• The Korean Journal of Nuclear Medicine
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• v.9 no.2
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• pp.13-22
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• 1975

The purpose of the present study is to evaluate the diagnostic value of the ETR test as compared to other thyroid function tests in normal persons, patients with thyroid disorders and patients with alterations of thyroxine-binding proteins. The ETR values were obtained from 35 cases as normal control, 63 hyperthyroid patients, 56 euthyroid patients, 23 hypothyroid patients, 10 pregnant women, 5 women taking oral contraceptive medication, 8 liver cirrhosis patients and 4 nephrotic syndrome patients. The results obtained were as follows. 1. The mean value of ETR obtained from the normal controls was $0.99{\pm}0.06$. 2. The mean ETR values of various thyroid states were $1.25{\pm}0.16$ in hyperthyroidism, $0.99{\pm}0.08$ in euthyroidism and $0.82{\pm}0.05$ in hypothyroidism and significant difference was found between these groups. 3. Seven out of 63 hyperthyroid patients(11.1%) and 2 out of 23 hypothyroid patients(8.7%) had ETR values within normal range and among the 56 euthyroid patients 6(10.7%) had ETR values outside normal range, so the diagnostic compatibility of ETR was 89.4% in thyroid diseases. 4. Even though the ETR value was well correlated with $^{131}I$-thyroid uptake rate, serum $T_3$ resin uptake rate and serum $T_4$, a high positive correlation was found (r=0.79) between ETR and $T_7$. 5. The mean ETR values from patients with alteration in TBG binding capacity were $0.99{\pm}0.05$ in pregnant women, $0.98{\pm}0.04$ in women with oral contraceptive medication, $1.04{\pm}0.09$ in liver cirrhosis patients and $0.94{\pm}0.02$ in nephrotic syndrome patients and most of them (85.2%) had ETR values within normal range. Our results, therefore, suggests that the ETR estimation does offer the simplest and most reliable single procedure for the screening and diagnosis of various thyroid diseases as a indirect indicator of serum-free thyroxine concentration without essential influence of changes in the thyroxine-binding proteins in serum.

• Pediatric Infection and Vaccine
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• v.21 no.1
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• pp.65-70
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• 2014

Although congenital syphilis can be prevented with prenatal screening, the disease remains problematic. Currently, there are no cases that describe hematuria and pneumonia related to congenital syphilis. We report a case of congenital syphilis that involved nephrotic syndrome and pneumonia alba in a 22-day-old male infant whose mother did not receive adequate prenatal care. The congenital syphilis diagnosis was confirmed with a serologic test and the patient recovered with penicillin treatment. Clinical findings may be subtle in neonates and delayed recognition occurs frequently, thus complete prenatal screening is critical for congenital syphilis prevention. Immediate serologic testing should be performed to obtain a differential diagnosis if an infant is delivered by a mother that has not received appropriate prenatal examinations.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.170-175
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• 2009

Purpose : FSGS do not respond well to any kind of therapy and gradually progress to end-stage renal disease. This study was conducted to investigate the difference of protein expression between MCNS and FSGS as a preliminary study for understanding the pathophysiology of FSGS. Methods : Renal biopsy samples of MCNS and FSGS were obtained, which was diagnosed by one pathologist. They were solubilized with a conventional extraction buffer for protein extraction. The solution was applied on immobilized linear gradient strip gel (pH 4-7) using IPGphor system. Silver staining was carried out according to standard method. Protein identification was done by searching NCBI database using MASCOT Peptide Mass Fingerprint software. Results : The differences in protein expressions between MCNS and FSGS were shown by increased or decreased protein spots. Most prominently expressed spot among several spots in FSGS was isolated and analyzed, one of which was glutathione S-transferase (GST) P1-1, whereas it was not found in MCNS. So GSTP1-1 was considered as the one of the key biomarkers in pathogenesis of FSGS. Conclusion : This result would be helpful in diagnosing FSGS and researching FSGS. Further studies for glutathione S-transferase P1-1 might be necessary to elucidate the mechanisms regarding FSGS.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.1-9
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• 2004

Purpose : Minimal Change Disease (MCD) is the most common primary nephrotic syndrome in children. Some suggested that tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$) are involved in the pathogenesis of MCD. Methods : This study was done to see the changes of plasma and urinary $TNF-{\alpha}$, and its effect on the determination of permeability of the glomerular basement membrane (BM) contributed by heparan sulfate proteoglycan (HSPG). Study patients consisted of 19 biopsy-proven MCD children aged 2-15 years old. Both plasma and urinary $TNF-{\alpha}$ were measured. Employing the Millicell system, $TNF-{\alpha}$ was screened for the permeability factors. We examined whether $TNF-{\alpha}$ regulated BM HSPG gene expression and HS synthesis in the glomerular epithelial cells (GECs). Results : Urinary $TNF-{\alpha}$ during relapse was significantly increased when compared with that of during remission or controls ($364.4{\pm}51.2$ vs $155.3{\pm}20.8,\;36.0{\pm}4.5$ ng/mg cr) (P<0.05). However, negative results were obtained in the permeability assay using the Millicell system. No difference was seen in the BM HSPG gene expression and HS synthesis in the GECs. Conclusion : It seems that $TNF-{\alpha}$ may not play a disease-specific role in the pathogenesis of MCD.

• Childhood Kidney Diseases
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• v.24 no.2
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• pp.120-125
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• 2020

Gorham-Stout syndrome is a rare bone disorder characterized by progressive massive osteolysis and proliferation of vascular and lymphatic vessels. A 15-year-old boy was initially diagnosed with Gorham-Stout at the age of 8 years based on clinical and radiological findings. Following diagnosis, he was treated with pamidronate, interferon alfa, propranolol, oral corticosteroids, and sirolimus. He developed proteinuria at the age of 15 and progressed into the nephrotic range 2 years later. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified variant. The sequential increase in proteinuria associated with medications suggested that the focal segmental glomerulosclerosis may be caused by pamidronate and sirolimus, but cannot completely rule out the possibility of kidney involvement of GSS itself.

• The Korean Journal of Cytopathology
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• v.11 no.2
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• pp.109-114
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• 2000

Amyloid golfer is a rare disease entity that is defined as a symptomatic mass or clinically detectable thyroid enlargement because of amyloid deposition. We present a case of amyloid golfer diagnosed in the fine needle aspiration cytology(FNAC) in a 73-year-old Korean woman presented with nephrotic syndrome and thyroid enlargement. The thyroid function was in normal range. Thyroid scan showed a nodule, $4{\time}2cm$ in the right lobe with underlying diffuse golfer. Aspirates revealed benign looking follicular cells and scattered eosinophilic material. The sections of the cell block showed nodular deposit of eosinophilic hyalinized material in the interfollicular area. It showed apple-green birefringence under polarization with Congo red stain. The renal biopsy also exhibited deposition of eosinophilic materials in the glomeruli and interstitial vascular wall, which were confirmed as amyloidosis. This material was morphologically distinct from the colloid.

• Journal of Chest Surgery
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• v.23 no.2
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• pp.253-259
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• 1990

Between January, 1970 and August, 1989, a total of 81 patients whose age were more than 20 years of life, received total correction for tetralogy of Fallot. This report analyzed 70 patients among them and excluded the remaining 11 patients whose clinical data could not be found. Their mean age was 25.750.39 years[range 20 \ulcorner50]. The clinical manifestations were cyanosis and clubbing [64 pts], frequent URI[40 pts], anoxic spell [19 pts], infective endo-carditis[4 pts], brain abscess[3 pts], pulmonary tuberculosis[3 pts] and CHF, chest tightness, nephrotic syndrome, left hemiplegia, and tamponade. The types of right ventricular outflow tract obstruction were combined[46 pts], pure infundibular [21 pts] and pure valvular[3 pts]. Associated cardiovascular anomalies were PFO [27 pts], ASDi8 pts], LSVC[8 pts], aortic regurgitation [5 pts], right aortic arch, coronary artery anomalies, PDA and dextrocardia. Hospital mortality was 5.7%. The causes of death ware low cardiac output [2 pts], aggravation of CRF[1 pts] and brain damage[1 pts]. There was one late death because of residual intracardiac shunt and congestive heart failure. During the follow-up period, 16 patients were lost and the remaining 49 patients were asymptomatic and leading normal lives. Residual intracardiac shunt was detected in 5 patients with radionuclide single pass study but all of them had Qp / Qs ratio less than 1.5.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.160-164
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• 2017

C3 glomerulopathy (C3G) is a recently defined pathological entity characterized by C3 accumulation with absent or scant immunoglobulin deposition, leading to variable glomerular inflammation. The clinical presentation of patients with C3G is highly variable, as they may present with symptoms ranging from microscopic or mild proteinuria to full-blown nephrotic syndrome, with or without renal impairment. However, there is no consensus recommendation for specific treatment in children with C3G. Recently, new therapies have been suggested to target complement pathways, owing to an improvement in the understanding of the pathogenesis of C3G. C3G complement blockade with eculizumab, a monoclonal antibody targeted against complement C5, inhibits activation of the alternative complement pathway. We could not use eculizumab owing to its high price; thus, we administered oral prednisolone and mycophenolate mofetil (MMF). MMF was replaced with cyclosporine because proteinuria persisted, with a consistently low serum C3 level; we tapered off the prednisolone because of a Cushingoid appearance and amenorrhea. Thereafter, proteinuria improved, and the serum C3 level returned to normal. Thus, we report the effectiveness of cyclosporine in a patient with C3G and an inadequate response to prednisolone and MMF, who was detected via school urinary screening.

• Childhood Kidney Diseases
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• v.21 no.2
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• pp.165-168
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• 2017

Focal segmental glomerulosclerosis (FSGS) in children, which is a kind of nephrotic syndrome showing steroid resistance, usually progresses to a substantial number of end stage renal disease (ESRD). Although the pathogenesis of primary FSGS is unclear, several recent studies have reported that FSGS is associated with circulating immune factors such as soluble urokinase-type plasminogen activator receptor (suPAR) or anti-CD40 autoantibody. We report a successfully treated case of a 19-year-old female patient who experienced a recurrence of primary FSGS. After the diagnosis of FSGS, the patient progressed to ESRD and received a kidney transplantation (KT). Three days later, recurrence was suspected through proteinuria and hypoalbuminemia. She has been performed plasmapheresis and high dose methylprednisolone pulse therapy and shown remission status without increasing proteinuria for four years after KT. In conclusion, strong immunosuppressive therapy may be helpful for a good prognosis of recurrent FSGS, suppressing several immunologic circulating factors related disease pathogenesis.

• Annals of Clinical Neurophysiology
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• v.17 no.1
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• pp.24-27
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• 2015

Amyloidosis is a systemic disorder associated with clonal plasma cell dyscrasia. Nephrotic syndrome, congestive heart failure, autonomic and peripheral neuropathy is often associated features in amyloidosis. Early diagnosis is most important because of different prognosis by stage. The diagnosis can be delayed since symptoms of amyloidosis may vary or nonspecific. We describe a patient of myeloma-associated amyloidosis, who showed orthostatic intolerance as the first symptom of the disease.