Management of the fetus with congenital hydronephrosis II: Prognostic criteria and selection for treatment

Selecting appropriate management for the fetus with bilateral congenital hydronephrosis depends on our ability to accurately assess the severity of existing renal damage and to predict the potential for recovery of renal and pulmonary function if the obstruction is relieved. We reviewed our experience with 20 fetuses with congenital bilateral hydronephrosis to determine the prognostic value of various criteria used to assess functional potential, including temporary catheter exteriorization to measure fetal urine output and composition. Based on autopsy, biopsy, or clinical outcome, ten fetuses were classified retrospectively as "poor function," and ten fetuses as "good function." The good function group could be distinguished from the poor function group by the following criteria: Amniotic fluid (AF) status at presentation (P less than .001), ultrasound appearance of the fetal kidneys (P less than .05), fetal urine sodium and chloride concentration and osmolarity (P less than .001), and hourly urine output (P less than .02), but not by fetal urine iothalamate excretion or potassium and creatinine concentrations (P greater than .05). Based on these results, we have identified prognostic criteria that accurately identify the fetus with "good function" from the fetus with "poor function." We also reviewed the clinical management of our last 12 unreported cases. Ten fetuses had undergone diagnostic catheter placement and in utero renal function testing. This led to placement of a therapeutic indwelling catheter-shunt in seven fetuses (three required multiple shunts) and a suprapubic vesicostomy in another. Catheter related complications, including three cases of chorioamnionitis, emphasize the need for better methods of in utero decompression in selected cases. Our ability to select appropriate management has improved markedly.     

Surgical indications for unilateral neonatal hydronephrosis in considering ureteropelvic junction obstruction

Prenatal hydronephrosis is one of the most common urological congenital abnormalities detected by ultrasound. The incidence ranges from 0.59% to 0.69%. Approximately 50% of these fetuses do not have hydronephrosis on postnatal examination, whereas 25–33% of the rest have persistent hydronephrosis leading to the diagnosis of ureteropelvic junction (UPJ) obstruction. Renal ultrasonography and renal radionuclide scanning are the major modalities used for assessment and follow-up. Three main criteria used to determine the presence of obstruction are: (1) the magnitude of hydronephrosis present on ultrasound, (2) the relative renal function (RRF) measured by renography, and (3) the response of radionuclide washout with furosemide. Unfortunately, it is not always easy to determine obstruction; different types of management have been developed. Without depending on the severity of renal pelvis dilation, percentage of RRF, and response of radionuclide washout in the initial presentation, early surgery to preserve renal function and aggressive observation to prevent unnecessary surgery are two extremes on the spectrum of management for neonatal UPJ obstruction. Relying on renal function in renography, <35–40% or 5–10% of a decrease in the percentage of RRF or on the enlarging of hydronephrosis, respectively, and parenchymal thinning on ultrasonography are the indications for the surgical management to recover renal function in time. In addition to renal function change and imaging progression, the follow-up protocol and family compliance are the other considerations in prevention of impaired renal function. Through more than 40 years of development in the field of UPJ obstruction in infants, there have been several advances in management but controversies remain to be resolved. In this review, we focus on the surgical indications for the UPJ obstruction in this cohort.     

Correction of congenital hydronephrosis in utero IV: in utero decompression prevents renal dysplasia

Renal dysplasia (RD) is commonly seen in babies with urinary tract obstruction (UTO). Recent experimental evidence suggests that early fetal UTO leads to the development of RD. The RD seen in children with congenital UTO is usually not reversible, even when the obstruction is relieved soon after birth. Is the RD associated with congenital UTO preventable or reversible by decompression of the urinary tract early in gestation? If so, at what stage of development must this decompression be performed? We produced complete unilateral ureteral obstruction in 25 early second trimester (62 to 65 days) lamb fetuses, a procedure that results in ipsilateral RD at term (140 days). At a second operation, 20, 40, or 60 days after the initial procedure, we decompressed the obstructed kidney by a cutaneous end-ureterostomy. The contralateral unobstructed kidneys served as controls. Renal function (urine output and iothalamate clearance) and histopathology were evaluated after delivery at term. Recovery of renal function was directly proportional to the duration of in utero decompression and inversely proportional to duration of obstruction. In addition, in utero decompression prevented or greatly ameliorated the development of RD. However, some postobstructive changes persisted; these were proportional to the length of in utero obstruction. These results substantiate the clinical impression that some human fetuses with congenital UTO may benefit from early in utero decompression.     

小儿肾积水术后短期肾盂内压力的恢复

【摘要】〓目的〓探讨先天性肾积水小儿行离断式肾盂输尿管成形术后肾盂内压力的恢复规律。方法〓回顾性分析30例单侧肾盂输尿管连接部狭窄行离断式肾盂输尿管成形术的患儿。术中放置肾造瘘管。手术后14天内,每天测量肾盂内压力、膀胱内压力、患侧尿量。结果〓术后第一天,患侧肾盂内压力即降低至和膀胱内压力无明显差异。术后7天左右,肾盂内压力短暂升高,之后再次降低并保持稳定。患肾尿量于术后7天左右短暂性增多。结论〓术后7天左右,吻合口存在“功能性”梗阻。肾造瘘管或者输尿管支架管至少应该放置1周。     

Histologic and molecular evidence of obstructive uropathy in rats with hereditary congenital hydronephrosis

Partial obstruction of the upper urinary tract, a frequent challenge for the pediatric urologist, leads to renal damage, if deobstruction is delayed. Several but sometimes unsatisfactory animal models have been developed to study this phenomenon. Obstruction created by surgical manipulation lacks adequate correlation with a developing congenital obstruction. In some animals with congenital hydronephrosis, evidence of renal obstruction is absent. A study of the renal morphology of rats with hereditary unilateral hydronephrosis has exhibited clear evidence of renal obstruction distinguishable from renal dilatation. The renal mRNA expression of renin and transforming-growth factor- β₁ (TGF-β₁) was measured by a semiquantitative RT-PCR technique. In hydronephrotic kidneys, a marked loss of parenchyma, atrophy and dilation of tubuli and collecting ducts and interstitial fibrosis was observed. The mRNA expression of renin was increased significantly in comparison to controls, whereas the contralateral kidneys showed renin activity below control levels. TGF-β₁ expression was markedly increased in hydronephrotic kidneys, whereas contralateral kidneys did not differ significantly from control values. These data suggest the presence of renal obstruction and not only renal dilatation in these rats with congenital hydronephrosis. This colony seems to be a representative animal model to study congenital renal obstruction even in the fetal period without the need of surgical manipulation. Received: 3 June 1999 / Accepted: 1 October 1999     

Increased transforming growth factor-β1 and tubulointerstitial fibrosis in rats with congenital hydronephrosis

OBJECTIVES: Most of our knowledge concerning renal obstruction has been derived from experimental animal models, and it is not yet well defined in spontaneous hydronephrosis. The aim of our study is to evaluate the roles of transforming growth factor-beta1 (TGF-beta1) and apoptosis in congenital hydronephrotic kidneys in comparison with experimental models. METHODS: We made histological studies on kidneys from 6-week-old Wistar-Imamichi rats with congenital unilateral hydronephrosis as well as surgical models of complete or partial unilateral ureteral obstruction. The severity of hydronephrotic kidneys was evaluated on routine hematoxylin and eosin (H&E) stained sections, and the tubulointerstitial fibrosis analyzed morphometrically on Masson's trichrome stained sections. Renal tubular atrophy was assessed on periodic acid Schiff (PAS) stained sections, and tubular cell apoptosis assessed with TUNEL technique. The renal TGF-beta1 level was determined by a sandwich enzyme-linked immunosorbent assay (ELISA). RESULTS: We observed a significant loss of kidney weight with profound compensatory growth of the contralateral kidney in rats with congenital hydronephrosis. Most of the hydronephrotic kidneys were markedly enlarged with dilatation of the collecting system, renal parenchymal thinning, tubular atrophy, interstitial infiltration and fibrosis. The renal TGF-beta1 level was markedly elevated in hydronephrotic kidneys as compared with normal controls (326.01 +/- 30.64 pg/mg protein vs 227.81 +/- 11.07 pg/mg protein, P < 0.01). The tubular apoptotic score in hydronephrotic kidneys was also significantly higher than normal controls (2.17 +/- 0.50/HPF [high power field]vs 0.14 +/- 0.04/HPF, P < 0.01). The increased TGF-beta1 and apoptotic status paralleled the histological changes of tubulointerstitial fibrosis and tubular atrophy. Similar findings were also obtained in experimental obstructive models. CONCLUSION: In comparison with surgical models of partial and complete ureteral obstruction, our data provide solid morphological and molecular evidences of renal obstruction in rats with congenital hydronephrosis.     

Assessment of regional kidney function may provide new clinical understanding and assist in treatment of children with prenatal hydronephrosis

PURPOSE: As calculated from a (99m)technetium (Tc)-mercaptoacetyltriglycine (MAG3) renogram, differential renal function is an important parameter affecting the clinical treatment of children with prenatal unilateral hydronephrosis. We determined whether value is potentially added by calculating a functional image from MAG3 renograms that represents proximal tubule ability to clear tracer from the blood, that is the MAG3 clearance image. MATERIALS AND METHODS: MAG3 clearance image findings and differential renal function at presentation and followup in 59 nonsurgically and 42 surgically treated children with prenatally diagnosed unilateral hydronephrosis were retrospectively reviewed. All patients underwent at least 3 99mTc-MAG3 renograms. RESULTS: In the surgical and nonsurgical groups there was no significant difference in followup (p = 0.11), age at presentation (p = 0.98) or age at last visit (p = 0.97), whereas differential renal function was significantly different (p = 0.01). A large affected kidney with focal defects was the most frequent finding in each group, including kidneys with a normal differential renal function of 45% to 55%. In the nonsurgical group the most common observation was improvement in the MAG3 clearance image in 26 of 59 cases (44%) and stabilized differential renal function in 41 (70%). Postoperatively the MAG3 clearance image improved in 31 of 42 cases (74%) and differential renal function remained stable in 25 (60%), while no significant increase in differential renal function was noted at the last visit (p = 0.74). CONCLUSIONS: This study shows that it is possible to assess regional parenchymal function using the MAG3 clearance image in children with unilateral hydronephrosis. This information is available in addition to 99mTc-MAG3 information on the whole kidney, that is differential renal function. The majority of affected kidneys with normal differential renal function showed parenchymal defects, suggesting regional renal dysfunction. However, functional improvement on a regional basis occurred in each group of children during followup.     

Predictive value of renal histological changes for postoperative renal function improvement in children with congenital ureteropelvic junction stenosis

BACKGROUND: The aim of this study was to evaluate the relationship between renal function, as measured by diuretic radionuclide renography, and the outcome of pyeloplasty. A study was designed to evaluate renal parenchymal biopsy specimens derived from children undergoing corrective surgery for ureteropelvic junction (UPJ) stenosis, and compare these to preoperative and postoperative renal function status. METHODS: Thirty-five children with congenital unilateral UPJ stenosis were evaluated. In addition to all conventional diagnostic procedures for UPJ stenosis, differential renal functional (DRF) activity was assessed in each of these children by obtaining 99mTc diethylenetriaminepentaacetic acid renogram curves. All children underwent dismembered pyeloplasty, and follow-up renogram evaluation was conducted 6 and 12 months after surgical repair. Biopsy specimens from renal cortical regions obtained during the surgical correction of UPJ stenosis were evaluated, and changes in renal histology were graded from I to V according to their severity. Spearman's correlation test was used to compare the histological evaluation results and the basal, 6- and 12-month follow-up DRF findings. A Wilcoxon paired test was used to evaluate statistical differences between values. RESULTS: The findings showed a positive correlation between the severity of histological changes and DRF activity. All kidneys (22) with a DRF activity value of < 40% preoperatively demonstrated at least grade III changes when biopsy specimens were examined. Of children with a DRF activity value > 40% (13), only three showed severe histological changes. Histological grades were correlated between basal (r = -0.4; P = 0.019), 6-month (r = 0.54; P = 0.002) and 12-month (r = 0.54; P = 0.02) findings. In the Wilcoxon paired test, there was a statistically significant difference between basal and 6-month values (P < 0.05), and also between basal and 12-month values (P < 0.01). There was no statistically significant difference between 6- and 12-month values (P > 0.20). CONCLUSION: Comparative evaluation of postoperative renal function with DRF activity and renal parenchymal histological alterations revealed a close correlation in terms of renal function improvement potential following reconstructive surgery in children with UPJ stenosis.     

Prospective comparison of POSSUM and P-POSSUM with clinical assessment of mortality following emergency surgery

BACKGROUND: Tools to accurately estimate the risk of death following emergency surgery are useful adjuncts to informed consent and clinical decisions. This prospective study compared the Physiological and Operative Severity Score for the Enumeration of Mortality and Morbidity (POSSUM) and Portsmouth POSSUM (P-POSSUM) scoring systems with clinical judgement in predicting mortality from emergency surgery. METHODS: Data were collected prospectively from 163 patients. Details of the physiological and operative severity scores were recorded for POSSUM and P-POSSUM. The estimates of both the surgeon and anaesthetist for 30-day and in-hospital mortality were also recorded pre-operatively. The accuracies of the four predictions were then compared with actual mortalities using linear and exponential analysis and receiver operator characteristics (ROC). RESULTS: P-POSSUM gave the most accurate prediction of 30-day mortality using linear analysis [observed to expected ratio (O : E) = 1.0]. POSSUM gave the most accurate prediction using exponential analysis (O : E = 1.15). Clinical judgement of mortality from both operating surgeons and anaesthetists compared favourably with the scoring systems for 30-day mortality (O : E = 0.83 and O : E = 0.93, respectively). ROC analyses showed both clinical judgement and the POSSUM scores to be good predictors of 30-day mortality with area under the curve values (AUC) of 0.903, 0.907, 0.946 and 0.940 for surgeons, anaesthetists, POSSUM and P-POSSUM respectively. CONCLUSIONS: POSSUM and P-POSSUM appear to be useful indicators for the prediction of mortality. Clinical judgement compares strongly with scoring systems in predicting post-operative mortality, but may underestimate mortality in very high-risk patients with more than 90% mortality.     

Gastric antrum hypertrophy causing outlet obstruction in an infant with congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is associated with multiple congenital anomalies affecting several organ systems, including the gastrointestinal system. Pyloric stenosis and bands are known and previously reported etiologies of gastric outlet obstruction in infants with CDH. We report the first case of gastric antrum hypertrophy causing gastric outlet obstruction in an infant with CDH.     

Helicopter emergency medical service in out-of-hospital cardiac arrest--a 10-year population-based study

BACKGROUND: In 1988, Norway established a countrywide, physician staffed helicopter emergency medical service (HEMS). The medical benefit remains controversial. The aim of this study was to estimate the population incidence of HEMS involvement in out-of-hospital cardiac arrest, report the patient outcome and evaluate the contribution of HEMS to survival. METHODS: We studied HEMS operations in central Norway (population 364,000) during a 10-year period (1988-1997). Missions were classified according to type and quality of intervention done by the primary care providers. HEMS witnessed cardiac arrests were not considered. Patient outcome was determined as survival to hospital discharge with cerebral performance category (CPC) score. The contribution made by HEMS in each survivor was assessed from the timing of return of spontaneous circulation (ROSC) and from subsequent need for advanced medical intervention. The relation between survival and HEMS response time was investigated by ordinal correlation. RESULTS: A total of 541 requests (14.9 per 100,000 inhabitants per year) were identified, of which 424 missions were completed. Overall survival to discharge was 36/541 (6.6%), yielding a population survival incidence of 1 per 100,000 per year. Ninety-five percent of survivors made a favourable cerebral outcome (CPC 1 or 2). General practitioners/ambulance personnel resuscitated 29 out of 36 survivors. The remainder achieved ROSC after HEMS arrival. Case by case, HEMS assistance was considered possibly important in 17 survivors. We found no relation between survival and HEMS response time (P=0.77). DISCUSSION: Survival following out-of-hospital cardiac arrest assisted by HEMS in this region is low, but not negligible. While primary care is most important, HEMS may possibly contribute to the additional survival of 0.19 to 0.46 patients per 100,000 per year. This benefit appears to be independent of HEMS response time.     

前列腺增生症患者的尿动力学诊断价值评估

目的　评估尿流动力学检查在前列腺增生症患者所致的下尿路症候群中的诊断价值。方法　采用自制半卧位床 ,常规行尿流率、压力 流率测定及尿道测压 ,并同步测定尿道外括约肌肌电图及残余尿量 ;并记录膀胱逼尿肌受损情况、膀胱顺应性和尿道外括约肌协调情况。联合应用A G图、P Q图及DS诊断BOO(膀胱出口梗阻 ) ,结果进行统计处理。结果　本组患者 338例 ,312例诊断为BOO ,71例可疑 ,5例无BOO。其中伴膀胱低顺应性 12 3例 ,膀胱逼尿肌功能受损 10 8例 ,尿道外括约肌功能失调 15 7例 ,不稳定膀胱 134例。随着BOO程度加重 ,Pdet Qmax、Popen、DS、IPSS积分及前列腺体积呈升高趋势 ,膀胱顺应性、Qmax呈下降趋势。结论　前列腺增生症患者排尿障碍的机制是排尿过程中机械因素和动力学因素共同作用的结果 ,尿流动力学检查在其诊断评估中扮演着一个极其重要的角色。BOO程度与Pdet Qmax、Popen、DS、IPSS积分及前列腺体积呈正相关 ,与膀胱顺应性、Qmax呈负相关     

Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children

Background

Congenital Hyperinsulinism (HI) causes severe hypoglycemia in neonates and children. We reviewed our experience with pancreatectomy for the various types of HI.

Environmental temperature stress on drugs in prehospital emergency medical service

BACKGROUND: Drugs used in prehospital emergency medical service (EMS) in principle are subject to the same storage restrictions as hospital-based medications. The prehospital emergency environment however, often exceeds these storage recommendations. Main stress factors are sunlight, vibration and extreme temperature, which may lead to alteration in chemical and physical stability of stored pharmaceuticals, as well as microbiological contamination and concentration enhancement of pharmacological inserts. METHODS: The purpose of this study was to determine the environmental temperature stress upon drugs used in the prehospital EMS under real mission conditions within different types of rescue vehicles (rescue helicopter [HEMS], ambulance [AMB] and emergency physician transport vehicle [EPTV]) during a 'summer' and 'winter' monitoring period (2 months duration each/location: southern Germany). RESULTS: Recorded temperatures varied from -13.2 degrees C to +50.6 degrees C. The recommended maximum storage temperature (+25 degrees C) was exceeded in all rescue vehicles (33-45% of total exposure time), whereas the recommended minimum storage temperature (0 degrees C) only fell short in the EPTV (19% of total exposure time). The daily maximum temperature variations ranged from 19.0 degrees C (winter) to 32.9 degrees C (summer). CONCLUSIONS: These results show that even in a moderate climatic zone, drugs used in prehospital EMS are significantly influenced by temperature stress; furthermore, these results recommend the usage of temperature-controlled drug boxes.     

Renal Function in Patients with Orthotopic Liver Transplantation

Due to multiple reasons, acute renal failure (ARF) commonly develops in the early postoperative period of orthotopic liver transplantation (OLT) recipients. The records of OLT recipients between 1999 and 2004 were evaluated. Age, gender, primary disease, history of diabetes, immunosuppressive drugs, pre- and postoperative renal function tests, serum electrolytes, dialysis, liver functions tests, and renal function tests in follow-up period were noted. We followed 16 patients with OLT in our center. ARF developed in 8 patients. Dialysis was performed in only 2 patients, and other patients with ARF were managed with conservative measures. Hypertensive crisis and cerebrovascular stroke developed in 1 diabetic hypertensive patient.     

Extracorporeal shock wave lithotripsy produces a lower stone-free rate in patients with stones and renal cysts

BACKGROUND: Renal cysts have a space-occupying effect and therefore can distort the pelvicalyceal anatomy. This distortion often produces abnormalities in normal urinary drainage. In the same way, it may effect the ability of the kidneys to become stone free after extracorporeal shock wave lithotripsy (ESWL). The purpose of the current study is to evaluate the effect of renal cysts on the outcome of ESWL. METHODS: We studied 15 patients who had renal stones and coexistent renal cysts. Four patients had polycystic disease, five patients had multiple cysts and six patients has solitary ones. All cysts produced a distortion to the calyceal system of the kidneys, a fact confirmed by intravenous urography (IVU) and computed tomography (CT). All patients underwent ESWL and their stone-free status was evaluated 1 month later by ultrasound and plain kidney ureter bladder (KUB) X-ray. RESULTS: We had a total 60% (9/15) stone-free patients in our study group and a stone fragmentation rate of 100%. Patients with more cysts had lower stone-free rates. Patients with polycystic kidneys had a 25% (1/4) stone-free rate, while patients with multiple cysts and solitary cysts had, 60% (3/5) and 83.3% (5/6), respectively. These results are lower than the rates reported in patients without renal cysts. CONCLUSIONS: We believe that renal cysts may interfere with the passage of stone fragments, due to the impediment of drainage and urinary stasis from the stretching and distortion of the calyceal system by the renal cysts.