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The article presents a case study of congenital chloride diarrhea (CCD) that was misdiagnosed as Hirschsprung's disease, highlighting the importance of accurate diagnosis to avoid unnecessary surgery. Topics discussed include the clinical presentation and genetic factors of CCD, diagnostic challenges associated with distinguishing CCD from similar conditions, and the management strategies that can be employed to treat the disorder effectively.

We describe a 17-year-old woman diagnosed with severe hypertension during routine follow-up after the prescription of a combined oral contraceptive pill. Initially, due to her age, the estradiol-containing contraception, and high-level sport practice, physicians suspected drug-induced hypertension. Blood tests showed hypokalemia, and further investigations revealed pseudoaldosteronism. After the exclusion of toxic causes, Liddle syndrome was suspected and confirmed by genetic testing. Optimal therapeutic management was limited by anti-doping rules. This case report emphasizes the need for an early and systematic workup for causes of secondary hypertension in young patients and underlines diagnostic and therapeutic challenges in the management of hypertension in athletes. [ABSTRACT FROM AUTHOR]

ABSTRACT Macronutrients such as nitrogen (N), phosphorus (P), potassium (K) and sulphur (S) are critical for plant growth and development. Field‐grown canola (Brassica napus L.) is supplemented with fertilizers to maximize plant productivity, while deficiency in these nutrients can cause significant yield loss. A holistic understanding of the interplay between these nutrient deficiency responses in a single study and canola cultivar is thus far lacking, hindering efforts to increase the nutrient use efficiency of this important oil seed crop. To address this, we performed a comparative quantitative proteomic analysis of both shoot and root tissue harvested from soil‐grown canola plants experiencing either nitrogen, phosphorus, potassium or sulphur deficiency. Our data provide critically needed insights into the shared and distinct molecular responses to macronutrient deficiencies in canola. Importantly, we find more conserved responses to the four different nutrient deficiencies in canola roots, with more distinct proteome changes in aboveground tissue. Our results establish a foundation for a more comprehensive understanding of the shared and distinct nutrient deficiency response mechanisms of canola plants and pave the way for future breeding efforts. [ABSTRACT FROM AUTHOR]

ABSTRACT Root System Architecture (RSA) is a crucial plant trait that governs a plant's ability to absorb water and nutrients. In this study, we describe a mutant with nutrient‐dependent defects in root development, affecting both the primary root and lateral roots (LRs). This mutant, identified through a screen for defects in LR development, has been designated dlr1‐1. The dlr1‐1 mutant exhibits impaired LR emergence rather than defects in the LR primordium (LRP) formation, particularly under potassium (K+)‐deprivation conditions. This impairment likely stems from inhibited cell proliferation caused by the dlr1‐1 mutation. K+ deprivation specifically leads to the accumulation of salicylic acid (SA) in the dlr1‐1 mutant, consistent with the upregulation of SA biosynthesis genes. Moreover, exogenous application of SA to wild‐type plants (B73) mimics the dlr1‐1 phenotype. Conversely, treatment of the dlr1‐1 mutant with 2‐aminoindane‐2‐phosphonic acid, an SA biosynthesis inhibitor, partially restores LR emergence, indicating that elevated SA levels may be responsible for the mutant's developmental defects. MutMap analysis and allelism tests confirmed that the phenotypes of the dlr1‐1 mutant results from the loss of the Na+/H+ antiporter, ZmNHX7. Additionally, the application of NaCl exacerbates the dlr1‐1 mutant phenotype, suggesting that the root defects in dlr1‐1 mutant depend on ion homoeostasis. In conclusion, our findings demonstrate that maize DLR1/NHX7 is essential for root development under potassium deprivation. [ABSTRACT FROM AUTHOR]

Introduction: Potassium deficiency significantly hinders cotton growth and development, adversely affecting yield and fiber quality. Applying potassium fertilizer is a common practice to address potassium deficiency in the soil. However, the effectiveness of potassium fertilizer application depends on the appropriate soil potassium levels in cotton fields. Methods: This study used a randomized block design with six different soil potassium levels and conducted experiments across 18 micro-zones in the field. This study aimed to investigate the response of cotton yield and quality to different soil potassium levels, to try to clarify the suitable soil potassium levels for cotton growth, so as to provide practical and effective help for determining the amount of potash fertilizer in the cotton field. Results: The results showed that the seedcotton yield was increasing, with the soil potassium level increased under no tillage. There was no significant difference among K4, K5, and K6 on seedcotton yield, which were significantly higher than K1 and K2. As soil potassium levels increased, the proportion of autumn boll and the proportion of outer boll also increased, indicating that higher soil potassium levels support the better growth and development of cotton in the middle and late stages, leading to increased boll sets and higher yields. Additionally, the available potassium content in the 0-40-cm soil layer was significantly correlated with yield and yield parameters but not with fiber quality indices. Discussion: It is concluded that K4 treatment could provide sufficient potassium to meet the growth and development needs of cotton. Potassium fertilizer application is recommended when the available potassium content in the 0-40-cm soil layer falls below 122.88 mg kg-1 in the cotton field. [ABSTRACT FROM AUTHOR]

The essential role of the inwardly rectifying potassium channel Kir5.1 (KCNJ16) in controlling electrolyte homeostasis and blood pressure has been demonstrated in human and animal studies. Previous studies have identified several bi‐allelic mutations of KCNJ16 in humans, causing severe hypokalemia, renal salt wasting, and disturbed acid–base homeostasis. Here, we identified a novel homozygous variant of KCNJ16, I26T, in an Amish patient affected with polydipsia, developmental delay, and chronic metabolic acidosis with low serum bicarbonate concentration. Subsequently, we generated the rat model with I26T mutation using Dahl salt‐sensitive rat (I26T rat) to characterize this variant. The male mutant rats displayed similar blood pressure and electrolyte homeostasis under baseline and with a high salt (4% NaCl) challenge. Blood pH, HCO3− and renal damage also remained similar between WT and I26T rats after high salt challenge. Additionally, single‐channel patch clamp analysis revealed similar channel activity in CHO cells overexpressed with WT and I26T mutant Kir4.1/5.1 channels. In summary, this study reported a novel variant in KCNJ16, namely I26T, which is likely a benign variant and not associated with pathologic phenotype in either human or Dahl salt‐sensitive rats, indicating that the type/location of variant should be considered when diagnosing and treating patients with KCNJ16 mutations. [ABSTRACT FROM AUTHOR]

Background: The two classic manifestations of primary aldosteronism are hypertension and hypokalemia. However, acute respiratory failure due to hypokalemia in primary hyperaldosteronism is rare. Case presentation: The patient was a 27-year-old female who presented with drowsiness and weakness in all extremities. She had been diagnosed with hypertension three years prior, with irregular follow-up, and had a history of preeclampsia one year later. She exhibited high blood pressure and severe hypokalemia (2 mEq/L), leading to respiratory depression and impending respiratory arrest. Consequently, the patient was intubated and transferred to the intensive care unit (ICU). She also developed rhabdomyolysis. Blood pressure tests, including hormonal tests (aldosterone: 13.2 ng/dL, plasma renin activity: 0.32 ng/mL/h), were conducted. Due to the high aldosterone-renin ratio, an abdominopelvic computed tomography (CT) scan was performed. The CT scan revealed a 14 × 12 mm round mass with a washout value above 60%, consistent with an adrenal adenoma, leading to a diagnosis of primary aldosteronism. The patient was discharged after stabilization, and one and a half months after ICU admission, a laparoscopic left adrenalectomy was successfully performed without post-operative complications. Histopathology showed encapsulated hypertrophy of the adrenal cortex with a predominance of large clear cells, confirming the diagnosis of adrenal adenoma. At the most recent follow-up, the patient had normal potassium levels, was normotensive without any medications, and exhibited no alarming signs or symptoms. Conclusion: Respiratory depression to the extent of impending respiratory failure and rhabdomyolysis as a result of hypokalemia in primary aldosteronism are extremely rare. In this patient, who developed respiratory depression due to resistant hypokalemia, timely investigation of secondary causes and diagnosis of adrenal adenoma were crucial. The surgery provided definitive treatment for the patient's blood pressure and prevented the recurrence of life-threatening complications. [ABSTRACT FROM AUTHOR]

Background: Primary aldosteronism is characterized by high plasma aldosterone and low renin. The plasma aldosterone-to-renin ratio is recommended for screening. Severe hydronephrosis leads to renal parenchymal ischemia, resulting in increased renin secretion. Since nonsuppression of renin may cause a negative result in the aldosterone-to-renin ratio test, severe hydronephrosis and primary aldosteronism occurring simultaneously in a patient are challenging to diagnose. Case presentation: A 54-year-old Chinese man of Han ethnicity was diagnosed with hypertension and severe hypokalemia (minimum 1.57 mmol/L) 13 years prior, and was also diagnosed with severe hydronephrosis due to congenital ureteral stenosis on the left side. His clinical features suggested primary aldosteronism, but the aldosterone-to-renin ratio result of the patient was negative every time he underwent the primary aldosteronism screening test. No further treatment for primary aldosteronism was performed, which led the patient to suffer from severe hypokalemia, such that he was taking 12–15 g/day potassium chloride orally to keep his blood potassium between 3.0 and 3.5 mmol/L (reference value, 3.5–5.5 mmol/L) for 13 years, and the patient needed to be hospitalized in the intensive care unit for rescue several times. At admission, although the aldosterone-to-renin ratio result of the patient was negative, we still did the saline stress test and captopril inhibition test, and the results showed that the plasma aldosterone level was not lower after the test than before the test. Adrenal enhanced computed tomography suggested an adenoma in the left adrenal gland, and the results of adrenal vein sampling suggested that the left side was the dominant side. Therefore, laparoscopic total resection of the left adrenal gland was performed, and 2 weeks later, the patient developed short-term renal function impairment and hyperkalemia, but his renal function and blood potassium returned to normal after treatment that included fluid rehydration. The patient's biochemical test results and clinical symptoms were completely normal after 1 year. Conclusion: We suggest that for patients with a high suspicion of primary aldosteronism in the clinic, comprehensive analysis must be performed in combination with clinical characteristic assessments, such as severe hydronephrosis, if renin is within the normal range or if the aldosterone-to-renin ratio result is negative at screening and diagnostic tests, and adrenal vein sampling should be performed if necessary. It can help avoid misdiagnoses and contribute to the treatment of patients with severe hydronephrosis and primary aldosteronism. [ABSTRACT FROM AUTHOR]

Methadone, a well-known drug used for pain control and as a treatment for opioid addiction, can cause arrhythmias, including torsades de pointes (TdP), which may progress to ventricular fibrillation and sudden death. We present a case of a middle-aged woman with a long history of methadone use who presented to the emergency department after experiencing cardiac arrest at home. During her hospitalization, she experienced multiple episodes of TdP that improved with isoproterenol and potassium correction. The initial diagnosis was methadone-induced prolonged QT. However, even with discontinuation of methadone, her QTc remained prolonged. Congenital long QT syndrome was suspected, and genetic testing was instructed to test in the outpatient setting. She was discharged on nadolol and a LifeVest. [ABSTRACT FROM AUTHOR]

Introduction: Periodic paralysis is a condition that causes recurrent episodes of flaccid paralysis, and it can be primary or secondary. Hypokalemic periodic paralysis is the most common type of primary periodic paralysis, and it is inherited through autosomal dominant gene transmission. Males are affected three times more often than females, and the paralysis attacks usually occur at night after a period of vigorous exercise. It is crucial to exclude other diagnostic entities based on the nature of presentation, physical examination, and paraclinical studies. Thyrotoxic periodic paralysis is more prevalent in Asian or Hispanic males with thyrotoxicosis, where up to 10% of thyrotoxic patients may experience periodic paralysis. Case Presentations: Here, we present 6 cases of patients who came to our care with varying degrees of muscle weakness, each showing interesting and diverse laboratory results. Conclusion: In patient assessment, it is crucial to consider social and family history. Even without this information, awareness of potential diagnoses is vital. The cause should be carefully considered for possible simple treatments. Failing to recognize and address this condition promptly could lead to severe outcomes. Timely identification and intervention are essential for effective disease management and patient welfare. [ABSTRACT FROM AUTHOR]

This review highlights the molecular basis of salt sensitivity in hypertension, with a focus on the regulation of sodium transport in the distal nephron. Sodium reabsorption in this region is often linked to the actions of aldosterone, although in recent years numerous findings have been reported on the aldosterone-independent pathway of acquiring salt sensitivity by potassium deficiency or potassium loading. The key to this discussion is the interplay, through extracellular potassium concentration, between the first part of the tubules expressing the Na+-Cl− cotransporter (NCC) and the second part expressing the epithelial Na+ channel (ENaC). The molecular pathways such as with-no-lysine 1 (WNK)-STE20/SPS1-related proline-alanine-rich kinase (SPAK)/oxidative stress-responsive kinase 1 (OSR1) signaling, Kelch-like family member 3 (KLHL3)-cullin 3 (CUL3) complex, protein phosphatases, and mechanistic target of rapamycin complex 2 (mTORC2)-Nedd4L pathway are described as the mechanism by which salt sensitivity on blood pressure is acquired in response to changes in physiological conditions including potassium depletion or loading. This review highlights the potential for targeting these molecular pathways to develop novel therapeutic strategies for the treatment of salt-sensitive hypertension, the mechanism of which remains to be elucidated. [ABSTRACT FROM AUTHOR]

Background Progressive disseminated histoplasmosis is a significant issue in Latin America, particularly in Brazil, contributing to high mortality rates. Objectives Our objectives were to comprehensively describe histoplasmosis treatment with various amphotericin B (AmB) formulations, including mortality rates, adverse effects and risk factors for mortality. Methods This multicentre retrospective cohort study (January 2014–December 2019) evaluated medical records of patients with proven or probable histoplasmosis treated with at least two doses of AmB in seven tertiary medical centres in Brazil. We assessed risk factors associated with death during hospitalization using univariate and multivariate analyses. Results The study included 215 patients, mostly male (n  = 158, 73%) with HIV infection (n  = 187, 87%), and a median age of 40 years. Only 11 (5%) patients initiated treatment with liposomal amphotericin B (L-AmB). Amphotericin B deoxycholate (D-AmB) was administered to 159 (74%) patients without changes in the treatment. The overall mortality during hospitalization was 23% (50/215). Variables independently associated with mortality were use of D-AmB (OR 4.93) and hospitalization in ICU (OR 9.46). There was a high incidence of anaemia (n  = 19, 90%), acute kidney injury (n  = 96, 59%), hypokalaemia (n  = 73, 55%) and infusion reactions (n  = 44, 20%) during treatment. Conclusions We found that D-AmB was the main formulation, which was also associated with a higher mortality rate. Lipid formulations of AmB have become more readily available in the public health system in Brazil. Further studies to evaluate the effectiveness of L-AmB will likely show improvements in the treatment outcomes for patients with disseminated histoplasmosis. [ABSTRACT FROM AUTHOR]

This report describes a suspected case of paraneoplastic syndrome of inappropriate antidiuretic hormone (SIADH) in a 5‐year‐old male entire Australian Kelpie associated with a gastric adenocarcinoma. The dog had a history of chronic vomiting and presented with hyponatraemia, hypokalaemia, hypochloraemia and increased urinary sodium. Computed tomography identified the presence of a gastric tumour and histopathology confirmed a pyloric gastric adenocarcinoma. Removal of the tumour via a Billroth I procedure resulted in rapid resolution of electrolyte derangements. The dog was still alive six months postoperatively with no recurrence of either SIADH or tumour at the time of publication. [ABSTRACT FROM AUTHOR]

Aims: This study describes the incidence of fatigue in kidney transplant recipients and analyses the relationship between physiological factors, psychological factors, situational factors and fatigue in kidney transplant recipients. Background: Fatigue, as a common symptom after kidney transplantation, is affected by many factors, but the influence of some factors on the fatigue of kidney transplant recipients is still controversial. Design: This cross‐sectional study was designed based on the theory of unpleasant symptoms. Methods: Our survey involved 307 participants attending the kidney transplant outpatient clinic of a tertiary Class A hospital (Changsha, Hunan, China). Data were collected between February and April 2021 using a structured questionnaire and electronic medical records. Data were analysed using IBM SPSS 25.0 (SPSS Inc.) Results: It was found that the incidence of fatigue in kidney transplant recipients was 53.1%. According to the binary logistic regression analysis, sleep quality, hypokalemia, anxiety, depression and education level were independent risk factors for fatigue in kidney transplant recipients. Conclusion: The incidence of fatigue in kidney transplant recipients was high and was influenced by physical, psychological and situational factors. Clinical nurses should assess fatigue levels in a timely and multidimensional manner in clinical practice and provide effective and scientific guidance about fatigue self‐coping and symptom management for kidney transplant recipients. Summary statement: What is already known about this topic? Fatigue is a common symptom of kidney transplant recipients and can impair postoperative recovery, reemployment, quality of life, social activities and physical and mental states, causing the body to be in a state of chronic energy consumption.Owing to the imperceptible physical injury upon kidney transplant recipients, fatigue is often underappreciated or even disregarded. What this paper adds? It was found that the rate of fatigue in kidney transplant recipients was high.The risk factors for fatigue in kidney transplant recipients based on the theory of unpleasant symptoms were analysed in three ways: physiological, psychological and situational. The results showed that sleep quality, duration after transplantation, serum potassium level, anxiety, depression, education level, financial burden and social support were associated with fatigue in kidney transplant recipients. The implications of this paper: Clinical nurses should conduct timely and multidimensional fatigue evaluations of kidney transplant recipients and pay more attention to those with a high level of fatigue.The findings can provide a scientific basis for kidney transplant specialist nurses to conduct targeted interventions and facilitate fatigue management for kidney transplant recipients. They can also help to develop fatigue intervention models based on the displeasure symptom theory to improve fatigue in kidney transplant recipients. [ABSTRACT FROM AUTHOR]

We aimed to investigate clinical and laboratory characteristics of acute alcohol intoxication (AAI) in adolescents who presented to the pediatric emergency department (ED) at a tertiary referral center from 2006 to 2019. All consecutive adolescents with AAI (n = 335) and their sex- and age-matched control subjects (n = 335) with undetectable ethanol levels were included in this case-matched study. Mean serum ethanol level was 156.4 ± 58.4 (range: 50.8-341.2) mg/dL in the acute alcohol intoxication (AAI) group. Glasgow coma scores were lower in AAI group (14 [14-15] vs 15 [15-15], P <.001). Acidosis (16.3%), hyperlactatemia (60.9%), hypoglycemia (1.7%), hypernatremia (2.2%), hypokalemia (12.3%), hyperchloremia (20.4%), hypocalcemia (13.9%), hypermagnesemia (9.7%), and hyperalbuminemia (10.4%) were significantly more common in the AAI group than the control group. Blood pH, lactate, Na+, K+, Ca++, Mg++, albumin, blood urea nitrogen (BUN), and uric acid levels were correlated with serum ethanol levels. This study shows that AAI frequently leads to mild to moderate metabolic/biochemical derangements in adolescents. [ABSTRACT FROM AUTHOR]

The article focuses on an 8-year-old girl diagnosed with atypical hemolytic uremic syndrome (aHUS) after presenting with non-bloody diarrhea, hemolytic anemia, and thrombocytopenia. Topics include her clinical presentation with elevated blood pressure and kidney involvement, the negative tests for shiga toxin and the normal ADAMTS-13 activity, and her subsequent treatment with plasma exchange and eculizumab, leading to improved kidney function and the cessation of dialysis.

Introduction: Hypoxic respiratory failure occurs when the respiratory system cannot adequately provide oxygen to the body, leading to hypoxemia. High-flow nasal cannula (HFNC) improved the survival rate among patients with acute hypoxic respiratory failure. Due to physiological alterations, pregnancy can exacerbate distal renal tubular acidosis (dRTA). Pregnancy complicated by hypokalemic periodic paralysis (HPP) poses significant risks due to the potential cardiac and respiratory failure related to low potassium levels. Case description: A woman, 21 years old, pregnant with her first child at 33-34 weeks gestation came with complaints of shortness of breath one day before admission. Complaints accompanied by cramps in both legs. On the second day, the respiratory rate (RR) suddenly increased to 35x/min, and oxygen saturation (SpO2) was 95% on a non-rebreathing mask (NRM) at 15 l/min. The patient was transferred to the Intensive Care Unit (ICU) with a diagnosis of primigravida at 33-34 weeks, respiratory failure, HPP, suspected dRTA, severe metabolic acidosis, sepsis, and urinary tract infection (UTI). She was given O2 via HFNC at flow 40 l/min and a fraction of inspired oxygen (FiO2) 66%. Improvement was seen on the 4th day; the patient's shortness of breath was reduced with HFNC, and the patient's motor strength improved. Conclusion: The use of HFNC showed a positive outcome and was proven to have been successful in treating hypoxic respiratory failure in pregnant women. Respiratory muscle weakness and severe metabolic acidosis caused by dRTA and HPP cannot be cleared due to the limited settings in rural areas. Furthermore, the assessment and management of HPP and dRTA are still limited in many hospitals in remote areas of Indonesia. [ABSTRACT FROM AUTHOR]

Hypokalemia is a common laboratory finding in hospitalized patients, typically resulting from insufficient potassium intake, renal or gastrointestinal losses, or intracellular shifts. While the underlying cause is often easily identifiable, certain cases present diagnostic challenges, and if left unrecognized, the consequences can be life-threatening. We report a rare and atypical case of severe symptomatic hypokalemia as the initial presentation of newly diagnosed Graves' disease. The condition was caused by thyrotoxic periodic paralysis, a rare but serious complication of thyrotoxicosis, predominantly seen in East Asian populations. This disorder is characterized by episodes of acute, reversible muscle weakness associated with transient hypokalemia, which increases the risk of falls and traumatic injuries. The prompt identification of the etiology in such cases is critical for preventing recurrence and avoiding potentially fatal complications. [ABSTRACT FROM AUTHOR]

Background: Renal hypokalemia is associated with mutation. This study aimed to investigate the clinical features and pathogenic mutations in patients with renal hypokalemia. Methods: The patients with hypokalemia were enrolled, and the renal function, thyroid function, renin-aldosterone system, urinary potassium excretion, and exome sequencing were performed. The correlation between the clinical phenotypes and causative genes was assessed. Results: Five patients with hypokalemia were enrolled and diagnosed as tubular hypokalemia. The patients with common clinical manifestations were difficult to differentiate based on atypical laboratory findings. The results of the genetic analysis were as follows: both patient 1 and patient 2 were heterozygous for the c.C625T mutation of the KCNJ1 gene, which is responsible for Bartter syndrome. Patient 3 was heterozygous for the c.G298A mutation of the ATP6V1B1 gene, which is responsible for renal tubular acidosis. Patient 4 had a compound heterozygous mutation of c.G893A of the BSND gene, responsible for Bartter syndrome, and c.1029+5G>A, the ATP6V0A4 gene responsible for distal renal tubular acidosis. Patient 5 had Gitelman syndrome and carried the compound heterozygous mutations c.C1963T and c.G2029A of the SLC12A3 gene. All the above loci were known heterozygous mutations. Conclusions: The unusual heterozygous mutations were identified in five renal hypokalemia patients. Molecular diagnosis of tubular hypokalemia was conducive to accurate diagnosis and treatment. [ABSTRACT FROM AUTHOR]

PURPOSE: High-dose methotrexate (HD-MTX) is the backbone of curative therapy for CNS lymphoma. Because of toxicity, MTX is administered in the inpatient setting along with hyperhydration and monitoring until MTX clearance is documented (3-5 days). Frequent hospitalizations result in patient time away from work, home, and exposure to potential iatrogenic/nosocomial complications. Here, we aim to demonstrate feasibility of HD-MTX administration in the outpatient setting with low-dose glucarpidase facilitating clearance. METHODS: This is a prospective nonrandomized study of outpatient HD-MTX followed by glucarpidase 2000u (ClinicalTrials.gov identifier: NCT03684980). Eligible patients had CNS lymphoma, creatinine <1.3 mg/dL, and previously tolerated HD-MTX. Patients were enrolled between May 2020 December 2021 for one HD-MTX treatment. Patients could re-enroll for subsequent doses of HD-MTX as eligibility and slots permitted. MTX 3.5 g/m2 was administered once over 2 hours, preceded by standard hydration and followed by an additional 2 hours of dextrose 5% in water with NaHCO3 75 mEq at 150 cc/h. Glucarpidase 2000u was administered once in the clinic 24 hours later. The primary end point was MTX level 48 hours after HD-MTX. RESULTS: Twenty doses of outpatient HD-MTX with glucarpidase were administered to seven patients. After 20 of 20 (100%) treatments, serum MTX levels were reduced to <100 nmol/L. Treatments were well-tolerated, and no admissions were required. One patient received additional outpatient hydration for elevated creatinine. Development of antiglucarpidase antibody was rare and did not affect treatment. CONCLUSION: Outpatient HD-MTX with glucarpidase is safe and well-tolerated and has the potential to alter standard treatment for CNS lymphoma. CNS lymphoma patients received glucarpidase to avoid hospitalization for MTX therapy. [ABSTRACT FROM AUTHOR]

Purpose: To explore the clinical, muscle pathological, and pathogenic gene mutation characteristics of Andersen-Tawil Syndrome (ATS) and enhance the understanding of ATS among clinical practitioners. Methods: Retrospective analysis of clinical data and muscle pathology of two ATS families, along with genetic testing for probands and some family members. Results: In Family 1, spanning four generations, four individuals were affected, while Family 2 had two affected individuals across four generations. All six patients in both families experienced onset in childhood, presenting with periodic paralysis, arrhythmias, and craniofacial skeletal abnormalities. In Family 1, the proband's periodic paralysis was more triggered by low temperature and exercise, occurring several times a year, lasting 4-7 days. All three adult patients in Family 1 had a history of hypokalemia, and the frequency and severity of attacks were reduced after regular oral potassium supplement therapy. Two adult females in Family 1 experienced limb weakness triggered by stress, exertion, and premenstrual period, with milder symptoms than the proband. In Family 2, the proband's periodic paralysis typically occurred the day after excessive exertion, with a frequency of approximately 2-3 months. Two years prior, the proband developed arrhythmias without palpitations or chest tightness. The proband's brother experienced intermittent limb weakness during adolescence, remained untreated, and had sudden death at age 40. Physical examination revealed characteristic features in Family 1 and both probands: small mandible, wide eye spacing, and fifth-digit clinodactyly. Four adult patients were shorter in stature, while the growth status of a pediatric patient was indeterminate. Supplementary tests showed a history of hypokalemia during muscle weakness episodes in Family 1, while Family 2 patients had normal potassium levels during episodes. The long exercise tests were positive in both probands. Muscle MRI showed no significant abnormalities, but muscle pathology revealed rimmed vacuoles and tubular aggregates. Genetic testing identified KCNJ2 gene mutations in two probands and some of their family members, with c.407C > T (p.S136F) heterozygous mutation in Family 1 and c.652C > T (p.R218W) heterozygous mutation in Family 2. Conclusion: Among the clinical symptoms of the patients with Andersen-Tawil Syndrome in this study, not everyone exhibits the full triad of signs: periodic paralysis is the most common initial symptom, craniofacial and digit skeletal abnormalities are characteristic signs, and ventricular arrhythmias pose the most serious potential risk. Given that these typical symptoms were observed in 5 out of 6 patients, clinicians should pay special attention to these typical symptoms, and patients with these symptoms should be followed up over time. Muscle biopsy May reveal pathological changes such as tubular aggregates, but genetic testing for KCNJ gene mutations remains a crucial diagnostic criterion for this syndrome. [ABSTRACT FROM AUTHOR]

Background: A decreased glomerular filtration rate (GFR), estimated using creatinine (Cr– eGFR), is often found at the initial presentation of anorexia nervosa (AN). Its pathophysiology has been explained mainly by dehydration, and chronic hypokalemia is also thought to be a cause. However, because we have often experienced cases of AN with decreased Cr-eGFR without these conditions, we must consider different etiologies. The focus of this paper is on low free triiodothyronine (FT3) syndrome. We also discuss the utility of eGFR, estimated using cystatin-C (CysC-eGFR), for these patients. Methods: The data of 39 patients diagnosed with AN between January 2005 and December 2023 was available for study. The characteristics of patients at the lowest and highest body mass index standard deviation score (BMI-SDS) were examined. Data on the parameters Cr-eGFR, CysC-eGFR, dehydration markers, potassium (K), and hormonal data and BMI-SDS were assessed during the treatment course to evaluate the correlations in these parameters. Blood hematocrit, uric acid (UA), blood urine nitrogen (BUN) level, and urine specific gravity were adopted as dehydration markers; FT3, free thyroxine, thyroid stimulating hormone, and insulin-like growth factor were adopted as hormonal data. Cr-eGFR and simultaneously evaluated dehydration markers, K, or hormonal data were extracted and correlations associated with the changes in BMI-SDS were examined. Furthermore, Cr-eGFR and simultaneously assessed CysC-eGFR were compared. Results: When the BMI-SDS was at the lowest value, low-FT3 syndrome was shown. Severe hypokalemia was not found in our study. A linear relation was not found between Cr-eGFR and BMI-SDS. A statistically significant correlation was found between Cr-eGFR and FT3 (p = 0.0025). Among the dehydration markers, statistically significant correlations were found between Cr-eGFR and BUN or UA. The difference between Cr-eGFR and CysC-eGFR was prominent, and CysC-eGFR showed much higher values. Conclusions: Our data indicates that low-FT3 syndrome and dehydration were related to the renal function of our patients with AN. Furthermore, our data suggest that caution is needed in the interpretation of kidney function evaluation when using CysC-eGFR in cases of AN. [ABSTRACT FROM AUTHOR]

Plant AT-rich sequence and zinc-binding proteins (PLATZs) are a novel category of plant-specific transcription factors involved in growth, development, and abiotic stress responses. However, the PLATZ gene family has not been identified in barley. In this study, a total of 11 HvPLATZs were identified in barley, and they were unevenly distributed on five of the seven chromosomes. The phylogenetic tree, incorporating PLATZs from Arabidopsis, rice, maize, wheat, and barley, could be classified into six clusters, in which HvPLATZs are absent in Cluster VI. HvPLATZs exhibited conserved motif arrangements with a characteristic PLATZ domain. Two segmental duplication events were observed among HvPLATZs. All HvPLATZs were core genes present in 20 genotypes of the barley pan-genome. The HvPLATZ5 coding sequences were conserved among 20 barley genotypes, whereas HvPLATZ4/9/10 exhibited synonymous single nucleotide polymorphisms (SNPs); the remaining ones showed nonsynonymous variations. The expression of HvPLATZ2/3/8 was ubiquitous in various tissues, whereas HvPLATZ7 appeared transcriptionally silent; the remaining genes displayed tissue-specific expression. The expression of HvPLATZs was modulated by salt stress, potassium deficiency, and osmotic stress, with response patterns being time-, tissue-, and stress type-dependent. The heterologous expression of HvPLATZ3/5/6/8/9/10/11 in yeast enhanced tolerance to salt and osmotic stress, whereas the expression of HvPLATZ2 compromised tolerance. These results advance our comprehension and facilitate further functional characterization of HvPLATZs. [ABSTRACT FROM AUTHOR]

Background: Potassium (K) is an essential nutrient for plant growth and development. Maize (Zea mays) is a widely planted crops in the world and requires a huge amount of K fertilizer. Arbuscular mycorrhizal fungi (AMF) are closely related to the K uptake of maize. Genetic improvement of maize K utilization efficiency will require elucidating the molecular mechanisms of maize K uptake through the mycorrhizal pathway. Here, we employed transcriptome and gene family analysis to elucidate the mechanism influencing the K uptake and utilization efficiency of mycorrhizal maize. Methods and results: The transcriptomes of maize were studied with and without AMF inoculation and under different K conditions. AM symbiosis increased the K concentration and dry weight of maize plants. RNA sequencing revealed that genes associated with the activity of the apoplast and nutrient reservoir were significantly enriched in mycorrhizal roots under low-K conditions but not under high-K conditions. Weighted gene correlation network analysis revealed that three modules were strongly correlated with K content. Twenty-one hub genes enriched in pathways associated with glycerophospholipid metabolism, glycerolipid metabolism, starch and sucrose metabolism, and anthocyanin biosynthesis were further identified. In general, these hub genes were upregulated in AMF-colonized roots under low-K conditions. Additionally, the members of 14 gene families associated with K obtain were identified (ARF: 38, ILK: 4, RBOH: 12, RUPO: 20, MAPKK: 89, CBL: 14, CIPK: 44, CPK: 40, PIN: 10, MYB: 174, NPF: 79, KT: 19, HAK/HKT/KUP: 38, and CPA: 8) from maize. The transcript levels of these genes showed that 92 genes (ARF:6, CBL:5, CIPK:13, CPK:2, HAK/HKT/KUP:7, PIN:2, MYB:26, NPF:16, RBOH:1, MAPKK:12 and RUPO:2) were upregulated with AM symbiosis under low-K conditions. Conclusions: This study indicated that AMF increase the resistance of maize to low-K stress by regulating K uptake at the gene transcription level. Our findings provide a genome-level resource for the functional assignment of genes regulated by K treatment and AM symbiosis in K uptake-related gene families in maize. This may contribute to elucidate the molecular mechanisms of maize response to low K stress with AMF inoculation, and provided a theoretical basis for AMF application in the crop field. [ABSTRACT FROM AUTHOR]

The present study demonstrates that low concentrations of azelaic acid (AZA) significantly impact the metabolism of Arabidopsis thaliana seedlings, leading to imbalances in numerous minerals and metabolites due to AZA‐induced stress. Untargeted metabolomic analyses were conducted on untreated and AZA‐treated seedlings at two time points: 7 and 14 days after treatment initiation. The results revealed a general accumulation of sugars (e.g., glucose, mannose, xylose), amino acids (e.g., lysine, GABA, threonine, glutamine), and organic acids (e.g., glutaric acid, shikimic acid, succinic acid) in AZA treated‐seedlings, suggesting that AZA triggers stress responses in Arabidopsis. Ionomic analysis revealed that AZA induces phosphorus deficiency, which plants compensate by increasing malate content in the roots. Additionally, AZA treatment induced putrescine accumulation within the root, a metabolic biomarker of potassium deficiency and plant stress. The metabolomic profile showed elevated levels of different specialized metabolites, such as nitrogen‐ and sulphur‐containing compounds, and altered levels of various phytohormones, including jasmonates and brassinosteroids, implicated in plant protection under biotic and/or abiotic stresses. These findings support the hypothesis that AZA's mode of action is associated with an auxin imbalance, suggesting its function as an auxinic herbicide. The observed increases in starch and jasmonates, coupled with the disruptions in potassium homeostasis, are linked to the previously reported alterations in the auxin transport, root architecture and gravitropic root response. Statistical analyses were applied, including Kruskal‐Wallis tests for ionomic data, as well as multifactor analysis, Principal Component Analysis, Orthogonal Partial Least Squares‐Discriminant Analysis, and enrichment pathway analysis for metabolomic data, ensuring the robustness and validity of these findings. [ABSTRACT FROM AUTHOR]

Hypokalemic paralysis is a clinical syndrome characterized by acute flaccid paralysis with concomitant hypokalemia. Complications, such as acute respiratory failure and cardiac arrhythmias, can be fatal. If treated appropriately, the patient can recover without any sequelae. We present a rare case of life-threatening hypokalemic paralysis following the ingestion of an unknown substance. At presentation, her serum potassium concentration was 1.9 mmol/L. A review of the patient's history confirmed the ingestion of barium chloride. She was diagnosed with acute barium poisoning characterized by high serum and urine barium levels. Aggressive potassium repletion was administered intravenously and orally. Her serum potassium concentration dropped to 1.5 mmol/L and peaked at 5.4 mmol/L following treatment. The patient achieved a complete recovery and was discharged without sequelae. Barium can competitively block the potassium inward rectifier channels and interfere with the efflux of intracellular potassium, leading to severe hypokalemia. Our report illustrates a rare presentation of acute barium intoxication and a differential diagnosis indicating hypokalemic paralysis. We also discuss the pathophysiological features and compare the clinical findings with cases of rebound hyperkalemia. [ABSTRACT FROM AUTHOR]

In traditional farming, fertilizers are often used without precision, resulting in unnecessary expenses and potential damage to the environment. This study introduces a new method for accurately identifying macronutrient deficiencies in Rhodena lettuce crops. We have developed a four-stage process. First, we gathered two sets of data for lettuce seedlings: one is composed of color images and the other of point clouds. In the second stage, we employed the interactive closest point (ICP) method to align the point clouds and extract 3D morphology features for detecting nitrogen deficiencies using machine learning techniques. Next, we trained and compared multiple detection models to identify potassium deficiencies. Finally, we compared the outcomes with traditional lab tests and expert analysis. Our results show that the decision tree classifier achieved 90.87% accuracy in detecting nitrogen deficiencies, while YOLOv9c attained an mAP of 0.79 for identifying potassium deficiencies. This innovative approach has the potential to transform how we monitor and manage crop nutrition in agriculture. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Several electrocardiographic alterations due to hypokalemia have been described, but the electrocardiographic presentation meeting criteria for occlusion of the left main coronary artery is very rare. We describe a case of hypokalemia simulating it. [ABSTRACT FROM AUTHOR]

Dietary potassium deficiency causes stimulation of sodium reabsorption leading to an increased risk in blood pressure elevation. The distal convoluted tubule (DCT) is the main rheostat linking plasma K+ levels to the activity of the Na-Cl cotransporter (NCC). This occurs through basolateral membrane potential sensing by inwardly rectifying K+ channels (Kir4.1/5.1); decrease in intracellular Cl−; activation of WNK4 and interaction and phosphorylation of STE20/SPS1-related proline/alanine-rich kinase (SPAK); binding of calcium-binding protein 39 (cab39) adaptor protein to SPAK, leading to its trafficking to the apical membrane; and SPAK binding, phosphorylation, and activation of NCC. As kidney-specific with-no-lysine kinase 1 (WNK1) isoform (KS-WNK1) is another participant in this pathway, we examined its function in NCC regulation. We eliminated KS-WNK1 specifically in the DCT and demonstrated increased expression of WNK4 and long WNK1 (L-WNK1) and increased phosphorylation of NCC. As in other KS-WNK1 models, the mice were not hyperkalemic. Although wild-type mice under low-dietary K+ conditions demonstrated increased NCC phosphorylation, the phosphorylation levels of the transporter, already high in KS-WNK1, did not change under the low-K+ diet. Thus, in the absence of KS-WNK1, the transporter lost its sensitivity to low plasma K+. We also show that under low K+ conditions, in the absence of KS-WNK1, there was no formation of WNK bodies. These bodies were observed in adjacent segments, not affected by the targeting of KS-WNK1. As our data are overall consistent with those of the global KS-WNK1 knockout, they indicate that the DCT is the predominant segment affecting the salt transport regulated by KS-WNK1. NEW & NOTEWORTHY: In this paper, we show that KS-WNK1 is a critical component of the distal convoluted tubule (DCT) K+ switch pathway. Its deletion results in an inability of the DCT to sense changes in plasma potassium. Absence of KS-WNK1 leads to abnormally high levels of WNK4 and L-WNK1 in the DCT, resulting in increased Na-Cl phosphorylation and function. Our data are consistent with KS-WNK1 targeting WNK4 and L-WNK1 to degradation. [ABSTRACT FROM AUTHOR]

We aimed to evaluate the utility of simple, cost-effective, and non-invasive strategies alternative to BIPSS and peripheral CRH stimulation in differential diagnosis of ACTH-dependent CS. First, we performed ROC analysis to evaluate the performance of various tests for differential diagnosis of ACTH-dependent CS in our cohort (CD, n=76 and EAS, n=23) and derived their optimal cut-offs. Subsequently, combining various demographic (gender), clinical (hypokalemia), biochemical (plasma ACTH, HDDST, peripheral CRH stimulation) and imaging (MRI pituitary) parameters, we derived non-invasive models with 100% PPV for CD. Patients with pituitary macroadenoma (n=14) were excluded from the analysis involving non-invasive models. Relative percent ACTH (AUC: 0.933) and cortisol (AUC: 0.975) increase on peripheral CRH stimulation demonstrated excellent accuracy in discriminating CD from EAS. Best cut-offs for CD were plasma ACTH<97.3 pg/ml, HDDST≥57% cortisol suppression, CRH stimulation≥77% ACTH increase and≥11% cortisol increase. We derived six models that provided 100% PPV for CD and precluded the need for BIPPS in 35/85 (41.2%) patients with ACTH-dependent CS and no macroadenoma (in whom BIPSS would have otherwise been recommended). The first three models included basic parameters and avoided both peripheral CRH stimulation and BIPSS in 19 (22.4%) patients, while the next three models included peripheral CRH stimulation and avoided BIPSS in another 16 (18.8%) patients. Using simple and non-invasive alternative strategies, BIPSS can be avoided in 41% and peripheral CRH stimulation in 22% of patients with ACTH-dependent CS and no macroadenoma; such patients can be directly referred for a pituitary surgery. [ABSTRACT FROM AUTHOR]

Aim: This study was designed to investigate the association between Charlson Comorbidity Index (CCI) and in‐hospital mortality and other clinical outcomes among patients with hyperglycemic crises. Method: This retrospective cohort study was conducted using data from electric medical records. A total of 1668 diabetic patients with hyperglycemic crises from six tertiary hospitals met the inclusion criteria. CCI < 4 was defined as low CCI and CCI ≥ 4 was defined as high CCI. Propensity score matching (PSM) with the 1:1 nearest neighbour matching method and the caliper value of 0.02 was used to match the baseline characteristics of patients with high CCI and low CCI to reduce the confounding bias. In‐hospital mortality, ICU admission, hypoglycemia, hypokalemia, acute kidney injury, length of stay (LOS), and hospitalisation expense between low CCI and high CCI were compared and assessed. Univariate and multivariate regression were applied to estimate the impact of CCI on in‐hospital and other clinical outcomes. Outcome: One hundred twenty‐one hyperglycemic crisis (HC) patients died with a mortality rate of 7.3%. After PSM, compared with low CCI, patients with high CCI suffered higher in‐hospital mortality, ICU admission, LOS, and hospitalisation expenses. After multivariate regression, age (aOR: 1.12, 95% confidence interval [CI]: 1.06−1.18, p < 0.001), CCI(aOR: 4.42, 95% CI: 1.56−12.53, p = 0.005), uninsured (aOR: 22.32, 95% CI: 4.26−116.94, p < 0.001), shock (aOR: 10.57, 95% CI: 1.41−79.09, p = 0.022), mechanical ventilation (aOR: 75.29, 95% CI: 12.37−458.28, p < 0.001), and hypertension (aOR: 4.34, 95% CI: 1.37−13.82, p = 0.013) were independent risk factors of in‐hospital mortality of HC patients. Besides, high CCI was an independent risk factor for higher ICU Admission (aOR: 5.91, 95% CI: 2.31−15.08, p < 0.001), hypoglycemia (aOR: 2.19, 95% CI:1.01−4.08, p = 0.049), longer LOS (aOR: 1.23, 95% CI: 1.19−2.27, p = 0.021), and higher hospitalisation expense (aOR: 2089.97, 95% CI: 193.33−3988.61, p = 0.031) of HC patients. Conclusion: CCI is associated with in‐hospital mortality, ICU admission, hypoglycemia, LOS, and hospitalisation expense of HC patients. CCI could be an ideal indicator to identify, monitor, and manage chronic comorbidities among HC patients. [ABSTRACT FROM AUTHOR]

Abstract Background Late-onset type II Bartter syndrome is an exceedingly rare condition, with only six documented cases presenting symptoms and signs beyond infancy. We report a unique case of late-onset type II Bartter syndrome with an atypical presentation and clinical course following chemotherapy treatment during childhood. Case presentation A 10-year-old boy, diagnosed with hepatoblastoma at age 2 and treated with cisplatin and epirubicin, presented with polyuria, polydipsia, failure to thrive, and electrolyte imbalances. He exhibited hypokalemia, metabolic alkalosis, and elevated urinary excretion of sodium, chloride, calcium, and magnesium. Whole exome sequencing and Sanger sequencing identified compound heterozygous variants in the KCNJ1 gene, confirming the diagnosis of type II Bartter syndrome. The patient’s clinical presentation was distinct from previously reported cases, with an absence of nephrocalcinosis, unusually small and hyperechoic kidneys, and a substantial decline in kidney function. Treatment included oral potassium supplementation, spironolactone, and angiotensin-converting enzyme inhibitors. Conclusions This case highlights the importance of considering late-onset Bartter syndrome in patients with a history of chemotherapy presenting with persistent electrolyte imbalances and ongoing renal dysfunction. The atypical features and rapid progression of chronic kidney disease in this patient may be attributed to the deleterious nature of the identified variants and the potential impact of previous chemotherapy on kidney susceptibility to damage. Careful monitoring and management of electrolyte imbalances and renal function are crucial in such cases.

Abstract Background Brugada syndrome (BrS) is associated with an increased risk of sudden death caused by ventricular arrhythmias. The characteristic electrocardiographic appearance of ST-segment elevation of more than 2 mm with coved-type morphology in more than 1 right precordial lead is seen. Hypokalemia is known to unmask the Brugada type-1 pattern, but its exact role and mechanisms in this context are not well understood. Case presentation We report a case of first-time diagnosis of BrS in a 51-year-old man with hypokalemia 2.8 mmol/L. Despite the normalization of potassium levels with potassium chloride (KCL), the Brugada type-1 pattern persisted on ECG. Interestingly, the corrected QT interval was shorter during hypokalemia (QTc 390 ms) compared to when potassium levels were normal (QTc 432 ms). Conclusions This case highlights that hypokalemia can unmask the Brugada type-1 electrocardiographic pattern, but does not alter it once unmasked. The observed shorter QT interval during hypokalemia challenges the assumption that QT prolongation is the sole mechanism by which hypokalemia influences Brugada syndrome. This underscores the need for further research into additional mechanisms by which hypokalemia might trigger ventricular arrhythmias in Brugada syndrome.

Abstract Background The two classic manifestations of primary aldosteronism are hypertension and hypokalemia. However, acute respiratory failure due to hypokalemia in primary hyperaldosteronism is rare. Case presentation The patient was a 27-year-old female who presented with drowsiness and weakness in all extremities. She had been diagnosed with hypertension three years prior, with irregular follow-up, and had a history of preeclampsia one year later. She exhibited high blood pressure and severe hypokalemia (2 mEq/L), leading to respiratory depression and impending respiratory arrest. Consequently, the patient was intubated and transferred to the intensive care unit (ICU). She also developed rhabdomyolysis. Blood pressure tests, including hormonal tests (aldosterone: 13.2 ng/dL, plasma renin activity: 0.32 ng/mL/h), were conducted. Due to the high aldosterone-renin ratio, an abdominopelvic computed tomography (CT) scan was performed. The CT scan revealed a 14 × 12 mm round mass with a washout value above 60%, consistent with an adrenal adenoma, leading to a diagnosis of primary aldosteronism. The patient was discharged after stabilization, and one and a half months after ICU admission, a laparoscopic left adrenalectomy was successfully performed without post-operative complications. Histopathology showed encapsulated hypertrophy of the adrenal cortex with a predominance of large clear cells, confirming the diagnosis of adrenal adenoma. At the most recent follow-up, the patient had normal potassium levels, was normotensive without any medications, and exhibited no alarming signs or symptoms. Conclusion Respiratory depression to the extent of impending respiratory failure and rhabdomyolysis as a result of hypokalemia in primary aldosteronism are extremely rare. In this patient, who developed respiratory depression due to resistant hypokalemia, timely investigation of secondary causes and diagnosis of adrenal adenoma were crucial. The surgery provided definitive treatment for the patient’s blood pressure and prevented the recurrence of life-threatening complications.

Introduction: Periodic paralysis is a condition that causes recurrent episodes of flaccid paralysis, and it can be primary or secondary. Hypokalemic periodic paralysis is the most common type of primary periodic paralysis, and it is inherited through autosomal dominant gene transmission. Males are affected three times more often than females, and the paralysis attacks usually occur at night after a period of vigorous exercise. It is crucial to exclude other diagnostic entities based on the nature of presentation, physical examination, and paraclinical studies. Thyrotoxic periodic paralysis is more prevalent in Asian or Hispanic males with thyrotoxicosis, where up to 10% of thyrotoxic patients may experience periodic paralysis. Case Presentations: Here, we present 6 cases of patients who came to our care with varying degrees of muscle weakness, each showing interesting and diverse laboratory results. Conclusion: In patient assessment, it is crucial to consider social and family history. Even without this information, awareness of potential diagnoses is vital. The cause should be carefully considered for possible simple treatments. Failing to recognize and address this condition promptly could lead to severe outcomes. Timely identification and intervention are essential for effective disease management and patient welfare.

Abstract Background Primary aldosteronism is characterized by high plasma aldosterone and low renin. The plasma aldosterone-to-renin ratio is recommended for screening. Severe hydronephrosis leads to renal parenchymal ischemia, resulting in increased renin secretion. Since nonsuppression of renin may cause a negative result in the aldosterone-to-renin ratio test, severe hydronephrosis and primary aldosteronism occurring simultaneously in a patient are challenging to diagnose. Case presentation A 54-year-old Chinese man of Han ethnicity was diagnosed with hypertension and severe hypokalemia (minimum 1.57 mmol/L) 13 years prior, and was also diagnosed with severe hydronephrosis due to congenital ureteral stenosis on the left side. His clinical features suggested primary aldosteronism, but the aldosterone-to-renin ratio result of the patient was negative every time he underwent the primary aldosteronism screening test. No further treatment for primary aldosteronism was performed, which led the patient to suffer from severe hypokalemia, such that he was taking 12–15 g/day potassium chloride orally to keep his blood potassium between 3.0 and 3.5 mmol/L (reference value, 3.5–5.5 mmol/L) for 13 years, and the patient needed to be hospitalized in the intensive care unit for rescue several times. At admission, although the aldosterone-to-renin ratio result of the patient was negative, we still did the saline stress test and captopril inhibition test, and the results showed that the plasma aldosterone level was not lower after the test than before the test. Adrenal enhanced computed tomography suggested an adenoma in the left adrenal gland, and the results of adrenal vein sampling suggested that the left side was the dominant side. Therefore, laparoscopic total resection of the left adrenal gland was performed, and 2 weeks later, the patient developed short-term renal function impairment and hyperkalemia, but his renal function and blood potassium returned to normal after treatment that included fluid rehydration. The patient’s biochemical test results and clinical symptoms were completely normal after 1 year. Conclusion We suggest that for patients with a high suspicion of primary aldosteronism in the clinic, comprehensive analysis must be performed in combination with clinical characteristic assessments, such as severe hydronephrosis, if renin is within the normal range or if the aldosterone-to-renin ratio result is negative at screening and diagnostic tests, and adrenal vein sampling should be performed if necessary. It can help avoid misdiagnoses and contribute to the treatment of patients with severe hydronephrosis and primary aldosteronism.

Abstract Background A decreased glomerular filtration rate (GFR), estimated using creatinine (Cr– eGFR), is often found at the initial presentation of anorexia nervosa (AN). Its pathophysiology has been explained mainly by dehydration, and chronic hypokalemia is also thought to be a cause. However, because we have often experienced cases of AN with decreased Cr-eGFR without these conditions, we must consider different etiologies. The focus of this paper is on low free triiodothyronine (FT3) syndrome. We also discuss the utility of eGFR, estimated using cystatin-C (CysC-eGFR), for these patients. Methods The data of 39 patients diagnosed with AN between January 2005 and December 2023 was available for study. The characteristics of patients at the lowest and highest body mass index standard deviation score (BMI-SDS) were examined. Data on the parameters Cr-eGFR, CysC-eGFR, dehydration markers, potassium (K), and hormonal data and BMI-SDS were assessed during the treatment course to evaluate the correlations in these parameters. Blood hematocrit, uric acid (UA), blood urine nitrogen (BUN) level, and urine specific gravity were adopted as dehydration markers; FT3, free thyroxine, thyroid stimulating hormone, and insulin-like growth factor were adopted as hormonal data. Cr-eGFR and simultaneously evaluated dehydration markers, K, or hormonal data were extracted and correlations associated with the changes in BMI-SDS were examined. Furthermore, Cr-eGFR and simultaneously assessed CysC-eGFR were compared. Results When the BMI-SDS was at the lowest value, low-FT3 syndrome was shown. Severe hypokalemia was not found in our study. A linear relation was not found between Cr-eGFR and BMI-SDS. A statistically significant correlation was found between Cr-eGFR and FT3 (p = 0.0025). Among the dehydration markers, statistically significant correlations were found between Cr-eGFR and BUN or UA. The difference between Cr-eGFR and CysC-eGFR was prominent, and CysC-eGFR showed much higher values. Conclusions Our data indicates that low-FT3 syndrome and dehydration were related to the renal function of our patients with AN. Furthermore, our data suggest that caution is needed in the interpretation of kidney function evaluation when using CysC-eGFR in cases of AN.

Abstract Background Familial hypokalemic periodic paralysis (HypoPP) is an uncommon genetic disorder characterized by recurrent episodes of muscle weakness and hypokalemia, typically starting in early adulthood. The existence of hyperthyroidism in the presence of HypoPP is more strongly associated with a diagnosis of thyrotoxic periodic paralysis (TPP), with most cases occurring in Asian males with pathogenic KCNJ2 or KCNJ18 variants and without a family history of the condition. This case is novel due to the combination of familial HypoPP and hyperthyroidism induced by Graves’ disease, a rare occurrence especially in non-Asian populations. Case presentation A 40-year-old African American man presented with profound muscle weakness after consuming a high-salt meal. He had a significant family history of hyperthyroidism and hypokalemia. On examination, he showed profound weakness in all extremities. Laboratory tests confirmed hypokalemia and hyperthyroidism, and genetic testing identified a pathogenic variant in the CACNA1S gene (c.1583 G > A, p. R528H), with normal SCN4A, KCNJ2 and KCNJ18 sequencing. He was diagnosed with familial HypoPP and hyperthyroidism due to Graves’ disease. He was started on PO methimazole 10 mg three times a day and PO acetazolamide 250 mg twice a day. He was advised to follow a low carbohydrate and low salt diet. Conclusions This case highlights the importance of considering a genetic basis for HypoPP in patients with a family history of the condition, even when hyperthyroidism is present. The combination of familial HypoPP and Graves’ disease is rare and emphasizes the need for careful genetic and clinical evaluation in similar cases. Management should focus on correcting hypokalemia, treating hyperthyroidism, and lifestyle modifications to prevent recurrence.

Abstract This article provides a comprehensive overview of electrolyte and water homeostasis in pediatric patients, focusing on some of the common serum electrolyte abnormalities encountered in clinical practice. Understanding pathophysiology, taking a detailed history, performing comprehensive physical examinations, and ordering basic laboratory investigations are essential for the timely proper management of these conditions. We will discuss the pathophysiology, clinical manifestations, diagnostic approaches, and treatment strategies for each electrolyte disorder. This article aims to enhance the clinical approach to pediatric patients with electrolyte imbalance-related emergencies, ultimately improving patient outcomes. Trial registration This manuscript does not include a clinical trial; instead, it provides an updated review of literature.

Abstract Background Hypokalemic rhabdomyolysis is a rare clinical manifestation of primary aldosteronism, making its diagnosis challenging, particularly when it becomes the primary presenting symptom. Herein, we present a case of primary aldosteronism with hypokalemic rhabdomyolysis and conduct a related literature review. Case presentation We report the case of a 54-year-old Chinese male patient who presented with intermittent weakness over the past year and was admitted with sudden limb paralysis for 2 days. The final diagnosis was primary aldosteronism accompanied by hypokalemic rhabdomyolysis syndrome. By reviewing the related Chinese and English literature, we noticed that only a few cases were published since 1978. After excluding irrelevant literatures, we summarized and analyzed 43 patients of with primary aldosteronism accompanied by hypokalemic rhabdomyolysis syndrome. All patients showed good recovery, with normalized blood potassium levels, and a majority achieved normalized blood pressure. Some patients still required medication for blood pressure control. Conclusions Primary aldosteronism rarely causes rhabdomyolysis; the occurrence of severe hypokalemia and rhabdomyolysis should prompt consideration of primary aldosteronism in the differential diagnosis. Early detection and treatment are crucial for determining patient prognosis.

Abstract Hypokalemia is a common issue in clinical settings, often indicating underlying systemic conditions that require careful evaluation. This study presents three cases where hypokalemic paralysis served as the initial symptom of systemic diseases. Key evaluation methods included clinical history, physical examination focusing on volume status, and acid–base assessment. The cases highlight the diverse etiologies of hypokalemia, including primary hyperaldosteronism, Sjogren’s syndrome, and Crohn’s disease.

Objective: Rare disease. Background: Gitelman syndrome (GS) is an uncommon autosomal recessive inherited disease caused by inactivating mutations in the SLC12A3 gene located on chromosome 16q13, resulting in distal tubular dysfunction. Most cases are detected during routine examinations in adulthood, due to hypokalemia and alkalosis. GS needs to be distinguished from diseases that cause hypokalemia, such as Classic Bartter syndrome and hyperthyroidism. In individual cases, GS and hyperthyroidism occur simultaneously, which is prone to misdiagnosis. Case Report: A 51-year-old woman with intermittent palpitations and lower limb fatigue for 4 years received a diagnosis of hypokalemia at a local hospital. Treatment with potassium supplementation did not improve the patient's palpitations and fatigue. After coming to our hospital for examination, it was found that the patient had hyperthyroidism. After receiving treatment of hyperthyroidism remission and sufficient potassium replacement, the patient's serum potassium level remained low. Meanwhile, the patient had hypomagnesemia and metabolic alkalosis. Subsequently, according to our suggestion, the patient continued to take oral supplements of potassium and magnesium, while also started on spironolactone. We convinced the patient to undergo genetic testing and discovered compound heterozygous mutations in the SLC12A3 gene, which presented a definitive diagnosis of GS. In the following 3 months, the patient's serum potassium level was within the normal range, and the dose of methimazole was reduced. Conclusion: As a rare disease, GS may have only mild or occasional manifestations, making it prone to misdiagnosis. GS remains therapeutically challenging, and future progress in treatment will depend on further research of the disease. [ABSTRACT FROM AUTHOR]

This article provides a comprehensive overview of electrolyte and water homeostasis in pediatric patients, focusing on some of the common serum electrolyte abnormalities encountered in clinical practice. Understanding pathophysiology, taking a detailed history, performing comprehensive physical examinations, and ordering basic laboratory investigations are essential for the timely proper management of these conditions. We will discuss the pathophysiology, clinical manifestations, diagnostic approaches, and treatment strategies for each electrolyte disorder. This article aims to enhance the clinical approach to pediatric patients with electrolyte imbalance-related emergencies, ultimately improving patient outcomes. Trial registration This manuscript does not include a clinical trial; instead, it provides an updated review of literature. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to describe the successful identification and treatment of severe hyperkalemia, cardiac arrhythmia, rhabdomyolysis, and acute kidney injury (AKI) in a domestic cat that underwent general anesthesia for abdominal exploratory surgery. The definitive underlying cause remains unknown; however, a reaction to propofol is suspected. Case summary: A 6-month-old intact male domestic short-hair cat underwent general anesthesia and developed severe intraoperative rhabdomyolysis, hyperkalemia, ventricular fibrillation, and AKI during surgery despite a documented mild hypokalemia and normal creatinine before inducing anesthesia. Propofol was administered as part of the anesthetic protocol. The patient was resuscitated successfully and responded well to advanced medical intervention. The hyperkalemia and AKI were resolved within less than 24 h from surgery and rhabdomyolysis was resolved at the time of recheck 5 days later. New or unique information provided: While previously suspected in dogs, to the authors' knowledge, propofol-related infusion syndrome (PRIS) has not been reported in domestic cats. Veterinary professionals should be aware that drug-induced intraoperative rhabdomyolysis and hyperkalemia can develop unexpectedly and should remain a differential for acute cardiac arrhythmias or cardiac arrest and AKI. [ABSTRACT FROM AUTHOR]

Cystic fibrosis (CF) is the most common life-threatening genetic disease in the United States and among people of European descent. Despite the widespread distribution of the cystic fibrosis transmembrane conductance regulator (CFTR) along kidney tubules, specific renal phenotypes attributable to CF have not been well documented. Recent studies have demonstrated the downregulation of the apical Cl-/HCO3 - exchanger pendrin (Slc26a4) in kidney B-intercalated cells of CF mouse models. These studies have shown that kidneys of both mice and humans with CF have an impaired ability to excrete excess HCO3 -, thus developing metabolic alkalosis when subjected to excess HCO3 - intake. The purpose of this minireview is to discuss the latest advances on the role of pendrin as a molecule with dual critical roles in acid base regulation and systemic vascular volume homeostasis, specifically in CF. Given the immense prevalence of vascular volume depletion, which is primarily precipitated via enhanced chloride loss through perspiration, we suggest that the dominant presentation of metabolic alkalosis in CF is due to the impaired function of pendrin, which plays a critical role in systemic vascular volume and acid base homeostasis. [ABSTRACT FROM AUTHOR]

Background: Hypokalemic rhabdomyolysis is a rare clinical manifestation of primary aldosteronism, making its diagnosis challenging, particularly when it becomes the primary presenting symptom. Herein, we present a case of primary aldosteronism with hypokalemic rhabdomyolysis and conduct a related literature review. Case presentation: We report the case of a 54-year-old Chinese male patient who presented with intermittent weakness over the past year and was admitted with sudden limb paralysis for 2 days. The final diagnosis was primary aldosteronism accompanied by hypokalemic rhabdomyolysis syndrome. By reviewing the related Chinese and English literature, we noticed that only a few cases were published since 1978. After excluding irrelevant literatures, we summarized and analyzed 43 patients of with primary aldosteronism accompanied by hypokalemic rhabdomyolysis syndrome. All patients showed good recovery, with normalized blood potassium levels, and a majority achieved normalized blood pressure. Some patients still required medication for blood pressure control. Conclusions: Primary aldosteronism rarely causes rhabdomyolysis; the occurrence of severe hypokalemia and rhabdomyolysis should prompt consideration of primary aldosteronism in the differential diagnosis. Early detection and treatment are crucial for determining patient prognosis. [ABSTRACT FROM AUTHOR]

Background: Syndrome of apparent mineralocorticoid excess (AME) is characterized by excessive MR stimulation despite low levels of aldosterone. 11Beta-hydroxysteroid dehydrogenase-2 (11βDSH-2) inactivates cortisol to cortisone, preventing cortisol-induced MR activation. Genetic defects in 11βDSH-2 cause AME through accumulation of cortisol in the distal nephron, leading to MR activation induced hypertension, hypokalemia and metabolic alkalosis. Acquired AME can occur due to the ingestion of glycyrrhizic acid, found in licorice root, which inhibits 11βDSH-2 and has additional effects on cortisol homeostasis through inhibition of 11βDSH-1. Case report: We present a case of acquired AME with a hyperadrenergic symptoms induced by ingestion of Advanced Liver Support, a nutritional supplement produced by Advanced BioNutritionals(R), in a 65-year-old Caucasian female who presented with accelerated hypertension, hypokalemia, metabolic alkalosis and adrenergic symptoms. Cessation of the licorice-containing supplement resulted in complete resolution of the patient's hypertension, symptoms and abnormal lab values. To our knowledge this is the first reported case of AME from this supplement, and the first to describe accompanying hyperadrenergic symptoms. Conclusions: Glycyrrhizic acid is increasingly being found in unregulated nutritional supplements and has the potential to induce a reversable syndrome of AME. Acquired AME should be suspected in individuals who present with hypertension along with hypokalemia, metabolic alkalosis and low plasma renin and serum aldosterone levels. [ABSTRACT FROM AUTHOR]