1例控制不良肾病综合征学龄前女童的显性甲状腺功能减退和严重生长迟缓:病例报告及文献复习

Q3 Medicine Acta Medica Lituanica Pub Date : 2023-10-18 DOI:10.15388/amed.2023.30.2.4
Joana Mikulevič, Karolina Tumelienė, Robertas Kemežys, Augustina Jankauskienė
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引用次数: 0

摘要

背景。由于尿中甲状腺激素的排泄,肾病儿童可能出现甲状腺激素功能障碍。与影响约30%肾病综合征(NS)患儿的亚临床甲状腺功能减退相比,本例患者有明显的甲状腺功能减退和严重的生长发育迟缓。临床病例。1例5岁零8个月大的类固醇依赖性NS患者因持续水肿和利尿减少而从另一个中心转诊,自甲状腺功能减退症导致NS发病4个月以来,一直接受霉酚酸酯(MMF) 250 mg / d和l -甲状腺素50 mcg / d治疗。白蛋白12.64 g/l,胆固醇25.64 mmol/l,蛋白尿5 g/l。患者生长发育严重迟缓,身高93.5 cm (-13 cm <3个百分位),体重17.2 kg(15-25个百分位)。她的发病时间超过3年。女孩的生长速度从3.5个月开始放缓。患者接受了高累积剂量的强的松龙治疗(约1。7800毫克(1年8个月)。促甲状腺激素升高(18.04 mU/L), FT4降低(11.43 pmol/ L), IGF-1 <15µg / L。肾脏活检显示疾病变化很小,基因检测为阴性。以甲泼尼龙脉冲疗法强化NS治疗,开始加大MMF剂量和白蛋白输注,l -甲状腺素剂量增加至75 mcg。TPOAb正常(12.65 IU/ml)。3周后出院,无水肿,停用甲泼尼龙治疗后甲状腺功能恢复正常,l -甲状腺素停用。两周后,可乐定标准生长激素刺激试验显示部分生长激素分泌不足。在NS缓解期间,甲状腺功能正常化(TSH 6.680 mU/l, FT4 13.85 pmol/l)和IGF-1水平正常化(132 mcg/l),观察到部分追赶性生长(基于4个月计算期,身高速度从3.5 cm/年增加到7.3 cm/年)。临床医生应意识到发生甲状腺功能减退的风险,并在治疗NS患儿时考虑甲状腺功能检测,同时积极治疗甲状腺功能减退并评估生长。
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Overt Hypothyroidism and Severe Growth Retardation in a Preschool Girl with Poorly Controlled Nephrotic Syndrome: Case Report and Literature Review
Background. Nephrotic children may develop thyroid hormone dysfunction due to urinary excretion of thyroid hormones. In contrast to the subclinical hypothyroidism that affects around 30% of children with nephrotic syndrome (NS), the patient in this case had overt hypothyroidism and severe growth retardation.Clinical case. A 5 years and 8 months old girl with steroid-dependent NS was referred from another center due to persistent edema and decreased diuresis, being treated with mycophenolate mofetil (MMF) 250 mg once a day and L-thyroxine 50 mcg daily since 4 months of NS onset because of hypothyroidism. Her albumin was 12.64 g/l, cholesterol 25.64 mmol/l and proteinuria 5 g/l. Severe growth retardation was observed: patient’s height was 93.5 cm (–13 cm <3 percentile), weighted 17.2 kg (15–25 percentile). Her disease vintage was over 3 years. Girl’s growth velocity has slowed down from 3.5 months. The patient received a high cumulative dose of prednisolone (approx. 7800 mg in 1 year and 8 months). Thyroid-stimulating hormone was higher (18.04 mU/L) with reduced FT4 11.43 pmol/l and IGF-1 < 15 µg/L. Kidney biopsy revealed minimal change disease, and genetic testing was negative. Intensive NS treatment with methylprednisolone pulse therapy, enlarged doses of MMF and albumin infusion were started and L-thyroxine dose was increased to 75 mcg. TPOAb was in normal range (12.65 IU/ml). After 3 weeks she was discharged with no edema and after stopping methylprednisolone treatment thyroid function normalized and L-thyroxin was discontinued. Two weeks later standard growth hormone stimulation test with clonidine showed partially insufficient growth hormone secretion. During NS remission with normalization of thyroid function (TSH 6.680 mU/l, FT4 13.85 pmol/l) and normalization of IGF-1 level (132 mcg/l) partial catch-up growth was observed (height velocity increased from 3.5 cm/year to 7.3 cm/year, based on 4-month calculation period).Conclusions. Clinicians should be aware of a risk of developing hypothyroidism and consider thyroid function testing during the treatment of children with NS, as well as actively treat hypothyroidism and evaluate growth.
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来源期刊
Acta Medica Lituanica
Acta Medica Lituanica Medicine-General Medicine
CiteScore
0.70
自引率
0.00%
发文量
33
审稿时长
16 weeks
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