• Clinical and Experimental Pediatrics
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• v.52 no.8
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• pp.938-943
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• 2009

Purpose : We performed this study in order to investigate the effect of direct renin inhibition on an experimental animal model with nephrotoxic serum nephritis and tried to give useful information for clinical research and renin inhibitor treatment. Methods : Thirty BALB/c 6-week-old male mice were divided into 4 groups: control group (CO, n=5), control-treatment group with aliskiren (CT, n=5), disease group (DO, n=10), and disease treatment group with aliskiren (DT, n=10). Nephritis was induced by an intravenous injection of 0.25 mg/g weight of rabbit anti-GBM immunoglobulin G. Model 2002 Alzet mini-osmotic pumps (Durect Corp.) for aliskiren infusion were implanted into CT and DT. Each group strain was sacrificed serially one at a time on day 14. We estimated the protein-creatinine ratio in 12-hour-collected urine (UP/Cr) and measured the mesangial matrix score in the PAS-stained kidney of each strain. Results : One strain at CT and DT died on day 6 and 7, respectively. Each group strain was sacrificed serially at a time on day 10 because DO were seriously ill. The UP/Cr of each group is as follows: CO, $31.24{\pm}6.54mg/mg$, CT, $23.38{\pm}13.60mg/mg$, DO, $112.72{\pm}10.97mg/mg$, DT $114.07{\pm}32.30mg/mg$. There was no significant difference between DO and DT. The mesangial matrix score of each group was CO, $0.23{\pm}0.10$; CT, $0.13{\pm}0.03$; DO, $1.90{\pm}0.48$; and DT, $1.28{\pm}0.41$, respectively, and there was a significant difference between DO and DT in the extent of mesangial matrix expansion (P=0.008). Conclusion : We found that renin inhibition was able to suppress the mesangial matrix expansion in experimental mice with acute nephritis, although there were no significant differences in UP/Cr.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1230-1234
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• 2003

Purpose : The aim of this study is to evaluate the value of lung biopsies for the management of children with lung disease. Methods : We retrospectively reviewed 19 lung biopsies done at Asan Medical Center, Seoul between 1993 and 2001. Data gathered included demographic information, underlying conditions, diagnosis before biopsy, final diagnosis, change in therapy, morbidity and mortality. Results : Nineteen patients underwent lung biopsy. Among them, 13 patients were male and six patients were female; the median age was 3.6 years(0.8 to 8.6 years). Twelve patients underwent open lung biopsies and seven patients had thoracoscopic biopsies. The overall diagnosis rate was 95 %. The most common diagnosis was interstitial lung disease(12 patients, 64%) and infection was detected in four patients(21%). The biopsy-proven bronchiolitis obliterance was confirmed in two of seven patients suspected by CT findings. Specific treatment was changed after biopsy in 16 patients (85%). The morbidity & overall mortality rates of the patients were 5%(one patient) and 21%(four patients) respectively. Only one complication was seen : empyema. The causes of death were acute respiratory distress syndrome(one patient), respiratory failure(two patients), and septicemia(one patient). Conclusion : The lung biopsy is a safe procedure and it contributes to more accurate diagnosis and proper management of pediatric lung diseases. We recommend lung biopsies should be considered more positively in the diagnosis of pediatric lung diseases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.13 no.1
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• pp.58-65
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• 2010

Purpose: Since there are few studies involving acute pancreatitis in children, we reviewed our experience with this medical condition to describe the clinical features. Methods: A retrospective analysis was conducted by reviewing the medical records of 41 patients with AP who were admitted to the Department of Pediatrics of Pusan National University Hospital between January 1996 and June 2007. Results: Twenty males and 21 females (mean age, 8.7${\pm}$4.5 years) were included. In 22 patients (53.7%), no definitive causes were found. The most common etiologies were choledochal cysts (22.0%). Necrotizing pancreatitis was diagnosed in 5 patients (12.2%), and recurrent acute pancreatitis in 4 patients (9.8%). CT findings included pancreatic swelling (43.9%), peripancreatic fluid collection (29.3%), ascites (24.4%), and peripancreatic fat necrosis (12.2%). Serum amylase and lipase levels at diagnosis were 535.3${\pm}$553.2 and 766.2${\pm}$723.6 U/L, respectively, and were normalized within 1 week in 22 and 14 patients, respectively. On the basis of the Balthazar scale, 2 patients were diagnosed with severe AP. In 4 patients (9.8%), a surgical procedure was indicated. Major complications included ascites (32.3%), sepsis (16.1%), and pseudocyst and renal impairments (12.9%). Two patients died from multi-organ failure. Conclusion: The etiologies of AP in children are varied. Most children have a single episode and a self-limited course. However, AP of childhood still carries significant morbidity and mortality. Early diagnosis, appropriate treatment according to disease severity, and management of complications are important.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.2
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• pp.55-62
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• 2020

A 22-month-old girl who had taken iron supplements due to iron deficiency anemia, presented bloody mucoid stool for one month. She had a bruise at the right periorbital area due to minor trauma and hepatosplenomegaly. Laboratory studies showed anemia, thrombocytopenia, elevated alkaline phosphatase (ALP), hypophosphatemia, decreased haptoglobin, hypocomplementemia, negative direct/indirect Coomb's test, normal vitamin D3 level and high PTHi. Wrist x-ray showed no signs of rickets. The abdominal ultrasound showed only accessory spleen. Tandem mass spectrometry was normal. During follow up, bloody stool regressed after seven days of withdrawal of iron supplement and cow milk, and the total CO2 level had been within 15-20 mEq/L with normal anion gap. NGS (next generation sequencing) panel test for evaluation of renal tubular acidosis showed negative results. After low dose steroid and vitamin D supplements under the impression of hypocomplementemic vasculitis, thrombocytopenia, C3/C4, decreased haptoglobin, and elevated ALP level became normal. At 57 months of age, laboratory findings showed elevated liver enzyme, ALP and gamma-glutamyl transferase again. And liver cirrhosis with splenomegaly and diffuse renal disease were reported with abdomen CT scan. Liver biopsy reported macro- and micronodular cirrhosis. Urine organic acid profile showed elevated succinylacetone level. Whole exome sequencing revealed novel compound heterozygous mutations (NM_00137.2:c.107T>C, NM_00137, 2:c.614T>C) in FAH gene and confirmed by Sanger sequencing. Consequently, the patient was diagnosed as chronic hereditary tyrosinemia type I. She started low phenylalanine/tyrosine diet and nitisinone treatment. Our case had presented symptoms very slowly, which is the first case of chronic tyrosinemia type I in South Korea.

• Radiation Oncology Journal
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• v.21 no.1
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• pp.94-99
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• 2003

Purpose : To explore a 3D conformal radiotherapy technique for a posterior fossa boost, and the potential advantages of a prone position for such radiotherapy. Materials and Methods :A CT simulator and 3D conformal radiotherapy Planning system was used for the posterior fossa boost treatment on a 13-year-old medulloblastoma patient. He was placed In the prone position and Immobilized with an aquaplast mask and immobilization mold. CT scans were obtained of the brain from the top of the skull to the lower neck, with IV contrast enhancement. The target volume and normal structures were delineated on each slice, with treatment planning peformed using non-coplanar conformal beams. Results : The CT scans, and treatment In the prone position, were peformed successfully. In the prone position, the definition of the target volume was made easier due to the well enhanced tentorium, In audition, the posterior fossa was located anteriorly, and with the greater choice of beam arrangements, more accurate treatment planning was possible as the primary beams were not obstructed by the treatment table. Conclusion : .A posterior fossa boost, in the prone position, Is feasible in cooperating patients, but further evaluation is needed to define the optimal and most comfortable treatment positions.

• Pediatric Infection and Vaccine
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• v.23 no.3
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• pp.217-222
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• 2016

Purpose: This study aimed to identify the differential clinical, laboratory, and imaging characteristics between patients with cervical lymphadenitis as first presentation of Kawasaki disease (CLKD) and those with acute unilateral cervical lymphadenitis (AUCL). Methods: We surveyed 372 patients who visited Pusan National University Children's Hospital because of fever and cervical lymph node enlargement, and underwent neck computed tomography (CT) from January 2010 to December 2014. We compared 28 confirmed cases of Kawasaki disease and 28 cases of AUCL based on a retrospective review of the medical records of the patients. Results: Patients with CLKD and AUCL showed no differential clinical characteristics in terms of the duration of fever, antibiotic use, or the size of lymph nodes. Patients with CLKD had higher white blood cell count, absolute neutrophil count, erythrocyte sedimentation rate, and C-reactive protein levels (P<0.05) than those of patients with AUCL. The presence of retropharyngeal edema on neck CT was similar between the groups (64% vs. 33%, P=0.686). Conclusions: CLKD and AUCL showed no differentiating clinical and radiological characteristics; hence, Kawasaki disease should be the presumptive diagnosis in patients with fever and cervical lymph node enlargements who fail to respond to antibiotic treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.13 no.1
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• pp.86-91
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• 2010

Retroperitoneal cystic lymphangiomas are benign, extremely rare tumors. Although surgical resection is the treatment of choice, sclerotherapy should be considered initially. A 9-year-old boy was admitted due do worsening abdominal pain of 4 days duration. Serial complete blood counts revealed a hemoglobin level of 12.8 g/dL on admission to 10.6 g/dL on hospital day 3. An abdominal computed tomography (CT) scan showed a large, lobulated, septated, retroperitoneal cystic mass ($10{\times}9.5{\times}5cm$) in the left anterior pararenal space with intracystic hemorrhage surrounding the inferior mesenteric vein (IMV). Because of the high operative risk, we performed a tubogram of the cystic mass, percutaneous catheter drainage (PCD), and ethanol sclerotherapy. The follow-up abdominal CT scan showed that the cystic mass had decreased in size. He is well without relapse of the retroperitoneal cystic mass for 13 months after discharge. Sclerotherapy with PCD should be considered as initial therapy for patients with retroperitoneal cystic lymphangiomas at high surgical risk.

• Childhood Kidney Diseases
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• v.14 no.2
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• pp.223-229
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• 2010

Percutaneous transluminal renal angioplasty (PTRA) is the current treatment of choice for renal artery revascularization, but renal autotransplantation has been an alternative treatment for complex cases. Here we report a 14-year-old boy with severe hypertension successfully treated with PTRA and renal autotransplantation. Doppler ultrasonography and computed tomography (CT) angiography revealed slight narrowing in the right renal artery ostium and complete obstruction in the left renal artery ostium with multiple collaterals. PTRA with stent insertion was performed for the treatment of the right renal artery, but it was impossible for the left renal artery due to the total obstruction. Therefore, left nephrectomy for autotransplantation was done with the peritoneal approach and the left kidney was autotransplanted to the ipsilateral iliac fossa. Postoperatively, Doppler ultrasonography and mercapto-acetyl-triglycine (MAG-3) renogram were performed, which showed normal renal artery blood flow and kidney function. Blood pressure was normalized and anti-hypertensive drugs were gradually tapered. Fibromuscular dysplasia was suspected to be responsible for the renal artery stenosis based on clinical aspects. In conclusion, renal autotransplantation is also a good treatment option for children with severe renovascular hypertension when endovascular treatment has failed or is not possible.

• Journal of Radiation Protection and Research
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• v.27 no.3
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• pp.155-164
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• 2002

Methodology to evaluate the effective doses to adults undergoing various diagnostic x-ray examinations were established by Monte Carlo simulation of the x-ray examinations. Anthropomorphic mathematical phantoms, the MIRD5 male phantom and the ORNL female phantom, were used as the target body and x-ray spectra were produced by the x-ray spectrum generation code SPEC78. The computational procedure was validated by comparing the resulting doses to the results of NRPB studies for the same diagnostic procedures. The effective doses as well as the organ doses due to chest, abdomen, head and spine examinations were calculated for x-rays incident from AP, PA, LLAT and RLAT directions. For instance, the effective doses from the most common procedures, chest PA and abdomen AP, were 0.029 mSv and 0.44 mSv, respectively. The fact that the effective dose from PA chest x-ray is far lower than the traditional value of 0.3 mSv(or 30 mrem), which results partly from the advances of technology in diagnostic radiology and partly from the differences in the dose concept employed, emphasizes necessities of intensive assessment of the patient doses in wide ranges of medical exposures. The methodology and tools established in this study can easily be applied to dose assessments for other radiology procedures; dose from CT examinations, dose to the fetus due to examinations of pregnant women, dose from pediatric radiology.

• Journal of Korean Neurosurgical Society
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• v.30 no.sup2
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• pp.309-315
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• 2001

The term slit ventricle syndrome(SVS) refers to an episodic occurrence of headache, vomiting, and possibly some degree of impaired consciousness in shunted hydrocephalic children in whom slit-like ventricles are seen on CT scan or MRI. Authors present 6 cases with SVS who were treated at our institute for last 10 years. From 1986 to 1996, 821 patients underwent shunt surgery for hydrocephalus. The etiology of hydrocephalus included brain tumor(140 patients), post-hemorrhagic(62 patients), idiopathic normotensive hydrocephalus(64 patients), post-meningitic( 58 patients), post-traumatic(54 patients), congenital(48 patients), neurocysticercosis(31 patients), and unknown etiology(364 patients). During the mean follow-up duration of 68 months, 232 shunt revisions were performed by a revision rate of 1.28 per patient. The incidence of SVS was 0.7%(6 patients). Most of them have been operated on in infancy. Time interval from the first operation to the development of slit-ventricle syndrome ranged from 4 to 8 years, the mean was 6 years. Shuntogram showed patent shunt in all patients. Two patients with less severe clinical symptoms improved with conservative treatment. These patients were not measured ICP because of good hospital course. One patient showed high ICP and needed only revision with same pressure valve as previous shunt. Low ICP was noted in 3 patients. Pressure augmentation using an anti-siphon device(ASD) or upgrading valve system were necessary in these patients. The authors stress that determining type of SVS is the first step in treatment planning and that the best treatment is a strategy aimed at resolving the specific type of SVS responsible for the symptoms.