Sven Starke

University of Leipzig · Department of Pediatric Oncology, Hematology and Blood Coagulation

Deficiency of adenosine deaminase 2 (DADA2) is a rare mendelian, autoinflammatory multi-organ disease. We report the case of a 3 8/12 year old female patient who was admitted with an acute brainstem stroke and was diagnosed with DADA2 by early initiation of exome sequencing. We recommend that DADA2 and a genetic workup should be taken into account,...

Aim In 2015, the revised International Pediatric Non-Hodgkin Lymphoma Staging System was published. It mentions [¹⁸F]-FDG-PET/MRI as the latest method to perform whole-body imaging. However, supporting data are pending. Our aim was to investigate the performance of whole-body [¹⁸F]-FDG-PET/MRI in pediatric non-Hodgkin lymphoma patients by using a l...

Background Short stature in pediatric patients has many different causes. Short stature in relation to intrauterine growth retardation (IUGR), developmental delay and/or specific stigmata should raise the suspicion of a syndromic cause.Objective This article aims to raise awareness of short stature syndromes, the diagnosis of which should be made a...

We determined the indication, outcome, and risk factors of single and multiple hematopoietic stem cell transplantation(s) (HSCT) in children and adolescents mostly with advanced disease. Forty-one out of 483 patients (8.5 %; median age 9 years) diagnosed at the University of Leipzig with hematological and oncological diseases required HSCT from 199...

Heme oxygenase (HO)-1 catalyzes the degradation of cytotoxic heme into biliverdin and blocks anti-tumor immune responses, thus protecting cancer against host defense. Whether this scenario also applies to neuroblastoma (NB), the most common extra-cranial solid childhood tumor, is not known. Here, we demonstrate for the first time a prognostic relev...

Evasion from the immune system has emerged as a novel hallmark in the genesis of neuroblastoma as well as in its progression. A profound knowledge of underlying immune regulatory mechanisms in neuroblastoma may be of vital importance to improve current and future immunotherapeutic approaches against this challenging childhood malignancy. This chapt...

Background Phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome (PHTS) is caused by germline mutations in the PTEN gene. Symptoms include cancer predisposition, immune deviations and lipomas/lipomatosis. No causal standard therapy is available. We describe a therapeutic attempt with the mammalian target of rapamycin (mTOR) inhibitor sirol...

Background. Cancer in childhood is rare, but nevertheless one of the most frequent causes of disease related death. Initial symptoms are often unspecific, frequently leading to a delay of cancer diagnosis. As a timely diagnosis can be crucial for the clinical outcome, our aim is to point out when unspecific symptoms should be considered suspect of...

The clinical course of a female patient affected by a progeroid syndrome with Restrictive Dermopathy (RD)-like features was followed up. Besides missing hairiness, stagnating weight and growth, RD-like features including progressive skin swelling and solidification, acrocontractures, osteolysis and muscular hypotension were observed until the patie...

Reliable diagnostic strategies for individuals with cancer demand practical methods for highly sensitive and specific detection of tumor cells. Amplification of genomic regions that include putative oncogenes is common in tumor cells of various types. Genomic array platforms offer the opportunity to identify and precisely map amplified genomic regi...

A very rare case of a congenital orbital fat herniation is demonstrated. Clinically, the child presented a progressive, small yellowish and mobile mass on the temporal orbital wall of her right eye. Magnetic resonance imaging showed a fat isointensive structure in T1-weighing without contrast enhancement. The possible aetiology of the herniation co...

Zusammenfassung Zu den in der pädiatrischen Onkologie wesentlichen höhergradigen gliomatösen Tumoren zählen das anaplastische Astrozytom, das maligne Hirnstammgliom sowie das Glioblastom. Trotz derzeitiger multimodaler Therapie, bestehend aus Tumorresektion, Bestrahlung und zytostatischer Therapie, ist die Prognose der betroffenen Kinder schlecht....

In response to stimuli that activate p53, cells can undergo either apoptosis or cell cycle arrest, depending on the precise pattern of p53 target genes that is activated. We show here that Zbtb4, a transcriptional repressor protein, associates with the Sin3/histone deacetylase co-repressor and represses expression of P21CIP1 as part of a heterodime...

Fifteen percent to 20% of human neuroblastomas show amplification of the MYCN oncogene physiologically located at chromosome 2p24-25, indicating an aggressive subtype of human neuroblastoma with a poor clinical outcome. Recent findings revealed that the structure of the amplicon differs interindividually and that coamplification of genes in telomer...