BERIBERI (synonym: avitaminosis of B 1 , alimentary polyneuritis, polyneuritis endemica) — the disease arising owing to insufficient receipt in an organism of vitamin B 1 or as a result of disturbance of its utilization in an organism and characterized by generalized defeat of peripheral nerves (polyneuritis), muscular atrophies, heavy frustration of cardiovascular system.
Mentioning of B.-. is available in the most ancient medical treatise which reached our days — in the Chinese text of the second millennium BC. The first detailed description of B.-. belongs to the Dutch doctor Eykman (S. of Eijkman, 1897).
The etiology and a pathogeny
the Disease meets hl. obr. in the countries of the Southern and East Asia. The main reason for a disease — consumption of the polished rice poor in not only thiamin, but also other vitamins of group B. Therefore B.-. it is necessary to consider as complex B-avitaminosis. The alimentary form B. - occurs among the population eating preferential only the polished rice. Phenomena of a hypovitaminosis of B 1 are noted at a long uniform diet, from to-rogo rough grades of bread (rye, gray wheat), beans, grain and other products containing vitamin B are excluded 1 , and also at patients with went. - kish. diseases at a long irrational diet. Unbalanced food with a significant amount of carbohydrates, thyrotoxicoses, pregnancy and feeding, steady physical stress, and also work at low or high temperature of the environment at which the need of an organism for thiamin increases promote development of an exogenous form B.-. Endogenous, or secondary, a form B.-. results from disturbance of absorption, assimilation or normal metabolism of thiamin. Binding and an inactivation of thiamin, and also its bystry destruction can be caused by excessive use of caustic solutions, the products containing a thiaminase, sulfanamide drugs and antagonists of thiamin (oxythiamine, pyrithiamine, butylthiamin).
The pathological anatomy
Is observed sharp general exhaustion, hypostases — limited or widespread. Heart is increased preferential due to expansion of its right departments, the arterial cone is sharply expanded. Adrenal glands, a hypophysis and a thyroid gland are increased. Muscle fibers of heart are moved apart by a large amount of serous liquid; fatty dystrophy of a myocardium, degenerative changes in a peripheral nervous system — disintegration of axons of nerve fibrils and their myelin covers are noted.
A clinical picture
For B.-. cardiovascular frustration, defeats of peripheral nerves and hypostases are characteristic. Usually allocate three forms of this disease: acute, or so-called wet, a form with hypostases and dominance of signs of defeat cordial sosudnstoy systems; dry, or atrophic, a form — hron, the disease which is characterized by disturbances of a peripheral nervous system which consequence defeat of various groups of muscles is (leads to paralyzes and paresis of the lower extremities more often); children's form — sharply proceeding disease.
The first complaints at B.-. — weakness, bystry fatigue, a headache, sleeplessness, an asthma at an exercise stress, interruption of heart and heartbeat. Then there are signs of defeat of peripheral nerves — paresthesias and decrease in sensitivity of toes, shins and feet («paper soles»), mezogastralny area (around a navel) and forearms. The patient complains of weight and weakness in legs, bystry fatigue during the walking, lameness, spasms. At a palpation sharp morbidity of gastrocnemius muscles, their testovidny swelling, terebrant pains on the course of nerves is noted. Preferential nerves of extremities are surprised, is more often than lower. In the subsequent paresis and paralyzes of various groups of muscles are observed, muscular atrophies develop, function of extensive muscles is broken. Sick B.'s gait - is characteristic.: it steps on fingers and on the outer edge of foot in the beginning, sparing a heel; further it is forced to use crutches.
At damage of upper extremities fingers hang down on a palm, and a palm as claws — on wrists. Similar motive and sensitive frustration can be observed also in muscles of a trunk. Electric excitability of the affected muscles varies from lowered to full reaction of degeneration; tendon jerks gradually die away.
Changes from cardiovascular system are connected with exchange frustration and the central disturbances of regulation of a vascular tone: tachycardia at rest, sometimes over 100 beats per minute, lowering of arterial pressure, generally diastolic, and increase in venous pressure; the blood stream is accelerated, the minute volume of heart increases; borders of heart are expanded, generally to the right; the epigastriß pulsation is observed. Systolic and presystolic noise at a top of heart are listened. Cardiac sounds are carried out on femoral, humeral arteries and arteries of the back of foot. On an ECG shortening of an interval of PQ, decrease in a voltage of teeth of T in I and II assignments, signs of an overload of a right ventricle are noted. Hypostases are connected both with exchange disturbances, and with insufficiency of cordial activity.
Also changes from outside are observed went. - kish. path: loss of appetite, heartburn, nausea, vomiting, locks. The patient loses flesh, signs of a cachexia appear.
Visual disturbances are noted: reduction of visual acuity, often the amblyopia reminding alcoholic, the central scotoma and narrowing of fields of vision; the mentality — concern, easy excitability, apathy is broken; the patient cannot concentrate attention on something, the sleep is interrupted, frequent headaches are noted.
At a dry form B.-. signs of defeat of a nervous system dominate, at wet — cardiovascular. Easy extent of cardiovascular disturbances at B.-. it is characterized by an asthma and heartbeat; at moderately severe disturbances and heavy the progressing circulatory unefficiency as right ventricular comes to light.
Most hard the kind of an acute form B. - proceeds. — so-called. Pernicious, or cardial, a form (on terminology of the Japanese authors, «шошин» — an average push) — the malignant fulminant form which is quite often coming to an end with death of the patient. Sometimes B.-. proceeds as a hemorrhagic polioencephalitis and quite often comes to an end with a coma (see. Polioencephalitis ). In this case into the forefront psychological frustration (anorexia, an adynamia, sleeplessness, hallucinations, a disorientation in space and time, loss of memory etc.) and disturbances act from c. N of page (paralyzes of oculomotor muscles, a diplopia, an ophthalmoplegia, a nystagmus, are more rare — loss of visual acuity, an ataxy).
A children's form
children of the first months of life whose mothers are sick with B. - Are ill., therefore in breast milk there is no vitamin B 1 . In view of a variety of clinical manifestations to distinguish B.-. at children often hardly, especially in the absence of a clear picture of a disease at mother. The child has an anorexia, he refuses to take a breast, loses flesh. The cold snap of extremities, the complicated breath, the expressed paroxysmal pains forcing to accept the child the motionless provision, an osiplost of a voice and an aphonia is noted (the child cries silently). The cardiovascular disturbances characteristic of B. - join it. Also motive concern, muscle tension of a nape, a spasm of muscles of extremities, paralyzes of returnable and oculomotor nerves are described. Big hypostases meet rather seldom. Within one-two days from the beginning of a disease at the child there comes the drowsiness which is replaced by coma. The disease often comes to an end with sudden death.
the Diagnosis in the expressed cases is easy and based first of all on data of the anamnesis (character of food, existence of the diseases interfering digestion of vitamins of group B, etc.). Biochemical researches facilitate diagnosis: at B.-. content of thiamin in daily urine is lower than 100 mkg, in an hour portion on an empty stomach it is lower than 10 mkg; the maintenance of a tiamindifosfat (cocarboxylase) in erythrocytes is lower than 2 — 4 mkg of %, pyroracemic to - you in plasma — is higher than 1 mg of %; activity transketomanholes in a hemolysate of erythrocytes is increased more than for 20%. To children for specification of the diagnosis sometimes urgently enter thiamin — improvement occurs very quickly.
The differential diagnosis
the Differential diagnosis should be carried out with infectious polyneurites (at poliomyelitis, diphtheria, etc.), with polyneurites at pernicious anemia, diabetes, pregnancy, alcoholism, poisonings with mercury, tetraethyl lead, methyl alcohol, carbon sulfur, phosphorus, arsenic, ceremonious. At B.-., as a rule, there is no fever, symptoms of right ventricular insufficiency prevail, there is labile frequent pulse, vascular tones on arteries of a hip, a shoulder and the back of foot. Function of kidneys is not broken, there are no skin phenomena, ponosa, changes of blood, sharp disturbances of mentality. From toxic polyneurites of B.-. differs in typical localization of defeats, lack of full anesthesia and a hyperesthesia, pain during the pressing on muscles, but not on nervous trunks, existence of parallel and symmetric disturbances of sensitivity and mobility, and also the cordial phenomena and hypostases. Unlike intoxication ceremonious (see. Lathyrism ), paralyzes not spastic, but atrophic.
Forecast in most cases favorable; the exception is made by the acute shoshin form, Vernike's encephalopathy (see. K. Vernike ) and sharply proceeding B.'s cases-. babies.
Treatment in the acute period in the presence of the cordial phenomena and hypostases is carried out in a hospital. At cordial symptoms usual cardiovascular means (a lily of the valley, a foxglove, etc.) have almost no effect. Introduction fiziol, solution and glucose contraindicated. For reduction of hypostases appoint enemas from an Euphyllinum (0,3 — 0,4 g). After an enema, in 10 hours — injections of mercusal of 0,2 — 0,4 ml. Are shown cold on area of heart, an artificial respiration, oxygen. For maintenance of a tone of a nervous system and a cardiac muscle appoint injections of strychnine (1: 1000 on 1,0 — 1,5 ml). The balanced clinical nutrition is recommended. At the acute phenomena of B.-. specific treatment is carried out: parenteral administration of thiamin on 30 — 50 mg a day within 15 — 20 days (effect of thiamin on heart is shown in 24 — 48 hours). Nervous disturbances disappear more slowly. On reaching effect of a dosage of thiamin reduce to 10 — 20 mg (orally) in day during the same time. A dose of thiamin for babies: to 10 mg a day parenterally, and then 5 mg a day orally (the same terms, as for adults). Along with it physiotherapeutic procedures are shown.
Prevention: inclusion in a diet of the products rich with thiamin (need of the healthy adult for thiamin makes 0,6 mg on 1000 kcal of a diet). At decrease in content of thiamin in milk of mother (there have to be 0,1 — 0,2 mg in 1 l) vitaminization of mother thiamin is necessary.
See also Vitamin deficiency .
Bibliography Efremov V. V. The most important avitaminosis of the person, page 29, M. — D., 1939, bibliogr.; Kassirsky I. A. and Plotnikovn. H. Diseases of tropical countries, page 423, M., 1964, bibliogr; Ostrovsky Yu. M. Thiamine, Minsk, 1971, bibliogr.; Ryss S. M. Nedostatochnost of polyneuramin i (thiamin), In t — avitaminosis, Much tomn. the management on vnutren. to diseases, under the editorship of E. M. Tareev, t. 8, page 521, M., 1965, bibliogr.; Shilov P. I. and Yakovlev T. N. Fundamentals of clinical vitaminology, page 135, L., 1974; Bicknell F. Prescott F. The vitamins in medicine, p. 183, L., 1953, bibliogr.; Review of Japanese literature on beriberi and thiamine, ed. by N. Shimazono a. E. Katsura, Tokyo, 1965; StannusH. S. Infantile beriberi and beriberi heart, Lancet, v. 1, p. 756, 1942, bibliogr.; S tep pW „Kiihnau J. u. Schroeder H. Die Yitamine und ihre klinische Anwen-dung, Bd 1, S. 103, Stuttgart, 1952, Bibliogr.; VedderE. B. Beriberi and vitamin Bt deficiency, Amer. J. trop. Med., v. 20, p. 625, 1940.
V. V. Efremov.