• Journal of Trauma and Injury
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• v.28 no.1
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• pp.34-38
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• 2015

The case of a patient with a transfusion-related acute lung injury (TRALI) to whom extracorporeal membrane oxygenation (ECMO) had been applied is reported. A 55-year-old male injured with liver laceration (grade 3) without chest injury after car accident. He received lots of blood transfusion and underwent damage control abdominal surgery. In the immediate postoperative period, he suffered from severe hypoxia and respiratory acidosis despite of vigorous management such as 100% oxygen with mechanical ventilation, high PEEP and muscle relaxant. Finally, ECMO was applied to the patients as a last resort. Aggressive treatment with ECMO improved the oxygenation and reduced the acidosis. Unfortunately, the patient died of liver failure and infection. TRALI is a part of acute respiratory distress syndrome (ARDS). The use of ECMO for TRALI induced severe hypoxemia might be a useful option for providing time to allow the injured lung to recover.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.10-18
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• 2014

Recent advances in the diagnosis and treatment of inborn errors of metabolism have improved substantially the prognosis of many of these diseases, if diagnosed early enough before irreversible damage occurs. This makes it essential that the practicing pediatrician, especially neonatologists be familliar with the clinical presentations and systematic approaches of these disorders. Characteristic clinical presentations, methods of systematic approach and typing of various disorders is discussed in this review. The signs of neurological dysfunctions of many IEMs manifesting in the neonatal period is very nonspecific, such as poor feeding, poor sucking, apnea or tachypnea, vomiting, hypertonia, hypotonia, seizure, letharginess, consciousness change and coma. Many other non-metabolic severe disorders of neonatal period such as neonatal sepsis and intracerebral hemorrhage share these nonspecific symptoms. Hyperammonemia, metabolic acidosis, ketosis and hyperlatic acidemia are observed in many of these conditions but there are exceptions in which conditions all basal laboratory tests are normal, such as NKH, sulfite oxidase deficiency and peroxisomal disorders. According to the results of basal laboratory tests, IEMs in the neonatal period can be categorized in to 6 types. Grouping of IEMs into 6 types will make confirmatory tests and early emergency treatment more efficient.

• Journal of The Korean Society of Clinical Toxicology
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• v.11 no.1
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• pp.36-40
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• 2013

Ethylene glycol poisoning is treated mainly by alcohol dehydrogenase inhibition therapy and hemodialysis. Early recognition and initiation of treatment is important because toxic metabolites increase over time by hepatic metabolism; however, there is no confirmative diagnostic tool in our clinical setting. Therefore, diagnosis is dependent on history, high anion gap acidosis, high osmolal gap, etc.. Diagnosis and treatment are delayed in cases where history taking is not possible, such as a mental changed patient. Authors report on two cases of ethylene glycol poisoning by contrasting clinical outcomes, demonstrating the importance of early diagnosis and treatment for achievement of a good outcome.

• Neonatal Medicine
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• v.28 no.2
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• pp.77-82
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• 2021

We present the case of a healthy 28-day-old female full-term neonate who was admitted to the neonatal intensive care unit for severe metabolic acidosis, hypoglycemia, and an initial sinus rhythm. The first diagnostic hypothesis was hypovolemic shock, and fluid resuscitation was started immediately. During fluid therapy, cardiovascular collapse occurred with supraventricular tachycardia. The latter was successfully treated with adenosine and beta-blockers. After 8 days, electrocardiography showed ventricular pre-excitation, and Wolff-Parkinson-White syndrome was diagnosed. A novel variant of the MYL2 gene that is related to hypertrophic cardiomyopathy and conduction defect was found after discharge. Cardiogenic shock should be considered, despite being a rare cause of shock in neonates.

• Electrolytes & blood pressure
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• v.16 no.2
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• pp.19-22
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• 2018

Renal Fanconi syndrome (RFS) is caused by generalized proximal tubular dysfunction and can be divided into hereditary and acquired form. Adult-onset RFS is usually associated with drug toxicity or systemic disorders, and modern molecular genetics may explain the etiology of previous idiopathic cases of RFS. Here, we report the case of a 52-year-old woman with RFS whose etiology could not be identified. She presented with features of phosphaturia, renal glucosuria, aminoaciduria, tubular proteinuria, and proximal renal tubular acidosis. Her family history was unremarkable, and previous medications were nonspecific. Her bone mineral density was compatible with osteoporosis, serum intact parathyroid hormone level was mildly elevated, and 25(OH) vitamin D level was insufficient. Her blood urea nitrogen and serum creatinine levels were 8.4 and 1.19 mg/dL, respectively (estimated glomerular filtration rate, $53mL/min/1.73m^2$). Percutaneous renal biopsy was performed but revealed no specific renal pathology, including mitochondrial morphology. No mutation was detected in EHHADH gene. We propose the possibility of involvement of other genes or molecules in this case of adult RFS.

• Journal of Genetic Medicine
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• v.16 no.1
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• pp.31-38
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• 2019

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a maternally inherited mitochondrial disorder of which m.3243A>G is the most commonly associated mutation, resulting in an inability to meet the energy requirements of various organs. MELAS poses a diagnostic challenge owing to its multiple organ involvement and great clinical variability due to its heteroplasmic nature. We report three cases from a family who were initially misdiagnosed with myasthenia gravis or undiagnosed. Although there is no optimal consensus treatment approach for patients with MELAS because of the disease's heterogeneity, our 21-year-long therapy regimen of ${\text\tiny{L}}-arginine$, ${\text\tiny{L}}-carnitine$, and coenzyme Q10 supplementation combined with dietary management appeared to provide noticeable protection from the symptoms and complications. Prompt early diagnosis is important, as optimal multidisciplinary management and early intervention may improve outcomes.

• Journal of The Korean Society of Clinical Toxicology
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• v.16 no.2
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• pp.165-171
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• 2018

An overdose of antihypertensive agents, such calcium channel blockers (CCBs) and angiotensin II receptor blocker (ARBs), and the antihyperglycemic agent, metformin, leads to hypotension and lactic acidosis, respectively. A 40-year-old hypertensive and diabetic man with hyperlipidemia and a weight of 110 kg presented to the emergency room with vomiting, dizziness, and hypotension following an attempted drug overdose suicide with combined CCBs, ARBs, 3-hydroxy-3-methylglutaryl-coemzyme A reductase inhibitors, and metformins. A conventional medical treatment initially administered proved ineffective. The treatment was then changed to simultaneous extracorporeal membrane oxygenation (ECMO) and continuous renal replacement therapy (CRRT), which was effective. This shows that simultaneous ECMO and CRRT can be an effective treatment protocol in cases of ineffective conventional medical therapy for hypotension and lactic acidosis due to an overdose of antihypertensive agents and metformin, respectively.

• Korean Journal of Veterinary Service
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• v.46 no.1
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• pp.81-85
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• 2023

This case report is about hemolymph nodes found in a dairy cow whose function is still not fully elucidated. A 4-month Holstein cow presented severe respiratory symptoms and hematochezia for a while with respiratory acidosis and metabolic alkalosis. Coccidiosis was diagnosed and treated immediately, but the cow died from respiratory acidosis and metabolic alkalosis. At necropsy, no abnormal appearance in thoracic and peritoneal organs was observed, but hemolymph nodes were observed being multifocally stuck on omasum serosa and the subcutaneous fascia of abdominal region, and the larger dark red lymph nodes were found along the omasum great curvature. Microscopically, lymphoid depletion and lymphadenitis in the lymph nodes were examined to point systemic infection, and in the hemolymph node, multifocally demarcated pale lesions with macrophage infiltration and fibrin deposition nearby subcapsular sinus. In subcapsular sinus of the hemolymph node, rod to linear gram-negative bacteria were found. Through this study, we might conclude that the hemolymph node is involved in pathogen phagocytosis.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.2
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• pp.102-108
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• 2016

Mitochondrial encephalopathy, lactic acidosis, and stroke-like episode (MELAS) syndrome is one of mitochondrial encephalopathy. As the early clinical manifestations can be variable, it is important to suspect the disease, especially in patients with multiple organ dysfunctions. A boy was diagnosed with epilepsy when he was 9 years old. Two years later, severe headache and blurred vision developed suddenly. On examination, left homonymous hemianopsia was detected with corresponding cerebral parenchymal lesions in right temporo-occipito-parietal areas. MELAS syndrome was confirmed by genetic test, which showed m.3243 A>G mitochondrial DNA mutation. Multivitamins including coenzyme Q10 were added to anticonvulsant. He experienced 4 more events of stroke-like episodes over 5 years, but he is able to perform normal daily activities. A 13-year-old boy was brought to the hospital due to suddenly developed respiratory arrest and asystole associated with pneumonia. Past medical history revealed that he had multiple medical problems such as epilepsy, failure-to-thrive, optic atrophy, and deafness. He has been on valproic acid as an anticonvulsant which was prescribed from local clinic. He recovered after the resuscitation, but his cognition and motor function were severely damaged. He became bed-ridden. He was diagnosed with MELAS syndrome by brain MRI, muscle biopsy, and clinical features. Genetic test did not reveal any mitochondrial gene mutation. Four years later, he expired due to suddenly developed severe metabolic acidosis combined with hyperglycemic hyperosmolar nonketotic coma. The clinical features of MELAS syndrome are variable. Early diagnosis before the presentation to the grave clinical course may be important for the better clinical outcome.

• Journal of Korean Academy of Nursing
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• v.26 no.2
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• pp.304-319
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• 1996

It is important to evaluate nutritional status of elderly patients receiving dialysis, since wasting and malnutrition are their common problems. This study aims at assessing their nutritional status by the type and duration of dialysis. The nutritional status such as somatic fat and protein storage was evaluated with anthropometric measure including weight/height ratio, triceps skinfold thickness and midarm muscle circumference. It was also measured with albumin, transferrin, C3 and IGF-1 and calorie and protein intakes. The general clinical condition of patients was evaluated with the severity of uremia and metabolic acidosis. which were measured through the levels of serum urea, creatinine and bicarbonateion. The data were analyzed by using t-test, ANOVA, Wilcoxon-rank sums test, Scheffe test, Kruskal-Wallis test and Pearson correlation coefficients. The results are following : 1. There was no significant difference in the calorie and protein intakes by the type and duration of dialysis received. 2. As for the anthropometric measures, no significant difference was found by the type of dialysis in body mass index triceps skinfold thickness and midarm muscle circumference. Yet these anthropometric measures differed significantly by the duration of dialysis in those elderly patients receiving hemodialysis(HD group), but this finding was not found in those receiving continuous ambulatory peritoneal dialysis(CAPD). 3. Regarding the indicators of uremia and metabolic acidosis, blood urea nitrogen(BUN) and creatinine were lower in the CAPD group than in the HD group, whereas bicarbonate ion was higher in the CAPD group than in the HD group, with no statistical significance. In the HD group, creatinine increased significantly with the increase of the duration of dialysis. 4. Serum trasferrin and C3 were significantly higher in the CAPD group than in the HD group. However. each of biochemical indices did not show statistical significance by the duration of dialysis in both HD and CAPD groups. 5. Anthropometric measures were significantly associated with dietary intake. Significant correlations were observed between biocarbonate ion, BUN and creatinine. In addition, the correlations between serum protein and albumin and between transferrin and C3 were statistically significant. Yet, IGF-1 revealed no significant correlation with other nutritional indices. The above findings indicate that there were no difference in nutritional status measured with protein and calorie intakes between the type and duration of dialysis, but CAPD seems to benefit correcting uremia and metabolic acidosis than HD. Studies of dietary management for dialysis patients need to be pursued in order to improved the quality of aged patients receiving dialysis.