• Childhood Kidney Diseases
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• v.19 no.1
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• pp.48-52
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• 2015

Streptococcus pneumoniae associated hemolytic uremic syndrome (SpHUS) is one of the causes of atypical hemolytic uremic syndrome, and increasingly reported. They are more severe and leave more long-term sequelae than more prevalent, typical hemolytic uremic syndrome. But it is not so easy to diagnose SpHUS for several reasons (below), and there was no diagnostic criteria of consensus. A 18 month-old-girl with sudden onset of oliguria and generalized edema was admitted through the emergency room. She had pneumonia with pleural effusion and laboratory findings of HUS, DIC, and positive direct Coombs' test. As DIC or SpHUS was suspected, we started to treat her with broad spectrum antibiotics, transfusion of washed RBC and replacement of antithrombin III. On the $3^{rd}$ day, due to severe hyperkalemia and metabolic acidosis, continuous renal replacement therapy (CRRT) was started. She showed gradual improvement in 4 days on CRRT and discharged in 16 days of hospital care. At the follow up to one year, she has maintained normal renal function without proteinuria and hypertension. We report this case with review of articles including recently suggested diagnostic criteria of SpHUS.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.256-260
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• 2009

The Russell-Silver syndrome (RSS) is a disease characterized by intrauterine growth retardation with preserved head circumference, facial dysmorphism and short stature. Reported renal and urinary manifestations of RSS include horseshoe kidney, renal tubular acidosis, hydronephrosis, ureteropelvic obstruction and vesicoureteral reflux. Here we report a case of end-stage renal disease associated with RSS, which, to the best of our knowledge, has not been reported yet.

• Journal of Pharmaceutical Investigation
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• v.41 no.6
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• pp.347-354
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• 2011

Repeated oral administration of loxoprofen can induce many side effects such as gastric disturbances and acidosis. Therefore, we considered alternative routes of administration for loxoprofen to avoid such adverse effects. The aim of this study was to develop an ethylene-vinyl acetate (EVA) matrix system containing a permeation enhancer for enhanced transdermal delivery of loxoprofen. The EVA matrix containing loxoprofen was fabricated and the effects of drug concentration, temperature, enhancer and plasticizer on drug release were studied from the loxoprofen-EVA matrix. The solubility of loxoprofen was highest at 40% (v/v) PEG 400. The release rate of drug from drug-EVA matrix increased with increased loading dose and temperature. The release rate was proportional to the square root of loading dose. The activation energy (Ea), which was measured from the slope of log P versus 1000/T, was 5.67 kcal/mol for a 2.0% loaded drug dose from the EVA matrix. Among the plasticizer used, diethyl phthalate showed the highest release rate of loxoprofen. Among the enhancers used, polyoxyethylene 2-oleyl ether showed the greatest enhancing effect. In conclusion, for the enhanced controlled transdermal delivery of loxoprofen, the application of the EVA matrix containing plasticizer and penetration enhancer could be useful in the development of a controlled drug delivery system.

• Journal of Biomedical Engineering Research
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• v.30 no.2
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• pp.147-152
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• 2009

This study was conducted to observe the change of limbs stroke and respiration gas parameters in our new bicycle fitness system. We hypothesized that the variable force of left and right limbs might be effective for sensing stimulation in modified new unequal pedal bicycle system. It has been developed, which can provide visual information and different length of pedal with left and right limbs. Experimental results showed different activities between the left and right limbs where the activity of the left limb increased than that of right limb. Especially, the soleous muscle activity increased both in control and experimental groups by this training method. But oxygen and carbon dioxide partial pressures in respiratory gas increased during training method. These results suggest that acidosis of blood was led by this process. Consequently, this bicycle training is concluded that aerobic training could affect different limb activities. Finally, we expect that our new bicycle system will be effective for healthcare with proper balance between the left and right limbs.

• Journal of Yeungnam Medical Science
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• v.3 no.1
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• pp.375-382
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• 1986

We studied the effects of the acute intermittent peritoneal dialysis in severe acute renal failure of 1 newborn infant and 2 young infants during 18 months period from February 1985 to April 1986. The predisposing illnesses were severe acute gastroenteritis with dehydration. Reye's syndrome, and bilateral nephrolithiasis with hyperuricemia. The concomittent illnesses were severe hypernatremia, hyponatremia, hyperkalemia, hypocalcemia, hypoglycemia, DIC(disseminated intravascular coagulopathy), paralytic ileus, metabolic acidosis and gastrointestinal bleeding. As a dialvsate, Imperinol $solution^R$, 1.5% was used in all cases. The cycles of dialysis were 8, 16, and 41 times in each cases. Observed complications during dialysis were leakage, and abdominal wall and scrotol swelling in 2 cases, hyperglycemia in 1 case, and peritonitis in 1 case. Acinetobacter calcoaceticus was cultured in peritoneal fluid of peritonitis. These complications were treated by stopping dialysis in leakage and abdiminal wall swelling, insulin therapy in hyperglycemia, and intraperitoneal and systemic antibiotics therapy in peritonitis. We experienced improvements of severe acute renal failure with variable concomittant illnesses by acute intermittent peritoneal dialysis despite of the treatable complications of dialysis in all cases.

• Journal of Chest Surgery
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• v.28 no.4
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• pp.381-386
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• 1995

In our hospital we have seen 20 cases of congenital diaphragmatic anomalies from June 1984 until December 1993. These were classified into 10 cases of diaphragmatic eventration, 8 cases of Bochdalek hernia, 1 case of Morgagni hernia, and 1 case of esophageal hiatal hernia. Diaphragmatic eventration cases were composed of 8 males and 2 females with ages varing from 3 hour to 42 year. They were discovered by symptoms: 5 cases of respiratory insufficiency; 3 cases of frequent respiratory infection; and 2 cases by chance; 6 cases involved the left side, 4 cases involved right side. Emergency operations were done to 4 patients. Among the 10 patients, only one operative mortality occurred; 3 hour old female.Bochdalek hernia cases composed 6 females and 2 males, 5 patients were less than 6 hour old. All patients were operated on an emergency status and three of them expired due to the vicious cycle of pulmonary hypertension and pulmonary vasoconstriction, persistent fetal circulation, hypoxia, and metabolic acidosis. Morgagni hernia was seen in one 69 year old female patient, she had no complaint of symptoms and was incidentally detected. Hernia was repaired through right thoracotomy. She was discharged with healthy appearence. Esophageal hiatal hernia was seen in a 10 month old male patient, his symptoms were persistent vomiting and coughing since birth. Sliding type of esophageal hiatal hernia repair was completed through left thoracotomy.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.183-191
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• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Biomolecules & Therapeutics
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• v.26 no.3
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• pp.225-241
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• 2018

Taurine is an abundant, ${\beta}-amino$ acid with diverse cytoprotective activity. In some species, taurine is an essential nutrient but in man it is considered a semi-essential nutrient, although cells lacking taurine show major pathology. These findings have spurred interest in the potential use of taurine as a therapeutic agent. The discovery that taurine is an effective therapy against congestive heart failure led to the study of taurine as a therapeutic agent against other disease conditions. Today, taurine has been approved for the treatment of congestive heart failure in Japan and shows promise in the treatment of several other diseases. The present review summarizes studies supporting a role of taurine in the treatment of diseases of muscle, the central nervous system, and the cardiovascular system. In addition, taurine is extremely effective in the treatment of the mitochondrial disease, mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), and offers a new approach for the treatment of metabolic diseases, such as diabetes, and inflammatory diseases, such as arthritis. The review also addresses the functions of taurine (regulation of antioxidation, energy metabolism, gene expression, ER stress, neuromodulation, quality control and calcium homeostasis) underlying these therapeutic actions.

• Journal of Genetic Medicine
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• v.10 no.2
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• pp.81-87
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• 2013

Pseudohypoaldosteronism (PHA), a rare syndrome of systemic or renal mineralocorticoid resistance, is clinically characterized by hyperkalemia, metabolic acidosis, and elevated plasma aldosterone levels with either renal salt wasting or hypertension. PHA is a heterogeneous disorder both clinically and genetically and can be divided into three subgroups; PHA type 1 (PHA1), type 2 (PHA2) and type 3 (PHA3). PHA1 and PHA2 are genetic disorders, and PHA3 is a secondary disease of transient mineralocorticoid resistance mostly associated with urinary tract infections and obstructive uropathies. PHA1 includes two different forms with different severity of the disease and phenotype: a systemic type of disease with autosomal recessive inheritance (caused by mutations of the amiloride-sensitive epithelial sodium channel, ENaC) and a renal form with autosomal dominant inheritance (caused by mutations of the mineralocorticoid receptor, MR). In the kidneys, the distal nephron takes charge of the fine regulation of water absorption and ion handling under the control of aldosterone. Two major intracellular actors necessary for the action of aldosterone are the MR and the ENaC. Impairment of the intracellular aldosterone signal transduction pathway results in resistance to the action of mineralocorticoids, which leads to PHA. Herein, ion handling the distal nephron and the clinico-genetic findings of PHA are reviewed with special emphasis on PHA type 1.

• Childhood Kidney Diseases
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• v.24 no.1
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• pp.58-61
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• 2020

Pseudohypoaldosteronism type 1 (PHA1) is a rare salt-wasting disorder caused by resistance to mineralocorticoid action. PHA1 is of two types with different levels of disease severity and phenotype as follows: systemic type with an autosomal recessive inheritance (caused by mutations of the epithelial sodium channel) and renal type with an autosomal dominant inheritance (caused by mutations in the mineralocorticoid receptor). The clinical manifestations of PHA1 vary widely; however, PHA1 commonly involves hyponatremia, hyperkalemia, metabolic acidosis and elevated levels of renin and aldosterone. The earliest signs of both type of PAH1 also comprise insufficiency weight gain due to chronic dehydration and failure to thrive during infancy. Here, we report a case of renal PAH1 in a 28-day-old male infant harboring a novel heterozygous mutation in NR3C2 gene (c.1341_1345dupAAACC in exon 2), showing only failure to thrive without the characteristic of dehydration.