Aurora magazine

The Bambino Gesù Hospital in Rome has been the stage for the incredible separation of two Algerian siamese twins. The operation lasted 10 hours and involved a team of 40 people. The Roman hospital is preparing for a new challenge. At the end of November, doctors will shed two Burundi sisters.

The new separation of Siamese is anticipated even more complicated than the previous one. The girls are two and a half months old and are tied to the lowest point of the back. They are attached to the legs, the spinal cord, the bladder and the rectum, sharing a single anus. This last point makes the operation even harder: physicians will have to create a further anus, to give the two girls the same life expectancy.

The news of separation action on Algerian girls has increased the optimism of the operation. But doctors keep us putting pallets: science has its limits and the conditions of Burundi's twins is not easy. In some cases it is impossible to separate a pair of siams without killing one or both of them. There is only one hope that the new case is not one of these.

Although doctors do not bother with the outcome of the future operation, there is good news about the past. One month after the intervention, the little Rayenne and Djihene are fine. They have crossed and traverse difficult times, but the hospital seems normal and devoid of surprises.

Source: ansa.it

Bullous epidermolysis is a rare genetic disorder that causes the formation of bubbles and skin lesions. There is just a minimum trauma to trigger the process, making it impossible to lead a normal life. That is why the little ones who suffer are called "butterfly children": because their skin is as fragile as the butterfly wings. Today, however, there is a new hope.

For the first time a baby butterfly is good: two years after the end of the treatment, little Hassan can live a normal life. The news was announced in an article in the medical journal Nature. The merit is a series of interventions conducted by doctors from the University of Modena and Reggio Emilia, who have operated within a European team.

In June 2015 Hassan arrives at the pediatric hospital in Bochum, Germany. He is 7 years old and suffers from junctal bullous epidermolysis. It is one of the most serious forms of the disease: in 40% of cases it kills before adolescence. There seems to be no hope for the baby, but doctors propose an experimental procedure to the parents. It is a combination of gene therapy and stem cells, developed by an Italian team.

The disease is caused by a defective version of the LAMB3 gene. Doctors use the baby's stem cells to convey the correct gene in the genome to take the place of the defective one. In this way they get healthy transgenic skin handkerchiefs, which gradually transplant to the baby. Two years after the last intervention, it's official: the therapy worked and Hassan is fine.

The case of little Hassan also revealed some obscure points of tissue regeneration. It was already known that the skin is renewed once every month. You did not know what to drive regeneration. It has come to light that there are few stem cells of long duration. Cells are continually renewed, creating new healthy cells to replace those deaths. The discovery will facilitate the processing of new treatments for many other incurable diseases today.

Source: lescienze.it

According to two studies, drugs for attention deficit and hyperactivity disorder (ADHD) could increase the risk of anomalies if they are pregnant. Their intake is due to a modest increase in perinatal and plancenter abnormalities. But the authors warn: the risks of untreated ADHD could be greater.

In some cases, medications are essential to controlling the symptoms of attention deficit. Only in this way can it be ensured that the mother pays due attention to her own health and that of the child. Conversely, untreated disorder could lead to increased stress and risky behaviors for the fetus as well.

Dr. Jacqueline M. Cohen points out that the risk rate caused by drugs does not justify abstinence from treatment. In fact, this is a small increase, especially if you consider the risks caused by some untreated forms of ADHD. The important thing is for the future mother to undergo constant screening to identify any complications for a while.

The second study was led by Dr. Nörby. The Swedish physician analyzed the data of 964,734 newborns, including 1591 exposed to prenatal ADHD drugs. Others 9475 were the children of women who used drugs before pregnancy, but not during. Data showed a higher rate of newborn infants in the first group. There have also emerged several cases of central nervous system disorders.

Compared to women with ADHD who did not undergo treatment, women who had continued to take the medications were healthier. There were fewer cases of obesity, nicotine abuse, or drugs. All the differences that contributed to a healthier pregnancy. Doctors then noted an increase in drug-related risk, but with a decrease in those related to other factors.

Source: medscape.com

Bullous epidermolysis is a rare genetic disorder also called "butterfly syndrome". The skin of those who suffers it is as fragile as the wings of a butterfly. Just a minor clutch to cause the appearance of bubbles and injuries.

The disease has three forms, ranging from the mild to the lethal:

• simplex;
• dystrophic;
• junctional.

In the case of joint juncture, the disease also affects the mouth and throat. This makes it difficult to eat and swallow. In some cases, the disease also affects internal mucous membranes and causes almost unbearable pain. Often injuries are also difficult to heal.

The disease usually occurs at birth or in the next few months. Localized shapes can, however, emerge even in late childhood or early adulthood. In Italy, bullous epidermolysis affects about 1 child every 82,000 births. The average is lower than the world average, which is around 1 child per 17,000 births. The total number of people in the world is about 500,000.

Bullous epidermolysis is inherited and can be autosomal dominant or recessive, depending on the subtype. If you are present in the family history of one of the couple's two members, you should contact specialists. In the case of recessive inheritance, in fact, both parents are healthy carriers and do not manifest the illness in any way. Nevertheless, the fetus has 1 out of 4 possibilities to manifest it.

The simplex form is the least serious and most common. It affects about 50% of affected subjects and affects almost exclusively hands and feet. In some cases, continuous scarring results in fusion of the fingers, reducing its functionality. It is common for patients to suffer from anemia, or who incur tumors and nephrologic problems. Junctional bumpy epidermolysis is the rarest (1% of cases) and is also the most severe. In many cases it is lethal since childhood.

Compared with other genetic diseases, bullous epidermolysis is easy to diagnose. There are no effective treatments, though. You can only avoid the appearance of bubbles as much as possible, protecting the skin from possible trauma. It is also important to prevent possible infections from wounds. Those who suffer from the most serious forms must undergo continuous monitoring, but there is nothing else to do.

Source: observatoriomalattierare.it