[Radiologic diagnostics of the gall bladder and bile ducts-part 1 : Imaging techniques-normal variants and congenital disorders-cholelithiasis]. / Radiologische Diagnostik der Gallenblase und der Gallenwege ­ Teil 1 : Bildgebende Verfahren ­ Normvarianten und Fehlbildungen ­ Gallensteinleiden.

Upper abdominal pain, icterus and cholestasis are the main symptoms leading to evaluation of the biliary tract. Since the advent of contrast-enhanced ultrasound techniques the accuracy of bile duct imaging has significantly improved. This is also true for computed tomography using isotropic secondary reconstructions as well as magnetic resonance imaging (MRI) including MR cholangiography (MRC), classically and after iv injection of hepatobiliary contrast agent. Diffusion-weighted imaging sequences have a recently proven ability for improving biliary tract imaging. These technical improvements provide the depiction of the non-dilated biliary tract system in diagnostic quality. Therefore, normal variants and congenital disorders of biliary tract anatomy are delineated as well as insights into the postoperative situs and complications related to surgery. Cholelithiasis is the most frequent disease of the gall bladder and biliary tract. Together with related complications, in the majority of cases it is also the reason for bile duct inflammation. Due to the immediate need for treatment special care has to be taken for hydrops of the gall bladder.

[Radiologic diagnosis of the gallbladder and bile ducts - part 2 : Acute and chronic cholecystitis, primary sclerosing cholangitis (PSC), benign and malignant masses of the biliary system]. / Radiologische Diagnostik der Gallenblase und der Gallenwege ­ Teil 2 : Akute und chronische Cholezystitis, primär sklerosierende Cholangitis, benigne und maligne Tumoren des Gallenwegsystems.

Upper abdominal pain, icterus and cholestasis are the symptoms leading to evaluation of the biliary tract. Together with its complications biliary stone disease is the main reason for inflammation of the biliary system. A distinction is made between acute and chronic variants. In chronic bile duct inflammation primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC) and more recently IgG4-associated sclerosing cholangitis are of particular importance. Besides benign and tumor-like-lesions, malignant entities as gallbladder carcinoma and cholangiocarcinoma (CCC) in its three locations have to be mentioned. Despite all recent improvements, specificity of bile-tract imaging still remains limited, especially regarding malignant masses. Therefore the final diagnosis is made in many cases by histological analysis.

[Reduction of treatment time for children in the trauma room care : Impact of implementation of an interdisciplinary trauma room concept (iTRAPS)]. / Verkürzung der Versorgungszeit im Trauma-Schockraum für Kinder : Auswirkungen der Einführung eines interdisziplinären Schockraumkonzepts (iTRAPS).

INTRODUCTION: In addition to infrastructural and conceptual planning, smooth interdisciplinary cooperation is crucial for trauma room care of severely injured children based on time-saving management and a clear set of priorities. The time to computed tomography (CT) is a well-accepted marker for the efficacy of trauma management. Up to now there are no guidelines in the literature for an adapted approach in pediatric trauma room care. METHODS: A step-by-step algorithm for pediatric trauma room care (Interdisciplinary Trauma Room Algorithm in Pediatric Surgery, iTRAPS) was developed within the framework of an interdisciplinary team: pediatric surgeons, pediatric anaethesiologists, pediatric intensivists and pediatric radiologists. In two groups of patients from January 2014 to April 2015 (group 1) and from July 2015 to January 2017 (group 2) process quality was monitored by the time required for trauma room treatment until the CT scan was performed and used as a surrogate marker. Inclusion criteria were patients aged 0-16 years, who were evaluated in a level 1 pediatric trauma room with an injury severity score (ISS) ≥8 and the necessity for a CT scan. RESULTS: Before (group 1) and after (group 2) implementation of iTRAPS 16 patients were included in each group. There were no significant differences between the age and the ISS in the two groups of patients. The required time for trauma room treatment was significantly reduced from an average of 33.6 min before to 15.2 min after implementation of iTRAPS (p < 0.01). DISCUSSION: The required time for the trauma care room treatment could be significantly reduced by more than half after the implementation of iTRAPS. The reasons were the interdisciplinary organization of the trauma room leadership, reorganization of patient transfer and improved briefing by emergency doctors. CONCLUSION: Besides a well-organized trauma team, it is essential that the trauma room workflow is adapted to the specific structure of the hospital. Despite the limitations of the study the data demonstrate that the trauma room workflow enables an efficient management. By the interdisciplinary reorganization of the pediatric trauma room treatment with improved structures and standardized processes, patient care was more effective with a significant reduction in the time required for trauma room treatment. The suggested iTRAPS concept could be used as a framework to establish individualized workflows for pediatric trauma room treatment in other hospitals. This algorithm should be supplemented by standardized operating procedures (SOPs) for the differentiated radiological diagnostic procedures in areas of traumatic brain injury (TBI), thoracic and abdominal trauma in children.

[Migraine in children and adolescents-brain and muscle? : Another example of why children are not small adults]. / Migräne im Kindes- und Jugendalter ­ Gehirn und Muskel? : Warum Kinder einmal mehr keine kleinen Erwachsenen sind.

Migraine as primary headache is a life-long disease which is relevant for the quality of life and is based on complex genetics. It often starts in childhood with symptoms typical for the specific age. These show different nuances compared to the migraine symptoms in adults, for example, regarding (bilateral/unilateral) localization of the acute migraine headache. Only over the course of years-during adolescence and young adulthood-do the more specific symptoms as defined by the International Classification of Headache Disorders (ICHD 3 beta) develop. In this article we focus on the clinical specifics of children and adolescents with migraine. We elaborately refer to the trigeminocervical complex (TCC) because it forms a conceptual bridge for the understanding of migraine, for psychoeducation, and for therapeutic options. We pragmatically discuss options and limits of treatments.

Inflammatory pseudotumor (IPT)-surgical cure of an inflammatory syndrome.

UNLABELLED: We report on four female adolescents, who presented with inflammatory symptoms. Extensive diagnostic workup revealed tumors on different locations. After surgical removal, clinical and laboratory signs of inflammation disappeared rapidly. On histology, the tumors showed a mixture of inflammatory cells characteristic of inflammatory pseudotumors in three of the patients. CONCLUSION: In patients with unclear inflammatory symptoms, inflammatory pseudotumor should be added to the differential diagnosis. WHAT IS KNOWN: • The inflammatory pseudotumor (IPT) is a mostly benign myofibroblastic tumor of the soft tissue and causes inflammatory symptoms. What is new: • IPTs have may wider than hitherto defined histologic features. Removal of IPT is curative.

Image-defined Risk Factors Correlate with Surgical Radicality and Local Recurrence in Patients with Neuroblastoma.

BACKGROUND: Neuroblastoma is the second most common solid pediatric tumor and the most common cancer to be detected in children younger than 12 months of age. To date, 2 different staging systems describe the extent of the disease: the International Neuroblastoma Staging System (INSS) and the International Neuroblastoma Risk Group Staging System (INRGSS). The INRGSS-system is characterized by the presence or absence of so called image-defined risk factors (IDRFs), which are described as surgical risk factors. We hypothesized that IDRFs correlate with surgical complications, surgical radicality, local recurrence and overall survival (OS). PATIENTS AND METHODS: Between 2003 and 2010, 102 patients had neuroblastoma surgery performed in our department. We analyzed medical records for IDRF-status and above named data. RESULTS: 16 patients were IDRF-negative, whereas 86 patients showed one or more IDRF. Intra- or postoperative complications have been reported in 21 patients (21%). 19 of them showed one or more IDRF and 2 patients were IDRF-negative (p=n.s.). Patients who suffered from intra- or postoperative complications demonstrated a decreased OS (p=0.011). Statistical analysis revealed an inverse correlation between the extent of macroscopical removal and IDRF-status (p=0.001). Furthermore, the number of IDRFs were associated with a decreased likelihood of radical tumor resection (p<0.001). 19 patients had local recurrence; all of them were IDRF-positive (p=0.037). CONCLUSIONS: Pediatric surgeons should consider IDRFs as a useful tool for risk assessment and therefore planning for neuroblastoma surgery.

[Voiding cystourethrography : Indications, fluoroscopy technique and radiation protection]. / Miktionszysturethrographie : Indikationen, Durchleuchtungstechnik und Strahlenschutz.

BACKGROUND: Voiding cystourethrography continues to be the gold standard in the diagnostics of a wide range of diseases of the urinary tract in children. MATERIAL AND METHODS: Indications, implementation of voiding cystourethrography in terms of preparation, materials used, dealing with the child and the parents as well as the standardized examination technique are presented. In particular, the technical aspects of fluoroscopy devices and criteria for good image quality are discussed. Case studies are used to illustrate the problems of frequent urological diseases. DISCUSSION: The three standard examinations for the detection of vesicoureteral reflux (VUR), radionuclide cystography, contrast-enhanced voiding urosonography and voiding cystourethrography are compared. Their potential for detecting VUR and additional urological pathologies is discussed in detail. Furthermore, the optimized examination technique of voiding cystourethrography is presented. The applicability of the current dose reference values of the German Federal Office for Radiation Protection (BfS) in the daily routine is discussed and the feasibility of the dose reference values is explained.

A human immunodeficiency syndrome caused by mutations in CARMIL2.

Human T-cell function is dependent on T-cell antigen receptor (TCR) and co-signalling as evidenced by immunodeficiencies affecting TCR-dependent signalling pathways. Here, we show four human patients with EBV+ disseminated smooth muscle tumours that carry two homozygous loss-of-function mutations in the CARMIL2 (RLTPR) gene encoding the capping protein regulator and myosin 1 linker 2. These patients lack regulatory T cells without evidence of organ-specific autoimmunity, and have defective CD28 co-signalling associated with impaired T-cell activation, differentiation and function, as well as perturbed cytoskeletal organization associated with T-cell polarity and migration disorders. Human CARMIL2-deficiency is therefore an autosomal recessive primary immunodeficiency disorder associated with defective CD28-mediated TCR co-signalling and impaired cytoskeletal dynamics.

Dissolution of calcified gallstones. Part I. Correlation of in vitro dissolution kinetics in methyl tert-butyl ether with patterns of calcification by computed tomography.

RATIONALE AND OBJECTIVES: The authors evaluated the relationship between stone computed tomography (CT) attenuation patterns and the kinetics of dissolution with methyl tertbutyl ether (MTBE). METHODS: Single moderately and heavily calcified gallstones from 40 patients were selected from a gallstone library and classified for pattern of calcification by in vitro CT scan (dense, rim, core, and laminated). Each stone was placed in a 10-mL aliquot of MTBE for 24 hours. Stone residue was blotted dry and weighted at 8, 16, and 24 hours. Results were normalized with respect to stone size. RESULTS: Only 1 of 40 (4%) specimens dissolved to particulate matter that was smaller than 2 mm. All (6 of 6) stones that were densely calcified showed virtually no dissolution. The rate of gallstone dissolution varied temporally within the rim, core, and laminated stone categories and was related to the composition of the layer exposed to the solvent at any given time. CONCLUSION: The success and rate of dissolution may be predicted by the pattern of calcification as determined by computed tomography (CT).

Dissolution of calcified gallstones. Part II. Evaluation of edetic acid preparations for dissolution of residue after in vitro methyl tert-butyl ether treatment.

RATIONALE AND OBJECTIVES: The authors assessed the potential of edetic acid (EDTA) preparations to dissolve the residue of calcified gallstones partially treated with methyl tert-butyl ether (MTBE). METHODS: Nineteen triplets (57 gallstones) were submitted to dissolution in EDTA, urea-EDTA, and an MTBE control for 48 hours after initial partial dissolution in MTBE for 24 hours. Results were compared with findings at specimen computed tomography and crystallographic analysis. All data were corrected for differences in stone size. RESULTS: In all three treatment groups (EDTA, urea-EDTA, MTBE), almost identical dissolution outcomes were observed within each triplet. Most triplets that dissolved displayed a laminated or a core-calcification pattern and consisted primarily of cholesterol. Specimens that dissolved poorly in all three groups displayed dense calcifications or thick calcified rims and were classified as pigment stones. CONCLUSION: Because no statistically significant differences in dissolution were found among the EDTA, urea-EDTA, and MTBE treatments, we conclude that EDTA preparations are not superior to the continued use of MTBE for dissolution of residue after initial MTBE treatment.

Rupture and aging of silicone gel breast implants.

This retrospective study evaluated aging and rupture of silicone gel breast implants in 31 women. The implants were removed at a large multispecialty clinic from 1987 to 1990. The implants ranged in age from 1 to 17 years. Of the 51 implants removed, 27 were ruptured, 7 were leaking, and 17 were in good condition. Common reasons for implant removal were discomfort, firmness, or a mass adjacent to a ruptured implant. Injury to the breast (trauma or mammography) led to a removal in only 4 patients. Closed capsulotomies were common in both ruptured and intact groups. The number of intact implants declined over time. All implants older than 10 years were leaking or ruptured. There was a positive correlation between the duration of implantation time and the number of ruptured and leaking implants.

[Inflammatory diseases of the gall bladder and biliary system. I. Imaging--cholelithasis--inflammation of the gall bladder]. / Entzündliche Erkrankungen der Gallenblase und der Gallenwege. Teil I: Bildgebende Verfahren--Cholelithiasis--Entzündungen der Gallenblase.

Cholelithiasis is the most common affliction of the gallbladder and biliary tract. Including its complications, gallstone disease represents the basis for cholecystitis and cholangitis in the majority of cases. Inflammatory diseases of the biliary system are divided into acute and chronic forms originating from the gallbladder as well as from the biliary tract. Although acute calculous cholecystitis is the most common form, gangrenous, and emphysematous inflammation of the gallbladder as well as gallbladder empyema are included in this group of diseases. In the chronic forms, calculous and acalculous inflammation is also differentiated. Recent developments in cross-sectional imaging in sonography, computed tomography, and magnetic resonance imaging offer numerous tools for depicting the biliary system with high diagnostic accuracy. Invasive imaging modalities of the biliary system are mainly used for therapeutic aspects.

[Inflammatory diseases of the gall bladder and biliary system. II. Acute and chronic inflammation of the biliary system--primary biliary cirrhosis]. / Entzündliche Erkrankungen der Gallenblase und der Gallenwege. Teil II: Akute und chronische Entzündungen der Gallenwege -- Primär Biliäre Zirrhose.

Inflammatory diseases of the biliary tract are divided into acute and chronic forms. Acute inflammation of the biliary tree is ascending cholangitis. Among chronic forms of the disease, primary sclerosing cholangitis, parasitic infection of the biliary tract, AIDS cholangiopathy, and chemotherapy cholangitis are recognised. Primary biliary cirrhosis and chronic inflammation of the papilla are also categorized within this group of inflammatory diseases of the biliary tree. Cross-sectional imaging modalities, such as sonography, computed tomography and magnetic resonance imaging, offer numerous possibilities for depicting the biliary system with high diagnostic accuracy. In contrast to the gallbladder, invasive imaging modalities of the biliary tree are also used for diagnostic purposes in addition to their therapeutic aspects.

Gastric ulceration as a complication of hepatic artery infusion chemotherapy.

Based upon the cases reported in this paper and upon a literature review encompassing 1,783 patients having received HAIC, it is our opinion that upper gastrointestinal tract ulceration is a potentially significant complication of hepatic artery infusion chemotherapy. In addition, we believe that the mechanism responsible for HAIC-induced ulceration often involves direct perfusion of the gastric arterial supply with chemotherapeutic agents. The occurrence of ulcerations in patients with surgically placed catheters is disconcerting, and does not allow for any apparent easy method of alleviating HAIC-induced ulceration. Clinicians should be alerted to the possibility of gastric and duodenal ulceration in patients treated with HAIC, and should afford particular attention to gastrointestinal symptoms during hepatic artery infusion chemotherapy.

Biosynthesis of taxol: enzymatic acetylation of 10-deacetylbaccatin-III to baccatin-III in crude extracts from roots of Taxus baccata.

The biosynthesis of taxol is a multistep process. One intermediate reaction is the acetylation of 10-deacetylbaccatin-III (10-DAB) to baccatin-III, an assumed precursor of taxol. Here we describe the cell free acetylation of 10-DAB in crude extracts from roots of Taxus baccata saplings using 14C-or 3H-labeled acetyl-coenzyme A as the acetyl donor. The reaction is strictly dependent on the addition of 10-DAB and is specific for the 10-hydroxyl group of the taxane ring. Formation of radiolabeled baccatin-III was confirmed by co-chromatography of the labeled product with authentic baccatin-III in different TLC-systems and HPLC. Furthermore, the acetylation product showed an identical UV spectrum as authentic baccatin-III. Crude extracts from cambium of stems yielded three- to fivefold lower activity. This is in agreement with our finding that the taxol titer in roots was considerably higher than that in cambium.

[Differential radiologic diagnosis of HIV-associated lung diseases]. / Radiologische Differentialdiagnostik HIV-assoziierter Lungenerkrankungen.

In 1993 in Germany an estimated number of 50,000-70,000 individuals were infected with the human immunodeficiency virus (HIV), WHO estimations ranged up to 14 million HIV-positive individuals including 1 million children. AIDS-related diseases frequently occur in the lung. 65% of all AIDS-defining illnesses begin with life-threatening pulmonary infections. Most frequently HIV-positive patients present with Pneumocystis carinii pneumonia, followed by atypical mycobacteriosis, bacterial infections, Kaposi's sarcoma and non-Hodgkin lymphoma. The purpose of this article is to identify pulmonary HIV-associated diseases by focusing on radiological patterns and correlating them with clinical findings.