Not afraid of allergy 不再恐懼過敏

中區兒童免疫氣喘專家會議

時間: 2016 / 03 / 19 (六)     地點: 中國醫藥大學附設兒童醫院 (兒童醫療大樓11樓會議室)

Case 1.

Erythroderma since birth，最後診斷 Omenn syndrome

身體檢查方面，出生就有發現肝脾腫大，Ｘ光上沒有明顯胸腺。

早產兒，因黃膽指數過高，經換血治療，在十年前，血液沒有 irradiation 的技術，因此 T-lymphocyte 沒有被殺死，因此早產兒/嬰兒(因黃膽指數過高)在換血後出現類似 GVHD 的現象。

治療: Advgraf (1mg) 1# HS, 等待 bone marrow transplantation

Important points:

1. GVHD 的皮疹很特別，可以去血液科看移植後病人的皮膚疹。
2. 一出生有erythroderma, 加上不斷反覆感染，要往先天免疫缺陷查原因。而要特別注意是否有Wiskott-Aldrich syndrome and Ataxia-Telangiectasia,

Case 2.

Hypereosinophilia，final diagnosis: AML, ambiguous type

Important points

1. 兒科的疾病，要非常注意是否有1. Leukemia 2. Bone tumor

如果血小板比較低，要注意是否是leukemia, check uric acid and Alk-P initially

2. Hypereosinophila may be due to underlying disease, watch out end organ damage

Case 3.

Hypoglycemia since birth, final diagnosis: Adrenal insufficiency due to maternal corticosteroid

Related history: Mother: SLE, Sjogren syndrome

Initial newborn with hypoglycemia,

Use hydrocortisone, then shift to cortisone acetate

Important points: 

1. after high dose cortico-steroids, if the patient got traffic accident or high fever, remember to add corticosteroid
2. Pregnant woman with SLE, Sjogren or rheumatology disease, suggest prednisone better than methylprednisolone
3. Hypoglycemia at birth, remember to think adrenal crisis/ adrenal insufficiency, try hydrocortisone first,

Case 4.

Intractable asthma patient

Post Xolair 33 doses treatment,

Obesity, BMI 33.

Chest CT:　Lymph node enlargement

Pathology: lymphoid hyperplasia, final diagnosis as Castleman disease.

Further strategy: bronchoscopy exam, keep Xolair

Important points:

1. Intractable asthma, remember imaging study to exclude lymphoma, other tumor, malignancy
2. Check underlying disease, e.g. cyctic fibrosis, Primary ciliary dyskinesia

Case 5.

Left axilla mass with fever.

Final diagnosis as BCG related lymphadenitis.  Treatment: isoniazid for short term

Important points:

1. Check CGD possibility
2. Try to apply VICP for this patient

結語:

1。中區兒童免疫的醫師，對於病人臨床討論互動非常熱絡。前輩醫師很願意分享自己的臨床經驗與心得，提昇後輩的診斷能力，提醒如何早期診斷罕見疾病，充分發揮醫學傳承的精神。

2。病人表面上的問題，往往只是冰上一角的狀況題，身為次專科醫師，一定要盡力把疾病的最源頭的原因找出來，雖然不一定可以治愈，但對病情的掌握與疾病的預後，會更加了解。