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Hypokalemic paralysis

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https://www.readbyqxmd.com/read/29907477/successful-treatment-of-normokalemic-periodic-paralysis-with-hydrochlorothiazide
#1
Yuichi Akaba, Satoru Takahashi, Yoshiaki Sasaki, Hiroki Kajino
BACKGROUND: Periodic paralysis (PP) is an autosomal dominant muscle disorder characterized by periodic muscle weakness attacks associated with serum potassium level variations. It is classified into hypokalemic (hypoKPP), hyperkalemic (hyperKPP), and normokalemic (normoKPP) forms based on the ictal serum potassium level. HyperKPP and normoKPP are caused by mutations of the same gene SCN4A, the gene encoding the skeletal muscle voltage-gated sodium channel. Prophylactic treatment with thiazide diuretics is highly effective in preventing attacks in hyperKPP...
June 12, 2018: Brain & Development
https://www.readbyqxmd.com/read/29896753/-hypokalemic-periodic-paralysis-a-pedigree-with-eight-cases
#2
Jiaxiu Yan, Jun Han, Ting Wu, Guohua Wang
No abstract text is available yet for this article.
June 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/29875526/managing-bipolar-affective-disorder-in-a-tribal-district-of-odisha
#3
Dheeraj Kattula, Jayaprakash Russell Ravan, Munaf Babajan Nandyal
Background: Managing any chronic illness in marginalized communities in resource-poor settings is always a challenge. Lack of facility to monitor lithium and the common morbidity of hypokalemic periodic paralysis and chronic renal failure among tribals of northern part of Odisha pose unique challenges in managing bipolar disorder. Methodology: This is a cross-sectional study done in a district-level hospital catering to predominantly tribal population. A part of the data was collected by a psychiatrist prospectively and analyzed...
May 2018: Indian Journal of Psychological Medicine
https://www.readbyqxmd.com/read/29871829/changing-management-guidelines-in-thyrotoxic-hypokalemic-periodic-paralysis
#4
Matthew Correia, Mark Darocki, Eva Tovar Hirashima
BACKGROUND: Periodic paralysis is a rare complication of hyperthyroidism. Patients of East Asian descent are most commonly affected. Presentation is characterized by recurrent episodes of painless, abrupt-onset weakness, with laboratory evaluation characterized by profound hypokalemia. Underlying hyperthyroidism may not be clinically evident, but differentiation from the familial variant is critical due to differing treatment pathways. CASE REPORT: We describe the presentation of a 22-year-old man with recurrent relapsing-remitting weakness with undiagnosed hyperthyroidism...
June 2, 2018: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29774167/complete-hypokalemic-quadriparesis-as-a-first-presentation-of-sj%C3%A3-gren-syndrome
#5
Jason An, Branko Braam
Rationale: We hope to increase awareness that hypokalemic paralysis may be the first presentation of Sjögren syndrome, for which potassium-sparing diuretics can be an effective adjunct to potassium replenishment. Presenting concerns: A 73-year-old female presented to a peripheral hospital with quadriparesis and a critically low serum potassium of 1.6 mmol/L with U waves on the electrocardiogram (ECG). The initial arterial blood gas showed a pH of 7.19, bicarbonate of 13 mEq/L, and a CO2 of 35 mm Hg...
2018: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/29674667/a-mixed-periodic-paralysis-myotonia-mutant-p1158s-imparts-ph-sensitivity-in-skeletal-muscle-voltage-gated-sodium-channels
#6
Mohammad-Reza Ghovanloo, Mena Abdelsayed, Colin H Peters, Peter C Ruben
Skeletal muscle channelopathies, many of which are inherited as autosomal dominant mutations, include myotonia and periodic paralysis. Myotonia is defined by a delayed relaxation after muscular contraction, whereas periodic paralysis is defined by episodic attacks of weakness. One sub-type of periodic paralysis, known as hypokalemic periodic paralysis (hypoPP), is associated with low potassium levels. Interestingly, the P1158S missense mutant, located in the third domain S4-S5 linker of the "skeletal muscle", Nav1...
April 19, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29667944/proximal-muscle-weakness-with-overlying-hypokalemic-periodic-paralysis-in-sj%C3%A3-gren-syndrome-report-of-6-cases
#7
Vimal K Paliwal, Anmol Singh Rai, Surendra Kumar, Ritu Verma, Vikas Agarwal
No abstract text is available yet for this article.
April 5, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29657223/distal-renal-tubular-acidosis-in-sj%C3%A3-gren-s-syndrome
#8
Ram Narayan, Mansoor C Abdulla, Jemshad Alungal, N C Krishnadas
Interstitial nephritis and immune complex-mediated glomerulonephritis are the two common renal manifestations of primary Sjögren's syndrome (SS). Here, we discuss three cases of primary SS where presenting manifestation was distal renal tubular acidosis. The possibility of an underlying autoimmune disorder should be considered in a patient presenting with distal tubular acidosis or recurrent hypokalemic periodic paralysis as treatment of primary disease improves the outcome of illness.
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29606556/prevalence-and-mutation-spectrum-of-skeletal-muscle-channelopathies-in-the-netherlands
#9
B C Stunnenberg, J Raaphorst, J C W Deenen, T P Links, A A Wilde, D J Verbove, E J Kamsteeg, A van den Wijngaard, C G Faber, G J van der Wilt, B G M van Engelen, G Drost, H B Ginjaar
Few reliable data exist on the prevalence of skeletal muscle channelopathies. We determined the minimum point prevalence of genetically-defined skeletal muscle channelopathies in the Netherlands and report their mutation spectrum. Minimum point prevalence rates were calculated as number of genetically-confirmed skeletal muscle channelopathy patients (CLCN1, SCN4A, CACNA1S and KCNJ2 gene mutations) in the Netherlands (1990-2015) divided by the total number of at-risk individuals. Rates were expressed as cases/100...
May 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29584616/phenotypic-presentation-of-adolescents-with-overt-primary-hypothyroidism
#10
Nandini Devru, Pramila Dharmshaktu, Gaurav Kumar, Deep Dutta, Bindu Kulshreshtha
BACKGROUND: The phenotypic presentation of overt hypothyroidism during adolescence is less well characterized. The aim of the study was to study the phenotypic presentation of patients with overt hypothyroidism presenting during adolescence (age 9-18 years). METHODS: Records of adolescent patients with overt hypothyroidism (thyroid stimulating hormone [TSH]>10 mIU/L) were retrospectively analyzed for presenting complaints, height and pubertal status. RESULTS: A total of 67 patients (40 females and 37 males, average age 13...
March 28, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29572832/when-muscle-ca-2-channels-carry-monovalent-cations-through-gating-pores-insights-into-the-pathophysiology-of-type-1-hypokalaemic-periodic-paralysis
#11
Bruno Allard, Clarisse Fuster
Patients suffering from type 1 hypokalaemic periodic paralysis (HypoPP1) experience attacks of muscle paralysis associated with hypokalaemia. The disease arises from missense mutations in the gene encoding the α1 subunit of the dihydropyridine receptor (DHPR), a protein complex anchored in the tubular membrane of skeletal muscle fibres which controls the release of Ca2+ from sarcoplasmic reticulum and also functions as a Ca2+ channel. The vast majority of mutations consist of the replacement of one of the outer arginines in S4 segments of the α1 subunit by neutral residues...
March 23, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/29564279/sporadic-hypothyroidism-related-hypokalemic-paralysis-diagnosis-in-a-resource-poor-setting
#12
Nadasha Kadeeja, Nivetha Senthilnathan, Stalin Viswanathan, Rajeswari Aghoram
Hypothyroidism and distal renal tubular acidosis causing hypokalemic paralysis (HP) have been described only in four female patients. HP as the initial manifestation of uncomplicated diabetes has been reported only in three young males. We report two middle-aged patients presenting with gradual-onset areflexic quadriparesis and neck flop, associated with urinary potassium losses, and recovering over 3 days. The male patient with alcohol abuse had urine pH >5.5 and hyperchloremic metabolic acidosis due to renal tubular acidosis and hypothyroidism...
October 2017: Journal of Family Medicine and Primary Care
https://www.readbyqxmd.com/read/29482687/life-threatening-hypokalemic-quadriplegia-in-a-postoperative-patient
#13
Manoj Kamal, Geeta Singariya, Om Prakash Suthar, Ashwini S
Acute hypokalemic paralysis is a reversible but potentially lethal clinical condition. We report a case, who developed rapidonset quadriparesis in immediate postoperative period after undergoing right percutaneous nephrolithotomy for bilateral renal stones. On evaluation, she was found to have hypernatremic, hyperchloremic, hypokalemic acidosis. This severe hypokalemia-induced quadriparesis was precipitated by repeated furosemide injections, use of potassium-free fluid as maintenance, intracellular shift due to free water administration in this patient, who had pre-existing distal renal tubular acidosis...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29468618/analysis-of-clinical-and-metabolic-profile-of-acute-neuromuscular-weakness-related-to-hypokalemia
#14
Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Mayur Deepak Thakkar, Anup Kumar Thacker
OBJECTIVE: Acute neuromuscular weakness related to hypokalemia is a readily treatable disorder associated with diverse aetiologies. In this study we aim to report clinical pattern and biochemical features to identify the different aetiologies of the hypokalemic neuromuscular weakness. METHODS: Retrospective reviews of the medical record were analysed. Evaluation included demography, clinical features, investigations performed to ascertain the aetiologies. All the patients were categorised in to 3 groups; Idiopathic hypokalemic paralysis (IHP), dengue associated hypokalemic paralysis (DHP) and secondary group (SG) which included renal tubular acidosis (RTA- 1 and 2), thyrotoxic periodic paralysis (TPP) and Gitelman's syndrome (GS)...
September 15, 2017: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/29450857/hypokalemia-associated-with-pseudo-cushing-s-syndrome-and-magnesium-deficiency-induced-by-chronic-alcohol-abuse
#15
Masafumi Kurajoh, Keiko Ohsugi, Miki Kakutani-Hatayama, Takuhito Shoji, Hidenori Koyama
Hypokalemia and hypomagnesemia are frequently observed in patients with chronic alcoholism. However, the involvement of deranged cortisol regulation in patients with those conditions has not been reported. A 63-year-old Japanese male with chronic alcoholism was referred to the Department of Diabetes, Endocrinology and Metabolism for examination and treatment of hypokalemic periodic paralysis. Laboratory findings showed hypokalemia (2.3 mmol/l), as well as a high level of urinary excretion of potassium and hypomagnesemia (1...
May 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29429799/accelerated-idioventricular-rhythm-degenerating-into-bidirectional-ventricular-tachycardia-following-acute-myocardial-infarction
#16
Yun-Tao Zhao, Hang Zhou, Yumin Cui
Bidirectional ventricular tachycardia (BVT) is a rare ventricular tachyarrhythmia. It is usually regular, demonstrating a beat-to-beat alternation in the QRS frontal axis that varies between -20° to -30° and +110°. The tachycardia rate is typically between 140 and 180 beats/min and the QRS is relatively narrow, with a duration of 120 to 150 ms. The etiology of published BVT cases is most commonly digitalis toxicity and, rarely, herbal aconitine poisoning, hypokalemic periodic paralysis, catecholaminergic polymorphic ventricular tachycardia (CPVT), myocarditis, and Andersen-Tawil syndrome...
April 2018: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29419865/-screening-of-genetic-mutations-in-a-chinese-pedigree-affected-with-hypokalemic-periodic-paralysis
#17
Manli Guo, Guowen Zhang, Shaogang Ma, Tie Xu, Yigen Peng
OBJECTIVE To screen for mutations in a Chinese pedigree affected with hypokalemic periodic paralysis. METHODS The proband and nine family members were enrolled for the analysis of CACNA1S and SCN4A gene mutations. Genomic DNA was extracted from peripheral blood samples. The coding regions of the two genes were amplified with PCR and subjected to Sanger sequencing. Potential impact of suspected mutations was predicted with Bioinformatics software. The mutations were also verified among 100 healthy controls. RESULTS The proband and 5 family members (including 5 males and 1 female) had presented with episodes of flaccid paralysis accompanied by low serum potassium...
February 10, 2018: Zhonghua Yi Xue Yi Chuan Xue za Zhi, Zhonghua Yixue Yichuanxue Zazhi, Chinese Journal of Medical Genetics
https://www.readbyqxmd.com/read/29391559/substitutions-of-the-s4div-r2-residue-r1451-in-na-v-1-4-lead-to-complex-forms-of-paramyotonia-congenita-and-periodic-paralyses
#18
Hugo Poulin, Pascal Gosselin-Badaroudine, Savine Vicart, Karima Habbout, Damien Sternberg, Serena Giuliano, Bertrand Fontaine, Saïd Bendahhou, Sophie Nicole, Mohamed Chahine
Mutations in NaV 1.4, the skeletal muscle voltage-gated Na+ channel, underlie several skeletal muscle channelopathies. We report here the functional characterization of two substitutions targeting the R1451 residue and resulting in 3 distinct clinical phenotypes. The R1451L is a novel pathogenic substitution found in two unrelated individuals. The first individual was diagnosed with non-dystrophic myotonia, whereas the second suffered from an unusual phenotype combining hyperkalemic and hypokalemic episodes of periodic paralysis (PP)...
February 1, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29386226/stac3-enhances-expression-of-human-ca-v-1-1-in-xenopus-oocytes-and-reveals-gating-pore-currents-in-hypopp-mutant-channels
#19
Fenfen Wu, Marbella Quinonez, Marino DiFranco, Stephen C Cannon
Mutations of CaV 1.1, the pore-forming subunit of the L-type Ca2+ channel in skeletal muscle, are an established cause of hypokalemic periodic paralysis (HypoPP). However, functional assessment of HypoPP mutant channels has been hampered by difficulties in achieving sufficient plasma membrane expression in cells that are not of muscle origin. In this study, we show that coexpression of Stac3 dramatically increases the expression of human CaV 1.1 (plus α2 -δ1b and β1a subunits) at the plasma membrane of Xenopus laevis oocytes...
January 31, 2018: Journal of General Physiology
https://www.readbyqxmd.com/read/29278563/recognizing-thyrotoxic-hypokalemic-periodic-paralysis
#20
Kamini Patel, Jonathan V McCoy, Patrick M Davis
Thyrotoxic hypokalemic periodic paralysis (THPP) is a rare but potentially serious complication of thyrotoxicosis. The resulting muscle weakness is profound, associated with more severe hypokalemia, yet reversible. However, clinicians must be cautious because patients can develop life-threatening hyperkalemia during treatment. Underlying causes should be investigated as repeated episodes of THPP may occur.
January 2018: JAAPA: Official Journal of the American Academy of Physician Assistants
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