Update on Primary Aldosteronism: Time to Screen All Hypertensives.

Primary aldosteronism (PA), characterized by autonomous renin-independent aldosterone production, is the most common endocrine cause of hypertension.1 PA was initially considered a rare cause of secondary hypertension, as experts described 0.451% prevalence in mild to moderate hypertension when hypokalemia was an essential reason for screening.1 However, recent data suggests that PA may be present even in patients with normokalemia, and 515% of patients in the hypertensive cohort have underlying overt PA.2.

Hypokalemia Due to Ectopic Adrenocorticotropic Hormone.

Hypokalemia is a common disorder in clinical practice. The underlying pathophysiology can be attributed to 3 main mechanisms: insufficient potassium intake, excessive urinary or gastrointestinal losses, and transcellular shift. Renal loss is the most common cause of hypokalemia. Renal loss of potassium can occur due to diuretics, mineralocorticoid excess or hypercortisolism (Cushing syndrome). Among patients with Cushing syndrome, ectopic adrenocorticotropic hormone (ACTH) is the most frequent cause. We present a case of hypokalemia and hypertension due to ectopic ACTH production leading to Cushing syndrome.

Unusual presentation of Sjogren's syndrome during pregnancy: a case report.

BACKGROUND: Pregnancy imposes significant physiological changes, including alterations in electrolyte balance and renal function. This is especially important because certain disorders might worsen and make people more susceptible to electrolyte abnormalities. One such condition is Sjogren's syndrome (SS), an autoimmune disease that can cause distal renal tubular acidosis (dRTA). This case report offers a unique perspective on the intricate physiological interplay during pregnancy, emphasizing the critical importance of recognizing and managing electrolyte abnormalities, particularly in the context of autoimmune disorders such as Sjogren's syndrome. CASE PRESENTATION: We report a case of a 31-year-old pregnant Indian woman at 24 weeks gestation presenting with fever, gastrointestinal symptoms, and progressive quadriparesis followed by altered sensorium. Severe hypokalaemia and respiratory acidosis necessitated immediate intubation and ventilatory support. Investigations revealed hypokalaemia, normal anion gap metabolic acidosis, and positive autoimmune markers for SS. Concurrently, she tested positive for IgM Leptospira. Management involved aggressive correction of electrolyte imbalances and addressing the underlying SS and leptospirosis. CONCLUSION: This case underscores that prompt recognition and management are paramount to prevent life-threatening complications in pregnant patients with autoimmune disease. This report sheds light on the unique challenge of managing hypokalaemic quadriparesis in the context of Sjogren's syndrome during pregnancy.

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.

Differences in the clinical and hormonal presentation of patients with familial and sporadic primary aldosteronism.

Purpose: To compare the clinical and hormonal characteristics of patients with familial hyperaldosteronism (FH) and sporadic primary aldosteronism (PA). Methods: A systematic review of the literature was performed for the identification of FH patients. The SPAIN-ALDO registry cohort of patients with no suspicion of FH was chosen as the comparator group (sporadic group). Results: A total of 360 FH (246 FH type I, 73 type II, 29 type III, and 12 type IV) cases and 830 sporadic PA patients were included. Patients with FH-I were younger than sporadic cases, and women were more commonly affected (P = 0.003). In addition, the plasma aldosterone concentration (PAC) was lower, plasma renin activity (PRA) higher, and hypokalemia (P < 0.001) less frequent than in sporadic cases. Except for a younger age (P < 0.001) and higher diastolic blood pressure (P = 0.006), the clinical and hormonal profiles of FH-II and sporadic cases were similar. FH-III had a distinct phenotype, with higher PAC and higher frequency of hypokalemia (P < 0.001), and presented 45 years before sporadic cases. Nevertheless, the clinical and hormonal phenotypes of FH-IV and sporadic cases were similar, with the former being younger and having lower serum potassium levels. Conclusion: In addition to being younger and having a family history of PA, FH-I and III share other typical characteristics. In this regard, FH-I is characterized by a low prevalence of hypokalemia and FH-III by a severe aldosterone excess causing hypokalemia in more than 85% of patients. The clinical and hormonal phenotype of type II and IV is similar to the sporadic cases.

Maximum serum K+ concentration within 1 hour with enteral replacement in severe hypokalemia.

Introduction: Introduction: we report two cases with severe hypokalemia. Patients and methods: a 68-year-old woman was admitted with lower limb swelling and urinary symptoms; on the fourth day serum K+ concentration (s[K+]) was 2.3 mmol/L. A 64-year-old woman was admitted with pain in the lumbosacral spine, she was diagnosed with multiple myeloma. After receiving specific therapy she showed s[K+] at 2.4 mmol/L. A KCl solution containing 26.8 mEq of K+ was administered enterally, which increased s[K+] by 0.7 mmol/L within 1 h. Results and conclusion: these cases reveal that peak s[K+] may be achieved within 1 hour after KCl intake in severe hypokalemia, which is probably faster than IV administration.

Introducción: Introducción: se presentan dos casos clínicos con hipopotasemia severa. Pacientes y métodos: mujer de 68 años que ingresó por edema en miembros inferiores y síntomas urinarios; al cuarto día, el nivel sérico de K+ ([K+]s) era de 2,3 mmol/L. Una mujer de 64 años ingresó por dolor en la columna lumbosacra y fue diagnosticada de mieloma múltiple; luego de recibir terapia específica, presentó una [K+]s de 2.4 mmol/L. Se administró por vía enteral una solución de KCl que contenía 26,8 mEq de K+, aumentando la [K+]s en 0,7 mmol/L en 1 h. Resultados y conclusión: estos casos revelan que la [K+]s máxima se alcanzaría 1 hora después de la ingestión de KCl en la hipopotasemia grave, probablemente en menos tiempo que por vía intravenosa.

Severe Multifactorial Metabolic Alkalosis in the Emergency Department: A Case Report.

BACKGROUND: Metabolic alkalosis is an uncommon clinical entity resulting from a wide variety of underlying etiologies including gastrointestinal, renal, endocrine, and metabolic causes. It is a typically clinically silent condition; however, severe cases can be life-threatening, mandating both a systematic investigative approach and an early aggressive management strategy. CASE REPORT: We present a case of a 58-year-old man with severe, multifactorial metabolic alkalosis (pH 7.72, HCO3- 42 mmol/L, pCO2 31 mm Hg) resulting from refractory vomiting, severe hypokalemia (2.0 mmol/L), and hypoalbuminemia (albumin 20 g/L). WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Severe metabolic alkalosis is associated with significant morbidity and mortality. Clinicians need to be aware of the potential underlying causes in these cases, as well as how to delineate between chloride- and non-chloride-depleted states, which dictates initial treatment. We provide a pragmatic summary of the evaluation, pertinent investigations, and early management of these cases.

Postoperative Prevalence and Risk Factors for Serum Hypokalemia in Patients with Primary Total Joint Arthroplasty.

OBJECTIVE: Regular monitoring of serum potassium after a total joint arthroplasty (TJA) is a form of routine examination that can help detect abnormal serum potassium levels and reduce the incidences of adverse events that may occur on account of postoperative hypokalemia. Previous studies rarely discussed hypokalemia after joint replacement. In the present study, our primary goal was to investigate the incidence and possible risk factors of hypokalemia after a total hip and knee replacement procedure was performed. METHODS: This study included patients who underwent a unilateral total knee or hip arthroplasty in our department between April 2017 and March 2018. Serum potassium levels pre and post operation were collected and retrospectively analyzed. The differences in age, gender, body mass index (BMI), history of diseases, red blood cell (RBC), hemoglobin, hematocrit, glomerular filtration rate, ejection fraction, blood glucose, urine creatinine, urea nitrogen, intraoperative blood loss, operation time, drainage, preoperative potassium, surgery type, were compared between those patients diagnosed with hypokalemia and their non-hypokalemia at different times post surgery. Thereafter, the risk factors of postoperative hypokalemia patients were analyzed using statistical procedure multiple logistic regression model. RESULTS: The risk of hypokalemia after TJA was 53.1%, while, that on the first, third, and fifth day after operation was 12.5%, 40.7%, and 9.6% respectively. The serum potassium level on the first, third, and fifth postoperative days was 3.84 ± 0.32, 3.59 ± 0.34, and 3.80 ± 0.32 mmol/l, respectively. However, the level on the third day appeared to be the lowest (p = 0.015) of them all. The independent risk factors for hypokalemia after a total hip and knee replacement were the level of preoperative serum potassium concentration (p = 0.011), preoperative red blood cells counts (p = 0.027), and history of diabetes (p = 0.007). CONCLUSION: Regular monitoring of serum potassium concentration should be performed post TJA. We need to pay more attention to the patient's preoperative potassium levels along with their red blood cell counts especially in patients diagnosed with type 2 diabetes mellitus.

A Prospective Observational Study of Factors Affecting the Change in Quality of Life in Patients With Primary Aldosteronism After Treatment.

OBJECTIVE: To explore the changes in the health-related quality of life (HRQoL) in patients with primary aldosteronism (PA) after standardized treatment and determine the effects of different variables on the change in the HRQoL of patients. METHODS: A total of 116 patients with PA were prospectively included from November 2020 to March 2022. Data were collected at their initial diagnosis and the follow-up after 12 months of treatment, including demographic and clinical data and the scores of the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36). The scores of each dimension of SF-36 of patients before and after treatment were compared, and the factors affecting their change in the quality of life were analyzed using multiple linear regression. RESULTS: After standardized treatment, the aldosterone-to-renin ratio (Z = -4.967, P < .001), systolic blood pressure (t = 8.985, P < .001), and diastolic blood pressure (t = 7.233, P < .001) of patients with PA decreased compared with baseline, and hypokalemia was effectively corrected (χ2 = 69.014, P < .001). In terms of quality of life, 6 of 8 dimensions of SF-36 and the total score of SF-36 significantly improved at 1-year follow-up compared with baseline (all P < .05). The results of multiple linear regression showed that the improvement in the HRQoL in patients with PA after standardized treatment was correlated with the change in the blood potassium level (P = .007) and systolic blood pressure (P = .003). CONCLUSION: Correction of hypokalemia and control of diastolic blood pressure are essential factors contributing to the improvement in the HRQoL in patients with PA regardless of the standardized treatment received.

Severe Alkalemia and hypokalemia after chronic exposure to alkaline water.

Consumption of alkaline electrolyzed water (AEW) has become increasingly popular for consumer use. Although these alkaline water products are now commonly used, they are of questionable health benefit. Some individuals believe that it may help their dyspepsia. Furthermore, there is a paucity of evidence on its toxicologic profile and adverse effects. This is a single case report of a 42-year-old female with a past medical history of gestational diabetes, necrotizing pancreatitis, presented to the Emergency Department for 3 weeks of lethargy, weakness, difficulty walking, and vomiting. She endorsed consuming 5  liters (L) of alkaline water daily for the past month. Initial labs showed pH 7.69, potassium 1.6meQ/L, sodium 133 meQ/L, chloride 65 mmol/L, magnesium 0.9 meQ/L, and bicarbonate 46 mmol/L, and lactate of 13.2 mmol/L. EKG showed sinus tachycardia with QTc of 630 milliseconds. Patient was treated supportively with intravenous fluids and electrolyte replacement. The potassium rose to 6.6 meQ/L, which then the patient was treated for hyperkalemia. After four days of intravenous fluid and electrolyte replacement, the patient's electrolytes and acid-base status normalized, and she was transferred to the medical floors for further management. This case report illustrates severe metabolic alkalosis and hypokalemia in the setting of chronic alkaline water exposure. It also is an example of alkalemia with hyperlactatemia, or "lactic alkalosis". To our knowledge, there is no previous literature reporting serious adverse effects of alkaline bottled water products.

"Ion-Flux": The natural course of ECG changes in severe hypokalemia.

Whereas the electrocardiogram (ECG) changes in hypokalemia are well known, they often receive less attention than the more striking features of hyperkalemia. Furthermore, there is a need for further discussion as to the subtleties of ECG changes that can aid in the differential diagnoses. This case study presents the ECG changes of a patient with severe hypokalemia due to diarrhea. It highlights how bifid T-waves in hypokalemia can be distinguished from other conditions such as coronary artery disease or pericarditis. Furthermore, it also shows the gradual reversal of ECG changes in the same patient when potassium is normalized.

Long-Term Indomethacin Treatment in a Chinese Child with Gitelman Syndrome: Case Report and Literature Review on its Efficacy and Tolerance.

BACKGROUND Gitelman syndrome (GS) is a rare inherited autosomal recessive salt-losing renal tubulopathy. Early-onset GS is difficult to differentiate from Bartter syndrome (BS). It has been reported in some cases that cyclooxygenase (COX) inhibitors, which pharmacologically reduce prostaglandin E2(PGE2) synthesis, are helpful for GS patients, especially in children, but the long-term therapeutic effect has not yet been revealed. CASE REPORT A 4-year-old boy was first brought to our hospital for the chief concern of short stature and growth retardation. Biochemical tests demonstrated severe hypokalemia, hyponatremia, and hypochloremic metabolic alkalosis. The patient's serum magnesium was normal. He was diagnosed with BS and treated with potassium supplementation and indomethacin and achieved stable serum potassium levels and slow catch-up growth. At 11.8 years of age, the patient showed hypomagnesemia and a genetic test confirmed that he had GS with compound heterozygous mutations in the SLC12A3 gene. At the age of 14.8 years, when indomethacin had been taken for nearly 10 years, the boy reported having chronic stomachache, while his renal function remained normal. After proton pump inhibitor and acid inhibitor therapy, the patient's symptoms were ameliorated, and he continued to take a low dose of indomethacin (37.5 mg/d divided tid) with good tolerance. CONCLUSIONS Early-onset GS in childhood can be initially misdiagnosed as BS, and gene detection can confirm the final diagnosis. COX inhibitors, such as indomethacin, might be tolerated by pediatric patients, and long-term therapy can improve the hypokalemia and growth retardation without significant adverse effects.

Primary aldosteronism without hypertension:A case report and review of the literature / Hyperaldostéronisme primaire sans hypertension artérielle : à propos d'un cas et revue de la littérature

We describe the case of a patient who presented with hyperaldosteronism without arterial hypertension. She had been referred for consultation for persistent severe hypokalaemia despite oral KCl supplementation. The absence of hypertension had been proven by repeated clinical measurements and by ABPM. Hyperaldosteronism had been demonstrated by hormonal assays and catheterization of the adrenal veins. Abdominal CT revealed a left adrenal adenoma. Finally, the anatomopathological examination of the surgical specimen confirmed the adenoma. After the intervention, serum potassium normalized. The clinical case is completed by a review of the literature of hyperaldosteronisms without arterial hypertension.

Nous décrivons le cas d'une patiente qui s'est présentée avec un hyperaldostéronisme sans hypertension artérielle. Elle a été adressée en consultation pour une hypokaliémie sévère persistante malgré une supplémentation orale en chlorure de potassium (KCl). L'absence d'hypertension a été prouvée par des mesures cliniques répétées et par mesure ambulatoire de la pression artérielle (MAPA). L'hyperaldostéronisme a été mis en évidence par des dosages hormonaux et un cathétérisme des veines surrénales. Le scanner abdominal a révélé un adénome surrénalien gauche. Enfin, l'examen anatomopathologique de la pièce opératoire a confirmé l'adénome. Après l'intervention, le potassium sérique s'est normalisé. Le cas clinique est complété par une revue de la littérature des hyperaldostéronismes sans hypertension artérielle.

Primary aldosteronism: Practical recommendations for treatment and follow-up / Hiperaldosteronismo primario: recomendaciones prácticas de tratamiento y seguimiento

Primary aldosteronism (PA) is the most common cause of secondary arterial hypertension. For unilateral cases, surgery offers the possibility of cure, with unilateral adrenalectomy being the treatment of choice, whereas bilateral forms of PA are treated mainly with mineralocorticoid receptor antagonists (MRA). The goals of treatment for PA due to either unilateral or bilateral adrenal disease include reversal of the adverse cardiovascular effects of hyperaldosteronism, normalization of serum potassium in patients with hypokalemia, and normalization of blood pressure. The Primary Aldosteronism Surgery Outcome group (PASO) published a study defining clinical and biochemical outcomes based on blood pressure and correction of hypokalemia and aldosterone to renin ratio (ARR) levels for patients undergoing total unilateral adrenalectomy for unilateral PA. In this review, we provide several practical recommendations for the medical and surgical management and follow-up of patients with PA. (AU)

El hiperaldosteronismo primario (HAP) es la causa más frecuente de hipertensión arterial secundaria. Para los casos unilaterales, la cirugía ofrece la posibilidad de curación, siendo la adrenalectomía unilateral el tratamiento de elección, mientras que las formas bilaterales de HAP se tratan principalmente con antagonistas del receptor de mineralocorticoides (ARM). Los objetivos del tratamiento del HAP debido a enfermedad suprarrenal unilateral o bilateral incluyen la reversión de los efectos cardiovasculares adversos del hiperaldosteronismo, la normalización del potasio sérico en pacientes con hipopotasemia y la normalización de la presión arterial. El grupo Primary Aldosteronism Surgery Outcome (PASO) publicó un estudio que define los resultados clínicos y bioquímicos en función de la presión arterial y la corrección de la hipopotasemia y los niveles del cociente aldosterona/renina (ARR) para pacientes sometidos a adrenalectomía unilateral total por HAP unilateral. En esta revisión ofrecemos varias recomendaciones prácticas para el manejo y el seguimiento médico-quirúrgico de los pacientes con HAP. (AU)

Manifestación inicial atípica de un hiperaldosteronismo primario / Atypical initial manifestation of primary hyperaldosteronism

Aproximadamente 10% de los casos de hipertensión arterial son debidos a una causa secundaria, encontrándose entre las más frecuentes el hiperaldosteronismo primario, caracterizado por hipertensión, alcalosis metabólica e hipopotasemia. No obstante, en raras ocasiones puede debutar de una forma atípica, en forma de debilidad muscular y mialgias secundarias a rabdomiólisis por hipopotasemia severa, como el caso que se describe. (AU)

Approximately 10% of cases of arterial hypertension are due to a secondary cause, being among the most frequent primary hyperaldosteronism, characterized by hypertension, metabolic alkalosis and hypokalemia. However, on rare occasions it can present in an atypical way, in the form of muscle weakness and myalgia secondary to rhabdomyolysis due to severe hypokalemia, as in the case described. (AU)

Gitelman syndrome combined with diabetes mellitus: A case report and literature review.

RATIONALE: Gitelman syndrome (GS) is an uncommon autosomal recessive tubulopathy resulting from a functional deletion mutation in the SLC12A3 gene. Its onset is typically insidious and challenging to discern, and it is characterized by hypokalemia, metabolic alkalosis, and reduced urinary calcium excretion. There is limited literature on the diagnosis and management of GS in individuals with concomitant diabetes. PATIENT CONCERNS: A 36-year-old male patient with a longstanding history of diabetes exhibited suboptimal glycemic control. Additionally, he presented with concurrent findings of hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis. DIAGNOSIS: Building upon the patient's clinical manifestations and extensive laboratory evaluations, we conducted thorough genetic testing, leading to the identification of a compound heterozygous mutation within the SLC12A3 gene. This definitive finding confirmed the diagnosis of GS. INTERVENTIONS: We have formulated a detailed medication regimen for patients, encompassing personalized selection of hypoglycemic medications and targeted electrolyte supplementation. OUTCOMES: Following 1 week of comprehensive therapeutic intervention, the patient's serum potassium level effectively normalized to 3.79 mmol/L, blood glucose parameters stabilized, and there was significant alleviation of clinical symptoms. LESSONS: GS has a hidden onset and requires early diagnosis and intervention based on patient related symptoms and laboratory indicators in clinical practice, and personalized medication plans need to be provided according to the specific situation of the patient.

Liddle syndrome presenting with normal aldosterone levels: A case report.

INTRODUCTION: Liddle syndrome is an autosomal dominant disorder characterized by hypertension, hypokalemia, low aldosterone levels, and reduced renin activity. Atypical Liddle syndrome can be easily misdiagnosed due to its clinical phenotypes resembling hyperaldosteronism. PATIENT CONCERN: The patient was diagnosed with primary aldosteronism due to hypertension and hypokalemia, and underwent left adrenalectomy. After the operation, the patient still had hypertension and hypokalemia that were not easy to control and correct, and had acute cerebral infarction. DIAGNOSIS: The genetic test showed that the base duplication in the coding region of SCN1B gene caused a frameshift mutation:c.1789dupC (p.Arg597fs), Liddle syndrome was diagnosed. INTERVENTION AND OUTCOMES: The patient was treated with a low-sodium diet and oral triamterene. The serum potassium level returned to normal and the blood pressure was controlled. LESSONS: Some Liddle syndrome may present with normal aldosterone levels, genetic testing is necessary for the diagnosis. If the diagnostic test of primary aldosteronism is positive, but the treatment with spironolactone is ineffective, we should actively search for other causes.

[Hyponatremia and Electrolyte Disorders in Cancer Patients].

Onconephrology is a rising and rapidly expanding field of medicine in which nephrology and oncology meet each other. Besides multidisciplinary meetings, oncologists and nephrologists often discuss on timing of the treatment, dosage, and side effects management. Cancer patients often encounter different electrolyte disorders. They are mostly secondary to the tumor itself or consequences of its treatment. In the last years, the great efforts to find new therapies like targeted, immune, and cell-based led us to many new side effects. Hyponatremia, hypokalemia, hyperkalemia, hypercalcemia, and hypomagnesemia are among the most common electrolyte disorders. Data have shown a worse prognosis in patients with electrolytic imbalances. Additionally, they cause a delay in chemotherapy or even an interruption. It is important to diagnose promptly these complications and treat them. In this review, we provide a special focus on hyponatremia and its treatment as the most common electrolytes disorder in cancer patients, but also on newly described cases of hypo- and hyperkalemia and metabolic acidosis.