• Title/Summary/Keyword: 고알도스테론혈증

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A Case of Pseudo-Bartter's Syndrome Due to Hypertrophic Pyloric Stenosis (비후성 유문 협착증에 의한 가성 Bartter 증후군 1례)

  • Kim, Yoon Hee;Lee, Tae Ho;Kim, Hong Bae
    • Clinical and Experimental Pediatrics
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    • v.45 no.11
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    • pp.1430-1434
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    • 2002
  • We report a 3-month old boy admitted to our hospital with Bartter's syndrome like symptoms and laboratory findings, which were vomiting, failure to thrive, hypochloremic and hypokalemic metabolic alkalosis associated with hyperreninemia, hyperaldosteronism and normal blood pressure. However, the urine chloride level was low. Hypertrophic pyloric stenosis was diagnosed through abdominal ultrasonography. Fredet-Ramstedt operation was done after electrolyte correction. After surgery he made a good recovery and gained body weight. The electrolytes maintained within a normal limit without any potassium supplementations after surgery. Differential diagnosis from Bartter's syndrome was made on the basis of a decrease in urine chloride and the non-necessity for potassium supplementation after surgery. It is relatively rare for hypertrophic pyloric stenosis to induce pseudo-Bartter's syndrome. The importance of considering this diagnosis in such cases is discussed.

Gitelman Syndrome with Normal Serum Magnesium (정상 마그네슘혈증의 Gitelman 증후군 1례)

  • Cheon, Younghee;Seo, Ji Hye;Cheong, Hae Il;Park, Yong Hoon
    • Childhood Kidney Diseases
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    • v.16 no.2
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    • pp.121-125
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    • 2012
  • Gitelman syndrome is an autosomal recessive renal tubular disorder characterized by hypokalemic metabolic alkalosis, and it is distinguished from Batter syndrome by hypomagnesemia and hypocalciuria. This disorder is caused by mutation in SLC12A3 gene which encodes thiazide-sensitive $Na^+-Cl^-$cotransporter (NCCT) which is expressed in the apical membrane of cells, lining distal convoluted tubule. A 8-year old boy who presented with Rolandic epilepsy, and horseshoe kidney accidentally showed clinical features of metabolic alkalosis, hypokalemia, hypocalciuria without hypomagnesemia. So we identified a heterozygote mutation and an abnormal splicing in the SLC12A3 gene, encoding NCCT. The mutation was detected in the exon 15 and 22 of SLC12A3 gene.

Adrenal Vein Sampling in Primary Aldosteronism: A Pictorial Essay for Optimal Left-Side Sampling (일차성고알도스테론혈증에서의 부신정맥채혈술: 최적의 좌측채혈을 위한 임상화보)

  • Gi Joo Kim;Myung Sub Kim;Hyun Pyo Hong;Young Rae Lee;Yeon Gyu Choi
    • Journal of the Korean Society of Radiology
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    • v.84 no.2
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    • pp.386-397
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    • 2023
  • Primary aldosteronism (PA) is a curable cause of hypertension. Recent studies have revealed that the actual prevalence of PA is higher than previously recognized. Adrenal vein sampling (AVS) is an essential diagnostic procedure for revealing the cause of PA and determining the treatment plan. The success of AVS is confirmed by comparing cortisol levels between the samples from each adrenal vein and peripheral vein. The failure rate of the procedure is reported to be high in the right adrenal vein, which is directly connected to the inferior vena cava, while that in the left adrenal vein is relatively low; however, this has rarely been reported. In this review, we introduce and analyze cases of failure in left adrenal vein sampling.

A Case Report of Conn's Myopathy (원발성 고알도스테론증에서 본 근육염 1례 (일명 : Conn's myopathy 1례))

  • Suh, Bo-Wan;O, Khyoung-Yhun;Byun, Young-Ju;Park, Choong-Suh;Kim, Hong-Jin
    • Journal of Yeungnam Medical Science
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    • v.4 no.1
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    • pp.133-137
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    • 1987
  • Myopathy in primary aldosteronism is relatively rare disease in Korea. A 42-year-lod woman with hypokalemic periodic paralysis, proximal muscle weakness and hypertension was found to have myopathy associated with adenoma in the right adrenal gland. She showed marked elevation of muscle enzymes and myopathic pattern in EMG.

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