• Childhood Kidney Diseases
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• v.3 no.2
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• pp.232-236
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• 1999

IgA 신병증은 사구체 중맥에 IgA가 침착하는 것이 특징적인 질환이며 막성 사구체 신염은 IgG가 사구체 기저막의 상피하에 미만성으로 침착하는 질환이다. 원발성 사구체 질환중 IgA 신병증과 막성 사구체신염은 비교적 흔한 질환이나 전체적인 빈도는 낮은 편으로, 한 환자의 사구체에서 두 질환이 동시에 발생하는 경우는 매우 드물다. IgA 신병증과 막성 사구체신염의 중복 신염은 Doi등이 1983년 원발성 신질환으로서 처음 보고한 이래, 성인에서 20여례가 보고되었다. 저자들은 신증후군이 발생한 환아에서 신생검을 시행한 결과 원발성 신질환으로서 IgA 신병증과 막성 사구체신염의 소견이 동시에 보이는 중복신염의 드문 예를 경험하였기에 보고하는 바이다. 환아는 7세된 남아로 내원 한달 전부터 발생한 전신부종을 주소로 내원하였다. 가족력과 과거력상 특이 소견없었으며, 내원시 이학적 소견상 전신적인 허약감과 안와부종, 복부팽만, 하지의 함요부종이 관찰되었고, 검사소견에서는 WBC $19,800/mm^3$, Hb 14.1g/dL, Platelet $397,000/mm^3$, BUN/Cr 10/0.4mg/dL, protein/albumin 4.43/2.73g/dL, cholesterol 429mg/dL, IgA 85mg/dL, $C_3$ 68.8mg/dL, $C_4$ 13.4mg/dL, ANA(-), ANCA(-), RF(-), HBsAg/Ab(-/-)이었다. 뇨검사에서는 RBC many/HPF, WBC 2-3/HPF, protein ${\le}\;300mg/dL$ 였으며, 24시간 소변 검사상 protein 9.18g/day, Ccr 66.67ml/min의 소견을 보였다. 신생검을 시행한 결과 광학현미경상에서 몇몇 사구체의 분절성 경화와 중맥역의 증식이 관찰되었고, 면역형광현미경검사에서는 IgA(3+)가 과립상으로 미만성 분포를 보이며 중맥역에 침착되어 있고, 미세한 과립상과 위선의 양상으로 IgG(1+)가 모세혈관벽에 침착되어 있었으며, 전자현미경 소견상 중맥역과 모세혈관 기저막 상피하에 소량의 전자 고밀도 침착이 함께 관찰되었다. 환아는 prednisolone을 경구 투여 받았으나 단백뇨와 혈뇨가 지속되어 solumedrol pulse therapy, captopril과 cyclophophamide로 치료 받은 후, 전신 상태 호전되고, 혈뇨가 사라졌으며, 24시간 소변 검사상 단백뇨가 487.5mg/day로 감소하여 외래에서 추적 관찰 중이다.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.38-45
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• 2005

Purpose : The purpose of this study was to analyze the therapeutic effect of plasmapheresis in various pediatric diseases. Methods : Therapeutic plasmapheresis was performed by COBE Spectra centrifugation. Nine cases were included in this study. The number an[;. method of plasmapheresis, together with the progress and prognosis of each case were retrospectively reviewed. Results : The patients' ages ranged from 26 mont]Is to 16 years of age, and the mean age was 9.9 years. There were S males and 4 females. The underlying diseases requiring plasmapheresis included 2 cases of hemolytic uremic svndrome(HUS), 1 case of lupus nephritis, 2 cases of rapidly Progressive glomerulonephritis(RPGN), 1 case of focal segmental glomorulosclerosis(FSGS), 1 case of systemic vasculitis after pulmonary hemorrhage, 1 case of acute renal failure associated with pulmonary hemoIThage, and 1 case of acute rejection after renal transplantation. The average number of plasmapheresis performed was 6.2 times with a range of 3 to 13 times. The patients with HUS, lupus nephritis, ANCA positive systemic vasculitis induced by pulmonary hemorrhage and ARF-associated pulmonary hemorrhage showed a good response to therapeutic plasmapheresis, but the patients with RPGN, refractory FSGS, and acute rejection after renal transplantation were not responsive to treatment. The most common side effect was hypocalcemia which was rarely symptomatic. Vital signs were not compromised. Conclusion : Although it is presumptuous to generalize the therapeutic effects of plasma pheresis in different diseases due to the small number of study subjects, this study shows that plasmapheresis may be an effective therapeutic modality in various pediatrics diseases and should be considered as a therapeutic option.

• Annals of Clinical Neurophysiology
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• v.4 no.2
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• pp.146-148
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• 2002

Progressive systemic sclerosis (PSS) is a multi-systemic disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. But it rarely affects the peripheral nervous system. We report a 36-year-old man of painful trigeminal neuropathy as a complication of PSS. He was referred from Rheumatology for the evaluation of abruptly developed bilateral facial pain. He had facial hypesthesia and paresthesia on neurologic examinations. In the blink reflex, ipsilateral and contralateral R1 and R2 responses were not detected during bilateral supraorbital stimulation. But normal latency and CMAP amplitude of facial NCV were found. Under the impression of trigeminal neuropathy caused by PSS, steroid therapy was tried, and his clinical symptoms and electrophysiologic findings were improved. PSS could be the cause of the painful trigeminal neuropathy.

• Childhood Kidney Diseases
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• v.13 no.1
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• pp.84-91
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• 2009

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene and G the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.26-33
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• 1998

This retrospective study was designed to evaluate the prognostic value and histologic correlation of sonography in childhood nephrotic syndrome. Sixty-nine patients with proteinuria over 2g per day at the time of presentation who were treated at the Korea University Hospital were included in this review. They were 1 to 15 years old(mean age, 7.8 years) with 49 males and 20 females. In each patient an ultrasound examination was done using SPA 1000(Diasonics, C.A., U.S.A.) on admission. Tissue specimens were obtained from 46 patients. The paraffin-embedded specimens were reviewed with special reference to interstitial edema, interstitial fibrosis, tubular atrophy, global sclerosis or inflammatory cell infiltrates. Biopsy proven renal disease were minimal change disease(n=20), focal segmental glomerulosclerosis(n=7), membranous glomerulonephritis(n=2), membranoproliferative glomerulonephritis(n=1), $Henoch-Sch\"{o}nlein$ purpura nephritis(n=6), IgA nephropathy(n=5), poststreptococcal glomerulonephritis(n=2), systemic lupus erythematosus(n=1) and Alport syndrome(n=2). There was a significant relationship between increased cortical echogenicity and global sclerosis or tubular atrophy(P<0.05). But no significant relationship was found between increased cortical echogenicity and interstitial fibrosis, interstitial edema, or inflammatory cell infiltration. In biopsy-proven primary nephrotic syndrome(n=30), no significant relationship was found between the increased conical echogenicity and the interstitial edema, interstitial fibrosis, global sclerosis, tubular atrophy or inflammatory cell infiltration. But there was a significant relationship between increased cortical echogenicity and resistance to corticosteroid (P<0.05). These results suggest that increased cortical echogenicity may be due to tubular atrophy or global sclerosis in patients with proteinuria and may be an effective indicator of resistance to corticosteroid in primary nephrotic syndrome.(J Korean Soc of Pediatr Nephrol 2:26-33, 1998)

• Korean Journal of Microbiology
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• v.53 no.4
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• pp.334-336
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• 2017

Porphyromonas gingivalis is a Gram-negative, obligately anaerobic, and nonmotile rod. P. gingivalis is a pathogen of periodontitis and endodontic infection as well as is associated with systemic diseases including atherosclerosis, preterm, and Alzheimer's diseases. P. gingivalis KCOM 2797 (= JS2) was isolated from a human periodontitis lesion. Here, we present the draft genome sequence of P. gingivalis KCOM 2797.

• The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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• v.31 no.4
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• pp.108-116
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• 2018

Objectives : A Report on the Improvement of korean medical treatment of Secondary Raynaud's Syndrome Caused by Systemic Sclerosis. Methods : We treated a Secondary Raynaud's syndrome 59-year-old woman with herbal medicine and acupuncture treatment for 3 months. The evaluation of treatment was confirmed by photographs before and after treatment, VAS, and palm skin temperature. Results : After treatment, the color of both fingernails and finger skins recovered from white to purplish red, and Raynaud phenomenon and palm flaring were decreased. VAS decreased from 8 to 2 and palm skin temperature increased by left hand 1.4 degrees right hand 1.2 degrees. Conclusions : It was shown that korean medical treatment was effective in the conservative treatment of Secondary Raynaud's syndrome caused by systemic sclerosis.

• Journal of the korean academy of Pediatric Dentistry
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• v.45 no.2
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• pp.257-264
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• 2018

Osteopetrosis is characterized by impaired osteoclast function and increased bone density. Infantile osteopetrosis is a severe form of the disease and has characteristics such as diffusely sclerotic skeleton, pancytopenia, cranial nerve entrapment, infection susceptibility, and abnormal craniofacial appearance. Patients with infantile osteopetrosis often experience developmental delay, and may have a short life span. A 14-month-old girl with osteopetrosis presented to the department of pediatric dentistry. Incipient caries on deciduous incisors were observed. The patient revisited 4 years of age. Besides medical problems, oral complications such as growth retardation, narrow upper arch, crowding, dental caries, and abnormal tooth development were observed. After consultation with her pediatrician, dental treatments were performed on the deciduous molars under sedation after a prophylactic antibiotic injection. At a periodic follow-up, multiple deciduous teeth were treated and extracted, and oralrehabilitation with a removable partial denture was initiated. Patient with osteopetrosis are highly susceptible to infection because of their compromised immune system and problems associated with wound healing that lead to osteomyelitis or sepsis development. Active participation in dental care for sugar intake management and proper oral hygiene are obligatory.

• Childhood Kidney Diseases
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• v.4 no.1
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• pp.25-32
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• 2000

Purpose: This retrospective study of 126 children with symptomless primary hematuria was undertaken to determine the distribution of various histologic types by renal biopsy, clinical outcome according to the biopsy findings and also to find out feasibility of performing renal biopsy in these children. Patients and Methods : Study population consisted of 126 children with symptom-less primary hematuria who have been admitted to the pediatric department of Kyung-poot National University Hospital for the past 11 years from 1987 to 1998 and renal biopsy was performed percutaneously. Hematuric children with duration of less than 6 months, evidences of systemic illness such as SLE or Henoch-Schonlein purpura, urinary tract infection, and idiopathic hypercalciuria were excluded from the study. Results : Mean age of presentation was 9.2${\pm}$3.3 years (range ; 1.5-15.3 years) and male preponderance was noted with male to female ratio of 2:1. IgA nephropathy was the most common biopsy finding occuring in 60 children ($47.6\%$), followed by MsPGN in 13 ($10.3\%$), MPGN in 5 ($3.9\%$), TGBM in 6 ($4.7\%$), Alport syndrome in 2 ($1.6\%$), FSGS in 1 ($0.8\%$), and in 39 children ($30.9\%$), 'normal' glomeruli were noted. Recurrent gross hematuria was more common than persistent microscopic hematuria (84 versus 42), and especially in IgA nephropathy, recurrent gross hematuria was the most prevalent pattern of hematuria. In 58 out of 126 cases ($46.0\%$), hematuria was isolated without accompa-nying proteinuria and this was especially true In cases of MsPGN and 'normal' glomer-uli by biopsy finding. Normalization of urinalysis (disappearance of hematuria) in IgA nephropathy, MsPGN and 'normal' glomuli group were similar and it was $14\%,\;27\%\;and\;21\%$ respectively during 1-2 years of follow-up period, and $37.1\%,\;40\%\;and\;35\%$ respectively during 3-4 years of follow-up periods. However, abnormal urinalysis persi-sted in the majority of children with MPGN, TGBM. Alport syndrome and FSGS. Renal function deteriorated progressively in 6 cases (3 with IgA nephropathy, 2 with Alport syndrome and 1 with TGBM). Conclusion : In summary, present study demonstrates that in 126 children with symptomless primary hematuria, IgA nephropathy was the most common biopsy findings followed by MsPGN, MPGN, TGBM, Alport syndrome and FSGS, and 'normal glomeruli' was also seen in 39 cases ($30.9\%$). Renal histology could not be predictable on the clinical findings, so that to establish appropriate long-term planning for these children, we would recommend to obtain precise histologic diagnosis by renal biopsy.

• The Journal of the Korean bone and joint tumor society
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• v.6 no.1
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• pp.41-46
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• 2000

We experienced the case of familiar hypercholesterolemia with multiple xanthomas which was treated by combined surgical and medical therapy. He was 26-year-old male patient of familiar hypercholesterolemia with multiple xanthomas in 22 sites on whole body, and was treated by 17 surgical excisions of the xanthomas and by medical therapy of the hypercholesterolemia. There was a normal healing process of the surgical wounds. Continual postoperative medical therapy of the hypercholesterolemia was done. There was no recurrence of the symptoms during more than 13 months of follow-up. But the serum level of the cholesterol was not lowered significantly, so we are treating him with drug therapy. Familial hypercholesterolemia is caused by a specific disorder of lipid metabolism, and is characterized by increased LDL cholesterol, tendon xanthomas, coronary disease associated with autosomal dominant transmission. Xanthomas usually appear in the second decade of life with familiar hypercholesterolemia which may have high risk for premature coronary atherosclerosis, which might be prevented with early diagnosis and medical treatment. So, orthopedic surgeons do not only excise the xanthomatosis surgically but also can diagnose the underlying hypercholesterolemia.