Measurement of fetal urine production in mild infantile polycystic kidney disease—A case report

It is generally recognized that the sonographic findings of infantile polycystic kidney disease (IPKD) are bilaterally enlarged kidneys, oligohydramnios, an absent fetal bladder, and the typical kidney texture. Since there is a broad spectrum of renal compromise with IPKD, in utero diagnosis is thought to be limited to the severe forms. This paper reports a mild case of IPKD, where the in utero diagnosis was established by measuring fetal urine production and amniotic fluid volume serially during pregnancy, and by ultrasonographic examination of fetal kidneys.     

Syndromic encephalocele in a fetal case with a 1p35-pter deletion and a 14q32-qter duplication inherited from a maternal balanced translocation

Occipital encephalocele belongs to the family of neural tube defects, which occur in one among 2000 to 5000 live births. Syndromic encephaloceles include Meckel–Gruber syndrome and various chromosomal abnormalities. We report on a fetal case (13 WG) with bilateral cleft lip and palate, choanal atresia, occipital encephalocele, bilateral club feet, bilateral multicystic kidneys, enlarged bladder and urethral atresia. The fetal chromosome analysis showed a maternally inherited unbalanced translocation between the short arm of chromosome 1 and the long arm of chromosome 14, resulting in 1p35-pter deletion and 14q32-qter duplication (46,XY,der(1),t(1;14)(p35;q32)). Since the chromosomal breakpoints have not previously been implicated in syndromic encephalocele, this observation is of interest for the identification of other genes responsible for occipital encephalocele. Copyright © 2007 John Wiley & Sons, Ltd.     

Prenatal diagnosis of de novo distal 11q deletion associated with sonographic findings of unilateral duplex renal system,pyelectasis and orofacial clefts

In utero diagnosis of de novo distal 11q deletion associated with renal and orofacial malformations has not been previously described. We present a 35-year-old pregnant woman with prenatal sonographic findings of a unilateral duplex renal system, pyelectasis and orofacial clefts at 20 weeks' gestation. Both genetic amniocentesis and postnatal cytogenetic analysis revealed de novo 46,XX,del(11)(q23). After birth, the fetus manifested a dysmorphic phenotype correlated with del(11q) syndrome. Genetic marker analysis showed a paternally derived distal deletion of chromosome 11q and a breakpoint centromeric to D11S1341. The present case represents the earliest prenatal diagnosis of a duplex renal system, pyelectasis and an additional feature of orofacial clefts associated with distal 11q deletion. Prenatal sonographic detection of a duplex renal system, pyelectasis and orofacial clefts should warrant a careful assessment of fetal anatomy and prompt cytogenetic analysis looking for chromosomal aberrations. Copyright © 2001 John Wiley & Sons, Ltd.     

海水管系材料与HDR双相不锈钢的电偶腐蚀研究

研究了4种常用海水管系材料和HDR双相不锈钢在静止天然海水中的自腐蚀情况,进行了极化曲线测试,并将4种常用海水管系材料分别和HDR双相不锈钢进行了电偶腐蚀试验。结果表明,HDR双相不锈钢具有很好的耐海水腐蚀性能;在常用海水管系材料和HDR双相不锈钢的电偶腐蚀试验中,HDR双相不锈钢作为阴极受到保护,与其偶合的材料腐蚀速度明显加快。     

Experimental investigation on mechanical and metallurgical properties of super duplex stainless steel joints using friction welding process

Friction welding is one of the most economical and highly practicable methods in joining similar and dissimilar metals. In this study, high-quality welds are produced in the super duplex stainless steel by continuous drive friction welding successfully. Design of experiment was done using central composite design of response surface methodology. In the present work, the microstructure and mechanical properties of friction welded super duplex stainless steel (UNS S32760) were examined. The base material has a microstructure consisting of the ferrite matrix with austenite islands. Ferrite content was analyzed through the phase analyzer software and found that it is in the range of 42–55% in all weld metals. The phases were further analyzed through X-ray diffraction method. All the weld metals have higher hardness than the base metals. Weld transverse tensile failures consistently occurred away from the weld zone and exhibit more hardness, yield and ultimate tensile strengths than the base material. The austenite content increases with nitrogen concentration. Nitrogen could enhance the yield stress and ultimately tensile strength of super duplex stainless steel. Secondary phase precipitation is not observed in the welded joint probably due to the shorter heating times.     

Left-sided gastroschisis and bilateral multicystic dysplastic kidneys: a rare combination of anomalies

Left-sided gastroschisis is very rare. We report a case of left-sided gastroschisis associated with bilateral multicystic dysplastic kidneys. This combination of anomalies is unknown. The pathogenesis of gastroschisis is not well understood. It is now viewed as a malformation rather than disruption. The findings in this case support this view. The combination of dysplastic kidneys with ventral body wall defect suggests an early developmental defect. Copyright © 2007 John Wiley & Sons, Ltd.     

Unusual sonographic features of ARPKD

The classic sonographic appearance of the kidneys in fetuses with autosomal recessive polycystic kidney disease (ARPKD) has been well described. We report a case of enlarged kidneys with pyramidal hyperechogenicity quite similar to medullary nephrocalcinosis found in a fetus at 34 weeks' gestation. At 39 weeks, a female neonate was delivered and died after 22 h due to pulmonary insufficiency secondary to severe oligohydramnios. On pathological analysis, the gross and microscopic findings were typical of ARPKD with diffuse dilatation of tubules throughout. The fetal renal lobulation was prominent and on section, the pyramids were delineated within each lobule, accounting for the clear image of the pyramids observed on sonography. Copyright © 2006 John Wiley & Sons, Ltd.     

Prenatal morphology in meckel's syndrome (with special reference to polycystic kidneys and double encephalocele)

Prenatal morphology of Meckel's syndrome was studied in five fetuses of different gestational age, that had been aborted because ultrasonography and elevated amniotic AFP-levels indicated neural tube defect. Histologically, the enlarged polycystic kidneys were completely alike with respect to the type of involvement and differed only in the severity of changes. They could be identified as type III cystic kidneys according to the classification of Potter. Proliferation of hepatic bile ducts and slight cystic dilatation of pancreatic ducts is already evident in the youngest fetus. Additional cyst formation in the epididymis was found in one of the cases. Occipital encephalocele, located within an apical occipital bone defect was always associated with a second mostly occult encephalocele protruding through a separate defect of the basal occipital squame and of the first and second vertebral arch. It is assumed that double encephalocele represents a constant finding in Meckel's syndrome, indicating a specific pattern within the disturbance of neural tube closure.     

Prenatal sonographic diagnosis of autosomal recessive polycystic kidney disease (arpkd) during the early second trimester

Autosomal recessive polycystic kidney disease (ARPKD) is a rare hereditary disease with a high neonatal mortality. Currently, prenatal diagnosis is possible only during the second half of pregnancy, when bilaterally enlarged, echogenic kidneys are visible by ultrasound. We describe a case in which a diagnosis of ARPKD was sought in the first half of pregnancy. High-resolution ultrasonography revealed echogenic, normal-sized kidneys at 15+4 weeks. Microsatellite DNA analysis of a chorionic villus sample, parental blood, and blood of an affected sibling showed that the fetus had the maternal haplotype and a recombination of the paternal haplotype. Thus, no distinction between homo- and heterozygosity for the ARPKD mutation in the fetus was possible. A further ultrasound examination at 19+4 weeks confirmed the previous results, indicating that the fetus was likely to be affected. After termination of the pregnancy, the diagnosis was confirmed on microscopic examination.     

黔城推覆体群的形态、结构和运动学特征

在华南中、新生代红盆中存在一系列由元古宇 -古生界地层组成的地质体 ,突起于红盆之中。许多学者对其成因进行了研究 ,得出了不同的结论。笔者对沅麻盆地南段主体由板溪群、震旦系组成的黔城推覆体群进行了研究 ,认为其成因与印支期以来川、黔、湘地区存在的、由南东向北西推覆的区域性构造事件有关 ,其形态、结构与国内外许多典型的推覆结构有相似之处。该推覆体由数条撕裂断层分为几个叠瓦体 ,各有不同的形态、结构特征。本文用被动顶板双重构造模式来解释该推覆体群的运动学模式 ,并据该区的地质特征对经典的被动顶板双重构造模式进行了补充。     

Interactions of copper,cadmium and molybdenum in buffalo calves:the levels of trace element in blood,urine and tissues

Ten male buffalo calves were randomly allotted into five groups of two each. Four groups were fed with cadmium, molybdenum, cadmium-molybdenum, and copper-cadmium-molybdenum respectively for 130 days to determine the elements' metabolic interactions in calves. These results indicated that cadmium and molybdenum could increase the accumulation of molybdenum and cadmium in liver and kidneys in buffalo calves, but copper could not. reduce to normal the levels of molybdenum and cadmium in liver and kidneys caused by cadmium-molybdenum. In addition, we found the copper concentrations in liver and kindeys was significantly greater in treatment calves than in controls.     

Fetal renal artery flow velocity waveforms in the presence of congenital renal tract anomalies

Colour Doppler flow mapping of the renal arteries and subsequent pulsed Doppler measurement of impedance to flow in these vessels were attempted in 33 fetuses with postnatally confirmed renal pathology. The majority presented with unilateral or bilateral hydronephro-sis (n = 21) and bilateral renal agenesis (n = 8). Renal artery blood flow could be visualized in all, except for the eight cases of bilateral renal agenesis. Bilateral flow velocity recordings were collected in six out of 12 cases of bilateral hydronephrosis and in five out of nine cases of unilateral hydronephrosis. The pulsatility index (PI), as a measure of downstream impedance, was in the normal range in 16 out of 18 kidneys (88 per cent) in bilateral hydronephrosis and in 12 out of 14 kidneys (85 per cent) in unilateral hydronephrosis. The PI was significantly higher in severe hydronephrosis compared with mild hydronephrosis. In four cases of unilateral multicystic kidney, the PI was always higher on the affected side. Colour Doppler flow mapping and pulsed Doppler evaluation may be helpful in our understanding of renal vascularization in renal pathology and in confirming the diagnosis of renal agenesis.     

聚苯乙烯微塑料对糖尿病小鼠肾脏的影响

以糖尿病小鼠为模式生物,探究了100nm和5μφ的聚苯乙烯微塑料(PSMPs)(200μg/L)通过饮水暴露28d后对小鼠肾脏的毒性.结果表明,糖尿病小鼠对PSMPs的暴露更加敏感,100nm和5μm PSMPs暴露导致糖尿病小鼠肾脏出现明显的炎性细胞浸润和淤血等病理损伤,且 100nm PSMPs对肾脏造成的病理损伤...     

Dysplastic and polycystic kidneys: diagnosis,associations and management

Cystic and bright kidneys can pose a significant diagnostic dilemma when discovered as an incidental finding at the time of a routine fetal ultrasound scan. There are diverse aetiologies with equally variable implications for the prognosis in the affected fetus, and for future pregnancies. Accurate antenatal diagnosis in the absence of any positive family history is often not possible and a team approach to management (to include the fetal medicine specialist, paediatric nephrologist or urologist, geneticists and in some cases, pathologist) is essential. In this review we will attempt to describe the embryology and aetiology of these conditions and suggest an approach to management. Copyright © 2001 John Wiley & Sons, Ltd.     

Unusual prenatal presentation of Beckwith–Wiedemann syndrome

When Beckwith–Wiedemann syndrome (BWS) is detected prenatally, it is usually on the basis of macroglossia, exomphalos or enlarged kidneys. We describe a case that presented as gross hepatomegaly and a suspected enlarged pancreas at 20 weeks' gestation, with none of the usual features. Copyright © 2004 John Wiley & Sons, Ltd.     

The early prenatal sonographic diagnosis of renal agenesis: Techniques and possible pitfalls

Out of 13 252 cases in which fetal bilateral echogenic kidneys were detected by transvaginal sonography between 12 and 18 weeks' gestation, there were nine fetuses where oval hypoechogenic masses were detected in the renal bed. In five fetuses where hypoechogenic masses in the renal bed were sonographically visualized, postabortal examination was compatible with renal agenesis and the hypoechogenic masses proved to be enlarged adrenals. In three additional cases, unilateral renal agenesis was accompanied by unilateral enlarged adrenals, radiologically confirmed postnatally. In one case, a false-positive sonographic diagnosis of Potter syndrome was made because of bilateral hypoechogenic masses in the renal bed. Postabortal examination detected hypoplastic kidneys, but of normal histology, in a dyskaryotic fetus with trisomy 22. In four cases of renal agenesis, the amniotic fluid was of normal volume until the 17th week. In two of the five cases of Potter syndrome, a cystic structure, compatible with the urinary bladder, was detected in the pelvis at 14 weeks. The diagnostic criteria for renal agenesis in the early fetus differ from those used in the second half of gestation.     

银矿与莫霍面的关系——以浙闽和天山地区为例

银是我国的紧缺矿种,也是全世界最走俏的金属商品,世界各国都在积极寻找新的银矿床。陆相火山岩 次火山岩型银矿床是最重要的银矿工业类型,多产于大陆板块边缘陆相火山岩广泛分布地区。根据元素组合可将银矿床分为单银型、金银型、银铅锌型和铜银型等等。它们在空间分布上与莫霍面有一定关系。单银型矿床多产于莫霍面比较浅的地区,铜银型矿床一般分布在莫霍面相对比较深的地区,金银型矿床和银铅锌型矿床通常置于单银型 铜银型中间地区。金银型与银铅锌型相比,前者分布地区的莫霍面深度比后者可能相对浅一些。     

Trisomy 16 fetus surviving into the second trimester

A 27-year-old gravida 4, para 3 was found to have anhydramnios at 14 weeks' gestation following a size/date discrepancy noted at her routine prenatal visit. A detailed ultrasound revealed multiple fetal anomalies including congenital heart defect, chest hypoplasia, and bilateral dysplastic kidneys. Karyotype revealed trisomy 16 in 15/15 cells from a tissue specimen obtained from the fetal cord insertion site following elective pregnancy termination.     

Terminal deletion of Xp in a dysmorphic anencephalic fetus

We report an anencephalic fetus with acrania, cervicodorsal rachischisis, and a 46,X,del(X)(p22·1) karyotype. Necropsy revealed a left diaphragmatic hernia, ipsilateral lung hypoplasia, and intestinal malrotation. The fetus also had horseshoe kidneys and adrenal gland hypoplasia with absence of the fetal zone.     

Artificial instillation of amniotic fluid as a new technique for the diagnostic evaluation of cases of oligohydramnios

In cases of severe oligohydramnios, sonographic diagnosis is hampered by poor visibility and by increased fetal flexion. Therefore, 74 artificial instillations of amniotic fluid were performed in 50 pregnancies by sonographic guidance. Fetal diseases included Potter's syndrome, obstructive uropathy, cystic kidneys (20 cases); Meckel's syndrome, C. de Lange syndrome, cytomegaly fetopathy (1 case each); VATER association (2 cases); triploidy (5 cases); severe intrauterine growth retardation (8 cases); and premature rupture of membranes (12 cases). By improved sonographic visibility and the observation of fetal behaviour (drinking, filling of stomach and bladder, voiding of bladder after artificial instillation of amniotic fluid), the fetal anatomy could be studied more accurately, malformations could be identified or excluded, and bladder function could be examined. Thus, differentiation between fetuses without functioning kidneys and those with severe intrauterine growth retardation becomes possible. Further, re-aspiration on the day following instillation permitted determination of the karyotype. Finally, the artificial instillation of amniotic fluid is a hazardous intervention (rupture of membranes, labour) and should be reserved only for a small number of selected cases with diagnostically unclear oligohydramnios. For these cases, it seems to be the method of choice permitting a variety of diagnostic information to be obtained.