关键词: Chronic disease / disability nursing Fluid electrolyte and acid-base disturbances

Mesh : Infant, Newborn Female Humans Bartter Syndrome / complications diagnosis drug therapy Hypokalemia / complications Diabetes Mellitus, Type 2 / complications drug therapy Phenotype Potassium / metabolism Indomethacin / therapeutic use Tablets

来  源:   DOI:10.1136/bcr-2023-257953   PDF(Pubmed)

Abstract:
Bartter syndrome (BS) is a rare genetic tubulopathy affecting the loop of Henle leading to salt wasting. It is commonly seen in utero or in the early neonatal period. Rare cases of acquired BS are reported in association with infections like tuberculosis, granulomatous conditions like sarcoidosis, autoimmune diseases and drugs. The mainstay of management includes potassium, calcium and magnesium supplementation. We report the case of a woman in her 50s with a history of type 2 diabetes mellitus for the last 10 years, who presented with diabetic foot ulcers and generalised weakness with ECG changes suggestive of hypokalaemia. She had severe hypokalaemia with high urine potassium excretion and hypochloraemic metabolic alkalosis. She poorly responded to intravenously administered potassium supplements and had persistent hypokalaemia. On further evaluation of the persistent hypokalaemia, a diagnosis of idiopathic Bartter-like phenotype was made. She responded well to tablet indomethacin and is presently asymptomatic and is being maintained on tablet indomethacin after 6 months of follow-up.
摘要:
Bartter综合征(BS)是一种罕见的遗传性肾小管病,影响Henle的循环,导致盐耗。常见于子宫内或新生儿早期。据报道,罕见的获得性BS病例与结核病等感染有关,肉芽肿疾病如结节病,自身免疫性疾病和药物。管理的主体包括钾,补充钙和镁。我们报告了一个50多岁的女性,过去10年有2型糖尿病史,患者出现糖尿病足溃疡和全身性无力,心电图改变提示低钾血症。她患有严重的低钾血症,尿钾排泄高和低氯血症代谢性碱中毒。她对静脉注射钾补充剂的反应不佳,并且患有持续性低钾血症。在进一步评估持续性低钾血症时,诊断为特发性Bartter样表型.她对吲哚美辛片剂反应良好,目前无症状,在随访6个月后仍在使用吲哚美辛片剂。
公众号