• Childhood Kidney Diseases
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• v.16 no.1
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• pp.51-53
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• 2012

Henoch-Sch$\ddot{o}$nlein purpura (HSP) is the most common vasculitis in children and is characterized by cutaneous purpura, arthritis, abdominal pain, and nephritis. Bullous skin lesions are rare in children. We report a case involving a 9-year-old female with HSP who displayed rapidly evolving hemorrhagic bullae from the primary purpuric lesions during systemic corticosteroid therapy. The bullae disappeared within 7 days of systemic corticosteroid therapy. Some scar lesions of the skin developed on acute phase recovered completely after 6 months. Bullae should not be considered as a poor prognostic factor of HSP and its renal outcome. Skin biopsy in HSP children with bullae is not necessary if clinical diagnostic criteria of HSP are met. However, further evaluation of more pediatric HSP with bullae is needed to get the clearer conclusions. We report a 9-year-old female with HSP who showed the rapidly evolving hemorrhagic bullae from primary purpuric lesions during systemic corticosteroid therapy.

• Korean Journal of Head & Neck Oncology
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• v.12 no.1
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• pp.58-64
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• 1996

The parathyroid adenoma is the most common cause of the primary hyperparathyroidism. The characteristic of primary hyperparathyroidism is hypercalcemia and high value of serum parathyroid hormone. The primary hyperparathyroidism with parathyroid adenoma is treated by excision of parathyroid gland involved. Especially, parathyroid storm in patients with primary hyperparathyroidism is more prevalent than commonly appreciated. The symptoms and signs of the syndrome are not only due to the hypercalcemia, but also to the toxic effects of the parathyroid hormone. Its wide, but nonspecific clinical presentations make it easily confused with other cardiovascular or renal diseases. The mortality rate in untreated cases of parathyroid storm is essentially 40%. A 33 year old woman with primary hyperparathyroidism was found to have a left lower parathyroid adenoma, presented with hypercalcemic crisis. Initially, good responsiveness to a saline infusion, furosemide administration was noted. Unfortunately, she became consciousness disturbance after fine-needle aspiration of the parathyroid tumor. The recurrent storm was refractory to medical therapy, but was treated succesfully by emergent surgical removal tumor revealed a parathyroid adenoma with parathyroid hormone. Hypercalcemia was alleviated postoperatively. These observations corroborated a functioning parathyroid adenoma.

• Clinical and Experimental Pediatrics
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• v.59 no.3
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• pp.139-144
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• 2016

Purpose: Acute pyelonephritis (APN) is a serious bacterial infection that can cause renal scarring in children. Early identification of APN is critical to improve treatment outcomes. The neutrophil-lymphocyte ratio (NLR) is a prognostic marker of many diseases, but it has not yet been established in urinary tract infection (UTI). The aim of this study was to determine whether NLR is a useful marker to predict APN or vesicoureteral reflux (VUR). Methods: We retrospectively evaluated 298 pediatric patients ($age{\leq}36months$) with febrile UTI from January 2010 to December 2014. Conventional infection markers (white blood cell [WBC] count, erythrocyte sedimentation rate [ESR], C-reactive protein [CRP]), and NLR were measured. Results: WBC, CRP, ESR, and NLR were higher in APN than in lower UTI (P<0.001). Multiple logistic regression analyses showed that NLR was a predictive factor for positive dimercaptosuccinic acid (DMSA) defects (P<0.001). The area under the receiver operating characteristic (ROC) curve was high for NLR (P<0.001) as well as CRP (P<0.001) for prediction of DMSA defects. NLR showed the highest area under the ROC curve for diagnosis of VUR (P<0.001). Conclusion: NLR can be used as a diagnostic marker of APN with DMSA defect, showing better results than those of conventional markers for VUR prediction.

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.114-119
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• 2002

Takayasu's arteritis(TA) is a chronic idiopathic vasculitis mainly involving the aorta and its main branches, such as brachiocephalic, carotid, subclavian, vertebral and renal arteries, as well as coronary and pulmonary arteries The clinical features usually reflect limb or organ ischemia resulting from gradual stenosis of involved arteries. We experienced a case of idiopathic Takayasu's arteritis with negative tuberculin test involving multiple main branch arteries at active stage without pulse. We treated this patient with combined therapy of steroid and azathioprine, with remission of disease activity. (J Korean Soc Pediatr Nephrol 2002 ;6 : 114-9)

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.120-122
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• 2002

A 9-year-old boy of B blood group with end-stage renal disease due to IgA nephropathy received group O kidney transplantation from his father On day 9, he developed intravascular hemolysis, and anti-B autoantibody formation was confirmed. We diagnosed as immune hemolytic anemia due to passenger lymphocyte from donor, and cyclosporine withdrawl was done. Anemia resolved spontaneously, but on day 18, graft dysfunction developed, and graft biopsy revealed acute allograft rejection. Although hemolysis due to autoantibody is very rare and often mild, and the role of hemoglobinuria on acute rejection in this case is not certain, we recommend consideration of aggressive management on severe hemolysis after minor mismatched kidney transplantation. (J Korean Soc Pediatr Nephrol 2002 ; 6 : 120-2)

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.97-101
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• 2002

Fraley's syndrome is a disorder of vascular impression on the renal infundibulum which is associated with unexplained disabling flank pain, painless microscopic hematuria, and urinary tract infection. We experienced one case of Fraley's syndrome in a 12-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures. (J Korean Soc Pediatr Nephrol 2002 ;6 : 97-101)

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.94-100
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• 2017

Purpose: The American Academy of Pediatrics provides guidelines for managing febrile urinary tract infection (UTI) in infants and children 2-24 months old, but little guidance is offered regarding UTIs in those younger than 8 weeks of age. The definition of UTI is unclear and whether to proceed with micturating cystourethrography (MCUG) or $^{99m}$technetium-dimercaptosuccinic acid (DMSA) scintigraphy scan in this age group is controversial. Methods: We retrospectively analyzed 29 neonates and infants younger than 2 months of age who underwent late DMSA scans 9 months following the first episode of febrile or symptomatic UTI between July 2009 and June 2016. Results: In total, 192 children aged 0-24 months underwent ultrasound and DMSA scans (MCUG in 174/192). Neonates and infants younger than 2 months of age were significantly less likely to develop fever, and had a lower fever peak, shorter duration of fever before admission and after starting antibiotics, longer hospitalization period, lower C-reactive protein, and greater incidence of nonEscherichia coli infection. There was no difference in pyuria response at diagnosis. The prevalence rates of an ultrasound abnormality (28%), vesicoureteral reflux (28%), UTI recurrence (38%), and renal scarring (10%) in infants younger than 8 weeks of age were similar to those in children 2-24 months old. Conclusion: Neonates and infants younger than 2 months of age with UTI warrant special consideration because the fever response used for diagnosis in older children may be absent or blunted. Clinical guideline is needed for the diagnosis and management of UTI in this age group.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.336-339
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• 1997

The surgical treatment of diseases of thoracic aorta has had much better success rate in recent years compaired to the past. Aortic aneurysms or dissections that extend along the entire thoracic aorta are usually approached in two or three stages. Recently we successfully perfofm d one-stage aortic replacement from the aortic root to the abdominal aorta in chronic DeBakey type I dissection. A 25 year-old man who had dyspnea on exertion (NYHA Fc II) and a Marfanoid feature was operated under the diagnosis of chronic type I dissection with severe aortic regurgitation. ' At operation, a huge ascending aorta with two intimal tearings was seen and the blood supply of intercostal arteries and right renal artery was done from the false lumen. Modified Bentall operation with total aortic replacement was done successfully, and the patient is being followed-up without major complications.

• The Korean Journal of Physiology and Pharmacology
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• v.8 no.3
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• pp.117-127
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• 2004

The heterodimeric amino acid transporter family is a subfamily of SLC7 solute transporter family which includes 14-transmembrane cationic amino acid transporters and 12-transmembrane heterodimeric amino acid transporters. The members of heterodimeric amino acid transporter family are linked via a disulfide bond to single membrane spanning glycoproteins such as 4F2hc (4F2 heavy chain) and rBAT $(related\;to\;b^0,\;^+-amino\;acid\;transporter)$. Six members are associated with 4F2hc and one is linked to rBAT. Two additional members were identified as ones associated with unknown heavy chains. The members of heterodimeric amino acid transporter family exhibit diverse substrate selectivity and are expressed in variety of tissues. They play variety of physiological roles including epithelial transport of amino acids as well as the roles to provide cells in general with amino acids for cellular nutrition. The dysfunction or hyperfunction of the members of the heterodimeric amino acid transporter family are involved in some diseases and pathologic conditions. The genetic defects of the renal and intestinal transporters $b^{0,+}AT/BAT1\;(b^{0,+}-type\;amino\;acid\;transporter/b^{0,+}-type\;amino\;acid\;transporter\;1)$ and $y^+LAT1\;(y^+L-type\;amino\;acid\;transporter\;1)$ result in the amino aciduria with sever clinical symptoms such as cystinuria and lysin uric protein intolerance, respectively. LAT1 is proposed to be involved in the progression of malignant tumor. xCT (x-C-type transporter) functions to protect cells against oxidative stress, while its over-function may be damaging neurons leading to the exacerbation of brain damage after brain ischemia. Because of broad substrate selectivity, system L transporters such as LAT1 transport amino acid-related compounds including L-Dopa and function as a drug transporter. System L also interacts with some environmental toxins with amino acid-related structure such as cysteine-conjugated methylmercury. Therefore, these transporter would be candidates for drug targets based on new therapeutic strategies.

• Journal of Nutrition and Health
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• v.34 no.2
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• pp.150-157
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• 2001

Intestinal absorption of dietary taurine is one of the regulatory component maintaining taurine homeostasis along with renal reabsorption, bile acid conjugation and secretion, and de nobo synthesis of taurine in mammals. Recent observations of decreased enterocytic levels of taurine in response to trauma, infection and surgical insults, postulate the possibility that intestinal taurine absorption might be impaired in such stressed conditions. The aim of the present study was to evaluate changes in enterocytic taurine transporter activity using the human intestinal colon carcinoma cell line, HT-29, in various stress-induced conditions. Pretreatment of the HT-29 cells with dexamethasone, a stress hormone(0.1,1,10 or 100$\mu$M) for 3 hrs, or with E coli heat-stable enterotoxin(10, 100, or 200nM) for 30 minutes in order to induce the condition of enterotoxigenic infection did not influence taurine uptake as compared to the value found in control cells. In contrast, pretreatment of the cells with cholera toxin(10, 100, 500, or 1000ng/ml)for 3hr or 24hr significantly decreased taurine uptake by HT-29 cells to 40~50% of the value found in untreated control cells. Kinetic studies of the taurine transporter activity were conducted in control and cholera toxin treated HT-29 cells with varying taurine concentrations(2~60$\mu$M) in the uptake medium. Pretreatment of the cells with cholera toxin(100ng/ml) for 3hr did not influence the Vmax, but resulted in a 55% increase in the Michaelis-Menten constant(Km) of the taurine transporter compared to those in control cells. These results suggest that cholera toxin-induced reduction in taurine transporter activity in HT-29 cells is associated with decreased affinity of the taurine transporter without altering the amount of transporter protein. Intestinal taurine absorption appears to be reduced in the condition of water-borne diseases caused by bacteria such as V. cholerae. This might influence the taurine status of infants and young children more readily, an age group in which the prevalence of intestinal infection is high and the role of intestinal absorption is crucial for maintaining the body taurine pool. (Korean J Nutrition 34(2) : 150-157, 2001)