الرعاية الداعمة لمتلازمة IPEX: اعتلال الأمعاء، وداء السكري عند حديثي الولادة، وإدارة متعددة الأجهزة

References

DOI: 10.3390/genes12030323

• Before diagnosis, patients usually require supportive care in hospital.
• For patients with severe enteropathy, nutritional treatment, including total parenteral nutrition or elemental or low-carbohydrate-containing formula and fluids, is indicated.
• Patients with neonatal diabetes type 1 need insulin therapy and continuous glycemic control.
• L-thyroxin is necessary for patients with hypothyroidism due to AITD.
• Other replacement therapies with glucosteroids and platelet transfusion are used in thrombocytopenia.
• Autoimmune granulocytopenia and pancytopenia need special hematologic procedures.
• Eczema is treated with antiallergic ointments, balms, and steroids.