Acute kidney injury as the onset of cystic fibrosis in a child of 1 year of age

A. I. Safina, T. V. Mikhailova, I. I. Zakirov, I. M. Suleimanova
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Abstract

Early diagnosis of the cystic fibrosis remains an urgent problem, despite ongoing nationwide neonatal screening. The uniqueness of the presented clinical case lies in the atypical onset of cystic fibrosis in a young child with clinical manifestations of acute kidney injury. The suddenly developed severe general condition in a child was due to manifestations of acute renal injury of the third stage (severe lethargy, anuria for 14 hours, an increase in blood creatinine to 121 µmol/L, a decrease in glomerular filtration rate to 17.4 mL/min), decompensated metabolic alkalosis with hypokalemia, hyponatremia, hypocalcemia (pH 7.6, K+ 2.6 mmol/L, Na+ 118 mmol/L, Ca2+ 0.96 mmol/L, HCO3 – 35.5 mmol/L). The observed disturbances in acid-base status and electrolyte metabolism were manifestations of the pseudo-Bartter syndrome. For the prevention of acute kidney injury in a patient with cystic fibrosis, timely electrolyte and fluid management is important to prevent the development of pseudo-Bartter’s syndrome, exsicosis and hypovolemia.
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一岁儿童因囊性纤维化引发急性肾损伤
尽管目前正在全国范围内开展新生儿筛查,但囊性纤维化的早期诊断仍然是一个亟待解决的问题。本临床病例的独特之处在于,囊性纤维化非典型地发生在一名伴有急性肾损伤临床表现的幼儿身上。患儿突然出现的严重全身状况是由于急性肾损伤第三阶段的表现(严重嗜睡、无尿 14 小时、血肌酐升高至 121 µmol/L、肾小球滤过率下降至 17.4 毫升/分钟),失代偿性碱中毒,伴有低钾血症、低钠血症和低钙血症(pH 值 7.6,K+ 2.6 毫摩尔/升,Na+ 118 毫摩尔/升,Ca2+ 0.96 毫摩尔/升,HCO3 - 35.5 毫摩尔/升)。观察到的酸碱状态和电解质代谢紊乱是假性巴特综合征的表现。为预防囊性纤维化患者急性肾损伤,及时处理电解质和液体对预防假性巴特综合征、外渗和低血容量的发生非常重要。
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