PELLAGRA

From Big Medical Encyclopedia

PELLAGRA (ital. pelle agra rough skin) — the disease connected with insufficient receipt in an organism or incomplete assimilation by it nicotinic to - you. In the past P.'s incidence in the majority of districts of the world was connected with preferential consumption of corn (the South of Europe, Africa, Latin America, the southern states of the USA). The exception was made by India and Egypt where in food of the population in the centers, endemic according to P., the ambercane prevailed. In the USSR there is no pellagra. In imperial Russia Bessarabia (Moldova) and to a lesser extent Georgia was the main region of P. B actively participated in studying and P.'s elimination. V. Efremov, A. N. Kryukov, I. A. Kassirsky, V. N. Pervushin, C. M. Ryss, L. A. Cherkes, etc.

the Aetiology and a pathogeny

In P.'s emergence plays a role not only low contents in food stuffs of niacin, but also and the insufficient content of tryptophane. High content in food stuffs of a leucine matters, to-ry slows down synthesis of a coenzyme of NADF in an organism, low level of coenzymes of a pyridoxine, existence in grain products of a niatsitin and niatsinogen, and also the connected forms nicotinic to - you, not acquired by an organism. At P. children usually develops at an unbalanced diet with dominance of carbohydrates; seldom or never it occurs at the children nursed as a result of insufficient contents in food of the feeding woman of vitamins.

Pathological anatomy

Fig. 1. Microdrug of skin of the patient with a pellagra: epidermis (1) otsloyen seroznognoyny exudate (2), in a derma the centers of a necrosis and inflammatory infiltration (2).

Pathoanatomical changes at P. are most expressed in skin, bodies of the alimentary system, c. N of page also depend on a stage and a form of a disease. At gistol, a research in an initial stage of a disease the hyperemia and a cutaneous dropsy, swelling of epidermis, the expressed leukocytic infiltration of a derma (fig. 1) are observed. In late stages there comes the atrophy of epidermis, especially mucous (malpigiyev) layer, the phenomena hyper - and a parakeratosis, a sclerosis of atrofichny nipples and a mesh (subpapillary) layer are observed.

Fig. 2. Microdrug of a mucous membrane of a large intestine of the patient with a pellagra: cysts (1), muciferous, glands of a mucous membrane (2) and leukocytic infiltration of a stroma are visible.

Changes at P. are found almost in all bodies of the alimentary system. In an oral cavity erosion or ulcers are noted; language smooth; on the course of a throat and gullet — the atrophic changes in a cover epithelium and glands similar described in skin. The stomach is reduced in sizes; folds of his mucous membrane are poorly expressed, it is anemic, with single hemorrhages. Atrophic changes in glands are histologically sharply expressed: the quantity them is considerably reduced, reorganization of glands on intestinal type with sites of a kistoobrazovaniye and limfoplazmotsitarny infiltration is noted. In a mucous membrane of a small and large intestine atrophic changes, intestinal glands on a big extent are observed either are absent, or are presented by small cysts with perifocal inflammatory reaction (fig. 2). In a final stage of a disease various forms of colitis are found (catarral, follicular and ulcer and diphtheritic). The liver is reduced in sizes, an atrofichna, rusty-brown color on a section. The phenomena of fatty dystrophy of hepatocytes, cirrhosis and a hemosiderosis of body are histologically noted. Obesity takes all segment or its peripheral departments. Cirrhotic changes find periportalno; in the same sites, and also in hepatocytes on the periphery of segments granules of hemosiderin come to light. The pancreas is atrofichny, it is reduced in sizes, histologically in it the atrophy of an exocrine parenchyma and a diffusion sclerosis are noted.

Kidneys are quite often increased in sizes, with signs gidropichesko-go a nephrosis. Damage of lungs is shown in development at early stages of focal pneumonia, tending in the subsequent to hron, to a recurrent current.

In heart the picture of a brown atrophy with diffusion obesity of a myocardium is observed. In hemadens there are atrophic and sclerous changes; in cortical substance of adrenal glands lrshoida disappear. The spleen is atrofichny, it is reduced in sizes, a fleshy consistence, rusty-brown color owing to plentiful adjournment of hemosiderin in it. Atrophic and sclerous changes are noted in limf, nodes and almonds. In bones — the phenomena osteoporosis (see), connected, apparently, not only with P., but also with exhaustion.

In a head and spinal cord, and also in a peripheral nervous system dystrophic changes of neurocytes with signs are found neyronofagiya (see), a degeneration of nerve fibrils of conduction paths in side and back columns of a spinal cord. In a cerebral cortex growth of a neuroglia is noted.

A clinical picture

P. generally are ill adults, at the age of 20 — 50 years; it meets at school and youthful age, is very rare at early children's age. Clinically expressed P. is preceded by weakness, bystry fatigue, apathy, decrease in working capacity, lack of appetite, weight loss, dizziness, irritability, a depression.

Fig. 3. Face of the patient with a pellagra (pellagrichesky erythema): the erythema around a mouth and a nose is expressed.
Fig. 4. Hands of the patient with a pellagra: erythema of skin of a dorsum of brushes and fingers
Fig. 5. Feet of the patient with a pellagra: erythema of skin of a dorsum.
Fig. 6. Girl sick with a pellagra: damage of skin of a neck, hands, knee joints, distal departments of shins and feet.

Wedge, P.'s symptoms (ponosa, erosion, ulcers in an oral cavity) come usually by the end of winter. Has diarrhea patients on 3 — 5 times a day and is more often. Excrements without impurity of blood and slime, watery, with a putrefactive smell. Through a nek-swarm time patients complain of burning to oral cavities and strong hypersalivation. Lips, in particular lower, swell. On a mucous membrane of lips of a crack. The mucous membrane of a mouth is sharply hyperemic, on gingivas and under language — ulcerations. Changes of language are characteristic. In the beginning its back is pruinose black-brown color, it is sometimes divided by cracks into fields, regions and a tip bright red. Gradually the redness passes to all surface of language, it shines as varnished. Threadlike and fungoid nipples are hypertrophied and act in the form of red points. Language swells, at the edges of it prints zubovg an epithelium are noticeable it is exfoliated — so-called naked, or pontifical, language. These changes resemble a glossitis at pernicious anemia and at a spra. Then the acute phenomena abate, the mucous membrane turns pale, nipples atrophy. On a surface deep furrows sometimes appear. Then on open sites of skin the sang-lagrichesky erythema appears. It is symmetrized on a face, around lips, on a nose (fig. 3), cheeks, on a neck and a forehead, on the back of hands (fig. 4) and feet (fig. 5), is more rare on a breast, forearms and shins (fig. 6). The affected skin of dark red color, edematous, itches. The erythema often is followed by blistering, to-rye burst and leave the becoming wet sites. A number of irritations, and first of all solar radiation can cause the Pellagrichesky erythema. In several days there comes the scaly peeling. After its termination there are sites or with intensive dark-brown pigmentation, or pigment-free, reminding vitiligo (see). At P. it is often observed hyperkeratosis (see) and dense pigmentation (see).

Allocate a so-called secondary pellagra. Cases such P. are described at the patients having diseases of bodies of the alimentary system with an anakhlorgidriya (at cancer of a gullet, an ulcer, cancer and syphilitic damage of a stomach and duodenum, hron, ulcer colitis, tuberculosis, after dysentery), after operations on bodies of the alimentary system, at hron, alcoholism, treatment of tuberculosis by an isoniazid.

The psychopathological picture at P. is very different. At the beginning of a disease asthenic frustration are characteristic (see. Asthenic syndrome ), designated as «a pellagrous neurasthenia». During the period preceding development of a cachexia acute exogenous types of reactions are observed, the delirium is more often (see. Delirious syndrome ) and twilight stupefaction (see). In especially hard cases long amental states develop (see. Amental syndrome ). At development of a cachexia there are Protragirovanny (prolonged) psychoses, manifestation to-rykh reminds endogenous diseases — schizophrenia, maniac-depressive psychosis with a depressive and paranoid or hallucinatory paranoid picture (see. Paranoid syndrome ), and also an apathetic stupor (see. Struporous states ). Depressive and paranoid psychoses — sharply developing states with alarm, agitation, the nigilistic nonsense reaching in some cases nonsense of hugeness (see. Kotara syndrome ). Gallyutsinatornoparanoidny psychoses in one cases remind acute nonsense (see), in others — represent crazy states, tending to systematization, followed by verbal illusions (see), separate hallucinations (see), senestopathetic avtomatizm (see. Kandinsky — Klerambo a syndrome ). The apathetic stupor developing at patients is shown by an aspontannost, an adynamia, absolute indifference to the state, to surrounding (see. Apathetic syndrome ). It is necessary to emphasize in a picture of the described psychoses constant asthenic frustration and possibility of episodes of the dulled consciousness in evening and night time. At the same patients perhaps consecutive development of depressivnoparanoidny, hallucinatory paranoid psychoses and pictures of an apathetic stupor. Such dynamics is observed at patients in cases of the accruing cachexia and a sharp aggravation of symptoms.

Less often at the phenomena of a cachexia there are pseudoparalytic states (see. Psychoorganic syndrome ) and Korsakovsky syndrome (see).

A pellagra at children

At early children's age classical symptoms of P. are expressed not so clearly. Prevail a glossitis, frustration from outside went. - kish. path, erythema. Changes of mentality meet seldom.

Complications

the heaviest complication of P. — encephalopathy (see) with the psychotic reactions arising probably as a result of a shortcoming or the hypoactivity of coenzymes of NAD, NADF and pyridoxal phosphate necessary for biochemical, reactions in a brain.

The diagnosis

the Diagnosis in the expressed cases is easy and based on data of the anamnesis on character of food, and also these researches (at P. Nj-metilniko's keeping by oozes yes in daily urine is lower than 4 mg, in hour urine in the morning on an empty stomach it is lower than 0,3 mg). The diagnosis of mental disturbances at P. is based on ascertaining of one of the types listed psikhopatol. frustration, and also on compliance of a picture of psychosis of a stage of a disease. The item can be a provocative factor to development of an endogenous disease: maniac-depressive psychosis (see), schizophrenia (see). In this case the picture of mental disturbances can be similar to the described depressive and paranoid or hallucinatory paranoid psychoses in the beginning. However lack of direct somatopsychic correlations, dominance in a wedge, a picture of the lines characteristic of an endogenous attack attracts attention afterwards.

Differential diagnosis it is necessary to carry out with system lupus erythematosus (see) and dermatosis.

Treatment

All patients with fresh and recurrent manifestations of P. are subject to hospitalization. Appoint nicotinic to - that or niacinamide (the last is more preferable since does not give unpleasant vascular reaction) inside, after meal, 100 mg 2 — 3 times a day within 15 — 20 days. At mental disturbances low doses of neuroleptics (aminazine, Frenolonum, Triphtazinum) in combination with antidepressants (amitriptyline) and tranquilizers (Seduxenum), to-rye enter intramusculary or intravenously. In cases of development of an organic psychosyndrome appoint high doses of thiamin or nootropil in the form of repeated courses.

It is necessary to lay down. the food rich with equivalents of Niacinum (a niatsinovy equivalent — 1 mg nicotinic to - you or 60 mg of tryptophane). At first appoint a proteinaceous diet without bread (the livers, kidneys, low-fat beef and veal passed via the meat grinder and roasted — on 200 g a day, low-fat fish), then a beef-infusion broth (200 — 400 ml a day), 2 — 3 soft-boiled eggs, dairy products, first of all acid milk, kefir, cottage cheese. After the termination of a diarrhea of patients transfer to the mixed food.

The forecast and Prevention

the Forecast in cases when mental disorders are characterized by an amentia or an apathetic stupor, concerning life is rather serious. If P. proceeding with a cachexia and mental disturbances comes to an end with recovery, reconvalescence usually is followed by the phenomena of an adynamy or a state emotional giperesteticheskoy weaknesses (see. Bongeffera exogenous types of reaction ). At the persons who transferred P. with mental disorders psychopatholike changes of the personality are in most cases observed (see. Psychopathies ) or organic psychosyndrome. Questions of rehabilitation and a readaptation shall be solved differentially depending on expressiveness and depth of the arisen disturbances.

Prevention: the processing of cornmeal a calcareous water releasing the connected form nicotinic to - you; enrichment of cornmeal and grain nicotinic to - that at mill plants or on small mills; enrichment of wheat flour of the highest and first grades nicotinic to - that at mill plants; dignity. education in the field of rational, balanced food (see).



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V. V. Efremov; A. I. Kliorin (ped.), G. M. Mogilevsky (stalemate. An.), A. S. Tygans (psikhiat.).

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