Outcome of isolated antenatal hydronephrosis: a prospective cohort study

The purpose of this study was to report the outcome of infants with antenatal hydronephrosis. Between May 1999 and June 2006, all patients diagnosed with isolated fetal renal pelvic dilatation (RPD) were prospectively followed. The events of interest were: presence of uropathy, need for surgical intervention, RPD resolution, urinary tract infection (UTI), and hypertension. RPD was classified as mild (5-9.9 mm), moderate (10-14.9 mm) or severe (>or=15 mm). A total of 192 patients was included in the analysis; 114 were assigned to the group of non-significant findings (59.4%) and 78 to the group of significant uropathy (40.6%). Of 89 patients with mild dilatation, 16 (18%) presented uropathy. Median follow-up time was 24 months. Twenty-seven patients (15%) required surgical intervention. During follow-up, UTI occurred in 27 (14%) children. Of 89 patients with mild dilatation, seven (7.8%) presented UTI during follow-up. Renal function, blood pressure, and somatic growth were within normal range at last visit. The majority of patients with mild fetal RPD have no significant findings during infancy. Nevertheless, our prospective study has shown that 18% of these patients presented uropathy and 7.8% had UTI during a medium-term follow-up time. Our findings suggested that, in contrast to patients with moderate/severe RPD, infants with mild RPD do not require invasive diagnostic procedures but need strict clinical surveillance for UTI and progression of RPD.     

Postnatal management of infants with antenatally detected hydronephrosis

With the increasing use of antenatal sonography, fetal hydronephrosis has been reported more frequently. Because of the lack of consensus regarding treatment of these infants, the postnatal approach toward fetal renal pelvis enlargement remains controversial. The aim of this prospective study is to demonstrate the postnatal investigation, treatment, and outcome of infants with prenatally diagnosed hydronephrosis. Infants whose antenatal ultrasound scan showed a fetal renal pelvis of 5 mm or greater were investigated postnatally using ultrasound (US) and voiding cystourethrography. When indicated, isotope studies and intravenous urograms were also performed. We followed prospectively neonates with antenatally diagnosed hydronephrosis and recommended management guidelines on the basis of our findings. In 156 neonates (193 kidney units) that were found to have hydronephrosis, the average gestational age at which the diagnosis was made was 32.94±5.10 weeks. The mean duration of postnatal follow-up was 26.3±13.56 months (range 3–60 months). The mean APPD of the fetal renal pelvis was 10.35±3.24 mm (5–9 mm in 84 kidneys, 10–14 mm in 96 kidneys and 15 mm in 13 kidneys). Of the 193 kidney units, 145 units were found to be pathological. The most common detected underlying abnormalities were ureteropelvic junction obstruction (in 91 kidneys; 62.7%) and vesicoureteral reflux (in 24 kidneys; 16.6%). Postnatally, 23 (45%) of 51 patients whose first US was normal were diagnosed postnatally as having urinary tract abnormality. There was a negative correlation between APPD and the rate of spontaneous resolution and positive correlation between APPD and the rate of surgery (P<0.01). In conclusion, because it is not possible to determine an upper limit of normal for the antenatal renal pelvis, any baby with AH should not be considered clinically insignificant. Infants with antenatal renal pelvis measurements 5 mm should be investigated postnatally. A normal postnatal ultrasound scan does not preclude the presence of urinary tract abnormality.     

Development of a fetal renal function test using endogenous creatinine clearance

Selecting appropriate management for the fetus with obstructive uropathy depends on our ability to accurately assess the severity of existing renal damage and to predict the potential for recovery of renal function if the obstruction is relieved. Diagnosis and treatment would be markedly enhanced by a simple, safe, quantitative fetal renal function test. To answer the question of whether endogenous fetal creatinine clearance (CrC) is an accurate measure of glomerular filtration rate (GFR) in the obstructed fetal kidney, we compared fetal CrC to a standard test for GFR--iothalamate clearance (IC). Six fetal lambs underwent unilateral ureteral ligation at 60 to 63 days gestation (term = 145 days). The contralateral unobstructed kidneys served as a control. At a second operation at 113 to 120 days, renal function was measured by hourly split urine collections for determination of CrC, IC, and fractional sodium excretion on each side over a 4-hr study period. There was excellent correlation of CrC with IC in all kidneys (r = 0.997, P less than 0.001, y = 1.14 x). Compared to the control side, the obstructed fetal kidneys had significantly decreased GFR and abnormal tubular function with marked sodium loss.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fluctuating fetal or neonatal renal pelvis: marker of high-grade vesicoureteral reflux

Fetal renal pelvic dilatation is a predictor of vesicoureteral reflux (VUR), but has low specificity. The aim of this study was to determine the clinical significance of fluctuating size of the renal pelvis on sonography, particularly in relationship to VUR. We prospectively recorded fetal renal pelvic diameter 4 mm in 1,092 fetuses (692 boys), from May 1989 to December 1995, with a minimum follow-up of 7 years. Fluctuation, defined as size of renal pelvis changing by more than 4 mm during the course of obstetric (23), postnatal (128), and both pre- and postnatal (7) renal sonograms, was recorded prospectively in 159 (117 boys). Of the 1,092, 849 (593 boys) had a voiding cystourethrogram at a mean age of 7 weeks (range 3–20 weeks). Of the 31 (16 girls) with high-grade VUR, fluctuation was observed in 20 (9 girls). Fluctuation was strongly associated with high-grade VUR (odds ratio 11.1, P=0.0000003) and with renal damage (sensitivity 61%, positive predictive value 31%). Primary high-grade VUR was persistent, seen equally in boys and girls, and required surgery in the majority. Fluctuation was associated with renal duplication anomalies (P=0.00009) and megaureter (P<0.00000001). Fluctuation of the renal pelvis on sonography is a marker for persistent high-grade VUR and renal damage in girls and boys.     

Comparing characteristics and outcomes in infants with prenatal and postnatal diagnosis of esophageal atresia

Background

Previous studies of infants with esophageal atresia (EA) suggest those diagnosed prenatally have worse outcomes because of a higher incidence of associated anomalies. The purpose of this study was to compare characteristics and outcomes of infants with EA diagnosed after fetal center evaluation to those diagnosed postnatally.

Methods

The records of all neonates treated for EA at our institution from 2002–2012 were reviewed. Infants with a prenatal diagnosis of EA were compared with those postnatally diagnosed using chi-square and Student t-test as appropriate.

Results

Of 91 patients treated with EA during the study period, 15 (16%) were diagnosed prenatally at our fetal center. Although those prenatally diagnosed had a higher incidence of pure EA and polyhydramnios, the gestational age and birth weight in that group were similar to those diagnosed postnatally. There were no differences in outcomes between groups with regard to the incidence of major cardiac anomalies, surgical complications, hospital length of stay, and survival.

Conclusions

Treatment at a tertiary care center provides excellent outcomes for all infants with EA, despite an 80% frequency of concurrent anomalies. Prenatal diagnosis of EA and attentive obstetric management of polyhydramnios decrease the risk for prematurity and prematurity-associated morbidity.     

Congenital high airway obstruction syndrome (CHAOS): Natural history,prenatal management strategies,and outcomes at a single comprehensive fetal center

PurposeCongenital high airway obstruction syndrome (CHAOS) is a devastating fetal condition of complete airway discontinuity resulting in significant hydrops and extreme lung hyperplasia. It is universally fatal with survival reported only in the rare spontaneous fistulization or EXIT intervention (Ex Utero Intrapartum Treatment). Even in these cases, mortality remains high, and current investigations are targeting prenatal interventions. This report describes our experience with management and fetal interventions for CHAOS, including laser laryngotomy.MethodsWe retrospectively reviewed all patients diagnosed with CHAOS at a single academic institution between 2006 and 2017.ResultsFifteen patients were identified. Eight had obstruction at the trachea and seven at the larynx. In the laryngeal obstructions, three expired shortly after birth, and one survived after spontaneous fistulization and subsequent EXIT to tracheostomy. The remaining three underwent in-utero treatment with laser laryngotomy. One had preterm premature rupture of membranes (PPROM), delivered 3 days post-operatively, and died. Two underwent EXIT to tracheostomy with one surviving to discharge and is currently 2 years old.ConclusionOur study demonstrates the outcomes of a large series of patients diagnosed with CHAOS. While mortality remains high, options for fetal intervention are being explored to allow alterations in the prenatal natural history and improve postnatal outcomes.Type of StudyRetrospective Treatment Study.Level of EvidenceLevel IV.     

Renal lymphangiomatosis: Imaging and management of a rare renal anomaly

Renal lymphangiomatosis is a rare developmental malformation resulting in the development of cystic masses in the peri-pelvic or peri-renal areas. We report our experience in the management of this rare anomaly. Herein, we also describe the successful laparoscopic ablation of an uncommon subcapsular variant of renal lymphangiomatosis, which failed percutaneous drainage.     

Fetal ovarian cysts management and ovarian prognosis: a report of 82 cases

Background/Purpose

Fetal ovarian cysts are frequently complicated by intracystic hemorrhage without associated clinical signs, which is often secondary to ovarian torsion leading to loss of the ovary. The aim of this study was to evaluate ovarian outcome and the place of prenatal management and surgery in the first few days of life in order to save the ovary.

Methods

Between January 1987 and June 2006, 82 fetal ovarian cysts in 79 patients were managed and clinically and ultrasonographically followed up for several months (median, 11 months; range, 6 months to 10 years) in all of the cases where the ovary was not removed. The ultrasonographic results regarding the ovarian parenchyma were broken down into 3 categories: follicular ovary, homogeneous ovary, and undetected ovary.

Results

Twenty-seven cysts remained simple throughout their evolution, and 55 were complicated by intracystic hemorrhage usually several weeks before birth. Overall, after disappearance of the cyst, a follicular ovary was detected in only 39% of the cases (32/82) and more often when the cyst was simple than when it presented an intracystic hemorrhage (85% vs 16.4%, χ², P < .0001).

Conclusions

A review of our series confirms the poor ovarian outcome linked to ultrasonographic signs of intracystic hemorrhage. Preventive action by puncture of “simple” cysts is still being studied. The presence of a bilateral cyst can, if pulmonary maturity has been reached, be an argument for inducement of premature birth with a view to performing conservative surgery. After birth, surgery in the first few days of life is only justified if the signs of intracystic hemorrhage appeared in the period very close to birth.     

Procalcitonin as a predictor of renal scarring in infants and young children

The aim of this study was to evaluate the usefulness of procalcitonin (PCT) as a marker of renal scars in infants and young children with a first episode of acute pyelonephritis. Children aged 7 days to 36 months admitted for first febrile urinary tract infection (UTI) to a pediatric emergency department were prospectively enrolled. The PCT concentration was determined at admission. Acute ^99mTc-dimercaptosuccinic acid (DMSA) scintigraphy was performed within 7 days of admission and repeated 12 months later when abnormal findings were obtained on the first scan. Of the 72 children enrolled in the study, 52 showed signs of acute pyelonephritis (APN) on the first DMSA scan. A follow-up scintigraphy at the 12-month follow-up performed on 41 patients revealed that 14 (34%) patients had developed renal scars; these patients also presented significantly higher PCT values than those without permanent renal lesions [2.3 (interquartile range 1–11.6) vs. 0.5 (0.2–1.4) ng/mL; p = 0.007]. A comparison of the PCT concentration in patients with febrile UTI without renal involvement, with APN without scar development and with APN with subsequent renal scarring revealed a significant increasing trend (p = 0.006, Kruskal–Wallis test). The area under the ROC curve for scar prediction was 0.74 (95% confidence interval 0.61–0.85), with an optimum statistical cut-off value of 1 ng/mL (sensitivity 78.6%; specificity 63.8%). Based on these results, we suggest that serum PCT concentration at admission is a useful predictive tool of renal scarring in infants and young children with acute pyelonephritis.     

Primary vesicoureteric reflux and renal damage in the first year of life

We retrospectively examined 93 children (47M/46F) with primary vesicoureteric reflux (VUR) followed for a mean period of 3.5 years. They were divided into two groups. Group A included 34 babies (25M/9F) with a prenatal diagnosis of pelvic dilatation. Mean age at presentation was 12 days and no urinary tract infection (UTI) occurred before our first examination. VUR was unilateral in 21 (62%) patients and bilateral in 13 (38%). It was mild (grades I–III) in 12 (25%) refluxing renal units (RRU) and severe (grades IV–V) in 35 (75%). Renal damage (RD) was present, at diagnosis, in 40 (85%) RRU. There was a greater prevalence of abnormal kidneys in male units (88%) than in female units (75%). Group B included 59 infants (22M/37F) less than 1 year old with UTI. The mean age at first examination was 7.6 months. VUR was unilateral in 32 (54%) infants and bilateral in 27 (46%), mild in 60 (70%) RRU and severe in 26 (30%). At diagnosis, 54 (63%) RRU presented RD, which was more common in females (66%) than in males (44%). Our study confirms that primary VUR associated with prenatal hydronephrosis usually affects males and is severe. VUR diagnosed after UTI, instead, is more common in females and is frequently mild. Although in the first type of reflux RD is often present at diagnosis, then probably congenital, it may always progress after UTI; hence the importance of early diagnosis and careful follow-up in each infant with primary VUR. Received: 9 August 1999 / Revised: 3 April 2000 / Accepted: 7 July 2000     

The etiology of renal scars in infants with pyelonephritis and vesicoureteral reflux

We aimed to investigate, by means of dimercaptosuccinic acid (DMSA) scan, the relations between vesicoureteral reflux (VUR) and its degree, pyelonephritis during infancy, and renal parenchymal findings. Seventy-four infants with pyelonephritis, 44 girls and 30 boys (mean age at their first pyelonephritic episode 4.12 months, median 3 months), were enrolled in the study. Voiding cystourethrography (VCU) and ultrasonography (US) were performed within 6 weeks following the infection. DMSA was performed at least 4 months after the urinary tract infection (UTI). The renal parenchymal pathology was defined as focal or multifocal defects or as a split renal uptake of less than 45%. DMSA scintigraphy revealed that 19% (14/74) of the children had renal damage. Renal parenchymal findings were observed only when VUR was present, and its grade was above 3/5. No abnormality was found in 51 renal units without reflux, 9 with VUR grade 1/5, and 54 with grade 2/5. Renal pathology was observed in 9/24 renal units with VUR grade 3, 3/8 with grade 4, and 2/2 with grade 5. No correlation was found between renal parenchymal defects and clinical presentation of the pyelonephritis, type of the microorganism, presence of bacteremia, or the number of recurrent infections. In adequately treated infants, renal damage is probably due to a reflux-associated, preexisting, congenital renal parenchymal pathology and not to the inflammatory process. We suggest that DMSA scintigraphy should not be performed routinely in every infant with UTI and should be reserved primarily for children with VUR grade 3 and above. Received: 17 February 1999 / Revised: 30 June 1999 / Accepted: 7 July 1999     

Current status of negative treatment decision-making for fetuses with a prenatal diagnosis of neonatal surgical disease at a single Japanese institution

Background/Purpose

The termination of pregnancy because of fetal abnormalities in Japan has not been described. The aim of the present study was to analyze the current status and to evaluate the medical and ethical relevance in our institution for negative treatment decision-making for fetuses demonstrating neonatal surgical disease with a prenatal diagnosis.

Materials and methods

The medical records of 209 fetuses with a prenatal diagnosis from 1999 to 2008 were retrospectively reviewed. The cases with a negative treatment policy were analyzed according to the potential for survival. The negative treatment policies were defined as those in which the pregnancy was not actively continued, including elective termination of pregnancy and palliative or limited treatment that are primarily provided after birth.

Results

The selected treatment policies were active in 162 cases and negative in 46 cases. Thirty-three cases with negative policies were in the second-half period of pregnancy. The potential for survival was high in 5 cases, moderate in 11 cases, and nonviable in 30 cases. Eight of the nonviable cases underwent either limited or palliative treatment, whereas the remaining 38 fetuses were aborted.

Conclusions

The negative treatment policies in the nonviable fetuses were considered to be medically and ethically relevant. However, the number of cases with negative policies increased over the last 5 years and is therefore associated with complex ethical issues.     

New renal scars in children with severe VUR: a 10-year study of randomized treatment

The International Reflux Study in Children was set up to compare prospectively the outcome of medical or surgical management of children with grade III or IV vesicoureteral reflux and a history of symptomatic urinary tract infection. Development of new radiological scars was the main end point. Of the 306 children randomized, 302 (153 medical, 149 surgical) were available for radiological follow-up at 5 years. New scars had developed in 19 medically and 21 surgically treated children. Among 223 patients (113 medical, 110 surgical) who continued follow-up with urography at 10 years, only 2 further new scars developed. Overall, 47 new scars were acquired in 42 patients (20 medical, 22 surgical), 25 of them in children with unscarred kidneys at entry (13 medical, 12 surgical). New scars occurred mostly in children under 5 years of age and were observed more frequently in children with grade IV than grade III reflux. We conclude that with careful management, only a small proportion of children with severe reflux developed new scars and rarely after the first 5-year follow up period, and that there was no difference between children treated medically or surgically.The authors are the writing committee of the European arm of the International Reflux Study in Children. H. Olbing is deceased. Co-ordinating center, Essen, Germany: chairman H. Olbing, scientific co-ordinator T. Tamminen-Möbius, statistics H. Hirche, documentation H. Lax. Participating university hospitals and investigators: Bonn, Germany: R. Mallmann, D. Emons; Brussels, Belgium: M. Hall, A. Piepsz, C. Schulmann; Essen, Germany: H.J. Bachmann, W. Rascher, E. Brunier, C. Reiners, J. Behrendt, P. Mellin (deceased); Gothenburg, Sweden: U. Jodal, K. Hjälmås, E. Hanson, N. Nilsson, J. Bjure (deceased), R. Sixt; Hamburg, Germany: R. Busch, C. Montz; Helsinki, Finland: O. Koskimies, S. Wikström, E. Marttinen, A. Kivisaari, T. Korppi-Tommola; Oulu, Finland: J. Seppänen (deceased), N.P. Huttunen, U. Seppänen, J. Heikkilä; Stockholm, Sweden: A. Aperia, G. Löhr, P. Herin, U. Freyschuss, L. Blom, U. Erasmi, B. Söderborg. Consultants: I. Claesson, K.-D. Ebel, R.A. Lebowitz, K. Parkkulainen, J.M. Smellie, I. Wikstad, and J. Winberg (deceased)     

Accuracy and precision of a new portable ultrasound scanner,the BME-150A,in residual urine volume measurement: a comparison with the BladderScan BVI 3000

The objective of the study was to determine the relative accuracy of a new portable ultrasound unit, BME-150A, and the BladderScan BVI 3000, as assessed in comparison with the catheterized residual urine volume. We used both of these machines to prospectively measure the residual urine volumes of 89 patients (40 men and 49 women) who were undergoing urodynamic studies. The ultrasound measurements were compared with the post-scan bladder volumes obtained by catheterization in the same patients. The ultrasounds were followed immediately (within 5 min) by in-and-out catheterizations while the patients were in a supine position. There were a total of 116 paired measurements made. The BME-150A and the BVI 3000 demonstrated a correlation with the residual volume of 0.92 and 0.94, and a mean difference from the true residual volume of 7.8 and 3.6 ml, respectively. Intraclass correlation coefficients for the accuracy of the two bladder scans were 0.90 for BME-150A and 0.95 for BVI 3000. The difference of accuracy between the two models was not significant (p = 0.2421). There were six cases in which a follow-up evaluation of falsely elevated post-void residual urine volume measurements on the ultrasound studies resulted in comparatively low catheterized volumes, with a range of differences from 66 to 275.5 ml. These cases were diagnosed with an ovarian cyst, uterine myoma, or uterine adenomyosis on pelvic ultrasonography. The accuracy of the BME-150A is comparable to that of the BVI 3000 in estimating the true residual urine volumes and is sufficient enough for us to recommend its use as an alternative to catheterization.     

Management of asymptomatic hyperuricaemia in patients with chronic kidney disease by Japanese nephrologists: a questionnaire survey

Aim: Hyperuricaemia is associated with chronic kidney disease (CKD) progression and cardiovascular events (CVE). In a US study, only 4% of rheumatologists initiated urate‐lowering therapy in patients with asymptomatic hyperuricaemia (AHU). The present study aimed to clarify how Japanese board‐certified nephrologists manage AHU in CKD patients. Methods: Questionnaires on management of AHU in CKD stage 3 or more were mailed to 1500 Japanese board‐certified nephrologists, excluding paediatricians and urologists, randomly selected from the directory of the Japanese Society of Nephrology (n = 2976). Results: Five hundred and ninety‐five nephrologists (40%) responded. Most nephrologists (84–89%) recommended that AHU in patients in CKD stages 3–5 should be treated, but fewer nephrologists (63%) recommended that AHU in patients of CKD stage 5D should be treated. The serum urate level to start urate‐lowering therapy and the target serum urate level to be achieved (mg/dL) were 8.2 ± 0.9 and 6.9 ± 0.9, 8.4 ± 0.9 and 7.0 ± 1.0, 8.6 ± 1.0 and 7.3 ± 1.1, and 9.1 ± 1.2 and 7.8 ± 1.3 at stages 3, 4, 5 and 5D, respectively. The most frequently used maximal dosage of allopurinol was 100 mg/day at each stage. Benzbromarone was used in 52% of patients at stage 3, but only in 29%, 13% and 5% of patients at stages 4, 5 and 5D, respectively. The most important reasons to treat AHU at CKD stages 3–5 were prevention of CKD progression (45%), CVE (33%), gout (18%) and urolithiasis (3%). Conclusion: Most Japanese nephrologists treat AHU in pre‐dialysis CKD with an aim to prevent CKD progression or CVE mainly by allopurinol.     

胎肾细胞移植对维甲酸所致大鼠骨质疏松症的实验研究

目的：探讨胎肾细胞悬液对维甲酸所致大鼠骨质疏松症的作用,为防治骨质疏松症寻找理想的药物。方法：通过静脉输液胎肾细胞悬液,观察胎肾对维甲酸所致骨质疏松大鼠血钙,磷,尿钙,磷,尿羟脯氨酸等生化指标和骨形态结构的影响。并与防治骨质疏松药物尼尔雌醇及生理盐水进行对照,观察其对上述生化指标及骨质疏松模型大鼠骨质的改善。结果：与尼尔雌醇组及生理盐水组相比,胎肾组大鼠血钙明显升高,尿羟脯氨酸水平降低（P＜0．05）,而尿钙、尿磷无显性差异（P＞0．05）。在骨显微结构的病理变化中,胎肾组大鼠的骨小梁厚度,面积及密度均高于尼尔雌醇组及生理盐水组,而骨小梁间隙和骨髓腔明显缩小（P＜0．05）,结论：胎肾细胞移植能有效治疗维甲酸所致大鼠骨质疏松。     

Bacteriuria management and urological evaluation of patients with spina bifida and neurogenic bladder: a multicenter survey

PURPOSE: We assessed how groups at spina bifida clinics evaluate and manage the urinary tract in patients with spina bifida, neurogenic bladder and bacteriuria. MATERIALS AND METHODS: A survey was mailed to all 169 clinics listed by the Spina Bifida Association of America. Survey items addressed baseline and surveillance evaluation, criteria used to assess urinary tract health and approaches to treatment in patients with spina bifida and neurogenic bladder. RESULTS: Of the 169 clinics personnel at 59 (35%) responded to the survey. Almost half of the respondents had an established protocol or standard of care. At most clinics the use of ultrasound (93%), voiding cystourethrograms (85%) and urodynamic testing (76%) was supported but not renal isotopic studies (14%) or excretory urograms (2%) for baseline evaluation. At all clinics ultrasound was supported for routine surveillance but there was no consensus for other imaging modalities. Assessment of clinic approaches to the evaluation and management of bacteriuria demonstrated variable results, although at most clinics fever, flank pain, dysuria, and changes in urinary pattern were identified as being consistent with true infection. Groups at clinics following their protocol or standard of care showed no significant differences in their approach compared to those at clinics lacking a protocol or standard of care. CONCLUSIONS: No consensus exists for the evaluation and management of bacteriuria in patients with spina bifida and neurogenic bladder at clinics specializing in the care of such patients, even at those with established standards of care. A clear need exists for an established, national set of evidence based guidelines to assist medical decision making in this high risk population and, thus, improve care.     

Clinical and pathological features of renal amyloidosis: An analysis of 32 patients in a single Chinese centre

Aim:   To summarize the clinical and pathological features of renal amyloidosis in order to achieve early diagnosis.
Methods:   The clinical and pathological data of 32 patients with renal amyloidosis, diagnosed by renal biopsy in one renal centre, were retrospectively analyzed. Immunohistochemistry of amyloid A protein and immunoglobulin light chains was further performed on the renal specimens for further classification.
Results:   Twenty-four out of the 32 patients (75%) were not considered to have renal amyloidosis by local physicians; 91.7% (22/24) of them had at least one of the following signs: bodyweight loss, organ enlargement and decreased blood pressure. Twenty-nine out of the 32 patients (90.6%) were over 40 years, 30 patients (93.8%) had nephrotic syndrome, and 21 patients (65.6%) were found to have monoclonal light chain in serum or urine by immunofixation. Six patients (18.8%) were negative by Congo red stain and were diagnosed as having early renal amyloidosis by electron microscopy. Twenty-eight patients were diagnosed as having AL amyloidosis, two were suspected of having AL amyloidosis, one had AA amyloidosis and the status of the remaining patient was undetermined.
Conclusion:   Renal amyloidosis is frequently neglected by local physicians in China. Middle-aged nephrotic patients with weight loss, organ enlargement and monoclonal light chains in serum or urine should be highly suspected of the disease. Renal biopsies, especially electron microscopy, play a crucial role in the early diagnosis of renal amyloidosis.