Spontaneous rupture of the spleen caused by a <Emphasis Type="Italic">Bacillus</Emphasis> infection: Report of a case

We report a case of spontaneous splenic rupture (SSR) caused by a Bacillus species (sp.) infection. A 36-year-old man on warfarin therapy since an aortic valve replacement at the age of 13 was admitted to our hospital with a 3-week history of a high fever. He had been asymptomatic until 4 months previously, when he suffered a cerebral embolism despite adequate oral anticoagulation. Abdominal computed tomography revealed splenic infarctions, which resulted in splenic rupture 2 days later. After embolization of the splenic artery, splenectomy was successfully performed. Pathologic examination revealed splenic infarction, resulting from septic emboli, with associated rupture of the splenic capsule, but no abscess was found. Bacillus sp. was isolated from cultures of arterial blood preoperatively, and the excised splenic specimens, postoperatively. In addition to rupture of the suppurating intrasplenic vessels with hematoma formation, the anticoagulant therapy possibly contributed to distension of the intrasplenic hematoma.     

Mycotic abdominal aortic aneurysm infected by <Emphasis Type="Italic">Vibrio mimicus</Emphasis>: Report of a case

This report describes a case of a mycotic aneurysm of the infrarenal abdominal aorta infected by Vibrio mimicus, which is the only such case ever reported in the literature. The 80-year-old male patient was first treated for gastroenteritis for 7 days. Two months later, he was admitted to the hospital and the aneurysm was diagnosed by three-dimensional computed tomography. The aneurysm was excised and the restoration was done using a 16-mm Dacron straight graft. The cultivation of the excised aneurysm and the inflammatory fluid was positive for V. mimicus. The postoperative period was free of complications and the patient has remained asymptomatic during the 4-year follow-up.     

Type B aortic dissection associated with <Emphasis Type="Italic">Salmonella</Emphasis> infection

A 59-year-old man with a long history of hypertension and diabetes was admitted to our hospital with acute type B aortic dissection 14 days after the sudden onset of back pain. The dissecting descending thoracic aorta was enlarged to 5.2 cm in diameter, and laboratory tests showed an elevated white blood cell count (15 530/mm³) and an increased C-reactive protein level (19.2 mg/dl). Computed tomography performed 2 days after admission revealed rapid growth of the aortic dissection. Blood cultures obtained upon admission were positive for Salmonella. Impending rupture of the aortic dissection complicated by Salmonella infection was strongly suspected, and the patient underwent emergency surgery consisting of debridement and prosthetic graft placement covered by an omental flap. In this case, it is believed that insidious Salmonella aortitis caused acute type B aortic dissection.     

Acute glomerulonephritis in three siblings and suspected <Emphasis Type="Italic">emm</Emphasis>49-type <Emphasis Type="Italic">Streptococcus pyogenes</Emphasis> infection

Three siblings with poststreptococcal acute glomerulonephritis are presented. Streptococcal infection, impetigo, and pharyngitis preceded the acute glomerulonephritis. In one patient, emm49-type Streptococcus pyogenes was isolated, a strain which has not been reported as nephritogenic in Japan.     

Overwhelming septic infection with a multi-resistant<Emphasis Type="Italic"> Staphylococcus aureus</Emphasis> (MRSA) after total knee replacement

Introduction The incidence of early deep infection after arthroplasty of the knee is very low but could represent a serious future problem.Material and methods The authors report on a 71-year-old woman with gonarthritis who was supplied with a total knee endoprosthesis and developed a local infection followed by septic shock.Results The infection was evidently caused by a multiresistant Staphylococcus aureus (Staph. aureus). The primary clinical signs closely resembled a necrotizing fasciitis. Systemic and local application of vancomycin led to an improvement of the symptoms at secondary sites, but only the amputation of the primarily infected leg was lifesaving.Conclusion In order to prevent such events, the authors recommend a number of additional presurgical measures. Firstly, a swab from the nose and throat should be taken prior to an elective surgery in patients with elevated risk of immunodeficiency, for example in patients with diabetes. The diabetes should be adequately treated before an elective operation is undertaken. Secondly, an early punction of the knee joint should be carried out if there is any doubt regarding inflammation. Isolated infectious agents should be grounds for early revision, which should always be completed with a rinsing procedure and with adequate antibiotics. Immunotherapy should be taken into consideration. Antiepidemic measures are recommended in cases with known Staph. aureus.     

<Emphasis Type="Italic">PIK3CA</Emphasis>, <Emphasis Type="Italic">KRAS</Emphasis>, and <Emphasis Type="Italic">BRAF</Emphasis> mutations in intraductal papillary mucinous neoplasm/carcinoma (IPMN/C) of the pancreas

Background and aims Recent studies have reported high frequencies of somatic mutations in the phosphoinositide-3-kinase catalytic-α (PIK3CA) gene in various human tumors. Three hot-spot mutations in the exons 9 and 20 have been proven to activate the Akt signalling pathway. The Raf/MEK/ERK (mitogen-activated protein kinase) signal transduction is an important mediator of a number of cellular fates including growth, proliferation, and survival. The BRAF gene is activated by oncogenic RAS, leading to cooperative effects in cells responding to growth factor signals. Here we evaluate the mutational status of PIK3CA, KRAS, and BRAF in intraductal papillary mucinous neoplasm/carcinoma (IPMN/IPMNC) of the pancreas. Materials and methods Exons 1, 4, 5, 6, 7, 9, 12, 18, and 20 of PIK3CA, exons 1 of KRAS, and exons 5, 11, and 15 of BRAF were analyzed in 36 IPMN/IPMC and two mucinous cystadenoma specimens by direct genomic DNA sequencing. Results We identified four somatic missense mutations of PIK3CA within the 36 IPMN/IPMC specimens (11%). One of the four mutations, H1047R, has been previously reported to be a hot-spot mutation. Furthermore, we found 17 (47%) KRAS mutations in exon 1 and one missense mutation (2.7%) in exon 15 of BRAF. Conclusion This data is the first report of PIK3CA mutation in pancreatic cancer and it appears to be the first oncogene to be mutated in IPMN/IPMC but not in conventional ductal adenocarcinoma of the pancreas. Our data provide evidence that PIK3CA and BRAF contribute to the tumorigenesis of IPMN/IPMC, but at a lower frequency than KRAS. German Society of Surgery, Surgical Forum 2008, Best Abstracts This work was supported by the National Cancer Institute (NCI) Temin Award CA95434 and the NCI R01 CA109525 (GHS).     

<Emphasis Type="Italic">Candida albicans</Emphasis>-Infected Pancreatic Pseudocyst: Report of a Case

We report the case of a 48-year-old man with a pseudocyst infected by Candida albicans, and review the relevant literature. The patient was successfully treated by a Roux-en-Y cystojejunostomy of the pseudocyst and adjunctive therapy with amphotericin B. Candida species isolated from a pancreatic pseudocyst or abscess should be considered pathogenic, and treated aggressively.     

Glomerulonephritis induced by methicillin-sensitive <Emphasis Type="Italic">Staphylococcus aureus</Emphasis> infection

A 57-year-old woman developed exacerbation of atopic dermatitis, fever, and nephrotic syndrome with microscopic hematuria. By bacteriological study, methicillin-sensitive Staphylococcus aureus (MSSA) was detected from each culture of pharyngeal mucus, stool, and blood samples. Renal biopsy specimens showed endocapillary proliferative glomerulonephritis with massive IgA deposition in the mesangium and along the capillary loops. After antistaphylococcal therapy with antibiotics, MSSA was negative for each culture and urinary protein decreased. Nine months after the first renal biopsy, a re-biopsy was performed, which revealed apparent disappearance of both endocapillary cell proliferation and IgA deposition. It is known that methicillin-resistant S. aureus (MRSA) infection causes glomerulonephritis through T-cell stimulation by superantigen presented by MRSA. The present results suggest that not only MRSA but also MSSA can cause this type of glomerulonephritis.     

<Emphasis Type="Italic">WT1</Emphasis> and <Emphasis Type="Italic">NPHS2</Emphasis> mutations in Korean children with steroid-resistant nephrotic syndrome

Although several genetic causes of steroid-resistant nephrotic syndrome (SRNS) have been identified, occurrence of these genetic abnormalities appears to be influenced by race. Seventy Korean children (39 girls, 31 boys) with SRNS underwent analysis for mutations of WT1 and NPHS2. Although NPHS2 mutations were not present in any of the patients, two different intronic mutations of WT1, IVS9+4 C>T and IVS9+5 G>A, were detected in four patients (three girls, one boy). Among the four patients with mutation, two girls with a karyotype of 46,XY had complete XY gonadal dysgenesis, one girl with a karyotype of 46,XX had normal genitalia, and one boy with a karyotype of 46,XY had hypospadia. A kidney biopsy conducted in three of the four patients revealed focal segmental glomerulosclerosis. The incidence of WT1 mutations observed in this study was similar to that of previous reports. However, the incidence of NPHS2 mutations seems to be very rare in Korean children. Genetic diagnosis of WT1 mutations should be recommended for children with SRNS, especially in cases involving a female phenotype or males with genital anomalies.     

Intratumoral Heterogeneity in Microsatellite Alterations in <Emphasis Type="Italic">BRCA1</Emphasis> and <Emphasis Type="Italic">PTEN</Emphasis> Regions in Sporadic Colorectal Cancer

Background: Chromosome regions 17q21 (BRCA1) and 10q23 (PTEN) have been found deleted in colorectal cancer.Methods: We studied the frequency of loss of heterozygosity (LOH) in these 2 regions in 214 patients with only 1 sample per tumor and in 100 patients with several samples per tumor. Three microsatellite markers of each region were used for the LOH test. The polymerase chain reaction product was electrophoresed in 8% polyacrylamide gels, and band intensity was shown by silver staining.Results: The proportions of LOH in the two regions were 38.4% for 17q21 and 30.8% for 10q23 in the group of 214 and were 47.7% for 17q21 and 34.7% for 10q23 in the group of 110434_2003_Article_876. We found a high correlation between the LOH in both regions (P < .001), where 81% of LOH in 10q23 region was matched by concomitant LOH in 17q21. In the group of tumors with several samples (group of 100), 39% and 68% did not present LOH in the 17q21 and 10q23 regions, respectively, in all of their tumor samples. However, in the 20 patients with LOH in both regions in the group of 100 (several samples per tumor), all samples with LOH in 10q23 also had LOH in 17q21, whereas not all samples with LOH in 17q21 had LOH in 10q23.Conclusions: These results show that colorectal cancer is highly heterogeneous, at least for these tumors markers, and suggest a sequential acquisition pattern of these anomalies during tumor growth, in which changes in 17q21 could occur before those in 10q23.     

No evidence for genotype/phenotype correlation in<Emphasis Type="Italic"> NPHS1</Emphasis> and<Emphasis Type="Italic"> NPHS2</Emphasis> mutations

Primary steroid-resistant nephrotic syndrome (SRNS) is characterized by childhood onset of proteinuria and progression to end-stage renal disease. In 26% of cases it is caused by recessive mutations in NPHS2 (podocin). Congenital nephrotic syndrome (CNS) is caused by mutations in NPHS1 (nephrin) or NPHS2. In three families mutations in NPHS1 and NPHS2 had been reported to occur together, and these tri-allelic mutations were implicated in genotype/phenotype correlations. To further test the hypothesis of tri-allelism, we examined a group of 62 unrelated patients for NPHS1 mutations, who were previously shown to have NPHS2 mutations; 15 of 62 patients had CNS. In addition, 12 CNS patients without NPHS2 mutation were examined for NPHS1 mutations. Mutational analysis yielded three different groups. (1) In 48 patients with two recessive NPHS2 mutations (11 with CNS), no NPHS1 mutation was detected, except for 1 patient, who had one NPHS1 mutation only. This patient was indistinguishable clinically and did not have CNS. (2) In 14 patients with one NPHS2 mutation only (4 with CNS), we detected two additional recessive NPHS1 mutations in the 4 patients with CNS. They all carried the R229Q variant of NPHS2. The CNS phenotype may be sufficiently explained by the presence of two NPHS1 mutations. (3) In 12 patients without NPHS2 mutation (all with CNS), we detected two recessive NPHS1 mutations in 11 patients, explaining their CNS phenotype. We report ten novel mutations in the nephrin gene. Our data do not suggest any genotype/phenotype correlation in the 5 patients with mutations in both the NPHS1 and the NPHS2 genes.Members of the Study Group of the Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN) participating in this study: J. Thaarup (Aalborg, Denmark); P. Henning (Adelaide, Australia); I. Attrach (Aleppo, Syria); A. Bakkaloglu (Ankara, Turkey); C. Rudin (Basel, Switzerland); R. Bogdanovic (Belgrade, Yugoslavia); S. Briese, J. Gellermann, T. Lennert, U. Querfeld, Sacherer, M. Schürmann, and M. Zimmering (Berlin, Germany); C. Roth, C. Schröter, and B. Utsch (Bonn, Germany); Matthes (Bremen, Germany); A. Heilmann and G. Kalvoda (Dresden, Germany); F. Wegner (Düren, Germany); V. Schumacher (Düsseldorf, Germany); Bär, B. Bosch, M. Kamm, S.M. Karle, K. Nüsken, C. Plank, W. Rascher, and B. Zimmermann (Erlangen, Germany); K. E. Bonzel, M. Bald, P. Hoyer, and U. Vester (Essen, Germany); U. Neyer (Feldkirch, Austria); Rippel (Frankfurt, Germany); M. Brandis, A. Fuchshuber, K. Häffner, A. Kirchhoff, and M. Pohl (Freiburg, Germany); J. Steiss (Giessen, Germany); J.P. Haas (Greifswald, Germany); L. Patzer (Halle, Germany); M. Kemper, H. Altrogge, D.E. Müller-Wiefel, U. Peters, and K. Timmermann (Hamburg, Germany); J.H.H. Ehrich, H. Haller, and C. Strehlau (Hannover, Germany); M. Daschner, S. Hessing, Janssen, D. Kiepe, S. Köpf, O. Mehls, and B. Tönshoff (Heidelberg, Germany); F. Prüfer and L.B. Zimmerhackl (Innsbruck, Austria); U. John, J. Misselwitz, G. Rönnefarth, and J. Seidel (Jena, Germany); D. Blowey and J. Scheinman (Kansas City, Mo., USA); B. Beck, K. Frankenbusch, B. Hoppe, C. Licht, D. Michalk, T. Ronda, and L. Stapenhorst (Cologne, Germany); D. Drozdz and A. Pogan (Krakau, Poland); Froster, E. Vogel and S. Wygoda (Leipzig, Germany); R. Hettenger (Los Angeles, Calif., USA); H. Schriewer and H.-P. Weber (Lüdenscheid, Germany); R. Beetz (Mainz, Germany); M. Konrad (Marburg, Germany); H. Fehrenbach (Memmingen, Germany); M. Griebel and B. Klare (München, Germany); M. Bulla, S. Fründ, E. Kuwertz-Bröking, A. Schulze-Everding and Yelbuz (Münster, Germany); L. Monnens (Nijmegen, The Netherlands); J. Janda and T. Seemann (Prag, Czech Republic); G. Adomssent, G. Krüger, Lorenzen, J. Muscheites, H.-J. Stolpe and M. Wigger (Rostock, Germany); W. Sperl (Salzburg, Austria); R. Egger (Schaffhausen, Switzerland); V. Tasic (Skopje, Macedonia); M. Bald and H.-E. Leichter (Stuttgart); O. Amon (Tübingen, Germany); T. Arbeiter, C. Aufricht and K. Müller (Vienna, Austria); D. Bockenhauer and N. Siegel (New Haven, Conn., USA); and T. Neuhaus and A. Staub (Zürich, Switzerland)     

Role of glycemic elements of <Emphasis Type="Italic">Cynodon dactylon</Emphasis> and <Emphasis Type="Italic">Musa paradisiaca</Emphasis> in diabetes management

The study defined the scientific evaluation of glycemic elements of extracts of Cynodon dactylon and Musa paradisiaca. A dose of 500 mg/kg body weight (bw) of C. dactylon produced maximum falls of 23.2% and 22.8% in blood glucose levels of normoglycemic rats during studies of fasting blood glucose and glucose tolerance, respectively, whereas the same dose of M. paradisiaca produced a rise of 34.9% and 18.4%. In diabetic rats during glucose tolerance tests, a fall of 27.8% and a rise of 17.5% were observed with the same dose of C. dactylon and M. paradisiaca, respectively. Laser-induced breakdown spectroscopy used for detection of glycemic elements present in both the extracts indicated that C. dactylon was rich in magnesium (Mg), whereas M. paradisiaca was rich in potassium (K) and sodium (Na), comparatively, suggesting thereby the defined roles of these elements in diabetes management.     

Polymorphisms in <Emphasis Type="Italic">ALOX12</Emphasis>, but not <Emphasis Type="Italic">ALOX15</Emphasis>, Are Significantly Associated With BMD in Postmenopausal Women

The murine arachidonate 15-lipoxygenase gene (Alox15) has recently been identified as a negative regulator of peak bone mineral density (BMD). The human ALOX15 gene shares significant sequence homology with the murine Alox15 gene; however, the human arachidonate 12-lipoxygenase gene (ALOX12) is functionally more similar to the mouse gene. Multiple single-nucleotide polymorphisms (SNPs) in the human ALOX15 and ALOX12 genes have previously been reported to be significantly associated with BMD in humans. On the basis of these data, we carried out our own investigation of the human ALOX15 and ALOX12 genes and their relationship with hip and spine BMD parameters. The study population consisted of 779 postmenopausal women with a mean (± standard deviation) age of 62.5 ± 5.9 years at BMD measurement and was recruited from a single large general practice in Chingford, northeast London. Three SNPs from ALOX15 and five from ALOX12 were analyzed. None of the SNPs that we analyzed in ALOX15 were significantly associated with any of the BMD parameters or fracture data. However, we found that three SNPs from ALOX12, all previously associated with spine BMD in women, were significantly associated with spine and various hip BMD parameters in our cohort (P = 0.029–0.049). In conclusion, we found no association between polymorphism in ALOX15 and BMD phenotypes but were able to replicate previous findings that genetic variation in ALOX12 seems to play a role in determining bone structure in Caucasian women.     

Effects of <Emphasis Type="Italic">Lactobacillus casei</Emphasis> and <Emphasis Type="Italic">Bifidobacterium breve</Emphasis> on urinary oxalate excretion in nephrolithiasis patients

It had been suggested that lactic acid bacteria (LAB) may degrade oxalate in the intestinal lumen, reducing urinary oxalate excretion. We aimed to evaluate the effect of a LAB mixture containing Lactobacillus casei (LC) and Bifidobacterium breve (BB) (LC + BB) upon urinary oxalate reduction in stone-forming (SF) patients without hyperoxaluria under conditions of an oxalate-rich diet. After an oxalate restriction period (7 days washout), 14 SF patients consumed an oxalate-rich diet during 4 weeks (200 mg/day) and a lyophilized LC + BB preparation was given t.i.d. after meals during the last 2 weeks. Twenty-four-hour urine samples were collected for determination of oxalate, calcium, magnesium, citrate, sodium, potassium and creatinine at baseline, after 2 weeks (DIET) and 4 weeks (DIET + LC + BB). The mean urinary oxalate excretion was significantly higher after DIET versus baseline (27 ± 8 vs. 35 ± 11 mg/24 h), but the mean decrease was not significant between DIET + LC + BB and DIET periods (35 ± 11 vs. 33 ± 10 mg/24 h). Seven out of 14 patients presented a reduction in oxaluria after LC + BB versus DIET, being the reduction higher than 25% in 4, and up to 50% in 2 of them. The latter two patients were those who had presented the greatest increase in oxaluria in response to dietary oxalate. In conclusion, this mixture of L. casei and B. breve was shown to possess a variable lowering effect upon urinary oxalate excretion that may be dependent on dietary oxalate intake.     

Radiation induces<Emphasis Type="Italic"> p53</Emphasis>-dependent cell apoptosis in bladder cancer cells with wild-type-<Emphasis Type="Italic">p53</Emphasis> but not in<Emphasis Type="Italic"> p53</Emphasis>-mutated bladder cancer cells

Purpose. It has been reported in several studies that the absence in cancer cells of the p53 tumor suppressor gene, mutations of which are frequently found in bladder cancer, increases their resistance to ionizing radiation. Other studies, however, suggest that mutations of the p53 gene could increase the radiosensitivity of cancer cells, although the evidence is still inconclusive. In the present study, we investigated the relationship between p53 status and radiation response in five different bladder cancer cell lines. Materials and Methods. Five different human bladder cancer cell lines (KK47: with wt-p53, RT4: with wt-p53, T24: with mutated p53, 5637: with mutated p53, UM-UC-3: with mutated p53) were used in the study. Cells were irradiated with 0, 2, 4, 6 or 8 Gy, then trypsinized and re-plated for clonogenic survival assay, quantitative RT-PCR assay, flow-cytometry analysis and TUNEL assay. Results. The clonogenic assay demonstrated that KK47 and RT4 had significantly higher radiosensitivity than other cell lines. Quantitative RT-PCR analysis showed that radiation induced increased expression of p53, Bax, and p21 mRNA in KK47 and RT4. After irradiation, G1 cell-cycle arrest was observed in KK47 and RT4 under flow cytometry analysis, while T24, 5637, and UM-UC-3 showed an increase in the proportion of G2 cells. Increased cell apoptosis was also observed under TUNEL assay in KK47 and RT4, but not in other cell lines. Conclusions: It was demonstrated that ionizing radiation induces p53-dependent cell apoptosis in bladder cancer cells with wt-p53 but not in those with mutated p53.     

<Emphasis Type="Italic">Candida</Emphasis> in Acute Pancreatitis

Purpose A Candida infection of the pancreas, which previously was considered extremely unusual, has been increasingly reported in recent years. The present study was conducted with the aim of performing a cohort analysis of our patients with acute pancreatitis to find out the incidence, sites, and species of Candida involvement; and to evaluate the risk factors, severity, and course of illness of such patients. Methods A total of 335 patients with acute pancreatitis were investigated for a possible Candida infection of the pancreas from January 2000 to May 2003. The clinical records of all those patients who were positive for Candida spp. isolation from pancreatic tissue were analyzed. The clinical records of 32 more cases, randomly selected from the patients who were investigated for candidal pancreatitis but were negative for Candida spp., were also analyzed in order to compare their findings with those patients with a true Candida infection of the pancreas. Results A true or possible Candida infection was observed in 41 (12.2%) of those 335 patients and Candida tropicalis was the most common isolate (43.9%). Candida spp. were isolated from pancreatic necrotic tissue in 22 (6.6%) patients (true infection). A possible Candida infection (positive drain fluid effluents at least twice, without any Candida isolation from pre/per operative samples from pancreas) was seen in 19 (5.7%) patients. Candida was also isolated exclusively from the blood in another 19 patients with a clinical diagnosis of acute pancreatitis. A risk factor analysis showed that patients with severe injury to the pancreas, on prophylactic fluconazole, and after surgical intervention were significantly more prone to develop a Candida infection. Patients with a Candida superinfection also had a significantly increased hospital stay and higher mortality. Conclusion This study thus emphasizes the important role of Candida infection in patients with acute pancreatitis and demonstrates the need for early attention.     

Reduction in oxalate-induced renal tubular epithelial cell injury by an extract from <Emphasis Type="Italic">Quercus salicina</Emphasis> Blume/<Emphasis Type="Italic">Quercus stenophylla</Emphasis> Makino

Urocalun, a herbal medicine prepared from an extract of Quercus salicina Blume/Quercus stenophylla Makino (QS extract), has been clinically used for the treatment of urolithiasis in Japan since 1969. In the present study, the effects of QS extract on oxalate-induced cell injury and NADPH-induced superoxide anion (O(2) (-)) production in the injured cells were investigated. Oxalate-induced cell injury was assessed by mitochondrial reduction of 3-(4,5-dimethyl-2-thiazol)-2,5-diphenyltetrazolium bromide and leakage of lactate dehydrogenase into the extracellular fluid. When NRK-52E cells were injured by exposure to oxalate for 24 h, QS extract prevented the injury in a dose-dependent manner. In addition, QS extract suppressed the increase in NADPH-induced O(2) (-) production, or NADPH oxidase activity, in the homogenate of cells injured by oxalate exposure. These findings suggest that the reduction in oxalate-induced O(2) (-) production contributes to the cytoprotective effect of QS extract.     

Absence of mutations in the <Emphasis Type="Italic">HOXA11</Emphasis> and <Emphasis Type="Italic">HOXD11</Emphasis> genes in children with congenital renal malformations

Experimental studies have shown that homeobox genes are essential for the development of the kidney and urinary tract. Hoxa11/Hoxd11 double mutant mice demonstrate renal agenesis or hypoplasia. Since, to our knowledge, these genes have never been examined for alterations in humans with congenital anomalies of the kidney and urinary tract (CAKUT), we investigated whether mutations of HOXA11/HOXD11 genes are associated with non-syndromal congenital renal parenchymal malformations. DNA samples from 26 unrelated children with unilateral renal agenesis (URA), 20 with renal hypodysplasia (RHD) and 13 with multicystic dysplastic kidney (MCDK) were included in the study. Exons 1 and 2 of the HOXA11/HOXD11 genes were amplified individually by polymerase chain reaction (PCR) using 12 unique oligonucleotide primers. Single-strand conformation polymorphism (SSCP) analysis of overlapping polymerase chain reaction products was performed. SSCP analysis revealed no variant band shifts in the samples of the amplified segments of the 59 patients, suggesting lack of either mutation or polymorphisms. Our findings do not support the hypothesis that mutations in the HOXA11/HOXD11 coding regions are involved in the pathogenesis of human non-syndromal congenital renal parenchymal malformations. Further studies are necessary, since other genes known to affect nephrogenesis, as well as genetic and environmental factors, may be involved.     

<Emphasis Type="Italic">Echinococcus granulosus</Emphasis> infection: the challenge of surgical treatment

Background Cystic echinococcosis (CE) is a worldwide zoonosis caused by larval stages of the cestode Echinococcus granulosus. Surgery, chemotherapy, and interventional procedures are the therapeutic options. Surgery can cure the patient if the parasite is removed entirely. However, the technical procedures are inconsistent and comprise partial liver resection or opening of the parasitic cyst and removal of the parasite. Laparotomy is the most common approach. In selected cases laparoscopic methods are successful. Retrospective studies outweigh prospective ones by far. However, proper management gives favorable results.Methods We critically review the literature and present a brief summary of current surgical strategy and focus on issues relevant for surgeons: diagnosis, indication for medical treatment, indication for surgical treatment, surgical procedures, scolicidal agents, morbidity, mortality, recurrence, perioperative medication, standards.Results All surgical procedures aim at the complete removal of the parasite. Liver resection and pericystectomy are procedures that resect the closed cysts with a fairly wide safety margin. A meta-analysis shows the best results regarding lethality (1.2%), morbidity (11.7%), and recurrence rates (2%) for resective operations. However, most surgeons consider these methods as too radical for a benign disease. Procedures that remove the parasite and keep the pericyst (=cystectomy) are easier to carry out than resective ones. The meta-analysis presented revealed a lethality of 2%, morbidity of 23%, and recurrence rate of 10.4% for these operations. Omentoplasty is the option of choice for the management of the remaining cyst cavity. Despite alternative procedures surgery is the treatment of choice. Supportive measures comprise the use of scolicidal agents and postoperative benzimidazole administration. However, a critical review of the literature disclosed a lack of scientific confirmation of established treatment modalities and procedures. The results of ultrasound imaging were classified and correlated to the developmental phases of CE.Conclusions Cystectomy and omentoplasty for CE should be the standard surgical procedure because it is safe, simple, and effective and meets all criteria of surgical treatment for hydatid disease: entire elimination of the parasite, no intraoperative spillage especially by using a cone, and saving healthy tissue. Pericystectomy should be used for peripherally located liver cysts that are surrounded by parenchyma only partially. Ultrasonic classification of the parasitic lesion should be used as a guideline for therapeutic measures.