[Inheritance of Wolff-Parkinson-White syndrome and evolution of its clinical symptoms in families of patients in a prospective trial]. / Nasledovanie sindroma Vol'fa-Parkinsona-Uaita i évoliutsiia ego klinicheskikh proiavlenii v sem'iakh bol'nykh pri prospektivnom nabliudenii.

Medicogenetic examination was conducted in families of 46 patients (21 women and 25 men aged 16-74 years) with Wolff-Parkinson-White (WPW) syndrome. A total of 256 relatives were investigated (136 women and 120 men aged 2 to 85 years). As a result, the diagnosis of preexcitation syndrome and phenomenon was made initially in 75(29.3%) of the relatives: WPW syndrome, Clerc-Levy-Cristesco (CLC) syndrome, CLC phenomenon was made in 6(2.3%), 27(10.6%) and 42(16.4%) relatives, respectively. Additional conduction pathways in the families with WPW syndrome are inherited by the autosome-dominant type with penetrability 0.94(94%) and clinical polymorphism. Prospective observation of the families revealed evolution of the clinical symptoms (development of arrhythmia) in the relative with CLC or WPW phenomenon in unfavorable exo- and endogenic factors. WPW syndrome evolution in the patients ran with aggravation of arrhythmia though 12 patients showed improvement.

[Chrono-ecological analysis of population health and ways of its recreation]. / Khronoékologicheskii analiz sostoianiia zdorov'ia naseleniia i sposoby ego rekreatsii.

Reported are results of multiyear observation of the structure of individual and population health in Vladikavkaz, and the authors' concept of health recovery and protection through application of modern high technologies of active chronoadaptation to extreme living conditions. The proposed methods of active chronoadapation, i.e. phytocorrection and phytopro-phylaxis, reflexocorrection, bioradiological correction, bioresonance chronolasercorrection can be also of interest for aerospace medicine.

[The Tel Hashomer camptodactyly syndrome]. / Sindrom kamptodaktilii Tel' Khashomera.

Tel Hashomer camptodactyly syndrome in 2 siblings was described for the first time in Russian literature. Together with camptodactyly flexion folds between phalanges were absent in patients and characteristic face alterations were presented such as asymmetry, hypertelorism, antimongolian ophthalmic eyes cut, high ridge of the nose. There were also observed diffuse skeletal musculature hypoplasia together with humeroradial muscle aplasia as well as two-sides talipes. Inheritance type of syndrome in this family was estimated as autosomal-recessive.

[Heredity in Wolff-Parkinson-White syndrome]. / Nasledovanie sindroma Vol'fa-Parkinsona-Uaita.

Medical genetic consultation was provided for 35 patients with Wolff-Parkinson-White syndrome (WPWS). The investigators ascertained the family history, inquired and examined patients, referred the patients' relatives for ECG and echocardiographic investigations. A newly diagnosed WPWS was reported in 3, Clerc-Levy-Cristesco (CLC) syndrome in 17, CLC phenomenon in 45 out of 132 grade I and II relatives of the proband. The study confirmed autosomal dominant inheritance of WPWS. More frequent occurrence of the syndrome was noted in the proband's relatives in case the disease occurred in both parents, less frequent in families with the history of one WPWS-affected parent. It is suggested that the disease should be considered associated with hereditary predisposition.

[Spinal amyotrophy in adults]. / Spinal'naia amiotrofiia vzroslykh.

The authors describe a rare and little studied form (distal) of spinal amyotrophy. 12 patients were placed under observation. A group of patients with symmetric distal pareses of the hands and legs has been distinguished. Patients with long existent distal monoparesis of the hand or leg are described. The role of EMG in the differential diagnosis is emphasized.

[Neurologic manifestations of Ehlers-Danlos syndrome]. / Nevrologicheskie proiavleniia sindroma Elersa-Danlosa.

Neurologic manifestations were examined in 25 patients with the Ehlers-Danlos syndrome. Injuries to vessels of the brain and spine, arteriovenous malformation, muscular and vertebrogenic lesions, headaches, and vegetative disorders were diagnosed. The clinical and laboratory data are provided whatever the presence or lack of neurologic symptomatology. The problem of the pathogenesis and treatment of neurologic manifestations associated with the Ehlers-Danlos syndrome are discussed.

[The inheritance of disorders of heart rhythm in 3 generations (a family history)]. / Nasledovanie narushenii serdechnogo ritma v trekh pokoleniiakh (opisanie sem'i).

The paper is concerned with the description of a family where father suffered from right bundle-branch block, his daughter--from Wolff-Parkinson-White syndrome (type B) with attacks of supraventricular paroxysmal tachycardia, one granddaughter--from Clerc-Lévy-Cristesco syndrome with attacks of sinus tachycardia, the two other granddaughters had a short P-R interval. Medicogenetic investigation revealed not only the inheritance of myocardial conduction changes of autosomal dominant type but also of syndromes characterizing primary dysplasia of connective tissue in 3 generations.

[Phenotypic characteristics of patients with the premature ventricular excitation syndrome]. / Fenotipicheskie osobennosti bol'nykh s sindromom prezhdevremennogo vozbuzhdeniia zheludochkov.

Wolff-Parkinson-White syndrome (WPWS) was combined in a majority of patients with various syndromes of primary dysplasia of connective tissue determining phenotypic characteristics of patients. They are also of considerable diagnostic importance. During examination of 200 WPWS patients, asthenic constitution, dolichocephalism and arachnodactyly were revealed in most of them. The most frequent stigmata of dysembryogenesis were the high palate (76%) the 3rd type of the ear lobe (36.6%), and epicanthus (20.5%). The funnel chest and thoracic kyphosis in the sagittal plane were noted in 30% of WPWS patients. The syndrome of articular hypermobility was diagnosed in 51.6% of the patients with WPWS, of them 37% had extraarticular manifestations in the form of varicosis of the crural veins.

[Neurologic manifestations of the Marfan syndrome]. / Nevrologicheskie proiavleniia sindroma Marfana.

Examination of 37 patients with Marfan's syndrome has revealed acute disorders of the cerebral circulation, aneurysms of the spinal vessels, varicose spinal veins, myopathy-like syndrome, atlantal semiluxation, spondylolisthesis, epilepsy and cephalgia. The data of additional examinations conducted in patients with Marfan's syndrome with and without neurological symptomatology are presented. The questions concerning the prevention, pathogenesis and therapy of neurological disturbances associated with Marfan's syndrome are considered.

[Positive therapeutic effect of diacarb in the syndrome of progressive muscle spasms, alopecia and diarrhea (Satoyoshi syndrome)]. / Polozhitel'nyu terapevticheskii éffekt diakarba pri sindrome progressiruiushchikh myshechnykh spazmov, alopetsii i diarei (sindrom Satoyoshi).

A 13-year-old girl with Satoyoshi's syndrome is described. The disease manifested itself with generalized cramps, myoclonus, alopecia, diarrhea, growth retardation, muscular hypertrophies, bone malformations, and uterine and gonadal aplasia in the presence of normally developed breasts. Acetazolamide therapy led to a considerable alleviation of cramps and myoclonuses. The child's father displayed gynecomasty, muscular hypertrophy, patent palate, early alopecia and intensified lumbar lordosis; as a child she had had marked cramps in the leg muscles. The examination of the karyotype in the proband and father revealed heteromorphism with regard to the central region of the 22nd chromosome in both cases. Differential staining of the chromosomes failed to show the deletion of any fragment of this chromosome which made it possible to consider this karyotype peculiarity as a variant of the norm.

[Clinical and genetic heterogeneity of the Ehlers-Danlos syndrome]. / Klinicheskaia i geneticheskaia geterogennost' sindroma Elersa--Danlosa.

A clinical description of Type I and II Ehlers-Danlos syndrome in a 3-year-old girl and a 19-year-old boy is presented. The syndrome is characterized by skin hyperelasticity, articular overextensibility, hemorrhagic manifestations and also by excessive vulnerability of tissues. Clinical descriptions are supplemented with roentgenologic, morphologic and electron microscopic findings as well as with the results of amino-acid analysis and of dermatoglyphic examination of the patient and her parents.