Neonatal systemic pseudohypoaldosteronism type I / 中国当代儿科杂志

An 18-day-old male infant was admitted to the hospital due to recurrent hyperkalemia for more than 10 days. The neonate had milk refusal and dyspnea. The blood gas analysis revealed recurrent hyperkalemia, hyponatremia and metabolic acidosis. Adrenocortical hormone replacement therapy was ineffective. Additional tests showed a significant increase in aldosterone levels. Family whole exome sequencing revealed that the infant had compound heterozygous in the SCNNIA gene, inherited from both parents. The infant was diagnosed with neonatal systemic pseudohypoaldosteronism type I. The infant's electrolyte levels were stabilized through treatment with sodium polystyrene sulfonate and sodium supplement. The infant was discharged upon clinical recovery. This study provides a focused description of differential diagnosis of salt-losing syndrome in infants and introduces the multidisciplinary management of neonatal systemic pseudohypoaldosteronism type I.

Prediction of perioperative hyperkalemia in dialysis patients with secondary hyperparathyroidism / 中华耳鼻咽喉头颈外科杂志

Objective: To explore the influencing factors for serum potassium >4.4 mmol/L in the morning of parathyroidectomy in hemodialysis patients with secondary hyperparathyroidism (SHPT). Methods: The clinical data of 72 patients with SHPT who received regular hemodialysis and underwent parathyroidectomy in Guangdong Provincial People's Hospital from January 2012 to December 2018 were analyzed retrospectively. There were 37 males and 35 females, aged from 25 to 69 years, and the dialysis timespan was from 0.5 to 11 years. The levels of parathyroid hormone, serum potassium and serum calcium before hemodialysis were examined one day before operation, and hemodialysis time and dewatering volume after hemodialysis without heparin were recorded, and also the level of serum potassium in the morning of parathyroidectomy was detected. The occurrences of hyperkalemia during and after operation were studied. The factors related to hyperkalemia in the morning of parathyroidectomy were evaluated by Pearson or Spearman correlation analysis, and the cut-off values of risk factors were calculated by receiver operating characteristic (ROC) curve. Results: Serum potassium >4.4 mmol/L in the morning of parathyroidectomy existed in 23 of 72 patients. Correlation analysis showed that serum potassium one day before operation ((4.93±0.56)mmol/L, r=0.656, P<0.001) and dehydration volume ((2.37±0.75)L, r=0.261, P=0.027) were positively correlated with serum potassium in the morning of parathyroidectomy((4.16±0.54)mmol/L). Serum potassium before hemodialysis one day before operation was a main predictor for serum potassium in the morning of parathyroidectomy (AUC=0.791, P<0.001). The cut-off value of serum potassium before hemodialysis one day before operation was 5.0 mmol/L. Conclusion: Serum potassium before hemodialysis one day before operation in patients with SHPT can predict serum potassium in the morning of parathyroidectomy, offering imformation for the safety of operation.

Hiperkalemia postoperatoria en síndrome de hueso hambriento post-paratiroidectomía por hiperparatiroidismo secundario en enfermedad renal crónica terminal / Hyperkalemia-associated hungry bone syndrome post-parathyroidectomy in end-stage denal disease

Introducción: El hiperparatiroidismo secundario (HPTS) es una complicación de la enfermedad renal crónica terminal (ERCT). A pesar de nuevas terapias médicas como calcimiméticos, en HPTS refractarios la paratiroidectomía (PTX) continúa siendo necesaria. Una complicación frecuente en estos pacientes posterior a la PTX es el síndrome de hueso hambriento (SHH), caracterizado por una profunda y prolongada hipocalcemia asociada a hipofosfatemia, secundaria a un excesivo aumento de su captación ósea. Una complicación menos descrita, pero con consecuencias graves e incluso fatales, es la hiperkalemia. El propósito de este trabajo consiste en enfatizar el riesgo de hiperkalemia por SHH a partir de un caso clínico, señalar los mecanismos fisiopatológicos, factores de riesgo y consideraciones terapéuticas. Caso clínico: Mujer de 35 años, con ERCT de causa desconocida, HPTS refractario con PTX total e implante de glándulas en antebrazo hace 9 años. Ingresa por recurrencia de HPTS. Cintigrama MIBI SPECT/CT® evidenció implante hiperfuncionante, indicándose PTX del injerto. Exámenes preoperatorios: calcemia 8.6 mg/dL, fosfatasas alcalinas 1115 UI/L (VN <100), PTH intacta (PTHi) 3509 pg/ml y kalemia 4.8 mEq/L. Biopsia: hiperplasia paratiroidea nodular. En postoperatorio inmediato presentó hiperkalemia de 7.1 mEq/L con cambios electrocardiográficos, requiriendo hemodiálisis de urgencia. Posteriormente desarrolló hipocalcemia, hipofosfatemia e hipomagnesemia, de difícil control. Discusión: El SHH post HPTS puede coexistir con hiperkalemia postoperatoria inmediata grave, incluso fatal si no se identifica y corrige a tiempo. El mecanismo fisiopatológico aún no está bien dilucidado. Varios factores pudieran intervenir, incluyendo aumento del metabolismo celular, traumatismo tisular, fármacos anestésicos, fluidos perioperatorios y flujo de iones transmembrana. El nivel de potasio previo a la cirugía, menor edad, género masculino, tiempo entre la última hemodiálisis y la cirugía, y duración de la PTX, son factores de riesgo para hiperkalemia postoperatoria. El conocimiento de esta grave complicación permitirá estar preparado para monitorizar y eventualmente tratar.

Introduction: Secondary Hyperparathyroidism (SHPT) is a complication of End-Stage Renal Disease (ESRD). Although new medical therapies (i.e.calcimimetics,) parathyroidectomy (PTX) continues to be necessary in refractory cases. A well-known complication after PTX is an entity called Hungry Bone Syndrome (HBS), characterized by deep and prolonged hypocalcemia associated with hypophosphatemia, secondary to an excessive increase in bone formation. A less reported complication, but with severe or even fatal consequences, is hyperkalemia. The purpose of this work consists of emphasizing the risk of hyperkalemia in HBS, reporting a clinical case that points out the physiopathological mechanisms, risk factors, and therapeutic considerations. Clinical case: 35-year-old woman with ESRD of unknown cause with refractory SHPT with total PTX and forearm gland grafts nine years ago. She presented SHPT recurrency. MIBI SPECT/CT® scan showed a hyperfunctioning implant, indicating graft PTX. Preoperative tests: calcemia 8.6 mg/dL, phosphatemia 7.3 mg/dL, alkaline phosphatases 1115 UI/L (VN<100), intact PTH (iPTH) 3509 pg/ml and kalemia 4.8 mEq/L. Biopsy: parathyroid nodular hyperplasia. In the immediate postoperative period, she presented hyperkalemia at 7.1 mEq/L with electrocardiographic changes, requiring emergency hemodialysis. Later she developed hypocalcemia, hypophosphatemia, and hypomagnesemia of difficult control. Discussion: HBS post PTX can coexist with severe immediate postoperative hyperkalemia, which can be even fatal if not detected and corrected. The physiopathological mechanism is still not entirely elucidated. Various factors could interfere, including an increase in cell metabolism, tissue traumatism, anesthetic drugs, intraoperative fluids, and transmembrane ion flow. Preoperative potassium levels, younger age, male gender, the time elapsed between last hemodialysis and surgery, and duration of PTX are risk factors for post-surgical hyperkalemia. Knowing this severe complication will allow the medical team to be prepared for monitoring and eventually treating it.

Hyperkalemia in chronic kidney disease

SUMMARY Hyperkalemia is a frequent finding in patients with chronic kidney disease (CKD). This increase in serum potassium levels is associated with decreased renal ion excretion, as well as the use of medications to reduce the progression of CKD or to control associated diseases such as diabetes mellitus and heart failure. Hyperkalemia increases the risk of cardiac arrhythmia episodes and sudden death. Thus, the control of potassium elevation is essential for reducing the mortality rate in this population. Initially, the management of hyperkalemia includes orientation of low potassium diets and monitoring of patients' adherence to this procedure. It is also important to know the medications in use and the presence of comorbidities to guide dose reduction or even temporary withdrawal of any of the potassium retention-related drugs. And finally, the use of potassium binders is indicated in both acute episodes and chronic hyperkalemia.

RESUMO A hiperpotassemia é um achado frequente em pacientes com doença renal crônica (DRC). Esta elevação do nível sérico de potássio está associada à diminuição da excreção renal do íon, assim como ao uso de medicações para retardar a progressão da DRC ou para controlar doenças associadas, como diabetes mellitus e insuficiência cardíaca. A hiperpotassemia aumenta o risco de episódios de arritmia cardíaca e morte súbita. Assim, o controle da elevação de potássio é essencial para a diminuição da taxa de mortalidade nessa população. O manejo da hiperpotassemia inclui, inicialmente, orientação de dietas com baixo teor de potássio e acompanhamento da aderência dos pacientes a esse procedimento. Também é importante conhecer as medicações em uso e a presença de comorbidades, a fim de orientar a redução de doses ou até mesmo a suspensão temporária de alguma das drogas relacionadas à retenção de potássio. E, finalmente, o uso de quelantes de potássio é indicado tanto em episódios agudos como nos casos de hiperpotassemia crônica.

Cecum perforation associated with a calcium polystyrene sulfonate bezoar - a rare entity / Perfuração do ceco associada a bezoar de poliestirenossulfonato de cálcio - uma entidade rara

Abstract Hyperkalemia is one of the most common electrolyte disorders, responsible for a high number of adverse outcomes, including life-threatening arrhythmias. Potassium binders are largely prescribed drugs used for hyperkalemia treatment but unfortunately, there are many adverse events associated with its use, mostly gastrointestinal. Identification of patients at highest risk for the serious complications associated with the current potassium binders, such as colon necrosis and perforation, could prevent fatal outcomes. The authors present a case of a 56-year-old man with secondary diabetes and chronic renal disease that was treated for hyperkalemia with Calcium Polystyrene Sulfonate (CPS). He later presented with acute abdomen due to cecum perforation and underwent ileocecal resection but ultimately died from septic shock a week later. During surgery, a solid white mass was isolated in the lumen of the colon. The mass was identified as a CPS bezoar, a rare drug-mass formed in the gastrointestinal tract that contributed to the perforation. A previous history of partial gastrectomy and vagothomy was identified as a probable risk factor for the CPS bezoar development. Hopefully, the two new potassium binders patiromer and (ZS-9) Sodium Zirconium Cyclosilicate will help treat such high-risk patients, in the near future.

Resumo A hipercalemia é um dos distúrbios eletrolíticos mais comuns, responsável por um grande número de desfechos adversos, incluindo arritmias potencialmente fatais. Quelantes de potássio são amplamente prescritos para o tratamento da hipercalemia, mas infelizmente são muitos os eventos adversos associados ao seu uso, em particular os gastrointestinais. A identificação de pacientes com risco mais elevado para complicações graves associadas aos quelantes de potássio atualmente em uso, como necrose e perfuração do cólon, pode evitar desfechos fatais. O presente artigo descreve o caso de um homem de 56 anos com diabetes secundário e doença renal crônica em tratamento por hipercalemia com poliestirenossulfonato de cálcio (PSC). Posteriormente o paciente apresentou abdômen agudo devido a perfuração do ceco e foi submetido a uma ressecção ileocecal, mas acabou indo a óbito por choque séptico uma semana mais tarde. Durante a cirurgia, uma massa branca sólida foi isolada no lúmen do cólon. A massa foi identificada como um bezoar de PSC, uma massa de fármaco de rara ocorrência formada no trato gastrointestinal que contribuiu para a perfuração. História pregressa de gastrectomia parcial e vagotomia foi identificada como provável fator de risco para o desenvolvimento do bezoar de PSC. Espera-se que os dois novos quelantes de potássio - patiromer e ciclossilicato de zircônio sódico - ajudem a tratar pacientes de alto risco em um futuro próximo.

Rabdomiólisis intraoperatoria en cirugía de resección de osteosarcoma de fémur: Reporte de caso y discusión / Case report: Intraoperative rhabdomyolisis after femur osteosarcoma resection

Rhabdomyolysis is a pathology that rarely has causes in the perioperative period, where it has been commonly related as a complication of malignant hyperthermia, prolonged patient positioning with intraoperative muscle compression, in the postoperative period of bariatric surgery and in children. The purpose of this review is to present the case of a 49 year-old male patient, who underwent limb salvage surgery for treatment of a left femur osteosarcoma, with reconstruction via bone transplant and joint prosthesis. During the procedure hyperkalemia and elevation of Creatine-Phosphokinase (CPK) enzyme levels where detected, without changes compatible with renal failure, which required repeated treatment to normalize and that, after ruling out other causes, it was attributed to skeletal muscle destruction during the procedure. Rhabdomyolysis is a phenomenon inherent to this sort of procedures and may manifest initially as laboratory findings and that, if not diagnosed in time, may lead to fatal arrhythmias and acute renal failure.

La rabdomiólisis es una patología que rara vez tiene origen en el período perioperatorio, donde comúnmente se le ha relacionado como complicación de la hipertermia maligna, de decúbitos prolongados con compresión muscular intraoperatoria, del posoperatorio de la cirugía bariátrica y en niños. El objetivo de este trabajo es presentar el caso de un hombre de 49 años, sometido a resección de un osteosarcoma de fémur izquierdo con reconstrucción mediante trasplante óseo y prótesis articular, durante el cual se detectan hiperpotasemia y aumento de la Creatin-Fosfokinasa (CPK), sin alteraciones compatibles con fallo renal, que requirió reiterados tratamientos para la normalización de los valores de kalemia, y que tras descartar otras causas se atribuyó a la destrucción de musculoesquelético durante el procedimiento. La rabdomiólisis es un fenómeno inherente a este tipo de procedimientos y puede manifestarse inicialmente con alteraciones analíticas que, de no ser diagnosticadas en tiempo y forma, pueden llevar a arritmias fatales y fallo renal agudo.

Potassium abnormalities in current clinical practice: frequency, causes, severity and management

We aimed to investigate the prevalence and etiology of potassium abnormalities [hypokalemia and hyperkalemia] and management approaches for hospitalized patients. Over a 4-month period, all hospitalized patients at Hacettepe University Medical Faculty Hospitals who underwent at least one measurement of serum potassium during hospitalization were included. Data on serum levels of electrolytes, demographic characteristics, cause[s] of hospitalization, medications, etiology of potassium abnormality and treatment approaches were obtained from the hospital records. Of the 9,045 hospitalized patients, 1,265 [14.0%] had a serum potassium abnormality; 604 [6.7%] patients had hypokalemia and 661 [7.30%] had hyperkalemia. In the hypokalemic patients, the most important reasons were gastrointestinal losses in 555 [91.8%] patients and renal losses in 252 [41.7%] patients. The most frequent treatment strategies were correcting the underlying cause and replacing the potassium deficit. Of the 604 hypokalemic patients, 319 [52.8%] were normokalemic at hospital discharge. The most common reason for hyperkalemia was treatment with renin-angiotensin-aldosterone system blockers in 228 [34.4%] patients, followed by renal failure in 191 [28.8%]. Two hundred and ninety-eight [45.0%] patients were followed without any specific treatment. Of the 661 hyperkalemic patients, 324 [49.0%] were normokalemic at hospital discharge. This study showed a high prevalence of potassium imbalance among hospitalized patients. Although most of the potassium abnormalities were mild/moderate, approximately half of the patients treated for hypokalemia or hyperkalemia were discharged from the hospital with ongoing dyskalemia

Hiperpotassemia pela solução Euro-Collins na anestesia para transplante renal: relato de caso / Hyperkalemia by Euro-Collins solution in anesthesia for renal transplantation: a case report / Hiperpotasemia por la solución de Euro-Collins en la anestesia para transplante renal: relato de caso

OBJETIVOS: Descrever uma anestesia para transplante renal que se complicou com a elevação brusca de potássio, pela reperfusão do rim com solução Euro-Collins no campo operatório. Também será relatado o diagnóstico e o tratamento empregados nessa complicação. CONCLUSÃO: O uso de soluções de perfusão no campo cirúrgico requer cuidados na monitoração, como eletrocardioscopia e dosagem de potássio sérico, e disponibilidade para uso imediato de gluconato de cálcio, insulina e salbutamol. A substituição da solução Euro-Collins por soro fisiológico imediatamente antes do implante pode ser uma opção útil em pacientes com níveis de potássio sabidamente elevados.

OBJECTIVE: To describe anesthesia for renal transplantation that progressed to a sharp potassium increase after kidney reperfusion with Euro-Collins' solution in the operative field. We will also report on diagnosis and treatment used. CONCLUSION: The use of infusion solutions in the surgical field requires careful monitoring, such as electrocardiography, measurement of serum potassium, and availability of calcium gluconate, insulin, and albuterol for immediate use. The replacement of Euro-Collins' solution for saline solution immediately before the implant may be a useful option in patients with high levels of potassium.

OBJETIVOS: Describir una anestesia para transplante renal que se complicó con la elevación brusca de potasio por la reperfusión del riñón con solución de Euro-Collins en el campo operatorio. También será relatado el diagnóstico y el tratamiento usados en esa complicación. CONCLUSIONES: El uso de soluciones de perfusión en el campo quirúrgico requiere cuidados en el monitoreo, como la electrocardioscopia, la dosificación de potasio sérico y la disponibilidad para el uso inmediato del gluconato de calcio, insulina y salbutamol. El reemplazo de la solución de Euro-Collins por suero fisiológico inmediatamente antes del implante, puede ser una opción útil en los pacientes con niveles de potasio consabidamente elevados.

Successful resuscitation of prolonged hyperkalemic cardiac arrest following succinylcholine administration in possibly undiagnosed myopathy

We report a 3 years old male with undiagnosed myopathy, who developed cardiac arrest secondary to hyperkalemia following succinylcholine administration.The patient underwent prolonged cardiopulmonary resuscitation [CPR], which ended successfully after applying external pace maker. He was discharged home eleven days after the cardiac arrest for follow up of his myopathy

Profound hyperkalemia and the electrocardiogram. Lack of correlation: a case report

We report a case of severe hyperkalemia as a result of treatment with potassium sparing diuretics, digoxin and angiotensin receptor antagonist valsartan in the presence of renal insufficiency. Inspite of a maximal serum potassium concentration of 10.3 mmol/l, only non-specific ECG changes were found. The patient survived after an uneventful dialysis. Thus severe hyperkalemia may present without typical ECG changes, and values exceeding 10.3 mmol/l may not necessarily be fatal

Complex glycerol kinase deficiency: an X-linked disorder associated with adrenal hypoplasia congenita.

Complex glycerol kinase deficiency (GKD) results from the contiguous deletion on Xp21 of all or part of the gene for glycerol kinase together with that for adrenal hypoplasia congenita (AHC) and /or Duchenne muscular dystrophy (DMD). The authors present the case of a newborn whose initial issues were refractory hypoglycaemia along with hyponatremia and hyperkalemia. He also had low serum cortisol levels and raised urinary excretion of glycerol and required steroid supplementation. His creatinine phosphokinase (CPK) levels were normal. Molecular studies revealed a contiguous Xp21 deletion. Therapy in such cases must be prompt and includes correction of hypoglycaemia and dyselectrolytemia, a low fat diet and steroid replacement.

Transient hyperkalemia and hypoaldosteronism in a patient with acute glomerulonephritis.

The authors describe a 7-year-old boy with acute glomerulonephritis, who developed acute renal failure in the early course of his disease. While the renal function and other clinical manifestations gradually improved, hyperkalemia occurred unexpectedly, and returned to normal level spontaneously after a short period of symptomatic treatment. With the result of a low transtubular potassium gradient (TTKG) level, it was concluded that hypoaldosteronism was the major cause of hyperkalemia in this patient.

Acidosis tubular renal / Renal tubular acidosis

Los transtornos de acidificación renal se manifiestan por síntomas y signos poco específicos, por lo que el pediatra debe sospechar el diagnóstico frente a un niño con dificultad en el progreso ponderal, anorexia, poliuria y que en los exámenes presenta acidosis metabólica hiperclómerica. Con estos antecedentes es importante la derivación oportuna al nefrólogo para la determinación del tipo de acidosis tubular renal y el tratamiento adecuado que repercutirá en el pronóstico vital del niño

Aetiology of hyperkalaemia in hospital inpatients.

BACKGROUND: Hyperkalaemia is a common metabolic disorder; if left untreated it can lead to life-threatening consequences. We conducted this study to determine the common aetiological factors for hyperkalaemia in hospital inpatients. METHODS: This prospective cross-sectional study was conducted in a referral teaching hospital in south India. One hundred and forty-three patients with hyperkalaemia (> 5 mEq/L) were selected on 20 random week days over a 3-month period. All the patients were clinically and biochemically evaluated for the aetiology of hyperkalaemia. RESULTS: Hyperkalaemia was twice as common amongst males. Potassium supplementation and drugs were the leading causes for hyperkalaemia, with renal failure being a distant second. Hyperkalaemia developed after admission to hospital in more than 75% of the patients. Severe hyperkalaemia (> 6 mEq/L) was seen in one-third of the patients. CONCLUSION: Potassium supplementation and other iatrogenic conditions lead to hyperkalaemia in inpatients. Males are at increased risk for hyperkalaemia.