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1.
Zhonghua Yi Xue Yi Chuan Xue Za Zhi ; 40(2): 166-170, 2023 Feb 10.
Artigo em Chinês | MEDLINE | ID: mdl-36709934

RESUMO

OBJECTIVE: To explore the clinical characteristics and genetic basis for three children with Congenital chlorine diarrhea (CCD). METHODS: Three children with CCD who attended the Affiliated Children's Hospital of Capital Pediatric Institute from June 2014 to August 2020 were selected as the research subjects. Peripheral blood samples of the three children and their parents were collected for genetic testing. And the results were verified by Sanger sequencing. RESULTS: The clinical manifestations of the three children have included recurrent diarrhea, with various degrees of hypochloremia, hypokalemia and refractory metabolic alkalosis. Genetic testing revealed that the three children have all carried variants of the SLC26A3 gene, including homozygous c.1631T>A (p.I544N) variants, c.2063_1G>T and c.1039G>A (p.A347T) compound heterozygous variants, and c.270_271insAA(p.G91kfs*3) and c.2063_1G>T compound heterozygous variants. Sanger sequencing confirmed that all of the variants were inherited from their parents. CONCLUSION: The variants of the SLC26A3 gene probably underlay the CCD in these children. Above finding has enriched the spectrum of SLC26A3 gene variants.


Assuntos
Cloro , Hipopotassemia , Humanos , Criança , Testes Genéticos , Hipopotassemia/genética , Homozigoto , Diarreia/induzido quimicamente , Diarreia/genética , Mutação
3.
Am Fam Physician ; 107(1): 59-70, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36689973

RESUMO

Hypokalemia and hyperkalemia occur when serum potassium levels are less than 3.5 mEq per L or greater than 5.0 mEq per L, respectively. The World Health Organization recommends a potassium intake of at least 3,510 mg per day for optimal cardiovascular health. Hypokalemia is caused by decreased intake, renal losses, gastrointestinal losses, or transcellular shifts. Severe features of hypokalemia that require urgent treatment include a serum potassium level of 2.5 mEq per L or less, electrocardiography abnormalities, or neuromuscular symptoms. The underlying cause should be addressed, and potassium levels replenished. An oral route is preferred if the patient has a functioning gastrointestinal tract and a serum potassium level greater than 2.5 mEq per L. Hyperkalemia is caused by impaired renal excretion, transcellular shifts, or increased potassium intake. Electrocardiography identifies cardiac conduction disturbances but may not correlate with serum potassium levels. Emergent treatment is recommended for patients with clinical signs and symptoms (e.g., muscle weakness, paralysis) or if electrocardiography abnormalities are present. Acute treatment may include intravenous calcium, insulin, sodium bicarbonate, diuretics, and beta agonists. Dialysis may be considered in the presence of end-stage renal disease, severe renal impairment, or ongoing potassium release. Patiromer and sodium zirconium cyclosilicate are newer potassium binders and may be used in chronic or acute hyperkalemia. Sodium polystyrene sulfonate is associated with serious gastrointestinal adverse effects. Long-term management of potassium disturbances includes correcting underlying conditions, dietary counseling, and adjusting causative medications.


Assuntos
Hiperpotassemia , Hipopotassemia , Falência Renal Crônica , Humanos , Hiperpotassemia/complicações , Hipopotassemia/terapia , Potássio , Insulina/uso terapêutico , Arritmias Cardíacas/etiologia
5.
Beijing Da Xue Xue Bao Yi Xue Ban ; 55(1): 186-189, 2023 Feb 18.
Artigo em Chinês | MEDLINE | ID: mdl-36718710

RESUMO

Severe hypokalemia is defined as the concentration of serum potassium lower than 2.5 mmol/L, which may lead to serious arrhythmias and cause mortality. We report an unusual case of potentially fatal ventricular arrhythmias induced by severe hypokalemia in a patient undergoing laparoscopic partial nephrectomy in Peking University Third Hospital due to irregular use of indapamide before operation. Indapamide is a sulfonamide diuretic with vasodilative and calcium antagonistic effects, which enhances sodium delivery to the renal distal tubules resulting in a dose-related increase in urinary potassium excretion and decreases serum potassium concentrations. The electrolyte disorder caused by the diuretic is more likely to occur in the elderly patients, especially those with malnutrition or long-term fasting. Hence, the serum potassium concentration of the patients under indapamide therapy, especially elderly patients, should be monitored carefully. Meanwhile, the potassium concentration measured by arterial blood gas analysis is different from that measured by venous blood or laboratory test. According to the previous research, the concentration of potassium in venous blood was slightly higher than that in arterial blood, and the difference value was 0.1-0.5 mmol/L. This error should be taken into account when rapid intravenous potassium supplementation or reduction of blood potassium level was carried out clinically. In the correction of severe hypokalemia, the standard approach often did not work well for treating severe hypokalemia. The tailored rapid potassium supplementation strategy shortened the time of hypokalemia and was a safe and better treatment option to remedy life-threatening arrhythmias caused by severe hypokalemia with a high success rate. Through the anesthesia management of this case, we conclude that for the elderly patients who take indapamide or other potassium excretion diuretics, the electrolyte concentration and the general volume state of the patients should be comprehensively measured and fully evaluated before operation. It may be necessary for us to reexamine the serum electrolyte concentration before anesthesia induction on the morning of surgery in patients with the history of hypokalemia. For severe hypokalemia detected after anesthesia, central venous cannulation access for individualized rapid potassium supplementation is an effective approach to reverse the life-threatening arrhythmias caused by severe hypokalemia and ensure the safety of the patients.


Assuntos
Hipopotassemia , Indapamida , Humanos , Idoso , Hipopotassemia/induzido quimicamente , Hipopotassemia/complicações , Indapamida/efeitos adversos , Arritmias Cardíacas/induzido quimicamente , Arritmias Cardíacas/terapia , Diuréticos/efeitos adversos , Potássio , Eletrólitos/efeitos adversos , Anestesia Geral/efeitos adversos
9.
Diabetes Obes Metab ; 25(3): 815-822, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36479786

RESUMO

AIM: To determine the effectiveness and safety of early combination of insulin glargine with intravenous (IV) insulin infusion compared with IV insulin infusion alone in the management of diabetic ketoacidosis (DKA). METHODS: This was a single-centre, open-label, randomized controlled trial of adults aged 18 years or older diagnosed with DKA. The 'early glargine' group was given subcutaneous insulin glargine 0.3 units/kg within the first 3 hours of DKA diagnosis, in addition to the standard IV insulin infusion. The control group received standard IV insulin treatment only. The primary outcome was the time to DKA resolution. The other outcomes included rebound hyperglycaemia, mortality, hypoglycaemia and hypokalaemia, as well as the length of hospital stay (LOS). RESULTS: A total of 60 patients (30 patients per group) were enrolled. Most patients (76.7%) had type 2 diabetes. Both groups were similar in baseline characteristics, except for higher serum beta-hydroxybutyrate and lower pH levels in the early glargine group. The mean ± standard deviation time to DKA resolution in the early glargine group was significantly faster than the control group (9.89 ± 3.81 vs. 12.73 ± 5.37 hours; P = .022). The median (interquartile range) LOS was significantly shorter in the early glargine group than in the control group (4.75 [3.53-8.96] vs. 15.25 [5.71-26.38] days; P = .024). The incidence of rebound hyperglycaemia, all-cause mortality, hypoglycaemia and hypokalaemia was similar between the groups. CONCLUSIONS: Early combination of insulin glargine with IV insulin infusion led to a faster DKA resolution and a shorter LOS, without increasing hypoglycaemia and hypokalaemia.


Assuntos
Diabetes Mellitus Tipo 2 , Cetoacidose Diabética , Hiperglicemia , Hipoglicemia , Hipopotassemia , Adulto , Humanos , Insulina/efeitos adversos , Insulina Glargina/efeitos adversos , Cetoacidose Diabética/tratamento farmacológico , Diabetes Mellitus Tipo 2/tratamento farmacológico , Hipopotassemia/induzido quimicamente , Insulina Regular Humana , Hiperglicemia/tratamento farmacológico , Hiperglicemia/prevenção & controle , Hipoglicemiantes/efeitos adversos
10.
Mult Scler Relat Disord ; 69: 104466, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36584554

RESUMO

BACKGROUND: Primary Sjögren's syndrome (SS) is a chronic systemic autoimmune disease with varied neurological manifestations. SS is associated with anti-aquaporin-4 antibody (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD), a demyelinating autoimmune disorder of the central nervous system (CNS). Intriguingly, there are reports of osmotic demyelinating syndrome (ODS), a supposedly non-inflammatory disorder, in the context of SS and renal tubular acidosis (RTA), both of which are not yet established risk factors for ODS. METHODS: A literature search was undertaken to identify case reports of ODS in patients with SS. Details of the clinical and laboratory features of these patients were compiled. Additionally, we searched for NMOSD in patients with SS. We looked for co-existing RTA in patients with SS-ODS as well as SS-NMOSD. We also screened for reports of ODS in RTA without underlying SS. RESULTS & DISCUSSION: We identified 15 patients (all women, median age 40 years) with ODS in SS, and all of these patients had comorbid RTA. There were only three reported cases of ODS in RTA without underlying SS. We identified a total of 67 patients with SS-NMOSD, of whom only 3 (4.5%) had RTA. Hence, unlike NMOSD, the development of ODS in SS requires a prolonged osmotic or electrolyte abnormality caused by the comorbid RTA. The 15 patients with ODS and SS -RTA, showed heterogeneous clinical manifestations and outcomes. The most common symptom was quadriparesis, seen in 14 of the 15 patients. Eleven of the 15 patients had one of the following features, either alone or in combination: worsening of the sensorium, extensor plantar response, dysphagia/dysarthria, and facial palsy. The latter four manifestations were present at the onset in 7 patients and later in the course of the illness in the remaining 4 patients. Ocular palsy was seen in only four of the 15 patients and was a late manifestation. One patient who had extensive long-segment myelitis and subsequent ODS died, but most patients recovered without significant sequelae. None had hyponatremia, while all patients had hypokalemia and/or hypernatremia. Hypokalemia causing nephrogenic diabetes insipidus (NDI) followed by rapid rise in sodium and the resultant osmotic stress could potentially explain the occurrence of ODS in SS-RTA. Aquaporin (AQP) in astrocytes is implicated in ODS, and renal AQP is downregulated in NDI. Antibodies against AQPs are present in some patients with SS. Defective AQP is therefore a common link underlying all the connected diseases, namely SS, NDI, and ODS, raising the possibility of immune-mediated AQP dysfunction in the pathogenesis. CONCLUSION: The hitherto unreported association between SS-RTA and ODS may implicate SS and/or RTA in the development of ODS. In the setting of SS-RTA, ODS must be suspected when a patient with flaccid quadriparesis does not respond to the correction of potassium or develops additional neurological features along with a rise in sodium. Defective functions of AQPs may be a possible mechanism linking demyelinating CNS lesions, SS, and RTA. Studies evaluating AQP functions and serum antibodies against AQPs in these conditions are warranted.


Assuntos
Aquaporinas , Doenças do Sistema Nervoso Central , Hipopotassemia , Neuromielite Óptica , Síndrome de Sjogren , Humanos , Feminino , Adulto , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Hipopotassemia/complicações , Neuromielite Óptica/complicações , Neuromielite Óptica/epidemiologia , Anticorpos , Doenças do Sistema Nervoso Central/complicações , Quadriplegia , Sódio , Aquaporina 4 , Autoanticorpos
11.
Sr Care Pharm ; 37(12): 605-611, 2022 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-36461138

RESUMO

Introduction The patient was a 72-year-old man with a history of hypertension, hyperlipidemia, benign prostatic hyperplasia, and oropharyngeal cancer. His home medications include amlodipine, atorvastatin, hydrochlorothiazide, and tamsulosin. He lives alone and eats a soft, bland, nutrient-poor diet. During his annual primary care visit, he is found to have a serum potassium level of 3.3 mEq/L (reference range 3.5-5.0). Assessment The use of hydrochlorothiazide, a thiazide diuretic, as well as his low consumption of dietary potassium, have likely contributed to his mild, asymptomatic hypokalemia. Outcome The patient's serum potassium normalizes following replenishment with a 10 mEq microencapsulated potassium chloride (KCl) extended release (ER) tablet three times a day with meals for one week. A registered dietitian was consulted to provide recommendations for a well-balanced diet, consistent with his dietary texture needs. Conclusions Hypokalemia is a commonly encountered electrolyte disorder, occurring in about 3 to 4% of community-dwelling elders.1 Though asymptomatic hypokalemia is often an incidental finding, it is associated with an increased risk of major adverse cardiovascular events if left untreated and thus should be promptly corrected when discovered.².


Assuntos
Hipopotassemia , Tiazidas , Masculino , Humanos , Idoso , Hipopotassemia/induzido quimicamente , Hidroclorotiazida/efeitos adversos , Tansulosina , Potássio/uso terapêutico
12.
Am J Case Rep ; 23: e938609, 2022 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-36523136

RESUMO

BACKGROUND Prevention of lethal arrhythmias in congenital long QT syndrome type 1 (LQT1) requires avoidance of sympathoexcitation, drugs that prolong QT, and electrolyte abnormalities. However, it is often difficult to avoid all these risks in the perioperative period of open heart surgery. Herein, we report hypokalemia-induced cardiac arrest in a postoperative cardiac patient with LQT1 on catecholamine. CASE REPORT A 79-year-old woman underwent surgical aortic valve replacement for severe aortic stenosis. Although the initial plan was not to use catecholamine, catecholamine was used in the Postoperative Intensive Care Unit with attention to QT interval and electrolytes due to heart failure caused by postoperative bleeding. Serum potassium levels were controlled above 4.5 mEq/L, and no arrhythmic events occurred. On postoperative day 4, the patient was started on insulin owing to hyperglycemia. Cardiac arrest occurred after the first insulin dose; the implantable cardioverter defibrillator was activated, and the patient's own heartbeat resumed. Subsequent examination revealed that a marked decrease in serum potassium level had occurred after insulin administration. The electrocardiogram showed obvious QT prolongation and ventricular fibrillation following R on T. Thereafter, under strict potassium management, there was no recurrence of cardiac arrest events. CONCLUSIONS A patient with LQT1 who underwent open heart surgery developed ventricular fibrillation after Torsades de Pointes, probably due to hypokalemia after insulin administration in addition to catecholamine. It is important to check serum potassium levels to avoid the onset of Torsades de Pointes in patients with long QT syndrome. In addition, the impact of insulin administration was reaffirmed.


Assuntos
Parada Cardíaca , Hipopotassemia , Insulinas , Síndrome do QT Longo , Síndrome de Romano-Ward , Torsades de Pointes , Feminino , Humanos , Idoso , Torsades de Pointes/etiologia , Torsades de Pointes/diagnóstico , Hipopotassemia/complicações , Fibrilação Ventricular/complicações , Valva Aórtica , Catecolaminas , Síndrome do QT Longo/diagnóstico , Eletrocardiografia , Parada Cardíaca/complicações , Arritmias Cardíacas/complicações , Potássio , Insulinas/efeitos adversos
13.
Vnitr Lek ; 68(E-8): 8-11, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36575060

RESUMO

Liddle syndrome is an inherited form of arterial hypertension with autosomal dominant pattern of inheritance. It is caused by activating mutation of genes coding of the epithelial sodium channel in distal nephron. Mutation leads to excessive reabsorbtion of sodium ions and volume expansion resulting in arterial hypertension. Antoher typical laboratory findings are hypokalaemia, low levels of serum aldosteron and metabolic alkalosis. Phenotypic variability makes it difficult to identify patients with Liddle syndrome, often resulting in misdiagnosis and severe complications at early age. Genetic studies should be done to confirm the diagnosis. Therapy of Liddle syndrome is based on administration of epithelial sodium channel blocker amilorid.


Assuntos
Hipertensão , Hipopotassemia , Síndrome de Liddle , Humanos , Síndrome de Liddle/diagnóstico , Síndrome de Liddle/genética , Síndrome de Liddle/terapia , Canais Epiteliais de Sódio/genética , Canais Epiteliais de Sódio/metabolismo , Hipopotassemia/diagnóstico , Hipopotassemia/etiologia , Hipopotassemia/terapia , Mutação
14.
J Ayub Med Coll Abbottabad ; 34(4): 864-868, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36566415

RESUMO

Acute hypokalemic paralysis (AHP) is a life-threatening emergency. It is exceptionally unusual for hypothyroidism to present with AHP. This association can be either primary or secondary through distal renal tubular acidosis. We report two cases who presented with acute quadriplegia. The succeeding investigations revealed severe hypokalemia and autoimmune hypothyroidism. The second case was found to have Sjogren's syndrome additionally. The underlying aetiology of hypokalemia in both cases was found to be dRTA. The combination of such conditions is reported sporadically. Here we also discuss the potential association of AHP with autoimmune conditions by proxy through dRTA.


Assuntos
Doenças Autoimunes , Hipopotassemia , Hipotireoidismo , Humanos , Hipopotassemia/complicações , Paralisia/etiologia , Doenças Autoimunes/complicações , Hipotireoidismo/complicações
15.
Yi Chuan ; 44(11): 1072-1078, 2022 Nov 20.
Artigo em Inglês | MEDLINE | ID: mdl-36384999

RESUMO

Primary aldosteronism (PA) is a disease characterized by hypertension and hypokalemia due to the excessive aldosterone secretion from the adrenal cortex, which leads to the retention of both water and sodium, and the inhibition of the renin-angiotensin system as well. Familial hyperaldosteronism type II (FH-II) is known as an autosomal dominant hereditary disease, which is a scarce cause of PA. In this report, we cllected the clinical data of a patient with repeated hypertension and hypokalemia of uncertain diagnosis since 2014. Nevertheless, we discovered by genetic sequencing in 2021 that the CLCN2 and WFS1 gene mutation of the patient, whose mother belongs to heterozygote genotype and father belongs to wild-type genotype. Combined with a series of endocrine function tests and imaging studies, the patient was finally certified her suffering from FH-II and WFS1 gene mutation. By summarizing and analyzing the characteristics and genetic test results of this case, we recommended gene sequencing for patients with PA whose etiology is difficult to be determined clinically. This case also provides new clinical data for subsequent genetic studies of the disease.


Assuntos
Hiperaldosteronismo , Hipertensão , Hipopotassemia , Humanos , Feminino , Hipopotassemia/complicações , Hipopotassemia/genética , Hiperaldosteronismo/diagnóstico , Hiperaldosteronismo/genética , Hiperaldosteronismo/terapia , Testes Genéticos , Mutação , Hipertensão/genética
16.
Sci Rep ; 12(1): 19781, 2022 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-36396697

RESUMO

Hypokalemia in dairy cows, which is characterized by too low serum potassium levels, is a severe mineral disorder that can be life threatening. In this paper, we explore different originating conditions of hypokalemia-reduced potassium intake, increased excretion, acid-base disturbances, and increased insulin-by using a dynamic mathematical model for potassium balance in non-lactating and lactating cows. The simulations confirm observations described in literature. They illustrate, for example, that changes in dietary intake or excretion highly effect intracellular potassium levels, whereas extracellular levels vary only slightly. Simulations also show that the higher the potassium content in the diet, the more potassium is excreted with urine. Application of the mathematical model assists in experimental planning and therefore contributes to the 3R strategy: reduction, refinement and replacement of animal experiments.


Assuntos
Hipopotassemia , Feminino , Bovinos , Animais , Hipopotassemia/veterinária , Lactação , Potássio , Minerais , Dieta
18.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 47(10): 1461-1466, 2022 Oct 28.
Artigo em Inglês, Chinês | MEDLINE | ID: mdl-36411698

RESUMO

Pseudohypoparayhyroidism (PHP) is a rare autosomal dominant or recessive genetic disorder characterized by low calcium, high phosphorus, and target organ resistance to parathyroid. The clinical characteristics and genetic features in 4 patients with Type Ib PHP in the Third Xiangya Hospital, Central South University, have been reviewed. All 4 patients had low calcium, high phosphorus, and parathyroid resistance. Among them, 2 patients had slightly elevated thyroid stimulating hormone and mild features of Albright's hereditary osteodystrophy, and one patient had hypokalemia. No guanine nucleotide-binding protein alpha-stimulating activity polypeptide 1 (GNAS) and gene variant associated with hypokalemia were identified using the whole exome sequencing. The results of the methylation-specific multiple ligation-dependent probe amplification showed that there were abnormal methylation of the upstream differentially methylated regions of GNAS in the 4 patients. There were phenotype overlap among the various subtypes of PHP. Detection of GNAS gene methylation in patients with clinical suspicion of Type Ib PHP is helpful for the diagnosis and treatment of PHP.


Assuntos
Hipopotassemia , Pseudo-Hipoparatireoidismo , Humanos , Cromograninas/genética , Subunidades alfa Gs de Proteínas de Ligação ao GTP/genética , Cálcio , Pseudo-Hipoparatireoidismo/genética , Fósforo
19.
JAMA Netw Open ; 5(11): e2240809, 2022 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-36346630

RESUMO

Importance: Eating disorders lead to increased mortality and reduced quality of life. While the acute presentations of eating disorders frequently involve electrolyte abnormalities, it remains unknown whether electrolyte abnormalities may precede the future diagnosis of an eating disorder. Objective: To determine whether outpatient electrolyte abnormalities are associated with the future diagnosis of an eating disorder. Design, Setting, and Participants: This population-level case-control study used provincial administrative health data for residents of Ontario, Canada aged 13 years or older from 2008 to 2020. Individuals without an eating disorder (controls) were matched 4:1 to individuals diagnosed with an incident eating disorder (cases) based on age and sex. Both groups had outpatient electrolyte measurements between 3 years and 30 days prior to index. Index was defined as the date of an eating disorder diagnosis in any inpatient or outpatient clinical setting for cases. Controls were assigned a pseudo-index date according to the distribution of index dates in the case population. Individuals with any prior eating disorder diagnosis were excluded. The data analyzed was from January 1, 2008, through June 30, 2020. Exposures: Any electrolyte abnormality, defined as abnormal test results for a composite of hypokalemia, hyperkalemia, hyponatremia, hypernatremia, hypomagnesemia, hypophosphatemia, metabolic acidosis, or metabolic alkalosis. Outcomes and Measures: Eating disorder diagnosis including anorexia nervosa, bulimia nervosa, and eating disorder not otherwise specified. Results: A total 6970 eligible Ontario residents with an eating disorder (mean [SD] age, 28 (19) years; 6075 [87.2%] female, 895 [12.8%] male) were matched with 27 878 age- and sex-matched residents without an eating disorder diagnosis (mean [SD] age, 28 [19] years; 24 300 [87.2%] female, 3578 [12.8%] male). Overall, 18.4% of individuals with an eating disorder had a preceding electrolyte abnormality vs 7.5% of individuals without an eating disorder (adjusted odds ratio [aOR], 2.12; [95% CI, 1.86-2.41]). The median (IQR) time from the earliest electrolyte abnormality to eating disorder diagnosis was 386 (157-716) days. Specific electrolyte abnormalities associated with a higher risk of an eating disorder were: hypokalemia (aOR, 1.98; 95% CI, 1.70-2.32), hyperkalemia (aOR, 1.97; 95% CI, 1.48-2.62), hyponatremia (aOR, 5.26; 95% CI, 3.32-8.31), hypernatremia (aOR, 3.09; 95% CI, 1.01-9.51), hypophosphatemia (aOR, 2.83; 95% CI, 1.82-4.40), and metabolic alkalosis (aOR, 2.60; 95% CI, 1.63-4.15). Conclusions and Relevance: In this case-control study, individuals with an eating disorder were associated with a preceding outpatient electrolyte abnormality compared with matched controls. Otherwise unexplained electrolyte abnormalities may serve to identify individuals who may benefit from screening for an underlying eating disorder.


Assuntos
Alcalose , Transtornos da Alimentação e da Ingestão de Alimentos , Hiperpotassemia , Hipernatremia , Hipopotassemia , Hiponatremia , Hipofosfatemia , Adulto , Masculino , Humanos , Adolescente , Feminino , Hipernatremia/diagnóstico , Hipernatremia/epidemiologia , Hiponatremia/diagnóstico , Hiponatremia/epidemiologia , Hipopotassemia/diagnóstico , Hipopotassemia/epidemiologia , Estudos de Casos e Controles , Qualidade de Vida , Transtornos da Alimentação e da Ingestão de Alimentos/diagnóstico , Transtornos da Alimentação e da Ingestão de Alimentos/epidemiologia , Eletrólitos , Ontário/epidemiologia
20.
Front Endocrinol (Lausanne) ; 13: 1017331, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36277689

RESUMO

Objective: The first-line treatment for Cushing's disease is transsphenoidal surgery, after which the rates of remission are 60 to 80%, with long-term recurrence of 20 to 30%, even in those with real initial remission. Drug therapies are indicated for patients without initial remission or with surgical contraindications or recurrence, and ketoconazole is one of the main available therapies. The objective of this study was to evaluate the safety profile of and the treatment response to ketoconazole in Cushing's disease patients followed up at the endocrinology outpatient clinic of a Brazilian university hospital. Patients and methods: This was a retrospective cohort of Cushing's disease patients with active hypercortisolism who used ketoconazole at any stage of follow-up. Patients who were followed up for less than 7 days, who did not adhere to treatment, or who were lost to follow-up were excluded. Results: Of the 172 Cushing's disease patients who were followed up between 2004 and 2020, 38 received ketoconazole. However, complete data was only available for 33 of these patients. Of these, 26 (78%) underwent transsphenoidal surgery prior to using ketoconazole, five of whom (15%) had also undergone radiotherapy; seven used ketoconazole as a primary treatment. Ketoconazole use ranged from 14 days to 14.5 years. A total of 22 patients had a complete response (66%), three patients had a partial response (9%), and eight patients had no response to treatment (24%), including those who underwent radiotherapy while using ketoconazole. Patients whose hypercortisolism was controlled or partially controlled with ketoconazole had lower baseline 24-h urinary free cortisol levels than the uncontrolled group [times above the upper limit of normal: 0.62 (SD, 0.41) vs. 5.3 (SD, 8.21); p < 0.005, respectively] in addition to more frequent previous transsphenoidal surgery (p < 0.04). The prevalence of uncontrolled patients remained stable over time (approximately 30%) despite ketoconazole dose adjustments or association with other drugs, which had no significant effect. One patient received adjuvant cabergoline from the beginning of the follow-up, and it was prescribed to nine others due to clinical non-response to ketoconazole alone. Ten patients (30%) reported mild adverse effects, such as nausea, vomiting, dizziness, and loss of appetite. Only four patients had serious adverse effects that warranted discontinuation. There were 20 confirmed episodes of hypokalemia among 10/33 patients (30%). Conclusion: Ketoconazole effectively controlled hypercortisolism in 66% of Cushing's disease patients, being a relatively safe drug for those without remission after transsphenoidal surgery or whose symptoms must be controlled until a new definitive therapy is carried out. Hypokalemia is a frequent metabolic effect not yet described in other series, which should be monitored during treatment.


Assuntos
Síndrome de Cushing , Hipopotassemia , Hipersecreção Hipofisária de ACTH , Humanos , Hipersecreção Hipofisária de ACTH/complicações , Hipersecreção Hipofisária de ACTH/tratamento farmacológico , Hipersecreção Hipofisária de ACTH/cirurgia , Cetoconazol/uso terapêutico , Estudos Retrospectivos , Hidrocortisona , Cabergolina , Hipopotassemia/tratamento farmacológico , Síndrome de Cushing/tratamento farmacológico
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