Posttransplant lymphoproliferative disease(PTLD) has emerged as a potential life-threatening complication of immunosuppressive therapy after organ transplantation. The occurrence of PTLD is usually associated with an Epstein-Barr virus(EBV) infection in patients who are treated by aggressive immunosuppressive therapy. PTLD is represented by diverse manifestations ranging from reactive lymphoid hyperplasia to high grade malignant lymphoma. This is a case report of a late PTLD in a child. The patient is a 14-year-old girl, who presented as malignant lymphoma 44 months after successful renal transplantation. There was no evidence of EBV infection. On bone marrow study, many neoplastic lymphoid cells were detected. Aggressive chemotherapy for PTLD had resulted in clinical remission. However the patient expired from uncontrolled sepsis and septic shock after 77 days.
Adolescent ; Bone Marrow ; Child* ; Drug Therapy ; Epstein-Barr Virus Infections ; Female ; Herpesvirus 4, Human ; Humans ; Kidney Transplantation* ; Lymphocytes ; Lymphoma ; Organ Transplantation ; Pseudolymphoma ; Sepsis ; Shock, Septic ; Transplants

99mTc-MAG3 Scintigraphic Scan is sensitive at depicting focal parenchymal abnormalities and can be used for the measurement of overall renal function. We experienced a 12-year-old girl presenting with fever and flank pain. On the ultrasonogram and post-voiding delayed image of 99mTc-MAG3 scintigraphic scan, severe right cortical atrophy and hydronephrosis with vesicoureteral reflux were detected. We could demonstrate the reflux nephropathy by these two diagnostic work-up without conventional voiding cystourethrography.
Atrophy ; Child ; Female ; Fever ; Flank Pain ; Humans ; Hydronephrosis ; Technetium Tc 99m Mertiatide ; Ultrasonography ; Vesico-Ureteral Reflux

Fanconi syndrome is a generalized functional disorder of the proximal tubule of the kidney and is characterized by aminoaciduria, glycosuria, hyperphosphaturia, dehydration, rickets, and growth failure. Nephrocalcinosis and hypercalciuria are rare manifestations of Fanconi syndrome. There is no case report of Fanconi syndrome complicated with nephrocalcinosis and hypercalciuria in Korea. A 6-year-old boy presented with genu valgum and waddling gaits for about 3 years. There was no family history of renal disease and his physical examination was normal except for genu valgum and corrected cleft lip and palate. Laboratory investigations showed generalized aminoaciduria, glycosuria, hyperphosphaturia, hypercalciuria, and low-molecular weight proteinuria including beta2-microglobulin. Serum 25-OH vitamin D3 was within the normal range, and 1,25-(OH)2 vitamin D3 was elevated. Bilateral renal medullary hyperechogenicity was demonstrated by ultrasonography. Analysis of the CLCN5 gene revealed no mutation. Here we describe a boy with Fanconi syndrome complicated with nephrocalcinosis and discuss the differential diagnosis.
Child ; Cholecalciferol ; Cleft Lip ; Dehydration ; Diagnosis, Differential ; Fanconi Syndrome* ; Gait ; Genu Valgum ; Glycosuria ; Humans ; Hypercalciuria ; Hypophosphatemia, Familial ; Kidney ; Korea ; Male ; Nephrocalcinosis* ; Palate ; Physical Examination ; Proteinuria ; Reference Values ; Rickets ; Ultrasonography

Mortality and morbidity of malignant hyperthermia has decreased markedly by the avoidance of succinylcholine, and the earlier detection and introduction of dantrolene. We report a fourteen-year-old boy who developed malignant hyperthermia during general anesthesia. He showed the earlier clinical signs, such as elevation of end-tidal CO2, tachycardia, and hypertension. After prompt administration of dantrolene, operation was continued with profopol and midazolam. Rhabdomyolysis and myoglobinuria followed, and were managed by hydration and alkalinization of urine. Azotemia did not occur, and he was discharged without any sequelae on the 10th postoperative day.
Anesthesia, General ; Azotemia ; Dantrolene ; Humans ; Hypertension ; Male ; Malignant Hyperthermia* ; Midazolam ; Mortality ; Myoglobinuria ; Rhabdomyolysis* ; Succinylcholine ; Tachycardia

Oligmeganephronia is congenital hypoplasia of kidney with renal pathology showing very small number of nephrons with compensatory hypertrophy of the remaining glomeruli. A 7- year-old girl was referred to our nephrology clinic due to hematuria detected on school screening urinalysis and diagnosed as chronic renal failure and oligomeganephronia on renal biopsy. We are reporting the clinical and histomorphometric changes for the four years follow-up with review of literatures.
Biopsy ; Female ; Follow-Up Studies* ; Hematuria ; Humans ; Hypertrophy ; Kidney ; Kidney Failure, Chronic ; Mass Screening* ; Nephrology ; Nephrons ; Pathology ; Urinalysis*

We report a case of severe(gradeV) unilateral vesicoureteral reflux(VUR) without any renal damage in a 6-month-old boy through the early sibling screening test for VUR, whose old brother had reflux nephropathy. The early detection of VUR aroused us to take special precautions to prevent urinary tract infection in this patient. We believe that this approach was helpful to reduce the risk of renal damage in this patient and are reporting the case with a brief review of related literatures. It is our firm suggestion that screening for vesicoureteral reflux should be carried out in every child who has siblings with reflux nephropathy.
Child ; Humans ; Infant ; Male ; Mass Screening* ; Siblings* ; Urinary Tract Infections ; Vesico-Ureteral Reflux*

Wilson s disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson s disease. However, nephrotoxicity can occur after D-penicillamine treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syndrome in a 3-year-old girl with Wilson s disease 3 weeks after initiation of D-penicillamine.
Brain ; Child, Preschool ; Cornea ; Female ; Glomerulonephritis, Membranous ; Humans ; Liver ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Penicillamine* ; Trientine ; Zinc Sulfate

Obesity-associated focal segmental glomerulosclerosis(OB-FSGS) has been known to progress into advanced renal insufficiency, and its clinicopathological features include obesity, FSGS lesions with glomerulomegaly and, nephrotic-range proteinuria without edema. A 14- year old girl with Prader-Willi syndrome showed nephrotic-range proteinuria without hypoalbuminemia or edema. The renal biopsy revealed focal segmental glomerulosclerosis together with glomerular hypertrophy and an increased mesangial matrix. We report here a case of OB-FSGS as one of the renal problems of Prader-Willi syndrome, and we came to the conclusion that Prader-Willi syndrome is one of the possible disease entities that can lead to renal insufficiency through obesity.
Biopsy ; Child* ; Edema ; Female ; Glomerulosclerosis, Focal Segmental* ; Humans ; Hypertrophy ; Hypoalbuminemia ; Obesity ; Prader-Willi Syndrome* ; Proteinuria ; Renal Insufficiency

Acute poststreptococcal glomerulonephritis(APSGN) is the most common form of postinfectious glomerulonephritis, and acute pyelonephritis(APN) is the most severe form of urinary tract infection in childhood. However, the concurrence of two diseases is uncommon in the literature. We describe a case of APSGN accompanied with APN in a 5-year-old female who presented with fever, left flank pain, headache and facial edema. Urinalysis showed pyuria, microscopic hematuria, and mild proteinuira. Serial urine cultures grew Escherichia coli. (99m)Tc-DMSA renal scan revealed a cortical defect in the upper pole of left kidney. She had a history of preceding pharyngitis, in addition, showed high blood pressure, high anti-streptolysin O titer, and low serum complement levels. The patient improved completely with supportive treatment, including antibiotic and antihypertensive therapy. These findings suggested that APSGN and APN could be manifested simultaneously or be superimposed on each other.
Child, Preschool ; Complement System Proteins ; Edema ; Escherichia coli ; Female ; Fever ; Flank Pain ; Glomerulonephritis* ; Headache ; Hematuria ; Humans ; Hypertension ; Kidney ; Pharyngitis ; Pyelonephritis* ; Pyuria ; Urinalysis ; Urinary Tract Infections

PURPOSE: This study was performed to elucidate the clinical pictures of acute focal bacterial nephritis(nephronia) in children. METHODS: We reviewed 9 children with nephronia diagnosed by ultrasonography or computed tomography of kidneys from September 1994 to August 2004. RESULTS: The overall male to female ratio was 2:1, and the age distribution ranged from 0.1 to 6 years(mean 2.8+/-2.2). The cardinal symptoms were fever, chills, abdominal pain and dysuria/frequency. The initial leukocyte count was 21,000+/-5,600/uL; ESR, 60+/-23 mm/hr; CRP, 17+/-10 mg/dl. Pyuria was noted in every patient and persisted for 10.5+/-7.8 days after antimicrobial treatment. Abdominal sonography demonstrated focal lesion of ill-defined margin and low echogenicity in 5 of 9 patients(55.6%), while computed tomography revealed nonenhancing low density area in all patients(100%). Three of 9 patients(33.3%) had vesicoureteral reflux, greater than grade III. The initial (99m)Tc-DMSA scan showed one or multiple cortical defects in every patient, and improvements were noted in 2(33.3%) of 6 patients who received follow up scan after 4 months. Intravenous antibiotics was given in every patient under admission. Total febrile period was 11.8+/-6.3 days(pre-admission, 4.0+/-3.0; post-admission, 7.8+/-5.5 days) and the patients needed hospitalization for 17.2+/-8.1 days. CONCLUSION: For the early diagnosis of 'acute focal bacterial nephritis' we should perform renal computed tomography first rather than ultrasonography, when the child has toxic symptoms and severe inflammatory responses in blood and urine.
Abdominal Pain ; Age Distribution ; Anti-Bacterial Agents ; Child* ; Chills ; Early Diagnosis ; Female ; Fever ; Follow-Up Studies ; Hospitalization ; Humans ; Kidney ; Leukocyte Count ; Male ; Nephritis* ; Pyuria ; Ultrasonography ; Vesico-Ureteral Reflux