Jens Peter Schenk

Current radiological management of intussusception in children

Intussusception is the most common abdominal emergency situation in infants and small children. There has been great progress in diagnostic and therapeutic management of intussusception. Ultrasound (US) has been shown to be the first-choice imaging technique in diagnosing intussusception for reasons of high accuracy, simultaneous exclusion of differential diagnoses, and disclosure of additional pathologies. Controversial opinions exist worldwide concerning the nonoperative treatment of intussusception in infants and children. Pneumatic reduction under fluoroscopic guidance and hydrostatic reduction under US monitoring are the preferred techniques. The aim should be a success rate of at least 90% in idiopathic intussusception. This review summarizes different types of intussusception and outlines diagnostic aspects as well as several treatment concepts.

Mesoblastic nephroma—A report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)†

Surgery alone is the appropriate first‐line treatment for patients with mesoblastic nephroma (MN). Nevertheless, there are reports of local recurrences and metastasis, especially in the cellular subtype. The authors evaluated the outcome of patients with MN who were enrolled in either the International Society of Pediatric Oncology (SIOP) 93‐01/GPOH or the SIOP 2001/GPOH Nephroblastoma Study and Trial.

Sexual Pseudo-Precocity Caused by a Somatic Activating Mutation of the LH Receptor Preceding True Sexual Precocity

Aim: We describe the clinical features of a 6-year-old boy with sexual precocity caused by a somatic activating mutation of the luteinizing hormone (LH) receptor gene preceding gonadotropin-releasing hormone (GnRH)-dependent sexual precocity. Study Design: Genomic DNA was extracted from the right testis and from the peripheral leukocytes followed by DNA amplification and sequencing of the LH receptor gene. We described the clinical characteristics including anthropometric parameters, bone age, and endocrine evaluation when the boy presented with sexual precocity. These data were compared with the clinical and hormonal evaluation after orchiectomy preceding GnRH-dependent sexual precocity and after subsequent treatment with GnRH agonist. Results: No mutation was found in the sequence of the LH receptor gene extracted from peripheral leukocytes. Interestingly, sequencing of the tumor LH receptor gene revealed a heterozygous mutation in exon 11 encoding a replacement of Asp578His. Despite normalization of plasma testosterone, true precocious puberty was triggered within a year. Conclusions: Inmales with GnRH-independent sexual precocity the presence of small testicular Leydig cell tumorous lesions harboring a somatic mutation of the LH receptor gene should be considered. A close follow-up of affected patients should be instigated in order to monitor recurrence or subsequent true precocity.

Measurement of real-time tissue elastography in a phantom model and comparison with transient elastography in pediatric patients with liver diseases

PURPOSE We aimed to determine the comparability of real-time tissue elastography (RTE) and transient elastography (TE) in pediatric patients with liver diseases. MATERIALS AND METHODS RTE was performed on the Elasticity QA Phantom Model 049 (Computerized Imaging Reference Systems Company Inc., Norfolk, Virginia, USA), which has five areas with different levels of stiffness. RTE measurements of relative stiffness (MEAN [mean value of tissue elasticity], AREA [% of blue color-coded stiffer tissue]) in the phantom were compared with the phantom stiffness specified in kPa (measurement unit of TE). RTE and TE were performed on 147 pediatric patients with various liver diseases. A total of 109 measurements were valid. The participants had following diseases: metabolic liver disease (n=25), cystic fibrosis (n=20), hepatopathy of unknown origin (n=11), autoimmune hepatitis (n=12), Wilsons disease (n=11), and various liver parenchyma alterations (n=30). Correlations between RTE and TE measurements in the patients were calculated. In addition, RTE was performed on a control group (n=30), and the RTE values between the patient and control groups were compared. RESULTS The RTE parameters showed good correlation in the phantom model with phantom stiffness (MEAN/kPa, r=-0.97; AREA/kPa, r=0.98). However, the correlation of RTE and TE was weak in the patient group (MEAN/kPa, r=-0.23; AREA/kPa, r=0.24). A significant difference was observed between the patient and control groups (MEAN, P = 5.32 e-7; AREA, P = 1.62 e-6). CONCLUSION In the phantom model, RTE was correlated with kPa, confirming the presumed comparability of the methods. However, there was no direct correlation between RTE and TE in patients with defined liver diseases under real clinical conditions.

A new pediatric liver transplantation program in Southern Germany. The Heidelberg experience.

Engelmann G, Schmidt J, Weitz J, Flechtenmacher C, Schenk JP, Weigand MA, Lenhartz H, Wenning D, Holland‐Cunz S, Hoffmann GF, Martin E, Büchler MW, Schmitt CP, Burdelski M, Meyburg J. A new pediatric liver transplantation program in Southern Germany. The Heidelberg experience.
Pediatr Transplantation 2010: 14: 12–18.

Limitations and opportunities of non-invasive liver stiffness measurement in children

Changes in liver structure are an important issue in chronic hepatopathies. Until the end of the 20th century, these changes could only be determined by histological analyses of a liver specimen obtained via biopsy. The well-known limitations of this technique (i.e., pain, bleeding and the need for sedation) have precluded its routine use in follow-up of patients with liver diseases. However, the introduction of non-invasive technologies, such as ultrasound and magnetic resonance imaging, for measurement of liver stiffness as an indirect marker of fibroses has changed this situation. Today, several non-invasive tools are available to physicians to estimate the degree of liver fibrosis by analysing liver stiffness. This review describes the currently available tools for liver stiffness determination that are applicable to follow-up of liver fibrosis/cirrhosis with established clinical use in children, and discusses their features in comparison to the “historical” tools.

Stridor, rezidivierende Bronchitiden und Gedeihstörung

DOI 10.1007/s10405-007-0151-2 Online publiziert: 12. April 2007