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Objective: According to Papoušek (2004), "disinterests in play" in early childhood is a possible early indicator of AD(H)D at school age. The phenomenon of disinterest in play is associated with psychological distress in parents and children as well as with disturbances in the parent-child-relationships Papoušek, 2003. We investigated the association between disinterest in play in early childhood and AD(H)D and their associations with maternal distress., Method: Mothers of 35 children with AD(H)D [age: M (SD)= 9.0 (1.1) years; males: 30 (85.7 %)] and mothers of 42 children without AD(H)D [age: M (SD) = 8.8 (1.0) years; males: 35 (83.3 %)] gave their written informed consent to participate in the study. Using a questionnaire on the ability to play during the first 4 years of age, subjects were grouped into 39 children with lower interest in play and 38 children with higher interest in play. Maternal stress was assessed using a German adaptation of the Parenting Stress Index Abidin, 1995., Results: The rate of AD(H)D and maternal stress was significantly higher in the group of children with lower interest in play. Mothers of children showing both AD(H)D and lower interest in play scored highest, mothers of children presenting neither behavior disorder scored lowest., Conclusions: The results agree with the assumption that disinterest in play in early childhood is associated with the development of AD(H)D and an additional stressor for mothers.

Objective: To present preliminary results of a pilot project on the prevention of alcohol-associated problems in adolescents with acute alcohol intoxication., Method: Questionnaires were filled in by 110 of 128 adolescents (85.9% response rate) who had been admitted to a hospital in Munich, Germany, between December 2007 and July 2008, because of alcohol intoxication. Data were obtained on sociodemographic characteristics, alcohol intoxication and drinking patterns, and were analysed using descriptive methods., Results: Half of these adolescents mainly suffered from moderate to severe degrees of alcohol intoxication. Drinking patterns leading to alcohol intoxication were characterized by relatively low drinking frequency interspersed by episodes of excessive alcohol intake (binge drinking). Thirteen (14.8%) of the adolescents reported previous hospital admissions for alcohol intoxication, nine (17.3%) did not know about the life-threatening danger of alcohol intoxication and twelve (22.2%) reported taking illegal drugs within the last 12 months., Conclusion: Adolescents who had been admitted to hospital because of alcohol intoxication had a drinking pattern which put them at a higher risk for alcohol intoxication and acute alcohol-related problems than adolescents in the general population. Re-admission to hospital within 12 months because of alcohol intoxication, revealing a lack of knowledge about the life-threatening danger of alcohol intoxications and of consuming illegal drugs, may indicate an increased risk for alcohol-related problems. This points to the need for preventive action in adolescents showing these indicators, a need that was met within this pilot project by brief intervention.

While infections with salmonella typhi may frequently result in encephalopathy, this complication is rare in infections with salmonella enteritidis and has been rarely reported. We report about a 21 months old girl which developed encephalopathy with convulsion, ataxia and cortical blindness following salmonella infection. Moreover, we show a summary of ten previous case reports.

Thromboembolic events in neonates are very rare. They are often associated with severe disease affecting the newborn or are secondary to central venous lines or arterial catheters. Most of the described cases of thromboses of the iliac or femoral arteries are associated with cardiac catheterisation or femoral invasive blood pressure monitoring. The relationship between single umbilical arteries and an increased incidence of structural and chromosomal anomalies is well known, but a higher rate of thromboembolic disease in infants with single umbilical arteries has not been described. Rt-PA (recombinant tissue plasminogen activator) has been successfully used in small studies and numerous case reports. To date controlled clinical trials giving guidelines for antithrombotic therapy using rt-PA are still lacking. We report the clinical course of a 700 g premature male, who was born by Caesarean section at 29 + 6 gestational weeks. On the fifth day the baby suffered from arterial thrombosis of the right pelvis axis. Antenatally a single umbilical artery was identified. Iliac arteries on the involved site appeared hypoplastic. Additionally, the prothrombin G20210A mutation was found. The patient was treated successfully using recombinant tissue plasminogen activator. In the case of a high risk of limb or organ loss due to arterial thrombosis, thrombolysis using rt-PA is justified. Appropriate rt-PA treatment has been studied for the adult but not the paediatric population. Hence, well-designed clinical trials are necessary to determine the pharmacokinetics and dynamics of thrombolytic agents in children.

Background: As a result of improved therapeutic and diagnostic modalities the survival rate of children with neoplastic disease has increased dramatically. The consequences of these scientific advances have led to increased malignancy-related critical complications requiring the expertise of intensive care practitioners., Patients: From all children admitted to the pediatric intensive care unit (PICU) of the Martin-Luther University Halle those with hematologic-oncologic condition were evaluated., Results: From 4068 PICU admissions 196 (4.8%) oncologic patients were identified. Most of them were admitted for postoperative care, monitoring or intervention. 24 patients were admitted because of severe disease or treatment related complications. 14 out of 24 (58%) patients died on PICU. Mortality was significant higher in a subgroup requiring mechanical ventilation or suffering from sepsis. All patients but two with multi-organ system failure (> or = 2 organs) died., Conclusions: Children with neoplastic disease can benefit from pediatric intensive care unit (PICU) support. Successful treatment of life-threatening complications requires a close cooperation of pediatric oncology and PICU. Further studies are necessary to improve therapeutic strategies in oncology patients requiring PICU admission.

Background: Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating disease of peripheral nerves. Antibodies acting against antigens on the myelin or the axons seem to play a causative role. In up to 80 % the onset of GBS follows an antecedent respiratory or gastrointestinal infection., Case Report: An 20 month old boy was referred to our hospital because of meningism and aspiration pneumonia. 10 days earlier the patient had experienced a period of fever. Because of respiratory insufficiency the patient was intubated, sedated and received mechanical ventilation. That's why a major neurological examination was not possible. The patient demonstrated a flaccid tetraplegic paralysis and autonomic dysfunction with elevated blood pressure, tachycardia, elevated ADH level and hyperglycaemia. Decreased motor nerve conduction and an increased CSF protein with normal CSF cell count confirmed diagnosis of GBS. Active CMV infection was diagnosed by PCR as the possible trigger factor. Intravenous immunoglobulins were given and the patient reached a complete remission except a slight disturbance of peroneal nerve., Conclusions: IVIG and PP therapy are equally effective in GBS. In contrast corticosteroids are not of benefit. Prognosis of childhood GBS is good. Only 4 % of affected children demonstrate persistent muscular weakness. The long interval between admission and diagnosis in the reported case emphasized the importance of neurological examination especially in sedated patients.

Osteoidosteomas are common bone tumours in childhood. Frequently they occur in the long bones of the lower extremities, less frequent in the humeri, phalanxes or the axial skeleton. The tumour is benign and noninfiltrative. Metastases do not occur. Typical complaints are nightly pain attacks, which are relieved by nonsteroidal antiinflammatory drugs. On X-ray, the classic finding is a small radiolucent area surrounded by sclerotic bone in the cortex. Surgical excision is often recommended, providing the possibility for a histological diagnosis. Therapeutic alternatives are percutaneous coagulation of the nidus by alcohol or laser, thermo-coagulation or high-frequency radioablation. Spontaneous remissions are well documented. Therefore the decision to wait and see and to treat with antiinflammatory medication is a considerable therapeutic option. We present two patient with an ostoidosteoma in the proximal femur. One of them underwent an unsuccessful trial for surgical extirpation and histological examination but afterwards developed a severe hip contraction with scoliosis. In the case of the other patient a primary operation was abandoned. Pain was completely controlled upon treatment with nonsteroidal antiinflammatory drugs in both patients. The contractions also disappeared completely upon physiotherapy. Serial MRI showed a significant regression of the inflammatory reaction in one case and the disappearance of the tumour in the other one. In conclusion, pharmacomedical therapy can be recommended, if the diagnosis is doubtlessly and a close follow up is established. The risk of anaesthesia and surgical treatment should be weighted against the risk of nonsteroidal antiinflammatory drug treatment.

We report an infant with severe hypotonia, feeding problems and failure to thrive in the neonatal period, followed by developmental delay. In addition, pale skin, eyelid and pedal edema, cryptorchidism and micrognathia were present. The tentative diagnosis of Prader-Labhart-Willi syndrome was made and confirmed by specific molecular testing at the age of 5 months. The Prader-Labhart-Willi syndrome is usually diagnosed in older infants when the main clinical features such as obesity, short stature, hypogonadism and developmental delay become obvious, in most of the patients typical clinical features are present already in the neonatal period. In conclusion, in neonates and young infants presenting with hypotonia and feeding problems, the Prader-Labhart-Willi syndrome should be considered.

Background: Patients (pts) with primary metastatic Ewing tumours (ET) have a poor prognosis for event free survival (EFS) compared to pts with localised disease. Following relapse the prognosis is extremely poor. Therefore these primary metastatic and relapsed pts were piloted for high dose therapy (HDT) for the last years., Patients and Methods: Between April 1984 and May 1997, 131 ET pts who underwent HDT were registered in the German CESS/EICESS office: 79 pts with primary metastases and 52 pts with relapsed tumours. After induction therapy, consisting of chemotherapy and local therapy, pts received high dose regimens, mainly based on melphalan and/or etoposide (92%). Stem cell rescue was applied from allogeneic bone marrow (n = 13), autologous bone marrow (n = 17), or peripheral blood stem cells (n = 95). The date of analysis was September 1st, 1998. Outcome was calculated by Kaplan-Meier-analyses., Results: The median time under study since high dose therapy was 3.7 years. 35/131 pts (26.7%) were in continuous complete remission, 80/131 pts (61.1%) had relapsed or progressed, 11/131 pts (8.4%) died of complications and 5/131 pts (3.8%) presented with secondary malignancies. For the total group of primary metastatic pts, EFS five years after diagnosis was 19% for pts with HDT and 27% for those without (p = 0.9209). The subgroup of pts with primary lung and bone metastases seemed to benefit from HDT (EFS five years after diagnosis: 34% versus 5%, p = 0.0001). Outcome of pts with an early ET relapse (< 2 years) was also improved by HDT (EFS four years after relapse: 17% versus 2%, p = 0.0001)., Conclusions: The total group of primary metastatic ET pts showed no obvious benefit from HDT, based on melphalan and/or etoposide. Pts with metastases to multiple organ systems, and early relapse seemed to benefit from HDT. The value of HDT should be assessed in prospective clinical trials.

Background: It has been shown that Interleukin 10 (IL-10) is able to inhibit alloreactivity in a mixed lymphocyte culture. Therefore, here IL-10 production in patients after allogeneic BMT was investigated and correlated with the incidence of acute GvHD., Patients: 14 patients after allogeneic BMT have been investigated. Patients' age ranged from 2.6 to 22 yrs. (median 7 yrs.). Patients were diagnosed with Ewing's sarcoma (1), ALL (4), AML (3), CML (2), Wiskott-Aldrich Syndrome (WAS;1), MDS (1) and SAA (2). GvHD > II degrees occurred in 5/14 patients. As control served 20 healthy volunteers., Methods: Mononuclear cells (MNC's) isolated from patients and 20 healthy controls were stimulated with an anti-CD3 monoclonal antibody for 72 hr. IL-10 was detected in cell-free supernatants by ELISA., Results: Anti-CD3-induced IL-10 production in MNC's isolated from patients (range/median: 0-1579 pg/10(6) MNC; 221 pg/10(6) MNC) was significantly reduced compared to healthy controls (160-5093 pg/10(6) MNC; 1250 pg/10(6) MNC; p < 0.01). 4/5 patients with low IL-10 production, but only 1/9 with a normal IL-10 production presented with GvHD > II degrees (p < 0.05)., Conclusion: Ex vivo IL-10 production was decreased in about one third of patients early after allogeneic BMT. The low IL-10 production was associated with a significantly increased risk of severe GvHD. Thus, supplementation of IL-10 might become a useful therapy to prevent GvHD.

This article describes the basic definitions, indications and complications of hematopoietic stem cell transplantation. The different modes of transplantation (autologous, allogeneic-related and allogeneic-unrelated transplantation) are explained with regard to the underlying immunological processes and consequences for duration of treatment, distribution of age, complications, lethality, and for the family of the patient. In our department, the duration of the hospital stay was (median) 44 days for autologous BMT, 45 days for allogeneic-related BMT and 66 days for allogeneic-unrelated BMT. Six to twelve years old children showed a peak for allogeneic related transplantations; these children were treated mainly for relapse of acute lymphoblastic or myeloid leukemias. Patients over 15 years old showed a peak for autologous transplantations; due to the research focus of our center, Ewing's sarcoma was the main underlying disease in this age group. Allogeneic-unrelated transplantations were uniformly distributed over the whole age range from nine months to 24 years.

Growth and differentiation of hematopoietic progenitor cells is regulated by a complex network of stimulatory and inhibitory cytokines. Bone marrow failures can be due to a decrease of stimulators or an increase of inhibitors. T cells produce both, hematopoiesis stimulating and inhibiting cytokines. Therefore, a role of T cells in regulating hematopoiesis can only be assumed if the gene expression of these antagonistic acting cytokines can be differentially induced in T cells. To establish a model of selective cytokine induction, we investigated the induction of IFN gamma as inhibitor and GM-CSF as stimulator of hematopoiesis in T cells. Our results showed that IFN gamma mRNA accumulates in T cells which have been pre-activated via the signal transduction unit CD3, but not in unstimulated T cells. This accumulation depends on the expression of the high affinity IL2 receptor which is including the IL2 receptor alpha-chain (IL2R alpha, CD25). In a study on children with constitutional (CAA) versus acquired aplastic (EAA) anemia, we investigated the relevance of this model for the pathogenesis of aplastic anemia in childhood. We compared the following parameters: 1. Incidence of hematopoietic progenitor cells and cloning efficiency, 2. activation status and IL2R alpha expression of bone marrow T cells, 3. T cell cytokine expression profile. Our results show: 1. The relative incidence of bone marrow progenitor cells is decreased in children with CAA and normal in children with EAA. 2. Clonogenic growth of hematopoietic progenitor cells is suppressed in children with EAA.(ABSTRACT TRUNCATED AT 250 WORDS)