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A Case of Posttransplant Lymphoproliferative Disease Following Renal Transplantation in a Child.

Won Kyoung JHANG ; Hyewon HAHN ; Mee Jeung LEE ; Young Seo PARK ; Thad T GHIM

Journal of the Korean Society of Pediatric Nephrology.2003;7(2):245-252.

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

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Reflux Nephropathy Detected by Post-voiding Image on Scintigraphic Tc-99m MAG3 Scan.

Hong Guk KIM ; Jeyun YU ; Ju Yeon HAM ; Ki Soo PAI ; Suk Nam YUN

Journal of the Korean Society of Pediatric Nephrology.2003;7(2):239-244.

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

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A Case of the Fanconi Syndrome with Nephrocalcinosis.

Young Mi KIM ; Seong Shik PARK ; Ju Suk LEE ; Su Yung KIM

Journal of the Korean Society of Pediatric Nephrology.2003;7(2):234-238.

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

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A Case of Childhood Malignant Hyperthermia Complicated by Rhabdomyolysis.

Bum Hee LEE ; Jin Sook LEE ; Hee Yeon CHO ; Ju Hyung KANG ; Hee Gyung KANG ; Il Soo HA ; Hae Il CHEONG ; Yong CHOI

Journal of the Korean Society of Pediatric Nephrology.2003;7(2):229-233.

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

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A Four-year Follow-up Case of Oligomeganephronia Detected Early by School Screening Urinalysis.

Ju Yeon HAM ; Nam Hyang KOO ; Ki Soo PAI ; Hyun Yi LIM ; Kee Hyuck KIM

Journal of the Korean Society of Pediatric Nephrology.2003;7(2):223-228.

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*

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Four Cases of Autosomal Recessive Polycystic Kidney Disease.

Woong Kyu CHOI ; Seung Cheol LEE ; Yong Won PARK ; Chong Guk LEE

Journal of the Korean Society of Pediatric Nephrology.1997;1(1):91-96.

Endometrial papillary serous carcinoma (EPSC) is a distinct variant of endometrial adenocarcinoma that histologically resembles ovarian serous papillary adenocarcinoma and has an aggressive clinical course. Usually, the tumor is diagnosed at the advanced stage. The tumor has well confused with metastatic ovarian tumor of identical histology. Dignosis of EPSC should be considered when the cervico-vaginal smear reveals numerous papillary clusters of tumor cells with macronucleoli and psammoma bodies. Recently, we have experienced two cases of EPSC diagnosed on cervico-vaginal smears, which revealed characteristic cytologic features including numerous papillary clusters of tumor cells with macronucleoli. The cytologic diagnoses were confirmed on histologic sections.
Adenocarcinoma ; Adenocarcinoma, Papillary ; Diagnosis ; Polycystic Kidney, Autosomal Recessive*

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A Case of Henoch-Schonlein Purpura with Epididymitis.

Yong Ho AHN

Journal of the Korean Society of Pediatric Nephrology.1997;1(1):86-90.

Small cell neuroendocrine carcinoma of the uterine cervix is a distinct subtype of cervical cancer that appears analogous to oat cell carcinoma and carcinoid tumors of the lung. It has been assumed to be derived from the neural crest via argyrophilic cells in the normal endocervix. We have recently encountered a case of small cell neuroendocrine carcinoma of the uterine cervix coexisting with adenocarcinoma which was argyrophil negative. A 66-year-old multiparous woman was admitted because of vaginal bleeding for 2 months. Cervicovaginal smear revealed several scattered clusters and sheets of monotonous small cells with some peripheral palisading in the background of hemorrhage and necrosis. Radical hysterectomy specimen revealed an ulcerofungating tumor on endocervical canal which was composed of two components. Major component of the tumor was made up of monomorphic population of small oval-shaped tumor cells arranged in sheets and partly in acinar structures or trabecular fashion. Other component was adenocarcinoma, endocervical well-differentiated type. Argyrophilia was present on the Grimelius stain and immunohistochemical studies revealed diffuse positivity to neuron-specific enolase and carcinoembryonic antigen. Electron microscopic examination showed clusters of small round to oval cells, which had a few well-formed desmosomes and several membrane-bound, dense-core neuro- sectetory granules.
Adenocarcinoma ; Aged ; Carcinoembryonic Antigen ; Carcinoid Tumor ; Carcinoma, Neuroendocrine ; Carcinoma, Small Cell ; Cervix Uteri ; Desmosomes ; Epididymitis* ; Female ; Hemorrhage ; Humans ; Hysterectomy ; Lung ; Male ; Necrosis ; Neural Crest ; Phosphopyruvate Hydratase ; Purpura, Schoenlein-Henoch* ; Uterine Cervical Neoplasms ; Uterine Hemorrhage

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A Case of Rapidly Progressive Glomerulonephritis in Henoch-Schonlein Purpura.

Soo Hee CHANG ; Dae Sun JO ; Dae Yeol LEE

Journal of the Korean Society of Pediatric Nephrology.1997;1(1):82-85.

A case of primary non-clear-cell adenocarcinoma of the vagina is reported occurring in a 65-year-old woman without exposure to diethylstilbestrol (DES) in utero. The adenocarcinoma did not appear to be associated with vaginal adenosis. It lacked clear cell component and interestingly composed of columnar epithelial cells of endocervical-type. Cytologically round to oval nuclei revealed one or more small nucleoli and fine granular chromatin pattern. Cytoplasm was plump, faintly basophilic and homogeneously stained. Histologically well differentiated columnar epithelial cells were arranged in trabecular pattern mainly, and also occasional glandular lumina and small solid sheets were found. Mitoses were hardly found.
Adenocarcinoma ; Aged ; Basophils ; Cellular Structures ; Chromatin ; Cytoplasm ; Diethylstilbestrol ; Epithelial Cells ; Female ; Glomerulonephritis* ; Humans ; Mitosis ; Purpura, Schoenlein-Henoch* ; Vagina

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Minimal Change Nephrotic Syndrome Presented with Acute Renal Failure in a Child.

Kwang Sik RHO ; Chang Youn LEE ; Soo Jun PARK ; Gu Hyun LEE ; Pyung Kil KIM

Journal of the Korean Society of Pediatric Nephrology.1997;1(1):79-81.

Hepatoblastoma (HB) is a rare embryonic malignant tumor of the liver. Most morphological studies on HB have limited to the histological characteristics and only 3 cases of HB have been described in the cytology literature. We present 2 cases of HB occurring in children aged 1 year and 3 years, respectively. The distinctive cytologic features of fine needle aspiration of HB were clusters of tumor cells showing acinar and trabecular pattern, smaller tumor cells with a high nuclear-cytopalsmic ratio and hyperchromatic nuclei having prominent nucleoli, and the presence of extramedullary hematopoiesis and osteoid material. These features were also found in the cell block and the biopsy specimen, and appeared very useful in the differentiation of HB from hepatocellular carcinoma.
Acute Kidney Injury* ; Biopsy ; Biopsy, Fine-Needle ; Carcinoma, Hepatocellular ; Child* ; Hematopoiesis, Extramedullary ; Hepatoblastoma ; Humans ; Liver ; Nephrosis, Lipoid*

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Threr cases of Hypertensive Encephalopathy in a cute Post-streptococcal Glomerulonephritis: MRI Findings.

Hyoung No KIM ; Sung Min YOON ; Jong Shin KIM ; Chang Youn LEE

Journal of the Korean Society of Pediatric Nephrology.1997;1(1):73-78.

Two cases of giant cell tumor of bone diagnosed by fine needle aspiration cytology are described. Case 1 was a 28-year-old male who had pain sense for one year at the right distal thigh. His radiologic finding revealed a destructive cortical lesion with soft tissue extension at medial side of epiphysis of the distal femur. Case 2 was a 21-year-old female complaining pain at left distal forearm for eight months and showed a well-demarcated expansile osteolytic lesion with multiseptation, and cortical destruction at epiphysis and metaphysis of the left distal radius on the X-ray. Fine needle aspiration of each lesion was performed. The aspirate of the case 1 revealed moderate cellularity, which was composed of scattered giant cells of osteoclastic type and small round to oval monotonous stromal cells in large areas. Giant cells were evenly distributed in single or small groups and had irregular but abundant cytoplasms with 10 to 20 nuclei in the center. The nuclei showed ovoid shape, fine granular chromatin, and a small but conspicuous nucleolus. Stromal cells were dispersed in isolated pattern or sometimes aggregated in clusters and showed the same nuclei as those of giant cells and scanty cytoplasms. Comparing to case 1, case 2 had a more translucent abundant cytoplasm in the giant cells and more spindled stromal cells. All two cases revealed neither nuclear atypism nor increased abnormal mitoses in both giant and stromal cells, suggesting no evidence of malignancy. Thereafter the lesions were treated with excision and curettage, and histologically confirmed as giant cell tumors of the bone.
Adult ; Biopsy, Fine-Needle ; Chromatin ; Curettage ; Cytoplasm ; Epiphyses ; Female ; Femur ; Forearm ; Giant Cell Tumor of Bone ; Giant Cell Tumors ; Giant Cells ; Glomerulonephritis* ; Humans ; Hypertensive Encephalopathy* ; Magnetic Resonance Imaging* ; Male ; Mitosis ; Osteoclasts ; Radius ; Stromal Cells ; Thigh ; Young Adult

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