The long-term outcome of prenatally detected posterior urethral valves: a 10 to 23-year follow-up study

OBJECTIVE

To document the functional outcome of patients with prenatally detected posterior urethral valves (PUV) in the second decade of life, and to evaluate the possible impact of prenatal diagnosis on the long‐term outcome of this condition

PATIENTS AND METHODS

We analysed the functional outcome of 25 patients with prenatally detected PUV born between 1984 and 1996, whose mean (range) age at follow‐up was 17.7 (10–23) years. The findings were compared with those in 17 patients (mean age 16.1 years) who had presented clinically to our unit during the same period. The duration of follow‐up in both groups was ≥10 years. Late outcomes were also compared with published data for PUV. Outcome measures included; death, incidence of end‐stage renal failure (ESRF), age at transplantation and the most the recently available plasma creatinine level in untransplanted patients. We also examined any possible association between functional outcome and early predictors, including nadir plasma creatinine level at <1 year and vesico‐ureteric reflux (VUR).

RESULTS

Three patients died (12%), two as neonates and one aged 3 years. Of five patients who had been shunted in utero, four died or developed early‐onset renal failure. In the 23 prenatally detected patients who survived the neonatal period, four (17%) had a renal transplant at a mean (range) age of 6.5 (3.0–12.0) years. Of 19 patients with prenatally detected PUV who had not been transplanted in the first 12 years of life, only one (5%) developed new‐onset ESRF at 10.0–23.4 years whilst 11 (58%) of these patients had normal creatinine values. In the untransplanted patients there was a statistically significant correlation between age and plasma creatinine level, but no correlation between late functional outcome and nadir creatinine in the first year of life, or bilateral VUR.

CONCLUSIONS

Prenatal diagnosis had little impact on mortality or ESRF in the first decade of life. This appears to be largely predetermined by renal dysplasia and the severity of intrauterine obstruction. However, the functional outcome of patients with prenatally detected PUV aged 10–23 years was considerably better than published long‐term data and the outcome of clinically presenting patients in our study. These findings suggest that the long‐term prognosis of PUV of intermediate severity might be improved by prenatal diagnosis.     

Emotional intelligence in relation to nursing leadership: does it matter?

Aim  Discuss the importance of studying emotional intelligence (EI) of nursing leaders and the job satisfaction of nursing staff.
Background  The nursing shortage and issues with retention signifies the importance of assessing the influence nurse managers have on staff job satisfaction.
Evaluation  A review of the literature on the development of EI and the level of study involving nursing leadership was conducted to determine the need for further research in this area.
Key issues  Neurobehavioural research involving the limbic system has indicated that EI can be learned through educational programmes.
Conclusion  There is a need for further research in the area of EI of nurse managers in their role as leaders and the impact they have on the job satisfaction level of their nursing staff.
Implications for nursing management  The increasing nursing shortage and turnover rates signify the importance of research in the EI level of nursing leaders. Future research may include implementing educational programmes in the area of EI for nursing leaders resulting in a more positive work environment. Determining if EI influences nursing job satisfaction will provide a foundation for ongoing programme implementation to support and develop our nursing leaders.     

Perceptions of postmastectomy patients. Part I. The relationships between social support and network providers

With the increased incidence of breast cancer in the United States, emotional adjustment of postmastectomy patients is a growing concern. This study was designed to examine demographic and social network variables that relate to social support. Breast cancer patients (N = 613) who had a mastectomy within the past 24 months (means = 12 months) responded to the Norbeck Social Support Questionnaire (NSSQ). To supplement the quantitative data, interviews were conducted with 27 women. Results from the NSSQ indicated that women perceived greater emotional support (means = 4.25) than aid (means = 3.97) from their network providers. The average number of persons (both professional and lay) in the women's total network was 8.1, comprising 57% family members and 27% friends. Relationships with network providers were characterized as stable (5-year) and frequent (weekly). Social support increased with age, but the size of the network decreased. No significant difference existed between marital categories in emotional support or frequency of contact. Married or widowed women perceived similar amounts of aid, whereas single and divorced women indicated less. Divorced women reported significantly larger networks than the other three marital status categories. Persons with less education perceived greater emotional support than those with more education. Being married had a significant positive relationship to emotional support (p less than .001).     

Lack of major involvement of human uroplakin genes in vesicoureteral reflux: implications for disease heterogeneity

BACKGROUND: Primary vesicoureteral reflux (VUR) is a hereditary disorder characterized by the retrograde flow of urine into the ureters and kidneys. It affects about 1% of the young children and is thus one of the most common hereditary diseases. Its associated nephropathy is an important cause of end-stage renal failure in children and adults. Recent studies indicate that genetic ablation of mouse uroplakin (UP) III gene, which encodes a 47 kD urothelial-specific integral membrane protein forming urothelial plaques, causes VUR and hydronephrosis. METHODS: To begin to determine whether mutations in UP genes might play a role in human VUR, we genotyped all four UP genes in 76 patients with radiologically proven primary VUR by polymerase chain reaction (PCR) amplification and sequencing of all their exons plus 50 to 150 bp of flanking intronic sequences. RESULTS: Eighteen single nucleotide polymorphisms (SNPs) were identified, seven of which were missense, with no truncation or frame shift mutations. Since healthy relatives of the VUR probands are not reliable negative controls for VUR, we used a population of 90 race-matched, healthy individuals, unrelated to the VUR patients, as controls to perform an association study. Most of the SNPs were not found to be significantly associated with VUR. However, SNP1 of UP Ia gene affecting a C to T conversion and an Ala7Val change, and SNP7 of UP III affecting a C to G conversion and a Pro154Ala change, were marginally associated with VUR (both P= 0.08). Studies of additional cases yielded a second set of data that, in combination with the first set, confirmed a weak association of UP III SNP7 in VUR (P= 0.036 adjusted for both subsets of cases vs. controls). CONCLUSION: Such a weak association and the lack of families with simple dominant Mendelian inheritance suggest that missense changes of uroplakin genes cannot play a dominant role in causing VUR in humans, although they may be weak risk factors contributing to a complex polygenic disease. The fact that no truncation or frame shift mutations have been found in any of the VUR patients, coupled with our recent finding that some breeding pairs of UP III knockout mice yield litters that show not only VUR, but also severe hydronephrosis and neonatal death, raises the possibility that major uroplakin mutations could be embryonically or postnatally lethal in humans.     

Recent insights into kidney diseases associated with glomerular cysts

Glomerular cysts can exist in the context of several different kidney diseases. Advances in the last few years have begun to unravel the genetic bases and pathogenesis of some of these entities, many of which have an origin in abnormal development. In this review, we highlight recent insights into three types of disease associated with glomerular cysts: (1) mutations of the hepatocyte nuclear factor 1β (HNF-1β) in the recently described renal cysts and diabetes syndrome, (2) mutations of OFD1 in the oral facial digital syndrome type 1 and (3) the role of fetal urinary tract obstruction. Received: 10 August 2001 / Revised: 7 December 2001 / Accepted: 10 December 2001     

Light delivery to tumour tissue through implanted optical fibres during photodynamic therapy

The maximum rate at which the light dose may be delivered during interstitial photodynamic therapy (PDT) has been measured for several types of delivery fibre. Measurements of light irradiance at the skin surface overlying subcutaneous experimental tumours were made during interstitial irradiation by 200m core diameter fibres whose output ends were either plane-cut or 5 mm long cylindrical diffusers. For plane-cut fibres, a rapid fall in light transmission, due to blood coagulation at the fibre tip, was observed at output powers greater than 229 mW and 104 mW in tumours with and without photosensitizer, respectively. Such a rapid fall was not observed with cylindrical diffusing fibres at output powers up to 1 W.In the clinical use of PDT the fibre output power is held constant at a level below which thermal effects may occur. In a second study, therefore, the irradiance at the skin surface was monitored for this treatment regime. A decrease in light transmission during treatment was observed. Plane-cut fibres showed a greater decrease than cylindrical diffusing fibres and for a given fibre type, non-photosensitized tumours showed a greater decrease than photosensitized tumours. For cylindrical diffusing fibres at output powers of 150 mW or less there was a 9% decrease in irradiance measured at the skin surface during the first 1000 s of treatment. The decrease was larger (30–40%) for fibre output powers in the range 200–300 mW.