Tongue reduction in Beckwith–Wiedemann syndrome: outcome and treatment algorithm

Beckwith–Wiedemann syndrome is a rare congenital overgrowth disorder with macroglossia being one of the cardinal symptoms. In pronounced cases, macroglossia can lead to airway obstruction, musculoskeletal alterations and functional deficits. Surgical tongue reduction is performed at varying ages and with different techniques. This study evaluated perioperative complications, as well as long-term aesthetic and functional outcomes, in a large cohort. A total of 68 patients, treated either surgically or conservatively, were included. Depending on the severity of macroglossia, patients were divided into three groups to determine the treatment algorithm. Complications after surgical tongue reduction were prolonged intubation and revision due to dehiscence or haematoma. In the long term, no patient suffered from impaired sense of taste or paresthesia, although the shape of the tongue was disproportional in 85%. With the present treatment algorithm, operative tongue reduction exerts a positive influence on skeletal, dentoalveolar and functional development with sufficient long-term outcome and high grade of satisfaction of the patients. Supportive therapy in an interdisciplinary centre is of fundamental importance for both surgical and conservative treatment.     

Clinical outcomes of patients with suspected pulmonary embolism using 99Tcm-Technegas as a ventilatory agent for lung scanning.

Clinical outcome analysis was carried out in 175 of 206 consecutive patients referred for a lung scan with clinical suspicion of pulmonary embolism (PE). The follow-up time period ranged from 4 to 18 months. High-quality ventilation images corresponding to the six standard perfusion images were obtained using Technegas as a ventilatory agent. Lung scan reports showed that 22% of the patients had a high, 14% indeterminate, 18% low and 9% very low probability for PE and 37% were normal lung scans. These reports usually supported the referring clinicians' provisional diagnosis and were confirmed by the clinical outcome analysis of these patients giving an apparent sensitivity for the lung scan of 96% and specificity 93%. A strategy for lung scanning in PE is proposed.     

Periovulatory 17 beta-estradiol pattern in conceptional and nonconceptional cycles during menotropin treatment of anovulatory infertility

The value of multiple parameters in the prediction of fertile cycles was prospectively evaluated in 52 menotropin-induced cycles. The periovulatory pattern of estradiol (E2) was found to correlate with conceptional cycles. E2 levels greater than 500 pg/ml on the day of human chorionic gonadotropin administration (day 0) with a further increase on day +1 (high ascending pattern--A1) were found to have a 51% predictive value for fertile cycles. Twelve of the 17 fertile cycles had an A1, type of response (71%), whereas the overall incidence of an A1 pattern was 42% (22 of 52). No pregnancies have occurred with preovulatory follicles less than or equal to 14 mm in diameter. The number of preovulatory follicles, E2 level on day 0, and midluteal progesterone had no predictive value for fertile cycles.     

Modified properties of serum cholinesterases in primary carcinomas

Cholinesterases were characterized in the serum of 77 treated and 11 untreated patients having primary carcinomas of various tissue origins and 21 healthy volunteers which served as controls. In most of the samples, pseudocholinesterase (BuChE) accounted for almost all cholinesterase (ChE) activity and was inhibited by the organophosphorous poison tetraisopropyl pyrophosphoramide (iso-OMPA). In samples from the tumor-bearing patients, ChE degraded 733 +/- 59 nmole acetylcholine/h/mg protein, lower than the 960 +/- 175 nmole/hour/mg levels measured in controls. Tumor serum ChE exhibited elevated sensitivity to 1,5-bis-(4-allyldimethyl ammonium phenyl)-pentan-3-one dibromide (BW), the selective bisquaternary inhibitor of "true" acetylcholinesterase (AChE), with no correlation to age, sex, staging of tumor, presence of metastases or the specific treatment protocol, and with a different distribution pattern from the decrease in ChE specific activity or the sensitivity to iso-OMPA. In sucrose gradients, ChE sedimented as 12S in controls whereas in tumor serum samples from treated patients an additional component of 6 to 7 S, inhibited by both iso-OMPA and BW, also was detected. However, the ChE activity in serum of patients with diagnosed carcinomas before surgery and medical treatment appeared to be nondistinguishable from controls. These findings suggest that the modified properties of serum cholinesterases in carcinoma patients are not the result of the tumor itself, but that the common therapy protocols used in the treatment of primary carcinomas may cause the appearance of soluble ChE activity with properties of both AChE and BuChE, which accumulates in the serum.     

Kunstfehler. Ärzterecht

Ohne Zusammenfassung     

A controlled, double-blind, randomized study on the efficacy of Lactobacillus plantarum 299V in patients with irritable bowel syndrome.

BACKGROUND: Irritable bowel syndrome (IBS) is a widespread functional disorder of the digestive tract. Its aetiology is unknown and therapeutic options are limited. Recent reports suggest that probiotics may have a role in regulating the motility of the digestive tract. AIM: To assess the efficacy of Lactobacillus plantarum 299V (LP299V) in patients with IBS. PATIENTS AND METHODS: Forty patients were randomized to receive either LP299V in liquid suspension (20 patients) or placebo (20 patients) over a period of 4 weeks. Clinical examination was performed at baseline and at the end of the study. Additionally, patients assessed their symptoms by applying a scoring system. RESULTS: All patients treated with LP299V reported resolution of their abdominal pain as compared to 11 patients from a placebo group (P = 0.0012). There was also a trend towards normalization of stools frequency in constipated patients in six out of 10 patients treated with LP299V compared with two out of 11 treated with placebo (P = 0.17). With regards to all IBS symptoms an improvement was noted in 95% of patients in the LP299V group vs 15% of patients in the placebo group (P < 0.0001). CONCLUSIONS: LP299V seems to have a beneficial effect in patients with IBS. Further studies on larger cohorts of patients and with longer duration of therapy are required in order to establish the place of L. plantarum in the treatment of IBS.     

Hepatic insulin resistance in mice with hepatic overexpression of diacylglycerol acyltransferase 2

Mice overexpressing acylCoA:diacylglycerol (DAG) acyltransferase 2 in the liver (Liv-DGAT2) have been shown to have normal hepatic insulin responsiveness despite severe hepatic steatosis and increased hepatic triglyceride, diacylglycerol, and ceramide content, demonstrating a dissociation between hepatic steatosis and hepatic insulin resistance. This led us to reevaluate the role of DAG in causing hepatic insulin resistance in this mouse model of severe hepatic steatosis. Using hyperinsulinemic-euglycemic clamps, we studied insulin action in Liv-DGAT2 mice and their wild-type (WT) littermate controls. Here, we show that Liv-DGAT2 mice manifest severe hepatic insulin resistance as reflected by decreased suppression of endogenous glucose production (0.8 ± 41.8 vs. 87.7 ± 34.3% in WT mice, P < 0.01) during the clamps. Hepatic insulin resistance could be attributed to an almost 12-fold increase in hepatic DAG content (P < 0.01) resulting in a 3.6-fold increase in protein kinase Cε (PKCε) activation (P < 0.01) and a subsequent 52% decrease in insulin-stimulated insulin receptor substrate 2 (IRS-2) tyrosine phosphorylation (P < 0.05), as well as a 64% decrease in fold increase pAkt/Akt ratio from basal conditions (P < 0.01). In contrast, hepatic insulin resistance in these mice was not associated with increased endoplasmic reticulum (ER) stress or inflammation. Importantly, hepatic insulin resistance in Liv-DGAT2 mice was independent of differences in body composition, energy expenditure, or food intake. In conclusion, these findings strengthen the link between hepatic steatosis and hepatic insulin resistance and support the hypothesis that DAG-induced PKCε activation plays a major role in nonalcoholic fatty liver disease (NAFLD)-associated hepatic insulin resistance.     

Forces charging the orbital floor after fractures

The objective of this study was first to establish a method to measure forces and displacement of the orbital content in defects of the orbital floor in truncated fresh and unfixed heads and second to characterize reconstruction materials with regard to punctuation strength and compression.Orbital floor defects (10 × 20 mm and 15 × 20 mm; 3 mm behind the orbital rim) were prepared after Le Fort I osteotomy. The values of force and displacement were recorded in 6 freshly frozen human heads. In addition, the punctuation strength of 2 reconstruction materials (polydioxanone [PDS] foil and collagen membrane) was evaluated using a Zwick Z010 TN1 universal testing machine. The forces of the orbital content (28.41 [SD, 1.6] g) applied to the defects of 10 × 20 mm and 15 × 20 mm with an intact periorbita were 0.04 (SD, 0.003) N (0.0002 MPa) and 0.07 (SD, 0.02) N (0.0002 MPa), respectively, and with a split periorbita were 0.06 (SD, 0.03) N (0.0003 MPa) and 0.08 (SD, 0.06) N (0.00026 MPa), respectively. The displacement values without reconstruction materials of the 10 × 20-mm and 15 × 20-mm defects were 0.94 (SD, 0.7) mm and 1.2 (SD, 0.5) mm, respectively. The PDS foil could withstand forces of 118.9 (SD, 14.1) N (0.375 MPa), and the collagen membrane could withstand forces of 44.5 (SD, 5.3) N (0.14 MPa). This is the first study to report forces charging the orbital floor. The presented results support the use of PDS foils and collagen membranes as reconstruction materials for orbital floor defects, at least in smaller and medium-sized fractures.