Oral Potassium Supplementation Research Articles

Persistent renal dysfunction post-chemotherapy: a diagnostic conundrum in pediatric cancer survivorship – a case report

BackgroundLate-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 presentationA 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.ConclusionsThis 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.

Pseudo-Hyperaldosteronism Arising from Licorice Cough Syrup Self-Ingestion: A Case Report

Background: Licorice (glycyrrhiza glabra) cough syrup intoxication is manifested with refractory hypokalemia, hypertension, and metabolic alkalosis. The transformation of glycyrrhiza glabra metabolic into glycyrrhetic acid after ingestion further inhibits the 11-β-hydroxysteroid dehydrogenase-2 enzyme, impeding the conversion of cortisol into cortisone. The accumulation of cortisol can also stimulate mineralocorticoid receptors, which leads to a pseudo-hyperaldosteronism-like effect. Case Presentation: We report a 60-year-old male patient with licorice intoxication due to the chronic consumption of licorice cough syrup. He exhibited a transient seizure lasting approximately one minute. Initially, hypokalemia (potassium level was 2.0 mmol/L), metabolic alkalosis, and QT interval prolongation with premature ventricular complexes were demonstrated on his electrocardiogram. Despite the administration of both intravenous and oral potassium supplements over two days, there was no significant improvement in hypokalemia. Spironolactone, an aldosterone receptor antagonist, was administered in addition to ongoing potassium supplementation from the 3rd day. This intervention led to a rapid normalization of hypokalemia in one day. The patient was ultimately discharged on the 6th day without any subsequent complications. Conclusions: The licorice-induced chronic intoxication, which led to pseudo-hyperaldosteronism and refractory hypokalemia, was successfully managed with aggressive potassium supplementation and spironolactone treatment.

7901 Clinical Challenges Of Paraneoplastic Endocrine Metabolic Syndromes: An Illustrative Example

Abstract Disclosure: S. Shankar: None. S.S. Sundar: None. M.S. Vaishnav: None. K. Thummala: None. L. Lekkala: None. S. Srikanta: None. K. Muniraj: None. T. Deepak: None. R.B. Vijay: None. V. Nath: None. C. Siddlingappa: None. P. Ravikumar: None. M.D. Chitra: None. Introduction: Paraneoplastic endocrine syndromes result from aberrant production by tumors of protein hormones, hormone precursors, or hormone like substances. In patients who are already receiving treatment for cancer, the onset of a paraneoplastic syndrome may be an indication of progression or relapse. Clinical Case: 2021: Age 81 female (hypertension). Carcinoma rectum; S/P surgery, colostomy, chemotherapy and radiotherapy. 2023: Palliative care, due to progressive metastatic (hepatic, pulmonary, bone) malignancy. 2023 Aug: Hospital admission for generalized weakness, not able to walk, severe pain and low-grade fever 10 days. A: Paraneoplastic Hyponatremia: Evaluation: S Sodium= 127, 129, 129, 137, 134 mmol/L (134-145); Spot urine sodium= 65 mmol; S Uric acid= 3.5 mg/dL (4.4-7.6); S Cortisol 8 am= 15.5 (6.7-22.6); T3 total= 0.34 ng/mL (0.97-1.69), T4 total= 6.87 mcg/dL (5.5-11), TSH= 1.87 mIU/mL (0.46-4.68); LH= 0.35 mIU/mL (16-54); FSH= 1.9 mIU/mL (23-116); S calcium= 8.9 mg/dL (8.4-10.2); S Phosphorus= 2.31mg/dL (2.5-4.5) Diagnosis: Possible paraneoplastic ADH secretion/ SIADH; pain/ narcotics-induced hypothalamic ADH secretion. Treatment: Fluid restriction, liberal salt intake, 3% saline; oral sodium bicarbonate and tolvaptan; tapentadol. B: Paraneoplastic Leukemoid reaction: Evaluation: Total leucocyte count= 29960, 36670, 44130, 58450 cells/mm3 (4000-11,000); Differential count: Neutrophils= 85-94%; Lymphocytes= 10-4%; CRP= 93.7, 24.0 mg/dL (<0.3); Procalcitonin= 0.097, 0.083 ng/mL (<0.1); Ruled out significant sepsis; no obvious laboratory or imaging evidence of any infectious or leukemic etiology. Diagnosis: Progressive leucocytosis due to Granulocyte Macrophage Colony Stimulating Factor (GM-CSF) secretion from various neoplasms has been reported in literature. Treatment: Empirical antibiotic coverage. C: Paraneoplastic Hypokalemia: Evaluation: S Potassium= 3.3, 3.4, 3.7, 2.9, 3.0 mmol/L (3.5-5.1). Diagnosis: Hypokalemia likely due to (in vivo and in vitro) intracellular potassium uptake by rapidly proliferating leukocytes (and neoplasm- primary and metastatic). (Ectopic ACTH secretion / ruled out). Therapy: IV and oral potassium supplements. D: Paraneoplastic Fever: Cytokines (e.g., tumor necrosis factor and interleukin-1) release from tumor cells or infiltrating mononuclear cells. Clinical Lessons: Endocrine paraneoplastic syndromes can complicate patient’s clinical course, response to treatment, impact prognosis and even be confused as metastatic spread or another “non-existent” clinical entity. They can precede, occur concomitantly or present at later stage of tumour development. Recognition of the diverse clinical manifestations improves clinical outcomes (earlier cancer diagnosis, better quality of life, optimal tumour-directed therapy or decision towards non-aggressive focussed palliative care). Presentation: 6/2/2024

Clinical, myopathological, and genetic features of two Chinese families with Andersen-Tawil syndrome.

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. Retrospective analysis of clinical data and muscle pathology of two ATS families, along with genetic testing for probands and some family members. 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. 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.

LAPORAN KASUS: HIPOKALEMIA PERIODIK PARALISIS

Hypokalemic periodic paralysis is one form of rare periodic paralysis that can result in sudden weakness. Comprehensive examination is required to determine the management in such cases. This case report discusses a 26-year-old woman who presented to the hospital with a sudden complaint of weakness in all limbs and no neurological deficits. The patient had a history of being admitted three times in the past year with the same complaint. Laboratory results revealed significantly low potassium levels. Although rare, periodic paralysis must be promptly distinguished from other causes of weakness and paralysis. Immediate and appropriate actions are crucial. After rapid administration of potassium through a central vein, the limb weakness disappeared. The patient had a history of three admissions with severe hypokalemia. Abdominal ultrasound revealed mild right hydronephrosis. In this case, the patient fully recovered after receiving both oral and intravenous potassium supplementation with strict ECG monitoring in the ICU. The patient returned to the ward after 2 days in the ICU and was subsequently referred to a nephrology specialist for the management of the observed hydronephrosis. This is important to identify the underlying cause of recurrent hypokalemic paralysis

A Severe Case of Hypokalemic Metabolic Alkalosis: A Quiz

A Severe Case of Hypokalemic Metabolic Alkalosis: A Quiz

JS-NG-9: PRIMARY HYPERALDOSTERONISM IN A TRIPLET PREGNANCY: CONSIDERATIONS FOR INTRA-PARTUM DIAGNOSIS AND MANAGEMENT

Primary aldosteronism (PA) is an uncommon etiology of hypertension. We report the first known case of PA in a triplet pregnancy to describe the intra-partum considerations in diagnosis, investigation and management of PA. A 33-year-old female G3P0A2 at 10 weeks gestation with triplet pregnancy was admitted for hyperemesis gravidarum and hypertension. Her comorbidities include chronic hypertension, uterine fibroids, and two previous pregnancy losses. Home medications are labetalol 100 mg BID. On presentation, the patient complained of nausea, vomiting and epigastric pain and headache. On examination, her blood pressure was 161/90 and heart rate of 75. The rest of the cardiopulmonary exam was normal. Laboratory values were notable for potassium:2.9 mmol/L (n 3.5–5mmol/L) and bicarbonate 26 mmol/L (n 24–31mmol/L). The platelets, liver function tests and renal function tests were normal. Despite continuous high dose oral and parenteral high dose potassium supplementations, she had hypokalemic episodes (nadir 2.7mmol/L). She remained hypertensive at 140/90 on labetalol 200 mg QID and nifedipine XL 30 mg q12 hours. PA was suspected and testing revealed aldosterone 1280 (n 166–2885), renin 0.49 (n 0.10–1.10), aldosterone-renin ratio (ARR) 2615 (n < 1500), which is indeterminate. Abdominal MRI demonstrated right adrenal mass, measuring 2.7 x 5.2 x 2.9 cm. Repeat testing revealed aldosterone 11,000, renin 0.34, ARR > 30,000, which is highly suggestive of PA. The uterus was too bulky with the triplet pregnancy and uterine fibroids to safely proceed with adrenalectomy. The patient consented to receive eplerenone 25 mg BID for medical management of PA in addition to nefidipine XL 60 mg BID and oral potassium supplementation. She re-presented to hospital at 31 weeks with spontaneous rupture of membranes. She had an emergency caesarian section and delivery three healthy male infants. At one month postpartum, she was switched to spironolactone 25 mg BID from eplerenone, in addition to nefidipine and potassium supplementation, and maintained blood pressure of 130–140/80–90. At five months postpartum, she had testing on doxazosin 4 mg daily, hydralazine 10 mg TID and potassium supplementations, which revealed aldosterone 2360 pmol/L, renin < 2, ARR > 1200. Adrenal vein sampling demonstrated marked lateralization of aldosterone production to the right adrenal gland. A right laparoscopic adrenalectomy was completed and she achieved normotension on post-operative day 2. She was discharged on no antihypertensive medication or potassium supplementation. At 8 months post-adrenalectomy, her blood pressure is 120/96 potassium is 4mmol/L and her aldosterone levels remain low at 252 on no medications. In this case of triplet pregnancy, the diagnosis of PA was difficult to establish given the impact of pregnancy on the renin-angiotensin-aldosterone-system. Treatment with eplerenone resulted in improvement of hypertension and good pregnancy outcome. Postpartum, adrenalectomy resulted in rapid and sustained improvement in hypertension and reversal of hypokalemia.

PSAT026 A Treatable but Commonly Missed Etiology of Cerebrovascular Disease in Younger Adults; A Case of Primary Hyperaldosteronism Presented with Hemorrhagic Stroke.

Abstract Introduction Primary hyperaldosteronism is the leading cause of secondary hypertension. Although PH is an independent risk factor for acute stroke, it seems to be underdiagnosed as a cause of hypertensive emergencies. Here we discuss a case of a patient who presented with hypertensive emergency and stroke-like symptoms, eventually found to have hyperaldosteronism. Case Presentation A case of a 46-year-old male with a past medical history of uncontrolled hypertension and hypokalemia was admitted for acute onset of left-sided numbness and weakness associated with slurred speech. On admission, blood pressure was 292/156mmHg; physical exam revealed flaccid paralysis on the left upper and lower extremities, decreased sensation to light touch, and pinprick in the left side of the body. Initial potassium was 2.2mmol/L. CT scan of the brain revealed intracranial hematoma in the right basal ganglia, which brain MRI confirmed. The patient was first started on strict blood pressure control with IV medications and transitioned to oral antihypertensive and potassium supplementation. Given resistant hypokalemia, plasma renin activity and serum aldosterone levels were ordered and revealed to be 0.771ng/mL/hr. and 24.4ng/dL with a ratio >20 (31.26). The patient was started on spironolactone, and CT abdomen and pelvis with contrast revealed a 1.8cm nodule of the left adrenal. The patient's blood pressure and electrolyte abnormalities improved. Further evaluation with adrenal vein sampling to localize the functional adrenal tumor and possible surgery deferred until his neurological deficits improve. Discussion This case report emphasizes the consequences of primary hyperaldosteronism given that the aldosterone excess has detrimental cardiovascular and cerebrovascular effects that are in part independent of high blood pressure. Early detection of a disease that was previously believed to be rare has an essential impact on clinical outcomes and survival, especially in the younger population. Studies have shown that the overall risk of stroke was almost three times higher than that of patients with essential hypertension. Despite this elevated risk, the most significant problem in underdiagnosis is that the screening test is often not even performed. Screening guidelines for primary hyperaldosteronism consist of serum aldosterone/renin ratio; only done when there is suspicion of a secondary hypertension causes. The diagnosis of PH should not only rest on endocrinologists but also on primary care providers, who are initially the point of contact when hypertension is first detected. Conclusion PH is an identified risk factor for CVA; however, its diagnosis can be challenging if the CVA is the initial feature. This case sheds light on considering PH among the differential diagnosis when facing younger patients presenting with CVA in the setting of persistent hypertension and hypokalemia. Once diagnosed and adequately treated, it decreases the risk of CVA and avoids further complications. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

PSAT026 A Treatable but Commonly Missed Etiology of Cerebrovascular Disease in Younger Adults: A Case of Primary Hyperaldosteronism Presented with Hemorrhagic Stroke

Abstract Introduction Primary hyperaldosteronism is the leading cause of secondary hypertension. Although PH is an independent risk factor for acute stroke, it seems to be underdiagnosed as a cause of hypertensive emergencies. Here we discuss a case of a patient who presented with hypertensive emergency and stroke-like symptoms, eventually found to have hyperaldosteronism. Case Presentation A case of a 46-year-old male with a past medical history of uncontrolled hypertension and hypokalemia was admitted for acute onset of left-sided numbness and weakness associated with slurred speech. On admission, blood pressure was 292/156mmHg; physical exam revealed flaccid paralysis on the left upper and lower extremities, decreased sensation to light touch, and pinprick in the left side of the body. Initial potassium was 2.2mmol/L. CT scan of the brain revealed intracranial hematoma in the right basal ganglia, which brain MRI confirmed. The patient was first started on strict blood pressure control with IV medications and transitioned to oral antihypertensive and potassium supplementation. Given resistant hypokalemia, plasma renin activity and serum aldosterone levels were ordered and revealed to be 0.771ng/mL/hr. and 24.4ng/dL with a ratio >20 (31.26). The patient was started on spironolactone, and CT abdomen and pelvis with contrast revealed a 1.8cm nodule of the left adrenal. The patient's blood pressure and electrolyte abnormalities improved. Further evaluation with adrenal vein sampling to localize the functional adrenal tumor and possible surgery deferred until his neurological deficits improve. Discussion This case report emphasizes the consequences of primary hyperaldosteronism given that the aldosterone excess has detrimental cardiovascular and cerebrovascular effects that are in part independent of high blood pressure. Early detection of a disease that was previously believed to be rare has an essential impact on clinical outcomes and survival, especially in the younger population. Studies have shown that the overall risk of stroke was almost three times higher than that of patients with essential hypertension. Despite this elevated risk, the most significant problem in underdiagnosis is that the screening test is often not even performed. Screening guidelines for primary hyperaldosteronism consist of serum aldosterone/renin ratio; only done when there is suspicion of a secondary hypertension causes. The diagnosis of PH should not only rest on endocrinologists but also on primary care providers, who are initially the point of contact when hypertension is first detected. Conclusion PH is an identified risk factor for CVA; however, its diagnosis can be challenging if the CVA is the initial feature. This case sheds light on considering PH among the differential diagnosis when facing younger patients presenting with CVA in the setting of persistent hypertension and hypokalemia. Once diagnosed and adequately treated, it decreases the risk of CVA and avoids further complications. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

Difficulties in the management of hypokalemia in a pregnant patient with Gitelman syndrome.

Gitelman syndrome (GS) is a rare renal disorder, and little is known about its impact on pregnancy. We report the successful outcome of pregnancy in a patient with GS that was managed with aggressive oral and intravenous potassium supplementation.

Bartter Syndrome in A Nigerian Child with Recurrent Vomiting

Bartter syndrome is a rare renal tubular disease characterized by hypokalemic metabolic alkalosis and renal salt wasting. There is paucity of report of this condition from Africa. We report a case of a four–year–old Nigerian boy who presented with growth failure, recurrent episodes of vomiting, features of severe dehydration and normal blood pressure warranting in–hospital care on several occasions over a period of 16 months. This patient had hypokalemia, hypochloremia, hyponatraemia and hypercalciuria. He also had abdominal x-ray findings of a triangular-shaped calcific density in the right renal bed suggestive of right medullary nephrocalcinosis. Diagnosis of Bartter syndrome was confirmed by elevated plasma renin of 8.1ng/ml/hr (normal 0.5 – 5.9). The patient was managed with intravenous normal saline, potassium chloride infusion, calcium gluconate, and then oral potassium, calcium and magnesium supplements. Follow up at the clinic revealed significant weight gain and stable clinical state. A high index of suspicion is needed for early diagnosis of this rare syndrome, particularly in children presenting with persistent vomiting, failure to thrive and dyselectrolytemia. This, invariably, will improve survival and health-related quality of life of such children, hence the need for this report.

Abstract 135: A case of acquired Gitelman syndrome presenting as hypokalemic paralysis and AKI

Introduction: Gitelman syndrome is usually an inherited disorder, it is induced by defect in the thiazide sensitive Na –Cl co-transporter in distal tubules. Case reports on hypokalemia, AKI secondary to GS has been discussed in present case report.Clinical Case: A 18 year old female referred from department of General Medicine as a case of AKI having recurrent hypokalemia. She gave history of hypokalemic paralysis. There was no potential cause of dehydration such as diarrhoea, vomiting, nephrotoxic agent intake or contrast exposure. Family history was negative. During current hospitalization, she was normotensive with normal renal function test. Other electrolyte levels including calcium and routine laboratory parameters were determined to be normal. Kidney dimensions and echogenicity were determined to be normal without hydronephrosis in the urinary system as oer USG finding. Further workup for hypokalemia revealed urine potassium creatinine ratio (17 mmol/g), ABG (pH 7.44, bicarbonate 26.5 mmol/l), urine chloride (111.3 mmol/l) and urine calcium creatinine ratio (0.05 mmol/mmol) respectively with serum Mg (1.7 mg/dl) on lower range. Based on above findings, a diagnosis of Gitelman syndrome has been made. Autoimmune etiology were ruled out. These finding suggested that patient had AKI secondary to hypokalemia. She was started on oral potassium supplementation and magnesium supplementation.Conclusion: GS is a rare tubulopathy transmitted as an autosomal recessive trait. It is caused by mutation in thiazide sensitive Na Cl co-transporter coding gene, SLC12A3. Patients may be referred with hypokalemia, hypomagnesemia and metabolic alkalosis associated muscular weakness and cramping. By contrast, worsening hypokalemia and profound volume depletion as the predisposing factors for ischemic AKI appears to coexist.

Gitelman Syndrome in a 32-Years-Old Female Patient

Introduction. Gitelman syndrome is an autosomal recessive disorder that is milder than Bartter syndrome and is often not diagnosed until late childhood or even adulthood. However, this syndrome is usually symptomatic and can result in serious clinical manifestations, such as muscle spasms that can be severe and involve the hands and feet. These symptoms are frequently observed in almost all patients, partly due to hypokalemia and hypomagnesemia.
 Case presentation. A 32-year-old female patient is being treated in the Internal Medicine ward of Dr. M. Djamil Padang General Hospital with the main complaint of cramping of the left hand, which has gotten worse since 1 day ago. The patient also complained that both legs were prone to cramping that went on and on since 1 month ago. The patient had been hospitalized with the same complaint and is currently taking slow release potassium (KSR) medication. Examination of the patient's limbs revealed a positive trousseau sign and carpopedal spasms. Laboratory examination of the patient showed potassium levels of 1.8 mmol/L, calcium levels of 5.7 mg/dl, Magnesium levels of 0.8 mg/dl, and electrolyte disturbances in the patient's urine. ECG results revealed a prolonged QT interval.
 Conclusion. Gitelman syndrome is an autosomal recessive disorder and often goes undiagnosed. However, this syndrome is usually symptomatic and can lead to serious clinical manifestations. Most patients require oral potassium and magnesium supplementation, as drug therapy is usually not fully effective.

Avoiding The Severe Gastrointestinal Side Effects Associated with High Dose Potassium and Magnesium Supplementation with Gitelman’s Syndrome Through Utilization of Canagliflozin, Aliskiren and Spironolactone

Gitelman’s Syndrome is an inherited renal tubulopathy that is characterized by renal potassium and magnesium wasting leading to hypokalemia and hypomagnesemia. Previously the only available therapies for the hypokalemia and hypomagnesemia associated with Gitelman’s Syndrome were high dose oral potassium and magnesium supplements. With the availability of the SGLT-2 receptor blocker canagliflozin the need for magnesium replacement therapy can be minimized. Similarly, utilizing blockade of the renin and mineralocorticoid receptors with aliskiren and spironolactone respectively the need for oral potassium replacement can be avoided. By eliminating or reducing the need for oral potassium and magnesium replacement therapy, gastrointestinal problems, particularly diarrhea, can be avoided and the quality of life improved while achieving normal serum potassium and magnesium levels. In addition, by normalizing serum potassium and particularly by correcting serum magnesium levels, the risk of a cardiac arrythmia is also reduced

A life-threatening case of pseudo-aldosteronism secondary to excessive liquorice ingestion

BackgroundLiquorice is found in many food products, soft drinks, and herbal medicines. Liquorice ingestion is an uncommon cause of apparent mineralocorticoid excess or pseudo-aldosteronism. The mechanism involves the inhibition of 11-beta-hydroxysteroid dehydrogenase type-2 by the active ingredient called glycyrrhizin. This leads to the uninhibited activation of mineralocorticoid receptors by cortisol. Confectionary products that contain liquorice are readily available in many countries around the world.Case presentationWe report a case of severe refractory hypokalaemia with hypertensive crisis and acute pulmonary oedema due to excessive liquorice consumption. A 79-year-old female presented to the emergency department following a road traffic accident. She described feeling weak and dizzy while driving before the collision. She attended her general practitioner (GP) several weeks earlier for fatigue and was being managed for hypokalaemia on oral potassium supplements. Investigations revealed hypertension (BP 180/69 mmHg), severe hypokalaemia (K 2.2 mmol/l), normal renal function, normal serum magnesium with metabolic alkalosis. Spot urinary potassium was 22 mmol/l. The patient denied taking medications including over-the-counter or herbal medication that can cause hypokalaemia. Hypokalaemia persisted despite aggressive intravenous (i.v.) and oral potassium replacement. She later developed a hypertensive crisis (BP 239/114 mmHg) with pulmonary oedema. She required admission to the intensive care unit and was managed with intravenous furosemide infusion and isosorbide dinitrate infusion. On further discussion, our patient admitted to struggling with nicotine cravings since quitting smoking two months earlier. She began eating an excessive amount of liquorice sweets to manage her cravings. Suppression of plasma renin and aldosterone supported the diagnosis of apparent mineralocorticoid excess secondary to excessive liquorice consumption. Her symptoms and hypokalaemia resolved after stopping liquorice intake.ConclusionsThis case highlights the life-threatening and refractory nature of hypokalaemia secondary to excessive liquorice consumption. This case also emphasizes the importance of comprehensive history taking including dietary habits. Increased awareness among the public is required regarding the potential health hazards of excessive liquorice consumption.

MO053GITELMAN'S SYNDROME AND PREGNANCY: TWO SUCCESSFUL CASE REPORTS

Abstract Background and Aims Gitelman’s Syndrome (GS) is a rare autosomal recessive hereditary salt-losing tubulopathy characterized by hypokalemic metabolic alkalosis with hypomagnesemia and hypocalciuria. Pregnancy in women with GS often aggravates hypokalemia and hypomagnesemia. However, there are few reports of pregnancies in GS. Here, we report the course of two Chinese women who were diagnosed as GS during pregnancy in 2019 and 2020 respectively. Method Case 1: A 21-year-old woman was referred to our hospital at 9 weeks gestation of her first pregnancy. She had complained of muscle weakness and cramps for one year. Before the referral she was diagnosed as hypokalemia and treated by oral potassium supplementation. However, her symptoms became severer after pregnancy. Case 2: A 20-year-old woman was admitted to the hospital because of elevated plasma glucose level and hypokalemia at 27 weeks gestation of her first pregnancy. The woman was asymptomatic and denied history of chronic diseases. The laboratory examinations were taken after admission. Genetic testing was conducted for pathogenic mutations in SLC12A3 (GS) and SLC12A1, KCNJ1, CLCKNB and BSND (Bartter syndrome 1-4). Results Case 1: Initial biochemistry examinations revealed hypokalemia (2.3 mmol/L, normal range 3.5-5.3 mmol/L) with inappropriate renal potassium wasting (urine potassium 254 mmol/24h, normal range < 20 mmol/24h), alkalosis (arterial blood gas pH 7.49), hypomagnesemia (0.55 mmol/L, normal range 0.67-1.04 mmol/L), hypocalciuria (urine calcium 1.6 mmol/24h, normal range 2.5-7.5 mmol/24h) and elevated renin (276 pg/ml, normal range 4-24 pg/ml) and aldosterone (825 pg/ml, normal range 10-160 pg/ml) levels. The blood pressure was normal-low (97/68 mmHg, 12.9/9.0 kPa) and the renal ultrasound was normal. Homozygous mutations [c.179C>T (Thr60Met)] were identified. The woman’s father and sister had a heterozygous c.179C>T, but had no electrolyte disorders. After the treatment of oral potassium supplementation (KCl 3g tid) and spironolactone (40mg bid), her serum potassium level increased to 3.4-4.0 mmol/L and muscle weakness was relieved. The woman delivered a healthy female infant weighing 2600 g at 39 weeks gestation via cesarean section. Maternal serum potassium level remained normal and no symptoms reoccured after delivery. Case 2: Initial biochemistry examinations identified hypokalemia (2.3 mmol/L, normal range 3.5-5.3 mmol/L) with inappropriate renal potassium wasting (urine potassium 81 mmol/24h, normal range < 20 mmol/24h), hypomagnesemia (0.49 mmol/L, normal range 0.67-1.04 mmol/L), hypocalciuria (urine calcium 0.3 mmol/24h, normal range 2.5-7.5 mmol/24h) and elevated renin (54 pg/ml, normal range 4-24 pg/ml) and aldosterone (834 pg/ml, normal range 10-160 pg/ml) levels. The blood pressure and renal ultrasound were normal. Heterozygous mutations [c.179C>T (Thr60Met), c.658G>A (Gly220Ser)] were identified. The woman was treated by oral potassium supplementation (KCl 3g tid) and her serum potassium level maintained normal during pregnancy. She had a normal delivery of a healthy female infant weighing 3050 g at 40 weeks gestation. After delivery she discontinued oral potassium supplementation and her serum potassium level ranged from 3.0-3.4 mmol/L without symptoms. Conclusion The outcome of mother and fetus of GS pregnancies appears favorable. Intensive monitoring of electrolyte levels and sufficient electrolyte supplementation are advised during pregnancy.

A Rare Case Report of Acquired Bartters With Positive Anti Scl70 Antibody

Introduction: Bartter syndrome autosomal recessive renal tubular disorders usualy presents in infant age group or antenatallyCase Description64 years housewife, a known case of hypothyroidism with in emergency with generalized weakness and hypotension. She had an small adrenal nodule work up which showed that it was non functional. On evaluation at time of stress (blood pressure 70/50) her serum cortisol was 300nmol/l and she was therefore started on hydrocortisone. Her records revealed hypokalemia for last 2 years. Workup of hypokalemia showed kaliuresis, metabolic alkalosis, hypercalciuria and excess chloride excretion in the urine, suggestive of bartter’s syndrome. But this needed further confirmation as bartter’s is rare in this age group and also the test was done at the time of stress. She was started on spironolactone and oral potassium supplementation. CECT head done for evaluation of postural dizziness showed chronic cortical venous thrombosis. On follow up ACTH stimulated cortisol was done which showed normal cortisol level so steroid was stopped. She was readmitted one month later with hypokalemia(K-1.8 meq/l) while being on same dosage of spironolactone and potassium. Workup of hypokalemia showed same feature of bartter’s syndrome. Considering the chain of events, a suggestion of bartter’s and response to steroid, a diagnosis of acquired autoimmune bartter’s syndrome along with hypothyroidism was made. An autoimmune work up showed anti SCL 70 positive2+(++). Patient responded to steroid, spironolactone, indomethacin and oral potassium supplementation. Discussion: Bartter’s syndrome can rarely happen in old age too. Seek for autoimmune cause once we suspect acquired bartter’s. To best of our knowledge previously only one case has been reported of acquired Bartter with systemic sclerosis. Patient may develop systemic sclerosis in future as anti SCL70 antibody is very specific for it. It is positive in less than 1 % of general population. Anti SCL70 antibody is rarely positive in Sjogrens syndrome. Our patient did not feature of sjogren or systemic sclerosis even after 4 years follow up

Primary Hyperaldosteronism in Pregnancy: A Case Report

Background: Primary aldosteronism (PA) is a renin-independent hypersecretion of aldosterone that remains an underdiagnosed etiology of hypertension. Less than 50 cases of primary hyperaldosteronism in pregnancy have been reported in literature that are associated with pregnancy complications including preeclampsia. Clinical Case: A 41-year-old female G9P4 at 32 weeks gestation was admitted for suspected preeclampsia due to elevated blood pressure during a routine prenatal visit. Her comorbidities include type 2 diabetes, uncontrolled hypertension, and preeclampsia in a previous pregnancy. Home medications included Lisinopril 10 mg once daily which was discontinued upon pregnancy and switched to Labetalol 400 mg twice daily and Nifedipine ER 30 mg daily. She was on a total of 220 units of insulin daily.On presentation, patient complained of anxiety but denied any headache, nausea, visual disturbances, chest pain, palpitations, muscle cramps, or fatigue. Her blood pressure was 180/100 mmHg with heat rate of 73. Laboratory values was notable for potassium: 2.8 mmol/L (n 3.5–5 mmol/L), bicarbonate: 28 mmol/L (n 24–31 mmol/L), and magnesium: 1.4 mEq/L (n 1.3–1.9 mEq/L). Liver function test and platelet count were normal. Spot protein-creatinine ratio was 0.3 (n 0–0.2). ECG was normal sinus rhythm with a heart rate of 82 without decreased T wave amplitude or U waves. She received three doses of oral 40 mEq potassium chloride over twelve hours and potassium level post repletion was 3.0 mmol/L.Initially, her hypokalemia was attributed to high insulin dose requirements. Despite down titration of insulin, patient continued to have hypokalemic episodes (nadir 2.7 mmol/L) refractory to oral and parenteral potassium supplementation. She remained hypertensive despite Labetalol 600 mg every 8 hours, Nifedipine ER 30 mg every 12 hours, with as needed hydralazine 10 mg IV pushes, and underwent urgent cesarean delivery. Uncontrolled hypertension persisted postpartum with recurrent episodes of hypokalemia. Further investigations revealed a plasma aldosterone concentration (PAC) and plasma renin activity (PRA) levels of 43.5 ng/dL (n <10 ng/dL) and 1.67 ng/mL/hr (n 0.17–5.38 ng/mL/hr) respectively, potassium at time of testing was 3.6 mmol/L. PAC to PRA ratio was greater than 20:1 with an absolute PAC greater than 15 ng/dL, which is highly suggestive of PA. Conclusion: We present a case of suspected PA with spontaneous hypokalemia potentially masked by the use of ACE inhibitors uncovered during pregnancy. Given the changes to the renin-angiotensin-aldosterone system (RAAS) during pregnancy, the diagnosis of PA is difficult to establish during gestation. Still, little is known on how to optimally treat these patients. Reference: Morton, A., 2015. Primary Aldosteronism and Pregnancy. Pregnancy and hypertension. An International Journal of Women’s Cardiovascular Health, Volume 5 Issue 4, 259–262.

Prevalence of hypokalemia in older persons: results from the PolSenior national survey

Key summary pointsAimTo examine what is the prevalence of hypokalemia in the older population.FindingsThe prevalence of hypokalemia was similar in older persons and younger ones. Hypokalemia is more often found in patients with arterial hypertension treated with diuretics. Oral supplementation of potassium in these patients did not prevent hypokalemia.MessageIn older persons, diuretic treatment is the most important cause of hypokalemia. Prevention of hypokalemia with the use of oral potassium supplementation is in this population insufficient.

Primary aldosteronism: from diagnosis to therapy in clinical practice

Primary aldosteronism (PA) is the single most common cause of secondary hypertension and is associated with increased target organ injury. It can be can either surgically cured or treated with targeted pharmacotherapy. PA is frequently undiagnosed and untreated, leading to aldosterone-specific cardiovascular morbidity and nephrotoxicity. Thus, clinicians should perform case detection testing for PA at least once in all patients with hypertension. The diagnostic work-up of PA is a sequence of three phases comprising screening tests, confirmatory tests and the differentiation of unilateral from bilateral forms. With appropriate surgical expertise, laparoscopic unilateral adrenalectomy is safe, efficient and curative in patients with unilateral adrenal disease. In patients who have bilateral aldosterone hypersecretion, the optimal management is a low sodium diet and lifelong treatment with a mineralocorticoid receptor antagonist administered at a dosage to maintain a high-normal serum potassium concentration without the aid of oral potassium supplements. In patients with PA, specific treatment provides prognostic benefit over optimal antihypertensive therapy and is therefore crucial to reduce mortality and morbidity in this subgroup of patients with hypertension.