[Testicular tumors in prepubertal boys-organ preservation possible more often than expected]. / Hodentumoren bei präpubertären Jungen ­ Organerhalt häufiger möglich als gedacht.

In prepubertal boys, testicular tumors are rare with an incidence between 2 and 5/million. In contrast to testicular tumors in adolescents and adults, more than 2/3 of these tumors are benign. Unfortunately, in Germany in most cases, only malignant tumors (usually yolk sac tumors) are reported to the study center (MAKEI IV and now V). Therefore, the incidence in Germany is unknown. Since the introduction of polychemotherapy in the 1970s, the prognosis of malignant testicular tumors has improved enormously and has become a curable disease, even in the case of recurrence. Today the orchiectomy, which was usually carried out in the past, appears to be no longer justified in most prepubertal boys due to the high incidence of benign tumors. It has been shown in various studies that organ-sparing surgery in germ cell tumors (epidermoid cysts, teratoma); gonadal stoma tumors (Sertoli, Leydig and granulosa cell tumors) and cystic lesions (intratesticular cysts and tubular ectasia of the rete testis) is reliable and safe. In cases with preoperative significantly increased AFP (caution: norm values not valid in the first year of life) and a clear testicular tumor in the ultrasound (yolk sac tumor) or if no testicular parenchyma is sonographically detectable, orchiectomy can still be carried out. Today orchiectomies in prepubertal boys should be an exception and the reasons for an orchiectomy must be well documented.

Region of interest-based versus whole-lung segmentation-based approach for MR lung perfusion quantification in 2-year-old children after congenital diaphragmatic hernia repair.

OBJECTIVE: With a region of interest (ROI)-based approach 2-year-old children after congenital diaphragmatic hernia (CDH) show reduced MR lung perfusion values on the ipsilateral side compared to the contralateral. This study evaluates whether results can be reproduced by segmentation of whole-lung and whether there are differences between the ROI-based and whole-lung measurements. METHODS: Using dynamic contrast-enhanced (DCE) MRI, pulmonary blood flow (PBF), pulmonary blood volume (PBV) and mean transit time (MTT) were quantified in 30 children after CDH repair. Quantification results of an ROI-based (six cylindrical ROIs generated of five adjacent slices per lung-side) and a whole-lung segmentation approach were compared. RESULTS: In both approaches PBF and PBV were significantly reduced on the ipsilateral side (p always <0.0001). In ipsilateral lungs, PBF of the ROI-based and the whole-lung segmentation-based approach was equal (p=0.50). In contralateral lungs, the ROI-based approach significantly overestimated PBF in comparison to the whole-lung segmentation approach by approximately 9.5 % (p=0.0013). CONCLUSIONS: MR lung perfusion in 2-year-old children after CDH is significantly reduced ipsilaterally. In the contralateral lung, the ROI-based approach significantly overestimates perfusion, which can be explained by exclusion of the most ventral parts of the lung. Therefore whole-lung segmentation should be preferred. KEY POINTS: • Ipsilaterally, absolute lung perfusion after CDH is reduced in whole-lung analysis. • Ipsilaterally, the ROI- and whole-lung-based approaches generate identical results. • Contralaterally, the ROI-based approach significantly overestimates perfusion results. • Whole lung should be analysed in MR lung perfusion imaging. • MR lung perfusion measurement is a radiation-free parameter of lung function.

High temporal versus high spatial resolution in MR quantitative pulmonary perfusion imaging of two-year old children after congenital diaphragmatic hernia repair.

OBJECTIVES: Congenital diaphragmatic hernia (CDH) leads to lung hypoplasia. Using dynamic contrast-enhanced (DCE) MR imaging, lung perfusion can be quantified. As MR perfusion values depend on temporal resolution, we compared two protocols to investigate whether ipsilateral lung perfusion is impaired after CDH, whether there are protocol-dependent differences, and which protocol is preferred. METHODS: DCE-MRI was performed in 36 2-year old children after CDH on a 3 T MRI system; protocol A (n = 18) based on a high spatial (3.0 s; voxel: 1.25 mm(3)) and protocol B (n = 18) on a high temporal resolution (1.5 s; voxel: 2 mm(3)). Pulmonary blood flow (PBF), pulmonary blood volume (PBV), mean transit time (MTT), and peak-contrast-to-noise-ratio (PCNR) were quantified. RESULTS: PBF was reduced ipsilaterally, with ipsilateral PBF of 45 ± 26 ml/100 ml/min to contralateral PBF of 63 ± 28 ml/100 ml/min (p = 0.0016) for protocol A; and for protocol B, side differences were equivalent (ipsilateral PBF = 62 ± 24 vs. contralateral PBF = 85 ± 30 ml/100 ml/min; p = 0.0034). PCNR was higher for protocol B (30 ± 18 vs. 20 ± 9; p = 0.0294). Protocol B showed higher values of PBF in comparison to protocol A (p always <0.05). CONCLUSIONS: Ipsilateral lung perfusion is reduced in 2-year old children following CDH repair. Higher temporal resolution and increased voxel size show a gain in PCNR and lead to higher perfusion values. Protocol B is therefore preferred. KEY POINTS: • Quantitative lung perfusion parameters depend on temporal and spatial resolution. • Reduction of lung perfusion in CDH can be measured with different MR protocols. • Temporal resolution of 1.5 s with spatial resolution of 2 mm (3) is suitable.

Prediction of chronic lung disease, survival and need for ECMO therapy in infants with congenital diaphragmatic hernia: additional value of fetal MRI measurements?

INTRODUCTION: The lung-to-head ratio (LHR), measured by ultrasound, and the fetal lung volume (FLV), measured by MRI, are both used to predict survival and need for extra corporeal membrane oxygenation (ECMO) in infants with congenital diaphragmatic hernia (CDH). The aim of this study is to determine whether MRI measurements of the FLV, in addition to standard ultrasound measurements of the LHR, give better prediction of chronic lung disease, mortality by day 28 and need for ECMO. MATERIALS AND METHODS: Patients with unilateral isolated CDH born between January 2002 and December 2008 were eligible for inclusion. LHR and FLV were expressed as observed-to-expected values (O/E LHR and O/E FLV). Univariate and multivariate analyses were performed. Receiver operating characteristic curves were constructed and areas under the curve (AUC) were calculated to determine predictive values. RESULTS: 90 patients were included in the analysis. Combined measurement of the O/E LHR and O/E FLV gave a slightly better prediction of chronic lung disease (AUC=0.83 and AUC=0.87) and need for ECMO therapy (AUC=0.77 and AUC=0.81) than standard ultrasound measurements of the O/E LHR alone. Combined measurement of the O/E LHR and O/E FLV did not improve prediction of early mortality (AUC=0.90) compared to measurement of the O/E LHR alone (AUC=0.89). An intrathoracal position of the liver was independently associated with a higher risk of early mortality (p<0.001), chronic lung disease (p=0.007) and need for ECMO therapy (p=0.001). DISCUSSION: Chronic lung disease and need for ECMO therapy are slightly better predicted by combined measurement of the O/E LHR and the O/E FLV. Early mortality is very well predicted by measurement of the O/E LHR alone. CONCLUSION: Clinical relevance of additional MRI measurements may be debated.

The impact of longitudinal intestinal lengthening and tailoring on liver function in short bowel syndrome.

INTRODUCTION: Short bowel syndrome is a functional or anatomic loss of major parts of the small bowel leading to severe malnutrition. The limiting factor for the survival of these patients remains parenteral nutrition-related liver damage leading to end-stage liver failure. Longitudinal intestinal lengthening and tailoring (LILT) has been proven to enhance peristalsis, to decrease bacterial overgrowth and to extend the mucosal contact time for the absorption of nutrients. The aim of this study was to show the impact of LILT on the development of parenteral nutrition-related liver damage. PATIENTS AND METHODS: A cohort of 55 patients with short bowel syndrome managed with LILT in our institution between 1987 and 2007 was retrospectively reviewed. LILT was performed at a mean age of 24 months (range 4 - 150 months). Mean follow-up time was 83.76 months (range 5 - 240 months). We obtained reliable data from 31 patients with regard to liver enzymes and function parameters in blood samples before LILT and at the present time. Liver biopsy was performed in 14 patients prior to LILT. RESULTS: Liver enzymes ALAT (mean 121 U/l), ASAT (mean 166 U/l) and bilirubin (mean 2.49 mg/dl) were elevated preoperatively in 27/31 children. After the lengthening procedure, ALAT (mean 50 U/l), ASAT (mean 63 U/l) and bilirubin (mean 1.059 mg/dl) normalized except in 5 of 8 patients who could not be weaned from parenteral nutrition after LILT. Liver function parameters such as the international normal ratio (INR) were slightly elevated in 5/31 patients. Albumin was generally low, probably due to parenteral nutrition. Liver biopsy was performed in 14 patients preoperatively, showing 4 patients with low-grade, 6 patients with intermediate and 4 patients with high-grade fibrosis. End-stage liver disease with cirrhosis was an exclusion criterion for LILT. All patients with liver fibrosis showed a normalization of liver enzymes when they were weaned from parenteral nutrition. But patients with higher grade liver fibrosis tend to develop more complications perioperatively. CONCLUSION: After LILT, all patients with liver fibrosis who could be weaned from parenteral nutrition showed a normalization of liver enzymes. Preoperative liver biopsy is mandatory in order to differentiate reversible liver fibrosis from end-stage liver disease. A higher grade of liver fibrosis and elevated INR has been shown to be a sensitive parameter for peri- and postoperative complications.

The influence of ticlopidine on the natural course of retinal vein occlusion.

A double-blind study was established to evaluate the platelet aggregation inhibiting effect of ticlopidine on the course of ocular vein occlusions. 35 patients with recent central retinal vein occlusions and 54 patients with retinal branch vein occlusions were treated for 6 months. Various ophthalmological and clinical-chemical parameters, including fluorescence angiography, were assessed. Compared with placebo therapy a significant improvement in visual acuity was observed with ticlopidine for branch vein occlusions; the same trend was seen for the central retinal vein occlusions. Increased ADP-induced platelet aggregation was frequently found: in 72% of the central retinal vein occlusions and 70% of the branch vein occlusions. For the Wu and Hoak test, these numbers were respectively 74% and 67%. Hypertension was seen in 55% of all occlusions, hyperlipaemia in 33%, and diabetes in 29%. The effect of ticlopidine was most pronounced in patients with increased platelet aggregation and least obvious in hyperlipaemia. Hypertension and diabetes did not apparently influence Ticlopidine's effects. It is concluded that the platelet aggregation inhibitor ticlopidine is effective in the treatment of recent ocular vein occlusions.

The synthesis of ninety proteins including actin throughout the HeLa cell cycle.

Abundant cytoplasmic proteins pulse-labeled with [35S]methionine at specific times throughout the HeLa cell cycle were analyzed with two-dimensional gel electrophoresis. More than 300 proteins could be resolved in this way. The frequency of appearance of label in the most abundant 90 proteins, ranging from 4% to less than 0.1% of the total methionine incorporated, was determined at six time points in the cell cycle. 84 of these proteins were made as a similar proportion of the total at all times during the cell cycle. A nonmuscle actin protein (spot 1) identified by molecular weight and isoelectric point represented 2-4% of the total methionine incorporated at all the time points. Only six proteins were found which varied by greater than fourfold during cell division, four appearing to represent a greater proportion of the total synthesis during the period at or immediately surrounding M (spots 31b, 44, 53, and 70d). Two appear to represent a smaller percentage of total synthesis during the early (spot 78) or the total (spot 74) G2 period.

Presumed histoplasmosis.

In a follow-up study of 30 eyes of 25 patients, three types of lesions could be distinguished: 1) the classical pigment ring, found in 13 eyes of 12 patients; 2) the cystous type, observed in 4 eyes of 4 patients, and 3) the multilocular type, found in 13 eyes of 9 patients. Up to the present, the cystous type of histoplasmosis has not been described.