The Role of Aldosterone on the Development of Renal Tubular Reabsorption in Low Birth Weight Infants

PURPOSE: To determine the postnatal changes in aldosterone action on the renal tubular reabsorption in low birth weight(LBW) infants, we assessed the relation of the aldosterone concentrations to renal parameters during the first 10 days of life. METHODS: Twenty LBW infants were evaluated and their gestational ages ranged from 32.4 to 39.3 weeks and their birth weights ranged from 1,440 to 2,500 g. Estimated glomerular filtration rate, fractional excretion of sodium(FENa) and potassium(FEK), and plasma aldosterone concentrations were analyzed according to the postnatal age and the conceptional age(CA). RESULTS: Glomerular functions were improved after birth and were correlated with CA. FENa and FEK decreased after birth and correlated with CA. Plasma aldosterone concentrations increased to 318.6 +/- 147.2 ng/dL at 48 hours and then decreased to 162.0 +/- 72.2 ng/dL at 10 days after birth. Plasma aldosterone concentrations of infants less than 38th week of CA were higher than that of infants more than 38th week. There was a significant negative correlation coefficient between plasma aldosterone concentrations and FENa in infants more than 34th week of CA, but not in that of less than 34th week. CONCLUSIONS: LBW infants have higher plasma aldosterone concentrations, but a poor correlation between plasma aldosterone concentration and urinary sodium excretion for the first few days of life and in lower chronologic aged infants. These results show that the renal tubule reabsorption of sodium is less responsive to plasma aldosterone in these infants and, therefore, the careful management of fluid and electrolyte balance is mandatory.

Long-term Cyclosporine A(Cypol(R)) Therapy in Children with Idiopathic Nephrotic Syndrome / 대한신장학회잡지

This multicenter collaboratory study was conducted to find out the long-term therapeutic efficacy and side effect of cyclosporine A(Cypol(R), Chong Kun Dang) on children with idiopathic nephrotic syndrome who experienced frequently relapsing(FR), steroid dependent(SD), or steroid resistant(SR) pattern. Forty-six children with SD/FR NS and 5 children with SR NS were enrolled in this study. After induction of remission(SD/FR NS) with steroid or after 4 weeks of steroid therapy(SR NS), cyclosporine A was started in a dose of 4-5mg/kg/day in two divided dose and steroid(prednisolone or equivalent dose of deflazacort) was tapered slowly. During 12 months of study period, monthly check up of physical examination and various laboratory tests including BUN, creatinine, Ccr and cyclosporine blood level were done. Out of 46 children with SD/FR NS, 28(60.9%) maintained sustained remission, 16(34.8%) showed 1 or 2 relapses during therapy and 2(4.3%) cases showed no response. At 4 weeks after therapy, values of serum protein, albumin, cholesterol, and 24 hours urinary protein excretion showed normal values. Four out of 5 children with SR NS showed complete or partial remission with cyclosporine A therapy and one child showed no response. Side reaction to cyclosporine A therapy showed hypertricosis in 14 cases, hyperuricemia in 8 cases and hypomagnesemia in 16 cases. However, other laboratory tests including CBC, liver profile, BUN, creatinine and GFR(creatinine clearance utilizing 24 hour urine) did not show any abnormalities during the 12 months of study period. We performed follow-up renal biopsy in 17 children after 12 months cyclosporine A treatment. Eight cases(47.1%) showed mild cyclosporine A nephrotoxicity like interstitial fibrosis and tubular atropy. In conclusion, present study shows that cyclosporine A(Cypol(R), Chong Kun Dang) can be used quitely effectively in maintaining remission and decreasing relapse rate on children with SD/FR or SR NS. However, because administration of cyclosporine A for 12 months is found to be associated with nephrotoxicity in a significant number of patients, we are planning further study using "smaller dosage" of cyclosporine A to reduce its nephrotoxic effect and for longer period of treatment(over 2 years).

Analysis of Risk Factors Affecting the Pediatric Renal Allograft Survival in Cyclosporine Era: A Single Center Study Based on 113 Renal Transplants / 대한이식학회지

Renal transplantation is now a well established mode of optimal therapy for children with end-stage renal disease. A total of 119 pediatric renal transplantations were performed during last 20 years but 6 cases (early 3 cases treated with azathioprine and most recent 3 cases) were excluded for this study. A total of 113 pediatric renal transplants out of total 1,906 kidney transplantation recipients receiving cyclosporine A and low dose prednisone as the main immunosuppressive agent were the subjects of this study to find out the risk factors which might influence the pediatric renal allograft survival in a single center. When the potential donor was living related, at least the HLA 1-haplotype matched relative was selected, but, when unrelated, at least DR-1/2 or A+B 2/4 matching was required for selection. Living related donation from parent, brothers, sisters (n=82), and unrelated donation (n=31) through the swap program or from fully motivated healthy volunteers were the major source of kidney for allograft. The mean age of the recipient was 14.1 years ranging from ages 2.1 to 19.9. During a mean follow-up of 68.1 months, there were 21 cases of graft loss, and 3 recipient deaths. The major causes of graft loss were acute and/or chronic rejection, poor compliance and patients death. The 1-, 3- and 5-year graft survival were 94.6%, 88.9% and 79.2% respectively. There was no significant difference between children and adult in graft survival rate. No significant graft survival difference between the related and unrelated donors (73.3 vs 77.2% at 5-year, p>0.05) was found. The significant risk factors for the outcome were the ABO compatibility (p=0.0001) and development of more than 1 episode of acute rejection within 6 month (p=0.01) and 1 year (p=0.0016). Graft survival decreased with increasing number of rejection episode within 6 month (p=0.009) and 1 year (p=0.002). Other factors such as recipients age, original kidney diseases, type and duration of dialysis before transplantation, combined native kidney removals did not influence the outcome of graft. And because of presence of only 2 cadaveric donor in this analysis, we could not demonstrate any benefit of living donor transplantation. In conclusion, pediatric renal transplantation in at least older children (>5 years) is encouraging. The outcome of pre- emptive renal transplantation is also promising. More aggressive ABO matching and effort for reducing the rejection episode within 6 months and 1 year might be important factors for the successful outcome of pediatric renal transplantation. So development and application of more effective immunosuppressive agents such as mycophenolate mofetil or rapamycin to reduce the rejection episodes is to be needed in near future.

Clinical Analysis of Children with Transitory Minimal Change Nehrotic Syndrome ( MCNS ) to Focal Segmental Glomerulosclerosis ( FSGS )

Mixed squamous cell carcinoma and papillary carcinoma in the thyroid gland is a very rare malignant tumor characterized by rapidly progressive clinical course and radioresistance. A 63-year-oid woman had mixed squamous cell carcinoma and papillary carcinoma in the thyroid gland diagnosed by fine needle aspiration cytology (FNA), and the diagnosis was confirmed by histological examination. She had complained of a fixed, egg-sized mass of the anterior neck with hoarseness for 1 year. The findings of FNA consisted of sheets and clusters of polygonal epithelial cells with hyperchromatic, pleomorphic nuclei and eosinophilic, abundant, laminated cytoplasm. These findings were consistent with squamous cell carcinoma. Also, foci of papillary carcinoma were noted, and the cells exhibited nuclear groovings and intranuclear cytoplasmic inclusions. Total thyroidectomy specimen showed a diffusely infiltrating tumor in the left thyroid which was composed of mixed papillary carcinoma and well-differentiated squamous cell carcinoma. In junction between two components, squamous metaplasia of papillary carcinoma was noted.

MHC Class II Allele Association in Korean Children With IgA Aephropathy an its Pol as a Prognostic Factor

Diagnosis of a thymic carcinoid was made on transthoracic fine needle aspiration in a 36-year old woman who had an anterior mediastinal mass on chest X-ray and CT scan. The aspiration smears showed numerous anastomosing ribbons and cords of small round tumor cells. The tumor cells had slightly eccentric nuclei and some granular cytoplasm. The small and uniform nuclei of the tumor cells had finely granular chromatin and thin nuclear membrane. The cytologic diagnosis of a carcinoid was confirmed on histopathologic, immunohistochemical, and electromicroscopic examination of surgical specimen.

Analysis of Children with Unilateral Multicystic Dysplastic Kidney ( MCDK )

In a six-year period(1988. 5-1994. 4), fine needle aspiration cytology(FNAC) of 322 pulmonary lesions from 296 patients were performed at Soonchunhyang University Hospital. Of these 322, malignancy was diagnosed cytologically in 139(43.2%), suspicious malignancy in 7(2.2%), negative in 164(50.8%), and insufficient material in 12(3.8%). Malignant lesions consisted of 54 cases of adenocarcinoma, 50 cases of squamous cell carcinoma, 18 cases of small cell carcinoma. They were verified by histologic examination in 70 cases. There were 2(0.6%) false positive cases due to florid bronchoalveolar hyperplasia and atypical bronchial epithelial cells associated with granulomatous lesion. The overall accuracy rate was 90%, the sensitivity 84.3% and the specificity 94.7%.

Risk Factors of Primary Nocturnal Enuresis

Urinary cytology has become an essential element in the diagnosis and management of transitional cell carcinoma(TCC) of the urinary tract. It has the advantage of being noninvasive, inexpensive, and easily accessible. Besides that it can even detect malignancy when unsuspected at cystoscopy. We report a retrospective review of urine cytology in the diagnosis of 83 TCC cases that underwent 295 cytologic evaluation. All patients had biopsy-proven TCC of the bladder, ureter and renal pelvis. The overall inci- dence of the positive cytology cases was 66.2%. To define the cytologic features of tumor cells, we tried to use three cytologic gradings such as "grade 1", "grade 2", and "grade 3" according to the cytologic degree of anaplastic neoplastic cells. These cytologic gades of TCC were relatively well correlated with the histologic grade and tumor invasiveness. This result suggests that the recognition of characteristic cellular features of TCC can suspect the histologic grade and tumor stage. The false negative TCC cases were 78.9%. They showed severe inflammatory or bloody background and a few neoplastic cells. Therefore, a cautious approach for accurate interpretation, personal experience, and proper fixation and processing could expand the role of urinary cytology.

Current Status of Children Born from Renal Transplanted Mother

Fine needle aspiration of the breast is an important diagnostic tool in malignant lesions, but is also useful in differentiation of inflammatory breast diseases mimicking carcinoma clinically and radiologically. Recently, the authors have experienced eight biopsy-proven cases of chronic inflammatory diseases of the breast, which consisted of 4 cases of duct ectasia, 2 cases of fat necrosis, and a case of tuberculous mastitis and granulomatous mastitis respectively, Their cytoiogic features mainly based on the components and the relative frequency of inflammatory cells were evaluated for differential diagnosis of chronic inflammatory breast diseases. The results are as follows ; 1. In cases of duct ectasia, varying amount of neutrophils, mononuclear leukocytes, histiocytes and multinucleated giant cells were intermixed with benign epithelial cell clusters. 2, Abundant fat tissue fragments were diagnostic for fat necrosis. Histiocytes and mononuclear cells were main components but not rich, and neutrophils and giant cells were infrequently observed. 3. Characteristic granulomas composed of epithelioid cells, mononuclear leukocytes and Langhans' type giant cells and lymphocytic infiltrates were conspicuous in tuberculous mastitis, and occasionally neutrophils, necrotic materials and epithelial cell clusters were found. 4. In granulomatous mastitis, epithelioid cell granulomas were also noted but numerous neutrophils and histiocytes were intermingled within or outside the granulomas.

Clinical Study on Hemolytic Uremic Syndrome in Children: Review of 23 Cases

PURPOSE: The hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. It is one of the most common cause of acute renal failure in children but few reports are available in Korea. Thus we investigated the 23 patients diagnosed as HUS during last 14 years. METHOD: We retrospectively investigated the etiologic factor, clinical manifestations, laboratory findings, treatment modalities, and final outcomes of the patients. Then patients were divided into two groups according to outcome, and comparison was performed. Group A(8) comprised patients who progressed to end-stage renal disease or expired. Group B(15) comprised patients who completely recovered after dialysis treatment. RESULT: The number of patients aged less than 4 years were 17; between 5 and 10 were 4 and more than 10 were 2. The gende ratio was M:F=2 : 1. The etiologic factors were as follows: acute gastroenteritis in 14 patients including 4 bloody diarrhea, upper respiratory tract infection in 7 patients, and 1 patient with herbal medication. The overall mortality rate was 22%: 2 patients died of CNS complications, 2 patients died of sepsis, and 1 patient died of pulmonary hemorrhage. Group A (Hb 4.8+/-1.2 g/dL) showed lower value in hemoglobin than group B (Hb 6.3+/-1.7 g/dL) during hospital stay (p<0.05), And the time interval between the disease onset and dialysis treatment was significantly longer in group A (11.9+/-9.1 days vs 2.8+/-2.1 days) (p<0.05). CONCLUSION: Overall mortality rate was 22%. Low hemoglobin value and the prolonged time interval between the disease onset and dialysis treatment were related with poor prognosis. So early diagnosis and appropriate intensive care including dialysis treatment is essential to achieve better outcome in children.

A Clinical Study of Acute Poststreptococcal Glomerulonephritis with Nephrotic Syndrome

The diagnosis of peripheral T cell lymphoma is difficult due to the varying size and shape of the neoplastic lymphoid cells and the frequent admixture of nonneoplastic mature lymphyocytes, histiocytes, eosinophils, and plasma cells. We report a case of peripheral T cell lymphoma, lymphoepithelioid cell type, which was difficult to differentiate from tuberculous lymphadenitis due to the aggregates of epithelioid histiocytes mimicking granuloma and the past history of pulmonary tuberculosis. Fine needle aspiration cytology of the inguinal lymph node in a 63-year-old male was characterized by hypercellular aspirates composed of a mixture of small and intermediate-size lymphoid cells and large lymphoid cells with background of confluent epithelioid histiocytes. The neoplastic lymphocytes demonstrated significant nuclear irregularity with protrusion and indentations of the nuclear membrane, prominent nucleoli, and frequent mitotic figures. The diagnosis of peripheral T cell lymphoma was confirmed by histological and immunohistochemical studies.

Clinical Analysis of Ectopic Kidney in Children

The diagnosis of carcinoma in situ of urinary bladder is difficult in that the symptoms and cystoscopic findings are nonspecific. The cytology of urine could be helpful for diagnosis of carcinoma in situ of urinary bladder. We present a case of bladder washing cytology of carcinoma in situ. A 54-year-old man presented with dysuria for 1 year. Cystoscopic findings revealed multifocal reddish trabeculated lesions. The bladder washing cytology revealed rather uniform tumor cells which were singly scattered or forming syncytium in the clean background. The nuclei were round to oval with inconspicious nucleoli. The cystoscopic biopsy revealed typical histologic features of carcinoma in situ of urinary bladder.

A Case of Unilaterally Involved Glomerulocystic Kidney Disease

Metastatic tumors occur more frequently in the liver than in any other organ, Guided percutaneous fine-needle aspiration (FNA) of the liver is often recommended for confirmative diagnosis of the metastatic lesion, because of its simplicity, high yield, and reasonable safety. The authors studied retrospectively cytologic findings of 110 cases of metastatic tumors to the liver. The frequent primary sites were the stomach (23 cases), pancreas (19 cases), gallbladder (12 cases), and periampullary lesions (6 cases). Most of the metastases were carcinoma (106 cases). There were only 4 cases of sarcoma. The characteristic cytologic findings of FNA of meatastatic tumors were dirty background, abrupt change between hepatocytes and malignant cells, and desmoplasia. Some tumors displayed rather distinctive cytologic appearance that suggests primary sites. For example, the colonic adenocarcinoma showed tall columnar cells with a palisading arrangement, adenocarcinoma of gallbaldder showed focal squamous differentiation in some cases, and metastatic renal cell carcinoma and neuroblastoma showed also distinctive cytologic findings. Because the cytologic features of metastatic tumor are very similar to those of primary tumor, correct cytologic typing may be helpful in pursuit of an occult primary site of metastatic liver lesions, reducing extensive diagnostic investigation in poor prognostic patients.

A Case of Crescentic Glomerulonephritis Superimposed on Membranous Glomerulonephropathy

Cytologic findings of pleural effusion in three cases of rhabdomyosarcoma are reported. Case 1 was a pleomorphic rhabdomyosarcoma which had devoped at the chest wall of an elderly male patient and caused pleural effusion. The cytologic features were consistent with pleomorphic rhabdomyosarcoma, that was, showing loose clusters, cellular pleomorphism, and abundant finely vesicular cytoplasm. Cases 2 and 3 were embryonal rhabdomyosarcomas in young adults. Primary site was the oral cavity in case 1, but unknown in case 2 and case 3. The effusion cytology was similar in these cases. Clustered or isolated small round cells with hyperchromatic nuclei and scanty cytoplasm were smeared. The cohesiveness of tumor cells was weak and the cells did not show linear arrangement or nuclear molding. Effusion cytology in a sarcoma patient would be diagnostic when the primary site and the type of sarcoma were already known.

Clinical Findings and Therapy of Ureteral Duplication in 61 Children

Osteoclast-like giant cell tumor of the liver is an extremely rare malignancy with poor prognosis. To our knowledge, 5 cases have been reported in English literatures, but there was no report about fine needle aspiration cytologic(FNAC) features. We experienced a case of osteoclast-like giant cell tumor of the liver obtained by computed tomography(CT)-guided FNAC and needle biopsy. The cytologic findings mimicked giant cell tumor of the bone. A large hepatic mass of the left lobe with abdominal wall invasion was found by CT in a 46- year-old female complaining of epigastric pain. The FNAC showed moderately cellular smears consisting of osteoclast-like giant cells and mononuclear cells, which were individually scattered or intermingled in clusters. The osteoclast-like giant cells had abundant cytoplasms and multiple small round nuclei with fine chromatin and distinct nucleoli. The mononuclear cells had moderate amount of cytoplasm and relatively bland-looking oval nuclei with single small nucleoli. All of the cytologic features recapitulated the histologic findings of bland-looking osteoclast-like multinucleated giant cells evenly dispersed throughout the background of mononuclear cell. The immunohistochemical study showed positive reaction for CD68 and vimentin, but negative for cytokeratin in both osteoclast-like giant cells and mononuclear cells.