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Biomedical subjects

Jei-Wen Chang

Publications and source records attributed to Jei-Wen Chang.

6 recordsLinked to original sources

Renal cell carcinoma in children and young adults.

Renal cell carcinoma (RCC) is a relatively uncommon tumor in childhood. Its biologic behavior and prognostic factors have rarely been documented. We report treatment and survival of 4 children (aged 8, 9, 11, and 14 years) who had RCC, along with a review of the literature to analyze the frequency of major symptoms, clinical stage, and prognostic factors based on 130 published cases of RCC in individuals younger than 20 years of age. Two of our cases had renal tumors detected by ultrasound screening, and all 4 cases were followed for a considerable length of time and were alive and free of disease after treatment. An analysis of these 130 published cases of pediatric RCC showed tumor staging and cell type to be the factors that affected patient survival. Tumors composed of granular cells or mixed cells, or at advanced stages, had a poor prognosis. Age, sex, tumor size, symptom duration, and cellular pattern were not related to patient prognosis. Children older than 10 years of age, who have an abdominal mass, flank pain, and/or hematuria should alert clinicians to consider the possibility of RCC. The importance of early diagnosis of renal tumors, using ultrasound as a tool of screening, is emphasized, since surgical treatment leads to a favorable prognosis only in the early stage of RCC.

Adolescent↗

Higher incidence of Dermatophagoides pteronyssinus allergy in children of Taipei city than in children of rural areas.

BACKGROUND AND PURPOSE: House dust mites are the most common cause of sensitization in wheezing children in most parts of the world. The aim of this study was to investigate prevalence of sensitization and the levels of specific immunoglobulin E (IgE) to Dermatophagoides pteronyssinus (Der p) in wheezing children in Taipei city and central rural Taiwan. METHODS: A total of 3546 children were enrolled in this study. Children were grouped into those living in Taipei city (n = 1340) and those residing in the rural part of central Taiwan (2206). The prevalence of sensitization and level of specific IgE antibody to Der p and cockroach were analyzed by age and geographic area. RESULTS: The results showed significantly higher sensitization rates and mean specific IgE levels to Der p with increasing age of patients in both Taipei city and rural central Taiwan. In addition, children from Taipei city had a significantly higher average sensitization rate of Der p than rural children (p<0.05). The proportion of children sensitized to cockroach increased with age both in Taipei city and central rural Taiwan, but the specific IgE levels to cockroach were not statistically different between the 2 groups (p=0.061). CONCLUSIONS: Sensitivity to aeroallergens varies with age and with geographical location (city vs rural) in asthmatic children. Circulating IgE antibodies against Der p were common at all ages. Cockroach allergen is also associated with recurrent wheezing in Taiwan. Avoidance of indoor aeroallergens such as Der p and cockroach allergen should be an important component in plans for the management of recurrent wheezing.

Animals↗

Xanthogranulomatous pyelonephritis treated by partial nephrectomy.

Xanthogranulomatous pyelonephritis is an uncommon disease in children. We report of a 2-month-old girl with urinary tract infection and with a renal mass detected by ultrasound scan. The preoperative differential diagnoses were Wilms tumor, multicystic dysplastic kidney, renal abscess, and mesoplastic nephroma. The subsequent histopathological findings allowed the diagnosis of xanthogranulomatous pyelonephritis (XGP). Although XGP is rare at this age, it must be considered in the differential diagnosis of a child presenting with renal mass, either with or without associated urolithiasis, anemia, and elevated inflammatory markers. The long-standing mainstay of therapy for diffuse XGP has been nephrectomy. However, we report the successful preservation of renal mass with partial nephrectomy for diffuse XGP. The affected kidney grew normally and preserved residual function after the operation. Hence we strongly suggest parenchymal saving in pediatric cases of XGP to preserve renal function.

Antihypertensive Agents↗

Long-term outcome of heavy proteinuria in patients under 2 years of age.

From January 1985 to July 2000, a retrospective study of 53 patients in Taiwan was performed in order to evaluate the underlying diseases causing heavy proteinuria and the clinical outcome in children under 2 years of age (33 boys and 20 girls). Renal biopsy or autopsy was performed in 26 of the children. Renal pathology revealed 2 patients with congenital nephrosis (CNS) (7.7%), 4 with diffuse mesangial sclerosis (DMS) (15.4%), 4 with minimal change nephrotic syndrome (MCNS) (15.4%), 5 with focal segmental glomerulosclerosis (FSGS) (19.2%), 9 with IgM nephropathy in (34.6%), and 2 with hepatitis B virus-associated membranous glomerulonephritis (7.7%). Based on available histology and family history of heavy proteinuria progressing to end-stage renal disease (ESRD), patients were divided into two groups. Group I comprised 10 patients, including CNS (2 cases), DMS (4 cases), and 4 children with a familial history of heavy proteinuria progressing to ESRD. All patients in group I were initially steroid resistant. After methylprednisolone pulse therapy plus cyclosporin A treatment, no patients with CNS or DMS responded, but the other 4 patients experienced a remission. Group II comprised 43 patients; 19 patients (44.2%) were initially steroid resistant. Of these steroid-resistant patients, all experienced remission after methylprednisolone pulse therapy plus cyclosporin A, except 3 children with FSGS. One experienced a thromboembolic event during his clinical course. In conclusion, steroid-resistant nephrotic syndrome (NS) was more common than steroid-sensitive NS in Chinese patients under 2 years of age. Patients with CNS, DMS, or a family history of heavy proteinuria progressing to ESRD had a poor prognosis. Methylprednisolone pulse therapy plus cyclosporin A treatment achieved remission in some children who were initially steroid resistant. This study indicates that children with conditions associated with poor steroid responsiveness (e.g., CNS, DMS) do not respond to immunosuppressive therapy, but other children under 2 years of age, including those with a family history of progression to ESRD, may benefit from aggressive immunosuppressive therapy.

Child, Preschool↗

Colon mucosal pathology in infants under three months of age with diarrhea disorders.

BACKGROUND: Diarrhea is a common disease in infants. It may result in catastrophic events, especially in early infancy. Sometimes it is difficult to point out the diagnosis after initial examination. Sigmoidoscopy with mucosal biopsy may help in assessing the nature of the disease. The goals of this study are to evaluate the colon mucosal pathology in young infants with unknown diarrhea disorders and the clinical courses of different etiologies. METHODS: We performed a retrospective analysis of 64 young infants (Mean +/- SD: 1.68 +/- 1.01 months) in whom a diagnosis of unknown diarrhea disorder had been made after an initial investigation. All infants received fiberoptic sigmoidoscopy and mucosal biopsy with consent obtained. RESULTS: The appearance of the colon mucosa under endoscopy were: 36 (56.2%) showing edema, 30 (46.8%) showing nodularity, 23 (35.9%) patients with focal erythema and friable mucosa, 11 (17.1%) showed erosion, 7 (10.9%) patients showing hemorrhage, and negative finding in one patient (1.6%). Colon mucosa pathologic findings were established: 40 (62.5%) had eosinophilic colitis (EC) and 19 (29.7%) had nonspecific colitis. The biopsies were normal in 5 cases; 81.5%(22/27) of patients with EC showed eosinophils in the stool smear which was significantly higher ( = 0.04) compared to the group II (9/17); 34 of 40 EC patients responded rapidly to changes in formula. CONCLUSION: Sigmoidoscopy and mucosal biopsy may be useful in the pathologic diagnosis of unknown diarrhea disorders. Eosinophilic colitis accounts for over half of very young infants with diarrhea disorders suggesting for allergic etiology. Patients with allergic colitis may exhibit a dramatic response to elimination of the offending proteins from the diet.

Biopsy↗

Congenital nephrotic syndrome: report of an infant with diffuse mesangial sclerosis.

A case of congenital nephrotic syndrome (CNS) caused by diffuse mesangial sclerosis (DMS) is presented. A female baby weighting 2,680 gm was delivered at 35 weeks' gestation. She had early onset of generalized edema, heavy proteinuria, oligouria, uncorrectable hypoalbuminemia, and rapid progression to renal failure. Even after being treated with strong antibiotics (teicoplanin and ceftazidine), the infant died of septic shock with Enterobacter cloacea, only sensitive to imipenem, at the age of 7 days. The necropsy showed diffuse mesangial sclerosis. This case demonstrates that prematurity, low birth body weight and early onset of symptoms are not pathognomonic of the congenital nephrotic syndrome of the Finnish type (CNF). It can also occur in DMS. Besides, empirical antibiotics should be started promptly and should cover the major hospital strains of bacteria if the patient is not well.

Female↗