Children do not like to eat when they are young, but after one year old, they overeat and lose their temper? wary! It could be this condition...

When he was born, he did not like to eat, his body was thin, and after two or three years of age, he increased his food intake and became white and fat, but his hands and feet were also small, and at this time, he (or she) could not control himself when he saw food, and sometimes he would lose his temper. You and I may have such children around us, what is wrong with them? Is it just gluttony?

The doctor reminds that if the child has such a change, it is necessary to be vigilant, it is likely that the child suffers from little fat Willie syndrome. Early diagnosis and long-term comprehensive treatment are very important for children with this disease.

【Case】Small food intake at birth Grow up but eat a lot

When Xiao Yuan (pseudonym) was a few months old, he was much smaller than children of the same age, not only small in stature, but also in small food, and his mother wanted to feed more milk, and Xiao Yuan would keep crying. Parents also think that the child's thinness is caused by the small amount of food, so they hold the child to the pediatric department of Zhongshan Hospital affiliated to Xiamen University. Chen Ling, the director of pediatrics, carefully examined Xiao Yuan after understanding the symptoms, "Isn't this the symptom of Little Fat Willy Syndrome in infancy?" After genetic testing, Chen Ling's speculation was confirmed.

Yin Yin (pseudonym) is more than 4 years old. At the time of birth, Yin Yin was hospitalized for more than ten days because of softness and poor eating, but the local hospital failed to detect the problem in time. After being discharged from the hospital, Yin Yin did not eat much and was still relatively thin. Unexpectedly, after the age of 1, everything is different. The amount of food heard suddenly became larger, and by the age of two or three, it was much fatter than children of the same age. Although he gained weight, his language skills were not good, and until the time of the visit, his speech was still difficult. Parents brought the sound to the Behavioral Development Department of the Municipal Maternal and Child Health Hospital for treatment. After understanding the child's daily situation, the behavioral development doctor referred the tone to Lu Mei, the deputy chief physician of the Department of Pediatric Internal Medicine. After genetic testing, Yin Yin was diagnosed with Chubby Willie's syndrome and is currently undergoing systemic treatment.

【Etiology】Chromosomal abnormalities cause different symptoms at different ages

Chubby Willie syndrome is a disease caused by chromosomal abnormalities, although it is a rare disease, but the incidence is between 1 in 10,000 and 1 in 30,000. Children of different ages have different symptoms.

Chen Ling and Lu Mei, two doctors, told reporters that in the newborn and infancy, the performance of children with Little Fat Willie Syndrome is mainly small food intake, poor sucking ability, and feeding difficulties. Because of low muscle tone, children tend to be thin. "If they don't get timely and effective treatment, they will not be able to thrive because of poor feeding, and they will easily combine symptoms such as hypoglycemia and pneumonia and lag behind in early motor language development." These children also have a special face in infancy, with white skin, almond eyes, long faces, small mouths, thin upper lips, downward corners of the mouth, and small hands and feet. Lu Mei said that after the age of one, the amount of food eaten by these children will gradually increase, and even overeating, which is why children with Little Fat Willie Syndrome are called "gluttonous angels". "Long-term dietary overdose can lead to obesity, and under obesity, there are many hidden crises, and even the threat of death may be faced at any time."

Chen Ling said that in the case of parents deliberately restricting diet, some children will find non-food to eat on their own, and there may be problems such as esophageal foreign bodies and digestive tract diseases. If the child snores at night, it is most likely that the apnea caused by breathing obstruction, in such sleep, the child will be deprived of oxygen, may affect the nervous system, and even sudden death.

Lu Mei said that excessive obesity may also cause heart and lung diseases. "This kind of child will never have enough to eat, there have been children who eat more than a dozen takeaways at a time, which increases the burden of obesity and various organs of the body, and finally the child died at home because of his heartbeat and breathing, which is a pity."

With age, children will have cognitive and behavioral abnormalities, such as developmental delay, weak language ability, backward sexual development, and lower intelligence than their peers. "Children will also have endocrine axis abnormalities." Chen Ling said. "In addition to development, their personalities also change, and they are prone to anger or compulsive behavior." Lu Mei said, "However, even when angry, most of the children are always very warm children." They may hurt themselves, such as self-inflicted skin damage, skin cutting, and rarely hurt others. ”

Lu Mei said that when she was still in her mother's belly, some children may have signs that they will not like to move or have abnormal fetal position, and need midwifery or caesarean section.

【Treatment】Early comprehensive treatment and training can prolong life and improve quality of life

Both doctors said that although the current medical treatment can not cure the little fat Willy syndrome, but after early comprehensive treatment and training, can extend the child's life and improve the child's quality of life.

Chen Ling said that under strict nutritional control, the endocrine level of the children can be improved by growth hormone treatment, so that their growth and development are close to the level of their peers, and their intelligence can be improved through training. "We have such a child on our side, and after active treatment, sometimes we can't even see that this is a little fat Willie syndrome child, especially when he is quietly doing handicrafts." Lu Mei said.

Chen Ling said that the follow-up treatment of Little Fat Willie syndrome is a comprehensive and long-term process. Children need to have a specialist for lifelong follow-up, who can supervise the damage and treatment of their various organ systems at any time, but more importantly, the parents' efforts. Only when parents are always with their children can they get better treatment results.

"For example, growth hormone is currently a clear and effective drug for the treatment of Little Fat Willie syndrome, and parents must learn to inject growth hormone." For another example, parents should strictly control their children's diet and let their children develop good eating habits. Continuous comprehensive training, such as language, behavior training and other skills, need to be mastered by parents to help children train in their daily lives. Lu Mei said.

【Recommendation】 Early genetic testing can improve the rate of early diagnosis

"Chubby Willie syndrome may be misdiagnosed as another disorder, such as obesity, appetite overdose, language retardation, mental retardation, hypogonadism, etc." Chen Ling said that in recent years, with the improvement of pediatric and neonatologists' awareness of Little Fat Willie syndrome, more children in our city have been diagnosed at an early stage. "Many children will seek medical treatment for sexual delay, endocrine disorders and other problems as the first symptoms, and the pediatric clinician will conduct a genetic examination for the child after making a preliminary judgment based on the symptoms, which can be determined."

"Children can be diagnosed even after birth or a few months after birth." Lu Mei said that there are corresponding scoring standards in the neonatal period, and children with low scores and special faces will cause doctors to be alert, and early genetic testing can further improve the early diagnosis rate.

【Reminder】 If there are such patients in the family, pregnant women should be genetically screened before giving birth

Chen Ling reminded that if there are patients with Little Fat Willie Syndrome in the family, when pregnant again, it is recommended to carry out prenatal diagnosis genetic screening to reduce the probability of this genetic disease.

A large part of genetic diseases or congenital diseases such as Little Fat Willie syndrome can be found and treated in time in the womb through prenatal diagnosis, thereby greatly reducing the incidence of birth defects, improving the quality of the population, and reducing medical investment.

【Explanation of terms】

Little Fat Willy syndrome, medically known as pradar-Willi syndrome, referred to as PWP, is a rare congenital disease, a genetic disease caused by the long arm abnormality of chromosome 15, and is a complex syndrome with abnormal multi-system differentiation. Common clinical manifestations are low muscle tone, motor retardation, hypogonadism, backward intellectual development, abnormal language and behavioral ability, abnormal development of teeth, eyes, and bones, long-term strong hunger and excessive eating, which can cause obesity, as well as various metabolic diseases secondary to obesity. There is no radical treatment, and it is necessary to live under supervision for life, mainly to control obesity and various metabolic disorders.

(Xiamen Evening News reporter Kuang Wei correspondent Shi Qingqing Chen Feng)