Periodic disease

Periodic disease - separate nosological form, characterized by periodic acute emerging brutal attacks of abdominal pain (often in the chest, joints), fever (temperature rises to 40 degrees and above). After the attack, which lasts 2-3 days, patients quickly recovered and restored to full working capacity. The disease is a chronic, often favorable for, continuing for decades.
The term " periodic disease" Rei-Mann (N. A. Beimann) joined in 1948 known before the disease: benign paroxysmal peritonitis, periodic fever and recurrent artralgia.
Periodic dropsy joints known since 1840 In 1898 Imperial Saucillo described in the thesis about neuropathic edema recurrent benign peritonitis, intermittently, the dropsy of the joints and other similar syndromes; in 1938, L. A. Oganesyan - abdominal paroxysms with fever as chronic alternating six-day fever.
For the first time in Soviet literature of modern lighting periodic disease gave E. M. Tareev and C. A. Nasonova (1959), watching over 30 patients after every 100 seizures and more; detailed periodic disease studied O.M. Vinogradova. To date, section b. had a lot of synonyms - periodic peritonitis, recurrent polverosa, familial Mediterranean fever, Mediterranean "epanaleptic", family recurrent polverosa, family periodic disease, disease Sigala - Mom etc.
The etiology and pathogenesis of P. B. not completely understood. However, affirms the doctrine of P. B. how about genetic disease affecting up to 4-5 generations, most of the Nations of the Mediterranean basin - Armenians (which is confirmed by the experience of domestic authors), Arabs, Jews.
Periodic disease often begins with the first years of life, usually in adolescence, can be complicated after years flow by amyloidosis; the last (installed observations in affected families) may occur independently as equivalent to the pain of febrile seizures.
Expected infectious nature of recurrent disease is not confirmed by observations of clinicians who sought all kinds of modern techniques, including the removal of some bodies, to identify and eliminate the source of infection in patients.
Pathogenesis manifestations P. B. approached angioneurosis, for which also the characteristic frequency of epileptic [for example, the patient described by Asirom (W. Osier)during 74 years of attacks of angioedema was always combined with fever], diencephalic syndrome, characterized by periodic syndromes and the emergence of a more significant violations of metabolism, in particular globulin, which allows to come nearer to understanding the development of amyloidosis. Not confirmed endocrine concept of recurrent disease [was found that the high content in the blood pyrogenic substances eticholeve (derivative of testosterone) is not characteristic P. b., and special ationallevel fever], and allergic or autoimmune, the concept of the authors, who tried to bring together P. B. with collagenoses. Kind of inflammatory-febrile reactions, development of amyloidosis in family genotypic the nature of the disease suggests the presence in section b. deeper metabolic and enzymatic disorders, as in several other genetic diseases. However, this area is still insufficiently studied.
Pathological anatomy. Morphological changes in periodic disease are reduced to congestion, edema serous membranes (the peritoneum, pleura) with deposition of fibrin and sterile effusion during the paroxysm-installed with lopartialkey and laparotomy, to the development of growths, especially on the liver and spleen.
Vasculitis, plazmotsytoma-reticular infiltrates not find.
With the development of purulent peritonitis from severe dyskinesia of intestines (intussusception), and when amyloidosis are usual for these States anatomical changes (with extensive amyloid deposits in the walls of blood vessels).
The clinical picture separate attacks P. B. a very characteristic. Unexpectedly or after brightly expressed prodromal phenomena outside breach of food mode, trauma, cooling played attack spilled pain near the navel or around the abdomen (often), lasting several hours, or even days, with the rigidity of the abdominal wall, vomiting, bloating, termination of discharge gas and feces, initial spectacular chills and high continuous fever to 39-41° with steep drop it on 2-3rd day of illness. It is pain, vomiting, chills most painful and afraid of the patient when waiting for the next episodes. When paroxysms of torakalgii the patient is forced to commit chest, unable to make a normal breath. During the attack are neutrophilic leucocytosis to 15 000, sometimes up to 40 000 and above, the acceleration ROHE up to 30 mm and above, other non-specific symptoms of the inflammatory process, find out laboratory, a little protein in the urine. Can priuchat the liver and spleen, which is accompanied by a small jaundice, rarely increase lymph nodes. Often the paroxysm permitted diarrhea lasting for 2-3 days and up to a week. The patient is losing weight, looks tired, depressed by fatality repetition of new seizures.

Radiographically during the paroxysm find bloated loop guts, especially thin, with a "levels", delay promotion of barium, phenomena paralytic ileus, in rare cases, the invagination of the large intestine as the result of a dyskinesia.
Laboratory find high concentrations of fibrinogen in the blood plasma at little changed in the rest of the protein formula, sometimes moderate increase bilirubinuria or protein in the cerebrospinal fluid.
With the development of amyloidosis there are usual for this state changes (laboratory and in organs).
For the flow of recurrent disease characterized by repeated bouts (up to 100-300 times and more during the years of illness), usually over time become more frequent, for example first time in 1-2 months, and later on a monthly or even more often.
For some patients seizures strictly linked, at least in some periods of the disease, with a certain number of month, or day of the week; women tend to have a relationship with the menstrual cycle. The attacks can be seasonal in nature, being absent in winter (often) or in summer (rarely); at this time the patients as if they forget about their disease, exercise, etc. But periodic disease can Deplete patients with frequent attacks, to break the power to cause asthenia personality, substance abuse (abuse of morphine injected). Pregnancy may reduce the frequency of seizures.
Periodic disease is not curable with antibiotics, antihistamines, chloroquine, difeninom, proteins, vitamins and corticosteroids.
Symptomatic analgesic effect ganglioblokatory (geksony). The settlement of the situation, mode and sedatus therapy can have a beneficial effect. Approximately 1/5 of patients develop amyloidosis, leading to death from uraemia.
The diagnosis is made on the basis of the clinical picture of the disease, familial nature, priority diseases of people of certain nationalities, inefficiency, anti-infective and other conventional therapy. When the first attacks and for admission under the observation of the new doctors ' patients are often subjected to surgery (removal of the Appendix, gallbladder, kidneys) due to the erroneous recognition of the local organ of the disease. When the diagnosis of recurrent disease it is necessary to rule out other diseases that occur with repeated exacerbations: infections (sepsis, brucellosis, malaria and so on), allergic [hay fever and asthma, first described by Vostokom (J. Bostock, 1819) as "periodic disease of the eye and chest", drug fever with rash, leukopenia, etc.]; collagenosis, retikulez, leukemia and so on; neurogenic fever - "feverish neurasthenia" (C. D. Shervinsky), the conditioned-reflex chills (I.e. Century Simonovic), vegetative disease (B. A. Chernogorov), vegetative fever and so on, it is Important to remember that P. B. first of all characterized by pain syndrome, guided by the idea of "acute surgical abdomen", which is not, as a rule, when the diseases listed in differential diagnosis.
Treatment and prevention are insufficiently developed. In rare true surgical complications (for example, when intussusception of the bowel, perforating peritonitis) shows the urgent surgical intervention. For the prevention and treatment of early amyloidosis recommend long hepatotherapy.