Extended Screening for Cystic Fibrosis-related Liver Disease Including Elastography in Children and Adolescents.

OBJECTIVES: Advances in treatment of cystic fibrosis (CF) have increased survival and thereby prevalence of patients with liver disease, making chronic liver disease one of the major complications of CF. We describe the prevalence of liver fibrosis, portal hypertension, and liver decompensation by extended screening for cystic fibrosis-related liver disease (CFLD) including ultrasound, elastography, and an extended panel of biochemical markers. METHODS: A cross sectional study of CFLD in all pediatric CF patients (1-18 years) from the Copenhagen CF Center. Screening for liver disease included physical examination, biochemical analysis, Vibration-Controlled Transient Elastography (FibroScan), conventional ultrasound, and Real-Time Shear Wave elastography (SWE). Patients were scored according to Williams ultrasound scoring scale (WUSS) within 6 months. RESULTS: A total of 84 consecutive patients (male sex 46.4%, median age 10.4 years) were included. Eight patients (9.5%) had both ≥2 abnormal results of sonographic methods and ≥2 abnormal biochemical results and were in this study categorized as having manifest CFLD. Manifest CFLD patients were significantly older and had a higher mean value of APRI, but no differences in gender, z-height, z-weight, z-BMI, FEV1%, or mean value of bilirubin or albumin were found. CONCLUSIONS: In total, 8 patients (9.5%) in this pediatric CF population were categorized as having CFLD according to both biochemical and sonographic tests. Consistency was found among the results of FibroScan and SWE. We suggest WUSS and either FibroScan or SWE, combined with GGT as diagnostic markers for CFLD.

Cancers of unknown primary origin (CUP) are characterized by chromosomal instability (CIN) compared to metastasis of know origin.

BACKGROUND: Cancers of unknown primary (CUPs) constitute ~5% of all cancers. The tumors have an aggressive biological and clinical behavior. The aim of the present study has been to uncover whether CUPs exhibit distinct molecular features compared to metastases of known origin. METHODS: Employing genome wide transcriptome analysis, Linear Discriminant Analysis (LDA) and Quadratic Discriminant Analysis (QDA), we defined the putative origins of a large series of CUP and how closely related a particular CUP was to corresponding metastases of known origin. LDA predictions were subsequently used to define a universal CUP core set of differentially expressed genes, that by means of gene set enrichment analysis was exploited to depict molecular pathways characterizing CUP. RESULTS: The analyses show that CUPs are distinct from metastases of known origin. CUPs exhibit inconsistent expression of conventional cancer biomarkers and QDA derived outlier scores show that CUPs are more distantly related to their primary tumor class than corresponding metastases of known origin. Gene set enrichment analysis showed that CUPs display increased expression of genes involved in DNA damage repair and mRNA signatures of chromosome instability (CIN), indicating that CUPs are chromosome unstable compared to metastases of known origin. CONCLUSIONS: CIN may account for the uncommon clinical presentation, chemoresistance and poor outcome in patients with CUP and warrant selective diagnostic strategies and treatment.

Cystic echinococcosis of the liver: experience from a Danish tertiary reference center (2002-2010).

BACKGROUND: Cystic echinococcosis (CE) of the liver can be treated with ultrasound-guided puncture, aspiration, injection, and re-aspiration (PAIR), with surgery and with benzimidazole derivatives. The aim of this study was to review available data concerning treatment modality and outcome for patients treated for CE of the liver in a Danish tertiary reference center. METHODS: A search was made for patients treated for CE infection between January 1, 2002 and January 1, 2010. All relevant patient records and radiology exams were scrutinized and all cysts were re-classified according to the WHO-IWGE, blinded as to which treatment the patient had received. PAIR was performed as a first choice treatment and surgery was reserved for cases where PAIR was impossible. Inactive cyst stages received medical treatment only. RESULTS: The search revealed 26 cases with confirmed CE of the liver. Nine patients underwent PAIR and nine patients surgery as a first choice treatment. Three patients were treated with PAIR secondary to surgery and one patient was treated with surgery secondary to PAIR. For all PAIR treatments, the success rate was 58% regardless of cyst stage and for surgery the success rate was 70%. The difference between the rates was not statistically significant (p = 0.67). CONCLUSION: CE is a rare disease in Denmark and our study is the first describing clinical management of CE in our institution.

Hemostatic multilayer coatings.

Spray layer-by-layer assembly is used to create hemostatic films containing thrombin and tannic acid. The spray assembly technique enables coating of porous and absorbent commercial gelatin sponges with these films. Coated sponges are able to promote instantaneous hemostasis in a porcine spleen bleeding model.

Influence of 2-(18F) fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography on recurrent ovarian cancer diagnosis and on selection of patients for secondary cytoreductive surgery.

The objective of this prospective study was to compare the sensitivities and the specificities of combined 2-(F) fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography (PET/CT), abdominal/transvaginal ultrasound (US), and CT for diagnosing recurrent ovarian cancer (OC) and to evaluate the influence of PET/CT on referral of patients with solitary recurrence to secondary cytoreductive surgery. From April 2005 to November 2007, 60 patients were consecutively included to PET/CT 68 times. The inclusion criteria were remission of 3 months or longer and recurrent OC suspected from physical examination, US, or increasing cancer antigen 125 (CA125) level (>50 U/mL or >15% above baseline level). Recurrent OC was diagnosed 58 times in 52 patients. The sensitivities of US, CT, and PET/CT for diagnosing recurrence were 66% (P = 0.003), 81% (P = 0.0001), and 97% (P < 0.0001), respectively. The specificity of US, CT, and PET/CT for diagnosing recurrence was 90%. Positron emission tomography/CT diagnosed recurrence in 19 (66%) of 29 patients without recurrence according to US and in 10 (50%) of 20 patients without recurrence after CT. Multiple recurrent tumors were found using PET/CT in 27 (69%) of 39 patients with solitary tumors on US and in 8 (42%) of 19 patients with solitary tumors on CT. We conclude that the diagnostic value of PET/CT for detecting recurrent OC was higher than those of US and CT and that PET/CT more accurately identified patients with solitary recurrence. However, prospective clinical trials are needed to specify the characteristics of patients most likely to undergo complete secondary surgery and to further clarify the role of PET/CT in selecting patients for secondary surgery.

Vaginal rupture caused by transvaginal ultrasonography in follow-up for ovarian cancer.

Vaginal rupture is a rare complication of hysterectomy. It is, among others, related to age and the incidence is higher in postmenopausal women. The rupture can occur spontaneously or in relation to clinical follow-up. In ovarian cancer the follow-up after surgery includes clinical examination, cancer antigen (CA)-125, and transabdominal and transvaginal ultrasonography. We experienced vaginal rupture in three patients with ovarian cancer. All patients had undergone surgery for ovarian cancer and were receiving chemotherapy. The rupture occurred shortly after transvaginal ultrasonography, performed by separate radiologists specialised in ultrasonography. All patients had acute surgery without any complications. Caution should be taken when performing transvaginal ultrasonography in hysterectomised patients and the complication of vaginal rupture should always be borne in mind.

[Spontaneous occlusion of a pseudoaneurysm in a thyroid cyst]. / Spontan okklusion af et pseudoaneurisme i en thyroideacyste.

A case of pseudoaneurysm formed in a thyroid cyst diagnosed by Doppler sonography is presented. Its genesis was regarded as idiopatic, and the condition resolved spontaneously less than four weeks after the initial diagnosis.

[Prenatal diagnosed hydronephrosis and other urological anomalies]. / Praenatalt diagnosticeret hydronefrose og andre urologiske anomalier.

By renal ultrasound examination, urological anomalies may be demonstrated in 1-2% of fetuses and in about 0.5% of newborns. Boys have about twice the frequency of girls. Surgical treatment is indicated in about one fourth of these urological anomalies. If all pregnant women in Denmark were to have fetal ultrasound examination of the kidneys and the urinary tract, about 70 children would be born each year with a prenatally diagnosed urological anomaly for which surgical procedure is or will be indicated. This paper provides Danish guidelines for prenatal diagnosis, follow-up and intervention in cases of urological anomalies and guidelines for post-natal diagnosis, follow-up and treatment of these anomalies, especially hydronephrosis.

A prospective randomized comparison of curved array and radial echoendoscopy in patients with esophageal cancer.

BACKGROUND: Both curved array and radial scanning echoendoscopy are used for locoregional staging of cancer arising in the esophagus or cardia. The accuracy of TNM staging of these malignancies by curved array and radial EUS was compared in a prospective, randomized study. METHODS: Patients with cancer of the esophagus or cardia were examined by both curved array and radial echoendoscopy in randomized order by the same endosonographer in an unblinded fashion. The staging results and the examination time for the two echoendoscopies were compared and statistically analyzed, and finally compared with surgical and histopathologic staging. RESULTS: A total of 104 patients underwent EUS; 36 had surgical resection of the tumor, 26 surgical exploration without resection, and 42 did not undergo surgery. Comparison of the TNM staging results for the two echoendoscopies gave high kappa values (T, 0.77; N, 0.75; M, 0.89), indicating excellent agreement. The accuracy of curved array echoendoscopy and radial echoendoscopy by component of the TNM staging system were, respectively, T, 72% and 73%; N, 70% and 77%; and M, 61% and 57%. Mean procedure time for the curved array and radial examinations was, respectively, 15 and 12 minutes (p<0.01). CONCLUSION: There was no clinically relevant difference between curved array and radial echoendoscopy in the staging of cancer of the esophagus or cardia. The choice of echoendoscope for TNM staging in patients with these malignancies is, therefore, a question of personal preference.