Chukwuedozie Vivian C, Garg Tulika, Chaudhry Hassan A, Shawl Saima H, Mishra Priya, Adaralegbe Ngozi J, Khan Aadil
Internal Medicine, Ebonyi State University Medical School, Abakaliki, NGA.
Medicine, Government Medical College and Hospital, Chandigarh, IND.
Cureus. 2022 Aug 5;14(8):e27695. doi: 10.7759/cureus.27695. eCollection 2022 Aug.
Proximal renal tubular acidosis (type 2 RTA) is a metabolic disorder characterized by an inability of the proximal renal tubules to reabsorb bicarbonate, resulting in excessive urinary loss of bicarbonate. In return, this causes a standard anion gap metabolic acidosis with aberrant renal acidification, culminating in excessive urinary potassium loss and hyperchloremic metabolic acidosis. Several sources can induce potassium deficiency, ranging from slight abnormalities in potassium homeostasis to catastrophic and occasionally lethal circumstances. Hypokalemic periodic paralysis (HPP) manifests with broad muscle weakness and the absence of deep tendon reflexes, with the facial, bulbar, and respiratory muscles spared, and it subsequently requires the administration of intravenous potassium chloride to address the potassium imbalance. Some patients suffering from chronic potassium shortage may have periods of weakness. The clinical symptoms of distal RTA are identical to those of attacks induced by familial hypokalemic periodic paralysis (FPP). Muscle weakness may begin slowly and worsen over 24-48 hours to flaccid quadriplegia. RTA and FPP typically spare speech, swallowing, and ocular and respiratory muscles. As a result, families with RTA children must be aware of this risk. We present a case of HPP in a female caused by type 2 RTA.
近端肾小管酸中毒(2型肾小管酸中毒)是一种代谢紊乱疾病,其特征为近端肾小管无法重吸收碳酸氢盐,导致碳酸氢盐经尿液大量流失。相应地,这会引发标准阴离子间隙代谢性酸中毒并伴有异常的肾脏酸化,最终导致尿液中钾过度流失以及高氯性代谢性酸中毒。多种因素可导致钾缺乏,从轻微的钾稳态异常到严重甚至偶尔致命的情况都有。低钾性周期性麻痹(HPP)表现为广泛的肌肉无力且无深腱反射,面部、延髓和呼吸肌不受影响,随后需要静脉输注氯化钾来纠正钾失衡。一些慢性钾缺乏患者可能会出现乏力期。远端肾小管酸中毒的临床症状与家族性低钾性周期性麻痹(FPP)发作时的症状相同。肌肉无力可能起病缓慢,在24 - 48小时内加重至弛缓性四肢瘫。肾小管酸中毒和FPP通常不累及言语、吞咽以及眼部和呼吸肌。因此,有患肾小管酸中毒孩子的家庭必须意识到这种风险。我们报告一例由2型肾小管酸中毒引起的女性低钾性周期性麻痹病例。