Diseases of the skin. Atlas.

Desmopathy is a rare hereditary disease associated with a mutation in the DES gene, which is a kind of myofibrillar myopathy. With this disease, it is possible to damage skeletal, cardiac, smooth muscles, as well as diaphragm. In clinical practice, desminopathy is a difficult diagnostic task. In this article, the clinical case of the family form of myofibrillar myopathy with the established mutation c.1021A> C (Thr341Pro) in the DES gene in the heterozygous state is considered. We present a family study in a cumulative calculus of over 100 years describing the dynamics of clinical manifestations, morphological, cardiological and electromyographic parameters.

Key words: desmin, DES, desminopathy, myofibrillar myopathy, mutation, cancer, electromyography, heart failure

A good leader must combine several important qualities at once and perform many functions: competently manage the budget, be a mentor for subordinates and a friend to his clients, create a working atmosphere, set an example, solve problems, set tasks ... Simply put, to be the center of your business .

But there is one more duty that among others is usually not called: if the boss defaults, encourages and directs, he must also punish, and sometimes - severely. Of course, this is very few people enjoy, but it is - as an integral part of the leadership process, like the others.

About what misdemeanors of subordinates deserve indulgence, and which ones can not be forgiven in any case, and also what will be the most serious punishment for a doctor working in a medical institution, we talked with the "Aesthetic Guide".

I want to take a breath – we have done it! The expedition Live Parallel 2013 – Papua New Guinea where we walked many kilometers through the jungle, spent tens of kilometers inside the tarantas and hundreds of kilometers on board small planes flying on the same principle as a route-taxi, has come to an end without loss and above all with the result! But it could be otherwise… Well, let us not talk about sad things but about interesting ones, so… Jakarta was the first place to see.

Parallel worlds exist not only in the books by science fiction writers. One can find them in medicine, too. In this article I will share the analogies that I saw visiting the production facility Lumenis in Israel. It turned out that we have a lot in common with the Israel developers. So ...