• Childhood Kidney Diseases
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• v.7 no.1
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• pp.86-90
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• 2003

An 8-month-old male infant presented with persistent, gross, orange-colored crystals in his urine. His physical and neurological development was normal. Laboratory study showed hyperuricemia, hyperuricosuria and urate crystaluria. He was determined to have partial hypoxanthine-guanine phosphoribosyl transferase(HPRT) deficiency. The molecular genetic analysis revealed a missense mutation in the patient's HPRT gene. By sequencing the patient's cDNA, we identified an A-to-G transition at nucleotide 239, resulting in the replacement of Aspartate with Glycine at amino acid 80 in the HPRT. To our knowledge, this mutation has not previously been reported. Our patient is now being placed on allopurinol therapy, and has had no problem since. Partial HPRT deficiency has been known to cause recurrent acute renal failure without the phenotypic features of Lesch-Nyhan syndrome. Therefore, we think that early diagnosis and treatment are very crucial in preventing acute renal failure.

• Journal of Yeungnam Medical Science
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• v.35 no.1
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• pp.84-88
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• 2018

A 33-year-old woman visited the emergency department presenting with fever and dyspnea. She was pregnant with gestational age of 31 weeks and 6 days. She had dysuria for 7 days, and fever and dyspnea for 1 day. The vital signs were as follows: blood pressure 110/70 mmHg, heart rate 118 beats/minute, respiratory rate 28/minute, body temperature $38.7^{\circ}C$, and oxygen saturation by pulse oximetry 84% during inhalation of 5 liters of oxygen by nasal prongs. Crackles were heard over both lung fields. There were no signs of uterine contractions. Chest X-ray and chest computed tomography scan showed multiple consolidations and air bronchograms in both lungs. According to urinalysis, there was pyuria and microscopic hematuria. She was diagnosed with community-acquired pneumonia and urinary tract infection (UTI) that progressed to severe sepsis and acute respiratory failure. We found extended-spectrum beta-lactamase producing Escherichia coli in the blood culture and methicillin-resistant Staphylococcus aureus in the sputum culture. The patient was transferred to the intensive care unit with administration of antibiotics and supplementation of high-flow oxygen. On hospital day 2, hypoxemia was aggravated. She underwent endotracheal intubation and mechanical ventilation. After 3 hours, fetal distress was suspected. Under 100% fraction of inspired oxygen, her oxygen partial pressure was 87 mmHg in the arterial blood. She developed acute kidney injury and thrombocytopenia. We diagnosed her with multi-organ failure due to severe sepsis. After an emergent cesarean section, pneumonia, UTI, and other organ failures gradually recovered. The patient and baby were discharged soon thereafter.

• Journal of Korean Neurosurgical Society
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• v.54 no.4
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• pp.296-301
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• 2013

Objective : We conducted a retrospective study examining the outcomes of intracerebral hemorrhage (ICH) in patients with chronic kidney disease (CKD) to identify parameters associated with prognosis. Methods : From January 2001 to June 2008, we treated 32 ICH patients (21 men, 11 women; mean age, 62 years) with CKD. We surveyed patients age, sex, underlying disease, neurological status using Glasgow Coma Scale (GCS), ICH volume, hematoma location, accompanying intraventricular hemorrhage, anti-platelet agents, initial and 3rd day systolic blood pressure (SBP), clinical outcome using the modified Rankin Scale (mRS) and complications. The severity of renal functions was categorized using a modified glomerular filtration rate (mGFR). Multifactorial effects were identified by regression analysis. Results : The mean GCS score on admission was $9.4{\pm}4.4$ and the mean mRS was $4.3{\pm}1.8$. The overall clinical outcomes showed a significant relationship on initial neurological status, hematoma volume, and mGFR. Also, the outcomes of patients with a severe renal dysfunction were significantly different from those with mild/moderate renal dysfunction (p<0.05). Particularly, initial hematoma volume and sBP on the 3rd day after ICH onset were related with mortality (p<0.05). However, the other factors showed no correlation with clinical outcome. Conclusion : Neurological outcome was based on initial neurological status, renal function and the volume of the hematoma. In addition, hematoma volume and uncontrolled blood pressure were significantly related to mortality. Hence, the severity of renal function, initial neurological status, hematoma volume, and uncontrolled blood pressure emerged as significant prognostic factors in ICH patients with CKD.

• Radiation Oncology Journal
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• v.4 no.1
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• pp.15-20
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• 1986

From 1979 to 1984, 39 local allograft irradiations were given to 29 patients: 10 irradiations were administered for prevention and 29 for reversal of acute rejection of transplanted kidney. Three doses of 150 cGy every other day were combined with high-dose of methylprednisolone pulse (1 gm/day) for 3 days. For prevention of acute rejection, local irradiation was delivered on the days 1, 3, and 5 after the transplantation, and for reversal, irradiation started after the diagnosis of acute rejection. Eight out of 10 patients irradiated for prevention had acute allograft rejection, and, what is more, there was no surviving graft at 15 months after transplantation. Reversal of acute rejection was achieved in $71\%$. When the pre-irradiation level of serum creatinine was below $5.5mg\%$, the reversal rate was $93\%$, but above $5.5mg\%$ the reversal rate was only $17\%$ (p<0.01). Reirradiation after failure was not successful. Among 15 reversed patients, $7(47\%)$ had subsequent rejection (s). The functional graft survivals at 6 month, 1, 2, and 3 year were $70\%,\;65\%,\; 54\%,\;and\;54\%$, respectively. Therapeutic irradiation resulted in better graft survival when serum creatinine was below $5.5mg\%$ (p<0.001) or when irradiation started within 15 days after the diagnosis of acute rejection (p<0.001).

• Journal of Chest Surgery
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• v.38 no.2 s.247
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• pp.146-151
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• 2005

Background: Preoperative elevated serum creatinine values are associated with increased risk for both morbidity and mortality in patients undergoing on-pump coronary artery bypass surgery (CABG). We investigated the postoperative changes of renal function and proper management in the patients. Material and Method: Among 74 consecutive patients who underwent isolated on-pump CABG, 17 patients with increased serum creatinine level $(creatinine\;\geqq\;1.5\;mg/dL)$ within preoperative one week wereincluded in the study. Seven patients showed pre­operative serum creatinine level of 2.0 mg/dL or higher, and 3 of them had been undergoing hemodialysis. Preoperative hemodialysis was performed in the 3 patients due to end-stage renal failure (ESRD) the day before the operation. We started peritoneal dialysis immediately after the cardiopulmonary bypass in patients with ESRD or postoperative acute renal failure if it was necessary to remove intravascular volume and lower serum creatinine level. Result In most of the patients with CABG, postoperative serum creatinine level increased and recovered to the preoperative level at the discharge. In 2 of the 4 patients with serum creatinine level of 2.0 mg/dL or higher and 3 patients with ESRD, intravascular volume, serum creatinine level and serum electrolyte were controlled with peritoneal dialysis. Conclusion: Postoperative serum creatinine level increased transiently in most of CABG patients, and intravascular volume and serum creatinine level were controlled by peritoneal dialysis only in the patients with acute renal failure postoperatively and those depending on hemodialysis.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.65-71
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• 2006

Idiopathic renal hypouricemia is a disorder characterized by impaired urate handling in the renal tubules. Most patients with hypouricemia are asymptomatic and are found incidentally, but the condition is known to be at high risk for exercise-induced acute renal failure or urolithiasis. URAT1 protein encoded by SLC22A12 gene has been identified recently as a urate/anion exchanger in the human kidney. Inactivation mutations in SLC22A12 gene have been shown to cause renal idiopathic hypouricemia. We experienced a 3-year-old boy who presented with persistent orange-colored urine since infancy. His urine contained many uric acid crystals, while the serum showed hypouricemia(0.7 mg/dL). The fractional excretion of uric acid was increased to 41.7%. SLC22a12 gene analysis revealed W258X homozygote alleles. Renal hypouricemia must be included in the differential diagnosis of red-urine and SLC22A12 gene analysis is recommended in idiopathic renal hypouricemia.

• Journal of Yeungnam Medical Science
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• v.34 no.1
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• pp.123-127
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• 2017

Drug-induced immune hemolytic anemia (DIIHA) is a rare side effect of drugs. DIIHA may cause a systemic inflammatory response that results in acute multi-organ failure and death. Ceftizoxime belongs to the class of third generation cephalosporins, which are the most common drugs associated with DIIHA. Herein, we present a case of a 66-year-old man who developed fatal DIIHA after receiving a second dose of ceftizoxime. He was admitted to receive photodynamic therapy. He had a history of a single parenteral dose of ceftizoxime 3 months prior to admission. On the day of the procedure - shortly after the infusion of ceftizoxime - the patient's mental status was altered. The blood test results revealed hemolysis. Oliguric acute kidney injury developed, and continuous renal replacement therapy had to be applied. On the suspicion of DIIHA, the patient underwent plasmapheresis. Diagnosis was confirmed by a detection of drug-dependent antibody with immune complex formation. Although his hemolysis improved, his liver failure did not improve. He was eventually discharged to palliative care, and subsequently died.

• Journal of Cardiovascular Imaging
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• v.26 no.4
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• pp.217-225
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• 2018

BACKGROUND: In patients with acute heart failure (AHF), diastolic dysfunction, especially pseudonormal (PN) or restrictive filling pattern (RFP) of left ventricle (LV), is considered to be implicated in a poor prognosis. However, prognostic significance of diastolic dysfunction in patients with ischemic heart disease (IHD) has been rarely investigated in Korea. METHODS: We enrolled 138 patients with IHD presenting as AHF and sinus rhythm during echocardiographic study. Diastolic dysfunction of LV was graded as ${\geq}2$ (group 1) or 1 (group 2) according to usual algorithm using E/A ratio and deceleration time of mitral inflow, E'/A' ratio of tissue Doppler echocardiography and left atrial size. RESULTS: Patients in group 1 showed higher 2-year mortality rate ($36.2%{\pm}6.7%$) than those in group 2 ($13.6%{\pm}4.5%$; p = 0.008). Two-year mortality rate of patient with LV ejection fraction (LVEF) < 40% ($26.8%{\pm}6.0%$) was not different from those with LVEF 40%-49% ($28.0%{\pm}8.0%$) or ${\geq}50%$ ($13.7%{\pm}7.4%$; p = 0.442). On univariate analysis, PN or RFP of LV, higher stage of chronic kidney disease (CKD) and higher New York Heart Association (NYHA) functional class were poor prognostic factors, but LVEF or older age ${\geq}75$ years did not predict 2-year mortality. On multivariate analysis, PN or RFP of LV (hazard ratio [HR], 2.52; 95% confidence interval [CI], 1.09-5.84; p = 0.031), higher stage of CKD (HR, 1.57; 95% CI, 1.14-2.17; p = 0.006) and higher NYHA functional class (HR, 1.81; 95% CI, 1.11-2.94; p = 0.017) were still significant prognostic factors for 2-year mortality. CONCLUSIONS: PN or RFP of LV was a more useful prognostic factor for long-term mortality than LVEF in patients with IHD presenting as AHF.

• Investigative Magnetic Resonance Imaging
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• v.17 no.3
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• pp.181-191
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• 2013

Purpose : To evaluate the usefulness of in vivo magnetic resonance (MR) imaging for tracking intravenously injected superparamagnetic iron oxide (SPIO)-labeled human umbilical vein endothelial cells (HUVECs) in an acute renal failure (ARF) rat model. Materials and Methods: HUVECs were labeled with SPIO and poly-L-lysine (PLL) complex. Relaxation rates at 1.5-T MR, cell viability, and labeling stability were assessed. HUVECs were injected into the tail vein of ARF rats (labeled cells in 10 rats, unlabeled cells in 2 rats). Follow-up serial $T2^*$-weighted gradient-echo MR imaging was performed at 1, 3, 5 and 7 days after injection, and the MR findings were compared with histologic findings. Results: There was an average of $98.4{\pm}2.4%$ Prussian blue stain-positive cells after labeling with SPIOPLL complex. Relaxation rates ($R2^*$) of all cultured HUVECs at day 3 and 5 were not markedly decreased compared with that at day 1. The stability of SPIO in HUVECs was maintained during the proliferation of HUVECs in culture media. In the presence of left unilateral renal artery ischemia, $T2^*$-weighted MR imaging performed 1 day after the intravenous injection of labeled HUVECs revealed a significant signal intensity (SI) loss exclusively in the left renal outer medulla regions, but not in the right kidney. The MR imaging findings at days 3, 5 and 7 after intravenous injection of HUVECs showed a SI loss in the outer medulla regions of the ischemically injured kidney, but the SI progressively recovered with time and the right kidney did not have a significant change in SI in the same period. Upon histologic analysis, the SI loss on MR images was correspondent to the presence of Prussian blue stained cells, primarily in the renal outer medulla. Conclusion: MR imaging appears to be useful for in vivo monitoring of intravenously injected SPIO-labeled HUVECs in an ischemically injured rat kidney.

• Journal of Korean Neurosurgical Society
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• v.58 no.4
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• pp.389-392
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• 2015

Brown tumors also called as osteoclastomas, are rare nonneoplastic lesions that arise in the setting of primary or secondary hyperparathyroidism. Parathyroid adenomas or hyperplasia constitute the major Brown tumor source in primary hyperparathyroidism while chronic renal failure is the leading cause in secondary hyperparathyroidism. Most of the patients with the diagnosis of primary hyperparathyroidism present with kidney stones or isolated hypercalcemia. However, nearly one third of patients are asymptomatic and hypercalcemia is found incidentally. Skeletal involvement such as generalized osteopenia, bone resorption, bone cysts and Brown tumors are seen on the late phase of hyperparathyroidism. The symptoms include axial pain, radiculopathy, myelopathy and myeloradiculopathy according to their locations. Plasmocytoma, lymphoma, giant cell tumors and metastates should be ruled out in the differential diagnosis of Brown tumors. Treatment of Brown tumors involve both the management of hyperparathyroidism and neural decompression. The authors report a very rare spinal Brown tumor case, arisen as the initial manifestation of primary hyperparathyroidism that leads to acute paraparesis.