Tubiana-Rufi N, Thizon-de Gaulle I, Czernichow P
Service d'Endocrinologie et de Diabétologie Pédiatriques, Hôpital Robert-Debré, Paris, France.
Horm Res. 1992;37(1-2):60-3. doi: 10.1159/000182283.
We report on a 5-year-old child who survived an intracerebral crisis, following ketoacidosis-revealing diabetes (DKA), with visual impairment due to a vascular occipital lesion. Two and 4 months after the initial episode, a unique hypothalamopituitary disorder consisting in GH, ACTH, TSH deficiencies and central precocious puberty, was detected. Cranial magnetic resonance images showed no visible lesion in the hypothalamopituitary region. The most likely hypothesis is the ischemia of hypothalamopituitary and occipital regions following possible cerebral edema after hyperhydration. She survived with low visual acuteness and received a combined replacement therapy for the neuroendocrinological deficiencies. This case emphasizes that the rehydration at the initial period of DKA is critical, especially when risk factors for cerebral edema are present (young age, marked hyponatremia). The neuroendocrinological consequences of acute cerebral edema are rare, but physicians must be attentive in survivors of these accidents.
我们报告了一名5岁儿童,该儿童在糖尿病酮症酸中毒(DKA)引发的脑内危机中幸存下来,但因枕叶血管病变导致视力受损。在初次发作后的2个月和4个月,检测出一种独特的下丘脑 - 垂体疾病,表现为生长激素(GH)、促肾上腺皮质激素(ACTH)、促甲状腺激素(TSH)缺乏以及中枢性性早熟。头颅磁共振成像显示下丘脑 - 垂体区域无可见病变。最可能的假说是在高水化后可能出现脑水肿,继而导致下丘脑 - 垂体和枕叶区域缺血。她虽视力低下但存活下来,并接受了针对神经内分泌缺陷的联合替代治疗。该病例强调,DKA初期的补液至关重要,尤其是存在脑水肿危险因素(年龄小、明显低钠血症)时。急性脑水肿的神经内分泌后果罕见,但医生必须对这些意外事件的幸存者保持警惕。