1.A Case of Autosomal Dominant Nephrogenic Diabetes Insipidus with Renal Failure.
Hag Ei KIM ; Hye Suk HAN ; Byung Chul KIM ; Joungho HAN ; Sung Soon PARK ; Min Ok KIM ; Soon Kil KWON ; Hye Young KIM ; Sung Soo KOONG
Korean Journal of Nephrology 2004;23(6):965-969
Congenital nephrogenic diabetes insipidus (NDI) is a rare inherited disorder. The X-linked recessive NDI, most of congenital DI (>90%), has been known to be caused by mutation of the AVPR2 gene. Less than 10% of congenital NDI has an autosomal recessive inheritance and mutations of the AQP2 gene. Although several adult cases of congenital NDI were reported in Korea, all of them had an X-linked recessive inheritance and no azotemia. We have experienced a case of autosomal dominant NDI with renal failure. A 38-year-old man was admitted due to polyuria and polydipsia. In his family, his grandfather, father, three sisters and nieces showed similar symptoms. On admission, serum creatinine level was 4.9 mg/dL and creatinine clearance decreased to 19.9 mL/min. On ultrasonography, both kidneys showed severe hydronephrosis. We performed water deprivation and vasopressin stimulation test, and the result was consistent with NDI. On gene mutational analysis of the two different genes, AVPR2 and AQP2, no specific mutations were found except polymorphism.
Adult
;
Azotemia
;
Creatinine
;
Diabetes Insipidus, Nephrogenic*
;
Fathers
;
Humans
;
Hydronephrosis
;
Kidney
;
Korea
;
Polydipsia
;
Polyuria
;
Renal Insufficiency*
;
Siblings
;
Ultrasonography
;
Vasopressins
;
Water Deprivation
;
Wills
2.A Case of Sclerosing Peritonitis Successfully Treated with Corticosteroid Therapy.
Su In YOON ; Hye Young KIM ; Min Ok KIM ; Hag Ei KIM ; Ki Won MOON ; Joung Ho HAN ; Jisook HAHN ; Hoe Bok CHAI ; Soon Kil KWON ; Il Hun BAE
Korean Journal of Nephrology 2004;23(1):169-173
Sclerosing peritonitis is a rare but fatal complication of peritoneal dialysis (PD). Management of sclerosing peritonitis includes cessation of PD, total parenteral nutrition, and surgery. Recently, a few reports have indicated immunosuppression might be beneficial in sclerosing peritonitis. In these reports, all of patients had the combination therapy of steroid and immunosuppressant. A 37-year old man develped sclerosing peritonitis 3 months after switching from PD to hemodialysis because of uncontrolled peritonitis. An abdominal computed tomography (CT) scan demonstrated massive ascites with multilocuated fluid collection and extensive enhancement of the peritoneum. A peritoneal biopsy showed proliferation of fibrous collagenous tissue with infiltration of lymphocytes. We started corticosteroid for one month. A follow-up CT scan showed complete resolution with absence of peritoneum thickness and fluid collection 16 months after corticosteroid therapy. The patient currently remains free of symptoms in an outpatient hemodialysis unit. To our knowledge, this is the first case of sclerosing peritonitis successfully treated with corticosteroid therapy alone in Korea.
Adult
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Ascites
;
Biopsy
;
Collagen
;
Follow-Up Studies
;
Humans
;
Immunosuppression
;
Korea
;
Lymphocytes
;
Outpatients
;
Parenteral Nutrition, Total
;
Peritoneal Dialysis
;
Peritoneum
;
Peritonitis*
;
Renal Dialysis
;
Tomography, X-Ray Computed
3.A Case of Multiple Renal Arteries Presented with the Manifestations of Renovascular Hypertension.
Ki Won MOON ; Su In YOON ; Joungho HAN ; Jisook HAHN ; Hag Ei KIM ; Min Ok KIM ; Soon Kil KWON ; Hye Young KIM
Korean Journal of Nephrology 2004;23(5):825-829
In 17-30% of subjects, at least one kidney is supplied by more than one artery arising from the aorta. Subjects with multiple renal arteries have been reported to suffer more frequently from hypertension, But the precise association between hypertension and multiple renal arteries was not yet defined. A 20- year old woman presented clinical manifestations of renovascular hypertension. Basal renin activity was elevated, and time-activity curves showed delayed peak time at captopril renal scan. Angiography showed multiple renal arteries with 2 right and left 3 arteries. There was neither stenosis nor inflammation. We strated angiotensin-receptor blocker, calcium channel blocker, and beta-blocker. The patient currently remains normotensive in an outpatient unit. In general, accessory renal arteries are narrower and longer than main artery. As a results, the renal segments supplied by accessory vessels might have lower levels of blood pressure than the remainder of the parenchyma, thereby increasing the renin secretion. So hypertension associated with multiple renal arteries might be involved in renin-angiotensin-aldosterone system activation.
Angiography
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Aorta
;
Arteries
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Blood Pressure
;
Calcium Channels
;
Captopril
;
Constriction, Pathologic
;
Female
;
Humans
;
Hypertension
;
Hypertension, Renovascular*
;
Inflammation
;
Kidney
;
Outpatients
;
Renal Artery*
;
Renin
;
Renin-Angiotensin System
4.A Case of Acute Renal Failure Caused by Cresol Ingestion.
Hye Suk HAN ; Hag Ei KIM ; Young Ook EUM ; Su In YOON ; Joungho HAN ; Ki Won MOON ; Soon Kil KWON ; Hye Young KIM ; Kyung Sook LEE
Korean Journal of Nephrology 2005;24(2):342-345
Cresol, a commonly used disinfectant, is an extremly toxic material and can cause systemic effects such as respiratory, neurological, gastrointestinal, hepatic and renal damage. Cresol intoxication can be caused by inhalation, cutaneous adsorption or oral ingestion. Cresol denatures and precipitates cellular proteins, thus exer ts their toxic effects directly on all cells. There is only one case of cresol intoxication in Korea, but a case of chemical burn. We report a case of acute renal failure caused by cresol ingestion. A 39-year-old male attempted suicide by ingesting 200 mL of 50% cresol solution. Acute renal failure developed, but he recovered by only supportive care. It was reported that the lethal dose of saponated cresol solution is approximately 60-120 mL and the lethal blood level is 71-190 microgram/mL. The amount of cresol ingested by the patient far exceeded the reported lethal dose. This is the first case of acute renal failure caused by cresol ingestion successfully treated with only supportive care in Korea.
Acute Kidney Injury*
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Adsorption
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Adult
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Burns, Chemical
;
Eating*
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Humans
;
Inhalation
;
Korea
;
Male
;
Suicide, Attempted
5.Relationship between Initial Leukocyte Count and the Extent of Myocardial Injury in Patients with Acute Myocardial Infarction Who Received Reperfusion Therapy.
Joungho HAN ; Tae Jin YOUN ; Eui Sil HONG ; Su In YOON ; Ki Won MOON ; Hag Ei KIM ; Jisook HAHN ; Ki Seok KIM ; Jangwhan BAE ; Kyung Kuk HWANG ; Dong Woon KIM ; Myeong Chan CHO
Korean Circulation Journal 2004;34(8):735-742
BACKGROUND AND OBJECTIVES: The presence of leukocytosis in patients with acute myocardial infarction (AMI) has been reported to be related to the extent of MI and with the prognosis. However, whether the leukocytosis itself is a cause or result of the myocardial injury has not been determined. The relationship between the leukocyte count and the extent of myocardial injury was investigated in patients with AMI that had undergone reperfusion therapy. SUBJECTS AND METHODS: Patients with AMI that had undergone thrombolysis (n=60) or primary PCI (n=36) were included. The initial leukocyte counts were analyzed with regard to the peak and initial CK-MB levels. The relationship between leukocytosis and the time elapsed from the onset of symptoms, infarct related coronary arteries and the proximity of the lesions were also investigated. RESULTS: In both groups, the initial leukocyte count did not show a significant relationship with the initial CK-MB level or the time elapsed from symptoms onset, which could be an indication of the extent of early myocardial injury. Furthermore, no significant relationship was shown with the infarct related coronary artery or proximity of the lesion. However, a relationship was shown with the maximum CK-MB level, which could be an indication of the extent of myocardial injury following reperfusion therapy in both groups (p<0.01). CONCLUSION: This study suggests that the initial leukocyte count in patients with AMI might is an important prognostic factor that determines the extent of myocardial injury following reperfusion therapy, rather than being a simple indicator of the extent of early myocardial injury.
Coronary Vessels
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Humans
;
Leukocyte Count*
;
Leukocytes*
;
Leukocytosis
;
Myocardial Infarction*
;
Myocardial Reperfusion
;
Prognosis
;
Reperfusion*