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Nefrologia 2021 Apr 23. Epub 2021 Apr 23.

Department of Pediatrics, Universidad de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina.

Two types of early-childhood hyperkalemia had been recognized, according to the presence or absence of urinary salt wasting. This condition was attributed to a maturation disorder of aldosterone receptors and is characterized by sustained hyperkalemia, hyperchloremic metabolic acidosis due to reduced ammonium urinary excretion and bicarbonate loss, and normal creatinine with growth delay. We present three patients of the type without salt wasting, which we will call transient early-childhood hyperkalemia without salt wasting, and discuss its physiopathology according to new insights into sodium and potassium handling by the aldosterone in distal nephron. Read More

Dtsch Med Wochenschr 2021 Apr 14;146(8):525-529. Epub 2021 Apr 14.

Fluid therapy is one of the basic and most frequently performed medical therapies in everyday clinical practice. However, optimal volume management is a challenge: the application is simple, but the whole volume management is a complex process and physicians have to pay attention on underlying pathophysiology. Intravenous fluids should be prescribed like medications, i. Read More

Eur J Hum Genet 2021 Apr 12. Epub 2021 Apr 12.

Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

KCNJ16 encodes K5.1 and acts in combination with K4.1, encoded by KCNJ10, to form an inwardly rectifying K channel expressed at the basolateral membrane of epithelial cells in the distal nephron. Read More

Front Endocrinol (Lausanne) 2021 17;12:652045. Epub 2021 Mar 17.

Centro Ipertensione Arteriosa e Studio Malattie Cardiorenali, S.S. Fisiopatologia Medica, Clinica Medica Generale e Terapia Medica, Parma, Italy.

Background: Pheochromocytoma (PHEO) clinical manifestations generally mirror excessive catecholamines secretion; rarely the clinical picture may reflect secretion of other hormones. Watery diarrhea, hypokalemia and achlorhydria (WDHA) is a rare syndrome related to excessive secretion of vasoactive intestinal peptide (VIP).

Clinical Case: A 73-year-old hypotensive man affected by adrenal PHEO presented with weight loss and watery diarrhea associated with hypokalemia, hyperchloremic metabolic acidosis (anion gap 15 mmol/l) and a negative urinary anion gap. Read More

BMC Nephrol 2021 Mar 26;22(1):111. Epub 2021 Mar 26.

University Clinic in Nephrology and Hypertension and University of Aarhus, Gødstrup Hospital, Laegaardvej 12, 7500, Holstebro, Denmark.

Background: Isotonic saline (IS) is widely used to secure perioperative cardiovascular stability. However, the high amount of chloride in IS can induce hyperchloremic acidosis. Therefore, IS is suspected to increase the risk of acute kidney injury (AKI). Read More

J Emerg Med 2021 Feb 16. Epub 2021 Feb 16.

Department of Emergency Medicine, Shuang-Ho Hospital, Taipei Medical University, New Taipei City, Taiwan.

Background: Acetazolamide is contraindicated in patients undergoing dialysis and should be used with caution in patients with chronic kidney disease (CKD). Here, we evaluate the effect of the concomitant use of aspirin by patient with CKD using acetazolamide.

Case Report: A 63-year-old man with CKD and multimorbidity presented at our Emergency Department (ED) with general weakness and dyspnea for 4 days. Read More

Cureus 2021 Jan 18;13(1):e12765. Epub 2021 Jan 18.

Diabetes and Endocrinology, Peterborough City Hospital, Peterborough, GBR.

Normal anion gap (non-gap) hyperchloremic acidosis with hypokalemia is a medical emergency. There are several causes of this metabolic phenomenon, of which distal renal tubular acidosis is among the very rare causes. In this report, we present an unusual case of a previously healthy woman who was admitted to the intensive care unit with a short history of severe muscle weakness. Read More

Cureus 2020 Dec 17;12(12):e12132. Epub 2020 Dec 17.

Internal Medicine, Creighton University, Omaha, USA.

Radiation cystitis can present as gross hematuria and occurs secondary to irritation of the bladder urothelium. Continuous bladder irrigation (CBI) is commonly used for the treatment of hemorrhagic cystitis for evacuation of blood clots and to maintain catheter drainage. Most commonly, CBI is performed using 0. Read More

Nefrologia 2020 Dec 29. Epub 2020 Dec 29.

Instituto de Investigación Sanitaria Biocruces Bizkaia, Barakaldo, Bizkaia, España; CIBERDEM, CIBERER, Endo-ERN; Sección de Nefrología Pediátrica, Hospital Universitario Cruces, Barakaldo, Bizkaia, España; Departamento de Pediatría, Universidad del País Vasco UPV/EHU, Barakaldo, Bizkaia, España. Electronic address:

Distal renal tubular acidosis (DRTA) is a rare disease resulting from a failure in the normal urine acidification process at the distal tubule and collecting duct level. It is characterised by persistent hyperchloremic metabolic acidosis, with a normal anion gap in plasma, in the presence of high urinary pH and low urinary excretion of ammonium. To date, 5 genes whose mutations give rise to primary DRTA have been described. Read More

Clin Neurol Neurosurg 2021 Feb 15;201:106435. Epub 2020 Dec 15.

Albany Medical Center, Albany, NY, 12208, United States. Electronic address:

Background: 3 % hypertonic saline (HS) is a hyperosmolar agent often used to treat elevated intracranial pressure (ICP). However, the resultant hyperchloremia is associated with adverse outcomes in certain patient populations. In this study, HS solution buffered with sodium acetate (HSwSA) is used as an alternative to standard 3 % formulations to reduce overall chloride exposure. Read More

Perioper Med (Lond) 2020 Dec 10;9(1):40. Epub 2020 Dec 10.

Department of Anesthesiology, Resuscitation and Intensive Care Medicine, University Hospital Hradec Kralove, Sokolská 581, 500 05, Hradec Kralove, Czech Republic.

Background: This systematic review discusses a clinical physiology aspect of chloride in fluid therapy. Crystalloid solutions are one of the most widely used remedies. While generally used in medicine for almost 190 years, studies focused largely on their safety have only been published since the new millennium. Read More

JAMA Netw Open 2020 11 2;3(11):e2024596. Epub 2020 Nov 2.

Division of Allergy, Pulmonary, and Critical Care Medicine, Vanderbilt University Medical Center, Nashville, Tennessee.

Importance: Saline (0.9% sodium chloride), the fluid most commonly used to treat diabetic ketoacidosis (DKA), can cause hyperchloremic metabolic acidosis. Balanced crystalloids, an alternative class of fluids for volume expansion, do not cause acidosis and, therefore, may lead to faster resolution of DKA than saline. Read More

Am J Case Rep 2020 Nov 13;21:e926357. Epub 2020 Nov 13.

Department of Orthopedics, College of Medicine, King Saud Bin Abdulaziz University of Health Sciences, Riyadh, Saudi Arabia.

BACKGROUND Airway compromise caused by massive fluid extravasation in association with arthroscopic shoulder surgery is rare. However, it is even more rare to occur as a result of pleural effusion. We present this case to increase the awareness of this rare complication and show how to minimize the likelihood of this uncommon incident. Read More

Medicine (Baltimore) 2020 Oct;99(40):e21987

Ministry of Education Key Laboratory of Child Development and Disorders.

Intravenous fluid prescription is an essential part of postoperative care and may play a causal role in postoperative complications. The objective of the present study was to evaluate the relationship between intraoperative fluid administration and postoperative outcomes in a large cohort of pediatric patients.This analysis included a retrospective review of 172 patients who underwent gastroenterological surgery from January 2012 to September 2018 at an academic tertiary care hospital. Read More

J Clin Invest 2020 12;130(12):6379-6394

Université de Paris, INSERM, PARCC, F-75006, Paris, France.

Gain-of-function mutations in with no lysine (K) 1 (WNK1) and WNK4 genes are responsible for familial hyperkalemic hypertension (FHHt), a rare, inherited disorder characterized by arterial hypertension and hyperkalemia with metabolic acidosis. More recently, FHHt-causing mutations in the Kelch-like 3-Cullin 3 (KLHL3-CUL3) E3 ubiquitin ligase complex have shed light on the importance of WNK's cellular degradation on renal ion transport. Using full exome sequencing for a 4-generation family and then targeted sequencing in other suspected cases, we have identified new missense variants in the WNK1 gene clustering in the short conserved acidic motif known to interact with the KLHL3-CUL3 ubiquitin complex. Read More

Case Rep Endocrinol 2020 23;2020:1635413. Epub 2020 Jul 23.

Department of Endocrinology and Metabolism, Adrenal Center, West China Hospital of Sichuan University, Chengdu, Sichuan 610041, China.

This study reported on a 24-year-old woman who complained of a paroxysmal headache for six months and elevated blood pressure for four months. Laboratory examination revealed increased serum potassium and chloride levels, metabolic acidosis, suppressed renin activity, and increased plasma aldosterone concentration. Whole-exome sequencing revealed a heterozygous mutation in exon 11 of the KLHL3 gene: c. Read More

Clin Exp Nephrol 2020 Dec 9;24(12):1140-1143. Epub 2020 Aug 9.

Dialysis Unit, Shin-Kuki General Hospital, Saitama, Japan.

Background: Amelioration of hyperchloremic acidosis (Cl-Ac), a common complication in chronic kidney disease (CKD), could preserve renal function in chronic kidney disease (CKD). However, the development of Cl-Ac in CKD has not been clarified yet.

Methods: The degree of Cl-Ac, which is indicated as the bicarbonate concentration decrease with serum chloride concentration increase (∆[HCO]), was compared with the estimated glomerular filtration rate (eGFR) by using CKD patient records. Read More

Clin Nephrol 2020 Sep;94(3):150-154

Hashimoto's thyroiditis (HT) is an autoimmune disease that can cause the dysfunction of glands. Moreover, autoimmune disease is an under-recognized cause of several types of tubular dysfunction such as renal tubular acidosis (RTA), Gitelman's syndrome (GS), and Bartter's syndrome (BS). However, the potential mechanism of acquired BS and RTA associated with autoimmune diseases remains unclear. Read More

Kidney Blood Press Res 2020 14;45(4):523-531. Epub 2020 Jul 14.

Zentrum Innere Medizin 1, Kardiologie, Angiologie, Nephrologie, Klinikum Brandenburg, Medizinische Hochschule Brandenburg, Brandenburg an der Havel, Germany,

Background: The etiology of acute metabolic acidosis (aMA) is heterogeneous, and the consequences are potentially life-threatening. The aim of this article was to summarize the causes and management of aMA from a clinician's perspective.

Summary: We performed a systematic search on PubMed, applying the following search terms: "acute metabolic acidosis," "lactic acidosis," "metformin" AND "acidosis," "unbalanced solutions" AND "acidosis," "bicarbonate" AND "acidosis" AND "outcome," "acute metabolic acidosis" AND "management," and "acute metabolic acidosis" AND "renal replacement therapy (RRT)/dialysis. Read More

Nephrology (Carlton) 2020 Nov 10;25(11):818-821. Epub 2020 Aug 10.

Department of Pediatrics, Fujieda Municipal General Hospital, Fujieda, Japan.

Pseudohypoaldosteronism type II (PHA II) is inherited in an autosomal dominant manner and is characterized by hypertension, hyperkalemia, and hyperchloremic metabolic acidosis. The enhancement of with-no-lysine kinase (WNK) functions is correlated to the pathogenesis of the condition. Cullin 3 (CUL3) forms an E3 ubiquitin ligase complex, and it can ubiquitinate WNK. Read More

Indian J Pediatr 2020 09 26;87(9):733-744. Epub 2020 Jun 26.

Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, 110029, India.

Renal tubular acidosis (RTA) comprises a group of disorders characterized by low capacity for net acid excretion and persistent hyperchloremic metabolic acidosis, despite preserved glomerular filtration rate. RTA are classified into chiefly three types (1, 2 and 4) based on pathophysiology and clinical and laboratory characteristics. Most patients have primary RTA that presents in infancy with polyuria, growth retardation, rickets and/or hypotonia. Read More

Intensive Care Med Exp 2020 Jun 23;8(1):25. Epub 2020 Jun 23.

School of Medicine, University of Nottingham, Nottingham, UK.

Purpose: Plasma-Lyte is a balanced, crystalloid intravenous fluid which has been shown to avoid the hyperchloremic metabolic acidosis associated with 0.9% sodium chloride. Data on physical, pH and chemical compatibility with other medicines are essential. Read More

Anasthesiol Intensivmed Notfallmed Schmerzther 2020 May 20;55(5):324-333. Epub 2020 May 20.

The composition and type of intravenous fluids during paediatric anaesthesia have been subjects of debates for decades. Errors in perioperative infusion therapy in children may lead to serious complications and a negative outcome. Therefore, in this review historical and recent developments and recommendations for perioperative fluid management in children are presented, based on physiology and focused on safety and efficacy. Read More

J Bras Nefrol 2020 Jul-Sep;42(3):380-383

Universidade Federal do Amazonas, Hospital Universitário Getúlio Vargas, Manaus, AM, Brasil.

We report an unusual case of a 24-year-old girl with a history of recurrent hypokalemic paralysis episodes and skin lesions on the lower limbs and buttocks, both of which had an acute evolution. In subsequent investigations, the patient also had nephrocalcinosis, nephrolithiasis, hyperchloremic metabolic acidosis and persistent alkaline urinary pH. The findings were consistent with distal renal tubular acidosis as the cause of hypokalemic paralysis. Read More

Int J Clin Pharmacol Ther 2020 May;58(5):261-267

Objective: Respiratory alkalosis (RA) and dilutional hyperchloremic acidosis (DHA) are the most common acid-base balance (ABB) disorders in patients with liver cirrhosis. The aims of this study were to clarify whether RA develops in relation to DHA via respiratory compensation of metabolic acidosis and whether spironolactone in combination with low-dose furosemide - diuretics known to ameliorate DHA - positively affects RA in liver cirrhosis patients.

Materials And Methods: 59 patients with advanced cirrhosis were divided into two groups. Read More

Afr J Emerg Med 2020 Mar 17;10(1):46-47. Epub 2019 Nov 17.

Emergency & ICU Department, Shree Hindu Mandal Hospital, Dar es Salaam, Tanzania.

Introduction: Diabetic ketoacidosis (DKA) often becomes the primary focus and in turn masks a similar serious condition like hyperchloremic metabolic acidosis.

Case Report: A 20 years old female with type 1 diabetes mellitus presented to the emergency department (ED) with signs and symptoms corresponding to DKA. Initial pH, HCO Na and Cl concentrations were 6. Read More

Can J Anaesth 2020 04 27;67(4):421-429. Epub 2020 Jan 27.

Department of Anesthesia and Critical Care, Pontchaillou, University Hospital of Rennes, Rennes, France.

Purpose: Isotonic 0.9% sodium chloride (normal saline; NS) solution use is common, but its high chloride content has been shown to contribute to acid-base disturbances and acute kidney injury (AKI). As kidney transplant recipients are at high risk of postoperative AKI and renal replacement therapy, we aimed to evaluate the impact of perioperative NS administration on graft function after kidney transplantation. Read More

Anesthesiology 2020 04;132(4):614-624

From the Department of General Anesthesiology (K.M., A.T., W.A.S.E., K.R., S.B., A.G.K., M.R.R., T.K., G.R.B., A.K.) Department of Outcomes Research (K.M., A.T., N.M., C.M., K.R., H.E., B.C., I.S., G.R.B., D.C., E.J.M., A.K., D.I.S.) Department of Quantitative Health Sciences (N.M., C.M., E.J.M.) Department of Orthopedic Surgery (C.H.-R.), Cleveland Clinic, Cleveland, Ohio the Division of Colon and Rectal Surgery, Mayo Clinic, Jacksonville, Florida (L.S.) the Division of Anesthesia, Critical Care, and Pain Management, Tel-Aviv Medical Center, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel (B.C.) the Department of Anesthesia, St. Elizabeth's Medical Center, Boston, Massachusetts (I.S.).

Background: Both saline and lactated Ringer's solutions are commonly given to surgical patients. However, hyperchloremic acidosis consequent to saline administration may provoke complications. The authors therefore tested the primary hypothesis that a composite of in-hospital mortality and major postoperative complications is less common in patients given lactated Ringer's solution than normal saline. Read More

Kidney Int 2020 03 22;97(3):567-579. Epub 2019 Oct 22.

Division of Nephrology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. Electronic address:

Distal renal tubular acidosis is a rare renal tubular disorder characterized by hyperchloremic metabolic acidosis and impaired urinary acidification. Mutations in three genes (ATP6V0A4, ATP6V1B1 and SLC4A1) constitute a monogenic causation in 58-70% of familial cases of distal renal tubular acidosis. Recently, mutations in FOXI1 have been identified as an additional cause. Read More

Saudi J Kidney Dis Transpl 2019 Nov-Dec;30(6):1442-1446

Department of Nephrology, Madras Medical Mission Hospital, Mogappair, Chennai, Tamil Nadu, India.

Homozygous autosomal recessive distal renal tubular acidosis (dRTA) is a rare entity. The intercalated cells in the collecting ducts are defective in apical proton secretion or basolateral bicarbonate reabsorption, due to mutations in genes encoding for proteins in a4 and B1 subunits of the V-ATPase and the anion exchanger Cl/HCO (kAE1). This results in decreased ammonium (NH) excretion and defective urine acidification. Read More