Imperforate hymen is, with an incidence of 0.1%, a rare female anomaly, which can appear with symptoms such as lower abdominal pain, primary amenorrhea, dysuria, anuria, caused by retention of menstrual blood after the onset of menstruation. Generally urinary retention is caused by psychological conditions, drug effect, infection or congenital anomaly causing acute urinary obstruction. We experienced a patient with symptoms of acute urinary retention, suggesting acute urinary obstruction. The cause for the retention turned out to be an imperforated hymen, which should therefore be mentioned in the literature as a possible cause in cases suspected of urinary retention.
Abdominal Pain ; Amenorrhea ; Anuria ; Dysuria ; Female ; Humans ; Hymen ; Incidence ; Menstruation ; Retention (Psychology) ; Urinary Retention

IgA nephropathy is the most common form of primary glomerulonephritis and chronic glomerular disease worldwide including Korea. Familial gathering of IgA nephropathy suggests that genetic factors contribute to the development of this disease. Although there have been many reports on familial IgA nephropathy with genetic analysis and their pedigrees, there has been few reports in Korea. We reported a partial familial IgA nephropathy pedigree with a brief review of the literatures.
Glomerulonephritis ; Glomerulonephritis, IGA ; Immunoglobulin A ; Korea ; Pedigree

Cytomegalovirus (CMV) is the single most common infection following kidney transplantation and despite prophylactic strategies and the development of new antiviral agents, it still remains a cause of considerable morbidity and mortality. Current literature suggests that CMV infection may trigger rejection. We report a case of late CMV disease in a preemptive seropositive recipient who did not receive CMV prophylaxis. Diarrhea and abdominal cramping persisted after the administration of mycophenolate mofetil (MMF) six months after transplantation and resulted in ileal perforation at eight months after transplantation. The boy recovered after six weeks of treatment with ganciclovir. MMF has been mooted as a risk factor for CMV infection since its introduction, and further investigations are required to confirm its role. More attention to infectious complications is necessary and serial monitoring of viral load is recommended when MMF is administered.
Antiviral Agents ; Colic ; Cytomegalovirus ; Diarrhea ; Ganciclovir ; Kidney ; Kidney Transplantation ; Mycophenolic Acid ; Risk Factors ; Transplants ; Viral Load

PURPOSE: Early diagnosis and treatment of febrile urinary tract infection (UTI) in children is important to prevent kidney damage. This study aims to evaluate the relationship between the presence of pyuria, the severity, and underlying genitourinary anomalies in patients with UTI. METHODS: We retrospectively reviewed 293 patients with febrile UTI who were admitted to Korea University Guro Hospital during the period from June, 2007 until January, 2010. We divided the patients into two groups, one with the finding of pyuria at admission, and the other without, and compared the fever duration, white blood cell counts (WBC) and C-reactive protein (CRP) in peripheral bloods, hydronephrosis, cortical defects, vesicoureteral reflux and admission period. RESULTS: Among the 293 patients with febrile UTI, 189 patients showed findings of pyuria whereas 104 patients did not. Patients with pyuria showed an increment of WBC (14,694+/-485.2 vs. 11,374+/-451.2/uL, P<0.05) and CRP (46.9+/-3.9 vs 17.1+/-3.6 mg/L, P<0.05) in peripheral blood sample. The presence of cortical defects (21.7 Vs 5.8%, P<0.05) and vesicoureteral reflux (15.9 Vs 6.7%, P<0.05) was also increased in patients with pyuria compared to patients without pyuria. There were no specific differences in fever duration, admission period, and hydronephrosis. Within the group with pyuria, CRP in peripheral blood sample increased proportionally with the increment of pyuria (P<0.05). CONCLUSION: In patients with febrile UTI, the increment of WBC in the urine sample can be a helpful predictor for increased CRP in peripheral blood and acute pyelonephritis.
C-Reactive Protein ; Child ; Early Diagnosis ; Fever ; Humans ; Hydronephrosis ; Kidney ; Korea ; Leukocyte Count ; Pyelonephritis ; Pyuria ; Retrospective Studies ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

PURPOSE: Some hormonal and electrolyte abnormalities have been reported in pediatric patients with urinary tract infection (UTI). This study aimed to investigate the relationships between the imbalance of electrolytes and the severity of infection and associated urologic anomalies in children with febrile UTI. METHODS: We retrospectively reviewed 267 patients with febrile UTI who were admitted to Korea University Guro Hospital during the period from January, 2007 until February, 2010. According to the presence of hyponatremia or hyperkalemia, clinical parameters and associated renal anomalies, such as hydronephrosis, cortical defects and vesicoureteral reflux, were compared. RESULTS: 42.7% of all patients had decreased concentration of serum sodium. In patients with decreased concentration of serum sodium, cortical defects were significantly increased compared to normal patients (40.4% vs. 14.4%, P<0.05). White blood cell (WBC) counts (15,721+/-6,553/uL vs. 12,885+/-5,367/uL, P<0.05), C-reactive protein (CRP) (61.8+/-56.1 mg/L, vs. 29.9+/-39.8 mg/L, P<0.05), and erythrocyte sedimentation rate (ESR) (43.9+/-34.3 mm/hr vs. 27.4+/-26.8 mm/hr, P<0.05) in peripheral blood showed significant increases in the group with decreased concentration of serum sodium. Duration of fever, presence of gastrointestinal symptom, the incidence of hydronephrosis and vesicoureteral reflux did not differ between the two groups. None of the patients had significant hyperkalemia. CONCLUSION: We suggest that decreased concentration of serum sodium in febrile UTI might be a helpful marker for leukocytosis and increased CRP and ESR in peripheral blood, and acute pyelonephritis.
Blood Sedimentation ; C-Reactive Protein ; Child ; Electrolytes ; Fever ; Humans ; Hydronephrosis ; Hyperkalemia ; Hyponatremia ; Incidence ; Korea ; Leukocytes ; Leukocytosis ; Pyelonephritis ; Retrospective Studies ; Sodium ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

PURPOSE: The aim of this study is to investigate the change in incidence and clinical characteristics of acute poststreptococcal glomerulonephritis (APSGN) through a single center's experience. METHODS: We retrospectively analyzed the medical records of 53 children who were diagnosed with glomerular nephritis at National Health Insurance Corporation Ilsan Hospital between March 2000 and December 2009. Twenty-six of 53 patients falled to meet the dragnostic criteria of APSGN and were excluded. Chief complaints, physical examinations, urinalysis, urine culture, laboratory results, chest x-ray, and clinical manifestations were all retrieved from the remaining 27 patients. RESULTS: The incidence of APSGN by age was highest in children between six to seven years of age, with similar distributions in both genders, and seasonal incidence was higher from November to February when compared to other months, which was similar to the previously reported studies. However, the annual number of APSGN patients did not show a significant decrease from 2000 to 2009. The incidences of edema and hypertension were 59.3% and 48.1% respectively, suggesting lower incidences than those from previous studies. Six patients (22.2%) experienced hematuria for more than 6 months. Hypertensive encephalopathy, one of APSGN critical complications occurred in one patient but resolved with conservative treatment. CONCLUSION: Our study shows that APSGN still occur as a common renal disease but the disease severity seems to decrease with milder clinical manifestations and less complications compared to past studies.
Child ; Edema ; Glomerulonephritis ; Hematuria ; Humans ; Hypertension ; Hypertensive Encephalopathy ; Incidence ; Medical Records ; National Health Programs ; Nephritis ; Physical Examination ; Retrospective Studies ; Seasons ; Thorax ; Urinalysis

PURPOSE: Focal segmental glomerulosclerosis (FSGS) is the most common glomerulopathy causing pediatric renal failure. Since specific treatment targeting the etiology and pathophysiology of primary FSGS is yet elusive, the authors explored the pathophysiology of FSGS by transcriptome analysis of the disease using an animal model. METHODS: FGS/kist strain, a mouse model of primary FSGS, and RFM/kist strain, as control and the parent strain of FGS/kist, were used. Kidney tissues were harvested and isolated renal cortex was used to extract mRNA, which was run on AB 1700 mouse microarray chip after reverse transcription to get the transcriptome profile. RESULTS: Sixty two genes were differentially expressed in FGS/kist kidney tissue compared to the control. Those genes were related to cell cycle/cell death, immune reaction, and lipid metabolism/vasculopathy, and the key molecules of their networks were TNF, IL-6/4, IFNgamma, TP53, and PPARgamma. CONCLUSION: This study confirmed that renal cell death, immune system activation with subsequent fibrosis, and lipid metabolism-related early vasculopathy were involved in the pathophysiology of FSGS. In addition, the relevance of methodology used in this study, namely transcriptome profiling, and Korean animal model of FGS/kist was validated. Further study would reveal novel pathophysiology of FSGS for new therapeutic targets.
Animals ; Cell Death ; Fibrosis ; Gene Expression Profiling ; Glomerulosclerosis, Focal Segmental ; Humans ; Immune System ; Kidney ; Mice ; Models, Animal ; Parents ; Renal Insufficiency ; Reverse Transcription ; RNA, Messenger ; Sprains and Strains ; Transcriptome

To understand the course of renal diseases well, we must have basic knowledges of histologic procedures of renal biopsy samples as well as basic pathologic changes. This article describes the method of dividing the biopsy samples , fixatives for various pathologic examinations and basic pathologic changes of glomerular diseases. For light microscopic examination, color changes of glomerular structures in PAS, trichrome and PAM stains, normal glomerular patterns compared to various glomerulopathies are introduced. While describing typical staining patterns and intensities of fluorescence in membranous glomerulopathy and IgA nephropathy, basic interpretation of immunofluorescent microscopic examination is described. To understand electron microscopic pictures of renal diseases, preference locations of electron dense deposits in various glomerulonephrites are described with schema. This article is the introduction part of the renal pathology and for the further detail changes of specific entities, we should reference the renal pathology textbooks or articles.
Biopsy ; Coloring Agents ; Electrons ; Fixatives ; Fluorescence ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranous ; Immunohistochemistry ; Light ; Microscopy, Electron ; Microscopy, Fluorescence

Hypertension is one of the most common chronic diseases in childhood and adolescence. Untreated hypertension adversely affects many organs including heart, brain, kidney and peripheral arteries. We reviewed the complication of central nervous system caused by pediatric hypertension. Cerebral blood flows are maintained constantly in response to changes in blood pressure by cerebral autoregulation. Severe hypertension which destructs cerebral autoregulation results in acute hypertensive encephalopathy syndrome, ischemic or hemorrhagic stroke. Chronic pediatric hypertension induces learning disability and cognitive defect which are subclinical symptom prior to brain damage caused by severe hypertension. We should consider the effect of hypertension on pediatric brain because appropriate antihypertensive drugs could prevent these complications.
Adolescent ; Antihypertensive Agents ; Arteries ; Blood Pressure ; Brain ; Central Nervous System ; Chronic Disease ; Communication Disorders ; Heart ; Homeostasis ; Humans ; Hypertension ; Hypertensive Encephalopathy ; Kidney ; Learning Disorders ; Stroke

Although left Left ventricular hypertrophy (LVH) is not only an adaptive response of the heart to increased cardiac workload in hypertension, it surelybut also is the most potent risk factor of overt cardiovascular complications such as coronary heart disease, heart failure, arrhythmia and stroke in the hypertensive population. Also it has become generally accepted that subclinical cardiovascular disease begins in childhood and LVH is the most readily assessed marker for that. As LVH can be seen in children and adolescents with even mild blood pressure elevation with the reported prevalence of 10 to 47%, aggressive antihypertensive treatment is critical in preventing the development of hypertensive heart disease in that those cases.
Adolescent ; Arrhythmias, Cardiac ; Blood Pressure ; Cardiovascular Diseases ; Child ; Coronary Disease ; Heart ; Heart Diseases ; Heart Failure ; Humans ; Hypertension ; Hypertrophy ; Hypertrophy, Left Ventricular ; Pediatrics ; Prevalence ; Risk Factors ; Stroke