Characterization of Demographic, Clinical, and Laboratory Risk Factors for Stroke in a Tertiary Hospital in Riyadh, Saudi Arabia.

Background Stroke is a major cause of death and long-term disability worldwide, with varying incidence and risk factors across different populations. This study aims to analyze demographic, clinical, and laboratory risk factors for stroke among the Saudi Arabian population to enhance the understanding of its behavior and associated mortality. Methods In this retrospective cohort study, we analyzed data from 3586 patients diagnosed with hemorrhagic or non-hemorrhagic stroke at King Fahad Medical City from January 1, 2020, to November 11, 2022. We collected data on demographic variables, past medical history, social history, nationality, and laboratory components. Statistical analyses were performed using IBM SPSS Statistics for Windows, Version 27.0. (Armonk, NY: IBM Corp.), with significance set at p<0.05. Results The study population was predominantly male (57.86%) and within the age group of 51 to 80 years (58.8%). A significant portion of patients were Saudi nationals (99.6%), with hypertension (50.2%) and diabetes (40.4%) being the most common comorbidities. Laboratory abnormalities related to sodium and potassium levels were strongly linked to mortality rates. Notably, ischemic stroke was the most common type across all age groups, except for patients under age 16, where hemorrhagic stroke was more prevalent. Conclusions Our findings reveal significant associations between stroke risk factors and mortality within the Saudi Arabian population, highlighting the impact of hypertension, diabetes, and electrolyte imbalances. The study underscores the need for targeted stroke prevention and management strategies in Saudi Arabia, aligning with global trends to mitigate the burden of this disease.

Can Patients with Electrolyte Disturbances Be Safely and Effectively Treated in a Hospital-at-Home, Telemedicine-Controlled Environment? A Retrospective Analysis of 267 Patients.

BACKGROUND: Morbidities indicated for hospital-at-home (HAH) treatment include infectious diseases and exacerbations of chronic conditions. Electrolyte disturbances are not included per se. However, their rate is high. We aimed to describe our experience via the monitoring and treatment of such patients. METHODS: This was a retrospective analysis of patients in the setting of telemedicine-controlled HAH treatment. We collected data from the electronic medical records of patients who presented electrolyte disturbances. RESULTS: For 14 months, we treated 267 patients in total in HAH settings, with a mean age of 72.2 + 16.4, 44.2% for males. In total, 261 (97.75%) patients were flagged with electrolyte disturbances, of whom 149 had true electrolyte disturbances. Furthermore, 67 cases (44.96%) had hyponatremia, 9 (6.04%) had hypernatremia after correction for hyperglycemia, 20 (13.42%) had hypokalemia and 27 (18.12%) had hyperkalemia after the exclusion of hemolytic samples. Ten (6.09%) patients had hypocalcemia and two (1.34%) had hypercalcemia corrected to albumin levels. Thirteen (8.72%) patients had hypomagnesemia and one (0.67%) had hypermagnesemia. Patients with electrolyte disturbances suffered from more chronic kidney disease (24.2% vs. 12.2%; p = 0.03) and malignancy (6.3% vs. 0.6%; p = 0.006), and were more often treated with diuretics (12.6% vs. 4.1%; p = 0.016). No patient died or suffered from clinically significant cardiac arrhythmias. CONCLUSIONS: The extent of electrolyte disturbances amongst HAH treatment patients is high. The monitoring and treatment of such patients can be conducted safely in this setting.

Risk Factors Asscociated with Hypokalemia during Postanesthesia Recovery and Its Impact on Outcomes in Gynecological Patients: A Propensity Score Matching Study.

OBJECTIVE: This study aimed to explore the risk factors and outcomes of hypokalemia during the recovery period from anesthesia in the gynecological population. METHODS: This retrospective cohort study included 208 patients who underwent gynecological surgery at our institution between January 2021 and March 2022. Data were collected for each patient, including demographics, disease status, surgical data, and clinical information. Preoperative bowel preparation, postoperative gastrointestinal function, and electrolyte levels were compared between the two groups using propensity score matching (PSM). RESULTS: The incidence of hypokalemia (serum potassium level <3.5 mmol/L) during the recovery period from anesthesia was approximately 43.75%. After PSM, oral laxative use (96.4% vs. 82.4%, P=0.005), the number of general enemas (P=0.014), and the rate of ≥2 general enemas (92.9% vs. 77.8%, P=0.004) were identified as risk factors for hypokalemia, which was accompanied by decreased PaCO2 and hypocalcemia. There were no significant differences in postoperative gastrointestinal outcomes, such as the time to first flatus or feces, the I-FEED score (a scoring system was created to evaluate impaired postoperative gastrointestinal function), or postoperative recovery outcomes, between the hypokalemia group and the normal serum potassium group. CONCLUSION: Hypokalemia during postanesthesia recovery period occurred in 43.75% of gynecological patients, which resulted from preoperative mechanical bowel preparation; however, it did not directly affect clinical outcomes, including postoperative gastrointestinal function, postoperative complications, and length of hospital stay.

A Case of Intractable Hypophosphatemia in a Patient with Guillain-Barré Syndrome and Encephalitis after SARS-CoV-2 Infection.

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection attacks the respiratory and nervous systems. Among patients with SARS-CoV-2 infection, cases with simultaneous central and peripheral nervous system damage are rare, and those with intractable hypophosphatemia and hypokalemia complicating the former have not been reported yet. CASE PRESENTATION: A 59-year-old woman presented to the emergency department with incoherent speech evolving for 3 days. She had tested positive for the SARS-CoV-2 RT-PCR assay 8 days earlier. Her physical examination showed progressive limb weakness with diminished tendon reflexes and normal sensory examination. Cranial MRI revealed multiple abnormal signals in the brain. Cerebrospinal fluid (CSF) analysis and electromyography revealed acute motor axonal neuropathy (AMAN), further diagnosed as encephalitis combined with Guillain­Barré syndrome (GBS). The patient received glucocorticoid therapy, intravenous immune globulin (IVIG), and rehabilitation therapy. The patient experienced an intractable hypophosphatemia and hypokalemia during the treatment period, which was not effectively corrected several times. The symptoms improved after 1 month of treatment. CONCLUSION: Early diagnosis is important for the management of Guillain-Barré syndrome associated with SARS-CoV-2 infection. Moreover, in order to prevent life-threatening long-term persistent electrolyte disturbances in non-seriously ill patients, clinicians should pay particular attention to their electrolyte status.

The impact of tubular dysfunction and its relationship with acute kidney injury in children.

BACKGROUND: Tubular dysfunction can cause electrolyte disturbances with potentially serious consequences. We studied the epidemiology and outcomes of electrolyte disturbances and tubular dysfunction among critically ill children and evaluated their relationships with acute kidney injury (AKI). METHODS: We conducted a prospective cohort study recruiting children aged 1 month to ≤ 18 years old admitted to the pediatric intensive care unit (PICU) from 6/2020 to 6/2021. The serum levels of sodium, potassium, calcium, phosphate, and magnesium were reviewed and simultaneous urinary investigations for tubular function were performed among children with electrolyte disturbances. RESULTS: Altogether there were 253 episodes of admission. The median (interquartile) age was 4.9 (1.3-11.0) years and 58.1% were male. The median number of electrolyte disorders was 3 (2-4) types. Hypophosphatemia (74.2%), hypocalcemia (70.3%) and hypermagnesemia (52.9%) were the three commonest types of disturbances. Urinary electrolyte wasting was commonly observed among children with hypomagnesemia (70.6%), hypophosphatemia (67.4%) and hypokalemia (28.6%). Tubular dysfunction was detected in 82.6% of patients and urinary ß2-microglobulin level significantly correlated with the severity of tubular dysfunction (p < 0.001). The development of tubular dysfunction was independent of AKI status. Tubular dysfunction was associated with mortality (p < 0.001) and was an independent predictor of PICU length of stay (LOS) (p < 0.001). The incorporation of the tubular dysfunction severity into the AKI staging system improved the prediction of PICU LOS. CONCLUSIONS: Tubular dysfunction was associated with both morbidity and mortality in critically ill children and its assessment may help to capture a more comprehensive picture of acute kidney insult.

Rebound hyperkalemia in a dog with albuterol toxicosis after cessation of potassium supplementation.

OBJECTIVE: To describe the presentation of rebound hyperkalemia as a delayed side effect of albuterol toxicity in a dog. CASE SUMMARY: A 3-year-old female neutered mixed-breed dog was presented for albuterol toxicosis that led to a severe hypokalemia, hyperlactatemia, and hyperglycemia. The dog also experienced sinus tachycardia and generalized weakness. Treatment was instituted with intravenous fluid therapy and potassium supplementation, and the dog was monitored with a continuous electrocardiogram. Resolution of hypokalemia was documented 12 hours after initial presentation, at which time fluid therapy and potassium supplementation were discontinued. There were no further periods of sinus tachycardia, but instead the dog developed ventricular ectopy with rapid couplets (instantaneous rates of 300/min). An echocardiogram revealed normal cardiac size and function. Twenty-four hours after presentation, the patient developed severe hyperkalemia, despite discontinuation of fluids and potassium supplementation for 12 hours. Serial venous and urinary electrolytes were performed for determination of the fractional excretion of electrolytes. These data confirmed rebound hyperkalemia (7.0 mmol/L), consistent with a markedly increased fractional excretion of potassium, and secondary to the release of potassium from inside the cells. Fluid therapy with dextrose supplementation was provided until 36 hours postpresentation. The hyperkalemia resolved, and the dog was discharged after 44 hours of hospitalization. NEW OR UNIQUE INFORMATION PROVIDED: This case documents rebound hyperkalemia following treatment of albuterol toxicosis in a dog. This case highlights the importance of understanding the distribution of total body potassium when treating serum hypokalemia. Transcellular shifts of potassium, as in the case of albuterol toxicosis, can lead to rebound hyperkalemia even after discontinuation of potassium supplementation. This case further explores the utility of fractional excretion of electrolytes in elucidating the etiology and management of electrolyte disturbances.

A Rare Case of Visual Hallucinations Associated With Hyponatremia.

Visual hallucinations are rare occurrences in patients presenting with hyponatremia. When they occur, the patient experiences these hallucinations with their eyes closed (vs. opened) and is insightful about the false perception. We present the case of a 64-year-old male diagnosed with hyponatremia caused by a gastrointestinal illness, which led to visual hallucinations. The patient was treated with electrolyte infusion, and the hallucinations were resolved. Detailed history-taking is significant when dealing with such cases as hallucinations to differentiate it from other causes. Hallucination caused by hyponatremia can resolve with prompt correction of sodium, and the patients can be reassured.

Six-Lead Electrocardiography Enables Identification of Rhythm and Conduction Anomalies of Patients in the Telemedicine-Based, Hospital-at-Home Setting: A Prospective Validation Study.

BACKGROUND: The hospital-at-home (HAH) model is a viable alternative for conventional in-hospital stays worldwide. Serum electrolyte abnormalities are common in acute patients, especially in those with many comorbidities. Pathologic changes in cardiac electrophysiology pose a potential risk during HAH stays. Periodical electrocardiogram (ECG) tracing is therefore advised, but few studies have evaluated the accuracy and efficiency of compact, self-activated ECG devices in HAH settings. This study aimed to evaluate the reliability of such a device in comparison with a standard 12-lead ECG. METHODS: We prospectively recruited consecutive patients admitted to the Sheba Beyond Virtual Hospital, in the HAH department, during a 3-month duration. Each patient underwent a 12-lead ECG recording using the legacy device and a consecutive recording by a compact six-lead device. Baseline patient characteristics during hospitalization were collected. The level of agreement between devices was measured by Cohen's kappa coefficient for inter-rater reliability (Ϗ). RESULTS: Fifty patients were included in the study (median age 80 years, IQR 14). In total, 26 (52%) had electrolyte disturbances. Abnormal D-dimer values were observed in 33 (66%) patients, and 12 (24%) patients had elevated troponin values. We found a level of 94.5% raw agreement between devices with regards to nine of the options included in the automatic read-out of the legacy device. The calculated Ϗ was 0.72, classified as a substantial consensus. The rate of raw consensus regarding the ECG intervals' measurement (PR, RR, and QT) was 78.5%, and the calculated Ϗ was 0.42, corresponding to a moderate level of agreement. CONCLUSION: This is the first report to our knowledge regarding the feasibility of using a compact, six-lead ECG device in the setting of an HAH to be safe and bearing satisfying agreement level with a legacy, 12-lead ECG device, enabling quick, accessible arrythmia detection in this setting. Our findings bear a promise to the future development of telemedicine-based hospital-at-home methodology.

Tumor Genesis Syndrome Presenting as Severe Hypophosphatemia in a Patient With T-Cell Acute Lymphoblastic Leukemia.

Tumor lysis syndrome (TLS) is a medical emergency that can develop in leukemias and lymphomas as a first presentation or after the initiation of anti-neoplastic regimens. On the other hand, tumor genesis syndrome (TGS) is a rare condition associated with certain malignancies, especially those with a high neoplastic burden characterized by rapid proliferation, leading to avid uptake of phosphorus from the serum and culminating in hypophosphatemia. Interestingly, a combination of TLS and TGS can occur simultaneously in a subset of patients. This leads to the development of hypophosphatemia instead of the hyperphosphatemia commonly associated with TLS. We herein present a case of severe asymptomatic hypophosphatemia in a patient with an incidental finding of T-cell acute lymphoblastic leukemia. The patient was initially diagnosed with TLS with hypophosphatemia, but further investigation revealed that the patient had isolated TGS.

Hypophosphatemia in Patients With Multiple Myeloma.

Hypophosphatemia is among the most common electrolyte abnormalities, especially among patients with underlying malignancies, and is frequently associated with adverse prognoses. Phosphorus levels are regulated through a number of mechanisms, including parathyroid hormone (PTH), fibroblast growth factor-23 (FGF-23), vitamin D, and other electrolyte levels themselves. Clinically, the findings are nonspecific, and the diagnosis is frequently delayed. This article is a narrative literature review. The PubMed database was searched for relevant articles pertaining to hypophosphatemia causes and consequences in patients suffering from multiple myeloma. We found a variety of causes of hypophosphatemia in patients with multiple myeloma. Tumor-induced osteopenia, although more common among patients with small squamous cell carcinomas, can occur with multiple myeloma as well. Additionally, both light chains themselves and medications can trigger Fanconi syndrome, which leads to phosphorus wasting by the kidney. Bisphosphonates, in addition to being a possible cause of Fanconi syndrome, lead to a decrease in calcium levels, which then stimulates parathyroid hormone (PTH) release, predisposing the patient to significant hypophosphatemia. Additionally, many of the more modern medications used to manage multiple myeloma have been associated with hypophosphatemia. A better understanding of those mechanisms may give clinicians a clearer idea of which patients may need more frequent screening as well as what the potential triggers in the individual patient may be.

Associations of hypomagnesemia in patients seeking a first treatment of alcohol use disorder.

INTRODUCTION: Hypomagnesemia (hypoMg) has not yet been extensively studied in alcohol use disorder (AUD) . We hypothesize that chronic, excessive alcohol consumption favors oxidative stress and pro-inflammatory alterations that may be exacerbated by hypoMg. The objective of this study was to analyze the prevalence and associations of hypoMg in AUD. PATIENTS AND METHODS: Cross-sectional study in patients admitted for a first treatment of AUD in six tertiary centers between 2013 and 2020. Socio-demographic, alcohol use characteristics, and blood parameters were ascertained at admission. RESULTS: 753 patients (71% men) were eligible; age at admission was 48 years [IQR, 41-56 years]. Prevalence of hypoMg was 11.2%, higher than that observed for hypocalcemia (9.3%), hyponatremia (5.6%), and hypokalemia (2.8%). HypoMg was associated with older age, longer duration of AUD, anemia, higher erythrocyte sedimentation rate, gamma-glutamyl transpeptidase, glucose levels, advanced liver fibrosis (FIB-4 ≥3.25) and estimated glomerular filtration rate (eGFR) < 60 mL/min. In multivariate analysis, advanced liver fibrosis (OR, 8.91; 95% CI, 3.3-23.9) and eGFR < 60 mL (OR, 5.2; 95% CI, 1.0-26.2) were the only factors associated with hypoMg. CONCLUSIONS: Mg deficiency in AUD is associated with liver damage and glomerular dysfunction suggesting that both comorbidities should be assessed in the course of serum hypoMg.

A Five-Month-Old Boy With Hypotonia, Electrolyte Derangements, and Failure to Thrive.

Failure to thrive in the setting of profound hypotonia and multiple electrolyte derangements is a challenging constellation of findings that offers a broad differential diagnosis for providers to consider. Initial management should focus on the stabilization of the patient and correction of potential life-threatening electrolyte derangements. Once completed, the diagnosis should be sought, and in this case, many were considered and ultimately ruled out with thorough history and physical examination. Laboratory abnormalities revealed the final diagnosis of pseudohypoaldosteronism and connected the case. With proper treatment, our patient had a resolution of laboratory anomalies along with improved growth and tone.

Total Fluid Administration and Weight Loss during the First 2 Weeks in Infants Randomized to Early Enteral Feeding after Extremely Preterm Birth.

BACKGROUND: Randomized trials have not reported the effects of the early progression of feeding volumes on fluid balance and neurodevelopment among infants born extremely preterm (≤28 weeks). METHOD: Fluid, electrolyte, and neurodevelopment data of 60 extremely preterm infants randomly assigned to receive either 1 (early feeding group) or 4 days (late feeding group) of trophic feeding volumes at 20-24 mL/kg/day were analyzed. RESULTS: Infants randomized to the early feeding group received less parenteral fluids, generated lower urine volumes, and had less excessive weight loss during the first 14 days after birth. The 7-point difference in cognitive scores and the 0.5 difference in weight-for-age z-scores favoring the early feeding group did not reach statistical significance. CONCLUSIONS: In extremely preterm infants, early enteral feeding is associated with less total fluid administration and with less excessive weight loss during the first 2 weeks after birth. These short-term effects could have long-lasting benefits.

Ascending Flaccid Paralysis Secondary to Hypokalemia in A Cancer Patient using Abiraterone - A Case Report.

BACKGROUND: Prostate cancer (PC) is the most common type of neoplasm in men and the fourth leading cause of mortality in Brazil. The prostate cancer refractory metastatic castration can be treated with abiraterone acetate (AA). CASE PRESENTATION: Its use has been associated with increased survival. However, there are also side effects associated with the use of this drug, such as severe electrolyte disturbances. CONCLUSION: The objective is to report the clinical case of a patient with castration-resistant metastatic prostate cancer who developed ascending flaccid paralysis secondary to severe hypokalemia, probably due to hyperaldosteronism secondary to the use of Abiraterone Acetate, despite the use of Prednisone.

Electrolyte disturbances in a sample of hospitalized patients from Iraq.

Electrolyte disturbances are common in ill patients. Several conditions in the intensive care unit (ICU) might be responsible for developing electrolyte disorders, and medications may also contribute to these disturbances. The current study aimed to determine the frequency of electrolyte disturbances and assess the pattern of electrolyte imbalance in hospitalized patients, determining the possible effects of these electrolyte disorders. This cross-sectional study included patients admitted to the intensive care unit, respiratory care unit (RCU), and coronary care unit (CCU) at the Al-Sadar teaching hospital, Najaf, Iraq, from November 2020 to April 2021. The study collected data from two hundred patients regarding demographics, categories of ICUs at admission, comorbidities, and laboratory values at admission. Also, electrolyte levels at ICU admission and during hospitalization were collected from the medical database record. In addition, the patient's age, sex, fasting blood sugar (FBS), body mass index (BMI), B.urea, and creatinine were matched. Na+, K+, ionized Ca++, and Cl serum levels were significantly different during hospitalization. Comorbidities with predominant hypokalemia were found in 80.5%, hypochloremia in 73%, hypocalcaemia in 72%, and hyponatremia in 56.7% of hospitalized patients. Studying the effect of co-morbidities indicated a higher percentage (44%) of admitted patients with ischemic heart diseases, 38 (19%) with digestive diseases, 21 (10.5%) with orthopedic surgery in an emergency, 14 (7%) with pneumonia and lung diseases, 12 (6%) with diabetics, 18 (9%) with sepsis, and 9(4.5%) with seizure. Hospitalized patients may be at higher risk of developing combined electrolytes disorder associated with decreased serum levels of K+, Na+, Ca++, and Cl-. Thus, doctors and clinicians are recommended to observe electrolyte changes and correct them as they seem to negatively impact the outcome and prognosis.

Acute Kidney Injury in the Inpatient and Outpatient Setting.

The incidence and prevalence of inpatient acute kidney injury is continuing to increase. Acute kidney injury (AKI) is an all-encompassing topic in renal care as well as critical care. The definition and criteria for diagnosis of AKI have evolved over time. There are many causes of AKI and identifying the cause is key in reversing and controlling the progression of disease. Metabolic acidosis, fluid overload, and sepsis require detailed evaluation to best provide the most appropriate plan and execution. Critical care nurses are vitally important when identifying and managing acute kidney injury. Renal replacement therapy is a remarkable tool that requires expertise from the critical nurse. Once it is mastered, the most vulnerable patient in the ICU can become the recipient of good and positive outcomes.

Transient pseudohypoaldosteronism in infancy mainly manifested as poor appetite and vomiting: Two case reports and review of the literature.

Introduction: Transient Pseudohypoaldosteronism (TPHA) is a very rare condition usually secondary to urinary tract malformations (UTM) and/or urinary tract infection (UTI). It is characterized by hyperkalemia, hyponatremia, metabolic acidosis, and elevated plasma aldosterone levels. Given that the predominant manifestations of TPHA patients are digestive tract symptoms, such as poor appetite, vomiting, and weight gain, it is easily misdiagnosed as digestive tract diseases. Case reports: Two children with poor appetite and vomiting were admitted to the Department of Gastroenterology, Children's Hospital of Nanjing Medical University, from 2020 to 2021. Laboratory test results of these two children revealed hyponatremia (< 135.00 mmol/L), hyperkalemia (> 5.50 mmol/L), and hyperaldosteronism (> 180.00 ng/L). Moreover, genetic tests demonstrated no genetic variants highly associated with the phenotype in both cases. The two patients were subsequently treated with electrolyte correction. One of them also treated with antibiotics and one of them underwent surgery. They were followed for 8 and 4 months, respectively. No complications were observed during the follow-up period. This review aimed to outline both cases with parental consent. Conclusion: Transient pseudohypoaldosteronism should be considered in children younger than 6 months, presenting with vomiting, poor appetite, unexplained hyponatremia, hyperkalemia, elevated aldosterone levels, and urethral malformation or urinary tract infection. Furthermore, attention should be paid to whether salt supplementation or anti-infection therapy is effective.

Alteraciones electrolíticas y del metabolismo en pacientes con diabetes mellitus y enfermedad renal crónica. Parte 1 / Electrolyte and metabolism alterations in patients with diabetes mellitus and chronic kidney disease. Part 1

En el paciente con diabetes mellitus (DM) y enfermedad renal crónica (ERC), las alteraciones electrolíticas y metabólicas constituyen un verdadero desafío. En noviembre de 2021, el Comité de Nefropatía de la Sociedad Argentina de Diabetes realizó una jornada científica con el objetivo de actualizar las alteraciones hidroelectrolíticas y del metabolismo óseo mineral, y las consideraciones dietarias en ERC y DM.

In patients with diabetes mellitus (DM) and chronic kidney disease (CKD), electrolyte and metabolic alterations constitute a real challenge. In November 2021, the Nephropathy Committee of the Argentine Diabetes Society held a scientific conference with the aim of updating hydroelectrolytic and mineral bone metabolism disorders, and dietary considerations in CKD and DM.

Diabetic Ketoacidosis With Refractory Hypokalemia Leading to Cardiac Arrest.

Diabetic ketoacidosis (DKA) is known to cause total body potassium depletion, but during initial presentation, very few patients are hypokalemic, and even fewer patients experience clinical effects. As the correction of acidosis and insulin drive potassium intracellularly, measured serum potassium levels decrease and require repletion. This phenomenon is well described, and severe hypokalemia necessitates delaying insulin therapy. Less well described is the kaliuretic nature of treatments of cerebral edema. We present a case of an adolescent male with new-onset type 2 diabetes who presented in DKA with signs of cerebral edema, hyperosmolarity, and hypokalemia. As insulin and cerebral edema therapy were initiated, his hypokalemia worsened despite significant IV repletion, eventually leading to ventricular tachycardia and cardiac arrest. Over the following 36 hours, the patient received >590 milliequivalents (mEq) of potassium. He was discharged home 12 days after admission without sequelae of his cardiac arrest.

Mechanisms of ion transport regulation by HNF1ß in the kidney: beyond transcriptional regulation of channels and transporters.

Hepatocyte nuclear factor 1ß (HNF1ß) is a transcription factor essential for the development and function of the kidney. Mutations in and deletions of HNF1ß cause autosomal dominant tubule interstitial kidney disease (ADTKD) subtype HNF1ß, which is characterized by renal cysts, diabetes, genital tract malformations, and neurodevelopmental disorders. Electrolyte disturbances including hypomagnesemia, hyperuricemia, and hypocalciuria are common in patients with ADTKD-HNF1ß. Traditionally, these electrolyte disturbances have been attributed to HNF1ß-mediated transcriptional regulation of gene networks involved in ion transport in the distal part of the nephron including FXYD2, CASR, KCNJ16, and FXR. In this review, we propose additional mechanisms that may contribute to the electrolyte disturbances observed in ADTKD-HNF1ß patients. Firstly, kidney development is severely affected in Hnf1b-deficient mice. HNF1ß is required for nephron segmentation, and the absence of the transcription factor results in rudimentary nephrons lacking mature proximal tubule, loop of Henle, and distal convoluted tubule cluster. In addition, HNF1ß is proposed to be important for apical-basolateral polarity and tight junction integrity in the kidney. Interestingly, cilia formation is unaffected by Hnf1b defects in several models, despite the HNF1ß-mediated transcriptional regulation of many ciliary genes. To what extent impaired nephron segmentation, apical-basolateral polarity, and cilia function contribute to electrolyte disturbances in HNF1ß patients remains elusive. Systematic phenotyping of Hnf1b mouse models and the development of patient-specific kidney organoid models will be essential to advance future HNF1ß research.