bile, and remain in an organism, are laid in a liver, in skin, emitted with urine.
At an urokoproporfiriya inheritance probably happens on autosomal dominantly type, at the same time not the disease, and disturbance of one of fermental systems is inherited. The nature of fermental disturbances at an urokoproporfiriya is up to the end not clear. So, Rimington (S. of Kimington) assumes that disturbance of recovery of an uroporfirin in the uroporphyrinogen necessary for synthesis gem is the cornerstone of a disease.
The disease is characterized by the expressed skin symptomatology, edges develops at persons more often 40 years are more senior, its main signs are hypersensitivity to a slight mechanical injury and solar radiation, the hypotrichosis, reduction or increase in pigmentation, diffusion thinning or a thickening of skin (a so-called pseudo-scleroderma). Hypersensitivity to a mechanical injury is shown on skin of fingers of hands, necks, persons. On a back of the hand after an injury or the bubble which resulted from influence of the sun there are painted small hems. Retentsionny cysts in skin of fingers, brushes are characteristic of an urokoproporfiriya, sometimes persons who have rounded shape and make in dia. 1 — 5 mm and more (see. Cyst ). At this form P. increase in the sizes of a liver is observed, it is frequent with functional disturbances.
Content of an uroporfirin is up to 2000 — 10 000 mkg / 1 of creatinine at norm to 20 mkg / 1; content of coproporphyrin in urine is increased to 300 — 800 mkg / 1 of creatinine at norm 20 — 80 MSC / 1 therefore to investigate the content of coproporphyrin insufficiently since it increases also at a plumbism and hepatitises. In the period of an aggravation the content of porphyrines in Calais can be normal, at improvement of a condition of patients it can raise.
An effective remedy of treatment is delagil (Chingaminum) which is appointed according to the following scheme: on 0,125 g 2 times a week (2 weeks), on 0,125 g every other day (2 weeks), at good tolerance on 0,125 g daily (1 month), on 0,125 g 2 times a day (1 month), on 0,25 g 2 times a day (3 months). Good and more bystry effect renders a combination of delagil to inosine (inosine) on 0,2 g 3 — 4 times a day within 2 — 3 months, and also use of photoprotective creams.
Forecast satisfactory. At most of patients there occurs remission.
The comparative characteristic of signs of the nek-ry forms P. and other diseases which are followed by increase in amount of porphyrines and their predecessors is presented in the table.
Table. The comparative characteristic of signs of some forms of a porphyria and other diseases which are followed by increase in amount of porphyrines and their predecessors
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L. I. Idelson; L. M. Popova (not BP.).