• Title/Summary/Keyword: Hyperreninemia

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Wilms' tumor with polydipsia, polyuria, hyponatremic hypertension and congestive heart failure : a case report (다음, 다뇨, 저나트륨성 고혈압, 심부전증을 보이는 윌름씨 종양 1례)

  • Woo, Chul Hee;Jang, Ji Min;Woo, Chan Wook;Lee, Ki Hyung;Lee, Kwang Chul
    • Clinical and Experimental Pediatrics
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    • v.49 no.1
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    • pp.99-102
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    • 2006
  • A 3-year-old girl presented with polydipsia, polyuria, hyponatremia, hypertension and congestive heart failure. Her polyuria was unresponsive to water restriction and vasopressin challenge tests, and her blood pressure was not effectively controlled by antihypertensive drugs. Radiologic examinations revealed a Wilms' tumor in the right kidney. Her plasma renin activity and aldosterone concentration were greatly increased. After surgical removal of the tumor, the congestive heart failure disappeared. Congestive heart failure due to Wilms' tumor is very rare and we report here on such a case, with a brief review of the literature.

A Case of Bartter Syndrome with Muscle Weakness and Short Stature (근무력증과 왜소증을 동반한 Bartter syndrome 1례)

  • Kim In-Sung;Kang Ju-Hyung;Shin Yun-Hei;Lee Dong-Kuk;Kim Soon-Nam;Pai Ki-Soo
    • Childhood Kidney Diseases
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    • v.6 no.2
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    • pp.259-265
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    • 2002
  • Bartter syndrome is a rare disorder characterized by the association of hypokalemic hypochloremic metabolic alkalosis, hyperreninemia, hyperaldosteronemia, short stature and nephrocalcinosis. This disorder presents with hyperplasia of juxtaglomerular apparatus on renal biopsy. We experienced a case of late-onset Bartter syndrome with nephrocalcinosis in a 9-year-old boy, whose chief pictures were muscle weakness, short stature, persistent sterile pyuria and microscopic hematuria. We report this case with a brief review of related literatures.

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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.

Hypertension, Polydipsia, Polyuria, and Hypokalemia Associated with Renin-Secreting Wilms' Tumor in a Child (소아에서 고혈압, 번갈 다음증, 다뇨증, 및 저칼륨증을 유발한 레닌-분비성 윌름스 종양(Wilms' Tumor))

  • Choi, Young-Whan;Lee, Nam-Hyuk;Kim, Sang-Youn;Kwak, Jung-Sik
    • Advances in pediatric surgery
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    • v.3 no.1
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    • pp.71-76
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    • 1997
  • A 3-year-old boy with a Wilms' tumor had unusually severe hypertension, polydipsia, polyuria and hypokalemia. Physical examination on admission was unremarkable except for the presence of a smooth, firm mass in the right abdomen. Computerized tomography showed a tumor occupying the upper two thirds of the right kidney. Plasma renin activity and aldosterone concentration were markedly elevated, 37.7 mg/ml/hour(normal in supine position 0.15-2.33 mg/ml/hour) and 120.1 ng/dL(normal in supine position 1 to 16 ng/dL), respectively. Hypertension varied from 150/90 mmHg to 240/180 mmHg, and was not effectively controlled by antihypertensive drugs. Right nephrectomy was performed on the sixth hospital day. At laparatomy, there was no evidence of mechenical compression of the renal artery by the tumor. The tumor, about 8 cm in diameter, was confined to the renal capsule without involvement of the renal blood vessels at the hilum. Histopathology was Wilms' tumor of favorable histology. On electron microscopy, tumor cells contained intracytoplasmic electron dense secreting graules, suggesting the possibility of renin secreting tumor cells. Shortly after nephrectomy, signs and symptoms were relieved dramatically, and plasma renin activity and aldosterone concentration were also decreased to normal.

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The clinical manifestations, the short- and long-term outcomes of Bartter syndrome (Bartter 증후군의 임상 양상과 장단기 치료 결과)

  • Park, Hye Won;Lee, Joo Hoon;Park, Young Seo
    • Clinical and Experimental Pediatrics
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    • v.50 no.12
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    • pp.1231-1240
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    • 2007
  • Purpose : Bartter syndrome is a renal tubular defect in electrolyte transport characterized by hypokalemia, metabolic alkalosis and other clinical signs and symptoms. The aims of this study were to analyze the clinical manifestations and the short- and long-term outcomes of Bartter syndrome. Methods : We retrospectively reviewed clinical history, laboratory finding of blood and urine, renal ultrasonography, and hearing tests of five patients who were diagnosed and managed with Bartter syndrome at Asan Medical Center from April 1992 to May 2007. We also evaluated height and body weight periodically after institution of therapy. Results : All patients had poor oral intake, failure to thrive and polyuria. Three of them had maternal history of polyhydramnios and premature delivery. The mean age at diagnosis was 11.8 months. All children presented with hypokalemia, metabolic alkalosis, hyperreninemia. Their blood pressures were normal. One patient had nephrocalcinosis on renal ultrasonography and all of them had normal result in hearing tests. After treatment with indomethacin or other prostaglandin inhibitors and potassium supplementation,their clinical features improved with catch-up growth and improvement in the development during long-term follow-up. Conclusion : We emphasize that early diagnosis and proper treatment in patient with Bartter syndrome are related to better prognosis.

DENTAL TREATMENT OF A PATIENT WITH BARTTER SYNDROME: CASE REPORT (Bartter 증후군 환아의 치아우식 치료: 증례보고)

  • Kim, Minji;Song, Ji-Soo;Shin, Teo Jeon;Hyun, Hong-Keun;Kim, Young-Jae;Kim, Jung-Wook;Lee, Sang-Hoon;Jang, Ki-Taeg
    • The Journal of Korea Assosiation for Disability and Oral Health
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    • v.15 no.1
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    • pp.45-49
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    • 2019
  • Bartter syndrome is an inherited renal tubular disorder characterized by hypokalemia, hypochloremic metabolic alkalosis, hyperreninemia, hyperprostaglandinism, and normal blood pressure. Bartter syndrome is classified by neonatal and classic type. Clinical manifestation of Bartter syndrome considered in dental treatment include tendency to dehydration, vomiting and erosion, attrition and abrasion of the teeth. A 2-year-4 month old boy with Bartter syndrome type III was referred to the Seoul National University Dental Hospital for dental evaluation and treatment. He showed hypokalemic hypochloremic metabolic alkalosis and had been treated with indomethacin and potassium chloride. He had hypocalcified teeth with or without multisurface caries lesions in all dentition and the erosion of maxillary teeth was detected. Dental procedure under general anesthesia was scheduled due to multiple caries and his medical condition. The dental procedure was successfully performed. This case suggests that electrolyte imbalances need to be treated prior to dental treatment and complete coverage restoration is necessary to protect the eroded teeth. An appropriate management plan for the patients with Bartter syndrome should include considerations of the need for close interaction with the pediatrician for pre- and post-operative care. General anesthesia may be recommendable to manage the patients having multiple caries with Bartter syndrome.