• Journal of Chest Surgery
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• v.37 no.5
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• pp.416-422
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• 2004

Acute renal failure (ARF) is a common postoperative complication after the cardiac surgery. Postoperative ARF have various causes, and are combined with other complications rather than being the only a complication. It deteriorates the general condition of the patient, and makes it difficult to manage the combined complications by disturbing the adequate medication and fluid therapy. We have planned this study to evaluate the effects of postoperative ARF after the on-pump coronary artery bypass surgery (CABG) on the recovery of patients and identify the risk factors. Method and Material: We reviewed the medical records of patients who underwent CABG with cardiopulmonary bypass by a single surgeon from Jan. 2000 to Dec. 2002, We checked the preoperative factors; sex, age, history of previous serum creationism over 2.0 mg/㎗, preoperatively last checked serum creatinine, diabetes, hypertension, left ventricular ejection fraction, intraoperative factors; whether the operation is an emergent case or not, cardiopulmonary bypass time, aortic cross clamp time, the number of distal anastomosis, postoperative factors: IABP. Then we have studied the relations of these factors and the cases of postoperative peak serum creatinine over 2.0 mg/㎗. Result: There were 19 cases with postoperative peak serum creatinine over 2.0 mg/㎗ in a total 97 cases. Dialysis were done in 3 cases for ARF with pulmonary edema and severely reduced urine output. There were 8 cases (42.1%) with combined complications among the 19 patients. This finding showed a significant difference from the 5 cases (6,4%) in the patients whose creatinine level have not increased over 2.0 mg/㎗. The mortalities are different as 1.3% to 10.5%. The risk factors that are related with postoperative serum creatinine increment over 2.0 mg/㎗ are diabetes, the history of previous serum creatinine over 2.0 mg/㎗ and left ventricular ejection fraction. Conclusion: Postoperative ARF after the on-pump CABG is related with preoperative diabetes, the history of previous serum creatinine over 2,0 mg/㎗ and left ventricular ejection fraction. Postoperative ARF could De the reason for increased rate of complications and mortality after on-pump CABG. Therefore, in the patients with these risk factors, the efforts to prevent postoperative ARF like off-pump CABG should be considered.

• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.32-36
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• 2007

Background: Although acute renal failure (ARF) after coronary artery bypass graft (CABG) is relatively rare, but devastating complication with high mortality. Our study aims to evaluate the effectiveness of early application of CRRT in patients with ARF which developed after on-pump CABG. Material and Method: Two hundred and eighty seven patients underwent isolated on-pump CABG between May 2002 and Feb. 2006 at our institution, of whom 15 (5.2%) needed CRRT (11 patients for postoperatively developed ARF and the remaining 4 patients with preexisting dialysis-dependent chronic renal failure (CRF) for postoperative hemodynamic and metabolic control). Criteria for early application of CRRT were as follows; decreased urine output less than 0.5cc/h/kg for 2 consecutive hours and elevated serum creatinine level greater than 2.0 mg/dL. Result: The incidence of ARF requiring CRRT after on-pump CABG was 3.9% (11/283) and the overall hospital mortality of patient with CRRT was 33.3% (5/15). Of 5 deaths, 4 were patients with postoperatively developed ARF, and 1 was a patient with pre-existing dialysis-dependent CRF patient. The mean time between the operation and the initiation of CRRT was $25.8{\pm}5.8$ hours and the mean duration of CRRT was $62.1{\pm}41.2$ hours. Of the 7 survivors who were not on dialysis-dependent preoperatively, 6 patients fully recovered renal function during hospital stay and 1 patient required permanent renal supportive treatment after discharge from hospital. Conclusion: Early application of CRRT could maintain stable postoperative hemodynamic status and make outcomes better than those of previous reports in patients with ARF which developed after on-pump CABG.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.62-67
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• 2018

Hunter syndrome, also known as mucopolysaccharidosis Type II (MPS II), is one of the lysosomal storage diseases caused by a lack of the enzyme iduronate 2-sulfatase (I2S). Lack of the I2S enzyme activity leads to accumulation of the glycosaminoglycans (GAG), causing dysfunction of multiple organs and systems. MPS II is an X-linked recessive disease due to mutation of IDS gene located on long arm of the X chromosome (Xq28). To date, more than 350 mutations of IDS gene have been identified in Hunter syndrome. Phenotypes of MPS II are classified as either severe or attenuated depending on the degree of cognitive impairment. Because the phenotype of MPS II is related to the type of mutation, identifying mutations is useful in predicting prognosis. We recently had a case of MPS II diagnosed by exome sequencing in a 7 month old boy with infantile spasm uncontrolled by AED. He was diagnosed with hearing loss at 2 months of age, and he took vigabatrin and prednisolone to control infantile spasms diagnosed at 3 months of age. At 6 months of age, whole exome sequencing was performed to evaluate the infantile spasm and hearing loss in this patient, and the mutation c.851C>T (p.Pro284Leu) inherited from hemizygous mother was revealed. The results of urine Cetylpyridinium Chloride (CPC) precipitation test, which were negative until 8 months of age, were positive from 9 months of age. We report a case of MPS II diagnosed by exome sequencing and treated through enzyme replacement therapy from 9 months after birth.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.136-146
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• 2001

Purpose : One of the most difficult problems in the care of children with nephrotic syndrome remains the occurrence of relapses, despite initial response to steroids. Constantinescu reported that rapidity of initial response to steroid therapy could predict fewer relapses in the first year. So we evaluated the changes in serum lipid abnormalities in children with corticosensitive nephrotic syndrome before steroid treatment and the correlation between serum lipid levels and renal function, days to remission. Methods . We analyzed the Medical records of children who were managed by us between October 1994 and August 2000. In 33 patients with corticosensitive nephrotic syndrome, we evaluated the correlation between serum lipid levels and renal function [Creatinine clearance(Ccr)] and proteinuria before steroid treatment, and days to remission defined as the third day when the patient's urine becomes protein free. Results : There were 21 males and 12 females. Median age at presentation was 6.4 years (range: 1.8-17.3 years). Median days to remission were 15.4 days (range 4-42 days) on Prednisolone $60mg/m^2$ daily. The increased levels of triglyceride, total cholesterol, LDL cholesterol, apolipoprotein B, total cholesterol/HDL cholesterol, Lipoprotein(a) were observed. But the level of HDL cholesterol was not increased. Serum albumin was decreased a]id proteinuria was increased before steroid treatment. But Ccr was not decreased. There were negative correlation between serum albumin and total cholesterol (r = -0.5157, P<0.005), LDL cholesterol (r = -0.5543, P<0.005), total cholesterol/HDL cholesterol (r = -0.4506, P<0.01), lipoprotein(a) (r = -0.4570, P<0.025), apolipoprotein B (r = -0.5297, P<0.025), apolipoprotein B/apolipoprotein Al (r = -0.5851, P<0.01), apolipoprotein B/HDL cholesterol (r = -0.4961, P<0.05) before steroid treatment. There was no correlation between proteinuria and serum lipid profiles. Also Ccr and serum lipid profiles were not correlated. There was positive correlation between days to remission and HDL cholesterol (r = +0.4511, P<0.05), apolipoprotein B (r = +0.5190, P<0.05), apolipoprotein B/HDL cholesterol (r = +0.7169, P<0.005). Conclusions : This results reveal that HDL cholesterol, apolipoprotein B and apolipoprotein B/HDL cholesterol can be used as a predictive factor in corticosensitive nephrotic syndrome. We could not determine the significant level of these lipids for insufficient patients number, but these level may predict future relapses of corticosensitive nephrotic syndrome patients and thus may allow to better management and treatment protocols. More data and long term follow up studies should be needed. (J Korean Soc Pediatr Nephrol 2001;5 : 136-46)