Respiratory Muscle Weakness in Thyrotoxic Periodic Palsy: A Lesson to Remember

Thyrotoxic periodic palsy (TPP) is a sporadic form of hypokalemic periodic palsy that may occur in association with hyperthyroidism mostly with Graves’ disease. Acute thyrotoxic periodic palsy is a disorder most commonly seen in Asian men and characterized by abrupt onset of hypokalemia and paralysis. The disorder primarily affects the lower extremities and can involve all four limbs and presents as acute flaccid paralysis. The diagnosis of thyrotoxic periodic palsy is not difficult, but the disease's low incidence and many differentials for acute flaccid paralysis delay and complicate the diagnosis. TPP is not related to the etiology, severity, and duration of thyrotoxicosis. The treatment is similar to hypokalemic periodic palsy with potassium supplementation and initiation of antithyroid drugs and beta-blocker therapy. Here a similar case of quadriparesis is reported, which got precipitated after abrupt cessation of carbimazole in a young male. This initially was thought to be a case of hypokalemic periodic palsy and was later diagnosed to be TPP and recovered after initiating antithyroid drugs and potassium supplementation.

A novel hypokalaemic polymyopathy and subsequent unrelated nutritional thiamine deficiency in a young Burmese cat

Case summary An 8-month-old female spayed Burmese cat was referred for investigation of reduced appetite, reluctance to walk and jump and amaurosis. On serum biochemistry there was severe hypokalaemia and marked elevation of creatine kinase, suggestive of hypokalaemic polymyopathy. The neurological signs were consistent with thiamine deficiency. The cat was negative for the periodic hypokalaemic polymyopathy (PHP) of Burmese cats, and was ultimately diagnosed with a previously undescribed potassium wasting nephropathy requiring ongoing oral potassium supplementation. The response to treatment was excellent and the cat has remained clinically normal over a 12-month follow-up period. Relevance and novel information PHP in Burmese cats has been well described, but all cases to date have been shown to be secondary to a genetic mutation in WNK4, resulting in potassium wasting into the urine. This is the first case report of another potassium wasting nephropathy in a young Burmese cat, with subsequent development of nutritional thiamine deficiency.

Association between potassium supplementation and the occurrence of acute kidney injury in patients with hypokalemia administered liposomal amphotericin B: a nationwide observational study

Background Hypokalemia and acute kidney injury (AKI) occur in patients administered liposomal amphotericin B (L-AMB), a wide-spectrum anti-fungicidal drug. However, the association between potassium supplementation and the occurrence of AKI in patients with hypokalemia who were administered L-AMB is not well understood. Methods Using nationwide claims data and laboratory data, the occurrence of AKI during L-AMB treatment was retrospectively compared between patients with hypokalemia who were or were not supplemented with potassium and between those adequately or inadequately supplemented with potassium (serum potassium levels corrected to ≥3.5 mEq/L or remained < 3.5 mEq/L, respectively) before or after L-AMB treatment initiation. Results We identified 118 patients who developed hypokalemia before L-AMB treatment initiation (43 received potassium supplementation [25 adequate and 18 inadequate supplementation] and 75 did not receive potassium supplementation), and 117 patients who developed hypokalemia after L-AMB initiation (79 received potassium supplementation [including 23 adequate and 15 inadequate supplementation] and 38 did not receive potassium supplementation). The occurrence of any stage of AKI was similar between patients with hypokalemia, regardless of potassium supplementation (i.e., before L-AMB treatment initiation [supplementation, 51%; non-supplementation, 45%; P = 0.570] or after L-AMB initiation [supplementation, 28%; non-supplementation, 32%; P = 0.671]). After adjusting for confounding factors, we found that the occurrence of any stage of AKI was not associated with potassium supplementation before L-AMB initiation (odds ratio [OR]: 1.291, 95% confidence interval [CI]: 0.584–2.852, P = 0.528) or after L-AMB initiation (OR: 0.954, 95% CI: 0.400–2.275, P = 0.915). The occurrence of any stage of AKI tended to decline in patients with hypokalemia who were adequately supplemented with potassium (44%) before, but not after, L-AMB initiation relative to that in patients inadequately supplemented with potassium (61%), however this result was not significant (P = 0.358). Conclusion Potassium supplementation was not associated with any stage of AKI in patients with hypokalemia who were administered L-AMB.

Weight Loss-Based Nutraceuticals

Thyrotoxic periodic paralysis (TPP) is characterized by muscle weakness, areflexia, and hypokalemia in the setting of thyrotoxicosis. We present the case of a 32-year-old male with multiple presentations to the emergency department for lower limb weakness, tremors, diaphoresis, and tachycardia. His initial blood work revealed T3-toxicosis and hypokalemia, and he was treated for TPP with intravenous fluids and potassium supplementation. He had been ingesting weight loss supplements containing iodine, kelp, licorice, and likely undeclared thyroid hormones or mimics. Following discontinuation of supplements, all laboratory investigations normalized and thyrotoxicosis symptoms resolved. This case illustrates that ingestion of thyroid hormone-based nutraceuticals should be considered as a cause of thyrotoxicosis and TPP. RésuméLa paralysie périodique thyréotoxique (PPT) se caractérise par de la faiblesse musculaire, une aréflexie et une hypokaliémie dans le contexte de la thyréotoxicose. Nous exposons le cas d’un homme de 32 ans qui s’est présenté au service des urgences pour de multiples symptômes, soit une faiblesse des membres inférieurs, des tremblements, une diaphorèse et une tachycardie. Son bilan sanguin initial a révélé une toxicose-T3 et une hypokaliémie, et il a été traité contre la PPT par des solutés intraveineux et une recharge en potassium. Il ingérait des suppléments pour la perte de poids contenant de l’iode, de la laminaire, de la réglisse et probablement des hormones thyroïdiennes ou leurs analogues non déclarés. Après l’arrêt des suppléments, tous les examens de laboratoire sont revenus à la normale et les symptômes de thyréotoxicose ont disparu. Ce cas montre que l’ingestion de nutraceutiques à base d’hormones thyroïdiennes devrait être considérée comme une cause de la thyréotoxicose et de la PPT.

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

MO053GITELMAN'S SYNDROME AND PREGNANCY: TWO SUCCESSFUL CASE REPORTS

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 &lt; 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&gt;T (Thr60Met)] were identified. The woman’s father and sister had a heterozygous c.179C&gt;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 &lt; 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&gt;T (Thr60Met), c.658G&gt;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.

Modelling the Cost-Effectiveness of Implementing a Dietary Intervention in Renal Transplant Recipients

Background: The Dietary Approach to Stop Hypertension (DASH) and potassium supplementation have been shown to reduce the risk of death with a functioning graft (DWFG) and renal graft failure in renal transplant recipients (RTR). Unfortunately, a key problem for patients is the adherence to these diets. The aim of this study is to evaluate the cost-effectiveness and budget impact of higher adherence to either the DASH or potassium supplementation. Methods: A Markov model was used to simulate the life course of 1000 RTR in the Netherlands. A societal perspective with a lifetime time horizon was used. The potential effect of improvement of dietary adherence was modelled in different scenarios. The primary outcomes are the incremental cost-effectiveness ratio (ICER) and the budget impact. Results: In the base case, improved adherence to the DASH diet saved 27,934,786 and gained 1880 quality-adjusted life years (QALYs). Improved adherence to potassium supplementation saved €1,217,803 and gained 2901 QALYs. Both resulted in dominant ICERs. The budget impact over a five-year period for the entire Dutch RTR population was €8,144,693. Conclusion: Improving dietary adherence in RTR is likely to be cost-saving and highly likely to be cost-effective compared to the current standard of care in the Netherlands.

A 29-year-old Bodybuilder with Liothyronine-induced Thyrotoxic Hypokalaemic Periodic Paralysis

We describe a 29-year-old male bodybuilder with recurrent attacks of myalgia and muscle weakness associated with hypokalaemia and thyrotoxicosis due to abuse of liothyronine. The attacks quickly resolved after potassium supplementation and liothyronine cessation. We concluded that the patient had thyrotoxic hypokalaemic periodic paralysis (TPP). Although muscle weakness and hypokalaemia are prominent symptoms of TPP, underlying thyrotoxicosis may be overlooked. Up to 25% of androgen abusers also abuse thyroid hormone. Lack of recognition of thyroid hormone abuse as a cause of hypokalaemic periodic paralysis may result in unnecessary, potentially harmful medical investigations and improper treatment and advice.