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.

Regulation of kidney on potassium balance and its clinical significance / 生理学报

Virtually all of the dietary potassium intake is absorbed in the intestine, over 90% of which is excreted by the kidneys regarded as the most important organ of potassium excretion in the body. The renal excretion of potassium results primarily from the secretion of potassium by the principal cells in the aldosterone-sensitive distal nephron (ASDN), which is coupled to the reabsorption of Na+ by the epithelial Na+ channel (ENaC) located at the apical membrane of principal cells. When Na+ is transferred from the lumen into the cell by ENaC, the negativity in the lumen is relatively increased. K+ efflux, H+ efflux, and Cl- influx are the 3 pathways that respond to Na+ influx, that is, all these 3 pathways are coupled to Na+ influx. In general, Na+ influx is equal to the sum of K+ efflux, H+ efflux, and Cl- influx. Therefore, any alteration in Na+ influx, H+ efflux, or Cl- influx can affect K+ efflux, thereby affecting the renal K+ excretion. Firstly, Na+ influx is affected by the expression level of ENaC, which is mainly regulated by the aldosterone-mineralocorticoid receptor (MR) pathway. ENaC gain-of-function mutations (Liddle syndrome, also known as pseudohyperaldosteronism), MR gain-of-function mutations (Geller syndrome), increased aldosterone levels (primary/secondary hyperaldosteronism), and increased cortisol (Cushing syndrome) or deoxycorticosterone (hypercortisolism) which also activate MR, can lead to up-regulation of ENaC expression, and increased Na+ reabsorption, K+ excretion, as well as H+ excretion, clinically manifested as hypertension, hypokalemia and alkalosis. Conversely, ENaC inactivating mutations (pseudohypoaldosteronism type 1b), MR inactivating mutations (pseudohypoaldosteronism type 1a), or decreased aldosterone levels (hypoaldosteronism) can cause decreased reabsorption of Na+ and decreased excretion of both K+ and H+, clinically manifested as hypotension, hyperkalemia, and acidosis. The ENaC inhibitors amiloride and Triamterene can cause manifestations resembling pseudohypoaldosteronism type 1b; MR antagonist spironolactone causes manifestations similar to pseudohypoaldosteronism type 1a. Secondly, Na+ influx is regulated by the distal delivery of water and sodium. Therefore, when loss-of-function mutations in Na+-K+-2Cl- cotransporter (NKCC) expressed in the thick ascending limb of the loop and in Na+-Cl- cotransporter (NCC) expressed in the distal convoluted tubule (Bartter syndrome and Gitelman syndrome, respectively) occur, the distal delivery of water and sodium increases, followed by an increase in the reabsorption of Na+ by ENaC at the collecting duct, as well as increased excretion of K+ and H+, clinically manifested as hypokalemia and alkalosis. Loop diuretics acting as NKCC inhibitors and thiazide diuretics acting as NCC inhibitors can cause manifestations resembling Bartter syndrome and Gitelman syndrome, respectively. Conversely, when the distal delivery of water and sodium is reduced (e.g., Gordon syndrome, also known as pseudohypoaldosteronism type 2), it is manifested as hypertension, hyperkalemia, and acidosis. Finally, when the distal delivery of non-chloride anions increases (e.g., proximal renal tubular acidosis and congenital chloride-losing diarrhea), the influx of Cl- in the collecting duct decreases; or when the excretion of hydrogen ions by collecting duct intercalated cells is impaired (e.g., distal renal tubular acidosis), the efflux of H+ decreases. Both above conditions can lead to increased K+ secretion and hypokalemia. In this review, we focus on the regulatory mechanisms of renal potassium excretion and the corresponding diseases arising from dysregulation.

Persistent and serious hyperkalemia after surgery of primary aldosteronism: A case report / 北京大学学报(医学版)

Hyperkalemia was one of the complications after primary aldosteronism surgery. Hyperkalemia after primary aldosteronism surgery was uncommon in clinical practice, especially persistent and serious hyperkalemia was rare. This complication was not attached great importance in clinical work. A case about persistent and serious hyperkalemia after primary aldosteronism adrenal adenoma surgery was reported and the patient was followed-up for fourteen months in this study. This patient had a laparoscopic adrenalectomy due to primary aldosteronism. Hyperkalemia was detected one month after surgery of this patient, the highest level of plasma potassium was 7.0 mmol/L. The patient felt skin itchy, nausea, palpitation. Plasma aldosterone concentration fell to 2.12 ng/dL post-operation from 35.69 ng/dL pre-operation, zona glomerulosa insufficiency was confirmed by hormonal tests in this patient after surgery. And levels of 24 hours urinary potassium excretion declined. Decrease of aldosterone levels after surgery might be the cause of hyperkalemia. Hyperkalemia lasted for 14 months after surgery and kalemia-lowering drugs were needed. A systemic search with "primary aldosteronism", "hyperkalemia", "surgical treatment" was performed in PubMed and Wanfang Database for articles published between January 2009 and December 2019. Literature review indicated that the incidence of hyperkalemia after primary aldosteronism surgery was 6% to 29%. Most of them was mild to moderator hyperkalemia (plasma potassium 5.5 to 6.0 mmol/L) and transient. 19% to 33% in hyperkalemia patients was persistent hyperkalemia. Previous studies in the levels of plasma potassium reached the level as high as 7 mmol/L in our case were rare. Whether hypoaldosteronemia was the cause of hyperkalemia was not consistent in the published studies. Risk factors of hyperkalemia after primary aldosteronism surgery included kidney dysfunction, old age, long duration of hypertention. This paper aimed to improve doctors' aweareness of hyperkalemia complication after primary aldosteronism surgery. Plasma potassium should be monitored closely after primary aldosteronism surgery, especially in the patients with risk factors. Some patients could have persistent and serious hyperkalemia, and need medicine treatment.

Hiponatremia e hiperpotasemia persistente inducida por enoxaparina y revertida con hidrocortisona en paciente con metástasis lumbares / Low molecular weight heparin-induced hyperkalemia and hyponatremia: report of one case

Low molecular weight heparin-induced hyperkalemia is not an uncommon side effect. The development of hyponatremia is well described although it is less common. We report a 72-year-old woman with lumbar metastases who developed hyponatremia and hyperkalemia on the tenth day of hospitalization. Hyponatremia, with limited criteria for syndrome of inappropriate secretion of antidiuretic hormone, did not resolve with vigorous volume restriction. Hyperkalemia without an acid-base disorder or baseline renal failure, did not resolve after losartan was stopped. Enoxaparin-induced hypoaldosteronism was proposed and the drug was discontinued. After four days' persistence of the electrolyte disturbance, dexamethasone was changed to Hydrocortisone, and parameters normalized in 24 hours. The patient remained well until discharge and during outpatient control.

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.

Hipodermóclise para correção de desidratação hiperosmolar e distúrbios eletrolíticos graves: relato de caso / Hypodermoclysis for correction of hyperosmolar dehydration and severe electrolyte disturbances: a case report

O tratamento de desidratação e distúrbios eletrolíticos graves é contraindicado por meio de hipodermóclise, mas o nível de evidência da recomendação se baseia na opinião de especialistas. Descreve-se caso clínico de paciente com idade avançada, com indicação de cuidados paliativos, que evoluiu com desidratação hiperosmolar com hipernatremia grave, injúria renal aguda e hipercalemia. Tratou-se a paciente com solução hipotônica por meio da técnica de hipodermóclise, com resolução completa do quadro. Conclui-se que há necessidade de mais estudos sobre o uso da hipodermóclise para o tratamento de distúrbios hidroeletrolíticos graves.

The treatment of dehydration and severe electrolyte disturbances using hypodermoclysis is usually contraindicated, but the level of evidence for its recommendation is based on expert opinion. This case report describes an older patient receiving palliative care who developed hyperosmolar dehydration with severe hypernatremia, acute kidney injury, and hyperkalemia. She was treated with hypodermoclysis using a hypotonic solution, which led to complete resolution of the condition. In conclusion, further studies on the use of hypodermoclysis for the treatment of severe hydroelectrolytic disorders are needed.

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.

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.

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.

Transient Pseudohypoaldosteronism in a 5-Month-old Infant Manifested as a Failure to Thrive / 이화의대지

Pseudohypoaldosteronism (PHA) in infants is manifested by presence of hyperkalemia, hyponatremia, and metabolic acidosis. At initial stages, PAH is generally suspected as congenital adrenal hyperplasia. Transient PHA has been reported in infants with urinary tract infection and urinary tract malformation. We report a case of 5-month-old infant with failure to thrive and finally diagnosed with transient PHA due to urinary tract infection with vesicoureteral reflux.

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.

Combination of extracorporeal membrane oxygenation and in-line hemofiltration for the acute hyperkalemic cardiac arrest in a patient with Duchenne muscular dystrophy following orthopedic surgery: a case report / 대한마취과학회지

BACKGROUND: Duchenne muscular dystrophy (DMD) is the most common childhood muscular dystrophy that anesthesiologists can encounter in the operation room, and patients with DMD are susceptible to complications such as rhabdomyolysis, hyperkalemic cardiac arrest, and hyperthermia during the perioperative period. Acute onset of hyperkalemic cardiac arrest is a crisis because of the difficulty in achieving satisfactory resuscitation owing to the sustained hyperkalemia accompanied by rhabdomyolysis. CASE: We here report a case of a 13-year-old boy who had multiple leg fractures and other trauma after a car accident and who had suffered from acute hyperkalemic cardiac arrest. He was refractory to cardiopulmonary resuscitation and showed sustained hyperkalemia. With extracorporeal membrane oxygenation and in-line hemofiltration, he recovered from repeated cardiac arrest and hyperkalemia. CONCLUSIONS: Combining ECMO and in-line hemofiltration might be a safe and effective technique for refractory hyperkalemic cardiac arrest and rhabdomyolysis in patients with DMD.

A physiology-based approach to a patient with hyperkalemic renal tubular acidosis / Abordagem diagnóstica de um paciente com acidose tubular renal hipercalêmica

ABSTRACT Hyperkalemic renal tubular acidosis is a non-anion gap metabolic acidosis that invariably indicates an abnormality in potassium, ammonium, and hydrogen ion secretion. In clinical practice, it is usually attributed to real or apparent hypoaldosteronism caused by diseases or drug toxicity. We describe a 54-year-old liver transplant patient that was admitted with flaccid muscle weakness associated with plasma potassium level of 9.25 mEq/L. Additional investigation revealed type 4 renal tubular acidosis and marked hypomagnesemia with high fractional excretion of magnesium. Relevant past medical history included a recent diagnosis of Paracoccidioidomycosis, a systemic fungal infection that is endemic in some parts of South America, and his outpatient medications contained trimethoprim-sulfamethoxazole, tacrolimus, and propranolol. In the present acid-base and electrolyte case study, we discuss a clinical approach for the diagnosis of hyperkalemic renal tubular acidosis and review the pathophysiology of this disorder.

RESUMO A acidose tubular renal hipercalêmica é uma acidose metabólica de ânion gap normal que invariavelmente indica anormalidade na secreção de íons potássio, amônio e hidrogênio. Na prática clínica, está geralmente atribuída a um estado de hipoaldosteronismo real ou aparente, causado por doenças ou toxicidade por drogas. Descrevemos um paciente de 54 anos, transplantado hepático, que foi admitido com fraqueza muscular associada à hipercalemia, potássio plasmático de 9,25 mEq/L. A investigação adicional revelou acidose tubular renal tipo 4 e importante hipomagnesemia com elevada fração de excreção de magnésio. A história patológica pregressa incluía um diagnóstico recente de Paracoccidioidomicose - uma infecção sistêmica fúngica endêmica que ocorre em algumas partes da América do Sul -, e as medicações de uso habitual continham sulfametoxazol-trimetoprim, tacrolimus e propranolol. No presente relato de caso, discutiremos uma abordagem clínico-laboratorial para o diagnóstico da acidose tubular renal hipercalêmica, assim como da hipomagnesemia, revisando a fisiopatologia desses transtornos.

Rectal ulcer due to Kayexalate deposition - an unusual case

SUMMARY Sodium polystyrene sulfonate (PSP) or Kayexalate is a cation-exchange resin, widely used in the management of hyperkalaemia due to renal disease. A rare, yet potentially dangerous, adverse event related to sodium polystyrene sulfonate use is intestinal mucosal injury, especially in the colon. The injury to the gastrointestinal mucosa can range from mild and superficial to wall necrosis and bowel perforation. The mechanism that leads to mucosal damage remains unclear. However, it is believed that sorbitol, commonly given to counteract PSP's tendency to cause constipation, may play an important role in the development of gastrointestinal injury. Other potential risk factors are uraemia or end-stage renal disease, hemodynamic instability, solid organ transplantation, postoperative status and concomitant opioid administration. The authors present a case of diarrhoea and haematochezia after the administration of PSP without sorbitol, in a patient with hyperkalaemia due to acute kidney injury, in the absence of other risk factors. A colonoscopy was performed and revealed a rectal ulcer which histological findings were suggestive of mucosal injury due to Kayexalate deposition. This case supports the concept that this widely used drug can itself, without sorbitol, cause injury to the gastrointestinal wall. Even though this is a rare adverse effect, the widespread use of this medication may put a large population at risk.

RESUMO O polistireno sulfonato de sódio (PSP) ou kayexalato é uma resina de troca iônica, amplamente usada no tratamento da hipercalemia associada à doença renal. Um efeito adverso raro, mas potencialmente grave, dessa terapêutica é a agressão à parede do trato gastrointestinal, principalmente ao nível do cólon, que pode ser ligeira e superficial ou culminar em necrose e perfuração intestinal. O mecanismo pelo qual o PSP lesa a mucosa intestinal não é totalmente conhecido. Contudo, pensa-se que o sorbitol, frequentemente administrado em simultâneo para contrabalançar o efeito obstipante do PSP, possa ter um papel preponderante no desenvolvimento de lesão gastrointestinal. Outros potenciais fatores de risco são a presença de uremia ou doença renal em estágio terminal, instabilidade hemodinâmica, pós-operatório, pós-transplante renal e a administração concomitante de opioides. Os autores descrevem um caso de diarreia e hematoquesias após a administração de PSP sem sorbitol, numa paciente com hipercalemia secundária a lesão renal aguda, sem outros fatores de risco para o desenvolvimento desse efeito adverso. A investigação etiológica com colonoscopia revelou a presença de uma úlcera retal, cujo estudo histológico foi compatível com lesão por deposição de cristais de kayexalato. Este relato incomum reforça o conceito de que este fármaco de uso frequente, mesmo na ausência de sorbitol, pode ser lesivo para a mucosa intestinal. Assim, e apesar de este ser um efeito adverso raro, a utilização difundida do PSP coloca uma vasta população em risco.

Intraoperative management of liver transplant recipients having severe renal dysfunction: results of 42 cases

PURPOSE: Whereas continuous renal replacement therapy (CRRT) has been utilized during liver transplantation (LT), there was a lack of evidence to support this practice. We investigated the adverse events at the perioperative periods in recipients of LT who received preoperative CRRT without intraoperative CRRT. METHODS: We retrospectively reviewed medical records of adult patients (age ≥ 18 years) who received LT between December 2009 and May 2015. Perioperative data were collected from the recipients, who received preoperative CRRT until immediately before LT, because of refractory renal dysfunction. RESULTS: Of 706 recipients, 42 recipients received preoperative CRRT. The mean (standard deviation) Model for end-stage liver disease score were 49.6 (13.4). Twenty-six point two percent (26.2%) of recipients experienced the serum potassium > 4.5 mEq/L before reperfusion and treated with regular insulin. Thirty-eight point one percent (38.1%) of recipients were managed with sodium bicarbonate because of acidosis (base excess 5.5 mEq/L), refractory acidosis, or critical arrhythmias. Mortality was 19% at 30 day and 33.3% at 1 year. CONCLUSION: Although intraoperative CRRT was not used in recipients with severe preoperative renal dysfunction, LT was safely performed. Our experience raises a question about the need for intraoperative CRRT.

Common features of atopic dermatitis with hypoproteinemia / 소아과

PURPOSE: The purpose of this study was to identify the causes, symptoms, and complications of hypoproteinemia to prevent hypoproteinemia and provide appropriate treatment to children with atopic dermatitis. METHODS: Children diagnosed with atopic dermatitis with hypoproteinemia and/or hypoalbuminemia were retrospectively reviewed. The patients’ medical records, including family history, weight, symptoms, treatment, complications, and laboratory test results for allergies and skin cultures, were examined. RESULTS: Twenty-six patients (24 boys) were enrolled. Seven cases had growth retardation; 7, keratoconjunctivitis; 6, aural discharges; 5, eczema herpeticum; 4, gastrointestinal tract symptoms; and 2, developmental delays. In 21 cases, topical steroids were not used. According to the blood test results, the median values of each parameter were elevated: total IgE, 1,864 U/mL; egg white-specific IgE, 76.5 kU(A)/L; milk IgE, 20.5 kU(A)/L; peanut IgE, 30 kU(A)/L; eosinophil count, 5,810/μL; eosinophil cationic protein, 93.45 μg/L; and platelet count, 666.5×10³/μL. Serum albumin and total protein levels decreased to 2.7 g/dL and 4.25 g/dL, respectively. Regarding electrolyte abnormality, 10 patients had hyponatremia, and 12, hyperkalemia. Systemic antibiotics were used to treat all cases, and an antiviral agent was used in 12 patients. Electrolyte correction was performed in 8 patients. CONCLUSION: Hypoproteinemia accompanying atopic dermatitis is common in infants younger than 1 year and may occur because of topical steroid treatment continuously being declined or because of eczema herpeticum. It may be accompanied by growth retardation, keratoconjunctivitis, aural discharge, and eczema herpeticum and can be managed through skin care and topical steroid application without intravenous albumin infusion.

Prevalence and risk factors of hyperkalemia after liver transplantation / Prevalência e fatores de risco associados à hipercalemia após o transplante hepático

ABSTRACT Background: There is a lack of data regarding hyperkalemia after liver transplantation. Aim: To evaluate the prevalence of hyperkalemia after liver transplantation and its associated factors. Methods: This retrospective cohort study evaluated 147 consecutive post-transplant patients who had at least one year of outpatient medical follow up. The data collection included gender, age, potassium values, urea, creatinine, sodium and medication use at 1, 6 and 12 months after. Hyperkalemia was defined as serum potassium concentrations higher than 5.5 mEq/l. Results: Hiperkalemia was observed in 18.4%, 17.0% and 6.1% of patients 1, 6 and 12 months after tranplantation, respectively. Older age (p=0.021), low creatinine clearance (p=0.007), increased urea (p=0.010) and hypernatremia (p=0.014) were factors associated with hyperkalemia, as well as the dose of prednisone at six months (p=0.014). Conclusion: Hyperkalemia was prevalent in less than 20% of patients in the 1st month after liver transplantation and decreased over time. Considering that hyperkalemia does not affect all patients, attention should be paid to the routine potassium intake recommendations, and treatment should be individualized.

RESUMO Racional: Existe lacuna na literatura em relação à presença de hipercalemia em pacientes submetidos ao transplante hepático. Objetivo: Avaliar a prevalência de hipercalemia após o transplante hepático; e avaliar os fatores de risco associados a essa condição. Métodos: Estudo de coorte retrospectivo, no qual foram avaliados 147 pacientes submetidos ao transplante hepático, com tempo de seguimento de um ano. A coleta de dados compreendeu gênero, idade, valores séricos de potássio, ureia, creatinina, sódio e medicamentos utilizados no tempo de 1, 6 e 12 meses após o transplante. Hipercalemia foi definida como valores de potássio sérico maiores do que 5,5 mEq/l. Resultados: Hipercalemia foi observada em 18,4%, 17,0% e 6,1% dos pacientes no 1º, 6º e 12º meses após o transplante, consecutivamente. Idade avançada (p=0,021), baixos valores de creatinina sérica (p=0,007), valores aumentados de ureia (p=0,010) e hipernatremia (p=0,014) foram fatores associados à hipercalemia, assim como a dose de prednisona no 6º mês após o transplante (p=0,014). Conclusão: Hipercalemia esteve presente em menos de 20% dos pacientes no primeiro mês após o transplante hepático, com diminuição da prevalência ao longo do tempo. Considerando que ela não afeta todos os pacientes, o tratamento dietético deve ser individualizado, com especial atenção à recomendação de administração de potássio de forma rotineira.