• Childhood Kidney Diseases
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• v.23 no.1
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• pp.43-47
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• 2019

Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (antiCFH-aHUS) primarily affects children aged 9-13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFH-aHUS) in Laos.

• Journal of Physiology & Pathology in Korean Medicine
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• v.17 no.2
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• pp.316-325
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• 2003

In this paper, the effect of Ganopoly(extracts of Ganoderma lucidum) and Ganopoly/C+(70% Ganopoly + 30% chitosan) on cisplastin-induced nephrotoxicity was investigated in Sprague-Dawley rats. A single dose of cisplastin(5 ㎎/㎏) kg) was administered intraperitoneally after pretreatment of saline, Ganopoly and Ganopoly/C+ for 7 days. The nephrotoxicity and renal function were manifestated by the changes of body weight, blood pressure, biochemical changes and solute in urine and plasma. After the treatment of CDDP(cis-dichlorodiamineplatinum), a significant elevation of kidney weight, serum urea, cretinine, urine volume for 24 hours, urine magnesium, and a severe or significant decrease in body weight, blood pressure, creatinine clearance, urine osmolarity, serum albumin, etc. The nephrotoxicity was further confirmed by a significant decrease in glutathione S-transferase(GSH) in urine and kidney homogenate, GSH, glutathione peroxidase(GSH-Px) and catalase in kidney tissue. And also the lipid peroxidation was significantly increased in kidney homogenate. These signs of nephrotoxicity was ameliorated by the pretreatment and consecutive administration of Ganopoly and Ganopoly/C+ for 14 days after the Lp. injection of CDDP on 7th day after pretreatment of Ganopoly and Ganopoly/C+. The amelioration of nephrotoxicity was evidenced by significant reduction in serum urea and creatinine concentration, and improvement of other index of renal function. And The activity of antioxidant enzymes were partially recovered in kidney tissue of rats treated by CDDP and the administration of Ganopoly and Ganopoly/C+. These results indicate the cispastin induced nephrotoxicity is due to an impairment of tubular reabsorption systems enhanced by necrosis of proximal tubule, and the Ganopoly and Ganopoly/C+ has a partial protective effect on nephrotoxicity induced by CDDP. The polysacchride of Ganoderma lucidum may improve the therapeutic index of nephrotoxicity induced by CDDP. However, it is needed to elucidate the mechanism for confirming the therapeutic effect.

• Journal of radiological science and technology
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• v.36 no.4
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• pp.299-304
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• 2013

Whole body bone scan has been used to confirm bone metastasis and follow-up study with radio isotope. However, if the factors related to $^{99m}Tc$ uptake and waiting time for study are inappropriate, it would be image of low quality. The purpose of present study was to investigate correlation between the evaluation index of renal function and uptake of radiopharmaceuticals. The population for this retrospective study consisted of 387 patients who underwent whole body bone scan between June 2012 and December 2012. As a result of quantitative and qualitative analysis, we were able to confirm that GFR of less than normal range and creatinine levels in blood of more than average are more likely to be under the mean uptake rate. As a result of analysis on the indicator affecting soft-tissue and bone uptake, the correlation of all elements was somewhat low. Also there are no statistically significances due to the other parameters we did not deal with. Therefore, further research on additional factors is needed for exact study and improvement of the image quality.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.3
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• pp.92-95
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• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Journal of Chest Surgery
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• v.17 no.3
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• pp.409-417
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• 1984

In the early days of open heart surgery, acute respiratory failure following extracorporeal circulation was a significant deterrent to an uncomplicated recovery. Although a marked improvement in prevention and treatment of postoperative respiratory failure has been achieved, the problem has not been completely eliminated and continues to be a causative factor in morbidity and mortality Fates following open heart surgery. We have attempted to evaluate postoperative respiratory failure in patients undergoing cardiac operation with the aid of extracorporeal circulation. Our series comprised 92 patients who underwent elective open heart surgery at the Department of Thoracic and Cariodvascular Surgery, School of Medicine, Kyungpook National University, from January, 1980 to December, 1982. In our study, the overall incidence of acute respiratory failure following open heart surgery was 18.8 percent. The duration of extracorporeal circulation in a series of 18 patients who developed postoperative respiratory failure [Group B] was longer in the mean value [120.3 minutes] than the uncomplicated 74 patients [Group A] [85.8 minutes]. The duration of artificial ventilation after open heart surgery in Group A averaged 13.4 hours as contrasted with 76.5 hours in Group B. In Group B, the inspired oxygen concentration [FiO2] in artificial ventilation was continued in the higher level than Group A until 18 hours after operation. Upon pulmonary function test performed pre-and postoperatively, residual volume[RV], RV/TLC and FEV 1.0/FVC were remained essentially unchanged following extracorporeal circulation, whereas forced vital capacity [FVC], FEV 1.0 and FEF 25-75% were significantly decreased in the early postoperative days. The incidence of acute respiratory failure was significantly higher in a series of patients who developed postoperative complications, such as re- exploration due to massive bleeding, low cardiac output, acute renal failure and arrhythmias. A total of 9 patients died, giving an overall mortality was 33.3 percent whereas the mortality was only 1.1 percent for patients without respiratory failure.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.2
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• pp.39-44
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• 2023

Hereditary tyrosinemia type 1 (HT-1) is a metabolic disorder caused by biallelic pathogenic variants in the fumarylacetoacetate hydrolase (FAH) gene, which impairs the function of the FAH enzyme, resulting in the accumulation of tyrosine's toxic metabolites in hepatocytes and renal tubular cells. As a consequence, individuals with HT-1 exhibit symptomatic manifestations. Rapid diagnosis and treatment of HT-1 can prevent short-term death and long-term complications. A 15-day-old boy presented to the outpatient department with elevated levels of tyrosine on his newborn screening tests conducted at the age of 3 and 10 days, respectively. Further blood tests revealed increased levels of alpha-fetoprotein and amino acids including tyrosine and threonine. Urine organic acid tests indicated a significant elevation in tyrosine metabolites, as well as the presence of succinylacetone (SA), which led to the diagnosis of HT-1. Two pathogenic and likely pathogenic variants of FAH compatible with HT-1 were also detected. He began a tyrosine-restricted diet at one month old and received nitisinone (NTBC) at two months old. With continued treatment, the patient's initially elevated AFP level, detection of SA in the urine, and mild hepatomegaly showed improvement. During four years and seven months of treatment, there were no exceptional complications apart from an increase in tyrosine levels and a delay in speech. We report a case of tyrosinemia type 1 detected through newborn screening, treated with dietary restriction and NTBC, with a good prognosis.

• Journal of Life Science
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• v.31 no.12
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• pp.1128-1141
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• 2021

'Angelica' is one of the most traditionally consumed medicinal herbs around Northeast Asia including Korea for treatments of various diseases or health care purposes like hematopoiesis, blood circulation for women, sedative, analgesic, and a tonic medicine etc. Angelica gigas Nakai, a Korean native species of Angelica, is clearly different from the others in containing a high concentration of active ingredients like pyranocoumarines including decursin, decursinol, and decursinol angelate. These compounds have various kinds of positive effects such as anti-tumor activity including the precaution of neutropenia occurred during anticancer drug administration, improvements of metabolic disorders, menstrual irregularity, impairment of renal function, respiration improvement, cognition-enhancement, anti-inflammatory effect, anti-oxidative effect, enhancing fertility and so forth. Thus it implies incredible potentialities in future development for foods and drugs. However, certain purity-related qualities and/or overdose in food products can cause side effects like toxicities; therefore, their safety profiles should also be considered. This review focuses on the positive and negative effects of three pyranocoumarines in Angelica gigas Nakai and some possibilities and considerations for future food and drug products development.

• Tuberculosis and Respiratory Diseases
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• v.55 no.5
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• pp.506-515
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• 2003

Background : A decreased level of serum arginine vasopressin(AVP) and an increased sensitivity to an exogenous AVP is expected in patients with septic shock who often require a high infusion rate of catecholamines. The goal of the study was to determine whether an exogenous AVP infusion to the patients with septic shock would achieve a significant decrement in infusion rate of catecholamine vasopressors while maintaining hemodynamic stability and adequate urine output. Method : Eight patients with septic shock who require a high infusion rate of norepinephrine had received a trial of 4-hour AVP infusion with simultaneous titration of norepinephrine. Hemodynamic parameters and urine output were monitored during the AVP infusion and the monitoring continued up to 4 hours after the AVP infusion had stopped. Results : Mean arterial pressure showed no significant changes during the study period(p=0.197). Norepinephrine infusion rate significantly decreased with concurrent AVP administration(p=0.001). However, beneficial effects had disappeared after the AVP infusion was stopped. In addition, hourly urine output showed no significant changes throughout the trials(p=0.093). Conclusion : Concurrent AVP infusion achieved the catecholamine vasopressor sparing effect in the septic shock patients, but there was no evidence of the improvement of renal function. Further study may be indicated to determine whether AVP infusion would provide an organ-protective effect to the septic shock patients.