3.Neonatal systemic pseudohypoaldosteronism type I.
Xin-Cheng CAO ; Yuan-Yuan CHEN ; Ke ZHANG ; Xun-Jie ZHANG ; Lin YANG ; Zhi-Hua LI
Chinese Journal of Contemporary Pediatrics 2023;25(7):774-778
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.
Infant
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Infant, Newborn
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Humans
;
Male
;
Pseudohypoaldosteronism/genetics*
;
Hyperkalemia/etiology*
;
Hyponatremia/diagnosis*
;
Diagnosis, Differential
4.Prediction of perioperative hyperkalemia in dialysis patients with secondary hyperparathyroidism.
Wei Jian OU ; Jing KANG ; Shuang Xin LIU ; Si Jia LI ; Shao Hua CHEN ; Si Yi ZHANG ; Ping Jiang GE
Chinese Journal of Otorhinolaryngology Head and Neck Surgery 2021;56(8):854-857
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.
Calcium
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Female
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Humans
;
Hyperkalemia/etiology*
;
Hyperparathyroidism, Secondary/surgery*
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Male
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Parathyroid Hormone
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Parathyroidectomy
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Renal Dialysis
;
Retrospective Studies
5.Hyperkalemia due to hyporeninemic hypoaldosteronism with liver cirrhosis and hypertension.
Choon Suhk KEE ; Jin Won CHOI ; Dae Kuk CHANG ; You Hern AHN ; Ho Jung KIM
Journal of Korean Medical Science 1993;8(6):464-470
A 49-year-old man with liver cirrhosis and hypertension was found to have hyperkalemia out of a degree of renal insufficiency and metabolic acidosis with low to normal anion gap, aggravated by volume contraction with diarrhea and medications (captopril, spironolactone and atenolol) interfering with potassium homeostasis. Plasma renin activity and serum aldosterone levels of this patient on a regular diet after discontinuation of medications were very low compared to those of five other cirrhotic patients with normokalemia as controls. Also, the renin-aldosterone stimulation testing on this patient performed by sodium restricted diet and furosemide, upright position and by angiotensin converting enzyme inhibition (captopril, 50 mg) showed the blunted renin and aldosterone responses to each of these stimuli, almost no changes from baseline renin and aldosterone levels, it was concluded that the underlying defect responsible for hyperkalemia in this case was hyporeninemic hypoaldosteronism and this was aggravated by other factors or drugs affecting potassium homeostasis.
Aldosterone/blood
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Captopril/pharmacology
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Furosemide/pharmacology
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Humans
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Hyperkalemia/*etiology
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Hypertension/*complications
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Hypoaldosteronism/*complications
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Liver Cirrhosis/*complications
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Male
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Middle Aged
;
Renin/blood
6.Colonic Mucosal Necrosis Following Administration of Calcium Polystryrene Sulfonate (Kalimate) in a Uremic Patient.
Mee JOO ; Won Ki BAE ; Nam Hoon KIM ; Seong Rok HAN
Journal of Korean Medical Science 2009;24(6):1207-1211
Colonic necrosis is known as a rare complication following the administration of Kayexalate (sodium polystryrene sulfonate) in sorbitol. We report a rare case of colonic mucosal necrosis following Kalimate (calcium polystryrene sulfonate), an analogue of Kayexalate without sorbitol in a 34-yr-old man. He had a history of hypertension and uremia. During the management of intracranial hemorrhage, hyperkalemia developed. Kalimate was administered orally and as an enema suspended in 20% dextrose water to treat hyperkalemia. Two days after administration of Kalimate enema, he had profuse hematochezia, and a sigmoidoscopy showed diffuse colonic mucosal necrosis in the rectum and sigmoid colon. Microscopic examination of random colonic biopsies by two consecutive sigmoidoscopies revealed angulated crystals with a characteristic crystalline mosaic pattern on the ulcerated mucosa, which were consistent with Kayexalate crystals. Hematochezia subsided with conservative treatment after a discontinuance of Kalimate administration.
Adult
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Colon/*pathology
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Gastrointestinal Hemorrhage/etiology
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Humans
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Hyperkalemia/drug therapy
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Intestinal Mucosa/*pathology
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Male
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Necrosis/*chemically induced/complications/pathology
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Polystyrenes/*adverse effects/therapeutic use
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Uremia/*physiopathology
7.Serum Electrolyte and Acid Base Composition in Patients with Graded Degrees of Chronic Renal Failure.
Ho Yung LEE ; Hyon Young JOO ; Dae Suk HAN
Yonsei Medical Journal 1985;26(1):39-43
One hundred sixty-three patients with graded degrees of uncomplicated stable chronic renal failure were studied to investigate the quantitative relationship between serum acid-base and electrolyte composition and serum creatinine level. Even in patients with a mild degree of renal failure, the serum total carbon dioxide (tCO2) content was reduced significantly. Progressive decrements in tCO2 were noted in the more severe degrees of renal failure with the reciprocal relationship between tCO2 and serum creatinine concentration. Depending upon the degree of chronic renal failure, the type of metabolic acidosis was different. In patients with a moderate degree of renal failure, hyperchloremic acidosis was noted with anion gap remaining normal. As the renal failure progressed to a more severe degree, this pattern of hyperchloremic acidosis changed to anion gap acidosis with a normal serum chloride level. The highest anion gap was 25 mEq/L in the patient with serum creatinine concentration 24.7mg/dL.
Acid-Base Equilibrium*
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Acidosis/etiology*
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Adult
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Carbon Dioxide/blood
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Chlorides/blood
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Creatinine/blood
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Electrolytes/blood*
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Human
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Hyperkalemia/etiology
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Kidney Failure, Chronic/blood*
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Kidney Failure, Chronic/complications
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Middle Age
;
Sodium/blood
8.WNKs: protein kinases with a unique kinase domain.
Chou Long HUANG ; Seung Kuy CHA ; Hao Ran WANG ; Jian XIE ; Melanie H COBB
Experimental & Molecular Medicine 2007;39(5):565-573
WNKs (with-no-lysine [K]) are a family of serine-threonine protein kinases with an atypical placement of the catalytic lysine relative to all other protein kinases. The roles of WNK kinases in regulating ion transport were first revealed by the findings that mutations of two members cause a genetic hypertension and hyperkalemia syndrome. More recent studies suggest that WNKs are pleiotropic protein kinases with important roles in many cell processes in addition to ion transport. Here, we review roles of WNK kinases in the regulation of ion balance, cell signaling, survival, and proliferation, and embryonic organ development.
Amino Acid Sequence
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Animals
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Cell Proliferation
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Cell Survival
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Humans
;
Hyperkalemia/enzymology/etiology/genetics
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Hypertension/enzymology/etiology/genetics
;
Kidney/enzymology
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Models, Molecular
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Molecular Sequence Data
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Mutation
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Neoplasms/enzymology/etiology/genetics
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Protein Structure, Tertiary
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Protein-Serine-Threonine Kinases/*chemistry/genetics/*metabolism
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Pseudohypoaldosteronism/enzymology/etiology/genetics
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Sequence Homology, Amino Acid
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Signal Transduction
;
Syndrome