Chất lượng cuộc sống của bệnh nhi dày móng bẩm sinh trước và sau khi can thiệp hỗ trợ chăm sóc

Evaluating the results of supportive care for patients with pachyonychia congenital in 6 months. Subjects: the patient was diagnosed with congenital pachyonychia by keratin gene analysis. Research method: describe a series of cases combined with clinical interventions to compare before and after. We studied 7 patients congenital pachyonychia with mutations in the keratin gene KRT6A included 3 female patients and 4 male patients. All patients have had clinical signs at less than 1 year of age, and they were diagnosed the disease at different ages. These patients have had characteristic manifestations of the disease such as hypertrophic nail dystrophy, focal palmoplantar keratoderma, plantar pain, oral leukoplakia. The patient's quality of life was affected such as limitations on daily living, social and recreational activities, their relationships with friends and neighbors and feelings of shame. We conducted a few basic interventions and obtained some initial results that improved the patient's relationship with other person, the patients were more confident, more comfortable in communication about their illness. Besides, the average foot pain in 1 week before the intervention was 8.00 ± 1.41, after 6 months of intervention was 7.57 ± 0.97 (p>0.05). Nail changes, pain from the palmoplantar keratoderma of pediatric patients greatly affected the psychosocial effects. The care intervention improved social confidence and reduced the average number of leg pain per week in sick children.