Prenatal diagnosis and postnatal outcome of fetal congenital knee dislocation: systematic review of literature.

OBJECTIVES: Congenital knee dislocation (CKD) is a rare condition, affecting 1 in 100 000 newborns. Its prenatal diagnosis is challenging and not well described in the literature, especially when it appears isolated and not as part of a complex malformation or syndromic pattern. The purpose of this study was to provide a comprehensive review of the available literature on the prenatal diagnosis and postnatal outcome of CKD and to summarize the current evidence on this topic. METHODS: A systematic review of the literature on the prenatal diagnosis of CKD was performed in PubMed, Scopus and EMBASE. A predefined combination of specific keywords was used, focusing on intrauterine manifestations, diagnostic methods, prenatal behavior, postnatal treatment and neonatal outcome as well as long-term outcome in terms of ambulation, motion and joint stability. The quality of studies was assessed using the National Institutes of Health tool for quality assessment of case series. A summary of results was carried out providing proportions and rates of diagnostic and prognostic features associated with this rare condition. RESULTS: In total, 20 cases were retrieved for analysis, of which 19 were obtained from the identified eligible studies (n = 16) and one was an unpublished case from our center. The median gestational age at prenatal diagnosis, which was made using ultrasound in most cases, was 20 weeks (range, 14-38 weeks). Bilaterality was observed in 11/20 (55%) cases. The condition was isolated in 7/20 (35%) cases and associated with other anomalies in 13/20 (65%) cases. An association was observed with oligohydramnios (4/20 (20%)), and an invasive procedure was performed in 13/20 (65%) cases, including 11 cases with an invasive procedure performed for diagnostic purposes. Genetic testing was normal in all isolated cases for which information was available (4/7), while a genetic syndrome was present in 10/13 (77%) non-isolated cases (Larsen, Noonan, Grebe, Desbuquois or Escobar syndrome). There were seven terminations of pregnancy, of which six were performed in cases with associated anomalies and one in an isolated case, 11 cases of postnatal survival, one case of intrauterine death and one of neonatal death. The fetal and neonatal deaths occurred in cases with associated anomalies or abnormal genetic findings. Postnatal treatment was mostly conservative, with only two reports (18% of the 11 surviving neonates) of surgical intervention, both in cases with associated anomalies. Postnatal follow-up was up to 1 year in most cases, and motor outlook appeared normal in all isolated cases. CONCLUSIONS: CKD is a rare fetal anomaly with a prenatal diagnosis achievable from the early second trimester, for which a favorable outcome can be expected when no associated anomalies are present. Prenatal diagnosis should include detailed ultrasound assessment and amniocentesis for extensive genetic studies, particularly in non-isolated cases. Early postnatal treatment achieves success in most cases without surgical intervention and leads to a normal motor outlook. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Pulmonary Langerhans cell histiocytosis: the many faces of presentation at initial CT scan.

OBJECTIVES: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Chest computed tomography (CT) allows a confident diagnosis of PLCH only in typical presentation, when nodules, cavitated nodules and cysts coexist and predominate in the upper and middle lungs. METHODS: This article includes a pictorial essay of typical and atypical presentations of PLCH at initial chest CT. Various appearances of PLCH are illustrated and possible differential diagnosis is discussed. RESULTS: PLCH can present with some aspecific features that may cause diagnosis of the initial disease to be overlooked or other pulmonary diseases to be suspected. In cases of nodule presentation alone, the main differential diagnosis should include lung metastasis, tuberculosis and other infections, sarcoidosis, silicosis and Wegener's disease. In cases of cysts alone, the most common diseases to be differentiated are centrilobular emphysema and lymphangiomyomatosis. Clinical symptoms are usually non-specific, although a history of cigarette smoking, coupled with the presence of typical or suggestive findings at imaging, is key to suspecting the disease. Atypical presentations require surgical biopsy for diagnosis. CONCLUSIONS: The radiologist should be familiar with PLCH imaging features to correctly diagnose the disease or need for further investigation. TEACHING POINTS: • PLCH is a rare interstitial smoking-related disease that usually affects young adults. • The typical first CT shows a mix of nodules, cavitary nodules and cysts in the upper-middle lungs. • Atypical appearance, either cysts or nodules alone, mandates that other diagnoses be considered. • Lung cystic involvement correlates with lung function abnormalities and predicts functional decline. • Integration of the clinical history and imaging results is key to diagnosis.

Is there a role of percutaneous drainage in non-parasitic splenic cysts? Case report.

Pathogenesis, classification and treatment of non-parasitic splenic cysts (NPSCs) are controversial. The utility of percutaneous aspiration of the cyst is not well understood. We report a case of a 32 year-old woman with a symptomatic giant epidermoid cyst of the spleen treated with laparoscopic splenectomy. A percutaneous transcatheter drainage was performed under ultrasound guidance before surgical procedure in order to classify the type of cyst and to choose the best treatment.

Search for the pygmy dipole resonance in 68Ni at 600 MeV/nucleon.

The gamma decay from Coulomb excitation of 68Ni at 600 MeV/nucleon on a Au target was measured using the RISING setup at the fragment separator of GSI. The 68Ni beam was produced by a fragmentation reaction of 86Kr at 900 MeV/nucleon on a 9Be target and selected by the fragment separator. The gamma rays produced at the Au target were measured with HPGe detectors at forward angles and with BaF2 scintillators at backward angles. The measured spectra show a peak centered at approximately 11 MeV, whose intensity can be explained in terms of an enhanced strength of the dipole response function (pygmy resonance). Such pygmy structure has been predicted in this unstable neutron-rich nucleus by theory.

Probing the order-to-chaos region in superdeformed 151Tb and 196Pb nuclei with continuum gamma transitions.

The gamma decay associated with the warm rotation of the superdeformed nuclei 151Tb and 196Pb has been measured with the EUROBALL IV array. Several independent quantities provide a stringent test of the population and decay dynamics in the superdeformed well. A Monte Carlo simulation of the gamma decay based on microscopic calculations gives remarkable agreement with the data only assuming a large enhancement of the B(E1) strength for 1-2 MeV gamma rays, which may be related to the evidence for octupole vibrations in both mass regions.

Treatment of Budd-Chiari syndrome with transjugular intrahepatic portosystemic shunt (TIPS).

PURPOSE: This study was performed to evaluate the safety and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of patients affected by Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: From January 1999 to December 2006, 15 patients (seven male and eight female subjects, age range 7-52 years) with BCS uncontrolled by medical therapy were treated with TIPS placement. In seven cases BCS was idiopathic, in four it was caused by myeloproliferative disorders and in four by other disorders. One patient also had portal vein thrombosis. In 5/15 cases TIPS was created through a transcaval approach. Eight patients (53.4%) received a bare stent, and seven (46.6%) received a stent graft. The follow-up lasted a median of 29.4 (range 3.2-68) months. RESULTS: Technical success was achieved in all patients without major complications. TIPS was very effective in decreasing the portosystemic pressure gradient from 26.2+/-5.8 to 10+/-6.2 mmHg. All patients but two were alive at the time of writing. Acute leukaemia was the cause of the single early death and was unrelated to the procedure. The patient with portal vein thrombosis underwent thrombolysis before TIPS, but the vein occluded again after 3 weeks, and the patient died 6 months later. The other patients showed significant improvements in liver function, ascites and symptoms related to portal hypertension. Primary patency was 53.3%, and primary assisted patency was 93.3%. No patient required or was scheduled for liver transplantation. CONCLUSIONS: TIPS is an effective and safe treatment for BCS and may be considered a valuable alternative to traditional surgical portosystemic shunting or liver transplantation.

Simultaneous photoproduction of eta and pi0 mesons on the proton.

The analysis of the gammap-->etapi(0)p reaction has been performed using data from the GRAAL experiment. The total and differential cross sections and the beam asymmetry have been obtained from threshold up to 1.5 GeV of beam energy. The two resonances S11(1535) and Delta(1700) are expected to be excited in the intermediate states of this reaction. The results are used to test predictions based on the assumption that both resonances are dynamically generated from the meson-baryon interaction provided by chiral Lagrangians. The term involving the Delta(1700) excitation, followed by the decay into etaDelta(1232), is found to be dominant.

[Plastic industry and exposure to carcinogenic chemical agents: an Italian Multicentric Study in Lombardy]. / Stampaggio della plastica ed agenti chimici cancerogeni: l'esperienza dello studio PPTP-Plastica.

The potential carcinogenic risk at the workplaces is a primary interest of occupational health, but some questions are also controversially discussed. Particularly, in the plastic forming industry a great attention was directed to the hot processing and their possible exposure to monomers, some of which were classified as carcinogen by the International Agency for Research on Cancer (IARC) and/or the European Union (EU). In Lombardy, a study on occupational exposure to chemical carcinogens in the plastic forming industry was planned during last years. The aim was to recognize and promote preventive technical and medical solutions, basing on efficacy. By an investigation at workplace supported with standardized questionnaires, the presence of chemical carcinogens was registered in 59% of a representative sample of firms; but an effective possibility of exposure was found only for 34% of cases. The evaluation of exposure to monomers by air monitoring (acrylonitrile, 1,3-butadiene, styrene, formaldehyde), involving a representative sample of factory with ABS and formaldehydic resins processing, showed low level exposure, because the common hygienic prevention measures were applied; some particular occupation shoved greater exposure to formaldehyde.

Compound and rotational damping in warm deformed rare-earth nuclei.

The gamma decay in the quasicontinuum from selected configurations of the rotational nucleus 163Er has been measured with the EUROBALL array. A new analysis technique has allowed for the first time to directly measure the compound and rotational damping widths Gamma (micro) and Gamma (rot). Values of Gamma (micro) approximately 20 keV and Gamma (rot) approximately 200 keV are obtained in the spin region I approximately 30-40 variant Planck's over 2pi, in good agreement with microscopic cranked shell model calculations. A dependence of Gamma (micro) and Gamma (rot) on the K-quantum number of the nuclear states is also presented.

Double pi(0) Photoproduction on the Proton at GRAAL.

The double pi(0) photoproduction off the proton has been measured in the beam energy range of 0.65-1.5 GeV. The total and differential cross sections and the Sigma beam asymmetry were extracted. The total cross section measured for the first time in the third resonance region of the nucleon shows a prominent peak. The interpretation of these results by two independent theoretical models infers mostly the selective excitation of P11- and D13-nucleon resonances.

[Lombardy regional project: prevention of occupational tumors]. / Progetto regione lombardia: prevenzione dei tumori professionali.

In Lombardy, the regional project "Prevention of occupational cancers" is ongoing. The main objectives of this project are to identify work environments in which there might be a possible exposure to carcinogenic substances and to elaborate preventive measures. A casual sample of 250 working settings representing the different economic activities has been selected and evaluated. The 10% of the examined workplaces showed a possible exposure to chemical carcinogens. The most common carcinogens were trichloroethylene, preparation of plastics materials containing acrylonitrile-butadiene-stirene, formaldehyde, wood dust, hexavalent chromium, silica and polycyclic aromatic hydrocarbons. Galvanic industries, Bitumen production and placing companies and Plastics processing plants will be studied for environmental and biological monitoring and for the development of preventive measures.

[Hepatic and hepatocarcinoma magnetic resonance: comparison of the results obtained with paramagnetic (gadolinium) and superparamagnetic (iron oxide particles) contrast media]. / Risonanza Magnetica epatica ed epatocarcinoma: confronto fra risultati ottenuti con mezzo di contrasto paramagnetico (gadolinio) e superparamagnetico (particelle di ossido di ferro).

PURPOSE: To compare prospectively dynamic gadolinium (Gd)-enhanced with superparamagnetic iron oxide (SPIO)-enhanced MRI for the detection of hepatocellular carcinoma (HCC). MATERIAL AND METHODS: Twenty-five patients with histologically proven HCC and liver cirrhosis (28% of them in B or C Child class) underwent dynamic Gd-enhanced MRI and, a few days later, (mean interval: three days) SPIO-enhanced MRI. Only patients with availability of clinical and imaging follow-up for at least seven months were enrolled in this prospective study. Axial dynamic Gd-enhanced imaging was performed with T1 gradient-recalled echo (GRE) sequences. Both axial and sagittal SPIO-enhanced imaging were performed with respiratory triggered T2-weighted turbo spin-echo (TSE) and T1-T2*-weighted GRE sequences. MR images were reviewed by two independent radiologists. The readers scored each lesion for the presence of HCC and assigned confidence levels based on a five-grade scale: 1, definitely or almost definitely absent; 2, possibly present; 3, probably present; 4, definitely present; 5, definitely present with optimal liver/lesion contrast or good liver/lesion contrast and morphological signs (intact capsule, intranodular septa, extra-capsular infiltration), useful for locoregional treatment planning. A positive diagnostic value was assessed for scores of 3 or higher. RESULTS: Gd-enhanced and SPIO-enhanced MRI found 44 lesions. The combined use of TSE and GRE SPIO-enhanced sequences detected 11 more lesions (25% improvement in sensitivity) than Gd-enhanced MRI. One lesion (2.27%) was detected only with Gd-enhanced MRI. Eight of twelve lesions visible with a single contrast agent measured less than 1 cm in diameter. HCC detectability was 75% with Gd-enhanced MRI and 97.7% with SPIO-enhanced MRI. SPIO-enhanced T2-weighted TSE images showed significantly higher diagnostic value than SPIO-enhanced T1-T2*GRE images only in three cases, while nodule morphological characteristics (capsule, septa, different cell differentiation components) were better depicted by TSE images. DISCUSSION AND CONCLUSIONS: In our study the combined use of SPIO-enhanced T2-weighted TSE and T1-T2*-weighted GRE sequences showed higher sensitivity than gadolinium-enhanced GRE dynamic imaging (97.7% versus 75%). These results are at least partly related to our study conditions, that is: 1) MRI was performed with a 1T system, 2) both axial and sagittal SPIO-enhanced imaging were performed with respiratory triggered T2-weighted TSE and T1-T2*-weighted GRE, 3) there was a low freaquency of severe cirrhosis.

A case of totally cystic fibroepithelial polyp of the renal pelvis.

Fibroepithelial polyps are the most frequently observed mesenchymal tumors of the renal pelvis. We report on one case of fibroepithelial polyp of the renal pelvis with unusual CT findings of totally cystic structure with septations.

[Hernioplasty using a Lichtenstein polypropylene mesh]. / Ernioplastica con rete in polipropilene sec. Lichtenstein. Risultati a distanza.

"Tension free" technique with prosthetic mesh for inguinal hernia repair was introduced since 1988 in Authors' Institution. In a review of 98 hernioplasties performed, only one relapse was observed (around 1%), while prosthetic infection cases were never observed. The disorder most frequently complained by the patients is a pain in the pubic area, persisting even months after the operation. Therefore this technique seems to be reliable, safe, and easy. While waiting for a long-term follow up to confirm these results, the Authors however suggest to limit the indications for this technique avoiding the use of prosthetic material in young patients since Shouldice's hernioplasty assures a low percentage of relapse for them.

Osteoblastic cells from rat long bone. II: Adhesion to substrata and integrin expression in primary and propagated cultures.

Propagation in vitro of rat tibial osteoblasts (ROB) is accompanied by increased expression of the early osteogenic marker alkaline phosphatase (AP) and maturation of the osteogenic phenotype. In order to establish the pattern of the integrin expressed in ROB during progression to the mature osteoblastic phenotype, we have used biosynthetic, immunoblotting and immunohistochemical assays. We immunoprecipitated from osteoblasts, expanded for 1.5- and 7.5-doubling, alpha 5 beta 1, alpha v beta 3, alpha 3 beta 1, alpha 6 beta 1 and alpha 1 beta 1 integrin heterodimers; furthermore beta 5, alpha 2 and alpha 4 chains were detected by immunoblots and indirect immunofluorescence. alpha v, alpha 1, alpha 6 subunits in most cells, and beta 3 and beta 1 subunits in a minority, were found to be associated with adhesion plaques in osteoblasts of 1.5-, 4.5- and 7.5-doubling grown in the presence of FCS, while all other subunits stained diffusely all the cells. Adhesion to fibronectin (FN), laminin (LN), collagen type I (COL I) and III(COL III) by ROB at different doubling (1.5-11) was dependent on substratum concentration, and after 2.5 h at 55 nM 60% of the cells adhered to all substrata. Arg-Gly-Asp-Ser (RGDS) containing peptides inhibited adhesion of cells differentially, according to substratum; no dependence on extent of progation in vitro was observed. In conclusion, ROB cultured in vitro for 1.5- to 11-doubling had an unchanged pattern of expression of integrin subunits, heterodimer association and cellular distribution. Adhesion specificity and affinity were also unchanged. These results suggest that the phenotypic maturation, detected as an increase in AP expression, is not accompanied by major changes in the potential for cell-matrix interactions, and does not correspond to changes in the type of integrin subunits expressed by osteoblasts.

Molecular Probe Data Base (MPDB).

Molecular Probe Data Base contains detailed information on synthetic oligonucleotides with a sequence of up to 100 nucleotides. This database prevalently contains information related to human oligonucleotides used in diagnostics. Molecular Probe Data Base has been made available on-line through the Internet by means of Network Information Retrieval (NIR) tools since 1993. Two years ago, a collaboration with EMBL Data Library was also set up, so that the Molecular Probe Data Base has been integrated with other molecular biology data banks in the sphere of the SRS WWW network browser. In this paper, the most recent enhancements and the current status of the Molecular Probe Data Base are briefly presented.

CT demonstration of liquid intratumoral fat layering in a necrotic renal cell carcinoma.

We describe a case of clear-cell adenocarcinoma of the kidney with CT evidence of fat that contradicts the rule that radiologically demonstrable fat is absent in renal carcinoma. The cyst-like appearance, egg-shell parietal calcifications, and extrarenal development of the mass suggested a preoperatively incorrect diagnosis of teratoma.

[The evolution of the liver metastases from colorectal carcinoma treated with hepatic intra-arterial chemotherapy. The echographic and CT aspects]. / Evoluzione delle metastasi epatiche da carcinoma del colon-retto trattate con chemioterapia intra-arteriosa epatica. Aspetti ecografici e tomodensitometrici.

The response of liver metastases to chemotherapy relies mainly on quantitative US and CT investigations, the two techniques being indifferently used. The morphologic changes of metastatic lesions during treatment have received little attention and their significance is still questionable. Based on the review of 53 US and 41 CT examinations of 15 patients treated with hepatic arterial chemotherapy for colorectal liver metastasis, our study was aimed at assessing: 1) the relationship between US and CT measurements of metastasis response to chemotherapy and 2) changes in the US and CT patterns of liver metastases during treatment and the existence of specific patterns of favorable response to chemotherapy or of disease progression. We concluded that: 1) as to quantitative response to chemotherapy, US and CT, performed on 13 patients within 1 month, were in agreement in all but 1 case, 2) US and CT patterns of treated liver metastases were different in case of favorable response and of disease progression; lesion outline, homogeneity and calcifications are useful diagnostic criteria to this purpose, 3) liver perfusion abnormalities may occur at various times during and after chemotherapy; these ischemic lesions must be differentiated from new metastases.

Sonography after renal biopsy: assessment of its role in 230 consecutive cases.

Among 230 patients undergoing ultrasound (US) guided renal biopsy, 218 had postbiopsy sonography. Clinical records were reviewed to correlate symptoms to US findings. In each case of large hematoma (thickness above 1 cm), all postbiopsy sonographic studies were analyzed to look for findings indicative of unfavorable outcome. A total of 96 subcapsular/perirenal hematomas were found. Large hematomas were observed in 20 patients (20/230 = 8.7%), seven of these (3%) were severely symptomatic. In the absence of clinical signs of bleeding, no patient had clinical consequences. In the presence of clinical signs of bleeding, serious complications occurred only in patients with large hematomas. US thickness of retroperitoneal hematoma correlated to clinical outcome: whenever measured thickness was less than 2 cm, clinical evolution was very favorable, whereas a thickness above 2 cm was invariably associated to clinical signs of bleeding. In six of seven cases of thickness exceeding 3 cm, severe complications developed. An unfavorable evolution was associated with increasing thickness and an echogenicity inappropriate with respect to the time elapsed since biopsy. Hydroureteronephrosis, peritoneal effusion, and anomalous vascular images were indicators of deterioration. We conclude that sonography is indicated only for symptomatic patients and that the monitoring of both thickness and changing echogenicity of retroperitoneal blood collections supplements clinical follow-up.